Overview
What is sickle cell disease (SCD)?
What are the signs and symptoms of sickle cell disease (SCD)?
What can trigger vaso-occlusive crisis in sickle cell disease (SCD)?
How is sickle cell disease (SCD) diagnosed?
Which lab tests are performed in with the workup of sickle cell disease (SCD)?
What is the role of imaging studies in the diagnosis of sickle cell disease (SCD)?
What are the goals of treatment in sickle cell disease (SCD)?
Which medications are used to treat sickle cell disease (SCD)?
What are the nonpharmacological approaches in the treatment of sickle cell disease (SCD)?
What is the benefit of the sickle cell trait (SCT) in malaria-endemic areas?
When is a less severe form of sickle cell disease (SCD) likely to occur?
What are the major sickle genotypes?
What is the sickle cell trait (SCT)?
What are the early manifestations of sickle cell disease (SCD)?
Is universal screening for sickle cell disease (SCD) mandated in the US?
What is the role of hemoglobin S (HbS) in the pathogenesis of sickle cell disease (SCD)?
What happens to hemoglobin S (HbS) under deoxy conditions?
What is the role of oxygen tension in the pathogenesis of sickle cell disease (SCD)?
What is the pathophysiology of the sickling process in sickle cell disease (SCD)?
What is the result of recurrent episodes of sickling in sickle cell disease (SCD)?
What happens when red blood cells (RBCs) sickle?
What is the roles of endothelial dysfunction in the pathophysiology of sickle cell disease (SCD)?
What is the role of very late antigen-4 (VLA-4) in the pathogenesis of sickle cell disease (SCD)?
What is the role of nitric oxide in the pathogenesis of sickle cell disease (SCD)?
What is the role of adhesion molecules in the pathogenesis of sickle cell disease (SCD)?
What causes sickle red blood cells (RBCs) to adhere to endothelium?
What causes sickle red blood cells (RBCs) to adhere to macrophages?
What is hemolysis and what is its role in the pathophysiology of sickle cell disease (SCD)?
When do the symptoms of sickle cell disease (SCD) develop?
Which physiological changes in red blood cells (RBCs) suggest sickle cell disease (SCD)?
Which processes result in bone and joint destruction in sickle cell disease (SCD)?
What causes osteonecrosis in sickle cell disease (SCD)?
Where does bone infarction typically occur in sickle cell disease (SCD)?
What is the presentation of osteonecrosis in sickle cell disease (SCD)?
What is the role of bone marrow hyperplasia in sickle cell disease (SCD)?
Which skeletal manifestations of sickle cell disease (SCD) are caused by bone marrow hyperplasia?
Which growth defects may occur in patients with sickle cell disease (SCD)?
What are the renal manifestations of sickle cell disease (SCD)?
What are the manifestations of sickle cell disease (SCD) in the spleen?
What is the pathogenesis of chronic hemolytic anemia in sickle cell disease (SCD)?
What is the pathogenesis of pulmonary hypertension in sickle cell disease (SCD)?
What is the major cause of morbidity and mortality in sickle cell disease (SCD)?
Why are patients with sickle cell disease (SCD) at higher risk of infection from common bacteria?
Where did sickle cell disease (SCD) originate?
How do variants of the hemoglobin S (HbS) gene affect the severity of malaria?
Which factors increase the risk of vaso-occlusive crises in sickle cell disease (SCD)?
Which factors often precede aplastic crises in sickle cell disease (SCD)?
What populations are most likely to develop sickle cell disease (SCD)?
What is the prevalence of the sickle gene in the US?
What is the prevalence of sickle cell disease (SCD) in the US?
What is the global prevalence of sickle cell disease (SCD)?
Is sickle cell disease (SCD) more common in males or females?
Does the clinical presentation of sickle cell disease (SCD) vary among age groups?
What is the prognosis of sickle cell disease (SCD)?
What is the socioeconomic impact of sickle cell disease (SCD)?
Which prognostic factors predict an adverse outcome in sickle cell disease (SCD)?
How does sickle cell disease (SCD) affect pregnancy?
What is the mortality rate of sickle cell disease (SCD)?
What is the survival rate for children with sickle cell disease (SCD)?
Has the mortality rate of sickle cell disease (SCD) improved over the past 30 years?
What is the life expectancy of patients with homozygous HbS (HbSS) sickle cell disease (SCD)?
Which factors have contributed to an increase in life expectancy in sickle cell disease (SCD)?
What are the possible complications for aging patients with sickle cell disease (SCD)?
What education should patients with sickle cell disease (SCD) receive?
When should medical care be sought by patients with sickle cell disease (SCD)?
What should be avoided by patients with sickle cell disease (SCD)?
How should families be educated about sickle cell disease (SCD)?
What encouragement can be given to families of patients with sickle cell disease (SCD)?
Presentation
What is the presentation of cholelithiasis (gallstones) in sickle cell disease (SCD)?
At what age do the symptoms of sickle cell disease (SCD) typically develop?
What is the most common clinical manifestation of sickle cell disease (SCD)?
How do pain crises develop in sickle cell disease (SCD)?
Which patterns of bone pain suggest sickle cell disease (SCD)?
What triggers vaso-occlusive crises in sickle cell disease (SCD)?
Can dehydration precipitate pain in vaso-occlusive crises in sickle cell disease (SCD)?
Can changes in body temperature trigger vaso-occlusive crises in sickle cell disease (SCD)?
Do patients with sickle cell disease (SCD) experience chronic pain?
When is anemia present in sickle cell disease (SCD)?
What are the symptoms of anemia in children with sickle cell disease (SCD)?
What causes aplastic crisis in sickle cell disease (SCD)?
When is splenic sequestration most likely to occur in patients with sickle cell disease (SCD)?
How is splenic sequestration managed in sickle cell disease (SCD)?
What causes extreme susceptibility to infection in sickle cell disease (SCD)?
Which organisms pose the greatest risk for infection in sickle cell disease (SCD)?
What are the effects of sickle cell disease (SCD) on growth and maturation?
What is the hand-foot syndrome in sickle cell disease (SCD)?
What is the disease course of hand-foot syndrome in sickle cell disease (SCD)?
How does acute chest syndrome present in children and adults with sickle cell disease (SCD)?
Should acute chest syndrome in sickle cell disease (SCD) be treated as a medical emergency?
How does acute chest syndrome in sickle cell disease (SCD) begin?
What are the central nervous system (CNS) manifestations of sickle cell disease (SCD)?
When are patients with sickle cell disease (SCD) at risk for convulsions?
Are patients with sickle cell disease (SCD) at increased risk for hemorrhagic stroke?
Which cardiac complications may be present in sickle cell disease (SCD)?
What findings suggest cholecystitis in patients with sickle cell disease (SCD)?
How is the renal system involved in sickle cell disease (SCD)?
What are the possible ocular (ophthalmic) complications of sickle cell disease (SCD)?
What causes leg ulcers in sickle cell disease (SCD)?
How common is priapism in sickle cell disease (SCD)?
Which patients with sickle cell disease (SCD) are at an increased risk for avascular necrosis (AVN)?
How is hip disease managed in patients with sickle cell disease (SCD)?
How does pulmonary hypertension develop in sickle cell disease (SCD)?
What is the incidence of pulmonary hypertension in patients with sickle cell disease (SCD)?
How is pulmonary hypertension characterized in sickle cell disease (SCD)?
What are the physical findings in sickle cell disease (SCD)?
What does hypotension and tachycardia indicate in sickle cell disease (SCD)?
Which symptoms suggest cardiovascular complications in sickle cell disease (SCD)?
What does a fever suggest in children with sickle cell disease (SCD)?
What is the role of auscultation in the physical exam in patients with sickle cell disease (SCD)?
How is spleen size measured in children with sickle cell disease (SCD)?
When do growth parameters typically fall in children with sickle cell disease (SCD)?
What physical exam should be performed in patients with sickle cell disease (SCD)?
What are ocular (ophthalmic) manifestations in sickle cell disease (SCD)?
When should meningitis be suspected in patients with sickle cell disease (SCD)?
Which skeletal findings are typical in children with sickle cell disease (SCD)?
Which physical findings suggest acute bone infarction in patients with sickle cell disease (SCD)?
What is hand-food syndrome in patients with sickle cell disease (SCD)?
What are the symptoms of osteonecrosis in patients with sickle cell disease (SCD)?
What causes osteomyelitis in patients with sickle cell disease (SCD)?
DDX
How is HbS-beta 0 thalassemia diagnosed?
How is the diagnosis of sickle cell disease (SCD) confirmed?
What is the presentation of hemoglobin C (HbSC) disease?
How is hemoglobin C (HbSC) disease diagnosed?
What is the sickle cell trait (SCT)?
How do hemoglobin S (HbS) variants in sickle cell disease (SCD) occur?
How are Gaucher disease and sickle cell disease (SCD) differentiated?
Which conditions should be included in the differential diagnoses of sickle cell disease (SCD)?
What are the differential diagnoses for Sickle Cell Disease (SCD)?
Workup
When is screening for hemoglobin S (HbS) done in the US?
Which test is performed for the prenatal diagnosis of sickle cell disease (SCD)?
What is the role of lumbar puncture in the management of children with sickle cell disease (SCD)?
Which high-risk features of sickle cell disease (SCD) require hospitalization?
What is the presentation of acute chest syndrome in patients with sickle cell disease (SCD)?
What are the newborn screening guidelines for hemoglobin disorders in the US?
Which hemoglobin screening results suggest sickle cell disease (SCD)?
What is the role of hemoglobin electrophoresis a in the diagnosis of sickle cell disease (SCD)?
Which hemoglobin electrophoresis findings suggest sickle cell disease (SCD)?
What are the typical baseline blood study abnormalities in patients with sickle cell disease (SCD)?
Which peripheral blood smear findings suggest sickle cell disease (SCD)?
What is the suggested schedule for routine lab testing for sickle cell disease (SCD)?
Which tests should be performed in febrile children with sickle cell disease (SCD)?
Which CBC count findings suggest hematologic crisis in sickle cell disease (SCD)?
What is the role of reticulocyte percentage testing in sickle cell disease (SCD)?
What additional tests may be useful for a diagnosis of hemolysis in sickle cell disease (SCD)?
When are ABG measurements indicated in patients with sickle cell disease (SCD)?
When should a urinalysis be performed in patients with sickle cell disease (SCD)?
When is a sickling test indicated in patients with sickle cell disease (SCD)?
When is chest radiography indicated in patients with sickle cell disease (SCD)?
What is the role of radiography of the extremities in patients with sickle cell disease (SCD)?
What is the role of MR) in the workup of sickle cell disease (SCD)?
What is the role of CT scanning in the management of sickle cell disease (SCD)?
What is the role of nuclear medicine scanning in the management of sickle cell disease (SCD)?
What is the role of 99m Tc bone scanning in the management of sickle cell disease (SCD)?
What is the role of indium-111 (111In) WBC scanning in the management of sickle cell disease (SCD)?
What is the role of abdominal ultrasonography in the management of sickle cell disease (SCD)?
What is the role of ultrasonography of the kidneys in the management of sickle cell disease (SCD)?
What is the role of ECG in the management of sickle cell disease (SCD)?
Treatment
According to the NIH, how is optimal care for sickle cell disease (SCD) achieved?
What is self-treatment for sickle cell disease (SCD)?
What should be included in pain management for sickle cell disease (SCD)?
What are the goals of treatment for sickle cell disease (SCD)?
Is there a cure for sickle cell disease (SCD)?
What are the guidelines for the treatment of sickle cell disease (SCD)?
What new drugs are approved for use in the treatment of sickle cell disease (SCD)?
What is the role of gene therapy in the treatment of sickle cell anemia (SCA)?
What are the effects of hydroxyurea in patients with sickle cell disease (SCD)?
Is hydroxyurea an effective treatment for adults with sickle cell disease (SCD)?
Is hydroxyurea an effective treatment for children with sickle cell disease (SCD)?
What are the indications for hydroxyurea in patients with sickle cell disease (SCD)?
What monitoring is required for patients with sickle cell disease (SCD) receiving hydroxyurea?
What are the possible adverse effects of hydroxyurea in patients with sickle cell disease (SCD)?
What are the treatment options after failed hydroxyurea therapy for sickle cell disease (SCD)?
When are transfusions indicated in the treatment of sickle cell disease (SCD)?
What are possible transfusion-related complications in sickle cell disease (SCD)?
Which agents are used for iron chelation in patients with sickle cell disease (SCD)?
What is the role of erythrocytapheresis in the treatment of sickle cell disease (SCD)?
How is an erythrocytapheresis performed in the treatment of sickle cell disease (SCD)?
How are ophthalmic manifestations of sickle cell disease managed?
How is elevated intraocular pressure treated in patients with sickle cell anemia (SCA)?
How are vaso-occlusive crises treated in patients with sickle cell disease (SCD)?
What are the NICE guidelines for pain control in sickle cell disease (SCD)?
What are the NHLB recommendations for pain management in the treatment of sickle cell disease (SCD)?
How should morphine be administered in patients with sickle cell disease (SCD)?
How is acute chest syndrome (ACS) treated in patients with sickle cell disease (SCD)?
How is chronic pain treated in patients with sickle cell disease (SCD)?
Is hydroxyurea used to treat chronic pain in patients with sickle cell disease (SCD)?
What are the nonpharmacological approaches to pain management in sickle cell disease (SCD)?
What can family members and patients do to manage recurring pain of sickle cell disease (SCD)?
When is hospitalization indicated for the management of pain in sickle cell disease (SCD)?
Which dosage regimen is used to treat chronic pain in patients with sickle cell disease (SCD)?
When is chronic opioid therapy (COT) indicated in the treatment of sickle cell disease (SCD)?
How is megaloblastic anemia prevented in patients with sickle cell disease (SCD)?
Does menstruation cause iron deficiency (chronic anemia) in women with sickle cell disease (SCD)?
What is the role of voxelotor in the treatment of sickle cell anemia (SCA)?
How is anemic crisis with splenic sequestration treated in patients with sickle cell disease (SCD)?
What is the role of immunization in preventing infection in children with sickle cell disease (SCD)?
How is acute cholecystitis treated in patients with sickle cell disease (SCD)?
What is the initial treatment for priapism in patients with sickle cell disease (SCD)?
What are the complications of priapism in patients with sickle cell disease (SCD)?
How is recurrent priapism prevented in patients with sickle cell disease (SCD)?
How are leg ulcers treated in patients with sickle cell disease (SCD)?
What is the standard care for stroke prevention in children with sickle cell disease (SCD)?
What is an alternative to simple transfusion in the treatment of sickle cell disease (SCD)?
How is symptomatic pulmonary hypertension managed in patients with sickle cell disease (SCD)?
How are hip complications of sickle cell disease (SCD) (avascular necrosis) treated?
Which psychiatric disorders can be caused by sickle cell disease (SCD) and how can they be treated?
What are the restrictions to stem cell transplantation (SCT) for sickle cell disease (SCD)?
Are there dietary restrictions for patients with sickle cell disease (SCD)?
Are there activity restrictions for patients with sickle cell disease (SCD)?
Which activities should be encouraged in children with sickle cell disease (SCD)?
Are there investigational treatments for sickle cell disease (SCD)?
Which specialist consultations may be required in the treatment of sickle cell disease (SCD)?
Which specialist consultations may be required for complications of sickle cell disease (SCD)?
How often is follow-up required in the treatment of sickle cell disease (SCD)?
What education should patients receive about long-term management of sickle cell disease (SCD)?
What follow-up care is required for patients with proliferative sickle retinopathy (PSR)?
Should fluid intake and output be monitored in the treatment of sickle cell disease (SCD)?
What is recommended long-term monitoring for infants and children with sickle cell disease (SCD)?
Guidelines
What does ASH recommend for the prevention of stroke in patients with sickle cell disease (SCD)?
What are the ASH guidelines on transfusion support for patients with sickle cell disease (SCD)?
Medications
What drugs are used in treatment of sickle cell disease (SCD)?
Which medications in the drug class Vitamins are used in the treatment of Sickle Cell Disease (SCD)?
Which medications in the drug class Vaccines are used in the treatment of Sickle Cell Disease (SCD)?
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Molecular and cellular changes of hemoglobin S.
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Skeletal sickle cell anemia. H vertebrae. Lateral view of the spine shows angular depression of the central portion of each upper and lower endplate.
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Peripheral blood with sickled cells at 400X magnification. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
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Peripheral blood smear with sickled cells at 1000X magnification. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
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Peripheral blood smear with Howell-Jolly body, indicating functional asplenism. Courtesy of U. Woermann, MD, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland.
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Effects of therapy with hydroxyurea.
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Skeletal sickle cell anemia. Bone-within-bone appearance. Following multiple infarctions of the long bones, sclerosis may assume the appearance of a bone within a bone, reflecting the old cortex within the new cortex.
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A 12-year-old boy with HgbSS disease presents to the pediatric emergency department after his mother tried to wake him for school this morning and noted altered mental status, left-sided gaze paralysis with his head tilted to the left, and flaccid paralysis of the right arm and leg. A CT scan of the brain was obtained immediately.
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Embolic stroke of the left middle cerebral artery. SCD is the most common cause of stroke in children and one of the most devastating complications of SCD. Clinically overt strokes are typically due to embolism of the intracranial internal carotid artery and proximal middle cerebral artery (MCA), while "silent strokes" more typically occur in the smaller lacunar and penetrating arteries. As RBCs undergo sickling and hemolysis within the cerebral circulation, they adhere to the vascular endothelium and promote a hypercoaguable state and fuel thromboembolism formation. Treatment options include prophylactic therapy with hydroxyurea to promote HgbF concentrations and monitoring via transcranial Doppler to evaluate MCA blood flow velocity. Children found to have high velocities are at increased risk for stroke and commonly receive RBC transfusions to decrease the concentration of HgbS.
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The spleen enlarges during the first year of life in SCD, as it becomes congested with trapped slow-flowing sickled cells within the splenic sinuses and reticuloendothelial system. The histology image shown demonstrates splenic congestion from sequestered sickled RBCs (arrows). Microvascular occlusions produce chronic tissue hypoxia and microinfarctions. Over time, fibrosis induces autosplenectomy. With functional asplenia, patients are particularly susceptible to infection by the encapsulated organisms Streptococcus pneumoniae and Haemophilus influenzae. Vaccination and prophylactic daily penicillin throughout childhood are mainstays of treatment to prevent sepsis and meningitis
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Splenic sequestration is an important cause of morbidity that occurs when sudden splenic pooling of blood within the reticuloendothelial system causes an acute drop in circulatory volume and shock-like symptoms (hypotension, tachycardia) with a rigid distended abdomen. It is an acute emergency and can be fatal in 1-2 hours secondary to circulatory hypovolemia. Treatment is with volume resuscitation and blood transfusion. The CT image shown demonstrates splenomegaly with a mass-like process (arrows) from splenic sequestration.
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Patients with SCD are also at increased risk of developing pulmonary arterial hypertension (PAH). The etiology is most likely multifactorial but likely related to increased cardiac output secondary to underlying chronic anemia. Impedance to the elevated blood flow will cause further dilation and increase in pulmonary pressures. Postsickling changes including interstitial fibrosis secondary to vaso-occlusive crisis of ACS and hypoperfusion with resultant hypoxia of the pulmonary vascular beds are both proposed offenders inciting further dilation and elevation of pulmonary pressures. A pulmonary arteriogram depicting the markedly dilated vascular supply of the lungs seen in PAH is shown.
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Proliferative sickle cell retinopathy. Sickle cell retinopathy is believed to be vaso-occlusion of peripheral arterioles of the retina leading to retinal hypoxia, ischemia, and infarction. New vessels then form at the junction of the vascular and avascular areas of retina. This neovascularization of retinal tissue and resultant traction of fibrovascularization places patients at risk for vitreous hemorrhage (arrows) and retinal detachment. Another common ocular manifestation is hyphema. Anterior chamber bleeding occurs spontaneously, but sickled erythrocytes obstruct the trabecular meshwork leading to significant elevations of intraocular pressure. Patients are particularly susceptible to glaucomatous optic nerve damage from even mildly elevated intraocular pressures. Pressures greater than 36 mm Hg for 24 hours are an indication for surgical drainage in both SCD and sickle cell trait, regardless of the size of hyphema. Image courtesy of the National Eye Institute, National Institutes of Health (NEI/NIH).
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A 19-year-old man with known HgbSS disease presents because his girlfriend reports his eyes are yellow. He has no complaints. Physical exam is notable for mild abdominal pain, but is otherwise within normal limits. What imaging test is warranted for this work-up? The image shown is of a male child with similar symptoms. Image courtesy of Wikimedia Commons.
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Right upper quadrant ultrasoundChronic hemolysis of sickled cells in HgbSS disease and high heme turnover yields hyperbilirubinemia and is associated with increased formation of bile stones. Stone formation occurs as substances in bile reach concentrations that approach the limits of their solubility. As saturation levels are reached, crystals precipitate, become trapped in mucus, and produce sludge (shown). Over time, the crystals aggregate and form stones. Occlusion of the biliary tree by sludge and/or stones produces clinical disease, typically right upper quadrant pain. The scleral icterus seen in the image of the previous slide is most likely secondary to the elevated circulating levels of bilirubin as a result of an acute hemolytic event (such as an acute vaso-occlusive crisis).
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Renal papillary necrosisThe microvascular beds of the renal parenchyma are susceptible to sickling and vaso-occlusive crisis because of their inherent low-oxygen and high-osmolarity state. Depending on the location of occlusion, symptoms vary from a decreased ability to concentrate urine (yielding nocturia and polyuria), a disruption of exchange mechanisms (yielding hyperkalemia) or hematuria, which further damages renal tubules. In renal papillary necrosis, repeated vascular occlusion infarcts the renal medullary pyramids and papillae. This causes sloughing of papillae, which obstructs the urinary tract. Treatment options include hydration, high-dose antibiotics for resulting pyelonephritis, and possible percutaneous nephrostomy tube or invasive retrieval of sloughed papillae in acute urinary obstruction. The intravenous pyelogram demonstrates the "egg-in-a-cup" appearance of sloughed renal papillae (arrows) secondary to renal papillary necrosis.
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Skeletal sickle cell anemia. Hand-foot syndrome. Soft tissue swelling with periosteal new-bone formation and a moth-eaten lytic process at the proximal aspect of the fourth phalanx.
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Skeletal sickle cell anemia. Advanced dactylitis. Lytic processes are present at the first and fifth metacarpals, along with periostitis, which is most prominent in the third metacarpal.
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Skeletal sickle cell anemia. Expanded medullary cavity. The diploic space is markedly widened due to marrow hyperplasia. Trabeculae are oriented perpendicular to the inner table, giving a hair-on-end appearance.
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Skeletal sickle cell anemia. Detailed view of the expanded medullary cavity in the same patient as in the previous image.
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Skeletal sickle cell anemia. Osteonecrosis. Image shows flattening of the femoral heads with a mixture of sclerosis and lucency characteristic of osteonecrosis.
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Skeletal sickle cell anemia. Osteonecrosis. Detail of the right hip.
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Skeletal sickle cell anemia. Osteonecrosis. Detail of the left hip.
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Skeletal sickle cell anemia. Bone infarct. Image shows patchy sclerosis of the humeral head and shaft representing multiple prior bone infarcts.
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Skeletal sickle cell anemia. Chronic infarcts and secondary osteoarthritis. Image shows advanced changes of irregular sclerosis and lucency on both sides of the knee joint reflecting numerous prior infarcts. The joint surfaces are irregular and the cartilages are narrowed due to secondary osteoarthritis.
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Skeletal sickle cell anemia. Osteonecrosis. Coronal T1-weighted MRI shows a slightly flattened femoral head with a serpentine margin of low signal intensity around an area of ischemic marrow with signal intensity similar to that of fat.
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Skeletal sickle cell anemia. Osteonecrosis in the same patient as in the previous image. Coronal T2-weighted MRI shows a serpentine area of low signal intensity and additional focal areas of abnormal low signal intensity in the femoral head; these findings reflect collapse of bone and sclerosis.
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Skeletal sickle cell anemia. Osteomyelitis. CT scan in a soft tissue window demonstrates a large abscess in the left thigh encircling the femur, with hypoattenuating pus surrounded by a rim of vivid enhancement.
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Skeletal sickle cell anemia. Osteomyelitis and bone-within-bone. Bone-window CT scan in the same patient as in the previous image shows a bone-within-bone appearance (concentric rings of cortical bone) in the right femur. On the left, a sinus tract (cloaca) traverses the lateral aspect of the femoral cortex, and a small, shardlike sequestrum is present deep to the sinus tract.
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Skeletal sickle cell anemia. Bone scan of bone infarct shows an area of increased uptake in the distal femoral metaphysis corresponding to the infarct demonstrated on the previous plain radiograph.
Tables
Tests |
Age |
Frequency |
CBC count with WBC differential, reticulocyte count |
3-24 mo >24 mo |
every 3 mo every 6 mo |
Percent Hb F |
6-24 mo >24 mo |
every 6 mo annually |
Renal function (creatinine, BUN, urinalysis) |
≥ 12 mo |
annually |
Hepatobiliary function (ALT, fractionated bilirubin) |
≥ 12 mo |
annually |
Pulmonary function (transcutaneous O2 saturation) |
≥ 12 mo |
every 6 mo |
What would you like to print?
- Overview
- Presentation
- DDx
- Workup
- Approach Considerations
- Baseline Blood Study Abnormalities
- Suggested Routine Clinical Laboratory Evaluations
- Laboratory Studies in the Ill Child
- Additional Tests
- Radiography
- Magnetic Resonance Imaging
- Nuclear Medicine Scans
- Transcranial Doppler Ultrasonography
- Abdominal Ultrasonography
- Echocardiography
- Show All
- Treatment
- Approach Considerations
- Hydroxyurea Therapy
- Transfusion
- Management of Ophthalmic Manifestations
- Vaso-Occlusive Crisis Management
- Control of Acute Pain
- Treatment of Acute Chest Syndrome
- Control of Chronic Pain
- Management of Chronic Anemia
- Prevention and Treatment of Infections
- Treatment of Gallstones
- Treatment of Priapism
- Treatment of Leg Ulcers
- Stroke Prevention
- Treatment of Pulmonary Hypertension
- Sickle Cell Nephropathy
- Treatment of Other Complications
- Stem Cell Transplantation
- Diet and Activity Restrictions
- Investigational Treatments
- Consultations
- Long-Term Monitoring
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- Guidelines
- Medication
- Medication Summary
- Antimetabolites
- Opioid Analgesics
- Nonsteroidal Analgesics
- Tricyclic Antidepressants
- Vitamins
- Nutritionals
- Hemoglobin Oxygen-Affinity Modulators
- P-Selectin Inhibitor
- Antibiotics
- Phosphodiesterase Type 5 Inhibitors
- Endothelin Receptor Antagonists
- Iron Chelators
- Antiemetics
- Adrenergic Agonists
- Vaccines
- Show All
- Questions & Answers
- Media Gallery
- Tables
- References