Growth deficiency and malnutrition in Bloom syndrome

Citation
C. Keller et al., Growth deficiency and malnutrition in Bloom syndrome, J PEDIAT, 134(4), 1999, pp. 472-479
Citations number
14
Categorie Soggetti
Pediatrics,"Medical Research General Topics
Journal title
JOURNAL OF PEDIATRICS
ISSN journal
00223476 → ACNP
Volume
134
Issue
4
Year of publication
1999
Pages
472 - 479
Database
ISI
SICI code
0022-3476(199904)134:4<472:GDAMIB>2.0.ZU;2-#
Abstract
Objectives: To describe the growth and nutritional status of a pediatric po pulation with Bloom syndrome. Study design: Longitudinal growth data from 148 patients in the Bloom's syn drome Registry (85 male, 63 female) were compiled retrospectively from phys ician and parent records to develop graphed statistics of weight-for-age, h eight-for-age, fronto-occipital circumference-for-age, and weight-for-heigh t for both sexes with comparisons with the normal population. Results: Term birth measurements confirm that the growth deficiency of Bloo m syndrome has prenatal onset. Stunting persists throughout life, and an ad olescent growth spurt is not apparent from the smoothed data. Growth contin ues by at least 1 cm/yr until age 21 years for both sexes. More than half o f children with Bloom syndrome are significantly wasted until age 8 years, which is not related to early death or underlying malignancy. The mean body mass index for adults with Bloom syndrome after age 25 years is low normal (n = 22, mean = 20.2 kg/m(2)). Conclusions: Children with Bloom syndrome have significant growth retardati on and wasting.