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. Comparative transcriptomic assessment of PTL tissue and stromal cells showed differential expression of 257 genes in PTL tissue and 189 genes in stromal cells. Notably, several transcription factors and regulators, including FOSB, FOS, JUN, and ATF3, were upregulated in PTL tissue. Additionally, genes associated with the extracellular matrix and connective tissue, such as COL1A1, MMP2, and SPARC, were significantly altered, indicating possible fibrotic changes. Gene set enrichment analysis highlighted the role of vascular development and extracellular matrix organization, and the Activator Protein-1 (AP-1) transcription factor was significantly activated in PTL tissue. Furthermore, the analysis highlighted an overlap of 25 genes between PTL tissue and stromal cells, underscoring the importance of shared molecular pathways in the pathogenesis of PTL. Among the shared genes, JUND, COL4A2, COL6A2, IGFBP5, and IGFBP7 were consistently upregulated, highlighting the possible involvement of AP-1-mediated signaling and fibrotic changes in the pathogenesis of PTL. The present findings pave the way for further research into the molecular mechanisms underlying PTL and offer novel insights for therapeutic interventions. Given the rarity of PTL, these molecular findings represent a significant step forward in our understanding this enigmatic disease.

Obliteration of the Processus Vaginalis After Testicular Descent

The testis develops in the abdominal cavity and descends into the scrotum. Although numerous theories have been proposed, the mechanism of descent and the reason for its inhibition remain unknown. Furthermore, none of the explanations account for the other occurrences related to the descent, such as failed obliteration of the processus vaginalis, or the reasons for the decrease in fertility and increase in the risk of malignancy associated with an undescended testis. The gubernaculum is a primitive mesenchymal tissue that was first described in 1786. However, the role of the gubernaculum in the descent process remains obscure. The testis descends through the processus vaginalis. Although the processus vaginalis (PV) is usually defined as a simple peritoneal protrusion, it actively develops into the gubernaculum. The gubernaculum gives rise to the smooth muscles that surround the processus vaginalis. The striated cremaster muscle (CM) is also derived from the gubernaculum. Because the testis descends through the processus vaginalis, the muscles develop to propel the testis. After propelling the testis, the smooth muscle (SM) undergoes programmed cell death. The initiation of programmed cell death through the intrinsic pathway requires activation of phospholipase C. A transient shift in the autonomic balance via a decrease in the sympathetic tonus and an increase in the parasympathetic tonus is essential for initiating this programmed cell death. Programmed cell death in the SM is the physiological pathway for the obliteration of the processus vaginalis. Differences in the timing, intensity, or duration of this physiological pathway result in pathological conditions. A shift before testicular descent diminishes the SM content that is required to propel the testis, and thus inhibits descent. The early shift persists throughout childhood and results in the decrease in fertility and increase in the risk of malignancy because of the differences in signal transduction. Despite a successful descent, persistence of the shift alters the contractility of the CM by increasing the cytosolic calcium levels. Contracted CMs retracts or even ascends the testis. Inadequate intensity or duration of the shift of autonomic tonus causes failure of the programmed cell death. Persistence of the SM hinders the obliteration of PV and gives rise to hydroceles or inguinal hernias depending on the amount of residual smooth muscles. Similar findings from different countries support these explanations. Thus, our proposed mechanism satisfactorily explains the process of descent while considering all the factors related to the process of testicular descent.

The Role of Oral Feeding Time and Sham Feeding on Oropharyngeal Swallowing Functions in Children with Esophageal Atresia.

A retrospective study was performed to evaluate the role of oral feeding (OF) time and sham feeding (SF) on oral-pharyngeal swallowing functions in children with esophageal atresia (EA). Patients with EA were evaluated for age, sex, and types of atresia, time to start OF and surgical complications. Three to six weeks after full OF, videofluoroscopic swallowing evaluation (VFSE) was performed, and Penetration Aspiration Score (PAS, no aspiration = 1, penetration = 2-5, aspiration = 6-8), delay in swallowing reflex and residue after liquid and pudding consistency were noted. Patients were divided into three groups according to repair time; early primary repair (EPR, < 1 month of age), delayed primary repair (DPR, ≥ 1 month of age) and colonic interposition (CI). VFSE findings were compared between groups. In CI group, patients without aspiration in VFSE were encouraged to sham SF before CI. Patients with and without SF in CI group were also compared for oro-pharyngeal dysphagia (OPD). PAS scores were significantly higher in DPR (n = 13) group when compared to CI group (n = 12) in liquid swallowing (p = 0.032) and higher than EPR (n = 30) in pudding consistency (p = 0.03). Patients with CI showed significant OPD when compared to EPR (p = 0.017). Swallowing reflex delay was similar in both groups (p > 0.05). DPR group had significantly higher liquid residue at the level of vallecula (p = 0.028). The residue at other levels (oral, tongue base, pharyngeal wall and pyriform sinus) were similar in all groups in both liquid and pudding consistencies. There was no significant difference between sham-fed (n = 6) and not sham-fed (n = 6) infants in VFSE of OPD (p > 0.05). Patients with DPR without SF had significantly higher incidence of OPD and PAS scores when compared to EPR and CI. Although CI groups has the latest OF time, SF may improve oral motor abilities and cause better OF swallowing functions than patients with delayed repair.

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). Therefore, a prospective study was performed to evaluate the miRNA-24 and 21 expression, and the matrix metalloproteinase-7 (MMP-7) levels in EBC of children with PSP. Children with PSP were evaluated for age, gender, clinical features and results of surgical treatment. EBC samples (500-1000 ml) were collected to evaluate the miRNA-21, 24 expressions, and MMP-7, and tissue-inhibitor-MMP-1 (TIMP-1) levels. miRNA expressions and MMP levels of patients were compared with healthy controls (control group (CG),n= 12). Subjects (n= 16) with a mean age of 15 years (10-19 years), and a male-to-female ratio of 14:2 were enrolled in this study. The most common presenting symptom was sudden chest pain (n= 14). In 62.5% of the cases an underlying bullous lung disease were detected. During an average of 16.6 months (1-60 months) follow up period, four subjects relapsed. The mean MMP-7 (1.74-1.57 ng ml-1), and TIMP-1 (1.92-1.84 ng ml-1) levels were similar between both groups (p> 0.05). miRNA-24 expression was significantly decreased in the PSP group, when compared to the CG (0.16-1 2-ΔΔCT,p< 0.05). In addition, the miRNA-21 expression was not different between the two groups (p> 0.05). In conclusion, the miRNA-24 levels were significantly decreased in children with PSP. Taken together, children with PSP, especially those with bullous disease, should be closely monitored in the long-term period.

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.

Clinical Features and Outcomes of Gallbladder Polyps in Children.

BACKGROUND: Gallbladder polyps are rare lesions protruding into the gallbladder lumen with variable clinical presentation. No standard treatment algorithm has been developed for pediatric gallbladders, and the malignant potential of the gallbladder is not clear in children. Therefore, a retrospective study was performed to evaluate the clinical features and treatment options of gallbladder polyps in children. METHODS: Between 2014 and 2020, children diagnosed with gallbladder polyps were evaluated for age, gender, clinical features, results of follow-up with ultrasound findings, and treatment options retrospectively. RESULTS: The records of 15 patients with a mean age of 13.2 years (2-20 years) were included. The male: female ratio was 7 : 8. Gallbladder polyps was detected incidentally in 73.3% (n = 11) of the patients. Four (20%) of the patients were symptomatic (26.7%) and complained about abdominal pain. Laboratory tests were normal except in 3 patients who showed slightly increased liver function tests. Two of the patients had 3 polyps on admission. The polyps were 2-10 mm in size. The size of the polyp increased in 5 patients (33.3%) and disappeared in 4 patients (26.6%) in follow-up ultrasound examinations. Five of the patients underwent cholecystectomy and 1 of them was scheduled for surgery. Five of the asymptomatic patients who have polyps less than 10 mm in size are still on follow-up. In patients with cholecystectomy, the histopathology of gallbladders revealed cholesterol polyps (n = 2) and hyperplastic polyps (n = 2). One of the patients with cholecystectomy showed no polyps in histopathological evaluation. CONCLUSION: Despite the lack of a standardized algorithm, our data suggested that multiple polyps, polyps with increased in size or greater than 10 mm, and the presence of symptoms might require cholecystectomy in children. Asymptomatic patients with small-sized polyps can be identified using ultrasound, and the polyps may disappear during the subsequent follow-up.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

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.

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. Twelve children (80%) had been in known close contact with a person with SARS-CoV-19 and 13 were positive for Anti-SARS-CoV-2 IgG. All the children had experienced fever for at least 1 day and had signs of involvement of two or more systems. Three patients required surgical intervention: one underwent surgical exploration with a presumptive diagnosis of acute appendicitis in the referring center and was transported to our center following clinical deterioration, where a diagnosis of MIS-C was confirmed; and the remaining two developed appendicitis during hospitalization for COVID-19. All three patients had a longer duration of abdominal pain, a higher number of lymphocytes, and a lower level of inflammatory markers than the non-surgically managed patients. None of the patients presenting with MIS-C underwent surgical exploration. CONCLUSION: Gastrointestinal involvement may mimic acute abdomen in children with COVID-19. Thus, children presenting with acute abdomen in the pandemic era require careful evaluation and prompt diagnosis to avoid unnecessary surgical intervention.

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.

An unusual complication of tumor surgery: chylous leakage.

PURPOSE: This study was designed to evaluate the effectiveness of conservative treatment for chylous leak after tumor surgery and to propose a management algorithm. METHODS: The data of patients with postoperative chylous leak after tumor surgery in our institution between 2010 and 2019 were retrospectively reviewed. In this study, 469 laparotomies, 89 thoracotomies, and 57 cervical excisions were performed for tumor surgery in our institution. RESULTS: Twelve patients with a median age of 4 (IQR, 3-8) years had postoperative chylous leak. All patients received total parenteral nutrition for a median of 13 days. Five patients had intravenous somatostatin for a median of 14 days (IQR, 9-16) to decrease chyle production. Eventually, chylous leak ceased in all patients with conservative treatment and surgical drains were removed after no leak was observed with enteral feeding. CONCLUSIONS: The incidence of chylous leak in childhood tumor surgery is approximately 2%. Extended tumor resection and lymph node dissection lead to the injury of the delicate structures that drain chyle. Conservative treatment with total parenteral nutrition and somatostatin seems to be effective. In particular, somatostatin may be used in resistant cases. Conservative treatment can take up to 1 month. The algorithm consists of how to manage postoperative chylous leak in childhood.

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.

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center.

AIM: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. MATERIAL AND METHODS: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. RESULTS: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18. CONCLUSION: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.

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. RESULTS: A total of 7 patients were included. The admission age was 3 days-10 years. The complaints were abdominal distention and constipation in all the patients. All the patients had passed meconium in the first 48 hours of their life. An upper gastrointestinal (GI) series revealed slow transit in 6 patients and malrotation in 2 patients. Full-thickness rectum biopsies revealed normal ganglion cells. Neurological examination revealed postinfectious pandysautonomy in 1 patient. Furthermore, 2 patients are under follow-up with ileostomy and TPN, 1 patient is with enteral feeding and ileostomy, and 3 patients are stable with pyridostigmine, enemas. Moreover, 1 patient died because of sepsis. The prognosis was not significantly correlated with initial presentation time, lag time, and presence of extraintestinal manifestations (p>0.05). The prognosis was significantly better when fewer number of operations were performed (p=0.029) Conclusion: PIPO is a broad-spectrum disease group that is difficult to diagnose and treat. It is mandatory to rule out the secondary causes of diagnosis. Medical and surgical treatments are used to support the nutritional status, prevent sepsis, and restore the intestinal motility. The prognosis was better when the secondary causes were identified and fewer operations were performed.

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. CASE: A 14-year-old girl was admitted with abdominal pain and elevated pancreatic enzyme levels. Abdominal US showed irregularity and rough echogenicity in pancreas revealing pancreatitis. Multiple stones were seen in main pancreatic duct on Magnetic resonance cholangiopancreatography (MRCP). Endoscopic retrograde cholangiopancreatography (ERCP) was performed and dilated pancreatic duct, thickened pancreatic secretion were detected. Endoscopic sphincterotomy was performed. Endoscopic removal of stones could not be achieved since the largest stone was 17x8 mm. Pancreatic extracorporeal shock wave lithotripsy (P-ESWL) was performed using electromagnetic lithotripter under general anesthesia. Following ESWL, fragmentation of stones in the main duct was confirmed with ERCP. After 3 sessions of ESWL, no ESWL-related complication was observed. Pain relief was achieved. The patient is still under follow-up regarding endocrine and exocrine function of pancreas. CONCLUSION: ESWL may be an effective and safe management option in pediatric PS which could not be removed by ERCP. The patients managed with ESWL should be followed-up for a long time regarding the endocrine and exocrine functions of the pancreas. As in management of adult pancreatitis, clinical guidelines are needed regarding the management of pediatric PS.

The results and outcome of primary spontaneous pneumothorax in adolescents.

AIM: Primary spontaneous pneumothorax (PSP) is a rare pulmonary pathology that occurs in the absence of known lung disease. A retrospective study was performed to evaluate the results and outcome of PSP treatment in adolescents. METHODS: The cases with PSP from January 2004 to December 2017 were evaluated for age, sex, family and smoking history, clinical and radiological findings and results of treatment. RESULTS: Ten cases with PSP were included. The mean age of the patients was 15 years (10-17 years) and the male to female ratio was 9:1. Two of the patients (20%) had family history of PSP and four cases (40%) had smoking history. The initial complaints were chest pain (n=8), acute onset of cough (n=1) and breathing difficulty (n=1). Tube thoracostomy was performed in nine cases in which three of them were bilateral. Chest computed tomography (CT) demonstrated bullae (n=4; 40%) and subpleural blebs (n=2; 20%). Pleurodesis with talc was performed in four patients with pneumothorax for longer than a week in follow-up (n=4; 40%). Five cases had recurrent PSP within one year (n=5; 50%) and underwent pleurodesis with talc (n=4), autologous-blood (n=1) and bleomycin (n=1). Bleb excision was performed in two cases with persistent pneumothorax despite pleurodesis. CONCLUSION: Tube thoracostomy and oxygen supplementation, are considered as initial and adequate treatment of PSP in most of the adolescents. Prolonged air leaks require pleurosdesis as the first line treatment and surgical excision of blebs should be reversed for the patients who are unresponsive to other treatment options.

New scoring system to predict foreign body aspiration in children.

PURPOSE: To propose a new scoring system to predict foreign body aspiration (FBA) in children. METHODS: Children who underwent bronchoscopy for FBA were evaluated for age, sex, history of aspiration, physical examination, radiological findings and results of bronchoscopy retrospectively. A new proposed FBA scoring including statements about history, physical and radiological findings were applied to all patients to define a total score. The results of each statement and total FBA score were compared according to bronchoscopy findings. The sensitivity and specificity of FBA score and cut-off values of total FBA score to predict positive cases were evaluated. RESULTS: Totally 720 patients with a male to female ratio of 227:133 were included. FBA was noted in 52.1% (n=375) of cases. When the scoring system compared with the existence of foreign body (FB), the patient history had no statistical significance to predict positive cases (p>0.05). The existence of FB was significantly associated with physical examination, radiological findings and total FBA score (p<0.001). The revised scoring system without history parameters had range of total scores 0 to 5. The total scores were obtained by sum of physical findings and radiological parameters and the cut-off value of 2 had the highest diagnostic performance with sensitivity and specificity of 77.9% and 74.8% to predict FBA in children. CONCLUSION: Our results suggest that the proposed scoring system can be utilized to predict FBA in children. The total score higher than 2 is predictive for FBA. Although, physical and radiologic findings are strong parameters for positive bronchoscopy, the history of FBA has no diagnostic utility. LEVEL OF EVIDENCE: Level III (retrospective comparative study) STUDY TYPE: Diagnostic study.

The relationship between oxidative stress markers in exhaled breath condensate and respiratory problems in patients with repaired esophageal atresia.

AIM: To evaluate the relationship between respiratory problems and oxidative stress markers in exhaled breath condensate (EBC) of patients with esophageal atresia (EA). METHODS: EA cases with respiratory problems were evaluated retrospectively for age, gender, the type of atresia, surgical treatment, outcome and respiratory symptoms. The results of gastroesophageal reflux (GER) treatment including the use of proton pump inhibitor (PPI) and fundoplication were also documented. EBC samples of 500-1000 µl were obtained by Ecoscreen machine in all cases. The levels of Glutathione (Glut), 8-isoprostane (8-iso), cysteinyl-leukotriene (Cys-LT) were measured with ELISA. Results were compared with healthy control subjects (CG, n = 26) and the relationship between oxidative stress markers and respiratory symptoms was evaluated. The results of GER treatment and oxidative stress markers in EBC were also correlated. RESULTS: Twenty-nine patients with a mean age of 8.8 years (3-14 years) were included. The male/female ratio was 16:13. The EA presented with distal fistula in 27 cases. While no fistula was observed in 1 case, both proximal and distal fistulae were present in another single case. Associated anomalies, most of which were cardiovascular anomalies, were observed in 65.5% (n = 19) of cases. The median Glut level was 1.03 mM/ml (0.93-1.15), iso-8 was 38.8 pg/mL (32.03-76.2) and Cys-LT was 0.44 pg/mL (20.17-61.3) in patients with EA. The median levels of oxidative markers in CG were 1.23 mM/mL (1.13-1.36), 66.3 pg/mL (33.5-106.7), and 56.9 pg/mL (27.4-80.1), respectively. Glut levels were significantly lower in EA cases compared to CG (p = 0.01). There was no significant difference between the groups regarding 8-iso and CYS-LT levels (p = 0.9, p = 1.0). Cys-LT levels were significantly lower in patients with PPI treatment [21.7 pg/mL (18.6-48.1)], when compared to patients without PPI treatment [41.1 pg/mL (22.5-83.1)] (p = 0.04) and healthy subjects [56.9 pg/mL (27.4-80.1)] (p = 0.017). The 8-iso levels were significantly decreased in cases with fundoplication compared to the patients without fundoplication (p = 0.02). CONCLUSION: Glut - an antioxidant agent - levels were significantly lower in EBC of EA cases. The decrease in Cys-LT levels in cases with PPI treatment and in 8-iso levels in patients with fundoplication suggests that the oxidative damage in EBC of EA cases may be correlated with GER and its management. TYPE OF STUDY: Case control study LEVEL OF EVIDENCE: Level III.