Flashcards in Pediatric heme/onc Deck (5)
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1
Hemolytic disease of the newborn
Anti-Rh abs against fetal RBCs causing hemolysis
must have been sensitized during a previous pregnancy
events that warrant rhogam administration:
Maternal-fetal hemorrhage
Abortion
Amniocentesis
3rd trimester bleeding
Trauma/motor vehicle accident
Delivery of baby
Domestic abuse
Give immunoglobulin within 72 hours of the mixing event
2
Fanconi anemia
Panconi anemia
Autosomal recessive disorder
Bone marrow failure
Pancytopenia:
-fatigue
-dyspnea on exertion
-infections
unique features:
short stature
abnormal skin pigmentation
-hyperpigmentation
-hypopigmented areas
-cafe-au-lait spots
-horseshoe kidney
-thumb abnormalities (absent or hypoplastic)
Labs:
Pancytopenia
serum alpha fetoprotein
hypocellularity
Treatment:
antibiotics (leukopenia)
transfusions (anemia)
bone marrow or hematopoietic stem cell transplant
Complications:
death from bone marrow failure
3
Diamond- Blackfan anemia
Pure RBC anemia: fatigue, dyspnea, cyanosis, pallor
increased erythropoetin (body wants to make more cells but can't)
Craniofacial abnormalities
Thumb abnormalities
Heart murmurs
Intellectual disability
Hypogonadism
Problems with
pure RBC anemia
face
brain
thumbs
heart
gonads
4
Neuroblastoma
adrenal neuroblastoma
most common tumor of adrenal medulla in children, of neural crest cell origin
May arise in the adrenal glands OR sympathetic ganglion
H and P:
abdominal distension and pain
weight loss
malaise
bone pain
diarrhea
abdominal mass
HTN
(unlike Wilm's tumor, no hematuria)
Horner syndrome if it occurs in the sympathetic ganglia of the chest
proptosis
movement disorders
hepatomegaly
fever
periorbital bruising
Labs: NE breakdown products:
increased VMA
increasd homovanilic acid (HVA) in the 24-hr urine collection
no episodic HTN, diaphoresis (this is not pheo)
Diagnostic testing:
CT scan shows tumor
Good prognosis if you can surgically resect this
5
