830 results match your criteria Prune Belly Syndrome


Are pressure pop-offs beneficial to the bladder in boys with posterior urethral valves?

J Pediatr Urol 2020 Jun 3. Epub 2020 Jun 3.

Division of Urology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E Chicago Ave, Chicago, IL, 60611, USA. Electronic address:

Background: Pressure pop-offs, such as high-grade vesicoureteral reflux with renal dysplasia, have historically been considered beneficial for renal and bladder outcomes in boys with posterior urethral valves (PUV). Recent longer-term studies have called into question the beneficial effects of pop-offs on renal function.

Objective: To evaluate how pop-offs affect bladder outcomes in boys with PUV. Read More

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http://dx.doi.org/10.1016/j.jpurol.2020.05.154DOI Listing

Demographics and baseline care among newly transitioning adult congenital urology patients.

J Pediatr Urol 2020 Jun 1. Epub 2020 Jun 1.

University of California, San Francisco, Mission Hall Global Health Sci Bldg, 550 16th Street, 6th Floor, Box 1695, San Francisco, CA, 94143, USA. Electronic address:

Introduction: In 2017, UCSF established a formal Transitional Urology (TU) clinic co-run by pediatric and adult urology aimed at providing comprehensive urologic care for people progressing into adulthood with complex urologic histories.

Objective: We aim to describe baseline demographic and disease characteristics of this population, understand gaps in care, and gauge follow-through.

Study Design: We performed a retrospective chart review of all new patients in the TU clinic at UCSF from February 2017 through January 2019. Read More

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http://dx.doi.org/10.1016/j.jpurol.2020.05.167DOI Listing

GONADAL FUNCTION AND REPRODUCTIVE SYSTEM ANATOMY IN POST- PUBERTAL PRUNE-BELLY SYNDROME PATIENTS.

Urology 2020 Jun 13. Epub 2020 Jun 13.

Human Reproduction Unit, Division of Urology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Brazil.

Objective: 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 post pubertal PBS patients.

Methods: We contacted all PBS patients 14 years-old or older treated and followed at our institution. Age at orchiopexy, type of orchiopexy (with or without ligation of gonadal vessels), testicular volumes and positions were evaluated. Read More

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http://dx.doi.org/10.1016/j.urology.2020.04.124DOI Listing

Challenges in pediatric urologic practice: a lifelong view.

World J Urol 2020 Apr 23. Epub 2020 Apr 23.

Department of Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany.

The role of the pediatric urologic surgeon does not end with initial reconstructive surgery. Many of the congenital anomalies encountered require multiple staged operations while others may not involve further surgery but require a life-long follow-up and often revisions. Management of most of these disorders must extend into and through adolescence before transitioning these patients to adult colleagues. Read More

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http://dx.doi.org/10.1007/s00345-020-03203-1DOI Listing

Prune belly syndrome in surviving males can be caused by Hemizygous missense mutations in the X-linked Filamin A gene.

BMC Med Genet 2020 02 21;21(1):38. Epub 2020 Feb 21.

Department of Urology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.

Background: 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) wrinkled flaccid ventral abdominal wall with skeletal muscle deficiency, 2) urinary tract dilation with poorly contractile smooth muscle, and 3) intra-abdominal undescended testes. Despite evidence for a genetic basis, previously reported PBS autosomal candidate genes only account for one consanguineous family and single cases. Read More

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http://dx.doi.org/10.1186/s12881-020-0973-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035669PMC
February 2020

Prune Belly Syndrome in the Pig (Sus scrofa domesticus).

J Comp Pathol 2020 Jan 13;174:81-85. Epub 2019 Dec 13.

Private Practitioner, Clínica Veterinaria Salud Animal, Griñón, Madrid, Spain.

Prune belly syndrome (PBS) is a rare multisystemic disease characterized by a deficiency of abdominal wall musculature, urinary tract anomalies and bilateral intra-abdominal testes. PBS in females is called pseudo prune belly syndrome (PPBS) and is characterized by abdominal wall laxity and urinary tract defects. We report three male and one female stillborn piglets with abdominal distension, hypoplastic abdominal wall musculature and urinary tract anomalies. Read More

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http://dx.doi.org/10.1016/j.jcpa.2019.11.004DOI Listing
January 2020

Prune belly syndrome.

Med Clin (Barc) 2019 Nov 25. Epub 2019 Nov 25.

Sección de Gastroenterología y Nutrición, Hospital Infantil Universitario Niño Jesús, Madrid, España.

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http://dx.doi.org/10.1016/j.medcli.2019.09.002DOI Listing
November 2019

Study of Prostate Growth in Prune Belly Syndrome and Anencephalic Fetuses.

J Pediatr Surg 2019 Nov 5. Epub 2019 Nov 5.

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

Background: To compare the growth of the prostate in anencephalic, prune belly syndrome (PBS) and control fetuses.

Methods: 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 the prostate length, width and thickness with the aid of a computer program (Image Pro and Image J). Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.10.054DOI Listing
November 2019

Renal Arterial Doppler Resistive Index in Differentiating Obstructive and Non-Obstructive Hydronephrosis in Children at Tikur Anbessa Specialized Hospital, Ethiopia, October, 2018.

Ethiop J Health Sci 2019 Sep;29(5):591-596

Department of Radiology, School of Medicine, College of Health Sciences, Addis Ababa University, Ethiopia.

Background: Most congenital anomalies of the urinary tract present with hydronephrosis. Some are physiological while others pathological. Ultrasound, micturating cyst urethrography, dynamic renal scintigraphy, intravenous urography, dynamic and static magnetic resonance urography are used for examination. Read More

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http://dx.doi.org/10.4314/ejhs.v29i5.9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6813268PMC
September 2019

Prune belly syndrome: current perspectives.

Pediatric Health Med Ther 2019 6;10:75-81. Epub 2019 Aug 6.

Emory University, Children's Healthcare of Atlanta, Atlanta, GA 30328, USA.

Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities. Read More

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http://dx.doi.org/10.2147/PHMT.S188014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6689549PMC
August 2019
10 Reads

A homozygous missense variant in CHRM3 associated with familial urinary bladder disease.

Clin Genet 2019 12 11;96(6):515-520. Epub 2019 Sep 11.

Manchester Centre for Genomic Medicine, Manchester University NHS Foundation Trust, Health Innovation Manchester, Manchester, UK.

CHRM3 codes for the M3 muscarinic acetylcholine receptor that is located on the surface of smooth muscle cells of the detrusor, the muscle that effects urinary voiding. Previously, we reported brothers in a family affected by a congenital prune belly-like syndrome with mydriasis due to homozygous CHRM3 frameshift variants. In this study, we describe two sisters with bladders that failed to empty completely and pupils that failed to constrict fully in response to light, who are homozygous for the missense CHRM3 variant c. Read More

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http://dx.doi.org/10.1111/cge.13631DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6899476PMC
December 2019
5 Reads

Long term follow-up in a patient with prune-belly syndrome - a care compliant case report.

Medicine (Baltimore) 2019 Aug;98(33):e16745

4th Pediatric Clinic, Department of Nephrology and Dialysis Children, Emergency Hospital for Children Iaşi, Romania.

Rationale: 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. Read More

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http://dx.doi.org/10.1097/MD.0000000000016745DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6831394PMC
August 2019
4 Reads

Do prune-belly syndrome and neural tube defects change testicular growth? A study on human fetuses.

J Pediatr Urol 2019 Oct 6;15(5):557.e1-557.e8. Epub 2019 Jul 6.

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

Background: There are no reports comparing testicular volume between normal fetuses, fetuses with prune-belly syndrome (PBS), and fetuses with anencephaly. The study hypothesis was that PBS and especially anencephaly alter the testicular volume during the human fetal period.

Aim: The objective of the study was to compare the testicular growth in fetuses with anencephaly, with PBS, and without anomalies. Read More

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http://dx.doi.org/10.1016/j.jpurol.2019.06.023DOI Listing
October 2019
4 Reads

Challenging diagnosis of prune belly syndrome antenatally: a case report.

J Med Case Rep 2019 Jun 29;13(1):198. Epub 2019 Jun 29.

Department of Obstetrics and Gynecology, College of Medicine, King Saud University, King Khalid University Hospital, King Saud University Medical City, P.O BOX 4663, 11412, Riyadh City, Almohammadiyah, Kingdom of Saudi Arabia.

Background: Prune belly syndrome is a rare congenital condition of uncertain etiology. It is characterized with a triad of abdominal distension due to deficient abdominal wall, genitourinary tract anomalies, and musculoskeletal anomalies. This condition varies in its severity which makes diagnosis challenging during early antenatal scanning. Read More

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http://dx.doi.org/10.1186/s13256-019-2120-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6599311PMC
June 2019
7 Reads

Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect.

Radiol Case Rep 2019 Aug 29;14(8):941-945. Epub 2019 May 29.

Department of Mother's and Child's health, Poliambulanza Foundation Hospital Institute, Via Leonida Bissolati, 57, 25124, Brescia, Italy.

Prune Belly syndrome occurs in 1/40,000 live births and predominantly in males. It is characterized by triad: cryptorchidism, abdominal wall, and urinary tract abnormalities. Patients with partial or unilateral abdominal wall deficiency, unilateral undescended testis, and female neonates with abdominal wall laxity are classified as Pseudo Prune Belly syndrome. Read More

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http://dx.doi.org/10.1016/j.radcr.2019.05.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543186PMC
August 2019
6 Reads

A Neonate with Prune Belly Syndrome and Limb Reduction Defect.

Indian J Pediatr 2019 11 8;86(11):1056-1057. Epub 2019 Jun 8.

Department of Pediatrics, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, 133207, India.

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http://dx.doi.org/10.1007/s12098-019-03003-5DOI Listing
November 2019
11 Reads

The Cost of Uncertainty: A Patient's Perspective.

J Am Coll Radiol 2019 May;16(5):737-739

Department of Radiology and the Department of Urology, Michigan Medicine, Ann Arbor, Michigan; Michigan Radiology Quality Collaborative, Ann Arbor, Michigan. Electronic address:

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http://dx.doi.org/10.1016/j.jacr.2019.01.007DOI Listing

Congenital Disorders of the Human Urinary Tract: Recent Insights From Genetic and Molecular Studies.

Front Pediatr 2019 11;7:136. Epub 2019 Apr 11.

Division of Cell Matrix Biology and Regenerative Medicine, Faculty of Biology Medicine and Health, School of Biological Sciences, University of Manchester, Manchester, United Kingdom.

The urinary tract comprises the renal pelvis, the ureter, the urinary bladder, and the urethra. The tract acts as a functional unit, first propelling urine from the kidney to the bladder, then storing it at low pressure inside the bladder which intermittently and completely voids urine through the urethra. Congenital diseases of these structures can lead to a range of diseases sometimes associated with fetal losses or kidney failure in childhood and later in life. Read More

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https://www.frontiersin.org/article/10.3389/fped.2019.00136/
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http://dx.doi.org/10.3389/fped.2019.00136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6470263PMC
April 2019
34 Reads

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

Fetal Pediatr Pathol 2019 Oct 19;38(5):412-417. Epub 2019 Apr 19.

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.

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
October 2019
28 Reads

Long-term Outcome of Renal Transplantation in Patients with Congenital Lower Urinary Tract Malformations: A Multicenter Study.

Transplantation 2020 Jan;104(1):165-171

Department of Urology and Renal Transplantation, Montpellier University Hospital, France.

Background: 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
January 2020
10 Reads

Differences in Linea Alba Stiffness and Linea Alba Distortion Between Women With and Without Diastasis Recti Abdominis: The Impact of Measurement Site and Task.

J Orthop Sports Phys Ther 2019 09 26;49(9):656-665. Epub 2019 Mar 26.

Background: The biomechanical implications of diastasis recti abdominis (DRA) are unknown.

Objectives: To (1) investigate the impact of DRA, measurement site, and task on inter-rectus distance (IRD), linea alba (LA) stiffness, and LA distortion measured at rest and during head-lift and semi-curl-up tasks; and (2) describe the relationships among IRD, LA stiffness, and LA distortion.

Methods: In this cross-sectional, observational cohort study, brightness-mode ultrasound imaging and shearwave elastography were used on a sample of 20 women. Read More

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https://www.jospt.org/doi/10.2519/jospt.2019.8543
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http://dx.doi.org/10.2519/jospt.2019.8543DOI Listing
September 2019
14 Reads

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

BJU Int 2019 11 18;124(5):876-882. Epub 2019 Mar 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
November 2019
12 Reads

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

Turk Patoloji Derg 2020 ;36(1):64-67

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 2020
35 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
11 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
11 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
12 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
43 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
13 Reads

Copy number variations in a population with prune belly syndrome.

Am J Med Genet A 2018 11 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
58 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
16 Reads

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

BJU Int 2019 01 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
19 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 08 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
24 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
14 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
28 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
15 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
14 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
27 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
October 2019
13 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
10 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
9 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
25 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
13 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
54 Reads

A newborn with abdominal distension.

Authors:

J Paediatr Child Health 2017 11;53(11):1133

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http://dx.doi.org/10.1111/jpc.2_13791DOI Listing
November 2017
4 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
7 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
11 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
12 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
110 Reads