Faculty Bibliography

The origin and histology of the cardiac mucosa remains controversial. The classical concept that the cardiac mucosa is of gastric origin has been challenged by those who advocate that the cardiac mucosa results from a metaplastic esophageal process. Some regard cardiac mucosa as consisting solely of pure mucous glands, whereas others accept the presence of isolated parietal cells within the mucous gland (mixed glands). In this study, we have clarified the presence and site of origin of the cardiac mucosa and its histological composition. To do so we studied the microscopic characteristics of the gastric side of the squamous-columnar junction (SCJ) of 77 autopsied fetuses of different gestational ages (prenatal group) and of infants, young children, and adolescents (postnatal group). We evaluated the presence or absence of a transitional zone, defined as the area between the squamous esophageal and oxyntic mucosa, the glandular composition of the transitional zone (i.e., pure mucous and mixed glands), and the presence or absence of inflammation. Our study revealed that a transitional zone with the microscopic characteristics of cardiac mucosa was universally present at the SCJ. The microscopic characteristics of this zone varied with age. Both pure mucous and mixed glands were observed. We conclude that the cardiac mucosa is partially if not entirely the result of normal embryonic gastric development. Both mucous and mixed glands constitute normal components of the cardiac mucosa

BACKGROUND & AIMS: Clinical experience suggests that 6-mercaptopurine (6-MP) is effective therapy for children with active steroid-dependent Crohn's disease (CD). We report the results of a prospective, placebo-controlled, multicenter trial evaluating the combination of 6-MP and prednisone as therapy for children with newly diagnosed moderate-to-severe CD. METHODS: Fifty-five children (age, 13+/-2 years) were randomized to treatment with 6-MP (1.5 mg x kg(-1) x day(-1)) or placebo within 8 weeks of initial diagnosis. Both groups also received prednisone (40 mg/day). Prednisone dosage adjustments were based on a defined schedule determined by the change in a subject's disease activity score, and steroid administration was discontinued as remission was achieved. Study treatment with 6-MP or placebo continued for 18 months. RESULTS: Groups were comparable for age, sex, and site and activity of disease. In the 6-MP group, the duration of steroid use was shorter (P<0.001) and the cumulative steroid dose lower at 6, 12, and 18 months (P<0.01). Although remission was induced in 89% of both groups, only 9% of the remitters in the 6-MP group relapsed compared with 47% of controls (P = 0.007). Growth was comparable in both groups. No clinically significant adverse events occurred, although mild leukopenia and increases in aminotransferase activity were noted in the 6-MP group. CONCLUSIONS: Addition of 6-MP to a regimen of corticosteroids significantly lessens the need for prednisone and improves maintenance of remission. 6-MP should be part of the initial treatment regimen for children with newly diagnosed moderate-to-severe CD

BACKGROUND: Pancreatic acinar tissue (PAT) at the gastroesophageal junction (GEJ) has been reported in 3% of adults with Barrett esophagus (BE) and in 24% of healthy subjects. The pathogenesis of this ectopic tissue is controversial. Both an acquired metaplastic process in the setting of BE and a congenital abnormality have been suggested in adults. OBJECTIVE: To clarify the origin of PAT at the GEJ. METHODS: We reviewed material obtained from the GEJ in 69 children and young adults. Each specimen was evaluated by 3 levels stained with hematoxylin-eosin for the presence of PAT, BE, esophagitis, and gastritis. Selected cases were also examined with immunohistochemical stains for lipase, trypsin, and amylase. RESULTS: In 16% of the study population, PAT was present at the GEJ and was not associated with BE. The prevalence of esophagitis and/or gastritis did not vary significantly between patients with and without PAT. CONCLUSIONS: Our data suggest that PAT at the GEJ develops independently of inflammation and is, therefore, likely to be congenital

Hepatotoxity associated with amoxicillin/clavulanic acid is usually a self-limited disease with complete recovery. We report a rapidly progressing liver disease with ductopenia and portal fibrosis in a 3-year-old boy treated with Augmentin

Tangier Disease (TD), a rare autosomal disorder, is characterized by low plasma cholesterol, decreased or absent A-I apolipoprotein and normal ol elevated plasma triglycerides. ID was diagnosed antemortem by serologic and electrophoretic studies. Careful examination of the organs at autopsy showed the presence of lipid-laden macrophages, the hallmark of TD, only in the rectosigmoid mucosa, and not in other organs usually affected in ID. These findings indicate that the widespread distribution of lipid deposits may be absent in patients with ID early in life. In addition, DiGeorge syndrome (DGS) was recognised at autopsy by absence of the thymus and presence of only one parathyroid, thus explaining the multiple opportunistic infections during life

Ivemark syndrome is a rare sporadic or autosomal recessive disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. There have been no data describing the renal changes during embryologic development in this syndrome. In this report, we document the pathological findings of the kidney in three subjects with Ivemark syndrome: 6 months, 21 weeks and 16 weeks, respectively. Kidneys of subjects and age-matched controls were examined by light microscopy and immunohistochemically for cytokeratin, AE1/AE3 and epithelial membrane antigen. Renal dysplasia in Ivemark syndrome becomes apparent at 16 weeks of gestation and progresses thereafter in severity. It is characterized by disturbance in glomerular differentiation, delay in tubular differentiation and abnormal expression of epithelial markers in glomeruli and tubules. Cytokeratin and epithelial membrane antigen expression of cysts is similar to that of the collecting ducts