Neonatal Neurology (Fourth Edition), 4th Edition by Gerald Fenichel MD

By Gerald Fenichel MD

Thoroughly up to date, completely referenced, and good illustrated, the fourth variation of Gerald Fenichel's vintage assessment, Neonatal Neurology walks you thru the most recent advances within the medical analysis and administration of neurological problems within the infant.

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The prognosis in newborns with hypoglycemia and seizures usually depends upon the underlying causes. Prolonged and severe episodes of hypoglycemia have a deleterious effect on the nervous system and require treatment along with the underlying causes. 0 g/kg of glucose is administered intravenously (IV) as 2 to 4 mL/kg of a 25% solution. Glucose infusions of 8 mg/kg/min follow as needed. A persistent but later-onset hypoglycemia of sufficient severity to cause seizures occurs in fructose intolerance, fructose-1,6-diphosphatase deficiency, and glycogen synthase deficiency.

Mutations in eleven different PEX genes cause this spectrum of disorders. PEX genes encode the proteins required for peroxisomal assembly. indd 45 9/11/06 8:11:11 PM 46 Neonatal Neurology The infantile syndromes of peroxisomal dysfunction are all disorders of peroxisomal biogenesis; the intrinsic protein membrane is identifiable, but all matrix enzymes are missing. The prototype, cerebrohepatorenal (Zellweger) syndrome (ZS), is a continuum of phenotypes that include neonatal adrenoleukodystrophy and infantile Refsum disease (see Ch.

Newborns that are hypotonic in utero and weak at birth may have difficulty adapting to extrauterine life and experience postnatal asphyxia and encephalopathy. Most breathe adequately at first and appear alert despite the generalized weakness because facial expression is relatively well preserved and extraocular movement is normal. Some newborns have paradoxical respiration because intercostal paralysis and thoracic collapse occur before diaphragmatic movement is impaired, while others have diaphragmatic paralysis as an initial feature.

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