This study reports the feasibility, validity and reliability of the Turkish versions of the Esophageal-Atresia-Quality-of-Life (EA-QOL) questionnaires, which were originally developed in Sweden and ...Germany.
After translation from Swedish to Turkish and cognitive debriefings, 51 families of children aged 2 to 7 years (parent-report, 17-items) and 54 families of children 8 to 17 years (child and parent-report, 24-items) responded to the EA-QOL questionnaires and a validated generic HRQOL-instrument (PedsQL4.0). Medical records of patients and questionnaires were used to obtain clinical data. The Turkish version of the EA-QOL questionnaires were evaluated for feasibility (<5% missing item responses), reliability (internal consistency/retest reliability for 3 weeks) and validity (known-groups/concurrent/convergent). Level of significance was p<0.05.
Feasibility of the Turkish version of the EA-QOL questionnaires was good. Internal consistency of all scales was satisfactory, as were the level of agreements of EA-QOL scores between the field- and retest study. Known-group and concurrent validity were achieved since the EA-QOL questionnaires showed that esophageal symptoms and feeding difficulties were negatively associated with EA-QOL total scores in both age specific versions (child-and parent report), and respiratory symptoms in the version for EA children 2 to 7 years (parent-report). A higher number of respiratory symptoms decreased the EA-QOL total scores in both age groups (parent-report, p<0.05). Correlations between the EA-QOL total scores and PedsQL-4.0 total scores supported convergent validity.
The Turkish version of the EA-QOL questionnaires are feasible, valid and reliable to assess condition-specific HRQOL in EA children.
Abstract
Aim
The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the survival rate and risk factors of mortality in infants with esophageal atresia (EA).
Methods
The ...data of 389 cases registered by 23 centers between 2014 and 2018 were evaluated for demographic features, associated anomalies, surgical treatment and mortality-related outcome. Each parameter was evaluated for cases with mortality (CM) and compared with the results of survivors. Also, prognostic factors were investigated in CM.
Results
The overall survival rate was 81.09% and the mortality was 19.02% (n = 74). The male/female ratio was 1:1 in CM and 1.2:1 in survivors. The mean weight and height measurements in CM were lower than in survivors (1956 ± 658 g- 43.2 ± 5.7 cm vs 2534 ± 609 g-46.4 ± 4.3 cm) (P < 0.05). The incidence of associated anomalies was 90.6% in CM and significantly higher than survivors (59.6%) (P < 0.05). Neurological, cardiovascular, vertebral, and extremity anomalies were significantly higher in CM (P < 0.001). The most common type of anomaly was EA with distal fistula 89.2% in CM and 74.6% in survivors. The rate of primary anastomosis was also significantly lower in CM (67.6% vs 84.3%, P < 0.05). The gap length did not show statistical difference between CM and survivors (P > 0.05). Tube feeding was more common in CM and the incidence of full oral feedings was significantly higher in survivors (P < 0.01). According to the Spitz classification, the survival rate was 87.1% in Class I, 55.3% in Class II, and 16.7% in Class III. The most common causes of mortality were associated with cardiovascular disease, pneumonia, and sepsis.
Conclusion
The majority of national TEAR data comes from tertiary healthcare centers in Turkey. Therefore, the slightly increased mortality rates could be related to the inclusion of more complex and complicated cases. Birth weight–height, associated anomalies, and Spitz classification are significant risk factors of mortality in cases with EA.
Experience in the use of endoscopic retrograde cholangiopancreatography (ERCP) for the investigation and treatment of pancreaticobiliary disorders is relatively limited in children. This report ...reviews the experience in a single institution with pediatric ERCPs and documents the indications, success rate, diagnostic and therapeutic yields, complications, and the impact on patient management.
The data of all consecutive patients aged < or = 18 years who underwent ERCP procedures between the years 1997 and 2007 were retrospectively identified through a computer database search. The database prospectively recorded the indications, findings, therapies, and complications.
During the study period, 32 ERCP procedures were performed in 28 children with a median age of 13 (range 8 - 18) years. ERCPs were performed for biliary pathology in 21 (75 %) and for pancreatic pathology in 7 (25 %) patients. The most common biliary indications were suspected choledocholithiasis and postoperative bile leaks. Hydatid disease was the most common diagnosis that yielded bile leaks. The pancreatic indications were recurrent pancreatitis and traumatic pancreatic duct disruption. Cannulation of the desired duct was successful in all procedures. An endoscopic sphincterotomy, stone/sludge removal or a stent placement was performed in 20 (63 %) procedures. According to the long-term follow-up, avoidance from any further surgical interventions was achieved in 11 (65 %) children, in whom ERCP was undertaken as a therapeutic intervention. The complication rate was 6 % with the development of mild self-resolving pancreatitis in one patient and stent occlusion in another.
ERCP in the pediatric population has a high success rate, both as a diagnostic tool and for therapeutic interventions, provided it is performed by experienced endoscopists. The delicate delineation of the anatomy by ERCP and its therapeutic potential make it absolutely superior to other less invasive tools such as magnetic resonance cholangiopancreatography.
Endothelins ( ETs ) are potent vasoconstrictors derived from vascular endothelium. They have primary roles in many pathophysiologic states including ischemia/reperfusion (I/R) injury. The ...relationships between nitric oxide (NO) and ETs are still under investigation. In this study on rats we want to focus on the interaction of NO and ET especially in I/R injury. For this purpose ET-1 and PD-156252, a nonselective ET receptor blocker, were given in a mesenteric I/R model and reactive oxygen species were detected directly using chemiluminescence of the ileal tissue. ET administrations to sham and I/R groups caused significant increases in NO concentrations whereas, in terms of peroxynitrite, which is a highly reactive group of free radicals, its increasing effects were seen only in I/R groups. This suggests that in I/R where superoxide levels increase together with NO, the conversion to peroxynitrite is likely and this effect is augmented with ET administration. On the other hand PD administration decreases superoxide and thereby peroxynitrite levels and this study shows that the effect of PD-156252 is established through this mode of action. These data suggest therapeutic approaches that may be beneficial in the treatment of I/R injury.
Abstract
Aim
The aim of this study is to evaluate the results of esophageal balloon dilatation (BD) for strictures after esophageal atresia (EA) surgery.
Methods
Flexible endoscopic BD was done under ...fluoroscopic and manometric control. The balloon placed in the stricture was inflated by contrast. The target pressure and the diameter were decided in compliance with the manufacturer's directions as well as fluoroscopic guidance. The balloon was kept inflated for 3 minutes. Prospectively collected data over 10 years were retrospectively evaluated. Cure was defined as no need for dilatation during the last 12 months.
Results
A total of 79 patients with variable diagnoses underwent 481 BD. Forty (51%) had strictures which developed after EA surgery. They underwent 175 (36%) BD (P < 0.05). There were 21 males and 19 females. The atresia was distal fistula type in 31 (77.5%) patients and isolated EA in nine (22.5%). The BD was done for primary esophago-esophageal anastomosis site in 37 patients. The remaining three patients with long gap EA had undergone previous replacement surgery and the BD was done for the proximal esophago-colonic anastomosis. The median age at the time of the first BD was 14 months with 17 (43%) patients below the age of 1 year. The maximal inflation diameter varied between 5–20 mm. Esophageal BD catheters were used in all except two occasions where 5 mm ureteral balloons were used. The median number of BD was four (n = 1–15). Thirty-four (85%) patients underwent more than one BD. A transmural perforation was encountered in one occasion (0.6%) and the patient eventually underwent esophageal replacement surgery with an uneventful outcome. There was no mortality. Fundoplication was done in nine patients. Thirty-three patients (82.5%) were regarded as ‘cure’.
Conclusion
Esophageal BD is employed for strictures caused by a variety of reasons. Patients with EA comprise a substantial number of cases needing BD and have strictures less resistant to dilatation. The technique employed in this study is advantageous because it enables direct endoscopic visualization of the stricture and both gradual and controlled increase of the dilatation pressure. BD is safe and efficient yet there is a risk of esophageal perforation as in the other dilatation techniques.
Abstract
Aim
This study presents a single institutional experience with H-type tracheoesophageal fistula (H-TEF).
Methods
Esophageal atresia (EA) patients who underwent primary surgery in a single ...center between 2006 and 2018 were retrospectively evaluated.
Results
Among 110 EA patients, 7 (6.4%) had H-TEF. There were 5 males and 2 females. The primary complaints were choking, cyanosis, and/or apnea with feeding, swallowing difficulty, recurrent bronchopneumonia, or bile in endotracheal tube. The relevant symptoms began during neonatal period with varying degrees of severity in all. The initial diagnosis was proximal esophageal web as demonstrated by contrast swallow and endoscopy in one patient; H-TEF was diagnosed during the course of treatment for the web. Hydrocephalus with severe neurological sequela unrelated to H-TEF and cardiac malformation were present in one. Contrast esophagography was employed in three patients for diagnosis two of which were diagnostic. All underwent diagnostic bronchoscopy. Delayed diagnosis (after 30 days of life) occurred in five (71%). The median age at operation was 3.8 months (11 days–33 months). The level of the fistulas was between C5 and T3. Preoperative bronchoscopic cannulation was successful in all but one. The operation was done via a right cervical approach in six patients and thoracotomy in one. All patients were followed up for at least one year and there was one recurrence detected five months after the first operation. She underwent a secondary surgery with a successful outcome. Right vocal cord paralysis was detected postoperatively in the patient with hydrocephalus.
Conclusions
H-TEF is a rare subtype of EA malformations with a delay in diagnosis in most cases. Suggestive clinical findings, especially neonatal respiratory symptoms should always be regarded as an indication for further investigations. Although contrast esophagograms may reveal the diagnosis, bronchoscopy is the gold standard diagnostic test in our practice. It should be done preoperatively in every patient not only for diagnostic confirmation but also for cannulation of the fistula to ease the operation. H-TEF might be considered a relatively innocent EA variant but its surgery is not devoid of complications. The patients should be followed up for recurrence and recurrent laryngeal nerve injury on the long term.
The EA-QOL questionnaire measures quality-of-life specifically for children born with esophageal atresia (EA) aged 8-18 and was completed in Sweden and Germany. This study aimed to describe an ...international collaborative initiative to establish a semantically equivalent linguistic version of the EA-QOL questionnaires in 12 new countries. The 24-item EA-QOL questionnaire was translated into the target languages and the translated questionnaire was evaluated through cognitive debriefing interviews with children with EA aged 8-18 and their parents in each new country. Participants rated an item as to whether an item was easy to understand and sensitive/uncomfortable to answer. They could choose not to reply to a non-applicable/problematic item and provide open comments. Data were analyzed using predefined psychometric criteria; item clarity ≥80%, item sensitive/uncomfortable to answer ≤20%, item feasibility(missing item responses ≤5%). Decision to improve any translation was made by native experts-patient stakeholders and the instrument developer. Like in Sweden and Germany, all items in the cross-cultural analysis of child self-report (
= 82, 4-10 children/country) met the criteria for item clarity in all 12 new countries, and in parent-report (
= 86, 5-10 parents/country) in 8/12 countries. All items fulfilled the criteria for sensitive/uncomfortable to answer (child-report 1.2-9.9%; parent-report 0-11.6%) and item feasibility. Poor translations were resolved. Hence, this study has established semantically equivalent linguistic versions of the EA-QOL questionnaire for use in children aged 8-18 with repair of EA in and across 14 countries.
In total, 74 pediatric oncology patients with hematologic malignancies (n=56) or solid tumors (n=18) and a median age of 78.5 months were included in this prospective study. The aims were to assess ...malnutrition risks and nutritional status over a 6-month treatment period measured at regular intervals. The rate of patients with high risk for malnutrition at diagnosis was 28.4% by Screening Tool for Risk of Impaired Nutritional Status and Growth tool and 36.5% by Pediatric Yorkhill Malnutrition Score. Body mass index (BMI) z-scores at diagnosis showed 12.3% undernutrition (<-2 SD) and 6.8% overnutrition (>2 SD), which changed to 6.7% and 11.1% at the sixth month, respectively. Malnutrition (BMI<5th age percentile) was detected in 13.7% at diagnosis. Despite an initial deterioration noted in BMI, BMI for age percentile, and z-scores at month 1 in all malignancy subgroups (at month 3 for acute lymphoblastic leukemia), the scores improved later on. There was an increase in weight from baseline in 88.2% of patients over 6 months. This study revealed a decrease in the prevalence of undernutrition and malnutrition over a 6-month treatment period with improved anthropometrics despite an initial deterioration in all malignancy subgroups and even in patients with high risk for malnutrition at baseline screening. Solid tumors and acute lymphoblastic leukemia seem to be associated with higher likelihood of undernutrition and overnutrition, respectively, during treatment.
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