Pediatric Cases Flashcards
when does the physiologic nadir for hemoglobin in infants occur?
at approximately 2 months of age
how does anemia effect the homoglobin-oxygen association curve?
the oxygen dissociation curve shifts to the right
how does the rate of onset of anemia affect a patient?
the slower the anemia develops the better the body can compensate. Rapidly developing anemia usually results in more dramatic symptoms
how could you tell if the bone marrow was inadequately responding to a severe anemia?
a low or low-normal reticulocyte count is an inadequate bone marrow response in the face of a significant anemia
what is diamond-blackfan anemia?
congenital pure red blood cell aplasia that almost always presents in infancy
what is occurring in diamond-blackfan anemia?
there is increased apoptosis in erythroid precursors
how does diamond-blackfan anemia look on the peripheral blood smear?
macrocytic with low/inadequate reticulocyte count
what is the most common inherited form of aplastic anemia?
fanconi anemia
when do you get diagnosed with fanconi anemia?
may be up to 10 years old before it presents
how does iron deficiency anemia look on the peripheral smear of a child?
microcytic, hypochromic with increased RDW; target cells on peripheral smears
if a question stems has a pale child and he like to drink a lot of cow’s milk, what should you think?
IDA
how does cyclic neutropenia present?
cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections
what is the inheritance pattern for cyclic neutropenia?
autosomal dominant
what are the symptoms associated with Schwachman-Diamond syndrome?
Triad of: neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities
what is the inheritance pattern for Schwachman-Diamond syndrome?
autosomal recessive
what is the inheritance pattern of fanconi anemia?
autosomal recessive
what is a classic example of congenital pancytopenia/ bone marrow failure/ aplastic anemia?
fanconi anemia
what are patients with fanconi anemia at an increased risk for?
AML, brain tumors, and Wilms tumor
how does leukocyte adhesion deficiency present?
- delayed separation of umbilical cord stump, 2. recurrent and severe bacterial and fungal infection without pus, and 3. poor wound healing
what is the inheritance pattern of leukocyte adhesion deficiency?
autosomal recessive
what are the symptoms associated with hyper-immunoglobulin E syndrome (Job syndrome)?
Triad of symptoms: severe eczema, recurrent bacterial infections (usually staph) of skin, recurrent pulmonary infections (bacterial or fungal)
the number 1 cause of death due to illness in the US is what?
brain cancer followed by leukemias
the overall number one cause on death in children is what?
unintentional injuries (accidents)
what is the most common malignancy between ages 15-19?
hodgkin lymphoma (HL)
what is the pathognomonic feature of HL?
reed-sternberg cells
what is the inheritance pattern of wiscott-aldrich syndrome?
x-linked recessive
what are the symptoms of wiscott-aldrich syndrome?
classic triad of: recurrent sino-pulmonary and ear infections, severe atopic dermatitis, and bleeding secondary to significant thrombocytopenia
name the condition responsible for the greatest percentage of childhood malignancies?
leukemias (#1 cause of death is brain malignancies, but #1 cause of malignancies is leukemias)
when does ITP usually present in kids?
50% follows a viral infection by 1-3 weeks
what do the labs show in a kid with ITP?
platelets will be less than 20k with all other cell lines being normal; PT/PTT is normal
how do you treat ITP in kids?
it will usually self resolve with supportive care, but prednisone is used when severe
how does kasabach-merritt phenomenon present?
thrombocytopenia and hypo-fibrinogenemia associated with giant hemangioma
