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      KCI등재 SCOPUS SCIE

      Fanconi 증후군 치료 후 골동통이 호전된 다발성 골수종 1예 = A Case of Multiple Myeloma with Ameliorated Bone Pain after Treatment of Adult Fanconi Syndrome

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      https://www.riss.kr/link?id=A104779284

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      다국어 초록 (Multilingual Abstract)

      Adult Fanconi syndrome is characterized by variable abnormalities caused by renal proximal transport defects, resulting in glycosuria, aminoaciduria, bicarbonaturia, uricosuria and phosphaturia. A 57-year- old man with κ-light chain multiple myeloma, undergoing chemotherapy with prednisolone and melphalan for 17 month, was admitted with spontaneous femoral neck fracture and was consulted due to polyuria and refractory metabolic acidosis immediately after hemiarthroplasty. The laboratory values showed normal anion gap metabolic acidosis with normal urinary anion gap, hypokalemia, hypouricemia, hypophosphatemia at the time of consultation. After partial correction of acidemia, the fractional excretion of HCO3- was 11.9%, it was interpreted as proximal renal tubular acidosis. 24-hour urine collection showed increased level of excretion for most aminoacids. Diffuse osteopenia and multiple compression fractures on spine were detected on radiological examinations. Also, osteoporosis and osteomalacia was suggested during his clinical course.
      After the diagnosis of Fanconi syndrome was made, treatment was started with sodium bicarbonate, potassium citrate, calcitriol, calcium carbonate along with phosphate rich diet. Laboratory abnormalities were corrected and refractory multiple bone pain was ameliorated with these treatment.
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      Adult Fanconi syndrome is characterized by variable abnormalities caused by renal proximal transport defects, resulting in glycosuria, aminoaciduria, bicarbonaturia, uricosuria and phosphaturia. A 57-year- old man with κ-light chain multiple myeloma,...

      Adult Fanconi syndrome is characterized by variable abnormalities caused by renal proximal transport defects, resulting in glycosuria, aminoaciduria, bicarbonaturia, uricosuria and phosphaturia. A 57-year- old man with κ-light chain multiple myeloma, undergoing chemotherapy with prednisolone and melphalan for 17 month, was admitted with spontaneous femoral neck fracture and was consulted due to polyuria and refractory metabolic acidosis immediately after hemiarthroplasty. The laboratory values showed normal anion gap metabolic acidosis with normal urinary anion gap, hypokalemia, hypouricemia, hypophosphatemia at the time of consultation. After partial correction of acidemia, the fractional excretion of HCO3- was 11.9%, it was interpreted as proximal renal tubular acidosis. 24-hour urine collection showed increased level of excretion for most aminoacids. Diffuse osteopenia and multiple compression fractures on spine were detected on radiological examinations. Also, osteoporosis and osteomalacia was suggested during his clinical course.
      After the diagnosis of Fanconi syndrome was made, treatment was started with sodium bicarbonate, potassium citrate, calcitriol, calcium carbonate along with phosphate rich diet. Laboratory abnormalities were corrected and refractory multiple bone pain was ameliorated with these treatment.

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      참고문헌 (Reference)

      1 Engle RL Jr, "The adult Fanconi syndrome. II. Review of eighteen cases" 22 : 13-23, 1957

      2 Roth KS, "The Fanconi syndrome and mechanisms of tubular transport dysfunction" 20 : 705-716, 1981

      3 Lin J, "Renal monoclonal immunoglobulin deposition disease : the disease spectrum" 12 : 1482-1492, 2001

      4 Sirota JH, "Renal function studies in a adult subject with the Fanconi syndrome" 16 : 138-152, 1954

      5 DeFronzo RA, "Renal function in patients with multiple myeloma" 57 : 151-166, 1978

      6 Clarke BL, "Osteomalacia associated with adult Fanconi's syndrome: clinical and diagnostic features" 43 : 479-490, 1995

      7 Bell NH, "Osteomalacia and rickets. In Principles and Practice of Endocrinology" Philadelphia, J. B. Linppincott 1990

      8 Aucouturier P, "Monoclonal Ig L chain and L chain V domain fragment crystallization in myeloma-associated Fanconi's syndrome" 150 : 3561-3568, 1993

      9 Guan S, "Inhibition of Na-K-ATPase activity and gene expression by a myeloma light chain in proximal tubule cells" 47 : 496-501, 1999

      10 Rao DS, "Hypophosphatemic osteomalacia and adult Fanconi syndrome due to light-chain nephropathy : Another form of oncogenous osteomalacia" 82 : 333-338, 1987

      1 Engle RL Jr, "The adult Fanconi syndrome. II. Review of eighteen cases" 22 : 13-23, 1957

      2 Roth KS, "The Fanconi syndrome and mechanisms of tubular transport dysfunction" 20 : 705-716, 1981

      3 Lin J, "Renal monoclonal immunoglobulin deposition disease : the disease spectrum" 12 : 1482-1492, 2001

      4 Sirota JH, "Renal function studies in a adult subject with the Fanconi syndrome" 16 : 138-152, 1954

      5 DeFronzo RA, "Renal function in patients with multiple myeloma" 57 : 151-166, 1978

      6 Clarke BL, "Osteomalacia associated with adult Fanconi's syndrome: clinical and diagnostic features" 43 : 479-490, 1995

      7 Bell NH, "Osteomalacia and rickets. In Principles and Practice of Endocrinology" Philadelphia, J. B. Linppincott 1990

      8 Aucouturier P, "Monoclonal Ig L chain and L chain V domain fragment crystallization in myeloma-associated Fanconi's syndrome" 150 : 3561-3568, 1993

      9 Guan S, "Inhibition of Na-K-ATPase activity and gene expression by a myeloma light chain in proximal tubule cells" 47 : 496-501, 1999

      10 Rao DS, "Hypophosphatemic osteomalacia and adult Fanconi syndrome due to light-chain nephropathy : Another form of oncogenous osteomalacia" 82 : 333-338, 1987

      11 Orfila C, "Fanconi's syndrome, kappa light-chain myeloma, non-amyloid fibrils and cytoplasmic crystals in renal tubular epithelium" 11 : 345-349, 1991

      12 Maldonado JE, "Fanconi syndrome in adults. A manifestation of a latent form of myeloma" 58 : 354-364, 1975

      13 Batuman V, "Effect of myeloma light chains on phosphate and glucose transport in renal proximal tubule cells" 17 : 294-300, 1994

      14 Truong LD, "Cytoplasmic crystals in multiple myeloma-associated Fanconi's syndrome. A morphological study including immunoelectron microscopy" 113 : 781-785, 1989

      15 Leboulleux M, "Aucouturier P: Protease resistance and binding of Ig light chains in myeloma-associated tubulopathies" 48 : 72-79, 1995

      16 Sewell RL, "Adult Fanconi syndrome progressing to multiple myeloma" 37 : 1256-1258, 1984

      17 Ma CX, "Acquired Fanconi syndrome is an indolent disorder in the absence of overt multiple myeloma" 104 : 40-42, 2004

      18 Yonemura K, "Acquired Fanconi syndrome associated with IgG kappa multiple myeloma : observations on the mechanisms of impaired renal acid excretion" 12 : 1251-1253, 1997

      19 Kim YT, "A case of adult fanconi syndrome in kappa-light chain myeloma" 48 : 403-407, 1995

      20 Kim CH, "A case of adult fanconi syndrome and osteomalacia associated with κ-light chain monoclonal gammopathy" 13 : 99-107, 1998

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      학술지 이력

      학술지 이력
      연월일 이력구분 이력상세 등재구분
      2023 평가예정 해외DB학술지평가 신청대상 (해외등재 학술지 평가)
      2020-01-01 평가 등재학술지 유지 (해외등재 학술지 평가) KCI등재
      2011-11-29 학술지명변경 한글명 : The Korean Journal of Nephrology -> Kidney Research and Clinical Practice
      외국어명 : 미등록 -> Kidney Research and Clinical Practice
      KCI등재
      2010-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2008-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2007-02-22 학술지명변경 한글명 : 대한신장학회지 -> The Korean Society of Nephrology KCI등재
      2007-02-22 학술지명변경 한글명 : 대한신장학회지 -> The Korean Journal of Nephrology KCI등재
      2005-01-01 평가 등재학술지 선정 (등재후보2차) KCI등재
      2004-01-01 평가 등재후보 1차 PASS (등재후보1차) KCI등재후보
      2002-01-01 평가 등재후보학술지 선정 (신규평가) KCI등재후보
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      학술지 인용정보

      학술지 인용정보
      기준연도 WOS-KCI 통합IF(2년) KCIF(2년) KCIF(3년)
      2016 0.21 0.21 0.17
      KCIF(4년) KCIF(5년) 중심성지수(3년) 즉시성지수
      0.14 0.1 0.422 0.11
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