Dramatic decreases of all haemorrhagic coagulation factors by acquired inhibitors after extended left lobectomy

ConclusionWe have described the first case of acquired inhibition of all coagulation factors associated with extended left lobectomy.
Source: International Journal of Surgery Case Reports - Category: Surgery Source Type: research

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Cystic artery pseudoaneurysm (CAP) is a rare disease, with small number of previous reports related to CAP. Besides, it is frequently prone to critical condition due to arterial bleeding. Here, we presented a case of ruptured CAP with acute calculus cholecystitis and its subsequent successful management with temporary endoscopic biliary drainage for obstructive jaundice and embolization for the culprit artery without cholecystectomy. Since CAP is at high risk of bleeding, intravascular treatment, which is only one currently available therapeutic option, is urgently required in the clinical sites.Case Rep Gastroenterol 2019;13:89 –94
Source: Case Reports in Gastroenterology - Category: Gastroenterology Source Type: research
Abstract Hereditary hemorrhagic telangiectasia (HHT) is a vascular rare disease characterized by nose and gastrointestinal bleeding, skin and mucosa telangiectasias, and arteriovenous malformations in internal organs. HHT shows an autosomal dominant inheritance and a worldwide prevalence of approximately 1:5000 individuals. In>80% of patients, HHT is caused by mutations in either ENG (HHT1) or ACVRL1 (HHT2) genes, which code for the membrane proteins Endoglin and Activin A Receptor Type II-Like Kinase 1 (ALK1), respectively, both belonging to the TGF-β/BMP signaling pathway. In this work, we describe a nov...
Source: Gene - Category: Genetics & Stem Cells Authors: Tags: Gene Source Type: research
This study sought to identify hemophilia carriers in C ôte d’Iv...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
AbstractUterine sarcomas are uncommon and aggressive tumors comprising 3 –7% of all uterine malignancies. The aim is to evaluate clinical presentation, histopathologic pattern, recurrence pattern, and outcome of patients with uterine sarcomas presenting to a tertiary care cancer center over an 8-year period. A total of 11 cases of uterine sarcoma were diagnosed. The me dian age of patients at presentation was 51 years (range 30–67 years). Six patients had leiomyosarcoma (54.5%), 4 had endometrial stromal sarcoma (36%), and 1 had adenosarcoma (9%). The main presenting symptoms were abnormal vaginal ble...
Source: Indian Journal of Surgical Oncology - Category: Cancer & Oncology Source Type: research
This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrosp ective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001‐2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to s urgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwe...
Source: Pediatric Transplantation - Category: Transplant Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
BackgroundAlthough significant advances have been made over the past 10 years in the treatment of Immune Thrombocytopenia (ITP), challenges remain in addressing the burden of disease and unmet needs of patients. In rare diseases especially, natural history studies and patient registries are crucial in establishing baseline information and identifying patient-reported outcomes.AimsThe registry collects data on the natural progression of ITP, and characterizes the ITP population as a whole. This preliminary analysis seeks to provide an abridged overview of registry findings.MethodsThe ITP Registry enrolled 843 patients (pts)...
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
In this study, the potential of PF-06741086 to restore thrombin generation in rare disease plasma was explored.Thrombin generation was measured in citrated platelet poor Factor XI (FXI), Factor V (FV), FVII, von Willebrand Factor (vWF) deficient (Type 1, 2A, 2B and 3) congenital donor plasma following the in vitro addition of PF-06741086 (0, 1, 10 or 100 nM) or a human IgG1 antibody; initiated with 1 pM TF and 4 µM phospholipid. FXI, FV, and FVII donors had less than 1% coagulation factor activity. Non-hemophilic plasma from healthy donors alone was also included in the analysis. In FXI deficient plasmas, a concentra...
Source: Blood - Category: Hematology Authors: Tags: 321. Blood Coagulation and Fibrinolytic Factors: Poster II Source Type: research
A 52-year-old male with history of excessive alcohol use was evaluated for abdominal pain and hematochezia. Colonoscopy was only significant for a single diverticulum in the transverse colon and esophagogastroduodenoscopy was negative. Deep anterograde and spiral retrograde endoscopies also did not reveal any abnormality. Intra-operative enteroscopy showed few non-bleeding small angioectasias in the jejunum and ileum that were ligated. Despite these interventions, the patient presented again with hematochezia and epigastric pain.
Source: Digestive and Liver Disease - Category: Gastroenterology Authors: Tags: Image of the Month Source Type: research
ConclusionThe characteristics of the umbilical tumor, associated with the cyclical nature of tumor bleeding in a patient without previous history of endometriosis, strongly suggest the diagnosis of Villar's nodule, but the confirmation is still histological. The treatment is always surgical and recurrence is very rare.
Source: International Journal of Surgery Case Reports - Category: Surgery Source Type: research
ConclusionsA pharmacometric approach guided the phase III dose selection of emicizumab in hemophilia A, without conducting a conventional dose-finding study. Phase III studies with the selected dosing regimens are currently ongoing. This case study indicates that a pharmacometric approach can substitute for a conventional dose-finding study in rare diseases and will streamline the drug development process.
Source: Clinical Pharmacokinetics - Category: Drugs & Pharmacology Source Type: research
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