Biomedical subjects
Publications and source records attributed to H Sevestre.
A case of multiple Glisson's capsule phleboliths is reported in a 29-year-old-woman who had portal vein obstruction with spontaneous shunting resulting in hepatic siderosis and cirrhosis. The phleboliths were visible as multiple linear calcifications on plain film and computed tomography. Diagnosis was established by surgical liver biopsy. The potential mechanisms of this unique entity is discussed.
In a rosette assay, 63 patients with recent-onset type I (insulin-dependent) diabetes mellitus had a higher (P less than .001) number of lymphocytes adhering to rat insulinoma RINm5F cells (diabetic rosettes) than 153 healthy control (background rosettes) or 20 nondiabetic subjects with other organ-specific autoimmune diseases. Furthermore, lymphocytes from diabetic patients displayed a highly correlated (r = .97, P less than .001) binding on two different xenogeneic beta-cell lines (RIN and hamster insulinoma HIT cells). This phenomenon was not found on a panel of seven non-beta-cell lines (e.g., exocrine pancreatic cells, endocrine cells). By increasing lymphocyte-to-RIN ratios (0.25:1 to 30:1), the supernumerary RIN-adherent lymphocytes from diabetic patients, expressed as the percentage of lymphocytes involved conjugates, were only detectable at lower ratios (0.25:1 to 4:1), and their binding efficiency was two times higher than that of control lymphocytes. This efficiency fell at higher ratios (greater than 4:1) to the level of background rosettes that remained constant through the ratio scale. This specific RIN-rosette formation was abrogated when lymphocytes from diabetic patients were preabsorbed on beta-cells (either HIT or RIN) but not on non-beta-cells, whereas preabsorption of control lymphocytes did not modify the number of background rosettes. In addition, diabetic rosettes, but not background rosettes, were inhibited by competition with RIN membrane extracts but not by non-beta-cell extracts. Moreover, diabetic rosettes were inhibited during blocking experiments with anti-CD3 monoclonal antibody (MoAb) but not with unrelated MoAbs.(ABSTRACT TRUNCATED AT 250 WORDS)
A case of gastric leiomyoblastoma is reported in a 57-year-old woman complaining of upper abdominal discomfort. Ultrasound and CT examinations discovered an epigastric mass, and ultrasound manoeuvers by multiple positioning allowed to refer it to a gastric origin. Definitive diagnosis was established by histological examination of the surgically removed tumor. The authors emphasize the interest of the radiological examinations, particularly ultrasound and CT, in this rare condition.
Collagenous colitis and microscopic colitis are histologic entities which do not have corresponding endoscopic features. Their precise incidence and role in the development of intestinal symptoms are poorly known. The aim of this study was to determine the frequency of these histologic abnormalities in patients with endoscopically normal colon and to correlate these findings with abdominal symptoms. Total colonoscopy was performed in 132 consecutive patients, 81 females and 51 males, aged 19 to 83 years (mean: 47.8 years). Patients complained of abdominal pain and/or diarrhea (66 cases), normal bowel transit or constipation (66 cases). Subjects were prepared for colonoscopy with polyethylene glycol 4,000. Three to 8 biopsies were taken from the rectum and the different parts of the colon. Histologic abnormalities were found in 36 patients (27.2 p. 100): collagenous colitis (7 cases), microscopic colitis (21 cases), and melanosis coli (8 cases). The frequency of diarrhea was significantly higher in patients with collagenous colitis and microscopic colitis than in those with melanosis coli or normal colonic mucosa. These results clearly demonstrate that routine biopsies of the rectum and colon are useful in patients with abdominal symptoms, particularly diarrhea, and normal endoscopy.
We report two exceedingly unusual and severe cases of Marfan syndrome with neonatal manifestations. Clinical features were caricatural. The skin was of the cutis laxa type. Both infants died shortly after birth as a result of severe cardiovascular lesions. Histologic examination of the aortic media confirmed the diagnosis. Because this genetic connective tissue dysplasia is inherited on an autosomal dominant basis with strong penetrance, and because in both instances the two parents had a negative family history, a dominant mutation seems likely in our cases and prompted us to be optimistic when giving genetic counseling. We point out the characteristic features of these exceptional early forms and describe the results of a detailed histologic examination of the skin that completes data from the literature.
Descending ocular myopathy is a rare progressive disease characterized by bilateral ptosis, external ophthalmoplegia and progressive dystrophy of the proximal skeletal muscles. A case of descending ocular myopathy with post-operative corneal ulceration is reported. Mitochondrial abnormalities are found on skeletal muscle biopsy. Nosologic classification of this case is discussed.
Circulating immune complexes are thought to play an essential part in the pathogenesis of necrosing angiitis. This theory also allows a role to be attributed to certain infectious agents (viral, bacterial, parasitic) in the development of periarteritis nodosa (PAN). An infectious syndrome was found in all our 9 patients, aged 26 to 69 years, with histologically confirmed PAN: previous infection (over 15 days before hospital admission): otitis, hepatitis B, tonsillitis, ascaris (Case n.7), pulmonary tuberculosis, brucellosis, seropositivity for Chlamydia trachomatis (Case n.9), paratyphoid (Case n.5), seropositivity for Yersiniosis pseudo-tuberculosis (Case n.2), seropositivity for Chlamydia trachomatis (Cases 3 and 4), seropositivity for toxoplasmosis (Cases 4 and 6), seropositivity for rubella (Case n.8). Recent infection (less than 15 days before hospital admission): staphylococcus aureus septicaemia (Case n.1); Group A betahemolytic streptococcal urinary infection (Case n.2); Group A betahemolytic streptococcal otitis media; pseudomonas aeruginosa and Klebsiella septicaemia; enterococcal cystitis (Case n.4); progressive pulmonary tuberculosis (Case n.6), acinetobacter pneumonia (Case n.9). The HBs antigen was only found in one patient (Case n.6), who had an active hepatitis.
Sixteen cases of anoxic encephalopathy in adult patients admitted to the intensive Care Department of Raymond Poincaré Hospital (Garches) have been selected on the following criteria: resuscitation by external cardiac massage for cardio-respiratory failure, without any previous history of anoxia and with detailed data on the subsequent evolution of neurological signs and symptoms. Main clinical features were compared to the results of the neuropathological examination. The pattern and the distribution of changes are detailed. The cerebral cortex was always involved by diffuse ischemic cell changes or by laminar necrosis. The Sommer's sector of Ammon's horn was affected in 15 cases, the basal ganglia in 14, the cerebellum in 11, the brain stem in 6. The very rarely mentionned necrotic brain stem changes, which involved inferior colliculi, periaqueductal gray matter and substantia nigra were sometimes associated with lesions of the floor of the IVth Ventricle and of the mamillary bodies. They were different from Wernicke's encephalopathy with could be dismissed on clinical and pathological grounds. No clinical signs or symptoms specific for this distribution of changes could be found by the retrospective study of the clinical files.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Histologic features of the gastric (antral and fundic) mucosa in 14 patients with portal hypertension due to alcoholic cirrhosis and a mosaic pattern of the fundic mucosa at endoscopic examination have been compared with those of the gastric mucosa in 14 control subjects. We attempted to correlate endoscopic and histologic aspects using a semiquantitative morphometric study in which the height of the mucosa, the number per mm2 and the diameter of vascular sections of the interglandular chorion, and the number per mm2 of large (greater than or equal to 20 micron) vascular sections of the superficial chorion were measured. For all parameters, the mean values were higher in cirrhotic patients than in controls, but significant differences were found only in the antral mucosa (height of the mucosa and number of large vascular sections in the superficial chorion). These results confirm the frequency and the importance of vascular abnormalities of the gastric mucosa in patients with portal hypertension, but do not explain, at least with the methodology used, the mosaic pattern of the fundic mucosa disclosed in most cirrhotic patients.
We report the case of a 66 year-old man who developed acute necrotizing pancreatitis. The usual etiologies of acute pancreatitis were easily excluded. Endoscopic retrograde cholangiopancreatography demonstrated a 10 mm diameter filling defect in the distal common bile duct. After endoscopic sphincterotomy, a polypoid tumour which appeared to arise from the common bile duct was delivered through the wound. Diathermic resection of the tumor was performed. Microscopic examination disclosed papillary adenoma, without histologic signs of malignancy. Follow-up was favorable.
The development of a pathological fracture of the lower third of the femur in which a total hip arthroplasty had been cemented 20 months earlier, was followed several months later by extensive osteolysis round the prosthesis. After much discussion, a diagnosis of malignant fibrous histiocytoma was made. A review of the literature revealed a similar case and confirmed the possibility of induction of this rare tumour in one of several ways. Whilst the oncogenic effect of an implant cannot be proved, it cannot be excluded.
A case of orbital amyloid pseudotumour is reported; this rare condition typically present as a ptosis followed by slowly growing orbital neoplasm, in middle-aged women. CT-scan disclose an extra-conal, extra-osseous, calcified mass; preoperative diagnosis of this rare condition might help in avoiding recurrence, probably due to incomplete excision. Pathophysiology of these amyloid deposits remain unknown.
The authors report the case of a patient with chronic lymphocytic leukemia well controlled during five years, and then complicated with immunoblastic lymphoma of the ileo-caecal area. The tumor was revealed by a protein-rich peritoneal effusion containing numerous large undifferentiated lymphoid cells. Only five cases of Richter's syndrome, apparently localized to the digestive tract, have been published to date.
Explore the source record for details and available documents.
The hitherto undescribed association of Gardner's syndrome (familial adenomatous polyposis coli, abdominal fibromas, osteoma, dental abnormality, cystic fundic gland polyposis) with a thymic epidermoid cyst in a 38 year old woman is reported. Thymic cyst was discovered on a routine chest roentgenogram, and diagnosis, suspected by CT findings, was established by histological examination of surgical material. The authors suggest that this association is not fortuitous.
Explore the source record for details and available documents.