Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver disease is relatively common during parenteral nutrition (PN). Cholestasis predominates in infants, and ranges in severity from mild increases in plasma conjugated bilirubin to progressive liver failure that results in death of the patient. Severity of liver disease depends primarily on the magnitude of the underlying intestinal problem that indicated PN. Transient ileus resulting from a non-intestinal disorder usually results in trivial, self-limited liver injury. Removal of a large segment of the intestinal tract because of necrotizing enterocolitis or a congenital malformation predicts a more prolonged course with a guarded prognosis, particularly when initially complicated by sepsis. Pathogenesis of PN-associated liver disease is not completely understood. There is no proven treatment short of ending PN through adaptation of remnant intestine or intestinal transplantation, with or without a concurrent liver graft. Effective interventions that are less radical than transplantation are needed. Research that includes prospective trials of novel therapies in PN-associated liver disease is the key to improving outcome.
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PMID:Parenteral nutrition associated liver disease. 1500 Nov 25

A primary small intestinal natural killer (NK) cell lymphoma with pathologic features of enteropathy but lack of association with celiac disease is reported. A 37-year-old man presented with tarry stool, coffee-ground vomitus, and mild fever. He did not have chronic diarrhea or malabsorption. Segmental resection of the duodenum and jejunum showed multicentric transmural infiltration by medium-sized lymphoma cells expressing CD3, CD8, cytotoxic granules, and Epstein-Barr virus by in situ hybridization. The nontumorous mucosa away from the main tumor revealed enteropathy with villous blunting and increased intraepithelial lymphocytes sharing the same immunophenotype as the lymphoma cells. Both lymphoma and nontumorous areas were germline for T-cell receptor-gamma and immunoglobulin heavy chain gene rearrangement. Serologic test by ELISA was negative for anti-transglutaminase. The patient died of repeated gastrointestinal bleeding and sepsis at 2 months. Differential diagnosis of this unique nasal-type NK-cell lymphoma with enteropathy-associated T-cell lymphoma is discussed.
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PMID:Natural killer cell lymphoma of small intestine with features of enteropathy but lack of association with celiac disease. 1513 43

A 51-year-old woman was admitted to our hospital with tonsillar swelling. After tonsillectomy was performed, she was diagnosed as having CD56-positive T-cell lymphoma, mainly composed of small and medium-sized atypical cells. An immunohistochemical study showed that the malignant lymphocytes were positive for CD3, CD8, CD56, TIA-1 and granzyme B, while negative for CD20, CD5 and CD10. Flowcytometry demonstrated the lymphocytes were positive for CD56. Southern blot analysis revealed a rearrangement of the T-cell receptor gamma chain. The disease stage by Ann Arbor staging classification was II B. We provided MCEC therapy followed by autologous peripheral blood stem cell transplantation, and complete remission (CR) was achieved. Two months after CR, however, the patient relapsed with peritonitis due to perforation of an ileal tumor, and died of sepsis. It is rare for CD56-positive T-cell lymphoma to occur primarily in the tonsils. Because small bowel ulcers were revealed during the course of induction chemotherapy, we report a valuable case in which suspected CD56-positive enteropathy-type T-cell lymphoma (ETL) occurred primarily in the tonsils.
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PMID:[CD56-positive peripheral T-cell lymphoma primarily presenting with tonsillar swelling]. 1555 48

Severe and protracted or persistent diarrhea (SPD) is the most severe form of diarrhea in infancy and has also been defined as intractable diarrhea when it leads to dependence on total parenteral nutrition (TPN). One of the rare causes of SPD is represented by autoimmune enteropathy that is characterized by life-threatening diarrhea mainly occurring within the first years of life, persistent villous atrophy in consecutive biopsies, resistance to bowel rest, and evidence of antigut autoantibodies. We evaluated 10 patients (seven boys, mean age at diagnosis 18 months; range: 0 to 160 months) fulfilling criteria of autoimmune enteropathy to assess dependence on TPN. TPN was first required in all patients to avoid dehydration and electrolytic imbalance. All patients were dependent on immunosuppressive therapy (steroid, azothioprine, cyclosporine, tacrolimus). Three patients died of sepsis: two during TPN while in the hospital, and one at home after he was weaned off TPN. Five patients are weaned off TPN after a mean period of 18 months; they are actually on oral alimentation with a cow milk-free diet after a period of enteral nutrition with elemental formula. One underwent total colectomy and bone marrow transplantation and one developed an IPEX syndrome. One patient is still dependent on TPN for 24 months. She is on home parenteral nutrition. Patients with diagnosis of IPEX syndrome require parenteral support with three or four infusion per week. TPN represents a fixed step in the management of autoimmune enteropathy, but it may be considered as an interim treatment while waiting for intestinal adaptation, at least in some selectioned case of autoimmune enteropathy. Bone marrow transplantation should be considered and reserved for those patients with severe complications due to home parenteral nutrition, or in those that are really dependent on parenteral nutrition.
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PMID:Long-term parenteral nutrition in pediatric autoimmune enteropathies. 1596 96

Mesenteric inflammatory veno-occlusive disease (MIVOD) is a relatively recently known and not very often diagnosed form of ischemic bowel disease of low incidence und unknown etiology. We present the case of a patient who after presentation of inconclusive signs of epigastric pain and rectal bleeding suddenly developed right abdominal pain with local peritonism. Suspecting intestinal ischemia or perforated appendicitis we first performed laparoscopy, which showed an inflammable tumor of cecum, ascending colon and appendix with massive adhesions to the abdominal wall. We performed an open right hemicolectomy with primary anastomosis. The patient developed a deep vein thrombosis of the vena tibialis post. and vena saphena parva. After 12 months our patient is free of complaints and recurrence. Investigations carried out showed no evidence of hypercoagulopathy. The presentation of MIVOD can range from chronic inflammatory bowel disease with recurrent abdominal pain in combination with nausea, emesis and bloody diarrhea to acute abdomen. Therefore diagnostic misinterpretation and mistherapy as well as underdiagnosis is common. Histologic investigation shows a variable inflammatory infiltration of multiple veins of the intestinal wall and the mesentery as well as thrombotic vessel occlusion in different stages without involvement of the arteries. All forms of hypercoagulopathy, parasitic disease, sepsis and malignancy have to be excluded. Therapeutic success can only be achieved with surgical resection of the affected bowel, whereon in general no recurrence will occur.
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PMID:[Mesenteric inflammatory veno-occlusive disease (MIVOD)--a rare cause of intestinal ischemia]. 1639 91

We report the case of a 45-year old man with non-healing ulcers located on his chest, lumbal, sacral, retroauricular areas and forehead. Both clinical and histopathological examinations suggested pyoderma gangrenosum (PG). For six months the diagnosis of ulcerative colitis was established. PG in our patient was presented as a rapidly enlarging, painful ulcer with purple, undermined edges and a necrotic, haemorrhagic base. Initially, he was treated with a high dosage of peroral glucocorticosteroid, sulfasalazine, and systemic antibiotics, together with daily wound care. Ulceration partially regressed. Total colonoscopy showed pancolitis. When the dose of glucocorticosteroids was tapered down to 35 mg, new ulcerations on his right thigh and abdomen were formed. He also developed E. coli sepsis and flare up of bowel disease. Azathioprine, together with two pulse doses of glucocorticosteroids and antibiotics, were administered. He was scheduled for a total colectomy. The management of PG continues to be a therapeutic challenge.
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PMID:Pyoderma gangrenosum associated with ulcerative colitis. 1660

We are reporting our 9-year experience of cyclic parenteral nutrition with 200 in-patients (mean age 52 years) treated 64 +/- (SEM)3 days (range: 15-230) for Gl diseases. During the first period (A, n = 38), all-in-one nutritive bags with intralipid 10% were used; in a second period (B, n = 135), intralipid 10% was infused separately over the first 6 h of nutrition; in the final period (C, n = 27), Intralipid 20% was used in all-in-one nutritive bags. Indications for parenteral nutrition were non-malignant digestive diseases in 89.5% of the cases. The frequency of complications appearing during cyclic parenteral nutrition was the following: catheter-related sepsis 8%, catheter obstruction 8%, hypertriglyceridemia 33%, hypercholesterolemia 14.7%, liver function test abnormalities 28% and biliary sludge and/or lithiasis 31.6%. 5 patients, with chronic obstructive small bowel disease, developed jaundice with no identifiable cause other than parenteral nutrition. The prevalence of catheter obstruction significantly decreased (P < 0.01) during period B, as compared with periods A and C. The prevalence of liver function test abnormalities decreased significantly (P < 0.01) during periods B and C, as compared with period A. Mortality rate related to cyclic parenteral nutrition was 3%. These results suggest that a) the separate infusion of lipid emulsion reduces the prevalence of catheter obstruction; b) as compared with 10% intralipid in all-in-one nutritive bags, the separate infusion of 10% Intralipid or the 20% Intralipid given in all-in-one nutritive bags is associated with a decreased prevalence of liver function test abnormalities; c) chronic small intestine obstruction seems to play a key role in parenteral-associated jaundice.
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PMID:Cyclic parenteral nutrition in hospitalized adult patients: a 9-year experience. 1684 48

We evaluated 131 patients (6 months-14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n=12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx.
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PMID:Factors influencing outcome after intestinal transplantation in children. 1690 49

Necrotizing enterocolitis (NEC) is an inflammatory intestinal disorder that affects 2%-5% of all premature infants. Enterobacter sakazakii, a common contaminant of milk-based powdered infant formula, has been implicated as a causative agent of sepsis, meningitis, and NEC in newborn infants, with high mortality rates. However, the role played by E. sakazakii in the pathogenesis of NEC is, to date, not known. Here, we demonstrate for the first time that E. sakazakii can induce clinical and histological NEC in newborn rats. E. sakazakii was found to bind to enterocytes in rat pups at the tips of villi and to intestinal epithelial cells (IEC-6) in culture, with no significant invasion. Exposure to E. sakazakii induced apoptosis and increased the production of interleukin-6 in IEC-6 cells and in the animal model. These data suggest that E. sakazakii could be a potential pathogen that induces NEC and triggers intestinal disease by modulating enterocyte intracellular signaling pathways.
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PMID:Enterobacter sakazakii enhances epithelial cell injury by inducing apoptosis in a rat model of necrotizing enterocolitis. 1858 83

Several studies have suggested that T cell-producing permeability factors might lead to proteinuria in minimal change nephrotic syndrome (MCNS). However, it is still unclear whether T-cell abnormalities cause MCNS. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder of the immune regulation system, which leads to severe autoimmune phenomena including autoimmune enteropathy, atopic dermatitis with high levels of serum immunoglobulin E (IgE), type 1 diabetes mellitus (T1DM), and severe infection such as sepsis, which frequently result in death within the first 2 years of life. This disease is caused by mutations in the FOXP3 gene that result in the defective development of regulatory T (Treg) cells. This report describes a 5-year-old boy with IPEX syndrome with a 3 bp deletion in the FOXP3 gene (c.748-750delAAG, p.250K.del) and a paucity of CD4(+) CD25(+) FOXP3(+) T cells. The boy's condition was complicated by MCNS in addition to many IPEX-related manifestations, such as atopic dermatitis, T1DM, enteropathy, sepsis and hemolytic anemia. This is the first report of IPEX syndrome complicated by MCNS, and our findings imply that Treg cell dysfunction may be crucial for the development of MCNS.
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PMID:Minimal change nephrotic syndrome associated with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. 1918 34


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