Basilar skull fractures in childhood with cranial nerve involvement.

Cranial nerve palsies developed in 23% of 73 children with basilar skull fractures. The majority occurred in patients 10 years of age or older. Our overall incidence was higher than that in a review in which basilar skull fractures were indiscriminately included with all head injuries. Our experience with meningitis was similar to that present in the literature and would seem to support avoidance of prophylactic antibiotics. Because there was no improvement in neurological deficits in patients treated with steroids (the only case of meningitis in our series was associated with steroids), we believe that their use is unwarranted. It is important to discuss the prognosis with the patient's family, because, although the majority (53.3%) of cases resolve completely, those with residual deficits can have significant morbidity.

Hemoglobin Louisville (beta-42 (CD1) phe-leu): an unstable variant causing mild hemolytic anemia.

An unstable hemoglobin variant termed Hb Louisville, was found in four members of a Caucasian family, who were suffering from a mild hemolytic anemia. The variant showed a decreased stability upon warming at 65 degrees C and an increased tendency to dissociate in the presence of sulfhydryl group-blocking agents. The structural abnormality was identified as a replacement of phenylalanyl residue in position 42 (CD1) by a leucyl residue. Substitution of this phenylalanyl residue, which participates in the contact with heme, by a nonpolar leucyl residue has apparently less severe consequences than a replacement of the same residue by a polar seryl residue as in Hb Hammersmith. Oxygen equilibrium studies of total hemolysate from one Hb Louisville heterozygote indicated a decreased oxygen affinity, a marked decrease in heme-heme interaction, and a normal Bohr effect. Studies with isolated Hb Louisville were not made because it was not possible to separate the variant from normal Hb A.