Table 1 46,XX* CAH patients with male external genitalia assigned/reared male.
(a) Death during infancy, most with evidence of adrenal insufficiency. | |||
|---|---|---|---|
Year | First author | Reference | Â |
| Krokiewicz | Virchow's Arch path Anat Physiol 146 : 525 |  |
| Fibiger | Virchow's Arch path Anat 181 : 1 |  |
| Benda | Berlin klin. Wschr 51 : 66 |  |
| Lehmann | Ugesk. Laeger 81 : 751 |  |
| Allibone | Arch Dis Child 22 : 210 |  |
| Marie | Arch franc pediat 9 : 785 |  |
| Perloff | J Clin Endocrinol 13 : 78 |  |
| Matheson | Arch Dis Child 29 : 22 |  |
| Rook | Am J Dis Child 101 : 645 |  |
(b) Diagnosed and reported during childhood, reared male. | |||
Year | First author | Reference | Age of diagnosis |
| Gross | Pediatrics 16 : 303 | ? |
| Bentinck | J Clin Endocrinol Metab 16 : 412 | 2 years |
| Wilkins | Dx and Rx of Endocrine Disorders, 2nd | 7.5 years |
| Prader | Helv Paediatr Acta 13 : 5 | 4 years |
| Weldon | N Engl J Med 274 : 829 | 2.5 years |
| Weldon | N Engl J Med 274 : 829 | 3.5 years |
| Redman | J Urol 108 : 500 | 6 years |
| Money | J Homosex 1 : 357 | 1 month |
| Money | J Homosex 1 : 357 | 12 years |
| Gillenwater | J Urol 103 : 500 | 4 years |
| Wyatt | J Pediatr Endocrinol 2 : 35 | 3.6 years |
| Rösler | Adrenal Disease in Childhood 13 : 47 | 1.9 years |
| Rösler | Adrenal Disease in Childhood 13 : 47 | 1 year |
| Rösler | Adrenal Disease in Childhood 13 : 47 | 1.6 years |
| Rösler | Adrenal Disease in Childhood 13 : 47 | 2 years |
| Chan-Cua | Amer J Med Genetics 34 : 406 | 8.2 years |
| Chan-Cua | Amer J Med Genetics 34 : 406 | 4.3 years |
| Sripathi | Brit J Urol 79 : 785 | 10 years |
| Sripathi | Brit J Urol 79 : 785 | 4 years |
| Sripathi | Brit J Urol 79 : 785 | 7 years |
| Sripathi | Brit J Urol 79 : 785 | 11 years |
| Sripathi | Brit J Urol 79 : 785 | 3.5 years |
| Sripathi | Brit J Urol 79 : 785 | 16.6 years |
| Woelfle | Clin Endocrinol 56 : 231 | 3.5 years |
| Woelfle | Clin Endocrinol 56 : 231 | 4.5 years |
| Woelfle | Clin Endocrinol 56 : 231 | 7 years |
(c) Diagnosed during infancy or childhood with reassignment. | |||
Year | First author | Reference | Age of reassignment |
| Ainger | AMA J Dis Child 95 : 410 | 3 years |
| Wiedemann | Monatsschr Kinderheilkd 113 : 434 | 14 months |
| Weldon | N Engl J Med 274 : 829 | 3 weeks |
| Weldon | N Engl J Med 274 : 829 | 8 weeks |
| Weldon | N Engl J Med 274 : 829 | 11 months |
| Rosenberg | N Eng J Med 280 : 131 | 3 weeks |
| Woelfle | Clin Endocrinol 56 : 231 | 2 days |
| Woelfle | Clin Endocrinol 56 : 231 | 1 month |
| Woelfle | Clin Endocrinol 56 : 231 | 1 month |
| Woelfle | Clin Endocrinol 56 : 231 | 1 month |
| Woelfle | Clin Endocrinol 56 : 231 | 1 month |
| Woelfle | Clin Endocrinol 56 : 231 | 19 months |
| Woelfle | Clin Endocrinol 56 : 231 | 7 years |
| Woelfle | Clin Endocrinol 56 : 231 | 9 years |
| Woelfle | Clin Endocrinol 56 : 231 | 11 Years |
(16–24) 2006 | Gupta | Pediatr Surg Int 22 : 905 | not stated |
(d) Diagnosed during childhood, reared male, reported >17 years. | |||
Year | First author | Reference | Age at report |
| Peris | Obstet Gynecol 16 : 156 | 18 years |
| Madsen | J Urol 90 : 446 | 35 years |
| Madsen | J Urol 90 : 446 | 30 years |
| Maxted | J Urol 94 : 266 | 21 years |
| Money | J Homosex 1 : 357 | adult |
| Money | J Homosex 1 : 357 | adult |
| Kiviat | Urol 12 : 75 | 17 years |
(8–15) 1984 | Rösler | Adrenal Disease in Childhood 13 : 47 | 17–33 years |
| Woelfle | Clin Enocrinol 56 : 231 | 36 years |
| Woelfle | Clin Enocrinol 56 : 231 | 31 years |
| Woelfle | Clin Enocrinol 56 : 231 | 50 years |
| Woelfle | Clin Enocrinol 56 : 231 | 24 years |
| Jones | J Pediatr Endocrinol Metab | Died at 31 years |
| Lee | J Urol 184 (suppl 4): 1786 | 35 years |
| Lee | J Urol 184 (suppl 4): 1786 | 36 years |
| Lee | J Urol 184 (suppl 4): 1786 | 45 years |
| Lee | J Urol 184 (suppl 4): 1786 | 46 years |
| Lee | J Urol 184 (suppl 4): 1786 | 47 years |
| Lee | J Urol 184 (suppl 4): 1786 | 49 years |
| Lee | J Urol 184 (suppl 4): 1786 | 49 years |
| Lee | J Urol 184 (suppl 4): 1786 | 53 years |
| Lee | J Urol 184 (suppl 4): 1786 | 57 years |
| Lee | J Urol 184 (suppl 4): 1786 | 69 years |
1896
1905
1914
1919
1947
1952
1953
1954
1961
1955
1956
1957
1958
1966
1966
1972
1976
1976
1970
1987
1984
1984
1984
1984
1989
1989
1997
1997
1997
1997
1997
1997
2002
2002
2002
1958
1965
1966
1966
1966
1969
2002
2002
2002
2002
2002
2002
2002
2002
2002
1960
1963
1963
1965
1976
1976
1978
2002
2002
2002
2002
2004
2010
2010
2010
2010
2010
2010
2010
2010
2010
2010
for those reported before the ability to determine the presence of an XX karyotype; this karyotype is presumed based upon the presence of differentiation consistent with this, either from autopsy findings or surgical observations.
those with reported gender problems.
for those reported before the ability to determine the presence of an XX karyotype; this karyotype is presumed based upon the presence of differentiation consistent with this, either from autopsy findings or surgical observations.
those with reported gender problems.