Journal of the Korean Pediatric Society 1985;28(4):353-371.
Published online April 30, 1985.
Clinico-pathologic Study Glumerulonephritis with Crescents in 25% or more of Glomeruli in Children.
Hoan Jong Lee, Kwang Wook Ko
Department of Pediatrics, College of Medicin,Seoul National University
25%以上의 絲球體에서 半月像 (crescents) 이 동반된 小兒 絲球體腎炎의 臨床-病理學的 考察
이환종, 고광욱
서울대학교 의과대학 소아과학교실
Abstract
We experienced 17 pediatric patients of glomerulonephritis with crescents in 25% or more of glomeruli, who were admitted to Department of Pediatrics, Seoul National University Hospital, from Jan. ’79 to Dec. ’84. A review of clinical and pathologic findings revealed as follows; 1)The 17 patients were 10 cases of Henoch-Schdnlein nephritis, 3 cases of idiopathic nephrotic syndrome which were due to diffuse proliferative GN in 2 and membranoproliferative GN in 1, 1 case of infective endocarditis complicated by diffuse proliferative GN, 2 cases of crescentic GN related to streptococcal infection, and 1 case of crescentic GN of unknown origin. 2)Ages of onset were between 9 and 14 10/12 years (mean; 12.26±1.80 years), and male to female ratio was 8 : 9. 3)Initial evaluation revealed URI-like symptoms in 6 cases and chicken pox in one before onset, hematuria in all (gross hematuria in 14, microscopic in 3), edema in 15, and hypertension in 6. 4)14 patients fell into the criteria of nephrotic syndrome (serum albumin 2.5 mg% daily urine protein 40 mg/kg/m2). Serum creatinine concentrations at the time of biopsy were over 1.5 mg% in 13 cases, 3 of them were over 5 mg%. Selectivity indices were over 0.2 in 10 out of 11 patients determined. 5)Dipyridamol, prednisolone, and/or cytoxan were administered to 3 patients of H-S nephritis with 62%, 43%, 83% crescents, followed by normalization of serum creatinine in 2 patients by 2 weeks and 3 months, whose initial creatinine concentrations were 1.8, 2.6 mg%. Infective endocarditis with 58% crescents was treated with antibiotics only, and the initially elevated serum creatinine(3.4 mg%) was normalized in 7 weeks. Methylprednisolone pulse therapy was tried to four cases such as 2 cases of H-S nephritis with 89% and 100% crescents, 1 case of poststreptococcal GN with 100% crescents, and membranoproliferative GN with 75% crescents. Increase of creatinine clearance(17.6±11.8->39.9±28.5 ml/min/1.73 m2) and reduction of serum creatinine(5.4+2.9-*2.0±l. 5 mg%) were noted in all four patients, by 2 to 4 weeks, but renal functions subsequently worsened 1 week; 2,3,25 months later and all progressed to renal failure. 6)Out of 16 patients with current follow-up study, 3 patients died of renal failure at 1,2 and 7 months after biopsy, and the periods from biopsy to the last evaluation were 5~42 months(20.8±10.7 months) in the remaining 13 patients. Out of 9 patients of H-S nephritis, 4 cases with 78%, 92%, 89%, 90% crescents progressed to renal failure, of whom renal transplantations were susccessfully performed in 2, and the remaining 1 case with 89% crescents and 4 cases with less than 75% crescents are stable with proteinuria. Of 7 patients other than H-S nephritis, 2 progressed to renal failure, 1 in mild renal insufficiency, and 4 are stable with proteinuria. 7)The periods from onset to renal failure (time to creatinine 5 mg%) were 2~37 months (mean 13±11.6 months). 8)2 out of 4 patients with crescents in 25~50% of glomeruli, 3 out of 5 with crescents in 50-75%, and all of 8 with crescents over 75% had renal insufficiency (creatinine 1.5 mg%) at the time of biopsy. 9)On follow-up, of 8 patients with crescents in less than 75% of glomeruli, only one had mild renal insufficiency, and of 8 patients with crescents over 75%, 6 progressed to renal failure.
Key Words: Crescentic glomerulonephritis, Honoch-Schonlein nephritis, methylprednisolone pulse therapy




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