Tsuyoshi Takashima

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.

Successful treatment of a patient with refractory nephrotic syndrome with PCSK9 inhibitors: a case report

BackgroundThe proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor evolocumab is a low-density lipoprotein (LDL)-lowering drug with a new mechanism, which is currently available in Japan. Here, for the first time, we report the successful use of the PCSK9 inhibitor in a patient with refractory nephrotic syndrome.Case presentationA 61-year-old woman was diagnosed with minimal change-type nephrotic syndrome in October 2012. She received prednisolone (PSL) and cyclosporin A (CyA), but she experienced several cycles of relapse and remission and was hospitalized in May 2016 due to relapse. However, in spite of steroid pulse therapy and adrenocorticotropic hormone (ACTH) administration, her urinary protein level did not improve. We started her on evolocumab with the expectation of equivalent LDL-lowering effects as seen with LDL apheresis. After that, the LDL cholesterol level and UP/UC were concomitantly decreased, and the serum albumin was increased. This was maintained even when we reduced the PSL dose. This suggests that evolocumab clinically improves the nephrotic condition.ConclusionNo other report has described the use of evolocumab for nephrotic syndrome (NS) or its effect on similar nephrotic conditions. We believe that the findings presented here are unique and may be beneficial when treating similar cases.

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.

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.

Is a liver biopsy necessary to diagnose hemodialysis-related portal-systemic encephalopathy (HRPSE)? A proposal of the concise diagnostic criteria for HRPSE

To the Editor Hepatic encephalopathy (HE) mostly occurs due to metabolic dysfunction of the liver, and the principal cause is hepatic cirrhosis. However, HE occasionally occurs in patients with a portal-systemic shunt (PSS) in the absence of severe liver dysfunction, called chronic portal-systemic shunt encephalopathy (CPSE) [1]. Then, it has been demonstrated that clinical symptoms occur with shunt diameters larger than 10 mm [2]. Hemodialysis patients may develop CPSE through characteristic mechanisms of the 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 hemodialysis-related portal-systemic encephalopathy (HRPSE) [3, 4]. In 2004, Ubara Y, et al. first named HRSPE [3]. A 68-year-old woman presented with consciousness disturbance after starting hemodialysis for the first time (Table 1, case 7). No liver dysfunction was found except for hyperammonemia, and abdominal imaging studies revealed a PSS. Ligation of the PSS was performed, and she recovered. Results of histologic examination of a liver biopsy specimen obtained intraoperatively were normal. However, the diagnostic criteria for HRPSE were not defined clearly in this report and have remained unclear until now. The diagnosis of CPSE does not depend on the presence of chronic liver disease [1]. Furthermore, liver biopsy is not necessarily possible in all cases suspected as HRPSE, due to various risks. We recently experienced a rare case of HRPSE but lacked the criteria for a definitive diagnosis [5]. Therefore, we would like to propose concise diagnostic criteria for HRPSE to help clinicians recognize HRPSE and diagnose it more easily. The patient should meet all the following diagnostic criteria.

Elevated serum thyroglobulin levels as a marker of reversible hypothyroidism in patients with end-stage renal disease due to chronic glomerulonephritis

Abstract Difference in thyroid function depending on the etiology of end-stage renal disease (ESRD) was evaluated in 124 Japanese patients on haemodialysis (HD) due to either chronic glomerulonephritis (CGN, n = 82) or lifestyle related systemic disease (non-CGN, n = 42), such as diabetes mellitus (n = 30) or hypertension (n = 12). There was no significant difference in serum free thyroxine, free triiodothyronine and thyroid-stimulating hormone (TSH) level, but serum thyroglobulin (Tg) level was significantly higher in CGN (p = 0.0151). Prevalence of the patients with hypothyroidism (TSH > 4.83 mU/l) was 11 or 13.4% in CGN and 4 or 9.5% in non-CGN (p = 0.017). The most striking finding was the elevated Tg in 38 or 46.3% in CGN and in 11 or 26.2% in non-CGN (p = 0.034). Logistic regression analysis revealed elevated serum TSH level and higher thyroid volume were the significant factors associated with elevated Tg level. Extreme Tg elevation over 100 ng/ml was found only in CGN (12 or 14.6%), and 2 of the patients were overtly hypothyroid but became euthyroid after iodide restriction. Elevated Tg responding to elevated TSH mainly found in CGN suggested the relatively preserved thyroid tissue and reversible recovery of the thyroid function.

A rare adult case of poststreptococcal acute glomerulonephritis with a retropharyngeal abscess

Retropharyngeal abscess is an infection involving the retropharyngeal space which is posterior to the pharynx and oesophagus, and it results as a complication of a primary infection elsewhere in the head and neck including the nasopharynx, paranasal sinuses, or middle ear, which drain lymph to the retropharyngeal lymph nodes. Their lymph nodes are prominent in children and atrophy with age. Therefore, retropharyngeal abscess is most frequently encountered in children, with 75% of cases occurring before the age of 5 years, and often in the first year of life. We experienced a rare adult case of poststreptococcal acute glomerulonephritis with a retropharyngeal abscess, and conservative therapy ameliorated them. According to past reports, only one child with a retropharyngeal abscess and poststreptococcal acute glomerulonephritis has been presented at a conference to date; this is the first adult case of poststreptococcal acute glomerulonephritis with a retropharyngeal abscess. Retropharyngeal abscess can be fatal including airway compression, so it is important to remember retropharyngeal abscess in a case of poststreptococcal acute glomerulonephritis with severe symptoms of neck.

Significance of Technetium-99m Human Serum Albumin Diethylenetriamine Pentaacetic Acid Scintigraphy in Patients with Nephrotic Syndrome

It is thought that a large amount of albumin leaking from the glomerulus in nephrotic syndrome (NS) is reabsorbed at the proximal tubule and catabolized. Therefore, it is possible the final quantity of urinary protein does not always reflect the amount of leakage of protein from the glomerulus. We experienced two cases without nephrotic range proteinuria thought to involve hypoproteinemia due to the same pathophysiology as NS. On these patients, we performed protein leakage scintigraphy with technetium-99m human serum albumin diethylenetriamine pentaacetic acid (99mTc-HSAD) to exclude a diagnosis of protein-losing gastroenteropathy and observed diffuse positive accumulation in the kidneys with more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration. In healthy adults intravenously given 99mTc-HSAD, the same dynamics are observed as in albumin metabolism, and the organ radioactivity of the liver and kidneys after 24 hours is equal. Therefore, we thought it was possible that the renal uptake 24 hours after 99mTc-HSAD administration was a characteristic finding of NS. In order to confirm it, the subjects were divided into two groups: the NS group (n = 10) and the non-NS group (n = 7). We defined more intense uptake in the kidney than the liver on the anterior view 24 hours after 99mTc-HSAD administration as Dense Kidney (+). Furthermore, we designed regions of interest in the right and left kidneys and liver on anterior and posterior images, then calculated the kidney-liver ratio. Nine of the ten patients had Dense Kidney (+) in the NS group, compared to none in the non-NS group. And the kidney-liver ratio was significantly higher in the NS group than in the non-NS group on each view in the bilateral kidneys. In conclusion, our results suggest that the renal uptake 24 hours after 99mTc-HSAD administration is a characteristic finding of NS.