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

Ulrike Mütze; Florian Gleich; Ivo Barić; Mathias Baumgartner; Alberto Burlina; Kimberly A. Chapman; Yin Hsiu Chien; Elisenda Cortès-Saladelafont; Corinne De Laet; Dries Dobbelaere; Francois Eysken; Matthias Gautschi; Rene Santer; Johannes Häberle; Clara Joaquín; Daniela Karall; Martin Lindner; Allan M. Lund; Chris Mühlhausen; Elaine Murphy; Dominique Roland; Angeles Ruiz Gomez; Anastasia Skouma; Sarah C. Grünert; Margreet Wagenmakers; Sven F. Garbade; Stefan Kölker; Nikolas Boy

doi:10.1002/jimd.12572

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

Ulrike Mütze
, Florian Gleich
, Ivo Barić
, Mathias Baumgartner
, Alberto Burlina
, Kimberly A. Chapman
, Yin Hsiu Chien
, Elisenda Cortès-Saladelafont
, Corinne De Laet
, Dries Dobbelaere
, Francois Eysken
, Matthias Gautschi
, Rene Santer
, Johannes Häberle
, Clara Joaquín
, Daniela Karall
, Martin Lindner
, Allan M. Lund
, Chris Mühlhausen
, Elaine Murphy

Dominique Roland, Angeles Ruiz Gomez, Anastasia Skouma, Sarah C. Grünert, Margreet Wagenmakers, Sven F. Garbade, Stefan Kölker, Nikolas Boy^*

^*Corresponding author for this work

Internal Medicine

University Hospital Heidelberg
University of Zagreb
University of Zurich
Azienda Ospedale - Università Padova
Children's National Medical Center
National Taiwan University
Josep Carreras Leukaemia Research Institute
Queen Fabiola Children's University Hospital
Université de Lille
Antwerp University Hospital
University Hospital Bern
University Medical Center Hamburg-Eppendorf
Innsbruck Medical University
University Hospital
Copenhagen University Hospital (Nordvest)
University of Göttingen
National Hospital for Neurology and Neurosurgery
Institute of Pathology and Human Genetics (IPG)
Hospital Universitario Son Espases
Institute of Child Health
University of Freiburg Medical Center

Research output: Contribution to journal › Article › Academic › peer-review

9 Citations (Scopus)

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Abstract

The SARS-CoV-2 pandemic challenges healthcare 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-CoV-2 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-CoV-2 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-CoV-2-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 language	English
Pages (from-to)	220-231
Number of pages	12
Journal	Journal of Inherited Metabolic Disease
Volume	46
Issue number	2
Early online date	22 Oct 2022
DOIs	https://doi.org/10.1002/jimd.12572
Publication status	Published - Mar 2023

Bibliographical note

Funding Information:
The authors thank all E‐IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E‐IMD registry for the support during the study period. Furthermore, we are indebted to all E‐IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E‐IMD patient registry has received funding by the European Union (E‐IMD; EAHC no 2010 1201; coordinator: Stefan Kölker), in the framework of the Health Program. After the end of the EU funding period the E‐IMD patient registry has been sustained by funding from the Kindness‐for‐Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL.

Funding Information:
The authors thank all E-IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E-IMD registry for the support during the study period. Furthermore, we are indebted to all E-IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E-IMD patient registry has received funding by the European Union (E-IMD; EAHC no 2010 1201; coordinator: Stefan Kölker), in the framework of the Health Program. After the end of the EU funding period the E-IMD patient registry has been sustained by funding from the Kindness-for-Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL.

Publisher Copyright:
© 2022 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.

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Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases—Data from the E-IMD consortiumFinal published version, 2.54 MBLicence: CC BY-NC-ND

Cite this

Mütze, U., Gleich, F., Barić, I., Baumgartner, M., Burlina, A., Chapman, K. A., Chien, Y. H., Cortès-Saladelafont, E., De Laet, C., Dobbelaere, D., Eysken, F., Gautschi, M., Santer, R., Häberle, J., Joaquín, C., Karall, D., Lindner, M., Lund, A. M., Mühlhausen, C., ... Boy, N. (2023). Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases—Data from the E-IMD consortium. Journal of Inherited Metabolic Disease, 46(2), 220-231. https://doi.org/10.1002/jimd.12572

@article{5e2db82ccd2f4ada823923f312c36f43,

title = "Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases{\textemdash}Data from the E-IMD consortium",

abstract = "The SARS-CoV-2 pandemic challenges healthcare 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-CoV-2 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-CoV-2 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\%{\textendash}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-CoV-2-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.",

author = "Ulrike M{\"u}tze and Florian Gleich and Ivo Bari{\'c} and Mathias Baumgartner and Alberto Burlina and Chapman, \{Kimberly A.\} and Chien, \{Yin Hsiu\} and Elisenda Cort{\`e}s-Saladelafont and \{De Laet\}, Corinne and Dries Dobbelaere and Francois Eysken and Matthias Gautschi and Rene Santer and Johannes H{\"a}berle and Clara Joaqu{\'i}n and Daniela Karall and Martin Lindner and Lund, \{Allan M.\} and Chris M{\"u}hlhausen and Elaine Murphy and Dominique Roland and \{Ruiz Gomez\}, Angeles and Anastasia Skouma and Gr{\"u}nert, \{Sarah C.\} and Margreet Wagenmakers and Garbade, \{Sven F.\} and Stefan K{\"o}lker and Nikolas Boy",

note = "Funding Information: The authors thank all E‐IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E‐IMD registry for the support during the study period. Furthermore, we are indebted to all E‐IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E‐IMD patient registry has received funding by the European Union (E‐IMD; EAHC no 2010 1201; coordinator: Stefan K{\"o}lker), in the framework of the Health Program. After the end of the EU funding period the E‐IMD patient registry has been sustained by funding from the Kindness‐for‐Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL. Funding Information: The authors thank all E-IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E-IMD registry for the support during the study period. Furthermore, we are indebted to all E-IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E-IMD patient registry has received funding by the European Union (E-IMD; EAHC no 2010 1201; coordinator: Stefan K{\"o}lker), in the framework of the Health Program. After the end of the EU funding period the E-IMD patient registry has been sustained by funding from the Kindness-for-Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL. Publisher Copyright: {\textcopyright} 2022 The Authors. Journal of Inherited Metabolic Disease published by John Wiley \& Sons Ltd on behalf of SSIEM.",

year = "2023",

month = mar,

doi = "10.1002/jimd.12572",

language = "English",

volume = "46",

pages = "220--231",

journal = "Journal of Inherited Metabolic Disease",

issn = "0141-8955",

publisher = "Springer Netherlands",

number = "2",

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Mütze, U, Gleich, F, Barić, I, Baumgartner, M, Burlina, A, Chapman, KA, Chien, YH, Cortès-Saladelafont, E, De Laet, C, Dobbelaere, D, Eysken, F, Gautschi, M, Santer, R, Häberle, J, Joaquín, C, Karall, D, Lindner, M, Lund, AM, Mühlhausen, C, Murphy, E, Roland, D, Ruiz Gomez, A, Skouma, A, Grünert, SC, Wagenmakers, M, Garbade, SF, Kölker, S & Boy, N 2023, 'Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases—Data from the E-IMD consortium', Journal of Inherited Metabolic Disease, vol. 46, no. 2, pp. 220-231. https://doi.org/10.1002/jimd.12572

TY - JOUR

T1 - Impact of the SARS-CoV-2 pandemic on the health of individuals with intoxication-type metabolic diseases—Data from the E-IMD consortium

AU - Mütze, Ulrike

AU - Gleich, Florian

AU - Barić, Ivo

AU - Baumgartner, Mathias

AU - Burlina, Alberto

AU - Chapman, Kimberly A.

AU - Chien, Yin Hsiu

AU - Cortès-Saladelafont, Elisenda

AU - De Laet, Corinne

AU - Dobbelaere, Dries

AU - Eysken, Francois

AU - Gautschi, Matthias

AU - Santer, Rene

AU - Häberle, Johannes

AU - Joaquín, Clara

AU - Karall, Daniela

AU - Lindner, Martin

AU - Lund, Allan M.

AU - Mühlhausen, Chris

AU - Murphy, Elaine

AU - Roland, Dominique

AU - Ruiz Gomez, Angeles

AU - Skouma, Anastasia

AU - Grünert, Sarah C.

AU - Wagenmakers, Margreet

AU - Garbade, Sven F.

AU - Kölker, Stefan

AU - Boy, Nikolas

N1 - Funding Information: The authors thank all E‐IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E‐IMD registry for the support during the study period. Furthermore, we are indebted to all E‐IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E‐IMD patient registry has received funding by the European Union (E‐IMD; EAHC no 2010 1201; coordinator: Stefan Kölker), in the framework of the Health Program. After the end of the EU funding period the E‐IMD patient registry has been sustained by funding from the Kindness‐for‐Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL. Funding Information: The authors thank all E-IMD (European Registry and Network for intoxication type metabolic diseases) sites that contributed to the data used in this publication, as well as the E-IMD registry for the support during the study period. Furthermore, we are indebted to all E-IMD individuals and their families for their trust, patience and participation in longitudinal registry studies for many years. The E-IMD patient registry has received funding by the European Union (E-IMD; EAHC no 2010 1201; coordinator: Stefan Kölker), in the framework of the Health Program. After the end of the EU funding period the E-IMD patient registry has been sustained by funding from the Kindness-for-Kids Foundation (Munich, Germany), the Kettering Fund, and Dietmar Hopp Foundation. Open Access funding enabled and organized by Projekt DEAL. Publisher Copyright: © 2022 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.

PY - 2023/3

Y1 - 2023/3

N2 - The SARS-CoV-2 pandemic challenges healthcare 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-CoV-2 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-CoV-2 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-CoV-2-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.

AB - The SARS-CoV-2 pandemic challenges healthcare 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-CoV-2 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-CoV-2 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-CoV-2-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.

UR - https://www.scopus.com/pages/publications/85142204589

U2 - 10.1002/jimd.12572

DO - 10.1002/jimd.12572

M3 - Article

C2 - 36266255

AN - SCOPUS:85142204589

SN - 0141-8955

VL - 46

SP - 220

EP - 231

JO - Journal of Inherited Metabolic Disease

JF - Journal of Inherited Metabolic Disease

IS - 2

ER -

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

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