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.

Scleroderma renal crisis during intravenous cyclophosphamide pulse therapy for complicated interstitial lung disease was successfully treated with angiotensin converting enzyme inhibitor and plasma exchange.

Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC. We report an SSc patient with SRC and progressive ILD who underwent treatment with CYC and successful treatment with ACE-i and plasma exchange (PE). SRC and ILD are significant contributors to morbidity and mortality among SSc patients, and the therapy for these disorders is of great interest to rheumatologists. This study presents the possibility of favorable effects of PE for SSc-associated ILD and SRC.

Views of Japanese patients on the advantages and disadvantages of hemodialysis and peritoneal dialysis.

PURPOSE: The preference for dialysis modalities is not well understood in Japan. This study explored the subjective views of Japanese patients undergoing dialysis regarding their treatments. METHODS: The participants were receiving in-center hemodialysis (CHD) or continuous ambulatory peritoneal dialysis (CAPD). In Study 1, 34 participants (17 CHD and 17 CAPD) were interviewed about the advantages and disadvantages of dialysis modalities. In Study 2, 454 dialysis patients (437 CHD and 17 CAPD) rated the advantages and disadvantages of CHD and CAPD in a cross-sectional survey. RESULTS: Interviews showed that professional care and dialysis-free days were considered as advantages of CHD, while independence, less hospital visits, and flexibility were considered as advantages of CAPD. Disadvantages of CHD included restriction of food and fluids and unpleasant symptoms after each dialysis session. Catheter care was an additional disadvantage of CAPD. Survey showed that the highly ranked advantages were professional care in CHD and less frequent hospital visits in CAPD, while the highly ranked disadvantages were concerns about emergency and time restrictions in CHD, and catheter care and difficulty in soaking in a bath in CAPD. The total scores of advantages and disadvantages showed that CHD patients subjectively rated their own modality better CHD over CAPD, while CAPD patients had the opposite opinion. CONCLUSIONS: The results of this study indicate that the factors affecting the decision-making process of Japanese patients are unique to Japanese culture, namely considering the trouble caused to the people around patients (e.g., families, spouses, and/or caregivers).