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What are the two categories of neonatal jaundice?
1. Benign, non pathologic hyperbilirubinemia
2. Pathologic hyperbilirubinemia
What are the causes of Benign, non- pathologic hyperbilirubinemia?
- Immaturity liver and gut
- inadequate breastmilk intake
- true "breast milk" jaundice
What is the result of immaturity of newborn liver and gut?
1) transient decrease in UGT
2) RBC quickly turn over
3) increase enterohepatic circulation
3) cannot conjugate bilirubin
4) increased Hgb metabolism
5) increased indirect bilirubin
What are the symptoms of physiologic or benign jaundice?
yellowing on days 2-5 of life that is usually mild
When does bilirubin levels fall in physiologic or benign jaundice?
within 1 week and normalize within 2 weeks
What is the management for physiologic or benign jaundice?
close monitoring +/- phototherapy or exchange transfusion if needed
What are the complications of persistently elevated bilirubin?
seizures, high-pitched crying, rigid muscles, developmental issues
How does inadequate breastmilk intake contribute to pediatric jaundice?
decreased gut motility in baby leads to elevated bilirubin that peaks in the first few days and should improve with time
What is the proposed hypothesis for breast milk jaundice?
lack of substances needed to digest breastmilk or break down bilirubin likely due to genetics
When does breast milk jaundice more often present?
presents later, within the first two
weeks of life, in infants who are predominantly breastfed
What is the management of breast milk jaundice?
phototherapy +/- pausing of breastfeeding
What is the onset of pathologic neonatal jaundice?
occurs in the first 24 hours of life
What are the potential causes of hemolytic disease leading to pathologic neonatal jaundice?
Rh incompatibility, Pyruvate Kinase deficiency, hereditary disease (spherocytosis)
What is Rh incompatibility?
If an Rh negative (-) mother is exposed to Rh positive (+) fetal blood, her immune system can make antibodies against baby's red blood cells
When can mothers be exposed to Rh (+)?
delivery, miscarriage, trauma, antepartum hemorrhage, amniocentesis, ectopic preganncy
What occurs when mother's antibodies attack and hemolyze baby's Rh (+) RBCs?
massive spillage of intracellular components, including hemoglobin which increases bilirubin
What are the clinical signs fo Rh incompatibility?
encephalopathy, lethargy, pallor, jaundice, hydrops fetalis
What are the symptoms of hydrops fetalis?
edema, pericardial effusion, pleural effusion, ascites
How is Rh incompatibility prevented?
RhoGAM shot to mom at 28 weeks and prior to any potential exposure to fetal Rh(+) blood
How is Rh incompatibility treated in neonates?
Phototherapy, IVIG +/- potential exchange transfusion
What is Crigler-Najjar Syndrome?
rare, genetic disorder causing unconjugated/ indirect hyperbilirubinemia due to either absent or severe decrease in UGT
What is Type I Crigler-Najjar Syndrome?
No UGT activity!
What is the presentation of Type I Crigler-Najjar Syndrome?
severe hyperbilirubinemia and kernicterus by week 2 of life if left untreated
What is the bilirubin level for Type I Crigler-Najjar Syndrome?
20-50mg/dL
What is the treatment for Type I Crigler-Najjar Syndrome?
daily phototherapy and liver transplant
What is Gilbert's syndrome?
hereditary disorder leading to increased levels of indirect/ unconjugated bilirubin due to decreased UGT
What can trigger episodes of Gilbert's Syndrome?
stress, fasting, alcohol, illness, dehydration, and overexertion
What is the treatment of Gilbert's syndrome?
Avoid triggers
What is Dubin-Johnson Syndrome?
Hereditary disorder of conjugated (direct) bilirubin metabolism causing a mutation of MRP2 gene
What is the clinical result of Dubin-Johnson Syndrome?
Buildup of direct bilirubin in hepatocytes turns liver dark because of DARK hepatocytes
What is the management of Dubin-Johnson Syndrome?
No treatment, benign
What is the typical work up for jaundice?
1) Check bilirubin levels with fractionate and nomograms
2) Peripheral smear
What are the endemic areas of malaria?
South and Central America, Africa, Middle East, Indian subcontinent, Asia
What causes the worst kind of malaria?
P. falciparum is responsible for the worst presentation and the most deaths
What is the vector for plasmodium?
female anopheles mosquito infect the red blood cells
What is the life cycle of malaria parasites?
1) infection of hosts with sporozoites
2) evolve into merozoites (infectious)
3) evolve into gametocytes
What are the symptoms of the paroxysmal episodes of malaria?
chills, high fever, sweats ("cyclic fever" or "episodic fevers alternative with chills")
What are the physical exam findings of malaria?
fatigued, pale, jaundiced, HSM
What are the lab findings of malaria?
anemia, elevated transaminases, elevated BUN/C
What diagnostics are used for malaria?
Giemsa stained blood smears or rapid Ab, Ag tests
What are the two major complications of P. falciparum?
Cerebral malaria and "Blackwater fever"
What are the symptoms of cerebral malaria?
altered mental status, delirium, seizure or coma
What are the symptoms of "Blackwater fever"?
kidney involvement, very dark urine, and renal failure from hemolysis
What is the clinical presentation of P. vivax and P. ovale?
Fever spikes every 48 hours
What is the clinical presentation of P. malariae?
Fever spikes every 72 hours
What is the clinical presentation of P. knowlesi?
Fever spikes every 24 hours
What is the treatment of choice for malaria?
Artemisinin combination therapy (ACT) given IV or IM
What are the common partner medications to extend the half life of artemsisn?
Mefloquine, Piperaquine, Sulfadoxine/Pyrimethamine
What are the malaria prevention strategies?
bed nets, clothes, long sleeves, repellent with permethrin, vaccine (endemic), chemoprophylaxis
What are the available prophylaxis regimens?
- Atovaquone-proguanil (daily, until 7 days after return)
- Doxycycline (daily, until 4 weeks after return)
- Mefloquine (once weekly in pregnancy, started 2 weeks prior and 4 weeks after return)