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Accentuated hyperparathyroidism in type II Bartter syndrome

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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.

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Abbreviations

BS:

Bartter syndrome

GFR:

Glomerular filtration rate

eGFR:

Estimated GFR

Pi:

Inorganic phosphorus

Pi-SDS:

Pi Standard deviations score

PTH:

Parathyroid hormone

TAL:

Thick ascending limb

TP/GFR:

Threshold for tubular phosphate reabsorption, corrected for GFR

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Acknowledgments

We thank Pearl Lilos, Biostatistician, for her help in the statistical assessment of the data.

Author information

Authors and Affiliations

  1. Department of Pediatrics B, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, Petach Tikva, 49202, Israel

    Daniel Landau

  2. Ben Gurion University of the Negev, Beer Sheva, Israel

    Daniel Landau, Evgenia Gurevich & Hannah Shalev

  3. Division of Pediatrics, Soroka University Medical Center, Beer Sheva, Israel

    Evgenia Gurevich & Hannah Shalev

  4. Department of Pediatrics, Kaplan Medical Center, Rehovot, Israel

    Levana Sinai-Treiman

Authors
  1. Daniel Landau
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  2. Evgenia Gurevich
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  3. Levana Sinai-Treiman
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  4. Hannah Shalev
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Corresponding author

Correspondence to Daniel Landau.

Ethics declarations

The investigative protocol was approved by the local Committee for Human Experimentation. This was a retrospective analysis of all children and young adults and as such separate informed consent was not required.

Conflict of interest

The authors declare no conflicts of interest.

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Landau, D., Gurevich, E., Sinai-Treiman, L. et al. Accentuated hyperparathyroidism in type II Bartter syndrome. Pediatr Nephrol 31, 1085–1090 (2016). https://doi.org/10.1007/s00467-016-3337-1

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  • DOI: https://doi.org/10.1007/s00467-016-3337-1

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