Renal transplantation in Thai children.

Renal transplantation in children has been performed for over 25 years in developed countries but has never been reported in Thai children. We report an 8 year-old girl with end-stage renal disease due to chronic glomerulonephritis who had been transplanted at Siriraj Hospital. She was treated with continuous peritoneal dialysis for 9 months and developed several episodes of bacterial peritonitis and hypertension. She was transplanted using her father’s kidney and her serum creatinine level was normal within 7 postoperative days. The patient developed severe hypertension which was controlled with 4 antihypertensive agents. Five weeks postoperation her new ureter leaked. Although surgery was performed with internal stent placed, the patient continued to have urinary leakage and ureteric reconstruction using bladder flap or her own ureter will be done later. The patient’s renal function was normal on follow up.

IgA nephropathy in Thai children.

We retrospectively studied 11 patients with IgA nephropathy admitted into the Department of Pediatrics, Siriraj Hospital from 1983 to 1993. These patients represented 2 percent of 552 patients with glomerular disease admitted at the same duration. Female to male ratio was 1.75 : 1. Average age of onset was 98.5 months (range 46-144 months). Common manifestations included macroscopic hematuria (72.7 percent), microscopic hematuria 1 patient (9.1 percent), edema (27.3 percent) and nephritic syndrome (18.2 percent). Proteinuria was found in 4 patients (36.2 percent) and 3 patients (27.2 percent) had increased serum creatinine concentration at the time of diagnosis. Serum immunoglobulin A was increased in 1 of 5 patients studied (20 percent). Percutaneous renal biopsy was performed in all cases. Nine patients had diffuse proliferative glomerulonephritis, the rest had focal segmental glomerulonephritis. Both patients with nephritic syndrome were given oral prednisolone and 1 patient with proteinuria and severe renal pathology was given the combination of prednisolone and cyclophosphamide, all responded well. Seven patients had been followed up for 1 to 9 years. Three patients (42.8 percent) had increased serum creatinine. We suggest that urine protein and renal function should be measured in all patients with IgA nephropathy at diagnosis and followed up periodically. We also recommend the use of prednisolone in patients with IgA nephropathy who presented with nephritic syndrome.

Nephropathy associated with wilms’ tumor (drash syndrome).

Nephropathy associated with Wilms’ tumor (Drash syndrome) is an uncommon disease with well-documented clinical course and prognosis regarding kidney function. We report a girl with Drash syndrome who presented with Wilms’ tumor and aniridia at the age of one year. Left nephrectomy with chemotherapy and radiotherapy were given. She remained in good health with normal renal function during the 7 years period of follow up. At the age of 9 years, she developed acute nephrotic syndrome, hypertension, and renal failure (GFR 15ml./min/1.73 square meter). Abdominal ultrasound revealed no evidence of Wilms’ tumor recurrence. Prednisolone, cyclophosphamide, and antihypertensive drugs were given. Renal function returned to normal (GFR 80/ml/min/1.73 square meter) at one month and serum albumin at 3 months after treatment. The case of nephropathy associated with Wilms’ tumor presented here has an atypical clinical manifestation as compared with the literature concerning it may throw some light on the pathogenesis of glomerulopathy in Drash syndrome.