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Plasma somatomedin activity in an infant with Beckwith Wiedemann syndrome
Insights
Elevated somatomedin activity and growth hormone levels in an infant with Beckwith-Wiedemann syndrome may contribute to excessive fetal growth. These levels normalized by 8 months, with growth parameters also returning to normal ranges.
Area of Science:
- Pediatric Endocrinology
- Genetics and Developmental Biology
Background:
- Beckwith-Wiedemann syndrome is associated with overgrowth.
- The role of specific growth factors in fetal overgrowth is not fully understood.
Observation:
- A neonate diagnosed with Beckwith-Wiedemann syndrome exhibited significantly elevated birth weight and length, exceeding the 97th percentile.
- Plasma samples at birth revealed high levels of both growth hormone and somatomedin activity compared to control groups.
Findings:
- At 8 months of age, the child's growth hormone and somatomedin activity normalized, with weight and length falling to the 75th percentile.
- The initial elevated somatomedin activity correlated with the excessive fetal growth observed.
Implications:
- High somatomedin activity is a potential contributor to the characteristic overgrowth in Beckwith-Wiedemann syndrome.
- Monitoring hormonal levels in infancy may provide insights into growth patterns and potential interventions.
Abstract:
Plasma growth hormone levels and somatomedin activity were determined in a child with Beckwith--Wiedemann syndrome at birth and at 8 mth of age. Birthweight and length were above the 97th centile. Somatomedin activity in the cord plasma was elevated (2.8 U/ml) compared with controls (0.15--1.3 U/ml; n = 15). Growth hormone was also high (76 ng/ml compared with control group range of 5.5--42.1 ng/ml, n = 26). At 8 mth of age both somatomedin activity and plasma growth hormone had fallen to normal levels and weight and length were on the 75th centile. It is suggested that the high somatomedin activity may have been a contributing factor in the excessive fetal growth of this child.
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