Literature DB >> 2605794

Familial Cushing's syndrome due to nodular adrenocortical dysplasia is an inherited disease of immunological origin.

F Teding van Berkhout1, R J Croughs, N M Wulffraat, H A Drexhage.   

Abstract

Circulating immunoglobulins may be involved in the pathogenesis of familial Cushing's syndrome due to nodular adrenocortical dysplasia. The disease may also be part of a complex including cutaneous and cardiac myxomas, spotty pigmentation of the skin, and other signs of endocrine overactivity. In the present study of two sisters treated by complete adrenalectomy for Cushing's syndrome due to nodular adrenocortical dysplasia, and of their first-degree relatives, no evidence of associated disorders was present. However, the serum of both girls and of their mother contained immunoglobulins capable of stimulating adrenal DNA synthesis and cortisol production in vitro. The study supports the theory that familial Cushing's syndrome due to adrenocortical dysplasia is an inherited disease of immunological origin.

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Year:  1989        PMID: 2605794     DOI: 10.1111/j.1365-2265.1989.tb01241.x

Source DB:  PubMed          Journal:  Clin Endocrinol (Oxf)        ISSN: 0300-0664            Impact factor:   3.478


  3 in total

1.  Encyclopaedia of tumour-associated familial disorders. Part I: from AIMAH to CHIME syndrome.

Authors:  Rolf H Sijmons
Journal:  Hered Cancer Clin Pract       Date:  2008-02-15       Impact factor: 2.857

2.  Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2.

Authors:  C A Stratakis; J A Carney; J P Lin; D A Papanicolaou; M Karl; D L Kastner; E Pras; G P Chrousos
Journal:  J Clin Invest       Date:  1996-02-01       Impact factor: 14.808

3.  Primary pigmented nodular adrenocortical dysplasia: a rare form of a rare disorder.

Authors:  A Jabbar; D Grant; M Savage; A Grossman
Journal:  J R Soc Med       Date:  1994-02       Impact factor: 18.000

  3 in total

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