Sickle Cell Anemia (Subscribe)

Categories

Patient Information SCD (8)
Information aimed at educating patients and parents of patients with Sickle Cell Disease

Links

Pages: 1 2 > >|

AVN of the femoral head in SCD

Avascular necrosis of the femoral head in sickle-cell disease. Treatment of collapse by the injection of acrylic cement. Hernigou P, Bachir D, Galacteros F. Henri Mondor Hospital, Creteil, France. In ten patients with sickle-cell disease, we used a new technique of cement injection for the treatment of 16 painful hips with a radiographic crescent line or flattening of the articular surface due to avascular necrosis. The necrotic bone and overlying cartilage are elevated by the injection to restore the sphericity of the femoral head. Five days after the operation, full weight-bearing was allowed with the help of crutches for three weeks. The time in hospital averaged eight days; the average blood loss was 100 ml. There was early pain relief and postoperative radiographs showed improvement in the shape of the femoral head. At a mean follow-up of 5 years (3 to 7), 14 of the 16 hips were still improved although some gave slight pain. Only two hips had required revision to total hip arthroplasty, at one year and two years respectively. The increasing longevity of patients with sickle-cell disease means that avascular necrosis will be an increasing problem. Total hip replacement has a poor prognosis because of the risks of infection, high blood loss, and early loosening. Cement injection does not have these problems and allows for earlier, more conservative surgery. (full text)

Review It Rate It Bookmark It

Bone and joint complications in sickle cell disease

Although sickle cell disease (SCD) is characterized by a marked heterogeneity in clinical and hematologic severity [1,2], bone and joint problems are the most common manifestations. Pain, caused by vaso-occlusive crises (VOC), may occur as often as every week or as rarely as once a year. Many patients also suffer from the long-term consequences of VOC, such as avascular necrosis of the femoral heads or collapsed vertebral bodies, leading to a chronic state of pain in addition to the more acute painful crises. Bone and joint complications in SCD are discussed here [3]. Other manifestations of the disease are discussed separately. Subscription need to see the full text

Review It Rate It Bookmark It

Case 53. Avascular Necrosis of the Hip in Sickle Cell Diseas

Avascular Necrosis of the Hip in Sickle Cell Disease

Review It Rate It Bookmark It

Google Search for Orthopaedic Aspects of SCD

Search String "sickle cell" osteonecrosis dactylitis osteomyelitis

Review It Rate It Bookmark It

Information Center for Sickle Cell and Thalassemic Disorders

The primary goal of the Information Center for Sickle Cell and Thalassemic Disorders is to provide the most accurate and up-to-date information on hemoglobin disorders, particularly sickle cell disease and thalassemia, as well as disordrers of iron metabolism. The information is designed to be useful to health care personnel, patients and students. Freely available information on diseases empowers patients to participate fully in their own care. The better one understands a problem, the better one can handle it.

Review It Rate It Bookmark It

Information for Health Professionals

This page is intended to give you the information you need to recognise sickle cell disease and to help someone who has it to lead a good life. Contents Basic Facts What are haemoglobin SC disease (Hb SC) and Sickle Beta-Thalassaemia (Hb S-Thal)? What are the symptoms of sickle cell disease? How to recognise a sickle cell crisis Diagnosis of Sickle Cell Anaemia and Sickle Cell Trait When to screen for sickle cell trait and disease Management of people who have sickle cell disorders Glossary Where to get further information

Review It Rate It Bookmark It

Management of Sickle Cell Disease

Sickle cell disease (SCD) results from the substitution of a valine residue for glutamic acid at position 6 in the beta-subunit of hemoglobin (Ingram, 1956). With a few minor exceptions, people with only one gene for hemoglobin S (Hb S) are phenotypically normal (sickle trait). People who inherit two Hb S genes from their parents have sickle cell disease. Deoxygenated Hb S tends to polymerize non-covalently into long strands that deform the erythrocyte, giving the characteristic "sickle cell" morphology (Eaton and Hofrichter, 1990). Hb S with bound oxygen (e.g., in the arterial circulation) does not polymerize. The mechanism by which these changes in the physical properties of the hemoglobin molecule produce the clinical manifestations of the disease is not unequivocally proven. The most widely accepted hypothesis is that erythrocytes deform as they release their oxygen in the capillaries and are trapped in the microcirculation (Eaton et al., 1976) (Kaul et al., 1989). The blockade of blood flow produces areas of tissue ischemia, leading to the myriad of clinical problems seen with sickle cell disease. Although a good deal of indirect evidence supports this theory, definitive proof that this is the pathophysiologic mechanism in sickle cell disease is lacking.

Review It Rate It Bookmark It

Managing sickle cell disease

Advances are being made in the management of sickle cell disease for all age groups. This review discusses the progress in amelioration of symptoms, problems unique to particular age groups, and the types of drugs and treatments currently under investigation (full text)

Review It Rate It Bookmark It

Multidisciplinary Management Ortho SuperSite

Multidisciplinary Management of Orthopedic Patients With Sickle Cell Disease Sickle cell disease, an autosomal-recessive disorder, produces abnormal hemoglobin and erythrocytes, which leads to hemolytic anemia and microvascular occlusion. Patients with sickle cell disease often present with orthopedic disease manifestations requiring surgical intervention, with the most common indications being osteonecrosis and osteomyelitis. This article includes a systematic approach to the surgical management of patients with sickle cell disease. (full text) By Sathappan S. Sathappan, MD; Daniel Ginat, MD, MS; Paul E. Di Cesare, MD ORTHOPEDICS 2006; 29:1094

Review It Rate It Bookmark It

Musculoskeletal Manifestations of Sickle Cell Disease

Bone Infarction Flow patterns in the marrow predispose it to infarction. The slowing of blood flow through the marrow space allows for regional hypoxia and thus sickling of red cells, leading to infarction. The proximal epiphysis of long bones, especially the humerus and femer is the most common location of infarction in sickle cell patients. Vertebral osteonecrosis and the formation of "fish vertebrae" is a classic finding. UW MSK Resident Projects

Review It Rate It Bookmark It

NHLBI Comprehensive Sickle Cell Centers

The Division of Blood Diseases and Resources (DBDR) of the National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), supports grants for the Comprehensive Sickle Cell Centers to focus on multi-disciplinary programs of basic, applied, and clinical research, and also to include relevant service activities in diagnosis, counseling and education concerning sickle cell disease and related disorders.

Review It Rate It Bookmark It

osteomyelitis in the sickle cell patient

Osteomyelitis: - look for OM in multiple areas and in the diaphisis of long bones as compared to the metaphysis in unaffected children; - diagnosis of osteomyelitis: in patients with sickle cell anemia is complicated by the difficulty in distinguishing the syptoms from those of acute bone crisis (bone infarction); - fever, localized or generalized bone pain, & localized erythema, tenderness, & swelling are characteristic of both conditions; - elevated sed rate & high leukocyte count are typical both w/ bone infarction & w/ osteomyelitis; - sickling crisises are thought to be more than 50 times more common than bacterial osteomyelitis; - aspiration of pus from bone confirms dx of osteomyelitis (more)

Review It Rate It Bookmark It

PubMed Search

Rapid Search using as seed, "Relative rates and features of musculoskeletal complications in adult sicklers" 2004 Paper from Cameroun

Review It Rate It Bookmark It

Sickle Cell Anaemia

Sickle cell disease is one of the more common abnormalities of haemoglobin. The single substitution of valine for glutamic acid alters the folding of the protein and its response to deoxygenation. The inheritance is autosomal dominent, but generally the disease is more severe in homozygous individuals. Other haemoglobinopathies may affect the expression of Sickle cell bone disease. The combination of Sickle and haemoglobin C, or B, thalassaemia have a higher risk of bone disease. Radiology Museum

Review It Rate It Bookmark It

Sickle Cell Anemia

Introduction Sickle Cell Anemia (SCA) is caused by a change in the chemical composition of the protein (hemoglobin or Hgb) that carries the oxygen inside of the red blood cells (RBC's). Normal Hgb is a round or ball-shaped folded molecule composed of 4 protein subunits - 2 alpha chains and 2 beta chains. The chemical change is a valine amino acid substituted for glutamic acid in both of the beta chains (HbSS). These chemical changes in hemoglobin cause the shape of the molecule to change under certain conditions such as lowered oxygen concentration and dehydration. Deoxygenated HgSS molecules can chemically link to each other, creating chains of molecules - a polymer. In turn, these abnormal elongated hemoglobin polymer structures distort the shape of the whole red blood cell. The abnormal RBC's can damage the vessels around them and the tissues that depend on the vessels for oxygen and nourishment. For example, the damaged RBC's can cause thrombosis (clotting) and then secondary ischemic damage to the adjacent and surrounding tissues - causing infarction (cellular death). (more information and images)

Review It Rate It Bookmark It

Pages: 1 2 > >|

Editors

  • Chris Oliver