Biomedical subjects
Publications and source records attributed to A Madani.
Embolotherapy has gained widespread acceptance as the modality of choice for the management of hemobilia because of its safety profile. Our case reports a successful transcatheter embolization of the right hepatic artery aneurysm causing hemobilia in a patient in hemodynamic instability.
An unusually small (8 kD) protein (p8MTCP-1) is coded by the putative oncogene MTCP-1 (also called c6.1B), involved in the translocation t(X;14)(q28;q11) associated with some mature T-cell proliferations. Here, we show by subcellular fractionation and by confocal microscopy that this protein is located in the mitochondria. This localization orientates toward a role of p8MTCP-1 in the mitochondrial metabolism which may be relevant for the oncogenic process.
The t(X;14)(q28;q11.2) translocation is associated with mature T-cell proliferations. Recently this translocation has been shown to implicate the MTCP-1/c6.1B gene on chromosome Xq28, leading to aberrant or overexpressed MTCP-1 transcripts. The potential coding role of this gene was made uncertain by the lack of a long open reading frame in its major transcripts. However, a short 204 bases open reading frame is potentially coding for a 68 amino-acid protein. Here, we show that this open reading frame sequence and the deduced product are well conserved in mouse. A 8 kD protein (p8), which corresponds to the predicted molecular weight was revealed in transient transfectants and in cell lines by Western blotting, using a rabbit antiserum. This product was absent in lymphoblastoid cell lines with deletions of the MTCP-1/c6.1B locus. A dramatic overexpression of p8 was found in leukemic cells from a patient with a t(X;14). This small protein was localized in the cytoplasm by immunofluorescence. In conclusion, MTCP-1 encodes for a cytoplasmic 8 kD product. Its potential role in leukemogenesis is supported by its overexpression in leukemia with t(X;14), but its function remains unknown.
The release of circumsporozoite (CS) protein by Plasmodium falciparum sporozoites was investigated to identify factors regulating this process within infected Anopheles gambiae mosquitoes. The potential for sporozoites to release CS protein in vitro was not dependent upon their site-specific developmental stage (i.e., mature oocysts, hemolymph, salivary glands), their duration in the vector, or their exposure to mosquito-derived components such as salivary glands or hemolymph. The capacity of sporozoites to release CS protein was depressed by mosquito blood feeding during periods of sporozoite migration to the salivary glands, but the effect was only temporary and those sporozoites already in the glands were not affected. Free CS protein in the salivary glands was present in 93.3% of 45 infective mosquitoes. Sporozoites from these same, individual mosquitoes were also tested in vitro for CS protein release. In both cases, the amount of soluble CS protein increased as a function of sporozoite density but the total amount of CS protein per sporozoite became progressively less with increasing numbers of sporozoites. Further experiments showed that sporozoite contact with increasing amounts of soluble CS protein caused a down-regulation of CS protein release. Thus, a primary factor regulating the production and release of CS protein by sporozoites is their contact with soluble CS protein within the mosquito.
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.
Balloon angioplasty (BA) for native coarctation of the aorta (CA) in infants and neonates remains controversial with a high incidence of restenosis. The purpose of this study is to analyze our acute and midterm results for BA of native CA in infants and neonates and try to identify factors that may be predictive of outcome. Between September 1991 and June 1999, 17 patients with CA underwent BA at a median age of 3 months (range 2 weeks--9 months) and median weight of 4.8 kg (range 2.8--7 kg). Fourteen patients had discrete CA and 3 had tubular hypoplasia. All patients were hemodynamically stable prior to BA and no patients had critical coarctation requiring prostaglandin E(1) infusion to maintain ductus arteriosus patency. Seven patients had other associated cardiac defects. All patients had significant initial improvement. The mean peak systolic gradient across the CA improved from 43 +/- 15 mmHg to 10 +/- 8 mmHg (p < 0.001), and the mean minimum diameter of the aortic lumen increased from 2.4 +/- 0.9 mm to 5.2 +/- 1.0 mm (p < 0.001). There was no mortality or major complication. At median follow-up interval of 2.7 years (0.15-7.75 years), 10 (59%) of 17 patients are clinically well and have an upper to lower limb systolic blood pressure difference of <20 mmHg. Seven (41%) of 17 patients developed significant restenosis (5 of these patients underwent repeat BA, which was successful in 3 patients). Four (24%) patients underwent surgical repair at a median age of 4.5 months (3--6.9 months) and a median time interval of 4 months (2--6.5 months) from the initial BA. All 3 patients with tubular hypoplasia type of CA underwent surgical repair. No patients developed aortic aneurysm following initial or repeat BA. All patients who underwent surgical repair were 1 month or less in age at the time of their initial BA. We conclude that BA of native CA in infants and neonates can be performed safely with low mortality and morbidity. It appears to offer the best results in patients who are older than 1 month with discrete CA and a well-developed aortic arch. Further restenosis of the discrete CA can be managed successfully by repeat BA.
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.
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.