Disequilibrium syndrome is linked to: Rapid changes in the osmotic gradient between the pla sma and the brain. This is especially so with rapid dialysis or in people with severe uremi a. Removal of urea during dialysis occurs slower across the blood \200\223brain barrier tha n from plasma. This generates a \200\230reverse osmotic gradient \200\231 causing CHAPTER 11.14 Neuropsychiatric disorders in chronic kidney disease 457 water movement into the brain leading to cerebral edema. A decrease in cerebral intracellul ar pH by mechanisms that are not completely understood has been proposed as an alternative hypothesis: intracellular acidosis is associated with an increase in unmeasured organic aci ds which increase cerebral osmolality thus leading to cerebral edema. Management is mainly focussed on prevention. Frequent dialysis (every 1 \200\2232 days) of short duration (2 h) using a smaller dialyser will facilitate slow removal of urea. patient starts dialysis) may occur. Clinical manifestations are not speci \201 c and can \202 uctuate. Cognitive impairment can be present. Wernicke \200\231s encephalopathy resul ting from thiamine de \201 - ciency (which may be made worse when the patient starts dialy sis due to thiamine egress during dialysis) can occur. An underlying genetic predisposition , chronic malnutrition with anorexia, and the administration of glucose containing IV \202 uids are contributory factors. The classic triad (oph- thalmoplegia, ataxia and confus ion) is rarely present. Treatment is by thiamine replacement. Early treatment prevents prog ression to Korsakoff \200\231s psychosis which is not amenable to thiamine replacement. Sle ep disorders. The commonest sleep problems reported with CKD are insomnia, restless leg syn drome and exces- sive daytime sleepiness. Other reported syndromes include obstructive slee p apnea syndrome, sleepwalking and nar- colepsy. Uremia is postulated as the common denomin ator and commencement of dialysis may improve symptoms but rarely results in complete resol ution. Dialysis dementia Dialysis dementia, also known as dialysis encephalopathy, is a progressiv e, often diffuse encephalopathy which occurs in some patients on chronic HD. Prevalence is estimated to be 1%. It is equally prevalent among the sexes and different age groups. Comm unication dif \201 culties are often the presenting fea- ture with dysarthria, dysphasia a nd slurred, stuttering speech. This may initially be present only during or after dialysis. Other cognitive signs such as dyslexia, dyspraxia and dys- calculia may be seen. Impairmen t of memory, depression and paranoid ideation may occur. Progression to myoclonus, asterixi s, seizures and personal- ity change may be seen. In the later stages the patient may becom e mute. Death usually occurs within 15 months. EEG abnormalities of bilateral spike and wav e complexes and frontal slow waves are seen, usually about 6 months prior to the onset of s ymptoms. There is a marked slowing of background rhythm. Neuroimaging has not been shown to be diagnostically helpful. Histopathological changes at postmortem are nonspeci \201 c. H istochemically there is significant elevation of brain aluminum content especially in those cases that are geographically clustered. Cerebrospinal \202 uid aluminum concentrations are of no diagnostic bene \201 t, the disorder occurring at widely varying concentrations. Management must focus on prevention by use of alumin- ium-free dialyzates, puri \201 cati on of water, and avoiding die- tary intake of aluminum. Benzodiazepines such as clonazepam and diazepam are effective in controlling myoclonus. Desferroxamine can be used to chelate aluminum and is used in the treatment of established cases. The chelated complex is removed by dialysis. Improvement is slow and treatment may need to be continued for a year. Chelat ion can lead to an initial deterioration that may be progressive and occasionally fatal. Sp oradic dementia not related to aluminum excess is not amenable to chelation treatment. Psychotropic drug use in the patient with failing renal function The common drugs and the i mplications for prescribing in patients with CKD and ESRD are reviewed in Table 11.14.1. Issues of capacity and consent The law (in England and Wales) assumes that all adults have the capacity to make decisions unless otherwise proven. Different laws may apply in other c ountries and the follow- ing are only general principles. Dif \201 culties and ethical dil emmas arise when issues regard- ing extent of capacity arise. Some important concepts to no te are: \200 \200 Capacity is speci \201 c to the planned treatment. Different suggested treatments may requ ire different levels of capacity. Generally more invasive treatments require a greater unde rstanding of risks and bene \201 ts. Capacity may be present to consent for one treatment but not for another even at the same time. Informed consent is the heart of clinical ethics and it is the duty of the treating physici an to satisfy the criteria required and to make an assessment of capacity. The principles o f assessment of capacity can be summa- rized in the following questions: \200 Does my patient understand the information provided regarding the treatment in question? Do es my patient believe the information provided? Does my patient understand the pros and con s of having this treatment versus any other treatment or even no treatment at all? Can my p atient retain the information provided long enough for him/her to make a decision? Can my p atient communicate (not necessarily by verbal means) the decision made? \200 If the answer to any of the above questions is \200\230No \200\231, it usu- ally signi \201 es a lack of capacity. Common law has supported the principles of Best Interests, Nece ssity and Duty of Care in managing patients without capacity. Other disorders Subdural hematomas, either due to an intrinsic coagulopa- thy in uremia (or due to administration of heparin when the T. FRIEDMAN AND P. RAO 458 The above principles hold true for any individual aged >18 years. For those aged <18 years, the law (in England and Wales) is different. Some important principles are: Young people ( <18 years) CAN CONSENT to treatment if they satisfy the requirements of capacity assessment . However, in practice an adult with parental responsibility is usually involved in the dec ision-making process. Young people (<18 years) CANNOT REFUSE to have any treatment if an ad ult with parental responsibility consents, even if the young person ful \201 lls the crite ria for capacity assessment. The decision of the adult with parental respon- sibility usual ly overrides any decision made by the young person. If in doubt, it is appropriate to consu lt with per- sons with experience in the legal aspects as they apply to young persons. If t he patient is deemed not to have capacity, The Mental Capacity Act (2005) (in England and W ales) provides a legal framework for decision-making. The Act also allows adults with capac ity to make decisions in the event of future incapacity in some areas (including refusal of speci \201 c treatments). When a patient is unable to make an autonomous choice, the heal th professional must act in the best interests of the patient. Second opinions are usually appropriate. The Act lays down some principles for acting in the patient \200\231s best interests: \200 To ensure that if the decision can be clinically delayed in the likely event of regaining c apacity, it is. To encourage and optimize the participation of the patient as far as is pos sible. To thoroughly investigate any past or present wishes or values of the patient or any other relevant factors. To consider the views of other relevant people who may be carers o r people interested in their welfare. The Court of Protection is the \201 nal arbiter (in England and Wales) in case of dispute . Finally, it is important to realize that people have the right to make unwise decisions a nd this does not necessarily indicate lack of capacity. In complex cases it would be rea- s onable to ask for expert advice. Further reading Brown T, Brown R. Neuropsychiatric consequences of renal failure. Psychosomatics 1995; 36: 244 \200\223253. Cohen LM, Tessier EG, Germain MJ, Levy NB. Update on psycho- tropic medication in renal dis ease. Psychosomatics 2004; 45: 34 \200\22346. Phipps A, Turkington D. Psychiatry in the renal unit. Adv Psychiat Treatment 2001; 7: 426 \200\223432. Table 11.14.1 Common drugs used for the treatment of neuropsychiatric disorders with speci \201 c consideration of changes in CKD and ESRD Drug group Notes on prescribing in renal impairment <1% of haloperidol is excreted by the kidneys (recommended dose in ESRD is 1 \200\2232 mg b d or tds). Antipsychotics <1% of aripiprazole is excreted by the kidneys but there are reports of drug interactions w ith other drugs; not much is known in terms of safety in renal impairment. No dose reductio n is recommended in ESRD. No dose adjustments recommended for olanzapine or quetiapine by t heir respective manufacturers. Clozapine excreted in only trace amounts in urine but the anticholinergic side-effects, esp ecially urinary retention, may be a problem. Sulpiride and amisulpiride are excreted by the kidneys and risperidone clearance is reduced in ESRD. Lithium is a common source of concern. Avoid altogether if possible. If not, reduce dose. Antimanic agents It can be removed by dialysis, hence best to administer as a single, reduced dose post dial ysis. Contraindicated in severe renal impairment. Other mood stabilizers such as sodium valproate, carbamazepine and lamotrigine are not sign i \201 cantly excreted in urine. There is a risk of increased free levels of valproate in ESRD, hence the need to monitor \200\230free \200\231 drug levels in severe renal impairme nt. Safest principle is to \200\230start low and go slow \200\231. SSRIs are generally drugs of \201 rst choice. Antidepressants If using paroxetine, reduce dose. No change recommended with other SSRIs. Tricyclic antidepressants are not signi \201 cantly excreted by the kidneys; however, thei r use is to be balanced with potential for adverse effects (especially anticholinergic) and risk of mortality in overdose. Nefazodone is not excreted by the kidneys but risk of hepatotoxicity is high, hence use wit h caution. Benzodiazepines are primarily excreted via the liver. Anxiolytics Adjust dose only in severe renal impairment and monitor for excessive sedation. Zolpidem and zopiclone may be used but it is prudent to start with a low dose. CHAPTER 11.14 Neuropsychiatric disorders in chronic kidney disease CHAPTER 11 Chronic kidne y disease 459 Internet resources National Institute of Mental Health: British Association for Behavioral and Cognitive Psychotherapies (BABCP): http://www.babcp.org.uk http://www.nimh.nih.gov National Phobics Society: See also Psychological aspects of treatment for renal failure, p. 538 B2M amyloidosis, p. 4 36 Drug-induced nephropathies, p. 698 http://www.phobics-society.org.uk Royal College of Psychiatrists: http://www.rcpsych.ac.uk/mentalhealthinforma- tion.aspx This page intentionally left blank 461 Chapter 12 Special problems in chronic kidney disease Chapter contents Chronic kidney disease in children 462 Chronic kidney disease in the elderly 468 Chronic ki dney disease in diabetic patients 472 Chronic kidney disease in pregnancy 476 A. WATSON 462 Chronic kidney disease in children \200 Introduction Children need to grow and develop into adults. Chronic kidney disease (CKD) ca n have a major impact upon physi- cal growth as well as psychological, social and scholasti c development. Autosomal recessive polycystic kidney disease Oxalosis Glomerulonephritis (10%) \200 \200 Focal and segmental glomerulosclerosis Other chronic glomerulonephritides Organization of facilities It is appropriate to treat and follow such children in spe- cial ized units which provide access to multidisciplinary teams including: \200 \200 \200 \200 \200 \200 \200 \200 \200 Multisystem disorders (6%) \200 \200 \200 Hemolytic uremic syndrome Henoch \200\223Sch nlein purpura Lupus erythematosus pediatric nephrologists; specialist nurses; pediatric urologists; dieticians; social worker s; psychologists; play specialists; hospital teaching staff; youth workers. Miscellaneous (9%) \200 \200 \200 Renal vascular disease Kidney tumor (usually bilateral Wilms \200\231) Denys \200\223Drash syndrome Cause unknown \200 3% Clinical presentation Children are often asymptomatic until CKD is very advanced. There may be nonspeci \201 c symptoms including: \200 \200 \200 \200 Pediatric units are likely to use the transplantation exper- tise of local adult units. Ped iatric and adult care is a continuum and young people with CKD should be prepared for trans ition to adult units. This should take place when they are mature and have completed growth and development. The same categories of chronic kidney disease (CKD 1 \200\2235) are used for children as with adults. Dialysis should be seen only as a temporary option in chil- dr en before transplantation becomes available. A good functioning transplant offers the best opportunity for growth and long-term rehabilitation. lethargy; tiredness after school and poor concentration; poor appetite; urinary symptoms in cluding polyuria or enuresis (bed- wetting). The use of antenatal USS has enabled the detection of renal tract malformations before symp toms develop or superimposed problems occur such as urinary tract sepsis with posterior ure thral valves. Modes of presentation include: \200 incidental on antenatal USS; \200 uri nary tract infection with underlying dysplasia and/or re \202 ux nephropathy; polyuria and enuresis (renal concentrating defect with nephronophthisis); failure to thrive and short s tature related to poor nutritional intake or tubulopathy; bone abnormalities or rickets due to tubulopathy (cystinosis or CKD); hematuria or proteinuria; hypertension and congestive cardiac failure; failure to recover from AKI; seizures and hypertensive encephalopathy; inv estigation of a child with lethargy or pallor; investigation of siblings with familial cond ition; investigation of child with syndrome known to have an associated renal abnormality. Epidemiology Incidence of CKD 5 is low compared to adults with an annual take on rate for d ialysis of 2 \200\2233 pmp (8 pmp aged <15 years). Prevalence of mild \200\223severe CKD ( stages 2 \200\2235) is 35 \200\22340 pmp. Prevalence of CKD 5 in South Asian children is 3 \227 that of Caucasian population due to high incidence of inherited disease. Prevalence o f CKD is higher in male patients (ratio of 1.54:1) due to predominance of renal dysplasia a nd obstructive uropathy in males. \200 \200 \200 \200 \200 \200 Etiology The majority of patients in childhood have an identi \201 able cause for CKD. Congenital abnormalities (55%) \200 Renal dysplasia/hypoplasia \200 Obstructive uropath ies \200 Re \202 ux nephropathy Investigations General investigations Urine \200 \200 Hereditary conditions (17%) \200 \200 \200 \200 Urinalysis (pH, blood, protein, glucose, nitrites, leukocytes); Urine culture if urinalysis positive, especially if any symp- toms; Proteinuria quanti \201 ed by early morning urine protein: creatinine ratio (PCR, normal <20 mg of protein/mmol of creatinine). Nephronophthisis Alport \200\231s syndrome Congenital nephrotic syndrome Cystinosis CHAPTER 12.1 Chronic kidney disease in children 463 Blood \200 For the child born with major urinary tract abnormalities the testing time is whether the c hild has suf \201 cient renal reserve to grow adequately in the \201 rst 2 years of life . Renal function often declines slowly in mid-childhood. The greatest challenge is whether the child has suf \201 cient renal reserve to get through the pubertal growth spurt. The d iagnosis of CKD in a child in any family can cause great anxiety, especially with the uncer tainty of deteriora- tion. Information and support need to be available to the family throu ghout the childhood period and are cornerstones of good practice. Full blood count, ferritin and transferrin saturation (TSAT) Urea and electrolytes, creatin ine, bicarbonate, calcium, phosphate, alkaline phosphatase and albumin Parathyroid hormone (PTH) Estimated GFR (Schwartz formula should be used for children between 2 and 14 years of age [mL/min/1.73 m2 = 40 \227 height cm/creatinine mol/l]) \200 Radiology \200 Renal tract USS \200\223 invaluable to have expert pediatric radiology advice as USS is in formative and noninvasive. Size of kidneys related to patient \200\231s height to de \201 ne hypo- plasia/dysplasia. Hand and wrist X-ray for bone age and signs of renal osteodystro phy. Prevention of progression of CKD Monitoring of all children with CKD should include meas- u rement of an early morning urine for PCR or albumin: creatinine ratio (ACR). Urine taken at a morning clinic is acceptable but beware orthostatic proteinuria, especially in adolescen ts. There is currently no agreement at what level of proteinu- ria treatment should be star ted (normal PCR <20 mg/ mmol). For increasing proteinuria an ACEI is \201 rst choice (usu ally enalapril) starting at 2.5 mg and gradually increasing to maximally tolerated dose. Mo nitor blood pressure and plasma potassium and creati- nine at clinic visits. Aim to maximal ly reduce proteinuria but without hypoten- sion or hyperkalemia. Concordance remains proble matical in adolescents. Teenage girls need to be informed of the risk from ACEIs of fetal m alformations in pregnancy. Evidence is emerging that children with CKD due to con- genital abnormalities of the urinary tract (the major group) do not respond to ACEIs as well as tho se with chronic glomerulonephritis. There is very limited experience of adding ARBs (e.g. l osa- rtan) to ACEI treatment and there are no recommenda- tions for this in children. Cardiac review \200 ECG/echocardiography: for signs of left ventricular hypertrophy, especially if hypertensive . Special investigations The choice will be guided by history (including detailed family hist ory), examination and general investigations especially urinalysis and USS. GFR can be form ally measured by [51Cr]EDTA method or inulin or iohexol clearance. Further radiological inv estigations are best discussed at nephrouroradiology conferences. Further imaging could inc lude micturating cystourethrography, radionuclide imag- ing (DMSA or MAG3), CT or MRI studi es. Children presenting with nephritic or nephrotic syndrome require complement levels, aut oantibody screen and ANCA levels ( \200\230nephritic screen \200\231). Children with urinar y calculi should be investigated with a \200\230stone-former protocol \200\231 looking for conditions such as hyperoxaluria and disorders of purine metabolism. Suspected cystinosis can be con \201 rmed by white cell cys- tine levels and slit lamp examination for corneal crystals. Liaison with the genetics department is important as a large number of hereditary /familial conditions can now be recognized by mutations: \200 \200 \200 \200 \200 \200 Nutrition The involvement of a renal dietician trained in pediatrics is essential. In mild renal failure healthy eating diet advice should be provided including advice on \200\230no added salt \200\231 (children love salty snack foods!). In CKD 3 \200\2235, phosphate rest riction may be required. Low protein diets are not advocated. Patients with CKD 4 and 5 req uire regular dietary assess- ments with comparisons to Reference Nutrient Intakes (RNIs) to optimize nutritional status. Supplements may be required to achieve RNI. Dialysis patients often require nasogastric or gastrostomy feeds. The required protein intakes depend upon s tage of CKD and the age of the patient. Vitamin supplements including B-complex, iron, zinc and copper should be given to dialysis patients and CKD 4 patients after assessment. Vitam in A needs to be avoided and a suitable vitamin and micronutrient is Pediatric Renal Dialyv it. oxalosis; congenital nephrotic syndrome; cystinosis; Denys \200\223Drash syndrome; nephrono phthisis; Alports. Renal biopsy is generally undertaken when chronic glomer- ulonephritis is suspected, the et iology of CKD unexplained and genetic testing not possible. Management Patients with CKD 1 and 2 can be seen infrequently (12- or 6-monthly respectivel y) and can attend shared care regional clinics to reduce travel time to the nephrology cent er. Disturbances in phosphate metabolism and rises in PTH have been documented in CKD 3 pat ients and so dietary evaluation should be undertaken. CKD 4 and 5 patients require more fre quent monitoring with access to the center \200\231s multidisciplinary team. Treatment has to be individualized for each child. A. WATSON 464 In renin-dependent hypertension use ACEI (captopril and then enalapril). Cautionary use in teenage girls who may become pregnant. Many drugs have not been fully evaluated in children but should be prescribed as advised in the British National Formulary for Children. Fluid and electrolyte balance Avoid potassium-containing foods, e.g. certain fruits, choc- olate. If hypertensive and \202 uid overloaded, sodium restriction and diuretics are requ ired. In children with congenital abnormalities of the urinary tract and polyuria, salt was ting may be prominent and sodium supplements may be required (also in infants also on perit oneal dialysis). Infection When children have congenital abnormalities of the uri- nary tract recurrent infe ction is common. Parents/carers should be properly informed about obtain- ing appropriate u rine samples (clean-catch urine preferable to bag urine) in primary care. Acid base balance Acidosis has an adverse effect on growth and sodium bicar- bonate supplem ents should be prescribed to maintain plasma bicarbonate in normal range. Prophylactic antibiotics Trimethoprim 2 mg/kg or nitrofurantoin 1 mg/kg are gen- erally emp loyed for those with grade 3 \200\2235 vesicoureteric re \202 ux for at least the \201 r st 2 years of life. Renal osteodystrophy This is particularly important in children because the lesions of hype rparathyroidism are superimposed on the growth zone lesions \200\223 hence the old term \200\230renal rickets \200\231. Such bones can deform easily and result in the need for ort hopedic intervention. Treatment primarily involves control of plasma phosphate by dietary i ntake along with phosphate binders which are usually calcium carbonate compounds. Patients should be involved in choice of medication which might improve compliance. Sevelamer is not licensed for children but increasingly used. Vitamin D analogs such as 1 -hydroxycholecal ciferol or 1,25-dihydroxycholecalciferol are prescribed aiming to keep the PTH level within the normal range. Three-monthly monitoring of PTH should be undertaken or monthly in young infants on dialysis. Annual X-ray to assess bone age. Surgical intervention Close liaison is required with pediatric urologists for those with re current infections and consideration given to proce- dures such as establishing bladder dra inage, ureteric reim- plantation or endoscopic correction (STING procedure) or, ultimately, nephrectomy in a kidney with <10% function. Growth Many of the above factors impact upon growth which is a good indicator of adequacy o f CKD treatment. There may also be psychosocial factors affecting the child \200\231s well- being which require careful exploration by a team member, such as a social worker and/or ps ychologist. Height and weight should be measured at each clinic visit and charted on the ap propriate growth charts (with cor- rections made for prematurity in the \201 rst 2 years) . Head circumference should be measured regularly in chil- dren <2 years of age and also pl otted on appropriate cen- tile charts. Growth hormone is licensed for use in children with CKD who fail to grow adequately when all other aspects of man- agement have been optimized. Child qualify for growth hormone if their height standard deviation score (SDS) is below \200\2232 or if their height velocity SDS is below the mean, but only if adequate nutrition and metabolic abnormalities have been corrected. Growth hormone is administered subcutaneo usly on a daily basis. Information on its use is provided by the rele- vant manufacturers. Anemia Assess iron status by hemoglobin, ferritin and TSAT. Oral iron supplements, e.g. Syt ron liquid in younger chil- dren or ferrous sulfate tablets in older children. Poor complia nce is again a problem and clear instructions regarding time of taking tablets is important . IV iron may be required in hemodialysis patients and can safely be administered on an out patient basis to patients on PD and before dialysis. SC erythropoetin to maintain hemoglobi n at target levels of \211 11 g/dL in older children and \211 10 g/dL if aged <2 years. Hypertension De \201 ned by reference to published centile charts for sex and height. Bloo d pressure should be at least <90th centile. Appropriate sized cuff should be used for each child. Automated oscillometric measurements are commonly used but cause discomfort in some children. Check blood pressure with aneroid sphygmomanometer and Doppler device for systol ic pressure in small infants. If doubt about \200\230white-coat hypertension \200\231 then blood pres- sure can be assessed using 24 h ambulatory blood pressure monitoring. Address salt intake which is invariably high in children. Calcium channel blockers such as nifedipi ne (twice a day modi \201 ed release) or once daily slow release preparations are favored as initial treatment. Preparation for dialysis and transplantation Children who have a slowly progressive deterio ration of renal function should be given information about future dialysis and transplant o ptions which can be discussed with the family over a long period. Some patients may present needing urgent chronic dialysis ( \200\230crash-landers \200\231). This invariably occurs when patients present with AKI such as a crescentic glomerulonephritis which then progresse s rapidly to a chronic state. Sometimes CKD is undetected and the child suddenly presents i n teenage years with persistent anorexia and anemia. Such acute patients and their families can have major dif \201 culties adjusting to the \200\230double-hit \200\231 of both acu te and chronic renal failure. Transplantation is the ultimate goal for children with CKD 5 and there has been increasing focus on pre-emptive trans- plantation which is regarded as t he treatment of choice. CHAPTER 12.1 Chronic kidney disease in children 465 Information needs of children and families can be addressed by multimedia methods which sho uld involve the whole family. Home visits by members of the team, including social worker a nd experienced nurse, are very beneficial in assessing family set-ups, facilities for possi ble home dialysis and allowing the family to ask questions in their own home environments. Families may bene \201 t from being linked either to other families with the same conditio n or to a child of similar age or same sex. If children have been seen only in a regional r eferral clinic, investigations that are needed before dialysis or transplan- tation can be co-ordinated on dedicated renal assessment days. Families should also have the opportunity to visit the dialysis unit and meet other members of the multiprofes- sional team. Some chi ldren are very apprehensive about needles (needle phobia) and it is essential that the unit has play therapists who can help relieve anxiety often in discus- sion or supervision with the clinical psychologist. A child may be depressed or have major behavioral distur- bance and may require assessment by a child psychiatrist. A number of children entering the dial ysis/transplant pro- gram have disabilities. If severe, the ethical imperative is to act in the best interests of the child and maximizing con- servative treatment without dialysis i s a treatment option. This will require discussions with a number of agencies and possibly a clinical ethics committee to help formulate the best plan. For many children with congenital or familial conditions causing their CKD, deterioration i n renal function (particu- larly around puberty) can be anticipated and pre-emptive transpl antation considered. Living \200\223related donor transplantation (LRD) is strongly favored , especially with laparoscopic donor nephrectomy. With decreasing numbers of deceased donor offers, living donation is now being extended from the parents to other family members or emotionally related donors. Altruistic donation and paired donor schemes are excep- tional at present. Evaluation for transplantation is similar to that of adult patients with a urol ogy opinion essential for children with suspected abnormal bladders. It is essential to ens ure that the bladder is of adequate capacity, empties adequately and does not generate high pressures before transplantation. Most pediatric centers use the transplantation expertise of local adult units with postoperative medical management being in a pediatric facility. Standard immunosuppressive regimes at present typically consist of: \200 tacrolimus (calcineurin inhibitor of choice based on ran- domized trials and fewer side-eff ects than ciclosporin); prednisolone (minimization or steroid-avoidance regimes are being t rialed); azathioprine. The latter may be substituted by mycophenolate mofetil after rejection episodes and increas ingly mycophenolate is used de novo. Concordance with therapy is a major problem in the lon g term, especially in the adolescent age group. Enquiries about who supervises medications as well as ongoing edu- cation and support from team members such as youth workers may assi st. Excellent patient survival rates (96% at 5 years) with 5 year graft survival rates of 7 8% for deceased donors and 83% for living related donors. Immunizations All children must complete routine childhood vaccinations including: \200 \200 \200 haemophilis in \202 uenzae Type B conjugate (HIB); pneumococcal vaccine; meningococcal gro up C conjugate. Infants with CKD should be considered for vaccination against rotavirus. Children approachi ng dialysis and transplantation require vaccination for: \200 hepatitis B; \200 varicel la; \200 BCG. Peritoneal dialysis therapy Automated peritoneal dialysis (APD) is preferred. It can be per formed overnight in the patient \200\231s home with hopefully minimal impact upon schooling and social life. APD machines are mobile and hence family holidays are possible. Two paren ts or carers should be trained \200\223 there is a very heavy burden of care on mothers wi th infants receiving APD as well as enteral feeds and medications. Home support and respite care need careful considera- tion. Peritonitis is the main risk. The Renal Association sta ndard is one episode in 18 patient-months (3 year average). Adequacy of dialysis should be assessed using both clinical and biochemical parameters. Hepatitis B vaccine can be given at any age at intervals of 0, 1 and 6 months. Check anti-H BS antigen antibodies 2 \200\2233 months after the 3rd dose. BCG may be given to some newbo rns routinely. Over the age of 6 years BCG should only be given if tuber- culin skin test n egative. Transplantation should be delayed for 3 months after BCG. Annual immunization with in \202 uenza vaccine is recom- mended. Hemodialysis Jugular venous catheters are the commonest form of vascu- lar access as there is usually only a short period of hemodi- alysis before transplantation. There are inherent dif \201 culties of creating arteriovenous \201 stulas in small children. Logistical pr oblems occur transporting patients from long distances to central specialized pediatric hem odialysis units. Dialysis and transplant options Transplantation For all children dialysis should be seen only as a temporary measure before transplantation . Restoration of good kidney function after transplantation offers the best opportunity for improved growth, school attendance and long-term psychosocial rehabilitation. A. WATSON 466 Further reading Batte S, Watson AR, Amess K. The effects of chronic renal failure on Hemodialysis is therefore chosen for mainly social reasons or when peritoneal dialysis acce ss is not possible, i.e. peri- tonitis or abdominal problems. Bicarbonate dialysis should b e standard and machines should be capable of ultra \201 ltration control. Hemodia \201 lt ration may have advantages over hemodialysis but requires ultrapure water. Hemodialysis reg imes are usually 4 h three times per week. Daily dialysis may result in better growth, impr oved bone disease and less anemia. Home hemodialysis is feasible but should only be attempt ed in those predicted to need long-term dialysis and who have good vascular access and a de dicated carer. siblings. Pediatr Nephrol 2006; 21: 246 \200\223250. Dixon P, Iurilli J, Watson AR, Neill E, Foy J, Martin M. Acceptability of a reformulated re nal-specific micronutrient supplement. Pediatr Nephrol 2004; 19: 1433 \200\2231443. Fischbach M, Edefonti A, Schroder C, Watson A, The European Pediatric Dialysis Working Grou p. Hemodialysis in children: gen- eral practical guidelines. Pediatr Nephrol 2005; 20: 1054 \200\2231066. Klaus G, Watson A, Edefonti A, et al. Prevention and treatment of renal oste odystrophy in children on chronic renal failure: European guidelines. Pediatr Nephrol 2006; 21: 151 \200\223159. Lewis M, et al. Demography and management of childhood estab- lished renal failure in the U K. In: Ansell D, Feest TG, Tomson C, Williams AJ, Warwick G (eds), UK Renal Registry Report . Bristol: UK Renal Registry; 2006. Chapter 13. Smith GC, Inward C. How and when to measure blood pressure. In: Webb N, Postlethwaite R (ed s), Clinical pediatric nephrology. Oxford: Oxford University Press; 2003. pp. 135 \200\2231 50. Psychosocial support This is an essential component of pediatric care. Every child should b e seen in the context of the family and stress upon the patient, parents and siblings recog nized. A social worker should assess each family at home to assess needs and any entitlemen t to state support, e.g. dis- ability allowance. A psychologist can help with the impact of illness upon the child, i.e. needle phobias, behavior disturbance, night- mares. Education al support is mandatory for children on hemodi- alysis but it is also important to liaise w ith schools if the child is on peritoneal dialysis and post transplantation. Most specialis t units have a holiday strategy which can include support for families, holidays for a pati ent group to build self-esteem and give families a break, and sport-ori- ented camps to inc rease participation and socialization. Youth workers can help support young people (aged 12 \200\22320 years) having major adjustment issues. Children and families should be discusse d at regular psy- chosocial team meetings in order to share knowledge and anticipate proble ms. Watson AR. Strategies to support the families of children with end stage renal failure. Pediatr Nephrol 1995; 9: 628 \200\223631. Watson AR. Meeting the information needs of children and their fami- lies . In: Postlethwai te R, Webb N (eds), Clinical pediatric nephrology, 3rd ed. Oxford University Press, Oxford; 2003. pp. 465 \200\223474. Watson AR. Hospital youth work and adolescent support. Arch Dis Child 2004; 89: 440 \200\223442. Watson AR. Problems and pitfalls of transition from pediatric to adult renal care. Pediatr Nephrol 2005; 20: 113 \200\223117. Internet resources British National Formulary for Children: http://www.bnfc.org NKF K/DOQI Guidelines. Clinical Practice Guidelines and Clinical Practi ce Recommendations. 2006 Updates. Peritoneal Dialysis Adequacy: http://www.kidney.org/professionals/kdoqi/ guideline_upHD_PD_VA/pd_wg.htm Child and carer i nformation on kidney disease: http://www.nuh.nhs.uk/ext/ckn/information.htm http://www.ich.ucl.ac.uk/factsheets/index.html NICE Clinical Guidance CG39. Anemia manageme nt in people with chronic kidney disease. Sept 2006: Follow-up The survival rate of children with CKD is good and the majority will transfer to adult care. In patients with CKD 1 \200\2233 this may be the primary care physician with in structions to refer back to adult services if increasing proteinuria or creatinine develop. Patients with CKD 4 or 5 need an organized transfer to adult units when they have followed a transition process with adequate information and preparation. Transition issues need to be addressed in both the pediatric and adult units and processes agreed so that follow-up a nd hopefully patient compliance is assured. http://guidance.nice.org.uk/CG39 See also Urinary tract infections in infancy and childhood, p. 252 Hypertensive children, p . 304 Acute kidney injury in infants and children, p. 372 Congenital abnormalities of the u rinary tract, p. 658 Vesicoureteric re \202 ux and re \202 ux nephropathy, p. 648 This page intentionally left blank K. BUCK 468 Chronic kidney disease in the elderly \200 Introduction Life expectancy in most developed countries has increased substantially in rec ent years. Around 20% of the total popu- lation are aged >65 years and 6% are >75 years; th ese \201 g- ures are likely to increase further. The greater life expectancy of females m eans that they increase as a pro- portion of the population in older cohorts and among thos e with CKD. The presence of ESRD requiring dialysis increases the risk of death three-fold at the age of 75 years compared to the healthy population. However, it should be noted that the increase in risk and years of life lost are substantially less than for the middle-age d dialysis patient. The incidence of newly diagnosed CKD increases signi \201 - cantly wit h age and the mean age for new referrals in developed countries is now 60 \200\22365 years. The median age of patients starting dialysis in the UK in 2006 was 65 years, which has cha nged little over the previous 6 years. Elderly patients with ESRD have been significantly d isadvantaged in terms of referral for RRT and tran- splantation. This has been because of a combination of reluctance to use scarce facilities for those with less to gain and a perce ption that the elderly will tolerate the process less well. Late referral of elderly patien ts has been a particular problem with its attendant increased mortality and morbidity, incr eased time of hospitalization, and dis- advantage with regard to choice of therapy and acce ss to transplantation. The introduction of reporting of eGFR in the UK in 2006 was primaril y to reduce late referral by highlighting that relatively low levels of creatinine in elder ly patients may represent advanced renal impairment. The marked aging of the dialysis popul ation in recent years can be attributed to: \200 \200 they are more vulnerable to complications; they have a greater mortality from infection and cardio- vascular disease; they survive for shorter periods;