Background: Non-autoimmune hypothyroidism has been reported in children with congenital nephrotic syndrome. The hypothyroid state was attributed to massive prolonged thyroid hormone loss. However, this endocrine abnormality has not been reported in steroid-resistant nephrotic syndrome (SRNS) despite similar long-standing proteinuria.
Method: We describe all the patients with SRNS in our clinic's follow-up who developed non-autoimmune hypothyroidism.
Results: Five children aged 3-11 years at diagnosis of SRNS and followed for 5-42 months developed hypothyroidism (depressed free thyroxin and elevated thyrotropin levels) without evidence of autoimmune thyroiditis. The diagnosis of hypothyroidism was not temporarily related to disease duration or renal function. The disease was resistant to all therapies, renal function deteriorated in all the patients within 1.5-14.5 years from diagnosis. Despite thyroxine treatment and a decline in renal function, thyroid hormone level normalized only after reaching end stage renal disease (ESRD) and hemodialysis start. Nephrotic syndrome recurrence after kidney transplantation (in three patients with focal segmental glomerulosclerosis) was not accompanied by recurrent hypothyroidism.
Conclusion: It is our impression that non-autoimmune hypothyroidism is a potential significant complication of SRNS, and should be actively sought for especially in cases with renal function deterioration. Hypothyroidism usually resolved when these patients reach ESRD. The incidence and pathogenesis of this condition require further study.