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)

Patients who lack a functioning spleen become vulnerable to sepsis caused by bacteria and, occasionally, protozoa. The risk is higher in children and in those who have had immunosuppressive treatment, and the risk remains lifelong. Overwhelming post-splenectomy infection (OPSI) occurs at an estimated incidence of 0.23-0.42% per year, with a lifetime risk of 5%. Episodes of OPSI are emergencies, requiring immediate parental antibiotics and intensive care; intravenous immunoglobulins may be useful. OPSI carries a mortality of 38-69%. Streptococcus pneumoniae is the commonest infecting organism, accounting for 50-90% of isolates from blood cultures in reported series; it is particularly common in children with sickle cell disease. Less commonly, the infecting organisms are other bacteria, Babesia or Ehrlichia. OPSI may be, to some extent, preventable by several interventions. These are surgical conservation of the spleen; immunization against S. pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis; prophylactic antibiotics; stand-by antibiotics; patient information sheets; and a medical alert bracelet. Asplenic patients living in malaria-endemic areas require optimal prophylaxis. The initial step in prevention of OPSI is the creation of an asplenia register, as many patients are not covered by these simple measures.
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PMID:Prevention and management of infections in patients without a spleen. 1266 59

Despite systematic antibiotic therapy, severe infections (septicemia, meningitis, or osteomyelitis) are a major cause of mortality and morbidity in children with sickle cell disease (SCD). In this study, we explored the possibility that polymorphism at the human leukocyte antigen (HLA) locus might constitute an immunogenetic modifying factor to the intrinsic susceptibility to infection in patients with SCD. A cohort of 80 SCD patients living in Paris, 43 with at least one major infectious complication and 37 without infections, were typed for HLA class II loci by polymerase chain reaction-sequence-specific primers (PCR-SSP). We found that significantly more patients without infections carry the HLA class II DRB1*15 specificity than did patients with infections (21.6% in the first group, versus 4.7% in the second group; chi(2) = 10.47, p(c) = 0.01), supporting a protective effect of this allele. Conversely, significantly more patients were found to carry the DQB1*03 specificity within the group of severe infections, supporting a negative effect (34.9% versus 12.2%, chi(2) = 9.41, p(c) = 0.01). These findings suggest a direct involvement of HLA polymorphism in the development of major infections in SCD. Together with previous data on polymorphism of the Fc receptor and of the mannose-binding lectin, they provide evidence for a polygenic immunomodulation of the constitutively increased infectious risk in SCD.
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PMID:Infectious complications in sickle cell disease are influenced by HLA class II alleles. 1187 37

Splenic infarcts are common in patients with sickle cell anemia (SCA), but these are usually small and repetitive, leading ultimately to autosplenectomy. Massive splenic infarcts on the other hand are extremely rare. This is a report of our experience with 8 (4 males and 4 females) cases of massive splenic infarction in patients with SCA. Their ages ranged from 16 to 36 years (mean 22 years). Three presented with left upper quadrant abdominal pain and massive splenic infarction on admission, while the other 5 developed massive splenic infarction while in hospital. In 5 the precipitating factors were high altitude, postoperative, postpartum, salmonella septicemia, and strenuous exercise in one each, while the remaining 3 had severe generalized vasoocclusive crises. Although both ultrasound and CT scan of the abdomen were of diagnostic value, we found CT scan more accurate in delineating the size of infarction. All our patients were managed conservatively with I.V. fluids, analgesia, and blood transfusion when necessary. Diagnostic aspiration under ultrasound guidance was necessary in two patients to differentiate between massive splenic infarction and splenic abscess. Two patients required splenectomy during the same admission because of suspicion of secondary infection and abscess formation, while a third patient had splenectomy 2 months after the attack because of persistent left upper quadrant abdominal pain. In all the 3 histology of the spleen showed congestive splenomegaly with massive infarction. All of our patients survived. Two patients subsequently developed autosplenectomy while the remaining 3 continue to have persistent but asymptomatic splenomegaly. Massive splenic infarction is a rare and unique complication of SCA in the Eastern Province of Saudi Arabia, and for early diagnosis and treatment, physicians caring for these patients should be aware of such a complication.
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PMID:Massive splenic infarction in Saudi patients with sickle cell anemia: a unique manifestation. 1189 8

Sickle cell disease (SCD) is a term used to describe a group of genetic disorders of hemoglobin production characterized by a predominance of the abnormal hemoglobin known as hemoglobin S. Common acute complications of SCD in children requiring hospitalization include painful episodes, febrile illness, and splenic sequestration. The staff nurse has an important role in providing prompt treatment and instituting preventative measures to avoid the adverse clinical outcomes of SCD such as acute chest syndrome, severe anemia, cardiovascular instability, and bacterial sepsis. A basic understanding of the pathophysiology of vaso-occlusion, the immune system, hemolysis, and the spleen is essential in the care of a child during an acute complication of SCD. Additionally important are a knowledge of the genetics, pathophysiology, medical and nursing management, and a familiarity with patient and family education material relating to sickle cell disease.
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PMID:Care of the child with sickle cell disease: acute complications. 1202 71

The purpose of this paper was to review the research examining the epidemiology of and costs associated with sickle cell anaemia (SCA). Although there is general acceptance that Black populations are at greatest risk of the disease, estimates of disease incidence and prevalence vary greatly among different Black populations. In addition, the sickle cell haemoglobinopathy poses a health problem to many other ethnic groups, including populations native to Italy, Greece, Turkey, Saudi Arabia, India, Pakistan, Bangladesh, China, and Cyprus. As penicillin prophylaxis has been shown to reduce the risk of sepsis among children with SCA, many governments have established newborn screening programmes to improve the health outcomes for patients with this disease. As a group, patients with SCA incur large numbers of hospital admissions, emergency department visits, and outpatient visits, often at substantial costs, hence, obtaining adequate health insurance is a problem for many patients. A common theme present in studies reviewed in this article is that a small proportion of patients tends to account for a majority of the total healthcare costs. As new diagnostic methods and treatment options become available, balancing costs associated with SCA and quality of healthcare will continue to present challenges to many healthcare providers and insurers.
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PMID:Sickle cell anaemia: epidemiology and cost of illness. 1205 95

Vasculo-occlusive crisis with organ infarctions occur in sickle cell disease (SCD). However, heart infarction is not commonly reported. We reviewed 19 cases of documented myocardial infarction (MI) in SCD patients. The true incidence may be higher because the diagnosis was often made at autopsy and was overshadowed during life by other musculoskeletal symptoms. Electrocardiography is frequently unhelpful. Skeletal muscle enzymes confound serum cardiac enzyme interpretation. The mechanism of MI in SCD is not exactly known, as coronary angiography is usually normal. MI frequently occurs in association with hypoxia, cor pulmonale, anemia, sepsis, acidosis, and renal failure. The aim of this article is to increase awareness for this complication and to prompt prospective studies to look at treatment strategies for myocardial infarction in SCD.
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PMID:Myocardial infarction in sickle cell disease. 1207 25

We assessed the efficacy of progressive soft tissue distraction using monolateral external fixation in the management of severe knee flexion contractures. We prospectively evaluated 10 knee deformities in seven pediatric patients. After gradual distraction using the modified Orthofix Limb Reconstruction System (LRS), most recent functional status and knee range of motion were determined. This treatment was applied to 10 extremities in seven patients, ranging in age from 2 to 16 years. Diagnoses included arthrogryposis (4), sickle cell disease (1), previous sepsis (1), and congenital pterygium (1). Average preoperative flexion contracture was 80.5 degrees. Each patient achieved full extension. There was one recurrence, despite bracing, which was managed with replacement of the fixator and soft tissue procedures. Management of knee flexion contractures using a monolateral fixator appears to be a viable alternative to extensive release or femoral osteotomy. Long-term follow-up will be essential to assess the overall risk of recurrence and complications.
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PMID:Knee flexion contractures: soft tissue correction with monolateral external fixation. 1213 40

Children with sickle cell anemia are more exposed to infection than healthy children. Indeed, infections are the major cause of morbidity and mortality in children with sickle cell anemia, especially those aged 6 months to 5 years. Phagocytosis is reduced in these children. Polynuclear neutrophils reveal various poorly understood irregularities and are associated with a reduction of phagocytic power: zinc deficiency, reduced post-phagocytic oxidative metabolism, and a prevalence of neutrophils not forming red sheep-like globule carriers of immunoglobulin H. The power of the antibody which renders germs susceptible to phagocytosis in the serum is reduced in sickle cell patients. This may be tied to a disorder in the alternate complementary route with reduction of C3 and properdin. Sequestration of sickle cell-shaped red blood cells, splenic congestion, and short circuits of important functional territories contribute to spleen dysfunction, which occurs early. Common pathogens attacking sickle cell patients are pneumococci, salmonella species, and Haemophilus influenzae. They cause very grave infections (e.g., septicemia and purulent meningitis). Prevention of infections dwells on three perspectives: early screening for sickle cell anemia and for spleen dysfunction, preventive penicillin therapy, and vaccination. In Benin, vaccination is the only means to prevent infections. Essential vaccinations for children with sickle cell anemia include BCG, diphtheria-pertussis-tetanus, polio, and Rouvax. Strongly recommended vaccinations are Pneumovax 23, HEVAC B, TAB, vaccine against H. influenzae, and vaccine against mumps. A vaccine calendar for children with sickle cell anemia guides health workers when they must administer the vaccines and their boosters over a six year period. It is not yet universal in health facilities in Benin. A short- and long-term evaluation of the calendar's efficacy would allow one to appreciate its real impact on reducing morbidity and mortality in children with sickle cell anemia.
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PMID:[Prevention of infectious diseases in the drepanocytic child]. 1229 Jan 82

The archives of the blood bank of the hospital of Dr. Louis Manual Morillo King, in the city of La Vega, Dominican Republic, were reviewed to identify all children who had been given blood transfusion during the period of July 1983 to July 1987 in order to identify HIV and the surface antigen of hepatitis B (HBsAg). Those who were released were visited in their homes for administration of HIV and hepatitis tests. Positive tests were confirmed by another test (AUSYME MONOCLONAL and Western Blot). Mothers were also tested to detect vertical transmission. 256 patients had been transfused, of whom 61 died. 80 of the 195 remaining patients could not be located. Of the 115 patients located, 52 had died in their homes after release from the hospital. Thus, the sample comprised 63 patients: 36 were 0-3 years old, 21 were 4-7 years old, and 6 were 8-11 years old. 50 lived in rural and 13 in urban areas. 56 patients had one transfusion and 4 had two transfusions. 28 patients had transfusion for anemia, 19 for malnutrition, 7 for sepsis, 6 for various reasons (meningitis, pleuritis, pneumonia), and 3 for sickle cell disease. 47 patients had been transfused at the hospital using the blood bank, 13 used blood from relatives, and 3 received blood from friends. Out of the 63 samples processed, 2 patients presented seropositivity for hepatitis B, while none were seropositive for HIV. Among the 2 patients who were seropositive for hepatitis B, the mother of one of them was also seropositive.
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PMID:[Human immunodeficiency virus and hepatitis B virus in children transfused in the Dr. Luis Manuel Morillo King Hospital]. 1234 60

Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Young children between the ages of 2 and 4 years were found to be at the greatest risk of developing anemic crises (severe anemia). There was a gradual but progressive decline in the incidence of severe anemia in the age range 8-16 years old. Upper respiratory tract infections are the most commonly associated infections in patients with severe anemia. Others included malaria, septicemia, urinary tract infection, acute chest syndrome, and osteomyelitis. Their role in precipitating episodes of severe anemia among the patients studied could not be fully evaluated. Pallor, jaundice, and fever were the most commonly encountered symptoms in patients with severe anemia on admission. About half of the parents/guardians failed to notice severe anemia among the patients studied, perhaps due to the dark color of the African skin. Caregivers need to be educated on how to recognize anemia among patients with sickle cell anemia when they develop febrile episodes.
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PMID:Clinical presentation of severe anemia in pediatric patients with sickle cell anemia seen in Enugu, Nigeria. 1260 90


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