[Familial hyperlipidaemia and sea-blue histiocyte syndrome ].
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Biomedical subjects
Publications and source records attributed to J Vilaseca.
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Two cases of transitory IgM monoclonal gammopathy associated with infectious disease are presented. One patient was a 29 year-old woman with acute brucellosis; electrophoresis showed a homogeneous spike of slow gamma mobility constituted of IgM lambda. The second case was a 52 year-old male with milliary tuberculosis, in whom a homogenous spike of rapid gamma mobility constituted of IgM kappa was found. The monoclonal component disappeared in both cases after treatment of the infections; at 2 and 21/2 months respectively. The fact that the association of transitory IgM paraprotein and infection is not common, especially with brucellosis, is commented upon and a direct relationship between the infection and the monoclonal component is suggested.
Serum activity of glutathione reductase (GR), glucose phosphate isomerase (GPI), aspartate aminotransferase (AST), alanine aminotransferase (ALT) phosphate alkaline (PAL), and gamma-glutamyl transferase (GGT) was studied in 142 patients, in all serum bilirubin was more than 2 mg/dl. Distribution was as follows; 68 cirrhosis of the liver; 27 acute hepatitis; 31 benign extra-hepatic biliary obstruction; and 16 neoplastic obstruction of the biliary tract without liver metastasis. Fifty-three healthy volunteer blood donors were used as the control group. Mean values for GR activity in our patients were significantly higher than those for the control group, although less so in benign obstruction (p less than 0.01) than in those with acute hepatitis (p less than 0.001), cirrhosis (p less than 0.01) and neoplasic biliary obstruction (p less than 0.001). The GPI values were higher than the control groups in patients with acute hepatitis (p less than 0.001) and obstructive neoplastic jaundice (p less than 0.02). In cases with cirrhosis, 87% presented slightly higher values of GR, while GPI was within normal levels in 93 % of all cases. In patients with acute hepatitis, 92% showed a definite increase in GPI and GR values. In 71% of those with benign biliary obstruction levels for both enzymes were normal, as they were in only 6% of those with obstructive neoplastic jaundice. These findings are statistically significant in all cases and of diagnostic value in establishing a differential enzymatic diagnosis in patients presenting with clinical and biological patterns of cholestasis.
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In 38 patients suffering from rickettsiosis caused by Rickettsia conorii (Mediterranean Exanthematous Fever), hepatic involvement was studied via laboratory tests and in 26 cases by means of liver biopsy. SGOT, SGPT and alkaline phosphatase were found to be elevated in more than half of the patients (SGOT 74.4 +/- 93 U.K., SGPT 82.2 +/- 93 U.K., a.p. 58 +/- 21 mU/ml). In 14 patients, liver biopsy showed the existence of inclusion corpuscles in Kupffer's cells. Electron microscopic study demonstrated the existence of phagosomes inside the epithelioid cells, which, however, were difficult to categorize. The frequent existence of granulomatous hepatitis in this rickettsiosis was confirmed, while the presence of the infecting agent in the liver could not be established.
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A 27-year-old male developed an autoimmune hemolytic anemia during the initial episode of chronic ulcerative colitis. Laboratory studies revealed a positive direct Coomb's test (IgG + C), and the presence of specific anti-Hr antibody. Salicylazosulfapyridine was tested by means of immunohematologic techniques, but results discarded this drug as the responsible agent for autoimmune hemolytic anemia. Association of ulcerative colitis and autoimmune hemolytic anemia is an uncommon finding; around 36 cases have been published in the literature. Hemolytic anemia may develop as a consequence of treatment with salicylazosulfapyridine, the antimicrobial of choice in the management of inflammatory diseases of the bowel.
Association of primary liver carcinoma with virus of type B hepatitis has been demonstrated around the world, especially in African and Asian countries where high titles for surface antigen of B hepatitis (HBsAg) have been found. Presence of viral markers of B hepatitis has been studied in a group of 34 patients with primary liver carcinoma, in 139 cirrhotic patients, and in 100 normal individuals. Positive titles of HBsAg or of antibodies against "core" antigen (anti-HBc) without evidence of antibodies against HBsAg (anti-HBs) were considered as positive markers for virus B infection. Percentages of positive markers in the series studied were as follows: 52% in the cases of primary liver carcinoma, 38% in the cirrhotic cases, and 5% in the control subjects. There is a significant difference in relation to the incidence of viral markers between patients with liver carcinoma and normal controls. A possible role of the virus of B hepatitis in the pathogenesis of primary liver carcinoma could be suggested on the basis of these results.
The clinical and bacteriological characteristics of eight cases with purulent pericarditis observed over the last five years are studied. The route of the infection and dissemination in the majority of the cases (75 percent) was through pleuropulmonary lesions in the form of pneumonia and/or empyema, attributing the remaining cases to a subhepatic abscess and a pericardial infection after a thoracic surgical operation. In seven patients the diagnosis of the disease was established while they were alive. The more orientative clinical data were the pericardial pain (50 percent), pericardial friction murmur (25 percent), and signs of cardiac tamponade (62.5 percent). The observation of the above mentioned clinical signs together with the presence of cardiomegaly and electrocardiographic alterations suggestive of pericarditis, obliged the practice of a pericardial puncture, which confirmed the diagnosis of a purulent pericarditis by the macro and microscopic characteristics of the fluid. Staphylococcus and pneumoncoccus were isolated in two cases, respectively; other Gram-negative bacillus (E. coli and Pseudomonas aeruginosa) were isolated in the remaining cases. All patients were treated with the appropriate antibiotic according to the isolated germ; surgical drainage was carried out in six cases, and a pericardiectomy in one. Two patients died, one as a consequence of a septic myocardiopathy and the other in which the diagnosis of purulent pericarditis was not clinically suspected. During the follow-up period one case presented a constrictive pericarditis, which was corrected by a pericardiectomy.
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