Changing biochemical presentation of primary hyperparathyroidism

Almquist, Martin; Bergenfelz, Anders; Mårtensson, Hans; Thier, Mark; Nordenström, Erik

doi:10.1007/s00423-010-0675-5

Cited by 33 publications

(28 citation statements)

References 14 publications

(23 reference statements)

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“…In recent large studies investigating the changing biochemistry of PHPT in adults, the serum calcium, serum ionized calcium, and serum PTH concentrations at presentation for surgery were 2.8 mmol/L (normal range 2.1–2.6), 50 1.45 nmol/L (normal range 1.15–1.2), and 103 pg/mL (normal range 15–65), respectively. 51 For comparison, in the studies reviewed here, the average serum calcium, serum ionized calcium, and serum PTH concentrations at presentation in children and adolescents were 3.27 mmol/L ( n = 226, normal range 2.1–2.6), 1.56 nmol/L ( n = 59, normal range 1.15–1.2), and 200 pg/mL ( n = 156, normal range 10–70), respectively. The increased magnitude of deviation from the normal range observed in childhood and adolescent PHPT biochemical measurements is consistent with the idea that PHPT is more severe in children and adolescents than in adults.…”

Section: Ascertainment and Diagnosismentioning

confidence: 99%

Primary hyperparathyroidism in children and adolescents

Roizen

Levine

2012

Journal of the Chinese Medical Association

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Primary hyperparathyroidism (PHPT) is a common endocrine disorder in adults in whom the typical presentation is incidentally discovered as asymptomatic hypercalcemia. PHPT is much less common in children and adolescents, but has greater morbidity in this age group, as most young patients with PHPT will have symptomatic hypercalcemia or complications such as kidney stones, abdominal pain, and skeletal fragility. An important feature of PHPT in younger patients is the relatively high prevalence of germline inactivating mutations of the CASR gene, which encodes the calcium-sensing receptor. Biallelic CASR mutations cause neonatal severe hyperparathyroidism, a life-threatening condition that presents within days of life with marked hypercalcemia, respiratory distress, failure to thrive, and skeletal demineralization. By contrast, more common heterozygous CASR mutations are generally associated with a benign variant of PHPT termed familial hypocalciuric hypercalcemia. Appropriate management of PHPT in children and adolescents requires distinction between familial hypocalciuric hypercalcemia, which generally requires no specific treatment, and other forms of PHPT that are best treated by parathyroidectomy.

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Section: Ascertainment and Diagnosismentioning

confidence: 99%

Primary hyperparathyroidism in children and adolescents

Roizen

Levine

2012

Journal of the Chinese Medical Association

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“…Prospective ascertainment of demographic, clinical, biochemical, and histological data in a significant PHPT dataset are required to identify potential contributing factors. However, the paucity and limited scope of previous studies, which have focused primarily on surgical outcomes, has limited the ability to characterize the population of patients with more severe PHPT [12, 13]. Given this knowledge deficit, our goals were (1) to evaluate relevant clinical data in a large population of unselected patients with established PHPT in order to better understand the phenotypic variability observed in this disease; and (2) to define parathyroid neoplasia cellular behavior functionally in a novel ex vivo platform, as a potential predictor of preoperative disease severity in patients referred for parathyroidectomy.…”

Section: 1 Introductionmentioning

confidence: 99%

Impaired calcium sensing distinguishes primary hyperparathyroidism (PHPT) patients with low bone mineral density

et al. 2017

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Context A subset of PHPT patients exhibit a more severe disease phenotype characterized by bone loss, fractures, recurrent nephrolithiasis, and other dysfunctions, but the underlying reasons for this disparity in clinical presentation remain unknown. Objective We sought to identify new mechanistic indices that could inform more personalized management of PHPT. Design Pre-, peri-, and postoperative data and demographic, clinical, and pathological information from patients undergoing parathyroidectomy for PHPT were collected. Univariate and partial Spearman correlation was used to estimate the association of parathyroid tumor calcium sensing capacity with select variables. Patients or Other Participants An unselected series of 237 patients aged >18 years and undergoing parathyroidectomy for PHPT were enrolled. Main outcome measures Calcium sensing capacity, expressed as the concentration required for half-maximal biochemical response (EC50), was evaluated in parathyroid tumors from an unselected series of 74 patients and assessed for association with clinical parameters. The hypothesis was that greater disease severity would be associated with attenuated calcium sensitivity and biochemically autonomous parathyroid tumor behavior. Results Parathyroid tumors segregated into two distinct groups of calcium responsiveness (EC50 <3.0 and ≥3.0 mM). The low EC50 group (n=27) demonstrated a mean calcium EC50 value of 2.49 mM [95% confidence interval (CI): 2.43–2.54 mM], consistent with reference normal activity. In contrast, the high EC50 group (n=47) displayed attenuated calcium sensitivity with a mean EC50 value of 3.48 mM [95% CI: 3.41–3.55 mM]. Retrospective analysis of the clinical registry data suggested that high calcium EC50 patients presented with a more significant preoperative bone mineral density (BMD) deficit with a T-score of −2.7, (95% CI: −3.4 to −1.9) versus 0.9, (95% CI: −2.1 to −0.4) in low EC50 patients (p < 0.001). After adjusting for gender, age, BMI, 25 OH vitamin D level and preoperative iPTH, lowest T-score and calcium EC50 were inversely correlated, with a partial Spearman correlation coefficient of −0.35 (p=0.02). Conclusions Impaired calcium sensing in parathyroid tumors is selectively observed in a subset of patients with more severe bone mineral density deficit. Assessment of parathyroid tumor biochemical behavior may be a useful predictor of disease severity as measured by bone mineral density in patients with PHPT.

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“…El peso promedio de los adenomas paratiroideos operados se ha estimado en 520 mg en la última década 7 . Un estudio analizó las características de 26 casos con adenomas paratiroideos determinó un peso de > 3,5 g (promedio de 9,8 g y el mayor de 40 g), 50% de los cuales fueron ectópicos y con un promedio de PTH de 451 pg/ ml; un tercio de los casos fueron descubiertos incidentalmente por hipercalcemia, sin reportarse lesiones malignas 8 .…”

Section: Discussionunclassified