Association between iron status markers and kidney outcome in patients with chronic kidney disease.

Several studies conducted in patients with various stages of chronic kidney disease (CKD) have investigated the association of iron status markers, such as transferrin saturation (TSAT) and serum ferritin, with kidney outcomes. However, the associations were inconsistent and remain strongly debated. Therefore, we aimed to investigate whether TSAT and serum ferritin levels were associated with kidney outcome in such a population. In this study, 890 patients who were admitted for the evaluation of and education for CKD were prospectively followed. Primary kidney outcome was a composite of doubling of serum creatinine, end-stage kidney disease, or death due to kidney failure. Participants were divided into quartiles (Q1-Q4) according to TSAT or serum ferritin levels. During a median follow-up period of 2.8 years, kidney events occurred in 358 patients. In the multivariable Cox analyses, compared with Q3 of TSAT, the hazard ratios (95% confidence intervals) for Q1, Q2, and Q4 were 1.20 (0.87, 1.66), 1.38 (1.01, 1.87), and 1.14 (0.82, 1.59), respectively. Compared with Q2 of serum ferritin, lower and higher quartiles had a significantly increased risk for kidney outcome; hazard ratios (95% confidence intervals) for Q1, Q3, and Q4 were 1.64 (1.18, 2.27), 1.71 (1.24, 2.37), and 1.52 (1.10, 2.10), respectively. A Fine-Gray model with death before kidney events as a competing risk showed results similar to the above. In CKD, lower and higher ferritin levels were independent risk factors for kidney disease progression.

Effectiveness of immunosuppressive therapy for nephrotic syndrome in a patient with late-onset Fabry disease: a case report and literature review.

BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations of the GLA gene, followed by deficiency in α-galactosidase A (α-gal) activity. Nephrotic syndrome, as the renal phenotype of FD, is unusual. Here, we report the rare case of a patient with FD with nephrotic syndrome whose proteinuria disappeared by immunotherapy. CASE PRESENTATION: A 67-year-old Japanese man was admitted to our hospital because of emesis, abdominal pain, and facial edema due to nephrotic syndrome. The patient was diagnosed with focal segmental glomerulosclerosis (FSGS) by renal biopsy before being diagnosed with FD, and immunotherapy was initiated. After treatment, the kidney biopsy results showed typical glycosphingolipid accumulation in the podocytes of this patient. The white blood cell α-gal activity was very low, and genetic analysis revealed a GLA gene variant (M296I), which is known as a late-onset genetic mutation of FD. Immunotherapy (steroids and cyclosporine A) dramatically improved the massive proteinuria. Currently, he has been undergoing enzyme replacement therapy, and his proteinuria has further decreased. There is the possibility that other nephrotic syndromes, such as minimal change nephrotic syndrome or FSGS, may co-exist in this patient. CONCLUSIONS: We experienced the rare case of a FD patient whose nephrotic syndrome disappeared by immunotherapy. These findings suggest that immunosuppressive treatment may be considered if nephrotic syndrome develops, even in patients with FD.

[Role of AGEs-RAGE system in CKD-MBD.]

Abnormalities, such as hyperparathyroidism, vascular calcification, and osteoporosis, are devastating complications in patients with end-stage renal disease(ESRD). These abnormalities significantly affect the quality of life and prognosis. Therefore, controlling the abnormalities of chronic kidney disease-mineral and bone metabolism play an important role in these patients. Conventionally, calcium and phosphorus metabolism abnormalities have been mainly attributed to the development of vascular calcification, but preventing vascular calcification is still difficult even if calcium and phosphorus levels are controlled. Additionally, the mechanisms of the development and progression of vascular calcification in patients with ESRD are still unknown. Recently, advanced glycation end products(AGEs)have been known to be involved in the development and promotion of bone abnormality, such as bone embrittlement and vascular calcification. Therefore, blockade of AGEs and the receptor for AGE(RAGE)system may be a novel therapeutic strategy to improve the prognosis of patients with ESRD by inhibiting bone embrittlement and vascular calcification.

[Examination of the shoulder joint using true anterior-posterior photography, employing a body surface index].

Radiographic examination of the anterior part of the shoulder includes routine anterior-posterior imaging that enables easy visualization of traumatic injuries and true anterior-posterior imaging that enables the visualization of intra-articular injuries. The X-ray incident angle of true anterior-posterior imaging is affected by physique and posture. However, in many reports, the angle is uniformly determined on the basis of the antero-posterior axis and the horizontal plane. We previously reported that the glenohumeral joint can be visualized with good reproducibility by establishing a reference line on the basis of three points on the body as indicators, namely the posterior view of the under-surface of the acromion, the coracoid process, and the inferior angle of the scapula. However, visualizing the undersurface of the acromion using physical indicators to set the angle for imaging remains problematic. In previous reports, the angle was consistently set at 20° to the horizontal plane, regardless of physique or posture, which resulted in poor reproducibility. After examining the imaging techniques described in previous reports, we describe here an imaging technique using a reference line based on indicators on the surface of the scapula that enables the glenohumeral joint and the undersurface of the acromion to be visualized with good reproducibility.

[Clinical study to obtain true view of the glenohumeral joint on scapula-45].

Scapula-45 is an original roentogenographical technique to clarify objectively the function of the rotator cuff and scapulothoracic joint. This examination enables assessment of the function of the rotator cuff and scapulothoracic joint utilizing the radiographs of the glenohumeral joint. However, in the former technique, it was difficult for the technician to properly align the patient for the view of the true glenohumeral joint because of the individual postural difference of the patients, such as kyphosis. In the present study, we reported a new technique with the use of the acromion, coracoid process, and inferior angle of the scapula as a landmark to obtain the consistent radiographs of the glenohumeral joint.