Glycogen in human peripheral blood leukocytes. II. The macromolecular state of leukocyte glycogen.

Glycogen of normal human blood leukocytes was studied in cell suspensions containing chiefly neutrophiles. In electron micrographs of neutrophiles stained with lead the glycogen particles appear to be relatively uniform with a diameter of 20 mmu. At high magnification the 20 mmu particle appears to be composed of at least eight subunits. Leukocyte glycogen released by lysis or homogenization sediments as a single peak of high molecular weight material. The great majority of the cell glycogen can be accounted for in the large molecular weight material. The large molecular weight material is degraded to small fragments by alpha-amylase and partially degraded by beta-amylase. Purification of cell glycogen by alkali extraction and ethanol precipitation produces a relatively uniform particle smaller than the original native macromolecule. Native glycogen was prepared in pure form by a sucrose density gradient technique and its purity demonstrated by its susceptibility to purified alpha-amylase and by analytical ultracentrifugation.

Manifestations of systemic light chain deposition.

In two patients with terminal renal failure, manifestations of disease developed in multiple organ systems. One had a previous diagnosis of multiple myeloma with kappa light chain proteinemia and proteinuria. The other had idiopathic lobular glomerulonephritis. Hepatic and neurologic abnormalities developed in both; in the latter gastrointestinal, cardiac and endocrine disease developed as well. Clinical and pathologic correlations suggest that the retention and tissue deposition of light chains produced the organ dysfunction, inasmuch as free kappa light chain determinants were demonstrated histologically in the clinically affected organs. The deposition in these patients may be an extreme example of a common but previously unrecognized form of plasma cell dyscrasia.

Sclerosing hemangioma of the lung: an endothelial or epithelial neoplasm?

The ultrastructural features of a sclerosing hemangioma of the lung in a 34 year old woman are described. The basic cellular response is thought to be endothelial and not epithelial. These conclusions are based on the finding of Weibel-Palade bodies within the tumor cells in addition to other characteristics generally associated with endothelial cells.

Severe combined immunodeficiency with thymic mast cell hyperplasia.

The autopsy finding on an infant with severe combined immunodeficiency was marked thymic mast cell hyperplasia. The clinical immune status showed a deficit in T (thymus derived) lymphocytes and low immunoglobulin levels. The autopsy showed the histologic pattern designated thymic alymphoplasia and a marked lack of development of the reticuloendothelial organs. The mast cell hyperplasia may be the result of an antigenic stimulus early in embryonic life and/or the result of mast cell differentiation at the expense of normal thymic lymphoid development caused by a genetic defect.

Intravascular fibrin deposits, hepatic infarcts and thrombocytopenia in parent/F mouse chimeras with host-versus-graft syndrome.

Host-versus-graft (HVG) disease is the fatal result of the allogenic reaction which occurs in parental strain mice perinatally inoculated with F(1) hybrid spleen cells. The principal manifestations of the syndrome in RFM/(T(6) X RFM)F(1) mice are thrombocytopenia, intestinal hemorrhage, hepatic necrosis, lymphoproliferative disorders and renal disease due to immune complexes. The discovery of intravascular fibrin deposits in the present studies establishes disseminated intravascular coagulation (DIC) as an intermediary mechanism of HVG disease. It is suggested that the characteristic declines in blood platelet levels, intestinal hemorrhages and hepatic infarcts are triggered principally by immune complexes. Cellular infiltrates of the liver, granulocytosis and hypergammaglobulinemia are other abnormalities which are regularly found in HVG mice and which are also thought to predispose to DIC.

Renal biopsies under ultrasound guidance: 100 consecutive biopsies in children.

To examine the number of renal biopsies in correlation with the pattern of referrals as well as the major categories of renal diseases for the procedure peculiar to children, the data of 100 consecutive percutaneous renal biopsies using disposable biopsy needles under ultrasound guidance in 99 children were reviewed. To examine the risks and accuracy of the procedure the estimated depth of the kidney by ultrasound, the actual biopsy depth, the success rate and the complications were evaluated. With rare exception, 1 core of renal tissue was obtained per single needle insertion; 109 needle insertions yielded 103 cores. Of the specimens 92 per cent contained more than 10 glomeruli. Gross hematuria with the first voiding post-biopsy occurred in 12 per cent of the cases but persisted beyond 24 hours in only 1 per cent. No long-term complications were observed. Percutaneous renal biopsy with the disposable needle guided by ultrasound represents a considerable advance in diagnostic technique and deserves wider application in the evaluation and management of a number of pediatric kidney disorders.

Platelet injury during phototherapy.

Phototherapy with blue fluorescent light is widely employed for treatment of neonatal hyperbilirubinemia. Functional, biochemical, and morphologic changes produced by blue fluorescent light in human platelets were identified and characterized. Platelet-rich plasma was exposed for up to 170 min to amounts of light equivalent to that used in phototherapy of neonatal hyperbilirubinemia. Within 110 min of light exposure, platelets were essentially no longer aggregable by ADP and connective tissue suspension and were depleted of ADP, ATP, and glycogen. Electron photomicrographs revealed these platelets to be swollen, depleted of glycogen granules and organelles, and to have ill-defined membranes. Platelet injury could be accelerated by adding a photosensitizing agent, hematoporphyrin, to platelet samples before exposure. In contrast, control platelets kept in the dark for 170 min or nonirradiated platelets resuspended in irradiated plasma maintained their integrity. The results indicate that platelets are damaged in vitro when exposed to amounts of blue light used in phototherapy.

Liver glycogen accumulation after cyanate treatment.

Sodium cyanate has been proposed as a therapy for sickle cell anemia. Histologic studies have suggested abnormal accumulation of glycogen in livers of rats. Quantitative liver glycogen determinations reported here showed a significant increase (P smaller than 0.05), which, however, was mobilized normally during fasting and after glucagon injections.

Acute hepatic coma successfully treated by extracorporeal baboon liver perfusions.

Two patients in deep hepatic coma due to fulminant viral hepatitis were treated by extracorporeal baboon liver perfusion after failing to respond to medical treatment and three consecutive exchange transfusions. Both patients recovered full consciousness after one liver perfusion, made a complete recovery, and were leading normal lives seven and eight months after treatment. Perfusions were maintained for 13(1/2) and 16(1/2) hours without complication, and neither clinical immunological reactions nor antibaboon serum antibodies developed as a result of treatment.Whereas normal consciousness could be restored only by liver perfusion, both exchange transfusion and liver perfusion were effective in clearing bilirubin and in raising the level of clotting factors. Extracorporeal baboon liver perfusion provides a safe and effective method for the treatment of acute hepatic coma.