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The reduction in human immunodeficiency virus (HIV) transmission through breastmilk with maternal combination antiretroviral therapy (cART) has led many pregnant women living with HIV and healthcare providers to question exclusive formula feeding in resource-rich settings. Here, we describe cART prophylaxis in 3 breastfed infants whose mothers had sustained virologic suppression; all 3 of these infants remained uninfected.

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OBJECTIVE: To describe and compare infants with severe hyperbilirubinemia, who presented with and without abnormal neurological findings and to identify associated risk factors. STUDY DESIGN: Data on infants with severe hyperbilirubinemia (>425 µmol l(-1) and/or received exchange transfusion) were collected prospectively through the Canadian Paediatric Surveillance Program (CPSP) from 2002 to 2004. Cases were categorized into two groups on the basis of information provided by the reporting physician: neurologically normal or abnormal. Demographic characteristics were compared and univariate logistic regression was performed to identify factors associated with acute neurological abnormalities in infants. RESULT: Of the initial cohort of 258 infants, 32 (12.4%) were identified to have neurological abnormalities. Infants in the highest peak bilirubin level group (>550 µmol l(-1)) had the greatest risk of acute neurological abnormalities. The mid range (451 to 550 µmol l(-1)) and lowest level (450 µmol l(-1)) groups were less likely to have abnormalities (odds ratio (OR)=0.174; P=0.0013 and 0.402; P=0.0613, respectively). Exchange transfusion and presentation within the first 2 days of age were positively associated with abnormal neurological findings in infants (OR=3.332, P=0.003 and OR=2.572, P<0.0001, respectively). CONCLUSION: In this national cohort of infants with severe hyperbilirubinemia, a significant percentage of infants developed acute bilirubin encephalopathy. Long-term neurodevelopmental follow-up is necessary to determine the incidence of permanent neurological sequelae.

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We report a twin pregnancy in which prenatal ultrasound revealed anhydramnios and bilateral absence of the kidneys in both fetuses. To overcome the difficulties faced in obtaining accurate information on fetal ultrasound in cases with oligohydramnios, an attempt was made to use fetal magnetic resonance imaging (MRI) to confirm the renal status. However, while the fetal MRI showed renal agenesis in both twins, postnatal follow-up revealed that one twin, which survived, had a small unilateral kidney not seen antenatally on fetal ultrasound or MRI. The second twin had bilateral renal agenesis and died of pulmonary hypoplasia. Care should be taken when using fetal MRI to replace fetal ultrasound in cases with poor visibility.

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Caroli's disease is a rare autosomal recessive condition characterized by cystic dilatation of the intrahepatic bile ducts and infantile polycystic kidney disease. We report a case with Caroli's disease detected prenatally at 33 weeks' gestation with fetal ultrasound findings of a cystic liver mass and echogenic kidneys. Postnatal investigation confirmed enlarged and echogenic kidneys with dilatation of the intrahepatic bile ducts consistent with the diagnosis of Caroli's disease. Genetic analysis of the gene, PKHD1, associated with autosomal recessive polycystic kidney disease (ARPKD) showed that the patient had compound heterozygous mutations, confirming that this early onset Caroli's disease was part of the spectrum of ARPKD. To our knowledge this is the third case of Caroli's disease detected prenatally and the first in which the infant survived.

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BACKGROUND: Attention deficit/hyperactivity disorder (ADHD), a well described, common problem affecting school-aged children, has an estimated prevalence in Ontario of 7% to 10% of boys and 3% of girls in the age range of four to 11 years. There has been a documented trend to increased use of stimulant medications in the treatment of this disorder in the United States. OBJECTIVE: To assess the prevalence of stimulant medication therapy for ADHD in three southern Ontario school boards. PATIENTS AND METHODS: A cross-sectional epidemiological study was performed by distributing a survey to all parents of children in kindergarten through grade 6 in six to eight schools selected randomly in each of the three participating school boards. The completed questionnaires were collated, and the comparative data were analyzed using chi(2). RESULTS: A total of 5100 surveys were distributed among the three school boards; 1465 (28.8%) questionnaires were returned completed. Within the three school boards - Hastings County Board of Education, Metropolitan Toronto Separate School Board and the East York Board of Education - the prevalence of ADHD for the age groups surveyed was 4.3%, 3.4% and 6.8%, respectively (average 4.7%), with a peak average of almost 9% by 12 years of age. The percentages of children with diagnosed ADHD who were on stimulant medication were 43%, 3% and 13%, respectively. The differences between the school boards were statistically significant (P<0.05). The male versus female prevalence of a diagnosis of ADHD was 7.1% versus 1.2%, 3.8% versus 3.3% and 10.1% versus 3.6%, respectively, with a combined school board average of 7.1% of males versus 2.9% of females. The average percentage of males versus females who were diagnosed with ADHD and who were on stimulant medication was found to be 27% versus 5%. CONCLUSIONS: The prevalence of ADHD was 4.7% in the study population. The overall percentage of children who were on stimulant medication was approximately 1%. Males were not only more likely to be diagnosed with ADHD but also more likely to be treated with stimulant medications if diagnosed. There was an increased prevalence of ADHD with older age, and the different school boards had significant differences in both the percentages of children who were diagnosed with ADHD and the percentages of children who were on medication, suggesting that individual school board policies or other factors may affect both the rate of diagnosis and the likelihood of stimulant drug treatment.

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We report a newborn, diagnosed prenatally with both cardiac rhabdomyomas and a brain tumor. To the best of our knowledge, this is the first report of central nervous system (CNS) lesions detected prenatally in a child with tuberous sclerosis with term follow-up. At 36 months, the child has normal growth and is developing appropriately. Thus the finding of CNS tumors on fetal ultrasound examination can help in the prenatal diagnosis of tuberous sclerosis but does not necessarily indicate a poor prognosis.

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We report a patient diagnosed prenatally on routine fetal ultrasound, at 30 weeks' gestation, with subdural haemorrhage. The mother had suffered a mild abdominal trauma and had Factor XI deficiency; however, both were felt to be aetiologically insignificant. Prenatal follow-up showed a complete resolution of the haematoma and no postnatal sequelae have been noted to date. The aetiologies and outcomes in the few previously reported cases are reviewed and compared with our case.

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