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Prune belly syndrome

Prune belly syndrome is a rare birth defect affecting about 1 in 40,000 births. About 96% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, characterized by a triad of symptoms. more...

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The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the abdomens of those with the disorder. Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett Syndrome, and Obrinsky Syndrome.

Symptoms

  • A partial or complete lack of abdominal muscles. There may be wrinkly folds of skin covering the abdomen.
  • Undescended testicles in males
  • Urinary tract abnormality such as unusually large ureters, distended bladder, accumulation and backflow of urine from the bladder to the ureters and the kidneys

Diagnosis

Prune belly syndrome is often diagnosed via ultrasound while a child is still in-utero. An abnormally large abdominal mass is the key indicator, as the abdomen swells with the pressure of accumulated urine. In young children, frequent urinary tract infections often herald prune belly syndrome, as they are normally uncommon. If a problem is suspected, doctors can perform blood tests to check kidney function. Another test that may reveal the syndrome is the "voiding cystourethrogram," in which a catheter is inserted into the urethra to fill the bladder with dye. An X-ray can detect if urine has a reverse flow into the ureters and kidneys (see example x-ray teaching file). Prune belly syndrome can also result in the distending and enlarging of internal organs such as the bladder and intestines. Surgery is often required to return these organs to their natural sizes.

A genetic predisposition has been suggested, and PBS is much more common when the baby is a twin, all though all reported twin births have been discordant.

Treatment

The type of treatment, like that of most disorders, depends on the severity of the symptoms. One option is to perform a vesicostomy, which allows the bladder to drain through a small hole in the abdomen. A more drastic procedure is a surgical "remodeling" of the abdominal wall and urinary tract. Boys may have an orchiopexy, which moves the testicles to their proper place in the scrotum.

Less intense cases can be delt with via multipul daily cathaderizations.

Even with treatment, many patients experience renal failure.

Read more at Wikipedia.org


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The County Lines
From Spokesman Review, The (Spokane), 10/30/99

WASHINGTON

Adams County

Othello

Residents have eaten a mountain of pizza so children at Lutacaga Elementary could fly high. The local PTA sold pizzas to fund a new six-seat swing set for the kids.

Ferry County

Republic

Local merchants are again sponsoring Hometown Holiday drawings. By shopping in Republic from Monday through Dec. 24, folks can earn tickets for "Gold Certificate" drawings. Each Gold Certificate is good for $50 when shopping in Republic.

Grant County

Ephrata

Local resident Jennifer L. Stewart, 57, was found to have about 40 animals living at her home. Police confiscated 30 dogs, five cats, three ferrets, four birds, a rabbit and a prairie dog and took them to the Grant County Humane Society. A city ordinance prohibits residents from owning more than three animals per household and from selling animals for profit.

Lincoln County

Almira

Volunteer work by the Almira Action Council has gotten many needed projects off the ground. Two bridges have been painted, though the bridge known locally as the "bowed" bridge is nearly falling down. A volunteer said the timbers are suffering from dry rot and that after she pounded nails back into the wood and she walked across the bridge, the nails popped back out. The city is looking into the matter.

Pend Oreille County

Newport

Don't expect trick-or-treaters tonight. Despite some communities changing Halloween festivities to Oct. 30, the local City Council decided the gathering of candy should remain on Oct. 31.

Spokane County

Spokane

The Emerson/Garfield Neighborhood Council will hold a special neighborhood design meeting at the Corbin Senior Center on Tuesday at 827 W. Cleveland at 7 p.m. Officials from WSU and SIRTI will be presenting design concepts. For more information, contact Jay Cousins at 328-8680.

Stevens County

Colville

The Colville KIPS gymnastics club is looking for a permanent home after being displaced from the Armory last spring. The state deemed the Armory unsafe as it is located in an airport zone. Until a new gym can be found, $70,000 of gymnastics equipment sits idle, along with scores of young tumblers.

IDAHO

Benewah County

Fernwood

The annual Tri-Community Library craft show and sale will be Nov. 10 in the CAF building, from 10 a.m. to 4 p.m. Tables can be reserved for $10 by calling Joanne O'Dwyer at (208) 245-4883. Those renting tables are asked to donate an item for the hourly raffle. Proceeds will go to the library for improvements.

Bonner County

Sandpoint

Video footage of the Schweitzer Snowmobile Hillclimb will be featured at the Kamloops Bar and Grill Nov. 13. Proceeds from the event will help pay Cody Walker's medical expenses. The 6-month-old boy suffers from Prune Belly Syndrome which prevents a person's ureter, bladder, kidneys and stomach from functioning properly.

Boundary County

Bonners Ferry

The Country Women's Group will award a handmade quilt during the Nov. 20 craft bazaar. Tickets are available for $1 each or six for $5 from members of the group. Proceeds will benefit the restoration of the West Fork Cabin at Smith Creek.

Kootenai County

Coeur d'Alene

The Dalton Country Cousins 4-H members met with residents of Pinewood Care Center on Oct. 23. The members planted a tree at the Dalton Gardens horse arena and carved pumpkins with senior residents. The tree-planting is the second of three projects planned for this year. The group installed bird feeders and a birdbath and built birdhouses with senior residents in June.

Latah County

Moscow

The Humane Society of the Palouse has 20 dogs on the waiting list to get adopted. If the waiting list gets too long or the kennels are full, the shelter will be forced to euthanize some of the animals. The Humane Society has put 120 cats and dogs to sleep this year. In 1998, 800 dogs and 1,200 cats were sheltered.

Shoshone County

Kellogg

Sunnyside Elementary School Principal Steve Shepperd has been named a runner-up for the Idaho State Principal of the Year award. Shepperd was one of six principals nominated by staff, administrators and parents of the respective school districts. Debbie Toy, a principal in the Boise School District, was named principal of the year.

MONTANA

Lincoln County

Libby

St. John's Lutheran Hospital's Outpatient Chemotherapy unit is trying a new approach to assisting cancer patients. The unit is working to provide a supply of various types of wigs and hairpieces for patients to use. When no longer needed, they can be returned, sanitized and used again by another patient. Turbans and hats also would be available through the service.

Copyright 1999 Cowles Publishing Company
Provided by ProQuest Information and Learning Company. All rights Reserved.

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