Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases - data from the E-IMD consortium

U Mütze, F Gleich, I Barić, M Baumgartner, A Burlina, K A Chapman, Y Chien, E Cortès-Saladelafont, C De Laet, D Dobbelaere, F Eysken, M Gautschi, R Santer, J Häberle, C Joaquín, D Karall, M Lindner, A M Lund, C Mühlhausen, E MurphyD Roland, A Ruiz Gomez, A Skouma, S C Grünert, M Wagenmakers, S F Garbade, S Kölker, N Boy*

*Corresponding author for this work


The SARS-CoV2 pandemic challenges health care systems worldwide. Within inherited metabolic disorders (IMDs) the vulnerable subgroup of intoxication-type IMDs such as organic acidurias (OA) and urea cycle disorders (UCD) show risk for infection-induced morbidity and mortality. This study (observation period February 2020 to December 2021) evaluates impact on medical health care as well as disease course and outcome of SARS-CoV2-infections in patients with intoxication-type IMDs managed by participants of the European Registry and Network for intoxication type metabolic diseases Consortium (E-IMD). Survey's respondents managing 792 patients (n=479 pediatric; n=313 adult) with intoxication-type IMDs (n=454 OA; n=338 UCD) in 14 countries reported on 59 (OA: n=36; UCD: n=23), SARS-CoV2-infections (7.4%). Medical services were increasingly requested (95%), mostly alleviated by remote technologies (86%). Problems with medical supply were scarce (5%). Regular follow-up visits were reduced in 41% (range 10-50%). Most infected individuals (49/59; 83%) showed mild clinical symptoms, while 10 patients (17%; n=6 OA including four transplanted MMA patients; n=4 UCD) were hospitalized (metabolic decompensation in 30%). ICU treatment was not reported. Hospitalization rate did not differ for diagnosis or age group (p=0.778). Survival rate was 100%. Full recovery was reported for 100% in outpatient care and 90% of hospitalized individuals. SARS-CoV-2 impacts health care of individuals with intoxication-type IMDs worldwide. Most infected individuals, however, showed mild symptoms and did not require hospitalization. SARS-CoV2-induced metabolic decompensations were usually mild without increased risk for ICU treatment. Overall prognosis of infected individuals is very promising and IMD-specific or COVID-19-related complications have not been observed.

Original languageEnglish
JournalJournal of Inherited Metabolic Disease
Issue number2
Pages (from-to)220-231
Number of pages12
Publication statusPublished - Mar 2023


  • Adult
  • COVID-19
  • Child
  • Humans
  • Metabolic Diseases
  • Pandemics
  • SARS-CoV-2
  • Urea Cycle Disorders, Inborn/complications


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