Kawasaki Disease

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Mapa Mental sobre Kawasaki Disease, creado por danny ramos el 27/04/2017.
danny ramos
Mapa Mental por danny ramos, actualizado hace más de 1 año
danny ramos
Creado por danny ramos hace más de 7 años
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Resumen del Recurso

Kawasaki Disease
  1. DEFINITION
    1. also known as
      1. mucocutaneous lymph node syndrome
      2. is a disease
        1. blood vessels
          1. through the body
            1. become inflamed
      3. EPIDEMIOLOGY
        1. vasculitis
          1. rare in children
            1. incidence
              1. 10 and 50 / 100.000
              2. < 5% of referrals to pediatric rheumatologist
                1. 2 common types deleted
                  1. < 1%
                    1. of chronic inflammatory disorders in childhood
                2. 2 common types
                  1. kawasaki disease
                    1. similar %
                      1. have evidence of infection
                        1. bacterial
                          1. viral
                            1. systemical
                              1. local
                            2. predominant children
                              1. < 2 years
                                1. rare in late childhood
                              2. henoch schonlein (HSP)
                                1. 1/3 of cases HSP
                                  1. after Streptococcal infection
                              3. posible pathogenesis
                                1. suggest a close connection
                                  1. transmisible agent
                              4. occurs regularly in 1980
                                1. average of patients
                                  1. age decreased
                                    1. % of girls increased
                                  2. decades of attempts to prove
                                    1. viruses
                                      1. bacterial toxins
                                        1. specific pathogens
                                          1. causes most cases
                                            1. not been subestimated
                                      2. RISK FACTOR
                                        1. most type of vasculitis
                                          1. not known
                                            1. rick factors are equally hidden
                                          2. infections
                                            1. inmunization
                                              1. genetic factors
                                                1. inmunomoduladory
                                                  1. haplotypic
                                                    1. vascular genes
                                                      1. do not explain phenomena
                                                    2. monogenic diseases
                                                      1. vasculopathy
                                                        1. associted with
                                                          1. mutations in adenosine deaminase 2
                                                    3. PATHOPHYSIOLOGY
                                                      1. pathology characteristics
                                                        1. primary target of inflammation
                                                          1. medium-sized muscular arteries
                                                          2. coronary arteries
                                                            1. most affected
                                                              1. most likely to suffer
                                                                1. chronic damage
                                                                2. neutrophils
                                                                  1. invade
                                                                    1. vessels walls
                                                                    2. neutrophil infiltrate
                                                                      1. evident in the arterial wall
                                                                        1. first 2 week of disease
                                                                3. immunologic response
                                                                  1. gene expression studies
                                                                    1. DNA microarrays
                                                                      1. from acute - phase whole blood
                                                                        1. confirm increased relative abundance with neutrophils
                                                                          1. adrenomodulin
                                                                            1. grancalcin
                                                                              1. granulin
                                                                                1. decreased NK
                                                                                  1. decreased CD8 + lymphocytes
                                                                        2. CLINICAL FEATURES
                                                                          1. differ from chronic vaasculophaties
                                                                            1. characteristics of any type of vasculitis
                                                                              1. fever
                                                                                1. malaise
                                                                                  1. fatigue
                                                                                    1. failure to thrive
                                                                                      1. increased levels
                                                                                        1. acute - phase reactant
                                                                                      2. progressions immits viral infections
                                                                                        1. rash
                                                                                          1. eritema
                                                                                            1. desquamation
                                                                                            2. mucosal inflammation
                                                                                              1. extremity changes
                                                                                              2. non - exudative conjuntivitis
                                                                                                1. cracked
                                                                                                  1. red lips
                                                                                                    1. strawberry tongue
                                                                                                      1. beau lines (nails)
                                                                                                        1. arthritis
                                                                                                          1. commonly affects
                                                                                                            1. lower extremities
                                                                                                          2. small joint polyarthritis
                                                                                                            1. cervical lymphoadenopaty
                                                                                                            2. LABORATORY FINDINGS
                                                                                                              1. systemic inflammation
                                                                                                                1. PCR
                                                                                                                  1. erytrocyte sedimentation rate
                                                                                                                    1. Alpha 1 antitripsine
                                                                                                                      1. leukocytosis
                                                                                                                        1. increased platelets levels
                                                                                                                        2. normonytic,normochromic anemia
                                                                                                                          1. Hb concentration
                                                                                                                            1. more than 2 DE
                                                                                                                            2. urianalysis
                                                                                                                              1. white blood cells
                                                                                                                              2. liver enzymes
                                                                                                                                1. increased transminase levels
                                                                                                                                  1. mild hyperbilirrubinemia
                                                                                                                                    1. obstructive jaundice
                                                                                                                                      1. from hydrops of gallbladder
                                                                                                                                    2. cerebrospinal fluid
                                                                                                                                      1. pleocythosis
                                                                                                                                    3. DIFFERENTIAL DIAGNOTICS
                                                                                                                                      1. mucocutaneos inflammation
                                                                                                                                        1. measles
                                                                                                                                          1. echovirus
                                                                                                                                            1. adenovirus
                                                                                                                                            2. toxin mediated illness
                                                                                                                                              1. mediated by B - hemolytic streptococcus
                                                                                                                                              2. rocky mountain fever and leptospirosis
                                                                                                                                                1. steven - johnson syndrome
                                                                                                                                                  1. drug reaction
                                                                                                                                                  2. mercury hipersensitive reaction
                                                                                                                                                    1. PAN
                                                                                                                                                      1. rheumatoid arthritis
                                                                                                                                                      2. MANAGMENT
                                                                                                                                                        1. IVIG
                                                                                                                                                          1. for 10 days
                                                                                                                                                            1. reduces incidence of
                                                                                                                                                              1. coronary artery aneurysm
                                                                                                                                                                1. by more than 70%
                                                                                                                                                          2. aspirin
                                                                                                                                                            1. antiinflammatory and antithrombotic effects
                                                                                                                                                              1. initial dose 100 mg/kg/d
                                                                                                                                                                1. once fever is solved
                                                                                                                                                                  1. 3 to 5 mg/kg/d

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