• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Journal of the Korean Society of Radiology
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• v.85 no.2
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• pp.327-344
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• 2024

Parathyroid glands are small endocrine glands that regulate calcium metabolism by producing parathyroid hormone (PTH). These are located at the back of the thyroid gland. Typically, four glands comprise the parathyroid glands, although their numbers may vary among individuals. Parathyroid diseases are related to parathyroid gland dysfunction and can be caused by problems with the parathyroid gland itself or abnormal serum calcium levels arising from renal disease. In recent years, as comprehensive health checkups have become more common, abnormal serum calcium levels are often found incidentally in blood tests, after which several additional tests, including a PTH test, ultrasonography (US), technetium-99m sestamibi parathyroid scan, single-photon-emission CT (SPECT)/CT, four-dimensional CT (4D-CT), and PET/CT, are performed for further evaluation. However, the parathyroid gland remains an organ less familiar to radiologists. Therefore, the normal anatomy, pathophysiology, imaging, and clinical findings of the parathyroid gland and its associated diseases are discussed here.

• Journal of the Korean Society of Radiology
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• v.84 no.1
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• pp.197-211
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• 2023

Purpose To evaluate the circuit patency after nitinol bare-metal stent (BMS) placement according to the type of access and location of the stent in dysfunctional hemodialysis access. Materials and Methods Between January 2017 and December 2019, 159 patients (mean age, 64.1 ± 13.2 years) underwent nitinol BMS placement for dysfunctional access. The location of stents was as follows: 18 brachiocephalic vein, 51 cephalic arch, 40 upper arm vein, 10 juxta-anastomotic vein, 7 arteriovenous (AV) anastomosis, and 33 graft-vein (GV) anastomosis. Circuit patency was evaluated by the Kaplan-Meier method, and cox regression model. Results A total of 159 stents were successfully deployed in 103 AV fistula (AVF) and 56 AV graft (AVG). AVG showed lower primary and secondary patency at 12-months compared with AVF (primary patency; 25.0% vs. 44.7%; p = 0.005, secondary patency; 76.8% vs. 92.2%; p = 0.014). Cox regression model demonstrated poorer primary patency at 12 months after stenting in the cephalic arch and GV anastomosis compared with the other sites. Conclusion AVF showed better primary and secondary circuit patency at 12 months following the placement of BMS compared with AVG. Stents in the cephalic arch and GV anastomosis were associated with poorer primary patency at 12 months compared to those in other locations.

• Journal of the Korean Society of Radiology
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• v.84 no.2
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• pp.477-482
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• 2023

The subclavian vein is an uncommon route for tunneled hemodialysis catheter (tHDC) placement because of its potency for future dialysis access. However, when favored access routes have been exhausted because of repeated catheterization or limited life expectancy, the subclavian vein can be used for urgent hemodialysis. A subclavian catheterization has a technical problem. The subclavian vein often forms a right angle with the vena cava, and advancing stiff peel-away sheath can cause a vascular injury. However, raising the patient's arm can alter the position of the guidewire and, therefore, change the angle of the vein favorable for tHDC placement. Herein, we report two patients who underwent subclavian catheterization; one experienced an injury to the superior vena cava after undergoing the conventional procedure, whereas the other patient with raised arm during the catheterization procedure had safe catheter placement.

• Korean Journal of Radiology
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• v.22 no.9
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• pp.1547-1554
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• 2021

Objective: We aimed to investigate whether repeated intravascular administration of iodinated contrast media (ICM) or gadolinium-based contrast agents (GBCAs) within a short interval was associated with an increased risk of post-contrast acute kidney injury (PC-AKI). Materials and Methods: This retrospective study included 300 patients (mean age ± standard deviation, 68.5 ± 8.1 years; 131 male and 169 female) who had undergone at least one ICM-enhanced perfusion brain CT scan, had their baseline and follow-up serum creatinine levels available, and had not undergone additional contrast-enhanced examinations 72 hours before and after a time window of interest were included. The study population was divided into three groups: single-dose group and groups of patients who had received multiple contrast administrations in the time window of interest with the minimum contrast repeat interval either within 4 hours (0-4-hour group) or between 4 to 48 hours (4-48-hour group). Multivariable logistic regression analysis was conducted to evaluate the association between AKI and repeated ICM administrations. A similar supplementary analysis was performed including both ICM and GBCA. Results: When ICM was only considered ignoring GBCA, among 300 patients, 207 patients received a single dose of ICM, 58 had repeated doses within 4 hours (0-4-hour group), and 35 patients had repeated doses between 4 to 48 hours (4-48-hour group). Most patients (> 95%) had a baseline estimated glomerular filtration rate (eGFR) of ≥ 30 mL/min/1.73 m². AKI occurred in 7.2%, 13.8%, and 8.6% of patients in the single-dose, 0-4-hour, and 4-48-hour groups, respectively. In the 0-4-hour and 4-48-hour groups, additional exposure to ICM was not associated with AKI after adjusting for comorbidities and nephrotoxic drugs (all p values > 0.05). Conclusion: Repeated intravascular administrations of ICM within a short interval did not increase the risk of AKI in our study patients suspected of acute stroke with a baseline eGFR of ≥ 30 mL/min/1.73 m².

• Annals of Occupational and Environmental Medicine
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• v.35
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• pp.22.1-22.9
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• 2023

Background: Shift work increases the risk of chronic diseases, including metabolic diseases. However, studies on the relationship between shift work and renal function are limited. The aim of this study was to investigate the association between shift work and a decreased glomerular filtration rate (GFR). Methods: Data were evaluated for 1,324,930 workers who visited the Korean Medical Institute from January 1, 2016 to December 31, 2020 and underwent a health checkup. Daytime workers were randomly extracted at a ratio of 1:4 after matching for age and sex. In total, 18,190 workers aged over 40 years were included in the analyses; these included 3,638 shift workers and 14,552 daytime workers. Participants were categorized into the shift work group when they underwent a specific health checkup for night shift work or indicated that they were shift workers in the questionnaire. The odds ratio was calculated using a conditional logistic regression to investigate the relevance of shift work for changes in GFR. Results: 35 workers in the shift group and 54 in the daytime group exhibited an estimated GFR (eGFR) value of < 60 mL/min/1.73m² (p < 0.01). The difference in eGFR values between two checkups differed significantly depending on the type of work (p < 0.01); the difference in the shift work group (-9.64 mL/min/1.73 m²) was larger than that in the daytime work group (-7.45 mL/min/1.73 m²). The odds ratio for eGFR reduction to < 60 mL/min/1.73 m² in the shift group versus the daytime group was 4.07 (95% confidence interval: 2.54-6.52), which was statistically significant. Conclusions: The results of this study suggest that eGFR decreases by a significantly larger value in shift workers than in daytime workers; thus, shift work could be a contributing factor for chronic kidney disease (CKD). Further prospective studies are necessary to validate this finding and identify measures to prevent CKD in shift workers.

• Journal of Korean Academy of Nursing
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• v.11 no.2
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• pp.83-104
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• 1981

Hemodialysis is essential treatment for the chronic renal failure patient's long-term cure and for the patient management before and after kidney transplantation. It sustains the endstage renal failure patient's life which didn't get well despite strict regimen and furthermore it becomes an essential treatment to maintain civil life. Bursing implementation in hemodialysis may affect the significant effect on patient's life. The purpose of this study was to obtain the basic data to solve the hypotension problem encountable to patient and the blood loss problem affecting hemodialysis patient'a anemic states by incomplete rinsing of blood in coil through all process of hemodialysis. The subjects for this study were 44 patients treated hemodialysis 691 times in the hemodialysis unit, The .data was collected at Gang Nam 51. Mary's Hospital from January 1, 1981 to April 30, 1981 by using the direct observation method and the clinical laboratory test for laboratory data and body weight and was analysed by the use of analysis of Chi-square, t-test and anlysis of varience. The results obtained an follows; A. On clinical laboratory data and other data by dialysis Procedure. The average initial body weight was 2.37 ± 0.97kg, and average body weight after every dialysis was 2.33 ± 0.9kg. The subject's average hemoglobin was 7.05±1.93gm/dl and average hematocrit was 20.84± 3.82%. Average initial blood pressure was 174.03±23,75mmHg and after dialysis was 158.45±25.08mmHg. The subject's average blood ion due to blood sample for laboratory data was 32.78±13.49cc/ month. The subject's average blood replacement for blood complementation was 1.31 ±0.88 pint/ month for every patient. B. On the hypotensive state and the coping approaches occurrence rate of hypotension was 28.08%. It was 194 cases among 691 times. 1. In degrees of initial blood pressure, the most 36.6% was in the group of 150-179mmHg, and in degrees of hypotension during dialysis, the most 28.9% in the group of 40-50mmHg, especially if the initial blood pressure was under 180mmHg, 59.8% clinical symptoms appeared in the group of“above 20mmHg of hypotension”. If initial blood pressure was above 180mmHg, 34.2% of clinical symptoms were appeared in the group of“above 40mmHg of hypotension”. These tendencies showed the higher initial blood pressure and the stronger degree of hypotension, these results showed statistically singificant differences. (P=0.0000) 2. Of the occuring times of hypotension,“after 3 hrs”were 29.4%, the longer the dialyzing procedure, the stronger degree of hypotension ann these showed statistically significant differences. (P=0.0142). 3. Of the dispersion of symptoms observed, sweat and flush were 43.3%, and Yawning, and dizziness 37.6%. These were the important symptoms implying hypotension during hemodialysis accordingly. Strages of procedures in coping with hypotension were as follows ; 45.9% were recovered by reducing the blood flow rate from 200cc/min to 1 00cc/min, and by reducing venous pressure to 0-30mmHg. 33.51% were recovered by controling (adjusting) blood flow rate and by infusion of 300cc of 0,9% Normal saline. 4.1% were recovered by infusion of over 300cc of 0.9% normal saline. 3.6% by substituting Nor-epinephiine, 5.7% by substituting blood transfusion, and 7,2% by substituting Albumin were recovered. And the stronger the degree of symptoms observed in hypotention, the more the treatments required for recovery and these showed statistically significant differences (P=0.0000). C. On the effects of the changes of blood pressure and osmolality by albumin and hemofiltration. 1. Changes of blood pressure in the group which didn't required treatment in hypotension and the group required treatment, were averaged 21.5mmHg and 44.82mmHg. So the difference in the latter was bigger than the former and these showed statistically significant difference (P=0.002). On the changes of osmolality, average mean were 12.65mOsm, and 17.57mOsm. So the difference was bigger in the latter than in the former but these not showed statistically significance (P=0.323). 2. Changes of blood pressure in the group infused albumin and in the group didn't required treatment in hypotension, were averaged 30mmHg and 21.5mmHg. So there was no significant differences and it showed no statistical significance (P=0.503). Changes of osmolality were averaged 5.63mOsm and 12.65mOsm. So the difference was smaller in the former but these was no stitistical significance (P=0.287). Changes of blood pressure in the group infused Albumin and in the group required treatment in hypotension were averaged 30mmHg and 44.82mmHg. So the difference was smaller in the former but there is no significant difference (P=0.061). Changes of osmolality were averaged 8.63mOsm, and 17.59mOsm. So the difference were smaller in the former but these not showed statistically significance (P=0.093). 3. Changes of blood pressure in the group iutplemented hemofiltration and in the Uoup didn't required treatment in hypotension were averaged 22mmHg and 21.5mmHg. So there was no significant differences and also these showed no statistical significance (P=0.320). Changes of osmolality were averaged 0.4mOsm and 12.65mOsm. So the difference was smaller in the former but these not showed statistical significance（P=0.199). Changes of blood pressure in the group implemented hemofiltration and in the group required treatment in hypotension were averaged 22mmHg and 44.82mmHg. So the difference was smatter in the former and these showed statistically significant differences (P=0.035). Changes of osmolality were averaged 0.4mOsm and 17.59mOsm. So the difference was smaller in the former but these not showed statistical significance (P=0.086). D. On the changes of body weight, and blood pressure, between the group of hemofiltration and hemodialysis. 1, Changes of body weight in the group implemented hemofiltration and hemodialysis were averaged 3.340 and 3.320. So there was no significant differences and these showed no statistically significant difference, (P=0.185) but standard deviation of body weight averaged in comparison with standard difference of body weight was statistically significant difference (P=0.0000). Change of blood Pressure in the group implemented hemofiltration and hemodialysis were averaged 17.81mmHg and 19.47mmHg. So there was no significant differences and these showed no statistically significant difference (P=0.119), But in comparison with standard deviation about difference of blood pressure was statistically significant difference. (P=0.0000). E. On the blood infusion method in coil after hemodialysis and residual blood losing method in coil. 1, On comparing and analysing Hct of residual blood in coil by factors influencing blood infusion method. Infusion method of saline 200cc reduced residual blood in coil after the quantitative comparison of Saline Occ, 50cc, 100cc, 200cc and the differences showed statistical significance (p < 0.001). Shaking Coil method reduced residual blood in Coil in comparison of Shaking Coil method and Non-Shaking Coil method this showed statistically significant difference (P < 0.05). Adjusting pressure in Coil at OmmHg method reduced residual blood in Coil in comparison of adjusting pressure in Coil at OmmHg and 200mmHg, and this showed statistically significant difference (P < 0.001). 2. Comparing blood infusion method divided into 10 methods in Coil with every factor respectively, there was seldom difference in group of choosing Saline 100cc infusion between Coil at OmmHg. The measured quantity of blood loss was averaged 13.49cc. Shaking Coil method in case of choosing saline 50cc infusion while adjusting pressure in coil at OmmHg was the most effective to reduce residual blood. The measured quantity of blood loss was averaged 15.18cc.

• Journal of Yeungnam Medical Science
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• v.14 no.1
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• pp.168-174
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• 1997

Alphafetoprotein(AFP) is a glycoprotein synthesized by the fetus early in gestation by the yolk sac and later by the gastrointestinal tract and liver. The concentration of AFP is highest in fetal serum and amniotic fluid around 13th week, and 32nd week in maternal serum. Some conditions are associated with abnormal maternal serum AFP concentration. For examples, neural tube defects, omphalocele, renal anomalies are associated with elevated maternal serum AFP and fetal death, chromosomal trisomies are associated with low level of maternal serum AFP. So maternal serum AFP screening plays a significant role in assessing candidates for prenatal diagnosis and prenatal counselling in pregnant women. This study evaluates the normal ranges of AFP using enzyme immunoassay in normal pregnant women. We studied 500 normal pregnant women who visited the Department of Obstetrics & Gynecology, Yeungnam Medical Center, Yeungnam University during the period through January, 1993 to September, 1996. The group of the study were selected randomly at various gestational ages from 8 to 41 weeks. The results were summarized as follows: 1. The lowest level of AFP in our study group was 2.1ng/ml at 8 weeks of gestation. Thereafter serum alpha-fetoprotein concentrations rose rapidly to reach a peak value at 32nd week. 2. The mean levels of AFP in the primipara and multipara were $166.37{\pm}12.06ng/ml$, and $223.78{\pm}14.00ng/ml$, respectively, showing stastiscally significant difference between these two groups(p<0.01). 3. The mean levels of AFP between mothers who delivered male and female babies were $192.96{\pm}13.00ng/ml$, and $194.29{\pm}13.84ng/ml$, respectively, without statistically significant difference(p>0.05). 4. The normal ranges of maternal serum AFP according to each gestational week were evaluated.

• Journal of agricultural medicine and community health
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• v.39 no.3
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• pp.161-175
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• 2014

Objectives: This study investigates the prevalence of suicide ideation and its the related factors by gender among Korean adults. Methods: National cross-sectional data from the database of the first and second year of $5^{th}$ Korean National Health and Nutritional Examination Survey (KNHANES V-1, V-2) were used for this study. 13,165 adults (${\geq}20$ years) were included in this analysis. Results: The prevalence of suicide ideation was 9.8% for men, 18.9% for women. Renal failure, depression, melancholy, stress, perceived health status, and smoking were significant risk factors among both men and women for suicide ideation. Divorced, separated or widowed of marital status and severe physical activity were related factors of suicide ideation for men. Low educational attainment, and alcohol dependency were associated factors of suicide ideation for women. Conclusions: There was gender difference of prevalence and risk factors of suicide ideation. These gender differences should be considered for planning and implementing suicide prevention program.

• Korean Journal of Veterinary Research
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• v.27 no.2
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• pp.277-290
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• 1987

This paper dealt with etiological studies on the acute fatal disease of Angora rabbits occurring as a group in Korea. The disease was confirmed as an acute infectious disease caused by virus. The results obtained were summarized as follows: The disease produced a high morbidity in the rearing Angora rabbits and a high mortality in the infected rabbits, and was acute. The infected rabbits died soon without premonitory signs after inappetence. The body temperature of the affected rabbits rose to $40^{\circ}C$ and nearly all deaths occurred within 48 hours after inoculation. In many cases a bloody foam was visible from the nostrils after death. According to the progress of the disease the nervous signs, such as ataxia, paralysis of the legs, and torticollis could be recognized in the some cases. Rabbits that had recovered from the disease were severe emaciation, and bristly and sparse hairs. In macroscopical findings, there were hemorrhage and edema of the lung, hemorrhage or hyperemia of the tracheal and broncheal mucosae, appearance of blood-tinged effusion in the respiratory tract. The principal lesions were found in the liver. Usually the lobular necrosis of the liver cells was progressed, and focal necrosis and hemorrhagic spots of various sizes were often observed in the liver. Liver was as a whole pale. In chronic cases, however, there was a slight liver cirrhosis with the atrophy of the parenchymal cells. The other lesions encountered grossly consisted of swelling and petechiae of the kidney, hyperemia and hemorrhage of the spleen, catarrh of the small intestine, and hyperemia of the brain. The urinary bladder contained a lot of turbid urine or bloody urine and urinary cast, and was distended with the urine. In microscopical findings, the most striking lesions occurred in the liver and may be classified as viral hepatitis. The hepatic lesions were initially characterized by progression from periportal to peripheral necrosis of the lobules with the infiltration of mononuclear cells. Focal necrosis of various sizes, hemorrhage and hyperemia were often observed in the hepatic lobules. In chronic cases, there were intensive infiltration of lymphocytes, proliferation of fibroblasts, appearance of plasmal cells, and atrophy of parenchymal cells in the hepatic tissue. Perivascular lymphocytic infiltration and meningitis were seen in the brain and spinal cord. In the kidney, there were acute glomerulonephritis, hemorrhage, necrosis of the uriniferous tubules, and retention of eosinophilic substance within the renal tubules. Proliferation of fibroblasts and infiltration of mono-nuclear cells were found in the interstitial stroma of the kidney in chronic case. There were also hemorrhage and edema in the lung, hyperemia and hemorrhage in the trachea and bronchus, perivascular lymphocytic infiltration and focal myocardial necrosis in the heart, hyperemia and hemorrhage in the spleen, vacuolization and desquamation of mucous epithelia in the urinary bladder, catarrhal inflammation of the small intestine, hemorrhage in the adrenal cortex and hyperemia in the other organs. In the electron microscopical findings of the hepatic tissue, crystals of viral particles appeared in the cytoplasm of the hepatocytes and the sinusoidal endothelial cells, and the viral particles, were small in size and polygonal. The authors suppose the virus may belong to picornaviridae family of RNA viruses. Also immature virus-like particles, dilated rough endoplasmic reticulum and destruction of nuclear membrane were seen in the hepatocytes. From these results, it is concluded that the sudden death is an acute viral disease characterized by hepatitis and the affected rabbits may be died of viremia.