Power, Marschelle H.

The theoretic considerations which indicate that fibrocystic disease of the pancreas is the result of a congenital deficiency of secretin have been outlined. The varied clinical and pathologic manifestations of this disease can, in our opinion, be more nearly adequately explained by this hypothesis than by any of the other current theories. The absence of secretin (S1) in the upper part of the intestinal tract of a patient with fibrocystic disease of the pancreas lends support to this theory; which is to say, that the condition is the result of a congenital deficiency of secretin. It has been shown to be possible to extract secretin from the upper part of the intestinal tract of persons two months to 73 years old. The only consistent, failures occurred in work with patients in the neonatal period. Successful extraction of secretin from these patients in the neonatal period can be carried out, however, by combining the crude extracts obtained from a number of patients. The extraction of secretin is successful if it is done up to 15 hours postmortem. If the specimen is kept in a frozen state, extraction can be delayed without loss of activity as long as 39 days. Because this method of extraction is successful in individual cases at necropsy, it becomes possible to correlate the presence or absence of secretin with varying pathologic conditions. This, in turn, may open up a broad field for the investigation of many pancreatic and intestinal disturbances in addition to fibrocystic disease of the pancreas.

A case is described in which oxalosis occurred in an 11-year-old boy. The patient had experienced the formation of numerous calculi and multiple operations for removal of the calculi. The clinical, pathologic and histochemical findings in the case are presented. The unusual features of the case are extensive deposits of calcium oxalate crystals in the kidneys, bone marrow, spleen, liver, blood vessels, and adrenal cortex. Calcium oxalate, a relatively inert substance, appears to effect renal damage by virtue of its deposition in the renal tissue. While many aspects of oxalate metabolism remain obscure, it is generally presumed that oxalosis represents an as yet undefined metabolic defect.

Observations on two girls in whom an unusual type of chronic renal insufficiency developed many months after the onset of nephrotic syndrome are reported. Each patient became free of edema in spite of persistent massive proteinuria. Growth was retarded and rickets and attacks of tetany developed. The chemical disturbances of the blood were characterized by hypocalcemia, hypokalemia, azotemia and metabolic acidosis. Hyposthenuria, proteinuria, amino-aciduria, and minimal erythrocyturia, cylindruria and glycosuria were present. Healing of the rickets and cessation of attacks of tetany followed the administration of vitamin D and calcium salts. Prednisone was administered to one patient and thereafter proteinuria decreased and renal tubular function improved. Both girls are relatively asymptomatic 11 and 9 years after the onset of nephrotic syndrome, although they are rather small and still have evidence of renal disease. It is possible that cells of the renal tubules have been damaged as a result of prolonged massive proteinuria.