Hemophilia and Von Willebrand Disease — Flashcards

Flashcard 1: Hb _____ levels are increased in juvenile chronic myeloid leukemia (JMML)

Answer: F

Flashcard 2: Which objective audiometry test is considered unsuitable for testing hearing in infants and young children because it is affected by sleep and sedation?

Answer: CERA (Cortical Evoked Response Audiometry)

Extra: CERA is a late latency response (LLR) that is highly dependent on the patient's state of alertness and is significantly affected or abolished by sleep and sedation. Since infants and uncooperative children often need to be asleep or sedated for objective testing, CERA is considered unsuitable for them. In contrast, BERA (Brainstem Evoked Response Audiometry) is a short-latency response that is not affected by sleep or sedation, making it the gold standard for objective hearing assessment in children.

Flashcard 3: Patients with beta-thalassemia major are at risk of _____ with parvovirus B19 infection of erythroid precursors

Answer: aplastic crisis

Flashcard 4: Anaemia in a 2-5 year old child is defined as a hemoglobin level of

Answer: < 11 g/dL

Flashcard 5: Is Ph chromosome seen in JMML?_____

Answer: No

Flashcard 6: On complete blood count, patients with Systemic Juvenile Idiopathic Arthritis will frequently present with: - _____ RBCs - increased WBCs - increased Platelets

Answer: decreased

Flashcard 7: Beta-thalassemia _____ is characterized by expansion of hematopoiesis into facial bones, leading to "chipmunk facies"

Answer: major

Flashcard 8: _____ syndrome is a syndrome of thrombocytopenia and consumptive coagulopathy in a patient of hemangioma

Answer: Kasabach-Merritt

Flashcard 9: _____ is a common presenting sign of sickle cell anemia in infants (age group)

Answer: Dactylitis

Flashcard 10: Kids with sickle cell disease should undergo _____ starting at age of 2 yrs to reduce the incidence of overt stroke

Answer: transcranial doppler ultrasound