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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 consortium.
Mütze, Ulrike; Gleich, Florian; Baric, Ivo; Baumgartner, Mathias; Burlina, Alberto; Chapman, Kimberly A; Chien, Yin-Hsiu; Cortès-Saladelafont, Elisenda; De Laet, Corinne; Dobbelaere, Dries; Eysken, Francois; Gautschi, Matthias; Santer, Rene; Häberle, Johannes; Joaquín, Clara; Karall, Daniela; Lindner, Martin; Lund, Allan M; Mühlhausen, Chris; Murphy, Elaine; Roland, Dominique; Ruiz Gomez, Angeles; Skouma, Anastasia; Grünert, Sarah C; Wagenmakers, Margreet; Garbade, Sven F; Kölker, Stefan; Boy, Nikolas.
Afiliação
  • Mütze U; Centre for Child and Adolescent Medicine, Division of Neuropaediatrics and Metabolic Medicine, Department of General Paediatrics, University Hospital Heidelberg, Heidelberg, Germany.
  • Gleich F; Centre for Child and Adolescent Medicine, Division of Neuropaediatrics and Metabolic Medicine, Department of General Paediatrics, University Hospital Heidelberg, Heidelberg, Germany.
  • Baric I; University of Zagreb, School of Medicine and Department of Pediatrics, University Hospital Center Zagreb, Zagreb, Croatia.
  • Baumgartner M; University Children's Hospital and Children's Research Center, University of Zurich, Zurich, Switzerland.
  • Burlina A; Division of Inherited Metabolic Disease, University Hospital Padova, Padova, Italy.
  • Chapman KA; Children's National Medical Center, Washington, DC, USA.
  • Chien YH; Department of Medical Genetics and Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
  • Cortès-Saladelafont E; University Hospital Germans Trias i Pujol, Department of Paediatrics, Unit of Inherited Metabolic Diseases and Neuropediatrics, Universitat Autònoma de Barcelona, Badalona, Spain.
  • De Laet C; Hôpital Universitaire des Enfants Reine Fabiola-Université Libre de Bruxelles, Brussels, Belgium.
  • Dobbelaere D; Medical Reference Center for Inherited Metabolic Diseases, Jeanne de Flandre University Hospital and RADEME Research Team for Rare Metabolic and Developmental Diseases, CHRU Lille, Lille, France.
  • Eysken F; Department of Pediatric Inherited Metabolic Diseases, University Hospital of Antwerp UZA, Antwerp, Belgium.
  • Gautschi M; Department of Paediatrics, Division of Paediatric Endocrinology, Diabetology and Metabolism, Inselspital, University Hospital Bern, Bern, Switzerland.
  • Santer R; University Children's Hospital, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.
  • Häberle J; University Children's Hospital and Children's Research Center, University of Zurich, Zurich, Switzerland.
  • Joaquín C; Endocrinology and Nutrition Department, Hospital Universitari Germans Trias i Pujol, Badalona, Spain.
  • Karall D; Clinic for Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.
  • Lindner M; Goethe University Frankfurt/Main, Uni.-Children's Hospital, Frankfurt/Main, Germany.
  • Lund AM; Centre Inherited Metabolic Diseases, Departments of Paediatrics and Clinical Genetics, Copenhagen University Hospital, Copenhagen, Denmark.
  • Mühlhausen C; Universitätsmedizin Göttingen, Georg-August-Universität, Göttingen, Germany.
  • Murphy E; National Hospital for Neurology and Neurosurgery, Charles Dent Metabolic Unit, London, UK.
  • Roland D; Institute of Pathology and Human Genetics (IPG), Charlerois, Belgium.
  • Ruiz Gomez A; Hospital Universitario Son Espases, Palma de Mallorca, Spain.
  • Skouma A; Institouto Ygeias Tou Paidiou, Institute of Child Health, Athens, Greece.
  • Grünert SC; Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Centre, University of Freiburg, Faculty of Medicine, Freiburg, Germany.
  • Wagenmakers M; Erasmus Medical Center, Rotterdam, The Netherlands.
  • Garbade SF; Centre for Child and Adolescent Medicine, Division of Neuropaediatrics and Metabolic Medicine, Department of General Paediatrics, University Hospital Heidelberg, Heidelberg, Germany.
  • Kölker S; Centre for Child and Adolescent Medicine, Division of Neuropaediatrics and Metabolic Medicine, Department of General Paediatrics, University Hospital Heidelberg, Heidelberg, Germany.
  • Boy N; Centre for Child and Adolescent Medicine, Division of Neuropaediatrics and Metabolic Medicine, Department of General Paediatrics, University Hospital Heidelberg, Heidelberg, Germany.
J Inherit Metab Dis ; 46(2): 220-231, 2023 03.
Article em En | MEDLINE | ID: mdl-36266255
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Distúrbios Congênitos do Ciclo da Ureia / COVID-19 / Doenças Metabólicas Limite: Adult / Child / Humans Idioma: En Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Distúrbios Congênitos do Ciclo da Ureia / COVID-19 / Doenças Metabólicas Limite: Adult / Child / Humans Idioma: En Ano de publicação: 2023 Tipo de documento: Article