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Biomedical subjects

R Hong

Publications and source records attributed to R Hong.

251 records · Page 14Linked to original sources

Deficient complement fixation by aggregated gamma globulin from hypogammaglobulinemic patients.

We found that several normal samples of aggregated gamma globulin consistently fixed essentially equal amounts of guinea pig complement, while samples of gamma globulin from three of four hypogammaglobulinemic patients were markedly deficient in this biologic function. Thus, the previously observed limited heterogeneity of gamma globulin in patients with hypogammaglobulinemia is also associated with inefficiency in at least one biological function.

Agammaglobulinemia↗

Limited heterogeneity of gamma globulin in hypogammaglobulinemia.

When serums of 11 patients with hypogammaglobulinemia were examined by acrylamide-gel electrophoresis, combined with diffusion analysis, for the gamma chain of immunoglobulin G, the electrophoretic distribution of immunoglobulin G differed from that of normal individuals. The differences consisted of limited heterogeneity and bimodal distributions of the proteins at times. Isolated immunoglobulin G from four of the patients showed similar phenomena. The findings indicate a deficient gene population in these patients.

Agammaglobulinemia↗

Postthymectomy wasting associated with autoimmune phenomena. I. Antiglobulin-positive anemia in A and C57BL-6 Ks mice.

1. Mice of A and C(57)BL/6 Ks strains, thymectomized at birth acquire wasting disease in 84.1% (A) and 77.1% (C(57)BL/6 Ks) of the cases. There is no sex predelection. 2. Anemia in these animals is characterized by shortened red cell survival and increased fragility to hypotonic salt solutions. Among thymectomized A mice reticulocytosis is absent and extramedullary hematopoiesis is found in the spleen in the presence of bone marrow hypoplasia for the erythroid and lymphocyte series. 3. Positive antiglobulin tests of the red cells were observed in all the thymectomized C(57)BL/6 Ks (7/7) and 71.2% of the A strains (62/87). Normal mice do not show positive Coombs' tests. 4. The globulin coat on the A strain consists of IgM, whereas beta(1C) and IgG are not detectable. By contrast, red cell coats of NZB mice developing spontaneous autoimmune hemolytic anemia show IgM and beta(1C), but these erythrocytes do not react with anti-gamma chain antibodies. Another difference in the globulin coats of the two types of erythrocytes is that the IgM on NZB red cells has available light chain determinants but these are apparently hidden or absent in the case of sensitized erythrocytes. The difference in antibody coating, association with a component of complement in one but not the other, suggests a different mechanism for the immune surface phenomenon in each instance. 5. Anemia in NZB mice is associated with reticulocytosis while that in thymectomized A mice is not. 6. Thymectomy appears to initiate a chain of events leading to a series of autoimmune phenomena which may be due to alteration in host response consequent to loss of thymic tissue and thymic dependent functions or alternatively to infection to which increased susceptibility exists as a result of thymic extirpation.

Anemia, Hemolytic↗

Light chain distribution in immune deposits on glomeruli of kidneys in human renal disease.

The gammaG-immunoglobulin (IgG) deposited on glomeruli of 32 percutaneous renal biopsies from 30 patients with various forms of renal disease was examined by fluorescent techniques to determine its light chain composition. Serum kappa: lambda light chain ratios were determined on 17 serum samples from 16 of the 30 patients. Glomerular IgG staining with only antikappa antiserum, only antilambda antiserum, and with both antisera was demonstrated. No correlation of serum kappa: lambda light chain ratios with the type or types of light chains demonstrable on the glomeruli was observed. We concluded that the IgG deposited on glomeruli of patients with various forms of renal disease is not a nonspecific sample of the circulating immunoglobulin pool but may well reflect more specific selection, as in the deposition of specific antibody formed against exogenous or endogenous antigens during the development of the renal lesion.

Anti-Glomerular Basement Membrane Disease↗

The IgA system. I. Studies of the transport and immunochemistry of IgA in the saliva.

1. Five patients with congenital or acquired agammaglobulinemia, lacking detectable IgA in serum or saliva, were transfused with 1 to 2 liters of normal plasma. In 2 of these patients IgA was demonstrated in parotid saliva collected after transfusion, but in none of the 5 was salivary IgG or IgM found. This observation indicates the selective transport of IgA into saliva. 2. The observation by others of an immunochemical difference between serum and sahvary IgA globulin was confirmed. In contrast to serum IgA, salivary IgA is attached to a protein having antigenicity which migrates as a gamma(1) globulin. We have termed this protein component "transport piece". 3. The transport piece has been found in an unbound form in the saliva of persons completely lacking IgA: agammaglobulinemic patients, ataxia-telangiectasia patients, a healthy person lacking IgA, and a newborn infant. Free transport piece still occurs in the normal child's saliva after IgA production begins. By adulthood there is usually no free transport piece in the saliva. 4. Heat-aggregated salivary IgA, like heat-aggregated serum IgA, does not fix complement. 5. Our findings offer support for the view that there is a distinct local antibody system for the protection of the mucous surfaces.

Agammaglobulinemia↗

Initiation of warfarin therapy: comparison of physician dosing with computer-assisted dosing.

In a prospective, randomized study at two university hospitals, the authors examined how effectively housestaff physicians (n = 36) managed the initiation of warfarin therapy compared with a computer-assisted dosing regimen (n = 39) using the software program Warfcalc, which was managed by one of the authors. Target prothrombin time ratios were selected by the physicians. Study endpoints included: the time to reach a therapeutic prothrombin ratio, the time to reach a stable therapeutic dose, the number of patients transiently overanticoagulated, the number of bleeding complications, and the accuracy of the predicted maintenance dose, which was assessed at steady-state 10-14 days later. Computer-assisted dosing consistently out-performed the physicians: a stable therapeutic dose was achieved 3.7 days earlier (p = 0.002), fewer patients were overanticoagulated (10% versus 41%), and the predicted maintenance dose was in the therapeutic range in 85% of the computer-dosed patients versus 42% of the physician group (p less than 0.002). For physicians who did not routinely manage warfarin therapy, computer-assisted dosing improved the accuracy of dosing and shortened the time required to achieve a stable therapeutic dose.

Adult↗

Cryofixation, cryosubstitution, and immunoelectron microscopy: potential role in diagnostic pathology.

In immunoelectron microscopic investigations, retention of antigenic sites is crucial. Methods for preparing samples for conventional electron microscopy involve chemical fixatives followed by dehydration in organic solvents and embedding in plastic resins, all procedures potentially detrimental to antigenicity. Cryomethods provide a physical fixation alternative for the preparation of biological samples for ultrastructural, immunocytochemical, and microanalysis studies without the use of any chemicals. This can be particularly useful in diagnostic pathology, providing an alternative to conventional fixation methods which sometimes destroy the antigen in question. The recent development of a portable cryofixation device, the PS1000 Portable Metal Mirror Ultra-Rapid Cryofixation Unit (Delaware Diamond Knives, Inc., Wilmington, DE, USA), provides an opportunity to freeze tissue immediately after procurement for use in diagnostic immunocytochemistry studies. This feasibility study examined the quality of tissue preservation with this device, in terms of both preservation of cellular ultrastructure and immunolabeling. Human tonsil and thymus tissue was slam frozen and, after cryosubstitution in Lowicryl K11M, was examined by immunoelectron microscopy. Good ultrastructural preservation was obtained and reasonable immunolabeling with antibodies to AE1/AE3 keratin filaments was also observed.

Cryopreservation↗

Isolated pulmonary Goodpasture syndrome.

The case of a 13-year-old girl with Goodpasture syndrome is reported. The presentation at this age with only pulmonary hemorrhage is unusual among Goodpasture syndrome patients. The case illustrates well the diagnostic difficulties in Goodpasture syndrome. The choices of treatment modalities available for this disease and the results and risks of such treatment options are reviewed. A discussion and classification of diffuse pulmonary hemorrhage is presented. The case emphasizes that Goodpasture syndrome should be considered in the differential diagnosis of diffuse pulmonary hemorrhage in spite of a lack of renal abnormalities and that serum anti-glomerular basement membrane antibody testing can be of great help in confirming the diagnosis.

Adolescent↗