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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

81. PillSupplier.com - Conditions And Diseases/Genetic Disorders/Prune Belly Syndrom
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82. Genitourinary And Kidney Disorders - Prune Belly Syndrome
Find a Physician, prune belly syndrome What is prune belly syndrome? prune belly syndrome is also known as triad syndrome or EagleBarrett syndrome.
http://www.mccg.org/childrenshealth/urology/pbs.asp

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Prune Belly Syndrome 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:

83. Prune Belly Syndrome
Tips for printing. Abdominal Muscles Prune Belly Syndrome. Special Resources SOS Ask experts or consultants for information prune belly syndrome.
http://ibis-birthdefects.org/start/prunebel.htm
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... Etchings Please Explore: Support Groups Professional Associations Key Information Sources Topics include Titles and Abdominal Muscle Deficiency A. Abdominal Muscle Deficiency S. Abdominal Musculature Aplasia S. Absence of Abdominal Muscle S. Aplastic Abdominal Muscle S. Congenital Absence of the Abdominal Muscles Defective Abdominal Wall S. Eagle - Barrett S. Fröhlich S. Obrinsky S.
Special Resources Prune Belly Syndrome
A Selection of Internet Sites [*] Outstanding [P] For Professionals [S] Support Group [Portuguese] [*] [P] ABDOMINAL MUSCLES, ABSENCE OF, WITH URINARY TRACT ABNORMALITY AND CRYPTORCHIDISM Alternative titles; symbols PRUNE BELLY SYNDROME from OMIM 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 ). ( Osler did not use the term "prune belly" ... "In the summer of 1897 a case of remarkable distension of the abdomen was admitted to the wards, with greatly distended bladder, and on my return in September, Dr. Futcher, knowing that I would be interested in it, sent for the child".) The full syndrome probably occurs only in males ... Multiple cases (of the full syndrome) in families have rarely been reported, ... In British Columbia, Baird and MacDonald (1981) found a frequency of 1 in 29,231 live births ... similar to Poland syndrome (173800) in being rather consistently reproduced in many cases but having no clearly demonstrable mendelian basis ...

84. Genitourinary And Kidney Disorders - Prune Belly Syndrome
Print Version. Genitourinary and Kidney Disorders prune belly syndrome. What is prune belly syndrome? What causes prune belly syndrome?
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Genitourinary and Kidney Disorders
Prune Belly Syndrome
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:
  • absence of abdominal muscles
  • undescended testicles - a condition seen in newborns whereby one (or both) of the male testes has not passed down into the scrotal sac
  • an abnormal, expanded bladder and problems in the upper urinary tract, which may include the bladder, ureters, and kidneys
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.

85. Virtual Children's Hospital: Paediapaedia: Prune-Belly Syndrome (Eagle-Barrett S
Paediapaedia Genitourinary Diseases. prunebelly syndrome (Eagle-Barrett syndrome) Michael P. D'Alessandro, M.D. Peer Review Status Internally Peer ReviewedClinical Presentation http//www.vh.org/pediatric/provider/ radiology/PAP/GUDiseases/prune.html
http://www.vh.org/Providers/TeachingFiles/PAP/GUDiseases/Prune.html
Paediapaedia: Genitourinary Diseases
Prune-Belly Syndrome (Eagle-Barrett Syndrome)
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed Clinical Presentation:
Triad of deficient or absent abdominal musculature, undescended testicles, and dysplasia of the urinary tract. Physical exam shows a wrinkled anterior abdominal wall. Etiology/Pathophysiology:
Etiology is unknown.Twenty percent are still born or die as neonates, and an additional 50% die in the first 2 years of life. Other associated anomalies include hip dislocations / club foot / limb deformities (40% ), cardiac defects(30% ), and malrotation (30% ). Pathology:
Aplasia of the muscles of the lower medial abdominal wall. The kidneys have a decreased number of functioning nephrons with variable cystic dysplasia. The dilated ureters and bladder have a patchy absence of smooth muscle. Imaging Findings:
The ureters and bladder are dilated. Urethral abnormalities that can be seen include posterior urethral dilation, diverticula, stenosis / atresia, and megaurethra. They can have umbilical-bladder attachment or a patent urachus. DDX:
  • Posterior urethral valves
References:
See References Chapter.

86. TheFetus.net - Prune-belly Syndrome -Keith A. Aqua, MD, Charles M. McCurdy, Jr.,
199402-03-21 prune-belly syndrome © Aqua www.TheFetus.net. prune-belly syndrome. MESH prune-belly syndrome ICD9 756.7 BDE 2007 CDC 756.720 MIM 100100 POS 3003.
http://www.thefetus.net/page.php?id=557

87. Virtual Children's Hospital: Paediapaedia: Prune-Belly Syndrome (Eagle-Barrett S
Paediapaedia Genitourinary Diseases prunebelly syndrome (Eagle-Barrett syndrome). Michael P. D Alessandro, MD Peer Review Status Internally Peer Reviewed
http://www.vh.org/pediatric/provider/radiology/PAP/GUDiseases/Prune.html
Paediapaedia: Genitourinary Diseases
Prune-Belly Syndrome (Eagle-Barrett Syndrome)
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed Clinical Presentation:
Triad of deficient or absent abdominal musculature, undescended testicles, and dysplasia of the urinary tract. Physical exam shows a wrinkled anterior abdominal wall. Etiology/Pathophysiology:
Etiology is unknown.Twenty percent are still born or die as neonates, and an additional 50% die in the first 2 years of life. Other associated anomalies include hip dislocations / club foot / limb deformities (40% ), cardiac defects(30% ), and malrotation (30% ). Pathology:
Aplasia of the muscles of the lower medial abdominal wall. The kidneys have a decreased number of functioning nephrons with variable cystic dysplasia. The dilated ureters and bladder have a patchy absence of smooth muscle. Imaging Findings:
The ureters and bladder are dilated. Urethral abnormalities that can be seen include posterior urethral dilation, diverticula, stenosis / atresia, and megaurethra. They can have umbilical-bladder attachment or a patent urachus. DDX:
  • Posterior urethral valves
References:
See References Chapter.

88. Health Information At Your Fingertips - Patient UK
prunebelly syndrome Contact Group - Patient UK. A directory of provide patient information. prune-belly syndrome Contact Group.
http://www.patient.co.uk/showdoc.asp?doc=26739570

89. Prune-Belly Syndrome With Congenital Cystic Adenomatoid Malformation - Eurorad -
teaching files,radiology,prunebelly syndrome with congenital cystic adenomatoid malformation,Newborn from healthy mother (gestational age 39 weeks 1 day) with
http://www.eurorad.org/case.cfm?uid=933

90. Eagle Barrett Syndrome (Also Known As Prune-Belly Syndrome)
From Applied Radiology. Radiological Case of the Month. Eagle Barrett syndrome (Also Known as prunebelly syndrome PBS) in an Adult.
http://www.medscape.com/viewarticle/475287

91. Prune Belly, Syndrome : Arborescences MeSH
Translate this page prune belly, syndrome arborescences MeSH. Menu général CISMeF. Vous pouvez aussi consulter toutes les arborescences des mots
http://www.chu-rouen.fr/navimesh/P/naviprunebellysyndrome.html
Prune Belly, syndrome : arborescences MeSH Menu général CISMeF Vous pouvez aussi consulter toutes les arborescences des mots clés utilisés dans CISMeF

92. ORPHANET - Maladies Rares - Médicaments Orphelins
prune belly syndrome, Maladie(s) incluse(s) Valve de l urètre postérieur, CIM Q79.4, Aucun descriptif disponible,
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=2970

93. ORPHANET® Prune Belly Syndrome
prune belly syndrome. Accès direct aux détails Alias Valve de l urètre postérieur.
http://www.orpha.net/static/FR/prunebellysyndrome.html

94. Prune Belly (syndrome De)

http://www.vulgaris-medical.net/textp/prunbell.html
Prune Belly (syndrome de) En anglais : Prune Belly syndrome, PBS. Synonyme : obstruction urétrale (séquence d'), Eagle-Barrett valve (syndrome d'), l'urètre postérieur (maladie de), ventre de pruneau. Pathologie concernant environ 40.000 naissances dont 95% des cas touche le sexe masculin et dont environ 20 % meurent au commencement de la vie Causes
Au cours de la troisième semaine, un troisième feuillet, le chordomésoblaste (appelé aussi mésoderme) vient se glisser entre l'ectoderme et l'endoderme.
Ce sont ces trois feuillets qui sont à l'origine des organes et de l'ensemble des systèmes qui vont permettre à l'organisme humain de fonctionner normalement (voir correspondance entre ces différents feuillets et les organes du corps). Progressivement, l'embryon est animé de flexions et de plicatures (plicature embryonnaire) dans le sens de sa longueur et de sa largeur (transversal). Le chordomésoblaste va donner un tube placé dans l'axe vertical (axe crâniocaudal : de la tête vers la queue) comportant de part et d'autre l'extrémité céphalique et l'extrémité caudale.
Autour de ce tube s'organisent d'autres tissus appelés les somites, à partir desquels se développeront les cartilages et les muscles. A la fin de la troisième semaine, les capillaires (micro-vaisseaux) sont présents : ils commencent à drainer dans le cordon ombilical par l'intermédiaire de l'artère et de la veine ombilicales.

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