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Biomedical subjects

A Thompson

Publications and source records attributed to A Thompson.

At least 235 records · Page 13Linked to original sources
PubMed1989

Evidence for male X chromosomal mosaicism in X-linked agammaglobulinemia.

X-Linked agammaglobulinemia (XLA) is a severe antibody deficiency disease in man, resulting from an arrest in differentiation of pre-B cells. XLA is recessive: female carriers do not exhibit antibody deficiency, but manifest an exclusive inactivation of the XLA-carrying X chromosome in all peripheral blood B lymphocytes. An exclusive inactivation of the paternal X chromosome in the B lymphocytes of all daughters of a male who had no agammaglobulinemia demonstrated that the XLA defect can originate from healthy males. These males are X chromosomal mosaics. X-Chromosomal RFLP segregation analyses in other XLA pedigrees suggest a frequent introduction of XLA by healthy males. This implies that XLA often originates from mitotic errors, either at postmeiotic or early postzygotic stages.

Agammaglobulinemia↗
PubMed1989

Why is the lower torso protected in traumatic asphyxia? A new hypothesis.

Traumatic asphyxia secondary to a crush injury of the chest is characterized by craniocervical cyanosis, subconjunctival hemorrhage, and severe vascular engorgement of the head and neck. These signs are believed to be due to high venous pressures causing stasis and capillary rupture. A "fear response" that produces a strong Valsalva maneuver is thought to be necessary for their development. The lower torso seems to be protected, and previously this was thought to be due to its superior system of valves. We present here ultrasonographic evidence that the inferior vena cava is compressed or obliterated during a Valsalva maneuver, and propose that this compression protects the lower torso during traumatic asphyxia.

Abdomen↗
PubMed1989

Mechanisms of hypertension during and after orthotopic liver transplantation in children.

The aim of this study was to assess the hormonal alterations that may mediate the systemic hypertension that develops in patients during the perioperative period of orthotopic liver transplantation. We studied nine pediatric patients without previous hypertension or renal disease during six time points, starting before transplantation and ending at 48 hours after surgery. Hypertension developed in all patients in association with central venous pressures less than 10 mm Hg. Free water clearance was negative in all nine patients. Vasopressin levels increased intraoperatively but fell as hypertension developed. Atrial natriuretic factor levels increased as systemic blood pressure rose. A high level of plasma renin activity was observed in four patients with renal insufficiency. In six patients, postoperative 24-hour urinary norepinephrine excretion was within the normal age-adjusted range. These findings suggest that the combination of cyclosporine, corticosteroids, and, in some patients, an elevated plasma renin activity prevents the kidney from responding to the acute volume and salt overload with an appropriate diuresis and natriuresis, thus leading to systemic hypertension. The treatment of hypertension after liver transplantation may include salt restriction, diuretics, and, in those patients with a low creatinine excretion index, angiotensin coverting enzyme inhibitors.

Adolescent↗
PubMed1989

Amrinone in neonates and infants after cardiac surgery.

Eighteen critically ill postoperative patients less than 1 yr of age were studied to determine the pharmacokinetics and adverse effects of amrinone. All patients had undergone cardiopulmonary bypass for repair of congenital heart lesions. Plasma samples were obtained every 12 h while patients were receiving amrinone to determine when steady state was achieved; samples were also obtained within 24 h after amrinone had been discontinued. Elimination half-life (T1/2), clearance, and volume of distribution were calculated from plasma amrinone concentrations, and the incidence of platelet transfusion was monitored. T1/2(22.2 vs. 6.8 h) and clearance (1.1 vs. 2.6 ml/min.kg), but not the volume of distribution (1.8 vs. 1.6 L/kg), differed significantly in patients less than 4 wk of age in comparison to patients greater than 4 wk of age. A negative correlation between T1/2 and age (r = -.79) was observed. Platelets were administered no more frequently in study patients than in a similar group that did not receive amrinone. To achieve the plasma concentration of amrinone that is therapeutic in adults, current dosage recommendations are inadequate in neonates and infants. Infants should receive an initial iv amrinone bolus of 3.0 to 4.5 mg/kg in divided doses followed by a continuous infusion of 10 micrograms/kg.min, while neonates should receive a similar bolus followed by a continuous infusion of 3 to 5 micrograms/kg.min.

Amrinone↗
PubMed1988

Serum cholesterol and coronary heart disease: Auckland general practitioners' attitudes and practices in 1986.

The relationship between elevated serum cholesterol and coronary heart disease, is now generally accepted as being causal. To examine current attitudes and practices regarding the treatment of high serum cholesterol, questionnaires were sent to a randomly selected sample of general practitioners in the Takapuna health district during 1986. The response rate among the 92 doctors in general practice at the time of the study was 80%. The majority of general practitioners (82.5%) believed that there was a casual relationship between high serum cholesterol and coronary heart disease and that reducing levels would help prevent coronary heart disease. Almost all general practitioners (96%) were screening some groups of patients for high serum cholesterol, with most screening those with symptomatic coronary heart disease or associated risk factors, and 15% screening all patients. Although almost 90% of general practitioners had patients on diet therapy and one third had patients on drug treatment, there was wide variation in attitudes regarding the serum cholesterol levels meriting dietary or drug treatment. This suggests that there is still considerable confusion as to when and how to treat high cholesterol levels and that specific national guidelines for the detection and management of high serum cholesterol are required as part of a comprehensive programme to prevent coronary heart disease.

Adult↗
PubMed1988

Two distinct deficits of visual tracking caused by unilateral lesions of cerebral cortex in humans.

We studied horizontal visual tracking in 20 patients with unilateral cerebral lesions and in 10 age-matched control subjects. Five patients, all with posterior lesions, showed impaired smooth pursuit of predictable targets moving toward the side of the cerebral lesion. Using nonpredictable step-ramp stimuli, we identified two distinct deficits of visual tracking. The first was a unidirectional deficit of smooth pursuit, for targets moving toward the side of the lesion, in response to stimuli presented into either visual hemifield. The second deficit, identified in a sixth patient who did not show pursuit asymmetry to predictable targets, was a bidirectional inability to estimate the speed of a moving target in the visual hemifield contralateral to the side of the lesion; this caused inaccurate saccades to moving (but not stationary) targets and impaired smooth pursuit initiation. These visual tracking deficits were independent of homonymous hemianopia or hemispatial neglect. These two tracking deficits are similar to those described in rhesus monkeys with lesions of the medial superior temporal and middle temporal visual areas.

Adolescent↗
PubMed1988

Bell's palsy and HIV infection.

Unilateral infranuclear facial palsy developed in three young homosexual men. All three were positive for antibodies to human immunodeficiency virus (HIV). Two had persistent generalised lymphadenopathy, but the clinical criteria for the acquired immune deficiency syndrome (AIDS) were not fulfilled. There were no features of generalised neuropathy, and no other cause for facial palsy was evident. Recovery was excellent in each patient.

AIDS-Related Complex↗
PubMed1988

Antisera to human IgE can also recognize IgA and IgM molecules as produced by selected B lymphoblastoid cell lines.

A series of polyclonal and monoclonal anti-human IgE sera reacted with a panel of 11 selected IgA and IgM producing B lymphoblastoid cell lines (BLCLs) in cytoplasmic fluorescence, ELISA and immunoprecipitation assays. The reactions were discordant between the BLCLs and the individual antisera. The panel of 11 BLCLs was selected from over 500 BLCLs by an ELISA spot assay using a polyclonal anti-human IgE serum. The BLCLs produced exclusively IgM or IgA, both with either kappa or lambda light chains as shown by immunoprecipitation/PAGE and mRNA analyses. The data indicate that IgE antisera may recognize IgE-like epitopes on the variable parts of some IgM or IgA molecules. These reactivities of IgE antisera may lead to false positive results of serum IgE determinations. Selected BLCLs are useful targets for exposing such reactivities in IgE antisera.

B-Lymphocytes↗
PubMed1988

Extracorporeal membrane oxygenation in children. New trends.

At the Children's Hospital of Pittsburgh the extracorporeal membrane oxygenation program was started in 1980. The results of our experience from 1980 to 1985 were previously reported. In the past 2 years 39 additional newborn infants have been treated with this modality, with an overall survival rate of 79% (31/39). This survival rate is much better than that obtained in 33 neonates who had been treated in the previous 5 years (54%; p less than 0.05). A new aspect of our extracorporeal membrane oxygenation program is the use of total apneic lung rest for persisting pulmonary interstitial emphysema during support with the oxygenator. Six neonates were treated with this technique because of worsening pulmonary interstitial emphysema during extracorporeal circulation. Five of them survived. Another indication for extracorporeal membrane oxygenation in our pediatric population has been left ventricular or biventricular failure after cardiopulmonary bypass. Four of our seven patients treated for this indication are long-term survivors. At present, because of the impossibility of using other forms of left ventricular assist devices in the pediatric population, it seems that extracorporeal membrane oxygenation is the most effective treatment for left ventricular failure after cardiopulmonary bypass. From our experience, even in the absence of long-term follow-up of patients supported with extracorporeal membrane oxygenation, it appears that the benefits of this therapeutic modality far exceed the risks in the high-risk population for which it is being used.

Cardiopulmonary Bypass↗
PubMed1987

X-linked immunodeficiency with hyperimmunoglobulinemia M appears to be linked to the DXS42 restriction fragment length polymorphism locus.

The gene involved in X-linked immunodeficiency with hyperimmunoglobulinemia M (XHM) was localized by the use of nine restriction fragment length polymorphic (RFLP) markers covering the entire X chromosome. Multipoint linkage analysis of RFLP data obtained in a three generation XHM pedigree indicates the Xq24-q27 area around the DXS42 RFLP locus as the most likely localization of the XHM locus.

Female↗
PubMed1987

Diffuse Lewy body disease. Neuropathological and biochemical studies of six patients.

Post-mortem pathological and biochemical studies are reported on six patients with progressive dementia. The characteristic pathological finding was neurofilament-containing cytoplasmic inclusions in cortical and subcortical neurons. The clinical and pathological findings were consistent with so-called diffuse Lewy body disease. The patients had variable changes of the Alzheimer type, with five of six patients displaying "plaques only" Alzheimer's changes. Biochemical studies showed profound decreases in neocortical choline acetyltransferase (ChAT) activities that correlated with marked neuronal loss in the basal nucleus of Meynert. ChAT activities were normal in the hippocampus in three patients who also had no significant Alzheimer type hippocampal changes. All patients had decreased cortical somatostatin-like immunoreactivity. Our observations suggest that dementia in diffuse Lewy body disease bears biochemical similarities to Alzheimer's disease, in that biochemical markers for both intrinsic cortical neurons and ascending cholinergic neurons are affected.

Aged↗