Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Low back pain in children and adolescents is often caused by a serious problem. The diagnostic investigation should begin with a complete patient history, complete physical examination, laboratory testing, and plain radiography. These results should be used to direct further imaging studies. Although most of the painful injuries that children sustain in recreational activities are mild, back pain that lasts for extended periods may be due to various disorders, including spondylolysis and spondylolisthesis, disk herniation, Scheuermann disease, or neoplasms. Low back pain can also be caused by diskitis and osteomyelitis, most commonly found in children younger than 10 years old. Primary osseous neoplasms of the lumbar spine are uncommon, with Ewing sarcoma, aneurysmal bone cyst, benign osteoblastoma, and osteoid osteoma being the most common followed by primary lymphoma. These lesions occur more often between the ages of 5 and 20 years. Other causes of low back pain include spinal cord tumors (eg, ependymoma), congenital disorders of the spine (eg, scoliosis), and systemic disease (eg, sickle cell disease).
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PMID:Common causes of low back pain in children. 182 29

Examined the relationship between perception of pain, personality, coping and the reactions of family members in three chronic pain groups (sickle cell anemia, arthritis, and low back pain). Sixty black Ss equally distributed in the three medically diagnosed pain groups completed the McGill Pain Questionnaire, Maudsley Personality Inventory, a self-control measure, and the modified spouse response questionnaire. Nonsignificant association was detected between measures of pain and neuroticism; a significant inverse relationship was found between self-controlling behaviors and the affective dimension of pain; the relationship between perceived solicitous behavior and intensity and quality of pain differentiated the sickle cell and arthritic groups from the low back pain group; for the low back pain group, intensity of pain was related significantly to solicitous behaviors of family members, but the reverse was true for the arthritic and sickle cell groups.
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PMID:The relationship between strategies of coping and perception of pain in three chronic pain groups. 623 46

There have been few epidemiological studies of bone and joint diseases in black Africa. Available data were generated by hospital studies which were inevitably flawed by selection bias. They found that the incidence and/or severity of rheumatoid arthritis were reduced in West Africa but not in urban areas of Southern and East Africa, as compared with industrialized countries. Ankylosing spondylitis was infrequent. The human immunodeficiency virus epidemic can be expected to increase the prevalence of spondyloarthropathies despite the fact that few black Africans are HLA B27-positive. Gout was the most common inflammatory joint disease seen in inpatients in West Africa and Equatorial Africa. Osteoarthritis of the fingers or hip and dysplasia of the hip were infrequent. The main causes of hip symptoms were sickle cell anemia and hemoglobin C disease whose manifestations include bone necrosis, osteomyelitis, and attacks of bone and joint pain. Osteoarthritis of the knee was common in West and Southern Africa, especially in obese women. Low back pain and sciatica due to disc herniation were as common as in Europe. Lumbar canal stenosis appeared more common in West Africa than in Southern Africa, with a predominance in females. Postmenopausal osteoporosis was exceedingly rare. Infectious diseases were prevalent as a result of underindustrialization and defective hygiene. The paucity of rheumatologists, young mean age of the population, and scarcity of population-based studies are sources of bias which should be taken into account when interpreting the available data on rheumatological diseases in black Africa. In the future, more rigorous studies made possible by increased access to health care will provide improved insight into the semiology and epidemiology of bone and joint diseases in this area.
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PMID:[Rheumatic diseases in black Africa]. 812 80

Pyogenic infection of the intervertebral disc (discitis) is a rare infection and the diagnosis often depends on a high index of suspicion. The cases of infective discitis described in the modern literature are similar to, if not identical with what was described as 'typhoid spine'. Salmonella infection of the musculoskeletal system on the other hand is more common in patients with sickle cell anaemia. This case report highlights the bizarre presentation of infective lumbar discitis in a sickler (HbSS) and calls attention to the need for a thorough evaluation of low back pain in these patients.
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PMID:Infective lumbar discitis in a sickler--an occult 'typhoid' spine? 1469 57

Disorders of the skeleton are one of the most common causes of chronic pain and long-term physical disability in the world. Chronic skeletal pain is caused by a remarkably diverse group of conditions including trauma-induced fracture, osteoarthritis, osteoporosis, low back pain, orthopedic procedures, celiac disease, sickle cell disease and bone cancer. While these disorders are diverse, what they share in common is that when chronic skeletal pain occurs in these disorders, there are currently few therapies that can fully control the pain without significant unwanted side effects. In this review we focus on recent advances in our knowledge concerning the unique population of primary afferent sensory nerve fibers that innervate the skeleton, the nociceptive and neuropathic mechanisms that are involved in driving skeletal pain, and the neurochemical and structural changes that can occur in sensory and sympathetic nerve fibers and the CNS in chronic skeletal pain. We also discuss therapies targeting nerve growth factor or sclerostin for treating skeletal pain. These therapies have provided unique insight into the factors that drive skeletal pain and the structural decline that occurs in the aging skeleton. We conclude by discussing how these advances have changed our understanding and potentially the therapeutic options for treating and/or preventing chronic pain in the injured, diseased and aged skeleton.
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PMID:The neurobiology of skeletal pain. 2449 89

Disorders of the skeleton are frequently accompanied by bone pain and a decline in the functional status of the patient. Bone pain occurs following a variety of injuries and diseases including bone fracture, osteoarthritis, low back pain, orthopedic surgery, fibrous dysplasia, rare bone diseases, sickle cell disease and bone cancer. In the past 2 decades, significant progress has been made in understanding the unique population of sensory and sympathetic nerves that innervate bone and the mechanisms that drive bone pain. Following physical injury of bone, mechanotranducers expressed by sensory nerve fibres that innervate bone are activated and sensitized so that even normally non-noxious loading or movement of bone is now being perceived as noxious. Injury of the bone also causes release of factors that; directly excite and sensitize sensory nerve fibres, upregulate proalgesic neurotransmitters, receptors and ion channels expressed by sensory neurons, induce ectopic sprouting of sensory and sympathetic nerve fibres resulting in a hyper-innervation of bone, and central sensitization in the brain that amplifies pain. Many of these mechanisms appear to be involved in driving both nonmalignant and malignant bone pain. Results from human clinical trials suggest that mechanism-based therapies that attenuate one type of bone pain are often effective in attenuating pain in other seemingly unrelated bone diseases. Understanding the specific mechanisms that drive bone pain in different diseases and developing mechanism-based therapies to control this pain has the potential to fundamentally change the quality of life and functional status of patients suffering from bone pain.
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PMID:Mechanisms that drive bone pain across the lifespan. 3035 85

Sickle cell anemia (SCA) is an inherited disorder of hemoglobin due to the presence of abnormal hemoglobin in a homozygous state. Manifestation is usually in infancy or early childhood due to gradual decrease in hemoglobin F level as age advances. Diagnosis in middle age is unusual. We present a woman who was diagnosed of SCA for the first time at middle age. The aim was to bring to the knowledge of physicians that patients with SCA can also present late so high index of suspicion is required to make diagnosis. A 52-year-old woman presented to orthopedic clinic with complaints of generalized bone pain and low back pain. There was no history of trauma prior to the onset of the pain. There was no associated fever, weight loss, loss of appetite, nor weakness of the lower limbs. X-ray of the spine done showed wedge collapse of the 12th thoracic and first lumbar vertebrae with posterior angulation of the thoracolumbar junction giving dorsal kyphosis. Her mode of presentation raised a suspicion of tuberculosis of the spine to rule out multiple myeloma. However, investigations for tuberculosis and multiple myeloma were all negative. This necessitated the investigation for SCA and the diagnosis was confirmed. The diagnosis of SCA is usually made in infancy or early childhood. High index of suspicion is required to make the diagnosis at middle age.
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PMID:Hemoglobin SS Nigerian Woman First Diagnosed at the Age of 52 years with Manifestation Mimicking Tuberculosis of the Spine. 3267 4