Journal of The Korean Society of Inherited Metabolic disease
/
v.16
no.2
/
pp.57-61
/
2016
Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.
Son, Moa;Moon, Jun young;Park, Sanggyu;Cho, Moonjae
Journal of Applied Biological Chemistry
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v.59
no.3
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pp.265-271
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2016
Recently, liver damage contributes to big percentage of the morbidity and mortality rates worldwide. Excessive intake of alcohol is one of the major causes of liver injury. When liver injury is repeated and becomes chronic, it leads to development of fibrosis and cirrhosis. In the liver, TGF-${\beta}$ is a profibrogenic cytokine, which participates in various critical events cause liver fibrosis. Seahorse (Hippocampus abdominalis) is a common traditional Chinese medicine and has been widely used for centuries. Seahorse has been known to have a variety of bioactivities, such as anti-oxidant, anti-fatigue, and anti-tumor. Peptide is one of the main compounds of seahorse. In this study, we isolated enzymatic hydrolysate from seahorse H. abdominalis by alcalase hydrolysis and investigated the effect of the hydrolysate on liver injury. In the present in vitro studies, the hydrolysate increases cell viability of Chang cells and protects Huh7 cells from ethanol toxicity. In addition, the hydrolysate inhibits TGF-${\beta}$-induced responses. In vivo studies show that the pretreatment of hydrolysate reduces alcohol-induced increases of serum Glutamic oxaloacetic acid transaminase and Glutamic pyruvate transaminase activities and increases liver weight and body weight. These results suggest that seahorse may have a hepatoprotective effect.
Sohn, Jang Won;Shin, Sung Joon;Kim, Tae Hyung;Yoon, Ho Joo;Shin, Dong Ho;Park, Sung Soo
Tuberculosis and Respiratory Diseases
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v.57
no.5
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pp.439-442
/
2004
Background : Extubation failure and reintubation increase the morbidity and the mortality rate. Several extubation criteria and risk factors for extubation failure have been recommended. However, some patients present with extubation failure even after a planned extubation. The aim of this study was to evaluate the clinical characteristics of patients with extubation failure after a planned extubation. Methods : Thirty one patients who presented with planned extubation were included. Extubation failure was defined as reintubation within 48 hours after extubation. The clinical, respiratory and hemodynamic parameters between extubation success and failure group were compared. Results : Six patients were included in the failure group. The extubation failure rate was 19.4%. The age, periods of intubation and heart rates were significantly different between the extubation success and failure group. In the success and failure group, the mean age were $60.4{\pm}15.65$ vs. $80.3{\pm}7.17$ year, the intubation periods were $7.12{\pm}2.47$ vs. $13.83{\pm}2.4$ day and the heart rates were $94.32{\pm}5.77$ vs. $110.67{\pm}3.78/min$, respectively. Conclusion : Old age and patients intubated for periods will require a will careful assessment before extubation. Extensive cardiac evaluations before extubation will also be needed.
Kim, Dong Hwan;Song, Bong Sup;Lee, Jun Ah;Kim, Dong Ho
Pediatric Infection and Vaccine
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v.19
no.1
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pp.1-11
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2012
Purpose : Although influenza is regarded as one of the major causes of morbidity and mortality in children with cancer, the actual vaccine coverage remains poor. We conducted evaluation of immunogenicity and safety of influenza vaccine in children with cancer. Methods : In this study, 25 children with cancer who received influenza vaccine (SK influenza IX vaccine$^{(R)}$) at the Korea Cancer Center Hospital between October and December 2009 were analyzed. Blood samples of patients were collected twice (at the beginning of this study and at 30th day after vaccination) and their antibody titers were measured using the hemagglutination-inhibition (HI) assay. Immunogenicity of the influenza vaccine was assessed by seroprotection rate on days 0 and 30, seroconversion rate on day 30, and mean fold increase (MFI) of geometric mean titer (GMT) of HI between days 0 and 30. Results : Any of the subjects in our study did not experienced serious adverse events after influenza vaccination. Seroprotection rates were 68% for H1N1, 40% for H3N2, and 36% for B. Seroconversion rates were 12% for H1N1, 16% for H3N2, and 20% for B. MFIs were 0.9 for H1N1, 1.2 for H3N2, and 1.8 for B. Conclusion : In the study, we found a limited protective immune response to influenza vaccine, among subjects with cancer. However, some subjects showed seroconversion, and there were no severe adverse events among all subjects, supporting the recommendation of annual influenza vaccination in children with cancer.
Although traumatic thoracic aortic rupture is potentially a fatal condition requiring surgical attention, the presence of concomitant injury involving other parts of the body may greatly increase the risk of cardio-pulmonary bypass. We report our experience of treating associated injuries prior to the thoracic aortic rupture in these patients. Material and Method: From 1997 to 2003, the medical records of 24 traumatic aortic rupture patients were retrospectively reviewed and checked for the presence of associated injury, surgical method, postoperative course, and complications. Surgical technique comprised thoracotomy with proximal anastomosis under deep hypothermic circulatory arrest followed by side arm perfusion to reestablish cerebral circulation. CSF drainage was performed to prevent lower extremity paraplegia. Result: Major concomitant injuries (n=83) were noted in all of the reviewed patients, Of these, there were 49 thoracic injuries, 18 musculoskeletal injuries, and 13 abdominal injuries, Operations for associated injuries (n=16) were performed in 12 patients on mean 7.6$\pm$12.6 days following the injury. The diagnosis of aortic rupture at the time of injury was detected in only 18 patients. Delayed surgery of the thoracic aorta was performed on average 695$\pm$1350 days after injury and there were no deaths or progression of rupture in any of these patients during the observation period. There were no operative deaths and no major postoperative complications. Conclusion: Treating concomitant major injuries prior to the aortic injury in traumatic aortic rupture may reduce surgical mortality and morbidity.
Kim Su Wan;Kim Jhingook;Shim Young Mog;Kim Kwhanmien;Choi Yong Soo;I Hoseok;Kim Hojoong;Chang Jee Won
Journal of Chest Surgery
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v.38
no.9
s.254
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pp.622-626
/
2005
Background: Non-invasive interventional therapy has been performed for main bronchial obstruction by endobronchial tuberculosis because of the risk of main bronchial reconstruction regardless of the pulmonary function. But, effects of the inteeventional therapy are attacked by arguments. This study was aimed at interpreting the risk and effectiveness of bronchoplasty for benign bronchial stenosis over the last ten years in our hospital by reviewing the results based on clinical progression. Material and Method: We retrospectively reviewed the clinical records and out-patient medical records including 2f consecutive patients who underwent main bronchial reconstruction for obstruction by endobronchial tuberculosis. All of them had past medical history of anti-tuberculosis medication. They were preoperatively evaluated by bronchoscopy and chest computed tomography. Result: There were no incidences of postoperative mortality and signifcant morbidity. There were 2 cases of retained secretions but these problems were resolved by therapeutic bronchoscopy or intubation. All of the patients are still alive without obstructive airway problem. Conclusion: Bronchoplasty should be considered as one of the primary treatment modalities, if it is anatomically feasible.
From August 1992 to July 1996, 63 consecutive patients underwent coronary artery bypass surgery. The mean age of these patient was 57 years(range form 30 to 71years). There were 44 men and 19 women. Preoperative 12 patients had stable angina pectoris and 23 patients were unstable angina pectoris. 8 patients had previous myocardial infarctation history and emergency or urgent myocardial revascularization were performed in 9 cases. In the risk factors of coronary atherosclerosis, 25 patients(40%) were hypercholesterolemia, 38 patients(60%) have smoking history and 19 patients(30%) have hypertension history. In the patterns of disease, 9 patients were single vessel disease, 18 patients were two vessele disease and 33 patients were three vessel disease. We performed total 284 distal anastomosis(mean 3.5 anastomosis per patient) and performed one case of ascending aorta graft interposition, two cases of mitral valve replacement, one case of aortic valve replacement, one case of ventricular septal defect repair and one case of atrial septal defect repair and the mean aortic cross clamp time was 115.3 minutes. The common complications were arrhythmia(7cases), wound infection(5cases), perioperative myocardial infarction(4cases), reoperation for bleeding control(4cases) and stroke(4cases). There were six hospital deaths due to low cardiac output syndrome, ventricular arrhythmia and respiratory failure. In the evaluation of operative risk factors, preoperative intravenous nitroglycerin requirement and prolonged aortic cross clamp tirne(>2hours) were found to be predective factor of morbidity and old age(>65years) was found to be predective factor of mortality.
Background: Hepatocellular carcinoma (HCC) is a dreadful complication of end stage liver disease with high morbidity and mortality. Aim: The aim was to assess the role of serum talin-1 and non-invasive fibrosis in patients with HCC. Materials and Methods: A total of eighty seven subjects were enrolled, with 22 two healthy individuals as a control group (n=22), 22 patients in the cirrhosis group and finally 43 in the group with HCC diagnosed with positive triphasic CT abdomen criteria. Serum talin-1 and noninvasive fibrosis parameters were assessed in all subjects. Results: Compared to the cirrhosis group, patients with HCC had higher serum talin-1 ($32.9{\pm}12.6$ vs. $11.1{\pm}2.79ng/ml$), FIB4 ($9.96{\pm}15.3$ vs. $2.90{\pm}1.87$) and $fibro-{\alpha}$ ($10.9{\pm}18.1$ vs. $1.55{\pm}0.28$) but not fibrosis index scores ($4.47{\pm}0.95$ vs. $4.98{\pm}0.96$; p=0.046). Patients with large focal lesions (${\geq}5cm$) had different ALBI scores ($-1.02{\pm}0.63$ vs. $-1.72{\pm}0.59$; p=0.001) serum talin-1 ($9.72{\pm}13.81$ vs. $28.6{\pm}38.89ng/ml$; p=0.007) and fibrosis index scores ($0.85{\pm}0.99$ vs. $4.20{\pm}4.85$; p=0.026). No statistical differences were noted between patients with and without portal vein thrombosis. For detection of HCC, serum talin-1 had 97.7% sensitivity and 100% specificity with a 17.2 ng/ml cutoff. AFP at a 13.7 ng/ml cutoff had 72.1% sensitivity and 6.3.6% specificity. The cutoff for the $fibro-{\alpha}$ score was 1.61 with 81.4% sensitivity and 77.3% specificity. Serum talin-1 (odds=1.08; C.I=1.016-1.150; p=0.014), fibrosis index score (odds=2.35; C.I=1.055-5.217; p=0.037) and the ALBI score (odds=6.9; C.I=1.924-24.708; p=0.003) were predictors of large focal lesions. Conclusions: Serum talin-1, AST/ALT ratio, $fibro-{\alpha}$ score are useful for the assessment of HCC patients.
Purpose : Acute diarrhea in one of the most important diseases in children with high morbidity and mortality worldwide. Most of acute diarrhea is induced by viruses. Rotavirus and adenovirus are leading causes of severe gastroenteritis among infants and young children worldwide. Studies for adenovirus gastroenteritis in Korea are limited. We studied the prevalence of rotavirus and adenovirus gastroenteritis from March 1998 to June 1999 in Chungju area. Methods : Stool samples were collected from 143 children with acute diarrhea. Specimens were tested for group A rotavirus antigen and for adenovirus type 40 and 41 by using available commercial kits. Results : Among 143 samples, 37% were positive for rotavirus and 16% were positive for adenovirus. Rotavirus was most prevalent from January to March, 1999 and adenovirus was prevalent during September 1999. The greatest number of rotavirus infections occurred under 24 months of age, followed by 2~4 years of age. Adenovirus was most common in 2~24 months of age. Conclusion : Rotavirus was most prevalent in winter and early spring. In our study, rotavirus was prevalent in early spring and adenovirus was in autumn.
Background: Recent advance in video technology, endoscopic equipments, and surgical techniques have expanded the use of thoracoscopy from diagnosis of the pleural diseases to treatment of the various intrathoracic diseases. Video Assisted Thoracoscopic Surgery(VATS) is a pretty new and fascinating thoracic surgical modality, and so we present our early VATS resuls. Methods: Using Video Thoracoscopic techniques in 30 patients for 10 months from July 1992 to April 1993, we had performed a variety of procedures. These incuded (1) bleb resections in 18 patients (19 cases), (2) mediastinal tumor excision in 4, (3) lung biopsies for parenchymal pulmonary disease in 3, (4) pleural biopasies in 3, (5) pleural tumor excision in 1, (6) and pleuropericardial window in 1. Results: There were no mortality associated with the procedures. We had minor 8 complications; prolonged air leak in 3 patients, prolonged serous drainage in 2, recurrence of pneumothorax in 1, Honer's syndrome in 1, and hoarseness in 1 patient. None of the 30 patients had reverted to the conventional full thoracotomies. Mean postoperative hospital stay of non-complicated pneumothoraces was about 5 days, which was a little shorter than conventional thoracotomy group. Conclusion: Though we had somewhat higher postoperative complication rate due to lack of experiences in the begining, we were able to convince that VATS had benifical value for patients; lesser postoperative pain, shorter hospitalization, quicker recovery time, and cosmetically superior scar. The role of VATS can be expanded to the diagnosis and treatment of various thoracic diseases, even to the cardiovascular diseases, with satisfactory outcome and less postoperative morbidity.
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