1/38. Heterotopic thyroid tissue at the porta hepatis in a fetus with trisomy 18.Heterotopic thyroid tissue at the porta hepatis in a fetus with trisomy 18 is described. The fetus had an eutopic thyroid gland without any abnormalities. The heterotopic thyroid was found at the porta hepatis and showed histological features similar to the eutopic thyroid. Immunohistochemically, the heterotopic follicles were positive for thyroglobulin, but no calcitonin-positive cells were found. Intra-abdominal heterotopic thyroid is exceedingly rare in locations other than the ovary, and to our knowledge, this is the first report of a fetal case. The present case provides clear evidence that abdominal heterotopic thyroid can occur as a congenital anomaly. Migration abnormality of the median anlage of the thyroid is the most likely histogenesis of heterotopic thyroid at the porta hepatis.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/38. Congenital generalized infantile myofibromatosis and neonatal hemochromatosis. An autopsy case report.An autopsy case of congenital infantile myofibromatosis and neonatal hemochromatosis is reported. A thirty-six-hour-old baby girl had multiple subcutaneous nodules in addition to multiple visceral involvement of heart, lungs, pharynx, larynx, stomach, small bowel, large bowel, pancreas, kidneys, spleen, thyroid, adrenal glands, lymph nodes, peripheral nerves, meninges and soft tissues. In these tumoral nodules, three types of histological patterns were observed: 1-hemangiopericytoma-like, 2-mixed, and 3-pure spindle cell. Tumor cells were immunohistochemically positive for actin, and negative for desmin, muscle-specific antigen, and estrogen, related protein. The histological and immunohistochemical findings of the case suggested that a close relationship may exist between infantile myofibromatosis and infantile hemangiopericytoma. In addition to infantile myofibromatosis, neonatal hemochromatosis characterized by iron deposition in parenchymatous organs such as liver, pancreas, lungs, thyroid, and adrenal glands was another important characteristic of the case.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
3/38. Troglitazone and liver function abnormalities: lessons from a prescription event monitoring study and spontaneous reporting.OBJECTIVES: To investigate whether there were any cases of liver function abnormalities possibly associated with troglitazone use in general practice in england. DESIGN: A prescription-event monitoring (PEM) study was undertaken between October 1997 and December 1997. SETTING: Data from prescriptions were obtained electronically for the troglitazone cohort in the immediate postmarketing period. STUDY PARTICIPANTS: Event data were obtained for a total of 1344 patients. RESULTS: Troglitazone was effective in 394 (75%) of the 529 patients for whom an opinion was given. The most frequent reasons for stopping treatment related to drug tolerability were malaise/lassitude (16 reports), abnormal liver function tests (II reports) and nausea/vomiting (9 reports). The major cause of stopping troglitazone was because the drug was withdrawn from the market (1101 reports). 30 patients with liver dysfunction were identified from the cohort. In 9 of these patients there were alternative explanations for the liver dysfunction and hence these patients were not followed up further. 21 patients were followed up, for whom 19 questionnaires were returned. In 5 patients their liver dysfunction was assessed as possibly related to troglitazone, in 6 patients the liver dysfunction was unlikely to be attributed to troglitazone, while in 7 patients it was difficult to assess the causality because of limited information and confounding factors. The remaining patient was not included as this individual did not fit the inclusion criteria of the study. CONCLUSION: Although the cohort is small (the drug was available for only 3 months in the UK), 5 patients with abnormal liver function, considered possibly related to troglitazone were detected in this PEM study. It is possible for PEM to contribute to the elucidation of safety signals in the UK.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/38. Multiple hepatic peribiliary cysts discovered incidentally at a medical examination.We report a living patient with multiple hepatic peribiliary cysts. It was discovered incidentally during an ultrasonographic screening at a medical examination. Peribiliary cysts are multiple retention cysts of peribiliary glands. Although many autopsy cases of peribiliary cysts have been reported, there are few clinical cases of it in living patients. A CT performed immediately after drip-infusion cholangiography (DIC) was most useful for diagnosis in various imaging tests we performed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/38. A case of histoplasma capsulatum causing granulomatous liver disease and Addisonian crisis.A 56-year-old man with persistently elevated liver enzyme levels, fatigue, lethargy and a 9.0 kg weight loss over six months underwent a percutaneous liver biopsy that demonstrated multiple granulomas. Screening serologies were positive for histoplasmosis, and he was started on itraconazole treatment. He returned to hospital the same night with coffee-ground emesis and in Addisonian crisis requiring parenteral steroids and intensive care unit support. An abdominal computed tomography scan revealed bilaterally enlarged, nonenhancing adrenal glands suggestive of infarcts, presumed secondary to histoplasmosis. Treatment was initiated with amphotericin b, and histoplasma capsulatum was cultured from his urine and cerebrospinal fluid. A serum immunodiffusion test was also positive for both H and M bands, indicating active infection with histoplasmosis species. His serum and urine samples were also weakly positive for the antigen. Despite complications of renal failure, pneumonia and congestive heart failure, he recovered with medical therapy and was discharged home to complete a prolonged course of itraconazole therapy. While hepatic granulomas often reflect an occult disease process, the cause may remain undiscovered in 30% to 50% of patients despite exhaustive investigations. H capsulatum is an uncommon cause of granulomatous liver disease, and with its protean clinical presentation, a high index of suspicion is needed to make the diagnosis and avoid the potentially high fatality rate associated with disseminated infection.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/38. heme oxygenase-1 deficiency: the first autopsy case.This article describes the first autopsy case of heme oxygenase (HO)-1 deficiency. A 6-year-old boy who presented with growth retardation; anemia; leukocytosis; thrombocytosis; coagulation abnormality; elevated levels of haptoglobin, ferritin, and heme in serum; a low serum bilirubin concentration; and hyperlipidemia was diagnosed as HO-1 deficient by gene analysis several months before death. autopsy showed amyloid deposits in the liver and adrenal glands and mesangioproliferative glomerular changes in kidneys, in addition to an irregular distribution of foamy macrophages with iron pigments. Fatty streaks and fibrous plaques were noted in the aorta. Compared with HO-1--targeted mice, the present case seems to more severely involve endothelial cells and the reticuloendothelial system, resulting in intravascular hemolysis, disseminated intravascular coagulation, and amyloidosis with a short survival. This contrasts to the predominant iron metabolic disorders of HO-1--targeted mice with a long survival.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/38. Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver.OBJECTIVE: To report the development, in an adult patient, of Langerhans' cell histiocytosis (LCH) involving the thyroid and most probably the pituitary gland, lungs, and liver. methods: We present a case report of a 29-year-old woman who requested a medical consultation because of polyuria and was found to have pituitary dysfunction. We describe the subsequent follow-up, which revealed progressive liver disease that necessitated transplantation and also the presence of a goiter, and discuss the unpredictable pathologic features of LCH. RESULTS: After the diagnosis of central diabetes insipidus and partial hypopituitarism, the patient was diagnosed 2 years later with sclerosing cholangitis. The hepatic involvement was progressive, and she required transplantation and immunosuppression. Three months before liver transplantation, a goiter was discovered. Fine-needle aspiration of the thyroid revealed infiltration by LCH. The goiter decreased in size after chemotherapy with vinblastine and prednisone. Computed tomography of the chest showed bilateral thin-walled cysts, consistent with eosinophilic granulomas. CONCLUSION: In patients with central diabetes insipidus and pituitary stalk thickening on imaging studies, LCH should be considered in the differential diagnosis. Other hormonal deficiencies may be present initially or may evolve after many years. Thus, continual surveillance is necessary. In addition, an ongoing potential exists for involvement of other organ systems such as the thyroid, liver, and lungs. We suggest consideration of LCH as a possible cause of a goiter in such patients. In our patient, it remains to be seen what effect the immunosuppressive therapy for the liver transplant has on the LCH disease process.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
8/38. Autoimmune cholangitis in patients with primary sjogren's syndrome. A case report.sjogren's syndrome is a very frequent autoimmune disease, characterised by exocrine gland involvement. Immunologic disorders are also responsive for extraglandular manifestations of the disease, mostly for the digestive involvement. We report a case of primary sjogren's syndrome with multiple extraglandular manifestations: vasculitis, cryoglobulinaemia, hepatic involvement and presumably neurologic involvement. The particularities of the case are the typical pattern of autoimmune cholangitis, hypogammaglobulinaemia, the absence of antinuclear antibodies and the association with vasculitis. To the best of our knowledge, this is the first case report of autoimmune cholangitis in sjogren's syndrome- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
9/38. Unusual hepatic tumor with features of mesenchymal hamartoma and congenital solitary nonparasitic cyst.We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy, measured 21 cm and was multicystic with solid areas. Microscopically, the cysts were lined by a mucous-producing or intestinal-type epithelium, associated with smooth muscle and small mucous-producing glands. The solid component contained fibrous and adipose tissue, smooth muscle and thick-walled vessels. aneuploidy was demonstrated by flow cytometry. We interpreted the tumor as having features of a mesenchymal hamartoma and congenital solitary nonparasitic cyst. It is conceivable that the lesions originated with small peribiliary glands with dilatation and intestinal metaplasia.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
10/38. Ultrasound visualization of hepatic peribiliary cysts: a comparison with morphology.We report herein a hitherto unrecognized, interesting ultrasound finding ("hilar multicystic echo complex"), the result of peribiliary cysts in the liver. This ultrasound finding was discovered around intrahepatic large bile ducts and large portal vein branches near the hepatic hilum in an autopsy case with hepatocellular carcinoma, submassive hepatic necrosis superimposed on chronic active hepatitis, and portal hypertension. Antemortem ultrasound examination revealed the hilar multicystic echo complex around the portal venous branches near the hepatic hilum. autopsy confirmed that the hilar multicystic echo complex was due to peribiliary cysts that were present around the bile ducts at the hilum. The peribiliary cysts were thought to have arisen from cystic dilatation of preexisting intrahepatic peribiliary glands. These peribiliary cysts reportedly occur in livers with portal hypertension (e.g., cirrhosis, hepatocellular carcinoma, idiopathic portal hypertension, extrahepatic portal obstruction, and portal thromboembolism), adult-type polycystic disease of the liver and kidneys, and systemic infection. Therefore, recognition of peribiliary cysts at sonography would have diagnostic value, and may indicate that presence of one of the above described liver diseases.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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