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Part 1
Farooq Ali Khan MDCM
PGY2 FRCP-EM
April 12th 2011
Patient in ambulatory care
 26 y.o. Male
 Moroccan, in Canada x2 y
 Married, works as manager at a wireless
phone company
 CC: Right flank pain
 PMH: none
 Meds: none
 Allergies: none
HPI
 Day 1: headache
 Day 2: nausea/vomiting, chills
 Day 3: watery diarrhea. Chills.
Decreased urine output.
 Day 4: goes to GP, given oral
rehydration and pepto bismol. Takes
tylenol and loratadine for symptoms.
Subsequently develops fever and
hematuria. No dysuria, frequency or
urgency
HPI
 Day 5: presents to ED with R flank pain
radiating to back, chest pain, abdominal
pain, vomiting, diarrhea and frank
hematuria
 No recent antibiotics, travel or exposure
to sick contacts
 Self-limited sore throat with swollen
tonsils for 2 days that occurred 10 days
prior to ED visit. No rhinorrhea, no
hemoptysis
Physical exam
 Triage vitals
 HR 120, BP 103/47, RR 38, T 38.3
 Looks unwell, dry mucus membranes, cracked
lips
 GCS 15, no nuchal rigidity
 No cervical lymphadenopathy
 Chest clear
 Normal S1 S2, no JVD, no murmur
 Abdo diffusely tender maximally in lower
quadrants with some guarding. Bowel sounds
positive. Frank hematuria
 Vitals after 2L bolus – HR 90, BP 96/56
Initial labs
 CBC
 Hgb 168, WBC 8.7, Plat 62
 SMA7
 Na+ 127, K+ 3.7, Cl- 96, HC03
- 16
 Gl 6.1, urea 23.7, creat 399
 LFTs
 T. Bili 22.7, AST 87, ALT 100, ALP 136, GGT 66
 Amylase 59
 LDH 259
 VBG
 pH 7.4, pCO2 21.4, HC03
-15.2, BE -8.2, lactate 2.1
1 shift and 4L of NS later...
 CBC
 Hgb 145, WBC 25, Plat 71
 Man. Diff. Neut 12.2, bands 3.9, echinocytes 2+, decreased
platelets
 SMA7
 Na+ 129, K+ 4.3, Cl- 97, HC03
- 20
 Gl 5.9, urea 26.7, creat 503
 LFTs
 T. Bili 25.2, AST 92, ALT 98, ALP 108, GGT 55
 Lipase 161, CRP 178, Haptoglobin 3.78
 Coags
 INR 1.26, PTT 47.7, D-dimer 3.28, fibrinogen 4.01
 UA
 Blood +++, protein +++, Bili +++, ketones +, glucose –
 Leukocyte esterase Ca 125, nitrite +
 pH 5.5, turbid, red, spec grav 1.015
Microbiology
 Blood gram stain: gram -ve rods
 Antistreptolysin O 569
 Stool C+S, O+P, C.diff negative
 Urine culture negative
 Urine legionella negative
 Sputum culture negative
 NPA for influenza negative
 HIV negative
 Hep A, B, C negative
Initial CXR
CPC Competition - Lemierre's Syndrome
CXR after 2L of fluid
Abdo US report
 CONCLUSION:
 Multiple hepatic cysts with a hypoechoic
liver lesion with cystic changes. This
should be further evaluated by MRI/CT
scan with contrast (if dialysis
considered).
 Slightly enlarged spleen.
 Enlarged and very echogenic kidneys,
consistent with renal medical disease,
consider acute glomerulonephritis.
Course in ED
 Given total 6L of fluid
 Consulted Medicine, ID, Nephro, GI
 Patient on his way to the ICU
Part 2
Course in hospital
 Admitted to the ICU
 Started on Timentin
 Received hemodialysis
 Clinical improvement
 Discharge from ICU after 4 days
Differential diagnoses for renal
failure in this patient
 Hemolytic Uremic Syndrome
 Post-Streptococcal Glomerulonephritis
 Sepsis-induced Acute Tubular Necrosis
HUS?
 In favor
 Diarrheal illness
 Gram -ve rods in
blood
○ (? E. Coli 0157:H7)
 Thrombocytopenia
 Renal dysfunction
 Fever, high WBC and
CRP
 Not in favor
 No anemia
 No hemolysis
 No other
microangiopathic
changes on smear
○ Teardrop cells
○ Helmet cells
○ Microspherocytes
PSGN?
 In favor
 Sore throat prior to
present illness
 Decreased urine
output and fluid
overload
 Gross hematuria and
proteinuria
 Elevated ASOT
 Renal ultrasound
consistent
 Not in favor
 No red cell casts on
microscopy
 C3 and C4 levels
were later found to be
normal
 Does not explain
other bacteremia and
GI symptoms
 Renal biopsy
inconsistent
CPC Competition - Lemierre's Syndrome
Sepsis
 In favor
 SIRS
 Metabolic acidosis
 Multisystem organ
dysfunction
○ GI symptoms
○ Elevated LFTs
○ Consumptive
coagulopathy
 Gram -ve rods in blood
culture
 Renal biopsy consistent
with ATN
 A few unanswered
questions..
 Why did someone so
young get sick so
quickly?
 Why were so many
systems involved
despite no evidence of
immune suppression?
 What was the
organism?
 Was the sore throat a
red herring?
CPC Competition - Lemierre's Syndrome
Today on grand rounds reruns...
Fusobacterium
necrophorum
 Anaerobe primarily responsible for
oropharyngeal infections
 Associated with Lemierre’s syndrome
Epidemiology
 F. Necrophorum causes endemic
pharyngitis
 Primarily adolescents and young adults (16-30)
 incidence of 10% (equal to group A strep)
 ~1 in 400 cases of F. Necrophorum
pharyngitis can lead to systemic
necrobacillosis and Lemierre’s syndrome
 Mortality 4.6%
 Resurgence in last 15 years
Pathophysiology
 Pharyngitis caused by
 Fusobacterium Necrophorum primarily
 Another organism (e.g. GAS) with
secondary colonization by F. Necrophorum
Pathophysiology
 Invades blood vessels in parapharyngeal
space causing bacteremia with a wide
range of presentations
 Classic fulminating Lemierre's syndrome
○ IJ thrombus
○ Rapid dissemination of septic microemboli to
lung, joints, intraabdominal organs, and CNS
 Transient bacteremia which leads weeks
later to a focal abscess
 Toxin production leading to sepsis with
thrombocytopenia and DIC
What can be done about it?
 Necrobacillosis and LS are preventable
 Think about it when seeing a young person
with a sore throat
 Consider throat culture for other organisms
besides Strep
 Red flags for pharyngitis
 Symptoms do not resolve in 3-5 days
 Symptoms rapidly worsen
 Unilateral neck swelling
 Systemic symptoms (e.g. Bacteremic)
 Treat with penicillin/flagyl or clindamycin
References
 Marx: Rosen's Emergency Medicine, 7th ed. - 2009 - Mosby, An Imprint
of Elsevier.
 Riordan T. Human infection with Fusobacterium necrophorum
(Necrobacillosis), with a focus on Lemierre’s syndrome. Clin
Microbiol Rev. 2007;20:622- 59. [PMID: 17934077]
 Centor RM. Expand the Pharyngitis Paradigm for Adolescents and
Young Adults. Annals of Internal Medicine. 2009;151(11):812-5
 Batty A, Wren MW. Prevalence of Fusobacterium necrophorum and
other upper respiratory tract pathogens isolated from throat swabs.
Br J Biomed Sci. 2005;62:66-70
 Amess JA, O’Neill W, Giollariabhaigh CN, Dytrych JK. A six-month
audit of the isolation of Fusobacterium necrophorum from patients
with sore throat in a district general Hospital. Br J Biomed Sci.
2007
 Hagelskjaer Kristensen L, Prag J. Lemierre’s syndrome and other
disseminated Fusobacterium necrophorum infections in Denmark: a
prospective epidemiological and clinical survey. Eur J Clin Microbiol
Infect Dis. 2008;27:779-89
References
 Syed MI, Baring D, Addidle M, Murray C, Adams C
(September 2007). "Lemierre syndrome: two cases
and a review". The Laryngoscope (The American
Laryngological, Rhinological & Otological Society;
Lippincott Williams & Wilkins) 117 (9): 1605–1610
 Screaton NJ, Ravenel JG, Lehner PJ, Heitzman ER,
Flower CD (November 1999). "Lemierre Syndrome:
Forgotten but Not Extinct-Report of Four
Cases". Radiology (Radiological Society of North
America) 213 (2): 369–374
 Beldman TF, Teunisse HA, Schouten TJ (November
1997). "Septic arthritis of the hip by Fusobacterium
necrophorum after tonsillectomy: a form of Lemierre
syndrome?". European journal of pediatrics (Springer-
Verlag) 156 (11): 856–857

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CPC Competition - Lemierre's Syndrome

  • 1. Part 1 Farooq Ali Khan MDCM PGY2 FRCP-EM April 12th 2011
  • 2. Patient in ambulatory care  26 y.o. Male  Moroccan, in Canada x2 y  Married, works as manager at a wireless phone company  CC: Right flank pain  PMH: none  Meds: none  Allergies: none
  • 3. HPI  Day 1: headache  Day 2: nausea/vomiting, chills  Day 3: watery diarrhea. Chills. Decreased urine output.  Day 4: goes to GP, given oral rehydration and pepto bismol. Takes tylenol and loratadine for symptoms. Subsequently develops fever and hematuria. No dysuria, frequency or urgency
  • 4. HPI  Day 5: presents to ED with R flank pain radiating to back, chest pain, abdominal pain, vomiting, diarrhea and frank hematuria  No recent antibiotics, travel or exposure to sick contacts  Self-limited sore throat with swollen tonsils for 2 days that occurred 10 days prior to ED visit. No rhinorrhea, no hemoptysis
  • 5. Physical exam  Triage vitals  HR 120, BP 103/47, RR 38, T 38.3  Looks unwell, dry mucus membranes, cracked lips  GCS 15, no nuchal rigidity  No cervical lymphadenopathy  Chest clear  Normal S1 S2, no JVD, no murmur  Abdo diffusely tender maximally in lower quadrants with some guarding. Bowel sounds positive. Frank hematuria  Vitals after 2L bolus – HR 90, BP 96/56
  • 6. Initial labs  CBC  Hgb 168, WBC 8.7, Plat 62  SMA7  Na+ 127, K+ 3.7, Cl- 96, HC03 - 16  Gl 6.1, urea 23.7, creat 399  LFTs  T. Bili 22.7, AST 87, ALT 100, ALP 136, GGT 66  Amylase 59  LDH 259  VBG  pH 7.4, pCO2 21.4, HC03 -15.2, BE -8.2, lactate 2.1
  • 7. 1 shift and 4L of NS later...  CBC  Hgb 145, WBC 25, Plat 71  Man. Diff. Neut 12.2, bands 3.9, echinocytes 2+, decreased platelets  SMA7  Na+ 129, K+ 4.3, Cl- 97, HC03 - 20  Gl 5.9, urea 26.7, creat 503  LFTs  T. Bili 25.2, AST 92, ALT 98, ALP 108, GGT 55  Lipase 161, CRP 178, Haptoglobin 3.78  Coags  INR 1.26, PTT 47.7, D-dimer 3.28, fibrinogen 4.01  UA  Blood +++, protein +++, Bili +++, ketones +, glucose –  Leukocyte esterase Ca 125, nitrite +  pH 5.5, turbid, red, spec grav 1.015
  • 8. Microbiology  Blood gram stain: gram -ve rods  Antistreptolysin O 569  Stool C+S, O+P, C.diff negative  Urine culture negative  Urine legionella negative  Sputum culture negative  NPA for influenza negative  HIV negative  Hep A, B, C negative
  • 11. CXR after 2L of fluid
  • 12. Abdo US report  CONCLUSION:  Multiple hepatic cysts with a hypoechoic liver lesion with cystic changes. This should be further evaluated by MRI/CT scan with contrast (if dialysis considered).  Slightly enlarged spleen.  Enlarged and very echogenic kidneys, consistent with renal medical disease, consider acute glomerulonephritis.
  • 13. Course in ED  Given total 6L of fluid  Consulted Medicine, ID, Nephro, GI  Patient on his way to the ICU
  • 15. Course in hospital  Admitted to the ICU  Started on Timentin  Received hemodialysis  Clinical improvement  Discharge from ICU after 4 days
  • 16. Differential diagnoses for renal failure in this patient  Hemolytic Uremic Syndrome  Post-Streptococcal Glomerulonephritis  Sepsis-induced Acute Tubular Necrosis
  • 17. HUS?  In favor  Diarrheal illness  Gram -ve rods in blood ○ (? E. Coli 0157:H7)  Thrombocytopenia  Renal dysfunction  Fever, high WBC and CRP  Not in favor  No anemia  No hemolysis  No other microangiopathic changes on smear ○ Teardrop cells ○ Helmet cells ○ Microspherocytes
  • 18. PSGN?  In favor  Sore throat prior to present illness  Decreased urine output and fluid overload  Gross hematuria and proteinuria  Elevated ASOT  Renal ultrasound consistent  Not in favor  No red cell casts on microscopy  C3 and C4 levels were later found to be normal  Does not explain other bacteremia and GI symptoms  Renal biopsy inconsistent
  • 20. Sepsis  In favor  SIRS  Metabolic acidosis  Multisystem organ dysfunction ○ GI symptoms ○ Elevated LFTs ○ Consumptive coagulopathy  Gram -ve rods in blood culture  Renal biopsy consistent with ATN  A few unanswered questions..  Why did someone so young get sick so quickly?  Why were so many systems involved despite no evidence of immune suppression?  What was the organism?  Was the sore throat a red herring?
  • 22. Today on grand rounds reruns...
  • 23. Fusobacterium necrophorum  Anaerobe primarily responsible for oropharyngeal infections  Associated with Lemierre’s syndrome
  • 24. Epidemiology  F. Necrophorum causes endemic pharyngitis  Primarily adolescents and young adults (16-30)  incidence of 10% (equal to group A strep)  ~1 in 400 cases of F. Necrophorum pharyngitis can lead to systemic necrobacillosis and Lemierre’s syndrome  Mortality 4.6%  Resurgence in last 15 years
  • 25. Pathophysiology  Pharyngitis caused by  Fusobacterium Necrophorum primarily  Another organism (e.g. GAS) with secondary colonization by F. Necrophorum
  • 26. Pathophysiology  Invades blood vessels in parapharyngeal space causing bacteremia with a wide range of presentations  Classic fulminating Lemierre's syndrome ○ IJ thrombus ○ Rapid dissemination of septic microemboli to lung, joints, intraabdominal organs, and CNS  Transient bacteremia which leads weeks later to a focal abscess  Toxin production leading to sepsis with thrombocytopenia and DIC
  • 27. What can be done about it?  Necrobacillosis and LS are preventable  Think about it when seeing a young person with a sore throat  Consider throat culture for other organisms besides Strep  Red flags for pharyngitis  Symptoms do not resolve in 3-5 days  Symptoms rapidly worsen  Unilateral neck swelling  Systemic symptoms (e.g. Bacteremic)  Treat with penicillin/flagyl or clindamycin
  • 28. References  Marx: Rosen's Emergency Medicine, 7th ed. - 2009 - Mosby, An Imprint of Elsevier.  Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007;20:622- 59. [PMID: 17934077]  Centor RM. Expand the Pharyngitis Paradigm for Adolescents and Young Adults. Annals of Internal Medicine. 2009;151(11):812-5  Batty A, Wren MW. Prevalence of Fusobacterium necrophorum and other upper respiratory tract pathogens isolated from throat swabs. Br J Biomed Sci. 2005;62:66-70  Amess JA, O’Neill W, Giollariabhaigh CN, Dytrych JK. A six-month audit of the isolation of Fusobacterium necrophorum from patients with sore throat in a district general Hospital. Br J Biomed Sci. 2007  Hagelskjaer Kristensen L, Prag J. Lemierre’s syndrome and other disseminated Fusobacterium necrophorum infections in Denmark: a prospective epidemiological and clinical survey. Eur J Clin Microbiol Infect Dis. 2008;27:779-89
  • 29. References  Syed MI, Baring D, Addidle M, Murray C, Adams C (September 2007). "Lemierre syndrome: two cases and a review". The Laryngoscope (The American Laryngological, Rhinological & Otological Society; Lippincott Williams & Wilkins) 117 (9): 1605–1610  Screaton NJ, Ravenel JG, Lehner PJ, Heitzman ER, Flower CD (November 1999). "Lemierre Syndrome: Forgotten but Not Extinct-Report of Four Cases". Radiology (Radiological Society of North America) 213 (2): 369–374  Beldman TF, Teunisse HA, Schouten TJ (November 1997). "Septic arthritis of the hip by Fusobacterium necrophorum after tonsillectomy: a form of Lemierre syndrome?". European journal of pediatrics (Springer- Verlag) 156 (11): 856–857