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Biomedical subjects

C Degott

Publications and source records attributed to C Degott.

At least 289 records · Page 16Linked to original sources
PubMed1978

[Pulmonary emphysema and hepatic involvement by alpha-1 antitrypsin deficiency in two adults with a PiZ phenotype (author's transl)].

Two unreleated adult males were found to be suffering from an association of pan-lobular severe emphysema and hepatomegally of unknown origin which led to the discovery of a marked deficit in alpha-1 antitrypsin (A1-AT) in relation to a PiZ phenotype. Liver biopsy revealed cirrhosis with portal fibrosis in one case and in both cases fatty infiltration with the accumulation of a glycoprotein antigenically identical to A1-AT. Electron microscopy showed this protein to be situated within the dilated lumina of the endoplasmic reticulum of the hepatocytes. A1-AT deficiency is usually associated with pulmonary involvement only in the adult and liver involvement only in the child. The association of the two remains rare--hence the interest of the two cases reported.

Adult↗
PubMed1978

Antibody to hepatitis B core antigen in chronic active hepatitis.

Antibody to hepatitis B core antigen (anti-HBc), which has been assumed to be a more sensitive indicator of hepatitis B virus replication than hepatitis B surface antigen (HBsAg), was detected in the sera of 26 of our 65 patients with HBsAg-negative chronic active hepatitis. Thus despite the absence of HBsAg the liver disease could be the consequence of chronic infection with hepatitis B virus in these patients. They differed, however, from a group of 35 patients with HBsAg-positive hepatitis in being older on average and having less active liver lesions. The two groups could represent either two stages of chronic infection with hepatitis B virus or two types of response to it.

Adolescent↗
PubMed1978

Transvenous (transjugular) liver biopsy. An experience based on 100 biopsies.

Transvenous (transjugular) liver biopsy consists in taking a liver specimen through a needle introduced into the liver parenchyma from the lumen of a hepatic vein. This procedure was attempted 104 times in 98 patients in whom percutaneous needle liver biopsy was contraindicated because of massive ascites and/or bleeding tendency. A liver specimen was obtained in 100 out of these 104 attempts; the tissue specimens were unfragmented and large enough to allow correct evaluation of liver architecture in 57 biopsies. The procedure was followed by no or only minor complications in all out patients except one who suffered a fatal intraperitoneal hemorrhage in relation to perforation of liver capsule; perforation was due to an excessive front rotation applied to the needle, a maneuver which therefore must be avoided. It is concluded that transvenous liver biopsy is a workable, efficient, and acceptably safe procedure for obtaining liver specimens in patients with massive ascites and/or bleeding tendency.

Adult↗
PubMed1978

Peliosis hepatis in recipients of renal transplants.

Peliosis hepatis, an uncommon liver lesion characterised by blood-filled cavities bordered by hepatocytic plates, was found in 12 patients three to 17 months after renal transplantation. Hepatomegaly and portal hypertension were present in five of the six patients with major peliosis hepatis, and were absent in the other six with minor hepatic lesions. Alterations of centrilobular vein walls in some of these patients suggest that peliosis hepatis could be the result of a blockade or liver blood outflow at the junctions of sinusoids and centrilobular veins. The cause of these alterations might be azathioprine.

Adolescent↗
PubMed1978

Portal hypertension in systemic mastocytosis.

We report the case of a 66-year-old male patient with portal hypertension related to systemic mastocytosis. The liver was enlarged; microscopic examination showed portal mast cell infiltration and fibrosis. Portal hypertension was evidenced by splenomegaly, esophageal varices, and increased wedged-free hepatic venous pressure gradient. Arteriography showed that portal vein was patent. Portal hypertension could be the consequence of intrahepatic block due to mast cell infiltration and/or fibrosis of the liver.

Aged↗
PubMed1978

Nodular regenerative hyperplasia of the liver. Report of six cases and review of the literature.

The authors report the cases of 6 adult patients suffering from nodular regenerative hyperplasia of the liver, an uncommon lesion characterized by small-sized hepatocytic nodules scattered throughout the liver, with no or slight fibrosis. The histological recognition of nodular regenerative hyperplasia of the liver was easy on large specimens taken intraoperatively, but was difficult or impossible on small specimens such as those provided by needle biopsy. In all of the patients of this series, the main consequence of nodular regenerative hyperplasia of the liver was portal hypertension. In this series, nodular regenerative hyperplasia of the liver was not associated with rheumatoid arthritis, whereas such an association has been noted in most of the cases previously reported. The etiology and pathogenesis of nodular regenerative hyperplasia of the liver is unknown.

Adolescent↗
PubMed1978

Iproclozide fulminant hepatitis. Possible role of enzyme induction.

The authors report the cases of 3 patients who died from fulminant hepatitis after receiving iproclozide, a hydrazine-containing monoamine oxidase inhibitor. Fulminant hepatitis in these patients resembled that reported in patients receiving other hydrazine-containing monoamine oxidase inhibitors: (1) the 3 patients were women; (2) the monoamine oxidase inhibitor has been ingested for 1 month or more; (3) the main clinical manifestations were jaundice and disorders of consciousness; (4) hypersensitivity manifestations were absent; (5) the predominant liver lesion was necrosis; (6) all 3 patients died. In our 3 patients, jaundice occurred 7 to 10 days after the adjunction to iproclozide of a microsomal enzyme inducer. These observations suggest that concomitant administration of iproclozide and of microsomal enzyme inducers may produce fulminant hepatitis in man. It is speculated that iproclozide could be, like iproniazid, transformed into a hepatotoxic metabolite, the production of which would be increased by microsomal enzyme induction.

Adult↗
PubMed1977

HLA-A1, B8-phenotype association and HBs antigenemia evolution in 440 hemodialyzed patients.

HBs antigen (HBsAg) has been followed up every month in 440 hemodialyzed patients, typed for 26 HLA alleles of the A and B loci. An abnormally high rate of the HL-A-A1, B8 association (18.6%) was found in the group of patients able to eliminate HBsAg, when compared with the normal French population (5.05%, p less than 10(-4), and with the group of patients unable to eliminate HBsAg (7.0%, less than 0.01). Chronic aggressive hepatitis was only found in the latter. This high frequency of the HLA-A1, B8 association has also been found in patients with seronegative active chronic hepatitis and suggests that this phenotype might be associated with high immune response against HBsAg.

Chronic Disease↗
PubMed1977

Isoniazid-rifampin fulminant hepatitis. A possible consequence of the enhancement of isoniazid hepatotoxicity by enzyme induction.

The authors report 6 cases of fulminant hepatitis in patients treated with isoniazid and rifampin. In 4 of these patients, the treatment had been started within 3 days after a general anesthesia. The course of the disease was remarkably similar in all 6 patients: (1) the time interval from the beginning of the isoniazid-rifampin administration to the onset of jaundice was 6 to 10 days; (2) disorders of consciousness appeared less than 3 days after the onset of jaundice; (3) serum transaminases were 26 to 80 times the upper limit of normal; (4) the main liver lesion was centrilobular necrosis; (5) hypersensitivity manifestations were absent; (6) all 6 patients recovered. Fulminant hepatitis might be attributable to a hepatotoxic metabolite of isoniazid, the production of which would be attributable to a hepatotoxic metabolite of isoniazid, the production of which would be increased as a consequence of the enzyme-inducing effect of rifampin and, possibly, other drugs administered for general anesthesia.

Adolescent↗
PubMed1976

[Hepatic puncture biopsy by the transjugular route].

The authors report their experience of transjugular liver biopsy, a method which consists in taking a liver specimen with a needle placed in a hepatic vein. This method was used in 13 patients in whom conventional liver needle biopsy was contraindicated because of massive ascites or bleeding tendency. In 10 of these patients, a liver specimen was obtained; in patients with cirrhosis or chronic active hepatitis, the liver specimens were more or less divided into small fragments, which prevented correct assessment of liver architecture in three of them. The examination was well tolerated. The authors conclude that transjugular liver biopsy is not technically difficult in centers where hepatic vein catheterization is available and that this method is indicated in patients in whom conventional liver needle biopsy is contraindicated.

Biopsy, Needle↗
PubMed1976

Portal hypertension and primary biliary cirrhosis.

Portal hypertension has been regarded as an uncommon and late complication of primary biliary cirrhosis (PBC). 24 patients with PBC were investigated for portal hypertension. Esophageal varices were present in 20, 50, and 90% of the patients 1, 3, and 9 years, respectively, after the onset of pruritus and/or jaundice. Portal hypertension was responsible for gastrointestinal bleedings in 11 patients; bleeding was the first clinical manifestation of PBC in two of them. Wedged hepatic venous pressure was increased in all the patients with portal hypertension whether regenerative nodules were present or absent. Portacaval shunt was performed in five patients and was well tolerated in three of them. It is concluded that (a) portal hypertension is common in PBC; (b) the intrahepatic block is of the so-called postsinusoidal type, even in patients without regenerative nodules; (c) gastro-intestinal bleeding due to portal hypertension occurs in about half of the patients and may be the first manifestation of PBC; (d) portacaval shunt seems to be indicated when gastro-intestinal bleeding occurs in earlier stage of the disease.

Adult↗
PubMed1975

[Malignant lymphoma with pulmonary localization. Attempt at an anatomo-pathological classification].

The authors reported 33 cases of malignant lymphoma in the lung observed over a period of 6 years on the Pathological Department of the Beaujon Hospital. Whatever their cytological, lymphocytic, lymphoblastic or reticular type (Hodgkin's disease was excluded from this study), they may be grouped in two main pathological categories: 1) Malignant lymphoma involving the lung and mediastinum without extra-thoracic lesions detected at the time of diagnosis (4 cases). In 3 cases, the disease had been present for 2 to 6 months. In 1 case the course was favourable for 5 years then the patient developed acute lymphoblastic leukemia. 2) The pulmonary lesions observed during general infections, whether as presenting symptoms in 9 cases, or simply discovered on autopsy as in 20 cases. Histological examination, essential for the diagnosis of malignant lymphoma, provides no evidence in favour of the primary pulmonary origin of the disease; this diagnosis can only be made after full blood investigations. The prognosis is also difficult to determine for survival for many years may be followed by sudden general malignant blood disease.

Autopsy↗
PubMed1975

[Benign giant cell tumors associated with Paget's disease. Apropos of 1 case].

The authors report the observation of two benign giant-cell tumours that developed in the cranium of Paget's disease patients. The two tumours were resected and cure was complete. Eighteen other cases of benign giant-cell tumours were found in the literature. All were discovered in relation to tumefaction occurring in an affected bone in a patient with generalized Paget's disease, often unrecognized. The tumours were usually unique although multiple tumours were found, with particular predilection for the bones of the cranium and the face. The radiological signs consisted of an osteolytic zone in an affected bone; there were no specific characteristics and it was not possible to distinguish the tumours from a malignant tumour. Diagnosis was based upon an anatomo-pathological examination. In the 18 cases in the literature, the benign caracter indicated by the biopsy was confirmed by the favourable evolution. In contrast in 17 other cases the atypical nature of the stroma, the irregular arrangement of the giant cells together with the occurrence of atypical mitoses and the abnormal character of the vascularization indicated straight away the malignant nature of the lesions, which was regularly and rapidly fatal.

Giant Cell Tumors↗