Painful crisis Clinical features

Pain of variable severity affects the juxta-articular areas of the long bones, particularly adjacent to the knees, the elbows, the shoulders and ankles, the sternum and ribs, the spine, particularly the lumbar region, and the pelvis. Pain may be localized to one or two sites, although multiple sites are frequently involved. Occasionally pains are described as 'all over'. Severity varies from transient pain barely limiting function to a severe debilitating pain requiring hospital admission and parenteral pain relief for days. Painful areas may be tender to palpation, although this is not always the case. Fever is common, and dehydration may occur if the patient has been reluctant to drink. The urine is commonly reported as dark or red and contains increased porphyrin levels at the time of the painful crisis.

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