Articles: disease.
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The Goodpasture syndrome is an extremely rare disease, with renal and pulmonary manifestations, and is mediated by anti-glomerular basement membrane (anti-GBM) antibodies. Renal pathological changes are mainly characterized by glomerular crescent formation and linear immunofluorescent staining for immunoglobulin G on the GBM. There are few reports on the atypical course of the syndrome involving serum-negative anti-GBM antibodies. Therefore, we present a case of Goodpasture syndrome that presented with nephrotic-range proteinuria and was seronegative for anti-GBM antibodies. ⋯ This extremely rare case of Goodpasture syndrome manifested with seronegativity for anti-GBM antibodies and nephrotic-range proteinuria. Our findings emphasize the importance of renal biopsy for the clinical diagnosis of atypical cases. Furthermore, because renal involvement achieved only partial remission despite therapy, early detection and active treatment of the Goodpasture syndrome is necessary to improve the prognosis of patients.
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The most common cardiac involvement of Fabry disease (FD) is left ventricular hypertrophy (LVH), which usually occurs in male patients over the age of 30. In rare cases, it can progress to ventricular dilation in the late stage of the disease. ⋯ Cardiac involvement of FD can progress rapidly in some cases. Screening for FD should be considered in patients with unexplained ventricular dilation, especially in those with a history of typical FD manifestations.
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Observational Study
Nomogram to predict risk for early ischemic stroke by non-invasive method.
Stroke is the acute onset of neurological deficits and is associated with high morbidity, mortality, and disease burden. In the present study, we aimed to develop a scientific, nomogram for non-invasive predicting risk for early ischemic stroke, in order to improve stroke prevention efforts among high-risk groups. Data were obtained from a total of 2151 patients with early ischemic stroke from October 2017 to September 2018 and from 1527 healthy controls. ⋯ A nomogram predicting ischemic stroke for all patients had an internally validated concordance index of 0.911. The area under the ROC curve for the logistic regression model was 0.782 (95% confidence interval [CI]: 0.766-0.799, P < .001), with a cutoff value of 2.5. The nomogram developed in this study can be used as a primary non-invasive prevention tool for early ischemic stroke and is expected to provide data support for the revision of current guidelines.
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Observational Study
Admitted AIDS-associated Kaposi sarcoma patients: Indications for admission and predictors of mortality.
Kaposi sarcoma (KS) is an AIDS-defining angioproliferative malignancy associated with high morbidity and mortality. Most KS patients in regions with high incidence such as sub-Saharan Africa present late with advanced stage disease. Admitted KS patients have high mortality rates. ⋯ However, only sepsis (or signs and symptoms of sepsis) were independently associated with mortality after controlling for confounders. In conclusion, common reasons for admission of epidemic KS patients include advanced disease, severe anemia, respiratory tract infections, and signs and symptoms of sepsis. Signs and symptoms of sepsis are independent predictors of mortality in these patients.
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Observational Study
Complete blood count reflecting the disease status of giant cell arteritis: A retrospective study of Chinese patients.
Giant cell arteritis (GCA) is the most common vasculitis in elderly, with ischemic and constitutional symptoms caused by vascular involvement and systemic inflammation. Early initiation of therapy results in prompt remission, while patients may still experience flares or severe complications during glucocorticoid tapering. This study was to identify the characteristics of Chinese GCA patients with different prognosis. ⋯ Lymphocyte counts were lower in patients with unfavourable prognosis (P = .014) after treatment. Complete blood count may reflect the disease status of GCA. Little change in monocyte during treatment and lower lymphocytes after treatment may serve as potential predictors of unfavourable clinical prognosis.