Gastrointestinal complications in pediatric lung transplant recipients: Incidence, risk factors, and effects on patient outcomes.

BACKGROUND: Gastrointestinal (GI) complications in lung transplant recipients can occur any time during the post-operative period, leading to prolonged morbidity and mortality. Despite the negative association between GI complications and patient outcomes, little is known about their incidence and risk factors for their development in pediatric lung transplant recipients. METHODS: We performed a retrospective chart review at one pediatric tertiary center to describe the frequency of GI complications in lung transplant recipients. We identified potential risk factors for the diagnosis of gastroparesis, gastroesophageal reflux disease (GERD) and aspiration in the post-transplant period. Lastly, we investigated the association of these complications with mortality and graft survival. RESULTS: 84.3% of lung transplant recipients experienced at least one GI complication in the post-transplant period. Gastroparesis (52.9%), GERD (41.2%), and oropharyngeal dysphagia/laryngeal penetration (33.3%) were the most common complications diagnosed. Post-operative opioid exposure was a risk factor for gastroparesis, with the odds increasing 3.0% each day a patient was prescribed opioids (p = .021). The risk of death or retransplant in individuals who experienced gastroparesis was 2.7 times higher than those not diagnosed with gastroparesis (p = .027). CONCLUSION: Exposure to opioids in the post-operative period is a risk factor for gastroparesis and a prolonged hospitalization placed patients at risk for aspiration. Gastroparesis was associated with increased patient mortality and graft failure, while aspiration and GERD had no effect on long term outcomes. Future prospective studies investigating the relationship between opioid use and the development of a gastroparesis are necessary to improve patient outcomes.

Pneumatosis intestinalis after thoracic organ transplantation in children: A case series and review of the literature.

Pneumatosis intestinalis (PI) is a rare complication after thoracic organ transplantation. There are several theories for explaining the pathophysiology of this disease. In this paper, we highlight three cases of PI in a single pediatric center, one after lung transplantation and two after heart transplantation. Although the presentations differed, all cases improved with non-surgical therapies. There are not many articles in the pediatric literature about post-transplantation PI, and there are still many questions regarding the incidence, etiology, and treatment for this disease.

Extracorporeal membrane oxygenation and paracorporeal lung assist devices as a bridge to pediatric lung transplantation.

BACKGROUND: "Bridging" is a term used to describe the implementation of various treatment modalities to improve waitlist survival while a patient awaits lung transplantation. ECMO and PLAD are technologies used to bridge patients to lung transplantation. ECMO and PLAD are cardiopulmonary support systems that help move blood forward while using an artificial membrane to remove CO2 from and add O2 to the blood. Recent studies showed that these technologies are increasingly effective in bridging patients to lung transplantation, especially with optimizing patient selection, implementing physical rehabilitation and ambulation goals, standardization of management decisions, and increasing staff experience, among other considerations. We review these technologies, their roles as bridges to pediatric lung transplantation, as well as indications, contraindications, complications, and mortality rates. CONCLUSION: Finally, we discuss the existing knowledge gaps and areas for future research to improve patient outcomes and understanding of lung assist devices.

Restrictive allograft syndrome after lung transplantation.

Despite recent advances over the past decade in lung transplantation including improved surgical technique and immunotherapy, the diagnosis and treatment of chronic lung allograft dysfunction remains a significant barrier to recipient survival. Aside from bronchiolitis obliterans syndrome, a restrictive phenotype called restrictive allograft syndrome has recently been recognized and affects up to 35% of all patients with CLAD. The main characteristics of RAS include a persistent and unexplained decline in lung function compared to baseline and persistent parenchymal infiltrates on imaging. The median survival after diagnosis of RAS is 6 to 18 months, significantly shorter than other forms of CLAD. Treatment options are limited, as therapies used for BOS are typically ineffective at halting disease progression. Specific medications such as fibrinolytics are lacking large, multicenter prospective studies. In this manuscript, we discuss the definition, mechanism, and characteristics of RAS while highlighting the similarities and differences between other forms of CLAD. We also review the diagnoses along with current and potential treatment options that are available for patients. Finally, we discuss the existing knowledge gaps and areas for future research to improve patient outcomes and understanding of RAS.

Intrinsic endobronchial obstructions in children from Turkey: evaluation of 2,555 flexible bronchoscopic procedures.

BACKGROUND: Endobronchial obstructions are rarely seen in children and are often misdiagnosed resulting in delay of definitive treatment. A variety of diseases can cause endobronchial obstructions in childhood, but data is limited as to the frequency, distribution and clinical characteristics of endobronchial obstructions diagnosed with flexible bronchoscopy (FB). OBJECTIVE: To document endobronchial obstructions detected by FB. METHODS: FB results from three pediatric pulmonology centers in Istanbul were evaluated. RESULTS: A total of 2,555 children underwent an FB procedure during the study period. Endobronchial obstructions were detected in 10% (n = 256) of the patients. Among FB in patients who had endobronchial obstructions, the four most common indications for bronchoscopy were persistent infiltrations (30%, n = 72), persistent wheezing (28%, n = 70), chronic cough (26%, n = 66) and atelectasis (23%, n = 59). The most common endobronchial obstructions detected in the patients were aspirated foreign bodies (35.9%, n = 92), endobronchial tuberculosis (31.6%, n = 81), mucous plugs occluding airway (16.7%, n = 43) and granulation scars (6%, n = 16). Other pathologies included hydatid cysts (n = 5), hemangiomas (n = 5), tumors (n = 5), submucosal nodules (n = 5) and polyps (n = 4). Endobronchial obstructions were most commonly located in the right bronchus (51%, n = 130) followed by the left bronchus (33%, n = 85), bilaterally (8%, n = 21) and trachea (8%, n = 20). CONCLUSIONS: Endobronchial obstructions can be caused by a number of different diseases which require various medical or surgical treatments. In the presence of clinical or radiological findings suggesting an endobronchial obstruction, FB should be performed promptly.

Dietary vitamin A intake and serum retinol concentrations of preschool children from different socio-economical levels in Izmir, Turkey.

AIM: Vitamin A deficiency (VAD), especially in its subclinical form, is a world health problem in young children. The aim of this study was to determine the prevalence of VAD among preschool children in various socio-economic groups in Izmir, Turkey. METHODS: One hundred and one children aged 24-59 months were selected for the study with cluster sampling method. A questionnaire was completed by the parents, and the dietary pattern was assessed by using a 3-day dietary record method. RESULTS: Mean serum retinol concentration of the children was 28.3 ± 10.2 µg/dL. Serum vitamin A concentration of two children (2%) was below 10 µg/dL (VAD), whereas 18 (18%) children had vitamin A concentrations between 10 and 20 µg/dL (subclinical deficiency). We did not find any relationship between mean serum retinol concentrations and the independent variables such as socio-economical levels, age groups, birth weight and gestational age. However, there was a statistically significant positive correlation between serum retinol concentrations and weight-for-age and weight-for-height Z scores of the children (p < 0.05). CONCLUSION: Because one-fifth of the children under 5 years have VAD in Izmir, Turkey, nutrition education must be given to the families during well-child care visits, and routine vitamin supplementation should be considered especially in children with malnutrition.

Rubella seroprevalence among healthy individuals in Izmir, Turkey.

In order to assess immunity to rubella in Izmir, Turkey, a total of 600 persons of 1-70 years of age were selected with cluster sampling. Of the 597 subjects, 120(20.1%) was susceptible to the rubella according to their serum antibody levels. Of children 1 to 6 years of age, 50.9% was found to be serologically susceptible to rubella. Rubella protection rates showed a age-related increase, reaching maximum in the 15-19 age group, in which 98.1% of these subjects had antibody titre above the full protective level. Of the 126 females in the reproductive age group, only 4(3.1%) were found to be serologically susceptible to rubella. Logistic regression analysis showed that among the several independent variables, only age (p< 0.001) was significantly associated with rubella protective antibody levels. Epidemiological studies should continue as the epidemiological characteristics of the disease may change depending on the uptake of MMR vaccination.

Scimitar syndrome associated with partial anomalous pulmonary venous draining into superior vena cava.

Scimitar syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.

Eponym. Scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.

Clinical Management of Children with Oropharyngeal Aspiration - Physician Survey.

Introduction: Pulmonary aspiration in infants and children is common, with 25% of the pediatric population reported to experience some type of nonspecific swallowing dysfunction. Diagnosing and managing oropharyngeal aspiration remain a challenging task due to the lack of distinguishing clinical or laboratory characteristics. We conducted a large, physician-based survey in an effort to determine the differences in the diagnosis and management of patients with oropharyngeal aspiration across different centers, using a quantitative online questionnaire. Methods: A survey was developed with the goal of understanding the physician demographics, diagnosis, management strategies, and the role of specialized centers for airway, voice, and swallowing disorders (also known as Aerodigestive Disease Centers). The questionnaire was sent to the pediatric membership of the American Thoracic Society. Results: A total of 136 questionnaires were completed and physicians from 42 different Aerodigestive Digestive Centers participated in the survey. Ninety-two percent of respondents believed that the evidence is unclear on the best methods for diagnosing lung disease related to oropharyngeal aspiration. Modified barium swallow (MBS) study and fiberoptic endoscopic evaluation of swallowing were the most useful studies in making the diagnosis of the oropharyngeal aspiration according to the participants. The majority of the participants preferred to repeat the feeding evaluations and MBS after the initial intervention. For children with suspected or confirmed aspiration, 68.6% of the respondents stated that they combine endoscopy and flexible and/or rigid bronchoscopy for further evaluation, while 27.1% preferred to perform the procedures individually at the discretion of each specialty provider. Inhaled corticosteroids were mostly prescribed if there was any component of bronchial hyperreactivity, and acid suppression therapy was primarily prescribed if there was a diagnosis or suspicion of gastroesophageal reflux disease. Empirical thickening of the feeds was not uniformly performed among the aerodigestive centers as a therapy method for the children with swallowing dysfunction with aspiration. Conclusion: In the survey, physicians mentioned about the importance of better guidelines for aspiration, the requirement of new diagnostic measures, the necessity of multicenter trials of sensitivity and specificity of current diagnostic tools, and developing a scoring system for reading the radiological studies for children with oropharyngeal aspiration.

A very rare cause of childhood paraparesis: primary intradural extramedullary spinal hydatid cyst.

Hydatid disease is caused by the larval form of Echinococcus. Lung and liver are the most commonly affected sites. Primary intradural extramedullary hydatid disease is extremely rare; a 13-year-old girl with primary intradural hydatid cyst who presented with symptoms of paraparesis is discussed in this article.

Cytoprotective effects of amifostine in the treatment of childhood malignancies.

BACKGROUND: Multi-systemic acute side effects occur, in response to intensive therapies that have been applied in childhood malignancies in recent years. Amifostine has rarely been used in the childhood cancers as a multisystemic protective agent for minimizing these side effects. PROCEDURE: In this study, the effectiveness of amifostine in combination with chemotherapy for childhood cancer treatment has been researched. Of 11 subjects (2.5 months-17 years) 4 subjects had leukemia, 4 had solid tumor, and 3 had lymphoma. For these 11 subjects, 29 chemotherapy courses were given in combination with amifostine, and 20 without amifostine. Their hematological, gastrointestinal and hepatic toxicity were evaluated according to the WHO toxicity criteria. Amifostine was given intravenously in a dose of 740 mg/m(2), one to three consecutive days depending on the chemotherapy regimen. RESULTS: The hemoglobin, leukocyte, and platelet levels of the two groups were not statistically different. However, when comparing the courses of the patients receiving the same medications at the same doses, in the group with amifostine, mean erythrocyte transfusion requirement was significantly reduced (P = 0.025). In 31% of the courses with amifostine and 50% of the courses without amifostine, febrile neutropenia developed. Gastrointestinal system and hepatic toxicity was significantly reduced in the courses with amifostine with respect to those without it (P = 0.001). Vomiting, hypotension and nausea were the only side effects related to amifostine. CONCLUSION: Use of amifostine during the treatment of childhood cancers with intensive chemotherapy and/or radiotherapy significantly reduced the erythrocyte transfusion requirements of the patients as well as gastrointestinal and hepatic toxicity.

Long-term effect of gluten-free diet on growth velocity in Turkish children with celiac disease.

We sought to analyze the long-term growth in children with celiac disease (CD) as well as the association of height and weight SD score at the end of 4 years with the demographic and clinical presentation at initial admission. Thirty-four children with CD were enrolled in the study and followed for at least 4 years. Patients were divided into three groups (Group 1: patients 10 years, at the time of diagnosis). Patients' charts were reviewed for demographic and clinical features at initial admission. Anthropometric measurements at initial admission, at 6th months, and 1st, 2nd, 3rd, and 4th years were recorded. Gluten-free diet led to rapid increase in weight SD and height SD score in patients

Juvenile psoriatic arthritis carrying familial Mediterranean fever gene mutations in a 14-year-old Turkish girl.

Juvenile psoriatic arthritis (JPsA) is characterized by asymmetric arthritis of big and small joints, enthesitis, dactylitis, psoriatic skin lesions and nail pitting. Investigators agree that JPsA is a relatively common chronic arthropathy of childhood that differs clinically, serologically, and genetically from both juvenile idiopathic arthritis and juvenile ankylosing spondylitis. Familial Mediterranean fever (FMF) is a multisystemic autosomal recessive disease occasionally accompanied by sacroiliitis. This is characterized by recurrent self-limited attacks of fever and accompanying abdominal, chest and arthricular pain. We present a 14-year-old Turkish girl with JPsA and carrying FMF gene mutations. In this patient, JPsA was diagnosed according to her physical, laboratory and skin biopsy findings and a treatment with methotrexate and sulfasalazine was started. As an inadequate clinical and laboratory response was obtained after the first month of therapy, the patient was investigated for FMF, and was diagnosed by molecular analyses of related gene (E148Q heterozygous/V726A homozygous mutation) besides clinical findings. After 2 weeks of the colchicine treatment, symptoms of the patient regressed and acute phase reactants decreased. To our knowledge, this is the first case presenting with psoriatic arthritis and FMF gene mutations together and responds to colchicine, methotrexate and sulfasalazine dramatically in clinical and laboratory findings. This case has been presented to remind that cases with psoriatic arthritis may also carry mutations in the MEFV gene.

Outcomes of mechanical support in a pediatric lung transplant center.

Pediatric lung transplantation is a lifesaving option for patients with end stage lung disease, although the scarcity of suitable donor organs results in long wait times and increased waitlist mortality. Many pediatric centers consider mechanical ventilatory support, such as long-term invasive ventilation and ECMO, a contraindication to lung transplantation. We hypothesized that current survival rates and outcomes for patients on mechanical ventilatory support in the pre-transplant period were not remarkably different. In our retrospective analysis we included patients between the ages of 0-21 years listed for lung transplantation from deceased donors between 2007 and 2014 at our institution. One-year survival outcomes were compared between three groups of patients: (i) patients bridged to transplant on ECMO (n = 6, 1-year survival = 67%); (ii) patients needing mechanical ventilation (either through endotracheal intubation or tracheostomy) but not ECMO (n = 12, 1-year survival = 75%); and (iii) patients who did not need endotracheal ventilation, tracheostomy, or ECMO (n = 25, 1-year survival = 88%). Comparison of outcomes of transplanted patients between these three groups were not statistically different in terms of successful hospital discharge and 1-year survival rates (P > 0.05). We believe that "bridging" the end-stage lung disease patient with long-term mechanical ventilation and/or ECMO support is a reasonable option in selected patients until suitable donors become available. Pediatr Pulmonol. 2017;52:360-366. © 2016 Wiley Periodicals, Inc.

MicroRNA expression profiling in children with different asthma phenotypes.

An improved understanding of the molecular mechanisms in asthma through exploring the role of microRNAs may offer promise to reveal new approaches for primary prevention and identification of new therapeutic targets in childhood asthma. The primary goal of this study is to identify the microRNAs that play a role in the pathogenesis of asthma in pediatric age group. The secondary goal is to analyze these microRNAs according to the asthma phenotype, atopic status, and severity of the disease exacerbation. To our knowledge, this is the first research project in the literature which studies the relationship between microRNA expression and the severity of childhood asthma. One hundred children between 6 and 18 years old with a diagnosis of asthma, and 100 age-matched healthy children were enrolled in this study, and the analyses of microRNA expression profiles were performed in the Medical Genetics Laboratories of Ege University between November 2009 and June 2010. The expression of 10 microRNAs were shown to be higher in patients with more severe asthma, and the expression of these microRNAs were also found to be higher in patients who present with more severe acute asthma exacerbation symptoms (P < 0.001). Also, five microRNAs were found to be expressed more than twofold in allergic patients when compared to non-allergic participants (P <0.001). Asthma is one of the best examples of complex genetic diseases, and further studies, which will investigate the relationship between these microRNA's and their target genes, are needed to learn more about the specific roles of microRNAs in respiratory diseases. Pediatr Pulmonol. 2016;51:582-587. © 2015 Wiley Periodicals, Inc.

Bronchoscopic evaluation in childhood pulmonary tuberculosis: risk factors of airway involvement and contribution to the bacteriologic diagnosis.

We aimed to describe the risk factors of airway involvement and to investigate the contribution of bronchoscopy in the bacteriologic diagnosis of tuberculosis. Airway involvement was more often present in patients with resistance to tuberculosis therapy than in the patients having bronchoscopy performed at initial presentation. Addition of bronchoalveolar lavage to the diagnostic workup increased the mycobacteriologic yield statistically.

Relapsing Polychondritis in a Nine-Year-Old Child Presenting with Subglottic Stenosis.

Relapsing polychondritis (RP) is a rare multisystem disorder characterized by inflammation and destruction of cartilaginous tissue. Atypical initial symptoms and signs may cause difficulties in the diagnosis of RP for pediatricians. In this article, we report a 9-year-old male who presented with stridor and hoarseness indicating upper respiratory tract involvement in the setting of chronic polyarthralgia. Flexible bronchoscopy revealed subglottic stenosis. Increasing awareness of early respiratory manifestations of RP will prevent life-threatening complications.

Upper airway abnormalities detected in children using flexible bronchoscopy.

OBJECTIVES: Rapid anatomical evaluation is essential to establish the severity of cases with upper respiratory obstruction and to define the degree of respiratory distress. Detailed airway endoscopy is required in most patients, not only for diagnosis, but also to treat the condition. In this study, as two of the largest paediatric pulmonology centres in Turkey, we reviewed the data of our bronchoscopy patients, and aimed to document the upper airway abnormalities that we detected during these procedures. PATIENTS AND METHODS: A retrospective analysis was made of the records of 1076 paediatric cases with pulmonary/airway disease who had undergone flexible bronchoscopy between 2007 and 2011. RESULTS: Upper airway malacia disorders were the most common (79.6%, n=259) bronchoscopic findings detected in the patients. The other most common pathologies were laryngeal edema (12.9%, n=42), external tracheal compression (12.3%, n=40), subglottic stenosis (4.0%, n=13), tracheal stenosis (2.8%, n=9), and vocal cord paralysis/irregularity (2.8%, n=9). The mean duration of symptoms was shortest in patients with vocal cord paralysis, and longest in patients with tracheal nodules (p<0.001). CONCLUSION: Paediatricians should keep in mind the possibility of malacia disorders and other congenital and acquired upper airway abnormalities in children with chronic respiratory problems. Diagnosis of underlying diseases, as soon as possible, permits the withdrawal of antibiotics or antiasthmatic drugs often used unnecessarily for long periods to treat these children.