Acromegaly resident survival guide

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Tayyaba Ali, M.D.[2]

Overview

This section provides a short and straight to the point overview of the disease or symptom. The first sentence of the overview must contain the name of the disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated.

Common Causes

Diagnosis

The approach to diagnosis of Acromegaly is based on a step-wise testing strategy. Below is an algorithm summarising the identification and laboratory diagnosis of Acromegaly.[1]


❑ Skin exam:
  • Thickening of the skin
  • Skin tags
  • Acne
  • Acanthosis nigricans
  • Hyperhidrosis}}
  •  
     
     
     
     
     
     
     
     
     
     
     
    Characterize the symptoms:
    Headaches
    ❑ Enlargement of the hands (change in ring or glove size) and feet (change in shoe size)
    Lethargy
    Hyperhidrosis (excessive sweating)
    Paraesthesia
    Sexual dysfunction [2]
    Fatigue
    Jaw pain
    ❑ Body odor
    Blood in the stool
    Sleep apnea
    Weight gain [3]
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Examine the patient:
    ❑ HEENT

    ❑ Musculoskeletal exam:

    ❑ Neurological exam:

    ❑ Cardiovascular exam:

     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    This algorithm developed and modified according to Endocrine Society (ES): Clinical practice guideline on acromegaly.

    Treatment

    Shown below is an algorithm summarizing the treatment of Acromegaly according the the Endocrine Society (ES): Clinical practice guideline on acromegaly.

     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Transphenoidal surgery
    ❑ Complete resection
    ❑ Tumors that are unresectable, a surgical debulking procedure may be performed followed by medical therapy
     
    Yes
     
    Patient is not a surgical candidate
    ❑ Patient preference
    ❑ High risk due to medical comorbidities
    ❑ Unresectable tumors
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Are the following criteria met postoperatively?
    ❑ Morning serum GH the day after surgery <1ng/ml
    ❑ 12 weeks postoperative:
    • Normal serum IGF-1 (for age and gender)
    • No evidence of residual tumor on pituitary MRI
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Yes
     
    No
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Remission
    ❑ Monitor with annual IGF-1
     
    Is there residual tumor that appears resectable and readily accessible (eg, not invading the cavernous sinus)?
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Perform MRI for clinical or biochemical evidence of recurrence
     
    Medical therapy

    Somatostatin analogs:
    • Preferred regimen (1): Octreotide 50 mcg q8hr subcutaneous as initial dose and 100 mcg q8hr as effective dose.
    • Preferred regimen (2): Lanreotide 90 mg q4week for 3 months every 4 weeks subcutaneous.
    • Preferred regimen (3): Pasireotide 40 mg q4wk intramuscular.
      Dopamine agonists:
    • Preferred regimen (1): Cabergoline 0.25 mg 2x/week orally.
    • Preferred regimen (2): Bromocriptine 1.25-2.5 mg qDay orally.
      GH receptor antagonist:
    • Preferred regimen (1): Pegvisomant 10 mg qDay subcutaneous[1].
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Failure of medical therapy
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
     
    Radiation therapy
    ❑ Stereotactic radiotherapy is most common method
     
     
     
     
     


    This algorithm developed and modified according to Endocrine Society (ES): Clinical practice guideline on acromegaly.

    Do's

    • The content in this section is in bullet points.

    Don'ts

    • The content in this section is in bullet points.

    References

    1. 1.0 1.1 Katznelson L, Laws ER, Melmed S, Molitch ME, Murad MH, Utz A; et al. (2014). "Acromegaly: an endocrine society clinical practice guideline". J Clin Endocrinol Metab. 99 (11): 3933–51. doi:10.1210/jc.2014-2700. PMID 25356808.
    2. 2.0 2.1 2.2 Molitch ME (1992). "Clinical manifestations of acromegaly". Endocrinol Metab Clin North Am. 21 (3): 597–614. PMID 1521514.
    3. 3.0 3.1 3.2 "Acromegaly: MedlinePlus Medical Encyclopedia".


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