Cross-cultural adaptation, reliability, and validity of the Turkish Pediatric Quality of Life Inventory-Cognitive Functioning Scale (PedsQLTM-CFS) in children with cancer.

The Pediatric Quality of Life Inventory-Cognitive Functioning Scale (PedsQLTM-CFS) was developed as a brief, general, symptom-specific tool to measure cognitive function. The 6-item PedsQL™ Cognitive Functioning Scale and PedsQL 3.0 Cancer Module answered 369 parents and 330 children with 5-18 years. Parents also completed Behavior Rating Inventory of Executive Function (BRIEF). The PedsQL™ Cognitive Functioning Scale evidenced excellent reliability (parent proxy-report α = 0.980/Fleiss Kappa: 0.794; children self-report α = 0.963/Fleiss Kappa: 0.790). Both child self-report and parent proxy-report PedsQL™ Cognitive Functioning Scale scores exhibited significant correlations with all parent-report BRIEF summary and subscale scores (p < .05). Both child self-report and parent proxy-report PedsQL™ Cognitive Functioning Scale scores exhibited significant correlations with PedsQL 3.0 Cancer Module total score and subscale scores (p < .05). The PedsQLTM-CFS can be used in high-risk populations with substantial to perfect reliability, both in regards to total/subcategory scores as well as in children with cancer.

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

Pediatric mediastinal tumors.

Mediastinal tumors are the most common thoracic tumor in the pediatric population. They include a spectrum of tumors, and most are malignant. These lesions can be anatomically and radiologically classified by means of compartments; anterior, middle, and posterior. Symptoms, signs, localization of the tumor, age of the child, and tumor markers are key points of diagnosis. Surgical approaches are typically needed for diagnosis, but sometimes tru-cut needle biopsies may be sufficient. Mediastinoscopy, mediastinotomy, and video-assisted thoracoscopic surgery may be used in the diagnostic workup of mediastinal tumors in children as they are used in adults. Frequently, diagnosis and treatment are both established by means of surgery. Surgery remains the mainstay of treatment of most benign and malignant nonlymphoid tumors. Combined modality of treatment incorporating chemotherapy and radiotherapy is often required in malignant tumors and is associated with high survival rates in these patients.

Clinical and Radiological Evaluation and Follow-Up of Patients with Noncardiac Plastic Bronchitis.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease characterized by obstruction of the airway by fibrinous mucus plugs. The etiology can be idiopathic or secondary to systematic diseases such as congenital heart diseases. Definitive diagnosis is made by pathological examination of the sputum or bronchial sample taken by bronchoscopy. In this study, the clinical status and treatment status of patients with PB were evaluated. MATERIALS AND METHODS: Medical records of the patients diagnosed as PB were reviewed ret- rospectively. Age, gender, clinical symptoms, radiology, bronchoscopic findings, and pathology results were documented. RESULTS: Six patients with PB were included in this study (female:male, 2:4). The median age of the diagnosis was 45 months. The most common symptoms are persistent wet cough and short- ness of breath. The duration of symptoms ranged from 30 to 90 days. Atelectasis was the most common radiological finding. Diagnosis was made with pathological examination of the mucus in all patients. All of the patients were treated with bronchoscopic removal of the mucus, and 4 patients required oral prednisolone therapy. Symptoms and radiological findings resolved completely in all patients. CONCLUSION: Although PB is a rare disease, it should be kept in mind in relation to patients with persistent radiological and clinical respiratory symptoms.

Cancer-Related Fatigue and Daily Living Activities in Pediatric Cancer Survivors.

Cancer-related long-term complications such as cardiovascular disease, fatigue, weight-related problems, and emotional disturbances are found to be increased in pediatric cancer survivors (PCSs). The relationship between daily living activities (DLAs) and such complications is still being investigated. Our aim in this study was to investigate the relationship between the cancer-related fatigue and DLA in PCS. Cancer-related fatigue was assessed by "PedsQL Multidimensional Fatigue Scale (MFS)" and "visual analog scale (VAS)." The DLA was evaluated by WeeFIM. There were 77 PCS (44 boys) with a mean age of 10.76 years. The mean value of fatigue scales were VAS 2.48, and MFS total score 71.14. WeeFIM total result was 118.64. While there was a statistically significant relationship between VAS fatigue score and WeeFIM total score ( r=- 0.387; P =0.007), there was no such correlation between WeeFIM total score with MFS total score ( r =0.250; P =0.080). It is established that there is a relationship between level of fatigue and DLA. Risk factors like tumor type or treatment modalities for fatigue should be investigated in larger samples of specific survivor groups. Fatigue and its association with DLA should be screened clinically as a routine surveillance in PCS and treatment options be planned.

Effect of virtual reality-based exercise intervention on sleep quality in children with acute lymphoblastic leukemia and healthy siblings: A randomized controlled trial.

OBJECTIVE: Sleep is one of the important measurements of the quality of life for children especially suffering from a chronic illness such as cancer. Our aim was to determine the changes in sleep quality and to investigate the effect of virtual reality-based exercise (VRBE) approaches on sleep in patients with acute lymphoblastic leukemia (ALL) off treatment. METHOD: The participants (ALL and healthy siblings) were evaluated for sleep quality with polysomnography and "Children's Sleep Habit Questionnaire" before and after 12 weeks. The study randomized into two groups: an exercise group who received VRBE in two days in a week, 45 min of each session for 12 weeks and an control group who were managed with supportive measures. The VRBE comprised of aerobic exercise in four different games by Nintendo Wii Fit Plus®. RESULTS: This randomized controlled trial was carried out on 38 participants. Before intervention, ALL patients (n = 24) and healthy siblings (n = 14) had similar sleep quality in terms of polysomnography and Children's Sleep Habit Questionnaire findings. After intervention, total time asleep (p = 0.023), respiratory disturbance index of hypopnea (p = 0.005), apnea/hypopnea index (p = 0.008), and number of apnea (p = 0.028) statistically significant improved. SIGNIFICANCE OF RESULTS: Patients with ALL off treatment had similar values of sleep quality with healthy siblings. Novel types of exercises like VRBE have positive effects on sleep disorders in children with ALL and also healthy siblings. Future studies are needed comparing the different types of interventions.

Evaluation of diagnostic components and management of childhood pulmonary tuberculosis: a prospective study from Istanbul, Turkey.

INTRODUCTION: The diagnosis of childhood tuberculosis is difficult and most of the patients are diagnosed clinically. The objective of this study is to reveal the diagnostic and therapeutic components of childhood pulmonary tuberculosis and to analyze the changes that occurred in our country over the years. METHODOLOGY: All patients diagnosed with tuberculosis between 2006 and 2016 were included. Demographic characteristics, diagnostic and treatment outcomes were recorded and patients were followed up prospectively. RESULTS: A total of 492 patients were included in the study. 97% had Bacillus Calmette-Guerin vaccine, 36% were diagnosed with microbiologically-confirmed tuberculosis and 64% were diagnosed with clinically-proven tuberculosis. 94% of the patients had symptoms consistent with tuberculosis, all patients had radiologic findings, 74% had a history of tuberculosis contact and 63% had tuberculin skin test positivity. The diagnoses included primary tuberculosis in 62%, secondary tuberculosis in 21%, progressive primary tuberculosis in 13% and miliary tuberculosis in 4%. 48% of the patients received a treatment regimen containing three drugs as the initial treatment, and drug-related side effects developed in 12%. Isoniazid resistance was detected in 13% of the patients and rifampicin resistance was detected in 8%. None of the patients died due to tuberculosis. In the last 50 years in Turkey, the rates of Bacillus Calmette-Guerin vaccination and diagnosis of tuberculosis cases have increased and the mortality rates have decreased over the years. CONCLUSIONS: Our study is one of the few prospective studies and revealed the differences between the recent data and the past 50 years in childhood tuberculosis in Turkey.

Post-infectious bronchiolitis obliterans in children: Clinical and radiological evaluation and long-term results.

BACKGROUND AND OBJECTIVE: This study aims to evaluate clinical and radiological findings and treatment outcomes of the patients with PIBO. METHODS: One hundred fourteen children were enrolled. Initial demographic and clinical findings were evaluated. Pre- and post-treatment clinical and radiological findings were compared. RESULTS: The median age of the patients at initial pulmonary injury was 7,2 months, the median age at diagnosis was 17.5 months. Persistent wheezing was the most common complaint. Thirty-five patients had mechanical ventilation history. 82,5% of patients had clinical improvement. Bronchiectasis, atelectasis, hyperinflation and air trapping in HRCT improved significantly with treatment. Post-treatment Bhalla scores decreased from 8.3 to 6.5 (p= 0,001). Improvement was observed in radiological and clinical findings after treatment. CONCLUSIONS: This study is one of the largest studies in the literature and one of the few studies that evaluate clinical and radiological outcomes of patients with PIBO.

Relationships Between Bronchoscopy, Microbiology, and Radiology in Noncystic Fibrosis Bronchiectasis.

Background: Published data on the correlations of bronchoscopy findings with microbiological, radiological, and pulmonary function test results in children with noncystic fibrosis (CF) bronchiectasis (BE) are unavailable. The aims of this study were to evaluate relationships between Bronchoscopic appearance and secretion scoring, microbiological growth, radiological severity level, and pulmonary function tests in patients with non-CF BE. Methods: Children with non-CF BE were identified and collected over a 6-year period. Their medical charts and radiologic and bronchoscopic notes were retrospectively reviewed. Results: The study population consisted of 54 female and 49 male patients with a mean age of 11.7 ± 3.4 years. In the classification according to the bronchoscopic secretion score, Grade I was found in 2, Grade II in 4, Grade III in 9, Grade IV in 17, Grade V in 25, and Grade VI in 46 patients. When evaluated according to the Bhalla scoring system, 45 patients had mild BE, 37 had moderate BE, and 21 had severe BE. Microbial growth was detected in bronchoalveolar lavage fluid from 50 of the patients. Forced expiratory volume in 1 s (FEV1) and functional vital capacity decreased with increasing bronchoscopic secretion grade (P = 0.048 and P = 0.04), respectively. The degree of radiological severity increased in parallel with the bronchoscopic secretion score (P = 0.007). However, no relationship was detected between microbiological growth rate and radiological findings (P = 0.403). Conclusions: This study showed that bronchoscopic evaluation and especially scoring of secretions correlate with severe clinical condition, decrease in pulmonary function test, worsening in radiology scores, and increase in microbiological bacterial load in patients. Flexible endoscopic bronchoscopy should be kept in mind in the initial evaluation of non-CF BE patients.

Palliative Care in High and Low Resource Countries.

Palliative Care (PC) is defined by the World Health Organization (WHO) as a support provided by multiple disciplines in order to improve the quality of life of both patients and their caregivers, throughout the disease course, from diagnosis to end-of-life. PC aims to prevent and treat symptoms and side effects of the disease and its treatment. PC is well developed in most high- -income countries; however in most low-income settings, where approximately 80% of patients with cancer requiring PC care for advanced disease live, PC services are still uncommon. Health indicators monitoring global PC development are policy, education, use of medicines, service provision and professional activity. Globally, PC development may be categorized as Group 1 (no known hospice-PC activity), Group 2 (capacity-building activity), Groups 3a Isolated PC provided, 3b Generalized PC provided, 4a hospice-PC services at a stage of integration into regular service provision, and 4b hospice-PC services at a stage of advanced integration into regular service provision. Spirituality is an essential element of patient-centered PC. The use of Complementary and Traditional Medicine (CTM) in Middle Eastern countries is widespread. There are wide discrepancies in cancer care and PC in many regions of the world. The Individualized Care Planning and Coordination (ICPC) Model is designed to facilitate the advance care planning with continuity of all the measures like symptom control or emotional, social and spiritual care of both the patient and the family during the disease steps like relapse or end of life.

Association of Myeloperoxidase Gene Polymorphism With Iron Deficiency Anemia in Turkish Children.

This study was performed to investigate the gene polymorphisms of the myeloperoxidase (MPO) enzyme and to determine whether MPO gene polymorphisms influence the response to iron therapy in pediatric patients with iron deficiency anemia (IDA). In this case-control study, 50 Turkish children with IDA and 50 healthy controls were enrolled. Three MPO gene alleles were selected for genotyping in the study: GG, AG, and AA. The relationships of alleles with IDA were analyzed and compared in patients and controls. Pretreatment and posttreatment laboratory parameters and gene polymorphisms were compared in the patient group. There was a significant difference between patients with IDA and controls regarding genotype frequencies of the AA, GG, and AG alleles (P=0.005). However, the AG allele was found to be associated with variations in hemoglobin, red blood cell, hematocrit, mean corpuscular volumes, and mean corpuscular Hb concentrations levels. The frequency of AA, GG, and AG alleles of the MPO gene was potentially associated with changes in iron metabolism and the AG allele led to variations in various hemogram parameters.

Persistent pleural effusion in an infant with an unusual diagnosis: congenital alveolar rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is a malignant form of neoplasm that originates from skeletal muscle. RMSs can exist anywhere in the human body but are more commonly detected in the neck region and extremities. The alveolar type is one of the subtypes of RMS that has a poor prognosis. Because the clinical manifestation of a tumour can be a painless mass, symptoms might be non-contributary to the diagnosis. Herein, a four-month-old girl was admitted to the emergency department with complaints of respiratory distress without a runny nose, cough, and fever. Recurrent effusions subsided with subsequent tube thoracostomy. Video-assisted thoracoscopic surgery (VATS) was performed to determine the aetiology of the recurrent effusion. The Tru-Cut biopsy obtained during VATS resulted in the diagnosis of alveolar rhabdomyosarcoma. Pleural effusion decreased, and the tube drainage was stopped rapidly after first vincristine, actinomycin-D, and cyclophosphamide chemotherapy cycle. Persistent and recurrent pleural effusions should alert physicians to rule out unusual diagnoses like that of our case.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey. Material and methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires. Results: Only 1 of the 21 participating centers had a separate LTFU clinic. The remaining centers provided LTFU care for childhood cancer survivors at the pediatric oncology outpatient clinic. Of these centers, 17 (80.9%) reported difficulty in transition from the pediatric clinic to the adult clinic, 14 (66.6%) reported insufficient care providers, and 12 (57.1%) reported insufficient time and transportation problems. As neglected late effects, 16 (76.1%) centers reported psychosocial and getty job problems and 11 (52.3%) reported sexual and cognitive problems. None of the centers had their own LTFU guidelines for their daily LTFU practice Conclusion: This study was the first to gain an overview of the needs of POCs and the gaps in survivorship services in Turkey. The results from this study will help to develop a national health care system and national guidelines for pediatric cancer survivors.

Post-intubation subglottic stenosis in children: Analysis of clinical features and risk factors.

BACKGROUND: Subglottic stenosis (SGS) is a complication that develops after intubation and is characterized by respiratory distress. The aim was to evaluate patients with post-intubation SGS and to discover the factors contributing to its development. METHODS: A total of 112 patients who had a history of intubation were included. The case group consisted of 50 patients with post-extubation persistent respiratory symptoms for which flexible bronchoscopy (FOB) was conducted and showed SGS. The control group consisted of 62 patient with no post-extubation persistent respiratory symptoms, for whom FOB was not done (n = 54), and who had post-extubation persistent respiratory symptoms and underwent FOB, which did not show subglottic stenosis (n = 8). RESULTS: No significant differences were detected related to age, gender, and gestational age. The median number of recurrent intubations was 2.5 and 3 in the case group and in control group, respectively (P = 0.14). The median duration of intubation was 20.5 days in the case group, and 6 days in the control group (P < 0.001). The Myer-Cotton classification indicated a degree of obstruction of grade 1 (mild) in 30% (n = 15), grade 2 in 16% (n = 8), grade 3 in 48% (n = 24), and grade 4 in 6% (n = 3) of the case group. CONCLUSION: The duration of intubation was found to be a significant risk factor for SGS development. Age at intubation, gender, gestational age, indication of intubation, and the number of recurrent intubations were found to have no significant association. Patients with post-extubation persistent respiratory problems, especially those with prolonged intubations, should be evaluated for SGS.

Testis Involvement in Neuroblastoma: Report of 3 Cases in the Turkish Pediatric Oncology Group-Neuroblastoma Study and Review of the Literature.

BACKGROUND: Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Primary and secondary testicular involvement is extremely uncommon in neuroblastoma. PROCEDURE: All children with neuroblastoma treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma (NB) Study and who had testis involvement either at diagnosis or at relapse were retrospectively evaluated. A review of all cases with neuroblastoma and testis involvement in the literature was done. RESULTS: There were 3 children with NB documented to have involvement of the testis, 2 at diagnosis, 1 at recurrence, within the 559 cases (0.5%) treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma Protocol. All had advanced stage. Two were infants. A total of 57 cases of testicular or paratesticular neuroblastoma have been reported in children, and most cases represent metastases as in the 3 cases in our series. CONCLUSIONS: Neuroblastoma should be considered in the differential diagnosis of testicular mass and work-up for neuroblastoma should be done before orchiectomy. Scrotal ultrasonography should be used as the first diagnostic tool and abdominal ultrasonography shall be done additionally. Testis examination should be performed at diagnosis and regularly during follow-up for boys diagnosed with neuroblastoma. Testes may be sanctuary sites when neuroblastoma is metastatic, as is the case in leukemia.

Nimotuzumab-containing regimen for pediatric diffuse intrinsic pontine gliomas: a retrospective multicenter study and review of the literature.

PURPOSE: Nimotuzumab is an IgG1 antibody that targets epidermal growth factor receptor (EGFR). Overexpression of EGFR is detected in some pediatric brain tumors including diffuse intrinsic pontine gliomas (DIPG)s. METHODS: Since May 2010, nimotuzumab, combined with carboplatin or vinorelbine or Temozolomide (TMZ), was administered during progressive disease (PD) after the use of the institutional protocol consisting of radiotherapy (RT) + TMZ and adjuvant TMZ. After May 2012, children with newly diagnosed disease received TMZ during RT, and nimotuzumab and TMZ after RT. Nimotuzumab was given as 150 mg/m2/dose once a week for 12 weeks, and then every other week with TMZ until PD. PD patients were switched to nimotuzumab + vinorelbine combination until death. RESULTS: Nimotuzumab was used in 24 children with DIPG (seven in the PD group, 17 in the newly diagnosed patient group). In the PD group, median survival time was 12 months (7-42 months); 1-year and 2-year overall survival (OS) rates were 42.9 ± 18% and 14.3 ± 13%, respectively. The median survival in this group, after the initiation of nimotuzumab was 6 months (3-8 months). In the newly diagnosed patient group, median survival time was 11 months (3-35 months) and median progression free survival was 4 months (1-21 months). The 1-year OS in this group was 35.3 ± 11% and 2 year OS was 11.8 ± 7%. Nimotuzumab ± chemotherapy was well tolerated with no major adverse effect. CONCLUSION: Nimotuzumab-containing regimens are feasible and tolerable; it might be that some patients either with newly diagnosed DIPG or with progressive disease may benefit modestly from nimotuzumab-containing combinations.