pituitary adrenocortical system in pancreatic hyperinsulinism

1
92 Western Society for Clinical Research l’alatini muscle, which causrs apparent disa- bility of thr ipsilatcral oropharynx. Distinction of actual front sremillg weakness ol’ the ron- strictor musculature is made I)y cvalualion of transverse movement of the posterior pharyn- geal wall. The role of the levator and constrictor muscles in function of the pharynx has been demon- strated in comparative dissection studies of cadavers and cinema studies of patients having the pharynx exposed by maxillofacial surgery. The various patterns of paralytic disability of the pharynx in bulbar poliomyelitis have been studied by cinematography and by serial radiographic studies. PITUITARY ADRENOCORTICAL SYSTEM IN PAN- CREATIC HYPERINSULINISM. Harold Brown (in- troduced by F. H. Tyler), Department of Medicine, Veterans Administration Hospital and the University of Utah College of Medicine, Salt Lake City, Utah. A fifty-eight year old man with severe hypo- glycemia of eight years’ duration which was due to an islet cell adenoma of the pancreas was successfully treated with ACTH and cortisone prior to operation. The results of oral and intravenous glucose tolerance tests, insulin and epinephrine tests during the control, treatment and postoperative periods are reported. A nitrogen balance study during cortisone therapy indicated that the correction of hypoglycemia could not be at- tributed to the protein catabolic effect of cortisone but was due, presumably, to an anti- insulin effect in the tissues. It is postulated that even though exogenous hypercorticism can correct the hypoglycemia of hyperinsulinism, the potent stress of the hypo- glycemia cannot induce persistent endogenous hypercorticism because of the self-regulating mechanism of the pituitary adrenocortical system. If persistent endogenous hypercorticism did develop, it would be detrimental to other body systems. URINARY CHROMOGENSIN ACUTE PORPHYRIA. I;. B. Davison and R. A. Aldrich (introduced by Arthur Seaman), Departments of Biochemis- try, Pediatrics, and the Division of Clinical Pathology, University of Oregon Medical School, Portland, Ore. The urine of patients suffering from an attack of acute porphyria contains a number of sub- stances including uroporphyrin which are probably the result of the disturbed pyrrole mrtabolism in this disease. On(t of thrsc sul)- stances is an Ehrlich reacting c.hromogen which \Valdenstr6nl originally described. Many Ix- l&c that this substance, dcsignatccl porpho- bilinogen, is closely related to the toxicity of the disease and consequently is of more pathologic importance than the uroporphyrin. Porpho- bilinogen has never been entirely purified al- though several investigators have contributed to our knowledge of its properties. Waldenstriim and others believe that the substance may form either uroporphyrin or a dark brown pigment, porphobilin. This dual role depends on the conditions under which the conversion occurs. The presence of a colorless chromogen for coproporphyrin in the urine has been proved beyond doubt. It appears in normal, porphyria and other pathologic urines. Thus there is reason to speculate on the existence of a similar chromogen for uroporphyrin in porphyria which could account for the dual behavior of the so far incompletely purified porphobilinogen. In short, porphobilinogen could be two or more substances. Sveinsson et al. have provided a technic by which all preformed porphyrin may be quanti- tatively removed from porphyric urine, leaving the residual urine with a strong porphobilinogen test. We have made a study of urine from a case of acute porphyria using this technic. Evidence will be presented which may be interpreted to indicate that there is a chromogen of uropor- phyrin in this residual prophyrin-free urine and that it is separable from the Ehrlich reacting substance. OBSERVATIONS ON THE NATURE OF THE “STOR- AGE LESION” IN PRESERVEDBLOOD. C. A. Finch,* B. W. Gabrio and A. R. Stevens, Depart- ment of Medicine, University of Washington School of Medicine, Seattle, Wash. Satisfactory preservation of blood for three weeks has been possible at 4’~. using an acidified citrate-glucose media. Characteristic structural and biochemical changes in the erythrocyte have been described during storage. Because of the critical role that glucose plays in erythrocyte preservation, particular atten- tion in in vitro studies has been directed at the carbohydrate metabolism of the erythrocyte. In vivo measurements of cell survival have been made using erythrocytes tagged with radioiron in animals and by the differential agglutination technic in man. Changes occurring during normal cell aging in vivo have been compared AMERICAN JOURNAL OF MEDICINE

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92 Western Society for Clinical Research

l’alatini muscle, which causrs apparent disa- bility of thr ipsilatcral oropharynx. Distinction of actual front sremillg weakness ol’ the ron- strictor musculature is made I)y cvalualion of transverse movement of the posterior pharyn- geal wall.

The role of the levator and constrictor muscles in function of the pharynx has been demon- strated in comparative dissection studies of cadavers and cinema studies of patients having the pharynx exposed by maxillofacial surgery. The various patterns of paralytic disability of the pharynx in bulbar poliomyelitis have been studied by cinematography and by serial radiographic studies. PITUITARY ADRENOCORTICAL SYSTEM IN PAN-

CREATIC HYPERINSULINISM. Harold Brown (in- troduced by F. H. Tyler), Department of Medicine, Veterans Administration Hospital and the University of Utah College of Medicine, Salt Lake City, Utah. A fifty-eight year old man with severe hypo-

glycemia of eight years’ duration which was due to an islet cell adenoma of the pancreas was successfully treated with ACTH and cortisone prior to operation.

The results of oral and intravenous glucose tolerance tests, insulin and epinephrine tests during the control, treatment and postoperative periods are reported. A nitrogen balance study during cortisone therapy indicated that the correction of hypoglycemia could not be at- tributed to the protein catabolic effect of cortisone but was due, presumably, to an anti- insulin effect in the tissues.

It is postulated that even though exogenous hypercorticism can correct the hypoglycemia of hyperinsulinism, the potent stress of the hypo- glycemia cannot induce persistent endogenous hypercorticism because of the self-regulating mechanism of the pituitary adrenocortical system. If persistent endogenous hypercorticism did develop, it would be detrimental to other body systems. URINARY CHROMOGENS IN ACUTE PORPHYRIA.

I;. B. Davison and R. A. Aldrich (introduced by Arthur Seaman), Departments of Biochemis- try, Pediatrics, and the Division of Clinical Pathology, University of Oregon Medical School, Portland, Ore. The urine of patients suffering from an attack

of acute porphyria contains a number of sub- stances including uroporphyrin which are probably the result of the disturbed pyrrole

mrtabolism in this disease. On(t of thrsc sul)- stances is an Ehrlich reacting c.hromogen which \Valdenstr6nl originally described. Many Ix- l&c that this substance, dcsignatccl porpho- bilinogen, is closely related to the toxicity of the disease and consequently is of more pathologic importance than the uroporphyrin. Porpho- bilinogen has never been entirely purified al- though several investigators have contributed to our knowledge of its properties. Waldenstriim and others believe that the substance may form either uroporphyrin or a dark brown pigment, porphobilin. This dual role depends on the conditions under which the conversion occurs.

The presence of a colorless chromogen for coproporphyrin in the urine has been proved beyond doubt. It appears in normal, porphyria and other pathologic urines. Thus there is reason to speculate on the existence of a similar chromogen for uroporphyrin in porphyria which could account for the dual behavior of the so far incompletely purified porphobilinogen. In short, porphobilinogen could be two or more substances.

Sveinsson et al. have provided a technic by which all preformed porphyrin may be quanti- tatively removed from porphyric urine, leaving the residual urine with a strong porphobilinogen test. We have made a study of urine from a case of acute porphyria using this technic. Evidence will be presented which may be interpreted to indicate that there is a chromogen of uropor- phyrin in this residual prophyrin-free urine and that it is separable from the Ehrlich reacting substance. OBSERVATIONS ON THE NATURE OF THE “STOR-

AGE LESION” IN PRESERVED BLOOD. C. A. Finch,* B. W. Gabrio and A. R. Stevens, Depart- ment of Medicine, University of Washington School of Medicine, Seattle, Wash. Satisfactory preservation of blood for three

weeks has been possible at 4’~. using an acidified citrate-glucose media. Characteristic structural and biochemical changes in the erythrocyte have been described during storage.

Because of the critical role that glucose plays in erythrocyte preservation, particular atten- tion in in vitro studies has been directed at the carbohydrate metabolism of the erythrocyte. In vivo measurements of cell survival have been made using erythrocytes tagged with radioiron in animals and by the differential agglutination technic in man. Changes occurring during normal cell aging in vivo have been compared

AMERICAN JOURNAL OF MEDICINE