Download PDF
Neuropediatrics 2022; 53(05): 381-384
DOI: 10.1055/s-0042-1750729
Short Communication

Therapeutic Management of COVID-19 in a Pediatric Patient with Neurodegenerative CLN2 Disease and ICV—Enzyme Replacement Therapy: A Case Report

Christoph Schwering 
1   Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Sofia Apostolidou
2   Division of Neonatology and Pediatric Intensive Care, Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Philipp Deindl
2   Division of Neonatology and Pediatric Intensive Care, Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Martin Christner
3   Institute of Medical Microbiology, Virology and Hygiene, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Johannes K.-M. Knobloch
3   Institute of Medical Microbiology, Virology and Hygiene, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Jochen Herrmann
4   Section of Pediatric Radiology, Department of Interventional and Diagnostic Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Robin Kobbe
5   Institute for Infection Research and Vaccine Development, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Angela Schulz
1   Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Dominique Singer
2   Division of Neonatology and Pediatric Intensive Care, Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Chinedu Ulrich Ebenebe
2   Division of Neonatology and Pediatric Intensive Care, Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
› Author Affiliations Funding None.
› Further Information
Also available at
    Permissions and Reprints

Abstract

The 12 years old male patient presented here suffers from neuronal ceroid lipofuscinoses 2 (CLN2) (MIM# 204500) and receives intracerebroventricular enzyme replacement therapy (ICV-ERT) every 14 days. After the emergence of the coronavirus disease 2019 (COVID-19) pandemic, routine care of children and adolescents with rare chronic diseases has become challenging. Although, in general, children do not develop severe COVID-19, when severe acute respiratory syndrome coronavirus 2 infection was detected by polymerase chain reaction-screening examination in our CLN2 patient before hospital admission for ICV-ERT, he was regarded to be at risk. Upon diagnosis, the patient developed respiratory deterioration symptoms and was admitted to our pediatric intensive care unit to receive oxygen, remdesivir, and steroids. As far as we know, this is the first CLN2 patient receiving intraventricular enzyme therapy with COVID-19 who required intensive care treatment and specific therapy.

Keywords

CLN2 disease - SARS-CoV-2 - respiratory symptom - COVID-19

Author Contributions

S.A., P.D., C.U.E., A.S., C.S., and D.S. contributed to clinical medical support and M.C., J.H., J.K-M.K., and R.K. contributed to diagnostic and therapeutic support.


Ethical Approval and Patient Consent

The parent has given written consent to the publication of the case.




Publication History

Received: 29 December 2021

Accepted: 17 March 2022

Article published online:
17 July 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Dong Y, Mo X, Hu Y. et al. Epidemiology of COVID-19 among children in China. Pediatrics 2020; 145 (06) e20200702
    CrossrefPubMedSearch in Google Scholar
  • 2 Götzinger F, Santiago-García B, Noguera-Julián A. et al; ptbnet COVID-19 Study Group. COVID-19 in children and adolescents in Europe: a multinational, multicentre cohort study. Lancet Child Adolesc Health 2020; 4 (09) 653-661
    CrossrefPubMedSearch in Google Scholar
  • 3 Kousi M, Lehesjoki AE, Mole SE. Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses. Hum Mutat 2012; 33 (01) 42-63
    CrossrefPubMedSearch in Google Scholar
  • 4 Mole SE, Cotman SL. Genetics of the neuronal ceroid lipofuscinoses (Batten disease). Biochim Biophys Acta 2015; 1852 (10 Pt B): 2237-2241
    CrossrefPubMedSearch in Google Scholar
  • 5 Schulz A, Ajayi T, Specchio N. et al; CLN2 Study Group. Study of intraventricular cerliponase alfa for CLN2 disease. N Engl J Med 2018; 378 (20) 1898-1907
    Search in Google Scholar
  • 6 Cohen-Pfeffer JL, Gururangan S, Lester T. et al. Intracerebroventricular delivery as a safe, long-term route of drug administration. Pediatr Neurol 2017; 67: 23-35
    CrossrefPubMedSearch in Google Scholar
  • 7 Slavc I, Cohen-Pfeffer JL, Gururangan S. et al. Best practices for the use of intracerebroventricular drug delivery devices. Mol Genet Metab 2018; 124 (03) 184-188
    CrossrefPubMedSearch in Google Scholar
  • 8 Kanne JP, Bai H, Bernheim A. et al. COVID-19 imaging: what we know now and what remains unknown. Radiology 2021; 299 (03) E262-E279
    CrossrefPubMedSearch in Google Scholar
  • 9 Méndez-Echevarría A, Pérez-Martínez A, Gonzalez Del ValleL. et al. Compassionate use of remdesivir in children with COVID-19. Eur J Pediatr 2021; 180 (04) 1317-1322
    CrossrefPubMedSearch in Google Scholar
  • 10 Goldman DL, Aldrich ML, Hagmann SHF. et al. Compassionate use of remdesivir in children with severe COVID-19. Pediatrics 2021; 147 (05) e2020047803
    CrossrefPubMedSearch in Google Scholar
  • 11 Eleftheriou I, Liaska M, Krepis P. et al. Sinus bradycardia in children treated with remdesivir for COVID-19. Pediatr Infect Dis J 2021; 40 (09) e356
    CrossrefPubMedSearch in Google Scholar
  • 12 Rietdorf K, Coode EE, Schulz A, Wibbeler E, Bootman MD, Ostergaard JR. Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity. Biochim Biophys Acta Mol Basis Dis 2020; 1866 (09) 165643
    CrossrefPubMedSearch in Google Scholar
  • 13 Moll-Khosrawi P. The CLN3 (Batten) Disease: a description of the clinical phenotype in 25 CLN3 patients with an identical genotype, with special focus on cardiological findings . Dissertation to obtain the degree of doctor of medicine; Medical Faculty University of Hamburg, 2017
    PubMedSearch in Google Scholar
  • 14 Boulad F, Kamboj M, Bouvier N, Mauguen A, Kung AL. COVID-19 in children with cancer in New York City. JAMA Oncol 2020; 6 (09) 1459-1460
    CrossrefPubMedSearch in Google Scholar
  • 15 Schwierzeck V. et al. First reported nosocomial outbreak of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a pediatric dialysis unit. Clin Infect Dis. 2021; 72 (02) 265-270
    PubMedSearch in Google Scholar