نتایج جستجو برای: vacterl

تعداد نتایج: 265  

2010
Charles Shaw-Smith

Esophageal atresia with/without tracheo-esophageal fistula is a relatively common malformation, occurring in around 1 in 3500 births. In around half of cases, additional malformations are present, forming either a syndrome of known genetic aetiology, or a recognised association, of which the VACTERL association (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistul...

Journal: :American journal of medical genetics. Part A 2012
Hannah M Tully Jennifer C Dempsey Gisele E Ishak Margaret P Adam Cynthia J R Curry Pedro Sanchez-Lara Alasdair Hunter Karen W Gripp Judith Allanson Christopher Cunniff Ian Glass Kathleen J Millen Daniel Doherty William B Dobyns

Rhombencephalosynapsis (RES) is an uncommon cerebellar malformation characterized by fusion of the hemispheres without an intervening vermis. Frequently described in association with Gómez-López-Hernández syndrome, RES also occurs in conjunction with VACTERL features and with holoprosencephaly (HPE). We sought to determine the full phenotypic spectrum of RES in a large cohort of patients. Infor...

Journal: :Archives of disease in childhood 1988
S Knowles R M Thomas R H Lindenbaum J W Keeling R M Winter

Pulmonary agenesis is an uncommon anomaly that has been reported in isolation and in association with other congenital defects. Such defects include oesophageal atresia, cardiac malformation, horseshoe kidney, and anal atresia. Over a period of three years we have seen five neonates or fetuses with unilateral agenesis of the lung. All the cases had three or more anomalies seen in the VACTERL se...

Journal: :Journal of Neonatal Surgery 2017

Journal: :Journal of medical genetics 1999
M C Digilio B Marino P Bagolan A Giannotti B Dallapiccola

Oesophageal atresia (OA) is a congenital defect associated with additional malformations in 30-70% of the cases. In particular, OA is a component of the VACTERL association. Since some major features of the VACTERL association, including conotruncal heart defect, radial aplasia, and anal atresia, have been found in patients with microdeletion 22q11.2 (del(22q11.2)), we have screened for del(22q...

Journal: :Birth Defects Research Part A: Clinical and Molecular Teratology 2011

Journal: :Journal of Clinical and Experimental Investigations 2014

Journal: :Turkish Journal of Pediatric Disease 2014

ژورنال: :مجله علوم پزشکی رازی 0
نسرین خالصی n khalesi استادیار و متخصص کودکان، بیمارستان علی بن ابیطالب، جاده خاش، دانشگاه علوم پزشکی و خدمات بهداشتی ـ درمانی زاهدان، زاهدان، ایران(*مؤلف مسؤ� فرود صالحی f salehi دستیار کودکان، دانشگاه علوم پزشکی و خدمات بهداشتی ـ درمانی زاهدان، زاهدان، ایران. کامبیز کشاورز k keshavarz دستیار کودکان، دانشگاه علوم پزشکی و خدمات بهداشتی ـ درمانی زاهدان، زاهدان، ایران.

مقدمه: آلبینیسم شامل گروهی از شرایط بالینی می باشد که به صورت ارثی منتقل می گردند. در مبتلایان به بیماری، رنگدانه ملانین در چشم، پوست و مو، کم است یا وجود ندارد. آلبینیسم، بیماری مجزا بوده و جز یک مورد به همراه میکروسفالی و هیپوپلازی انگشتان، تا به حال با هیچ گونه آنومالی دیگر گزارش نگردیده است. سندرم vacterl مشتمل بر آنومالی های متعددی از جمله آنومالی های ستون مهره ها، آنوس بسته، مشکلات قلبی، ...

Journal: :Indian pediatrics 2004
Dheeraj Shah Shailesh Sharma M M A Faridi Kiran Mishra

We report a term, small for gestational age neonate having full spectrum of VACTERL association. In addition, the neonate also had triad of signs and symptoms associated with prune belly syndrome. The concurrence of these two syndromes could lie in their common etiology of defect in mesodermal differentiation. Such a combination is extremely rare and is generally incompatible with life.

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