prune belly syndrome

プルーンベリー症候群

WordNet

1. the shape of a bell (同)bell_shape, campana
2. (nautical) each of the eight half-hour units of nautical time signaled by strokes of a ships bell; eight bells signals 4:00, 8:00, or 12:00 oclock, either a.m. or p.m. (同)ship''s bell
3. attach a bell to; "bell cows"
4. a hollow device made of metal that makes a ringing sound when struck
5. the flared opening of a tubular device
6. the sound of a bell being struck; "saved by the bell"; "she heard the distant toll of church bells" (同)toll
7. swell out or bulge out (同)belly out
8. a part that bulges deeply; "the belly of a sail"
9. the underpart of the body of certain vertebrates such as snakes or fish
10. a protruding abdomen (同)paunch
11. the hollow inside of something; "in the belly of the ship"
12. a pattern of symptoms indicative of some disease
13. a complex of concurrent things; "every word has a syndrome of meanings"
14. dried plum
15. the act of trimming a plant
16. something that has been pruned off of a plant

PrepTutorEJDIC

1. 『鐘』,釣り鐘;『鈴』,『ベル』,りん / 〈U〉鐘(鈴,ベル)の音 / 〈C〉鐘(ベル)状のもの / 〈C〉点鐘(船の当直交替を0時から4時間ごとに区切り,その間を30分ごとに1‐8点鐘で時を報じる.例えば2つ鳴らすことを2点鐘といい,午前または午後の1時,5時,9時を指す.8点鐘は当直交替の時間) / …‘に'鈴を付ける,‘に'ベルを取り付ける / (鈴・鐘のように)…'を'ふくらませる
2. 『腹』,腹部(abdomen) / 胃 / (物の)内側,内部(inside) / ふくれた部分,胴 / 前面[部],下面[部] / …'を'ふくらませる　ふくらむ
3. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
4. (草木から)…‘を'切り取る / 〈草木〉‘を'刈り込む / (不要なものを)…‘から'除く
5. 干しスモモ / 《英話》間抜け
6. (庭木などの)刈り込み,剪定(せんてい),枝打ち

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/06/02 09:01:48」(JST)

wiki en

[Wiki en表示]

Prune belly syndrome
Classification and external resources
Prune belly syndrome in an Egyptian child with Down syndrome.
ICD-10	Q79.4
ICD-9	756.71
OMIM	100100
DiseasesDB	31089
MedlinePlus	001269
eMedicine	med/3055 radio/575
MeSH	D011535

Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 40,000 births. About 97% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, 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 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, Obrinsky Syndrome, Fröhlich Syndrome, or rarely, Triad 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
• Frequent urinary tract infections due to the inability to properly expel urine.
• Later in life, a common symptom is post-ejaculatory discomfort. Most likely a bladder spasm, it lasts about two hours.

Diagnosis

Prune belly syndrome can be diagnosed via ultrasound while a child is still in-utero. An abnormally large abdominal cavity resembling that of an obese person 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.

A genetic predisposition has been suggested, and PBS is much more common in males. discordant.

Complications

Prune belly syndrome can result in the distending and enlarging 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. Also many complications can come from enlarged/malformed kidneys which warrant the child to go on dialysis or require a kidney transplant. With proper treatment long healthy lives are possible.

Musculoskeletal abnormalities include pectus excavatum, scoliosis, and congenital dislocations including the hip. Diagnosis of prune belly syndrome necessitates thorough orthopaedic evaluation because of the high prevalence of associated musculoskeletal abnormalities.

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, 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 may have an orchiopexy, which moves the testicles to their proper place in the scrotum.

Even with treatment, many patients experience renal failure.

References

External links

• http://jbjs.org/article.aspx?articleid=22890
• http://www.prunebelly.org what-is-pbs from the Prune Belly Syndrome Network or call 855-ASK-PBSN
• Marty Sheedy, A motivational speaker and activist with Arthrogryposis and Prune Belly Syndrome.
• Redditor with PBS Reddit AMA (Ask Me Anything)

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. プルーンベリー症候群 prune belly syndrome
• 2. 小児における鼠径部腫脹の評価 evaluation of inguinal swelling in children
• 3. 先天性腎尿路異常（CAKUT）の評価 evaluation of congenital anomalies of the kidney and urinary tract cakut
• 4. 小児における鼡径ヘルニアの概要 overview of inguinal hernia in children
• 5. 小児における停留精巣：臨床的特徴および評価 undescended testes cryptorchidism in children clinical features and evaluation

English Journal

• Feasibility and early outcomes of robotic-assisted laparoscopic Mitrofanoff appendicovesicostomy in patients with prune belly syndrome.

• Wille MA, Jayram G, Gundeti MS.SourcePediatric Urology, Section of Urology, Comer Children's Hospital, The University of Chicago Pritzker School of Medicine, Chicago, IL, USA.
• BJU international.BJU Int.2012 Jan;109(1):125-9. doi: 10.1111/j.1464-410X.2011.10317.x. Epub 2011 Jul 28.
• Study Type - Therapy (case series) Level of Evidence 4 What's known on the subject and What does the study add There is a single case reported in the literature describing this approach in prune belly syndrome. To our knowledge there are two case reports and two series in which laparoscopic appe
• PMID 21794067

• Genetic Basis of Prune Belly Syndrome: Screening for HNF1β Gene.

• Granberg CF, Harrison SM, Dajusta D, Zhang S, Hajarnis S, Igarashi P, Baker LA.SourceDepartment of Urology, University of Texas Southwestern, Dallas, Texas.
• The Journal of urology.J Urol.2012 Jan;187(1):272-8. Epub 2011 Nov 23.
• PURPOSE: Although the cause of prune belly syndrome is unknown, familial evidence suggests a genetic component. Recently 2 nonfamilial cases of prune belly syndrome with chromosome 17q12 deletions encompassing the HNF1β gene have made this a candidate gene for prune belly syndrome. To date, there h
• PMID 22114815

Japanese Journal

• 症例報告 十二指腸前門脈の合併による総胆管拡張を示したプルーンベリー症候群の1例

• 小幡 聡,木下 義晶,川久保 尚徳 [他]
• 日本小児外科学会雑誌 48(6), 926-930, 2012-10
• NAID 40019466091

• オープンシステムを利用して全身麻酔下での歯科治療を行った prune belly 症候群の1例

• 兼松 義典,内藤 克美
• 障害者歯科 32(4), 628-631, 2011-10-31
• NAID 10030300179

Related Links

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If you have stumbled across this site, the chances are that you or someone you know was born with Eagle-Barrett, commonly referred to as prune belly syndrome. Other names for prune belly syndrome include triad syndrome and ...

• Gallery | Prune Belly Syndrome

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Related Pictures

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★リンクテーブル★

リンク元	「プルーンベリー症候群」
関連記事	「belly」「pruning」「bell」「prune」「syndrome」

「プルーンベリー症候群」

　　[★]

英

prune belly syndrome prune-belly syndrome PBS

同

干しスモモ症候群、腹壁無形成症候群 abdominal wallaplasiasyndrome

• 腹壁形成不全、尿路奇形、両側停留精巣を3主徴とする症候群。

「belly」

　　[★]

• 腹、腹部

関

abdomen、abdominal、abdominis、ventral

　 　 　 　 　 　

「pruning」

　　[★]

• n.

• 刈り込み、枝打ち、剪定

関

prune

　 　 　

「bell」

　　[★]

• n.

• ベル、鐘、鈴

　 　 　 　 　 　 　

「prune」

　　[★]

• v.

• 剪定する

関

pruning

　 　 　

「syndrome」

　　[★]

• n.

• 症候群