med to be keratan sulfate-like mucopolysaccharide, with similar material and probably a ganglioside in the Kupffer cells. In type 2 GM1 gangliosidosis, there is minimal storage of water soluble material and abundant presumed ganglioside in the Kupffer cells. The histochemical and ultrastructural features support the chemical findings and indicate a quantitative and morphologic difference in storage between the two types. These morphologic differences may provide an additional diagnostic parameter in the delineation of the two clinical variants.

Distal renal tubular acidosis: molecular and clinical aspects.
The traditionally descriptive classification of DRTA disorders is being refined as molecular defects are identified. Diagnosis of DRTA is easy: Hyperchloremic acidosis should arouse suspicion, and confirmation can be obtained by measuring urinary pH and ammonia excretion. Treatment is also relatively straightforward and readily corrects all manifestations of the acidification defect.

Universal screening for prenatal alcohol exposure: a progress report of a pilot study in the region of Grey Bruce, Ontario.
The main objective of this study is to evaluate the clinical utility of meconium analysis for fatty acid ethyl esters as a universal screening tool intended for the detection of newborns at risk for fetal alcohol spectrum disorder. This will be accomplished by assessing the rate of voluntary participation in a nonanonymous neonatal screening program and by determining the logistics of implementing the necessary follow-up and interventions as part of routine care. Additionally, this study will determine the predictive value of fatty acid ethyl ester-positive meconium with regard to neurodevelopmental delays. This is an ongoing prospective cohort study. Written informed consent is sought from all Grey Bruce women delivering at participating birthing sites. Collected meconium samples are tested for fatty acid ethyl esters by headspace-solid-phase microextraction followed by gas chromatography-mass spectrometry.