PMID- 30620818
OWN - NLM
STAT- MEDLINE
DCOM- 20190329
LR  - 20190329
IS  - 0385-2385 (Print)
IS  - 0385-2385 (Linking)
VI  - 58
IP  - 7
DP  - 2016
TI  - [A case of renal Fanconi syndrome due to Bence Jones' protein K-type multiple 
      myeloma].
PG  - 1088-1094
AB  - A 60-year-old man, who had been treated for chronic kidney disease and chronic 
      hepatitis B infection, was referred to our hospital following presentation with 
      thoracic bone pain and exacerbation of proteinuria and hematu- ria. On admission, 
      laboratory test results showed evidence of hypophosphatemia, glucosuria and 
      elevated levels of both urinary NAG and 62MG.The patient was diagnosed with 
      Fanconi syndrome based on findings indicating the presence of pan-aminoaciduria, 
      elevated urinary excretion of uric acid and an increased phosphorus reabsorption 
      rate. Furthermore, bone scintigraphy showed increased multiple symmetric uptake 
      of radiotracer in both sides of the ribs, leading to the diagnosis"of 
      hypoposphatemia-related osteomalacia with renal Fanconi syndrome. Urinary 
      immunoelectrophoresis indicated the presence of K Bence Jones' protein (BJP). A 
      bone marrow biopsy examina- tion showed that the plasma-to-cell ratio was less 
      than 10%. However, the patient had over lg/day of proteinuria and suppression of 
      serum IgM (18mg/dL) and was, therefore, diagnosed with multiple myeloma based on 
      SWOG criteria. Light microscopic examination showed evidence of 
      glomerulosclerosis, intimal thickness of interlobular arteries and acidophilic 
      granular deposits in the cytoplasm of the proximal epithelial tubular cells. 
      Immunofluores- cence indicated positive anti-K staining in these regions. 
      Electron microscopic examination of the proximal tubular epithelial cells 
      revealed the presence of numerous diamond-shaped and oval crystals, thought to be 
      the K light chain of BJP. In general, cast nephropathy, light chain deposition 
      disease (LCDD) and AL amyloidosis are recog- nized renal injuries caused by 
      myeloma. However, there have been few clinical reports of Fanconi syndrome with 
      multiple myeloma, such as the case study we have described here. In addition, 
      histological examination of a biopsy sample provided further evidence of K BJP in 
      the proximal epithelial tubular cells.
FAU - Inomata, Miho
AU  - Inomata M
FAU - Tokunaga, Koki
AU  - Tokunaga K
FAU - Nakahara, Mai
AU  - Nakahara M
FAU - Deguchi, Hiroyuki
AU  - Deguchi H
FAU - Kojyo, Takuma
AU  - Kojyo T
FAU - Abe, Masaharu
AU  - Abe M
FAU - Oyamada, Miki
AU  - Oyamada M
FAU - Oku, Man-Ei
AU  - Oku ME
FAU - Ido, Akio
AU  - Ido A
LA  - jpn
PT  - Case Reports
PT  - Journal Article
PL  - Japan
TA  - Nihon Jinzo Gakkai Shi
JT  - Nihon Jinzo Gakkai shi
JID - 7505731
RN  - 9006-99-9 (Bence Jones Protein)
SB  - IM
MH  - Bence Jones Protein/*analysis
MH  - Biopsy
MH  - Fanconi Syndrome/*etiology/pathology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Myeloma/chemistry/*complications
EDAT- 2016/01/01 00:00
MHDA- 2016/01/01 00:01
CRDT- 2019/01/09 06:00
PHST- 2019/01/09 06:00 [entrez]
PHST- 2016/01/01 00:00 [pubmed]
PHST- 2016/01/01 00:01 [medline]
PST - ppublish
SO  - Nihon Jinzo Gakkai Shi. 2016;58(7):1088-1094.