Kidney Podocyte Zebra Bodies after Lung Transplantation for Lymphangioleiomyomatosis.

A 55-year-old woman showed progressive renal dysfunction after unilateral deceased-donor lung transplantation for lymphangioleiomyomatosis. A kidney biopsy showed a striped pattern of interstitial fibrosis, suggesting calcineurin inhibitor toxicity, and zebra body accumulation predominantly in the podocytes, characteristics of Fabry disease. Nevertheless, she had no extra-renal symptoms of the disease, and gene testing identified no known pathogenic variant or exon deletion. Our case report and literature review suggest that this atypical lysosomal inclusion may be phospholipidosis induced by sertraline. Potential underlying etiologies linking zebra body deposits may be not only hereditary but also drug-induced phospholipidosis.

Comparison of fracture risk between proton pump inhibitors and histamine-2 receptor antagonists in ANCA-associated vasculitis patients: a nested case-control study.

OBJECTIVE: Whether acid suppressants [proton pump inhibitors (PPIs) and histamine-2 receptor antagonists (H2RAs)] are associated with bone fractures in patients with ANCA-associated vasculitis (AAV) treated with glucocorticoids remains unclear. This study compared PPIs with H2RAs in terms of the risk of bone fractures in patients with AAV who received in-hospital induction therapy with glucocorticoids. METHODS: We retrospectively identified 149 patients with fractures among 22 821 patients newly diagnosed with AAV in 1730 hospitals using a nationwide inpatient database from July 2010 to March 2018. We conducted 1:4 case-control matching. Age, sex, duration of AAV treatment and fiscal year were matched between the cases and controls. A conditional logistic regression analysis was conducted to assess the association between acid suppressants and fractures. RESULTS: Of all enrolled patients with fractures, the median age was 77 years, and 99 (66%) were female. The median duration from AAV treatment to fracture was 52 days. The proportion of patients using PPIs was 91.3% (136 of 149) and 80.2% (478 of 596) in the case and control groups, respectively. Compared with H2RA use, PPI use was significantly associated with fractures after adjustment for age, sex, BMI, smoking habit, Charlson comorbidity index, renal failure, bisphosphonate and same fiscal year according to a multivariate analysis (adjusted odds ratio, 3.76; 95% CI: 1.37, 10.3). CONCLUSION: PPI users had a higher risk of fractures than H2RA users among mostly advanced-age patients with AAV with remission induction therapy.

Discontinuation of Hemodialysis in a Patient with Anti-GBM Disease by the Treatment with Corticosteroids and Plasmapheresis despite Several Predictors for Dialysis-Dependence.

A 26-year-old man highly suspected of having antiglomerular basement membrane (GBM) disease was treated with corticosteroid pulse therapy 9 days after initial infection-like symptoms with high procalcitonin value. The patient required hemodialysis the next day of the treatment due to oliguria. In addition to corticosteroid therapy, plasmapheresis was introduced and the patient could discontinue hemodialysis 43 days after the treatment. Kidney biopsy after initiation of hemodialysis confirmed anti-GBM disease with 86.3% crescent formation. Physician should keep in mind that active anti-GBM disease shows even high procalcitonin value in the absence of infection. To pursue recovery of renal function, the challenge of the immediate and persistent treatment with high-dose corticosteroids plus plasmapheresis for highly suspected anti-GBM disease is vitally important despite the presence of reported predictors for dialysis-dependence including oliguria and requiring hemodialysis at presentation.