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A 41-year-old woman diagnosed with seronegative myasthenia gravis struggled to maintain remission for a decade, facing crises every 3 months for several years. After repeated apheresis using a non-tunneled non-cuffed central venous dialysis catheter (NTNCC), complications such as catheter-related thrombus in the internal jugular veins and morbid obesity from steroids made the insertion of NTNCC increasingly difficult, leading to consideration of an alternative permanent vascular access (VA) approach. Thus, we created a subcutaneously superficialized brachial artery as the VA, which allowed the patient to undergo safe and uninterrupted apheresis therapy.

Background: Dialysis central venous catheters (DCs) are used for various blood purification therapies. Accidental removal of a DC is a potentially life-threatening event. However, the risk factors associated with accidental removal have rarely been discussed in the literature., Methods: We performed a 5 year retrospective, single-center, cohort study of patients who were admitted to the ward other than intensive care units and were inserted DCs. We compared patient characteristics between patients who used DCs with and without a history of accidental removal., Results: There were 17 cases of accidental removal (3.5%) out of 489 DC insertions during the observation period. There was no significant difference between patients with and without accidental removal in terms of age, sex, reason for DC insertion, catheter type, or insertion site. The presence of dementia was 35 and 11% (p = 0.006), and that of delirium was 88 and 13% (p < 0.0001) in those with and without accidental removal, respectively. The median days from insertion to accidental removal were 5 (range 0-21) days, and removal occurred mainly at night (71%). One patient with accidental removal developed hemorrhagic shock, but there no deaths occurred due to accidental removal., Conclusions: The present findings suggest that to prevent accidental removal, sufficient attention should be paid to delirium that occurs after admission as well to pre-existing dementia at the time of admission. As accidental removal occurs most commonly early after insertion and at night, special attention should be paid during these periods., (© 2022. The Author(s), under exclusive licence to The Japanese Society of Nephrology.)

We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. A muscle biopsy of the right bicep showed polymyositis. Computed tomography showed a right renal mass, and an analysis after right nephrectomy identified clear cell carcinoma. The muscle weakness subsided one month after nephrectomy and intravenous immunoglobulin therapy. Therefore, we suspect that the development of polymyositis in this patient was closely related to renal cell carcinoma.

In the original publication, some errors have been found in the alignment of Table 9. The corrected table is given below.

Background: Practice patterns and bleeding complications of percutaneous native kidney biopsy (PNKB) have not recently been investigated and the Japanese Society of Nephrology performed a nationwide questionnaire survey in 2018., Methods: The survey consisted of nine sections about PNKB: (1) general indications; (2) indications for high-risk patients; (3) informed consent; (4) pre-biopsy evaluation; (5) procedures; (6) sedation; (7) post-biopsy hemostasis, bed rest, and examinations; (8) bleeding complications; and (9) specimen processing. A supplementary survey examined bleeding requiring transcatheter arterial embolization (TAE)., Results: Overall, 220 directors of facilities (nephrology facility [NF], 168; pediatric nephrology facility [PF], 52) completed the survey. Indications, procedures, and monitoring protocols varied across facilities. Median lengths of hospital stay were 5 days in NFs and 6 days in PFs. Gauge 14, 16, 18 needles were used in 5%, 56%, 33% in NFs and 0%, 63%, 64% in PFs. Mean limits of needle passes were 5 in NFs and 4 in PFs. The bed rest period was 16-24 h in 60% of NFs and 65% of PFs. Based on 17,342 PNKBs, incidence rates of macroscopic hematuria, erythrocyte transfusion, and TAE were 3.1% (NF, 2.8%; PF, 6.2%), 0.7% (NF, 0.8%; PF, 0%), and 0.2% (NF, 0.2%; PF, 0.06%), respectively. Forty-six percent of facilities processed specimens all for light microscopy, immunofluorescence, and electron microscopy, and 21% processed for light microscopy only. Timing of bleeding requiring TAE varied among PNKB cases., Conclusion: Wide variations in practice patterns of PNKB existed among facilities, while PNKBs were performed as safely as previously reported.

Background: Unintentional renal artery occlusion after endovascular aneurysm repair (EVAR) for abdominal aortic aneurysm remains one of the most unfavorable complications. Renal salvage options include percutaneous transluminal renal artery angioplasty (PTRA) and open hepatosplenorenal bypass. However, the usefulness of kidney autotransplantation (AutoTx) remains unclear., Case Presentation: A 76-year-old woman with a right solitary kidney attributable to a left renal thromboembolism had previously undergone EVAR with a stent graft for an infrarenal aortic aneurysm, which led to ostial occlusion of the right renal artery. In addition, she had undergone PTRA and stenting. Two days before admission, she developed leg edema and hypertension, leading her to visit the hospital. Her serum creatinine level was 2.4 (baseline, 1.0) mg/dL. Acute kidney injury due to renal artery in-stent restenosis was suspected; re-angioplasty was attempted on day 2 of hospitalization, but was unsuccessful. Her renal function did not improve and anuria persisted; thus, hemodialysis was initiated on the same day. The right kidney size (8.6 cm) was preserved relative to her body size, with only mild cortical atrophy. Doppler ultrasonography and mercaptoacetyltriglycine scintigraphy revealed minimal but significant perfusion of the right kidney. Therefore, we considered that kidney perfusion was sustained and renal function could be reversed. On day 25 of hospitalization, right kidney AutoTx to the right iliac fossa was performed to reestablish adequate renal perfusion and reverse the need for dialysis. Soon after the procedure, the patient started passing urine. Her renal function improved; her serum creatinine level decreased to 1.0 mg/dL on day 33 of hospitalization. Hemodialysis was discontinued after the surgery. Zero-hour kidney biopsy showed only mild tubular injury, with neither tubular necrosis nor glomerular abnormalities., Conclusions: Kidney AutoTx can be performed for patients with renal artery in-stent occlusion after unsuccessful PTRA who previously underwent EVAR. Our case showed successful recovery of renal function nearly 1 month after renal artery occlusion, indicating that revascularization should be considered even if it is delayed, as the kidney might be perfused through collateral circulation.

A 29-year-old woman with past medical history of hypertension was referred to our hospital for the evaluation of kidney dysfunction (serum creatinine 1.0 mg/dL), proteinuria (0.54 g/gCre), and microscopic hematuria. Renal biopsy before the first pregnancy was supportive for benign nephrosclerosis with no evidence of vasculitis. After her second pregnancy and delivery when she was 32 years old, she developed proteinuria of 3.2 g/gCre, hematuria, and elevated serum creatinine level of 2.6 mg/dL. Second renal biopsy revealed necrotizing glomerulonephritis and her serum MPO-ANCA was positive, leading to the diagnosis of MPA/renal-limited vasculitis (RLV). Interestingly, frozen preserved serum from 4 years earlier also tested positive for MPO-ANCA. Despite intensive treatment, hemodialysis was required 10 years later due to progressive deterioration of renal function. At that time, she developed pericarditis, bloody cardiac tamponade, and pulmonary alveolar hemorrhage, resulting in a diagnosis of systemic vasculitis MPA. She received living donor kidney transplantation at the age of 44 years, after which serum creatinine has been stable around 1.1 mg/dL without proteinuria or hematuria and MPO-ANCA has remained negative. The association of vasculitis with pregnancy and delivery is not well documented, especially in patients with MPA. Here, we report this MPO-ANCA positive woman developing MPA necrotizing glomerulonephritis after her second pregnancy and a 20-year clinical course.

Although primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) causes various symptoms depending on the tumor region, there has been no previous report of PCNS-PTLD in the cerebellopontine angle that was diagnosed due to peripheral facial nerve palsy. We herein report a case involving a 62-year-old man with PCNS-PTLD in the cerebellopontine angle who was diagnosed due to peripheral facial nerve palsy. The reduction of immunosuppressive therapy, whole-brain radiotherapy, intrathecal chemotherapy, and rituximab were effective in treating this patient. Physicians should therefore be mindful that PCNS-PTLD can cause peripheral facial nerve palsy in renal transplant recipients.

A 14-year-old Japanese girl was admitted to our institution for the evaluation of renal dysfunction. Her serum creatinine was 1.1 mg/dL, proteinuria was 1.5 g/day, the urine sediment contained numerous erythrocytes per high-power field, and she was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Proteinuria was first noted at the age of 12 years. Renal biopsy showed crescentic glomerulonephritis with slight immunoglobulin A (IgA) deposition. A diagnosis of ANCA-associated vasculitis was made. Immunosuppressive therapy was initiated, including steroid pulse therapy and intravenous cyclophosphamide pulse therapy, but hemodialysis was required after 6 years. Eight months after the patient became anuric and her MPO-ANCA titer became negative, living-related donor kidney transplantation was done from her mother. ANCA became slightly positive 2 years later, but the patient remains stable without proteinuria or hematuria at 4 years after surgery. This case suggests that kidney transplantation can be performed successfully for a patient with refractory childhood-onset ANCA-associated vasculitis, and that remission of vasculitis associated with ANCA negativity at transplantation may contribute to a better renal prognosis in this patient.

We evaluated the influence of kidney volume (KV) and liver volume (LV) on continuation of peritoneal dialysis (PD) in patients with autosomal dominant polycystic kidney disease (PKD). Twenty-two PKD patients on PD were retrospectively investigated after being divided into two groups. Group 1 comprised 15 patients who started PD at our hospital and group 2 was composed of seven patients referred from other hospitals for treatment of renomegaly by transcatheter arterial embolization (TAE) at 47.1 ± 21.8 months after commencing PD. In group 1, KV for both kidneys (mean ± SD) was 2787 ± 1945 mL (range: 1043 to 6816 mL), LV was 2198 ± 1139 mL (1005 to 4116 mL), and the total organ volume (TV=KV+LV) was 4985 ± 1815 mL (2320 to 8912 mL). In the patient with the largest TV from group 1 (KV of 6816 mL, TV of 8912 mL, and TV/BMI ratio of 426, PD was stopped due to dialysate leakage. However, dialysate leakage did not occur in the other 14 patients (TV ≦ 7963 mL and TV/BMI ratio of 353 at the start of PD). In group 2, KV was 5822 ± 1597 mL (3832 to 8862 mL), LV was 1776 ± 519 mL (1271 to 2671 mL), and TV was 7597 ± 1431 mL (5505 to 10358) before TAE. Leakage of dialysate did not occur with a mean infusion volume of 1530 ± 370 mL (1000 mL to 2000 mL), even after renomegaly and hepatomegaly progressed to the maximum TV/BMI ratio of 359. Six patients from the two groups developed new abdominal hernias at 36 ± 5 months (6-55 months) after starting PD. These findings suggest that performance of PD may be limited by renomegaly and hepatomegaly in patients with PKD., (© 2015 The Authors. Therapeutic Apheresis and Dialysis © 2015 International Society for Apheresis.)

We designed a new method of measuring the length of the ulnar nerve and determining standard values for F-wave parameters of the ulnar nerve in clinically normal beagles. Nerve length must be precisely measured to determine F-wave latency and conduction velocity. The length of the forelimb has served as the length of the ulnar nerve for F-wave assessments, but report indicates that F-wave latency is proportional to the length of the pathway traveled by nerve impulses. Therefore, we measured the surface distance from a stimulus point to the spinous process of the first thoracic vertebra (nerve length 1) and the anterior horn of the scapula (nerve length 2) as landmarks through the olecranon and the shoulder blade acromion. The correlation coefficients between the shortest F-wave latency and the length of nerves 1, 2 or the forelimb were 0.61, 0.7 and 0.58. Nerve length 2 generated the highest value. Furthermore, the anterior horn of the scapula was easily palpated in any dog regardless of well-fed body. We concluded that nerve length 2 was optimal for measuring the length of the ulnar nerve.