PMID- 34768698
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20211118
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 10
IP  - 21
DP  - 2021 Nov 5
TI  - Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A 
      Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype?
LID - 10.3390/jcm10215179 [doi]
LID - 5179
AB  - 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(2+)) 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 (p < 0.0001 in both cases). 
      Fasting FeCa(2+) is also surprisingly low in pHPT SCD patients, and thus 
      inadequate to be considered hypercalcemia, recalling the FHH phenotype. FeCa(2+) 
      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(2+) 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.
FAU - Khan, Edmat Akhtar
AU  - Khan EA
AD  - Service de Nephrologie, Universite de Lorraine, CHRU-Nancy, 54500 
      Vandoeuvre-les-Nancy, France.
FAU - Cheddani, Lynda
AU  - Cheddani L
AD  - Unite HTA, Prevention et Therapeutique Cardiovasculaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Hotel Dieu, 75004 Paris, France.
AD  - Centre de Diagnostic et de Therapeutique, Hotel-Dieu, Universite de Paris, 75006 
      Paris, France.
FAU - Saint-Jacques, Camille
AU  - Saint-Jacques C
AD  - Service des Explorations Fonctionnelles Multidisciplinaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
AD  - Unite Mixte de Recherche (UMR) S 1155, Institut National de la Sante et de la 
      Recherche Medicale, Sorbonne Universite, Hopital Tenon, 75020 Paris, France.
FAU - Vargas-Poussou, Rosa
AU  - Vargas-Poussou R
AUID- ORCID: 0000-0002-4169-0680
AD  - Centre d'Investigation Clinique, Centre de Reference des Maladies Renales 
      Hereditaires de l'Enfant et de l'Adulte, Assistance Publique-Hopitaux de Paris, 
      Hopital Europeen Georges Pompidou, 75015 Paris, France.
FAU - Frochot, Vincent
AU  - Frochot V
AD  - Service des Explorations Fonctionnelles Multidisciplinaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
AD  - Unite Mixte de Recherche (UMR) S 1155, Institut National de la Sante et de la 
      Recherche Medicale, Sorbonne Universite, Hopital Tenon, 75020 Paris, France.
FAU - Chieze, Remi
AU  - Chieze R
AD  - Service des Explorations Fonctionnelles Multidisciplinaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
AD  - Unite Mixte de Recherche (UMR) S 1155, Institut National de la Sante et de la 
      Recherche Medicale, Sorbonne Universite, Hopital Tenon, 75020 Paris, France.
FAU - Letavernier, Emmanuel
AU  - Letavernier E
AD  - Service des Explorations Fonctionnelles Multidisciplinaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
AD  - Unite Mixte de Recherche (UMR) S 1155, Institut National de la Sante et de la 
      Recherche Medicale, Sorbonne Universite, Hopital Tenon, 75020 Paris, France.
FAU - Avellino, Virginie
AU  - Avellino V
AD  - Service de Medecine Interne, Centre de Reference de la Drepanocytose, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
FAU - Lionnet, Francois
AU  - Lionnet F
AD  - Service de Medecine Interne, Centre de Reference de la Drepanocytose, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
FAU - Haymann, Jean-Philippe
AU  - Haymann JP
AUID- ORCID: 0000-0002-2756-2287
AD  - Service des Explorations Fonctionnelles Multidisciplinaires, Assistance 
      Publique-Hopitaux de Paris, Hopital Tenon, 75020 Paris, France.
AD  - Unite Mixte de Recherche (UMR) S 1155, Institut National de la Sante et de la 
      Recherche Medicale, Sorbonne Universite, Hopital Tenon, 75020 Paris, France.
LA  - eng
PT  - Journal Article
DEP - 20211105
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC8584729
OTO - NOTNLM
OT  - FeCa2+
OT  - familial hypocalciuric hypercalcemia
OT  - hemolysis
OT  - primary hyperparathyroidism
OT  - sickle cell disease
OT  - urinary calcium
COIS- The authors declare no conflict of interest.
EDAT- 2021/11/14 06:00
MHDA- 2021/11/14 06:01
PMCR- 2021/11/05
CRDT- 2021/11/13 01:02
PHST- 2021/08/22 00:00 [received]
PHST- 2021/09/21 00:00 [revised]
PHST- 2021/11/03 00:00 [accepted]
PHST- 2021/11/13 01:02 [entrez]
PHST- 2021/11/14 06:00 [pubmed]
PHST- 2021/11/14 06:01 [medline]
PHST- 2021/11/05 00:00 [pmc-release]
AID - jcm10215179 [pii]
AID - jcm-10-05179 [pii]
AID - 10.3390/jcm10215179 [doi]
PST - epublish
SO  - J Clin Med. 2021 Nov 5;10(21):5179. doi: 10.3390/jcm10215179.