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3 cases of minimal change with Immunoglobulin A deposits
분당서울대학교병원
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정광록, 진호준
Introduction: Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephropathy (GN). Clinical manifestations are diverse from isolated hematuria to rapidly progressing GN. However, it is rare with IgAN constituting nephrotic range proteinuria and uncommonly associated with po- docyte foot process effacement which is the characteristic feature of minimal change disease (MCD). Case Report: Of total 1,600 kidney pathology re- ports from May 2003 to September 2017 in Seoul National University Bundang Hospital, only 3 has been identified as MCD with mesangial IgA deposits.
A 49-year-old female was present with proteinuria without any clinical symptom. Initial urinalysis reported proteinuria (3+), hematuria (RBC 10-19/HPF), and urine spot protein/creatinine was 1.24g/g. Biopsy identified focal slight mesangial hyper-
cellularity, IgA deposits in immunofluorescence (IF), and diffuse podocyte foot process effacement in electron microscopy (EM). Her proteinuria did not reach nephrotic range and spontaneously im- proved to 0.7g/d and there was no further aggravation. Another 49-year-old female was also present with proteinuria without symptoms. Initial urinalysis reported proteinuria (3+), hematuria (RBC ≥ 100/HPF), and urine spot protein/creatinine was 4.01g/g. Biopsy described minimal histologic change, IgA and C3 deposits in IF, and focal moderate podocyte foot process effacement in EM (Figure1). Spontaneous resolution was achieved without further disease relapse. Lastly, a 56-year-old male experienced 2+ lower extremity edema and uri-
nalysis indicated proteinuria (3+), hematuria (RBC 5-9/HPF), and urine spot protein/creatinine was 24.4g/g. Biopsy identified minimally increased me- sangial cellularity, IgA and C3 deposits in IF, and dif- fuse podocyte foot process effacement in EM.
Glucocorticoid therapy successfully achieved com- plete remission. However, 4 time relapses during ste- roid tapering were observed, so cyclophosphamide was added. After then, he no longer visited clinic.
Discussion: Present cases even with mesangial IgA deposits exhibit distinctive traits of MCD in terms of steroid-response, clinical course, and frequent ten- dency of relapse.
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Treatment of sodium thiosulfate on subcutaneous and vascular calcification in ESRD patients
조선대학교병원 내과학교실
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김수영, 김현우, 신병철, 김현리, 정종훈
Calcific uremic arteriolopathy is a severe complication of end-stage renal disease (ESRD) characterized by subcutaneous calcification of the small arteries and tissue necrosis. A case of a 30-year-old female was ESRD due to diabetic nephropathy and prescribed peritoneal dialysis treatment regimen for four years. At her monthly clinic visit, the patient complained of burning sensation in the palm of right hands with limited wrist motor movement due to edema.
A presumptive diagnosis of subcutaneous and vascular calcification led to hospital admission with confirmation by X-ray and MRI of her wrist. The patient was continued to peritoneal dialysis with low calcium diet, aggressive reduction in phosphorus, diet counseling, use of cinacalcet, and three months of in- travenous sodium thiosulfate infusion three times for week. Another case of a 30-year-old female was ESRD due to chronic glomerulonephritis prescribed peritoneal dialysis treatment regimen for three years. She was complained the palpable mass in the second finger of right hand and right heel skin defect, and diagnosed subcutaneous calcification and calciphylaxis. The patient was switched to hemodialysis with low calcium dialysate and three months of in- travenous sodium thiosulfate infusion with hemodialysis treatments. The patient's condition improved with resolution of symptoms and radiologic findings.
Keywords: Sodium thiosulfate, calcification, end-stage renaldisease