The 46, XX male syndrome is rare disease that is characterized by a phenotypic male who has a 46, XX female karyotype. Since the first report by de la Chapelle and associates in 1964, several cases have been reported, but it is still a rare entity. Recently we examined a 20-year-old XX male who had the symptoms of gynecomastia, an infantile appearance of the external genitalia, scanty pubic hair, no Adams apple, and no axillary hair. We presently describe a patient with the 46, XX male syndrome who showed a 46, XX karyotype on chromosomal study and review the literatures.
46, XX Testicular Disorders of Sex Development* ; Female ; Genitalia ; Gynecomastia ; Hair ; Humans ; Karyotype ; Male ; Rare Diseases ; Young Adult

BACKGROUND AND OBJECTIVES: The recurrence rate of patients with Graves' disease (GD) is estimated to be 50-55% after withdrawal of antithyroid drug therapy, and relapse is frequent in the first year after discontinuing the medication. Follow-up examination of these patients frequently reveals laboratory findings consistent with subclinical thyrotoxicosis in the first year after stopping the antithyroid agents. We investigated the risk of recurrence of GD among patients with resurfacing subclinical thyrotoxicosis state after remission of initial GD with antithyroid treatments. MATERIALS AND METHODS: We reviewed the patients diagnosed with GD who visited the Department of Endocrinology at two tertiary medical centers: Wonju Severance Christian Hospital and Gangneung Asan Hospital. We enrolled patients whose GD was completely treated after initial treatment with antithyroid agents who then developed subclinical thyrotoxicosis after discontinuation of antithyroid agents. RESULTS: We reviewed a total of 44 patients (29 females, 15 males; age, 48.93±18.04; range, 17-85 years). The recurrence rate was 27.3% (12/44 patients), and recurrence occurred 3 months to 12 months later resurfacing of subclinical thyrotoxicosis. Patients with recurred GD was significantly older than non-recurred patients (44.63±17.75 years vs. 58.58±15.48 years, p=0.02). Other clinical parameters measured at the time of initial diagnosis were not different between the two groups. CONCLUSION: The recurrence rate of GD in patients with resurfacing subclinical thyrotoxicosis after initial remission of the disease was less than 30%. A close monitoring is recommended in these subgroup patients, especially in older patients.
Antithyroid Agents* ; Chungcheongnam-do ; Diagnosis ; Drug Therapy ; Endocrinology ; Female ; Follow-Up Studies ; Gangwon-do ; Graves Disease* ; Humans ; Male ; Recurrence* ; Thyrotoxicosis*

In 2012, the American Diabetes Association (ADA) and the European Association for the Study of Diabetes (EASD) published new guidelines for the management of type 2 diabetes, emphasizing the need to individualize treatment goals with preference, need and cost-effects compared with the 2008 ADA/EASD algorithm. These ADA/EASD recommendations provided characteristics of medications in view of improved pharmacodynamics, effects, side effect and cost. The ADA/EASD explained stratification of treatment based on HbA1c and no preferred sequential order for regimens involving dual and triple therapy, indicating the strong effects of insulin therapy in the case of triple therapy. In this section, we summarize "Management of Hyperglycemia in Type 2 Diabetes: A Patient-Centered Approach by the American Diabetes Association (ADA) and the European Association for the Study of Diabetes" in Korean.
Diabetes Mellitus, Type 2 ; Disease Management ; Hyperglycemia ; Insulin ; Patient-Centered Care

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BACKGROUND/AIMS: The present study aimed to investigate the associations between a change in body weight from 20 years of age to the present with insulin resistance and several parameters of metabolic syndrome in individuals at an increased risk of diabetes. METHODS: From patients with a diagnosis related to an increased risk of diabetes who were examined at our endocrinology clinic from January 2012 to December 2014, the present study recruited 125 individuals between 30 and 64 years of age with hemoglobin A1c levels from 5.7 to 6.4%. The change in body weight of each patient from 20 years of age to the present was retrospectively determined, and the associations of this change with insulin resistance and several parameters of metabolic syndrome were analyzed. RESULTS: The average gain in body weight after 20 years of age was 11.3 kg and there was no significance difference between the male and female patients. The proportion of overweight and obese patients at 20 years of age was 14%, but was 74% at the time of the visits to our endocrinology clinic. A change in body weight was significantly correlated with body mass index, insulin resistance, and waist circumference. Additionally, as the tertile of change in body weight increased, fasting insulin levels, insulin resistance, and the prevalence of abdominal obesity significantly increased. CONCLUSIONS: In the present study, an increase in body weight from 20 years of age to the present was correlated with insulin resistance and several parameters of metabolic syndrome in individuals at an increased risk of diabetes.
Body Mass Index ; Body Weight* ; Diagnosis ; Endocrinology ; Fasting ; Female ; Humans ; Insulin Resistance* ; Insulin* ; Male ; Obesity ; Obesity, Abdominal ; Overweight ; Prevalence ; Retrospective Studies ; Waist Circumference

Tracheopathia osteoplastica is a rare disease of unknown cause and characterized by cartilaginous or bony projection into the tracheobronchial lumen, usually not involved posterior membranous portion of tracheobronchial tree. In the past, most of the cases were diagnosed incidentally at autopsy. But after the introduction of bronchoscopy and computed tomography, antemortem diagnosis was reported. Because of initial presenting symptoms were indolent and non-specific, misdiagnosis was reported frequently and correct diagnosis was delayed usually. We report two cases of tracheopathia osteoplastica diagnosed by fiberoptic bronchoscopic biopsy.
Autopsy ; Biopsy ; Bronchoscopy ; Diagnosis ; Diagnostic Errors ; Rare Diseases

Autoimmune thyroiditis in Turner syndrome is more prevalent in women with the X isochromosome karyotype, compared with other karyotypes. The cause of obesity in Turner syndrome is not to be sure, it may be related to metabolic syndrome inducing insulin resistance, hyperlipidemia, and cardiovascular diseases. Also the influence of each karyotypes on degree of obesity is unclear in Turner syndrome. We experienced a case of X isochromosome Turner syndrome with metabolic syndrome and autoimmune thyroiditis and report it with reviews of literatures.
Cardiovascular Diseases ; Female ; Humans ; Hyperlipidemias ; Insulin Resistance ; Isochromosomes* ; Karyotype ; Obesity ; Thyroiditis, Autoimmune* ; Turner Syndrome*

Autoimmune thyroiditis in Turner syndrome is more prevalent in women with the X isochromosome karyotype, compared with other karyotypes. The cause of obesity in Turner syndrome is not to be sure, it may be related to metabolic syndrome inducing insulin resistance, hyperlipidemia, and cardiovascular diseases. Also the influence of each karyotypes on degree of obesity is unclear in Turner syndrome. We experienced a case of X isochromosome Turner syndrome with metabolic syndrome and autoimmune thyroiditis and report it with reviews of literatures.
Cardiovascular Diseases ; Female ; Humans ; Hyperlipidemias ; Insulin Resistance ; Isochromosomes* ; Karyotype ; Obesity ; Thyroiditis, Autoimmune* ; Turner Syndrome*

We report a case of acute suppurative thyroiditis complicated by idiopathic hypoparathyroidism. The patient was a 49-year-old female who visited our clinic with a painful goiter on the left lobe of the thyroid gland. She was hypocalcemic, which was accompanied unusually by acute suppurative thyroiditis. She also suffered from tetany of both hands and legs. She had undergone a right subtotal thyroidectomy at another hospital 23 years previously due to a benign thyroid nodule. Intact parathyroid hormone (PTH) levels were increased, but other laboratory findings were normal. No special treatment was administered for the acute suppurative thyroiditis, except antibiotics, to observe its natural course. Her PTH levels decreased and serum calcium returned to normal. The complication of acute suppurative thyroiditis suggested the possible involvement of idiopathic hypoparathyroidism.
Anti-Bacterial Agents ; Calcium ; Female ; Goiter ; Hand ; Humans ; Hypocalcemia* ; Hypoparathyroidism ; Leg ; Middle Aged ; Parathyroid Hormone ; Tetany ; Thyroid Gland ; Thyroid Nodule ; Thyroidectomy ; Thyroiditis, Suppurative*

Autoimmune hypoglycemia is characterized by hyperinsulinemia, fasting hypoglycemia, and the presence of insulin auto- antibodies without previous exposure to exogenous insulin. We experienced a case of autoimmune hypoglycemia without diabetes mellitus or any evidence of insulinoma. The insulin auto-antibody and insulin receptor auto-antibody were present. We diagnosed the patient as having autoimmune hypoglycemia and treated with glucocorticoid. After treatment, the hypoglycemic symptoms were resolved. However, four months later, the patient was readmitted with transient diabetic ketoacidosis. After recovery, he showed no signs of diabetes mellitus. We believe that insulin auto-antibodies may play a role in autoimmune hypoglycemia and diabetic ketoacidosis, but its role and mechanism are not precisely known. Further studies are needed to define the action mechanisms and the functions of insulin auto-antibodies: here we present case with a relevant literature.
Diabetic Ketoacidosis/*complications/*immunology/pathology ; Human ; Hypoglycemia/*complications/*immunology/pathology ; Insulin Antibodies/*blood ; Magnetic Resonance Imaging ; Male ; Middle Aged