Acromegaly E22.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 28.12.2023

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Pachyakrie; (Pierre-)Marie syndrome; Pierre Marie syndrome

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Saucerotte, 1772; Marie, 1886

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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.

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Rarely. Incidence: 1/3-5 million inhabitants/year.

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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").

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Predominantly in adulthood, especially 40th-50th LJ.

Clinical features
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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"):

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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Growth hormone STH in the sense of increased, in the OGT no suppression below 2 ng/ml after administration of 100 g glucose. Increased somatomedin (IGF-I) levels, hyperprolactinaemia (in 20-40% of patients). Secondary diabetes mellitus (determine several FC values in the daily profile!). Somatomedin C reduced.

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Clinic; Laboratory; X-ray and CT of the skull: Sella enlargement; X-ray of the paranasal sinuses, hands, feet (enlargement, cortical thickening). Comparison with previous photographs of the patient.

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Treatment of the underlying disease, e.g. tumour removal surgically or by radiotherapy. Otherwise, inhibition of STH secretion with bromocriptine (e.g. Pravidel) or application of octreotide by the endocrinologist.

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  1. Braham C et al (2002) Skin tensile properties in patients treated for acromegaly. dermatology 204: 325-329
  2. Klibanski A, Zervas NT (1991) Diagnosis and management of hormone-secreting pituitary adenomas. N Engl J Med 324: 822-831
  3. Lami MC et al (2003) Hair loss in three patients with acromegaly treated with octreotide. Br J Dermatol 149: 655-656
  4. Marie P (1886) Sur deux cas d'acromégalie: hypertrophy singulière non congénitale des extrémités supérieures, inférieures et céphalique. Rev de Med 6: 297-333
  5. Melmed S (1992) Acromegaly. Endocrinology and Metabolism Clinics of North America 21: 483-765
  6. Melmed S et al (2002) Guidelines for acromegaly management. J Clin Endocrinol Metab 87: 4054-4058
  7. Saucerotte N (1801) Mélanges de surgery. Paris, Vol. 1, p. 477
  8. Umahara M et al (2003) Glucose-Dependent Insulinotropic Polypeptides Induced Growth Hormone Secretion in Acromegaly. Endocr J 50: 643-650
  9. Verstraeten (1889) L'acromégalie. Rev méd 9: 377-493
  10. Wass JA (2003) Dynamic testing in the diagnosis and follow-up of patients with acromegaly. J Endocrinol Invest 26: 48-53


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Last updated on: 28.12.2023