Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype?
Primary hyperparathyroidism (pHPT) has been reported to have a higher prevalence in sickle cell disease (SCD) patients, including a high rate of recurrence following surgery. However, most patients are asymptomatic at the time of diagnosis, with surprisingly infrequent hypercalciuria, raising the is...
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2021
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oai:doaj.org-article:0f788fe4cd18494499b3ee8edc2a11e42021-11-11T17:47:20ZPrimary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype?10.3390/jcm102151792077-0383https://doaj.org/article/0f788fe4cd18494499b3ee8edc2a11e42021-11-01T00:00:00Zhttps://www.mdpi.com/2077-0383/10/21/5179https://doaj.org/toc/2077-0383Primary hyperparathyroidism (pHPT) has been reported to have a higher prevalence in sickle cell disease (SCD) patients, including a high rate of recurrence following surgery. However, most patients are asymptomatic at the time of diagnosis, with surprisingly infrequent hypercalciuria, raising the issue of renal calcium handling in SCD patients. We conducted a retrospective study including (1) 64 hypercalcemic pHPT non-SCD patients; (2) 177 SCD patients, divided into two groups of 12 hypercalcemic pHPT and 165 non-pHPT; (3) eight patients with a diagnosis of familial hypocalciuric hypercalcemia (FHH). Demographic and biological parameters at the time of diagnosis were collected and compared between the different groups. Determinants of fasting fractional excretion of calcium (FeCa<sup>2+</sup>) were also analyzed in non-pHPT SCD patients. Compared to non-SCD pHPT patients, our data show a similar ionized calcium and PTH concentration, with a lower plasmatic calcitriol concentration and a lower daily urinary calcium excretion in pHPT SCD patients (<i>p</i> < 0.0001 in both cases). Fasting FeCa<sup>2+</sup> is also surprisingly low in pHPT SCD patients, and thus inadequate to be considered hypercalcemia, recalling the FHH phenotype. FeCa<sup>2+</sup> is also low in the non-pHPT SCD control group, and negatively associated with PTH and hemolytic biomarkers such as LDH and low hemoglobin. Our data suggest that the pHPT biochemical phenotype in SCD patients resembles the FHH phenotype, and the fasting FeCa<sup>2+</sup> association with chronic hemolysis biomarkers strengthens the view of a potential pharmacological link between hemolytic by-products and calcium reabsorption, potentially through a decreased calcium-sensing receptor (CaSR) activity.Edmat Akhtar KhanLynda CheddaniCamille Saint-JacquesRosa Vargas-PoussouVincent FrochotRemi ChiezeEmmanuel LetavernierVirginie AvellinoFrancois LionnetJean-Philippe HaymannMDPI AGarticleprimary hyperparathyroidismsickle cell diseaseurinary calciumfamilial hypocalciuric hypercalcemiaFeCa<sup>2+</sup>hemolysisMedicineRENJournal of Clinical Medicine, Vol 10, Iss 5179, p 5179 (2021) |
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primary hyperparathyroidism sickle cell disease urinary calcium familial hypocalciuric hypercalcemia FeCa<sup>2+</sup> hemolysis Medicine R |
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primary hyperparathyroidism sickle cell disease urinary calcium familial hypocalciuric hypercalcemia FeCa<sup>2+</sup> hemolysis Medicine R Edmat Akhtar Khan Lynda Cheddani Camille Saint-Jacques Rosa Vargas-Poussou Vincent Frochot Remi Chieze Emmanuel Letavernier Virginie Avellino Francois Lionnet Jean-Philippe Haymann Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
description |
Primary hyperparathyroidism (pHPT) has been reported to have a higher prevalence in sickle cell disease (SCD) patients, including a high rate of recurrence following surgery. However, most patients are asymptomatic at the time of diagnosis, with surprisingly infrequent hypercalciuria, raising the issue of renal calcium handling in SCD patients. We conducted a retrospective study including (1) 64 hypercalcemic pHPT non-SCD patients; (2) 177 SCD patients, divided into two groups of 12 hypercalcemic pHPT and 165 non-pHPT; (3) eight patients with a diagnosis of familial hypocalciuric hypercalcemia (FHH). Demographic and biological parameters at the time of diagnosis were collected and compared between the different groups. Determinants of fasting fractional excretion of calcium (FeCa<sup>2+</sup>) were also analyzed in non-pHPT SCD patients. Compared to non-SCD pHPT patients, our data show a similar ionized calcium and PTH concentration, with a lower plasmatic calcitriol concentration and a lower daily urinary calcium excretion in pHPT SCD patients (<i>p</i> < 0.0001 in both cases). Fasting FeCa<sup>2+</sup> is also surprisingly low in pHPT SCD patients, and thus inadequate to be considered hypercalcemia, recalling the FHH phenotype. FeCa<sup>2+</sup> is also low in the non-pHPT SCD control group, and negatively associated with PTH and hemolytic biomarkers such as LDH and low hemoglobin. Our data suggest that the pHPT biochemical phenotype in SCD patients resembles the FHH phenotype, and the fasting FeCa<sup>2+</sup> association with chronic hemolysis biomarkers strengthens the view of a potential pharmacological link between hemolytic by-products and calcium reabsorption, potentially through a decreased calcium-sensing receptor (CaSR) activity. |
format |
article |
author |
Edmat Akhtar Khan Lynda Cheddani Camille Saint-Jacques Rosa Vargas-Poussou Vincent Frochot Remi Chieze Emmanuel Letavernier Virginie Avellino Francois Lionnet Jean-Philippe Haymann |
author_facet |
Edmat Akhtar Khan Lynda Cheddani Camille Saint-Jacques Rosa Vargas-Poussou Vincent Frochot Remi Chieze Emmanuel Letavernier Virginie Avellino Francois Lionnet Jean-Philippe Haymann |
author_sort |
Edmat Akhtar Khan |
title |
Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
title_short |
Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
title_full |
Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
title_fullStr |
Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
title_full_unstemmed |
Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype? |
title_sort |
primary hyperparathyroidism in homozygous sickle cell patients: a hemolysis-mediated hypocalciuric hypercalcemia phenotype? |
publisher |
MDPI AG |
publishDate |
2021 |
url |
https://doaj.org/article/0f788fe4cd18494499b3ee8edc2a11e4 |
work_keys_str_mv |
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