Paediatric Hematology - Introduction, Bleeding, and Clotting Disorders MCQs

Dehydration (hypovolemic) shock is the most common type of shock in paediatric population. Common Causes in Paediatrics Gastrointestinal Losses: Diarrhea (e.g., rotavirus, cholera). Vomiting. Hemorrhage: Trauma (e.g., accidents). Post-surgical bleeding. Burns: Fluid loss through damaged skin. Other Causes: Inadequate fluid intake (e.g., neglect, illness). Third-spacing (e.g., sepsis, nephrotic syndrome). Septic shock is most common in neonates. Tags: Topic: SHOCK Difficulty level: MUST KNOW Reference: Manual of paediatric emergencies & critical care, 2 nd ed., pg. 158

Immune (or idiopathic) thrombocytopenic purpura (ITP) is common in children. In most cases, a preceding viral infection can be documented. The platelet count frequently is less than 20,000/mm3, but other laboratory test results are normal, including the bone marrow aspiration (which may show an increase in megakaryocytes). These patients have no hepatosplenomegaly or generalized lymphadenopathy. Treatment consists of observation or possibly intravenous immunoglobulin (IVIG), intravenous anti-D (in Rh-positive patients), immunosuppressives, or steroids. The history must be reviewed for other possible causes of thrombocytopenia, including recent MMR vaccination, drug ingestion, and human immunodeficiency virus. Reference: Nelson textbook of Pediatrics, 1 st SA edition, pg. 2402

Type 2B vWD results from gain-of-function mutations that increase the ability of vWF to bind platelets. This leads to increased clearance of both vWF and platelets from circulation and results in the loss of high-molecular-weight multimers and decreased vWF activity, similar to that seen in type 2A vWD. Desmopressin is relatively contraindicated in type 2B vWD as it may accelerate VWF-platelet binding and clearance. Please note that Desmopressin is highly effective in Type 1 vWD Reference: Nelson textbook of pediatrics, 1 st SE edition, pg. 2390

In emergencies when factor IX is unavailable, only FFP is used as cryoprecipitate does not contain factor IX. Cryoprecipitate contains Fibrinogen, VWF, factor VIII, XIII & fibronectin, but no Factor IX. Hence it will not be useful in Factor IX deficiency, i.e., Hemophilia B Reference: OP Ghai 9 th Ed/Page 351

Prothrombin G20210A mutation occurs in approximately 3% of the general population and increases the risk for thrombosis mainly by causing excess prothrombin production. Regarding other options Factor V deficiency is associated with increased bleeding, not with increased thrombosis. (Factor V Leiden is the most common inherited thrombophilia and the mutation causes a resistance of factor V to inactivation by activated proteins C and S.) Patients with nephrotic syndrome, not nephritic syndrome, are at an increased risk of thrombosis due to loss of clotting factors in the urine. Hyperhomocysteinemia is a risk factor for thrombosis and stroke in children, but not Homocysteine Deficiency. Reference: Nelson textbook of Pediatrics, 1 st SA edition, pg. 2393

Rule of 2's in Meckel's Diverticulum Seen in 2% population Two times common in F>M - 2:1 Presents before 2 years of age 2 inches length Present 2 feet proximal to ileocecal value, along the antimesenteric border Ectopic tissue common in meckel diverticulum : Gastric tissue(>50% cases)>Pancreatic tissue>jejunum Meckel's diverticulum with ectopic gastric mucosa secretes hydrochloric acid (HCl), which can lead to ulceration in the adjacent intestinal mucosa and result in gastrointestinal bleeding. Reference: Nelson textbook of paediatrics

A Neonate delivered at home would not have likely got Vitamin K injection at birth. Now she is presenting with bleeding manifestations. Classical vitamin K deficiency bleeding in newborns presents in the first week of life. Typical symptoms include easy bruising, mucus membrane bleeding, and hematemesis. Risk factors include babies who have not been given vitamin K treatment, are breastfed, or are poor feeders. Such patients will have low levels of vitamin K-dependent factors: II, VII, IX, and X. Prothrombin time will be prolonged Factor V and VIII levels are unaffected. Reference: Nelson textbook of Pediatrics, 1 st SA edition, pg. 2397

Rigid bronchoscopy is both diagnostic and therapeutic procedure in children who aspirate foreign body into the lower airway in children. Flexible fiberoptic bronchoscopy is sensitive to visualize but cannot be used to remove stuck FB. As per Nelson 21 st Ed, "The treatment of choice for airway foreign bodies is prompt endoscopic removal with rigid instruments by a specialist (otolaryngologist or pulmonologist)." Potential complications of rigid bronchoscopy include bronchospasm, desaturation, bleeding, and airway edema, in addition to the inherent risks of anesthesia. Reference: Nelson 21 st Edition, Chapter 414.