Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute respiratory distress syndrome (ARDS) - is a life-threatening acute clinical syndrome of pulmonary insufficiency with high mortality. The causes of the syndrome are of every description - from crustacean poisoning to cardiopulmonary bypass. The rate of ARDS is not clear, because of diagnostical variety of ARDS and acute pulmonary dysfunction. The discussion on ARDS diagnostical criteria lasted for many years. The clinical criteria depend on how the essence of the disease is understood, on the size of lung infiltration, lung compliance and failure as well as degree of hypoxemia. The risk of ARDS increases with the increase of number of predisposing factors. The investigation data depend mainly on used datum-point for diagnostics of ARDS. It is obvious that non-cardiogenic light pulmonary edema is found very often, and serious lung lesions are rare enough. The aim of nowadays treatment is to cure the disease that causes the syndrome and to sustain vital functions. The early diagnostics of ARDS predisposing factors and minimization of their influence, the prevention, early diagnostics and timely treatment of complications are essential. In many cases ARDS caused by collateral factors is the constituent of multiple organ dysfunction syndrome. Anyway the ARDS predisposed by direct factors is often complicated by other organ (cardiovascular, renal, hepatic, hematogenous, central nervous system, gastrointestinal tract, etc.) dysfunction. In this case the treatment becomes more difficult and includes therapy correcting the function of other systems. In spite of intensive treatment, mortality of this syndrome still remains 50-90% according to the literature data. Though some authors state that recently the ARDS mortality decreased, but most of the authors did not notice any improvement during the last 20 years. The prognosis is determinated not only by pulmonary insufficiency itself (the cause of death in 5% of patients), but by the ARDS predisposing factor (the worst is sepsis and septic shock), multiple organ dysfunction syndrome, difficult physical state of the patient, sepsis as a cause or as a complication of ARDS, nosocomial pneumonia, progressive fibroproliferation in the lung. Considering the actuality of ARDS, the diagnostical criteria of ARDS, pathogenesis, clinical course and new treatment methods are reviewed in the publication.
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PMID:[Acute respiratory distress syndrome]. 1464 57

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton tyrosine kinase (BTK) that result in the deficient development of B lymphocytes and hypogammaglobulinemia. Because the disorder is uncommon, no single institution has had sufficient numbers of patients to develop a comprehensive clinical picture of the disorder. Accordingly, a national registry of United States residents with XLA was established in 1999 to provide an updated clinical view of the disorder in a large cohort of patients. A total of 201 patients were registered by 66 physicians. The estimated birth rate for the 10-year period of 1988-1997 was 1/379,000. Infection was the most common initial clinical presentation (85%), followed by a positive family history (41%) and neutropenia (11%). Although the average age of diagnosis was younger in patients with a positive family history (mean, 2.59 yr) than in patients with a negative family history (mean, 5.37 yr) (p < 0.001), only 34.5% of patients with a positive family history at the time of their birth were diagnosed before clinical symptoms developed-that is, based on family history alone. Seventy percent of patients had at least 1 episode of otitis, 62% at least 1 episode of pneumonia, 60% at least 1 episode of sinusitis, 23% at least 1 episode of chronic/recurrent diarrhea, 21% at least 1 episode of conjunctivitis, 18% at least 1 episode of pyoderma and/or cellulitis, 11% at least 1 episode of meningitis/encephalitis, 10% at least 1 episode of sepsis, 8% at least 1 episode of septic arthritis, 6% at least 1 episode of hepatitis, and 3% at least 1 episode of osteomyelitis. Fourteen of 201 (6.9%) patients were dead at the time they were entered in the Registry. However, in a prospective 4 /4-year follow-up of living patients, only 3/80 (3.75%) patients died. Causes of death included disseminated enterovirus infection (n = 6), pulmonary insufficiency (n = 5), adenovirus infection (n = 1), sepsis (n = 1), acquired immunodeficiency disease syndrome (AIDS) (n = 1), myocarditis (n = 1), hepatitis (n = 2), and stem cell transplantation (n = 1).
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PMID:X-linked agammaglobulinemia: report on a United States registry of 201 patients. 1686 44

Cholestasis progressing to end-stage liver disease (ESLD) is common in intestinal failure (IF) in infants. Isolated liver transplantation (OLT) is performed when eventual enteral sufficiency is expected. We reviewed our experience with OLT for ESLD in patients with residual IF. From 1998 to 2004, four IF patients underwent OLT for ESLD at our institution. Three were performed as UNOS status I for acute decompensation of chronic liver failure; one other with severe cholestasis with a living donor. The living donor recipient died within months after OLT of chronic respiratory failure despite normal liver function. One recipient remains on parenteral nutrition (PN) and continues to receive partial enteral feeds with normal liver function. Two other recipients became enterally sufficient after the OLT. Biliary complications occurred in two patients, one with late hepatic artery thrombosis. Resolution was achieved with serial balloon dilatations and the other by conversion from duct-to-duct anastomosis to a choledochoduodenostomy. The morbidity of OLT for this indication is higher than for others likely because of comorbidities like sepsis and pulmonary insufficiency. OLT is complicated further by the attempt to maximally preserve residual bowel length for the biliary reconstruction. OLT can be an emergent life-saving procedure in IF patients despite higher morbidity. Improved liver function and diminished portal pressures may shorten the time to enteral sufficiency. Management of parenteral nutrition after OLT can avoid ESLD and eliminate small bowel transplantation in selected patients.
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PMID:Isolated liver transplantation for decompensated end-stage liver disease in children with intestinal failure. 1690 53


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