The prevalence of juvenile-onset gout has been increasing. Hereditary factors and
secondary diseases should be considered in these patients. Adipsic diabetes
insipidus (ADI) is characterized by arginine vasopressin (AVP) deficiency, which
results in hypotonic polyuria, and dysfunction of thirst osmoreceptors, which
results in failure to generate a thirst sensation in response to hypernatremia.
We herein report a case of a boy with gouty arthritis, refractory hyperuricemia,
prominent hypernatremia, a high creatinine concentration, and a history of
surgery for a hypothalamic hamartoma. The patient was diagnosed with central
diabetes insipidus after endocrine evaluation. Because he never had symptoms of
thirst, the final diagnosis was corrected to ADI. This is the first report of
gout due to chronic ADI in an adolescent. Volume contraction due to ADI might be
one cause of hyperuricemia and renal impairment in such patients. Moreover, AVP
deficiency might directly lead to low urate clearance due to the lack of
vasopressin receptor 1 stimulation. Lack of polydipsia and polyuria may delay
the diagnosis of ADI and lead to severe complications of a chronic hyperosmolar
status. Sufficient and effective establishment of normovolemia is critical for
these patients.