ACTH-independent macronodular adrenal hyperplasia (AIMAH) is an uncommon cause of Cushing's syndrome (CS). The pathophysiology of this disorder is heterogeneous in its molecular origin and also in its clinical presentation. AIMAH can present mainly as an incidental radiological finding with sub-clinical CS or rarely with overt CS. In a few familial cases reported with AIMAH, specific aberrant G-protein coupled receptors were expressed in the adrenals of all affected members, but sporadic cases are more common. The aberrant adrenal function of G-protein coupled receptors can lead to cell proliferation and abnormal regulation of steroidogenesis. Unilateral or bilateral adrenalectomy has been the most frequently used treatment for this adrenal disorder; alternatively, the identification of aberrant receptors using in vivo protocol of investigation can offer specific pharmacological approach to control abnormal steroidogenesis and possibly prevent AIMAH progression.
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Eun Shil Hong, Jung Hun Ohn, Jung Hee Kim, Yul Hwang-Bo, Jin Joo Kim, Jung Hee Kwon, Jung Won Lee, Se Youn Choi, Eun Kyung Lee, Sun Wook Cho, Chan Soo Shin, Kyong Soo Park, Hak Chul Jang, Bo Youn Cho, Hong Kyu Lee, Choong Ho Shin, Sei Won Yang, Seong Yeon Kim
Endocrinol Metab. 2011;26(1):38-43. Published online March 1, 2011
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease that involves a clonal proliferation of Langerhans cells. LCH has a predilection for hypothalamo-pituitary axis (HPA) dysfunction, and this leads to diabetes insipidus (DI) and/or anterior pituitary dysfunction. Here, we describe the endocrine dysfunction and clinical characteristics of adult patients with LCH and we analyzed the differences between an adult-onset type and a childhood-onset type. METHODS: The data was obtained from a retrospective chart review of the patients with LCH that involved the HPA and who attended Seoul National University Hospital. The patients were classified into the adult-onset type (age at the time of diagnosis > or = 16) and the childhood-onset type (age at the time of diagnosis < or = 15). RESULTS: Ten patients (9 males and 1 female) were diagnosed with LCH involving the HPA. Five patients were classified as an adultonset type and the other five patients were classified as a childhood-onset type. The median follow-up duration was 6 (3-12) years for the adult-onset type and 16 (15-22) years for the childhood-onset type. All the patients presented with DI as the initial manifestation of HPA involvement. Four adult-onset patients and three childhood-onset patients had a multi-system disease. Panhypopituitarism developed in three adult-onset patients and in one childhood-onset patient. The pituitary lesion of the three adult-onset patients had spread to the brain during the follow-up duration. In contrast, the pituitary lesion of the other two adult-onset patients without panhypopituitarism and all the childhood-onset patients had not changed. CONCLUSION: DI was the initial presentation symptom of HPA involvement. Anterior pituitary hormone deficiency followed in some patients. Compared with the childhood-onset patients, the adult-onset patients were more likely to have panhypopituitarism and a poor prognosis.
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Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report Yeun Seoung Choi, Jung Soo Lim, Woocheol Kwon, Soon-Hee Jung, Il Hwan Park, Myoung Kyu Lee, Won Yeon Lee, Suk Joong Yong, Seok Jeong Lee, Ye-Ryung Jung, Jiwon Choi, Ji Sun Choi, Joon Taek Jeong, Jin Sae Yoo, Sang-Ha Kim Tuberculosis and Respiratory Diseases.2015; 78(4): 463. CrossRef
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BACKGROUND Cardiovascular disease is the leading cause of death in patients with type 2 diabetes. Clinically, evaluating cardiovascular autonomic neuropathy (CAN) is important to predict cardiovascular mortality because it is correlated with cardiovascular death. The pulse wave velocity (PWV) correlates well with arterial distensibility and stiffness. It is also a useful approach for evaluating the severity of systemic atherosclerosis in adults. So, we evaluated that the relationship between cardiac autonomic neuropathy and the brachial-ankle pulse wave velocity (baPWV) in patients with type 2 diabetes. METHODS: We retrospectively analyzed 465 patients (209 men and 256 women) with type 2 diabetes. We checked the clinical characteristics and the laboratory tests and we assessed the diabetic complications. Standard tests for CAN were performed by DiCAN (Medicore, Seoul, Korea): 1) heart rate variability during deep breathing (the E/I ratio), 2) a Valsalva maneuver, 3) 30:15 ratio of R-R interval the blood pressure response to standing, and 5) the blood pressure response to handgrip. The CAN score was determined according to the results of the test as following: 0 = normal, 0.5 = borderline, 1 = abnormal. We also measured the baPWV by using a VP 1000 (Colin, Japan) and all the analyses were performed with the SPSS version 14.0. P values < 0.05 were considered significant. RESULTS: The CAN score is associated with the maximal baPWV, age, systolic blood pressure, microalbuminuria, the duration of diabetes, angiotensin II receptor blocker treatment, calcium channel blocker treatment, beta-blocker treatment and nephropathy. After adjusting for age, the baPWV is a independent predictor of the risk for CAN (beta = 0.108, P = 0.021). CONCLUSION: The CAN is associated with the baPWV in patient with type 2 diabetes.
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Relationship between Cardiac Autonomic Neuropathy and Brachial-ankle Pulse Wave Velocity in Type 2 Diabetics Sin Gon Kim Endocrinology and Metabolism.2011; 26(1): 33. CrossRef
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Won Gu Kim, Eui Young Kim, Ji Hye Yim, Ji Min Han, Min Ji Jeon, Tae Yong Kim, Jin Sook Ryu, Gyungyub Gong, Suck Joon Hong, Won Bae Kim, Young Kee Shong
Endocrinol Metab. 2011;26(1):53-61. Published online March 1, 2011
BACKGROUND Various staging systems for thyroid cancer that focus on cancer specific death have been suggested, but this approach had a limitation due to the relatively long clinical course and very low rate of cancer death. This study was performed to evaluate the staging systems and to determine the most predictive staging system for predicting recurrence. METHODS: The patients who underwent first total or near total thyroidectomy due to papillary thyroid cancer (PTC) at Asan Medical Center between January 1995 and December 2001 were the subjects of this study. The commonly used 8 staging systems were applied to these subjects. Disease free survival (DFS) and the relative importance of each staging system were determined by the Kaplan-Meier method, the Cox-proportional hazards model and the proportion of variation in the survival time explained (PVE). RESULTS: A total of 952 patients (M = 117, F = 835) were enrolled and their mean age was 45 years. During a median of 10 years of follow-up, 146 (15.3%) of 952 patients had recurred tumor. The independent prognostic factors were male gender, tumor size, extrathyroidal invasion and cervical lymph node metastasis. Risk stratification according to the American thyroid association (ATA) guideline was the most predictive staging system for recurrence of PTC (PVE 88.6%). The staging systems from EORTC (PVE 79.5%), and MACIS (PVE 68.4%) had significant values for predicting recurrence of PTC. The stage of NTCTCS could not predict recurrence (PVE 4.5%, P = 0.11). CONCLUSION: Risk stratification according to the ATA was most predictive staging system for predicting recurrence of PTC. The MACIS and EORTC staging systems have good value for predicting recurrence of PTC.
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Clinical Value of Lymph Node Ratio Integration with the 8th Edition of the UICC TNM Classification and 2015 ATA Risk Stratification Systems for Recurrence Prediction in Papillary Thyroid Cancer Jandee Lee, Seul Gi Lee, Kwangsoon Kim, Seung Hyuk Yim, Haengrang Ryu, Cho Rok Lee, Sang Wook Kang, Jong Ju Jeong, Kee-Hyun Nam, Woong Youn Chung, Young Suk Jo Scientific Reports.2019;[Epub] CrossRef
Clinical prognostic significance of cancer stem cell markers in patients with papillary thyroid carcinoma Yoon‑Jong Ryu, Ji‑Young Choe, Kyoungyul Lee, Soon‑Hyun Ahn Oncology Letters.2019;[Epub] CrossRef
Dynamic Risk Stratification for Predicting Recurrence in Patients with Differentiated Thyroid Cancer Treated Without Radioactive Iodine Remnant Ablation Therapy Suyeon Park, Won Gu Kim, Eyun Song, Hye-Seon Oh, Mijin Kim, Hyemi Kwon, Min Ji Jeon, Tae Yong Kim, Young Kee Shong, Won Bae Kim Thyroid.2017; 27(4): 524. CrossRef
Optimal cut-off age in the TNM Staging system of differentiated thyroid cancer: is 55 years better than 45 years? Mijin Kim, Young Nam Kim, Won Gu Kim, Suyeon Park, Hyemi Kwon, Min Ji Jeon, Hyeon Seon Ahn, Sin-Ho Jung, Sun Wook Kim, Won Bae Kim, Jae Hoon Chung, Young Kee Shong, Tae Hyuk Kim, Tae Yong Kim Clinical Endocrinology.2017; 86(3): 438. CrossRef
Sub-Classification of Lateral Cervical Lymph Node Metastasis in Papillary Thyroid Carcinoma by Pathologic Criteria Min Ji Jeon, Won Gu Kim, Eun Kyung Jang, Yun Mi Choi, Dong Eun Song, Tae-Yon Sung, Jong Ho Yoon, Ki-Wook Chung, Suck Joon Hong, Jin-Sook Ryu, Ji Min Han, Tae Yong Kim, Young Kee Shong, Won Bae Kim, Konradin Metze PLOS ONE.2015; 10(7): e0133625. CrossRef
Recent Changes in the Clinical Outcome of Papillary Thyroid Carcinoma With Cervical Lymph Node Metastasis Min Ji Jeon, Won Gu Kim, Yun Mi Choi, Hyemi Kwon, Dong Eun Song, Yu-Mi Lee, Tae-Yon Sung, Jong Ho Yoon, Suck Joon Hong, Jung Hwan Baek, Jeong Hyun Lee, Jin-Sook Ryu, Tae Yong Kim, Young Kee Shong, Ki-Wook Chung, Won Bae Kim The Journal of Clinical Endocrinology & Metabolism.2015; 100(9): 3470. CrossRef
Differentiating the location of cervical lymph node metastasis is very useful for estimating the risk of distant metastases in papillary thyroid carcinoma Min Ji Jeon, Tae Yong Kim, Won Gu Kim, Ji Min Han, Eun Kyung Jang, Yun Mi Choi, Dong Eun Song, Jong Ho Yoon, Ki‐Wook Chung, Suck Joon Hong, Young Kee Shong, Won Bae Kim Clinical Endocrinology.2014; 81(4): 593. CrossRef
Influences of Hashimoto's Thyroiditis as Prognostic Factor of Papillary Thyroid Carcinoma Hyun Ju Park, Dong Kun Lee, Ji Won Seo, Myung Koo Kang, Heon Soo Park, Rock Bum Kim, Sung Hwan Suh, Mi Kyoung Park, Duk Kyu Kim, Jong Chul Hong Korean Journal of Otorhinolaryngology-Head and Neck Surgery.2014; 57(5): 320. CrossRef
Yul Hwang-Bo, Jung Hee Kim, Jee Hyun An, Eun Shil Hong, Jung Hun Ohn, Eun Ky Kim, Ah Reum Khang, Sun Wook Cho, Do Joon Park, Kyong Soo Park, Seong Yeon Kim, Bo Youn Cho, Chan Soo Shin
Endocrinol Metab. 2011;26(1):62-66. Published online March 1, 2011
BACKGROUND The objective of this study is to demonstrate the relationship between the volume of a parathyroid adenoma and the preoperative biochemical parameters in patients undergoing surgery for primary hyperparathyroidism. METHODS: The medical records of 68 patients who underwent a parathyroidectomy for a single parathyroid adenoma were retrospectively reviewed. The volume of the adenoma was estimated using its measured size and a mathematical formula. The correlation between the volume of the parathyroid adenoma and the preoperative laboratory data was assessed. RESULTS: There were no correlations between the estimated volume of the adenoma and the serum calcium, alkaline phosphatase and parathyroid hormone levels. However, the volume of the adenoma was associated with the preoperative level of serum phosphorus. After excluding 5 adenomas with cystic degeneration, a positive correlation was noted between the adenoma volume and the preoperative levels of alkaline phosphatase and parathyroid hormone. CONCLUSION: The preoperative serum levels of calcium, alkaline phosphatase and parathyroid hormone are of limited use to predict the volume of the parathyroid adenoma in patients with a single parathyroid adenoma. We suggest that the absence of a correlation between the volume of the adenoma and the biochemical parameters can be attributed to the cystic degeneration of the adenomas.
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Differentiating pathologic parathyroid glands from thyroid nodules on neck ultrasound: the PARATH-US cross-sectional study Dolly Yazgi, Carine Richa, Sylvie Salenave, Peter Kamenicky, Amel Bourouina, Lorraine Clavier, Margot Dupeux, Jean-François Papon, Jacques Young, Philippe Chanson, Luigi Maione The Lancet Regional Health - Europe.2023; 35: 100751. CrossRef
Primer hiperparatiroidide Tc99m-MIBI sintigrafi sonuçlarının biyokimyasal ve histopatolojik bulgular ile ilişkisi Seda TURGUT, Ezgi Başak ERDOĞAN OSMANGAZİ JOURNAL OF MEDICINE.2020;[Epub] CrossRef
Letter: Association of the Parathyroid Adenoma Volume and the Biochemical Parameters in Primary Hyperparathyroidism (Endocrinol Metab 26:62-66, 2011, Yul Hwang-Bo et al.) Dong Jin Chung Endocrinology and Metabolism.2011; 26(2): 185. CrossRef
Pituitary stalk interruption and ectopic neurohypophysis seen on magnetic resonance Imaging (MRI) are often associated with either isolated growth hormone (GH) deficiency or combined anterior pituitary hormone deficiency, but their pathogenesis is not clear and the clinical data regarding these anatomical defect is limited. We experienced a 23-year-old male with the absence of secondary sexual characteristics and this was accompanied with pituitary stalk dysgenesis and ectopic neurohypophysis. He received growth hormone for a year when he was 12 years old due to his short stature. Sella MRI showed no visible pituitary stalk with minimal high signal change, suggesting ectopic neurohypophysis. The combined pituitary stimulation test revealed blunted responses of growth hormone, follicle stimulating hormone and luteinizing hormone. For the hypogonadotropic hypogonadism, the patient was given testosterone intramuscularly and he gradually developed secondary sexual characteristics. We concluded that the hypogonadism and growth hormone deficiency in this patient was caused by hypopituitarism due to pituitary stalk dysgenesis and ecopic nuerohypophysis.
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MRI of ectopic posterior pituitary gland with dysgenesis of pituitary stalk in a patient with hypogonadotropic hypogonadism Ashim Kumar Lahiri, Ramanivas Sundareyan, David Jenkins, Anjumara Nilak Radiology Case Reports.2018; 13(4): 764. CrossRef
Hypothalamic Hypopituitarism Caused by Pituitary Stalk Dysgenesis Seong-Ju Lee, Hye-Jin Yoon, A-Reum Cho, Yoo-Jin Um, Keun-Young Park, Dong-Mee Lim, Byung-Joon Kim Korean Journal of Medicine.2013; 85(4): 420. CrossRef
Jin Sun Jang, Jae Seung Yun, Jung Ah Shin, Min Hee Kim, Dong Jun Lim, Jae Hyung Cho, Kun Ho Yoon, Moo Il Kang, Bong Yun Cha, Ho Young Son, Yong Kil Hong
Endocrinol Metab. 2011;26(1):72-77. Published online March 1, 2011
Pituitary abscess is a rare pathology, but it is a potentially life-threatening condition. Therefore, timely intervention, including antibiotics and an operation, can prevent the morbidity and mortality in such cases. A 31-year-old woman, who was 16 months after her second delivery, presented with intermittent headache for 3 months. Amenorrhea, polyuria and polydipsia were noticed and the endocrinological hormone studies were compatible with panhypopituitarism and diabetes insipidus. Pituitary MRI demonstrated a 2.3 cm sized cystic mass with an upper small nodular lesion. Her symptoms such as headache and fever were repeatedly improved whenever corticosteroid was administered, which led us to suspect the diagnosis of an inflammatory condition like lymphocytic hypophysitis. During the hormone replacement therapy, her cystic pituitary mass had grown and her symptoms progressively worsened for another two months. The patient underwent trans-sphenoidal exploration and she turned out to have a pituitary abscess. At the 3-month follow-up, amenorrhea was noticed and her residual function of the pituitary was tested by a combined pituitary stimulation test. The results were compatible with panhypopituitarism. She received levothyroxine 100 microg, prednisolone 5 mg and desmopressin spray and she is being observed at the out-patient clinic. The authors experienced a patient with primary pituitary abscess that was confirmed pathologically and we report on its clinical course with a literature review.
Although pulmonary metastasis of thyroid cancer is not uncommon, it mostly occurs as multiple discrete nodules on the lung parenchyma. Because thyroid cancer presenting with an isolated large lung mass is extremely rare and the diagnosis is frequently based on small pieces of tissue obtained by a fine needle, the wrong diagnosis such as lung cancer is prone to be made. A 60-year-old man was admitted for evaluation of a lung mass detected on chest radiography. Cytological examination of the bronchial washing specimens suggested adenocarcinoma. Surgery for early lung cancer was performed considering that no other abnormalities were found during the work-up that included 18-fludeoxyglucose positron emission tomography computer tomography (18FDG-PET/CT). Unexpectedly, the diagnosis of papillary thyroid cancer with lung metastasis was made, which prompted us to evaluate the thyroid gland and then remove the primary cancer by subsequent operation. Although it is uncommon, physician should be aware of this possibility, which could help to avoid the wrong diagnosis. Here we report on a typical case of solitary pulmonary metastasis of thyroid cancer and we summarize the previously reported cases with a review of the relevant literature.
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Lung nodule detected by F-18 fluorodeoxyglucose positron emission tomography-computed tomography in patients with papillary thyroid cancer, negative 131I whole body scan, and undetectable serum-stimulated thyroglobulin levels: two case reports Chan-Hee Jung, Hyeon-Jeong Goong, Bo-Yeon Kim, Jung-Mi Park, Jeong-Ja Kwak, Chul-Hee Kim, Hyun-Sook Hong, Sung-Koo Kang, Ji-Oh Mok Journal of Medical Case Reports.2012;[Epub] CrossRef
We report here on a case of genetically confirmed type Ia glycogen storage disease (GSD) that was diagnosed in the military hospital. A twenty-year old soldier was admitted to the hospital with abdominal fullness. He had a past medical history of hepatomegaly that was firstly recognized at six months after birth, and he had been followed-up at an outpatient clinic with the presumptive diagnosis of type III GSD. He also had a history of growth hormone therapy because of growth retardation. However, he arbitrarily refused medical observation from 14 years of age. On the physical examination, the height of the patient was 163.1 cm and significant hepatomegaly was observed. Significantly abnormal liver-associated paramters were observed on the laboratory findings and multiple hepatic adenomas were observed on the CT exam and MRI scan. To determine the proper treatment, we tried to confirm the exact type of GSD in the patient. By mutational analysis, we found the c.648G>T homozygote splicing mutation in the G6PC gene and the patient was confirmed as having the type Ia GSD.
Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that results from a germline mutation of the VHL gene. The affected individuals might develop several benign or malignant tumors such as central nervous system or retinal haemangioblastomas, endolymphatic sac tumors, renal cell carcinomas, pheochromocytomas or pancreatic cysts and neuroendocrine tumors. We report here on a case of a 21 year old female with von Hippel-Lindau disease and she presented with only pancreatic neuroendocrine tumor and no evidence of haemangioblastomas or other visceral complications. Further, direct sequencing of the VHL gene reveals a novel germline frameshift mutation of codon 198 from the deletion of nucleotide 592 (cytosine), leading to truncation of the VHL protein.
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Germline mutation of Glu70Lys is highly frequent in Korean patients with von Hippel–Lindau (VHL) disease Sena Hwang, Cheol Ryong Ku, Ji In Lee, Kyu Yeon Hur, Myung-Shik Lee, Chul-Ho Lee, Kyo Yeon Koo, Jin-Sung Lee, Yumie Rhee Journal of Human Genetics.2014; 59(9): 488. CrossRef
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Endocrinol Metab. 2011;26(1):92-96. Published online March 1, 2011
Intra-operative parathyroid hormone (IOPTH) assay is a useful tool to confirm complete excision of all hyper-functioning parathyroid gland tissue. In this report, we describe a case with successful localization of distant metastasis in a patient with parathyroid carcinoma using the IOPTH assay. A 53-year-old man presented to our clinic with a serum calcium level of 11.8 mg/dL and an intact PTH level of 233.3 pg/mL. He had been treated for parathyroid carcinoma eleven years ago. Two suspected metastatic lesions were detected on the chest computed tomography. Due to the vastly different surgical field necessary to excise each lesion, we preferentially removed only one lesion and we monitored the other remaining suspected lesion without resection via IOPTH assay. Six months later, the patient's serum calcium and intact PTH levels remained within their normal ranges. To the best of our knowledge, this is the first case to effectively utilize IOPTH assay for the management of metastatic parathyroid carcinoma.
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