920

Clinical and laboratory observations

The Journal of Pediatrics December 1992

Congenital idiopathic growth hormone deficiency associated with prenatal and early postnatal growth failure Peter D. G l u c k m a n , MBChB, MMedSc, DSc, FRACP, Alistair J. Gunn, MBChB, FRACP, Alison Wray, MBChB, W a y n e S. Cutfield, MBChB, DCH, FRACP, Pierre G. C h a t e l a i n , MD, Olivier G u i l b a u d , Phb, G e o f f r e y R. A m b l e r , MBBS,FRACP, Patrick Wilton, MD, PhD, a n d Kerstin Albertsson-Wikland, MD, PhD, on b e h a l f of t h e International Board of t h e Kabi P h a r m a c i a I n t e r n a t i o n a l G r o w t h Study* From the Departments of Paediatrics, Universityof Auckland, Auckland, New Zealand, the H6pital Edouard Herriot, Lyon, France, Kabi Pharmacia Peptide Hormones, Stockholm, Sweden, and the Department of Paediatrics II, GOteborg University and East Hospital, GOteborg, Sweden To assess the role of growth hormone in fetal and infant growth, we analyzed the pretreatment data on 52 patients with a diagnosis of congenital growth hormone deficiency before 2 years of age, obtained from the Kabi Pharmacia International Growth Study. These infants had reduced birth-length standard deviation scores, an excess of birth weight relative to length, and progressive growth failure. We conclude that congenital growth hormone deficiency may cause impaired growth in utero and early infancy, and that growth hormone plays an important role in perinatal and infantile growth. (J PEDIATR1992;121: 920-3)

It is often stated that congenital growth hormone deficiency is not associated with growth failure at birth and that growth of such infants is generally normal for some months after birth, l, 2 The evidence is largely anecdotal and obtained from small series, 3 but the conclusion is supported by experimental finding of relatively few GH receptors in fetal tissues.4 Furthermore, studies of animals have suggested that GH is not a major determinant of fetal growth, 5 although contradictory evidence is seen in children with GH receptor dysfunction (Laron dwarfism), who are short at birth and have severe growth failure within the first few months of life, 6 as do many members of sibships with deletions of the GH gene. 7, 8 In view of the contradictory evidence regarding the role Supported by Kabi Pharmacia Peptide Hormones. Submitted for publication April 22, 1992; accepted July 7, 1992. Reprint requests: Peter D. Gluckman, IVlBChB, MMedSc, DSc, Department of Paediatrics, University of Auckland, Private Bag, Auckland, New Zealand. *For a list of executive committee members and coordinators, see the acknowledgmentsat the end of the text. 9/22/40813

of GH in fetal and infant growth, our aim was to determine whether growth failure had occurred prenatally and postnatally in children with presumed CGHD. A preliminary report of the birth data on the Swedish patients has been published.9 METHODS The Kabi Phffrmacia International Growth Study had data on more than 6000 patients who had received GH therapy by the time of the review in September 1990. The

CGHD GH LthsDs ~SDS WtSDS

Congenital growth hormone deficiency G r o w t hhormone Length SDS Standard deviation score Weight SDS

study population was selected according to the following criteria: (1) the diagnosis was idiopathic GH deficiency or septo-optic dysplasia, with peak serum GH level

Congenital idiopathic growth hormone deficiency associated with prenatal and early postnatal growth failure. The International Board of the Kabi Pharmacia International Growth Study.

To assess the role of growth hormone in fetal and infant growth, we analyzed the pretreatment data on 52 patients with a diagnosis of congenital growt...
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