Acromegaly E22.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 13.10.2022

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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 characterized by overproduction of pituitary growth hormone (STH=somatotropin, GH= growth hormone) in the anterior pituitary lobe (HVL).

  • After completion of the growth age (closed epiphyseal gaps), STH overproduction leads to the picture of acromegaly with disproportionate growth of various organs. Characteristic is a selective increase in size of the acras, especially the nose, ears, chin, hands and feet.
  • Before completion of the 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 pituitary (>95% of patients).
  • Mutations of regulatory genes of adenylate cyclase ( G proteins of the signal transduction pathway).
  • Ectopic neuroendocrine active tissue: GH-RF stimulates oncogene c-fos in STH-producing cells in extrahypophyseal tumor (pancreas, lung, ovary, mamma).
  • Excessive secretion of growth hormone releasing factor (GH-RF): Eutop in hypothalamic hamartomas, ganglioneuromas, ectop in tumors (carcinoid, pancreatic, small cell carcinoma of lung, adrenal, pheochromocytoma).
  • Increased action 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 seen in acromegaly are due to:

  • local effects of the hypophyenadenoma
  • growth effects of the STH
  • endocrine concomitant diseases

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

Extracutaneous manifestations of acromegaly:

  • Weight gain
  • Generalized visceromegaly (splanchnomegaly).
  • Deeper voice (laryngeal growth), slurred speech due to macroglossia (may lead to sleep apnea syndrome).
  • Skeletal: Osteoporosis, osteoarthritis, thoracic spine kyphosis (due to cartilage growth on joints and discs), progeny, supraorbital bulges, enlargement of hands (pratate hands), feet, carpal tunnel syndrome, .
  • Cardiovascular: Frequently cardiac enlargement (expression of myocardial hypertrophy caused by high growth hormone levels, hypertension and coronary sclerosis with corresponding scarring myocardial changes as partial factors of cardiomegaly. Manifest heart failure occurs in 25% of cases.
  • Local parasellar manifestations: In pituitary tumor: bitemporal hemianopsia and congestive papilla; headache;
  • Endocrine disorders: euthyroid goiter (nodosa colloides), amenorrhea, dysmenorrhea, loss of potency (gonadotropin deficit); pathologic glucose tolerance or manifest diabetes mellitus. In the late stage of acromegaly, the occurrence of panhypopituitarism is possible due to displacement and elimination of 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: 13.10.2022