Inomata M, Tokunaga K, Nakahara M
… +6 more, Deguchi H, Kojyo T, Abe M, Oyamada M, Oku ME, Ido A
Nihon Jinzo Gakkai Shi
· 2016 · PMID 30620818
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...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.
Mizukaki Y, Maruyama T, Hamada H
… +4 more, Maruyama T, Satomura A, Kawamoto S, Sudo Y
Nihon Jinzo Gakkai Shi
· 2016 · PMID 30620817
BACKGROUND AND OBJECTIVE: Progression of chronic kidney disease (CKD) leads to the onset of cardiovascular dis- eases and an increase in the number of patients requiring dialysis initiation. In prder to promote the early...BACKGROUND AND OBJECTIVE: Progression of chronic kidney disease (CKD) leads to the onset of cardiovascular dis- eases and an increase in the number of patients requiring dialysis initiation. In prder to promote the early detection and treatment of CKD, we assessed the effects of blood pressure control on renal impairment based on common test items and verified the usefulness of the target blood pressure for patients with CKD described in the Japanese Society of Hypertension Guidelines for the Management of Hypertension in 2014 (JSH 2014). SUBJECTS AND METHODS: Among patients who had been regularly visiting the outpatient clinic of Kasukabe Kisen Hospital for 24 months or more, 67 with a serum creatinine level of 1.2 mg/dL or higher were included in this study. Clinical blood pressure measurements obtained at the start of follow-up and at 6, 12, 18, and 24 months were averaged to serve as the 2-year mean blood pressure, and the progression rates of renal impairment were com- pared between patients achieving and those not achieving the target blood pressure of the JSH 2014. RESULTS: Among the diabetic patients with CKD, significant differences in renal impairment progression rates were observed between those achieving and those not achieving the target blood pressure. Among the non-diabetic patients with CKD, those achieving the target blood pressure tended to show slower progression of renal impair- ment, but their progression rates were not significantly different from those of the patients not achieving the target blood pressure. CONCLUSION: Blood pressure control is essential for patients with CKD. In patients with diabetes mellitus, the pro- gression rates of renal impairment can be substantially reduced by maintaining blood pressure below the target blood pressure described in the JSH 2014.
Watanabe R, Ito K, Yasuno T
… +7 more, Abe Y, Hamauchi A, Yasunaga T, Sasatomi Y, Hisano S, Saito T, Nakashima H
Nihon Jinzo Gakkai Shi
· 2016 · PMID 30375826
This case describes a 68-year-old woman exhibiting initial proteinuria at age 55. Subsequently, at age 57, a mixed-type of amyloidosis consisting of amyloid amyloidosis (A A) and immunoglobulin (Ig) light chain amyloidos...This case describes a 68-year-old woman exhibiting initial proteinuria at age 55. Subsequently, at age 57, a mixed-type of amyloidosis consisting of amyloid amyloidosis (A A) and immunoglobulin (Ig) light chain amyloidosis (AL) was diagnosed by a renal biopsy examination. Monoclonal paraproteinemia was concurrently identified and diagnosed as monoclonal gammopathy of undeterminate significance (MGUS). Combined melphalan and prednisolone (MP) therapy was initiated. At age 65, anti-hypertensive drugs were administered upon finding an increased urine protein concentration and elevated blood pressure. Because there was no change in the state of MGUS detected by a bone marrow biopsy examination, MP therapy was discontinued. However, the urinary protein concentration increased, and a renal biopsy was performed again at age 66. This revealed a mixed-type amyloidosis of AA and AL, as diagnosed earlier, but AL amyloid deposition in the glomeruli had increased during the intervening period. Life-preserving treatment was continued thereafter, but nephrotic syndrome and renal dysfunction progressed rapidly. End-stage renal failure deposition is rarely seen in the same individual. Although amyloidosis is generally thought to cause a rapid decline in renal function, the patient's renal function was maintained for 13 years. This could be attributed to the following factors : l)the underlying etiology of the AA amyloidosis, which was not clear, 2)a lack of any current evidence of chronic inflammation, and 3) MGUS as the cause of AL amyloidosis. This, together with MP therapy, may have slowed down the pathological decline normally associated with AL amyloidosis.
Amemiya N, Sugiura H, Kamiyama T
… +6 more, Ubukata M, Nokiba H, Yamazaki M, Takei T, Honda K, Nitta K
Nihon Jinzo Gakkai Shi
· 2016 · PMID 30375822
Medullary cystic kidney disease (MCKD) is usually associated with slowly progressive kidney injury. However, we encountered a case of MCKD with rapidly progressive kidney injury and irreversible renal dysfunction. A 63-y...Medullary cystic kidney disease (MCKD) is usually associated with slowly progressive kidney injury. However, we encountered a case of MCKD with rapidly progressive kidney injury and irreversible renal dysfunction. A 63-year-old woman presented with a 4-month history of hypertension and rapidly progressive renal dysfunction. On admission, her blood pressure was slightly elevated (158/85 mmHg). The scrum creatinine (11.57 mg/dL) was markedly elevated. Urinalysis showed occult hematuria and proteinuria(1.06 g/gCr). /β2- microglobulin 45,000 μg/ L, N-acetyl-/β-D-glucosaminidase 5.6 U/L. Neither ultrasonography nor computed tomography revealed any evidence of renal medullary cysts. Both kidneys showed an irregular surface and enlargement. Microscopic evaluation of the renal biopsy revealed extensive tubular dilatation and atrophy with interstitial fibrosis. Often glomeruli, one had global sclerosis and the others were normal. The tubular dilatation was more marked in the distal than in the proximal tubules, according to the immunohistochemical findings of positivity for epithelial membrane antigen (EMA), a marker of distal tubules, and negativity for CD 10, a marker of proximal tubules. No immunoglobulin or complement deposition was detected in either the glomeruli or the tubules. Electron microscopy revealed disintegration of the tubular basement membrane with fragile thinning and lamination of the membrane. These pathological findings were compatible with MCKD. This was a case of MCKD diagnosed incidentally in an elderly patient who presented with rapidly progressive kidney injury accompanied by hypertension. Renal biopsy was necessary for the diagnosis.