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2.
J Infect Dev Ctries ; 18(1): 116-121, 2024 01 31.
Article in English | MEDLINE | ID: mdl-38377098

ABSTRACT

INTRODUCTION: Biologic modifying agents are associated with an increased risk for infection with mycobacteria. The aim of this study is to document patients who received different biologic modifying therapies in our pediatric rheumatology department and the possibility of development of tuberculosis (TB). METHODOLOGY: This retrospective study was conducted in Ankara City Hospital. Pediatric patients who were treated with biologic modifying agents between 2010-2020 were documented. Development of TB and the risk factors were assessed in this patient group. RESULTS: There were 72 patients who were treated with different biologic modifying agents. Tuberculin skin test (TST) was positive in 7 (9.7%) patients during follow up. Three patients whose TST was positive had received canakinumab, 2 received etanercept, 1 received adalimumab and 1 received anakinra. Median duration of therapy was 43.5 (16.5-168) months for these patients and the duration was longer than patients who did not develop latent tuberculosis (p = 0.04). Patients who developed latent TB under treatment were significantly older than the patients who did not (p = 0.01). CONCLUSIONS: According to our findings, 9.7% of pediatric patients who received biologic modifying agent therapy developed latent TB. Patients who developed latent TB were older, and the duration of treatment was longer than patients who did not develop latent TB. Although not statistically significant, canakinumab, which is known as an agent less likely to cause TST conversion, was in fact the most common agent that caused TST conversion.


Subject(s)
Biological Products , Latent Tuberculosis , Tuberculosis , Humans , Child , Latent Tuberculosis/diagnosis , Latent Tuberculosis/drug therapy , Latent Tuberculosis/epidemiology , Retrospective Studies , Interferon-gamma Release Tests , Adalimumab , Tuberculin Test , Tuberculosis/drug therapy , Biological Products/adverse effects
3.
Eur J Pediatr ; 183(1): 295-304, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37875631

ABSTRACT

The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data-entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3-11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (- 2.0 [- 3.36 to - 0.81]) than in group 2 (- 0.80 [- 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040).   Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.


Subject(s)
Lung Diseases, Interstitial , Lymphadenopathy , Child , Humans , Lung , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/epidemiology , Lung Diseases, Interstitial/therapy , Prospective Studies , Registries , Turkey/epidemiology , Infant , Child, Preschool
4.
Pediatr Pulmonol ; 58(9): 2505-2512, 2023 09.
Article in English | MEDLINE | ID: mdl-37278544

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.


Subject(s)
Cystic Fibrosis , Quinolones , Humans , Cystic Fibrosis/complications , Cystic Fibrosis/drug therapy , Cystic Fibrosis/genetics , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use , Retrospective Studies , Aminophenols/therapeutic use , Quinolones/therapeutic use , Mutation
5.
Turk J Pediatr ; 65(2): 257-268, 2023.
Article in English | MEDLINE | ID: mdl-37114691

ABSTRACT

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.


Subject(s)
Cystic Fibrosis , Lung Transplantation , Humans , Cystic Fibrosis/epidemiology , Cystic Fibrosis/surgery , Cystic Fibrosis/complications , Routinely Collected Health Data , Lung , Forced Expiratory Volume , Referral and Consultation
6.
Int J Rheum Dis ; 26(8): 1582-1585, 2023 Aug.
Article in English | MEDLINE | ID: mdl-36852875

ABSTRACT

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by muscle weakness and specific skin lesions, as well as non-muscular involvement such as interstitial lung disease (ILD), cardiac involvement and arthritis. Anti-melanoma differentiation-associated protein 5 (anti-MDA5)-positive JDM patients are typically characterized by the presence of skin ulcers and rapidly progressing ILD (RP-ILD). Although cardiac involvement is not an expected involvement in anti-MDA5-positive JDM cases, it is significant because it can be fatal. In this report, an anti-MDA5 myositis-specific autoantibody-positive JDM case referred with the diagnosis of psoriatic arthritis in whom cardiomyopathy and arrhythmia were detected in follow-up is presented. Since cardiac involvement is associated with mortality, it would be useful to follow up anti-MDA5 positive patients for cardiac involvement in addition to lung involvement. Tofacitinib is a promising treatment option in treatment-resistant JDM.


Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Melanoma , Humans , Dermatomyositis/complications , Dermatomyositis/diagnosis , Dermatomyositis/drug therapy , Prognosis , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/drug therapy , Lung Diseases, Interstitial/etiology , Autoantibodies
7.
Pediatr Int ; 65(1): e15421, 2023.
Article in English | MEDLINE | ID: mdl-36413116

ABSTRACT

BACKGROUND: Many possible underlying causes can be seen in children with unexplained peripheral eosinophilia (UPE) with suspected pulmonary involvement. Here, we aimed to demonstrate the clinical characteristics and diagnoses of children evaluated for UPE who underwent diagnostic bronchoscopy. METHODS: Thirty-one children who underwent diagnostic bronchoscopy for UPE between 2006-2018 were included. Demographic characteristics, bronchoscopy findings and final diagnosis, and treatments were evaluated. RESULTS: The median age at the time of diagnosis was 5 (0.25-17.5) years. The final diagnoses of 26 patients were as follows: immunodeficiency (n = 6); hyper-IgE syndromes (n = 4), DOCK8 deficiency + HES (idiopathic hypereosinophilic syndrome; n = 1), and severe combined immunodeficiency (n = 1), HES (n = 3), idiopathic chronic eosinophilic pneumonia (ICEP; n = 3), idiopathic pulmonary hemosiderosis (n = 1), B cell lymphoma (n = 1). In one child, an integrin α3 mutation + cytomegalovirus (CMV) pneumonia was detected. Congenital pneumonia was found in one patient, and parasitic infection in another. In two, eosinophilia was attributed to underlying asthma and atopy. In four, the underlying reasons could not be elucidated. Two children with HES and one with ICEP were lost to follow-up. There was no significant relationship between the peripheral eosinophil count at the time of diagnosis and the percentage of bronchoalveolar lavage eosinophils (BAL). Bronchoscopy contributed to the management of 14 (53.9%) patients. CONCLUSIONS: Bronchoscopy has potential diagnostic contribution in patients with UPE suspected of having pulmonary involvement. Numerous various underlying causes may be present in this patient group. There is no relation between peripheral eosinophil count and BAL eosinophil percentage.


Subject(s)
Lung Diseases , Pneumonia , Pulmonary Eosinophilia , Humans , Child , Child, Preschool , Adolescent , Bronchoscopy , Pulmonary Eosinophilia/diagnosis , Eosinophils , Lung Diseases/diagnosis , Bronchoalveolar Lavage Fluid , Guanine Nucleotide Exchange Factors
8.
Pediatr Int ; 64(1): e15386, 2022 Jan.
Article in English | MEDLINE | ID: mdl-36225107

ABSTRACT

BACKGROUND: Community-acquired pneumonia (CAP) in children continues to be one of the prominent causes of pediatric morbidity and mortality worldwide. By determining the risk factors associated with the development of complicated CAP (CCAP), new approaches for early diagnosis and effective treatment can be identified. METHODS: This retrospective cohort study enrolled patients with CAP and CCAP who visited the pediatric ward of the study hospital between January 1, 2017 and December 31, 2017. For patients with CCAP, data regarding medical procedures performed, surgical intervention, and hospitalization duration were collected. RESULTS: A total of 111 patients, 93 (83.7%) with CAP and 18 (16.3%) with CCAP, aged between 3 months and 18 years were hospitalized because of severe pneumonia. The mean age of the patients was 3.6 ± 1.2 years and 60 (54%) of them were female. The mean age of patients with CCAP was higher than that of patients with CAP (4.2 ± 3.3 vs. 2.8 ± 2.1 years respectively); however, the difference was not significant (p = 0.012). Patients with CCAP exhibited a significantly higher C-reactive protein level than those with CAP (10.06 ± 7.55 vs. 4.43 ± 3.37 g/L respectively; p = 0.007). Hypoxia upon admission was noted more commonly in the CCAP group than in the CAP group (p < 0.001). CONCLUSION: Findings related to hypoxia, respiratory distress, and pleural effusion on imaging are important distinguishing factors associated with the development of complications in patients hospitalized with CAP. Therefore, CCAP etiology, diagnosis, and treatment approaches should be established and protective measures adopted.


Subject(s)
Community-Acquired Infections , Pleural Effusion , Pneumonia , Child , Humans , Female , Infant , Child, Preschool , Male , Retrospective Studies , Pneumonia/diagnosis , Pneumonia/epidemiology , Pneumonia/complications , Risk Factors , Hospitalization , Pleural Effusion/etiology , Community-Acquired Infections/diagnosis , Community-Acquired Infections/epidemiology , Community-Acquired Infections/therapy
9.
Pediatr Allergy Immunol Pulmonol ; 35(3): 133-138, 2022 09.
Article in English | MEDLINE | ID: mdl-36121783

ABSTRACT

Background: Previously, lipopolysaccharide-responsive beige-like anchor (LRBA) deficiency was categorized as a subtype of common variable immune deficiency. Research shows that LRBA deficiency is caused by dysregulation of T cell activation and expansion; it is placed under the category of immune dysregulation with cytotoxic T lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency. Cohort studies have revealed a broad spectrum of clinical manifestations and variable phenotype expression, including immune dysregulation [enteropathy, autoimmune cytopenia, interstitial lung disease (ILD), etc.] on 1 hand and immune deficiency (hypogammaglobulinemia, recurrent infections, bronchiectasis, etc.) on the other hand. Chronic lung disease is frequently seen in LRBA deficiency and is associated with poor outcomes. Case Presentation: This case report evaluates a female who presented with recurrent pneumonia and bronchiectasis but did not respond to treatment; she was lastly diagnosed with ILD with detailed clinical, radiological, and pathological workup. Conclusions: The respiratory characteristics of patients with LRBA deficiency should be investigated, monitored, and treated from the time of its diagnosis. The awareness and involvement of pulmonologists to pulmonary morbidity of patients with LRBA deficiency in workup and clinical decision making are crucial.


Subject(s)
Bronchiectasis , Common Variable Immunodeficiency , Lung Diseases, Interstitial , Adaptor Proteins, Signal Transducing/genetics , Adaptor Proteins, Signal Transducing/metabolism , Bronchiectasis/diagnosis , CTLA-4 Antigen/metabolism , Female , Humans , Lipopolysaccharides , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/genetics
10.
Tuberk Toraks ; 70(3): 263-270, 2022 Sep.
Article in English | MEDLINE | ID: mdl-36164950

ABSTRACT

Introduction: To evaluate the quality of life and anxiety level of school-age children with chronic cough, and changes with treatment. Materials and Methods: Patients aged between 6-18 years with a chronic cough were included in this study. A control group was designed, and the scale scores were compared with each other. Result: The mean age of the 82 patients was 10.9 ± 3.8 years, 62 (75.6%) had at least one specific cough marker. Forty patients (48.8%) were diagnosed with asthma. At their first visit, the psychosocial health scores and the total scale scores (sum of physical and psychosocial total scores) were lower than the control group for both patients and parents. After the resolution of cough, their scores increased to the same level with the control group. It was also found that the level of anxiety was significantly higher than in the control group both before treatment and after the resolution period (p<0.001 and =0.008, respectively). Conclusions: Asthma was the leading cause of chronic cough. Quality of life is impaired in children with chronic cough. Anxiety level in these patients increases and after symptoms improve, continues to be higher than that of healthy children.


Subject(s)
Asthma , Cough , Adolescent , Anxiety/etiology , Asthma/complications , Child , Chronic Disease , Cough/diagnosis , Cough/etiology , Humans , Quality of Life
11.
Pediatr Pulmonol ; 57(10): 2344-2355, 2022 10.
Article in English | MEDLINE | ID: mdl-35676765

ABSTRACT

OBJECTIVE: Hypersensitivity pneumonitis (HP) is rare in the pediatric population. To date, there are no studies defining a correlation between clinical, radiological, and pathological findings in children with HP. The objective of this study is to define the clinical, and radiological characteristics and prognosis of childhood HP and to examine the clinical, radiological, and pathological correlation between HP stages. METHODS: Patients with suspected HP and followed at two tertiary care hospitals between 2000 and 2020 were retrospectively evaluated. Computed tomography (CT) of the chest of patients was evaluated by a single radiologist. The interagreement between clinical and radiological severity of the patients was calculated with the κ test. RESULTS: Fourteen children with suspected HP were identified. The results of 10 patients with the definitive diagnosis were as follows: one patient (10%) had acute, five patients (50%) had subacute, and four patients (40%) had chronic HP. The most prominent findings in chest CT were hilar, or hilar and subcarinal lymphadenopathy (80%), centrilobular nodules (60%), patchy or diffuse ground-glass opacities (50%), and cysts (50%). The interagreement between clinical and radiological severity of the patients was 100% (approximate significance: 0.003). The diagnosis of four patients with suspected HP who were unresponsive to standardized medical treatments or developed multisystem involvement was diagnosed with other diseases. One patient (10%) with definitive chronic HP died due to respiratory failure during follow up. CONCLUSION: Similar to adult HP, the prognosis is worse in children with existing fibrotic equivalents in chest CT. Patients who are not responding to standard medical treatments or develop multisystem involvement should be evaluated for other lung diseases.


Subject(s)
Alveolitis, Extrinsic Allergic , Adult , Alveolitis, Extrinsic Allergic/diagnosis , Child , Fibrosis , Humans , Prognosis , Retrospective Studies , Tomography, X-Ray Computed
12.
Pediatr Allergy Immunol Pulmonol ; 35(2): 74-78, 2022 06.
Article in English | MEDLINE | ID: mdl-35588286

ABSTRACT

Background: Previous studies reported that the prevalence of drug allergy is higher in patients with cystic fibrosis (CF) than in the general population. It is important to exclude or confirm the drug allergy diagnosis with detailed allergic evaluation for preventing drug allergy overdiagnosis. Our study aims to determine the actual frequency of drug allergy proven by diagnostic tests in children with CF and to compare it with the control group. Methods: Patients diagnosed with CF who were followed up in the Pediatric Pulmonology Clinic were included in the study group. Children with similar gender and age characteristics who did not have any chronic diseases and who applied to the Pediatric Polyclinics were included in the control group. We reviewed the medical data of patients with CF. Also, we evaluated the parents of the patients via phone conversation and/or during the control of the outpatient clinic and questioned them in terms of drug allergy. In addition, we assessed those with suspected drug allergies in the pediatric allergy clinic for diagnostic tests and compared it to the control group. Results: CF patients (n = 44) and control group (n = 100) were included in the study. Only 1 patient (2.2%) out of the 44 patients in the study group had a suspicion of drug-related hypersensitivity history. In the control group, 1 patient had a history of rash, provocation test was performed to rule out drug hypersensitivity reaction, and it was evaluated as a negative result. Conclusions: The result of our study showed that the frequency of drug allergy in children diagnosed with CF was not different from the control group. However, it will be useful to confirm the data of pediatric patients with CF in larger groups. In the presence of suspicion of drug allergy, a diagnostic evaluation can prevent unnecessary drug allergy diagnoses.


Subject(s)
Cystic Fibrosis , Drug Hypersensitivity , Exanthema , Child , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Drug Hypersensitivity/diagnosis , Drug Hypersensitivity/epidemiology , Humans , Prevalence , Skin Tests
13.
J Pediatr Hematol Oncol ; 44(5): e881-e883, 2022 07 01.
Article in English | MEDLINE | ID: mdl-35383694

ABSTRACT

Hypereosinophilic syndrome (HES) is a very rare disease during childhood. It involves the different organs like skin, gastrointestinal system, heart and lungs, besides pulmonary hypertension (PHT) is a very rare morbidity of HES that may cause life-threatening complications. PHT improves with the treatment of hypereosinophilia, without the need for pulmonary vasodilator therapy. Here, we present a case of PHT developed after recovery of pulmonary infiltration in an infant with idiopathic HES. We revealed that pulmonary pressure returned to normal range in parallel with the decrease in eosinophil count with steroid treatment.


Subject(s)
Hypereosinophilic Syndrome , Hypertension, Pulmonary , Heart , Humans , Hypereosinophilic Syndrome/complications , Hypereosinophilic Syndrome/drug therapy , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/etiology , Infant , Leukocyte Count , Lung
14.
Pediatr Surg Int ; 38(4): 559-568, 2022 Apr.
Article in English | MEDLINE | ID: mdl-35212777

ABSTRACT

INTRODUCTION: Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by partial obstruction and occurs at a rate of 1/20,000-30,000 live births. Here, we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature. METHODS: We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18 years at our center between 2013 and 2020. In addition, we examined the symptoms and findings recorded during the patients' follow-up in this retrospective descriptive study. RESULTS: The median age of 20 patients with CLE at diagnosis was 3.2 years (range 1 day-17 years). Respiratory distress and mediastinal shift were more prominent in patients who underwent surgery, and they were diagnosed at an earlier age compared with patients who were followed up conservatively (p = 0.001, 0.049, 0.001, respectively). Neither the pulmonary lobe involvement nor the bronchoscopy findings were found to be indicative of surgery. DISCUSSION: We observed that respiratory distress and mediastinal shift were more prominent in patients with a diagnosis of CLE who underwent surgery compared with patients who were conservatively followed up. Moreover, we observed that those who underwent surgery were diagnosed with CLE at an earlier age. In line with the literature, the pulmonary symptoms and CLE-related imaging findings in our study were reduced during conservative follow-up.


Subject(s)
Conservative Treatment , Pulmonary Emphysema , Adolescent , Child , Child, Preschool , Follow-Up Studies , Humans , Infant , Infant, Newborn , Pulmonary Emphysema/congenital , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/surgery , Retrospective Studies
15.
Pediatr Pulmonol ; 57(5): 1245-1252, 2022 05.
Article in English | MEDLINE | ID: mdl-35102722

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. METHODS: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. RESULTS: There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). CONCLUSIONS: The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.


Subject(s)
Bartter Syndrome , Cystic Fibrosis , Bartter Syndrome/complications , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Cystic Fibrosis Transmembrane Conductance Regulator , Female , Humans , Infant, Newborn , Male , Patient Care , Registries , Turkey/epidemiology
16.
Pediatr Int ; 64(1): e14888, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34131975

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. METHODS: In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). RESULTS: In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). CONCLUSIONS: Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.


Subject(s)
Cystic Fibrosis , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/genetics , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Humans , Infant, Newborn , Neonatal Screening/methods , Staphylococcus aureus , Trypsinogen
17.
J Pediatr Hematol Oncol ; 44(2): e554-e556, 2022 Mar 01.
Article in English | MEDLINE | ID: mdl-33370001

ABSTRACT

Chylothorax is an unusual complication of childhood cancer. It causes to additional morbidity and mortality during management. It should be kept in mind that chylothorax may occur due to mass shrinkage during treatment in patients with mediastinal mass and ductus thoracicus invasion at the initial diagnosis and necessary measures should be taken. This case was presented because of the rarity in pediatric oncology practice.


Subject(s)
Ataxia Telangiectasia , Chylothorax , Lymphoma, T-Cell , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Ataxia Telangiectasia/complications , Child , Chylothorax/etiology , Humans , Induction Chemotherapy/adverse effects , Lymphoma, T-Cell/complications , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , T-Lymphocytes/pathology
18.
Int J Clin Pract ; 75(9): e14471, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34107136

ABSTRACT

OBJECTIVE: Although the initial reports of COVID-19 cases in children described that children were largely protected from severe manifestations, clusters of paediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease, toxic shock syndrome and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Our objective was to develop a protocol for the evaluation, treatment and follow-up of patients with MIS-C. METHODS: The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of paediatric critical care, cardiology, rheumatology, surgery, gastroenterology, haematology, immunology, infectious disease and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback. CONCLUSION: Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a paediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients' phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.


Subject(s)
COVID-19 , Algorithms , Child , Humans , SARS-CoV-2 , Syndrome , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/therapy
19.
Pediatr Pulmonol ; 56(6): 1626-1634, 2021 06.
Article in English | MEDLINE | ID: mdl-33559955

ABSTRACT

OBJECTIVE: Recurrent respiratory problems are common in patients with cerebral palsy (CP). Detection of risk factors could prevent respiratory problems and reduce permanent lung damage. We aimed to provide a multidisciplinary approach for the causes, frequency, and concomitant problems and solutions of respiratory problems of these patients. METHOD: Medical records of CP patients who were followed-up and treated due to respiratory problems requiring hospitalization were examined retrospectively, and factors affecting the frequency and duration of hospitalization in wards and in the intensive care unit (ICU) were evaluated. RESULTS: Among 292 hospitalizations of 83 CP patients, 91% of them were hospitalized for pneumonia, and only 15.4% of them had major aspiration-related pneumonia in the history. 97.4% of the hospitalized patients had swallowing dysfunction in detailed history, but videofluoroscopic swallowing studies could be performed only to 24 patients and aspiration had been demonstrated in 96%. Patients who had been hospitalized more than two times during the oral feeding period, before switching to artificial feeding (nasogastric tube, gastric tube +/- fundoplication), had higher total and ICU hospitalization frequency/duration than the patients who had been hospitalized twice or less (0.12 vs. 0.17/0.005106 vs. 0.005353). CONCLUSION: The most common pulmonary complication in patients with CP is pneumonia due to chronic aspiration. To prevent recurrent lung infections and consequently chronic respiratory failure, it is necessary to evaluate these patients in terms of feeding difficulties in the early period with appropriate methods and apply interventions before affecting the lung parenchyma.


Subject(s)
Cerebral Palsy , Deglutition Disorders , Pneumonia, Aspiration , Cerebral Palsy/complications , Cerebral Palsy/epidemiology , Deglutition , Deglutition Disorders/epidemiology , Deglutition Disorders/etiology , Hospitalization , Humans , Pneumonia, Aspiration/epidemiology , Pneumonia, Aspiration/etiology , Pneumonia, Aspiration/prevention & control , Retrospective Studies
20.
Turk Thorac J ; 21(6): 419-432, 2020 Nov.
Article in English | MEDLINE | ID: mdl-33352098

ABSTRACT

It has been more than 3 months now since the first case of COVID-19 was reported in Turkey. Globally, the number of confirmed cases and deaths reached 9,653,048 and 491,128 respectively, as reported by 216 countries by June 27, 2020. Turkey had 1,396 new cases, 194,511 total cases, and 5,065 deaths by the same date. From the first case until today, the Turkish Thoracic Society (TTS) has been very proactive in educating doctors, increasing public awareness, undertaking academic studies, and assisting with public health policies. In the present report, social, academic, and management perspectives of the pandemic are presented under appropriate subtitles. During this critical public health crisis, TTS has once again demonstrated its readiness and constructive stance by supporting public health, healthcare workers, and the environment. This review summarizes the perspective of TTS on each aspect of the COVID-19 pandemic and casts light on its contributions.

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