• Journal of Veterinary Clinics
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• v.23 no.1
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• pp.72-76
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• 2006

A 1-year-old, female English cocker spaniel (ECS) dog was presented with 3-month history of vomiting and retaking of the vomitus, and chronic weight loss. The client had noticed mild abdominal distension 10 days before. The dog was diagnosed as chronic hepatitis with hepatic cirrhosis based on complete blood count (CBC), serum chemistry profiles, radiography, ascites assessment, bile acid evaluation, and liver biopsy through exploratory laparotomy and necropsy. CBC and serum chemistry profiles revealed mild anemia, slightly elevated hepatic enzymes (ALT and AST), increased creatinine kinase (CK), hyperammonemia, and hypoproteinemia with hypoalbuminemia. Ascites was transudate according to analysis of components. Bile acid assessment (fasting; $174.4{\mu}mol/L$ and postprandial; $198.4{\mu}mol/L$) showed strongly suspected hepatic insufficiency. On radiological findings, ascites was evident. Atrophied liver (especially left side lobes) and distended mesenteric vasculatures were observed by exploratory laparotomy. Histopathological examination of marginal lesion of left lateral lobe of liver by biopsy revealed the necrosis of hepatic cells, dilation of sinusoids, infiltration of neutrophils in sinusoids, and vacuolation of hepatic cytoplasm. The patient had been managed with careful low protein diet and specific supportive therapy (ursodeoxycholic acid, prednisolone, vitamine E, and interferon). Vomiting and ascites disappeared with medical management. The dog was monitored periodically by CBC, serum chemistry and radiographic examination. The dog survived more 18 months with medical therapy. After spontaneous death, necropsy and histopathologic examination were performed.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.26-33
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• 2006

Background: Noninvasive positive pressure ventilation(NPPV) has been increasingly used over the past decade in the management of acute or chronic respiratory failure and weaning of mechanical ventilation. We performed this clinical study to evaluate the usefulness of NPPV in patients who developed acute respiratory failure or post-extubation respiratory failure. Methods: We analysed thirty four patients(sixteen males and eighteen females, mean ages 58 years) who applied NPPV(BIPAP S/T, Respironics co., USA) for respiratory failure or weaning difficulty at medical intensive care unit(MICU), emergency room and general ward of a tertiary hospital. We evaluated the underlying causes of respiratory failure, duration of treatment, the degree of adaptation, complication and predictive parameters of successful outcome. Results: The overall success rate of NPPV was seventy-one percent. The duration of NPPV applying time, baseline blood pressure, pulse rate, respiration rate, $PaO_2$, $PaCO_2$, $SaO_2$ were not different between success group and failure group. But, the baseline pH was higher in the success group. Predictors of success were higher baseline pH, patients with underlying disease of COPD, improvement of vital sign and arterial blood gas value after NPPV application. The success rate in patients with post-extubation respiratory failure was eighty percent. There were no serious complication on applying NPPV except minor complications such as facial skin erythema, abdominal distension & dry mouth. Conclusion: NPPV may be effective treatment in patients with acute respiratory failure or post-extubation respiratory failure in selected cases.

• Radiation Oncology Journal
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• v.23 no.2
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• pp.78-84
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• 2005

Purpose: The studied the effect of such variables as the symptom improvement rate, survival and prognostic factors on the treatment results of radiation therapy for Superior Vena Cava Syndrome (SVCS). Materials and Methods: From 1988 to 2003, seventy two patients with SVCS were treated with radiation therapy at the Department of Radiation Oncology, Keimyung University Dongsan Medical Center. The patients' ages ranged from 10 to 83 years old with the median age being 61, and sixty four patients were male. For the causes of the SVCS, 64 patients had lung cancer, four had metastatic lung cancer, two had malignant lymphoma and two had thymoma. The radiotherapy was delivered with 6-MV X-rays and all patients received above 900 cGy up to 6,600 cGy, with the median dose being 4,000 cGy The follow-up periods were from 1 to 180 months with a median of 5.6 months. Results: The main clinical manifestations were dyspnea ($84.7\%$), facial edema ($81.9\%$), arm edema ($22.2\%$), neck vein distension ($25\%$), hoarseness ($12.5\%$) and facial plethora ($5.6\%$). Eighty percent of patients achieved excellent to good symptom improvement and $19.4\%$ experienced minimal improvement. The median survival period was 5.1 months, and overall survival rates were $17.7\%$ at 2 years (2YOS) and $14.8\%$ at five years (5YOS) for all the patients. The median survival period, the two and five year disease free survival rates were 4.3 months, $16.7\%$ and $13.4\%$ for the lung cancer patients, respectively. The total tumor dose was a statistically significant survival factor on the univariate analysis for the patients with lung cancer (2YSR; > 30 Gy, $25.6\%$, $\leq$ 30 Gy $6.7\%$, p<0.01). On the multivariated analysis, a higher total tumor dose (p<0.01) and younger age (p<0.05) were statistically significant factors of survival for the lung cancer patients. Patients with NSCLC showed better survival than did the patients with SCLC, but this was not statistically significant (p > 0.05), Conclusion: Radiation therapy for the patients with SVCS due to malignancy could be an effective treatment. We considered that radiation therapy above 30 Gy of the total tumor dose may improve survival for SVCS due to lung cancer.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.31-38
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• 2006

Purpose: Infantile constipation is one of the most common problems in pediatric gastrointestinal outpatient clinic. We planed to show the clinical feature of infantile constipation and explore the possible relationship between diets and symptoms of constipation. Methods: We analyzed the medical records and telephone questionnaire about infants under 2 years of age with constipation, who visited outpatient clinic of Department of Pediatrics, Ilsan Paik Hospital during the time from March 2002 to February 2005. Data including the symptoms and signs of constipation, diet history, and past-medical history were analyzed. Results: Total 96 infants, 40 male and 56 female, were enrolled in this study. The mean age was 9 months. Twenty-three infants were exclusively breast milk fed (BMF), 20 infants were cow's milk fed (CMF) and 53 infants had a history of mixed feeding with cow's milk and breast milk (MMF). Patients showed painful defecation (95.8%), abdominal distension (53.1%), palpable rectal stool (35.1%), hard stool (30.2%), blood-tinged stool (29.2%) and anal fissure (16.7%). Patients with exclusive cow milk feeding had prominent clinical features, such as anal fissure (p=0.03), hematochezia (p=0.04) and palpable rectal stool (p=0.025). Patients who had a history of larger intake of liquid food had a tendency to get anal skin tag (p<0.05). Conclusion: Exclusive breast milk feeding seemed important to avoid constipation with clinical significance. To educate caregivers in appropriate way of the weaning food may help the infants with constipation.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.27-32
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• 2006

Purpose : Conservative management of multicystic dysplastic kidney(MCDK) without nephrectomy has recently been advocated. The purpose of this study was to determine the clinical course of conservatively managed MCDK and to find out possible predictive factors for involution of MCDK by ultrasonography(US). Methods : A retrospective analysis was made on 45 patients(26 boys and 20 girls) in whom MCDK was detected and had been traced by US between Dec. 1993 and Aug. 2005 at Severance Hospital. Results : Median follow-up time was 30 months(range 2-102 months). All patients under-went radionuclide scans and voiding eystourethrograms. The serial follow-up US showed complete involution in 11(24%), partial involution in 19(41%), and no interval change or increased in cyst size in 13(28%) patients. Nephrectomy was done in 3 patients(7%) due to relapsing urinary tract infection(UTI) and severe abdominal distension. The mean age of complete involution of MCDK was 37 months(range 12-84 months). Episodes of UTI were present in 17 patients(37%) and additional genitourinary(GU) abnormalities were found in 22 patients(44%). Hypertension and renal insufficiency was complicated in one patient. No child developed malignant tumor. Univariate analysis showed that five variables were associated with complete involution of the MCDK; gender, site, UTI episode, additional GU abnormalities, and renal length on initial US. After adjusting using the Pearson model, the presence of additional GU abnormalities was exclusively associated with complete involution among the 5 variables(P=0.034). Conclusion : In our review of 46 cases of MCDK, non-surgical approach for patients with MCDK was advisable and we could predict poor prognosis when MCDK is associated with other GU anomalies.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.25-30
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• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1204-1212
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• 2002

Purpose : Early surfactant therapy with either gentle ventilation, high-frequency ventilation or aggressive weaning of mechanical ventilation are principles for the treatment of respiratory distress syndrome(RDS). We studied to determine the accessibility of noninvasive nasal continuous positive airway pressure(CPAP) rather than mechanical ventilation by invasive intubation after early surfactant therapy. Methods : The study group consisted of 14 infants who were born and diagnosed with moderate respiratory distress syndrome and received early surfactant therapy with nasal CPAP of PEEP 5-6 cm $H_2O$ within two hours after birth in the Fatima neonatal intensive care unit for two years from January 1999 to August 2001. The control group consisted of 15 infants who were diagnosed with the disease and could be weaned from mechanical ventilator within five days after birth during the same period. Results : The characteristics, the severity of clinical symptoms and laboratory findings in the two groups at birth showed no significant difference. Neither did the interim analysis of laboratory data in two groups. Of 14 infants in the study group who received nasal CPAP after early surfactant therapy, only two infants showed weaning failure with this therapy. In the response cases, duration of CPAP was five days and mean airway pressure was $5.4{\pm}0.5cm$ $H_2O$. Two had the complication of CPAP with abdominal distension. Final complications and outcomes in the two groups showed no signifcant difference(P>0.05). Conclusion : The clinical courses in the two groups showed no significant difference. Therefore, we suggest that early surfactant therapy with noninvasive nasal CPAP is a simple and safe method rather than aggressive weaning after invasive mechanical ventilation in moderate respiratory distress syndrome.

• Tuberculosis and Respiratory Diseases
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• v.43 no.6
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• pp.987-996
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• 1996

Background : We prospectively evaluated the applicability and effect of noninvasive ventilation (NIV) in acute respiratory failure and tried to find out the parameters that could predict successful application of NIV. Methods : Twenty-six out of 106 patients with either acute ventilatory failure (VF: $PaCO_2$ > 43 mm Hg with pH < 7.35) or oxygenation failure (OF: $PaO_2/AO_2$ < 300 mm Hg with $pH{\geq}7.35$) requiring mechanical ventilation were managed by NIV (CPAP + pressure suppon, or BiPAP) with face mask. Eleven out of 19 cases with VF (57.9%) (M : F=7 : $55.4{\pm}14.6$ yrs) and 15 out of 87 cases with OF (17.2%) (M : F=12 : 3, $50.6{\pm}15.6$ yrs) were s uilable for NIY. Respiratory rates, arterial blood gases and success rate of NIV were analyzed in each group. Results: 81.8% (9/11) of YF and 40% (6/15) of OF were successfully managed on NIV and were weruled from mechanical ventilator without resorting to endotracheal intubation. Complications were noted in 2 cases (nasal skin necrosis 1, gaseous gastric distension 1). In NIV for ventilatory failure, the respiration rate was significantly decreased at 12 hour of NIV ($34{\pm}9$ /min pre-NIV, $26{\pm}6$ /min at 12 hour of NIV, p=0.045), while $PaCO_2$ ($87.3{\pm}20.6$ mm Hg pre-NIV, $81.2{\pm}9.1$ mm Hg at 24 hour of NIV) and pH ($7.26{\pm}0.04$, $7.32{\pm}0.02$, respectively, p <0.05) were both significantly decreased at 24 hour of NIV In NIV for oxygenation failure, $PaCO_2$ were not different between the successful and the failed cases at pre-NIV and till 12 hours after NIV. The $PaO_2/FIO_2$ ratio, however, significantly improved at 0.5 hour of NIV in successful cases and were maintained at around 200 mm Hg (n=6 : at baseline, 0.5h, 6h, 12h : $120.0{\pm}19.6$, $218.9{\pm}98.3$, $191.3{\pm}55.2$, $232.8{\pm}17.6$ mm Hg, respectively, p=0.0211), but it did not rise in the failed cases (n=9 : $127.9{\pm}63.0$, $116.8{\pm}24.4$, $100.6{\pm}34.6$, $129.8{\pm}50.3$ mm Hg, respectively, p=0.5319). Conclusion : From the above results we conclude that NIV is effective for hypercapnic respiratory failure and its success was heralded by reduction of respiration rale before the reduction in $PaCO_2$ level. In hypoxic respiratory failure, NIV is much less effective, and the immediate improvement of $PaO_2/FIO_2$ ratio at 0.5h after application is thought to be a predictor of successful NIV.