• Title/Summary/Keyword: Young-onset

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Clinical and Pathologic Analysis of Thin Glomerular Basement Membrane Disease in Children (소아 비박형 기저막신증의 임상 및 병리학적 분석)

  • Ko Myoung Jin;Yang Tae Jin;Kim Young Ju;Chung Woo Yeong
    • Childhood Kidney Diseases
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    • v.5 no.1
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    • pp.1-8
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    • 2001
  • Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

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Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨)

  • Cho Min-Hyun;Jang You-Cheol;Kim Young-Cheol;Koo Ja-Hoon;Ko Cheol-Woo
    • Childhood Kidney Diseases
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    • v.8 no.2
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    • pp.166-175
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    • 2004
  • Purpose: Present study has been undertaken to determine the distribution of various renal diseases causing asymptomatic hematuria in children and to evaluate the benefit of doing renal biopsy in these children. Methods: Study population consisted of 146 children with asymptomatic primary hematuria who had been admitted to the pediatric departmen of Kyungpook National University Hospital for the past 4 years from 1999 to 2002. In 122 out of 146 cases, renal biopsy was performed percutaneously and in 24 out of 146 cases, diagnosed as idiopathic hypercalciuria, oral calcium loading test was performed. Results: The age$(mean{\pm}SD)$ at onset or discovery of hematuria of the 146 children in-cluded in this study was $8.0\pm3.2$ years and the proportion of boys and girls was 54.8% and 45.2%, respectively. In 76 out of 146 cases(52%), asymptomatic hematuria was first diagnosed by school urinalysis screening. The proportion of histopathologic findings based on 122 biopsies was as follows : Thin Glomerular Basement Membrane(TGBM) 73 cases(50%): IgA nephropathy 20 cases(14%): Alport syndrome 6 cases(4%), Membranous Glomerulonephropathy(MGN) 4 cases(3%): Membranoproliferative Glomerulonephritis(MPGN) 2 cases(1%); IgA nephropathy with TGBM 3 cases(2%): 'normal' glomeruli 14 cases(10%) Twenty four cases (16%) were diagnosed as idiopathic hypercalciuria. During follow-up periods, 15% of 146 cases became hematuria-free and renal function did not deteriorate in any cases. Conclusion: Unless hematuric children manifest poor prognostic indicators for renal survival, we would recommend long term regular follow-up prior to a renal biopsy.

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C-reactive Protein and Erythrocyte Sedimentation Rate Discrepancies and Variations after Intravenous Immunoglobulin Therapy in Kawasaki Disease (가와사끼병에서 C-반응 단백질과 적혈구 침강속도의 불일치 및 정맥내 면역글로불린 치료 후의 변화)

  • Lee, Yoon Suk;Lee, Jihyen;Hong, Young Mi;Sohn, Sejung
    • Pediatric Infection and Vaccine
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    • v.23 no.1
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    • pp.25-30
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    • 2016
  • Purpose: We undertook this study to investigate discrepancies in C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values, and variations following intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD). Methods: A total of 123 KD patients were retrospectively enrolled. Patients were treated with IVIG 2 g/kg at 2 to 9 days after disease onset. We obtained white blood cell (WBC) count, percentage of neutrophils (% neutrophils), CRP, ESR, and N-terminal pro-brain natriuretic peptide (NT-proBNP) values before and 48 to 72 hours after IVIG treatment. Discrepancy was defined as $CRP{\geq}10mg/dL$ and ESR <50 mm/hr (Group 1), or CRP <10 mg/dL and $ESR{\geq}50mm/hr$ (Group 2). Results: Thirty-six of 123 subjects (29.2%) had a discrepancy: 25 (20.3%) in Group 1 and 11 (8.9%) in Group 2. In Group 1, 15 patients (60%) had fever for <5 days (early presenter) and 10 (40%) had fever for ${\geq}5days$ (late presenter). There were six early presenters (55%) and five late presenters (45%) in Group 2. Late presenters had higher ESR than early presenters ($34.3{\pm}21.0mm/hr$ vs. $26.3{\pm}19.3mm/hr$, P=0.029). After IVIG treatment, elevated WBC count, % neutrophils, CRP, and NT-proBNP levels normalized. In contrast, ESR increased from $37.4{\pm}21.9mm/hr$ to $48.0{\pm}22.7mm/hr$ (n=36, P=0.051). Conclusions: A discrepancy may be related to the duration of fever. Due to discrepancies in CRP and ESR values in acute KD, both should be measured to assess the degree of inflammatory activity before IVIG treatment. After IVIG treatment, the ESR should not be used as a marker of response to therapy in KD.

Clinical and Epidemiological Study of 1,165 Hospitalized Cases of Rotaviral Gastroenteritis Before and After the Introduction of Rotavirus Vaccine, 2006-2013 (로타바이러스 백신 도입 전후 입원한 로타바이러스 위장관염 1,165례의 역학 및 임상적 연구, 2006-2013년)

  • Sohn, Tae-Young;Lee, Chan-Jae;Kim, Yoon-Joo;Kang, Min-Jae;Kim, Sung-Hye;Lee, So-Yeon;Lee, Dae-Hyoung;Lee, Hae-Ran;Kim, Kwang-Nam
    • Pediatric Infection and Vaccine
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    • v.21 no.3
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    • pp.174-180
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    • 2014
  • Purpose: This study was performed to assess the clinical and epidemiological changes after the introduction of the rotavirus vaccine in Korea, as well as to determine the efficacy of the rotavirus vaccine among hospitalized rotaviral gastroenteritis patients over the past two years. Methods: We analyzed yearly and seasonal patterns of 1,165 inpatients who were hospitalized for rotaviral gastroenteritis under the age of 5 years between 2006 and 2013. We also conducted a survey among 460 gastroenteritis patients who were hospitalized between 2012 and 2013 regarding the rotavirus vaccination and the symptoms of gastroenteritis. Among those individuals surveyed, clinical indices were analyzed for 124 patients who were tested positive for the rotavirus antigen. Results: The incidence of Rotaviral gastroenteritis have decreased significantly by year 2010. After the introduction and widespread dissemination of the rotavirus vaccine, the onset of the disease and the seasonal peak have been delayed. Overall, the vaccinated group showed a lower rate of positivity than the unvaccinated group. Among the hospitalized rotaviral gastroenteritis patients, the vaccinated group had a shorter hospitalization period, less severe clinical symptoms of gastroenteritis, and better laboratory test results. Conclusions: After introduction of the rotavirus vaccine in Korea, there were two main trends observed: 1) the overall level of disease incidence was reduced; 2) the severity of rotaviral gastroenteritis cases also decreased. Based on this data, more children should receive vaccination in order to prevent the rotavirus infection and decrease the severity of rotaviral gastroenteritis.

Video-Assisted Thoracoscopic Pleural Adhesiotomy and Decortication for Complicated Pleural Space Occupying Lesions (복잡한 흉막강내 공간차지병소의 흉강경적 흉막 유착박리술 및 박피술)

  • Jo, Min-Seop;Cho, Deog-Gon;Moon, Seok-Whan;Moon, Young-Kyu;Kang, Chul-Ung;Cho, Kyu-Do;Jo, Keon-Hyeon
    • Journal of Chest Surgery
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    • v.42 no.3
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    • pp.350-354
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    • 2009
  • Background: Complicated pleural space occupying lesions (SOL) have been treated by thoracentesis, closed thoracotomy drainage (CTD) or surgical intervention with using a video thoracosocpe or open thoracotomy depending on the extent of the disease. With the development of video assisted thoracoscopic surgery (VATS), VATS pleural adhesiolysis and decortication have revealed good results as compared to those for open thorcotomy. To assess the effectiveness of VATS pleural adhesiolysis and decortication, we retrospectively analyzed the medical record and radiologic findings of the patients with complicated pleural SOL and who were treated by this surgery. Material and Method: From May 1996 to April 2006, 64 patients (mean age: 41.8 years) with complicated pleural SOL underwent 65 VATS. To analyze the surgical outcome, we classified the postoperative findings on the simple chest X-rays into 4 classes as Class I: no or minimal pleural lesion, Class II: blunting of the cardiophrenic angle and mild pleural thickening, Class III: an elevated diaphgram or persistent lung collapse and Class IV: complicated or recurrent effusion. Result: Before VATS, the patients underwent the diagnostic or therapeutic procedures: single or repeat diagnostic tapping for 41, thoracoscotomy drainage for 11, pigtail catheter drainage for 10 and intrapleural fibrinolytics for 10. The mean duration between the onset of symptom and surgery was 18.4 days. There was neither mortality nor severe complications. The surgical outcomes were class 1 for 28, class 2 for 13, class 3 for 19 and class 4 for 5. There were statistically significant differences between the symptom duration and the classes, and between the operation time and the classes. Conclusion: VATS pleural adhesiolysis and decortication are effective, safe treatments for managing complicated pleural SOL, and an earlier operation is needed for obtaining a better surgical outcome.

Effects of Electrical Stimulation on Physicochemical and Rheological Properties of Plaice, Paralichthys olivaceus Muscle at Early Period after Death (전기자극이 넙치육의 사후조기의 물리화학적 및 물성적 변화에 미치는 영향)

  • LEE Nam-Geoul;YANG Mu-Hae;CHO Young-Je
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.28 no.1
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    • pp.23-30
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    • 1995
  • This study was undertaken to clarify the effects of electrical stimulation on physicochemical and rheological properties of the plaice (Paralichthys olivaceus) muscle at early period after death. The plaices were electrically stimulated in seawater bath (110V/60Hz) for 15sec., 35sec., and 60sec. and killed instantly with spiking at the head. Killed samples were stored at $5^{\circ}C$, and the changes in rigor index, ATP breakdown, lactate accumulation, and breaking strength of muscle through storage were investigated. Electrical stimulation effectively accelerated rigor-mortis, lactate accumulation , and ATP breakdown. As the time of electrical stimulation was lengthened, the onset of rigor-mortis of all samples were accelerated Just after killing, and the amount of lactate was rapidly increased, But, significant differences were not observed in variance of rigor-mortis and lactate concentration. Electrically stimulated plaices showed decreasing in ATP to $4.58{\mu}mole/g$ for 15sec., $4.13{\mu}mole/g$ for 35sec., and $2.39{\mu}mole/g$ for 60sec. samples as compared with $5.5{\mu}mole/g$ of unstimulated samples. As the time of electrical stimulation was lengthened, ATP in samples were decomposed more rapidly. The rate constant of ATP breakdown were $0.244hr^{-1}$ for 15sec., $0.358hr^{-1}$ for 35sec., and $0.479hr^{-1}$ for 60sec.. The level of breaking strength in muscle of the plaice was $1050.30\pm50.23g$ immediately after killing. Values of breaking strength in samples electrically stimulated for 35sec. increased rapidly just after killing among all samples. However, the breaking strength was not increased through the whole storage time in samples stimulated for 60sec.. The value and time roaching to the maximum breaking strength for each samples stimulated electrically for 15, 35 and 60 second were $1264.43\pm35.76g$ and 2hr, $1357.68\pm22.50g$ and Ohr, and $1012.18\pm57.36g$ and Ohr. Breaking strength in all samples electrically stimulated decreased significantly (P<0.05) after reaching the maximum values.

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Human Leukocyte Antigen(HLA) Genotypes and Thyroid Autoimmunity in Korean Patients with Type 1 Diabetes (한국인 제 1형 당뇨병 환자들의 HLA 유전자형 및 자가면역성 갑상선 질환의 병발 양상)

  • Kang, So Young;Shin, Chung Ho;Yang, Sei Won;Park, Myoung Hee;Yu, Jeesuk
    • Clinical and Experimental Pediatrics
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    • v.48 no.6
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    • pp.624-633
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    • 2005
  • Purpose : This study analyzed the expression of HLA-DR and DQ genotypes and anti-thyroid autoantibodies[anti-thyroid peroxidase(TPO) and anti-thyroglobulin(TG) antibodies] in Korean patients with type 1 diabetes(T1DM) to investigate the susceptible HLA alleles to T1DM in Korea and the prevalence of thyroid autoantibodies and their significance for the development of thyroid disorders. Methods : A total of 59 Korean patients with type 1 diabetes[26 males, median age 13.7 years(range 5.7-29.9 years), diabetes duration 7.6 years(-1.7-22.5 years)] were enrolled in this study, and 200 healthy Koreans without a family history of diabetes were selected as a normal control for the comparison of HLA genotypes. Seventeen patients with anti-TPO or anti-TG were followed [median duration 3.96 years(1 day-10.7 years)] with measurement of anti-TPO, anti-TG, $T_3$, $T_4$ or free $T_4$, TSH levels and physical examinations. HLA-DR and DQ genotyping were done by PCR-SSO, PCR-SSCP, PCR-RFLP and PCR-SSP methods. Results : HLA analysis showed higher frequencies of HLA-DRB1*0301, *090102 and DQB1*0201, *030302 alleles, DRB1*0301/*090102, *090102/*090102 and DQB1 *0201/*030302, *030302/*030302, *0201/ *0302 genotypes in T1DM patients compared to controls(Pc<0.05). Fifteen(25.4 percent) had anti-TPO antibody, 12(20.3 percent) had anti-TG, 17(28.8 percent) had either autoantibody and 10(16.9 percent) had both autoantibodies. No clinical or subclinical hypothyroidism developed during follow-up after the first detection of anti-thyroid autoantibody. There was no significant correlation between thyroid autoimmunity and gender, onset age of T1DM, and diabetes duration, respectively(P>0.05). Conclusion : We thought this unique HLA-DR, DQ allele distribution might be an important factor for the low incidence of T1DM in Korea. And a high prevalence of thyroid autoantibodies in these populations suggests examinations of thyroid antibodies should be performed regularly. Optimal age for the initial screening and the frequency of re-screening for associated thyroid autoimmune diseases in T1DM remains to be determined through prospective follow-up.

Infantile Hypertrophic Pyloric Stenosis - Report of 35 cases - (영아 비후성 유문 협착증 - 35례 보고 -)

  • Huh, Young-Soo;Kim, Gyu-Rag;Shin, Son-Moon
    • Journal of Yeungnam Medical Science
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    • v.13 no.2
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    • pp.199-210
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    • 1996
  • Infantile hypertrophic pyloric stenosis(IHPS), which occurs three of 1,000 live births, is a major cause of nonbilious vomiting of early infancy but its etiology and pathogenesis are still obscure. The operation of pyloromyotomy as described by Ramstedt in 1912 remains the standard of care for the treatment of IHPS. From January 1993 to October 1996, 35 infants with IHPS were surgically treated and the following results were obtained. 1. Thirty-five patients comprised 32 males and 3 females, and the ratio of male to female was 10.7:1. 2. The most prevalent age group was between 2 weeks and 8 weeks. 3. Of 35 infants, first born babies were 23 cases(65.7%). 4. Breast feeding was in 23 cases(65.7%). 5. The body weight percentile at admission was lower than 50 percentile in all 35 cases. 6. Onset of symptoms was predominantly between 1week and 2 weeks in 11 cases(31.4%). 7. All had a history of nonbilious vomting, generally projectile in nature. 8. In the measured serum electrolytes, hypokalemia was noted in 9 cases(25.7%), hypochloremia was observed in 14 cases(40.0%). 9. In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.2mm, 12.1mm, and 17.9mm, respectively. 10. Among 35 cases, significant sonographic criteria of IHPS, muscle thickness of 5mm or more, were noted in 26 cases(83.9%). 11. A total of seven associated anomalies were noted in six patients. 12. All 35 cases were treated with Fredet-Ramstedt pyloromyotomy. 13. There were postoperative complications of wound infection in 2 cases. Intermittent nonprojectile vomiting was presented in 8 cases(22.9%) after operation, but one of them was relieved in 13 days and the rest were relieved within one week by adjustment of oral intake.

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Investigating Cognitive Process and Brain Activation Study on the Rational/Emotional Advertising Appeals: Emphasis on fMRI Experiments (이성적 자극과 감성적 자극에 따른 인지처리 기능 및 재인효과 차이에 관한 연구: fMRI 분석을 중심으로)

  • Choi, Do Young;Lee, Kun Chang
    • Korean Journal of Cognitive Science
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    • v.27 no.1
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    • pp.61-99
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    • 2016
  • This research investigated that participants' response time and recognition in the decision-making situation would vary according to either rational or emotional stimuli and analyzed how brain functions are related to each type of stimuli by means of fMRI. We tried to address the difference of cognitive processing between rational stimuli and emotional stimuli in the perspective of information processing theory. In order to achieve the research purpose above, we conducted two kinds of experiment studies. In study 1, subjects conducted decision-making task which selected which kind of information type the stimuli was after stimuli - rational stimuli or emotional stimuli - was randomly seen during experiment. During this experiment, we investigated the effect of each stimuli by measuring the duration from the onset time at which stimuli was shown to the response time at which subjects conducted decision-making. Furthermore, we compared the brain functions by finding out what kinds of brain areas were activated during the decision-making task. In study 2, subjects conducted recognition task at which subjects made a decision whether the stimuli was sees in the previous experiment or not. During the second experiment, we investigated the recognition effect by measuring the memory for each stimuli type. Moreover, we compared the cognitive processes during recognition by analyzing the differences of brain area functions. The results of two experiments above were as following. Firstly, regarding the response time as the effect of stimuli, we found that the effect of emotional stimuli was higher than that of rational stimuli. And regarding the recognition as the effect of stimuli, it was found that the effect of rational stimuli was higher than that of emotional stimuli. Secondly, the explanation about the characteristics of cognitive processes with the result of behavioral response by analyzing brain functions was as following. First of all, regarding the decision-making task which conducted for analyzing the effect of response time, the relatively high activated brain areas of rational stimuli were related with the functions of movement control or working memory, and the relatively high activated brain areas of emotional stimuli were connected with the functions of lingual processing.

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Apo E4 Genotype as the Alzheimer Indictor in Korean Senior Subjects Aged 50 to 64 Years Old (남녀 노인층에서 Alzheimer위험 예측 인자로써의 Apo E4 Genotype에 관한 연구)

  • Oh, Hyun-Hee;Shin, Eun-Jung;Kim, Hyun-Sook;Lim, Yun-Sook;Park, Mi-Young;Kim, Kkoch-Byul;Kim, Eun-Mee;Lee, Myoung-Sook
    • Journal of Nutrition and Health
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    • v.40 no.7
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    • pp.593-600
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    • 2007
  • Recent studies described the ${\varepsilon}4$ allele of apoE confers a two-to fourfold increased risk for late-onset Alzheimer#s disease (LOAD), but LOAD pathology does not all fit neatly around apo E. Therefore, the goal of this study was to find the association between Alzheimer and apo E4 genotype in the 107 elderly between 50 to 64 years old who visited to FHWC of Sungshin Women#s University. We conducted the questionnaire survey (general & 24 hr dietary recall), anthropometerics (BP, waist & BMI) and blood biochemistry (FBS & lipid profiles). LDL-c and HOMA-IR were calculated by Friedwald#s and Matthew#s formulas. The apo E genotyping was performed by PCR-RFLP method and subjects were divided into three allele groups (${\varepsilon}3$; wild, ${\varepsilon}2$ & ${\varepsilon}4;$ mutants). The apo E allele frequencies were 7.0% for the ${\varepsilon}2$, 83.6% for the ${\varepsilon}3$ and 9.3% for the ${\varepsilon}4$. In comparison with biochemistry characteristics by apo E genotype, FBS was significantly higher in ${\varepsilon}4(129.2{\pm}6.8)$ than that in the others (${\varepsilon}2$: $117{\pm}7.4$, ${\varepsilon}3$: $107.3{\pm}2.2)$ (p<0.01). More than forty percents of ${\varepsilon}4$ group shown the dyslipidemia [high TG (>150mg/dl) & low HDL (<40 mg/dl:male or <50 mg/dl: female)]. The cytokines levels such as IL-1 ${\beta}$, IL-6 and $TNF-{\alpha}$ were not different among three apoE alleles. After the adjusting sex, age & dietary fiber, LDL-c level was siginificantly higher in ${\varepsilon}4$ ($108.3{\pm}7.7$) than that in ${\varepsilon}2$ ($100.4{\pm}8.4$) (p<0.05). According to food intake and the recipe on the basis of 24 hr dietary recall, the elder]y with ${\varepsilon}4$ allele took higher intake frequency of the light -colored vegetable (radish, onion & cabbage) and pan-fried foods (sauteed beef and vegetables, stir-fried vienna with vegetables) than the others. We knew that the elderly with ${\varepsilon}4$ allele had been restricted the calories intakes with high dietary fiber (33.6+2.5 g/d) to maintain the normal level of FBS and LDL-c. On next study, the prevalence of Alzheimer#s disease in this population who has ${\varepsilon}4$ allele on the condition of calories restriction will be continually follow-up.