Increased catabolism and decreased unsaturation of ganglioside in patients with inflammatory bowel disease.

AIM: To investigate whether accelerated catabolism of ganglioside and decreased ganglioside content contribute to the etiology of pro-inflammatory intestinal disease. METHODS: Intestinal mucosa from terminal ileum or colon was obtained from patients with ulcerative colitis or inflammatory Crohn's disease (n = 11) undergoing bowel resection and compared to control samples of normal intestine from patients with benign colon polyps (n = 6) and colorectal cancer (n = 12) in this observational case-control study. Gangliosides and phospholipids of intestinal mucosa were characterized by class and ceramide or fatty acid composition using liquid chromatography triple-quad mass spectrometry. Content and composition of ganglioside classes GM1, GM3, GD3, GD1a, GT1 and GT3 were compared among subject groups. Content and composition of phospholipid classes phosphatidylcholine (PC) and phosphatidylethanolamine were compared among subject groups. Unsaturation index of individual ganglioside and phospholipid classes was computed and compared among subject groups. Ganglioside catabolism enzymes beta-hexosaminidase A (HEXA) and sialidase-3 (NEU3) were measured in intestinal mucosa using western blot and compared among subject groups. RESULTS: Relative GM3 ganglioside content was 2-fold higher (P < 0.05) in intestine from patients with inflammatory bowel disease (IBD) compared to control intestine. The quantity of GM3 and ratio of GM3/GD3 was also higher in IBD intestine than control tissue (P < 0.05). Control intestine exhibited 3-fold higher (P < 0.01) relative GD1a ganglioside content than IBD intestine. GD3 and GD1a species of ganglioside containing three unsaturated bonds were present in control intestine, but were not detected in IBD intestine. The relative content of PC containing more than two unsaturated bonds was 30% lower in IBD intestine than control intestine (P < 0.05). The relative content of HEXA in IBD intestine was increased 1.7-fold (P < 0.05) and NEU3 was increased 8.3-fold (P < 0.01) compared to normal intestine. Intestinal mucosa in IBD is characterized by increased GM3 content, decreased GD1a, and a reduction in polyunsaturated fatty acid constituents in GD3, GD1a and PC. CONCLUSION: This study suggests a new paradigm by proposing that IBD occurs as a consequence of increased metabolism of specific gangliosides.

Colocolic intussusception in a preterm infant with intestinal malrotation.

The association of intussusception and intestinal malrotation is referred to as Waugh syndrome. A prospective study among 49 children with intussusception found a 40% incidence of malrotation. We describe the rare occurrence of colocolic intussusception in a neonate with malrotated intestine. We believe this is the first reported case in the neonatal period where the pathogenic lead point was an intestinal lymphangioma.

Esophageal mucoceles causing airway obstruction as a complication of esophageal diversion: two rare pediatric cases and a review of the literature.

An esophageal mucocele causing airway obstruction is an exceptionally rare complication of esophageal diversion in children. In this instance, they are fluid-filled dilatations of the esophageal remnant following bipolar exclusion of the thoracic esophagus. Only six pediatric cases have been reported previously in the literature. We present two consecutive cases of esophageal mucoceles causing respiratory distress in children following surgical exclusion of the esophagus. Bronchoscopy followed by imaging (computerized tomography or magnetic resonance imaging) was used to reach the diagnosis. Complete resection of the thoracic esophagus was required in both patients. Esophageal mucoceles can occur many years after esophageal exclusion, and the clinical features are often non-specific. Furthermore, complex co-morbidities may mask the underlying etiology of the respiratory distress, thus the diagnosis may be difficult to delineate. A high degree of suspicion, clinical awareness, and the use of the proper diagnostic tools, are essential for a diagnosis of mucoceles in children with a past history of esophageal exclusion.

Autonomous thyroid nodule in an adolescent 10 years after total body irradiation for bone marrow transplant.

A male patient with B-cell lymphoma was treated with chemotherapy and allogeneic bone marrow transplant, including preparatory total body irradiation. Ten years later, at age 15 years, the patient developed an autonomous thyroid nodule and an incidental papillary microcarcinoma. This is the first report of an autonomous thyroid nodule after total body irradiation for bone marrow transplant. The case is presented and the literature is reviewed.

Association between surgical margins and long-term outcome in advanced hepatoblastoma.

BACKGROUND: Many patients with hepatoblastoma present with unresectable disease. Neoadjuvant therapy has improved resectability rates to as high as 70% to 90%. Despite this improvement, many patients will be left with tumors that are of borderline resectability. The authors hypothesize that favorable outcomes may be achieved even with resection margins less than 1 cm thus sparing the need for liver transplantation. METHODS: Between January 1981 and March 2003, 23 patients age less than 16 years with a diagnosis of hepatoblastoma undergoing surgical resection were identified. The clinical characteristics, pathologic resection margins, and survival status were reviewed. RESULTS: Eighteen (78%) of the patients were alive with no evidence of recurrence at last follow-up. Thirteen (56.5%) had > or =1 cm resection margins, whereas 10 (43.5%) had resection margins less than 1 cm. Eleven (47.8%) presented with PRETEXT III tumors. There was no significant difference in survival rate between resection margins less than 1 cm and > or =1 cm (P =.13; 95% CI 0.91 to 2.61). Thirteen patients (56.5%) presented with synchronous pulmonary metastatic disease, where survival was significantly worse (P =.04; 95% CI 1.10 to 2.50). Subgroup analysis confirmed that margins less than 1 cm did not significantly affect survival after controlling for pulmonary metastatic disease (P =.56; 95% CI 0.71 to 3.61). CONCLUSIONS: Surgical resection with margins less than 1 cm are associated with survival that is equivalent to resection with margins > or =1 cm. Our findings suggest it is preferable to preserve key structures with a small resection margin and therefore spare the need for liver transplantation in patients with advanced hepatoblastoma.