Pediatric emergency medicine trisk 554

baseline Elevated reticulocyte count Elevated bilirubin Aplastic anemia Fatigue Pallor crisis Tachycardia Absence of jaundice Altered mental status (severe anemia) Headache Dyspnea Fall in the hematocrit from baseline Low reticulocyte count 10 Absence of acute hemolysis Bacteremia/sepsis Fever Tachycardia Hypotension Lethargy Usually self-limited, Small routine supportive care transfusions Transfusion of pRBCs often sufficient if severe symptoms to support (altered mental status, oxygen impaired oxygen delivery while delivery, significant awaiting bone tachycardia) marrow recovery Often parvo B19 related, though other infections as well Cultures of blood, and High risk for if indicated, urine and infection with spinal fluid Streptococcus Prompt antibiotic pneumoniae, administration (don’t Haemophilus delay if culture difficult influenzae, to obtain) ThirdSalmonella, generation Staphylococcus cephalosporin, consider aureus, addition of Escherichia vancomycin If allergy coli to cephalosporins, Can rapidly consider deteriorate vancomycin/gentamicin in hospitalizated patients; for wellappearing allergic patients, consider quinolones (e.g., levofloxacin), or clindamycin IV fluids Simple or exchange transfusion for sepsis, acidosis, or hypoxia Admission (consideration of outpatient management in stable patients with isolated fever) Management/Diagnostic Testing If the patient describes pain typical in location and quality to a vasoocclusive crisis, begin fluids, provide analgesia, and observe the patient for improvement Elevated aspartate aminotransferase (AST) and hyperbilirubinemia may be present in infarction as well as biliary disease Abdominal ultrasound can assess the anatomy of the liver, gall bladder, and biliary tree Cholelithiasis is the most common hepatic and biliary tract complication in children with sickle cell disease, with an incidence of 12% in 2- to 5-year olds and approximately 40% by the age of 15 to 18 years Patients can present with acute right upper quadrant pain and tenderness, hyperbilirubinemia, and elevated liver enzyme levels Typically, surgery is delayed until acute inflammation has subsided to reduce the risk of complications The optimal treatment includes elective laparoscopic cholecystectomy after adequate preparation for surgery (e.g., transfusions) Rarely, acute intrahepatic sickling or viral hepatitis can result in a similar clinical presentation with massive hyperbilirubinemia and elevated enzyme levels Fulminant hepatic failure with hepatic encephalopathy and shock can also occur as a rare, often fatal, syndrome that may be amenable to exchange transfusion Priapism Initial Assessment Priapism is a painful vasoocclusive crisis causing a tender and engorged penis that may persist for hours Urination may be difficult Management/Diagnostic Testing Treatment is similar to other painful crises with management directed at hydration and analgesia A number of systemic therapies can also be utilized Pseudoephedrine can be given orally, while terbutaline delivery is either oral or subcutaneous Terbutaline has been studied for the treatment of priapism in nonsickle cell disease patients and has some demonstrated efficacy in the sickle cell disease group Consider early consultation with a pediatric hematologist and urologist Infection/Sepsis Initial Assessment Sickle cell patients have a higher risk of bacterial infection than the general population due to functional asplenia, low serum immunoglobulin levels, and abnormal opsonization and complement activation The risk is highest for serious infections with encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and Salmonella species, as well as S aureus and E coli Sepsis, meningitis, pneumonia, osteomyelitis, abscesses, and septic arthritis are all possible The use of oral penicillin prophylaxis until age and vaccination for H influenzae and S pneumoniae have decreased the rate of serious bacterial infection; however, infection remains a meaningful concern in the setting of an acutely ill, febrile patient with sickle cell anemia In one large retrospective study at a tertiary care center, the bacteremia rate in 1,118 acutely febrile patients with sickle cell was 0.8%; the nine cases of bacteremia included four cases of Salmonella, two S pneumoniae, two E coli, and one S aureus Management/Diagnostic Testing In the emergency department, a toxic-appearing febrile child with sickle cell disease requires careful and immediate evaluation, monitoring, fluid resuscitation, and rapid administration of antibiotics A pathway for the management of sickle cell disease with fever is available at https://www.chop.edu/clinical-pathway/sickle-cell-disease-withfever-clinical-pathway Obtain cultures of blood, and, if indicated, urine and spinal fluid Include a type and cross Do not delay antibiotic administration due to difficulty obtaining labs or performing procedures In regions with endemic resistant S pneumoniae strains, use vancomycin in addition to a third-generation cephalosporin Consider broader-spectrum antibiotics as indicated based on concern for abdominal process or for severe presentations As in other patients with reduced or absent splenic function, clinical deterioration may be extremely rapid The patient who arrives alert to the ED may become moribund and hypotensive within 30 minutes In the ill patient with sepsis, acidosis, or hypoxia, provide simple or exchange transfusions to decrease massive sickling, which can cause secondary organ damage Mortality from bacteremia may approach 20% to 30% Have a high level of suspicion for meningitis in the young, irritable child with sickle cell disease and unexplained fever Perform a lumbar puncture on toxic children and anyone with signs or symptoms of meningitis Use the same antibiotic therapy for meningitis as recommended for children with meningitis with hematologic disease Simple or exchange transfusion to lower the percentage of sickle hemoglobin may reduce the risk of intracerebral sickling in areas of brain swelling that can lead to infarction When hemoglobin S is less than 30% of the total hemoglobin, sickling is unlikely, and decisions regarding fluid management can be directed by the central nervous system findings as opposed to the need to ameliorate sickling The nontoxic, febrile child (temperature >38.5°C) with sickle cell disease requires a thorough history and physical examination Detailed history should focus on length, duration, and height of fever, and the presence of associated symptoms such as cough, respiratory distress, and abdominal pain Elicit immunization status and compliance with prophylactic antibiotics Without a clear source of infection, laboratory assessment should include a CBC with a reticulocyte count, blood culture, and a type and screen Other evaluations may include a chest x-ray, urinalysis with culture, or cerebrospinal fluid analysis as clinically indicated A type and cross should be sent if history suggests splenic sequestration, acute chest syndrome, or significant neurologic symptoms Administer antibiotics promptly, prior to knowledge of laboratory results Typically, administer ceftriaxone (50 mg/kg up to 2,000 mg) for broad-spectrum antibiotic coverage lasting 24 hours while cultures are pending; outpatient management is appropriate in stable patients in whom there are no early concerns for developing complications and who have reliable caretakers with the means to return if necessary Acute Chest Syndrome Initial Assessment Acute chest syndrome is a life-threatening complication of sickle cell disease indicative of pneumonia, pulmonary infarction, or both Lifetime risk of acute chest syndrome is 48% and causes 25% of sickle cell disease–related deaths The patient usually presents with fever, tachypnea, chest pain, rales, and hypoxia In a dehydrated patient, the initial physical examination findings may be minimal Management/Diagnostic Testing Any sickle cell patient who presents with chest pain requires immediate assessment, monitoring, and treatment including IV access, fluids, oxygen administration, and laboratory testing including CBC with reticulocyte count, type and screen, blood culture, and chest x-ray (see Table 93.6 ) Any acute infiltrate should be concerning for acute chest syndrome and the patient should be treated with antibiotics including a thirdgeneration cephalosporin such as ceftriaxone and atypical coverage with a macrolide Add vancomycin in the severely ill patient All patients with suspected acute chest syndrome require admission for further management Closely monitor patients with chest pain without radiographic findings or hypoxia and encourage incentive spirometry, as splinting puts them at risk for the development of acute chest syndrome which may not be present on presentation Consider simple transfusion in the presence of severe anemia (hemoglobin