Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood
Abstract
1. Introduction
2. Methods
Statistical Analysis
3. Results
3.1. Clinical Characteristics
3.2. Biological Characteristics
3.3. Bone Mineral Density
3.4. Parathyroid Imaging
3.5. Genetic Abnormalities
3.6. pHPT Treatment
3.7. Follow-Up
4. Discussion
4.1. General Considerations
4.2. Bone Involvement
4.3. Treatment
4.4. Pathophysiology
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Demographic parameters Age at diagnosis (years) | 41 (31.5–49.2) |
Female | 18 (64%) |
Body mass index (kg/m2) | 21.8 (19.4–25.1) |
Homozygous (SS) sickle cell disease | 22 (79%) |
Sub-Saharan African origin | 23 (82%) |
SCD treatment | |
Hydroxyurea * | 15/27 (56%) |
Median dosage (mg/day) | 1000 (800–1500) |
Exchange transfusion * | 6/27 (22%) |
Folic acid | 27/27 (100%) |
Vitamin D intake | 22/27 (81%) |
pHPT clinical complication | |
Asymptomatic | 16 (57%) |
Kidney stone | 3 (11%) |
Gastric ulcer | 2 (7%) |
Bone mineral density (n = 16) | |
Low BMD (< −2.5 < T-score < −1 SD) | 2 (12.5%) |
Very low BMD (≤−2.5 SD) | 7 (44%) |
Lumbar spine T-score | −1.1 (−3.05; +0.45) |
Femoral neck T-score | −0.8 (−1.2; +2) |
Distal radius T-score (n = 5) | −2.6 (−5.1; −1) |
n | ||
---|---|---|
Serum total calcium level (mmol/L) | 2.62 (2.60–2.78) | 28 |
Serum ionized calcium (mmol/L) | 1.38 (1.35–1.42) | 18 |
Serum phosphate level (mmol/L) | 0.90 (0.81–0.99) | 28 |
Hypophosphatemia * | 8 (29%) | 28 |
PTH (pg/mL) | 105 (69–137) | 27 ** |
Increased PTH level * | 21 (78%) | 27 |
Serum creatinine level (µmol/L) | 58.5 (50.5–70.2) | 28 |
eGFR (mL/min/1.73 m2) | 110.5 (93.7–129.2) | 28 |
25(OH)D (ng/mL) | 25.9 (12.7–48.2) | 24 |
1,25(OH)2D (pg/mL) | 85 (53–121) | 15 |
Hemoglobin (g/dL) | 8.45 (7.7–10) | 28 |
HbF (%) | 8 (4–15) | 24 |
MCV (fL) | 91.5 (79.7–107.5) | 28 |
Reticulocyte count (G/L) | 200 (115–252) | 27 |
Total bilirubin (µmol/L) | 30.5 (19–43) | 24 |
LDH (UI/L) | 444 (305–716) | 26 |
Neutrophil count (G/L) | 3.8 (2.2–4.3) | 28 |
CTX (nmol/L) (normal < 1.8) | 4.3 (2.6–7.1) | 12 |
Osteocalcin (ng/mL) (normal range 14–32) | 35 (23–38) | 13 |
Patients | Controls | p | |
---|---|---|---|
n = 16 | n = 32 | ||
Age | 40 (34–49) | 35 (31–43) | 0.31 |
Female * | 11 (69) | 21 (66) | |
Homozygous SCD | 12 (75) | 21 (66) | |
Body mass index (kg/m2) | 21.9 (19.6–25.8) | 22.1 (20.3–25.2) | 0.64 |
Hemoglobin (g/dl) | 9 (7.7–10.7) | 8.8 (7.5–10.5) | 0.9 |
HbF (%) | 7.8 (5–15) | 8 (5.5–16) | 0.8 |
Reticulocytes (G/L) | 183 (88–240) | 190 (100–250) | 0.8 |
Neutrophil count (G/L) | 3.6 (2.2–4.4) | 4 (2.4–4.5) | 0.7 |
Lumbar spine T-score | −1.1 (−3.05; +0.45) | −0.1 (−2.1; +0.72) | 0.38 |
Femoral neck T-score | −0.8 (−1.2; +2) | 0.4 (−0.65; +1.25) | 0.68 |
Low BMD (<−2.5 < T-score < −1 SD) | 2 (12.5) | 10 (31) | 0.073 |
Very low BMD (≤−2.5 SD) | 7 (44) | 4 (12.5) | 0.027 |
25(OH)D (ng/mL) | 26 (14–68) | 15 (10–25) | 0.05 |
Before Surgery | After Surgery | p | |
---|---|---|---|
Calcemia (mmol/L) | 2.65 (2.54–2.86) | 2.23 (2.13–2.3) | 0.0025 |
Phosphatemia (mmol/L) | 0.89 (0.82–0.96) | 1.2 (0.96–1.28) | 0.056 |
PTH (pg/mL) | 106 (93–145) | 34 (31–50) | 0.004 |
eGFR (ml/min/1.73 m2) | 103 (91–131) | 104 (85–124) | 0.26 |
Hemoglobin (g/dL) | 7.9 (6.75–9.7) | 8.7 (7.8–9.6) | 0.38 |
Reticulocyte count (G/L) | 208 (135–285) | 154 (122–237) | 0.50 |
Surgery (n = 14) | No Surgery (n = 14) | p | |
---|---|---|---|
Age at diagnosis (years) | 46 (30.5–51.5) | 39 (34.2–44.7) | 0.59 |
Female (%) | 11 (79) | 7 (50) | 0.24 |
SCD genotype SS | 12 (86) | 10 (71) | 0.65 |
Body mass index (kg/m2) | 21.6 (19.5–23.7) | 22.3 (19.5–26) | 0.68 |
Low BMD (T-score < −1 SD) | 6/9 (67) | 2/7 (29) | 0.66 |
Calcemia (mmol/L) | 2.65 (2.54–2.86) | 2.62 (2.6–2.71) | 0.98 |
PTH (pg/mL) | 106 (93–145) | 88.5 (59.5–129.7) | 0.19 |
Hemoglobin (g/dL) | 7.9 (6.7–9.7) | 9.2 (8.2–10.4) | 0.09 |
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Denoix, E.; Bomahou, C.; Clavier, L.; Ribeil, J.-A.; Lionnet, F.; Bartolucci, P.; Courbebaisse, M.; Pouchot, J.; Arlet, J.-B. Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood. J. Clin. Med. 2020, 9, 308. https://doi.org/10.3390/jcm9020308
Denoix E, Bomahou C, Clavier L, Ribeil J-A, Lionnet F, Bartolucci P, Courbebaisse M, Pouchot J, Arlet J-B. Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood. Journal of Clinical Medicine. 2020; 9(2):308. https://doi.org/10.3390/jcm9020308
Chicago/Turabian StyleDenoix, Elsa, Charlène Bomahou, Lorraine Clavier, Jean-Antoine Ribeil, François Lionnet, Pablo Bartolucci, Marie Courbebaisse, Jacques Pouchot, and Jean-Benoît Arlet. 2020. "Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood" Journal of Clinical Medicine 9, no. 2: 308. https://doi.org/10.3390/jcm9020308
APA StyleDenoix, E., Bomahou, C., Clavier, L., Ribeil, J.-A., Lionnet, F., Bartolucci, P., Courbebaisse, M., Pouchot, J., & Arlet, J.-B. (2020). Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood. Journal of Clinical Medicine, 9(2), 308. https://doi.org/10.3390/jcm9020308