Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report an interesting case of congenital inflammatory bowel disease and intestinal epithelial immaturity that presented as secretory diarrhea. No infectious, metabolic, or anatomical basis for these findings was identified. As differentiated from previous reports of neonatal enteropathies, this infant demonstrated involvement of both the small and large intestine with histopathologic findings of acute and chronic inflammation, extensive submucosal fibrosis, and "flat" small intestine mucosa. In addition, this patient had a polyamine deficiency (a primary or secondary phenomenon), which may have contributed to delayed epithelial maturation. These findings suggest that the inflammatory bowel disease and altered epithelial maturation contributed to a fatal intractable diarrhea.
 ...
PMID:Congenital diarrhea with intestinal inflammation and epithelial immaturity. 379 28

As in any operation for IBD, colectomy and ileorectal anastomosis should be performed only after every effort has been made to control the disease medically. Only in uncontrolled disease should early proctectomy be advised on the grounds of lack of normal physical development and sexual immaturity. Ileorectal anastomosis should not be performed upon every patient requiring surgical treatment any more than proctocolectomy and ileostomy should be performed upon every patient. Unless there is severe persistent disease of the rectum or destruction of the anal sphincter, the rectum should be preserved because severe ulcerative proctitis may heal or improve postoperatively with further medical treatment. If further surgical treatment is necessary, conversion to an ileostomy can be undertaken, and there are now other alternatives, such as the continent ileostomy and the ileoanal anastomosis, with or without a pelvic pouch.
 ...
PMID:Ileorectal anastomosis for inflammatory bowel disease in children and adolescents. 664 77

Neonatal necrotizing enterocolitis is a devastating inflammatory bowel disease of premature infants. The pathogenesis remains incompletely understood and there is no specific treatment. Efforts are ongoing to understand aspects of intestinal immaturity which contribute to susceptibility to this disease. This review focuses on bacterial colonization patterns, intestinal barrier function, and inflammatory responses of immature enterocytes leading to a unique vulnerability of the preterm gut. In addition the possible therapeutic potential of factors in human milk and probiotic bacteria is discussed.
 ...
PMID:Neonatal Necrotizing Enterocolitis -Inflammation and Intestinal Immaturity. 2049 29

Inflammatory Bowel Diseases (IBD) are chronic inflammatory disorders, where epithelial defects drive, at least in part, some of the pathology. We reconstituted human intestinal epithelial organ, by using three-dimension culture of human colon organoids. Our aim was to characterize morphological and functional phenotypes of control (non-IBD) organoids, compared to inflamed organoids from IBD patients. The results generated describe the epithelial defects associated with IBD in primary organoid cultures, and evaluate the use of this model for pharmacological testing of anti-inflammatory approaches. Human colonic tissues were obtained from either surgical resections or biopsies, all harvested in non-inflammatory zones. Crypts were isolated from controls (non-IBD) and IBD patients and were cultured up to 12-days. Morphological (size, budding formation, polarization, luminal content), cell composition (proliferation, differentiation, immaturity markers expression), and functional (chemokine and tight junction protein expression) parameters were measured by immunohistochemistry, RT-qPCR or western-blot. The effects of inflammatory cocktail or anti-inflammatory treatments were studied in controls and IBD organoid cultures respectively. Organoid cultures from controls or IBD patients had the same cell composition after 10 to 12-days of culture, but IBD organoid cultures showed an inflammatory phenotype with decreased size and budding capacity, increased cell death, luminal debris, and inverted polarization. Tight junction proteins were also significantly decreased in IBD organoid cultures. Inflammatory cytokine cocktail reproduced this inflammatory phenotype in non-IBD organoids. Clinically used treatments (5-ASA, glucocorticoids, anti-TNF) reduced some, but not all parameters. Inflammatory phenotype is associated with IBD epithelium, and can be studied in organoid cultures. This model constitutes a reliable human pre-clinical model to investigate new strategies targeting epithelial repair.
 ...
PMID:Characterization of Human Colon Organoids From Inflammatory Bowel Disease Patients. 3258 90