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Biomedical subjects

J Cohn

Publications and source records attributed to J Cohn.

At least 55 records · Page 3Linked to original sources

Hepatitis, type B in haemophiliacs. Relation to the source of clotting factor concentrates.

36 children aged 3 to 18 years receiving substitution therapy for haemophilia during the period 1970 to 1976 were studied for infection with hepatitis B virus by assays for HBsAg, anti-HBs, and anti-HBc. Clinical hepatitis B occurred in 3 patients (8%) and serological evidence of infection was found in further 13 (36%). The occurrence of infection was associated with age but less so with the total amount of transfusion. Estimates of the risk of infection by clotting factor material of different origin indicated a figure of 1:53,000 I.U. for Danish volunteer donor preparations as well as for commercial products, the risk being apparently increased following the use of pooled blood donor material and non-Scandinavian products respectively.

Adolescent

Transmission of hepatitis type B from healthy HBsAg-positive mothers.

Seventeen mothers, all apparently healthy carriers of hepatitis-B surface antigen (HBsAg) during pregnancy, and their children were studied for four to five years to determine the transmission rate of hepatitis-B virus infection. All the mothers had antibody against hepatitis-B core antigen in addition to HBsAg. One of them, a renal transplant recipient, was persistently positive for hepatitis-B-associated e antigen (HBeAg), while the remaining 16, who were detected during screening of healthy pregnant women were positive for anti-HBe. Evidence of infection was found in the child and husband of the woman positive for HBeAg, while none of the 29 children and five husbands of the anti-HBe-positive women became infected.

Antibodies, Viral

Foamy histiocytosis of the spleen in patients with chronic thrombocytopenia.

In 17 of the 64 spleens removed from patients with chronic thrombocytopenia lipid-laden histiocytotes were demonstrated. No correlation was found between these pathological findings and the age of the patients at diagnosis or splenectomy, the duration before splenectomy of therapy with glucocorticoids, the period with thrombocytopenia or the platelet count. 7 of the patients, who relapsed after splenectomy had lipid-laden histiocytes in their spleens i.e. 58% of the patients with relapse, compared with 10 patients with foamy histiocytosis and without relapse i.e. 19% of the patients without relapse. At follow up, 3 of the 17 patients with splenic histiocytosis still had thrombocytopenia, compared with 1 of the 47 patients without foamy cells. Foamy histiocytosis of the spleen from a patient with chronic thrombocytopenia may indicate a dubious prognosis.

Child

Thrombocytopenia in childhood: an evaluation of 433 patients.

An evaluation of 433 children with thrombocytopenia is presented. The material comprises all cases diagnosed in Denmark during the period 1959-1969. The incidence was found to be 31.9 cases per 1,000,000 inhabitants less than or equal to 15 years of age per annum with a peak at the age of 3 to 4 years; the majority of the cases was diagnosed in the spring. Preceding infection, usually associated with fever and localized in the upper respiratory tract, was the most common cause. The majority of the congenital cases was hereditary and associated with other malformations involving in particular the immune system. In about half of the cases the course was spontaneous and no treatment was necessary; the remaining patients were treated with glucocorticoids, including 46 patients, who had undergone splenectomy. 22 patients died, including 16 cases due to serious underlying disorders; in 6 cases the cause of death was haemorrhage (mortality rat=1.4%). At the follow up the platelet count was below 50 mia/1 in 14 patients (4%). No difference was found concerning the platelet count at the follow up between the patients with spontaneous recovery, the patients treated with glucocorticoids and the patients, in whom splenectomy had been performed. During the course the disease autoimmune haemolytic anaemia occurred in 2 patients, whereas no patient developed systemic lupus erythematosus.

Adolescent

Immunological studies in children before and after splenectomy.

Fourteen children underwent splenectomy for congenital spherocytosis, splenomegaly, or thrombocytopenia. The patients were studied twice before the operation, three times during the first postoperative month, and one year later. A transitory rise in neutrophils and serum IgA was seen postoperatively; there was a modest but long-lasting increase in lymphocytes and a marked elevation of eosinophils. An immediate decline in serum IgM concentration was observed only in patients with an uncomplicated postoperative course, but one year after splenectomy the average IgM concentration had decreased by 23%. The in vitro lymphocyte transformation response to a panel of mitogens and antigens fell in the immediate postoperative period but was largely normalized 10 days postoperatively, except in the youngest of the patients who had repeated infections following the splenectomy. One year postoperatively the transformation response and the number of T- and B-lymphocytes in the blood were normal.

Child

Intra-erythrocytary enzymes before and after splenectomy.

The activity of the intraerythrocytary enzymes glucose-6-phosphate dehydrogenase, pyruvate kinase, glutathione reductase and ATPase was measured before and after splenectomy in 13 patients with congenital hemolytic anemia and 3 patients suffering from chronic thrombocytopenia. All patients were treated successfully, as reflected by clinical and basal hematological parameters. Glucose-6-phosphate dehydrogenase and pyruvate kinase were significantly depressed after splenectomy. It was not possible to set up prognostic criteria of splenectomy from the intraerythrocytary enzymes.

Adenosine Triphosphatases

Amylo-1,60glucosidase deficiency (glycogenosis type III) in the Faroe Islands.

Seven cases of glycogenosis type III (amylo-1,6-glucosidase deficiency) in two probably related families from the Faroe Islands are presented. The group of patients comprised two pairs of sibs. In a total of 78 members of the two families case histories were obtained and clinical examinations, analyses of amylo-1,6-glycosidase activity in erythrocytes and leucocytes, determinations of red cell, serum and enzyme groups as well as HL-A types were performed. In addition, all patients were subjected to studies of liver function. The distribution patients in these families supports the assumption of autosomal recessive inheritance. Heterozygotes could not be diagnosed with certainty by the methods of enzyme activity analysis employed. The incidence of glycogenosis type III with amylo-1,6-glucosidase deficiency was found to be high in the Faroe Islands.

Adolescent

Sex-linked hereditary thrombocytopenia with immunological defects.

14 cases of severe thrombocytopenia in one family are presented. Case histories, clinical examination, analyses of platelets, haemoglobin, reticulocytes, leucocytes, eosinophilocytes, differential counts of leucocytes, serum immunoglobulin IgA, IgM, IgG, IgE concentrations, complement fixing platelet antibodies, isohaemagglutinins, colour perception, determination of red cell and serum groups as well as HL-A types were obtained from a total of 59 members of the family. The in vitro blast transformation response of blood lymphocytes was studied in 6 patients and 45 relatives. The pattern of transmission of the disease was in full agreement with X-linked recessive inheritance. Investigation of the immune system revealed impaired responses to microbial antigens in the 6 patients so studied. All relatives examined had normal haematological status, whereas approximately half showed a subnormal response to one microbial extract. The low responders were evenly distributed within the family, and it was not possible to correlate low response and presumed carrier state.

Adolescent