ABSTRACT
Introduction: SARS-CoV-2 in children with CF has been reported to cause mild illness in the absence of pre-existing severe lung disease. In this review, we aim to describe the clinical presentation and course of COVID-19 infection in CF population in Qatar. Method(s): Our Pediatric CF registry (51 patients) was reviewed for COVID-19 cases from February 2020 to 2022. Demographics, vaccination status, symptoms and course were reviewed. Data were expressed as median (Range) and frequencies. Result(s): Eight patients with CF < 18 years of age had COVID-19 infection based on positive PCR in 6/8 or antigen in 2/8 of the cases. Incidence of COVID-19 in our CF population was 15.7%. Median age was 11 (2-18) years. Four were males. Seven patients were pancreatic sufficient (I1234V mutation). Median baseline FEV1%predicted was 91 (78-107)%. None had received CFTR modulators or underwent lung transplant. Three patients were vaccinated prior to their infections;two of them were asymptomatic. Six patients had cough and flu-like symptoms;three of them had concurrent fever. None reported dyspnea, hemoptysis, myalgia or gastrointestinal symptoms. Two patients were hospitalized due to pulmonary exacerbation, both had mild CF-lung disease. None required respiratory support. Three patients required oral antibiotics. Conclusion(s): We report a favorable outcome in COVID-19 infection in children with CF that is comparable to the published study (Bain R et al. Journal of Cystic Fibrosis, 2020.11.021). Our findings may be related to our unique milder CFTR mutation;precautionary measures;awareness and vaccination campaigns. Nevertheless, vaccinations and maintaining precautionary measures remain essential.
ABSTRACT
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and small-vessel vasculitis. We report a case of EGPA in an adolescent with uncontrolled asthma who was receiving montelukast. Case: A 12-year-old boy who is known to have asthma and allergic rhinitis which were previously controlled on ICS, intranasal steroids, and prolonged use of montelukast for 4 years. He presented with cough and nasal blockage for 2 months. He also reported an increase in the frequency of asthma attacks and received multiple courses of systemic steroids. Subsequently, his asthma controller medications were upgraded to ICS/LABA few weeks prior to admission. His symptoms were also associated with weight loss, diarrhoea and haematochezia. He was vitally stable and maintained oxygen saturation on room air. Physical examination revealed nasal polyps, purple skin flat lesions on palms and feet (Figure1), and bilateral crackles on chest auscultation. His blood investigations were significant for leukocytosis with marked eosinophilia (11x103/uL, (51%)), high inflammatory markers and total-IgE (1975 kU/L). Initial chest XR showed bilateral interstitial thickening and small pleural effusions (Figure2). Chest CT showed centrilobular nodules and peripheral ground-glass opacities, tree-in-bud appearance with no peripheral sparing in addition to moderate pericardial effusion and bilateral mild pleural effusion (Figure3). Sinus CT showed extensive sino-nasal polyposis with pansinusitis (Figure4). Initial echocardiography showed moderate pericardial effusion with normal biventricular function. Patient was started on IV furosemide. During his hospitalization, patient developed chest pain. His serial troponin was rising and LV contractility was depressed. ECG showed ST-segment depression. Therefore, EGPA with cardiac involvement was suspected. Cardiac MR showed features of a peri-myocarditis. IVIG was commenced for suspicion of coronary artery involvement, which was later disputed by cardiac cath. He was also started on IV pulse steroids at a dose of 30 mg/kg for 3 days which resulted in dramatic decrease in troponin level, eosinophil count and CRP. Skin biopsy, which was later performed after administration of steroids, showed perivascular non-necrotizing granulomas. His ANA, ANCA and COVID-19 PCR came negative. Serum chemistries and urine microscopy were unremarkable. Patient was later started on Rituximab with significant clinical, serological and radiological (Figure5,6) improvement after 10-months of follow-up. Discussion: EGPA is rare but should be considered in children with uncontrolled asthma, eosinophilia and rhino-sinusitis. This case shows the importance of being aware that montelukast could cause EGPA, in spite of the uncertainty about its mechanism. (Figure Presented).