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

Abstract

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.

Prader-Willi syndrome

“…49 Concern about the possible contribution of GH administration to unexpected death has been raised by reported deaths of individuals within a few months of starting GH therapy. [50][51][52][53][54][55] The few reported deaths were mostly in obese individuals who had preexisting respiratory or cardiac disorders with evidence of upper airway obstruction and uncorrected tonsillar and adenoidal hypertrophy. In the database of one pharmaceutical company, five of 675 children treated with GH died suddenly of respiratory problems.…”

Abordagem terapêutica da obesidade na Síndrome de Prader-Willi

“…Existem alguns relatos de caso de morte súbita em pacientes portadores de SPW após início da terapia de reposição com GH (28)(29)(30)(31). Em razão disso, foram realizados estudos com a finalidade de elucidar o…”

Growth hormone supplement treatment reduces the surgical risk for Prader–Willi Syndrome patients

“…However, there are some concerns regarding the risks involved in GH treatment of PWS patients. First, there was a report of sudden death owing to the use of GH treatment [12]. Although the relationship between the sudden death and GH treatment was completely unclear, Nagai et al [13] subsequently reported that the causes of sudden unexpected death did not differ between PWS patients with or without GH treatment.…”