• Title/Summary/Keyword: idiopathic

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A Case of Atypical Thrombotic Microangiopathy (비전형적 혈전성 미세병증 1례)

  • Oh, Ji Young;Park, Se Jin;Kim, Ki Hwan;Lim, Beom Jin;Jeong, Hyeon Joo;Ki, Jung Hye;Kim, Kee Hyuck;Shin, Jae Il
    • Childhood Kidney Diseases
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    • v.17 no.2
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    • pp.149-153
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    • 2013
  • We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

Slit Ventricle Syndrome in Children : Clinical Presentation and Treatment (소아에서 틈새뇌실 증후군 : 임상 양상 및 치료)

  • Shin, Beom-Sik;Yang, Kook-Hee;Kim, Dong-Seok;Choi, Joong-Uhn
    • Journal of Korean Neurosurgical Society
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    • v.30 no.sup2
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    • pp.309-315
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    • 2001
  • The term slit ventricle syndrome(SVS) refers to an episodic occurrence of headache, vomiting, and possibly some degree of impaired consciousness in shunted hydrocephalic children in whom slit-like ventricles are seen on CT scan or MRI. Authors present 6 cases with SVS who were treated at our institute for last 10 years. From 1986 to 1996, 821 patients underwent shunt surgery for hydrocephalus. The etiology of hydrocephalus included brain tumor(140 patients), post-hemorrhagic(62 patients), idiopathic normotensive hydrocephalus(64 patients), post-meningitic( 58 patients), post-traumatic(54 patients), congenital(48 patients), neurocysticercosis(31 patients), and unknown etiology(364 patients). During the mean follow-up duration of 68 months, 232 shunt revisions were performed by a revision rate of 1.28 per patient. The incidence of SVS was 0.7%(6 patients). Most of them have been operated on in infancy. Time interval from the first operation to the development of slit-ventricle syndrome ranged from 4 to 8 years, the mean was 6 years. Shuntogram showed patent shunt in all patients. Two patients with less severe clinical symptoms improved with conservative treatment. These patients were not measured ICP because of good hospital course. One patient showed high ICP and needed only revision with same pressure valve as previous shunt. Low ICP was noted in 3 patients. Pressure augmentation using an anti-siphon device(ASD) or upgrading valve system were necessary in these patients. The authors stress that determining type of SVS is the first step in treatment planning and that the best treatment is a strategy aimed at resolving the specific type of SVS responsible for the symptoms.

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Clinical Evaluation of Exudative Pleural Effusion (삼출성 늑막염의 임상적 고찰)

  • Kwon, Kyeong-Soon;Yang, Chang-Heon;Lee, Kwan-Ho;Lee, Yeung-Hyun;Chung, Jae-Chun;Lee, Hyun-Woo
    • Journal of Yeungnam Medical Science
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    • v.6 no.1
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    • pp.69-80
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    • 1989
  • From December 1987 to September 1988, clinical evaluation were performed at the Yeungnam University Hospital on 138 patients with exudative pleural effusion comparing with biochemical, bacteriologic, cytologic and pathologic studies. The results were as follows 1. Among thease 138 cases, Incidence of tuberculosis was 57.3%, neoplasm 26.8%. High tendency in malignant pleural effusion occured in elder age. 2. In tuberculosis pleural effusion, the rate of positive smear and culture for acid-fast bacilli in the pleural fluid was 3.7% and positive biopsy for granuloma 75%. 3. In malignant pleural effusion, the rate of positive cytology for cancer cell in the fluid was 42% and positive biopsy 60%. 4. Analysis in tuberculosis and malignancy showed the tendency of high pH, WBC, protein and of low glucose, but there were clinically not significant in differentiating malignant pleural effusion from tuberculous pleural effusion. 5. Among 23 cases in which the pleural tissue findings were chronic nonspecific reaction pathologically, tuberculosis(52.2%), malignancy(26%) and idiopathic(21.8%) eventually in follow up studies.

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Hemiplegic Migraine Presenting with Unilateral Facial Palsy: A case report (편측안면마비로 발현한 편마비편두통 1예)

  • Yeon, Gyu Min
    • Journal of the Korean Child Neurology Society
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    • v.26 no.4
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    • pp.288-291
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    • 2018
  • Hemiplegic migraine (HM) is a rare subtype of migraine with aura and is accompanied by a fully reversible motor aura. HM can occur in two forms: familial or sporadic. Currently, three genes are related to familial HM. Typically, HM occurs in the first or second decade of life and involves gradually progressing aura symptoms in succession, accompanied by headaches. The aura includes visual, sensory, motor, aphasic and often basilar-type symptoms. Motor aura (weakness) is related to the regions where the sensory aura is involved, and it usually starts at the hand before spreading to the arm and face. Aphasia is a common form of speech aura, but does not typically present as a difficulty in understanding. In this case report, the sensory-motor aura started at the right face and then gradually progressed to the right leg without any symptoms in the ipsilateral upper extremity. To the best of my knowledge, there has been no previous case report for the presentation of a hemiplegic migraine, as in this case report. As there is a possibility of misdiagnosis of Bell's palsy at the early stage of this case, this case report suggests that a physician should consider the rare possibility of stroke or HM when a patient presents with unilateral facial palsy.

MTHFR 3'-untranslated region polymorphisms contribute to recurrent pregnancy loss risk and alterations in peripheral natural killer cell proportions

  • Kim, Eun Sun;Kim, Jung Oh;An, Hui Jeong;Sakong, Jung Hyun;Lee, Hyun Ah;Kim, Ji Hyang;Ahn, Eun Hee;Kim, Young Ran;Lee, Woo Sik;Kim, Nam Keun
    • Clinical and Experimental Reproductive Medicine
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    • v.44 no.3
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    • pp.152-158
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    • 2017
  • Objective: To identify the associations between polymorphisms of the 3'-untranslated region (UTR) of methylenetetrahydrofolate reductase (MTHFR) gene, which codes for an important regulatory enzyme primarily involved in folate metabolism, and idiopathic recurrent pregnancy loss (RPL) in Korean women. Methods: The study population comprised 369 RPL patients and 228 controls. MTHFR 2572C > A, 4869C > G, 5488C > T, and 6685T > C 3'-UTR polymorphisms were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis or by TaqMan allelic discrimination assays. Natural killer cell proportions were determined by flow cytometry. Results: The MTHFR 2572-5488-6685 (A-C-T) haplotype had an adjusted odds ratio of 0.420 (95% confidence interval, 0.178-0.994; p= 0.048) for RPL. Analysis of variance revealed that MTHFR 4869C > G was associated with altered $CD56^+$ natural killer cell percentages (CC, $17.91%{\pm}8.04%$; CG, $12.67%{\pm}4.64%$; p= 0.024) and folate levels (CC, $12.01{\pm}7.18mg/mL$; CG, $22.15{\pm}26.25mg/mL$; p= 0.006). Conclusion: Variants in the 3'-UTR of MTHFR are potential biomarkers for RPL. However, these results should be validated in additional studies of ethnically diverse groups of patients.

Bioequivalence Test of Fexofenadine Hydrochloride 120 mg Tablets (염산펙소페나딘 120밀리그람 정제의 생물학적동등성시험)

  • Cho, Hea-Young;Kang, Hyun-Ah;Kim, Se-Mi;Lee, Yong-Bok
    • YAKHAK HOEJI
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    • v.52 no.3
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    • pp.188-194
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    • 2008
  • Fexofenadine, ($\pm$)-4-1-hydroxy-4-{4-(hydroxydiphenylmethyl)-1-piperidinyl}-butyl-a,a-dimethyl benzeneacetic acid, is a selective histamine $H_1$ receptor antagonist, and is clinically effective in the treatment of seasonal allergic rhinitis and chronic idiopathic urticaria as a first-line therapeutic agent. The purpose of the present study was to evaluate the bioequivalence of two fexofenadine hydrochloride tablets, $Allegra^{(R)}$ (Handok Pharmaceuticals Co., Ltd.) and Alecort (Samchundang Pharmaceutical Co., Ltd.), according to the guidelines of the Korea Food and Drug Administration (KFDA). The release of fexofenadine from the two fexofenadine hydrochloride formulations in vitro was tested using KP VIII Apparatus II method with various dissolution media. Twenty six healthy male subjects, 25.62$\pm$3.35 years in age and 70.05$\pm$11.71 kg in body weight, were divided into two groups and a randomized 2$\times$2 cross-over study was employed. After a single tablet containing 120 mg as fexofenadine hydrochloride was orally administered, blood samples were taken at predetermined time intervals and the concentrations of fexofenadine in serum were determined using HPLC with fluorescence detector. The dissolution profiles of two formulations were similar in all tested dissolution media. The harmacokinetic parameters such as $AUC_t$, $C_{max}$ and $T_{max}$ were calculated, and ANOVA test was utilized for the statistical analysis of the parameters using logarithmically transformed $AUC_t$, $C_{max}$ and untransformed $T_{max}$. The results showed that the differences between two formulations based on the reference drug, $Allegra^{(R)}$, were -1.37, 5.22 and 16.50% for $AUC_t$, $C_{max}$ and $T_{max}$, respectively. There were no sequence effects between two formulations in these parameters. The 90% confidence intervals using logarithmically transformed data were within the acceptance range of log 0.8 to log 1.25 (e.g., log 0.83$\sim$log 1.08 and log 0.81$\sim$log 1.03 for $AUC_t$ and $C_{max}$, respectively). Thus, the criteria of the KFDA bioequivalence guideline were satisfied, indicating Alecort tablet was bioequivalent to $Allegra^{(R)}$ tablet.

Investigation of the Current Clinical Result of Korean Medical Treatment of Infertility -In Major University Korean Medicine Hospitals and Local Korean Medicine Clinics Participated in the Research- (한방 난임 치료의 특성과 결과에 대한 분석 -2011년 주요 한의과대학 부속한방병원 및 연구 참여 한의원을 중심으로-)

  • Lee, Dong-Nyung;Choi, Min-Sun;Kim, Dong-Il
    • The Journal of Korean Obstetrics and Gynecology
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    • v.27 no.4
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    • pp.69-87
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    • 2014
  • Objectives: We analyzed retrospective clinical data of Korean medical institutes for infertility care and investigated current status and outcome of the Korean medical treatment of infertility as a part of foundational research for verifying validity of constructing national support system and developing appropriate policy on Korean medical treatment of infertility. Methods: We investigated data uploaded on the homepage of The Society of Korean Medicine for Subfertility (http://www.okinfertility.org) by Korean medical institutes for infertility care to get informations such as patients' age, body height, weight, methods of Korean medical treatment, cost and duration of treatment, success or failure of pregnancy and result of treatment. Results: The average age of patients was $33.1{\pm}3.8$ and the average body height was $161.2{\pm}5.3cm$ and the average body weight was $55.2{\pm}8.5kg$. The method of Korean medical treatment was Herbal medicine (97.5%), acupuncture (80.4%), moxibustion (57.7%), cupping (32.7%). The average cost of treatment was $1,160,625{\pm}882,499$ won, and the average medical cost per visit was $357,845{\pm}241,602$ won. The average duration of treatment was $11.26{\pm}10.58$ weeks, and the average number of visits per patient was $4.78{\pm}6.10$ times. The average duration of treatment was the longest in the group of infertile patients with the highest average medical cost per visit. The average pregnancy success rate was 30.9% overall, and that of the group of infertile patients was 25.8%. Conclusions: When we develop the standard project model with expectation for about 25% success rate of pregnancy and delivery in the group of patients who have idiopathic and ovulatory factors, it is desirable to perform acupuncture and moxibustion treatment 1-2 times a week with herbal medicine. The treatment period is set to at least 12 weeks. Average treatment fee maybe calculated by converting the averaged treatment cost of clinic's one month worth of daily treatment fee and medical hospital's ten day treatment fee into weekly treatment cost.

Thickness of the Macula, Retinal Nerve Fiber Layer, and Ganglion Cell-inner Plexiform Layer in the Macular Hole: The Repeatability Study of Spectral-domain Optical Coherence Tomography

  • Lee, Woo Hyuk;Jo, Young Joon;Kim, Jung Yeul
    • Korean Journal of Ophthalmology
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    • v.32 no.6
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    • pp.506-516
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    • 2018
  • Purpose: We measured the thicknesses of the ganglion cell and inner plexiform layer (GCIPL), the macula, and the retinal nerve fiber layer (RNFL) using spectral-domain optical coherence tomography in patients with idiopathic macula holes to analyze the repeatability of these measurements and compare them with those of the fellow eye. Methods: We evaluated 85 patients who visited our retinal clinic. The patients were divided into two groups according to their macular hole size: group A had a size of $<400{\mu}m$, while group B had a size of ${\geq}400{\mu}m$. Repeatability was determined by comparing the thicknesses of the GCIPL, macula, and RNFL with those of the normal fellow eye. Results: The average central macular thickness in patients with macular holes was significantly thicker than that in the normal fellow eye ($343.8{\pm}78.6$ vs. $252.6{\pm}62.3{\mu}m$, p < 0.001). The average thickness of the GCIPL in patients with macular holes was significantly thinner than that in the normal fellow eye ($56.1{\pm}23.4$ vs. $77.1{\pm}12.8{\mu}m$, p < 0.001). There was no significant difference in the average RNFL thickness between eyes with macular holes and fellow eyes ($92.4{\pm}10.0$ vs. $95.5{\pm}10.7{\mu}m$, p = 0.070). There were also no significant differences in the thicknesses of the GCIPL and RNFL among the two groups (p = 0.786 and p = 0.516). The intraclass correlation coefficients for the macula and RNFL were 0.994 and 0.974, respectively, in patients with macular holes, while that for the GCIPL was 0.700. Conclusions: Macular contour change with macular hole results in low repeatability and a tendency of thinner measurement regarding GCIPL thickness determined via spectral-domain optical coherence tomography. The impact of changes in the macular shape caused by macular holes should be taken into consideration when measuring the GCIPL thickness in patients with various eye diseases such as glaucoma and in those with neuro-ophthalmic disorders.

Long-Term Outcomes of Adult Lung Transplantation Recipients: A Single-Center Experience in South Korea

  • Jo, Kyung-Wook;Hong, Sang-Bum;Kim, Dong Kwan;Jung, Sung Ho;Kim, Hyeong Ryul;Choi, Se Hoon;Lee, Geun Dong;Lee, Sang-Oh;Do, Kyung-Hyun;Chae, Eun Jin;Choi, In-Cheol;Choi, Dae-Kee;Kim, In Ok;Park, Seung-Il;Shim, Tae Sun
    • Tuberculosis and Respiratory Diseases
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    • v.82 no.4
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    • pp.348-356
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    • 2019
  • Background: Recently, the number of lung transplants in South Korea has increased. However, the long-term outcome data is limited. In this study, we aimed to investigate the long-term outcomes of adult lung transplantation recipients. Methods: Among the patients that underwent lung transplantation at a tertiary referral center in South Korea between 2008 and 2017, adults patient who underwent deceased-donor lung transplantation with available follow-up data were enrolled. Their medical records were retrospectively reviewed. Results: Through eligibility screening, we identified 60 adult patients that underwent lung (n=51) or heart-lung transplantation (n=9) during the observation period. Idiopathic pulmonary fibrosis (46.7%, 28/60) was the most frequent cause of lung transplantation. For all the 60 patients, the median follow-up duration for post-transplantation was 2.6 years (range, 0.01-7.6). During the post-transplantation follow-up period, 19 patients (31.7%) died at a median duration of 194 days. The survival rates were 75.5%, 67.6%, and 61.8% at 1 year, 3 years, and 5 years, respectively. Out of the 60 patients, 8 (13.3%) were diagnosed with chronic lung allograft dysfunction (CLAD), after a mean duration of $3.3{\pm}2.8years$ post-transplantation. The CLAD development rate was 0%, 17.7%, and 25.8% at 1 year, 3 years, and 5 years, respectively. The most common newly developed post-transplantation comorbidity was the chronic kidney disease (CKD; 54.0%), followed by diabetes mellitus (25.9%). Conclusion: Among the adult lung transplantation recipients at a South Korea tertiary referral center, the long-term survival rates were favorable. The proportion of patients who developed CLAD was not substantial. CKD was the most common post-transplantation comorbidity.

Effects of Breathing, Meditation and Qigong on the Impairable Dysfunction of Olfactory Sense in the Parkinson's Disease (파킨슨 병(PD)의 후각기능 장애에 대한 호흡 명상 기공 효과)

  • An, So Jung
    • Journal of Naturopathy
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    • v.9 no.2
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    • pp.37-45
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    • 2020
  • Purpose: Symptoms of idiopathic Parkinson's disease (PD) include tremors, bradykinesia, and rigidity. The purpose was to explore the effects of breathing, meditation and qigong on the improving of insight, behavior, mood discomfort, depression, anxiety, and olfactory dysfunction, which are PD non-motor symptoms. Methods: Three stages of An's-4444 healing breathing, An's Gwanjeong healing meditation, and healing qigong performed 12 times for 80 minutes at a time in subjects with PD (11 patients), and pre- and post-measurements compared and evaluated. Results: The Integrated Parkinson's Rating Scale (UPDRSI) for mood discomfort after 12 healings was 69%. The Depression Scale (61%) for HAMD, and 64% for Anxiety (HAMA)), and the smell identification test (TSI) for a trial for olfactory dysfunction, improved to 82%, respectively. However, the numerical values after one month after 12 healing were almost same in all four scales. This means that the healing effect maintained until after one month. Conclusions: An's healing breathing, meditation and qigong therapy significantly improved insight, behavior, and mood discomfort, and non-motor symptoms such as depression, anxiety, and olfactory dysfunction. These results suggest that An's breathing, meditation and qigong therapy are valuable as a primary therapy to improve and heal non-motor symptoms in Parkinson's disease patients. Further research in biomedical science is needed.