Hematological and clinical data are presented for a young Malay patient with a homozygous (delta beta)zero-thalassemic condition. His red blood cells contained 100% fetal hemoglobin with alpha and G gamma chains only. Detailed gene mapping defined a large deletion with a 5' end between the Aha III and Apa I sites, some 200-400 bp 5' to the A gamma globin gene and a 3' end beyond sequences 17-18 kb 3' to the beta globin gene. This G gamma (A gamma delta beta)zero-type of thalassemia is different from all the other six types described before. Comparison of the hematological data of this patient with those of homozygotes for either the Sicilian or Spanish types of G gamma A gamma (delta beta)zero-thalassemia showed no differences; all homozygotes have a moderate anemia which is accentuated by the relatively high oxygen affinity of the Hb F containing erythrocytes.
Rajakumar MK, Ahmad MD, Balasundaram R, Low BT, Tan FEH, Wan KC, et al.
Citation: Rajakumar MK, Ahmad MD, Balasundaram R, Low BT, Tan FEH, Wan KC, Catterall RA. Specialisation in Primary Health Care: Training for the New General Practice in Malaysia. Kuala Lumpur: College of General Practitioners Malaysia, 1986
MeSH terms: Education, Graduate; Family Practice; Malaysia
Skeletal proteins play an important role in determining erythrocyte membrane biophysical properties. To study whether membrane deformability and stability are regulated by the same or different skeletal protein interactions, we measured these two properties, by means of ektacytometry, in biochemically perturbed normal membranes and in membranes from individuals with known erythrocyte abnormalities. Treatment with 2,3-diphosphoglycerate resulted in membranes with decreased deformability and decreased stability, whereas treatment with diamide produced decreased deformability but increased stability. N-ethylmaleimide induced time-dependent changes in membrane stability. Over the first minute, the stability increased; but with continued incubation, the membranes became less stable than control. Meanwhile, the deformability of these membranes decreased with no time dependence. Biophysical measurements were also carried out on pathologic erythrocytes. Membranes from an individual with hereditary spherocytosis and a defined abnormality in spectrin-protein 4.1 association showed decreased stability but normal deformability. In a family with hereditary elliptocytosis and an abnormality in spectrin self-association, the membranes had decreased deformability and stability. Finally, membranes from several individuals with Malaysian ovalocytosis had decreased deformability but increased stability. Our data from both pathologic membranes and biochemically perturbed membranes show that deformability and stability change with no fixed relationship to one another. These findings imply that different skeletal protein interactions regulate membrane deformability and stability. In light of these data, we propose a model of the role of skeletal protein interactions in deformability and stability.
A study was undertaken to evaluate the efficiency of health services at two maternal and child health (MCH) clinics in Kuala Lumpur. The time spent for individual patient care by the clinic staff was used as an indicator for the evaluation. The main objective was to derive a model of ideal operation time for specific patient care activities at a MCH clinic. Primary data was collected through systematic random sampling of patients between 25/7/1985 and 31/7/1985. Secondary data was obtained from available clinic records for July, 1985. The time a patient spent at the clinic and the time she spent at each examination station and waiting between stations was recorded with digital watches. Results show tha patients spent 80% of their time at the clinics waiting. The actual time spent on each patient was found to be considerably less than the ideal time measured under ideal conditions. Various recommendation are presented in the paper to reduce waiting time and extend examination time. The ideal patient capacity for the two clinics was also calculated.
MeSH terms: Ambulatory Care Facilities; Humans; Malaysia; Maternal-Child Health Centers