Background: Necrotizing pneumonia rarely occurs in children, but when it does it can be complicated by bronchopleural fistula, empyema, pneumothorax, sepsis, and acute respiratory distress syndrome (ARDS). Antimicrobial therapy is the cornerstone of its management; however, surgery is necessary in some cases. Ideally, surgical interventions are kept to a minimum, but this is not always possible if there is a mass effect from air and fluid in the pleural space, pulmonary necrosis leading to massive hemoptysis, uncontrolled sepsis, or difficulties with assisted ventilation. Case Presentation: Herein we present a patient with refractory pyopneumothorax and ARDS due to pneumococcal necrotizing pneumonia complicated by a bronchopleural fistula. The patient's clinical condition deteriorated despite antibiotics, surgical drainage, and assisted ventilation. Owing to pneumothorax with a high percentage of air leakage, bilateral diffuse collapse of the lungs, and insufficient oxygenation, surgical treatment was considered, but because of the patient's lack of tolerance for surgery due to hemodynamic reasons and the complications associated with surgery, medical treatment was determined to be more appropriate. Surfactant treatment was administered to the patient, resulting in significant clinical improvement. Conclusion: To the best of our knowledge, this is the first report of the use of surfactant to treat ARDS due to necrotizing pneumonia. Based on the presented case, we think surfactant can be considered as a salvage treatment for such patients.
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.
Background: Mutations in the ATP-binding cassette transporter A3 (ABCA3) gene are one of the most common surfactant disorders leading to interstitial lung diseases (ILD). The clinical spectrum and severity of lung disease caused by ABCA3 deficiency due to missense variants is variable. Case Presentations: A novel ABCA3 c.3135G>C (p.Gln1045His) mutation was identified at the homozygous state in 3 subjects from 2 unrelated families: one 19-month-old boy with severe ILD and his homozygous pauci-symptomatic mother, and one 10-year-old girl with moderate late-onset ILD. Corticosteroid pulses associated with hydroxychloroquine were beneficial for both children. Conclusion: We illustrate here the huge intra- and interfamilial phenotypic variability associated with the same homozygous missense ABCA3 mutation, and the benefit of identifying the disease for treatment, follow-up, and appropriate genetic counseling.
Introduction: Sideroblastic anemia with B cell immunodeficiency, periodic fevers, and developmental delay (SIFD) syndrome is caused by biallelic TRNT1 mutations. TRNT1 gene encodes a CCA-adding tRNA nucleotidyl transferase enzyme. Mutant TRNT1 results in immunodeficiency and anemia in various degrees, accompanied by several organ involvement. Case Presentation: We present here a 15-month old male, demonstrated brittle hair, growth hormone deficiency, recurrent fever, arthritis, recurrent infections, mild anemia, and hypogammaglobulinemia. The patient did not respond to colchicine treatment, and after establishing SIFD diagnosis with the presence of homozygote c.948-949delAAinsGG (p.Lys317Glu) mutation in TRNT1 gene, we commenced monthly intravenous immunoglobulin replacement and weekly subcutaneous etanercept. A rapid resolution of fever episodes and infections occurred after initiation of this treatment regimen. Afterward, both anemia and growth parameters have improved during follow-up. Conclusion: SIFD syndrome should be considered in patients with recurrent fever, arthritis, and growth retardation even in the absence of severe anemia and prominent hypogammaglobulinemia.
Background: Children with respiratory technologies, particularly those with mechanical ventilation, represent a growing population that require complex home nursing, medical equipment, outpatient medical and habilitative supports to live and thrive in their community. Care coordination is essential to support these children and their families to navigate and integrate key community-based health and educational services, however, care is often fragmented and care coordination needs unmet. Therefore, to fully support children with respiratory technologies, it is critical to understand the role of care coordinators (CCs) and how to sustain this workforce. The aim of this article is to describe CCs' perspective on (1) their role in supporting families in a home care program for children with respiratory technologies and home nursing, and (2) the core components of recruiting into and sustaining the CC workforce. Methods: Semistructured interviews were conducted with 15 CC from the Division of Specialized Care for Children (DSCC) Home Care program for children with technology dependence and home nursing in Illinois. Two independent coders utilized a modified template approach and discussed to agreement to analyze transcripts. Results: CC averaged 6.6 years of CC experience; the majority had social work or nursing backgrounds. CCs' job satisfaction was derived from their role supporting hospital discharge, seeing children improve over time, and navigating challenges with families. CCs enjoyed working in a collaborative environment where they could draw from their colleagues' experience to solve problems. Job dissatisfaction and job turnover stemmed from difficult family interactions, high caseloads, and redundant and time-intensive administrative tasks, which interfered with family engagement. Conclusions: CCs for children with respiratory technologies require diverse skills, but interdisciplinary teams enable collaborative support of families. Seeing children thrive can sustain the workforce, however, CCs report challenges due to high caseloads and administrative tasks, which impede direct family involvement.
Background: Drug provocation tests (DPTs) are the gold standard for the diagnosis of drug hypersensitivity reaction (DHR). To the best of our knowledge, there is no previous study reporting DPT-related anxiety levels in children and their parents. This study aimed to determine the difference in pre- and post-DPT anxiety levels of parents and children who were informed of the possibility of another DHR during the DPT, and to evaluate the relationship between parental psychological distress and anxiety levels. Methods: The study included children who underwent DPT in our clinic between July 1, 2019, and February 29, 2020, and accompanying parents who consented to participate. Age-appropriate State-Trait Anxiety Inventory scales were used to assess levels of state and trait anxiety in the patients and parents. The Symptom Checklist-90-Revised (SCL-90-R) was used to screen for psychological symptoms in parents. Results: Data were collected from the parents of 69 children who underwent DPTs. The patients' median age was 7.28 (interquartile range: 4.52-10.06) and their parents' mean age was 35.28 ± 5.38 years. Anxiety-related data were collected from 21 pediatric patients. The children and parents had higher state anxiety scores before DPT compared to after DPT. There was a positive correlation between the parents' trait anxiety and pre-DPT state anxiety scores. In addition, parental pre-DPT state anxiety scores were positively correlated with SCL-90-R general severity index, somatization, anxiety, obsessive-compulsive, and depression subscale scores. Conclusion: The risk of allergic reaction in DPT may cause anxiety. A high level of parental anxiety before DPT, which gradually decreased after negative test results, was associated with history of drug-induced anaphylaxis in their children and high trait anxiety. Appropriate evaluation of patients and parents before DPT and providing detailed information may be important to reduce this anxiety.
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.
Background and Objective: Although high-flow nasal cannula (HFNC) is widely used in children, there is no consensus on the methods for starting, maintenance, and weaning. The aim of this study was to compare weaning methods in children. Methods: The study included all patients in pediatric intensive care unit (PICU) who were started on HFNC treatment. The respiratory assessment score was used in the decisions for starting, continuing, and weaning from HFNC. The patients who responded and for whom weaning was planned were randomized by month into 2 groups as directly weaned from HFNC and weaned by reducing the flow. Success rates, treatment, and length of stay (LOS) in weaning methods were compared. Results: Of the 145 patients initially included in the study, 32 (22%) were excluded, and analysis was made of 113 patients. Successful weaning from HFNC was obtained in 76.9% of the patients, in 82.1% of flow weaning, and 73.6% of direct weaning, with no statistically significant difference determined between the groups (P = 0.286). The median duration of HFNC and the median LOS in PICU were determined to be statistically significantly shorter in direct weaning than in flow weaning [36 h interquartile range (IQR) 24-48 h] versus 60 h (IQR 60-72 h), P < 0.001 and 6 days (4-14 days) versus 9.5 days (5.25-20.75 days, P = 0.043, respectively). Conclusion: In patients who responded to HFNC in PICU, the responses to direct weaning and flow reduction were seen to be similar. In patients directly weaned off, both the HFNC duration and LOS in PICU were significantly shorter.
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