1. Administer fluids aggressively in the management of shock.

2. Diagnose shock based on the criteria of the Centers for Disease Control and Prevention.

3. Recognize various presentations of acute lymphoblastic leukemia.

4. Evaluate and treat associated complications of oncologic emergencies.

5. Apply practical techniques for management of lacerations in the emergency department.

Toxic Shock Syndrome
Reza J. Daugherty, MD, Assistant Professor of Pediatrics, Jefferson Medical College, and Attending Physician, Division of Pediatric Emergency Medicine, Alfred I. duPont Hospital for Children, Wilmington, DE

Case: girl, 3 yr of age, presents to hospital with high-grade fever for 2 days; complains of runny nose, cough, and sore throat; had 1 to 2 episodes of vomiting; “seeing things” when febrile; throat culture performed; given intravenous (IV) fluids, dexamethasone (Decadron), and intramuscular (IM) penicillin; presents to another emergency department (ED) 8 hr later with profuse watery diarrhea, intractable vomiting, and rash (prominent in axilla and groin); acting unusual; history unremarkable; initial physical examination shows fever, tachycardia, and tachypnea (hyperpnea); blood pressure (BP) appears normal but with widened pulse pressure; patient crying, irritable, and inconsolable; oropharynx injected but no exudate; mucous membranes dry; no evidence of meningismus and no photophobia; lungs clear; bounding pulses and capillary refill 3 to 4 sec; abdominal examination unremarkable; neurologic examination nonfocal; skin shows diffuse erythroderma with macular rash accentuated in groin and axilla; patient in early compensated shock (BP normal, increased heart rate, altered perfusion, and altered mental status); laboratory tests—white blood cell (WBC) count 7100/ µL, with shift (42% neutrophils, 43% bands, and immature cells); platelet count slightly decreased (120,000/µL); serum urea nitrogen (BUN) and creatinine elevated; bicarbonate 16 mEq/L; elevated transaminases; urine relatively concentrated but unremarkable; patient placed on continuous heart monitor; given IV access and fluid resuscitation; fever and heart rate decreasing; pulse pressure widening, with systolic pressure decreasing slightly

Circulation: clinically useful definition of shock—hypo- or hyperthermia, altered mental status, and peripheral perfusion abnormality (vasodilation [warm shock] or vasoconstriction [cold shock]); in children, abnormal BP not necessary for shock; vasodilation causes relative hypovolemia, leading to reduced organ perfusion pressure surpassing autoregulation critical point, resulting in organ dysfunction; goal to maintain perfusion pressure above critical point and maintain normal organ function; Society for Critical Care Medicine recommendation 20 mL/kg of normal saline within first 5 to 10 min; must be aggressive with fluids (≤60 mL/kg); monitor capillary refill (<2 sec); goal normal mental status and urine output; patient in shock should have Foley catheter to measure urine output; consider pressors and central access if patient in fluid-refractory shock; dopamine—commonly used in children; relatively safe; first-line agent; causes vasoconstriction by releasing norepinephrine; not as effective in infant with inadequate norepinephrine stores and in chronically ill catecholamine-depleted patient; norepinephrine and epinephrine used for dopamine-resistant fluid-refractory shock (norepinephrine for warm shock and epinephrine for cold shock); within first 5 min, should establish access to start providing fluids; within 15 min, should be 60 mL/kg (5-min boluses hand-pushed)

Toxic shock syndrome (TSS): should know causative organism; case patient had TSS due to Staphylococcus aureus pneumonia (met criteria); Centers for Disease Control and Prevention (CDC) major diagnostic criteria—fever ≥39°C; hypotension; erythroderma with late desquamation; ≥3 of minor criteria, which include oral inflammation or conjunctivitis, vomiting or diarrhea, myalgia or elevated creatine phosphokinase, acute respiratory distress syndrome, altered mental status, elevated transaminases, elevated BUN and creatinine, and thrombocytopenia; not necessary for all signs and symptoms to present at once; TSS toxin (TSST)-1—predominant toxin produced by staphylococci; superantigen; bacterial DNA cosegregates with staphylococcal enterotoxin (causing profuse watery diarrhea); streptococci also produce superantigens (pyrogenic exotoxin A [PEA] predominant); source possibly pneumonia, osteomyelitis, cellulitis, or never identified; superantigens— bypass major histocompatibility complex (MHC) class II molecules; 1 in 5 T cells activated (overwhelming systemic inflammatory response); Dr. Eagle—observed that penicillin ineffective in severe Streptococcus pyogenes infection; theorized that bacteria entering stationary phase and using protein synthesis inhibitor would result in more effective bacteriostatic or bactericidal activity; penicillin and cephalosporins require active cell turnover to interject themselves into cell wall; recommendation clindamycin; disease course highly unpredictable; speaker believes child with TSS should be admitted to hospital; children with toxin-mediated syndrome (not all criteria for TSS met) should be watched for 24 hr; case patient discharged on day 13 with some fine motor and cognitive delays

Oncologic Emergencies
John M. Howard, DO, Senior Fellow, Pediatric Emergency Medicine, Alfred I. duPont Hospital for Children, Wilmington

Case: boy, 3.5 yr of age, has chief complaint of limp associated with unobserved fall 10 days earlier; left-sided knee and hip pain; boy appeared ill and pale; diffuse petechiae noted over extremities and trunk; laboratory tests showed severe anemia (hemoglobin 6 g/dL), elevated WBC count, and thrombocytopenia; differential count showed blast count of 72%; C-reactive protein and lactate dehydrogenase (LDH) elevated; electrolytes, blood glucose, and renal function within normal limits (WNL); slight elevation in one of liver function tests (LFTs); extremity x-rays WNL

Cancer: acute leukemias—most common childhood malignancy (one-third of all cancers); acute lymphoblastic leukemia (ALL) more prevalent than acute myelogenous leukemia (AML) by 5:1 ratio; occurs more often in boys than in girls and in white children more than black children; risk factors include immunodeficiency syndromes, environmental factors (eg, ionizing radiation), advanced maternal age, drugs, and alkylating agents; poor prognosis—in child <1 or >10 yr of age; WBC count of >100,000/µL at diagnosis; lack of response to initial chemotherapy; specific chromosomal abnormalities; good prognosis—rapid response; hyperdiploidy on genetic analysis; certain translocations

Acute lymphoblastic leukemia (ALL): presentation sometimes vague and nonspecific; most common presenting signs and symptoms include hepatosplenomegaly (60%), fever (60%), enlarged nodes (50%), bleeding petechiae (40%), and bone pain (25%); laboratory testing—all patients anemic, although severity varies; majority of children present with WBC count <10,000/µL (≈8% have WBC count >100,000/µL); majority present with platelet count of 20,000/µL to 100,000µL; transfusion indicated if platelet count <20,000/µL (if more conservative, <50,000/µL); some present with mediastinal mass and central nervous system (CNS) findings

Presenting symptoms: musculoskeletal pain—long bone pain from leukemic involvement of periosteum (seen in one- third of cases); osteonecrosis of bone marrow; radiographic changes in 50%; possibly confused with rheumatologic process; headache—due to leukemic invasion of CNS; uncommon (<5%); may also present with vomiting, lethargy, papilledema, and nuchal rigidity; rarely, cranial nerve palsies; lymphadenopathy—present in ≈50%; enlarged if >10 mm, except epitrochlear (threshold ≈5 mm) and inguinal nodes (≈15 mm); concerning lymph nodes nontender, firm, rubbery, matted, and unresponsive to antibiotics; testicular enlargement—rare; usually painless and unilateral; evaluated with ultrasonography (US) and possible biopsy; mediastinal mass—causes problems due to direct airway compression, creation of pleural effusions, and superior vena cava syndrome; presenting symptoms varied, depending on compartment in which tumor located

Evaluation for leukemia: CBC with manual differentiation; electrolytes; renal function and uric acid; coagulation panel; LDH; blood group and type; antibody screen; LFTs; chest x-ray (CXR) to assess for mediastinal mass; blood culture if patient febrile; definitive diagnosis from peripheral smear evaluation; >25% lymphoblasts helps establish diagnosis; lumbar puncture (LP) to determine whether leukemic infiltrate spread to cerebrospinal fluid; indications for bone marrow biopsy—atypical cells in peripheral blood associated with unexplained depression in >1 blood elements; cytopenia with associated lymphadenopathy or hepatomegaly; in ED—obtain thorough history, involve hematologist or oncologist immediately, and discuss issue with family; helpful to have social worker or child-life specialist on hand

ED care: general supportive care—IV access; fluids recommended include 5% dextrose in water (D5W) with 0.25 normal saline (NS) with NaHCO3 instead of KCl; avoid KCl in initial phase, due to concerns with tumor lysis syndrome (TLS); IV fluids run at 1.5 to 2 times maintenance; allopurinol in children >6 yr of age (to stem effects of TLS); broad- spectrum antibiotics if patient febrile or concern with sepsis present; transfusion if patient significantly symptomatic from anemia, thrombocytopenia, or hyperleukocytosis; corticosteroids not indicated (may affect bone marrow studies)

Treatment: 3-step process; induction—goal achieve remission; accomplished using current treatment protocols in 90% of patients; next phases consolidation and maintenance; patients seen in ED during these phases; process requires 2 to 3 yr; one study showed 2.9% mortality during treatment phase (over 10-yr period); several emergencies associated with ALL; if patient anemic or actively bleeding, replace with packed red blood cells (RBCs) ≈10 mL/kg over 3 to 4 hr; transfuse platelets if low; in patient on broad-spectrum antibiotic that destroys gut flora, consider replacing vitamin K

Tumor lysis syndrome: due to destruction of tumor with chemotherapy or spontaneous lysis; rupture of cells causes release of potassium, phosphates, uric acid, and calcium; unbound calcium and uric acid form crystals and cause anuria or oliguria in kidneys (renal insufficiency, even renal failure may occur); treatment—IV fluids (1.5 to 2 times maintenance) used to increase excretion of crystals; allopurinol to reduce uric acid; goal of alkalinizing urine to inhibit urate crystal formation

Superior vena cava syndrome: patient presents with dyspnea, face flushing, facial edema, conjunctivitis, jugular venous distention, anxiety, and distress; initial approach includes obtaining CXR (if inconclusive, consider computed tomography [CT] of chest); patients often in significant respiratory distress and may not tolerate sedation for CT; treat urgently with corticosteroids (eg, methylprednisolone); possible trial of radiation therapy, although leukemias respond better to chemotherapy; complete cardiovascular collapse

Fever and neutropenia: ongoing problem once patient starts therapy; high risk for fungal, bacterial, and viral infections; neutropenia—defined as absolute neutrophil count (ANC) <1500/µL; for patients with leukemia, neutropenia defined as ANC <500/µL (or <1000/µL if in stage of disease where neutrophils expected to decrease); chemotherapy causes mucosal breakdown (mouth to anus) and altered humoral and cellular immunity; patients may have indwelling catheters; fever—defined as temperature ≥38.4°C; oral temperatures preferred; avoid rectal temperatures in younger children; fever may be absent in some patients who instead have hypothermia, hypotension, listlessness, and altered mental status; rate of documented infection 10% to 40%; most common infection bacteremia; girls more prone to urinary tract infections; in study of 72 participants with 115 episodes of fever and neutropenia, only 21% had positive blood cultures; also consider fungi and viruses; diagnosis—blood cultures; whether from central or peripheral line, treatment same; prudent to repeat blood cultures; consider true infection if >2 positive blood cultures; take samples from every lumen (discordant results from cultures drawn from different lumens occurred in approximately one-third of 41 episodes, and estimated sensitivity of culture drawn from single lumen, ≈84%); urine culture; CXR in patient with respiratory symptoms; LP in patient with altered mental status or meningismus; stool studies in patient with diarrhea; Gram stain in patient with skin lesion

Treatment: monotherapy—broad-spectrum agent; if episode uncomplicated and patient not toxic in appearance; standard therapy includes fourth-generation cephalosporin (eg, cefepime) or extended-spectrum agents (eg, imipenem, meropenem); combination therapy—aminoglycoside with antipseudomonal agent or agents used in monotherapy; synergistic effect desired; monitor for nephrotoxicity with aminoglycosides; indications for vancomycin—added to regimen if patient has hypotension or cardiovascular compromise, suspected catheter-related infection, colonization with methicillin-resistant S aureus (MRSA), cephalosporin-resistant infection, positive blood culture for gram-positive cocci before final identification of organism, or substantial mucositis; change regimen if—clinical status worsening; vital signs unstable; fever persistent; identification of organism from blood culture; evidence of localized infection; antifungal coverage—in patients who remain febrile and neutropenic after receiving broad-spectrum antibiotic coverage, consider clinically occult fungal infections or fungal superinfections; treatment of choice amphotericin B; duration of treatment—discontinue antibiotics after 48 hr if no identified source of fever, patient appears well, afebrile for 24 hr, and shows evidence of bone marrow recovery; role of granulocyte colony-stimulating factor (G- CSF)—study showed no significant benefit in children with high-risk ALL; no difference with episodes of febrile neutropenia, positive blood cultures, serious infection, rate of hospitalization, induction therapy completion time, 6-yr event-free survival, and overall survival

Relapse: symptoms usually similar to those of initial presentation; most commonly occurs in bone marrow; consider if patient has persistent cytopenias; refer promptly for bone marrow evaluation; second site for relapse CNS (better since introduction of intrathecal chemotherapy); boys more prone to testicular relapse (uncommon)

Tricks of the Trade
Michelle Lin, MD, Assistant Clinical Professor, University of California, San Francisco, School of Medicine, and Associate Program Director, UCSF-San Francisco General Hospital Emergency Medicine Residency

Measuring length of wound: accurate length of wound required for good documentation and for billing; speaker’s technique to measure length of left hand and use hand as guide; if wound >8 cm, wide range of errors present; using stethoscope as ruler, can also put tiny marks at 1-cm intervals on earpiece

Minimizing missing second laceration: always look for things in multiples; in scalp—good lighting key to avoid missing laceration; disposable penlights becoming obsolete with development of light-emitting diode (LED) light; methodical manual palpation of scalp better technique; closure of scalp wound traditionally performed with staples; speaker uses double- gun technique; hair apposition technique—indicated for patients unreliable for staple or suture removal; contraindications include lacerations >10 cm, grossly contaminated wounds, active bleeding from laceration, active wound tension, and hair strands <3 cm