Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From March 1988 to March 1990, 11 children with cystic fibrosis (age 5-15 years) underwent combined heart-lung transplantation at our institutes. Maintenance immunosuppression consisted of cyclosporin and azathioprine with corticosteroids and antithymocyte globulin used perioperatively and during rejection episodes. Six patients (55%) survive from 1.5-23 months all of whom have improved life quality. Actuarial survival to 1 year was 55%. At six months after transplant, mean forced expiratory volume at one second was 73.5% of predicted normal, compared with 25% before transplant. There was one perioperative death, three later deaths associated with obliterative bronchiolitis at two, eight, and nine months, and one from mediastinitis at four months. Of the 15 children accepted for transplantation but not receiving grafts, 10 have died (eight within four months of being placed onto the transplant list). Early postoperative problems included acute reversible rejection (n = 10), meconium ileus equivalent (n = 3), and pancreatitis (n = 1). There was a high incidence of later pulmonary rejection with a mean of 5.7 episodes per patient in the first six months. Pulmonary infection occurred relatively infrequently, with Pseudomonas aeruginosa being the most common pathogen. Persistent diabetes mellitus requiring insulin occurred in four and systemic hypertension developed in one.
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PMID:Heart-lung transplantation for cystic fibrosis. 2: Outcome. 192 6

Modern cephalosporins are of considerable importance for the therapy of severe infections by multiresistant organisms. According to in-vitro-findings on ampicillin-resistant E. coli as well as Klebsiella spp., Proteus spp., and serratia spp., altogether 159 strains, instead of cefotaxime nearly always also cefotiam can be used. The two remedies are clearly superior to cephalothin. cefotiam is ineffective to Pseudomonas aeruginosa. But in this case also cefotaxime is clearly inferior to azlocillin. In 6 of 7 casuistic instances the clinical effectiveness of cefotiam could be confirmed with good tolerability. The contemporary establishment of staph. aureus in mixed infections of serratiastaphylococci proved as as particular advantage. A primary therapeutic failure referred to a necrotizing pancreatitis, when no causative organism was proved, in which case also cefotaxime remained without any effect. Despite the improved individual medical possibilities the control of the infectious hospitalism by critical administration of antibiotics and improved hospital hygiene, particularly strict non-infection, must remain the pre-eminent task.
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PMID:[Microbiologic and clinical significance of cefotiam]. 385 92

Hypotension is a serious problem in septic patients. We investigated regional perfusion in several organs during treatment of hyperdynamic sepsis in sheep. Sepsis was induced and maintained for the entire experiment with a continuous infusion of live Pseudomonas aeruginosa. Treatment with either norepinephrine or the nitric oxide synthase inhibitor L omega-mono-methyl-arginine (L-NMMA) was begun after 24 h of sepsis and continued for 24 h. The norepinephrine dosage was adjusted to achieve the same increase in mean arterial pressure as that obtained by a fixed dose of L-NMMA (7 mg/kg/h). Blood flows were analyzed by the microsphere technique. Both compounds restored blood pressure effectively, but only L-NMMA caused a significant increase in systemic vascular resistance, concomitant with a significant fall in cardiac output. Sepsis caused an increase in myocardial blood flow and a redistribution of blood flow away from the pancreas and the stomach. Renal blood flow was not significantly elevated. During treatment with either compound, renal blood flow remained unchanged, despite a fall in cardiac output in the L-NMMA group. Unchanged renal blood flow combined with the restoration of arterial blood pressure caused a significant increase in urine output. Both L-NMMA and norepinephrine caused a redistribution of blood flow to the colon. Pancreatic blood flow was further reduced by L-NMMA but the oxygen extraction improved simultaneously, so that oxygen availability in the pancreas might have been unchanged. Because ischemic pancreatitis in sepsis is likely to trigger multiorgan failure, further investigations in that area are desirable.
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PMID:Nitric oxide synthase inhibition versus norepinephrine in ovine sepsis: effects on regional blood flow. 915 93

The most important risk factor in patients suffering from acute necrotizing pancreatitis is pancreatic infection, a factor that determines the course of the disease, its therapeutic management, and its outcome. The bacterial infection route is very likely via the colon. In patients with acute pancreatitis, the infection rate is about 40 to 70% within the first 3 wks. Bacteria most frequently found are those from the gastrointestinal tract: Escherichia coli, Pseudomonas species, Streptococcus fecalis, Enterococcus, and Staphylococcus aureus. Screening methods for infected necrotizing pancreatitis include fine needle puncture by ultrasonography or computed tomographic guidance with Gram staining and culture of the aspirate. We previously investigated different broad-spectrum antibiotics with regard to their efficacy at preventing infection. This analysis indicated that antibiotics have different efficacy factors based on pharmacodynamic properties. Imipenem and quinolones, in combination with metronidazole, are the drugs of choice for treating or preventing pancreatic infection, whereas aminoglycosides do not enter the pancreas and therefore are not indicated. Based on increasing evidence that patients with acute necrotizing pancreatitis will benefit by early and appropriate antibiotic therapy, we altered the approach in such patients with an immediate start of antibiotic therapy continued for at least 14 days. We have found a reduction of the infection rate to 33% (11/32) in the third week after the onset of the disease. This treatment of the infection and the possibility of delaying operative intervention resulted in optimal surgical conditions. However, further prospective, controlled, and randomized studies are necessary to determine which antibiotics and antimycotic therapeutic regimens should be chosen.
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PMID:Infections complicating pancreatitis: diagnosing, treating, preventing. 965 15

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations of the CFTR gene. The number of adult CF patients increased dramatically, since life expectancy is now around thirty years. CF is usually a pediatric disease. In adult patients the disease associate a diffuse bronchectasia with chronic colonisation of sputum with Pseudomonas aeruginosa, and pancreatic insufficiency. Mortality is usually related to respiratory insufficiency. One third of adult patients develop diabetes mellitus. A diagnosis of CF can be made in adult patients particularly when it exists male infertility with congenital absence of vas deferens, chronic sinusitis or diffuse bronchectasia or chronic pancreatitis, acute and recurrent pancreatitis, allergic bronchopulmonary aspergillosis. The diagnosis is established with positive sweat chloride concentration, or double CFTR mutations and/or other suggestive organ involvement.
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PMID:[Cystic fibrosis in adulthood]. 1179 81

Patients with sphincter of Oddi dysfunction have a significantly increased rate of pancreatitis after manometry or sphincterotomy, but septic complications after diagnostic endoscopic retrograde cholangiopancreatography (ERCP) in patients with sphincter of Oddi dysfunction type 2 have not been reported. We describe two patients with sphincter of Oddi dysfunction type 2 in whom Pseudomonas aeruginosa serotype 10 septicemia and multiple small hepatic abscesses developed, all within 48 h after they underwent diagnostic ERCP. The sepsis and hepatic abscesses resolved after successful intravenous antibiotic administration. Despite scrupulous examination of the duodenoscope washing machine and the bottle of water, the bacteria responsible for the sepsis could not be isolated. It is possible that despite disinfection, a nondetectable colony of P. aeruginosa remained in a part of duodenoscope and proliferated to reach a potentially hazardous level the following day. This report highlights the importance administering antibiotic prophylaxis to patients with sphincter Oddi dysfunction type 2 who undergo ERCP, despite the functional nature of the disease.
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PMID:Pseudomonas aeruginosa liver abscesses after diagnostic endoscopic retrograde cholangiography in two patients with sphincter of Oddi dysfunction type 2. 1208 36

Severe impairment of exocrine pancreatic secretion has recently been demonstrated in a clinical study in sepsis and septic shock patients. The purpose of this study was to further evaluate involvement of the pancreas in the acute phase reaction in sepsis. Using a normotensive rat model of Pseudomonas pneumonia-induced sepsis, we assessed the expression of PAP-I, amylase and trypsinogen mRNA, PAPI protein levels, and cytokine expression in the pancreas by Northern and Western blot analysis and RT-M PCR, respectively. Presence of several well-established features of pancreatitis in sepsis-induced animals were examined by biochemical and histopathological methods as well as by a determination of both water and myeloperoxidase content. Sepsis resulted in an up-regulation of PAP-I gene expression and increase in its protein level in pancreas while the mRNA levels of amylase and trypsinogen were down-regulated. Differences in the pancreatic cytokine expression, serum amylase and serum lipase levels, the occurrence of pancreatic edema as well as the severity of inflammatory infiltration and necrosis were not significantly different between sham and pneumonia groups. Acinar cells showed increased vacuolization in pneumonia animals 24 hours after the treatment. These findings demonstrate that the pancreas is actively involved in the acute phase reaction in sepsis of remote origin. This involvement occurs without concomitant biochemical and histopathologic alterations observed in pancreatitis. Taken all together, these features are indicative of a sepsis-specific dysfunction of the pancreas.
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PMID:Pseudomonas pneumonia-mediated sepsis induces expression of pancreatitis-associated protein-I in rat pancreas. 1521 Nov 9

D1152H is a type IV cystic fibrosis transmembrane regulator (CFTR) mutation associated with abnormal chloride gating. Although comprising 5-6% of mutations on genetic screening, clinical reports of cystic fibrosis (CF) are rare, suggesting that the disease is mild, atypical, or even absent. We describe our experience, which contrasts with this assumption, in a retrospective case series encompassing 91 CF patients (74 Jewish) aged 8 months to 56 years, from 2000-2005. Nine patients of varied Jewish ethnic origins were homozygous (2 patients) or compound heterozygous for D1152H with 11 of 182 potential alleles (6%). Five were diagnosed at age 33-49 years. Of 4 infants, 1 was diagnosed by prenatal screening, 1 had a prenatal dilated bowel, and 1 had pulmonary symptoms. Sweat chloride was 28-120 meq/l. Three adults had chronic mucoid Pseudomonas aeruginosa in sputum, and a forced expired volume in 1 sec (FEV1) of 20-55%. One was on bilevel positive airway pressure (BIPAP) ventilation. The infants had pulmonary symptoms that responded well to therapy. All 9 patients had good nutrition, 6 were pancreatic-sufficient, and 3 adults had subclinical pancreatic insufficiency. Three adults had recurrent pancreatitis. None had a bowel obstruction. Two of 3 adult males were fertile. Although asymptomatic at times, the D1152H mutation is associated with a broad clinical spectrum. This information is crucial for genetic counseling. Lung disease may be evident from infancy, and is severe in some adults, although all have outlived the median life expectancy of CF. Hopefully, with early diagnosis and therapy, prognosis can be good. A multicenter study of this mutation is warranted.
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PMID:Cystic fibrosis mutations with widely variable phenotype: the D1152H example. 1642 25

The incidence of pancreatitis in patients with haematopoetic neoplasms who are treated with L-asparaginase is fom 2 to 24%. In majority of cases the pancreatitis is oedematous and self-limiting. Acute haemorrhagic or necrotizing pancreatitis caused by L-asparaginase is rare but potentially life-threatening complication. We present 2 cases of acute pancreatitis in children aged 2 and 4 years. They were diagnosed to have acute lymphoblastic leukaemia and were treated according to the ALLLIC BFM 2002 protocol. Acute pancreatitis developed in these children after induction therapy and was followed by formation of a pseudocyst. In both cases the diagnosis of this complication was made directly after phase I of the protocol I (after eighth dose of L-Asparaginase). In the first case the course of acute pancreatitis was mild. Normalization of the amylase levels occurred after 7 days and the diagnosis of post inflammatory cyst was made 15 days after the first signs of the disease. But thereafter, during the additional complication (pneumonia with Pseudomonas aeruginosa bacteriemia) the pancreatic cyst became infected. In the second case acute pancreatitis had a severe course and the child required treatment in the Intensive Care Unit for 21 days. The cyst was diagnosed after 20 days from the beginning of symptoms. The surgical procedure, applied in both cases was internal drainage by anastomosis of the cyst with the back wall of the stomach. Antileukaemic treatment was recommenced after 6-8 weeks when complications resolved. Currently both children are well and remain in haematological remission and continue maintenance chemotherapy.
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PMID:[Acute pancreatitis during chemotherapy of acute lymphoblastic leukaemia complicated with pseudocyst]. 1953 25

Cystic fibrosis (CF) is a common autosomal-recessive inherited disease, which often results in premature death. Due to treatment advances, life expectancy has however continuously improved in recent years. Currently about half of all patients are adults. There are also "atypical" variants of CF with symptoms occurring in late adulthood. CF is caused by a mutation in the gene coding for a chloride ion channel, known as the cystic fibrosis transmembrane conductance regulator (CFTR). This mutation results in abnormally viscous mucosal secretions, leading to multi-organ disease with particular emphasis in the respiratory and digestive tracts. Impaired mucociliary clearance results in bacterial colonization of the airways (e. g. Pseudomonas aeruginosa) and consequently in chronic pulmonary inflammation, inevitably leading to progressive bronchiectasis and combined ventilatory disorders. Typical acute complications are infective exacerbations - the most frequent cause of death in cystic fibrosis - along with allergic bronchopulmonary aspergillosis, haemoptyses and pneumothoraces. Involvement of the gastrointestinal tract generally manifests as exo- and later endocrine pancreatic insufficiency with diabetes mellitus, malabsorption and sometimes biliary liver cirrhosis. Typical acute complications are pancreatitis and ileus. The article describes epidemiology and pathophysiology of CF and focuses on the signs and symptoms, as well as the diagnostic and multi-modal therapeutic strategies used in adult patients.
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PMID:[Cystic fibrosis in adults]. 2012 4


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