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Table 1 Clinical data of children with CAH who died between 1973 and 2004 in Germany

From: Mortality in children with classic congenital adrenal hyperplasia and 21-hydroxylase deficiency (CAH) in Germany

Cases

CAH Form

Sex

Age at Death

Symptoms

Place of death

Comments

1

SW

F

6 wks.

fever, vomiting

at home

no GC increase

2 a

SW

M

2.5 yrs.

fever, respiratory infection

at home

cerebral oedema

3

SW

M

3.5 yrs.

fever, seizure

tertiary centre

cerebral oedema

4 a

SV

F

5.0 yrs.

fever, seizure

tertiary centre

cerebral oedema

5

SW

F

2.5 yrs.

fever, vomiting

at home

no GC increase

6

SV

F

16 yrs.

gastroenteritis, salmonella

at home

no GC increase

7

SW

F

1 yr.

fever, dehydration

secondary centre

no GC increase

8

SW

M

13 yrs.

fever, vomiting, seizure

secondary centre

no GC increase

9 a

SW

F

10 mo.

fever, MCAD deficiency

at home

unclear?, SIDS?

10 a

SW

F

3 yrs.

fever, gastroenteritis, diarrhoea

secondary centre

cerebral oedema

11

SW

M

7 yrs.

fever, seizure

tertiary centre

no GC increase cerebral oedema

12

SW

M

16.5 yrs.

fever, chicken pox

at home

no GC increase

13

SW

F

2.5 yrs.

fever, vomiting

tertiary centre

no GC increase

14

SV

F

1.5 yrs.

fever, vomiting

at home

no GC increase

  1. Autopsya; performed on 4 children
  2. Case 8: Addisonian crisis at the age of 18 months; since that event: persistent vegetative state (children’s home)
  3. Case 9: MCAD = Medium-Chain-Acyl-CoA-Dehydrogenase
  4. Abbreviations: SW salt wasting, SV simple virilizing, GC glucocorticoid dose, SIDS sudden infant death syndrome