The KID-syndrome in Finland. A report of four cases

We describe a rare syndrome in a Finnish family, in which the father and his two sons are congenitally deaf and also exhibit corneal vascularization, and hyperkeratosis of the skin of the palms, soles, elbows and knees. In addition, all 3 suffer from fungal infections of the skin and nails. We also describe a fourth, sporadic case of a 9-year-old girl with the same disease. The KID (k = keratitis, i = ichthyosis, d = deafness) syndrome is an ectodermal dysplasia that probably arises through mutation, and is transmitted as an autosomal dominant in the family described by us.     

Success and complications of pneumatic retinopexy

Fifty-eight consecutive patients with rhegmatogenous retinal detachment were treated by pneumatic retinopexy using intravitreal perfluoropropane gas. Reattachment was achieved in 29 of 35 eyes (83%) having single retinal breaks (up to 45 degrees in extent), including dialyses (up to 60 degrees), and groups of breaks within one clock hour. The cumulative reattachment rate was 37 of 58 eyes (64%), including eyes with detachments with multiple breaks 30 to 120 degrees apart and vitreous hemorrhage as well as aphakic and pseudophakic eyes. Pneumatic retinopexy was successful in 18 of 22 (82%) myopic eyes (-3 to -11 diopters). Virtually all complications developed in the inferior retinal quadrants, and included preretinal vitreous condensations or membranes in 27 of 58 eyes (47%), new retinal breaks in seven eyes (12%), and rhegmatogenous or tractional detachments in previously attached areas in 14 eyes (24%). Postoperative proliferative vitreoretinopathy occurred in six eyes (10%) and macular pucker in two (3%).     

Influence of extracellular calcium in the effects of dibekacin and diltiazem on indirectly elicited tetanic responses in the rat phrenic-hemidiaphragm preparation

1. Dibekacin (70 microM-3 mM) produced a decrease of peak tetanic tension in a concentration-dependent manner and this effect was dependent on extracellular calcium (0.3-2.5 mM Ca2+). Only minimal fade was observed and it was not related with extracellular calcium concentrations. 2. Diltiazem (30-300 microM) decreased peak tetanic tension and produced tetanic fade. Both effects were independent of extracellular calcium, although a significant potentiation was observed at 0.3 mM calcium. 3. It is concluded that tetanic parameters are related differently to extracellular calcium.     

Synthesis and anticonvulsant properties of some arylsuccinate methylpyridylimides

In reaction of alpha-phenyl, alpha-p-chlorophenyl and alpha-m-chlorophenylsuccinic acid with various aminopyridines, N-pyridyl-substituted succinimides (compounds 1-14) were obtained. These compounds were investigated for their CNS activity. Compounds 1, 2, 5, 6 and 7 displayed anticonvulsant properties in the maximum electroshock test. Compounds 5 and 6 were also active in the pentetrazole test.     

Pathogenesis and changes in the aetiology of arterio-venous fistulae

There has been a marked shift in the causes of arterio-venous fistulae. Gunshot and stab wounds used to be the most frequent cause but iatrogenic fistulae, especially those arising as a result of reconstructive skeletal operations or following diagnostic and therapeutic procedures, are now becoming increasingly common. If morphological vascular changes have not yet occurred and cardiac volume overloading has not led to permanent damage, a recently established arterio-venous fistula can be easily corrected by resection and oversewing of the artery and vein. The special cases of arterio-venous fistulae arising as a result of catheter procedures in the groin have to be identified in terms of cause and morphology in order to facilitate early surgical correction.     

Chronic ethanol ingestion enhances catabolism and muscle protease activity in acutely uremic rats

Skeletal muscle wasting in men as well as enhanced urea production in animals due to ethanol consumption has been demonstrated by numerous authors. Furthermore, the outcome of acute renal failure is closely related to the extent of catabolism. The present study was performed to investigate whether chronic ethanol exposition prior to binephrectomy (BN) may represent a predisposing factor for enhanced protein breakdown. Rats underwent BN after exposure to ethanol or isocaloric substrate for 4 weeks. Blood chemistries and muscle samples were obtained 48 h after BN. Animals fed with ethanol revealed significantly higher levels of serum urea nitrogen (SUN) and urea nitrogen appearance (UNA) in comparison to controls. Preconditioning on ethanol-derived calories induced an accelerated fractional degradation rate of myofibrillar protein as demonstrated by a significantly enhanced serum Nt-methylhistidine/creatinine ratio. The increase in serum indicators of enhanced myofibrillar breakdown correlated with the stimulated activities of alkaline myofibrillar protease and cathepsin B. Finally, serum corticosterone levels were enhanced in the experimental group in comparison to controls, indicating an ethanol-related adrenocortical stimulation to be a possible mediating factor of enhanced catabolism in ARF. Thus, chronic ethanol intake prior to the onset of ARF seems to be a risk factor for enhanced catabolism in the course of acute uremia.