hematologic system, pediatrics ch31

Question 1

Fe Deficiency Anemia

Answer 1

Iron essential for normal brain development, deficiency results in irreversible damage
• Inadequate intake most common cause in first few years, then;
• blood loss (micro GIB r/t cow’s milk allergy)
Clinical manifestations
• Irritability, activity intolerance,
• Pallor, tachycardia, murmur when severe
• Splenomegaly, widened skull sutures, decreased growth, developmental delay, pica
• Deficiency in attention span, alertness, learning ability

Question 2

(G6PD) glucose-6-phosphate dehydrogenase Deficiency

Answer 2

Inherited genetic disorder (x -linked recessive) involved in the processing of carbohydrates
• Most common disorder of RBC’s
• Cannot protect cells from ROS, resulting in damaged RBC’s that break down prematurely/ hemolysis
• Can be asymptomatic unless triggered by stressor such as :
• Hepatitis, pneumonia, typhoid fever

Clinical manifestations
• jaundice
• Pallor, dark urine, back pain
• When severe: shock, cardiovascular collapse and death
• Fava bean ingestion with hemolytic reaction

Question 3

Hemolytic Disease of the Fetus and Newborn

Answer 3

When antigens on fetal erythrocytes differ from antigens on maternal erythrocytes
• RH positive (antigen -D) or RH negative
• Can be from blood type incompatibility or RH incompatibility, does not occur in each instance

• Mother makes antibodies against fetus and subsequent incompatible pregnancy(s)
Clinical manifestations
• Can result in fetal loss or still birth,
• Hyperbilirubinemia
• Splenomegaly
• Hepatomegaly
• Kernicterus

Question 4

Hemophilia

Answer 4

• Hemorrhagic bleeding disorder that is inherited, usually due to impairment of either factor VIII, IX, or XI
• Affects males, females are carriers
• Inefficiency in forming blood clots in response to injury
Clinical manifestations
• Prolonged bleeding times, there is not always excessive bleeding with circumcision due to extrinsic clotting cascade
• Hematomas from immunizations, firm grasps
• Usually diagnosed once mobile
• Hemorrhage into joints, elbows, knees, and ankles
• Degenerative joint changes

Question 5

immune (idiopathic)Thrombocytopenia Purpura

Answer 5

Most common cause of thrombocytopenia due to increased platelet destruction
• Acute form frequently observed in children
• Most often lasts 1- 2 months with full remission
• Can occasionally advance to chronic condition
Acute form usually attributed to infection, allergy, or lupus
• the acute ITP resolves when underlying cause resolves
chronic ITP more often in adults
• result of autoantibodies against platelet specific antigens
• most common in women 20-40yrs
• chronic form gets progressively worse
clinical manifestation:
• petechia, purpura
• mucosal bleeding, gums, epistaxis, menorrhagia, hematuria
• associated with fetal thrombocytopenia with affected mothers

Question 6

Sickle Cell Disease

Answer 6

Genetic (autosomal recessive)
• Atypical hemoglobin S changes erythrocytes to sickle shape
• Anemia, microvascular obstruction, ischemic tissue damage,
• Thalassemia
• Triggers include low temperatures, hypoxia, increased plasma osmolality, decreased plasma volume,
Clinical manifestations:
• Usually first seen at 6-12 mos. as fetal hgb is replaced.
• Chronic with acute exacerbations
• Pallor, fatigue, jaundice, irritability
• Severe pain
• Acute chest syndrome account for 25% of deaths in people with SCD
• Bed wetting in children with kidney involvement
• gallstones