• Clinical and Experimental Pediatrics
• /
• v.45 no.7
• /
• pp.828-835
• /
• 2002

Purpose : The outcomes of infants weighing less than 1,500 gm(very low birth weight infant : VLBWI) reflect recent progress in neonatal intensive care. In this study, we analyzed changes over time in survival rate and morbidity of VLBWIs during the past seven years. Methods : A retrospective review of medical records was analyzed for VLBWIs admitted to the neonatal intensive care unit of Samsung Medical Center within three days from birth. We compared the outcomes of previous corresponding data(period I : Oct. 1994 to Sept. 1996), with the outcomes of period II(Oct. 1996 to Dec. 1998) and period III(Jan. 1999 to Dec. 2000). Results : As shown in Tables 1 and 3, the distribution of birth weight, gestational age(GA), gender, and inborn admissions did not change during the 7-year study. The overall survival rate of VLBWI increased significantly over time(period I : 72% vs period III : 88.3%, P<0.05). Between period I and period II, the birth weight-specific survival rate increased by 23.6%(75% vs 92.7%, P<0.05) for infants 1,000 to 1,249 gm. Between period II and period III, the birth weight-specific survival rate increased three times(20% vs 66.7%, P<0.05) for infants <750 gm. The survivors of lowest birth weight included infants at 624 gm(GA : $26^{+5}$ weeks), 667 gm(GA : $25^{+6}$ weeks) and 480 gm(GA : $26^{+2}$ weeks) in each period. The gestational age-specific survival rate in period III increased significantly in GA 25-26 weeks and 29-30 weeks(vs period I & period II, P<0.05). The survivors of lowest gestational age included infants at GA 26 weeks(970 gm), GA $23^{+5}$ weeks(791 gm) and GA $24^{+1}$ weeks(740 gm) in each period. The incidence of severe IVH(grade III, IV) and the early death rate(< seven days) decreased in period III(vs period I; P<0.05), reflecting improvements in neonatal intensive care. Conclusion : The survival rate of VLBWI continues to increase, particularly for BW < 750 gm, GA < 26 weeks. This increase in survival is not associated by any increase in major morbidities.

• Neonatal Medicine
• /
• v.16 no.1
• /
• pp.55-63
• /
• 2009

Purpose: This study was conducted to investigate the incidence and clinical manifestations of rotaviral infections in the neonatal intensive care unit (NICU). We also investigated whether neonates referred from other hospitals cause outbreaks of rotaviral infections in the NICU. Methods: Neonates diagnosed wit rotaviral infections in the NICU at Korea University Ansan Hospital between January 2002 and December 2006 were evaluated retrospectively. Results: Of 1,501 neonates admitted to the NICU, the number of rotaviral infections was 94 (6.3%) and the prevalence was 4.9 cases per 1,000-hospital days. Of 223 neonates referred from other hospitals to our NICU, 24 (10.8%) were confirmed to have rotaviral infections at the time of referral. Common symptoms of rotaviral infections were jaundice diarrhea or loose stools, vomiting, fever, apnea, irritability, seizures, and moaning sounds. Necrotizing enterocolitis of stage II or more was diagnosed in 3 premature neonates, accounting for 3.2% of total infected neonates and 6.8% of premature infected patients. There were no statistically significant differences in the prevalence of symptoms between term and preterm neonates, except for jaundice that is more frequent in premature infants. In time series analysis, the admission of neonates who were born at other hospitals and diagnosed with rotaviral infections were associated 1 month later by increased cases of neonates with rotaviral infections who were born at our hospital, whereas new cases of rotaviral infections among inborn neonates were decreased 2 and 4 months later (P<0.05). Conclusion: Rotaviral infections in NICU were not rare and showed vague symptoms. Three cases (3.2%) were diagnosed with stage II or more necrotizing enterocolitis. The referred neonates with rotaviral infections may induce outbreaks in the NICU, therefore awareness and strict surveillance for rotaviral infections should be performed at the time of referral.

• Journal of Korean Medical classics
• /
• v.13 no.2 s.17
• /
• pp.174-188
• /
• 2000

The meaning of Tiangui(天癸) in $Suwen{\cdot}Shanggutianzhenlun\;素問{\cdot}上古天眞論$ is very important because it has the cause of 'having a child' and it shows the signs to being healthy. But until now there have been many arguments about what the correct meaning of Tiangui is. The most important thing in these arguments is to compare and analyze Neijing(內經) commentators' views, and to understand differences between their views. So I compared and analyzed by focusing on Neijing(內經) text, the other books with explanatory notes, and other commentators' views, and, after that. I got the following results. 1. On the meaning of Tian(天), Wangbing(王氷) and Mashi(馬蒔) considered that it is inborn. Zhangjiecong(張志聽) considered it as the Yang(陽) producing Yin(陰) Zhangjiebin(張介賓) regarded it as the Yang(陽) of Gua symbol. On the meaning of the Gui(癸). Zhangjiebin(張介賓) said that it is Yin qi(陰氣) which is the prior step to Xing(形). Mashi(馬蒔) and Zhangzhicong(張志聽) said that it is spirit or Jingxue(spiritual sanguine) which is the concrete constituents in our body. 2. On the relation bet ween Tian(天) and Gui(癸), Mashi(馬蒔) and Zhangzhicong(張志聽) said that Gui(癸) is made from Tian(天), and Zhangjiebin(張介賓) said that Tian is intrinsic in Gui(癸). 3. On the relation between Tiangui(天癸) and Jingxue(精血), Yangshangshan(楊上善). Wangbing(王氷), and Zhangjiebin(張介賓) regarded Tiangui(天癸) as a concrete matter which is the prior step to becoming Jingxue(精血). Mashi(馬蒔) and Zhangjiecong(張志聽) considered Tiangui(天癸) as a concrete matter. Mashi(馬蒔) considered Tiangui(天癸) as Jing(精)which is directly related to pregnancy. Zhangjiecong(張志聽) regarded Tiangui(天癸) as Jingxue(精血) which controls general physiology of men and women. 4. On the function of Tiangui(天癸), Yangshangshan(楊上善) and Wangbing(王氷) considered that Tiangui (天癸) has relation to menstruation. pregnancy. and, production and extinction of Jing(精). Zhangjiecong(張志聽) argued that Tiangui(天癸) strengthens and warms muscle and derma. and controls differential physiology between men and women, and said that the maintenance of its activity is based on the acquired spirit of food. A book of 『Huangti Neijing Yanjiu Dacheng(黃帝內經硏究大成)』 said that the function of Tiangui(天癸) is to promote generation, to develop the second sexual symbol, and to make growing and aging in body. It also said that Tiangui(天癸) has some relation to kidney and other organs, Chong Meridian, Ren Meridian, Du Meridian, and Dai Meridian. 5. Other commentators of 『Neijing(內經)』 accepted the meaning of Tiangui(天癸) as the prior step of both man's spiritual overflowing and woman's menstruation. 6. On the relation between Tiangui(天癸). and, Cheng and Ren Meridians, Yangshangshan(楊上善) and Zhangjiecong(張志聽) said that Tiangui(天癸) has direct relation with two meridians. Wangbing(王氷) said that Tiangui(天癸) and two meridians have no direct relation. Now I compared commentaors' views of Tiangui(天癸) and studied the differences between their views. I concluded that on the concept of Tiangui(天癸), Zhangjiebin(張介賓)'s explanations express well its connotative meaning. And on the function of Tiangui (天癸), Zhangjiecong(張志聽)'s explanations are excellent because he organized well his seniors' views, and extended its meaning by showing Neijing(內經)'s phrases related to Chong and Ren Meridians. Also, Mashi(馬蒔) suggested that if Tiangui(天癸) comes earlier than normal. people will die soon. But I think that more studies on male and female bodies are needed as to Mashi(馬蒔)'s argument.

• Clinical and Experimental Pediatrics
• /
• v.48 no.10
• /
• pp.1102-1106
• /
• 2005

Purpose : The present study examined the etiology, management, and the difference of prognosis according to methodology of treatment in severe hyperbilirubinemia with total serum bilirubin levels of more than 25 mg/dL. Methods : Medical records of severe hyperbilirubiemia in newborns(serum level=25 mg/dL) admitted to the NICU of Samsung Medical Center between October 1994 and June 2004 were reviewed retrospectively. Infants were grouped according to methodology of treatment : Group I(phototherapy only, n=42), Group II(exchange transfusion, n=6). And In addition, we evaluated the etiology and the difference of prognosis. Results : A total of 48 documented cases of severe hyperbilirubinemia were identified. Birth weight was significantly lower in Group 2($2,852{\pm}1,085g$) compared to Group 1($3,137{\pm}437g$)(P<0.05). There were no significant differences in gestational age, sex, mode of delivery, inborn, age at presentation, and age at first examination and admission between the two study groups. Maximal bilirubin level was significantly higher in Group 2($45{\pm}16mg/dL$) compared to Group 1($29{\pm}6mg/dL$) (P<0.05). But there were no significant differences in neurologic outcome. Conclusion : Our study suggests that the present guidelines for managing hyperbilirubinemia in newborns should be effective but follow-up with the first postnatal week would be necessary for each detection and treatment in the newborn infants with high risk of severe hyperbilirubinemia.

• Clinical and Experimental Pediatrics
• /
• v.52 no.10
• /
• pp.1140-1146
• /
• 2009

Purpose:The pathophysiology of magnesium, the second highest common compound in humans, is still unclear, especially in preterm babies. We accessed the association between total magnesium (tMg), ionized Mg (iMg), and gestational age (GA) and that between serum magnesium (sMg) and intraventricular hemorrhage (IVH) in preterm babies. Methods:In all, 119 inborn preterm infants admitted between July 2006 and February 2008 were divided into the IVH group (19) and the control group (100) and were prospectively analyzed. TMg, iMg, pH, total calcium (tCa), and ionized Ca (iCa) levels were determined immediately after delivery or within 3 hours after birth, and their correlation with GA were investigated. Results:TMg was not correlated with GA, tCa, iCa, and pH. IMg was correlated with tMg (r=0.288, P=0.002) and iCa (r=0.212, P=0.021); however, it was not correlated with GA and pH. Mean GA and birth weight were significantly lower (P=0.002) and smaller (P=0.030) in the IVH group. Mean sMg was higher in the IVH group ($2.5{\pm}0.9mg/dL$) than in the control group ($2.1{\pm}0.6mg/dL$) (P=0.021). SMg was a risk factor even after logistic regression analysis (OR, 2.798; 95% C.I., 1.265-6.192; P=0.011). Conclusion:In less than 37-week-old preterm babies, tMg and iMg were similar, regardless of GA. High sMg may be a risk factor for IVH in premature babies, regardless of their exposure to antenatal magnesium.

• Journal of Genetic Medicine
• /
• v.5 no.1
• /
• pp.26-33
• /
• 2008

Purpose : Phenylketonuria(PKU) is an inborn error of metabolism and a genetic disorder resulting from a deficiency of phenylalanine hydroxylase(PAH) and decreased activity of tetrahydrobiopterin(BH4).In this study the correlation between the DNA mutation and clinical manifestations was investigated and PAH DNA mutations were compared bewteen Asian and Caucasian populations. Methods : DNA was isolated from peripheral leukocytes. The PAH gene was amplified by Polymerase Chain Reaction(PCR) and the sequence was analyzed with Multiplex Ligation-dependent Probe Amplification(MLPA). Results : We characterized the PAH gene of 102 independent Korean patients with PKU. PAH nucleotide sequence analysis revealed 44 different mutations, including 10 novel mutations comprising 9 missense mutations(N207D, K95del, A447P, G344D, P69S, S391I, A202T, G103S, and I306L) and 1 novel splice-site variant mutation(IVS10-3C>G). R243Q was the most prevalent mutation in this study. A259T has not previously been reported in Asian populations, but we found that this mutation had a frequency of 10.1% in our study. Furthermore, the genotypes of $BH_4$ responsive patients were analyzed and were divided into two groups: $BH_4$ medication-only group and $BH_4$ medication with diet therapy group. In the $BH_4$ medication-only group and $BH_4$ medication with diet therapy group, R241C was the most common mutation. Conclusion : Novel mutations in the PAH gene of PKU patients are still being discovered. Additional information as to the frequency of mutations in the tetrahydrobiopterine responsive gene is also accumulating. We anticipate that knowledge of these PKU gene mutations will assist the diagnosis, genetic counseling, and therapeutic treatment of PKU patients in future.

• Clinical and Experimental Pediatrics
• /
• v.51 no.9
• /
• pp.964-970
• /
• 2008

Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.

• Cartoon and Animation Studies
• /
• s.20
• /
• pp.75-86
• /
• 2010

Recently, as the number of female players has increased rapidly, the electronic gaming industry has begun to look at ways to appeal to the largely untapped female market. According to the latest game market investigative report by China Internet Network Information Center (CNNIC), the total number of game players in China increased by 24.8% in 2009, reached 69,130,000 people, and 38.9% of them are female players. This growth in the number of female player is corroborated by a series of investigative reports from IResearch Company in Shanghai, China: from 2003 to 2009, the number of female players grew from 8% to more than 49%. Therefore, no matter how much attention the game production companies have given to male players or how they have ignored the female players before, the companies would be sensible to face up this reality and adjust their marketing policy a bit more. This article analyzes gender preferences in video games which shows that male players are more likely to be attracted to elements of aggression, violence, competition and fast action in electronic game-playing, while female players are drawn to emotional and social aspects of the games such as an understanding of character relationships. The literatures cited indicates that female players also show apparent preference for games with familiar environments, games that allow players to work together, games that have more than one way to win, and games in which characters do not die. It also discusses the characteristics of female-friendly games from the aspect of emotion, pointing out that the simulation games involving pet, dressing-up, and social simulation games are very popular with female players. Because these are the most suitable game types to fill with emotions of love, share, jealousy, superiority, mystery, these are absolutely attractive to female players. Finally, in accord with the above, I propose some principles of designing female-oriented games, including presenting a good-looking leading character, making the story interesting with "live" NPCs(Non-Playing Characters), and finding ways to satisfy female nature instincts such as taking care of others and the inborn interest of classifying and selecting.

• Journal of Life Science
• /
• v.21 no.1
• /
• pp.119-126
• /
• 2011

There is a growing recognition of the significance of $H_2S$ as a biological signaling molecule involved in vascular and nervous system functions. In mammals, two enzymes in the transsulfuration pathway, cystathionine ${\beta}$-synthase (CBS) and cystathionine ${\gamma}$-lyase (CGL), are believed to be chiefly responsible for $H_2S$ biogenesis. Genetic inborn error of CGL leads to human genetic disease, cystathioninuria, by accumulating cystathionine in the body. This disease is secondarily associated with a wide range of diseases including diabetes insipidus and Down's syndrome. Although the human CGL (hCGL) overexpression is essential for the investigation of its function, structure, reaction specificity, substrate specificity, and protein-protein interactions, there is no clear report concerning optimum overexpression conditions. In this study, we report a detailed analysis of the overexpression conditions of the hCGL using a bacterial system. Maximum overexpression was obtained in conditions of low culture temperature after inducer addition, performing low aeration during overexpression, and using a low concentration inducer (0.1 mM, IPTG) for induction. Expressed hCGL was purified by His-tag affinity column chromatography and confirmed by Western blot using hCGL antibody and enzyme activity analysis. We also report that the His tag with TEV site attached protein exhibits 76% activity for ${\alpha}-{\gamma}$ elimination reaction with L-cystathionine and 88% for ${\alpha}-{\beta}$ elimination reaction with L-cysteine compared to those of wild type hCGL, respectively. His tag with TEV site attached protein also exhibits a 420 nm absorption maximum, which is attributed to the binding cofactor, pyridoxal 5'-phosphate (PLP).

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.21 no.1
• /
• pp.15-21
• /
• 2021

Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.