Pertussis (Last Updated - 4/30/2005)

-aka "whooping cough" or "the 100-day cough"
-acute
-highly infectious
-char. by paroxysmal coughing episodes ending in whooping inspiration

-Epidemiology:
     -1-2% mortality in children < 1 y.o. (highest in 1st month of life)
     -71% of cases --> children < 5 y.o.
     -38% of cases --> children < 6 months old
     -Incidence:
          -~15,000 per yr. in US
          -peaks in summer & fall (although occurs year-round)
          -cyclical outbreaks Q 3-5 yrs. (despite vaccination)
     -affex 80-100% of un-immunized household contacts
     -affex ~20% of well-immunized household contacts
     -no animal or environmental reservoir

-Causative Agent:
     -aspiration of: Bordetella pertussis
          -Gram neg. bacillus (rod)
          -differentiated from other Bordetella species via oxidase pos. and urease neg.
          -fastidious --> requires charcoal-horse blood agar medium plus nicotinamide
          -slow-growing
          -non-motile
          -strictly aerobic
          -non-fermentative --> does not ferment carb's
          -invades oropharyngeal mucosa --> increased mucous secretion

          -Virulence Factors:
               1) Pertussis Toxin
                    -bacterial exotoxin
                    -composed of a B oligomer-binding subunit and an enzymatically-active A protomer
                    -it ADP-ribosylates a Gi protein in target cells
                         -guanine nucleotide-binding regulatory protein
                         -via permanent adenylate cyclase stimulation by ADP ribosylation --> increased cAMP --> increased mucous production
                    -serves as an adhesin for bacterial binding to ciliated resp. epithelium
                    -also affex circulation of lymphocytes --> inhibits chemokine receptors --> lymphocytosis
                    -induces high-titer Ab's (antitoxins) --> toxoids used as vaccines

               2) Filamentous Hemaglutinin
                    -cell-wall component
                    -bacterial adhsein

               3) Pertactin
                    -outer-membrane protein
                    -bacterial adhesin

               4) Fimbriae
                    -bacterial appendages --> role in bacterial adhesion
                    -major Ag's to which Ab's are directed

               5) Tracheal Cytotoxin
                    -induces resp. epithelial damage

               6) Adenylate Cylcase Toxin
                    -impairs host immune cell function

               7) Dermonecrotic Toxin
                    -contributes to resp. epithelial damage

               8) Lipo-oligosaccharide
                    -bacterial endotoxin

-Incubation Period:
     -7-10 days

-Stages:
     -lasts 6-10 wks. (avg = 7 wks.)
     1) Catarrhal --> inflammation of mucous membranes (esp. throat)
          -symptoms suggestive of common cold:
               -fever
               -sneeze
               -rhinitis
               -dry cough
               -irritability
               -decreased appetite
          -difficult to distinguish from bronchitis or influenza at this stage

     2) Paroxysmal --> sudden periodic attack or recurrence of symptoms
          -occurs after ~2 wks.
          -cough is more violent proceeding to inspiratory whoop (or choking in infants)
               -whoop is caused by spasmodic contraction of the glottis
               -whoop is uncommon in children < 6 months old & frequently absent in older children & adults
                    -present in only ~50% of all cases
          -posttussive vomiting is common at end of paroxysmal coughing episodes
          -may see:
               -face becomes cyanosed
               -eyes become injected
               -veins become distended
               -epistaxis (nosebleed)
               -subconjunctival hemorrhages
          -may also see anywhere from 3-50 paroxysmal coughing episodes QD
          -coughing fits often worse @ night

     3) Convalescent / Decline
          -occurs after several wks.
          -cough progressively ceases

-Diagnosis:
     -suspect pertussis in anyone w/ cough for < 14 days
     A) nasopharyngeal cultures positive in 80-90% of cases --> gold standard
          -nasopharyngeal aspirates preferred or use synthetic fiber swabs (calcium-alginate or Dacron)
               -NOT cotton swabs --> cotton contains FAcids --> toxic to B. pertussis
          -must innoculate immediately (at the bedside) onto charcoal-horse blood agar medium
               -otherwise sample will quickly dry out and die
          -results take ~5 days
     B) pertussis PCR
     C) microscopy via DFA (Direct Fluorescent Ab's)
          -only ~50% sensitive & specific --> not recommended for use

-Differential Diagnosis:
     1) Bronchitis
     2) Influenza
     3) TB
     4) Adenovirus Infec.
     5) RSV (Respiratory Syncytial Virus) Infec.
     6) Pneumonia
          -Mycoplasma or
          -Chlamydia
     7) ACE Inhibitor-Induced cough
     8) Reactive Airway Disease (Ex.) Asthma)
     9) GERD

-Labs:
     -lymphocytosis --> WBC 15,000-20,000 (usually) --> 60-80% lymphocytes
          -may be normal though

-Treatment:
     -symptomatic and supportive
     -may treat close contacts (esp. high-risk pts. --> children < 1 y.o.) with PO erythromycin 12.5 mg/kg QID x 7 or 14 days (not to exceed 2 g/day)
          -treat ONLY during the incubation period --> no benefit of tx if cough has already started
          -erythromycin decreases length of infectivity
          -studies have shown no added benefit to treating for 14 days as opposed to 7 days
               -bacteria are cleared after 5 days
     -use Bactrim (Trimethoprim-Sulfamethoxazole) in pts. unable to tolerate erythromycin
          -4 / 20 mg/kg/day BID x 7 or 14 days

-Prevention / Chemoprophylaxis:
     -immunization (80-85% effective) (DTaP or DTP x 5 IM doses):
          A) 1st dose - 2 months
          B) 2nd dose - 4 months
          C) 3rd dose - 6 months
          D) 4th dose (booster) - 15-18 months (may be given as early as 12 months if 6 months have passed since 3rd dose)
          E) 5th dose (booster) - 4-6 yrs.
     -natural infection likely does NOT confer life-long immunity

     A) DTaP --> Diptheria, Tetanus & acellular Pertussis
          -preferred over DTP (or DTwP) --> less adverse effex
          -no data on long-term immunity (likely not more than 15 yrs.)

     B) DTP (or DTwP)--> Diptheria, Tetanus & whole-cell Pertussis
          -more adverse effex than DTaP
          -contraindicated in children & adults > 7 y.o.
          -short-lived long-term immunity --> 10-12 yrs.

     -prevent spread after onset of symptoms --> isolation
     -treat close contacts (esp. high-risk pts. --> children < 1 y.o.) with PO erythromycin 12.5 mg/kg QID x 14 days (not to exceed 2 g/day)
          -treat ONLY during incubation period --> no benefit if cough is already present
          -erythromycin decreases length of infectivity
          -studies have shown no added benefit to treating for 14 days as opposed to 7 days
               -bacteria are cleared after 5 days
     -use Bactrim (Trimethoprim-Sulfamethoxazole) in pts. unable to tolerate erythromycin
          -4 / 20 mg/kg/day BID x 7 or 14 days

Complications:
     1) Bronchopneumonia --> most common cause of pertussis-induced death (may be confused with SIDS)
          -usually diffuse bilateral primary infec. in infants
          -in older children & adults pneumonia often due to 2nd-ary bacterial infec. (staph or strep)
     2) Petechial Hemorrhages --> from cough
          -subconjunctival, facial or truncal
     3) Syncope
          -likely from cough-induced hypoxia
     4) Seizures
          -likely from cough-induced hypoxia
     5) Encephalopathy
          -likely from cough-induced hypoxia
     6) Asphyxia (esp. in infants)
     7) Pneumothorax --> increased intrathoracic pressures during coughing episodes
     8) Atelectasis --> mucous plugs clog bronchioles
     9) Umbilical Hernia
          -likely from increased abdominal pressure during the coughing fits
     10) Rectal Prolapse
          -likely from increased abdominal pressure during the coughing fits
     11) Otitis Media (esp. in children)

==============================================
References:

1) Taber's Cyclopedic Medical Dictionary: 19th Edition. 1997: pg. 1631-1632
2) First Aid For The USMLE Step 1. Bhushan et al. 2002. pg. 179-180, 188
3) The Merck Manual: 17th Edition. 1999: pg. 1123, 2096-2097, 2306-2308
4) Medical Microbiology: 3rd Edition. 1998: pg. 265-269
5) Harrison's Principles Of Internal Medicine: 15th Edition. 2001: pg. 949-952