Literature DB >> 5173168

Unusual facies, joint hypermobility, genital anomaly and short stature: a new dysmorphic syndrome.

C I Scott.   

Abstract

Three brothers with a previously unrecognized pattern of malformations are presented. The syndrome is characterized by short stature; a broad, prominent forehead, hypertelorism, congenital ptosis, a broad, short nose with anteverted nostrils, a long, broad upper lip, low-set, abnormally shaped and posteriorly rotated ears; simian palmar creases; brachyclinodactyly; short fingers; ligamentous laxity allowing for hyperextensibility of the fingers, genu recurvatum, flat feet; and an anomalous penoscrotal configuration resulting in "saddle" deformity with scrotal folds incircling the base of the penis. This disorder is apparently transmitted as an X-linked recessive trait. It is important to recognize this syndrome because of its heritability and for prevention of neurologic problems consequent to ligamentous laxity and malformation of cervical vertebras.

Entities:  

Mesh:

Year:  1971        PMID: 5173168

Source DB:  PubMed          Journal:  Birth Defects Orig Artic Ser        ISSN: 0547-6844


  13 in total

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5.  Intelligence and development in Aarskog syndrome.

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6.  Faciogenital dysplasia protein (FGD1) regulates export of cargo proteins from the golgi complex via Cdc42 activation.

Authors:  Mikhail V Egorov; Mariagrazia Capestrano; Olesya A Vorontsova; Alessio Di Pentima; Anastasia V Egorova; Stefania Mariggiò; M Inmaculada Ayala; Stefano Tetè; Jerome L Gorski; Alberto Luini; Roberto Buccione; Roman S Polishchuk
Journal:  Mol Biol Cell       Date:  2009-03-04       Impact factor: 4.138

7.  New autosomal recessive faciodigitogenital syndrome.

Authors:  A S Teebi; K K Naguib; S Al-Awadi; Q A Al-Saleh
Journal:  J Med Genet       Date:  1988-06       Impact factor: 6.318

8.  Cerebrovascular disease associated with Aarskog-Scott syndrome.

Authors:  Michael L Diluna; Nduka M Amankulor; Michele H Johnson; Murat Gunel
Journal:  Neuroradiology       Date:  2007-02-10       Impact factor: 2.995

9.  Identification of novel mutations in Mexican patients with Aarskog-Scott syndrome.

Authors:  Mariana Pérez-Coria; José J Lugo-Trampe; Michell Zamudio-Osuna; Iram P Rodríguez-Sánchez; Angel Lugo-Trampe; Beatriz de la Fuente-Cortez; Luis D Campos-Acevedo; Laura E Martínez-de-Villarreal
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Review 10.  Syndromic disorders with short stature.

Authors:  Zeynep Şıklar; Merih Berberoğlu
Journal:  J Clin Res Pediatr Endocrinol       Date:  2014
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