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Biomedical subjects

R Habib

Publications and source records attributed to R Habib.

At least 109 records · Page 6Linked to original sources
PubMed1985

The nephropathy associated with male pseudohermaphroditism and Wilms' tumor (Drash syndrome): a distinctive glomerular lesion--report of 10 cases.

We report on 10 children, less than 2 years of age, who presented with a genuine type of glomerulopathy: diffuse mesangial sclerosis. In 5, the nephropathy was associated with male pseudohermaphroditism (MPH) and Wilms' tumor (WT); in 3 with MPH and in 2 with WT. The nephropathy was characterized by its very early onset, between the age of 2 weeks and 18 months. Eight patients presented with a nephrotic syndrome with (7 cases) or without (1 case) hypertension. All, but one, who is in advanced RF at 11 years of age, progressed to chronic or end-stage renal failure (ESRF) within a few months to 2 years from the onset. One additional child presented with advanced renal failure at the age of 8 months and the last one, who was hypertensive, developed an anuria related to thrombosis of renal veins at 1 year of age. Drash syndrome is characterized by the association of a "nephron disorder" with MPH and WT. We propose, on the basis of our histological findings, to extend the concept of Drash syndrome to patients who, in addition to the nephropathy, have either WT or MPH and to consider the distinctive glomerular lesions presented by all these patients as their common denominator. The pathogenesis of this glomerulopathy is obscure. Its early onset, its association with a dysembryoplastic tumor and/or with gonadal dysgenesis both suggest an antenatal dysgenetic process.

Disorders of Sex Development↗
PubMed1985

[Alpha-1-antitrypsin deficiency and renal involvement].

In a young girl with alpha 1-antitrypsin deficiency (Pi Z), cirrhosis, hypocomplementemia and proteinuria, renal biopsy showed features of type I membranoproliferative glomerulonephritis. Deposits contained IgA, IgG, IgM, Clq, C4 and C3 but not alpha 1-antitrypsin. A review of the literature found 10 similar cases. Type I membranoproliferative glomerulonephritis was present in all. The respective roles of alpha 1-antitrypsin deficiency and hepatic disease in the pathogenesis are discussed; membranoproliferative glomerulonephritis is probably the consequence of the chronic liver disease.

Child↗
PubMed1985

Berger's disease in children. Natural history and outcome.

The clinical course and outcome of 91 children less than 15 years of age at onset and followed for at least 1 year have been retrospectively analyzed. The course has been characterized by recurrent macroscopic hematuria in 74 patients, by proteinuria-microscopic hematuria and a single episode of macroscopic hematuria occurring either at onset or a few months later in 8, by proteinuria-microscopic hematuria in 7, and by proteinuria only in 1. Lastly, one patient showed rapidly progressive renal failure. Four groups were identified by light microscopy: minimal glomerular changes (26), focal and segmental glomerulonephritis (41), pure mesangial proliferation (3) and proliferative glomerulonephritis with crescents (21). A good correlation was found between the glomerular lesions observed by light microscopy and the outcome. In this series we have not observed a dramatic clinical deterioration suggesting a transformation from one histologic type to another, as reported by others. None of the 70 patients belonging to the first three groups has impaired renal function but two with focal and segmental glomerulonephritis have developed hypertension. Although the clinical course is benign, many patients have, at the last observation, an abnormal urinalysis characterized by microscopic hematuria and/or mild proteinuria; the proteinuria is over 1 g/24 h in six patients with focal and segmental glomerulonephritis. Ten patients remained in clinical remission for several years, but mesangial IgA deposits were still present in the only patient who had a repeat biopsy while in remission. In contrast, none of the patients with proliferative glomerulonephritis with crescents has had a prolonged remission. Six patients developed terminal renal failure 0.7, 0.11, 2, 4, 8 and 10 years after onset. Two additional patients are in moderate chronic renal failure with hypertension 10 and 12 years after onset. Most children show a persistent nephropathy, (in five proteinuria is over 1 g/24 h), and two of them have developed hypertension. Therapeutic trials using drugs with side-effects should, therefore, be used only in this group of patients.

Adolescent↗
PubMed1985

[Diffuse arterial calcified elastopathy].

Diffuse arterial calcified elastopathy was observed in 6 pediatric patients presenting with severe renovascular hypertension. Renal ultrasonography showed a characteristic pattern, the dotted corticomedullary junction, related to the increased echogenicity of the interlobar and/or arcuate arteries. Superficial temporal artery biopsy demonstrated the presence and the extension of the calcifying process involving the elastic layers of the muscular arteries. This clinicopathological syndrome may be heterogeneous from an etiological point of view: etiologic investigations led to the diagnosis of pseudoxanthoma elasticum in 2 patients; no etiology could be found in the others.

Arteries↗
PubMed1985

[Familial Budd Chiari syndrome and glomerular involvement].

We report a case of a child with familial Budd-Chiari syndrome and proteinuria. Renal biopsy disclosed predominant arteriolo-capillary endothelial and mesangial lesions suggesting a microangiopathy. This may be due to hypoxemic damage with polycythemia and high venous pressure resulting from precapillary pulmonary arteriovenous shunts.

Budd-Chiari Syndrome↗
PubMed1984

[Treatment of childhood hemolytic-uremic syndrome with urokinase. Cooperative controlled trial].

The results of a controlled therapeutic trial comparing 2 groups of patients presenting with hemolytic-uremic syndrome (HUS) are reported. Group A (15 children) was given urokinase (UK) and heparin; group B (18 children) received no treatment. Ages of patients, the delay before admission, the severity of anemia, thrombocytopenia and initial renal failure were similar in both groups. UK was responsible for bleedings in 12 children, minimal in 8, severe in 4. No child died in group A, 3 children died in group B (n.s.). Durations of hemolysis, thrombocytopenia and anuria were similar in both groups. Long-term evolutions of renal function and arterial pressure were comparable in both groups. Needle kidney biopsy (26 cases) showed cortical necrosis in 3 children of group A and in 2 of group B, and glomerular thrombotic microangiopathy in 10 children of group A and in 11 of group B. The average ratio of injured glomeruli was 40 (19 to 80) in group A, and 38 (21 to 75) in group B. Two children in group A and 3 children in group B presented with 50 to 80% of glomerular lesions. This trial suggests that UK is of no significant value in the treatment of HUS.

Adolescent↗
PubMed1982

Alport's syndrome: experience at Hôpital Necker.

We review the characteristic morphologic features identifiable by electron microscopy that have been described in patients presenting with Alport's syndrome. They are diffuse thickening and splitting of the glomerular basement membrane (GBM), which are either isolated or associated with thinning. In occasional cases, only diffuse thinning can be seen. Our study of 100 families followed in Necker's hospital, of which 60 patients have had electron microscopic examination of their renal parenchyma, demonstrates that these GBM changes are highly suggestive of Alport's syndrome. All the patients included in the study fulfilled the following clinical criteria: familial incidence, nerve deafness in the propositus or in another member of the family, renal disease with progression to renal failure in the proband or in another member of the kindred. Although a failure in the proband or in another member of the kindred. Although a normal GBM was found in five patients, the GBM changes should be one of the criteria for the definition of the syndrome. Results dealing with a few other problems raised by this syndrome are reported. They concern the antigenicity and the biochemical composition of the GBM, the incidence of macular and perimacular changes, and the genetic transmission of the disease. It is concluded that Alport's syndrome is genetically heterogeneous and that the GBM ultrastructural changes are observed in most patients whatever the type of genetic transmission.

Adolescent↗
PubMed1982

[Comparative experimental study of the renal consequences of vesico-ureteric reflux and of ureteric obstruction in the sheep foetus (author's transl)].

The authors sought to determine what links existed between foetal vesico-ureteric reflux and renal dysplasia. Using sheep with operation upon the foetuses in utero, at a fairly early stage of gestation, the following were obtained: 1) Foetal vesico-ureteric reflux by unilateral excision of a ureteric meatus. 2) Stenosis of the foetal ureter by ligation. In the first case, no renal dysplasia occurred. In the second case, dysplasia-type parenchymatous lesions were found. This led them to conclude that the direct relationship between isolated foetal vesico ureteric reflux created experimentally in a normal ureter (without any additional functional or organic obstructive factor) and renal dysplasia could not be demonstrated in the sheep foetus.

Animals↗