Panhypopituitarism

Author: Robert P Hoffman, MD; Chief Editor: Stephen Kemp, MD, PhD more...

Updated: Oct 21, 2009

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Background
Pathophysiology
Epidemiology
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Background

The pituitary gland is called the master endocrine gland of the body because it controls the function of other endocrine organs. The anterior pituitary produces the hormones thyrotropin (thyroid-stimulating hormone [TSH]), corticotropin (adrenocorticotropic hormone [ACTH]), luteinizing hormone (LH), follicle-stimulating hormone (FSH), growth hormone (GH), and prolactin (PRL). The anterior pituitary is controlled by specific hypothalamic-releasing hormones. The posterior pituitary produces vasopressin (antidiuretic hormone [ADH]) and oxytocin.

Panhypopituitarism is a condition of inadequate or absent production of the anterior pituitary hormones. It is frequently the result of other problems that affect the pituitary gland and either reduce or destroy its function or interfere with hypothalamic secretion of the varying pituitary-releasing hormones. Panhypopituitarism can be the end result of various clinical scenarios. The signs and symptoms are diverse. Manifestations of congenital anterior hypopituitarism include micropenis, midline defects, optic atrophy, hypoglycemia, and poor growth.

Pathophysiology of panhypopituitarism.

Pathophysiology

The effects of hypopituitarism in children depend on the affected hormones. GH deficiency can result in hypoglycemia and short stature. Gonadotropin deficiency leads to prenatal micropenis and delayed or interrupted puberty in older children. Corticotropin deficiency interferes with normal carbohydrate, protein, and lipid metabolism and may result in weight loss, hypoglycemia, fatigue, hypotension, and death. Thyrotropin deficiency leads to hypothyroidism.

Frequency

United States

Hypopituitarism is caused by various conditions and is associated with various hormonal deficiencies. Thus, data are limited regarding frequency rates of the various etiologies and components. An Italian study reported GH deficiency prevalence to be approximately 9 cases per 1000 individuals in a pediatric population.^[1]Data from the Northwest Regional Screening program estimate the frequency of congenital TSH deficiency at 1 case per 29,000 live births.^[2]

Mortality/Morbidity

Morbidity and mortality due to hypopituitarism are caused by the individual hormone deficiencies or the underlying cause of hypopituitarism. Individual hormonal deficiencies are discussed in greater detail in the specific articles, and the underlying causes of death are not discussed here.

Acute mortality due to hormonal deficiencies is rare. When deaths occur due to hormonal deficiencies, they are usually caused by adrenal insufficiency secondary to ACTH deficiency. These deaths are most likely to occur when an accompanying illness prevents appropriate oral glucocorticoid replacement.

Growing, but not completely conclusive, evidence indicates that childhood hypopituitarism may be associated with a shortened adult lifespan, even with adequate hormonal replacement.^[3]The increased mortality is due to cardiovascular abnormalities that are related to GH deficiency and past practices of not treating a GH deficiency when growth is complete.^{[4, 5]}Children and adolescents with GH deficiency have been shown to have impaired vascular function.^[6]In adults, GH treatment restores improves cardiovascular risk factors, but long-term studies that demonstrate reduced cardiovascular disease have not been reported.^{[7, 8]}

Proceed to Clinical Presentation

Contributor Information and Disclosures

Author

Robert P Hoffman, MD Associate Professor of Pediatrics, Department of Pediatrics, Ohio State University College of Medicine

Robert P Hoffman, MD is a member of the following medical societies: American Diabetes Association, American Pediatric Society, Christian Medical & Dental Society, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Thomas A Wilson, MD Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine

Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor, Department of Pediatrics, Harvard University Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds PI, also occasional consultant

Merrily P M Poth, MD Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfizer, Inc. Honoraria Consulting

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