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Neonatal Iron Storage Disease {NISD}

  1. NISD, also known as neonatal hemochromatosis, is a rare form of fulminant liver disease that presents in the 1st few days of life.
  2. Evidence of a gestational insult is given by the fact that affected infants may be born prematurely or with intrauterine growth retardation.
  3. Several infants with NISD also have renal dysgenesis. Q
  4. Clinically, NISD is a rapidly fatal, progressive illness christened by hepatomegaly, hypoglycemia, Q hypoprothrombinemia, hypoalbuminemia, hyperferritinemia, and hyperbilirubinemia.
  5. The coagulopathy Q is refractory to therapy with vitamin K.
  6. The diagnosis can be confirmed through documentation of extrahepatic siderosis (biopsy material of buccal mucosal glands is laden with iron) or MRI determination of iron storage in organs such as the pancreas.
  7. NISD has a high rate of recurrence in families, Q with =80%.
  8. All infants survived with medical therapy or no therapy.
  9. The majority of babies had biochemical evidence of liver disease with elevated serum -fetoprotein and ferritin.
  10. Some patients with NISD have been successfully treated with iron chelating agents (deferoxamine) combined with aggressive antioxidant therapy.
  11. This may be effective if initiated very early- Liver transplantation should also be an early consideration.

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