• Title/Summary/Keyword: Neural tube defect

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Intraoperative Neurophysiology Monitoring for Spinal Dysraphism

  • Kim, Keewon
    • Journal of Korean Neurosurgical Society
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    • v.64 no.2
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    • pp.143-150
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    • 2021
  • Spinal dysraphism often causes neurological impairment from direct involvement of lesions or from cord tethering. The conus medullaris and lumbosacral roots are most vulnerable. Surgical intervention such as untethering surgery is indicated to minimize or prevent further neurological deficits. Because untethering surgery itself imposes risk of neural injury, intraoperative neurophysiological monitoring (IONM) is indicated to help surgeons to be guided during surgery and to improve functional outcome. Monitoring of electromyography (EMG), motor evoked potential, and bulbocavernosus reflex (BCR) is essential modalities in IONM for untethering. Sensory evoked potential can be also employed to further interpretation. In specific, free-running EMG and triggered EMG is of most utility to identify lumbosacral roots within the field of surgery and filum terminale or non-functioning cord can be also confirmed by absence of responses at higher intensity of stimulation. The sacral nervous system should be vigilantly monitored as pathophysiology of tethered cord syndrome affects the sacral function most and earliest. BCR monitoring can be readily applicable for sacral monitoring and has been shown to be useful for prediction of postoperative sacral dysfunction. Further research is guaranteed because current IONM methodology in spinal dysraphism is still deficient of quantitative and objective evaluation and fails to directly measure the sacral autonomic nervous system.

Spinal Dysraphism in the Last Two Decades : What I Have Seen during the Era of Dynamic Advancement

  • Wang, Kyu-Chang
    • Journal of Korean Neurosurgical Society
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    • v.63 no.3
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    • pp.272-278
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    • 2020
  • Compared to any other decade, the last two decades have been the most dynamic period in terms of advances in the knowledge on spinal dysraphism. Among the several factors of rapid advancement, such as embryology during secondary neurulation and intraoperative neurophysiological monitoring, there is no doubt that Professor Dachling Pang stood high amidst the period. I review here the last two decades from my personal point of view on what has been achieved in the field of spinal dysraphism, focusing on occult tethered cord syndrome, lumbosacral lipomatous malformation, terminal myelocystocele, retained medullary cord, limited dorsal myeloschisis and junctional neural tube defect. There are still many issues to revise, add and extend. Profound knowledge of basic science is critical, as well as refined clinical analysis. I expect that young scholars who follow the footsteps of precedent giants will shed bright light on this topic in the future.

The recent trend of prenatal screening (산전 검진의 최신 지견)

  • Hwang, Do-Yeong
    • Journal of Genetic Medicine
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    • v.5 no.1
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    • pp.7-14
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    • 2008
  • Twenty years have passed since a prenatal screening for Down syndrome and neural tube defect was applied to obstetric field. The Quad test (AFP, hCG, uE3, Inhibin-A) of the second trimester and the combination test (PAPP-A, hCG, NT) of the first trimester became popular now. The recent trend of prenatal screening is to combine these two screening tests together in order to increase a detection rate of Down syndrome. Three types of screening methods are introduced as follows; integrated test, sequential test and contingent test. In addition to combination of each test, an incorporation of characteristic ultrasound findings of Down syndrome is suggested for its risk calculation. The absence of fetal nasal bone would be a very useful marker especially in the first trimester screening test. According to a change of way calculating risk of Down syndrome, obstetrician's role will be more increased not by passive participation, but by active participation using ultrasound in risk calculation.

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Bilateral Fasciocutaneous Sliding V-Y Advancement Flap for Meningomyelocele Defect (척수수막류 결손 재건을 위한 양측 V-Y 전진피판술)

  • Shin, Jong-Weon;Oh, Deuk-Young;Lee, Jung-Ho;Moon, Suk-Ho;Seo, Je-Won;Rhie, Jong-Won;Ahn, Sang-Tae
    • Archives of Plastic Surgery
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    • v.37 no.6
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    • pp.823-826
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    • 2010
  • Purpose: Meningomyelocele is the most common type of neural tube defect disease. Early surgical treatment is recommended to prevent central nervous system infection. Several reconstruction methods were reported previously regarding surgical wound defect closure following meningomyelocele excision. In this article, we report two successful patients using the bilateral fasciocutaneous sliding V-Y advancement flap as a covering for meningomyelocele defects. Methods: Two patients with meningomyelocele were evaluated. Both patients were male and received their operations on the 1st and 4th day of life. After neurosurgeons completed their part of the operation, the V-Y advancement flap was used to close the defect. Initially a bilateral V-shape incision design was made on the skin such that the base of the V-flap measures identical to the size of the wound defect. An incision was made down to the fascia in order to allow the V-flaps to slide into the defect. Subfascial dissection was performed up to 1/3 to 1/4 the length of the V-flap from the wound while minimizing injury to the perforating vessels. Results: Both patients were treated successfully and there was no evidence of complication in 2 months follow up. Conclusion: Several reconstruction methods such as local flaps, skin graft and myocutaneous flaps were reported regarding meningomyelocele surgical wound defect closure. Bilateral fasciocutaneous sliding V-Y advancement flap is an easy method without involving the underlying muscles or a secondary skin graft in a short operation time. Therefore we recommend this treatment option for reconstruction of the wound defect following meningomyelocele excision.

Normal and Abnormal Development of the Heart (심장의 정상 및 이상발생)

  • Seo, Jeong-Uk;Choe, Jeong-Yeon;Seo, Gyeong-Pil;Ji, Je-Geun
    • Journal of Chest Surgery
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    • v.29 no.2
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    • pp.136-146
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    • 1996
  • Studies on normal human embryos and on malformed human hearts have been two main sources of the information on the developmental cardiology, Recent advances in the biological technology has opened a new era and descriptive embryology is being shifted into dynamic developmental biology. In this review, we discuss the current understanding on the cardiac embryology relevant to clinical practices of pediatric cardiology. Classical cardiac embryology starts with understanding on five segments of a straight heart tube : the sinus venosus, the primitive atria, the embryonic left ventricle, the embryonic right ventricle and the truncus arteriosus. Key steps in the normal morphogenetic process are the complex spiral septation of ventriculoarterial junction and two jumping connections : between the embryonic right atrium and embryonic right ventricle, and between the embryonic left ventricle and the aorta. Only after these two steps are successfully completed, the third fetal stage tak s place, when myocardial growth and remodeling take place There are two outstanding progresses on the cardiac embryology during recent five-year period. One is immunohistochemical mapping of the conduction system in the developing heart and the other is the understanding on the neural crest cell migration followed by molecular detection of the microdeletion of chromosome 22. A balanced progress of classical morphological studies, modern biological technics and advanced clinical medicine is an urgent task for doctors and scientists dealing with children with sick hearts.

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Controlled release of nerve growth factor from heparin-conjugated fibrin gel within the nerve growth factor-delivering implant

  • Lee, Jin-Yong;Kim, Soung-Min;Kim, Myung-Jin;Lee, Jong-Ho
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.40 no.1
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    • pp.3-10
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    • 2014
  • Objectives: Although nerve growth factor (NGF) could promote the functional regeneration of an injured peripheral nerve, it is very difficult for NGF to sustain the therapeutic dose in the defect due to its short half-life. In this study, we loaded the NGF-bound heparin-conjugated fibrin (HCF) gel in the NGF-delivering implants and analyzed the time-dependent release of NGF and its bioactivity to evaluate the clinical effectiveness. Materials and Methods: NGF solution was made of 1.0 mg of NGF and 1.0 mL of phosphate buffered saline (PBS). Experimental group A consisted of three implants, in which $0.25{\mu}L$ of NGF solution, $0.75{\mu}L$ of HCF, $1.0{\mu}L$ of fibrinogen and $2.0{\mu}L$ of thrombin was injected via apex hole with micropipette and gelated, were put into the centrifuge tube. Three implants of experimental group B were prepared with the mixture of $0.5{\mu}L$ of NGF solution, $0.5{\mu}L$ HCF, $1.0{\mu}L$ of fibrinogen and $2.0{\mu}L$ of thrombin. These six centrifuge tubes were filled with 1.0 mL of PBS and stirred in the water-filled beaker at 50 rpm. At 1, 3, 5, 7, 10, and 14 days, 1.0 mL of solution in each tubes was collected and preserved at $-20^{\circ}C$ with adding same amount of fresh PBS. Enzyme-linked immunosorbent assay (ELISA) was done to determine in vitro release profile of NGF and its bioactivity was evaluated with neural differentiation of pheochromocytoma (PC12) cells. Results: The average concentration of released NGF in the group A and B increased for the first 5 days and then gradually decreased. Almost all of NGF was released during 10 days. Released NGF from two groups could promote neural differentiation and neurite outgrowth of PC12 cells and these bioactivity was maintained over 14 days. Conclusion: Controlled release system using NGF-HCF gel via NGF-delivering implant could be an another vehicle of delivering NGF to promote the nerve regeneration of dental implant related nerve damage.

The Study of Spontaneous Developmental Abnormalities and Toxicology of Benomyl and Its Metabolite on Salamander, Hynobius leechii.

  • Park, Yong-Uk
    • Proceedings of the Korea Society of Environmental Biology Conference
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    • 2005.12a
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    • pp.38-45
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    • 2005
  • The egg bags of Korean salamander(Hynobius leechii) were collected from farmlands in Gyeongsangnam-do area. The assumed breeding time, numerical variation of embryos in each egg bag, mortality and the rates of abnormalities were investigated. The toxicity of benomyl, the metabolite carbendazim and BIC which were frequently spread in agricultural area and caused spontaneous embryonic malformation was investigated. The assumed breeding time between the end of February and the end of March has the difference about a month because of a habitat and it takes about 2 or 3 weeks from laying eggs to hatching. The length of each egg bag and the number of embryos were very varied in each area. It is due to geographical variation. Among egg bags in total study area, only 406 of egg bags(17.70% of total egg bags) developed all of embryos to normal larvae, and 78.49% of total embryos were normally developed. The patterns of spontaneous embryonic malformation were 26 species from A to Z and the abnormal patterns in individual were 8 species and above. the geographical differences about the abnormal pattern were identified and 11 habitats categorized 4 groups. The most frequent abnormality in Gyeongsangnam-do area is the dysplasia of external gill. The caudal dysplasia, abdominal blister and dysplasia of fin were also frequently observed. Individuals showing severe external defect were histologically studied and they showed retinal hypo-pigmentation, thyroid carcinoma, somatic muscular dysplasia, degeneration of cephalic neuron and various organ dysplasia. Benomyl and carbendazim were treated by 10pM$^{\sim}$10uM and BIC was treated by 1$^{\sim}$40ppm to know the effect of toxicity about toxic substance of salamander. After benomyl was treated, a survival rate was sharply dropped from 2 to 8 days. $LC_{100}$ identified in $1{\mu}M$, $LC_{50}$ identified between 100nM and $1{\mu}M$. $EC_{50}$ was assumed between 10nM and 100nM. The prevalent external malformation was abdomen swelled abnormally and histo-pathological effects were abdomen, neural tube and lens hernia. This suggests that benomyl is the toxicitic substance which inhibits the development of digestive system and nervous system. The result of treated carbendazim was similar to that of the treated benomyl. The survival rate is sharply dropped between 2 and 6 days. $LC_{100}$ was identified $1{\mu}M$ and $LC_{50}$ was identified between 10nM and 100nM. This shows that cabendazim has stronger lethal toxicity than benomyl. Ventral blister, eye dysplasia and cephalic dysplasia in the individual of external malformation mean that cabendazim affected nervous system much more than benomyl. Because the toxicity of BIC affected less in the beginning but affected more in the near hatching period, the period causing toxicity is somewhat different. $LC_{100}$ identified near 40ppm and $LC_{50}$ identified near 25ppm. The external defect shows mainly ventral blister and histo-pathological results show intestinal deformities. This result suggests the BIC inhibited strongly the development of digestive system. These abnormal developments may be caused by antimitotic action, inhibition of tubulin complex, destruction of microtubule, inhibitions of neurulation and closing of neural fold, and by the inhibition of movement of neural crest cells of benomyl. These abnormal developments may be caused by the rupture of epithelium, the loss of microtubule, the reduction of spindle size, the inhibition of spindle assembly formation, the destruction of spindle poles of carbendazim. These abnormal developments may be caused cytotoxicity by inhibition of the synthesis of a number of macromolecules and similar reaction the inhibition of benomyl.

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Methylenetetrahydrofolate Reductase C677T Polymorphism in Gastric Cancer (위암에서 Methylenetetrahydrofolate Reductase C677T의 유전자 다형성)

  • Seo Won;Park Won Cheol;Lee Jeong Kyun;Kim Jeong Jung
    • Journal of Gastric Cancer
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    • v.5 no.1
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    • pp.10-15
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    • 2005
  • Purpose: Recently the role of vitamins, folate in particular, has been emphasized in the maintenance of health. Folate deficiency is known to give rise to developmental delay, immature vascular disease, neural tube defect, acute leukemia, atherosclerotic vascular disease, delivery defects, breast cancer, and particularly gastrointestinal neoplasia. Methylenetetrahydrofolate reductase (MTHFR) is an essential enzyme in folate metaboism, and influences DNA synthesis and DNA methylation. Generally, folate deficiency is associated with gastrointestinal neoplasms. The amino-acid- changing and enzyme-activity-reducing nucleotide polymorphism (766C$\rightarrow$T/ Ala222Val) has been described in the MTHFR polymorphism and leads to low enzyme activity that may reduce the capacity of DNA methylation and possibly uracil mis-incorporation into DNA. These processes may be critical in the oncogenic transformation of human cells, especially in colorectal carcinomas. We investigated the relationship between the MTHFR polymorphism in gastric cancer and the tumor site, the smoking history, and the alcoholic history. Materials and Methods: Ninety-six (96) individuals with gastric cancer and 287 healthy persons were analyzed. Blood sampling was performed, PCR-RFLP was analyzed, and MTHFR polymorphism genotypes of C/C, C/T, and T/T were obtained and analyzed statistically for their correlation. Results: In the gastric cancer group there were 69 ($72\%$) males and 27 ($28\%$) females. There were also 58 cases ($60\%$) involving the gastric lower body, 20 cases ($21\%$) the gastric mid-body, and 18 cases ($19\%$) the gastric upper body. In the control group there were 169 ($59\%$) males and 118 ($41\%$) females. Among the gastric cancer, 56 ($61\%$) smoking patients, 40 ($39\%$) non-smoking patients, 45($47\%$) alcoholic patients, 51 ($53\%$) non-alcoholic patients. In the gastric cancer group, MTHER polymorphisms were C/C in 18 ($19\%$) cases, C/T in 59 ($61\%$) cases, T/T in 19 ($20\%$) cases. In the control group polymorphisms were C/C 116 ($40\%$) cases, C/T 103 ($36\%$) cases, and T/T 68 ($24\%$) cases (P=0.045). In cases of lower gastric body cancer, polymorphisms were C/C in 16 ($24\%$) C/C in 16 ($24\%$) cases and C/T or T/T in 42 ($72\%$) cases. In cases of upper and mid-body cancer, polymorphisms were C/C in 2 ($5\%$) cases and C/T or T/T 36 ($95\%$) cases (P=0.006). In the non-smoking patient group, polymorphisms were C/C in 5 (12%) cases and C/T or T/T in 35 ($88\%$) cases. In the smoking patient group, C/C in 13 ($23\%$) cases and C/T or T/T in 43 ($77\%$) cases (P=0.189). In the non-alcoholic patient group, polymorphisms were C/C in 6 ($12\%$) cases and C/T or T/T in 45 ($88\%$) cases. In the alcoholic patient group, polymorphisms were C/C in 12 ($26\%$) cases and C/T or T/T in 33 ($74\%$) cases (P=0.063) Conclusion: MTHFR polymorphisms are associated with gastric cancer and tumor site, but not with smoking and alcohol drinking.

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