Ae. Czeizel et al., CAUSAL STUDY OF ISOLATED ULNAR-FIBULAR DEFICIENCY IN HUNGARY, 1975-1984, American journal of medical genetics, 46(4), 1993, pp. 427-433
A population-based and validated data set of 114 cases with isolated u
lnar-fibular deficiency was evaluated in Hungary, 1975-1984. Ulnar-fib
ular type had the third most common birth prevalence (0.07 per 1000) a
mong isolated congenital limb deficiency types. This type is relativel
y rarely associated with nonlimb defects, a single limb is affected in
two-thirds of cases, nearly all other cases had femur-fibula-ulna (FF
U) dysostosis. Upper and lower limbs, right and left sides, are equall
y affected, however, there is an obvious male excess (71%). Case-contr
ol 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 identifi
ed one familial cluster (one siboccurrence) among 331 first-degree rel
atives.