ABSTRACT
We report the case of a young white man with Charcot-Marie-Tooth disease type 1 that began at 4 years. At 15 years, he developed proteinuria, arterial hypertension, and renal insufficiency. Renal biopsy specimens studied by electron microscopy showed deposition of nonamyloidotic microfibrils. This is the first report of fibrillary glomerulopathy associated with this neurological disorder.
Subject(s)
Charcot-Marie-Tooth Disease/complications , Glomerulonephritis/complications , Glomerulonephritis/diagnosis , Actin Cytoskeleton/ultrastructure , Adolescent , Glomerular Mesangium/pathology , Humans , MaleSubject(s)
Intestines/drug effects , Muscle, Smooth/drug effects , Phosphocreatine/pharmacology , Uterus/drug effects , Animals , Female , Mice , Rabbits , RatsABSTRACT
In the dog, rat and chick, phosphocreatine-Na has not caused, from an experimental point of view, significative modifications of the cardiovascular- and respiratory-apparatus, of the reactivity of the cardio-regulator centers, of the baroreceptorial carotid-sinus and glomus reactivity, of the gangliar-, muscarinic-, histaminergic-, dopaminergic-, beta-adrenergic- and serotoninergic- vasomotor reactivity; only the vasomotor reactivity of a constrictive-type induced by epinephrine, nor-epinephrine, occlusion of the two common carotid arteries, hypertension and by BaCl2 is moderately reduced. It is interesting to note that the hypotensive response evoked by adenosine was augmented.
