Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, focal palmoplantar keratoderma and blistering, oral leukokeratosis, cyst formation, palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities. PC is diagnosed by clinical findings and molecular genetic studies. A 26-year-old man presented with hypertrophic nail dystrophy and subungual debris of all 20 nails, hyperkeratotic plaques on the heels of both soles, and oral leukokeratosis. He had no family history of similar clinical findings. The patient's clinical presentation and history were consistent with PC. Herein we report on a rare case of pachyonychia congenita with a review of literature.
Adult ; Blister ; Extremities ; Heel ; Humans ; Keratoderma, Palmoplantar ; Keratosis ; Leukoplakia, Oral ; Molecular Biology ; Nails ; Pachyonychia Congenita

BACKGROUND: Generalized granuloma annulare (GGA) is a benign granulomatous disease of an unknown etiology. Although numerous studies about GGA have been reported, publications that describe the general clinical features of the disease are very sparse. OBJECTIVE: This study aimed to identify the clinical characteristics of Korean GGA cases. METHODS: We reviewed and analyzed the clinical data derived from four patients diagnosed with GGA at our hospital and the clinical data from 58 patients diagnosed with GGA at other Korean hospitals between 1995 and 2011. RESULTS: The cutaneous lesions could be divided into the annular (n=30, 48%) and nonannular (n=32, 52%) types, and the lesions were more common in males than in females, with 33 males and 29 females affected. The GGA incidence showed a bimodal distribution with respect to age at disease onset. Twenty-six cases (42%) presented within the first decade of life and 29 cases (47%) presented when they were in the fifth decade of life or older. Twelve patients (19%) had systemic diseases. Of note, diabetes mellitus (DM) occurred only in adult GGA patients who were aged over 40 years. CONCLUSION: In contrast to previously reported studies, this study shows that the age at GGA onset has a bimodal distribution, and that GGA occurs more often in males. The prevalence of DM among GGA-affected individuals was higher than that found in the general Korean population. Therefore, a DM workup should be undertaken for GGA-affected patients who are over 40 years of age.
Adult ; Diabetes Mellitus ; Female ; Granuloma Annulare* ; Humans ; Incidence ; Korea ; Male ; Prevalence

PURPOSE: We conducted a retrospective analysis to determine if adjuvant chemotherapy prolongs overall survival in patients with pathologic stage IB lung adenocarcinoma who had undergone complete resection and were defined as high-risk by a newly developed recurrence risk scoring model. MATERIALS AND METHODS: Patients who underwent curative resection for stage IB lung adenocarcinoma were analyzed with a newly developed recurrence risk scoring model and divided into a low-risk group and a high-risk group. The patients in the high-risk group were retrospectively divided into two groups based on whether they underwent adjuvant chemotherapy or observation. Recurrence-free survival and overall survival were compared between these two groups. RESULTS: A total of 328 patients who underwent curative resection between 2000 and 2009 were included in this study, of whom 110 (34%) received adjuvant chemotherapy and 218 (67%) underwent observation without additional treatment. According to our risk model, 167 patients (51%) were high-risk and 161 (49%) were low-risk. The 5-year recurrence-free survival rates and overall survival were 84.4% and 91.5% in low-risk patients and 53.9% and 74.7% in high-risk patients (p < 0.001). In high-risk patients, the 5-year overall survival rates were 77% among patients who underwent observation and 87% among those who underwent adjuvant chemotherapy (p=0.019). CONCLUSION: Adjuvant chemotherapy prolonged overall survival among high-risk patients who had undergone complete resection for stage IB lung adenocarcinoma.
Adenocarcinoma* ; Chemotherapy, Adjuvant* ; Humans ; Lung* ; Recurrence* ; Retrospective Studies ; Survival Rate

No abstract available.
Aneurysm* ; Coronary Vessels* ; Sarcoidosis*

No abstract available.
Aneurysm* ; Coronary Vessels* ; Sarcoidosis*

BACKGROUND: Up to now, lobectomy, bilobectomy and pneumonectomy combined with extensive lymph node dissection have been regarded as the standard procedures for non-small cell lung cancer (NSCLC). In high-risk patients, however, limited resection (LR) has been attempted as a salvage procedure, and, recently, indication for LR has been extended to selected cases with early-stage NSCLC. MATERIAL AND METHODS: Among the 773 patients who underwent surgical procedures for NSCLC in Seoul National University Bundang Hospital from May 2003 to December 2008, 43 patients received LR. Medical records of these patients were retrospectively reviewed. RESULTS: Mean age at operation was 66.0+/-12.4 years, and there were 30 males. Twenty-five patients underwent conservative limited resection (CLR) and 18 underwent intentional limited resection (ILR). Indications for CLR were multiple primary lung cancer in 9 (9/25, 36%) and severe concomitant diseases in 5 (5/25, 20%). Of these, 6 patients underwent segmentectomy and 19 received wedge resection. During the follow-up period of 28.0+/-17.8 months, 15 patient developed recurrent lung cancer. ILR was selectively performed in lesions almost purely composed of ground glass opacity (> or =95%), or in small solid lesions (< or =2 cm). Of these, 11 patients underwent segmentectomy and 7 underwent wedge resection. During the follow-up period of 31.7+/-11.6 months, no patient developed recurrence. CONCLUSION: Intermediate-term outcome of LR for early-stage lung cancer is comparable to that of standard operation. For the delineation of the indications and appropriate surgical techniques for LR, prospective randomized multi-institutional study may be expedient.
Carcinoma, Non-Small-Cell Lung ; Follow-Up Studies ; Glass ; Humans ; Lung ; Lung Neoplasms ; Lymph Node Excision ; Male ; Mastectomy, Segmental ; Medical Records ; Pneumonectomy ; Retrospective Studies

PURPOSE: One of the features in cancer development is the migration of cancer cells to form metastatic lesions. CYR61 protein promotes migration and the epithelial-mesenchymal transition in several cancer cell types. Evidence suggests that CYR61 and dexamethasone are relevant to colorectal cancer. However, relationships between them and colorectal cancer are still unclear. Understanding the molecular mechanism of colorectal cancer progression related with CYR61 and dexamethasone, which is widely used for combination chemotherapy, is necessary for improved therapy. MATERIALS AND METHODS: We used colorectal cancer cells, HCT116, co-treated with transforming growth factor β1 (TGF-β1) and dexamethasone to examine the inhibitory migration effect of dexamethasone by migratory assay. Alternatively, both migratory pathways, expression of AKT and ERK, and the target factor CYR61 was also tested by co-treatment with TGF-β1 and dexamethasone. RESULTS: We report that dexamethasone significantly inhibited TGF-β1-induced cell migration, without affecting cell proliferation. Importantly, we observed that TGF-β1 promoted the epithelial-mesenchymal transition process and that dexamethasone co-treatment abolished this effect. ERK and AKT signaling pathways were found to mediate TGF-β1-induced migration, which was inhibited by dexamethasone. In addition, TGF-β1 treatment induced CYR61 expression whereas dexamethasone reduced it. These observations were compatible with the modulation of migration observed following treatment of HCT116 cells with human recombinant CYR61 and anti-CYR61 antibody. Our results also indicated that TGF-β1 enhanced collagen I and reduced matrix metalloproteinase 1 expression, which was reversed by dexamethasone treatment. CONCLUSION: These findings suggested that dexamethasone inhibits AKT and ERK phosphorylation, leading to decreased CYR61 expression, which in turn blocks TGF-β1-induced migration.
Cell Movement* ; Cell Proliferation ; Collagen ; Colon* ; Colonic Neoplasms* ; Colorectal Neoplasms ; Cysteine-Rich Protein 61 ; Dexamethasone* ; Drug Therapy, Combination ; Epithelial-Mesenchymal Transition ; HCT116 Cells ; Humans* ; Matrix Metalloproteinase 1 ; Phosphorylation ; Transforming Growth Factors

BACKGROUND: Goal of the initial treatment of primary spontaneous pneumothorax is re-expansion of the lung by evacuation of air from pleural space. Authors thought small caliber catheter could reach to this goal instead of conventional large bore chest tube. This retrospective study was undertaken to assess the effectiveness of 7-French (Fr) catheter for the initial treatment of primary spontaneous pneumothorax. MATERIAL AND METHOD: Between May 2003 and April 2005, 111 patients with primary spontaneous pneumothorax were managed with tube drainage; 7 Fr catheter for 86 patients and 24-French chest tube for 25 patients. We analyzed catheter indwelling time, use of analgesics, re-expansion of the lung, and catheter related problems by medical records. RESULT: Mean catheter indwelling time was 2.4+/-1.1 days in 7 Fr group and 2.3+/-1.3 days in chest tube group (p>0.05). All patients with 24 Fr catheter needed analgesics injection but never in 7Fr group. Complete re-expansion of the lung based on plane chest radiograph was obtained in 77% of 7 Fr group. The problem related with 7 Fr catheter was kinking, which showed in 5.6%. CONCLUSION: Application of the 7 Fr catheter for initial management of primary spontaneous pneumothorax was as effective as 24 Fr catheter.
Analgesics ; Catheters* ; Chest Tubes ; Drainage ; Humans ; Lung ; Medical Records ; Pneumothorax* ; Radiography, Thoracic ; Retrospective Studies

Sunitinib is a multi-target tyrosine kinase inhibitor used to treat gastrointestinal stromal tumors, renal cell carcinoma, and pancreatic neuroendocrine tumors. The most common adverse reactions are known to be nausea, fatigue, diarrhea, stomatitis, esophagitis, hypertension, skin toxicity (hand-foot syndrome), hypothyroidism, and reduction in the cardiac output of the left ventricle. Herein, we report the case of a 57 year-old female who visited our hospital complaining of epigastric pain. She had been taking sunitinib at 25 mg/day to treat a metastatic pancreatic neuroendocrine tumor. Upon computed tomography performed on admission, we observed that fluid had collected around the pancreas. Laboratory analysis revealed hypertriglyceridemia (triglycerides 993 mg/dL). Tyrosine kinase inhibitors are known to have limited effects on lipid metabolism. In this case, we suggest that hyperglycemia seems to have had a limited effect on lipid levels. We are rather of the view that hyperglycemia, a history of distal pancreatectomy, and hypothyrodisim, indirectly caused the observed hypertriglyceridemia.
Carcinoma, Renal Cell ; Cardiac Output ; Diarrhea ; Esophagitis ; Fatigue ; Female ; Gastrointestinal Stromal Tumors ; Heart Ventricles ; Humans ; Hyperglycemia ; Hypertension ; Hypertriglyceridemia* ; Hypothyroidism ; Lipid Metabolism ; Nausea ; Neuroendocrine Tumors* ; Pancreas ; Pancreatectomy ; Protein-Tyrosine Kinases ; Skin ; Stomatitis

BACKGROUND: The aim of this study was to evaluate the feasibility of single-port video-assisted thoracic surgery (VATS) in the treatment of secondary spontaneous pneumothorax (SSP). METHODS: Twenty-four patients who were scheduled to undergo single-port VATS for SSP were studied. The medical records of the patients were retrospectively reviewed. The mean follow-up duration was 26.1+/-19.8 months. In order to evaluate the feasibility of single-port VATS for SSP, the postoperative results of single-port VATS (n=15) in patients with emphysema were compared with those of emphysematous patients who underwent three-port VATS (n=15) during the study period. RESULTS: Single-port VATS was feasible in 19 of 24 patients (79.2%), while an additional port was needed in five patients. In the single-port VATS patients, the median operation time, duration of chest tube drainage, and hospital stay were 84.0 minutes, one day, and two days, respectively. Postoperative complications included prolonged chest tube drainage for more than five days (n=1), wound infection (n=1), and vocal fold palsy (n=1). No recurrence of pneumothorax was observed during the follow-up period. The median operation time, duration of chest tube drainage, and hospital stay of the emphysematous patients who underwent single-port VATS were shorter than those who underwent three-port VATS group (p<0.05 for all parameters). CONCLUSION: Single-port VATS proved to be a feasible procedure in the treatment of patients with secondary spontaneous pneumothorax.
Chest Tubes ; Drainage ; Emphysema ; Follow-Up Studies ; Humans ; Length of Stay ; Medical Records ; Pneumothorax* ; Postoperative Complications ; Recurrence ; Retrospective Studies ; Thoracic Surgery, Video-Assisted* ; Vocal Cord Paralysis ; Wound Infection