Pediatric systemic lupus erythematosus in Siriraj Hospital.

A descriptive study of one hundred and one pediatric patients with systemic lupus erythematosus treated between July 1985 and March 2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital was conducted. According to existing database, there were a total of 181 patients, 101 of them (55.8%) had available data for review. The female to male ratio was 6.2:1. The mean and median ages of onset were 9.7 +/- 2.8 and 10 +/- 2.2 years, respectively (range 4-14 years). The clinical presentations were renal involvement in 87 patients (86.2%), skin and mucocutaneous involvement 77 patients (76.3%), hematological abnormalities 74 patients (73.4%), musculoskeletal involvement 32 patients (31.7%), prolongedfever 24 patients (23.8%), neuropsychiatric symptoms 21 patients (20.8%), gastrointestinal involvement 20 patients (19.8%), cardiac involvement 14 patients (13.9%), lymphadenopathy 13 patients (12.9%), and pulmonary involvement 7 patients (6.9%). The most common renal, skin and mucocutaneous, and hematological manifestations were proteinuria, malar rash, and anemia, respectively. Lupus nephritis with WHO class IV was the most common histopathological finding of the initial renal biopsies. The most common neuropsychiatric, gastrointestinal, cardiac, and pulmonary involvements were seizure, hepatomegaly, pericarditis, and pleuritis, respectively. Ninety-two percent of patients reported as having significant ANA positive results using rat liver tissue as a substrate. Sixty-six out of 94 patients (70.2%) had positive test result of Anti-dsDNA. In conclusion, the age at onset, clinical manifestations and laboratory investigation results of SLE in children at Siriraj Hospital are comparable to other studies in the Country and also to other Asian and Western studies.

Difficult-to-treat nephrotic syndrome: management and outcome.

A retrospective study was performed in 68 patients diagnosed as having idiopathic nephrotic syndrome with steroid-dependent, steroid-resistant or frequent relapse subtypes at the Department of Pediatrics, Siriraj Hospital during Jan 1996-Dec 2004. Male to female ratio was 3.3:1 and mean age (+/- SD) was 8.4 +/- 3.5 years. Mean follow up time (+/- SD) was 47.4 +/- 30.5 months. Renal biopsy was done in 60 patients, showing IgM nephropathy in 73.3%. Fifty-four patients (79.4%) received cyclophosphamide at a dose (+/- SD) of 2.2 +/- 0.5 mg/kg/d for 11.6 +/- 3.4 weeks. Negative proteinuria at 1 year was found in 70% and prednisolone was discontinued in 52%. Leucopenia was found in 9.2%. At last follow up, 34% of the patients were still in remission. Enalapril was prescribed in 50 patients for 12.4 +/- 10.0 months. Thirty-six patients also received cyclophosphamide. Remission at 1 year was achieved in 66% and prednisolone discontinued in 28%. Twelve patients (24%) were still in remission at last follow up. The results of 3 regimens: cyclophosphamide, enalapril, and cyclophosphamide plus enalapril were compared using chi-square test. Remission was significantly better in cyclophosphamide group (p = 0.014). Dipyridamole was prescribed in 14 patients due to thrombocytosis. Only 2 of 14 patients achieved remission although 11 patients received cyclophosphamide plus enalapril, and another 2 patients received only cyclophosphamide. Complications included hypertension (44%), cataract (40%), glaucoma (15%), short stature (17.6%), and obesity (5.9%). Recurrent infection was found in 69%, including dental caries (16.29%), urinary tract infection (14.7%), intestinal parasitic infestration (10.3%), respiratory tract infection (8.8%), and skin infection (7.4%). Chronic renal failure was found in 3 patients and portal vein thrombosis was found in 1 patient. We suggest that cyclophosphamide should be used as first line drug in difficult-to-treat nephrotic syndrome patients. Enalapril may be beneficial in some patients. Thrombocytosis may be associated with poor response to both medications. Difficult-to-treat patients also need long-term follow up and surveillance for complications due to disease and/or treatment.

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.

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.

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.

Urinary tract infection in Thai children.

A retrospective analysis was made of the hospital records of 122 children below 12 years of age who presented at the Dept of Pediatrics, Siriraj Hospital, May 1989 to April 1993 with first episodes of urinary tract infection (UTI). The male to female ratio was 1:1.3. The largest group of patients were under 1 year of age (23%). In young infants signs and symptoms were frequently non-specific. Urinary symptoms, were found in only 18%. Older children had more specific symptoms i.e. pollakiuria (24%), gross hematuria (22.3%) and dysuria (21.3%). Urine culture was done before antibiotics were given in 101 patients (83%) with positive results in 52 (51.5%) of them. E. coli was the most common organism identified (59.6%). Responses to empirical antibiotics were good with parenteral ampicillin (78.6%), gentamicin (83.3%), and ampicillin combined with gentamicin (90.9%). Cotrimoxazole orally was used with a response of 63%. Radiological investigations of the KUB were performed in 54 patients and abnormalities found in 25 (46.3%) in which hydronephrosis was the most common (13 patients). Vesicoureteric reflux (VUR) was found in 7 patients. The highest prevalence of KUB anomalies and VUR was found in the under 1-year-old group. Mean duration of follow up was 7.5 months. Recurrent infection was found in 18 patients, 2 of them were on antibiotics prophylaxis at the time of infection. Hypertension was also noted in 2 patients. The authors suggest that radiologic evaluations should be done in all children with first episodes of UTI, especially if they are younger than one year. Long term follow up is also recommended to prevent recurrent UTI and complications which may lead to permanent renal damage.

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.