Biomedical subjects
Publications and source records attributed to G Humphries.
In a Greek Cypriot family in which genes for both alpha and beta thalassaemias were expressed, haematological and biosynthetic investigations indicated that one family member was homozygous for beta thalassaemia and had alpha-thalassaemia1 trait. The concurrent inheritance of an alpha-thalassaemia gene in the beta-thalassaemia homozygote seemed to have modified his degree of chain imbalance and to have reduced the clinical severity of the disease.
The surgical aspects of the management of patients with homozygous beta-thalassaemia are reviewed, and the operative findings in 10 patients with the disease who underwent splenectomy are described. Postoperatively, blood consumption was reduced and there was a longer time interval between transfusions needed to maintain the haemoglobin level. Hyperplastic lymph nodes containing erythropoietic elements are always present, and the first reported case of extrabiliary obstructive jaundice caused by this tissue is included. All the patients survived for 2-3 years after treatment and there were no serious infections. All received prophylactic oral penicillin after surgery. Cholelithiasis was not found. Two malleolar ulcers were successfully grafted with split skin after preparation of the craters with silver sulphadiazine cream.
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