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)

Familial visceral neuropathy is a rare cause of chronic intestinal pseudo-obstruction. It is characterized by progressive destruction of the gastrointestinal myenteric plexus resulting in dysmotility and associated early satiety, post-prandial bloating, recurrent nausea and vomiting, abdominal distension, chronic diarrhea, weight loss, and malnutrition. In its varying forms, there may be neuronal destruction in other parts of the peripheral and central nervous system. We report on four siblings who presented in their third or fourth decades with initial clinical features of chronic intestinal pseudo-obstruction and eventual progressive diffuse neuronal disease, characterized by leukoencephalopathy and peripheral neuropathy. Within 5 yr of presentation, all four patients died from inanition and sepsis, despite aggressive nutritional support. Their clinical and pathological features are characteristic of familial visceral neuropathy of the autosomal recessive form. This presentation may represent a unique syndrome characterized by a tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.
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PMID:Familial visceral neuropathy as part of a diffuse neuronal syndrome: four fatal cases in one sibship. 817 58

Clinically significant anastomotic strictures usually only occur with very low colorectal anastomoses below the level of the peritoneal reflection. The reported rate averages 8 percent and has been attributed to tissue ischemia, localized sepsis, anastomotic leak, proximal fecal diversion, radiation injury, inflammatory bowel disease, and recurrent rectal cancer. Most patients will have symptoms of obstipation, frequent small bowel movements, and bloating. Symptomatic strictures are often approached by dilation (balloon or Hegar) or less often repeat resection. Many of these patients have anastomoses that are too low to consider repeat resection. Strictureplasty with linear stapling devices, stricture resection by use of the circular stapling device, and repeat dilations have all been described. Steroid injections into the stricture have been described in strictured esophagogastric anastomoses but have not been commonly used for strictured coloproctostomies. We describe three cases of coloanal stricture following resections that were complicated by postoperative pelvic abcesses, anastomatic leaks, and pelvic fibrosis. Two cases had undergone low coloanal anastomosis that was protected by a loop ileostomy and developed as significant stricture in the early postoperative period. The third case was managed without a protective loop ileostomy. These were initially managed by repeated dilation of the anastomosis. Each episode was followed by rapid recurrence of the stricture. All patients underwent subsequent dilation with injection of 40 mg of triamcinolone acetate (divided dose in four quadrants) into the stricture and subsequent complete resolution of the stricture. Those patients with loop ileostomies had them taken down and all have been followed for up to 12 months without clinical or endoscopic evidence of recurrent stricture.
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PMID:The strictured anastomosis: successful treatment by corticosteroid injections--report of three cases and review of the literature. 1574 75

A diverticulum is a bulging sack in any portion of the gastrointestinal tract. The most common site for the formation of diverticula is the large intestine. Small intestine diverticular disease is much less common than colonic diverticular disease. The most common symptom is non-specific epigastric pain and a bloating sensation. Major complications include diverticulitis, gastrointestinal bleeding, acute perforation, pancreatic or biliary (in the case of duodenal diverticula) disease, intestinal obstruction, intestinal perforation, localized abscess, malabsorption, anemia, volvulus and bacterial overgrowth. We describe the clinical case of a 65-year-old female patient with a diagnosis on hospital admittance of acute appendicitis and a intraoperative finding of diverticular disease of the small intestine, accompanied by complications such as intestinal perforation, bleeding and abdominal sepsis. This was surgically treated with intestinal resection and ileostomy and a subsequent re-intervention comprising perforation of the ileostomy and stomal remodeling. The patient remained hospitalized for approximately 1 month with antibiotics and local surgical wound healing, as well as changes in her diet with food supplements and metabolic control. She showed a favorable clinical evolution and was dismissed from the hospital to her home. We include here a discussion on trends in medical and surgical aspects as well as early handling or appropriate management to reduce the risk of fatal complications.
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PMID:Diverticular disease of the small bowel. 2318 48

Hepatic portal venous gas (HPVG) has rarely been reported in patients undergoing chemotherapy. We encountered a case of a 64-year-old man with stage IIIA lung adenocarcinoma who received adjuvant chemotherapy with pemetrexed and carboplatin and developed HPVG 1 day after the second chemotherapy. An emergency operation was performed, but the patient died 2 days after the operation because of multiple organ failure caused by sepsis. Since the patient had complained of alternating abdominal bloating and diarrhea during chemotherapy, we considered that the cause of HPVG was intestinal mucosal disruption and increased intraluminal pressure due to the chemotherapy.
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PMID:A Case of Hepatic Portal Venous Gas in a Patient Treated with Pemetrexed and Carboplatin for Lung Cancer. 2572 May 67

Chylomicron retention disease (CMRD), also known as Anderson's disease, is an autosomal recessive condition with a genetic mutation in the secretion associated Ras related GTPase 1B (SAR1B) gene, a protein coding gene. CMRD classically manifests as steatorrhea, vomiting, failure to thrive or abdominal bloating shortly after birth or in childhood. Here, we report a rare case of a 50-day-old male infant who was, at first, overseen as a case of acute gastroenteritis with sepsis owing to the non-specific symptoms i.e. multiple episodes of loose stools with a low-grade fever and failure to thrive, and was managed accordingly. However, the symptoms did not resolve; moreover, the clinical condition deteriorated. Later, lipid profile, clinical presentation and pathological features led to a presumptive diagnosis of CMRD. Our patient showed significant improvement when treated with a trial of medium- and short-chain fatty acids. We conclude that, in resource-restricted countries, a therapeutic trial with the dietary changes is essential to not only prevent the devastating complication but also support the diagnosis.
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PMID:Chylomicron Retention Disease in A Male Infant: A Rare Case from Pakistan. 3225 23

BACKGROUND Diverticulosis and its complication of diverticulitis is a common condition that can be found in up to 35% of the population. Giant colonic diverticulum is a rare entity with fewer than 200 cases reported in the scientific literature. Development of a giant diverticulum as a sequelae of laparoscopic washout is an unreported event in current literature. CASE REPORT The patient was a 74-year-old female who had a well-known history of diverticulosis and diverticulitis. She developed perforated sigmoid diverticulitis, underwent laparoscopic washout and recovered without colon resection. Within a year after washout, she developed abdominal distention and bloating, and computed tomography (CT) imaging revealed a giant diverticulum. She went on to undergo surgery for resection of her sigmoid colon, which contained the giant diverticulum. Her recovery was otherwise uneventful. CONCLUSIONS To our knowledge, this is the first case report of giant diverticulum presenting as a complication of abdominal washout for management of acute diverticulitis. Initial CT scan performed at the time of perforation did not demonstrate this diverticulum, indicating that it developed within the year after abdominal washout for sepsis and acute rupture, likely due to weakening of the colonic wall secondary to ongoing inflammation. The very rare presentation of giant diverticulum makes it difficult to establish a clear link to washout, however, this case establishes a groundwork for further investigation as our fund of knowledge on the subject continues to grow.
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PMID:Surgical Management of Giant Colonic Diverticulum Presenting After Laparoscopic Lavage for Diverticular Perforation and Sepsis. 3271 6