Androgen Receptor-Positive Penile Acrochordon: A Case Report.

Acrochordons are polypoid, skin-colored lesions usually present at the site of skin folds. They are extremely rare in the preputial area of the penis and do not tend to grow. To the best of our knowledge, in English literature, this report presents the first case of an androgen receptor-positive penile acrochordon, which is located on the penis and showed rapid growth along with body development during puberty with no underlying causes such as acromegaly, diabetes, obesity, and trauma.

Management of Retinitis Pigmentosa Via Wharton's Jelly-Derived Mesenchymal Stem Cells or Combination With Magnovision: 3-Year Prospective Results.

To investigate whether the natural progression rate of retinitis pigmentosa (RP) can be decreased with subtenon Wharton's jelly-derived mesenchymal stem cell (WJ-MSC) application alone or combination with Magnovision. The study included prospective analysis of 130 eyes of 80 retinitis pigmentosa patients with a 36-month follow-up duration. Patients constitute 4 groups with similar demographic characteristics. The subtenon WJ-MSC-only group consisted of 34 eyes of 32 RP patients as Group 1; the Magnovision-only group consisted of 32 eyes of 16 RP patients as Group 2; the combined management group consisted of 32 eyes of 16 RP patients who received combined WJ-MSC and Magnovision as Group 3; the natural course (control) group consisted of 32 eyes of 16 RP patients who did not receive any treatment were classified as Group 4. Fundus autofluorescence surface area (FAF-field), horizontal and vertical ellipsoid zone width (EZW), fundus perimetry deviation index (FPDI), full-field electroretinography magnitude (ERG-m), and best corrected visual acuity (BCVA) changes were compared within and between groups after 36 months follow up period. FAF-field delta changes were detected 0.39 mm2 in Group 1, 1.50 mm2 in Group 2, 0.07 mm2 in Group 3 and 12.04 mm2 in Group 4 (Δp 4 > 2 > 1 > 3). Horizontal EZW, Vertical EZW, BCVA, and FPDI delta changes were detected Δp 4 > 1,2 > 3. ERG-m delta changes were detected Δp 3 > 1,2,4. Retinitis pigmentosa characterized by progressive loss of photoreceptors eventually leading to total blindness. The combination of WJ-MSC and Magnovision can significantly slow the progression of the disease in comparison to natural progression rate for 3 years in appropriate cases. Trial Registration: ClinicalTrials.gov, NCT05800301.

Evaluating patient outcomes in postoperative pain management according to the revised American Pain Society Patient Outcome Questionnaire (APS-POQ-R).

BACKGROUND: In the first 24 h after surgery, it is necessary to evaluate the patient responses to pain, analgesia and patient satisfaction to prevent complications related to the pain management process. AIM: To evaluate patients' outcomes (pain qualities, side effects of the pain management, pain treatment satisfaction, non-pharmacological pain treatment methods, predictors of pain management satisfaction and percentage of pain relief) according to the Revised American Pain Society Patient Outcome Questionnaire (APS-POQ-R) in the first 24 h. DESIGN: Cross-sectional study. METHODS: The study sample was comprised of 700 patients, who were surgically treated at the surgical clinics of a university hospital and completed the first postoperative 24 h. The data was collected through the "Patient Information Form" and the "Turkish version of the revised American Pain Society Patient Outcome Questionnaire (APS-POQ-R)". RESULTS: The medians of the lowest and the worst postoperative pain severity level were 3.0 and 7.0, respectively. Patients experienced severe pain in 60 % of the first postoperative 24 h and reported that 70 % of their pain eventually decreased. A positive and significant correlation was found between pain interference, pain-affected mood/emotions, the severity of pain-related side effects, the least and worst pain severity levels and severe pain, and the percentage of time experienced with severe pain. CONCLUSIONS: Most of the patients experienced severe pain, which restricted their daily life activities and led to negative emotions. Acute postoperative pain may negatively affect patient outcomes and delay postoperative recovery during the early period. Therefore, pain should be managed in the early period to prevent physical and psychological side effects.

Evaluation of daily and social participation of children with Cerebral Palsy across different age groups with a focus on the 'F'-words: Function, family, fitness, fun, friends and future.

BACKGROUND: The impact of disability differs across cultures. This study aimed to determine the predictors of participation in children with cerebral palsy (CP) in Turkey, based on the six F-words. METHODS: Cross-sectional study exploring participation profiles of 450 children with CP, aged between 2 and 18 years. Pediatric Evaluation of Disability Inventory (PEDI) evaluated functional skills, and Assessment of Life Habits (LIFE-H) version 3.0 assessed daily and social participation. Hierarchical linear regression models were done to determine the predictors of participation in daily activities (PDA) and social roles (PSR) in three age groups (2-4, 5-13 and 14-18 years) based on the 6 F-words (mobility of PEDI for 'fitness'; four classification systems and self-care of PEDI for 'functioning'; social functions of PEDI for 'friends'; demographic information by parents for 'family'; the recreation of LIFE-H for 'fun'; and different stages of development for 'future'). RESULTS: The most important predictors for total PDA by age group were: self-care (p = 0.012) of PEDI in 2-4 y; self-care (p = 0.001) and mobility (p = 0.005) of PEDI in 5-13 y; GMFCS (p = 0.006) and mobility (p = 0.002) of PEDI in 14-18 y. Significant predictors for PSR differed by age group: self-care (p = 0.001) of PEDI in 2-4 y; self-care (p = 0.023) and mobility (p = 0.006) of PEDI in 5-13 y; and GMFCS (p = 0.004) and MACS (p = 0.003) in 14-18 y. CONCLUSIONS: Six F-words of function and fitness focussed on self-care in younger children with an increasing emphasis on mobility and ability levels according to age. Therefore, rehabilitation for different aspects of the functional levels is needed to improve participation in life across the six F-words framework; plus take into consideration context, age-differences, family's expectations, life requirements, environmental needs, and cultural differences.

Body image dissatisfaction among school children in Turkey and its potential effect on body esteem.

BACKGROUND: This study aimed to validate the Turkish version of Collins` Body Figure Perceptions and Preferences (BFPP) scale. The second aim of this study was to investigate the relationship between body image dissatisfaction (BID) and body esteem (BE), and between body mass index (BMI) and BID, among Turkish children. METHODS: A descriptive cross-sectional study was conducted among 2066 4th grade children (mean age was 10.06 ± 0.37 years) in Ankara, Turkey. The Feel-Ideal Difference (FID) index from Collins` BFPP was used to assess the degree of BID. FID ranges from -6 to +6, with scores below or above 0 indicating BID. Collins` BFPP`s test-retest reliability was evaluated in a subset of 641 children. The Turkish version of the BE Scale for Adolescents and Adults was used to evaluate the children` BE. RESULTS: More than half of the children were dissatisfied with their own body images (57.8% of girls vs. 42.2% of boys, p < .05). The lowest BE score in both genders was among adolescents who desired to be thinner (p < .01). The criterion-related validity of Collins` BFPP, in relation to BMI and weight, was at an acceptable level in girls (BMI rho= 0.69, weight rho= 0.66) and boys (BMI rho= 0.58, weight rho= 0.57), and was statistically significant in all cases (p < .01). The test-retest reliability coefficients of Collins` BFPP were found to be moderately high for both girls (rho=0.72) and boys (rho=0.70). CONCLUSIONS: Collins` BFPP scale is a reliable and valid tool for Turkish children aged 9-11 years. This study demonstrates that more Turkish girls than boys were dissatisfied with their bodies. Children who were affected by overweight/obesity and underweight had a higher BID than those with a normal weight. It is important to evaluate adolescents` BE and BID in addition to their anthropometric measurements during their regular clinical follow-up.

The effects of fatigue, gross motor function, and gender on participation in life situations of school-aged children with cerebral palsy: A parental perspective.

OBJECTIVE: To investigate the effects of fatigue, gross motor function, and gender on participation in life situations of school-aged children with cerebral palsy (CP) from a parental perspective. METHODS: The study included 209 children with CP aged between 5 and 13 years (mean age, 8.06 ± 2.41 years; girls, 45.5%) and their parents. Fatigue, gross motor function, and participation status were evaluated with the Pediatric Quality of Life (PedsQL), Gross Motor Function Classification System (GMFCS), and the Assessment of Life Habits (Life-H) questionnaire, respectively. The effects of fatigue, gross motor function, and gender on participation were investigated with linear regression analysis. RESULTS: According to parental reports, 79.9% of the children had fatigue. Children in all GMFCS levels experienced fatigue. Fatigue and GMFCS levels were dependent variables, and therefore only simple linear regression analyses were performed. Fatigue explained 38-43% of the variances in daily activities, social roles, and total Life-H scores, while gross motor function explained 48-65% of the variances in scores (p < 0.001). Gender had no effect on participation scores (p > 0.05). CONCLUSION: More than two thirds of the school-aged children with CP had fatigue. Fatigue and poor gross motor function had a negative effect on participation in daily activities and social roles.

Evaluation of the predictability of clinical and radiological findings in the diagnosis of malrotation.

BACKGROUND: To evaluate the predictability of clinical and radiological findings in the diagnosis of malrotation. METHODS: Between 2010 and 2020, children with presumptive diagnosis of malrotation were included. The demographic features, clinical and radiological findings, operative findings and outcome were recorded. The upper gastrointestinal series (UGIS) were evaluated by two radiologists. All parameters were correlated with surgical findings to evaluate the predictability. RESULTS: Seventy patients were included. The presenting symptom was bilious vomiting in 29 cases (41.4%), and atypical symptoms (non-bilious vomiting, food refusal, etc.) in 40 cases (57%). One of the cases (1.6%) was asymptomatic and diagnosed incidentally during UGIS. 52 cases had abdominal X-ray and 14 (26.9%) of them were normal. Doppler ultrasonography (US) (n=20) revealed evidence of malrotation in 13 cases (65%). The location of duodenojejunal junction (DJJ) in UGIS was compatible with malrotation in 33 cases. 48 (61%) cases underwent surgical exploration; 35 cases had malrotation and seven cases had midgut volvulus. Median followup time was one year (0.5-7 years). Volvulus has recurred in one case and another case operated for volvulus died because of short bowel syndrome. The statistical analysis for predictability revealed that bilious vomiting (sensitivity: 57.1%, specificity: 82.1%), Doppler US (sensitivity: 92.3%, specificity: 75%) and right-sided DJJ in UGIS (sensitivity: 96.8%, specificity: 75%) have highest predictability. CONCLUSIONS: The bilious vomiting, Doppler US findings and right-sided DJJ have the highest predictability to confirm the diagnosis. However, presenting with atypical symptoms and having atypical or normal findings in UGIS do not rule out malrotation.

The effect of male and female factor infertility on women's anxiety, depression, self-esteem, quality of life and sexual function parameters: a prospective, cross-sectional study from Turkey.

OBJECTIVES: Despite the development of diagnosis and treatment methods, the psychological effects of infertility on women were not adequately addressed. This study investigated the effect of male and female factor infertility on women's anxiety, depression, self-esteem, quality of life, and sexual function parameters. METHODS: In this prospective, cross-sectional study, 480 women [n = 234, with male factor infertility (MFI) (MFI group) and n = 246, with female factor infertility (FFI) (FFI group)], who could not conceive despite unprotected intercourse for 1 year, and 242 fertile healthy women (control group) who had children within the last 1 year were included. Beck Anxiety Inventory (BAI), Beck Depression Inventory-II (BDI-II), Rosenberg Self Esteem Scale (RSES), Fertility Quality of Life (FertiQol) and Female Sexual Function Index (FSFI) questionnaires were used for patients' evaluation. RESULTS: RSES, BAI and BDI-II scores were significantly higher and FSFI score was significantly lower in the FFI group compared to other groups. RSES, BAI and BDI-II scores were also significantly higher and FSFI score was significantly lower in the MFI group compared to the control group. FertiQol total score was significantly lower in the FFI group compared to the MFI group. RSES score was positively correlated with BDI-II and BAI scores; however, it was negatively correlated with FertiQol and FSFI scores. CONCLUSIONS: The negative psychological effects of infertility are ignored by many centres, especially in the treatment process of infertility. Regardless of the infertility factor (male or female), we believe that psychological support should be given to all women to improve their life quality.

The cause of dysphagia in patients with esophageal atresia: a systematic review and meta-analysis.

PURPOSE: Dysphagia is the most common symptom in patients with esophageal atresia (EA) of all ages. There is no study addressing the direct relation between dysphagia and surgical results. Therefore, a systematic review was performed to define the relationship between surgical complications and dysphagia in patients with EA. METHODS: The systematic review was drafted under PRISMA guidelines. Systematic literature search was performed for the period 2000 (Jan) to 2019 (Dec)-20 years-in the databases: MEDLINE, EMBASE, and PubMed. Statistical analysis was performed using Comprehensive Meta-Analysis Version 3.0 software. RESULTS: Among 767 articles, 486 abstracts were screened for the inclusion criteria. The full-texts of 64 articles were assessed for eligibility. The sub-group analysis could be performed in 4 articles for anastomotic strictures. Heterogeneity was calculated by I2 statistic as 18,487 and pooled odds ratio was measured under the fixed effect model (Q = 3.68; P = 0.298, I2 = 18,487). There was no significant relationship with an odds ratio of 1.37 between anastomotic stricture (AS) and dysphagia (95% CI 0.631-2.973, p = 0.426). There was no publication bias for the data (Begg's test, p = 0.496; Egger's tests, p = 0.335). CONCLUSION: This meta-analysis did not reveal a significant relationship between AS and dysphagia in children with EA. Since many other factors contribute to dysphagia, comprehensive variable information such as detailed standardized registry systems for rare diseases for pooling analysis is needed regarding other potential factors including surgical complications.

Environmental exposure in the etiology of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis.

PURPOSE: To investigate the occupational and environmental factors in the etiology of infantile hypertrophic pyloric stenosis (IHPS). METHODS: Protocol was drafted according to the PRISMA guidelines and registered on PROSPERO (CRD42020152460). A search for a combination of terms related to IHPS, fetus and neonates, and environmental exposure was performed for studies published between 2000 and 2020 in the EMBASE, Pubmed, and MEDLINE databases. RESULTS: Overall, 2203 abstracts were identified and 829 were screened. The full text of the selected articles (N = 98) was assessed for eligibility. Fifteen studies were included in quantitative synthesis. IHPS risk was significantly lower in black and Hispanic mothers than in white mothers [OR 0.47 (95% CI 0.44-0.51, p < 0.001), OR 0.85 (95% CI 0.77-0.94, p = 0.002), respectively]. Lower maternal education level and maternal smoking were risk factor for IHPS. We further observed a non-significant association between maternal folic acid usage and IHPS risk. Data were insufficient to evaluate occupational exposure. CONCLUSION: This review provides an understanding of the role of environmental exposures in IHPS etiology. Lower maternal educational level, maternal smoking, and white ethnicity are associated with a significantly increased risk of IHPS, while folic acid use seems non-significantly associated with IHPS risk. LEVEL OF EVIDENCE: III.

Effects of Transcutaneous Electrical Nerve Stimulation on Mastectomy Pain, Patient Satisfaction, and Patient Outcomes.

PURPOSE: Mastectomy is one of the most painful surgical procedures. Postoperative pain guidelines recommend transcutaneous electrical nerve stimulation (TENS) as a reliable non-pharmacological analgesic method. The aim of this study was to investigate the effects of TENS on postoperative pain and outcomes in patients undergoing modified radical mastectomy (MRM). DESIGN: A single-center, single-blind, prospective, randomized-controlled study. METHODS: This single-center, single-blind, randomized-controlled study included a total of 80 patients who underwent MRM at general surgery clinic of a tertiary center were included. The pain management of the patient outcomes were evaluated using the Turkish Revised American Pain Society Patient Outcome Questionnaire (APS-POQ-R-TR). FINDINGS: The pain levels of the intervention group were lower than the control group. There were significant improvements in the patient outcomes such as mobilization, position, sleep, anxiety, and fear in the intervention group. CONCLUSIONS: Our study results suggest that TENS reduces MRM pain. Thus, TENS can be recommended as a useful analgesic method in MRM.

Structural equation model of physical activity in Turkish schoolchildren: an application of the integrated behavioural model.

OBJECTIVES: Childhood obesity is increasingly prevalent in the developing world including Turkey. This study examined constructs of the integrated behavioural model associated with physical activity in a sample of schoolchildren in Ankara, Turkey using structural equation modelling. DESIGN: Cross-sectional survey by probability sampling. SETTING: Fifteen schools of different socioeconomic strata in Ankara, Turkey with grade 4 students. PARTICIPANTS: 2066 (969 girls and 1097 boys) grade 4 schoolchildren and their parents selected using a probability-based sampling frame. PRIMARY OUTCOME MEASURES: Three primary outcomes were used: moderate-to-vigorous physical activity, team sport participation, sedentary behaviour. RESULTS: Data were collected from 2066 fourth-grade children from schools of three socioeconomic strata. Missing data were imputed using multiple imputation. To examine the integrated behavioural model, a structural equation model containing latent constructs for physical activity outcome expectancies, self-efficacy, home environment and social norms were fitted with the three outcomes above. Adequate model fit was achieved in the structural equation model (χ2=1821.97, df=872, p<0.001, Comparative Fit Index=0.91, Tucker Lewis Index=0.91, root mean square error of approximation=0.02, standardised root mean square residual=0.04). All scale items were significantly associated with their respective latent constructs (all p<0.001). Several significant pathways between latent constructs and outcomes of interest were observed (p<0.05). Self-efficacy was positively associated with moderate-to-vigorous physical activity (p<0.001) and team sport participation (p<0.001) and negatively associated with sedentary behaviour (p<0.001). Negative outcome expectancies were negatively associated with moderate-to-vigorous physical activity (p<0.01) and sedentary behaviour (p<0.01) while positive outcome expectancies were positively associated with team sport behaviour (p<0.001) and negatively associated with sedentary behaviour (p<0.05). Home support was positively associated with moderate-to-vigorous physical activity (p<0.01) and team sport participation (p<0.05). Finally, physical activity social norms were negatively associated with sedentary behaviour only (p<0.05). CONCLUSIONS: This study supported the extension to Turkish children of the integrated behavioural model in relation to physical activity behaviours. Results illustrate multiple targets for interventions to increase physical activity.

Management of toxic optic neuropathy via a combination of Wharton's jelly-derived mesenchymal stem cells with electromagnetic stimulation.

PURPOSE: To investigate the effect of the combination of Wharton's jelly derived mesenchymal stem cells (WJ-MSC) and high frequency repetitive electromagnetic stimulation (rEMS) in the therapy of toxic optic neuropathies with severe symptoms after the available current therapy modalities which were unsucessful. MATERIAL AND METHODS: This prospective, open-label clinical phase-3 study was conducted at Ankara University Faculty of Medicine, Department of Ophthalmology between April 2019 and April 2021. Thirty-six eyes of 18 patients with toxic optic neuropathy (TON) were included in the study. Within 1-3 months after the emergency interventions, patients with various degrees of sequela visual disturbances were studied in this clinical trial. The cases were divided into three groups according to similar demographic characteristics. Group 1: Consists of 12 eyes of 12 patients treated with the WJ-MSC and rEMS combination in one eye. Group 2: Consists of 12 eyes of 12 patients treated with only rEMS in one eye. Group 3: Consists of 12 eyes of six patients treated with only WJ-MSC in both eyes. The course was evaluated by comparing the quantitive functional and structural assessment parameters measured before and at the fourth month of applications in each group. RESULTS: The mean best corrected visual acuity (BCVA) delta change percentages of the groups can be ranked as: Group 1 (47%) > Group 3 (32%) > Group 2 (21%). The mean fundus perimetry deviation index (FPDI) delta change percentages of the groups can be ranked as: Group 1 (95%) > Group 2 (33%) > Group 3 (27%). The mean ganglion cell complex (GCC) thickness delta change (decrease in thickness) percentages can be ranked as: Group 1 (- 21%) > Group 3 (- 15%) > Group 2 (- 13%). The visual evoked potential (VEP) P100 latency delta change percentages of the groups can be ranked as: Group 1 (- 18%) > Group 3 (- 10%) > Group 2 (- 8%). The P100 amplitude delta change percentages of the groups can be ranked as: Group 1 (105%) > Group 3 (83%) > Group 2 (24%). CONCLUSION: Toxic optic neuropathies are emergent pathologies that can result in acute and permanent blindness. After poisoning with toxic substances, progressive apoptosis continues in optic nerve axons and ganglion cells. After the proper first systemic intervention in intensive care clinic, the WJ-MSC and rEMS combination seems very effective in the short-term period in cases with TON. To prevent permanent blindness, a combination of WJ-MSC and rEMS application as soon as possible may increase the chance of success in currently untreatable cases. Trial Registration ClinicalTrials.gov ID: NCT04877067.

Feasibility, Reliability, and Validity of the Turkish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: The Turkish version of the EA-QOL questionnaires are feasible, valid and reliable to assess condition-specific HRQOL in EA children.

Investigation of genotype-phenotype relationship in Turkish patients with inherited retinal disease by next generation sequencing.

BACKGROUND: Inherited retinal dystrophies (IRDs) are a group of retinal diseases genetically and clinically highly heterogeneous and associated with more than 300 genes. This study aims to investigate the genetic basis of Turkish patients with IRDs. MATERIALS AND METHODS: In the study, genes related to retinal diseases in 86 IRDs patients were analyzed using the Next Generations Sequencing method (NGS). RESULTS: The mean age of 86 patients was 35 and the mean age at diagnosis was 18. There was consanguinity between the parents of 62% of these patients. Fifty-six retinal disease-associated genes of 46 patients and 230 retinal disease-associated genes of 40 patients were examined. Genetic analysis provides a molecular diagnosis in a total of 53 (61.6%) patients. The genes responsible for the IRDs phenotype were frequently identified as ABCA4 (25%), EYS (11%), and RDH12 (9%). There was no significant difference between those with and without a molecular diagnosis in terms of demographic characteristics and family history. CONCLUSIONS: Determination of genetic cause by NGS method in IRDs subgroups that are difficult to define by ophthalmic examination ensures that patients receive accurate diagnosis, treatment and counseling. This study contributed to the understanding of the genotype-phenotype relationship of Turkish patients with IRDs.

Evaluation of the effect of subtenon autologous platelet-rich plasma injections on visual functions in patients with retinitis pigmentosa.

Aim: The photoreceptors in retinitis pigmentosa (RP) remain in dormant status for a while with a decrease in the growth factors in their microenvironment before apoptosis. Growth factors reduce retinal degeneration and apoptosis in animal models. Materials & methods: The data of 188 eyes of 94 patients who were injected with autologous platelet-rich plasma (PRP) into the subtenon space three-times every 2 weeks were evaluated retrospectively. Results: Statistically significant improvements in visual acuity, visual field and fixation stability were detected after treatment. When the treatment response of the patients' better-seeing eye compared with the response of the other eye, there was no statistically significant difference. Conclusion: The PRP treatment has a favorable effect on visual functions in patients with RP. This approach is promising as it is safe and easy.

Protocol: A systematic review and meta-analysis of the role of fetal and infantile environmental exposure in etiopathogenesis of infantile hypertrophic pyloric stenosis.

Infantile hypertrophic pyloric stenosis (IHPS) is one of the hallmark pediatric surgical diseases. However, its etiology remains incompletely understood. By systematically reviewing the literature, we aim to clarify the effect of the effect of occupational and environmental factors and role of nitric oxide (NO) metabolism in the etiopathogenesis of IHPS. The systematic review is drafted with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement (PRISMA) and the Meta-analysis of Observational Studies in Epidemiology (MOOSE). Systematic literature search will be performed for the period 2000 (Jan) to 2020 (Dec) in the databases: MEDLINE, EMBASE, PubMed. The systematic search will cover the literature in English and Turkish language and will be limited to studies on human subjects. Four investigators will independently search the databases (MEDLINE, EMBASE, PubMed) according to the defined search strategy. The full-text of the selected articles will be screened independently by four reviewers, against the inclusion criteria. Descriptive data will be extracted from each study regarding: study details, methods, participants, outcomes and calculations of association for potential further statistical analysis. If meta-analysis could not be undertaken, systematic approach to analyzing the findings of included multiple studies will be described. Heterogeneity will be assessed by quantifying the inconsistency across studies using I2 statistic. Statistical analysis will be performed using Comprehensive Meta-Analysis Version 3.0 software. The p values lower than 0.05 will be considered statistically significant for all analyses.

The effect of azygos vein preservation on postoperative complications after esophageal atresia repair: Results from the Turkish Esophageal Atresia Registry.

AIM: Preservation of the azygos vein (AV) maintains normal venous drainage of the mediastinum and decreases postoperative congestion. The modification of esophageal atresia (EA) repair by preserving AV may prevent postoperative complications and may lead to better outcomes. The data from the Turkish Esophageal Atresia Registry (TEAR) were evaluated to define the effect of AV preservation on postoperative complications of patients with EA. METHODS: Data from TEAR for a period of five years were evaluated. Patients were enrolled into two groups according to the preservation of AV. Patients with divided (DAV) and preserved AV (PAV) were evaluated for demographic and operative features and postoperative complications for the first year of life. The DAV and PAV groups were compared according to the postoperative complications, such as fistula recanalization, symptomatic strictures, anastomotic leaks, total number of esophageal dilatations, and anti-reflux surgery. In addition, respiratory problems, which required treatment, were compared between groups. RESULTS: Among 502 registered patients; the data from 315 patients with the information of AV ligation were included. The male female ratio of DAV (n = 271) and PAV (n = 44) groups were 150:121 and 21:23, respectively (p > 0.05). The mean body weight, height, gestational age, and associated anomalies were similar in both groups (p > 0.05). The esophageal repair with thoracotomy was significantly higher in DAV group, when compared to the PAV group (p < 0.05). The rates of primary anastomosis and tensioned anastomosis were similar in both groups (p > 0.05). There was no difference between DAV and PAV groups for anastomotic leaks, symptomatic anastomotic strictures, fistula recanalization, and the requirement for anti-reflux surgery (p > 0.05). The rate of respiratory problems, which required treatment, was significantly higher in the DAV group (p < 0.05) CONCLUSION: The data in the TEAR demonstrated that preserving the AV during EA repair led to no significant advantage on postoperative complications, with exception of respiratory problems. AV should be preserved as much as possible to maintain a normal mediastinal anatomy and to avoid respiratory complications.

Management of cystoid macular edema secondary to retinitis pigmentosa via subliminal micropulse yellow laser.

To investigate the effects of subliminal micropulse yellow laser application on the central macular thickness and best-corrected visual acuity in cystoid macular edema secondary to retinitis pigmentosa patients. This prospective open-label clinical trial, conducted between January 2018 and October 2019, included 32 eyes of 29 patients who had cystoid macular edema secondary to retinitis pigmentosa. Patients were treated by subliminal micropulse yellow laser for one session. Central macular thickness and best-corrected visual acuity changes were investigated just before the treatment and 1 year later after the one session of the treatment. The mean central macular thickness was 651.3 µm before the treatment and 247.7 µm at 12 months after the treatment. The decrease in mean central macular thickness was statistically significant (p = 0.01). Median best-corrected visual acuity was 66.8 ETDRS letters before the treatment and 70.0 letters at 12 months after the treatment. The increase in best-corrected visual acuity was not statistically significant (p = 0.18). Eighty-six percent of the patients stated that the quality of central vision increased and that color vision, contrast sensitivity, and distortion improved. We did not encounter any serious adverse events related to the application of subliminal micropulse yellow laser. The subliminal micropulse yellow laser seems to be a therapeutic, effective, and safe option for the treatment of non-inflammatory and resistant cystoid macular edema secondary to retinitis pigmentosa patients. ClinicalTrials.gov ID: NCT04234438, January 17, 2020.

Further defining the clinical and molecular spectrum of acromesomelic dysplasia type maroteaux: a Turkish tertiary center experience.

Acromesomelic dysplasia type Maroteaux (AMDM, OMIM #602875) is an autosomal recessive disorder characterized by severe short stature, shortened middle and distal segments of the limbs, redundant skin of fingers, radial head subluxation or dislocation, large great toes and cranium, and normal intelligence. Only the skeletal system appears to be consistently affected. AMDM is caused by biallelic loss-of-function variants in the natriuretic peptide receptor B (NPRB or NPR2, OMIM #108961) which is involved in endochondral ossification and longitudinal growth of limbs and vertebrae. In this study, we investigated 26 AMDM patients from 22 unrelated families and revealed their genetic etiology in 20 families, via Sanger sequencing or exome sequencing. A total of 22 distinct variants in NPR2 (14 missense, 5 nonsense, 2 intronic, and 1 one-amino acid deletion) were detected, among which 15 were novel. They were in homozygous states in 19 patients and in compound heterozygous states in four patients. Parents with heterozygous NPR2 variants were significantly shorter than the control. Extra-skeletal abnormalities, including global developmental delay/intellectual disability, nephrolithiasis, renal cyst, and oligodontia were noted in the patient cohort. The high parental consanguinity rate might have contributed to these findings, probably associated with other gene variants. This study represents the largest cohort of AMDM from Turkey and regional countries and further expands the molecular and clinical spectrum of AMDM.