Literature DB >> 6683267

The Prader-Willi syndrome: neuroendocrine study of identical twins.

C Trevisan, A Saia, E Schergna, F Mantero.   

Abstract

Identical twins with the Prader-Willi syndrome are reported. Apart from hypogonadism, hypomentia, hypotonia and obesity, they presented shorter than normal stature and the peculiar facies of this syndrome. Both twins also suffered from arterial hypertension with secondary hyperaldosteronism, an abnormality never previously recorded. The endocrinological study showed the presence of hypogonadotrophic hypogonadism in both twins. The GnRH and clomiphene tests suggested a hypothalamic disorder. Although the vast majority of cases with the Prader-Willi syndrome are isolated, the expression of this disorder in two identical twins enhances the possibility of a genetic determination.

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Year:  1983        PMID: 6683267     DOI: 10.1007/bf02043442

Source DB:  PubMed          Journal:  Ital J Neurol Sci        ISSN: 0392-0461


  16 in total

1.  Hypogonadism in Prader-Willi syndrome.

Authors:  N Wannarachue; R H Ruvalcaba
Journal:  Am J Ment Defic       Date:  1975-03

2.  The Prader-Willi syndrome with a 15/15 translocation. Case report and review of the literature.

Authors:  C J Hawkey; A Smithies
Journal:  J Med Genet       Date:  1976-04       Impact factor: 6.318

3.  Anterior pituitary function in the Prader-Labhart-Willi (PLW) syndrome.

Authors:  G Tolis; W Lewis; M Verdy; H G Friesen; S Solomon; G Pagalis; F Pavlatos; P Fessas; J G Rochefort
Journal:  J Clin Endocrinol Metab       Date:  1974-12       Impact factor: 5.958

4.  Prader-Willi syndrome in infant monozygotic twins.

Authors:  J E Brissenden; E P Levy
Journal:  Am J Dis Child       Date:  1973-07

5.  Syndrome of hypotonia-hypomentia-hypogonadism-obesity (HHHO) or Prader-Willi syndrome.

Authors:  H Zellweger; H J Schneider
Journal:  Am J Dis Child       Date:  1968-05

6.  Response to LH-RH and clomiphene citrate in two women with the Prader-Labhart-Willi syndrome.

Authors:  W L McGuffin; A D Rogol
Journal:  J Clin Endocrinol Metab       Date:  1975-08       Impact factor: 5.958

7.  Absence of spermatogonia in the Prader-Willi syndrome.

Authors:  M L Katcher; G J Bargman; E F Gilbert; J M Opitz
Journal:  Eur J Pediatr       Date:  1977-03-18       Impact factor: 3.183

8.  15/15 translocation in Prader-Willi syndrome.

Authors:  M Fraccaro; O Zuffardi; E M Buhler; L P Jurik
Journal:  J Med Genet       Date:  1977-08       Impact factor: 6.318

9.  Endocrine function in the Prader-Willi syndrome.

Authors:  W J Jeffcoate; B M Laurance; C R Edwards; G M Besser
Journal:  Clin Endocrinol (Oxf)       Date:  1980-01       Impact factor: 3.478

10.  Prader-Willi syndrome and scoliosis.

Authors:  V A Holm; E L Laurnen
Journal:  Dev Med Child Neurol       Date:  1981-04       Impact factor: 5.449

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  1 in total

1.  A genetic model for the Prader-Willi syndrome and its implication for Angelman syndrome.

Authors:  I Kennerknecht
Journal:  Hum Genet       Date:  1992 Sep-Oct       Impact factor: 4.132

  1 in total

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