Spondylometaphyseal dysplasia(SMD) is very rare disease characterized with platyspondyly and metaphyseal changes in the long bone. Shrot status and later abnormal gait associated with coxa vara are common clinical feature. A 6 years 4 months old boy progressive bilateral coax vara were transferred with abduction limitation of the hip. With the skeletal survey, multiple dysplastic changes of the metaphysic of the long bone including knee, shoulder, ankle, and flatterning of the body of lower thoracic and upper lumbar spine(T11,12,L1) were noted. A corrective valgus osteotomy on the both hip were followed with improvement of the range of motion.
Ankle ; Coxa Vara ; Gait ; Hip ; Humans ; Knee ; Male ; Metaphysics ; Osteotomy ; Range of Motion, Articular ; Rare Diseases ; Shoulder

PURPOSE: To evaluate incidentally observed thickened mucosa of paranasal sinuses on brain MRI of patients without evidence of sinusitis. MATERIALS AND METHODS: We reviewed brain MRI of 82 adults aged over 20 ; 45 were males and 37 were females. Brain axial MRI was obtained from the hard palate with 8mm thickness and 2mm gap. The mucosal thickness of incidentally observed paranasal sinuses seen on brain MRI was measured at the mostly thickened portion by T2- and T1-weighted images. RESULTS: The mean mucosal thickness at the most thickened portion of paranasal sinuses, regardless of their location was 3.5mm with S.D. of 1.5mm. The mucosal thickening was observed more commonly in maxillary (79 patients, mean 3.0mm, S.D. 1.4mm) and ethmoid sinuses (80 patients,mean 2.7mm, S.D. 1.1mm) than in sphenoid (39 patients, mean 1.6mm, S.D. 1.4mm) or frontal sinuses (38 patients, mean 1.9mm, S.D. 1.4mm). CONCLUSION: Mucosal thickening of up to 6.5mm was a common finding on brain MRI of patients without evidence of sinusitis ; accuracy was 95%.
Adult ; Brain* ; Ethmoid Sinus ; Female ; Frontal Sinus ; Humans ; Magnetic Resonance Imaging ; Male ; Mucous Membrane ; Palate, Hard ; Paranasal Sinuses* ; Reference Values* ; Sinusitis

BACKGROUND: Vitiligo is an acquired depigmentary disorder of the skin and hair. The etiology is unknown, however an autoimmune hypothesis is favored. OBJECTIVE: We performed this study to develop a better understanding of the clinical features of vitiligo patients. METHOD: We evaluated clinical manifestations of 1203 vitiligo patients(556 males and 647 females). RESULTS AND CONCLUSIONS: 1. The mean age of onset and that of the first visits made by patients were 22.9+/-18 and 27.9+/-19 years, respectively. 2. The face(37.4%) was the most common site of initial involvement. In decreasing order of frequency, the common sites of involvement were the face and neck(65.9%), thorax and abdomen(42.9%), upper extremities(42.3%). 44.5% of the cases had the vulgaris type, 26.1% the focal type, and 21.1% the segmental type. During the three months before a visit, 44% of patients experienced progression of disease. Within one year, about 75% of patients reported that the disease had progressed. 3. Precipitating or aggravating factors such as trauma(13.1%), psychological stress(9.2%), sun light (2.8%) and pregnancy(2.5%), were found in 30.9% of patients. Thyroid disease was the most common associated disease.
Age of Onset ; Hair ; Humans ; Male ; Skin ; Solar System ; Thorax ; Thyroid Diseases ; Vitiligo*

BACKGROUND/AIMS: Focal nodular hyperplasia (FNH) is a benign hepatic tumor with few serious complications and no malignant transformation. However, differential diagnosis between FNH and other liver tumors, especially hepatocellular carcinoma, is often difficult. METHODS: Clinical features of surgically resected FNH were reviewed. From January, 1995 to February, 2003, 10 patients with surgically resected FNH were enrolled. Their age, sex, results of laboratory examination, imaging studies and pathologic findings were evaluated. RESULTS: Median age was 37.5 years and sex ratio (male:female) was 1.5:1. In 5 cases, resection to exclude hepatic adenoma or HCC was performed. Four cases were diagnosed incidentally after surgery. Four patients had risk factors for HCC, such as hepatitis B virus infection, liver cirrhosis or both. The size of FNH was 3.2 +/- 2.2 cm. The most common site of the tumor was segment 6 (30.0%). Differential diagnosis with HCC was difficult in 5 of six cases in whom CT was performed. Although needle biopsies were performed preoperatively in 4 cases, it was difficult to distinguish FNH from hepatic adenoma or HCC. CONCLUSIONS: FNH was resected due to uncertainty of diagnosis, or incidentally during hepatectomy in patients with other liver disease. In the former, differential diagnosis with hepatic adenoma or HCC was a major problem despite extensive work-up including dynamic CT or biopsy.
Adult ; English Abstract ; Female ; Focal Nodular Hyperplasia/*diagnosis/pathology/surgery ; Humans ; Infant ; Liver/pathology ; Male ; Middle Aged

PURPOSE: Percutaneous radiofrequency ablation (RFA) for hepatocellular carcinoma (HCC) has some limitations such as poor sonic window and injury to adjacent organs. The laparoscopic approach has been suggested as an alternative option. The aim of this study was to show the safety and efficacy of laparoscopic RFA for single, small (≤3 cm), and primary or recurrent HCC that is not suitable for percutaneous RFA or surgical resection. METHODS: We reviewed the cases of 37 patients (32 men and 5 women, mean age 61 ± 8.1 years) who underwent laparoscopic RFA for single, small HCC (≤3 cm) that was unsuitable for percutaneous RFA or surgical resection. RESULTS: The technical success rate was 94.6% and 34 patients (95%) had no complications. There were no conversions to open RFA and no operative mortality. The primary effectiveness rate 1 month after the procedure was 100%. The overall recurrence rates at 3, 6, 12, and 24 months after the laparoscopic RFA were 8.1%, 14.4%, 25%, and 35.7%, respectively. The local tumor progression rate was 4.2% at 6 months and 8.7% at 9 months. CONCLUSION: Laparoscopic RFA is a safe and effective treatment for HCC cases that are unsuitable for percutaneous RFA.
Carcinoma, Hepatocellular* ; Catheter Ablation* ; Female ; Humans ; Laparoscopy ; Male ; Mortality ; Neoplasm Recurrence, Local ; Recurrence

Background: The risk of device thrombosis and device-oriented clinical outcomes with bioresorbable vascular scaffold (BVS) was reported to be significantly higher than with contemporary drug-eluting stents (DESs). However, optimal device implantation may improve clinical outcomes in patients receiving BVS. The current study evaluated mid-term safety and efficacy of Absorb BVS with meticulous device optimization under intravascular imaging guidance. Methods: The SMART-REWARD and PERSPECTIVE-PCI registries in Korea prospectively enrolled 390 patients with BVS and 675 patients with DES, respectively. The primary endpoint was target vessel failure (TVF) at 2 years and the secondary major endpoint was patientoriented composite outcome (POCO) at 2 years. Results: Patient-level pooled analysis evaluated 1,003 patients (377 patients with BVS and 626 patients with DES). Mean scaffold diameter per lesion was 3.24 ± 0.30 mm in BVS group.Most BVSs were implanted with pre-dilatation (90.9%), intravascular imaging guidance (74.9%), and post-dilatation (73.1%) at proximal to mid segment (81.9%) in target vessel.Patients treated with BVS showed comparable risks of 2-year TVF (2.9% vs. 3.7%, adjusted hazard ratio [HR], 1.283, 95% confidence interval [CI], 0.487–3.378, P = 0.615) and 2-year POCO (4.5% vs. 5.9%, adjusted HR, 1.413, 95% CI, 0.663–3.012,P = 0.370) than those with DES. The rate of 2-year definite or probable device thrombosis (0.3% vs. 0.5%, P = 0.424) was also similar. The sensitivity analyses consistently showed comparable risk of TVF and POCO between the 2 groups. Conclusion With meticulous device optimization under imaging guidance and avoidance of implantation in small vessels, BVS showed comparable risks of 2-year TVF and device thrombosis with DES.