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Collapsing glomerulopathy in a child with LCHAD deficiency: a rare association

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Abstract

Metabolic disorders, although rare, can involve multiple organ systems and have a varied presentation. Renal involvement has been reported in several metabolic disorders in the pediatric age group. We report a rare metabolic disorder, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, in a child who developed steroid-resistant nephrotic syndrome at the age of 5 years. Renal biopsy showed features of collapsing glomerulopathy. The child had progressive chronic kidney disease. Alternative immunosuppressants including tacrolimus failed to show any clinical improvement. There have been no reports of children with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency developing steroid-resistant nephrotic syndrome and collapsing glomerulopathy. This case highlights the need to monitor renal function and proteinuria among this group of children.

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Authors and Affiliations

  1. Department of Paediatrics, Sheikh Khalifa Medical City, P O Box 51900, Abu Dhabi, United Arab Emirates

    Gurinder Kumar, Rajendran Nair, Omar Ahmed AlShkeili, Ahmed Abdulla Alabdouli, Adnan Mohamed Al Ali & Amal Mohamed Jasem AlTenaiji

  2. Department of Pathology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates

    Bassem Soliman Hendawy

Authors
  1. Gurinder Kumar
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  2. Rajendran Nair
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  3. Bassem Soliman Hendawy
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  4. Omar Ahmed AlShkeili
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  5. Ahmed Abdulla Alabdouli
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  6. Adnan Mohamed Al Ali
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  7. Amal Mohamed Jasem AlTenaiji
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Contributions

GK planned, conducted, and reported the work described in the article. RN was involved in conception and design of the case report. GK and RN clinically managed the child. BSH reviewed the histopathology slides of renal biopsy. OAA, AAA, AMAA drafted the article. AMJA critically reviewed the manuscript. AT will act as guarantor of the paper.

Corresponding author

Correspondence to Gurinder Kumar.

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All Authors declare that they have no conflict of interest.

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Kumar, G., Nair, R., Hendawy, B.S. et al. Collapsing glomerulopathy in a child with LCHAD deficiency: a rare association. CEN Case Rep 8, 173–177 (2019). https://doi.org/10.1007/s13730-019-00387-3

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  • DOI: https://doi.org/10.1007/s13730-019-00387-3

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