Aim-To confirm the mutation of the keratoepithelin gene in patients with a severe form of superficial juvenile granular corneal dystrophy (GCD). Methods-Five Japanese probands in whom GCD was diagnosed after histopathological examination and who developed severe manifestations of GCD in their first decade of life were investigated. Other aVected family members of two probands were also examined. All probands were the oVspring of consanguineous parents. DNA was extracted from their peripheral blood leucocytes and mutational analysis of the gene was performed by the polymerase chain reaction and direct sequencing. Results-Four of the five probands underwent their first keratectomy or keratoplasty in their teens and subsequently underwent a second or third keratoplasty. Each proband had a homozygous G → A transition at codon 124, replacing Arg → His, of the keratoepithelin gene. Their moderately aVected family members were heterozygous for the mutation. Conclusions-This finding suggests that the severity of the corneal phenotype depends on the dose eVect of the mutant gene. (Br J Ophthalmol 1998;82:1280-1284 Granular corneal dystrophy (GCD) is inherited in an autosomal dominant manner and becomes manifest in the first decade of life.
The observed decrease in drug clearances as a result of coadministration of flunixin and enrofloxacin indicates that these drugs interact during the elimination phase. Consequently, care should be taken during the concomitant use of flunixin and enrofloxacin in dogs to avoid adverse drug reactions.
Axial and secondary velocity profiles were measured in a model human central airway to clarify the oscillatory flow structure during high-frequency oscillation. We used a rigid model of human airways consisting of asymmetrical bifurcations up to third generation. Velocities in each branch of the bifurcations were measured by two-color laser-Doppler velocimeter. The secondary velocity magnitudes and the deflection of axial velocity were dependent not only on the branching angle and curvature ratio of each bifurcation, but also strongly depended on the shape of the path generated by the cascade of branches. Secondary flow velocities were higher in the left bronchus than in the right bronchus. This spatial variation of secondary flow was well correlated with differing gas transport rates between the left and right main bronchus.
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