Pseudo-pseudohypoparathyroidism and hereditary multiple exostoses

Pseudo-pseudohypoparathyroidism and hereditary multiple exostoses

Southern Society for Clinical high latex titers were subjected to three different methods for the isolation of the euglobulin fraction. Methods of i...

124KB Sizes 2 Downloads 91 Views


Society for Clinical

high latex titers were subjected to three different methods for the isolation of the euglobulin fraction. Methods of isolation were: (1) Euglobulin precipitation with hydrochloric acid, (2) Svartz cold agglutination, and (3) continuous electrophoresis. After their isolation the following tests were performed: Paper electrophoresis for proteins and carbohydrates, total protein-bound hexoses, hexosamine and sialic acid. One of the cold agglutination samples was subjected to further isolation studies for the isolation of mucopolysaccharides. The mucopolysaccharides were determined by Dishe’s carbazole reaction. The various euglobulin fractions were found to contain rather large amounts of glycoproteins. If one compares them with gamma globulin, the concentration of total protein-bound hexoses and hexosamines per gram of total protein is markedly elevated. Also a mucopolysaccharide has been partially identified by absorption spectra studies. SPIROPENTANE




Frank W. Summers. Dept. of Anesthesia, Charity Hospital, and Dept. of Surgery, Louisiana State Univ., School of Medicine, New Orleans, La.


Spiropentane is an isomer of pentane which has not been studied for anesthetic activity. The unique bicyclic ring, relatively low molecular weight, and high volatility of spiropentane suggests anesthetic properties. Only a small quantity was available for testing. Sixteen mice and two dogs were observed under spiropentane anesthesia. Electroencephalogram, electrocardiogram, blood pressure and respiratory exchange were observed in the dogs. Concentrations of spiropentane in the anesthesia circuit were determined by gas chromatography. A concentration of 3 vol. per cent produced anesthesia in one minute and 7 vol. per cent caused respiratory failure within twenty-five minutes. Convulsions, running movements, tachypnea and tachycardia were observed in concentrations producing anesthesia. This preliminary survey of the pharmacological properties in animals indicates that undesirable side actions of spiropentane would preclude its use as a clinically useful anesthetic agent. PSEUDO-PSEUDOHYPOPARATHYROIDISM



John N. Todd, III, John F. Nickerson and S. R. Hill, Jr. * Dept. of Medicine and Univ. Hospital, Univ. of Alabama REDITARY







Medical Center, and the Medical Hospital, Birmingham, Ala.


V. A.

During the evaluation of three generations of a family with eight members showing the characteristic stigmas of hereditary multiple exostoses, the striking similarity of this disorder to pseudo-pseudohypoparathyroidism became evident. Some of the members of this family not only had multiple exostoses, but also the short, thick set stature, round face and dyschondroplasia involving the metacarpal bones characteristic of pseudo-pseudohypoparathyroidism. The affected members of the family had normal serum calcium, phosphorus, alkaline phosphatase, creatinine and total protein levels. One member of the family, who was extensively studied, had a normal tubular reabsorption of phosphate, an adequate serum phosphate elevation following calciuminfusion, and an unsatisfactory phosphaturic response to the intravenous administration of parathyroid hormone (as did a control subject). In view of the similarity of pseudo-pseudohypoparathyroidism to a spectrum of disorders which includes not only idiopathic hypoparathyroidism and pseudohypoparathyroidism but also myositis ossificans progressiva, familial brachydactyly, epiphyseal dysplasia, and (as demonstrated by this family) hereditary multiple exostoses, a serious question is raised as to the advisability of classifying pseudopseudohypoparathyroidism as a distinct and separate syndrome. A clearer understanding of these disorders might be obtained by considering them as a spectrum of genetically determined bone, calcium and phosphorus disturbances with or without hypoparathyroidism. SECRETION GLAND.


Hospital, Arkansas,



J. Towbin and W. H. Perkins. V. A. and Dept. of Medicine, Univ. of School of Medicine, Little Rock, Ark.

In twenty-three technically satisfactory experiments on anesthetized dogs catheters were placed in a parotid vein, femoral artery and parotid duct. Salivary secretion was induced by pilocarpine or electrical stimulation approximately one hour after the intravenous infusion of NaI13i. The first specimen of saliva almost always had a much greater iodide concentration than subsequent specimens. In eighteen experiments parotid venous 1131 concentration rose above the arterial level for the first minute