Tomoya Kishi

A Report of an Adult Case of Tubulointerstitial Nephritis and Uveitis (TINU) Syndrome, with a Review of 102 Japanese Cases.

Patient: Female, 44 Final Diagnosis: Tubulointerstitial nephritis • uveitis syndrome Symptoms: — Medication: Loxoprofen sodium hydrate Clinical Procedure: Renal biopsy Specialty: Nephrology Objective: Rare disease Background: Although TINU syndrome is characterized by idiopathic TIN with bilateral anterior uveitis, few reports have provided a comprehensive summary of the features of this disorder. Previous reports have suggested that many Japanese patients had HLA-A2 and -A24 (7), but there is no evidence. Case Report: A 44-year-old female was referred to our hospital due to renal dysfunction in March 2012. After admission, her symptoms improved spontaneously without medication within 2 weeks. In the outpatient clinic, she was diagnosed with idiopathic bilateral anterior uveitis in May, and her renal dysfunction relapsed in November. A renal biopsy showed diffuse TIN. We made a diagnosis of TINU syndrome because we could not explain the origin, and treated her with a systemic corticosteroid. Her renal function and ocular symptoms have been improving. The patient had HLA-A24, -B7, -DR1, -C*07: 02 and -DQB1*05: 01: 01. We collected 102 Japanese cases in PubMed, Ovid MEDLINE, and the Japanese Medical Abstracts Society and compared our case with the previous cases. Conclusions: This disorder affects primarily young females (median age, 14 years), and the most common symptom is fever (44/102 cases). We conducted a statistical analysis using contingency table and Pearson’s chi-square test, for HLA-A2 and A24, and calculated the odds ratio (OR). There are no significant differences (A2 was present in 7/22 cases and in 19/50 controls, p value (P) 0.61, OR 0.76 (95% confidence interval (CI)) 0.27–2.2; A24 was present in 10/22 cases and in 33/50 controls, P 0.10, OR 0.43, CI 0.16–1.2).

A Case Report of Nephrotic Syndrome Due to Collapsing Focal Segmental Glomerulosclerosis Treated With Low-density Lipoprotein Apheresis

Abstract:  We herein report the case of a 73‐year‐old woman with steroid and cyclosporine resistant collapsing focal segmental glomerulosclerosis (FSGS) whose refractory proteinuria and hypoproteinemia were controlled with low‐density lipoprotein apheresis (LDL‐A). She was initially treated with steroid therapy, including methylprednisolone pulse and cyclosporine therapy. However, her hypoproteinemia, accompanied with renal insufficiency, persisted despite these therapies. We treated her using LDL‐A and found improvement in her urine protein excretion, hyperlipidemia, hypoproteinemia, and renal function as a result of this treatment. This suggests that LDL‐A may therefore be an effective therapy for nephrotic syndrome due to collapsing FSGS.

Prolonged effect of fluid flow stress on the proliferative activity of mesothelial cells after abrupt discontinuation of fluid streaming.

Encapsulating peritoneal sclerosis (EPS) often develops after transfer to hemodialysis and transplantation. Both termination of peritoneal dialysis (PD) and transplantation-related factors are risks implicated in post-PD development of EPS, but the precise mechanism of this late-onset peritoneal fibrosis remains to be elucidated. We previously demonstrated that fluid flow stress induced mesothelial proliferation and epithelial-mesenchymal transition via mitogen-activated protein kinase (MAPK) signaling. Therefore, we speculated that the prolonged bioactive effect of fluid flow stress may affect mesothelial cell kinetics after cessation of fluid streaming. To investigate how long mesothelial cells stay under the bioactive effect brought on by fluid flow stress after removal of the stress, we initially cultured mesothelial cells under fluid flow stress and then cultured the cells under static conditions. Mesothelial cells exposed to fluid flow stress for a certain time showed significantly high proliferative activity compared with static conditions after stoppage of fluid streaming. The expression levels of protein phosphatase 2A, which dephosphorylates MAPK, in mesothelial cells changed with time and showed a biphasic pattern that was dependent on the duration of exposure to fluid flow stress. There were no differences in the fluid flow stress-related bioactive effects on mesothelial cells once a certain time had passed. The present findings show that fluid flow stress exerts a prolonged bioactive effect on mesothelial cells after termination of fluid streaming. These findings support the hypothesis that a history of PD for a certain period could serve as a trigger of EPS after stoppage of PD.

A comparison of the long-term effects of lanthanum carbonate and calcium carbonate on the course of chronic renal failure in rats with adriamycin-induced nephropathy.

Lanthanum carbonate (LA) is an effective phosphate binder. Previous study showed the phosphate-binding potency of LA was twice that of calcium carbonate (CA). No study in which LA and CA were given at an equivalent phosphate-binding potency to rats or humans with chronic renal failure for a long period has been reported to date. The objective of this study was to compare the phosphate level in serum and urine and suppression of renal deterioration during long-term LA and CA treatment when they were given at an equivalent phosphate-binding potency in rats with adriamycin (ADR)-induced nephropathy. Rats were divided into three groups: an untreated group (ADR group), a CA-treated (ADR-CA) group and a LA-treated (ADR-LA) group. The daily oral dose of LA was 1.0 g/kg/day and CA was 2.0 g/kg/day for 24 weeks. The serum phosphate was lower in the ADR-CA or ADR-LA group than in the ADR group and significantly lower in the ADR-CA group than in the ADR group at each point, but there were no significant differences between the ADR and ADR-LA groups. The serum phosphate was also lower in the ADR-CA group than in the ADR-LA group, and there was significant difference at week 8. The urinary phosphate was significantly lower in the ADR-CA group than in the ADR or ADR-LA group at each point. The urinary phosphate was also lower in the ADR-LA group than in the ADR group at each point, and significant difference at week 8. There were no significant differences in the serum creatinine or blood urea nitrogen among the three groups. In conclusion, this study indicated the phosphate-binding potency of LA isn’t twice as strong as CA, and neither LA nor CA suppressed the progression of chronic renal failure in the serum creatinine and blood urea nitrogen, compared to the untreated group.

Episodic confusion in a haemodilaysis patient due to haemodialysis-related portal-systemic encephalopathy and a shunt between the left portal vein and left external iliac vein: Correspondence

A77-year-oldwomanpresentedwith consciousness disturbance 4 years after the initiation of haemodialysis for the first time. Glasgow Coma Scale was 13, no flapping tremors. Laboratory examinations revealed hyperammonaemia (NH3 207μg/dL: normal 30–100), but neither liver damage nor hepatitis B&C although abdominal computed tomography (CT) revealed chronic liver disease pattern. Three-dimensional CT angiography demonstrated a long portal-systemic shunt (PSS) from the left portal vein to the left external iliac vein, with a diameter of 14.5mm (Fig. 1). Brain magnetic resonance imaging (MRI) did not show any abnormality. A diagnosis of haemodialysis-related portal-systemic encephalopathy (HRPSE) was made and therapy with branched-chain amino acids and lactulose ameliorated her symptom and hyperammonaemia (29μg/dL). Hyperammonaemia due to the flow of ammonia-rich portal vein blood through the PSS causes chronic portal-systemic shunt encephalopathy (CPSE), and it has been demonstrated clinical symptoms occur with shunt diameters larger than 10mm. Haemodialysis patients may develop CPSE through characteristic mechanisms of PSS growth due to fluid overload and an increase in back flow via the PSS to the systemic circulation as a consequence of hemodialysis-related fluid removal, known as HRPSE. Haemodialysis-related portal-systemic encephalopathy is a very rare complication and most shunts connect the left gastric vein or splenic veinwith the left renal vein or the inferior vena cava. To the best of our knowledge, this is the first case of HRPSE with a portal-iliac shunt. Management of this condition can be via conservative therapy as used in this case, or by closure of the shunt flow using either surgical ligation or embolization of the shunt (balloon-occluded retrograde transvenous obliteration: B-RTO). It is important to suspect HRPSE, even if liver function is normal.

Two cases of minor glomerular abnormalities with proteinuria disproportionate to the degree of hypoproteinemia

We experienced two female cases of minor glomerular abnormalities with proteinuria disproportionate to the degree of hypoproteinemia. They did not have adequately large amounts of urine protein so as to cause nephrotic syndrome; however, we were unable to determine any cause of hypoproteinemia other than proteinuria. The renal pathology revealed foot process effacement, and hyaline droplet degeneration, suggesting urine protein hyper-reabsorption in the proximal convoluted tubule. Therefore, we thought these cases involved pathophysiological conditions, such as minimal change nephrotic syndrome. In both cases, the hypoproteinemia improved following the administration of oral prednisolone. As in past reports, it is thought that the principal causative factor of hypoalbuminemia in patients with nephrotic syndrome is a catabolic reaction after the serum albumin filtered at the glomerulus is reabsorbed in the proximal tubule. In the present two cases, it is supposed that a large amount of urine protein was filtered in the primitive urine; however, the amount of final urinary protein did not reach the nephrotic range because most of it was reabsorbed in the proximal tubule and reabsorbed in the blood after being disintegrated into amino acids by a catabolic reaction. Or we might simply observe the process before the case 1 got nephrotic syndrome and the healing process of nephrotic syndrome in the case 2.

Acute renal failure associated with acute non-fulminant hepatitis B

A 38-year-old female presenting with a high fever of 39 °C developed severe liver dysfunction and acute renal failure (ARF). In tests for a hepatitis associated virus, an Immunoglobulin M-anti-hepatitis B virus core antibody was the only positive finding. Moreover, the progression of ARF coincided with the pole period of liver damage and all the other assumed causes for the ARF were unlikely. Therefore, this case was diagnosed as ARF caused by acute hepatitis B. ARF associated with non-fulminant hepatitis has been infrequently reported, usually in association with acute hepatitis A. This case is considered to be an extremely rare and interesting case.

Masked hyperthyroidism in a haemodialysis patient successfully treated by potassium iodide.

Hyperthyroidism should be suspected in end-stage renal disease (ESRD) patients who exhibit signs and symptoms including weight loss, tremor, palpitation or atrial fibrillation (Af) [1–5]. This report presents the case of an 82-year-old Japanese female on maintenance haemodialysis (HD) and paroxysmal Af (pAf). Although the serum thyroid hormone levels were within normal reference values for a young healthy control, she was found to have masked hyperthyroidism.

A case of endocapillary glomerulonephritis associated with peripheral blood natural killer cell proliferation

A 69-year-old male was admitted to our hospital due to rapidly progressive glomerulonephritis. A peripheral blood smear showed a marked increase in large granular lymphocytes. Flow cytometry analysis of the blood showed a marked increase in CD3-negative and CD56-positive natural killer (NK) cells. A renal biopsy showed a characteristic pathological pattern that involved endocapillary proliferation, a predominance of mononuclear cells and mesangiolysis. Prednisolone was administered, and the patient’s renal function subsequently improved concomitant with the amelioration of NK cell proliferation. In our case, there was evidence of a strong association between NK cell proliferation and glomerulonephritis.

Long-term Outcomes of Tonsillectomy for IgA Nephropathy Patients. : A Retrospective Cohort Study, Two-center Analysis with the Inverse Probability Therapy Weighting Method

The effect of tonsillectomy on IgA nephropathy remains controversial. The aim of this study was to compare the effect of tonsillectomy on the outcome, end stage kidney disease (ESKD) and all‐cause death in IgA nephropathy patients who did and did not undergo tonsillectomy.