Database : MEDLINE
Search on : Prune and Belly and Syndrome [Words]
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[PMID]: 29356335
[Au] Autor:Ortiz D; Harden A; Corrales-Medina FF; Saigal G; Tekin A; Garcia J
[Ad] Address:Department of Pediatrics, University of Miami-Holtz Children's Hospital, Miami, FL, USA.
[Ti] Title:Pediatric patient with end-stage kidney disease secondary to Eagle-Barrett syndrome and metastatic unresectable hepatoblastoma treated successfully with chemotherapy and liver-kidney transplant.
[So] Source:Pediatr Transplant;22(2), 2018 Mar.
[Is] ISSN:1399-3046
[Cp] Country of publication:Denmark
[La] Language:eng
[Ab] Abstract: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. We report case of a 2-year-old male patient with EBS diagnosed with stage IV, metastatic HBL successfully treated with multi-agent chemotherapy while on dialysis whom then underwent a simultaneous liver-kidney transplant followed by adjuvant chemotherapy. Ultimately, the patient achieved cancer remission with normalization of his renal function. Our report emphasizes that patients with HBL in the setting of EBS will not only require careful kidney function monitoring while receiving chemotherapy, but they might also need to undergo multi-organ transplantation in order to achieve adequate cancer control and also normalization of their kidney function. Awareness of this unusual association calls for further investigation to potentially establish a genetic association between these two disease processes.
[Pt] Publication type:CASE REPORTS
[Em] Entry month:1801
[Cu] Class update date: 180220
[Lr] Last revision date:180220
[St] Status:In-Data-Review
[do] DOI:10.1111/petr.13123

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[PMID]: 29456226
[Au] Autor:Solarin AU; Disu EA; Gbelee HO; Animasahaun AB; Aremu OE; Ogbuokiri E; Ogunnaike GO; Oladimeji A
[Ad] Address:Department of Pediatrics, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria.
[Ti] Title:Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja.
[So] Source:Saudi J Kidney Dis Transpl;29(1):178-184, 2018 Jan-Feb.
[Is] ISSN:1319-2442
[Cp] Country of publication:Saudi Arabia
[La] Language:eng
[Ab] Abstract: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. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.
[Pt] Publication type:CASE REPORTS
[Em] Entry month:1802
[Cu] Class update date: 180219
[Lr] Last revision date:180219
[St] Status:In-Data-Review
[do] DOI:10.4103/1319-2442.225190

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[PMID]: 29174092
[Au] Autor:Boghossian NS; Sicko RJ; Giannakou A; Dimopoulos A; Caggana M; Tsai MY; Yeung EH; Pankratz N; Cole BR; Romitti PA; Browne ML; Fan R; Liu A; Kay DM; Mills JL
[Ad] Address:Department of Epidemiology and Biostatistics, Arnold School of Public Health, University of South Carolina, Columbia, SC, United States; Division of Intramural Population Health Research, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health,
[Ti] Title:Rare copy number variants identified in prune belly syndrome.
[So] Source:Eur J Med Genet;61(3):145-151, 2018 Mar.
[Is] ISSN:1878-0849
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract: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 HumanOmni2.5 microarrays. CNVs were prioritized if they were absent from in-house controls, encompassed ≥10 consecutive probes, were ≥20 Kb in size, had ≤20% overlap with common variants in population reference controls, and had ≤20% overlap with any variant previously detected in other birth defect phenotypes screened in our laboratory. We identified 17 candidate autosomal CNVs; 10 cases each had one CNV and four cases each had two CNVs. The CNVs included a 158 Kb duplication at 4q22 that overlaps the BMPR1B gene; duplications of different sizes carried by two cases in the intron of STIM1 gene; a 67 Kb duplication 202 Kb downstream of the NOG gene, and a 1.34 Mb deletion including the MYOCD gene. The identified rare CNVs spanned genes involved in mesodermal, muscle, and urinary tract development and differentiation, which might help in elucidating the genetic contribution to PBS. We did not have parental DNA and cannot identify whether these CNVs were de novo or inherited. Further research on these CNVs, particularly BMP signaling is warranted to elucidate the pathogenesis of PBS.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 180210
[Lr] Last revision date:180210
[St] Status:In-Process

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[PMID]: 29198187
[Au] Autor:Demisse AG; Berhanu A; Tadesse T
[Ad] Address:Department of Pediatrics and Child Health, School of Medicine, College of Medicine and Health Science, University of Gondar, Gondar, Ethiopia. ababiya@yahoo.com.
[Ti] Title:Unusual presentation of prune belly syndrome: a case report.
[So] Source:J Med Case Rep;11(1):337, 2017 Dec 04.
[Is] ISSN:1752-1947
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract: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. CASE PRESENTATION: A 12-year-old Amhara boy from the Northwest Gondar Amhara regional state presented to our referral hospital with a complaint of swelling over his left flank for the past 3 months. Maternal pregnancy course and medical history were noncontributory, and he had an attended birth at a health center. He has seven siblings, none of whom had similar symptoms. On examination he had a distended abdomen, asymmetric with bulging left flank, visible horizontal line, upward umbilical slit, and absent rectus abdominis muscles. His abdomen was soft with a tender cystic, bimanually palpable mass on the left flank measuring 13 × 11 cm. Both testes were undescended and he also has developmental dysplasia of the hips. An abdominal ultrasound revealed a large cystic mass in his left kidney area with echo debris and a hip X-ray showed bilateral developmental dysplasia of the hip. Intraoperative findings were cystic left kidney, both testes were intraperitoneal, tortuous left renal vein, enlarged bladder reaching above umbilicus, and left megaureter. INTERVENTIONS: bilateral orchidectomy and left nephrectomy were done. He was given intravenously administered antibiotics for treatment of pyelonephritis and discharged home with an appointment for follow up and possible abdominoplasty. CONCLUSIONS: In the current report delayed presentation contributed to testicular atrophy and decision for orchidectomy. Furthermore, he will be at potential risk for sex hormone abnormality. Therefore, diagnosis of prune belly syndrome in resource-limited settings requires a high index of suspicion. We recommend further research to determine the optimal management and early diagnosis of prune belly syndrome in resource-limited settings.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 171220
[Lr] Last revision date:171220
[St] Status:In-Process
[do] DOI:10.1186/s13256-017-1487-9

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[PMID]: 28779237
[Au] Autor:Yalcinkaya F; Bonthuis M; Erdogan BD; van Stralen KJ; Baiko S; Chehade H; Maxwell H; Montini G; Rönnholm K; Sørensen SS; Ulinski T; Verrina E; Weber S; Harambat J; Schaefer F; Jager KJ; Groothoff JW
[Ad] Address:Department of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey.
[Ti] Title:Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.
[So] Source:Pediatr Nephrol;33(1):117-124, 2018 Jan.
[Is] ISSN:1432-198X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract: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). RESULTS: Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups. CONCLUSIONS: This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 171205
[Lr] Last revision date:171205
[St] Status:In-Process
[do] DOI:10.1007/s00467-017-3770-9

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[PMID]: 29100970
[Au] Autor:Kapoor S
[Ad] Address:1100 Lake Hearn Drive Atlanta, GA 30342, USA. Electronic address: kkhh4971@gmail.com.
[Ti] Title:"Pseudo-prune belly syndrome": An under-reported phenotypic variant of prune belly syndrome.
[So] Source:J Pediatr Urol;, 2017 Sep 30.
[Is] ISSN:1873-4898
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1711
[Cu] Class update date: 171104
[Lr] Last revision date:171104
[St] Status:Publisher

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[PMID]: 29049186
[Au] Autor:Ye Q; Chen Y; Zhu J; Wang Y
[Ad] Address:Department of General Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine.
[Ti] Title:Combined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome.
[So] Source:Medicine (Baltimore);96(42):e7921, 2017 Oct.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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. RESULTS: The patient successfully underwent abdominal wall repair by combining laparoscopic and open technique. Postoperative recovery was uneventful, and improvement in symptom was significant in follow-up after 3, 6, 12, and 24 months. CONCLUSIONS: Combining laparoscopic and open technique for repair of deficiency of abdominal wall musculature in PBS was an exploratory way to improve life quality.
[Mh] MeSH terms primary: Gastroscopy/methods
Hernia, Abdominal/surgery
Herniorrhaphy/methods
Laparoscopy/methods
Prune Belly Syndrome/complications
[Mh] MeSH terms secundary: Adult
Combined Modality Therapy/methods
Hernia, Abdominal/congenital
Humans
Male
Prune Belly Syndrome/surgery
Treatment Outcome
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171122
[Lr] Last revision date:171122
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:171020
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007921

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[PMID]: 28770135
[Au] Autor:Raj P; Birua H
[Ad] Address:Department of Pediatric Surgery, Rajendra Institute of Medical Sciences, Ranchi.
[Ti] Title:Type V Pouch Colon, Prune Belly Syndrome, and Congenital Anterior Urethrocutaneous Fistula.
[So] Source:J Neonatal Surg;6(2):38, 2017 Apr-Jun.
[Is] ISSN:2226-0439
[Cp] Country of publication:Pakistan
[La] Language:eng
[Ab] Abstract: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).
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170807
[Lr] Last revision date:170807
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.21699/jns.v6i2.481

  9 / 781 MEDLINE  
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Costa, Waldemar S
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[PMID]: 28607553
[Au] Autor:Favorito LA; Costa SF; Costa WS; Vieiralves R; Bernardo FO; Sampaio FJB
[Ad] Address:Urogenital Research Unit, State University of Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
[Ti] Title:Study of Testicular Structure in Fetuses with Prune Belly Syndrome.
[So] Source:Adv Urol;2017:3254980, 2017.
[Is] ISSN:1687-6369
[Cp] Country of publication:Egypt
[La] Language:eng
[Ab] Abstract: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. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera. The stereological analysis was done with the Image Pro and Image J programs. Means were statistically compared using the Mann-Whitney test ( < 0.005). RESULTS: Quantitative analysis documented no differences ( = 0.4) in number of seminiferous tubules (ST) in PBS testes (mean = 8.87%, SD = 1.59), when compared to the control (mean = 11.4%, SD = 2.99) and no differences ( = 0.8) in diameter of ST in PBS testes (mean = 52.85 m, SD = 1.58) when compared to the control group (mean = 53.17 m, SD = 1.55), but we did observe a lower number ( = 0.0002) of Leydig cells in the PBS testes (mean = 67.03% and SD = 3.697) when compared to the control group (mean = 90.1% and SD = 2.986). CONCLUSIONS: Our study showed a lower concentration of Leydig cells in the triad syndrome fetuses.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1706
[Cu] Class update date: 170616
[Lr] Last revision date:170616
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1155/2017/3254980

  10 / 781 MEDLINE  
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Costa, Waldemar S
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[PMID]: 28598513
[Au] Autor:Julio Junior HR; Costa SF; Costa WS; Barcellos Sampaio FJ; Favorito LA
[Ad] Address:Urogenital Research Unit, State University of Rio de Janeiro, Rio de Janeiro, Brazil.
[Ti] Title:Structural study of the bladder in fetuses with prune belly syndrome.
[So] Source:Neurourol Urodyn;, 2017 Jun 09.
[Is] ISSN:1520-6777
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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). Immunohistochemistry with tubulin (Tubulin, beta III, Mouse Monoclonal Antibody) was applied to observe the bladder nerves. The images were captured with an Olympus BX51 microscope and Olympus DP70 camera. The stereological analysis was done with the Image Pro and Image J programs, using a grid to determine volumetric densities (Vv). Means were statistically compared using the Mann-Whitney test (P < 0.05). RESULTS: Quantitative analysis documented that smooth muscle fibers were significantly smaller (P = 0.04) in PBS fetuses (9.67% to 17.75%, mean = 13.2%) compared to control group (13.33% to 26.56%, mean = 17.43%). The analysis of collagen fibers showed predominance of green in the control group, suggesting collagen type III presence, and predominance of red in the in PBS fetal bladders, suggesting collagen type I presence in this group. The qualitative analysis of the nerves with immunohistochemistry with tubulin showed predominance of nerves in the control group. CONCLUSION: The bladder in PBS had lower concentrations of smooth muscle fibers, collagen type III, and nerves. These structural alterations can be one of the factors involved in urinary tract abnormality such as distended bladder in patients with PBS.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1706
[Cu] Class update date: 170609
[Lr] Last revision date:170609
[St] Status:Publisher
[do] DOI:10.1002/nau.23327


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