Development of Graves' Hyperthyroidism Caused an Adrenal Crisis in a Patient with Previously Unrecognized Non-classical 21-hydroxylase Deficiency

Abstract

A 75-year-old woman was found to be unconscious in hospital. She was febrile with a temperature of 38.4 . She had hypotension (blood pressure 80/40 mmHg) with serum Na 132 mEq/L and K 5.7 mEq/L (serum Na/K = 23.2), and serum cortisol 0.91 μg/dL, indicative of adrenal failure. She was admitted for the treatment of Graves' hyperthyroidism, and was found to be unconscious in hospital. We encountered a patient with unrecognized adrenocortical disease, in whom development of Graves' hyperthyroidism caused an adrenal crisis. The ACTH stimulation test indicated that she had 21-hydroxylase deficiency (21OHD); after ACTH stimulation, 17-OH-progesterone increased from 0.6 to 10.4 ng/mL (17.3 times), and 17-OHprogesterone/cortisol from 0.0049 to 0.045 (9.2 times). She did not have clinical signs of classical 21OHD. She had non-classical 21OHD (NC21OHD). Development of Graves' hyperthyroidism caused an adrenal crisis in a patient with previously unrecognized NC21OHD.A patient with unrecognized adrenocortical disease developed Graves' hyperthyroidism, which induced an adrenal crisis. She had NC21OHD.

Association of Vitamin D Repletion with Normalization of Elevated Serum 17-OH-Progesterone

“…One can easily appreciate that the same intervention with the same percentage elevation of 17-OHP in a female patient with amenorrhea, acne, hirsutism, alopecia, or infertility might have been very clinically useful in addressing these issues. Furthermore, NCAH patients under extreme stress may experience adrenal crisis [12], possibly justifying its treatment even in asymptomatic patients.…”

Thyrotoxic Periodic Paralysis with Graves' Disease Leading to the Discovery of a Hidden Nonclassic 11β Hydroxylase Deficiency

“…Therefore, in the present case, the symptoms of occult nonclassic 11β OHD, including hypertension and hypokalemia, seem to have aggravated the symptoms of TPP. Takasu et al reported a case of Grave's disease caused an adrenal crisis in a patient with previously unrecognized nonclassic 21 hydroxylase deficiency (10). Increased levels of thyroid hormone may play an etiologic role in the development and aggravation of symptoms if unrecognized nonclassic congenital adrenal hyperplasia is present.…”

Roux-en-Y gastric bypass in the treatment of non-classic congenital adrenal hyperplasia due to 11-hydroxylase deficiency

“…It has been shown that elevated thyroid hormone levels in the setting of Graves’ disease can unmask previously undiagnosed NCAH 24 25. Elevated thyroid hormone can increase the turnover of cortisol and consequently an occult NCAH may become symptomatic, or even lead to adrenal crisis.…”

“…Elevated thyroid hormone can increase the turnover of cortisol and consequently an occult NCAH may become symptomatic, or even lead to adrenal crisis. Takasu et al 24 have reported Graves’ disease causing adrenal crisis in a 75-year-old patient with previously unrecognised non-classical 21-hydroxylase deficiency. Also Kim et al 25 reported a case of thyrotoxic periodic paralysis with Graves’ disease leading to a late diagnosis of non-classic 11β-hydroxylase deficiency.…”