Exp Clin Endocrinol Diabetes 2015; 123 - P01_01
DOI: 10.1055/s-0035-1547615

Teriparatide as a novel therapeutic option in the treatment of ADH – A case report

B Schellhaas 1, B Mayr 2, HG Dörr 3, C Schoefl 2
  • 1Medizinische Klinik 1; Universitätsklinikum Erlangen
  • 2Division of Endocrinology and Diabetes, Department of Medicine I, University Hospital Erlangen, Friedrich-Alexander University
  • 3Kinder- und Jugendklinik der Universität; Pädiatrische Endokrinologie und Diabetologie; Pädiatrische Endokrinologie und Diabetologie

Context:

Activating mutations in the calcium sensing receptor (CaSR) gene cause autosomal-dominant hypoparathyroidism (ADH). Low serum (S) calcium (Ca), inadequately low PTH, and inappropriately high urinary (U) Ca excretion are typical, predisposing these patients to seizures and chronic renal failure. Symptomatic hypocalcemia is generally treated with calcium and vitamin D, which, however, may worsen hypercalciuria and renal complications. Recent evidence suggests beneficial effects of PTH therapy in hypoparathyroidism.

Objectives:

To investigate therapeutic effects of PTH in an ADH patient

Methods:

A 29-year-old male ADH patient (CaSR mut. Exon 4 Pro221Leu) was treated with teriparatide (rhPTH1 – 34) 2 or 3 × 20 µg s.c. daily for 32 days. Parameters of calcium homeostasis, bone turnover, cAMP and quality of life (SF-36) were assessed.

Results:

Baseline S-Ca, S-phosphate (PO4), PTH, U-PO4, and CaCl/CreaCl were 2.0 mmol/L, 1.77 mmol/L, < 3 pg/mL, 10.6 mmol/L and 0.013 under therapy with daily vitamin D (2000 I.E.). Treatment with rhPTH1 – 34 2 × 20 µg s.c. daily for 10 days had no effect on S-Ca, but decreased S-PO4 (1.52 mmol/L) and increased U-PO4 (18.7 mmol/L) and U-Ca excretion (CaCl/CreaCl 0.021). Increasing rhPTH1 – 34 to 3 × 20 µg daily caused a marked rise in S-Ca (max. 2.7 mmol/L), further decreased S-PO4 (min. 0.97 mmol/L), doubled U-PO4 (max 25.5 mmol/L), and increased the CaCl/CreaCl to 0.024. Upon rhPTH1 – 34 treatment, 1,25-vitamin D3 and U-cAMP increased, markers of bone formation (P1NP, OC) and resorption (U-pyridinolines) rose in a dose-dependent manner, and QoL (SF-36) improved.

Conclusion:

Teriparatide normalised S-Ca und S-phosphate, caused a marked increase in markers of bone turnover, and improved QoL, but failed to prevent hypercalciuria. Thus, therapy with rhPTH may offer additional benefits to patients with ADH compared to conventional vitamin D and calcium supplementation.