PubMed Health⌕ Search

Biomedical subjects

A L Markenson

Publications and source records attributed to A L Markenson.

13 recordsLinked to original sources
PubMed1981

Impaired T-cell mitogen responses in some patients with thalassemia intermedia.

Ten thalassemia intermedia patients were tested for their mitogenic responses to phytohemagglutinin P (PHA), concanavalin A (Con A) and pokeweed mitogen (PWM). Impaired responses to PHA and Con A were observed in patients with serum-iron levels higher than 200 microgram/dl. Responses within or slightly higher than those in the normal range were observed in patients with serum-iron lower than 200 microgram/dl. By contrast, no significant changes occurred in the majority of the responses to PWM.

Adolescent↗
PubMed1980

Depressed serum somatomedin activity in beta-thalassemia.

We have found bioassayable somatomedin activity to be subnormal in 20 of 32 children and adults with beta-thalassemia. The levels were comparable to values reported in growth hormone-deficient subjects. Since patients with thalassemia are not growth hormone deficient, the data suggest the possibility of defective hepatic biosynthesis of somatomedin. Increased iron stores in these patients, who have secondary hemosiderosis of many organs, including the liver, may depress somatomedin activity. Therapy for one year with daily subcutaneous infusions of the iron-chelating agent deferoxamine had no effect on mean bioassayable serum somatomedin activity.

Adolescent↗
PubMed1978

Thalassemic osteoarthropathy.

Patients with beta thalassemia major may develop a specific osteoarthropathy as they approach the second and third decades of life. Twenty-five of 50 patients between the ages of 5 and 33 years had evidence of periarticular disease that consisted of dull-aching ankle pain exacerbated by weight bearing and relieved by rest. Involvement was symmetrical with mild swelling and pain on bone compression. Arthrocentesis showed no evidence of inflammation. Radiographic changes included osteopenia, widened medullary spaces, thin cortices with coarse trabeculations, and evidence of microfractures. Histologic studies confirmed the presence of microfractures and showed osteomalacia and an increase in osteoblastic and osteoclastic surface area with iron deposites at the calcification front and cement lines. The relative roles of iron overload, divalent cation metabolism, erythroid hyperplasia, or other factors in the pathogenesis of the syndrome remain unknown, and no specific therapy can be recommended at present.

Adolescent↗
PubMedDate not supplied

HLA-A, B, C, and DR antigen frequencies in relation to development of diabetes and variations in white cell antibody formation in highly transfused thalassemia patients.

Eighty-five patients with thalassemia and all available immediate family members were typed for HLA-A,B, C, and DR antigens, and the patients were tested for clinical diabetes and white cell antibodies in response to multiple blood transfusions. The antigen Bw35 was increased among both patients and their parents. This finding is consistent with previous data suggesting that this antigen may offer an independent selective advantage in populations at risk for both thalassemia and malaria. No association of the HLA system to the development of diabetes was noted. A wide variation was observed in the degree of white cell antibody response to transfusions: 25 of the 84 patients tested had significant levels of white cell antibodies while the majority (49) of the patients had essentially no antibodies. The frequency of the antigen DR2 was significantly increased in the high-response group, while the antigens Bw35 and DR7 were significantly increased in the low-response group. This finding suggests that an HLA-linked immune response or immune suppression factor or an HLA-linked susceptibility to iron toxicity may play a role in the development of these antibody responses.

Blood Transfusion↗
PubMedDate not supplied

Lens opacities in thalassemia.

This is the first case report of cataracts in patients with thalassemia major. Desferrioxamine, an iron-chelating agent is being used with increasing frequency in the treatment of transfusion-induced iron overload. There has been some concern in the literature about possible cataract formation with use of this drug. It is therefore important to document any lens opacities seen prior to administration of desferrioxamine, or record the appearance of lens opacities after its use. The possible eitology of these lens opacities is discussed.

Adolescent↗