Bone maturation in girls with Turner's syndrome

Abstract

The mechanism of growth retardation in Turner's syndrome has not been resolved. It is often referred to as a bone dysplasia, although endocrine derangement has not been ruled out. The present study was undertaken to evaluate the maturation of individual bones of the hand and wrist in girls with Turner's syndrome and thereby obtain information which may aid in elaborating the possible mechanism of the growth retardation in girls with Turner's syndrome. Hand and wrist films of 24 girls with Turner's syndrome, 11 normal girls with short stature and 23 normal controls were evaluated, using the references of Greulich and Pyle. Each bone or epiphysis was given an individual 'age'. During childhood the Turner patients showed the greatest delay in bone age of the phalangeal bones while the least delayed were the radius and ulna (long bones) and metacarpals. The carpal bones showed intermediate retardation. This pattern and extent of maturational retardation was clearly different from that of the short stature normal group, who showed uniform retardation of all bones. During adolescence, the phalangeal bones were further retarded and the carpal bones showed a moderate retardation. The unique profile of bone maturation in Turner's syndrome suggests an insult to chondroplasia, which may be related to estrogen deficiency or to an as yet undetermined endocrine or paracrine derangement.

Kyphosis in a Turner Syndrome Population

“…15 Developmental abnormalities of individual bones that may affect the spine include hypoplasia of cervical vertebrae, coalition of vertebrae and hemivertebrae, although these were not found in our study population. One might also hypothesize that variability in rate of bone maturation, a phenomenon well documented for bones in the hand, 16 may also occur in the spine and lead to abnormal mechanical stresses. Finally, osteoporosis is more common in individuals with TS.…”

The Use of Bone Age in Clinical Practice – Part 1

“…Examples include disorders affecting cartilage and/or bone formation that often result in typical images such as broader and stunted bones (hypochondroplasia), irregular metaphysis and increased triangularization index (associated with the SHOX haploinsufficiency of the Turner syndrome and Léri-Weill syndrome) [23,24], or shortness of the 4th and 5th metacarpals (pseudohypoparathyroidism). Many endocrine diseases are also associated with typical hand-wrist radiographic findings, for examplerickets (abnormal growth plate mineralization), hypothyroidism (delayed carpal development), and hyperparathyroidism (cortical resorption).…”

Adulthood in Women with Turner Syndrome

“…This may be related to oestrogen deficiency or other endocrine/ paracrine derangement [39]. Measurement of BMD is affected by stature, and it therefore remains to be clarified whether the low BMD found in women with TS reflects true osteoporosis or is, in fact, an artefact of short stature.…”