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권순효 ( Soon Hyo Kwon ),나선재 ( Sun Jae Na ),염꽃보라 ( Kkot Bora Yeom ),허선영 ( Sun Young Huh ),나정임 ( Jung Im Na ) 대한피부과학회 2011 대한피부과학회지 Vol.49 No.8
Multiple congenital melanocytic nevi (MCMN), defined as the distribution of more than three small- or medium-sized congenital melanocytic nevi (CMN) on the body without a giant CMN, is a rare disease comprising about 4% of patients with CMN. Because MCMN accompanies neurodevelopmental delay, including seizures in 25% of patients as well as the risk of malignant melanoma, it must be carefully followed-up. We report a case of MCMN with developmental delay in a 19-month-old Korean boy. He had a history of febrile seizure when he was 18 months old. He showed a speech delay after the 1-year-follow up, even though there was no evidence of neurocutaneous melanosis (NCM) on brain magnetic resonance imaging (MRI) at the first visit. As MRI has a low sensitivity for detecting NCM in patients with MCMN older than 4-months, close neurodevelopmental assessments should be considered to provide a chance for early rehabilitation. (Korean J Dermatol 2011;49(8):753∼756)
만성 신부전 환자에서 은단 복용과 동반되어 발생한 전신적 은 중독증
길효욱 ( Gil Hyo Ug ),이준영 ( Lee Jun Yeong ),권순효 ( Kwon Sun Hyo ),조원영 ( Jo Won Yeong ),박종근 ( Park Jong Geun ),양종오 ( Yang Jong O ),이은영 ( Lee Eun Yeong ),홍세용 ( Hong Se Yong ) 대한신장학회 2004 Kidney Research and Clinical Practice Vol.23 No.2
Generalized hyperpigmentation has a wide range of causes, including endocrine diseases, including endocrine diseases, uremia, drugs, and heavy metals. Cutaneous pigmentation observed in 70% of uremic patients on hemodialysis. However, it is clinically difficult to define the exact cause of hyperpigmentation in chronic renal failure patients. We experienced a case of cutaneous hyperpigmentation caused by generalized argyria in chronic hemodialysis patient. A 38-year-old male patient presented with slowly progressive blue-black discoloration of skin. He had been on maintenance hemodialysis for 5 yars and took sliver pills 36 g daily during 2.5 years to relieve thirst. The other source of silver exposure could not be determined. Pathologic examination of skin biopsy specimen showed focal deposits of fine, uniform, brownish granules along the basement membrane zone of hair in the dermis, which is characteristic findings of argyria. The silver deposits were also confirmed by increase in serum silver level and silver amount in liver and skin tissue. Argyria should be suspected in chronic hemodialysis patients presenting with a diffuse bluish-gray discoloration of the skin and nails and evaluated carefully by skin biopsy with quantitation of tissue silver level. (Korean J Nephrol 2004;23(2):345-348)
최재우 ( Jae Woo Choi ),권순효 ( Soon Hyo Kwon ),염꽃보라 ( Kkot Bora Yeom ),허선영 ( Sun Young Huh ),조광현 ( Kwang Hyun Cho ),송계용 ( Kye Yong Song ),윤상웅 ( Sang Woong Youn ),박경찬 ( Kyoung Chan Park ),나정임 ( Jung Im Na 대한피부과학회 2011 대한피부과학회지 Vol.49 No.10
Mycosis fungoides (MF) is the most frequent cutaneous T cell lymphoma (CTCL). Since the major tumor cell of MF is the helper T cell, positive markers are usually CD3, CD4 and CD45RO. Some MFs show CD30 positivity and the major differential diagnosis for MF with CD30 positivity includes transformed MF and MF concurrent with primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. As each disease shows a different prognosis, an exact diagnosis is crucial for proper treatment. We now report a case of 44-year-old male patient with mycosis fungoides which developed several papules on preexisting MF patches. On biopsy of the newly formed papules, CD 30 positive cells were observed and the histologic features were consistent with lymphomatoid papulosis. Both the lesions of lymphomatoid papulosis and MF responded well to narrow band ultraviolet B phototherapy. (Korean J Dermatol 2011;49(10):931∼935)
Erythropoietin 항체에 의해 유발된 진성 적혈구 무형성증 (Pure Red-cell Aplasia)
양종오 ( Yang Jong O ),길효욱 ( Gil Hyo Ug ),권순효 ( Kwon Sun Hyo ),최영진 ( Choe Yeong Jin ),이은영 ( Lee Eun Yeong ),홍세용 ( Hong Se Yong ) 대한신장학회 2004 Kidney Research and Clinical Practice Vol.23 No.3
Recombinant human erythropoietin (rHuEPO) is extensively used in anemic patients on dialysis. We experienced a case of pure red-cell aplasia (PRCA) caused by anti-rHuEPO Antibody. A 48-year-old male with CRF was placed on chronic hemodialysis. He developed severe anemia (Hb 6.6 g/dL) without any apparent blood loss. A transfusion was given and the rHuEPO dose was increased to 12,000 U/week SC. A bone marrow study was carried out. Anti-rHuEPO antibodies were positive. rHuEPO therapy was stopped. No improvement in the anemia was apparent. Although very rare, anti-rHuEPO an tibodies should be evaluated in patients with severe anemia without obvious etiology. (Korean J Nephrol 2004;23(3):528-531)
박은경 ( Eun Kyung Park ),윤영섭 ( Yeong Seop Yun ),권순효 ( Soon Hyo Kwon ),전진석 ( Jin Seok Jeon ),노현진 ( Hyun Jin Noh ),이선영 ( Sun Young Lee ),진소영 ( So Young Jin ),한동철 ( Dong Cheol Han ) 대한신장학회 2008 Kidney Research and Clinical Practice Vol.27 No.1
서론: 최근 비만과 대사증후군을 만성콩팥병의 발병과 진행의 위험인자들로 인식하고 있다. 비만환자에서 사구체비대와 국소분절 사구체경화증이 발견되어, 비만관련 사구체병증의 구조와 기능관계에 대한 연구의 필요성이 대두되고 있다. 국내에서는 아직 국내보고가 없었던 이 질병의 병리학적 특성을 분석하고, 다른 임상인자들과의 관련성을 분석하였다. 방법: 비만은 BMI 25 kg/m2 이상으로 정의하였다. 비만관련 사구체병증은 비만환자에서 국소분절 사구체경화증과 사구체비대 혹은 사구체비대만 관찰되는 경우로 정의하였다. 비만관련 사구체병증으로 진단된 14예를 대상 환자들과 연령을 맞춘 얇은 기저막병 환자를 대조군으로 하여 임상자료 및 병리학적 소견과 사구체 크기를 비교분석하였다. 사구체 크기는 각 환자에서 가장 큰 사구체 5개의 장축을 측정하여 평균을 내었다. 또한 각 임상인자들과 사구체 크기와의 관련성을 분석하였다. 결과: 모든 비만 환자에서 신증후군이나 함요부종은 관찰되지 않았다. 사구체의 크기는 비만군에서 240±21 μm, 대조군에서는 193±23 μm로 비만군에서 사구체 크기가 의미있게 증가되어 있었다 (p=0.001). 대상환자 모두에서 사구체비대가 관찰되었고 이 중 절반에서 국소분절 사구체경화증이 동반되었다. 과반수 이상의 비만 환자에서 경도의 세관 위축과 간질성 섬유화, 세동맥경화를 보였다. 비만 환자 중 사구체 비대만 보인 군과 비교하여 국소분절 사구체경화증을 보인 군에서 수축기 고혈압과 세관 간질성 섬유화증이 심했다. 수축기 혈압과 허리둘레가 사구체의 크기에 영향을 미치는 독립적인 위험인자로 나타났다. 결론: 경한 비만에도 불구하고 대부분에서 사구체비대 혹은 국소분절 사구체경화증이 관찰되었으나 임상양상은 경미하였다. 이는 비만환자에서 사구체질환에 대한 임상적인 주의가 필요하다는 것을 지적하고 있다. Purpose: Recently, obesity with metabolic syndrome is considered as an important risk factor in the development and progression of chronic kidney disease (CKD). Glomerulomegaly and focal segmental glomerulosclerosis (FSGS) are found in the obese patients, suggesting that investigation of structuralfunctional relationship in the obesity-related glomerulopathy (ORG) is needed to prevent CKD. Thus, we report here clinical and pathologic characteristics of ORG and its association with other clinical variables. Methods: Obesity was defined by body mass index >25 kg/m2 and ORG morphologically by FSGS and glomerulomegaly or glomerulomegaly alone. Clinicopathologic findings and glomerular sizes of ORG (14 cases) were compared with age-matched controls with thin basement membrane disease. Multiple variable analysis was performed between glomerular size and clinical variables. Results: There was no nephrotic syndrome or pretibial pitting edema in all obese patients. Mean glomerular diameter was increased in obese patients compared to controls (240±21 μm vs 197±21 μm, p=0.001). Seven cases had lesions with FSGS with glomerulomegaly and seven cases glomerulomegaly alone. Mild tubular atrophy, interstitial fibrosis and arteriolosclerosis were observed in more than half of patients. In obese patients, seven patients with FSGS had more elevated systolic blood pressure and tubular interstitial fibrosis compared to patients with glomerulomegaly only. Patients` systolic blood pressure and waist circumference were independent risk factors influencing the glomerular size in obese patients. Conclusion: FSGS or glomerulomegaly are prominent even in the mild obesity with insignificant clinical symptoms. This indicates that the clinical attention to glomerular disease is needed in obese patients.