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J Lister

Publications and source records attributed to J Lister.

At least 73 records · Page 4Linked to original sources

Development profile of neuron-specific enolase in human gut and its implications in Hirschsprung's disease.

The most widely held view on the pathogenesis of Hirschsprung's disease as an arrest of neuroblast migration in the gut was based on the hypothesis of a single craniocaudal gradient of development of enteric neurons. Recent experimental studies in animals, however, have revived a contradictory hypothesis of a dual gradient of neuronal development; such data are not available in humans. To test these hypotheses in humans, we studied the pylorus, ileum, and colon of 28 fetuses with gestational ages of 9-21 wk, using immunohistochemical localization of neuron-specific enolase, a specific neuronal marker indicative of differentiation. Development of the enteric nervous system was shown to be most advanced in the pylorus, less so in the colon, and least so in the ileum. The findings support the hypothesis of a dual gradient of neuronal development proceeding from both ends to the middle of the gut in midtrimester human fetuses and suggest that the pathogenesis of Hirschsprung's disease needs to be reconsidered.

Colon↗

Maldevelopment of spinal cord--substance P in spina bifida.

Recent works have shown that the neuropeptide, substance P, is an important element of the central nervous system, functioning as a neurotransmitter as well as moderating neural development. In a previous communication, we provided indirect evidence that substance P played a role in the pathogenesis and pathophysiology of spina bifida by showing an elevation of CSF substance P concentrations in affected foetuses and babies. In this study, we investigated directly the occurrence of substance P in spinal cord tissues in spina bifida and compared it to that found during normal development. Using radioimmunoassay, concentrations of substance P were measured in the spinal cords of 20 normal human foetuses (aged 9-21 weeks) and 4 foetuses with spina bifida (aged 17-19 weeks). Substance P was detected in substantial amounts in the normal foetal spinal cord as early as 9 weeks' gestation, and there was progressive accumulation with age: mean concentrations +/- S.E.M. in 3 subgroups were 56.3 +/- 4.9 pmol g-1 (9-12 weeks), 59.5 +/- 7.2 pmol g-1 (13-16 weeks) and 117.3 +/- 7.2 pmol g-1 (17-21 weeks). In spina bifida, substance P was severely depleted in the dysraphic segment of the cord (mean concentration +/- S.E.M. = 22.1 +/- 2.3 pmol g-1) and moderately so in the segment above the dysraphism (mean concentration +/- S.E.M. = 47.2 +/- 4.2 pmol g-1).(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗

Substance P concentrations in human cerebrospinal fluid vary inversely with age.

The mean concentrations of immunoreactive substance P in cerebrospinal fluid (CSF) of 10 fetuses (gestational age 11-20 weeks) were 22.7 +/- 8.3 pmol/ml, compared with 250.0 +/- 28.2 fmol/ml in premature babies (gestational age 25-31.5 weeks, n = 8), 141.0 +/- 14.2 fmol/ml in full term newborn babies (n = 5), 50.0 +/- 2.3 fmol/ml in children (age 1-6 years, n = 6), and 9.5 +/- 1.5 fmol/ml in 5 adults. The differences between successive age groups were all statistically significant. The high concentration of substance P in human CSF in the early stages of development and its continuous decline towards maturity encourages the idea that substance P plays a role in neuro-development.

Adult↗

Deficiency of a major myeloid antigen in neutrophils of patients with refractory anemia and excess myeloblasts.

The expression of a major granulocyte-related antigen was assessed on the peripheral blood neutrophils of patients with the syndrome of refractory anemia with excess blasts. Of thirteen patients studied six showed a marked decrease in the number of cells with detectable staining. These results indicate that, although the neutrophils may appear normal by standard morphological criteria in this disease, they may be defective in a major component associated with myeloid maturation.

Anemia, Aplastic↗

Acute pancreatitis in children.

Twenty-nine children with acute pancreatitis were managed during the period 1971 to 1983. Aetiology included trauma (5), mumps (5), drug therapy (4), biliary disease (1), and cystic fibrosis (1); 13 cases were classified as idiopathic. Diagnosis could be difficult, and unnecessary laparotomies were performed in 7 instances for suspected appendicitis. One patient, however, had a well-justified laparotomy revealing coexisting severe appendicitis and pancreatitis. Morbidity included relapses (7), pseudocysts (3), obstructive duodenal hematoma (1), and miscellaneous problems (4). Improvements in management included endoscopic retrograde cholangio-pancreatography (ERCP) to exclude anatomical anomalies in relapsing cases, ultrasonography for the diagnosis of pseudocysts and for follow-up measurements in two such cases successfully managed conservatively, and increasing use of total parenteral nutrition in cases with protracted disease or serious complications.

Acute Disease↗

Fluctuating islet-cell autoimmunity in unaffected relatives of patients with insulin-dependent diabetes.

Complement-fixing islet-cell antibodies (CF-ICA) were found in 20 out of 685 unaffected first-degree relatives of children with type 1 diabetes. During a 5-year follow-up, 7 of the 20 became diabetic, 1 continued to show the antibodies without any abnormality of glucose tolerance, and 12 subjects lost them without the disease developing. Although CF-ICA are useful as a marker of active insulitis they should not at present be used to define subjects who might benefit from preventive immunosuppression.

Antibodies↗

Femoral hernia in children.

During a period of ten years, 20 children with femoral hernias were treated at Alder Hey Children's Hospital, Liverpool, England. Unfamiliarity with the occurrence of femoral hernias in children resulted in a correct diagnosis in only three (15%) of the 20 cases by the referring physician and in only 13 (65%) of the cases by the first surgeon to see the child. Misdiagnosis included inguinal hernia (four cases), lymphadenitis (two cases), and lymphangioma (one case). In two cases, misdiagnosis resulted in mistreatment; one child had the wrong operation performed, and delay in surgery in another resulted in strangulation of the omentum in the hernial sac. Treatment is by operation, which, despite the wide variety of procedures employed, was successful in 18 of the 20 cases. The two recurrences were due to the performance of inadequate operations; recurrence was really persistent, as the femoral pad of fat was removed but the sac was missed. The positive identification of the hernial sac is imperative.

Child↗

Acute fatty liver of pregnancy. Survival with early cesarean section.

We have cared for two women with acute fatty liver of pregnancy in the past two years. Both patients survived as did three of babies. This compares with published mortality rates of up to 85% with this condition. Both patients presented with symptoms and signs of impending liver failure during the third trimester of pregnancy. Both patients were jaundiced, had elevated aminotransferases, leukocytosis, hepatic encephalopathy, and disseminated intravascular coagulation. The diagnosis was confirmed by liver biopsy in both. We attribute the unusually good outcome of these women and their children to early recognition of their disorder and prompt delivery once the diagnosis was considered.

Acute Disease↗

Endoscopic diathermy obliteration of recurrent tracheoesophageal fistulae.

Recurrence of tracheoesophageal fistula after surgical repair for esophageal atresia occurs in approximately 5% to 15% of cases. Further surgery may be hazardous and mortality rates of up to 50% have been reported. We report two patients in whom successful obliteration of a recurrent fistula has been achieved by diathermy at bronchoscopy. This experience is compared to previous reports of fistula obliteration using tissue adhesives.

Bronchoscopy↗

Elevation of cerebrospinal fluid substance P concentrations in spina bifida.

Concentrations of substance P, a peptide believed to have neurotransmitter and possible trophic functions, were determined by radioimmune assay in the cerebrospinal fluids of ten babies and one foetus with spina bifida, and compared to those of five normal babies and ten normal foetuses. The results (mean +/- S.E.M.) were: spina bifida babies (864 +/- 357.6 fmol ml-1), normal babies (141 +/- 14.2 fmol ml-1), spina bifida foetuses (150,000 fmol ml-1), normal foetuses (22,673 +/- 8,340 fmol ml-1). Hence, in spina bifida cerebrospinal fluid substance P concentrations were grossly elevated both pre- and post-natally. The significance of these results can be considered from two aspects: there is the possibility that substance P plays a role in the development of the spinal cord which is disturbed in spina bifida; as substance P has been shown to be involved with sensory perception and autonomic reflexes, abnormal concentration of substance P in cerebrospinal fluid may aggravate the neurological deficit in spina bifida. Possibly, therapeutic manipulation of substance P, when available in the future, may be considered in these patients.

Female↗