Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
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Forty-three patients with fractures of the thoracolumbar spine submitted to surgical treatment using the Harms method (dorsoventral operations) were studied prospectively with a follow-up of at least 12 months and evaluated on the basis of clinical and radiologic parameters and in relation to their professional activities. Thirty-five patients (81.3%) were males and eight (18.7%) females, ranging in age from 17 to 67 years (mean 34.08+/-11.51 years). Seven patients (16.2%) presented fractures of more than one vertebra, and associated lesions were present in 15 patients (34.8%). Monosegmental fixation was performed in 7 patients (16.3%), bisegmental fixation in 29 (67.4%), and trisegmental fixation in 7 (16.3%). No patient was submitted to any type of external immobilization during the postoperative period and all patients were allowed to sit up in bed and to walk as soon as their clinical conditions permitted. Thirty-nine patients were followed up for a period ranging from 12 to 36 months (mean 16.58+/-6.83 months). Four patients died during the postoperative period (three of pulmonary embolism and one of septicemia). Forty-two patients sat up in bed between the 2nd and 6th postoperative day, and those who did not present a disabling lesion (Frankel D or E) or other associated lesions walked between the 4th and 10th postoperative day (mean 6.14+/-6.06 days). The neurological signs and symptoms improved in 16 patients (37.3%), were unchanged in 26 (60.4%), and worsened in 1 (2.3%). Twenty-three patients (87.5%) who had no neurological damage (Frankel E) returned to their professional activities after respective periods of disability of 1 month (three patients), 2 months (four patients), 3 months (one patient), 4 months (seven patients), 5-7 months (five patients), 8-12 months (one patient), and more than 12 months (three patients). The ability to work of the 24 patients without neurological damage was 100% in 21, 50% in 2, and zero in 1. The ability to walk of this group of patients was 1-5 km for 4 and more than 5 km for the remaining 20 patients. The complications observed were death (four patients; three cases of pulmonary embolism and one case of septicemia), infection (two patients), Stevens-Johnson syndrome (one patient), and meningitis (one patient). The mean kyphosis of the fractured segment was 22.17 degrees +/- 10.97 degrees preoperatively, 8.55 degrees +/- 6.9 degrees postoperatively, and 10.30 degrees +/- 8.84 degrees on the occasion of late evaluation. No loss of correction occurred in 28 patients (71.8%), a 5 degrees loss was observed in 3 patients (7.6%), a 6 degrees loss in 3 (7.6%), a 7 degrees loss in 3 (7.6%), and a loss of more than 10 degrees in 2 (5.2%).
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PMID:Treatment of fractures of the thoracolumbar spine by combined anteroposterior fixation using the Harms method. 968 50

Severe drug eruptions are rare, life-threatening events. The management begins with the withdrawal of the suspect drug(s). We recently confirmed that an earlier withdrawal of drugs with short elimination half-life was associated with a better survival of patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). In cases of "acute skin failure" (exfoliative dermatitis, extensive SJS or TEN), management of patients must be undertaken in specialized intensive care units or in burn units. The main principles of symptomatic therapy are the same as for major burns: warming of the environment, correction of electrolyte disturbances, high caloric intake, and prevention of sepsis. The suspected immunologic orgin of drug eruptions prompted the use of corticosteroids, immunosuppressive drugs, and anti-cytokines. Systemic corticosteroids are useful in "hypersensitivity syndrome" when visceral lesions depend on infiltration by activated cosinophils. Systemic corticosteroids were shown to be deleterious in cases of advanced TEN. Their potential usefulness at earlier stages of SJS or TEN remains controversial. High intravenous doses of cyclophosphamide or oral cyclosporin have been administered to a few patients with TEN, most often following ineffective treatment with corticosteroids for 1 to 5 days. It remains doubtful that the progression of the lesions was shortened. A few patients appeared to benefit from treatment with pentoxifyllin, a drug suppressing the production of TNF. Thalidomide, another suppressor of TNF production, significantly increased the death rate when tested in a double-blind placebo controlled trial in patients with early TEN. High dose intravenous immunoglobulins were used in 10 patients with TEN on the basis of their ability to inhibit fas-fas ligand mediated apoptosis. The potential benefit of this treatment needs confirmation by further studies. Patients and their first degree relatives should be advised to avoid the responsible drug and chemically related compounds. Regulatory agencies should be notified of such cases.
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PMID:Treatment of severe drug eruptions. 1063 13

Severe skin adverse drug reactions can result in death. Toxic epidermal necrolysis (TEN) has the highest mortality (30-35%); Stevens-Johnson syndrome and transitional forms correspond to the same syndrome, but with less extensive skin detachment and a lower mortality (5-15%). Hypersensitivity syndrome, sometimes called Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), has a mortality rate evaluated at about 10%. Drug reactions are self-limited diseases and therefore, generally treatment is symptomatic. Prompt diagnosis, identification of, and early withdrawal of all suspect drugs are the most important preliminaries. The management of the patients must be undertaken in specialized intensive care units, with the same main types of therapy as for burns: warming of the environment, correction of electrolyte disturbances, administration of a high caloric enteral intake, and prevention of sepsis. Efficacy of drugs used in some case reports is difficult to evaluate: intravenous immunoglobulins, cyclosporin, cyclophosphamide, pentoxyfilline, and thalidomide have all been tried. Corticosteroid use is debated and is probably deleterious in late forms of TEN. For DRESS, corticoids are used in cases of life-threatening systemic impairment. Specific nursing care and adequate topical management reduce associated morbidity and allow a more rapid re-epithelialization of skin lesions. After healing, follow-up is needed for ophthalmologic and mucous membrane sequelae. Sunblocks are recommended. Testing for glycemia must be done. Avoidance of the responsible drug and chemically related compounds is essential for the patient and first-degree relatives.
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PMID:Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. 1216 15

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare cutaneous diseases marked by substantial epidermal denudation and are often complicated by sepsis and multiple organ failure. They are most commonly caused by drug therapy. Patients afflicted with these diseases require care that may exhaust the capabilities of medicine wards and medical intensive care units alike; however, their mortality is reduced when treated at burn centers, which are better equipped to treat extensive skin denudation. We report a case of TEN and propose an algorithm to provide guidance for making the critical decision to transfer patients with SJS and TEN to burn units.
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PMID:A case report and a proposed algorithm for the transfer of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis to a burn center. 1218 46

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin toxicities that may occur in patients with cancer. They are caused by infection or a drug reaction and can result in sepsis, severe ocular complications, and even death. Skin lesions usually are preceded by prodromal flulike symptoms. A rash with subsequent blistering and denudation follows. Diagnosis is made by skin biopsy, and classification is based on body surface area and visceral organ involvement. Because many of the drugs associated with SJS and TEN are used to treat cancer, early recognition and intervention are critical to achieving a favorable outcome. Interventions include stopping the suspected offending agent. The use of steroids is controversial. Healthcare professionals always should consider an early transfer to a burn unit for patients with a comorbid condition such as cancer because this action is associated with improved outcomes. Implications for oncology nurses and a case study are presented in this article.
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PMID:Stevens-Johnson syndrome and toxic epidermal necrolysis: oncologic considerations. 1498 59

Toxic epidermal necrolysis (TEN) is a life threatening desquamating disease that is often an adverse reaction to drugs. Because mortality is so high, up to 30% nationally, and the morbidity significant, these cases are managed in burn centers. This study was conducted to evaluate what drugs were given to children who developed exfoliating skin disease and to identify the complications that these patients suffered. Thirty-two pediatric cases of erythema multiforme, Stevens-Johnson syndrome (SJS), and TEN were identified during a period of 8 years in which the average number of admissions to the burn center was 200 per year. Age, sex, drug history before admission, drug treatment during hospital stay, and clinical outcomes were noted. Several drugs were identified as probable causative agents. The most common cause of exfoliating disease was a combination of azithromycin and ibuprofen, followed by ibuprofen alone. Notably, the combination of ibuprofen and another drug was responsible for four additional cases, making the total percentage of pediatric cases involving ibuprofen 47%. Although no children died, several children with TEN and SJS suffered severe ocular involvement, sepsis, pneumonia, and genitourinary complications. All of the children who experienced complications had received ibuprofen. Chi-square analysis showed the correlation between ibuprofen and complications to be statistically significant (<0.05). This association was not observed with any other drug administered. Not only is ibuprofen a potential etiologic agent of exfoliating skin disease in children, it also may contribute to the development of complications in pediatric patients with the disease. Although this association does not prove that ibuprofen is the definitive cause of complications in these cases, caution is advised when giving this drug to children with suspected erythema multiforme, SJS, and TEN.
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PMID:Morbidity and mortality of mucocutaneous diseases in the pediatric population at a tertiary care center. 1792 57

Toxic epidermal necrolysis (TEN) is a rare drug-related life-threatening acute condition. Sepsis is the main cause of mortality. Skin colonization on top of impaired barrier function promotes bloodstream infections (BSI). We conducted this study to describe the epidemiology, identify early predictors of BSI, and assess the predictive value for bacteremia of routine skin surface cultures. We retrospectively analyzed the charts of all patients with Stevens-Johnson syndrome (SJS) and TEN hospitalized over an 11-year period. Blood cultures and skin isolates were recovered from the microbiology laboratory database. Early predictors of BSI were identified using a Cox model. Sensitivity, specificity, and negative and positive predictive values of skin cultures for the etiology of BSI were assessed. The study included 179 patients, classified as having SJS (n = 54; 30.2%), SJS/TEN overlap (n = 59; 33.0%), and TEN (n = 66; 36.9%). Forty-eight episodes of BSI occurred, yielding a rate of 15.5/1000 patient days. In hospital mortality was 13.4% (24/179). Overall, 70 pathogens were recovered, mainly Staphylococcus aureus (n = 23/70; 32.8%), Pseudomonas aeruginosa (n = 15/70; 21.4%), and Enterobacteriaceae organisms (n = 17/70; 24.3%). Variables associated with BSI in multivariate analysis included age >40 years (hazard ratio [HR], 2.5; 95% confidence interval [CI], 1.35-4.63), white blood cell count >10,000/mm3 (HR, 1.9; 95% CI, 0.96-3.61), and percentage of detached body surface area >or=30% (HR, 2.5; 95% CI, 1.13-5.47). Skin cultures had an excellent negative predictive value for bacteremia due to S aureus (especially methicillin-resistant strains) and P aeruginosa, but not for those due to Enterobacteriaceae organisms. In contrast, the positive predictive value was low for all pathogens studied.To our knowledge, this is the largest study describing the epidemiology and risk factors of BSI in patients with SJS/TEN. The body surface area involved is the main predictor of BSI. Excellent negative predictive values of skin cultures for S aureus and P aeruginosa bacteremia should help clinicians consider targeted empirical antibiotic choices when appropriate.
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PMID:Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. 2007 2

Although pancreatitis is rare in pediatric burn patients, elevated pancreatic enzymes have been recently observed among toxic epidermal necrolysis (TEN) patients. This clinical phenomenon has implications particularly for the nutritional management of patients involved. The objective of this study was to assess the frequency of sustained, elevated amylase, and lipase enzymes among children with TEN or Stevens Johnson Syndrome (SJS) and to evaluate the utilization of enteral nutrition support in this population. Medical records of 24 patients admitted to our hospital between January 1994 and October 2008 with TEN or SJS were retrospectively reviewed. Only patients with > or =4 consecutive measures for both amylase and lipase were included in this study (n = 10). Serial laboratory values were collected during the first 30 days of disease. Four patients (40%) had elevated amylase and lipase values, whereas six patients had values within normal limits. Patients with elevated pancreatic enzymes were significantly younger in age (4.7 +/- 1.7 years) than patients without elevated enzymes (11 +/- 5.9 years) and also had a higher incidence of sepsis. All other characteristics were similar between the groups. Enteral nutrition support was initiated within 4 days of admission in all 10 patients and did not correlate with elevated enzymes. Our findings suggest that hyperlipasemia and hyperamylasemia can occur in the pediatric population with TEN or SJS. Although the sample size in this study makes it difficult to determine the cause, sepsis may have been a contributing factor. In the absence of symptomatic pancreatitis, patients with TEN can safely meet nutritional goals orally or with standard enteral nutrition support.
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PMID:Asymptomatic hyperamylasemia and hyperlipasemia in pediatric patients with toxic epidermal necrolysis. 2018 82

The use of immunoglobulins in the treatment of infectious diseases has a long tradition. Initially immunoglobulins from hyperimmunised animals were used for their antitoxic and antimicrobial activity. The development of preparations of human intravenous immunoglobulin (IVIG) and the observations of their long-term use enabled to assess their usefulness in the treatment of the diseases of proven or probable infectious etiology. In the treatment of infectious diseases IVIG are currently used as immunomodulating drugs or immunosuppressive therapy, more frequently than the specific antibodies against the viruses, bacteria or their toxins. In practice of the infectious ward IVIG are used as a drug of choice in the treatment of Kawasaki disease, in toxic epidermolysis and Stevens-Johnson syndrome. As adjunctive therapy IVIG are used in the infection with parvovirus B19, in hemophagocytic syndrome, for treatment of infections presenting with a severe toxemia caused by Clostridium difficile, Streptococcus pyogenes and Staphylococcus aureus. The rationale for the use of IVIG may be also serious infections caused by enteroviruses, particularly neuroinfections. The use of IVIG in the treatment of sepsis is controversial, since their effectiveness is not proven.
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PMID:[The use of immunoglobulins in the treatment of infectious diseases]. 2175 56

Stevens-Johnson syndrome (SJS) is a severe and rare immune-mediated cutaneous reaction usually induced by drugs or infections. Few case reports have demonstrated SJS associated with adult systemic lupus erythematosus (SLE), and rarely in juvenile SLE (JSLE) patients. However, to the best of our knowledge the prevalence of this life-threatening cutaneous disease in the pediatric lupus population has not been studied. Therefore, from January 1983 to December 2010, 5508 patients were followed-up at the Pediatric Rheumatology Unit of our University Hospital and 279 (5%) of them met the American College of Rheumatology (ACR) classification criteria for SLE. Only one (0.4%) of our JSLE patients had SJS and was described. This female patient was diagnosed with JSLE at 14 years old. After four years of follow-up, she was hospitalized due to congestive heart failure and renal insufficiency. During hospitalization, the patient developed sepsis with positive blood culture for Stenotrophomonas maltophilia and was treated with vancomycin and meropenem. One week later, she developed septic shock and chest x-ray showed acute widespread pulmonary infiltrate. Antimicrobials were changed to linezolid and trimethoprim-sulfamethoxazole. After four days, the blood culture isolated Staphylococcus aureus resistant to vancomycin, and she presented with erythematous cutaneous lesions involving her face, trunk, and limbs, with evolution in a few hours to diffuse hemorrhagic vesicles and blisters. Epidermal detachment was observed on 5% of the body surface area. Concomitantly, she had conjunctivitis, cheilitis, oral erosions, and hemorrhagic crust on the nasal mucosa. Vulva, vagina, and perianal erosions were also evidenced. The diagnosis of SJS was established and intravenous immunoglobulin was promptly administered. Three days later, she died of pulmonary hemorrhage. The autopsy findings demonstrated generalized infection and widespread subepidermal detachment with necrotic keratinocytes. In conclusion, SJS is a rare and severe vesiculobullous disease in a pediatric lupus population and is probably associated with infections and drug therapy.
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PMID:Stevens-Johnson syndrome in a juvenile systemic lupus erythematosus patient. 2176 72


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