• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.213-221
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• 2005

Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

• Journal of agricultural medicine and community health
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• v.25 no.2
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• pp.265-273
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• 2000

Benign prostatic hyperplasia (BPH) is a highly prevalent, age-related disorder in men which place a considerable burden on health care resources worldwide. While BPH and hypertension are apparently diverse disease processes, they have some features in common(e.g. underlying etiology of the sympathetic nervous system). The purpose of this study is to estimate the prevalence of lower urinary tract symptoms using I-PSS(international prostate symptom score) and to investigate the association of hypertension with I-PSS. This study was carried out to 390 men(40~86 year old) at 3 Myun in Chungju City from July to August, 1997. Subjects answered on questionnaire for I-PSS and were checked age, education, marital status, annual income, blood pressure. The mean age of subjects was 59.8 year old. The mean of I-PSS were increased by age decades(40~49, 50~59, 60~69, 70~86) and it's scores were 4.4, 7.0, 8.2, 12.3 respectively. Strong correlations were observed between the I-PSS and the QUL(quality of life)(P=0.0001). I-PSS(mean-value) were 7.85 in hypertensive group and 8.39 in normotensive group but there was no statistically significance between the two groups(P>0.05). The proportion of mean scoring greater than 8 was 42.5%. There have been reports of association between lower urinary tract symptoms and hypertension, but there was no consistent suggestion that such an association could be casual. The need for high-quality epidemiological information and consequent increased prospects for prevention is obvious.

• Radiation Oncology Journal
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• v.17 no.2
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• pp.141-145
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• 1999

Purpose : Granisetron is a potent, the most selective 5-HT3 receptor antagonist and is reported to b effective in treatment of radiation-induced emesis. The antiemetic efficacy and safety of oral granisteron was evaluated in patients with receiving highly emetogenic treatment by conventional fractionated irradiation. Materials and Methods : Patients with various cancers who were being treated with irradiation were accrued into the present study. The intensity of nausea was evaluated on first 24 hours and on day-7 by patients according to the degree of interference with normal daily life as followings; a) none; b) present but no interference with normal daily life (mild): c) interference with normal daily life (moderate): and d) bedridden because of nausea (severe). Non or mild state was considered to indicate successful treatment. The efficacy of antiemetic treatment was graded as follows; a) complete response; no vomiting, no worse than mild nausea and receive no rescue antiemetic therapy over the 24h period, b) major response; either one episode of vomiting or moderate/severe nausea or had received rescue medication over 24h period, or any combination of these, c) minor response; two to four episodes of vomiting over the 24h period, regardless of nausea and rescue medication, d) failure; more than four medication. The score of the most symptom was recorded and the total score over 24 hours was summarized. The complete or major response was considered to indicate successful treatment. Results : A total of 10 patients were enrolled into this study, and all were assessable for efficacy analysis. Total nausea control was achieved in 90$\%$ (9/10:none=60$\%$ plus mild=30$\%$) of total patients after 7 days. The control of vomiting by granisteron was noted in seven patients (70$\%$) of complete response and three (30$\%$) of major response with a hundred-percent successful treatment over 7 days. The minor response or treatment failure were not observed. No significant adverse events or toxicities from granisetron were recorded in patient receiving granisetron. Conclusion : We concluded that granisetron is a highly effective antiemetic agent in controlling radiotherapy-induced nausea or vomiting with a minimal toxicity profile.

• Pediatric Infection and Vaccine
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• v.21 no.1
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• pp.22-28
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• 2014

Purpose: This study aimed at determining the detection rate of respiratory viruses and at investigating the risk factors associated with respiratory virus detection in young infants. Methods: From September 2011 to August 2012, nasopharyngeal swabs were obtained from 227 infants aged ${\leq}90$ days with suspected infectious diseases, including sepsis. We performed a retrospective analysis of their clinical characteristics. The prevalence of respiratory viruses in their nasopharyngeal swabs was assayed by real-time polymerase chain reaction (real-time PCR). Results: In total, 157 (69.2%) infants had more than one of the following respiratory viruses: respiratory syncytial virus (n=75), rhinovirus (n=42), influenza virus (n=18), parainfluenza virus (n=15), human metapneumovirus (n=9), coronavirus (n=9), adenovirus (n=4), and bocavirus (n=3). During the same period, bacterial infections were confirmed in 24 infants (10.6%). The detection of respiratory viruses was significantly associated with the presence of cough, a family history of respiratory illness, and a seasonal preference (fall/winter). Using logistic regression analysis, these 3 variables were also identified as significant risk factors. During fall and winter, detection of respiratory viruses was significantly higher in infants who did not have a bacterial infection. Conclusion: Respiratory virus is an important pathogen in young infants admitted to a hospital, who are suspected with infectious diseases. Detection of respiratory viruses in young infants was associated with seasonality (fall/winter), presence of respiratory symptoms and a family history of respiratory illness.

• Pediatric Infection and Vaccine
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• v.29 no.2
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• pp.70-76
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• 2022

Coronavirus disease 2019 (COVID-19) in patients with underlying diseases, is associated with high infection and mortality rates, which may result in acute respiratory distress syndrome and death. Mucopolysaccharidosis (MPS) type II is a progressive metabolic disorder that stems from cellular accumulation of the glycosaminoglycans, heparan, and dermatan sulfate. Upper and lower airway obstruction and restrictive pulmonary diseases are common complaints of patients with MPS, and respiratory infections of bacterial or viral origin could result in fatal outcomes. We report a case of COVID-19 in a 16-year-old adolescent with MPS type II, who had been treated with idursulfase since 5 years of age. Prior to infection, the patient's clinical history included developmental delays, abdominal distension, snoring, and facial dysmorphism. His primary complaints at the time of admission included rhinorrhea, cough, and sputum without fever or increased oxygen demand. His heart rate, respiratory rate, and oxygen saturation were within the normal biological reference intervals, and chest radiography revealed no signs of pneumonia. Consequently, supportive therapy and quarantine were recommended. The patient experienced an uneventful course of COVID-19 despite underlying MPS type II, which may be the result of an unfavorable host cell environment and changes in expression patterns of proteins involved in interactions with viral proteins. Moreover, elevated serum heparan sulfate in patients with MPS may compete with cell surface heparan sulfate, which is essential for successful interaction between the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein and the host cell surface, thereby protecting against intracellular penetration by SARS-CoV-2.

• Anxiety and mood
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• v.7 no.1
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• pp.48-53
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• 2011

Objective : Korean Version of Beck-II Depression Inventory to verify the reliability and validity of the proposed standards are practical and standardized, cut-off score by establishing a baseline indicating the presence of depression and depression On in the evaluation was to evaluate the clinical usefulness. Methods : 739 patients with major depression using the SCID and normal controls were 302 study subjects. Of patients with clinically significant medical condition, or psychotic disorders, organic mental disorder, epilepsy or seizure disorder, eating disorders are associated with patients taking anti-convulsants experienced in the past, patients were excluded from the study. Results : The main findings of this study were as follows. First, with respect to the KBDI-II items, the correlation between them ranged from 0.51 to 0.74, and was 0.60 over all questions. Further, the overall correlation of the KBDI-II plates showing confidence 'normal' than it was verified that. Second, the BDIII was used in each group to examine internal consistency and thus, whether Cronbach's alpha values were greater than 0.94. Third, the principal component analysis sought to extract factors in a way consistent with the results inspected last 3 factors were extracted and the total variance explained was 47.3%. Fourth, the Cutting calculated the score on the KBDI-II for ROC (Receiver operator characteristic) analysis yielding 18 dot, with the highest sensitivity and specificity was seen. Conclusion : Based on the results of this Study, the KBDI-II cut-off point should be valid as prescribed in 18 is considered.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.19 no.1
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• pp.20-25
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• 2019

Very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (OMIM#201475) is an autosomal recessively inherited metabolic disorder of mitochondrial long-chain fatty acid oxidation. The clinical features of VLCAD deficiency is classified by three clinical forms according to the severity. Here, we report a case of later-onset episodic myopathic form of VLCAD deficiency whose diagnosis was confirmed by plasma acylcarnitine analysis and" multigene panel multigene panel sequencing. A 34-year old female patient visited genetics clinic for genetic evaluation for history of recurrent myopathy with intermittent rhabdomyolysis. She suffered first episode of rhabdomyolysis with acute renal failure requiring hemodialysis at twelve years old. After then, she suffered several times of recurrent rhabdomyolysis provoked by prolonged exercise or fasting. Physical and neurologic exam was normal. Serum AST/ALT and creatinine kinase (CK) levels were mildly elevated. However, according to her previous medical records, her AST/ALT, CK were highly elevated when she had rhabdomyolysis. In suspicion of fatty acid oxidation disorder, multigene panel sequencing and plasma acylcarnitine analysis were performed in non-fasting, asymptomatic condition for the differential diagnosis. Plasma acylcarnitine analysis revealed elevated levels of C14:1 ($1.453{\mu}mol/L$; reference, 0.044-0.285), and C14:2 ($0.323{\mu}mol/L$; 0.032-0.301) and upper normal level of C14 ($0.841{\mu}mol/L$; 0.065 -0.920). Two heterozygous mutation in ACADVL were detected by multigene panel sequencing and confirmed by Sanger sequencing: c.[1202G>A(;) 1349G>A] (p.[(Ser 401Asn)(;)(Arg450His)]). Diagnosis of VLCAD deficiency was confirmed and frequent meal with low-fat diet was educated for preventing acute metabolic derangement. Fatty acid oxidation disorders have diagnostic challenges due to their intermittent clinical and laboratorial presentations, especially in milder late-onset forms. We suggest that multigene panel sequencing could be a useful diagnostic tool for the genetically and clinically heterogeneous fatty acid oxidation disorders.

• Tuberculosis and Respiratory Diseases
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• v.60 no.5
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• pp.548-553
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• 2006

Background : Alcoholic ketoacidosis(AKA) is a metabolic disturbance that is caused by prolonged and excessive alcohol consumption. Though the prognosis is reportedly good, its outcome is unclear in some cases that are combined with multi-organ failure. There are few reports of an analysis of cases admitted to an intensive care unit(ICU) Method: Cases of AKA admitted to the ICU over the last 5 years were retrospectively analyzed. Severe AKA was characterized by multi-organ failure that required treatment in an ICU Results : All patients were males with a history of excessive alcohol consumption. Five of them (50%) mainly complained of gastrointestinal symptoms (nausea, vomiting, diarrhea), showing metabolic acidosis with an increased asmolar and anion gap. Rhabdomyolysis with acute renal failure was the most common combined organ failure. Mechanical ventilation was performed in 80%. Six patients died and 4 patients survived. In the surviving patients, the arterial blood gas analysis(ABGA) was normalized within 12 hours after admission. Conclusion : In severe AKA patients, rhabdomyolysis with acute renal failure was the most common complication. The mortality rate was high and death from shock occurred within 3 days.

• Tuberculosis and Respiratory Diseases
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• v.60 no.4
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• pp.397-403
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• 2006

Background: Chronic obstructive pulmonary disease(COPD) is categorized by the percentage of the predicted $FEV_1$(Forced expiratory volume in 1 second) result which is highly correlated with disease severity(morbidity and mortality). In COPD patients, dyspnea seems to be different from disease severity. We investigated whether dyspnea is correlated with disease severity, as measured by $FEV_1$, quality of life(QoL), occupation, and supporting level of family members and neighbors. Method: Thirty-six clinically stable patients with chronically irreversible airflow limitation were enrolled. We used the Medical Research Council(MRC) dyspnea scale to assess the level of dyspnea and the Korean St. Goerge's respiratory questionnaire(SGRQ) as measure the QoL. Result: The mean percentage of the predicted $FEV_1$ was 32.0%. Dyspnea was not correlated with GOLD stage using $FEV_1$(p=0.114). With deteriorating level of dyspnea the scores of symptoms(p=0.041), activity(p=0.004), impact(p=0.001), and total SGRQ score(p<0.001) were significantly increased. Dyspnea was not correlated with the level of occupation(p=0.259). The supporting level of family members and neighbors was significantly negatively correlated with dyspnea scale(p=0.011). Conclusion: In the management of COPD patients, we have to remember that the level of subjective dyspnea is correlated with QoL(symptom, activity and impact on society) and social supporting level as well as GOLD stage($FEV_1$).

• Sleep Medicine and Psychophysiology
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• v.21 no.1
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• pp.5-13
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• 2014

Objectives: Depression, sleep complaints and cognitive impairments are commonly observed in the elderly. Elderly subjects with depressive symptoms have been found to show both poor cognitive performances and sleep disturbances. However, the relationship between sleep complaints and cognitive dysfunction in elderly depression is not clear. The aim of this study is to identify the association between sleep disturbances and cognitive decline in late-life depression. Methods: A total of 282 elderly people who underwent nocturnal polysomnography in a sleep laboratory were enrolled in the study. The Korean version of the Neuropsychological Assessment Battery developed by the Consortium to Establish a Registry for Alzheimer's Disease (CERAD-K) was applied to evaluate cognitive function. Depressive symptoms were assessed with the geriatric depression scale (GDS) and subjective sleep quality was measured using the Pittsburg sleep quality index (PSQI). Results: The control group ($GDS{\leq}9$) when compared with mild ($10{\leq}GDS{\leq}16$) and severe ($17{\leq}GDS$) depression groups, had significantly different scores in the Trail making test part B (TMT-B), Benton visual retention test part A (BVRT-A), and Stroop color and word test (SCWT)(all tests p<0.05). The PSQI score, REM sleep duration, apnea-hypopnea index and oxygen desaturation index were significantly different across the three groups (all indices, p<0.05). A stepwise multiple regression model showed that educational level, age and GDS score were predictive for both TMT-B time (adjusted $R^2$=35.6%, p<0.001) and BVRT-A score (adjusted $R^2$=28.3%, p<0.001). SCWT score was predicted by educational level, age, apnea-hypopnea index (AHI) and GDS score (adjusted $R^2$=20.6%, p<0.001). Poor sleep quality and sleep structure alterations observed in depression did not have any significant effects on cognitive deterioration. Conclusion: Older adults with depressive symptoms showed mild sleep alterations and poor cognitive performances. However, we found no association between sleep disturbances (except sleep apnea) and cognitive difficulties in elderly subjects with depressive symptoms. It is possible that the impact of sleep disruptions on cognitive abilities was hindered by the confounding effect of age, education and depressive symptoms.