Count me in as well. And, please, someone here hold me to that As ashamed as I am, I've been one that has said I wanted to take it for about 3 years now and, of course, haven't yet.
The last Cervantes placement exam I took put me at B1.4, which was a little of a disappointment. Perhaps, it's time to try it again since it's been a while.
The SIELE sounds interesting. There is a testing center just 2.5 hours from me, that's definitely doable. I might have to consider taking it instead of the DELE since there would be a flight to reach a DELE testing center (too far to drive). Only being good for two years is disappointing but maybe I can live with that.
Six members have discussed the exam in the log area since December.
reineke wrote: Six members have discussed the exam in the log area since December.
Ah, I've found Sgt Schultz and FrannieB. Could you post links or names of the others, please?
Well I would be one of those members discussing in the log area.
I was going to take the DELE was completely all set and then started researching the SIELE and shifted focus.
I am learning Spanish for me so this is a hobby for me. I had reached a point of not having good study habits between my travels to Central America and my husband was having a hard time continuing to support that habit when I was not studying that much at home.
I have a fairly academic job which requires a great deal of creating output for said job. So finding a sustainable study program has been a challenge. For me meeting with my Italki tutor 4 days a week works along with a bunch of other stuff. At this point he has been my tutor for over 2 years and I spend so much time with him on skype he is like family. Before 4 times a week I was doing 2 times a week.
I will be taking the SIELE on March 23rd in Chicago. My confidence level of hitting a B1 is about a 5 - talking is going to kill me I do not spontaneously use the future, imperative or subjunctive. There is an outside chance I could hit a B2 and that is simply because I'm a good test taker and can usually figure out how to hack a test for a better score. Using the test prep books I have gotten some 100% on practice quizzes because I simply know how to test well. You can't hack the spoken part which will be my downfall.
If I hit B1 I will go for B2 in November most likely on the DELE (maybe the SIELE) if I hit a B2 by change I will go for the C1 at about this time again next year.
FrannieB wrote: Well I would be one of those members discussing in the log area.
I am looking forward to reading of your experience!
A question: there are preparatory books for the SIELE already?
In case of Spanish, I am likely to opt for this temporary solution. But I am certainly grateful my DALF and CAE were completed ages ago and are on my CV forever. I am maintaining both those languages, no need to feel like a fraud. But I simply cannot imagine having to pay, prepare, and sit each exam every two years (as I am still in the phase of life, when you might need a CV at any moment without a warning ).
Cavesa - you bring up a great point about the test. I am not doing it to add to add to my CV. I'm doing it to grow myself. Also I don't see this as my final test - I do see myself taking the DELE C1 next year. I'm hoping to get to a B2 on the test which at this point I think is possible with comp, listening and speaking. Writing well that is a whole other issue and well I struggle with writing in English so why would it be any different then Spanish.
Andy E - the book is now winging its way to me.


As mentioned previously, since graduating I've filled out what feels like and endless amount of surveys (probably actually something like 4). One of them is longitudinal and they have followed up from time to time. Last week I got an email from them asking about doing a phone interview. I don't particularly like talking on the phone so of course I whined a nearby relative.
Family sure has a way of giving one perspective, eh? Happy new year!


The Advisor's Journey to CRM Success, Pt. 3: Which CRM to Choose?
Just like selecting the right vehicle for your transportation needs, your CRM choice can make for a challenging or pleasant ride.
Following a thorough business readiness evaluation, it is time to begin the platform selection process. Platform specialization, budget and functionality (e.g., contact and activity management, templates, workflows and sales management) are a few of the considerations in this early part of the journey. With many good CRM options available on the market, sorting through the potential solutions requires a rigorous process and well-defined criteria.
There are several high-quality CRMs that are focused on the wealth management space. Industry-specific solutions tend to be better bets since ongoing product development will benefit from a critical mass of like-minded customers requesting similar functionality, such as integration with financial planning and portfolio management softwar). The end result is a product with superior out-of-the-box functionality and integration with other software solutions within the wealth management industry.
Next is the balance between budget and functionality. We break functionality into two categories, essential and advanced.
Essential functions include: Contact Management, 360° Client Management, and Activity Management.
Advanced functions include: Templates, Checklists, Workflows (Process Automation), Sales Management, and Mass Communication.
First, determine what functionality you want in a CRM system today as well as anticipating your future needs for the next five years. Then sift through the options balancing functionality needs with budget to narrow down the options.
At this stage of the journey, leveraging the knowledge and experience of others is the best thing you can do. Just like any trip, advice from those who have been there is irreplaceable. Get referrals, talk to independent third parties, read reviews and reports to get as much information as possible before making a final decision. Access free resources like the ActiFi CRM Software Key Insights Report.
CRM vendors, custodians, broker-dealers and third-party implementation partners can be great resources as well. Successful implementations and happy customers are in their best interest, so most are willing to share a plethora of useful information with potential customers. Most of the industry-specific vendors do an excellent job of help prospective customers determine if their solution is a good fit for the advisor's needs.
Platform selection is a prime time to make sure there are key internal voices at the table. Ensuring that input from all parts of the organization that will use the system is gathered and considered will make the rest of the journey significantly more comfortable and productive for everyone.
Leaders and project champions will set the tone internally for everyone else who is eventually touched by the project. If leadership and project champions are fully on board with the software selected, their behavior will be significantly more positive and drive much better results.
Once you have selected your platform it is time to finalize preparations for your Journey to CRM Success.
In my next post I will discuss the planning, data review and set-up required for success – a little like packing for a successful vacation.
View the entire series, so far, of Spenser Segal on CRM Success.


A group formed to educate people about the dangers of pesticide use. Let's keep our roots non-toxic.
Sometimes ignorance can be an excuse. It was mine.
Although I knew pesticides weren't safe, I didn't realize they were lethal. So my dog Lilac lead a good life, running on the grass alongside my bike, until she died young of cancer in 1992. That's when I discovered that many dogs were dying young. If dogs who sniff the grass die of cancer, what happens to babies who crawl, children who play, families that picnic on treated grass? What happens when we track pesticides onto our carpets?
On August 11, 2001, a pesticide sign got caught in my spokes; my bike landed on my foot and broke it. Jim Stingl wrote a column in the Journal-Sentinel about the irony of an eco-poet breaking her foot on a pesticide sign, accompanied by a photo of me in my cast, and I became a magnet for everyone's pesticide horror story. I felt I had to do something, so I formed Grass Roots. I don't want ignorance to be anyone's excuse.
The Inside Story of My Foot, Suzanne's journal of her broken foot.
"One day the vet said Lilac had cancer, the next day Lilac was dead! That's how I discovered, in 1992, that dogs all over the Milwaukee area were dying of cancer, apparently triggered by pesticides. So it follows that pesticides also affect humans..."
Then perhaps we look more closely.
Click the titles below to view the whole poem.
As I bike around the neighborhood, I often think of Lilac, who loved to run alongside my bike, sniffing the lawns. This is the poemsong I wrote in her honor.
A healthier community starts in your own back yard.
"Healthy Communities Project (HCP) started very simply, with a young mother's letter to the local paper expressing concern about the widespread use of pesticides in the community and their potential effects on children and the environment, namely Lake Michigan... "
The Organic Consumers Association (OCA) is a grassroots non-profit public interest organization which deals with crucial issues of food safety, industrial agriculture, genetic engineering, corporate accountability, and environmental sustainability.
"In every yard and on every property, there's the need and potential to improve environmental health and habitat quality. With the right tools and information, we all can do simple things in the course of our everyday life to create healthier habitat for birds, plants, and other wildlife..."
The Cancer Prevention Coalition, Inc.
The Cancer Prevention Coalition, Inc. (CPC), which opened its national office in Chicago in July 1994, is a unique nationwide coalition of leading independent experts in cancer prevention and public health, together with citizen activists and representatives of organized labor, public interest, environmental and women's health groups.
GMHLT is a group of citizens in the Madison, Wisconsin area who are concerned with the health and environmental effects of pesticide and fertilizer use in our community.
PANNA (Pesticide Action Network North America) works to replace pesticide use with ecologically sound and socially just alternatives.
"one-stop location for current toxicity and regulatory information for pesticides"
The Center for Children's Health and the Environment (CCHE) is the nation's first academic research and policy center to examine the links between exposure to toxic pollutants and childhood illness.
"CHEC's mission is to inform parents and all those concerned with the welfare of children about preventable health and development problems caused by exposures to toxic substances in homes, schools and communities and to encourage the public to take action to protect children against these toxic threats."
Beyond Pesticides seeks safe air, water, land and food for ourselves and future generations.
Natural Yard Care - natural lawn care, waterwise and organic gardening, biological pest control, mulching, etc.
Practical information from John Sankey, field-naturalist in the Ottawa Valley, eastern Ontario, Canada.
Grass Roots member Kate Teipel's site has information plus you can order Pesticide Free Lawn signs . Sales help support cancer research and environmental causes.
Global Recognition Campaign for Multiple Chemical Sensitivity and other Chemically Induced Illnesses, Diseases & Injury. Extensive data and links on chemicals and the serious health effects thereof on human health & environmental health. A lot of information also on safer alternatives and some personal stories of chemically induced illnesses.
You may use this form to contact me for more information about Grass Roots. When you have completed filling in the form, please click the Submit button below to send the email message. Note, all fields are required.
How did you hear about our web site?
Copyright © 2003-6 Suzanne Rosenblatt. All rights reserved.


text:
Resort Fashion
Published by On the Beach 1st March 2012 | Tips and Guides
Resort wear has come quite a long way since the Victorian Age, when women layered sleeved woolen bathing dresses, bloomers, hose and shoes for a day at the beach. Beginning with the advent of the two-piece bathing suit, first introduced in the 1930s, swimwear has become a mode of fashion all its own. If you're planning beach holidays this summer, take a trip back in time with us for a lesson in the evolution of the swimsuit.
The Fab Fifties
By the 1950s, it was common for women's resort wear to feature swimsuits that showed a narrow strip of bare mid-drift and bare shoulders. Still, a full-on bikini was considered rather risqué, even in liberal fashion strongholds like Paris. In Cannes, however, saucy starlets began showing up to media events in tight-fitting two-piece suits with very small tops and bottoms that hit several inches below the navel. An 18-year-old Brigitte Bardot and a young Marilyn Monroe were at the forefront of the movement.
The Swinging Sixties
In the 1960s, bikinis began to slowly shift from costumes for pin-ups and film stars to common wear for ordinary women on beach holidays. Straps were removed, waistlines lowered and the navel was almost always exposed toward the end of the decade. Fuelling the fire were cultural innovations: "Sports Illustrated" issued its first swimsuit edition in 1962, and Hollywood's beach movie craze dominated the era.
The Sexy Seventies
The loosening sexual attitudes of the late 1960s ushered in a new breed of bathing suit in the 1970s. Designers begin making dramatic changes to proportion and silhouette. Resort lines featured string bikinis for the first time, with bottoms held together by ties, knots, straps and rings. In Europe, the topless bikini was making its mark in print and on real women on beach holidays in Tenerife, Monaco and other popular destinations. In 1975, French "Elle" featured models sporting topless G-string bikinis on public beaches. The tanga suit – characterised by a high cut leg line and zero rear-end coverage – also emerges on the fashion scene.
Evolution in the Eighties
By the mid-1980s, the string bikini had permanently stepped out of the pages of the glossies and firmly established its presence on the beach holidays scene. Average women embraced the sexy silhouette; the smaller, the better. Another hot trend on the beaches in the '80s was the sky-high leg line. This was usually accomplished by narrowing the front of the suit bottom, resulting in the aptly named V-kini. The classic bandeau top – by this time just a thin strip of Spandex held on the body by elastic – was revived during the decade.
Old is New Again
Today's top resort wear trends feature swimwear as clothing, paired with blazers and skirts; flowing maxi dresses and billowing pyjama pants as cover-ups; dresses and separates inspired by wetsuits; and lots of vintage silhouettes, caftans and capes. Of course, there will always be a market for skimpy swimwear. After all, isn't that part of the fun of beach holidays?
Tags: Blog, Fashion, On the Beach
Written on 1st March 2012 by

redpajama_set_name: RedPajamaCommonCrawl


A FORCED WEDDING A DOUBLE EXECUTION TWO THIEVES HAVE OTHER PLANS.The New Empire intends to celebrate its victory over the Nationalists with a day that will never be forgotten On the high holiday of Wintertide, they plan to execute two traitors Degan Gaunt and the Witch of Melengar as well as force the Empress into a marriage of their own design But they didn t accounA FORCED WEDDING A DOUBLE EXECUTION TWO THIEVES HAVE OTHER PLANS.The New Empire intends to celebrate its victory over the Nationalists with a day that will never be forgotten On the high holiday of Wintertide, they plan to execute two traitors Degan Gaunt and the Witch of Melengar as well as force the Empress into a marriage of their own design But they didn t account for Royce and Hadrian finally locating the Heir of Novron or the pair s desire to wreak havoc on the New Empire s carefully crafted scheme.
Did you miss The Disappearance of Winter s Daughter Kickstarter You can buy copies from our online store, now The book will be in the retail chains in October 6 time Goodread Choice Award Nominee I m honored that Age of Myth was one of the 200 most popular books of 2016 Thanks for visiting my page I m Michael J Sullivan, and I first opened the door to my imagination with typewriter keys while playing hide and seek and finding a black behemoth when I just ten years old These days I use computer keys and write classic fantasy and sci fi with unlikely heroes.I m thrilled with the reception my books have received, and delighted that they have appeared on than 150 best of or most anticipated lists including those compiled by 4 Choice Award Nominations , Barnes and Noble, and Audible Here s a bit about my series THE LEGENDS OF THE FIRST EMPIRE A new adventure begins 3,000 years in the pastSince time immemorial, humans have worshipped the gods they call Fhrey, truly a race apart invincible in battle, masters of magic, and seemingly immortal But when a god falls to a human blade, the balance of power between men and those they thought were gods changes forever Now, only a few stand between humankind and annihilation Raithe, reluctant to embrace his destiny as the God Killer Suri, a young seer burdened by signs of impending doom And Persephone, who must overcome personal tragedy to lead her people The Age of Myth is over the time of rebellion has begunE RIYRIA REVELATIONS CHRONICLES The best friendships are forged in blood.They killed the king They pinned it on two men They chose poorly.There s no ancient evil to defeat or orphan destined for greatness, just two guys in the wrong place at the wrong time Royce Melborn, a skilled thief, and his mercenary partner, Hadrian Blackwater, are enterprising thieves who end up running for their lives when they re framed for the death of the king Trapped in a conspiracy that goes beyond the overthrow of a tiny kingdom, their only hope is unraveling an ancient mystery before it s too late.
Excellent ending to a fine fantasy series.
2016 reread verdict:I have now officially decided that I will reread this series once a year due to it's excessive kickassyness. Yep. If too much of a good thing is bad for you, then this book is POISON. As Alice Cooper meant to say - "I wanna read you, but I want it too much"Original review:What a great series. Michael J. Sullivan has captured the essence of fantasy and spoiled us with a well written story that ticked all the boxes and more.
This series is a masterpiece and the final book is the worthy culmination.


text:
https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTUxODQwLXRyZWF0bWVudA==
Drugs & Diseases > Pediatrics: Genetics and Metabolic Disease
Metachromatic Leukodystrophy Treatment & Management
Author: Anna V Blenda, PhD; Chief Editor: Luis O Rohena, MD, PhD, FAAP, FACMG more...
Metachromatic Leukodystrophy
Sections Metachromatic Leukodystrophy
Symptomatic supportive care is indicated for problems including, but not limited to, behavioral disturbances, feeding difficulties, seizures, and constipation. No effective treatment is available to reverse the deterioration and loss of function that metachromatic leukodystrophy causes.
Several treatment approaches are promising and include bone marrow or blood transplantation, gene therapy, enzyme replacement therapy, and cell therapy.
Marrow or blood transplantation
Allogeneic blood or marrow transplantation is a standard of care for patients with certain inborn errors of metabolism. [33] Early and late outcomes show promise for MLD pediatric patients after cord blood transplantation. [34] Remyelination was observed after hematopoetic stem cell transplantation (HSCT), and importance of immunomodulation in addition to metabolic correction in MLD patients was highlighted for promotion of white matter recovery. [35]
In individuals with asymptomatic late infantile and early juvenile forms of the disease, bone marrow or cord blood transplantation may stabilize neurocognitive function [36, 37] ; however, symptoms of motor function loss frequently progress. Mildly symptomatic and asymptomatic late juvenile and adult-onset forms are more likely to be stabilized with bone marrow or blood transplantation because of slower disease progression.
Patients should be carefully evaluated and counseled prior to bone marrow transplantation. The migration of hematopoietically derived cells in sufficient numbers to treat the affected areas usually requires 6 months to 1 year. During this interval, the patient's condition continues to deteriorate. Although transplantation may be successful, enzyme release to surrounding tissues can widely vary, often with unpredictable benefits.
In addition, the transplantation conditioning regimen and the catabolic state of the patient during transplantation may contribute to a brief period of accelerated deterioration. A study has shown that in children with juvenile metachromatic leukodystrophy, if disease progression does happen after hematopoetic stem cell transplantation (HSCT), it happens early after HSCT and proceeds faster than in children that did not go throught the transplantation. [38]
The bone marrow transplantation procedure can potentially carry significant morbidity and mortality rates. Therefore, patient counseling regarding the risks versus the potential for later stabilization of the disease is necessary.
Evaluation for transplantation includes careful neuropsychological and developmental testing to establish current levels of function and to provide findings for comparison with future results. Organ systems, including cardiac, pulmonary, liver, and kidney functions, require assessment. Brain MRI and a thorough neurologic examination should be included.
If patients are asymptomatic or mildly symptomatic, the evaluations mentioned above should be performed and multidisciplinary treatment needs to be discussed. The treatment may involve a geneticist, a metabolic specialist, a neurologist, a neuropsychologist, a pediatrician, a transplantation specialist, or a combination of several experts.
An appropriately matched and unaffected relative, in whom the cells manufacture adequate levels of arylsulfatase A, should serve as a donor. An appropriately matched unrelated donor may be used in centers with experienced staff, although this transplantation process carries higher morbidity and mortality rates. Bone marrow or placental (cord) blood may serve as the source of stem cells.
In addition to bone marrow transplantation, gene therapy is under development as a possible solution to correct the underlying genetic abnormality. [39, 40, 41, 42] Hematopoietic stem cell gene therapy shows evidence of safety and clinical benefit for late-infantile [43] and older pediatric patients with MLD. [44]
The study has shown that gene therapy can result in stable ARSA gene replacement with high enzyme expression, including in the cerebrospinal fluid. In the 3 patients who were treated prior to onset of symptoms, the early data suggest that the regimen is a safe method to halt progression of the disease. [45] Further information can be obtained regarding this clinical trial at the ClinicalTrials.gov website under its identifier: NCT01560182.
Researchers are developing innovative methods to overcome the barrier of getting adequate ARSA enzyme activity into the CNS. One such procedure involves transduction of neurospheres with a vector containing arylsulfatase A. [46]
Enzyme replacement
A therapeutic strategy that has been useful for patients with other metabolic storage diseases is direct enzyme replacement. The difficulty with this strategy has always been getting adequate enzyme activity into the CNS. Intravenous injections of a recombinant human arylsulfatase A in a mouse model of MLD initially demonstrated no evidence of impact on CNS stores of sulfatide. However, with a significant increase in the injection frequency, researchers were able to demonstrate a reduction in CNS stores. [47]
A new strategy called evolutionary redesign was used for murinization of 1 or 3 amino acid positions in the human ARSA (hARSA) protein, which increased the hARSA activity 3- and 5-fold, respectively, and no significant impact on the protein stability was observed. [48]
In the United States and Europe, clinical trials are being carried out to evaluate the safety and efficacy of a recombinant human ARSA (rhARSA) enzyme, Metazym (Shire HGT). The drug had obtained orphan drug status from the US Food and Drug Administration in early 2008. The phase I clinical trial for its use in children with late-infantile metachromatic leukodystrophy showed that the drug was safe. Unfortunately, the extension study was terminated because of a lack of efficacy (ClinicalTrials.gov, identifier NCT00681811).
With the theory that the route of administration may allow for better drug concentrations in the CNS [49] , a multicenter phase I/II clinical trial has been developed to evaluate the safety and efficacy of rhARSA administered intrathecally (ClinicalTrials.gov, identifier NCT01510028). This study is ongoing but closed to accrual. The open-label extension arm of this study is now open by invitation (ClinicalTrials.gov, identifier NCT01887938).
Results of the safety evaluation of intrathecal rhARSA administration showed that it was generally well tolerated by patients, and preliminary data suggest promise in further development of rhARSA therapy for patients with MLD. [50] A population pharmacokinetic model was developed on the basis of the above-described trial (NCT01510028) to further evaluate and predict the potential efficacy of intrathecal enzyme replacement therapy. [51]
Another therapeutic approach under study in mice is the use of oligodendroglial cell therapy. Givogri and colleagues [52] reported their transplantation of oligodendrocyte progenitors into mouse neonatal MLD brain. These cells engrafted and integrated without disruption or tumor formation. Compared with untreated control mice, the treated mice had reduced sulfatide accumulation in the CNS with increased enzyme activity and prevention of motor deficits. This therapeutic approach is not available for humans at this time.
Appropriate consultations involve the following specialists:
Genetic counselor
Neurodevelopmental psychologist
Bone marrow transplant physician
Genetic, metabolic disease specialist, or both
Referral or transfer to a major medical center with experience in treating inherited neurodegenerative and metabolic disorders in a multidisciplinary setting is highly recommended.
Follow-up evaluation and treatment are often needed. A physical therapist, occupational therapist, orthopedist, ophthalmologist, neuropsychologist, and other specialists may be involved.
Genetic counseling is important to inform the family regarding the risk of occurrence in future pregnancies. Metachromatic leukodystrophy is transmitted as an autosomal-recessive trait. Multiple genetic mutations have been implicated as causes of this disorder. [7] Available methods of prenatal testing should be discussed. Tests for a deficiency in enzyme activity in amniocytes or amniotic chorionic villi and gene mutation analysis may be available.
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Morena F, Argentati C, Acquati S, et al. Toward Reference Intervals of ARSA Activity in the Cerebrospinal Fluid: Implication for the Clinical Practice of Metachromatic Leukodystrophy. J Appl Lab Med. [published online ahead of print, 2020 Sep 10]. [QxMD MEDLINE Link]. [Full Text].
Meikle PJ, Grasby DJ, Dean CJ. Newborn screening for lysosomal storage disorders. Mol Genet Metab. 2006 Aug. 88(4):307-14. [QxMD MEDLINE Link]. [Full Text].
Faerber EN, Melvin J, Smergel EM. MRI appearances of metachromatic leukodystrophy. Pediatr Radiol. 1999 Sep. 29(9):669-72. [QxMD MEDLINE Link]. [Full Text].
Wadhwa A, Chen Y, Holmqvist A, et al. Late Mortality after Allogeneic Blood or Marrow Transplantation for Inborn Errors of Metabolism: A Report from the Blood or Marrow Transplant Survivor Study-2 (BMTSS-2). Biol Blood Marrow Transplant. 2019 Feb 01. 25(2):328-334. [QxMD MEDLINE Link]. [Full Text].
van den Broek BTA, Page K, Paviglianiti A, et al. Early and late outcomes after cord blood transplantation for pediatric patients with inherited leukodystrophies. Blood Adv. 2018 Jan 04. 2(1):49-60. [QxMD MEDLINE Link]. [Full Text].
Wolf NI, Breur M, Plug B, et al. Metachromatic leukodystrophy and transplantation: remyelination, no cross-correction. Ann Clin Transl Neurol. 2020 Feb. 7(2):169-180. [QxMD MEDLINE Link]. [Full Text].
Krivit W. Allogeneic stem cell transplantation for the treatment of lysosomal and peroxisomal metabolic diseases. Springer Semin Immun. 2004. 26:119-132. [QxMD MEDLINE Link]. [Full Text].
Martin PL, Carter SL, Kernan NA. Results of the cord blood transplantation study (COBLT): outcomes of unrelated donor umbilical cord blood transplantation in pediatric patients with lysosomal and peroxisomal storage diseases. Biol Blood Marrow Transplant. 2006 Feb. 12(2):184-94. [QxMD MEDLINE Link]. [Full Text].
Beschle J, Döring M, Kehrer C, et al. Early clinical course after hematopoietic stem cell transplantation in children with juvenile metachromatic leukodystrophy. Mol Cell Pediatr. 2020 Sep 03. 7(1):12. [QxMD MEDLINE Link]. [Full Text].
Consiglio A, Quattrini A, Martino S, et al. In vivo gene therapy of metachromatic leukodystrophy by lentiviral vectors: correction of neuropathology and protection against learning impairments in affected mice. Nat Med. 2001 Mar. 7(3):310-6. [QxMD MEDLINE Link]. [Full Text].
Matzner U, Habetha M, Gieselmann V. Retrovirally expressed human arylsulfatase A corrects the metabolic defect of arylsulfatase A-deficient mouse cells. Gene Ther. 2000 May. 7(9):805-12. [QxMD MEDLINE Link]. [Full Text].
Penati R, Fumagalli F, Calbi V, Bernardo ME, Aiuti A. Gene therapy for lysosomal storage disorders: recent advances for metachromatic leukodystrophy and mucopolysaccaridosis I. J Inherit Metab Dis. 2017 Jul. 40 (4):543-554. [QxMD MEDLINE Link]. [Full Text].
Rosenberg JB, Kaminsky SM, Aubourg P, Crystal RG, Sondhi D. Gene therapy for metachromatic leukodystrophy. J Neurosci Res. 2016 Nov. 94 (11):1169-79. [QxMD MEDLINE Link]. [Full Text].
Calbi V, Fumagalli F, Consiglieri G, et al. Use of Defibrotide to help prevent post-transplant endothelial injury in a genetically predisposed infant with metachromatic leukodystrophy undergoing hematopoietic stem cell gene therapy. Bone Marrow Transplant. 2018 Jul. 53(7):913-917. [QxMD MEDLINE Link]. [Full Text].
Tucci F, Frittoli M, Barzaghi F, et al. Bone marrow harvesting from paediatric patients undergoing haematopoietic stem cell gene therapy. Bone Marrow Transplant. 2019 Dec. 54(12):1995-2003. [QxMD MEDLINE Link]. [Full Text].
Biffi A, Montini E, Lorioli L, Cesani M, Fumagalli F, Plati T. Lentiviral hematopoietic stem cell gene therapy benefits metachromatic leukodystrophy. Science. 2013 Aug 23. 341(6148):1233158. [QxMD MEDLINE Link]. [Full Text].
Kawabata K, Migita M, Mochizuki H. Ex vivo cell-mediated gene therapy for metachromatic leukodystrophy using neurospheres. Brain Res. 2006 Jun 13. 1094(1):13-23. [QxMD MEDLINE Link]. [Full Text].
Matzner U, Herbst E, Hedayati K, et al. Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy. Hum Mol Genet. 2005 May. 14(9):1139-1152. [QxMD MEDLINE Link]. [Full Text].
Simonis H, Yaghootfam C, Sylvester M, Gieselmann V, Matzner U. Evolutionary redesign of the lysosomal enzyme arylsulfatase A increases efficacy of enzyme replacement therapy for metachromatic leukodystrophy. Hum Mol Genet. 2019 Jun 1. 28(11):810-1821. [QxMD MEDLINE Link]. [Full Text].
van der Veldt N, van Rappard DF, van de Pol LA, van der Knaap MS, van Ouwerkerk WJR, Becher JG, et al. Intrathecal baclofen in metachromatic leukodystrophy. Dev Med Child Neurol. 2019 Feb. 61 (2):232-235. [QxMD MEDLINE Link]. [Full Text].
Í Dali C, Sevin C, Krägeloh-Mann I, et al. Safety of intrathecal delivery of recombinant human arylsulfatase A in children with metachromatic leukodystrophy: Results from a phase 1/2 clinical trial. Mol Genet Metab. 2020 Jul 16. S1096-7192(20):30152-9. [QxMD MEDLINE Link]. [Full Text].
Troy S, Wasilewski M, Beusmans J, Godfrey CJ. Pharmacokinetic Modeling of Intrathecally Administered Recombinant Human Arylsulfatase A (TAK-611) in Children With Metachromatic Leukodystrophy. Clin Pharmacol Ther. 2020 Jun. 107(6):1394-1404. [QxMD MEDLINE Link]. [Full Text].
Givogri MI, Galbiati F, Fasano S. Oligodendroglial progenitor cell therapy limits central neurological deficits in mice with metachromatic leukodystrophy. J Neurosci. 2006 Mar 22. 26(12):3109-19. [QxMD MEDLINE Link]. [Full Text].
Table 1. Characteristics of the 4 Forms of Metachromatic Leukodystrophy
Age at
Effect of Bone
Marrow
Late infantile
Autosomal recessive
Motor milestones lost, neurocognitive function lost
Death within 5-6 y
Not helpful in symptomatic patients; may halt cognitive deterioration in asymptomatic patients
Early juvenile
Less common
Motor milestones lost, learning and behavior impaired
Death within 10-15 y
May be beneficial in symptomatic and asymptomatic patients
Late juvenile
Personality changes, behavioral changes, dementia, psychoses, decline in school or work performance
May be beneficial in asymptomatic or mildly symptomatic patients
Anna V Blenda, PhD Clinical Associate Professor, Department of Biomedical Sciences, University of South Carolina School of Medicine, Greenville
Anna V Blenda, PhD is a member of the following medical societies: American Association for Cancer Research, American College of Medical Genetics and Genomics, American Society for Microbiology
Luis O Rohena, MD, PhD, FAAP, FACMG Deputy Chief, Department of Pediatrics, Chief, Medical Genetics, San Antonio Military Medical Center; Associate Professor of Pediatrics, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine; Associate Professor of Pediatrics, University of Texas Health Science Center at San Antonio
Luis O Rohena, MD, PhD, FAAP, FACMG is a member of the following medical societies: American Academy of Pediatrics, American Chemical Society, American College of Medical Genetics and Genomics, American Society of Human Genetics
Karl S Roth, MD Retired Professor and Chair, Department of Pediatrics, Creighton University School of Medicine
Karl S Roth, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Nutrition, American Pediatric Society, American Society for Nutrition, American Society of Nephrology, Association of American Medical Colleges, Medical Society of Virginia, New York Academy of Sciences, Sigma Xi, The Scientific Research Honor Society, Society for Pediatric Research, Southern Society for Pediatric Research
Robert D Steiner, MD, FAAP, FACMG Professor (Clinical), Department of Pediatrics, University of Wisconsin School of Medicine and Public Health; Editor-in-Chief, Genetics in Medicine; Chief Medical Officer, PreventionGenetics
Robert D Steiner, MD, FAAP, FACMG is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Medical Genetics and Genomics, American Society of Human Genetics, Society for Inherited Metabolic Disorders, Society for Pediatric Research, Society for the Study of Inborn Errors of Metabolism
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: PreventionGenetics, ACMG, Acer Therapeutics, Biomarin; Best Doctors, Health Advances, Precision for Health, PTC Therapeutics, CTX Alliance, Aeglea, Eton, Leadiant<br/>Serve(d) as a speaker or a member of a speakers bureau for: France Foundation, Medscape<br/>Received research grant from: Alexion<br/>Received income in an amount equal to or greater than $250 from: Marshfield Clinic, France Foundation, Medscape, PreventionGenetics, ACMG, Acer Therapeutics; Raptor, Retrophin/Travere, Censa; Biomarin; Best Doctors, Health Advances, Precision for Health, PTC Therapeutics, Aeglea, Leadiant<br/>Travel Support for: CTX Alliance.
Theodore Moore, MD, MS Professor and Chief, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Director of Pediatric Blood and Marrow Transplant Program, University of California, Los Angeles, David Geffen School of Medicine
Theodore Moore, MD, MS is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, Society for Pediatric Research, American Society for Blood and Marrow Transplantation, Western Society for Pediatric Research, American Society of Hematology
David Flannery, MD, FAAP, FACMG Vice Chair of Education, Chief, Section of Medical Genetics, Professor, Department of Pediatrics, Medical College of Georgia
David Flannery, MD, FAAP, FACMG is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics and Genomics
Alan K Ikeda, MD Interim Medical Director, Director of Oncology, Children's Specialty Center of Las Vegas
Alan K Ikeda, MD is a member of the following medical societies: American Academy of Pediatrics, American Society for Blood and Marrow Transplantation, American Society of Pediatric Hematology/Oncology
Disclosure: I own stocks in various pharmaceutical companies. This varies on a given week for: multiple and varies.
encoded search term (Metachromatic Leukodystrophy) and Metachromatic Leukodystrophy
Lysosomal Storage Disease
Inclusion-Cell (I-Cell) Disease (Mucolipidosis Type II)
Fast Five Quiz: Glycogen Storage Disease Type II (Pompe Disease)
Sialidosis (Mucolipidosis I)
Lipid Storage Disorders
Genetics of Glycogen-Storage Disease Type II (Pompe Disease)
Treating Deadly Disease in Utero Called 'Revolutionary' Advance
NICE Grants Routine Access to Treatment for a Rare Metabolic Disorder
Next-generation Antibody-based Therapies in Neurology
20021182830-overviewDiseases & Conditions
Diseases & Conditions Lysosomal Storage Disease
Diseases & Conditions Lipid Storage Disorders
Diseases & Conditions Sialidosis (Mucolipidosis I)


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Boris Johnson and health chiefs to face questions on coronavirus vaccine rollout
January 13 2021, 12.10am Updated: January 13 2021, 1.06am
Boris Johnson and health chiefs will be questioned on the rollout of the coronavirus vaccine after warnings to abide by lockdown restrictions were stepped up to ease pressure on the NHS (Eddie Mulholland/The Daily Telegraph/PA)
Boris Johnson and health chiefs will be questioned on the rollout of the coronavirus vaccine after warnings to abide by lockdown restrictions were stepped up to ease pressure on the NHS.
The Prime Minister will be grilled by senior MPs on the liaison committee after facing Labour leader Sir Keir Starmer during PMQs on Wednesday.
Vaccines minister Nadhim Zahawi and health bosses crucial to the jabs programme will also be questioned by the Commons science and technology committee.
With rolling out the vaccines key to easing lockdowns, the latest NHS figures showed 2,431,648 in the UK have received a first dose.
But the health service was under extraordinary pressure with the number of Covid-19 patients in hospital in England at a record 32,202 as of 8am on Tuesday – up 22% on a week ago, and 82% since Christmas Day.
Latest figures showed that as of Tuesday, a further 1,243 people in the UK had died within 28 days of testing positive for Covid-19, with a further 45,533 cases being confirmed by labs.
Separate figures published by the UK's statistics agencies for deaths where Covid-19 has been mentioned on the death certificate, together with additional data on deaths that have occurred in recent days, show there have now been 99,000 deaths involving Covid-19 in the UK.
The Scottish Government is considering further Covid-19 restrictions as the death toll from the virus passed 5,000. First Minister Nicola Sturgeon will outline any changes to the Scottish Parliament on Wednesday.
NHS Providers chief executive Chris Hopson told MPs the peak demand on the health service may not be reached until early to mid-February.
To offer some respite, hospital patients are to be discharged early from one London hospital in order to free beds for critically-ill Covid-19 patients.
The owners of the Best Western chain, the London Hotel Group (LHG), will accommodate patients deemed ready to leave King's College Hospital.
A hospital spokesman said: "To create capacity in the hospital to care for the high number of patients requiring admission, we have partnered with a local hotel to temporarily accommodate mainly homeless patients who are ready to safely leave hospital and will benefit from further support from community partners."
The Guardian reported an LHG spokesman saying the patients "are recovered or recovering from Covid and who are medically fit for discharge" so do not need specialist medical supervision.
Meanwhile, the NHS denied a report in the Telegraph that suggested the vaccine rollout was being paused in some areas to allow other parts of the country to catch up.
A spokesman said: "Actually, all GP sites are getting vaccine deliveries this week and next."
Scotland could vaccinate people against coronavirus round the clock in a bid to see people given protection against the virus as quickly as possible, Ms Sturgeon said.
She did not rule out younger people being offered vaccination appointments on a 24/7 basis, saying: "We will look at anything and everything that allows us to get this vaccination programme done as quickly as possible."
The latest pandemic lockdown in Northern Ireland is likely to last beyond February 6, the region's chief medical officer said.
Dr Michael McBride said restrictions will be required for a "considerable number of months".
Amid concerns the current restrictions in England may not sufficiently supress the virus, Home Secretary Priti Patel said a minority of the public are "putting the health of the nation at risk" as she backed a tougher police approach to lockdown rules.
She told a Downing Street press conference on Tuesday that officers are moving more quickly to issuing fines where people are clearly breaching coronavirus regulations, with nearly 45,000 fixed penalty notices issued across the UK since March.
Chairman of the National Police Chiefs' Council Martin Hewitt said that more officers will be out on dedicated patrols to deal with rule breakers, while Communities Secretary Robert Jenrick has also spoken to councils about enforcing the regulations.
But leaders of police groups called on the Government to clarify the "incredibly vague" rules, which saw two women incorrectly fined for driving five miles from their Derbyshire homes to meet for a walk while the Prime Minister went for a bike ride seven miles from Downing Street.
In other developments:
– The Government faced calls to urgently roll out its national free school meal voucher scheme after one mother posted an image of a £30 parcel estimated to contain just over £5 worth of food.
– Tesco, Asda and Waitrose became the latest supermarkets to take a tougher stance on customers who refuse to wear a face covering without a medical exemption.
– Data from the Office for National Statistics showed that more than 40,000 extra deaths have taken place in private homes in England and Wales since the start of the coronavirus pandemic.
Dame Cressida Dick
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text:
Elections Are Like Water
Manuel Quezon III
Elections are like water, missed only in its absence
For the overwhelming majority of Filipinos who have no recollection of life before martial law, elections are like water – a requisite for political life, as essential for the body politic as water is for the human body. For the older generation, too, elections are like water – but as they view the arid desert that is our current political culture, they yearn for a time when the political landscape was lush, abloom with idealism, fragrant with the virtue of the leaders and the led, far, far removed from the horrifying sight of a nation tearing itself apart at the polls and beyond.
Elections are like water – a means of cleansing the body politic, a roaring torrent of votes channeled, like a river, to clean out the Augean stables every administration seems to become
In 1935, when the country had its first national election, fully a third of the voters didn't bother to cast their ballots. We had a million and a half registered voters then, all male, all literate. We were looking forward to self-rule preparatory to full independence; it would seem logical that the voters would be interested in participating in the creation of the foundations of their future nation state. But a significant number were not, and the reason they were not should come as no surprise. The choices at the time were limited: three candidates for president, and of that limited number, one was the overwhelming favorite. The result was a foregone conclusion, and at a time when all seemed to be going well, a third of the electorate found no reason even to bother. Manuel L. Quezon won by a landslide, with 68 percent of the vote, against Emilio Aguinaldo and Gregorio Aglipay, neither of whom managed to get even 18 percent. In 1941, with women added to the roster of qualified voters, the turnout remained about the same, that is, a third continued to find it pointless to vote, even though the incumbent, Quezon, this time got 81 percent of the vote.
In the democracies we Filipinos like to envy, a turnout of 66 percent of registered voters would be phenomenal; the United States regularly involves a far smaller percentage of its population in elections that not only determine the direction of their country, but profoundly affect the rest of the world as well. The turnout of voters in the supposedly halcyon days of Philippine democracy before the war points to a defining characteristic of our democracy: that a sizeable majority of Filipinos have put, and continue to put, great stock in the voting process. The difference is that the predictable nature of our political and national development before the war was ended by a series of shocks and disappointments in the national trauma that was World War II.
Between 1941 and 1946, the Philippines went through six heads of government: Manuel L. Quezon, Jorge Vargas, Jose P. Laurel, Sergio Osmeña, and Manuel Roxas. Within that five-year period, two leaders contested the title of legitimate president of the Philippines (Laurel sponsored by the Japanese at home; Quezon, then Osmeña, sponsored by the Americans in exile). Taking sides was no longer a political business, it was a bloody business. There were collaborators and guerrillas, officials-in-exile and officials in the hills, officials in Manila who claimed to be secret guerrillas and officials who were overtly pro-Japanese.
The end of World War II and the first national elections held after the wartime trauma set the stage for elections as we know them today. The pretense of political virtue, so carefully nurtured prior to the war, was difficult to sustain in a nation for which voting had become a life-or-death matter. Before World War II, elections were like water – they had a sacramental aspect, the anointing of a leader by his people. After World War II, elections were like water – they were viewed not only as a means to cleanse away filth, but as a fundamental requirement for survival. Voters torn and divided, guerrillas, fake guerrillas, genuine collaborators and the unfairly accused, landlords who had fled their estates to seek refuge behind Japanese bayonets (and who now clung to America for dear life), disgruntled peasants – whatever their circumstances, survivors all – now had a desperate stake in the outcome of elections.
In the elections of 1946, genuine guerrillas, radicals, and leaders who had been ruthlessly defeated during the two decades of Quezon's ascendance in national life fought desperately for their time in the sun. Behind Roxas rallied the orphaned apparatus of Quezon's party machine, both guerrillas and collaborators, but most importantly those accused of collaboration for whom political survival offered their only prospect for rehabilitation and escape from disgrace. Both sides courted a destitute nation that viewed independence with mixed excitement and dread, conditioned to think of 1946 as the year in which the carefully-built-up development of the prewar years would find fruition, only to find that independence would be a flag waving over ruins emanating the stench of death and decay.
A nation literally parched, its infrastructure in ruins, its population decimated, its ideals proven woefully hollow by the war, viewed elections like water – and like the fight of parched people for water, the fight for the elections was desperate. 1946 saw no landslide, but a simple majority. It also saw vote buying, allegations of vote rigging, and election-related violence on a scale previously inconceivable. It would only get worse in 1949, when Elpidio Quirino won in an election that achieved shocking notoriety around the world for raising the dead and even drafting flora and fauna to cast votes. Yet in 1953, the much-anticipated deluge came, when the charasmatic Ramon Magsaysay beat Quezon's 1935 record with 68.9 percent of the vote.
But by 1957 Magsaysay was dead. His successor, Carlos P. Garcia, in a field of seven candidates, was elected with just 41.3 percent of the vote. With the election of the first president by plurality, the Philippine political system of today was born. A nation doesn't always have leaders with flair and charisma who, by hard work and ruthless plotting, like Quezon, or sheer flair and even greater charisma, like Magsaysay, dominate national life. The overwhelming majority of politicians are humdrum, devious, but not outstandingly cunning people, and it is impossible for someone always to shine.
Add to this the weaning of the electorate from an era in which politicians were expected to be reserved and to view appealing to the masses as a vulgarity not to be indulged. Furthermore, add the natural shift in tastes and expectations from those of the jaded and, in a sense, morally bankrupt generation of politicians active from before the war, to those of the young, brash generation that actually fought in the war or had grown up since. And add, further, the steady erosion of presidential power and institutional mechanisms, such as block voting, that Quezon had carefully put in place and that Magsaysay had wielded by sheer force of character. The following was the result:
Garcia was a president shorn of many of the basic levers of control that his predecessors had taken for granted. A president born and bred by the party machine, whose partymates had eliminated block voting, a bulwark of party-oriented voting. A president who inherited the steady removal of local government appointments from the perogative of the presidency, a process begun in response to the domineering nature but weak political gifts of Quirino. An old-style politician at a time after Magsaysay had eroded the ability of old-style politicians to achieve majorities by simply commanding their surrogates to issue instructions to voters. A man who spoke Spanish and who was inaugurated in an old fashioned cutaway, when the electorate far preferred the rustic, barong Tagalog-wearing style of the late Magsaysay. This was an electorate poised to raise to the Senate Rogelio de la Rosa, whose only qualification was as a matinee idol. Garcia was a president occupying an office that voters expected to be wielded with the aplomb and color of a Quezon or Magsaysay.
The problem was, Garcia was like neither of these men. What Garcia was, as he himself said, "was not stupid," and for a time he and his successors proved that they were clever enough to grasp the remaining levers of power to make it to the presidency.
The popular vision of the presidency – both among voters and among politicians aspiring to the position – thus suffered from the larger-than-life personas of past presidents at a time when contemporary presidents could not (though try, they did) wield the powers of the executive with the same overarching authority and effectiveness. The means, under the law and under the system, simply wasn't there. But expectations on the part of voters remained the same; the ambition afflicting politicians remained the same; and the intensity of popular interest in elections remained at the fever pitch it had been at since 1946, especially after the brief, meteoric rise of Magsaysay. Garcia was turned out by Diosdado Macapagal for much the same reasons Quirino had lost to Magsaysay, but Macapagal proved woefully ill-equipped to wield executive power. He was duly thrust aside in 1965 by Ferdinand E. Marcos, who eventually saw no other way to achieve the power he craved, and retain the position he had no intention of relinquishing, than by scrapping the system altogether.
In 1969 Marcos received 61 percent of the vote, giving him the fourth-highest percentage of votes in a national election and making him the only president to achieve a second full term. Just as Quezon had set out to change the system to make it more conducive to executive control, so did Marcos set the stage for something even more daring. This infrastructure-minded president would view elections as a dam – a means to channel political control, allowing him to irrigate the fields of his cronies, leave parched the lands of his opponents, and present to the people a pharaoh-like image of irresistible and indomitable will that could change the course of nature.The water in the vast manmade lake of Marcos's New Society proved muddy, shallow, polluted, and foul. In 1986 the dam was breached and a more natural course of water flow restored. Corazon Aquino lost the official count but won where it counted, a moral victory in the eyes of her people and the world. From the start, she shed the reluctance of a shy widow for the increasingly confident role of a revolutionary restorer, accomplishing what her husband had set out to do – restoring elections, bringing back water, so to speak, to a nation parched for elections.
Cory Aquino was brought to power by election, indeed, by referendum, and she used elections-as-plebiscites as her key to maintaining legitimacy.
But the stage of development – or underdevelopment – of the political system that had emerged with the election of Garcia came back with a vengeance under Fidel Ramos, who holds the unenviable record of winning the smallest plurality (28 percent) in our electoral history. His success had a baleful influence on the idea of elections as legitimation for presidential governance. What was important in the post-Edsa [ed: 1986 People Power revolution] scheme of things was neither popularity nor machinery (his opponents had both), but tactical supremacy: doing the most with less. After the strange rise to power of Ramos, rejected by an overwhelming number of his countrymen, but esconced in the presidency anyway because he proved less unpopular than his opponents, it was no surprise that Joseph Estrada's election six years later took on the characteristics of a landslide, although in fact, it wasn't even a plurality as healthy as Garcia's. Both Ramos and Estrada were minority presidents occupying an office burdened with expectations institutionally impossible to fulfill. At the same time, the electorate was increasingly polarized, desperate, and despairing; a change in popular tastes and mass communications made Joseph Estrada the heir of Rogelio de la Rosa.
Without Ramos's tactical cunning and long experience at handling subordinates, Estrada proved inept at maintaining himself in power. His vice president, Gloria Macapagal-Arroyo, on the other hand, had achieved a post-Edsa first: a near-majority [ed: Filipinos cast separate votes for president and vice-president]. This armed her with the ability to invoke a mandate of her own as putative successor to Estrada. When Estrada fled Malacañang Palace, Arroyo deftly moved in (to cut a long story short), but under circumstances so unique as to be denied the natural advantages a successor-president historically enjoyed as heir apparent, keeper of the flame, inheritor of legitimacy.
The 2004 presidential campaign was a quest for legitimacy – legitimacy lost by Estrada's anointed candidate, not yet earned by the incumbent. A quest for legitimacy by other candidates who were dependent on neither the discredited leader in jail nor the incumbent grievously wounded by allegations that she deserved to be in jail as well. Both candidates and the electoral contest itself were overshadowed by the maturing of a political culture born in the 1960s, made ruthless in the 1970s, and morally bankrupted in the 1990s – yet which both leaders and led view through the rose-tinted spectacles of the struggle to restore credible elections in the 1980s. We have elections, just as we have water – not as the result of a rational, well thought-out plan of national action, but because of fits and starts by leaders who view voting as they view water – as handouts to inculcate gratitude among a mendicant populace.
The day must come when fully one-third of the electorate can decide to sit home and not vote, because whatever the results of the elections, nothing fundamental is at stake. Since 1946, the stakes have always been fundamental, which is why graft and corruption have always been fundamental national issues in those elections. What is at stake, for so many people, is the very hope of a life in which water, quite literally, can be theirs to drink and to bathe in, a life that isn't measured in one faucet per barangay [neighborhood] and by garbage-infested canals.
Elections are like water – essential to the parched, a source of power to those who control it or even own it. Elections are like water – possessing different meanings for different people. Elections are like water – for us, at least, in a country where each upper-class swimming pool represents scorn for the many barangays that must line up hours for something murky and foul to drink.
Manuel L. Quezon III is a columnist and contributing editor at the Philippine Daily Inquirer and History Curator at the Ayala Museum.
Kyoto Review of Southeast Asia. Issue 6 (March 2005). Elections and Statesmen
Pemilihan Umum Seperti Air
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text:
TCC scholarships help Big Bend area get back to work
Community college training workers in weeks
Florida experienced a loss of 418,500 jobs between November 2019 and November 2020.
By: Channing Frampton
TALLAHASSEE, Fla. (WTXL) — Florida experienced a loss of 418,500 jobs between November 2019 and November 2020. The regional unemployment rate for Gadsden, Leon, and Wakulla counties was 5.6 percent for November 2020. Gadsden County had the highest rate at 7.5 percent.
To bring about some relief, Tallahassee Community College is rolling out new opportunities outside of the city. They're continuing programs that could help you rebound into a new job this spring.
"It's a big deal," said Jessica Griffin, the director of Continuing Workforce Education at TCC.
"When we talk about Gadsden County, this definitely helps residents become upskilled and employed in areas where there is a high demand," she added.
TCC's scholarship program called "Gadsden Connect" is an opportunity for up to 60 people living in the county to get back on their feet.
It covers tuition, it covers books and uniforms. The idea gets people on track for a job in the healthcare field in a matter of weeks. Through a partnership with Sunshine Health, home health aides and certified nursing assistants are the main focus.
"They're beginning on a career ladder, a ladder that means promotion," explained Kim Moore, the vice president for Workforce Innovation at TCC.
Moore says Gadsden Connect along with their ongoing Be Essential program are filling jobs when more than 10,000 people in the Capital Region are out of work.
Outside of healthcare, Moore says some programs take no longer than 90 days to complete. They train people to work in areas including truck driving, IT, and public safety.
"It really is a commitment that they can continue to build on," Moore added.
While Gadsden Connect focuses on Gadsden County only, Be Essential is open to just about anyone.
There is still scholarship money available. The cutoff to apply is in March. For more information or to apply, click here.


Be on your guard: don't immerse yourselves in a life of pleasure, drunkenness and worldly cares, lest that day catch you unaware, like a trap! For, like a snare, will that day come upon all the inhabitants of the earth. But watch at all times and pray, that you may be able to escape all that is going to happen, and to stand before the Son of Man.


Distribution—North from Gloucester, New South Wales and Queensland.
Description—Climber with twining stems, latex milky, stems quickly developing a white fissured corky covering.
Cape Hawke, Tallwoods and Wingham Brush.
Inflorescence—Flowers 3 to 4 mm diam., white. Corolla with throat densely bearded, long hairs obscuring column; corolla lobes 2 to 3 mm long, reflexed, bearded near base. Corona segments short, basal part without wings or lobes, free upper part thin, broad, obtuse. Style head broad, shortly umbonate, covered by long anther appendages.
Fruit—Follicles ovoid, turgid, c. 6 cm long, c. 3 to 3.5 cm diam.


This has been a brilliant week at New Bridge Horizons. Firstly, I want to once again congratulate Sam who was the winner of the NHS Christmas card competition. Sam was presented with his prize and certificate on Tuesday and is absolutely thrilled to have won.
Sam continued his winning streak with a richly deserved 'Inspiration of 2015' Award from POINT, presented at their Annual Conference last week. Andrew Robinson explained that Sam has grown in confidence and skills over the last year (he designed the North West SEND logo along with POINT's own Christmas Cards) and has contributed massively to POINT with his volunteering. However, it is the art classes that Sam runs at Mahdlo on Sundays that really stand out – he has succeeded in reaching children who are having real difficulties at school or are not currently able to engage, and it is wonderful to see them focused and working with him. What a fantastic achievement, well done, Sam!
This week Jeff's group have been busy making Christmas decorations. I really enjoyed attending the DIY session this week and I am looking forward to seeing the finished results.
Today Emma's group have finally made it to Wheels for All on a dry day! After last week's wash out, it was a very enjoyable morning for all. The group also visited the brand new leisure centre for a swimming session and the staff and young people were really impressed with the new facilities!
On Thursday we had a leaving party for Thomas and I am still recovering from attempting the cha cha slide and have booked in for physio over the weekend! Just kidding! We had a lovely afternoon but were of course sad to see Thomas leave us. It has been a joy to have him with us for the past few years and we wish him every luck for the future. We will miss you, Thomas, and we hope that you liked your presents and cards.
Last night we held a meeting for families about our move to Medtia Square. If you missed it don't worry, we will be writing to you all in the coming weeks to share all the details. We are planning to move up to the new building from Monday 4th January and we have already started the transition process with our young people. We will, of course, keep you updated on the latest developments. Debbie Hall, our Director of Autism, has prepared a social story relating to the move which we will send home for our young people to be looking at over the coming weeks. We are currently preparing the building by installing new adapted bathrooms and decorating throughout. Once the work is complete we will be inviting our young people and their families to look around.
Our Future Finders have been working hard on their coursework this week to gain their City and Guilds qualification in Employability. Margaret Taylor, our teacher on the project, is extremely impressed by how much the students have developed their skills and knowledge in such a short time. We are also very proud of Alice who started her internship at the Oldham Coliseum today! Alice is working as an admin assistant within the education and marketing teams and is having a brilliant day! A huge thank you to the staff at Oldham Coliseum who made her feel so welcome.
That is all from me for this week. Have a lovely weekend… I hope you picked up some Black Friday bargains for Christmas! Only 4 more 'get ups' before we can open the first window on the advent calendar!


text:
namespace bart {

namespace system {

namespace moments {

SphericalHarmonic::SphericalHarmonic(const int total_groups,
                                     const int max_harmonic_l)
    : total_groups_(total_groups),
      max_harmonic_l_(max_harmonic_l) {

  AssertThrow(total_groups > 0,
              dealii::ExcMessage("Error system::moments::SphericalHarmonic "
                                 "constructor, total_groups must be > 0"));
  AssertThrow(max_harmonic_l >= 0 ,
              dealii::ExcMessage("Error system::moments::SphericalHarmonic "
                                 "constructor, l_max must be >= 0"));

  for (int group = 0; group < total_groups; ++group) {
    for (int l = 0; l <= max_harmonic_l; ++l) {
      for (int m = -l; m <= l; ++m) {
        moments_.insert(
            std::make_pair<MomentIndex, MomentVector>({group, l, m}, {}));
      }
    }
  }
}
MomentsMap::const_iterator SphericalHarmonic::cbegin() const {
  return moments_.cbegin();
}
MomentsMap::iterator SphericalHarmonic::begin() {
  return moments_.begin();
}
MomentsMap::const_iterator SphericalHarmonic::cend() const {
  return moments_.cend();
}
MomentsMap::iterator SphericalHarmonic::end() {
  return moments_.end();
}
} // namespace moments

} // namespace system

} // namespace bart

redpajama_set_name: RedPajamaGithub


A rare "love song" from Anna Tivel. Dust & Magic is a song about loving someone, learning their colors, their depths. It's about trying to see someone honestly, even when they're hard to see. It's about knowing and hoping the person you love can find their way back from a darker place, believing in the raw power of their beautiful spirit.


With over 400 million active users, Facebook has become a prime spot for brands to advertise and promote themselves. Over 1.4 million local businesses have a presence on Facebook, reaching out to consumers through pages, events and applications.
Diesel Spain is taking social marketing to the next level, taking Facebook off the Web and putting it in their dressing rooms by introducing Diesel Cam, an interactive installation that allows shoppers to photograph themselves and post the pictures immediately to their Facebook profiles. Shoppers can photograph themselves as they try on clothes and ask their Facebook friends for advice about which garments they should buy, or they can publish photos to show off all the new clothes they are buying.
Shoppers connect to Facebook via Facebook Connect, using a touch screen monitor on the installation. They then have the opportunity to photograph themselves in Diesel clothes and publish their photos immediately to Facebook. The photos come complete with a Diesel logo in the corner, so it's great advertising for the company, not to mention tons of fun for shoppers.
Facebook Connect has given third party websites the opportunity to connect with users through Facebook, but Diesel is taking it to a new level. The Diesel Cam gives live users the opportunity to enter social media directly from their stores. What a great idea, allowing all your friends to help you to decide on what looks good on you.


text:
Yataqoba är en ort i Azerbajdzjan.   Den ligger i distriktet Xaçmaz Rayonu, i den nordöstra delen av landet,  km nordväst om huvudstaden Baku. Yataqoba ligger  meter över havet och antalet invånare är .

Terrängen runt Yataqoba är platt. Den högsta punkten i närheten är Zindanmuruqqışlaq,  meter över havet,  km sydväst om Yataqoba. Närmaste större samhälle är Xaçmaz,  km söder om Yataqoba.

Trakten runt Yataqoba består till största delen av jordbruksmark. Runt Yataqoba är det ganska tätbefolkat, med  invånare per kvadratkilometer.  Klimatet i området är tempererat. Årsmedeltemperaturen i trakten är  °C. Den varmaste månaden är augusti, då medeltemperaturen är  °C, och den kallaste är januari, med  °C. Genomsnittlig årsnederbörd är  millimeter. Den regnigaste månaden är september, med i genomsnitt  mm nederbörd, och den torraste är augusti, med  mm nederbörd.

Kommentarer

Källor

Orter i Xaçmaz Rayonu

redpajama_set_name: RedPajamaWikipedia


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text:
Home › Audiobooks › Beautiful Boy
Beautiful Boy Audiobook
By: David Sheff
Length: 11 hours and 29 minutes
(76679 ratings)
Narrated by: Anthony Heald
Beautiful Boy Audiobook Summary
What had happened to my beautiful boy? To our family? What did I do wrong? Those are the wrenching questions that haunted every moment of David Sheff's journey through his son's drug addiction. David's story is a first: a teenager's addiction from the parent's point of view—a real-time chronicle of the shocking descent into substance abuse and the gradual emergence into hope.
Before meth, Sheff's son, Nic, was a varsity athlete, honor student, and award-winning journalist. After meth, he was a trembling wraith who lied, stole money from his eight-year-old brother, and lived on the streets. With poignant candor, Sheff traces the first warning signs—denial, 3 a.m. phone calls—the attempts at rehabilitation, and, at last, the way past addiction. He shows us that, whatever an addict's fate, the rest of the family must care for one another too, lest they become addicted to addiction.
Beautiful Boy is a fiercely candid memoir that brings immediacy to the emotional rollercoaster of loving a child who seems beyond help.
Beautiful Boy Audiobook Narrator
Anthony Heald is the narrator of Beautiful Boy audiobook that was written by David Sheff
Anthony Heald, an Audie Award–winning narrator, has earned Tony nominations and an Obie Award for his theater work; appeared in television's Law & Order, The X-Files, Miami Vice, and Boston Public; and starred as Dr. Frederick Chilton in the 1991 Oscar-winning film The Silence of the Lambs. Heald has also won numerous AudioFile Earphones Awards. He lives in Ashland, Oregon, with his family.
About the Author(s) of Beautiful Boy
David Sheff is the author of Beautiful Boy
David Sheff's work has appeared in the New York Times, Rolling Stone, Wired, and Fortune. As a contributing editor to Playboy, he has interviewed John Lennon, the founders of Google, Gore Vidal, and others.
Beautiful Boy Full Details
Narrator Anthony Heald
Length 11 hours and 29 minutes
Author David Sheff
According to Blackstone Publishing, the Publisher of Beautiful Boy Audiobook, Beautiful Boy includes the following subjects: memoir, addiction, father, son, family, relationships, parents, parent, substance abuse, drugs, alcohol, meth, rehab, hope, new york times bestseller, movie, movie tie-in The BISAC Subject Code is
Tearing Us Apart Ryan T. Anderson The political philosopher Ryan T. Anderson, bestselling author of When Harry Became Sally: Responding to the Transgender Moment, teams up with the pro-life journalist Alexandra DeSanctis to expose the catastrophic failure—social, political, legal, and personal—of legalized abortion. Hope in the Ruins of Roe With the Supreme Court poised to return abortion law to the democratic process, a ... Read more about Tearing Us Apart
Better Than We Found It Frederick Joseph From the New York Times best-selling author of The Black Friend and a seasoned activist comes an indispensable guide to social and political progressivism for young people and anyone wanting to get more involved. Every generation inherits the problems created by the ones before them, but no generation will inherit as many problems—as many crises—as the current generation of young people. From ... Read more about Better Than We Found It
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text:
WFF NEEDS ASSESSMENT
WFF CAL SURVEY
CYBERSECURITY SERVICE MANAGEMENT
CYBERSECURITY TRAINING & AWARENESS
NASA CALIBRATION LABORATORY
CYBERSECURITY SERVICE MANAGEMENTcharleshollman12022-12-23T09:46:42-06:00
Our Agency's Cybersecurity Service Line & Management
Our customers and end users are vital to the success of the Cybersecurity Service Line, which is why we strive to ensure the delivery of cybersecurity services is efficient and effective across the Agency. Our goal is to provide superior customer service to maximize the value of cybersecurity services offered, optimize service delivery, and improve overall customer experiences. Our team uses a holistic, strategic approach in pursuit of excellence in IT cybersecurity service management. Our team uses service management best practices, including Information Technology Infrastructure Library (ITIL), Lean Six Sigma (LSS), Project Management Institute (PMI), and Agile to provide consistent, efficient, and effective service management delivery
ITIL Service Management Processes
Strategy & Business Management
Strategic Planning & Road Mapping
Demand Management Services
Service Portfolio Management
Service Catalog Management
Service Now Development
Service Request Fulfillment
Metrics & Reporting
ITSM Incident Management
Our Strategy and Business Management team works to ensure the delivery of IT services is efficient and effective to meet our customer's needs, service requirements, and desired outcomes. We leverage a Voice of the Customer (VoC) initiative for the Business Relationship Manager to host customer engagement workshops, conduct surveys, and participate in outreach activities to stay connected with our customers, stakeholders, and end users. The feedback and insights collected through this process allow us to improve the cybersecurity services that are currently offered and provide opportunities for the development of new service offerings that meet end-user demands.
Our Project Management team works with the Agency Enterprise Project Management Office (EPMO) and provides consultation and guidance on NASA's process for applicable IT projects, project methodologies that support project planning, and strategic roadmap alignment. Our team strives to provide quality service delivery and works within the given constraints of scope, time, and budget while driving change and delivering value.
Our Operations Management team is responsible for the day-to-day operations of NASA's cybersecurity services across the Cybersecurity Service Line. Our goal is to ensure seamless performance and delivery of services while maximizing efficiencies, increasing stability, improving the quality of services offered, and aiding in the detection and resolution of service disruptions. Our team uses best practices and service operational principles to help govern the transition of services, ensuring they are correctly prioritized, accessed, and implemented in a timely manner.
When a problem is identified within a cybersecurity service, a Problem Management Review is scheduled so that the Problem Management team can assist the problem owner identified within the cybersecurity service management office or Service Portfolio. Our Problem Management team uses standardized methods and procedures to review, categorize, analyze, and report problems in an effective and timely manner. To help determine the cause of a problem, our team uses a Root Cause Analysis Toolkit and a Service Now ticketing system to track and document important details. A Problem Review Board meeting is held to authorize and review problems within the Cybersecurity Services organization that will manage improvement opportunities and reduce the number of problems and incidents that occur going forward.
Our Strategic Planning and Road Mapping team ensures services within the Cybersecurity Service Line and their plans remain aligned with the overall organizational strategy, including changes made to the strategy, roadmaps, and portfolios. Our team has implemented a Portfolio Item Change Request process that allows users to submit a change to an existing Portfolio Item that is documented through a request workflow. Our team manages the governance of the portfolio changes and communicates with Leadership teams, the portfolio status on a regular cadence to keep them informed and stay abreast on important actions, projects, and initiatives.
The Risk Management discipline is an integrated process throughout the entire CyS Service Line. Our CyS Risk Management team ensures that potential shortfalls are identified while making sure that explicitly established and stated objectives are still achieved. Determination of the probability of such occurrences and the magnitude of the potential consequences enable risk-informed decisions, proactive task planning, and resource allocation decisions designed to ensure the success of the CyS Service Line. Risks are identified, discussed, and escalated to the appropriate Risk Reviews and are managed at the level providing budget and resources.
We are continuously maturing our service management processes to ensure we provide consistent, efficient, and effective methodologies when adding new services to our Cybersecurity Portfolio. Our Enterprise Service team develops and delivers (assesses and implements) new enterprise services that meet customer and stakeholder cybersecurity needs across the Agency that are aligned to the Cybersecurity Service Line. We engage with customers, stakeholders, and technical subject matter experts to deliver cybersecurity services driven by customer needs, meet cybersecurity requirements, and add value to NASA centers and missions, while continuously providing optimized service delivery.
Our Demand Management team ensures that there are standardized workflows, methods, and procedures to vet new demands that will ensure proper analysis prior to project initiation. Our team uses various tools and techniques to forecast, plan, and manage the demand for products and services to ensure alignment with our customer's strategic direction. We offer assistance, guidance, and consultation to help streamline the workflow processes.
The CyS Service Level Management team maintains and approves IT service quality through a constant cycle of agreeing, monitoring, and reporting upon IT achievements and actions. Our team helps standardize processes between the cybersecurity services offered and the service providers who serve them. We work one-on-one with the service portfolios to establish Service Level Agreements and Operational Level Agreements which would guarantee that specific and measurable targets are met and that both parties have clear expectations of the level of services that will be delivered.
Our CyS Service Portfolio Management team evaluates and prioritizes incoming products or service proposals and change requests based on resource constraints, existing commitments, the organization's strategy and objectives, and other criteria. Our CyS Portfolio provides a comprehensive listing of cybersecurity services that are in the production service pipeline, located within the Cybersecurity Catalog, or retired. Our CyS Service Line currently consists of three portfolios: Cybersecurity Infrastructure, Security Operations, and Cyber Protection.
The Cybersecurity Service Catalog is available on the Enterprise Service Desk website at NASA and can be accessed by all NASA users. The catalog provides a standardized listing of the cybersecurity offerings available, making it a quick and easy resource guide for users to order the cybersecurity services they need in one centralized location. Our CyS Service Catalog Management team continually matures and expands the service request offerings in the Cybersecurity Catalog to ensure the services offered are meeting the NASA Agency cyber needs.
Our Service Now Development team is responsible for gathering the requirements, writing the user stories, and managing the functional testing of those developed stories in the configuration, development, and maintenance of the CyS Service Portal and Cybersecurity Catalog, located on our customer's Enterprise Service Desk Service Portal. Our goal is to make it easy for users to access and order cybersecurity services across the organization while delivering optimal cybersecurity services that are customized to meet our customer's and end-user needs.
The Service Request Fulfillment process manages the entire lifecycle of a user request for information, advice, a standard change, or access to an IT service. Our CyS Service Now Development team was instrumental in developing a Cybersecurity Catalog, located on the Enterprise Service Desk website at NASA, where NASA users can go to order cybersecurity services or provide feedback on improving the productivity and quality of the services offered – all within one location.
The Knowledge Management team ensures that the help desk staff, our service customers, and end users have accurate and sufficient cybersecurity knowledge and resources available to them 24/7/365. Our team is responsible for establishing and maintaining standardized workflows and processes that continually improve the quality of information available. The team works with each Service Portfolio area to ensure that all published cybersecurity services knowledge articles are included within our customer's Knowledge Base library and that users and help desk staff have easy access to this information.
Metrics and Reporting
The Metrics and Reporting team provides consultation, guidance, and support on performance data metrics and reporting for the Cybersecurity Service Line. We use standardized performance metrics and reporting tools to generate operational reports, dashboards, and scorecards that measure the alignment of cybersecurity service offerings and service management functions within the Cybersecurity Service Line.
Our Continual Improvement team follows the ITIL best practice for continual improvement (Plan, Do, Check, Act) to ensure that our cybersecurity services are meeting the demands of our customers and end users. We use an automated process for users to submit suggestions or ideas to strengthen the effectiveness and efficiency of IT processes and cybersecurity services across NASA. We leverage existing or cross-integration of services to determine improvement solutions that align services to meet customer needs and use tools to help evaluate, analyze, and document cybersecurity maturity levels to continually refine and improve processes for service enhancement, waste reduction, and variation.
Our Change Management team ensures that there are standardized methods and procedures in place to record, control, and coordinate cybersecurity-related changes. Our team is responsible for managing and processing cybersecurity-related change requests and assisting submitters throughout the entire workflow process, which includes a two-phased governance process needed to obtain the necessary authorization to proceed with the implementation of a change. Our goal is to provide users with a better overall user experience. Our team is continuously improving our processes by providing customers with streamlined workflow activities and automation, improved online forms, and real-time dashboard reporting.
When an incident occurs, our ITSM Incident Management team executes the ITIL best practice of Detecting, Recording, Classifying, and Resolving to restore normal service operations as quickly as possible to minimize the impact on business operations and ensure that the best possible levels of service quality and availability are maintained. Service Level Agreements) are in place with each of our customer's Service Portfolio areas that document the parameters for 'normal service operations' and provide guidelines to resume full operational performance when an incident occurs.
It is important that the communications received by customers, stakeholders, and end users are consistent, efficient, and effective. Our Communications team manages and outlines all communication channels and processes, and enforces official communication standards, policies, guidelines, and best practices. We provide guidance and support to our customers to be able to send official communications through their appropriate channels. As authorized senders, our team is responsible for releasing agency-wide cybersecurity-related notifications, including Emergency Communications that alert users of a service outage or security vulnerability. Our team also provides processes and guidance on official written documentation, logos, graphics, and digital email signatures. We work closely with the Agency Office of Communications to stay abreast of the latest communication policies and guidelines to make certain that our processes and standards remain aligned.
Find Out More About Rothe Enterprises, Inc
Rothe Enterprises, Inc. (REI) was founded in 2000 as a Women-Owned and HUBZone-certified Small Business. REI provides unique services such as calibration & metrology, engineering & technical services, and cybersecurity to the National Aeronautics and Space Administration (NASA). Headquartered in San Antonio, TX, REI has offices in Houston, TX, and workforces that span several NASA centers such as Johnson Space Center (TX), Glenn Research Center (OH), Marshall Space Flight Center (AL), and Kennedy Space Center (FL), and Wallops Flight Facility (VA).
Rothe Enterprises, Inc. (HQ)
4535 E. Houston St.
San Antonio, TX. 78220
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text:
Q: Why is vdirsyncer choking? I had vdirsyncer running just fine for eons, on a computer that I don't use every day (or even every week). And then about a month ago I tried to run vdirsyncer sync and it choked with:
Could not find platform independent libraries <prefix>
Could not find platform dependent libraries <exec_prefix>
Consider setting $PYTHONHOME to <prefix>[:<exec_prefix>]
Fatal Python error: Py_Initialize: Unable to get the locale encoding
ImportError: No module named 'encodings'

Current thread 0x00007f9038c2b740 (most recent call first):
Aborted (core dumped)

I don't remember screwing around in my python settings so I don't know why it would have just stopped working. I suspect that someone who spends more time in Python than I do these days might know what happened?
I'd like to fix this without trashing python, which currently defaults to 2.7 unless I specify that I want python3.
I found a question that cites the same error text, but the answer there recommends unsetting $PYTHONHOME and I haven't set it:
Fatal Python error: Py_Initialize: Unable to get the locale encoding … SyntaxError: invalid syntax Aborted (core dumped)
Answers to questions in the comments:

*

*I'm on Ubuntu 18.04

*I don't remember how I initially installed but I reinstalled with apt which didn't solve anything.

A: I reinstalled with pip3 install --user --ignore-installed vdirsyncer which seems to have gotten it working again. Still not sure how I broke it.

redpajama_set_name: RedPajamaStackExchange


Celltrion, Inc. (KRX:068270) is set to launch global Phase 3 clinical trial for its bevacizumab biosimilar 'CT-P16' for the treatment of cancer.
Celltrion successfully completed Phase 1 clinical study on the safety and pharmacokinetic assessment of CT-P16 in June 2018. Further, it recently submitted its Clinical Trial Application for Phase 3 clinical study to the National Authority of Medicines and Health Products, I.P. (Infarmed) of Portugal.
Beginning with Portugal, Celltrion is set to conduct Phase 3 clinical trial for CT-P16 in about 150 sites in some 20 nations across Europe, Asia and South America.
Meanwhile, Roche's Avastin®, the originator of CT-P16, is a therapeutic anti-cancer drug for the treatment of metastatic colorectal cancer, metastatic breast cancer, non-small cell lung cancer, and glioblastoma. It last year recorded global sales of about USD 6.7 billion1, making it blockbuster medicine.
"We are successfully conducting the clinical trial for the bevacizumab biosimilar 'CT-P16' as planned. We will secure the competitiveness for CT-P16 compared with its competitive biosimilars." says an official of Celltrion.