Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\29884168
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 BMC+Endocr+Disord
2018 ; 18
(1
): 37
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Mortality in children with classic congenital adrenal hyperplasia and
21-hydroxylase deficiency (CAH) in Germany
#MMPMID29884168
Dörr HG
; Wollmann HA
; Hauffa BP
; Woelfle J
BMC Endocr Disord
2018[Jun]; 18
(1
): 37
PMID29884168
show ga
BACKGROUND: Adrenal crises in children with classic congenital adrenal
hyperplasia due to 21-hydroxylase deficiency (CAH) are life-threatening and have
the potential to death. METHODS: A survey was performed among Paediatric
Endocrinologists in Germany to report on deceased children with CAH. Our survey
covered the whole of Germany. RESULTS: The participating centres reported 14
cases of death (9 female, 5 male) from 1973 until 2004, but no deaths thereafter.
11 children had the SW form and 3 the simple virilizing (SV) form. All patients
were on glucocorticoid replacement, and the SW forms additionally on
mineralocorticoid replacement. The age at death varied between 6 weeks and
16.5 years. Seven children died before introduction of general neonatal
screening, and 7 children thereafter. Before death, the clinical signs of
impending crisis were nonspecific. Five patients developed hypoglycaemia and
convulsions with cerebral oedema. Half of the deceased patients died at home. The
hydrocortisone dosage was only doubled in two of the 14 cases. CONCLUSIONS:
According to the assessments by the attending centres, almost all deaths could be
related to an inadequate administration of stress doses of hydrocortisone. Since
no deceased CAH children were reported in Germany from 2005 on, we assume the
effectiveness of educational programs over the past years.