Paediatrics Marrow Pg 231-240 (Hematology)
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Questions and Answers

What is the recommended dosage of intravenous immunoglobulin (IVIG) for treating rheumatic disorders in children?

  • 2 g/kg (correct)
  • 1 g/kg
  • 3 g/kg
  • 4 g/kg

    • The anti-inflammatory dose of aspirin for children with rheumatic disorders is 150 mg/kg.

    False

    What should be done if an echocardiography result is normal?

    Stop aspirin.

    The risk of coronary abnormalities is reduced to ___% with appropriate treatment.

    <p>2-3</p> Signup and view all the answers

    Match the treatment with its associated detail:

    <p>Intravenous immunoglobulin (IVIG) = Reduces risk of coronary abnormalities to 2-3% Aspirin (anti-inflammatory dose) = 75 mg/kg until afebrile for 48 hours Aspirin (anti-platelet dose) = 3-5 mg/kg for 6-8 weeks Echocardiography = Determine continuation of aspirin treatment</p> Signup and view all the answers

    What finding is associated with a complete blood count in anemia cases?

    <p>Associated pancytopenia</p> Signup and view all the answers

    Folic acid should be administered alone in cases of megaloblastic anemia.

    <p>False</p> Signup and view all the answers

    What is the MCV value associated with Vitamin B12 deficiency anemia?

    <p>100-120 fL</p> Signup and view all the answers

    For pernicious anemia, Vitamin B12 is administered in __________ doses lifelong.

    <p>monthly</p> Signup and view all the answers

    Match the following types of anemia with their recommended treatments:

    <p>Vitamin B12 deficiency = Vitamin B12 1000 mcg I.m daily for 2 weeks Folic acid deficiency = Oral folic acid 1-5 mg daily for 3-4 weeks Pernicious anemia = Monthly doses of Vitamin B12 lifelong Megaloblastic anemia = Both Vitamin B12 and folic acid</p> Signup and view all the answers

    What is the hemoglobin cut-off level for anemia in children aged 6 months to 5 years?

    <p>11 g/dL</p> Signup and view all the answers

    The WHO definition includes a hemoglobin cut-off of 10 g/dL for children aged 6 months to 5 years.

    <p>False</p> Signup and view all the answers

    What is the age range considered for anemia evaluation according to WHO?

    <p>6 months to 5 years</p> Signup and view all the answers

    The WHO definition states that anemia in children aged _____ years has a hemoglobin cut-off of 11 g/dL.

    <p>5</p> Signup and view all the answers

    Match the following age groups with their hemoglobin cut-off for anemia:

    <p>6 months - 5 years = 11 g/dL 5 - 12 years = 12 g/dL 12 - 18 years = 13 g/dL Adults = 13.5 g/dL</p> Signup and view all the answers

    Which of the following is NOT a classification of anemia mentioned?

    <p>Hypochromic anemia</p> Signup and view all the answers

    Aplastic anemia is a form of hemolytic anemia.

    <p>False</p> Signup and view all the answers

    What is one of the rare causes of iron deficiency anemia mentioned?

    <p>Atransferrinemia</p> Signup and view all the answers

    In iron deficiency anemia, there is a decrease in total body iron stores which results in _____ erythropoiesis.

    <p>decreased</p> Signup and view all the answers

    Match the following conditions with their descriptions:

    <p>Aplastic anemia = Bone marrow failure leading to reduced blood cell production Megaloblastic anemia = Impaired DNA synthesis usually due to B12 or folate deficiency Pure red cell aplasia = Severe reduction of red blood cell precursors in the marrow Hemolytic anemia = Increased destruction of red blood cells</p> Signup and view all the answers

    Which condition is most commonly associated with low mean corpuscular volume (MCV)?

    <p>Iron Deficiency Anemia</p> Signup and view all the answers

    Generalized lymphadenopathy is a typical feature of hemolytic anemia.

    <p>False</p> Signup and view all the answers

    What does a corrected reticulocyte count greater than 2% indicate?

    <p>Adequate bone marrow response</p> Signup and view all the answers

    In the presence of low serum ferritin, the diagnosis is likely to be _____ anemia.

    <p>iron deficiency</p> Signup and view all the answers

    Match the types of anemia with their characteristics:

    <p>Iron Deficiency Anemia = Microcytic anemia, most common Thalassemia = Normal serum ferritin despite low MCV Anemia of Chronic Disease = High serum ferritin Macrocytic Anemia = High mean corpuscular volume</p> Signup and view all the answers

    What is the most reliable clinical sign of anemia in children?

    <p>Pallor</p> Signup and view all the answers

    Low serum ferritin levels are indicative of nutritional anemia in children.

    <p>True</p> Signup and view all the answers

    What is the dose of Ferrous Sulphate recommended for treating anemia in children?

    <p>3-6 mg/kg/day (maximum: 200 mg/day)</p> Signup and view all the answers

    The condition characterized by shiny, smooth tongues in anemia is called _____.

    <p>atrophic glossitis</p> Signup and view all the answers

    Match the following time periods with their corresponding responses to iron therapy.

    <p>12-24 hours = Replenishment of intracellular iron enzymes 36-48 hours = Bone marrow response: Erythroid hyperplasia 48-72 hours = Reticulocytosis: Peaks by 5-7 days 1-3 months = Replenishment of iron stores</p> Signup and view all the answers

    What is the inheritance pattern of hereditary spherocytosis?

    <p>Autosomal dominant</p> Signup and view all the answers

    Sickle cell anemia is considered a hemoglobinopathy.

    <p>True</p> Signup and view all the answers

    Name one clinical feature commonly associated with hereditary spherocytosis.

    <p>Splenomegaly</p> Signup and view all the answers

    In hereditary spherocytosis, red blood cells are described as being _____ due to membrane defects.

    <p>spherical</p> Signup and view all the answers

    Match the following conditions with their associated features:

    <p>Hereditary spherocytosis = Spherical red blood cells with increased hemolysis G6PD deficiency = Defect in red cell metabolism Sickle cell anemia = Abnormal hemoglobin leading to sickle-shaped cells Thalassemia = Disorder of hemoglobin synthesis</p> Signup and view all the answers

    What is one of the diagnostic criteria for Kawasaki disease?

    <p>Fever ≥ 5 days</p> Signup and view all the answers

    Increased platelet count indicates an acute phase of Kawasaki disease.

    <p>False</p> Signup and view all the answers

    What are the typical inflammatory markers that increase during Kawasaki disease?

    <p>CRP and ESR</p> Signup and view all the answers

    Echocardiography should be evaluated after _____ weeks of illness.

    <p>6-8</p> Signup and view all the answers

    Match the following features with their phases in Kawasaki disease:

    <p>Acute Phase = Fever for ≥ 5 days Subacute Phase = Increased platelet count Convalescent Phase = Resolution of symptoms Cardiac Involvement = Detected through echocardiography</p> Signup and view all the answers

    What is the primary age group affected by Kawasaki Disease?

    <p>Under 5 years old</p> Signup and view all the answers

    Kawasaki Disease is known for having a good prognosis.

    <p>True</p> Signup and view all the answers

    What is the mnemonic used to remember the acute manifestations of Kawasaki Disease?

    <p>CREAM</p> Signup and view all the answers

    A significant clinical manifestation during the subacute stage of Kawasaki Disease is the presence of a __________ tongue.

    <p>strawberry</p> Signup and view all the answers

    Match the stages of Kawasaki Disease with their corresponding features:

    <p>Acute = Non-pitting edema &amp; rash Subacute = Coronary aneurysm and thrombocytosis Convalescent = Beau's lines on nails</p> Signup and view all the answers

    Which of the following is a cause of megaloblastic anemia?

    <p>Pernicious anemia</p> Signup and view all the answers

    Neurological manifestations in vitamin B12 deficiency can occur after the onset of anemia.

    <p>False</p> Signup and view all the answers

    What is a characteristic clinical feature that may be observed in a patient with megaloblastic anemia?

    <p>Beefy red tongue</p> Signup and view all the answers

    The presence of __________ neutrophils, defined as at least one neutrophil with 6 or more lobes, is indicative of megaloblastic anemia.

    <p>hypersegmented</p> Signup and view all the answers

    Match the following conditions with their associated features:

    <p>Loss of position/vibration sense = Earliest neurological symptom Ataxia = Unsteadiness of gait Paresthesia = Abnormal sensation Peripheral neuropathy = Nerve damage</p> Signup and view all the answers

    Study Notes

    Rheumatic Disorders of Childhood

    • Intravenous immunoglobulin (IVIG) is the preferred treatment for rheumatic disorders in children.
    • The recommended dosage is 2 g/kg.
    • IVIG reduces the risk of coronary abnormalities to 2-3%.
    • Aspirin is used as an anti-inflammatory medication at a dose of 75 mg/kg until the child is afebrile for 48 hours.
    • An anti-platelet dose of 3-5 mg/kg is administered for 6-8 weeks.
    • An echocardiogram is performed to assess the heart's condition.
    • If the echocardiogram is normal, aspirin is stopped.
    • If the echocardiogram is abnormal, aspirin is continued long-term.

    Anemia Work-up

    • Normal:
      • Peripheral smear findings may reveal hypochromia and microcytosis.
    • Pancytopenia:
      • Bone marrow failure: Aplastic anemia.
      • Megaloblastic anemia.
      • Pure red cell aplasia (Diamond-Blackfan syndrome).
    • Hemolytic anemia:
      • Aplastic crisis (Parvovirus B19).
      • Autoimmune hemolytic anemia.
    • Acute blood loss:

    Iron Deficiency Anemia (IDA)

    • Iron stores are decreased in IDA.
    • IDA leads to decreased erythropoiesis and anemia.
    • Iron stores decline after a few months of inadequate iron intake.
    • Predisposing factors include decreased dietary iron intake, increased demand (growth spurts), malabsorption (e.g., celiac disease), blood loss, and worm infestation.
    • Onset of IDA is typically between 9-24 months.
    • Late onset IDA can occur due to good iron stores from the mother.
    • Atransferrinemia and transferrin receptor antibodies are rare causes.

    Focused Physical Examination

    • Universal feature: Pallor.
    • Associated congenital defects:
      • Skeletal defects (absent radius/thumb): Observed in Fanconi anemia (bone marrow failure).
    • Petechiae/purpura (thrombocytopenia): Seen in leukemia, aplastic anemia, and hemolytic uremic syndrome (acute onset).
    • Splenomegaly: Seen in hemolytic anemia and leukemia.
    • Generalized lymphadenopathy: Associated with leukemia.

    Investigations

    • Low MCV: Suggestive of microcytic anemia.
      • Iron Deficiency Anemia (IDA): Most common cause.
      • Anemia of chronic disease/inflammation (ACD/ ACI).
      • Thalassemia.
    • Normal MCV: Indicative of normocytic anemia.
    • High MCV: Associated with macrocytic anemia.
    • Low MCV Anemia:
      • History and findings suggestive of IDA warrant a therapeutic iron trial.
      • Assess the response to iron therapy.
      • If positive response, treat as IDA.
      • Serum ferritin:
        • Low: IDA.
        • Normal: Thalassemias.
        • High: ACD/ACI.
    • Normal/High MCV Anemia:
      • Further evaluation based on reticulocyte count (normal: 1-2%).
      • Corrected reticulocyte count provides information on bone marrow response.

      • 2% indicates adequate bone marrow response.

    Clinical Features of Iron Deficiency Anemia

    • Clinical features:
      • Tiredness.
      • Easy fatigability.
      • Pallor (oral mucosa, nail beds, palpebral conjunctiva).
      • Koilonychia (spooning of nails).
      • Platynychia (flat nails).
      • Atrophic glossitis (shiny, smooth tongue).
      • Associations: pica, temper tantrums, breath-holding spells, restless leg syndrome.

    Investigations for Iron Deficiency Anemia

    • Peripheral smear:
      • Blood parameters: Low MCV and serum ferritin.

    Treatment of Iron Deficiency Anemia

    • Ferrous Sulphate:
      • Most effective and economical form of iron.
      • 20% elemental iron is present.
      • Dose: 3-6 mg/kg/day (maximum 200 mg/day).
      • Duration: 4-6 months (to replenish iron stores).

    Response to Iron Therapy

    Time after Iron Administration Response
    12-24 hours Replenishment of intracellular iron enzymes. Clinically seen as subjective improvement.
    36-48 hours Bone marrow response: Erythroid hyperplasia
    48-72 hours Reticulocytosis: Peaks by 5-7 days
    4-30 days ↑ Hb levels
    1-3 months Replenishment of iron stores

    Supplementary Therapy

    • Dietary modification: Consumption of iron-rich foods.
    • Deworming: Albendazole.

    Peripheral Smear Findings in IDA

    • Hypochromia (pallor 1/3 of cell).
    • Microcytosis.
    • Anisopoikilocytosis (varying size and shape).

    Kawasaki Disease

    • The most common childhood vasculitis in India.
    • Typically affects children under 5 years old.
    • Etiology is idiopathic.

    Kawasaki Disease Stages and Clinical Manifestations:

    Stage Features
    Acute (1-2 weeks) * > 5 days of fever * Conjunctivitis: 8/1 non-purulent, spares limbus.* Rash: Non-specific maculopapular rash.* Extremities: Non-pitting edema & erythema of limbs.* Lymphadenopathy : w/I cervical.* Mucocutaneous involvement.* Oral cavity erythema * Cracking of lips
    Subacute (3-4 weeks) * Lips: Cheilitis * Strawberry tongue * Coronary aneurysm: ~25% of cases.* Predisposes to myocardial ischemia/thrombosis/rupture.* Periungual desquamation (skin peeling around nails) * Thrombocytosis
    Convalescent (6-8 weeks) * Stage of recovery * Beau's lines on nails

    Diagnostic Criteria (Clinical)

    • Fever ≥ 5 days
    • 4 out of 5 CREAM findings (Conjunctivitis, Rash, Erythema of extremities, Mucocutaneous involvement, Lymphadenopathy)

    Investigations for Kawasaki Disease

    1. Blood count: Platelet count
      • Normal: Acute phase
      • Increased: Subacute phase
    2. Inflammatory markers:
      • ↑ CRP
      • ↑ ESR
    3. Echocardiography
      • Normal
      • Frequent evaluation during the second week of illness
      • Evaluate after 1 year

    Atypical/Incomplete Kawasaki Disease

    • Fever ≥ 5 days + 2/3 CREAM features
    • OR Infant with fever ≥ 7 days & no other manifestations
    • ESR ≥ 40 mm/hr
    • CRP ≥ 3 mg/dL
    • Echocardiography → Cardiac involvement seen*
    • Treat as Kawasaki Disease*

    Prognosis of Kawasaki Disease

    • Good prognosis.
    • Long-term follow-up required if renal involvement.

    Treatment of Kawasaki Disease

    • Supportive treatment (self-limiting).
    • Steroids: Severe GI involvement.
    • 10-20% have recurrence.

    Congenital Hemolytic Anemia - Hereditary Spherocytosis

    • Inheritance: Autosomal dominant.
    • Pathogenesis:
      • Defect in RBC cytoskeletal protein.
      • Spherical RBCs.
      • Reduced deformability.
      • Entrapment in splenic microcirculation.
      • Hemolysis (destruction of RBCs).
      • Splenomegaly (enlarged spleen).

    Clinical Features of Hereditary Spherocytosis

    • Clinical features:
      • Variable onset of anemia and jaundice (increased RBC lysis).
      • 50% cases show neonatal jaundice.
      • Splenomegaly.
      • Family history often present.
      • Pigment gallstones (increased bilirubin production).
      • Aplastic crisis (acute presentation).
      • Parvovirus B19 infection may occur in patients with hereditary spherocytosis.
      • Bone marrow dysfunction.
      • Pancytopenia.

    Other Forms of Congenital Hemolytic Anemia

    • Defects of red cell membrane: Hereditary spherocytosis.
    • Disorders of red cell metabolism: Glucose-6-phosphate dehydrogenase (G6PD) deficiency.
    • Hemoglobinopathies: Sickle cell anemia and thalassemia.

    Megaloblastic Anemia

    • Causes:*
    • Pure vegetarian/vegan diet.
    • Pernicious anemia (auto-antibodies against intrinsic factor).
    • Vitamin B12 malabsorption (terminal ileum).
    • Inborn errors of metabolism:
      • Homocystinuria.
      • Orotic aciduria.
      • Methylmalonic acidemia.
    • Predisposing Factors:*
    • Vitamin B12: Deficiency
    • Folic acid (FA):
      • Diet: Increased goat milk consumption.
      • Infection: Giardiasis.
      • Drugs: Interference with FA utilization (e.g., 6-mercaptopurine, methotrexate).

    Clinical Features of Megaloblastic Anemia

    • Clinical features:
      • Pallor, tiredness, and easy fatigability.
      • Characteristic features:
        • Beefy red tongue.
        • Hepatosplenomegaly (in 30-40% of cases).
        • Knuckle hyperpigmentation.

    Neurological Manifestations of Megaloblastic Anemia

    • Seen in vitamin B12 deficiency due to decreased methylation reactions in the nervous system.
    • Can occur before the onset of anemia.
    • Features:
      • Loss of position/vibration sense (earliest symptom).
      • Ataxia (unsteadiness of gait).
      • Paresthesia.
      • Peripheral neuropathy.
      • Subacute combined degeneration (SACD) of the spinal cord.

    Investigations for Megaloblastic Anemia

    • Peripheral smear:
      • Macro-ovalocytes (large, oval RBCs).
      • Hypersegmented neutrophils: At least one neutrophil with ≥ 6 lobes on ≥5% of neutrophils.
      • Delayed nuclear maturation.

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    Description

    This quiz covers the essential aspects of rheumatic disorders in childhood, specifically focusing on treatment options such as intravenous immunoglobulin and aspirin usage. It also delves into the work-up for anemia, including peripheral smear findings and various types of anemia. Test your knowledge on these crucial topics in pediatric healthcare!

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