Post-parathyroidectomy restoration of normal calcium homeostasis in neonatal primary hyperparathyroidism

1984 ◽  
Vol 105 (3) ◽  
pp. 350-353 ◽  
Author(s):  
J. L. C. Ch'ng ◽  
A. Kaiser ◽  
J. Lynn ◽  
G. F. Joplin
Keyword(s):  
Primary Hyperparathyroidism ◽  
Calcium Homeostasis ◽  
Feedback Inhibition ◽  
Hormone Secretion ◽  
Parathyroid Tissue ◽  
Total Parathyroidectomy ◽  
Total Removal ◽  
Ionic Calcium ◽  
Ectopic Parathyroid ◽  
Normal Calcium

Abstract. Total parathyroidectomy is required to cure neonatal primary hyperparathyroidism (NPH) as any parathyroid remnant quickly becomes hyperplastic, causing recurrent hypercalcaemia. We present a patient with NPH who had total removal of his eutopic parathyroid glands but continued to have parathyroid hormone secretion from presumed ectopic parathyroid tissue. Hypercalcaemia initially recurred but normal calcium homeostasis was established as the child grew older. We postulate that the underlying defect in NPH is decreased sensitivity to the serum ionic calcium feedback inhibition at the parathyroid receptor level and that this sensitivity can improve with age.

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.

scholarly journals Fatal Hypocalcaemia Due to Hungry Bone Syndrome with Secondary Refractory HyperParathyroidism after Parathyroidectomy: A Case Report

2019 ◽  
Vol 5 (4) ◽  
pp. 140-144
Author(s):  
Corina Pop Radu ◽  
Valentin Daniealopol ◽  
Ario Santini ◽  
Ruxandra Darie ◽  
Daniela Tatiana Sala
Keyword(s):  
Case Report ◽  
Cardiopulmonary Resuscitation ◽  
Initial Period ◽  
Parathyroid Tissue ◽  
Total Parathyroidectomy ◽  
Cardiac Symptoms ◽  
Hungry Bone Syndrome ◽  
Ionic Calcium ◽  
Hungry Bone ◽  
Secondary Refractory

Abstract Introduction Hungry bone syndrome (HBS) refers to the rapid, profound, and prolonged hypocalcaemia associated with hypophosphatemia and hypomagnesaemia, and is exacerbated by suppressed parathyroid hormone (PTH) levels, which follows parathyroidectomy in patients with severe primary hyperparathyroidism (PHPT) and preoperative high bone turnover. [1] Case report This report concerns a dialysed patient who underwent surgical treatment for secondary refractory hyperparathyroidism. Haemodialysis was carried out pre-operatively, and subsequently, a total parathyroidectomy with auto-transplantation of parathyroid tissue in the sternocleidomastoid muscle (SCM) was performed. Rapid and progressive hypocalcaemia symptoms developed during the second day postoperatively. Acute cardiac symptoms with tachyarrhythmia, haemodynamic instability and finally asystole occurred, which required cardiopulmonary resuscitation (CPR). The ionic calcium level was 2.2 mg/dL being consistent with a diagnosis of HBS. A second cardiac arrest unresponsive to CPR followed an initial period of normal sinus rhythm. Death ensued shortly after. Before death, the ionic calcium was 3.1 mg/dL. Conclusion HBS, after parathyroidectomy in patients with secondary hyperparathyroidism (SHPT), may be severe, prolonged and sometimes fatal. Generally, HBS symptomatology is that of a mild hypocalcaemia. It can, however, include heart rhythm disturbances with haemodynamic alterations requiring intensive care measurements and even cardiopulmonary resuscitation. A close clinical and laboratory post-parathyroidectomy monitoring of dialysed patients is of the utmost importance.

Neonatal Primary Hyperparathyroidism: Total Parathyroidectomy with Autotransplantation of Cryopreserved Parathyroid Tissue

1986 ◽  
Vol 75 (1) ◽  
pp. 179-182 ◽  
Author(s):  
P. LUTZ ◽  
O. KANE ◽  
A. PFERSDORFF ◽  
F. SEILLER ◽  
P. SAUVAGE ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Tissue ◽  
Total Parathyroidectomy

scholarly journals Radio-guided parathyroidectomy for recurrent renal hyperparathyroidism caused by graft hyperplasia

2014 ◽  
Vol 61 (3) ◽  
pp. 73-76
Author(s):  
Z. Loncar ◽  
K. Tausanovic ◽  
N. Kozarevic ◽  
V. Zivaljevic ◽  
B. Oluic ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Tissue ◽  
Preoperative Assessment ◽  
Sternocleidomastoid Muscle ◽  
Total Parathyroidectomy ◽  
Renal Hyperparathyroidism ◽  
Guided Surgery ◽  
Initial Surgery ◽  
Surgical Options ◽  
99Mtc Sestamibi

Radio-guided surgery offers several advantages in treatment of primary hyperparathyroidism. It is considered less helpful in renal hyperparathyroidism, but it could be of great advantage in the treatment of persistent or recurrent secondary hyperparathyroidism. One of the surgical options for symptomatic renal hyperparathyroidism is total parathyroidectomy with autotransplantation of hyperplastic parathyroid tissue in forearm muscles or sternocleidomastoid muscle. Recurrence can occur and is most likely caused by graft hyperplasia. In this report we present the case of 54-year-old woman with recurrent renal hyperparathyroidism caused by hyperplasia of the graft in sternocleidomastoid muscle. Unfortunately no sutures or clips were placed at initial surgery to identify the location of the parathyroid tissue. The preoperative assessment consisting of 99mTc-sestamibi scintigraphy identified a parathyroid tissue in the in the middle third of sternocleidomastoid muscle. The patient underwent a radio-guided neck re-exploration that allowed a rapid localization and excision of the hyperplastic graft.

scholarly journals Video-assisted thoracoscopic surgery for primary hyperparathyroidism with ectopic parathyroid adenoma in thymus

2021 ◽  
pp. 42-42
Author(s):  
Radoica Jokic ◽  
Jelena Antic ◽  
Ivana Vorgucin ◽  
Mila Stajevic ◽  
Zoran Nikin ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Bone Fractures ◽  
Thoracoscopic Surgery ◽  
Parathyroid Tissue ◽  
Normal Serum ◽  
Video Assisted ◽  
Ectopic Parathyroid ◽  
Mediastinal Parathyroid ◽  
Video Assisted Thoracoscopic Surgery

Introduction. Primary hyperparathyroidism (PHPT) is rare pathology in children (2-5:100,000). In more than 85% of patients, a single adenoma is present, and its extirpation is usually the only treatment a patient needs. In approximately 15-80%of cases, ectopic mediastinal parathyroid tissue can be found inside the thymus. Case outline. Our patient was a 13-year-old boy, who presented with multiple bone fractures in the previous period of time, and fatigue. PTH levels preoperatively were extremely high (1320 pg/ml - more than 19 times higher than normal). Serum calcium was also elevated (total 3.55 mmol/l; ionized 1.41 mmol/l). He was examined and diagnosed as PHPT by a pediatric endocrinologist. Imaging procedures for the preoperative localization of parathyroid adenomas were done (99mTc sestamibi scintigraphy and MRI suggested ectopic mediastinal parathyroid adenoma). The patient underwent video-assisted thoracoscopic surgery procedure. After exploration of the mediastinum and chest, no ectopic parathyroid tissue was found, so total thoracoscopic thymectomy was performed. Final pathological section confirmed parathyroid adenoma inside the thymus. Conclusion. We believe that if no parathyroid tissue is found during surgical exploration of mediastinum, in a child with preoperatively detected parathyroid adenoma in anterior mediastinum, recommendation is to think about possible intrathymal localization and consider removing the thymus. Greater sample size is necessary for higher reliability of this statement.

scholarly journals Nonadenomatous nonencapsulated thymic parathyroid tissue concomitant with primary hyperparathyroidism due to ectopic parathyroid adenoma

2013 ◽  
Vol 57 (9) ◽  
pp. 739-742
Author(s):  
Askin Gungunes ◽  
Mustafa Sahin ◽  
Salih Sinan Gultekin ◽  
Demet Yilmazer ◽  
Alper Dilli ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Parathyroid Adenoma ◽  
Parathyroid Tissue ◽  
Ectopic Parathyroid Adenoma ◽  
Ectopic Parathyroid ◽  
First Time

Primary hyperparathyroidism due to ectopic parathyroid adenoma is not infrequent. Primary hyperparathyroidism caused by unusual thymic nonadenomatous nonencapsulated parathyroid tissue has been reported before. Both can cause unsuccessful neck explorations. Here we presented for the first time a patient with hyperparathyroidism due to ectopic parathyroid adenoma concomitant to the presence of thymic nonadenomatous nonencapsulated parathyroid tissue.

Familial Hyperparathyroidism – Disorders of Growth and Secretion in Hormone-Secretory Tissue

2017 ◽  
Vol 49 (11) ◽  
pp. 805-815 ◽  
Author(s):  
Stephen Marx ◽  
Delmar Lourenco
Keyword(s):  
Primary Hyperparathyroidism ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Parathyroid Tissue ◽  
Total Parathyroidectomy ◽  
Familial Hypocalciuric Hypercalcemia ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Activating Mutation ◽  
Familial Hyperparathyroidism

AbstractSix syndromes of familial hyperparathyroidism are compared: 1) Familial hypocalciuric hypercalcemia (FHH) expresses primary hyperparathyroidism (PHPT) beginning at birth with lifelong hypercalcemia. There is nonsuppressed PTH secretion from outwardly normal parathyroid glands. It reflects germline heterozygous mutation in CASR, GNA11, or AP2S1. 2) Neonatal severe primary hyperparathyroidism is severest of the six syndromes. It requires urgent total parathyroidectomy in infancy. It usually reflects biallelic inactivation of the CASR. 3) Multiple endocrine neoplasia type 1 (MEN1) is most frequently expressed as PHPT with asymmetric enlargement of 3–4 parathyroids. Benign or malignant tumors may occur among 30 other tissues. It is predisposed by germline inactivation of MEN1 or rarely by inactivation of a cyclin dependent kinase inhibitor, and then termed MEN4. 4) Multiple endocrine neoplasia type 2A from RET activating mutation rarely presents as familial hyperparathyroidism, because medullary thyroid cancer and pheochromocytoma are more prominent. 5) Hyperparathyroidism-jaw tumor syndrome (HPT-JT) has frequent PHPT and benign jaw tumors. Twenty percent develop parathyroid cancer. It is predisposed by inactivating mutation in CDC73. 6) Familial isolated hyperparathyroidism causes multiple parathyroid tumors. It can be an incomplete expression of FHH, MEN1, HPT-JT or even of relatives without a shared driver mutation. However, in 20% of families it reflects GCM2 activating mutation. Five of the PHPT syndromes reflect overgrowth of parathyroid tissue; in contrast, familial hypocalciuric hypercalcemia reflects dysregulation of PTH secretion with little or no parathyroid overgrowth. These differences underlie major differences in clinical expression.

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