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Biomedical subjects

A Bodner

Publications and source records attributed to A Bodner.

17 recordsLinked to original sources

Liver transplantation in maple syrup urine disease.

UNLABELLED: Maple syrup urine disease (MSUD) is an autosomal recessive disorder. Impaired activity of the branched-chain 2-oxoacid dehydrogenase complex (BCOA-DH) causes accumulation of branched-chain L-amino (BCAA) and 2-oxoacids (BCOA) which may exert neurotoxic effects. Treatment comprises dietary management with strictly reduced quantities of protein and BCAA as well as aggressive intervention during acute neonatal and subsequent metabolic complications. MSUD is regarded as a metabolic disorder with potentially favourable outcome when the patients are kept on a carefully supervised long-term therapy. Up to now, three MSUD patients, exhibiting the classical form of the disease, have received orthotopic whole liver transplantation (OLT). Liver replacement resulted in a clear increase in whole body BCOA-DH activity to at least the level of very mild MSUD variants. These patients no longer require protein restricted diets and the risk of metabolic decompensation during catabolic events is apparently abolished. CONCLUSION: Considering the overall expenses, risks, and outcome, however, the benefit of OLT, even in the most severe form of MSUD, may not be significantly different from that of a classical strict dietary management. Thus, OLT appears not to represent a specific option in the treatment in MSUD.

Amino Acids↗

Assessment of whole body L-leucine oxidation by noninvasive L-[1-13C]leucine breath tests: a reappraisal in patients with maple syrup urine disease, obligate heterozygotes, and healthy subjects.

Suitability of a recently proposed noninvasive L-[13C]leucine breath test for assessment of whole body leucine oxidation in maple syrup urine disease (MSUD) was examined. Oral L-[1-13C]leucine loads (38 micromol/kg body weight) were performed in overnight fasted MSUD patients (n = 6, classical form), obligate heterozygote parents (n = 6), and control subjects (n = 10). Three-hour 13CO2 exhalation kinetics were evaluated using curve fitting procedures. Venous blood was obtained in most cases and analyzed for 13C-labeled plasma metabolites. In control subjects, maximal 13CO2 exhalation was reached at tmax = 55 +/- 18 min. Cumulative 13CO2 output at 3 h amounted to 4.7 +/- 0.7 micromol x (kg body weight)(-1). Estimated total 3CO2 exhalation was 7.2 +/- 1.4 micromol x (kg body weight)(-1) (19.0 +/- 3.6% of the dose). Half of this amount was expired at t1/2 = 130 +/- 18 min. The data show a considerable degree of intersubject variability. Intraindividual variability was comparable, however, when checked in two volunteers. In obligate heterozygotes, 13CO2 kinetics were similar to controls (tmax = 35 +/- 8 min, t1/2 = 95 +/- 16 min). Total 13CO2 output [5.7 +/- 1.4 micromol x (kg body weight)(-1)] tended to be in the lower control range. None of the MSUD patients under study exhibited a significant increase in 13CO2 output after load. Maximal increase of label in plasma 4-methyl-2-oxopentanoate, the physiologic precursor of 13CO2, was 16.1 +/- 3.5 MPE in control subjects. In MSUD, label dilution was increased and correlated with the patients' leucine/4-methyl-2-oxopentanoate plasma levels. Considering the generally high variability of 13CO2 output and the unstable substrate pools in MSUD, we discuss the limitations of whole body leucine oxidation measurements by noninvasive approaches.

Adolescent↗

[The anti-inflammatory action of an oral sustained-release theophylline preparation. Results of a clinical pilot study with low dosages].

A pilot study in 14 patients with mild asthma was performed to study the anti-inflammatory efficacy of theophylline (CAS 58-55-9). At the start, during and at the end of a 3 months' treatment with oral sustained release theophylline and during 1 week thereafter, the influence on airway hyperreactivity and ECP (eosinophil cationic protein) serum levels was investigated. Airway responsiveness was expressed as the cumulative provocative dose of methacholine necessary to decrease FEV1 by 20% (PD20-FEV1). Data of 8 patients were suitable for evaluation. At a mean predose serum concentration of 6.5 mg/l, theophylline increased the mean PD20-FEV1 for methacholine from 151 micrograms (at start) to 332 micrograms (at the end of medication), and reduced the mean ECP serum concentration from 34.6 to 24.5 micrograms/l. Up to 1 week after theophylline treatment, the improvement of airway hyperreactivity compared to the baseline was maintained, whereas ECP-serum levels tended to increase. Thus, theophylline markedly attenuated airway hyperreactivity in patients with bronchial asthma at "subtherapeutic" serum theophylline concentrations as well as ECP serum concentrations, suggesting the anti-inflammatory efficacy of theophylline. These observations may have therapeutic implications in the treatment of patients with mild asthma. A slight improvement of lung function and dyspnoea, and a reduction of additional beta 2-bronchodilator use was also observed. Two patients only complained of slight nausea, tremor and restlessness.

Adult↗

Frequency of adult T-cell leukaemia/lymphoma and HTLV-I in Ibadan, Nigeria.

Sera from a small sample of adult blood donors, healthy school children and patients with lymphoma, leukaemia, non-haematologic cancer, congenital and inflammatory disorders from Ibadan, Nigeria were screened for HTLV-I antibody by an enzyme-linked immunoabsorbent assay and confirmed by investigational Western blot. Seventy-nine of 236 positively screened samples could not be tested for confirmation. Seropositive reactivity was observed in nine of 123 blood donors, and 3 of 46 healthy school children but banding patterns on Western blot were often sparse. Among non-Burkitt's non Hodgkin's lymphoma patients six of 30 were HTLV-I positive including four of four with clinical features of adult T-cell leukaemia (ATL). Other clinical conditions had a frequency of positivity indistinguishable from healthy donors. Western blot patterns ranged from strong with multiple bands, which were uncommon, to those with only p24 and p21 envelope positive which were frequent. Given the relative paucity of clinical ATL and the unusual Western blot patterns the true rate of HTLV-I infection may be lower than estimated. It is possible that a cross-reactive HTLV-I-like virus accounts for this pattern.

Adolescent↗

Transcultural nursing care values, beliefs, and practices of American (USA) Gypsies.

This ethnonursing qualitative investigation was focused on the domain of culture care values, expression and meanings of selected American Gypsies. The purpose of the study was to explicate culture care American Gypsy lifeways in order to help nurses understand this largely unknown culture, and to offer guidelines for providing culturally congruent nursing care. Leininger's theory of Culture Care Diversity and Universality was the appropriate theory to use for this study, along with the ethnonursing research method to generate emic and etic grounded data. Findings substantiated that the world view, ethnohistory, religion (moral code), kinship and cultural values, and generic folk practices were powerful influences of Gypsy lifeways and supported culture congruent nursing care. Ethnohistorical facts strongly buttressed the cultural values, norms, and moral codes for culture specific care practices. Several Gypsy culture specific and dominant care meanings, expressions, and actions were confirmed and made credible from raw data and thematic analysis. They were: 1) protective in-group caring; 2) watching over and guarding against Gadje; 3) facilitating care rituals; 4) respecting Gypsy values; 5) alleviating Gadje harassment; 6) remaining suspicious of outsiders; and 7) dealing with purity and impurity moral codes and rules. Culture specific and congruent care generated from Leininger's theory with the three predicted modes were identified to guide nursing decisions and actions.

Attitude to Health↗

Follicular involution in HIV lymphadenopathy. A morphometric study.

Lymph node biopsies from 75 HIV infected patients (71 homo- and bisexual men, 3 hemophiliacs and 1 woman) were studied using immunohistochemical methods with monoclonal antibodies (Mabs) against B lymphocytes, subsets of T lymphocytes, follicular dendritic cells (FDC) and HIV gag proteins p24 and p18. Histopathological changes were classified as follicular hyperplasia (FH), fragmentation (FF), atrophy (FA) and depletion (FD). Immunohistochemical stainings were quantified with the help of an Image Quantifier (IQ) and the reactivity for respective Mab-defined antigen was related quantitatively to other antigens and histopathological changes. Such measurements showed an increase in FDC in biopsies with FH and FF histology and a decrease in FA and FD cases in comparison with cases with non-HIV related lymphadenopathy. In addition it was found that the decrease in FDC was correlated with an increase in CD8+ within the follicles. Double immunostainings for p24 and various cellular markers showed that p24 was predominantly associated with follicular dendritic cells. Essentially the same findings were observed in the lymph nodes irrespective of risk group. Possible mechanisms involved in follicular involution in HIV-related lymphadenopathy are discussed.

AIDS-Related Complex↗

Concomitant infection of HTLV-I and HIV-1: prevalence of IgG and IgM antibodies in Washington, D.C. area.

Serum samples collected from four groups of individuals in the Washington, D.C. area were examined for the presence of IgG and IgM classes of antibody reacting against HTLV-I and HIV-1. These four groups were: (1) healthy adults with negative premarital VDRL test for syphilis (n = 113), (2) miscellaneous common disease patients (n = 155), (3) drug abusers (n = 130), and (4) homosexual men (n = 187). The former two groups are considered to be low-risk groups, and the latter two, high-risk groups. The prevalence of IgG antibody on ELISA/Western blot tests for these groups were respectively: (1) 5.3%/1.8%, (2) 5.2%/1.9%, (3) 13.9%/4.6%, and (4) 4.3%/1.6% for HTLV-I, and (1) 2.7%/0.9%, (2) 4.5%/0%, (3) 12.3%/5.4%, and (4) 8.0%/5.9% for HIV-1. Instances of possible concomitant infection as shown by the presence of antibodies against both HTLV-I and HIV-1 were found only in the latter two high-risk groups, i.e. two (1.5%) in group (3), and three (1.6%) in group (4) as confirmed by both Western blot and immunofluorescence tests. Out of 97 sera collected from drug abusers in 1985-86 which had IgG antibody by Western blot test against HIV-1, 23 (23.7%) were HTLV-I antibody positive by ELISA test (Group 5), and 8 of these were confirmed by Western blot test. Among these 8 persons, IgM antibody against HTLV-I was found in 2, while that against HIV-1 was positive in 7 persons.(ABSTRACT TRUNCATED AT 250 WORDS)

Acquired Immunodeficiency Syndrome↗

Lymphadenopathy in HIV (HTLV-III/LAV) infected subjects: the role of virus and follicular dendritic cells.

Studies of human immunodeficiency virus (HIV) associated lymphadenopathy by histopathology and immunopathology showed conspicuous changes of follicular B-cell areas from a marked hyperplasia to complete involution. Immunohistochemistry showed a corresponding increase in follicular dendritic reticulum cells (FDRC) followed by progressive destruction of these cells during involution, concomitant with invasion of follicles by T-cells. HIV gag antigens were predominantly associated with FDRC in hyperplastic follicles and diminished during involution. Virus replication was by in situ hybridization seen predominantly in follicles, presumably reflecting productive infection of CD4+ cells and/or FDRC. It is concluded that local effects of the virus play an important role in HIV lymphadenopathy. The marked cytopathogenic effects on FDRC indicate that HIV infection with lymphadenopathy represents not only a disease of CD4+ cells but also of follicular antigen presenting cells (FDRC).

AIDS-Related Complex↗

Dose to lung in primary breast irradiation.

Using anatomic data derived from computerized tomography (CT) scans of the torso, the volume of lung irradiated during primary breast treatment has been measured for a variety of irradiation techniques. Two-field tangential plans which are angled into lung to treat also the internal mammary nodes have been compared to three-field plans which include a separate internal mammary field (IMF). The volume of lung achieving high dose (greater than 3000 rad) is similar in both techniques when photons only are used. Electron beam treatment of the IMF is successful in lowering the lung dose. Additional treatment plans that angle the IMF parallel to the tangential fields may offer some theoretical advantage.

Breast Neoplasms↗

Induction of morphological and functional differentiation of human promyelocytic leukemia cells (HL-60) by componuds which induce differentiation of murine leukemia cells.

Various compounds active in promoting in vitro differentiation of certain murine leukemia cell lines (Friend erythroleukemia cells and mouse myeloid leukemia cells) were tested for their capacity to induce differentiation of HL-60 cells, a human promyelocytic leukemia cell line capable of terminally differentiating in vitro to functionally mature granulocytes. Polar planar compounds including hexamethylene bisacetamide (HMBA), certain purines (particularly hypoxanthine), and actinomycin-D induced morphological and functional (as assessed by the capacity to reduce NBT dye) differentiation of HL-60. In contrast, hemin, ouabain, prostaglandin E1, X-irradiation, dexamethasone and some other anti-leukemic chemotherapeutic agents induced little if any significant differentiation of HL-60 cells. These results, together with previous observations with murine leukemia cells, suggest that the human HL-60 cells share common cellular target sites for the inducing action of polar planar compounds, hypoxanthine and actinomycin-D with some murine leukemic cells. In contrast, hemin, ouabain and prostaglandin E1 may be specific for mouse erythroleukemia cells, while X-irradiation and chemotherapeutic agents induce differentiation of both types (erythroid and myeloid) of mouse leukemic cells, but have little effect on HL-60 cells.

Animals↗

[Cytobiopsy as the best present method of intraoperative diagnosis of tumors of the pancreas].

A report has been made over six years experience with a fine needle aspiration biopsy, from authors who have developed a cytological intraoperative quick diagnosis of pancreas tumours. 116 patients have been treated up to this time. This method has proved to be important for the diagnosis of small pancreas tumours, while especially in such cases the indication to pancreas resection is used only in case of malignant tumours. In relation to other normal histological freezer microtome, the cytological method of fine needle biopsy has the advantage of being completely without complication. One can make as many punctures as needed and exactly this is what assues accurate diagnosis. In 90 cases with certain diagnosis, 65 correct conclusions and 12 suspect conclusions of tumour were obtained, and only five cases were erroneously judged negative; in eight cases the poor material did not allow a diagnosis. Therefore, wide application of cytobiopsy can be recommended.

Biopsy, Needle↗