With the increasing use of culture-expanded mesenchymal stromal cells (MSCs) for cell therapies, factors that regulate the cellular characteristics of MSCs have been of major interest. Oxygen concentration has been shown to influence the functions of MSCs, as well as other normal and malignant stem cells. However, the underlying mechanisms of hypoxic responses and the precise role of hypoxia-inducible factor-1alpha (Hif-1alpha), the master regulatory protein of hypoxia, in MSCs remain unclear, due to the limited span of Hif-1alpha stabilization and the complex network of hypoxic responses. In this study, to further define the significance of Hif-1alpha in MSC function during their self-renewal and terminal differentiation, we established adult bone marrow (BM)-derived MSCs that are able to sustain high level expression of ubiquitin-resistant Hif-1alpha during such long-term biological processes. Using this model, we show that the stabilization of Hif-1alpha proteins exerts a selective influence on colony-forming mesenchymal progenitors promoting their self-renewal and proliferation, without affecting the proliferation of the MSC mass population. Moreover, Hif-1alpha stabilization in MSCs led to the induction of pluripotent genes (oct-4 and klf-4) and the inhibition of their terminal differentiation into osteogenic and adipogenic lineages. These results provide insights into the previously unrecognized roles of Hif-1alpha proteins in maintaining the primitive state of primary MSCs and on the cellular heterogeneities in hypoxic responses among MSC populations.
*Cell Differentiation ; Cell Proliferation ; Humans ; Hypoxia-Inducible Factor 1, alpha Subunit/genetics/*metabolism ; Kruppel-Like Transcription Factors/genetics/metabolism ; Mesenchymal Stromal Cells/cytology/*metabolism/physiology ; Octamer Transcription Factor-3/genetics/metabolism ; Protein Stability

Pachyonychia congenita (PC) is a rare autosomal-dominant ectodermal dysplasia, which is characterized by hypertrophic nail dystrophy, diffuse or focal symmetrical hyperkeratosis of the palms and soles, oral leukokeratosis, follicular keratosis on the extensor surfaces of the extremities, and other ectodermal defects. However, the variable degree of gene mutation may result in nail abnormalities alone. The nail changes presented in our patient are the only clinical manifestation. Hypertrophic nail dystrophy and yellowish discoloration, without involving any other organs, were shown in both mother and baby since birth. To the best of our knowledge, the case of PC affecting only the nails has never been reported in Korea before. Herein, we report monosymptomatic form of PC, which is notable for its rareness and unique pattern, distinct from previously reported cases.
Ectoderm ; Ectodermal Dysplasia ; Extremities ; Humans ; Keratosis ; Korea ; Leukoplakia, Oral ; Mothers ; Nails ; Nails, Malformed ; Pachyonychia Congenita ; Parturition

Parry and Romberg described progressive facial hemiatrophy, characterized by progressive unilateral wasting of the skin, fat, muscle and bone in 1825 and 1846, respectively. Involvement of the central nervous system, with impairment of neurologic function, occurs infrequently. Linear scleroderma on frontal and parietal scalp, named 'en coup de sabre', is considered an overlapping condition. An 11 year-old girl was presented to our office with left facial hemiatrophy and transverse linear band-like alopecia on her posterior parietal scalp. Histopathologically, there was a sclerotic change of the collagen fibers and dermal lymphocytic infiltrations around the skin appendages. Her skin lesion has improved by intralesional corticosteroid injection. Linear scleroderma frequently coexists with Parry-Romberg syndrome. But in this case, she had a unique pattern of scleroderma, which is distinct from the previously reported cases.
Alopecia ; Central Nervous System ; Collagen ; Facial Hemiatrophy ; Muscles ; Scalp ; Scleroderma, Localized ; Skin

Although the incidence of bleeding complications during extracorporeal membrane oxygenator (ECMO) support has decreased in various trials, bleeding is still the most fatal complication. We investigated the ideal dosage and efficacy of nafamostat mesilate for use with ECMO in patients with acute cardiac or respiratory failure. We assessed 73 consecutive patients who received ECMO due to acute cardiac or respiratory failure between January 2006 and December 2009. To evaluate the efficacy of nafamostat mesilate, we divided the patients into 2 groups according to the anticoagulants used during ECMO support. All patients of nafamostat mesilate group were male with a mean age of 49.2 yr. Six, 3, 5, and 3 patients were diagnosed with acute myocardial infarction, cardiac arrest, septic shock, and acute respiratory distress syndrome, respectively. The mean dosage of nafamostat mesilate was 0.64 mg/kg/hr, and the mean duration of ECMO was 270.7 hr. The daily volume of transfused packed red blood cells, fresh frozen plasma, and cryoprecipitate and the number of complications related to hemorrhage and thrombosis was lower in the nafamostat mesilate group than in the heparin group. Nafamostat mesilate should be considered as an alternative anticoagulant to heparin to reduce bleeding complications during ECMO.
Acute Disease ; Anticoagulants/*administration & dosage ; Dose-Response Relationship, Drug ; *Extracorporeal Membrane Oxygenation ; Female ; Guanidines/*administration & dosage ; Heart Failure/diagnosis/mortality/therapy ; Heparin/administration & dosage ; Humans ; Male ; Middle Aged ; Myocardial Infarction/diagnosis/mortality/therapy ; Respiratory Distress Syndrome, Adult/diagnosis/mortality/therapy ; Retrospective Studies ; Shock, Septic/diagnosis/mortality/therapy ; Survival Analysis

PURPOSE: A laparoscopic cholecystectomy is the treatment of choice for gallbladder disease. The aim of this study is to analyse 2,239 cases of laparoscopic cholecystectomy (LC) and observe the changes of 15th-year gallbladder disease. METHODS: We performed a retrospective analysis of 2,239 patients that underwent a LC by a single surgeon, from March 1993 to December 2007. We divided it into 3 groups; group 1 was from March 1993 to December 1996 and group 2 was from January 1998 to December to 2001, group 3 was from January 2003 to December 2007. RESULTS: The mean age, hospital stay, mean operation time and conversion rate was decreased. Gallbladder cancer of group 1 was 0.6%, group 2 was 0.5%, and group 3 was 1.6% (P<0.05). Gallbladder polyps of group 1 was 3.3%, group 2 was 5.1%, and group 3 was 11.8%. There was statistically significance (P<0.05). Gallstone was significantly decreased (group 1 was 98.1%, group 2 was 97.6%, group 3 was 92.8%) in proportion, pigment stone was significantly decreased (group 1 was 31.5%, group 2 was 27.5%, group 3 was 21.7%) and Cholesterol & Mixed stone was significantly increased (group 1 was 68.2%, group 2 was 72.5%, group 3 was 78.3%)(P<0.05). CONCLUSION: As surgical experience increased with a rising number of cases, the conversion rate, complications, hospital stay and mean operation time was decreased. Pigment gallstone was decreased and cholesterol gallstone was increased owing to western diet, old age and fatness.
Cholecystectomy, Laparoscopic ; Cholesterol ; Diet ; Gallbladder ; Gallbladder Diseases ; Gallbladder Neoplasms ; Gallstones ; Humans ; Length of Stay ; Polyps ; Retrospective Studies

PURPOSE: Most technical difficulties in intracranial stenting are derived from the vascular resistance caused by the severe tortuousness of intracranial arteries. The purpose of this study was to develop a practical method for measuring vascular tortuousness so that it would be possible to predict technical difficulties requiring further technical support. MATERIALS AND METHODS: We developed a best-fit circle metrics which made measurement of vascular tortuousness feasible, which was called "curve index (CI)". We compared the curve index in 56 consecutive patients who underwent M1 stenting for symptomatic severe stenosis. The difference in the CI between the successful and the aborted groups was statistically compared by using the Mann-Whitney U test. ROC curve analysis was performed to evaluate the diagnostic performance of the best-fit circle metrics. RESULTS: There was no statistically significant difference between the successful and the aborted cases in the CIs of each curve segment. However, the sum of all CIs of the aborted group was significantly larger (3.49) than that of the successful group (2.53) (p=0.013). On ROC curve analysis, the area under the curve was 0.806. When we took the cut-off value to be 3, the sensitivity was 75% and the specificity 85%. CONCLUSION: We developed a practical method for measuring the CI of vessel curves in order to estimate the tortuousness of the internal carotid artery. A CI less than 3, therefore, indicates a favorable vascular curvature for the intracranial stenting procedure. A vessel having a higher curve index was more likely to be aborted.
Arteries ; Carotid Artery, Internal* ; Constriction, Pathologic ; Humans ; ROC Curve ; Sensitivity and Specificity ; Stents ; Vascular Resistance

Choledochoduodenal fistula (CDF) occurring simultaneously with pancreaticoduodenal fistula is extremely rare. CDF has known to be a chronic sequela of cholelithiasis, but it is unknown whether pancreaticoduodenal fistula results from chronic cholelithiasis as well. We report a case of cholelithiasis accompanied with choledochoduodenal and pancreaticoduodenal fistula opening into small suprapapillary diverticulum in a 80-year-old woman.
Aged, 80 and over ; Biliary Fistula/*complications ; Cholelithiasis/complications ; Common Bile Duct Diseases/*complications ; Diverticulum/*complications ; Duodenal Diseases/*complications ; Female ; Humans ; Intestinal Fistula/*complications ; Pancreatic Fistula/*complications

A paraffin injection is regarded as to be a simple and effective method of breast augmentation. However, there are few reports on the long-term complication of a paraffin injected breast. The generation of breast cancer from a paraffinoma of the breast is rare, even though there is no clear evidence to suggest any relationship between a paraffin injection and breast cancer. We encountered a case of infiltrative ductal carcinoma arising from paraffinoma tissues. A 49-year-old woman had undergone bilateral breast augmentation by paraffin injection 20 years earlier. She presented with bilateral diffuse painless palpable masses with reddish discoloration and painful swelling in her left breast. She had been managed with a bilateral simple mastectomy including the mass. After discharge, the pathology report showed infiltrative ductal carcinoma of the left breast mass. The patient was readmitted for additional surgery, and she underwent a modified radical mastectomy. Ultrasonography is a useful diagnostic tool for detecting a breast mass, particularly in a dense breast. However, ultrasonography has a limitation in the case of a paraffinoma. Through this case, it is necessary to review the radiological (mammography, ultrasonography, magnetic resonance imaging, plain film) appearances and the histopathological feature to help make an accurate diagnosis and to differentiate between a carcinoma and a paraffinoma. In rare cases, a breast ductal carcinoma can be combined with a paraffinoma. Therefore, a paraffinoma must be carefully managed due to the potential risk of a carcinoma.
Breast Neoplasms* ; Breast* ; Carcinoma, Ductal ; Diagnosis ; Female ; Humans ; Magnetic Resonance Imaging ; Mastectomy, Modified Radical ; Mastectomy, Simple ; Middle Aged ; Paraffin ; Pathology ; Ultrasonography

A paraffin injection is regarded as to be a simple and effective method of breast augmentation. However, there are few reports on the long-term complication of a paraffin injected breast. The generation of breast cancer from a paraffinoma of the breast is rare, even though there is no clear evidence to suggest any relationship between a paraffin injection and breast cancer. We encountered a case of infiltrative ductal carcinoma arising from paraffinoma tissues. A 49-year-old woman had undergone bilateral breast augmentation by paraffin injection 20 years earlier. She presented with bilateral diffuse painless palpable masses with reddish discoloration and painful swelling in her left breast. She had been managed with a bilateral simple mastectomy including the mass. After discharge, the pathology report showed infiltrative ductal carcinoma of the left breast mass. The patient was readmitted for additional surgery, and she underwent a modified radical mastectomy. Ultrasonography is a useful diagnostic tool for detecting a breast mass, particularly in a dense breast. However, ultrasonography has a limitation in the case of a paraffinoma. Through this case, it is necessary to review the radiological (mammography, ultrasonography, magnetic resonance imaging, plain film) appearances and the histopathological feature to help make an accurate diagnosis and to differentiate between a carcinoma and a paraffinoma. In rare cases, a breast ductal carcinoma can be combined with a paraffinoma. Therefore, a paraffinoma must be carefully managed due to the potential risk of a carcinoma.
Breast Neoplasms* ; Breast* ; Carcinoma, Ductal ; Diagnosis ; Female ; Humans ; Magnetic Resonance Imaging ; Mastectomy, Modified Radical ; Mastectomy, Simple ; Middle Aged ; Paraffin ; Pathology ; Ultrasonography

Eosinophilic fasciitis (EF) is a rare disorder of unknown etiology characterized by eosinophilia, indurations of the skin, and inflammation of the fascia. Magnetic resonance imaging (MRI) has been used to assist the diagnosis and to monitor the response to treatment, and the commonly used therapy has been corticosteroids. However, the use of ultrasonography (US) in the diagnosis and the management of EF has not been reported previously in Korea. In addition, the effects of methotrexate (MTX) treatment have rarely been reported in corticosteroid-resistant patients with EF. Here, we describe two cases of EF resistant to corticosteroid therapy alone where the combination of MTX and corticosteroids was effective. Furthermore, we show that US is useful modality in supporting the clinical diagnosis and monitoring the response to treatment of patients with EF.
Adrenal Cortex Hormones ; Diagnosis ; Eosinophilia ; Eosinophils* ; Fascia ; Fasciitis* ; Humans ; Inflammation ; Korea ; Magnetic Resonance Imaging ; Methotrexate* ; Skin ; Ultrasonography*