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. 2021 Jul 8;385(2):189-191.
doi: 10.1056/NEJMc2034981.

PTH Infusion for Seizures in Autosomal Dominant Hypocalcemia Type 1

Ana Sastre  1 , Kevin Valentino  2 , Fadil M Hannan  3 , Kate E Lines  3 , Anna K Gluck  3 , Mark Stevenson  3 , Michael Ryalls  4 , Rebecca J Gorrigan  1 , Debbie Pullen  5 , Jackie Buck  6 , Sailesh Sankaranarayanan  7 , Jeremy Allgrove  8 , Rajesh V Thakker  9 , Evelien F Gevers  10
Affiliations

PTH Infusion for Seizures in Autosomal Dominant Hypocalcemia Type 1

Ana Sastre et al. N Engl J Med. .
No abstract available

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Figures

Figure 1
Figure 1. Clinical findings and responses to PTH infusion in ADH1 patients.
Panel A shows clinical characteristics and treatments for all six unrelated ADH1 patients (P1-P6). aM, male; F, female. bS, seizures; NC, nephrocalcinosis; RI, renal impairment; BC, bilateral cataracts; BS, Bartter syndrome; H, hyperinsulinism. cReceived phenobarbitone anticonvulsant therapy. df, familial; dn, de novo; c, constitutively active. eNormal ranges: adjusted-calcium, 2.20-260 mmol/L; phosphate, 1.30-2.60 mmol/L (<1 month), 1.30-2.40 mmol/L (1 month-1 year), 0.90-1.80 mmol/L (1-16 years), 0.80-1.50 mmol/L (>16 years); magnesium, 0.70-1.0 mmol/L; creatinine, 27-77 μmol/L (<1 month), 14-34 μmol/L (<1 year), 15-31 μmol/L (1-3 years), 23-37 μmol/L (3-5 years), 25-42 μmol/L (5-7 years), 30-47 μmol/L (7-9 years), 29-56 μmol/L (9-11 years), 39-60 μmol/L (13-15 years), 40-68 μmol/L (>15 years); parathyroid hormone (PTH) 1.6-6.9 pmol/L; urine calcium: creatinine (Ca:Cr) ratio, <1.50 mmol/mmol (0-1 years), <1.25 mmol/mmol (1-2 years), <1.0 mmol/mmol (2-5 years), <0.70 mmol/mmol (5-10 years), <0.60 mmol/mmol (10-18 years). fCSPI, continuous subcutaneous PTH(1-34) infusion; NA, not available; gMg, magnesium; K, potassium; T, thiazide, Di, diazoxide; Do, doxazosin; Fe, ferrous fumarate; Epo, erythropoietin. All children were taking cholecalciferol or ergocalciferol. Panel B shows effects of CSPI compared to prior therapy with oral calcium supplements and vitamin D analogs (Ca+D) on serum concentrations of adjusted-calcium; phosphate; calcium-phosphate product (Ca x Pi); and magnesium. Number of serum biochemical values per treatment group in each patient (P1-P6) are shown below respective box and whisker plot. Panel C shows effects of CSPI compared to prior therapy with Ca+D (Pre-CSPI) on numbers of seizures per month and hospital admissions per month, in six ADH1 patients (P1-P6).
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References

    1. Pearce SH, Williamson C, Kifor O, et al. A familial syndrome of hypocalcemia with hypercalciuria due to mutations in the calcium-sensing receptor. N Engl J Med. 1996;335:1115–22. - PubMed
    1. Hannan FM, Kallay E, Chang W, Brandi ML, Thakker RV. The calcium-sensing receptor in physiology and in calcitropic and noncalcitropic diseases. Nat Rev Endocrinol. 2018;15:33–51. - PMC - PubMed
    1. Raue F, Pichl J, Dorr HG, et al. Activating mutations in the calcium-sensing receptor: genetic and clinical spectrum in 25 patients with autosomal dominant hypocalcaemia - a German survey. Clin Endocrinol (Oxf) 2011;75:760–5. - PubMed
    1. Winer KK, Zhang B, Shrader JA, et al. Synthetic human parathyroid hormone 1-34 replacement therapy: a randomized crossover trial comparing pump versus injections in the treatment of chronic hypoparathyroidism. J Clin Endocrinol Metab. 2012;97:391–9. - PMC - PubMed
    1. Winer KK, Fulton KA, Albert PS, Cutler GB., Jr Effects of pump versus twice-daily injection delivery of synthetic parathyroid hormone 1-34 in children with severe congenital hypoparathyroidism. J Pediatr. 2014;165:556–63.:e1. - PMC - PubMed

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