Presentation on theme: "Chapter 23. Bleeding disorders associated with coagulopathy"— Presentation transcript: 1 Chapter 23. [slideplayer.com]

The clinical presentation of a severe factor deficiency is spontaneous bleeding or excessive bleeding following minor trauma in otherwise healthy infants. ICH may be the presenting symptom. [onlinelibrary.wiley.com]

If an abnormal bleeding history is present, focus on disorders associated with bleeding. See table below. [arupconsult.com]

Silberstein, MD, Helen Heslop, MD, Jeffrey Weitz, MD, John Anastasi, MD, and a host of world-class contributors present the expert, evidence-based guidance you need to make optimal use of the newest diagnostic and therapeutic options. [books.google.com]

Control mechanisms to prevent unregulated activity (and tissue damage) are present in each pathway. [primaryimmune.org]

• Sepsis

  Dysgenesis Ace renovascular hypertension Ace Reperfusion Injury Ace, Klk1, Serping1 respiratory system disease Ace rheumatoid arthritis A2m, Bdkrb2 rhinitis Bdkrb1, Bdkrb2 Right Ventricular Hypertrophy Ace sarcoidosis Ace sciatic neuropathy Bdkrb1, Bdkrb2 Sepsis [rgd.mcw.edu]

  DIC • an attempt to correct any reversible cause of DIC; (2) measures to control the major symptom, either bleeding or thrombosis; and (3) a prophylactic regimen to prevent recurrence in cases of chronic DIC. • abruptio placentae or acute bacterial sepsis [slideshare.net]

  Congenital infection, sepsis, significant metabolic disorders (such as tyrosinaemia) and Kasabach‐Merritt syndrome [ 4 ] are a few of the many conditions that need to be considered. [onlinelibrary.wiley.com]

  […] decrease gut flora), malabsorption/malnutrition (vit K is fat-soluble) and Warfarin therapy 1. disseminated intravascular coagulation 2. acquired syndrome characterized by intravascular activation of coagulation 3. causes include - endotoxin causing sepsis [brainscape.com]

• Surgical Procedure

  The emphasis is placed on identification of patients with a potential bleeding problem and management of these patients if surgical procedures are needed. [pocketdentistry.com]

  procedures [ 35 ]. [onlinelibrary.wiley.com]

  DDAVP also has been effectively used as a prophylactic agent in preparation for elective surgical procedures in patients with mild hemophilia A and vWD. 29,31,33 If used as a prophylactic agent to prevent surgical bleeding, it is suggested that an infusion [ahcmedia.com]

• Disability

  Reperfusion Injury Serping1 Splenomegaly Bdkrb1 Spontaneous Abortion F12 Staphylococcal Infections Ace Stomach Neoplasms Ace Stroke Ace, F12, Kng2 substance-induced psychosis Ace Sudden Death Ace Sudden Death, Cardiac Kng2 syndromic X-linked intellectual disability [rgd.mcw.edu]

  KDM6A Xp11.3 Kabuki syndrome 2 300867 300128 X-linked dominant KHK 2p23.3 [Fructosuria] 229800 614058 Autosomal recessive KIAA0319 6p22.3 {Dyslexia, susceptibility to, 2} 600202 609269 Autosomal dominant KIDINS220 2p25.1 Spastic paraplegia, intellectual disability [mnglabs.com]

  […] external ophthalmoplegia POLG Parkinson disease, early-onset POLG SANDO POLG Progressive external ophthalmoplegia POLG2 Xeroderma pigmentosum, variant POLH Obesity, adrenal insufficiency and red hair POMC Obesity POMC Muscle-eye-brain disease POMGNT1 Motor disability [springer.com]

• Congestive Heart Failure

  heart failure Ace coronary artery disease Ace Coronary Disease Ace, F12 coronary restenosis Ace Coronavirus Infections Ace Cough Ace, Bdkrb2, Kng2 Death F12 Diabetes Complications Bdkrb1, Bdkrb2 Diabetes Mellitus, Experimental Ace, Bdkrb1, Bdkrb2, Klk1 [rgd.mcw.edu]

  […] diseases ) Nephrotic syndrome Congestive heart failure Paroxysmal nocturnal hemoglobinuria Physiologic states Pregnancy ( especially postpartum ) Obesity Postoperative state Immobilization Old age DISORDERS OF PLATELET AND VESSEL WALL • usually bleed [slideshare.net]

• Aspiration

  She also had a history of in creased arterial blood pressure in a previous pregnancy in 1982 and therefore had been given 60 mg aspir in per day dur in g this pregnancy. [yumpu.com]

  Xpnpep2 Angioedema Induced by ACE Inhibitors Xpnpep2 Angiotensin I-Converting Enzyme, Benign Serum Increase Ace Animal Disease Models Bdkrb2, Kng2 Animal Mammary Neoplasms Kng2 ankylosing spondylitis Ace Aortic Coarctation Ace Arteriovenous Fistula Ace aspiration [rgd.mcw.edu]

  • examination of the peripheral blood film, an assessment of marrow morphology by examination of an aspirate or biopsy, and an estimate of splenic size by bedside palpation supplemented, if necessary, by ultrasonography or computed tomography (CT) • [slideshare.net]

• Thrombosis

  She had no history of gum or nose bleeds, easy bruis in g, menorrhagia, excessive bleed in g after surgery, or deep ve in thrombosis. She had never required a blood transfusion. [yumpu.com]

  (PMID: 19583818) Li Y … Rosendaal FR (Journal of thrombosis and haemostasis : JTH 2009) 3 22 45 60 Common variation in KLKB1 and essential hypertension risk: tagging-SNP haplotype analysis in a case-control study. [genecards.org]

  Jeffrey Weitz, Professor of Medicine at McMaster University School of Medicine and Executive Director of the Thrombosis and Atherosclerosis Research Institute in Ontario. [books.google.com]

  […] is more common 5. thrombosis is likely due to platelet aggregation and activation of coagulation 6. symptoms include thrombocytopenia, thrombosis, and allergic reactions 1. due to congenital X-linked recessive deficiency of Factor VIII 2. results in [brainscape.com]

  BLEEDING AND THROMBOSIS ! Vessel wall, blood platelets and plasma proteins ! Normal hemostatic system = balance between thrombosis and bleeding ! [slideshare.net]

• Purpura

  It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy, and effects on many othe … Thrombotic Thrombocytopenic Purpura Thrombotic thrombocytopenic purpura (TTP or Moschcowitz syndrome[1]:822) is a [checkrare.com]

  Hematologic disease Brite Human diseases [BR: br08402 ] Cardiovascular diseases Hematologic diseases H01078 Fletcher factor deficiency Human diseases in ICD-11 classification [BR: br08403 ] 03 Diseases of the blood or blood-forming organs Coagulation defects, purpura [genome.jp]

  50,000 - 100,000 "mild prolonged BT ; < 50,000 " easy bruising, purpura, mucosal bleeding, < 20,000 " spontaneous bleeding, internal or intracranial bleed ! [slideshare.net]

  1. immune thrombocytpenic purpura 2. thrombotic thrombocytopenic purpura 3. hemolytic uremic syndrome 4. heparin induced thrombocytopenia 1. immune thrombocytopenic purpura 2. caused by antibodies to platelet antigens 3. antigenic target varies - GPIIb [brainscape.com]

  […] chronic aseptic meningitis, mental retardation, headache lymphatic splenomegaly, occasional lymphadenopathy splenomegaly, occasional lymphadenopathy cervical adenopathy in children not seen rare hepatosplenomegaly, adenopathy vasculitis Henoch-Schonlein purpura [ufrgs.br]

• Arthritis

  […] in knee or hip arthritis in large joints, arthralgia arthralgia, symmetric polyarthritis polyarthralgia polyarthralgia, oligoarthritis, clubbig epiphyseal overgrowth, contractures, intermittent or chronic arthritis, clubbing cutaneous erysipeloid erythema [ufrgs.br]

  […] produces k in in s from high molec- ular weight k in in ogen, which are in volved in various in flammatory processes, uter in e contractility dur in g labor and delivery (3), and various pathological states such as shock, allergic conditions, burns, and arthritis [yumpu.com]

  Kng2 end stage renal failure Ace, Bdkrb1, Klk1 endometriosis Cpm endomyocardial fibrosis Ace Endotoxemia Ace, F12, Serping1 Enterocolitis Klkb1 Eosinophilia Bdkrb2, Kng2 epilepsy Bdkrb1, Bdkrb2 essential thrombocythemia Bdkrb1, Bdkrb2 Experimental Arthritis [rgd.mcw.edu]

  Pancytopenia, arthritis, butterfly rash, diffuse proliferative glomerulonephritis, Libman-Sacks endocarditis. ANA, anti-DNA, anti-Sm, anti-histone (drug-induced lupus). Rx.: steroids Autoantibodies against lacrimal and salivary glands. [cram.com]

  Hypertension Leukemia (chronic lymphocytic) Lung cancer Lupus Macular degeneration (Age-related) Melanoma Multiple Sclerosis Myocardial infarction Obesity Osteoarthritis Peripheral arterial disease Prostate cancer (males only) Psoriasis, Rheumatoid arthritis [21gfox.ca]

• Epistaxis

  Resurfacing the nasomaxillary cavity with radial forearm fasciocutaneous free flaps has been reported to be effective in patients with refractory epistaxis. [pocketdentistry.com]

  (see Epistaxis, [[Epistaxis]]): most common symptom in hereditary hemorrhagic telangiectasia and in young males with Von Willebrand Disease Excessive Menstrual Bleeding Menorrhagia (see Menorrhagia, [[Menorrhagia]]): loss of >80 mL of blood per cycle [mdnxs.com]

  Oral mucous membrane bleeding is common for thrombocytopenic infants; however, gum bleeding and epistaxis hardly ever present in neonates. Joint haemarthroses, typical for severe factor deficiencies, rarely occur before ambulation. [onlinelibrary.wiley.com]

  Common presenting complaints are easy bruising and mucous membrane bleeding, such as epistaxis, gingival, gastrointestinal (GI), and vaginal bleeding. [ahcmedia.com]

  Lacrimo-auriculo-dento-digital syndrome FGFR3 Achondroplasia FGFR3 Crouzon syndrome FGFR3 Thanatophoric dysplasia FGFR3 Hypochondroplasia FGFR3 Thanatophoric dwarfism FGFR3 Craniosynostosis FGFR3 Skeletal dysplasia FGFR3 Menorrhagia FGG Hypofibrinogenaemia FGG Epistaxis [springer.com]

• Suggestibility

  Researchers suggest that this lack (deficiency) of functional plasma kallikrein protein does not generally cause any symptoms because another process called the extrinsic coagulation pathway (also known as the tissue factor pathway) can compensate for [ghr.nlm.nih.gov]

  Additions of fresh frozen plasma corrected her aPTT to 27.6 s at 0 h and 30.7 s at 2 h, suggest in g a factor deficiency rather than the presence of an in hibitor. [yumpu.com]

  Indications for Testing Patients should be evaluated if they have abnormal bleeding history suggestive of an inherited blood coagulation factor deficiency, with or without abnormal PT or PTT, after exclusion of the most common deficiencies (eg, hemophilia [arupconsult.com]

  Neonatal history An otherwise normal neonate with thrombocytopenia is suggestive of NAIT or transfer of maternal antibodies. On the other hand, coagulopathies are usually secondary events. [onlinelibrary.wiley.com]

  Our observations suggest that vitamin D may be considered as an alternative therapy in younger children with congenital ichthyosis and vitamin D deficiency, especially in pigmented skin types. [pediatrics.aappublications.org]

• Seizure

  Babies with this condition are born with multiple brain abnormalities, seizures, lack of muscle tone and other issues concerning their heart, lungs, skin, and bones. This condition usually causes death shortly after birth. [diseaseinfosearch.org]

  Degenerative Myelopathy (SOD1A) Fetal-Onset Neonatal Neuroaxonal Dystrophy (MFN2) Hypomyelination and Tremors (FNIP2) Shaking Puppy Syndrome, X-linked Generalized Tremor Syndrome (PLP) L-2-Hydroxyglutaricaciduria (L2HGDH) Neonatal Encephalopathy with Seizures [embarkvet.com]

  including: Febrile convulsions; Generalized epilepsy with febrile seizure plus (GEFS+); Dravet syndrome/ Severe myoclonic epilepsy in infancy (SMEI)31692 222ds:H00784 Localized autosomal recessive hypotrichosis13892 223ds:H00787 Congenital stationary [carlsbad.health.unm.edu]

  […] plus SCN1A Hemiplegic migraine SCN1A Myoclonic epilepsy of infancy SCN1A Febrile seizures SCN1A Generalised epilepsy with febrile seizures plus 2 SCN1A Epilepsy, generalised, with febrile seizures SCN1B Febrile and afebrile seizures SCN2A Neonatal-infantile [springer.com]

  […] plus, type 5, susceptibility to} 613060 137163 Autosomal dominant GABRG2 5q34 Epilepsy, generalized, with febrile seizures plus, type 3 611277 137164 Autosomal dominant GABRG2 5q34 Febrile seizures, familial, 8 611277 137164 Autosomal dominant GABRG2 [mnglabs.com]

• Stroke

  A few people with prekallikrein deficiency have experienced health problems related to blood clotting such as heart attack, stroke, a clot in the deep veins of the arms or legs ( deep vein thrombosis ), nosebleeds, or excessive bleeding after surgery. [ghr.nlm.nih.gov]

  The condition is usually discovered when blood tests are done for other reasons.A few people with prekallikrein deficiency have experienced health problems related to blood clotting such as heart attack, stroke, a clot in the deep veins of the arms or [ncbi.nlm.nih.gov]

  Bdkrb1, Bdkrb2 Sepsis A2m, Klkb1 severe acute respiratory syndrome Ace Shock, Traumatic Serping1 silicosis Cpm Skeletal Muscle Reperfusion Injury Serping1 Splenomegaly Bdkrb1 Spontaneous Abortion F12 Staphylococcal Infections Ace Stomach Neoplasms Ace Stroke [rgd.mcw.edu]

  White thrombi : arteries ; high blood flow ; rich in platelets ; little fibrin ; emboli " occlusion of arteries like TIA and Stroke ( cerebral arteries ), 6. [slideshare.net]

• Cesarean Section

  We describe the anesthetic management of a cesarean section for a patient with prekallikre in deficiency. [yumpu.com]

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]

Treatment of Hemophilia The cornerstone of the treatment of hemophilia is factor replacement. As with vWD, this is undertaken with the assumption that at the time of an acute hemorrhage, the factor activity level is 0%. [ahcmedia.com]

Features sweeping content updates throughout, including basic science research which serves as a foundation for modern hematology, recent advances in stem cell transplantation, clinical advances in the treatment of each of the hematologic malignancies [books.google.com]

Prophylactic treatments include attenuated androgens and C1 inhibitor. [primaryimmune.org]

CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes. [resourcerepository.org]

Treatment Therapy for HHT remains fragmented and problematic, consisting of laser treatment for cutaneous lesions; split-thickness skin grafting, embolization of arteriovenous communications, or hormonal therapy (estrogen or estrogens plus progesterone [pocketdentistry.com]

Prognosis - Hemophilia C Not supplied. Resources - Hemophilia C Not supplied. [checkorphan.org]

PMID: 2014441 Prognosis Girolami A, Ferrari S, Cosi E, Girolami B Blood Coagul Fibrinolysis 2018 Jul;29(5):423-428. doi: 10.1097/MBC.0000000000000735. [ncbi.nlm.nih.gov]

Etiology Curr Opin Hematol 2016 Sep;23(5):445-52. doi: 10.1097/MOH.0000000000000271. [ncbi.nlm.nih.gov]

Etiology Disorders of Primary Hemostasis (Platelet Plug) Thrombocytopenia See Thrombocytopenia, [[Thrombocytopenia]] Platelet Adhesion Defect Von Willebrand Disease (see Von Willebrand Disease, [[Von Willebrand Disease]]) Inherited Von Willebrand Disease [mdnxs.com]

Therapy of bleeding episodes in the neonate relies upon proper replacement and repeated haemostatic evaluation of patients’ status, while dealing with underlying etiological causes. [onlinelibrary.wiley.com]

Aged plasma: lacks factors V and VIII Fresh absorbed plasma lacks vitamin K factors X, IX,VII, II Aged serum lacks VIII, V, II, I determine etiology of prolonged PT, APTT, identify specific factor of deficiency or inhibitor, reference graph prepared with [quizlet.com]

Genatlas disease for KLKB1 Gene Fletcher factor deficiency,without bleeding diathesis Relevant External Links for KLKB1 Genetic Association Database (GAD) KLKB1 Human Genome Epidemiology (HuGE) Navigator KLKB1 Atlas of Genetics and Cytogenetics in Oncology [genecards.org]

[…] adjusting hormones to levels similar to pregnancy. [ medical citation needed ] See also [ edit ] References [ edit ] ^ a b c d e f g eMedicine - Hemophilia C : Article by Prasad Mathew, MBBS, DCH ^ a b c "Factor XI Deficiency: Background, Pathophysiology, Epidemiology [en.wikipedia.org]

Hemophilia A (factor VIII deficiency) Hemophilia B (factor IX deficiency) Other coagulation factor deficiencies Antithrombin III deficiency Protein C deficiency Protein S deficiency Factor V Leiden mutation Prothrombin G2021A mutation Hyperchomocysteinemia Epidemiology [pocketdentistry.com]

[…] ability by adjusting hormones to levels similar to pregnancy. [ medical citation needed ] See also [ edit ] References [ edit ] ^ a b c d e f g eMedicine - Hemophilia C : Article by Prasad Mathew, MBBS, DCH ^ a b c "Factor XI Deficiency: Background, Pathophysiology [en.wikipedia.org]

Pathophysiology The three phases of hemostasis for controlling bleeding are vascular, platelet, and coagulation. The vascular and platelet phases are referred to as primary, and the coagulation phase is secondary. [pocketdentistry.com]

Anaerobic glycolysis produces lactate with decreased intracellular pH which denatures proteins (coagulation necrosis), cell swelling, entry of calcium and apoptosis Pathophysiology of cell injury in hypoxia ETC fails due to lack of oxygen; 2. [cram.com]

The pathophysiology of bleeding disorders presenting as abnormal uterine bleeding. Am J Obstet Gynecol 1996;175: 770-777. 21. Edlund M, Blomback M, von Schoultz B, et al. On the value of menorrhagia as a predictor for coagulation disorders. [ahcmedia.com]

Prevention - Hemophilia C The drug Cyklokapron is often used as a preventative measure to avoid excessive bleeding during oral surgery. Diagnosis - Hemophilia C Not supplied. Prognosis - Hemophilia C Not supplied. [checkorphan.org]

Blood clots protect the body after an injury by sealing off damaged blood vessels and preventing further blood loss. [ghr.nlm.nih.gov]

On chromosome 17 Normally prevents a cell from entering S phase. Retinoblastoma, osteogenic sarcoma. Inactivated product by cyclin/cdk. [cram.com]

Control mechanisms to prevent unregulated activity (and tissue damage) are present in each pathway. [primaryimmune.org]

This level of personalization may help you take action to detect health conditions early, reduce their effects or aid you in preventing them. [21gfox.ca]