Long-term outcome of the difficult nephrotic syndrome in children.

To determine the long-term outcome of nephrotic syndrome (NS) in children, we studied 48 patients with the NS aged seven months to 12 years at onset and followed for a long period (3-9 years). Consanguinity was positive in 31.2%. Patients' history of atopy was present in 25%, while family history of allergy was present in 18 (37.5%) patients. Renal impairment at initial presentation was observed in 12.5% of the patients. Among 32 biopsied patients, 11 (34.3%) had focal segmental glomerulosclerosis and eight (25%) revealed mesangial IgM nephropathy. Outcome at two years of presentation showed 41.6% patients as frequent relapsers, 39.5% as steroid dependent and 18.7% as steroid resistant. Forty-three patients were available for follow-up after ten years of presentation, 22 (51%) patients had complete remission, 15 (34.8%) were steroid dependent, two (4.6%) developed chronic renal failure and two (4.6%) died. Two patients (4.6%) developed insulin-dependent diabetes mellitus, two (4.6%) had cataract and one (2.3%) had documented peritonitis. In conclusion, the high incidence of steroid-dependent, frequent relapses and steroid resistance in children can be explained by different factors, including consanguinity, atopy and severe presentation at onset of disease. We suggest longer initial treatment at onset for this group of patients. The low incidence of infection in this group needs to be addressed in future studies.

Post renal transplantation tubulopathies in children: a 9-year experience at a tertiary care centre.

To evaluate the incidence of tubulopathies in the long-term follow-up of children post renal transplantation, we reviewed the records of 43 patients from 1987-1996. There were 24 (56%) boys. The age of patients at the time of transplant ranged from 2.7 to 15 years. Eighteen children (78%) had transplantation from cadaver donors (CAD). Thirty-two (74%) patients were transplanted in Saudi Arabia and 11(26%) were transplanted abroad. Significant tubular dysfunction developed in 72% of patients. Renal Tubular Acidosis (RTA) occurred in 23/43 (53%) patients. The patients who received CAD grafts required higher mean dose of bicarbonate and longer duration of therapy compared to living related donors (LRD) recipients ( mean dose of 1.7 Vs 0.5 meq/kg/day and mean duration of 18 Vs 3 (1/2) months, respectively). Hypophosphatemia of various degrees of severity (0.4-0.8 mmol/1) was detected in 12 (28%) patients. Those who received CAD grafts required higher mean dose of phosphate and longer period of therapy than those who received LRD grafts. Hypomagnesemia requiring supplemental magnesium therapy occurred in 4 (9%) patients, all received tacrolimus therapy. In four patients with hypomagnesemia, this was mild and transient. Hypokalemia was found in 5 (11.5%) patients; all had CAD grafts. We conclude that tubulopathies were a frequent complication post renal transplantation in our population. They were more severe in the patients who received CAD grafts. However, the defects were controllable and transient.

Paralysis Episodes in Carbonic Anhydrase II Deficiency.

Carbonic anhydrase II (CAII) deficiency is an autosomal recessive disorder manifest by osteopetrosis, renal tubular acidosis, and cerebral calcification. Other features include growth failure and mental retardation. Complications of the osteopetrosis include frequent bone fractures, cranial nerve compression, and dental mal-occlusion. A hyper-chloremic metabolic acidosis, sometimes with hypokalemia, occurs due to renal tubular acidosis that may be proximal, distal, or more commonly, the combined type. Such patients may present with global hypotonia, muscle weakness or paralysis. We report a case of CA II deficiency with recurrent attacks of acute paralysis which was misdiagnosed initially as Guillian-Barre syndrome.

Levamisole Therapy as a Second-line Immunosuppressive Agent in Corticosteroid-sensitive Nephrotic Syndrome in Children.

To evaluate the effect of levamisole therapy combined with corticosteroid in children with nephrotic syndrome and frequent relapses (FR) or steroid dependence (SD), we studied retrospectively 24 children (18 boys and six girls) with a mean age of 32 months. Eleven (46%) patients had FR and 13 (54%) had SD. Levamisole was used for a mean period of 8.5 months. The relapse rate decreased from a mean of 4 relapses/year during steroid therapy alone to 1.3 relapses/ year during the combined therapy in 17 (71%) of the patients. Remission was sustained, after discontinuing levamisole, for more than six months in 11 of the 17 responders (65%), and six patients relapsed after discontinuing levamisole. Seven of the 24 study patients (29%) had no response to levamisole. The seven patients who failed to respond to levamisole and the six initial responders who could not maintain remission after discontinuing the drug were treated with cyclophosphamide for 12 weeks. Eight of these patients (61.5%) had remission sustained for a mean duration of 10 months. The FR patients sustained remission for a longer period of time than SD patients in both groups. Levamisole was more effective than cyclophosphamide in patients with the age of onset of the disease above 2 years (10 patients out of 11 (91%). The side effects of levamisole and cyclophosphamide in our patients were transient and manageable. We conclude that levamisole is a safe and effective drug if combined with corticosteroid therapy in children with nephrotic syndrome. Further studies may be required.

Urinary tract infection and vesicoureteral reflux in saudi children.

This is a retrospective study of 82 children with urinary tract infection (UTI) evaluated for the prevalence of vesicoureteral reflux at a community hospital in Riyadh, Saudi Arabia from 1997 to 2000. There were 73 (89%) girls and nine (11%) boys; 58 (71%) were at an age between 1-5 years, 15 (18%) were between 0-1year and nine (11%) were more than 5 years of age. All patients were documented to have UTI by history and laboratory investigations. There were 29 patients (35%) who had acute pyelonephritis at the initial clinical presentation and 53 (65%) had recurrent UTI. Escherichia coli was the isolated bacterium from urine in 79(96.4%) patients. Thirty-four (41.5%) patients had vesicoureteral reflux (VUR); 17 (50%) had it bilaterally and 14 (41%) had renal scarring. There were 9/82 (11%) patients who had renal scarring without reflux. Twenty-six (77%) of the VUR patients had mild to moderate reflux (grade 1-3) and eight (23%) had severe reflux (grade 4-5). Two patients with bilateral reflux had mild to moderate reflux on one side and severe reflux on the other. The age of the patients with VUR was below one year in 11 (32%), between 1 year and 5 years in 21(62%), and between 6 and 12 years in two (6%) patients. There were 11/ 29 (38%) patients with acute pyelonephritis who had reflux. Follow-up of the VUR patients showed that reflux disappeared without surgical intervention in 15 (44%), improved in two (6%) to lower grade and worsened in two (6%) to higher grade. Seven (20.5%) patients underwent ureteral reimplantation; all of them had recurrent UTI and were more than one year of age. While on chemoprophylaxis, two (28%) of the reimplanted patients developed breakthrough infections and the remaining five (72%) had a radiological picture of chronic pyelonephritis. None of the study patients developed new scars, hypertension or renal failure during follow-up; the duration of follow-up was from 5 months to 3 1/2 years and only seven (20.5%) patients had less than one-year follow-up. We conclude that Saudi children with UTI below 7 years of age have high incidence of reflux and scarring especially in patients presenting with acute pyelonephritis. A multi center study is needed to evaluate the size of the problem and its complications in the Saudi children besides screening of the siblings of patients with reflux.