Pediatric kidney transplantation: Siriraj Hospital experience.

We reported six children with end stage renal disease (ESRD) who received kidney transplantation in our unit from 1996 to 2000. They were 5 boys and 1 girl and their mean age was 9.7 +/- 2.7 years (range 6.8 to 13.2). Etiologies of ESRD were congenital anomalies (3 patients), chronic glomerulonephritis (2 patients), and rapidly progressive glomerulonephritis (1 patient). Prior to the transplantation, chronic peritoneal dialysis was used in 5 patients, including one who had to switch to hemodialysis due to chronic exit site infection and 1 had preemptive kidney transplantation. All children received a kidney from living-related donors, 4 from their fathers, 1 from his mother, and 1 from his elder brother. Triple immunosuppressive drug therapy (prednisolone, azathioprine, and cyclosporine A) was initially given to all patients. Serum creatinine returned to normal within the first week in all patients and 4 patients were discharged home by the end of the second week post operation. Immediate complications included severe hypertension (all patients), ureteral leakage (2 patients), neutropenia (3 patients) and nephrotic syndrome (1 patient). Azathioprine was discontinued in 2 patients due to persistent neutropenia. Cyclosporine A was discontinued in 1 patient due to hepatotoxicity, this patient was maintained on mycophenolate mofetil and prednisolone. Serum creatinine levels at last follow-up (mean 24.3 +/- 19.0 months, range 8-55) were normal in 5 patients and slightly increased (1.5 mg/dl) in one. Five patients returned to school full time within 1 year after kidney transplantation. Height standard deviation score improved markedly as early as 6 months post transplant. The cost of maintenance of the immunosuppressive drugs was similar to adults, i.e. 6,859.1 +/- 1,151.8 Baht per month at 6 months post kidney transplantation. We concluded that kidney transplantation can be performed successfully in selected Thai children with very good results and similar cost of treatment as for adults.

Chronic renal failure in Thai children: etiology, cost, and outcome.

We studied the epidemiology, cost and outcome of chronic renal failure (CRF) in Thai children by sending questionnaires to all university hospitals, all government general service hospitals and all pediatric nephrologists in the country. A total of 238 cases (107 from 8 university hospitals and 131 from 70 government general service hospitals) were diagnosed from 1996 to 1998. Mean age of the patients was 8.3 +/- 4.9 yr, male to female ratio was 1.4:1. Congenital KUB anomalies (obstructive uropathy and hypo/dysplasia) were the main causes of CRF in these patients, especially in the under five age group. Only a small number of patients received renal replacement therapy (chronic dialysis and kidney transplant) and the mortality rate was 18.7 per cent in university hospitals. Renal transplantation was performed in only 5 patients in 2 pediatric units and another 2 patients in adult renal units. The outcome of renal transplantation in this small group of patients was very satisfactory. The cost of CRF treatment in children was comparable to adults. The main problems in the management of CRF in Thai children included the lack of experienced personnel, lack of equipment and funding. We conclude that in order to improve the care of CRF in Thai children, a training program for health personnel and budget allocation should be established.

Renal pathology and long-term outcome in childhood SLE.

Renal histology is increasingly used as a guide for therapy and prognosis in SLE but data in children are few and/or short-term. We assessed renal histological features in 19 children with SLE to determine whether these features are useful in predicting long-term outcome. Mean age at biopsy was 10 +/- 1.7 years old, male to female ratio was 1:2.8. Fourteen patients (73%) had diffuse proliferative lupus nephritis. Renal histology was evaluated using an activity index (AI) and chronicity index (CI). Clinical assessment of renal function at biopsy and outcome were graded according to urinalysis and serum creatinine. Renal function at biopsy correlated well with AI (p < 0.001) but not CI. At short-term follow-up (30 months), 3 patients had died from sepsis and another 2 reached end-stage renal disease. CI predicted poor clinical outcome, i.e. death or renal failure (p < 0.005) but AI did not. At long-term follow-up (mean 92.1 +/- 26.8 months) only one more patient reached end-stage renal disease. In others renal function assessment showed improvement or were stable. Neither CI nor AI correlated with clinical outcome. We conclude that although AI correlates well with renal function at biopsy and CI with short-term prognosis, neither can predict long-term outcome. Treatment may have altered the natural course of disease in these patients.

Renal tubular function in beta-thalassemia.

Studies of the renal involvement in thalassemic syndromes have been varied and few. This study was designed to define the renal abnormalities associated with beta-thalassemia and to correlate the renal findings with clinical parameters. One hundred and four beta-thalassemic children with various disease severity were studied. The patients were divided into three groups: 48 with severe anemia [hematocrit (Hct) < 25%], 31 on a hypertransfusion program and desferrioxamine treatment, and 25 with moderate anemia (Hct > 25%). The results were compared with 15 normal children. Significantly higher levels of proteinuria and low molecular weight proteinuria were found in all patients compared with normal children. Aminoaciduria was detected in one-third of patients. Thalassemic patients had significantly lower morning urine osmolarity, higher urine N-acetyl-beta-D-glucoseminidase and malondialdehyde (MDA, an indicator of lipid peroxidation). Patients with severe anemia had significantly higher low-molecular weight proteinuria and MDA, and lower urine osmolarity than those with moderate anemia. Our data confirmed the high frequency of renal abnormalities in beta-thalassemia patients and indicated some degree of proximal tubular dysfunction. Severity of the abnormalities correlated with the degree of anemia and were least severe in patients on hypertransfusion and desferrioxamine therapy. This suggested that the damage might be caused by anemia and increased oxidation induced by excess iron deposits.

Renal failure in two patients with Wolfram syndrome.

We describe a Thai family with three children, two of whom presented with Wolfram syndrome, which is a rare syndrome characterised by diabetes insipidus, diabetes mellitus, optic atrophy, deafness and urinary tract dilatation. A girl and her younger brother had insulin-dependent diabetes mellitus at 11 years old with early onset of renal impairment, proteinuria and hypertension. Urinary tract dilatation was demonstrated in both patients. Kidney biopsies were compatible with diabetic nephropathy. Both children also had bilateral sensorineural hearing loss. Optic atrophy with severe loss of vision was detected in the girl and bilateral cataract in her brother. Both patients were HLA DR2 positive. At 16 years old, her creatinine clearance was 16 ml/min/1.73 m2. Her brother's creatinine clearance was 25 ml/min/1.73 m2 at 13 years old. We conclude that renal function should be evaluated in patients with Wolfram syndrome and the cause of renal failure in these patients may be rapid and severe diabetic nephropathy.

Adrenal function after prednisolone treatment in childhood nephrotic syndrome.

Nephrotic syndrome in children is a very common disease in Thailand. Most of the patients respond well to oral prednisolone treatment but side effects of the drug especially adrenal insufficiency remains a threat to all. We studied the adrenal function by studying the response to ACTH stimulation test in 14 Thai children, nine girls and five boys, with idiopathic nephrotic syndrome: immediately, 3, 6 and 9 months after discontinuation of oral prednisolone treatment. Average age on entry to the study was 104.4 months (25-183 months). Prednisolone was given every day for 29 days (6-64 days) then every other day for 542 days (178-1,562 days). Side effects of steroid treatment were gross obesity BMI > 30 (one patient), moderate hypertension (one patient), and marked cushingoid features (two patients). ACTH stimulation tests were normal in 64 per cent of patients within 7 days, 64 per cent at 3 months, 73 per cent at 6 months, and 90 per cent at 9 months after discontinuation of oral prednisolone. We suggest that adrenal insufficiency has to be considered in all children on prolonged prednisolone unit at least 9 months of treatment-free period.

[Neuraminidase induced hemolytic anemia. Experimental and clinical observations (author's transl)]. / Neuraminidase-induzierte Hämolyse. Experimentelle und klinische Untersuchungen

Recently, increasing attention has been focussed on the in vivo action of neuraminidase as possible pathogenetical factor of hemolytic anemia and even hemolytic-uremic syndrome. Neuraminidase action in red cell membranes results in the release of neuraminic acid, and thereby the uncovering of previously hidden receptors, socalled cryptantigens. With special reference to the phythemagglutinin Anti-TAh from the peanut (Arachis hypogae) and the agglutinin Anti-AHP from the albumin gland of the small Helix pomatia we describe some new methods for the detection of these cryptantigens. In addition to the screebubg genagglutination test with Anti-TAh we developed an "Anti-T-consumption test" for quantitative detection of neuraminidase action on red cells. With the purified reagents we developed an indirect fluorescnet antibody method on blood smears for the detection of cryptantigens on single cells. By animal experiments we could show that not only the membranes of red cells but the intima of renal capillaries as well are damaged by neuraminidase. With these new methods we observed 14 patients suffering from hemolytic anemia due to bacterial or viral neuraminidase. Some of these patients developed a hemolytic-uremic syndrome. We believe that the positive reaction with Anti-T Ah should lead to prophylactic heparinization to prevent dissiminated intravascular coagulation. Neuraminidase is the first identified toxin which directly acts on the membranes of red cells and the intima of renal capillaries as well, and thereby in some patients may induce hemolytic-uremic syndrome. Possibly, these results may stimulate the development of further testsystems for the detection of still unknown toxins which are not tested with our reagents, but may equally be involved in the damage of cell membranes.