Renal function greatly influences mortality rates in the early phase following hematological stem cell transplantation (HSCT) in childhood, as well as the quality of life in long-term survivors. Nevertheless, the number of studies in pediatric populations is limited and some important aspects of kidney function after HSCT have only been elucidated in adults. The incidence of acute renal failure (ARF) immediately after HSCT in pediatric patients is between 25% and 50%, with 5%–10% of children requiring renal replacement therapy. Doubling of serum creatinine is associated with a twofold increase in mortality. However, the need for dialysis leads to a further increase in mortality rates to 80%–90%. Specific renal syndromes appear at different times following HSCT, revealing a similar pattern in children and adult patients. In both children and adults, impaired renal function associated with liver impairment (hepatorenal syndrome) is the most important cause for ARF. Therapeutic approaches have not been able to reduce the frequency or to improve outcome so far. In adults surviving long term, bone marrow transplant (BMT) nephropathy is the most frequent renal complication, although a considerable variation in incidence (up to 70%) has been published, partly due to various definitions and manifestations. Little is known about the long-term outcome of renal function in patients treated with HSCT in childhood. However, chronic renal failure has been reported in 0%–28%, but no end-stage renal failure has been published so far. Tubular function following HSCT is rarely investigated, although its impact on long-term survivors of BMT in childhood might be of some importance, especially for growth and bone metabolism.

BACKGROUND:The epidemiology and clinical characteristics of the hemolytic-uremic syndrome (HUS) caused by Escherichia coli O157:H7 are well-known, but HUS attributable to non-O157:H7 Shiga toxin (Stx)-producing E. coli (STEC) are less thoroughly described. Here we report a cluster of HUS cases caused by STEC O26:H11 the most common non-O157:H7 STEC isolated from sporadic cases of HUS in Europe. METHODS:Three children between 13 and 17 months of age, living in the same small town, developed HUS within an interval of 5 days. We present clinical and microbiologic data on the patients and their infecting isolates. RESULTS:The clinical course ranged from mild uncomplicated HUS to severe HUS complicated by multiorgan involvement. Microbiologic investigation demonstrated STEC of serotype O26:H11 in stools of all the patients. The phenotypic and molecular characterization of the STEC O26:H11 isolates demonstrated that these strains were identical and, unusual for STEC O26, they harbored the stx2 but not the stx1 gene. None of the patients had evidence of STEC O157:H7 infection either by culture or by E. coli O157 serology. The source of the STEC O26:H11 infection was undetermined. CONCLUSIONS:Our results demonstrate that diagnostic procedures based on the detection of stx genes and/or Stx production and subsequent subtyping of the isolates using molecular methods are necessary to identify such outbreaks caused by non-O157:H7 STEC.

The aim of this prospective study was to assess glomerular and tubular renal function before, and 1 and 2 years after hematological stem cell therapy (HSCT) in children and adolescents. 137 consecutive patients undergoing HSCT, for malignant diseases, were included in a prospective trial. Forty-four patients were followed for up to 1 year after HSCT and 36 for up to 2 years, without relapse. Ninety healthy school children were used as a control group. The following parameters were investigated: inulin clearance (GFR), urinary excretion of albumin, α 1 -microglobulin (α 1 -MG), calcium, β-N-acetylglucosaminidase (β-NAG) and Tamm–Horsfall protein (THP), tubular phosphate reabsorption (TP/Cl cr ) and percent reabsorption of amino acids (TAA). Significantly lower GFR was found 1 and 2 years after HSCT but within the normal range in the period before HSCT. There was no correlation between GFR within the first month after HSCT and long-term outcome of GFR. Tubular dysfunction was found in 14–45% of patients 1 and 2 years after HSCT depending on the parameter investigated. Pathological values 1 and 2 years after HSCT were found for α 1 -MG excretion in 40% and 39%, respectively, for TP/Cl cr in 44% and 45%, for β-NAG in 26% and 19%. Median TP/Cl cr was significantly lower 2 years after HSCT than before. TAA was mildly impaired in 7/14 patients before, in 5/29 one and in 9/29 2 years after HSCT, but median TAA was within normal range at all times. The median excretion of albumin, THP and calcium was within the normal range at all investigations. No influence of ifosfamide pre-treatment on the severity of tubulopathy was found. The investigation of tubular renal function should be part of a long-term follow-up in children after HSCT. Bone Marrow Transplantation (2001) 27, 319–327.

HUS in children is caused in about 90% of cases by Shiga-toxin-producing E. coli (STEC), especially O157:H7, and therefore called typical or D+ HUS. The most important reservoir for STEC is the intestine of cattle. The transmission takes place by contaminated beef products or from person-to-person. The infection manifests itself with bloody diarrhea. 10% of cases develop HUS, especially young children. After absorption into circulation Shiga-toxins bind to specific glycolipid-receptors (Gb3) to endothelial cells of the microvasculature of the kidney, colon and central nervous system. Various pathomechanisms finally lead to thrombotic microangiopathy with hemolytic anemia, thrombocytopenia and renal failure. Therapy consists of supportive measures. Two thirds will need dialysis. Acute mortality is caused above all by severe extrarenal complications. It has decreased drastically over the last decades and now amounts to only 5% of cases. A further 5% develop primary end-stage renal disease (ESDR). About 30% sustain less severe chronic problems (proteinuria, hypertension, decreased GFR) with the risk of developing ESDR. Until now there have been no proven prophylactic measures except hygienic ones. The early administration of substances for binding of Shiga-toxins in the intestine is under clinical investigation. Improvement of prognosis is possible, however, only through early diagnosis of HUS, treatment in pediatric nephrology centers and long-term follow-up.

We report a patient who developed Epstein–Barr virus (EBV)-induced transverse myelitis 19 months after unrelated bone marrow transplantation (BMT). The disease was diagnosed by physical examination, serologic determinations, EBV-specific polymerase chain reaction in peripheral blood lymphocytes and cerebrospinal fluid, and characteristic magnetic resonance imaging scan of the spine. The patient was treated with ganciclovir and cytomegalovirus (CMV) hyperimmune globulin. He gradually improved and recovered completely within 4 weeks. This case suggests that ganciclovir and CMV hyperimmune globulin appear to be effective for the treatment of EBV-induced transverse myelitis in immunocompromised patients following BMT.