Min Jae Hong;Paek Kyung Hoon;Park Kyung Mi;Kim Jung Sue;Ha Il Soo;Cheong Hae Il;Kim Joong Gon;Choi Yong
Childhood Kidney Diseases
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v.3
no.1
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pp.80-87
/
1999
Purposes : Renal involvement is a potentially serious complication of systemic lupus erythematosus (SLE). There have been only few studies of lupus nephritis in pediatric age. In this study, the clinical manifestations, pathologic findings, response to treatment, and clinical course of lupus nephritis in children were analyzed. And the results will provide basic data for future nation-wide prospective multi-center study. Methods . The medical records of 46 children clinically and pathologically diagnosed to have lupus nephritis at Seoul National University Children's Hospital during 1986 to 1997 were analyzed retrospectively. Results : 1) The median age of diagnosis of lupus nephritis was 12.8 years ($2\;years\~\;15year$ 8months), and the sex ratio was 1:2.5. 2) FANA($85.7\%$), anti-ds-DNA antibody ($78.0\%$), and malar rash ($60.8\%$) were the most common findings among the classification criteria by ARA Decreased C3 was detected in $88.9\%$ of patients. 3) Hematuria ($87.0\%$) was the most common renal symptom, and WHO class IV lupus nephritis was identified in 41 cases by renal biopsy. 4) In most of patients, the disease activity was controlled relatively well with a single or combined therapy of prednisolone, azathioprine, or cyclophosphamide. The response revealed no difference according to the mode of treatment. 5) Infection, especially of Varicella-Zoster virus and candida, was the most common complication during the disease course. Conclusion : The renal involvement was noted in $87.0\%$ of childhood SLE, and $89.1\%$ of renal lesions was WHO class IV lupus nephritis known to associated with poor long-term prognosis. So, aggressive treatment using immunosuppressants in the early disease course may be helpful to increase long-term prognosis of lupus nephritis. A prospective multi-center study is necessary to analyze the therapeutic efficacy of various treatment modalities.
Background: Anti-cardiolipin antibody (Anti-CL Ab) is one of the various antiphospholipid antibodies (Anti-PL Abs) and found in the plasma of patients with systemic lupus erythematosus (SLE), atherosclerosis, and other infectious diseases. While anti-PL Abs found in the sera of patients with infectious diseases bind directly to CL, binding of anti-PL Abs to CL circulating in the sera of patients with autoimmune diseases is mediated by $\beta_2-$glycoprotein 1 ($\beta_2-GP1$). The purpose of this study is to investigate the effect of <$\beta_2-GP1$ on the antigen binding assay of anti-CL Abs present in the sera of patients with atherosclerosis, which has been known as one of autoimmune diseases. Methods: ELISA was performed with sera containing anti-CL Abs from three patients with atherosclerosis in the presence or absence of $\beta_2-GP1$ or FBS. Results: Reactivity of anti-CL Abs to CL was increased in the presence of $\beta_2-GP1$ or FBS in a dose dependent manner. Conclusion: <$\beta_2-GP1$ or FBS could be used as co-factor in CL ELISA with anti-CL Abs present in the sera of patients with atherosclerosis. It is suggested that anti-CL Abs found in atherosclerosis patients are similar in terms of antigen binding property to those circulating in the patients with autoimmune diseases, not to infectious diseases.
Kim Woo-Hyeok;Ha Il-Ju;Yoon Jung-Han;JaeGal Young-Jong
Korean Journal of Head & Neck Oncology
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v.16
no.2
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pp.212-215
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2000
Background and Objective: Kikuchi's disease(KD) is an idiopathic, self-limited lymphadenopathy that was described as a distinctive type of necrotizing lymphadenitis affecting primarily cervical lymph nodes of young adults independently by Kikuchi and Fujimoto et al at first in 1972. The purpose of this study is a knowledge about clinicopathologic findings, many laboratory tests and differentiation of KD from other lymphadenitis due to lymphoma, systemic lupus erythematosus(SLE) and many viral disease. Materials and Methods: Thirty-four case of KD collected at Chonnam University Hospital in Kwang-Ju from 1992 through 2000 were evaluated with retrospective chart review. Results: The patients were consisted of 11 men and 23 women. All patients had tender or nontender cervical mass and fever was the most common associated symptom. The others was pain, weight loss, chills, cold sweating and headache et al. Multiple bilateral involvement of cervical lymphnodes was 25 cases(74%) and solitary involvement was 9 cases(26%). In laboratory tests, leukopenia was 12 cases(75%), elevated ESR 5 cases (34%) and elevated LDH 11 cases(69%). Conclusion: KD is necessary to differentiate from lymphoma and SLE, because of the different of therapeutic modality and prognosis. The diagnosis is established on the basis of histopathologic studies with excisional biopsy of lymph node.
Kim, Kyung-Ran;Kim, Jong Min;Kang, Ji-Man;Kim, Yae-Jean
Clinical and Experimental Pediatrics
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v.59
no.6
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pp.252-255
/
2016
Purpose: Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center. Methods: We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review was performed. Results: Fifteen episodes of P. jirovecii pneumonia in 14 patients were identified with median age of 8.3 years (range, 0.4-18.6 years). Among these patients, 11 patients had hematology-oncology diseases, 2 had primary immunodeficiency disorders (one with severe combined immunodeficiency and the other with Wiskott Aldrich syndrome), 1 had systemic lupus erythematosus and 1 received kidney transplant. Four patients were transplant recipients; 1 allogeneic and 2 autologous hematopoietic cell transplant and 1 with kidney transplant. The median absolute lymphocyte count at the diagnosis of P. jirovecii pneumonia was $5,156cells/mm^3$ (range, $20-5,111cells/mm^3$). In 13 episodes (13 of 15, 86.7%), patients were not receiving prophylaxis at the onset of P. jirovecii pneumonia. For treatment, trimethoprim/sulfamethoxazole was given as a main therapeutic agent in all 15 episodes. Steroid was given in 9 episodes (60%). Median treatment duration was 15 days (range, 4-33 days). Overall mortality at 60 days was 35.7% (5 of 14). Conclusion: Majority of our patients developed P. jirovecii pneumonia while not on prophylaxis. Continuous efforts and more data are needed to identify high risk patients who may get benefit from P. jirovecii pneumonia prophylaxis.
Kim, Soo Young;Choi, Young Seok;Kim, Young Ok;Woo, Young Jong
Clinical and Experimental Pediatrics
/
v.51
no.8
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pp.886-891
/
2008
Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report a rare childhood case of MCTD in a 7-year-old girl presenting initially with Raynaud's phenomenon, swollen hands, and ulceration of the right index finger tip followed by alopecia and arthritis during follow-up.
This study was designed to offer descriptive data for nursing intervention for relief of fatigue and pain, and to distinguish by the characteristic difference and the symptoms such as fatigue and pain on Ankylosing Spondylitis (AS), Fibromyalgia(FM), and Systemic Lupus Erythematosus(SLE) patients. The sample consisted of 92 patients(AS 29; FM 30; SLE 33) who visited H-University Rheumatism Hospital in Seoul. The data were collected by a structured questionnaire from May 1, 1999 to April 30, 2000. The results were as follows: Patients of 95% experienced fatigue in the last week and a fatigue score of three disease groups were above average. The fatigue score of FM patients was highest in the other disease, but which was not a statistically significant difference(F=1.417, p=.248). The mean score of AS and FM patients in pain was higher than the SLE patients, and there was the statistical significance among the three groups on pain (F=8.239, p=.001). There wasn't a statistical difference among three groups on coping wtih pain(F=1.451, p=.240). There wasn't any correlation between fatigue and pain in each disease (AS: r=.008, p=.966; FM: r=.328, p=.077; SLE: r=.237,p=.185). Therefore, morning stiffness and pain management during sleeping is needed through good body alignment in the AS patients. Adequate rest for fatigue and multiple coping strategies for pain maybe basic nursing intervention in FM and SLE. According to their fatigue rhythm, a regular exercise program is needed for rheumatic disease because they complained of fatigue above average and their fatigue was repeated better and worse only during the one week.
Fibromyalgia syndrome(FMS) is a chronic pain disorder of unknown etiology characterized by widespread musculoskeletal aches and pains, stiffness, and general fatigue, disturbed sleep and sleepiness. Frequently misdiagnosed, FMS is often confused with myofascial pain syndrome, polymyalgia rheumatica, polymyositis, hypothyroidism, metastatic carcinoma, rheumatoid arthritis (RA), juvenile rheumatoid arthritis, chronic fatigue syndrome, or systemic lupus erythematosus, any of which may occur concomitantly with FMS. The management of FMS often begins with a thorough examination and a diagnosis from a physician who is formally trained in tender-point/trigger-point recognition. An initial diagnosis provides reassurance to the patient and often reduces the anxiety and depression patterns associated with FMS. The most common goals in the management of FMS are (1) to break the pain cycle, (2) to restore sleep patterns, and (3) to increase functional activity levels. Because FMS is a multifactorial syndrome, it is likely that the best treatment will encompass multiple strategies. Medication with analgesics and antidepressants and also physiotherapy, are often prescribed and give some relief. The other most effective intervention for long-term management of FS to date is physical exercise. Physical therapists can instruct patients in the use of heat at home (moist hot packs, heating pads, whirlpools, warm showers or baths, and hot pads) to increase local blood flow and to decrease muscle spasm and tension. Also instruct patients in the proper use of cold modalities (ice packs, ice massage, and cool baths) to anesthetize localized areas of pain (tender points) and break the pain cycle. Massage and tender-point massage also may promote muscle relaxation. To date, the two most important interventions for the long-term management of FS are patient education and physical exercise. Lately, is handling FMS and Chronic Fatigue syndrome(CFS) together, becuase FMS and CFS are poorly understood disorders that share similar demographic and clinical characteristics. Because of the clinical similarities between both disorders it was suggested that they share a common pathophysiological mechanism, namely, central nervous system dysfunction.
Due to the development in computer mediated communication, there are many homepages on the web, which provide medical on health counseling and education. The internet is becoming increasingly important and essential in medicine. But little was known about what problems can be counseled and what difficulties are met during the computer mediated counseling. This study was performed to investigate the content and purpose of health counseling on the web. The data were collected from March 1st, 2002 to February 28th, 2003, from 199 questioners visited one rheumatic center homepage questionary/answering(Q/A) site. Most of the questioners consulted health problems of their own or their families. The 51.3% of them were diagnosed medically Rheumatoid arthritis was the most frequent disease and followed systemic lupus erythematosus, gout and ankylosing spondylitis. Most of the questioners needed to know treatment principles, diagnosing process, and information regarding the clinic. During the one-year period, the average number of users questioning was 29.4 cases and answering was 44.8 cases. Above findings suggest that computer mediated health counseling of rheumatic patients may be useful supplement of health education for them. Communication using internet has become popular, but this kind of approach needs a proper guideline. It is highly recommended that nurses be familiar with the e-health and e-medicine.
Journal of Physiology & Pathology in Korean Medicine
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v.21
no.6
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pp.1663-1669
/
2007
Acute transverse myelitis(ATM) is defined as an acute intramedullary dysfunction of the spinal cord, ascendng or static involving both halves of the cord and appearing without any history of previous neurological diseases due to traumatic accident, tumor of all kind, encephalitis and of course excluding all possible viral, bacterial and fungal infection. It is mainly characterized by acute motor disorder of both limbs in respect to which spinal segments are affected as well as sensory disorder and dysuria & dyschezia. The exact cause is unknown, however it is recently suggested that immunological factors are highly involved. It has been reported by several reliable sources that it is often accompanied by immunological diseases such as systemic lupus erythematosus(SLE). As treatments non steroid anti-inflammatory drugs(NSAIDS) are primarily recommended as to steroids, limited doses are injected only with the proper prescription from the physician. Operative methods are not options as traumatic accidents and tumors are excluded as factors. To enhance muscle strength and prevent articular contracture physical therapy and passive exercise is imperative. The following patient whose chief complaints were mainly about hypoesthesia of Rt. lower limb and stiffness of phalanges of both fingers as well as to weakness of lower extermity. Therefore it has been diagnosed as arthalgia syndrome. In oriental medicine factors such as wind evil heat-evil, dampness-heat evil, cold evil cause the arthalgia syndrome. In this case the patient was diagnosed as dampness-heat evil and herbal medicine Chunglijagam-Tang and Dong-Qi acupuncture was applied to treat bladder disorder.
The authors present the fine needle aspiration cytology (FNAC) cytologic findings of a case of extranodal marginal zone B cell lymphoma (MZBCL), which featured abundant plasma cells and eosinophilic histiocytes arising in both parotid glands. A 49-year-old female presented with palpable masses in both parotid glands. She had been suffering from systemic lupus erythematosus and rheumatoid arthritis. The lesions were evaluated by FNAC and smears showed a small number of clusters of oncocytic cells with abundant eosinophilic granular cytoplasm and small nuclei, intermixed with small to medium-sized lymphoid cells containing round to lobulated nuclei, which suggested Warthin's tumor. Some of lymphoid cells had a plasmacytoid appearance, and some scattered large cells contained a large amount of eosinophilic cytoplasm. Bilateral superficial parotidectomy was performed and a histopathologic study indicated MZBCL with abundant plasma cells, intermixed with eosinophilic histiocytes. The presence of oncocytic cells and a mixture of lymphoid and plasma cells indicates Warthin's tumor, but the cytologic features of a relatively monotonous small to medium-sized lymphoid infiltrate suggest the possibility of MZBCL in the clinical setting of an FNAC study performed on a patient suffering from a connective tissue disease.
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