Acromegaly

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Acromegaly (pronounced /ˌækrɵˈmɛɡəli/; from Greek άκρος akros "extreme" or "extremities" and μεγάλος megalos "large") is a syndrome that results when the pituitary gland produces excess growth hormone (hGH) after epiphyseal plate closure at puberty. A number of disorders may increase the pituitary's GH output, although most commonly it involves a GH producing tumor called pituitary adenoma, derived from a distinct type of cell (somatotrophs).

Acromegaly most commonly affects adults in middle age,[1] and can result in severe disfigurement, serious complicating conditions, and premature death if unchecked. Because of its insidious pathogenesis and slow progression, the disease is hard to diagnose in the early stages and is frequently missed for many years, until changes in external features, especially of the face, become noticeable.

Acromegaly is often also associated with gigantism.

Contents

Signs and symptoms

Features that result from high level of GH or expanding tumor include:

  • Soft tissue swelling visibly resulting in enlargement of the hands, feet, nose, lips and ears, and a general thickening of the skin. In particular the appearance of the hands can indicate to a knowledgeable person that a stranger may be developing acromegaly; there are documented instances of physicians warning strangers that they had acromegaly.[2][3]
  • Soft tissue swelling of internal organs, notably the heart with attendant weakening of its muscularity, and the kidneys, also the vocal cords resulting in a characteristic thick, deep voice and slowing of speech
  • Generalized expansion of the skull at the fontanelle
  • Pronounced brow protrusion, often with ocular distension
  • Pronounced lower jaw protrusion with attendant macroglossia (enlargement of the tongue) and teeth gapping
  • Hypertrichosis, hyperpigmentation, and hyperhidrosis may occur in these patients.[4]:499

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