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Biochemical Studies in Thalassemia
- ANANDA S. PRASAD, M.B., B.S., PH.D.;
- M. DIWANY, M.D., M.R.C.P.(LONDON) ;
- MAMDOUH GABR, M.D.;
- HAROLD H. SANDSTEAD, M.D.;
- NEWAL MOKHTAR, M.B., B.S.; and
- ANISA EL HEFNY, M.B., B.S.
- Requests for reprints should be addressed to Ananda S. Prasad, M.D., Wayne State University School of Medicine, Department of Medicine, 1400 Chrsyler Expressway, Detroit 7, Mich.
Excerpt
Since the description of a syndrome comprised of erythroblastosis, splenomegaly, and anemia with a familial and racial incidence by Cooley and Lee (1) in 1925, many cases from various parts of the world have been reported (2-4). From Egypt, Diwany (5) first reported two cases in 1944, and since then others have been described (6-9). These reports have documented the presence of this disease in Egypt, but do not provide detailed biochemical and hematological data on the cases.
Abnormal iron metabolism in Cooley's anemia (thalassemia) has been established (10-12), and the presence of high serum levels of copper has been
Acknowledgments
We gratefully acknowledge the help of Dr. James H. Boyers, Commanding Officer, NAMRU-3, and Dr. William J. Darby, Professor and Chairman, Department of Biochemistry, Vanderbilt University, Nashville, Tenn., in the preparation of this manuscript. We also owe thanks to Mr. Atif R. Kozman for his valuable technical assistance.
Summario in Interlingua
Dece-un patientes con thalassemia major esseva studiate in Egypto. Lor crescentia esseva retardate marcatemente. Le anemia variava in severitate. Electrophorese de hemoglobina a gel de amylo (modificate con chloruro de ammonium trimethyloctadecylic) revelava le presentia de un componente characteristic designate como Tht. Ferro e cupro del sero esseva augmentate, sed le zinc del plasma esseva reducite significativemente. In vista de un recente reporto de carentia de zinc incontrate in association con nanismo human, le basse nivello plasmatic de zinc in iste subjectos con retardate crescentia es suggestive de un relation fundamental. Le nivello seral de dehydrogenase lactic (un enzyma a contento de zinc) esseva augmentate sed monstrava nulle correlation con le hemolyse. Purificate dehydrogenases del gruppo a zinc (tal como dehydrogenase lactic) es activate per le elimination de contamination de non-essential iones metallic, e quando zinc—que es essential pro le activitate del enzyma—es addite a illo post su purification, le subsequente elimination del zinc resulta in le activation del enzyma. Si o non isto pote explicar in parte le augmento de dehydrogenase lactic in despecto del reducite concentration de zinc in le plasma non pote esser determinate a iste tempore.
In conclusion: Multiple anormalitates del oligometallos esseva notate in patientes con thalassemia major. Un possibile relation inter le basse contento plasmatic de zinc e le retardate crescentia in iste subjectos es considerate. Un characteristic componente hemoglobinic (Tht) esseva notate in le electrophorese a modificate gel de amylo.
Article and Author Information
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From the Medical Department, U. S. Naval Medical Research Unit No. 3, Cairo, Egypt, U. A. R., the Division of Nutrition, Departments of Medicine and Biochemistry, Vanderbilt University, Nashville, Tenn., and the Pediatric Department, Faculty of Medicine, Munira Children's Hospital, Cairo University, Cairo, Egypt, U.A.R.
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This study was supported by research contract Nonr 2149 from the Navy Department, Office of Naval Research to Vanderbilt University, Nashville, Tenn., and by an agreement with the National Institutes of Health under Public Law 480.
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- Received July 9, 1964.
- Accepted August 25, 1964.
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