Presentation
Diagnostic Evaluation Presentation Profile Adolescents with heavy menstrual bleeding should be carefully evaluated and the presentation profile is quite important when one is considering a platelet function disorder etiology. [omicsonline.org]
The most common factor deficiencies are Factor VIII (Hemophilia A) and Factor IX (Hemophilia B), which are indistinguishable based on clinical presentation alone. [iupui.edu]
Thus, the clinical presentation (mild or moderate bleeding) and laboratory profile for these disorders are similar. [clinchem.aaccjnls.org]
The current pregnancy had a breech presentation, for which the patient was scheduled for a cesarean delivery. [anesthesiology.pubs.asahq.org]
Common symptoms are present across all variances. Severe bleeding disorders tend to result in more severe symptoms. [babymed.com]
Workup
If the patient has a strong personal or family history of mucocutaneous bleeding, VWD testing should be pursued early in the workup. [clinchem.aaccjnls.org]
A peripheral smear serves several purposes in a bleeding workup. [aafp.org]
A more in-depth hematologic workup is required to confirm the diagnosis of vWD. Hemophilia A (factor VIII deficiency) does not affect the platelet count or the PT. [ahcmedia.com]
Causes, etiology and diagnosis of acquired von Willebrand disease: a prospective diagnostic workup to establish the most effective therapeutic strategies. Acta Haematol 2009; 121 : 177–182. 84. Nichols WL, Rick ME, Ortel TL, et al. [nature.com]
Serum
- Anisocytosis
Laboratory studies show decreased numbers of large platelets and anisocytosis, but the platelets show no in vitro functional abnormalities. [malacards.org]
Laboratory studies show decreased numbers of large platelets and anisocytosis, but the platelets show no in vitro functional abnormalities. {ECO:0000269 PubMed:23434115, ECO:0000269 PubMed:24069336}. [genecards.org]
Laboratory studies show decreased numbers of large platelets and anisocytosis, but the platelets show no in vitro functional abnormalities. {ECO:0000269|PubMed: 23434115, ECO:0000269|PubMed: 24069336 }. [web.expasy.org]
Treatment
Covering how to assess both bleeding children and adults, Haemophilia A and B, molecular basis of the disease, the role of factors in coagulation, epidemiology, pharmacokinetics, and treatment of inhibitors. [books.google.de]
Treatments The attending physician will prescribe treatment based on the type of bleeding disorder. [babymed.com]
The average cost for treating hemophilia is about $60,000 annually, but some may need hundreds of thousands of dollars worth of treatment every year. There are no generic substitutes for these treatments. [newenglandhemophilia.org]
Screening for disorders of hemostasis before starting hormonal treatment is recommended because treatment may interfere with the results and combined oral contraceptive treatment should be interrupted for accurate von Willebrand disease testing. [acog.org]
Prognosis
Life Expectancy Prognosis for patients with bleeding disorders is disease or disorder specific. If the bleeding disorder is caused by an underlying disease, the prognosis will depend on treatment of the disease. [babymed.com]
Prognosis Good Poor A variety of drugs have been reported to cause thrombocytopenia either by drug-induced platelet destruction or bone marrow suppression. Heparin merits special emphasis because it is so commonly used. [hawaii.edu]
Prognosis Poor prognosis is associated with persistent severe thrombocytopenia (less than 10,000/uL) that is refractory to platelet transfusions; prolonged PT or PTT values that show modest reductions toward normal with plasma or factor concentrates; [clinicaladvisor.com]
Plasma exchange therapy, introduced 30 years ago, has dramatically improved the prognosis for patients with TTP. [emedicine.medscape.com]
Prognosis. Scot Med J 1957; 2: 200-15. Pubmed van Swieten JC, et al. Interobserver agreement for the assessment of handicap in stroke patients. Stroke 1988; 19: 604-7. [att.ebm-library.jp]
Etiology
Results: Heavy menstrual bleeding affects the quality of life of many adolescents, and PFDs appearing to be an important underlying etiology of this disorder. [omicsonline.org]
[…] factor VIII has shorter half life and its plasma levels are lower Note: type O patients have lower levels of vWF (75 IU/dl), type AB patients have highest levels (123 IU/dl) ( Blood 1987;69:1691 ); levels increase with age and with acute phase reactions Etiology [pathologyoutlines.com]
If initial testing does not reveal an etiology in a patient with a high suspicion for a bleeding disorder, the patient should be referred to a hematologist for additional evaluation. [aafp.org]
In addition, the etiology of the thrombocytopenia is often unclear. [anesthesiology.pubs.asahq.org]
Disease monitoring, follow-up and disposition Follow-up Thrombocytopenias with a variety of etiologies: follow platelet count. [clinicaladvisor.com]
Epidemiology
Covering how to assess both bleeding children and adults, Haemophilia A and B, molecular basis of the disease, the role of factors in coagulation, epidemiology, pharmacokinetics, and treatment of inhibitors. [books.google.de]
Relevant External Links for ACTN1 Genetic Association Database (GAD) ACTN1 Human Genome Epidemiology (HuGE) Navigator ACTN1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: ACTN1 No data available for Genatlas for ACTN1 Gene Postsynaptic [genecards.org]
[…] intravascular coagulation Impaired hepatic production of coagulation factors: e.g., liver cirrhosis Other important types Von Willebrand disease Definition : : a disorder of primary hemostasis characterized by either a deficiency or disorder of vWF Epidemiology [amboss.com]
Epidemiology Most bleeding is a result of a structural lesion. If the ICU patient is bleeding from a single site, the likelihood of bleeding from a structural lesion, rather than a coagulopathy, is high. [clinicaladvisor.com]
Pathophysiology
Due to its unique features and its similarity to the more common type 2B VWD, many investigators were intrigued by its nature, pathophysiology and molecular genetics aspects. [ncbi.nlm.nih.gov]
Nature 369:64–67 PubMed CrossRef Google Scholar Bick RL (1998) Disseminated intravascular coagulation: pathophysiological mechanisms and manifestations. [link.springer.com]
References: [1] [2] [3] [4] [5] [6] [7] [8] Pathophysiology Activation of hemostasis Primary hemostasis : hemostasis achieved via platelet adhesion and aggregation at the site of endothelial injury Initiation : endothelial injury results in transient [amboss.com]
Pathophysiology When an injury to the blood vessel wall disrupts its integrity, bleeding can result. [clinicaladvisor.com]
Thus, the pathophysiology of this disease is similar to that of the hemolytic disease of newborns. Unlike hemolytic disease, however, thrombocytopenia occurs during the first pregnancy in 50% of cases. [emedicine.medscape.com]
Prevention
On the other hand, infected people don’t require isolation—it does little to prevent the transmission of hepatitis A, and it won’t prevent the transmission of hepatitis B or C. Vaccinations are available against hepatitis A and B. [arizonahemophilia.org]
Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection. [books.google.de]
According to the US Centers for Disease Control and Prevention, hemophilia occurs in approximately 1 in 5,000 live births. There are about 20,000 people with hemophilia in the US. All races and ethnic groups are affected. [sangredeoro.org]
Bleeding Problems Platelet transfusions are typically not used prophylactically in WAS to increase the platelet count in an attempt to prevent bleeding episodes. [primaryimmune.org]