• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.376-379
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• 2009

We report a case of a premature newborn baby who presented with hyponatremia, hyperkalemia, and metabolic acidosis accompanied by severe polyhydramnios in utero. The baby was diagnosed with pseudohypoaldosteronism on the basis of normal 17-hydroxyprogesterone levels, elevated aldosterone, and clinical symptoms. His serum electrolyte levels were corrected with sodium chloride supplementation. Sodium supplementation was reduced gradually and discontinued at 5 months of age. At 5 months, the child was able to maintain normal serum electrolyte levels without oral sodium chloride supplementation, and showed normal physical and neurological development. This case illustrates that pseudohypoaldosteronism must be considered if a newborn infant with an antenatal history of severe polyhydramnios shows excessive salt loss with normal levels of 17-hydroxyprogesterone.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.18-24
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• 2015

Glycogen storage disease type Ib (GSD Ib) is one of the rare inherited metabolic disease caused by mutation of SLC37A4 gene. Clinical characteristics include hepatomegaly, hypoglycemia, lactic acidosis, hyperlipidemia and high serum uric acid concentration. The authors analyzed clinical and molecular characteristics of three Korean patients (one male and two females) with GSD Ib by retrospective review of medical records. Two patients were diagnosed in toddler period by hypoglycemia and hepatomegaly. One patient was diagnosed by growth retardation and short stature in puberty. c.412T>C (p.Trp138Arg) (3/6 alleles, 50.0%) was most frequently observed, following by p.Leu348Valfs*53 (1 allele), p.Pro191Leu (1 allele), p.Ala148Val (1 allele) in molecular analysis. Uncooked corn starch and allopurinol was administered. Because all three patients had neutropenia and recurrent infections, G-CSF was administered. Two patients had severe osteoporosis needing calcium supplement. The patient who diagnosed at puberty had relatively poor prognosis demonstrated by having severe infection and complications in liver and kidney.

• Tuberculosis and Respiratory Diseases
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• v.41 no.5
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• pp.504-512
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• 1994

Background: It is well known that severe hypoxemia is often associated with liver cirrhosis without preexisting cardiac or pulmonary diseases. Pulmonary vascular impairments, more specifically, intrapulmonary shunting have been considered as a major mechanism. Intrapulmonary shunting arises from pulmonary vascular dilatation at the precapillary level or direct arteriovenous communication and has relationship with the characteristic skin findings of spider angioma. However, these results are mainly from Western countries where alcoholic and primary biliary cirrhosis are dominant cuases of cirrhosis. It is uncertain that the same is true in viral hepatitiss associated liver cirrhosis, which is dominant causes of liver cirrhosis in Korea. We investigated the incidences of hypoxemia and orthodeoxia in Korean cirrhotic patients dominantly composed of postnecrotic cirrhosis and the significance of intrapulmonary shunting as the suggested mechanism of hypoxemia, Method: We performed the arterial blood gas analysis separately both at the supine and errect position in 48 stable cirrhotic patients without the evidences of severe complications such as ascites, variceal bleeding, and hepatic coma. According to the results of arterial blood gas analysis, all patients were divided into hypoxemic and normoxemic group. In each group, pulmonary function test and Tc-99m-MAA whole body scan were performed. The shunting fraction was calculated based on the fact that the sum of cerebral and bilateral renal blood flow is 32% of the systemic blood flow. Results: The hypoxemia of $PaO_2$ less than 80 mmHg was observed in 9 patients(18.8%) and Orthodeoxia more than 10 mmHg was observed in 8 patients(16.7%). But there was no patient with significant hypoxemia of $PaO_2$ less than 60 mmHg. $PaO_2$ was significantly decreased in the patients with spider angioma than the pathients without spider angioma and showed no correlation with the serologic type and severities of liver function test findings. Any parameters of pulmonary function test did not demonstrate the difference between normoxemic and hypoxemic group. But hypoxemic group showed significantly increased shunt fraction of $11.4{\pm}4.1%$ than normoxemic group of $4.1{\pm}2.0%$ (p<0.05). Conclusions: Hypoxemia is not infrequently observed complication in liver cirrhosis and intrapulmonary shunting is suggested to p1ay a major ro1e in the development of hypxemia. But there was no great likelihood of clinically significant hypoxemia in our domestic cirrhotic patients predominantly composed of postnecrotic type.

• Childhood Kidney Diseases
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• v.16 no.1
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• pp.54-57
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• 2012

A 6-month-old boy with vesicoureteral reflux exhibited features of transient type 1 pseudohypoaldosteronism (PHA) in the course of urinary tract infection. PHA presents hyponatremia, hyperkalemia, and metabolic acidosis, accompanying with high urinary sodium, low potassium excretion, and high plasma aldosterone concentration. Severe electrolyte disturbance can occur in an infant with vesicoureteral reflux because of secondary PHA. Appropriate treatment of dehydration and sodium supplementation induces rapid improvement of electrolyte imbalance and metabolic acidosis resulting from secondary PHA associated with vesicoureteral reflux.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.263-268
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• 2005

Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets as sociated with epidermal nevus syndrome with review of the literature. (J Korean Soc Pediatr Nephrol 2005;9:263-268)

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.261-266
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• 2009

Rhabdomyolysis, the clinical syndrome caused by the injury to skeletal muscle resulting in the release of muscle cell contents into the systemic circulation, has been described in association with various factors. The causes include crush injury, skeletal muscle overuse, heat, drug, abuse of alcohols and metabolic disorders as well as several types of viral and bacterial infections. We report two cases of rhabdomyolysis, which were complicated by uncommon causes, parainfluenza virus type I infection and hypernatremia.

• The Journal of Pediatrics of Korean Medicine
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• v.21 no.2
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• pp.51-67
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• 2007

Objectives The study was designed to find out the relationships between short stature and low weight among children and teenagers. Methods This research was performed in 21 children and teenagers from August 2006 to June 2007. We measured height, weight, body mass index, obesity rare, bone age, and HTMA. Moreover, we analyzed correlation with height, weight, mineral concentrations and ratios obtained from HTMA. Results There were significant correlations between HTMA and short stature and low weight. 1. Most subjects(76%) had slow metabolic types. 2. Ca, Mg, Zn, Ca/P, and Cr had significant differences between high bone age group and high chronological age group. 3. Fe/Cu had significant differences between a group that recent height is more smaller than inherited height and a group that recent height is more taller than inherited height. 4. Ca, Mg, Ca/P had significant differences between a low weight group and a normal weight group classified by obesity rate. Conclusions Children and teenagers with short stature and low weight have specific mineral characters.

• Journal of Sericultural and Entomological Science
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• v.14 no.2
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• pp.105-112
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• 1972

The effects of the stretching speed and the swelling degree of cocoon filaments on load-elongation curve were tested by tensilon with special attachments. The values of breaking strength, elongation at break and initial tensile strength obtained from load elongation curves are summarized as follows; 1. The breaking strength of the swollen cocoon filament appeared to increase at the high stretching speed, while elongation at break decreased. 2. Load-elongation curve became crinkled at the low speed of stretching. It suggests that both slip and break of the macrofibrils may occur in swelling of cocoon bave. 3. Breaking strength appeared to considerably decrease and elongation at break not to increase in swelling test at 9$0^{\circ}C$ for 60 min. 4. The initial tensile strength was influenced by the stretching speed and swelling degree of cocoon filaments.

• Investigative Magnetic Resonance Imaging
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• v.17 no.1
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• pp.19-25
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• 2013

Purpose : Renal blood oxygen level-dependent (BOLD) MRI has been used in the evaluation of renal oxygenation. We tried to provide the normal $R2^*$ value of the human kidney with 3.0 T, and evaluated the differences in $R2^*$ values according to gender and location. Materials and Methods: Twenty-four healthy volunteers underwent BOLD MRI at 3.0 T. Multi gradient echo-echo planar imaging sequence with seventeen echoes was used. After generation of the $T2^*$ map, the $R2^*$ was calculated. The statistical differences in $R2^*$ values between the cortex and medulla, males and females, and the right and left kidney were analyzed. The regional differences of $R2^*$ within the both kidneys were evaluated respectively. Results: BOLD MRI was successful in all participants. No gross artifact interfered with $R2^*$ measurement. The mean $R2^*$ at 3.0 T was $17.1{\pm}2.60s^{-1}$ in the cortex and $27.7{\pm}4.83s^{-1}$ in the medulla (p < 0.001). The $R2^*$ value in the medulla was significantly higher in the male than female volunteers (p = 0.025). There were no statistical differences of $R2^*$ according to the side and location in the kidney (p = 0.197). Conclusion: Renal BOLD MRI can be efficiently performed with 3.0 T MRI. Renal medullary hypoxia is present in normal volunteers. Our results may be used as reference values in the evaluation of pathologic conditions using BOLD MRI.

• Journal of Preventive Medicine and Public Health
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• v.28 no.4 s.51
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• pp.783-794
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• 1995

This study was conducted to clarify the relationship of body fat percent with serum lipid level and blood pressure in adults. The study subjects were 472 men and 189 women who visited Multiphasic Health Screening Center of Yeungnam University Hospital in Taegu from May 20 to September 30, 1994. The relationship of serum lipid and blood pressure with BMI, Katsura index, atherogenic index, which calculated from the health screening data and body fat percent measured by impedance fat meter(model SIF-819) were analyzed. Three groups were classified as Group I (men : body fat Percent $\geq$ 20, women : body fat percent $\geq$ 25), Group II (men : 15 $\leq$ body fat percent< 20, women : 20 $\leq$ body fat percent< 25), Group III(men : body fat percent < 15, women body fat percent <20). In this study, Group I accounted for 3.2% in men, 3.7% in women. Weight was significantly different among three groups in both sexes(p<0.01) and height was not significantly different among three groups. In men, serum total cholesterol, triglyceride, high density lipoprotein, low density lipoprotein, atherogenic index were significantly different(p<0.01). In women, serum total cholesterol and low density lipoprotein were significantly different(p<0.05) but there was no differences in triglyceride and high density lipoprotein among three groups. BMI and Katsura index were significantly different among three groups in both sexes(p<0.01). In men, body fat percent was positively correlated with weight, BMI, Katsura index, total cholesterol, triglyceride, low density lipoprotein, atherogenic index and systolic and diastolic blood pressures, and negatively correlated with high density lipoprotein. In women, body fat percent was positively correlated with age, height, weight, BMI, Katsura index, total cholesterol, triglyceride, low density lipoprotein and atherogenic index, and negatively correlated with high density lipoprotein. But there was no significant correlation between body fat percent and blood pressure in women. In multiple regression analysis for total cholesterol, fat percent, age and BMI were significant independent variables in men$(p<0.05,\;R^2=0.1286)$, and body fat percent and age in women$(p<0.05,\;R^2=0.3399)$. In case of LDL/HDL ratio, only BMI was a significant independent variable in menu$(p<0.01,\;R^2=0.0954)$, and body fat percent, age and BMI in women$(p<0.05,\;R^2=0.3164)$. In multiple regression analysis, age, low density lipoprotein and total cholesterol were significant independent variables on systolic blood pressure in men$(p<0.05,\;R^2=0.1297)$, age and total cholesterol in women$(p<0.05,\;R^2=0.1705)$. On diastolic blood pressure, only age was a significantly independent variable in men$(p<0.01,\;R^2=0.0972)$ and women$(p<0.01,\;R^2=0.1218)$. From the result of this study, it could concluded that body fat percent was significantly associated with other obesity indices and serum lipid, but had no significant association with blood pressure. To establish the relationship of body fat percent with blood pressure, further study which consider other variables that may have an effect on blood pressure should be performed.