Internal medicine
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Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a lack of alpha-galactosidase A (AGALA) activity in lysosomes. We herein report a patient with FD revealed by a renal biopsy who survived seven years after the introduction of peritoneal dialysis despite having severe heart failure due to left ventricular hypertrophy (LVH). ⋯ In our case, cardiac involvement determined the prognosis. Peritoneal dialysis is the modality of choice in the long-term management of dialysis patients with FD.
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Observational Study
Changes in the Left Ventricular Ejection Fraction and Outcomes in Hospitalized Heart Failure Patients with Mid-range Ejection Fraction: A Prospective Observational Study.
Objective Current clinical guidelines have proposed heart failure (HF) with mid-range ejection fraction (HFmrEF), defined as a left ventricular ejection fraction (LVEF) of 40-49%, but the proportion and prognosis of patients transitioning toward HF with a reduced LVEF (LVEF <40%, HFrEF) or HF with a preserved LVEF (LVEF ≥50%, HFpEF) are not fully clear. The present study prospectively evaluated the changes in the LVEF one year after discharge and the outcomes of hospitalized patients with HFmrEF. Methods We prospectively studied 259 hospitalized patients with HFmrEF who were discharged alive at our institutions between 2015 and 2019. ⋯ After adjustment, patients in the worsened group had an increased risk of all-cause mortality compared with those in the improved group [hazard ratio 7.02, 95% confidence interval (CI) 1.13-43.48]. The baseline LVEF (per 1% decrease) and tricuspid annular plane systolic excursion (per 1 mm decrease) were independent predictors of the worsened LVEF category (odds ratio 2.13, 95% CI 1.25-3.63 and odds ratio 1.31, 95% CI 1.01-1.70, respectively). Conclusion Our study showed that a worsened LVEF one year after discharge was associated with a poor prognosis in hospitalized patients with HFmrEF.
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Paraneoplastic neurological syndromes (PNS), such as sensory polyneuropathy, are rare, and serum neuronal antibodies that are used for diagnosing this syndrome are occasionally positive. Similarly, neurological immune-related adverse events due to immune checkpoint inhibitors (ICIs) are also rare. ⋯ We herein report a patient with suspected small cell lung cancer who showed sensory polyneuropathy after treatment with atezolizumab in combination with cytotoxic agents (carboplatin and etoposide) and was doubly positive for serum anti-Hu and anti-SOX-1 antibodies. Treatment with ICI and cytotoxic agents may sometimes lead to the development of PNS.
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TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their underlying pathogenic mechanisms remain unclear. Similarities have been reported in the pathological findings of the lymph nodes of TAFRO syndrome, Multicentric Castleman disease (MCD), and some cases of POEMS syndrome. ⋯ We encountered a case of lymphoproliferative disorder that presented with manifestations of both TAFRO syndrome and POEMS syndrome. This case may be a subtype of idiopathic MCD and will be very important for establishing the disease concept of TAFRO syndrome and POEMS syndrome.