James Lister

Segmental dilatation of the ileum

Three new cases of congenital segmental dilatation of the ileum are presented and added to 12 cases of segmental dilatation found in the literature. The aspect of the condition which is of particular interest is the presence in 3 of the dilated segments of heteroplastic foregut derivatives of unusual varieties. The etiology of the condition is discussed; it does not appear to be a single distinct pathological entity.

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.

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 Hirschsprungs 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 Hirschsprungs disease needs to be reconsidered.

Cystic duplications in the tongue.

Abstract Cysts in the floor of the mouth are not uncommon and they are usually regarded as retention cysts of small sublingual mucous glands. Cysts of the tongue are much more rare and they may consist of lymphangiomata, dermoid cysts, or thyroglossal cysts. In addition, duplication of the foregut has been reported as a cause of lingual cysts. Three such cases have been seen at the Childrens Hospital Sheffield, and five others reported in the literature.

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.

Response to gastrin pentapeptide in children

Abstract Few studies of maximal acid secretion in children have been published and none using the gastrin analogue, pentagastrin. Pentagastrin is a synthetic polypeptide containing the active C-terminal pentapeptide of the gastrin molecule and it exhibits all the physiologic actions of pure gastrin. The gastrin analogues have infrequent and minor side effects and are likely to replace histamine and ametazole in tests for gastric function in man, since pentagastrin elicits peak secretion more quickly and is at least as potent a stimulant of gastric acid secretion as histamine and ametazole. 1–4 The aim of the study was to assess the use of pentagastrin to stimulate maximal gastric acid secretion in children. It was also hoped to recognize cases of hiatal hernia that are liable to develop complications, recurrent esophagitis, or stricture, in spite of medical supervision.

Pelvicalyceal cysts in children

Abstract Eight cases of calyceal cyst in children are described, only three of them producing signs and symptoms. Operation is indicated only in those cases in which there is recurrent infection, recurrent pain, or possibly stone formation. A simple unroofing of the cyst appears to be the operation of choice, since it reduces to the minimum damage to normal renal parenchyma.

The control of fluid loss in long-segment Hirschsprung's disease

Abstract Two children with total colonic aganglionosis, both of whom were in considerable trouble from fluid loss derived from short-circuiting ileostomies, had modified Duhamel procedures preserving a long segment of aganglionic rectum and colon to increase fluid absorption. By using an anastomosing stapling instrument, a long side-to-side ileorectal anastomosis can be made with no residual functional obstruction from the aganglionic bowel. The procedure can be carried out in a child of 3 months or less.

Incidence of diabetes in families of patients with Hirschsprung's disease

Summary We draw attention to the possible increased incidence of diabetes in the families of children with Hirschsprungs disease and the increased severity of Hirschsprungs disease occurring in diabetic families. If a similar incidence were found in other series the total figures might achieve statistical significance.

Is there a decline in neonatal surgery

Abstract The apparent severe fall in admissions to the neonatal surgical unit in the Sheffield Childrens Hospital, after corrections have been made for known changes in the disposal of case material in the region, is almost completely in line with the falling curve of the birth rate. The constancy of this curve is due to the fact that the reduction in cases of neural tube defects is offset by an increase in other neonatal surgical problems.