• Title/Summary/Keyword: Pediatrics of Korean Medicine

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Oseltamivir efficacy, side effects, and safety in children with influenza (인플루엔자 바이러스 감염 소아 환아에서 Oseltamivir 약제 효용성과 단기간.장기간 부작용 및 안전성에 관한 임상연구)

  • Seo, Eun Sun;Park, Geun Hwa;Kim, Sung Mi;Kim, Sung Won;Jung, Woo Sik;Cho, Kyung Soon;Park, Yeon Gyeong;Lee, Chang Kyu;Kang, Chun;Lee, Joo Yeon;Choi, Woo Young
    • Clinical and Experimental Pediatrics
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    • v.53 no.1
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    • pp.56-66
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    • 2010
  • Purpose : Although oseltamivir is widely used for treatment of influenza, few clinical studies of its efficacy and resistance have been performed in Korea. We evaluated the safety, side effects, and efficacy of oseltamivir treatment in Korean pediatric patients. Methods : We analyzed 321 children diagnosed with influenza at Busan St. Mary's Medical Center, Korea, between January 2008 and June 2008 (first study period) and November 2008 and January 2009 (second study period). Patients were divided into two groups: those receiving oseltamivir treatment for 5 days and those receiving only symptomatic treatment. We investigated clinical symptoms, side effects, and resistance to oseltamivir. We also identified influenza strains and evaluated resistance to oseltamivir using an influenza virus culture. Results : One hundred eighty-six patients were assigned to the treatment group, and 135 were assigned to the control group. The treatment group showed shorter admission duration (4.4 days) compared with controls (5.0 days) (P =0.000) and had fewer lower respiratory tract complications compared with controls (P <0.05). No significant statistical difference in the virus antigenic type was observed between the groups. In the first study period, virus culture showed influenza B (41.7% vs. 49.6%), A/H3N2 (7.9% vs. 8.4%), and A/H1N1 (9.4% vs. 6.5%). In the second study period, only A/H1N1 (55.3% vs. 50.0%) was isolated, except for one case of A (H3N2) in the treatment group. No differences in short- and long-term side effects, including neuropsychologic side effects, were noted between groups. There was no resistance to oseltamivir before or after treatment in the first study period. Conclusion : Based on our results, we suggest that osetalmivir therapy in pediatric patients is effective.

Nutritional Assessment and Dietary Management during Anti-Cancer Chemotherapy in Pediatric Oncology Patients (항암 화학요법 중인 소아암 환자의 영양 평가 및 식이 관리)

  • Jang, Se-Ri;Kim, Hee-Sun;Lee, Young-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.2
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    • pp.204-210
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    • 1999
  • Purpose: We assessed the nutritional status and the alterations of oral diets during anti-cancer chemotherapy in pediatric oncology patients. Methods: Twenty children with malignancy were evaluated from day 0 until day 21 of post-chemotherapy. Nutritional status was assessed by body weight and biochemical parameters. The amount and calories of oral diets were assessed and food preference before and during chemotherapy were analysed by questionnelle. Results: 1) The underlying diseases of 20 patients were 11 acute lymphoblastic lekemia, 2 non-Hodgkin's lymphoma, 2 Langerhans cell histiocytosis, 2 Wilm's tumor, 2 brain tumor, 1 rhabdomyosarcoma. 2) There were weight loss during chemotherapy in 8 patients (40.0%), weigt gain in 5 patients (25.0%), and no significant changes in 7 patients (35.0%). 3) Biochemical parameters showed no significant interval changes during chemotherapy except elevation of serum ALT level. 4) The daily caloric intakes of oral diets during chemotherapy were 310~600 Kcal which was much lower than average of daily recommended calory for Korean children. 5) The most favorate food was altered by chemotherapy, from meats to carbonated beverages and unfavorate food was not altered as vegetables. Conclusion: The periodic assessment of nutritional status and dietary supplements according to preferred foods of patients will be required for the optimal nutrition care in cancer patients.

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암환자 인식에 관한 연구 - 간호사ㆍ의사를 중심으로

  • Jo, In-Hyang
    • Korean Journal of Hospice Care
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    • v.2 no.1
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    • pp.58-74
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    • 2002
  • This paper constitutes a descriptive investigation and used a structured questionnaire to investigate nurses' and doctors' recognition of cancer patients. The subjects were extracted from the medical personnel working at the internal medicine, the surgery ward, the obstetrics and gynecology department, the pediatrics department, the cancer ward, and the emergency room of five general hospitals located in Seoul and Gyeonggi Province. The research lasted from August, 2001 to September 2001. Total 137 nurses and 65 doctors were included and made out the questionnaires directly distributed by the investigator. The study tool was also developed by the investigator and consisted of such items as the demographic and social characteristics, the medical personnel's recognition degree of cancer and cancer patients, their recognition of the management of cancer patients, and their participation in a hospice. The results were analyzed using the SPSS Window program in terms of technological statistics, ranks, t-test, and ANOVA. The reliability was represented in Cronbach' α=.75. The nurses' and doctors' recognition degree of cancer and cancer patients had an overall average of 3.86 at the 5 point-scale. The items that received an average of 4.0 or more included 'Medical personnel should explain about the cancer cure plans to the cancer patient and his or her family', 'A patient whose case has been diagnosed as a terminal cancer should be notified of it, 'If I were a cancer patient, I would want to get informed of it,' and 'Cancer shall be conquered whenever it is'. In the meantime, the items that received an average of 3.0 or less was 'My relationship with the cancer patient's family has gotten worse since I announced his or her impending death.' And according to the general characteristics and the difference test, the recognition degree of cancer and cancer patient was high among the subgroups of nurses, females, married persons, who were in their 30s, who had a family member that was a cancer patient, and who received a hospice education. The biggest number of the nurses and doctors saw 'a gradual approach over several days'(68.8%) as a method to tell a cancer patient about his or her cancer diagnosis or impending death. Those who usually tell tragic news were the physician in charge(62.8%), the family members or relatives(32.1%) and the clergymen(3.8%) in the order. The greatest number of them recommended a cancer patient's home as the place where he or she should face death because they thought 'it would stabilize his or her mentality'(91.9%) while a number of them recommended the hospital because they 'should give the psychological satisfaction to the patient'(40%) or 'should try their best until the last moment of the patient's death'(30%). A majority of the medical personnel regarded 'smoking or drinking' and 'diet' as the causes of cancer. The biggest symptom of a cancer patient was 'pain' and the pain management of a cancer patient was mostly impeded by the 'excessive fear of drug addiction, tolerance to drugs and side effects of drugs' by medical personnel, the patient, and his or her family. The most frequently adopted treatment plan of a terminal cancer patient was 'to do whatever the patient or his or her family wants' to resort to a hospice' and 'to continue active treatment efforts' in the order. The biggest reasons why a terminal cancer patient went to see a doctor were 'pain alleviation' 'control of symptoms other than pain(intravenous supply)' and 'incapability of the patient's family' in the order. Terminal cancer patients placed their major concern in 'spiritual(religious) matter' 'emotional matters' their family' 'existence' and 'physical matters' in the order. 113(58.5%) of the whole medical personnel answered they 'would recommend' an alternative treatment to a terminal cancer patient mostly because they assumed it would 'stabilize the patient's mentality.' Meanwhile, 80(41.5%) of them chose 'not to recommend it mostly due to the unverified effects and high cost of it(78.7%). A majority of them, I. e. 190(94.1%) subjects said they 'would recommend' a hospice to a terminal cancer patient mostly because they thought it would help the patient to 'mentally prepare'(66.6%) Only 17.3% of them, however, had received a hospice education, most of which was done through the hospital duty education(41.4%) and volunteer training(34.5%). The follows are results of this study: 1. The nurses and the doctors turned out to be still passive and experience confusion in dealing with a cancer patient despite their great sense of responsibility for him or her. 2.Nurses and Doctors realize the need of a hospice, but an extremely small number of them participate in a hospice education or performance. Thus, a whole recognition of a hospice should be changed, for which purpose a hospice education for nurses and doctors should be provided. 3.Terminal cancer patients preferred their home to a hospital as the place to face their impending death because they felt it would bring 'mental stability.' And most of nurses and doctors think it would be unnecessary for them to be hospitalized just for control of their symptoms. Accordingly a terminal cancer patient can be cared at home, and a home hospice care needs to be activated.

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EFFECT OF NERVE GROWTH FACTOR GENE INJECTION ON THE NERVE REGENERATION IN RAT LINGUAL NERVE CRUSH-INJURY MODEL (백서 설신경 압박손상모델에서 신경성장인자 유전자 주입이 신경재생에 미치는 영향)

  • Gao, En-Feng;Chung, Hun-Jong;Ahn, Kang-Min;Kim, Soung-Min;Kim, Yun-Hee;Jahng, Jeong-Won;Lee, Jong-Ho
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.28 no.5
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    • pp.375-395
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    • 2006
  • Purpose: Lingual nerve (LN) damage may be caused by either tumor resection or injury such as wisdom tooth extraction, Although autologous nerve graft is sometimes used to repair the damaged nerve, it has the disadvantage of necessity of another operation for nerve harvesting. Moreover, the results of nerve grafting is not satisfactory. The nerve growth factor (NGF) is well-known to play a critical role in peripheral nerve regeneration and its local delivery to the injured nerve has been continuously tried to enhance nerve regeneration. However, its application has limitations like repeated administration due to short half life of 30 minutes and an in vivo delivery model must allow for direct and local delivery. The aim of this study was to construct a well-functioning $rhNGF-{\beta}$ adenovirus for the ultimate development of improved method to promote peripheral nerve regeneration with enhanced and extended secretion of hNGF from the injured nerve by injecting $rhNGF-{\beta}$ gene directly into crush-injured LN in rat model. Materials and Methods: $hNGF-{\beta}$ gene was prepared from fetal brain cDNA library and cloned into E1/E3 deleted adenoviral vector which contains green fluorescence protein (GFP) gene as a reporter. After large scale production and purification of $rhNGF-{\beta}$ adenovirus, transfection efficiency and its expression at various cells (primary cultured Schwann cells, HEK293 cells, Schwann cell lines, NIH3T3 and CRH cells) were evaluated by fluorescent microscopy, RT-PCR, ELISA, immunocytochemistry. Furthermore, the function of rhNGF-beta, which was secreted from various cells infected with $rhNGF-{\beta}$ adenovirus, was evaluated using neuritogenesis of PC-12 cells. For in vivo evaluation of efficacy of $rhNGF-{\beta}$ adenovirus, the LNs of 8-week old rats were exposed and crush-injured with a small hemostat for 10 seconds. After the injury, $rhNGF-{\beta}$ adenovirus($2{\mu}l,\;1.5{\times}10^{11}pfu$) or saline was administered into the crushed site in the experimental (n=24) and the control group (n=24), respectively. Sham operation of another group of rats (n=9) was performed without administration of either saline or adenovirus. The taste recovery and the change of fungiform papilla were studied at 1, 2, 3 and 4 weeks. Each of the 6 animals was tested with different solutions (0.1M NaCl, 0.1M sucrose, 0.01M QHCl, or 0.01M HCl) by two-bottle test paradigm and the number of papilla was counted using SEM picture of tongue dorsum. LN was explored at the same interval as taste study and evaluated electro-physiologically (peak voltage and nerve conduction velocity) and histomorphometrically (axon count, myelin thickness). Results: The recombinant adenovirus vector carrying $rhNGF-{\beta}$ was constructed and confirmed by restriction endonuclease analysis and DNA sequence analysis. GFP expression was observed in 90% of $rhNGF-{\beta}$ adenovirus infected cells compared with uninfected cells. Total mRNA isolated from $rhNGF-{\beta}$ adenovirus infected cells showed strong RT-PCR band, however uninfected or LacZ recombinant adenovirus infected cells did not. NGF quantification by ELISA showed a maximal release of $18865.4{\pm}310.9pg/ml$ NGF at the 4th day and stably continued till 14 days by $rhNGF-{\beta}$ adenovirus infected Schwann cells. PC-12 cells exposed to media with $rhNGF-{\beta}$ adenovirus infected Schwann cell revealed at the same level of neurite-extension as the commercial NGF did. $rhNGF-{\beta}$ adenovirus injected experimental groups in comparison to the control group exhibited different taste preference ratio. Salty, sweet and sour taste preference ratio were significantly different after 2 weeks from the beginning of the experiment, which were similar to the sham group, but not to the control group.

Nucleotide Sequence Analysis of the Hemagglutinin-Neuraminidase Gene of Urabe AM-9 Strain (Urabe AM-9 볼거리 백신주의 Hemagglutinin-Neuraminidase 유전자 염기서열 분석)

  • Lee, Joo Yeon;Kim, Jee Hee;Lee, Jin Soo;Park, Ji Ho;Sohn, Young Mo
    • Pediatric Infection and Vaccine
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    • v.7 no.1
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    • pp.83-93
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    • 2000
  • Purpose : Urabe AM-9 strain was known to be associated with increased aseptic meningitis. The reason for high incidence of vaccine-associated meningitis was known that nucleotide(nt) substituted form G to A at position 1081 of the hemagglutinin-neuraminidase(HN) gene and therefore, glutamic acid changed to lysine at amino acid 335. We assessed by comparing nt sequence of the HN gene form Urabe AM-9 strain with wild strain and documented the correlation between nt substitution and vaccine-associated meningitis. Methods : Two lots of Urabe AM-9 vaccine distributed in Korea and mumps wild strains isolated from 1998 through 1999 were analysed. Analysis was made by nt sequencing following amplification of HN gene by RT-PCR. Results : Nucleotide substitution at position 343, 1476, 1570 was not found in both Urabe AM-9 vaccines and wild strains. But analysis of vaccine strains and wild strains isolated from patients revealed substitution from G to A at nt 1081 of the HN gene. Therefore, it encodes lysine instead of glutamic acid at amino acid 335. There was no mixture from of G and A at nt 1081. Nt at 1470 of one lot of Urabe AM-9 vaccines changed from C to A after Vero cell passage. Nt at 1727 of vaccines and wild strains was substituted A to G, so it encodes glycine instead of aspartic acid. Conclusion : Nucleotide analysis of HN gene revealed that nt 1081 of Urabe AM-9 vaccines and wild strains had wild type AAA($Lys^{335}$) instead of variant type GAA($Glu^{335}$). The results of this study suggest that there was a probability of vaccine-associated meningitis due to Urabe AM-9 in Korea before. But incidence of actual side effect was not evaluated because there was no reporting system in Korea.

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Reduction of Allergenicity of Domestic Pork Ham and Bacon by Autoclave Treatment (가압가열 처리에 의한 시판 돈육 햄과 베이컨의 알레르겐성 저감화 효과)

  • Kim, Seo-Jin;Kim, Koth-Bong-Woo-Ri;Song, Eu-Jin;Lee, So-Young;Yoon, So-Young;Lee, So-Jeong;Lee, Chung-Jo;Kim, Kyu-Earn;Ahn, Dong-Hyun
    • Food Science of Animal Resources
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    • v.30 no.1
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    • pp.133-140
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    • 2010
  • The pork hams and bacon comprising the most popular processed pork were treated with autoclave to investigate application of hypoallergenic pork. Among pork hams and bacon, two products with the highest binding ability were selected for experiments. The results of ci-ELISA on pork hams treated with autoclave showed that the binding ability of p-IgG and pigallergic patient's sera (P2) to PSA (porcine serum albumin) from pork ham samples by autoclave treatment at $121^{\circ}C$ for 30 min was slightly decreased. The binding ability to p-IgG of b and c bacon treated with autoclave was declined to below 16% and 11% as compared with control sample that showed 60% and 91% binding ability. The binding ability to P2 of b and c bacon treated with autoclave decreased to below 22% and 34% as compared with control sample that showed 95% and 126% binding ability. A result of immunoblotting on bacon showed that p-IgG as well as pig patient's sera did not recognize PSA well in autoclave treatment. The results obtained from this work indicated that autoclave treatment was effective for a reduction of allergenicity of pork hams and bacon. Therefore the autoclave treatment may be applied to development of hypoallergenic pork.

Development of Protein-rich Food Mixtures for Infants and Growing Children in Korea -(4) Nutritional and Biochemical Evaluation of Formulas F-P-5, F-P-6, F-P-7 and storage stability of F-P-4- (유유아(乳幼兒) 및 성장기아동을 위한 영양식품 개발에 관한 연구 -(4) F-P-5, F-P-6 및 F-P-7의 영양학적, 생화학적 검토 및 그 저장성-)

  • Kwon, Tai-Wan;Cheigh, Hong-Sik;Kim, Sook-He;Lee, Hyun-Keum
    • Journal of Nutrition and Health
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    • v.3 no.3
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    • pp.129-135
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    • 1970
  • From the previous studies, F-P-4 formula was found to be comparable to full fat dry milk in its nutritive value and feeding performance. However, an attempt was made in order to make sure whether or not any possibility might exist, by which further improvement of nutritive quality and simultaneous reduction of product costs may be achieved. Using F-P-4 as a control, modifications were made in new formulas, F-P-5, F-P-6 and F-P-7 by reducing FPC, eliminating yeast from the mixture, and by enriching with methionine as needed. In particular, F-P-7 is completely free of FPC, hydrogenated oil and yeast. Yet, levels of total protein and fat were kept equal to those of F-P-4 in all formulas. An animal feeding test for all formulas using 10 female rats per group for 8 weeks and an infant feeding trial for F-P-5 and F-P-6 with 5 of each female infants under age of one for one month were conducted along with F-P-4 as a control. Almost the same results were obtained with F-P-4, 5 and 6, but F-P-7 showed the lowest body weight gain. FER of F-P-5 and 6 was 0.20 as was with F-P-4, while that of F-P-7 was 0.16. Acceptability to infants was excellent; growth, appearance and biochemical data were normal. As an example F-P-4 packed in 0.04mm polyethylene bags was used for storage study at $25^{\circ}C$ and relative humidity of $65{\sim}85%$ for 8 months. Although viable bacterial counts and vitamin C contents were reduced, peroxide and TBA values were increased gradually during such storage. Since there are also significant changes in color and organoleptic quality, the expected shelf life under the given conditions is considered to be about 2 months and thus further works are needed both on the product and packaging in order to improve the storage stability. Either elimination of yeast form F-P-4, that is F-P-5, or partial replacement of FPC with methionine, that is F-P-6 may well reduce material costs about 10%. Considering blending process of ingredients, F-P-5 is thus found to be the best formula developed. While F-P-7 free of FPC is inferior in its nutritive quality than that of others, but significantly superior than of rice. Furthermore, the material cost of the product can be reduced about 20% from that of F-P-4. And thus this vegetable blend is considered to be useful as a low cost supplementary food mixture for growing children.

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Quantitative Analysis of Small Intestinal Mucosa Using Morphometry in Cow's Milk-Sensitive Enteropathy (우유 과민성 장병증(cow's milk-sensitive enteropathy)에서 소장 생검조직의 형태학적 계측을 이용한 정량적 분석)

  • Hwang, Jin-Bok;Kim, Yong-Jin
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.1 no.1
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    • pp.45-55
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    • 1998
  • Purpose: To make objective standards of small intestinal mucosal changes in cow's milk-sensitive enteropathy (CMSE) we analyzed histological changes of endoscopic duodenal mucosa biopsy specimens from normal children and patients of CMSE. Methods: We review the medical records of patients who had been admitted and diagnosed as CMSE by means of gastrofiberscopic duodenal mucosal biopsy following cow's milk challenge and withdrawal. Thirteen babies with CMSE, ranging from 14 days to 56 days of age, were studied. Five non-CMSE patients were used as control, ranging from 22 days to 72 days of age. The morphometric parameters under study were villous height, crypt zone depth, ratio of villous height to crypt zone depth, total mucosal thickness and length of surface epithelium by using H & E stained specimens under the drawing apparatus attached microscope. In addition, the numbers of lymphocytes in the epithelium and eosinophil cells in the lamina propria and epithelium were measured. Results: In the duodenal mucosal biopsy specimens in CMSE we found partial and subtotal villous atrophy with an increased number of interepithelial lymphocytes. The mean villous height($135{\pm}59\;{\mu}m$), ratio of villous height to crypt zone depth ($0.46{\pm}0.28$), total mucosal thickness ($499{\pm}56\;{\mu}m$), length of surface epithelium of small intestinal mucosa ($889{\pm}231\;{\mu}m$) in CMSE was significantly decreased compared with the control (p<0.05). The mean crypt zone depth ($311{\pm}65\;{\mu}m$) was significantly greater than the control ($188{\pm}24\;{\mu}m$)(p<0.05). Infiltration of interepithelial lymphocytes ($34.1{\pm}10.5$) were significantly greater than the control ($13.6{\pm}3.6$)(p<0.05). The number of eosinophil cells in both lamina propria and epithelium was no significant differences between groups (p>0.05). The small intestinal mucosa in treated CMSE showed much improved enteropathy of villous height, crypt zone depth, interepithelial lymphocytes compared with the control as well as untreated CMSE. Conclusion: Quantitation of mucosal dimensions confirmed the presence of CMSE. It seems to be a limitation in the capacity of crypt cells to compensate for the loss of villous epithelium in CMSE. Specimens obtained by gastrofiberscopic duodenal mucosal biopsy were suitable for morphometric diagnosis of CMSE. Improvement of CMSE also can be confirmed histologically after the therapy of protein hydrolysate.

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The Results of Extracardiac Fontan Operation in the Patients with Heterotaxy Syndrome (이소성증후군에서의 심장외도관 폰탄 수술의 결과)

  • Lim Hong Gook;Kim Soo-Jin;Lee Chang-Ha;Kim Woong-Han;Hwang Seong Wook;Lee Cheul;Oh Sam-Sae;Baek Man-Jong;Na Chan-Young;Kim Jae Hyun;Seo Hong Joo;Jung Sung Chol;Kim Chong Whan
    • Journal of Chest Surgery
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    • v.38 no.8 s.253
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    • pp.529-537
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    • 2005
  • Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

Expression Pattern of Kir6.2 in Skeletal Muscle Cells of Patients with Familial Hypokalemic Periodic Paralysis (가족성 저칼륨성 주기성마비 환자의 골격근 세포내 Kir6.2의 발현 및 분포 양상)

  • Kim, Sung-Jo;Yoon, Dong-Ho;Kim, June-Bum
    • Journal of Life Science
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    • v.20 no.3
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    • pp.457-461
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    • 2010
  • Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant disorder characterized by reversible flaccid paralysis and intermittent hypokalemia. Although it has been reported that decreased activity in the $K_{ATP}$ channels of the skeletal muscle cell membrane plays a role in the pathogenesis of HOKPP, a clear mechanism has not yet been established. This study aimed to investigate the molecular biological mechanism underlying the decreased activity of $K_{ATP}$ channels in the skeletal muscles of familial HOKPP patients by studying the levels of the $K_{ATP}$ channel subunit Kir6.2. We found that when cells obtained from healthy individuals (normal cells) and HOKPP patients (patient cells) were treated with 4 mM potassium buffer, there was no quantitative change in the KCNJ11 mRNA levels and no difference in the Kir6.2 protein expression in the cytosol and cell membrane. On the other hand, when 1 mM potassium buffer was used, normal cells showed decreased expression of KCNJ11 mRNA as well as decreased expression of Kir6.2 protein in the cell membrane. However, patient cells treated with the same buffer showed no quantitative change in the levels of KCNJ11 mRNA or in the levels of Kir6.2 protein in the cytosol and cell membrane. Thus, in HOKPP patients, the Kir6.2 protein cannot be transported from the cell membrane to the cytosol, leading to closure of the $K_{ATP}$ channels, induction of depolarization, and subsequently, to the paralytic symptoms observed in the patient. Our findings thus provide new insights into the pathogenesis of HOKPP.