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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemoglobinopathies are a major public health problem in Saudi Arabia. We studied the effect of splenectomy in 16 Saudi Arabian children with compound hemoglobinopathies. Seven patients no longer require regular blood transfusions, and transfusion requirements were decreased by 30 to 60% in the other eight patients. Three patients whose heights and weights were below the 5th percentile before splenectomy reached the 25th percentile 1 year after the surgery. In spite of preoperative pneumococcal vaccination and the penicillin prophylaxis after the surgery, one patient died of Haemophilus influenzae Group B bacteremia, and three others had six episodes of pneumonia.
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PMID:Splenectomy in compound heterozygous hemoglobinopathies in Saudi Arabia. 224 Apr 76

Haemophilus influenzae type B vaccine is recommended for children 1.5 to 6 years of age with sickle cell anemia, but the adequacy of their response is unknown. A total of 69 children with sickle cell syndromes, 1.5 to 5.6 years of age, were immunized with two vaccines alternatively, single blind. PRP vaccine was given to 36 children and a diphtheria toxoid conjugated vaccine, PRP-D, was given to 36. Coded pre- and postvaccine sera were tested by radioimmunoassay for anti-PRP antibody. The groups did not differ in age distribution or type of sickle hemoglobinopathy. Preexisting antibody levels were low in both vaccine groups; 65% were less than 0.15 microgram/mL. The vaccines were safe but associated with frequent minor reactions. PRP-D gave higher geometric mean titers and mean fold titer increase than PRP in all children (15.58 micrograms/mL [234-fold] v 2.63 micrograms/mL [29-fold]) and in the subgroups 1.5 to 2.5 years of age or with pretiter values less than 0.15 microgram/mL. Titers for 64% of children receiving PRP and 94% receiving PRP-D were greater than or equal to 1.0 microgram/mL. Thus, both vaccines were useful in this population, but PRP-D was more immunogenic. Duration of antibody levels postvaccination, booster responses, and PRP-D immunogenicity in younger children with sickle cell syndromes all require further study.
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PMID:Haemophilus influenzae type b immunization of children with sickle cell diseases. 326 57

In the past 15 years, it has become increasingly evident that diverse conditions may be linked with hyposplenism and that hyposplenic individuals are susceptible to overwhelming infection, particularly by the encapsulated organisms such as pneumococci, meningococci, and Haemophilus influenzae. Identification of Howell-Jolly bodies in the peripheral blood smear is the most important clue to the diagnosis. Associated diseases include gastrointestinal disorders, hemoglobinopathies, autoimmune disorders, amyloidosis, neoplasms, circulatory disturbances, and other diseases. Hyposplenic patients should be instructed to report immediately to their physician if they suspect any infection. These patients should receive empiric treatment with antibiotics on an emergency basis at the earliest sign or symptom of infection, and they should also receive prophylactic vaccination against pneumococcal microorganisms.
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PMID:Functional hyposplenism. 330 64

These clinical practice guidelines set forth a comprehensive program for identifying, diagnosing, and treating newborns and infants with sickle cell disease and recommend education and counseling strategies for their parents. Sickle cell disease comprises a group of genetic disorders characterized by the production of hemoglobin S, anemia, and acute and chronic tissue damage secondary to the blockage of blood flow by abnormally shaped red cells. Sickle cell anemia is the most common form of the disease, and it affects approximately 1 in 375 African-American infants. Although in the United States sickle cell disease is most commonly found in persons of African ancestry, it also affects other populations. The panel recommends screening of all newborns for sickle cell disease, since targeting specific groups will miss some infected infants. Samples of dried blood on filter paper or liquid blood samples should be used for hemoglobinopathy screening. Hemoglobin electrophoresis, isoelectric focusing, and high performance liquid chromatography are acceptable, reliable, and accurate testing methods. Infants identified on initial screening must be retested to establish a definitive diagnosis. Affected infants must be given twice-daily oral penicillin beginning at 2 months of age to reduce pneumococcal, conjugated Haemophilus influenzae, and hepatitis B vaccines. Infants with sickle cell disease require the same well-child care as infants without the disease. Education and nondirective genetic counseling should be offered to all parents of infants with sickle cell disease. The guidelines stress the need for a comprehensive and fully integrated approach to reduce morbidity and mortality from sickle cell disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Update: new guidelines for the treatment of infants with sickle cell disease. Agency for Health Care Policy and Research. 827 35

Children with SCD are prone to invasive infections caused by S. pneumoniae and H. influenzae. Osteomyelitis is caused most often by Salmonella species and less often by S. aureus. The chest syndrome and its associated microvascular disease carry a risk of prolonged and severe infections for Mycoplasma, Chlamydia, and probably other lower respiratory pathogens, particularly in the group of children with SCD prone to pain or microvascular sequestration, such as those with SC hemoglobinopathy. Despite three decades of investigation, the immunopathologic mechanisms leading to these increased risks is not completely clear. Bone infarction and microvascular disease probably play a part in the predisposition to osteomyelitis. Dysfunctional IgG and IgM antibody response, a lack of splenic clearance, defects in alternative pathway fixation of complement, and opsonophagocytic dysfunction play a role in the predisposition to invasive infection from polysaccharide-encapsulated organisms. Immunization with the conjugate Haemophilus vaccines has largely controlled infections caused by this pathogen. Early recognition of SCD through neonatal screening allows early and vigorous antibiotic management of febrile episodes in children with SCD and has perhaps provided the greatest benefit. Treatment of acute febrile episodes should include antibiotics active against regional strains of S. pneumoniae and H. influenzae, whereas treatment of febrile lower respiratory infections should include macrolide antibiotics that are active against Chlamydia and Mycoplasma, as well as pneumococci and Haemophilus. To date, no convincing evidence exists for the efficacy of pneumococcal polysaccharide vaccines in children with SCD, but preliminary data with the conjugate pneumococcal vaccines in normal children and those with SCD suggest that they may be as successful as Haemophilus vaccines in controlling this infection once they are available. Prophylaxis with daily penicillin administration is recommended and is well founded on clinical trials. However, problems with pneumococcal penicillin resistance and the association of failure with a lack of compliance to antibiotic regimens will dictate continued reexamination of this modality for the prevention of pneumococcal infections.
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PMID:Infections and immunizations of children with sickle cell disease. 1007 55

Fulminant, potentially life-threatening infection is a major long-term risk after splenectomy or in persons who are functionally hyposplenic as a result of various systemic conditions. Most of these infections are caused by encapsulated organisms such as pneumococci, Haemophilus influenzae and meningococci. A splenectomized patient is also more susceptible to infections with intraerythrocytic organisms such as Babesia microti and those that seldom affect healthy people, such as Capnocytophaga canimorsus. Most patients who have lost their spleens because of trauma are aware of their asplenic condition, but some older patients do not know that they are asplenic. Other patients may have functional hyposplenism secondary to a variety of systemic diseases ranging from celiac disease to hemoglobinopathies. The identification of Howell-Jolly bodies on peripheral blood film is an important clue to the diagnosis of asplenia or hyposplenia. Management of patients with these conditions includes a combination of immunization, antibiotic prophylaxis and patient education. With the increasing prevalence of antibiotic-resistant pneumococci, appropriate use of the pneumococcal vaccine has become especially important.
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PMID:Detection, education and management of the asplenic or hyposplenic patient. 1127 93