We report a male infant with Menkes’ disease who showed, at the age of 3 months, slow growth, hair abnormalities such as pili torti and white hair, and low levels of serum copper and ceruloplasmin. The exceptionally bright portions of his hair contained eumelanin and pheomelanin at levels only half those of normal Japanese controls. After subcutaneous administration of copper-histidinate for 2 months, his scalp hair changed to dark brown.

Abstract-To elucidate the pharmacokinetics of local anesthetics with respect to corneal permeability in the rabbit, we examined the relationship between the corneal permeability velocities of three agents, cocaine•HCI, procaine•HCI and tetracaine, HCI and corneal hydration.The corneal permeability velocity constants (k) of these three ester-type local anesthetics were approximately 0.5-6.0X10-6 cm/sec and the membrane permeability constants of these agents were approximately 0.5-4.0X10-7 cm2/sec, whereas the rabbit corneal hydration values were 3.2-4.2. Tetracaine-HCI with the strongest topical anesthetic action showed the greatest corneal hydration and the smallest corneal permeability velocity constant among these local anesthetics.

Salmon calcitonin (sCT) suppresses small intestinal transit (SIT) or motility, but the mechanism is not well understood. Bolus s. c. administration of a pharmacologic dose of sCT (140 IU/kg) to mice significantly decreased plasma calcium and phosphorus, and suppressed SIT from 1 to 8 h for plasma calcium and phosphorus or 20 h for SIT (respective maximal effects were seen at 5 h, between 2 and 8 h, and between 1 and 5 h). Significant SIT inhibition did not occur at doses smaller than 140 IU/kg. Reverse transcription-polymerase chain reactions and Southern analysis demonstrated high levels of calcitonin receptor mRNA in diencephalon and lung, moderate levels of mRNA in cerebellum, kidney, and muscle, and barely detectable amounts in cerebral cortex and thymus. No message was detectable in duodenum, jejunum, liver, testis, or heart. Specific binding of [125I] sCT was demonstrated in the diencephalon. Intracerebroventricular (i.c.v.) administration of sCT inhibited SIT time- and dose-dependently. Maximal inhibition was obtained at a dose of 4 IU/kg, 20 min after injection. Pretreatment with sCT (140 IU/kg s.c.) completely abolished inhibition of SIT by i.c.v. sCT (4 IU/kg). These results suggest that sCT binds to receptors in the central nervous system and inhibits small bowel transit.

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Corneas isolated from the pig eyeball have been treated in vitro with varying concentrations of befunolol hydrochloride, in order to test the topical effect of befunolol on the cornea in terms of opacity. Experiments were carried out with the corneas treated as follows: intact, epithelium-removed, endothelium-removed, and both epithelium- and endothelium-removed. Solutions of the drug were applied to both epithelial and endothelial surfaces, to the epithelial surface only, or to the endothelial surface only. When the drug was applied either to both surfaces or to the endothelial surface only, there was a significant increase in opacity. However, when applied to the epithelial surface only, befunolol caused an insignificant increase in opacity as compared with that of control. It is suggested, therefore, that corneal opacity due to befunolol hydrochloride topically administered to the eye, is concerned mainly with the epithelium in cornea, since the drug is uncapable of passing barrier probably existing in surface layer of epithelium but it easily penetrates in corneal layers from endothelial side to attain reversely to epithelial cells. Thus, it is probable that the risk to induce opacity is minor in case that befunolol is topically given in drops to the eye.