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

Sickle cell disease is the commonest of the haemoglobinopathies. The pathophysiology consists on the one hand of chronic haemolytic anaemia whose course is punctuated by acute crises with pain, fever and severe haemolysis, and on the other of a tendency to microthrombotic accidents. Of all the very varied clinical features of the disease, cardiac signs are both common and variable. The authors report a series of 92 cases, and compare their findings with those in the literature.
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PMID:[Cardiovascular signs in drepanocytosis. Apropos of 12 cases]. 13 57

Recurrences of CNS infarction often lead to progressive neurologic disability in sickle cell anemia. To prevent such reccurrence, a periodic blood transfusion program was begun in 1969. Currently, 27 patients are on this regimen. Before inclusion in the program, 12 patients had had one to nine CNS recurrences each. Since the program was started, two patients have had transient CNS ischemia. There were no other recurrences and none of the patients have shown progression of neurologic abnormalities. In addition, there was a striking decrease in bacterial infection and pain. We conclude that periodic transfusions are effective in preventing recurrent CNS infarction in sickle cell anemia. The benefits must be weighed against the potentially serious problem of iron overload, as evidenced by moderately elevated serum ferritin values.
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PMID:Periodic transfusions for sickle cell anemia and CNS infarction. 51 76

An evaluation of acupuncture for pain relief was made in 10 patients with sickle cell anaemia during 16 pain crises. A model was developed in which the patient served as his own control and in which both patient and examiner were unaware of whether an acupuncture point or a sham site was treated. The results show (1) that pain relief was obtained in 15 of the 16 painful episodes regardless of whether an acupuncture point or a sham site was treated, demonstrating considerable overlap between the effects of needling acupuncture points and sham sites; (2) that needling at acupuncture points for pain relief is not significantly superior to treatment at sham sites; (3) that needling, per se, whether at acupuncture points of at sham sites can be useful for alleviating pain in sickle cell crises. The model could be useful for evaluation of pain relief by needling in other diseases.
Pain 1979 Oct
PMID:Acupuncture: an evaluation in the painful crises of sickle cell anaemia. 52 74

We studied 270 Saudi Arabs with homozygous sickle cell anemia, using chart review, a register (since 1969), and home visiting in 42 cases. Average follow-up for the total group was 10 years. Seventy-four percent of those diagnosed by age 3 years presented on screening or with merely anemia; 26% presented with illness, abnormal physical findings, or pain. Compared with American or Jamaican blacks, serious complications occurred only 6% to 25% as frequently; leg ulcers did not occur at all; the mortality under age 15 years was 10% as great; mean levels of blood hemoglobin were higher (10 g/dl), reticulocyte count was lower (5% to 6%), and mean fetal hemoglobin (HbF), which was inversely correlated with reticulocytes, was higher (22% to 26.8%). The high HbF is believed to account for the very mild clinical manifestations.
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PMID:Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects. 61 31

99mTc-diphosphonate bone scans of 13 patients in acute sickle crisis were analyzed. Twelve of the 13 patients had abnormal scans, with increased activity in joints and skull being a common finding. One patient with splenic calcification and two patients without splenic calcification had splenic uptake of diphosphonate. Most of the abnormal scans showed hot areas, with cold areas seen only in four cases. There was no correlation between the site of pain clinically and the abnormal areas on scanning. The pathophysiology of the abnormal uptake of radioactivity and the role of bone scanning on sickle cell disease are discussed.
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PMID:Bone scan in sickle cell crisis. 65 73

Fulminant pneumococcal infections are rare in teen-agers with sickle cell anemia. A 16-year-old black male with sickle cell anemia was treated as an outpatient for cryptogenic pain crisis, which delayed antibiotic therapy for primary pneumococcal septicemia for seven hours. This patient did not appear ill upon initial presentation but rapidly developed disseminated intravascular coagulation and died.
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PMID:Concurrent primary pneumococcemia, disseminated intravascular coagulation, and sickle cell anemia. 66 36

The effects of extracorporeal carbamylation were determined in four patients with sickle cell anemia. Treatments were carried out in clusters every 4 to 6 weeks over a 6-month period. Mean carbamylation achieved in vivo was 1.3 moles cyanate per mole of hemoglobin. The mean in vitro P50 during treatment was 22.1 mm. Hg as compared to the pretreatment value of 30 mm. Hg. Hemoglobin concentration was increased over the 6 months by 24 per cent. The treatment resulted in the stimulation of erythropoiesis. Treated cells had a prolonged survival of about 40 days, but bilirubin did not fall. Blood volume and heart size remained unchanged. Carbamylation did not appear to alter the degree of sickling at any given O2 saturation. Painful attacks continued through the treatment period with a frequency of 84 per cent and a severity-frequency index of 77 per cent of that in the control period. There was no evidence of cyanate toxicity associated with extracorporeal therapy.
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PMID:Extracorporeal treatment with cyanate in sickle cell disease: preliminary observations in four patients. 124 77

S-Homozygotes, SC heterozygotes, and S-beta-thalassaemia heterozygotes are the haemoglobinopathies which make up sickle cell disease. Although their clinical features are similar, as regards complications during pregnancy, Hb S-beta-thalassaemia most dangerous, the main causes of mortality being severe anemia, acute sequestration crisis, bacterial infections, painful episodes, and pulmonary bone marrow fat embolism. Folic acid and antimalarials (where these are indicated) are often successful in preventing severe anaemia. It is best to reserve blood transfusion to replace moderate loss or to correct gross anaemia quickly when this is considered severe enough to threaten life. Painful crises are particularly common towards the end of pregnancy and in treating these episodes, analgesics, antibiotics, and sometimes heparin are used. S-homozygote carries additional hazards. Because of the prevalence of pelvic contraction, fetopelvic disproportion is common and so the incidence of operative deliveries is high. Many fetuses are lost through an increased incidence of abortion and perinatal mortality. In the survivors, there is evidence of intrauterine growth retardation brought about by continuous maternal anaemia throughout pregnancy.
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PMID:Sickle cell disease in pregnancy. 126 39

Severe hyponatremia has been observed in three children with sickle cell disease, and mild hyponatremia was noted during 36% of random hospitalizations for sickle crisis secondary to vasoocclusion or infection. Serum and urinary electrolytes were therefore studied in such patients. Hyponatremia was found in 52% of patients hospitalized with pain and/or fever, even though they received large amounts of sodium intravenously. Urine sodium losses were high with frequent negative sodium balance and weight loss. When well, these patients did not demonstrate hyponatremia, although urinary salt losses appeared to be just as high, suggesting compensatory salt intake when the children are well. It is essential to monitor electrolytes and urinary losses to manage sickle crisis properly. Six to 11 mEq/kg/day of sodium is suggested as a usual need of these patients during crisis. Sickle cell disease patients have, in addition to the better known defect in concentration of urine, a functional defect in dilution of urine at least during periods of "crisis".
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PMID:Hyponatremia in sickle cell disease. A renal salt-losing state. 127 Nov 42

A significant number of children with sickle cell disease suffer from episodes of acute recurrent vaso-occlusive pain crisis. Unfortunately, very limited published information is available about specific pain management practices used for these patients. There is even less information available on the use of an interdisciplinary approach for management of sickle cell crisis pain. The purpose of this article is to review the genetic cellular pathophysiology, the sickling process, vaso-occlusive crisis, and management of pain associated with sickle cell disease in children. The article further examines an interdisciplinary approach for the management of vaso-occlusive pain crisis.
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PMID:A holistic approach to vaso-occlusive pain crisis in children with sickle cell disease. 129 5


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