- Adrenal gland
- Limited Diagnostic Utility of Plasma Adrenocorticotropic Hormone for Differentiation between Adrenal Cushing Syndrome and Cushing Disease
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A Ram Hong, Jung Hee Kim, Eun Shil Hong, I Kyeong Kim, Kyeong Seon Park, Chang Ho Ahn, Sang Wan Kim, Chan Soo Shin, Seong Yeon Kim
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Endocrinol Metab. 2015;30(3):297-304. Published online August 4, 2015
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DOI: https://doi.org/10.3803/EnM.2015.30.3.297
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- Background
Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level. MethodsWe performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. ResultsFifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001). The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively. ConclusionSignificant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.
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Citations
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- Development of diagnostic algorithm for Cushing’s syndrome: a tertiary centre experience
A. Efthymiadis, H. Loo, B. Shine, T. James, B. Keevil, J. W. Tomlinson, A. Pal, R. Pofi Journal of Endocrinological Investigation.2024;[Epub] CrossRef - Eurasian clinical guidelines for the diagnosis and treatment of secondary (symptomatic) forms of arterial hypertension (2022)
I. E. Chazova, N. M. Chikhladze, N. V. Blinova, Zh. E. Belaya, N. M. Danilov, E. M. Elfimova, A. Yu. Litvin, L. Ya. Rozhinskaya, N. Yu. Sviridenko, M. Yu. Shvetsov, V. A. Azizov, E. A. Grigorenko, N. P. Mit’kovskaja, I. I. Mustafaev, A. G. Polupanov, A. S Eurasian heart journal.2023; (1): 6. CrossRef - Diagnosis and Management of Pituitary Adenomas
Nicholas A. Tritos, Karen K. Miller JAMA.2023; 329(16): 1386. CrossRef - Distinct serum steroid profiles between adrenal Cushing syndrome and Cushing disease
Chang Gao, Li Ding, Xiaona Zhang, Menghua Yuan, Shaofang Tang, Wei Li, Yuanyuan Ye, Ming Liu, Qing He Frontiers in Endocrinology.2023;[Epub] CrossRef - The Importance of DHEA-S Levels in Cushing’s Syndrome; Is
There a Cut-off Value in the Differential Diagnosis?
Sema Ciftci, Ozlem Soyluk, Alev Selek, Selvinaz Erol, Zeliha Hekimsoy, Ayse Esen, Huseyin Dursun, Serdar Sahin, Gonca Oruk, Meral Mert, Huseyin Soylu, Banu Sarer Yurekli, Melek Eda Ertorer, Tulay Omma, Mehtap Evran, Mine Adas, Seher Tanrikulu, Kadriye Ayd Hormone and Metabolic Research.2022; 54(04): 232. CrossRef - Epidemiology and Comorbidity of Adrenal Cushing Syndrome: A Nationwide Cohort Study
Chang Ho Ahn, Jung Hee Kim, Man Young Park, Sang Wan Kim The Journal of Clinical Endocrinology & Metabolism.2021; 106(3): e1362. CrossRef - Metabolic changes in serum steroids for diagnosing and subtyping Cushing’s syndrome
Chang Ho Ahn, Chaelin Lee, Jaeyoon Shim, Sung Hye Kong, Su-jin Kim, Yong Hwy Kim, Kyu Eun Lee, Chan Soo Shin, Jung Hee Kim, Man Ho Choi The Journal of Steroid Biochemistry and Molecular Biology.2021; 210: 105856. CrossRef - Clinical and Molecular Characteristics of PRKACA L206R Mutant Cortisol-Producing Adenomas in Korean Patients
Insoon Jang, Su-jin Kim, Ra-Young Song, Kwangsoo Kim, Seongmin Choi, Jang-Seok Lee, Min-Kyeong Gwon, Moon Woo Seong, Kyu Eun Lee, Jung Hee Kim Endocrinology and Metabolism.2021; 36(6): 1287. CrossRef - Clinical Parameters to Distinguish Silent Corticotroph Adenomas from Other Nonfunctioning Pituitary Adenomas
Daham Kim, Cheol Ryong Ku, Se Hee Park, Ju Hyung Moon, Eui Hyun Kim, Sun Ho Kim, Eun Jig Lee World Neurosurgery.2018; 115: e464. CrossRef - Cushing's syndrome: a practical approach to diagnosis and differential diagnoses
Joseph M Pappachan, Christian Hariman, Mahamood Edavalath, Julian Waldron, Fahmy W Hanna Journal of Clinical Pathology.2017; 70(4): 350. CrossRef
- Primary Hyperaparathyroidism due to Cystic Parathyroid Adenoma not Detected on 99mTc-Sestamibi Scan.
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Ah Reum Khang, Eun Ki Kim, Eun Young Nam, Sun Ju Byeon, Jung Hee Kim, Jung Hun Ohn, Eun Shil Hong, Hyung Jin Choi, Kyeong Cheon Jung, Myung Whun Sung, Chan Soo Shin, Kyong Soo Park, Seong Yeon Kim
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Endocrinol Metab. 2012;27(1):83-88. Published online March 1, 2012
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DOI: https://doi.org/10.3803/EnM.2012.27.1.83
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42,158
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- Parathyroid cysts, which can be divided into functional and non-functional cysts, are rare causes of primary hyperparathyroidism. A technetium-99m-methoxyisobutylisonitrile (99mTc-sestamibi) parathyroid scan is a sensitive diagnostic tool for the localization, although it sometimes shows a false-negative result. Here we report a case of presumed cystic parathyroid adenoma based on clinical findings and analysis of cystic fluid with negative findings in a parathyroid scan. A 44-year-old male patient visited the hospital due to leg pain and compressive symptoms (dysphagia, hoarseness) that had started 4-5 months before. His serum calcium level was 14.4 mg/dL and his intact parathyroid hormone (iPTH) had increased to 478.1 pg/mL. On neck computed tomography, a cystic nodule measuring 6.2 cm was detected in the inferior part of the right thyroid gland. Sestamibi uptake for this nodule was not detected on 2-h delayed imaging, and fluorodeoxyglucose positron emission tomography showed only subtle uptake. Fine-needle aspiration was performed and intracystic iPTH had increased to 61,600 pg/mL. Focused parathyroidectomy guided by intraoperative iPTH monitoring led to successful enucleation of the right inferior parathyroid gland. A parathyroid adenoma was confirmed, and his laboratory results had normalized. This study shows that cystic parathyroid adenoma can sometimes be difficult to detect on a 99mTc-sestamibi parathyroid scan.
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Citations
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- Functional parathyroid cystic adenoma: A rare cause of hypercalcemic crisis with primary hyperparathyroidism
BS Sumana, M Sabaretnam, Vijaya Sarathi, Akshatha Savith Indian Journal of Pathology and Microbiology.2015; 58(4): 487. CrossRef - Normocalcemic primary hyperparathyroidism: long-term follow-up associated with multiple adenomas
Larissa Pimentel, Sirley Portela, Alyne Loureiro, Francisco Bandeira Arquivos Brasileiros de Endocrinologia & Metabologia.2014; 58(5): 583. CrossRef
- A Case of von Hippel-Lindau Disease Presenting with Pancreatic Neuroendocrine Tumor.
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Jung Hun Ohn, Junghee Kim, Hyun Jung Lee, Won Woo Seo, Yul Hwang-Bo, Eun Shil Hong, Jin Joo Park, Seong Yeon Kim
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Endocrinol Metab. 2011;26(1):89-91. Published online March 1, 2011
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DOI: https://doi.org/10.3803/EnM.2011.26.1.89
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2,220
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- 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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Citations
Citations to this article as recorded by
- 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
- Association of the Parathyroid Adenoma Volume and the Biochemical Parameters in Primary Hyperparathyroidism.
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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
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Endocrinol Metab. 2011;26(1):62-66. Published online March 1, 2011
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DOI: https://doi.org/10.3803/EnM.2011.26.1.62
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- 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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Citations
Citations to this article as recorded by
- 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
- Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement.
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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
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Endocrinol Metab. 2011;26(1):38-43. Published online March 1, 2011
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DOI: https://doi.org/10.3803/EnM.2011.26.1.38
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- 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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Citations
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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 - Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy
Jung-Eun Choi, Hae Ri Lee, Jung Hun Ohn, Min Kyong Moon, Juri Park, Seong Jin Lee, Moon-Gi Choi, Hyung Joon Yoo, Jung Han Kim, Eun-Gyoung Hong Endocrinology and Metabolism.2014; 29(3): 394. CrossRef
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