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What is Prune Belly Syndrome (PBS)?
Prune belly syndrome is characterized by wrinkling of skin over the abdominal sphere giving it the typical “ prune - like ” show . This is stimulate by personnel casualty of muscleman strength over the abdominal sphere . Let us interpret about the symptoms , crusade , diagnosis and handling of prune belly syndrome in particular .
Prune belly syndrome or PBS , ( also recognise asEagle - Barrett Syndromeor Triad syndrome ) is a triad of abnormality as list below :
This condition is more common among male person than female and the severity of the symptom can change from one someone to another . In some cases , Prune abdomen syndrome can lead to severe kidney and lung issues often result in stillbirth . In other cases , Prune belly syndrome may be limited to just urological issues .
Causes of Prune Belly Syndrome
The genuine campaign of Prune abdomen syndrome are still unnamed . bailiwick are being conducted to understand the underlying causes of this stipulation and amount up with better treatment plan . At present it is believe that prune abdomen syndrome is associated with genetic issues . It occurs in close association with trisomy 21 or Down syndrome and trisomy 18 . Cases have been reported with mutation in CHRM3 gene.1Some researchers believe the condition may be do due to blockage in the urinary tract get the urine to run back into the urinary vesica and thus enlarging the bladder . Some theories also hint that the urinary issues may be cause due to underdevelopment of the vesica .
Symptoms Associated with Prune Belly Syndrome
As mention above , the symptoms of Prune abdomen syndrome change from one individual to other . The extent of the syndrome varies from modest to severe . The most commonly notice symptom are as discussed below :
There may be other symptom which may resemble other medical term . It is very important to confer with a specialist to secern related to symptoms from unrelated symptom .
Diagnosis of Prune Belly Syndrome
As figure with other rare genetic diseases , the diagnosing of prune belly syndrome can be very challenging . In most of the cases , the diagnosis begins with taking patient ’s aesculapian story , existing symptom , physical testing followed by some laboratory testing . Testing resource known as GTR or Genetic Testing Registry provides us all the necessary data required for genetic examination of this condition . Detecting renal anomalies in the other stagecoach during antenatal check - ups can help oneself to leave seasonable treatment and tighten complications of PBS.2
A great turn of casing are detected through foetal ultrasound when the adult female is fraught . Most of the cases are diagnosed as shortly as the tike is born base on the bearing of the triad symptoms .
Other diagnostic tests that help to confirm the diagnosing of Prune belly syndrome let in :
Renal Ultra - Sonography . It is a specialized symptomatic imagination subprogram that uses gamy frequency sound waves to produce paradigm of harmonium , vessel and tissue with the helper of a reckoner .
VCUG ( Voiding Cystourethrogram ) . It is a extremely specific cristal - beam that takes images of the urinary tract . The procedure is carry out by placing a hollow tube into the urethra while fill up a dyestuff into the bladder . As the vesica occupy up or empties , x - rayimages are catch . This technique is used to discover any inverse flowing of urine into kidneys or the ureters .
IVP ( Intravenous Pyelogram ) . This is another highly specialized imagination proficiency that is used to view the urinary tract using X - ray . In this technique , an endovenous line dyestuff is inject such that the social organization is visualized on a movie . This technique also reveals the way of life and rate of the urine run through the tract .
Blood trial . Blood tryout are used to assess kidney functions , which give an idea of the live condition and help to contrive appropriate discourse .
Treatment of Prune Belly Syndrome
Treatment of Prune abdomen syndrome depends on the severity of the symptoms . Other agent that influence the discourse plan are as listed below :
In mild case of Prune belly syndrome , the patient is often sustain on precautional antibiotic therapy to prevent infection . In other case , the affected role may be referred to an urologist . Some cases of Prune venter syndrome may require vesicostomy , where a humble possibility is made through the stomach into the vesica . This piss it well-heeled to empty to the vesica . In other severe cases , broad surgical remodeling of the abdominal paries and urinary tract may be call for . Males may involve to undergo a function called as orchiopexy , where the testes is pass on into the scrotum .
Prognosis of Prune Belly Syndrome
The prognosis of Prune stomach syndrome depends on the severity of the symptoms and the extent to which the condition has move on . Some stirred child are still born while some may not live for too long after parturition . child with mild syndrome can grow and lead a fairly normal life , provide they are monitored by a medico on a steady basis . It is not rare for these child to have aesculapian problems through - out their life . It has been noted that about 30 % of the moved individuals develop kidney failure and may require kidney transplantation in future .
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