Objective: Differential diagnosis of dyspnea is vital for the management of respiratory failure, where routine parameters can now be integrated with thoracic ultrasound data. The objective of this study was to evaluate the validity and accuracy of this approach in a department of internal medicine. Materials and methods: We enrolled 152 patients consecutively hospitalized with a diagnosis of dyspnea. After clinical evaluation, chest radiography, biochemical assays (NT-proBNP), and emergency treatment, patients underwent ultrasound examination of the lungs. Results were considered positive if the total number of lines B was higher than 8. The ultrasound examination and NT-proBNP assay were repeated after 48 h. The gold standard was the clinical diagnosis of heart failure made by medical experts in accordance with AHA guidelines. Results: The group of patients with positive ultrasound findings had a higher frequency of heart failure diagnoses (X 2 92.5, p < 0.005) and significantly higher values of NT-proBNP (10,384 ng/l vs 3889 ng/l, p < 0.05). Moreover, the decrease in the number of B lines at 48 h was significantly greater (p < 0.005) among patients treated for heart failure. There were no significant changes in the values of NT-proBNP (p Z 0.37). Discussion: In conclusion we have shown that even in a department of internal medicine, lung ultrasonography is a useful tool for diagnosing respiratory insufficiency and monitoring its response to therapy.Sommario La diagnosi differenziale di dispnea è fondamentale per la gestione dell'insufficienza respiratoria in cui, ai parametri routinari, può essere ora affiancata l'ecografia toracica. L'obiettivo di questo studio è stato valutare la validità e l'accuratezza di questa metodica anche in un reparto di medicina interna. dell'NT-proBNP sono stati ripetuti dopo 48 ore. Il gold standard di riferimento è stato la diagnosi clinica di scompenso cardiaco fatta da medici esperti secondo le linee guida dell'AHA. Risultati: Il gruppo di pazienti che mostrava un esame ecografico positivo riceveva in percentuale maggiore la diagnosi finale di insufficienza cardiaca (X 2 92.5; p < 0.005) e valori significativamente più elevati di NT-proBNP (10,384 ng/l vs 3889 ng/l; p < 0.05).Inoltre la diminuzione delle linee B a 48 ore era significativamente maggiore (p < 0.005) nel gruppo di pazienti trattati per scompenso cardiaco mentre non vi erano cambiamenti significativi nei valori di NT-proBNP (p Z 0.37). Discussione: In conclusione abbiamo dimostrato che anche in un reparto di medicina interna l'ecografia polmonare è uno strumento diagnostico utile per le gestione dell'insufficienza respiratoria e il suo monitoraggio durante la terapia. ª

The occurrence of thrombotic events in patients with congenital bleeding conditions has received considerable attention in recent years. The same is true for asymptomatic defects of factors of the contact phase of blood coagulation, mainly FXII. Anecdotal reports on thrombosis in patients with prekallikrein deficiency have occasionally been reported. These involved both arterial and venous thrombosis. The purpose of the present article is to analyze the stories and the clinical pictures of all 75 cases of prekallikrein deficiency published so far. Among these patients were 9 with thrombosis, 6 arterial (myocardial infarction and ischemic stroke) and 3 venous (deep vein thrombosis with or without pulmonary embolism). In 6 cases acquired thrombosis risk factors were present; in 2 cases no associated risk factors were present and in 1 case no information was supplied in this regard. One patient who presented both a stroke and a pulmonary embolism had a fatal outcome. The article clearly indicates that prekallikrein deficiency does not protect from thrombosis in spite of the severe in vitro coagulation defect.

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Congenital Factor X deficiency is commonly classified as type I, in which there is a concomitant decrease of activity and antigen (CRM negative), and in type II, in which activity is low but antigen is normal or near normal (CRM positive). During the past decades it was shown that type II was by itself very heterogeneous. It was shown in fact that some forms showed a defect in all three assay systems (extrinsic, intrinsic, and RVV dependent), whereas others showed a defect only in two of the three systems. Molecular biology analysis, whenever available, has failed so far to supply clear explanations for these discrepancies. The purpose of the present article was an attempt to correlate the clotting activities seen in these two defects with other clotting, chromogenic, immunological assays, and molecular biology results. There are in the literature 10 families that show a predominant defect in the extrinsic system, and four families that show a predominant defect in the intrinsic system. All patients showed a normal, near normal, or reduced level of antigen that is always definitively higher than the clotting counterpart. Molecular biology studies revealed mutations in different exons, namely 2, 4, 5, 6, and 8. These mutations in different exons do not allow any clear genotypephenotype conclusions, but indicate that mutations in different exons may give rise to the same phenotype. The study underlines the importance of a multipronged evaluation of all cases with Factor X deficiency. In fact only by this approach can an acceptable classification of the defect be reached. Am. J. Hematol. 83:668-671, 2008. V