Hypopituitarism - Thomas

Hasan AYDIN, MD
Endocrinology and Metabolism

Yeditepe University Medical Faculty
 The deficiency (hypo) of one or more hormones
of the pituitary gland
 Deficiency of one or multiple hormones of the
anterior pituitary …….-hypopituitarism
 Deficiency of the posterior lobe …..-central
diabetes insipitus
 Deficiency of all pituitary hormones….-
panhypopituitarism
 Either result from hypothalamus or pituitary
 Symptoms and signs frequently protean and

nonspecific
 Abnormalities in electrolyte levels,
 Altered mental status,
 Abnormal glucose levels,
 Altered body temperature,
 Increased heart rate
 Patients at risk of developing hypopituitarism

 Traumatic brain injury (TBI),
 Cocaine use,
 Subarachnoid hemorrhage,
 Postpartum hypotension (Sheehan syndrome).
 76% tumor or treatment of tumor
 Mass effect of adenoma on other hormones
 Surgical resection of non-adenomatous tissue
 Radiation of pituitary
 13% extrapituitary tumor

 Craniopharyngioma
 8% unknown
 1% sarcoidosis
 0.5% Sheehan’s syndrome

 In US
 Prevelance of pituitary adenoma 10-20%
 Hypopituitarism 2-8/100,000 persons/year
 World
 Incidence of 4.2 cases per 100,000 per year
 Prevalence of 45.5 per 100,000 without gender
difference
 Missed or delayed diagnosis could potentially lead to
permanent disability or death
 Female patients with hypopituitarism have more

than a 2-fold increase in cardiovascular mortality
 Cardiovascular disease is significantly higher among

hypopituitary patients (incidence ratio, 3.7; 95%
confidence interval)
 Hypopituitary patients have lower high-density

lipoprotein cholesterol and higher low-density/high-
density lipoprotein ratio
 Primary pituitary disease  Interruption of the
 Tumors pituitary stalk
 Pituitary surgery
 Radiation treatment
 Extrasellar disorders
 Hypothalamic
 Craniopharyngioma
disease  Rathke pouch
 Functional suppression of axis
 Exogenous steroid use
 Extreme weight loss
 Exercise
 Systemic Illness
 Developmental and  Acquired causes:
genetic causes  Infiltrative disorders
 Dysplasia  Cranial irradiation
 Septo-Optic dysplasia  Lymphocytic hypophysitis
 Developmental  Pituitary Apoplexy
hypothalamic dysfunction  Empty Sella syndrome
 Kallman Syndrome
 Laurence-Moon-Bardet-
Biedl Syndrome
 Frohlich Syndrome (Adipose
Genital Dystrophy)
 Hypothalamic dysfunction and hypopituitarism
 may result from dysgenesis of the septum pellucidum or
corpus callosum
 Affected children have mutations in the HESX1 gene
 These children exhibit variable combinations of:

 cleft palate
 syndactyly
 ear deformities
 optic atrophy
 micropenis
 anosmia
 Pituitary dysfunction
 Diabetes insipidus
 GH deficiency and short stature
 Occasionally TSH deficiency
 Defective hypothalamic gonadotropin-releasing
hormone (GnRH) synthesis
 Associated with anosmia or hyposmia due to olfactory

bulb agenesis or hypoplasia
 May also be associated with: color blindness,

optic atrophy, nerve deafness, cleft palate,
renal abnormalities, cryptorchidism
 GnRH deficiency prevents progression

through puberty
 Characterized by
 low LH and FSH levels
 low concentrations of sex steroids
 Males patients
 Delayed puberty and hypogonadism, including micropenis
 Long-term treatment:
 human chorionic gonadotropin (hCG) or testosterone
 Female patients
 Primary amenorrhea and failure of secondary sexual
development
 Long-term treatment:
 cyclic estrogen and progestin
 Repetitive GnRH administration restores normal puberty
 Fertility may also be restored by the administration of

gonadotropins or by using a portable infusion pump to
deliver subcutaneous, pulsatile GnRH
 Rare autosomal recessive disorder
 Characterized by mental retardation; obesity;

hexadactyly, brachydactyly, or syndactyly
 Central diabetes insipidus may or may not be

associated
 GnRH deficiency occurs in 75% of males and half of

affected females
 Retinal degeneration begins in early childhood

 most patients are blind by age 30
 A broad spectrum of hypothalamic lesions
 hyperphagia, obesity, and central hypogonadism
 Decreased GnRH production in these patients results in

 attenuated pituitary FSH and LH synthesis and release
 Deficiencies of leptin, or its receptor, cause these clinical

features
 Infiltrative disorders
 Cranial irradiation
 Lymphocytic hypophysitis
 Pituitary Apoplexy
 Empty Sella syndrome

 Etiology
 Presumed to be autoimmune
 Clinical Presentation
 Women, during postpartum period
 Mass effect (sellar mass)
 Deficiency of one or more anterior pituitary hormones
 ACTH deficiency is the most common
 Diagnosis
 MRI - may be indistinguishable from pituitary adenoma
 Treatment
 Corticosteroids – often not effective
 Hormone replacement
Patoloji Radyoloji
 Hemorrhagic infarction of a pituitary
adenoma/tumor
 Considered a neurosurgical emergency
 Presentation:
 Variable onset of severe headache
 Nausea and vomiting
 Meningismus
 Vertigo
 +/ - Visual defects
 +/ - Altered consciousness
 Symptoms may occur immediately or may develop
over 1-2 days
 Risk factors:
 Diabetes
 Radiation treatment
 Warfarin use
 Usually resolve completely
 Transient or permanent hypopituitarism is possible
 undiagnosed acute adrenal insufficiency
 Diagnose with CT/MRI
 Differentiate from leaking aneurysm
 Treatment:
 Surgical - Transsphenoid decompression
 Visual defects and altered consciousness
 Medical therapy – if symptoms are mild
 Corticosteroids
Radiology
 Infarction of pituitary after substantial blood loss
during childbirth
 Incidence: 3.6%
 No correlation between severity of hemorrhage and

symptoms
 Severe: recognised days to weeks PP

 Lethargy, anorexia, weight loss, unable to breast feeding
 Typically long interval between obstetric event and
diagnosis
 Of 25 cases studied:
 50% permanent amenorrhea
 The rest had scanty-rare menses
 Most lactation was poor to absent
 Dx: MRI empty sella turcica

Often an incidental MRI finding
 Usually have normal pituitary function
 Implying that the surrounding rim of pituitary tissue is fully
functional
 Hypopituitarism may develop insidiously
 Pituitary
masses may undergo clinically silent infarction
with development of a partial or totally empty sella by
cerebrospinal fluid (CSF) filling the dural herniation.
 Rarely, functional pituitary adenomas may arise within

the rim of pituitary tissue, and these are not always
visible on MRI
 Can present with features of deficiency of one or
more anterior pituitary hormones
 Clinical presentation depends on:
 Age at onset
 Hormone affected, extent
 Speed of onset
 Duration of the deficiency

Radiology
Women Men
 Oligomenorrhea or  Loss of libido
amenorrhea
 Erectile dysfunction
 Loss of libido
 Infertility
 Vaginal dryness or
dyspareunia  Loss of secondary sex
characteristics
 Loss of secondary sex  Atrophy of the testes
characteristics (estrogen
deficiency)  Gynecomastia (testosterone
deficiency)
 Results in hypocortisolism
 Malaise
 Anorexia
 Weight-loss
 Gastrointestinal disturbances
 Hyponatremia
 Pale complexion
 Unable to tan or maintain a tan
 No features of mineralocorticoid deficiency

 Aldosterone secretion unaffected
 Hypothyroidism
 Atrophic thyroid gland

 Inability to lactate postpartum
 Often 1st manifestation of Sheehan syndrome

 Adults
 Often asymptomatic
 May complain of
 Fatigue
 Degrees exercise tolerance
 Abdominal obesity
 Loss of muscle mass
 Children
 GH Deficiency
 Constitutional growth delay

 Biochemical diagnosis of pituitary
insufficiency
 Demonstrating low levels of trophic hormones in

the setting of low target hormone levels
 Provocative tests may be required to assess

pituitary reserve
 Basal ACTH secretion
 Cortisol < 3 μg/dL, cortisol deficiency
 Cortisol > 18μg/dL, sufficient ACTH
 Cortisol > 3 μd/dL but < 18 μg/dL - test ACTH reserve
 ACTH reserve
 Metyrapone test (750 mg q 4hr for 24 hrs)
 Cortisol < 7 μg/dL, 11-deoxycortisol > 10 μg/dL
 ITT ( 0.1 U/kg BW)
 Cortisol > 18 μg/dL, normal
 Cosyntropin stimulation test
 Cortisol > 18 μg/dL, normal
 Serum T4 should be measured
 TSH may not be helpful

 MEN
 Testosterone low, LH normal or low
 Sperm count
 WOMEN
 LH-FSH,
E2, vaginal cytology, response to
medroxyprogesterone 10 mg qd for 10 days
 Peak GH response < 5 ng/ml
 ITT
 Arginine ( 0.5 g/ kgBW i.v.)
 L-DOPA ( 0.5 g orally )
 Clonidine ( 0.15 mg orally )
 Glucagon ( 0.03 μg/kg BW s.c. + 40 mg propranolol )

 Cortisol deficiency ( 5 - 2.5 mg prednisone qd)
 Levothyroxine ( 0.075 -0.15 mg qd )
 Gonadal steroids (E2 ,P, Testosterone)
 Growth hormone
 Vasopressin (desmopressin 10 μg x 2)
 Hormone replacement therapy
 usually free of complications
 Treatment regimens that mimic physiologic

hormone production

 allow
for maintenance of satisfactory clinical
homeostasis

Hypopituitarism - Thomas

Uploaded by

Copyright:

Available Formats

Hypopituitarism - Thomas

Uploaded by

Document Information

Original Description:

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Hypopituitarism - Thomas

Uploaded by

Copyright:

Available Formats

What are the different causes of hypopituitarism?

What are the different causes of hypopituitarism?

What are some of the clinical manifestations of hypopituitarism?

What are some of the clinical manifestations of hypopituitarism?

Hasan AYDIN, MD

Endocrinology and Metabolism

 Symptoms and signs frequently protean and

 Patients at risk of developing hypopituitarism

 13% extrapituitary tumor

 0.5% Sheehan’s syndrome

 Female patients with hypopituitarism have more

 Cardiovascular disease is significantly higher among

 Hypopituitary patients have lower high-density

 Affected children have mutations in the HESX1 gene

 These children exhibit variable combinations of:

 Associated with anosmia or hyposmia due to olfactory

 May also be associated with: color blindness,

 GnRH deficiency prevents progression

 Repetitive GnRH administration restores normal puberty

 Fertility may also be restored by the administration of

 Characterized by mental retardation; obesity;

 Central diabetes insipidus may or may not be

 GnRH deficiency occurs in 75% of males and half of

 Retinal degeneration begins in early childhood

 Decreased GnRH production in these patients results in

 Deficiencies of leptin, or its receptor, cause these clinical

 Empty Sella syndrome

 No correlation between severity of hemorrhage and

 Severe: recognised days to weeks PP

 50% permanent amenorrhea

 The rest had scanty-rare menses

 Most lactation was poor to absent

 Dx: MRI empty sella turcica

 Hypopituitarism may develop insidiously

 Rarely, functional pituitary adenomas may arise within

 Clinical presentation depends on:

 Hormone affected, extent

 Duration of the deficiency

 No features of mineralocorticoid deficiency

 Atrophic thyroid gland

 Often 1st manifestation of Sheehan syndrome

 Constitutional growth delay

 Demonstrating low levels of trophic hormones in

 Provocative tests may be required to assess

 TSH may not be helpful

 Testosterone low, LH normal or low

 Arginine ( 0.5 g/ kgBW i.v.)

 L-DOPA ( 0.5 g orally )

 Clonidine ( 0.15 mg orally )

 Glucagon ( 0.03 μg/kg BW s.c. + 40 mg propranolol )

 Levothyroxine ( 0.075 -0.15 mg qd )

 Gonadal steroids (E2 ,P, Testosterone)

 usually free of complications

 Treatment regimens that mimic physiologic

You might also like