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THE INDIAN JOURNAL OF PEDIATRICS

G r o w t h H o r m o n e Therapy for Turner S y n d r o m e - Is It Indicated? S

hort stature is the single most common physical finding in Turner syndrome (TS). The mean height varies from 132 cm to 147 cm in different populations. The important point is that there is a difference of 20 cm between the mean normal adult female height, and adult height in TS in a given population. I The pathogenesis of this growth failure is not clear. Hormonal abnormalities as well as cartilage insensitivity to growth factors have been implicated, but not proven. Growth hormone (GH), which is the most important hormone influencing both growth rate and final height is not deficient in TS except in certain individual cases. 2 Despite this, all growth promoting therapies for TS use growth hormone either alone, or in combination with low dose estrogens or anabolic steroids like oxandrolone. Major trials using GH in TS have shown a growth velocity increment of 1.9/4.5 c m / y e a r during the first year. 3 In the second year of treatment the increment is less. 4 The eventual effect on adult height has not been established, but in the US trial, after 5 years treatment with G H the patients were still growing at higher than TS specific height velocities, s Many patients in this study have already exceeded the height projected at start of therapy, some after only 3 years of treatment. Addition of low dose estrogen to GH therapy does increase short term growth velocity, but also enhances bone maturation thus compromising final height achieved. The largest increments were

obtained with the addition of oxandrolone to GH in a daily dose of 0.05 to 0.10 m g / k g . Bone maturation is enhanced as well, but not unduly. 6 However, additive effects of GH and oxandrolone on carbohydrate metabolism render strict monitoring of blood glucose mandatory. Side effects are rare. The dose of GH in TS is higher than replacement therapy for treatment of GH deficiency. The usual dose is 0.1 to 0.14 u n i t s / k g / d a y given as once daily subcutaneous injection. The dose is usually increased in the second year of therapy as deceleration of growth occurs. 3 At current prices, for a 20 kg child getting 0.1 u n i t / k g / d a y of GH, the cost of a years therapy would be Rs. 3.24 lacs. At the higher dose, the second year of treatment would cost Rs. 4.2 lacs for the same child. The m a x i m u m increment in growth velocity expected in the first year of therapy is 4.5 cm with GH alone and 7.9 cm with GH and oxandrolone. However, these never forms of treatment are still in experimental stage, and need to be carried out under superision. 7 The cost makes it prohibitive for routine use in Turner syndrome in India. Gurmeet Singh, M o h n i s h Suri and Madhulika

Department of Pediatrics, All hTdia Institute of Medical Sciences, New Delhi-110029 References 1. Ranke MB, Stubbe P, Majewski F et al.

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Spontaneous growth in Turner syndrome. Acta Pediatr Scand (Suppl) 1988; 343 : 22-30. Brook CGD. G r o w t h h o r m o n e deficiency in Turner syndrome. N Engl J Med 1978; 298 : 1203-1204. Ranke MB. G r o w t h disorder and its treatment in Turner syndrome. International Growth Monitor 1991; 1 : 2-9. Lin TH, Kirkland JC, Kirkland RT. Growth hormone assessment and short term treatment with growth hormone in Turner syndrome. J Pediatr 1988; 112 : 919-922. Rosenfield RG. The Genetech Collaborative Study of growth hormone in Turner

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syndrome : long term effects of growth hormone and oxandrolone on height in Turner syndrome : 5 year results. In : Ranke MB, Rosenfield RG eds, Turner

Syndrome : Growth Promoting Therapies. Amsterdam : Elsevier, 1990 : pp 221-224. 6. Rosenfield RG, Hintz RL, Johanson AJ et al. Three year results of randomized prospective trial of methionyl h u m a n growth hormone and oxandrolone in Turner syndrome. J Pediatr 1988; 113 : 393-400. 7. Ranke MB. Current concepts in the treatment of Turner syndrome with special reference to the treatment of short stature. Acta Pediatr Jpn 1992; 34 : 183-194.