نتایج جستجو برای: ectrodactyly

تعداد نتایج: 300  

Journal: :Journal of medical genetics 1989
K Tucker A Lipson

We report here a father and daughter with digital abnormalities, nasolacrimal duct obstruction, and variable alopecia. The father had a cleft lip and palate and the daughter had choanal atresia. We propose they both have the EEC syndrome and show the variable expressivity of this disorder. Choanal atresia has not been previously reported in this condition.

Journal: :American journal of human genetics 1998
J R O'Quinn R C Hennekam L B Jorde M Bamshad

Congenital limb malformations rank behind only congenital heart disease as the most common birth defects observed in infants. Finding genes that cause defects in human limb patterning should be straightforward but has been limited, in part, by the bewildering spectrum of phenotypes, which are difficult to separate into etiologically distinct disorders. One approach to the identification of rele...

2017
Leiping Zhou Mingli Lv Min Zeng Yun Zhou Tian Yang Yu Yang Yunyun Cao Xiaoxiao Kong Jianmei Niu

We explored the value of the metacarpal bone plane in screening for serious fetal forearm and hand deformities, excluding simple polydactyly and dactylion deformity, by ultrasonographic examination. Observed the second to fifth metacarpal bone plane of fetuses in 20,139 pregnant women at a gestational age of 16 to 30 weeks in The International Peace Maternity &Child Health Hospital of China Wel...

2018
Gyu Hyeong Lee Ja Hea Gu

Ulnar ray deficiency is the least common type of congenital longitudinal deficiency, and it shows several distinct manifestations, including various finger deformities [1]. To date, no reports have been published describing ulnar ray deficiency with multiple finger deformities, including central polysyndactyly, ectrodactyly, and first-web space narrowing. We describe the case of a patient with ...

2015
Wei Yin Yaling Song Yangge Du Zhuan Bian Tao Cai

OBJECTIVES Ectrodactyly ectodermal dysplasia cleft lip/palate (EEC) syndrome and limb-mammary syndrome (LMS) share a similar phenotype and the same pathogenic gene, which complicates the ability to distinguish between these diagnoses. The current study aims to identify a potential and practical clinical biomarker to distinguish EEC from LMS. METHODS Two EEC pedigrees and one LMS pedigree that...

Journal: :AJP reports 2016
Matthew J Blitz Burton Rochelson

Introduction Ectrodactyly, also known as split hand/foot malformation, is a rare developmental abnormality of the limbs that consists of absent central digits, a deep median cleft, and fusion of the remaining lateral digits, ultimately producing clawlike extremities. This case represents one of the earliest reported diagnoses of this anomaly to utilize three-dimensional (3D) ultrasonography. Ca...

Journal: :Journal of medical genetics 2012
Eva Klopocki Silke Lohan Sandra C Doelken Sigmar Stricker Charlotte W Ockeloen Renata Soares Thiele de Aguiar Karina Lezirovitz Regina Celia Mingroni Netto Aleksander Jamsheer Hitesh Shah Ingo Kurth Rolf Habenicht Matthew Warman Koenraad Devriendt Ulrike Kordass Maja Hempel Anna Rajab Outi Mäkitie Mohammed Naveed Uppala Radhakrishna Stylianos E Antonarakis Denise Horn Stefan Mundlos

BACKGROUND Split-hand/foot malformation (SHFM)-also known as ectrodactyly-is a congenital disorder characterised by severe malformations of the distal limbs affecting the central rays of hands and/or feet. A distinct entity termed SHFLD presents with SHFM and long bone deficiency. Mouse models suggest that a defect of the central apical ectodermal ridge leads to the phenotype. Although six diff...

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