Childhood interstitial lung disease in Turkey: first data from the national registry.

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.

Psychosocial factors affecting the quality of life of parents who have children with home mechanical ventilation.

INTRODUCTION: Most children with medical complexity have to live with home mechanical ventilation (HMV). Undertaking the care of a child with HMV creates a psychosocial burden on parents. This study investigated the impact of selected potential determinants on the quality of life of parents who have children with HMV. METHODS: A cross-sectional survey study was conducted using a structured questionnaire to determine the sociodemographic characteristics of the parents. The World Health Organization Quality of Life Assessment-Brief version, the Beck Depression Inventory (BDI), and the Multidimensional Scale of Perceived Social Support were applied. RESULTS: A total of 35 participants responded to the questionnaires. Paired data from mothers and fathers were obtained from 12 families. A moderately significant positive correlation was found between the perceived social support levels of the parents and all domains of the quality of life scale (for the physical domain: r = .455, p = .006; for the psychological domain: r = .549, p = .001; for the social domain: r = .726, p = .000; and for the environment domain: r = .442, p = .008). A moderate negative relationship was found between parents' perceived social support levels and BDI scores (r = -.557, p = .001). The multivariate regression analysis determined that being a mother, quitting a job to become a caregiver, being the only caregiver at home, and having a neurological/neuromuscular disease as the primary disease of the child were associated with lower scores in more than one quality of life domain. CONCLUSION: Our results emphasize that appropriate social support is important for improving the quality of life scores of parents of children with HMV.

Caregiver education before hospital discharge for children on home-invasive mechanical ventilation.

Children on home invasive mechanical ventilation necessitate specialized equipment, continuous monitoring, and multidisciplinary care. Transitioning these children from hospital to home care is complex, demanding careful planning. Guidelines and observational studies emphasize the importance of a standardized, comprehensive, and staged educational approach for caregiver education in discharge planning, yet program variations persist, lacking standardized checklists. This review aims to offer insights into crucial factors for a successful transition from hospital to home care for children using home-invasive mechanical ventilation while also developing comprehensive caregiver checklists to ensure high-quality care, taking into account families' socioeconomic status.

Chronic Hepatitis B In Children: Case Series From A Tertiary Paediatric Hospital.

BACKGROUND: Chronic hepatitis B (CHB) remains an important global public health problem. Seroconversion is highly correlated with favourable long-term outcome. There is no consensus on the treatment method, onset time and duration for paediatric CHB patients. In this study, we aimed to evaluate the course of children with CHB, to determine the characteristics of the patients who developed spontaneous seroconversion and treatment indications, and to compare the effectiveness of different treatment modalities. PATIENTS AND METHODS: Patients aged 1-18 years with a diagnosis of CHB were included in this study and evaluated for the development of spontaneous hepatitis B envelope antigen (HBeAg)/surface antigen (HBsAg) seroconversion and treatment modalities. The treated patients were divided into two groups according to their treatment regimen. RESULTS: Of the 114 patients, the median age at diagnosis was 8.4 years and median follow-up period was 5.2 years. Spontaneous HBeAg and HBsAg seroconversion developed in 18 (20.6%) of 87 HBeAg positive patients and two (1.8%) patients, respectively. Thirty-one patients were treated. The follow-up period was higher in the group with HBeAg seroconversion (p:0.005). There was no statistical difference between the patients in terms of seroconversion development and treatment status. The serum alanine aminotransferase (ALT) values at the time of diagnosis were statistically higher in treated patients. CONCLUSION: HBV infection and CHB continue to be an important problem for children in our country. Consensus on the appropriate ALT ULN is needed for the treatment of hepatitis in children.

Diffuse alveolar haemorrhage in children: an international multicentre study.

Background: Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods: A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Results: Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Conclusions: Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

Risk factors for first nonscheduled hospital admissions of pediatric patients on home mechanical ventilation.

OBJECTIVES: The number of children on home mechanical ventilation (HMV) has increased. Understanding the reasons for nonscheduled hospital admissions during HMV is critical. This study aims to investigate the risk factors of first nonscheduled hospital admissions of pediatric patients on HMV. METHODS: A retrospective analysis of patients on HMV between May 1, 2014 and October 1, 2020 was performed. Patients' demographic characteristics, duration of the education of the primary caregiver; time of first nonscheduled visit; and type of HMV (noninvasive mechanical ventilation [NIV] or invasive mechanical ventilation [IMV]) were analyzed. The reasons for first nonscheduled hospital visits were categorized as respiratory problems and other reasons. RESULTS: Of 97 patients, 41 were female (42.3%), and 70 (72%) were on IMV. The median age was 23 months (IQR, 10-91). Twenty-nine patients (30%), were admitted to hospital before scheduled visit with a mean duration of 18.1 ± 11.6 days; of them, 14 (48.2%) admitted because of respiratory problems. IMV increases the risk of first nonscheduled visit compared to NIV (OR, 16.3; 95% CI, 2.1-127.4; p = .008). If a caregiver spends less than 14 days in hospital for education, risk of nonscheduled visits increases (OR, 4.0; 95% CI, 1.5-11.2; p = .007). CONCLUSION: A minimum 14 days seems to be necessary for education of the caregivers of the patients with HMV to reduce the number of nonscheduled visits, which is a burden for both patients and healthcare system.

A 2-Year-Old Boy With Hypoxemia, Pulmonary Hypertension, and Digital Clubbing.

CASE PRESENTATION: A 2-year-old boy was referred to the Ankara University School of Medicine Children's Hospital with a history of recurrent respiratory distress and cyanosis since birth. His medical history was significant for premature birth at 31 weeks via cesarean section, as an infant of a diabetic mother. There was no parental consanguinity. He was hospitalized in the neonatal ICU after birth because of respiratory distress. After receiving invasive mechanical ventilation for 4 days, noninvasive mechanical ventilation and oxygen therapy were given gradually. As a result of further investigations, he received a diagnosis of situs inversus totalis and pulmonary hypertension. He was discharged on postnatal day 53 without supplemental oxygen therapy or treatment for pulmonary hypertension. Up to the age of 2 years, the patient had a history of multiple admissions to hospital for respiratory distress, lower respiratory tract infection, and cyanosis as an inpatient and outpatient. After starting to walk, shortness of breath and coughing occurred with effort.