Clearance and removal of oxalate in children on intensified dialysis for primary hyperoxaluria type 1.

Patients with end-stage renal failure owing to primary hyperoxaluria type 1 (PH1) receive dialysis while waiting for transplantation. So far, dialysis has not been shown to overcome the problem of ongoing oxalate production and deposition at extrarenal sites. We report on six children with PH1 who had to be dialyzed for a median period of 2.5 years while awaiting liver transplantation. Aiming at preventing oxalate tissue accretion, oxalate mass transfer was studied and dialysis intensified accordingly. Mean plasma oxalate concentration was between 51 and 137 micromol/l. In three of the six patients with a urinary output between 630 and 3140 ml, urinary removal of oxalate was between 5.6 and 12.4 mmol/week/1.73 m2. Hemodialysis (HD) in five of the six patients demonstrated a mean oxalate dialysance between 158 and 444 l/week/1.73 m2. Peritoneal dialysis (PD) in two of the six patients showed mean oxalate clearances of 66 and 103 l/week/1.73 m2. One patient received HD and PD. By adding all modes of elimination, a mean total oxalate mass between 10.1 and 24.1 mmol/week/1.73 m2 was removed. Dialysis is still necessary as a temporary therapy for a number of patients with PH1. Dialysis should be instituted pre-emptively and maximally exploited by intensified HD/PD treatment protocols, without, however, cutting back urinary output.

[Electron beam computed tomography of the heart before kidney transplantation. Severe coronary disease in a 21-year old woman with nephrocalcinosis due to infantile hypercalcemia syndrome]. / Elektronenstrahltomographie des Herzens vor Nierentransplantation. Schwere koronare Herzerkrankung bei einer 21-Jährigen mit Nephrokalzinose bei infantilem Hyperkalzämiesyndrom.

BACKGROUND: Cardiovascular diseases are very common in patients with end-stage renal disease and are the underlying cause of approximately half the deaths in dialysis patients. In those patients vascular calcifications are typically seen in the tunica media and therefore represent histopathological changes different to those in atherosclerosis. For the evaluation of cardiovascular risk in chronic dialysis patients, a method is needed to reliably identify patients who have to undergo invasive diagnostics. Coronary artery calcium assessed by electron beam computed tomography (EBT) has been suggested to measure the extent of coronary atherosclerosis in patients with normal renal function. It might also be of value in the stratification of cardiovascular risk in patients with renal failure. CASE REPORT: We report the case of a 21-year-old female with end-stage renal disease as a result of idiopathic infantile hypercalcemia with nephrocalcinosis and extensive vascular calcification. Due to a total atrioventricular block, a 2-chamber pacemaker had been implanted in 1998. Because of a very high calcium score in EBT, depressed left ventricular function in echocardiography, and limited physical strength she was sent to coronary angiography. Severe 2-vessel coronary artery disease was found and successfully treated by balloon angioplasty. She was then listed for renal transplantation. Angiographic 6-month follow-up showed an excellent long-term result. CONCLUSION: This case demonstrates that coronary artery disease can also be present in young dialysis patients. Discrimination of atherosclerotic vascular calcification and tunica media sclerosis typical in dialysis patients without hemodynamic effects is difficult. EBT allows for the direct detection of coronary calcium. Its extent correlates with coronary atherosclerosis and the probability of acute coronary syndromes in patients with normal renal function. This evidence has not been proved for dialysis patients, yet. However, EBT may be of value in the stratification of cardiovascular risk for patients awaiting renal transplantation.

Platelet adenylyl cyclase signaling remains unaltered in children undergoing hemodialysis treatment.

Patients with chronic renal failure exhibit multiple endocrine, gastrointestinal and cardiovascular abnormalities, many of which may be explained by alterations of adenylyl cyclase (AC) responsiveness and/or G-protein expression. Since such alterations were previously reported, e.g., for platelets of adult chronic renal failure patients undergoing hemodialysis treatment (HD), we have investigated whether children with chronic renal failure undergoing HD exhibit similar alterations. Eleven uremic children undergoing HD were compared with 11 age-matched healthy controls. Platelet AC activity was determined in the absence (basal) and presence of a receptor agonist, direct G-protein activators and direct AC stimulators. G-protein alpha-subunits were measured by quantitative immunoblotting. Basal and stimulated platelet AC and immunoreactivity for platelet G-protein alpha-subunits did not significantly differ between HD and control children. We conclude that HD in children is associated with much smaller, if any, abnormalities of blood cell signal transduction than in adult patients. We speculate that quality of dialysis, age, and underlying disease might differentially influence blood cell signal transduction cascades.

[Home blood pressure self-measurement in children and adolescents with renal replacement therapy]. / Häusliche Blutdruck-Selbstmessung bei Kindern und Jugendlichen mit Nierenersatztherapie.

BACKGROUND: Most patients with terminal renal failure show arterial hypertension. In addition to casual blood pressure measurements in the clinic, home blood pressure measurement is recommended for these patients to control arterial blood pressure. PATIENTS: The study was performed in children with hemodialysis (HD; n = 11), peritoneal dialysis (PD; n = 14) or after renal transplantation (NTX; n = 21) from one department of Pediatric Nephrology. We performed a retrospective analysis of home blood pressure values from patients' diaries. METHODS: The average number of blood pressure measurements per day and the mean blood pressure values were calculated from the blood pressure data documented during one month at home. Single measurements above the 95th percentile for height and gender were defined to be hypertensive and the frequency as percentage of all documented values was calculated. RESULTS: Four patients did not document any blood pressure values at home. The other patients documented an average of 2.3 measurements per day. Systolic hypertension was found in 7% of patients defined by home BP measurements compared to 30% defined by casual BP measurements. Prevalence of diastolic hypertension did not differ between both methods (35% vs. 46%). Mean home BP was significantly higher than values after HD and lower than values before HD. Mean clinic BP was significantly higher in PD-patients compared to home BP. Home and clinic blood pressure values did not differ in patients after renal transplantation. The mean percentage of hypertensive readings for systolic BP was 5 and for diastolic BP 39%. CONCLUSIONS: Blood pressure measurement at home is performed reliably by most children and adolescents with chronic renal failure and shows lower values than clinic BP in many patients. It is an important method for control of blood pressure and a valuable supplement to 24 h blood pressure monitoring.

Mutations in the chloride channel gene, CLCNKB, leading to a mixed Bartter-Gitelman phenotype.

Gitelman syndrome is an inherited renal disorder characterized by impaired NaCl reabsorption in the distal convoluted tubule and secondary hypokalemic alkalosis. In clinical practice, it is distinguished from other hypokalemic tubulopathies by the presence of both hypomagnesemia and normocalcemic hypocalciuria. To date, only mutations in a single gene encoding the thiazide-sensitive NaCl cotransporter have been found as the molecular basis of GS. We describe three unrelated patients presenting with the typical laboratory findings of GS. Mutational analysis in these patients revealed no abnormality in the SLC12A3 gene. Instead, all patients were found to carry previously described mutations in the CLCNKB gene, which encodes the kidney-specific chloride channel ClC-Kb, raising the possibility of genetic heterogeneity. Review of the medical histories revealed manifestation of the disease within the first year of life in all cases. Clinical presentation included episodes of dehydration, weakness, and failure to thrive, much more suggestive of classic Bartter syndrome than of GS. The coexistence of hypomagnesemia and hypocalciuria was not present from the beginning. In the follow-up, however, a drop of both parameters below normal range was a consistent finding reflecting a transition from cBS to GS phenotype. The phenotypic overlap may indicate a physiologic cooperation of the apical thiazide-sensitive NaCl cotransporter and the basolateral chloride channel for salt reabsorption in the distal convoluted tubule.

Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis maps to chromosome 3q27 and is associated with mutations in the PCLN-1 gene.

Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis (FHHNC, MIM 248250) is a complex renal tubular disorder characterised by hypomagnesaemia, hypercalciuria, advanced nephrocalcinosis, hyposthenuria and progressive renal failure. The mode of inheritance is autosomal recessive. A primary defect in the reabsorption of magnesium in the medullary thick ascending limb of the loop of Henle (mTAL) has been proposed to be essential in FHHNC pathophysiology. To identify the underlying genetic defect we performed linkage analysis in eight families, including three with consanguineous marriages. We found linkage to microsatellite markers on chromosome 3q27 with a maximum two-point lod score (Zmax) of 5.208 for D3S3530 without evidence for genetic heterogeneity. Haplotype analysis revealed crucial recombination events reducing the critical interval to 6.6cM. Recently, mutations in the gene PCLN-1, mapping to 3q27 and coding for paracellin-1, were identified by Simon et al (1999) as the underlying genetic defect in FHHNC. Paracellin-1 represents a renal tight junction protein predominantly expressed in the TAL. Mutational analysis in our patient cohort revealed eight different mutations in the PCLN-1 gene, within six novel mutations. In seven of 13 mutant alleles we detected a Leu151 substitution without evidence for a founder effect. Leu151 is a residue of the first extracellular loop of paracellin-1, the part of the protein expected to bridge the intercellular space and to be important for paracellular conductance. This study confirms the implication of paracellin-1 defects in FHHNC and points to a predominant role of this protein in the paracellular reabsorption of divalent cations in the TAL.

Middle molecules in peritoneal equilibration test as a marker of peritoneal stress in children on continuous peritoneal dialysis.

At 1 month, 3 months, 6 months, and more than 6 months after healed peritonitis, we evaluated repeated peritoneal equilibration tests (PETs) for small molecules such as urea, and middle molecules such as cystatin C, beta 2-microglobulin, and alpha 1-microglobulin. We analyzed a total of 104 PETs in 21 children aged 1.7-18.6 years (median: 9.9 years). Equilibration quotients (D/P)--that is, substrate concentration in dialysis fluid (D) divided by substrate concentration in plasma (P)--were calculated after a dwell time of 4 hours. The D/P for urea did not change after healed peritonitis. In a cross-sectional study, the D/P for middle molecules showed an increase in peritoneal permeability between 3 months and 6 months after a healed peritonitis. In a consecutive follow-up of 4 patients for more than 6 months, beta 2-microglobulin and, more impressively, alpha 1-microglobulin showed a statistically significant increase in D/P (p < 0.05) 3 months after a healed peritonitis. All differences seen were completely reversible after more than 6 months, showing that peritoneal function is rather stable if peritonitis is healed. It is noteworthy that peritoneal dysfunction lasts for up to 6 months after a completely healed peritonitis. This period might be a vulnerable phase in continuation of peritoneal dialysis.

Spectrum of early onset nephrotic syndrome associated with WT1 missense mutations.

We investigated 17 children with nephrotic syndrome (NS) of early onset (14 aged < 1 year) and rapid progression to end-stage renal disease for the presence of mutations in the Wilms' tumor suppressor gene WT1 on chromosome 11. In eight children (7 genotypic males) an association with Wilms' tumor and/or ambiguous genitalia (Denys-Drash syndrome) was observed. In these eight and two additional female patients with NS only constitutional missense mutations in the WT1 gene were detected; four children presented the so-called hot spot mutation in exon 9 (R394N) and six had different mutations in exons 8 and 9 (4 not previously described). Renal biopsy showed diffuse mesangial sclerosis in eight and focal segmental sclerosis in two cases. End-stage renal disease was reached either concomitantly or within four months after onset of NS in seven of ten patients. A unilateral Wilms' tumor was found before or concomitant with NS in four children (3 males, 1 female). From the seven genotypic males with WT1 mutations, five presented ambiguous genitalia and two a female phenotype. No mutation of the WT1 gene was found in seven other children with isolated congenital or infantile NS with or without DMS who appeared to have a slower progression than the first group. It is proposed that patients with early onset, rapidly progressive NS and diffuse mesangial or focal segmental sclerosis should be tested for WT1 mutations to identify those at risk for developing Wilms' tumor.

Variability of peritoneal equilibration test in children on continuous peritoneal dialysis by repeated testing with solutions of different osmolarity.

To evaluate (1) differences in the peritoneal equilibration test (PET) achieved using continuous peritoneal dialysis (CPD) solutions containing different amounts of glucose and (2) intraindividual reproducibility of PETs performed twice within an interval of 8 months on CPD, we investigated 39 PETs in 13 children aged 2.4-19.0 years (median 10.6 years) on stable CPD regimens. The fill volume was 1 L/m2 body surface area. We used a standard CPD solution (Fresenius) with a 2.3% glucose content (groups 2.3a and 2.3b) two times within an interval of 1-8 months. A third test was done between the two with a CPD solution of 1.5% glucose (group 1.5). Equilibration quotients, that is, substrate concentration in dialysis fluid divided by substrate concentration in plasma (D/P), did not show any statistically significant differences between groups 1.5 and 2.3a or between groups 2.3a and 2.3b. A significant difference was seen in the decline of glucose content of dialysate between groups 1.5 and 2.3 but not between groups 2.3a and 2.3b. Ultrafiltration was higher in groups 2.3a and 2.3b compared with group 1.5. Inter- and intraindividual variability between solute transfer was small during follow-up in stable CPD patients. Different glucose contents of 1.5 and 2.3 g/dL dialysis fluid had no measurable influence on PET results of stable CPD patients. For standard PETs, reducing the glucose content of dialysis fluid to isoosmolarity is not necessary.

Value of diuresis renography in the post-natal period of assumed physiological renal immaturity.

The aim of this study was to determine if it is possible to exclude renal obstruction using diuresis renography in the first 6 weeks of life (the period of physiological renal immaturity), thus avoiding unnecessary invasive procedures, such as the Whitaker test or surgery. Diuresis renography with 123I-hippuran was performed in 27 patients aged less than 6 weeks and in 50 older children who acted as a reference group (age 6 weeks to 1 year, n = 28; age 1-10 years, n = 22). All 27 patients had significant dilatation of the pelvicalyceal system on ultrasonography. Renal curves were evaluated by mathematical curve characteristics (split renal function, counts, T-max, etc.) as the visual grade of obstruction. Whole-kidney regions of interest were defined on images summed over 30 min; renal parenchyma on images summed over 5 min. The renal curves of 18/27 patients indicated tracer accumulation and led to frusemide administration. Only two patients showed no significant response to frusemide and had to be further investigated by the Whitaker test. The frequency of kidneys with no response to frusemide revealed no significant differences in the three groups. Whole-kidney evaluation resulted in an overestimation of obstruction in 9/150 kidneys, which matches the lower correlation to the DMSA separation values for this method of evaluation. In contrast with the literature, significant post-renal obstruction can be excluded by diuresis renography in most cases in spite of renal immaturity and can help to avoid invasive procedures.

Calcium acetate versus calcium carbonate as oral phosphate binder in pediatric and adolescent hemodialysis patients.

Calcium carbonate is widely used as an oral phosphorus binder to control hyperphosphatemia in children on maintenance hemodialysis. Intestinal calcium absorption may induce hypercalcemia, particularly if calcitriol is given simultaneously. In adults, calcium acetate binds phosphorus more effectively than calcium carbonate, while reducing the frequency of hypercalcemic events. We therefore compared calcium acetate with calcium carbonate in nine pediatric patients on long-term maintenance hemodialysis. Following a 1-week withdrawal of phosphorus binders, calcium carbonate was administered for 7 weeks; after a second withdrawal, calcium acetate was given for another 7 weeks. All patients received calcitriol regularly. Both agents lowered the serum phosphorus concentration significantly (calcium carbonate 5.7 +/- 1.4 vs. 7.7 +/- 2.1 mg/ dl, P < 0.005; calcium acetate 5.8 +/- 1.4 vs. 7.8 +/- 2.0 mg/dl, P < 0.005). Significantly less elementary calcium was ingested with calcium acetate than with calcium carbonate: 750 (375-1,500) vs. 1,200 (0-3,000) mg calcium/day, P < 0.0001. Wit calcium carbonate serum calcium increased significantly. The number of episodes of hyperphosphatemia or hypercalcemia did not differ between treatments. Intact plasma parathyroid hormone (PTH) decreased significantly with both phosphate binders, and serum 25-hydroxyvitamin D3 increased. There was a close relationship between serum phosphorus and PTH in prepubertal but not in pubertal patients. We conclude that hyperphosphatemia can be controlled effectively by both calcium acetate and calcium carbonate in pediatric hemodialysis patients. The oral load of elementary calcium is reduced significantly by binding phosphorus with calcium acetate instead of calcium carbonate; nevertheless, hypercalcemic episodes remain equally frequent with both phosphate binders.

24-hour blood pressure monitoring in children and adolescents after recovery from hemolytic uremic syndrome.

24-hour blood pressure monitoring is a valuable method for the diagnosis of arterial hypertension as well as for assessment of the diurnal rhythm of the arterial blood pressure (BP). The nocturnal decrease of blood pressure ("dipping") may be attenuated or abolished in children with advanced renal failure and glomerular diseases. Arterial hypertension is a longlasting problem in children who had recovered from hemolytic uremic syndrome (HUS). We therefore performed BP monitoring in 11 children and adolescents (age 1.3 to 18.8 years, 6 males, 5 females) after HUS using a portable oscillometric device (SpaceLabs 90207). Six of the subjects had a normal renal function (group A). The other 5 patients had impaired renal function with a glomerular filtration rate <60 ml/min/1.73 m2 (group B). Nocturnal dipping was calculated as nocturnal mean blood pressure minus diurnal mean blood pressure given in per cent of diurnal mean blood pressure. Two of the patients in group A had diurnal mean BP above the 95th percentile of the German collaboration study, but none of the group was hypertensive during the night, and nocturnal dipping was 13.6% (9.7-15.5%, median and range) for systolic BP and 23.7% (15.5-29.9%) for diastolic BP which is very similar to healthy children. All of the patients had a normal diurnal BP rhythm. From patients of group B, 4 had elevated diurnal mean BP and also 4 were hypertensive during the night. Nocturnal dipping was 1.4% (0.7-4.1%) for systolic and 6.8% (0-10.7%) for diastolic BP which is clearly attenuated compared to group A. We therefore conclude that arterial hypertension is more common in patients after HUS if they have impaired renal function, and diurnal rhythm of arterial blood pressure is attenuated in these patients. However, nocturnal dipping of blood pressure is not disturbed in children after HUS without renal insufficiency, even if they were hypertensive.

Blood pressure monitoring at the wrist: is it reliable in children and adolescents?

We studied in children and adolescents the performance of two devices for blood pressure measurements at the wrist: "Blood pressure watch" (BPW) and Omron R1 (OR1). They were tested against auscultatory blood pressure readings at the upper arm. Wrist circumference correlated to the aged was greater than 13.5 cm in nearby all subjects older than 12 years. BPW overestimated systolic blood pressure for more than 10 mm Hg and diastolic blood pressure for 4 to 7 mm Hg in all subjects. OR1 underestimated both systolic and diastolic blood pressure by 5 to 6 mm Hg in the group with a wrist greater than 13.5 cm and by 8 to 9 mm Hg in the group with a smaller wrist. No difference was found between left and right wrists by OR1.

Atrial natriuretic peptide and cyclic guanosine-monophosphate in children and adolescents on peritoneal dialysis.

Plasma concentrations of atrial natriuretic peptide (ANP) and its second messenger cyclic guanosine-monophosphate (cGMP) were studied in 28 children and adolescents (1 to 19 years) on peritoneal dialysis and compared to 55 healthy children (1 to 20 years). Dialysate concentrations of the hormones were measured also in the patients. Plasma ANP was not significantly different in patients and controls (28.8 pmol/l [15.5-53.6 pmol/l] [median, lower and upper quartile] versus 26.3 pmol/l [19.9-31.8 pmol/l]). In seven children on peritoneal dialysis it exceeded an upper normal limit of 50 pmol/l, but it fell to normal values in four of them after forced fluid withdrawal. Plasma cGMP was elevated in the patients compared to the control children (1.6 nmol/l [1.1-1.7 nmol/l] versus 1.0 nmol/l [0.8-1.2 nmol/l]; p < 0.05). There were only weak correlations between plasma and dialysate concentrations of ANP and cGMP. Plasma concentrations of ANP seem to be elevated in children on peritoneal dialysis in case of fluid overload.

Primary obstructed megaureter in neonates. Treatment by temporary uretero-cutaneostomy.

During the last 10 years we have treated 24 newborn and young infants (27 ureters) in whom the diagnosis of primary obstructed megaureter was established by diuresis-enhanced radionuclide renography and/or by antegrade pressure flow studies (Whitaker test). Temporary uretero-cutaneostomy was performed immediately after diagnosis. After 1 year a second modified Whitaker test showed normal passage of the contrast medium into the bladder in 23 ureters. In only 4 ureters did obstruction persist and uretero-cystoneostomy was performed. Ureterocystoneostomy was also performed on 8 ureters without obstruction but with persistent vesicoureteric reflux, ectopic ureter or diverticula. In 11 patients a temporary uretero-cutaneostomy was closed without corrective surgery at the vesicoureteric junction. Temporary uretero-cutaneostomy is a safe and effective therapy in primary obstructed megaureter in newborns. Spontaneous relief of obstruction appears to be possible in primary obstructed megaureter.

[Secondary rhabdomyolysis and acute renal failure in gastroenteritis with hypernatremic dehydration]. / Sekundäre Rhabdomyolyse und akutes Nierenversagen bei Gastroenteritis mit hypernatriämischer Dehydratation.

A child with severe gastroenteritis developed acute rhabdomyolysis combined with acute renal failure. The pathophysiological mechanisms of this clinical syndrome are not completely understood. Rhabdomyolysis is mainly due to osmotic changes on both sides of the cell membrane. The preventive and therapeutic measures consist of adequate fluid substitution, correction of electrolyte and acid-base imbalances, administration of diuretics and alkalization of the urine. Dialysis treatment may be necessary. In this particular case renal failure resolved but the child died of other organ failure.

Bent subcutaneous peritoneal catheter shape for regular dialysis treatment in children.

A permanent bowing of the subcutaneous part of the Tenckhoff-type catheter (bent neck--Quinton, and swan neck--Accurate Surgical Instruments) enables the catheter to turn from an upward direction of the subcutaneous tunnel to a downward direction by a smooth 160 degrees-180 degrees bend creating a downward skin exit. We have used this catheter shape in combination with a coiled intra-abdominal edge. Two sizes are available for children. We use 2 cuffs and glue them ourselves according to the body size. In this study we compare the durability of the traditional subcutaneously straight catheter in 8 children aged 0.1-12.6 years (Group A) with the bent shaped catheter in 8 children aged 3.7-15.8 years (Group B). Median duration of function was 10.5 (2-34) and 8 (3-36) months, respectively. Frequency of complications was equal in both groups: peritonitis episodes 0.69/year in Group A and 0.53/year in Group B; tunnel infection 0.16 vs 0.11/year; skin exit infection 0.54 vs 0.53/year; noninfectious complications 0.16 vs 0.32/year; mean number of catheters used was 1.0 vs 1.1/treatment year. Treatment had to be terminated in some patients: kidney transplantation 5, kidney recovery 1, severe peritonitis 1. The bent subcutaneous catheter shape did not show any medical or technical disadvantage compared with the straight type, but the downward directed catheter skin exit can be covered invisibly under bikini or bermuda shorts which means aesthetic and social advantage. Whether the downward drainage of secretes and cell detritus influences the rate of tunnel infection positively cannot be answered to date.

Peritoneal morphology in children treated by continuous ambulatory peritoneal dialysis.

Fifty peritoneal biopsies (PB) from 35 patients with end-stage renal disease, treated by continuous ambulatory peritoneal dialysis (CAPD) and aged 2 months to 18 years, were examined by light microscopy (n = 50) and/or scanning electron microscopy. PB were performed during surgical procedures immediately before the start of, during, or after the cessation of CAPD treatment. PB from 15 children without renal disease undergoing laparatomy were examined similarly. Before the start of CAPD, a scarcity and shortening of the mesothelial microvilli was observed by scanning electron microscopy. During and after CAPD, variable alterations of mesothelium, interstitium and capillaries were found. The mesothelial layer was absent in all 5 PB obtained during episodes of active peritonitis. In patients treated by CAPD for longer than 6 months, mesothelial denudation was observed more frequently (6/11) than in children treated for shorter periods (1/7) (P < 0.08). Fibrosis of the peritoneal membrane was present in about 50% of patients during or after the cessation of CAPD without impairment of peritoneal function. No correlation was found between the presence of fibrosis and the frequency of peritonitis or the duration of CAPD treatment.

Psychosocial adaptation of children and adolescents with chronic renal failure.

In a multicentre study comprising five paediatric nephrology centres in Western Germany, psychosocial and educational parameters were assessed (during 1987) in 479 children and adolescents with chronic renal failure (CRF) in order to gain insight into their psychosocial adaptation to the disease. At the time of assessment, 31% of patients were on conservative treatment, 14% on haemodialysis, 9% on continuous ambulatory peritoneal dialysis and 46% had a functioning transplant. The mean age at assessment was 13.6 years. Additional disabilities were noted in 29% of patients. School attendance of the 233 children of school age was in general satisfactory; 22% of patients attended schools for disabled or handicapped children. Vocational training was frequently inadequate, especially for dialysed patients, and only 14 of 53 adolescents over 16 years had graduated. Of 49 adult patients, only 21 were in some form of employment. A lack of age-appropriate independence was observed in a large proportion (86%) of patients over 17 years, who continued to live with their parents or other persons taking care of them, whilst only 14% were living alone or with a partner. We conclude that, despite improved survival, psychosocial adaptation continues to be impaired in paediatric patients with CRF, especially in adolescents and those on dialysis.

[Congenital malformations of the urinary tract. Rational postpartum diagnosis]. / Angeborene Fehlbildungen des Harntrakts. Rationelle postpartale Diagnostik.

More than one third of the anomalies diagnosed antenatally by ultrasound comprises the urinary tract. The criteria of the diagnostic and therapeutic approach, which resulted from our experience in children with symptomatic urinary tract anomalies cannot be necessarily applied to asymptomatic newborns. Not all urinary tract anomalies are obstructive and need surgical correction. Therefore, rational diagnostic approach after birth is necessary, which starts with ultrasonography. The first radiological diagnostic procedure is always a voiding cystourethrography in order to diagnose or exclude infravesical obstruction or a vesicoureteric reflux. The dynamic radionuclide nephrography (123I-Hippuran) under standardized conditions with adequate hydration and Furosemide-induced diuresis is able to detect a significant obstruction at the pelvi-ureteric or uretero-vesical junction. The intravenous urography is limited to specific questions and a computerized tomography is rarely indicated. In rare cases when dynamic investigations are equivocal, antegrade pressure flow studies according to Whitaker can be reliably exclude or prove obstruction.