TY  - JOUR
A1  - Nowotny, Hanna
A1  - Ahmed, S. Faisal
A1  - Bensing, Sophie
A1  - Beun, Johan G.
A1  - Brösamle, Manuela
A1  - Chifu, Irina
A1  - Claahsen van der Grinten, Hedi
A1  - Clemente, Maria
A1  - Falhammar, Henrik
A1  - Hahner, Stefanie
A1  - Husebye, Eystein
A1  - Kristensen, Jette
A1  - Loli, Paola
A1  - Lajic, Svetlana
A1  - Reisch, Nicole
T1  - Therapy options for adrenal insufficiency and recommendations for the management of adrenal crisis
JF  - Endocrine
N2  - Adrenal insufficiency (AI) is a life-threatening condition requiring life-long glucocorticoid (GC) substitution therapy, as well as stress adaptation to prevent adrenal crises. The number of individuals with primary and secondary adrenal insufficiency in Europe is estimated to be 20–50/100.000. A growing number of AI cases are due to side effects of GC treatment used in different treatment strategies for cancer and to immunotherapy in cancer treatment. The benefit of hormone replacement therapy is evident but long-term adverse effects may arise due to the non-physiological GC doses and treatment regimens used. Given multiple GC replacement formulations available comprising short-acting, intermediate, long-acting and novel modified-release hydrocortisone as well as subcutaneous formulations, this review offers a concise summary on the latest therapeutic improvements for treatment of AI and prevention of adrenal crises. As availability of various glucocorticoid formulations and access to expert centers across Europe varies widely, European Reference Networks on rare endocrine conditions aim at harmonizing treatment and ensure access to specialized patient care for individual case-by-case treatment decisions. To improve the availability across Europe to cost effective oral and parenteral formulations of hydrocortisone will save lives.
KW  - adrenal insufficiency
KW  - congenital adrenal hyperplasia
KW  - adrenal crisis
KW  - glucocorticoid replacement
KW  - hydrocortisone
KW  - stress instructions
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-308769
SN  - 1355-008X
SN  - 1559-0100
VL  - 71
IS  - 3
ER  - 
TY  - JOUR
A1  - Zopf, Kathrin
A1  - Frey, Kathrin R.
A1  - Kienitz, Tina
A1  - Ventz, Manfred
A1  - Bauer, Britta
A1  - Quinkler, Marcus
T1  - \(Bcl\)I polymorphism of the glucocorticoid receptor and adrenal crisis in primary adrenal insufficiency
JF  - Endocrine Connections
N2  - Context: Patients with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) are at a high risk of adrenal crisis (AC). Glucocorticoid sensitivity is at least partially genetically determined by polymorphisms of the glucocorticoid receptor (GR).
Objectives: To determine if a number of intercurrent illnesses and AC are associated with the GR gene polymorphism \(Bcl\)I in patients with PAI and CAH.
Design and patients: This prospective, longitudinal study over 37.7 ± 10.1 months included 47 PAI and 25 CAH patients. During the study period, intercurrent illness episodes and AC were documented.
Results: The study period covered 223 patient years in which 21 AC occurred (9.4 AC/100 pat years). There were no significant differences between \(Bcl\)I polymorphisms (CC (n=29), CG (n=34) and GG (n=9)) regarding BMI, hydrocortisone equivalent daily dose and blood pressure. We did not find a difference in the number of intercurrent illnesses/patient year among \(Bcl\)I polymorphisms (CC (1.5±1.4/pat year), CG (1.2±1.2/pat year) and GG (1.6±2.2/pat year)). The occurrence of AC was not significantly different among the homozygous (GG) genotype (32.5 AC/100 pat years), the CC genotype (6.7 AC/100 pat years) and the CG genotype (4.9 AC/100 pat years). Concomitant hypothyroidism was the highest in the GG genotype group (5/9), compared to others (CC (11/29) and CG (11/34)).
Conclusions: Although sample sizes were relatively small and results should be interpreted with caution, this study suggests that the GR gene polymorphism \(Bcl\)I may not be associated with the frequencies of intercurrent illnesses and AC.
KW  - medicine
KW  - adrenal crisis
KW  - adrenal insufficiency
KW  - cortisol
KW  - hydrocortisone
KW  - polyglandular autoimmune syndrome
Y1  - 2017
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-173276
VL  - 6
IS  - 8
ER  -