• Childhood Kidney Diseases
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• v.5 no.2
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• pp.219-224
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• 2001

Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)

• Tuberculosis and Respiratory Diseases
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• v.50 no.6
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• pp.732-739
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• 2001

Systemic lupus erythematosus frequently has thoracic involvement among connective tissue diseases. One of the pleuropulmonary manifestations is diffuse interstitial lung disease including nonspecific interstitial pneumonia(NSIP). NSIP is a newly classified disease among interstitial lung diseases. Systemic lupus erythematosus has a better prognosis than usual interstitial peumonia(UIP) and responds well to steroids. In this report, a 34 year-old woman who complained of a dry cough, and exertional dyspnea for 2 months is described. The chest X-ray showed fine reticular opacities and a mild honeycomb appearance in both basal lungs. High resolution computed tomography(HRCT) showed bilateral patchy areas of ground-glass attenuation and a mild honeycomb appearance in the subpleural of both the lower and the middle portion of the lung fields. An open lung biopsy showed prominent lymphocytic interstitial inflammation and fibrosis with small areas with a honeycomb appearance. This case was diagnosed as NSIP associated with systemic lupus erythematosus and was managed with oral steroids. Here we report a case of nonspecific interstitial pneumonia associated with systemic lupus erythematosus confirmed by HRCT and an open lung biopsy with a review of the relevant literature.

• The Journal of Korean Medicine
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• v.16 no.1 s.29
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• pp.51-70
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• 1995

To find the cause, pathology, oriental presciptions for SLE(systemic lupus erythematous) in the field of oriental medicine, we studied the clinical reports. The results were obtained as follows: 1. SLE can' be thought to be a category of chronic fatigue, numbness, yang poison' & erythema. 2. The chief cause of SLE could be a febrile poison and the lack of physiological fluid and the mechanism was that febrile poison attacted area of circulation and blood and injuried the physiological fluid to arise edema and clotted blood. 3. The therapy of SLE was chiefly clarifying heat and toxiciding in acute stage while reinforcing kidney and liver and supplementing Yin & clarifying in chronic stage, sometimes supplementing Qi, eliminating clotted blood, excuding wind and draining water could be applied. 4. The therapy for SLE nephritis was shown to be increasing yin and clarifying heat with toxiciding, reinforcing spleen and kidney, draining water, supplementing yin & Qi etc. The chief prescriptions were Seogakjihwangtang(犀角地黃湯), Wookakjihwangtang(牛角地黃湯), yeechihwan(二至丸), Daepowonjeon(大補元煎), Daepoeumhwan(大補陰煎), Kikukjihwangtang(杞菊地黃湯), Yookmijihwangtang(六味地黃湯) which indicates Rhemanniae radix added prescrition could be used chiefly for the treatment of SLE.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.89-94
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• 1992

A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.6 no.3
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• pp.464-476
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• 1999

This study was conducted between October 1, 1998, and December 31, 1998, in order to provide basic data to develop better self-management educational programs for systemic lupus erythematosus (lupus) patients. In the study, the extent of each patients knowledge on the disease and their learning needs about it were examined for 100 lupus inpatients and outpatients at the Kangnam St. Marys Hospital. Data were collected by using questionnaires to those patients. Frequencies, percentage, average, standard deviation, t-test, ANOVA, and Duncans multiple range test were examined through the SAS program. The result of study follows : 1) The average score of patients knowledge on the disease was 15.7 (range, 7-20; standard deviation, 2.74). Subjects scored higher as far as the extent of their knowledge in the following categories : management of daily life, diet, characteristics of the disease, risk factors, and medication. Two categories shared the highest percentage of correct answers : 99% of subjects correctly identified that fatigue and stress aggravate symptoms of lupus and symptoms of lupus vary among individuals in range and type. However, when subjects were asked if patients should get a regular examination by an ophthalmologist every 4-6 months while they are on medication to treat rashes, lupus can be completely cured, and contraceptives that include a female hormone (estrogen) are good as contraceptive methods, the percentages of correct answers were low (32%, 31%, and 20%, respectively). In terms of subjects knowledge about the disease by their general characteristics, single subjects had more knowledge about the disease than married subjects did (t=2.14, p=0.0353). The extent of knowledge also varied by monthly income (F=4.96. p=0.0097). Those with more formal education had more knowledge about lupus than those who had less formation education did (t=2.95, 0.0039). Additionally, those who were satisfied with their education about the disease had better knowledge about it than those who were dissatisfied with their education did (t=2.71, p=0.0090). 2) The extent to which lupus patients wanted to be educated about the disease was, on average, 64.5 (range, 46-75; standard deviation, 7.91). Areas for which patients requested education are listed here in order : risk factors, sexual lives and patients associations. Patients who had been hospitalized demonstrated wanting more education than those who had not been did(t=3.73, p=0.0003). The extent to which they wanted the information was different by the number of educational sessions they had (F=3.98, p=0.0249). In conclusion, the results above would be considered when the education programme is planned for SLE patients.

• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.96-102
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• 1999

A 40-year-old female presented with fever and cough which had lasted for 3 months. Three years previously systemic lupus erythematosus had been diagnosed, and at that time 10mg of prednisone per day was prescribed. Crackles were heard at the base of the lung and her chest roentgenogram revealed diffuse infitrates in both lower lung fields. Despite of empirical antibiotics and antituberculous medication consisting of isoniczid, rifampicin and ethambutol, the patients condition deteriorated. We performed open lung biosy and numerous nodules with necrosis as well as acid fast bacilli were observed. After biochemical tests and sequencing, the organism was identified as Mycobacterium abscessus. The patient was treated with imipenem and amikacin ; patient's condition, as indicated by both symptoms and roentogenogram, subsequently improved.

• Journal of Oral Medicine and Pain
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• v.32 no.1
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• pp.69-79
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• 2007

Condylar resroption, or condylysis can be defined as progressive alteration of condylar shape and decrease in mass. Although the cause is unknown, condylar resorption has been assocated with rheumatoid arthritis, systemic sclerosis, systemic erythematous, steroid usage, orthodontic treatment and orthognathic surgery. In most case, however, there is no identifiable precipitating event. Hence the term is idiopathic condylar resorption. With condylar resorption, the bone loss is resulting in a loss of posterior support in the involved condyle and the mandible can then shift. As a result, the most patients exhibit occlusal change, openbite, retrognathism, and a decrease in posterior face height. This article reports cases of condylar resorption. And the author will review factors capable of changes of condyle, pathogensis and management of conylar resorption.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

• The Journal of Korean Medicine
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• v.29 no.1
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• pp.67-84
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• 2008

Objective : The main purpose of this study was to evaluate the effect of Jasinwhalhyul-tang (Zishenhuoxue-tang, JWT) on MRL/MpJ-Ipr/Ipr mouse model with systemic lupus erythematosus. Methods: The effect of JWT on MRL/MpJ-Ipr/Ipr mice that have autoimmune disease similar to SLE in humans was evaluated after JWT per oral in the present study. Mice were administered with Jasinwhalhyul-tang (Zishenhuoxue-tang, JWT) (80 or 400mg/kg) or distilled water for control group from experimental week 10 for 22 weeks. Results : The amount of erythematosus skin lesion and proteinuria were significantly decreased. The size and weight of cervical lymph nodes and spleen were significantly reduced. The ratio between activated $CD3^+CD69^+$ T-cells and undifferentiated $CD3^+CD4^-CD8^-$ T-cells in lymph nodes, spleen and kidney was effectively reduced. The gene expression of TGF-$\beta$ in spleen and kidney was increased. The amount of anti-dsDNA IgG in blood was decreased. The gene expression of TGF-$\beta$ in normal mouse spleen cells was increased depending on concentration by treatment of with T cell stimulating agent. In the histological examination of skin and kidney, the amount of infiltration of immune cells involved in the inflammatory response was decreased. Conclusions : According to the above results, JWT should be considered as an applicable therapeutic agent to SLE in clinical practice. Further research is required to investigate other efficacies of JWT on SLE.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.886-891
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• 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.