The Challenging Diagnosis of Interstitial Lung Disease in Children-One Case Report and Literature Review.

Childhood interstitial lung disease (chILD) includes a heterogeneous spectrum of rare respiratory disorders in children associated with substantial morbi-mortality. Interstitial tissue, and other pulmonary structures, epithelium, blood vessels, or pleura are involved, resulting in a restrictive lung disfunction. Respiratory symptoms set in progressively and are often subtle, making thorough clinical history and physical examination fundamental. The etiology often is obscure. The clinical presentation mimics pneumonia or asthma, leading to a diagnostic delay. Challenging diagnosis may require genetic tests, bronchoalveolar lavage, or lung biopsy. Alongside general supportive therapeutic measures, anti-inflammatory, immunosuppressive or antifibrotic agents may be used, based on data derived from adult studies. However, if accurate diagnosis and treatment are delayed, irreversible chronic respiratory failure may ensue, impacting prognosis. The most frequent chILD is hypersensitivity pneumonitis (HP), although it is rare in children. HP is associated with exposure to an environmental antigen, resulting in inflammation of the airways. Detailed antigen exposure history and identification of the inciting trigger are the cornerstones of diagnostic. This article provides the current state of chILD, revealing specific features of HP, based on a clinical case report of a patient admitted in our clinic, requiring extensive investigations for diagnosis, with a favorable long-term outcome.

The role of lung ultrasonography in predicting the clinical outcome of complicated community-acquired pneumonia in hospitalized children.

AIMS: This study's objective was to analyze lung ultrasonography (LUS) characteristics in hospitalized pediatric patients with complicated community-acquired pneumonia (CAP). We hypothesized that LUS could be correlated with the clinical outcome in these cases. MATERIALS AND METHODS: In this retrospective study, we evaluated the LUS appearances (at admission and five days after the beginning of the treatment) and the progression of complicated CAP. RESULTS: We identified 45 patients who fulfilled the inclusion criteria. Several complications occurred in these subjects during follow-up including: serofibrinous pleurisy (62.2%), empyema (15.6%), encapsulated pleurisy (11.1%), lung abscess (6.7%) and necrotizing pneumonia (2.2%). In addition, 22.2% of the patients required surgical treatment: draining tube (11.1%), decortication (6.7%) and resection (4.4%). Intensive care unit admission was needed in 8.9% of patients. The median duration of hospitalization was 14 [9.7; 19.7] days. The thickness of pleural effusion with a cut-off value of 10 mm seen by LUS was a predictor for the need for continuous thoracic drainage (p<0.01), segmentectomy or thoracoscopic surgery (p=0.03) and prolonged hospitalization over 10 days (p<0.01). Hyperechogenic pleural effusion, presence of septa and fluid bronchogram on 1st LUS evaluation were independent predictors of segmentectomy or thoracoscopic decortication (p<0.01) and of longer hospitalization (p=0.02, p<0.01, p<0.01 respectively). CONCLUSIONS: The ultrasound characteristics of complicated CAP can offer valuable information to predict the clinical evolution of CAP and so can help the development of personalized medical management plans in these patients.

Respiratory Failure in a Rare Case of Juvenile Dermatomyositis - Systemic Scleroderma Overlap Syndrome.

Juvenile dermatomyositis (JDM) is one of the pediatric systemic connective tissue disorders, consisting of an idiopathic inflammatory myopathy, affecting primarily skin and muscle, representing approximately 85% of cases in this group. A significant source of morbidity is the occurrence of overlap characteristics with other connective tissue disorders, including systemic sclerosis (SSc). Overlap JDM/SSc syndrome is rare in children, with only a few reported cases. The diagnosis is often challenging, presence of anti-PM/Scl antibodies playing a pivotal role. Although SSc/JDM overlap syndrome has less frequent visceral involvement, pulmonary dysfunction may occur. The respiratory function evaluation using overnight cardiorespiratory polygraphy may reveal important alveolar hypoventilation with impact on therapeutic approach. Non-invasive ventilation may be indicated to potentiate medical treatment. In the acute phase, non-invasive ventilation is a life-saving therapeutic option until the maximum efficiency of drug treatment is reached. In the case of a complex respiratory pathology, associating elements of nocturnal alveolar hypoventilation specific to neuromuscular disease, with that of chronic interstitial lung disease, the evaluation of respiratory sleep disorders should be considered, sometimes requiring home nocturnal noninvasive ventilatory support. We present the case of a 15-year-old girl who was admitted to our clinic with a history of high fever, productive cough and severe dyspnea. Detailed anamnesis revealed that the patient accused oneyear history of proximal muscle weakness of the lower limbs, with functional limitations, weight loss, dysphonia, swallowing difficulties and dyspnea at minimal efforts. Following the physical examination, laboratory and imagistic investigations were all suggestive for an inflammatory myopathy. Anti-PM/Scl antibodies were positive, confirming the diagnosis of a severe form of JDM/SSc overlap syndrome, with minimal cutaneous changes, significant muscle involvement and respiratory distress. Complex therapy using antimicrobial agents, steroid pulse therapy, immunosuppressive agents, non-invasive ventilation, masticaoxygen supplementation and physiotherapy was started, with significant status improvement. However, pulmonary function tests maintained severe restrictive aspect and nocturnal cardio-pulmonary polygraphy revealed residual pulmonary failure with important nocturnal alveolar hypoventilation. Nocturnal non-invasive ventilation was continued at home, along with medical treatment. Her disease was clinically well controlled, immunosuppressive therapy was decreased and interruption of ventilatory support was possible at six months after the diagnosis.

The Prognostic Value of Mannose-Binding Lectin in Community-Acquired Pneumonia.

Introduction: Community-acquired pneumonia is among the most widespread health issues in the pediatric population, affecting children around the world. Mannose-binding lectin is a component of the innate immune system that binds to carbohydrate fragments expressed by various microorganisms, thus aiding in their recognition and eventually activating the complement system through a specific pathway called "the lectin pathway". Materials and methods: 204 pediatric patients whose mannose-binding lectin levels were evaluated at the beginning of infection were included in the study. Results: Mannose-binding lectin deficit was observed Conclusion: This study makes use of the relevant literature and tackles somewhat controversial aspects, as mannose-binding lectin deficit is classified as a fairly common disturbance of the immune system. For a comprehensive understanding of mannose-binding lectin role in infectious diseases, it is necessary to take into account even contextual factors.