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      KIGS Highlights: Growth Hormone Treatment in Prader-Willi Syndrome

      a , b

      Hormone Research in Paediatrics

      S. Karger AG

      Prader-Willi syndrome, KIGS, Growth hormone

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          Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome.

           T Nagai,  R Sakuta,  K Obata (2006)
          Growth hormone (GH) therapy for short stature in patients with Prader-Willi syndrome (PWS) has started worldwide, and various favorable effects have been reported. However, the possibility of progression of scoliosis arises as a new problem of the GH therapy. In this study, we analyzed whether 72 patients who have been followed up in our hospital have such a problem. They included 46 males and 26 females (41 patients with the GH therapy and 31 without it) aged from one to 49 years. Consequently, 33 (45.8%) of 72 patients had scoliosis with the Cobb angle of >10 degrees. Twenty (48.8%) of forty-one patients who received a GH therapy and 13 (41.9%) of 31 patients without the therapy had scoliosis, the frequency of scoliosis between the two groups showing no statistical difference (P = 0.56). Height velocity of scoliotic and non-scoliotic patients during the first year of the therapy was 8.59 +/- 1.92 and 10.70 +/- 2.54 cm, respectively, showing a significant difference (P < 0.001). This shows that accelerated height velocity may not induce scoliosis. Comparison of starting age of a GH treatment revealed that non-scoliotic patients received the therapy earlier than scoliotic patients (P = 0.021). Among 20 scoliotic patients who received the GH therapy, the degree of scoliosis progressed during the therapy in six patients, improved in three and fluctuated in one. Many patients showed progression of scoliosis with age irrespective of the use of GH, and some patients improved their scoliosis during the GH therapy. These findings showed that a GH therapy increases height velocity of PWS patients but does not necessarily develop scoliosis, and early start of the therapy may not be an exacerbating factor of scoliosis. Copyright 2006 Wiley-Liss, Inc.
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            Psychomotor development in infants with Prader-Willi syndrome and associations with sleep-related breathing disorders.

             E Festen,  B Otten,  M Wevers (2007)
            Prader-Willi syndrome (PWS) is a neurogenetic disorder with hypotonia, psychomotor delay, obesity, short stature, and sleep-related breathing disorders. The aim of this study was to evaluate the association between psychomotor development and sleep-related breathing disorders in PWS infants. Bayley Scales of Infant Development were performed in 22 PWS infants, with a median (interquartile range, IQR) age of 1.8 (1.1-3.4) y, and a body mass index SD score (BMISDS) of -0.5 (-1.3 to 1.6). We evaluated psychomotor development in relation to results of polysomnography. Median (IQR) mental and motor development was 73.1% (64.3-79.6%) and 55.2% (46.5-63.1%) of normal children, respectively. All infants had sleep-related breathing disorders, mostly of central origin. The apnea hypopnea index was not associated with psychomotor development. Only four infants had obstructive sleep apnea syndrome (OSAS). They had a significantly delayed mental development of 65.5% (60.0-70.3%) of normal. They had a median BMISDS of 1.4 (0.1-1.6), which tended to be higher than in those without OSAS. Our data indicate that psychomotor development in PWS infants is not related to central sleep-related breathing disorders, but infants with OSAS have more severely delayed mental development, suggesting that PWS infants should be screened for OSAS.

              Author and article information

              Horm Res Paediatr
              Hormone Research in Paediatrics
              S. Karger AG
              December 2007
              10 December 2007
              : 68
              : Suppl 5
              : 48-50
              aHôpital des Enfants, Pediatrie-Endocrinologie, Génétique et Gynécologie Medicale, Toulouse, France; bLiggins Institute, Department of Pediatrics, University of Auckland, Auckland, New Zealand
              110475 Horm Res 2007;68:48–50
              © 2006 S. Karger AG, Basel

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              Figures: 1, Tables: 1, References: 3, Pages: 3
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