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.