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Biomedical subjects

G Pearl

Publications and source records attributed to G Pearl.

7 recordsLinked to original sources
PubMed2001

Profiling the membership of self-help groups for aphasic people.

We surveyed the membership of aphasia self-help groups in England. We wanted to know what kinds of people become members and the factors associated with taking an active role. So we aimed to describe the membership in terms of age, aphasia severity, physical state, socio-economic status and group roles. We collected data on 135 members and found them relatively less severe but mainly chronically aphasic, living in their own homes and close to the group meeting place, using their own or public transport, with little community or state aid. While hemiplegia was common, few were wheelchair bound. Those taking active roles were younger and less severely aphasic and from more professional and managerial socio-economic groups.

Adult↗
PubMed2001

Intussusception due to Yersinia enterocolitica enterocolitis in a patient with beta-thalassemia.

Patients who are homozygous for thalassemia major are at risk for Yersinia enterocolitica infections. We present a case of a 4-year-old child with intussusception of the terminal ileum whose past medical history was significant for beta-thalassemia. His monthly blood transfusions for this condition may have put him at risk for Y enterocolitica enterocolitis. The pathogenesis of this disease relates to the role of iron as an essential growth factor for Yersinia, and this patient's transfusions left him in an iron-overloaded state, despite treatment with Desferal. Our patient's unusual presentation of intussusception was secondary to the mass effect caused by lymphoid hyperplasia, specifically hypertrophied Peyer's patches in the ileum caused by Y enterocolitica infection. To our knowledge, this is the first such case of intussusception caused by Yersinia to be reported.

Child, Preschool↗
PubMed1999

Sensitivity and specificity of three clinical criteria for dementia with Lewy bodies in an autopsy-verified sample.

OBJECTIVE: To evaluate the sensitivity and specificity of the clinical features of three published diagnostic criteria for diffuse Lewy body disease (DLBD) using autopsy-confirmed Alzheimer's (AD), DLBD and AD+DLBD (mixed) dementia cases. DESIGN: Retrospective chart review of an autopsy series of 56 patients selected from the State of Florida Brain Bank on the basis of a pathological diagnosis of either pure AD, DLBD (pure and common forms) or AD+DLBD (mixed) dementia. Clinical features were assessed by three raters blind to the pathological diagnosis. RESULTS: The existing criteria for a clinical diagnosis of DLBD were highly specific (90-100%) but not very sensitive (49-63%) in the differential diagnosis of DLBD versus AD; sensitivity did improve (61-74%) when mixed AD+DLBD cases were eliminated. Clinical features that occur more frequently in DLBD than in AD were unspecified hallucinations, unspecified EPS, fluctuating course and rapid progression. Post-hoc analysis also indicated that hallucinations and EPS were more common early in the disease course of DLBD than in AD. Empirically derived criteria, formulated using the most prevalent clinical features, demonstrated sensitivity values of 57-96% for pure forms and 43-91% for mixed forms. CONCLUSIONS: This study demonstrated that additional improvements in the established criteria for DLBD are needed. Our empirically derived criteria enhanced the distinction of DLBD from AD while allowing the clinician the choice of maximizing sensitivity with acceptable specificity, and vice versa.

Aged↗
PubMed1995

Embryonic expression of the chicken Sox2, Sox3 and Sox11 genes suggests an interactive role in neuronal development.

Three chicken Sox (SRY-like box) genes have been identified that show an interactive pattern of expression in the developing embryonic nervous system. cSox2 and cSox3 code for related proteins and both are predominantly expressed in the immature neural epithelium of the entire CNS of HH stage 10 to 34 embryos. cSox11 is related to cSox2 and cSox3 only by virtue of containing an SRY-like HMG-box sequence but shows extensive homology with Sox-4 at its C-terminus. cSox11 is expressed in the neural epithelium but is transiently upregulated in maturing neurons after they leave the neural epithelium. These patterns of expression suggest that Sox genes play a role in neural development and that the developmental programme from immature to mature neurons may involve switching of Sox gene expression. cSox11 also exhibits a lineage restricted pattern of expression in the peripheral nervous system.

Amino Acid Sequence↗
PubMed1991

A case of glutaric acidemia type II (severe multiple acyl-CoA dehydrogenation disorder) with subsequent prenatal exclusion in a sibling.

A case of severe multiple acyl-CoA dehydrogenation disorder is described. This is the second such case reported to have had an elevated maternal serum alpha-fetoprotein and normal amniotic alpha-fetoprotein. The child's 3-day extrauterine life was characterized by intractable acidosis, respiratory distress, and ventricular fibrillation. The characteristic biochemical, morphologic, and microscopic findings of this condition are reviewed. A subsequent pregnancy was evaluated using chorionic villus sampling and analysis of cultured trophoblasts. The trophoblasts were biochemically normal, and a normal child was subsequently delivered. Since the manifestations of this disorder developed in utero, prenatal diagnosis and therapy offer the only hope for a more prolonged survival.

Acidosis↗
PubMed1988

Local effect of neurotensin on canine ileal blood flow, and its release by luminal lipid.

This study was performed to determine whether the level of neurotensin in mesenteric venous blood after lipid perfusion is sufficient to establish neurotensin as a mediator of lipid-induced mesenteric vasodilation. In anesthetized dogs, arterial flow to segments of the ileum was recorded, and blood was collected for measurement of neurotensin-like immunoreactivity, and neurotensin and metabolites. Perfusion of the lumen with micellar lipid resulted in an increase in blood blow from 37.7 +/- 4.1 to 44.5 +/- 3.9 ml/min/100 g (p less than 0.01; n = 8); flow to a control segment did not change. Venous plasma neurotensin-like immunoreactivity doubled, and neurotensin also increased (to 11.3 +/- 3.9 fmol/ml; p less than 0.05). Close intra-arterial infusion of neurotensin at 5 pmol/min increased blood flow to 44.3 +/- 3.4 ml/min/100 g (p less than 0.025; n = 5); flow to a control segment did not change. Neurotensin-like immunoreactivity increased to the same extent as with lipid perfusion, and neurotensin increased to 28.6 +/- 6.1 fmol/ml (p less than 0.05). No accumulation of metabolites was detected in either experiment. Thus, infused neurotensin caused increased ileal blood flow at a level in venous plasma comparable to that present after lipid perfusion, suggesting that neurotensin may have a role in the local regulation of ileal blood flow.

Animals↗