hypogonadotrophic hypogonadism
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HypogonadotrophicHypogonadism(HH)
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CNS-Hypothalamus-Pituitary
Ovary-uterus InteractionNeural control Chemical control
Dopamine
(-)
Norepiniphrine
(+)
Endorphines
(-)
Hypothalamus
Gn-RH
Ant. pituitary
FSH, LH
Ovaries
Uterus
ProgesteroneEstrogen
Menses
?
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What is hypogonadotrophic hypogonadism?A state of gonadal hypofunction
(hypoestrognemia-anovulation-
amenorrhea) due to anatomic or
functional disorder in
hypothalamic/pituitarycompartments.
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How does HH presents? In childhood pubertal delay or
arrest and primary amenorrhea.
In sexually mature women it
presents with 2ry amenorrhea.
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Endocrine basis of HH
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1 ry amenorrhea evaluation
Four categories of patients are identified
1. Amenorrhea with absent or poorsecondary sex Characters
2. Amenorrhea with normal 2rysex characters
3. Amenorrhea with signs ofandrogen excess
4. Amenorrhea with absent uterusand vagina
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Kallmann syndrome
Kallmann syndrome is a rare inheriteddisorder.
Three forms are known X-linked, autosomaldominant and autosomal recessive.
The major characteristics of Kallmannsyndrome, in both men and women, are thefailure to experience puberty and thecomplete or partial loss of the sense of
smell. Associated findings might include midline
facial defects, neurosensory deafness,cerebellar ataxia, and epilepsy.
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Kallmann syndrome
In Kallmann syndrome, the GnRH
neurons do not migrate properly from
the olfactory placode to the
hypothalamus during development.
GnRH neurons remain in nasal
epithelium but locally produced Gn-RH
fails to reach pituitary and stimulategonadotrophins.
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Olfactory neurons and Gn-RH neurons
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Idiopathic Hypogonadotrophic
Hypogonadism
Hypoplasia in Gn-RH neurons causingisolated gonadotropin deficiency.
Distinguished from Kallmann syndrome
by olfactory testing with strong odors.Interestingly many of these patients areunaware of their defect.
Treatment in both cases is cyclic hormonal
treatment, or ovulation induction bygonadotropin which requires long time butalmost always successful.
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Other hypothalamic syndromes
Adiposogenital Dystrophy( Babinski-
Froelich Syndrome)
Leurence Moon -Biedel syndrome.
Hypothalamic Infantilism-Obesity
Launois-Cleret Syndrome
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What is pituitary adenoma?
Pituitary adenoma is a tumorof the pituitary gland.
The word adenomaspecifically means tumor ofglandular tissue.
Pituitary adenomas are quitecommon in the generalpopulation. It is estimatedthat around 20% of allindividuals will develop apituitary tumor at some point
during their lifetime, thoughmany do not cause anyproblems and are neverdiagnosed.
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What is pituitary adenoma?
Most pituitary tumors arebenign.
Two-thirds of pituitaryadenomas will remaincompletely confined to thepituitary gland.
Approximately one-third willexpand into tissues that arein the immediate vicinity ofthe pituitary gland, such asthe brain.
Less than one percent of all
pituitary adenomas aremalignant.
Pituitary tumors occur inevery age group but arerarely diagnosed before agetwenty.
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Classification of pituitary adenomas:
The most common type ofpituitary tumor is prolactinoma.These account for nearly 30% ofall pituitary tumors.
The second most prevalenttype, comprising approximately25% of pituitary tumors, iscalled a null cell adenoma. Nullcell adenomas do not produceany hormone.
Growth hormone-secretingtumors, seen in about 10-15% ofpatients, are associated withacromegaly.
Another 10-15% of pituitaryadenomas are accounted for byACTH-secreting tumors, whichare often the cause ofCushing's disease.
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Pituitary lactotrophs
Hypothalamus
PIF (Dopamine)TRH
(-)(+)
Prolactin
Prolactin control
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HyperprolactinemiaCauses: Prolactin secreting pituitary adenoma ( prolactinoma) categorized by size
into microadenoma1 cm.
Non-functioning pituitary adenoma or craniopharyngioma compressing
the pituitary stalk preventing PIF from reaching pituitary.
Primary hypothyroidism because TRH acts as prolactin stimulating
factor.
Iatrogenic due to drugs that inhibit dopaminergic activity through direct
effect on secretion or receptor blockade like metoclopramide,
phenothiazines and opioids.
Chronic renal failure.
Ectopic prolactin secretion by extr-pituitary tumor.
Idiopathic.
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HyperprolactinemiaInvestigations:
Serum prolactin levels. Levels >250 ng/mL are
almost always associated with a prolactinoma.
Magnetic resonance imaging (MRI) with gadolinium
enhancement provides the best visualization of the
sellar area, especially if there is a microadenoma,
defined as a lesion
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Hyperprolactinemia
Clinical presentation:High prolactin levels particularly
with prolactin secreting pituitary
adenoma typically present with
non-peurperal amenorrhea-
Galactorrhea (Forbes-Albright
syndrome)-(Ahumada-Del Castillo
syndrome).
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Axial CoronalSagital
MRI with gadolinium enhancement
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Hyperprolactinemia
Treatment: Medical treatment is the main stay
using dopamine agonists including
lysergic acid derivatives like
Bromocreptine (Parlodel) and ergots
derivatives like Cabergoline (Dostinex).
Thyroid hormone replacement is
indicated for cases of hypothyroidism.
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HyperprolactinemiaTreatment:
Surgical treatment is considered for tumors
compressing the stalk or causing pressure
symptoms like cranio-pharyngeoma.
Trans-sphenoidal resection of pituitary macro-
prolactinoma but experience is mandatory to avoid
the 2 common complications of CSF rhinorrhea, and
subsequent pan-hypo-pituitarism.
Recently Gamma knife surgery has been introduced
for large tumors with good results.
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Sheehans syndrome
Hypertrophy and hyperplasia of lactotrophs during
pregnancy results in the enlargement of the anterior
pituitary, without a corresponding increase in blood
supply.
Secondly, the anterior pituitary is supplied by a low
pressure portal venous system.
These vulnerabilities, when affected by major
hemorrhage or hypotension during the peripartum
period, can result in ischemia of the affectedpituitary regions leading to necrosis.
The posterior pituitary is usually not affected due to
its direct arterial supply.
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Sheehans syndrome
Pituitary necrosis following massiveobstetric hemorrhage.
Pan-hypo-pituitarism might occur
although pituitary cells have differentsensitivity to damage.
The most sensitive are lactotrops followedby gonadotrops then somatotrophs,corticotrops and least are thyrotrops
Therefore the first manifestation is usuallyfailure of lactation.
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Simmonds Disease
(Pituitary cachexia)
Deficiency of all of pituitary hormones (i.e)pan-hypopituitarism.
The first description of the condition
was made in 1914 by the Germanphysician DrMorris Simmonds.
http://en.wikipedia.org/wiki/Morris_Simmondshttp://en.wikipedia.org/wiki/Morris_Simmonds -
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Functional HH (Hypothalamic
amenorrhea)
Eating disorders.
Exercise induced amenorrhea.
Stress induced amenorrhea.
Pseudocyesis.
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The neuronal clamp(hypothalamic gonadostat)
The mechanism whereby
reproduction is shut down
when the nutritional status and
energy balance is shifted or
compromised .
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A Little About the Central Players
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Weight-related amenorrhoea
Anorexia Nervosa
1o or 2o Amenorrhea is often first sign
A body mass index (BMI)
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Anorexia Nervosa
Has a low body weight.
Refuses to keep a normal bodyweight.
Extremely afraid of becomingfat.
Believes she is fat even whenshe's very thin.
Misses three (menstrual)periods for girls/women whohave started having theirperiods.
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Anorexia Nervosa
AA is a life threateningcondition.
Treatment can beextremely difficult withfrequent relapses.
Intense psychiatricadvice and continuoussupport are mandatory.
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Exercise-associated amenorrhoea
Common in competitiveathletes, long distance runnersand ballet dancers.
Might delay puberty in girlsbeginning before menarche orcause 2ry amenorrhea orirregular cycles.
Low body fat and high levels ofendogenous opioids areresponsible for decrease Gn-RHpulsatility.
O i H h l i
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Organic Hypothalamic-
pituitary destruction
Tumours like craniopharyngeoma, glioma,
and metastasis.
Infilteration like sarcoidosis and
hemochromatosis.
Infections like meningitis or encephalitis.
Trauma like fracture base skull.
Irradiation.
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