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Collapsing glomerulopathy in a child with LCHAD deficiency: a rare association.
- Source :
-
CEN case reports [CEN Case Rep] 2019 Aug; Vol. 8 (3), pp. 173-177. Date of Electronic Publication: 2019 Feb 12. - Publication Year :
- 2019
-
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.
- Subjects :
- Child, Preschool
Humans
Kidney Diseases pathology
Male
Cardiomyopathies complications
Kidney Diseases etiology
Kidney Glomerulus pathology
Lipid Metabolism, Inborn Errors complications
Mitochondrial Myopathies complications
Mitochondrial Trifunctional Protein deficiency
Nervous System Diseases complications
Rhabdomyolysis complications
Subjects
Details
- Language :
- English
- ISSN :
- 2192-4449
- Volume :
- 8
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- CEN case reports
- Publication Type :
- Report
- Accession number :
- 30747351
- Full Text :
- https://doi.org/10.1007/s13730-019-00387-3