[Glucose-6-phosphate dehydrogenase deficiency of the mediterranean type B minus. 2. Etiological basis for severe hyperbilirubinemia in the newborn].
After having described in detail the pathophysiology, symptomatology, X-chromosomal inheritance and some laboratory methods in detecting G-6-PD-deficiency by demonstrating a case of favism (Schulz et al. 1977), the authors now discuss the particularities of the enzyme deficiency in the newborn. These are complicated by additional physiological and transient deficiency of the enzymes catalase, NAD-diaphorase, glutathione peroxidase, and glucuronyl transferase. Several chemical substances, acidosis, hypoxia, hypoglycemia, and immaturity may cause a severe hyperbilirubinemia in G-6-PD-deficient newborns. The development of a kern-icterus in these cases may be prevented by early exchange transfusion. From clinical findings and some observations in different regions of Greece an additional factor influencing the liver function has been postulated which favors the development of hyperbilirubinemias in G-6-PD-deficient newborns. The nature of this possible factor is discussed. The authors emphasize the necessity of screening for G-6-PD-deficiency during pregnancy in families of mediterranian descent.
D002506:Cephalexin / Q000627:therapeutic use; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000209:etiology*; D008297:Male; D011015:Pneumonia, Aspiration; D014552:Urinary Tract Infections / Q000188:drug therapy
D002506:Cephalexin
Glukose-6-Phosphat-Dehydrogenase-Defizienz vom mediterranen Typ B minus. 2. Atiologische Grundlage für schwere Neugeborenen-Hyperbilirubinämien.