أسئلة Bleeding أطفال

Introduction to Hemostasis

• Hemostasis is the body's ability to minimize blood loss after a blood vessel injury.
• Hemostasis depends on the function of blood vessels, platelets and coagulation.
• Blood vessels contribute through contraction.
• Platelets need to be in normal numbers, between 150,000 – 400,000 / mm3.
• Coagulation requires interaction between 13 clotting factors

Bleeding Disorders: Etiology

• Can be caused by vascular disorders, platelet disorders, or coagulation disorders

Vascular Disorders

• Result in vascular purpura.

Platelet Disorders

• Are characterized by decreased platelet number, leading to thrombocytopenia, or impaired platelet function, leading to thrombasthenia
• Can lead to purpura and ecchymosis

Coagulation Disorders

• Caused by a deficiency in one or more coagulation factors, such as hemophilia.

General Clinical Picture

• Includes external bleeding, internal bleeding, and cutaneous bleeding

External Bleeding

• Manifests as epistaxis, bleeding gums, hematemesis, hemoptysis, hematuria, or bleeding per rectum

Internal Bleeding

• May involve intracranial or intra-abdominal bleeding

Cutaneous Bleeding

• Includes petechiae and ecchymosis.

Petechiae

• Are small, intradermal hemorrhages, usually a few mm in size
• Not related to trauma, non-raised, red-purple color
• Do not disappear with color changes

Ecchymosis

• Subcutaneous hemorrhages, usually a few cm in size
• Usually related to trauma, raised surface, typically blue
• Disappear with color changes

Causes of Cutaneous Bleeding

• Either vascular disorder or platelet disorder may cause petechiae
• May be caused by vascular, platelet, or clotting factor disorders may cause ecchymosis

Vascular Purpura: Etiology

• Can be acquired or congenital
• Acquired causes fall under “STUDY FAD”

Acquired Vascular Purpura

• Scurvy (vitamin C deficiency)
• Steroid use
• Trauma, Uremia
• Disseminated intravascular coagulation (DIC)
• Vasculitis-related infections like IE, Typhoid, or Meningococcemia
• Fat embolism after a bone fracture
• Purpura factitia (self-induced, more common in females)
• Anaphylactoid purpura (most common type)
• Drug hypersensitivity

Congenital Vascular Purpura

• Congenital Amyloidosis
• Ehlers-Danlos syndrome (↑ Skin elasticity, joint extensibility, capillary fragility)
• Osler-Weber-Rendu disease (thin dilatation of capillary walls, leading to rupture and bleeding)

Scurvy

• Bleeding caused by decreased platelet aggregation and vascular defects.
• Vitamin C is vital for collagen formation linking endothelial cells
• Gum hypertrophy and tender bone condition may result if vitamin C deficient
• Treat with Vitamin C at 200-500 mg/day orally

Steroid Purpura

• Prolonged steroid use thins blood vessel walls
• Elevated levels of endogenous steroids such as Cushing syndrome increases chance

Traumatic Asphyxia

• Increased intra-capillary pressure ruptures blood vessels
• Caused by crushing injuries, epileptic fits or severe coughing fits

Anaphylactoid Purpura (Henoch-Schönlein Purpura)

• Small blood vessel allergic vasculitis similar to rheumatic fever.
• IgA mediated

Epidemiology of HSP

• Most common cause of non-thrombocytopenic purpura in children
• Typically affects individuals aged 2-8 years, with a higher incidence in males

Etiology of HSP

• Immunoglobulin A mediated, triggered by infections, insect bites, or food allergies

Clinical Presentation of HSP

• Presents with elevated purpura on the buttocks and extremities, ecchymosis and angioneurotic edema
• Migratory polyarthritis
• Acute abdominal pain, diarrhea, bloody stool
• Asymptomatic proteinuria and hematuria, nephrotic or nephritic syndrome, acute kidney injury

Skin Manifestations of HSP

• Skin manifestations are essential to diagnose HSP.

Renal Involvement in HSP

• Renal involvement carries the worst prognosis.

Complications of Bleeding Disorders

• Intussusception is the most dangerous
• Chronic Renal Failure
• Sub arachnoid or cerebral hemorrhage
• Testicular Torsion

Bleeding Disorder Investigations

• Bleeding and clotting times are normal except for the Hiss test

Lab Findings

• Elevated ASO and Serum IgA
• Stool analysis reveals occult blood.
• Urine analysis indicates renal affection.
• Skin biopsy shows Vasculitis with IgA deposition

HSP Treatment

• HSP is self-limited, but recurrence is possible within 6 months
• Treat streptococcal infections to prevent secondary causes
• Try to identify and remove the cause of condition
• Aspirin is administered for arthritis
• Corticosteroids and immunosuppressive drugs helps GIT and renal affections

Treatment of Acute Abdomen

• Anaphylactoid purpura can cause acute abdomen and should be tested for

Platelet Purpura

• Platelet numbers = 150,000-400,000/mm3.
• ⅓ located in spleen, ⅔ in the blood stream
• Life span is 7-10 days.
• Size is 1-4 µm (younger platelets larger)
• Mean platelet volume is 7.1 FL

Platelet Function

• Action is related to platelet aggregation, adhesion and clot retraction

Thrombocytopenia

• Characterized by a platelet count 40,000/mm3
• Associated with very high levels of antiplatelet antibodies with high IgA levels
• Typically responds well to corticosteroids, but has a less favorable prognosis

Persistent ITP

• Exists for 3-12 months

Clinical Findings in ITP

• Purpura, ecchymosis, and hematoma with orificial bleeding
• There is normally no splenomegaly, hepatomegaly, or lymphadenopathy

Intracranial Hemorrhage (ICH) and ITP

• If ICH happens it happens 10 days after ITP attack when PLT count is less than 10.000/mm³
• ICH happens in 0.1% of cases, and requires patient hospitalization for 10 days.

Infantile ITP

• ITP is more abrupt, severe and frequent in infants
• Involves prolonged test times, lowered platelet numbers (below 150,000)/mm3, a positive capillary fragility test, and elevated levels of anti-platelet antibodies
• Bone marrow examination indicates diagnostic test

Lab Findings

• Megakaryocytes are normal with D2 rapid release

ITP Treatment

• Self-limiting, patients should be under observation for fear of ICH

Medical Intervention

• Includes Corticosteroids and Immunosuppressive drugs and IV Immunoglobulin
• Splenectomy is done under precautions

Platelet Function Defect (Thrombasthenia)

• PLT Transfusion is done, with antioxidant supplement (C or E vitamin)

Glanzmann Thrombasthenia

• Inherited bleeding disorder, patients need platelet aggregations
• Deficiency of platelet integrin impacts homeostasis

von Willebrand Disease (Pseudo Hemophilia - Vascular)

• Congenital disorder affects coagulation, and platelets, which in turn impacts the transportation to factor VIII

Defect

• Platelet dysfunction.
• Prolonged bleeding time, defect adhesion

Treatment

• Desmopressin and Platelet transfusion
• Factor VIII vWF concentrate

Clotting Factor Disorders

• Deficiency in one or more clotting factors

Hemophilia

• Deficiency of factor VIII is known as Hemophilia A (XR)
• Deficiency of factor IX is known as Hemophilia B (XR) or Christmas Disease
• Deficiency of factor XI is known as Hemophilia C (AR)
• Deficiency of factor V is known as Parahemophilia (AR)
• Pseudohemophilia is a Von Willebrand disease (AD) mostly causes prolonged APTTs

Hemophilia A

• Inherited disorder that mostly affects males.
• caused by deficiency of factor VII and the anticoagulant deficient

Clinical Picture of Hemophilia

• Bleeding happens upon CNS damage, in the umbilical cord, after circumcisions and in joints or the mucosa
• Is marked by Prolonged APTTs and Normal PTs

Hemophilia Testing

• Testing can be done antenatally, via amniocentesis

Hemophilia Treatment

• Fresh Blood and Plasma are used.
• Cryopecipitate also helps with the unstable amount of factor 8
• VIII concentrate and desmopressin may stop additional bleeding