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

All acute admissions of patients with sickle cell disease who lived in the London borough of Brent and attended this hospital were analysed for a period of one year. Sixty three of the 211 patients who were followed up by the haematology department required 161 acute admissions during the year. Most admissions (126) were for the 42 patients with homozygous sickle cell disease; 147 (91%) were for vaso-occlusive episodes, 142 of which were for painful crises, three for cerebrovascular accidents, and two for renal papillary necrosis. Preschool children with sickle cell disease were admitted predominantly with limb pain, whereas in schoolchildren and adults the incidence of trunk pain was higher. Twenty four of the 93 episodes of trunk pain culminated in an episode of severe visceral sequestration usually affecting the lungs, the liver, or the mesenteric circulation. Two patients died: an 18 month old baby with an acute splenic sequestration crisis and a 19 year old man with a severe girdle syndrome (sickling in the mesenteric circulation, liver, and lungs). Infective episodes were rare (11 episodes) but severe: one haemophilus meningitis, two salmonella infections, and three aplastic crises due to parvovirus infections. The average duration of the hospital stay was 7.4 days per admission. It is concluded that because sickle cell disease causes appreciable morbidity in older children, adolescents, and adults a systematic approach to management is needed to deal with acute episodes such as sequestration syndromes.
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PMID:Acute admissions of patients with sickle cell disease who live in Britain. 310 83

The diagnosis of acute autoimmune rheumatic disorders in sickle cell disease (SCD) can be challenging. Polymyositis is an inflammatory myopathy which, like SCD, may present with myalgia but is usually associated with proximal muscle weakness. We describe an adolescent boy presenting with limb pain, difficulty in mobilisation, with progressive loss of motor function and later bulbar weakness. Investigations showed massive elevation of creatine kinase, and MRI and muscle biopsy findings consistent with severe polymyositis. The patient was treated with corticosteroids, intravenous immunoglobulin and intensive rehabilitation therapy. He made a good recovery and was discharged on azathioprine and prednisolone. In the context of SCD, multisystem symptoms, unexplained muscle pain and weakness, unresponsive to conventional treatment in the presence of steady state haemoglobin, should alert the clinician to autoimmune phenomena. Key factors in making a diagnosis are an autoimmune screen and early discussion with a rheumatology expert.
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PMID:Juvenile polymyositis with unremitting pain and progressive loss of motor and bulbar function on a background of sickle cell disease. 2581 17

We report the case of a 22-year-old primigravida detected as having sickle cell disease (SCD), initially presenting in the third trimester (30th week) of pregnancy. The patient came to our center with a complaint of severe lower limb pain. The peripheral smear showed marked anisopoikilocytosis, numerous leptocytes, sickle cells, and target cells. High-performance liquid chromatography corroborated the diagnosis of SCD, showing a significant peak in the sickle window. The patient was conservatively managed and delivered a healthy baby through normal vaginal delivery. Delayed presentation of SCD in the third trimester of pregnancy is unusual. This report aims to bring attention to the possible causes of such a lag in detection. We also suggest measures to refine the antenatal healthcare screening at multiple levels, with regard to the detection of sickle cell hemoglobinopathy.
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PMID:Sickle cell disease presenting in the third trimester of pregnancy: Delayed detection heralding a public health problem? 3218 93