Amelioration of Sickle Cell Pain after Parathyroidectomy in Two Patients with Concurrent Hyperparathyroidism: An Interesting Finding

Muthu, John; Ali, Mir Inzamam

doi:10.1155/2016/3263951

Cited by 4 publications

(6 citation statements)

References 15 publications

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“…We observed a tendency toward a higher hemoglobin level and a lower reticulocyte rate after surgery (which was not statistically significant), which could support this hypothesis. In the two cases of SCD patients with pHPT reported in the literature [9,10], the frequency of painful episodes decreased after parathyroid surgery. Unfortunately, the number of vaso-occlusive crisis events was not properly recorded for our patients.…”

Section: Pathophysiologymentioning

confidence: 94%

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Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood

Denoix

Bomahou

Clavier

et al. 2020

JCM

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Primary hyperparathyroidism (pHPT) is the third most common endocrine disorder and usually affects patients between 60 and 70 years of age. To our knowledge, this condition has never been studied in young patients with sickle cell disease (SCD). Our objective was to describe the clinical and biological characteristics of pHPT in adult patients with SCD and its management. We conducted a retrospective study that included SCD patients who were diagnosed with pHPT in four SCD referral centers. pHPT was defined by the presence of elevated serum calcium levels with inappropriate normal or increased parathyroid hormone (PTH) serum levels or histopathological evidence of parathyroid adenoma or hyperplasia. Patients with severe renal impairment (GFR <30 mL/min) were excluded. Twenty-eight patients (18 women, 64%; 22 homozygous genotype, 79%) were included. The median age at pHPT diagnosis was 41 years (interquartile range –IQR- 31.5–49.5). The median serum calcium and PTH concentration were, respectively, 2.62 mmol/L (IQR 2.60–2.78) and 105 pg/mL (IQR 69–137). Bone mineral density (BMD) revealed very low BMD (≤−2.5 SD) in 44% of patients explored (vs. 12.5% among 32 SCD patients matched for SCD genotype, sex, age, and BMI, p = 0.03). Fourteen patients (50%) received surgical treatment, which was successful in all cases, but four of these patients (29%) presented with pHPT recurrence after a median time of 6.5 years. Three of these patients underwent a second cervical surgery that confirmed the presence of a new parathyroid adenoma. These results suggest that SCD is a condition associated with pHPT in young subjects. SCD patients with pHPT have a high risk of very low BMD. A diagnosis of pHPT should be suspected in the presence of mild hypercalcemia or low BMD in SCD patients.

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Section: Pathophysiologymentioning

confidence: 94%

“…SCD is not considered a condition associated with pHPT, and, to our knowledge, only two case reports involving SCD patients with pHPT have been previously reported: a 17-year-old girl [9] and a 59-year-old woman [10]. They both had a parathyroid adenoma.…”

Section: Introductionmentioning

confidence: 96%

Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood

Denoix

Bomahou

Clavier

et al. 2020

JCM

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“…Approximately 60% of SCD patients also have severe vitamin D deficiency, worsening predisposition to low BMD [ 7 ]. Previous studies have also documented the role of primary and secondary hyperparathyroidism in bone loss in SCD [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, impaired renal function and eventual development of chronic kidney disease, a late finding of SCD, drive inappropriate parathyroid hormone (PTH) secretion. Increased glomerular filtration seen in SCD can increase phosphate absorption and decrease free serum calcium, leading to increased PTH secretion by kidneys to predispose to secondary hyperparathyroidism [ 8 , 9 ]. Current guidelines recommend vitamin D supplementation in all deficient patients [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Low Bone Mass and Recurrent Fractures in Neurofibromatosis With Concomitant Hemoglobin Sickle Cell (SC) Disease

Alkaissi¹,

Ji²,

Saxena³

et al. 2023

Cureus

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Bone disease and bone loss are common features in certain monogenic diseases such as RASopathies, including neurofibromatosis (NF). Similarly, bone complications are frequent in hemoglobinopathies, another group of Mendelian diseases. This paper reports a young patient with both NF and hemoglobin sickle cell (HbSC) diseases who had multiple vertebral fractures with osteopenia. We also discuss the cellular and pathophysiological mechanisms underlying both diseases and the factors responsible for bone pain and low bone mass in NF and hemoglobinopathies such as HbSC. This case emphasizes the importance of careful evaluation and management of osteoporosis in patients with HbSC and NF1, as both are relatively common monogenic diseases in certain communities.

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“…Primary hyperparathyroidism (pHPT) is an endocrine disorder caused by the excessive secretion of parathyroid hormone (PTH) by the parathyroid glands, with the diagnosis generally being established on the association of hypercalcemia, elevated PTH levels and high urinary calcium excretion [ 18 ]. This disease was not a focus in SCD patients, despite several case reports [ 19 , 20 ], until a recent paper by Denoix et al reported a 5% prevalence of pHPT [ 21 , 22 ]. SCD patients with pHPT were further analyzed and reported to mainly experience adenoma (90% of patients who underwent surgical treatment) and to be mostly clinically asymptomatic with mild hypercalcemia.…”

Section: Introductionmentioning

confidence: 99%

Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype?

Khan

Cheddani

Saint-Jacques

et al. 2021

JCM

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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 (FeCa2+) 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 FeCa2+ is also surprisingly low in pHPT SCD patients, and thus inadequate to be considered hypercalcemia, recalling the FHH phenotype. FeCa2+ 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 FeCa2+ 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.

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Amelioration of Sickle Cell Pain after Parathyroidectomy in Two Patients with Concurrent Hyperparathyroidism: An Interesting Finding

Cited by 4 publications

References 15 publications

Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood

Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood

Low Bone Mass and Recurrent Fractures in Neurofibromatosis With Concomitant Hemoglobin Sickle Cell (SC) Disease

Primary Hyperparathyroidism in Homozygous Sickle Cell Patients: A Hemolysis-Mediated Hypocalciuric Hypercalcemia Phenotype?

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