paeds II Flashcards
congneital heart defect
clasification: acyantoic L-> R shunt)
common in paediatric popil.
VSD common. can be simple-> complex.
etiology: chromosome (downs, trisomy 18 (ewards)), turner and klinefelt), infections, drugs, smoking, chemical, genetics.
-VSD, ASD,PDA.
cyanotic: TOF, TFA, TA. (squattin, bypass lungs, boot shaped heart, RSV increase)
acyantoic without shunt (coA0), pumo stenosis (right ventricular obstruction outflow)
congential heart defect with L-R (acyanotic)
VSD (common- chromosomal abnormalities)
ASD
PDA
BF in direct of least resistance, difference b/w defect, size of defect (assoc w/ murmurs).
anatomy: size varies, located in membraneous septum, muscular part, signular or be w/ cardiac defect. pansysoltic, close spontaneous, p2 normal.
large: grade 3-6) LLSB. murmur= size defect (louder) CHF 2 mnths, pulmo HTN. delayed growth, decrease tolerance, recurrent RTI’s /asthma,
trx: surgical closure via 1 yr.
dx: cxr: shows defect, hyperdynamic pulmo HTN, cardiomegaly, prominent PA, enhanced vascular marking on R-side (hilar).
trx: closure, medis for CHF, surgery (3-5 yr)
ASD
osticum primum - 80% associated with MV cleft-> down syndrome.
sinus venosus and defects near VSC exist.
if < 8mm : resolve spontaneously.
patient-> CHF & P.HTN adult.
sx: p2 splits & fixed. systolic murmur (2-6 asymptomatic.
cxr: cardiomegaly w/ pulmo edma.
ecg: RVH+icnomplete atrial AVblock. (abnormally delayed/ blocked ->arrytmia or cardiac arrest (no escape rythme)-> AV dissocition
echo- dilated atrium+RVH. trx: elective surgical / catheter closure by 2 yr.
PDA
high in premature.
jumping carotid, sstolic-diastolic murmur (LUSB) hyerpactive precordium.
LVH seen on EKG. echo shows defect.
trx/: surgical ligation/catheter.
indomethacin (premature): pe2 antagonist -> only premature!!!
congenital HD w/out shunt
co-arction of aorta: constriction of aorta. turner and bicuspid aortic valve associated.
juxtaductal coarction!
sx: HTN, upper extremity higher systolic (difference), decreased flow to lower limb, CHF if severe in1st week.
dx: echo, ECG (LVH), CXR (cardiomegaly/pulmo congestion, rib notching by collateral arteries).
trxX: CHF trx.
balloon arterioplasty if sx.
1) subclavian A used t enalrge constricted part. or cut and anastomose end- to - end,
angiolasty-> balloons to increase diameter.
pulmo stenosis
congenital, rheumatic and extrinsic compression.
RVH , Triscipud regurgitation occurs due to high pressure-> SVC and IVC (abnormal).
only severe then CF (dyspnea, syncope,), JVD, thrills occasionally.
investigation: physical+ history.
RVH and RV overload.
dilated pulmo arteries. echo, catherization shows obstruction and hemodynamic. (measure pressure)
medical: invasive > LV failure, sx only, diuretic and fluid restriction to decrease preload.
surgical: valvuloplasty, PT replacement indicated to inflate balloon.
juvenile rheumatoid arhtirtis
chroicautoimmuneinflammatory disease of synovium. painful joint swelling, febirle.> 6 wks.
F> M. etiology unknown. synovial proliferation, redness, swelling and erosive destruction of cartilage.
oligoarticualr JRA: joint < 6 (smal and medium bones0, absent fever, ANA + (uveitits), leg length discerpancy, growth disturbance. non - destructive
ssytemic: daily high fever.become chronic, hepatomegly, affect all organs, M=F.
polyarthricular JRA: > 6 oints. rarely hip involement. small and large joints. low grade fever, symmetricl, morning stiffness, gelling, bone deformity.
RF+ indicate poor prognosis (bad marker).
evascence rash,
dx of JRA
bloods: wbc, esr elevated, platelets elevated, crp.
RF rarely positive, ANA not specific (uveitits), physcial & hisotry (idiopahtic), MRI and Xray to show inflammation/ erosion. ddx:infectious arthirtis, rheumatic fever, RA., other collagen vascular disease.
trx: steroids, long term NSAIds, methotrexate, systemic injection steroids.
NSAIDs: asprin (gi issue), keptofren, idomethacin, diclofenac sodium,ibruprofen.
2nd line- costosteroids. o (if iridocyclines ) r slower acting (chronic phase) - hhydroxychloroquiunne.
cytotoxic agent.
mutlitdisciplinary approach
SLE
autoimmune disorde characterised of type 2 and 3 hypersensitivity reactin affecting kidneys, skin 9malar, discoid rash), mucosa of some organs (ulcers), brain (neuralgic psychosis, sezirues0, joints-> arthritis.
affects over 5 yrs old childrens.
atinuclear antigen-complex detected.
triggers susceptible genes-> Uv radiation, medications (hydralaine, isoniazid, procainamide), estrogen (predominant in girls).
pleuritis/ pericarditis.
immune system attakca immune system.
antismoth (ribonuclear). anti-dsDNA -specific (active disease), anti-phospholipids.
low c3 - c4 (complement system involved).
trx: anti inflammatory -> biologics, immunosuppressive / corticosteroids.
dermatomyosicites
skin rash- muscle inflammation. rare. idiopathic.
erythematous rash over joints, upper eyelid (purple/ edematous) , muscle weakness-> myalgia).
dx: CF, CK , LDH elevated, EMG, biospy-> atrophy.
same treatment as SLE.
scleroderma-
hardening of the skin, shiny, tight and without skin folds.
non-inflammatory. disease, idiopathic. non inflammatory disease-> fibrosis and vasculopathy.
sclerodactyly, lost fat pads, ulcerate-> chronic.
telangiectasia-> diluted BV small.
calcinoses (deposit under skin),, Raynaud’s phenomena-> vasoconstriction.
dysmotility (reflux, dysphagia),
scleroderma renal crisis.
auto-Ab- extend and organs affected-> ANA.
anti-scl-70/ centromere Ab.
capillaroscopy nailfold-/> health of peripheral capillaries.
same trx.
complications treated separately. gentle skin, emollient, physiotherapy.
PAN
Necrotizing vasculitis involving medium- sized vessels -ANCA negative.
associated with hep B (M: F ).
systemic involvement-> kidney involved, lungs spared.
S+S: constitutional sx (fatigue, weight loss, myalgia, neuropathy, specific rashes
ACR criteria-> clinical, history and lab finding. livedo reticularis. high diastolic TN.
BUN/ creatine ARF, hep B positive, arteriographic abnormality, artery biospy9 mononuclear leukocyte. granulocytes in BV).
trx: cychophosphide, prednisolone 1 mg/kg/ day IV. antiviral if hep B associated.
necrotizing inflammatory lesion occur at bification and branch ppints.
complications: aneyrusm-> hemorhage/ ruptre, thrombi, pan.
mixed CT disease
disorders comibing fearures of different tissue disorders.
sx: raynaids phenonemn, swollen fingers, therapy: same as SLE>
1. reactive arthritis: aspetic arthritis,-> pst GI infection (strep. salmonelle, shigella), 1-2 wk post GI sx.
prognosis-> reiter (conjucitivitis, urethritis) and chronic illness. therapy: AB, NSAIDS< physiotherapy, exercise,local protection, systemic corticosteroids.
psioarisis- lesions with psoariasis. erythematos silvery scale plaque, oligoarhtiritis aymmetricla presentation. conjuctiviits, conjuctivitis.
therapy: steroid cream, retinoic cream, UV, biologics, nsaids.
lyme- borreloa burgdorferi. 5-10 yr old.
months after initial infection-> larger joints affected. macule erythema, febrile, erythema migrans.
trx: doxy/ amoxicillin (under 8 yrs to avoid teeth discoloration) for 30 days.
acute rhemautic fever
molecular mimicry between GAS and human antigen. inflammatory response after 2-34 weeks.
5015 years, or in adulthood.
autoimmune-> genetic predisposition & interaction.
b lymphocytes-> m protein antibody formed_. after sensitization-> similar tissues seen in cardiac myosin lamin, neuronal / subcutaneous and dermal tissues.
s+ s: fever, high grade > 39 degrees. migrating polyarthritis 9large and migrate upwards. arthralgia, pericarditis, valvulitis, SOB, angina, palpitations.
mitral regurgitation, AF risk, HF, infective endocarditis, collagen nodules in extensor surface, erythema migrants, Sydenham chorea.
major: JONES
Minor : CAFEPAL (CRP increase, arthralgia, fever, ESR, prolonged PR interval, amnesias, leucocytosis.
total overall GAS infection and 2 major criteria needed or 1 major and 2 minor.
dx: 1-3 degree AV block.
congested cardiomegaly, echo, throat sputum, serology.
T: symptomatic relief, pain relief, self limiting, no NSAIDS, benzathine benzylpenicillin or erythromycin if GAS>
cardiac-ace , diuretic.
chorea- carbamazepine, valproic acid.
prophylaxis 3-4 w AB.
vasculitis syndromes:
inflammation of BV, thick wal, stenosis, occlusion, subsequent ischmiea. an vessel & disruption. primary are henoch-schoenlein purpura, kawasaki disease. secondary are related to infection, malignancy, collagen vascular disease (SLE< dermatomyosities, drug hypersensitivity).
