Colonic ganglioneuroma presenting as filiform polyposis

Hypereosinophilia is a rare manifestation of cancer. We reported four cases of tumor-associated hypereosinophilia. These cases presented with peripheral hypereosinophilia and disseminated metastatic malignancies. All cases were male, including two cases with cancer of unknown primary site, one with hepatocellular carcinoma, and one with a liver tumor. The age ranged from 27 to 55 years. They all had liver involvement. Two cases had bone marrow metastases. The leukocyte counts ranged from 78,600/microliters to 190,000/microliters. The percentage of eosinophils ranged from 20% to 77%. The eosinophil counts ranged from 15,720 to 126,350/microliters with a mean of 74,700/microliters. The first three cases died within 8 days after the malignancies were pathologically confirmed. We suggest that peripheral hypereosinophilia is a poor prognostic sign which is frequently associated with disseminated cancer.     

Colonic histiocytic neoplasm mimicking malignant histiocytosis and presenting as intussusception

It is now apparent that distinction between the so-called malignant histiocytosis and lymphoma can be made using panels of established immunohistochemical markers and/or genotypic analysis. Many, if not all, of the previously diagnosed cases of malignant histiocytosis have been shown to be of lymphoid, rather than histiocytic, lineage. We report a rare case of colonic histiocytic neoplasm accompanied by a lymphoreticular dissemination that mimicked that of malignant histiocytosis. In addition, barium studies and computed axial tomography confirmed an intussusception that subsequently developed. The histiocytic nature of the neoplastic cells was supported by immunohistochemical, ultrastructural, and cytochemical studies. To our knowledge our case may represent the fifth documented case of a histiocytic malignancy reported in the literature. The relationship among the various cases will be discussed as well as the significance of the focal S-100 immunoreactivity observed in the present case.     

Colonic strictures in children with cystic fibrosis

PURPOSE: To determine the radiographic, clinical, surgical, and histologic findings in children with cystic fibrosis who develop strictures of the colon. MATERIALS AND METHODS: Ten children (five boys, five girls; age range, 2.5-9.0 years; mean age, 5.5 years), who were treated at the practices of the authors, were retrospectively identified and their medical records reviewed. RESULTS: Radiographic manifestations of the colonic disease included mucosal irregularity and spiculation with nodular thickening of the colonic wall and loss of normal colonic haustration. Luminal narrowing involved long segments of the colon. Longitudinal shortening of the colon was also a prominent feature. The decrease in caliber of the bowel ranged from mild narrowing to complete occlusion of the lumen. Histologic examination revealed severe submucosal fibrosis and fatty infiltration with transmural extension of the fibrosis to involve the serosa in some cases. Unlike in Crohn disease, however, acute inflammatory changes were minimal or absent. CONCLUSION: Colonic stricture in children with cystic fibrosis is due to irreversible and frequently progressive narrowing of the colonic lumen.     

Role of bacterial colonization in neonatal necrotizing enterocolitis and its prevention

Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in premature infants. A major component of the pathophysiology of NEC is the nature of the interaction of bacteria with the premature gut. Intestine microflora are important to the host in resistance to bacterial infections. Diet and environmental conditions can influence this ecosystem. A breast-fed full-term infant has a preferred intestine microbiota in which bifidobacteria predominate over the potentially harmful bacteria, whereas in formula-fed infants coliforms, enterococci and bacteroides predominate. The pattern of bacterial colonization in the premature neonate gut is quite different from that in the gut of the healthy full-term infant. Those infants requiring intensive care acquire intestinal organisms slowly, and the establishment of bifidobacterial flora is retarded. A delayed bacterial colonization of the gut with a limited number of bacterial species tends to be virulent. Bacterial overgrowth is one of major factors promoting bacterial translocation. The aberrant colonization of the premature infant may contribute to the development of NEC. Breast feeding protects infants against NEC. Oligosaccharides and glycoconjugates, natural components in human milk, may prevent intestinal attachment of enteropathogens by acting as receptor homologues. Probiotics and prebiotics modulate the composition of human intestine microflora to the benefit of the host. The beneficial effects may result in the suppression of colonization of harmful microoganisms and/or the stimulation of bifidobacterial growth. In the future, control and manipulation of bacterial colonization in the neonate gut may be a new approach to the prevention and treatment of bacterial intestinal disease of various etiologies.     

Congenital malformations presenting during the neonatal period

Discharge of the newborn within 24 to 48 hours after birth makes it more difficult to detect some congenital malformations and increases the need for careful examination and review of the history of the pregnancy, delivery, and nursery course. Progressive physiologic changes after birth, especially in the cardiovascular system, precede the development of signs indicative of disease for certain congenital malformations. Discharge before these changes occur may delay their detection because the newborn is not being monitored by medical or nursing caregivers. The AAP Committee on the Fetus and Newborn has published guidelines for criteria for safe discharge and follow-up evaluation to help create a safe situation for such early discharges. Some specific observations at birth may lead to earlier diagnosis. Careful attention to subtle differences between the initial and follow-up examination, such as a changing cardiac murmur or quality of pulses or abdominal fullness, may provide clues to the diagnosis of congenital malformations. Coordinated suck and swallow with successful feeding and passage of stool and urine within 24 hours after birth should occur before discharge. Reports of feeding difficulties should be reviewed. Although a thorough examination is facilitated by a sleeping infant, documentation of a normal pitched cry helps in the evaluation of the upper airway. Parents should be counseled about signs of illness that warrant medical attention, and early follow-up is needed to detect problems early enough to intervene effectively. In addition, although passage of a feeding tube through each nare and to the stomach with aspiration and measurement of gastric volume is not a routine procedure in the well, term newborn, this may be a useful early diagnostic tool in infants with signs or a maternal or nursery history suggestive of nasal or GI obstruction.     

Neonatal pneumatosis coli: a mild form of neonatal necrotizing enterocolitis

A distinctive form of necrotizing enterocolitis was observed in seven newborn infants in a 4 1/2-year period. Characteristically these patients had gross blood in the stools, but other local and systemic signs were mild or absent. Radiography mainly revealed submucosal intramural intestinal gas which was limited to the colon (pneumatosis coli). There was no evidence of small bowel involvement as judged by the absence of small intestinal pneumatosis or distension. This form of colonic disease should be recognized as a benign variety of neonatal necrotizing enterocolitis that carries a favorable prognosis and responds to medical management without sequelae.     

Use of multiple urolume endourethral prostheses in complex bulbar urethral strictures

PURPOSE: We evaluated patients who received multiple UroLume Wallstents during the North American UroLume trial for the treatment of recurrent bulbar urethral strictures. MATERIALS AND METHODS: A total of 41 patients received multiple UroLume stents. The clinical histories and therapeutic outcomes of these patients were reviewed. RESULTS: Of the patients 23% required placement of multiple urethral stents. Stents placed at the initial procedure were required for strictures longer than 2.5 cm. and for multiple, separate strictures. Indications for secondary insertion included recurrent stricture adjacent to the stent, hyperplastic tissue growth within the stent and gaps between previously adjacent stents. The repeat treatment rate was 43.9% versus 14.3% for the study group overall. Urine flow rates and symptom scores in the multiple stent group showed improvement similar to that of the study group overall. CONCLUSIONS: Patients who required multiple stents had greater rates of repeat treatment but similarly improved urine flows and symptom scores, which were maintained at 2 years.     

Alterations of the enteric nervous system in neonatal necrotizing enterocolitis revealed by whole-mount immunohistochemistry

We report the results of a comprehensive search of Drosophila melanogaster DNA sequences in GenBank for di-, tri-, and tetranucleotide repeats of more than four repeat units, and a DNA library screen for dinucleotide repeats. Dinucleotide repeats are more abundant (66%) than tri- (30%) or tetranucleotide (4%) repeats. We estimate that 1917 dinucleotide repeats with 10 or more repeat units are present in the euchromatic D. melanogaster genome and, on average, they occur once every 60 kb. Relative to many other animals, dinucleotide repeats in D. melanogaster are short. Tri- and tetranucleotide repeats have even fewer repeat units on average than dinucleotide repeats. Our WorldWide Web site (http://www.bio.cornell.edu/genetics/aquadro/+ ++aquadro.html) posts the complete list of 1298 microsatellites (> or = five repeat units) identified from the GenBank search. We also summarize assay conditions for 70 D. melanogaster microsatellites characterized in previous studies and an additional 56 newly characterized markers.     

Toxic megacolon as a complication of Campylobacter jejuni enterocolitis

We report the case of a previously healthy 53-year-old white male who developed an extraordinary complication of acute Campylobacter jejuni colitis. Toxic megacolon occurred while the patient was treated with a fluoroquinolone antibiotic and glucocorticoids, which were given for endoscopically suspected Crohn's colitis. During the course of the disease no cause of colitis was found other than C. jejuni. Despite the extreme dilatation, the patient was treated conservatively with parenteral nutrition and repeated decompression colonoscopies and made a full, though slow, and uneventful recovery. Follow-up colonoscopies for up to 4 years showed persistent scarring of the transverse colon, probably due to the extreme dilatation, and mild unspecific inflammation of the terminal ileum without histological evidence of inflammatory bowel disease. A comparison with the 6 previously published cases leads to the following conclusions: in most cases the transverse colon is most severely affected. Treatment with either antimotility agents or systemic glucocorticoids does not seem to promote colonic dilatation. The complication has affected patients of both sexes (4 women, 3 men), in the age range of 21 to 83 years, most of them without an underlying disease. The interval between the start of diarrhea and development of the megacolon ranged widely from 3 to 33 days, as did recovery time (2 days to several months). Three of the 7 patients underwent colectomy for imminent or actual colonic perforation. The delayed recovery of our patient was partly attributed to colonic damage caused by extreme dilatation, leading to ischaemia and subsequent scarring of the mucosa, which persisted. Histologically no Crohn's disease or ulcerative colitis could be found at any stage. A rapid increase in resistance of C. species against fluoroquinolone antibodies has been observed in recent years, due to use of the antibiotics in farming. Our patient's severe illness may partly have resulted from delayed effective antibiotic treatment due to resistance. Antibiotic resistance to common enteropathogens should be considered in the case of unusually prolonged or severe enterocolitis. The level of suspicion for either infection or inflammatory bowel disease should remain high as it may be impossible to distinguish between them on the basis of clinical or endoscopic criteria alone.     

Escherichia coli transcytosis in a Caco-2 cell model: implications in neonatal necrotizing enterocolitis

Necrotizing enterocolitis (NEC) is a serious gastrointestinal disorder of preterm infants. Other than an association with prematurity and gastrointestinal feeding, no single factor or mechanism has been consistently linked to this disease. We have previously demonstrated that Escherichia coli isolates obtained from the stool of infants with NEC caused NEC-like injury in a weanling rabbit ileal loop model; this injury, in turn, could be blocked by coinfection with selected Gram(+) bacteria (Enterococcus faecium) isolated from asymptomatic controls. Using Caco-2 cells in a trans-well system, we now demonstrate that the same E. coli isolates can cross epithelial cell monolayers in the absence of ultrastructural change or damage. These results with E. coli contrast with those seen with Salmonella typhimurium, which passed through the monolayer at a higher rate and were associated with striking ultrastructural damage. Transcytosis of E. coli was reduced 3-5-fold in the presence of E. faecium previously shown to block NEC-like injury in the loop model. There was a mild increase in the rate of E. coli transcytosis when studies were conducted with younger, undifferentiated cells; these immature cells had no brush border, had decreased production of brush border-specific enzymes, but retained well defined tight junctions, as demonstrated by transepithelial electrical resistance and electron microscopy. A further reduction/ complete blockage of E. coli transcytosis was observed when E. faecium was used as the coinfectant in studies with these undifferentiated cells. We hypothesize that the ability of E. coli to cross epithelial cell layer is a critical initial step in the cascade of events which lead ultimately to NEC; blockage or reduction in E. coli transcytosis in the presence of certain Gram(+) organisms may play a significant role in prevention of NEC.     

The role of recombinant platelet-activating factor acetylhydrolase in a neonatal rat model of necrotizing enterocolitis

Previous studies have shown that the endogenous inflammatory mediator platelet-activating factor (PAF) plays an important role in the pathophysiology of neonatal necrotizing enterocolitis (NEC). This study was designed to investigate the role of the PAF-degrading enzyme acetylhydrolase (PAF-AH) in a neonatal rat model of NEC. To study the absorption, localization, and activity of human recombinant PAF-AH (rPAF-AH), newborn rats were treated with enteral rPAF-AH, and plasma and intestines were sampled at 8 and 24 h for determination of PAF-AH enzyme activity and rPAF-AH concentration using a specific enzyme-linked immunoassay. To study the effect of rPAF-AH on neonatal NEC, rats were treated with rPAF-AH via the enteral route every 3 h, and then subjected to formula feeding and asphyxia per an established neonatal rat protocol for NEC. Pretreatment with enteral rPAF-AH significantly reduced the incidence of NEC compared with controls (6/26 versus 19/26, p < 0.001). We found that enteral rPAF-AH administration resulted in significant intestinal PAF-AH activity but no circulating PAF-AH activity despite immunohistochemical localization of the administered rPAF-AH to the intestinal epithelial cells. These findings suggest that rPAF-AH is functional and stable in the gut of neonatal rats. We conclude that enteral administration of rPAF-AH remains locally active and reduces the incidence of NEC in our experimental animal model.     

Sarcoidosis presenting as obstructive jaundice

Sarcoidosis is an uncommon disorder characterized by a multi-systemic granulomatous disease of undetermined etiology and pathogenesis. The diagnosis is established by the presence of a compatible clinical illness and by histologic demonstration of noncaseating epithelioid cell granulomas in the affected organs. Accurate diagnosis requires a thorough evaluation to exclude infectious and neoplastic diseases that can mimic sarcoidosis. Although all organs and systems can be affected, the lungs and intrathoracic lymph glands are the most common sites of involvement. We describe an unusual case of extrapulmonary sarcoidosis presenting as obstructive jaundice.