PubMed Health⌕ Search

Biomedical subjects

D E Schofield

Publications and source records attributed to D E Schofield.

24 records · Page 2Linked to original sources
PubMed1991

Intestinal neuronal dysplasia.

Four hundred ninety-eight acetylcholinesterase-stained suction rectal biopsies from 456 children were examined to assess the occurrence of "isolated" or "primary" intestinal neuronal dysplasia at Children's Hospital of Pittsburgh. Cases of proven Hirschsprung's disease were excluded. In 38 biopsies from 38 patients, we found a mild to moderate increase in mucosal acetylcholinesterase staining and abundant submucosal ganglion cells (large, irregular ganglia or at least five ganglia per HPF). This group was clinically heterogeneous with a frequent history of prematurity and small left colon/meconium plug syndrome, protein/formula intolerance, or obstructive anatomic gastrointestinal abnormalities. We feel that isolated "intestinal neuronal dysplasia," as diagnosed by suction rectal biopsy and the above criteria, is a descriptive biopsy appearance. Rather than characterizing a unique disease entity, it is encountered in a variety of clinical situations. Based on our observations and review of the literature, we therefore conclude that "intestinal neuronal dysplasia" not associated with Hirschsprung's disease, neurofibromatosis, or multiple endocrine neoplasia syndrome has yet to be well defined in clinical and histopathologic terms. The histologic diagnosis, at this time, should be reserved for those rare, florid cases of parasympathetic hyperganglionosis that are documented by adequate tissue sampling.

Acetylcholinesterase↗
PubMed1990

Acetylcholinesterase-stained suction rectal biopsies in the diagnosis of Hirschsprung's disease.

Conflicting reports in the literature regarding the sensitivity and specificity of the acetylcholinesterase (AChE) stain in establishing or excluding the diagnosis of Hirschsprung's disease (HD) prompted this review of 497 rectal biopsies performed on 455 children. Using hematoxylin and eosin (H&E) to stain formalin-fixed, paraffin-embedded tissue sections is our preferred method of identifying ganglion cells. In this series, however, there were eight children with HD, and nine without HD in whom the AChE-stained portion of the sample provided invaluable diagnostic information not obtained from the concomitant, formalin-fixed, H&E-stained portion of the sample. The AChE stain also provided at least suggestive evidence of HD in some of the anal or anorectal biopsy specimens.

Acetylcholinesterase↗
PubMed1988

Common arterial trunk with pulmonary atresia.

We describe a congenitally malformed heart with usual atrial arrangement and concordant atrioventricular connexions in which a solitary arterial trunk left the base of the heart and supplied the coronary and systemic arteries. Pulmonary blood supply was derived from systemic to pulmonary collateral arteries but, in addition, hypoplastic intrapericardial pulmonary arteries were found in each lung hilum. The two sets of hilar arteries were discontinuous, but it seemed likely that this discontinuity had been artefactually produced by prior dissection. Irrespective of this matter, the left pulmonary arteries were connected by a thread-like atretic cord to the left-sided sinus of the solitary arterial trunk. This indicates that, initially, there had been a common trunk which now demonstrates pulmonary atresia. The significance of this lesion is discussed in the light of the nomenclature and description of hearts with common arterial trunk.

Heart Defects, Congenital↗
PubMedDate not supplied

Monozygotic twins concordant for congenital neuroblastoma: case report and review of the literature.

To date, five sets of monozygotic twins concordant for neuroblastoma have appeared in the literature. Review of the clinical information available for these cases suggests that they represent congenital, versus acquired, diseases in both twins from each twin pair. The question arises, then, whether some or all sets of twins represent simultaneous-onset malignancy of both twins within a twin pair or whether metastasis via placental anastomoses from one twin with congenital disease to the cotwin occurs. This report includes a sixth set of monozygotic twins concordant for congenital neuroblastoma. From analysis of the clinical data from all cases, it appears that two of six twin pairs may represent simultaneous-onset tumors in each twin from a twin pair, and two of the remaining four twin pairs, including the present case, may represent placental metastases from one twin with congenital neuroblastoma to the other. In the remaining two twin pairs insufficient data are available upon which to draw any conclusions. Now that molecular and genetic methodology is available to characterize neuroblastomas, these techniques may be utilized in future cases of monozygotic twins concordant for congenital neuroblastoma, to help clarify whether the neoplasms represent simultaneous primary tumors versus metastatic spread from one twin to another.

Adrenal Gland Neoplasms↗