In case of the acute intake of radionuclide, an early medical treatment may be necessary, but the little is established the procedures to decontaminate the victims of internal contamination in Korea. The purpose of the present investigation is to study chemical agents to remove radiocobalt from the victims and to provide a more reliable procedure for the treatment. The removals of radiocobalt from the NIH-CGP)mice injected intraperitoneally with $1{\mu}Ci$ of $^{58}Co$ as $CoCl_2$ were investigated with doses of either $CaNa_3$ DTPA 8.4mg/0.2ml saline, $CoNa_3$ DTPA 8.4mg/0.2ml saline, or saline 5ml. The radioactivity was determined by MCA and Ge-detector on 4, 8, 12, 48 hours and 7 days for the whole body, organ distribution and urine excretion. Six mice per each group were sacrificed for the measurement of cobalt retention in the parenchymal tissue. The cobalt trisodium chelate had a pronounced effect on reducing the whole body retention and increasing the excretion rate. Regarding to the systemic protective effects, $CoNa_3$ DTPA, $CaNa_3$ DTPA and saline were effected significantly in order. In conclusion, the extrapolations from these results to human were suggested that the rapid administration of cobalt trisodium chelate and an amount of saline to the contaminated person after internal contamination of radiocobalt were markedly increasing the decontamination effects.
During the past few years it has been proposed that lithium clearance can be used as a reliable measure for the outflow of tubular fluid from the proximal tubule. This study was aimed to characterize the inflow dependent reabsorption of Na in renal tubule beyond the proximal tubule. For this purpose, lithium clearance was used as a measure for the inflow from the proximal tubule and the changes in reabsorption fraction of Na and water were determined in rabbits. Rabbits were pretreated with hypotonic saline solutions for an hour (50 mM/L NaCl, 20 ml/hr/kg). And then a hypertonic solution of 500 mM/L NaCl (20 ml/kg) was administered intraperitoneally in conjunction with a bolus of LiCl solution (2 mM/kg, i.v.) for conditioning the $C_{Li}$ and urine flow rate. To rule out the effect of $Li^+$ on tubular functions, a bolus of NaCl solution (2 mM/kg, i.v.) was administered. Fifteen, thirty, and sixty minutes after injection of hypertonic saline arterial blood and urine samples were taken. Urinary and plasma concentrations as well as urinary output of $Li^+,\;Na^+\;and\;K^+$ were measured. From these $C_{Li},\;C_{Na}$ and the reabsorption fraction of Na and water $(Fr_{Na}\;&\;FrH_2O)$ were calculated. These results were compared with those from control groups in which the same amount of isotonic saline (145 mM/L NaCl) and of 15% dextran solution were administered in the same way as that in experimental group. Followings are the results obtained. 1) The plasma concentration of $Na^+$ in rabbits injected with hypertonic saline reached the peak value after 15 min and thereafter no significant change was observed. Hematocrit values did not show any change, while urinary excretion of $Na^+$ increased markedly during the first 15 min and decreased thereafter. These results were not affected by an injection of a small amount of LiCl. 2) The clearances of $Li^+,\;Na^+\;and\;K^+$ in rabbits injected with hypertonic saline and LiCl solution decreased. 3) In spite of the variation in $C_{Li},\;Fr_{Na}$ did not show any significant change while $FrH_2O$ increased gradually. 4) $C_{Li}$ decreased also in rabbits received isotonic saline. $Fr_{Na}$ tended to be higher than that in hypertonic saline group, while $FrH_2O\;and\;Fr_{Na}$ did not associated with the decrease in $C_{Li}$. 5) $C_{Li}$ of the rabbits received dextran solution fluctuated persistently and $Fr_{Na}\;and\;FrH_2O$ did not change in along with $C_{Li}$ although $Fr_{Na}$ had a tendency to be higher than that in hypertonic saline group. 6) From the above results it was concluded that: (a) In rabbits with normal body store of $Na^+$, the $Fr_{Na}$ of renal tubule beyond proximal tubule. calculated from $C_{Li}$ as a measure of inflow from proximal tubule is constant in spite of variations in $C_{Li}$. (b) The $FrH_2O$ calculated from $C_{Li}$ is dependent largely upon ADH rather than inflow from proximal tubule. (c) When there is a decrease in plasma $Na^+$ concentration or ineffective body fluid. $Li^+$ reabsorption may occur in the thick segnent of Henle's loop and hence the determination of $Fr_{Na}$ and $FrH_2O$ will not be easy one, but $Fr_{Na}$ is constant under the same experimental conditions.
Journal of The Korean Society of Inherited Metabolic disease
/
v.16
no.1
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pp.24-33
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2016
Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.
Kim, Young-Myoung;Byun, Jee-Young;Han, Chan-Kyu;Sung, Ki-Seung;Namgung, Bae
Korean Journal of Food Science and Technology
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v.41
no.2
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pp.203-209
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2009
This study was conducted to evaluate the effects of the salts fortified with Hizikia component on blood pressure and mineral composition in spontaneously hypertensive rats (SHR/NCrj) and normotensive rats (WKY/NCrj). The two species rats were assigned to three groups and were fed with drinking water to which Hizikia salts were added for 6 weeks. The final blood pressures (BP) among groups of SHR were increased to 12, 4, and 21%, respectively. In terms of urine and fecal minerals, the $Na^+$ contents in two species were significantly higher in the control than in the salt groups, and vice versa with regard to the $ K^+$ contents. The amount of excretion mineral was higher in urine than in feces. The $Na^+$, $Ca^{++}$ and $Mg^{++}$ contents of the liver in SHR were higher in the control than in the salt groups (p<0.05). The $Na^+$ content of the kidney in SHR was higher in the control than in the salt groups, but the content was shown an opposite trend in WKY. The $K^+$ contents were higher in the low salt group (p<0.05). These results may indicate that the salts fortified with the Hizikia component may be useful in lowering systolic blood pressure owing to the maintenance of positive mineral metabolism.
Since ${\beta}-aminoethylphosphonic$ acid was discovered in the living organism, the biosynthesis and biological function of aminophosphonic acids have been extensively studied. The purpose of this project consists in the two parts: 1)the preparation of DL-1-amino-2-phenylethylphosphonic acid (Phenylalanine aminophosphonic acid) and DL-1-amino-3-methylbutyl-phosphonic acid (Isoleucine aminophosphonic acid) by the method of Chamber and Isbell. 2) the study of metabolism and biological functions of those synthetic materials by the animal experiment (white rats) The importance of this project proved to be the first experience fed by animals for the elucidation of biochemical and metabolic functions in the animal body. The following organic synthesis of DL-1-amino-3-methylbutylphosphonic acid and DL-1-amino-2-phenylethylphosphonic acid are studied. 1)Synthesis of DL-1-amino-3-methylbutylphosphonic acid a) Synthesis of Iso-butylbromide b) Synthesis of Ethyl iso-butylmalonate c) Synthesis of Iso-caproic acid d) Synthesis of $Ethyl-{\alpha}-bromo$ iso-caproate e) Synthesis of $Triethyl-{\alpha}-phosphono$ iso-caproate f) Synthesis of DL-1-amino-3-methylbutylphosphonic acid 2)Synthesis of DL-1-amino-2-phenylethylphosphonic acid a) Synthesis of Diethyl phosphite b) Synthesis of Ethylchloro acetate c) Synthesis of Triethyl phospho acetate d) Synthesis of Triethyl benzyl phospho acetate e) Synthesis of DL-1-amino-2-phenylethylphosphonic acid The synthetic compounds; DL-1-amino-3-methylbutylphosphonic acid and DL-1-amino-2-phenyl ethylphosphonic acid which are essential amino acid (isoleucine, phenylalanine)analogue are supplemented to the animal diet at the level of 0.2% and 0.4% for isoleucine analogue and 0.35% and 0.7% for phenylalanine analogue. The plain isoleucine and phenylalanine at the same level in the diet are fercilitated as comparable groups in this study. Two sets of experience including 100 male rats were carried out for seven weeks each total 14 weeks. During this period, urine samples, and each big organs were collected for the analysis of total nitrogen, phosphorus, and glycogen contents in the individual samples by Micro Kjeldahl Fisk & Subbarow and Nelson Somogye, method. 1) The result of the project a) The yield of DL-1-amino-3-methylbutylphosphonic acid and DL-1-amino-2-phenylethylphosphonic acid showed low tendency at the level of 12.5% and 20% Melting point of those two compounds were very high and the ${\alpha}-amino$ group in the synthetic compounds showed positive reaction with ninhydrin in the violet color. b) Ail the experimental groups included in this study revealed statistically no significant difference in the organ weight, total body nitrogen retention and urinary phosphorus excretion This means isoleucine aminophosphonic acid and Phenylalanine aminophosphonic acid were utilized in the body as much as the plain amino acids, isoleucine and phenylalanine did. c) The glycogen contents in the liver of the phenylalaine aminophosphonic acid gruop showed higher statistically significant(p<0.05) in the comparision with the group of the Phenylalanine and the Standard-2. It was noteworthy that the higher glycogen content in the liver might indicate the significance in the incorporation of phenylalanine aminophosphonic acid into the intermediate of tricarboxylic acid cycle as activated state.
Kim, Chang-Woo;Cho, Min-Hyun;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
Childhood Kidney Diseases
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v.6
no.1
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pp.48-55
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2002
Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)
Choi Hyun Jin;Cho Hee Yeon;Kim Eo Jin;Lee Byong Sop;Kang Hee Gyung;Ha Il Soo;Cheong Hae Il;Choi Yong
Childhood Kidney Diseases
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v.9
no.2
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pp.183-192
/
2005
Purpose : The long term disease course and prognostic factors were evaluated in childhood Henoch-$Sch{\ddot{o}}nlein$ puruura nephritis(HSPN). Methods : A total of 75 children(44 boys and 31 girls) with HSPN were included in this study. The onset age was $8.0{\pm}3.1$ years(2.3-l5.3 years), and the follow-up period was $4.3{\pm}3.6$ years(1.0-17.1 years). Kidney biopsy was done in 24 children(32$\%$). Initial clinical and laboratory findings were evaluated. In addition, polymorphisms of the renin angiotensin system(RAS) genes(insertion/deletion in intron 16 of ACE gene, M235T in AGT gene, and A1166C in AGTR gene) were analysed. The initial and last clinical states were classified into 4 groups as follows A, normal; B, minor urinary abnormalities; C, active renal disease (nephrotic-range proteinuria and/or hypertension with serum creatinine $\leq$1.5 mg/dL); D, renal insufficiency. Results : At the onset, the clinical states of the patients were B in 26(35$\%$), C in 46(61$\%$), and D, in 3(4$\%$). The distribution of the RAS gene polymorphism of HSPN were not different from that of 100 healthy control subjects. At the last follow-up, the clinical states of the patients were A in 23(31$\%$), B in 38(50$\%$), C in 9(12$\%$), and D in 5(7$\%$). A multiple logistic regression identified age at the onset and initial urine protein excretion as significant prognostic factors. Analysis of genotypes of the 3 RAS genes as prognostic values revealed no statistical significance. Conclusion : Older age at onset and severe proteinuria were identified as poor prognostic factors of childhood HSPN. Implication of the RAS gene polymorphism In HSPN could not be validated in this small-scale retrospective study. (J Korean Soc Pediatr Nephrol 2005;9:183-192)
Hong Ihn Hee;Ko Cheol Woo;Koo Ja Hoon;Kim Ji-Hong;Kim Pyung-Kil;Cho Byoung Soo
Childhood Kidney Diseases
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v.3
no.1
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pp.48-56
/
1999
Purpose : This multicenter collaboratory study was conducted to see the therapeutic efficacy and side effect of cyclosporine A (Cipol-$N^{(R)}$, Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing (FR), steroid dependent (SD), or steroid resistant (SR) pattern. Patients and methods : Thirty-nine children with SD/FR NS and 3 children with SR NS were enrolled in the study. After induction of remission (SD/FR NS) with steroid or after 4 weeks of steroid therapy (SR NS), cyclosporine A was started in a dose of 4-5 mg/Kg/day in two divided dose and steroid (prednisolone or equivalent dose of deflazacort) was tapered slowly. During 16 weeks of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done. Results : Out of 39 children with SD/FR NS, 35($89.7\%$) maintained sustained remission and at 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Two out of 3 children with SR NS showed and sustained remission with cyclosporine A therapy. Side reaction to cyclosporine A therapy showed hypertrichosis in 8 cases and hyperuricemia in 5 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR (creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 16 weeks of study period. Conclusion : Cyclosporine A (Cipoi-$N^{(R)}$ Chong Kun Dang) can be utilized quite effectively on children with SD/FR or SR NS and further trial of cyclosporine A on long-term basis (1-2 year period) is needed to determine it's efficacy and side effect (especially nephrotoxicity) of long-term administration of cyclosporine A.
This experiment was performed to investigate the effect of the frequency of meals on the metatolism and the body composition of rats when equal amount of purified diet was ingested. Thirty approximately days old rats weighing 290 g and thirty-two about 40 days old rats weighing 180 g were employed for the period of 34 days. Rats fed ad libitum (10 to 15 meals per day) and two-meal per day were pair-fed and equal amount of diet was fed to each rat in pair. The experimental results obtained are summarized as follows: 1. Frequency of meal did not exert any effect on the body weight gain. However, rats fed two-meal per· day gained significantly (p <0.005) more fat and energy than ad libitum group. The rate of gain of protein in ad libitum group was higher than that of two-meal group. No difference was observed for the mineral deposition of rat body. 2. From the preperation of rat liver it was found that the activity of glucose-6-phosphate dehydrogenase was much higher for the rats fed two-meals per day than those fed ad libitum. Therefore, it is suggested that the metabolic pathway of carbohydrate for two-meal group has been shifted from glycolysis to Hexose Monophosphate Shunt and produced more NADPH which would be the essential cofactor of fatty acids synthesis. 3. The rate of excretion of urinary nitrogen for two-meal group was significantly (p<0.005) higher than that of ad libitum group. It is apparent that considerable amount of over-loaded amino acids by feeding two-big-meal daily· could not be used for the protein biosynthesis all at once and excreted following deamination through urine. The residual carbon chain could be served as a precursor of fatty acids synthesis. 4. The heat production rate of rats fed two-meal group was significantly (p<0.005) lower than that of ad libitum group. It seems possible that the activity of thyroid gland (and consequently BMR) can be depressed by the frequency of meal.
This study was done to investigate the effects of rapidly-rotating shift work of two-day interval on fatigue level and the concentration of urinary 17-KS, $Na^+,\;Cl^-$. The subjects were 20 nursing college students(control group) and 15 nurses in a university hospital and the study was done from Apr. 21 to May 4th, 1999. In the test group, each 5 nurses were allocated to day shift(8 AM-4 PM), evening shift(4 PM-12 MN) and night shift(12 MN-8 AM) respectively. The fatigue level were measured 30 minutes after work start on the 2nd day of work shift. Urine specimens were collected at 8 AM, 4 PM and 12 MN on the 2nd day of work shift in the control group and 30 minutes before and after work on the 2nd day of work shift in the test group. The data were analyzed with SPSS(for Window, ver 7.5). Statistical analysis was performed by using t-test, paired t-test and ANOVA. The results were as follows. 1. The perceived fatigue level in shift work 1) The physical and mental fatigue level were significantly higher in night shift than that in day or evening shift(p<0.05). In the neuro-sensory fatigue level, night shift showed higher tendency than that in day or evening shift, but there were no significant differences between each shifts. 2) Comparison between the control group and the test group: Physical fatigue level was significantly higher in night shift than that in day or evening shift of the control group(P<.001). Mental fatigue level was significantly higher in day or night shift than that in evening shift of the control group(P<.05). In the neuro-sensory fatigue level, test group showed higher tendency than that in the control group, but there were no significant differences between two groups. 3) The total fatigue level was higher in night shift than that in day shift or evening shift(P<.05). In comparing with the control group, night shift and day shift showed higher total fatigue level than that in the control group(p<0.05). 2. The concentration of urinary 17-KS, $Na^+$ and $Cl^-$ In the control group, urinary 17-KS, $Na^+$ and $Cl^-$ showed higher level in afternoon that in morning and night. In the test group, cr in day and evening shift and $Na^+$ in evening shift showed higher level at the end of work. The 17-KS concentration at the begining and the end of work in three shift groups were lower than those in control group(p<0.05), however, $Cl^-$ concentration at the begining of work in day shift, and the end of work in day and evening shift were higher than those in control group(p<0.05). $Cl^-$ concentration at the begining and end of work in night shift were considerably higher than those in control group repectively(p<0.1, p<001). $Na^+$ concentration showed a higher tendency in three shift groups except at the begining of work in night shift, but there were no statistical difference. In comparing concentration of the 17-KS, $Na^+$ and $Cl^-$ among the shift groups, 17-KS concentration showed a lower tendency and $Na^+,\;Cl^-$ showed a higher tendency in night shift: The result of this study showes that biorhythm of shift work nurse was irregular. Fatigue level as the subjective index for evaluating the health problem concerning shift work was higher in night shift and proved to be in accordance with the concentration of urinary 17-KS, $Na^+$ and $Cl^-$ used as objective indices. Disturbation of biorhythm and work stress due to night shift seems to cause the health problem of nurses and decrease of work efficiency. It is considered that work regualtion is necessary for the rational management of the nursing administration.
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