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Children with Silver-Russell syndrome gain height with growth hormone therapy
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Growth hormone therapy appears to yield height increases in children with Silver-Russell syndrome comparable to those gained by treated children without the disorder, according to recent findings.
In a prospective longitudinal study, Carolina C.J. Smeets, of the endocrinology division of the pediatrics department at Erasmus University Medical Center in Rotterdam, Netherlands, and colleagues evaluated 62 children with Silver-Russell syndrome (SRS) and 227 children without SRS born small for gestational age. Of the SRS group, 31 children had 11p15 aberrations and 11 had mUPD7 aberrations; 20 had idiopathic SRS. All patients were assigned GH 1 mg/m2 daily. Researchers recorded height, weight and Tanner stage three times a month.
Carolina C.J. Smeets
Baseline height and weight were lower in the SRS group vs. the non-SRS group. Mean height at start of GH treatment was standard deviation score (SDS) -3.67 for the SRS group and -2.92 SDS for the non-SRS group (P < .001). Weight was 1.36 SDS lower in the SRS group. Both groups had low baseline serum levels of IGF-1 and IGFBP-3.
Over the first year of GH therapy, the SRS group gained a mean 0.96 SDS in height vs. 0.84 SDS in the non-SRS group (P = .08). The SRS group had an increase in serum IGF-1 to 1.15 SDS vs. 0.63 in the non-SRS group (P < .001).
From onset of puberty until attainment of adult height, researchers noted a decrease in height SDS in both groups for a mean adult height SDS of -2.17 in the SRS group and -1.65 in the non-SRS group (P = .002) and a larger distance to target height in SRS participants (P < .001). However, the total height gain in SDS from initiation of treatment until attainment of adult height was comparable between the groups. A trend toward greater height gain was seen in patients with mUPD7 compared with 11p15 aberrations.
“We showed that SRS patients respond well to the GH treatment and that total height gain is similar in SRS and non-SRS subjects born [small for gestational age],” the researchers wrote. – by Jennifer Byrne
Disclosure: One researcher reports no reports receiving an independent research grant from Novo Nordisk B.V.
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