Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), also known as autoimmune polyglandular syndrome type 1 (APS-1) is a rare autosomal recessive inborn error of immunity (IEI), which is accompanied by immune dysregulation. Hypoparathyroidism, adrenocortical failure and candidiasis are its typical manifestations. Here we report about recurrent COVID-19 in a 3-year-old boy with APECED, who developed retinopathy with macular atrophy and autoimmune hepatitis after the first episode of SARS-CoV-2 infection. Primary Epstein-Barr virus infection and a new episode of SARS-CoV-2 infection with COVID pneumonia triggered the development of severe hyperinflammation with signs of hemophagocytic lymphohistiocytosis (HLH): progressive cytopenia (thrombocytopenia, anemia, lymphopenia), hypoproteinemia, hypoalbuminemia, high levels of liver enzymes, hyperferritinemia, increased triglycerides levels; and coagulopathy with a low level of fibrinogen. Treatment with corticosteroids and intravenous immunoglobulins did not lead to a significant improvement. The progression of HLH and COVID-pneumonia resulted in a fatal outcome. The rarity and varied presentation of the HLH symptoms led to diagnostic difficulties and diagnosis delay. HLH should be suspected in a patient with immune dysregulation and impaired viral response. Treatment of infection-HLH is a major challenge due to the difficulties in balancing immunosuppression and management of underlying/triggering infection.
The problem of thyroid dysfunction related to SARS-CoV-2 infection remains unclear in children. Therefore, the study aimed to reveal the interrelationship between thyroid dysfunction and COVID-19 severity as well as to determine optimal cut-off values for screening for thyroid disorders in children. A total number of 90 children aged from 1 month to 17 years were involved in the study. Patients with known thyroid disease were not recruited for the research. A thyroid panel was assessed for all participants that included: free triiodothyronine (FT3), free thyroxine (FT4), thyroid-stimulating hormone (TSH) and anti-thyroid peroxidase (ATPO) antibodies. Statistical analysis was done using the computer software Statistica 13.0. Research has revealed euthyroid sick syndrome (ESS) in 14.10% of SARS-CoV-2 infected children more often among patients with severe COVID-19 and multisystem inflammatory syndrome (33.33%) compared to mild COVID-19 course (6.67%) and moderate disease severity (8.89%) (P < 0.05). Significant correlation relationships were revealed for next values – FT3 and erythrocyte sedimentation rate (ESR) (rs = -0.22; P < 0.05); FT3 and C-reactive protein (CRP) (rs = -0.33; P < 0.05); FT3 and procalcitonin (rs = -0.43; P < 0.05). The next cut-off values for ESS determination were revealed: ESR 18.5 mm/h (AUC 0.803); CRP 11.5 mg/l (AUC 0.763); ferritin 84.8 ng/ml (AUC 0.733). Results suggest that pediatricians should pay attention to the endocrine disruptions by COVID-19 in children. Keywords: COVID-19, euthyroid sick syndrome in children, inflammatory markers
Метою роботи був аналіз клінічних особливостей та лікування мультисистемного запального синдрому, асоційованого із SARS-CoV-2, на прикладі клінічного випадку у хлопчика раннього шкільного віку.
Наведено динаміку клінічних проявів, лабораторно-інструментальних показників у дитини з Кавасакі-подібним захворюванням, асоційованим із COVID-19, під час стаціонарного лікування у відділенні інтенсивної терапії міської дитячої комунальної лікарні м. Тернополя. Акцентується увага практичних лікарів на важливості своєчасної діагностики рідкісного, але потенційно тяжкого мультисистемного запального синдрому, асоційованого з COVID-19, у дітей.
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