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    Acromegaly: Atwebembere Raymond 214-083011-07132 Dr. Ssebuliba Moses

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    Nina
    The document provides an overview of Acromegaly including: 1) It is a hormonal disorder caused by excess growth hormone resulting in enlarged features and organs. 2) Symptoms include joint pain, enlarged extremities and facial features, and organ enlargement. 3) Diagnosis involves blood tests showing elevated GH and IGF-1 levels and imaging to identify pituitary tumors. 4) Treatment goals are to reduce hormone levels, relieve tumor pressure, and preserve pituitary function. Options include surgery, medication, and radiation.

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    Acromegaly: Atwebembere Raymond 214-083011-07132 Dr. Ssebuliba Moses

    Uploaded by

    Nina
    48 views32 pages
    The document provides an overview of Acromegaly including: 1) It is a hormonal disorder caused by excess growth hormone resulting in enlarged features and organs. 2) Symptoms include joint pain, enlarged extremities and facial features, and organ enlargement. 3) Diagnosis involves blood tests showing elevated GH and IGF-1 levels and imaging to identify pituitary tumors. 4) Treatment goals are to reduce hormone levels, relieve tumor pressure, and preserve pituitary function. Options include surgery, medication, and radiation.

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    Acromegaly

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    The document provides an overview of Acromegaly including: 1) It is a hormonal disorder caused by excess growth hormone resulting in enlarged features and organs. 2) Symptoms include joint pain, enlarged extremities and facial features, and organ enlargement. 3) Diagnosis involves blood tests showing elevated GH and IGF-1 levels and imaging to identify pituitary tumors. 4) Treatment goals are to reduce hormone levels, relieve tumor pressure, and preserve pituitary function. Options include surgery, medication, and radiation.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    48 views32 pages

    Acromegaly: Atwebembere Raymond 214-083011-07132 Dr. Ssebuliba Moses

    Uploaded by

    Nina
    The document provides an overview of Acromegaly including: 1) It is a hormonal disorder caused by excess growth hormone resulting in enlarged features and organs. 2) Symptoms include joint pain, enlarged extremities and facial features, and organ enlargement. 3) Diagnosis involves blood tests showing elevated GH and IGF-1 levels and imaging to identify pituitary tumors. 4) Treatment goals are to reduce hormone levels, relieve tumor pressure, and preserve pituitary function. Options include surgery, medication, and radiation.

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    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 32

    ACROMEGALY

    ATWEBEMBERE RAYMOND
    214-083011-07132
    DR. SSEBULIBA MOSES
    Course OUTLINE
    • Introduction

    • Definition

    • Epidemiology

    • Physiology and pathophysiology

    • Clinical features

    • Investigations

    • Management
    Introduction

    • Acromegaly is a hormonal disorder that results from too


    much growth hormone (GH) in the body.
    • Men >5ng/ml, women >10ng/ml, children >20ng/ml

    • Usually the excess GH comes from benign, or


    noncancerous, tumors on the pituitary. These benign
    tumors are called adenomas.
    • Hormones produced by pituitary gland:

    • Anterior
    • GnRH
    • GHRH
    • Corticotrophin releasing Hormone
    • Dopamine
    • Thyroid releasing hormone

    • Posterior
    • Vasopressin
    • Oxytocin
    • The most serious health consequences:
    • type 2 diabetes
    • high blood pressure,
    • increased risk of cardiovascular disease,
    • and arthritis.
    • Patients are at increased risk for colon polyps, which may
    develop into colon cancer if not removed.
    Epidemiology
    • Population prevalence of 60 per million.

    • An incidence of 3-4 per million per year.

    • Males and females appear to be equally affected with an


    average age of presentation of 44 years.
    • Younger patients may have more aggressive tumors and
    higher GH concentrations.
    physiology
    Pituitary Tumors
    • If the pituitary continues to make GH independent of the
    normal regulatory mechanisms.
    • level of IGF-I continues to rise, leading to bone
    overgrowth and organ enlargement.
    • High levels of IGF-I also cause changes in glucose
    (sugar) and lipid (fat) metabolism and can lead to
    diabetes, high blood pressure, and heart disease.
    • > 95 percent of people with acromegaly, a benign tumor
    of the pituitary gland, an adenoma, producing excess GH.
    • Pituitary tumors are labeled either micro- or macro-
    adenomas. Most GH-secreting tumors are macro-
    adenomas, larger than 1 centimeter.
    • Most pituitary tumors develop spontaneously and are not
    genetically inherited. They are the result of a genetic
    alteration in a single pituitary cell, which leads to
    increased cell division and tumor formation.
    • The mutation occurs in a gene that regulates the
    transmission of chemical signals within pituitary cells
    • A tumor growing upward may affect the optic chiasm
    leading to visual problems and vision loss.
    • If the tumor grows to the side, it may enter an area of the
    brain called the cavernous sinus, damaging nerves.
    • Compression of the surrounding normal pituitary tissue
    can alter production of other hormones leading to changes
    in menstruation and breast discharge in women and
    erectile dysfunction in men
    • . If the tumor affects the part of the pituitary that controls
    the thyroid hormones which may decrease. causing
    weight gain, fatigue, and hair and skin changes.
    • If it affects the part controlling the adrenal gland, the
    hormone cortisol may decrease leading to weight loss,
    dizziness, fatigue, low blood pressure, and nausea.
    • Some GH-secreting tumors may also secrete too much
    prolactin, the hormone that stimulates the mammary
    glands to produce milk.
    NON-PITUITARY TUMORS
    • Rarely, acromegaly is caused by tumors of the pancreas,
    lungs, GIT and other parts of the brain.
    • These tumors also lead to excess GH, either because they
    produce GH themselves or, more frequently, because they
    produce GHRH.
    CLINICAL FEATURES

    • Swelling of the hands and feet; is often an early feature,


    patients noticing a change in ring or shoe size(width).
    • Gradually, bone changes alter the patient’s facial features:
    The brow and lower jaw protrude, the nasal bone
    enlarges, and the teeth space out.
    • Overgrowth of bone and cartilage leads to arthritis.

    • When tissue thickens, it may trap nerves, causing carpal


    tunnel syndrome, which results in numbness and
    weakness of the hands.
    • Body organs, including the heart, may enlarge.
    • joint aches

    • thick, coarse, oily skin

    • skin tags

    • enlarged lips, nose, and tongue

    • deepening of the voice due to enlarged sinuses and vocal cords

    • sleep apnea—breaks in breathing during sleep due to


    obstruction of the airway
    • excessive sweating and skin odor

    • fatigue and weakness

    • headaches

    • impaired vision

    • abnormalities of the menstrual cycle and sometimes breast discharge


    in women
    • erectile dysfunction in men

    • decreased libido
    DIAGNOSIS
    • Blood tests:
    • Oral glucose tolerance test because drinking 75 to 100 grams
    of glucose solution lowers blood GH levels to less than 1
    nanogram per milliliter (ng/ml) in healthy people. In people
    with GH overproduction, this suppression does not occur.
    • Elevated IGF-I levels almost always indicate acromegaly.
    However, a pregnant woman’s IGF-I levels are two to three
    times higher than normal. Levels lower with age and in
    uncontrolled diabetes.
    • Imaging
    • CT scan
    • MRI
    • If a head scan fails to detect a pituitary tumor, the physician
    should look for non-pituitary “ectopic” tumors in the chest,
    abdomen, or pelvis as the cause of excess GH.
    Management
    • Goals of Tx:
    • reduce excess hormone production to normal levels
    • relieve the pressure that the growing pituitary tumor may be
    exerting on the surrounding brain areas
    • preserve normal pituitary function or treat hormone
    deficiencies
    • improve the symptoms of acromegaly
    • Potential treatment options for acromegaly include
    surgery to remove the tumor,
    • and/or medical treatment that either reduces the amount
    of GH that the pituitary tumor secretes or blocks the
    excess circulating GH from binding to receptor sites
    • Surgery:
    • Transsphenoidal surgery; Surgery is most successful in
    patients with blood GH levels below 5 ng/ml before the
    operation and with pituitary tumors no larger than 10
    millimeters (mm) in diameter.
    • A possible complication is damage to the surrounding normal
    pituitary tissue, requiring lifelong hormone replacement. The
    part of the pituitary that stores antidiuretic hormone may be
    temporarily or, rarely, permanently damaged.
    • Other potential problems include cerebrospinal fluid leaks
    and, rarely, meningitis.
    Medical Managment
    MOA Type of Medication Name
    Directly inhibit GH Somatostatin Octreotide, Lanreotide
    secretion analogs(IM)
    Dopamine agonists(oral) Bromocriptine,
    Cabergoline

    MOA Type of Medication Name

    Blocks GH receptor GH receptor antagonist Pegvisomant


    • There is only one medical treatment option that works by
    blocking the actions of GH on the liver, thereby lowering
    IGF-1 levels in the circulation.
    • The GH receptor antagonist, pegvisomant, decreases IGF-
    1 levels in more than two-thirds of patients and is
    generally well tolerated. It is administered daily by
    injection and side effects include liver function
    abnormalities and infrequent development of fatty tissue
    deposits under the skin.
    radiation
    • therapy is usually reserved for people who have some tumor
    remaining after surgery and do not respond to medications.
    Because radiation leads to a slow lowering of GH and IGF-I
    levels, these patients often also receive medication to lower
    hormone levels
    • Conventional and stereotactic.

    • Conventional radiation delivery targets the tumor with external


    beams but can damage surrounding tissue. The treatment
    delivers small doses of radiation multiple times over 4 to 6
    weeks, giving normal tissue time to heal between treatments
    • Stereotactic delivery allows precise targeting of a high-
    dose beam of radiation at the tumor from varying angles.
    proton beam, linear accelerator (LINAC), and gamma
    knife.
    • With stereotactic delivery, the tumor must be at least 5
    mm from the optic chiasm to prevent radiation damage.
    References

    • National Endocrine and Metabolic Diseases


    Information Service. HATIONAL INSTITUTE OF
    HEALTH,
    • THE PITUITARY SOCIETY.

    • KUMAR AND CLERK.

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