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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We examined clinical and laboratory data and the liver pathology of 48 patients in whom porphyria cutanea tarda was related to alcohol ingestion, estrogen use and pregnancy, or was idiopathic. Biochemical test results, when abnormal, tended to be mild in most cases, with less than two-fold elevations of serum aminotransferases and alkaline phosphatase and mild hyperbilirubinemia. Fatty change, liver cell and Kupffer cell hemosiderosis and glycogenation of hepatocyte nuclei were frequent histologic findings in the 58 liver specimens studied. Alcoholic hepatitis, chronic hepatitis and cirrhosis were uncommon. Granuloma-like lobular aggregates consisting of iron- and ceroid-laden Kupffer cells, chronic inflammatory cells and fat droplets ("lobular lesions of porphyria cutanea tarda") were found in nearly two-thirds of specimens and appeared to be the most characteristic form of parenchymal damage in this form of porphyria. These lesions may be associated with pericentral fibrosis in alcoholic as well as estrogen-treated patients and may remit following therapeutic phlebotomy.
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PMID:Hepatic pathology in porphyria cutanea tarda. 619 67

We report the findings in 53 biopsies from 45 patients with porphyria cutanea tarda (PCT). Red autofluorescence and birefringent acicular cytoplasmic inclusions were constant findings in all untreated cases. Autofluorescence occurs in other hepatic porphyrias, but acicular inclusions appear to be specific for PCT; we have seen them in subclinical porphyria and before development of cutaneous symptoms. They are probably uroporphyrins and they trend to disappear during rinsing by water during most staining procedures. We recommend unstained paraffin sections for their demonstration. Liver damage in PCT has features distinct from other liver diseases, including alcoholic liver disease. These include constant but mild periportal siderosis, focal lipofuscin deposition, focal lobular hepatocyte necrosis associated with groups of pigment-laden macrophages, focal steatosis, marked hepatocyte hyperplasia and the presence of periductal lymphocyte aggregates. The latter have not been previously described in PCT and were present in 43% of our cases. There is a direct relationship between increasing age and progressive distortion of liver architecture, with fibrosis present at a mean age of 48 years, cirrhosis at 57 and hepatocellular carcinoma at 66. The characteristic liver histology and the natural history of PCT are against this being the result of any non-specific liver damage and favour instead a specific liver disease whose pathogenesis may be mainly the result of the metabolic defect of PCT.
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PMID:The pathology of the liver in porphyria cutanea tarda. 625 81

A case of ruptured hepatocarcinoma of the left lobe in a cirrhotic patient with a 7 years' history of porphyria cutanea tarda (PCT) is reported. Emergency left lobectomy resulted in 3 years' survival with no sign of recurrence at present. Comments are made concerning the clinical association PCT-hepatoma: (1) either PCT is associated with a hypersecretory hepatoma and behaves as a neoplastic syndrome following the same course as the tumour; (2) or PCT is associated with a cirrhosis (usually alcoholic) which facilitates the development of a hepatoma. In such cases, PCT precedes the tumour and follows a separate course.
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PMID:[Haemoperitoneum due to ruptured hepatoma in a cirrhotic patient with porphyria cutanea tarda. Three years' survival (author's transl)]. 628 97

Hemochromatosis is a syndrome which, when fully expressed, is manifested by melanoderma , diabetes mellitus, and liver cirrhosis, with iron overload involving parenchymal and reticuloendothelial cells in many organ systems. This clinical presentation may arise as a consequence of either hereditary or acquired abnormalities of iron overload, although the mechanisms are quite different. In hereditary hemochromatosis (also known as primary, or idiopathic, hemochromatosis), increased intestinal iron absorption leads to excessive accumulations of iron, throughout the body, particularly in parenchymal cells. In secondary forms of iron overload including transfusional hemosiderosis, alcoholic cirrhosis, thalassemia, sideroblastic anemia, and porphyria cutanea tarda, iron accumulates in the reticuloendothelial system initially, but with increasing amounts of total body iron, excessive iron deposits eventually accumulate in parenchymal cells throughout the body producing a picture indistinguishable from hereditary hemochromatosis. In this article, the course, prognosis, and therapy of iron overload will be reviewed in detail. Clinical and experimental data concerning the pathogenesis of the different forms of iron overload will be examined critically. In particular, information relating to possible abnormalities of reticuloendothelial function, intestinal mucosal iron transport, and alterations in serum and tissue isoferritin patterns in hereditary hemochromatosis will be analyzed, and possible directions for future research will be suggested. The mode of inheritance and linkage with the major histocompatibility (HLA) complex will be discussed. Theories on the pathogenesis of tissue damage by excess iron will be evaluated. Methods for measuring the extent of iron overload in clinical practice will be described, including measurements of serum iron, serum ferritin, iron absorption, cobalt excretion, desferrioxamine excretion, liver biopsy and tissue iron determinations, and HLA typing. Finally, unresolved problems in the understanding of the disease process, diagnosis, and therapy will be delineated.
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PMID:Iron overload disorders: natural history, pathogenesis, diagnosis, and therapy. 637 41

Some parameters of iron metabolism in 26 patients with porphyria cutanea tarda (PCT) which is often associated with mild iron overload and hepatic siderosis, are studied. Serum iron, percent transferrin saturation and ferritin were pathologically increased. Statistical comparisons were performed between PCT patients and healthy controls, liver disease patients (cirrhosis, chronic active hepatitis) and patients with associated liver siderosis (alcoholic cirrhosis, cirrhosis and chronic active hepatitis in thalassemia). Ferritin levels are higher in patients with porphyria than in healthy controls (p less than 0,001) and in patients without liver siderosis (p less than 0,001). No statistical difference is observed between patients with porphyria and patients with siderosis. A significant decrease in ferritin levels is registered after venesection therapy. The conclusion is drawn that serum ferritin increase in PCT is related to hepatic iron store amounts rather than hepatic necrosis. It is assumed that ferritin follow-up during phlebotomy therapy and also during remission is useful to indicate the exhaustion or an early replenishment of hepatic iron stores.
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PMID:[Determination of serum ferritin in porphyria cutanea tarda. A reliable sign of hepatic siderosis]. 670 23

In the liver of 12 patients with porphyria cutanea tarda (PCT) the following parameters were measured: protein-content, NADPH-cytochrome c-reductase, 7-ethoxycoumarin-deethylase. The results were compared with the findings of patients with chronic liver disease (chronic hepatitis or cirrhosis) and patients without any liver affection. In addition the in vitro inhibition of 7-ethoxycoumarin-deethylase by metyrapone (phenobarbital induced cytochrome P-450) or naphthoflavone (benzo[a]pyrene induced cytochrome P-448) was estimated. No differences were found between the various groups. It is assumed that the induction of the mixed function monooxygenases is not an essential condition for the development or the persistence of the human porphyria cutanea tarda.
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PMID:[Cytochrome P-450 dependent enzymatic activity in the liver of patients with porphyria cutanea tarda (author's transl)]. 677 32

The existence of serum antibodies against porphyric or normal rat hepatocytes was investigated in patients with porphyria cutanea tarda and in other forms of chronic liver disease. Ten patients with porphyria cutanea tarda, 7 of them with chronic active hepatitis or cirrhosis (group 1a) and 3 without significant liver damage (group 1b), 8 patients with nonporphyric chronic active hepatitis (group 2), and 8 alcoholic cirrhotics, 3 of them with superimposed severe alcoholic hepatitis (group 3), were studied. In an antibody-dependent cell-mediated cytotoxicity test using isolated hepatocytes from normal and hexachlorobenzene-treated (porphyric) rats as targets, it was found that sera from group 1a produced high cytotoxicity against porphyric hepatocytes and low or zero cytotoxicity against normal hepatocytes (p less than 0.001). The opposite cytotoxic pattern was observed when sera from group 2 was tested. Sera from groups 1b and 3 failed to produce cytotoxicity against both targets. The cytotoxic effect on porphyric hepatocytes was significantly reduced by preincubation of serum with free uroporphyrin or by serum absorption with a sepharose-uroporphyrin immunosorbent. Immunofluorescence studies confirmed the existence of antiporphyric hepatocyte antibodies in group 1a. In conclusion, our results show that antiporphyric hepatocytes antibodies are present in some patients with porphyria cutanea tarda and indicate that hepatocellular porphyrin might be partially responsible for the antigenicity of the liver cells. The role of these antibodies in the pathogenesis of the liver lesion remains to be elucidated.
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PMID:Serum antibodies against porphyric hepatocytes in patients with porphyria cutanea tarda and liver disease. 684 Apr 77

Revision of 167 bioptic cases of porphyria cutanea tarda failed to confirm porphyrin crystals in diseased hepatocytes. The only peculiar finding was a fine honey-comb microvacuolisation of hepatocytes corresponding to dilated sacs of degranulated endoplasmatic reticulum in electron microscopy. The lesion was closely connected with regressive changes of mitochondria which often were giant and sometimes unspecifically paracrystalline in their inner structure. Steatosis and haemosiderosis were frequent and taken for secondary as well as the hepatic proliferation in terminal fibrosis or cirrhosis. Any increase of hepatocytic lipopigment was not found.
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PMID:[Changes in the liver in porphyria cutanea tarda]. 717 88

A case report is presented of a young woman in whom symptomatic porphyria cutanea tarda (PCT) developed during copper chelation therapy for Wilson's disease. The 22 year old white woman was seen in the summer of 1978 because of development of blisters on the dorsa of the hands associated with focal atrophic hypopigmentation, generalized hyperpigmentation of the skin, and hpertrichosis of the lateral forehead and face. A sibling had died in childhood with Wilson's disease. When the patient developed hepatomegaly, ascites, and an acute hepatitis syndrome at the age of 11, penicillamine therapy was empirically started, with gradual symptomatic improvement. When evaluated at the age of 22, abnormal laboratory values included a total bilirubin of 1.2 mg%; alkaline phosphatase, 96 U; serum glutamic oxaloacetic transaminase (SGOT), 175 U; serum glutamic pyruvic transaminase (SGPT), 122 U; gamma glutamyl trans peptidase (GGTP), 64 U; and Bromsulphalein (BSP) retention, 21% at 45 minutes. Skin biopsy from the hand revealed a noninflammatory subepidermal bulla with prominently PAS positive vessel walls in the festooned dermal papillae at the base of the blister. A fragmented liver biopsy failed to reveal evidence of active hepatitis or cirrhosis, but considerable stainable iron was present in both hepatocytes and Kupffer cells. A rubeanic acid stain for copper was negative. The patient was diagnosed as having Wilson's disease, hepatic hemosiderosis, and PCT. Cessation of all ethanol consumption and discontinuation of the oral contraceptives which she had been taking for 6 years, was recommended. On examination 9 and 22 months after these modifications were instituted, the patient felt asymptomatic and was without evidence of any new blisters or scars of her skin. The hyperpigmentation and hypertrichosis persisted, but she rigidly adhered to a program of penicillamine, topical sunscreen application, and abnegation of alcohol. Liver function studies were normal, and urinary porphyrin levels returned toward normal values. The clinical onset of this patient's blistering disease was temporally associated with ethanol and exogenous estrogen medication.
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PMID:Porphyria cutanea tarda complicating Wilson's disease. 720 91

In thirty-four patients with porphyria cutanea tarda treated with small doses of chloroquine, liver biopsies were performed before and after treatment. In seventeen cases (50%) the morphological patterns before treatment corresponded to unstabilized fibrosis, while in eleven (32.4%) there were non-specific changes in the form of focal fatty change, haemosiderosis, and mild fibrosis of the portal tracts. Active chronic hepatitis was found in three patients (8.8%), and cirrhosis also in three cases. Although in all patients a clinical and metabolic remission of porphyria cutanea tarda occurred during treatment, the morphological patterns in the liver parenchyma remained on the whole unchanged. Only in five cases was there an increase in the inflammatory changes, while in two patients these changes tended to disappear.
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PMID:Liver changes in porphyria cutanea tarda patients treated with chloroquine. 737 Jan 74


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