Endocrinology Book

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Hypopituitarism

Aka: Hypopituitarism, Panhypopituitarism, Pituitary Insufficiency, Sheehan Syndrome
  1. See Also
    1. Pituitary Gland
    2. Pituitary Adenoma
    3. Hypogonadotropic Hypogonadism
    4. Short Stature
    5. Amenorrhea
  2. Definitions
    1. Hypopituitarism
      1. Decreased or loss of pituitary Hormone secretion due to surgery, radiation, CNS mass, infarction or pituitary apoplexy
      2. Decreased Luteinizing Hormone (LH), Follicle Stimulating Hormone (FSH), Somatotropin (GH) and Corticotropin (ACTH)
    2. Sheehan Syndrome
      1. Associated with severe Postpartum Hemorrhage with prolonged Hypovolemia, and secondary pituitary ischemia and necrosis
      2. Presents with peripartum failed Lactation, Fatigue and Hypotension; later Amenorrhea
      3. Severe cases may be associated with Adrenal Insufficiency
      4. Best outcomes with early pituitary Hormone Replacement
  3. Causes
    1. Congenital
      1. Perinatal Injury
      2. Developmental Disorders
      3. Anencephaly
      4. Lipid storage disease
      5. Genetic
        1. PIT-1 (affects GH, TSH and Prolactin)
        2. PROP-1 (affects GH, LH, FSH, TSH, ACTH, Prolactin)
        3. HESX1 (affects GH, LH, FSH, TSH, ACTH, ADH)
        4. LHX3, 4 (affects GH, LH, FSH, TSH, Prolactin)
        5. PITX2 (affects GH, Prolactin)
        6. Isolated defects affect a single pituitary Hormone (>10 genetic defects identified)
    2. Trauma
      1. Closed Head Injury
      2. CNS Surgery
      3. Irradiation to brain, pituitary or nasopharynx
    3. CNS Mass (may be associated with pituitary apoplexy, CVA related dysfunction)
      1. Intrasellar
        1. Pituitary Adenoma
        2. Craniopharyngioma
      2. Parasellar
        1. Meningioma
        2. Optic Nerve glioma
      3. Metastatic cancer
        1. Breast Cancer
        2. Lung Cancer
        3. Melanoma
        4. Renal cell cancer
      4. Other malignancy
        1. Leukemia
        2. Lymphoma
        3. Germ cell tumors
    4. Ischemic Pituitary necrosis
      1. Postpartum Hemorrhage (Sheehan Syndrome)
      2. Diabetes Mellitus
      3. Sickle Cell Disease
      4. Temporal Arteritis
      5. Eclampsia
    5. Vascular conditions
      1. Cavernous Sinus Thrombosis
      2. Internal carotid aneurysm (intracranial)
    6. Infectious Disease
      1. Tuberculous Meningitis
      2. Malaria
      3. HIV Infection
      4. Fungal infections
    7. Infiltrative Disease
      1. Hemochromatosis
      2. Amyloidosis
      3. Lipid storage disease
      4. Granulomatous Disease
        1. Sarcoidosis
        2. Hypophysitis
    8. Miscellaneous Primary Causes
      1. Primary Empty Sella Syndrome
      2. Monohormonal idiopathic defects
    9. Miscellaneous Secondary Causes
      1. Nutritional Deficiency (Starvation, Anorexia Nervosa)
  4. Findings: ACTH Deficiency (Corticotroph Deficiency)
    1. See Adrenal Insufficiency (Addison's Disease)
    2. Acute
      1. Fatigue
      2. Weakness
      3. Dizziness
      4. Nausea or Vomiting
      5. Hypotension
      6. Hypoglycemia
    3. Chronic
      1. Fatigue
      2. Pallor
      3. Weight loss
      4. Hypoglycemia
  5. Findings: TSH Deficiency (Thyrotropin Deficiency)
    1. See Hypothyroidism
    2. Fatigue
    3. Cold Intolerance
    4. Constipation
    5. Weight gain
    6. Alopecia
    7. Dry Skin
    8. Bradycardia
    9. Hoarse voice
    10. Cognitive slowing
  6. Findings: LH or FSH Deficiency (Gonadotropin Deficiency)
    1. See Hypogonadotropic Hypogonadism
    2. Male
      1. See Male Hypogonadism or Testicular Failure
      2. Decreased sexual function and decreased libido
      3. Decreased Muscle mass
      4. Decreased Bone Mineral Density
      5. Decreased body hair
    3. Female
      1. See Premature Ovarian Failure
      2. Amenorrhea or Oligomenorrhea
      3. Infertility
      4. Decreased libido
      5. Osteoporosis
  7. Findings: GH Deficiency (Growth Hormone Deficiency)
    1. See Growth Hormone Deficiency
    2. Child (Congenital GH Deficiency)
      1. Decreased linear growth
        1. Normal size at birth
        2. Subsequently falls off height growth curve
        3. Weight for age is preserved
      2. Episodic Hypoglycemia
      3. Prolonged Neonatal Jaundice
      4. Microphallus
      5. Midline craniofacial abnormalities
    3. Adult
      1. Fatigue
      2. Decreased Muscle mass and strength
      3. Increased visceral fat
      4. Premature atherosclerosis
  8. Findings: Prolactin Deficiency
    1. See Prolactin
    2. Lack of Breast alveolar milk secretion in pregnancy and failed Lactation
  9. Findings: ADH Deficiency (Antidiuretic Hormone Deficiency)
    1. See Central Diabetes Insipidus
    2. Polyuria
    3. Polydipsia
    4. Nocturia
  10. Labs
    1. Functional or stimulation tests are typically not needed for diagnosis
    2. Obtain Basal Hormone levels (morning Fasting levels)
      1. Growth Hormone (GH)
      2. Luteinizing Hormone (LH)
      3. Follicle Stimulating Hormone (FSH)
      4. Thyroid Stimulating Hormone (TSH) with reflex to Free Thyroxine (Free T4)
      5. Adrenocorticotropic Hormone (ACTH)
  11. Management
    1. Treat specific Hormone deficiencies affected and the underlying cause
    2. See Adrenal Insufficiency (Addison's Disease)
    3. See Hypothyroidism
    4. See Hypogonadotropic Hypogonadism
    5. See Male Hypogonadism or Testicular Failure
    6. See Premature Ovarian Failure
    7. See Growth Hormone Deficiency
    8. See Central Diabetes Insipidus
  12. References
    1. Gounden (2021) Hypopituitarism, in StatPearls, Treasure Island, accessed 3/28/2022
      1. https://www.ncbi.nlm.nih.gov/books/NBK470414/
    2. Kim (2015) Endocrinol Metab 30:443-55 [PubMed]

Hypopituitarism (C0020635)

Definition (NCI) A condition of diminution or cessation of secretion of one or more hormones from the anterior pituitary gland. This may result from surgical or radiation ablation, non-secretory pituitary neoplasms, metastatic tumors, infarction, pituitary apoplexy, infiltrative or granulomatous processes, and other conditions.
Definition (MSH) Diminution or cessation of secretion of one or more hormones from the anterior pituitary gland (including LH; FOLLICLE STIMULATING HORMONE; SOMATOTROPIN; and CORTICOTROPIN). This may result from surgical or radiation ablation, non-secretory PITUITARY NEOPLASMS, metastatic tumors, infarction, PITUITARY APOPLEXY, infiltrative or granulomatous processes, and other conditions.
Definition (CSP) diminution or cessation of secretion of one or more hormones from the anterior pituitary gland (including luteinizing hormone, follicle stimulating hormone, somatotropin; and corticotropin); may result from surgical or radiation ablation, non-secretory pituitary neoplasms, metastatic tumors, infarction, pituitary apoplexy, infiltrative or granulomatous processes, and other conditions.
Concepts Disease or Syndrome (T047)
MSH D007018
ICD10 E23.0
SnomedCT 267482009, 237681002, 154703006, 190469009, 74728003
French HYPOPITUITARISME, INSUFFICIANCE HYPOPHYSAIRE, Hypopituitarisme, Insuffisance antéhypophysaire, Insuffisance hypophysaire
English HYPOPITUITARISM, PITUITARY INSUFFICIENCY, Hypopituitarism NOS, Hypopituitarism, HYPOSECRET SYNDROME ANTERIOR PITUITARY, ANTERIOR PITUITARY HYPOSECRET SYNDROME, ADENOHYPOPHYSEAL HYPOSECRET, HYPOSECRET ADENOHYPOPHYSEAL, hypopituitarism (diagnosis), hypopituitarism, Hypopituitalism, Insufficiency pituitary, Adenohypophyseal Hyposecretion, Hyposecretion, Adenohypophyseal, Anterior Pituitary Hyposecretion Syndrome, Hyposecretion Syndrome, Anterior Pituitary, Pituitary insufficiency NOS, Hypopituitarism [Disease/Finding], pituitary failure, insufficiency pituitary, pituitary insufficiency, deficiencies pituitary, pituitary hypofunction, Hypopituitarism NOS (disorder), Pituitary deficiency, Pituitary failure, Pituitary insufficiency, Deficient secretion of one OR more pituitary hormones, Hypopituitarism (disorder), Pituitary hypofunction, hypofunction; pituitary, hypopituitarism; syndrome, insufficiency; pituitary, pituitary; hypofunction, pituitary; insufficiency, syndrome; hypopituitarism, Deficient secretion of one or more pituitary hormones, Hypopituitarism, NOS, Pituitary insufficiency, NOS, Insufficiency, Pituitary, Pituitary Insufficiency
Portuguese HIPOPITUITARISMO, Insuficiência hipofisária, INSUFICIENCIA PITUITARIA, Hipossecreção Adeno-Hipofisária, Hipossecreção Adenoipofisária, Hipofunção hipofisária, Hipopituitarismo, Síndrome da Hipossecreção Hipofisária Anterior
Dutch hypofyse-insufficiëntie, insufficiëntie hypofyse, hypofunctie; hypofyse, hypofyse; hypofunctie, hypofyse; insufficiëntie, hypopituïtarisme; syndroom, insufficiëntie; hypofyse, syndroom; hypopituïtarisme, hypopituïtarisme, Adenohypofyse-hyposecretie, Hyposecretiesyndroom, adenohypofyse-, Ziekte van Simmonds-Sheehan, Hypopituïtarisme
German Insuffizienz, Hypophyse, HYPOPHYSE UNTERFUNKTION, Hypopituitarismus, Hyposekretionssyndrom des Hypophysenvorderlappens, Adenohypophysäre Hyposekretion, Hypophyseninsuffizienz, Hyposekretion, adenohypophysäre
Italian Insufficienza ipofisaria, Iposecrezione adenoipofisaria, Sindrome dell'iposecrezione dell'ipofisi anteriore, Insufficienza pituitaria, Ipopituitarismo
Spanish Insuficiencia hipofisaria, Insuficiencia pituitaria, INSUFICIENCIA HIPOFISARIA, hipopituitarismo, SAI, hipopituitarismo, SAI (trastorno), Hipofunción Pituitaria Anterior, hipopituitarismo, insuficiencia hipofisaria (trastorno), insuficiencia hipofisaria, insuficiencia pituitaria, secreción insuficiente de una O más hormonas hipofisarias, Hipopituitarismo, Hiposecreción Adenohipofisiaria, Síndrome de Hiposecreción Pituitaria Anterior
Swedish Hypopituitarism
Japanese カスイタイキノウテイカショウ, カスイタイキノウフゼン, 下垂体機能不全症, 下垂体低下症, Simmonds病, 脳下垂体機能低下症, 下垂体不全症, シーハン症候群, 下垂体機能低下, 下垂体機能不全, 下垂体不全, シモンズ病, 下垂体前葉機能低下症, 下垂体低下, Sheehan症候群, 下垂体機能低下症
Czech hypopituitarismus, Hypopituitarismus, Hypofyzární insuficience, Hypofyzární nedostatečnost
Finnish Hypopituitarismi
Russian SHIKHANA SINDROM, GIPOPITUITARIZM, SIMMONDSA SINDROM, ГИПОПИТУИТАРИЗМ, СИММОНДСА СИНДРОМ, ШИХАНА СИНДРОМ
Korean 뇌하수체 기능저하증
Croatian Not Translated[Hypopituitarism]
Polish Choroba Glińskiego-Simmondsa, Niedoczynność przysadki, Zespół podwzgórzycy pociążowej, Zespół Sheehana
Hungarian Hypophysis elégtelenség, Hypopituitarismus, Hypophysis insufficientia
Norwegian Hypopituitarisme, For lav hormonproduksjon i hypofysen
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Sheehan Syndrome (C0242342)

Definition (NCI) An uncommon cause of hypopituitarism seen after severe postpartum hemorrhaging. Prolonged hypovolemia leads to ischemic necrosis of the pituitary. Clinical signs typically present in the puerperium and include failure to begin lactation, fatigue, hypotension and eventual amenorrhea. Clinical course is usually mild, however extreme cases may progress to adrenal failure. Prognosis is most favorable when hormone replacement is initiated soon after symptom onset.
Concepts Disease or Syndrome (T047)
MSH D007018
ICD10 E23.0
SnomedCT 237684005, 154703006, 267482009, 190469009, 15045007, 32390006, 290653008
English Sheehan's Syndrome, Sheehans Syndrome, Syndrome, Sheehan's, SIMMONDS DIS, Sheehan's syndrome (diagnosis), postpartum pituitary necrosis, Sheehan's syndrome (postpartum necrosis), Syndrome, Sheehan, Sheehan syndrome, sheehan syndrome, simmonds disease, sheehan's syndrome, postpartum hypopituitarism, sheehans's syndrome, simmond's disease, simmonds' disease, sheehans syndrome, Ischemic postpartum pituitary necrosis, Ischemic postpartum pituitary necrosis (disorder), Ischaemic postpartum pituitary necrosis, postpartum hypopituitarism (diagnosis), Simmond's disease, Sheehan's syndrome, Postpartum pituitary necrosis, Simmonds' disease, Postpartum hypopituitarism, Postpartum hypopituitarism (disorder), Sheehan's syndrome (disorder), Sheehan, Simmonds, hypopituitarism; postpartum, postpartum; hypopituitarism, postpartum; panhypopituitarism, syndrome, syndrome; postpartum panhypopituitary, Postpartum pituitary infarction, Reye-Sheehan syndrome, Ischemic postpartum pituitary necrosis (disorder) [Ambiguous], Simmonds disease, Sheehan Syndrome, Postpartum necrosis of pituitary, Simmonds Disease, Simmonds' Disease, Simmond's Disease, Disease, Simmonds, Postpartum Hypopituitarism, Hypopituitarism, Postpartum, Panhypopituitarism, Postpartum, Pituitary Insufficiency, Postpartum, Postpartum Panhypopituitarism, Postpartum Pituitary Insufficiency
Dutch syndroom van Sheehan, hypopituïtarisme; post partum, post partum; hypopituïtarisme, post partum; panhypopituïtarisme, syndroom, syndroom; post partum panhypopituïtarisme, postpartum hypopituïtarisme, Sheehan-ziekte
Japanese シーハン症候群, 分娩後下垂体機能低下症, シーハンショウコウグン, ブンベンゴカスイタイキノウテイカショウ
French Syndrome de Sheehan, Hypopituitarisme du post-partum, Cachexie hypophysaire, Maladie de Simmonds, Nécrose hypophysaire du post-partum, Nécrose pituitaire du post-partum
German Postpartaler Hypopituitarismus, postpartaler Hypopituitarismus, Simmonds-Sheehan-Syndrom, Sheehan-Syndrom, Simmonds-Krankheit, Postpartale Hypophyseninsuffizienz, Postpartaler Panhypopituitarismus
Italian Morbo di Simmonds, Insufficienza pituitaria postpartum, Sindrome di Sheehan, Ipopituarismo postpartum, Panipopituitarismo postpartum
Czech Poporodní hypopituitarismus, poporodní hypopituitarismus, Simmondsova nemoc, Sheehanův syndrom
Hungarian Postpartum hypopituitarismus, Sheehan-syndroma
Spanish necrosis pituitaria isquémica posparto, infarto pituitario postparto, necrosis hipofisaria isquémica postparto, infarto hipofisario postparto, síndrome de Reye - Sheehan, enfermedad de Simmonds, hipopituitarismo posparto (trastorno), hipopituitarismo posparto, hipopituitarismo postparto, necrosis hipofisaria posparto, necrosis pituitaria posparto, síndrome de Sheehan (concepto no activo), síndrome de Sheehan (trastorno), síndrome de Sheehan, Hipopituitarismo postparto, Enfermedad de Simmonds, Síndrome de Sheehan
Portuguese Hipofunção hipofisária pós-parto, Doença de Simmonds, Síndrome de Sheehan
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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