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Objective. Ibuprofen enhances cerebral blood flow autoregulation and was shown to protect neurologic functions after oxidative stresses in an animal model. For these reasons, we hypothesized that the prophylactic use of ibuprofen would reduce the occurrence of intraventricular hemorrhage (IVH) and its worsening toward grades 2 to 4 among preterm infants. To confirm this hypothesis, we planned the present prospective study. Methods. This was a double-blind, randomized, controlled trial in which preterm infants with gestational ages of Results. We studied 155 infants. Grade 2 to 4 IVH developed for 16% of the ibuprofen-treated infants and 13% of the infants in the placebo group. The occurrence of patent ductus arteriosus was less frequent only on day 3 of life in the ibuprofen group. There were no significant differences with respect to other complications or adverse effects. Conclusions. Our study demonstrated that prophylactic ibuprofen is ineffective in preventing grade 2 to 4 IVH and that its use for this indication cannot be recommended. Pediatrics 2005;115:1529-1535; ibuprofen, intraventricular hemorrhage, patent ductus arteriosus, infants. ABBREVIATIONS. IVH, intraventricular hemorrhage; PDA, patent ductus arteriosus; CBF, cerebral blood flow; iRDS, infant respiratory distress syndrome; ROP, retinopathy of prematurity; OR, odds ratio; CI, confidence interval; PVL, periventricular leukomalacia; PPHN, persistent pulmonary hypertension of the newborn., Despite improvements in the assistance and treatment of preterm infants, intraventricular hemorrhage (IVH) remains a frequent complication among these patients. (1) This point is crucial because the most severe IVH [...]

The two types of Crigler-Najjar (CN) disease, a rare disorder of bilirubin metabolism that can lead to severe neurological problems, may be distinguished by their response to phenobarbital treatment. Descriptions of three infants with CN revealed that two patients had type 2 CN, and were successfully treated with phenobarbital. The third infant's serum bilirubin did not decrease with phenobarbital treatment, and she eventually had a successful liver transplant. This third patient had type 1 disease. Bile pigment analysis has been relied on to differentiate the two types of CN, but this study suggests that only the response to phenobarbital therapy distinguishes the two. Phenobarbital therapy, however, may sometimes fail in the first few months of life of patients with type 2 CN. It is important to determine whether a patient has type 1 or type 2 CN, as type 1 CN may require liver transplantation., Objective. To differentiate between Crigler-Najjar (CN) disease types 1 and 2. Design. The patterns of serum bilirubins, bile pigment composition, and phenobarbital response were studied. Patients. Three infants, affected by high serum unconjugated bilirubin concentrations, previously classified as type 1 CN. Methods. Serum and bile bilirubin pigment composition, both before and after phenobarbital (PB) treatment, were determined by alkaline methanolysis and high-pressure liquid chromatography. PB was given for at least 3 weeks by oral administration (5 mg/kg bw per day). Results. No diconjugated bilirubin was found either before or after PB treatment in the serum of the three studied infants. In two patients traces of monoconjugated bilirubin were detected before PB therapy, and the ratio of conjugated/total bilirubin (percent) was increased by the PB response. In the third patient, traces of monoconjugated bilirubin appeared only after PB administration. However, the serum unconjugated bilirubin concentration decreased significantly only in the second patient, following the second cycle of PB treatment, leading to the diagnosis of type 2 CN. The analysis of the methyl ester derivatives of bile pigments was also performed on bile samples obtained in two patients by Entero-Test (R) both before and after PB treatment. An absolute increment in monoesterified bilirubin concentration was found after PB administration, although the percent concentration increased in one case and decreased in the other. No diesterified bilirubin was detected in the bile samples. Conclusions. The present results show that in types 1 and 2 CN disease it is possible to detect traces of monconjugated but not diconjugated bilirubin both in serum and in bile. Whereas PB treatment is effective in slightly increasing the serum monoconjugated bilirubin concentration even in type 1 CN disease, the diagnosis of type 1 or 2 is based on finding a substantial decrease of serum unconjugated bilirubin following PB administration. Pediatrics 1994;94:553-556; Crigler-Najjar disease, congenital hyperbilirubinemia, phenobarbital, pigment analysis, bile analysis.

The use of tin-mesoporphyrin (SnMP) to treat infants with jaundice may reduce the need for phototherapy. SnMP was used to successfully treat a 21-month-old infant with Crigler-Najjar (CN) type 1 disease. In CN type 1 disease, the body cannot remove bilirubin and jaundice results. The child received tin-protoporphyrin (SnPP) when six months old, but treatment was stopped when he developed a skin inflammation related to phototherapy. SnMP treatment was given at a low dose for 10 days, and at a higher dose for two weeks. The child received phototherapy for 10 hours each night. The bilirubin plasma level was much lower when the child was receiving the higher dose of SnMP than with the lower dose or with SnPP. Blue lamps should be used to prevent photoactivity problems resulting from SnMP., ABBREVIATIONS. SnPP, tin-protoporphyrin; SnMP, tin-mesoporhyrin; CN, Crigler-Najjar; BW, body weight. Tin-protoporphyrin (SnPP) and tin-mesoporphyrin (SnMP) have been proposed for the treatment of Crigler-Najjar (CN) disease type 1;(1)(2) in fact, both [...]