• 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 Life Science
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• v.29 no.11
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• pp.1294-1303
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• 2019

Perchlorate is an anionic pollutant that is very soluble and stable in water. It has been detected not only in soil/ground water but also in surface water, drinking water, food, fish, and crops. Perchlorate inhibits iodine uptake by the thyroid gland and reduces production of thyroid hormones that are primarily responsible for regulation of metabolism. Although various technologies have been developed to remove perchlorate from the environment, biodegradation is the method of choice since it is economical and environmentally friendly. However there is limited information on perchlorate biodegradation in salt environment such as salt water. Therefore this paper reviews biodegradation of perchlorate in salt water and related microorganisms. Most biodegradation research has employed heterotrophic perchlorate removal using organic compounds such as acetate as electron donors. Biodegradation research has focused on perchlorate removal from spent brine generated by ion exchange technology that is primarily employed to clean up perchlorate-contaminated ground water. Continuous removal of perchlorate at up to 10% NaCl was shown when bioreactors were inoculated with enriched salt-tolerant perchlorate-reducing bacteria. However the reactors did not show long-term stable removal of perchlorate. Microorganisms belonging to ${\beta}$- and ${\gamma}$-Proteobacteria were dominant in bioreactors used to remove perchlorate from salt water. This review will help our understanding of perchlorate removal from salt water to develop a decent biotechnology for the process.

• Journal of Chest Surgery
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• v.35 no.7
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• pp.530-534
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• 2002

Background: In aortic surgery, division and ligation of the left brachiocephalic vein(LBV) may improve exposure of the aortic arch but controversy continues about the safety of this division and whether a divided vein should be reanastomosed after arch replacement was completed. The safety of LBV division and the fate of the left subclavian venous drainage after LBV division were studied. Material and Method: From November 1998 to January 2001, planned division and ligation of the LBV on the mid-line after median sternotomy was peformed in 10 patients during the aortic surgery with the consideration of local anatomy and distal aortic anastomosis. Assessment for upper extremity edema and neurologic symptoms, measurement of venous pressure in the right atrium and left internal jugular vein, and digital subtraction venography(DSV) of the left arm were made postoperatively. Result: In 10 patients there was improvement in access to the aortic arch for procedures on the ascending aorta or aortic arch. The mean age of patients was 62 years(range 24 to 70). Follow-up ranged from 3 weeks to 13 months. One patient died because of mediastinitis from methicilline-resistant staphylococcus aureus strain. All patients had edema on the left upper extremity, but resolved by the postoperative day 4. No patient had any residual edema or difficulty in using the left upper extremity during the entire follow-up period. No patient had postoperative stroke. Pressure difference between the right atrium and left internal jugular vein was peaked on the immediate postoperative period(mean peak pressure difference = 25mmHg), but gradually decreased, then plated by the postoperative day 4. In all DSV studies left subclavian vein flowed across the midline through the inferior thyroid venous plexus. Conclusion: We conclude that division of LBV is safe and reanastomosis is not necessary if inferior thyroid vein, which is developed as a main bridge connecting the left subclavian vein with right venous system, is preserved.

• 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.

• Journal of Nutrition and Health
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• v.9 no.2
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• pp.68-86
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• 1976

The primary purpose of this study is to evaluate the correct nutritional status on pre-school children in Korea. Furthermore, it made an attempt to find and define nutrional problems, and assist in establishment on their nutritional improvement plan. For this, food intake and health condition (physical, clinical, biochemical and parasitological) survey on 109 Pre-school children in both sexes, randomly selected from Yang-Gu area in Gang-Won province and Rea-ju area in Kyong-gy Province, were conducted by means of three-day records, during the two periods of Spring and Fall season in 1975. The results obtained are summerized as follows: 1. The food intake; Average food intake of the subjects per day were $508{\sim}647g$ ($83{\sim}91%$ in vegetable foods and $5.5{\sim}11.7%$ in animal foods) in Yang-gu area, and $587{\sim}698g$ ($88{\sim}89%$ in vegetable foods and $6.3{\sim}7.6%$ in animal foods) in Rea-ju area. 2. The intake of energy and nutrients; a) Calory intake. Average energy intake of subjects per day in Yang-gu area$(1120{\sim}1415kcal)$ were all lower than the Korean Recommended Dietary Allowances (RDA) in either Spring and Fall survey, whereas the subjects in Rea-ju area were lower intake $(1213{\sim}1418kcal)$ than the RDA in the Spring but higher intake$(1516{\sim}1755kcal)$ than the RDA in the Fall, and the average intake were similar level with that of RDA. b) Protein intake. Average protein intake of the subjects per day in Yang-gu area $(33{\sim}43g)$ girl subjects in Rea-ju area $(35{\sim}39g)$ were lower than the RDA in either Spring and Fall survey, whereas the boy subjects in Rea-ju area$(36{\sim}38g)$ were lower in Spring and higher $(49{\sim}57g)$ in the Fall than that of the RDA, but the average $(43{\sim}47g)$ were similar level with the RDA. The protein intake from animal sources in all subjects were much lower $(5.5{\sim}11.7\;of\;total\;protein)$ than the RDA. c) Fat intake. Average fat intake were very lower in all subjects of both area $(14{\sim}24g\;in\;Yang-gu,\;10{\sim}12g\;in\;Rea-ju)$ than that of RDA which is recommended $12{\sim}14%$ of total energy to be supplied from fat. d) Calcium intake. Average calcium intake were very low in all subjects of both area $(264{\sim}355mg\;in\;Yang-gu\;and\;283{\sim}429mg\;in\;Rea-ju)$, especially, these in Spring were about a half level of the RDA, and it was much increased in the Fall due to increased intake of milk, but it was still not enough than the RDA. e) Vitamin A intake. Average intake of V.A ($703{\sim}1465\;IU$ in Ynag-gu and $750{\sim}1521\;IU$ in Rea-ju) were also lower than the RDA, moreover their V-A sources were mainly vegetable, so that the V-A supply might be critical one for the subjected. f) Riboflavin intake. Average riboflavin intake on all subjects in both area except boys in Rea-ju area in Fall, were very lower than the RDA. 3. The physical status; a) Average weight and height of boys aged 4 and 5 in Yang-gu area and girls of aged 5 in Rea-ju area were lower than those of Korean Standard of 1967 report, but those by age of girls in Yang-gu area and boys in Rea-ju area were a little heigher than the Korean Standard. It is, hower, present Korean standard of physical status might be somehow heigher than the 1967, since the socio-economical situation has been much improved during past a decade. So that, if one considered on this sense, the physical status of the subjects on this survey might be somehow lower than those of present Korean standard. b) Average upper arm circumference in both area were no difference each other, and their mean values of age 4, 5 and 6 in boy and girl were 15.6, 16.5, 16.4 and 15.5, 16.5, 16.4cm respectively. c) Average chest girth of boys were similar to those of Korean standard whereas the girls were smaller than the Korea standard. The average head circumference also showed similar tendency with the chest girth. 4. The clinical findings; The most popular clinical signs were angular stomatitis and dental caries, and boys had more heigher incidence then the girls. 5. The biochemical findings; a) Hemoglobin and anemia Average Hb value of boys and girls were 11.4 and 10.9g per 100 ml of blood respectively. The incidence of anemia (Hb value below 11 g/100 ml, by WHO) was increased by age, and girls had more heigher incidence than the boy (34% : 48%). The incidence of anemia in age of 4,5, and 6 in boys and girls were 28%, 41% 34%, and 33%, 50%, 49% respectively. The degree of the anemia was not severe, and the anemia of there subjects may be caused mainly low intake of better quality protein and low iron intake as well. b) Hematocrit. Average Ht value of whole subject were $39.9{\sim}41.6%$. c) Blood plasma protein. Average blood plasma protein contents of whole subjects were $6.6{\sim}7.4gm$ per 100 ml. The incidence of deficient range (<6.0g%, by ICNND) was only one girl of age 4 in yang Gu area. 6. Parasitological findgs; The most popular parasitism were asicris lumbicoides and trichocephalus trichiura, and about 2/3 of the whole subjects were suffering one or more of these parasitism.