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Biomedical subjects

A Rubinstein

Publications and source records attributed to A Rubinstein.

At least 181 records · Page 10Linked to original sources
PubMed1988

Pediatric acquired immunodeficiency syndrome. Neurologic syndromes.

Central nervous system (CNS) dysfunction was documented in 61 of 68 infants and children with symptomatic human immunodeficiency virus infection. The most frequent manifestations included acquired microcephaly, cognitive deficits, and bilateral pyramidal tract signs. Lymphoma of the CNS, cerebrovascular accidents, and CNS infection caused by conventional pathogens were documented in only ten children (15%). Neurologic deterioration in 11 children was subacute but steadily progressive; in 31 the course was more indolent and began with a plateau. Of these 31 children, 13 had further neurologic deterioration and the conditions of three improved. Seventeen children had a static course with cognitive deficits (seven children) or cognitive plus neurologic impairment (ten children). Neuroradiologic studies in the children with a subacute progressive or plateau course disclosed cerebral atrophy, white matter abnormalities, and calcification of the basal ganglia. Postmortem findings included variable degrees of inflammatory response, multinucleated cells, calcific vasculopathy, and pyramidal tract degeneration. Computed tomographic studies of the children with a static course were normal or showed mild atrophy, but poor brain growth was documented by serial head circumference measurements.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Abnormalities in lipoprotein metabolism in Gaucher type 1 disease.

We have previously described an association between Gaucher type 1 disease and reduced levels of total, low density lipoprotein (LDL), and high density lipoprotein (HDL) cholesterol. Plasma concentrations of apolipoprotein B and apolipoprotein AI were reduced in these subjects, while plasma apolipoprotein E (apoE), which can be synthesized and secreted by macrophages, was increased. To study the pathophysiologic basis for these changes in lipoprotein and apolipoprotein levels, we studied very low density lipoprotein (VLDL), LDL, and HDL metabolism in-depth in four subjects with Gaucher disease. Gel filtration of their plasma revealed that apoE was present in essentially a single population of lipoproteins in the large HDL range. In subject no. 4, studied presplenectomy and post-splenectomy, plasma apoE levels fell after surgery in association with a redistribution of apoE among the plasma lipoproteins to a pattern seen in normal subjects. Determination of the rates of secretion and catabolism of VLDL apoB and triglyceride were within normal limits. The reduced plasma levels of LDL and HDL cholesterol, and of both plasma apoB and apoAI, were associated with increased fractional catabolic rates of these apolipoproteins in LDL and HDL. These results indicate that the hypocholesterolemia present in subjects with Gaucher type 1 disease is associated with increased fractional catabolism of LDL and HDL. These findings, together with the evidence for alternations in plasma apoE metabolism in this disorder, suggest a role for the macrophage as the basis for these abnormalities.

Adult↗
PubMed1988

Improved intestinal cannula for drug delivery studies in the dog.

A modified light-weight intestinal cannula, based on the modified Thomas type (Thomas, 1941; Jones et al., 1971) was fabricated and tested. The design extends the useful life of the cannula and expands the versatility of the canine gastrointestinal (GI) system for pharmaceutical and physiological research. The cannula permits easy administration of pharmaceutical dosage forms directly into or access to ingested substances anywhere along the small intestine.

Animals↗
PubMed1988

Lipids and lipoproteins in new immigrant Ethiopian Jews in Israel.

In an investigation of lipid levels following a period of deprivation and prolonged travel, 206 male and 272 female Ethiopian Jewish immigrants to Israel were evaluated at the end of 1984. This ethnic group, most of whom are lean persons who had apparently consumed a high simple carbohydrate diet, revealed distinct (cross-sectionally evaluated) age and sex patterns of lipid distribution. Total cholesterol in the Ethiopians resembled that in Israeli-born adolescents or adult resident Israelis at ages 8 years through 20-29 years, but mean levels were considerably lower in Ethiopians at ages 30-39 years through 60 years, where they do not exceed 175 mg/dl for males and 190 mg/dl for females. Mean triglyceride levels were higher for Ethiopian females than in Israeli females up to age range 40-49 years. While the levels in Israelis increase steadily with age, no clear age pattern was discernible in the levels in the Ethiopians (at least cross-sectionally). Among the Ethiopian males, levels were considerably higher than those among Israeli males in childhood and adolescence. In Ethiopian adults, the triglyceride levels appeared to be slightly lower than those in resident Israelis. The Ethiopian immigrants displayed markedly reduced high density cholesterol (38 mg/dl for males, 39 mg/dl for females) compared with levels of between 50 and 53 mg/dl for Israelis at age 8-9 years. These differences diminished with age, until, at around age 20-29 years, mean levels became comparable (around 42 mg/dl for males and 50 mg/dl for females) and remained so at older ages. The distinct lipid patterns in the Ethiopian Jewish immigrants to Israel are subject to ongoing follow-up to investigate the effect of a quasi-western diet and a western lifestyle on this ethnic group.

Adolescent↗
PubMed1988

Pulmonary disease in infants and children.

Pulmonary diseases contribute significantly to the morbidity and mortality in children infected with the human immunodeficiency virus. The wide array of lung diseases spans from early acute bacterial infections, to the lymphoid interstitial pneumonitis/pulmonary lymphoid hyperplasia complex, to opportunistic infections. The unique clinical and histopathologic features of these diseases are reviewed.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Acquired circulating anticoagulants in children with acquired immunodeficiency syndrome.

The mechanism underlying the prolonged activated partial thromboplastin time (APTT) seen in some pediatric patients with acquired immunodeficiency syndrome (AIDS) and opportunistic infections was studied. A circulating inhibitor of coagulation was demonstrated in three patients. The inhibitor appears to be an immunoglobulin that interferes with some of the phospolipid-dependent coagulation reactions of the intrinsic pathway. This "AIDS anticoagulant" does not predispose the patient to clinical bleeding despite its ability to cause a marked prolongation of the APTT. As such, careful laboratory diagnosis of the cause of abnormal coagulation test results is necessary for children with AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Thrombocytopenia and human immunodeficiency virus in children.

Thrombocytopenia occurs in 13% of children with symptomatic human immunodeficiency virus (HIV) infection. The clinical and laboratory course of 19 children infected with HIV with thrombocytopenia is described. Bone marrow aspirates showed normal to increased numbers of megakaryocytes. Levels of antiplatelet antibodies were increased in 80% of the children and circulating immune complexes were found in 74%. Clinically significant hemorrhage leading to anemia occurred in five patients, and CNS bleeding led to a fatal outcome in an additional three children. Spontaneous remission of thrombocytopenia occurred in three of the 19 subjects. High-dose IV gamma-globulin was effective in increasing the platelet counts of six of 15 patients (40%) but resulted in a sustained remission in only one subject. Oral prednisone was effective in increasing the platelet count of two thirds of those whose platelet counts could not be controlled by IV gamma-globulin. Bleeding manifestations were eliminated in all patients whose platelet counts increased significantly. Of the 11 children whose counts increased either spontaneously or as a result of therapy, eight remain alive (72%). In contrast, all of the eight patients whose platelet counts did not improve have died. Thrombocytopenia in children with HIV disease is engendered by immune mechanisms and is a major cause of morbidity and mortality. High-dose IV gamma-globulin and/or corticosteroids are temporarily effective in increasing the platelet count and reducing bleeding in about half of thrombocytopenic patients and are recommended for use. The ability to respond to therapy correlates with improved survival.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Live virus vaccines in human immunodeficiency virus-infected children: a retrospective survey.

Live virus vaccines can cause serious adverse reactions when administered to immunocompromised patients. Because children infected with human immunodeficiency virus (HIV) may be immunosuppressed, immunization of these children with live virus vaccines is a potential problem. A retrospective survey was conducted by the New York City Department of Health, with consultation from the Centers for Disease Control, to evaluate the frequency of serious adverse events following receipt of live vaccines among children with HIV infection receiving pediatric care in New York City and New Jersey. Outpatient records of 319 children being cared for by 16 participating physicians were reviewed. Of the 319 charts, 221 (69%) contained vaccination histories. Perinatal transmission of HIV infection was suspected for 208 (94%) of the 221 cases and infection via transfusion for the remaining 13 (6%). Of the 221 for whom immunization histories were available, 180 (81%) had received at least one dose of live oral polio vaccine and 70 (32%) had received measles, mumps, and rubella vaccine. There were 120 children for whom a temporal relationship between immunization and onset of symptoms of immunodeficiency could be seen; 46/120 had received at least one dose of oral polio vaccine and 23/45 had received measles, mumps, and rubella vaccine after onset of symptoms. Although follow-up of this population has been limited, there were no reports of serious adverse events such as typical or atypical measles, paralytic poliomyelitis, or aseptic meningitis in the month following vaccination.(ABSTRACT TRUNCATED AT 250 WORDS)

Acquired Immunodeficiency Syndrome↗
PubMed1987

Thymic hypoplasia associated with isotretinoin embryopathy.

A newborn had isotretinoin embryopathy, including thymic hypoplasia. Evaluation of her immune functions demonstrated progressive attrition of T cells over the nine weeks until her death due to pneumonia. These studies strongly suggest an insult by isotretinoin to the immune system. Immune abnormalities may predispose infants with isotretinoin embryopathy to infection and contribute to their high mortality.

Abnormalities, Drug-Induced↗
PubMed1987

Fetal AIDS syndrome score. Correlation between severity of dysmorphism and age at diagnosis of immunodeficiency.

To objectively evaluate the fetal acquired immunodeficiency syndrome, we have developed a scoring system based on the presence of the characteristic features that we have previously reported. Using this scoring system, 37 children seropositive for the human immunodeficiency virus were classified into three groups: dysmorphologically severely affected (12 children); moderately affected (15 children); and mildly affected (ten children). There was a statistically significant correlation between the severity of the dysmorphic features and both the presence of opportunistic infections within the first year of life and the age at onset of symptoms associated with immune dysfunction, with the more severely stigmatized children manifesting symptoms at a younger age. There was no correlation, however, between severity of the dysmorphic features and presence of opportunistic infections at the time of our examination. We conclude that this scoring system may be useful in presymptomatic identification of severely dysmorphic human immunodeficiency virus-infected infants.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Gastric emptying of nondigestible solids in the fasted dog.

Gastric emptying of nondigestible solid particles was studied in the fasted dog. Particles of varying sizes (0.5-6.4 mm), density (0.5-2.9 gm/cm3), and surface characteristics were coadministered orally with 50 mL of saline and collected from a permanent duodenal cannula implanted approximately 15 cm from the gastroduodenal junction. The phase of the motility pattern was ascertained by the appearance of bile, which occurs during phase II, as well as by mucus discharge, which stops at the onset of phase I. A lag phase, due to the 'quiet' phase I, was observed in the gastric emptying of coadministered saline. This is in contrast with gastric processing of large volumes (i.e., greater than 200 mL) which can usually be approximated by first-order discharge. Most coadministered saline was discharged before the solid particles. The pH of the duodenal effluent was elevated approximately 1 pH unit during mucus discharge and its pH can be as high as 8.3. In the fasted dog, gastric emptying of nondigestible particles closely followed the gastric motility patterns. The onset of discharge of the particles correlated with the late phase II and the phase III activity. Except for a few cases, which took two migrating motor complexes (MMC), greater than 90% of administered particles was discharged from the stomach after one MMC. The discharged particles were entrapped within mucous plugs. In the fasted state, gastric emptying of nondigestible particles appeared to be independent of size, density, and surface characteristics. Mucus seems to play a significant role in the distribution and discharge of the administered particles.

Animals↗
PubMed1987

Effect of two approaches to auditory training on speech recognition by hearing-impaired adults.

Twenty adults with mild to moderate sensorineural hearing impairments were given three tests of speech recognition: the CUNY Nonsense Syllable Test (NST), the low predictability items of the Revised Speech Perception in Noise (RSPIN) test, and the high predictability items of the RSPIN test. They were tested on four occasions: at the beginning of the study, after one month of "no treatment," after a month of intensive auditory training, and after a further month of "no treatment." During the treatment period, 10 of the subjects spent all of the time on activities involving sentence perception and perceptual strategy while the other 10 spent half of the time on activities involving consonant recognition. A small, but statistically significant increase in speech recognition performance on the high probability material was observed in both groups subsequent to training, but the effect of training method was not significant. In addition, the gains achieved were not lost in the month following the end of training. The findings suggest that the benefits of auditory training were found in an increased use of sentence context as an aid to word recognition.

Aged↗