"Prune belly syndrome"

A case of urethral hypoplasia in prune belly syndrome successfully managed with vesicoamniotic shunts and a P.A.D.U.A. procedure. Urethral atresia is a rare but clinically significant cause of congenital lower urinary tract obstruction. Initial management options include urinary diversion until definitive urethral reconstruction or progressive urethral dilation. Given the overall rarity

Bladder Cancer in a Long-term Survivor of the Prune Belly Syndrome. Prune belly syndrome (PBS) is a rare but morbid complexity of congenital anomalies that consists of abdominal wall defect, bilateral cryptorchidism, and urinary tract dilation. With the recent advances in perinatal care, the life expectancy of patients with PBS has improved. Although renal and sexual outcomes are frequently

Medium and Long-Term Clinical Outcomes of Kidney Transplantation in Patients With Prune Belly Syndrome: A Single-Centre Experience. To present the medium and long-term outcomes of kidney transplantation performed in patients with Prune Belly Syndrome (PBS) which is one of the congenital anomalies of the kidney and urinary tract (CAKUT). Thirteen kidney transplantations were performed in nine

Study of Penis Structure and Development in Fetus With Prune Belly Syndrome. To evaluate the influence of prune belly syndrome (PBS) on the development of penises in human fetuses. We studied 39 human fetuses (including 4 with PBS) aged 11-22 weeks post conception. We measured the length and width of the free portion and penis root and the penis total length (PTL). In 21 fetuses of the control

Prune belly syndrome in surviving males can be caused by Hemizygous missense mutations in the X-linked Filamin A gene. Prune belly syndrome (PBS) is a rare, multi-system congenital myopathy primarily affecting males that is poorly described genetically. Phenotypically, its morbidity spans from mild to lethal, however, all isolated PBS cases manifest three cardinal pathological features: 1

GONADAL FUNCTION AND REPRODUCTIVE SYSTEM ANATOMY IN POST- PUBERTAL PRUNE-BELLY SYNDROME PATIENTS. To gain insight into the causes of infertility in Prune Belly Syndrome (PBS) by evaluating reproductive system anatomy and gonadal function in a cohort of postpubertal PBS patients. We contacted all PBS patients 14 years old or older treated and followed at our institution. Age at orchiopexy, type

Prune Belly Syndrome We value your privacyWe and our partners store and/or access information on a device, such as cookies and process personal data, such as unique identifiers and standard information sent by a device for personalised ads and content, ad and content measurement, and audience insights, as well as to develop and improve products. With your permission we and our partners may use obstruction is made, it may be possible to perform intrauterine surgery to prevent the development of prune belly syndrome.[10] The results seem promising.[11, 12]Join our weekly wellness digestfrom the best health experts in the businessEnter your email Join nowBy clicking ‘Join now’ you agree to our Terms and conditions and Privacy policy.FURTHER READING AND REFERENCESBaird PA, MacDonald EC

Long term follow-up in a patient with prune-belly syndrome - a care compliant case report. Malformative uropathies represent a major cause of Chronic Kidney Disease (CKD) in children. Genitourinary system is the most frequent and sever affected in Prune-Belly syndrome cases. That is why the findings of early diagnosis and vigilant monitoring for these situations remain a major challenge for the medical team. We present the clinical course of a 10 years old child with diagnosis of Prune-Belly syndrome. A urinary tract abnormality was suspected starting 25 weeks of gestation, when a routine ultrasound showed oligohydramnios, increased size urinary bladder, bilateral hydronephrosis and megaureters, thin abdominal wall. Prenatal suspicion of Prune-Belly syndrome plays a deciding role in renal

Study of Prostate Growth in Prune Belly Syndrome and Anencephalic Fetuses. To compare the growth of the prostate in anencephalic, prune belly syndrome (PBS) and control fetuses. We studied 35 prostates from normal human fetuses aged 11-22 weeks postconception (WPC); 15 from anencephalic fetuses aged 13-19 WPC; and 6 from PBS fetuses aged 13-31WPC. After prostate dissection, we evaluated

Phenotypic Severity Scoring System and Categorization for Prune Belly Syndrome: Application to a Pilot Cohort of 50 Living Patients. To design a novel system of scoring prune belly syndrome (PBS) phenotypic severity at any presenting age and apply it to a large pilot cohort. From 2000 to 2017, patients with PBS were recruited to our prospective PBS study and medical records were cross

Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center Review outcomes of Prune Belly Syndrome (PBS) with the hypothesis that contemporary management improves mortality. A retrospective chart review of inpatient and outpatient PBS patients referred between 2000 and 2018 was conducted to assess outcomes at our institution. Data

Exploration of the fetus with gross anomaly: a case of pseudo prune belly syndrome Prune belly syndrome is a rare congenital anomaly usually presented with triad characteristic feature of deficient abdominal muscles, cryptorchidism, and urinary tract anomalies. Here, we present a case with all the characteristic features of prune belly and the associated secondary features which were observed

Living-related kidney transplantation with catheterizable urinary conduit in prune belly syndrome: A case report Prune belly syndrome (PBS) presents with both renal dysplasia and urinary tract abnormalities. When performing kidney transplantation in PBS patients with kidney failure, extensive pretransplant urinary tract preparation may be necessary. We report the case of a 36-year-old man

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry. As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association

Combined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome. Prune belly syndrome (PBS) is a rare congenital disorder among adults, and the way for repairing abdominal wall musculature has no unified standard. We described combining laparoscopic and open technique in an adult male who presented with PBS. Physical examination

Unusual presentation of prune belly syndrome: a case report Prune belly syndrome is a rare congenital malformation of unknown etiology, with the following triad of findings: abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. In most cases, detection of prune belly syndrome occurs during neonatal or infancy period. In this case report, we describe a 12-year-old boy from Ethiopia with the triad of findings of prune belly syndrome along with skeletal malformations. We are unaware of any previous report of prune belly syndrome in Ethiopia. A 12-year-old Amhara boy from the Northwest Gondar Amhara regional state presented to our referral hospital with a complaint of swelling over his left flank for the past 3 months. Maternal pregnancy course and medical

Study of Testicular Structure in Fetuses with Prune Belly Syndrome To compare the structure of the testis in fetuses with prune belly syndrome (PBS) to normal controls. We studied 6 testes obtained from 3 fetuses with PBS and 14 testes from 7 male fetuses. The testicular specimens were cut into 5-m thick sections and stained with hematoxylin and eosin (HE), to observe the seminiferous tubules

Pseudo Prune Belly Syndrome: Diagnosis Revealed by Imaging – A Case Report and Brief Review Prune Belly Syndrome (PBS) is a rare entity, usually found in male neonates. It comprises complex urinary tract anomalies, bilateral undescended testis and absence of anterior abdominal wall muscles. Patients with unilateral abdominal wall deficiency, unilateral undescended testis and female neonates with abdominal wall laxity are classified as Pseudo Prune Belly syndrome (PPBS). Reports on PPBS do not highlight the radiological and imaging characteristics of this syndrome and the current literature on the role of newer imaging modalities, such as Magnetic Resonance Imaging (MRI), remains relatively sparse. We describe a new case of PPBS and emphasize the role of imaging, especially ultrasound and MRI

Quadratus lumborum block for post-operative pain relief in patient with Prune belly syndrome Abdominal field blocks are commonly used as part of multimodal analgesia for post-operative pain relief in patients undergoing abdominal surgery. Conventionally, transversus abdominis plane block is used, but has the disadvantage of limited spread only to T10-T12 segments, providing only partial pain relief. The new quadratus lumborum (QL) block has the advantage of providing wider sensory block from T6 to L1 and thus has an evolving role in opioid-free anaesthesia. Opioid-induced cough depression, urinary retention, and drowsiness can be problematic in patients with Prune belly syndrome, who have deficient abdominal muscles and myriad of genitourinary problems. We report a case of a young male

Type V Pouch Colon, Prune Belly Syndrome, and Congenital Anterior Urethrocutaneous Fistula Congenital pouch colon (CPC) or short colon syndrome is a rare type of anorectal malformation(ARM). Type V is the rarest form of CPC. We present a 1-day-old male child with type V CPC with prune belly syndrome and congenital anterior urethrocutaneous fistula (CAUF).