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Biomedical subjects

A Rubinstein

Publications and source records attributed to A Rubinstein.

At least 199 records · Page 11Linked to original sources

Developmental abnormalities in children with acquired immunodeficiency syndrome (AIDS): a follow-up study.

Developmental abnormalities in 16 pediatric patients with AIDS or AIDS-Related Complex (ARC) were previously described. Neurological deterioration was in evidence on follow-up in 9 of the children, 5 died since the original assessments were performed. Ten patients were reevaluated 14 months later by cognitive testing. Two showed greater progress than expected on the basis of earlier test results; 6 showed the expected level of developmental progress; and the remaining 2 showed regression in cognitive functioning. All patients who exhibited regression in their developmental course showed deterioration in their neurological examinations. Developmental progression was noted in some children who on follow-up serial examinations exhibited a clinically deteriorating neurological picture. Pediatric AIDS patients manifest variable neurodevelopmental courses. As a result, rehabilitative intervention services must be tailored to meet individual needs.

AIDS-Related Complex↗

Characterization of cognitive functioning in a subgroup of children with congenital HIV infection.

The cognitive status of 12 clinically stable children with congenital HIV infection, nine of whom were neurologically impaired, age three to nine years, was assessed using the Kaufman ABC test. Seven of the children had ARC; five were diagnosed as having AIDS. The same children were evaluated by standard neurologic examinations with Characterization of tone and fine motor functioning. Two were diagnosed as being mildly retarded; six were borderline; and four tested as being of average intelligence. Visual-spatial perceptual based functioning was found to be more impaired than were abstract reasoning and verbally mediated skills in six (50%) of the patients. This pattern of impaired information processing was found irrespective of overall cognitive status. On neurological and physiatric examination abnormal developmental histories were obtained, or poor fine motor coordination, abnormal tone and gait, and impaired rapidly alternating movements were found in 9 of the 12 subjects. These findings suggest selective impairment in distinct areas of neurologic and neuropsychological functioning during stable phases of HIV infection in a select group of children. These patterns appear to persist over time. They differ from the clustering of impaired skills seen in children of comparable socio-cultural backgrounds without HIV infection. Similarities in functioning are noted between this subgroup of children with AIDS and ARC and comparable groups with cerebral palsy.

Journal Article↗

Visceral leishmaniasis presenting as fever of unknown origin.

A 41-year-old male presented with a prolonged febrile disease with marked splenomegaly. Bone marrow and liver biopsy smears and cultures for Leishmania were repeatedly negative and there was no hyperglobulinemia. Leishmania parasites were ultimately demonstrated only in the spleen at laparotomy, performed because of severe pancytopenia. The epidemiology of leishmaniasis in Israel is reviewed.

Adult↗

In vitro metabolism of apolipoprotein E.

Apolipoprotein E plays a major role in the uptake of chylomicrons and of very-low-density lipoprotein (VLDL) remnants by the liver. It has also been clearly demonstrated that apolipoprotein E rapidly and spontaneously exchanges between lipoproteins. To assess whether all lipoprotein-bound apolipoprotein E is available to participate in spontaneous transfer and/or exchange, the present study followed the fate of radiolabeled apolipoprotein E in an in vitro system. The results show that in vitro, apolipoprotein E can be considered as having both a spontaneously exchangeable pool and a nonexchangeable pool. Based upon specific radioactivity data, only a limited amount of apolipoprotein E originating in VLDL or in high-density lipoproteins (HDL) was capable of in vitro exchange with that in other lipoprotein fractions. Lipolysis of VLDL triacylglycerol by milk lipoprotein lipase, however, resulted in complete transfer of VLDL apolipoprotein E mass and radioactivity to HDL, supporting the potential for transformation of exchangeable apolipoprotein to a transferable pool in vivo. The results of these studies indicate that during the course of lipoprotein metabolism, conformational changes occur which alter the accessibility of apolipoprotein E. Such dynamic heterogeneity may have implications for the regulation of lipoprotein metabolism.

Apolipoproteins E↗

Restoration of suppressor T-cell functions in children with AIDS following intravenous gamma globulin treatment.

Suppressor T-cell function was analyzed in seven children with acquired autoimmunodeficiency syndrome (AIDS) or AIDS-related complex (ARC). Four of the patients had markedly increased serum IgG levels. All patients had elevated percentages and absolute numbers of peripheral blood T8 cells. In vitro concanavalin A generation of suppressor cells for T-cell mitogenic responses and suppression of pokeweed mitogen-driven immunoglobulin secretion were diminished in all patients. After intravenous treatment with gamma globulin, four patients regained in vitro suppression of pokeweed mitogen-driven gamma globulin secretion. Treatment with intravenous gamma globulin also modified in vitro suppressor T-cell functions in children with AIDS or ARC.

Acquired Immunodeficiency Syndrome↗

Human T-cell lymphotropic virus type III (HTLV-III) embryopathy. A new dysmorphic syndrome associated with intrauterine HTLV-III infection.

Twenty infants and children with positive serologic tests for the human T-cell lymphotropic virus type III (HTLV-III) were noted to have similar features including growth failure (75%), microcephaly (70%), and craniofacial abnormalities consisting of ocular hypertelorism (50%); prominent box-like appearance of the forehead (75%); flat nasal bridge (70%); mild upward or downward obliquity of the eyes (65%); long palpebral fissures with blue sclerae (60%); short nose with flattened columella and well-formed, triangular philtrum (65%); and patulous lips (60%). These features constitute a new and distinct dysmorphic syndrome, the HTLV-III embryopathy.

Acquired Immunodeficiency Syndrome↗

Computerized model for evaluating the kinetics of in vitro release of valpromide from controlled-release tablets under nonsink conditions.

A general mathematical model was developed to describe a dissolution system for tablets that have undergone attrition and maintained their geometric shape as concentration changed, i.e., starting with sink and ending with nonsink conditions. A computer program, designed for the microcomputer, was used to test the goodness of fit of the experimental data to the theoretical data by the chi 2 test. This program is more significant than a previous published program that relates to the more specific kinetic analysis of multidispersed powders undergoing dissolution under sink conditions. Concentration data obtained after dissolution tests of valpromide (2-propylvaleramide) controlled-release tablets performed under changing concentration conditions were checked in the two computerized models, which showed a better fit to the general model.

Biotransformation↗

Hyperlipidemias and rheumatic manifestations.

There is an increased incidence of arthritides and tendinitis in patients with hyperlipidemias, especially types II and IV. The mechanism responsible for these manifestations is at present not known. It is important to recognize this association and to test plasma lipid levels in any case of arthritis which does not fit into one of the well-established diagnostic entities.

Arthritis↗

Pediatric AIDS.

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Acquired Immunodeficiency Syndrome↗

Isolation of apolipoprotein E-containing lipoproteins by immunoaffinity chromatography.

These data suggest that subfractionation of lipoproteins by immunoaffinity chromatography according to apolipoprotein content provides a valuable method for physical isolation of intact lipoprotein subclasses. While this study has focused specifically on apoE-containing lipoproteins, the technique of immunoaffinity isolation clearly has wider application. It must be emphasized that antibodies differ with regard to affinity and avidity, and that both binding and elution conditions may need to be adjusted for each group of antibodies. Maintenance of physical integrity of lipoproteins thus isolated must also be evaluated independently for each antibody. Nevertheless, immunoaffinity isolation of apolipoprotein-specific subclasses offers a powerful tool for preparing lipoproteins which should reflect physical and/or metabolic properties conferred by the apolipoprotein of interest.

Antigen-Antibody Complex↗

The epidemiology of pediatric acquired immunodeficiency syndrome.

HTLV III infection of children exhibits a relatively narrow spectrum in which the majority of patients develop clinical manifestations before the age of 2 years. In most instances the disease is transmitted in utero, whereby 35-65% of HTLV III-positive women give birth to an infected child. Discordant infection in twins is described. In fewer cases the disease is acquired through blood transfusions. In exceptional cases transmission via sexual abuse or use of needles may occur in young children. We have no evidence of intrafamilial horizontal disease transmission from child to child. The number of children with the disease may increase sharply. Around 2000 pregnancies in HTLV III-infected women are projected in New York City in the next year. Should all these pregnancies be completed, several hundred additional cases of pediatric AIDS may be expected in 1986.

Acquired Immunodeficiency Syndrome↗

Circulating thymulin and thymosin-alpha 1 activity in pediatric acquired immune deficiency syndrome: in vivo and in vitro studies.

Twenty-five children with acquired immune deficiency syndrome (AIDS) or AIDS-related complex had a characteristic pattern of T cell deficiency. Abnormally low plasma thymulin levels preceded the development of peripheral blood T cell abnormalities. In contrast to patients with congenital T cell deficiencies, our patients had elevated serum levels of thymosin-alpha 1. Treatment with thymosin fraction 5 in three children with AIDS resulted in only transient clinical and immunologic improvement.

Acquired Immunodeficiency Syndrome↗

Pulmonary disease in children with acquired immune deficiency syndrome and AIDS-related complex.

Two major pulmonary diseases were defined on the basis of lung biopsies in 15 children with acquired immune deficiency syndrome (AIDS) or AIDS-related complex. Pneumocystis carinii pneumonia was observed in eight children, and pulmonary lymphoid hyperplasia in six. One child had nonspecific interstitial pneumonitis. Children with P. carinii pneumonia had more severe hypoxemia, with higher alveolar-arterial oxygen gradients, and higher isomorphic elevations of serum lactate dehydrogenase. Clinically, children with pulmonary lymphoid hyperplasia were older, and had digital clubbing, parotid gland enlargement, and elevated serum IgG levels. Results of serologic assays and lung tissue analysis were suggestive of persistent Epstein-Barr virus infection exclusively in patients with pulmonary lymphoid hyperplasia. Recognition of the clinical and laboratory findings characteristic of each entity may assist in the differential diagnosis without the need of surgical biopsy.

Acquired Immunodeficiency Syndrome↗

Candida retinitis in bare lymphocyte syndrome.

Bare lymphocyte syndrome (BLS) is a rare, severe combined immunodeficiency characterized by lack of expression of HLA A, B and C antigens and the absence of B2 microglobulins. Patients with BLS exhibit functional deficiency of both T and B cells resulting in bacterial as well as viral and fungal infection. Ophthalmic findings in this group of disorders have not been reported. We present a case of candida retinitis in a terminally ill 5-year-old girl with BLS.

Amphotericin B↗

Apolipoprotein B metabolism in subjects with deficiency of apolipoproteins CIII and AI. Evidence that apolipoprotein CIII inhibits catabolism of triglyceride-rich lipoproteins by lipoprotein lipase in vivo.

Previous data suggest that apolipoprotein (apo) CIII may inhibit both triglyceride hydrolysis by lipoprotein lipase (LPL) and apo E-mediated uptake of triglyceride-rich lipoproteins by the liver. We studied apo B metabolism in very low density (VLDL), intermediate density (IDL), and low density lipoproteins (LDL) in two sisters with apo CIII-apo AI deficiency. The subjects had reduced levels of VLDL triglyceride, normal LDL cholesterol, and near absence of high density lipoprotein (HDL) cholesterol. Compartmental analysis of the kinetics of apo B metabolism after injection of 125I-VLDL and 131I-LDL revealed fractional catabolic rates (FCR) for VLDL apo B that were six to seven times faster than normal. Simultaneous injection of [3H]glycerol demonstrated rapid catabolism of VLDL triglyceride. VLDL apo B was rapidly and efficiently converted to IDL and LDL. The FCR for LDL apo B was normal. In vitro experiments indicated that, although sera from the apo CIII-apo-AI deficient patients were able to normally activate purified LPL, increasing volumes of these sera did not result in the progressive inhibition of LPL activity demonstrable with normal sera. Addition of purified apo CIII to the deficient sera resulted in 20-50% reductions in maximal LPL activity compared with levels of activity attained with the same volumes of the native, deficient sera. These in vitro studies, together with the in vivo results, indicate that in normal subjects apo CIII can inhibit the catabolism of triglyceride-rich lipoproteins by lipoprotein lipase.

Adult↗