Summary: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, ␣ 1 -microglobulin (␣ 1 -MG), calcium, ␤-Nacetylglucosaminidase (␤-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 ␣ 1 -MG excretion in 40% and 39%, respectively, for TP/Cl cr in 44% and 45%, for ␤-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.

Shiga toxin-producing Escherichia coli (STEC) strains are primarily food-borne pathogens that may cause diarrheal outbreaks and are associated with severe complications, specifically hemolytic-uremic syndrome (HUS). We report here ge-nome sequence data for STEC O26:H11, which is associated with a cluster of cases of HUS, a rarely described syndrome in South Africa.

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.

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