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Queen Elizabeth II's Sparkling Emerald South African Banquet Tiara
John Stillwell/PA Images/Alamy
Ahead of next week's South African state visit to the United Kingdom, we're devoting this week's Sparkling Spotlight series to some of the glittering jewels that Queen Elizabeth II wore for South African state banquets in the later years of her reign. We begin with a set of gorgeous emeralds worn for Nelson Mandela's 1996 state visit to Britain.
President Mandela's landmark state visit to Britain took place in July 1996. He was accompanied by his daughter, Zenani Mandela-Dlamini, who is a princess through her marriage to Prince Thumbumuzi Dlamini of Eswatini. (Her brother-in-law is the current monarch, King Mswati III.) The Brits rolled out the traditional red carpet for their guests, throwing a grand state banquet at Buckingham Palace in their honor on the first evening of the visit. The Queen hailed Mandela's "wisdom, understanding, and experience" during her speech at the dinner.
POOL/AFP via Getty Images
Queen Elizabeth II wore an ivory evening gown decorated with large sequin bows for the banquet. The gold color of the sequins coordinated with the gold and green sash of her South African decoration, the Order of Good Hope.
She coordinated her jewels with the emerald green shade from her order sash, wearing pieces from Queen Mary's famed Cambridge Emerald collection. The feature piece was the Vladimir Tiara in its emerald setting. She also added the necklace, earrings, and bracelet from the Delhi Durbar Parure. Her brooch is also from the same suite—it's part of the larger stomacher from the set. She finished off the look with a diamond evening watch and the Royal Family Orders of her father and grandfather.
President Mandela wore an important decoration as well: the neck badge of the Order of Merit. Queen Elizabeth II had appointed him as an honorary member of the order the previous year.


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Genetics| July 01 2007
Mutations in ATP-Sensitive K+ Channel Genes Cause Transient Neonatal Diabetes and Permanent Diabetes in Childhood or Adulthood
Sarah E. Flanagan;
Sarah E. Flanagan
1Institute of Biomedical and Clinical Science, Peninsula Medical School, Exeter, U.K
Ann-Marie Patch;
Ann-Marie Patch
Deborah J.G. Mackay;
Deborah J.G. Mackay
2Wessex Regional Genetics Laboratories, Salisbury District Hospital, Salisbury, U.K
3Division of Human Genetics, Southampton University, Southampton, U.K
Emma L. Edghill;
Emma L. Edghill
Anna L. Gloyn;
Anna L. Gloyn
4Diabetes Research Laboratories, Oxford Centre for Diabetes Endocrinology and Metabolism, University of Oxford, Oxford, U.K
David Robinson;
Julian P.H. Shield;
Julian P.H. Shield
5The Royal Hospital for Children, Bristol, U.K
Karen Temple;
Karen Temple
6Wessex Clinical Genetics Service, National Health Service Trust, Southampton, U.K
Sian Ellard;
Sian Ellard
Andrew T. Hattersley
Address correspondence and reprint requests to Professor Andrew T. Hattersley, Peninsula Medical School, Barrack Road, Exeter, EX2 5DW, U.K. E-mail: a.t.hattersley@exeter.ac.uk
Diabetes 2007;56(7):1930–1937
A correction has been published: Erratum
Sarah E. Flanagan, Ann-Marie Patch, Deborah J.G. Mackay, Emma L. Edghill, Anna L. Gloyn, David Robinson, Julian P.H. Shield, Karen Temple, Sian Ellard, Andrew T. Hattersley; Mutations in ATP-Sensitive K+ Channel Genes Cause Transient Neonatal Diabetes and Permanent Diabetes in Childhood or Adulthood. Diabetes 1 July 2007; 56 (7): 1930–1937. https://doi.org/10.2337/db07-0043
Transient neonatal diabetes mellitus (TNDM) is diagnosed in the first 6 months of life, with remission in infancy or early childhood. For ∼50% of patients, their diabetes will relapse in later life. The majority of cases result from anomalies of the imprinted region on chromosome 6q24, and 14 patients with ATP-sensitive K+ channel (KATP channel) gene mutations have been reported. We determined the 6q24 status in 97 patients with TNDM. In patients in whom no abnormality was identified, the KCNJ11 gene and/or ABCC8 gene, which encode the Kir6.2 and SUR1 subunits of the pancreatic β-cell KATP channel, were sequenced. KATP channel mutations were found in 25 of 97 (26%) TNDM probands (12 KCNJ11 and 13 ABCC8), while 69 of 97 (71%) had chromosome 6q24 abnormalities. The phenotype associated with KCNJ11 and ABCC8 mutations was similar but markedly different from 6q24 patients who had a lower birth weight and who were diagnosed and remitted earlier (all P < 0.001). KATP channel mutations were identified in 26 additional family members, 17 of whom had diabetes. Of 42 diabetic patients, 91% diagnosed before 6 months remitted, but those diagnosed after 6 months had permanent diabetes (P < 0.0001). KATP channel mutations account for 89% of patients with non-6q24 TNDM and result in a discrete clinical subtype that includes biphasic diabetes that can be treated with sulfonylureas. Remitting neonatal diabetes was observed in two of three mutation carriers, and permanent diabetes occurred after 6 months of age in subjects without an initial diagnosis of neonatal diabetes.
Transient neonatal diabetes mellitus (TNDM) is a clinically defined subgroup affecting ∼50% of children with neonatal diabetes. TNDM is differentiated from permanent neonatal diabetes mellitus (PNDM) because the diabetes remits in infancy or early childhood. Relapse in childhood or adolescence occurs in up to 50% of cases (1).
There has been considerable progress in defining the genetic etiology of TNDM. The major breakthrough was establishing that the majority of cases result from anomalies of the imprinted region on chromosome 6q24, which encodes the ZAC and HYMAI genes (2,3). Three types of abnormalities have been identified to date that result in the overexpression of the paternal allele: paternally inherited duplication of chromosome 6q24, paternal uniparental isodisomy of chromosome 6, or a methylation defect.
Recent studies have identified 14 patients with TNDM resulting from mutations in the KCNJ11 and ABCC8 genes that encode the Kir6.2 and SUR1 subunits of the potassium ATP-sensitive K+ channel (KATP channel) in the pancreatic β-cell (4–6,8). Heterozygous activating mutations in the KCNJ11 gene are the commonest cause of PNDM but also have been described in five patients with TNDM (4–6). Functional work illustrated that TNDM mutations were functionally less severe in vitro than mutations resulting in PNDM (4). Activating heterozygous ABCC8 mutations have recently been reported in three patients with PNDM and seven with TNDM (7,8). Mutations in either gene encoding the subunits of the KATP channel cause diabetes by reducing the ability of the channel to close in response to increased ATP and reduced ADP concentrations. This altered sensitivity of the channel to the ratio of ATP to ADP results in increased potassium efflux from the cell and hyperpolarization of the membrane, thus reducing insulin secretion from the β-cell. The mechanism by which KATP channel mutations result in a remitting/relapsing diabetes phenotype is not known.
The prevalence of KATP channel mutations in TNDM and the associated clinical characteristics are uncertain. The initial descriptions suggest that the onset of diabetes and remission is later in TNDM patients with KATP channel mutations (1,4). Since the original criteria for TNDM are based on a series of patients with 6q24 diabetes (diabetes diagnosed within 6 weeks of birth with remission by 18 months) (9), this definition may exclude some cases with KATP channel mutations. We and others (10,11) have shown that type 1 diabetes is very rare before 6 months of age and suggests a genetic etiology for most patients diagnosed before 6 months. TNDM might therefore be redefined as diabetes diagnosed before 6 months of age that later remits.
We investigated the genetic etiology of 97 patients whose diabetes was diagnosed within the first 6 months of life but entered remission before age 5 years. Our aim was to assess the prevalence of 6q24 abnormalities and KATP channel mutations within this cohort and to investigate the clinical features of mutation carriers.
We studied 97 patients with diabetes diagnosed in the first 6 months of life whose diabetes remitted before the age of 5 years. Sixty-four have been previously reported (1,3,4,12–15). The patients were referred from 14 different countries across four continents and were either recruited following a request for referrals to the International Society of Pediatric and Adolescent Diabetes rare diabetes collection or following referral to the Wessex Regional Genetics Laboratory for 6q24 abnormality testing. The study was conducted in accordance with the Declaration of Helsinki, as revised in 2000. Informed consent was obtained from all patients, with parental consent given on behalf of children.
Genetic analysis.
Genomic DNA was extracted from peripheral lymphocytes using standard procedures. In all patients, analysis of the 6q24 locus was undertaken using previously described methods to detect duplications, uniparental isodisomy and methylation abnormalities (1,13). In patients for whom no 6q24 abnormality was identified, the single exon of KCNJ11 was amplified in three overlapping fragments, as previously described (16). The 39 exons of ABCC8 were analyzed in all patients in whom no KCNJ11 mutation was identified. The ABCC8 gene was amplified in 38 fragments using previously described primers (7). PCR products were sequenced using standard methods on an ABI 3100 or ABI 3730 (Applied Biosystems, Warrington, U.K.). Sequences were compared with the published sequences ([KCNJ11 − NM_000525.3] [ABCC8 − NM_000352.2]) using Staden analysis or Mutation Surveyor version 2.61. Mutations were tested for cosegregation with diabetes in other family members and in 200 normal chromosomes from U.K. Caucasians. Where possible, family relationships were confirmed using a panel of six microsatellite markers on chromosome 11p (16). Clinical characteristics were obtained from the patient's hospital records with assistance from their physician. Clinical characteristics are presented as median (range), and comparative statistics predominantly used the Mann-Whitney U test.
Prevalence of different genetic aetiologies.
We have determined the genetic etiology in 94 of 97 (97%) probands diagnosed with diabetes before the age of 6 months whose diabetes had subsequently entered remission (Fig. 1). The majority of patients (69 of 97) had an abnormality at the 6q24 locus. Of these patients, 27 (39%) had paternal uniparental isodisomy, 27 (39%) had a duplication of the paternal allele, and 15 (22%) had a methylation defect. For the 28 remaining patients, in whom no 6q24 aberration was identified, sequencing of KCNJ11 and ABCC8 demonstrated that 25 probands were heterozygous for KATP channel mutations.
Mutation characteristics
KCNJ11 mutations.
Twelve of the probands had a heterozygous mutation in the KCNJ11 gene; six of these patients (with five different mutations) have been reported previously (4,12). We found eight different KCNJ11 mutations; R34C (c.100C>T), G53R (c.157G>C), G53S (c.157G>A), E179A (c.536A>C), I182V (c.544A>G), E227K (c.679G>A), E229K (c.685G>A), and R365H (c.1094G>A). Two mutations were identified in more than one proband (E227K, n = 2, and E229K, n = 4). The mutations R34C, E179A, and R365H are novel. We did not observe these mutations in 200 normal chromosomes, and all mutations affect residues that are conserved in human, mouse, rat, and dog (http://genome.ucsc.edu).
ABCC8 mutations.
Ten different ABCC8 gene mutations were identified in 13 probands: D209E (c.627C>A), D212N (c.634G>A), D212I (c.634 G>A 635A>T), V324M (c.970G>A), L451P (c.1352T>C), R826W (c.2476C>T), R1183W (c.3547C>T), R1183Q (c.3548G>A), R1380C (c.4138C>T), and R1380H (c.4139G>A). All were identified in one proband, with the exception of R1183W, which was identified in four probands. All mutations were novel except for R1380C and R1183Q (8). We did not observe these mutations in 200 normal chromosomes, and all affect residues that are conserved in human, mouse, rat, and dog (http://genome.ucsc.edu).
Inheritance of mutations.
Seven (28%) probands had de novo mutations as confirmed by the absence of the mutation in the unaffected parents (KCNJ11, n = 3, and ABCC8, n = 4). Microsatellite analysis was used to verify the family relationships in all but one case with an R1380C ABCC8 mutation, in whom parental DNA was unavailable. In the remaining 17 probands, the mutation had been inherited from a parent (partial pedigrees are shown in Fig. 2). In these families, 17 of 26 parents or siblings with the KATP channel mutation had diabetes; in contrast, only 1 family member without the mutation was affected (P < 0.001). However there was not complete cosegregation of the mutations with diabetes because in eight probands the mutation was inherited from a nondiabetic parent. Neonatal diabetes (diagnosed in the first 6 months) was described in five siblings and four parents. Two mothers (identical twins, Fig. 2) were reported to have had diabetes in the neonatal period, although information regarding their ages at diagnosis and remission was not available. Nonpenetrance was seen in families with eight different mutations, suggesting that it was not a feature of specific mutations.
Clinical characteristics of probands with KCNJ11 or ABCC8 mutations
The characteristics of diabetes in the probands with KCNJ11 or ABCC8 mutations are shown in Table 1. There was no significant difference in clinical characteristics between these two groups. Reduced birth weight was a consistent feature of both KCNJ11 and ABCC8 mutations (25th vs. 11th percentile; P = 0.26). Diabetes was most frequently diagnosed between 0 and 8 weeks (86%), with all cases diagnosed before 17 weeks. None of the patients with an ABCC8 mutation had relapsed at the time of this study; however, this may be due to the younger age of these patients compared with those with KCNJ11 mutations.
Neurological features.
In addition to diabetes, neurological features were identified in four probands (16%) and were associated with both KCNJ11 and ABCC8 mutations. Two probands and one family member with a KCNJ11 mutation had neurological features. The first proband with an E229K KCNJ11 mutation has reported speech delay and was diagnosed with autistic spectrum disorder at the age of 3 years. Interestingly, the affected sibling and unaffected mother who both carry the same mutation have no reported speech problems, although the sibling is currently 2 years of age and still in the early stages of language development. The E229K mutation has been identified in five affected patients from three further families, with no reports of speech or developmental delay in any of the mutation carriers. Mild learning difficulties were observed in a second proband and his mother who both have the G53R KCNJ11 mutation. This mutation has not been identified in any other families to date; therefore, it is difficult to conclude whether these complications are a feature of the mutation or whether environmental and/or other genetic factors are involved.
Neurological features were also reported in three patients with an ABCC8 mutation. Muscle weakness was identified in two cousins with a D212I mutation. The proband had muscle hypotonia until the age of 8 months, and her cousin has been diagnosed with motor developmental delay, although no muscle weakness was reported in either of their affected mothers. The most severe neurological phenotype was identified in a proband with a de novo ABCC8 mutation (R1183W). This patient had one episode of tonic posturing with right facial involvement following admission to the hospital with diabetic ketoacidosis. He subsequently had two episodes of generalized seizures shortly after diagnosis of diabetes. No further seizures have been reported since insulin therapy was started, and it is likely that these seizures were a result of the severe hyperglycemia rather than as part of the DEND (Developmental Delay, Epilepsy, and Neonatal Diabetes) syndrome (17). Neurological features have not been reported in any of the five other patients with the R1183W mutation.
The clinical characteristics of the probands with KATP channel mutations were markedly different when compared with subjects with 6q24 abnormalities (Table 2). Patients with a 6q24 abnormality presented with a more severe clinical phenotype, as shown by their lower percentile birth weight (<1st vs. 12th; P < 0.001) and earlier age of diagnosis (0 vs. 4 weeks; P < 0.001). For patients with a KATP channel mutation, the initial episode of diabetes was longer, with diabetes remitting significantly later than in patients with a 6q24 abnormality (35 vs. 13 weeks; P < 0.001). There is a trend toward an earlier age of relapse in patients with a KATP channel mutation (P = 0.07), and this may become more evident as the patients are studied over a longer period of time.
Clinical characteristics of the KATP channel mutations vary according to the age of diagnosis of initial diabetes.
The characteristics of the diabetes varied according to the age at which it was first diagnosed (see Table 3). Remission of diabetes occurred in 32 of 35 (91%) patients diagnosed before 6 months but in none of those diagnosed after 6 months (P < 0.001) (Table 3 and Fig. 3). The median age at remission was 35 weeks (range 2–208). The three patients diagnosed before 6 months who had not entered remission were 1, 2, and 43 years of age at mutation screening.
All patients were treated with insulin during their initial episode of diabetes and during any subsequent relapse of diabetes, except for one proband and his father with a KCNJ11 mutation (E227K), a father with a KCNJ11 mutation (E229K), and a mother with an ABCC8 mutation (D209E), who were treated with sulfonylureas. Two probands and four family members have now attempted to transfer off insulin and onto sulfonylureas using a protocol similar to that described for KCNJ11 mutations (18). Transfer was successful in five cases (83%) but unsuccessful in a mother with a G53R mutation. Her son, who carries the same mutation, has successfully transferred onto sulfonylureas, suggesting that the observed lack of response to sulfonylureas is not a feature of the mutation.
Phenotype of patients in whom the genetic etiology is not defined.
We were able to determine the genetic etiology in all but 3 of 97 probands diagnosed with TNDM. Interestingly, all three patients had an atypical phenotype. The first patient was born at 30 weeks' gestation, and his transient hyperglycemia was possibly a consequence of the premature birth. Early gestational age was not a consistent feature in patients with either a 6q24 anomaly or a KATP channel mutation. The second patient stopped insulin treatment at the age of 4 years, but this was recommenced 1 year later. He also developed epilepsy at the age of 9 years, which has not been reported in any other patients in our series. The third patient has had four episodes of remission and relapse, a unique phenotype that has also not been described in any other patients included in this study. In addition, the coding sequence of the glucokinase gene was normal in all three patients. This phenotypic variation suggests further rare distinct etiologies for TNDM.
We have shown that mutations in the KCNJ11 and ABCC8 genes that encode the Kir6.2 and SUR1 subunits of the KATP channel are a major cause of TNDM, accounting for 29% of all cases and 89% of non-6q24 TNDM. As a result, a genetic diagnosis could be made in 97% of patients with neonatal diabetes that remitted. These estimates of the relative prevalence of the three major genetic subgroups are likely to be robust, as this study of a consecutive series of 97 TNDM patients is more than twice the size of the largest previous study. In our series, KCNJ11 (n = 12) and ABCC8 (n = 13) mutations were equally represented, which is in marked contrast to PNDM, for which there are >60 KCNJ11 mutations and only 3 ABCC8 mutations reported (7,8). The only previous comprehensive study is from France, which showed that a genetic diagnosis was possible for 33 of 44 patients with TNDM (8). This is in contrast to the current study, in which the genetic etiology remains unknown in only 3 of 97 patients. The reason for this discrepancy is unknown; it could result from differences in the patients studied or in the detection of KATP channel mutations. KCNJ11 mutations have now been identified in a total of 18 patients with TNDM (5,6,19). Our study emphasizes that both KCNJ11 and ABCC8 mutations are an important cause of TNDM.
Defining mutations.
There is strong evidence that the mutations detected in KCNJ11 or ABCC8 are the cause of TNDM in these patients. All of the mutations occur at residues that are conserved across multiple species, alter the coding sequence (an essential requirement for an activating channel mutation), and are not found in normal control subjects or patients with PNDM. The strongest evidence for an etiological role is the de novo mutations, as spontaneous mutations are rare, even in genes as large as ABCC8 (8.5 × 10−5; 20). However, de novo mutations are only seen for 4 of the 14 novel mutations (29%) in contrast to PNDM, in which 84% of cases represent spontaneous mutations (21). For the majority of cases (68%), the mutation is inherited from a parent, and although cosegregation of TNDM is rare, all cases of TNDM in the families have inherited the mutation. Fifty percent of parents have diabetes diagnosed before 45 years, which is rare in the general population (<0.2%) (calculated from 22) and is not seen in any of the non–mutation-carrying parents. The final evidence that these mutations are pathogenic is that functional studies for all mutations performed to date (G53R, G53S, I182V, E227K, and E229K in KCNJ11) have shown altered response to ATP in the presence of magnesium ions (4,8,19). The presence of de novo mutations identified in five probands strongly supports an etiological role for the I182V, E229K, R1380C, and R1183Q mutations. Therefore, these mutations are highly likely to be causative of TNDM, even in the absence of complete cosegregation.
Location and function of mutations.
We have identified a number of commonly mutated residues in patients with TNDM within both KATP channel genes. KCNJ11 mutations in our TNDM series cluster with residue G53 mutated in 2 of 12 probands and E227 and E229 residues mutated in 6 of 12 probands. Structural analysis has shown that G53 lies in a region linking the ATP binding site to the transmembrane domain (4), whereas E227 and E229 lie distant to the ATP binding site at the interface between Kir6.2 subunits and are thought to influence channel gating (19).
The ABCC8 mutations are located throughout the SUR1 protein. The residue R1183, which is located at a position involved in joining transmembrane domain 2 to nucleotide binding domain 2, was mutated in five probands with two different residues, tryptophan and glutamine substituting arginine. The second most commonly affected residue was R1380, which is located in nucleotide binding domain 2 and is mutated in two probands. There are a cluster of mutations (D209E, D212N, and D212I) in the intracellular region that links the transmembrane domain with the gatekeeper module (8).
Functional studies of channels with the TNDM-associated KCNJ11 mutations G53S, G53R, and I182V showed a moderate reduction in activity in the presence of ATP, which is less marked than the reduction seen with PNDM mutations (4). This suggested clear association of the genotype with functional effect as well as clinical phenotype, but further studies have shown that there is some overlap between the magnitude of the KATP channel currents in TNDM- and PNDM-associated mutations (19). Furthermore, the V252A and R201H mutations have been described in both patients with PNDM and TNDM (6,19), so the genotype/phenotype correlation is not absolute.
A biphasic course for diabetes in KCNJ11 and ABCC8 mutations associated with TNDM.
We have shown that patients with KATP channel mutations, when diagnosed within the neonatal period, have similar clinical characteristics irrespective of whether KCNJ11 or ABCC8 is mutated (Table 1). The TNDM probands with KATP channel mutations show a biphasic course in which diabetes is diagnosed before age 6 months. They then typically go into remission between age 6 and 12 months and are likely to relapse during adolescence or early adulthood. None of the patients diagnosed with diabetes after 6 months of age had a remission of diabetes, even if they had the same mutation as patients who remitted. Interestingly, birth weight, a reflection of insulin-mediated growth and, hence, insulin secretion in utero, is reduced to a similar extent whether the patient is diagnosed before 6 months or not. These findings imply that the KATP channel mutations have a biphasic course, and patients diagnosed later may have had a period of hyperglycemia that was undetected in the neonatal period. This could explain the apparent phenotypic heterogeneity with the same mutation. Importantly, this means that KATP channel mutations can present outside the neonatal period, and, if the parents in our families had not had a child with TNDM, they would have been diagnosed as having type 1 or type 2 diabetes. It will be important to test cohorts with a clinical diagnosis of type 1 diabetes to see how prevalent these mutations are, especially because it will have implications for treatment.
The explanation of the biphasic course in both types of KATP channel mutations is not known. A fixed-channel defect would be expected to produce a fixed phenotype, as seen with KATP channel mutations causing PNDM. The change seen in glucose tolerance during remission and subsequent relapse likely reflects either changes in insulin requirements or changes in the number of β-cells or both. As insulin levels are very low in nondiabetic neonates (23), this would suggest that the initial diabetes does not reflect increased insulin requirements at this time, although the relapse around puberty may reflect the associated increase in insulin resistance. The biphasic course seen in 6q24 TNDM is still not fully understood, even with the creation of a good mouse model (24).
Neurological complications were present in addition to diabetes in six patients with an ABCC8 or KCNJ11 mutation. In all cases, the affected individual had been diagnosed with diabetes before the age of 6 months. In one family, both mutation carriers had learning difficulties. In all other families, neurological features did not cosegregate with the mutation, suggesting that neurological phenotype is not a consistent feature of these mutations.
6q24 TNDM differs from TNDM resulting from KATP channel mutations.
Our series provides strong evidence that clinical characteristics differ markedly between patients with a KATP channel mutation and those with a chromosome 6q24 anomaly. In addition to diabetes, seven patients with a 6q24 abnormality (30%) had macroglossia and two patients had umbilical hernia (9%). These abnormalities were not present in any of the patients with a KATP channel mutation. Patients with KATP channel TNDM have a higher birth weight (2,570 vs. 1,950 g), are diagnosed later (4 vs. 0 weeks), and enter remission later (35 vs. 13 weeks) than patients with 6q24 abnormalities. Although these clinical characteristics can aid in the identification of the cause of TNDM, there is overlap in the range of values (Table 2). Therefore, while clinical features can guide the order in which genetic tests are performed, a molecular genetic diagnosis is still required.
Phenotype of TNDM versus PNDM mutations.
There is a clear genotype/phenotype relationship. None of the KATP channel mutations identified within this series have been found in patients with PNDM. Only one report, to date, has identified R201H, a well-characterized PNDM mutation, in a patient with TNDM (6). Between the TNDM and PNDM groups, there are no significant differences between the percentile birth weights and age of diagnosis (12th vs. 3rd percentile and 4 vs. 5 weeks) (16). Distinction between the two subgroups can be made only by the presence of a period of remission in the TNDM patients. One difference between the groups is the mode of inheritance. In our series, a greater number of probands (68%) have a familial mutation. This is in contrast to PNDM, in which 84% of all mutations occur de novo (21).
Therapeutic implications.
Identification of a KATP channel mutation has important implications for patient clinical management. Recent reports (19,25,26) have shown that most patients with a KCNJ11 mutation and PNDM are able to transfer from insulin onto sulfonylurea treatment with an improvement in glycemic control in all cases reported. Babenko et al. (8) reported two patients with an ABCC8 mutation who were successfully treated with sulfonylureas after their TNDM relapsed. In our series, 3 of the 14 patients who developed diabetes outside the initial 6 months (either as a relapse or initial diagnosis) were initially treated with sulfonylureas. Since the diagnosis was made, a further five patients have successfully transferred onto sulfonylureas from insulin using a similar protocol to that described for the transfer of patients with PNDM and a KCNJ11 mutation (18). For the remaining patients, transfer is either in progress or the patients are currently in remission.
In conclusion we have shown that a genetic diagnosis is possible for 97% of patients diagnosed with TNDM. Mutations of the KATP channel genes, ABCC8 and KCNJ11, account for the majority (89%) of non-6q24 TNDM. Diabetes associated with these KATP channel mutations has a biphasic course, although neonatal diabetes that enters remission may not be detected. Patients therefore may present with permanent diabetes diagnosed in childhood or adulthood. In addition, not all mutation carriers may be diabetic when the mutation is detected in a proband. The therapeutic implications for those patients found to have a KATP channel mutation highlights the importance of screening all patients with non-6q24 TNDM for mutations in ABCC8 and KCNJ11.
Number of patients diagnosed with TNDM and results of genetic testing. UPD, uniparental isodisomy.
Partial pedigrees of families showing inheritance of KCNJ11 or ABCC8 mutations. Squares represent male family members, and circles represent female subjects. Filled symbols denote patients with diabetes. Genotype is shown underneath each symbol, residue number and amino acid change are given for the mutation carriers, and N/N denotes no mutation identified. Directly below the genotype is the age of initial diagnosis for the mutation carriers, followed by the age of remission and the age of relapse. A dash represents a nonevent, and N/A denotes information not available. An arrow with the letter P points to the proband in each family.
A: Patients diagnosed with diabetes before 6 months of age, which subsequently entered remission. The diagram illustrates the percentage of patients diabetic at any given age. Black columns represent the initial diagnosis, and grey columns represent the second episode of diabetes (relapse). Percentages were calculated according to the number of patients who had reached that given age. B: Patients diagnosed with diabetes after 6 months of age. The diagram represents the percentage diabetic at any given age. Percentages were calculated according to the number of patients who had reached that given age.
Clinical characteristics of probands with an unknown genetic etiology and patients with a KATP channel mutation
Characteristic .
Genetic etiology unknown .
KCNJ11 .
ABCC8 .
Comparison of KCNJ11 and ABCC8 mutation carriers (P value) .
n (% male) 3 (100) 12 (58) 13 (54) 0.33
Age at diagnosis (weeks) 3 (1–4) 5 (0–16) 4 (0–9) 0.53
Age at remission (weeks) 8 (17–208) 45 (2–208) 22 (7–52) 0.14
Age when entering study (years) 14 (1–31) 7.5 (0.84–17) 5 (0.84–16) —
Diabetes relapsed (n) 2 4 0 —
Age at relapse (years) 11 (5–17) 4.7 (3–15) — —
Gestation (weeks) 38 (30–40) 38 (30–40) 39 (30–41) 0.17
Birth weight (g) 1,620 (1,100–3,373) 2,570 (1,535–3,570) 2,575 (1,360–3,400) 0.73
Centile birth weight 6 (<1st to 35th) 25 (<1st to 89th) 11 (<1st to 32nd) 0.26
Data are median (range), unless otherwise indicated. Comparison of clinical characteristics of patients with KCNJ11 mutation and ABCC8 mutation. Differences between groups were calculated using Mann-Whitney U and χ2 tests. Centile birth weights were calculated according to U.K. growth charts (27) because the majority of patients were of U.K. white origin.
Clinical characteristics of probands grouped by genetic etiology
Combined KCNJ11 and ABCC8 .
6q24* .
Comparison of KCNJ11 and ABCC8 combined data and 6q24 patients (P value) .
n (% male) 25 (56) 23 (53) 0.94
Age at diagnosis (weeks) 4 (0–16) 0 (0–4) <0.001
Age at remission (weeks) 35 (2–208) 13 (5–60) <0.001
jAge when entering study (years) 6 (0.84–17) 12 (1–36) —
Diabetes relapsed (n) 4 7 —
Age at relapse (years) 4.7 (3–15) 16 (4–25) 0.073
Gestation (weeks) 38 (30–41) 40 (36–42) 0.059
Birth weight (g) 2,570 (1,360–3,570) 1,950 (1,600–2,670) <0.001
Centile birth weight 12 (<1st to 89) <1st (<1st to 21) <0.001
Data are median (range), unless otherwise indicated. Comparison of probands with a KATP channel mutation (combined results) to patients with a 6q24 abnormality.
Data previously reported (1). Differences between groups were calculated using Mann-Whitney U and χ2 tests. Centile birth weights were calculated according to U.K. growth charts (27) because the majority of patients were of U.K. white origin.
Comparison of clinical and biochemical characteristics of patients with a KATP channel mutation diagnosed before 6 months of age with patients whose diabetes was not diagnosed before age 6 months and the number of each mutation identified within each group
Mutation carriers diagnosed with diabetes within 6 months .
Mutation carriers who did not have diabetes diagnosed within the first 6 months .
P value .
Probands (n) 25 0
Age when entering study (years) 6 (0.8–43) 42 (5–56) —
Ever diagnosed with diabetes (n) 35 7 1*10−6
Age at diagnosis (weeks) 4 (0–17) 1196 (260 to >2496) 3.7*10−5
Diabetes remitted (n) 32 0/7 3.7*10−10
Age at remission (weeks) 35 (2–208) — —
Diabetes relapsed (n) 7 — —
Age at relapse (years) 13 (3–25.5) — —
Birth weight (g) 2,695 (1,360–3,570) 2,810 (907–3,090) 0.9
Gestation (weeks) 39 (30–42) 38 (34–40) 0.74
Centile birth weight 18 (<1st to 89th) 15 (<1st to 79th) 0.94
KCNJ11 mutations
R34C 1 2
G53R 2 0
G53S 2 1
E179A 1 0
I182V 1 0
E227K 4 2
R365H 1 1
ABCC8 mutations
D209E 1 1
D212N 2 1
D212I 4 0
V324M 1 1
L451P 2 1
R826W 1 0
R1183W 4 2
R1183Q 1 0
R1380C 1 0
R1380H 1 1
Data are median (range), unless otherwise indicated. Differences between groups were calculated using Mann-Whitney U and χ2 tests. Percentile birth weights were calculated according to U.K. growth charts (27) because the majority of patients were of U.K. white origin.
Published ahead of print at http://diabetes.diabetesjournals.org on 19 April 2007. DOI: 10.2337/db07-0043.
We acknowledge funding from the Sir Graham Wilkins studentship to S.E.F. The study was funded by the Wellcome Trust and was supported by the European Union (Integrated Project EURODIA LSHM-CT-2006-518153 in the Framework Programme 6 of the European Community). A.T.H. is a Wellcome Trust Research Leave fellow. A.L.G. is a Diabetes UK R.D. Lawrence Research Fellow.
The authors thank all of the families for participating in this study. Our thanks go to the many referring clinicians who include Ijaz Ahmad, Jesús Argente, Chandar Batra, Mark Bone, Jill Challener, Ethel Codner, Elizabeth Davis, Dorothy Deiss, Jan Fairchild, Peter Fowlie, Mathias Herr, Sabine Hofer, Mary Jetha, Olga Kordonouri, Michael MacDonald, Kathryn Noyes, Laurent Legault, Derek Sandeman, Annabelle Slingerland, Zdenek Sumnick, Peter Swift, Charalambos Theodoridis, Verena Wagner, and Esko Wiltshire. We also thank Andrew Parrish for his technical assistance and Dr. Beverley Shields for statistical analysis.
Temple IK, Gardner RJ, Mackay DJ, Barber JC, Robinson DO, Shield JP: Transient neonatal diabetes: widening the understanding of the etiopathogenesis of diabetes.
Temple IK, Gardner RJ, Robinson DO, Kibirige MS, Ferguson AW, Baum JD, Barber JCK, James RS, Shield JPH: Further evidence for an imprinted gene for neonatal diabetes localised to chromosome 6q22–q23.
Hum Molec Genet
Gardner RJ, Mackay DJ, Mungall AJ, Polychronakos C, Siebert R, Shield JP, Temple IK, Robinson DO: An imprinted locus associated with transient neonatal diabetes mellitus.
Hum Mol Genet
Gloyn AL, Reimann F, Girard C, Edghill EL, Proks P, Pearson ER, Temple IK, Mackay DJ, Shield JP, Freedenberg D, Noyes K, Ellard S, Ashcroft FM, Gribble FM, Hattersley AT: Relapsing diabetes can result from moderately activating mutations in KCNJ11.
Yorifuji T, Nagashima K, Kurokawa K, Kawai M, Oishi M, Akazawa Y, Hosokawa M, Yamada Y, Inagaki N, Nakahata T: The C42R mutation in the Kir6.2 (KCNJ11) gene as a cause of transient neonatal diabetes, childhood diabetes, or later-onset, apparently type 2 diabetes mellitus.
J Clin Endocrinol Metab
Colombo C, Delvecchio M, Zecchino C, Faienza MF, Cavallo L, Barbetti F: Transient neonatal diabetes mellitus is associated with a recurrent (R201H) KCNJ11 (KIR6.2) mutation.
Proks P, Arnold AL, Bruining J, Girard C, Flanagan SE, Larkin B, Colclough K, Hattersley AT, Ashcroft FM, Ellard S: A heterozygous activating mutation in the sulphonylurea receptor SUR1 (ABCC8) causes neonatal diabetes.
Babenko AP, Polak M, Cave H, Busiah K, Czernichow P, Scharfmann R, Bryan J, Aguilar-Bryan L, Vaxillaire M, Froguel P: Activating mutations in the ABCC8 gene in neonatal diabetes mellitus.
Temple IK, Shield JP: Transient neonatal diabetes, a disorder of imprinting.
J Med Genet
Edghill EL, Dix RJ, Flanagan SE, Bingley PJ, Hattersley AT, Ellard S, Gillespie KM: HLA genotyping supports a nonautoimmune etiology in patients diagnosed with diabetes under the age of 6 months.
Iafusco D, Stazi MA, Cotichini R, Cotellessa M, Martinucci ME, Mazzella M, Cherubini V, Barbetti F, Martinetti M, Cerutti F, Prisco F: Permanent diabetes mellitus in the first year of life.
Edghill EL, Gloyn AL, Goriely A, Harries LW, Flanagan SE, Rankin J, Hattersley AT, Ellard S: Origin of de novo KCNJ11 mutations and risk of neonatal diabetes for subsequent siblings.
Mackay DJ, Temple IK, Shield JP, Robinson DO: Bisulphite sequencing of the transient neonatal diabetes mellitus DMR facilitates a novel diagnostic test but reveals no methylation anomalies in patients of unknown aetiology.
Hum Genet
Mackay DJ, Boonen SE, Clayton-Smith J, Goodship J, Hahnemann JM, Kant SG, Njolstad PR, Robin NH, Robinson DO, Siebert R, Shield JP, White HE, Temple IK: A maternal hypomethylation syndrome presenting as transient neonatal diabetes mellitus.
Temple IK, James RS, Crolla JA, Sitch FL, Jacobs PA, Howell WM, Betts P, Baum JD, Shield J: An imprinted gene(s) for diabetes?
Nat Genet
Flanagan SE, Edghill EL, Gloyn AL, Ellard S, Hattersley AT: Mutations in KCNJ11, which encodes Kir6.2, are a common cause of diabetes diagnosed in the first 6 months of life, with the phenotype determined by genotype.
Hattersley AT, Ashcroft FM: Activating mutations in kir6.2 and neonatal diabetes: new clinical syndromes, new scientific insights, and new therapy.
Pearson ER, Flechtner I, Njolstad PR, Malecki MT, Flanagan SE, Larkin B, Ashcroft FM, Klimes I, Codner E, Iotova V, Slingerland AS, Shield J, Robert JJ, Holst JJ, Clark PM, Ellard S, Sovik O, Polak M, Hattersley AT: Switching from insulin to oral sulfonylureas in patients with diabetes due to Kir6.2 mutations.
Girard CA, Shimomura K, Proks P, Absalom N, Castano L, Perez de Nanclares G, Ashcroft FM: Functional analysis of six Kir6.2 (KCNJ11) mutations causing neonatal diabetes.
Kondrashov AS: Direct estimates of human per nucleotide mutation rates at 20 loci causing mendelian disease.
Hum Mutat
Slingerland AS, Hattersley AT: Mutations in the Kir6.2 subunit of the KATP channel and permanent neonatal diabetes: new insights and new treatment.
Ann Med
Owen KR, Stride A, Ellard S, Hattersley AT: Etiological investigation of diabetes in young adults presenting with apparent type 2 diabetes.
Shields BM, Knight B, Shakespeare L, Babrah J, Powell RJ, Clark PM, Hattersley AT: Determinants of insulin concentrations in healthy 1-week-old babies in the community: applications of a bloodspot assay.
Early Hum Dev
Ma D, Shield JP, Dean W, Leclerc I, Knauf C, Burcelin RR, Rutter GA, Kelsey G: Impaired glucose homeostasis in transgenic mice expressing the human transient neonatal diabetes mellitus locus, TNDM.
Zung A, Glaser B, Nimri R, Zadik Z: Glibenclamide treatment in permanent neonatal diabetes mellitus due to an activating mutation in Kir6.2.
Tonini G, Bizzarri C, Bonfanti R, Vanelli M, Cerutti F, Faleschini E, Meschi F, Prisco F, Ciacco E, Cappa M, Torelli C, Cauvin V, Tumini S, Iafusco D, Barbetti F: Sulfonylurea treatment outweighs insulin therapy in short-term metabolic control of patients with permanent neonatal diabetes mellitus due to activating mutations of the KCNJ11 (KIR6.2) gene.
Cole TJ, Freeman JV, Preece MA: British 1990 growth reference centiles for weight, height, body mass index and head circumference fitted by maximum penalized likelihood.
Stat Med

redpajama_set_name: RedPajamaCommonCrawl


text:
Q: Battleheart 2: How do you beat Lady of the Deep? My party is level 29 already, but she just shreds them.
I play Mint, Cedric, November, Kaimani. Tried to weaken her with Rynn, but didn't succeed (is she immune to his effects?)

A: I used cleric, rynn, witch and fire mage. I was level 30. Had legendary weapon for dps. Dps had 4 passive talents each. Cleric and tank each had 3 passive talents. Dps has to focus fire adds fast. Keep her out of the big blue pool it heals her. Stay spread and drop adds fast

redpajama_set_name: RedPajamaStackExchange


Does anybody else in the U.K. have trouble finding stocks of time-delayed RCDs?
Many of the local suppliers in my area either do not carry them as regular items, or never seem to have any in stocks.
Given the modern trend toward split-load boards and considering that we're a predominantly rural area with TT systems by the thousand, it seems rather odd, as there must be quite a call for them.
What's also curious is that many of the national mail-order suppliers don't seem to carry them as standard stock either.
Are you talking about the 100-300mA Selective Type RCD that we use down here as a back-up to the standard 30mA, installed downstream?.
BTW these are not an RCBO, they require extra MCB protection.
Yep, they're the ones, also known as "Type S" here.
The typical split-bus board on a TN-S or TN-C-S supply has a 100A isolator switch which feeds the non-RCD side of the board to which MCBs are fitted for lights, water heater, etc. That bus also feeds a sub-main RCD (typically 80A, 30mA trip) and the bus from that carries the MCBs for all the other circuits.
The problem comes on a TT supply where all circuits need RCD protection due to the high loop impedance. We need to replace the plain 100A D.P. switch with a main RCD, but make it a 100mA delay type to achieve discrimination with the 30mA RCD downstream.
The alternative is to use a single bus board with one main RCD. And I think you already know my views on that method!
What would you think of an installation that used one fuse to protect say 6 final circuits? Why then do we seem to accept out of hand such a situation for RCDs? Single stand alone RCDs will still have their uses but with the availability and relatively low cost of RCBOs I can see no justification for one RCD protecting a number of circuits. Further, I would argue that it could be deemed a breach of BS7671 2001 (Regulation 314-01-01).
Time delayed RCDs were always difficult to pick off the shelf from my own wholesaler.However, he is now selling single-module RCBOs from CHINT for £6.30 each!
Lyle, did I read that correctly or have you missed a digit there, RCBO £6.30 what make are those? Thats cheaper than I am buying mcb's.
With an RCD protecting all of the circuits in a TT system, how do you get around the problem of appliances and other equipment, tripping the RCD, due to leakage currents to earth, caused by the equipment itself?.
The things that spring to mind are Refrigerators/Freezers, Water heaters (Storage type)and Bench-top ovens.
However, he is now selling single-module RCBOs from CHINT for £6.30 each!
I'll second Alan's question -- Never seen them that cheap before. And where or what is CHINT, or is that a typo?
With an RCD protecting all of the circuits in a TT system, how do you get around the problem of appliances and other equipment, tripping the RCD, due to leakage currents to earth, caused by the equipment itself?
You just have to hope that the total leakage is under 30mA!
Seriously though, on a small domestic installation you will find a main 30mA RCD feeding everything, and it does lead to tripping problems once you get a few heating elements starting to go leaky.
On larger commercial installations a solution is to break the system up into sections, each fed by an appropriate RCD. If there is a need for main RCD protection (e.g. bank of RCD's at the end of a sub-feeder) then it can be a time-delayed type of higher trip current (e.g. 300mA).
you have me doubting now! I will check next time I am in. Watch out for Chint! Chinese made but with all necessary standards.Stack em high and sell em cheap philosophy!
Lyle, thanks will watch for Chint with interest. Just as an asside have you used any of these, and if so what do the RCD tests come out like? my experience is that on cheapy gear they test very close to the top end of the range ie 37,38,39 m.secs then after a few years service with no testing they then fail to test within the recomended times, so you finish up having to change them. I would be interested to hear from you on that one.
sorry, i have no experience. I do take your point but I have to acknowledge that many products are made in the far east and are as good if not better than the home grown variety. Commercialism and globalisation are bed-fellows that might be the demise of local industry. Unless perhaps they follow companies like Dyson and simply pack up and go! I wouldnt be so quick to denounce some far east electrical products, especially where they are legitimately meeting international, european and british standards. Afterall, as sparks we are entitled to take advantage of commercial opportunities just like the next guy!


text:
SYSTEM_TESTS_FOLDER=system-tests
rm -rf $SYSTEM_TESTS_FOLDER || echo "folder does not exist"
git clone https://github.com/cloudify-cosmo/cloudify-js.git $SYSTEM_TESTS_FOLDER
cd $SYSTEM_TESTS_FOLDER

if [ "$TEST_BRANCH" = "" ]; then
    TEST_BRANCH="master"
fi
echo "TEST_BRANCH is $TEST_BRANCH"
git checkout $TEST_BRANCH

sudo npm cache clean
npm install

sudo npm install -g bower
bower install --config.interactive=false

export TEST_BROWSER="PhantomJS"

echo "browser type is $TEST_BROWSER"
echo "uploading blueprints and creating deployments for cloudify-js tests"
echo "virtual env folder is: [$SYSTEM_TESTS_VIRTUAL_ENV]"
source $SYSTEM_TESTS_VIRTUAL_ENV/bin/activate
cfy blueprints publish-archive -l test/resources/testHelloWorld.tar.gz -b HelloWorld -n blueprint.yaml
cfy blueprints publish-archive -l test/resources/cloudify-nodecellar-example.tar.gz -b nodecellar -n openstack-blueprint.yaml
cfy blueprints publish-archive -l https://github.com/cloudify-cosmo/cloudify-nodecellar-example/archive/3.4m5.zip -b nodecellar_updated -n local-blueprint.yaml
cfy deployments create -b HelloWorld -d HelloWorld
cfy deployments create -b nodecellar -d nodecellar --inputs test/resources/cloudify-nodecellar-example-inputs.yaml
cfy deployments create -b nodecellar_updated -d nodecellar_updated

if [ "$TEST_TYPE" = "" ];then
    TEST_TYPE="test mochaTest"
fi
echo "TEST_TYPE is $TEST_TYPE"

export REPORTS_BASE=`echo ~`/reports

grunt # first build the dist files
grunt $TEST_TYPE

redpajama_set_name: RedPajamaGithub


It's time for me to update my MSR AC Bivy review. I've spent quite a few nights in this yellow cocoon and have formed some new opinions on it.
UPDATE | After having two Sporks develop stress fractures and snap in the middle, I'm hesitant to recommend Light my Fire's spoon/fork combo. They're fairly durable but don't expect them to last more than a few months of continuous use.


Listen to the story of van Berg and his adventure!
In this five-part intervention created by the duo Serotonin, the artists have developed five acoustic dioramas located in three exhibition rooms to comment subtly and productively on the history of research and collecting at the Museum für Naturkunde. The acoustic dioramas consist of five small presentation spaces that have been designed and constructed by the artists themselves – white cubes set up in peep-boxes belonging to the museum's collection.
These visual, audio, and conceptual exhibits correspond to the five chapters of a bizarre expeditionary tale, written and produced by Serotonin. The year is 1884. The recently established German nation is struggling to gain recognition and a prominent place on a map of the world. The setting is the Tiergarten park in Berlin, where the crown prince grants a highly unofficial audience, and a cave in the future colony of Deutsch-Südwestafrika. Inspired by the levitation of a floating virgin presented in a variety show at the city's Wintergarten venue, the young van Berg initially dreams of "an imperial fleet in the ocean of heaven", but ultimately, thanks to a chain of coincidences to do with complex political interests of the time, becomes the leader of an expedition to Afrika.
Serotonin has turned the story of this research expedition into a humorous show compressing cultural history from a critical perspective. The five exhibits contain both sculptural and narrative elements, broaching complex topics such as crossing boundaries, defining oneself through the other, and the origins of national identity and a sense of Heimat.
Marie-Luise Goerke and Matthias Pusch are Serotonin. The intervention is curated by Gaby Hartel. Insights into the creative process can be found here!
On 3 September 1884 van Berg finally sits with Prince Wilhelm on a bench in the Tiergarten park.


text:
Varijabilni (promjenjivi) troškovi mijenjaju se u ukupnom iznosu razmjerno s promjenom opsega proizvodnje.

U hotelijerstvu je jedan od glavnih predstavnika varijabilnih troškova trošak materijala (namirnica i pića).

U hotelijerstvu vanjski troškovi iznose 30% do 40% ukupnih troškova.

S obzirom na brzinu povećanja u odnosu na stupanj iskorištenosti kapaciteta:

Proporcionalno varijabilni - povećavaju se ili smanjuju proporcionalno (u istom razmjeru s povećanjem ili smanjenje iskorištenosti kapaciteta).

Progresivno varijabilni - povećavaju se brže nego što se povećava stupanj iskorištenosti kapaciteta (često povećanje iskorištenosti kapaciteta rezultira i većim škartom ili gubitcima u proizvodnji).

Degresivno varijabilni - povećavaju se sporije nego što se povećava stupanj iskorištenosti kapaciteta.

Računovodstvo

redpajama_set_name: RedPajamaWikipedia


The new Alexa Skill for the German Central Library for the Blind (Deutsche Zentralbücherei für Blinde – DZB) is now available. You may activate it here.
Alexa Skills let you interact with smart speakers and smartphones featuring the Alexa voice assistant by talking to them. These voice-controlled devices are especially useful to people who are blind or partially sighted. We think the new Alexa Skill will make it even easier to access books and audio-described films available through the DZB.
What are the features of the DZB Alexa Skill?
What do you need to use the DZB Skill?
In addition to your Alexa-enabled device, you will need your DZB login information also used in the DZB App for iPhone or Android. All DZB readers may request login information. To do so, you may Email us at hoerbuecherei@dzb.de or call us at +49 341 7113116. Once you have borrowed your books or films from the library, you can listen to them. You may borrow books via the iPhone or Android app, via the DZB website or by calling us.


Kirrawee Kangaroos is based in the southern area of Sydney, within the Sutherland Shire.
Teams play within the Sutherland Shire Football Association competition.
Our grounds are located at Kareela Oval, Princes Highway, Kirrawee.
The clubhouse is located at the south east corner of the field.
We host teams from Under 6 right through to Over 45's, including womens teams. Games are played on Saturday and Sunday.
Kirrawee Kangaroos Football Club is a community club which relies on support and help from players, parents, friends and other volunteers. Without your support the Club can not function and we can not get the games on the filed each week end.
The Club committee meets on average once a month to discuss issues, plan events, make decisions and generally perform tasks necessary in the running of the task. The more people we have on the committee, the less work each person has to do. If you would like to play a role in the running of the club, please talk to a committee member or send an email to info@kirraweekangaroos.com.au or to president@kirraweekangaroos.com.au.
Even if you are not able to join the committee, we will always welcome any help you can offer. Through out the seson we need people to help with ground set up and packing up, in the canteen, on the BBQ, with ground control, fund raising, social events etc. Even a little time will be greatly appreciated.
If you can help in any way please talk to a committee member or send an email to info@kirraweekangaroos.com.au.
The running of a football club is a expensive. To help in reducing the costs to the player and/or parents, we are seeking sponsorship.
If you or your business can help us in any way with money, goods or services, please contact the club President (president@kirraweekangaroos.com.au) to discuss.


By re-electing Adam Bandt and Ellen Sandell in Melbourne, and by electing Kathleen Maltzahn to turn the seat of Richmond for the first time ever, we could secure the balance of power in both the Victorian and Commonwealth parliaments. So we're bringing all three electorates into one huge campaign.
That's why we'll be doorknocking across inner Melbourne to talk to our community about issues that matter to them.
You don't need to be an expert to have these meaningful conversations, all you need to know is why you care.


Vega Light Control Systems' LED Shoebox Retrofit offers customers a high performance retrofit for a wide variety of shoebox style fixtures. This system will replace up to a 750w HID light source while producing excellent photometrics and up to 75% energy and labor savings. Plus, this system is available with customized mounting hardware for quick and easy installation without performance or system life compromise. This engineered system utilizes best in class name brand components, 36 kV of surge suppression standard, and a high temperature silicone optic for long term performance and color stability. Available in 80w,120w, 150w and 250w LED watt sizes, this system will replace all common HID light sources up to 750w HID. Specifically designed and tested in common shoebox fixtures, this system is rated for 100,000 hours life in the most demanding applications.


text:
|THE NATURAL STORYTELLER|
|DIGITAL CONTENT
MARKETING|
|COPYWRITING|
|EDITING|
|REPORTING|
|THE NATURAL SCOOP|
|AUTHOR – 50 SIMPLE STEPS|
|CONTACT|
|805 279 4342|
Purchase your copy now!
Kim Henderson is the author of 50 Simple Steps to Save the World's Rainforests (Freedom Press, 2012), recipient of the 2012 Green Book Festival, "Best How-to" award, Runner Up in the "Best How-to" category of the 2013 Paris Book Festival and Finalist in the Science/Nature/Environment category of the 2013 Next Generation Indie Book Awards. Rainforest activists "Amazon John" Easterling and Olivia Newton-John Easterling contribute the book's foreword.
About the Book: While many of us know the sobering facts about tropical rainforests, few know what we can do to help. That's where 50 Simple Steps to Save the World's Rainforests comes in. With demand fueling the most destructive forces driving deforestation—agriculture, logging, and resources extraction, the future of the world's rainforests lies to a large extent in our collective "consumer" hands in how we choose to shop and live.
The book's 50-step journey is designed to both illuminate where your consumer and life choices may unwittingly be contributing to rainforest destruction and more importantly, precisely how you can make different choices that strengthen your relationship to our natural world and help preserve our rainforests.
For example, you will learn how your paper use and purchases of rayon clothing affect Indonesian rainforests where Sumatran tigers are critically endangered or how simply eating Brazil nuts helps save the Amazon rainforest.
You will discover how the cultivation of palm oil—a common ingredient in confections, baked goods, biofuels, personal care products and cleaning products—is fueling rampant deforestation in Malaysia, Indonesia and Papua New Guinea, and how you can avoid it. You will also learn about how closely connected you are to the rainforest in your daily living.
50 Simple Steps to Save the Rainforests is the ultimate primer for anyone wanting to do something to preserve what remains of this precious ecosystem for future generations.
"50 Simple Steps to Save the World's Rainforests gives practical ways to stem the disaster of rainforest destruction. Read it and you'll learn a lot, follow its advice and you'll make a difference. I highly recommend this book to all who care about planet Earth."
Chris Kilham, "Medicine Hunter"
"It's real simple: This is a book that should be owned by every person from every country in the world. It's that useful, and that important."
William Gazecki, Academy Award-nominated
Documentary Filmmaker
"I'm grateful for a book that gives people greater insight about the ultimate importance of the rainforest to humanity and simple ways to make a difference with our everyday choices."
David Karr, Co-founder, Guayaki
The Back Story
In 2008, Kim Henderson's job as managing editor of Healthy Living magazine took her to Costa Rica. The magazine was developing a cover feature about the efforts being made by Whole Foods, nutritional supplement manufacturer New Chapter and other independent health food stores in partnership with the Costa Rica's Monteverde Conservation League to raise money for a wildlife corridor extending the Children's Eternal Rain Forest along the Chachagua River. The corridor would allow predators such as puma and jaguar a larger area to roam.
The morning after the group arrived at Finca Luna Nueva, the sustainable farm owned by New Chapter, which borders the rainforest, they discovered a jaguar paw print on the property—a rare occurrence and an auspicious sign for the story the magazine aimed to tell. After traipsing the property and the forests' border, the crew conducted the photo shoot in the Chachagua River!
Kim in Costa Rica with Tom Newmark, CEO of New Chapter, Steven Farrell, general manager of Finca Luna Nueva and environmentalist and owner of Freedom Press, David Steinman.
Kim was profoundly affected by the vibrant energy and allure of the rainforest. On the return flight home, she suggested to the magazine's editor and owner of Freedom Press, that the next environmental book the company published needed to be about the rainforests. Freedom Press had just published 50 Steps to Save the World from Global Warming with the Green Patriot Working Group*. Kim knew that Westerners like herself were far removed from tropical rainforests around the world and that this book would need to connect the two worlds and provide simple, easy steps that would make a difference in conservation efforts. Her publisher loved the idea. Six months later she was asked to lead the project along with the Green Patriot Working Group. 50 Simple Steps to Save the World's Rainforests was published in 2012 and is widely available at fine booksellers and at Amazon.com.
*The Green Patriot Working Group consists of concerned individuals and organizations who realize that underlying many of our nation's most pressing challenges is an environmental crisis that demands a balanced and nonpartisan approach in order to preserve our future.
Here are some of Kim's favorite rainforest friendly products and conservation organizations. Each one is making a positive difference in the world. Please enjoy exploring what they offer!
Earth Friendly Products — No palm oil!
Coffee/Chocolate
Newman's Own Organics
Eco-friendly Wood/Furniture
TerraMai
EcoTimber
RainTees —Each purchase plants a rainforest tree!
Jewelry (recycled gold)
EcoPaper.com
Living Tree Paper Co.
The Amazon Herb Company
Amazon Watch
Chevrontoxico
The Amazon Center for Education and Environmental Research (ACEER)
Forest Stewardship Council
Mongabay.com
Monteverde Conservation League
No Dirty Cold
Rainforest Relief
Sumatran Tiger Trust
Survival International


Durable and highly capable mobile TV floor stand with one TV bracket for large flat screen TVs, two monitor arm with extendable VESA mounts and four heavy duty twin casters and hard steel frame.
Unit height is 72 inch from the floor to the top of the mounting pole.
VESA compliant monitor mounts provided. The smaller mounts extend a full 20 inch and support flat screens weighing up to 25 lbs. The larger mount can handle a screen up to 60 inch and150 lbs.
30.5 inch W x 29 inch D footprint is angled for increased stability.


text:
Q: Libgdx Game - Texture Scaling I am using LibGdx to develop a simple game for Android devices. I wish to show a progress bar using the Texture  by scaling it up / down. When we do scaling the image is changing in its shape. Could you please let me know a best approach to achieve the below mentioned scenario.

Scale up is fine..

Scale down is really a problem.

A: If you want to do it purely by scaling, you can do one of the following

*

*Use a mask on the green bit (use for example the blue part as a mask)

*Chop the green part up in three parts: two for the round end caps and a rectangular middle piece you can scale. The problem with this is that the smalles progress you can display will be one endcap.


text:
REPORT: Chrysler, Fiat partnering on electric car
Jonathon Ramsey
Oct 12th 2009 at 5:58PM
Dodge Circuit EV – Click above for high-res image gallery
In a little more than three weeks, Sergio Marchionne will reveal his 5-year plan for Chrysler. According to information revealed by a Fiat exec and Chrysler board member Alfredo Altavilla last week, at least one electric car will be part of it. While visiting a gearbox factory in Italy, Altavilla confirmed that the presumed-dead electric car program is in progress and being worked on by Chrysler and Fiat. He gave no further comment on the matter.
Chrysler, through its ENVI in-house electric propulsion division, currently has four electric vehicles: the Dodge Circuit EV (shown), Jeep Patriot EV, Jeep Wrangler Unlimited EV, and Chrysler Town & Country EV. The ENVI site claims that you'll be able to own at least one electric model by 2010, and there will be "at least three more models available by 2013." Those claims could be outdated, however; we'll have to wait until November 4 to get clarity on the future. But if there's an electric Fiat 500 in there, well, let's just say we won't be disappointed...
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[Source: Wall Street Journal]
ElectricCar
ElectricVehicle
dodge circuit ev
DodgeCircuitEv
Japanese car-sharing service members are renting cars — but not for driving
VW Type 20 Concept is an organic, retro EV
Watch the VW ID.R break a 20-year-old record at Goodwood


UFC fighter Conor McGregor has just launched his own Irish whiskey called Proper No. Twelve, named for the district he grew up in Ireland.
McGregor turned to master distiller David Elder (formerly of a small, not-famous beer company called Guinness), and together they created close to 100 blends at the world's oldest whiskey distillery, ultimately selecting one that met the athlete's high standards.
The result is a mixture of very fine golden grain and single malt, produced in an area renowned for its rich soil and pure spring water, triple-distilled and aged in oak barrels. The taste: complex yet approachable so, with notes of vanilla, honey and toasted wood.


In the blogging business, do you think that passion should make your business decisions? Probably not. But should it be the motivating force behind your work? No harm there.
I have a confession to make. I have a passion. That passion has been alive for 5 years. During that time, I've read, researched and learned a plethora of information. I've hungered for more information and found myself starved. I desired to give people what I had been craving. More information.
hair. I spent April designing a website and compiling all of this information into an organized manner to give other naturals a whisper of the information we yearn for. This whisper will transform into a shout because my passion still burns and will grow.
I'm not going to go into great detail about why I decided to write about natural hair. I'm not going to tell you my personal story with natural hair because I doubt that you care. What I want you to know is that this passion of mine has taken hold of my time and energy. Since I've always been one to keep my writing business hours at a part time level, that means letting some things go.
My guest blogging here is one of those things. Jael Strong too will cease guest blogging and focus on pursuing creative writing, her passion.
But, it might make think about evaluating your circumstances, including your work schedule and productivity. Are you working your passion? If you are, are you spending enough time to develop it? Are you spending too much time on it? Are you being reasonable?
be honest with yourself. There's no sense in lying to yourself.
As for me, if you're interested in the natural hair scene, you will see me around.
If not, know that I'm very grateful to have been here. Thank you, Liz, for the opportunity to know you and learn from your great wisdom. Thank you, readers, for checking in, commenting, tweeting and driving me to success.
And yes, I do think I'm successful, even though I've just recently started this endeavor.
Thanks, Terez, for sharing yourself and your insights on my blog!
Should You Dish What You Take?
One of the editors that I write for usually doesn't get back with me about my articles until right before publication. That means that I have to scramble to make any changes. She hardly ever responds to my first e-mail, and oftentimes doesn't "get" the e-mails I send. I've had so many issues with this woman, from getting no check at all to getting double on my check, that I've contemplated ceasing my business relationship with her.
No matter how angry and wronged I feel, I tell myself that I have to maintain a professional demeanor.
Do you pay in kind? Or, do you turn the other cheek?
In the blogging business world, you might feel that paying in kind is a necessity. With your blog, you have a means to communicate your unbridled ideas and opinions with the world. You might feel that it's your duty to be brutally honest.
Personally, I agree that a blog should keep it real.
On the other hand, perhaps you are more of a turn the other cheek person. It's not that you aren't being true to yourself. Being yourself just means avoiding confrontation. You actually just might not care about an issue one way or the other. You might prefer to ban ranting from your blog.
That's OK, too. Once again, be real.
What about a personal level?
With a blog, you don't usually get more personal than comments and e-mails. What are people saying about what you write? What are people saying about you?
The in between approach is best. When you deal with a confrontational reader, you have to always be tactful. Just because a person is rude to you, does not mean that you have to be the exact same way. Say how you feel as professionally as possible.
Be careful when you choose to ignore a person. That individual, while agitated, might be expecting a response from you. If that is the case, do so in the same tactful spirit I mentioned before. If you still just don't want to deal with it, you can still let a person know that you are not going to respond to that type of negativity and leave it at that.
I'm still not sure what to do about writing for this editor. I try to be a very organized woman, and when that organization is dramatically disrupted time and time again, I have a difficult time rolling with the punches. I probably will not dish what she serves. I probably will not try to approach her again about these issues. (Yes, I've already made some attempts to smooth things out).
What would you do? Do you dish what people serve?
Life Under A Rock Promotes Productivity?
Do you ever find yourself furiously typing at your computer, scripting blog posts, guest blog posts, website pages and the like, and you haven't been attuned to what's going on in the world? When I say that, I don't mean to say that you don't keep tabs on major current events. Instead, you find yourself so immersed in your work that you haven't taken the time (or had the time, for that matter) to stay abreast on the trends in your niche.
That's how I feel right now.
That's what I said. I don't mind being under a rock. It's quiet. I get a whole lot accomplished. I don't have to really deal with anyone because I'm working on my own personal projects. It's really not that bad.
Increased productivity. I don't just sit in front of a computer screen and randomly surf the Internet. I have a job to do that is close to my heart, and I've shut off nearly everything else to achieve it.
Super strong determination. I WILL succeed. I have decided that I am not allowing any outside influences to thwart my goals.
Sharpened skills. I have been writing and writing and researching and writing. I feel that my art has improved.
In a way, I prefer life under a rock.
I do enjoy people. I'm no introvert. But there's something to be said when all you have to contend with is writer's block, research options and sentence structure. No bad attitudes. No annoying behaviors. Nothing to deal with that I cannot control.
That said, I feel like an outsider to the blogging world.
Even though life under a rock can be a productive experience, it can leave you missing some things. For instance, I haven't been able to regularly keep up with any other bloggers. Sure, I catch a post here and there. But I miss reading their regular work. I haven't even gotten to stay up-to-date with my favorite bloggers, the people whose information I admire and treasure.
Also, and most shocking to me, I have had to turn down work. (This statement is shocking to me because not so long ago, I didn't imagine I would be one of those freelancers that had a booked schedule).
I only have so many hours that I have allotted to my writing work, and I will not exceed my limit. I Don't Want to Be Rich. I want to do what I love, but I want to have time for the people I love. I have chosen to trade time with my family for time that I could have used to make money.
I want to reasonably spend most of my work time under my rock with my personal writing projects and at least some time out of the rock, connecting with the bloggers and other professionals I've met. I suppose the best solution is a schedule. I've used my schedule to plan out my time under the rock. Now, I need to schedule time out of the rock.
It can be an overwhelming task – reading blogs, commenting, tweeting, etc. It all takes time. How do you allot time to stay out of life under a rock?
Are You A Cookie Cutter Writer Or Something Else?
I am a writer. If you stood over me while I type at my computer, you would wonder how anything discernable is going to come from what you see. I don't normally write from sentence to sentence and then follow up by writing one paragraph after another in sequential order. My mind processes information in a somewhat scattered manner.
The mess going on in mind has to be sorted, processed and regurgitated in an intelligible way. That means, I think of a thought and type it. That sentence, paragraph or even single word might get moved all around my blog post before it finds a home.
I start a sentence, and sometimes I finish it. (This sentence happened to be the second sentence of this post, and I think it's going to stay here now). I like to attribute this writing style to my journalism background. When you write for the newspaper on deadline and you're sorting through notes and remembering key ideas in all sorts of orders, you realize that you better get what you know on paper fast. Organize later.
I've maintained that style when I blog.
This was new to me. I recently purchased an e-book that actually was about building AdSense websites, and the author spoke about using a template to write articles. I appreciated these simple template ideas, like Q and A's, Myths vs. Facts and 10 (or whatever number) Reasons For Something. I said to myself, I'm going to do this. It will make writing much easier.
About two or three articles into it, I totally forgot about the templates and went back to my old ways. I'm not cut out for templates. (Ha, pun).
For people who aren't much for writing or who have a difficult time coming up with ideas, I find templates to be a fabulous spring board. You fill in the blanks and voila. You have a well-thought-out, organized, helpful blog post.
Tutorial. A step-by-step guide of what to do.
The interview. You ask questions, and someone important answers.
Pros and cons. You say what is good and what is bad about a certain issue. You take a side, or you don't.
Review. You review a product, service or other website in your niche. You review your own blog.
A blend. Blend some of the template ideas I listed here. For instance, write a review based on a pros and cons list.
Writing against a template is not for me. I feel like my freedom of expression is inhibited in a cookie cutter post. I can definitely see how a template would be beneficial to people who do not like to write or struggle with what to write about. But for those of us that just want to get our thoughts on the page, we don't do it.
I guess it's more of a matter of order. I put writing first and organization second. A template writer puts organization first and writing second.
One is not better than the other. Templates are for some, and free writing is for others. Which do you prefer?
In classic Western shows, cowboys are white men. But coyboys were also of African American, Mexican and Native American decent. And we all know that the Indians that go with cowboys are not from India.
I don't really know. I believe that someone or group of people somewhere decide that this is going to be the norm. Cowboys will be white, and Indians will be Native Americans. They will never get along, and it will be a fun game for kids to play. Then, that norm transforms into a stereotype. Before long, the actual truth of a matter is lost. A stereotype becomes truth.
This is why when most people speak of a stereotype, it engenders thoughts of something bad.
What is the stereotypical blogger?
I have been thinking about this for a while. I suppose the stereotypical blogger knows everything there is to know in her niche, at least everything thinks she does. She posts frequently, well, regularly. She is witty, conversational and informative, all wrapped up in a delicious but very real blog. Readers hang on her every word. She posts pictures and video, too, because she wants her blog to have it all. She knows everyone, and everyone knows her.
The stereotypical blogger doesn't sound bad at all. In fact, it sounds like the type of person that every serious blogger wants to become. But, why? Because those characteristics equate success? Is it because that is what all of the top, authority bloggers are doing? Just because they're doing it, you have to do it to. They're successful, and there's no other way for you to be a successful blogger. Is that so?
Yep. I'm not that kind of blogger. I'm not saying that I don't want to be. I wish that I could post every single day and that I understood the ins and outs of html. I don't, and I can't. I am not ashamed.
I have made another observation about the stereotypical blogger. She has very limited time for the rest of her life. She's always answering e-mails. She's constantly tweeting her stuff and everyone else's. She always seems to be on every blog related to her niche, commenting and guest blogging. She is everywhere, and everyone loves it.
If you're not going to be the stereotypical blogger, then you know by now that you are letting some things go. Your blog might not have everything, every tool and form of media, right away. I believe that if you want to have a life outside of your blog and you're patient, you can eventually have everything on your blog.
Also, your name will not get to be all over the Internet immediately when you aren't the stereotypical blogger. You will not have the time. Slowly, you can make connections that will boost your image. You may never be No. 1 in your niche. Is that OK?
It's OK with me. No. 1 has too much responsibility. I'm content doing what I can do, having fun writing, collecting my money and then having a life.
What do you think? Are you or do you want to be the stereotypical blogger?
What Is Your Title, And Why Do I Care?
I have a secret to share with you. After years of studying online writing, I decided to become a "professional blogger." And you know what? I made that title up.
I was in an interview once, and I was asked what it took to be a professional blogger. I was thinking, I made that title up to tell people who I am without having to explain who I am. Now you want me to explain it?
When you introduce yourself to someone, you say, "Hi. My name is (insert name)." The stranger always gets around to asking, "So, what do you do?" That's your opportunity to give your title. If you're like my husband, you say that you are a teacher and a professional violinist. If you're my niece, you say that you are a dental hygienist. But, when you're a freelancing jack-of-all-trades, what do you call yourself?
Your title is part of your brand. It automatically gives people a mental image of what you do. A blogger blogs, and a marketing specialist markets. It is a snapshot of your work.
Your title can open doors. When you tell someone what you do, it can be an opportunity for a super short sales pitch. For instance, my husband and I took a visit to an insurance agent who asked me what I did. When I explained my work, he said that he wished he knew me a few months ago because he was looking for a writer. I am confident that he will remember me if he needs a writer in the future.
Your title makes you feel like you have a real job. When you blog online, some tend to get the idea you are practicing some sort of hobby to pass time. They don't realize you are building a business and get paid in dollars and cents. Some of my friends still seem to not understand that I actually work from home. I have a job, and here is my title. Even if they never get it, I can feel like I am a member of the workforce. It's a boost.
Yes. You get to choose what to be called if you work from home as a freelancer. It is an easy, simple way to build your brand. Pick the right title, the one that tells people exactly what you do.
Be as specific as possible. If you blog about travel, then call yourself a freelance travel blogger. If you write press releases, then you are a press release writer. If you want to blog about and review screenplays, call yourself a screenplay blogger. That's right. Even if you don't have all the references, testimonials and samples to prove it, choose the title that will best describe who you want to be.
If you need to make a change in the process, do so. If you see that your type of blogging is evolving and that you are beginning to become someone other than the person that your title embodies, gently transition to your new title.
If you just happen to do more than your title says, don't stress over it. Yes, I call myself a professional blogger. But I also write for a magazine, edit, build websites and create online content. I still call myself a professional blogger. However, if I find that the scale tips more toward another niche, I'm not afraid to make a change.
In the end, you are who you say you are. By the way, who are you, and why should I care?


The way they always smell good even if it's just shampoo.
The way they always find the right spot on our chest to lay their head.
The ease in which they fit into our arms with their tender frames.
The way they kiss you and make your worries disappear at least for that moment.
How cute they are when they smile heartily.
The way they take hours to get dressed but in the end its all worth while.
The way their bodies are always warm even if the world is freezing.
The way they look good when they dress simple.
How cute they look when they are angry.
How their hand always seems to find ours.
When you see her name on the caller ID after you've had a big fight.
The way she says "lets not fight anymore" and you see tenderness and sincerity in her eyes.
The way they kiss you when you've done something nice for her.
The way they fall into your arms when they cry.Then apologizing for crying over something that silly.
The way they say "I missed you"
The way their tears make you want to change the world so that it doesn't hurt her anymore.
When you stare into their eyes traveling to the depths of their souls, you see a million things and hear a million thoughts.We love them for a million reasons, no paper would do it justice. That's just the way it is.


Many specifiers base some of their requirements on the slight degradation in appearance of the parts tested, measured in terms of contrast.
The evaluation of the contrast is detailed and defined by international standard ISO 105 A02.
It involves comparing the degradation of the test piece using a grey scale, in a light booth in which the only illumination is standardised (type D65 Daylight).
This type of booth can also be used to inspect parts in other types of light, e.g. to check fluorescence phenomena.
This measurement is made by qualified technicians whose results are regularly compared with those obtained by the leading manufacturers.


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Q: PhpStorm doesn't show background color in .sass or .less I am really interested in showing background color in sass or less. When I put variable there it shows a background color, but when I set color to the element, the background color isn't shown.

A: At the moment such functionality is not available if you choose to display it as a background color -- it's available for actual colors only and not for variables.
https://youtrack.jetbrains.com/issue/WEB-14338 -- watch this ticket (star/vote/comment) to get notified on any progress.
Consider also watching related tickets -- WEB-6015 and other related to the last one.


This song is from the album Roses are Red, Violets are Blue.
Nico: they say it's easy to write about it if you've lived it. I guess that's true. This tune has two things which save it from being a complete YAID (Yet Another Indie Dirge): Wendy's awesome string arrangement, and, to use the words of mastering engineer Mark Donahue: "the loudest twenty seconds I've mastered this year."


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NeverDead
NeverDead - Co-Op Review
Review 2/16/2012 at 2:04 PM by Paul Acevedo
You'll lose your head over this game, DeCapAttack-style.
Every game needs a hook, and when I learned of NeverDead's, I just had to give it a try. Protagonist Bryce Boltzmann is a demon hunter who has been cursed with immortality for the last 500 years. As a result, things that would signal a Game Over in other games are barely a roadblock to Bryce's progress. The dude can literally tear either of his arms off and throw it at enemies, subsequently signaling it to fire a gun or just plain explode like a grenade. It's okay – he'll just grow a new one. Bryce also yanks his head off and tosses it through basketball hoops, which doesn't hurt enemies but can solve a simple puzzle or two. Yes, this game is a bit nuts.
NeverDead basically plays like a Japanese action game (Bayonetta, etc.) mixed with a dark, British sense of humor. Bryce has two main forms of attack: his sword and his guns. Players swing the sword using the right analog stick. This allows for a fair degree of freedom in the sword's arc, but it lacks a robust combo system typical of the genre. The sword is generally the most effective method of dealing damage, especially since some demons are immune to bullets. Still, at any time our hero can switch to his dual-wielded guns in order to take on flying threats or explosive enemies. Guns initially lack stopping power, only becoming really effective late in the game. We'll get to that momentarily.
Bryce may be an immortal, but he's a lot more fragile than you might think. Just about any contact from an enemy causes him to literally fall apart. 99% of blows will sever one or more appendages. When his happens, you'll need to run or hop over to the fallen limb and then roll to reattach it. You can also regrow any missing parts once the timed regeneration meter fills up or after grabbing a certain item. The limb loss mechanic may have sounded good on paper, but in practice it's mostly annoying. Having to stop, drop, and roll every time you take a glancing blow plays hell with the pace of the combat. You get used to it after a while, but I fear many players will get discouraged early on, before they really understand the combat system.
Even more troublesome, severe blows knock Bryce's head right off. While sometimes it's necessary to self-decapitate in order to move through tight places (the hero's head can roll around and jump like a Metroid morph ball), Bryce's head form is extremely vulnerable during combat. Regular enemies pay it no mind, but tiny demons called Granbabies exist solely to chase after your limbs and eat them. If they swallow Bryce's head, a short timing based minigame commences. Pass and you're spit out, free to roll back to your torso. Fail (which will surely happen now and then) and it's Game Over, as Bryce supposedly spends eternity trapped in the Granbaby's stomach. Granbabies tend to respawn infinitely while other enemies are around, and killing them doesn't even provide XP for some reason, so you're bound to hate them with a passion.
So the protagonist's tendency to fall apart detracts from the game more than it adds, and yet I still had a blast slicing and shooting hellspawn to kingdom come. That's mostly due to the excellent upgrade system. At any time after the first mission, you can spend XP gained from killing enemies on a variety of abilities for Bryce. More abilities unlock for purchase as the game's 8 lengthy story missions are cleared. These abilities include enhancements for Bryce's sword, guns, running and jumping, XP modifiers, and lots more. You have a limited number of ability slots, so choosing the right ones for your play-style and the situation at hand is paramount.
NeverDead Launch Trailer Released
NeverDead DLC Coming Later This Month
New Screens and Videos for NeverDead
E3 2011 Hands On Preview NeverDead
Konami Releases New NeverDead Screenshots
NeverDead Release Date Announced


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An overlooked hunting destination
Hunting Stories
Hunting in France - a wonderful hunt in beautiful hilly terrain
Excellent Hunting for Small Game, Roebucks, Red Stag and Chamois
Charming French Countryside in Hilly Terrain
Old Hunting Traditions
Excellent Cousine
Hunting in France
Hunting has been an important part of French history and culture for many thousand years, cave paintings showing hunting scenes found in France can be dated to far back into prehistoric times. Hunting was the most important leisure activity for the French kings and aristocracy, many of whom built grandiose hunting lodges in their country estates. French literature and culture also has a rich store of hunting stories and legends.
Today there are 1,350,000 registered hunters in France, who mainly enjoy rough shooting and wild boar hunts. In 2007 the country set a quota of 600,000 roe deer, 13,500 alpine chamois, 3,100 Pyrenean chamois. 3,200 mouflon and 500,000 wild boar. The quota in general is increasing for the different species, largely due to improving game habitats, and the selective shooting policies.
Even though France is one of the world's most important tourist destinations, practically no hunting has been available to visiting foreign hunters. We are therefore very happy to now be able to offer hunting at a number of different locations in this beautiful country, which also can offer a wealth of other attractions to visitors, which makes it an ideal destination to bring the rest of the family to. Here it is possible to combine high quality hunting with French culture, cuisine, wine and the country's wonderful countryside.
 Video Gallery
Buck Hunt in France with Erik Hahn Pedersen
Sud Chasse - Partner
James on Buck Hunting in France
Who says France is just for lovers? It is for hunters, too!
One of the benefits of hunting in Europe is the exposure to history. I got my first taste of Continental hunting served up with a dash of history when I was stationed in Germany with the US Army in the early 1970s. I found that I enjoyed it and have been tramping around the Continent ever since. The best times to stalk roebuck are in the Spring or early Summer ( April through early June) and again at the time of the rut, late July and early August.
My most recent excursions have been to France. The first time in October and on the second trip, June. Before my arrival, all I knew about French hunting was the long history of "parforce" mounted pursuit of stags and the French love for wild boar hunting. This was Gaul after all, the country of Axterix and Obelix! I answered an ad from Diana Hunting Tours a couple of years ago which lead to a three day roebuck stalking session near Auch followed by a few days in the Mid Pyrennes stalking the red stag. Two years later, I returned for roebucks only, with the private hope of bagging a boar.
I got more history than I could digest and some of the most memorable hunting experiences of my life in France.Southen France, in particular, with the Pyrenees often in view. Mix the tribe of the Vascones, the regions of Bearn, Basque Country, Aquitaine, before and after the Roman conquest, the Kingdom of Navarre, and Gascony all together then throw in a couple of famous kings born in Pau, the Good King Henry IV of France and King Karl Johan of Sweden, born Jean Bernadotte,
and you get a historical "soup" that is almost too thick to spoon. For the topping,add the saga of the Mann family escaping from the Nazis by climbing over the Pyrenees and you have a soup for all seasons.
On my first trip, I landed at Toulouse, the home of the Airbus, and hunted the roebuck on the rolling fields and creeks near Auch. It was after the grain harvest and not a "classic" time for roebuck stalking, but the game was plentiful and the hunt was successful. On the second trip, I landed in Pau. The two kings hometown. My guide picked me up the next morning for the short trip to Poudenx and to my hotel. Again, I found great French hospitality, clean comfortable quarters and great food.
Off for an evening stalk with Frederic. Short stop for a test shot. It was about 2"hi, but I thought for shooting from over the hood of the Suzuki, the rifle was on. Then, when the time came, I missed the first buck. Too high. Back to the range. This time I shot twice. Tight group, but 3" high. I clicked the scope down a couple of clicks and was on the bull's eye. I still do not know the science behind the difference in impact from at home 1" high and in Poudenx. Happily, there were no futher problems.
This area was had much more vegetation that at Auch. Oak forests and small meadows and fields. Perfect roe deer country. We always saw many animals, but far more does than bucks. That not withstanding, I did fine. We even managed to discover a Rotte of boar at the edge of a forest one evening. I bagged a big one, but it was a sow with no tusks.
But Fortune is fickle. Sometimes she is with the quarry, and sometimes with the hunter. Up until now, I thought she was giving me the "Fickle Finger of Fate" but work duties took Frederic back to his shop and Lucas took over as the guide. The day before, he had seen a good buck and wanted to take me to see if we could find him.
We stalked over a kilometer, maybe two, always going uphill through the forest. Then we came to a series of small meadows and small corn fields. The corn was about 12 inches high. We stalked extremely carefully. In the tall meadow grass, Lucas saw ears. A doe. She saw us, too, but could not wind us and she soon lay down again. No buck stood up.
On to the edge of the corn field the forest jutted out into the field like an isthmus. This place became our stand with our view to the East. The sun had just gone down. On the very first glassing of the corn field Lucas got a bit excited and set up the shooting sticks. Meanwhile, I could see the buck, too. He was bedded down between the corn rows. I could not see any of this body, but plenty of antler sticking up. I got the rifle settled in on the sticks and said to Lucas, "OK. Get him up." Lucas yelled. Nothing happened. He yelled again and louder. The ears twitched. The he really yelled very loud and there was movement. I knew from experience, even as athletic and graceful and quick as roe deer are, they do not arise from sleep any too fast. First front legs, then stand, and then stretch. And old fickle Fortune, she was sitting right there on my shoulder. Up he stood. The shot was good and the buck was down.
In 48 years of hunting roe bucks, the one that lay before me in the corn was the best I had ever seen, let alone to have shot. An old buck, with heavy atypical thick beamed antlers. A dream buck. The buck of a lifetime!
- James Lowell Males
Red Deer/Red Stag (Europe)
Videnskabeligt navn: Cervus elaphus Underarter: For Europa har vi medtaget følgende underarter: Østeuropæisk kronhjort (C.e.montanus) Corsikansk kronvildt (C.e.corsicanus) Udbredelse: Kronvildtet findes i de fleste europæiske lande. Levested: Skov, agerland, mose og hedearealer. Findes i både lavland, højland og bjerge. Beskrivelse: Stor hjorteart, der...
The two roe deer districts are respectively in the Gers region, close to the town of Auch , and in the Pyrenees Atlantique, where the regional center is Pau. From Toulouse it's around a 1½ hour drive to Auch, while the districts close to Pau are circa a 3-3½ hour drive from Toulouse. It's also possible to fly to Pau, from where it's about an hours drive from the...
Hunting Area - Chamois hunting in France
Through our well known French partner in Mauritius we have now been granted access to an exclusive hunting district in the South of France just 75 km from Nice. This makes this tour absolutely perfect for a family holiday, as your family can enjoy the delights of Nice while you are out hunting. A wide range of flights to Nice are available, and we would be happy to help with making flight...
As far as mountain hunters are concerned, here we can offer 2 different hunting districts. The first district, the Hautes-Pyrenees, lies south west of Toulouse in the 65th Departement close to the well known town of Lourdes. Here the hunting season is from 15th October to 15th November, and it is only possible to hunt on Saturdays and Sundays.The second district, the Pyrenees Orientales,...
Red Stag hunting in France
In the Pyreneese we can offer two different red deer districts. The first is close to Font-Romeu which is to the west/south west of Perpignan and the other is near Massat, to the south of Toulouse. Here you will enjoy challenging hunting in very beautiful, varied, forest-clad terrain. You can come across both dense forest, clearings, beautiful mountain meadows and barren rocky areas. There...
Sudchasse
Our partner, Julien, is both an outfitter and professional hunter, who has been carefully building up his successful company over a number of years. We have enjoyed an excellent working relationship with Julien since 2012, and he has really taken great care of our hunters, giving them some great hunting experiences and some fine trophies. Julien offers roebuck hunting during the...
Erik Hahn Pedersen
E-mail: ehp@diana.dk
Telephone: +(45) 22 19 86 54
Choose Contact Person Erik Pedersen BrinkmannErik Hahn Pedersen