Systemic lupus erythematosus in Thai children: clinicopathologic findings and outcome in 82 patients.

OBJECTIVES: To define the patterns of clinicopathologic findings and to identify the risk factors for renal failure and mortality of childhood-onset systemic lupus erythematosus (SLE) in Thailand. MATERIAL AND METHOD: The study is a retrospective analysis of clinical manifestations, laboratory data, and pathologic findings, treatment modalities, and outcome of 82 patients with biopsy-proven lupus nephritis (LN) with disease onset between I January 1987 and 31 December 1997. All children developed these first manifestations at the age 13 years or under RESULTS: Sixty-four (789%) patients were females and eighteen (22%) were males (ratio female/male = 3.5:1). The patients were followed for a mean period of 53.6 months (range 1 -141). The mean age at disease onset was 9.2 years (range 2-12.6). Class-IV LN, observed in 40 (48.8%) patients, was the most frequent histopathology on initial renal biopsy. Less frequent findings were class-II (30.5%), V (14.6%), I (3.7%) and III (2.4%) LN. Based on the renal histopathology and clinical presentations, patients were treated with corticosteroids alone or in combination with azathioprine or with intravenous cyclophosphamide (CYC). Methylprednisolone pulses were given in patients with clinically more severe disease. Follow-up biopsies, performed in 12 patients, showed no change in 4 patients, and were progressive in 8 patients. On final clinical evaluation, 20 patients died, 65% died from serious infections, 15% from cardiopulmonary complications, and 10% from end stage renal disease. As the whole group, survival rates were 89% and 74% at 12 and 60 months, respectively. The 5-year patient survival in class-II, class-IV and class- VLN patients were 83%, 67% and 64%, respectively. Within the group of class-IV LN, the 5-year survival in the patients treated with intravenous CYC was significantly better than those receiving prednisolone with or without azathioprine. Five-year kidney survival rates from the time of diagnosis to the endpoints of terminal renal failure were 94% for the whole group, and 100%, 96%, 91% in the class- V, class-II, and class-IV group, respectively. Initial presence of hypertension, hematuria, renal insufficiency were independent factors significantly associated with lower patient survival probabilities. There was no association of either patient and kidney survival with gender, age, cytopenia, and autoantibody level. CONCLUSIONS: Infectious complications were the most common cause of morbidity and mortality in our pediatric patients with SLE. The immunosuppressive agents used to treat SLE seemed to be a major contribution to the patient survival. With judicious use of corticosteroid, intravenous CYC in severe SLE showed superior efficacy over oral prednisolone with or without azathioprine.

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.

Percutaneous renal biopsy in children.

The authors studied the percutaneous renal biopsies performed in the Department of Pediatrics, Siriraj Hospital from January 2000 to March 2001 in order to evaluate the safety and benefit of the procedure. Eighty-five patients (90 episodes) were included in the study, aged 7.8+/-3.7 year (range 16 months to 16 years), with a male to female ratio of 1.2:1. Nephrotic syndrome (42.3%) and systemic lupus erythematosus (23.5%) were the two most common indications for biopsy. The kidney was localized by ultrasound prior to the procedure in nearly all cases (97.7%). Premedication with Ketamine was adequate in most patients (91.1%). A modified 13 G Vim-Silverman needle was used to obtain 1-4 biopsy cores. The mean number of glomeruli obtained was 44.0+/-29.9, with failure to obtain renal tissue in 6 episodes (6.6%). Percutaneous biopsy was performed twice in one patient without success and the patient eventually underwent an open biopsy. The most common complication was hematuria (74.4%), of these, gross hematuria was found in 23.3 per cent. Blood transfusion was needed in 2 patients, one of them also needed embolization to control bleeding. Transient hypotension occurred in 1 patient. Transient hypertension occurred in 6 episodes (6.6%). Muscle twitching occurred in 2 episodes and was treated with diazepam intravenously. Hypertension and muscle twitching only occurred in those who received ketamine. The Clinical Benefit Score was 2 (information yielding a definite diagnosis and/or prognosis, alternatively allowing a change in, or support of, therapy) in 89.4 per cent. It was concluded that the present practice of renal biopsy is safe, with high clinical benefit score. It remains to be studied whether an ultrasound guidance biopsy with a newer biopsy device will lower the incidence of complications even further.

Persistent hypertension in Thai children.

We reviewed the records of 36 children with persistent hypertension who were admitted to the Department of Paediatrics, Siriraj Hospital between 1987 and 1993. The male to female ratio was 1.4:1, and mean age at diagnosis was 7.6 years. The cause of hypertension in 16 children (44.44 percent) was renal parenchymal disease, while four children (11.11 percent) had renovascular disease, five chil-dren (13.9 percent) had cardiovascular disease and eight children (22 percent) had primary hypertension. Miscellaneous causes were found in three (one adrenal gland tumor, one Wilms’ tumor and one unknown). Hypertension was controlled by medication alone in 70 oercent and combined medical and surgical treatment in 20 percent of patients. Hydralazine, furosemide and propranolol were most common drugs used. Five patients had hypertensive crises. Five patients (13.9 percent) died; the cauese of death were multifactorial and were not directly related to hypertension.

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.

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.