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1.
Pediatr Nephrol ; 2024 Aug 16.
Artículo en Inglés | MEDLINE | ID: mdl-39150522

RESUMEN

We report on an infant with features of intermittent obstructive uropathy, acute kidney injury, hypertension and type 4 renal tubular acidosis (RTA) despite urethral catheterisation and fluid resuscitation. Radiological findings showed upper tract dilatation, likely bilateral vesicoureteric junction obstruction and bladder base thickening which was concerning for possible malignancy. Renal biopsy demonstrated eosinophilic infiltrate, suggestive of kidney involvement. Bladder biopsy was diagnostic for eosinophilic cystitis (EC) showing mature degranulating eosinophils. EC is a rare, easily treatable and important differential of bladder mass in children which may present with an atypical obstructive uropathy. This report adds to the limited literature of this condition within the paediatric population. EC should be considered early in children presenting with eosinophilia, urinary tract obstruction and kidney dysfunction, with uncertain aetiology. This case also highlights the need for detailed imaging, including visualisation of the bladder base, in cases of likely obstructive uropathy.

2.
Am J Pathol ; 192(6): 943-955, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35358476

RESUMEN

Bladder exstrophy (BEX) is a rare developmental abnormality resulting in an open, exposed bladder plate. Although normal bladder urothelium is a mitotically quiescent barrier epithelium, histologic studies of BEX epithelia report squamous and proliferative changes that can persist beyond surgical closure. The current study examined whether patient-derived BEX epithelial cells in vitro were capable of generating a barrier-forming epithelium under permissive conditions. Epithelial cells isolated from 11 BEX samples, classified histologically as transitional (n = 6) or squamous (n = 5), were propagated in vitro. In conditions conducive to differentiated tight barrier formation by normal human urothelial cell cultures, 8 of 11 BEX lines developed transepithelial electrical resistances of more than 1000 Ω.cm2, with 3 squamous lines failing to generate tight barriers. An inverse relationship was found between expression of squamous KRT14 transcript and barrier development. Transcriptional drivers of urothelial differentiation PPARG, GATA3, and FOXA1 showed reduced expression in squamous BEX cultures. These findings implicate developmental interruption of urothelial transcriptional programming in the spectrum of transitional to squamous epithelial phenotypes found in BEX. Assessment of BEX epithelial phenotype may inform management and treatment strategies, for which distinction between reversible versus intractably squamous epithelium could identify patients at risk of medical complications or those who are most appropriate for reconstructive tissue engineering strategies.


Asunto(s)
Carcinoma de Células Escamosas , Vejiga Urinaria , Carcinoma de Células Escamosas/patología , Diferenciación Celular , Células Epiteliales/metabolismo , Humanos , Vejiga Urinaria/metabolismo , Urotelio/metabolismo
3.
Neurourol Urodyn ; 41(1): 448-455, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34888917

RESUMEN

AIM: To describe a technique for on-table urodynamic with ureteric occlusion (OUUO) and present a case series comparing bladder capacity and pressure measurements performed with and without ureteric occlusion in patients with gross vesico-ureteric reflux (grade ≥ IV VUR) to highlight the clinical utility. METHODS: We performed OUUO in seven patients who were being considered for surgical management of VUR and/or nephro-ureterectomy. We occluded the refluxing ureters using hysterosalpingogram catheters (HSG, single lumen 5.5 Fr catheter with 1.5 ml balloon), guidewires, and rigid cystoscope. A 6 Fr dual-lumen urodynamic (UD) catheter is then inserted into the bladder to perform OUUO. Bladder capacity and Compliance (C = ∆bladder volume/∆detrusor pressure) are recorded and compared with values obtained at standard UD (without ureteric occlusion). RESULTS: The age range of the seven patients was 2.0-15.5 years. The etiology for the gross VUR were posterior urethral valve (PUV) and neuropathic. The bladder capacity and compliance finding between UD and OUUO findings were comparable in five patients. However, there was a clinically significant difference in bladder capacity (20%-50% decrease) and compliance (50%-90%) between UD and OUUO findings in two out of seven patients. CONCLUSION: In the presence of gross VUR, OUUO allows for a potentially more accurate assessment of bladder capacity and compliance. OUUO should be considered when planning ureteric opening bulking/reimplantation and nephro-ureterectomy.


Asunto(s)
Uréter , Vejiga Urinaria Neurogénica , Reflujo Vesicoureteral , Niño , Humanos , Uréter/cirugía , Vejiga Urinaria , Vejiga Urinaria Neurogénica/complicaciones , Urodinámica , Reflujo Vesicoureteral/cirugía
4.
Pediatr Surg Int ; 38(5): 665-677, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35316841

RESUMEN

PURPOSE: Enterocystoplasty is adopted for patients requiring bladder augmentation, but significant long-term complications highlight need for alternatives. We established a protocol for creating a natural-derived bladder extracellular matrix (BEM) for developing tissue-engineered bladder, and investigated its structural and functional characteristics. METHODS: Porcine bladders were de-cellularised with a dynamic detergent-enzymatic treatment using peristaltic infusion. Samples and fresh controls were evaluated using histological staining, ultrastructure (electron microscopy), collagen, glycosaminoglycans and DNA quantification and biomechanical testing. Compliance and angiogenic properties (Chicken chorioallantoic membrane [CAM] assay) were evaluated. T test compared stiffness and glycosaminoglycans, collagen and DNA quantity. p value of < 0.05 was regarded as significant. RESULTS: Histological evaluation demonstrated absence of cells with preservation of tissue matrix architecture (collagen and elastin). DNA was 0.01 µg/mg, significantly reduced compared to fresh tissue 0.13 µg/mg (p < 0.01). BEM had increased tensile strength (0.259 ± 0.022 vs 0.116 ± 0.006, respectively, p < 0.0001) and stiffness (0.00075 ± 0.00016 vs 0.00726 ± 0.00216, p = 0.011). CAM assay showed significantly increased number of convergent allantoic vessels after 6 days compared to day 1 (p < 0.01). Urodynamic studies showed that BEM maintains or increases capacity and compliance. CONCLUSION: Dynamic detergent-enzymatic treatment produces a BEM which retains structural characteristics, increases strength and stiffness and is more compliant than native tissue. Furthermore, BEM shows angiogenic potential. These data suggest the use of BEM for development of tissue-engineered bladder for patients requiring bladder augmentation.


Asunto(s)
Ingeniería de Tejidos , Vejiga Urinaria , Animales , Colágeno , Matriz Extracelular , Humanos , Porcinos , Ingeniería de Tejidos/métodos , Vejiga Urinaria/cirugía , Procedimientos Quirúrgicos Urológicos
5.
Pediatr Surg Int ; 37(7): 951-956, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33683431

RESUMEN

AIM: Hypertension in children with abnormal kidneys often requires multiple antihypertensive agents (complex), or could present with complications (e.g. hypertensive encephalopathy). Our objective in this report is to evaluate blood pressure control following unilateral or bilateral laparoscopic native nephrectomy in children with renal hypertension. MATERIALS AND METHODS: Single-centre retrospective review of all children who underwent nephrectomy for management of hypertension over a recent study period (2008-2017) with post-operative follow-up of at least 3 years. We describe the association of age, primary kidney disease and blood pressure and its management including time to resolution following unilateral or bilateral nephrectomy. RESULTS: During the 9-year study period, 21 of 215 (9.8%) children underwent nephrectomy for management of hypertension. We included 19 children [6 with unilateral native nephrectomy (UNN) and 13 with bilateral native nephrectomy (BNN)] in this study as they continued with their follow-up at our centre. Out of the 19 children, 15 had laparoscopic retroperitoneoscopic nephrectomies and 4 had laparoscopic transperitoneal nephrectomies. Six children had unilateral nephrectomy and 13 children had bilateral nephrectomies [7 were pre-transplant (haemodialysis-6, peritoneal dialysis-1) and 6 were post-kidney transplant]. Fifteen of 19 children (79%) had complete resolution [5 UNN and 10 BNN] and 3 (16%) partial resolution [1 UNN and 2 BNN]. One patient with BNN was observed to have no change in blood pressure control. CONCLUSION: Our data demonstrate improved management of hypertension in 95% of the children. Nephrectomy could offer a reasonable treatment option for selected group of complex and complicated renal hypertension.


Asunto(s)
Presión Sanguínea/fisiología , Hipertensión/etiología , Enfermedades Renales/complicaciones , Laparoscopía/métodos , Nefrectomía/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Hipertensión/fisiopatología , Hipertensión/cirugía , Enfermedades Renales/diagnóstico , Enfermedades Renales/cirugía , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento
6.
J Urol ; 203(2): 413-419, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31518199

RESUMEN

PURPOSE: Intradetrusor botulinum toxin is an established part of the treatment pathway for pediatric patients with neurogenic bladder. We determined the urodynamic effect of single and multiple administrations of abobotulinum toxin A in pediatric patients with neurogenic bladder, and determined the urodynamic efficacy of abobotulinum toxin A in low compliance vs overactive bladders. MATERIALS AND METHODS: We conducted a single center retrospective review of all pediatric patients with neurogenic bladder treated with abobotulinum toxin A. Videourodynamic data on cystometric capacity, maximum neurogenic detrusor overactivity pressure and compliance were gathered before and after the first abobotulinum toxin A administration and after the last administration. Patients were divided into low compliance and overactive bladder groups depending on initial videourodynamics findings. Paired t-test was used to compare videourodynamic outcomes before vs after abobotulinum toxin A injection. The Mann-Whitney U test was used to compare bladder groups. RESULTS: A total of 30 patients were included in the study. Of these patients 15 (50%) received multiple abobotulinum toxin A injections. There were 16 patients (53%) with overactive bladder. Abobotulinum toxin A administration significantly improved cystometric capacity (p <0.0001) and maximum neurogenic detrusor overactivity (p=0.0001). Overall, compliance did not change significantly (p=0.25). There was no significant difference in urodynamic parameters between first and last abobotulinum toxin A injections. Improvement in cystometric capacity (p=0.05) and maximum neurogenic detrusor overactivity (p=0.25) was similar between the low compliance and overactive bladder groups. Compliance significantly improved in the low compliance group vs the overactive bladder group (p=0.016). CONCLUSIONS: Intradetrusor abobotulinum toxin A improves cystometric capacity and maximum neurogenic detrusor overactivity in pediatric patients with neurogenic bladder. This effect is maintained over multiple injections. Compliance is significantly improved in patients with low compliance bladder vs overactive bladder.


Asunto(s)
Toxinas Botulínicas Tipo A/administración & dosificación , Vejiga Urinaria Neurogénica/tratamiento farmacológico , Vejiga Urinaria Neurogénica/fisiopatología , Vejiga Urinaria Hiperactiva/tratamiento farmacológico , Vejiga Urinaria Hiperactiva/fisiopatología , Urodinámica , Administración Intravesical , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
8.
Pediatr Surg Int ; 31(5): 465-71, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25801417

RESUMEN

AIM: Right-sided congenital diaphragmatic hernia (R-CDH) occurs in 14 to 25% of all CDH cases. The current literature comparing the outcome of R-CDH vs left CDH (L-CDH) is inconsistent, with some studies reporting higher and others lower mortality in R-CDH compared to L-CDH. The aim of our multicentre study was to analyse characteristics and outcome of R-CDH. METHODS: We retrospectively reviewed the medical records of 178 consecutive infants with CDH who underwent surgical repair of CDH at three European tertiary pediatric surgical centres from three different countries between 2000 and 2009. The analysis focused on demographic data, morbidity and mortality in R-CDH compared with L-CDH. RESULTS: Out of a total of 178 children, 32 (18.0%) right-sided and 146 (82.0 %) left-sided cases of CDH were identified. Prenatal diagnosis was made in 8 R-CDH vs 67 L-CDH (25.0 vs 45.9%, p = 0.030). Median gestational age in R-CDH was 39 weeks (range 29-42 weeks) and 39 weeks in L-CDH (range 28-43 weeks, p = 0.943). Median birth weight in R-CDH was 3233 g (range 905-4480 g) and in L-CDH was 3060 g (range 1065-5240 g, p = 0.184). Major associated anomalies were present in 19 R-CDH vs 46 L-CDH (59.4 vs 31.5%, p = 0.003). Extracorporeal membrane oxygenation (ECMO) was required in 3 R-CDH vs 19 L-CDH (9.4 vs 13.0%, p = 0.571). A diaphragmatic patch was used in 13 R-CDH and 59 L-CDH (40.6 vs 40.4%, p = 0.982). Fundoplication for GERD was required in 1 R-CDH and 19 L-CDH (3.1 vs 13.0 %, p = 0.109). No significant differences were observed in recurrence rate (9.4 vs 8.9%, p = 0.933). Postoperative mortality rate was significantly higher in R-CDH compared to L-CDH (21.9 vs 8.2%, p = 0.023). In R-CDH, prenatal diagnosis and patch repair correlated with mortality by univariate regression (p = 0.005 and p = 0.019). CONCLUSION: This multicentre study shows that prenatal diagnosis and patch repair were associated with an increased mortality rate in R-CDH. However, the morbidity following repair of R-CDH was not significantly different from that in L-CDH in survivors.


Asunto(s)
Hernias Diafragmáticas Congénitas/epidemiología , Hernias Diafragmáticas Congénitas/cirugía , Herniorrafia/estadística & datos numéricos , Complicaciones Posoperatorias/epidemiología , Austria/epidemiología , Peso al Nacer , Diafragma/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Irlanda/epidemiología , Londres/epidemiología , Masculino , Diagnóstico Prenatal , Estudios Retrospectivos , Resultado del Tratamiento
9.
FASEB J ; 27(12): 4853-65, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23995291

RESUMEN

Functional smooth muscle engineering requires isolation and expansion of smooth muscle cells (SMCs), and this process is particularly challenging for visceral smooth muscle tissue where progenitor cells have not been clearly identified. Herein we showed for the first time that efficient SMCs can be obtained from human amniotic fluid stem cells (hAFSCs). Clonal lines were generated from c-kit(+) hAFSCs. Differentiation toward SM lineage (SMhAFSCs) was obtained using a medium conditioned by PDGF-BB and TGF-ß1. Molecular assays revealed higher level of α smooth muscle actin (α-SMA), desmin, calponin, and smoothelin in SMhAFSCs when compared to hAFSCs. Ultrastructural analysis demonstrated that SMhAFSCs also presented in the cytoplasm increased intermediate filaments, dense bodies, and glycogen deposits like SMCs. SMhAFSC metabolism evaluated via mass spectrometry showed higher glucose oxidation and an enhanced response to mitogenic stimuli in comparison to hAFSCs. Patch clamp of transduced hAFSCs with lentiviral vectors encoding ZsGreen under the control of the α-SMA promoter was performed demonstrating that SMhAFSCs retained a smooth muscle cell-like electrophysiological fingerprint. Eventually SMhAFSCs contractility was evident both at single cell level and on a collagen gel. In conclusion, we showed here that hAFSCs under selective culture conditions are able to give rise to functional SMCs.


Asunto(s)
Líquido Amniótico/citología , Diferenciación Celular , Linaje de la Célula , Células Madre Fetales/citología , Células Madre Multipotentes/citología , Miocitos del Músculo Liso/citología , Actinas/genética , Actinas/metabolismo , Potenciales de Acción , Proteínas de Unión al Calcio/genética , Proteínas de Unión al Calcio/metabolismo , Medios de Cultivo Condicionados/farmacología , Citoplasma/metabolismo , Citoplasma/ultraestructura , Proteínas del Citoesqueleto/genética , Proteínas del Citoesqueleto/metabolismo , Desmina/genética , Desmina/metabolismo , Células Madre Fetales/efectos de los fármacos , Células Madre Fetales/metabolismo , Células Madre Fetales/fisiología , Glucosa/metabolismo , Glucógeno/metabolismo , Humanos , Proteínas de Microfilamentos/genética , Proteínas de Microfilamentos/metabolismo , Células Madre Multipotentes/efectos de los fármacos , Células Madre Multipotentes/metabolismo , Células Madre Multipotentes/fisiología , Proteínas Musculares/genética , Proteínas Musculares/metabolismo , Miocitos del Músculo Liso/metabolismo , Miocitos del Músculo Liso/fisiología , Factor de Crecimiento Derivado de Plaquetas/farmacología , Factor de Crecimiento Transformador beta/farmacología , Calponinas
10.
J Pediatr Urol ; 2024 May 21.
Artículo en Inglés | MEDLINE | ID: mdl-38816324

RESUMEN

Anastomosis of catheterising channels (Mitrofanoff and ACE) to the skin can be a challenge. The Kelly VV plasty is a straightforward solution but has been described only as a point of technique. We used the previously described method with minor modification. The technique has been used in 14 patients, including 9 children and 5 adults for Mitrofanoff,ACE and Monti channels. At a median follow-up of 25 months all patients continue to catheterise; none have required revision surgery. The Kelly VV plasty is a potentially robust solution to the problem of skin anastomosis; technique merits wider adoption and evaluation.

11.
J Pediatr Urol ; 20(4): 729.e1-729.e7, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38719686

RESUMEN

INTRODUCTION AND AIM: Posterior urethral valves (PUV) are often associated with bladder dysfunction. Our primary aim was to investigate bladder status following primary valves resection to gather evidence of function and to guide early clinical management. PATIENTS AND METHODS: Between July 2015-2020, we prospectively evaluated bladder function of PUV infants. Primary outcomes measured were number of voids, voided volume (VV), post-void residual (PVR) and bladder capacity (BC). Statistical comparisons and descriptive analysis were carried out between groups using 2-tails T test and Chi square test using an IBM SPSS Statistics program version 25. RESULTS: Sixty-one infants were included. Median age at resection was 28 days (5 days-11 months). In thirty-eight patients (62%) diagnosis was suspected antenatally. Vesico-ureteric reflux (VUR) was present in 16 (26%). The 4-h observational study was performed at median 4 days following primary cold-knife valve resection and 1 day (range from 1 to 12 days) after catheter removal. Infants voided an average 6 times (2-13 voids). Bladder capacity was large in 20 (33%) and small in 4 (6.5%) patients with a median ratio BC/eBC = 1.2 (0.49-22.6). Median residual urine was 9 mls but with great variability among the cohort (0-121 mls). A big variability in bladder emptying was noted between patients and between voids performed in one patient. In 13 infants (21%) average PVR was larger than the average voided volume. In 26 (42.6%) PVR was larger than VV at least once. Thirty-eight infants (62.3%) emptied their bladders almost completely at least once (with PVR <5 mls). No difference was found in PVR, Median BC/EBC and PVR/BC between infants with and without VUR (p = 0.654, P: 0.594 and p = 00.481, respectively). DISCUSSION: We presented our experience of non-invasive bladder function assessment in infants affected by PUV following primary valve resection. An interesting data is the great variability identified both between patients and between voids performed in a single patient. Average number of voids was similar to the voiding pattern in healthy newborns what reported by Gladh but variability was greater. We observed up to 13 voids in some infants and we can speculate this could reflect the presence of an overactive bladder. We observed at least 1 complete emptying in only 62% of infants and 21% of the cohort had PVR bigger than VV. In almost half of the infants (42.6%) PVR was larger than VV at least once. CONCLUSIONS: Around 40% of infants affected by PUV have abnormal bladder capacity and almost half of them have significant post void residuals following primary resection. Although controversies and limitations are present, we believe that this non-invasive study can provide valuable information to understand the dynamic of the bladder, particularly in children affected by PUV and allow early intervention in children considered "at risk". Having a non invasive way of assessing can help tailor intervention and be useful for future research into early bladder intervention and improving outcomes.


Asunto(s)
Uretra , Micción , Humanos , Lactante , Uretra/anomalías , Uretra/cirugía , Masculino , Estudios Prospectivos , Recién Nacido , Micción/fisiología , Factores de Tiempo , Vejiga Urinaria/cirugía , Vejiga Urinaria/anomalías , Vejiga Urinaria/fisiopatología , Urodinámica/fisiología
12.
J Pediatr Urol ; 20(3): 522-525, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38360426

RESUMEN

This educational article highlights the critical role of pediatric urology nursing research in improving the care of children with urological conditions. It discusses the multifaceted nature of pediatric urology nursing, addresses challenges such as limited nurse scientists and resource constraints, and highlights the need to overcome barriers to increase research involvement. The authors emphasize the importance of prioritizing research areas, the promotion of collaboration, and the provision of adequate funding and academic time for pediatric nurses to contribute to evidence-based practice, to improve patient outcomes. Furthermore, it highlights the importance of research in advancing nursing practice, shaping protocols, and advocating for the rights and needs of children with urological conditions and their families.


Asunto(s)
Investigación en Enfermería , Enfermería Pediátrica , Mejoramiento de la Calidad , Humanos , Niño , Urología , Enfermedades Urológicas/terapia , Enfermedades Urológicas/enfermería
13.
J Pediatr Urol ; 20(2): 240.e1-240.e8, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37957075

RESUMEN

INTRODUCTION: Paediatric urologists manage a spectrum of conditions, much of the evidence for relevant treatment pathways is of low quality. For many conditions treatment varies according to location and surgeon; children with the same condition might have surgery in one unit but watchful waiting in another. Underlying this variation are differences in opinion, and insufficient high-level evidence with few prospective randomized studies. Such studies may be challenging to design, fund and recruit into, and are more likely to succeed if there is a collaborative approach. Research prioritization is a tool to identify the research of most value. Delphi methodology is an interpretive technique aiming to gain the consensus view of interested parties. The British Association of Paediatric Urologists (BAPU) set out to ascertain consensus on what paediatric urologists, working in the UK, consider to be areas of priority for research. This paper describes the process used, and the resulting list of research questions. METHODS: A scoping survey of paediatric urologists in the UK was undertaken to identify an initial set of research questions. These were refined by the BAPU research committee (BAPU RC), then prioritized using a modified Delphi process. During Stage 1a multiple new research questions were submitted leading to Stage 1b, an interim process. All UK paediatric urologists were invited to take part in Stage 2 of the prioritization process. RESULTS: Sixty-five questions were submitted to the scoping survey by 24 paediatric urologists. The BAPU RC refined these to 60 questions, which were submitted to Stage 1a of the modified Delphi process. Sixty-seven people completed Stage 1a, at the same time submitting 224 additional research questions. The BAPU RC revised the entire question set, ensuring the key subject of the original question was not altered and novel questions were retained. The BAPU RC undertook interim scoring of the resultant 79 questions, the top scoring 25 questions plus 5 lower scoring 'wild card' questions (to ensure the breadth of the specialty was represented) were put forward to Stage 2. A total of 65 people completed Stage 2, including a lay representative. A list of 30 priority research questions was generated; the top 10 includes management of neuropathic bladder, posterior urethral valves, antibiotic prophylaxis, DSD & CAH, continence, male external genitalia, VUR and transition care (Table). CONCLUSION: This process has provided BAPU, paediatric urologists in the UK, and funders with areas of research considered a priority in the specialty.

14.
J Pediatr Urol ; 20(2): 312-314, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38199905

RESUMEN

Why and when is animal experimentation relevant? The answer to this question depends on the research question. In this short educational article we aim to raise awareness of the importance of formulating a very specific research question before choosing an animal species. An awareness of anatomical and physiological differences vis-a-vis similarities between species, will increase the potential for obtaining data that is relevant for translation to human conditions.


Asunto(s)
Experimentación Animal , Urología , Animales , Niño , Humanos
15.
J Pediatr Urol ; 20(2): 315-317, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38238242

RESUMEN

When performing animal experimentation in Pediatric Urology studies, it is important to be aware of physiological differences between species and to understand when relevant disease models are available. Diseased animal models may be more relevant in many cases, rather than performing studies in healthy and normally developed animals. For example, they may be more appropriate for the study of congenital malformations, to investigate the secondary effects of prenatal urinary obstruction, to study the effect of prenatal exposure to endogenous or exogenous factors which may lead to disease, or in testing bioengineered structures. In this short educational article, we aim to describe some disease models that have been used to simulate human pathologies and how, if properly designed, these studies can lead to important new knowledge for human translation. In addition, we also highlight the importance of formulating a research question(s) before deciding on the animal experimental model and species to choose.


Asunto(s)
Experimentación Animal , Urología , Animales , Humanos , Niño , Modelos Animales
16.
Ann Surg ; 258(6): 895-900, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23604057

RESUMEN

OBJECTIVE: We aimed to evaluate the effect of thoracoscopy in neonates on intraoperative arterial blood gases, compared with open surgery. BACKGROUND: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be repaired thoracoscopically, but this may cause hypercapnia and acidosis, which are potentially harmful. METHODS: This was a pilot randomized controlled trial. The target number of 20 neonates (weight > 1.6 kg) were randomized to either open (5 CDH, 5 EA/TEF) or thoracoscopic (5 CDH, 5 EA/TEF) repair. Arterial blood gases were measured every 30 minutes intraoperatively, and compared by multilevel modeling, presented as mean and difference (95% confidence interval) from these predictions. RESULTS: Overall, the intraoperative PaCO2 was 61 mm Hg in open and 83 mm Hg [difference 22 mm Hg (2 to 42); P = 0.036] in thoracoscopy and the pH was 7.24 in open and 7.13 [difference -0.11 (-0.20 to -0.01); P = 0.025] in thoracoscopy. The duration of hypercapnia and acidosis was longer in thoracoscopy compared with that in open. For patients with CDH, thoracoscopy was associated with a significant increase in intraoperative hypercapnia [open 68 mm Hg; thoracoscopy 96 mm Hg; difference 28 mm Hg (8 to 48); P = 0.008] and severe acidosis [open 7.21; thoracoscopy 7.08; difference -0.13 (-0.24 to -0.02); P = 0.018]. No significant difference in PaCO2, pH, or PaO2 was observed in patients undergoing thoracoscopic repair of EA/TEF. CONCLUSIONS: This pilot randomized controlled trial shows that thoracoscopic repair of CDH is associated with prolonged and severe intraoperative hypercapnia and acidosis, compared with open surgery. These findings do not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH, calling into question the safety of this practice. The effect of thoracoscopy on blood gases during repair of EA/TEF in neonates requires further evaluation. (ClinicalTrials.gov Identifier: NCT01467245).


Asunto(s)
Acidosis/etiología , Atresia Esofágica/cirugía , Hernias Diafragmáticas Congénitas , Hipercapnia/etiología , Complicaciones Intraoperatorias/etiología , Toracoscopía/efectos adversos , Femenino , Hernia Diafragmática/cirugía , Humanos , Recién Nacido , Masculino , Proyectos Piloto , Estudios Prospectivos , Procedimientos Quirúrgicos Operativos/métodos
17.
Pediatr Surg Int ; 29(2): 185-9, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23143132

RESUMEN

PURPOSE: Morgagni diaphragmatic hernia can be repaired laparoscopically. The aim of this study is to evaluate the outcome of this minimally invasive approach. METHODS: A retrospective review was conducted on all consecutive children who underwent repair of Morgagni hernia from January 2002 to December 2011 in our hospital. Data are expressed as median (range). RESULTS: There were 12 children with Morgagni hernia. Age at surgery was 7.5 months (2-125). Associated malformations were present in 7 children (58 %). All children underwent initial laparoscopic approach. Two children (16 %) underwent conversion to open surgery. The hernia was closed primarily in 11 children (92 %), using a polyester patch in 1 (8 %). There were no intraoperative or immediate postoperative complications. Five children (42 %), all repaired initially without a patch, had a recurrence of the Morgagni hernia. The repair of the recurrent hernia was performed laparoscopically in four out of the five children, and a patch was used in two patients with no further recurrences or complications. CONCLUSIONS: There is a high rate of recurrence after laparoscopic Morgagni hernia repair. This is exclusively associated with laparoscopic repair without patch, and it is in contrast with the low recurrence rate reported previously. More frequent use of patch may be beneficial.


Asunto(s)
Hernia Diafragmática/cirugía , Herniorrafia/métodos , Laparoscopía/métodos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Niño , Preescolar , Femenino , Herniorrafia/estadística & datos numéricos , Humanos , Lactante , Laparoscopía/estadística & datos numéricos , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos/estadística & datos numéricos , Recurrencia , Estudios Retrospectivos , Mallas Quirúrgicas/estadística & datos numéricos , Resultado del Tratamiento
18.
Pediatr Surg Int ; 29(1): 87-95, 2013 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23124129

RESUMEN

PURPOSE: Tissue engineering of the oesophagus has been proposed as a therapeutic alternative to organ transplantation. We previously demonstrated that a detergent enzymatic treatment (DET) is a valid method to obtain an acellular matrix with preservation of the native architecture. In this study, we aimed to develop a natural acellular matrix from pig oesophagus, as a valid framework for oesophageal replacement. METHODS: Pig oesophagi (n = 4) were decellularized with continuous luminal infusion of DET. To evaluate the efficiency of the decellularization, samples were assessed by histology and DNA quantification. Moreover, the ultra-structural characteristics of the acellular matrix were investigated by scanning electron microscopy (SEM) and transmission electron microscopy (TEM). RESULTS: Decellularization of the oesophagus was achieved after three cycles of DET. Histological analysis showed the maintenance of tissue matrix architecture with absence of cellular elements, verified by measurement of DNA. SEM and TEM analysis confirmed preservation of the ultra-structural characteristics of the native tissue. CONCLUSIONS: Oesophageal acellular matrix can be successfully obtained by decellularization of pig oesophagus using a gentle DET via the oesophageal lumen. This decellularization method preserves the ultrastructure of the native tissue and could represent the basis for a tissue-engineered oesophagus.


Asunto(s)
Detergentes , Esófago/cirugía , Ingeniería de Tejidos/métodos , Animales , Enzimas , Medicina Regenerativa , Porcinos
19.
J Pediatr Urol ; 19(6): 700.e1-700.e10, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37775457

RESUMEN

INTRODUCTION: Foreskin reconstruction (FR) at the time of primary hypospadias repair is a truly anatomically complete reconstruction of the hypospadic penis. We prospectively collected penile and preputial measurement of children undergoing single-stage hypospadias repair and FR with the aim to identify possible relations between penile and preputial anatomy and the likelihood to develop complications. MATERIALS AND METHODS: We prospectively studied children who underwent single stage hypospadias repair associated with FR from 2016 to 2019. We recorded intra-operative foreskin and penile measurements and post-operative outcomes. Logistic Regression analysis was performed to explore independent factors affecting urethroplasty and skin complications. Chi square test was used to compare outcomes in different groups based on ventral foreskin defect (VFD) width, Glans size, age at surgery and meatal location. RESULTS: From a total of 181 consecutive patients, 86 boys who underwent a single stage hypospadias repair combined with FR were included in the study. Patients were excluded because they were either lost at follow up (n = 10), required a 2-stage repair (n = 2), were circumcised at birth (n = 3) or parents requested a circumcision (n = 78); in 2 patients, a decision to perform circumcision was made intraoperatively due to aesthetic reasons (monk-hood deformity of the prepuce). Median age at surgery was 17 months. Mean glans width was 14.4 mm. Mean unstretched and stretched foreskin circumference were 29.5 mm and 40.9 mm, respectively. Mean VFD (the distance between the proximal insertion of the foreskin hood on either side of the midline at the level of the coronal sulcus) was 7.2 mm (Fig. 1). At median follow-up of 8 months (6-23), 9 complications were recorded (10.4%): foreskin dehiscence occurred in 1% (1/86), a foreskin fistula was noted in 4.6% (4/86), tight, non-retractile, foreskin in 1% (1/86); urethrocutaneous fistula in 2.3% (2/86) and complete dehiscence of the glans and foreskin in 1 (1.2%). Multiple logistic regression analysis demonstrated that none of the measurements obtained was an independent risk factor for developing urethroplasty or skin complications. There was no significant difference in complications between wide VFD (>7 mm) vs. narrow VFD (≤7 mm), large glans (>14 mm) vs. small glans (≤14 mm), age at surgery ≤24 months vs. > 24 month and meatal location distal (glanular, coronal, subcoronal and distal penile) vs. proximal (midpenile, proximal penile and penoscrotal). CONCLUSION: To the best of our knowledge, this is the first study reporting a prospective and objective assessment of the foreskin in the context of single stage hypospadias repair. Individual anatomical differences in preputial and penile anatomy do not seem to affect the likelihood of skin and urethroplasty complications. FR can, therefore, be offered to all boys undergoing primary single stage hypospadias repair . Further studies on larger numbers and external validation of these measurements is necessary.


Asunto(s)
Hipospadias , Masculino , Niño , Recién Nacido , Humanos , Lactante , Preescolar , Hipospadias/cirugía , Hipospadias/etiología , Prepucio/cirugía , Estudios Prospectivos , Resultado del Tratamiento , Procedimientos Quirúrgicos Urológicos Masculinos/efectos adversos , Uretra/cirugía , Estudios Retrospectivos
20.
Afr J Paediatr Surg ; 20(1): 51-58, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36722570

RESUMEN

Aim: The management of a complicated duplex kidney remains a challenge for paediatric urologists. The aim of this study is to report a 10-year experience of the surgical management of complicated duplex kidney in a single tertiary care paediatric referring hospital. Materials and Methods: Clinical records of all children who undergone a surgical procedure for complicated duplex systems between January 2009 and March 2019 at our institution were retrospectively reviewed. Clinical manifestations, surgical procedures, complications and follow-up were collected and analysed. Logistic regression was performed to explore if any patient's characteristic or underlying associated comorbidity (ureterocoele, ectopic ureter, obstruction, etc.,) could be positively linked to the chance to develop recurrent urinary tract infections (UTIs). Results: We have identified 95 children who received a surgical treatment for 102 complicated duplex kidneys. The presence of an ureterocoele was recorded in 41 (43.2%) patients, an ectopic ureter in 25 (26.3%), a vesicoureteral reflux (VUR) in 40 (42.1%), a vesicoureteric junction obstruction in 24 (25.3%) and an ureteropelvic junction obstruction in 3 (3.2%). An invasive approach such as an heminephrectomy (71.6%) was required in the majority of cases. Higher risk of developing a UTI has been demonstrated in children diagnosed postnatally (P < 0.001) and in those with an associated obstruction (P < 0.05). Conclusions: No standardised management could be recommended for the surgical treatment of complicated duplex kidney. Children without antenatal diagnosis and with either an upper tract obstruction or VUR are at greater risk to develop UTI and need to be looked after more closely.


Asunto(s)
Obstrucción Ureteral , Ureterocele , Embarazo , Humanos , Femenino , Niño , Estudios Retrospectivos , Centros de Atención Terciaria , Nefrectomía , Obstrucción Ureteral/etiología , Obstrucción Ureteral/cirugía , Riñón/cirugía
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