摘要:

Most of the hereditary sideroblastic anaemias are inherited as x-linked recessive traits and are often pyridoxine responsive. The present paper describes the classical features of sideroblastic anaemia in 2 male and 2 female siblings of a Libyan family. All 4 children had severe anaemia and moderate hepato-splenomegaly. The equal severity of the disease in all 4 family members suggests autosomal inheritance.

ISSN:0001-5792    

DOI:10.1159/000207001

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Some of the constant characteristics of Mendelian autosomal dominant diseases are non-penetrance and variable expressivity. However, sickle cell anaemia (SSA) is probably unique among the autosomal recessive diseases in the variable expressivity of its natural history. It therefore seems likely that other factors extraneous to the sickle gene locus may play a part in full phenotypic expression of SSA. To find out the possible role of red cell antigens in the variable expressivity of SSA, ABO and Rhesus antigens have been determined in 200 electrophoreücally confirmed Hb SS patients and 250 sex- and age-matched Hb AA controls; the Hb SS group was subsequently divided into ‘mild’ and ‘severe’ sicklers and the results analysed. There were no statistically significant differences in the ABO and Rhesus blood group antigen distributions between the sicklers and Hb AA controls. There were also no correlations between the severity of the Hb SS disease and either the ABO or Rhesus group distributions. We conclude from these studies that ABO or Rhesus blood group antigens do not have significant moderating effect on the natural histor

ISSN:0001-5792    

DOI:10.1159/000207002

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

3 cases of aplastic anaemia occurring in association with anti-tuberculosis chemotherapy are reported. The patients had been on anti-tuberculosis chemotherapy for 13, 11 and 14 months, respectively, prior to the diagnosis of aplastic anaemia. Agents used included streptomycin, thiacetazone, isoniazid, p-aminosalicylic acid and dimethylcarbazine. Recovery from aplasia did not occur 6, 1.5 and 0.8 months, respectively, after the discontinuation of the suspect myelotoxic agents and despite the use of myelostimulatory agents. All 3 patients died of haemorrhage secondary to thrombocytopenia. The observations are consistent with a protracted and probably irreversible damage of the bone marrow by anti-tuberculosis agent(s) in susceptible individuals.

ISSN:0001-5792    

DOI:10.1159/000207003

出版商:S. Karger AG    

年代:1982

数据来源: Karger

摘要:

Plasma factor VIII procoagulant activity was determined in rabbits in hypoxic and hyperoxic conditions and was found significantly elevated in both conditions. Although the elevations were not enough to influence the partial thromboplastin time, they were consistently present in each animal in hypoxia as well as hyperoxia.

ISSN:0001-5792    

DOI:10.1159/000207004

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207005

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207006

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207007

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207008

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207009

出版商:S. Karger AG    

年代:1982

数据来源: Karger

ISSN:0001-5792    

DOI:10.1159/000207010

出版商:S. Karger AG    

年代:1982

数据来源: Karger