Severe Primary Hyperparathyroidism in a Neonate With Two Hypercalcemic Parents: Management With Parathyroidectomy and Heterotopic Autotransplantation

PEDIATRICS ◽  
1986 ◽  
Vol 78 (2) ◽  
pp. 263-268
Author(s):  
Linda Cooper ◽  
Joseph Wertheimer ◽  
Raphael Levey ◽  
Edward Brown ◽  
Meryl Leboff ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Confounding Factor ◽  
Parathyroid Tissue ◽  
Normal Serum ◽  
Familial Hypocalciuric Hypercalcemia ◽  
Normal Range ◽  
Hormone Levels ◽  
Immunoreactive Parathyroid Hormone

A neonate with severe primary hyperparthyroidism was successfully managed by parathyroidectomy and heterotopic autotransplantation (one third of one gland of the infant was implanted in the forearm). In vitro studies of parathyroid tissue from the infant revealed a severe defect in parathyroid suppressibility. Postoperatively, the infant had modest hypercalcemia, normal serum immunoreactive parathyroid hormone levels, hypermagnesemia, and relative hypocalciuria. The parents were related and both had asymptomatic hypercalcemia with mean serum immunoreactive parathyroid hormone levels that were within the normal range. Similar to the findings in the infant postoperatively, relative hypocalciuria in the presence of hypercalcemia was found in the mother; in contrast, the father had hypercalciuria. The presumed dominantly transmitted hypercalcemia in this kindred is consistent with familial hypocalciuric hypercalcemia with a confounding factor of ethanol possibly accounting for the hypercalciuria in the father.

PARATHYROID HORMONE SECRETION IN DISORDERS OF CALCIUM METABOLISM STUDIED BY MEANS OF EDTA

1974 ◽  
Vol 75 (2) ◽  
pp. 286-296 ◽  
Author(s):  
J. H. Lockefeer ◽  
W. H. L. Hackeng ◽  
J. C. Birkenhäger
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Calcium Metabolism ◽  
Hormone Secretion ◽  
Parathyroid Tissue ◽  
Small Mass ◽  
Idiopathic Hypercalciuria ◽  
List Type ◽  
Immunoreactive Parathyroid Hormone ◽  
Pth Level

ABSTRACT In 22 of 28 cases of primary hyperparathyroidism (PHP) the rise in the serum immunoreactive parathyroid hormone (IRPTH or PTH) level observed in response to lowering of the serum calcium by EDTA, exceeded that obtained in 8 control subjects. In 5 of these 22 patients who were studied again after parathyroidectomy the supranormal response was abolished. Fifteen of these 22 hyper-responsive PHP patients had basal IRPTH levels not exceeding the highest level in the controls and that of other groups of patients investigated (idiopathic hypercalciuria, non-parathyroid hypercalcaemia, operated PHP). Fourteen of the 22 hyper-reactive patients with PHP did not show hypocalcaemia during the infusion of EDTA. The extent of the release of PTH elicited by EDTA in cases of PHP does not as yet allow a prediction of the amount of pathological parathyroid tissue present, although all the PHP patients showing a normal release of PTH had a relatively small mass of parathyroid tissue (up to about 1 g) subsequently removed. In 9 cases of nephrolithiasis (8 of whom had idiopathic hypercalciuria) and in 7 cases of non-parathyroid hypercalcaemia, a normal PTH release was found.

Parathyroid Hormone Assay in Primary Hyperparathyroidism: Experiences with a Radioimmunoassay Based on Commercially Available Reagents

1974 ◽  
Vol 20 (3) ◽  
pp. 369-375 ◽  
Author(s):  
M Kleerekoper ◽  
J P Ingham ◽  
S W McCarthy ◽  
S Posen
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Human Serum ◽  
Serum Calcium ◽  
Parathyroid Tissue ◽  
Normal Range ◽  
Serum Parathyroid Hormone ◽  
Skeletal Changes ◽  
Parathyroid Hormone Assay ◽  
Positive Correlations

Abstract A radioimmunoassay is described for parathyroid hormone in human serum, in which commercially available reagents are used almost exclusively. This assay can be done by any laboratory with experience in radloimmunoassay. Thirty-two of thirty-three patients with surgically proven primary hyperparathyroidism had detectable concentrations of parathyroid hormone in their serum, and concentrations of the hormone exceeded the normal range in 24 of them. Significant positive correlations were found between pre-operative serum calcium, pre-operative serum parathyroid hormone, and the weight of parathyroid tissue removed at operation. These three parameters were also significantly correlated with severity of the skeletal changes as assessed by semiquantitative histological methods.

scholarly journals A quick intraoperative parathyroid hormone assay in the surgical management of patients with primary hyperparathyroidism: a study of 206 consecutive cases

2002 ◽  
pp. 783-788 ◽  
Author(s):  
E Vignali ◽  
A Picone ◽  
G Materazzi ◽  
S Steffe ◽  
P Berti ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Serum Calcium ◽  
False Negative ◽  
Parathyroid Tissue ◽  
Normal Serum ◽  
Neck Exploration ◽  
True Negative ◽  
Normal Serum Calcium ◽  
Intraoperative Parathyroid Hormone

OBJECTIVE: The traditional surgical approach for patients with primary hyperparathyroidism (PHPT) consists of the identification of at least four glands and in the removal of all hyperfunctioning parathyroid tissue. DESIGN: To evaluate whether intraoperative parathyroid hormone (PTH) monitoring will allow a more limited surgical procedure by confirming complete removal of all hyperfunctioning tissue. METHODS: Plasma samples were obtained from 206 consecutive patients with sporadic PHPT before skin incision, during manipulation of a suspected adenoma, and 5 min (T-5) and 10 min after removal of abnormal parathyroid tissue. PTH was measured by a quick immunochemiluminescent assay (QPTH). The operative success was defined by a decrease of PTH greater than 50% of the highest pre-excision value. RESULTS: A >50% decrease of PTH occurred in 203 patients and was evident at T-5 in the majority of cases. All but three had normal serum calcium the day after surgery and afterwards. PTH concentration did not show a >50% decrease in the remaining three cases after completion of surgery. One patients had negative neck exploration and remained hypercalcemic; the other two had normal serum calcium at follow-up. Thus, the intraoperative QPTH correctly predicted the outcome of surgery in 201 patients (97.5%) (200 true positive and 1 true negative), and provided three false positive and two false negative results. CONCLUSIONS: The intraoperative QPTH measurement represents a useful tool to assist the surgeon during parathyroidectomy. It indicates whether all hyperfunctioning parathyroid tissue has been removed, limiting the procedure to a unilateral neck exploration in most cases.

Calcium-activated proteases in the bovine parathyroid gland: potential role in degradation of parathyroid hormone to peptide fragments

1995 ◽  
Vol 15 (1) ◽  
pp. 61-71 ◽  
Author(s):  
P H Watson ◽  
S T Mortimer ◽  
K K W Wang ◽  
D E Croall ◽  
D A Hanley
Keyword(s):  
Parathyroid Hormone ◽  
Parathyroid Tissue ◽  
Area Under The Curve ◽  
Molar Ratio ◽  
Cell Protein ◽  
Parathyroid Cell ◽  
Peptide Fragments ◽  
Intracellular Degradation ◽  
Peak Areas

ABSTRACT Our studies suggest that protein kinase C is involved in low calcium (Ca2+)-stimulated secretion of parathyroid hormone (PTH) but not directly in high Ca2+-stimulated intracellular degradation of PTH to secreted carboxyl-terminal fragments (C-PTH), an important component of Ca2+-regulated PTH secretion. The present study was undertaken to determine the presence of calciumactivated proteases, 84 kDa (micro)-calpain and 80 kDa (milli)-calpain, in the bovine parathyroid, and whether they could degrade PTH to C-terminal fragments. Immunocytochemistry of bovine parathyroid tissue using antibodies raised against bovine heart micro- and milli-calpain detected both isoforms of calpain. Western blotting of total bovine parathyroid cell protein prepared from primary cell cultures confirmed the presence of both isoforms of calpain, demonstrated by specific milli- and micro-calpain bands. Purified bovine PTH (bPTH) was incubated in vitro with human erythrocyte micro-calpain and the cleavage products were separated by reverse-phase HPLC. Eluant fractions were assayed with an RIA with equimolar sensitivity to C-PTH and bPTH, and peak areas integrated. Micro-calpain produced a C-PTH peak from bPTH which co-eluted with the major C-PTH secreted by parathyroid cells in culture. C-PTH production by micro-calpain, expressed as per cent area under the curve, increased from 0% in the absence of either micro-calpain or Ca2+, to 71·5% when a 5:1 molar ratio of bPTH to calpain was used. Amino acid sequencing and analysis of the immunoreactive PTH cleavage products indicated the presence of two fragments of bPTH in the C-PTH peak, bPTH47–84 and bPTH69–84. In summary, both isoforms of calpain are present in the bovine parathyroid and calpains may play a role in the Ca2+-dependent degradation of PTH to secreted C-terminal fragments.

ENDOCRINE EFFECTS OF LITHIUM:

1978 ◽  
Vol 88 (3) ◽  
pp. 528-534 ◽  
Author(s):  
C. Christiansen ◽  
P. C. Baastrup ◽  
P. Lindgreen ◽  
I. Transbøl
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Serum Calcium ◽  
Lithium Treatment ◽  
Serum Magnesium ◽  
Endocrine Effects ◽  
Manic Depressive ◽  
Depressive Patients ◽  
Immunoreactive Parathyroid Hormone

ABSTRACT Ninety-six manic-depressive patients were studied during long-term lithium treatment. Highly significant elevations were observed respecting the levels of serum immunoreactive parathyroid hormone (P < 0.001) as well as the protein-corrected levels of serum calcium (P < 0.001) and serum magnesium (P <0.001), thus indicating a state of 'primary' hyperparathyroidism. The patients as a group had normophosphataemia and normophosphatasia supporting the impression of a rather mild state of biochemical hyperparathyroidism.

Intact parathyroid hormone levels and primary hyperparathyroidism

2017 ◽  
pp. 1-5 ◽  
Author(s):  
Haidar Al-Hraishawi ◽  
Peter J. Dellatore ◽  
Xinjiang Cai ◽  
Xiangbing Wang
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Intact Parathyroid Hormone ◽  
Hormone Levels

scholarly journals A novel CASR variant in a family with familial hypocalciuric hypercalcaemia and primary hyperparathyroidism

2020 ◽  
Vol 2020 ◽  
Author(s):  
Satyanarayana V Sagi ◽  
Hareesh Joshi ◽  
Jamie Trotman ◽  
Terence Elsey ◽  
Ashwini Swamy ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Primary Hyperparathyroidism ◽  
Organ Damage ◽  
Urinary Calcium ◽  
Interesting Case ◽  
List Type ◽  
Loss Of Function ◽  
Clearance Ratio ◽  
Hormone Levels ◽  
Severe Hypercalcaemia

Summary Familial hypocalciuric hypercalcaemia (FHH) is a dominantly inherited, lifelong benign disorder characterised by asymptomatic hypercalcaemia, relative hypocalciuria and variable parathyroid hormone levels. It is caused by loss-of-function pathogenic variants in the calcium-sensing receptor (CASR) gene. Primary hyperparathyroidism (PHPT) is characterised by variable hypercalcaemia in the context of non-suppressed parathyroid hormone levels. Unlike patients with FHH, patients with severe hypercalcaemia due to PHPT are usually symptomatic and are at risk of end-organ damage affecting the kidneys, bone, heart, gastrointestinal system and CNS. Surgical resection of the offending parathyroid gland(s) is the treatment of choice for PHPT, while dietary adjustment and reassurance is the mainstay of management for patients with FHH. The occurrence of both FHH and primary hyperparathyroidism (PHPT) in the same patient has been described. We report an interesting case of FHH due to a novel CASR variant confirmed in a mother and her two daughters and the possible coexistence of FHH and PHPT in the mother, highlighting the challenges involved in diagnosis and management. Learning points: Familial hypocalciuric hypercalcaemia (FHH) and primary hyperparathyroidism (PHPT) can coexist in the same patient. Urinary calcium creatinine clearance ratio can play a role in distinguishing between PHPT and FHH. Genetic testing should be considered in managing patients with PHPT and FHH where the benefit may extend to the wider family. Family segregation studies can play an important role in the reclassification of variants of uncertain significance. Parathyroidectomy has no benefit in patients with FHH and therefore, it is important to exclude FHH prior to considering surgery. For patients with coexisting FHH and PHPT, parathyroidectomy will reduce the risk of complications from the severe hypercalcaemia associated with PHPT.

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