the appearance of a new infiltrate on chest x-ray accompanied by acute respiratory symptoms<\/em><\/p>\nCAP agents are the main cause in addition to contribution from thrombosis and thromboembolism.<\/p>\n
2 of the following: chest pain, fever, infiltrate or V\/Q abnormality, respiratory symptoms, hypoxemia \nAdult patients with ACS tend to be afebrile, have severe pain, and often have multilobar disease combined with high mortality.<\/p>\n
In contrast, children are more often febrile and usually present with cough without chest pain.<\/p>\n
While the chest film is important in diagnosing ACS, the initial radiograph is normal in almost half of patients who ultimately develop the syndrome. Even when positive, chest radiography often underestimates vascular damage and accompanying physiologic derangement. The degree of hypoxia measured by pulse oximetry or by arterial blood gas is usually out of proportion to the findings seen on chest x-ray.<\/p>\n
Rx<\/em><\/strong><\/p>\nEarly administration of broad-spectrum antibiotics<\/p>\n
One randomized, double-blind, placebo-controlled trial showed that IV dexamethasone (0.3 mg\/kg q12h x 4 doses) had a beneficial effect in hospitalized children with mild to moderately severe ACS. Mean hospital stay was shorter in the dexamethasone-treated group, and the steroids prevented clinical deterioration and reduced the need for blood transfusions.<\/p>\n
Some patients with ACS may require exchange transfusions.<\/p>\n
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Always consider PE in the differential though it is nearly impossible to distinguish between ACS and PE in the ED.<\/p>\n
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<\/span>Infectious Sequelae<\/span><\/h2>\nImmune defect for encapsulated organisms, as sicklers are often functionally asplenic<\/p>\n
Encapsulated organisms such as Streptococcus<\/em> pneumoniae<\/em> and Escherichia coli, Klebsiella <\/em>sp., and Salmonella <\/em>sp.<\/p>\nAbsolute band count of >3000. Children with high white counts should always be cultured and treated with broad spectrum ABX.<\/p>\n
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<\/span>Bone and Joint Pain<\/span><\/h2>\nDifferential is crisis pain vs. bone infarction vs. osteomyelitis (often secondary to salmonella)<\/p>\n
Infarction and infection usually present with fever and wbc above baseline<\/p>\n
Joint Pain<\/p>\n
Osteonecrosis is a painful and often disabling complication of repeated bony infarctions. By age 35, half of all sickle cell patients have evidence of hip and shoulder osteonecrosis.6 Patients complain of pain and\/or limited range of motion of the affected joint. In the early stages of disease, radiographs will often appear normal,29 although MRI may be diagnostic.6<\/p>\n
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The most important entity to rule out in a sickle cell patient with joint pain is septic arthritis. Suspect septic arthritis when joint pain is accompanied by fever, significant pain on range of motion, or erythema\/edema involving the joint. Joint aspiration is the most vital (and the only definitive test) in such cases.<\/p>\n
<\/span>Dactylitis<\/strong><\/span><\/h2>\nAlso known as hand-foot syndrome, dactylitis may be the earliest manifestation of SCD.48,112 This condition is usually seen in children younger than 6 months of age, but can occur up to 4 years of age.19,112 Physical exam reveals swelling of the hands or feet, pain, and fever. There may also be erythema, mimicking osteomyelitis,113 and the WBC count and sedimentation rate may be elevated. Initial radiographs are normal, but radiographs taken several days after the onset of symptoms may show periosteal elevation. Treatment is supportive, with analgesia, hydration, and warm compresses. Symptoms are usually self-limited.80<\/p>\n
<\/span>Abdominal Pain<\/span><\/h2>\nPotential Causes Of Abdominal Pain In Sickle Cell Anemia<\/p>\n
\nSpleen\n\nSequestration<\/li>\n Hemorrhage<\/li>\n Infarction<\/li>\n Abscess<\/li>\n<\/ul>\n<\/li>\n Hepatobiliary\n\nHepatitis<\/li>\n Cholelithiasis\/cholecystitis<\/li>\n Hepatic sequestration<\/li>\n Intrahepatic cholestasis<\/li>\n<\/ul>\n<\/li>\n Renal\n\nStone<\/li>\n Clot<\/li>\n Papillary necrosis<\/li>\n Cystitis<\/li>\n<\/ul>\n<\/li>\n Bone\n\nInfarction: ribs, spine, femoral head<\/li>\n Osteonecrosis<\/li>\n Vertebral collapse<\/li>\n<\/ul>\n<\/li>\n Miscellaneous\n\nPneumonia<\/li>\n Mesenteric ischemia<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n <\/p>\n
One study retrospectively examined findings in 53 patients with SCA who presented with abdominal pain.30 A vaso-occlusive crisis was responsible for the pain in 57%, while 23% had a surgical entity and 20% had a nonsurgical genitourinary disorder. Overall, 77% of the patients with painful sickle crises\u0097but no <\/em>patient with an acute surgical process\u0097complained of coexistent abdominal and remote (usually extremity) pain. In this series, all patients with a surgical condition complained of localized rather than diffuse pain. Of note, laboratory parameters, including the leukocyte count, <\/em>did not distinguish sickle crisis from a surgical condition. (Am Surg <\/em>1989;55(11))<\/p>\nBiliary tract and parenchymal liver disease are the most prevalent and serious complications that affect the digestive system.6 Five hepatobiliary syndromes that are especially common include viral hepatitis, hepatic crisis, cirrhosis, cholelithiasis with or without cholecystitis, and intrahepatic cholestasis.31 Of these, the most concerning diagnosis is intrahepatic cholestasis. It is characterized by sudden onset of severe right upper quadrant pain, progressive hepatomegaly, coagulopathy, and extreme hyperbilirubinemia.28,31 Treatment is supportive, with transfusion and correction of coagulopathy. The mortality is very high.<\/p>\n
<\/p>\n
<\/span>Genitourinary Complications <\/strong><\/span><\/h2>\nThere are a number of genitourinary complications seen in SCA. The primary renal complications seen in SCD include hematuria, nephrotic syndrome, and, in rare circumstances, renal failure.<\/p>\n
Hematuria is usually self-limited, resolving with bed rest alone. It may occur in those with sickle cell trait, and this diagnosis should be considered in the patient of African descent who presents with painless hematuria.137 Transfusion may be needed if the hematuria is severe.138 Years of glomerular hyperfiltration results in renal damage and the inability to concentrate urine (isothenuria), but chronic renal failure in SCD is uncommon overall.6 A prospective longitudinal study followed a cohort of 725 patients with SCA and found chronic renal failure in only 4.2 %.139<\/p>\n
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<\/span>Priapism<\/span><\/h2>\nOccurs in 10-40% of male Sicklers and can result in permanent damage.<\/p>\n
Urologic consult and .25-.5 mg of SQ terbutaline (not in penis)<\/p>\n
Hydrate, transfuse, and possibly hyperbaric O2.<\/p>\n
Priapism occurs when sickled cells congest the corpora and prevent emptying of blood from the penis. It is a prolonged, usually painful penile erection not initiated by sexual stimuli.140 The priapism that is seen in sickle cell patients is termed a “low flow” or ischemic priapism. Low-flow priapism is a time-sensitive emergency, as irreversible cellular damage and fibrosis can occur if treatment is not administered in 24-48 hours.140 (A full discussion on priapism can be found in the November 2000 issue of Emergency Medicine Practice, <\/em>“Male Genitourinary Emergencies: Preserving Fertility And Providing Relief.”) Treatment of priapism in sickle cell patients includes analgesia, hydration, oxygen, and, occasionally, exchange transfusion. These measures are successful in about 80% of patients.141 If simple interventions fail, corporal aspiration and injection of a vasoconstrictor are occasionally successful in sickle cell patients.141 Urology consultation is indicated.<\/p>\nintracavernosal epinephrine 1:1,000,000<\/p>\n
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<\/span>Ophthalmologic Complications<\/strong><\/span><\/h2>\nThe most serious ocular emergency in sickle cell patients is hyphema in the setting of trauma. The sickled cells tend to obstruct the flow of aqueous humor and may result in elevation of intraocular pressure and subsequent acute angle closure glaucoma. If the intraocular pressure is 24 mmHg or greater in the setting of hyphema, treatment for acute angle closure glaucoma should be initiated. Urgent ophthalmologic consultation should be obtained in all sickle cell patients with hyphema.115<\/p>\n
Give atropine drops but do not give diamox\/acetazolamide as it precipitates sickling<\/p>\n
<\/span> labs<\/span><\/h3>\nsend cbc, lfts, and retic counts in almost all cases<\/p>\n
be gentle with hydration; excess fluids have been linked to atelectasis which can lead to ACS<\/p>\n
Use D5 1\/2 NS per the EM practice article, \nwhich actually makes no sense, b\/c acidosis promotes sickling<\/p>\n
D5LR is probably a better choice<\/p>\n
no supplemental O2<\/p>\n","protected":false},"excerpt":{"rendered":"
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Sickle Cell Anemia - Crashing Patient<\/title>\n \n \n \n\t \n\t \n\t \n