Lee, Ju Young;Shim, Jeong Ok;Yang, Hye Ran;Chang, Ju Young;Shin, Choong Ho;Ko, Jae Sung;Seo, Jeong Kee;Kim, Woo Sun;Kang, Gyeong Hoon;Song, Jeong Han;Kim, Jong Won
Clinical and Experimental Pediatrics
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v.51
no.6
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pp.650-654
/
2008
Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.
This is one case report of surgically treated partial atrioventricular canal. The 22 year-old male patient had no definitive history of frequent respiratory infection and cyanosis in his early childhood. Since his age of 7 years, dyspnea was manifested on exertion. First appearance of congestive heart failure was at his age of 16 years old. The physical examination revealed that the neck veins were distended and heaving of precordium. A thrill was palpable on the left 3rd-4th intercostal space extending from the sternal border toward the apex and Grade IV/VI systolic ejection murmur was audible on it. Neither cyanosis nor clubbing was noted. Liver was palpable about 5 finger breadths. Chest X-ray revealed increased pulmonary vascularity and severe cardiomegaly (C-T ratio = 74%). EKG revealed LAD, clockwise rotation, LVH and trifascicular block. Echocardiogram showed paradoxical ventricular septal movement, narrowed left ventricular outflow tract and abnormal diastolic movement of the anterior leaflet of mitral valve. Right heart catheterization resulted in large left to right shunt (Qp : Qs = 5.7: 1), ASD and moderate pulfllonary hypertension. Finally, left ventriculogram revealed typical goose neck appearance of left ventrlcalar outflow tract. On Oct. 10, 1980, open heart surgery was performed. Operative findings were: 1. Large primum defect ($6{\times}5$ Cm in diameter) 2. Cleft on the anterior leaflet of mitral valve. 3. The upper portion of ventricular septum was descent but no interventricular communication. 4. Downward attachment of the atrioventricular valves on the ventricular muscular septum. 5. Medium sized secumdum defect ($2{\times}1$ Cm in diameter). The cleft was repaired with 4 interrupted sutures. The primum defect was closed with Teflon patch and the secundum defect was closed with direct suture closure. Postoperatively atrial flutter-fibrillation in EKG and Grade U/VI apical systolic murmur were found. The postoperative course was uneventful and discharged on 29th postoperative day in good general conditions.
LBD-001, a recombinant human interferon $\gamma$ produced by genetically engineered yeast as a host system, was intravenously administered to pregnant female rats (Sprague-Dawley) from day 17 of gestation to day 21 of lactation at dose levels.of $0.35 \times 10^6$, $0.69 \times 10^6$, and $1.38 \times 10^6$ I.U./kg/day. In vasopressin-treated group, vasopressin (5 I.U./kg/day) was intravenously injected only for 5 days of perinatal period. (1) No signicant changes by the treatment of LBD-001 were observed in the body weights, food and water consumption, feeding and nurshing behaviors, and the weights of main organs of mother rats. In vasopressin-treated group, no significant changes were observed except the decrease in the food consumption on day 18 of gestation and one case of abnormal offspring with bleeding spots on the skin. (2) No significant changes in the body weights, survival rates, locomotor activity, emotional development. and the motor coordination of offsprings (F1) by the treatment of LBD-001 were observed except the fact that increase of ambulation in the female offsprings of LBD-001 ($0.69 \times 10^6$ or $1.38 \times 10^6$ I.U./kg/day)-treated groups and the increase of rearing in the males of LBD-($1.38 \times 10^6$ I.U./kg/day)-treated group, and the increase of the weight of liver and ovaries in the female offsprings in the LBD-001 ($1.38 \times 10^6$ I.U./kg/day)-treated group were observed. Altogether, the results show that LBD-001 at the dose of $1.38 \times 10^6$ I.U./kg/day or less does not significantly affect the mother rats and their offsprings (F1) except the minor influences when treated during the perinatal and postnatal period.
Chitosan is known to have antibacterial, antitumorogenic, hypolipidemic and immunopotentiating activities, hence finding diverse uses as a component in varying functional foodstuffs. However, some investigators reported it caused mineral absoiption inhibition and excess coagulation. From the chemical viewpoint, conventional chitosans are high-molecule polymers lacking water solubility, which could be related with their possible toxicity. A newly developed low- molecule water soluble chitosan is thought to have low toxicity compared to conventional chitosans. But no investigation was carried out to evaluate its toxicity. In this study, a 28-day subacute oral toxicity study of the water-soluble chitosan was performed in Sprague-Dawley rats of both sexes. Each 36 male and female rats were orally administered with 500, 1,000 and 2,000 mg/kg/day for 28 consecutive days, respectively. Clinical parameters (growth rate, feed and water consumption, daily inspection, urine analysis) during the 28 days indicated the water-soluble chitosan did not induce any abnonnal changes. There were no abnormal findings due to the administration of the test substance in gross and microscopic findings. We had not found alteration in absolute and relative organ weight between the control and treated groups, with only exception in the liver but lacking dose-dependency. The results of hematology and serum biochemistry examination revealed that no treatment related changes were between control and all dose groups. In conclusion, it was suggested that subacute toxicity of the water-soluble chitosan was low and the no-observed adverse effect level was considered to be over 2,000 mg/kg in rats.
Kim, Dong Kyu;Lee, Ji Min;Heo, Seon Yeong;Jung, Jong Pil;Park, Chang Ryul;Lee, Yong Jik;Lee, Sang Cjeol;Hwang, Su Kyung;Kim, Gwan Sic
Journal of Chest Surgery
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v.53
no.5
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pp.321-323
/
2020
We describe the occurrence of acute type A aortic dissection in a patient with situs inversus totalis. A 37-year-old man presented to the emergency department with acute chest pain. Initial chest X-ray findings showed a right-sided heart and a left-sided liver. Contrast-enhanced computed tomography revealed a Stanford type A acute aortic dissection, aortic root dilatation, and situs inversus totalis. All of the thoracic structures were mirror-image reversed and an abnormal coronary artery was observed. The Bentall operation was performed. This report demonstrates that computed tomography and echocardiography were useful for understanding the anatomy and the presence or absence of concurrent anomalies in a patient with situs inversus totalis. The patient's postoperative course was uneventful.
Bronchobiliary fistula (BBF), defined as an abnormal communication between the biliary duct and bronchial trees, is a very rare condition. Bilioptysis is a pathognomonic finding for BBF. We studied a 58-year-old man, who had a BBF complicated by liver biloma that occurred after radiofrequency ablation. The diagnosis was confirmed by the presence of bile-stained sputum and an Endoscopic Retrograde Cholangio-Pancreatography. BBF was treated successfully by endoscopic sphincterotomy and biliary drainage with insertion of a double pig-tail plastic stent into the biloma. We suggest that the optimal choice of treatment modality for BBF depends on the natural course of the underlying disease, and the status of the biliary stricture.
The body mass index, blood pressures and blood profiles of adults, who worked at three colleges in the Daejeon area, were evaluated for use in developing well-balanced menus at their workplace cafeterias and/or in creating nutrition education programs tailored to their health statuses. The data analyzed were from physical examinations performed in 2002 and in 2004 by the National Health Insurance Corporation. Overall, levels of systolic and diastolic blood pressure and serum cholesterol were lower in the 2004 samples than in those of 2002, but most samples in 2004 showed higher levels of SGOT. In 2002, statistical differences occured in the levels of blood pressure and serum cholesterol among age groups, but not in 2004. And in 2004, the older the age group, the higher the BMI. Among subjects in their twenties, three variables (systolic and diastolic blood pressure, and serum cholesterol) showed statistical differences; this increased to four variables (systolic and diastolic blood pressure, blood glucose and serum cholesterol) among subjects in their thirties and to six variables (Hb, SGOT, systolic and diastolic blood pressure, blood glucose and serum cholesterol) among those in their forties, followed by a decline to four variables (systolic and diastolic blood pressure, Hb and serum cholesterol) among subjects in their fifties. In general, the results of physical examinations showed improvements in blood pressure and blood profiles in 2004 in comparison with results in 2002. The level of SGOT was the exception. Despite these improvements, of those employees who had physical examinations in 2004, $5.2\%$ had high blood glucose and $18.1\%$ hypertension, $27.9\%$ hypercholesterolemia, $8.3\%$ registered abnormal liver functions, and $31.3\%$ were classified as overweight and obese. Moreover, because some vulnerable blood profiles were found in the older age groups, various programs, including nutrition education, and/or development of healthful diets as well as excercise activities, may be needed within their workplaces.
I would like to state my own opinion on arthralgia syndrome(痺病) through the literatural studies. First of all, arthralgia symdrome(痺病) must be classified into six type basically, which are migratory arthralgia(痺病(行痺)), arthritis of heat type(濕痺), arthritis due to blood stasis(瘀血痺) and deficient rheumatism(虛痺), and then could be considered to try the compound names of arthralgia syndrome. These can come from according to the rise and decline of causes in wind(風), cold(寒), damp(濕), heat(熱), blood stasis(瘀血) and qi-blood(氣血). For example, it would be possible to apply the wind-dampness rheymatism(風濕痺) of damp-heat rheumatism(濕熱痺) in terminology of arthralgia syndrome(痺病). As rheumatoid arthritis(歷節風), rheumatoid arthritis like white tiger bite (白虎歷節風) and gout (痛風) not to mean the gout in western medicine have been announced a kind of arthralgia syndromes(痺病) by many doctors since Ming dynasty(明代) and proved it to be true, it is reasonabie not to try it any longer. And tingling and deficiency of sensation(廢木 不仁) is a symptome showing the decline of muscle power including mainly the abnormal sensation of skin, it would be recommended to be classified into fliaccidity syndrome(?痺). And then the names rheumatism invoiving lendon and ligament(筋痺), rheumatism involving blood vessels(脈痺), rheumatism involving muscle(肌痺), numbness of skin (皮痺) and rheumatism involving bone(骨痺), which have been used as the classification title with the season be received bad-qi(邪氣), must be classlfied to the location appearing aymptomes. Though obstruction of the liver-qi(肝痺), obstruction of the heart-qi(心痺), stagnation of the spleen-qi(脾痺), stagnation of the lung-qi(肺痺), stagnation of the kidney-qi(腎痺) and dysfunction of the bladder(胞痺) that used visceral and bladder name, that stated a kind of arthralgia syndrome(痺病), but it must be classified into a different diseases from arthragia syndrome.
Park, Jong-Won;Shin, Dong-Hoon;Lee, Mi-Young;Suh, Suk-Kwon
Journal of Preventive Medicine and Public Health
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v.28
no.3
s.51
/
pp.543-550
/
1995
The authors conducted a cross-setional study to evaluate the differences of screening test results between clerks and laborers in an occupational health center in Taegu, 1992. A total of 10, 207 workers was included in the study. Of these, male were 6,597 and female 3,610. Constitutional variables included were items of health examination and some confounding variables(sex, age, body weight and work duration). All analyses were conducted separately for each sex through the use of multiple logistic regression analysis on occupation, controlling for age, work duration, and body weight. Laborers showed abnormal hearing test more often than clerks in both sexes. The blood pressure that showed statistical significance in univariate analysis in both sexes lost its significance after controlling covariates. Liver function and urine protein had statistically significant differences between two occupational groups in males and visual correction did in females. The results suggest that there are differences clerks and laborers in some of screening tests and analytic studies are needed to identify the causes of the differences.
In this study, bovine Dnmt1 cDNA was sequenced and detected Dnmt1 mRNA level in bovine tissues by northern blot, methylation pattern of genome by southern blot, specific localization of Dnmt1 in mouse and bovine preimplantation embryos by immunocytostaining and Dnmt1 protein level in ovary and testis by western blot. Bovine Dnmt1 cDNA sequence showed more homology with that of human than mouse and rat. The RNA level of Dnmt1 was 10 times higher expression in placenta than other tissues. This indicates that placenta was hypermethylated compared to others organs. The genomic DNA could not be cut by a specific restriction enzyme (HpaII) in placenta, lung and liver of bovine. It suggests that Dnmt1 in some somatic cells was already methylated. Dnmt1, which has the antibody epitope 1316~1616, was distributed in nucleus and cytoplasm including the stage of pronuclear stage and maturation of oocyte and gradually weaken to blastocyst stage compare to negative. In addition, Dnmt1 was strongly expressed in tetraploid embryo and cloned 8-cell than IVF 8-cell. An aberrant pattern of DNA methylation in cloned embryo may be abnormal development of fetus, embryonic lethality and placenta dysfunction. The somatic specific band (190kDa) was appeared in ovary and testis, but oocyte specific band (175kDa) was not. Further investigations are necessary to understand the complex links between the methyltransferases and the transcriptional activity of genes in the cloned bovine tissues.
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