Download Rh-Mediated Isoimmune Hemolytic Disease

Rh-Mediated Isoimmune
Hemolytic Disease
Suzanne Reuter MD
SDPA ~ Spearfish
September 8, 2016
Objectives
 Understand heme catabolism
 Define Rh-isoimmune disease as it relates to a newborn
 Review abnormal indices in Rh-isoimmune disease
 Discuss therapies for hyperbilirubinemia
 Review complications of exchange transfusion
 Identify complications of phototherapy
Day of Delivery
 34 weeks gestation
 Maternal anti-D (1:64) and anti-C (1:8)
 2240 grams (5 lb)
 Apgars 71, 85
 Initial respiratory compromise necessitating use of:
 NIPPV
 NCPAP
 NC
Day of Delivery
 Physical Exam notable for:
 Pallor
 Jaundice
 Mild subcostal retractions
 Grade II/VI systolic murmur
 Mildly hyperdynamic precordium
 Liver 4 cm below the right costal margin
 Palpable spleen
Day of Delivery
 Initial lab results:
 Hematocrit: 22.6 %
 Platelets: 119,000
 Reticulocyte count: 23%
 Unconjugated bilirubin: 9.1
 Conjugated bilirubin: 0.1
 Blood type: A+ (antibody +)
 Maternal blood type: A- (antibody + x2)
 16 hours of age:
 Unconjugated bilirubin:18.8
What’s the concern?
Heme Catabolism
Kernicterus –
Bilirubin-Induced Neurologic
Dysfunction
 Bilirubin staining of the basal ganglia, hippocampus,
substancia nigra, brainstem nuclei
 Neuronal necrosis
 Choreoathetosis cerebral palsy
 High-frequency hearing loss
 Paralysis of upward gaze
 Dental enamel hypoplasia
 Cognitive function intact
Treatment
Treatment
 Intravenous Immunoglobulin – A LOT!
Threshold
 Bilirubin nomograms for 35 weeks and older
Threshold
 Premature infants: Half the first 2 numbers in the weight
 2240 grams = half of 22 = 11
 Bilirubin/albumin ratio (term infants)
B/A ratio > 7
→→→
 All bilirubin binding sites on albumin are saturated
Exchange Transfusion
Exchange Transfusion - Effects
 Positive
 Unconjugated bilirubin decreases
 Negative
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Acidosis
Hypernatremia
Hypocalcemia
Hypoglycemia
Hyperkalemia
Air embolus
Infection
Clots
Arrhythmia
BP instability
Intraventricular hemorrhage (premature infants)
Necrotizing Enterocolitis (NEC)
Increase in conjugated bilirubin (peak 0.7)
s/p Exchange Transfusion
 Bilirubin rebounded to 16.3 and albumin dropped to 2.9
 B/A ratio was 5.6
Complicating Factors…
Maternal Drug Use
 Suboxone (buprenophine) – Treatment of narcotic addiction
 Withdrawal in babies following birth (usually peak at 3d):
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Hypertonia
Tremors
Agitation
Myoclonic jerks
Apnea
Bradycardia
Neonatal Abstinence Syndrome
 Agitation, sneezing, jitteriness
 Treated with Fentanyl (narcotic), Phenobarbital (anti-seizure)
Photosensitivity Skin Rash
Photosensitivity Skin Rash
Remaining hospital course…
 Struggled with nipple feeds
 Slowly weaned off Fentanyl and Phenobarbital
 Phototherapy rash completely resolved with no residual skin
changes
Questions