Increased intracranial pressure in adrenoleukodystrophy.

Increased intracranial pressure developed as a terminal event in a 5-year-old boy with adrenoleukodystrophy. The CSF protein concentration was 420 mg/dL. Computed tomography showed extensive areas of decreased density in the posterior cerebral white matter. At autopsy, brain weight exceeded the expected value by 20%, and white matter water content was 87.6% (expected value, 72.5%). In rare instances, brain edema may contribute to the development of increased intracranial pressure in adrenoleukodystrophy.

Kinetic nephelometric determination of transthyretin and retinol-binding protein in neonatal serum.

We have developed a kinetic immunonephelometric method for the determination of retinol-binding protein and modified the method of Jacob et al (Clin Chem 1983; 29: 564) for the determination of transthyretin (prealbumin) in neonatal serum specimens from small, premature infants. The methodologies allow detection of 17.5 mg/l transthyretin and 1.7 mg/l retinol-binding protein in 25 microliter of serum. Between-run precision studies using pooled neonatal serum gave CV values of 3% and 5-6% for transthyretin and retinol-binding protein, respectively. Results obtained for neonatal specimens using this method agreed well with those obtained for the same specimens using radial immunodiffusion. Mean (SD) serum concentrations for 39 neonatal specimens were 100.4 (46.6) and 26.3 (10.8) mg/l for transthyretin and retinol-binding protein, respectively.

Hemorrhagic shock and encephalopathy. Clinical definition of a catastrophic syndrome in infants.

We treated nine infants who unexpectedly developed shock, seizures, and fever, followed by diarrhea, consumption coagulopathy, and hepatorenal dysfunction. Despite vigorous treatment, three infants died and all except one of the six survivors have severe residual neurologic abnormalities. Postmortem findings included cerebral edema, white matter petechial hemorrhages, gastrointestinal hemorrhages, and fatty liver. These clinicopathologic features are similar to those previously described in 10 infants as being due to hemorrhagic shock and encephalopathy, except for the presence of fatty liver in our patients. Based on the combined experience of 19 infants, we propose diagnostic criteria for hemorrhagic shock and encephalopathy that may facilitate recognition and differentiation from other shock syndromes in infancy.

New prospective studies of the association of Ureaplasma urealyticum colonization and chronic lung disease.

This study examined the association between Ureaplasma urealyticum colonization and the development of chronic lung disease (CLD) in 93 premature infants who were treated with surfactant and who had birth weights < 1251 g. Nasopharyngeal and tracheal cultures for U. urealyticum were obtained at 2 +/- 1 and at 14 +/- 1 days after birth and were positive in 17 (18%) of 93 patients. Infants born vaginally were 4.5 times more likely to be colonized than were those born by cesarean section. Colonization with U. urealyticum was associated with 1.66 (95% confidence interval, 1.24-2.20, P = .024) times the risk of developing CLD and with a greater incidence of > or = 2+ polymorphonuclear leukocytes in the tracheal aspirate at 2 +/- 1 days of age compared with uncolonized infants (P = .025). We conclude that U. urealyticum colonization is associated with CLD even after surfactant treatment. The presence of U. urealyticum is also associated with inflammatory cells in the tracheal aspirate.

Mortalities associated with use of a commercial suspension of polyvinyl alcohol.

Two cases of symptomatic neonatal hepatic arteriovenous malformation (AVM) are presented. Both were treated with percutaneous transcatheter embolization and a commercially available polyvinyl alcohol suspension. Both infants died soon after AVM embolization. The results of laboratory examination of particle-size homogeneity of this commercial suspension demonstrate marked inhomogeneity of particle size very probably contributed to the death of these patients. A protocol has been developed to help determine appropriate particle size in individual cases; this may help prevent such catastrophic results during transcatheter embolization.