No abstract available.
Dyskeratosis Congenita*

BACKGROUND: Many difficulties exist in establishing a treatment plan for slow-flow vascular malformation (SFVM). In particular, little research has been conducted on the surgical treatment of SFVMs. Thus, we investigated what proportion of SFVM patients were candidates for surgical treatment in clinical practice and how useful surgical treatment was in those patients. METHODS: This study included 109 SFVM patients who received care at the authors' clinic from 2007 to 2015. We classified the patients as operable or non-operable, and analyzed whether the operability and the extent of the excision varied according to the subtype and location of the SFVM. Additionally, we investigated complications and self-assessed satisfaction scores. RESULTS: Of the 109 SFVM patients, 59 (54%) were operable, while 50 (46%) were non-operable. Total excision could be performed in 44% of the operable SFVM patients. Lymphatic malformations were frequently non-operable, while capillary malformations were relatively operable (P=0.042). Total excision of venous malformations could generally be performed, while lymphatic malformations and combined vascular malformations generally could only undergo partial excision (P=0.048). Complications occurred in 11% of the SFVM patients who underwent surgery; these were minor complications, except for 1 case. The average overall satisfaction score was 4.19 out of 5. CONCLUSIONS: Based on many years of experience, we found that approximately half (54%) of SFVM patients were able to undergo surgery, and around half (44%) of those patients were able to fully recover after a total excision. Among the patients who underwent surgical treatment, high satisfaction was found overall and relatively few complications were reported.
Capillaries ; Follow-Up Studies ; Humans ; Treatment Outcome ; Vascular Malformations*

PURPOSE: The aim of this study was to estimate the antioxidant activities of 50%, 70%, and 100% ethanol extracts of Lycium barbarum leaf and chlorophyll removal extract. METHODS: The antioxidant activities were estimated by measuring total polyphenol content and by assays of 2,2-diphenyl-1-picrylhydrazyl (DPPH) and 2,2′-azino-bis (3-ethylbenzothiazoline-6-sulfate) (ABTS) radical scavenging activities and ferric reducing antioxidant power (FRAP). In addition, reactive oxygen species (ROS) production, DNA fragmentation, and antioxidant enzyme (superoxide dismutase and catalase) activities of the extracts were measured in hydrogen peroxide (H2O2)-stressed HepG2 cells. RESULTS: The total polyphenol content, DPPH and ABTS radical scavenging activities, and FRAP value of the extracts increased in an ethanol concentration-dependent manner. The antioxidant activities of the chlorophyll-removal extracts were much higher than those of the chlorophyll-containing extracts. Cytotoxicity was not observed in HepG2 cells with extracts up to 1,000 µg/mL. All extracts inhibited ROS production in a concentration-dependent manner from 31.3 µg/mL and inhibited DNA damage at 250 µg/mL. The SOD and catalase activities of cell lines treated with the extracts and H2O2 were similar to those of normal cells, indicating a strong protective effect. CONCLUSION: Lycium barbarum leaf extracts had high antioxidant activities and protected H2O2-stressed HepG2 cells. Since the chlorophyll-removal extract exhibited higher antioxidant activities than the chlorophyll-containing ones and the cytoprotective effect was similar, chlorophyll removal extract of Lycium barbarum leaf could be developed as ingredients of functional food and cosmetics.
Catalase ; Cell Line ; Chlorophyll ; DNA Damage ; DNA Fragmentation ; Ethanol ; Functional Food ; Hep G2 Cells ; Hydrogen Peroxide ; Lycium ; Reactive Oxygen Species

Hemophagocytic syndrome (HPS) is a rare but potentially life-threatening disease in kidney transplant recipients, and is caused by systemic proliferation of macrophages actively phagocytizing other blood cells in the bone marrow, lymph nodes, and the spleen.Here, we report a 40-year-old male kidney transplant recipient who presented with fever, bicytopenia, and elevated liver enzymes 2 months after transplantation. Given that cytomegalovirus antigenemia and real-time polymerase chain reaction tests were positive, liver biopsy was performed under an assumption of cytomegalovirus-induced hepatitis. Hepatic histology revealed multifocal microabscess with cytomegalovirus inclusion bodies, marked Kupffer cell hyperplasia, and erythrophagocytosis by activated macrophages. As laboratory findings such as hyperferritinemia, elevated serum lactate dehydrogenase, low natural killer cell activity, and high soluble interleukin-2 receptor were also compatible with HPS, the recipient was diagnosed as having cytomegalovirus-induced hepatitis combined with reactive HPS. Following intravenous ganciclovir therapy with continuous administration of tacrolimus and corticosteroid, the symptoms resolved and laboratory findings were normalized. As far as we know, this is the first report of cytomegalovirus-induced hepatitis combined with reactive HPS in a kidney transplant recipient that is diagnosed by liver biopsy.

PURPOSE: This study was designed to evaluate the mid-term results and efficacy of subtalar distraction double bone-block arthrodesis for calcaneal malunion. MATERIALS AND METHODS: From January 2004 to June 2007, we operated on 6 patients (10 cases). There were 5 males (9 cases) and 1 female (1 case), four of which presented with bilateral calcaneal malunion. Seven cases were operated on initially. The period between initial injury and arthrodesis was 23 months, and the average follow up period was 58 months. In operation, we applied an extensile lateral approach and arthrodesis was performed through a tricortical double bone-block and cannulated screws. The American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Hindfoot scale was used for clinical evaluation. In radiologic analysis, plain X-ray and CT were examined to assess union and various parameters. RESULTS: The mean age of the patients was 41 years. All cases achieved radiologic union at the final follow-up. The mean AOFAS Ankle-Hindfoot scale (maximum of 94 points) increased from 43.3 points preoperatively to 85.4 points at the final follow-up. The radiologic analysis of the pre- and postoperative standing lateral radiographs showed improvements of 5.6 mm in talo-calcaneal height, 1.8degrees in talocalcaneal angle, 5.1degrees in talar declination angle and 5.3degrees in talo-first metatarsal angle. CONCLUSION: Subtalar distraction two bone-block arthrodesis provides overall good results not only in the short term but also the mid-term with significant improvement in clinical and radiologic outcomes. This procedure warrants consideration for managing calcaneal malunion with loss of height and subtalar arthritis.
Adult ; Arthrodesis/methods ; Calcaneus/*injuries/surgery ; Female ; Fractures, Malunited/*surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome

BACKGROUND: Lifestyle is an important risk factor for many diseases. We need to educate medical students for advocating health promoting behaviors to their patients. Teaching knowledge, however, was not enough for medical students to change their health-related lifestyle. In this study, we investigated health promoting behaviors of medical students, and the improvement of their health behaviors after competitive lifestyle modification program. METHODS: All sophomores of medical college in Seoul National University were included in this study. We performed lifestyle modification program consisting of lecture, group competition, and feedback. We focused on reforming health behaviors like smoking, drinking, exercise, stress management and eating habits. And we also checked common symptoms of functional diseases, medication frequency for relieving these symptoms and general life satisfaction. RESULTS: After 2 months of lifestyle modification program, the obese (P = 0.0455) and the average value of BMI (P < 0.001) were decreased and two of smokers quitted smoking. Numbers of medical students with health related habits such as regular exerciser (P = 0.027), frequent user of symptom-relief medication (P < 0.001), breakfast skipper (P = 0.005) were signifi cantly changed in positive way, and the average value of general life satisfaction score (P = 0.003) was improved. CONCLUSION: Lifestyle modification program improved medical students' health habits and enhanced general life satisfaction.
Breakfast ; Drinking ; Eating ; Health Behavior ; Humans ; Life Style ; Risk Factors ; Smoke ; Smoking ; Students, Medical

PURPOSE: The pertussis vaccines are divided into whole-cell vaccine and acellular vaccine. Purified component acellular pertussis vaccine, originally developed in Japan, has been used for primary immunization series in Korea since 1984. We performed this study to evaluate the prevalence of pertussis antibodies following vaccination with acellular pertussis vaccine. METHODS: We randomized 80 children who were immunized according to vaccine schedules including less than 2 month-old infants at Department of Pediatrics, Catholic University Medical College from February 1995 to October 1995. We measured agglutination titers of children by bacterial agglutination test. RESULTS: 1) The age distribution ranged from 5 days to 7 years old, the mean age was 14.5+/- 12.8 months and male to female ratio was 1:1. 2) Only 23 cases (28.8%) of the 80 cases were positive results. 3) Among 7 infants who were less than 2 months-old and were not immunized, 5 infants (71.4%) were seropositive. 4) There was no significant difference in positive rates among age groups which include 2 to 6 months age group, 7 to 18 months age group, and older than 18 months age group. CONCLUSIONS: The prevalence of pertussis positive rates is only 28.8% and there was no difference among age groups.
Age Distribution ; Agglutination ; Agglutination Tests ; Antibodies* ; Appointments and Schedules ; Child* ; Female ; Humans ; Immunization ; Infant ; Japan ; Korea ; Male ; Pediatrics ; Pertussis Vaccine ; Prevalence ; Vaccination* ; Vaccines ; Whooping Cough*

In this study, we report that an acute phase reactant, serum amyloid A (SAA), strongly inhibits dendritic cell differentiation induced by GM-CSF plus IL-4. SAA markedly decreased the expression of MHCII and CD11c. Moreover, SAA decreased cell surface GM-CSF receptor expression. SAA also decreased the expression of PU.1 and C/EBPα, which play roles in the expression of GM-CSF receptor. This inhibitory response by SAA is partly mediated by the well-known SAA receptors, Toll-like receptor 2 and formyl peptide receptor 2. Taken together, we suggest a novel insight into the inhibitory role of SAA in dendritic cell differentiation.
Dendritic Cells* ; Granulocyte-Macrophage Colony-Stimulating Factor* ; Interleukin-4 ; Receptors, Formyl Peptide ; Receptors, Granulocyte-Macrophage Colony-Stimulating Factor* ; Serum Amyloid A Protein* ; Toll-Like Receptors

BACKGROUND: The postoperative speech outcomes of submucous cleft palate (SMCP) surgery are known to be poorer than those of other types of cleft palate. We attempted to objectively characterize the postoperative complications and speech outcomes of the surgical treatment of SMCP through a comparison with the outcomes of incomplete cleft palate (ICP). METHODS: This study included 53 SMCP patients and 285 ICP patients who underwent surgical repair from 1998 to 2015. The average age of the patients at the time of surgery was 3.9±1.9 years for the SMCP patients and 1.3±0.9 years for the ICP patients. A retrospective analysis was performed of the complications, the frequency of subsequent surgical correction for velopharyngeal dysfunction (VPD), and speech outcomes. RESULTS: In both the SMCP and ICP patients, no cases of respiratory difficulty, bleeding, or wound disruption were noted. Delayed wound healing and fistula occurred in 18.9% and 5.7% of the SMCP patients and in 14% and 3.2% of the ICP patients, respectively. However, no statistically significant difference in either delayed wound healing or fistula occurrence was observed between the two groups. The rate of surgical correction for VPD in the SMCP group was higher than in the ICP group. In the subset of 26 SMCP patients and 62 ICP patients who underwent speech evaluation, the median speech score value was 58.8 in the SMCP group and 66 in the ICP group, which was a statistically significant difference. CONCLUSIONS: SMCP and ICP were found to have similar complication rates, but SMCP had significantly worse speech outcomes.
Cleft Palate* ; Fistula ; Hemorrhage ; Humans ; Postoperative Complications ; Retrospective Studies ; Velopharyngeal Insufficiency ; Wound Healing ; Wounds and Injuries

The HACEK group of bacteria (Haemophilus parainfluenzae, H. aphrophilus, H. paraphrophilus, Actinobacilus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corodens, and Kingella kingae) are the normal flora of the upper respiratory tract and oropharynx. The organisms infect abnormal cardiac valves, causing subacute native endocarditis or prosthetic valve endocarditis more than one year after valve surgery. Haemophilus species are responsible for only 0.5~1% of all infective endocarditis cases. Embolization occurs in 60% and the mortality rate ranges from 16~45% of cases of infective endocarditis caused by H. parainfluenzae. We experienced a case of infective endocarditis due to H. parainfluenzae in a 37-year-old male admitted with high fever, chills, nausea & vomiting, chest discomfort, and blurred vision. The organism was isolated from a blood culture and was identified as H. parainfluenzae by factor V requirement, negativity at urea, positivity at ornithine decarboxylase, and acid production from glucose and maltose. The patient was treated with antibiotics and symptoms and signs were improved
Adult ; Anti-Bacterial Agents ; Bacteria ; Cardiobacterium ; Chills ; Eikenella ; Endocarditis ; Factor V ; Fever ; Glucose ; Haemophilus ; Haemophilus parainfluenzae ; Heart Valves ; Humans ; Kingella ; Male ; Maltose ; Nausea ; Ornithine Decarboxylase ; Oropharynx ; Paramyxoviridae Infections ; Respiratory System ; Thorax ; Urea ; Vision, Ocular ; Vomiting