Immunoodeficiencies Flashcards

Question

Grip: 3 month - ? 6 month - ? 10 month - ? Bricks 1.5yrs - ? bricks 2yrs - ? bricks 3yrs - ? bricks ``` Drawing ? - 2yrs ? - 3yrs ? - 4yrs ? - 5yr ```

Answer 1

3 month - reaches 6 month - palmar grip/pass 10 month - pincer 1.5yrs - 3 bricks 2yrs - 6 bricks 3yrs - 9 bricks Line - 2yrs Circle - 3yrs Cross - 4yrs Square - 5yr

Answer 2

< 1 year 300 mg 1 - 10 years 600 mg > 10 years 1200 mg

Answer 3

Down trisomy 21 30 <1/1000 35 1/(1000/3) = approx 1/270 40 1/(270/3) = approx 1/90 45 1/(90/3) = approx 1/30 If the trisomy 21 is a result of a TRANSLOCATION the risk is much HIGHER Which genetic group of diseases show genetic ANTICIPATION ? ``` fraGile x (CGG) HuntingtAn (CAG) myTonic dysTrophy (CTG) ``` EARLIER onset in successive generations Down's likely in which genetic kind of issue?

Answer 4

``` Gingival hyperplasia: , ciclosporin, CCBs AML Phenytoin ______ ``` Duodenal Atresia -double bubble Biliary Atresia -CB>UCB #backlogging ______ RAD vs LAD A(R>S @ V1) - WWPW - B (S>>>R + Tinvert)+ VT AAAArm switch/dextrocardia RRRRVH - LVH Lat (circumflex) - MMMMI - Inf (RCA) TTTTall thin = RAD Left post fasicle - HHHHemiblock - left ant fasicle/(bifasicular) p176 ECG John Hampton book

Answer 5

Hygiene + Mebendazole @ >6 months or older for EVERYONE Ix: Sellotape to the perianal area —> MCS

Answer 6

-hot young hijabi @ WINTER prone to get? major risk factors for SIDS are: Sleep prone Smoke parental Share bed/head cover So hot, so young - hyperthermia/premature -hot young hijabi @ WINTER prone to get?

Answer 7

Trinucleotide repeat disorder ``` fraGile x (CGG) HuntingtAn (CAG) myTonic dysTrophy (CTG) ``` Friedreich's ataxia* (GAA) spinocerebellar ataxia spinobulbar muscular atrophy dentatorubral pallidoluysian atrophy Down's likely in which genetic kind of issue? TRANSLOCATION

Answer 8

``` Age Milestone 3 months Quietens to parents voice Turns towards sound Squeals ``` 6 months Double syllables 'adah', 'erleh' 9 months Says 'mama' and 'dada' Understands 'no' 12 months Knows and responds to own name 12-15 months Knows about 2-6 words (Refer at 18 months) Understands simple commands - 'give it to mummy'

Answer 9

Malabsorption Causes may be broadly divided into - biliary - pancreatic (pancreatic enzyme deficiency - intestinal (e.g. villous atrophy) Ai eg. syst sclerosis Cancer eg. lymphoma #BACTovergrowth

Answer 10

dexamethasone to the MOTHER!!!!!!!!!

Answer 11

Caffiene can be used as a RESP STIMULANT @newborn babies WEAN off ventilator Pulmonary hypertension -Sildefanil / ECMO Sildenafil CI -Nitrates/Nicorandil @ED Check: - Q10risk - f.Testost > FSH LH/Prolactin - ---ED + poor libido = psychogenic - ---ED + normal libido = likely vascular dx -viagra->vacuum

Answer 12

``` SPASm HEMIFACIAL/Blepharo SPASticity SWeating axilla SWallowing achalasia ___________ ``` S1 = AV valves mitral/tricuspid closing soft @Regurg loud @MS ``` S2 = Aortic/pul closing soft @ASten Loud @ -HTN, Hyperdymamic states, -ASD-PulHtn ``` Wide s2- delay RV empty -(PS; PAH{MRegurg severe}; RBBB) Paradox s2 -WPW-b, AS/LBBB, RVPacing, PDA Fixed s2 - ASD S4 = atria contract against STIFF ventricle HOCM/HTN ASten ``` S3 = diastolic filling of ventricle Const pericarditis - pericard knock, X+Y, X ✔️; Dilated CM, MRegug NORMAL<30y ____________ ``` 1st and 2nd degree: K+low; IHD; myocarditis; Beta-blockers; Athletes; Digoxin 3rd degree complete block: Fibrosis; AS; Surg Trauma; IHD/Congen ____________ Pericardial rub - pericarditis Pleural rub - pneumonia/PE Pericardial knock - C. Pericarditis ____________ LBBB=CM, HTN, AS, IHD RBBB=PE, ASD, Normal

Answer 13

Acrocyanosis Central cyanosis can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds 5g/dl The Nitrogen-Washout test (aka the Hyperoxia test) may be used to differentiate CARDIAC from NON-CARDIAC causes. The infant is given 100% O2 for 10 mins --> ABGs taken --> pO2 <15 kPa indicates cyanotic ConHD: (TAtr, TGa TOf ) Initial management of suspected cyanotic congenital heart disease = - Supportive - PGE1 = maintain PDA @ ductal-dependent ConHD Indomethacin PGE2i closes PDA

Answer 14

WPW A - left sided RAD = dom R wave @ V1 B - right sided LAD = no dom R wave @ V1 Assoc: MESH MVP, Ebstein anomaly, Secundum ASD, HOCM/HyperT Tx: radioFreq ablation of acc pathway FAPS ``` Avoid sotalol @AF cos it -prolongs refractory period @AVN -> -inc transmission rate through acc pathway -> -Inc vent rate = VF ____________ Infantile spasms EEG = Hyps-Arrythmia -babieeees <8m _________ ``` Infantile spasm --> lennox Gastaut EEG=slow spike < 5yrs __________ Benign Rolandic epilepsy EEG = Centrotemporal spikes -Older kids <12 years _______ Girl TEENS > boys - AM seizures - AM absences - sudden Myoclonic seizure - -----Juvenile Myoclonic Janz Syndrome

Answer 15

JIA - ADMIT URGENT -cos septic arth can present similarly!!!! ____________ Tamponade/ Severe asthma: - PAH, AR/ASD, High Left EDV AS _________ AR/PDA/ Incr requirement LVF _________ HOCM/Aortic valve Dx HOCM _________ J = hypothermia HyperCalcemia Widespread ST elevate = pericarditis _________ PR depression = most sensitive for pericarditis!!!!! pericardial knock = constr pericard _______. Collapsing pulse = AR/PDA/ Incr requirement Wide Pulse Pressure = PDA/3rd HB/AR Narrow Pulse Pressure = ASten

Answer 16

Whooping cough - AZITHRO / CLARITHRO if the onset of cough is within the previous 21 days School exclusion 2 days after ABx 21 days since syx, if NO ABx

Answer 17

A child aged < 3 months with a fever > 38ºC should be assessed as high risk of serious illness If amber: safety-net or refer The 2007 NICE Feverish illness in children guidelines introduced a 'traffic light' system for risk stratification of children under the age of 5 years presenting with a fever. These guidelines were later modified in a 2013 update. Consider a diagnosis of pneumonia if the child has: ``` high fever (over 39°C) and/or persistently focal crackles. ```

Answer 18

Romano Ward, KCN(Q1+H2) fucked K channels Jervell Nielsen Brugada = tachy-arrhythmias, sudden cardiac death. ICD!! Gene SCN5A mutation -> fucked Na Channel Not FAPS - SSRI/TCA; APsych; Li - ABx = MACROLIDES - Low Mg K Ca/ Low Temp HypoThermia - Typ>>>>Atyp CASQ2 = calsequestrin fucked -> Ca can't bind -> Catecholaminergic Polymorphic VT (CPVT) and RYR2 = ryanodine receptor -> CPVT also _____________ Labial adhesions: fusion of the labia minora in the midline. girls between the ages of 3 months - 3 years resolve @ around PUBERTY 1. Conservative tx 2. UTI: Trimeth + Oest cream 3. Fail: Surgery

Answer 19

1. AR dx = 'METabolic' - exceptions: inherited ataxias 2. AD dx = 'STRUCTural' - exceptions: Gilbert's, HyperLipidaemia type 2 *MA-SHg Ar: Met + Ataxias AD: Struct + Hyperlipidemia 2/Gilbert _________________ ``` XLr = FAVRett Fragile X Alport Vit D Resistance #RicketsOM Rett Syndr Duchenne ``` G6PH Haemophilia f8+9 Kallowwwman

Answer 20

sickle cell crisis to hospital unless they are: A well adult w/ mild/mod PAIN and TEMP < 38°C or less. A well child w/ mild/mod PAIN and No Incr TEMP.

Answer 21

Growing pains are a common complaint in children aged 3-12 years. These usually present with children complaining of pains in their legs. When seeing children who are presenting with these symptoms it is important to check that there are no 'red flags' Osteosarcoma is rare, but it is an important diagnosis to rule out. Features of osteosarcoma include an unexplained lump, unexplained bone pain or unexplained swelling. Juvenile rheumatoid arthritis usually presents as fever, rash and symmetrical joint pain and swelling. OSTEOCHONDRITIS DISSECANS is a joint disorder in which cracks form in the articular cartilage and underlying subchondral bone. This results in joint pain, locking and swelling. Bipartite patella is a condition in which the kneecap is formed of 2 separate bones -it is usually asymptomatic. Chondromalacia patellae

Answer 22

Aspirin Antiplatelet - inhibits thromboxane A2 production Clopidogrel Antiplatelet - inhibits ADP + plt receptor binding Enox/fonda = Activates AT3 -> -stop f8-12a Bivalirudin Reversible DTi Abciximab, eptifibatide, tirofiban GP2b/3a receptor blockers ____________ 5 rescue breaths check circ 15:2

Answer 23

CROUP -steeple Management DONa Dexamethasone / Pred PO O2 HF Nebulised Adr ``` 1963 BC: Bronchiolitis RSV seen what age? 1m-9m Croup PIV seen what age? 6m-3y ________ Epiglottitis -THUMBprint sign @lat-view ```

Answer 24

AStenosis -S4=HOCM/HTN/ASten Asyx = OBSERVE Asyx >40/50mmHg + LV sys dx = SURG Syx = valve replacement -> balloon valvuloplasty Ax Aortic stenosis: <65 - bicuspid aortic valve >65 - calcification Rheumatic Fever LVH= deep S @V1-2; tall R @V5-6 -inverted T @V5-6 (I, II, VL) RVH= RAD+tall R @V1 -inverted T @V1-2, I II, aVF wave inversion in the leads looking at the right ventricle (T wave inversion is normal in lead Vl , and may be normal in lead V2, but in white adults is abnormal in lead V3) ________________ ``` For bioprosthetic valve: Inc risk of calcification >65 get aortic one >70 get mitral one Long term AC not needed, give aspirin ``` For mechanical valve: Inc risk of thrombosis Give warfarin + aspirin if IHD. ____________ NICE recommend -TCI: Tailored Clinical Intervention if BMI @91st centile or above -ComorbiditiesAssx if BMI @98th centile or above RFs: Females, Asians, Tall Endo: Cushing's, HypoT, GH deficiency Genetic: Down's Prader-Willi

Answer 25

Sotos syndrome - XS Phys Growth - MacroDOLICHOcephaly: head>expected - POINTED CHIN Fragile X -big brain, big balls, long face/ears

Answer 26

Migraine!!!! Ibuprofen first line!!! Triptan >12yrs

Answer 27

Vit AAAAAAAAAAAAA!!!

Answer 28

Day 5-9 ``` CF HypoT Maple Syrup PKU Sickle Cell ``` MCADD/MSUD GA1/HCystUria/IVA ____________ Fair, blue eyes, retarded = PKU Sweaty feet = IVA movement dx - GA1 Hartnup Dx -High prot diet

Answer 29

Epsteins pearl Resolve spontaneously

Answer 30

BAD!!!!!! Hemolysis (rhesus/ABO) Hereditary Spherocytosis ADom G6PD XLr After 2 days is legit. Usually breastfeeding After 2 weeks is NOT legit: CB > UCB - ?Biliary atresia (UCB -> CB then cos of atresia gets backlogged into blood) FBC U+E LFT / TFT / Coombs / Urine MCS / Sugar

Answer 31

Breastfed babies are at risk of vitamin K deficiency

Answer 32

CARED 1. Cause: Constipation, DM, UTI 2. Advise: fluid intake, diet and toileting 3. Reward systems: Star charts for agreed behaviour > dry nights 4. Enuresis alarm <7yr 5. Desmopressin >7yr @EnuresisAlarm fail/unacceptable alarm VS drug treatment - depending on age/maturity/abilities ______________ ADHD inattention/hyperactive Tx?? -What age give DRUGS? ``` WW10W Education Inattention/Hyperactive Refer paeds/cahms Drugs 5+yrs: Methylphenidate: n+v, AP, gord LisDexAmfetaminECG DexAmfetaminECG ```

Answer 33

Thrombotic crises | -sickle cell

Answer 34

Sequestration crises

Answer 35

Aplastic crises

Answer 36

Bronchiolitis - RSV - Ix: NasoPharyngeal Aspirate - Tx: SUPPORTIVE Tx!!!!!!!!!!!!!!!!!! Refer! Palivizumab 1963 BC: Bronchiolitis RSV seen what age? 1m-9m Croup PIV seen what age? 6m-3y _____ Random but... CXR steeple sign = Croup DONa LatXR = Thumb sign = Epiglittitis HiB

Answer 37

HAD: HypoT/Hurler mucopolysaccharidosis ACromegaly Amyloidosis DuchenneMD Gower's sign: child use ARMS from: squat -> stand

Answer 38

PBC Bili > 100 Recurrent cholangitis Refractory Itching Ascites

Answer 39

HBsssssssAg: acute/chronic>6m dx --- ALTTTT@ ACTIVE #CARRIER/INFECTIOUS HbsAg Anti-HBcccccc: prev/current --- IgMMM @ ACUTE -> IgGGG chronic HBV-DDDDDDDDDDNA: acute/chronic>6m (high lvls assoc with HCC) HBeeeeAg infectivity marker --> anti HBeeeee @ resolving anti-HBsss POS only = IMMUNE - vaccine anti-HBsss POS, anti-HBccc/eee POS = IMMUNE prev hep B : anti-HBc only: Resolved/Acute resolving/Chronic low level / False positive > 100 booster at 5 years 10 - 100 - one more vaccine dose + test @immunocomp < 10 Non-responder - 3 doses again + testing SCDE - - > @fail = HBIg

Answer 40

Bronzing skin Cardiomyopathy #REVERSIBLE Rest IRReversible

Answer 41

Management depends on severity - mild-mod: baby shampoo/oils - severe: top 1% mild HYDROcort

Answer 42

Pure tone audiometry is done at school ENTRY in most areas of the UK

Answer 43

Laryngomalacia CONGENTIAL cause of stridor _________ Inhaled Foreign Body

Answer 44

1-Ejections* - turb OUTFLOW tract 2-Venous - turb INFLOW venous tract 3- stiLLSe - LLSE low pitched _________ *EJECTION: Pulmonary=soft blowing/Aortic=short Buzzing -Assoc w/Valsalva #3 innocent murmurs

Answer 45

AOM: MUSIC FBI PUNK Admit @ - Mastoiditis/Meningitis - Unwell systemically#<3m >38deg - Sinus Thrombosis - IC Abscess - CN 7 paralysis ABx @: - Fail tx / 4/+ days - Bilat @<2yr - IC - Perf /Discharge - Unwell - Kidney liver heart etc dx Tx: Analgesia + Amox/Clari- > worsen but NO MUSIC = Co-Amox ______ Sinusitis <10d Syx - NO ABx Sinusitis >10d Syx: -nasal c.sted ABx only @ Cx: - Systemic dx - Peri-orbital/orbital cellulitis - Ophthalmoplegia - Sub-periosteal abscess - Meningitis Tx = PMP-V -> Co-Amox/ Doxy @allergy __________ 1. Fever > 38/ 3-14y 2. Purulent exudate Admit <3d 4. No cough/Coryza Inflamed tonsils 6. C.LNopathy FeverPAIN 4/5 = PMP-V Centor 3/4 = PMP-V ________ ``` -Persistent OME IN BETWEEN episodes due to EUSTACHAIN BLOCKAGE -Persistent C.LNopathy -Epistaxis Tx = 2ww NPCancer!! ``` ``` If recurrent AOM @... -Unexplained -Adult -Downs/Cleft #Craniofacial dx --> Refer __________ ``` OME: ASAP refer @Downs/Cleft / Cholesteatoma/ Hearing-loss WW <12w (± Auto-inflation @older kids): -2 PTAudio+Tympano-metries 12w apart - OM -> perf = Amox - Hearing-aid(/Surg* @Down's/Cleft) @BILAT -OME - Auto-inflation: CI @URTI/pain - Myringotomy + grommet ± Addenoidectomy* Grommets usually stop functioning after 10m CSOM >2w = ENT -Cleaning, ABx, Top c.steds Cholesteatoma = ENT -CT + Audiology _________ ________ ``` Allergic Rhinitis: Mild-Mod: AHist > MastCellStab 1. AHist: - a. Intranasal Azelastine > - b. Oral AHist > ``` 2. MastCellStab-NaCromoGlic Mod-Severe/ Mild fail: -Intranasal Csted Chronic Bilat rhino-sinusitis? -saline nasal douches -Chronic UNILAT rhino-sinusitis = 2WW!!! ________ -PERENNIAL all year - house dust mites -seasonal hay-fever - spring/summer* -Occupational ________ 1. House dust mites - all the time/ALLyear #PERENNIAL 2. Pollens:* - Tree = spring - Grass = early summer - Weed = spring/summer/autumn 3. Occupational _________ _________ Otitis Externa 1. -Otomise -> -Fluclox/Erythro 2. -REFER + Cipro @malig otitis ext -> 3. Tx fail = ?dermatitis/?fungal -top c.sted/top a.fungal

Answer 46

Ear wax imapction Perf Tymp Memb ______ AOM: earache/TUGGING/rubbing/crying/restlessness ear reveals a BULGING tympanic membrane -H.Flu !!! OME (glue ear) — @otoscope = effusion and air fluid levels/bubbles w/ normal/RETRACTED tympanic membrane landmarks #conductive hearing loss. speech and language delay, behavioural or balance problems CSOM — 2 WEEKS!!!! persistent inflammation and PERF of the tympanic membrane with discharge Myringitis-bullous -mycoplasma -erythema/injection of tymp memb _________ Otitis Externa 1. -Otomise -> -Fluclox/Erythro 2. -REFER + Cipro @malig otitis ext -> 3. Tx fail = ?dermatitis/?fungal -top c.sted/top a.fungal Viral conjunctivitis _________ Cholesteatoma -pars FLACCIDA Tympanosclerosis _________ Allergic Rhinitis

Answer 47

Bastards: ``` Acoustic neuroma: #NF2 CN 5 7 8 affected -corneal reflex dx V1 -facial nerve palsy - CN4 -sensorineural vertigo tinnitus CN8 ``` MRI cerebello-pont angle -> Surg ________ Most common salivary gland tumour - parotid 80% I--> most common paroid tumour = Pleomorphic Adenoma > Warthin's tumour __________ ``` Recurrent unilat pain/swelling @EATING -submandible = Wharton -@face-side = Stenson @parotid -infected = Ludwig angina - ivdu floor of mouth dx ___________ ``` Tonsilar SCC is associated with HPV infection Audiogram: -if ONE ear low than other AND -Bone > Air Dx = MIXED hearing loss Presbycusis - Sensori A>B - HIGH-freq -B/L Otoscloersis #flamingo-schwartz -Conductive B>A - LOW-frew -B/L Hearing Aid, Na Flouride, Stapedectomy Low libido + ED -> Psycho-Somatic ED Ix -morning Testost > FSH/LH/Prolactin Normal libido + ED -> Organic cause... need to Ix (usualy vascular dx) B P P V = Vertical nystag Vestib = horizontal nysag -Still going on -> Vestib REHAB exercises!!!! Aspirin + NSAIDs taken in HIGH doses can cause tinnitus ``` UTI 4w Biopsy 6w Ex 48hr Ejac 48hr DRE 7d ``` Perf Tym Memb -NO infectoin -hx of barotrauma WW 6-8 weeks Post-tonsillectomy haemorrhages should be assessed by ENT Primary haemorrhage WITHIN HOURS hours after tonsillectomy = immediate RETURN 2 theatre Haemorrhage 5-10 days AFTER tonsillectomy = Wound infection -Tx = ABx AOM pathogen = H. Flu Branchial cyst: - benign, lateral, UNI-lateral neck mass - acellular CHOLESTEROL crystals Top decongestants for prolonged periods = TachyPhylaxis Prostate Cancer: RT risk = COLOrectal cancer ``` Fluid AROUND testicle #CANNOT FEEL testes -TRANSILLUMINATES Dx = HYDROCELE _____________ ``` Venous Sinus Thrombosis = LMWH -5d-> Warf 2-3 Art Diss = AP/AC Prosthetic valve --> stroke AND ICH risk --> Stop AC, Start AP ``` HR bleed (surg) + HR stroke (AF/prev stroke) --> Stop AC, Start LMWH ``` Stable CVD + AF --> Stop AP, Start AC Isch stroke --> AF = Asp 300 mg 2w --> AC

Answer 48

The Green Book recommends allowing: 3 months between doses to maximise the response rate. if > 10 years - period of 1 month is adequate In an urgent situation (e.g. an outbreak) then a 'shorter period of 1 month' can be used in younger children.

Answer 49

Intermittent squint in newborns <3m NORMAL Corneal light/Cover test. Eye-patches--> refer @older kids - refer ASAP cos not legit ____________ Bow legs in a child < 3yr = NORMAL Bow legs Resolves by 4 y/o Sitting without support = achieved around 7-8 months, refer if still not achieved by 12 months

Answer 50

Airway suction should not be performed unless there is thick meconium causing obstruction Airway suction can cause reflex bradycardia* in babies. CPR should only be commenced at a HR < 60 bpm. @ no signs of breathing due to fluid @ lungs give 5 breaths via a 250 ml bag via face mask. Paeds ALS - 5RescueBreaths - Circ signs? - 15:2 *reflex TACHY @ SA DHP eg Nifedipine

Answer 51

1. AR 2. AR 3. XLr - f8/9 / Ar - f10 4. 1+2=AD ; 3=Ar 5. XLr MANOR MAFIA 6. AD SE3A @HETERO (XD Xd) female carrier: Each male child has 50% chance of being affected Each female child has a 50% chance of being a carrier. Male CANNOT pass to male Male --> ALL female carrier Female --> 50% Male affected / 50% femalr carrier Turner 45 XO female - wide neck/nipples Klein 47 XXY male - tits Kall XLr male > female - anosmia

Answer 52

Exclusions 1 day after commencing antibiotics Scarlet fever 2 days after commencing antibiotics Azith/Clari -or 21 days from onset of symptoms if no antibiotics ) Whooping cough Until lesions are Crusted and Healed, OR 2 days after commencing antibiotic treatment - Impetigo _____________ 4 days from RASH onset - Measles/Rubella 5 days from onset of SWOLLENglands-Mumps All lesions crusted over Chickenpox* Until symptoms have settled for 48 hours D+V __________ Until treated Scabies Until recovered Influenza(egg allergyCI)

Answer 53

Give according to CHRONOLOGIC age ___________ 3. CMV SEEEE-MV Sensorineural SMALL brain / plts sensorineural = cmv + rubella - ganciclovir ________________ 4. Toxo -spiramycin _______ 5. Rubella - ears, eyes, heart - PDA

Answer 54

commonest cause of F2T in UK = SOCIAL issues __________ Sepsissssssssssssssssssssssss COngen: HypoT/Down's-Prader Wili NEuro: SMA Werdnig Hoffman #WATFR ________ Congen HypoT ______ ``` GH deficiency - growth hormone: -immature face features -smaller than rest -INUSLIN TOL TEST + MRI for tumour _______ ``` CDiP: Constitutional Delay in Puberty - absence of secondary sex characteristics - delayed BONE age - presnet LATER than GH def

Answer 55

Resp Distress @neonate -GFR>60 resp rate _______________ - c-section --> - lung FLUID clearance DELAY #amniotic #vag-deliveryUSUALLYclears --> - TTN PREM / low BW --> surfactant def --> delivery --> RDS --> prog WORSE w/out tx --> Alv collapse @EXP -> ?Px Thick meconium-stained amniotic baby is CYANOSED and TACHYPNOEIC w/ chest wall retraction. CXR shows patchy infiltrations and atelectasis (slightly collapsed cos thick meconium!!!!) C-section -> TTN PREM -> Surf Def -> RDS

Answer 56

1. PBC - AMA/SMA IgM 2. Autoimmune hepatitis - ANA/SMA LKM1kids - IgG 3. PSC 4. PBC liver transplant @: - Bili >100 - Recurrent cholangitis - Refractory itching - Ascities ``` 5. Kolestyramine for ITCH Usda #FIRST-LINE BASTARD!!!!! Transplant ADEK MONITOR AFP LFT USS Stop Smoke ``` 6. PBC: HCC PSC: Cholangiocarcinoma/Colorectal/UCC

Answer 57

Common congenital heart disease cyanotic: TGA most @ birth, ToF overall, TrAtresia acyanotic: VSD most common cause Downs - A-VSD

Answer 58

Irritant dermatitis: Flexure Creases SPARED - irritant effect of piss/poo Candida: Flexure Creases AFFECTED w/ satellite lesions Seborrhoeic dermatitis: Erythematous rash with flakes. May be coexistent scalp rash disposable nappy Expose skin / Emollients Barrier METONIUM / Steroid imidazole @ candida Baby oil/shampoo Hydrcort

Answer 59

CARED 1. Cause: Constipation, DM, UTI 2. Advise: fluid intake, diet and toileting 3. Reward systems: Star charts for agreed behaviour > dry nights 4. Enuresis alarm <7yr 5. Desmopressin >7yr @EnuresisAlarm fail/unacceptable alarm VS drug treatment - depending on age/maturity/abilities ______________ ADHD inattention/hyperactive Tx?? -What age give DRUGS? ``` WW10W Education Inattention/Hyperactive Refer paeds/cahms Drugs 5+yrs: Methylphenidate: n+v, AP, gord LisDexAmfetaminECG DexAmfetaminECG ```

Answer 60

Sandifer Syndrome _______ Duodenal atresia -Duodenoduodenostomy Jej/ileal atresia Meconium ileus -surg Decomp / resection @serosal dx ______________ Malrotation with volvulus -Ladds procedure NEC Necrotizing Enterocolitis _________ iNTUSSUSCEPTION - Tx = barium enema - surgery @peritonism

Answer 61

Retractile testes Nosepicking > FOREIGN BODY insertion - pressure necrosis and - secondary sinusitis (cos its blocked) - covered in minerals eg Mg PO4 Ca

Answer 62

``` Congenital inguinal hernia – paediatric surgery ASAP incarceration risk <6w - surg <2d <6m - surg <2w <6y - surg <2m _____________ ``` Infanta UMBILICAL hernia No tx - resolve <3yrs -Assoc with HypoT !!! - If syx/ large = Surg @2-3yr - If Asyx+Small = ?Surg @4-5yr

Answer 63

Hirchsprungs - ganglionic dx Washout Rectal - > PullThrough AnoRectal

Answer 64

Cryptorchidism undescended testicle OrchidoPexySexySixy..= 6+m OrchidecTWOmy = 2+ yr

Answer 65

Live vaccine <4w Ig tx / 3m Preg avoid @MMR<4w Preg avoid @MMR<4w IC Neomycin allergy EGG ALLERGY @IntraNasal flu vaccine

Answer 66

- Chediak higashi - PAIN - Wiskott Aldrich - TAME - Bruton X-linked AGammaGlob - CV-ID - -Bronchiectasis/Ai dx/Lymphoma/SinoPul infections -SCID - Sotos - -head>expected = macrodOlichOcephaly - Fragile X - Cri du chat

Answer 67

Retractile testis _______ Duodenal Atresia

Answer 68

Erythema Toxicum Neonatorum infantile eczema

Answer 69

NAI | -bruises on bony prominces e.g. elbow knees is normal

Answer 70

Neonate <28 days Infant 1-12 months <1yr Toddler 1-3 years <3yr Pre-schooler 3-5 years <5yr School age 5-12 years <12yr Adolescent >13 years 13/+

Answer 71

2m: DTaP *6in1, MenB, Rota 3m: DTaP *6in1, PCV, Rota 4m: DTaP *6in1, MenB 12m: MMR HiBBB-MenCCC-MenBBB PCCCV 3yr4m: MMR *4in1 DTaP 12-13yr - HPV ``` 13-18yr '3-in-1' Diphtheria, Tetanus Polio Men ACWY ________________ ``` 6in1: Diphtheria, Tetanus, Pertussis, Polio, Hib, Hep B 4in1: Diphtheria, Tetanus, Pertussis, Polio _____________ Mother: - BabyBond - PPH/BCancer Reduce Kid: i-AIRD - Infections - Allergy/ IBD / RA / DM 1

Answer 72

12-13y = HPV 13-18y = - Diphtheria, Pertussis - Polio - MenACWY

Answer 73

EJECTION MidSys@RUSE -Aortic Stenosis Sys-mid C-D EJECTION MSys@LUSE= -i-PATH -innocent - PS, ASD-fixedsplit, ToF, HOCM S4, !!!! LUSE diastolic murmur: AR: early, Austin Flint #rumbling #MussetAustinflintDariuszCorriganAT-PistolAQuincke PR: Graham Steel Late MESys @ back -Coarctation Carcinoid -> TR/PS Cont machine-like murmur = PDA ___________ PSys LLSE = - TR = louder @insp #incrVenReturn - VSD=harsh Dias LLSE = TSten PSys = - MR* blowing high = radiate 2 axilla - MP(actually is late sys) Late Diastolic = MS -opening snap + Rumbling ____________ *S3= CPercardKnock, DCM, MR, Norm<30

Answer 74

Oligohydramnios < 500ml @ T3 AFI < 5th centile Renal agenesis / ACEi IUGR PROM/Pre-Ecl/Post-term>42w

Answer 75

PDA= pul art + aorta connection inutero, baby gets O2 from mum Doesn't need lungs #pul HTN -> R->L shunt -i.e. need it go through PDA after born, Pul HTN gone -> blood go to lung for oxygenation #dont need PDA ``` If persists #uncorrected, you get: L->R shunt -> PAH + RVH -> R->L shunt @Eisenmenger --> -murmur = disappears --> infant = CYANOTIC, not shocked ``` Pulse = Bounding + COLLAPSING* Murmur = continuous MACHINE -Wide pulse pressure Tx = Indomethacin closes PDA!! Prostaglandins keeps it open @ TGA to allow some oxygenation before surgical fixing *Collapsing pulse = AR/PDA/ Incr requirement

Answer 76

``` Eisenmenger: If persists #uncorrected, you get: L->R shunt -> PAH + RVH -> R->L shunt @Eisenmenger --> -murmur = DISAPPEARS --> infant = CYANOTIC #not shocked ``` Ax = VSD, ASD, PDA. _____________ ASD: RBBB+RAD = secundum dx -EMBOLUS SHOOT OFF -> STROKE!!!!!! RBBB+LAD = primum dx -prime lad __________ Coarctation - Aorta NARROW near PDA -> - HTN in Bracioceph + LSubclavian - CXR = collats eroding ribs -> notched ribs

Answer 77

MR - Pansys blowing high pitched -> Axilla MVP = Pansys + EMSyst click MS -opening snap + Rumbling ________ ``` Collapsing pulse = AR/PDA/ Incr requirement Wide Pulse Pressure = AR/PDA/ 3rdHB -Narrow Pulse Pressure = ASten -slow rising pulse = ASten _________ ``` Pansystolic @LLSE - louder @insp #incrVenReturn=TR - harsh=VSD

Answer 78

LVH: R>25mm @V5+6 Inverted T @ V5+6, 1, VL LBBB+LAD RVH: R tall @ V1 Inverted T @ V1+2, RBBB+RAD Bifid/Broad P-mitrale +/- AF = LAH -MS -> LAH Peaked P-pulmonale #RAH -TS>RVH(PS/PAH) As per John Hampton p112

Answer 79

Codeine to PO morphine /10 PO morphine = to... SC moprhine /2 OXYCOD PO /2 SC diamorphine /3 IV moprhine /3 OXYCOD SC /4 Alcohol units? 4.BSA.kg = half in 8hr, half in 16hr 20 cig/day/1 Yr 100ml/kg/d @1st 10kg 50ml/kg/d @2nd 10kg 20ml/kg/d @rest weight.. (Na+K) - (Cl + HCO3) 2(Na+K) + (BM+Urea)

Answer 80

Monoplegia - 1 limb Hemiplegia - Unilat 2 limbs Quadriplegic - 4 limbs -Paraplegia - Bilat LOWER limbs ACA MCA PCA* L>UL ; UL>L < -- HemiParesis ........Aphasia -- > Agnosia ........Sensory ....HomoHAnopia -- > Mac-Sparing *PCA - Weber Midbrain -Same CN3, opp HemiParesis -Agnosia -Macular sparing HomoHNopia ________________ Amaurosis fugax - Retinal/Ophthalmic Artery Locked in syndrome - Basilar Artery ____________ Pontine bleed - Absent < -- > horizontal eye-move - Miosis - Paralysis=Quadriplegia AICA: Lat Pont - Same FACE: PD/PT (paralysis/deaf // pain/temp) - Opp limb - Nystagmus - Ataxia PICA: Lat Med Wallenburg - same as above EXCEPT - paralysis and deafness ______________ Anterior Circulation Stroke: 3=TotalACS 2=PartialACS - Unilat sensory/motor FAL - Cog dx - VisuoSpatial/Dysphasia - HomoHNopia ``` LacACS Assoc w/ HTN 1 of: -Sensory -AtaxicHemiParesis -Motor PURELY _________ ``` 4-6-4 H: CN4 vertical nystagmus CN3 Ptosis, Dilated, Vertical nystagmus CN6 horizontal nystagmus Nystagmus: Central v Periph: central is: - B/L - Assoc sens/motor dx - Direction = multi / purely uni or rotatory ``` Brainstem Death: Coma unknown Ax Reversible ax excluded Sedation X Electrolytes fine ``` ``` Bronchial stim -> no cough Response to sound/Supra-Orb Pressure Occ-Vestib Reflex absent Corneal Reflex absent Disconnect ventilator 5-mins -> no resp support _______ ``` - Emotions = fear, agitation - Fluct Syx = worse @night, normal periods - GCS impaired - Hallucinations/Illusions/Delusions #perception

Answer 81

UTI tx? - Cath change @7/+d - ABx/Analgesia - MSU/ Dipstix - Preg = Tx NOW; ANC @GBS-agalactae Refer: - S+F dx - Atyp org - Recurrence/Persistence - CATHETER - Atyp org - S+F dx -TwoWW@ 45/+ + vHU 45/+ + vHU + (UTI + Tx fail) 60/+ nvHU + - dysuria / inc WCC - recurrent/persistence ``` IC/ Urology dx @prostatitis = REFER -Acute = cipro+CS --f/u=2d-> C+S result d/w @f/u -ABx accordingly -GUM @STD ``` Chronic = -Lactulose @pain-poo, Alpha-blocker, CBT/ADep, Trimeth ________ 45/+ + vHU: - w/ UTI + Tx fail - w/out UTI