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1.
Surg Endosc ; 2024 Jul 17.
Artículo en Inglés | MEDLINE | ID: mdl-39020118

RESUMEN

BACKGROUND: This study aimed to analyze the results, feasibility and safety of the thoracoscopic approach for patients with esophageal atresia with tracheoesophageal fistula (EA/TEF) depending on the patient's birth weight. METHODS: The study involved only type C and D EA/TEF. Among the analyzed parameters were the patients' characteristics, surgical treatment and post-operative complications: early mortality, anastomosis leakage, anastomosis strictures, chylothorax, TEF recurrence, and the need for fundoplication or gastrostomy. RESULTS: 145 consecutive newborns underwent thoracoscopic EA with TEF repair. They were divided into three groups-A (N = 12 with a birth weight < 1500 g), B (N = 23 with a birth weight ≥ 1500 g but < 2000 g), and C-control group (N = 110 with a birth weight ≥ 2000 g). Primary one-stage anastomosis was performed in 11/12 (91.7%) patients-group A, 19/23 (82.6%)-group B and 110 (100%)-group C. Early mortality was 3/12 (25%)-group A, 2/23 (8.7%)-group B, and 2/110 (1.8%)-group C and was not directly related to the surgical repair. There were no significant differences in operative time and the following complications: anastomotic leakage, recurrent TEF, esophageal strictures, and chylothorax. There were no conversions to an open surgery. Fundoplication was required in 0%-group A, 4/21 (19.0%)-group B, and 2/108 (1.9%)-group C survivors. Gastrostomy was performed in 1/9 (11.1%)-group A, 3/21 (14.3%)-group B and 0%-group C. CONCLUSION: In an experienced surgeon's hands, even in the smallest newborns, the thoracoscopic approach may be safe, feasible, and worthy of consideration. Birth weight seems to be not a direct contraindication to the thoracoscopic approach.

2.
J Pediatr Nurs ; 50: e85-e90, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-31027866

RESUMEN

PURPOSE: Most of the research in the field of esophageal atresia (EA) is focused on diagnostic problems and surgery. There is scarce literature addressing the impact of EA on the lives of families of patients. The aim of this paper is to investigate whether the presence of underlying associated malformations, disease-specific feeding problems and prematurity would have a significant influence on the family of a child after surgical repair of EA. DESIGN AND METHODS: The study sample consisted of 73 participants who were parents of children after surgery of EA. The impact of EA on families was assessed using an Authors-Designed Questionnaire (ADQ) to collect medical and sociodemographic background data as well as standardized questionnaire: the PedsQL™ Family Impact Module (PedsQL-FIM). RESULTS: The presence of cardiac impairment significantly (p = 0.037) affects the functioning of the family in the emotional domain. The coexistence of skeletal impairment seems to have the greatest impact on the functioning of the family, three statistically significant correlations have been demonstrated: (p = 0.021) - in the social domain, (p = 0.009) - in the cognitive domain and (p = 0.023) - in the domain of communication. The families of patients with tracheoesophageal fistula (TEF) had the statistically lower (p < 0.05) score of functioning in the emotional domain than those with children without TEF. CONCLUSION: Feeding problems and the presence of associated anomalies significantly affect the functioning of the family of the child with EA.


Asunto(s)
Atresia Esofágica/psicología , Relaciones Familiares/psicología , Trastornos de Alimentación y de la Ingestión de Alimentos/psicología , Calidad de Vida , Anomalías Múltiples/psicología , Adolescente , Adulto , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Padres/psicología , Complicaciones Posoperatorias/psicología , Encuestas y Cuestionarios , Fístula Traqueoesofágica/psicología
4.
World J Urol ; 34(7): 939-48, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26577623

RESUMEN

PURPOSE: To compare the outcome of laparoscopic and retroperitoneoscopic approach for partial nephrectomy in infants and children with duplex kidneys. METHODS: Data of 102 patients underwent partial nephrectomy in a 5-year period using MIS procedures were analyzed. Fifty-two children underwent laparoscopic partial nephrectomy (LPN), and 50 children underwent retroperitoneoscopic partial nephrectomy (RPN). Median age at surgery was 4.2 years. Statistical analysis was performed using χ (2) test and Student's t test. RESULTS: The overall complications rate was significantly higher after RPN (15/50, 30 %) than after LPN (10/52, 19 %) [χ (2) = 0.05]. In LPN group, complications [4 urinomas, 2 symptomatic refluxing distal ureteral stumps (RDUS) and 4 urinary leakages] were conservatively managed. In RPN group, complications (6 urinomas, 8 RDUS, 1 opening of remaining calyxes) required a re-operation in 2 patients. In both groups no conversion to open surgery was reported. Operative time (LPN:166.2 min vs RPN: 255 min; p < 0.001) and hospitalization (LPN: 3.5 days vs RPN: 4.1 days; p < 0.001) were significantly shorter in LPN group. No postoperative loss of renal function was reported in both groups. CONCLUSIONS: Our results demonstrate that RPN remains a technically demanding procedure with a significantly higher complications and re-operation rate compared to LPN. In addition, length of surgery and hospitalization were significantly shorter after LPN compared to RPN. LPN seems to be a faster, safer and technically easier procedure to perform in children compared to RPN due to a larger operative space and the possibility to perform a complete ureterectomy in refluxing systems.


Asunto(s)
Riñón/anomalías , Riñón/cirugía , Laparoscopía , Nefrectomía/métodos , Niño , Preescolar , Femenino , Encuestas de Atención de la Salud , Humanos , Lactante , Masculino , Espacio Retroperitoneal , Estudios Retrospectivos
5.
Acta Chir Belg ; 116(2): 89-95, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-27385295

RESUMEN

BACKGROUND: Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. METHODS: A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. RESULTS: Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. CONCLUSIONS: Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.


Asunto(s)
Obstrucción Duodenal/cirugía , Obstrucción de la Salida Gástrica/etiología , Obstrucción de la Salida Gástrica/cirugía , Píloro/anomalías , Anastomosis Quirúrgica , Diagnóstico Diferencial , Obstrucción Duodenal/diagnóstico , Femenino , Estudios de Seguimiento , Obstrucción de la Salida Gástrica/complicaciones , Obstrucción de la Salida Gástrica/diagnóstico , Humanos , Recién Nacido , Atresia Intestinal , Laparotomía/métodos , Masculino , Diagnóstico Prenatal , Píloro/cirugía , Estudios Retrospectivos , Medición de Riesgo , Muestreo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Ultrasonografía Prenatal/métodos
6.
Dev Period Med ; 20(2): 134-8, 2016.
Artículo en Polaco | MEDLINE | ID: mdl-27442698

RESUMEN

Hematoma duodenum is a very rare complication of diagnostic endoscopy of the upper gastrointestinal tract when biopsy of the duodenum is performed (average frequency is estimated as 1:1,250 biopsies). Most often, it affects children and young adults without any risk factors. Symptoms result from obstruction of the duodenum and compression of the adjacent structures. Conservative treatment, which consists of parenteral nutrition and aspiration of gastric contents until the absorption of hematoma and patency of the gastrointestinal tract returns, is preferred. This paper describes a 6-year-old boy diagnosed due to short stature and low weight in whom the diagnostic biopsy of the duodenum caused formation of a hematoma in the descending duodenum and led to total ileus and acute pancreatitis. The boy was treated conservatively with good result and complete resolution of symptoms was achived.


Asunto(s)
Biopsia/efectos adversos , Obstrucción Duodenal/etiología , Duodeno/lesiones , Hematoma/etiología , Pancreatitis/etiología , Niño , Obstrucción Duodenal/terapia , Hematoma/terapia , Humanos , Mucosa Intestinal/lesiones , Masculino , Pancreatitis/terapia
7.
Surg Endosc ; 29(12): 3469-76, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25673347

RESUMEN

BACKGROUND: We aim to report a 5-year retrospective multicentric European survey about the outcome of laparoscopic partial nephrectomy in infants and children with duplex kidneys. METHODS: The data of fifty-two children underwent laparoscopic partial nephrectomy (42 upper-pole nephrectomies and 10 lower-pole nephrectomies) in six European centers of Pediatric Surgery, were collected and analyzed. Median age at surgery was 5.1 years (range 6 months-9.7 years). There were 32 girls and 20 boys. In 37 patients, the left side was affected and in 15 patients the right side. For the right side, 4 trocars were used and for the left side 3/4 trocars. Special hemostatic devices were used for dissection and parenchymal section in all centers. We assessed intraoperative and postoperative morbidity. RESULTS: Median length of surgery was 166.2 min (70-215 min). No conversion to open surgery nor intraoperative bleeding was reported. Mean hospitalization was 3.5 days. We recorded 10/52 complications (4 urinomas, 2 recurrent UTIs, 4 prolonged urinary leakage), all managed conservatively. Reoperation rate was 0%. No loss of renal function on the residual kidney moiety was recorded in all operated patients. CONCLUSIONS: Laparoscopic partial nephrectomy remains a technically challenging procedure performed only in pediatric centers with high experience in minimally invasive surgery. Although the median operative time was higher than 2 h, we recorded no conversions in our series. The complication rate remains high (10/52-19.2%). All were II grade complications according to Clavien-Dindo classification and were treated conservatively without the need of other surgical procedures.


Asunto(s)
Enfermedades Renales/cirugía , Riñón/anomalías , Laparoscopía/métodos , Nefrectomía/métodos , Niño , Preescolar , Conversión a Cirugía Abierta/estadística & datos numéricos , Europa (Continente) , Femenino , Humanos , Lactante , Riñón/cirugía , Tiempo de Internación , Masculino , Tempo Operativo , Complicaciones Posoperatorias/etiología , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Especialidades Quirúrgicas , Encuestas y Cuestionarios
8.
Pol Merkur Lekarski ; 39(230): 109-10, 2015 Aug.
Artículo en Polaco | MEDLINE | ID: mdl-26319386

RESUMEN

A case of a 4.5-year-old girl from a twin pregnancy, who was diagnosed after birth with the congenital esophageal atresia (type 3), and at the age of 4 with the potential coeliac disease. Congenital esophageal atresia was successfully treated surgically in infancy with the thoracoscopic method. The potential coeliac disease was detected in the child with a correct histopathological examination of intestinal villi and showing no enteropathy symptoms based on the presence of antibodies against tissue transglutaminase and against endomysial antibodies of smooth muscles in serum and the presence of HLA DQ2.5. In the treatment of the potential coeliac disease the girl followed a gluten-free diet.


Asunto(s)
Enfermedad Celíaca/complicaciones , Enfermedades en Gemelos/diagnóstico , Enfermedades en Gemelos/terapia , Atresia Esofágica/complicaciones , Enfermedad Celíaca/dietoterapia , Enfermedad Celíaca/patología , Preescolar , Atresia Esofágica/cirugía , Femenino , Humanos , Toracoscopía/métodos
9.
Pol J Radiol ; 79: 279-82, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25191530

RESUMEN

BACKGROUND: Hemobilia in children is a rare phenomenon which has been described mostly in the context of traumas. The descriptions of massive hemobilia in children after liver biopsy are a rarity in the scientific literature because there are only a few examples of it. Hemobilia rarely develops spontaneously. Generally, this is a complication after a blunt abdominal trauma or after medical (especially surgical) procedures. Correct diagnosis and treatment of hemobilia are essential, especially in the case of patients with severe - sometimes life-threatening - haemorrhage from biliary ducts. It should be remembered that the symptoms of hemobilia do not necessarily occur immediately after surgery or trauma. In some cases hemobilia occurs after a changeable, asymptomatic period of time. CASE REPORT: We would like to present a case of a severe form of hemobilia caused by arterio-biliary fistula which developed incidentally after liver biopsy in a 10-year-old boy with chronic hepatitis B. Symptoms of hemobilia appeared on the seventh day after the diagnostic biopsy when the patient's general condition began to deteriorate. The diagnosis of arterio-biliary fistula was established after angio-CT examination of the liver. A selective embolization of the right hepatic artery was carried out. Hemobilia in children is a rare phenomenon which has been described mostly in the context of traumas. The cases of massive hemobilia in children after liver biopsy are a rarity in the scientific literature because there are only a few examples of it. Hemobilia very rarely develops spontaneously. Generally, this is a complication after a blunt abdominal trauma or after medical (especially surgical) procedures. RESULTS: Correct diagnosis and treatment of hemobilia are essential, especially in the case of patients with severe - sometimes life-threatening - haemorrhage from biliary ducts. It should be remembered that the symptoms of hemobilia do not necessarily occur immediately after surgery or trauma. In some cases hemobilia occurs after a changeable, asymptomatic period of time.

10.
Eur J Pediatr Surg ; 34(1): 36-43, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38154482

RESUMEN

OBJECTIVE: Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers. METHODS: We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared. RESULTS: The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis-Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively-all patients had a contrast study-and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B. CONCLUSION: Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.


Asunto(s)
Atresia Esofágica , Lactante , Humanos , Atresia Esofágica/complicaciones , Tracción/métodos , Resultado del Tratamiento , Fuga Anastomótica/etiología , Anastomosis Quirúrgica/efectos adversos
11.
J Pediatr Surg ; 2024 Mar 16.
Artículo en Inglés | MEDLINE | ID: mdl-38594136

RESUMEN

INTRODUCTION: Thoracic musculoskeletal deformities are significant complications following open correction of esophageal atresia (EA) during long-term follow-up. We aimed to compare the frequency and severity of thoracic musculoskeletal deformities after open and thoracoscopic repair of EA. We hypothesized that fewer deformities would occur following the less invasive thoracoscopic approach. METHODS: This retrospective study analyzed patients treated at two pediatric surgery departments in Poland between 2005 and 2021. The patient groups differed in surgical approach, operative techniques, indications for multi-staged surgery, and postoperative complications. The study encompassed all types of EA/TEF. The first group comprised 68 patients who underwent thoracoscopic esophageal atresia repair (Wroclaw), while the second group involved 44 patients who underwent open repair (Warsaw). Clinical data were retrospectively reviewed, with results considered significant at p < 0.05. RESULTS: The median age at examination was 6 years in the thoracoscopy group and 5.5 years in the thoracotomy group. In the thoracoscopy group, 53 out of 68 patients (77.9%) and in the thoracotomy group - 35 out of 44 patients (79.5%) were treated in one stage. The incidence of thoracic musculoskeletal deformities was significantly lower in the thoracoscopy group (1.5%) compared to the thoracotomy group (34.1%, p < 0.001). Scoliosis occurred significantly more often after thoracotomy (13.6% vs 1.5%, p = 0.016). There was no rib fusion (0% vs 37.1%, p < 0.001) and no scoliosis of =>20° (0% vs 6.8%, p = 0.058) after thoracoscopy. The coincidence of rib fusion and scoliosis was significant (9.1%, p = 0.022) for the open approach. In the thoracotomy group, multi-staged surgery and more frequent reoperations due to major complications were significantly associated with an increased occurrence of deformities. None of the patients after thoracoscopic multi-stage or complicated EA/TEF repair developed scoliosis. CONCLUSIONS: The frequency and severity of thoracic musculoskeletal deformities were significantly lower after the thoracoscopic approach. Thoracoscopy may be a more advantageous and preferred surgical approach for the EA/TEF treatment, although further randomized, controlled studies are necessary. Post-thoracotomy scoliosis may progress to a severity requiring surgery.

12.
J Clin Med ; 13(7)2024 Apr 04.
Artículo en Inglés | MEDLINE | ID: mdl-38610874

RESUMEN

Introduction: Etiopathogenesis and the symptomatology of ureteropelvic junction obstruction (UPJO) in the pediatric population has not yet been definitely clarified, suggesting a multifactorial nature of the condition. The aim was to analyze the association between the number of Interstitial Cells of Cajal (ICCs), as well as P2X3 receptors in ureteropelvic junction (UPJ) and the pain response in pediatric patients with hydronephrosis. Methods: 50 patients with congenital hydronephrosis underwent open or laparoscopic pyeloplasty at one of two departments of pediatric surgery and urology in Poland. Patients were divided into two groups according to the pain symptoms before surgery. A total of 50 samples of UPJ were obtained intraoperatively and underwent histopathological and immunohistochemical (IHC) analysis. Quantitative assessment of ICCs was based on the number of CD117(+) cells of adequate morphology in the subepithelial layer and the muscularis propria. Expression of P2X3 receptors was evaluated as the intensity of IHC staining. Results: Patients with hydronephrosis and accompanying pain were on average 60 months older (77 vs. 17 months) than children with asymptomatic hydronephrosis (p = 0.017). Symptomatic children revealed higher numbers of ICCs in both the subepithelial layer and in the lamina muscularis propria. In particular, symptomatic patients aged 2 years or more exhibited significantly higher numbers of ICCs in the subepithelial layer. Significant differences in the distribution of ICCs between the subepithelial layer and the lamina muscularis propria were observed in both groups. Expression of P2X3 receptors was limited to the urothelium and the muscle layer and correlated between these structures. There was no relationship between pain response and the expression of P2X3 receptors. Conclusions: ICCs and P2X3 receptors may participate in the pathogenesis of UPJO and in the modulation of pain response to a dilatation of the pyelocaliceal system. Explanation of the role of ICCs and P2X3 receptors in propagation of ureteral peristaltic wave and the modulation of pain stimuli requires further studies.

13.
J Pediatr Surg ; 58(2): 204-208, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36402593

RESUMEN

Almost 23 years have passed since the first thoracoscopic procedure was done. However, according to the recent study in USA, only 16% of newborns with oesophageal atresia and distal tracheooesophageal fistula are managed by thoracoscopic approach with a very high 53% conversion rate. The aim of presentation based on experience of one paediatric surgery centre is to prove that thoracoscopic approach for this malformation is a powerful tool with a potential to change the current results. It requires considerable experience that comes only from the high number of operated cases. The technique is one amongst the many others means which taken together may really help to improve the results. Thus, the question of centralisation for special rare congenital malformations comes back to mind. Is it rational to operate on only 2 or 3 cases a year in a single centre, or is it the time for centralisation and close co-operation, as has been done with biliary atresia treatment in the UK? LEVEL OF EVIDENCE: III.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Niño , Recién Nacido , Humanos , Atresia Esofágica/cirugía , Toracoscopía/métodos , Fístula Traqueoesofágica/cirugía
14.
J Clin Med ; 12(23)2023 Nov 23.
Artículo en Inglés | MEDLINE | ID: mdl-38068303

RESUMEN

BACKGROUND: Recurrent tracheoesophageal fistula (RTEF) is usually a consequence of leakage or other complications after esophageal atresia repair performed through right-sided access. This results in extensive intrapleural adhesions, and open redo surgery poses a challenge. Alternatively, endoscopic endotracheal fistula obliteration usually requires repetitive procedures, and its success rate varies significantly between centers. We present a novel approach to recurrent fistulas. The innovation is in reaching the fistula through the virgin field via left-sided three-port thoracoscopy instead of classical right-sided thoracotomy. METHODS: This is a presentation of a new operative technique based on a retrospective case series of patients operated on at our department between 2016 and 2023. RESULTS: Eight patients after esophageal atresia repair (six with RTEF and two with post-fistula tracheal diverticula) were successfully treated with left-sided thoracoscopy. There were no conversions. One patient required rethoracoscopy for chylothorax. Another one, after RTEF closure, underwent multiple endoscopic obliterations of subsequent tracheal diverticulum. No other major complications nor re-recurrences were noted. CONCLUSIONS: Left-sided thoracoscopy in redo esophageal atresia has the advantage of a "virgin" operative field and grants feasible access to the RTEF or tracheal diverticulum. We believe that this approach is worth further exploration because it combines minimal invasiveness with high effectiveness without all the consequences of a thoracotomy.

15.
PLoS One ; 18(10): e0292485, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37796812

RESUMEN

Ureteral ectopia is rare and requires surgical treatment after a thorough diagnostic workup. Open surgical techniques for repositioning ectopic ureters have been known for many years and are well described in the literature. However, to the best of our knowledge, no laparoscopic method of correcting this pathology has been described, which, in our opinion, would benefit the animal in terms of the healing process and overall clinical outcomes. This study aimed to evaluate the possibility of laparoscopic treatment of ureteral ectopia, which causes urinary incontinence in dogs. All of the operated ten dogs presented in this study were client-owned females with symptoms of urinary incontinence due to a unilateral intramural ectopic ureter. A three-trocar laparoscopic technique was used to perform the ureteroneocystostomy of the ectopic ureter. In this article, clinicopathological data, imaging features, procedural findings, complications, and short- and long-term outcomes are presented. The procedure was feasible in all cases. No major postoperative complications were observed. Among the minor complications, slight hematuria was observed in three dogs, which resolved spontaneously. In the period of at least one year after surgery, no negative impact of the procedure was observed. Seven of the ten operated dogs regained urinary continence. The remaining three dogs required additional surgery (urethral bulking) because of a lack of improvement after adjuvant pharmacological treatment. Overall, good-to-excellent long-term outcomes can be achieved; however, dogs that remain incontinent after laparoscopic ureteroneocystostomy may require additional treatment.


Asunto(s)
Coristoma , Enfermedades de los Perros , Laparoscopía , Uréter , Enfermedades Ureterales , Obstrucción Ureteral , Incontinencia Urinaria , Perros , Femenino , Humanos , Animales , Uréter/cirugía , Uréter/patología , Proyectos Piloto , Enfermedades Ureterales/diagnóstico , Enfermedades Ureterales/cirugía , Enfermedades Ureterales/veterinaria , Enfermedades de los Perros/patología , Estudios Retrospectivos , Incontinencia Urinaria/etiología , Incontinencia Urinaria/cirugía , Incontinencia Urinaria/veterinaria , Laparoscopía/efectos adversos , Obstrucción Ureteral/complicaciones , Coristoma/patología
16.
Genes (Basel) ; 14(9)2023 09 20.
Artículo en Inglés | MEDLINE | ID: mdl-37761962

RESUMEN

Esophageal atresia (EA) is the most common malformation of the upper gastrointestinal tract. The estimated incidence of EA is 1 in 3500 births. EA is more frequently observed in boys and in twins. The exact cause of isolated EA remains unknown; a multifactorial etiology, including epigenetic gene expression modifications, is considered. The study included six pairs of twins (three pairs of monozygotic twins and three pairs of dizygotic twins) in which one child was born with EA as an isolated defect, while the other twin was healthy. DNA samples were obtained from the blood and esophageal tissue of the child with EA as well as from the blood of the healthy twin. The reduced representation bisulfite sequencing (RRBS) technique was employed for a whole-genome methylation analysis. The analyses focused on comparing the CpG island methylation profiles between patients with EA and their healthy siblings. Hypermethylation in the promoters of 219 genes and hypomethylation in the promoters of 78 genes were observed. A pathway enrichment analysis revealed the statistically significant differences in methylation profile of 10 hypermethylated genes in the Rho GTPase pathway, previously undescribed in the field of EA (ARHGAP36, ARHGAP4, ARHGAP6, ARHGEF6, ARHGEF9, FGD1, GDI1, MCF2, OCRL, and STARD8).


Asunto(s)
Atresia Esofágica , Masculino , Niño , Humanos , Atresia Esofágica/genética , Gemelos Monocigóticos/genética , Gemelos Dicigóticos , Islas de CpG/genética , Epigénesis Genética , Proteínas Proto-Oncogénicas , Factores de Intercambio de Guanina Nucleótido , Factores de Intercambio de Guanina Nucleótido Rho
17.
Children (Basel) ; 9(5)2022 Apr 23.
Artículo en Inglés | MEDLINE | ID: mdl-35626777

RESUMEN

Micturition is an involuntary process based on spinal arcs in infants and children until a defined age. The awareness and voluntary control of voiding depends on specific areas in the central nervous system, especially cortical regions. The cells and connections between these areas develop over time and regulate the voiding process. The ability to maintain continence and to adjust physiological needs to appropriate environmental conditions is considered to be acquired through systematic behavioral education, especially toilet training. The recommendations specify the age at which to start establishing the relevant habits. The purpose of these guidelines is to achieve proper micturition control development and to avoid functional lower urinary tract (LUT) disorders. We present a case of a patient who underwent complete urinary diversion in infancy and reconstruction of the urinary tract eleven years later. For eleven years, she had an empty bladder and no toilet training. After undiversion, she regained full continence in a short space of time. The presence of proper LUT function and a controlled micturition process raises the question of the standard toilet training recommendations' validity. The aim of our work focuses on the following question: Is toilet training the only way to achieve micturition skills and proper urinary tract function? The history of our patient and the literature reveal that voluntary micturition may develop without stimulating signals of filling from bladder receptors and independently of recommended behavioral education, so toilet training seems to not be necessary.

18.
Artículo en Inglés | MEDLINE | ID: mdl-35805703

RESUMEN

Aim: This study reports the reliability and validity of the Polish version of the Esophageal Atresia Quality of Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. Methods: A total of 50 families of children (23 aged 2 to 7, and 27 aged 8 to 17) with EA/TEF (esophageal atresia/tracheoesophageal fistula) participated in the study. The development and validation of the Polish version of the EA-QOL involved forward-backward translation of the survey items following the guidelines for cross-cultural translation, cognitive debriefing and evaluation of psychometric properties, including assessment of internal and retest reliability, linguistic validity, content validity, known-group validity and convergent validity. The medical records of patients and standardized questionnaires were used to obtain clinical data. The level of significance was p < 0.05. Results: The Polish versions of the EA-QOL questionnaires demonstrated strong linguistic and content validity, are slightly discriminative for esophageal and respiratory problems, but do not show convergent validity with the PedsQL 4.0 generic core scales. In terms of reliability, the internal consistency of the subscale and total scale of Polish versions as measured by Cronbach's alpha is good, and retest reliability is excellent. Conclusions: The Polish versions of the EA-QOL questionnaires meet most psychometric criteria that confirm the EA-QOL questionnaires' reliability and validity. This study enables application of these questionnaires in future research among children with EA in Poland and participation in international multicenter studies focusing on advancing knowledge of condition-specific QOL in this population. Future cross-cultural research using larger sample sizes is still needed to better address the relationship between condition-specific and generic QOL, as well as the discriminative ability of the EA-QOL questionnaires.


Asunto(s)
Atresia Esofágica , Calidad de Vida , Adolescente , Niño , Humanos , Polonia , Psicometría , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , Fístula Traqueoesofágica
19.
Int Urol Nephrol ; 54(10): 2503-2509, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35861906

RESUMEN

PURPOSE: This multi-institutional study aimed to assess the outcomes of laparoscopic ureterocalicostomy (LUC) and robot-assisted laparoscopic ureterocalicostomy (RALUC) and compare them with laparoscopic pyeloplasty (LP) and robot-assisted laparoscopic pyeloplasty (RALP) in children with pelvi-ureteric junction obstruction (PUJO). METHODS: The data of 130 patients (80 boys), with median age 7.6 years and median weight 33.8 kg, receiving minimally invasive treatment of PUJO over a 6-year period, were retrospectively analyzed. Patients were grouped according to the operative approach: G1 included 15 patients, receiving LUC (n = 9) and RALUC (n = 6), and G2 included 115 patients, receiving LP (n = 30) and RALP (n = 85). Patient characteristics and operative outcomes were compared in both groups. RESULTS: The median patient age and weight were significantly higher in G1 than in G2 [p = 0.001]. The median operative time was similar in both groups (157.6 vs 150.1 min) [p = 0.66] whereas the median anastomotic time was shorter in G1 than in G2 (59.5 vs 83.1 min) [p = 0.03]. The surgical success rate was similar in both groups (100% vs 97.4%) [p = 0.33]. Post-operative complications rate was higher in G1 than in G2 (20% vs 6.1%) but all G1 complications were Clavien 2 and did not require re-intervention. CONCLUSION: LUC/RALUC can be considered safe and effective alternative approaches to LP/RALP for PUJO repair and reported excellent outcomes as primary and salvage procedures. Robot-assisted technique was the preferred option to treat most patients with recurrent PUJO in both groups.


Asunto(s)
Laparoscopía , Procedimientos Quirúrgicos Robotizados , Robótica , Obstrucción Ureteral , Niño , Humanos , Hidronefrosis/congénito , Pelvis Renal/cirugía , Laparoscopía/efectos adversos , Laparoscopía/métodos , Masculino , Riñón Displástico Multiquístico , Estudios Retrospectivos , Procedimientos Quirúrgicos Robotizados/efectos adversos , Procedimientos Quirúrgicos Robotizados/métodos , Resultado del Tratamiento , Obstrucción Ureteral/cirugía , Procedimientos Quirúrgicos Urológicos/métodos
20.
Sci Rep ; 12(1): 3420, 2022 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-35233044

RESUMEN

Esophageal elongation is one of the methods of long gap esophageal atresia treatment. The aim of the study was to determine the best type of traction suture for esophageal lengthening on an animal model. White Pekin Duck's esophagi were used as a model (fresh-frozen and thawed). The esophagus was cut in half, then both ends were sutured together and extended on a tensiometer. Tested sutures involved simple suture, suture aided by a single or double clip, and suture aided by pledget (10 samples each). Constant and 2 methods of intermittent traction were also compared. The histological study showed similarities between duck's and newborn's esophagus. The highest maximal force was achieved with pledget suture (F = 8.59 N ± 1.45 N), then with double clip (F = 5.74 N ± 1.29 N) and the lowest with single suture (F = 3.80 N ± 0.54 N) (p < 0.001). Pledget suture also allowed for the greatest elongation (p < 0.01). Intermittent traction results in better elongation at the same breaking strength as constant traction (p < 0.05) if traction is maintained during breaks. Reinforced sutures (pledget or double clip) should be taken into consideration in internal traction. When performing traction sutures, it is worth step by step carefully tightening the sliding knot in short periods before its final binding.


Asunto(s)
Técnicas de Sutura , Tracción , Anastomosis Quirúrgica/métodos , Animales , Fenómenos Biomecánicos , Modelos Animales de Enfermedad , Esófago/cirugía , Suturas , Resistencia a la Tracción , Tracción/métodos
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