Causal study of isolated ulnar-fibular deficiency in Hungary, 1975-1984.
A population-based and validated data set of 114 cases with isolated ulnar-fibular deficiency was evaluated in Hungary, 1975-1984. Ulnar-fibular type had the third most common birth prevalence (0.07 per 1000) among isolated congenital limb deficiency types. This type is relatively rarely associated with nonlimb defects, a single limb is affected in two-thirds of cases, nearly all other cases had femur-fibula-ulna (FFU) dysostosis. Upper and lower limbs, right and left sides, are equally affected, however, there is an obvious male excess (71%). Case-control analysis indicated a lower birth weight due to intrauterine growth retardation, higher birth order, lower socioeconomic status of parents, and a more frequent reported subfertility. The family study identified one familial cluster (one siboccurrence) among 331 first-degree relatives.
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Department of Human Genetics and Teratology, National Institute of Hygiene, WHO Collaborating Centre for the Community Control of Hereditary Diseases, Budapest, Hungary.
Vitéz
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Kodaj
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Lenz
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D000014:Abnormalities, Drug-Induced / Q000453:epidemiology; D000015:Abnormalities, Multiple / Q000453:epidemiology / Q000209:etiology*; D016022:Case-Control Studies; D005260:Female; D005360:Fibula / Q000002:abnormalities*; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications / Q000453:epidemiology; D012042:Registries; D015203:Reproducibility of Results; D012189:Retrospective Studies; D014457:Ulna / Q000002:abnormalities*