809 results match your criteria Prune Belly Syndrome


Pfeiffer Syndrome Type 3 and Prune Belly Anomaly in a Female: Case Report and Review.

Fetal Pediatr Pathol 2019 Apr 19:1-6. Epub 2019 Apr 19.

a Center for Registry and Research in Congenital Anomalies (CRIAC), Service of Genetics and Cytogenetics Unit, Pediatrics Division, Dr. Juan I. Menchaca Civil Hospital of Guadalajara , Guadalajara , Jalisco , Mexico.

Background: Pfeiffer syndrome (PS) is an autosomal dominant entity characterized by craniosynostosis, broad thumbs, and preaxially deviated great toes. It is classified in three types depending on the severity. Type 1: Mild to moderate severity, Type 2: Severe presentation with cloverleaf skull, and Type 3: Severe craniosynostosis with prominent ocular proptosis. Read More

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https://www.tandfonline.com/doi/full/10.1080/15513815.2019.1
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http://dx.doi.org/10.1080/15513815.2019.1603256DOI Listing
April 2019
1 Read

LONG-TERM OUTCOME OF RENAL TRANSPLANTATION IN PATIENTS WITH CONGENITAL LOWER URINARY TRACT MALFORMATIONS: A MULTICENTER STUDY.

Transplantation 2019 Apr 4. Epub 2019 Apr 4.

Urology and renal transplantation, Montpellier University hospital, France.

Introduction: Renal insufficiency can occur in patients with congenital lower urinary tract malformations (LUTM) even when managed during infancy. Data in the current literature concerning this subject remain sparse. The aim of this study was to report the feasibility and long-term results of renal transplantation during adulthood in patients with a congenital LUTM. Read More

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http://dx.doi.org/10.1097/TP.0000000000002746DOI Listing
April 2019
1 Read

Prenatal imaging of genital defects: clinical spectrum and predictive factors for severe forms.

BJU Int 2019 Feb 18. Epub 2019 Feb 18.

Unité de Chirurgie Viscérale et Urologique Pédiatrique, Hôpital Lapeyronie - CHU Montpellier, Montpellier, France.

Objectives: To report the clinical spectrum of genital defects diagnosed before birth, identify predictive factors for severe phenotypes at birth, and determine the rate of associated malformations.

Patients And Methods: A retrospective study (2008-2017) of 4580 fetuses, identified prenatally with abnormalities evaluated by our Reference Center for Fetal Medicine, included cases with fetal sonographic findings of abnormal genitalia or uncertainty of fetal sex determination. Familial, prenatal and postnatal data were collected via a standardised questionnaire. Read More

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http://dx.doi.org/10.1111/bju.14714DOI Listing
February 2019
1 Read

An Autopsy Case Report: Prune Belly Syndrome with Overlapping Presentation of Partial Urorectal Septum Malformation Sequence.

Turk Patoloji Derg 2019 Jan 11. Epub 2019 Jan 11.

Department of Obstetrics and Gynecology, Istanbul University, School of Medicine Istanbul, ISTANBUL, TURKEY.

Prune belly syndrome (PBS) is characterized by a classical triad of congenitally absent abdominal muscles, bilateral cryptorchidism, and a malformed urinary tract. Urorectal septum malformation sequence (URSM) is identified with the absence of urogenital and anal openings. This case report describes a 15-week-old female fetus with megacystis, ascites and oligohydramnios in a 19-year-old nulliparous woman. Read More

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http://dx.doi.org/10.5146/tjpath.2018.01440DOI Listing
January 2019
14 Reads

A potential serious complication in infants with congenital obstructive uropathy: Secondary pseudohypoaldosteronism.

J Pak Med Assoc 2019 Jan;69(1):108-112

Department of Neonatology, Erciyes University Medical Faculty, Turkey.

Patients who have secondary pseudohypoaldosteronism (PHA) in addition to hyponatraemia, hyperpotassaemia and high serum aldosterone levels for the age were included in this retrospective study.Among eight patients, seven patients were diagnosed with PHA secondary to obstructive uropathy (OUP), whereas one patient had PHA secondary to ileostomy. Six patients with OUP had simultaneous urinary tract infection (UTI) and in all except one patient, secondary PHA recovered with only UTI treatment before applying surgical correction. Read More

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January 2019
4 Reads

Prune belly syndrome: Approaches to its diagnosis and management.

Intractable Rare Dis Res 2018 Nov;7(4):271-274

Neonatology Department, Maternity and Neonatology Center, Faculty of Medicine, Tunis-El Manar University, Tunis, Tunisia.

Prune Belly syndrome (PBS) or Eagle-Barrett syndrome is an anatomo-radiological syndrome consisting of a complex and rare malformation characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract, and bilateral cryptorchidism. The exact etiology is unknown, though PBS predominantly occurs in males. The clinical manifestations can vary widely, from stillbirth to renal and major respiratory dysplasia to almost normal children. Read More

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https://www.jstage.jst.go.jp/article/irdr/7/4/7_2018.01094/_
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http://dx.doi.org/10.5582/irdr.2018.01094DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290839PMC
November 2018
5 Reads

Abdominell rektusdiastas kan ge funktionella besvär - Indikation för behandling måste förtydligas.

Lakartidningen 2018 11 19;115. Epub 2018 Nov 19.

Norrlands universitetssjukhus - Kirurgcentrum Umeå, Sweden Norrlands universitetssjukhus - Kirurgcentrum Umeå, Sweden.

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November 2018
8 Reads

Transitional Urology.

Urol Clin North Am 2018 Nov 7;45(4):601-610. Epub 2018 Sep 7.

Department of Urology, University College London Hospitals, 16-18 Westmoreland Street, London W1H 6PL, UK.

The field of transitional urology has taken on an increasing importance in recent years as more individuals with congenital urologic issues are living and thriving into adulthood. This article reviews the transitional process itself including barriers to successful transition and the consequences of failing to properly transition. Also provided is a broad overview of the urologic issues faced by patients who may benefit from lifelong care and the providers who will be helping them with transition and assuming their care. Read More

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http://dx.doi.org/10.1016/j.ucl.2018.06.007DOI Listing
November 2018
14 Reads

Exploration of the fetus with gross anomaly: a case of pseudo prune belly syndrome.

Anat Cell Biol 2018 Sep 28;51(3):205-208. Epub 2018 Sep 28.

Department of Anatomy, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India.

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 on detailed dissection and exploration of the fetus. Read More

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http://dx.doi.org/10.5115/acb.2018.51.3.205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6172593PMC
September 2018
7 Reads

Copy number variations in a population with prune belly syndrome.

Am J Med Genet A 2018 Nov 4;176(11):2276-2283. Epub 2018 Oct 4.

University of Texas Southwestern Medical Center at Dallas, Children's Health, Center for Pediatric Urology, Dallas, TX.

Prune Belly Syndrome (PBS) is a congenital multisystem myopathy with mild to lethal severity. While of uncertain etiology, 95% male predominance and familial occurrence suggest a genetic basis. As copy number variations (CNVs) can cause unexplained genetic disorders, we tested for novel CNVs in a large PBS population. Read More

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http://dx.doi.org/10.1002/ajmg.a.40476DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289753PMC
November 2018
23 Reads

Living-related kidney transplantation with catheterizable urinary conduit in prune belly syndrome: A case report.

Int J Surg Case Rep 2018 19;51:150-153. Epub 2018 Aug 19.

Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.

Introduction: 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.

Presentation Of Case: We report the case of a 36-year-old man with PBS who underwent living-related kidney transplantation with urinary diversion using the Mitrofanoff principle. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22102612183032
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http://dx.doi.org/10.1016/j.ijscr.2018.08.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6122431PMC
August 2018
10 Reads

Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients.

BJU Int 2019 Jan 19;123(1):130-139. Epub 2018 Sep 19.

Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Objective: 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.

Patients And Methods: From 2000 to 2017, patients with PBS were recruited to our prospective PBS study and medical records were cross-sectionally analysed, generating individualised RUBACE scores. We designed the pragmatic RUBACE-scoring system based on six sub-scores (R: renal, U: ureter, B: bladder/outlet, A: abdominal wall, C: cryptorchidism, E: extra-genitourinary, generating the acronym RUBACE), yielding a potential summed score of 0-31. Read More

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http://dx.doi.org/10.1111/bju.14524DOI Listing
January 2019
13 Reads

Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction.

J Pediatr Urol 2018 Aug 24;14(4):320.e1-320.e6. Epub 2018 Jul 24.

Division of Pediatric Urology, University of Miami Miller School of Medicine/Jackson Memorial Hospital, Miami, FL, USA.

Background: Vesicoamniotic shunting (VAS) and other bladder drainage techniques for fetal lower urinary tract obstruction (LUTO) have been proven to ameliorate pulmonary hypoplasia and increase survival in patients with an initial poor prognosis. Currently there are limited prognostic tools available during gestation to evaluate and predict postnatal renal function.

Objective: The aim was to describe the prenatal growth of the renal parenchymal area (RPA) in patients with LUTO and determine its application as a predictor of renal function at one year of life. Read More

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http://dx.doi.org/10.1016/j.jpurol.2018.07.004DOI Listing
August 2018
6 Reads
1.413 Impact Factor

Management of Congenital Urethral Strictures In Infants. Case Series.

Urol J 2019 Feb 21;16(1):67-71. Epub 2019 Feb 21.

Paediatric Urology, University of Padova, Via Giustiniani 2, 35100 Padova, Italy.

Purpose: Infra-vesical obstruction is uncommon in infants and generally due to urethral valves. Congenital urethral strictures (CUS), instead, defined as a concentric narrowing of the urethral lumen, are exceedingly rare in infants.

Materials And Methods: We reviewed our experience with 7 patients treated at our institution for CUS Read More

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http://dx.doi.org/10.22037/uj.v0i0.4045DOI Listing
February 2019
7 Reads

Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

Front Pediatr 2018 3;6:180. Epub 2018 Jul 3.

Scott Department of Urology, Baylor College of Medicine, Houston, TX, United States.

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 collected included age at diagnosis, concomitant medical conditions, imaging, operative management, length of follow-up, and renal function. Read More

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http://dx.doi.org/10.3389/fped.2018.00180DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6038357PMC
July 2018
6 Reads

Collagen I and III in women with diastasis recti.

Clinics (Sao Paulo) 2018 06 7;73:e319. Epub 2018 Jun 7.

Servico de Cirurgia Digestiva, Hospital de Clinicas de Porto Alegre (HCPA), Porto Alegre, RS, BR.

Objectives: Interest in elucidating the etiology of hernias has encouraged countless studies of musculoaponeurotic structures in individuals with and without hernias. Studies of hernia patients have firmly demonstrated a correlation between hernias and collagen alterations in their fascia. Diastasis recti is an increased width of the abdominal midline that is exclusively composed of interlacing aponeurotic expansions of the anterolateral abdominal muscles. Read More

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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971415/?report
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http://dx.doi.org/10.6061/clinics/2018/e319DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971415PMC
June 2018
4 Reads

Minimal Invasive Linea Alba Reconstruction for the Treatment of Umbilical and Epigastric Hernias with Coexisting Rectus Abdominis Diastasis.

J Laparoendosc Adv Surg Tech A 2018 Oct 5;28(10):1223-1228. Epub 2018 Apr 5.

4 Department of General Surgery, Landeskrankenhaus Hochsteiermark , Bruck an der Mur, Austria .

Introduction: Patients with umbilical or epigastric hernias benefit from mesh- based repairs, and even more so if a concomitant rectus diastasis (RD) is present. The ideal technique is, however, still under debate. In this study we introduce the minimal invasive linea alba reconstruction (MILAR) with the supraaponeurotic placement of a fully absorbable synthetic mesh. Read More

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http://dx.doi.org/10.1089/lap.2018.0018DOI Listing
October 2018
9 Reads

Diastasis recti abdominis - a review of treatment methods.

Ginekol Pol 2018 ;89(2):97-101

Institute of Physiotherapy, Jan Kochanowski University in Kielce, Kielce, Poland.

Diastasis recti abdominis is a condition in which both rectus abdominis muscles disintegrate to the sides, this being ac-companied by the extension of the linea alba tissue and bulging of the abdominal wall. DRA may result in the herniation of the abdominal viscera, but it is not a hernia per se. DRA is common in the female population during pregnancy and in the postpartum period. Read More

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http://dx.doi.org/10.5603/GP.a2018.0016DOI Listing
July 2018
20 Reads

Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja.

Saudi J Kidney Dis Transpl 2018 Jan-Feb;29(1):178-184

Department of Pediatrics, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria.

Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Read More

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http://www.sjkdt.org/text.asp?2018/29/1/178/225190
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http://dx.doi.org/10.4103/1319-2442.225190DOI Listing
February 2018
10 Reads

Pediatric patient with end-stage kidney disease secondary to Eagle-Barrett syndrome and metastatic unresectable hepatoblastoma treated successfully with chemotherapy and liver-kidney transplant.

Pediatr Transplant 2018 03 22;22(2). Epub 2018 Jan 22.

Department of Pediatrics, University of Miami-Holtz Children's Hospital, Miami, FL, USA.

HBL is the most common malignant liver neoplasm in children. The etiology of HBL is largely unknown but there are certain syndromes, such as Beckwith-Wiedemann syndrome, that have been clearly associated with an increased incidence of this malignancy. EBS, also known as prune belly syndrome, is a congenital anomaly characterized by lax abdominal musculature, bilateral cryptorchidism requiring, in some cases, hemodialysis due to significant kidney and urinary tract dysfunctions. Read More

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http://dx.doi.org/10.1111/petr.13123DOI Listing
March 2018
4 Reads

Quadratus lumborum block for post-operative pain relief in patient with Prune belly syndrome.

Indian J Anaesth 2017 Oct;61(10):840-842

Department of Anaesthesia and Critical Care, Medanta - The Medicity Hospital, Gurugram, Haryana, India.

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. Read More

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http://dx.doi.org/10.4103/ija.IJA_246_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5664891PMC
October 2017
3 Reads

Prune belly syndrome: A report of 15 cases from Sudan.

Sudan J Paediatr 2017 ;17(1):42-48

Department of Paediatrics, Soba university hospital, Khartoum, Sudan.

Prune belly syndrome is a rare congenital malformation of unknown aetiology, composed of a triad of deficient abdominal wall muscle, cryptorchidism and urinary tract anomalies. The majority of patients have associated pulmonary, skeletal, cardiac, and gastrointestinal defects. This was a prospective, case finding study that was conducted in the main paediatric hospitals in Khartoum state, during the period December 2015 to September 2016. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5621858PMC
January 2017
16 Reads

Unusual presentation of prune belly syndrome: a case report.

J Med Case Rep 2017 Dec 4;11(1):337. Epub 2017 Dec 4.

Department of Radiology, School of Medicine, College of Medicine and Health Science, University of Gondar, Gondar, Ethiopia.

Background: 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. Read More

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http://dx.doi.org/10.1186/s13256-017-1487-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5713656PMC
December 2017
7 Reads

Rare copy number variants identified in prune belly syndrome.

Eur J Med Genet 2018 Mar 23;61(3):145-151. Epub 2017 Nov 23.

Division of Intramural Population Health Research, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, United States.

Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome, is a rare congenital disorder characterized by absence or hypoplasia of the abdominal wall musculature, urinary tract anomalies, and cryptorchidism in males. The etiology of PBS is largely unresolved, but genetic factors are implicated given its recurrence in families. We examined cases of PBS to identify novel pathogenic copy number variants (CNVs). Read More

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http://dx.doi.org/10.1016/j.ejmg.2017.11.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5803418PMC
March 2018
29 Reads

"Pseudo-prune belly syndrome": An under-reported phenotypic variant of prune belly syndrome.

J Pediatr Urol 2017 12 30;13(6):656-657. Epub 2017 Sep 30.

1100 Lake Hearn Drive Atlanta, GA 30342, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2017.08.015DOI Listing
December 2017
2 Reads

Combined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome.

Medicine (Baltimore) 2017 Oct;96(42):e7921

Department of General Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine.

Background: Prune belly syndrome (PBS) is a rare congenital disorder among adults, and the way for repairing abdominal wall musculature has no unified standard.

Materials And Methods: We described combining laparoscopic and open technique in an adult male who presented with PBS. Physical examination and radiological imaging verified the case of PBS. Read More

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http://dx.doi.org/10.1097/MD.0000000000007921DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5662352PMC
October 2017
5 Reads

Diastasis of rectus abdominis muscles in low back pain patients.

J Back Musculoskelet Rehabil 2018 Feb;31(1):107-112

Department of Rehabilitation and Sports Medicine, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.

Background: Abdominal muscles are important spinal stabilizers and its poor coordination, as seen in diastasis of rectus abdominis (DRA), may contribute to chronic low back pain (LBP). However, this has not yet been studied directly.

Objectives: To conduct a pilot study to examine the association between DRA and LBP. Read More

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http://dx.doi.org/10.3233/BMR-169687DOI Listing
February 2018
5 Reads

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

Pediatr Nephrol 2018 Jan 4;33(1):117-124. Epub 2017 Aug 4.

Department of Pediatric Nephrology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

Background: 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 (ESPN/ERA-EDTA) Registry data.

Methods: Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). Read More

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http://dx.doi.org/10.1007/s00467-017-3770-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5700229PMC
January 2018
67 Reads

Type V Pouch Colon, Prune Belly Syndrome, and Congenital Anterior Urethrocutaneous Fistula.

J Neonatal Surg 2017 Apr-Jun;6(2):38. Epub 2017 Apr 15.

Department of Pediatric Surgery, Rajendra Institute of Medical Sciences, Ranchi.

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). Read More

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http://dx.doi.org/10.21699/jns.v6i2.481DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5538604PMC
April 2017
5 Reads

Study of Testicular Structure in Fetuses with Prune Belly Syndrome.

Adv Urol 2017 21;2017:3254980. Epub 2017 May 21.

Urogenital Research Unit, State University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

Purpose: To compare the structure of the testis in fetuses with prune belly syndrome (PBS) to normal controls.

Materials And Methods: 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; Weigert's solution to observe elastic fibers; and picrosirius red to observe collagen. Read More

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http://dx.doi.org/10.1155/2017/3254980DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5457755PMC
May 2017
17 Reads

Structural study of the bladder in fetuses with prune belly syndrome.

Neurourol Urodyn 2018 Jan 9;37(1):148-152. Epub 2017 Jun 9.

Urogenital Research Unit, State University of Rio de Janeiro, Rio de Janeiro, Brazil.

Aims: To study the bladder structure of fetuses with prune belly syndrome (PBS).

Methods: We studied three bladders obtained from three male fetuses with PBS and seven bladders from seven male fetuses without anomalies. Each bladder was dissected and embedded in paraffin, from which 5 μm thick sections were obtained and stained with Masson's trichrome (to quantify connective tissue and smooth muscle) and picrosirius red with polarization (to observe collagen). Read More

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http://dx.doi.org/10.1002/nau.23327DOI Listing
January 2018
21 Reads

Prune Belly Syndrome with Sacrococcygeal Teratoma.

Indian J Pediatr 2017 11 8;84(11):869-870. Epub 2017 Jun 8.

Department of Pediatrics, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.

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http://dx.doi.org/10.1007/s12098-017-2396-zDOI Listing
November 2017
9 Reads

Pseudo Prune Belly Syndrome: Diagnosis Revealed by Imaging - A Case Report and Brief Review.

Pol J Radiol 2017 7;82:252-257. Epub 2017 May 7.

Department of Radiology and Imaging, Government Medical College and Rajindra Hospital, Patiala, Punjab, India.

Background: 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). Read More

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http://dx.doi.org/10.12659/PJR.899743DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5436412PMC
May 2017
34 Reads

Prenatal detection of megacystis: not always an adverse prognostic factor. Experience in 25 consecutive cases in a tertiary referral center, with complete neonatal outcome and follow-up.

J Pediatr Urol 2017 Oct 14;13(5):486.e1-486.e10. Epub 2017 Apr 14.

Department of Obstetrics and Gynecology, Fetal Diagnosis and Therapy Unit, Catholic University of Sacred Heart, Policlinico A. Gemelli Hospital, Rome, Italy.

Introduction: Megacystis is a condition of abnormal enlarged fetal bladder for gestational age, which is usually associated with urological malformations that may constitute a life-threatening condition for the baby.

Objective: The purpose of this study was to assess the prognostic and etiological criteria of fetal megacystis and to describe the neonatal outcome in a large series collected in a single tertiary center.

Study Design: A retrospective observational study was conducted between 2008 and 2012. Read More

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http://dx.doi.org/10.1016/j.jpurol.2017.04.001DOI Listing
October 2017
38 Reads

Abdominoplasty in prune belly syndrome: Modifications in Monfort technique to address variable patterns of abdominal wall weakness.

J Pediatr Urol 2017 Oct 22;13(5):502.e1-502.e6. Epub 2017 Mar 22.

Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA, USA.

Introduction: Abdominoplasty is an important component of the management of children with prune belly syndrome (PBS). While there are features of the abdominal defect in PBS which are common to all patients, there will be differences unique to each patient that should be taken into consideration in surgical planning. Specifically, we have come to realize that although the Monfort procedure assumes a symmetric pattern of abdominal wall laxity, this symmetry is rarely present. Read More

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http://dx.doi.org/10.1016/j.jpurol.2017.02.020DOI Listing
October 2017
19 Reads

Fetal megacystis: A systematic review.

J Pediatr Urol 2017 Feb 8;13(1):7-15. Epub 2016 Oct 8.

Department of Paediatric Surgery, Wellington Children's Hospital, Wellington, New Zealand; Department of Paediatrics and Child Health, University of Otago, Wellington, New Zealand.

Fetal megacystis is variably defined and understood. The literature on fetal megacystis was systematically reviewed, focusing on prenatal diagnosis, associations and outcomes. This yielded a total of 18 primary references and eight secondary references. Read More

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http://dx.doi.org/10.1016/j.jpurol.2016.09.003DOI Listing
February 2017
16 Reads

[Video-assisted sigmoidectomy as an option for Sigmoid volvulus management in the pediatric population].

Cir Pediatr 2015 Apr 15;28(2):88-94. Epub 2015 Apr 15.

Unidad de Cirugía Pediátrica. Facultad de Medicina. Universidad Nacional de Colombia. Sede Bogotá. Fundación HOMI Hospital de La Misericordia. Bogotá.

Introduction: The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children. Sigmoid volvulus is one of the three leading causes of acute obstruction of the colon and is between 50 and 90% of all large bowel volvulus. In the pediatric population only 3 to 5% of bowel obstructions are caused by volvulus and there are less than 100 cases reported in the literature. Read More

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April 2015
10 Reads

Prune Belly Syndrome with Situs Inversus Abdominus.

J Neonatal Surg 2016 Jul-Sep;5(3):38. Epub 2016 Jul 3.

Department of Pediatrics, JJ Group of Hospitals, Grant Medical College, Mumbai.

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http://dx.doi.org/10.21699/jns.v5i3.349DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942438PMC
July 2016
4 Reads

It's not All Doom and Gloom: Prune Belly Syndrome Associated with VACTERL.

J Neonatal Surg 2016 Jul-Sep;5(3):33. Epub 2016 Jul 3.

Department of Paediatric Surgery, Newcastle Upon Tyne Hospitals, Newcastle Upon Tyne, UK.

Prune belly syndrome is a rare abnormality; its association with VACTERL is even rarer. This association has been reported in literature a few times since first reported in 1993 and so far the majority have either been stillbirths or died shortly after birth. We present a case of Prune belly syndrome associated with VACTERL who is now one year old. Read More

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http://dx.doi.org/10.21699/jns.v5i3.337DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4942433PMC
July 2016
7 Reads

PATTERN AND OUTCOME OF RENAL DISEASES IN HOSPITALIZED CHILDREN IN TIKUR ANBESSA SPECIALIZED TEACHING HOSPITAL, ADDIS ABABA, ETHIOPIA.

Ethiop Med J 2016 Jul;54(3):117-23

Background: Renal diseases are major causes of morbidity and mortality in pediatric practice. Pediatric patients with renal disease, especially younger ones may present with nonspecific signs and symptoms unrelated to the urinary tract. Unexplained fever or failure to thrive may be the only manifestation. Read More

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July 2016
3 Reads

What is the outcome of paediatric gastrocystoplasty when the patients reach adulthood?

BJU Int 2016 Dec 24;118(6):980-986. Epub 2016 Jul 24.

Aix-Marseille Université, Marseille, France.

Objective: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications.

Patients And Methods: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients. Read More

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http://dx.doi.org/10.1111/bju.13558DOI Listing
December 2016
38 Reads

Further evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female.

J Pediatr Surg 2016 Aug 1;51(8):1390-3. Epub 2016 Jun 1.

Christchurch Hospital, University of Otago.

We present a female neonate born with prune belly syndrome (PBS) in whom a large intraabdominal cyst was diagnosed at 12weeks of gestation. Rapid and exponential growth of the cyst caused pressure effects on the intraabdominal organs and stretching of the anterior abdominal wall by 19weeks of gestation. This led to drainage of the massive cyst at 20weeks of gestation to prevent fetal demise. Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2016.05.017DOI Listing
August 2016
7 Reads

Clinical manifestations of prune belly syndrome.

Clin Med (Lond) 2016 Jun;16 Suppl 3:s5

Beaumont Hospital, Dublin, Ireland; Temple Street Children's University Hospital, Dublin, Ireland;

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http://dx.doi.org/10.7861/clinmedicine.16-3-s5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4989957PMC
June 2016
6 Reads

Regarding commentary to: Detailed evaluation of the upper urinary tract in patients with Prune Belly Syndrome using magnetic resonance urography.

J Pediatr Urol 2016 Apr 6;12(2):124-5. Epub 2016 Jan 6.

From the Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University, Atlanta, GA, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2015.12.001DOI Listing
April 2016
5 Reads

Genetics of Vesicoureteral Reflux.

Curr Genomics 2016 Feb;17(1):70-9

Pediatric Surgery Unit - Salesi Children s Hospital - UniversitPolitecnica delle Marche - Ancona,Italy.

Vesicoureteral reflux (VUR) is the retrograde passage of urine from the bladder to the upper urinary tract. It is the most common congenital urological anomaly affecting 1-2% of children and 30-40% of patients with urinary tract infections. VUR is a major risk factor for pyelonephritic scarring and chronic renal failure in children. Read More

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http://dx.doi.org/10.2174/1389202916666151014223507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4780477PMC
February 2016
4 Reads

Trends in Renal Transplantation Rates in Patients with Congenital Urinary Tract Disorders.

J Urol 2016 Apr 28;195(4 Pt 2):1257-62. Epub 2016 Feb 28.

Department of Urology, Cleveland Clinic, Cleveland, Ohio.

Purpose: Improved bladder and renal management benefit patients with congenital uropathy and congenital pediatric kidney disease. This may translate to delayed initial renal transplantation in these patients, and improved graft and patient survival. Our primary study purpose was to determine whether patients with congenital uropathy and congenital pediatric kidney disease have demonstrated later time to first transplantation and/or graft survival. Read More

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http://dx.doi.org/10.1016/j.juro.2015.10.004DOI Listing
April 2016
11 Reads

Commentary to "Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography".

J Pediatr Urol 2016 Apr 29;12(2):123. Epub 2015 Dec 29.

University of Ottawa, Department of Surgery, 401 Smyth Road, Ottawa, ON K1H 8L1, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.jpurol.2015.11.013DOI Listing
April 2016
5 Reads

Proposed technique for open repair of a small umbilical hernia and rectus divarication with self-gripping mesh.

Authors:
B J Privett M Ghusn

Hernia 2016 08 17;20(4):527-30. Epub 2016 Feb 17.

The Tweed Hospital, Tweed Heads, Australia.

Introduction: There are a group of patients in which umbilical or epigastric hernias co-exist with rectus divarication. These patients have weak abdominal musculature and are likely to pose a higher risk of recurrence following umbilical hernia repair. We would like to describe a technique for open repair of small (<4 cm) midline hernias in patients with co-existing rectus divarication using self-adhesive synthetic mesh. Read More

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http://link.springer.com/10.1007/s10029-016-1470-1
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http://dx.doi.org/10.1007/s10029-016-1470-1DOI Listing
August 2016
5 Reads

Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography.

J Pediatr Urol 2016 Apr 17;12(2):122.e1-7. Epub 2015 Dec 17.

Department of Pediatric Urology, Children's Healthcare of Atlanta and Emory University, Atlanta, GA, USA. Electronic address:

Introduction: Magnetic resonance urography (MRU) has proven to be useful in the setting of complex urologic anatomy. Prune belly syndrome (PBS) patients are known to have malformed and highly variable urinary tract anatomy due to significant dilation and renal dysplasia.

Objective: To further characterize the renal and ureteral anatomy and renal function in patients with PBS via MRU. Read More

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http://dx.doi.org/10.1016/j.jpurol.2015.11.008DOI Listing
April 2016
15 Reads