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

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.

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.

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.

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 Bone and Mineral Metabolism Phenotypes in MEN1-Related and Sporadic Primary Hyperparathyroidism, before and after Parathyroidectomy

Cells ◽  
2021 ◽  
Vol 10 (8) ◽  
pp. 1895
Author(s):  
Francesca Marini ◽  
Francesca Giusti ◽  
Federica Cioppi ◽  
Davide Maraghelli ◽  
Tiziana Cavalli ◽  
...  
Keyword(s):  
Primary Hyperparathyroidism ◽  
Bone Mass ◽  
Bone Metabolism ◽  
Mineral Metabolism ◽  
Parathyroid Tissue ◽  
Young Patients ◽  
Surgical Removal ◽  
Mineral Density ◽  
Bone Mass Loss ◽  
Before And After

Primary hyperparathyroidism (PHPT) is the most common endocrinopathy in multiple endocrine neoplasia type 1 (MEN1). Persistent levels of increased parathyroid hormone (PTH) result in a higher incidence of osteopenia and osteoporosis compared to the general population. Surgical removal of hyper-functioning parathyroid tissue is the therapy of choice. This retrospective study evaluated the effect of parathyroidectomy (PTX) on bone metabolism and bone mass in two series of patients with MEN1 PHPT and sporadic PHPT (sPHPT) by comparing bone metabolism-related biochemical markers and bone mineral density (BMD) before and after surgery. Our data confirmed, in a higher number of cases than in previously published studies, the efficacy of PTX, not only to rapidly restore normal levels of PTH and calcium, but also to normalize biochemical parameters of bone resorption and bone formation, and to improve spine and femur bone mass, in both MEN1 PHPT and sPHPT. Evaluation of single-patient BMD changes after surgery indicates an individual variable bone mass improvement in a great majority of MEN1 PHPT patients. In MEN1 patients, PTX is strongly suggested in the presence of increased PTH and hypercalcemia to prevent/reduce the early-onset bone mass loss and grant, in young patients, the achievement of the bone mass peak; routine monitoring of bone metabolism and bone mass should start from adolescence. Therapy with anti-fracture drugs is indicated in MEN1 patients with BMD lower than the age-matched normal values.

scholarly journals An Unusual Case of Primary Hyperparathyroidism: Case Report of a Bifocal Intrathyroidal Parathyroid Carcinoma

2020 ◽  
pp. 014556132097746
Author(s):  
Maxime Damien ◽  
Alexandra Rodriguez ◽  
Pierre Kleynen ◽  
Didier Dequanter ◽  
Cyril Bouland
Keyword(s):  
Computed Tomography ◽  
Primary Hyperparathyroidism ◽  
Parathyroid Carcinoma ◽  
Photon Emission ◽  
Parathyroid Tissue ◽  
Emission Computed Tomography ◽  
Thyroid Lobe ◽  
Imaging Results ◽  
Pet Ct ◽  
Left Thyroid Lobe

Intrathyroidal parathyroid carcinoma is an extremely rare cause of primary hyperparathyroidism. We reported a 51-year old woman who presented symptoms of hypercalcemia. 99mTc sestamibi single-photon emission computed tomography/computed tomography (CT) revealed a large hypermetabolic nodule in the left thyroid lobe suggestive of hyperfunctioning parathyroid tissue. 11C-methionine positron emission tomography/computed tomography (PET/CT) and 18F-fluorocholine PET/CT confirmed the nodule in the left thyroid lobe and also revealed a hypermetabolic activity on the posterior surface of the lower left pole. The patient underwent a total thyroidectomy and parathyroidectomy, and a diagnosis of bifocal intrathyroidal parathyroid carcinoma was confirmed. We present the first reported case of bifocal intrathyroidal carcinoma and discuss the discordant imaging results.

scholarly journals Comparison of Different Parathyroid Autograft Project after Total Parathyroidectomy in Patients with Secondary Hyperparathyroidism

2020 ◽  
Author(s):  
mengjia FEI ◽  
dong XU ◽  
yi LAI ◽  
yanan XU ◽  
jingwen ZHANG ◽  
...  
Keyword(s):  
Secondary Hyperparathyroidism ◽  
Parathyroid Tissue ◽  
Total Parathyroidectomy ◽  
Intact Parathyroid Hormone ◽  
Remarkable Difference ◽  
Recurrence Rates ◽  
Biochemical Characteristics ◽  
Parathyroid Function ◽  
Simple Alternative ◽  
Autograft Group

Abstract Objective To evaluate the feasibility and efficacy of total parathyroidectomy followed by modified needle-quantified injection of parathyroid autograft compared with classic incision and transplantation.Methods We conducted a retrospective study of 171 patients with secondary hyperparathyroidism treated by hemodialysis or peritoneal dialysis. These patients were included in our study from April 2006 to December 2016,who had undergone total parathyroidectomies with autotransplantation . Patients were divided into classic incision for transplantation of parathyroid autograft group and modified needle-quantified injection group. Clinical and biochemical characteristics, including preoperative and postoperative intact parathyroid hormone levels were recorded and compared between two group patients. Results To compare the techniques of modified needle-quantified injection and classic incision and transplantation, pre- and postoperative biochemistry and length of operation was recorded and analyzed. Preoperative biochemistry was similarly in both groups. However, autograft function achieved was significantly faster in the group with modified needle-quantified injection compared with classic incision and transplantation (P = 0.03). Median time to parathyroid function regain was 3 months for injection compared with 7 months for classic incision. There was no remarkable difference in the recurrence rates between the two groups.Conclusion The modified needle-quantified injection of parathyroid tissue is a feasible and simple alternative to the more commonly used method of classic incision and transplantation.

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.

scholarly journals SAT-058 A Rare Case of Primary Hyperparathyroidism in a Pediatric Patient

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Natalia Salazar ◽  
Jeff M Merz ◽  
Liliana Burdea ◽  
Carla Minutti
Keyword(s):  
Abdominal Pain ◽  
Primary Hyperparathyroidism ◽  
Clinical Significance ◽  
Parathyroid Tissue ◽  
High Serum ◽  
Genetic Evaluation ◽  
Atypical Presentation ◽  
Pediatric Endocrinology ◽  
Endocrine Disorder ◽  
Ret Gene

Abstract Introduction: Primary hyperparathyroidism (PHPT) is the third most common endocrine disorder in adult patients, but it is rare in pediatric patients. It is usually diagnosed when patients present with symptomatic hypercalcemia or known complications. In children, atypical presentation often results in delayed diagnosis and increased morbidity. We report a 10-year-old boy presenting with abdominal pain and emesis, found to have hypercalcemia and, ultimately, PHPT. His genetic evaluation was notable for a mutation in the RET gene (3.2C>A) of unknown clinical significance. Case presentation: A 10-year-old male with a history of constipation presented to the emergency department with five days of abdominal pain and emesis. Initial workup revealed high serum calcium (Ca) of 17.3 mg/dL (8.7-10.7) and ionized Ca (ICal) of 2.01 mmol/L (0.95-1.32). Further evaluations revealed low phosphorus level 3.5 mg/dL (4.5-6.5) and high parathyroid hormone level (iPTH) of 329.2 pg/mL (8.0-85.0). These findings were consistent with PHPT. Neck US demonstrated a cervical mass in the mid-right thyroid measuring 0.5 x0.3 x0.5cm, questionable for parathyroid adenoma, which was confirmed with 99mTc-MIBI scintigraphy and neck CT. His Ca level initially responded to fluid resuscitation and Lasix, with Ca level decreasing to 13.6. However, on hospital day two, his Ca level became refractory to all interventions, rising to 16 and prompting the use of bisphosphonates. The patient underwent neck exploration with partial parathyroidectomy and lymph node excision. Pathology revealed hypercellular parathyroid tissue consistent with parathyroid hyperplasia. Intraoperatively iPTH was reduced from 3,134.7 to 79.8 and remained within normal limits since. Postoperatively course was uncomplicated, and the patient was discharged home on oral Ca carbonate and vitamin D. A genetic evaluation was remarkable for a change in the RET gene (3.2C>A), a finding of unknown clinical significance. This change has not been seen in association with an individual who fulfills the clinical diagnosis of MEN2A. To further determine if the variant identified in the RET gene is a benign variant, the mother was tested for MEN2A and was negative. Father could not be tested, but family history was significant for thyroid malignancies. The patient is currently doing well four months postoperatively. His Ca level remains normal. Due to the genetic finding and the concern of MEN2A syndrome, the patient is followed closely by pediatric endocrinology and genetics. Conclusion: PHPT is a common endocrine disorder in adults but rare in children. The diagnosis of pediatric PHPT is almost always delayed due to atypical presentation and rarity of the disease. As secondary organ damage is common, a multi-organ assessment is mandatory. Due to the association with other syndromes, a genetic evaluation should be performed.

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