paeds II

Question 1

congneital heart defect

Answer 1

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)

Question 2

congential heart defect with L-R (acyanotic)

Answer 2

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)

Question 3

ASD

Answer 3

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.

Question 4

PDA

Answer 4

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!!!

Question 5

congenital HD w/out shunt

Answer 5

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.

Question 6

pulmo stenosis

Answer 6

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.

Question 7

juvenile rheumatoid arhtirtis

Answer 7

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,

Question 8

dx of JRA

Answer 8

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

Question 9

SLE

Answer 9

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.

Question 10

dermatomyosicites

Answer 10

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.

Question 11

scleroderma-

Answer 11

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.

Question 12

PAN

Answer 12

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.

Question 13

mixed CT disease

Answer 13

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.

Question 14

acute rhemautic fever

Answer 14

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.

Question 15

vasculitis syndromes:

Answer 15

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).

Question 16

heoch -schonlein purpura (HSP)

Answer 16

IgA vasculitis affecting lower limb, buttocks.
Ia deposit in BV.
GI, kidney, skin affected, triggered via GI or URTI’s (under 10).
purpura (100%) , 70% arthralgia)
15% in kidney issue.
abdominal pain.
leaking of blood, inflammation-> SV -> red .[purple non blanching rash under skin (palpable).
skin ulcer-> necrosis occurs.
features: knee. ankle arthritis.
abdominal (50%) if preceeded gastroenteritis infection-> Intussception bowel infarction, hemorrhage.
igA nephritis, nephrotic syndrome, haematuria, proteinuria.
dx: sepsis, blood culture, CRP, albumin, renal profile, FBC.
criteria: biopsy, arthritis, urine dipstick, diffuse abdo pain
mng: rest, supportive, hydration. steroids ??? if severe only!

Question 17

kawaski disease

Answer 17

acute systemic vasculitis affecting small-medium sized arteries (coronary-> aneurysm). acquired HD.
favourable prognosis following trx. idiopathic: genetic pre-disposed, infectious trigger. IgA plasma cells in affected tissue,
transition of matrix metalloproteinase in collage/ elastin degradation-> aneurysm.
S+ S: fever > 5 days without resolving with paracetamol. cardiac abnormality in subacute stage (2-4 wks) -> MI / sudden cardiac death.
CREAM (exudative bilateral conjuctiviative), polymorphous in trunk/ groin, edema/ erythema, adenopathy. mucosal involvement (strawberry tongue).
dx: lab values, anemia, clinical diagnosis 4/5 criteria met. if les than atypical.
trx: iv immunogloblim, aspirin (4-6 wks), infliximab, anti-tnf.
foliw up depends on Z score (evaluate CA abnormality diameter).
<2 dilution only.
>10: large/giant aneursm (LMH & warfarin heparin), stress echo.
antiplatelet- clopidogrel +aspirin.
beta blockers, imaging, avoid high intensity activity.

Question 18

joint disease

Answer 18

nocturnal awaking w/ leg pain. child will have anaemia, irritability, bruising and infections (leukaemia, growing pain, lymphoma, neuroblastoma, osteoid osteoma).
clunk hip movement-> limp.
(asymmetrical upper leg skin folds, limited hip abduction (development dysplasia of the hip).
sudden limb transient synovitis of the hip, perthes disease).
hip pain in adolescent boy-> skipped femoral epiphysis.
mild -> severe affecting everyday movmne.t
injury / disease to tendon. ligament, cartilage bursa can affect it.
examples: bone infection, bone cancer, broken bone, ankylosis spondylitis, lupus, bursitis.
eg. osteomyelitis, complex regional syndrome,

Question 19

UTI/ pyelonephritis

Answer 19

causes: female, catheteriation trauma (prtoeus, pseudomonas), obstructive stones, VUR, constopation,, habitual postphoning of voiding.
KES (klebsiella, ecoli, enterobacter).
C.F: neonates- diffused sx (vomiting, fever, jaundice, poor weight gain, diarrohea.
infants: crying/ straining during urination, dribbling, abnormal urinary stream, palpable bladder. convulsion, fever, hematuria.
difficult to distinguish pyel and UTI. all cases r treated as pyelo.
complicated: fever, persistent V (infants), dehydration, increased creatine, tender renal angle.
uncomplicated: low grade, dysuria, polyuria, urgency.
urinalysis) bacteruria, microscope visualisation nitrates
trx: based on age and whether its complicated (< 3 months, complicated, PO Ab-> 3rd generation cephalosporin, aminoglycoside 1 dose 10-14 days).
> 3 months, simple: oral Ab (7-10 days)
adolescent 72 hrs.
no trx if asx.

Question 20

imaging studies for RS

Answer 20

renal US
mCU (micturating cystourothrography)
DMSA scan.
prevention: long term prophylaxis: cephalexin. renal insufficiency and more than 3 months (nitrofurantoin).

Question 21

acute and chronic glomerulonephritis

Answer 21

causes->hematuria, proteinuria, RBC casts, RF. cystitis -> wbc casts (ddx)
, hypertension (abnormal renin secretion)
acute: RBC cast. primary: kidney r secondary (systemic)
chronic: no casts.
acute- primary PSGN, infectious, igA nephropathy (Berger’s), mpgn.
secondary-(bleeds)> PAN, good pasture (BM inflammation)., HUS, subacute endocarditis (flick clot).hsp

chronic - primary: MPGN, membranous nephropathy, focal glomerulosclerosis, mesangial proliferative nephritis.
dx: biopsy if unclear.
target therapy for underlying diseaes
c3 and c4 distinguish ddx.
low c3 ad normal c4: psgn.
low c4: shunt nephritis, endocarditis, sle.
prognosis-> excellent.
trx:supportive.

Question 22

nephrotic syndrome

Answer 22

heavy proteinuria >40 mg/m2. hypoalbuminemia (2.5 gm.dl), edema (pitting) and hyperlipidaemia) cholesterol 250 mg/l.
primary/ idiopathic- MCS.
significant glomerular histological lesion- mesangial proliferation fsgs.
secondary-infection (HIV, HCV, HBV).
autoimmune disorder, allergy, drugs.
congenital- torch. finish type (common).
podocute damage in mcs- increased permeability.
loss of negatively charged or mutation in protein.
loss of albumin-> lipid/ protein synthesis increase/
hypovolemia/ edema -> decrease renal perfusion-> release adh (edema).
2-5 years.
periorbital/ face puffy), genital and regress. spread downwards to limbs.
fatigue / lethargy, anorexia, bw increase, git, oliguria, PE, gross hematuria, dyspnea, bP.
investigate: urinalysis, blood tests, proteinuria, 24 hr > 40 mg.
protein/ creatine. culture/ sensitivity (exclude UTI).

c3 and c4 abnormal if nmcs.
renal biopsy idnciated if htn, hematuria, outside range age, remission, renal insufficiency.
supprotive:low salt and high protein, diuretics.
specific : non minimal can be ace -I (rampiri)
predniskg.
one/ (initial 2 mg/ x6 wks. up till 3 months.
early vs late vs steroid resistant.
remisson-> protein free 3 days.
replapse 3/4+ proteins or steroid dependent.
levamitisine or cyclophsopamide (more SE). SE reverse after cesssation.

Question 23

congenital anomlaie of renal system

Answer 23

bilaterlal/ unilateral agenesis (bilateral not comaptible w/ life, associated defects).
unilateral _> hypertrophic compensation.
hydroamniosis -> potter facies.
hypoplasia-> congeital or acquired causes.
congenital: no sccarring.
biateral and smal kidneys.
ectopic kidney: above pelvic brim, ureter kinked-> obstrution.
iv pyelourethrography visualize with contrast kidneys tubule.
horshe shoe kidney (turners+ edwards.
)lower pole fusion common. ureteral obstruction-> presure-> hydronephorsis (stone and improper drainage).
multicsystic renal dysplasia-> metanephric differentiation abnormal.mutlicsytic, enlarged, irreguala.

Question 24

febrile seizures

Answer 24

benign and generalized.
< 12 months then have 50% to have 1.
febrile during or after seizure.
last for 5 minute with no brain damage.
cytokine il-1 released-> inflammatory.
6 months- 6 years of age.
only 1 seizure in 24 hrs (rule can be violated)
fhx.
management: call help if worried.
cyanotic (ARD’’s) , if longer than 5 min, time seizure duration. Anti-pyretic don’t make a difference