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Detailed information of NEONATAL GRAVES' DISEASE
NEONATAL GRAVES' DISEASE
DEFINITION:
A disorder caused by the transplacental passage of maternal
Thyroid-stimulating Immunoglobulin (TSI) to the fetus resulting in
thyrotoxicosis.
EPIDEMIOLOGY:
- incidence: 1% of infants born to mothers with Graves' Disease
- age of onset:
- risk factors:
- mother with Graves' Disease, rarely with lymphocytic
thryoiditis
- M = F
PATHOGENESIS:
- maternal levels of TSI must be at least 5x normal to produce
neonatal thyrotoxicosis thus only 1% of infants born to mothers
with Graves' Disease will be affected
- may occur in the absence of clinical maternal
hyperthyroidism
- clinical onset, severity, and course may be affected by the
concomitant transplacental passage of TSH-inhibiting antibodies
(TBIAb) and antithyroid medications (both tend to delay the
onset of thyrotoxicosis)
CLINICAL FEATURES:
- fetal tachycardia in 3rd trimester (>160 beats/min)
- many are premature
2. Neonatal
- thyrotoxicosis usually evident within hours of birth
although may be delayed for 4-6 weeks
- exophthalmos, flushing, hyperactive, irritable, tremor
- tachycardia, tachypnea, hypertension, elevated
temperature
- arrhythmias, congestive heart failure
- vomiting and diarrhea with weight loss despite a
voracious appetite
- hepatosplenomegaly, jaundice
- thyroid - goiter +/- airway obstruction
INVESTIGATIONS:
- increased T3, total and free T4; decreased TSH
- increased maternal TSI levels
- hyperbilirubinemia, prolonged PT, thrombocytopenia
2. Imaging Studies
- advanced bone age
- craniosynostosis
- frontal bossing
MANAGEMENT:
- titrate to keep heart rate <160 beats/min
- 5-15 mg/day po tid
2. Lugol Solution
3. For Cardiovascular Compromise
- IV fluids
- digitalization
- propranolol
2. Natural History
- usually remits spontaneously within 6-12 weeks but may
rarely persist for longer or years depending on the level of TSI
- 16% mortality rate
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Pediatric Database - NEONATAL GRAVES' DISEASE
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