Common Disorders of the Newborn

jaundice
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Terms in this set (97)
jaundice
_____ is a yellowing of the skin and scleral due to accumulation of UNconjugated bilirubin
indirect/(unconjugated)
_____/(_____) bilirubin is normally at least 2 and averages 5-7 mg/dL in the first week
13-14
indirect bilirubin usually peak at _____-_____ on days 3-5 (total bilirubin usually peaks at 14-15
65%
_____ of newborns have visible jaundice with TSB > 6mg/dL in 1st week of life
face
clinical pearl: jaundice starts on the _____ as total bilirubin levels reach 5mg/dL and progresses down the body with increasing levels
heme
_____ from RBCs is converted to unconjugated (lipid soluble) bilirubin in the RES and bone marrow
liver
bilirubin is transported to the _____ bound to albumin
hepatocytes
in the liver, bilirubin is conjugated in _____ and becomes water soluble
biliary tree
once bilirubin is conjugated, it now gets secreted in the _____ _____ for excretion through GI tract (also some renal excretion)
hydrolyzed
in the GI tract, some bilirubin is _____ to become lipid soluble again and reabsorbed (enterohepatic recirculation)

-this is pronounced by absence of gut flora and slow intestinal motility in the first few days of life
reasons newborns are more susceptible to increased bilirubin-*shortened RBC life* (70-90 days compared to 90-120 days in adults) -*increased degradation of RBC precursors* in marrow, spleen, ad liver -*immature system to change unconjugated bilirubin to conjugated bilirubin in the liver* (*low* levels of *UDPGT*) -*absence of intestinal flora and slow intestinal motility increases enterohepatic recirculation* and results in higher leaves of bilirubinRh or ABOabnormal presentations: jaundice in first 24hrs; could be due to _____ or _____ *incompatibility* (will probably have increase reticular count and positive Coombs test*investigated*bilirubin increasing more than 0.5mg/dL per hour* *bilirubin increasing more than 5.0mg/dL per 24hrs* these levels must be _____jaundice that develops day 2-3 and lasts 7-10 days is usually *physiologic jaundice*-clinical jaundice develops after 24hrs -<5 mg/dL day rise in total bilirubin -peak total bilirubin at day 3-5 (usually around day 3 in a term newborn and day 5 in preterm newborn) with total bilirubin <15 mg/dL -clinical (visible) jaundice resolved in 1 week in term pt; 2 weeks in premature ***anything outside these parameter must be investigated (blood type, Coombs, CBC with smear, total bilirubin)sepsisjaundice *after day 2-3* but in the first week may be _____breast milk jaundicejaundice *after first week* may be "_____ _____ _____" -this is considered a normal extension of physiologic jaundice 0lasts up to 3 weeks in most and up to 3 months in some -though to be due to *increased enteric reabsorption of unconjugated bilirubin*unconjugated bilirubin is *neurotoxic*why does jaundice matter?acute bilirubin toxicity_____ _____ _____-signs and symptoms of evolving brain damage earliest symptoms can be noted after 4 days: -high pitched cry -lethargy -poor suck/failure to eat -hypotonia (decreased muscle tone) -irritability later symptoms: -bulging fontanel -arching of neck (retrocollis) and trunk (opisthotonos) -hypertonia -seizures -apnea -there is a *high mortality rate* -those who do survive have long term sequelaeretrocollisarching of the neck due to acute bilirubin toxicityopisthotonosarching of the trunk due to acute bilirubin toxicitykernicterus_____ (*chronic bilirubin toxicity*) is the term that described the *irreversible chronic brain injury from bilirubin)120how many incidences of *kernnicterus* cases in US since 1900?discharge before 48hrs breastfeeding delayed measurements of TSB unrecognized hemolysis lack of post discharge follow uo(5) risk factors for *kernicterus*?extrapyramidal movement*kernicterus* is characterized by: _____ _____ which are disordered (choreoathetoid cerebral palsy)gaze abnormalities*kernicterus* is characterized by: _____ _____, especially *limiation* of *upward gaze*auditory disturbances*kernicterus* is characterized by: _____ _____ ei *deafness*intellectual disability*kernicterus* is characterized by: _____ _____ ei *mental retardation*dysplasia*kernicterus* is characterized by: _____ of the *enamel* of deciduous ("baby") teeth< 2*direct bilirubin* > _____mg/dL is *never physiologic*neurotoxic*direct bilirubin* is NOT _____cholestasis or hepatocellulardirect bilirubin indicated _____ or _____ injury20jaundice with a bilirubin *below* _____g/dL rarely causes kernicterusphototherapy and exchange transfusionwhat are the 2 treatments for jaundice?phototherapy-should be initiated when TSB is *5-6mg/dL below what is recommended for exchange transfusion* -*_____ change unconjugated bilirubin in the skin to a water soluble photo isomer that is excreted in bile* -_____ should decrease TSB by *30-40%* in the *first 24hrs* (most of this is in the first 4-6hrsexchange transfusion-initiated when TSB levels are becoming *toxic* as per established charts indications: -any infant with *TSB > 30mm/dL* -signs of encephalopathy -*failure of phototherapy to lower TSB by 0.5mg/dL per hour after 4hrs50% 80%*exchange transfusion decreases TSB by _____* and *removes approximately _____ of sensitized or abnormal RBCs* and antibodies1-5%exchange transfusions are not without complication! -*mortality rate* is _____-_____% -should be done at *referral center* -do phototherapy in transportRh imcompatabillityRh isoimmunization due to _____ _____ -*Rh negative mother is sensitized from a previous pregnancy and develops antibodies* -subsequent pregnancies show increased severity of disease -second child may show no serous diseaseRhoGAMprevention: _____ at *28 weeks gestation* and within *3 days of miscarriage, abortion, or delivery of an Rh positive fetus*D antigenthe *RhoGAM* only *prevents sensitization* of _____ _____ (the most common of the 5 antigens- C, D, E, c and e)anemiaRh Isoimmunization: affected neonates are usually _____ at brith and *continued hemolysis caused hyperbilirubinemia*Erythroblastosis FetalisRh Isoimmunization: most severe form caused _____ _____ which is characterized by 1 fetal anemia 2 heart failure 3 hypoalbuminemia also called *fetal hydrops*deathwith Rh Isoimmunization: high risk of fetal or neonatal _____ascitesRh Isoimmiunization: clinically manifested as _____, *pleura and pericardial effusion, anasarca* (severe generalized edema)anasarca_____ is severe *generalized edema*intrauterine transfusion of Rh negative cells into the umbilical vein or into the fetal abdominal cavity possible early delivery intravenous immune globulin; given immediately upon diagnosis; may decrease need for exchange transfusion (given to mom)treatment for Erythroblastosis Fetalis (Fetal Hydrops)? **before birthphototherapy and or exchange transfusiontreatment for Erythroblastosis Fetalis (Fetal Hydrops)? **after delivery2 - 3 monthsin treatment for Erythroblastosis Fetalis (Fetal Hydrops) infants require *monitoring* for _____-_____ month shemolysisRh Isoimmunization: less severe cased cause _____ which results in *anemia and hyperbilirubinemia*phototherapy and or exchange transfusionless severe cases of Rh Isoimmunization treatment?ABO incompatibility_____ _____ most common cause of *isoimmune hemolysis* in newbornCoombs testwith ABO incompatibility the _____ _____ may only positive in 33%ABO incompatibility_____ _____ usually occurs when a *Type O mother has IgG antibodies to A or B* as well as ABsensitizedwith *ABO incompatibility* the mother is more commonly _____ than Th so the *first born may be affected*hemolysisin *ABO incompatibility* _____ is *less severe* than in Rh and not progressive with subsequent pregnancies; less than 10% require treatmentanemia and jaundice as necessaryTx for *ABO incompatibility*?hereditary spherocytosis_____ _____ is the *most common cell membrane defect*; RBCs; heterogenous presents with variable degrees of -anemia -jaundice -splenomegalyhereditary spherocytosis_____ _____ pathophysiology: decreased red blood cell deformity causes *RBCs to get trapped in microvascualture of spleen* -RBCs then become engulfed by macrophages -hemolysis causes hyperbilirubenima in 50% of all affected pts -anemia may be severe -*splenomegaly* may be present at brith but will develop in the majority by *5 years old*hereditary spherocytosisLabs: hemoglobin: normal to low anemia is usually *normocytic (normal MCV) and hyperchromic (high MHC)*microspherocytesin *hereditary spherocytosis*; a peripheral smear shows numerous _____; spherocytes can also be present in forms of innumerable hemolysisphototherapy treat jaundice in newborn; transfusion may be needed for anemia or jaundice folic acid given to prevent RBC hypoplasia splenectomy performed by age 5 (pneumococcal, meningococcal, and HIB vaccine given prior to splenectomy)treatment for *hereditary soherocytosis*?G6PD deficiency (glucose 6 phosphate dehydrogenase deficiency)*most common red cell enzyme defect* that causes hemolytic anemia -more common in African, Mediterranean, and asian descentX-Linked Disorder*G6PD deficiency* is an _____ _____; even heterozygous females are at risk for hyperbilirubeminaoxidant stress episodes-G6PD deficiency -older cells are more deficient and unable to hold up against _____ _____ -results in _____ of hemolysis rather than a chronic hemolytic anemiahemoglobin retic "bite"Labs: for *G6PD deficiency* during episodes of hemolysis -low _____ -increased _____ count -"_____" or blister cells may be seen in peripheral smearreducedG6PD deficiency *confirmation by finding _____ levels of G6PD* in erythrocytesnorrmalsince *G6PD is found in higher amounts in reticulocytes*, confirmation must be done when reticular count is near _____phototherapy and or exchange transfusionTx of *G6PD deficiency*: infants usually have significant jaundice and anemia requiring _____ and or _____ _____ -onset of jaundice usually later than in autoimmune hemolysisepisodic transfusionsTx of *G6PD deficiency*: *older children* need _____ _____fava beansTx *G6PD deficiency*: infections need prompt treatment with appropriate antibiotics; *avoid medications* associated with hemolysis and _____ _____cardiac or *pulmonary**respiratory distress in the newborn* may be due to _____ or _____ disease -*respirations > 60* -retractions -cyanosis -nasal flaring -expiratory gruntingtransient tachypnea of the newborn (TTN)-also called *Retained Fetal Lung Fluid* -due to *delayed clearance* of fetal lung fluid (alveolar) via circulation/pulmonary lymphatics and replacement with air -distress is present from *brith usually lasts 24hrs*; temporary respiratory distress -CxR shows *fluid in interloper tissues* and *perihilar streaking*(nasal CPAP* can be helpful in addition to general measures for respiratory distressTx for *transient tachypnea of the newborn*?meconium aspirations-newborn will usually have *meconium in the amniotic fluid at brith*; low APGAR scores -cyanosis -retractions -over distended barrel chest -coarse breath sounds -CxR: hyperinflation and irregular infiltrates; occasional lobar consolidationpneumonia-lungs* are most common *site of infection in newborns* -usually *infection ascends through genital tract before or during labor* -*Group B Street (+) and E. Coli (-)* most Lilley agentspneumoniaincreased risk of _____ if: -*rupture of membranes > 24hrs before delivery* -chorioaminoitis -*maternal antibiotic administration*pneumonia-usually Sx *appear within 6-12hrs* -respiratory distress may be present at brith or be delayed for a few hrs -shock, poor perfusion, absolute neutropenia (<2000/mL) and elevated CRP support the diagnosispneumoniaCxR may resemble that of transient tachypnea r hyaline membrane disease rarely a lobar infiltrate treat: Ampicillin (+) and Gentamicin (-) 3rd gen covers + and -; cephalospontaneous pneumothorax-breath sounds will be *decreased on ONE side* -respiratory distress usually present at brith -heart sounds may be shifted toward opposite side and may be distant -collapsed lungspontaneous pneumothoraxTx: supplemental oxygen; watchful waiting -if large _____ or not resolving; draining by needle *thoracentesis or tube thoracotomy* may be necessaryrenal abnormalitiesslight *increased risk of _____ _____* in newborn with *spontaneous pneumothorax*hyaline membrane disease (respiratory distress syndrome or RDS)-*#1 cause of respiratory distress* in a *PREterm newborn* -caused by a *deficiency of surfactant*surfactant_____ *decreases surface tension* in the *alveolus* during expiration which allows the alveolus to remain *partially expanded*compliance and atelectasis*lack of surfactant* results in poor lung _____ and _____hyaline membrane disease (respiratory distress syndrome or RDS)exam: decreased air exchange desire *great respiratory effort* -baby has to use a lot of effort to expand lungs with each breathe- tires daily -CxR: *ground glass appearance*; from diffuse atelectasishyaline membrane disease (respiratory distress syndrome or RDS)treatment for which disease? -oxygen supplementation -nasal CPAP -intubation for surfactant administration; 1sr dose within 2-4hrs -placement of umbilical arterial and venous lineshyaline membrane disease (respiratory distress syndrome or RDS)3 commercially available surfactants given *intratracheally* -total of 2-3 doses given 8-12 hrs apart may be administered *Bovine derived* Surbanta *Calf lung extract* (Infasurf) *Porcine derived* (Curosurf)overall treatment for respiratory distress-oxygen to maintain *PAO2 at 60-70* mmhG and *O2 saturation at 92-95%* -O2 should be *warmed*, humidified, and delivered through *air blender* -blood gases done by umbilical catheter -CBC, CxR, blood glucose -*intubate and ventilate impending respiratory failure (PAO2< 60 mmhm)* -unless infection ruled out, blood cultures and begin *broad spectrum antibiotics*sepsis (early onset)-*early onset day 1-5* -most early onset _____ caused by *Group B Beta hemolytic strep (GBS)* or *gram negative enteric pathogens* -risk factors include: vaginal colonization with GBS, prolonged rupture of membranes, amnioitis, maternal fever, preterm brithsepsis (early onset)-most common presentation is *pneumonia with respiratory distress and low APGAR scores without fetal distress* -may also have respiratory failure, shock, meningitis, *peripheral gangrene*spinal tap for culture and cells, blood culture, U/A and CxRDx for *sepsis*?ampicillin and gentamicin for 10-14days or 3rd generation cephalosporinTx for *sepsis*?sepsis (late onset)-*day 5-28* -often in a *newborn that has been sent home* -_____ _____ sepsis often caused by GBS, staph, enterococcus, or gram negatives -also may be caused by organisms found in *older children* such as H. Flu, strep pneumococcus, and neisseria meningtiditidssepsis (late onset)-may have a more subtle presentation with lethargy, good feeding, hypotonia, temperature instability, apnea -may progress to seizures, bulging fontanelle, fever, jaundice -*usually hematogenous spread less to focal infections such as meningitis, osteomyelitis, arthritis, and UTI*sepsis (late onset)-evaluation is similar to that of its opposing sepsis but there is *increased attention to bones and urinary tract* -labs may show low WBC, neutropenia <1000/mL, and increased immature neutrophils -*hypoglycemia or hyperglycemia without charge in glucose administration* -*unexpected metabolic acidosis* -*increased CRP*sepsis (late onset)Tx: similar to the other form, but need to use *Staph coverage* especially if history of *indwelling line* (catheter) -*Vacomycin 10-15days* mg/kg q *8-24hrs* until cultures are back -may be used with 3rd generation cephalosporin when Pseudomonas is suspected35-37 weekssepsis (late onset) prevention of GBS: pregnant women screened at _____-_____weeks by vaginal culture -if positive--> *Penicillin given > 4hrs prior to delivery*