An unexpected infection in loss-of-function mutations in STAT3: malignant alveolar echinococcosis in liver

Loss-of-function (LOF) mutations in signal transducer and activator of transcription 3 (S TAT 3) gene causes autosomal dominant hyper immunoglobulin E syndrome (AD-HIES or Job's Syndrome), a rare and complex primary immunodeficiency (PID) syndrome characterized by increased levels of IgE (>2000 IU/mL), eosinophilia, recurrent staphylococcal skin abscesses, eczema, recurrent pneumonia, skeletal and connective tissue abnormalities. Although bacterial and fungal infections are common in AD-HIES, susceptibility to parasitic infections has not been reported. Alveolar echinococcosis (AE), a zoonosis caused by the growth of the Echinococcus multilocularis (EM) metacestode, mimics slow-growing liver cancer. The mortality rate of AE is very high when it is diagnosed late or undertreated. Here, we report a 14-year-old boy with AE infections of the liver and the lung resulting in liver failure and diagnosed as STAT3-LOF. To our knowledge, the association between these two conditions has not been reported in the literature before

Rapid intraoperative tissue expansion with Foley catheter in a challenging cripple Hypospadias

ABSTRACTFailed hypospadiass cases may result in hypovascular, scarred penis with residual penile chordee and leave the patient with minimal residual skin for penile resurfacing and urethroplasty. Local tissue expansion has become a good alternative to provide skin for penis by using expanders however they require long periods of time for expansion. Besides, rapid tissue expansion was also described in different tissues. We used rapid intraoperative expansion technique by using a Foley catheter in a failed hypospadias case who had minimal residual skin secondary to infection and we concluded that rapid intraoperative tissue expansion with Foley catheter is an effective, feasible reconstructive method for easy dissection and penile resurfacing in failed hypospadiass cases.</p

A Complication in Hypospadias Surgery Due to Anchoring Suture

The complication rates are still 1–90% both in proximal and distal hypospadias regardless of the surgeon’s experience and the usage of most developed techniques. The literature survey revealed few complications of glans penis including meatal stenosis, prolapsus and retraction. Despite our literature survey, we could not find any article concerning the permanent scar tissue at the dorsal part of glans following hypospadias surgeries. A new complication can be added to these, concerning glans which is the formation of postoperative scar tissue as a reaction to suture material used in traction, stent anchoring or both. The aim of this paper is to demonstrate four cases which had glanular scar due to traction suture following the surgical procedures for hypospadias repair

A Complication in Hypospadias Surgery Due to Anchoring Suture

Bahadir, Gulnur Gollu/0000-0001-8163-2226WOS: 000360366100014PubMed: 26180503The complication rates are still 1-90% both in proximal and distal hypospadias regardless of the surgeon's experience and the usage of most developed techniques. The literature survey revealed few complications of glans penis including meatal stenosis, prolapsus and retraction. Despite our literature survey, we could not find any article concerning the permanent scar tissue at the dorsal part of glans following hypospadias surgeries. A new complication can be added to these, concerning glans which is the formation of postoperative scar tissue as a reaction to suture material used in traction, stent anchoring or both. The aim of this paper is to demonstrate four cases which had glanular scar due to traction suture following the surgical procedures for hypospadias repair

Danger in unlabeled bottles: Analysis of corrosive substances

KISA, Ucler/0000-0002-8131-6810; Bahadir, Gulnur Gollu/0000-0001-8163-2226; Soyer, Tutku/0000-0003-1505-6042WOS: 000351642900018PubMed: 25698279

Cognitive and behavioral characteristics of children with caustic ingestion

Kilic, Birim Gunay/0000-0002-4566-2564; Bahadir, Gulnur Gollu/0000-0001-8163-2226; Sertcelik, Mehmet/0000-0001-7031-3318; Soyer, Tutku/0000-0003-1505-6042WOS: 000352140500009PubMed: 25840059Background/aim: Children with attention-deficit/hyperactivity disorder (AD/HD) have risk taking behavior and are more prone to sustaining injury. It is aimed to evaluate the cognitive and behavioral characteristics of children with caustic ingestion. Patients and methods: Ninety two children with a history of nonsuicidal caustic ingestion (CI, n = 46) and healthy subjects (HS, n = 46) admitted to pediatric surgery department were enrolled into the study. Patients in groups were evaluated for age, sex, number of siblings and educational status of the parents. Before filling the questionnaires, the children were undergone flexible endoscopy and treated accordingly. Conners Parents Rating Scale-revised long form (CPRS-R:L), validated for Turkish Children, was used to evaluate the cognitive and behavioral characteristics of children. Parents rate their child's behavior with a four-point Likert scale. Subscales of CPRS-R: L including cognitive problems/inattention (CG/I), hyperactivity (H), attention deficit hyperactivity disorder index (AD/HD-I), Conners' Global Index-discomfort-impulsivity (CGI-DI), DSM-IV-symptom subscale-inattention (DSMIV, SS-I), DSM-IV-symptom subscale-hyperactivity-impulsivity (DSM-IV, SS-HI), DSMIV-symptom subscale-total score (DSM-IV SS-T) were used to determine the severity of the AD/HD symptom. Demographic features and cognitive/behavioral characteristics of children with caustic ingestion were compared with healthy subjects. Results: The median age of the patients was 4 (2-14 years) in both CI and HS groups. Female male ratio was 13:33 in CI and 12:34 in HS. Sixty seven percent of patients were preschool children (younger than 5 years of age) in both CI and HS groups. There was no difference between groups for number of siblings (p > 0.05). Parents of HS group had higher educational status than parents in CI (p < 0.05). When subscale scores of CPRS-R: L compared between CI and HS groups, CI group had higher CGI-DI scores than HS (p < 0.05). Children younger than five years of age had higher scores of H, emotional instability and total CG/I in CI than HS group (p < 0.05). Conclusion: Children with caustic ingestion had impulsiveness behavior when compared to healthy children. In addition to impulsivity, hyperactivity can be also assessed as a risk factor for caustic ingestion in children younger than 5 years of age. We suggest that association between AD/HD behavior and risk of sustaining injuries was also confirmed for caustic ingestion in children. (C) 2015 Elsevier Inc. All rights reserved

Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry

Introduction The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). Materials and Methods The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW 1,500g), LWB=1,500-2,500g), and normal BW (NBW; >2,500g). Results Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups ( p 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases ( p 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis ( p 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p 0.05). According to Spitz's 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 diseases, pneumonia, and sepsis. Conclusion The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA

The Effects of Local and Systemic Growth Hormone Treatment on Germ Cell Population and Fertility in an Experimental Unilateral Testicular Torsion and Orchiectomy Model

Purpose: We evaluated the effects of local and systemic growth hormone on the germ cell population of the contralateral testes of pubertal rats subjected to unilateral testicular torsion and orchiectomy 24 hours later