Baggenstoss, Archie 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.

The records in 40 cases of congenital atresia of the extrahepatic biliary tract, proved at surgical exploration or necropsy or both, were studied at the Mayo Clinic with reference to clinical and pathologic aspects. Histologic examination of the liver was carried out in 21 of these cases; the muscular injections of vitamin K; each of these patients died during hospitalization.

Abstract A patient with symptomatic primary cryoglobulinemia was followed for 9 yr and was found at autopsy to have generalized amyloidosis. There was a monoclonal serum protein spike of IgG kappa type that was cryoglobulin. The cryoglobulin was further characterized as IgG2 kappa Gm (n-). This case constitutes, to our knowledge, the first example of amyloidosis associated with monoclonal IgG cryoglobulinemia, with characterization of the monoclonal serum cryoglobulin, and also the first reported case of successful symptomatic treatment of cryoglobulinemia by plasmapheresis.