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Biomedical subjects

G Klatskin

Publications and source records attributed to G Klatskin.

At least 19 recordsLinked to original sources
PubMed1985

Asymptomatic primary biliary cirrhosis. A progress report on long-term follow-up and natural history.

Thirty-six patients presenting with asymptomatic primary biliary cirrhosis have been followed for a median period of 11.4 yr, extending by 5 yr a previously reported median follow-up study of 6 yr. Life table survival analysis indicates that the overall survival of this subgroup of patients with primary biliary cirrhosis continues to remain similar to that of the general population (p = 0.91). Over this period, 15 patients developed symptoms and 8 patients died, 6 from liver disease; 21 patients remained in an asymptomatic state. Portal granulomas on initial liver biopsy were the only finding that correlated with a normal survival and a continued asymptomatic state (p = 0.03). In contrast, associated autoimmune disorders (thyroiditis, sicca syndrome, CRST syndrome, Raynaud's phenomenon) correlated with decreased survival (p = 0.01). No other clinical, laboratory, or histologic features correlated with survival or the development of symptoms. This extended follow-up study (median 11.4 yr) indicates that many patients with asymptomatic primary biliary cirrhosis have a benign outcome. Although 42% developed signs or symptoms of progressive disease at variable times up to 14 yr from presentation, the group survival remained similar to the general population.

Adult↗
PubMed1983

The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis.

To determine the life expectancy of patients with primary biliary cirrhosis, we analyzed survival data from 280 patients with either symptomatic (243) or asymptomatic (37) disease. Patients were followed for up to 19 years (mean, 6.9 years). The average length of survival was 11.9 years--nearly twice that reported in other studies. In contrast, over a 12-year period the survival of the asymptomatic patients after diagnosis did not differ from that of a control population matched for age and sex. Jaundice, weight loss, hepatomegaly, splenomegaly, and ascites were each associated with a poor prognosis. Prognosis also correlated with the histologic stages of hepatic fibrosis, cholestasis, and periportal-cell necrosis. A multivariate analysis of clinical features revealed that at the onset of disease, age, hepatomegaly, and elevated levels of serum bilirubin were independent discriminators of a poor prognosis. A histologic finding of fibrosis limited to portal areas improved this discrimination, correlating with prolonged survival. No other factors enchanced the prediction of risk.

Ascites↗
PubMed1979

Enzymatic fluorometry for estimating serum total bile acid concentration.

Fasting serum total bile acid (SBA) levels were estimated by enzymatic fluorometry (EF) in 36 subjects without liver disease, 28 with hepatic lesions and impaired hepatic function, and 79 with hepatic lesions and normal function. Fasting and postprandial EF-SBA levels were compared in nine normal subjects and nine patients with cholestasis, and SBA assays by EF and gas-liquid chromatography (GLC) were compared in 28 patients with hepatic lesions and impaired function. Levels of SBA were below 9 mumole/L in all but two of the 36 subjects without liver disease, and above that level in all 28 with impaired hepatic function and 17 (24%) of the 70 with hepatic lesions and normal liver function. In most subjects, EF detected notable postprandial rises in SBA. Enzymatic fluorometric and GLC-SBA values were closely correlated (coefficient of correlation, 0.869).

3-Hydroxysteroid Dehydrogenases↗
PubMed1979

Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.

Arteriohepatic dysplasia (Alagille's syndrome) is presumed to be one of the familial intrahepatic cholestatic syndromes, all of which present with neonatal jaundice or failure to thrive, or both. We report the findings in five patients with this syndrome, four of whom have been followed into adulthood. In addition to hepatic dysfunction, patients had abnormalities of the cardiovascular system, eyes, bones, central nervous system, kidney, endocrine system, and habitus. Analysis of these cases allows a more complete characterization of this syndrome and shows that the cholestasis improves, although the abnormalities of the hands and face become more pronounced, with age. Patients with arteriohepatic dysplasia display the variability in expression seen in many autosomal-dominant conditions. New findings in the eye and spine provide markers specific for this syndrome and serve to differentiate it from other forms of cholestatic liver disease.

Adolescent↗
PubMed1978

A father and son with cholestasis and peripheral pulmonic stenosis: a distinct form of intrahepatic cholestasis.

We report a father and son with the syndrome of cholestasis and peripheral pulmonic stenosis. These cases demonstrate the clinical and biochemical features noted in previous reports of this entity and allows us to differentiate clearly this syndrome, with its benign course, from other more progressive forms of intrahepatic cholestasis. The vertical transmission supports a genetic etiology for this disease. Although serum bile acid levels are elevated in these patients, the individual bile acids do not display a distinctive pattern and no abnormal bile acids are identified.

Adult↗
PubMed1978

Halothane hepatitis: benign resolution of a severe lesion.

Three patients with halothane hepatitis were studied during the acute phase of their illness and for 10 to 14 months thereafter. Clinical, biochemical, and histologic data were obtained initially and during the course of follow-up. Despite initially severe clinical and biochemical presentations, with extensive bridging hepatic necrosis on liver biopsy, all three patients resolved completely and had minimally abnormal liver biopsy appearances at last follow-up. The results of this study suggest that hepatic necrosis associated with halothane hypersensitivity is self-limited and that despite the initial severity of the hepatic lesion, postnecrotic cirrhosis does not develop. Based on these three patients' courses, survival of the acute bout of halothane hepatitis is apparently accompanied by an excellent prognosis ultimately, provided that reexposure to halothane is avoided.

Acute Disease↗
PubMed1978

Hepatitis B in hemodialysis patients: significance of HBeAg.

Twenty-four HBsAg-positive (HBsAg+) hemodialysis patients were prospectively studied to assess (1) the prognostic value of HBeAg and its HBe1Ag and HBe2Ag components and (2) whether a difference in cellular immune status between HBeAg+ and HBeAg-negative (HBeAg-) patients could be defined. Sixteen patients were HBeAg+ and 8 were HBeAg- initially. After a mean follow-up period of 23.7 months, it was concluded that the initial presence of HBeAg correlated with the persistence of HBsAg because 15 of the 16 HBeAg+ patients were still HBsAg+ at death or last follow-up, whereas 7 of the 8 HBeAg- patients had become HBsAg- at a mean period of 3.8 months. HBe1Ag was consistently present in 15 of the 16 patients when HBeAg was detectable, whereas HBe2Ag fluctuated widely in individual patients over time. No difference in cellular immune status between HBeAg+ and HBeAg- patients could be defined; although both HBeAg+ and HBeAg- patients had a similar decrease in peripheral blood T cells and poor responsiveness to purified HBsAg, both groups of patients had stimulation indices to nonspecific mitogens within the normal range.

Adult↗
PubMed1977

Identification of lymphocytes in percutaneous liver biopsy cores. Different T:B cell ratio in HB sAg-positive and -negative hepatitis.

The lymphocytes infiltrating the liver were isolated and characterized as T or B cells in three groups of patients: 20 patients with hepatitis B surface antigen (HB sAg)-positive acute and chronic hepatitis, 8 patients with HBsAg-negative chronic hepatitis with prior evidence for hepatitis B virus (HBV) infection, and 5 patients with HBsAg-negative chronic hepatitis without prior evidence for HBV infection. The predominant cell infiltrating the liver was shown to be a T cell in all categories; however, the ratio of T:B cells was significantly lower (1.96) in the patients without evidence for HBV infection than in the patients who were HBsAg-positive at (7.86), or before (8.85) the time of study. The significantly (P less than 0.001) higher number of B cells in the patients with chronic hepatitis of unknown etiology suggests that a different immunopathogenetic mechanism is operative in this group. A peripheral T lymphocytopenia was observed in patients with both antecedent and existent HBs-antigenemia, but not in the patients without evidence for HBV infection.

Acute Disease↗
PubMed1977

Serum alpha-fetoprotein in patients with massive hepatic necrosis.

Serum concentrations of alpha-fetoprotein (AFP) were measured by radioimmunoassay in 12 patients with massive hepatic necrosis, 11 of whom died. Levels were significantly elevated after the 8th day of illness in 8 of the 9 patients who died between the 10th and 60th day, and in the 1 patient who survived. All 9 patients with increased levels of serum AFP exhibited histological evidence of hepatic regeneration. These findings indicate that the rise in serum AFP in massive hepatic necrosis is related to the duration of survival after the onset of illness, but does not necessarily imply ultimate recovery. Because available evidence suggests that the serum AFP level reflects hepatic regenerative activity, it appears that the onset of regeneration in fulminant hepatitis is delayed until the 2nd week of illness.

Adolescent↗
PubMed1977

Elevations in skin tissue levels of bile acids in human cholestasis: relation to serum levels and topruritus.

To define the relationship of bile acid retention to the pruritus of cholestasis, we quantified individual bile acids in serum, acetone swabs of skin, and skin tissue in 13 patients with cholestasis undergoing laparotomy and in 8 controls. There was no consistent relationship between pruritus and concentrations of either total or individual bile acids in serum. Skin tissue concentrations of bile acids were elevated in patients with cholestasis, were linearly related to serum levels, and did not differentiate between those patients with and those without pruritus. Concentrations of bile acids on the skin surface, which were lower than those reported by others, did not correlate with pruritus, and were decreased by simple soap and water washing. These data indicate that the pruritus of cholestasis is not directly related to the skin tissue concentration of any of the major bile acids, although a relationship to a particular molecular form of bile acids could not be excluded.

Adult↗
PubMed1976

Nonhuman primate-associated viral hepatitis type A. Serologic evidence of hepatitis A virus infection.

Since 1961, viral hepatitis has been recognized as an occupational hazard among handlers of newly imported chimpanzees and other nonhuman primates. To determine whether previously reported cases were caused by human viral hepatitis type A, we tested paired serum samples from two outbreaks for antibody to hepatitis A antigen (anti-HA) by immune adherence hemagglutination (IAHA), recently available test. In both outbreaks, one of hepatitis transmitted from chimpanzee to man (Michigan, 1964), the second from chimpanzee to chimpanzee, man, and woolly monkey (Connecticut, 1971), serologic data documented recent hepatitis A virus infection among contacts-human and nonhuman primate-of implicated chimpanzees. This confirms serologically a previously noted epidemiologic association between nonhuman primate-associated hepatitis and human viral hepatitis, type A.

Animals↗
PubMed1976

Serum bile acids in alcoholic liver disease. Comparison with histological features of the disease.

Fasting serum bile acids were measured by gas-liquid chromatography in 64 patients with alcoholic liver disease and compared with histological features in their percutaneous liver biopsy specimens. Total bile acid concentrations were normal (less than 2 mug/ml) or minimally increased in 6 patients in whom fatty infiltration was the only hepatic lesion. In the remaining 58 patients with more severe histological lesions, levels were increased in 93%, whereas serum bilirubins were elevated in only 43%. Chenodeoxycholic acid was usually the predominant serum bile acid, regardless of the degree of necrosis or connective tissue change in the biopsy specimen. Only small amounts of the secondary bile acids, deoxycholic acid and lithocholic acid, were detected. Levels of these secondary bile acids did not correlate with histological features.

Alcoholism↗
PubMed1976

Hepatic granulomata: problems in interpretation.

Granulomata occur in the liver not only in patients with systemic granulomatous disease, but also in a variable number with underlying liver disease and in a heterogeneous group of disorders that appear to be neither hepatic nor granulomatous in nature. The hepatic granulomata found in association with liver disease are rarely attributable to complicating systemic granulomatous disease, and probably represent a nonspecific response to the underlying hepatic disease. In the heterogeneous group of diseases that appear to be neither hepatic nor granulomatous in nature, hepatic granulomata may (in some instances) represent a nonspecific response to such conditions as intraabdominal malignancy and ulcerative bowel disease. However, in others, particularly those with unexplained prolonged fever, hepatic granulomata may be attributable to specific agents that are overlooked or escape detection by currently available diagnostic measures. The etiology of hepatic granulomata can seldom be established on histological grounds alone, and usually requires collateral clinical and laboratory evidence for identification.

Biopsy, Needle↗