O Mäkitie1, I Kaitila. 1. Children's Hospital, Helsinki University Hospital, Finland.
Abstract
OBJECTIVE: To study the growth and its correlates in diastrophic dysplasia (DTD) and to construct standard growth curves for DTD. METHODS: Growth data of 121 Finnish patients with DTD were collected. Percentile growth curves for height and relative weight were constructed by using interpolated values from individual curves. Pubertal growth, intrafamilial variability, and correlates of adult height were assessed. RESULTS: The median adult height was 135.7 cm for the male and 129.0 cm for the female subjects. The growth failure was progressive partly because of absent or weak pubertal growth spurt. The severity of the growth failure varied greatly, even among siblings. The final height did not correlate with the midparental height but correlated well with the height at 1.0 and 5.0 years. The relative weight was normal in childhood but increased before puberty; 28% of the adults were overweight. Head circumference was normal. CONCLUSIONS: DTD interferes profoundly with normal growth and results in progressive growth failure of varying severity. The growth charts should prove useful in following up an individual patient with DTD; they may be used to predict adult height and to evaluate growth-promoting therapies.
OBJECTIVE: To study the growth and its correlates in diastrophic dysplasia (DTD) and to construct standard growth curves for DTD. METHODS: Growth data of 121 Finnish patients with DTD were collected. Percentile growth curves for height and relative weight were constructed by using interpolated values from individual curves. Pubertal growth, intrafamilial variability, and correlates of adult height were assessed. RESULTS: The median adult height was 135.7 cm for the male and 129.0 cm for the female subjects. The growth failure was progressive partly because of absent or weak pubertal growth spurt. The severity of the growth failure varied greatly, even among siblings. The final height did not correlate with the midparental height but correlated well with the height at 1.0 and 5.0 years. The relative weight was normal in childhood but increased before puberty; 28% of the adults were overweight. Head circumference was normal. CONCLUSIONS: DTD interferes profoundly with normal growth and results in progressive growth failure of varying severity. The growth charts should prove useful in following up an individual patient with DTD; they may be used to predict adult height and to evaluate growth-promoting therapies.
Authors: Edward L Mertz; Marcella Facchini; Anna T Pham; Benedetta Gualeni; Fabio De Leonardis; Antonio Rossi; Antonella Forlino Journal: J Biol Chem Date: 2012-05-03 Impact factor: 5.157