AP-1-dependent fibrosis: Exploring its potential role in the pathogenesis of placental transmogrification of the lung (PTL) via tissue-level transcriptome analysis.

Placental transmogrification of the lung (PTL) is a rare pulmonary condition characterized by the presence of immature placental villous structures. The etiology and molecular mechanisms underlying this disease remain largely unknown. This functional study aimed to identify the molecular signatures in the pathogenesis of PTL via comprehensive transcriptome analysis.

A novel GRK2 variant in a patient with Jeune asphyxiating thoracic dysplasia accompanied by Morgagni hernia.

Skeletal ciliopathies constitute a subgroup of ciliopathies characterized by various skeletal anomalies arising from mutations in genes impacting cilia, ciliogenesis, intraflagellar transport process, or various signaling pathways. Short-rib thoracic dysplasias, previously known as Jeune asphyxiating thoracic dysplasia (ATD), stand out as the most prevalent and prototypical form of skeletal ciliopathies, often associated with semilethality. Recently, pathogenic variants in GRK2, a subfamily of mammalian G protein-coupled receptor kinases, have been identified as one of the underlying causes of Jeune ATD.

Dynamic Imaging Grade of Swallowing Toxicity in Children with Esophageal Atresia.

Introduction:  The Dynamic Imaging Grade of Swallowing Toxicity (DIGEST) scale was developed to evaluate the safety, efficiency, and overall pharyngeal swallowing performance in patients with dysphagia (DIGESTs, DIGESTe, and DIGESTt, respectively). Although various types of swallowing dysfunction are encountered in children with esophageal atresia (EA), oropharyngeal dysphagia poses risk for aspiration. Therefore, a retrospective study was performed to evaluate the safety and efficacy of swallowing by using DIGEST score in children with EA.

Effect of prostaglandin-E1 treatment on pyloric wall thickness in newborns with ductal-dependent critical congenital heart diseases.

Background: Prostaglandin E1 (PGE1) is used in the medical treatment of ductal-dependent critical congenital heart disease (CCHD) in neonates. Apnea/bradycardia, hypotension, hypokalemia, and fever are the most important side effects of PGE1. Moreover, gastric outlet obstruction has been reported in a few case reports.

The miRNA-24, miRNA-21 expressions and matrix metalloproteinase-7 level in exhaled breath condensate of children with primary spontaneous pneumothorax.

Bullous lung diseases may cause primary spontaneous pneumothorax (PSP) in children. The microRNAs (miRNAs) are non-coding RNAs that participate in regulation of inflammation and cancer. We hypothesized that children with bullous lung disease and PSP may have altered miRNA expressions in their exhaled breath condensates (EBCs).

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.

Lung ultrasound findings in children with foreign body aspiration.

Objectives: Foreign body aspiration (FBA) has a wide clinical spectrum, patients may be asymptomatic or present with cardiopulmonary arrest. Radiological imaging methods are used in addition to history and physical examination findings for certain diagnosis. Lung ultrasonography (LUS), whose usage area is increasing year by year, can be included in these.

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.

Continuous Hepatogonodal and Splenogonogal Fusion: A Rare Cause of Bilateral Intra-Abdominal Testis in an 18-Month-Old Boy.

The fusion of gonadal structures with internal organs is very rare. The close proximity between the left gonad and spleen during embryogenesis may result in splenogonadal fusion (SGF). Moreover, the trapping of hepatocyte-destined mesenchyme cells in gonads is defined as hepatogonadal fusion (HGF).

Management of acute abdomen during the active disease course of COVID-19 and multisystem inflammatory syndrome in children.

Purpose: To evaluate the management of children with severe gastrointestinal symptoms during the disease course of COVID-19 and multisystem inflammatory syndrome (MIS-C).

Methods: After ethical approval, we reviewed the medical records, retrospectively, of children with COVID-19 or MIS-C requiring surgical consultation for severe gastrointestinal symptoms.

Results: The subjects comprised 15 children, 13 with MIS-C and 2 with COVID-19.

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.

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).

A rare congenital esophageal anomaly mimicking an isolated esophageal atresia: Kluth Type IV membranous esophageal atresia.

Congenital esophageal web is a very rare type of esophageal atresia (EA) and is considered as Type IV atresia in Kluth's classification. Type IV EA is further classified into subgroups according to the location of web and the presence of tracheoesophageal fistula (TEF). Herein, we present a newborn having Type IV EA who was initially suspected as isolated EA and has co-existing malrotation together with the review of the literature.

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 effects of ozone on the acute phase of intestinal ischemia-reperfusion injury in rats.

Background: In this study, we aimed to examine the therapeutic effects of ozone on the acute phase of intestinal ischemia-reperfusion (I/R) injury in rats to resemble clinical practice.

Methods: Eighteen Wistar albino rats were assigned to control (CG, n=6), sham (SG, n=6) and ozone groups (OG, n=6). A midline laparotomy was performed and a superior mesenteric artery (SMA) in the SG and OG was occluded with a 0/0 catgut suture, but in the CG, the incision was closed without any intervention.

Challenges in management and prognosis of pediatric intestinal pseudo-obstruction.

Background/aims: Pediatric intestinal pseudo-obstruction (PIPO) is the most severe form of intestinal dysmotility in children. This study aims to present the cases of PIPO to discuss its diagnosis, management, and prognosis.

Materials And Methods: We retrospectively analyzed the medical records of the patients with PIPO between 2010 and 2018.

Extracorporeal shock wave lithotripsy in the management of a 14-year-old girl with chronic calcific pancreatitis.

Background: Chronic pancreatitis is very rare in childhood and causes chronic/relapsing abdominal pain, frequent hospitalizations, malnutrition, growth retardation, and stone formation in the main duct. Although pancreatic extracorporeal shock wave lithotripsy (P-ESWL) is commonly used in the treatment of pancreatic stones (PS) in adults, the use in children is still controversial. An adolescent girl with multiple PS is presented to discuss the use of ESWL as a treatment alternative in children with PS.

Pyloroduodenal intussusception due to diffuse juvenile polyposis in a 3 year-old child: case report.

Background: Pyloroduodenal intussusception (PDI) due to gastric and pyloric polyps is very rare and has not been reported previously in children.

Case: A 3 year-old boy was admitted with non-bilious vomiting and abdominal distention. Abdominal X-ray showed gastric air-fluid level and ultrasonography showed 5 cm intussusception at right upper quadrant.

Cystoscopy-Guided Laparoscopic Excision of Prostatic Utricle: Report of a Case.

Prostatic utricle (PU) is incomplete regression of Müllerian duct and may cause recurrent urinary tract infections (UTIs), stone formation, postvoid dribbling, and recurrent epididymitis. Although surgical excision is recommended to avoid complications, surgical access to PU has been challenging. Cystoscopy-guided laparoscopic management of PU in a 3-year-old boy is reported to discuss use of other endoscopic aids in the surgical treatment of PU.

A rare cause of acute abdominal pain in a patient with Primary ciliary dyskinesia with situs inversus totalis.

Primary ciliary dyskinesia (PCD) is a rare, genetic disease characterized by ciliary dysfunction. Patients may present with respiratory distress during neonatal period; chronic sinopulmonary disease, bronchiectasis, recurrent otitis media, sinusitis and infertility in later periods. About 50% of PCD patients have situs inversus totalis and 6-12% have situs ambiguous known as heterotaxy syndromes.

Assessment of pediatric cricopharyngeal achalasia with high resolution manometry.

Boybeyi Türer Ö, Demir N, Ciecieraga T, Günaydın RÖ, Soyer T. Assessment of pediatric cricopharyngeal achalasia with high resolution manometry. Turk J Pediatr 2019; 61: 804-809.