NAME syndrome

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Classification according to ICD-11
2F01 Benign neoplasm of intrathoracic organs
ICD-11 ( WHO version 2019)

The NAME syndrome is a rare disease in the atria of the skin lesions and by a combination of (so called) benign tumors heart distinguished. Symptoms consist of heart failure and embolism of the arterial circulation in people with freckles and can be quite malignant.

The syndrome from the group of familial myxomas was first described in 1980.

The eponymous term is an acronym for a clinical syndrome .

NAME stands for

Nevus ,
Atrial ( atrial ) - myxoma ,
Myxoid neurofibroma and
Ephelids ( freckles ).

Therapy consists in the surgical removal of the myxomas of the heart, and rarely also the fibromas of the skin.

Possibly the NAME syndrome and also the LAMB syndrome are variants of the Carney complex with only latent hyperfunction of the endocrine glands.

Individual evidence

  1. David J. Atherton, DW Pitcher, RS Wells, DM MacDonald: A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata, and atrial myxoma. The NAME syndrome. In: British Journal of Dermatology , Vol. 103 (1980), No. 4, pp. 421-429, ISSN  0007-0963
  2. Manfred Dietel , Norbert Suttorp, Martin Zeitz (eds.); Dennis C. Kasper, Eugene Braunwald, Anthony Fauci , Stephen L. Hauser, Dan L. Longo, J. Larry Jameson (Eds.): Harrison's Internal Medicine. German edition in cooperation with the Charité ("Harrison's principles of internal medicine"). 16th edition McGraw Hill, ABW-Wissenschaftsverlag, Berlin 2005, ISBN 3-936072-29-9 (2 vols.).
  3. ^ Douglas P. Zipes, Peter Libby , Robert O. Bonow, Eugene Braunwald (eds.): Braunwald's Heart Disease. A Textbook of Cardiovascular Medicine. 7th edition Elsevier Saunders, Philadelphia 2005, ISBN 0-8089-2305-6 (+ 1 CD-ROM).