Henoch-schonlein purpura in children: A 17-year experience.

A descriptive study of one hundred and five Henoch-Schonlein purpura (HSP) patients (57 males, 48 females) treated during 1987-2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital, was conducted. The male to female ratio was 1.2:1. The mean age of the patients was 7.1 years (range 2-15). Most patients lived in Bangkok and the central region of Thailand. HSP most commonly occurs in the rainy season. Clinical manifestations were rash (100%), arthralgia (61.9%), arthritis (25.7%), abdominal pain (66.7%), gastrointestinal bleeding (14.3%: stool occult blood 11.4% and hematemesis or melena 2.9%), nephritis (37.1%: microscopic hematuria 29.5%, gross hematuria 7.6%), proteinuria (23.8%), nephritic syndrome (1%), and hypertension (1%). The most characteristic rash was purpura, mainly on the lower extremities. Arthritis and/or arthralgia commonly affected feet ad ankles. The abdominal pain was commonly localized at epigastrium and umbilical area. Gastrointestinal complications included upper gastrointestinal bleeding, 34.3% developed recurrent symptoms including abdominal pain, nephritis, and rash mostly occurring within the first 3 months after the initial resolution (range 2 days-9.6 years). The mainstay of management was supportive care. The patients with severe abdominal pain received prednisolone. Prednisolone and cyclophosphamide were only given to serve nephritis patient with a good outcome.

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.

Primary hyperoxaluria: Cases report.

Primary hyperoxaluria is a rare disease characterized by recurrent calcium oxalate nephrolithiasis and nephrocalcinosis. The most diagnostic laboratory finding if an increased amount of urinary oxalate. The inheritance is presumed to be autosomal recessive but autosomal dominant has been repoeted. Here are reported cases of a family: a boy, his brother, and his father. The boy, the most severely affected died at the age of 8 years. His 6-year-old brother, treated by 6 episodes of Extracorporeal Piezo Electric Lithotripsy (EPL); followed with high doses of pyridoxine and thiazides orally, is still alive with normal renal function. His father was treated by 6 episodes of EPI but without medication. The authors suggest that investigations for metabolic causes should be done in children with positive family history of nephrolithiasis.