A case of myositis ossificans progressiva is reported. The patient, a 22-year -old progressive ossification for 16 years with severe motion limitation. Bone CT and MRI, bone scanning and muscle biopsy showed multiple ossifying lesions in the connective tissue. All labolatory findings are normal. But serum alkaline pbosphatase become worse with disodium etidronate(EHDP) therapy. The case is presented with the review of literatures.
Biopsy ; Connective Tissue ; Humans ; Magnetic Resonance Imaging ; Myositis Ossificans* ; Myositis*

The acute technical failure of endovascular treatment of chronic total occlusions is most often due to the inability to re-enter the true lumen after occlusion is crossed in a subintimal plane. True lumen re-entry catheters are very effective at gaining wire passage back to the true lumen and facilitating successful endovascular treatment of chronic total occlusions that would otherwise require open bypass. These case reports describe our initial experiences with a new catheter system (the Outback(R) LTDtrade mark catheter) that is designed to allow fluoroscopically controlled re-entry of the true arterial lumen after subintimal guidewire passage during recanalization procedures of arterial occlusions.
Atherosclerosis ; Catheters ; Femoral Artery

Klinefelter syndrome is the most common form of male hypogonadism. It is characterized by small, firm testis, gynecomastia, a variable degree of eunuchoidism, azoospermia, elevated gonadotropin level. Increased frequency of diabetes mellitus, breast cancer, empysema, chronic bronchitis, varicose vein, germ cell neoplasia occurs in Klinefelter syndrome. We report a 19 year-old male patient with diabetes mellitus in association with Klinefelter syndrome, which was confirmed by chromosome analysis. The patient is being treated with insulin for diabetes mellius and with testostrone replacement for Klinefelter syndrome.
Azoospermia ; Breast Neoplasms ; Bronchitis, Chronic ; Diabetes Mellitus ; Eunuchism ; Germ Cells ; Gonadotropins ; Gynecomastia ; Humans ; Hypogonadism ; Insulin ; Klinefelter Syndrome ; Male ; Testis ; Varicose Veins

Peripheral venous hypertension is a rare complication in end-stage renal disease patients undergoing maintenance hemodialysis. Primarily, it is secondary to frequent venous accesses, thrombosis, and to other causes. Venous hypertension may cause pain and edema of the ipsilateral arm; increased venous pressure can prevent acceptable flow rates during dialysis. In this case report, we describe our experience using a Gore-Tex tube graft to treat a venous bypass complicated by peripheral venous hypertension with digital pigmentation and an ulcer with crust. Based on our experience, this technique is as an effective and low-risk surgical option to treat this condition.
Dialysis ; Edema ; Humans ; Hypertension ; Kidney Failure, Chronic ; Pigmentation ; Polytetrafluoroethylene ; Renal Dialysis ; Reoperation ; Thrombosis ; Transplants ; Ulcer ; Venous Pressure

PURPOSE: Precocious puberty has significantly increased recently. While obesity is associated with puberty timing, the relationship between obesity and central precocious puberty (CPP) remains controversial. The purpose of this study was to determine whether insulin resistance is associated with bone age (BA) advancement in girls with CPP. METHODS: We retrospectively analyzed the records of 804 girls referred for puberty evaluation. Anthropometric measurements, BA, sex hormone, sex hormone binding globulin (SHBG), and insulin levels, lipid profiles, and gonadotropin releasing hormone stimulation tests were assessed. Insulin resistance parameters were calculated using the homeostasis model assessment-insulin resistance (HOMA-IR) and quantitative insulin sensitivity check index (QUICKI) models. RESULTS: BA, BA advancement, free estradiol index, insulin, and HOMA-IR increased significantly in girls with high body mass index (BMI) compared with that of girls with low BMI in cases of CPP. HOMA-IR was positively correlated with BA advancement and BMI but negatively correlated with SHBG. QUICKI was negatively correlated with BA advancement and BMI and positively correlated with SHBG. When HOMA-IR increased by 1, the odds for BA advancement increased 120% after adjusting for age and BMI (P=0.033). CONCLUSION: Insulin resistance could be associated with BA advancement in girls with CPP.
Adolescent ; Body Mass Index ; Estradiol ; Female* ; Gonadotropin-Releasing Hormone ; Homeostasis ; Humans ; Insulin Resistance* ; Insulin* ; Luteinizing Hormone ; Obesity ; Puberty ; Puberty, Precocious* ; Retrospective Studies ; Sex Hormone-Binding Globulin

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.
Adolescent ; Bronchi ; Cardiovascular System ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diabetes Mellitus* ; Diagnosis ; Double Outlet Right Ventricle ; Female ; Heterotaxy Syndrome* ; Humans ; Insulin ; Lung ; Pancreas* ; Pulmonary Valve Stenosis

PURPOSE: To evaluate the effectiveness and safety of a new liquid embolic agent in renal arterial embolization in the rabbit, and its clinical applicability. MATERIALS AND METHODS: A new embolic agent, Embol, was obtained by partial hydrolysis of polyvinyl acetate and dissolved in a mixture of 45% ethanol and 55% non-ionic contrast medium. Its radioopacity was therefore good. An average of 0.8 cc(0.5-0.9 cc) of Embol was used to embolize the renal artery of one kidney in 15 rabbits. The immediate effect of this was examined angiographically 5 minutes after the procedure. To permit histologic examination, five rabbits in each group were sacrificed 3 days (I), 2 weeks (II), and 4 weeks (III) after embolization: prior to embolization and prior to sacrifice, one rabbit in each group underwent renal scanning, and prior to sacrifice all underwent follow-up angiography. In three rabbits, blood urea nitrogen (BUN), creatinine, sodium(Na), and potassium(K) levels were measured before and 1, 3, 5, 7 and 14 days after embolization. RESULTS: Embol was easy to use and its radiopacity was good. Five minutes after embolization, angiography showed that total occlusion of the main renal or interlobar artery had been achieved in all rabbits. Serum BUN, creatinine, Na and K levels were within normal limits. Follow-up angiogram obtained in each group showed persistent occlusion of the renal artery in all but one rabbit in group I and one in group III. Renal scans revealed no evidence of radionuclide uptake in embolized kidneys, which were slightly enlarged in group I but became gradually smaller in groups II and III. In all animals, histologic examination showed diffuse coagulation necrosis of the embolized kidneys and in group III the cortex of these was extensively calcified. In group I the renal artery showed an apparently fresh occluding thrombosis, and in groups II and III a completely organized thrombosis was present. In group III this was calcified. CONCLUSION: Because of its good radioopacity, Embol is easy to controa, and is effective for renal artery embolization. As a permanent embolic agent, it appears suitable for clinical applications.
Angiography ; Animals ; Arteries ; Blood Urea Nitrogen ; Creatinine ; Ethanol ; Follow-Up Studies ; Hydrolysis ; Kidney ; Necrosis ; Polyvinyls ; Rabbits ; Renal Artery* ; Thrombosis

Systemic hypotension has been traditionally used to facilitate deployment of thoracic stent grafts. Decreasing blood pressure with vasodilating agents further increases cardiac output and, consequently, the cardiac output-mediated windsock effect during deployment. Use of rapid ventricular pacing reduces the windsock effect during stent graft deployment and allows the graft to appose to the aortic wall under zero cardiac output, thus minimizing aortic wall shear stress. In this case we report the use of transvenous rapid ventricular pacing, a safe and reproducible technique to allow precise deployment of a Valiant Captivia stent graft in the distal thoracic arch for a saccular thoracic aneurysm.
Aneurysm ; Aorta, Thoracic ; Blood Pressure ; Cardiac Output ; Hypotension ; Stents ; Transplants

BACKGROUND/AIMS: Aims: Inflammation is an important factor in renal injury. Ferritin, an inflammatory marker, is considered an independent predictor of rapid renal progression in patients with chronic kidney disease. However, the relationship between ferritin and residual renal function (RRF) in patients undergoing peritoneal dialysis (PD) remains unclear. METHODS: We reviewed the medical records of patients who started PD between June 2001 and March 2012 at Soonchunhyang University Bucheon Hospital, Korea. A total of 123 patients were enrolled in the study. At 1 month after the initiation of PD, RRF was determined by a 24-hour urine collection and measured every 6 months thereafter. Clinical and biochemical data at the time of the initial 24-hour urine collection were considered as baseline. RESULTS: The RRF reduction rate was significantly greater in patients with high ferritin (ferritin > or = 250 ng/mL) compared with those with low ferritin (ferritin < 250 ng/mL; -1.71 +/- 1.36 mL/min/yr/1.73 m2 vs. -0.84 +/- 1.63 mL/min/yr/1.73 m2, respectively; p = 0.007). Pearson correlation analysis revealed a significant negative correlation between the baseline serum ferritin level and the RRF reduction rate (r = -0.219, p = 0.015). Using multiple linear regression analysis and adjusting for other risk factors, baseline serum ferritin was an independent factor for the RRF reduction rate (beta = -0.002, p = 0.002). CONCLUSIONS: In this study we showed that a higher ferritin level was significantly associated with a more rapid RRF decline in patients undergoing PD.
Adult ; Aged ; Biological Markers/blood ; Chi-Square Distribution ; Disease Progression ; Female ; Ferritins/*blood ; Hospitals, University ; Humans ; Inflammation Mediators/*blood ; Kaplan-Meier Estimate ; Kidney/*physiopathology ; Kidney Failure, Chronic/blood/diagnosis/physiopathology/*therapy ; Linear Models ; Male ; Middle Aged ; Multivariate Analysis ; Peritoneal Dialysis/*adverse effects ; Predictive Value of Tests ; Proportional Hazards Models ; Republic of Korea ; Risk Factors ; Time Factors ; Treatment Outcome ; Up-Regulation

Portal vein thrombosis (PVT) is commonly associated with liver cirrhosis, irrespective of the presence of hepatocellular carcinoma (HCC). Given that malignant PVT is a poor prognostic factor in patients with HCC, it is important to differentiate malignant PVT from benign PVT. Because malignant PVT has been reported to be contiguous with parenchymal HCC, in most cases, the presence of PVT alone indicates a benign entity. We report the case of a patient with rapid progression of malignant PVT mimicking benign PVT but without definite parenchymal HCC on imaging modalities.
Carcinoma, Hepatocellular ; Disease Progression ; Humans ; Liver Cirrhosis ; Magnetic Resonance Imaging ; Portal Vein ; Thrombosis