Combined Pituitary Hormone Deficiency

A hereditary autosomal recessive form of hypopituitarism that is characterized by growth retardation, HYPOTHYROIDISM, reduced levels of CORTISOL, and absent or DELAYED PUBERTY due to low levels of LUTEINIZING HORMONE or FOLLICLE-STIMULATING HORMONE . Rarely, INTELLECTUAL DISABILITY, neck abnormalities, and OPTIC NERVE defects may also occur. Mutations in the PROP1 gene have been identified in some cases. OMIM: 262600

Also Known As:

Networked: 590 relevant articles (15 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hypothyroidism
2.	Adrenal Insufficiency (Adrenal Gland Hypofunction)
3.	Acromegaly
4.	Diabetes Insipidus
5.	Hypogonadism (Hypergonadotropic Hypogonadism)

Experts

1.	Dattani, Mehul T: 9 articles (01/2020 - 01/2004)
2.	Brue, Thierry: 8 articles (11/2021 - 08/2005)
3.	Saveanu, Alexandru: 6 articles (11/2019 - 08/2005)
4.	Reynaud, Rachel: 5 articles (11/2019 - 08/2005)
5.	Enjalbert, Alain: 4 articles (06/2016 - 09/2006)
6.	Ogata, Tsutomu: 4 articles (01/2013 - 02/2010)
7.	Rhodes, Simon J: 4 articles (07/2011 - 01/2002)
8.	Mehta, Ameeta: 4 articles (09/2009 - 05/2005)
9.	Arnhold, Ivo Jorge Prado: 3 articles (03/2022 - 01/2007)
10.	Sertedaki, Amalia: 3 articles (01/2022 - 08/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Combined Pituitary Hormone Deficiency:

1.	Hormones (Hormone)IBA 12/01/2001 - "The visual acuity postoperatively improved while she needed hormone replacement due to panhypopituitarism. " 12/01/2023 - "A hormone profile showed abnormal anterior pituitary assay suggestive of panhypopituitarism. " 09/01/2023 - "Combined pituitary hormone deficiency (CPHD) is a rare disorder caused by a complete absence of the anterior pituitary gland hormones. " 01/01/2021 - "This paper highlights some of the most common and novel genetic anomalies concerning growth hormones, which are responsible for various genetic defects in isolated growth and combined pituitary hormone deficiency disease." 08/01/2020 - "These patients suffer from pituitary hormone deficits ranging from IGHD to panhypopituitarism. "
2.	Growth Hormone (Somatotropin)IBA 01/01/2022 - "Growth Hormone Is Beneficial for Induction of Spermatogenesis in Adult Patients With Congenital Combined Pituitary Hormone Deficiency." 04/01/2019 - "The objective of the study is the functional characterization of a novel POU1F1 c.605delC mutation in combined pituitary hormone deficiency (CPHD) and to report the clinical and genetic details of 160 growth hormone deficiency patients. " 01/01/2020 - "A Finnish family of 2 siblings with panhypopituitarism, absent anterior pituitary, and mild craniofacial dysmorphism, and a Pakistani family with a proband with growth hormone deficiency, anterior pituitary hypoplasia, and developmental delay. " 03/01/2017 - "Growth without growth hormone in combined pituitary hormone deficiency caused by pituitary stalk interruption syndrome." 01/01/2016 - "Effect of Growth Hormone on Endometrial Thickness and Fertility Outcome in the Treatment of Women with Panhypopituitarism: A Case Report."
3.	Thyroxine (Levothyroxine)FDA LinkGeneric 01/01/2017 - "Endocrinology blood tests showed hypocortisolemia, hyperprolactinemia and low levels of free thyroxine (fT4), suggesting secondary combined pituitary hormone deficiency. " 09/01/2009 - "Here we describe the case of a patient with panhypopituitarism due to a craniopharyngioma, who developed thyrotoxic periodic paralysis due to excessive L-thyroxine replacement. " 03/01/1991 - "Among the 144 men, 2 had markedly abnormal values; L-thyroxine treatment had been prescribed for both, one because of primary hypothyroidism (concentrations at screening were high for TSH and low for free T4) and one because of panhypopituitarism (low free T4 at screening). " 12/01/2010 - "Panhypopituitarism was adequately treated with substitution of hydrocortisone, thyroxine and transdermal oestrogen which resulted in haemoglobin increase to nearly normal levels and symptoms improved immediately. " 08/01/1989 - "Replacement with cortisone and levothyroxine resulted in an improvement in the mental symptoms in two patients with panhypopituitarism. "
4.	SteroidsIBA 01/01/2019 - "Subjects with panhypopituitarism, chromosomal pathology, chronic steroids, or stimulant use were excluded. " 08/01/1997 - "Panhypopituitarism is characterized by the absence of secretion of adrenal and gonadal steroids and thus the production of their metabolites. " 07/01/2021 - "Imaging studies and laboratory data showed swelling of the pituitary gland and panhypopituitarism, which dramatically improved following steroid therapy. " 02/01/2015 - "Sex steroids, precursors, and metabolite deficiencies in men with isolated hypogonadotropic hypogonadism and panhypopituitarism: a GCMS-based comparative study." 01/01/2014 - "Eight years after an episode of multiple IgG4-related disease, a pituitary mass with panhypopituitarism and a visual disturbance developed in a 70-year-old man under low-dose steroid therapy. "
5.	Hydrocortisone (Cortisol)FDA LinkGeneric 12/01/2014 - "Cortisol rhythmicity and treatment of other possible concomitant conditions often associated (e.g., autoimmune disorders and panhypopituitarism) are essential to improve outcome of AI. " 01/01/2008 - "Design Eleven patients with panhypopituitarism taking 20-30 mg/day of hydrocortisone, but on no GH replacement were switched to 10-15 mg of hydrocortisone daily. " 11/01/1993 - "Physiological cortisol supplementation (20 mg 3 hr prior to test, and 1 mg/hr during test) increased basal K excretion (from 10.6 +/- 1.8 to 19.2 +/- 1.9 mmol/5 hr, P < 0.01) and KCl stimulated kaliuresis (from 47.9 +/- 6.1 to 54.8 +/- 4.7 mmol/5 hr, P = 0.06) to normal levels in panhypopituitarism. " 01/01/2017 - "She was diagnosed with panhypopituitarism and started on cortisol replacement therapy. " 10/01/2020 - "The tumor decreased in size after administration of hydrocortisone for panhypopituitarism. "
6.	GonadotropinsIBA 01/01/2022 - "Gonadotropins are effective in inducing spermatogenesis in patients with congenital combined pituitary hormone deficiency (CCPHD). " 09/01/2015 - "Recovery of HPA Axis Function After Successful Gonadotropin-Induced Pregnancy and Delivery in a Woman With Panhypopituitarism: Case Report and Review." 11/12/2004 - "The present finding will provide new insights into the development of pituitary cell lineage and combined pituitary hormone deficiency (CPHD), since why the defect of Prop-1 causes CPHD including gonadotropins (FSH and LH) has yet to be clarified." 09/01/2001 - "Both showed combined pituitary hormone deficiency of GH, TSH, PRL, and gonadotropins, as is typical for PROP-1 deficiency. " 06/01/2000 - "Isolated gonadotropin deficiency, in addition to central DI, preceded panhypopituitarism. "
7.	Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric 09/09/2020 - "Oral desmopressin led to initial symptoms resolution but, in the following months, an anterior panhypopituitarism developed, in spite of appropriate treatment and, by that time, the brain MRI also revealed lesion growth, which prompted a biopsy recommendation. " 05/01/2020 - "Severe Symptomatic Hypernatremia in a Patient with Panhypopituitarism after Short-Term Discontinuation of Desmopressin." 12/06/2019 - "After immunochemotherapy, glucocorticoids administration and desmopressin acetate replacement therapy, the symptoms of fever and panhypopituitarism improved, and all the lesions reduced in size. " 09/07/2023 - "His panhypopituitarism was managed with hydrocortisone, levothyroxine and desmopressin." 02/17/2021 - "Further endocrinological investigations confirmed panhypopituitarism and she commenced desmopressin, hydrocortisone and thyroxine. "
8.	GlucocorticoidsIBA 03/01/2005 - "Glucocorticoid replacement in panhypopituitarism complicated by myelinolysis." 10/01/1996 - "We describe a middle-aged profoundly hypogonadal man with panhypopituitarism since infancy who was treated only with glucocorticoid and thyroid replacement. " 03/01/2017 - "Here, we describe a case we examined from the primary diagnosis to final discharge, showing the serial progression of lymphocytic infundibuloneurohypophysitis (LINH) to panhypopituitarism with extrapituitary inflammatory invasion in a short period, and responding favorably to high-dose glucocorticoid treatment. " 03/01/2005 - "We report this case to illustrate the fact that glucocorticoid substitution can lead to rapid rise in serum sodium and myelinolysis in panhypopituitarism. " 03/01/2005 - "To report a case of glucocorticoid substitution in panhypopituitarism that can lead to uncontrolled rise in serum sodium and myelinolysis. "
9.	CortisoneFDA Link 05/01/1952 - "The variability of the lowered glomerular filtration rate in Addison's disease and panhypopituitarism, and the effect of cortisone thereon." 01/01/1954 - "The use of cortisone in the treatment of the panhypopituitarism due to postpartum necrosis of the pituitary (Sheehan's syndrome)." 08/01/1989 - "Replacement with cortisone and levothyroxine resulted in an improvement in the mental symptoms in two patients with panhypopituitarism. " 05/01/1952 - "The diuretic response to ingested water in Addison's disease and panhypopituitarism and the effect of cortisone thereon." 02/01/1951 - "Observations during ACTH and cortisone administration to a patient with longstanding panhypopituitarism and rheumatoid arthritis."
10.	Methotrexate (Mexate)FDA LinkGeneric 10/09/2013 - "R-CHOP and high-dose methotrexate therapy followed by autologous hematopoietic stem cell transplantation was quite effective, and panhypopituitarism and adrenal insufficiency improved to almost normal values after successful treatment of the lymphoma with chemotherapy." 04/01/2022 - "This report presents an unusual case of panhypopituitarism in a 32-year-old previously healthy male patient due to isolated hypothalamo-pituitary sarcoidosis confirmed histologically, refractory to pulse-dose glucocorticoids and then successfully treated by methotrexate. " 07/01/2021 - "Our case and the pertinent literature demonstrated that IVLBCL with pituitary involvement predominantly occurred in women at a mean age of 64 years, and most of them showed panhypopituitarism that was reversible after standard therapy of rituximab-containing chemotherapy with intrathecal methotrexate. "

Therapies and Procedures

1.	Therapeutics 02/01/1999 - "Long-term combined hCG and FSH therapy is effective in inducing puberty, increasing testicular volume, and stimulating spermatogenesis in adolescent males with isolated HH and panhypopituitarism-associated HH." 10/01/2021 - "This case report emphasizes the importance of careful adjustment of hormonal replacement therapy in managing pregnant women with panhypopituitarism." 10/01/2021 - "The authors describe the case of a woman with congenital panhypopituitarism who had a successful pregnancy after ovulation induction and optimization of hormonal replacement therapy. " 01/01/2021 - "He was on hormonal replacement therapy for panhypopituitarism that complicated the surgery. " 01/01/2021 - "She was started on antifungal and hormonal replacement therapy for panhypopituitarism. "
2.	Drug Therapy (Chemotherapy) 08/01/1993 - "As far as we know this is the first report of panhypopituitarism caused by a hypophyseal NHL in the hypophysis and successfully treated by intravenous chemotherapy." 09/09/2020 - "After chemotherapy and radiotherapy, there was complete disappearance of the pituitary lesion, but the panhypopituitarism persisted. " 09/01/2003 - "Although infundibular thickening resolved and diabetes insipidus improved after chemotherapy, panhypopituitarism persisted." 01/01/2020 - "There was a significant difference in univariate analysis, including panhypopituitarism (OR = 4.394, 95% CI, 1.794-11.008, p = 0.002), supratentorial location of brain tumor (OR = 2.616, 95% CI, 1.245-5.499, p = 0.011), and chemotherapy (OR = 2.867, 95% CI, 1.018-8.069, p = 0.046). " 07/10/2004 - "[Marginal zone B-cell lymphoma of MALT with improvement of panhypopituitarism by chemotherapy]."
3.	Radiotherapy 10/31/2023 - "All patients undergoing radiotherapy had secondary panhypopituitarism. " 05/16/2023 - "Other features found to contribute to panhypopituitarism prediction were age, volume of tumour, and the use of radiotherapy. " 01/01/2021 - "Anterior panhypopituitarism was not affected by treatment modality (surgery vs surgery and radiotherapy) (P = .23). " 01/01/2019 - "Postoperative panhypopituitarism rate after first surgery was lower than expected; after radiotherapy, our results were comparable to other series." 04/01/2018 - "He presented with panhypopituitarism 2 years after radiation therapy. "
4.	Hormone Replacement Therapy (Therapy, Hormone Replacement) 01/01/2021 - "At 59 years of age, he was diagnosed with panhypopituitarism, and persistent hormone replacement therapy was subsequently started by an endocrinologist. " 12/01/2011 - "With the exception of bitemporal visual loss and panhypopituitarism requiring hormone replacement therapy, the patient had a relatively uneventful course until 2005. " 08/01/2005 - "Following total resection there is a very high incidence of panhypopituitarism requiring lifelong multiple hormone replacement therapy. " 01/01/2021 - "Long-term hormone replacement therapy due to panhypopituitarism can lead to serious complications and thus, pituitary transplantation is considered a more desirable. " 07/01/2023 - "We reported a 23-year-old male patient with panhypopituitarism who underwent two resections for craniopharyngioma and received postoperative hormone replacement therapy. "
5.	Hematopoietic Stem Cell Transplantation 10/09/2013 - "R-CHOP and high-dose methotrexate therapy followed by autologous hematopoietic stem cell transplantation was quite effective, and panhypopituitarism and adrenal insufficiency improved to almost normal values after successful treatment of the lymphoma with chemotherapy."