Early diagnosis and multidisciplinary care reduce the hospitalization time and duration of tube feeding and prevent early obesity in PWS infants

被引:45
作者
Bachere, N.
Diene, G.
Delagnes, V.
Molinas, C.
Moulin, P.
Tauber, M.
机构
[1] Hop Enfants, Div Endocrinol Genet Gynaecol & Bone Dis, Toulouse, France
[2] Hop Purpan, INSERM, IFR30, U563, Toulouse, France
关键词
neonatology; hypotonia; growth hormone;
D O I
10.1159/000111795
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background/Aims: To describe and evaluate the impact of very early diagnosis and multidisciplinary care on the evolution and care of infants presenting with Prader-Willi syndrome (PWS). Methods: 19 infants diagnosed with PWS before the second month of life were followed by a multidisciplinary team. Median age at the time of analysis was 3.1 years [range 0.4-6.5]. The data were compared with data collected in 1997 from 113 questionnaires filled out by members of the French PWS Association. The patients from this latter data set were 12.0 years [range 4 months to 41 years] at the time of analysis, with a median age of 36 months at diagnosis. Results: The duration of their hospitalization time was significantly reduced from 30.0 [range 0-670] to 21 [range 0-90] days (p = 0.043). The duration of gastric tube feeding was significantly reduced from 30.5 [range 0-427] to 15 [range 0-60] days ( p = 0.017). Growth hormone treatment was started at a mean age of 1.9 +/- 0.5 years in 10 infants and L-thyroxine in 6 infants. Only 10 infant became obese at 2.5 years. Conclusion: Early diagnosis combined with multidisciplinary care decreases the hospitalization time, duration of gastric tube feeding and prevents early obesity in PWS infants. Copyright (C) 2007 S. Karger AG, Basel.
引用
收藏
页码:45 / 52
页数:8
相关论文
共 32 条
  • [1] Angulo M, 1996, J PEDIATR ENDOCR MET, V9, P393
  • [2] PHYSICAL FEATURES OF PRADER-WILLI SYNDROME IN NEONATES
    AUGHTON, DJ
    CASSIDY, SB
    [J]. AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1990, 144 (11): : 1251 - 1254
  • [3] Peculiar body composition in patients with Prader-Labhart-Willi syndrome
    Brambilla, P
    Bosio, L
    Manzoni, P
    Pietrobelli, A
    Beccaria, L
    Chiumello, G
    [J]. AMERICAN JOURNAL OF CLINICAL NUTRITION, 1997, 65 (05) : 1369 - 1374
  • [4] Endocrine dysfunction in Prader-Willi syndrome:: A review with special reference to GH
    Burman, P
    Ritzén, EM
    Lindgren, AC
    [J]. ENDOCRINE REVIEWS, 2001, 22 (06) : 787 - 799
  • [5] GROWTH-HORMONE (GH) RESPONSE TO COMBINED PYRIDOSTIGMINE AND GH-RELEASING HORMONE ADMINISTRATION IN PATIENTS WITH PRADER-LABHARD-WILLI SYNDROME
    CAPPA, M
    GROSSI, A
    BORRELLI, P
    GHIGO, E
    BELLONE, J
    BENEDETTI, S
    CARTA, D
    LOCHE, S
    [J]. HORMONE RESEARCH, 1993, 39 (1-2) : 51 - 55
  • [6] Growth hormone improves mobility and body composition in infants and toddlers with Prader-Willi syndrome
    Carrel, AL
    Moerchen, V
    Myers, SE
    Bekx, T
    Whitman, BY
    Allen, DB
    [J]. JOURNAL OF PEDIATRICS, 2004, 145 (06) : 744 - 749
  • [7] Benefits of long-term GH therapy in Prader-Willi syndrome: A 4-year study
    Carrel, AL
    Myers, SE
    Whitman, BY
    Allen, DB
    [J]. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2002, 87 (04) : 1581 - 1585
  • [8] GROWTH-HORMONE SECRETION IN PRADER-WILLI SYNDROME
    COSTEFF, H
    HOLM, VA
    RUVALCABA, R
    SHAVER, J
    [J]. ACTA PAEDIATRICA SCANDINAVICA, 1990, 79 (11): : 1059 - 1062
  • [9] Deaths in children with Prader-Willi syndrome - A contribution to the debate about the safety of growth hormone treatment in children with PWS
    Eiholzer, U
    [J]. HORMONE RESEARCH, 2005, 63 (01) : 33 - 39
  • [10] Eiholzer U, 2004, J PEDIATR ENDOCR MET, V17, P1153