Congenital nephrotic syndrome: a clinico-pathologic study of thirty children.

BACKGROUND: Congenital nephrotic syndrome is an uncommon disorder that may be caused by several diseases. These may be inherited, sporadic, acquired or part of a general malformation syndrome. METHODS: We reviewed the clinical characteristics, pathologic findings, and results of medical management in 30 infants who presented to Jordan University Hospital with congenital nephrotic syndrome in the years 1989 to 1999. RESULTS: Most patients (80%) had parents who were consanguineous. Most patients (80%) were born premature, with an average gestational age of 36 weeks. Most infants (77%) presented the nephrotic syndrome in the first three months of life and 26 (87%) had significant growth retardation. Twenty-five verified episodes of serious bacterial infections occurred in 18 patients. Antibiotic therapy however was successful in all these episodes. Light microscopy of the renal biopsies was consistent with the Finnish type of congenital nephrosis in most patients (83%). Chronic renal insufficiency developed in 17, and five of them needed chronic peritoneal dialysis. Most patients were given albumin transfusion and diuretic therapy especially during episodes of severe edema. Captopril alone or in combination with ibuprofen was given to eight patients, but without a response in any of them. All patients died before the age of 5 years. Most deaths occurred at an average age of 15 months (range 1-60). CONCLUSION: The Finnish type of congenital nephrosis was the most common type in our patients, most of whom died within a few months of the onset of disease. In the developing countries, the management of patients with congenital nephrosis may have to be different from that in the developed countries in view of the high cost of medical management, poor outcome, high risk of serious complications, and high mortality rate.

Treatment of idiopathic nephrotic syndrome with cyclosporin A in children.

Twenty-two children (15 boys, 7 girls), aged from 1 to 9 years (mean 4.6 years) at the onset of idiopathic nephrotic syndrome (INS) received cyclosporin A (CsA) because of steroid toxicity or failure to respond to steroids. CsA was given at an initial dose of 5 mg/kg body weight per day, and adjusted to maintain whole blood trough levels at 60 to 180 ng/ml (HPLC). The duration of treatment ranged between 4 and 33 months. In patients who responded to CsA, treatment was continued for 6-33 months (average 12 months). Treatment was stopped it found to be ineffective after four months. All patients had normal kidney function at the onset of CsA therapy. Of the 22 cases 10 were frequent-relapsing, steroid-responsive patients who suffered serious side effects of steroid therapy. Six steroid-responsive patients were dependent on high-dose prednisolone for maintenance of remission. Twelve patients were steroid-resistant (SRT), eight of them with mesangial hypercellularity (MES), three focal segmental glomerulosclerosis (FS-GS), and one minimal change disease (MCD). Seventeen patients (77%) responded favorably to CsA, 13 of them with complete remission, three with partial response (two of whom had MES, and one steroid-resistant FSGS), and one relapsed while on CsA. Only five patients in the whole study group showed no response to CsA, two of them had steroid-resistant FS-GS, both of whom developed renal failure in follow-up, and the other three had MES. In conclusion, therapy with CsA may be helpful in resolving nephrotic syndrome in SRT patients. CsA can be used to maintain remission in frequently relapsing nephrotic children. Patients who respond to CsA may have a lasting remission after the cessation of therapy.

Abdominal angina and intestinal gangrene--a catastrophic presentation of arterial fibromuscular dysplasia: case report and review of the literature.

A 5-year-old boy presented with episodic, postprandial abdominal pain and hypertension. A few days after the onset of symptoms, the pain became more severe, and progressed into a picture of acute abdomen and intestinal obstruction. Urgent laparotomy findings showed the presence of small bowel gangrene. Pathology findings of the superior mesenteric artery (SMA), which was found to be occluded, showed intimal fibroplasia. The patient died 7 weeks after the onset of symptoms.

Captopril test in children with reflux nephropathy.

We studied the plasma renin activity (PRA) and blood pressure (BP) responses to a single oral dose of captopril in children with renal scarring due to vesicoureteral reflux. The test was performed on 19 children, of whom 13 were normotensive and 6 hypertensive. Basal PRA was within the normal range for age for all except 1 patient. Mean basal PRA values for normotensive and hypertensive patients were not significantly different. At 90 min post captopril, the mean PRA increase was 3.61 ng/l per second (SD = 7.07) in the normotensive group but only 0.77 ng/l per second (SD = 0.83) in the hypertensive group. In 11 patients the average systolic BP 60-90 min post captopril was 0.9%-12.4% lower than the basal value. Diastolic BP decreased after captopril administration in 10 patients and increased in 9. The changes in mean arterial pressure and PRA between 0 and 90 min post captopril were inversely correlated (r = 0.605, P less than 0.01). Because of the PRA responsiveness in the normotensive patients with renal scarring, we suggest that there was activation of the renin-angiotensin axis in this group. However, it is not apparent whether such patients are more at risk of developing hypertension at a later time.

Use of the captopril test to assess renin responsiveness in children with hypertension and renal disease.

The captopril test was performed on 49 children of whom 36 were hypertensive, and the remainder were normotensive but were at risk for developing hypertension because of scarred kidneys secondary to vesico-ureteral reflux. Blood pressure (BP) was monitored in fasting supine patients throughout the duration of the test. Blood was taken for measurement of plasma renin activity (PRA); then captopril (0.7 mg/kg of body weight) was administered orally. A second blood sample was taken for PRA at 90 min postcaptopril. The mean (SEM) PRA at 90 min was 11.90 (4.01) ng/l/s [42.84 (14.44) ng/ml/h] in 7 patients with renovascular disease. In 4 patients with essential hypertension corresponding values were 0.88 (0.38) ng/l/s [3.17 (1.37) ng/ml/h]. Patients with other renal diseases showed variable values. Some individuals had PRA values as high as those of patients with renovascular disease, but the etiology of their hypertension was usually clinically evident. Our preliminary data would suggest that the captopril test may help differentiate between patients with essential hypertension and those with renovascular disease, or may help select patients that should be followed up by more definitive diagnostic procedures.

Use of the captopril test to assess renin responsiveness in children with hypertension and renal disease.

The captopril test was performed in 49 children of whom 36 were hypertensive, and the remainder were normotensive but at risk for developing hypertension because of scarred kidneys secondary to vesicoureteral reflux. The blood pressure was monitored in fasting supine patients throughout the duration of the test. Blood was taken for measurement of plasma renin activity (PRA), then captopril (0.7 mg/kg body weight) was administered orally. A second blood sample was taken for PRA measurement 90 min after captopril administration. The mean PRA at 90 min was 11.90 +/- (SEM) 4.01 ng/l/s (42.84 +/- 14.44 ng/ml/h) in 7 patients with renovascular disease. In 4 patients with essential hypertension, the corresponding value was 0.88 +/- 0.38 ng/l/s (3.17 +/- 1.37 ng/ml/h). Patients with other renal diseases showed variable values. Some individuals had PRA values as high as those of patients with renovascular disease, but the etiology of their hypertension was usually clinically evident. Our preliminary data would suggest that the captopril test may help differentiate between patients with essential hypertension and those with renovascular disease or may help select patients that should be followed up by more definitive diagnostic procedures.