Hypoparathyroidism (HypoPT) is the only hormone deficiency syndrome whose standard treatment is not based on the replacement of the missing hormone. Although most cases of postsurgical HypoPT can be effectively managed with the conventional use of oral calcium and active vitamin D (SOC therapy), some patients require very high doses and develop complications such as hypercalciuria, renal stones, nephrocalcinosis and ectopic calcifications. In the last few years, recombinant human PTH (rhPTH) has become an appealing option for patients affected by chronic HypoPT who are refractory to SOC therapy. Winer et al have adapted an insulin pump delivery system to treat patients with a continuous infusion of rhPTH (134) (teriparatide). We describe the case of a 33-years old woman who was referred to our Clinic for severe chronic HypoPT after total thyroidectomy for multinodular goiter. Despite being on therapy with high doses of oral calcium and active vitamin D, she reported muscle spasms, cramps and perioral paresthesia on a daily basis and was frequently admitted to the Emergency Room to be treated with intravenous calcium infusion. At our evaluation, we confirmed a severe hypocalcemia (7.5 mg/dl), hyperphoshatemia (5.2 mg/dl) and hypercalciuria (388 mg/24 h). The abdomen ultrasound showed the presence of medullary nephrocalcinosis and right microlithiasis. After using teriparatide 20 mcg twice-daily injection without success, we decided to treat the patient with a continuous subcutaneous perfusion of rhPTH (134) by using an insulin pump. rhPTH (134) (Terrosa®, 20 μg/80 μL) was diluted with distilled water and the patient start continuous administration of subcutaneous rhPTH (134) via a Medtronic® pump with a rate of 0.8 UI/h (equivalent to 11 mcg of teriparatide/day). The patient was monitored by daily clinical evaluation (symptoms and Chvostek and Trousseau signs) and assessment of serum calcium and ionized calcium levels. Moreover, we assessed the patients quality of life by using the SF-36 questionnaire at baseline and at 6 months. The daily dose was progressively uptitrated until 22 mcg/day and the SOC therapy was gradually reduced until discontinuation. At the last evaluation (9 months) serum calcium was 9.5 mg/dl, serum phosphate was 3.3 mg/dl and 24 h urine calcium was 100 mg/24 h. Abdomen ultrasound did not show the nephrolithiasis. The SF-36 test showed a significant improvement of the scores. In conclusion, the continuous infusion of rhPTH (134) in our patient was the only treatment option able to restore long-term calcium homeostasis and improve the quality of life.
21 May 2022 - 24 May 2022