• Title/Summary/Keyword: 특발성

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Multiple Mandibular Idiopathic Bone Cavities in a Patient with Immune Thrombocytopenia : A Case Report (면역성혈소판감소증 환자의 하악골에 발생한 다발성 특발성골강 : 증례보고)

  • Baek, Young-Jae;Geum, Dong-Ho;Lee, Jung-Han;Kim, Sung-Hee;Ahn, Kyung-Yong;Kim, Uk-Kyu
    • The Journal of the Korean dental association
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    • v.51 no.1
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    • pp.25-32
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    • 2013
  • The immune thrombocytopenia(ITP) is defined as a platelet count of less than $100,000/{\mu}L$. It is gene rally known as characterized by the bleeding manifestations of skin and/or mucosa like ecchymosis due to low platelet count, but reports of the related intraosseous lesions are not common. The idiopathic bone cavity(IBC) is an empty space of the bone, which occurs mainly in the long bones. It is found predominantly in the mandible in case of the maxillofacial area. In general, it appears as an isolated unilocular lesion without the correlation of the teeth. Although the cause of the IBC is supposed to be associated with hemostatic problems, the etiology is unclear and it was not disclosed the relevance of specific systemic disease. In this present case, IBCs that occurred in mandible of patient who has IPT was treated by curettage with platelet transfusion.

THE STUDY ON RELATIONSHIP BETWEEN PSYCHOPATHOLOGY AND NEUROLOGICAL FACTORS IN CHRONIC EPILEPTIC CHILDREN (경련 질환 환아의 정신병리와 신경학적 요인과의 관계에 대한 연구)

  • Kim, Bung-Nyun;Cho, Soo-Churl;Hwang, Yong-Seung
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.7 no.1
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    • pp.92-109
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    • 1996
  • The objectives of the present study were to provide comprehensive assessment of the impact of epilepsy on the psychological well-being of children with epilepsy and to identify the neurological factors associated with the psychopathology. The participant patients were recruited from the population of children and adolescent aged 7 to 16 attending the OPD of department of pediatric neurology in Seoul National University Hospital in Korea. We exclude mental retardation, pervasive developmental disorder and brain organic pathology. As control group, formal students were chosen and their sex, age, achievement, socioeconomic status were matched to patients. The first author interviewed the children and their family members and obtained the developmental history and family information. We used the following 10 scales for assessing psychological and behavioral problems in patients and their family member. The scales were standardized and their validity and reliability were confirmed before. Parent rating scales : Yale children's inventory, Disruptive behavior disorder scale, Parent's attitude to epilepsy questionnaire, Family environment scale, Symptom check-list-90 revision, Children behavior check-list. Children's self rating scales : Children's depression inventory, Spielberger's state-trait anxiety anxiety, Piers-Harris self-concept inventory and Self-administered Dependency questionnaire for Mother. The result showed the risk factors associated depression were early onset, complex partial seizure, lateralized temporal focal abnormality on EEG, Drug polypharmacy, high seizure frequency and sick factors associated anxiety were old age of patient, lateralized temporal focal abnormality EEG, Drug polypharmacy, high seizure frequency. Also the result of this present study indicated that risk factors associated oppositional defiant disorder, conduct disorder and attention deficit hyperactivity disorder were young age, male, early onset, lateral temporal EEG abnormality and high seizure frequency. According to these results, common risk factors associated psychological and behavioral problems were lateralized EEG temporal abnormality, high seizure frequency in neurological factors.

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Identification of Characteristics and Risk Factors Associated with Mortality in Hydrops Fetalis (태아수종의 특성 및 사망률과 연관된 위험인자)

  • Ko, Hoon;Lee, Byong-Sop;Kim, Ki-Soo;Won, Hye-Sung;Lee, Pil-Ryang;Shim, Jae-Yoon;Kim, Ahm;Kim, Ai-Rhan
    • Neonatal Medicine
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    • v.18 no.2
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    • pp.221-227
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    • 2011
  • Purpose: The objectives were to identify the characteristics of neonates with hydrops fetalis, and to identify the risk factors associated with mortality. Methods: A retrospective review of AMC (Asan Medical Center) dataset was performed from January 1990 to June 2009. The characteristics of 71 patients with hydrops fetalis were investigated and they were divided into two groups: the survived group and the expired group. Various perinatal and neonatal factors in two groups were compared to find out risk factors associated with mortality based on univariate analysis, followed by multiple regression analyses (SPSS version 18.0). Results: Of those 71 neonates (average gestational age: 33 weeks, birth weight: 2.6 kg), 38 survived, 33 died, resulting in overall mortality rate of 46.5%. The most common etiology was idiopathic followed by chylothorax, cardiac anomalies, twin-to-twin transfusion, meconium peritonitis, cardiac arrythmias, and congenital infections. Factors that were associated independently with mortality in logistic regression analyses were low 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days. Conclusion: In this study, 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days were significant risk factors associated with mortality in hydrops fetalis. Therefore, the risk of death among neonates with hydrops fetalis depends on the illness immediately after birth and severity of hydrops fetalis. Informations from this study may prove useful in prediction of prognosis to neonates with hydrops fetalis.

The Prognostic Value of Fibroblastic Foci Quantity in Patients with Usual Interstitial Pneumonia (UIP 환자에서 측정된 Fibroblastic Foci량이 예후에 미치는 영향)

  • Park, Sung-Woo;Lee, Young-Mok;Lee, June-Hyeuk;Kim, Ki-Up;Kim, Do-Jin;Uh, Soo-Taek;Kim, Yong-Hoon;Park, Choon-Sik;Kim, Hyun-Jo;Youm, Wook
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.6
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    • pp.540-549
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    • 2001
  • Background : Usual interstitial pneumonia (UIP) is a progressive fibrous lung disease with occasional fatal outcomes. However, the extent and rate of progression varies markedly from one patient to another. As a result, it is difficult to determine the time of the initial treatment and assess the disease activity and course. Fibroblast foci (FF) is well known to synthesize collagen actively by their myofibroblasts component. However, the prognostic value of the FF have not been evaluated in patients with VIP. Therefore this study was undertaken to determine how the number of fibroblastic foci can reflect the disease activity and progression. Methods: Twenty patients with UIP(M : F=13 : 7), who were diagnosed by a surgical lung biopsy. The number of fibroblastic foci was analyzed in terms of its correlation with the clinical manifostations, pulmonary function test, arterial blood gas analysis, and a bronchoalveolar lavage(BAL). Results : The number of fibroblastic foci did not correlate with the various lung function tests and the other clinical parameters. Interestingly, the percentage of neutrophils in the bronchoalveolar lavage fluid did correlate with the quantity of the normalized Vv of FF(r=0.60, p<0.05). The patients were divided into 2 groups, group I and II, arbitrarily, according to the value of the normalized Vv. The clinical parameters and the PIT results were not different between the two groups. In particular, the survival rate between the two groups according to the Kaplan-Meier analysis were not different. Conclusion : A large number of FF does not imply a bad prognosis in patients with UIP.

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Clinical Significance of Plasma TGF-${\beta}_1$ in Coal Workers' Pneumoconiosis (탄광부 진폐증에서 혈장 Transforming Growth Factor-${\beta}_1$의 의의)

  • Kim, Chong-Ju;Lee, Won-Yeon;Hong, Ae-Ra;Shin, Pyo-Jin;Yong, Suk-Joong;Shin, Kye-Chul
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.1
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    • pp.76-83
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    • 2001
  • Background : Coal workers' pneumoconiosis is a fibrotic lung disease resulting from chronic inhalation of coal dust. The precise mechanism of lung fibrosis in coal workers' pneumoconiosis is uncertain. However, a relationship between the stimulation of fibroblast proliferation and collagen production by mediators released from in flammatory and resident lung cells is thought to be a major factor. The transforming growth factor-$\beta$(TGF-$\beta$), a multifunctional cytokine and growth factor, plays a key role in the scarring and fibrotic processes due to its ability to induce extracellular matrix proteins and modulate the growth and immune function of many cell types. To determine the involvement of TGF-$\beta$ in the development of lung fibrosis in coal workers' pneumoconiosis, the TGF-${\beta}_1$ level in plasma was measured in patients with coal workers' pneumoconiosis. Methods : Plasma was collected from 40 patients with coal workers' pneumoconiosis (20 with simple coal workers' pneumoconiosis and 20 with complicated coal workers' pneumoconiosis) and from 10 normal controls. The ELISA method was used to measure the plasma TGF-${\beta}_1$ concentration. Results : Compared to the control group ($0.63{\pm}01.8$ ng/mL), there was no significant difference in the plasma TGF-${\beta}_1$ level in patients with simple coal workers' pneumoconiosis ($0.64{\pm}0.17$ ng/mL) (p>0.05). However, in patients with complicated coal workers' pneumoconiosis the plasma TGF-${\beta}_1$ level ($0.79{\pm}0.18$ ng/mL) was significantly higher than in patients with simple coal workers' pneumoconiosis and the control group (p<0.05). Conclusion : The data suggests that TGF-${\beta}_1$ has some influence in the development of lung fibrosis in coal workers' pneumoconiosis.

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Asymptomatic Primary Hematuria in Children (소아의 무증상성 일차성 혈뇨에 관한 고찰)

  • Lee, Jung-Mi;Park, Woo-Saeng;Ko, Cheol-Woo;Koo, Ja-Hoon;Kwak, Jung-Sik
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.25-32
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    • 2000
  • Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

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Etiology, Management, and Prognosis of Severe Hyperbilirubinemia (Serum Bilirubin Level=25 mg/dL) in Newborn (중증 고빌리루빈혈증(혈청 빌리루빈 >25 mg/dL)의 발병 원인과 치료 및 예후)

  • Hwang, Jong Hee;Lee, Ji Hyun;Kim, Yu Jin;Koo, Su Hyun;Lee, Jang Hun;Choi, Chang Won;Chang, Yun Sil;Park, Won Soon
    • Clinical and Experimental Pediatrics
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    • v.48 no.10
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    • pp.1102-1106
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    • 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.

Pulmonary Tuberculosis Mimicking Pneumonia on CT : Retrospective Analysis of Clinical and CT Features (전산화단층촬영에서 폐렴양 소견을 보였던 폐결핵: 임상 및 전산화단층촬영 소견의 후향적 고찰)

  • Paeng, Mi Hye;Kim, Yoo Kyung;Shim, Sung Shin;Chang, Jung Hyun;Lee, Jin Hwa;Kwag, Hyon Joo
    • Tuberculosis and Respiratory Diseases
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    • v.55 no.1
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    • pp.31-40
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    • 2003
  • Background : A CT scan is a useful modality for the diagnosis and evaluation of disease activity in patients with pulmonary tuberculosis. However, the CT diagnosis of pulmonary tuberculosis is sometimes difficult in patients with an atypical CT pattern, especially with lobar consolidation mimicking pneumonia. The aim of this study was to evaluate the clinical and CT features of pulmonary tuberculosis, simulating pneumonia, from a CT scan. Materials and Methods : The clinical and CT features in 21 patients, where the CT diagnosis was pneumonia, or the CT differential diagnosis included pneumonia, were retrospectively analyzed. Results : Of the 21 patients, 6 were immunocompromised, 15 presented with fever or leukocytosis and 15 showed positive sputum smear test for acid fast bacilli. Also, 17 of the 21 patients showed a positive sputum culture test. On the CT scan, consolidation was noted in all patients (100%), volume loss of the involved lobe or segment in 12 (57%), bronchogenic spread in 15 (71%), a cavity in 7 (33%) and bronchial wall thickening also in 7 (33%). The location of the consolidation revealed a relatively even distribution, with no specific predilection site. The other associated pulmonary diseases included ARDS, bronchiectasis, severe pulmonary emphysema, idiopathic pulmonary fibrosis and pulmonary alveolar proteinosis. Conclusion : In the immunocompromised patients, or patients with an underlying pulmonary disease, whose CT scans showed pulmonary consolidation, especially in association with findings of bronchogenic spread, a cavity or bronchial wall thickening, meticulous examination for pulmonary tuberculosis is recommended.

Long-term outcomes of infantile spasms (영아 연축 환아의 장기적 예후에 관한 고찰)

  • Oh, Seak Hee;Lee, Eun-Hye;Joung, Min-Hee;Yum, Mi-Sun;Ko, Tae-Sung
    • Clinical and Experimental Pediatrics
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    • v.53 no.1
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    • pp.80-84
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    • 2010
  • Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

A Follow-Up Study after Discontinuation of Antiepileptic Drug Therapy in Children with Well-Controlled Epilepsy : The Factors that Influence Recurrence (항전간제로 조절된 간질 환아에서 약물 중지 후 추적 관찰 - 재발위험인자)

  • Chung, Sa Jun;Chung, Hye Jeon;Choi, Young Mi;Cho, Eu Hyun
    • Clinical and Experimental Pediatrics
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    • v.45 no.12
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    • pp.1559-1570
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    • 2002
  • Purpose : There has been no exact answer to the question of when to discontinue antiepileptic drugs(AEDs) in children with well-controlled epilepsy for a long period. This study is about the risk factors of relapse after withdrawal of AEDs in seizure(Sz)-free patients to show a guideline for discontinuation of AEDs. Methods : One hundred and sixty-nine children were diagnosed as epileptic at the Pediatric Dept. of Kyung-Hee Univ. between 1993 to 1998, in whom AEDs had been withdrawn after at least two years of Sz-free period. Univariate analysis using Kaplan-Meier survival analysis and multivariate analysis using Cox-proportional hazard model were performed for sixteen risk factors. Results : Forty-nine of the 169 patients(28.9%) had recurrence of Szs. The mean follow-up after withdrawal of AEDs was 4.1 years, mean treatment period was 4.1 years, and the mean Sz-free period was 3.3 years. Factors associated with an increased risk of relapse were young age at onset, symptomatic Sz, Sz type in West and Lennox-Gastaut syndrome, neurologic deficit, longer Sz-controlling period, shorter total treatment period, number of AEDs used(more than one drug), age at withdrawal of AEDs, and Sz-free period less than two years in univariate analysis using Kaplan-Meier mothod. From multivariate analysis, the factors indicating a significantly higher relapse risk were pre-treatment period after first Sz attack, Sz-controlling period, Sz-free period, number of AEDs used, neurologic abnormalities. Conclusion : For epileptic children who were Sz-free for more than two years, and were more than six-years-old, the discontinuation of AEDs should be considered positively, according to age of onset, Sz type, age at withdrawal of AEDs, total treatment period, Sz-controlling period, number of AEDs used, etiology, neurologic deficit, and the wishes of the patients and the their parents.