abdominal muscle deficiency syndrome

(redirected from prune belly)

ab·dom·i·nal mus·cle de·fi·cien·cy syn·drome

[MIM*100100, MIM*264140]
congenital absence (partial or complete) of abdominal muscles, in which the outline of the intestines is visible through the protruding abdominal wall; in men, genitourinary anomalies (urinary tract dilation and cryptorchidism) are also found; genetics unclear.
See also: prune belly syndrome.

abdominal muscle deficiency syndrome

n.
Congenital absence of abdominal muscles, in which the outline of the intestines is visible through the protruding abdominal wall and the skin in the abdominal region is wrinkled.
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References in periodicals archive ?
In the literature, cases of congenital mydriasis accompanied by coronary artery disease, aortic aneurism, smooth muscle cell dysfunction (6), gene mutations (ACTA2) (7), Prune Belly syndrome (7), septo-optic dysplasia which may be observed in association with cerebrovascular diseases (8) and megacystic microcolon intestinal hypoperistaltism syndrome (9) have been reported.
Eight-year-old Fuller was diagnosed with prune belly syndrome at birth, which requires her to have kidney dialysis three times a week.
James suffers from a birth defect known as prune belly syndrome, which affects the development of abdominal muscles.
The renal dysplasia-associated syndromes include Meckel syndrome, VATER association, renal coloboma syndrome, Herlyn-Werner-Wunderlich syndrome, prune belly syndrome, branchio-oto-renal dysplasia, renal-hepatic-pancreatic dysplasia, and multiple endocrine neoplasia type 2A.
INTRODUCTION: Prune belly syndrome is a rare congenital disease characterized by abdominal wall muscle abnormalities, urinary tract abnormalities and cryptorchidism.
Table 2 shows that the most commonly observed abnormalities associated with CKD development were Ochoa syndrome, renal hypoplasia, prune belly syndrome, and posterior urethral valves.
La etiologia de esta condicion es poco conocida y se ha asociado con el fallo de diferenciacion de las columnas mesodermicas, encontrandose mayormente asociado a sindrome Prune Belly.
Aidan Pert, 12, is one of just four people in the UK with prune belly syndrome, named after sufferers' wrinkled abdomens.
Sin embargo, presenta malformaciones mayores en aproximadamente el 10% de los casos; (4,5) se encuentran reportes de gastrosquisis, atresia colonica, atresia del apendice cecal, (6) sindrome de Prune Belly, (7) transposicion de grandes vasos, (8) necrosis cortical renal, asociacion de VATER, ventriculo unico (9) y defectos del sistema nervioso central como necrosis cerebelar, hidranencefalia, porencefalia y microcefalia.
Also known as Prune Belly, the condition leaves sufferers with a range of problems including weak abdominal muscles and major kidney trouble.