A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

Objective: If empirical antibiotics must be administered, the pharmacy needs to deliver the drug to emergency patients as soon as possible. The purpose of this study was to investigate the impact of the installation of an automated dispensing cabinet system in an emergency center. Methods: A retrospective study was conducted through the analysis of medical records in an emergency medical center for 12 months before and after the installation of the system. The order-to-antibiotic time and door-to-antibiotic time were collected retrospectively through a system log analysis. For the satisfaction survey, nursing providers who use the dispensing system were administered a survey questionnaire related to the system. Results: This study covered 1,292 prescriptions before and 1,377 after the introduction of the system in the emergency center. It was observed that the average time from the order-to-drug administration decreased (48.7±57.9 vs. 41.1±52.3, P=0.001) after the introduction of the system. The door-to-drug administration time also showed a decrease (293.6±260.2 vs. 267.4±221.1, P=0.006). A satisfaction survey was conducted on 38 users of the system who agreed to participate and respond. The score of the satisfaction survey was 3.63±0.44. The user response showed that they were most satisfied with the prevention of medication errors (4.00±0.62) and system control (4.00±0.57). Conclusion The automated dispensing cabinet system in an emergency center can shorten the order-to-drug time and door-to-drug time. In addition, it helps to improve the satisfaction and work efficiency of emergency medical workers related to medication dispensation.

Non-tuberculous mycobacterial (NTM) disease is a relatively rare cause of neutropenic fever in patients with hematologic malignancies. During the neutropenic period, performing invasive procedures for microbiological or pathological confirmation is difficult. In addition, the optimal treatment duration for NTM disease in patients with leukemia, especially prior to stem cell transplantation (SCT), has not been documented. Therefore, we report a case of pneumonia with necrotizing lymphadenitis caused by Mycobacterium kansasii diagnosed during chemotherapy being performed for acute myeloid leukemia. The radiologic findings were similar to those of invasive fungal pneumonia; however, a bronchoalveolar washing fluid culture confirmed that the pathogen was M. kansasii. After 70 days from starting NTM treatment, allogeneic SCT was performed without any complications. The patient fully recovered after 12 months of NTM treatment, and neither reactivation of M. kansasii infection nor related complications were reported.
Drug Therapy ; Fever ; Hematologic Neoplasms ; Humans ; Leukemia ; Leukemia, Myeloid, Acute* ; Lymphadenitis* ; Mycobacterium kansasii* ; Mycobacterium* ; Pneumonia* ; Stem Cell Transplantation* ; Stem Cells*

PURPOSE:To investigate whether airway eosinophilic degranulation develops in Mycoplasma pneumonia (M. pneumonia), and to elucidate the association between M. pneumonia and asthma. METHODS:Forty patients with M. pneumonia, 20 stable asthma patients (stable asthma) and 20 normal controls were recruited from October 2005 to February 2007. In the M. pneumonia, blood and induced sputum sampling were collected at admission (acute stage) and 6 to 8 weeks later (convalescent stage). Eosinophil-derived neurotoxin (EDN) and eosinophil cationic protein (ECP) levels in sputum and serum were measured in all 3 groups. RESULTS:Serum levels of EDN and ECP in the acute stage of M. pneumonia were comparable to those in the stable asthma group. However, in the convalescent stage of M. pneumonia, EDN and ECP levels were significantly lower than in the stable asthma (P<0.01 and P<0.05, respectively). Sputum levels of EDN and ECP levels in the acute stage of M. pneumonia were comparable to those in the stable asthma. Sputum EDN levels in the convalescent stage of M. pneumonia were significantly lower than those in the stable asthma (P<0.05), and sputum ECP levels were lower than those in the stable asthma, which was not statistically significant. CONCLUSION:Eosinophilic degranulation may play an important role in the pathogenesis of M. pneumonia, which suggests the association between M. pneumonia and asthma.
Asthma ; Eosinophil Cationic Protein ; Eosinophil-Derived Neurotoxin ; Eosinophils ; Humans ; Inflammation ; Mycoplasma ; Pneumonia ; Pneumonia, Mycoplasma ; Sputum

BACKGROUND: The Korean HHBES, conducted in July 1995, was a nationwide population-based survey of the health and health-seeking behavior of Koreans. Prevalence of significant depressive symptomatology was measured by the Korean version of CES-D. METHOD: The Korean HHBES used a complex, multistage, stratified, clustered sampling scheme as a national probability sample. Using 1990 National Census data, 110 Primary Sampling Units were selected out of 185,149 sampling units in whole country after stratification by region. Of the 1,258 elderly aged 55 to 69 years who were sampled, 628 persons completed the CES-D. The response rate was 50%. RESULT: The caseness rates of cutoff score 16 (probable depression) and cutoff score 25 (definite depression), were 25.28 (male 19.10, female 29.32) and 10.81 (male 7.20, female 13.42). And mean CES-D score was 11.03. Women had significantly higher CES-D caseness rates than men (odds ration (O.R.)=1.227,95% confidence interval (C.I.) 0.793-1.901). Respondents without education also had higher rates than did respondents experienced any educational level (O.R.=1.442,95% C.I. 0.941-2.21). Respondents in disrupted marital status also had higher rates than did respondents with spouses (O.R.=1.362, 95% C.I. 0.885-2.168). Current employment seemed not to be a risk of depressive symptomatology in the elderly (O.R.=1.01,95% C.I. 0.624-1.636). Persons earning less than one million won a month had rates more than 6 times as high as any of the other income categories (O.R.=6.138, 95% C.I. 1.545-27.668). There were no clear trends in the area and age. CONCLUSION: Poverty was the only statistically significant risk factor of depression in Korean elderlys. One possible hypothesis is that poor health, physical disability, and social isolation are the major factors responsible for the observed inverse relationship between income and symptoms of depression in economically disadvantaged older populations. Much attention has to be paid to poor elderlys from the standpoint of mental health for the elderly.
Aged* ; Censuses ; Surveys and Questionnaires ; Depression ; Education ; Employment ; Female ; Humans ; Korea ; Male ; Marital Status ; Mental Health ; Odds Ratio ; Poverty ; Prevalence* ; Risk Factors* ; Sampling Studies ; Social Isolation ; Spouses ; Vulnerable Populations

MELAS is the condition associated with mutant mtDNA that most closely mimics thrombotic cerebrovascular disease. It is characterized by mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes. These children develop short stature and either a focal or generalized seizure disorder. Ultimately, the patient presents with an acute hemiparesis that can alternate from side to side. In this article, we report the MELAS in siblings having point mutation in the mitochondrial DNA with an A to G transition at the 3,243rd position. MELAS is recognized as one of the several distinct syndromes containing cerebral infarct. And, mitochondrial DNA analyses, serum lactate level, and muscle biopsy are diagnostic clue of this syndrome.
Acidosis, Lactic ; Biopsy ; Child ; DNA, Mitochondrial ; Epilepsy, Generalized ; Humans ; Lactic Acid ; MELAS Syndrome* ; Mitochondrial Encephalomyopathies ; Paresis ; Point Mutation ; Siblings*

PURPOSE: Zinc ion is critical for the functional and structural integrity of eukaryotic cells and participate in the regulation of apoptosis. In general, zinc inhibits a nuclear endonuclease, thereby causing inhibition of apoptosis. Recent studies have pointed to a role for a family of caspase proteases that act upstream of endonuclease. The widely used chemotherapeutic agents exert effects by inducing of apoptosis in sensitive tumor cells. In this study, we investigated the effects of zinc ion and other divalent cation on the idarubicin (IDA)-induced apoptosis of HL-60 cells. In addition, to determine whether Zn inhibits an event upstream of endonuclease activation, we analysed the activity of caspase-3, 9 and proteolytic cleavage of procaspase-3 and PARP [poly (ADP-ribose) polymerase]. METHODS: HL-60 cells were cultured in RPMI 1640 and treated with various doses and time periods of IDA with or without pretreatment of ZnCl2, CaCl2 and MgCl2. Cell viability was measured by trypan blue staining. For detection of apoptotic death, cells were stained with Hoechst dye and observed under fluorescence microscopy. The activities of caspase-3 and caspase-9 were measured by the proteolytic cleavages of Ac- DEVD-AMC and Ac-LEHD-AFC as flurogenic substrates, respectively. The proteolytic cleavages of procaspase-3 and PARP were analyzed by Western blotting using anti- caspase-3 and anti-PARP antibody, respectively. RESULTS: IDA induced the apoptotic death of HL-60 cells in a dose and time dependent manner, which was characterized by increasing chromatin condensation and DNA fragmentation. Pretreatment of HL-60 cells with ZnCl2 caused potent inhibition of IDA-induced apoptosis. Consistent with apoptotic death of HL-60 cells, IDA induced the catalytic activation of caspase-3 and caspase-9. After pretreatment of ZnCl2, the activation of caspase- 3 and the proteolysis of PARP induced by IDA were potently inhibited. But, after pretreatment of CaCl2 and MgCl2, there were no significant changes of IDA-induced apoptosis and proteases activity. CONCLUSION: Zinc ion suppressed the IDA-induced apoptosis via the inhibitions of caspase-9 and caspase-3. But calcium and magnesium ions didn't affect the IDA-induced apoptosis.
Apoptosis* ; Blotting, Western ; Calcium ; Caspase 3 ; Caspase 9 ; Cell Survival ; Chromatin ; DNA Fragmentation ; Eukaryotic Cells ; HL-60 Cells ; Humans ; Idarubicin ; Ions ; Magnesium ; Magnesium Chloride ; Microscopy, Fluorescence ; Peptide Hydrolases ; Proteolysis ; Trypan Blue ; Zinc

Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case of the Sturge-Weber syndrome in combination with the Klippel-Trennaunay syndrome and phakomatosis pigmentovascularis in a 4-month-old infant. He showed nevus flameus on the right leg and both part of the face and back, leptomeningeal angiomatosis on right hemisphere, hypertrophy of the right leg, hemiconvulsion on the left and also evidences of congenital glaucoma and nevus of Ota. Very rare case combined with these three kinds of phakomatosis has been reported.
Humans ; Infant ; Klippel-Trenaunay-Weber Syndrome/*complications/diagnosis ; Male ; Neurocutaneous Syndromes/*complications/diagnosis ; Research Support, Non-U.S. Gov't ; Sturge-Weber Syndrome/*complications/diagnosis