• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.74-78
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• 2007

Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.15 no.2
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• pp.244-251
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• 2002

Introduction: SLE is autoimmune disease to appear systemic pathology in the connective tissue. Perniotic LE is sub-type of chronic dermal LE which causes purple macules on the terminal part of body-nose, ends of fingers, heals, calfs of the legs, elbows and knees. Objective: The purpose of this study was to estimate the efficiency of oriental medical treatment and management on the SLE and perniotic LE. Subject: We studied 2 patients who visited and hospitalized in Daejon University Oriental Hospital Dept. of Dermatology, with SLE and pemiotic LE from April 2002 till May 2002 Method: We treated them with herb medication, acupuncture and in addition to we consulted western medication if they were needed Results: In case of SLE patient, we considered it to be Yeol-Dok-Chi-Seong, so we priscriped Ga-Mi-So-Dok-Eum1, 2 , Yang-Dok-Baek-ho-Tang first. and till 22 days of hospitalization, we considered to be Gi-Eum-Yang-Heo and priscriped Ji-Hwang-Tang-Hab-Cheong-Sim-Yeon- Ja -Eum-Ga-Mi. but it wasn't adjust and high fever was reaccurred. And till 24 days of hospitalization we priscriped Gal-Geon-Hae-Gi-Tang, and becomed to 25 days, high fever was disappeared and the patient discharged and she has remained generally good condition. In case of Perniotic LE, at first we misdiagnosed her disease as a acral lentiginous melanoma, and we considered it to be Gan-Soo-Yeol-Li-Yeol- Jeung and priscriped Cheong-Pae-Sa-Gan-Tang and transferred her to another hospital, but she was diagnosed Perniotic LE Conclusion: Considering the above results, in case of SLE, in acute phase, we could treat her with oriental-western treatment, and in relief phase, we could treat her with only oriental treatment very efficiently. but in case of perniotic LE, we misdiagnosed it as a acral lentiginous melanoma, but we could experienced very rare perniotic LE case. And also further study is needed on the SLE, and perniotic LE, and we have to systematize of dyagnosis and treatment system.

• 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.

• Korean Journal of Psychosomatic Medicine
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• v.5 no.1
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• pp.89-96
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• 1997

This study was designed to determine whether cognitive impairment was evident in patients with SLE. Also, it aimed to examine the association of cognitive impairment with other clinical variables. The subjects consisted of 20 patients with mildly active SLE and 20 healthy controls. Methods : A total of 20 SLE patients and 20 normal controls completed a computerized neuropsychological test battery using Vienna Test System. These included Cognitrone test, Continuous attention test, Corsi block tapping test, Standard progressive matrices. Also, neuro-behavioral cognitive status examination was done. The symptom severity of depression was measured with Beck Depression Inventory, Hamilton Depression Rating Scale, and current medications were documented. Disease activity was rated using the SLE diasease activity index (SLEDAI). Results : SLE patients had poorer performance than normal controls on the tests of Cognitrone, attention, nonverbal IQ and memory, independent of age, education, disease activity, steroid use and depression status. Conclusion : Cognitive dysfunction was not uncommon in ambulatory SLE patients as measured by standardized neuropsychological tests. It seemed to occur independently of various clinical variables. These findings would suggest that cognitive dysfunction in SLE may be explained by reflecting subclinical central nervous system(CNS) involvement, rather than coexisting psychological distress due to chronic illness or side effect of medication.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.288-293
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• 2007

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

• Journal of Veterinary Clinics
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• v.13 no.1
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• pp.81-86
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• 1996

Systemic lupus erythematosus in a dog, suspected systemic lupus erythematosus in a dog, and autoimmune thrombocytopenic purpura hemmorrhagica in a dog are reported. A fice-year old, female Chihuahua (Case 1) showed initially hemorrhagic diathesis and purpura hemorrhagica. Afterward, it showed polymyositis and polyarthritis. LE-cell was demonstrated on LE-cell preparation trom blood. Systemic lupus erythematosus was diagnosed. This reponded well to the immunosuppressants, but developed iatrogenic Cushing syndrome and steroid hepatopathy. A two-and-half-year old, male toy poodle (Case 2) had chief complaint of red urine. Occult blood test for the urine sediment. This did not respond at all to antibiotics and carbazochrome, which is one of systemic coagulants. LE-cell was demonstrated on LE-cell preparation from blood. This responded relatively well to immunosupressants such as prenisolone, azathioprine and cyclophosphamide. systemic lupus erythematosus is suspected. A nine-year-and-three-month old, female Maltese (Case 3), which had history of congestive heart failure and ovariohysterectomy showed purpura hemorrhagica in the skin of chest. This had severe thrombocytopenia and leukocytosis. As prednisolone was administered before immunological examination or demonstration of LE-cell, it was impossible to diagnose whether purpura hemorrhagica developed as a member of systemic lupus erythematosus or independent of systemic lupus erythematosus. This responded well to prednisolone, and so autoimmune thrombocytopenic purpura hemorrhagica was diagnosed.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.80-87
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• 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.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.227-232
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• 2008

A 16-year-old girl presented with proteinuria and microscopic hematuria detected through mass urinary screening and was diagnosed as having suspected postinfectious glomerulonephritis by renal biopsy. However, heavy proteinuria did not respond to angiotensin converting enzyme inhibitor therapy. After 6 months, cervical lymphadenitis developed and a neck node biopsy showed subacute necrotizing lymphadenitis. After an additional 2 months, she developed facial erythema and thrombocytopenia. A repeat renal biopsy demonstrated lupus nephritis class IV. She was treated with pulse methylprednisolone(500 mg/day intravenously for 3 consecutive days) followed by oral deflazacort and monthly intravenous cyclophosphamide pulse(1 g/$m^2$) for 6 months. We report a case diagnosed as systemic lupus erythematosus(SLE) during medical follow-up after urinary screening.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.875-881
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• 2006

Purpose : The aims of this study were to analyze the clinical characteristics of children with Kikuchi's disease(KD) at a medical center and to investigate the etiologic role of human herpesvirus 8(HHV 8) or Epstein-Barr virus(EBV) in children with KD. Methods : Twenty six children who were diagnosed as KD between Jan. 1998 and Dec. 2005 were included. Medical records were reviewed on the clinical characteristics of children with KD. Follow up data were collected by chart review and telephone contact. Polymerase chain reaction(PCR) was performed in order to detect HHV 8 DNA, and in situ hybridization(ISH) was perfomed in order to detect EBV RNA from 20 lymph node tissues. Results : There were 15 girls and 11 boys with a mean age of 13 years. Posterior cervical lymph nodes were involved in 72 percent(18/25) of the patients. Extracervical lymphadenopathy was associated in one patient. Fever was an associated symptom in 31 percent(8/26) of the patients. Leukopenia was observed in six (46 percent) patients. The cervical lymphadenopathy usually resolved spontaneously within 6 months. Only one patient had a recurrence of lymphadenopathy with fever during follow-up. No children with KD in our series developed systemic lupus erythematosus. HHV 8 DNA was not amplified by nested PCR in any of the cases, and all cases were negative for EBV RNA by ISH. Conclusion : KD should be differentiated as a cause of cervical lymphadenopathy in children. HHV 8 and EBV may not play major causative roles in KD in children.