Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0030193 (pain)
 261,466 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Haematological, clinical and some molecular genetic features of homozygous sickle cell (SS) disease in Saudi Arabia have been compared in 33 patients from the Eastern Province (Eastern) and 30 from the South Western Province (Western). Eastern patients all had the Asian beta globin haplotype whereas Western patients were more variable but predominantly of the Benin haplotype. Eastern patients had more deletional alpha thalassaemia, higher total haemoglobin and fetal haemoglobin levels, and lower HbA2, mean cell volume, reticulocytes, and platelet counts. Clinically, Eastern patients had a greater persistence of splenomegaly, a more normal body build and greater subscapular skin fold thickness, and Western patients had more dactylitis and acute chest syndrome. Painful crises and avascular necrosis of the femoral head were common and occurred equally in both groups. The disease in the Eastern province has many mild features consistent with the higher HbF levels and more frequent alpha thalassaemia but bone pathology (painful crises, avascular necrosis of the femoral head, osteomyelitis) remains common. The disease in the West is more severe consistent with the Benin haplotype suggesting an African origin.
 ...
PMID:Two different forms of homozygous sickle cell disease occur in Saudi Arabia. 171 63

There is wide variation in the clinical manifestations of sickle cell disease (SCD) from one affected individual to another. Many investigators have sought to discern parameters that would explain this variability. In the present studies we have attempted to correlate the frequency of painful events and the extent of end organ failure in SCD with rheologic properties of packed suspensions of sickle cells, using a magneto-acoustic ball microrheometer developed in our laboratory. Using this device we have measured the steady-state viscosity, and the viscous and elastic moduli of cell suspensions in 16 individuals with hemoglobin SS disease who were untransfused and in their steady state. The rheologic parameters were then correlated with clinical parameters. The clinical parameters measured were emergency department visits, hospitalizations, hemoglobin, reticulocyte count, age, and end organ failure (nephropathy, avascular necrosis of bone, stroke, retinopathy, resting hypoxemia after acute chest syndrome(s), leg ulcer, and priapism with impotence). The P value for the correlation between the steady state viscosity and end organ failure was .001 with a correlation coefficient (R value) of .73. The P value for the correlation between the viscous modulus of viscosity and end organ failure was .00006 with an R value of .83. The P value for the correlation between the elastic modulus of viscosity and end organ failure was .0006 with an R value of .76. However, there was no significant correlation between any component of packed cell rheology and emergency department visits or hospitalizations for pain.
 ...
PMID:Relationship of clinical severity to packed cell rheology in sickle cell anemia. 182 65

Polymorphonuclear leukocyte (PMN) aggregation and chemotaxis were studied in 27 patients with sickle cell disease (SCD). Pain-free patients with SCD had a significantly impaired aggregation response to stimulation with n-formylmethionyl-leucyl-phenylalanine (FMLP) with or without cytochalasin B (CB), compared with normal volunteers (p less than 0.001). Patients with SCD in vaso-occlusive crisis had PMN aggregation induced by FMLP with or without CB that was significantly increased compared with the cohort of pain-free SCD patients (p less than 0.001). PMN from pain-free patients had normal chemotaxis, whereas patients in vaso-occlusive crisis had a significant impairment in PMN chemotaxis. PMN chemotaxis was inversely related to the PMN aggregation response to FMLP with CB (r = -0.75). Thus, the PMN from pain-free patients with SCD appears to have normal or decreased "stickiness" and to develop increased stickiness during vaso-occlusive crisis. The mechanisms responsible for these changes need further elucidation. Alterations in PMN function may be responsible, in part, for the increased risk of infection noted in individuals with SCD and may play a role in the development of the acute chest syndrome.
 ...
PMID:Vaso-occlusive crisis-associated neutrophil dysfunction in patients with sickle-cell disease. 366 21

Early symptoms were observed in a representative sample of 166 children with sickle cell-hemoglobin C disease diagnosed at birth. Symptoms were uncommon in the first year of life; in approximately 50% specific symptoms had developed by 5 years, but 22% remained without specific symptoms to 10 years. The age at presentation was significantly earlier in patients with low hemoglobin F levels, but was not influenced by heterozygous alpha-thalassemia-2. Painful crisis was the initial manifestation in 77% of the children; other symptoms included dactylitis (14%) and pneumococcal septicemia and acute splenic sequestration (4% each). The commonest nonspecific symptom was acute chest syndrome. The relatively mild early clinical course of sickle cell-hemoglobin C disease indicates that neonatal diagnosis does not have the same urgency as for homozygous sickle cell disease.
 ...
PMID:Clinical presentation of sickle cell-hemoglobin C disease. 376 Oct 71

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.
 ...
PMID:Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. 749

The acute chest syndrome is a clinical entity appearing in patients suffering from sickle cell anaemia. It presents with pleuritic pain, fever, leucocytosis and pulmonary infiltrates in the thoracic radiology. The etiological diagnosis is difficult, and it is necessary to distinguish between pneumonia and pulmonary infarction. This syndrome is quite frequent among the patients at risk, and can be lethal according to the severity and the etiology of the event. A case of acute chest syndrome due to a S. pneumoniae sepsis is presented. The interest of the case lies in the rareness of this disease in our population and the peculiar evolutive clinical features of this case, with the development of intracranial hypertension and death.
 ...
PMID:[Acute thoracic syndrome]. 798 60

In order to determine if a relationship exists between rib infarction and the acute chest syndrome (ACS) in sickle cell disease patients, bone scans were reviewed in 55 episodes in 38 patients with pain of suspected osseous origin. A bone scan was positive for thoracic bone infarction if abnormally increased or decreased uptake was present in ribs, sternum or thoracic spine. Radiographs were considered to be positive for ACS if there was pulmonary infiltrate or pleural effusion in the absence of laboratory or clinical evidence of bacterial pneumonia. ACS by chest x-ray was present in 22 episodes, 21 of which showed evidence of infarction of the bony thorax on bone scan. Thoracic bone infarction occurred in the absence of chest x-ray changes in only 11 episodes. This association between bone infarction and radiographic ACS was statistically significant (p < 0.001, Fisher's exact test). A strong association exists between ACS and infarction of the bony thorax. It is possible that bone infarction leads to pain, hypoventilation and the clinical picture of ACS.
 ...
PMID:Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. 845 78

Two hematologic emergencies are reviewed in this article: transfusion reactions and crises in patients who have sickle cell disease. Transfusion reactions may be due to incompatibility, IgA deficiency, allergy or, rarely, bacterial contamination of the blood product. A major hemolytic reaction due to incompatibility may progress to hypotension and shock. To prevent this type of reaction, blood products should be given only when necessary and attention should be given to eliminating clerical errors, which are responsible for many hemolytic reactions. In patients with sickle cell disease, a painful crisis due to vascular occlusion is the most common emergency. Rehydration is essential, and narcotics may be needed to relieve pain. Aplastic crisis is managed by transfusion of packed red blood cells and supportive care. Sickle cell crisis may affect major organ systems. The acute chest syndrome can be complicated by pneumonia; rapid respiratory failure may occur if multiple lobes are involved. Splenic or hepatic sequestration requires aggressive rehydration and transfusion. In patients who have had stroke or subarachnoid hemorrhage, a long-term exchange transfusion program is needed to keep hemoglobin S levels below 30%.
 ...
PMID:Hematologic emergencies. Management of transfusion reactions and crises in sickle cell disease. 846 76

Sickle cell lung disease occurs in all of the more common sickle hemoglobinopathies. Of the acute pulmonary diseases, acute chest syndrome (ACS) is most common. The risk of recurrence after one episode of ACS ranges from 20% to 80%. Repeated episodes of ACS contribute to the development of earlier and potentially rapid pulmonary deterioration particularly in young adults. ACS is the second most common cause for hospital admission and has been reported to be responsible for 25% of sickle cell deaths. The exact etiology of ACS may be unclear and is often caused by the interaction of a number of factors. Although infection is most likely in the young child, infarction and thromboembolism are the more likely causes in older patients. Outcome is dependent on immediate recognition and rapid institution of therapy. Maintenance of adequate oxygenation treatment of possible underlying infection, and adequate hemoglobin delivery are essential. Simple or exchange transfusion is vital to improve hemoglobin delivery and decrease hemoglobin S concentration. Caution is advised when administering drugs such as morphine for pain control because of risk of exacerbation of pulmonary symptomatology.
 ...
PMID:Chest infections and syndromes in sickle cell disease of childhood. 888 74

Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease (SCD). Previous studies reported conflicting pictures of ACS making therapeutic interventions difficult. The Cooperative Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled from birth to 66 years of age for ACS. Data on presenting signs and symptoms, laboratory findings, and hospital course were collected. There were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. Upper lobe disease was more common in children; multilobe and lower lobe disease affected adults more often. Severe hypoxia occurred in 18% of adults tested and could not be predicted by examination or laboratory findings. Bacteremia was documented in 3.5% of episodes, but was strongly influenced by age (14% of infants and 1.8% of patients > 10 years). ACS was most common in winter with children having the most striking increase. Transfusion was used less frequently, but earlier in children. Young children were hospitalized for 5.4 days versus 9 days for adults. Fifty percent of adults had a pain event in the 2 weeks preceding ACS and children were more likely to have febrile events. The death rate was four times higher in adults than in children. Fatal cases generally developed rapid pulmonary failure and one third were associated with bacteremia. Age has a striking effect on the clinical picture of ACS. In children, ACS was milder and more likely due to infection, whereas in adults ACS was severe, associated with pain and had a higher mortality rate.
 ...
PMID:Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. 905 64


1 2 3 4 5 6 7 8 9 10 Next >>