Hemolytic paroxysmal nocturnal hemoglobinuria: 20 years of medical progress

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by intravascular hemolysis, thrombosis and bone marrow failure. Prior to the availability of specific therapy, PNH led to the death of around half of affected individuals, mainly through thrombotic complications, with a particular grim prognosis for patients presenting with classic PNH. The anti-C5 monoclonal antibody eculizumab has revolutionized treatment, controlling intravascular hemolysis and thrombosis occurrence, with improved long-term survival. However, eculizumab is infused on a lifelong 2 week basis and most of the patients are anemic, with some remaining transfusion-dependent. New anti-C5 agents reproduce the safety and efficacy of eculizumab, with improved patient convenience, while proximal complement inhibitors have been developed to address C3-mediated extravascular hemolysis, aiming to eventually improve hematological response. This review will describe the spectacular medical progress in PNH of the last 20 years, as well as the risks and benefits of a novel approach.     

Colorectal cancer screening:20 years of development and recent progress

Colorectal cancer(CRC)is the second most common cancer in Europe and its incidence is steadily increasing.This trend could be reversed through timely secondary prevention(screening).In the last twenty years,CRC screening programs across Europe have experienced considerable improvements(fecal occult blood testing;transition from opportunistic to population based program settings).The Czech Republic is a typical example of a country with a long history of nationwide CRC screening programs in the face of very high CRC incidence and mortality rates.Each year,approximately 8000 people are diagnosed with CRC and some 4000 die from this malignancy.Twenty years ago,the first pilot studies on CRC screening led to the introduction of the opportunistic Czech National Colorectal Cancer Screening Program in 2000.Originally,this program was based on the guaiac fecal occult blood test(FOBT)offered by general practitioners,followed by colonoscopy in cases of FOBT positivity.The program has continuously evolved,namely with the implementation of immunochemical FOBTs and screening colonoscopy,as well as the involvement of gynecologists.Since the establishment of the Czech CRC Screening Registry in 2006,2405850 FOBTs have been performed and 104565 preventive colonoscopies recorded within the screening program.The overall program expanded to cover 25.0%of the target population by 2011.However,stagnation in the annual number of performed FOBTs lately has led to switching to the option of a population-based program with personal invitation,which is currently being prepared.     

Then and now: The progress in hepatitis B treatment over the past 20 years

The ultimate goals of treating chronic hepatitis B(CHB)is prevention of hepatocellular carcinoma(HCC)and hepatic decompensation.Since the advent of effective antiviral drugs that appeared during the past two decades,considerable advances have been made not only in controlling hepatitis B virus(HBV)infection,but also in preventing and reducing the incidence of liver cirrhosis and HCC.Furthermore,several recent studies have suggested the possibility of reducing the incidence of recurrent or new HCC in patients even after they have developed HCC.Currently,six medications are available for HBV treatment including,interferon and five nucleoside/nucleotide analogues.In this review,we will examine the antiviral drugs and the progresses that have been made with antiviral treatments in the field of CHB.     

Successful defibrillation using double sequence defibrillation: Case reports

Introduction:Defibrillation is effective and the most common treatment for ventricular fibrillation (VF) and pulseless ventricular tachycardia in patients with cardiac arrest.Patient concerns:Recently we experienced 3 cases refractory ventricular fibrillation (RVF) which was successfully terminated with double sequence defibrillation (DSD) in our emergency department, so we’d like to report and discuss it.Diagnosis:Cardiac arrestInterventions:A single defibrillation 200J was performed twice for patients with ventricular fibrillation in the initial rhythm of the emergency room. At the same time, intubation and intravenous access were achieved and epinephrine and amiodarone were administered. The 400J DSD was performed on RVF patients with sustained VFs, despite several trials of 150-200J defibrillation and adherence to advanced cardiac life support.Outcomes:All three RVF patients recovered spontaneous circulation after DSD.Conclusion:The three cases we have shown are small, but DSD improves the chance of spontaneous circulation. Therefore it is suggested that attempts of DSD to patients with RVF, especially in the prehospital stages as a way to improve the return of spontaneous circulation.     

Takayasu arteritis: Follow-up studies for 20 years

We reviewed retrospectively 126 (5 male, 121 female) patients suffering from Takayasu arteritis who had been treated in our clinics from 1971 to 1990. The patients' ages ranged from 19 to 80yrs old (1990) with a mean age of 48.7 ± 11.8 years. HLA typing analysis in 98 patients revealed that 45 patients (47%) were confirmed as carrying the Bw52 antigen, a high result that is statistically significant as compared with that in healthy Japanese. Arteriograms (performed in 75 patients) revealed that 28 patients (37%) were affected in the aorta and its main branches by this disease (type IV by Nasu's classification) and 23 patients (31%) were affected only in the main branches (type I). The C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) improved significantly from 2.55 ± 0.28(+) and 57.0 ± 5.69 mm/hr to 0.53 ± 0.12(+) and 31.2 ± 3.45 mm/hr, respectively after treatment including steroid and antiplatelet therapy (P < 0.01).=" patients=" with=" bw52=" exhibited=" more=" severe=" inflammatory=" conditions=" than=" those=" without=" bw52.=" lung=" scintillations=" performed=" in=" 81=" patients=" showed=" pulmonary=" arterial=" lesions=" in=" 50=" patients=" (62%).=" echocardiograms=" revealed=" aortic=" regurgitation=" (ar)=" in=" 44=" patients=" (35%),=" with=" a=" significant=" difference=" noted=" between=" the=" bw52=" positive=" group=" and=" the=" bw52=" negative=" group=" [29/40=" (73%)=" versus=" 11/47=" (23%),=">P < 0.001].=" patients=" with=" bw52=" were=" prescribed=" higher=" doses=" of=" steroids=">P < 0.05)=" for=" longer=" periods=">P < 0.01)=" than=" those=" without=" bw52.=" of=" 11=" patients=" who=" died=" during=" our=" study=" period,=" 7=" died=" of=" cardiac=" complications,=" all=" of=" whom=" were=" suffering=" from=" ar.=" hla=" analysis=" performed=" in=" 6=" of=" these=" 7=" patients=" revealed=" that=" all=" carried=" the=" bw52=" antigen.=" in=" conclusion,=" the=" retrospective=" survey=" revealed=" that=" patients=" carrying=" the=" bw52=" antigen=" showed=" more=" severe=" inflammatory=" conditions=" and=" progressed=" more=" rapidly=" to=" complications=" and=" the=" fatal=" morbid=" condition,=" as=" compared=" with=" those=" without=" bw52.=" this=" suggests=" the=" important=" role=" of=" gene=" disequilibrium=" with=" this=" hla=">