Bleeding disorders

Hemophilia is a rare bleeding disorder caused by a genetic defect on chromosome X. It is inherited as an X-linked trait, and hence, it is more frequently diagnosed in males, whereas women have been traditionally considered only as carriers of the disease. However, the role of women in families of patients with hemophilia is pivotal. As mothers, sisters, daughters, and female partners of patients with hemophilia, they play a central role in the management of the patient, considering healthcare, social, and familial aspects, but they might be affected by the disease as well, particularly in regions where consanguinity is frequent...

A 75-year-old woman on tumor necrosis factor inhibitors for rheumatoid arthritis presented with hematemesis and a gastric biopsy revealed diffuse large B-cell lymphoma with possible bulky left liver tumor involvement. On the second day of treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, the patient experienced abdominal pain followed by shock vitals. A contrast-enhanced computed tomography scan revealed a ruptured liver. Transcatheter arterial embolization (TAE) was performed to stop the bleeding...

BACKGROUND: β-thalassemia is a group of inherited blood disorders that affect the production of β-globin chains, leading to the reduction or absence of these chains. One of the complications observed in patients with β-thalassemia major (β-TM) is thrombosis, especially in those who receive frequent blood transfusions. This may be due to a decrease in the levels of the natural anticoagulants: protein C (PC), total protein S (PS), and antithrombin (AT). METHODS: In this case-control study, patients with β-TM, who had received at least 20 packed cell transfusions during their lifetime, were included...

Chronic neutrophilic leukemia (CNL) is a rare BCR::ABL1-negative myeloproliferative neoplasm (MPN) defined by persistent mature neutrophilic leukocytosis and bone marrow granulocyte hyperplasia. Atypical chronic myeloid leukemia (aCML) (myelodysplastic "[MDS]/MPN with neutrophilia" per World Health Organization [WHO]) is a MDS/MPN overlap disorder featuring dysplastic neutrophilia and circulating myeloid precursors. Both manifest with frequent hepatosplenomegaly and less commonly, bleeding, with high rates of leukemic transformation and death...

OBJECTIVE: The PN Naloxone Nasal Swab (Pocket Naloxone Corp., Bethesda, MD) is a swab optimized for drug delivery and intended for use by non-medical personnel for the emergency treatment of opioid overdose. The aim of this study (PNC-20-003) is to determine the safety of this nasal swab in a real-world environment. METHODS: This was a single-institution, quantitative-qualitative prospective trial performed at an outpatient clinic. Patients with normal or abnormal nasal structure were recruited...

BACKGROUND: For the majority of patients with atrial fibrillation (AF), disease management has improved in recent years. However, there are still populations underrepresented or excluded in current registries and randomized controlled trials. HERA-FIB (Heidelberg Registry of Atrial Fibrillation) was planned to assess real-world evidence for the prevalence, demographic characteristics and management of patients with the diagnosis of AF presenting consecutively to a chest pain unit. METHODS AND RESULTS: HERA-FIB is a retrospective, observational, single-center study on patients with a diagnosis of AF presenting to a chest pain unit from June 2009 until March 2020...

BACKGROUND: Although age and body mass index (BMI) significantly affect platelet reactivity units and clinical outcomes after percutaneous coronary intervention, there are limited data on the relationship between high on-treatment platelet reactivity (HPR) and clinical outcomes on age and BMI differences. Thus, we investigated the association of HPR with clinical outcomes according to age and BMI. METHODS AND RESULTS: The study analyzed 11 714 patients who underwent platelet function tests after percutaneous coronary intervention...

BACKGROUND: Next-day discharge (NDD) outcomes following uncomplicated self-expanding transcatheter aortic valve replacement have not been studied. Here, we compare readmission rates and clinical outcomes in NDD versus non-NDD transcatheter aortic valve replacement with Evolut. METHODS AND RESULTS: Society of Thoracic Surgeons/American College of Cardiology TVT (Transcatheter Valve Therapy) Registry patients (n=29 597) undergoing elective transcatheter aortic valve replacement with self-expanding supra-annular valves (Evolut R, PRO, and PRO+) from July 2019 to June 2021 were stratified by postprocedure length of stay: ≤1 day (NDD) versus >1 day (non-NDD)...

Immune thrombocytopenic purpura (ITP) is a hematologic condition characterized by decreased circulating platelets, resulting in bruising, bleeding gums, and internal bleeding. This disorder can be categorized into two primary forms based on the duration of symptoms and underlying causes. Acute ITP primarily affects young children, typically between the ages of two and six, but it can also impact older children and adults. Viral infections like chickenpox, respiratory infections, or gastroenteritis often precede it...

Abnormal uterine bleeding (AUB) during the menopausal transition results in reproductive endocrine disorders and both physiological and pathological changes, substantially impacting women's health. This study aimed to investigate the factors influencing AUB in perimenopausal women. Between April 2021 and June 2022, 120 perimenopausal women with AUB in the menopausal transition, diagnosed and treated at the Gynaecology Department of Kunming Tongren Hospital, were included in the case group. Concurrently, women undergoing routine health examinations at the same hospital were randomly selected as the control group...

INTRODUCTION: Angiodysplasia is the most common vascular abnormality in gastrointestinal tract, commonly occurs in right colon. CASE PRESENTATION: 42-year-old male presented with massive per rectal bleeding for one day duration. He is a known type 2 diabetes mellitus patient and not known to having any bleeding disorders or on any anticoagulant medication. He had no gastrointestinal red flag symptoms. He was hemodynamically stable, and rest of the examination was unremarkable...

Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by autoantibodies against FVIII. Severe AHA is life-threatening. Currently, licensed hemostatic agents for the treatment of severe AHA have short half-lives and require intravenous administration, leading to a need for hospitalization, higher costs, and negative effects on quality of life. We present two cases of severe AHA with high inhibitor titers where emicizumab was safely and effectively used with intensive immunosuppression...

BACKGROUND: Relaparotomy following a cesarean delivery (CD) is an infrequent complication, with inconsistency regarding risk factors and indications for its occurrence. We therefore aimed to determine risk factors and indications for a relaparotomy following a CD at a single large tertiary center. METHODS: A retrospective case-control single-center study (2013-2023). We identified all women who had a relaparotomy up to six weeks following a CD (study group). Maternal characteristics, obstetrical and surgical data were compared to a control group in a 1:2 ratio...

INTRODUCTION: Venlafaxine (VEN) is a selective norepinephrine reuptake inhibitor (SNRI) that mainly helps treat major depressive disorder and anxiety and panic disorders. It works by inhibiting the reuptake of serotonin (5-hydroxytryptamine, 5-HT) and noradrenaline (NA) by presynaptic neurons. Additionally, VEN administration has been linked with a bleeding predisposition, that may be due to the inhibition of NA and 5-HT uptake by platelets which have their own receptors on their surface and are implicated in platelet aggregation...

INTRODUCTION: Many women experience bleeding disorders that may have an anatomical or unexplained origin. Although hysterectomy is the most definitive and common treatment, it is highly invasive and resource-intensive. Less invasive therapies are therefore advised before hysterectomy for women with fibroids or bleeding disorders. This study has two aims related to treating bleeding disorders and uterine fibroids in the Netherlands: (1) to evaluate the regional variations in prevalence and surgical approaches; and (2) to assess the associations between regional rates of hysterectomies and less invasive surgical techniques to analyze whether hysterectomy can be replaced in routine practice...

BACKGROUND: Non-cirrhotic portal hypertension (NCPH) is a spectrum of liver diseases, including porto-sinusoidal vascular disorder, with portal hypertension (PH) in the absence of cirrhosis. The natural history and diagnostic approach to NCPH are not well understood. AIM: We aimed to evaluate disease progression and outcomes in NCPH. METHODS: Patients with or at risk for NCPH were enrolled in a single centre prospective study; two groups were formed based on the presence of specific features of PH, such as varices, collaterals, portal hypertensive gastropathy or portal hypertensive bleeding...

BACKGROUND: Individuals with bleeding disorders have been reported to have a number of oral health issues due to varying conditions. A comprehensive evaluation of the different oral health conditions has not been carried out in the past. This systematic review and meta-analysis was carried out to collate and critically analyse existing research, and provide a comprehensive overview of the current state of knowledge on oral health. METHODS: A comprehensive search was conducted in electronic databases, including PubMed, Scopus and Embase, in October 2023...

OBJECTIVE: To investigate the efficacy of using pre-sliding technique to prevent postoperative shortening of displaced femoral neck fracture fixed with femoral neck system (FNS). METHODS: Retrospective analysis of 110 cases of displaced femoral neck fracture treated with femoral neck system from September 2019 to November 2022 in our center, which were divided into 56 cases in the pre-sliding group and 54 cases in the traditional group. The baseline data such as gender, age, side, mechanism of injury, fracture type, operation time, intraoperative bleeding were recorded and compared between the two groups, and the quality of fracture reduction, shortening distance, Tip Apex Distance (TAD), union time, Harris score of the hip were also compared between the two groups...

In the era of direct oral anticoagulants, vitamin K antagonists retain a clinically relevant role in thrombotic disorders. In Italy, approximately 20% of the patients on anticoagulant therapies receives a VKA, in most cases warfarin. The optimal management of this drug is challenging and cannot disregard its intricate and unpredictable pharmacokinetic properties and patient's thrombotic and bleeding risk. Several clinical issues encountered during warfarin treatment are still unanswered and are tentatively addressed by physicians...

BACKGROUND:  Hemophilia A (HA) is an inherited bleeding disorder caused by a deficiency or defect in factor VIII (FVIII). METHODS:  We investigated the role of clot waveform analysis (CWA) of activated partial thromboplastin time in the diagnosis and therapeutic monitoring of HA. The changes in CWA parameters the maximum clotting velocity (|Min1|), maximum clotting acceleration (|Min2|), and maximum clotting deceleration (|Max2|) were detected among mild, moderate, and severe HA groups...