Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
 ...
PMID:Clinical presentation of severe anemia in pediatric patients with sickle cell anemia seen in Enugu, Nigeria. 1260 90

Adenotonsillar hypertrophy and chronic tonsillitis are common findings in patients with sickle cell disease (SCD). Various preoperative transfusion regimens have been suggested to reduce the population of sickle erythrocytes and correct the anemia, ranging from conservative (correcting the anemia) to aggressive (lowering the level of HbS to less than 30%). A total of 39 patients with SCD were included in the study. They were divided into 2 groups. Fourteen patients in group 1 were assigned aggressive exchange transfusion and 25 patients in group 2 were assigned a conservative (simple) transfusion. The 2 groups were compared for possible operative and postoperative complications. Thirty percent of patients in both groups had postoperative complications. They ranged from mild local infection to acute chest syndrome. Simple transfusion was not associated with higher incidence of complications and resulted in only one-third as many transfusion requirements.
 ...
PMID:A comparison of two transfusion regimens in the perioperative management of children with sickle cell disease undergoing adenotonsillectomy. 1268 48

Pregnancy in SCD women, with a major sickle cell syndrome, is a high-risk situation and is associated with raised incidence of maternal and fetal morbidity and mortality, mainly in late pregnancy, during delivery and in the postpartal periods. Pregnancy increases the incidence of sickle cell specific complications such as anaemia, vaso-occlusive crisis, abdominal, pulmonary (acute chest syndrome) or placental thrombosis, infections (urinary tract infection, pyelonephritis, pneumonia), and toxemia. Maternal death can occur. Sickle cell disease is responsible for high risk of spontaneous abortion, intra-uterine growth retardation, intra-uterine fetal death, preterm delivery, and perinatal mortality related to hypoxemia and placental thrombosis. More and more sickle cell affected women reach adulthood and reproductive age (fertility of sickle cell women is normal). The knowledge of these risks has contributed to the implementation of specific management program and to a better outcome of pregnancy. Such a program includes a close multidisciplinary approach for the duration of the pregnancy, the delivery and the postpartal period, in tertiary maternal health services level. Blood transfusion depends on teams and is related to strictly restricted maternal, obstetrical and hematologic indications.
 ...
PMID:[Sickle cell disease and pregnancy]. 1555 71

Sickle cell anaemia (SCA) is the most common cause of childhood stroke, occurring with the highest frequency before the age of 6 years. Despite the relative frequency of stroke in SCA, few predictors of risk exist. Anaemia, leucocytosis, hypertension, silent infarction, and history of acute chest syndrome are well-documented risk factors for ischaemic stroke in SCA. Recent data suggest that other environmental and genetic factors, many unrelated to SCA, influence the development of cerebrovascular disease. Non-invasive assessment of individual stroke risk using transcranial Doppler ultrasonography has provided a means of selecting and prophylactically treating SCA children at highest risk. With the ultimate goal of preventing stroke, the information gained from the studies reviewed here may lead to improved prediction of stroke so that clinical trials to assess risk-based therapy may be carried out on selected children with SCA.
 ...
PMID:Defining stroke risk in children with sickle cell anaemia. 1661 14

Conflicting ventilatory defects have been reported in children with sickle cell disease (SCD). In Kuwait, the disease is relatively mild with a low incidence of acute chest syndrome and other complications, presumably due to the Arab-Indian haplotype chromosomal background and elevated Hb F levels. There have been no previous studies of pulmonary function in patients with this haplotype. Pulmonary function test (PFT) was carried out on 28 steady state children with SCD (21 homozygous sickle cell (SS), seven S beta(o) thal) and two group of controls: 17 age- and sex-matched healthy children and 10 children with HbH disease. The charts of the SCD patients were reviewed for frequency of acute chest syndrome and vaso-occlusive crisis. The mean values of forced vital capacity (FVC) (83.2 +/- 11.9 vs. 91.2 +/- 11.7) and vital capacity (VC) (81.5 +/- 11.8 vs. 90.5 +/- 10.9) were significantly lower in the SS patients compared with healthy controls (p < 0.05). Similarly, these values were significantly lower than in those of the HbH group (p < 0.001 for VC and p < 0.01 for FVC). The mean forced expiratory volume in 1 s (FEV1) was lower in SS patients (86.4 +/- 11.5) compared with healthy controls (94.2 +/- 14.2), but the difference was not significant (p = 0.07). Also, the FEV1 was significantly lower in SS patients than in the HbH group (p < 0.001). There was no significant difference in the PFT parameters between SS patients with acute chest syndrome and those without. Although patients with frequent vaso-occlusive crisis had lower PFT parameters, the differences were not significant in comparison to those with infrequent crisis. This study revealed an early restrictive and obstructive pulmonary function pattern in steady state children with SCD. The finding also indicates that the changes of PFT parameters in SS patients could not be attributed to anaemia per se as patients with HbH who also have chronic anaemia did not show similar changes. This observation underscores the early occurrence of pulmonary involvement, even in patients with an otherwise relatively mild SCD.
 ...
PMID:Pulmonary function studies in Kuwaiti children with sickle cell disease and elevated Hb F. 1585 91

Individuals with sickle cell disease (SCD) may have oxyhaemoglobin desaturation during the steady-state, the causes of which are incompletely known. We studied a cohort of 585 children who have sickle cell anaemia (SS), sickle beta0-thalassaemia (Sbeta0), sickle-haemoglobin C disease (SC), or sickle beta+-thalassaemia (Sbeta+) to determine the relationships between steady-state oxyhaemoglobin saturation (SpO2) and SCD genotype, age, gender, steady-state haemoglobin (Hb) and reticulocyte count, and rate of acute chest syndrome (ACS). The SS/Sbeta0 group (n = 390) had lower mean SpO2 than the SC/Sbeta+ group (n = 195) (96.3% vs. 98.7%, P < 0.001). Among SS/Sbeta0 subjects, a decrease in steady-state SpO2 correlated with a decrease in Hb, an increase in reticulocytes, older age and male gender. These correlations were not found in the SC/Sbeta+ group. Prior ACS did not correlate with steady-state SpO2. A multivariate model explained 45% of the variability in SpO2, but only 5% of the variation in SpO2 was explained by Hb. We conclude that steady-state desaturation is common in individuals with SCD, but it appears to be unrelated to prior episodes of ACS and largely unexplained by chronic anaemia.
 ...
PMID:Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease. 1617 73

The erythropoietic system plays a major role in tissue oxygenation because the erythrocytes are the primary carriers of oxygen in the form of oxyhemoglobin. Therefore, clinical entities such as abnormal hemoglobins, polycythemia, anemia, and significant changes in blood volume frequently produce alterations in various respiratory functions. The pulmonary manifestations can vary from mild respiratory illness to life-threatening emergencies with high mortality rates. Among the hemoglobinopathies, sickle cell disease is clinically the most important and commonly associated with serious pulmonary consequences, including acute chest syndrome, pneumonia, infarction due to in situ thrombosis, bone marrow fat embolism of pulmonary vasculature, bone marrow infarction, pulmonary hypertension, and other abnormalities. Hemoglobinopathies with high and low affinity for oxygen and other abnormal hemoglobinopathies occasionally cause clinically significant respiratory complications by interfering with normal tissue oxygenation. Acquired methemoglobinemia can cause alarming cyanosis and medical emergency. Erythrocyte disorders are associated with pulmonary complications, including pulmonary hypertension, alveolar fibrosis, and pulmonary dysfunction. Coagulation disorders, both the inherited and acquired types, have the potential to affect the respiratory system in the form of hemorrhage from the airways, lung parenchyma, or pulmonary hypertension. The following paragraphs describe the common pulmonary complications and manifestations associated with hemoglobinopathies, erythrocyte disorders, and coagulation abnormalities.
 ...
PMID:Lungs in hemoglobinopathies, erythrocyte disorders, and hemorrhagic diatheses. 1626 4

We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.
 ...
PMID:Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. 1645 94

Human parvovirus (HPV) B19, a common infection, frequently causes transient red cell aplasia in children with hemolytic anemia, such as sickle cell disease (SCD). It was considered to be a self-limited condition, easily treated with blood transfusion. However, acute splenic sequestration, acute chest syndrome, nephrotic syndrome, and stroke have been reported in SCD patients following HPV B19 infection. We report a 3-year-old child with SCD who developed fulminant myocarditis following HPV B19-related aplastic crisis. The diagnosis of myocarditis should be considered in a patient with hemolytic anemia with an infection with HPV B19 who develops signs of cardiopulmonary failure despite correction of anemia.
 ...
PMID:Life threatening parvovirus B19 and herpes simplex virus associated acute myocardial dysfunction in a child with homozygous sickle cell disease. 1670 44

An infant with Hb SE disease is reported. He was clinically well. Review of the literature shows that patients aged 18 and younger are usually well. On the other hand, more than half of those aged 20 and older developed sickling-related complications, including potentially life-threatening acute chest syndrome. These patients have 60-65% Hb S, similar to the percent Hb S in patients with Hb S/beta(+)-thalassemia. Their hematological features and clinical course appear to parallel those of Hb S/beta(+)-thalassemia. Patients have variable levels of anemia, and some develop clinical complications. With population migrations and increasing racial intermarriages, Hb SE disease is expected to be encountered more often around the globe. Patients with Hb SE disease should be followed and managed in a similar fashion as those with Hb S/beta(+)-thalassemia, and treated appropriately when they develop sickling-related symptoms and complications.
 ...
PMID:Hemoglobin SE disease: a concise review. 1727 12


<< Previous 1 2 3 4 5 6 7 Next >>