Prevalence of Maturity-onset Isolated ACTH Deficiency (IAD) in 2005: Japanese Cohort Studies

Abstract

Abstract. The prevalence of isolated ACTH deficiency (IAD) in Japan remains to be determined. Both authors (T.Y. & K.K.) had unique opportunities to see all patients with maturity-onset IAD in particular areas. T.Y. regularly visited Tokunoshima Island in Kagoshima Prefecture (population of about 28,500) which has two acute-care hospitals. He has taken care of endocrine-metabolism cases in one hospital and kept in touch with an endocrine-oriented physician in another. K.K. has provided glucocorticoid supplementation for all patients with maturity-onset IAD as the patients' own physician in a hospital which provided medical care for the Chuetsu district in Niigata Prefecture with 527,407 inhabitants in 2005. Four male patients (average age at onset, 70.0 years; range, 67-75 years) were identified in Tokunoshima over the 10-year period and 20 patients (15 males and 5 females; average age at onset, 63.9 years; range, 49-77 years) were cared for in the Chuetsu district in 2005. The estimated prevalence of IAD from the numbers of IAD patients and of inhabitants in the periods cited from the national population survey was 7.3 per 100,000 (an average in 10-year period) in Tokunoshima and 3.8 per 100,000 in the Chuetsu district in 2005. Maturity-onset IAD in Japan thus is not very rare in the elderly.

Catecholamine-resistant shock and hypoglycemic coma after cardiotomy in a patient with unexpected isolated ACTH deficiency

“…The resulting decline in production and secretion of ACTH causes chronic secondary adrenocortical insufficiency. Although considered an extremely rare disease, the number of diagnosed cases is increasing because knowledge of the disease and detailed endocrinology testing have become more widespread [3]. In particular, incidence is relatively high among men at 40-60 years of age.…”

A patient with isolated adrenocorticotropin deficiency presenting with repetitive oesophageal ulcer and stenosis

“…Over 300 cases of IAD were reported between 1969 and 1994 in Japan, and an increasing number of cases have been reported since then 3. The prevalence of diagnosed cases is 1.91/1 000 000 in the Miyazaki prefecture, 7.3/1 000 000 in Tokushima area and 3.8/1 000 000 in Chuetsu district in Japan 5 6. There seems to be a substantial number of undiagnosed patients because clinical diagnosis is made when the patient becomes severely ill due to physical stress 7 8.…”

Ping-pong champion with adrenal insufficiency

“…While it has been believed that isolated ACTH deficiency is rare in old people, a recent study from Japan showed this is not necessarily true 5. The management of corticosteroid deficiency during exercise remains controversial partly because patients with adrenal insufficiency are heterogeneous, and it is difficult to perform statistical analyses between groups using any end point such as endurance and performance.…”