Pulmonary function abnormalities in Prader-Willi syndrome

Hákonarson, Hákon; Moskovitz, Jay; Daigle, Karen; Cassidy, Suzanne B.; Cloutier, Michelle M.

doi:10.1016/s0022-3476(95)70350-0

Cited by 57 publications

(35 citation statements)

References 18 publications

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“…However, the measured PEF was far from the normal range. Restrictive ventilatory impairment has been demonstrated in PWS children and adults, mainly due to respiratory muscle weakness [25]. Studies with GH intervention in PWS children has demonstrated improved pulmonary function [13,26,27].…”

Section: Discussionmentioning

confidence: 99%

Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome

Sode-Carlsen

Farholt

Rabben³

et al. 2011

Growth Hormone & IGF Research

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Section: Discussionmentioning

confidence: 99%

Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome

Sode-Carlsen

Farholt

Rabben³

et al. 2011

Growth Hormone & IGF Research

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“…The decreased lean body mass, already present in infants with PWS [18], might be another important factor involved. The resulting decrease in respiratory muscle mass [19], together with a defect in the function and architecture of the throat due to hypotonia, may be the main reason for disturbed respiration leading to an increase of the respiratory drive set-point in the brain stem and to central hypoventilation. Indeed, as shown in two recent studies, GH therapy in children with PWS improves not only lean body mass [20], but also respiratory function [21], leading to an increase in CO 2 sensitivity [16, 22].…”

Section: Discussionmentioning

confidence: 99%

Fatal Outcome of Sleep Apnoea in PWS during the Initial Phase of Growth Hormone Treatment

Eiholzer¹,

Nordmann²,

l’Allemand³

2002

Horm Res Paediatr

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The case of a boy with Prader-Willi syndrome (PWS) who suffered from respiratory problems since birth and suddenly died at the age of 6.5 years, 4 months after initiation of GH therapy, is presented. This case indicates the possibility of fatal courses in infants and children with PWS as a consequence of respiratory problems and raises the question as to a causal connection between the initiation of GH therapy and the sudden death of this child.

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“…The decreased lean body mass in infants with PWS [20] might be another important factor. The decrease in respiratory muscle mass [21], together with a defect in the function and architecture of the throat due to hypotonia, may be the main reason for a disturbed respiration, leading to an increase in respiratory drive set point in the brain stem and central hypoventilation. Indeed, as shown in two recent studies, GH treatment in children with PWS improves not only lean body mass but also respiratory function, leading to an increase in CO 2 sensitivity [22,23].…”

Section: Discussionmentioning

confidence: 99%

Sudden Death of an Infant with Prader-Willi Syndrome – Not a Unique Case?

Nordmann¹,

Eiholzer²,

l’Allemand³

et al. 2002

Neonatology

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Only in the recent past has Prader-Willi syndrome (PWS) also been diagnosed in newborns and infants, and the first descriptions of respiratory disturbances in young, not yet obese patients have been published only recently. We report an infant with PWS and respiratory problems, who suffered sudden death. To our knowledge, this is the first such case. In order to avoid fatalities, it seems mandatory to monitor respiratory function in infants with PWS and to suspect PWS in all hypotonic newborns to determine the frequency of the syndrome more accurately, and, more importantly, to diagnose PWS at an earlier stage in order to take appropriate measures.

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Pulmonary function abnormalities in Prader-Willi syndrome

Cited by 57 publications

References 18 publications

Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome

Growth hormone treatment for two years is safe and effective in adults with Prader-Willi syndrome

Fatal Outcome of Sleep Apnoea in PWS during the Initial Phase of Growth Hormone Treatment

Sudden Death of an Infant with Prader-Willi Syndrome – Not a Unique Case?

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