Acromegaly

1- Clinical diagnosis:

A- Hypersecretion of GH:

* Morphological transformations of progressive appearance:

– Thickening of the skin, lips, nose, macroglossia.

– Sweats, hyperseborrhea.

– Spacing teeth, undershot, protruding brows.

– Hypoaccousie, hoarseness, snoring, sleep apnea.

– Enlargement of the extremities: hands (sign of the ring) and feet.

– Carpal tunnel syndrome.

– Enlargement of the thorax, thoracic kyphosis, lymphadenopathy, back pain (spinal stenosis).

– Spanchnomégalie, goiter.

* Cardiovascular Manifestations that make the disease severity:

– HTA.

– Hypertrophic cardiomyopathy.

B- Tumor signs:

– Headache.

– Amputation of the visual field: bitemporal hemianopia with visual field examination.

– Gynecomastia and impotence in men (hyperprolactinemia disconnection or mixed secretion GH and prolactin, gonadotropin deficiency.

– Failure Signs antéhypophysaire more or less complete by tumor invasion of the pituitary gland.

2- Laboratory confirmation of diagnosis:

* Brake test of GH under OGTT:

-> Brake Absence of GH in OGTT (N: Nadir GH <0.3 mg / L or 0,9mUl / L).

* Elevation of IGF1 (insulin growth factor I).

3- Other laboratory abnormalities:

* Metabolic events related to hypersecretion of GH:

– Glucose intolerance, diabetes.

– Abnormal lipid profile, mixed hyperlipidemia.

– Hyperphosphatemia, hypercalciuria.

* Hyperprolactinaemia disconnection or mixed GH and prolactin.

* Biological signs of hypopituitarism related to pituitary tumor syndrome.

4- Morphological examinations:

* Radiographic signs of hypersecretion of GH:

-> Thickening of the cranial vault.

-> Pneumatisation sinuses.
-> Hypertrophy of the external occipital protuberance.

-> Look at tuft phalanges.

-> Thickening footpad.

* Pituitary MRI:

-> Visualize adenoma: hypointense on T1 enhancement to Gadolinium.

-> Specifies the extension (cavernous sinus) and has a prognostic value.

Once the diagnosis is made, it must carry out an assessment of the impact.

5- Total complications:

– Cardiac Ultrasound: study of diastolic function, LVEF.

– Polysomnography in search of a sleep apnea syndrome (SAS).

– Total Colscopie looking for colorectal polyps.

– Abdominal ultrasound looking for gallstones and / or kidney.

– Total other pituitary axes:

+ FT3, FT4, TSHus, E2 prolactin, testosterone, FSH, LH;

+ Test synacthen on cortisol.

– Assessment of diabetes.

6- Treatment:

A- Surgical treatment: adenomectomy transsphenoidal route

– Result evaluated the dosing of GH under OGTT postoperatively and on the rate of IGF1.

– Healing is rarely obtained when macroadenoma with extension to the cavernous sinus requiring further treatment.

B- Medical Further treatment:

* Dopamine agonists in responders:

– Cabergoline (Dostinex)

+ 0.5 to 4.5 mg / week

+ If dosage> 1 mg / week: Split in 2 doses;

+ If dose> 2 mg / week cardiac ultrasound monitoring.

– Bromocriptine (PARLODEL endocrinology and neurology 2.5mg tablet):

+ 2.5 mg to 15 mg / day.

* Somatostatin analogues: octreotide (Sandostatin LP 10mg, 20 mg, 30 mg injectable suspension) lanreotide (SOMATULINE LP 60 mg, 90 mg, 120 mg):

– Sandostatin LP:

+ 1 injection of 20 or 30 mh every 4 weeks in IM.

+ Adaptation of psologie based on GH and IGF1.

– SOMATULINE LP:

+ 1 injection of 60 to 120 mg / 28 days IM.

– Side effects: digestive disorders especially diarrhea, risquede cholelithiasis.

* In the resistant forms: antagonists of GH:

– Pegvisomant (SOMAVERT 10 mg, 15 mg, 20 mg powder and solvent for solution for injection =.

* Radiotherapy pituitary 45 gray:

– Action for long time, the risk of anterior pituitary insufficiency sequelae.

– Infringing visual pathways and brain radiation necrosis.