• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.523-527
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• 2008

Purpose : Several cytokines play important roles in the inflammatory process of Henoch-$Sch\ddot{o}lein$ Purpura (HSP). It is likely that transforming growth $factor-{\beta}$ ($TGF-{\beta}$) is involved in the pathogenesis of HSP. The purpose of this study is to investigate whether $TGF-{\beta}$ promoter polymorphism is associated with the renal involvement of childhood HSP. Methods : Thirty-four patients younger than 15 years, who had been diagnosed with HSP, as well as 27 controls, were examined. Patients and controls were genotyped for $TGF-{\beta}$ C-509T by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Results : The T allelic frequencies in patients and controls showed no difference (45% vs. 48.8%). No allele or genotype differences between the group of HSP group and control group were observed. The frequencies of $TGF-{\beta}$ 509 genotypes TT, TC, and CC were no different between patients and controls (26% vs. 22%). The TT genotype of polymorphism of the $TGF-{\beta}$ C-509T gene had no relation to the susceptibility of children to HSP and renal involvement in HSP. Conclusion : $TGF-{\beta}$ T allele may not be related to the susceptibility of children to HSP. The TT genotype of polymorphism of the $TGF-{\beta}$ C 509T gene does not appear to have an influence on renal involvement in childhood HSP.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.405-409
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• 2008

Churg-Strauss syndrome is a rare form of systemic necrotizing vasculitis that occurs exclusively in patients with asthma, and is associated with blood and tissue eosinophilia. The classic pathology findings in the lung include a combination of eosinophilic pneumonia, granulomatous inflammation and necrotizing vasculitis. However, there are few reports of tracheobronchial mucosal lesions in Churg-Strauss syndrome. We report a case of Churg-Strauss syndrome with multiple tracheobronchial mucosal lesions in a 33-year-old man with a history of bronchial asthma and allergic rhinitis. He had been diagnosed with community acquired pneumonia at another hospital and was treated with antibiotics. However, the chest radiographic findings were aggravated and showed multifocal consolidations in the whole lung fields. He was transferred to the Asan Medical Center. Fiberoptic bronchoscopy revealed multiple nodular mucosal lesions of the trachea and bronchi. The histopathology of the mucosal lesions revealed necrotizing bronchial inflammation with eosinophilic infiltration. Video Assisted Thoracic Surgery was performed. The wedge resected lung tissue revealed chronic eosinophilic pneumonia that was consistent with Churg-Strauss syndrome. Methylprednisolone (1 mg/kg q 8 hr) was prescribed and his symptoms resolved gradually. The chest radiographic findings improved significantly, and a follow-up fiberoptic bronchoscopy performed eight days later showed that the tracheobronchial mucosal lesions had resolved. The patient was prescribed oral prednisolone for 20 months after discharge. Currently, the patient is not taking steroids and is being followed up.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.575-579
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• 2009

Behcet's disease(BD) is a multisystem vasculitis that almost any organ in the body may be involved. Its diagnosis bases on clinical criteria in the absence of any specific laboratory test to confirm it. The clinical criteria proposed for the diagnosis for BD include recurrent oral and genital ulceration, skin lesion, ocular changes, and positive response to pathergy test. Besides these essential features, BD may manifest itself on the gastrointestinal tract, central nervous system, and other systemic involvements. Among these clinical features, recurrent oral ulcers are present in most BD patients, often as the initial symptom, preceding other manifestations by many years. Therefore, dentists are in the important position for the early detection and diagnosis of BD. In this case report, we are presenting a case of 6 year-old girl, who came to the pediatric dentistry department for evaluation and treatment of pain and dysphagia caused by severe recurrent oral ulcers. Through taking detailed dental and medical history and clinical examination, we reported the possibility of BD to the medical team and prescribed topical lidocaine to relief pain during swallowing. The medical team, which involved pediatrics, ophthalmology, dermatology, and gastroenterology, finally confirmed BD through various examinations. Detection of the possibility of BD in the dental field helped early diagnosis and proper management by the medical team.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.185-194
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• 2007

Purpose : $Henoch-Sch\ddot{o}nlein$ Purpura(HSP) is a self-limited systemic small vessel vasculitis, however, renal involvement is considered to contribute to the outcome of this disease. Therefore, identifying the renal risk factors in HSP and prevention of renal involvement are important. The aim of this study is to investigate whether early steroid administration in HSP could reduce the rate of renal involvement. Methods : We retrospectively studied two hundred children with HSP. We had administrated steroids orally to resolve of severe abdominal pain, joint and scrotal symptoms. We analyzed the relationship between the steroid therapy to relieve systemic symptoms and the subsequent renal involvement in HSP. Results : There were no significant differences in the incidence and duration of renal involvement according to steroid administration and its duration. In HSP patients with renal manifestations, steroid administration group showed a tendency of hematuria and steroid non-administration group showed a tendency of proteinuria, however, we could not find statistically significant differences in each group. There was no significant difference in the duration of purpura presence according to steroid administration. However, persistent purpura increased the incidence and the duration of renal involvement. Conclusion : Early steroid administration did not reduce the risk of renal involvement, there-fore, steroid could not prevent delayed nephritis in children with HSP On the other side, Persistent Purpura, known to be not related to steroid therapy, was associated with renal involvement. We suggest that early steroid administration could not be useful in preventing the renal involvement in HSP. (J Korean Soc Pediatr Nephrol 2007;11:185-194)

• Tuberculosis and Respiratory Diseases
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• v.58 no.1
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• pp.78-82
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• 2005

Propylthiouracil(PUT) is a drug which used at Grave's disease. But PTU has recently been observed to associated with antineutrophil cytoplasmic antibody(ANCA)-positive vasculitis resulting in, infrequently, diffuse alveolar hemorrhage. We report the case of a patient who developed diffuse pulmonary hemorrhage after she had been taking PTU for two years. She had received a diagnosis of Grave's disease at two years ago. The serologic study was positive for ANCA with myeloperoxidase(MPO) specificity. Bronchoalveloar lavage(BAL) fluid analysis revealed hemosiderinladen macrophages. Such findings suggested propylthiouracil-induced dffuse pulmonary hemorrhage associated with antineutrophil cytoplasmic antibody. To our knowledge, this represents the first documentation in a case of PTU-induced diffuse pulmonary hemorrhage in Korea.

• Journal of the Korean Chemical Society
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• v.50 no.2
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• pp.123-128
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• 2006

Behcet's disease (BD) is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. Although the exact pathogenesis for BD is not completely understood, it has been suggested that the disease is triggered in genetically susceptible individuals by environmental factors, such as microbial agents. It is noted that multiple genes, including MHC (major histocompatibility complex) and non-MHC genes, are implicated in the pathogenesis of BD. This study tries to determine whether IL-6 gene polymorphisms are associated with susceptibility to Behcet's disease in Koreans. Gene polymorphisms were typed by VNTR (variable number of tandem repeat), RFLP (restriction fragment length polymorphism), DHPLC (denaturing high performance liquid chromatography).There were no evidences for genetic association conferred by the IL-6prom polymorphism. However, significant differences in the IL-6vntr genotype and allele frequencies were found between patients with BD and controls. The IL-6vntr*C allele appeared to be an additional susceptibility gene to Korean BD. Further studies in other populations and gene are required to confirm these results.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1212-1217
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• 1996

From Dec. 1993 to May 1995, 9 male and 5 female patients ranging in age from 25 to 65 years, were operated on for ascending aorta and/or aortic arch diseases. Six patients had acute aortic dissection, type A(ruptured in 4 cases); four had ruptured ascending aortic aneurysm; three had annuloaortic ectasia(ruptured in 1 cases); one had aortic arch aneurysm. The diagnostic procedures were echo cardiography and dynamic CT scan in all patients having acute dissection or rupture. The aortic angiography was performed in two cases. Indications for operations were rupture in five cases, acute aortic dissection in five cases, severe congestive heart failure in two cases, progressive aortic insufficiency in one case and impending rupture in one case. The emergent repair was performed in ten cases(71%). The surgical treatment consisted of 6 Cabrol operations, a Cabrol operation combined with arch replacement, a modified Bentall operation, 4 replacement of ascending aorta, a replacement of aortic arch, and a replacement of ascending aorta and aortic arch. Complications were a hypoxic encephalopathy, two atrial fibrillations, a sternal deheiscence, and a mediastinitis. Two early mortality(14%) were due to intractable bleeding and multiple organ failure, and one late mortality(7%) was due to ventricular arrhythmia. In eleven survivors, follow-up period was from 2 months to 12 months and the course was uneventful.

• Journal of Life Science
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• v.23 no.2
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• pp.311-318
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• 2013

Schisandra chinensis containing a variety of pharmacologically active lignans has been traditionally used in oriental medicine. In this study, anti-inflammatory compounds were screened from the hexane extracts of S. chinensis by activity-guided fractionation. First, we investigated the regulatory effects on the expression of E-selectin, intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) with 38 fractions from the hexane extracts of S. chinensis in human umbilical vein endothelial cells (HUVECs). As a result, SCKH1 among the 38 fractions from the hexane extract of S. chinensis was selected for further analysis based on its unique regulatory effect on cell adhesion molecules, especially on VCAM-1, in LPS-stimulated HUVECs. The subsequent activity-guided fractionation of SCKH1 resulted in the purification of SCKH1PAIBPB, which was found to suppress the expression of VCAM-1, MCP-1, IL-6 and IL-8 in HUVECs stimulated with LPS, and to inhibit the adhesive capacity between HUVECs and monocytes. Taken together, our data indicate that SCKH1PAIBPB can be proposed as an effective anti-inflammatory compound that may have a potential therapeutic use for the prevention and treatment of various inflammatory diseases as well as ischemic vascular diseases.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.32 no.3
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• pp.116-135
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• 2019

Objectives : The purpose of this study is to analyze how purpura and vasculitis have been treated with Korean medicine and potentially to present with future direction of research and treatment. Methods : We searched clinical studies from the Korean databases including Oriental medicine Advanced Searching Integrated System(OASIS), Korean Traditional Knowledge Portal(KTKP), National Discovery for Science Leader(NDSL), Research Information Sharing Service(RISS), using keywords related to "Purpura" and "Vasculitis" from January 2000 to May 2019. Results : A total of 20 studies were selected for analysis. More than half of the patients provided with detailed information were under 19 year-olds, and upper respiratory infection, stress and fatigue, seasonal factor were among the most frequently stated as predisposing factors. Among many treatment modalities, herbal medicine was the most frequently used, followed by acupuncture and herbal acupuncture. 23 basic herbal medicine formulas were retrieved from 20 articles, most frequently used being Guibi-tang(歸脾湯加味), Samul-tang(四物湯加味) and Yukmijihwang-tang(六味地黃湯加味). In total, 122 Korean medicine herbs were used, most frequently used herbs being Glycyrrhizae Radix et Rhizoma(甘草), Angelicae Gigantis Radix(當歸), Poria Sclerotium(茯?) and Paeoniae Radix(芍藥). By its category, herbs were mostly classified into Tonyfying and replenishing medicinal(補益藥), Heat-clearing medicinal(淸熱藥) and Exterior-releasing medicinal(解表藥). Patients with total treatment period of 3 months or under were 2/3 of all cases provided with detailed information. Follow up periods were short in general with only 6 cases of over 6 months. The primary motive of patients to receive Korean medicine treatments was unresponsiveness or adverse effects of western medication. Conclusion : Through this literature review, we could find out tendencies of Korean medicine treatments of purpura and vasculitis up to date and some points that may have clinical significance.