Changing perspective of lipoid nephrosis in Iranian children.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to E Bodaghi.
Explore the source record for details and available documents.
Between 1972 and 1984 148 children with urolithiasis were studied and managed at the University Clinic Children's Hospital of Teheran. In 125 children the calculi were in the upper and in 23 children in the lower urinary tract. The maximum incidence was between the ages of 5-8 and 12 years. 25 children had malformations and 16 had metabolic disorders. Cystinuria was observed in 6 and xanthinuria in 3 cases. The main constituents of calculi analyses in the upper urinary tract were calcium oxalate followed by ammonium acid urate. In the lower urinary tract ammonium acid urate and oxalate were seen with equal frequency, followed by uric acid. 16 children had staghorn calculi with an age profile of 5-13 years. Predominant symptoms were flank pain and gross hematuria. In 4 cases the calculi were bilateral. The calculi were removed successfully by pyelotomy and extensive pyelolithotomy. In 2 cases with more branched-out staghorn calculi and separate fragments, a logitudinal extensive nephrotomy was performed. In the absence of recurrent stones, renal growth and function were satisfactory postoperatively. In the majority of the cases the analyses of the staghorn calculi revealed phosphate.
The authors report the case of a 33 month-old child who presented some of the classical symptoms of the Henoch-Schoenlein purpura - arthritis, purpuric lesions of the lower extremities - associated with the full-blown picture of a post-infectious glomerulonephritis - low level of C3, proliferation of mesangial cells, exudation with large number of leukocytes, and C3 glomerular deposits. These findings and others previously described suggest that the Henoch-Schoenlein purpura is a syndrome and that some of its manifestations may occur in patients with post-infectious glomerulonephritis.
Among 25 cases of double ureter which required surgical treatment, six were accompanied by ureterocele. Interpelvic anastomosis was performed in two cases with uretero-ureteral reflux. En bloc reimplantation was performed for ten double ureters, three of which had common ureteral orifice with reflux in both ureters. In three other cases there was a common segment with low bifurcation and ureteroureteral reflux which had to be excised in order to create two ureteral orifices in the bladder. Heminephrectomy with excision of the ureterocele combined with a simultaneous reimplantation of the ipsilateral ureter was performed in three cases. These cases were accompanied by a large ureterocele and reflux in the ipsilateral ureter. Upper-pole nephrectomy and partial ureterectomy without excision of the stump and ureterocele was performed in one case of small ureterocele without reflux. Excision of the ureterocele combined with en block reimplantation was performed in one case with relatively well preserved renal tissues. Follow-up results were satisfactory in the majority of the cases.
A total of 411 children, aged from 0.3 to 18 years, suffering from glomerular diseases, were studied by renal biopsy between 1976 and 1985. The clinical presentation included nephrotic syndrome (79% of cases), renal failure (43%), and arterial hypertension (38%). In all, 177 cases presented with primary nephrotic syndrome; all had complicated courses and most were either corticosteroid-dependent or -resistant. Only 26.6% had minimal change disease on renal biopsy; 56.5% had focal-segmental sclerosis; and immunofluorescent deposits were observed in half of the group. Acute poststreptococcal (36 cases), mesangiocapillary (80 cases), and lupus (34 cases) glomerulonephritis occurred frequently; IgA glomerulopathy (10 cases) and haemolytic uraemic syndrome (6 cases) were uncommon. Glomerular crescents were observed in 71 cases. These observations illustrate the types of glomerular diseases seen in Iranian children.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
We describe the case of a 22-month-old girl who presented with a severe nephritic-nephrotic syndrome associated with arterial hypertension, remained symptomatic throughout the follow-up period and met advanced renal failure at 6 3/4 years of age. The initial renal biopsy revealed a mesangiocapillary glomerulonephritis with mesangial deposits of immunoglobulins (IgA, IgG, IgM), C3 and fibrin, extending into the capillary wall. Diffuse glomerular sclerosis was observed in the second biopsy. To our knowledge there are no reports of similar cases in the literature.
The incidence of calculus anuria is relatively high in Iran. Eighteen children with this picture were studied in our departments between 1972 and 1984. The cause of anuria was bilateral obstruction by the calculi (14 cases), unilateral obstruction with a nonfunctioning kidney (3 cases) or aplasia on the other side (1 case). As demonstrated by our study, the cause of stone formation was a metabolic disorder in 50% of the children. This indicates that with anuria created by calculi formation, more consideration should be given to the possibility of metabolic disorders than has so far been the case. Further research should therefore be carried out in this respect, particularly in cases of the lower age range with indications of parental consanguinity, multiple and bilateral calculi, repeated calculi and repeated admissions, which were frequently observed by us.
Juvenile nephronophthisis is a common cause of end stage renal failure in children and adolescents. The early occurrence with rapid progression to advanced renal failure has not previously been described. In this report we present three babies in whom failure to thrive, dehydration and a renal concentrating defect presented soon after birth; they were subsequently investigated for renal failure during the first year of life. In two cases the association with congenital hepatic fibrosis was noted. A family history of nephronophthisis was not present but the parents were consanguineous and one sibling had died in infancy.
Four children are described with a severe acute streptococcal glomerulonephritis. Olyguria, long term renal failure, arterial hypertension, nephrotic syndrome, unconsciousness, marked anemia, abdominal pain and gastrointestinal bleeding were the most prominent findings. The renal biopsy revealed a diffuse endocapillary glomerulonephritis with crescents and vasculitis. One patient had demonstrated multiorgan vasculitis. The patients were managed by conventional methods, three of them received anticoagulants of whom, two association of prednisolone-cyclophosphamide. All recovered.
Three hundred and ten children with primary nephrotic syndrome were studied since 1972. 190 patients are classified as nephrosis (61%); including minimal glomerular lesions (22.9%), diffuse mesangial proliferation (6.77%) and focal and segmental sclerosis (31.6%). Corticoresponsivity was limited: 53.75% with a higher success rate in minimal glomerular lesions: 74% versus 38% of diffuse mesangial proliferation and 42.5% focal and segmental sclerosis. Immunosuppressive drugs were used for 96 patients corticodependent or resistant. They were more effective in dependent than resistant cases 94% versus 38%. A total of 149 cases were followed (mean: 3.9 years). In the last evaluation 67% were in remission, 8.10% in chronic renal failure and 24.8% with other abnormalities.
Explore the source record for details and available documents.