Painful immunoglobulin G4-related thyroiditis treated by total thyroidectomy

No abstract available.

Diagnosis of cyclic Cushing syndrome using the morning urine free cortisol to creatinine ratio

No abstract available.

A novel PRKAR1A mutation resulting in a splicing variant in a case of Carney complex

No abstract available.

Muscular Sarcoidosis Detected by F-18 FDG PET/CT in a Hypercalcemic Patient

Sarcoidosis is a systemic granulomatous disease of unknown etiology that involves many organs, occasionally mimicking malignancy. We herein report a 50-yr-old woman of muscular sarcoidosis of chronic myopathic type, manifested by hypercalcemia and muscle wasting. Besides insignificant hilar lymphadenopathy, her sarcoidosis was confined to generalized atrophic muscles and therefore, F-18 FDG PET/CT alone among conventional imaging studies provided diagnostic clues for the non-parathyroid-related hypercalcemia. On follow-up PET/CT during low-dose steroid treatment, FDG uptake in the muscles disappeared whereas that in the hilar lymph nodes remained. PET/CT may be useful in the evaluation of unexpected disease extent and monitoring treatment response in suspected or known sarcoidosis patients.

A Case of Incidental Struma Ovarii Confounded with the Metastasis of Papillary Thyroid Cancer

A focal radioactive iodine uptake in the pelvis of a patient with differentiated thyroid cancer needs differential diagnosis besides bone metastasis. Struma ovarii is a rare monodermal ovarian teratoma composed predominantly of mature thyroid tissue; 5-10% of these tumors are malignant. As diagnosis and surgery of thyroid cancer have increased recently, incidental cases of struma ovarii, after radioactive iodine treatment, were occasionally reported. Rare cases of ovary metastasis of thyroid cancer were also reported. We report a case of benign struma ovarii incidentally found in a patient with papillary thyroid cancer. The patient showed a sustained high level of thyroglobulin and focal radioactive iodine uptake in the right pelvis, confused with distant metastasis, after total thyroidectomy and radioactive iodine treatment.

Case of Myotonic Dystrophy with Hypogonadotropic Hypogonadism / 대한내과학회지

Myotonic dystrophies (DM) are genetic neuromuscular diseases that have autosomal dominant inheritance and are characterized by progressive muscular weakness. Myotonic dystrophy type 1 (DM1) is caused by the expansion of an unstable CTG repeat in the DMPK (myotonic dystrophy protein kinase) gene on chromosome 19q13.3. Endocrine disorders associated with DM1 include primary hypogonadism with testicular atrophy and insulin resistance. However, DM1 accompanying hypogonodotropic hypogonadism has not previously been reported in Korea. A 56-year-old man who suffered from progressive weakness and walking disturbance for many years was hospitalized due to pneumonia. During his treatment for pneumonia, he received oral hypoglycemic agents because of hyperglycemia. He was diagnosed with DM1, based on the results of an EMG and genetic analyses. He also displayed anosmia and gynecomastia and was diagnosed with hypogonodotropic hypogonadism, based on the results of hormone tests.

A Case of Gastrointestinal Stromal Tumor with Recurrent Hypoglycemia

Non-islet cell tumor induced hypoglycemia (NICTH) is attributable to overproduction of insulin-like growth factor-II (IGF-II) by solid tumors, and these tumors usually originate from mesenchymal or epithelial cells. Gastrointestinal stromal tumor (GIST) is a rare mesenchymal tumor and most commonly find in the gastrointestinal tract. It is usually expresses the CD117 (stem cell factor receptor, c-kit) detected by immunohistochemistry. Hypoglycemia associated with GIST is very rare and this has not yet been reported in Korea. A 72-year-old man was hospitalized due to frequent episodes of confusion. It was observed that non-hyperinsulinemic hypoglycemia, an elevated serum IGF-II level and a huge liver mass. The histology of liver mass showed c-kit (CD117) positivity, which was consistent with GIST, but it was surgically unresectable. He was treated with imatinib mesylate. Although he recieved palliative treatment, he still experienced intermittent fasting hypoglycemia. After 2 months, the serum IGF-II level was even higher than before. We changed imatinib mesylate to sunitinib malate and performed radiotherapy on the liver mass. Although the change of the liver mass was not significant, he did not suffer from hypoglycemia for three months afterwards.

Effect of Octreotide on Patients with Malignant Bowel Obstruction / 한국호스피스완화의료학회지

PURPOSE: Malignant bowel obstruction causes gastrointestinal symptoms and leads to diminished quality of life in patients with advanced cancer. Several studies have shown the efficacy of octreotide for the relief of malignant bowel obstruction-related symptoms. The aim of this study is to assess the efficacy and safety of octreotide in patients with malignant bowel obstruction. METHODS: We retrospectively reviewed medical records of twenty nine patients who had suffered from malignant bowel obstruction without clinical improvement of conservative care and subsequently, received octreotide treatment. Initial dosage of octreotide was 0.1 mg/day, and dose was escalated depending on the clinical effect. For each patient, we assessed visual analogue scale (VAS) of pain, number of vomiting episode, and amount of nasogastric tube drainage. RESULTS: Median dosage of octreotide was 0.2 mg/day (range 0.1~0.6), and median duration from initial medication to death was 20 days (range 2~103). VAS before and after octreotide treatment were 5.6+/-1.24, and 2.7+/-0.96, respectively. The numbers of vomiting episode before and after octreotide treatment were 3.6/day+/-2.5, and 0.4/day+/-0.8, respectively. The mean amounts of nasogastric tube drainage before and after octreotide treatment were 975+/-1,083 cc/day and 115+/-196 cc/day, respectively. Statistically significant reduction in VAS, the number of vomiting episode and the amount of nasogastric tube drainage were observed after octreotide treatment (P<0.05). CONCLUSION: Administration of octreotide in patients with malignant bowel obstruction, which is uncontrolled by other medication, was effective and safe. In such clinical situations, physicians should consider to add of octreotide for symptomatic control.

A case of insulin autoimmune syndrome related to alpha-lipoic acid / 대한내과학회지

Insulin autoimmune syndrome is characterized by spontaneous hypoglycemia, high concentrations of serum immunoreactive insulin, and the presence of autoantibodies to insulin without previous insulin injection. A 71-year-old woman with diabetes, who had been treated with oral hypoglycemic agents, suffered from frequent hypoglycemia. High insulin levels and the presence of insulin autoantibodies were shown, so insulin autoimmune syndrome was diagnosed. Drugs containing sulfhydryl groups play an important role in the pathogenesis of insulin autoimmune syndrome. Alpha-lipoic acid, which contains a sulfhydryl group, was administered before the onset of hypoglycemia. The patient's human leukocyte antigen (HLA) type was HLA-Cw4 and DRB1*0406. There is a strong correlation between HLA-DRB1*0406 and insulin autoimmune syndrome. The patient was treated with prednisolone and has not had a hypoglycemic attack since. We report a case of insulin autoimmune syndrome, possibly associated with alpha-lipoic acid.

A Case of Atypical McCune-Albright Syndrome Associated with Hyperthyroidism / 대한내분비학회지

McCune-Albright syndrome (MAS) is a sporadic disease that's characterized by polyostotic fibrous dysplasia, cafe-au-lait pigmentation of the skin, and multiple endocrinopathies, including sexual precocity, hyperthyroidism, acromegaly, and hypercortisolism. Recent evidence has shown that the clinical manifestations are caused by a postzygotic activating missense mutation in the gene coding for the alpha-subunit of Gs protein that stimulates c-AMP formation in the affected tissues. Substitution of the Arg(201) residue in Gsalpha with cysteine or histidine have been identified in many MAS patients and Arg(201) to Gly or Leu mutations have also been recently identified. We identified the Arg(201) to His mutation in the gene encoding Gsalpha in the thyroid tissue from a 36-year-old man who was suffering with polyostotic fibrous dysplasia and hyperthyroidism.

A Case of Early Gastric Cancer Coincidentally Developed in a Patient with Acromegaly / 대한내분비학회지

Acromegaly is associated with an increased risk for a variety of cancers such as colon cancer, prostate cancer and breast cancer. However, there have been few reports of gastric cancer developing in an acromegaly patient. A 66-year-old man suffered with diabetes mellitus and hypertension for 15 years, and he visited the endocrinology department due to dizziness. On physical examination, the biochemical studies and the sella MRI, he showed the typical features of acromegaly with pituitary microadenoma. During the cancer screening studies to find the cause of anemia, early gastric cancer was diagnosed by pathologic examination of the tissue biopsies. We described the summary of characteristics of the patient and reviewed literature.

A Case of Cerebral Infarction in Young Woman with Graves' Disease and Atrial Fibrillation / 대한내분비학회지

Thyrotoxicosis associated atrial fibrillation occurs in 9 to 22% of hyperthyroidism patients; its prevalence increases after the age 60 years. Atrial fibrillation is known to be major independent risk factor for a thromboembolic stroke. The characterization of patient subgroups with atrial fibrillation, with high or low rate risk factor of a stroke, would help clinicians decide the benefit or harm to patient of long term anticoagulation therapy. Thyrotoxicosis, old age, hypertension, diabetes, heart failure, history of stroke and thromboembolism are all high risk factors for a stroke in atrial fibrillation patients. Thus, anticoagulation therapy is recommended for stroke prevention in those groups with atrial fibrillation and thyrotoxicosis. Herein is reported a case of acute cerebral infarction, with thyrotoxic atrial fibrillation and congestive heart failure, in a young woman

A case of thoracic aortic aneurysm presenting as hoarseness of voice: ortner's syndrome / 대한내과학회지

Ortner's syndrome is a clinical entity with hoarseness due to a left recurrent laryngeal nerve palsy caused by cardiac disease. It is a recognized complication of a number of conditions including, atrial septal defect, patent ductus arteriosus, primary pulmonary hypertension, Eisenmenger's syndrome and aortic aneurysm. A 71-year-old woman with a history of hypertension and diabetes complained hoarseness and hemoptysis for 3 days. Chest X-ray showed a soft tissue mass shadow in the left upper lung field and chest CT scan revealed a thoracic aortic aneurysm. We report a case of left recurrent laryngeal nerve palsy due to a huge thoracic aortic aneurysm in an old woman.

Two cases of severe cholesterol embolism after coronary angiography : Proven by skin biopsy / 대한내과학회지

Cholesterol embolism is due to dislodgment of cholesterol crystals from the atheromatous aorta resulting in an occlusion of small arteries. Cholesterol emboli may occur spontaneously but usually develops as a complication of vascular procedures. The organs most frequently involved are the skin and the kidneys but any organ can be affected. Cutaneous manifestations include livedo reticularis, indurated firm violaceous painful plaques and noduli tending to show central necrosis and tender blue toes. We report two cases of cutaneous cholesterol embolization of lower extremities with the impairment of renal function after coronary angiography, proven by skin biopsy.

A Case of Left Circumflex Coronary Artery Arising as a Terminal Extension of Right Coronary Artery

No abstract available.

A case of spontaneous pneumomediastinum and pneumopericardium in a young adult

Spontaneous medialstinal emphysema (pneumomediastinum) and pneumopericardium may be defined as the presence of free air or gas in the mediastinal structures and in the pericardial sac without an apparent precipitating cause. It most frequently occurs in young healthy adults without serious underlying pulmonary disease. Although pneumomediastinum and pneumopericardium is often asymptomatic, it may cause pain in the neck and chest, dysphonia and shortness of breath. Treatment is supportive unless the patient has a history of trauma from foreign body aspiration. The course of spontaneous pneumomediastinum and pneumopericardium is usually benign and self-limited. A case of spontaneous pneumomediastinum, pneumopericardium and subcutaneous emphysema in a 20-year-old male is reported in this paper

A Case of Acute Cholestatic Hepatitis Caused By the Seeds of Psoralea-corylifolia / 대한간학회지

The potential hepatotoxicity of herbal remedies is usually ignored in daily life. One such compound, Boh-Gol-Zhee (in Chinese, Bu Ku Zi), appeared to be the cause for acute cholestatic hepatic injury in the following case. Psoralea corylifolia is known to contain a sufficient amount of phyto-estrogen. Some alternative medicine therapists claim that Psoralea corylifolia is effective for the treatment of osteoporosis. So far, at least 35 chemical components in the fruits of Psoralea corylifolia have been identified. High concentrations of phyto-estrogen, such as genistein, have been found in the leaves of Psoralea corylifolia. Phyto-estrogen has structural similarity to estrogen and exhibits beneficial effects on osteoporosis under estrogen-deficient conditions. The authors observed a case of acute cholestatic hepatitis in a post-menopausal woman caused by seeds of Psoralea corylifolia in amounts over 10 times the usual dose. Other possible causes of liver injury were excluded by laboratory tests and imaging studies. After discontinuation of this remedy, elevated liver enzyme and total bilirubin rapidly returned to normal. Liver biopsy showed zone 3 necrosis, degenerating cells, cholestasis and infiltrations with inflammatory cells. This case stresses the need to warn the potential hepatotoxicity of Psoralea corylifolia seeds, especially in a large dose.