[Diagnosis with hair].
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Biomedical subjects
Publications and source records attributed to H Sevestre.
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We report the case of a 14-year-old girl with a testicular feminization syndrome. The inguinal cryptorchid testis contained plurifocal hamartomas ranging from 0.5 to 1 cm. They were composed of tubules lined by cylindrical Sertoli cells immunoreactive for alpha-inhibin and p30/32(MIC2). The stroma contained few Leydig cells. Ultrastructural study showed tubules with immature Sertoli cells. The testicular feminization syndrome is caused by mutations of the androgen receptor gene. Patients with male genotype 46, XY have a female morphotype with external sexual organs without ambiguity. They have neither uterus nor ovary but two cryptorchid testis in which sex-cord stromal tumors can develop. Their malignant transformation is rare but requires preventive bilateral orchidectomy.
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Portal hypertension in chronic lymphocytic leukemia is rare. A 66 year-old man was admitted for splenomegaly, thrombopenia and cholestasis. Endoscopy showed esophageal varices. The hepatic venous pressure gradient was 15 mmHg. The liver biopsy showed dense leukemia cells in sinusoidal and portal sites. After splenectomy, the hepatic venous pressure gradient normalized, but esophageal varices and cholestasis persisted. The authors discuss the mechanisms of portal hypertension in chronic lymphocytic leukemia. Previously reported cases are summarized.
INTRODUCTION: Among several adverse effects following treatment with minocycline, certain cases of autoimmune hepatitis, associated with lupus erythematosus, have been described. The possibility of hepatic damage, although rare, is important to keep in mind because of its delicate diagnostic. EXEGESIS: We report one case of autoimmune hepatitis following treatment with minocycline for acne, in a 25-year-old woman. This autoimmune hepatitis was associated with induced lupus syndrome. Usual causes of hepatitis were eliminated. Evolution was spontaneously favorable upon minocycline treatment interruption, with the disappearance of clinical symptoms and normalization of hepatic and immunologic biological values. CONCLUSION: The possibility of hepatic damage and lupus syndrome, following treatment with minocycline, should be recalled and verified in cases of long-term prescription. This observation stresses the difficulties of anamnesis in internal medicine. For those who know how to listen cautiously and rigorously, anamnesis may prove more helpful than many complementary examinations.
Malignant tumors arising from venous walls in the lower extremity are uncommon. Histologically they are divided into two groups: hemangioendotheliomas of intermediate malignancy and leiomyosarcomas. This report describes a retrospective series of seven primary venous tumors observed in four men and three women with a mean age of 49.8 years (range: 18 to 64 years) who underwent surgical treatment between 1985 and 1995. The tumor was located in the superficial femoral vein in four patients, common femoral vein in two patients, and greater saphenous vein in one patient. A palpable tumor was present in five patients, localized pain in two patients, and metastasis in two patients. The histological diagnosis was leiomyosarcoma in six patients and hemangioendothelioma in one patient. Surgical treatment consisted of complete resection in six patients and partial excision in one patient. Venous reconstruction was performed in two patients and adjuvant radiation therapy in four patients. There was no operative morbidity/mortality. Median survival was 31 months. Four patients with leiomyosarcoma died from metastasis. Two patients with leiomyosarcoma and one with hemangioendothelioma are alive at 9 years, 16 months, and 9 months, respectively. Local recurrence was never observed after complete resection. The prognosis of venous leiomyosarcoma of the lower extremities is poor due to early occurrence of metastasis. Doppler ultrasound and MRI are useful to establish early diagnosis at the nontumoral stage. Improvement in the prognosis of leiomyosarcoma may justify perioperative chemotherapy before and after radical surgical excision.
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Liver involvement manifesting as hepatomegaly in Langerhans cell granulomatosis (LCG) is well known, but the definitive diagnosis is generally possible because other organs are involved. We report a 41-year-old white man who presented with cholestasis and liver nodules as an isolated hepatic LCG. The diagnosis of LCG was suspected based on routine histopathologic examination; the diagnosis became definitive 4 years later when Birbeck granules were found in the liver, an uncommon occurrence in this organ. This is an unusual presentation of a benign form of this disease and one of the first that reported Birbeck granules in the liver.
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Inflammatory pseudo-tumor of the liver (IPT) are benign encapsulated masses. IPT or inflammatory myofibroblastic tumor is a myofibroblastic proliferation with chronic inflammatory cell infiltration of unknown origin. A four-year-old girl, with cutaneous flushing of the face and biological inflammatory syndrome was referred for abdominal investigation. Ultrasound examination showed a 5-cm mass located in the anterior segment of the right hepatic lobe. After abdominal CT showing slightly vascular feature of this right hepatic mass, diagnosis was made through percutaneous US-guided fine needle biopsy. Surgical resection was performed and pathologic examination of the mass confirmed preoperative diagnosis. Clinical outcome was good.
Idiopathic adult ductopenia is very rare. We report one case in a 30-year-old man, whose clinical course was characterized by jaundice and pruritus. Laboratory investigations revealed cholestasis and polyclonal hypergammaglobulinemia. Serum antinuclear, antimitochondrial, and anti-smooth muscle antibodies and serological markers for viral hepatitis were negative. Endoscopic retrograde cholangiography showed no liver or biliary tract abnormalities. Histological examination of a liver specimen showed a vanishing bile duct syndrome and moderate portal infiltration with lympho-histiocytic cells; there were no granulomas. Liver transplantation was performed due to rapid development of cirrhosis. The differential diagnosis of idiopathic adult ductopenia with small duct primary sclerosing cholangitis, auto-immune cholangiopathy, and non syndromic paucity of intrahepatic bile ducts is unclear.
An experimental animal model has been used to study the separate and combined use of antibiotics and anti-inflammatories in the treatment of salpingitis. The addition of an anti-inflammatory did not seem to help preserve fertility.
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An unusual case of leiomyosarcoma of the prostate presented as a recurrent pelvic cystic mass. Prostatic sarcoma are rare in adults with a poor prognosis. It is often difficult to determine a definite origin. The authors reviewed the differential diagnosis with pseudosarcoma, inflammatory fibrosarcoma and others rare tumors of the prostate and the seminal vesicle.
A case of gastric hyperplastic polyposis is reported in a 48-year old woman, with iron deficiency anemia. An hyperplastic gastric polyposis was discovered. This patient had been operated 17 years previously for a large adenomatous polyp of the caecum. Her son had also several adenomatous polyps of the right colon. A gastrectomy was performed. Hyperplastic gastric polyposis is very rare, and is quite always associated with colorectal adenomas. The relationship between gastric hyperplastic polyposis and intestinal polyposis is not quite clear.
Two cases of fibrotic stricture of the extrapancreatic common bile duct were observed 3 and 5.5 months after severe acute alcoholic pancreatitis. The diagnosis was made by endoscopic retrograde cholangiography in both cases. Although colonic or ureteric stenosis have been reported after acute pancreatitis, this is the first report of extrapancreatic biliary stricture occurring after acute pancreatitis. The strictures could have arisen by either an enzymatic or ischemic mechanism. Outcome was favorable after surgical hepaticojejunostomy.
The authors report a case of recurrent pigmented intraspinal schwannoma with malignant progression. Electron-microscopic study confirmed that melanin pigment was produced by tumor cells and that these cells were derived from the nerve sheath. The histogenesis of pigmented tumors is explained by their neural crest origin and the problem of differential diagnosis of the malignant form with melanoma is discussed.
The authors report two cases of non functional parathyroid cyst both found by a systematic clinical examination in two women of 56 and 67 years. In the first case, the cyst was associated to a thyroid dystrophy; the other patient had a history of thyroid dystrophy but also of pituitary prolactin adenoma.