65:
37:
253:, which allows the bladder to drain through a small hole in the abdomen, thus helping to prevent urinary tract infections. Similarly, consistent self-catheterization, often several times per day, can be an effective approach to preventing infections. A more drastic procedure is a surgical "remodeling" of the abdominal wall and urinary tract. Boys often need to undergo an
200:
Prune belly syndrome can result in distention and enlargement of internal organs such as the bladder and intestines. Surgery is often required but will not return the organs to a normal size. Bladder reductions have shown that the bladder will again stretch to its previous size due to lack of muscle.
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while a child is still in-utero. Urinary tract dilation, bladder outlet obstruction, distended bladder wall and an abnormally large abdominal cavity with deficient abdominal wall musculature are key indicators, as the abdomen swells with the pressure of accumulated urine.
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Musculoskeletal abnormalities include pectus excavatum, scoliosis, and congenital joint dislocations including the hip. Diagnosis of prune belly syndrome necessitates a thorough orthopaedic evaluation because of the high prevalence of associated musculoskeletal
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and the child's going on dialysis or requiring a kidney transplant. Many individuals with prune belly syndrome have good physical and mental health, despite all the concerns. With proper treatment, however, a longer, healthier life is possible.
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often herald prune belly syndrome, as they are normally uncommon. If a problem is suspected, doctors can perform blood tests to check renal function. Another study that may suggest the syndrome is a
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Abdominal muscle deficiency syndrome, congenital absence of the abdominal muscles, Eagle-Barrett syndrome, Obrinsky syndrome, Fröhlich syndrome, triad syndrome
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Eagle JF, Barrett GS (1950). "Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome. Report of 9 cases".
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Brinker MR, Palutsis RS, Sarwark JF 1995. The orthopaedic manifestations of prune-belly (Eagle-Barrett) syndrome. J Bone Joint Surg Am. 77(2):251-7
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89:, characterized by a triad of symptoms. The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the
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367:"An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births"
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417:
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Obrinsky W (1949). "Agenesis of abdominal muscles with associated malformation of the genitourinary tract; a clinical syndrome".
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The type of treatment, like that of most disorders, depends on the severity of the symptoms. One option is to perform a
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inheritance has been suggested in some cases. A homozygous mutation in the muscarinic cholinergic receptor-3 gene (
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Frolich, F. Der Mangel der
Muskeln, insbesondere der Seitenbauchmuskeln. Dissertation: Wurzburg 1839.
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492:"Prune belly syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
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affecting about 1 in 40,000 births. About 97% of those affected are male. Prune belly syndrome is a
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Docimo, Steven G; Canning, Douglas; Pippi Salle, Joao Luiz; El-Khoury, Antoine E, eds. (2006).
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Alford, Bennett A.; Peoples, W. M.; Resnick, Jack S.; L'Heureux, Philippe R. (November 1978).
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Complications may also arise from enlarged/malformed kidneys, which may result in
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Later in life, a common symptom is post-ejaculatory discomfort. Most likely a
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445:"Pulmonary Complications Associated with the Prune-Belly Syndrome"
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Lacher, Martin; St. Peter, Shawn D.; Zani, Augusto, eds. (2021).
530:. Medical radiology (2 ed.). Berlin: Springer. p. 331.
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muscles. There may be wrinkly folds of skin covering the abdomen.
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The
Kelalis--King--Belman Textbook of Clinical Pediatric Urology
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Comprehensive
Neonatal Care: An Interdisciplinary Approach
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627:"OMIM Entry - # 100100 - PRUNE BELLY SYNDROME; PBS"
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697:The Journal of Bone & Joint Surgery | Article
241:) on chromosome 1q43 was reported in one family.
257:to move the testes to their proper place in the
645:"default - Stanford Medicine Children's Health"
44:Prune belly syndrome in an Egyptian child with
842:Congenital disorders of musculoskeletal system
101:Prune-belly triad consists of signs such as:
8:
150:due to the inability to properly expel urine
578:Obstetric Imaging: Fetal Diagnosis and Care
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668:Lott, Judy Wright; Kenner, Carole (2007).
214:Prune belly syndrome can be diagnosed via
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35:
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580:. Elsevier Health Sciences. p. 574.
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130:from the bladder to the ureters and the
526:Fotter, Richard; Avni, E. Fred (2008).
270:
837:Congenital disorders of urinary system
603:Pearls and tricks in pediatric surgery
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335:10.1001/archpedi.1949.02030040372008
672:. Saunders Elsevier. p. 193.
118:Urinary tract abnormality such as
14:
233:PBS is far more common in males.
115:(undescended testicles) in males
857:Syndromes affecting the kidneys
605:. Cham, Switzerland: Springer.
365:Baird PA, MacDonald EC (1981).
126:, accumulation and backflow of
406:Bhat, Sriram M. (2016-06-30).
105:A partial or complete lack of
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555:. United Kingdom: CRC Press.
222:In young children, frequent
93:of those with the disorder.
170:, it lasts about two hours.
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649:www.stanfordchildrens.org
496:rarediseases.info.nih.gov
153:Ventricular septal defect
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228:voiding cystourethrogram
224:urinary tract infections
148:urinary tract infections
409:SRB's Manual of Surgery
120:unusually large ureters
576:Copel, Joshua (2017).
528:Pediatric uroradiology
157:Malrotation of the gut
142:Other signs include:
136:vesicoureteral reflux
292:10.1542/peds.6.5.721
178:Pulmonary hypoplasia
75:Prune belly syndrome
22:Prune belly syndrome
235:Autosomal recessive
77:is a rare, genetic
787:External resources
97:Signs and symptoms
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612:978-3-030-51066-4
537:978-3-540-33005-9
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251:vesicostomy
186:atelectasis
27:Other names
831:Categories
772:DiseasesDB
654:2023-08-25
280:Pediatrics
265:References
255:orchiopexy
216:ultrasound
190:lung lobes
188:involving
83:congenital
815:radio/575
807:eMedicine
510:synd/1499
470:0033-8419
450:Radiology
245:Treatment
210:Diagnosis
182:pneumonia
162:Club foot
146:Frequent
54:Specialty
812:med/3055
343:18116668
308:26235702
300:14797335
766:D011535
393:6454342
384:1685049
259:scrotum
132:kidneys
124:bladder
91:abdomen
801:001269
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304:S2CID
239:CHRM3
128:urine
761:MeSH
750:OMIM
739:9-CM
674:ISBN
607:ISBN
582:ISBN
557:ISBN
532:ISBN
474:PMID
466:ISSN
414:ISBN
389:PMID
339:PMID
296:PMID
735:ICD
720:ICD
513:at
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379:PMC
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