Objective A rise in infections with multidrug-resistant Pseudomonas aeruginosa (MDR-PA) is a significant contributor to increased morbidity and mortality of patients with hematologic malignancies. ...The aim of this study was to determine the efficacy and safety of colistin (colistimethate sodium) in the treatment of serious infections caused by MDR-PA in these patients. Patients and Methods A matched pair analysis of renal function, toxicities, and outcome of 26 patients receiving colistin and control subjects was done. All patients had clinical signs of sepsis; P. aeruginosa was isolated from blood in 69% of patients in colistin group and 84% in control group. Patients treated with colistin received 3 million units every 8 hours for a median duration of 13 days. Additionally, patients received at least two additional antimicrobial or antifungal drugs. Results Resolution of infection was achieved in twenty patients (76.9%) receiving colistin and in 17 (65.4%) control subjects. Mortality rate was 11% in both groups. There was no statistically significant difference in the level of serum creatinine, creatinine clearance, or potassium levels before and after treatment between groups. Only one patient receiving colistin developed de novo renal failure and one displayed transient neurologic toxicity. Conclusion Our results suggest that in patients with hematologic malignancies, colistin is effective in treating severe infections caused by MDR-PA while maintaining an acceptable toxicity profile. Prospective randomized studies comparing efficacy and safety of colistin with those of other antipseudomonal drugs are needed.
Over the last decades, individualized approaches and a better understanding of coagulopathy complexity in end-stage liver disease (ESLD) patients has evolved. The risk of both thrombosis and bleeding ...during minimally invasive interventions or surgery is associated with a worse outcome in this patient population. Despite deranged quantitative and qualitative coagulation laboratory parameters, prophylactic coagulation management is unnecessary for patients who do not bleed. Transfusion of red blood cells (RBCs) and blood products carries independent risks for morbidity and mortality, including modulation of the immune system with increased risk for nosocomial infections. Optimal coagulation management in these complex patients should be based on the analysis of standard coagulation tests (SCTs) and viscoelastic tests (VETs). VETs represent an individualized approach to patients and can provide information about coagulation dynamics in a concise period of time. This narrative review will deliver the pathophysiology of deranged hemostasis in ESLD, explore the difficulties of evaluating the coagulopathies in liver disease patients, and examine the use of VET assays and management of coagulopathy using coagulation factors. Methods: A selective literature search with PubMed as the central database was performed with the following.
Currarino syndrome is a rare set of congenital anomalies that include partial sacral agenesis, anorectal anomalies, presacral mass, urogenital malformation, and fistula between pelvic structures. We ...present a case of a 4-year and 10-month-old boy with incomplete Currarino syndrome, who was born with anus atresia, rectovesical fistula, and permanent perimembranous VSD. At the age of 3, he was diagnosed with neurogenic bladder and sacrococcygeal agenesis. Early psychomotor development was normal.
Cytogenetic GTG-banding test confirmed a male karyotype 46, XY with high heterochromatin in chromosome 9, without mutation of the MNX 1 gene (chromosome 7q36). This genetic analysis is a result of "de novo mutation" or it is the disorder of DNA methylation.
Further genetics analyses like whole-exome sequencing - WES should have been preformed if the test had been availble.
The existence of Currarino syndrome should be suspected among the children born with anorectal malformation. Prompt diagnosis with multidisciplinary monitoring improves the care and quality of life of the patient, reduces morbidity and mortality.
florio HAEMO is a new hemophilia treatment monitoring application consisting of a patient smartphone application (app) and a web‐based dashboard for healthcare professionals, providing several novel ...features, including activity tracking, wearable connectivity, kids and caregiver mode, and real‐time pharmacokinetic factor level estimation.
To assess intuitiveness, ease‐of‐use, and patient preference of florio HAEMO in Central Europe using a cross‐sectional survey.
This survey was conducted in six Central European countries between 9 December 2020 and 24 May 2021. The online questionnaire included 17 questions about overall satisfaction, ease‐of‐use, intuitiveness, and patient preference. Adults or children with hemophilia on regular prophylaxis and using the florio HAEMO app for a minimum of 1 week were invited to complete the online questionnaire by their treating physician.
Sixty‐six participants took part in the survey. The median duration for all respondents using the florio HAEMO app was 3 to 4 weeks. Overall, 89.4% of users reported being very satisfied or rather satisfied after using florio HAEMO. Of the 23 respondents who had switched from another hemophilia app, 87.0% indicated that they strongly preferred or preferred using florio HAEMO. Most florio HAEMO users reported that the app was very easy or rather easy to use (97.0%) and intuitive (94.0%). florio HAEMO had a positive impact on daily living, with 78.8% of users reporting that the app was very important or rather important to them.
This survey suggests that florio HAEMO is an easy‐to‐use and intuitive app to assist self‐management of home prophylaxis.
We here present the successful initial treatment and secondary prophylaxis of superficial venous thrombosis secondary to Behçet’s disease by a novel anticoagulant drug, rivaroxaban (Xarelto®). To our ...knowledge, this is the first case of using an oral direct inhibitor of FXa in this setting. Our findings are promising; the outcome was favourable without any adverse effect noted. We propose that the patients with Behçet’s disease and venous thrombosis might benefit from the advantages of the new anticoagulant drug.
: Hemophilia A is an inherited disorder that is characterized by decreased or absent factor (F)VIII and an increased risk of bleeding. Clinical presentation of the severe form of the disease includes ...spontaneous bleeding into the joints and muscles, while patients with milder forms usually exhibit trauma-associated bleeding. The treatment of hemophilia aims to prevent bleeding. A number of clotting FVIII concentrates are available for managing hemophilia A, which have different safety and efficacy characteristics. Advancements in biotechnology have enabled development of recombinant factor concentrates, which thus minimize the risk of transmitting infectious diseases. Turoctocog alfa (NovoEight®, Novo Nordisk A/S, Bagsvaerd, Denmark) was the first third-generation B-domain truncated recombinant FVIII.
: The manuscript describes the characteristics of turoctocog alfa, as well as its efficacy and safety for prophylaxis and on-demand treatment for patients with severe hemophilia A without inhibitors.
: In clinical trials, turoctocog alfa has demonstrated very good efficacy and safety for the prophylaxis and on-demand treatment of hemophilia A patients, as well as high hemostatic activity during surgery and in managing bleeding episodes. Post-marketing studies and real-life data are anticipated to further reinforce the value of long-term prophylaxis, and estimate the incidence of inhibitors to FVIII.
Although venous thromboembolism (VTE) including deep venous thrombosis (DVT) and pulmonary embolism is a major health problem in the world, it is an infrequent disease among young people. It is ...always mandatory to look at the underlying conditions for VTE, and in young patients, inherited prothrombotic factors should also be evaluated, especially in case of unprovoked VTE. Anomalies of inferior vena cava (IVC) are very rare in the general population. In this case report we describe rare occurrence of extensive DVT in a young male patient with rare anomaly of IVC - duplication of IVC - as a predisposition factor for DVT. Physicians need to be reminded of the IVC anomalies that should be considered in young patients with idiopathic DVT of lower extremity, which may require extended anticoagulant treatment.
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