Although nitric oxide (NO) plays an important role in the pathophysiological process of cerebral ischemia or severe traumatic brain injury, its contribution to the pathogenesis of moderate diffuse axonal injury (mDAI) remains to be clarified. The alterations in nitric oxide synthase (NOS) activity and the histopathological response after mDAI was investigated. Forty anesthetized Sprague-Dawley adult rats were injured with a Marmarou's weight-drop device through a Plexiglas guide tube. These rats were divided into 8 groups (control, 1 hr, 2 hr, 3 hr, 6 hr, 12 hr, 24 hr, 48 hr after trauma). The temporal pattern of apoptosis in the adult rat brain after mDAI was characterized using TUNEL histochemistry. In addition, the cDNA for NOS activity was amplified using RT-PCR. The PCR products were electrophoresed on a 2% agarose gel. eNOS activity was not detected, but nNOS activity was expressed after 3 hr and continuously 48 hr after impact, which was approximately double that of the control group at 12 and 24 hr. Subsequently, there was a decrease in activity after 48 hr. The iNOS activity increased dramatically after 12 hr and was constant for a further 12 hr followed by a dramatic decrease below the level of the control group. Significant apoptotic changes occurred 12 and 24 hr. after insult. nNOS and iNOS activity were affected after moderate diffuse axonal injury in a time-dependent manner and there was a close relation between the apoptotic changes and NOS activity. Although the nNOS activity was expressed early, its activity was not stronger th an iNOS, which was expressed later.
Animal ; *Apoptosis ; Craniocerebral Trauma/enzymology/*physiopathology ; Diffuse Axonal Injury/enzymology/*physiopathology ; Nitric-Oxide Synthase/*metabolism ; Rats ; Rats, Sprague-Dawley ; Wounds, Nonpenetrating/enzymology/*physiopathology

Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Severity is defined as mild (> 5% of factor activity), moderate (1%-5%), and severe (< 1%). The median age at diagnosis, six males and four females, was 26 years (range, 1 month-73 years). Six of 10 patients were classified as moderate, three as mild, and one as severe disease. Eight patients were diagnosed as inherited FV deficiency. The most frequent symptoms were mucosal tract bleedings (40%) such as epistaxis, and menorrhagia in female. Hemarthroses and postoperative bleeding occurred in one and four patients, respectively. Life-threatening bleeding episodes occurred in the peritoneal cavity (n = 2), central nerve system (n = 1), and retroperitoneal space (n = 1). No lethal haemorrhages happened to patients with mild disease. The majority of bleeding episodes were controlled with local measures and fresh-frozen plasma replacement. Two acquired FV deficient-patients showing life-threatening haemorrhages received the immunosuppressive therapy, but one of them died from postoperative bleeding complications. Despite the small sample size of this study due to rarity of the disease, we found that Korean patients with FV deficiency had similar clinical manifestations and treatment outcomes shown in previous studies.
Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; Blood Transfusion ; Child ; Databases, Factual ; Factor V Deficiency/drug therapy/*pathology ; Female ; Hemorrhage/etiology ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Infant ; Male ; Middle Aged ; Plasma ; Republic of Korea ; Retrospective Studies ; Severity of Illness Index ; Treatment Outcome ; Young Adult

Due to rarity of factor V (FV) deficiency, there have been only a few case reports in Korea. We retrospectively analysed the clinical-laboratory features of FV deficiency in 10 Korean patients. Between January 1987 and December 2013, 10 case reports published in a Korean journal or proceedings of Korea Society on Thrombosis and Hemostasis were reviewed. Severity is defined as mild (> 5% of factor activity), moderate (1%-5%), and severe (< 1%). The median age at diagnosis, six males and four females, was 26 years (range, 1 month-73 years). Six of 10 patients were classified as moderate, three as mild, and one as severe disease. Eight patients were diagnosed as inherited FV deficiency. The most frequent symptoms were mucosal tract bleedings (40%) such as epistaxis, and menorrhagia in female. Hemarthroses and postoperative bleeding occurred in one and four patients, respectively. Life-threatening bleeding episodes occurred in the peritoneal cavity (n = 2), central nerve system (n = 1), and retroperitoneal space (n = 1). No lethal haemorrhages happened to patients with mild disease. The majority of bleeding episodes were controlled with local measures and fresh-frozen plasma replacement. Two acquired FV deficient-patients showing life-threatening haemorrhages received the immunosuppressive therapy, but one of them died from postoperative bleeding complications. Despite the small sample size of this study due to rarity of the disease, we found that Korean patients with FV deficiency had similar clinical manifestations and treatment outcomes shown in previous studies.
Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; Blood Transfusion ; Child ; Databases, Factual ; Factor V Deficiency/drug therapy/*pathology ; Female ; Hemorrhage/etiology ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Infant ; Male ; Middle Aged ; Plasma ; Republic of Korea ; Retrospective Studies ; Severity of Illness Index ; Treatment Outcome ; Young Adult

BACKGROUND: This study evaluated the effects of family type and sociodemographic factors on medical treatment, health behavior, and subjective health status in Koreans 60 years and older. METHODS: This study was based on data from the 5th Korea National Health and Nutrition Examination Survey (KNHANES), a nationwide health interview survey using a rolling sampling design involving complex, stratified, multistage, probability cluster. Our subjects included those > or =60 years of age (n=1,946) participating in the KNHANES 2010. Family type was categorized into 7 groups according to cohabitation: alone, couple cohabitation (living with a spouse), parent(s) cohabitation (couple living with parent(s)), offspring cohabitation (no spouse, living with offspring), couple offspring cohabitation (couple living with offspring), couple offspring parents cohabitation (couple living with parent(s) and offspring), and others. Logistic regression analyses were used to examine the association of family type and sociodemographic factors with health care. RESULTS: The most common family type was couple cohabitation (38.26+/-1.8%). In this group, the percentage of non-receipt of needed medical care (odds ratio [OR], 0.60; 95% confidence interval [CI], 0.42 to 0.87) and current smoking rate (OR, 0.32; 95% CI, 0.20 to 0.50) were significantly lower than those living alone. Among the sociodemographic charicteristics, male gender, apartment dwelling, high middle household income, 2 member family, education higher than middle school, and possessing own house were associated with good health care behavior. CONCLUSION: This study shows that couples have better health care behavior than other family types, in particular, limited smoking, appropriate medical treatment, and positive subjective health status. Those living alone visit medical clinics less often and have a negative subjective health status.
Delivery of Health Care ; Family Characteristics ; Health Behavior ; Humans ; Korea ; Logistic Models ; Male ; Nutrition Surveys ; Parents ; Smoke ; Smoking ; Spouses

Primary fish-odor syndrome (FOS) is a genetic disorder caused by defective flavin-containing monooxygenase 3 gene (FMO3) with deficient N-oxidation of trimethylamine (TMA), causing trimethylaminuria (TMAU). By contrast, secondary FOS can be acquired by decreased FMO activities in patients with chronic liver diseases, but the underlying mechanisms are unknown. In the present study, we examined plasma NOx concentrations and viral DNA contents as well as in vivo FMO activities and their correlations in chronic viral hepatitis (CVH) patients. Plasma concentration of NOx was significantly increased by 2.1 fold (56.2+/-26.5 vs. 26.6+/-5.4micrometer, p< 0.01), and it was positively correlated with plasma hepatitis B virus (HBV) DNA contents (r2=0.2838, p=0.0107). Furthermore, the elevated plasma NOx values were inversely and significantly correlated with in vivo FMO activities detected by ranitidine-challenged test (8.3% vs. 20.0%, r2=0.2109, p=0.0315). TMA N-oxidation activities determined in CVH patients without challenge test were also significantly low (73.6% vs. 95.7%, p< 0.05). In conclusion, these results suggested that secondary FOS could be acquired by the endogenously elevated NO in patients with CVH.
DNA ; DNA, Viral ; Hepatitis B virus ; Hepatitis B* ; Hepatitis* ; Humans ; Liver Diseases ; Nitric Oxide ; Plasma ; Ranitidine

Hematopoietic stem cell transplantation remains the only curative option for myelodysplastic syndrome (MDS), but the prevalence of the disease in elderly people limits broad application of the procedure, particularly in lower risk group. Azacitidine has been recently approved by the U.S. Food and Drug Administration for MDS regardless of subtype on French-American-British classification. Adverse effects of azacitidine include gastrointestinal, hematological and infusion-related reactions. Azacitidine induced hepatotoxicity has been reported mainly in patients with previous hepatobiliary disease, e.g., extensive tumor infiltration in liver, liver cirrhosis and cholelithiasis. We report here a case of azacitidine-induced hepatitis under no predisposition to hepatobiliary disease.
Aged ; Azacitidine ; Cholelithiasis ; Classification ; Hematopoietic Stem Cell Transplantation ; Hepatitis ; Humans ; Liver ; Liver Cirrhosis ; Myelodysplastic Syndromes* ; Prevalence ; United States Food and Drug Administration

Invasive aspergillosis is a serious infectious complication, which can occur after hematopoietic stem cell transplantation (HSCT). In particular, despite antifungal treatment, invasive aspergillosis involving the central nervous system (CNS) shows very high mortality. In principle, a neurosurgical procedure with an antifungal agent is recommended for treatment of CNS invasive aspergillosis. We encountered a patient suffering from disseminated invasive aspergillosis involving the lung, brain, and soft tissues after allogeneic HSCT for treatment of relapsed acute myeloid leukemia who was cured with voriconazole and stereotactic drainage of the brain abscess.
Aspergillosis ; Brain ; Brain Abscess ; Central Nervous System ; Drainage ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Leukemia, Myeloid, Acute ; Lung ; Neurosurgical Procedures ; Pyrimidines ; Stress, Psychological ; Triazoles

BACKGROUND: BMI alone may not serve as an index of obesity because it does not reflect body composition. The present study aimed to compare arterial stiffness as assessed by the brachial-ankle pulse wave velocity (ba-PWV) among groups defined by body fat percentage (pBF) and BMI. METHODS: This cross-sectional study was based on 1,700 participants (1,044 men and 656 women) who completed a health screening examination at a national hospital between January 2011 and February 2016. Participants were divided into four groups according to BMI and pBF: normal fat and normal weight (NFNW); excessive fat and normal weight (EFNW); normal fat and obese (NFO); and excessive fat and obese (EFO). The ba-PWV and other cardiometabolic factors were compared among the four groups in men and women separately. RESULTS: For both sexes, the NFNW group had a lower metabolic risk compared to that in the other groups (EFNW, NFO, and EFO). After adjusting for multiple variables, the NFO males had a significantly lower ba-PWV compared to those in the other groups, including NFNW males. The NFO group had significantly more skeletal muscle mass and muscle mass compared the other groups (P < 0.05). Among women, the NFNW group had a significantly lower ba-PWV compared the other groups, even after adjusting for multiple variables. CONCLUSION: Lower pBF in obese men may be associated with improved cardiovascular risk.
Adipose Tissue* ; Body Composition ; Cross-Sectional Studies ; Female ; Humans ; Male ; Mass Screening ; Muscle, Skeletal ; Obesity ; Pulse Wave Analysis* ; Sex Characteristics ; Vascular Stiffness*

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from professional antigen presenting cell and primarily found in lymph nodes, with rarer case report about extranodal presentation of IDCS. A 71-yr-old man was admitted with progressively enlarging and painless mass in the right parotid area for 2 months. Computed tomography of the neck and chest revealed enhancing mass in right parotid gland, multiple lymphadenopathies around neck and mediastinum, and an osteolytic metastasis at thoracic spine. Morphological and immunohistochemical analysis of an excisional biopsy specimen from parotid mass were consistent with a diagnosis of IDCS. Palliative chemotherapy with 6 cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) regimen and 2 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) regimen plus radiotherapy on parotid mass failed in tumor reduction. We describe a rare case of disseminated extranodal IDCS arising from parotid gland.
Biopsy ; Bleomycin ; Dendritic Cell Sarcoma, Interdigitating* ; Dendritic Cells* ; Diagnosis ; Doxorubicin ; Drug Therapy ; Lymph Nodes ; Mediastinum ; Neck ; Neoplasm Metastasis ; Parotid Gland* ; Radiotherapy ; Spine ; Thorax ; Vinblastine ; Vincristine

PURPOSE: Nitric oxide synthesized by neuronal nitric oxide synthase (nNOS) has been described as a mediator of smooth muscle relaxation in the mammalian gastrointestinal tract. Impaired expression of the nNOS gene is suggested in the development of infantile hypertrophic pyloric stenosis (IHPS). We examined the expression of nNOS mRNA in pyloric muscle biopsy specimens obtained from 8 patients with IHPS and attempted to correlate the results with the clinical characteristics. METHODS: The expression of nNOS mRNA in pyloric muscle biopsy specimens for 8 patients with IHPS was examined using a reverse transcription- polymerase chain reaction (RT-PCR) technique. For the control, a smooth muscle layer specimen of a neonate with a normal pylorus was used. RESULTS: In the control specimen, the level of nNOS mRNA expression was 48.4% of beta-actin mRNA. In the two thinnest (each 3 mm) of pyloric muscle thicknesses as determined by ultra-sonography, the expressed nNOS mRNA were 16.7% and 30.3%. The two thickest (each 8.3 mm) expressed as 35.3% and 22.9% nNOS. The two samples from the earliest age of symptomatic onset (1 day, 7 days after birth) expressed as 25.6% and 4.8%. The two from the latest age of onset (each 30 days) expressed as 7.4% and 10.5%. The control specimen revealed a higher level of nNOS mRNA expression than those of the IHPS specimens. There was no significant correlation between the clinical characteristics and the levels of nNOS mRNA in the IHPS specimens. CONCLUSION: Since a low level of nNOS mRNA expression may lead to impaired production of NO, our observations indicate that the hypertrophic pyloric muscle of an IHPS patient may be the result of a reduced expression of the nNOS gene at the mRNA level. In IHPS patients, there was no correlation between the clinical characteristics and the levels of expressed nNOS mRNA.
Actins ; Age of Onset ; Biopsy ; Gastrointestinal Tract ; Humans ; Infant, Newborn ; Muscle, Smooth ; Neurons* ; Nitric Oxide ; Nitric Oxide Synthase Type I* ; Polymerase Chain Reaction ; Pyloric Stenosis, Hypertrophic* ; Pylorus ; Relaxation ; RNA, Messenger*