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

Neonatal seizures in the neonatal period are symptoms of numerous underlying disorders of the neonate. We present a case in which neonatal seizures due to cerebral infarction led to a diagnosis in the mother. Neonatal convulsions caused by cerebral artery thrombosis is relatively rare in the neonatal period and is often secondary to indwelling intravascular catheters that cause thromboembolism, but may be associated with many conditions.1 Cerebral artery thrombosis in newborns, in which antiphospholipid antibodies (APA) were found in the mother, has been described in three case reports. Two of these premature infants were born with other risk factors for thrombosis. APA could not be identified in any of these three infants. In the two cases reported by Silver et al the diagnosis was made several months after birth. This case is unique in the fact that no other risk factors for thrombosis could be identified to explain the infarction, and that APA were found in the offspring of an apparently healthy mother. Whether the prior fetal death was caused by APA remains unclear. The finding of lupus anticoagulant in her child led to the diagnosis of antiphospholipid antibody syndrome in her. We believe that in case of cerebral artery thrombosis in a neonate, with no trivial cause such as an indwelling catheter or sepsis, both mother and infant should be tested for presence of APA, even when the mother seems healthy.
 ...
PMID:An unusual cause of neonatal seizures in a newborn infant. 931 May 41

We describe here the broad spectrum of acute renal insufficiency occurring in the course of human immunoinsufficiency virus infection. In our renal unit in Tenon hospital, 90 human immunoinsufficiency virus-infected adult patients were admitted for acute renal insufficiency between June 1988 and December 1996. Sixty out of them had a pathological diagnosis. The remaining patients did not have renal biopsy because of obstructive renal failure (n = 2), bleeding risk (n = 11), or clinically evident hypovolemic and/or sepsis-related acute tubular necrosis (n = 17). Nine different causes of acute renal insufficiency were listed. Human immunoinsufficiency virus-associated nephropathy, the most specific human immunoinsufficiency virus-related renal disease, which was diagnosed in 14 patients, is characterized by focal and segmental glomerulosclerosis with an important hyperplasia and/or proliferation of podocytes and huge tubular distension. The rapid progression to end-stage renal failure was not a constant feature since 10/14 patients had a partial renal recovery. Hemolytic-uremic syndrome was the other major cause of acute renal failure in these patients (32 cases) and was found to be associated with active cytomegalovirus infection. Cytomegalovirus-infected cells were present in half of the renal biopsies performed in this group of patients. Furthermore, these patients had an increased plasma tissue-type plasminogen activator activity whereas its type 1 inhibitor was not significantly increased, as opposed to non human immunoinsufficiency virus-associated hemolytic-uremic syndrome. Half of the patients had a complete renal recovery. The other causes of acute renal insufficiency were 1) intratubular deposition of either drugs (Adiazine, Foscavir, Indinavir) in 13 patients, or monoclonal light chain in one patient with B cell-lymphoma; 2) lupus-like glomerulonephritis characterized in one case by a complete clinical remission after 6 month-treatment by antiproteases; 3) acute tubular necrosis. In this setting, rhabdomyolysis could reveal HIV infection. The heterogeneity of renal diseases could be explained by the variation of human immunoinsufficiency virus-associated infections along time and by the different drugs which permit a better survival. We can hypothesize that new HIV-associated diseases will occur with the long term use of antiproteases.
 ...
PMID:[Human immunodeficiency virus and acute renal insufficiency]. 961 98

Significant advances have been made in the capabilities to remove white blood cells (WBCs) from blood by both centrifugal and filtration techniques. New techniques have applications for both donor products (and their effects upon the recipients) and for selected disease therapeutics. The immunomodulatory effects of donor WBCs may be therapeutic, e.g., granulocytes harvested by apheresis may be used for the treatment of sepsis, or mononuclear cells collected by apheresis for peripheral blood progenitor (stem) cell transplantation or graft versus leukemia effect. In contrast, WBCs are removed from many transfusable components to decrease the immune effects in recipients. This has been accomplished primarily by the use of leukoreduction filters although newer adaptations of centrifugal equipment allow for the reduction of WBCs to target range of <1 x 10(6) WBCs/product. Therapeutic WBC removal by centrifuge has been used for treatment of the effects due to elevated levels of WBCs or platelets. More specific cellular immunotherapy has included lymphocytapheresis for the treatment of autoimmune diseases such as systemic lupus erythematosis (SLE). Various mononuclear cell fractions collected by apheresis have been used for lymphokine activated killer cells (LAK) and tumor infiltrating lymphocytes (TIL) cell therapy or autologous stem cell transplantation. The development of WBC adsorbent filters for therapeutic use has evolved as nonspecific filter materials have been demonstrated to show selective WBC removal, and filter columns permit therapeutic reductions in WBCs using online filtration therapy. Specific adsorption techniques, e.g., CD-34 selection, are in use in vitro and indicate directions for further developments in cellular immunotherapy.
 ...
PMID:Apheresis techniques and cellular immunomodulation. 1022 38

Although the short- and medium-term (5-10 years) outcome of patients with lupus nephritis has been studied extensively, there are very few data on the second and subsequent decades. We studied outcome in 110 local patients investigated at a single centre before 1986, who all had potential follow-up of more than 10 years (actual 2-31 years, median 15.5 years). At last follow-up, 40 patients were dead and 70 alive, nine of whom were on maintenance dialysis or transplanted, actuarial survivals being 84%, 72%, 62%, 61% and 54% at 5, 10, 15, 20 and 25 years for the group as a whole. Survival was better in the cohort 1976-86 (n = 60) than in that from 1963-75 (n = 50) (90, 81 and 76% vs. 78, 56 and 43% at 5, 10 and 15 years, p < 0.001). Sepsis (12) and myocardial infarction (8) were the principal causes of death. Of living patients with renal function, 38% had normal urine and renal function, 11 were off all treatment (19%), 62% had persistent proteinuria and 18% had reduced but generally stable renal function. Renal failure, in those patients who developed it, occurred during the first decade and none of 67 patients actually followed more than 10 years subsequently went into renal failure. Induction treatment was with prednisolone, combined with azathioprine in more severe forms of nephritis, and from the middle 1970s to 1986, 30 with methylprednisolone and in 12 cases plasma exchange. Seventeen other patients were treated using oral cyclophosphamide during the 1960s. No patient received i.v. cyclophosphamide as induction therapy, although nine patients had this form of treatment later, largely because of non-compliance. Serious complications of lupus and/or its treatment occurred in 49%: sepsis in 32, ischaemic heart disease in 20, thrombosis in one and avascular necrosis of bone in eight. In contrast, fracturing osteoporosis occurred in only three, and cataracts requiring surgery and diabetes mellitus in none. The very long-term outlook of lupus nephritis, especially its more severe forms, has improved, but that with current management strategies only a minority of patients are able to stop treatment altogether, and the incidence of serious complications is high.
 ...
PMID:The very long-term prognosis and complications of lupus nephritis and its treatment. 1039 9

Reactive haemophagocytic syndrome is characterized by activation and uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine overproduction, which accounts for the main biological signs. Children usually present with an acute febrile illness, fulminant pancytopenia and hepatosplenomegaly, posing a problem of differential diagnosis with severe sepsis. Hemopoietic cells are actively ingested by monocytes/macrophages in various organs, including lymph nodes, bone marrow, liver and spleen. This exarcerbation of the histiocytic system is currently classified among the reactional histiocytoses. It reflects an inappropriate host immune response. Most patients have a known underlying disease (hemopathy, lupus, systemic juvenile arthritis, HIV infection). In the few cases that occur in the apparent absence of any risk factors, investigations should be made to look for predisposing inherited diseases, such as familial lymphohistiocytosis or Purtilo's disease in boys. The treatment rests on immunosuppressive agents, followed by bone marrow transplantation, which can provide a definitive cure in genetic forms.
 ...
PMID:[Reactive hemophagocytic syndrome in children]. 1076 6

Vibrio vulnificus infection with septicemia is a life threatening disease in the immunocompromised hosts. Renal involvement has not been documented. We reported herein 8 patients with V. vulnificus septicemia. All were immunocompromised hosts. Four patients had cirrhosis of the liver, 3 were heavy alcohol drinkers and one had systemic lupus erythematosis. Presenting symptomatology included fever, chills, leg pain and skin rash. Renal failure was observed in 6 patients. Four patients died shortly after admission. Two survived with clinical course of tubular necrosis. Renal failure is therefore common in V. vulnificus infection. This should be brought to attention, and vigorous antibiotic treatment is required. The disease may be confused with leptospirosis, scrub typhus, malaria and other forms of sepsis which also present with renal failure.
 ...
PMID:Renal failure in vibrio vulnificus infection. 1084 44

A registry of United States residents with chronic granulomatous disease (CGD) was established in 1993 in order to estimate the minimum incidence of this uncommon primary immunodeficiency disease and characterize its epidemiologic and clinical features. To date, 368 patients have been registered; 259 have the X-linked recessive form of CGD, 81 have 1 of the autosomal recessive forms, and in 28 the mode of inheritance is unknown. The minimum estimate of birth rate is between 1/200,000 and 1/250,000 live births for the period 1980-1989. Pneumonia was the most prevalent infection (79% of patients; Aspergillus most prevalent cause), followed by suppurative adenitis (53% of patients; Staphylococcus most prevalent cause), subcutaneous abscess (42% of patients; Staphylococcus most prevalent cause), liver abscess (27% of patients; Staphylococcus most prevalent cause), osteomyelitis (25% of patients; Serratia most prevalent cause), and sepsis (18% of patients; Salmonella most prevalent cause). Fifteen percent of patients had gastric outlet obstruction, 10% urinary tract obstruction, and 17% colitis/enteritis. Ten percent of X-linked recessive kindreds and 3% of autosomal recessive kindreds had family members with lupus. Eighteen percent of patients either were deceased when registered or died after being registered. The most common causes of death were pneumonia and/or sepsis due to Aspergillus (23 patients) or Burkholderia cepacia (12 patients). Patients with the X-linked recessive form of the disease appear to have a more serious clinical phenotype than patients with the autosomal recessive forms of the disease, based on the fact that they are diagnosed significantly earlier (mean, 3.01 years of age versus 7.81 years of age, respectively), have a significantly higher prevalence of perirectal abscess (17% versus 7%), suppurative adenitis (59% versus 32%), bacteremia/fungemia (21% versus 10%), gastric obstruction (19% versus 5%), and urinary tract obstruction (11% versus 3%), and a higher mortality (21.2% versus 8.6%).
 ...
PMID:Chronic granulomatous disease. Report on a national registry of 368 patients. 1084 35

We analyzed the clinical and laboratory features, treatment, and course of twenty-one children with systemic-onset juvenile rheumatoid arthritis (S-JRA) encountered at our institution over the past ten years. There were eleven boys and ten girls. The mean age at onset was 11.6 +/- 4.2 years. The mean duration of symptoms prior to diagnosis was 5.5 +/- 1.7 months, and the mean follow-up period was 45.7 +/- 9.5 months. The clinical and laboratory features at presentation were similar to previous reports, except that peripheral blood smear revealed toxic granulation of neutrophils in 60% of our patients. Although systemic manifestation could be readily controlled by non-steroidal anti-inflammatory drugs (NSAIDs) with or without additional steroids, nine patients suffered from chronic arthritis (duration > 6 months) requiring disease-modifying anti-rheumatic drugs (DMARDs). Of the nine children with chronic arthritis, six (67%) had a monocylic systemic course, and seven (78%) had polyarticular disease (five or more joints affected) at the disease onset. Five patients developed severe destructive polyarthritis, with persistent anemia, thrombocytosis, elevated serum C-reactive protein (CRP) levels, and marked functional limitation during follow-up. One of the five patients with severe arthritis developed systemic lupus erythromatosis after 8-year follow-up, and died of sepsis. Our study indicated significant morbidity in children with S-JRA in Taiwan.
 ...
PMID:A follow-up study of systemic-onset juvenile rheumatoid arthritis in children. 1091 Jun 10

The objectives were to determine causes of consultation, hospitalization and outcome in a cohort of lupus patients in an emergency unit. Patients with systemic lupus erythematosus (SLE) who visited the emergency department for consultation from 1 September 1996 to 17 May 1997 were included in the study. They were evaluated during the visit by looking at 100 variables such as demographic, socioeconomic, clinical, therapeutical, behavioral, (compliance), emotional (Beck depression inventory), disease activity, (Mex-SLEDAI), disease severity (Lupus SDI), chronic damage (SLICC-ACR), and physician's and patient's global assessments of severity. All causes of consultation, hospitalization and outcome were registered. Descriptive statistics, univariate analysis and multiple logistic regression were used for analysis. Significance was set at the 0.05 level. 180 patients were included. 164 were female, mean age 31.7/11.39 y, mean Mex SLEDAI score 3.8, mean SLICC-ACR 1.3. Fever, poliarthralgia and abdominal pain were the main causes of consultation with 26, 25 and 18 cases each. 49 patients were hospitalized and these were statistically different than non-hospitalized patients in level of formal education (10.2 vs 11.8, P=0.03); compliance (7.6 vs 9, P=0.0001); malar rash (57% vs 82%, OR, 95% CI=0.28, 0.13-0.62, P=0.0008), chloroquine daily dose intake (45 vs 77 mg, P=0.04); disease severity in physician's global assessments (5.6 vs 2.1, P=0.0001) and Beck depression inventory (21 vs 16, P=0.01). Multiple logistic regression identified physician's global assessment, fewer ACR criteria and higher SLICC-ACR scores as the main variables associated with hospitalization. Five patients died; two with community acquired pneumonia, one with pancreatitis, multiple thromboses, and sepsis, one with pulmonary hemorrhage; and one with pulmonary thromboembolism. In conclusion, poor compliance, low level of formal education, severity, depression, lower ACR criteria and higher SLICC-ACR scores were important variables identified with hospitalization. Chloroquine use seemed to have a protective effect. Causes of death were related to infections and antiphospholipid syndrome.
 ...
PMID:Lupus patients in an emergency unit. Causes of consultation, hospitalization and outcome. A cohort study. 1103 35

The objective of this study was to characterize the clinical profile of lupus patients with non-typhoidal salmonellosis. A retrospective review of the clinical charts of lupus patients diagnosed with bacteriologically proven non-typhoidal salmonellosis over the last 20 y was undertaken, paying special attention to risk factors, clinical presentation and treatment outcome. Most episodes were bacteraemic without a localizing focus; and some patients were afebrile. They usually occurred in patients prone to opportunistic infections, and at times of increased immunosuppression given for lupus flares (especially nephritis). However, salmonellosis also occurred in some patients presenting with lupus. The C-reactive protein level was found to be significantly higher during the infective episodes compared to episodes of non-infective febrile lupus flare. All isolates were sensitive to the usual first-line antibiotics and eminently treatable with 3 weeks of appropriate antibiotics without recurrence/persistence or significant morbidity/mortality, the exceptions being spinal osteomyelitis and septic arthritis involving deformed joints requiring surgical debridement and prolonged antibiotic therapy for eradication. Mortality occurred in the setting of septic shock from mixed-microbial sepsis and major organ failure from active lupus. There is a high association of non-epidemic, non-typhoidal salmonellosis with SLE, especially in patients with active disease on intensified immunosuppression. The C-reactive protein value may be helpful in distinguishing between fever from a pure lupus flare and one complicated by infection.
 ...
PMID:Non-thyphoidal salmonellosis in patients with systemic lupus erythematosus. A study of fifty patients and a review of the literature. 1123 31


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>