Adrenocorticotropic hormone deficiency

A congenital autosomal recessive form of adrenal insufficiency that is characterized by very low plasma ACTH and CORTISOL levels with no significant response to CORTICOTROPIN-RELEASING HORMONE. Affected neonatal patients may present with OBSTRUCTIVE JAUNDICE, severe HYPOGLYCEMIA, and SEIZURES. Mutations in the TBX19 gene have been identified. OMIM: 201400

Networked: 84 relevant articles (6 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Adrenal Insufficiency (Adrenal Gland Hypofunction)
2.	Hypopituitarism (Sheehan's Syndrome)
3.	Thyroiditis
4.	Proteinuria
5.	Fatigue

Experts

1.	Ohara, Nobumasa: 4 articles (07/2021 - 03/2019)
2.	Yoneoka, Yuichiro: 4 articles (07/2021 - 03/2019)
3.	Chemaitilly, Wassim: 3 articles (01/2021 - 02/2015)
4.	Merchant, Thomas E: 3 articles (12/2020 - 03/2008)
5.	Fukuda, Izumi: 2 articles (08/2022 - 05/2021)
6.	Ono, Mayo: 2 articles (08/2022 - 05/2021)
7.	Sugihara, Hitoshi: 2 articles (08/2022 - 05/2021)
8.	Koda, Ryo: 2 articles (07/2021 - 04/2020)
9.	Takada, Toshinori: 2 articles (07/2021 - 03/2019)
10.	van Iersel, Laura: 2 articles (01/2021 - 12/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adrenocorticotropic hormone deficiency:

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 09/29/2020 - "Isolated adrenocorticotropic hormone deficiency was diagnosed, and corticosteroid replacement therapy was administered, resulting in an improvement of his symptoms. " 10/18/2021 - "All patients experiencing hypopituitarism presented with adrenocorticotropic hormone deficiency, causing secondary adrenal insufficiency, which required permanent corticosteroid replacement therapy. " 09/24/2022 - "Isolated adrenocorticotropic hormone deficiency was diagnosed, and corticosteroid replacement therapy was administered, leading to a significant improvement of his symptoms while ACTH level maintaining low level. " 01/01/2007 - "After corticosteroid and sodium supplementation improved the status of the patient, endocrinological examinations revealed that he suffered from isolated adrenocorticotropic hormone deficiency followed by transient thyroiditis that induced an adrenal crisis. " 03/26/2019 - "Four months after nivolumab therapy was discontinued, she developed isolated adrenocorticotropic hormone deficiency; corticosteroid replacement therapy relieved her secondary adrenal insufficiency symptoms, such as anorexia and fatigue. "
2.	Hydrocortisone (Cortisol)FDA LinkGeneric 01/01/2016 - "Treatable Bedridden Elderly -Recovery from Flexion Contracture after Cortisol Replacement in a Patient with Isolated Adrenocorticotropic Hormone Deficiency." 03/05/2014 - "Hydrocortisone replacement therapy for isolated adrenocorticotropic hormone deficiency effectively avoids the unnecessary cessation of chemotherapy." 04/01/2010 - "Hydrocortisone therapy (30 mg/day) for isolated adrenocorticotropic hormone deficiency resulted in marked improvement of adrenal and thyroid functions, and amelioration of proteinuria (from 2.8 to 1.0 g/day) over a two-month period. " 09/15/2017 - "Endocrine test results supported a diagnosis of isolated adrenocorticotropic hormone deficiency due to hypophysitis, and replacement therapy using hydrocortisone has been successful. " 01/01/2001 - "Firstly, the GH-mediated increase in cortisol metabolism (mediated via reduced E to F conversion) may precipitate adrenal insufficiency in hypopituitary patients with partial adrenocorticotropic hormone deficiency commencing GH therapy. "
3.	Growth Hormone (Somatotropin)IBA 08/01/1978 - "Endocrine studies revealed growth hormone and gonadotropic deficiency and possible adrenocorticotropic hormone deficiency and possible adrenocorticotropic hormone deficiency. " 01/01/2011 - "Is adrenocorticotropic hormone deficiency really rare in patients with idiopathic growth hormone deficiency and normal thyroid function tests?" 06/03/2008 - "9 of 61 boxers (15%) had growth hormone (GH) deficiency and 5 of 61 boxers (8%) had adrenocorticotropic hormone deficiency. " 07/01/1995 - "Isolated adrenocorticotropic hormone deficiency associated with growth hormone deficiency and empty sella." 02/01/2009 - "The growth hormone (GH) axis is the most vulnerable to radiation damage, and isolated GH deficiency can occur after doses as low as 18 Gy. Furthermore, the frequency of GH deficiency can reach 50-100% within 3-5 years of cranial irradiation with doses of 30-50 Gy. TSH and adrenocorticotropic hormone deficiency occur in 3-6% of patients after conventional irradiation (30-50 Gy). "
4.	NivolumabIBA 02/19/2019 - "Late-onset isolated adrenocorticotropic hormone deficiency caused by nivolumab: a case report." 01/01/2019 - "Thyroid disorder and isolated adrenocorticotropic hormone deficiency have frequently been reported as nivolumab-induced immune-related adverse events. " 10/01/2018 - "Nivolumab-induced isolated adrenocorticotropic hormone deficiency may not present with typical symptoms, and can occur even after discontinuing nivolumab. " 03/26/2019 - "We report a rare case of patient with isolated adrenocorticotropic hormone deficiency that occurred after nivolumab therapy. " 04/01/2017 - "Isolated adrenocorticotropic hormone deficiency associated with nivolumab therapy."
5.	Adrenocorticotropic Hormone (ACTH)FDA Link 09/01/1980 - "[A case report of isolated adrenocorticotropic hormone (ACTH) deficiency: a statistical analysis of 32 cases of isolated adrenocorticotropic hormone deficiency reported in Japan (author's transl)]." 03/05/2014 - "Isolated adrenocorticotropic hormone deficiency is an endocrinological disorder characterized by loss of adrenocorticotropic hormone and resultant adrenal insufficiency. " 09/24/2022 - "Isolated adrenocorticotropic hormone deficiency was diagnosed, and corticosteroid replacement therapy was administered, leading to a significant improvement of his symptoms while ACTH level maintaining low level. " 01/01/2020 - "Isolated adrenocorticotropic hormone deficiency (IAD) is a cause of adrenal insufficiency (AI), which shows impaired secretion of adrenocorticotropic hormone (ACTH) with the preserved secretion of other anterior pituitary gland hormones. " 12/12/2019 - "BACKGROUND Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder characterized by central adrenal insufficiency (AI) but normal secretion of pituitary hormones other than adrenocorticotropic hormone. "
6.	Thyrotropin (Thyroid-Stimulating Hormone)FDA Link 01/01/2020 - "All 15 patients with HH had associated thyroid-stimulating hormone and adrenocorticotropic hormone deficiency except for 3 girls with partial HH. " 10/01/1992 - "None of the girls with adrenocorticotropic hormone deficiency, either originally or subsequently, had spontaneous puberty, but 4 of 12 girls with thyrotropin deficiency, either originally or subsequently, had complete puberty. " 03/01/2008 - "The cumulative incidence of GHD, thyroid-stimulating hormone (TSH) deficiency, adrenocorticotropic hormone deficiency, and primary hypothyroidism at 4 years from diagnosis was 93% +/- 4%, 23% +/- 8%, 38% +/- 6%, and 65% +/- 7%, respectively. " 05/01/2009 - "In two of the patients, hormonal analyses were performed, which showed anterior pituitary insufficiency (thyroid-stimulating hormone and adrenocorticotropic hormone deficiency), symptoms which improved with hormone replacement therapy. " 12/01/2006 - "To date, 16 distinct human Prophet of Pit-1 (Prop1) gene mutations have been identified in patients with CPHD, inducing a phenotype involving GH, follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin and thyroid-stimulating hormone (TSH), and rarely adrenocorticotropic hormone, deficiency. "
7.	Growth Hormone-Releasing Hormone (Somatotropin Releasing Hormone)IBA 07/20/2009 - "Improved response of growth hormone to growth hormone-releasing hormone and reversible chronic thyroiditis after hydrocortisone replacement in isolated adrenocorticotropic hormone deficiency."
8.	SodiumIBA 01/01/2007 - "After corticosteroid and sodium supplementation improved the status of the patient, endocrinological examinations revealed that he suffered from isolated adrenocorticotropic hormone deficiency followed by transient thyroiditis that induced an adrenal crisis. "
9.	pembrolizumabIBA 01/01/2020 - "From multiple tolerance tests, we diagnosed isolated adrenocorticotropic hormone deficiency caused by pembrolizumab. " 01/01/2020 - "A Case of Isolated Adrenocorticotropic Hormone Deficiency Caused by Pembrolizumab." 01/01/2020 - "We report here a case of pembrolizumab-induced isolated adrenocorticotropic hormone deficiency in a patient with metastatic colon cancer. " 09/12/2023 - "Metachronous immune-related adverse events involving type 1 diabetes and isolated adrenocorticotropic hormone deficiency associated with pembrolizumab monotherapy for metastatic head and neck cancer: a case report." 11/01/2021 - "We report a case of secondary adrenocortical insufficiency due to isolated adrenocorticotropic hormone deficiency with empty sella syndrome after pembrolizumab treatment in a patient with metastatic renal pelvic cancer. "
10.	Immune Checkpoint InhibitorsIBA 07/06/2023 - "Isolated adrenocorticotropic hormone deficiency following immune checkpoint inhibitors treatment often occurs in polyglandular endocrinopathies." 01/01/2023 - "Paraneoplastic isolated adrenocorticotropic hormone deficiency revealed after immune checkpoint inhibitors therapy: new insights into anti-corticotroph antibody." 09/29/2020 - "We report the first case of immune checkpoint inhibitor-induced simultaneous diabetic ketoacidosis and isolated adrenocorticotropic hormone deficiency following combination treatment with immune checkpoint inhibitors. " 07/06/2023 - "With the increasing application of immune checkpoint inhibitors (ICI) in cancer therapy, the occurrence of isolated adrenocorticotropic hormone deficiency (IAD), as an adverse effect, is also on the rise. " 06/05/2021 - "Isolated adrenocorticotropic hormone deficiency and hypophysitis have been reported in patients treated with nivolumab, another programmed cell death protein 1 inhibitor. "

Therapies and Procedures

1.	Therapeutics 01/01/2023 - "Paraneoplastic isolated adrenocorticotropic hormone deficiency revealed after immune checkpoint inhibitors therapy: new insights into anti-corticotroph antibody." 09/29/2020 - "Isolated adrenocorticotropic hormone deficiency was diagnosed, and corticosteroid replacement therapy was administered, resulting in an improvement of his symptoms. " 03/26/2019 - "We report a rare case of patient with isolated adrenocorticotropic hormone deficiency that occurred after nivolumab therapy. " 04/01/2017 - "Isolated adrenocorticotropic hormone deficiency associated with nivolumab therapy." 03/05/2014 - "Hydrocortisone replacement therapy for isolated adrenocorticotropic hormone deficiency effectively avoids the unnecessary cessation of chemotherapy."
2.	Hormone Replacement Therapy (Therapy, Hormone Replacement) 05/01/2009 - "In two of the patients, hormonal analyses were performed, which showed anterior pituitary insufficiency (thyroid-stimulating hormone and adrenocorticotropic hormone deficiency), symptoms which improved with hormone replacement therapy. "
3.	Renal Dialysis (Hemodialysis) 04/28/2020 - "Only a few cases of isolated adrenocorticotropic hormone deficiency (IAD) among hemodialysis patients have been reported. " 04/28/2020 - "Isolated Adrenocorticotropic Hormone Deficiency and Primary Hypothyroidism in a Patient Undergoing Long-Term Hemodialysis: A Case Report and Literature Review." 08/01/2003 - "Isolated adrenocorticotropic hormone deficiency presenting with hypercalcemia in a patient on long-term hemodialysis."
4.	Cranial Irradiation 02/01/2009 - "The growth hormone (GH) axis is the most vulnerable to radiation damage, and isolated GH deficiency can occur after doses as low as 18 Gy. Furthermore, the frequency of GH deficiency can reach 50-100% within 3-5 years of cranial irradiation with doses of 30-50 Gy. TSH and adrenocorticotropic hormone deficiency occur in 3-6% of patients after conventional irradiation (30-50 Gy). "
5.	Urinary Diversion (Ileal Conduit) 07/15/2021 - "Isolated Adrenocorticotropic Hormone Deficiency Associated with Severe Hyperkalemia During Pembrolizumab Therapy in a Patient with Ureteral Cancer and an Ileal Conduit: A Case Report and Literature Review."