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         Prune Belly Syndrome:     more detail
  1. Prune-belly syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by David, MD Greenberg, 2005

41. Dr. Koop - Prune Belly Syndrome
prune belly syndrome, Jul 29, 2005 obstruction is made, it may be possibleto prevent it from progressing to prune belly syndrome with prenatal surgery.
http://www.drkoop.com/ency/93/001269prv.html
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Prune belly syndrome
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Prune belly syndrome
Alternative Names: Eagle-Barrett syndrome
Prevention: There is no known guaranteed prevention. If a prenatal diagnosis of urinary tract obstruction is made, it may be possible to prevent it from progressing to prune belly syndrome with prenatal surgery.
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42. Rosser
prune belly syndrome and Tracheoesophageal Fistula in a Premature Neonate prune belly syndrome, also known as Eagle-Barrett syndrome, is awell known
http://www.duj.com/Article/Rosser2/Potter.html
ORIGINAL REPORTS
Prune Belly Syndrome and Tracheo-esophageal Fistula in a Premature Neonate
Lance Potter
Charles J. Rosser
S. Iskandar
R. Lawrence Kroovand
Departments of Urology and Pathology
Wake Forest University School of Medicine, Winston-Salem, North Carolina27157
Prune Belly Syndrome, also known as Eagle-Barrett syndrome, is awell known triad of abdominal muscle deficiency or hypoplasia, urinarytract anomalies, and bilateral cryptorchidism. Tracheo-esophagealfistula is a sporadically occurring defect estimated to occur in 1 out4425 live births. Only one case of Prune Belly Syndrome, tracheo-esophagealfistula associated with VATER syndrome, and urethral atresia occurringin the same individual has been reported. We present a uniquecase involving a 33 week fetus with bladder distention and bilateral hydroureteronephrosisdiagnosed in utero who postnatally was diagnosed with Prune Belly Syndrome,tracheo-esophageal fistula, and urethral atresia.
CASE REPORT After the placement of a 8 Fr. percutaneous suprapubic tube, a cystogramwas performed which demonstrated a large bladder with severe trabeculation.After instillation of 200 milliliters of cystograffin under gravity, bilateralgrade 5 ureteral reflux was evident by the presence of contrast in therenal pelvis bilaterally On day four of life, the patient was noted to have a creatinine of 1.0mg/dl. Urine output during this time was between 1.5-3 ml/kg/hr. Repeatabdominal ultrasonography demonstrated worsening hydroureteronephrosis.The patient was taken to the operating room for an exploratory laparotomyand urinary diversion. The bladder was noted to have two large

43. Rosser
prune belly syndrome and Tracheoesophageal Fistula in a Premature Neonate prune belly syndrome, also known as Eagle-Barrett syndrome, is a well known
http://www.duj.com/Article/Potter.html
ARTICLES
Prune Belly Syndrome and Tracheo-esophageal Fistula in a Premature Neonate
Lance Potter
Charles J. Rosser
S. Iskandar
R. Lawrence Kroovand
Departments of Urology and Pathology
Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157
Prune Belly Syndrome, also known as Eagle-Barrett syndrome, is a well known triad of abdominal muscle deficiency or hypoplasia, urinary tract anomalies, and bilateral cryptorchidism. Tracheo-esophageal fistula is a sporadically occurring defect estimated to occur in 1 out 4425 live births. Only one case of Prune Belly Syndrome, tracheo-esophageal fistula associated with VATER syndrome, and urethral atresia occurring in the same individual has been reported. We present a unique case involving a 33 week fetus with bladder distention and bilateral hydroureteronephrosis diagnosed in utero who postnatally was diagnosed with Prune Belly Syndrome, tracheo-esophageal fistula, and urethral atresia.
CASE REPORT After the placement of a 8 Fr. percutaneous suprapubic tube, a cystogram was performed which demonstrated a large bladder with severe trabeculation. After instillation of 200 milliliters of cystograffin under gravity, bilateral grade 5 ureteral reflux was evident by the presence of contrast in the renal pelvis bilaterally (Figure 1) . Next, abdominal ultrasonography was performed revealing moderate bilateral hydroureteronephrosis, thickened bladder wall, and free intraperitoneal fluid. The patientís initial laboratory results revealed a normal serum creatinine of 0.3 gm/dl. Her postnatal course was complicated by the discovery of free intraabdominal air on day 1 of life. The patient was taken immediately to the operating room where a gastric perforation was discovered secondary to a distal tracheo-esophageal fistula with a proximal pouch. No esophageal atresia was present. The esophagus was ligated and a gastrotomy tube placed.

44. Short Description Of Cell Lines. Pathology: Prune Belly Syndrome 264140
Pathology prune belly syndrome 264140 OMIM record. By selecting the cell linename, you will receive the detailed description of the cell line
http://www.biotech.ist.unige.it/cldb/pat260.html
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Short description of cell lines.
Pathology: Prune Belly syndrome
OMIM record
By selecting the cell line name , you will receive the detailed description of the cell line
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human, Caucasian
amnion GGB
human, Caucasian
...
By Beatrice...

45. Prune Belly Syndrome Information Diseases Database
prune belly syndrome,EagleBarrett syndrome, Disease Database Information.
http://www.diseasesdatabase.com/ddb31089.htm
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46. Indian Pediatrics - Editorial
prune belly syndrome is the triad of deficiency of abdominal muscles, The complete form of prune belly syndrome consists of an abdominal wall deficient
http://www.indianpediatrics.net/aug2004/aug-845-847.htm
Home Past Issue About IP About IAP ... Subscription Case Reports Indian Pediatrics 2004; 41:845-847 VACTERL Association with Prune-Belly Syndrome
Dheeraj Shah
Shailesh Sharma
M.M.A. Faridi
Kiran Mishra*
From the Division of Neonatology, Department of Pediatrics and *Department of Pathology, University College of Medical Sciences, Dilshad Garden, Delhi 110 095, India. Correspondence to: Dr. Dheeraj Shah, 7184 Birla Officers Flats, Kamla Nagar, Delhi 110 007, India. E-mail: shahdheeraj@hotmail.com Manuscript received: September 9, 2003; Initial review completed: September 24, 2003; Revision accepted: February 3, 2004. Abstract:
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. Keywords: Prune-belly syndrome, VACTERL association.

47. Prune Belly Syndrome Topic - Unified Search Environment
prune belly syndrome Topic Tree prune belly syndrome MSH/MH/D011535 ICD9CM/PT/756.71 MSH/PM/D011535 MSH/PM/D011535 MSH/EN/D011535
http://www.use.hcn.com.au/portals/shared/subject.`Prune Belly Syndrome`/home.htm
Prune Belly Syndrome Topic Tree Definition:
A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from its characteristic distended abdomen with wrinkled skin. Synonyms and Source Vocabularies:
Prune Belly Syndrome
Eagle-Barrett syndrome Congenital Abnormality

48. Prune Belly Syndrome
Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume IV 2/PRUNE BELLY SYN

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our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Prune belly syndrome, a congenital absence or hypoplasia of the anterior abdominal wall musculature, accompanied by a generalized dilatation of the urinary tract and bilateral cryptorchidism. The condition is also known as Eagle Barrett or triad syndrome. The incidence is 1 in 35,00050,000 live births. Prune belly syndrome occurs almost exclusively in males. The condition occurs sporadically, and the aetiology is unknown. A genetic predisposition has been proposed, and the occurrence of prune belly syndrome in siblings has been reported. Curiously, the condition is commoner in twin pregnancies. All reported twins have been discordant for the syndrome. renal failure Antenatal ultrasound (US) may suggest the diagnosis of prune belly syndrome, when hydroureteronephrosis with a large bladder and flaccid-appearing abdominal wall are detected. However, the prenatal distinction from severe vesicoureteric reflux or posterior urethral valves may be difficult. Postnatally, hydroureteronephrosis and bladder enlargement may be demonstrated by US, CT, MRI, or intravenous urography. Voiding cystourethrography typically shows a large bladder which empties slowly and incompletely. Vesicoureteric reflux is common (

49. Prune Belly Syndrome
Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/PRUNE BELLY SYND

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our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Prune belly syndrome, a triad of features consisting of deficient abdominal wall muscles, urinary tract dilatation and cryptorchidsim. The most recognizable feature of this condition is irregularity of the skin of the abdominal wall with underlying abdominal wall muscle atrophy. The urinary tract manifestations include dilatation of the bladder, hydroureter, hydronephrosis and bladder neck dilatation. The kidneys may be small and dysplastic but stretched around the dilated collecting systems ( Fig.1 The condition may be suspected in utero although the diagnosis is rarely made until post natal life. The bladder may have a prominent superior margin due to a urachal remnant. The ureter is often dysplastic and has a prostatic utricle ( Fig.2 ). The primary imaging investigation is ultrasound ( Fig.3 ) which will demonstrate bladder dilatation, hydroureter and hydronephrosis and absent testes. Micturating cystourethrography demonstrates poor bladder emptying and vesicoureteric reflux. Renal function is variable but may proceed to renal failure even in neonatal life secondary to pulmonary hypoplasia
DWP
The Encyclopaedia of Medical Imaging Volume VII Prune belly syndrome, Fig. 1

50. Article : Images- Prune Belly Sydrome: Antenatal Ultrasound ; Author : GANESHAN
The prune belly syndrome (abdominal muscles deficiency syndrome, Eagle Barretsyndrome) Sonographic observations in three cases of prune belly syndrome,
http://www.ijri.org/articles/archives/20011101/images01.htm
Images: Prune Belly Sydrome: Antenatal Ultrasound GANESHAN S, INDRAJIT IK,
Ind J Radiol Imag 2001; 11:1: 25-28 Key words: Congenital anomalies, Genitourinary system, Ultrasound, prune belly syndrome
The Prune Belly syndrome (abdominal muscles deficiency syndrome, Eagle Barret syndrome) in its complete form is characterized by deficiency of abdominal wall muscles, undescended testis and dilated urinary tract. This syndrome has derived its name from the wrinkled prune like appearance of the abdominal wall [ ]. Sonographic observations in three cases of Prune Belly syndrome, prenatally diagnosed and subsequently confirmed are presented in a pictorial essay. Pathology Prune Belly syndrome (PBS), an uncommon condition, results from failure of the lateral mesoderm to migrate or differentiate into the musculature of the abdominal wall and urinary tract which normally occurs by about the tenth week of fetal life [ ]. Even though associated ureteral anomalies like megaloureter, bulbar or penile urethral diverticulum may occur, classically no evidence of any intrinsic urethral obstruction can be demonstrated [

51. Prune Belly Syndrome - Washington DC
prune belly syndrome Washington Hospital Center is located in Washington DC.
http://www.whcenter.org/14749.cfm
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Prune belly syndrome
Definition: Prune belly syndrome is a group of congenital anomalies characterized by three major findings:
  • Deficient development of abdominal muscles (causing the skin of the abdomen to wrinkle like a prune) Undescended testicle Urinary tract anomalies

Alternative Names: Eagle-Barrett syndrome
Causes, incidence, and risk factors: The underlying causes of prune belly syndrome are unknown. 96-97% of cases are boys. Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid ) that make it likely the infant will have lung problems. (See also Potter syndrome The newborn infant has a wrinkled abdomen that looks like a prune. This is because the abdomen swelled with fluid in the womb, then lost that fluid after birth, leading to wrinkles of excess skin. The appearance is accentuated by the lack of adequate abdominal musculature. 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.

52. Prune Belly Syndrome
prune belly syndrome. Absence of abdominal muscles with urinary tract abnormalityand cryptorchidism. 16 images from 1 to 15 , 1 2 next
http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=243

53. Prune Belly Syndrome
prune belly syndrome. Absence of abdominal muscles with urinary tract abnormalityand cryptorchidism. 16 images from 16 to 16 , previous 1 2
http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?offset=15&cat3=24

54. Log In Problems
Early Comprehensive Surgery Often Effective in prune belly syndrome CME.News Author Laurie Barclay, MD CME Author Désirée Lie, MD, MSEd Disclosures
http://www.medscape.com/viewarticle/492316
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55. Prune Belly Syndrome : Genetic Disorders : Disorders And Diseases : Health And F
prune belly syndrome Network http//www.prunebelly.org/. Devoted to healtheducation, information and support for the person with the condition as well as
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http://www.prunebelly.org/ Devoted to health education, information and support for the person with the condition as well as their friends, families or the health professionals who treat them. Also known as Eagle-Barrett syndrome. More Details Review It Rate It Bookmark It Sponsor Links Love Romance Dating
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56. Rare Pediatric Disease Database
WHAT prune belly syndrome is a rare disease that causes 3 main This disorderwas originally named the prune belly syndrome because infants born with
http://www.madisonsfoundation.org/content/3/1/display.asp?did=506

57. Prune Belly Syndrome 2nd Annual Music Benefit
prune belly syndrome, also known as EagleBarrett Syndrome, The Prune BellySyndrome Network provides support and information to those who have prune
http://www.foreverfalling.net/pbs2004.htm
    PRUNE BELLY SYNDROME NETWORK
    2nd ANNUAL MUSIC BENEFIT (2004)
    Feb 20th / 21st / 22nd
    Prune Belly Syndrome, also known as Eagle-Barrett Syndrome, effects 1 in 40,000 live births. Because it is so rare and uncommon, the media has been slow to respond to it. 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.
    The Prune Belly Syndrome Network
    provides support and information to those who have prune belly syndrome, and to their families, friends and health care professionals.
    Frank Walker (born with Prune Belly Syndrome) has put together a 3-day benefit to raise money for greater awareness of this condition. The first will take place on Feb 20th at Munchaba Lounge (Levittown), the second on Feb 21st at Caps on the River (Seaford), and the final day of the benefit will be Sunday Feb 22nd at the Village Pub South (Amityville).
    To read more about Prune Belly Syndrome, click the following links:
    The Prune Belly Syndrome Network

    Prune Belly Support Forum

    Prune Belly Syndrome Benefit Feb 2003

    Prune Belly Syndrome 2004 Convention
    ... "Not The Only One" CD single now on sale! (Written and arranged by Frank Walker). All proceeds from the CD Single go directly to the

58. Baby Boy Undergoes Complex Operation To Correct Prune Belly Syndrome
About as complex as it gets—that’s how pediatric urologist Andrew Freedman, MD,director of pediatric urology at CedarsSinai Medical Center’s Endourology
http://www.innovations-report.de/html/berichte/medizin_gesundheit/bericht-14102.
Weitere Förderer des Forums Content Partner des Forums Medizin Gesundheit Cedars-Sinai Medical Center Baby boy undergoes complex operation to correct Prune Belly Syndrome
About as complex as it gets—that’s how pediatric urologist Andrew Freedman, M.D., director of pediatric urology at Cedars-Sinai Medical Center’s Endourology Institute, describes the surgery he performed this summer on then 17- month-old Jalen Brown, born with Prune Belly Syndrome. That surgery required reconstructing the toddler’s urinary system in a nearly 10-hour procedure.
Prune Belly Syndrome, also known as Eagle-Barrett Syndrome, is a very rare occurrence: just one in 80,000 births. An estimated 99 percent of those affected are boys. The cause is still unknown, though some theories suggest urinary tract obstructions and resulting distention or developmental defects in utero.
Like Jalen, most born with Prune Belly Syndrome exhibit three main features: 1) an absence of abdominal muscles, contributing to a “wrinkled” appearance of the skin and a “pot belly” profile, 2) dilated urinary tract and associated problems, and 3) intra-abdominal (undescended) testicles.
These trademark characteristics are typically discovered either prenatally via ultrasound or at birth. Associated medical problems—primarily related to renal function—can be life-threatening. “We weren’t sure he was going to make it,” says Dr. Freedman, citing concerns at birth. For Jalen—and most others with the syndrome—survival depends on medical intervention.

59. Can A "prune Belly" Have A Successful Kidney Transplant?
The prune belly syndrome does not exclude the possibility of successful kidney The first kidney transplant in a patient with prune belly syndrome was
http://www.transweb.org/qa/asktw/answers/answers9509/kidneytransplfor.html
Can a "prune belly" have a successful kidney transplant?
Question was submitted by: David Tyburski on 9/7/95.
Question:
Is it possible for a prune belly to have a successful kidney
transplant? My nephew has to start dialysis and has been told there has
never been a successful prune belly transplant. Is this true? Can you suggest anyone
he could see?
Answer:
The prune belly syndrome is a congenital abnormality that consists of deficient abdominal wall muscles, urinary tract abnormalities, and undescended testicles. The prune belly syndrome does not exclude the possibility of successful kidney transplantation. The first kidney transplant in a patient with prune belly syndrome was reported in 1977 (Schenasky and Whelchel, Journal of Urology, Jan 1976, p112). You should recommend that your nephew's parents seek a referal to a center with experience with pediatric kidney transplantation. UNOS can provide the names of centers that perform pediatric kidney transplants in your area. Jeff Punch, M.D. (transplant surgeon), University of Michigan
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60. Prune Belly (Eagle-Barrett) Syndrome
prune belly syndrome, also known as EagleBarrett or triad syndrome, is a distinctive prune belly syndrome occurs in 1 in 40000 live births in the US
http://www.malattiemetaboliche.it/articoli/Prune_ Belly_syndrome.htm
Prune Belly (Eagle-Barrett) Syndrome Set of distinctive physical problems What is it?
Prune Belly syndrome, also known as Eagle-Barrett or triad syndrome, is a distinctive set of physical problems that a child is born with. These problems are:
  • The abdominal muscles in the front are weak or missing
  • Parts of the urinary tract develop abnormally
  • In males (95% of cases), the testes stay up inside the abdomen and don't come down into the scrotum.
There may be other physical problems in the infant, such as spinal curvature, hip dislocations, clubfoot, respiratory or heart problems, and gastrointestinal problems. What causes it?
The cause of the syndrome is unknown, but researchers believe that disruption of the growth of the fetus causes the problems to develop. It is thought that something blocks a part of the fetus' urinary tract, and this causes other parts of the tract to develop abnormally. Prune Belly syndrome occurs in 1 in 40,000 live births in the U.S. How is it diagnosed?

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