treatment bleeding diathesis

OBJECTIVE: To assess patient factors to predict treatment success of Naseptin for recurrent paediatric epistaxis. METHODS: This prospective cohort study of paediatric patients referred to a tertiary paediatric otolaryngology clinic with recurrent epistaxis treated with Naseptin cream and education. Patients with red flag symptoms and bleeding diathesis were omitted, along with patients with concurrent otolaryngology complaints. Statistical analysis included logistic regression analysis to assess for predictive factors contributing to treatment success...

An altered thrombo-hemorrhagic profile has long been observed in patients with myeloproliferative neoplasms (MPNs). We hypothesized that this observed clinical phenotype may result from altered expression of genes known to harbor genetic variants in bleeding, thrombotic, or platelet disorders. Here, we identify 32 genes from a clinically validated gene panel that were also significantly differentially expressed in platelets from MPN patients as opposed to healthy donors. This work begins to unravel previously unclear mechanisms underlying an important clinical reality in MPNs...

Although the presence of glycogen in platelets was established in the 1960s, its importance to specific functions ( i.e ., activation, secretion, aggregation, and clot contraction) remains unclear. Patients with glycogen storage disease often present with increased bleeding and glycogen phosphorylase (GP) inhibitors, when used as treatments for diabetes, induce bleeding in preclinical studies suggesting some role for this form of glucose in hemostasis. In the present work, we examined how glycogen mobilization affects platelet function using GP inhibitors (CP316819 and CP91149) and a battery of ex vivo assays...

PURPOSE OF REVIEW: Chediak-Higashi syndrome is a rare autosomal recessive disorder characterized by congenital immunodeficiency, bleeding diathesis, pyogenic infection, partial oculocutaneous albinism, and progressive neurodegeneration. Treatment is hematopoietic stem cell transplantation or bone marrow transplantation; however, this does not treat the neurologic aspect of the disease. Mutations in the lysosomal trafficking regulator (LYST) gene were identified to be causative of Chediak-Higashi, but despite many analyses, there is little functional information about the LYST protein...

Vaccination against SARS-CoV2 has been the largest vaccination campaign over the past two decades. The aim of this study is to qualitatively assess the reported cases of acquired hemophilia A (AHA) that developed after COVID-19 vaccination to further elaborate on incidence, presentation, treatment, and outcomes.We queried Medline (PubMed), Google Scholar, and Embase databases to find reported cases of AHA after COVID-19 vaccines. We found 14 studies (19 cases) for this descriptive analysis. Most patients were elderly (mean age 73 years) and males ( n = 12) with multiple comorbidities...

Amyloidosis is a diverse entity that poses both diagnostic and treatment challenges. Whether systemic or local, amyloidosis has varied manifestations including occasional hepatic involvement. Hepatic amyloidosis, although rare, should be on the differential for those with unexplained hepatomegaly, cholestasis, alkaline phosphatase elevations, other associated organomegaly, and those with certain epidemiologic risks. In this study, we report a case of a man with systemic amyloid light chain amyloidosis with multiorgan involvement, acute liver injury, cholestasis, nephrotic syndrome, cardiomegaly, and bleeding diathesis...

With the contemporary KDOQI, a patient-focused approach in vascular access care is emphasized more than ever when planning RRT. Nevertheless, functional vascular access continues to be the Achilles' heel for successful hemodialysis in specific patient sub-groups, such as the Hemophilia-A population. The newer percutaneous endovascular approach is a safer alternative when conventional surgical AVF poses high bleeding risks perioperatively, which subsequently prevents ESKD patients to have desired permanent dialysis access...

Presentation of cervico-thoracic extradural hematoma in pediatric age is rare with stroke-like features. Its association with COVID-19 in the active stage of the disease had not been reported and its management presents a management dilemma as COVID-19 with stroke-like features. A 14-year-old boy was referred to our institute with complaints of sudden-onset upper and middle back pain, associated with loss of sensation below the middle of the back, sudden progressive weakness of both lower limbs (power 0/5) and upper limbs (power grade-2/5), and incontinence of urine, following bouts of vomiting 12 days back...

Uncontrolled hemorrhage is a leading cause of death, emphasizing the need for novel hemostatic agents. Here, a novel hemostatic polysaccharide hemoadhican (HD) has been screened out by analyzing the rheological properties of screened material mixed blood sludges, which is prepared by mixing polysaccharide granules and whole blood to mimic the coagulation in vitro. HD is produced by a bacterial isolate Paenibacillus sp.1229, and the repeating units of HD were →)-α-L-Rhap-(1→3)-β-D-Glcp-(1→4)[4,6-ethylidene-α-D-Galp-(1→4)-α-D-Glcp-(1→3)]-α-D-Manp-(1→...

BACKGROUND: The present study aimed to compare clinical features, management, and outcomes between children and adolescents admitted as cases of multi-system inflammatory syndrome in children (MIS-C) in Indira Gandhi Medical College (IGMC), Shimla. MATERIAL AND METHODS: We conducted a cross-sectional study for MIS-C from January to July 2021, in the pediatric ward of IGMC in Himachal Pradesh. All children admitted with a diagnosis of MIS-C were included in the study...

Coagulation is a crucial biological mechanism in human bodies to prevent blood loss. Abnormal coagulation can cause bleeding diathesis or thrombosis, common pathologic conditions in our clinical practice. Many individuals and organizations have dedicated their efforts in the past decades to understanding the biological and pathological mechanisms of coagulation and developing laboratory testing tools and treatment options to help patients with bleeding or thrombotic conditions. Since 1926, the Mayo Clinic coagulation group has made significant contributions to the clinical and laboratory practice, basic and translational research on various hemostatic and thrombotic disorders, and the education and collaboration to share and advance our knowledge in coagulation through a highly integrated team and practice model...

ANAMNESIS AND CLINICAL EXAMINATION: A 40-year-old male patient presented to our emergency department with a new onset of hemorrhagic diathesis. Clinically, there were marked bleeding stigmata with extensive ecchymosis in the thigh area and oral mucosal hemorrhage with otherwise general well-being. DIAGNOSTICS: The coagulation diagnostics performed were consistent with the picture of disseminated intravascular consumption coagulopathy. Microscopic blood count also revealed 74% morphologically atypical promyelocytes...

Even with extensive transfusion support, trauma-induced bleeding often leads to death. Early intervention may improve outcomes, yet which blood products, factor concentrates, or other drugs constitute optimal treatment is unclear. Patients with acute traumatic coagulopathy (ATC), arising from trauma and haemorrhagic shock, have the worst prognosis. Here, multiple interventions were compared in a mouse model of ATC. After the trauma of tissue excision, anaesthetized mice were bled to 35 mm Hg mean arterial pressure, maintained under shock for 60 min, and resuscitated with fluids equal in volume to the shed blood...

Although transcatheter aortic valve implantation (TAVI), which is a less invasive standard treatment for aortic stenosis than surgery, has been recommended even in low-risk patients, its effectiveness in bicuspid aortic valve is still unclear. Cardiac surgery has been proven to cause serious complications in hematological diseases with factor deficiency or bleeding diathesis. In this case, which is the first in the literature to our knowledge, we tried to present the successful TAVI procedure in a young patient with bicuspid aortic stenosis and factor 7, 11 deficiency complicated by atrial fibrillation...

PURPOSE: Noonan syndrome (NS) is a rare neurodevelopmental syndrome characterized by dysmorphic features, congenital heart defects, neurodevelopmental delay, and bleeding diathesis. Though rare, several neurosurgical manifestations have been associated with NS, such as Chiari malformation (CM-I), syringomyelia, brain tumors, moyamoya, and craniosynostosis. We describe our experience in treating children with NS and various neurosurgical conditions, and review the current literature on neurosurgical aspects of NS...

BACKGROUND: Delayed diagnosis of hypothyroidism may result in atypical presentations. Here, we report a case with decreased serum level and activity of von Willebrand factor due to untreated profound hypothyroidism. OBSERVATION: A 9-year-old girl, presented with prolonged gingival bleeding after dental extraction. Clinical findings of the case were consistent with hypothyroidism, and the laboratory workup results revealed decreased serum level and activity of von Willebrand factor associated with profound hypothyroidism...

Factor V deficiency is a congenital bleeding diathesis that, in selected cases, may be managed with liver transplant. In this case, we describe the treatment of an adult patient with kidney failure secondary to juvenile onset polycystic kidney disease who received a combined liver-kidney transplant as a method to manage the risks associated with the need for a kidney transplantin the setting of factorV deficiency and high sensitization.

Identifying underlying bleeding diathesis that is amenable to medical therapy must be determined to provide timely treatment and minimize morbidity. Nasal bleeding is viewed as an annoyance by most who suffer from its episodes. However, it can at times be a baleful ailment that can compromise a patient's airway, breathing, and circulation, which can result in death. A 75-year-old Hispanic man presented with life-threatening epistaxis and was ultimately diagnosed with multiple myeloma (MM). The patient suffered profuse bleeding and hemodynamic compromise, requiring endoscopic nasal packing, red cell transfusions, platelet transfusions, and right external carotid artery angiogram with maxillary arteries embolization prior to chemotherapy...

PURPOSE: To discuss the pathophysiology, etiology, and current management strategies of uveitis-glaucoma-hyphema (UGH) syndrome. METHODS: Literature review. RESULTS: The classic UGH syndrome associated with anterior chamber intraocular lenses (ACIOL) have decreased in incidence with the modernization of IOL design and surgical techniques. The current UGH syndrome is increasing in prevalence largely related to a parallel increase in late onset dislocations of intraocular lenses (IOLs) and the developing techniques to remedy that condition...

BACKGROUND: The aim of this study is to compare the postoperative functional outcomes of two different surgical techniques for the correction of senile lower lid entropion. It was a Quasi experimental study, carried out at BHY Hospital, Karachi, Pakistan from January to December 2018. METHODS: This study recruited fifty patients belonging to either gender with an age range of 60-70 years having unilateral lower lid senile entropion. All the patients were briefed about the study dynamics and divided into two groups with twenty-five patients in each group via convenience-based sampling technique...