Successful Treatment of Refractory Anemia in a Patient With Glycogen Storage Disease Type Ia Undergoing Hemodialysis.

Glycogen storage disease type Ⅰa (GSDIa), also known as von Gierke disease, is a rare inherited metabolic disorder caused by defective glucose 6-phosphatase (G6Pase) activity. Although anemia, renal failure, and hepatic adenoma are the major clinical manifestations of GSDIa, there has been no report of refractory anemia in GSDIa patients on maintenance hemodialysis (HD) concomitant with multiple liver adenomas. Herein, we present a case of refractory anemia in a patient with GSDIa undergoing HD with multiple hepatic adenomas, successfully managed through aggressive treatment for renal anemia and intravenous iron therapy (IIT). A 26-year-old man with GSDIa who had been on HD for a year suffered from refractory anemia. He had experienced hypoglycemia and hyperlactic acidemia repeatedly and unusual hypertriglyceridemia had been observed for a long time. In addition, multiple hepatic adenomas developed and his renal function gradually declined, eventually progressing to end-stage kidney disease, and HD was started. Despite 120 µg/week of darbepoetin alfa (DA), 200 mg/day of oral sodium ferrous citrate, and 600 mg/week of roxadustat, the anemia persisted and iron deficiency gradually progressed. We considered that renal anemia, blood loss by each HD session, and decreased intestinal iron absorption due to inappropriately increased hepcidin from hepatic adenomas were the main etiology of the anemia; hence, we changed oral sodium ferrous citrate to intravenous saccharated ferric oxide along with continuous aggressive treatment of renal anemia, and the anemia resolved quickly within three months. We believe that refractory anemia was mainly induced by renal anemia and chronic iron deficiency due to blood loss during HD and inappropriately elevated hepcidin levels in hepatic adenomas. Aggressive treatment of renal anemia, along with IIT, may be a promising treatment option. Strict monitoring of iron overload is essential for safe treatment.

Atypical, Levetiracetam-induced Hypersensitivity Syndrome Complicated by Fulminant Liver Failure in a Patient Undergoing Hemodialysis.

A 59-year-old man undergoing hemodialysis was administered levetiracetam, after which he developed a systemic rash, high fever, severe liver dysfunction, and leukocytopenia with reactivation of human herpes virus 6. Atypical drug-induced hypersensitivity (DIHS) was diagnosed, and prednisolone was administered at 60 mg/day. However, liver failure rapidly progressed, and the patient died 12 days following treatment. Despite the rarity of DIHS with concomitant fulminant liver failure from levetiracetam and sufficient clearance thereof by hemodialysis, our case suggests that this syndrome may still ensue, resulting in mortality, even in hemodialysis patients. Although no treatment has yet been established, strict monitoring and aggressive treatment may be required.

IgA Vasculitis in an Oldest-old Patient Successfully Treated with Glucocorticoid.

A 94-year-old man was diagnosed with immunoglobulin A vasculitis (IgAV), and losartan was initiated. His renal function rapidly deteriorated over a month; therefore, methylprednisolone was administered intravenously for three days followed by oral prednisolone. Renal function improvement and both proteinuria and hematuria remission were observed within six months. Prednisolone tapering was completed at eight months. In this case, we monitored the patient carefully and started glucocorticoids as soon as the patient's renal function deteriorated. We were thus able to treat the patient with a relatively small dose of glucocorticoids in a short treatment period without any adverse events due to glucocorticoids.

In Situ Activation of a Manganese Perovskite Oxygen Reduction Catalyst in Concentrated Alkaline Media.

The reaction pathway of the oxygen reduction reaction (ORR) is strongly affected by the electrolytic environment. Meanwhile, the ORR mechanism on transition-metal oxide catalysts has not been studied intensely in very concentrated alkaline solutions that are used in practical metal-air batteries. Herein, we report the in situ activation of ORR catalysis on manganese perovskite in a concentrated alkaline solution, mediated by the spontaneous formation of oxygen vacancy sites. Electrochemical analyses of the (100) epitaxial film electrodes reveal that the exchange current and electron number of the ORR on La0.7Sr0.3Mn0.9Ni0.1O3 significantly increase with the duration of the ORR when the KOH concentration is greater than 4 M. However, these values remain unchanged with time at less than 1 M KOH concentration. Operando synchrotron X-ray spectroscopy of the (100) epitaxial film confirmed that La0.7Sr0.3Mn0.9Ni0.1O3 involves the oxygen vacancy sites with the reduction of Mn atoms in concentrated KOH solution via the hydroxylation decomposition of perhydroxyl intermediates. Hence, the O2 adsorption switched from an end-on to a bidentate mode because the cooperative active sites of the oxygen vacancy and neighboring Mn allow bidentate adsorption of the dissolved O2. Due to the simultaneous interaction with the oxygen vacancy and Mn sites, the O-O bonds are activated and the potential barrier for the electron transfer to adsorbed O2 is lowered, resulting in a shift in the reaction mechanism from that involving an indirect "2 + 2" transfer pathway to a direct 4-electron pathway.

In vivo regeneration of interspecies chimeric kidneys using a nephron progenitor cell replacement system.

Kidney regeneration is expected to be a new alternative treatment to the currently limited treatments for chronic kidney disease. By transplanting exogeneous nephron progenitor cells (NPCs) into the metanephric mesenchyme of a xenogeneic foetus, we aimed to regenerate neo-kidneys that originate from transplanted NPCs. Previously, we generated a transgenic mouse model enabling drug-induced ablation of NPCs (the Six2-iDTR mouse). We demonstrated that eliminating existing native host NPCs allowed their 100% replacement with donor mouse or rat NPCs, which could generate neo-nephrons on a culture dish. To apply this method to humans in the future, we examined the possibility of the in vivo regeneration of nephrons between different species via NPC replacement. We injected NPCs-containing rat renal progenitor cells and diphtheria toxin below the renal capsule of E13.5 metanephroi (MNs) of Six2-iDTR mice; the injected MNs were then transplanted into recipient rats treated with immunosuppressants. Consequently, we successfully regenerated rat/mouse chimeric kidneys in recipient rats receiving the optimal immunosuppressive therapy. We revealed a functional connection between the neo-glomeruli and host vessels and proper neo-glomeruli filtration. In conclusion, we successfully regenerated interspecies kidneys in vivo that acquired a vascular system. This novel strategy may represent an effective method for human kidney regeneration.

Peritoneal dialysis-associated infection caused by Mycobacterium abscessus: a case report.

BACKGROUND: Peritoneal dialysis (PD)-associated infection caused by Mycobacterium spp. is rare. Mycobacterium abscessus is one of the most resistant acid-fast bacteria, and treatment is also the most difficult and refractory. Thus, we report a case of PD-associated peritonitis caused by Mycobacterium abscessus that was difficult to treat and led to PD failure. CASE PRESENTATION: We recently encountered a 56-year-old man who developed PD-associated infection. We initially suspected exit-site infection (ESI) and tunnel infection (TI) caused by methicillin-resistant coagulase-negative Staphylococcus. However, antibiotic therapy did not provide any significant improvement. Thus, we performed simultaneous removal and reinsertion of a PD catheter at a new exit site. The patient subsequently developed peritonitis and Mycobacterium abscessus was detected in the peritoneal effluent. Thus, the reinserted catheter was removed, hemodialysis was started, and the patient was eventually discharged. CONCLUSIONS: In cases of refractory ESI or TI, it is important to consider non-tuberculous mycobacteria as the potentially causative organism. Even if acid-fast bacterial staining is negative or not performed, detection of Gram-negative bacillus may lead to suspicion and early identification of Mycobacterium spp. In PD-associated infection by Mycobacterium abscessus, catheter removal is necessary in many cases. Simultaneous removal and reinsertion of the catheter is not recommended, even in cases of ESI or TI. Reinsertion should only be attempted after complete resolution of peritoneal symptoms. After removal of the catheter, careful follow-up is necessary, paying attention to complications such as wound infection, peritonitis, and ileus. In addition, the selection and treatment period of antibiotics in PD-associated infection by Mycobacterium abscessus remains unclear, and it is an important topic for future discussion.