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18 Cards in this Set

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Hydrops:
Define
Adult form?
Hydrops = overaccumulation of serous fluid in interstitial tissue or body cavities

In adults known as anasarca
Non-immune Hydrops Fetalis:
CV Causes (4 examples)
CV malformations: anything causing high pressure in RV/RA
ex: tricuspic atresia, pulm valve atresia, premature closure of DA or FO

Manifests as tachyarrhythmias, high output failure
Non-immune Hydrops Fetalis:
Chromosomal Causes
Turner (45XO)--may also have nuchal cystic hygroma (due to abnl lymph drainage; cause of webbed neck)
Trisomy 21, 18
What is a nuchal cystic hygroma?
Abnl lymph drainage in neck; can cause webbed neck appearance in Turner's Dz
Hydrops Fetalis:
Hemolytic Causes (Immune and Non-Immune)
Immune:
Rh incompatibility (Erythroblastosis fetalis)
ABO incompatibility

Non-immune:
Hg-opathies
Membrane defects
Enzyme Dz
What is eryhtroblastosis fetalis?
Rh- mother develops IgG Ab's to D antigen on fetal cells; cross placenta and hemolyze fetal RBCs (firstborn spared)

Fetus develops hemolytic anemia
What is kernicterus? How does it occur?

Effects?
Who is affected by this?
Kernicterus is a rare neurological condition that occurs in some newborns with severe jaundice

Inability of newborn to conjugate bilirubin-->increase in unconj'd bilirubin (injures brain; esp BS and BG)-->CEREBRAL PALSY

SEEN IN BABIES WITH ERYTHROBLASTOSIS FETALIS
ABO Incompatibility:
Under what circumstances would a fetus develop hyrdops?
Type O mothers with A or B fetus in which IgG anti-A or anti-B cross placenta.

Usually produce IgM which don't cross placents, but sometimes IgG produced even without prior sensitization (CAN AFFECT NEWBORN).

Type A or B moms have predominantly IgM Ab's which don't cross placenta.
What is Hemoglobin S and does it cause hydrops?
Hg S = sickle Hg; not a problem for hydrops bc of protective effect of Hg F (not switched to S until near birth)

Not a problem is mother is Hg S either.
alpha-thalassemia:
Gene deletions and traits.
Hb A not properly developed

1 alpha gene deleted: no anemia, nl RBCs (HbA)--silent carrier

2 genes: mild anemia, HbA (Hb B5-10%), Thal trait

4 genes deleted: DEATH in utero--severe anemia; HYDROPS
A hydropic fetus is born to a --/-a mother and a aa/-- father.

What are the traits of its parents and what deletions are present in the fetus?
Mom: --/-a (3 deletions: HgH)
Father: aa/-- 2 deletions (Thal trait)

Fetus ended up with 4 deletions
What populations are most vulnerable to hydropic alpha-thal?

Which are least vulnerable?
Southeast asian populations most vulnerable

Does not occur in fetuses of Mediterranean or African heritage (always have at least one alpha-gene on each allele)
What is MCV? When should a woman be screened for alpha-thal according to MCV?
MCV = mean cell (or corpuscular) volume; avg RBC volume

If SE ASIAN and MCV below 75, need to study mother and father of baby, to check for alpha-thal.
When does Hb H disease occur?
Three alpha-thal deletions
Infectious causes of hydrops fealits
CMV
Parvovirus (transmitted from mother), known as Fifth Dz in children
Fifth Disease
AKA Erythema infectiosum (parvovirus): rosy cheeks in children
Rapidly dividing cell lines located in _____ are infected by this virus.

What populations are affected?
Parvovirus infects rapidly dividing cell lines, esp ones in bone marror (esp erythroid progenitor cells).

Not important in the immunocompetent.
Affects sicklers and other hemolytic disorders.
In fetal hydrops, where are nuclear inclusions of parvovirus likely to be seen?
Liver, lung, heart; less so in placenta.