ABSTRACT
Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.
ABSTRACT
Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.
ABSTRACT
The accuracy and convenience of continuous glucose monitoring (CGM), which efficiently evaluates glycemic variability and hypoglycemia, are improving. There are two types of CGM: professional CGM and personal CGM. Personal CGM is subdivided into real-time CGM (rt-CGM) and intermittently scanned CGM (isCGM). CGM is being emphasized in both domestic and foreign diabetes management guidelines. Regardless of age or type of diabetes, CGM is useful for diabetic patients undergoing multiple insulin injection therapy or using an insulin pump. rt-CGM is recommended for all adults with type 1 diabetes (T1D), and can also be used in type 2 diabetes (T2D) treatments using multiple insulin injections. In some cases, short-term or intermittent use of CGM may be helpful for patients with T2D who use insulin therapy other than multiple insulin injections and/or oral hypoglycemic agents. CGM can help to achieve A1C targets in diabetes patients during pregnancy. CGM is a safe and cost-effective alternative to self-monitoring blood glucose in T1D and some T2D patients. CGM used in diabetes management works optimally with proper education, training, and follow up. To achieve the activation of CGM and its associated benefits, it is necessary to secure sufficient repetitive training and time for data analysis, management, and education. Various supports such as compensation, insurance coverage expansion, and reimbursement are required to increase the effectiveness of CGM while considering the scale of benefit recipients, policy priorities, and financial requirements.
ABSTRACT
Growth hormone (GH) deficiency is caused by congenital or acquired causes and occurs in childhood or adulthood. GH replacement therapy brings benefits to body composition, exercise capacity, skeletal health, cardiovascular outcomes, and quality of life. Before initiating GH replacement, GH deficiency should be confirmed through proper stimulation tests, and in cases with proven genetic causes or structural lesions, repeated GH stimulation testing is not necessary. The dosing regimen of GH replacement therapy should be individualized, with the goal of minimizing side effects and maximizing clinical improvements. The Korean Endocrine Society and the Korean Society of Pediatric Endocrinology have developed a position statement on the diagnosis and treatment of GH deficiency. This position statement is based on a systematic review of evidence and expert opinions.
ABSTRACT
The Korean Endocrine Society (KES) published clinical practice guidelines for the treatment of acromegaly in 2011. Since then, the number of acromegaly cases, publications on studies addressing medical treatment of acromegaly, and demands for improvements in insurance coverage have been dramatically increasing. In 2017, the KES Committee of Health Insurance decided to publish a position statement regarding the use of somatostatin analogues in acromegaly. Accordingly, consensus opinions for the position statement were collected after intensive review of the relevant literature and discussions among experts affiliated with the KES, and the Korean Neuroendocrine Study Group. This position statement includes the characteristics, indications, dose, interval (including extended dose interval in case of lanreotide autogel), switching and preoperative use of somatostatin analogues in medical treatment of acromegaly. The recommended approach is based on the expert opinions in case of insufficient clinical evidence, and where discrepancies among the expert opinions were found, the experts voted to determine the recommended approach.
Subject(s)
Acromegaly , Consensus , Expert Testimony , Insurance Coverage , Insurance, Health , Octreotide , SomatostatinABSTRACT
Acromegaly is a chronic disorder caused by excessive growth hormone (GH) secretion. In most cases, the excess GH originates from GH-producing pituitary adenomas. Surgery is the preferred first-line treatment for patients with acromegaly, but medical management is considered when the disease persists after surgery or in cases where patients refuse surgery or are poor candidates for surgery. Somatostatin analogues are commonly used to treat acromegaly. The Korean Endocrine Society and the Korean Neuroendocrine Study Group have developed a position statement for the use of somatostatin analogues in the medical treatment of acromegaly. This position statement is based on evidence from the current literature and expert opinions. In the case of discrepancies among expert opinions, the experts voted to determine the recommended approach.
ABSTRACT
Acromegaly is a chronic disorder caused by excessive growth hormone (GH) secretion. In most cases, the excess GH originates from GH-producing pituitary adenomas. Surgery is the preferred first-line treatment for patients with acromegaly, but medical management is considered when the disease persists after surgery or in cases where patients refuse surgery or are poor candidates for surgery. Somatostatin analogues are commonly used to treat acromegaly. The Korean Endocrine Society and the Korean Neuroendocrine Study Group have developed a position statement for the use of somatostatin analogues in the medical treatment of acromegaly. This position statement is based on evidence from the current literature and expert opinions. In the case of discrepancies among expert opinions, the experts voted to determine the recommended approach.
Subject(s)
Humans , Acromegaly , Expert Testimony , Growth Hormone , Octreotide , Pituitary Neoplasms , SomatostatinABSTRACT
PURPOSE: The volume of thyroid cancer screening and subsequent thyroid fine-needle aspiration (FNA) have rapidly increased in South Korea. We analyzed the thyroid cancer diagnoses/thyroid FNA ratio according to the annual number of FNA to evaluate changes in the diagnosticefficiency of FNA. MATERIALS AND METHODS: This was a nationwide population-based retrospective cohort study. The overall thyroid cancer diagnoses/thyroid FNA ratio and annual incremental thyroid cancer diagnoses/incremental thyroid FNA ratio were indirectly calculated using data obtained from the Korea Central Cancer Registry database and the Korean National Health Insurance Service claims database from 2004 to 2012. Pearson correlation analyses were performed to evaluate the strength of linear associations between variables. RESULTS: The number of thyroid FNA increased from 28,596 to 177,805 (6.2-fold increase) from 2004 to 2012. The overall thyroid cancer diagnoses/thyroid FNA ratio decreased from 36.5% in 2004 to 25.1% in 2012 and was negatively correlated to the number of FNA (R=‒0.977, p < 0.001). The annual incremental thyroid cancer diagnoses/incremental thyroid FNA ratios (range, 15.3% to 30.7%) were always lower than the overall thyroid cancer diagnoses/thyroid FNA ratio in each year and also worsened according to the increase in the number of FNA (R=‒0.853, p=0.007). CONCLUSION: The diagnostic performance of both overall and annual incremental thyroid FNA worsened, whereas the number of thyroid FNA procedures increased. More sophisticated indications for FNA are required to improve its diagnostic efficiency, considering the increased burden of screening-detected thyroid nodules.
Subject(s)
Biopsy, Fine-Needle , Cohort Studies , Korea , Mass Screening , National Health Programs , Retrospective Studies , Thyroid Gland , Thyroid Neoplasms , Thyroid NoduleABSTRACT
BACKGROUND: The aim of this study was to estimate the prevalence and incidence of type 1 diabetes mellitus (T1DM) in Korea. In addition, we planned to do a performance analysis of the Registration Project of Type 1 diabetes for the reimbursement of consumable materials. METHODS: To obtain nationwide data on the incidence and prevalence of T1DM, we extracted claims data from July 2011 to August 2013 from the Registration Project of Type 1 diabetes on the reimbursement of consumable materials in the National Health Insurance (NHI) Database. For a more detailed analysis of the T1DM population in Korea, stratification by gender, age, and area was performed, and prevalence and incidence were calculated. RESULTS: Of the 8,256 subjects enrolled over the 26 months, the male to female ratio was 1 to 1.12, the median age was 37.1 years, and an average of 136 new T1DM patients were registered to the T1DM registry each month, resulting in 1,632 newly diagnosed T1DM patients each year. We found that the incidence rate of new T1DM cases was 3.28 per 100,000 people. The average proportion of T1DM patients compared with each region's population was 0.0125%. The total number of insurance subscribers under the universal compulsory NHI in Korea was 49,662,097, and the total number of diabetes patients, excluding duplication, was 3,762,332. CONCLUSION: The prevalence of T1DM over the course of the study was approximately 0.017% to 0.021% of the entire population of Korea, and the annual incidence of T1DM was 3.28:100,000 overall and 3.25:100,000 for Koreans under 20 years old.
Subject(s)
Female , Humans , Male , Diabetes Mellitus, Type 1 , Epidemiology , Incidence , Insurance , Korea , National Health Programs , PrevalenceABSTRACT
PURPOSE: The severity of non-alcoholic fatty liver disease (NAFLD) in type 2 diabetes mellitus (T2DM) population compared with that in normal glucose tolerance (NGT) individuals has not yet been quantitatively assessed. We investigated the prevalence and the severity of NAFLD in a T2DM population using controlled attenuation parameter (CAP). MATERIALS AND METHODS: Subjects who underwent testing for biomarkers related to T2DM and CAP using Fibroscan® during a regular health check-up were enrolled. CAP values of 250 dB/m and 300 dB/m were selected as the cutoffs for the presence of NAFLD and for moderate to severe NAFLD, respectively. Biomarkers related to T2DM included fasting glucose/insulin, fasting C-peptide, hemoglobin A1c (HbA1c), glycoalbumin, and homeostasis model assessment of insulin resistance of insulin resistance (HOMA-IR). RESULTS: Among 340 study participants (T2DM, n=66; pre-diabetes, n=202; NGT, n=72), the proportion of subjects with NAFLD increased according to the glucose tolerance status (31.9% in NGT; 47.0% in pre-diabetes; 57.6% in T2DM). The median CAP value was significantly higher in subjects with T2DM (265 dB/m) than in those with pre-diabetes (245 dB/m) or NGT (231 dB/m) (all p<0.05). Logistic regression analysis showed that subjects with moderate to severe NAFLD had a 2.8-fold (odds ratio) higher risk of having T2DM than those without NAFLD (p=0.02; 95% confidence interval, 1.21-6.64), and positive correlations between the CAP value and HOMA-IR (ρ=0.407) or fasting C-peptide (ρ=0.402) were demonstrated. CONCLUSION: Subjects with T2DM had a higher prevalence of severe NAFLD than those with NGT. Increased hepatic steatosis was significantly associated with the presence of T2DM, and insulin resistance induced by hepatic fat may be an important mechanistic connection.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Biomarkers/metabolism , C-Peptide/metabolism , Case-Control Studies , Diabetes Mellitus, Type 2/complications , Glycated Hemoglobin/metabolism , Insulin Resistance , Non-alcoholic Fatty Liver Disease/epidemiology , Odds Ratio , PrevalenceABSTRACT
Humoral hypercalcemia of malignancy (HHM) is rarely associated with cholangiocarcinoma (CC), and represents dismal prognosis. A 63-year-old male was admitted for evaluation of an intrahepatic mass. He was diagnosed with HHM associated with locally advanced CC. As the tumor responded to the concurrent chemoradiotherapy with capecitabine and cisplatin, serum calcium level was normalized. However, according to the disease progression, he suffered recurrence of HHM and he expired approximately one year after initial diagnosis. A 68-year-old male who presented with abdominal pain was diagnosed with metastatic CC. After the eighth cycle of gemcitabine and cisplatin, progression of the disease was found with HHM. He was treated with the best supportive care, until his demise approximately one month after the diagnosis of HHM. We report on two cases of HHM associated with CC that demonstrate strong correlation between hypercalcemia and disease burden.
Subject(s)
Humans , Male , Abdominal Pain , Calcium , Chemoradiotherapy , Cholangiocarcinoma , Cisplatin , Deoxycytidine , Disease Progression , Fluorouracil , Hypercalcemia , Paraneoplastic Syndromes , Parathyroid Hormone-Related Protein , Prognosis , Recurrence , CapecitabineABSTRACT
Thyroid disorder is a common disease. Graves' disease is the most frequent cause of thyrotoxicosis and pharmacological treatment is current trends worldwide. Because of the severe adverse effects of propylthiouracil, methimazole or carbimazole should be selected as the drug of choice except for special situations such as women in the first trimester of pregnancy, thyroid storm, or in patients with severe side effects to methimazole. Treatment should continue for 12 to 18 months, but duration can be adjusted depending on the patient. For hypothyroidism, synthetic levothyroxine is the mainstay of treatment. In order to avoid overtreatment, the dosage of levothyroxine should be determined in consideration of the patient's age, sex, bodyweight, general condition, and comorbidities. In subclinical hypothyroidism, thyroid hormone replacement is suggested in patients with thyroid stimulating hormone concentrations >10 mIU/L. For non-elderly patients with high titers of thyroid autoantibodies, patients with dyslipidemia, pregnant patients, and women with infertility or ovulatory dysfunction, treatment with levothyroxine can be considered.
Subject(s)
Female , Humans , Pregnancy , Antithyroid Agents , Autoantibodies , Carbimazole , Comorbidity , Dyslipidemias , Graves Disease , Hyperthyroidism , Hypothyroidism , Infertility , Methimazole , Pregnancy Trimester, First , Propylthiouracil , Thyroid Crisis , Thyroid Diseases , Thyroid Gland , Thyroiditis , Thyrotoxicosis , Thyrotropin , ThyroxineABSTRACT
Reports of metastatic hepatocellular carcinoma (HCC) without a primary liver tumor are rare. Here we present a case of isolated HCC that had metastasized to the pelvic bone without a primary focus. A 73-year-old man presented with severe back and right-leg pain. Radiological examinations, including computed tomography (CT) and magnetic resonance imaging (MRI), revealed a huge mass on the pelvic bone (13x10 cm). He underwent an incisional biopsy, and the results of the subsequent histological examination were consistent with metastatic hepatocellular carcinoma. The tumor cells were positive for cytokeratin (AE1/AE3), hepatocyte paraffin 1, and glypican-3, and negative for CD56, chromogranin A, and synaptophysin on immunohistochemical staining. Examination of the liver by CT, MRI, positron-emission tomography scan, and angiography produced no evidence of a primary tumor. Radiotherapy and transarterial chemoembolization were performed on the pelvic bone, followed by systemic chemotherapy. These combination treatments resulted in tumor regression with necrotic changes. However, multiple lung metastases developed 1 year after the treatment, and the patient was treated with additional systemic chemotherapy.
Subject(s)
Aged , Humans , Male , Bone Neoplasms/diagnosis , Carcinoma, Hepatocellular/pathology , Chemoembolization, Therapeutic , Combined Modality Therapy , Glypicans/metabolism , Keratin-1/metabolism , Keratin-3/metabolism , Liver Neoplasms/pathology , Magnetic Resonance Imaging , Paraffin/metabolism , Pelvic Bones/pathology , Positron-Emission Tomography , Tomography, X-Ray ComputedABSTRACT
Bisphosphonates are the mainstay of osteoporosis treatment. Despite the fact that bisphosphonates have a relatively good safety record and are tolerated well by the majority of patients, serious adverse events have been associated with their use. A 41-year-old man had been diagnosed with osteoporosis and had taken etidronate 200 mg/day daily for 2 years due to the judgmental error. He was referred for the management of refractory bone pain and generalized muscle ache. Serum calcium, phosphate, 25-hydroxy-vitamin D (25(OH)D), and immunoreactive parathyroid hormone (iPTH) were within normal range. Plain X-ray showed multiple fractures. Whole body bone scan confirmed multiple sites of increased bone uptakes. Tetracycline-labeled bone biopsy showed typical findings of osteomalacia. He was diagnosed with iatrogenic, etidronate-induced osteomalacia. The patient received daily parathyroid hormone (PTH) injection for 18 months. PTH effectively reverses impaired bone mineralization caused by etidronate misuse. Currently, he is doing well without bone pain. Bone mineral density significantly increased, and the increased bone uptake was almost normalized after 18 months. This case seems to suggest that human PTH (1-34) therapy, possibly in association with calcium and vitamin D, is associated with important clinical improvements in patients with impaired bone mineralization due to the side effect of bisphosphonate.
Subject(s)
Adult , Humans , Biopsy , Bone Density , Calcification, Physiologic , Calcium , Diphosphonates , Etidronic Acid , Judgment , Muscles , Osteomalacia , Osteoporosis , Parathyroid Hormone , Reference Values , Vitamin DABSTRACT
Development of myoclonus can manifest as a side effect of antiepileptic drugs in subjects with preexisting epilepsy, post-traumatic brain injury, encephalopathy, or focal and multifocal brain lesions. A 69-year-old male showed new onset severe myoclonus and confusion two days after taking 1200 mg gabapentin. The patient had end-stage renal disease secondary to type 2 diabetes and was receiving hemodialysis twice a week. After increasing hemodialysis to three times a week and discontinuing gabapentin, myoclonus spontaneously resolved. Here we report the first case of myoclonus in a Korean subject with diabetic renal failure. We recommend caution in the administration of gabapentin for diabetic subjects with renal disease.
Subject(s)
Aged , Humans , Male , Amines , Anticonvulsants , Brain , Brain Injuries , Cyclohexanecarboxylic Acids , Diabetic Neuropathies , Epilepsy, Post-Traumatic , gamma-Aminobutyric Acid , Kidney Failure, Chronic , Myoclonus , Renal Dialysis , Renal InsufficiencyABSTRACT
Several autoimmune and chronic inflammatory conditions have been consistently linked with an increased risk of hematologic malignancies. Although ankylosing spondylitis (AS) is a chronic inflammatory disease, previous studies have demonstrated that it is not associated with an increase in risk of malignant lymphomas. Cases of AS accompanied by hematologic malignancies such as multiple myeloma, chronic myelogenous leukemia, and Hodgkin's disease have been reported. In Korea, AS with non-Hodgkin's lymphoma or follicular lymphoma has not been reported. We experienced a 38-year-old male who had been diagnosed with follicular lymphoma with bone metastasis, who achieved complete remission after having been treated with chemotherapy, developed new inflammatory back pain. An MRI of his hip showed an active inflammation of the left sacroiliac joint and a positive HLA-B27. The patient was diagnosed with AS and was treated with naproxen, which improved the pain in his back and buttock.
Subject(s)
Adult , Humans , Male , Back Pain , Buttocks , Hematologic Neoplasms , Hip , HLA-B27 Antigen , Hodgkin Disease , Inflammation , Korea , Leukemia, Myelogenous, Chronic, BCR-ABL Positive , Lymphoma , Lymphoma, Follicular , Lymphoma, Non-Hodgkin , Multiple Myeloma , Naproxen , Neoplasm Metastasis , Sacroiliac Joint , Spondylitis, AnkylosingABSTRACT
AA amyloidosis is one of the most significant complications of rheumatoid arthritis characterized by the deposition of amyloid A (AA) in multiple organs and tissues in the body. This disorder displays variable clinical symptoms depending upon the involved organ and a diagnosis is rendered through a biopsy of the affected organ, followed by staining using Congo-red which reveals an apple-green birefringence. Fundamental disease control is critical in the treatment of AA amyloidosis. Anti-tumor necrosis factor alpha (anti TNF-alpha) agents are promising in inducing clinical remission by suppressing systemic inflammation in AA amyloidosis. We report a case of AA amyloidosis in a 71 year old woman with rheumatoid arthritis that responded well to infliximab therapy.