摘要:

ABSTRACT: Insulin-dependent diabetes mellitus (IDDM) is caused by the destruction of the beta cells of the pancreas. This process is associated with the presence of islet cell antibodies (ICA). The risk for developing IDDM is associated closely with the presence of the HLA type DR3 and/or DR4. The risk is greatest for those with both DR3 and DR4. Recent evidence indicates that the DR antigens are only expressed in the beta cells of individuals who are developing IDDM. Activation of the DR antigen in beta cells apparently plays a role in the pathogenesis of IDDM. A process that turns off the expression of the DR antigen may stop the destruction of the beta cells. Preliminary evidence indicates that total suppression of beta cell function with an artificial pancreas (Biostator) significantly prolongs beta cell function well beyond that reproted for immunosuppressive drugs. The Biostator may work by total suppression of beta cell function, which turns off the expression of the DR antigen with a resultant cessation of beta cell destruction.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: Allogeneic bone marrow transplantation (BMT) was applied in 1968 to treat severe combined immunodeficiency disease (SCID). Almost simultaneously, marrow from an MHC-matched donor corrected the immunological deficiency of a patient with Wiscott-Aldrich Syndrome (WAS). In the first successful treatment of X-linked SCID the match was imperfect and, although SCID was cured, a graft vs. host reaction caused pancytopenia. A second BMT from the same donor successfully treated a complicating aplastic anemia. Subsequently, it has been possible to cure most patients with SCID who are in reasonably good condition at the time of BMT without other manipulation if a matched sibling donor is available. Successes are reported from Holland, France, Italy, England, Scandinavia, Japan, Germany, and from many centers in the United States. Similarly, BMT is used to correct SCID due to adenosine deaminase (ADA) deficiency or nucleoside phosphorylase (NP) deficiency, which underlie two forms of SCID. Bone marrow transplantation using HLA-matched sibling donors can now treat, successfully, at least eight genetically separable forms of SCID. Highly lethal defects of phagocytic function (including LFA-1, MO-1, CR-3 deficiencies, IL-2 and IL-1 receptor deficiencies), defects of killing after phagocytosis (as in chronic granulomatous disease, WAS, and Kostmann's Syndrome), and certain inborn errors of metabolism can be cured by BMT. In addition, occasional patients with combined immunodeficiency diseases, common variable immunodeficiency, X-linked infantile agammaglobulinemia, and even IgG3 subclass deficiency have been successfully treated by BMT. Successful treatment of SCID has been accomplished repeatedly by using marrow from an MHC-matched nonsibling relative donor when an MHC-matched sibling donor has not been available. More recently, successful BMT has employed haploidentical sibling or parental donors. Purging of the marrow of all T-cells and T-cell precursors is necessary if the latter is to work. The Chediak-Higachi anomaly and osteopetrosis have been successfully and repeatedly treated by BMT. Malignancy, which occurs with high frequency in SCID patients and also in high frequency in patients with WAS, has been completely prevented in patients with both disorders by fully successful BMT that entirely corrects the immunodeficiency. Partial correction of immunological function permits frequent occurrence of cancers.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: The results of high-dose chemoradiotherapy and marrow transplantation from an HLA-identical sibling for various stages and types of leukemia and lymphoma are summarized. It has been shown that marrow transplantation can be carried out successfully using partially matched family members or matched unrelated donors. Some of the complications of marrow transplantation are discussed.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: The first girl in a family was affected with late infantile metachromatic leukodystrophy (MLD) and had the expected characteristic central nervous system progressive deterioration, which resulted in deterioration and death. The second girl (propositus) demonstrated similar symptoms and signs at the same age. Both girls had characteristically low arylsulfatase A levels. The propositus underwent allogeneic bone marrow transplantation (BMT) from a normal histocompatible sibling. Two and a half years later, the propositus has not developed the intellectual and neurologic impariment demonstrated by the first sibling, although nerve conduction has continued to worsen. These results suggest that the induction of normal enzyme levels by BMT may be retarding or inhibiting CNS deterioration. These results, confirming earlier results of others, are suffciently promising to warrant a larger scale critical trial of BMT early in the course of MLD.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: To better define the role of antimicrobial therapy among U.S. travelers in Mexico, clinical and nonculture laboratory parameters were compared for 56 patients with shigellosis and 204 others with diarrhea of other causes. The presence of fever, stool mucus and blood, and fecal leukocytes were significantly more common among patients with shigellosis (p<0.001) who also tended not to present with mild diarrhea (p<0.05). However, clinical and laboratory parameters were either too insensitive or too nonspecific to be useful in identifying most cases of shigellosis or in excluding the likelihood of its presence. Patients with mild clinical presentations, regardless of etiology, experienced resolution of disease sooner than those with moderate to severe presentations (p<0.01), but withholding therapy from patients with mild presentations resulted in 48% of these patients remaining ill at the end of 48 hours. Based on these findings, the authors advise empiric use of antimicrobial agents in travelers with diarrhea associated with fever, bloody stools, or fecal leukocytes, and for all travelers with moderate and severe diarrhea. If therapy is withheld from patients with initially mild presentations, a proportion might still require therapy is withheld from patients with initially mild presentations, a proportion might still require therapy, possibly an antimicrobial agent, for optimal control of symptoms.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: The source for nosocomial Legionnaires' disease is the water distribution system. However, the implications for legionella contamination in a hospital without known Legionnaires' disease is unclear. Therefore, culturing forLegionella pneumophilain the environment has not been routinely recommended. The authors conducted a prospective pneumonia study in three hospitals, none of which was known to have a major problem with endemic legionellosis. The water system of Hospital 1 was colonized withL. pneumophila, serogroup 1; Hospital 2 was colonized byL. pneumophila, serogroup 5 (which is rarely associated with disease); Hospital 3 was essentially free ofL. pneumophila. Sputum culture on selective legionella media, direct fluorescent antibody testing, and serology were performed for all nosocomial pneumonias regardless of clinical impression. At the end of the study the incidence of nosocomial legionnaires' disease was found to be 9%, 0%, and 0% in Hospitals 1, 2, and 3, respectively. In Hospital 1, monoclonal antibody subtyping confirmed that the patient isolates were identical to the environmental isolates. The authors conclude that environmental culturing, despite the absence of known Legionnaires' disease, is useful. Positive cultures from the hospital water supply would mandate the introduction of legionella testing into the laboratory and stimulate physicians to consider Legionnaries' disease when encountering nosocomial pneumonias.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: The authors assessed the performance of a Bayesian and a least squares method for predicting individual pharmacokinetic parameters for vancomycin. For clearance, the best performance of both methods was an absolute error of approximately 5%. This level of accuracy required 4 serum vancomycin concentrations with the least squares method but could be achieved with a peak and trough concentration with the Bayesian method. For volume of distribution, the best performance occurred with 3 or 4 levels with both methods and amounted to an error of about 15%. In conclusion, both methods of estimating vancomycin pharmacokinetics perform comparably, but the Bayesian method appears to require fewer data.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: A variety of thiol reactive agents have been found to have antisickling properties thought to be due to the ability of these drugs to bind to hemoglobin, resulting in increased hemoglobin-oxygen affinity. Because thiol reactive agents also influence K movements in red cells and deoxygenation leads to K loss and Nagain in sickle erythrocytes, the authors investigated the possibility that deoxygenation-induced K loss could be influenced by thiol agents, independent of an effect on hemoglobin-oxygen affinity. Experiments were performed with the thiol crosslinking agent N,N'-p-phenylenedimaleimide (PMD). The authors found that PMD inhibited deoxygenation-induced K loss in sickle erythrocytes. This effect was not due to sickling inhibition as PMD-treated cells gained Na with deoxygenation, nor could the effect be explained by monofunctional PMD binding to membrane sulfhydryl groups, as a monofunctional analogue of PMD was not able to retard deoxygenation-induced K loss. These findings support a role for membrane sulfhydryl groups in deoxygenation-induced K movements in sickle red cells and sugges that this K loss may be prevented by crosslinking of certain membrane sulfhydryl groups.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID

摘要:

ABSTRACT: A chronic hemodialysis patient developed severe marrow granulocytic hypoplasia and peripheral blood neutropenia related to vancomycin therapy for an infected arteriovenous fistula. Neutropenia was prolonged and associated with sustained serum levels of vancomycin that persisted for more than 4 weeks following the last dose of vancomycin. No vancomycin-dependent leukoagglutinins were demonstrable in the patient's serum. Although a direct toxic effect on marrow granulocyte production seems likely, a vancomycin-dependent immune suppression of granulopoiesis cannot be ruled out.

ISSN:0002-9629    

出版商:OVID    

年代:1987

数据来源: OVID