Linear growth response to exogenous growth hormone in Prader‐Willi syndrome

Lee, Phillip; Wilson, Darrell M.; Rountree, Lois; Hintz, Raymond L.; Rosenfeld, Ron G.; Reynolds, James F.; Ledbetter, David H.

doi:10.1002/ajmg.1320280411

Cited by 52 publications

(25 citation statements)

References 16 publications

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“…Studies by both Ruvalcaba and Holm [21] and Angulo et al [3] also indicate increases in growth velocity (>3 cm/year) in PWS children after initiation of GH therapy, as well as improved weight status. Similar improvements in growth velocity in PWS children were noted by Lee et al [4]. …”

Section: Discussionsupporting

confidence: 86%

“…Growth failure is a common feature of this disorder; however, there is controversy surrounding the etiology. Some studies have suggested that it is the result of inadequate growth hormone (GH) secretion resulting from hypothalamic dysfunction [2, 3, 4]. However, other authors have argued that the obesity associated with PWS causes an abnormal response to GH secretagogues [5].…”

Section: Introductionmentioning

confidence: 99%

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Growth Failure in Prader-Willi Syndrome Is Secondary to Growth Hormone Deficiency

Thacker

Hainline²,

Dennis-Feezle³

et al. 1998

Horm Res Paediatr

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Growth failure is a recognized feature of the Prader-Willi syndrome (PWS). Despite evidence that hypothalamic dysfunction accompanies the syndrome, the etiology of this growth failure remains controversial because most patients with PWS are obese. In order to contribute to resolution of this controversy, we performed a retrospective analysis of 16 obese and non-obese PWS children. GH deficiency was diagnosed in 12 of the 16 subjects and occurred independently of weight status. All of the non-obese subjects were GH deficient. Of the 4 GH-sufficient children, 2 were moderately obese and 2 were morbidly obese. One of these children had clinical evidence of GH deficiency including a low IGF-1 level. Only one of the children had evidence of GH deficiency and a normal IGF-1 level, a pattern that could be attributable to obesity. We conclude that most short children with PWS have growth hormone deficiency and that this deficiency probably results from hypothalamic dysfunction.

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Section: Discussionsupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Growth Failure in Prader-Willi Syndrome Is Secondary to Growth Hormone Deficiency

Thacker

Hainline²,

Dennis-Feezle³

et al. 1998

Horm Res Paediatr

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“…There is recent evidence for growth hormone deficiency at least in some patients with PWS and favorable responses to growth hormone therapy [16] . The use of our anthropometric standards may be helpful in those patients who have PWS being treated with growth hormone .…”

Section: Methodsmentioning

confidence: 99%

Standard Growth Curves in Prader-Willi Syndrome in Japan

Nagai

Tsuchiya

Fukushima

et al. 1993

Clin Pediatr Endocrinol

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Abstract.We report standard growth curves in Japanese Prader -Willi syndrome (PWS) for height. Growth patterns for height are quite different between PWS and normal controls ,showing the mean height in PWS ranging at -2SD of normal control data until pubertal age and dropping off far below -2SD of normal control after puberty . These data indicate that the pathogenesis of the short stature of PWS originates from both chromosomal abnormalities and hypogonadism.In addition to these factors we suspect that bone dysplasia seen in patients with this syndrome is also one of the factors in the short stature of PWS .

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“…Short stature is one of the syndrome's distinctive fea tures [27], Its cause has not yet been clearly established, although the involvement of a hypothalamic-pituitary dysfunction has been suspected for years [28]. Low GH concentrations were found in a few patients [29.…”

Section: Prader-willi Syndromementioning

confidence: 99%

Growth Hormone and Dysmorphic Syndromes

Galasso¹,

Scirè²,

Boscherini³

1995

Horm Res

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In order to study the pathogenesis of short stature in some of the best known and most frequent dysmorphic syndromes, we have reviewed the most significant studies conducted on somatrotropin secretion and on response to replacement treatment with human growth hormone in pediatric patients. In particular, the study examines the results presented in the literature, and in a few of our cases, those obtained with regard to Noonan, Silver-Russell and Prader-Willi syndrome patients, to achondroplasia and hypochondroplasia patients, and to Down syndrome patients. Finally, we shall present a review of a few, less frequent dysmorphic syndromes with short stature, in which a growth hormone deficiency has been diagnosed and replacement treatment attempted.

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Linear growth response to exogenous growth hormone in Prader‐Willi syndrome

Cited by 52 publications

References 16 publications

Growth Failure in Prader-Willi Syndrome Is Secondary to Growth Hormone Deficiency

Growth Failure in Prader-Willi Syndrome Is Secondary to Growth Hormone Deficiency

Standard Growth Curves in Prader-Willi Syndrome in Japan

Growth Hormone and Dysmorphic Syndromes

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