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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The microflora and its significance was investigated in 82 leg ulcers in patients with sickle cell disease. Staphylococcus aureus, Pseudomonas aeruginosa and group A streptococci were the most common isolates. The majority of ulcers which yielded these organisms showed signs of local inflammation, regional lymphadenopathy and healed slowly as compared with ulcers which did not show these organisms. Repeat culture swabs demonstrated persistence of these organisms. A topical spray containing neomycin, polymyxin B and bacitracin helped to eradicate these organisms and hastened the healing process of ulcers. Coliforms and anaerobes were also isolated but were transient in nature and of no significance in the pathology of such ulcers.
Infection
PMID:Microbial flora and its significance in pathology of sickle cell disease leg ulcers. 158 89

Infection is the most common cause of high morbidity, hospitalization, and mortality in children with sickle cell anemia. In this study of pediatric sickle cell anemia patients, aged 1 to 19, we explore the hypothesis that gallstones (usually pigment stones) create a nidus of infection, predisposing the affected patients to high morbidity. Our study involved 86 children with sickle cell anemia at the Howard University Center for Sickle Cell Disease, who had been followed at the clinic for a total of 602 patient years. Review of their records revealed that patients with gallstones had a mean number of 10.24 hospitalizations and 25.35 ambulatory visits; those without gallstones had a mean number of only 4.26 hospitalizations and 13.41 ambulatory visits. In children with sickle cell anemia and gallstones, elective cholecystectomy (or, in the future, cholelithotripsy) could reduce the high morbidity caused by infection.
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PMID:The significance of gallstones in children with sickle cell anemia. 221 13

The pattern of illness in 60 consecutive children with homozygous sickle cell disease who attended the Paediatric Emergency Room of a busy Lagos hospital with acute illness was studied prospectively. Their ages ranged from 3 months to 13 years with a peak in the 2nd year. There were twice as many boys as girls. The commonest symptoms were fever, limb or abdominal pain and cough, and the commonest signs were pallor and hepatomegaly. Painful crises occurred in 27, anaemic crises in 11, and a combination of these in 12 children. Infection was detected in 76% of subjects in crises. Infection was found in 82% of all the children and was mainly bacterial. The commonest infections were pneumonia (35%), bacteraemia (32%), tonsillitis/pharyngitis (17%) and osteomyelitis (8%). The predominant bacteria isolated were Klebsiella spp (38%), E. coli (23%), Staph. aureus (23%), Staph. albus (23%) and Pseudomonas spp (23%). Some children had multiple isolates. Bacterial infection was a major cause of morbidity in very young children and merits appropriate control and preventive measures in this age group. The spectrum of bacteria isolated makes it unlikely that the specific anti-pneumococcal measures widely advocated in Europe and America for young children with SCA would be appropriate in Nigeria.
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PMID:Acute illness in Nigerian children with sickle cell anaemia. 244 66

B19 virus is the first human virus to be shown to be a member of the parvovirus genus. This review is concerned with the diseases associated with B19 virus, their nature, pathogenesis and diagnosis. The virus was discovered by chance in blood donors but has been shown to be a common infection of childhood. Infection may be asymptomatic or associated with mild, non-specific symptoms. The most common specific clinical manifestation is an erythematous rash illness which often has the classical features of erythema infectiosum. Often, however, it is described simply as rubelliform and only laboratory tests can distinguish B19 and rubella virus infections. Joint involvement is the most common complication of B19 virus infection occurring especially in adult females. It often involves the joints of the hands and wrists, clears rapidly in most patients but may persist for months or years in a few. B19 virus is also the principle cause of the transient aplastic crisis which complicates chronic haemolytic anaemia. This has been demonstrated repeatedly in sickle cell anaemia and hereditary spherocytosis and in individual cases of other haemolytic anaemias. The pathogenesis of the aplastic crisis is related to the ability of B19 virus to infect and damage early erythroid progenitor cells. Volunteer studies in normal individuals have demonstrated that this is a regular event occurring about a week after infection via the respiratory tract. Rash illness and joint involvement occur 7 to 10 days later and are presumably immune mediated. Diagnosis of B19 virus infection can be achieved by detection of the viraemia (aplastic crisis) or by detection of virus specific IgM antibody (all diseases).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:B19 virus--a pathogenic human parvovirus. 284 30

Human parvovirus (HPV) infection has recently been implicated as the cause of aplastic crisis in patients with hemolytic anemias such as congenital spherocytosis and sickle cell anemia. The virus causes a transient red cell aplasia which, in patients with a shortened red cell life span, is manifested as a rapid worsening of the anemia and an absence of peripheral reticulocytosis. Recovery is associated with the presence of giant pronormoblasts in the bone marrow, and several days later, a brisk peripheral reticulocytosis. In normal subjects, HPV causes erythema infectiosum (fifth disease) but is not associated with symptomatic anemia, probably because of the duration of the normal red blood cell life span. A case of HPV infection producing severe anemia in an immunocompromised patient without an underlying hemolytic anemia is presented here. Infection in this patient, a 3-year-old boy with acute lymphoblastic leukemia in remission, may have been prolonged by immunosuppression, leading over a 4-week period to a severe anemia. The immunosuppressed appear to be another group of patients at risk of developing symptomatic anemia when infected by HPV.
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PMID:Human parvovirus-associated red cell aplasia in the absence of underlying hemolytic anemia. 302 Oct 15

Infection with the human immunodeficiency virus (HIV) leads to selective depletion of the helper/inducer lymphocyte subset and a subsequent state of acquired cellular immunodeficiency. Simultaneously, evidence of B-cell hyper-activity may exist. A subset of patients infected with HIV demonstrates a syndrome of persistent generalized lymphadenopathy (PGL). Lymph node biopsies reveal benign reactive changes with a pattern of florid follicular hyperplasia. A polyclonal hypergammaglobulinemia reflects humoral immune dysfunction. Patients with PGL are similar to those with full-blown AIDS with regards to demographics, immune and virologic studies. Our prospective natural history study of PGL patients initiated in November 1981 reveals a 15% rate of evolution to AIDS in the 200 patient cohort. Factors associated with increased risk of transformation to AIDS include severity of constitutional symptoms, shrinking adenopathy, oral candidiasis or viral hairy leukoplakia, peripheral cytopenias, elevated erythrocyte sedimentation rate or an antecedent episode of herpes zoster. Therapeutic interventions to prevent evolution to AIDS in high risk subsets of lymphadenopathy patients have been investigated. In addition to benign B-cell proliferation associated with HIV infection, malignant lymphomas have also been diagnosed in 29 patients in AIDS risk groups in our clinic population. All patients were male; 26 homosexuals, 2 IV drug abusers and 1 multiply transfused sickle cell anemia patient. Seven patients had antecedent PGL. Non-Hodgkin's lymphoma was diagnosed in 19 patients. Histologies were predominantly diffuse undifferentiated or large cell. Eleven patients were Stage IV at diagnosis. Of 10 patients with mixed cellularity Hodgkin's disease, 7 were Stage IV-B at presentation. Extranodal disease was frequent in patients with lymphomas. Fourteen patients lacked peripheral lymphadenopathy. Response to chemotherapy was good, but complicated by prolonged marrow suppression and development of AIDS-related opportunistic infections. Median survival was 7 months. Laboratory studies investigating the possible role of lymphotropic retroviruses in the development of AIDS-related lymphomas revealed that serum from all patients with high grade non-Hodgkin's lymphoma contained antibodies to HIV and that the majority also expressed antibodies to HTLV-I. This degree of seroreactivity to HTLV-I and HIV was characteristic only of lymphoma patients as sera from only 10 - 15% of AIDS and ARC patients in San Francisco had similar findings.
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PMID:AIDS-related benign lymphadenopathy and malignant lymphoma: clinical aspects and virologic interactions. 382 9

An approach that is useful in classifying the risk factors for the development of acute osteomyelitis is the same as one commonly employed to discuss the mechanisms responsible for pathogenesis: hematogenous dissemination, direct inoculation, and contiguous spread from an adjacent area of soft tissue infection. Acute hematogenous osteomyelitis is predominantly a disease of children. Factors that favor the development of acute bone infection are those that predispose to bacteremia. These include indwelling intravascular catheters, distant foci of infection, and intravenous drug abuse. The distant sites of focal infection that are most commonly associated with acute osteomyelitis include the skin as well as urinary and respiratory tracts. Two patient groups with an usual susceptibility to acute skeletal infections are those with sickle cell anemia and chronic granulomatous disease. The second major mechanism for the development of acute osteomyelitis is by direct inoculation. Injuries due to penetrating bites and puncture wounds of the food may serve to infect bone directly. Diagnostic procedures (lumbar puncture, fetal monitoring electrodes, suprapubic aspiration, and heel sticks) may result inadvertently in the inoculation of a neighboring osseous structure. Surgical procedures such as internal fixation of long bone fractures and skeletal traction may cause an infection of the bone. Osteomyelitis may develop as a consequence of contiguous spread of infection from adjacent soft tissue, particularly if vascular insufficiency complicates the clinical picture. Infection of the mandible, maxilla, and frontal or mastoid bones may result from persistent or neglected infection of the teeth, paranasal sinuses, or middle ear cavity, respectively. The major risk factor for chronic infection of bone is inadequate or delayed management of acute osteomyelitis or completely unrecognized bone infection.
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PMID:Risk factors for osteomyelitis. 389 17

Infection with Streptococcus pneumoniae after immunization with polyvalent pneumococcal polysaccharide vaccine has been reported recently in some patients with sickle cell disease (SCD). Since previous studies have suggested that patients with functional hyposplenia may not be able to produce normal levels of protective antibody and/or to switch from IgM to IgG class of antibody in response to some forms of antigenic challenge, we investigated the ability of patients with SCD to form IgM and IgG antibodies after immunization with pneumococcal polysaccharide. Our results demonstrate that both normal individuals and patients with SCD respond with both IgM and IgG antibodies. The level and predominant class of antibody varied with the serotype of antigen tested. Although statistically significant differences were not found, slightly lower responses, especially to pneumococcal polysaccharide type 6, were noted for patients with SCD. Further investigation of the immunoglobulin class of antibody produced by patients with vaccine failure will be necessary to determine if inadequate IgG or IgM response contributes to the lack of protection for these patients.
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PMID:Pneumococcal vaccine in sickle cell disease: IgG and IgM antibody response. 616 29

Sickle cell anemia is a congenital haemolitic anemia. The replacement of the hydrophilic glutamine residue at the 6 position of the beta chain by the hydrophobic valine leads to severe conformation change in the molecule resulting in sickling. The consequences are haemolysis, vascular stasis and thrombotic crisis. Infection triggers thrombosis and vice-versa. Clinical manifestation is anemia punctuated by intermittent crisis producing infarction. Pneumococcal and salmonella infections are frequent mainly in the lungs and bones. Prognosis is poor, half of the patients dying before 20 years. Prompt treatment of infection is important. Treatment includes good nutrition and administration of folic acid. Transfusions are often usefull to avoid hazards of anesthesia or surgery. Ante natal diagnosis based on smaller sample of foetal blood or amniotic liquid is now possible.
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PMID:[Sickle cell anemia, an example of a constitutional disease of hemoglobin]. 657 34

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.
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PMID:Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. 749


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