• Title/Summary/Keyword: Associated features

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The Etiology and Clinical Features of Acute Osteoarthritis in Children; 2003-2009 (최근 6년간 소아청소년기 급성 화농성 골관절염의 원인균과 임상 양상; 2003-2009)

  • Choi, Jin Hyoung;Choe, Young June;Hong, Ki Bae;Lee, Jina;Yoo, Won Joon;Kim, Han Soo;Park, Moon Seok;Cho, Tae Joon;Chung, Chin Youb;Lee, Hoan Jong;Choi, In Ho;Choi, Eun Hwa
    • Pediatric Infection and Vaccine
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    • v.18 no.1
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    • pp.31-39
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    • 2011
  • Purpose : This study was performed for the purpose of finding causative organisms and clinical features of septic arthritis or acute osteomyelitis in children. Methods : The study involved a retrospective review of the medical records of 63 microbiologically confirmed cases of acute pyogenic arthritis and osteomyelitis. All of the cases were brought about by community-associated infections and managed at the Seoul National University Children's Hospital or Seoul National University Bundang Hospital from June 2003 to July 2009. Results : The median age of all cases was 60 months and there were 35 males and 28 females. Major involved joints included the hip joint (15 cases), knee joint (7 cases), shoulder joint (4 cases), and elbow joint (4 cases). Also, major involved bones included the femur (20 cases), tibia (13 cases), humerus (7 cases), and radius (7 cases). Staphylococcus aureus was the most commonly identified causative organism, accounting for 49 cases (77.8%). Of the 49 isolates of S. aureus, methicillin-resistant S. aureus (MRSA) accounted for 8 cases (16.3%). Group B streptococcus spp. (GBS) and Salmonella spp. accounted for 3 cases, respectively. Nafcillin or cefazolin was often prescribed as an initial empirical antibiotic. There were 9 cases that were managed by a regimen that included vancomycin as the first choice. Fifty four cases (85.7%) recovered without any complications. Methicillin-sensitive S. aureus (MSSA), responsible for 41 cases, caused chronic complications in 3 cases. Of 8 cases caused by MRSA, 1 case showed chronic complication. There were no fatal cases. Conclusion : S. aureus remains the most common organism causing acute pyogenic arthritis and osteomyelitis of childhood acquired in the community. The high prevalence of methicillin resistance among S. aureus should be considered carefully in the selection of initial empirical antibiotics.

Clinical and Cytogenetic Analysis of Children with Maternal Chromosomal Balanced Translocation (모체의 염색체 균형전좌를 가진 환아들의 임상적 세포 유전학적 관찰)

  • Lim, Han Hyuk;Jeong, Hee Jeong;Park, Kyung Duk;Kim, Sook Ja
    • Clinical and Experimental Pediatrics
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    • v.48 no.7
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    • pp.701-705
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    • 2005
  • Purpose : Parents' genetic information plays an important role in their children's genetic expression. Human chromosome has 23-paternal chromosomes and 23-maternal chromosomes. Parental chromosomal translocation can induce clinical problems in their children because of imbalance in genetic information. We intent to analyze the cytogenentic and clinical features about children with maternal balanced translocation between chromosome 15 and 18. Methods : We detected by one family's FISH study of chromosome 15. We have evaluated children born to clinically normal parents about peripheral bood analysis, endocrine, metabolic, radiologic study, electroencephalogram and social & intelligence scale. and We analysis their clinical manifestation by hospital records. Results : Patient's father and elder sister are normal clinically and genetically. Her mother's chromosome show balanced translocation, 46, XX, t(15;18)(p11.2;p11.3). One child has 46, XX, der(18) t(15;18)(p11.2;p11.3), mental retardation, growth retardation, speech & social developmental delay, recurrent infection and mild mitochondria dysfunction. Her young brother has 46, XY, der(15) t(15;18) (p11.2;p11.3), mental retardation, aggressive behavior, obesity and speech developmental delay. Conclusion : In this study we observed the children with developmental delay, dysmorphic facial features, mental retardation, growth retardation associated with growth hormone deficiency and aggressive behavior due to unbalanced translocation between chromosome 15 and 18.

Clinical characteristics of congenital myotonic dystrophy diagnosed by molecular genetic method (분자 유전학적 방법으로 진단된 선천성 근육긴장성 이영양증 환자의 임상 양상)

  • Nam, Sook Hyun;Son, Young Bae;Lee, Bo Lyun;Lee, Jeehun;Ki, Chang-seok;Lee, Munhyang
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.868-874
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    • 2007
  • Purpose : We performed this study to investigate the perinatal and developmental features of the patients with congenital myotonic dystrophy (CDM) confirmed by the molecular genetic method and the clinical characteristics of their mother, and to identify the relation between the number of CTG repeats and the clinical severity.Methods : A retrospective review of the medical records and the results of the dystrophia myotonica protein kinase (DMPK) gene test was done for the patients who were confirmed as CDM through gene analysis from January 2001 to September 2006. Results : All of the eight patients (male 2, female 6) showed moderate to severe degree of perinatal distress and feeding difficulty associated with profound hypotonia. Three patients had the history of polyhydramnios and two patients had equinovarus deformity. The developmental milestones were delayed in all patients, which improved gradually with age. All of their mothers demonstrated myotonic symptoms and typical myopathic face. The number of CTG repeats in DMPK gene analysis ranged 1,000-2,083, and there was no significant correlation between the number of CTG repeats and the time of walking alone. Conclusion : All patients with CDM presented with severe hypotonia in perinatal period, and developmental delay thereafter, which were improved with age. All of their mothers manifested myotonic symptoms with typical myopathic face, and the identification of such features greatly contributed to the diagnosis of the patients. The number of CTG repeats had no significant influence on the motor development.

THE CLINICAL FEATURES OF THREE SUB-DIAGNOSED GROUPS OF TIC DISORDERS AND FACTORS RELATED WITH ILLNESS SEVERITY (틱 장애의 진단분류에 따른 임상특징과 질환 심각도와 연관된 변인들)

  • Jung, Hee-Yeon;Hwang, Jeong-Min;Chung, Sun-Ju
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.12 no.1
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    • pp.115-124
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    • 2001
  • Objective:The purpose of this study was to compare the clinical features of children with three subdiagnoses of tic disorder(transient tic disorder(TTD), chronic tic disorder(CTD) and Tourette’s disorder (TD)) and to exam the factors related with illness severity of them. Method:Subjects were 69 children who met DSM-IV criteria for tic disorder and 43 control children. All subjects were investigated demographic and clinical factors related to tic. The severity of tic was assessed with the Yale Global Tic Severity Scale(YGTSS) in tic disorder children. The Child Behavior Checklist(CBCL) was accomplished by parents of all subjects. Results:Children with TTD had not only shorter duration of tic symtoms but also milder tic severity and impairment than those with CTD and TD. They also had significantly lower scores on most CBCL subscales than children with CTD and TD, while they were similar with controls in all the CBCL subcale scores except aggressive behavior. Children with CTD and those with TD were similar to each other in clinical variables except number of tic symptom and scores on CBCL social problem subscale. The interference and intensity of motor tic symptoms and duration of tic symptoms were significant predictors of global impairment score on YGTSS, while the presence of comorbid ADHD was a preictor of the total behavior problem score of CBCL. Conclusion:These findings indicate that duration of tic symptoms and the presence of comorbid ADHD as well as the severity of tic symptoms strongly associated with the illness severity of children with tic disorder. These results also suggest that those clinical factors may be more important for assessing the severity of illness and determining the treatment strategy than the sub-diagnosis itself in children with tic disorder.

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Evaluation of Patients with Hemoptysis and A Normal Chest Roentgenogram (정상 흉부 X-선 소견을 가진 객혈환자의 관찰)

  • Han, Chun-Duk;Kim, Yeon-Jae;Lee, Yeung-Suk;Park, Jae-Yong;Jung, Tae-Hoon
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.1
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    • pp.42-49
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    • 1995
  • Background: Hemoptysis always merits thorough investigation because even minimal bleeding may be an early indicator of the presence of significant bronchopulmonary disease. But in patients with hemoptysis & a normal chest roentgenogram, there are no clear guidelines for a diagnostic approach, including the indications of bronchoscopy. Methods: Eighty patients with hemoptysis and a normal chest roentgenogram were involved in this study. We evaluated the cause of hemoptysis in these patients by bronchoscopy and/or bronchogram or high-resolution CT of the lung and we analyzed the relationship of clinical features, such as age, sex, smoking and properties of hemoptysis, to the cause of hemoptysis. Results: 1) They were 34 men and 46 women, with the mean age of 46.7 and 41.8 years old, respectively. 2) Initial bronchoscopy provided a diagnosis in 8 patients - bronchogenic carcinoma in 3 patients (3.8%), metastatic cancer in 1 patient(1.3%) and endobronchial tuberculosis in 4 patients(5.0%). 3) Two clinical findings of patients over 50 years and/or with more than 30 pack-year smoking history were associated with bronchogenic carcinoma, and among these two factors, a more than 30 pack-year smoking history was the best predictor for diagnosis of bronchogenic cancer. 4) The 72 patients in whom no specific cause of hemoptysis was identified by initial bronchoscopy underwent bronchogram and/or high resolutional CT of the lung. Then, 6 patients were diagnosed as bronchiectasis and 5 patients rebleeded in the follow up period of 9 to 90 weeks. Of the remaining 66 patients, 33 were followed for 7 to 80 weeks. Among these patients, only 5 patients had recurrent episodes of hemoptysis & they were diagnosed as bronchiectasis in 1 patient, tuberculosis in 2 patients and catamenial hemoptysis in 2 patients. Conclusion: We conclude that patients with hemoptysis and a normal chest roentgenogram who are more than 50 years old or have more than 30 pack-year smoking history should undergo bronchoscopy to exclude possible bronchogenic carcinoma. In patients without these clinical features, a conservative approach with observation appears justified. If hemoptysis recurs to these patients, bronchogram or high-resolutional CT of the lung with sputum examination are necessary.

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Textural and Geochemical Characteristics and their Relation of Spinel Peridotite Xenoliths from Jeju Island (제주도 첨정석 페리도타이트 포획암의 조직 및 지화학적 특성과 그 관련성)

  • Yu, Jae-Eun;Yang, Kyoung-Hee;Kim, Jin-Seop
    • The Journal of the Petrological Society of Korea
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    • v.19 no.3
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    • pp.227-244
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    • 2010
  • Abundant spinel lherzolite xenoliths showing distinctively different textural types such as protogranular, porphyroclastic, and mylonitic texture are trapped in the basaltic rocks from southeastern part of Jeju Island. These xenoliths show the textural spectrum from coarse-grained protogranular through porphyroclastic with bimodal grain size to fine-grained and foliated mylonitic texture. They tend to decrease in grain sizes and show more linear grain boundaries and more frequent triple junctions from protogranular through porphyroclastic to mylonitic. Spinel has different occurrence mode according the textural type. Spinel is always associated with orthopyroxene in protogranular texture, whereas it is scattered and independent of orthopyroxene in mylonitic texture. Additionally, porphyroblast from porphyroclastic and mylonitic textures has internal deformation features such as kink band, undulatory extinction and curved lamella, whereas neoblast is strain-free. These textural features indicate increasing degree of static/dynamic recrystallization from protogranular through porphyroclastic to mylonitic texture. The mg#[$=100{\times}Mg/(Mg+Fe_t)$] of olivine, orthopyroxene and clinopyroxene is relatively constant (ol: 88-91; opx: 89-92; cpx: 89-92) regardless of textural differences. The mg# of constituent minerals, NiO content (0.3~0.4 wt%) and MnO content (0.1~0.2 wt%) of olivine are similar to those of mantle xenoliths worldwide, also indicating that studied spinel lherzolite xenoliths were mantle residues having experienced 20~25% partial melting. The geochemical and textural characteristics have close relations showing that LREE and incompatible trace elements content of orthopyroxene and clinopyroxene increases from protogranular through porphyroclastic to mylonitic. These observations suggest that the studied mantle xenoliths experienced metasomatism by LREE enriched melt or fluid after partial melting, indicating a close relation between deformation and metasomatism. The metasomatism was possibly confined to narrow shear zones from where porphyroclastic and mylonitic textured xenoliths originated. These shear zones might favorably drive the percolation of LREE-enriched melts/fluids responsible for the metasomatism in the lithospheric mantle below the Jeju Island.

Clinical Features of Isolated Noncompaction of the Ventricular Myocardium (심근의 단독 비경화증(Isolated Noncompaction of Ventricular Myocardium)의 임상 양상)

  • Moon, Eun-Kyoung;Lee, Hoon-Young;Chang, Mea-Young;Kil, Hong-Ryang;Chung, Yong-Hun
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1528-1533
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    • 2002
  • Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

The Role of Immunohistochemical Biomarkers as Prognostic Factors by the Use of a Tissue Microarray in Breast Cancer Patients Under 45-years-old (45세 이하의 유방암환자에서 조직미세배열법을 이용한 면역조직화학적 생체표지자의 역할)

  • Kim, Eun-Seog;Choi, Doo-Ho;Jin, So-Young;Lee, Dong-Wha;Park, Hee-Sook;Lee, Min-Hyuk;Won, Jong-Ho;Kim, Yong-Ho;Lee, Kyu-Taek;Kim, Sung-Yong
    • Radiation Oncology Journal
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    • v.26 no.1
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    • pp.45-55
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    • 2008
  • Purpose: This study evaluates the association of estrogen receptor(ER), progesterone receptor(PR), Her-2, COX-2, and survivin with the clinicopathological features and outcomes in young Korean women with breast cancer using recently developed tissue microarray(TMA) technology. Materials and Methods: A cohort of 212 young patients with breast cancer diagnosed at the age of 45 years or younger from March 1994 to August 2005, were enrolled in this study. The age range of patients was $23{\sim}45$ years(median age, 39 years). The minimum and median follow-up periods were 24 months and 60 months, respectively. Serial sections of primary tumors were processed by the use of a TMA for immunohistochemical staining for five biomarkers. The correlation of these five biomarkers and the clinicopathological features and outcomes were analyzed by statistical methods. Results: The majority of the patients were stage T1(90 patients) or T2(101 patients), and 105 patients(49.5%) had an axillary node metastasis. The 5-year overall and relapse free survival rates for all of the patients were 90.4% and 82.3%, respectively, and 36 patients had a locoregional or distant metastasis as a first event. Positive expression of ER, PR, Her-2, COX-2, and survivin was determined in 38.2%, 45.3%, 25.9%, 41.5%, and 43.4%, of the tumor samples, respectively. Tumor stage, nodal status, age, as well as expression of ER, PR, and HER-2 status were significantly associated with the disease free survival rate. Tumor stage, nodal status, as well as expression of ER, PR, and HER-2 were significantly related with the overall survival rate. Expression of COX-2 and survivin were not single independent prognostic factors for the disease free and overall survival rate although co-expression of HER-2 and COX-2 had a tendency as a poor prognostic factor. By multivariate analysis, only T stage and lymph node status were significant prognostic factors, and ER status was a marginally significant prognostic factor(p=0.075). Conclusion: Expression of ER, PR and HER-2 were significant prognostic factors for the relapse free and overall survival rate. Expression of COX-2 and survivin were not prognostic factors for young women with breast cancer.

Nonalcoholic Fatty Liver Disease in Children with Hypopituitarism (뇌하수체저하증 소아에서 발생한 비알코올성 지방간질환)

  • Yoon, Jung-Min;Ko, Jae-Sung;Seo, Jeong-Kee;Shin, Choong-Ho;Yang, Sei-Won;Moon, Jin-Soo;Yang, Hye-Ran;Chang, Ju-Young
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.13 no.1
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    • pp.51-57
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    • 2010
  • Purpose: It has been reported that children with hypopituitarism have features of metabolic syndrome, including obesity, impaired glucose tolerance, and dyslipidemia. The aim of this study was to investigate the clinical features and liver histology of pediatric non-alcoholic fatty liver disease (NAFLD) associated with hypopituitarism. Methods: We reviewed the clinical data of 11 children diagnosed with NAFLD among patients with hypopituitarism. Results: The mean age at the time of diagnosis of hypopituitarism was 10.4${\pm}$3.2 years, and the mean age at the time of diagnosis of NAFLD was 13.1${\pm}$2.7 years. A craniopharyngioma was the most common cause of pituitary dysfunction. At the time of diagnosis of NAFLD, 9 patients (82%) had a body mass index greater than the 85th percentile, 5 patients (45%) had elevated fasting blood glucose levels, and 9 patients (82%) had hypertriglyceridemia. The mean height SD score was significantly lower at the time of diagnosis of NAFLD than at the time of diagnosis of hypopituitarism. Of the six patients who were biopsied, one had cirrhosis, two had non-alcoholic steatohepatitis (NASH) with bridging fibrosis, two had NASH with mild portal fibrosis, and one had simple steatosis. Conclusion: Children with hypopituitarism are at risk of short stature, obesity, dyslipidemia, and NAFLD. The early diagnosis of NAFLD is important in children with hypopituitarism because advanced fibrosis is common.

Microsporogenesis of Hibiscus syriacus L and Its Sporoderm Differentiation (무궁화의 화분형성 및 화분벽의 분화발달)

  • 김인선
    • Journal of Plant Biology
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    • v.38 no.1
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    • pp.95-105
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    • 1995
  • Complete microsporogenesis of Hibiscus syriacus L. were carried out employing LM, TEM, and SEM to investigate the pollen ontogeny that undergoes considerable structural differentiation. The process first began with several cell diYisions in the anther primordium that produces 3 different tissues of epidennal, archesporial, and connective tissues. Only archesporial tissue involved further differentiation into the tapetum and formation of reproductive cells, pollen mother cells (PMC). The tapetum and PMC were closely associated with each other structurally and metabolically by exhibiting numerous plasmodesmata, mitochondria, and many small vacuoles in their dense cytoplasm. A callosic wall began to surround the PMC while meiosis took place in the PMC to produce 4 microspores. When thick callose encircled each microspore as a frame, the sporodenn development initiated from the plasma membrane of a pollen grain in a tetrad. The first fonned sporoderm layer was bacules and tectum of sexine that originated from the plasma membrane. After the dissolution of a callose, further development Qf sporoderm continued in the order of nexine 1, nexine 2, and intine layer. The nexine layer was thicker (ca. $2-3.5\;\mu\textrm{m}$) than the intine layer whose thickness was about $0.9-1.5\;\mu\textrm{m}$. Upon completion of the sporoderm development, that is after intine formation, spines and apertures of pollen surface ornamentation initiated from the tectum. Spines were dimorphic, about $4-9\;\mu\textrm{m}\;an;15-20\;\mu\textrm{m}$ in length, and no basal cushion was detected. The mature pollen grains ranged $100-200\;\mu\textrm{m}$ in diameter, but their average was about $170\;\mu\textrm{m}$. About 120 spines were observed over the spheroidal pollen surface. Apertures were simple punctures of $2-3\;\mu\textrm{m}$ in diameter and about 50 apertures were arranged somewhat helically over the surface. Comparing such features of form and size of the pollen, sporodenn sculpture and structure, and aperture and spine conditions with known evolutionary trends in the genus Hibiscus, Hibiscus syriacus seemed to possess many advanced features in the sporodenn differentiation.iation.

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