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)

The purpose of our study was to determine the usefulness of echo-planar diffusion-weighted imaging (EPDI) in the evaluation of watershed hypoxic-ischemic brain injury in pediatric patients. Eighteen patients ranging in age from 3 weeks to 12 years were evaluated for evidence of ischemic/infarction changes on conventional MR and EPDI. Included in the study group were five patients with sickle cell disease, four with congenital heart disease, four with hypotensive episodes with various etiologies, three with sepsis, and two with encephalitis or meningitis. Patients were examined 2 h to 6 days after the initial insult, with follow-up studies in four patients at 1 to 62 days after the initial examination. After conventional MR imaging (T1, FSE T2, and FLAIR), diffusion-weighted MR imaging was performed using high-speed, single-shot EP techniques with TR 6000, TE 144, matrix 96 x 128, FOV 23.3 x 31 and five b values of 0, 160, 360, 640, and 1,000 s/mm2. EPDI demonstrated abnormally increased signal in watershed ischemic/infarction zones in all initial cases. Apparent diffusion coefficients (ADC) were obtained in 59 lesions. When compared with radiographically normal (on EPDI) contralateral brain parenchyma, 45 demonstrated a relatively decreased ADC, while eight had normal ( +/- 10%) and six had increased ADC. In four cases, signal abnormalities on EPDI were not seen or exceeded that seen with conventional MR imaging. In the remaining cases, signal abnormalities were obvious on EPDI and more subtle on conventional MR imaging. Follow-up studies demonstrated resolution of abnormal EPDI signal with persistent abnormalities on conventional imaging in some cases, while others revealed an increase in size or number of EPDI signal abnormalities, suggesting ongoing acute ischemic/infarctive changes. EPDI is a rapid, sensitive technique for detecting watershed ischemic/infarction changes in pediatric patients with hypoperfusion episodes, at times before such changes are apparent on conventional MR images and/or are clinically apparent.
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PMID:Diffusion-weighted imaging in the evaluation of watershed hypoxic-ischemic brain injury in pediatric patients. 1176 Jul 94

Painful episodes are the most frequent complaints of patients with sickle cell disease. The Emergency Department (ED) has provided management for acute events using the usual triage format for emergencies. A prospective study evaluated the role of the ED in the care of adults with sickle cell disease (SCD). The protocol, thus, addressed issues of acute events related to SCD and provided better care for patients with SCD in the ED. Approximately 37% of ED visits were for painful events. An inciting cause was identified in 35% of painful events and 75% of these required admission to the hospital. A 15-year follow-up prospectively showed similar results and that uncomplicated pain crisis can be treated with ED protocols. Outpatient clinics and urgent centers could reduce these visits. Absolute indications for admission include sepsis, fever >102 degreeF, white cell counts >20 000, worsening anemia, hypoxemia, acute chest syndrome and new CNS events. Patient database in the ED must be revised annually to avoid extensive workup in the ED and a complete history/physical examination, and a CBC could be sufficient for triage in an uncomplicated pain crisis. An acceptable protocol for care should be available at all EDs and a registry and information system for SCD will discourage overutilization of investigational tests and visits to multiple EDs.
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PMID:Evaluation and Management of Sickle Cell Disease in the Emergency Department (An 18-year Experience): 1974--1992. 1183 5

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

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

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

The dramatic advances that have taken place in recent years in the care of sick and premature infants also have been matched by a similar increase in the use of blood transfusion therapy. Haematological features indicate that a newborn has a blood volume of 85-125 ml/kg the foetal haemoglobin is 60-85% and average Hb in full term infant is 18 gm/dl. By 2-3 months it falls to 11-12 g/dl the main cause of anemia are iron poor diet, weaning diets recurrent or chronic infections and hemolytic episodes in malarious areas. The red cells transfusions are usually top up transfusions, exchange transfusions, partial exchange transfusions. Top up- are for investigational losses and correction of mild degrees of anemias, upto to 5-15 ml/kg. They comprise 90% of all neonatal transfusions and are used in low birth babies in special care units for a maximum of 9-10 episodes. The walk in donor programs once popular are not much in vogue. The threshold for transfusion is 8-10 g/dl Hb for upto 5 weeks. Exchange transfusions are done for correction of anemia, removal of bilirubin, removal of antibodies and replacement of red cells. Ideally plasma reduced red cells that are not older than 5 days are used. It is prepared by removal of 120 ml of standard whole blood donation. The advantage of fresh cells is that hyperkalemia is avoided and good post transfusion survival acceptable red cell oxygen affinity. However it has to be screened for sickle cell disease and G6PD deficiency. Indications for exchange transfusion are kernicterus, neonatal hemolysis, G6PD deficiency, ARDS, neonatal sepsis, DIC and neonatal isoimmune thrombocytopaenia. Complications include over transfusion, perforation of major vessels, hypocalcaemia, citrate toxicity, hypothermia, hypoglycaemia, thrombocytopenia, necrotizing enterocolitis, GVHD, bacterial, viral infections. Partial exchange transfusions are done for symptomatic anemia, where Hb<10 g/dl, it is indicated in polycythemia and hyperviscosity syndromes. Exchange volume = Blood volume x (observed Hct-Desired HCt) divided observed Hct. Points to consider-there is weak expression of ABO antigens so particular care while grouping. Transfusing volumes should be 2-5 ml/kg/hour in paediatric bags of 50-100 ml with infusion devices. Platelet transfusion are indicated in neonatal throbocytopaenia, thrombocytopaenia due to sepsis, DIC, bacterial pathogens, CMV, TORCHS, Obstetric conditions such as pre eclampsia, intrauterine death abruption placenta birth injury hypoxia schock neonatal iso immune thrombocytopaenia and maternal ITP. Administration 1 RDE/pack per 2.5 kg single dose of fresh platelets less than 24hrs which contains 55 x 10(9) cells. This also contributes fresh plasma so is useful for coagulation defects also, though there is a risk of CMV and GVHD due to leucocyte contamination. Granulocyte concentrate; Gravity leucopheresis-1:8 ratio of 60 ml of 6% HES made to stand for 1hr.
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PMID:Component therapy. 1451 88

Acute chest syndrome (ACS) is the most common cause of death in patients with sickle cell anemia. Its management is primarily palliative. We performed a Phase I evaluation of purified poloxamer 188 (a non-ionic surfactant) in the management of ACS. Forty-three patients with sickle cell disease and ACS were treated with doses as high as 2960 mg/day by continuous intravenous (IV) infusion. The maximum tolerated dose has not been identified. No evidence of renal toxicity or other limiting adverse events were found. One adult patient died due to sepsis and adult respiratory distress syndrome, which were unrelated to treatment. Poloxamer 188 is safe to administer to patients with ACS, and preliminary data suggest that it may shorten its duration and the length of hospitalization in a dose related manner. Children appeared to benefit more than adults. The data and safety profile justify further studies with purified poloxamer 188 in the treatment of ACS.
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PMID:Safety of purified poloxamer 188 in sickle cell disease: phase I study of a non-ionic surfactant in the management of acute chest syndrome. 1518 51


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