Acromegaly E22.0

Clinical manifestation of hyperpituitarism, which is characterized by an overproduction of the pituitary growth hormone (STH=somatotropin, GH= growth hormone) in the anterior pituitary (HVL).

• Aftercompletion of growth age (closed epiphyseal fissures), STH overproduction leads to acromegaly with disproportionate growth of various organs. This is characterized by a selective increase in the size of the acres, in particular the nose, ears, chin, hands and feet.
• Before the end of growth age (open epiphyseal joints), STH overproduction leads to hyophyseal gigantism.

Possible causes are:

• Overproduction of growth hormone (STH = somatotropic hormone), usually due to an eosinophilic adenoma of the anterior lobe of the pituitary gland (>95% of patients).
• Mutations of regulatory genes of adenylate cyclase ( G-proteins of the signal transduction pathway).
• Ectopic, neuroendocrine active tissue: GH-RF stimulates the oncogene c-fos in STH-producing cells in extrahypophyseal tumors (pancreas, lung, ovary, breast).
• Excessive secretion of growth hormone releasing factor (GH-RF): Eutopic in hypothalamic hamartomas, ganglioneuromas, ectopic in tumors (carcinoid, pancreas, small cell carcinoma of the lung, adrenal glands, pheochromocytoma).
• Increased effect or secretion of somatomedins ("acromegalodism").

The symptoms and findings associated with acromegaly are due to:

• local effects of the pituitary adenoma
• growth effects of the STH
• concomitant endocrine diseases

Cutaneous manifestations of acromegaly (image of the "wild man"):

• Coarsening of facial features, thickened and wrinkled facial skin,
• Effluvium, hair trophism disorders (often mild hirsutism in female patients, androgenetic-type alopecia in male patients)
• Hyperhidrosis (especially palmar and plantar)
• Hypertrichosis (in 40-50% of patients)
• Onychodystrophy
• Cutis verticis gyrata
• Hyperpigmentation (in 40-50% of patients)
• Acanthosis nigricans.

Extracutaneous manifestations of acromegaly:

• Weight gain
• General visceromegaly (splanchnomegaly)
• Deeper voice (laryngeal growth), lumpy speech due to macroglossia (can lead to sleep apnea syndrome)
• Skeleton: osteoporosis, arthrosis, kyphosis of the thoracic spine (caused by cartilage growth in joints and intervertebral discs), progenia, supraorbital bulges, enlargement of the hands (claw hands), feet, carpal tunnel syndrome
• Cardiovascular: Frequent heart enlargement (expression of myocardial hypertrophy caused by high growth hormone levels, hypertension and coronary sclerosis with corresponding scarred myocardial changes as subfactors of cardiomegaly; in 25% of cases, manifest heart failure occurs).
• Local-parasellar manifestations: In pituitary tumor: bitemporal hemianopsia and congestive papilla; headache;
• Endocrine disorders: euthyroid goiter (nodosa colloides), amenorrhea, dysmenorrhea, loss of potency (gonadotropic deficiency); pathological glucose tolerance or manifest diabetes mellitus. In the late stages of acromegaly, panhypopituitarism may occur due to the displacement and elimination of the pituitary tissue (hypothyroidism, adrenocortical insufficiency, hypogonadism).
• GI: adenomatous colonic polyps (malignant tumors of the GI tract must be expected in about 10% of patients).

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