CEN case reports · 2019
Collapsing glomerulopathy in a child with LCHAD deficiency: a rare association.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Kumar Gurinder, Nair Rajendran, Hendawy Bassem Soliman, AlShkeili Omar Ahmed, Alabdouli Ahmed Abdulla, Ali Adnan Mohamed Al, AlTenaiji Amal Mohamed Jasem
Аннотация
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.