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A 33-year-old man with type 1 diabetes mellitus was admitted to the Internal Medicine Unit due to subacute onset of exertional dyspnea, with evidence at initial blood exams of severe macrocytic anemia with thrombocytopenia, biohumoral signs of hemolysis and 5 schistocytes per magnified field on the blood smear. A thrombotic microangiopathy (TMA) was suspected and plasma exchange (PEX) was started soon, since the risk of a life threatening condition. On the second day, after the results of A Disintegrin And Metalloproteinase with ThromboSpondin-1 motif, member 13 (ADAMTS-13) and reticulocytes were available, a critical reappraisal of the clinical scenario was done. B12 vitamin deficiency was evident after completing the diagnostic work-up. Finally, a diagnosis of "pseudo TMA vitamin B12 deficiency-related" was done. This is an intriguing and rare manifestation of cobalamin deficiency, given the very uncommon occurrence of schistocytes in this condition. "Pseudo TMA vitamin B12 deficiency-related" should be kept in mind when facing the differential diagnosis of microangiopathic anemia in the presence of a low proliferative index. [ABSTRACT FROM AUTHOR]

We aimed to explore the role of procalcitonin (PCT) for the diagnosis of Candida spp. bloodstream infections in a population of critically ill septic patients admitted to internal medicine units. This is a retrospective case-control study considering all cases of candidemia identified in three internal medicine units, from January 1st 2012 to May 31st 2016. For each case of candidemia, two patients with bacteremic sepsis were included in the study as control cases. The end point of the study was to evaluate the diagnostic performance of PCT for the diagnosis of Candida spp. blood stream infections in patients with objectively documented sepsis. Sixty-four patients with candidemia and 128 controls with bacteremia were enrolled. Median and interquartile range (IQR) PCT values are significantly lower in patients with candidemia (0.73; IQR 0.26-1.85 ng/mL) than in those with bacteremia (4.48; IQR 1.10-18.26 ng/mL). At ROC curve analysis, values of PCT greater than 2.5 ng/mL had a negative predictive value (NPV) of 98.3% with an AUC of 0.76 (0.68-0.84 95% CI) for the identification of Candida spp. from blood cultures. At multivariate analysis, a PCT value <2.5 ng/mL showed an odds ratio of 8.57 (95% CI 3.09-23.70; p < 0.0001) for candidemia. In septic patients at risk of Candida infection, a PCT value lower than 2.5 ng/mL should raise the suspicion of candidemia, adding value for considering prompt initiation of antifungal therapy. [ABSTRACT FROM AUTHOR]

Amyloidosis prognosis is often related to the onset of heart failure and a worsening that is concomitant with kidney-liver dysfunction; thus the Model for End-stage Liver disease (MELD) may be an ideal instrument to summarize renal-liver function. Our aim has been to test the MELD score as a prognostic tool in amyloidosis. We evaluated 128 patients, 46 with TTR-related amyloidosis and 82 with AL amyloidosis. All patients had a complete clinical and echocardiography evaluation; overall biohumoral assessment included troponin I, NT-proBNP, creatinine, total bilirubin and INR ratio. The study population was dichotomized at the 12 cut-off level of MELD scores; those with MELD score >12 had a lower survival compared to controls in the study cohort (40.7 vs 66.3 %; p = 0.006). Either as a continuous and dichotomized variable, MELD shows its independent prognostic value at multivariable analysis (HR = 1.199, 95 % CI 1.082-1.329; HR = 2.707, 95 % CI 1.075-6.817, respectively). MELD shows a lower prognostic sensitivity/specificity ratio than troponin I and NT-proBNP in the whole study population and AL subgroup, while in TTR patients MELD has a higher sensitivity/specificity ratio compared to troponin and NT-proBNP (ROC analysis-AUC: 0.853 vs 0.726 vs 0.659). MELD is able to predict prognosis in amyloidosis. A MELD score >12 selects a subgroup of patients with a higher risk of death. The predictive accuracy seems to be more evident in TTR patients in whom currently no effective scoring systems have been validated. [ABSTRACT FROM AUTHOR]

Amyloidosis comprises a unique group of diseases that share in common the extracellular deposition of insoluble fibrillar proteins in organs and tissue including the heart. Cardiac amyloidosis could be primary a part of systemic acquired amyloidosis, or a result of heredofamilial amyloidosis. Although the infiltration of the heart from different types of amyloid results in restrictive cardiomyopathy that manifests with refractory congestive heart failure and conduction abnormalities, unequivocal identification of the deposited amyloidogenic protein is mandatory in order to avoid misdiagnosis and inappropriate treatment. Recent developments in imaging techniques and extracardiac tissue biopsy have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis of a patient with amyloidosis is still poor and depends upon the underlying disease, and the type and degree of dysfunction of the involved organs. Thus, early diagnosis is mandatory because patients with advanced disease are usually too ill for intensive therapy. This review outlines current approaches to diagnosis, assessment of disease severity, and treatment of cardiac amyloidosis. [ABSTRACT FROM AUTHOR]