hrp0082p1-d2-40 | Bone | ESPE2014

Effects and Limitations of Cinacalcet Therapy in Neonatal Severe Hyperparathyroidism

Doehnert Ulla , Goepel Wolfgang , Hoeppner Wolfgang , Hiort Olaf

Background: Neonatal severe hyperparathyroidism (NSHPT) has been associated with inactivating mutations of the calcium-sensing receptor (CASR) gene. Impaired inhibition of PTH secretion by extracellular ionized calcium and decreased urinary excretion of calcium leads to severe hypercalcemia in the first days of life. Calcium responsiveness of the CaSR is amplified by type 2 calcimimetic agents like cinacalcet, which has been able to normalize PTH and calcium levels in cases of...

hrp0097p1-178 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Inhibin B- a functional marker to screen gonadal function in CAIS patients?

Viola Wagner Isabel , Tyutyusheva Nina , Bertelloni Silvano , Doehnert Ulla , Simon Frielitz Fabian , Hiort Olaf

Introduction and objective: Gonadectomy was carried out for a long time after the diagnosis of complete androgen insensitivity syndrome (CAIS). It is now recommended to leave the gonads in situ in order to guarantee endogenous hormone production. It is unclear how best to clinically monitor testicular function. The aim of the study was to investigate whether inhibin B can be used as a future follow-up parameter to screen for gonadal function in CAIS p...