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A bilirubin level ≥ 20 mg/dL (342 μmol/L) was chosen to define severe hyperbilirubinemia since an infant with this degree of jaundice is thought to be at major risk for neurologic damage [ 9, 17- 19].
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Treatment for infants with this syndrome includes replacement therapy with surfactant.
Prognosis is poor most infants with this syndrome do not live beyond one year.
Although some infants with this finding have associated anomalies, this patient was otherwise well.
Up to 20% of infants with this condition have associated anomalies.
Infants with this condition die despite maximal medical intervention including inhaled nitric oxide therapy and extracorporeal membrane oxygenation.
Infants with this condition appear normal at birth, but, after a few days of milk feeding, they begin to vomit, become lethargic, fail to gain weight, and show an enlargement of the liver.
Over the last thirty years, our fundamental understanding of the genetics and pathogenesis of congenital heart disease has lagged the tremendous advances in the surgical and clinical care of infants with this group of disorders.
Since 1982 it has been possible, by rigorous depletion of T cells from the donor marrow, to use related marrow donors other than HLA-identical siblings for successful treatment of infants with this condition.
Infants with this disorder can be distinguished from those with organic illness by their rapid physical improvement after hospitalization.
As with IFN-γ, deficient IL-10 responses are early markers of atopic dermatitis [18] and are reported in infants with this disease [17].
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