Diagnostic Challenges in Hemophagocytic Lymphohistiocytosis, a Rare, Potentially Fatal Disease: Two Case Studies.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, and potentially fatal, syndrome, characterized by immune system dysregulation, with excessive activation of the macrophages and cytotoxic T cells. It can be classified into primary (genetic) and secondary (acquired) forms. HLH presents with fever, hepatosplenomegaly, cytopenia, and hyperferritinemia, with involvement of various organs. The initial symptoms of HLH are non-specific, but as, if untreated, it can progress rapidly to multiorgan failure, timely diagnosis is essential. We present here two cases of HLH in infants that illustrate the importance of early diagnosis and appropriate treatment, along with a short review of HLH.

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.

The Challenging Diagnosis of Pediatric Multisystem Inflammatory Syndrome Associated with Sars-Cov-2 Infection-Two Case Reports and Literature Review.

Severe acute respiratory coronavirus 2 (SARS-CoV-2) is a novel coronavirus discovered in 2019 that caused the coronavirus disease 2019 (COVID19). During the last year, over 70 million people were infected and more than 1.5 million people died. Despite the tremendous number of people infected, children were less affected and presented milder forms of the disease. A short time after the pandemic was declared, a new hyperinflammatory syndrome resembling Kawasaki disease (KD) was described in children with confirmed or suspected SARS-CoV-2 infection named multisystem inflammatory syndrome in children (MIS-C). The incidence of MIS-C is low and it has a polymorphous clinical presentation, making the diagnosis difficult. Although the incidence is reduced, there is a high risk of cardiovascular complications. In order to raise awareness, we present the cases of two pediatric patients diagnosed with MIS-C in our clinic.

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.

The role of glomerular morphometric features in pediatric podocytopathies - a single center study.

Podocytopathies represent a well-studied subgroup of glomerulopathies, being characterized by proteinuria due to damage or dysfunction of podocytes. Glomerular size in podocytopathies has been studied in different population, but only a few studies take in consideration the pediatric population. There are different methods to assess the glomerular size, but most of the studies report the maximal profile area as being the most accurate one. The aim of this study is to determine the range values of glomeruli in pediatric population with glomerulopathies and to establish a correlation between the measured size and several laboratory features. The patients that undergo renal biopsy in the Department of Nephrology, "Maria Sklodowska Curie" Clinical Emergency Hospital for Children, Bucharest, Romania, were divided into two groups: control vs. affected∕patient group. The control group included children that require renal biopsy for renal impairments other than high-range proteinuria (most of them recurrent microscopic asymptomatic hematuria), while the affected group had nephrotic-range proteinuria. Thirty patients were selected to be part of the control group and 30 patients in the affected group. In control group, the mean value diameter was 166.23±13.04 µm, and the area of the glomerulus had a mean value of 19 126.86±3070.83 µm². In the affected group, we obtained the following results: the mean value diameter was 192.42±28.15 µm, while the glomerular cross-sectional area had a mean value of 23 535.55±6456.57 µm². Using the linear regression, we concluded that all the cases with increased-size glomeruli had more urinary protein loss compared with the ones that had small-size glomeruli and low-range proteinuria.