Renovascular hypertension: a unique cause of unilateral focal segmental glomerulosclerosis.

A 48-year-old man presented with malignant hypertension and massive proteinuria. Renal angiography showed complete obstruction of the left renal artery and 99mTc-mercaptoacetylglycine (MAG3) renography showed a nonfunctioning left kidney. Percutaneous transluminal renal angioplasty of the left renal artery was unsuccessful; hence, the patient underwent left nephrectomy because of uncontrolled hypertension and proteinuria. Histological examination of a right kidney specimen revealed lesions of focal segmental glomerulosclerosis with benign nephrosclerosis. In contrast, histology of the left kidney showed typical ischemic kidney with hypertrophy of arteriolar smooth muscle cells. The patient responded favorably to the nephrectomy, as his blood pressure and urinary protein dramatically decreased with no antihypertensive medication. This case illustrates the heterogeneous effect of the renin-angiotensin system on either kidney in patients with renovascular hypertension due to unilateral renal artery stenosis.     

Myocardial perfusion and clearance of 99mTc teboroxime assessed by SPECT]

99mTc teboroxime is a new myocardial imaging agent that has characteristics of high accumulation in the heart and rapid clearance. We performed tomographic teboroxime study and compared the findings with that of 201Tl. Myocardial teboroxime clearance was calculated by dynamic single photon emission computed tomography (SPECT) using continuous repetitive rotation acquisition method. Teboroxime SPECT image was reconstructed by the three-minute data started from 4 minutes after injection. In 45 myocardial regions (15 patients), complete agreement between 201Tl and 99mTc teboroxime was obtained in 33 regions (73%), when the findings were classified as normal, ischemia and infarction. Significant delay in clearance was seen in the region of coronary stenosis (greater than or equal to 75%) compared with that in the control region (p = 0.0087 at rest, and p = 0.0385 at peak exercise by paired T test). Septum-to-lateral ratios of the clearance and myocardial initial count showed positive correlation (r = 0.743). Further clinical application of this radiopharmaceutical is expected as a new myocardial imaging agent.     

A digest of the Evidence-Based Clinical Practice Guideline for Nephrotic Syndrome 2020

Clinical and Experimental Nephrology -     

Evaluation of transcutaneous and end-tidal carbon dioxide levels during inhalation sedation in volunteers

Measurement of end-tidal carbon dioxide (P_ETCO₂) is useful because of its noninvasiveness, continuity, and response time when sudden changes in ventilation occur during inhalation sedation. We compared the accuracy of P_ETCO₂ using a nasal mask and nasal cannula with the accuracy of transcutaneous carbon dioxide (TC-CO₂) and determined which method is more useful during inhalation sedation in volunteers. We used a modified nasal mask (MNM) and modified nasal cannula (MNC) for measurement of P_ETCO₂. The capnometer measured P_ETCO₂ in the gas expired from the nasal cavity by means of two devices. The volunteers received supplemental O₂ by means of each device at a flow rate of 6 L/min. After the volunteers lay quietly for 5 min with a supply of 100 % O₂, they received supplemental N₂O by means of each device at concentrations of 10, 20, and 25 % for 5 min and 30 % for 25 min. The correlation coefficient was poorer in the MNM than in the MNC, and the mean difference between TC-CO₂ and P_ETCO₂ in the MNM was greater than that in the MNC. The difference between the TC-CO₂ and P_ETCO₂ ranged from 3 to 6 mmHg in the MNM and from 2 to 5 mmHg in the MNC. The difference between two variables against the TC-CO₂ and the CO₂ waveforms obtained by means of the two devices were within the clinically acceptable range. Our two devices can provide continuous monitoring of P_ETCO₂ with a supply of N₂O/O₂ in patients undergoing inhalation sedation.     

Interaction between ACE and ADD1 gene polymorphisms in the progression of IgA nephropathy in Japanese patients

An interaction effect between the angiotensin-converting enzyme insertion/deletion (ACE I/D) and alpha-adducin (ADD1) Gly460Trp polymorphisms (G460W) on blood pressure regulation has recently been suggested, although its significance in the prognosis of renal function in IgA nephropathy (IgAN) has not been fully investigated. Therefore, we evaluated the clinical manifestations and renal prognosis in 276 Japanese patients with histologically proven IgAN with respect to their ACE I/D and ADD1 G460W polymorphisms. The prognosis of renal function was analyzed by Kaplan-Meier survival curves and multivariate Cox proportional-hazards regression models. Baseline data, including blood pressures, proteinuria, renal function, and incidence of hypertension, were similar for the different genotypes of ACE and ADD1. The individual genotypes taken alone were not associated with the progression of renal dysfunction. However, renal survival of patients with the 460WW polymorphism of ADD1 was significantly worse within the group with the II genotype of ACE (Kaplan-Meier, log rank test; chi2=6.062, P=0.0138) but not for those with other ACE genotypes. In the Cox proportional-hazards regression model with adjustment for clinical risk factors, including hypertension, proteinuria, and no administration of an angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers, the 460WW variant of ADD1 was a highly significant and independent risk factor only for patients with the ACE II genotype, with a hazard ratio of 3.65 (P=0.0016), but not for those with other ACE genotypes (hazard ratio=0.65, P=0.2902). These findings suggest an interaction between ACE and ADD1 polymorphisms not only on blood pressure regulation but also on the progression of renal dysfunction in patients with IgAN.     

A case of endocapillary proliferative glomerulonephritis with macrophages phagocytosing monoclonal immunoglobulin lambda light chain

Multiple myeloma (MM) is a plasma‐cell neoplasm that can cause renal disorders. Renal lesions in MM can present with a very rare pathological manifestation involving a specific monoclonal immunoglobulin (Ig). We report the case of a 33‐year‐old woman who had edema, fatigue, elevated serum creatinine levels, hypoalbuminemia, and hypercholesterolemia. She had persistent hematuria and proteinuria lasting 3 years. Serum protein electrophoresis showed an M‐spike, and serum immunofixation demonstrated the presence of monoclonal IgG λ. She had proteinuria in the nephrotic range, and a monoclonal λ fragment was present on urine immunofixation. Renal biopsy showed proliferative glomerulonephritis with λ light chain and C3c deposition and inflammatory cell infiltration with CD68. Macrophage lysosomes contained λ light chains, suggesting their partial phagocytosis. She was diagnosed with symptomatic MM and was treated with bortezomib and dexamethasone and an autologous peripheral stem cell transplant conditioned with intravenous melphalan. She achieved a partial response with decreased serum monoclonal protein and improved renal function. This case may be categorized as a monoclonal gammopathy‐associated proliferative glomerulonephritis. The biopsy finding of partially phagocytosed Ig λ light chains by macrophages is very rare; this pathological condition is similar to crystal‐storing histiocytosis.     

Association between clinical parameters and amyloid-positive area in gastroduodenal biopsy in reactive amyloidosis associated with rheumatoid arthritis

Our study was aimed to clarify an association between gastrointestinal (GI) amyloid-positive area and various kinds of factors including renal function in reactive amyloidosis associated with rheumatoid arthritis (RA). Twenty-five patients with an established diagnosis of reactive AA amyloidosis participated in the study between January 1989 and December 2009. Each patient satisfied the 1987 American Rheumatism Association criteria for RA. All patients showed amyloid deposits in both of GI and renal tissues. The average amyloid-deposited area was 2.2% in renal tissues and 3.7% in GI tissues although the difference was not statistically significant. Twenty-two patients out of 25 patients showed less than 5% of amyloidosis in renal tissues and nineteen patients showed 5% of amyloidosis in GI tissues. In 5 out of a total of 25 cases, the amyloid-deposited area in GI tissues was lesser than that in renal tissues. Mesangial proliferative glomerulonephritis, thin basement membrane disease (TBMD) and membranous nephropathy were frequently combined with renal amyloidosis. For statistical analyses, renal and GI tissues of % amyloid-positive areas were transformed to common logarithmic values (Log₁₀%amyloid), since the histograms showed log-normal distribution. Clinical data were assessed by patient record at the time of GI biopsy. The correlation between Log₁₀%GI-amyloid and age, creatinine (Cr), creatinine clearance (Ccr), blood urea nitrogen (BUN), and estimated glomerular filtration rate (eGFR) were not significantly associated with Log₁₀%GI-amyloid in crude correlation analyses and also in sex- and age-adjusted linear regression analyses. Although GI biopsy was not correlated with clinical factors, GI amyloid-positive areas were larger than renal amyloid-positive areas. Endoscopic screening of the upper GI tract is common in Japan, and amyloid-deposited area in GI tissues was sufficient to use for the diagnosis of amyloidosis compared with renal tissues in terms of convenience and sensitivity.