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2.6 REHERVIDOR .1 INTRODUCCIÓN

   

What  is  fever?    

What  is  the  mechanism  behind  fever?    

Why  can  some  people  with  no  obvious  illness  have  fever?  

Why  do  some  people  with  illness  have  NO  fever?    

I  want  to  EMPHASIZE  strongly  that  the  elderly  may  not  have  the  ability  to  develop   Fever  when  ill.  Absence  of  fever  does  not  mean  anything  

Chee  Yong  Chuan:  Fever  is  defined  as  the  elevation  of  core  body  temperature  above   normal.  In  normal  adults,  the  average  oral  temperature  is  37  degrees  Celsius.  

 

The  febrile  response  is  a  complex  physiologic  reaction  to  disease  involving  a  

cytokine-­‐mediated  rise  in  body  temperature  and  generation  of  various  acute  phase   reactants.  Fever  is  thus  considered  a  hallmark  of  IMMUNE  SYSTEM  ACTIVATION,   resulting  in  a  regulated  rise  in  body  temperature.  So  what  happens  is  that  

exogenous  pyrogens  from  infectious  agents,  toxins,  tumours  induce  production  of   pro-­‐inflammatory  cytokines,  such  as  interleukins,  TNF  which  subsequently  enter  the   hypothalamic  circulation  and  stimulate  release  of  local  prostaglandins(this  is  where   our  antipyretics  and  NSAID  exert  their  effects).  The  body  will  then  react  to  rise  the   temperature  to  this  new  thermal  set  point(manifested  by  chills  and  shivering).    

 

Why  do  some  people  with  illness  have  no  fever?  I  can  observe  that  response  to   fever  varies  with  age.  Elderly  patients  especially  are  unable  to  regulate  their  body   temperature  to  the  same  degree  as  young  adults.  Prof  Wong  had  reiterated  many   times  that  older  patients  with  serious  infections  have  substantial  prevalence  of  a   pyrexia  or  LOWER  febrile  responses!  Don't  be  surprised  to  see  hypothermia  instead   in  full  blown  sepsis  in  this  group  of  patients.  Fever  is  also  considered  to  be  an  

important  host  defence  mechanism,  hence  in  those  who  are  immunocompromised   i.e  HIV,  patients  receiving  steroid  therapy,  neutropenic  patients,  due  to  the  inability   to  mount  an  adequate  immune  response,  might  not  give  you  the  textbook  febrile   response  that  you  would  have  expected.  I  can  think  of  a  few  examples  where   patients  who  are  well  but  developing  fever.    

 

1)  Transfusion  associated  fever.  Again,  due  to  activation  of  the  immune  system   against  antigen  on  the  donor  blood  

2)  Drug  induced,  probably  affects  the  ability  of  the  body  to  dissipate  heat,  or  

through  immune  system  activation,  serum  sickness,  allergy   3)  Factitious  fever  

 

Prof  :  difference  between  fever  and  hyperthermia  

   

Prof  :  Dear  Yin  Ling,    

I  hate  all  these  definitions  as  my  RAM  is  simply  too  small  to  process  them.  I  think  of   FEVER  as  a  problem  but  you  are  sitting  for  exams  and  hence  stuck  in  the  system.  

 

DEFINITIONS  

FUO—>38.3C  [>101.8F],  duration  >3  weeks,  diagnosis  uncertain  after  3  days  in   hospital  or  "three  outpatient  visits"!!  This  is  close  to  our  old  definition.  

 

NOSOCOMIAL  FUO—hospitalized  patients,  >38.3C  [>101.8F],  diagnosis  uncertain   after  3  days  and  infection  not  present  or  incubating  on  admission  

 

IMMUNE-­‐DEFICIENT  (NEUTROPENIC)  FUO—  >38.3C  [>101.8F],  >3  days,  neutrophil   count  <500/mm3.    

 

HIV-­‐RELATED  FUO—HIV  patients,  >38.3C  [>101.8F],  duration  >3  weeks  for   outpatients  or  ">3  days  for  inpatients"  

The  era  of  high  technology  has  changed  the  goalposts  considerably,  note  inpatients   now  are  THREE  days  to  a  diagnosis.  With  every  scan  thrown  in  from  PET  CT  to  MRI,  3   days  in  the  IDEAL  hospital  appears  enough.  

 

FEVER,  NYD—persistent  fever  that  has  not  yet  met  the  definition  for  FUO.  

 

In  the  HISTORY    

-­‐the  pattern  and  duration  of  fever,    

-­‐the  associated  symptoms  (cough,  dyspnea,  hemoptysis,  chest  pain,  diarrhea,   abdominal  pain,  dysuria,  urethral  discharge,  hematuria,  neck  stiffness,  headache),     -­‐any  rash  (palpable  purpura,  exanthem),    

-­‐any  exposure  (food,  water,  plants,  animals,  insects,  infected  human  secretions),     -­‐weight  loss,  night  sweats,    

-­‐travel  history,  sexual  history,  HIV  risk  factors,  immunizations,    

-­‐past  medical  history  (rheumatologic  disorders,  malignancy,  alcohol),     -­‐medications  

are  ALL  CRUCIAL.  

 

PHYSICAL  exam—  

vitals  (tachycardia,  tachypnea,  hypotension,  fever,  hypoxemia),     oral  ulcers,  lymphadenopathy,    

nuchal  rigidity,    

respiratory  and  cardiac  examination  (murmurs),     temporal  artery,    

abdominal  examination  (hepatosplenomegaly),     prostate  examination,    

skin  lesions  (morphology,  distribution),  insect  bite  marks,     joint  examination  

 

Always  think  of:  

 

INFECTIONS—TB  (pulmonary,  extrapulmonary,  miliary),    

abscess  (liver,  splenic,  perinephric,  psoas,  diverticular,  pelvis),  osteomyelitis,     endocarditis    

 

NEOPLASTIC—hematologic  (lymphoma,  leukemia,  multiple  myeloma,   myelodysplastic  syndrome),  solid  tumors  (renal  cell,  hepatoma)      

COLLAGEN-­‐VASCULAR—vasculitis  (giant  cell  arteritis,  Still’s  disease,  polyarteritis   nodosa,  Takayasu’s  arteritis,  Wegener’s  granulomatosis,  mixed  cryoglobulinemia),   lupus,  rheumatoid  arthritis    

 

DRUGS—antimicrobials  (sulfonamides,  penicillins,  nitrofurantoin,  antimalarials),   antihistamines,  antiepileptics  (barbiturate,  phenytoin),  NSAIDs/  ASA,  

antihypertensives  (hydralazine,  methyldopa),  antiarrhythmics  (quinidine,   procainamide),  antithyroid,  iodides,  quinine,  illicit  (cocaine)    

 

UNCOMMON  CAUSES  OF  FUO—  

endocrine  (hypothalamic  dysfunction,  hyperthyroidism,  pheochromocytoma,   adrenal  insufficiency),    

infections  (dental  abscess,  leptospirosis,  psittacosis,  melioidosis,  syphilis,  

gonococcemia,  hereditary  periodic  fever  syndromes  (familial  Mediterranean  fever,   alcoholic  hepatitis,  hematoma,  factitious  fever  

 

Pls  do  not  forget  the  good  old  CLASSIC  DEFINITION  (1961)—>38.3C  [>101.8F],   duration  >3  weeks,  diagnosis  uncertain  after  7  days  of  investigation  in  hospital    

Prof  :  Malignancies  have  now  superseded  infections  as  the  most  common  cause  of   fever  of  unknown  origin  (FUO).  Did  you  know  this?    

In  the  past  infectious  diseases  were  the  most  common  etiology  of  FUO,  and  

neoplasms  constituted  the  second  most  frequent  category.  This  shift  from  infectious   to  malignant  etiology  as  the  most  frequent  cause  of  FUO  is  related  to  several  

factors.    

 

Firstly,  due  to  the  widespread  introduction  of  computed  tomography  (CT)  and   magnetic  resonance  imaging  (MRI),  many  intra-­‐abdominal  causes  of  infection  are   diagnosed  early  and  therefore  do  not  meet  the  definition  of  FUO.  Sub  phrenic   abscesses,  pelvic  pathologies  and  even  sinus  infections  well  hidden  from  the   clinician  Is  not  exposed  clearly.    

 

Secondly,  radionucleotide  imaging  studies,  that  is,  indium  scans,  gallium  scans,  and   bone  scans,  have  been  useful  in  identifying  occult  malignancies  undetectable  by   other  means.    

 

Now  PET  CT  has  made  detection  of  both  well  hidden  infections  and  malignancies   much  easier.    

Prof  :  lymphoma  is  the  most  common  etiology  of  neoplastic  fever  of  

underdetermined  origin.  The  pathophysiology  of  tumor-­‐induced  fever  may  be  due   to  several  mechanisms  of  which  release  of  cytokines  from  tumor  cells  or  tumor   necrosis  factor  and  interleukin-­‐1;  necrosis  of  tumoral  tissue;  all  contribute.  

I  cannot  resist  asking  you,  What  is  the  Naproxen  challenge  in  FUO?  ?    

THE  NSAID  naproxen  Is  very  effective  in  suppressing  tumor  fever  and  this  property   may  be  useful  in  elucidating  suspicion  of  cancer  in  patients  with  prolonged,  

undiagnosed  fever.  

 

Naproxen  IS  THE  classically  touted  agent  for  suppressing  tumor  fever  due  to  its   unique  ability  to  suppress  tumoral  cytokines  in  preference  over  infectious   cytokines.    

 

While  the  “naproxen  challenge”  may  be  useful  in  evaluating  prolonged  fever  

suspected  to  be  of  neoplastic  origin,  it  must  be  utilized  in  the  context  of  a  thorough,   clinically-­‐driven  assessment.    

 

14)  NMS      

A  66-­‐year-­‐old  male  was  hospitalized  for  increasingly  aggressive  behavior.  He  had  no   prior  psychiatric  admissions.  On  the  day  of  admission  after  he  sustained  a  fall,  a  CT   scan  of  the  brain  revealed  a  subarachnoid  hemorrhage  at  the  right  superior  sulcus   and  a  possible  hemorrhagic  contusion  at  the  left  frontal  lobe.    

 

Over  the  course  of  hospitalization,  the  patient  had  a  series  of  CT  scans  showing   resolution  of  the  hemorrhage.    

 

He  was  started  on  olanzapine  for  intermittent  agitation.  Olanzapine  was  titrated  to   7.5  mg  daily.Ten  days  later  the  patient  became  abruptly  somnolent  with  body   temperature  reaching  39.7  º  C  and  severe  muscle  rigidity  in  both  upper  and  lower   extremities.  He  had  severe  diaphoresis  and  fluctuation  of  blood  pressure  and  pulse.  

 

Laboratory  data  revealed  elevation  of  white  blood  cells  to  14800  K/L,  creatine  

phosphokinase  to  2800  U/L  (normal  <  174  U/L),  and  mild  elevation  of  serum  alanine   and  aspartate  aminotransferase.  MRI  of  the  brain,  CSF  studies,  and  chest  radiograph   were  unremarkable.  

 

What  is  the  diagnosis?    

What  will  you  do  now?                  

Pathophysiology  fr  Medscape    

''  The  most  widely  accepted  mechanism  by  which  antipsychotics  cause  neuroleptic   malignant  syndrome  is  that  of  dopamine  D2  receptor  antagonism.  In  this  widely   accepted  model,  central  D2  receptor  blockade  in  the  hypothalamus,  nigrostriatal   pathways,  and  spinal  cord  leads  to  increased  muscle  rigidity  and  tremor  via   extrapyramidal  pathways.  

 

Hypothalamic  D2  receptor  blockade  results  in  an  elevated  temperature  set  point   and  impairment  of  heat-­‐dissipating  mechanisms.  Peripherally,  antipsychotics  lead  to   increased  calcium  release  from  the  sarcoplasmic  reticulum,  resulting  in  increased   contractility,  which  can  contribute  to  hyperthermia,  rigidity,  and  muscle  cell   breakdown.  

 

Beyond  these  direct  effects,  D2  receptor  blockade  might  cause  neuroleptic   malignant  syndrome  by  removing  tonic  inhibition  from  the  sympathetic  nervous   system.  The  resulting  sympathoadrenal  hyperactivity  and  dysregulation  leads  to   autonomic  dysfunction.  This  model  suggests  that  patients  with  baseline  high  levels   of  sympathoadrenal  activity  might  be  at  increased  risk.  While  this  has  not  been   proven  in  controlled  studies,  several  such  states  have  been  proposed  as  risk  factors   for  neuroleptic  malignant  syndrome.''              

Diagnostic  features  of  NMS:  

 

Neuroleptics  within  1  to  4  weeks.  

Hyperthermia  (above  38°).  

Muscle  rigidity  lead  pipe.  

Five  of  the  following:  

 

Changed  mental  status.Tachycardia.Hypotension  or  

hypertension.Tremor.Incontinence.Diaphoresis  (excessive  sweating)  or  sialorrhoea.  

Increased  creatine  phosphokinase  (CPK)  or  urinary  myoglobin.  

Metabolic  acidosis.Leukocytosis.  

Exclusion  of  other  illnesses  (neuropsychiatric,  drug-­‐induced,  systemic).  

 

Prof  :  Have  you  heard  of  'an  extreme  Parkinson's  crisis'  ?    

It  may  be  possible  that  NMS  is  as  an  extreme  parkinsonian  crisis  resulting  from   overwhelming  blockade  of  dopamine  pathways  in  the  brain.  

 

In  this  view,  NMS  resembles  the  parkinsonian-­‐hyperthermia  syndrome  that  can   occur  in  Parkinson’s  disease  patients  following  abrupt  discontinuation  

or  loss  of  efficacy  of  dopaminergic  therapy,  which  can  be  treated  by  reinstituting   dopaminergic  agents.  

 

Evidence  to  support  this  view  includes:  

 

•  Parkinsonian  signs  are  a  cardinal  feature  of  NMS    

.•  Withdrawal  of  dopamine  agonists  precipitates  the  syndrome.  

 

•  All  triggering  drugs  are  dopamine  receptor  antagonists.  

 

•  Risk  of  NMS  correlates  with  drugs’  dopamine  receptor  affinity.  

 

•  Dopaminergic  agonists  may  be  an  effective  treatment.  

 

•  Lesions  in  dopaminergic  pathways  produce  a  similar  syndrome.  

 

•  Patients  with  NMS  have  demonstrated  low  cerebrospinal  fluid  concentrations  of   the  dopamine  metabolite  homovanillic  acid.  

 

Yin  ling:  this  patient  is  likely  having  what  we  know  as  a  Neuroleptic  Malignant   Syndrome  due  to  the  Olanzapine  that  was  just  started  ten  days  ago.    

 

Neuroleptic  malignant  sydrome  is  caused  by  drugs  causing  Dopamine  receptor   antagonists  (commonly  antipsychotics)  and  also  withdrawal  of  Dopamine  agonists   (commonly  anti  Parkinsonian  drugs).  temperature  regulation  became  haywire  and   patient  presents  with  classic  tetrad  of  hyperthermia  which  is  unable  to  be  brought   down  by  antipyrogens,  RIGIDITY,  mental  confusion/somnolence/coma,  and  

autonomic  dysfunction  (perfused  sweating,  tachycardia,  labile  BP  and  etc).  it  can   occur  after  a  few  days  of  taking  the  drugs  up  to  years  after  that.  classically  it  occurs   within  a  few  weeks.    

 

Labs  wise:  increase  total  white  and  v  high  CK,  deranged  transaminases  and  decrease   in  se  iron  is  classical  for  NMS.    

 

Stopping  the  culprit  drug  is  first  and  foremost.  Secondly  bring  down  the  patients   temperature  with  methods  like  cooling  blanket,  hydration,  and  medical  therapy  with   dopamine  agonists  like  bromocriptine  can  help.  Dantrolene  a  muscle  relaxant  is   used  too  but  not  available  in  many  hospitals.  Supporting  patient's  ABC  is  of  course   vital    

 

NMS  has  been  claimed  to  have  some  similar  genetic  profile  as  Malignant   Hyperthermia  that  occurs  when  anaest  drugs  classically  succinylcholine  is   administered.  MH  is  autosomal  dominant  and  a  drug  history  will  help  us  with   diagnosis.    

 

It  is  often  difficult  to  differentiate  Serotonin  syndrome  and  NMS.  even  more  so   when  serotonin  containing  drugs  will  also  have  dopamine  antagonist  effect.    

 

Serotonin  syndrome  is  due  to  a  high  level  of  serotonin  in  the  body  caused  by  giving   two  serotonin  containing  drugs  concurrently,  giving  these  drugs  tgt  with  CYP  2D6/  

3A4  inhibitors  OR  giving  a  Long  Acting  drugs  which  increase  serotonin  eg  

Prozac(fluoxetine).  classical  drugs  that  causes  SS  are  antidepressants  like  SSRI,  SNRI,   MAOi-­‐A  (MAOi-­‐B  that  we  are  giving  to  our  parkinsons  disease  patients  won't  cause   that  effect.  i  don't  know  why).  other  common  drugs  are  PAIN  CONTROL  drugs  eg   Tramadol,  fentanyl,  meperidine;  ANTIEMETRICS  eg  Maxolon,  granisetron(Kytril),   ondansetron;  TRIPTANS,  LITHIUM.  

 

Pathophysiology  of  NMS  and  SS  is  different  because  SS  does  not  cause  

hyperthermia  by  altering  the  hypothalamus  setpoint.  it  is  due  to  the  overall   hyperexcitability.  

 

The  few  ways  to  differentiate:    

1)  Serotonin  syndrome  causes  neuromuscular  excitation  :  hyperreflexia,  myclonus,   clonus,  increase  in  bowel  sounds,  pupils  dilatation,  as  opposed  to  LEAD  PIPE  

RIGIDITY  in  NMS.  

 

2)  Lab  reuslts  of  increase  total  white  and  CK  and  transminases  and  decrease  se  iron   will  point  to  NMS.  SS  less  likely    

 

3)  SS  can  occur  immediately  after  serotonin  containing  drugs  is  given  while  NMS  

might  occur  after  a  few  weeks.    

 

Mx  of  SS  :  diazepam  for  the  hyperexcitability,  intubation  and  sedation  may  be   needed.  anti  serotonergic  drug  like  cyproheptadine  can  be  given.  

 

Dr  Hu  :  Neuroleptic  malignant–like  syndrome  (NMLS)  ……  

 

Withdrawal  or  dose  reduction  of  levodopa  in  patients  with  Parkinson’s  disease  (PD)   has  been  reported  to  precipitate  a  potentially  fatal  syndrome  closely  resembling   neuroleptic  malignant  syndrome  (NMS).  This  syndrome  is  referred  to  as  neuroleptic   malignant–like  syndrome  (NMLS)  and  parkinsonism-­‐hyperpyrexia  syndrome  by   some  authors.  

 

Clinical  features  of  NMS  and  NMLS  are  very  similar  and  include  high  fever,  marked   rigidity,  altered  consciousness,  leukocytosis,  autonomic  dysfunction,  rhabdomyolysis   with  elevated  serum  creatine  kinase  (CK)  levels,  and  renal  failure.  NMS  and  NMLS   share  a  similar  pathophysiologic  mechanism  consisting  of  an  acute  reduction  in   nigrostriatal  and  hypothalamic  cerebral  dopaminergic  transmission.  In  the  case  of   NMS,  the  primary  mechanism  occurs  when  neuroleptics  block  D2  dopamine  

receptors,  while  the  cause  of  NMLS  is  the  withdrawal  of  exogenous  dopamine.  This   usually  occurs  in  the  form  of  its  precursor,  levodopa.  Clinical  conditions  that  may   contribute  to  the  occurrence  of  NMLS  include  disturbance  in  the  gastrointestinal   tract  resulting  in  poor  absorption  of  levodopa,  intercurrent  infection,  dehydration   due  to  heat,  and  poor  adherence  to  medications.  

 

It  is  well  recognized  that  “high  dietary  protein”  intake  can  impair  the  absorption  of   levodopa,  leading  to  loss  of  efficacy  and  PD  symptom  fluctuations.  The  amino  acids   of  the  protein  may  compete  with  levodopa  for  absorption  in  the  gut  and  for  

transport  through  the  blood-­‐brain  barrier.  (Health-­‐care  professionals  should  be   aware  of  the  interaction  between  levodopa  and  protein  content  of  enteral   nutrition  to  avoid  the  occurrence  of  NMLS  in  patients  with  PD.)  

 

The  treatment  of  NMLS  due  to  levodopa  withdrawal  consists  of  supportive   measures,  hydration,  and  Levodopa  reintroduction  or  increase  in  dose  can  be   effective.  

 

(The  Annals  of  Pharmacotherapy    2010  September,  Volume  44)    

15)  on  Cardiac  Tamponade    

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