Table 1.
The diagnostic dyad† and the most common disease components and median age ranges at their appearance in the series of 91 APECED patients studied (modified from [48]).
| Age range (median) at appearance (years) | |
|---|---|
| Diagnostic dyad† | 2·2–35 (7·0) |
| Classic triad | |
| •Candidiasis | 0·2–31 (5·4) |
| •Hypoparathyroidism | 1·6–43 (6·0) |
| •Adrenal failiure | 3·5–41 (10·0) |
| •All three | 3·5–43 (11·3) |
| Other endocrine disorders | |
| •Ovarian failure | −36 |
| •Testicular failure | −37 |
| •Diabetes mellitus | 4·1–58 (23·5) |
| •Hypothyroidism | 4·7–45 (26·5) |
| Skin disorders | |
| •Alopecia | 2·5–30 (10·3) |
| •Vitiligo | 0·7–45 (12·7) |
| •Rash with fever | 0·7–31 (2·8) |
| Gastrointestinal disorders | |
| •Pernicious anaemia | 6·1–48 (17·2) |
| •Severe obstipation | 1·0–31 (13·0) |
| •Chronic diarrhoea | 2·5–27 (6·6) |
| •Hepatitis | 0·7–16 (8·0) |
| Eye disorders | |
| •Keratoconjunctivitis | 1·3–16 (5·4) |
Estimated from the observed incidence rates over the age intervals, assuming that all patients live until the age of 50 years.
†
At least two of candidiasis, hypoparathyroidism and adrenocortical failure.