Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross-sectional study.

Background and Aims: Sputum neutrophil elastase (NE) is a marker of neutrophilic airway inflammation in bronchiectasis. Yet, not much is known about its role in pediatric bronchiectasis severity. This study aimed to assess the sputum NE value as a biomarker of clinical and radiological severity in pediatric bronchiectasis. Methods: This was a cross-sectional study assessing sputum NE in a total of 50 bronchiectasis patients under the age of 18 years-30 patients with cystic fibrosis (CF) and 20 patients with non-CF bronchiectasis were included. Bronchiectasis severity was assessed using Shwachman-Kulczycki (SK) score, CF-ABLE score, and CF risk of disease progression score, among CF patients, and bronchiectasis severity index (BSI) and FACED criteria among non-CF bronchiectasis patients, associations between sputum NE and bronchiectasis severity were assessed in both patient groups. Results: Sputum NE was directly correlated with C-reactive protein (r = 0.914, p < 0.001), (r = 0.786, p < 0.001), frequency of exacerbations (r = 0.852, p < 0.001) (r = 0.858, p < 0.001), exacerbations severity (r = 0.735, p = 0.002), (r = 0.907, p < 0.001), and the number of hospital admissions (r = 0.813, p < 0.001), (r = 0.612, p =0.004) in the last year among CF, and non-CF bronchiectasis patients, respectively. Additional linear correlations were found between sputum NE, CF risk of disease progression score (p < 0.001), CF-ABLE score (p < 0.001), and lower forced expiratory volume 1% of predicted (p = 0.017; ρ = -0.8) among CF patients. Moreover, sputum NE was positively correlated with the neutrophil count (p = 0.018), and BSI severity score (p = 0.039; ρ = 0.465) among non-CF bronchiectasis patients. Conclusions: Sputum NE may be considered a good biomarker of bronchiectasis severity in both CF and non-CF bronchiectasis patients, as confirmed by the exacerbations rate, CF risk of disease progression, and BSI scores.

Serum homocysteine level in pediatric patients with COVID-19 and its correlation with the disease severity.

BACKGROUND: Thrombosis and embolism are possible complications in coronavirus disease 2019 (COVID)-19-positive pediatric patients. Although the risk is lesser in children than it is in adults, it does exist during acute infection and multi-inflammatory syndrome in children. Biomarkers such asd-dimer, prothrombin time, and fibrinogen degradation products are ineffective at detecting disease severity. Homocysteine (Hcy) is a prothrombotic factor that has been reported to be higher in adult COVID-19 patients, leading to speculation that it could be used as a biomarker for disease severity. PURPOSE: To detect the correlation between serum total homocysteine (tHcy) level and the severity of COVID-19 in pediatrics. METHODS: A cross-sectional study was conducted on 40 children with COVID-19 and 40 healthy control subjects. Serum tHcy was measured by enzyme-linked immunosorbent assay and correlated with the clinical, laboratory, and radiological parameters of the patients. RESULTS: The median serum tHcy level in COVID-19 patients was 27.5 (interquartile range [IQR]: 23-31.75) µmol/L, while that in the controls was 1.8 (IQR: 1.6-1.875) µmol/L. There was a statistically significant increase in the tHcy level in cases compared to controls (p < 0.001). There was a statistically significant positive correlation between serum tHcy and d-dimer, ferritin, alanine transaminase, aspartate transaminase, blood urea nitrogen, and a highly significant positive correlation between tHcy and COVID-19 reporting and data system score, pediatric intensive care unit admission, and the disease severity classification. CONCLUSION: Hcy could be a biomarker of importance in predicting the severity of COVID-19 in pediatrics.

COVID-19-Related Multisystem Inflammatory Syndrome in Children Presenting With New-Onset Type 1 Diabetes in Severe Ketoacidosis: A Case Series.

OBJECTIVE: To report and describe cases of children presenting with coronavirus disease 2019 (COVID-19)-related multisystem inflammatory syndrome in children (MIS-C) with new-onset type 1 diabetes mellitus (T1DM) in severe diabetic ketoacidosis (DKA). RESEARCH DESIGN AND METHODS: This prospective observational study was conducted to characterize children with COVID-19-related MIS-C and new-onset T1DM who were in DKA. MIS-C was diagnosed if Centers for Disease Control and Prevention and World Health Organization criteria were fulfilled. RESULTS: Six cases were identified. The patients were critically ill and in nonfluid responsive shock (combined hypovolemic and cardiogenic or distributive shock). All had cardiac involvement. One patient had a Kawasaki shock-like presentation. All needed aggressive treatment with careful monitoring of fluid balance (because of associated cardiac dysfunction), early institution of vasoactive/inotropic supports, and use of methylprednisolone and intravenous immunoglobulins. The latter are better administered after DKA resolution to avoid undue volume overload and fluid shifts while the patients are in DKA. CONCLUSIONS: Awareness of MIS-C coexistence with DKA at T1DM onset is crucial for rapid proper management.

Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt.

BACKGROUND: Childhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing Middle Eastern countries, chILD is still underdiagnosed. Our objective was to describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt. METHODS: We analysed data of consecutive subjects (aged <18 years) referred for further evaluation at the Children's Hospital, Ain Shams University (Cairo, Egypt). Diagnosis of chILD was made in accordance with the ChILD-EU criteria. The following information was obtained: demographic data, clinical characteristics, chest computed tomography findings, laboratory studies, spirometry, bronchoalveolar lavage and histopathology findings. RESULTS: 22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5-14 years). The most common manifestations were dyspnoea (100%), cough (90.9%), clubbing (95.5%) and tachypnoea (90.9%). Systematic evaluation led to the following diagnoses: hypersensitivity pneumonitis (n=3), idiopathic interstitial pneumonias (n=4), chILD related to chronic granulomatous disease (n=3), chILD related to small airways disease (n=3), post-infectious chILD (n=2), Langerhans cell histiocytosis (n=2), idiopathic pulmonary haemosiderosis (n=2), granulomatous lymphocytic interstitial lung disease (n=1), systemic sclerosis (n=1) and familial interstitial lung disease (n=1). Among the subjects who completed the diagnostic evaluation (n=19), treatment was changed in 13 (68.4%) subjects. CONCLUSION: Systematic evaluation and multidisciplinary peer review of chILD patients at our tertiary hospital led to changes in management in 68% of the patients. This study highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving healthcare for children with chILD.

The "Golden Hours" Algorithm For the Management of the Multisystem Inflammatory Syndrome in Children (MIS-C).

The global concern of increasing number of children presenting with multisystem inflammatory syndrome in children (MIS-C) related to the coronavirus disease (COVID-19) has escalated the need for a case-oriented clinical approach that provides timely diagnosis and management. The aim of this study is to share our experience in managing 64 MIS-C patients of North African ethnicity guided by a risk-based algorithm. Sixty-four patients met the inclusion criteria, 19 (30%) patients were categorized as mild and moderate risk groups and cared for in an isolation ward and 45 patients who belonged to the high-risk group (70%) were admitted to the pediatric intensive care unit (PICU). Positive laboratory evidence of COVID-19 was found in 62 patients. Fever and dysfunction in 2 or more organs were confirmed in all cases (100%). Fifty patients (78%) presented with gastrointestinal symptoms, meanwhile only 10 patients (16%) had respiratory manifestations. Cardiac involvement was reported in 55 (86%) cases; hypotension and shock were found in 45 patients (70%) therein circulatory support and mechanical ventilations were needed for 45 and 13 patients respectively. Intravenous immunoglobulins (IVIG) were used for all cases and methylprednisolone was used in 60 patients (94%). Fifty-eight (91%) patients were discharged home after an average of 9 days of hospitalization. The mortality rate was 9% (6 patients). Conclusion. A single Egyptian center experience in the management of MIS-C patients guided by a proposed bed side algorithm is described. The algorithm proved to be a helpful tool for first-line responders, and helped initiate early treatment with IVIG.

Genetic variants in vitamin D pathway in Egyptian asthmatic children: a pilot study.

OBJECTIVES: Asthma is a genetically heterogeneous disease. Genetic variants in vitamin D pathway have been reported to be involved with asthma risk. The study aimed to test whether vitamin D binding protein (VDBP or GC-group component) and vitamin D receptor (VDR) gene polymorphisms were associated with asthma characteristics as well as vitamin D level in Egyptian children. DESIGN AND METHODS: The study included 51 asthmatic children and 33 healthy controls of matched sex and age. All participants were genotyped for two SNPs; GC (rs2282679) and VDR (rs2228570) using TaqMan allele discrimination assays. RESULTS: Genotype distribution of GC and VDR showed a significant association with asthma (P = 0.02, P = 0.002). Children carrying the risk "G" allele for GC SNP are 2.22 times more prone to develop asthma [OR = 2.22, 95% CI (1.18-4.2)] whereas those carrying the risk "F" allele for VDR SNP are nearly twice and half times susceptible for asthma development [OR = 2.68, 95% CI (1.36-5.28)] than healthy individuals. For the GC SNP, homozygous children "GG" exhibited significant difference in pulmonary functions (FEV1, FEV1/FVC), asthma severity and asthma control, IgE and vitamin D levels compared to pooled cases of GT and TT genotypes. For the VDR SNP, no significant association between VDR variants and the tested characteristics except for the pulmonary functions where the FEV1/FVC in asthmatic children with "FF " genotype differ significantly from those carrying "Ff"genotype. CONCLUSION: GC and VDR variants may be implicated in asthma susceptibility; hence, further larger studies are still needed to extrapolate our findings to the general population.