Hyperoxia upregulates lung trypsin and metalloproteinase (MMP-8) noted in the bronchoalveolar lavage fluid along with an increase in alveolar capillary permeability. This suggests a role in the pathogenesis of acute lung injury constituting potential targets for therapy

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See page 395

Abnormal distribution of elastin in the persistent patent ductus arteriosus of Brown-Norway rats suggests impaired elastin metabolism thereby implicating a genetically determined factor that links PDA with aortic vascular fragility.

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See page 407

The novel ornithine transporter (ORNT1) genotype (T32R) in two families with the hyperornithine-hyperammonemia-homocitrullinuria (HHH) syndrome, a disorder of the urea cycle and ornithine degradation pathway, presents with a variable phenotype. Factors such as redundant transporters and mitochondrial lineage may contribute to this variable neurophysiological presentation.

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See page 423

Visual thresholds in very low birth weight infants without serious retinal or neurological abnormalities are not significantly different from those of term infants, suggesting that increased visual experience fails to influence visual sensitivity. The higher amplitudes in VLBW infants support a role for visual experience in responses to suprathreshold stimuli.

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See page 435

Cardiorespiratory dysregulation in girls with MeCP2 mutation confirmed Rett syndrome consisted of exaggerated irregular breathing with increased breathing frequency, mean airflow and heart rate during and around both normal breaths and breatholds. This dysregulation may render increased vulnerability to sudden death.

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See page 443

Children with congenital nasal pyriform aperture stenosis, a genetically heterogeneous disorder with phenotypic variability, have a higher frequency of hypothalamo-pituitary axis abnormalities justifying early life screening with brain magnetic resonance imaging and pituitary insufficiency evaluation.

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See page 478