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Ohapi principles with sickle cell pdf#

It results from the substitution of glutamic acid by valine at position 6 of the β-chain of hemoglobin. These guidelines address more than 40 specific issues, such as abdominal pain, congestive heart failure, and infections.Sickle cell disease (SCD) was the first human monogenic disorder to be characterized at the molecular level ( 1). These guidelines supplement to current texts in general medicine, surgery and pediatrics. These protocols are guidelines in use at the Sickle Cell Center at Grady Health System, and they are intended for use by heath care providers treating patients with sickle cell syndromes.

Ohapi principles with sickle cell pdf#

Read more about these practice guidelines » pdf icon external icon Problem Oriented Clinical Guidelinesįrom the Sickle Cell Information Center, 1991

Hydroxyurea decreases crises in patients with severe sickle cell disease.

Transfusions to reduce Hb S levels to below 30 percent prevent strokes in children with high central nervous system blood flow.

Transfusions to maintain a hematocrit of more than 36 percent do not reduce complications of pregnancy.In surgical settings, simple transfusions to increase hemoglobin (Hb) levels to 10 g/dL are as good as or safer than aggressive transfusions to reduce sickle hemoglobin (Hb S) levels to below 30 percent.Pneumococcal vaccine prevents pneumococcal infection in children.Penicillin prophylaxis prevents pneumococcal sepsis in children.

The practice guidelines best supported by scientific evidence are:

pdf icon The Management of Sickle Cell Diseaseįrom the National Heart Lung and Blood Institute (NHLBI), 2014 Treat pain of patients with SCD promptly. It is essential that every child with SCD receive comprehensive care that is coordinated through a medical home with appropriate expertise. Timely and appropriate treatment of acute illness is critical, because life-threatening complications develop rapidly. The provision of comprehensive care is a time-intensive endeavor that includes ongoing patient and family education, periodic comprehensive evaluations and other disease-specific health maintenance services, psychosocial care, and genetic counseling. Specialized comprehensive medical care decreases morbidity and mortality during childhood. This statement provides pediatricians in primary care and subspecialty practice with an overview of the genetics, diagnosis, clinical manifestations, and treatment of SCD. Health Supervision for Children with Sickle Cell Diseaseįrom the American Academy of Pediatrics (AAP), 2011 Stem Cell Transplantation external icon.Management of Acute and Chronic Pain external icon.

Cardiopulmonary and Kidney Disease external icon.

The recommendations address treatment of both adult and pediatric SCD. ASH formed a committee of medical experts, researchers and patients to review evidence and form recommendations on SCD. In 2016, the American Society of Hematology (ASH) initiated an effort to develop clinical practice guidelines on Sickle Cell Disease (SCD). ASH Clinical Practice Guidelines on Sickle Cell Disease