Kim, Seok Kwun;Lee, Jang Ho;Han, Jae Jung;Jung, Ki Hwan;Lee, Keun Cheol;Park, Jung Min
Archives of Plastic Surgery
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v.32
no.1
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pp.29-36
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2005
Craniosynostosis is the premature fusion of one or more sutures of either cranial vault or base. Fused sutures may impede normal growth of the calvaria, leading to characteristic skull deformities; Morphological craniosynostosis is classified descriptively. Being craniosynostosis uncorrected the deformity progresses continuously and causes an increase of intracranial pressure. The surgical involvement aims at the expansion of intracranial space as well as satisfactory achievement of craniofacial shape. Early surgical correction in infancy prevents the deformity from the further progression and possible associated complication of high intracranial pressure. A long period of follow-up is essential to asses the outcome of an effectiveness of the surgery. measurement of intracranial volume has been concerned in medical personnel and anthropologists for many years. A reliable and accurate measurements of the intracranial volume facilitates to make a diagnosis and treatment of craniosynostosis. Pre-and postoperative change of intracranial volume was evaluated with 3D CT scanning in 12 cases of craniosynostosis who underwent frontal advancement and total cranial vault remodeling. Increased intracranial volume is attributed to surgical release of craniosynostosis and natural growth. We conceive that the intracranial volume is significantly increased after surgical correction of fused cranial sutures and along with natural growing. A procedure of frontal advancement and total cranial vault remodeling is very useful to correct such a deformity as craniosynostosis. And also 2 cases out of five mentally retarded patients improved remarkably and Forehead retrusion or temporal depression followed in another two cases.
This 3-year-old girl was observed frequent exertional dyspnea and cyanosis at crying since birth. She was not premature baby and delivered at full term normally. On physical examination, she was underdeveloped-body weight 13.5 kg, height 99 cm.- and cyanotic. There was severe clubbing on fingers. There was grade II/VI ejection systolic murmur on left lateral border of the sternum. The preoperative examinations [EKG, echocardiogram, cardiac catheterization and biventriculogram] showed that complicated T.G.A. combined vena cava[S.D.D.]. Preoperatively, we decided the corrective surgery of Rastelli operation using a. pulmonary valved conduit. The operation was performed under total circulatory arrest using deep profound hypothermia combining with extracorporeal circulation. On operation, the anatomy of the heart showed that, 1. The subaortic conus was seen and subaortic muscles were hypertrophied. 2. The VSD[type II], behind the subaortic conus-about 1 cm. in diameter, was visible only through LV cavity and, 3. The pulmonary valve ring was hypoplastic and pulmonary valvular stenosis was seen also. The subpulmonic area [LV outflow tract] was obstructed with hypertrophied muscle and mitral valve. 4. Left superior vena cava was drained to RA via coronary sinus. 5. LAD coronary artery was originated from right coronary artery and ran anterior to the pulmonary artery. According to above anatomy, we performed the VSD closure with Teflon patch, and Mustard operation combined with LV-to-pulmonary artery bypass graft using the valve contained [Hancock 16 mm] conduit. Postoperatively, adequate blood pressure could be maintained under the state of using inotropic agent [epinephrine]. On the second postoperative day, the patient died of cardiac arrest due to low cardiac output syndrome, acute renal failure and pulmonary edema.
Bak, Ung-Bok;Lim, Chang Hyeong;Cheong, Chang Kook;Hwang, Woo Suk;Cho, Myung Rae
Korean Journal of Veterinary Research
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v.20
no.1
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pp.65-78
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1980
From November 1978 to April 1979, there occurred among cows many cases of abortion, premature birth, stillbirth and congenital arthrogryposis or hydranencephaly in Kyongki-do province in Korea. Epizootics recurred between February and May, 1980 in the same area. Six calves born at 8 or 10 months of gestation were examined by means of pathology and one of the calves was examined by serological test. And a survey on local occurrence of the disease in other than Kyongki-do province, was conducted. 1. At necropsy, some calves showed distinct lesions such deformities of the limbs and the vertebrae as arthrogryposis or scoliosis. Other calves revealed arthrogryposis and partial or total deficience of the cerebral hemispheres with hydranencephaly. 2. In the calves with deformities the skeletal muscle showed histologically severe hypoplasia of the muscle fibers with cellular infiltration. In the central nervous system of the calves with hydranencephaly there were minute cystic cavitation and the decrease of ventral horn cells of the spinal cord in the number. 3. Similar epizootics were also observed in the same season in the other several provinces such as Chungoheongnam-do, Kangwon-do and Jeonrabuk-do. 4. Anti-Akabane virus antibody was detected in precolostral blood from the calf with arthroglposis and hydranencephaly syndrome. 5. On these findings of the disease it was diagnosed as Akabane disease presenting the first report on the epizootics in Korea.
We have previously shown that microRNAs (miRNAs) miR-760, miR-186, miR-337-3p, and miR-216b stimulate premature senescence through protein kinase CK2 (CK2) downregulation in human colon cancer cells. Here, we examined whether these four miRNAs are involved in the replicative senescence of human lung fibroblast IMR-90 cells. miR-760 and miR-186 were significantly upregulated in replicatively senescent IMR-90 cells, and their joint action with both miR-337-3p and miR-216b was necessary for efficient downregulation of the ${\alpha}$ subunit of CK2 ($CK2{\alpha}$) in IMR-90 cells. A mutation in any of the four miRNA-binding sequences within the $CK2{\alpha}3^{\prime}$-untranslated region (UTR) indicated that all four miRNAs should simultaneously bind to the target sites for $CK2{\alpha}$ downregulation. The four miRNAs increased senescence-associated ${\beta}$-galactosidase (SA-${\beta}$-gal) staining, p53 and $p21^{Cip1/WAF1}$ expression, and reactive oxygen species (ROS) production in proliferating IMR-90 cells. $CK2{\alpha}$ overexpression almost abolished this event. Taken together, the present results suggest that the upregulation of miR-760 and miR-186 is associated with replicative senescence in human lung fibroblast cells, and their cooperative action with miR-337-3p and miR-216b may induce replicative senescence through $CK2{\alpha}$ downregulation-dependent ROS generation.
Park, Min Ji;Lee, Dong Hun;Shin, Young Lim;Hong, Yong Hee
Journal of Genetic Medicine
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v.13
no.1
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pp.41-45
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2016
Marfan syndrome (MFS) is an inherited connective tissue disorder with a mutation in the fibrillin-1 (FBN1) gene. Fibrillin is a major building block of microfibrils, which constitute the structural component of the connective tissues. A 10-year-old girl visited our hospital with the chief complaint of precocious puberty. According to her medical history, she had a pulmonary wedge resection for a pneumothorax at 9 years of age. There was no family history of MFS. Mid parental height was 161.5 cm. The patient's height was 162 cm (>97th percentile), and her weight was 40 kg (75th-90th percentile). At the time of initial presentation, her bone age was approximately 11 years. From the ophthalmologic examination, there were no abnormal findings except myopia. There was no wrist sign. At the age of 14 years, she revisited the hospital with the chief complaint of scoliosis. Her height and weight were 170 cm and 50 kg, respectively, and she had arachnodactyly and wrist sign. We performed an echocardiograph and a test for the FBN1 gene mutation with direct sequencing of 65 coding exons, suspecting MFS. There were no cardiac abnormalities including mitral valve prolapse. A cytosine residue deletion in exon 7 (c.660delC) was detected. This is a novel mutation causing a frameshift in protein synthesis and predicted to create a premature stop codon. We report the case of a patient with MFS with a novel FBN1 gene missense mutation and a history of pneumothorax at a young age without cardiac abnormalities during her teenage years.
Journal of The Korean Society of Inherited Metabolic disease
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v.11
no.1
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pp.88-98
/
2011
Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.
We attempted to control the maturation promoting factors (MPF) activity and nuclear remodeling of somatic cell nuclear transfer (NT) bovine embryos. Bovine ear skin fibroblasts were fused to enucleated oocytes treated with either 5 mM caffeine for 2.5 h or 0.5 mM vanadate for 0.5 h and activated. The nuclear remodeling type of the reconstituted embryos was evaluated 1.5 h after activation. MPF activity was assessed in enucleated and chemical treated oocytes before the injection of a donor cell. Effect of chemicals on the embryonic development was evaluated with parthenogenetic embryos. MPF activity increased significantly by caffeine treatment, but decreased by vanadate treatment (p<0.05). Caffeine or vanadate had no deleterious effect on the parthenogenetic embryo development. In caffeine treated group, premature chromosome condensation (PCC) was occurred in 72.2% of NT embryos (p<0.05). In contrast, vanadate induced the formation of a pronucleus-like structure (PN) in a high frequency (68.9%, p<0.05) without PCC (NPCC). Blastocyst development of NT embryos increased by treating with caffeine (30.3%), whereas decreased by treating with vanadate (11.4%) compared to control (22.1%, p<0.05). The results indicate that caffeine or vanadate can control of MPF activity and remodeling type of NT embryos, resulting in the increased or decreased in vitro development.
The radical development of modern mathematics is due to the appearance of Collection Theory by George Cantor. The Set Theory is independent as an area and also closely interrelated with other areas. So its content becomes a common sense and a basic part across the whole area of modern mathematics. Accordingly, the basic element of modern mathematics is helping young children get familiar with set as early as possible. The thinking of set by which children can categorize, make partial sets and correspondences, understand the general characteristic, and conceptualize the discovered relationships is very important for young children. At this point where the Math education for young children is emphasized under the influence of the modernization movement of Math education, the systematic education for building up the set concept as the basic background of number concept during the early childhood is required. On current mathematics education for young children, graphs, the foundation of geometry, time, and patterns have been included in the traditional and practical content related to numbers. However, the education on collection which is the foundation of number concept is insufficient. A study shows that the level of young children's understanding on set is quite high, but the set concept isn't reflected in current Math curriculum for young children. And basic activities neccesary on building up the set concept, such as categorization, comparison, etc. are conducted in kindergardens but unsatisfactory because of those kindergarden teachers' premature understanding on the set concept. In conclusion, the curriculum for young children should be reorganized based on the set concept as the kernel concept. Also, the reappraisal of the training curriculum and the supplementary efucation for kindergarden teachers are urgent for raising the teaching ability of those kindergarden teachers.
Journal of The Korean Society of Grassland and Forage Science
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v.17
no.1
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pp.1-10
/
1997
Unreduced (2n) gametes are meiotic products (pollen or egg) having a sporophytic (somatic) chromosome number, resulting from abnormalities during either microsporogenesis or megasporogenesis. They occur naturally at a low frequency in many plant species. Unreduced (2n) gametes in plants can be identified for four possible ways as follow i) pollen size and/or shape differences between haploid (n) and diploid (2n) pollen, ii) ploidy analysis (chromosome number) of progeny or meiotic analysis (presence of dyads andlor triads at the microspore stage), iii) progeny performance and fertility and iv) dosage of isozyme and DNA markers. Unreduced (2n) gametes can be an effective breeding tool in synthesizing new cultivars, providing a unique method to maximizing heterozygosity, i.e., transferring a large proportion of the non-additive genetic effects (intra- and inter- locus interactions) h m parent to offspring and can also be used to overcome infertility of interploidy crosses. Sexual polyploidization through 2n gametes has been a major route to the formation of naturally occurring polyploids. The three mechanisms of 2n pollen formation in potato have been discovered as follow: i) parallel spindles (ps) or tripolar spindles (ts), ii) premature cytokinesis (pc-I, pc-2) and iii) synaptic mutants (sy-2, sy-3, sy-4). Genetic analysis indicated that the mechanisms of 2n gamete formation were controlled by single recessive gene in potato, alfalfa, red clover, etc., and by two recessive genes in wheat. The use of 2n gametes which can efficiently transfer germplasm fiom wild relatives to cultivated species, especially fiom diploid to tetraploid could make a contribution to the improvement of germplasm base in breeding programs.
Based on a literature review regarding shift work, it is recognized that it has an adverse effect on workers' health. Especially, the night shift rather than the day shift imposes severe disorders on workers, which are indicated to dyssomnia, maladaptation to social life, and health problems such as gastroenteric trouble, cardiovascular diseases and depression. As the shift work can be explainable by using workers' labor ability necessarily to maintain company business consistently, it does not consider biorhythm, active mass and health condition of workers Actually duration of shit work would deprive workers of fundamental life rights by causing physical and mental effects. As a result of reviewing previous case studies related to effect of work pattern (day shift and night shift) on workers' health, an incidence of physical diseases like dyssomnia, gastroenteric trouble, cardiovascular diseases and premature delivery was higher in shift workers than normal workers. Additionally the incidence of mental disorders such as busy brain, social isolation, depression and work stress was also higher in shift workers than normal workers. These adverse physical and mental problems were intensified to night shift workers compared to day shift workers. Considering current various reports and study results, it is recommended that the shift work, especially the night work pattern, should not apply to contemporary work situation for sustaining workers' health condition constantly.
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