True Pituitary Addison's Disease—a Pituitary Unitropic Deficiency (Fifteen-Year Follow-Up)*

Steinberg, Arthur G.; Shechter, Fred R.; Segal, Helene

doi:10.1210/jcem-14-12-1519

Cited by 94 publications

(31 citation statements)

References 7 publications

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“…Isolated ACTH deficiency is a rare disorder and causes adrenal insufficiency, which was first reported by Steinberg et a1. in 1954 [1]. Most cases of isolated ACTH deficiency reported are caused by ACTH deficit at the level of the pituitary gland [2], but corticotropin-releasing hormone (CRH) deficiency in the hypothalamus could also cause isolated ACTH deficiency [3].…”

Section: Patientsmentioning

confidence: 99%

A Case of Adrenal Insufficiency Due to Acquired Hypothalamic CRH Deficiency.

et al. 1997

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Abstract.A 40-year-old woman with adrenal insufficiency was clinically diagnosed and examined with human corticotropin releasing hormone (CRH). This patient with secondary hypo-adrenalism has shown a normal serum cortisol response to exogenous ACTH administration and has been examined with CRH, lysine-vasopressin (LVP) and insulin tolerance test (ITT), respectively. Success in secreting ACTH in response to both CRH and LVP tests, but not ITT, suggests that this disorder was possibly due to a hypothalamic CRH deficiency rather than pituitary corticotroph dysfunction.A combination of the CRH test and ITT has come to play an increasingly significant role in the diagnosis and differential diagnosis of isolated ACTH deficiency syndrome.

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Section: Patientsmentioning

confidence: 99%

A Case of Adrenal Insufficiency Due to Acquired Hypothalamic CRH Deficiency.

et al. 1997

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“…(ACTH) deficiency was first described by Steinberg and colleagues [1]. Accumulated evidence indicates clinical characteristics due to heterogeneous causes [2] and frequent association with other autoimmune endocrine disorders [3].…”

Section: Isolated Adrenocorticotrophinmentioning

confidence: 99%

Isolated Adrenocorticotrophin Deficiency Associated with Anti-Pituitary Antibodies, Pituitary Cyst, Sphenoidal Cyst and Pineal Tumor.

et al. 1994

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Abstract. This paper reports a rare case of isolated ACTH deficiency associated with anti-pituitary antibodies, pituitary cyst, sphenoidal cyst and pineal tumor. A 68-year-old man consulted our clinic for general fatigue. Laboratory data showed low plasma adrenocorticotrophin (ACTH) and cortisol levels with blunted responses to insulin-induced hypoglycemia and corticotrophin releasing factor (CRF). Urinary 17-OHCS was low but responded to ACTH-Z administration. No other pituitary functions were impaired. Antibodies to the cytoplasm of rat pituitary and the surface of GH3 cells were detected in the serum. The magnetic resonance imaging (MRI) showed a high signal intensity mass in the anterior pituitary and in the sphenoidal sinus in both T1 and T2 weighted images as well as a low signal intensity mass in a Ti weighted image of the pineal region. Transsphenoidal surgery was performed to resect the mass in the sphenoid sinus and in the pituitary. Pathological studies showed a benign cyst in the sphenoid sinus, and fibrous degeneration and decreased basophils in the pituitary. No infiltrative mononuclear cells were detected in the pituitary. Immunohistochemical studies revealed a decrease in the number of ACTH-producing cells in the pituitary. The patient was well maintained by glucocorticoid replacement without any growth of a possibly benign pineal tumor.

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“…These results indicate that there is marked hyponatremia and that in the presence of hypoosmolality the sustained secretion of ADH is the key factor in causing the impaired water excretion and hyponatremia in isolated ACTH deficiency. (Endocrinol Japon 38: 325-330, 1991) ISOLATED adrenocorticotropic hormone (ACTH) deficinecy is a rare state of hypopituitarism [1]. This is only a deficit of ACTH without any abnormality in other pituitary hormones, and the cause of isolated ACTH deficiency has not been determined.…”

Section: Introductionmentioning

confidence: 99%

Role of Antidiuretic Hormone in Hyponatremia in Patients with Isolated Adrenocorticotropic Hormone Deficiency.

Ishikawa¹,

Fujisawa²,

Tsuboi³

et al. 1991

Endocrinol Japon

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Abstract.We found symptomatic hyponatremia in four elderly patients in which serum sodium (Na) levels ranged from 101 to 122 mEq/l. All 4 patients had low levels of plasma adrenocorticotropic hormone (ACTH), serum cortisol, and urinary excretion of 17-OHCS, and poor responses of ACTH to exogenous insulin and antidiuretic hormone (ADH). Other pituitary hormones were all normal. They were therefore diagnosed as having isolated ACTH deficiency. Plasma ADH was relatively high despite hypoosmolality which was associated with the hyponatremia. Water loading test revealed impaired water excretion and poor suppression of plasma ADH. Replacement with 20-30 mg hydrocortisone completely restored the serum Na level and restored the plasma ADH level to the normal range in all 4 patients. Other factors such as decreased glomerular filtration, enhanced urinary Na loss and decreased Na intake were also included. These results indicate that there is marked hyponatremia and that in the presence of hypoosmolality the sustained secretion of ADH is the key factor in causing the impaired water excretion and hyponatremia in isolated ACTH deficiency.

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True Pituitary Addison's Disease—a Pituitary Unitropic Deficiency (Fifteen-Year Follow-Up)*

Cited by 94 publications

References 7 publications

A Case of Adrenal Insufficiency Due to Acquired Hypothalamic CRH Deficiency.

A Case of Adrenal Insufficiency Due to Acquired Hypothalamic CRH Deficiency.

Isolated Adrenocorticotrophin Deficiency Associated with Anti-Pituitary Antibodies, Pituitary Cyst, Sphenoidal Cyst and Pineal Tumor.

Role of Antidiuretic Hormone in Hyponatremia in Patients with Isolated Adrenocorticotropic Hormone Deficiency.

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