• Title/Summary/Keyword: 성인 간질환자

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A case of hepatopulmonary syndrome in a child with fatty liver disease secondary to hypopituitarism after craniopharyngioma resection (지방간을 가진 소아에서 두개인두종 절제술 후의 뇌하수체기능저하증으로 인해 급격하게 진행된 간-폐 증후군 1예)

  • Im, Sun Ju;Park, Hyeon Seok;Lee, Hyoung Doo;Park, Jae Hong;Park, Hee Ju
    • Clinical and Experimental Pediatrics
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    • v.50 no.8
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    • pp.794-798
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    • 2007
  • Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year-old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

A Case of Bronchiolitis Obliterans Organizing Pneumonia anteceded by Rheumatoid Arthritis (류마티스 관절염에서 동반된 폐쇄성 세기관지염.간질성 폐염 1예)

  • Kim, Sin Gon;Kim, Jin Yong;Lee, Young Ho;Cho, Jae Youn;Kim, Han Kyeom;Song, Gwan Gyu
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.4
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    • pp.630-636
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    • 1996
  • Bronchiolitis obliterans organizing pneumonia(BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It clad been described in association with several connective tissue diseases, i.e. systemic lupus erythematosus, Behcet's disease, polymyositis-dermatomyositis, polymyalgia rheumatica and Sjogren's syndrome. Recently, there were few additional reports on BOOP, anteceded by rheumatoid arthritis. We clad experienced a case of 60 years old female patient who clad been admitted for acute respiratory difficulty and abnormal chest roentgenographic findings. She was diagnosed as having rhematoid arthritis 20 years ago. On the course of our treatment, she did not respond to antibiotics. Moreover, no microorganism grew in the sputum and blood. Thus, the bronchoscopic biopsy was done. The patient showed a dramatic response to steroid therapy, and the pathologic bindings of the bronchoscopic biopsy confirmed as BOOP. To our knowledge, this is the first reported case of BOOP anteceded by rheumatoid arthritis in Korea.

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Usefulness of video-EEG monitoring in paroxysmal nonepileptic events of children and adolescents (소아와 청소년의 돌발적 비간질 발작의 진단에 있어 비디오-뇌파 모니터링의 유용성)

  • Lee, Jee Yeon;Lee, Hee Sun;Choi, Wook Sun;Eun, So Hee;Lee, Ki Hyung;Enu, Baik Lin;Lee, Joo Won
    • Clinical and Experimental Pediatrics
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    • v.51 no.1
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    • pp.62-66
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    • 2008
  • Purpose : In addition to epileptic seizures (ES), a variety of physiologic, organic and psychogenic disorders can manifest as paroxysmal behavioral events. Paroxysmal nonepileptic events (PNEs) are quite encountered in infants, young children, and adolescents. In a substantial proportion of cases, a careful history and examination will elucidate their nature. However, in other cases, it is necessary to differentiate PNEs from ES by video-electroencephalographic (EEG) monitoring. We report our experiences with PNEs in a group of children and adolescents who underwent video-EEG monitoring. Methods : From September, 2004 to June, 2006, one hundred thirty patients were monitored in the Pediatric Epilepsy Monitoring Units of Korea University Guro and Ansan hospitals. Their hospital charts were reviewed and video records of these events were analyzed. We observed all patients after video-EEG monitoring for more than 3 months. Results : Typical spells occurred during monitoring in 33 patients, not associated with a seizure pattern on EEG recordings. Two patients were diagnosed as frontal lobe epilepsy on basis of typical semiology and clinical characteristics, so 31 patients were documented to have PNEs finally. The mean age of patients was $7.2{\pm}5.8\;years$. The male to female ratio was 15 (48.4%) to 16 (51.6%). Among 31 patients, fifteen patients had associated disorders such as epilepsy, developmental delay, cerebral palsy, gastric ulcer, attention deficit hyperactivity disorder or depressive disorder. Somatoform disorder and factitious disorder was frequently seen in children more than 5 years old (P<0.05). Psychogenic disorder was more frequent in female (n=6) than in male (n=2) but there was no statistical significance (P>0.05). Conclusion : Our study suggests that video-EEG monitoring is an important diagnostic tool in the evaluation of paroxysmal behavioral events. With correct diagnosis of the PNEs, several unnecessary treatment could be avoided.

The Relationship between Epileptic Focus and Psychiatric Symptoms of the Refractory Epileptic Patients (난치성 간질환자의 간질초점 위치와 정신증상과의 관련성)

  • Han, Wou-Sang;Kim, Jong-Hoon;Lee, Sang-Kun;Cho, Doo-Young;Kwon, Jun-Soo;Ha, Kyoo-Seob
    • Korean Journal of Psychosomatic Medicine
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    • v.4 no.1
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    • pp.64-70
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    • 1996
  • The prevalence rate of psychiatric symptoms of the refractory epileptic patients was evaluated according to the location of the epileptic focus. The subjects were 91 patients admitted to Epilepsy Monitoring Unit of Seoul National University Hospital. The psychiatric symptoms were assessed by Korean version of Symptom Checklist-90-R(SCL-90-R). The locus of epileptic focus was assessed by clinical features, 2-hour interictal EEG, long-term video-EEG monitoring, brain MRI, interictal and ictal brain SPECT, and interictal brain PET The subjects were divided into three groups according to the epileptic focus, non-temporal(N=29), left temporal (N=26), and right temporal(N=32). There were no statistical differences in demographic and seizure-related variables among groups. The number of patients with $T-score {\geq} 65$ at any subscale of the SCL-90-R were compared by $X^2-test$ among groups. The mean T-scores of each subscale of the SCL-90-R were compared by oneway-ANOVA among groups. The prevalence rate of psychiatric symptoms of the refractory epileptic patients was 38.5%. There was no statistical difference in the prevalence rate of psychiatric symptoms among groups. However, the patients with non-temporal or right temporal epileptic foci showed statistically significant higher mean T-scores of interpersonal sensitivity, depression, hostility, and phobic subscales than the patients with left temporal epileptic foci. These results suggest that the epileptic focus plays an important role in the production of interictal psychiatric symptoms of the refractory epileptics.

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Nonalcoholic Fatty Liver Disease Progressing to Cirrhosis in an Obese Boy with Hypopituitarism (청소년기에 간경화증으로 진행된 비알콜성 지방간질환 1예)

  • Park, Ji-Yong;Ko, Jae-Sung;Seo, Jeong-Kee;Lee, Ran;Shin, Choong-Ho;Kang, Gyeong-Hoon;Kim, Woo-Sun
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.204-209
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    • 2008
  • Non-alcoholic fatty liver disease (NAFLD) is typically associated with obesity and insulin resistance. Non-alcoholic steatohepatitis (NASH) is a more serious form of NAFLD. Although fibrosis is common in pediatric NASH, cirrhosis has been rarely reported. Patients with hypothalamic or pituitary dysfunction are at risk for obesity and insulin resistance with subsequent development of NAFLD. We report a case of NAFLD progressing to cirrhosis in an obese 16 year-old boy with hypopituitarism.

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The Expression of Adhesion Molecules on Alveolar Macrophages and Lymphocytes and Soluble ICAM-1 Level in Serum and Bronchoalveolar Lavge(BAL) Fluid of Patients with Diffuse Interstitial Lung Diseases(DILD) (간질성 폐질환환자들의 기관지 폐포세척액내 폐포 대식세포와 임파구의 접착분자 발현 및 Soluble ICAM-1 농도에 관한 연구)

  • Kim, Dong-Soon;Choi, Kang-Hyun;Yeom, Ho-Kee;Park, Myung-Jae;Lim, Chai-Man;Koh, Yoon-Suck;Kim, Woo-Sung;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.4
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    • pp.569-583
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    • 1995
  • Background: The expression of the adhesion molecules on the cell surface is important in the movement of cells and the modulation of immune response. DILD starts as an alveolitis and progresses to pulmonary fibrosis. So adhesion molecules in these patients is expected to be increased. There are several reports about adhesion molecules in DILD in terms of the percentage of positive cells in immuno-stain, in which the interpretation is subjective and the data were variable. Methods: So we measured the relative median fluorescence intensity(RMFI) which is the ratio of the FI emitted by bound primary monoclonal antibody to FI emitted by isotypic control antibody of the cells in BALF of 28 patients with DILD(IPF:10, collagen disease:7, sarcoidosis:9, hypersensitivity pneumonitis:2) and 9 healthy control. Results: RMFI of the ICAM-1 on AM($3.30{\pm}1.16$) and lymphocyte($5.39{\pm}.70$) of DILD were increased significantly than normal control($0.93{\pm}0.18$, $1.06{\pm}0.21$, respectively, p=0.001, P=0.003). RMFI of the CD18 on lymphocyte was also higher($24.9{\pm}14.9$) than normal($4.59{\pm}3.77$, p=0.0023). And there was a correlation between RMFI of ICAM on AM and the % of AM(r=-0.66, p=0.0001) and lymphocyte(r=0.447, p=0.0116) in BALF. Also RMFI of ICAM on lymphocyte had a significant (r=0.593, p=0.075) correlation with the % of IL-2R(+) lymphocyte in BALF. The soluble ICAM(sICAM) in serum was also significantly elevated in DILD($499.7{\pm}222.2\;ng/ml$) compred to normal($199.0{\pm}38.9$) (p=0.00097) and sICAM in BAL fluid was also significantly higher than normal control group($41.8{\pm}23.0\;ng/ml$ vs $20.1{\pm}13.6\;ng/ml$). There was a Significant correlation between sICAM level in serum and the expression of ICAM-l on AM(r=0.554, p=0.0259).Conclusion: These data suggest that in DILD the expression of adhesion molecules is increased in the AM and BAL lymphocytes with elevated serum sICAM, and these parameter may be useful in determining disease activity.

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Clinicopathologic features of Acute Interstitial Pneumonia (급성 간질성 폐렴의 임상적 고찰)

  • Shim, Jae-Jeong;Park, Sang-Muyn;Lee, Sang-Hwa;Lee, Jin-Gu;Cho, Jae-Yun;Song, Gwan-Gyu;In, Kwang-Ho;Yoo, Se-Hwa;Kang, Kyung-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.1
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    • pp.58-66
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    • 1995
  • Background: Acute interstitial pneumonia is a relatively rare form of interstitial pneumonia, since the vast majority of interstitial pneumonia have a more chronic course. It corresponds to the lesion described by Hamman and Rich, as Hamman-Rich disease in 1944. Another name in the clinical literature is accelerated interstitial pneumonia, idiopathic acute respiratory distress syndrome (idiopathic ARDS), and the organizing stage of diffuse alveolar damage. Acute interstitial pneumonia differs from chronic interstitial pneumonia by clinical and pathologic features. Clinically, this disease is characterized by a sudden onset and a rapid course, and reversible disease. Method and Purpose: Five cases of pathologically proven acute interstitial pneumonia were retrospectively studied to define the clinical, radiologic, and pathologic features. Results: 1) The five cases ranged in age from 31 to 77 years old. The onset of illness was acute in all patients, it began with viral-like prodrome 6~40 days prior to shortness of breath, and respiratory failure eventually developed in all patients. In 2 cases, generalized skin rash was accompanied with flu-like symptoms. Etiologic agent could not be identified in any case. 2) All patients had leukocytosis and severe hypoxemia. Pulmonary function test of 3 available cases shows restrictive ventilatory defect, and one survived patient(case 5) has a complete improvement of pulmonary function after dismissal. 3) Diffuse bilateral chest infiltrates were present radiologically. Theses were the ground-glass, consolidation, and reticular densities without honeycomb fibrosis in all patients. The pathologic abnormalities were the presence of increased numbers of macrophages and the formation of hyaline membranes within alveolar spaces. There was also interstitial thickening with edema, proliferation of immature fibroblast, and hyperplasia of type II pneumocyte. In the survived patient(case5), pathologic findings were relatively early stage of acute interstitial pneumonia, such as hyaline membrane with mild interstitial fibrosis. 4) Of the 5 patients, four patients died of respiratory failure 14~90 days after onset of first symptom, and one survived and recovered in symptoms, chest X ray, and pulmonary function test Conclusion: These results emphasize that acute interstitial pneumonia is clinically, radiologically, and pathologically distinct form of interstitial pneumonia and should be separated from the group of chronic interstitial pneumonia. Further studies will be needed to evaluate the pathogenesis and the treatment of acute interstitial pneumonia.

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Update in Diagnosis of Idiopathic Pulmonary Fibrosis and Interstitial Lung Abnormality (특발폐섬유증 진단의 최신 지견과 간질성폐이상)

  • Bo Da Nam;Jung Hwa Hwang
    • Journal of the Korean Society of Radiology
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    • v.82 no.4
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    • pp.770-790
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    • 2021
  • Idiopathic pulmonary fibrosis (IPF), based on the 2018 international clinical practice guidelines, can be diagnosed with a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and compatible clinical findings. Given that imaging is pivotal for IPF evaluation and diagnosis, more emphasis should be placed on the integration of clinical, radiological, and pathologic findings for multidisciplinary diagnosis. Interstitial lung abnormality (ILA), on the other hand, has a purely radiological definition based on the incidental identification of CT abnormalities. Taken together, differentiation between ILA and clinically significant interstitial lung disease (ILD) must be based on proper clinical evaluation. With this review, the recent updates in IPF diagnosis and the radiologic considerations for ILA can be well understood, which can be helpful for the proper diagnosis and management of patients with diffuse interstitial pulmonary fibrosis.

Analysis of Image for Liver Disease using Blood Test in the Ultrasound Fibroscan (Fibroscan에서의 혈액검사를 이용한 간질환의 영상분석)

  • Lee, Jeong-Hyun;Kim, Dong-Hyun;Kim, Changsoo
    • The Journal of the Korea Contents Association
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    • v.15 no.8
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    • pp.389-396
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    • 2015
  • The liver fibrosis is a disease we often see in clinical medicine, and the persistence and repeatition of inflammation and necrosis of liver cells continue for several years, and it is proceeded to cirrhosis. So decrease of death rate and prevalence rate by complications of cirrhosis and hepatocellular carcinoma is main task of clinical medicine by protection of chronic liver ailment patients from proceeding to cirrhosis and hepatocellular carcinoma. So this study tried to represent the ultrasonic image, blood test, the relationship with liver stiffness of diffuse liver ailment patients as numbers. This study was performed with patients from whom the image was taken by ultrasonic and 141 people who were treated by fibroscan, the basic data for blood test was obtained from the test results at the time when ultrasonic image and liver fibroscan was performed. The statistical analysis was performed by One-way analysis of variance(ANOVA) to verify difference between groups. The value of liver stiffness was increased in the order of normal, chronic liver disease and cirrhosis. As a results, ALT and Albumin have no statistical difference between object groups, and there are statistical differences in the results of ultrasonic decoding at age, AST, ALP, Bilirubin, PLT, PT, and kPa, and they are statistically meaningful(p<0.005). And the value of liver stiffness of chronic liver ailment was presented only as over 12.5kPa in other study, but it was represented as numbers for quantitative diagnosis by presenting average kPa threshold value according to disease in this study. And by presenting relationship of diagnosed results, it is considered that it could be used as first tool to diagnose chronic liver ailment patients according to their disease.

Combined Tubal and Interstitial Pregnancies after IVF-ET: a Case Report (체외수정 및 배아 이식 후 난관과 반대편 간질 부위에 동시에 생긴 복합성 자궁외 임신 1예)

  • Oh, Yoe-Un;Kim, Mi-Kyoung;Lee, Seo-Yeong;Kim, You-Shin
    • Clinical and Experimental Reproductive Medicine
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    • v.36 no.2
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    • pp.129-136
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    • 2009
  • The incidence of ectopic pregnancy is increased since in vitro fertilization-embryo transfer (IVF-ET) has started. And because of the possibility of combined pregnancy after IVF-ET, bilateral adnexal area and other portion of pelvis should be examined carefully by transvaginal ultrasonography during follow-up period. The rate of combined pregnancy after IVF is estimated to be as high as 0.2~1%. Since the incidence of combined pregnancies is markedly higher with IVF, special attention to this possibility after ET is warranted. According to a recent report, the occurrence of interstitial pregnancy is estimated to be 1:3600 for all pregnancies achieved with IVF. The prevalence of interstitial pregnancies is 2~4% of ectopic pregnancies. Interstitial pregnancies tend to be diagnosed later than most other ectopic pregnancies, and if uterine rupture occurs, the resulting hemorrhage can be severe. Currently, about 2% to 3% of interstitial pregnancies are thought to carry a maternal death, which is twice that of other tubal gestations. We report a case of simultaneous left tubal and right interstitial pregnancies after IVF-ET with a brief review of literature.