PANDAS

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Strep infectionsWe have previously addressed the notion that “Not All That Shakes is a Seizure.” Often, just by witnessing the “shaking” it is possible to lower the concern for seizures; however, refrain from being dismissive, as there are a number of other conditions that may lead to unusual shaking, twitching, and tic-ing.  Chorea, for example, can be quite alarming to see.  Occasionally, someone may also mention a condition that causes me to stare blankly. One of these befuddling conditions is even associated with Strep Infection… perfect… kids love to collect strep!  Let’s take a moment to review PANDAS (no, not the large furry mammal that eats bamboo) so that we may appear less befuddled.

 

PANDAS: Background

  • PANDAS = Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections
  • Relatively new condition that was first described in 1998. [Swedo, 1998]
  • Association with Strep Infections is still debated, but condition recognized a homogenous group of children. [Williams, 2014]
    • Some chose to view PANDAS as a subset of PANS (Pediatric Acute-onset Neuropsychiatric Syndrome).
    • Essentially, the understanding of the condition is still in its infancy.
  • Etiology:
    • Exact pathogenesis of PANDAS is not yet clarified.
    • Possibly related to molecular mimicry between GAS and CNS epitopes leading to post-infectious autoimmunity in susceptible kids. [Esposito, 2014]

 

PANDAS: Diagnostic Criteria

  • MUST HAVE[Swedo, 2015; Swedo, 1998]
    1. Presence of OCD and/or Tic Disorder
      • Obsessional fears of contamination or harm to self/others
      • Compulsions involving washing, checking, repeating, and counting
      • Tic disorder – involuntary motor tics (ex, nose twitching, eye blinking, head jerking, vocal tics, coughing, throat clearing)
      • Often also have neuropsychiatric symptoms:
        • Ex, anxiety, emotional lability, ADHD, Oppositional-defiant disorder, behavior regressions
        • Again, these develop acutely and may be what catches family’s attention.
    2. Prepubertal onset
      • Mean age = 6 years.
      • Also distinguishes it from typical OCD, which has later onset.
    3. Abrupt onset or Exacerbation of symptoms with an episodic course
      • Key feature is the acute and rapid onset of symptoms!
      • Typically, patient was healthy previously.
    4. Associated neurologic abnormalities
      • Choreiform movements
      • Urinary urgency/ frequency due to enuresis
      • Sleep disorders (ex, parasomnias)
    5. Temporal association between symptoms and Group A Strep infection
      • No defined time window, but typically within 6 weeks
      • Debated feature, as GAS infection is common in children
      • GAS leading to pharyngitis or skin infections (ex, perianal) have been implicated. [Toufexis, 2014]

 

PANDAS: Treatment

  • Primary treatment is symptomatic.  [Esposito, 2014]
    • Cognitive behavioral therapy
      • Methods and tools to recognize triggers for tics and means to counteract them.
      • Stress is a common exacerbating cause.
    • Family counseling
    • Psychoeducation
  • Antibiotics (Penicillin)
    • Mixed results about utility of antibiotic therapy.  [Esposito, 2014]
    • Some evidence that Penicillin is associated with improvement in tics.
    • If the condition is related to autoimmune disorder, antibiotics being effective seems odd.
  • Dopamine Antagonists
    • Haloperidol and risperidone – used in severe cases of OCD.
    • Tiapride (not available in the USA) – useful for motor tics.
  • SSRIs may also be used for severe OCD.
  • Immune-modulatory Therapy
    • Plasma Apheresis or IVIG have been used, but should be reserved for severe cases.
    • Plasma Apheresis may even be helpful after the acute presentation. [Latimer, 2015]

 

References

Swedo SE1, Seidlitz J, Kovacevic M, Latimer ME, Hommer R, Lougee L, Grant P. Clinical presentation of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections in research and community settings. J Child Adolesc Psychopharmacol. 2015 Feb;25(1):26-30. PMID: 25695941. [PubMed] [Read by QxMD]

Williams KA1, Swedo SE2. Post-infectious autoimmune disorders: Sydenham’s chorea, PANDAS and beyond. Brain Res. 2015 Aug 18;1617:144-54. PMID: 25301689. [PubMed] [Read by QxMD]

Latimer ME1, L’Etoile N, Seidlitz J, Swedo SE. Therapeutic plasma apheresis as a treatment for 35 severely ill children and adolescents with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. J Child Adolesc Psychopharmacol. 2015 Feb;25(1):70-5. PMID: 25658452. [PubMed] [Read by QxMD]

Toufexis M, Deoleo C, Elia J, Murphy TK. A link between perianal strep and pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection (PANDAS). J Neuropsychiatry Clin Neurosci. 2014 Apr 1;26(2):164-8. PMID: 24763762. [PubMed] [Read by QxMD]

Stagi S1, Rigante D2, Lepri G3, Bertini F3, Matucci-Cerinic M3, Falcini F3. Evaluation of autoimmune phenomena in patients with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Autoimmun Rev. 2014 Dec;13(12):1236-40. PMID: 25151976. [PubMed] [Read by QxMD]

Esposito S1, Bianchini S, Baggi E, Fattizzo M, Rigante D. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: an overview. Eur J Clin Microbiol Infect Dis. 2014 Dec;33(12):2105-9. PMID: 24953744. [PubMed] [Read by QxMD]

Wolf DS1, Singer HS. Pediatric movement disorders: an update. Curr Opin Neurol. 2008 Aug;21(4):491-6. PMID: 18607212. [PubMed] [Read by QxMD]

Swedo SE1, Leonard HL, Garvey M, Mittleman B, Allen AJ, Perlmutter S, Lougee L, Dow S, Zamkoff J, Dubbert BK. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry. 1998 Feb;155(2):264-71. PMID: 9464208. [PubMed] [Read by QxMD]

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UTI Empiric Antibiotics

UTI AntibioticsLast week we discussed making the diagnosis of an urinary tract infection.  Essentially, we reinforced the fact that we can only make a presumptive diagnosis of UTI in the ED.  In some situations, you may prefer to await the urine culture for more definitive answers; however, there will be many occasions when it is deemed appropriate to initiate empiric antibiotics.  When that is the case, what should you choose? Let’s look at some options for Empiric Antibiotics for UTI.

 

UTI: The Bugs

  • Worldwide, over the past several decades, antibiotic resistance has continued to evolve and increase. [Mishra, 2015; Echeverri, 2014; Mirsoleymani, 2014; Edlin, 2013]
  • E. coli is responsible for majority of UTIs (~80%). [Edlin, 2013; Bhat, 2011]
  • Other encountered uropathogens:
    • Klebsiella
    • Proteus
    • Group B Strep – consider in neonates and young infants.
    • Enterococcus – consider in neonates and young infants.
    • Staph saprophyticus
    • Pseudomonas
    • Fungi – seen in immunocompromised or diabetic patients or those with indwelling catheters or on long-term antbiotics.
  • Common contaminants:
    • Cornebacterium species
    • Coag-negative staph
    • Lactobacillus species
    • Alpha-hemolytic strep

 

UTI: Management

  • Local antimicrobial susceptibility patterns are the most important tool to help determine initial therapy. [Edlin, 2013; Jerardi, 2012; Subcommittee AAP, 2011]
  • Duration of therapy:
    • Short course (5 days) is as effective as 7-14 days for uncomplicated cystitis in children
    • Consider short courses for older children.
  • PO vs IV:
    • PO and IV therapies are equally efficacious. [Kowalsky, 2013; Subcommittee AAP, 2011]
    • Children with severe disease (ex, pyelonephritis) or medical complications (ex, immunocompromised) need to be treated with IV therapy until afebrile for 24 hours. Can be converted to oral afterwards.
    • Neonates require full sepsis evaluation and IV antibiotics!
    • >2 month olds:
      • Well appearing >2 month olds have low risk of UTI and concomitant meningitis[Tebruegge, 2011]
      • If considering outpatient management:
        • Kid has to look awesome!
        • Kid has to have excellent access to care.
        • Discuss with Primary Care Provider to ensure that everyone is on the same management page!

 

UTI: The Bugs

  • NO initial empiric choice is perfect.
    • Send the Culture!
    • Ensure follow-up!
  • Cephalosporins [Subcommittee AAP, 2011]
    • Have become a commonly selected 1st choice for UTI. [Bhat, 2011]
    • Some evidence that Nitrite-Negative U/A results may indicate increased risk for resistance to 1st and 2nd generation (Enterocococcus do not convert Nitrate to Nitrite). [Weisz, 2010]
    • Cefaclor
      • 50-100 mg/kg/Day divided in 3 doses
    • Cefixime
      • 8 mg/kg once a day
    • Cephalexin
      • Useful first generation cephalosporin.
      • Will NOT cover enterococcus.
      • 50-100 mg/kg/D divided in four doses.
    • Ceftriaxone
      • IM dosing can be used in those who won’t tolerate oral meds.
      • 50-100 mg/kg every 24 hours.
  • Amoxicillin / Ampicillin [Subcommittee AAP, 2011]
    • A large percentage of coli are resistant to ampicillin. [Gaspari, 2006]
    • Does attain high levels in the urine and is effective against coli. [Betrosian, 2009]
    • Recommendation is to use Amoxicillin with Clavulanate (20-40 mg/kg/D divded TID).
  • Trimehoprim-Sulfamethoxazole (TMP-SMZ) [Subcommittee AAP, 2011]
    • Ever increasing resistance, plus not an innocuous medicine. [Edlin, 2013]
    • Bacteria that are resistant to ampicillin are commonly also resistant to TMP-SMZ. [Gaspari, 2006]
    • The high resistance makes this a poor choice for empiric therapy of UTI. [Gaspari, 2006]
  • Nitrofurantoin
    • Attains good levels in urine, but not serum.
    • Good for nitrite-negative UTIs. [Weisz, 2010]
    • Should not be used to treat pyelonephritis or febrile infants. [Bhat, 2011; Subcommittee AAP, 2011]
  • Ciprofloxacin
    • Often avoided due to concern for cartilage injury, but this is not supported in the literature.
    • Reserved for resistant organisms. [Bhat, 2011]
    • Can use if need in special cases (ie, cystic fibrosis).

 

The Moral of the Morsel

  • Know your local antibiotic resistance patterns.
  • Avoid using TMP-SMZ empirically for UTI!
  • Ensure the Urine Culture gets sent (it is crucial for the diagnosis and for tailoring therapy).
  • Not every child needs empiric therapy for an abnormal urinalysis. Discuss with the Primary Care Physician when not clearly requiring empiric antibiotics.

 

References

Becknell B1, Schober M, Korbel L, Spencer JD. The diagnosis, evaluation and treatment of acute and recurrent pediatric urinary tract infections. Expert Rev Anti Infect Ther. 2015 Jan;13(1):81-90. PMID: 25421102. [PubMed] [Read by QxMD]

Mishra MP1, Sarangi R2, Padhy RN3. Prevalence of multidrug resistant uropathogenic bacteria in pediatric patients of a tertiary care hospital in eastern India. J Infect Public Health. 2015 Nov 23. PMID: 26617250. [PubMed] [Read by QxMD]

Dev Period Med. 2014 Oct-Dec;18(4):470-6. PMID: 25874786. [PubMed] [Read by QxMD]

Mirsoleymani SR1, Salimi M2, Shareghi Brojeni M3, Ranjbar M3, Mehtarpoor M4. Bacterial pathogens and antimicrobial resistance patterns in pediatric urinary tract infections: a four-year surveillance study (2009-2012). Int J Pediatr. 2014;2014:126142. PMID: 24959183. [PubMed] [Read by QxMD]

Vélez Echeverri C1, Serna-Higuita LM1, Serrano AK2, Ochoa-García C2, Rojas Rosas L2, María Bedoya A3, Suárez M4, Hincapié C4, Henao A4, Ortiz D4, Vanegas JJ1, Zuleta JJ5, Espinal D4. Resistance profile for pathogens causing urinary tract infection in a pediatric population, and antibiotic treatment response at a university hospital, 2010-2011. Colomb Med (Cali). 2014 Mar 30;45(1):39-44. PMID: 24970958. [PubMed] [Read by QxMD]
Mikrobiyol Bul. 2013 Oct;47(4):603-18. PMID: 24237429. [PubMed] [Read by QxMD]

Edlin RS1, Shapiro DJ, Hersh AL, Copp HL. Antibiotic resistance patterns of outpatient pediatric urinary tract infections. J Urol. 2013 Jul;190(1):222-7. PMID: 23369720. [PubMed] [Read by QxMD]

Kowalsky RH1, Shah NB. Update on urinary tract infections in the emergency department. Curr Opin Pediatr. 2013 Jun;25(3):317-22. PMID: 23652682. [PubMed] [Read by QxMD]

Jerardi KE1, Auger KA, Shah SS, Hall M, Hain PD, Myers AL, Williams DJ, Tieder JS. Discordant antibiotic therapy and length of stay in children hospitalized for urinary tract infection. J Hosp Med. 2012 Oct;7(8):622-7. PMID: 22833498. [PubMed] [Read by QxMD]

Tebruegge M1, Pantazidou A, Clifford V, Gonis G, Ritz N, Connell T, Curtis N. The age-related risk of co-existing meningitis in children with urinary tract infection. PLoS One. 2011;6(11):e26576. PMID: 22096488. [PubMed] [Read by QxMD]

Bhat RG1, Katy TA, Place FC. Pediatric urinary tract infections. Emerg Med Clin North Am. 2011 Aug;29(3):637-53. PMID: 21782079. [PubMed] [Read by QxMD]

Subcommittee on Urinary Tract Infection, Steering Committee on Quality Improvement and Management, Roberts KB. Urinary tract infection: clinical practice guideline for the diagnosis and management of the initial UTI in febrile infants and children 2 to 24 months. Pediatrics. 2011 Sep;128(3):595-610. PMID: 21873693. [PubMed] [Read by QxMD]

Betrosian AP1, Douzinas EE. Ampicillin-sulbactam: an update on the use of parenteral and oral forms in bacterial infections. Expert Opin Drug Metab Toxicol. 2009 Sep;5(9):1099-112. PMID: 19621991. [PubMed] [Read by QxMD]

Arredondo-García JL1, Soriano-Becerril D2, Solórzano-Santos F3, Arbo-Sosa A4, Coria-Jiménez R1, Arzate-Barbosa P1. Resistance of uropathogenic bacteria to first-line antibiotics in mexico city: A multicenter susceptibility analysis. Curr Ther Res Clin Exp. 2007 Mar;68(2):120-6. PMID: 24678125. [PubMed] [Read by QxMD]

Gaspari RJ1, Dickson E, Karlowsky J, Doern G. Multidrug resistance in pediatric urinary tract infections. Microb Drug Resist. 2006 Summer;12(2):126-9. PMID: 16922629. [PubMed] [Read by QxMD]

Bonsu BK1, Shuler L, Sawicki L, Dorst P, Cohen DM. Susceptibility of recent bacterial isolates to cefdinir and selected antibiotics among children with urinary tract infections. Acad Emerg Med. 2006 Jan;13(1):76-81. PMID: 16365328. [PubMed] [Read by QxMD]

Marcus N1, Ashkenazi S, Yaari A, Samra Z, Livni G. Non-Escherichia coli versus Escherichia coli community-acquired urinary tract infections in children hospitalized in a tertiary center: relative frequency, risk factors, antimicrobial resistance and outcome. Pediatr Infect Dis J. 2005 Jul;24(7):581-5. PMID: 15998996. [PubMed] [Read by QxMD]

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Urinary Tract Infection (UTI)

UTIOften, it seems as if the quest for “an answer” can cause us to see what is not there.  This is particularly problematic with respect to the quest to decipher the cause of fever.  Appropriately, we may consider the potential for a Urinary Tract Infection (UTI) as the source of the fever.  We have previously discussed how the duration of fever can influence the likelihood of finding a UTI as well as how finding a UTI can diminish the likelihood of there being meningitis.  The question, though is whether we are making the correct diagnosis. Let us take a minute to review the Diagnosis of UTI.

 

UTI: Basics

  • UTI is the 2nd most common bacterial infection, following AOM.
  • UTI is the most common serious bacterial infection in febrile infants/children.
    • 10% of all febrile children
    • 7% of febrile new-borns with high rates of concurrent bacteremia
    • Uncircumcised male infants < 3 months of age and females < 12 months have the highest prevalence of UTI
  • UTI can become a big problem in children!
    • Pyelonephritis
    • Chronic UTIs
    • Renal scarring (still being debated) [Becknell, 2015]
    • Chronic renal insufficiency
    • Hypertension
    • Urosepsis

 

UTI: Presentation

  • Unfortunately, the classic presentation (dysuria, frequency, and hesitancy) is not reliably found in children.
  • Children often present with non-specific symptoms (just because they like to be difficult).
    • Infants may have vomiting, unexplained fever, failure to thrive, irritability, lethargy or jaundice.
    • Older children who can communicate better may complain of dysuria, but may also report non-specific abdominal pain and/or vomiting.
    • Older children may also present with hematuria or even incontinence / enuresis.

 

UTI: Making the Diagnosis

  • As with any diagnosis, you first need to consider the probability of it existing in an individual patient.
  • Weighing Risk Factors can be helpful in doing this. [Roberts, 2011]
    • Girls:
      • Characteristics:
        • White race
        • Age < 12 months
        • Fever for 2 or more days
        • Temperature of 39 C (102.2F) or higher
        • Absence of other source of infection
      • If 0 or 1 characteristic – probability of UTI is </= 1%
      • If 2 characteristics – probability of UTI is </= 2%
    • Boys:
      • Characteristics:
        • Non-Black race
        • Fever for 24 hours or greater
        • Temperature of 39C (102.2F) or higher
      • If Uncircumcised, probability of UTI is > 1%.
      • If Circumcised and 0-2 characteristics – probability of UTI is </= 1%
      • If Circumcised and 3 characteristics – probability of UTI is </= 2%
  • AAP Practice Guideline states that a diagnosis of UTI requires BOTH: [Roberts, 2011]
    • Pyuria and/or Bacteriuria on urinalysis AND
    • >50,000 colony forming units/mL of a single uropathogen on urine culture.
      • Bagged urine samples CANNOT be used to make the diagnosis! [Roberts, 2011]
      • This means that without culture results, we can, at best, only make a presumptive diagnosis of UTI.
      • Additionally, this also means that a Urine Culture result with >50,000 CFUs/mL does not necessarily equate to a UTI either. If no pyuria, it is asymptomatic bacteriuria.
  • Other test considerations:
    • Point of Care Urinalysis may have more rapid turn-around times, but it has lower sensitivities compared to a laboratory performed U/A. [Kazi, 2013]
    • Urinalysis is NOT a substitute for urine culture.
      • Nitrites:
        • Low sensitivity
          • Not all pathogens convert nitrate to nitrite. (False negative)
          • It takes at least 4 hours for conversion to happen. (False negative)
        • High specificity, though, as there are few False positives.
      • Leukocyte Esterase:
        • Some have found it to be very sensitive. [Schroeder, 2015]
        • Has 20% False negative rate. (High urine flow rate can prevent LE from accumulating).
        • Has numerous causes of False positives (ex, Strep infection, Kawasaki’s Disease).
    • Urine Microscopy
      • Pyuria alone does not constitute UTI.
      • Normal Urine WBC count < 5 /hpf
      • Presence of bacteria in unspun sample correlates with 10×5 CFUs/mL on culture.

 

Moral of the Morsel

  • Be mindful not to satisfy your desire to have an “answer” by equating LE+ on U/A with a UTI.
  • Acknowledge the fact that we can only make a presumptive diagnosis of UTI without the culture result.
    • If the patient is at higher risk for UTI:
      • It may be appropriate to treat with antibiotics empirically.
      • Discuss with the family the need to have the culture results reviewed to make the definitive diagnosis (and either continue therapy or stop it).
    • If the patient is at lower risk for UTI:
      • Discuss taking a watchful waiting approach. [Newman, 2013]
      • Having the culture results will help avoid unnecessarily and potentially harmful antibiotic exposure.

 

References

Stein R1, Dogan HS2, Hoebeke P3, Kočvara R4, Nijman RJ5, Radmayr C6, Tekgül S2; European Association of Urology; European Society for Pediatric Urology. Urinary tract infections in children: EAU/ESPU guidelines. Eur Urol. 2015 Mar;67(3):546-58. PMID: 25477258. [PubMed] [Read by QxMD]

Becknell B1, Schober M, Korbel L, Spencer JD. The diagnosis, evaluation and treatment of acute and recurrent pediatric urinary tract infections. Expert Rev Anti Infect Ther. 2015 Jan;13(1):81-90. PMID: 25421102. [PubMed] [Read by QxMD]

Schroeder AR1, Chang PW2, Shen MW3, Biondi EA4, Greenhow TL2. Diagnostic accuracy of the urinalysis for urinary tract infection in infants <3 months of age. Pediatrics. 2015 Jun;135(6):965-71. PMID: 26009628. [PubMed] [Read by QxMD]

Newman DH1, Shreves AE, Runde DP. Pediatric urinary tract infection: does the evidence support aggressively pursuing the diagnosis? Ann Emerg Med. 2013 May;61(5):559-65. PMID: 23312370. [PubMed] [Read by QxMD]

Kazi BA1, Buffone GJ, Revell PA, Chandramohan L, Dowlin MD, Cruz AT. Performance characteristics of urinalyses for the diagnosis of pediatric urinary tract infection. Am J Emerg Med. 2013 Sep;31(9):1405-7. PMID: 23891600. [PubMed] [Read by QxMD]

Subcommittee on Urinary Tract Infection, Steering Committee on Quality Improvement and Management, Roberts KB. Urinary tract infection: clinical practice guideline for the diagnosis and management of the initial UTI in febrile infants and children 2 to 24 months. Pediatrics. 2011 Sep;128(3):595-610. PMID: 21873693. [PubMed] [Read by QxMD]

Bhat RG1, Katy TA, Place FC. Pediatric urinary tract infections. Emerg Med Clin North Am. 2011 Aug;29(3):637-53. PMID: 21782079. [PubMed] [Read by QxMD]

The post Urinary Tract Infection (UTI) appeared first on Pediatric EM Morsels.

Hereditary Angioedema

Hereditary AngioedemaWe have previously discussed Chronic Recurrent Abdominal Pain. Certainly, this is not a sensational topic, yet it is one that is encountered often and can not only derail your shift, but may also obscure our view of the true underlying, etiology.  As is always the case, our job is to remain vigilant for the subtle presentation of the serious condition, while remaining reasonable in our approach to evaluation.  Most often, this challenge is accomplished by honing our curiosity and obtaining a thoughtful history and physical exam.  With these “simple” and elegant tools, perhaps we can discover a cause of recurrent abdominal pain, like Hereditary Angioedema!

 

Hereditary Angioedema: Basics

  • Inherited in an Autosomal Dominant fashion!
    • Although can occur de novo.
    • There may be a delay in diagnosis in those without a family history. [Nada, 2015]
  • Due to Protein C1-Inhibitor inactivity or inadequate activity. [MacGinnitie, 2014]
    • C1-Inhibitor is a protease that inhibits (you guessed it) C1.
      • C1 is the 1st component in the classical complement cascade.
      • C1-Inhibitor also inhibits other enzymes (kallikrein, Factors XI and XII, and tissue plasminogen activator).
    • Low Protein C1-Inhibitor activity leads to increased levels of bradykinin (due to increased kallikrein activity).
    • Increased bradykinin leads to increased vascular permeability and, thus, edema.

 

Hereditary Angioedema: Presentation

  • Typically presents in childhood. [Christiansen, 2015]
    • Median age of 1st swelling – 11 years
    • Median age of diagnosis – 19 years
      • Those who develop symptoms earlier seem to have longer delays in diagnosis.
      • Those who develop symptoms earlier seem to have more morbidity.
  • Characterized by recurrent attacks.
    • Attacks resolve slowly, usually over 2-5 days.
    • Non-pruritic swelling.
    • Often painful swelling.
  • Attacks can be triggered stress and trauma.
  • Causes symptoms through swelling (angioedema) of the:
    • Intestinal tract 
      • Maybe the only location involved.
      • Can mimic surgical process (like appendicitis). [Rubinstein, 2014]
      • Can make diagnosis a challenge (and is why we are discussing it now).
      • Can lead to delayed diagnosis.
    • Skin
      • May develop preceding altered sensation/tingling of the skin.
      • May develop erythema marginatum (non-pruritic, flat red eruption with central clearing).
      • Hives are not a part of hereditary angioedema, but can occur concurrently.
    • Upper Airway
      • Obviously, this is the most dangerous.
      • Those who have yet to be correctly diagnosed with hereditary angioedema are at greater risk for asphyxiation. [Bork, 2012].
    • Also can involve other regions:
      • Esophagus
      • Kidneys
      • Brain
      • Pancreas

 

Hereditary Angioedema: Diagnosis

  • History of recurrent attacks and family history are important!
  • C4 levels are low at all times in the majority of patients. [MacGinnitie, 2014]
  • C1-Inhibitor level and activity level can also be obtained.

 

Hereditary Angioedema: Treatment

  • Since the edema is bradykinin mediated, it will not respond to antihistamines, corticosteroids, or epinephrine. [Christiansen, 2015; MacGinnitie, 2014]
  • Often patients and families will come to the ED with their own specialized medications.
    • Look them up to re-familiarize yourself with that child’s medications.
    • Call the child’s allergist / immunologist early in the management course.
  • “Fancy Meds” (things that I always have to look up again)
    • C1-Inhibitor replacement
      • Two versions exist:
        • Purified-plasma C1-Inhibitor (Berinert or Cinryze)
        • Recombinant C1-Inhibitor (Ruconest)
      • Augments C1-Inhibitor levels.
      • Can be self administered at home. [Kreuz, 2012]
      • Can be used for prophylaxis as well as acute therapy. [Schneider, 2012; Martiez-Saguer, 2010]
      • Often used as 1st line therapy in pediatric patients. [Craig, 2015]
    • Kallikren Inhibitor
      • Ecallantide (Kalbitor)
      • Given SubCutaneously!
      • For acute therapy and typically given by health care providers. [MacGinnitie, 2013]
    • Bradykinin Receptor Antagonist
      • Icatibant (Firazyr)
      • Given SubCutaneously!
      • Can be self-administered.
  • Other options
    • Antifibrinolytics
      • Tranexamic Acid (TXA) and Epsilon-Aminocaproic Acid
    • Fresh Frozen Plasma
    • Attenuated androgens

 

Moral of the Morsel

  • Certainly we will encounter many more cases of chronic recurrent abdominal pain that are due to idiopathic or benign entities, but keeping Hereditary Angioedema on our differential will allow us look for the clues that may help positively impact that child’s life.
  • Additionally, it is a good thing to keep in mind the pathophysiology when considering therapies for children that have known Hereditary Angioedema (the typical allergic reaction medications won’t work). Thankfully, the parents/patients often come with their own medications!

 

References

Nanda MK1, Elenburg S2, Bernstein JA3, Assa’ad AH4. Clinical features of pediatric hereditary angioedema. J Allergy Clin Immunol Pract. 2015 May-Jun;3(3):392-5. PMID: 25609346. [PubMed] [Read by QxMD]

Christiansen SC1, Davis DK2, Castaldo AJ2, Zuraw BL3. Pediatric Hereditary Angioedema: Onset, Diagnostic Delay, and Disease Severity. Clin Pediatr (Phila). 2015 Nov 18. PMID: 26581355. [PubMed] [Read by QxMD]

Craig TJ1, Schneider LC1,2,3, MacGinnitie AJ1,2,3. Plasma-derived C1-INH for managing hereditary angioedema in pediatric patients: A systematic review. Pediatr Allergy Immunol. 2015 Sep;26(6):537-44. PMID: 26111105. [PubMed] [Read by QxMD]

Lumry W1, Soteres D2, Gower R3, Jacobson KW4, Li HH5, Chen H6, Schranz J6. Safety and efficacy of C1 esterase inhibitor for acute attacks in children with hereditary angioedema. Pediatr Allergy Immunol. 2015 Nov;26(7):674-80. PMID: 26171584. [PubMed] [Read by QxMD]

MacGinnitie AJ1. Pediatric hereditary angioedema. Pediatr Allergy Immunol. 2014 Aug;25(5):420-7. PMID: 24313851. [PubMed] [Read by QxMD]

Rubinstein E1, Stolz LE, Sheffer AL, Stevens C, Bousvaros A. Abdominal attacks and treatment in hereditary angioedema with C1-inhibitor deficiency. BMC Gastroenterol. 2014 Apr 9;14:71. PMID: 24712435. [PubMed] [Read by QxMD]

Schneider L1, Hurewitz D, Wasserman R, Obtulowicz K, Machnig T, Moldovan D, Reshef A, Craig TJ. C1-INH concentrate for treatment of acute hereditary angioedema: a pediatric cohort from the I.M.P.A.C.T. studies. Pediatr Allergy Immunol. 2013 Feb;24(1):54-60. PMID: 23173714. [PubMed] [Read by QxMD]

MacGinnitie AJ1, Davis-Lorton M, Stolz LE, Tachdjian R. Use of ecallantide in pediatric hereditary angioedema. Pediatrics. 2013 Aug;132(2):e490-7. PMID: 23878046. [PubMed] [Read by QxMD]

Farkas H1, Zotter Z, Csuka D, Szabó E, Nébenfűhrer Z, Temesszentandrási G, Jakab L, Varga L, Harmat G, Karádi I. Short-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor–a long-term survey. Allergy. 2012 Dec;67(12):1586-93. PMID: 23025435. [PubMed] [Read by QxMD]

Bork K1, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012 Sep;130(3):692-7. PMID: 22841766. [PubMed] [Read by QxMD]

Kreuz W1, Rusicke E, Martinez-Saguer I, Aygören-Pürsün E, Heller C, Klingebiel T. Home therapy with intravenous human C1-inhibitor in children and adolescents with hereditary angioedema. Transfusion. 2012 Jan;52(1):100-7. PMID: 21756262. [PubMed] [Read by QxMD]

Martinez-Saguer I1, Rusicke E, Aygören-Pürsün E, von Hentig N, Klingebiel T, Kreuz W. Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study. Transfusion. 2010 Feb;50(2):354-60. PMID: 19788511. [PubMed] [Read by QxMD]

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Peritonsillar Abscess

Peritonsillar AbscessThroat pain in a child will catch a parent’s attention, which, in turn leads to it catching your attention. Most often the parental concern is over whether this is due to Strep Throat or Mono. Naturally, there are a variety of other issues that we must consider. Perhaps this is merely a Tonsillolith. Of course, we also worry much more about Retropharyngeal Abscesses, Oropharyngeal trauma, and Lemierre’s Syndrome.  Yet, let us not overlook, the most common deep space infection in the neck: Peritonsillar Abscess.

 

Peritonsillar Abscess: Basics

  • Peritonsillar abscess is the most common deep space head and neck infection.
    • In children, it accounts for 49% of head and neck infections. [Ungkanont, 1995]
    • Retrophayrngeal abscess account for 22%. [Ungkanont, 1995]
  • Collection of pus located between the tonsillar capsule, the superior constrictor and the palatopharyngeus muscle. [Schraff, 2001]
  • Complications:
    • Spread to other contiguous structures and into the parapharyngeal space and deeper.
    • Acute enlargement of the tonsillar pillars causing airway constriction!
    • Spontaneous rupture of tonsil and acute aspiration of purulent material. (yuk)

 

Peritonsillar Abscess: Diagnostic Challenge

  • Peritonsillar Abscess should be a clinical diagnosis.
  • The issue is when your clinical exam is limited!
    • Some children will not be the most cooperative with the exam.
      • Trimus (30-60% of the time) may actually prevent their cooperation. [Schraff, 2001; Ungkanont, 1995]
      • Small mouth opening can also obscure your view. [Brodsky, 1988]
    • May need to enhance your exam!
      • Positioning
        • Have child sit on parent’s lap, facing parent, with legs extended around parent’s waist.
        • Have child lay backwards onto parent’s legs, ideally so that head extends slightly over the parent’s knees.
        • This often leads to some “disorientation” and makes the mouth easier to open.
      • Tools
        • Ensure good lighting with headlamp (super cool looking too).
        • Tongue displacement and good lighting with Magill Laryngoscope (See ALIEM Tricks of Trade)
    • Often leads to potential over utilization of CT scans, especially in younger children. [Baker, 2012]
    • May require sedated exam in the OR or ED to more accurately make diagnosis. [Schraff, 2001]

 

Peritonsillar Abscess: Imaging

  • Ideally the image you need is made with your eyeballs, but… if you need see better:
  • Certainly CT is very sensitive [Scott, 1999], but comes with other issues.
    • CT is close to 100% sensitive.
    • Most useful if you are concerned for deeper space infection, like Retropharyngeal Abscess.
  • Ultrasound
    • Intra-oral Ultrasound (using endocavitary probe)
      • Has been shown to be very specific.  [Scott, 1999]
      • Can be somewhat intimidating for children.
    • Trans-cervical Ultrasound (using linear, high frequency probe)
      • Tolerated well and is less intimidating (as there is no large probe being projected into the kid’s painful mouth).
      • Uses the submandibular and midline approaches. [See Bandarkar, 2015 for nice description]
      • Found to be helpful in determining who will not need surgical intervention. [Fordham, 2015]

 

Peritonsillar Abscess: Management

  • Analgesia (please don’t be cruel).
  • Optimize hydration status.
  • Antibiotics
    • Coverage for Streptococcus species is necessary.
    • Don’t forget about Fusobacterium!
    • Often polymicrobial infections are present.
    • Some can even be treated with antibiotics alone. [Nguyen, 2015; Hsiao, 2011]
  • Drainage of Abscess
    • Needle Aspiration
    • Incision and Drainage
      • There has been a trend favoring Incision and Drainage over Tonsillectomy. [Qureshi, 2015]
    • Quinsy Tonsillectomy
      • Tonsillectomy during acute infection.
    • Delayed / Interval Tonsillectomy
      • Tonsillectomy after resolution of infection.
      • Often reported to have less complications, but not necessarily true. [Simon, 2013]

 

Moral of the Morsel

  • Peritonsillar Abscess is common so be familiar with its management.
  • The diagnosis is primarily clinical, but the clinical exam can be challenging, so know how to augment your exam.
  • If uncertain, start with ultrasound, preferentially starting with trans-cervical images!
  • Realize that there are options for management that even include a trial of antibiotic only and reassessment (although I wouldn’t send that one home).
  • In older, cooperative patients, it is completely reasonable to manage as you would an adult with Needle Aspiration in the ED.

 

References

Qureshi H1, Ference E2, Novis S3, Pritchett CV4, Smith SS5, Schroeder JW4. Trends in the management of pediatric peritonsillar abscess infections in the U.S., 2000-2009. Int J Pediatr Otorhinolaryngol. 2015 Apr;79(4):527-31. PMID: 25708703. [PubMed] [Read by QxMD]

Kim DK1,2, Lee JW1, Na YS1, Kim MJ1, Lee JH1,2, Park CH1,2. Clinical factor for successful nonsurgical treatment of pediatric peritonsillar abscess. Laryngoscope. 2015 Nov;125(11):2608-11. PMID: 26152910. [PubMed] [Read by QxMD]

Nguyen T1, Haberland CA1, Hernandez-Boussard T2. Pediatric Patient and Hospital Characteristics Associated With Treatment of Peritonsillar Abscess and Peritonsillar Cellulitis. Clin Pediatr (Phila). 2015 Nov;54(13):1240-6. PMID: 25589309. [PubMed] [Read by QxMD]

Bandarkar AN1, Adeyiga AO2, Fordham MT3, Preciado D4, Reilly BK4. Tonsil ultrasound: technical approach and spectrum of pediatric peritonsillar infections. Pediatr Radiol. 2015 Dec 5. PMID: 26637999. [PubMed] [Read by QxMD]

Fordham MT1, Rock AN2, Bandarkar A3, Preciado D1, Levy M1, Cohen J4, Safdar N3, Reilly BK. Transcervical ultrasonography in the diagnosis of pediatric peritonsillar abscess. Laryngoscope. 2015 Dec;125(12):2799-804. PMID: 25945805. [PubMed] [Read by QxMD]

Simon LM1, Matijasec JW, Perry AP, Kakade A, Walvekar RR, Kluka EA. Pediatric peritonsillar abscess: Quinsy ie versus interval tonsillectomy. Int J Pediatr Otorhinolaryngol. 2013 Aug;77(8):1355-8. PMID: 23810548. [PubMed] [Read by QxMD]

Hsiao HJ1, Huang YC, Hsia SH, Wu CT, Lin JJ. Clinical features of peritonsillar abscess in children. Pediatr Neonatol. 2012 Dec;53(6):366-70. PMID: 23276441. [PubMed] [Read by QxMD]

Baldassari C1, Shah RK. Pediatric peritonsillar abscess: an overview. Infect Disord Drug Targets. 2012 Aug;12(4):277-80. PMID: 22338588. [PubMed] [Read by QxMD]

Baker KA1, Stuart J, Sykes KJ, Sinclair KA, Wei JL. Use of computed tomography in the emergency department for the diagnosis of pediatric peritonsillar abscess. Pediatr Emerg Care. 2012 Oct;28(10):962-5. PMID: 23023458. [PubMed] [Read by QxMD]

Schraff S1, McGinn JD, Derkay CS. Peritonsillar abscess in children: a 10-year review of diagnosis and management. Int J Pediatr Otorhinolaryngol. 2001 Mar;57(3):213-8. PMID: 11223453. [PubMed] [Read by QxMD]

Blotter JW1, Yin L, Glynn M, Wiet GJ. Otolaryngology consultation for peritonsillar abscess in the pediatric population. Laryngoscope. 2000 Oct;110(10 Pt 1):1698-701. PMID: 11037828. [PubMed] [Read by QxMD]

Scott PM1, Loftus WK, Kew J, Ahuja A, Yue V, van Hasselt CA. Diagnosis of peritonsillar infections: a prospective study of ultrasound, computerized tomography and clinical diagnosis. J Laryngol Otol. 1999 Mar;113(3):229-32. PMID: 10435129. [PubMed] [Read by QxMD]

Ungkanont K1, Yellon RF, Weissman JL, Casselbrant ML, González-Valdepeña H, Bluestone CD. Head and neck space infections in infants and children. Otolaryngol Head Neck Surg. 1995 Mar;112(3):375-82. PMID: 7870436. [PubMed] [Read by QxMD]

Brodsky L1, Sobie SR, Korwin D, Stanievich JF. A clinical prospective study of peritonsillar abscess in children. Laryngoscope. 1988 Jul;98(7):780-3. PMID: 3164436. [PubMed] [Read by QxMD]

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