Accentuated hyperparathyroidism in type II Bartter syndrome

Daniel Landau*, Evgenia Gurevich, Levana Sinai-Treiman, Hannah Shalev

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Bartter syndrome (BS) may be associated with different degrees of hypercalciuria, but marked parathyroid hormone (PTH) abnormalities have not been described. Methods: We compared clinical and laboratory data of patients with either ROMK-deficient type II BS (n = 14) or Barttin-deficient type IV BS (n = 20). Results: Only BS-IV patients remained mildly hypokalemic in spite of a higher need for potassium supplementation. Estimated glomerular filtration rate (eGFR) was mildly decreased in only four BS-IV patients. Average PTH values were significantly higher in BS-II (160.6 ± 85.8 vs. 92.5 ± 48 pg/ml in BS-IV, p = 0.006). In both groups, there was a positive correlation between age and log(PTH). Levels of 25(OH) vitamin D were not different. Total serum calcium was lower (within normal limits) and age-related serum phosphate (Pi)-SDS was increased in BS-II (1.19 ± 0.71 vs. 0.01 ± 1.04 in BS-IV, p < 0.001). The GFR threshold for Pi reabsorption was higher in BS-II (5.63 ± 1.25 vs. 4.36 ± 0.98, p = 0.002). Spot urine calcium/creatinine ratio and nephrocalcinosis rate (100 vs. 16 %) were higher in the BS-II group. Conclusions: PTH, serum Pi levels, and urinary threshold for Pi reabsorption are significantly elevated in type II vs. type IV BS, suggesting a PTH resistance state. This may be a response to more severe long-standing hypercalciuria, leading to a higher rate of nephrocalcinosis in BS-II.

Original languageEnglish
Pages (from-to)1085-1090
Number of pages6
JournalPediatric Nephrology
Volume31
Issue number7
DOIs
StatePublished - 1 Jul 2016
Externally publishedYes

Keywords

  • Bartter syndrome
  • Barttin
  • Hypercalciuria
  • Nephrocalcinosis
  • Parathyroid hormone
  • ROMK

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