Search PubMed⌕ Search

Biomedical subjects

S Sherlock

Publications and source records attributed to S Sherlock.

At least 289 records · Page 16Linked to original sources

Red cell aplasia following prolonged D-penicillamine therapy.

Red cell aplasia developed in a case of Wilson's disease following an increase in D-penicillamine dosage after 14 years' treatment. In vitro study of the effect of D-penicillamine on 59Fe incorporation by marrow cells did not suggest that the patient's erythropoiesis was particularly sensitive to D-penicillamine or determine the mechanism of drug toxicity. However, three weeks after the drug was withdrawn, evidence of marrow regeneration was apparent, and within 10 weeks the haemoglobin had returned to normal. The patient has subsequently remained asymptomatic on an alternative chelating agent, triethylenetetramine dihydrochloride.

Adolescent↗

Pseudo-metastases in the liver: a presentation of the Budd-Chiari syndrome.

Angiographic and scintigraphic findings closely mimicked vascular liver metastases in a young woman whose Budd-Chiari syndrome is probably the result of oral contraceptive use. Histological proof should be obtained before the diagnosis of hepatic neoplasm is accepted in patients with this syndrome.

Adult↗

alpha-1-antitrypsin deficiency in liver disease: the extent of the problem.

A prospective screening program was undertaken at the Royal Free Hospital, London, to ascertain the incidence of alpha-1-antitrypsin (AAT) deficiency in patients with liver disease. Quantitative determinations of serum alpha-1-antitrypsin were performed on 469 patients with hepatobiliary disease and 98 subjects with no known liver disease. Sera with low values of AAT were phenotyped. The homozygous state was rare and comprised only 1% of the patients with liver disease. All of the 5 homozygous deficient (ZZ phenotype) patients had a history of neonatal liver disease. Other phenotypes (partial deficiency) were found in 4.7% of patients with liver disease and 6.1% of subjects with normal liver function. Types of liver disease in the patients with other phenotypes were widely varied. Routine determination of serum AAT level and phenotype and special staining for AAT in liver biopsies in all adults with liver disease appears unnecessary. Investigation of possible AAT deficiency should be carried out, however, in children and young adults, in those with a history of neonatal liver disease, and possibly in all patients with liver disease of unknown aetiology.

Adolescent↗

Peripheral blood lymphocyte populations in chronic liver disease.

Mature T lymphocyte concentrations are reduced, null cell concentrations are increased, and Fc receptor bearing (B and K) lymphocyte concentrations are normal, in the peripheral blood of patients with chronic hepatocellular or cholestatic liver disease. Some null cells can be stimulated by either thymosin or levamisole to form rosettes with sheep erythrocytes. These changes are present in viral, alcohol associated and 'autoimmune' liver disease and are therefore probably secondary phenomena relating to liver damage.

Adult↗

Double-stranded DNA-binding capacity of serum in acute and chronic liver disease.

Serum antibodies to double-stranded 'native' DNA have been measured in acute and chronic liver diseases using the Farr technique. Elevated levels of DNA binding were found in all groups of patients, with the highest levels in acute viral hepatitis and lowest in primary biliary cirrhosis. All patients with hepatitis B surface antigen-positive chronic active hepatitis had elevated levels, hence persistent elevation of DNA binding after acute type B hepatitis might be an unfavourable prognostic marker indicating progression to chronic active hepatitis, DNA antibody levels will not offer diagnostic help in liver diseases, or help to follow the response of patients with 'lupoid' hepatitis to corticosteroid therapy. Production of DNA antibody may be a response to release of DNA from damaged hepatocytes.

Alcoholism↗

The biliary system in primary biliary cirrhosis. A study by endoscopic retrograde cholangiopancreatography.

Endoscopic retrograde cholangiograms from 23 patients with primary biliary cirrhosis (PBC), 10 controls with either normal livers or hepatocellular disease, and 4 patients with sclerosing cholangitis, were compared. Of the PBC group, 39% had gallstones. The calibers of the common bile duct and left and right intrahepatic ducts were comparable in the PBC and control groups. The small intrahepatic ducts, while normal in the control group were abnormal in 7 of the 23 PBC patients. These small ducts were irregular in caliber and had a tortuous course. The changes were not related to the presence of gallstones or the duration of the disease, but all the patients had histologically proven cirrhosis. Two patients with cirrhosis had normal intrahepatic ducts. We conclude that whereas the major bile ducts are normal in PBC, there is a high incidence of gallstones (39%), and the changes that do occur in the intrahepatic ducts are probably related to the distorted hepatic architecture due to cirrhosis and may be used as a sign that cirrhosis has supervened.

Bile Ducts, Intrahepatic↗

Piperazine hepatitis.

The first reported case of piperazine toxicity resembling viral hepatitis is described in a 25-year-old woman. The illness, which was severe, occurred after administration of the drug on two separate occasions. The evidence favors a hypersensitivity mechanism rather than a direct toxic effect of piperazine or its metabolites on the liver. The humoral or cellular immune basis for such hypersensitivity has not been elucidated.

Acute Disease↗

A prospective controlled trial of azathioprine in primary biliary cirrhosis.

Between 1968 and 1974, azathioprine has been used in a controlled prospective trial to treat patients with symptomatic but precirrhotic primary cirrhosis. Forty-five patients were admitted, of whom 22 were given azathioprine in a dose of 2 mg per kg of body weight. During the 1st year, serum aspartate transaminase levels showed a significant change in favor of the treated group, but improvement did not continue. Throughout the trial, serum alkaline phosphatase, bilirubin, cholesterol, albumin and immunoglobulin M values showed no significant change. Titers of serum mitochondrial antibodies tended to become negative more often in the treated than the untreated. Pruritus cannot be assessed objectively, but seemed less in the treated than in controls. Serial hepatic biopsy specimens showed the development of cirrhosis equally in the two groups. Survival, as judged by the life table method, was similar for the first 5 years of the trial. There was, however, a significant difference in favor of the treated group in the 6th year, although the number of patients available for assessment at that time was extremely small.

Azathioprine↗

A randomized trial of percutaneous transhepatic cholangiography with the Chiba needle versus endoscopic retrograde cholangiography for bile duct visualization in jaundice.

Sixty consecutive patients, who were deeply jaundiced or in whom intravenous cholangiography had failed, were randomized to retrograde endoscopic cholangiography or percutaneous transheptic cholangiograhy with the "skinny" Chiba needle technique. Twenty-eight patients were assigned to retrograde cholangiography, which succeeded in 17 (65%). Percutaneous cholangiography was successful in 16 (50%) of the remaining 32 patients. When patients in whom the first procedure was unsuccessful were reinvestigated by the alternative technique, retrograde cholangiograms were obtained in 13 (81%) of 16, and percutaneous cholangiograms in 8 (73%) of 11. Thus, one or the other technique was successful in 54 (90%) of 60 patients. When the results were analyzed separately for extrahepatic (29 patients) or intrahepatic (31 patients) cholestasis, percutaneous cholangiography was successful in 95% of patients with extrahepatic cholestasis but in only 25% with intrahepatic cholestasis. Endoscopic retrograde cholangiography successded in 63% of patients with extrahepatic and 76% with intrahepatic causes of cholestasis. Complications occurred only in patients with extrahepatic cholestasis. Cholangitis and septicemia occurred in 1 patient after retrograde cholangiography and in 2 after the percutaneous technique. An intraperitoneal bile leak occurred in one other patient after percutaneous cholangiography. Percutaneous cholangiography with the narrow needle is a simple, inexpensive, and reliable method for demonstrating the biliary system and is usually successful when an extrahepatic cause of cholestasis is present. The occurrence of serious complications in patients with extrahepatic cholestasis, despite prophylactic antibiotics, makes provision for early surgery mandatory after both techniques.

Adult↗