ABSTRACT
AIM: There are no recommended guidelines or clinical studies on safety of COVID-19 vaccines in patients with inborn errors of metabolism (IEMs). Here, we aimed to examine the relationship between COVID-19 vaccination and metabolic outcome in paediatric IEM patients. METHODS: Patients with IEM between the ages of 12 and 18 were enrolled. Term metabolic decompensation was defined as acute disruption in metabolic homeostasis due to vaccination. Clinical and biochemical markers were compared between pre- and post-vaccination periods. RESULTS: Data from a total of 36 vaccination episodes in 18 patients were included. Thirteen patients had intoxication-type metabolic disorders including organic acidemia (OA), urea cycle disorders (UCDs), maple syrup urine disease (MSUD) and phenylketonuria (PKU); 4 patients had energy metabolism disorders including fatty acid metabolism disorders and LIPIN 1 deficiency; and 1 patient had glycogen storage disorder (GSD) type 5. Seventeen patients received BNT162b2, and 1 received CoronaVac because of an underlying long QT syndrome. Fatty acid metabolism disorders, LIPIN 1 deficiency and GSD type 5 were included in the same group named 'metabolic myopathies'. In two PKU patients, plasma phenylalanine level increased significantly within 24 h following the second dose of vaccination. None of the OA, UCD, MSUD and metabolic myopathy patients experienced acute metabolic attack and had emergency department admission due to metabolic decompensation within 1 month after vaccination. CONCLUSIONS: COVID-19 vaccines did not cause acute metabolic decompensation in a cohort of 18 children with IEM.
ABSTRACT
Introduction: SARS-CoV-2 infection can lead to a life-threatening acute metabolic decompensation in children with inborn errors of metabolism (IEM), so vaccination is mandatory. However, IEMs can also impair innate or adaptive immunity, and the impact of these immune system alterations on immunogenicity and vaccine efficacy is still unknown. Here, we investigated humoral immune responses to the BNT162b2 mRNA COVID-19 vaccine and clinical outcomes in pediatric IEM patients. Methods: Fifteen patients between 12-18 years of age with a confirmed diagnosis of IEM, and received BNT162b2 were enrolled to the study. Patients with an anti-SARS-CoV-2 IgG concentration >50 AU/mL before vaccination were defined as "COVID-19 recovered" whereas patients with undetectable anti-SARS-CoV-2 IgG concentration were defined as "COVID-19 naïve". Anti-SARS-CoV-2 Immunoglobulin G (IgG) and SARS-CoV-2 neutralizing antibody (nAb) titers were measured to assess humoral immune response. Results: Anti-SARS-CoV-2 IgG titers and nAb IH% increased significantly after the first dose. The increase in antibody titers after first and second vaccination remained significant in COVID-19 naïve patients. Complete anti-SARS-CoV-2 IgG seropositivity and nAb IH% positivity was observed in all patients after the second dose. Vaccination appears to be clinically effective in IEM patients, as none of the patients had COVID-19 infection within six months of the last vaccination. Discussion: Humoral immune response after two doses of BNT162b2 in pediatric IEM patients was adequate and the immune response was not different from that of healthy individuals.
Subject(s)
COVID-19 , Metabolism, Inborn Errors , Humans , Child , BNT162 Vaccine , COVID-19 Vaccines , COVID-19/prevention & control , SARS-CoV-2 , Antibodies, Neutralizing , Vaccination , Immunoglobulin GABSTRACT
Amaç: Hangi kritik Koronavirüs hastalığı-2019 (COVID-19) hastalarının fungal enfeksiyon geliştirme olasılığının daha yüksek olduğu konusu ve olası risk faktörleri belirsizliğini korumaktadır. Özellikle COVID-19 hastalarında invaziv fungal enfeksiyon gelişiminde kortikosteroidlerin ve diğer immün modülatörlerin rolü henüz aydınlatılamamıştır. Bu çalışmanın amacı, kritik COVID-19 hastalarında fungemi ilişkili klinik özellikleri, sonuçları ve risk faktörlerini tanımlamaktır. Gereç ve Yöntem: Etik kurul onamı sonrası üniversite hastanesi düzey 3 yoğun bakım ünitesi COVID-19 tanısı ile kabul edilen 550 hasta tarandı. Hastaların verileri kaydedilerek analizleri sağlandı. Bulgular: Taranan 550 hastadan fungal enfeksiyon tanısı alan 14 hasta çalışmaya dahil. Bu hastaların 5’i kadındı ve yaş ortalaması 55 yıl idi. Hastaların kabul anı karakteristik özellikleri Tablo 1’de gösterildi. Hastalar yüksek Akut Fizyoloji ve Kronik Sağlık Değerlendirmesi II ve Ardışık Organ Yetmezliği Değerlendirme Skoruna sahiptiler. Yoğun bakım yatış süresinde hastalara uygulanan tedaviler ve primer, sekonder sonlanımlar Tablo 2’de gösterilmekle beraber fungemi hastalarının %85,7’si kaybedildi. Hastalarda uzun süre hastane ve yoğun bakım yatışı gözlendi. Fungal enfeksiyon anındaki karakteristik özellikler ve fungal enfeksiyona ait bilgiler Tablo 3’te gösterildi. Bu hastalarda enfeksiyon öncesi uzun süre steroid kullanım zamanıyla göze çarparken beraber en sık odak periferik kan en sık üreyen mikroorganizma Candida türleri idi. Sonuç: Çalışmamız sonucunda en sık fungemi etkeni Candida türleri olarak belirlenmiştir. COVID-19 hastalarında normal popülasyonla karşılaştırdığımızda fungemi insidansı 1.000 yoğun bakım günü başına normal popülasyon daha yüksekti. Fungemi saptanan hastalarda %85,7 gibi yüksek mortalite oranları saptandı. Fungemi saptanan hastalarda mortalite için risk faktörleri uzun süre steroid kullanımı, uzun süre yoğun bakım yatışı olarak bulundu. Çalışmanın karşılaştırmalı analizleri yapılmaya devam edilmektedir. Ön analiz sonuçları sunulmuştur. (Turkish) [ FROM AUTHOR] Copyright of Turkish Journal of Intensive Care is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full . (Copyright applies to all s.)