Respiratory function in the prune-belly syndrome.

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Prune Belly Syndrome

Prune belly syndrome is a rare congenital disorder of the urinary system, characterized by a triad of abnormalities. The aetiology is not known. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies.

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The Phenotypic and Pathological Features of Prune-Belly Syndrome Prune-belly sendromunda Fenotipik ve Patolojik özellikler

Objective: Prune-belly syndrome is a rare congenital disorder characterized by musculature deficiency in the abdominal wall, lower urinary tract obstruction, other urinary tract anomalies, and bilateral cryptorchidism. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Over 95% of patients are male. Urinary tract disease is the major prognostic ...

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Prune Belly Syndrome with Situs Inversus Abdominus

A full term male baby born to a 23-year-old primigravida did not develop spontaneous respiration after birth. His heart rate was <40/min and required bag and tube ventilation; and shifted to NICU for further management. Antenatal ultrasound at 36 weeks showed single live fetus with grossly distended bladder with dilated proximal urethra, bilateral hydroureters causing significant intra-abdomina...

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VACTERL association with Prune-Belly syndrome.

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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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 1993

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.68.4.505