tetano
Editor, Senior Moderator
J Inherit Metab Dis
. 2022 Oct 20.
doi: 10.1002/jimd.12572. Online ahead of print.
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[SUP] 1 [/SUP], F Gleich[SUP] 1 [/SUP], I Barić[SUP] 2 [/SUP], M Baumgartner[SUP] 3 [/SUP], A Burlina[SUP] 4 [/SUP], K A Chapman[SUP] 5 [/SUP], Y Chien[SUP] 6 [/SUP], E Cortès-Saladelafont[SUP] 7 [/SUP], C De Laet[SUP] 8 [/SUP], D Dobbelaere[SUP] 9 [/SUP], F Eysken[SUP] 10 [/SUP], M Gautschi[SUP] 11 [/SUP], R Santer[SUP] 12 [/SUP], J Häberle[SUP] 3 [/SUP], C Joaquín[SUP] 13 [/SUP], D Karall[SUP] 14 [/SUP], M Lindner[SUP] 15 [/SUP], A M Lund[SUP] 16 [/SUP], C Mühlhausen[SUP] 17 [/SUP], E Murphy[SUP] 18 [/SUP], D Roland[SUP] 19 [/SUP], A Ruiz Gomez[SUP] 20 [/SUP], A Skouma[SUP] 21 [/SUP], S C Grünert[SUP] 22 [/SUP], M Wagenmakers[SUP] 23 [/SUP], S F Garbade[SUP] 1 [/SUP], S Kölker[SUP] 1 [/SUP], N Boy[SUP] 1 [/SUP]
Affiliations
Abstract
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.
Keywords: COVID-19; Coronavirus; E-IMD; IMD; Pandemic; SARS-CoV-2; Survey; intoxication-type inherited metabolic diseases.
. 2022 Oct 20.
doi: 10.1002/jimd.12572. Online ahead of print.
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[SUP] 1 [/SUP], F Gleich[SUP] 1 [/SUP], I Barić[SUP] 2 [/SUP], M Baumgartner[SUP] 3 [/SUP], A Burlina[SUP] 4 [/SUP], K A Chapman[SUP] 5 [/SUP], Y Chien[SUP] 6 [/SUP], E Cortès-Saladelafont[SUP] 7 [/SUP], C De Laet[SUP] 8 [/SUP], D Dobbelaere[SUP] 9 [/SUP], F Eysken[SUP] 10 [/SUP], M Gautschi[SUP] 11 [/SUP], R Santer[SUP] 12 [/SUP], J Häberle[SUP] 3 [/SUP], C Joaquín[SUP] 13 [/SUP], D Karall[SUP] 14 [/SUP], M Lindner[SUP] 15 [/SUP], A M Lund[SUP] 16 [/SUP], C Mühlhausen[SUP] 17 [/SUP], E Murphy[SUP] 18 [/SUP], D Roland[SUP] 19 [/SUP], A Ruiz Gomez[SUP] 20 [/SUP], A Skouma[SUP] 21 [/SUP], S C Grünert[SUP] 22 [/SUP], M Wagenmakers[SUP] 23 [/SUP], S F Garbade[SUP] 1 [/SUP], S Kölker[SUP] 1 [/SUP], N Boy[SUP] 1 [/SUP]
Affiliations
- PMID: 36266255
- DOI: 10.1002/jimd.12572
Abstract
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.
Keywords: COVID-19; Coronavirus; E-IMD; IMD; Pandemic; SARS-CoV-2; Survey; intoxication-type inherited metabolic diseases.