• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.63-70
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• 2001

Purpose: The aim of this study is to evaluate the effective role of living-related liver transplantation (LRLT) on posttransplant linear growth in children. Methods: Thirty six children were enrolled who received LRLT at Asan Medical Center from December, 1994 to February, 1999 and showed more than one-year postoperative survival. Mean height standard deviation score (zH) was analyzed according to medical records including heights during pretransplant and posttransplant follow-up periods. Results: zH of total children showed significant linear growth after LRLT from -1.58 to 0.33 at 24 posttransplant month (p<0.05). zH in children under 6 years of age, to exclude the effect of adolescent linear growth spurt, showed increment in height (p<0.05). Linear growth of children with liver cirrhosis improved and that with fulminant hepatitis was matained same. While stunted children (mean zH=-2.30) achieved good catch-up growth after transplantation, children with normal growth remained same. Children with significant hepatic dysfunction after LRLT such as chronic rejection or posttransplant lymphoproliferative disorder showed retarded posttrasplant linear growth. There was no statistical difference according to the type of immunosuppressants. Conclusion: LRLT resulted in adequate or catch-up linear growth in children with acute, chronic and metabolic liver disease. Successful LRLT suggested to be a promising option not only in long term survival but also in normal linear growth.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1983.05a
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• pp.11.2-11
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• 1983

Vocal nodules and polyps are much more frequent in singers, public speakers, teachers and actors. Voice trauma and voice misuse, at times associated with mild inflammatory reaction, appear to be important in their etiology. It is generally agreed that vocal cord nodules and polyps are inflammatory in nature and they arise in the subepithelial layer of loose connective tissue of the vocal cord. Since the junction of anterior and middle thirds of the membranous cord and has the greatest amplitude of vibration. This is the site of predilection for vocal cord nodules. The author performed laryngomicrosurgery for 70 cases of vocal nodules and polyps at Ewha Womans University Hospital during the period of 5 years. The result obtained were as follows ; 1) Surgical excision is not necessarily the best approach because vocal nodules in the early stages will resolve with the simplest voice therapy. 2) In children, surgery is rarely indicated because most nodules in children regress during adolescence. 3) For patients who use their voices professionally, voice therapy is indicated for three months. 4) If after three month of conservative treatment the cord lesion does not improve and the patient it still dissatisfied with his voice, laryngomicrosurgery can then be considered. 5) The small cuffed endotracheal tube in the interarytenoid space helps to keep the cords immobile and in an abducted position. 6) Removal of the nodule shoule be started by gentle retraction posteriorly and as soon as a tear appears anterior to the nodule. 7) On occasion it is preferable to start the dissection with a siccle knife while the nodule is held on the stretch. 8) Voice rest should be maintained for a week following which the free edges of the cords are usually healed.

• Korean Journal of Food Science and Technology
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• v.35 no.5
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• pp.980-987
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• 2003

In this report, we investigated the detoxication effects of Saururus chinenis, Geranium nepalense, Lonicera japonica, Cassia obtusifolia, Glycyrrhiza uralensis, or their mixtures by employing acute toxicity tests for nicotine and dioxin. When fatal doses $(LD_{100}\;=\;42\;mg/kg)$ of nicotine were injected into the abdominal cavities of ICR mice, those treated with OHEM showed delayed paralysis, half the duration of hyperactivity, and a 73 % survival rate. The results revealed the strong detoxicating effects of the mixtures. We also measured the amount of the degradation product of nicotine and cotinine in humans. Consumption of OHEM promoted (he more specific) the metabolic pathways of nicotine, increasing continine excretion by 1.5 times. As a result the amount of cotinine in urine was reduced to less than 5% after treatment with OHEM. In order to test the toxicity of dioxin, we used TcnN(SD)BR rats exposed to TCDD. While TCDD treatment reduced the blood levels of hemoglobin and platelet, OHEM consumption relieved these effects and, furthermore, helped to recover the number of platelet to the normal level (p<0.05). Moreover, neutrophils (%) and monocytes (%), which were reduced by the injection of TCDD, recovered to normal levels upon treatment with OHEM. The amount albumin reduced by TCDD (p<0.05) normalized, while the activities of GOT and GTP increased by TCDD were reduced. Increases in total cholesterol and neutral fatty acids induced by TCDD were also reduced by OHEM injection (p<0.05). In the kidney, TCDD-induced rises in creatinine were suppressed by OHEM treatment, while decreases in iron levels from TCDD were raised to normal. The treatment of TCDD had more toxic effects in the blood and pancreas than on the liver, kidney and heart. On the other hand, the detoxication of OHEM had significant effects on the liver and pancreas. The normalization by OHEM of various clinical abnormalities induced by TCDD demonstrates the detoxicating effect of OHEM that ameliorates systemic metabolism not properly functioning.

• Journal of Preventive Medicine and Public Health
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• v.26 no.2 s.42
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• pp.222-230
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• 1993

Industrial glues, known as 'Bonds' in Korea, contain many kinds of organic solvents, and glue sniffing of youths became one of the social problems in Korea. Mixed exposures to solvents by glue sniffing may induce chronic toxicities different from those by exposures to solvents of single component. To test effects of the glue sniffing on weight gain or central nervous system, two groups of 20 male Sprague-Dawley rats were exposed to air(control group) or vapors of the glues to narcotic status(exposed group), and weight check, tail flick test, hot plate test, rotarod treadmill test were done on the 14th,24th, 36th, 45th, 53rd, 86th, 102nd, 117th, 134th and 151st days after the first exposure. On the 188th day, their brains were excised and examined by a pathologist. Weight gain, controlled against time change, showed significant difference between the groups, but response times in tail flick test, hot plate tests, and rotarod treadmill test didn't. In pathological examination with blind method, no macroscopic or microscopic differences were found between the two groups. These results suggests that organic lesion in central nervous system may not ensue glue sniffing, but, before firm conclusion, more studies in various exposure conditions should be followed.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.64-70
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• 2014

Purpose: The aims of this study were to assess the clinical and laboratory profiles of chronic kidney disease-mineral bone disorder (CKD-MBD) and to assess the effects of treatment of active vitamin D analogs on severe hyperparathyroidism (SHPT) in pediatric patients on chronic peritoneal dialysis. Methods: This is a retrospective study included 53 patients who had been undergoing dialysis for more than 1 year, between January 2003 and December 2012. Results: Even after treatment with phosphate binders and active vitamin D analogs, the $mean{\pm}standard$ deviation of the percentage of time during peritoneal dialysis that the patients' serum concentrations of phosphorus, corrected total calcium, and parathyroid hormone (PTH) fell within the Kidney Disease Outcomes Quality Initiative recommended ranges was $25.06{\pm}17.47%$, $53.30{\pm}23.03%$, and $11.52{\pm}9.51%$, respectively. Clinical symptoms or radiological signs of CKD-MBD were observed in 10 patients (18.9%). There were significant differences in percentage of time that the serum intact PTH concentration was outside of the recommended range between patients with and without symptoms or signs of CKD-MBD (below recommended range, $11.74{\pm}7.37%$ vs. $40.77{\pm}25.39%$, P <0.001; above the recommended range, $63.79{\pm}27.86%$ vs. $37.09{\pm}27.76%$, P =0.022). Of the 25 patients with SHPT, high-dose alfacalcidol treatment was required in 13 patients that controlled SHPT in 7 of these patients, without marked complications. Conclusion: Despite our efforts to manage CKD-MBD, patients' met the recommended ranges from relevant guidelines at a low frequency. The treatment of high-dose active vitamin D analogs was required in about half of the patients with SHPT and effective in about half of them.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.51 no.3
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• pp.248-259
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• 2001

Background : In diagnosis or monitor of the airway obstruction in bronchial asthma, the measurement of $FEV_1$ in the standard method because of its reproducibility and accuracy. But the measurement of peak expiratory flow(PEF) by peak flow meter is much simpler and easier than that of $FEV_1$ especially in children. Yet there have been still no data of the predicted normal values of PEF measured by peak flow meter in Korean children. This study was conducted to provide equations to predict the normal value of PEF and correlation between PEF and $FEV_1$ in healthy children. Method : PEF was measured by MiniWright peak flow meter, and the forced expiratory volume and the maximum expiratory flow volume curves were measured by Microspiro HI 501(Chest Co.) in 346 healthy children(age:5-16 years, 194 boys and 152 girls) without any respiratory symptoms during 2 weeks before the study. The regression equations for various ventilatory parameters according to age and/or height, and the regression equations of $FEV_1$ by PEF were derived. Results : 1. The regression equation for PEF(L/min) was: $12.6{\times}$age(year)+$3.4{\times}$height(cm)-263($R^2=0.85$) in boys, and $6{\times}$age(year)+$3.9{\times}$height(cm)-293($R^2=0.82$) in girls. 2. The value of FEFmax(L/sec) derived from the maximum expiratory flow volume curves was multiplied by 60 to compare with PEF(L/min), and PEF was faster by 125 L/min in boys and 118 L/min in girls, respectively. 3. The regression equation for $FEV_1$(ml) by PEF(L/min) was:$7{\times}$PEF-550($R^2=0.82$) in boys, and $5.8{\times}$PEF-146 ($R^2=0.81$) in girls, respectively. Conclusion : This study provides regression equations predicting the normal values of PEF by age and/or height in children. And the equations for $FEV_1$, a gold standard of ventilatory function, was predicted by PEF. So, in taking care of children with airway obstruction, PEF measured by the peak flow meter can provide useful information.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.7-14
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• 2021

Purpose: Pseudohypoparathyroidism (PHP) is caused by genetic and epigenetic alteration in the GNAS locus, and characterized by the resistance to multiple hormones and the Albright's hereditary osteodystrophy (AHO) phenotype. This study investigated the phenotypic characteristics and molecular features of PHP. Methods: Eight patients who diagnosed as PHP were enrolled at Pusan National University Children's hospital and clinical features, biochemical and genetic findings were retrospectively reviewed. Results: Of a total of 8 patients, 5 were diagnosed with PHP1a, and 3 were diagnosed with PHP1b. Patients with PHP1a had three different mutations in the GNAS gene, and patients with PHPIb had imprinting defect in differentially methylated regions (DMRs) of the GNAS locus. Two novel GNAS variants were identified in patients with PHP1a, including c.313-2A>T and c.1094G>A. All patients with PHP1a displayed AHO features; short stature (80%), brachydactyly (80%), a round face (80%), obesity (40%), heterotopic ossification (60%), and intellectual disability (60%), whereas only one patient (33.3%) with PHP1b showed AHO feature such as a round face. When phenotypic features between PHP1a and PHP1b patients were compared, patients with PHP1b showed a tendency of higher current height standard deviation scores (SDS) compared to patients with PHP1a, (-3.2±2.1 vs.-1.1±0.8; P=0.06) Conclusions: This study summarizes the phenotypic and genetic features of the PHP patients. Although we found considerable clinical overlap between PHP1a and PHP1b, further long-term follow-up is needed to evaluate the growth and development of children with PHP, as well as the effects of end-organ resistances to endocrine hormones.

• Korean Journal of Food Science and Technology
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• v.48 no.6
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• pp.604-609
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• 2016

In postmenopausal women, estrogen deficiency can be associated with metabolic diseases, such as obesity, diabetes, and cardiovascular diseases. The purpose of this study was to investigate factors related to lipid, glucose and antioxidnat metabolism. Sprague-Dawley female rats were subjected to either bilateral ovariectomy or sham operation and randomly divided into 3 dietary groups, (n=8) sham-operated fed with normal diet (SHAM), ovariectomized fed with normal diet (OVX), and ovariectomized fed with normal diet supplemented with Eucommia ulmoides extract (OVX-EU). The OVX-EU group showed significantly lower body weight, triacylglycerol, and total cholesterol than that showed by the OVX group. In addition, the OVX-EU group showed improved lipogenesis, glucose-regulating enzyme activities, adipokine and antioxidant enzyme activities. These findings demonstrate that extracts from E. ulmoides extract can be used as a functional food.

• Journal of Life Science
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• v.29 no.3
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• pp.318-324
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• 2019

Squamous cell carcinoma is the primary tumor type in head and neck cancers, the fifth most common malignant neoplasm world-wide. Survivin, a member of the inhibitor of apoptosis family, is highly expressed in head and neck carcinoma patients and correlated with more aggressive forms. In this study, we investigated whether YM155, a specific survivin inhibitor, could induce apoptosis in head and neck AMC-HN4 cells. YM155 was found to markedly induce apoptosis and cleavage of PARP, a marker of apoptosis. Furthermore, YM155 promoted apoptosis in other cancer cells, such as glioma (U251MG) and renal carcinoma (Caki) cells. In contrast, YM155 had no effect on apoptosis in normal mesangial cells. YM155 significantly induced caspase activation, and pan caspase inhibitor z-VAD-fmk markedly blocked apoptosis, PARP cleavage, and caspase-3 cleavage. Therefore, YM155 was seen to instigate caspase-dependent apoptosis in head and neck AMC-HN4 cells, inducing downregulation of survivin as well as other apoptotic proteins such as c-FLIP and Mcl-1. In addition, the induction of apoptosis and PARP cleavage by YM155 treatment was effectively inhibited in survivin-, c-FLIP- and Mcl-1-over-expressing head and neck AMC-HN4 cells. In conclusion, YM155 is a potent candidate for inducing cell death in head and neck AMC-HN4 cells.