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.

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.

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.

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.

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.

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.

Effects of Malnutrition on Neutrophil/Mononuclear Cell Apoptotic Functions in Children with Acute Lymphoblastic Leukemia.

BACKGROUND: Recent studies claim that apoptosis may explain immune dysfunction observed in malnutrition. OBJECTIVE: The objective of this study was to determine the effect of malnutrition on apoptotic functions of phagocytic cells in acute lymphoblastic leukemia (ALL). MATERIALS AND METHODS: Twenty-eight ALL patients (13 with malnutrition) and thirty controls were enrolled. Neutrophil and mononuclear cell apoptosis of ALL patients and the control group were studied on admission before chemotherapy and repeated at a minimum of three months after induction of chemotherapy or when the nutritional status of leukemic children improved. RESULTS: The apoptotic functions of both ALL groups on admission were significantly lower than those of the control group. The apoptotic functions were lower in ALL patients with malnutrition than those in ALL patients without malnutrition, but this was not statistically significant. The repeated apoptotic functions of both ALL groups were increased to similar values with the control group. This increase was found to be statistically significant. CONCLUSIONS: The apoptotic functions in ALL patients were not found to be affected by malnutrition. However, after dietary intervention, increased apoptotic functions in both ALL patient groups deserve mentioning. Dietary intervention should always be recommended as malnutrition or cachexia leads to multiple complications. Enhanced apoptosis might originate also from remission state of cancer.

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.

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 diffuse intrinsic pontine glioma patients from a single center.

BACKGROUND: The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. This study aims to evaluate the characteristics and treatment outcome of children with DIPG in a single center. METHODS: We reviewed the outcome of children with DIPG treated at the Oncology Institute of Istanbul University from February 1999 to May 2012. RESULTS: Fifty children (26 female, 24 male) with the median age of 7 years were analyzed. The median duration of symptoms was 30 days. All patients received radiotherapy (RT). Before the year 2000, 12 patients received only RT. Thirty-eight had concomitant and/or adjuvant chemotherapy with RT. Between 2000 and 2004, 17 patients received cis-platinum or vincristine as sensitizers during RT and CCNU + vincristine combination after RT. Since 2004, 21 patients received temozolomide (TMZ) concomitantly during RT and as adjuvant chemotherapy after RT. The median survival time of all patients was 13 months (1-160 months). Patients receiving RT + TMZ had a significantly higher overall survival than patients with only RT (p = 0.018). Patients receiving RT + chemotherapy other than TMZ also had a significantly higher overall survival than patients receiving only RT (p = 0.013). Patients receiving RT + TMZ + and chemotherapy other than TMZ had a significantly higher survival than patients receiving only RT (p = 0.005). CONCLUSION: In our series, patients receiving RT + TMZ and also patients receiving RT + chemotherapy other than TMZ had a significantly higher overall survival than patients treated with only RT. Hence, administering chemotherapy during and after RT seems to prolong survival in some DIPG patients.

A modified protocol with vincristine, topotecan, and cyclophosphamide for recurrent/progressive ewing sarcoma family tumors.

PURPOSE: Topotecan has recently been used in the treatment of pediatric cancer. We evaluated our experience with the modified combination of vincristine, topotecan, and cyclophosphamide (VTC) given in 3 days, in children with recurrent Ewing sarcoma. METHOD: Children received vincristine (1.5 mg/m(2)/1st day), cyclophosphamide (600 mg/m(2)/day × 2 days) + mesna, and topotecan (1 mg/m(2)/day × 3 days) every 21 days. RESULT: A total of 118 courses of VTC were given to 13 patients. One patient received VTC both at first and at second relapse. Thus, 14 relapse episodes in 13 patients were evaluated. After three courses of VTC chemotherapy (CT), two achieved complete response (CR), five achieved partial response, thus an objective response was attained in 7/14 (50%) episodes. Two patients had stable disease and two patients progressed. In three episodes, CR was achieved by surgery before CT. One of them had a second relapse and attained CR with VTC. Median time from diagnosis to relapse was 23 months (5-45 months). Site of relapse was local in four patients, and metastatic in 10 episodes of nine patients. Seven patients are alive, three with no evidence of disease and four alive with disease; six have died of disease. Local treatment was used in 11 episodes. The toxicity of the VTC combination was limited mainly to the hematopoietic system. CONCLUSION: In conclusion, the modified VTC protocol in 3 days every 3 weeks seems to be effective and tolerable in children and adolescents with recurrent/progressive Ewing sarcoma.

False positivity of magnetic resonance imaging under the effect of granulocyte-colony stimulating factor in a child with leukemia.

Granulocyte-colony stimulating factor (G-CSF) increases the proliferation and maturation of committed polymorphonuclear leukocyte precursors, as well as the function of mature polymorphonuclear leukocytes. It has previously been shown in pediatric patients that G-CSF induces reconversion of fatty bone marrow to hematopoietic bone marrow in the pelvis and lower extremities that is detectable by magnetic resonance imaging (MRI). Here, we report a 13-year-old Burkitt leukemia patient with bone pain while he was in remission. He was on G-CSF after cessation of high-dose and low-dose cytarabine chemotherapy. He was suspected to have a leukemia relapse. Pelvic MRI was consistent with leukemic infiltration. However, the pathology of bone marrow biopsy resulted in normal findings. Thus it was suggested that concurrent administration of G-CSF could be the causative agent for both bone pain and false-positive MRI findings. The control MRI after interruption of G-CSF revealed normal findings. In conclusion, radiologists should be informed about the type of therapy, including G-CSF administration, in order to overcome misinterpretation of bone marrow MRI.

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.