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associated with some defect of normal hemoglobin production. The latter two conditions are frequently difficult to distinguish. The G6PD variants are also distinguished by the prefix Gd, for example, GdMadrona. The positive variants are not indicated as such, but the negative ones are, for example, GdB and GdA-. In some studies the negative variants are not so distinguished since they were not so indicated in the source. The G6PD deficiency is indicated by gdef. In many studies partial or intermediate or weak deficients are differentiated from total deficients and are abbreviated as part, int, or wk, respectively. Many times, and especially in females, different genotypes cannot be separated, so that these are indicated by a "/" meaning "or" as, for example, GdAA/AAor GdAB/A-B. Many different notations have been used for the thalassemias, primarily because of the many kinds of tests involved. Osmotic Resistance (OsmRes) is the least accurate test of thalassemia since other conditions can result in smaller red cells. And, of course, it does not differentiate between a and {3-thalassemia. Elevated amounts of A 2 and fetal hemoglobins have been the most used tests for (3-thalassemia,and these have been recorded as individuals with an elevation of either one of these or both. For example, A2 = 2/A2F = 4/F = 3(9.0), where the "/" means "or" and the percentage refers to the total number with any elevation. More recent and sophisticated techniques can now distinguish between {30 and {3+-thalassemia which have no or some {3 chain production, respectively. Some individuals have now been found to have deletions of one or two a chains on a chromosome, and these are recorded as - - or -a, where the normal allele is aa. Thus, the genotypes are given as - - /aaor -a/-aorevena-/a-,allofwhich have two deleted a genes, and the "/" in this case'does not mean "or" but rather "and." 12 Finally, in some few cases two different systems have been examined, and a "+" has been used in these cases, for example, AS+gdef means heterozygosity for hemoglobin Sand G6PD deficiency. A great many assumptions have been necessary at times to reduce the data to this simplified form, and there are inconsistencies as to when gene frequencies have been calculated or percentages of the raw data computed. But the actual numbers are presented whenever possible. Some investigations, however, did not give the number examined so that only the percentage with the abnormal phenotype is known, or in some cases the recording of a hemoglobin variant in a particular country was considered to be of sufficient significance to be reported as an individual case or family study. This computer file is an ongoing project, and any unpublished or omitted data on the frequencies of these genes would be appreciated as additions to our knowledge.
Polynesia Micronesia Melanesia New Guinea Australia Philippines Indonesia Malaysia Singapore Burma Thailand Cambodia Laos Vietnam China Taiwan Korea japan' Mongolia Sri Lanka India Bangladesh Bhutan Sikkim Nepal Pakistan Afghanistan Iran Iraq Iraq & Turkey SEQUENCE OF COUNTRIES The sequence of the countries or areas in which the data are organized is shown below (Polynesia is first. Micronesia second, and Ecuador last); an index with page numbers is also provided. Kuwait Bahrain & Qatar United Arab Emirates Muscat & Oman South Yemen Aden Yemen Saudi Arabia jordan Syria Lebanon & Syria Lebanon Israel Cyprus Malta Turkey Greece Yugoslavia Bulgaria Romania U.S.S.R. Poland Hungary Czechoslovakia Italy Spain Portugal France Switzerland Germany Benelux Denmark Norway Sweden Finland Iceland Great Britain Europe Egypt Libya Tunisia Algeria Morocco Mauritania Cape Verde Senegal Guinea Guinea-Bissau Gambia Sierra Leone Liberia Ivory Coast Mali Upper Volta Niger Ghana Togo Benin Nigeria Chad 13 Cameroon Sao Tome & Principe West Africa Central African Republic Equatorial Guinea Gabon Congo Republic Zaire Angola Botswana Namibia South Africa Mozambique Malawi Zimbabwe Zambia Burundi Rwanda Uganda Tanzania Kenya Sudan Djibouti Ethiopia Somalia Seychelles Comoros Madagascar Mauritius & Reunion Greenland Canada U.S.A. Mexico Guatemala Belize Honduras EI Salvador Nicaragua Costa Rica Panama Cuba jamaica Haiti Dominican Republic Puerto Rico Bahamas Lesser Antilles Trinidad Colombia Venezuela Guyana Surinam French Guiana Brazil Paraguay Argentina Chile Bolivia Peru Ecuador
Page 5 and 6: FREQUENCIES OF HEMOGLOBIN VARIANTS
Page 7: -', CONTENTS 1. Introduction, 6 2.
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Page 16: Hb Th Gd Ov HTGd EXPLANATION OF SYM
Page 20 and 21: REF. NO. POPULATION POLYNESIA 1028
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REF. NO. POPULATION THAILAND PLACE
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REF. NO. POPULATION CAMBODIA 374 Ca
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REF. NUMBER GENE NIL POPULATION PLA
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REF. NO. POPULATION CHINA 2147 Chin
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REF. NUMBER GENE NO. POPULATION PLA
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REF. NO. POPULATION INDIA 1373 Oris
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REF. NUMBER GENE NO. POPULATION NEP
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REF. NO . POPULATI ON IRAN 1495 Tur
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REF. NO. POPULATION JORDAN 1844 Gaz
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REF. NO. POPULA TI ON LEBANON 1984
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REF-. NUMBER GENE NO. POPULATION PL
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REF. NO. POPULATION MALTA 452 Malte
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REF. NO. POPULATION GREECE 1941 Gre
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REF. NO. POPULATION YUGOSLAVIA 1734
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REF. NO. POPULATION U.S.S.R. 774 Ta
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REF. NUMBIiR GENE NO. POPULATION PL
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REF. NO. POPULATION ITALY 1418 Ital
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REF. NO. POPULATION ITALY PLACE 212
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REF. NUMBER GENE NO_._POPULATION PL
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REF. NO. POPULATION PLACE ITALY 221
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REF. NO. POPULATION PORTUGAL 2043 P
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FRANCE 1527 Melano-Africans 1527 Ot
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REF. NO. POPULATION BELGIUM 1145 No
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REF. NO. POPULATION GREAT BRITAIN 1
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REF. NO. POPULATION EGYPT 1640 Egyp
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REF. NO. POPULATION TUNISIA 603 Tun
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REF. NO. POPULATION ALGERIA 402 Ara
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REF. NUMBER GENE llih- POPULATION P
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REF. NO. POPULATION SENEGAL 1164 Se
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REF. NUMBER GENE NO. POPULATION SEN
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REF. NO. POPULATION PLACE LIBERIA 2
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REF. NO. POPULATION IVORY COAST 409
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REF. NO. POPULATION IVORY COAST 403
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REF. NO. POPULATION PLACE NUMBER TE
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REF. NO. POPULATION PLACE UPPER VOL
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REF. NUMBER GENE NO. POPULATION GHA
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REF. NO. POPULATION CAMEROON 213 Mu
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REF. NUMBER GENE NO_._POPULATION PL
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REF. NO. POPULATION MOZAMBIQUE 1481
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REF. NO. POPULATION ZAMBIA 688 Mamb
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REF . NUMBER GENE NO. POPULATION PL
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REF. NO. POPULATION SUOAN 1515 Oink
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REF. NUMBER \;it.Nt. NO. POPULATION
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REF. NO. POPULATION CANADA 2094 Ame
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REF. NO. POPULATION UNITED STATES 7
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REF . NUMBER GENE NO. POPULATION PL
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UNITED STATES 1732 Blacks(pregnant)
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REF. NO. POPULATION UNITED STATES P
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REF. MO. POPULATION MEXICO 1972 Chi
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REF. NO. POPULATION BELIZE 552 Blac
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REF. NO. POPULATION COSTA RICA 1310
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REF. NO. POPULATION HAITI 525 Haiti
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REF. NO. POPULATION VENEZUELA 109 G
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REF. NO. POPULATION BRAZIL PLACE 44
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Cf) UJ U Z UJ c.::= UJ U UJ c.::=
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466 110 Arends T (1984) Personal co
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469 provincia di Milano. La Rlforma
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288 289 290 291 292 293 294 295 296
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389 Buettner-Janusch J (1965) Unpub
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infection. Ann Trop Med Parasitol 6
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591 Demeocq F, Clamen G, Menut G. M
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482 662 Efremov GO. Huisman THv (19
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484 73:161-172 728 Fleming PJ, Arno
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486 799 Gentilini M. Richard-Lenobl
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905 Helms MW (1964) Glucose-6-phosp
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491 structural variants of hemoglob
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493 49:970-982 1048 Kalmus H (1957)
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495 1115 Konigsberg W, Huntsman RG,
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haemoglobins in the Ibo. Nature 183
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500 1290 Marti HR,- Butler R (1961)
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504 1428 Munoz uA Risueno CE, Gil u
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508 Province of Huelva. Am J Hum Ge
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510 1636 Raccurt CP, Seytor S. Sain
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513 groups of Indians. Hum Hered 21
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515 1808 Schneider RG (1956) Incide
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517 1879 Sllvestroni E, Bianco I (1
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519 1946 Stein J, Berg C. Jones JA.
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522 2048 Tuchinda S, Beale 0, Lehma
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