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https://www.readbyqxmd.com/read/29456226/three-cases-of-prune-belly-syndrome-at-the-lagos-state-university-teaching-hospital-ikeja
#1
Adaobi U Solarin, Elizabeth A Disu, Henry O Gbelee, Adeola B Animasahaun, Oluwatosin E Aremu, Eucharia Ogbuokiri, Gbemisola O Ogunnaike, Alaba Oladimeji
Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29356335/pediatric-patient-with-end-stage-kidney-disease-secondary-to-eagle-barrett-syndrome-and-metastatic-unresectable-hepatoblastoma-treated-successfully-with-chemotherapy-and-liver-kidney-transplant
#2
Daniel Ortiz, Avis Harden, Fernando F Corrales-Medina, Gaurav Saigal, Akin Tekin, Jennifer Garcia
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. Despite an improvement on the survival rates of patients with advanced-stage HBL, the presence of concomitant end-stage renal disease that occurs in patients with EBS constitutes a therapeutic challenge for the clinician not only due to the use of nephrotoxic chemotherapy but also due to the potential need for multi-organ transplant...
January 22, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29242658/quadratus-lumborum-block-for-post-operative-pain-relief-in-patient-with-prune-belly-syndrome
#3
Chitra Garg, Sangeeta Khanna, Yatin Mehta
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...
October 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/29213170/prune-belly-syndrome-a-report-of-15-cases-from-sudan
#4
Abdelmoneim E M Kheir, Eltigani M A Ali, Safaa A Medani, Huda S Maaty
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. A total of 15 patients with prune belly syndrome were collected. Patients' characteristics were noted including socio-demographic data, laboratory and radiological investigations and any medical or surgical intervention...
2017: Sudanese Journal of Paediatrics
https://www.readbyqxmd.com/read/29198187/unusual-presentation-of-prune-belly-syndrome-a-case-report
#5
Abayneh Girma Demisse, Ashenafi Berhanu, Temesgen Tadesse
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. We are unaware of any previous report of prune belly syndrome in Ethiopia...
December 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29174092/rare-copy-number-variants-identified-in-prune-belly-syndrome
#6
Nansi S Boghossian, Robert J Sicko, Andreas Giannakou, Aggeliki Dimopoulos, Michele Caggana, Michael Y Tsai, Edwina H Yeung, Nathan Pankratz, Benjamin R Cole, Paul A Romitti, Marilyn L Browne, Ruzong Fan, Aiyi Liu, Denise M Kay, James L Mills
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). A total of 34 cases (30 males and 4 females) with PBS identified from all live births in New York State (1998-2005) were genotyped using Illumina HumanOmni2...
November 22, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29049186/combined-laparoscopic-and-open-technique-for-repair-of-congenital-abdominal-hernia-a-case-report-of-prune-belly-syndrome
#7
Qinghuang Ye, Yan Chen, Jinhui Zhu, Yuedong Wang
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. The deficiency of abdominal wall musculature was repaired by combining laparoscopic and open technique using a double-deck complex patch...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28770135/type-v-pouch-colon-prune-belly-syndrome-and-congenital-anterior-urethrocutaneous-fistula
#8
Prince Raj, Hirendra Birua
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).
April 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28607553/study-of-testicular-structure-in-fetuses-with-prune-belly-syndrome
#9
Luciano A Favorito, Suelen F Costa, Waldemar S Costa, Rodrigo Vieiralves, Fabio O Bernardo, Francisco J B Sampaio
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-μm 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. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera...
2017: Advances in Urology
https://www.readbyqxmd.com/read/28598513/structural-study-of-the-bladder-in-fetuses-with-prune-belly-syndrome
#10
Helce R Julio Junior, Suelen F Costa, Waldemar S Costa, Francisco J Barcellos Sampaio, Luciano A Favorito
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)...
June 9, 2017: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/28580040/pseudo-prune-belly-syndrome-diagnosis-revealed-by-imaging-a-case-report-and-brief-review
#11
Hemal Grover, Sanjay Sethi, Jatin Garg, Amrit Pal Ahluwalia
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). 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...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28495235/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
#12
Marcella Pellegrino, Daniela Visconti, Vincenzo Davide Catania, Luisa D'Oria, Carlo Manzoni, Maria Giovanna Grella, Alessandro Caruso, Lucia Masini, Giuseppe Noia
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...
October 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28373000/abdominoplasty-in-prune-belly-syndrome-modifications-in-monfort-technique-to-address-variable-patterns-of-abdominal-wall-weakness
#13
Edwin A Smith, Arun Srinivasan, Hal C Scherz, Anthony J Tracey, Bruce Broecker, Andrew J Kirsch
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. OBJECTIVE: The aim of this report is to describe our modifications and review our outcomes for the Monfort procedure which more completely address correction of the abdominal wall laxity including both common and uncommon features while positioning the umbilicus to a more anatomically correct position (Figure)...
October 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27889224/fetal-megacystis-a-systematic-review
#14
REVIEW
K Taghavi, C Sharpe, M D Stringer
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. Fetal megacystis has an estimated first-trimester prevalence of between 1:330 and 1:1670, with a male to female ratio of 8:1. In the first trimester, megacystis is most commonly defined as a longitudinal bladder dimension of ≥7 mm. Later in pregnancy, a sagittal dimension (in mm) greater than gestational age (in weeks) + 12 is often accepted...
February 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27775288/-video-assisted-sigmoidectomy-as-an-option-for-sigmoid-volvulus-management-in-the-pediatric-population
#15
S Castañeda, I Molina, P Jaimes, J Beltrán, J Valero, F Fierro
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. The presence of a redundant sigmoid with a narrow mesentery (dolicosigmoide) is a prerequisite for the volvulus formation...
April 15, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27433456/prune-belly-syndrome-with-situs-inversus-abdominus
#16
Ganavi Ramagopal, Ganesh Narayana, Ashok Rathod
No abstract text is available yet for this article.
July 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27433451/it-s-not-all-doom-and-gloom-prune-belly-syndrome-associated-with-vacterl
#17
Karim Awad, Anupam Lall
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.
July 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27322857/what-is-the-outcome-of-paediatric-gastrocystoplasty-when-the-patients-reach-adulthood
#18
Romain Boissier, Eugenie Di Crocco, Alice Faure, Geraldine Hery, Véronique Delaporte, Eric Lechevallier, Pierre D E Mouriquand, Jean-Michel Guys, Gilles Karsenty
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...
December 2016: BJU International
https://www.readbyqxmd.com/read/27252342/clinical-manifestations-of-prune-belly-syndrome
#19
R Chhabra, A Awan, C Stapleton, G Cavalleri, P Conlon
No abstract text is available yet for this article.
June 1, 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/26994452/signs-and-symptoms-of-developmental-abnormalities-of-the-genitourinary-tract
#20
REVIEW
Paulo Cesar Koch Nogueira, Isabel de Pádua Paz
OBJECTIVE: The abnormalities of the genitourinary tract development are the leading cause of chronic kidney disease (CKD) in children. The diagnosis of this disease in Brazil is late and incomplete, which results in increased morbidity and mortality in this age group. Early diagnosis of this condition is the prerogative of generalist pediatricians, and the aim of this study was to review the clinical signs and symptoms associated with developmental abnormalities of the genitourinary tract...
May 2016: Jornal de Pediatria
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