Extractions: to webmaster@prunebelly.org Welcome! Here you will find a collection of information about Prune Belly Syndrome which is also known as Eagle-Barrett Syndrome. The Prune Belly Syndrome Network, Inc. provides support for those who have Prune Belly, their families, friends and the health care professionals who treat them. NEW!
Pediatric Database Offers a definition of prune belly syndrome, the epidemiology, pathogenesis, pathology, clinical features, investigations and management. http://www.icondata.com/health/pedbase/files/PRUNEBEL.HTM
Extractions: Pediatric Database (PEDBASE) Discipline: GU Last Updated: 6/05/94 A disorder characterized by the triad of abdominal musculature deficiency, anomalous development of the urinary tract, and bilateral cryptorchidism. risk factors: markedly enlarged capacity with thickened wall may show an "hourglass configuration" on voiding cystogram
Extractions: About Prune Belly ... Links Also known as Eagle-Barrett syndrome, the Prune Belly syndrome consists of a triad of features: Anterior abdominal wall: musculature is deficient or absent. Urinary tract anomalies (mega-ureters, large bladder). Bilateral cryptorchidism (undescended testicles). The incidence of prune belly syndrome is 1 in 40,000 live births; 95% of cases occur in males. In the past, it was suggested that the abdominal wall was deformed by pressure from a distended bladder due to bladder outlet obstruction in utero. Currently it is thought that prune belly syndrome is a multisystem disease complex which derives from a primary defect in mesodermal development. The major prognostic factor is the degree of dilation of the urinary tract; 20% of patients are stillborn, 30% die of renal failure or urosepsis within the first two years of life, and the remaining 50% have varying degrees of urinary pathology. Renal abnormalities vary widely. There may be agenesis or hypoplasia, which is rare, or hydronephrosis. On IVP, 80% of patients have evident tortuous ureters with dilatation distally. Vesicoureteral reflux is common. The bladder is generally dilated, and the trabecula are thinned. A patent urachus may allow the bladder to decompress.
Prune Belly Syndrome prune belly syndrome. INTRODUCTION. Management of the child with prune belly syndrome(PBS) has posed a significant amount of trouble to the pediatric urologist. http://www.pedsurology.com/prune.htm
Extractions: Prune Belly Syndrome INTRODUCTION Management of the child with prune belly syndrome (PBS) has posed a significant amount of trouble to the pediatric urologist. There have been calls for conservative management of the urinary tract in these boys by some authors while others have advocated an aggressive approach to the management of these patients, operating on them at ten days of life. At the present time, no definitive timing for therapy has been substantiated one way or the other. What we have been able to see is that prune belly syndrome can present with a spectrum of abnormalities ranging from the stillborn infant with severe urogenital and pulmonary problems to the child with little, if any, urological abnormalities requiring no therapy other than orchidopexies to correct the undescended testes. It appears that the decision to intervene in the management of the urinary tract of these children should be based on the clinical presentation and not solely on radiographic appearance. There is little controversy in the literature as to the present management of the undescended testes in these children. If controversy or confusion arises, it is to the optimal timing for the surgery for the orchidopexy as well the type of orchidopexy to be performed. Similarly, the management of the abdominal wall abnormalities has several camps with some going for no surgical reconstruction of the abdominal wall while others advocate surgical reconstruction and this group being divided amongst two separate factions advocating different techniques of abdominal wall reconstruction.
Prune Belly Syndrome prune belly syndrome. National prune belly syndrome Association. 30 Salem Blvd. http://www.kumc.edu/gec/support/prunbely.html
Prune Belly (Eagle-Barrett) Syndrome NORD prune belly syndrome - Offers a list of synonyms, a general discussion and further resources. http://rarediseases.about.com/library/weekly/aa032302a.htm
Extractions: Prune Belly (Eagle-Barrett) Syndrome Set of distinctive physical problems Join the Discussion "I just wanted to point out to any parent or relative of a child with this disease that the outcome is often far better than conventional medical wisdom would have it. To ask questions or talk to others who are dealing with this rare and orphan disease, please visit www.prunebelly.org. There is a support forum."
NORD - Prune Belly Syndrome Offers a list of synonyms, a general discussion and further resources. http://www.stepstn.com/cgi-win/nord.exe?proc=GetDocument&rectype=0&recnu
Virtual Children's Hospital: Paediapaedia: Prune-Belly Syndrome (Eagle-Barrett S DEVELOPMENT OF THE URINARY SYSTEM prune belly syndrome Select Entry from OMIM This page is for computers without external internet access. List of OMIM search results The appellation 'prune belly syndrome' is descriptive because the intestinal pattern is evident through the thin, lax, http://www.vh.org/Providers/TeachingFiles/PAP/GUDiseases/Prune.html
Prune Belly Syndrome - Lucile Packard Children's Hospital Genitourinary Kidney Disorders. prune belly syndrome What is prunebelly syndrome? Prune What causes prune belly syndrome? Prune http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/urology/pbs.html
Extractions: What is prune belly syndrome? Prune belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome. It is characterized by a triad of abnormalities that include the following: Because of the substantial involvement of the urinary tract, children with prune belly syndrome are usually unable to completely empty their bladders and have serious bladder, ureter, and kidney impairment. A child with prune belly syndrome may also have other birth defects. Most commonly, these defects involve the skeletal system, intestines, and heart. Girls may have defects in their external genitalia, as well. Some infants who have prune belly syndrome may be stillborn or die within a few months of birth.
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Radiology Pediatrics Last Updated: December 7, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: congenital absence of abdominal musculature, deficiency of abdominal musculature, Eagle-Barrett syndrome, triad syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Differentials X-ray ... Bibliography Author: Beverly P Wood, MD, MS , Professor, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California Beverly P Wood, MD, MS, is a member of the following medical societies: American Academy of Pediatrics American Association for Women Radiologists American College of Radiology American Institute of Ultrasound in Medicine ... American Roentgen Ray Society , Association of University Radiologists, Radiological Society of North America , and Society for Pediatric Radiology Editor(s): Robert J Starshak, MD
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Urology Last Updated: August 24, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: PBS, Eagle-Barrett syndrome, triad syndrome AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications ... Bibliography Author: Israel Franco, MD , Assistant Professor, Departments of Urology and Pediatrics, Division of Pediatric Urology, New York Medical College Israel Franco, MD, is a member of the following medical societies: American Academy of Pediatrics American College of Surgeons American Urological Association Endourological Society ... Medical Society of the State of New York , and Society of Laparoendoscopic Surgeons Editor(s): Peter Langenstroer, MD , Assistant Professor, Department of Surgery, Division of Urology, Medical College of Wisconsin; Francisco Talavera, PharmD, PhD
Birth Disorder Information Directory - P List of Sites. Proximal Femoral Focal Deficiency List of Sites. prune belly syndrome(Abdominal Muscle Absence/Aplasia/Deficiency/Defect Anomalad/Syndrome, Eagle http://www.bdid.com/defectp.htm
Extractions: HOME P-4 Deletion Syndrome p47-PHOX, Deficiency of p67-PHOX, Deficiency of Pachydermopreiostosis (Hypertrophic Osteoarthropathy, Primary or Idiopathic) Pachyonychia Congenita Pagod Syndrome Pagon Bird Detter Syndrome (Anemia Sideroblastic Spinocerebellar Ataxia) Pagon Syndrome Pallister Syndrome Pallister Hall Syndrome (Hypothalamic Hamartoblastoma, Hypopituitarism, Imperperforate Anus, and Postaxial Polydactyly) Pallister Killian Syndrome V-KI-RAS2 KIRSTEN RAT SARCOMA 2 VIRAL ONCOGENE HOMOLOG; KRAS2
UNSW Embryology-OMIM PRUNE BELLY SYNDROME Embryology Home Page. prune belly syndrome. Select Entry from OMIM. Alternativetitles; symbols. prune belly syndrome. TABLE OF CONTENTS. http://anatomy.med.unsw.edu.au/cbl/embryo/OMIMfind/kidney/OMIM-100100.htm
Extractions: DEVELOPMENT OF THE URINARY SYSTEM Embryology Home Page Online Mendelian Inheritance in Man (Internet Link) This page is for computers without external internet access. Back to UNSW Embryology-Kidney Notes List of OMIM search results TEXT REFERENCES SEE ALSO CREATION DATE ... "16 MEDLINE Citations" Note: pressing the symbol will find the citations in MEDLINE whose text most closely matches the text of the preceding OMIM paragraph, using the Entrez MEDLINE neighboring function. This condition was first described by Frolich (1839) . The appellation 'prune belly syndrome' is descriptive because the intestinal pattern is evident through the thin, lax, protruding abdominal wall in the infant ( Osler, 1901
UNSW Embryology-OMIM PRUNE BELLY SYNDROME prune belly syndrome. Select Entry from OMIM. 264140 prune belly syndrome WITHPULMONIC STENOSIS, MENTAL RETARDATION, AND DEAFNESS. TABLE OF CONTENTS. http://anatomy.med.unsw.edu.au/cbl/embryo/OMIMfind/kidney/OMIM-264140.htm
Extractions: DEVELOPMENT OF THE URINARY SYSTEM Embryology Home Page Online Mendelian Inheritance in Man (Internet Link) This page is for computers without external internet access. Back to UNSW Embryology-Kidney Notes List of OMIM search results TEXT REFERENCES CREATION DATE EDIT HISTORY ... "1 MEDLINE Citation" Lockhart et al. (1979) described a family in which 2 brothers and a sister had this combination. A fourth sib may have been affected; the mother reported that the male, who died 25 hours after birth, had 'a big bladder, big ureters, bad kidneys.' The hearing loss was sensorineural in nature. Because the involvement in the female was less marked than in the males, the possibility of X-linked inheritance was suggested. No information concerning abnormalities in the mother was provided. 1. Lockhart, J. L.; Reeve, H. R.; Bredael, J. J.; Krueger, R. P. :
OHSU Doernbecher - Genitourinary & Kidney Disorders prune belly syndrome, RELATED SERVICES. , Urology. What is prune belly syndrome?prune belly syndrome is also known as triad syndrome or EagleBarrett syndrome. http://www.ohsuhealth.com/dch/health/urology/prune.asp
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MEDLINEplus Medical Encyclopedia: Prune Belly Syndrome prune belly syndrome. Causes, incidence, and risk factors Return totop The underlying causes of prune belly syndrome are unknown. http://www.nlm.nih.gov/medlineplus/ency/article/001269.htm
Extractions: Skip navigation Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Contents of this page: Abdominal muscles Alternative names Return to top Eagle-Barrett syndrome Definition Return to top A disease characterized by three major findings (triad): deficient abdominal muscles (causing the skin of the abdomen to wrinkle like a prune), undescended testicles , and a dilated abnormal urinary tract. Causes, incidence, and risk factors Return to top The underlying causes of prune belly syndrome are unknown. There is blockage to the flow of urine from the kidney to the outside of the body during fetal life. For example, dilatation of the urinary tract may develop as a result of urethral obstruction in the womb. The incidence is approximately 1 in 30,000 to 40,000 births. Boys are almost solely affected.
Prune Belly Syndrome (PBS) View the Full Record Syndrome, prune belly syndrome (PBS). prune belly syndrome mayoccur with or without pulmonic stenosis, deafness, and mental retardation. http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome542.html
Extractions: Syndrome prune belly syndrome (PBS) Synonyms Eagle-Barrett syndrome Fröhlich syndrome Obrinsky syndrome abdominal muscle deficiency anomalad abdominal muscle deficiency syndrome abdominal musculature aplasia syndrome absence of abdominal muscle syndrome aplastic abdominal muscle syndrome defective abdominal wall syndrome Summary A syndrome of hypoplasia or aplasia of the abdominal muscle, presenting a thin, loose, wrinkled and shriveled (prunelike) abdominal wall, with furrowlike umbilicus, and other anomalies, including persistent urachus, pigeon breast deformity, and a variety of gastrointestinal, cardiovascular, urogenital, and skeletal abnormalities. Prune belly syndrome may occur with or without pulmonic stenosis, deafness, and mental retardation. Distention of the bladder and urethra in utero is considered as a possible cause of abdominal overdistention. Major Features Nose: Recurrent epistaxis. Mouth and oral structures: Occasional cleft lip.
Child Health Library - Urology - Prune Belly Syndrome Information Concerning prune belly syndrome from the Child Health Libraryof Children's Hopital of Pittsburgh. prune belly syndrome http://www.chp.edu/greystone/urology/pbs.php
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