Download presentation
Presentation is loading. Please wait.
Published byScot Matthews Modified over 9 years ago
1
Hasan AYDIN, MD Endocrinology and Metabolism Yeditepe University Medical Faculty
2
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
3
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).
4
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
5
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
6
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
7
Primary pituitary disease Tumors Pituitary surgery Radiation treatment Hypothalamic disease Functional suppression of axis Exogenous steroid use Extreme weight loss Exercise Systemic Illness Interruption of the pituitary stalk Extrasellar disorders Craniopharyngioma Rathke pouch
8
Developmental and genetic causes Dysplasia Septo-Optic dysplasia Developmental hypothalamic dysfunction Kallman Syndrome Laurence-Moon-Bardet- Biedl Syndrome Frohlich Syndrome (Adipose Genital Dystrophy) Acquired causes: Infiltrative disorders Cranial irradiation Lymphocytic hypophysitis Pituitary Apoplexy Empty Sella syndrome
9
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
10
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
11
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
12
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
13
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
14
Infiltrative disorders Cranial irradiation Lymphocytic hypophysitis Pituitary Apoplexy Empty Sella syndrome
15
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
16
Patoloji Radyoloji
17
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
18
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
19
Radiology
20
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
21
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
22
Often an incidental MRI finding
23
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
24
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
25
Radiology
27
Women Oligomenorrhea or amenorrhea Loss of libido Vaginal dryness or dyspareunia Loss of secondary sex characteristics (estrogen deficiency) Men Loss of libido Erectile dysfunction Infertility Loss of secondary sex characteristics Atrophy of the testes Gynecomastia (testosterone deficiency)
28
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
29
Hypothyroidism Atrophic thyroid gland
30
Inability to lactate postpartum Often 1 st manifestation of Sheehan syndrome
31
Adults Often asymptomatic May complain of Fatigue Degrees exercise tolerance Abdominal obesity Loss of muscle mass Children GH Deficiency Constitutional growth delay
32
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
33
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 10 μg/dL ITT ( 0.1 U/kg BW) Cortisol > 18 μg/dL, normal Cosyntropin stimulation test Cortisol > 18 μg/dL, normal
34
Serum T 4 should be measured TSH may not be helpful
35
MEN Testosterone low, LH normal or low Sperm count WOMEN LH-FSH, E 2, vaginal cytology, response to medroxyprogesterone 10 mg qd for 10 days
36
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 )
37
Cortisol deficiency ( 5 - 2.5 mg prednisone qd) Levothyroxine ( 0.075 -0.15 mg qd ) Gonadal steroids (E 2,P, Testosterone) Growth hormone Vasopressin (desmopressin 10 μg x 2)
38
Hormone replacement therapy usually free of complications Treatment regimens that mimic physiologic hormone production allow for maintenance of satisfactory clinical homeostasis
Similar presentations
© 2025 SlidePlayer.com. Inc.
All rights reserved.