摘要:

SummaryA family showing the Pelger‐Huët anomaly of the neutrophil polymorphonuclear leucocytes is described, the first to be reported in England. Thirty‐four affected individuals have been examined; thirty‐one of these and thirty‐eight unaffected people, either parents or sibs of affected members, have been grouped for theABO, MNS, Eh, P, Lewis, Kell, Lutheran and Duffy blood‐group systems. No linkage was demonstrated.We are indebted to Dr A. E. Mourant for most of the anti‐sera used in this investigation and to Drs D. Lehane and P. L. Mollison for supplies ofanti‐Fya. We are grateful to the members of the family described here for their co‐operation. We would like to thank Mr W. Rivers, Senior Technician, King's College Hospital, for preparing the pedigree chart and Mr W. Smith, Medical Photographer, King's College Hospital, for the

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01259.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01260.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

摘要:

Summary1. In Africa, as in the United States, the great majority of cases of sickle‐cell anaemia are homozygous for the sickle‐cell gene.2. Carriers of the sickle‐cell trait, i.e. individuals heterozygous for the sickle‐cell gene, occur in frequencies up to 40 % in Africa, 30 % in India and 17 % in Greece. It is difficult to explain the distribution of the sickle‐cell trait in terms of ancestral origins alone.3. The incidence of sickle‐cell anaemia is not accurately known, but there is indirect evidence that in Africa, as in the United States, the majority of homozygotes for the sickle‐cell gene are affected. A minority of these homozygotes reaches adult life and reproduces. It is estimated that the fitness of the sickle‐cell homozygote is not more than a quarter that of the heterozygote.4. The elimination of sickle‐cell genes in cases dying of sickle‐cell anaemia is so rapid that it is very unlikely + hat these genes could be replaced by reourrent mutation. It appears that those who are heterozygous for the sickle‐cell gene are naturally resistant to subtertian malaria, so that they have a selective advantage over the normal homozygotes in regions where malaria is hyperendemic. The sickle‐cell polymorphism would then be the result of a balance determined mainly by the severity of subtertian malaria, which would tend to increase the frequency of the gene, and the elimination of genes in cases of sickle‐cell anaemia. The sickle‐cell trait attains high frequencies, in fact, only in regions where malaria is severe.5. It is estimated that the fitness of the sickle‐cell heterozygote in malarious environments is 1–26 X the fitness of the normal homozygote.6. The expected rate of change of the sickle‐cell carrier frequency in populations under different conditions has been calculated. In two instances such an estimate corresponds approximately with the observed data. It seems that the incidence of the sickle‐cell trait may be greater in adults than in young children in populations exposed to malaria, as expected. And the United States Negroes have a lower frequency of the sickle‐cell trait than would be expected on the basis of a one‐third admixture with Caucasian and Indian blood. The fall in frequency would correspond to an elimination of three‐quarters of the homozygotes without heterosis for

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01261.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01262.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01263.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

摘要:

Book reviewed in this article:The Conception of Disease: its History, its Versions and its Nature. By WaltherRiese, M.D.‘A Genetic and Neuropsychiatric Investigation of a North‐Swedish Population with special regard to Schizophrenia and Mental Deficiency’. By J. A. Böök. Acta Oenetica et Statistica Medica, vol. 4, pp. 1–100, 133‐9 and 345–414. Basel: S. Karger.*The Facts of Life. By C. D. DarlingtonLife and other Contingencies. Vol. I. By P. F. Hooker, F.I.A., A.S.A. and L. H. Longley‐Cook, M.A., F.I.A., F.C.A.S., A.S.A. Published for the Institute of Actuaries and the Facu

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01264.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY

ISSN:0003-4800    

DOI:10.1111/j.1469-1809.1954.tb01265.x

出版商:Blackwell Publishing Ltd    

年代:1954

数据来源: WILEY