Cloacal Exstrophy: a complex disease

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Cloacal exstrophy: a complex disease.

INTRODUCTION Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary app...

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A newborn with trisomy 13 presenting with cloacal exstrophy.

Trisomy 13 syndrome is a rare disorder that carries a high mortality rate due to abnormal prenatal development resulting in serious birth defects. Although genitourinary malformations are commonly seen in trisomy 13 syndrome, to our knowledge, the association of cloacal exstrophy with trisomy 13 has been extremely rarely reported. Herein, a newborn with trisomy 13 syndrome having multiple conge...

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Cloacal Exstrophy with Intravesical Phallus: An Intra-operative Revelation in a case of OEIS Complex

Cloacal exstrophy is a complex congenital malformation and presents more often than not as a spectrum of anomalies. Gender assignment is difficult in these babies because of the ambiguity of the genitalia and the major surgical undertaking which may make the salvage of certain delicate structures an arduous task. Awareness of the variety of ways in which the phallus may be positioned with respe...

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Urea salvage in a neonate with cloacal exstrophy.

Urea kinetics were measured in a child with congenital absence of the colon on days 15, 19, and 23 of age. Urea salvage was 5% of urea production in the first study, increasing to 79% by the third. This provides evidence that the colonic microflora play a more active part in urea salvage than the mucosa and that the establishment of an active lower ileal microflora takes over some of the metabo...

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Y chromosome aberration in a patient with cloacal-bladder exstrophy-epispadias complex: an unusual finding.

Chromosome aberrations or genetic syndromes associated with cloacal-bladder exstrophy complex have rarely been reported. The aim of this report is to describe a 14 year-old female Brazilian patient with a complex urogenital malformation, short stature, lack of secondary se-xual characteristics and Y chromosome aberration. A girl with cloacal bladder exstrophy complex was referred for evaluation...

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ژورنال

عنوان ژورنال: International braz j urol

سال: 2013

ISSN: 1677-5538

DOI: 10.1590/s1677-5538.ibju.2013.06.19