• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.2
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• pp.197-202
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• 2010

Purpose: Recently, many hospitals have been tried to increase the satisfaction of the outpatients through blood-gathering, exam, result notice and process in a day. Each laboratory has been used the automatic equipment for the rapid requests of the result notice and the increase of the reliability and efficiency. Current automatic equipments that have been limited short TAT(Turn-Around Time)because of the restricted batch lists and 1 tip-5 detectors. The Gamma Pro which is made in Korea to improve the shortcomings of existing automation equipment, complemented with capacity to perform a wide range of domestic automation equipment. In this study, we evaluated the usefulness and reliability of short TAT by comparing Gamma Pro with current automatic equipment. Materials and Methods: We studied the correlation between Gamma Pro and RIA-mat 280 using the respective 100 specimens of low or high density to the patients who were requested the thyroid hormone test (Total T3, TSH and Free T4) in Samsung Medical Center Sep. 2009. To evaluate the split-level Gamma Pro, First, we measured accuracy and carry over on the tips. Second, the condition of optimal incubation was measured by the RPM (Revolution Per Minute) and revolution axis diameter on the incubator. For the analysis for the speed of the specimen-processing, TAT was investigated with the results in a certain time. Result: The correlation coefficients (R2) between the Gamma Pro and RIA-mat 280 showed a good correlation as T3 (0.98), TSH (0.99), FT4 (0.92). The coefficient of variation (C.V) and accuracy was 0.38 % and 98.3 % at tip 1 and 0.39 % and 98.6 % at tip 2. Carry over showed 0.80 % and 1.04% at tip 1 and tip 2, respectively. These results indicate that tips had no effect on carry over contamination. At the incubator condition, we found that the optimal condition was 1.0mm of diameter at 600RPM in 1.0mm and 1.5mm of at 500RPM or 1.0mm and 1.5 mm of diameter at 600 RPM. the Gamma Pro showed that the number of exam times were increased as maximum 20 times/day comparing to 6 times/day by current automatic equipment. These results also led to the short TAT from 4.20 hour to 2.19 hours in whole processing. Conclusion: The correlation of between the Gamma Pro and RIA-mat 280 was good and has not carry over contamination in tips. The domestic automation equipment (Gamma Pro) decreases the TAT in whole test comparing to RIA-280. These results demonstrate that Gamma Pro has a good efficiency, reliability and practical usefulness, which may contribute to the excellent skill to process the large scale specimens.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.195-204
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• 2004

Purpose: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin $D_3$, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. Methods: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. Results: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin $D_3$ Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. Conclusion: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.527-533
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• 2005

Purpose : Long-term administration of anticonvulsants in children with epilepsy may cause short stature, hypocalcemia and low bone mineral density. This study was performed for the early detection of abnormal bone metabolism in children with epilepsy on taking anticonvulsants. Methods : Thirty children aged 5 to 16 years who were diagnosed with epilepsy were enrolled in this study. All had taken anticonvulsants for more than one year. Bone mineral density of lumbar vertebra was measured by dual-energy X-ray absorptiometry. Serum calcium, phosphorous, alkaline phosphatase, 25-hydroxycholecalciferol[$25(OH)D_3$], parathyroid hormone, and urine deoxypyridinoline were measured as biochemical bone markers. Bone age and body mass index were also calculated. Results : Bone minreal density, body mass index, bone age, and height were significantly decreased in two female patients who had taken two antiepileptic drugs for more than four years and they also had chronic diseases such as cerebral palsy with microcephaly, encephalomalacia, and microcephaly with atrial septal defect. Bone mineral density had significant positive correlations with body mass index(P<0.01) and bone age(P<0.01). Conclusion : This study showed chronic medication of anticonvulsants in children may cause low bone mineral density and short stature. Bone age and body mass index could be the important surrogate markers to find the population at risk. More studies, including a large study population and long term cohort study, will be required.

• Korean Journal of Fisheries and Aquatic Sciences
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• v.30 no.2
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• pp.305-312
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• 1997

Juveniles of black seabream, Acanthopagrus schlegeli were fed with the diets containing 0, 10, 20, 50 and 100 ppm of 3,5,3'-triiodo-1-thyronine $(T_3)$ respectively to assess the effect of this hormone on skeletal development and the change of physiological conditions for 50 days. $(T_3)$ treatment lasted for initial 40 days. Fish were fed the prescribed diet by hand to satiation in $2\~4$ times per day. After an initial 40 days period, skeletal development and abnormality were examined, and after a 50 days period, food intake, hepatosomatic index (HSI), thyroid cell height (TCH) and body proximate composition were also examined. Although toed intake was not different among 0, 10 and 20 ppm, the food intake of black seabream fed with the diets containing 50 and 100 ppm of $T_3$was significantly lower than those of 10 ppm. After the initial 40 days of $T_3$ administration, $T_3$ increased the relative growth of operculum, head, caudal fin and pectoral fin to body length, resulting in severe morphological abnormalities at the highest dose. Black seabream treated with 50 and 100 ppm of $T_3$ had abnormal shapes such as lordosis and opercular curl. The HSI parameters were reversely correlation with the dietary concentration of $T_3$. After the initial 40 days of this experiment, atrophy of thyroid gland was observed in fish administered with 50 and 100 ppm of $T_3$. On the 50th day of this experiment, atrophy of thyroid gland was observed only in the group administered with 100 ppm of $T_3$, and no difference was observed on TCH among the rest fours of experimental groups. At the end of the experiment the whore body proximate analyses indicated that there were significant effects of $T_3$ level on moisture, protein, lipid and ash contents.

• The Korean Journal of Nuclear Medicine Technology
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• v.14 no.1
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• pp.138-142
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• 2010

Purpose: PTH (parathyroid hormone) level is a useful index for prediction of hypocalcemia after thyroidectomy. The fast results are required for an early diagnosis of hypocalcemia. In this study, we evaluated the PTH change according to incubation time, and investigated the usefulness of hypocalcemia diagnosis of PTH results in early incubation time. Materials and Methods: The subjects were 131 patients who had taken the PTH test from July to August in 2009. All experiments were used IRMA method. PTH value were evaluated with the correlation between precision (10 times repeat) and recovery rate and at 0.5, 3, 6 and $18{\pm}2$ (below overnight) hours following incubation time. Data analysis was investigated with relationship of the sensitivity, specificity, PPV (positive predictive value) and accuracy. Results: The correlation was time-dependent with levels reaching $R^2$=0.987 at 0.5 hours, $R^2$=0.993 at 3 hours and $R^2$=0.996 at 6 hours compare to overnight levels. The precision (%CV${\pm}$SD) were $15.92{\pm}15.54$ at 0.5 hours, $6.91{\pm}7.38$ at 3 hours, $4.30{\pm}4.69$ at 6 hours and $4.59{\pm}2.59$ at overnight. The recovery rate (%Mean${\pm}$SD) were $96.8{\pm}5.44$ at 0.5 hours, $102.6{\pm}4.35$ at 3 hours, $100.7{\pm}2.56$ at 6 hours and $102.2{\pm}5.98$ at overnight. When 15 pg/ml of overnight density was set up as criteria, we measured the sensitivity, specificity and PPV, accuracy at 0.5, 3, 6 hours. The sensitivity was shown to 97.5% at all times. The specificity was 96.0% at 0.5 hours, 100% at 3 hours and 92.3% at 6 hours for control, respectively. The PPV was 86.6% at 0.5 hours, 100% at 3 hours and 92.8% at 6 hours. The accuracy was shown to 84.7% at 0.5 hours, 97.5% at 3 hours and 90.6% at 6 hours. These data were accompanied by a corresponding PTH value of overnight incubation time, which significantly correlated with early time results. Conclusion: The values of PTH at 3 hours has favorable the rate of concordance of 94.1% and may be useful for prediction of hypocalcemia, and it responses to overnight incubation PTH values. Therefore, This method may be an attractive alternative to proper treatment to stop symptom revelation by giving a calcium agent to the patient.

• 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 Sasang Constitutional Medicine
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• v.8 no.1
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• pp.295-317
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• 1996

It is researched to elucidate the effects of Taeumjowuitang(TE,太陰調胃湯), Sibimikwanjungtang(SE, 十二味寬中湯) and Yangkeogsanwhatang(SY,凉膈散火湯) on the obesity induced by gold thioglucose and the differentiation and growth of preadipocyte 3T3-L1 in the mouse. The result were as follows: 1. TE,SE and SY extracts improved the blood level of transaminase in the obese mouse induced by gold thioglucose. 2. TE,SE and SY extracts inhibited the increase of liver fat and body fat in the obese mouse induced by gold thioglucose. 3. TE,SE and SY extracts inhibited the increase of body weight in the obese mouse induced by gold thioglucose. 4. TE,SE and SY extracts inhibited the growth of undifferentiate preadipocyte 3T3-L1. 5. TE,SE and SY extracts showed inhibitory effect on the differentiation of preadipocyte 3T3-L1. The above results suggest that the TE,SE and SY extracts may be used on the obesity induced by the overgrowth and differentiation of adipocyte, and the accumulation of fat in liver and body.