Early kidney transplantation improves neurocognitive outcome in patients with severe congenital chronic kidney disease.

Renal replacement therapy has become available for the majority of patients suffering from severe congenital chronic kidney disease (CKD). Data on the long-term neurocognitive outcome and the impact of early kidney transplantation (KTx) in this setting is unclear. Neurocognitive outcomes in 15 patients (11 male) with isolated congenital CKD (stage 3-5) requiring KTx at a mean age of 2.8 ± 1.3 were assessed at a mean age of 8.3 ± 1.4 years. Patients underwent neurological examination and testing for neuromotor and neurocognitive function using three independent tests. Pre-emptive KTx was performed in six patients, and nine patients were dialyzed prior to KTx for a mean period of 11.1 ± 8.6 months. Neuromotor function was abnormal in 8/15 patients. HAWIK-III showed a global intelligence quotient (IQ) of 93.5 ± 11.4 (P = 0.05) due to a significantly reduced performance IQ of 89.1 ± 11.3 (P < 0.01). In three patients, the global IQ was clinically significantly reduced by >1 SD to <85. In patients with neuromotor dysfunction, performance IQ was lower than in patients with normal neuromotor function (83.8 ± 10.2 vs. 96.2 ± 9.0, P = 0.04). Time on dialysis was inversely correlated to verbal IQ (r = 0.78, P = 0.02). Pre-emptive KTx and duration of dialysis treatment <3 months was associated with superior neurocognitive outcome. Early (pre-emptive) KTx results in superior long-term neurocognitive outcome in children with severe congenital CKD.

Effective treatment of anemia in pediatric kidney transplant recipients with methoxy polyethylene glycol-epoetin beta.

MPG-EPO is a continuous erythropoietin receptor activator with a longer half-life than darbepoetin, hence requires less frequent injections. It has been successfully used in adults, but currently, there are no published data available for its use in children. This pilot study was performed to verify the effect of MPG-EPO on Hb levels in children. Twelve patients (age 6.4-17.2 yr) were treated with MPG-EPO as an individual "Heilversuch" according to German law after RTx. Five patients were switched from DA, and seven were naïve to erythropoietin. Over a period of six months, Hb levels were measured monthly. A median MPG-EPO dose of 2.5 µg/kg was administered intravenously in a single dose every four wk. The median Hb value increased in naïve patients from 9.9 to 11.2 g/dL (median, p = 0.004) and from 10.3 to 11.6 g/dL (median, p = 0.39) in patients switched from DA to MPG-EPO. No adverse events secondary to MPG-EPO therapy were detected. Our results indicate that a once-monthly injection of MPG-EPO is an effective treatment of anemia in children after renal transplantation. Larger randomized trials will have to confirm our findings.

Aetiology and outcome of acute and chronic renal failure in infants.

BACKGROUND: The aetiology and outcome of acute (ARF) and chronic renal failure (CRF) in infants were analysed in a retrospective study. METHODS: Between January 1997 and April 2004 all children <1 year of age with a serum creatinine >100 mumol/l at Hannover Medical School were followed up for up to 6 years. One hundred and nineteen children with a serum creatinine >100 mumol/l were identified, 70 infants suffering from ARF and 49 from chronic kidney disease (CKD), stages 3-5. RESULTS: Renal failure was caused in 49/119 (41%) by congenital and in 70/119 (59%) by acquired diseases. The aetiology of ARF (n = 70) included cardiac (27%), prematurity (27%), septic (10%), hepatic (9%), renal (9%) and other (18%) causes. Twelve infants needed transient dialysis treatment. Renal function recovered in all surviving children. The mortality rate was 37%. Causes of death were unrelated to kidney function. Twenty-one of 49 infants with CKD were dialyzed with a median age of 65 days at the start of dialysis, and 23/49 children received a kidney transplant (RTx). The 5-year patient and graft survival for RTx-children of 95.5% was not different from older children. The 5-year patient survival rate of 26 children with CKD without RTx was 63%. The causes of death were parental refusal of therapy in neonates (n = 4) and life-threatening extra-renal comorbidity (n = 3). CONCLUSION: Renal replacement therapy offers good chances of survival in infants without life-threatening comorbidity. Patient survival of infants treated for CKD in the first year of life was comparable to that of older children.

Renal replacement therapy in infants with chronic renal failure in the first year of life.

BACKGROUND AND OBJECTIVES: Although results of renal replacement therapy (RRT) in small children have improved during recent years, data about RRT in neonates are scarce. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In a retrospective study, we analyzed the outcome of infants who had chronic kidney disease and started RRT within their first year of life. Between 1997 and 2008, all 29 infants who were younger than 1 yr, had end-stage renal failure, and underwent RRT (dialysis or transplantation) at Hannover Medical School were analyzed for up to 12 yr. RESULTS: Twenty-seven of 29 infants with chronic kidney disease received peritoneal dialysis, starting at a mean age of 112 d; two children received preemptive renal transplantation (RTx). During follow-up, 21 of 29 children survived with RTx. The 5-yr patient and graft survival rate after RTx was 95.5%. Six of 29 children died, one with a functioning graft and five while on peritoneal dialysis. The main causes of death were severe cardiovascular and cerebral comorbidities. The mean GFR at last follow-up of patients who underwent RTx (mean time after RTx 5.1 yr) was 63.2 ml/min per 1.73 m(2). CONCLUSIONS: RRT in infants who are younger than 1 year offers excellent chances of survival and should be offered to all infants who do not have severe, life-limiting extrarenal comorbidity. Contrary to previous observations, the long-term outcome of infants may be comparable to that of older children who undergo RRT.