Steroid 21-hydroxylase autoantibodies

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

21-hydroxylase antibody; NNR antibody; steroid 21-hydroxylase antibody

Definition
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Antibodies against steroid 21-hydroxylase, which occur mainly in Addison's disease (E27.1) with autoimmune etiology (approx. 80% of cases) and in autoimmune polyendocrinopathies (E31.0) and are of great diagnostic importance for these diseases.

General information
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The antibody titers show no correlation to the course of the disease.

Note(s)
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If NNR antibodies are present, additional tests for marker antibodies (e.g. against thyroid, parathyroid, pancreas, and other organ-specific antibodies) may be indicated. Determination from serum, EDTA or heparin plasma.

Literature
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  1. Bansal S et al (2015) Presence of 21-hydroxylase Antibodies in a Boy with X-Linked Adrenal Hypoplasia Congenita. Horm Res Paediatr 84:408-413.
  2. Dörr HG et al (2018) Mortality in children with classic congenital adrenal hyperplasia and 21-hydroxylase deficiency (CAH) in Germany. BMC Endocr Disord 18:37.

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Last updated on: 29.10.2020