Uncontrolled postoperative bleeding in woman with pemphigus and undiagnosed acquired haemophilia A

Nabyta hemofi lia A (AHA) jest skazą krwotoczną wywołaną przez nagłe pojawienie się przeciwciał przeciw czynnikowi krzepnięcia VIII u osoby z negatywnym wywiadem w kierunku zaburzeń krzepnięcia krwi. W obrazie klinicznym choroby dominują wynaczynienia krwi, które prowadzą do zgonu nawet w 22% przypadków. Pacjentka w wieku 55 lat z rozpoznaniem pęcherzycy liściastej była hospitalizowana z powodu nasilenia zmian skórnych, pod postacią sączących się nadżerekz tendencją do erytodermii. W trakcie hospitalizacji obserwowano znaczne pogorszenie stanu ogólnego, narastanie niedokrwistości, silne bóle brzucha oraz wydłużenie czasu częściowej tromboplastynypo aktywacji. W badaniu tomografi i komputerowej stwierdzono obecność rozległych krwiaków w obrębie jamy otrzewnej oraz przestrzeni zaotrzewnowej. Chorej przetoczono świeżo mrożoneosocze (FFP) i poddano ją zabiegowi chirurgicznej ewakuacji krwiaków. Dodatkowo w trakcie zabiegu usunięto torbiel jajnika oraz śledzionę z powodu krwawienia w lewej okolicy podprzeponowej. W okresie pooperacyjnym obserwowano uporczywe krwawienie z rany pooperacyjnej mimo codziennych transfuzji FFP. Podejrzenie nabytej hemofi lii A potwierdzono, oznaczając aktywnośćczynnika VIII (20 jm./dl) i stwierdzając obecność inhibitora czynnika VIII w mianie 1,8 jB./ml. Zastosowano rekombinowany aktywny czynnik VII (rFVIIa) i rozpoczęto leczenie immunosupresyjne. Podczas zmniejszania dawki rFVIIa obserwowano nawrót krwawienia. Chora otrzymywała koncentraty omijające inhibitor łącznie przez 39 dni. W trakcie terapii rozpoznano zewnętrzną przetokę moczową oraz obserwowano przetrwałe krwiaki o podobnej lokalizacji i rozmiarze, jakwyjściowo. Po 6 tygodniach leczenia immunosupresyjnego wyeliminowano inhibitor, a chorą poddano operacjom chirurgicznym, których celem było usunięcie zhemolizowanych krwiaków orazrekonstrukcja pęcherza moczowego. W okresie okołooperacyjnym nie obserwowano skłonności do nadmiernych krwawień. Podsumowując, u chorego z obrazem niewyjaśnionej skazy krwotocznej nie należy przeprowadzać żadnych procedur inwazyjnych. W przypadku rozpoznania AHA zabiegi chirurgiczne, o ile to możliwe, należy odłożyć do czasu wyeliminowania inhibitoraAcquired haemophilia A (AHA) is caused by sudden appearance of autoantibodies against factor VIII (FVIII). The disease presents with severe or life-threatening haemorrhage in patients with nopersonal history of bleeding. The mortality in AHA patients is estimated at even 22%. A 55-year-old female was admitted to the local hospital due to exacerbation of pemphigus foliaceus. After admission she presented rapid deterioration of general condition. Laboratory tests revealed rapidly increasing anaemia and prolongation of activated partial thrombin time (APTT). Truncal CT-scan showed extensive haematomas localized intra-abdominally as well as within left iliac and obturatormuscles. The patient received fresh frozen plasma (FFP) followed by surgical intervention. Additionally, an ovarian cyst was removed. Due to unlocalized intraoperative bleeding from leftsubphrenic area a formal splenectomy was performed. The uncontrolled bleeding from postoperative wound was observed after surgery. Daily FFP transfusions did not reduce blood loss and theAPTT was not corrected. Detailed hematological tests revealed decreased factor VIII activity to 20 IU/dl and the presence of antibodies against factor VIII in the titer of 1.8 BU/ml. The AHAwas diagnosed. To control the bleeding recombinant FVIIa was used successfully. Synchronously, the immunosupressive treatment was administered. Due to recurrent bleeding the treatment withby-passing agents was continued for 39 days. During therapy urinary cutaneous fi stula was observed. In the control CT-scan a persistent intraabdominal and intramuscular haematomas were presented (localized similarly as before the treatment). Six weeks of immunosupressive therapy eradicated the FVIII inhibitor. After eradication of FVIII inhibitor a surgical bloodless removal of hematomas and open bladder reconstruction were performed. The additional transfusions of red blood cells and fresh frozen plasma were not necessary. In conclusion, in patients presenting spontaneous bleeding to muscles and/or retroperitoneal space we suggest the delaying of surgical intervention until the detailed coagulation tests have been performed. Ideally, patients diagnosed with AHA should not undergo surgical interventions

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore, early diagnosis should be established. A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-five inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs, e.g., sotatercept, have been intensively investigated in clinical trials. We aim to review the literature on recent advances in the treatment strategy and prognosis of patients with PAH-CTD. In this manuscript, we discuss the mechanism of action of PAH-specific drugs and new agents and the latest research conducted on PAH-CTD patients

Machine-learning-based diagnostics of cardiac sarcoidosis using multi-chamber wall motion analyses

Background: Hindered by its unspecific clinical and phenotypical presentation, cardiac sarcoidosis (CS) remains a challenging diagnosis. Objective: Utilizing cardiac magnetic resonance imaging (CMR), we acquired multi-chamber volumetrics and strain feature tracking for a support vector machine learning (SVM)-based diagnostic approach to CS. Method: Forty-five CMR-negative (CMR(−), 56.5(53.0;63.0)years), eighteen CMR-positive (CMR(+), 64.0(57.8;67.0)years) sarcoidosis patients and forty-four controls (CTRL, 56.5(53.0;63.0)years)) underwent CMR examination. Cardiac parameters were processed using the classifiers of logistic regression, KNN(K-nearest-neighbor), DT (decision tree), RF (random forest), SVM, GBoost, XGBoost, Voting and feature selection. Results: In a three-cluster analysis of CTRL versus vs. CMR(+) vs. CMR(−), RF and Voting classifier yielded the highest prediction rates (81.82%). The two-cluster analysis of CTRL vs. all sarcoidosis (All Sarc.) yielded high prediction rates with the classifiers logistic regression, RF and SVM (96.97%), and low prediction rates for the analysis of CMR(+) vs. CMR(−), which were augmented using feature selection with logistic regression (89.47%). Conclusion: Multi-chamber cardiac function and strain-based supervised machine learning provides a non-contrast approach to accurately differentiate between healthy individuals and sarcoidosis patients. Feature selection overcomes the algorithmically challenging discrimination between CMR(+) and CMR(−) patients, yielding high accuracy predictions. The study findings imply higher prevalence of cardiac involvement than previously anticipated, which may impact clinical disease management

1000 Liver Transplantations at the Department of General, Transplant and Liver Surgery, Medical University of Warsaw - Analysis of Indications and Results

The aim of the study was to analyze indications and results of the first one thousand liver transplantations at Chair and Clinic of General, Transplantation and Liver Surgery, Medical University of Warsaw.Material and methods. Data from 1000 transplantations (944 patients) performed at Chair and Clinic of General, Transplantation and Liver Surgery between 1994 and 2011 were analyzed retrospectively. These included 943 first transplantations and 55 retransplantations and 2 re-retransplantations. Frequency of particular indications for first transplantation and retransplantations was established. Perioperative mortality was defined as death within 30 days after the transplantation. Kaplan-Meier survival analysis was used to estimate 5-year patient and graft survival.Results. The most common indications for first transplantation included: liver failure caused by hepatitis C infection (27.8%) and hepatitis B infection (18%) and alcoholic liver disease (17.7%). Early ( 6 months) retransplantations were dominated by hepatic artery thrombosis (54.3%) and recurrence of the underlying disease (45%). Perioperative mortality rate was 8.9% for first transplantations and 34.5% for retransplantations. Five-year patient and graft survival rate was 74.3% and 71%, respectively, after first transplantations and 54.7% and 52.9%, respectively, after retransplantations.Conclusions. Development of liver transplantation program provided more than 1000 transplantations and excellent long-term results. Liver failure caused by hepatitis C and B infections remains the most common cause of liver transplantation and structure of other indications is consistent with European data

1000 Liver Transplantations at the Department of General, Transplant and Liver Surgery, Medical University of Warsaw - Analysis of Indications and Results

The aim of the study was to analyze indications and results of the first one thousand liver transplantations at Chair and Clinic of General, Transplantation and Liver Surgery, Medical University of Warsaw.Material and methods. Data from 1000 transplantations (944 patients) performed at Chair and Clinic of General, Transplantation and Liver Surgery between 1994 and 2011 were analyzed retrospectively. These included 943 first transplantations and 55 retransplantations and 2 re-retransplantations. Frequency of particular indications for first transplantation and retransplantations was established. Perioperative mortality was defined as death within 30 days after the transplantation. Kaplan-Meier survival analysis was used to estimate 5-year patient and graft survival.Results. The most common indications for first transplantation included: liver failure caused by hepatitis C infection (27.8%) and hepatitis B infection (18%) and alcoholic liver disease (17.7%). Early ( 6 months) retransplantations were dominated by hepatic artery thrombosis (54.3%) and recurrence of the underlying disease (45%). Perioperative mortality rate was 8.9% for first transplantations and 34.5% for retransplantations. Five-year patient and graft survival rate was 74.3% and 71%, respectively, after first transplantations and 54.7% and 52.9%, respectively, after retransplantations.Conclusions. Development of liver transplantation program provided more than 1000 transplantations and excellent long-term results. Liver failure caused by hepatitis C and B infections remains the most common cause of liver transplantation and structure of other indications is consistent with European data