Fatal neonatal liver failure and mitochondrial cytopathy: an observation with antenatal ascites.

Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day).

Liver adenomatosis with granulomas in two patients on long-term oral contraceptives.

We report two cases of liver cell adenomatosis associated with granulomatous hepatitis, both developed in white women (52 and 39 years of age) on long-term oral contraceptives (for 18 and 12 years, respectively). The first patient underwent surgery for five hepatic tumors 1-7 cm in diameter; the other patient had a partial segmentectomy for a 4-cm hepatic nodule of the right lobe. In both cases, dissection of the liver showed many other diffuse and smaller nodules.

Nodular regenerative hyperplasia in the rat induced by a selenium-enriched diet: study of a model.

Weaned male rats were fed a 4 ppm selenium diet. Compared after 2 mo with a control group fed a 0.4 ppm diet, the rats' body weights had not significantly decreased and liver function was normal, but portal pressure was 1.

Lipid-laden perisinusoidal cells in patients with acquired immunodeficiency syndrome.

Liver biopsies were taken from ten AIDS patients. Liver architecture was normal in all patients. On 1-micron-thick sections stained with toluidine blue, all ten cases showed lipid overload of perisinusoidal cells (1 massive, 5 moderate and 4 mild) compared to 2/8 in control patients, who had mild lipid overload.

Characterization of liver-associated natural killer cells in patients with liver tumors.

The existence of a marginal lymphocyte population in rat liver sinusoids has already been demonstrated using the sinusoidal lavage method. We used the same technique to study the lymphocyte population in human liver obtained ex vivo after partial hepatectomy for benign or malignant tumors and compared it with peripheral and portal blood lymphocyte populations. Percentages of lymphocyte surface phenotypes were evaluated by flow cytometry.

Late recurrence of a hepatocellular carcinoma in a patient with incomplete Alagille syndrome.

In this study, the case of a patient presenting a second hepatocellular carcinoma 13 years after resection of a first tumor of the same type is reported. In this case, etiological investigations remained negative, but an incomplete form of syndromatic Alagille syndrome with paucity of bile ducts in the nontumoral tissue was detected and associated with nodular regenerative hyperplasia and foci of dysplasia. Malignant transformation in Alagille syndrome seems to be extremely rare.

Modifications of the sinusoidal barrier in a model of postsinusoidal hypertension in the rat. An ultrastructural study of endothelial cells.

Sinusoids and sinusoidal cells were examined by light and electron microscopy, using a rat model of postsinusoidal hypertension. One month after partial ligation of the vena cava (PLVC) above the hepatic veins, subcapsular hemorrhagic areas were visible with proliferation of hepatic veins; in non hemorrhagic areas, sinusoidal congestion was found. Postsinusoidal hypertension led to a significant increase in sinusoidal volume and to major abnormalities of the endothelium such as endothelial processes and pouches with numerous diaphragmed fenestrae; some red blood cells could be seen in these pouches.

Perisinusoidal cell hypertrophy in a patient with acquired immunodeficiency syndrome.

A 33-year-old heterosexual white man underwent a liver biopsy for determination of mild elevation of aminotransferase levels (aspartate aminotransferase, two times; alanine aminotransferase, three times). The patient had acquired immunodeficiency syndrome (stage IVC2) with tuberculosis of the lymph nodes. Antibody to hepatitis B surface antigen and antibody to hepatitis B core antigen were positive.

Fine structure of hepatic sinusoids and sinusoidal cells in disease.

Liver sinusoids are special capillaries that are limited by fenestrated endothelial cells, without a genuine basement membrane, surrounded by perisinusoidal cells storing vitamin A, and harbouring Kupffer cells and pit cells, resident macrophages, and large granular lymphocytes, respectively. Each nonparenchymal cell and parenchymal cell of the liver interacts with all others and with the extracellular matrix. Therefore, the functional ability of each cell is constantly being modified by the metabolic activity of the others.

Nerves and perisinusoidal cells in human liver.

Unmyelinated nerve fibres are visible in the human hepatic lobule. They extend through the Disse space, surrounded by Schwann cell processes, often close to perisinusoidal cell processes. A few bare nerve endings or varicosities are found contiguous to either hepatocytes or perisinusoidal cells.

Chance discovery of hepatic fibrosis in patient with asymptomatic hypervitaminosis A.

A liver biopsy specimen was obtained from a 50-year-old patient whose clinical and functional liver tests showed no abnormalities but who had for some time a high vitamin A intake (109 X 10(6) IU over four years). Liver architecture was normal. Sinusoids were slightly dilated in zone 2.

The increase in the number of liver sinusoidal pit cells in four patients with primary or metastatic cancer of the liver.

Four patients with liver carcinoma (case 1: hepatocellular carcinoma; cases 2 and 3: metastases; case 4: adenocarcinoma possibly of hepatic origin) underwent a wedge liver biopsy taken at some distance from the tumor. Liver histology was normal in cases 2 and 3. Sinusoids were dilated in case 4.

Hepatic fibrosis in patients with idiopathic thrombocytopenic purpura.

Three patients showing all the symptoms of idiopathic thrombocytopenic purpura underwent a splenectomy. A wedge liver biopsy revealed hepatic fibrosis around central veins and an increased perisinusoidal network on Sirius red staining. Fibrosis was moderate or mild.

Liver sinusoids and sinusoidal cells in patients with agnogenic myeloid metaplasia.

The origin of sinusoidal portal hypertension often remains unknown in patients with agnogenic myeloid metaplasia. Four consecutive patients with agnogenic myeloid metaplasia had liver biopsies examined under light and electron microscopy. Two of the four had obvious clinical portal hypertension; of these two, only one had portal vein thrombosis.

Perisinusoidal fibrosis and basement membrane-like material in the livers of diabetic patients.

A direct correlation exists between collagenization of Disse's space and the presence of diabetic microangiopathy in type I diabetes. To confirm and extend this finding, we studied four liver biopsy samples from two patients with type I diabetes (one with retinopathy) and two patients with type II diabetes (no retinopathy). All had normal or subnormal results on liver function tests and normal liver architecture.

[Perisinusoidal fibrosis in idiopathic thrombopenic purpura].

Perisinusoidal fibrosis of the liver was discovered in a 22 year-old woman with idiopathic thrombocytopenic purpura requiring splenectomy. No etiology of fibrosis could be found. As shown by immunocytochemistry, collagen types I, III, IV, laminin, and fibronectin were increased.

Agnogenic myeloid metaplasia, portal hypertension, and sinusoidal abnormalities.

A patient with agnogenic myeloid metaplasia suffered from gastrointestinal bleeding due to ruptured esophageal varices. The portal vein and its intrahepatic branches were patent. Except for the presence of myeloid cells, mainly megakaryocytes, in the sinusoids, liver histology was more or less normal.

Hepatocellular carcinoma developed on noncirrhotic livers. Sinusoids in hepatocellular carcinoma.

In hepatocellular carcinoma, there is modification of cell-to-cell and cell-to-extracellular matrix interactions. Two cases of well-differentiated hepatocellular carcinoma that developed in noncirrhotic livers were explored by light and electron microscopy on perfusion-fixed liver biopsy specimens. In addition, immunocytolocalization of collagen types I, III, and IV, laminin, and fibronectin was assessed.