Memorize

Question 1

What is age of consent for sex?

Answer 1

16 in canada
12-13 year old can have sex 2 years older
14-15 can upto 5 years older 
not authority
and not exploitation (until 18yo)

approx 2/3 youth have one partner only, and 1/3 of youth have had sex.
Pregnancy rates are delcining, and 50/50 do abortion. same rates post part depression, and 1/3 will have another babe within 2 years

Question 2

Birth control - OCP
Absolute CI
s/e

Also depo s/e

Answer 2

HTN -160/100
migraine wiht aura
DVT hx
liver cirrhosis/hepatitis, DM with vessel disease
s/e -NOT weight gain, (estrogen effect: nausea and h/a), breatkrhoguh bleeding (may not be high enough estrogen) , breast tender. Reduced PMS, ovarian cyst, acne, certain cancers.
Intrxn with anticonvulsants, abx OK

–
Depo - no inc DVT risk but amenorrhea, weight gain and reduced BMD, deprresion. USE vit D and Ca with it

Question 3

Diagnostic criteria for Anorexia nervosa and hospital admission criteria

Answer 3

1) restriction of energy intake leading to low weight relative to whats appropriate for developmental trajectory/age
2) intense fear of gaining weight and compensatory behaviours to prevent weight gain
3) Disturbance in the way in which one’s body weight or shape is experienced, undue influence of body weight or shape on self-evaluation, or persistent lack of recognition of the seriousness of the current low
body weight

ADMISSION - low HR 45/50, hypotension, electrolyte abn, orthostatic changes, ECG changes/abn, psych (SI, abrupt food refusal)

Question 4

diagnostic criteria for Bulimia

Answer 4

1) binge eating (lack of control of eating large amount + discreet amount of time)
2) compensatory behavior to prevent weight gain
3) occur 1x/w for 3 months min
4) self evaluation is based on weight/body shape
5) does not occur during AN

(if just bringing and no compensatory behav then its binging disorder)

Question 5

Define 1 key characteristic for age -
EARLY adolescence
MID adolescence
LATE adolescence

Answer 5

early (10-13yo) - concrete operation, self conscious about appearance, start of puberty

middle (14-16) - more conflict with parents, abstract thinking start, struggle for autonomy, sexual orientation questions. sense of immortality

late 17-20yo - idealism and absolutism, future oriented, emancipation complete, increased autonomy, focus on plannning and formation of stable relationships

Question 6

which tanner stage does

1) testis grow
2) scrotum enlarges
3) phallus length increase
4) scrotum darkens

Answer 6

which tanner stage does

1) testis grow – tanner 2
2) scrotum enlarges – tanner 3
3) phallus length increase – tanner 3
4) scrotum darkens – tanner 4

Question 7

for females which tanner stage does

1) breast bud
3) breast tissue beyonf areola
3) secondary mound
4) hair not on thigh only vagina

Answer 7

1) breast bud - tanner 2
3) breast tissue beyond areola - tanner 3
3) secondary mound - tanner 4
4) hair not on thigh only vagina - tanner 4

Question 8

what are four things to rule out before diagnosing PUBERTAL gynecomastia in males?

Answer 8

can f/u in 6 month if just pubertal (firm or rubber mass that is subareolar, with normal testis exam
COME

Cancer (thyroid, pit, adrenal)
Obesity
Medications (steroids, TCA, weed, ETOH)
Endocrinopathy (klinefelter, CAH)

Question 9

What are indications for tubes

Answer 9

1-recurrent AOM with middle ear effusions
2-bilateral OME>3 months with CHL
3-unilateral OME ?3 month with other issues (discomfort school perf)
40 others - complications like mastoiditis, lack of response, chronic retracted TM

Question 10

with TM perforation what is false

1) most heal iwthin 6 weeks
2) you cannot use ciprodex
3) repaired at 10yo

Answer 10

you CAN use ciprodex drops and should be used.

Question 11

12 yo draining left ear for one year - what is likely diagnosis

1) cholesteatoma
b) AOME
c) Otitis externa
d) perforated AOM

Answer 11

cholesteatoma - unilateral foul smell
persistant and recurrent and responds to ototopicals but recurs by months

(intracranial complications in 0.36%)

Question 12

Deafness can be caused by all except (if parents have normal hearing)
1-cmv
2-ionic homeostasis within cochlea affected
3-branchial oto rental sx
4-mondini deformity

Answer 12

branchial oto rental sx

(AD - would also be in parents)

monidni is inner ear issue with 1.5 coils instead of 2

Question 13

what are the ABCDs of hearing loss
50% acquired
50% inherited
- connexin 26 most cmoon genetic AR cause , transmembrane protein that recyclkes K

Answer 13

Affected family member
Bili high
Congenital TORCH
Deformity
s- SMALL <1500g, low apgar, nicu stay

without screen detect at 18mo, goal is diagnosis and habilitation by 6 mo

Question 14

13yo M wiht severe chronic epistaxis with normal bleeding tests
1- rpt tests
2- transfuse prophylactic
3- refer to ENT
d- start steroids

Answer 14

REFER - need to rule out juvenile nasal angiofibroma

Question 15

secondary indications of OSA include?

Answer 15

enuresis
ADHD
daytime somnelence
FTT
cor pulonale

Question 16

name 6 causes of stridor organized:
supraglottic
subglottic
and tracheal

Answer 16

laryngomalacia, epiglottitis

subglocttic- stenosis, hemangioma, croup

tracheal- tracheomalacia, FB, complete tracheal ring, TEF

Question 17

patient presents with torticollis and drooling with fever at 4yo
diagnosis
treatment
bugs

remember trismus - peritonsilar abscess , or supporative parotitis (can also happen with RPA)

torticollis ddx - pseudotumor of infancy, CN4 palsy, cerebellar tumor of post fossa

Answer 17

RPA
(involutes by 5yo)
GAS, oropharnygeal and staph
Xray C2-7mm, C6-14mm or >1veretbrae, loss of normal lordosis
treatment IV clinda and ceftriaxone +/- drainage
Complications - Upper airway, lemieres syndrome (throbophlebitis of JV), coronary artery sheath erosion

VERSUS lemierre DISEASE is infxn of oropharynx causing septic thrombophlebiyis and metastatic emboli to lungs, fusobacteriu, neocrophorum cxr cavitations and effusion

Question 18

what are 4 absolute indications for T and A
and 4 elective ones

risk of re bleed 7-10 days after seperation of eschar

Answer 18

ABSOLUTE
tumor of tonsil
uncontrollable hemm
extreme obst causng apnea
interfereing withs wallow

ELECTIVE
infxn >7/y, >5/2yr, >3 /yr/3years
>1 PTA, or PTAx1 if recc infxn
PANDAS
PSG - disordered breathing of >10 episodes per hour

Question 19

name tumors of the necl

Answer 19

small round blue cell tumor - lymphoma,r habdo, ewing
thyroid carcinoma
neuroblastoma
langerhand cell histiocytosis

Question 20

Marfan disease associated with which heart defect

Answer 20

MVP (mid systolic click), dilation of ascending aorita

Question 21

whats the following murmurs

fixed split S2, low pitched systolic ejection
systolic ejection murmur radiated to neck
systolic ejection murmur radiating to back
high piched systolic regurg murmur at LLSB

Answer 21

fixed split S2, low pitched systolic ejection- ASD
systolic ejection murmur radiated to neck - AS
systolic ejection murmur radiating to back - PS

high pitched systolic regurg murmur at LLSB - VSD, MR

Question 22

which long QTc associateed with hearing loss

Answer 22

Jerve Diel Nielson

treatment is with beta blocker
cannot interpret QTc if have WPW or bundle branch block

Question 23

who qualifies for palizvumab

**rememebr norwood/sano or shunts oxygen 75-85% can be normal
arterial swtihc sat 100%

wiht single outlet ventricle - first surgery is GLENN at 4-6mo (sat 75-85)
and then fontan at 2-4yo sat >90%

Answer 23

1) CHD or CLDneeding oxygen or meds and are <12 months, can consider for 2nd season in weaned off oxygen in last three months or still on oxygen
2) <30 week without CLD who will be 6 months at start of season
3) inuit/remote that is <36week and will need to be flown in if <6mo start of season
4) NOT needed it pid, CF or DS –prophylaxis may be considered for children <24 months of age who are on home oxygen, have had a prolonged hospitalization for severe pulmonary disease or are severely immunocompromised.

Question 24

for KD - what is the long term management for anticoag for the following

1) normal or transient dilation z score <2.5 -
2) 2.5-5 small aneursyms -
3) medium anyresym is 5-10 long term antiplatelet therapy (ASA for life)
4) >10 or large

Answer 24

1) normal or transient dilation z score <2.5 - antiplatelet) x 6 w
2) 2.5-5 small aneursyms - low dose ASA beyond 6 w
medium anyresym is 5-10 long term antiplatelet therapy (ASA for life)
3) >10 or large - ASA for life and anticoag with maybe beta blocker

Question 25

endocarditis prophylaxis needed for dental procedures or GU or GI or skin msk surgeries if there is an infection cover for entterococi with amp or vanco or resp - invasive procedure of the respiratory tract that involves incision or biopsy of the respiratory mucosa, such as tonsillectomy and adenoidectomy, NOT just bronch Amox before the procedure as one dose - upto 2hours after surgery

Answer 25

Prosthetic cardiac valves Previous infective endocarditis Unrepaired cyanotic congenital heart disease, including palliative shunts and conduits Completely repaired congenital heart defect with prosthetic material or device, during the first six months after the procedure Repaired congenital heart disease with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization) Cardiac transplant recipients with cardiac valvulopathy Rheumatic heart disease if prosthetic valves or prosthetic material used in valve repair

Question 26

what are the four innocent murmurs

Answer 26

1) venous hum - louder when sitting , from jugular turbulence 2) stills murmur, ejection murmur (AV/MV position) 3) peripheral pulomary artery stensos in new borns till 3 months (lung/back are) 4) physiolical pulm flow murmur (P area over 3yo and louder when lying down)

Question 27

classic places for these murmurs to be heard? ``` ASD AS PDA PS VSD ```

Answer 27

``` fASD - fixed split, low pitch AS - radiate to carotids PDA - radiate to back coarct - radiate to back PS - radiate to back VSD - LLSB. regurg murmur or at apex if MR ``` single S2 is never normal (usu due to having only one valve) To and fro is TOF with absnet pulmonary valve click is a valve stenosis

Question 28

what are two genetic conditions that cause LQTS and their associated findings

Answer 28

Jervell Lange Nielson - AR, SNHL assocaited and CHD Romanoward - AD and only cardiac phenotype SCREEN - ecg, and can do parental ecg too >470 seconds considered long males 440 female 460 is normal (other causes low Ca, Mg, or TCAs)

Question 29

``` name four causes of acquired LQTS w workup hx/physical ecg serial parental ecg holter echo (exercise test u have to watch them after) ```

Answer 29

1) hypo K, hypo Mg, hypo Ca 2) meds (benadryl, psychotopics like rispirdone and like haldol, furosemide, abx) 3) endo hypothyroidism, pheo, hyperparathy tx beta blocekr is first like and may need pacemaker if profound

Question 30

WPW - three findings on ecg two outcomes possible

Answer 30

cause - accessory pathway in bundle of KENT (AV path) short PR interval wide qrs delta wave Can cause sudden death or SVT - must avoid DIGOXIN

Question 31

SVT presentation ECG findings Tx

Answer 31

commonly idiopathic cause commonly within 4mo life present wiht CHF HR>220 absnet P waves. narrow cqrs, regular rhythm (no variance in RR) tx- stable adenosine, or synchronized 0.5-1k/kg maintenace is beta blocker

Question 32

match these conditions with associated cardiac abn ``` t18-edward t13-patau VACTERK turner FAS digeorge william alagile marfan ```

Answer 32

``` t18 - edward- vsd, polyvalvular t13- pda turner-coarct and bicuspid aortiv valve and AS FAS- VSD, conotruncal VACTERAL-asd csd pda digeoge, TA, inturrupted aortic arch william supravalcuar AS alagile - periperhal {PS, DORV marfan MVP and aortic root dilation ```

Question 33

name 5 duct dependant lesions

Answer 33

``` transpostion of GA with intact septum PS PA AS TOF severe coactation hypoplastic L heart ```

Question 34

ASD findings on ecg usu asymptmatic - usually poor growth, reccurent infections These get LAD VSD present 6 weeks when PVR decreases - most self fix by 4yo AVSD similar presentation but repair at 6 mo PDA (wide pulse pressure with continous machine like murmur)

Answer 34

fixed split s2 systolic ejecton murmur wiht diastolic rumble due to inc flow acorss trivuspid usu if closed b4 25 does ok if secundum then RAD if pri,um then LAD and endocardial cusion defect remenber VSD most common congenital lesion

Question 35

Diagnosis and treatment of Tet spell most common cyanotic CHD repaoir best at 4-6mo still at risk of cardiac death, arrythmias, RV dysfunction

Answer 35

TOF- PS, overriding aortic arch, VSD, RVH - ant displacement of infundivular septum- sx based on RVOT obstruction, at risk of stroke Single S2, RVH, RAD, boot shape Treat with oxygen and fluids, knee to chest, morphine, inc SVR with phenylepherine betablocker- to lesson RV outflow obstruction

Question 36

presentation timing of heart lesions NEWBORN 6 weeks when PVR drops LATER

Answer 36

``` newborn: TGA - egg on string TOF - boot TA: left axis+LVH TAPVD (PGE make it worst) - snowman Tingle ventricle, days-1w when PDA closes ``` 6weeks: VSD, PDA, coarct, Truncus (as PVR drops)

Question 37

Managemnet of HLHS ``` Longterm complications arrythmias protein losing enteropathy cyanosis for AVMs and collatorals plastic bronchitis lymphangectasia ```

Answer 37

single ventricle - present ok then when PDA closes shock and poor feeding 1st suergery at 4-6 months is GLEN to SVC and PA connection then FONTAN at 2-4 y to cnnect IVC to RPA

Question 38

infective carditis organisms: duke criteria for diagnosis exam findings a hemloytic strep, staph aureus, CONS exam: splinter hem, janeway (non painful on pal and sole) osler painful on pulp fingers roth- exudate edematous retinal hemm

Answer 38

DUKE criteria MAJOR 1) Cultures - positive bcx x 2 (drawn 12 h apart) - single positive coxiella burnetti or anti-phase I IgG ab titre>1:800 - all 3 or majority of >4 separate cultures 2) Evidence of endocardial involvement - positive echo or new regurg (change in murmur not enough) (minor criteria include: predis heart conditon, fever, vascular change, immunological change, microbio evidence) need surgery if: vegetation/septic emboli, valvular dysfxn or perivalvular extension

Question 39

cardiomyopathy - HCM - sarcomeric protein cause, most common cause of death sudden in child puverty to adulthood Restrictive is DESMIN with skeletal myopathy and is AD, assoctaited with GSD lysosomal storage disorders, treatment is transplant Arrythmmogenic right ventricular cardiomtopahthy (with R ventricule getting fat/fibrous

Answer 39

treatment - beta blocker no strenous physical activity if fmhx arrhtymia put ICD screen all 1st degree relatives ECH and echo 3-5 years <12y and then yearly if older sudden death if family member is a poor prognosis

Question 40

what are risk factors for pHTN >25mmHg (normal 8-20) MAS, pulm hypoplasia, early onset sepsis, hypoglycemia, RDS, polycythemia, matenral NSAID

Answer 40

``` treatment oxygen intubate inhaled ntiric anticoag CCB phosphodiesterase inhibitor (sildenafil) transplant ```

Question 41

differnetial for pulsus paradoxus (change in BP by more hten 10 with inspiration (SBP)

Answer 41

``` OSA chronic pericarditis cardiac tamponade tension pneumo asthma pregnancy ```

Question 42

Rheumatic fever | fidings and secondary treatment to prevent

Answer 42

usually affects mitral THEN aortic valvaes with ECG prolonged PR severe carditis pred 2 mg/kg, mod 1-2mg+ ASA, mild just ASA prevention penicillin q3-4w oIM or pen V 250mg po BID IF carditis - upto 5 y or 21 y, later one carditis without seequale 10y or 25 carditis with valvular FOREVER, or 40

Question 43

heart failure by age causes: first week of life weeks 2-6w adolescent manage: head up, tube feeds, high cal formula, salt restriction, fluid limits diuretics aceinhibitor dopamine to inc contractlity minimize ONGOING damage with beta blocker

Answer 43

first week its HLHS severe AS coarctation 2-6w: VSD, AVSD, PDA (NOT ASD) adolescemt: DCM, myocarditis, tachycaridas

Question 44

name six causes of primary amenorrhea (14 without breast development or 16 with breast development)

Answer 44

1) Xm: turner, androgen insensitivity, 5alpha reductase, kallman(LHRH defect, anomsia) 2) anatomic: vaginal septum, imperforate hymen 3) hypothalamic: stress, exercise, ED 4) CNS tumor - prolactinoma, craniopharyngioma 5) ENdo: CAH, thrdoid OVarian; COS, POF 6) prengnacy

Question 45

Name 6 causes of secondary amnorrhea - cessation >3mo consectuveively (althou first two years irregular is normal) inv: ultrasound, FSH, LH prolactin, karyotype, TSH, 17OHP, testosterone DHEAS If FSH and LH high then ovarian primary issue If low - hypothalamic for PCOS LH:FSH ratio is 2:1 wiht low estrogen and high androgens

Answer 45

1) pregnancy 2) hypothyroidism 3) PCOS 4) function hypothyrdoism like BMI exercise, ED 5) prolactinoma 6) late onset CAH 7) Drug - rispierdone (anything tha t increases prolactin) Traetment - try induce progesterone withdrwal bleed and if it does bleed its due to anovulation

Question 46

Describe how anovulation works with physiology and H

Answer 46

immature HPO axis, hence estrogen does not suppress FSH, ongoing FSH, prevent LH surge, hence no ovulation and no CL formation which no progesterone to stabilize endometrium.

Question 47

effects good and bad of estrogen and progesterone Prevents ovulation, thickens cervical mucus, inhospitable endometrium. blocks sperm penetration reduces ovarian and endometrial cancer

Answer 47

estrogen good: controls hirutism and acne, cycle control bad: boob tender, bloating, mood changes, headache, n/v, liver and gallbladder diseaase, inc cervical dysplasia over 5y use Progesterone good: dec bleeding, prevents ovulation BAD: acne hirtusism break throgh bleeds

Question 48

with RAPE | investigations and treatment

Answer 48

inv: cultures for STI, bloodwork for syphillis and HIV Treatment 1-emergency contraception 2- STI prop: cefixime and azithro metrondiazole for BV 3- rpt HIV and and hepatitis syphillis at 12 w 4- rpt wet mount, cultures and pregnancy test at 12w 5- psych support

Question 49

STI- investigation, sx, treatmebt BV Trichomonas Candida

Answer 49

BV-wet mound CLUE cells, fish ph>4.5, grey copious not an STI tx flagyl test x 7d Trichomonas - wet mound flagellated protazoa, ph>4.5, frothy and strawberry cervi tx flagylx1 d candida, white yeast on wet mount or pseudhyphae - treatment topical or oral fluconazole

Question 50

differential for red lesion on labia

Answer 50

``` 1 -chancre (syphillis) tropenoma pallidum 2- chancroid 3- herpes 4- trauma 5- HPV ```

Question 51

6 A's of smokiing cessation\ | can use patch plus CBT

Answer 51

``` ask about smoke status advise to quit assess readiness assist with effort arrange fu anticipate risk of use in adolescent ```

Question 52

what are side effects of androgen and steroids some athletes also use GH: causes insulin resistnace, hyperglycemia, DM, Na retention, cardiomegaly, carpal tunner

Answer 52

hepatotosixc, gynecomastia, erythrocystosis, premature fusion of epiphseal, infxn, virilization in women

Question 53

6 things for adolescent transition u can do

Answer 53

1) see patient alone 2) adol manage their own stuff 3) autonomy and independance 4) peer support 5) transition clinic with adult provders 6) tranistion letter 7) involve family MD

Question 54

``` Difference between PROXIMAL (Type 2) RTA and distal - physiology Urine pH lyte abn associated condn ``` RTA -4 behaves like aldosterone deficeincy or resistance - so hyponat and hyper K

Answer 54

PROXIMAL - failure to reabsorb HC03- (more losses) urine Ph<7 hypoNat, hypoPhos, glucosuria, aminocadiruai/proteinuria, Fanconi (caused by cystonosis, tyrosenimia, galactosemia) DISTAL- type 1, failure to secrete H, urine ph>7, normal sodium and nephrocalcinosis, hearing loss, caused by obstructive/renal dysplasia, lupus

Question 55

Proteinuria Diff btwn nephrotic and nephritic remember proteinuria predicts CKD progression in children

Answer 55

Nephrotic: proteinuria (>40mg/m2/hour) or 250mg/mmol, edema, hypoalbumin<25, high TG Nephritic is PHAROH proteinuria, hematuria, azotemia, RBC cast, oliguria, HTN

Question 56

Diagnosis of nephrotic - ususally its minimal change Lab investigations ABn findings Treatment Considered steroid dependant if relapse in taper within 2 weeks or with discontinuiation Steroid resistant if cannot induce remission within 4 weeks of daily steroids- usually FSGS and 50% need transplant in 5 years ABN presentation <12mo,>10yo, persistant HTN, impaired renal fxn, hematuria, low c3, positive hepB or C

Answer 56

protein range >40mg/m2/hour edema, high TG labs: cbcd, lytes, cholesterol, glucose, early morning pr:cr ratiom, urine microscope and C&S, albumin, VZV, Hep B/C and HIV, complement ANA if <2yo low salt, fluid resitrict, steroids 2mg/kg/d for 6 weeks and then taper, +/- diuretics and albumin Remission if dipstick negative for 3 days or protein/Cr ratio <20g/L frequent relapsers are 2 or more within 6 months or more then 4 in 12 months. Need to move to cyclphosmpahde, MMF or cyclo, bx

Question 57

What are complications of neprhortic syndrome remember to vaccinate normally except live vaccines need 3 months of pred or CSA, and 6 months of cyclophosphamide

Answer 57

1) SBP with (s pneumo, ecoli, h flu) 2) hypercoagulable state (loss antirhombin and Protein c and S) 3) hyperlipiedemia (takes the longest to crrect)

Question 58

``` Differnetial for GROSS hematuria Glomerular: deformed red cells, casts, acanthocyte Interstital: Malignancy Tract- ```

Answer 58

Glomerular: nephritis (post strep, IgA, HSP) or alport, benign familial hematuria Interstital: pyelno Malignanc: wilms Tract- UTI, stones, FB

Question 59

post strep glomerulonephritis order of resolvement of HTN, Proteinuria, C3, hematuria

Answer 59

first HTN resolves by 2 w then C3 norma by 6-8w then proteinuria 4 months then microscophic hematuria upto 2 years (but mainly gone by 3-6 months)

Question 60

Differential for low c3 and normal C3 most common cause of MICROScopic hematuria is idiopathic hypercalcuria Also get it in ALPORT (xlinked, hearing loss collagen d/o, anterior lenticonus-eye) HSP can cause - REFER if macroscopic hematura, proteinuria or HTN

Answer 60

``` low c3 lupus psgn shunt nephritis MPGN endocarditis ``` normal c3 IgA nephropathy HSP - remember weekly urine for 1 month, then biweeklyx2 month,then monthly x 3 months- total 6 months, 1% will get ERSD. Higher risk of renal disease IF older, perisstant purpura or severe abdo sx alport anti GBM

Question 61

Diagnosis of SLE | 4/11

Answer 61

``` MD SOAP BRAIN Malar rash discoird rash Serosistis Oral ulcer Arhtriits Photosensitivity ``` ``` Blood dyscaria (penia) Renal ANA positive Immune changes (false VRL, anti dsdna, ant sm anti ro Neuro changes ```

Question 62

defintiion of HTN and who to treatment and treatment options Measure with a cuff on R arm, sit for 3 minutes with arm heart level, should be80-100% of cirucmfrence with width being 40% treatment: non pharm wieght reduction, exercise, dec salt, smoke cessation pharm ACE inbhibtor, NOT beta blocekr if black try thaizide if renal artery stenosis CANNOT use ARB or ace inhibitor

Answer 62

``` DEFINITION 1-13yo: preHTN is<90th or 120/80 Stage 1, >95th+12 or 130-139/80 stage 2: >95+12 or 140/90 ``` 13 and above preHTN 120-129 stage 1 130-139 stage 2 >140/90 Treat: symtpmatic, stage 2, stage 1 if failed 6mo nonpharm, stage 1 with DM or CVD, and preHTN if CKD and DM

Question 63

post transplant kidney monitor CMV EBV opportunistic infxn

Answer 63

infxn include mycoplasma, cmv, PJP avoid macrolides as interact with immunosuppresion ebv look for lymphoproliferative disorders (risk factor if recipient neg and donor positive, thymboglobulin, infxn with CMV) look for HARD node, weight loss and recc pharyngitis

Question 64

What is considered a simple cyst and when to refer vs unilateral cyst differnetial includes multcystic dysplastic kidey, but there is no URETER, usually L sided and M?F and the other side hypertrophy (ALSO UPJ and reflux) - malignancy risk <1/1000

Answer 64

rare in peds less then 1cm, <3 , unilateral and increase slowly if at all and no echogenic focus refer if calcifications - septae, loculated or thickened

Question 65

Prune Belly syndrome TRIAD? associations? (renal dysplasias in general assocaited with reflux, PUV and spina bifida)

Answer 65

mesodermal defect, more common in males abdo muscle defieicny, urinary tract abn (hydronephrosis, dilated ureter and distended bladder) and bilateral cryptochordism association skeletal clubfoot, dislocated hyips, torticollis, lung hypoplasia and heart rare abnormalities (Potter sequence)

Question 66

``` HUS - triad? - investigations - risk factors for dveloping prognosis ```

Answer 66

triad: MAHA, thrombocytopenia, AKI inv: coombs, lipase, LDH, G and scren, bili and calcium RF: age <5yo or >75, vomitting, more then 3 d diarrhea, blood in stool and WBC >13 if fluid resus well in dairhea statges then better prognosis but 30% have renal or neuro sequale

Question 67

``` 3 week old male referred to you for failure to thrive by the family doctor • Birth weight 3.5kg • Current weight 3.3 kg • Noted to be fussy in the evenings, feeding well but needing tops by bottle after every feed Polyhydramnios identified during the pregnancy • Na 130 • K 2.5 • Chloride 79 • HCO3 34 • Creatinine 25 ```

Answer 67

Bartters cant reabsorn Na/Cl/K as they are all dwon like being i=in lasix polyhdramnios - problem with GI (swallowing and atresias or tubulopathy if JUST Na and CL were down and presented in adolescent and chldhood with weakness and fatigue and palpitaiton think GITELMAN syndrome as behaves lke a THIAZIDE acting on distal tubule

Question 68

Causes of Hypernatremia Hypervolemic (Na excess) Euvolemic (water deficit) Hypovalemic (water and Na deficit) Symptoms - thirst, irritabile, vomit, restless, lethargy, weak, coma

Answer 68

Hypervolemic Hypernatremia - IV hypernatremic fluid - excess formula- - hyperadolesteronism Euovolemic- - DI (low ADH) - increased loss (prem, phototherapy) - low intake: breastfeeding ineffective, adipsia Hypovolemic -GI loss (diarrhea emesis) Cutaneous loss (burn or sweat) - renal loss: diuretics, DM, CKD, ATN, postobstructive diuresis)

Question 69

Causes of hyponatremia symptoms, nausea, letahrgy, seizure, headache

Answer 69

to correct- Na deficit = 0.6x weight (Goal Na-actual Na) = Na mmol needed to correct) remember 10/12mmol in a day and 0.5mmol/h HYPOVOLEMIC HYPOnatremia Non renal Una<10 - GI loss, skin loss Renal cause UNA>20 - salt wasting or hypoaldoesteronism, RTA Give : saline EUvolemia:- Na normal, high water Non renal Renal UNA>2Na, urine Osm >300 thyroid or glucocoritcoid HYPERvolemic (++high water/+salt) Renal: failure Non renal: cirrhosis and CHF treatment: fluid and salt restrict

Question 70

HyperK causes (remmeber RTA 4 has hyper K) HypoK causes

Answer 70

Hyper K decreased excretion: RTA4, hypoaldoesterone, ACE inhibitor cell exchange: hyperglycemia, cell turnover, acidosis, rhabdo, hemolysis Hypo K increased excretioN: RTA1/2, Fanconi, Bartter Gitelman, diuretics and diarrhea, metabolic alkalosis, DKA

Question 71

Nephrotic syndrome - proteinuria >40mg/m2/h, ACR>200mg/mmol - hypoalbuminiemia -- causes high TG - edema Management 1) fluid restrict, salt <1500mg/day 2) steroids 60mg/m2 x 6 w then taper 3) vaccinate encpauslated (h flu, strep pneumo and meningitis) 4) acute HTN tx with beta blocker/CCB 5) fluid overload, lasix

Answer 71

Types (atypica; <12mo,>12yo, if HTN, high Cr, gross hematuria) 1) Minimal change most common 2) FSGS 1/3 go to renal failure 3) MPGN most likely to go into renal failure, hypocomplement 4) membaranous uncommon, mainly in adoelscents with infection (HEP, syphillis and malaria) Congenital - rare, 1st 2 mo of life wiht AR mutation npehrin protein and prental onset shows elevated AFP

Question 72

Causes of proteinuria: 1) transient proteinuria (with fever, cold, does not exceed 1-2+ on disptick 2) orthostatic - do first AM urine sample x3d Others: glomerular - PSGN, SLE, HUS, mylemoma, rhabdo, tubular : ATN, PCKD Hematuria causes: >5 RBC/hpf- highly sensitive dipstick (false positive: oxidingzing agents, bacteria, beets, 1) UTI 2) hypercalciuria (ISOLATED hematuria) 3) benign familial hematuria

Answer 72

``` hematuria: SHIT stones, sle, structural H hematological, hypercalciruia, sickle, HSP infectious: IgA T-trauma, tumor, TB, toxin ``` If its glomerulonephritis (BOTH hematuria and proteinuria) - think PGSN, else bx nephritis - Proteinuria, Hematuria, Azotemia, Oliguria, Red cell casts, HTN Ddx (normal C3 - IgA nephropathy, antiGBM alports, and low c2 is SLE, PGSN, MPGN, shunt, and endocarditis) WORKUP: Urine: protein, Cr, calcium, culture, analysis and micropscy Blood: CBC. lytes, Urea and Cr. Cal albumi, protein ASOT, ANA c3 and C3, rneal u/s bx- if neprhotic range proteinruia, renal insufficeincy or fmhx of CKD

Question 73

with ARF - causes, lyte abnormalities? K, Ca, phosphate management if its CKD - supplement Ca, get phosphate binders (CaC03) and restrict phospahte. need epo

Answer 73

pre RENAL/renal/POSTrenal dehydration, meds, vascular, DIC. ATN, obstructive FENA: U/P NA dvideid by U/P Cr <1% pre renal HyperK, HypoCa, Hyperphos, acidosis management in/out, fluid: insens wiht ongoing loss, lyte, renal dosing, treatment of electyrlytes, dialysis if uremia, hypervolemia unrepsonive or major lyte abn

Question 74

workup for HTN and organ damage workup

Answer 74

``` ORGAN echo retinal exam urinalysis for proteinuria and ACR neuro exam for stroke ``` w/u: u/a lytes BUN cr renal us CBC d and dppler urine catehcolamine, TSH CVD risk factor lipids, uric acid.

Question 75

RF for renal vein thrombosis

Answer 75

common form in neonates not assoc with vascular catheter RF- prem, asphyxia, dehydration, polycy, CHD, maternal diabietes present with flank mass, hematuria, low plt

Question 76

Work up for stones and treatment idopathic hypercalcuria= AD, sacroidipsis, recurrent gross hematuria with peristant micrscopic hematura with 24h urine Ca>4,g/kg and treat with oral thaizide diurteics

Answer 76

1- urinalysis and microscopy 2- lytes, c02, gas, Ca, Mg, p04, urate, urea, Cr, PTH and VIt D Urine spt Ca, ur, notroprssode screne 24 hour urine: Ca, oxalate, citrate, urate, cr and volume 4- renal u/s ``` Management inc fluid intake inc citrate dont limit Ca (unless hyper Ca) limit salt minimize steroids, vit D and excessive steroids ```

Question 77

proximal vs distal RTA think of it when its non anion gap hyperchloremic metabolic acidosis remember type 4 has hyperkalemia =with aldoesterone deficeincy

Answer 77

type 2 rta proximal - impaired HC03 reabsorption, PH urne <5.5, caused by Fanconi - caused by cystonosis present with FTT, vx/dx, polydip,polyuria IN URINE: glucosuria, proteinuria, amoniacirduria, phosphaturia (measure cystiene levels in the lekocytes that diagnosis cystonosis) - have hypoK,p04, hyperurisua type1 - rta distal - think impaired H sectetion, Ph urine>5,5, they get stones NEPHROCALCINOSIS hence have high Ca levels, low citrate levels, provs with primary hyperparahytroidism, vit D intox, can get in SLE too and post obsturctive uropathy

Question 78

name causes of HYPOnatermic, HYPOchloremic, HYPOkalemic metabolic alkalosis

Answer 78

lasix use PS CF BArter

Question 79

indications for dialysis

Answer 79

``` uremia wiht sx severe lyte derangements high ammonia drug- salcicyalte,e thylene glyclol, methaonl, isopronaol, fluid overload ```

Question 80

causes of wilms tumor | NOT li fraumeni (AD, leukemia, p54, breash scarcoma and leukemia)

Answer 80

WAGR (wilms, aniridia, gu issues, retard) Denys Drash, renal sclerosis Wilm tumor Beckwith Weidemann Russel silver- 11p15 hypometyliation, ska, triangle face, cafe au laid, cardiac, clindodatyl, fasting hypoglycaemia -also HCC limb asymetrry- short stature

Question 81

Autosomal dominant inherited disoders name 5 peutz jegher - hypermelanosis of th lips increase risk of cancers - not super high risk, inc risk of intuss also OI -hearing loss short stature

Answer 81

Marfan - pectus, ectopic lentis, MVP, aortic root dilatation, scoliosis, NORMAL skin elasticity, thumb sign, wrist sign Waardenburg syndrome - neural crest cells, get white lock hair, heterochromia irides, hearing loss and hirshsprung Achondroplasia - OSA, foreman magnum narrowing and brainstem compression, stenotic spinal canal , lordosis, rhizomelic shorten limbs NF1 Tubrous sclerosis DCornelia Delange Rbinstein Taybi - beak nose, broad thumbs, cardiac, mental retard Noonan (downslanting palpebral , renal , ps, coat issue, pectus, HCM, snhl, gets better with age, crypthochordism, vision issues strabismus and amblyopia

Question 82

Tuberous sclerosis

Answer 82

Diagnostic criteria: (2 major, or 1 major with 2 minor) MAJOR criteria: - facial angiofibroma - non traumatic ungula or periungual fibroma - hypopig macules - shagreen patch - retinal nodular hamartomas - cortical tuber - subependymal nodule - SGCT - cardiac rhabdomyoma - lymphangioleiomyomatosis - renal angiomyolipoma - can become RCC MINOR featuers: - pits in dental enamal - hemartomatous rectal polyp - bone cysts - cerebral whtie matter migration - gingivial fibromas - nonrenal hamartoma - retinal chromic patch

Question 83

Diff btwn homocystien and marfan

Answer 83

MARFAN - AD, superior ectopic lentis, no hyperoacgulable, tx with Losartan to dec TGF beta (prevent aneurysm) Homocysteine- AR, hypercoagulable, inferior ectopic lentis

Question 84

Turner need referall to endo for GH therpy if height less tehn 3rd centile at 12yo need referal for estrogen streak goands inc risk of hypothyroidism hashimitor, celiac (also dm1 and Down syndrome) and IBD no increased risk of cancer except pilomatricoma which is benign skin lesion ``` managemnet ash yearly echo urine yearly and blood glucose for renal Diane diabetes liver enz yealy hearing q3-5y optho exam lh and fish before hormone replace at 12yo psych assessment ```

Answer 84

``` Signs and Sx: short stature, short 4th metacarpal, high arch palate, nail dusplasia broad chest low posterior hairline and low set ears sterility, neck webbing rudimentary ovaries - can carry pregnancy with donor egg amennorhea - premature ovarian failure inc weight and obesity Aortic valve stenoisis, coarctation of aorta, bicuspid aortic valve horseshoe kidey visual impairments, glaucoma ear infxn and hearing loss - SNHL ADHD LD MORTALITY DUE TO RUPTURE AORTA WITH PREGN hypothyroidism HTN T1DM, DM2 ```

Question 85

Monitoring in BWS

Answer 85

MONITOR inc chance of embryological cancers (wilms, neuroblastoma, rhabdo) hepatoblastoma AFP q 3 month for first 4 years of life AUS every 3 months till 8 years old 8- adolescence, check for nephrocaclinosis, medullary sponge kidney disease, consider urine Ca/Cr ratio

Question 86

``` CF - manage Clinical presentation: RESP - chronic cough - bronchiectasis - pneumothorax - hemoptsis - pHTN/ heart failure ``` GI - protein and fat malabs, loose stools - FTT - meconium ileus - distal intestianl obstruction - obstructive jaundice - focal biliary cirrhosis - rectal prolapse - recc pancreatitis UPPER AIRWAY - chronic pansinusitis - nasal polyposis OTHER: DM1, digital clubbing, dehydration, ADEK def, zinc dermatitis, infertility in males

Answer 86

PULMONARY - antimicrobials, chest physio, hypertonic saline, transplant considered if FEV1<40 PANCREATIC - ADEK vitamins, pancreatic enz pre meal, suppl calories WATCH GROWTH - and BMI DIABETES - sclerosis of islet of langerhan INTESTINE - obstruction, illeusm visceral perforation, give laxatives, can have rectal prolapse

Question 87

Name 2 X linked dominant disorders side note- IPS screen is HCG, UE3 and AFP all low for trisomies, except for down syndrome HCG high

Answer 87

incontinentia pigmenti | RETT syndroime- mainly FEMALES

Question 88

Uniparental disomy conditions Prader Willi- rapid growth 1-6yo - test with methylation or fISh studies , with hypothalamic and gh deficiency, good at jigsaw. need optho for myopia, endo for h, respect for sleep,. development for motor and speech Angelman BWS

Answer 88

prader willi, paternal imprinting (deleted parenternal Xm 15) - hypotonia, grwoth delay, obesity, hypogonatodroptic ghypogonaidsm, developmetnal delay, alond shaped eyes, small hands and feet, micriopenis and cryptohochrodism Angelman, no speech and seizures, happy puppet, deletin matenral Xm, mental retard, walking broad based, ataxia, wide space teeth proturde tonge, TEST methylation specific tst BWS: patenral Xm 11 macrogloassia, omphalocele, hepatomegayl, cardiomegaly, Wilms, hepatoblasoma, neirpblastoma, adrenacotrcicl carcionma, VERTICAl ear creases

Question 89

Expansion of trinucleotide rpt mutation includes fragile X, what are the rpt and some findings in premutation Other conditions: Huntington, Freidrecih ataxia, Myotonic dystrophy

Answer 89

CGG rpt in FMR1- >200 affected, premutation mother has POF and neurocognicitive defects or males have fargile X tremor axtaxia syndrome pt has large head, mitrl propalpse, large ears, flat feet, MACRO orchidism, mild CTD, ASD, PDD, NO regression of milestones

Question 90

MATCH these with upslanting or downslanting or narrow palpebral fissures ``` T21, prader willy Marfan FAS William Difeorge Sotos Lennox Gatuaut\ Ehler dan ```

Answer 90

UPSLANT- t21 and prader willi DOWNSANT - think CTD- marfan, EHS, Sotos (pda, and, kidney, cerebral gigantism,overgrwoth) lennox gestaut, cri du chat (5p deletion), Noonan SHORT - FAS, Wililam and Digeorge side note XXY (klinfelter- most common cause of hypognadism and infertlity in men) present at puberty gynecomastia, inc risk of rbeast and medistinal tumor. small testis, normal TESTOSTERONE, osteopenia, low ingeliggence, inc risk of psych issues

Question 91

Fetal efects of maternal drugs: 1) tobacco 2) cocaine 3) matenral insulin 4) phenytoin 5) alcohol

Answer 91

1) tobacco- placental abruption, PROM, placenta previa, PTL, LBW, increased mortaliy, SID 2) cocaine - hyperjittery baby (not withdrawal) inc suck, high pitch cry 3) maternal insulin causes caudal/sacral agenesis 4) phenytoin: fetal hydantoid syndrome (cleft lip, CHD, hypoplastic digits) 5) alcohol - FAS - short papebral fissures, smooth filtrum, thin upper lip, upturn nose, railroad ears

Question 92

``` what is the most common trisomy 16 21 13 18 ```

Answer 92

16 - causes SA but most common if alive its 21 - sandal gap toe, upslanting palpebral fissure, single palmar crease, flat nasal bridge, epicahtnal folds, brush feild spot, AVSD, DA, annular pancrease, imperforate anus, hypothyrodisims, inc ALL, alzeiheimer

Question 93

Management t21

Answer 93

birth - eye exam, hearing tst, echo TSH and CBC 1-5yo watch for OM, eye, TSH anually, xray if symptoms 5-13yo hearing and vision annually, TSH, OSA

Question 94

High risk ALL stratification name 5 things

Answer 94

``` age <1, >10 wbc >50 000 hypoploidy cns positive disease mrd ```

Question 95

William syndrome 7p deletion perfect pitch and musical abilities

Answer 95

``` chd suprvalvular ps and as elf facies fullness of lip blue irises friendly and social moderate mental retard dd hypercalcemia in neonate and nephrocalcinosis hen hypothyroid hyperacusis adhd ```

Question 96

what does CHARGE AND VACTERL stand for think CHARGE is heart and above (no renal stuff)

Answer 96

coloboma, heart effect, atresia choanael, retarded, genital, ear vacterl vertebrae , anus imperforate, cardiac, tef, renal, limb

Question 97

AR disorders

Answer 97

smith lemli optiz -cholesterol metabolism issue 7DHC | short stature, microcephalic, mod/severe disability, syndactyl of 2-3 toes, geniterlalie may be ambiguas in male

Question 98

dmd - x linked recessive

Answer 98

``` diagnose dystrophin gene via molecular testing eledvated ck oust 2-5yo weak, calf hypertrophy, intellcular challenge monitor for dcm ciao echo q2y resp oft and nocturnal hypoventilation, ortho for scoliosis bmd testing ```

Question 99

causes of snhl - hypertrophy of nerve bundles and absence of ganglion cells - absence of anal spinhciter relaxation causes hirshsprung Down syndrome, Waardenburg, Haddad syndrome (phox2b central hypoventilation, waardenberg, CHH, smith lemli optiz

Answer 99

``` cmv waardenberg - also synophyrns and hirshcpsring turner down syndrome haddad syndrome ```

Question 100

ehler danloe

Answer 100

smooth velvet skin, skin hyperedtendible, jt hyper mobile, hernia anal propels dna sequence col5a1, AD mullusoid pseudtumor which is violacious subcutaneous mass over pressure points

Question 101

random conditions to know mccune albright abetalipoporteiniema bardat biedel

Answer 101

mccune albright: cafe au lait, fibrous dysplasia and endocrine- hyperthyroidism and hyperpara, and cushion and precocious puberty, hyperprolactin bardat biedel - truncal obesity infancy with vision loss due to rod cone dystrophy, post axial polydactyl, gu and renal abs abetalipproteinemia is dietary fat absorption issue and ADEK, AR with low ldl levels and steatorrhea fat euro issues and acanthocytosis, retinits pigments

Question 102

male puberty leydig from LH - testosterone make pubes and penile growth FSH to sertoli - go to seminiferous tubules and make testicular growth age 9-14 females gnrh pulse lh and ash go to granulose cell and estrogen and and=rigen made which causes breast development uterine grwoth and pubic hair ages 8-13

Answer 102

pubarche or adrenarch is driven by the secretion of androgens by the adrenals and that gets pubic hair and acne from here - boobs pubes grow flow - get boobs then 2 y later get menarche the HPG axis - to gonads for females get breast development and menarhc for males get penile size and testis enlargement order for males is testis then pubic hair

Question 103

``` precocious puberty in males -alway pathological CENTRAL - HPG driven if central - all males get an MRI as hypothalamic hematoma (with gelastic seizures) - causes LH/FSH to go up ``` FOR GIRLS IF Under 6 yo then MRI other causes include any Cns cause cysts trauma meningitis cerebral malformatins primary hypothyroidism

Answer 103

peripheral precocious puberty- doesn't follow expected path of puberty ie - for male testis is not the first thing to get large 3 sources - testosterone and estrogen - this feeds back to DECREASE LH/FSH 1- exogenous sex hormones 2- gonads 3- ectopic from Germ cell tumor

Question 104

pulse oximtery R hand and either foot 24-36 hours a pass is >95% with less then 3% diff Whats a fail?

Answer 104

<90 rpt x3 with an hour in btwn or 90-94 with >3% diff, rpt x 3 with an hour in btwn if fail do ecg, xray and 4 limb BP difficult for coarct- ensure femoral pulses checked

Question 105

HSP diagnosis small vessel IgA vasculitis ``` normal or HIGH platelet mild anemia, WBC elevated low albumin elevated markers Cr may be higher elevated IgA ```

Answer 105

purpura +2/3 of arhritis, abdo pain and renal disease ttreatment with NSAID and suppprotve - steroids only if GI or severe renal disease and taper slowly recur 30% in first 2 months. no permanent damage from arthritis check urine and BP -q1week x 1 month, q2 weeks x 2 months, an monthly for 3 months -ERSD 1-2%

Question 106

KD poor prognostics medium size HOT CREAM - diagnosis 4/5 CREAM criteria ``` conjuncvititis rash - extremity changes Adenopathy- unilateral - cervical LAD >1.5cm mm changes strawberry tonge ``` other manifestation - hydrops of gallbladder, aseptic meningitis, urethretis, anterior uveitis, SNHL cardiac manfiestation, do at time and rpt 6-8w Mg't - 2g/kg IVIG, low dose ASA (3-5mg/kg/d)

Answer 106

poor prognosis-age <1yr, >9yr, male, hypoalbuminemia, low plt, prominant inflm markers, duration of fever long 3 layers of vascular wall destructing internal elastic lamina (weakens vessels) Iga plasma cells go in , can form thrombi 3 phase- acute (fever and perineal desquam) subacute - plt elevation and desquamation, high risk of sudden death - lasts 2 w then convaslecnt till ESR down (2 months) investigations: neutrophilia, anemia, thrombocytosis, low albumin, transminitis, hyponatremia, sterile pyuria, pleocytosis of CSF

Question 107

Diagnosis of JIA (F>M) mono - 1-4 within 6 mo poly>5 within 6 mo systemic M=F +1/4 - bioligic for il1/6 (anakinra, tocilizumab) entethesis M>F +2/5 psoriatic +1/3 INV: cbc, esr, crp, RF, ANA, PPD, aspirate jt RF for uveitis :younger age, girls , positive ANA, oligo (HLA DR5/6/8) general: RF+, systemic, poor therapy response, erosions on xray tx- NSAID, jt injection, then DMARD (MTX) then biologics TNFalpha blocker infliximab or etanercept complications - synechiae, leg length discrep, atrophy, contractures, psychosocial, MAS (cytopenia, high ferritin , low fibronogen and LOW esr, high TG, high CD25) with tx - mtx (monitor CBC and lfts q 3 mo) and no live vaccine, no septra, no preg with anti tnf - get tb reactviation, do cxr and tv, pancytopenia, MS like sx

Answer 107

must be<16yo, with arthritis x6weeks mono < or equal to 4 jt involvements within first 6 months of disease (uveitis RF-female- traet with NSAID) poly (RF NEG more common) >5 in 1st 6 mo of diagnosis, RF positive tend to be adolescent with wrost prognosis systemic: min 2 week fever with 3 days consecutive with 1 or more of: HSM, rash (evanescent, salmon), serositis and LAD entethesitis (arthritis or entethesitis) with 2 of: HLA b27, sacroilliac tender, age M>6, sx ant uveitis, 1st degree relative with anklyosing, entehsis,s acro, ibd, reiter,) psoriasis - psoriasis and arthritis and 2 of: dactylitis, nail pit, 1st deg relative of psoriasis) - usu arthritis is first uveitis monitoring if <6 and positive ANA with <4years of disease then 3 months, but >7 years then q12 month basically - q3-12 month based on age, ANA status and disease duration

Question 108

lyme disease bug early and late manifestations treatment if just erythema migran - (3-30d) then doxy>10yo or amox if <10 x 2 weeks. Ceftriaxone if any disseminated findings or late findings

Answer 108

borrelia burgdorferi screen with ELISA - and confirm wtih western blot - also PCR early: uveitis, carditis, facial nerve palsy CN7, lymphositic meningitis, arthralgia, heart block late - arthritis, enecephalomyelitis (chronic), acrodermatitis chronica

Question 109

rheumatic fever diagnostic criteria JONES CAFE PAL need 2 + 1 (major and minor or vise versa) + ASOT or positive culture ``` Joints (migratory polyarthritis) O - pancarditis N - subcut nodules E- erythemaga marginatum - serpigneous rash S - syndeham ``` Minor- cafe PAL crp, arthralgia, fever, esr pr interval, anemesis of rhemaism, leuko

Answer 109

treatiwht 10 days pen need prop pen for 5 years or 21 years old - whatevr is later if carditis - life long treatment with prop

Question 110

hip pain 2-6 yo transient M?F 4-10yo leg vave perhtes AVN- diagnose MRI, bone scan ina cute stage 10-14 SCFE (does not casue AVN itself, only pinning does) - external rotation with hip flexion

Answer 110

diagnosis of SLE - MDSOAP BRAIN 4/11 malar rash/discoid rash/serositis/Oral ulcer painless/Arhtirtis/Photo sens Blood - leukopenia and anemia and thrombocytopenia R-renal disease - protenuria ANA positive Immune - anti dsDNA, anti Sm, FALSE positive RPR- dt antiphospholipid ab, positive lupus coagulant Neuro - range, enecophalitis, CVST, chorea can also have low c3/c4 with activei disease monitor ESR for disease CRP is NORMAL - if high infxn or serositis/pericarditis tx- NSAID and hydroxychloroquine (see optho q6 mon), low dose pred if sever then high dose steroids - immunosupp (MMF), rituximab), cyclophosphamide for renal disease

Question 111

med side effects drug induced lupus - minocycline, phenytoin, MTX - liver and cbc q 6 months hydroxychlorquine (plaquinil) eye exam q6 mo for color blindness and retinal toxicity tacro - liver and kidney , hyper K, hypoMg cyclosporine- high blood pressure, headache, kidney problems, increased hair growth, and vomiting.

Answer 111

cyclophosphamide - skin hyperpig, hemmorhagic cystitis, BM suppresion, vomitting, alocpecia

Question 112

Charactersitics of neonatal lupus antiphospholipid antibody syndrome - TRIAD recurr miscarriage, thrombocytopenia and thombosis (+/- hemolytic anemia) can hve long PTT anticardiolipin antibody and assoc with lupus

Answer 112

``` anti ro and anti la ab skin - discoid rash brain -hydrocephalus heart block - hepatitis panyctopenia, stippling og epiphysis ```

Question 113

Diagnosis of JDM NOT assocaited with cancer 3D's dysphonia, dysphagia, dyspnea can get calcinosis usually do MRI Treatment- avoid sun, MTX, steroids, Ca Vit D IVIG adjunct if weakness severe

Answer 113

classic rash (heliotropic or gottron) + 3/4 weakness, symetric and proximal muscle enz elevation CK AST LDH aldolase EMG change of fibrillation and sharp wave muscle bx - necrosis and inflammation ask about difficulty getting out of chair, climbing stairs, brushing hair, positive gower sign complications if nail fold disease persist - may take longer to remit DM2 - due to lipodystrophy contractures, calcinosis, s/e of steroids

Question 114

granulomatosis with polyangiitis GPA this is small vesssle disease C- ANCA necrotizing granulomatous treat with steroids, Cyclophosphamide, imuran, retux MTX

Answer 114

saddle deform nose, sinusitis and nasal ulcers, EPISTAXIS, with infiltrates in lung and pauci immune GN CONSITITUTIONAl sx, and eye inflm (episcleritis), vasculitis rash MPA (microscopic) is similar with less SINUS but P ANCA is positive instead churg staruss is allergic small vessel necrotizing granulomas hx of refractory asthma and periphera Eo

Question 115

Diagnosis of periodic fever PFAPA - 3-5yo, 3-6 weeks MAX 5 days, resolve with steroid x1, T+A, anakinra (IL1) FMF: <5yo, 1-4d plsu (arthritis, peritonitis, pleuritis) with erisipelas rash, hypothy, amyloidoisis- colchicine daily, anakinra, can cause hearing loss (in jew, turkey, free) for pyrin protein

Answer 115

recurrent UNEXPLAINEd at least 3x/6 months, associated with at leaast a week of being well in btwn with NORMAL growth HIDS (hyer IGD) in babes<6mo, with 3-7 d fever and diarrhea with ulcers, rash, flare at Bday, holiday and vacation and have mental retard, cataract and FTT with INC mevaolnate (DIAGNOSIS) TRAPS - AD, first decade fever 3d upto weeks, wioth nausea, diarrhea, myalgia, rash migrating distally, treat with NSAID or etanercept (TNF against) CAPS - cryopryin like NLRp3 assocaited early infancy usually resolve 24 hours, hands knees and ankles

Question 116

You see a biopsy with non caseating epithelioid granulomatous lesions - whats teh diagnosis

Answer 116

sarcoid - children<4 for early onset triad rash, arthritis and uveitis bilateral hilar lymphadenopathy

Question 117

differential for uveitis

Answer 117

``` IBD SLE JIA CMV/HSV bartonella - unilateral syphillis TB sarcoidosis ```

Question 118

differential for erythema multiforme

Answer 118

``` mycoplasma ebv cmv NSAID sulfonamide hsv HIV IBD SARCOID ```

Question 119

Position plagiocephaly RF and management side note KOCHER criteria - 1) fever, no weight bear, WBC>12, ESR >40 septic athrits treat abxx21 d with ANCEF, may add clinda vnaco if MRSA

Answer 119

worst at 4 month then resolve by 2 y rf: male, bottle feed, congenital torticollis, no tummy time, supine sleeping, first born management: tummy time 3x/d x15min, altenrate sleep position to prevent helmet moulding on max age 8 months

Question 120

indications for surgery for scoliosis common is r thoracic and l lumbar, PA view IF ITS L thoracic - need MRI some findigns include elevation of shoulder, lateral trunk shift, apparent leg length discrepency test via ADAM forward bend and ribs are prominent on convex side as vertebral bodies go in that direction if its congenital MUST DO RENAL U/S to rule out renal ageneiss, horse shoe kidney, genital abnormalities

Answer 120

``` indications for surgery prepub and cob>45 post pubert and cob >50 progressing despite brace >5 on cob angle cosmesis unacceptale or affecting QOL ``` complications of sugery- blood loss, SMA syndrome, infection, neurological injurty, pseudoarthrosis (fialure of fusion bracing for >30 (ineffecitve at more then 45) observe if less then 20

Question 121

name four causes for congenital scoliosis and acquired scoliosis

Answer 121

congenital - tumor of sternocleidomastoid, branchial cleft cyst, clavicle fracture, hemivretebrae, posterior fossa tumor acquired - infxn - rpa, tb, lemierre ......cancer posterior fossa tumor...s04 palsy, clavicle fracture,, ligamentous injury, polio, wilsons, myesthenia gravis

Question 122

diagnosis with athlete pain on back extension versus flexion management remember young ppl at risk due to 1- incomplete ossification of pars interarticularis inc risk of injury 2- growth rapid which can lead to poor techniqe 3- over training

Answer 122

extension - spondylolysis - seperate of pars interarticularis - pain on extension, hamstring tightness, and paraspinal muscle spasm (if vertebra anterior slips as well then called spondylolitsthesis) mg't, physio, rest, 2 month rest with brace, 6 month without posterior element overuse syndrome same presentation and investigations negative treatment is physio rest with or without brace and better by 2 months flexion disc herniation - rare in child - surgery if cauda quina, neuro sx, or refractor pain and also positive leg test is diagnostic vertebral avlusion fracture - displacement and 3 of disk into spinal canal and xrays show it - ct imaging choice and excise fragment if neuro signs takes 3-6mo

Question 123

SCFE @ hypertrophic zone of physis - limp external rotation abduction of hip limp- PAIN IN KNEE/thigh diagnosis L more then R RF-hypothyroid, hypoparathyroid, renal osteodystropohy, obesity investigations management - pin it, mild<30, severe>60deg, stable if child can walk with or without crutches. monitor with serial xray to ensure stable and physis closing prognostics/complications - AVN - chondrolysis - femoroactebular impingement distinguish from salter harris1

Answer 123

slipped capital femoral epiphysis - 11-16 yo obese black, can be acute/acute-chronic/chronic - acute - <3w with groin/knee pain with maybe minor injury , distinguish from salter-harris1-high risk AVN - chronic most common - inv: BOTH HIPS-xray ap and lateral frog leg - draw klein line from femoral neck should hit the epiphysis. shows post migration of femoral epiphysis and remodel of neck with upper femur bending neck, wide irregular physis - younger child <10yo look at endocrine abn - thyroid pit, GH, hypogonadism

Question 124

RF for DDH L hip more common neonate- barlow and ortalani untreated - scoliosis, limb length inequality, arhtritis, hyperlordosis if bilateal, ipsilateral knee pain

Answer 124

first born, female, breech, family hx, any tight intrauterine space - oligo, large weight, twins, swaddling tight-acquired by bringing hips together

Question 125

exam on ddh 4 of them neonate - 1) abduct hip restriction red flag, 2) barlow positive clunk, ortalani clunk into place gone by 3 months 3) positive galeazzi sign - apparent shortening of thigh 4) gluteal thigh fold asym older child walking- painless limp

Answer 125

before 6 mo screen with u/s, if they have high risk baby if BREECH and FHMX consider wait 6 weeks if they have a neg or equivocal exam xray if >4-6mo as femoral ossification there now tx <6mo pavlic harness with serial u/s qw abort if no change in 3w, and complications of AVN and femoral nerve palsy older- closed reduction 6m-2y >2yo open reduction with cast spica x6-12w

Question 126

leg calves perthes - will hv symptoms of pain in thigh and groin and limp age group; xray findings management RF prognosis- worst is older girls complication; leg length discrep, muscle atrophy, hip flexion contracture

Answer 126

4-8yo age group, M>F avn of the femoral head continue rom and dec weight bearing petrie casts -internal rotation and abduction NSAID for pain surgical containment - varus osteotomy

Question 127

Diff and similarities | btwn SCFE and Legg perthes

Answer 127

age group LPS- 4-8yo, SCFE 11-16 LPS - pain in groin, SCFE pain in KNEE SCFE associated with endo findings (esp in the young) Similiarities - limp, AVN involved, both more common in males

Question 128

RF for AVN

Answer 128

``` steroids alcohol sickle cell SLE trauma, antiphospholipid ab gaucher disease (peroxisomal/lysosomal d/o) ```

Question 129

Differential for knee pain 1) discoid meniscus 2) osteochondritis dissecans 3) patellafemoral syndrome 4) Osgoode schlatter - repdoruce pain with squatting, ant knee pain. xray shows soft tisse ant tibials welling, calc patellar tending- DONT avoid activities and no steroids (complications persistant paina nd prominence, knee hyperextension rare)

Answer 129

1) discoid meniscus (high risk mensical tear, incidental finding, snapping knee if unstable - shave it down) 2) osteochondritis dissecans - disrupted blood supply to a piece of bone, causing focal ncrosis, good if open growth plate and self limited) 3) patellafemoral syndrome-adol girls, relief with extension 4) Osgoode schlatter - pain at tibial tubercle (9-14yo) rapid growth and active kids M>F

Question 130

osteo - usuall staph with | neonate - GBS, <5yo kingella, sickle salmonella and bartonella in pelvis and verebrae

Answer 130

usually femur then tibia, usually long bone tx ancef +/- clinda or vanco if sickler then cefotax and clinda/vanco with 3-6w

Question 131

Differential of varous Rickett findings- craniotabes, frotnal bossing,r achitic rosary, widening of wrists and ankles (cupping metaphysis)

Answer 131

1) physiological: RF obese, fmhx, early walker, resolve 2yo 2) Metabolic bone disease- Ricketts 3) blount disease - medial grwoth plate is diseased-progressive

Question 132

age it shows, exam, resolving metatarsus adductus internal tibial torsion increased femoral anteversion

Answer 132

MA - birth to one yaer, heel bissector line LATERAL to 2nd toe, package issue, resolve by 1yo ITT- 1-3 yo, gone by 5yo, thigh foor angle, neutral or internal (Usually its 10 deg EXTERNAL), gone by 5 yo IFA- W sitting, increased internal hip rotation, inc angle btwn axis femoral neck and transcondylar axis of femur, usu 3yo, gone by 11 yo, F?M

Question 133

four exam findings of talipes equino cavo varous CAVE TEV- talipes equinas varous

Answer 133

clubfoot ``` hindfoot equino deep posterior crease empty heel hindfoot varous midfoot cavus ``` surgery 3-6mo of age with percutaneous tendoachilles tenotomy Ponseti method - serial casting with boot and denis browne bar - full time 3 months

Question 134

Precocious puberty <9 boy, <8 girl Central - follows expected path (male: hemartoma- testis first), FSH and LH high Peripheral random path - ADVANCED bone age, if hair usually adrenal, low FSH and LH, exogenous, adrenal or gonad, or ectopic production of germ cell tumor. r/o premature therlarche and adrenarche that are BENIGN (bone age = height age, NORMAL growth velocity)

Answer 134

``` workup for precocious puberty BW: FSH LH, testosterone, DHEA-S, androstenedione GnRh stim TSH MRI if boys, or <6yo girls ``` treatment is LUPRON if central - GnRH analog if McCUNE albright (peripheral cause) precocious puberty, fibrous bone dysplai and cafe au lait then need anti H treatment

Question 135

DSD in males and females differential Females CAH, viritizling maternal disease, maternal andorgen use, gonadal dysgenesis W/u - 17 OHP, serum lytes, glu, ACTH, renin, testosterone, FSH and LH

Answer 135

``` Males leydig cell failure 5 alpha reductase deficiency androgen receptor disorder gonadal dysgenesis CAH rare forms ``` lab: testosterone, dihydrotestosterone (DHT), LH FSH MIS, lytes and glucose

Question 136

delayed puberty >12 with no boobs females>14 males female - think turner male - think klinefelter Normal ages female 8-13, male 9-14 yo inv: biochem, bone age basal serum LH, FSH, IGF1, fT4 (testoserone in boys), if FSH high then do karyotype if low LH or FSH - and prepubertal growth rate - GnRH defieincy or CDGP (consitutional delay of growth and puberty) Treatment - males replace testosterone, females low dose estrogen then cycle E and P (needed for bone haeltha nd psych)

Answer 136

divide into 1) Hypogonadotropic hypogonadism (permanent: KALLMAN (anosmia) or tumor (craniopharyngioma) or transient: constituional delay of growth and puberty, functional : AN, chronic illness, hypothyroidism) 2) Hypergonadotropic Hypogonadism: high lh/fsh, low testosterone and estradiol (KARYOTYPE) - OVARIAN failure: POF, Turner, dysgenesis, AIS - testicular: klinefelter, torsion ,infection (mumps)

Question 137

for primary amenorrhea 1) hypogonadotripic hypogonadism again - aka tumors, kallman or chronic disease issues 2) hypergonadotropic - turner again, POF, AIS 3) structural 4) other hormones -thyroid or prolactin

Answer 137

w/u do US- if uterus absent - then karyotype and serum testosteron if uterus there - FSH, karytype, prolactin tsh, testisterine DHEAS, 17OHP +/- MRI secondary amen - PCOS, chronic disease, anovulatory, thyroid, POF, also has to be more then 3 months (or 6months if they are irregular) PCOS- triad hyperandrogens, anovulation and cysts of ovaries on ultrasound

Question 138

when do primitirve reflex go and which one comes on at 4-5 mo and 9-10 month

Answer 138

by 6 mo, moro grasp and rootting are gone and fencing with drunk incurve parashut 8-10 month and for life landau comes 4-5mo then GONE by 15 month-2yo (held prone horizantally and they go straight out)

Question 139

when to image a spinal dysraphism myelomeningocele - aperta worst form need surgery, vs tethered is when spinal cord goes to L2

Answer 139

>25mm from anal verge or larger then 5mm, mass or lipoma hairy patch, dermal sinus, vascular lesions

Question 140

diagnosis and management of migraine =2/5 SULTANS for 5 attacks lasting 1-72 hours vs tension headaches dont have n/v and have only ONE of photo/phonophobia, and not aggrevated by activity , can tx with ibuprofen and prevent with amitriptyline treatment abortive - ibuprofen, tyl, triptans preventative if more then 1 a week and affecting function: lifestyle, Mg, sandomigran, propanolol, ccb, VPA, topiramate, amitrptiline , cryoheptadine, coenzyme q10, riboflavin, butterbur for 4-6mo THEN WEAN

Answer 140

severe, unilat, throbb, aggrevated by activity, plus nausea or sensitivies and not from another cause , TX IM keterolac antiemetic and consider steroids with iv fluids and sedation childhood variants 1- abdominal migraine 2- cyclical vomitting (5 attacks, or min 3 in 6mo), reccurent vomit lasting 1h-10h, one week apart, stereotype occurs 4x/h for 1 hour, sterotypical pattern for patient, return to baseline btwn and not another condn 3- benign paroxysmal vertigo - child appears frigehtnened, n/v/diaphroetic , gone by 6yo, normal workup 4- hemiplegic migraine - r/o stroke, focal defect precedres h/a by 30min - chanelopathy confirmed

Question 141

diff btwn partial and complex partial seiure partial - no LOC, one body part vs complex consciousness is impaired and have focal neuro defects, can have pre aura and post ictal state UNPROVOKED seizure, need EEG, reccurence 40%, usu within 6mo, and if theres abn neuro exam high chance of recurrence

Answer 141

what are three benign neonatal seizure conditions 1- idiopathic neonatal convulsions- fits - 5th day, gone by 6w, no fmhx, CLONIC 2-3min 2- benign familial neonatal convulsions - 30x/day, TONIC fits, start 1 week gone by 6 month, channelopathy with family hx, normal perinatal hx - no tx 3- neonatal infantile myoclonus at 4-6mo, happens only in sleep, gone by 5yo, ensure not infantile spasms

Question 142

infantile spasms ``` tx vigabatrim (renal tox, dec peripheral vision) ACTH steroids, bz valpraote ``` can lead to lennox gastaut where have diff seizure types 1-2hz spike and slow wave, bursts in sleep and slow background in wakefullness

Answer 142

brief contraction followed by sustained muscle contraction - 3 subtypes and 2-125 spasms 13 per min with eeg showing hypsarrythmia (high voltge and slow chaotic background with multifocal spikes) peak 8 months, regression and devt delay cause TUBROUS, DOWNS, sturge weber, torch, aircardi syndrome (no corpus)

Question 143

abscence seizures 3hz spike peak 6yo treat with ethosuximide or valproic and usu resolve by adolescnece, common to hv psych or beh comorbidities

Answer 143

rolandic epilespy, 5-10yo, nocturnal hemiface and 1-2 min with guttural drooling and arm - CONSCIOUS AND AWARE, centrotemporal can give carbamazepine if more then 3 seizures self resolve by puberty

Question 144

``` febrile seizure -<15min - 1 in 24 hour GTC -6mo-6yo, no post ictail neurological n child ``` chance recurr 50% if<12mo, 30% if older, within 1-2years epilepsy risk goes from 1 to 2% RF to develop recurrence: <1yo, fever <24 h and fever 38-39deg (minor-fmhx, daycare,male, complex, low Na) for Afebrile seizure -LP<6mo with afeb seizure, consider uine and stool cx, EEG, neonate metabolic w/u, CT vs MRI (urgent if post ictal deficit or focal neuro deficit-r/o stroke)

Answer 144

RF to develop febrile seizures: nicu stay 28d, developmental delay, fmhx, and daycare developing epilepsy - complex seizure disk of epi 6%, if focal 30% and if fever<1hr before fever 18%, neurodev abnormality, family hx investigate if complex/high risk of epilepsy, eeg and imaging and prn antiepileptics status = >20min seizure or intermittent without return to consciousnes x30min, mortality<10% mg't abc, oxygen IV access, glu and lytes, AED levels, loraz 0.1mg/kg x2 doses, tx pyridoxine (neonate b6), LP if <6mo afebrile seizure,EEG 24 h later

Question 145

dianostic criteria for nf-1 (AD) 2 of CROPLAND at risk of moya moya, leykemia cns tumor, htn, wilms, rhabdomyoscarcoma, pheo FASI - bright areas on MRI go away with age ``` mg't annnual optho till 10 bp monitor scoliosis regulular neuro psycho ed ```

Answer 145

``` Cafe au lait >6, 5 and 15mm relative optic glioma pseudoarthrosis lisch nodule ax freckle neurofibroma or plexiform dysplasia of sphenoid ``` can also have learning diseability, adhd seizure remember nf2- merlin issue with vestibular schwanoma, meniingioma

Question 146

TSC tuberous sclerosis diagnosis with A LA GRASS HUT 2 major, or 1 maj, 2 minor AD - tuberous and hamartin gene IS as baby assoc with renal carcinoma nad brain tumor, autsim, seizurs, cardiac failure, autism do mri q1-3y, renal mri/us 1-3y, neurodev teting grade 1 optho yearly htn and gfr yearly ``` MINOR - CHORRD Confetti skin dental enam pit oral fibroma retinal achrom patch renal cyst hemartoma non renal ```

Answer 146

minor: cerebral white matter radialmline, ginvigial fibroma, rential achromatic, dentla pitting, confetti skin, non renal hamrartoma, bone cysts, renal cysts adenoma sebacium lymphangiomyomatosis of liung ash leaf spot ``` giant cell subepyndmal astrocytoma rhabdomyoma heart angiomyolipoma kidney shagreen patch subependymal nodules ``` hamartoma of retina ungal fibroma tubers

Question 147

sturge weber - angiomatosis of leptomengingis, causes glacuoma nad port wine stian of v1v2 of CN5, also get seizures, hemo paresis, headache. dev disbality, hemianopsia

Answer 147

``` PHACES post fossa hemagnioma arterial anoalies coarct aorta eye abn sternal cleft/supraumbical raphe ```

Question 148

bells palsy features and treatent usu 2./3 ant tonge cant taste ramsay hunt is when TRIAD facial paralysis, ear pain and vescile - treat with acyclovir too mobieus is congentail 6,7 palsy also cn7 bilateral palsy pahtopneumonic for lyme disease

Answer 148

cn7 palsy, does nto spare foreherad bugs: hsv,vzv, ebv, lyme, mycoplasma and mumps 2w post infection STEROIDS 1MG/KG/D x1 w then taper, and can give qacylovir, METHYLCELLULOSE drops in eye to protect against keratitis 90% recover sponatneous can also happen post delivery, give artiifical tears

Question 149

congenital absence of the depressor angularis oris muscle - no treatment, affected side gets pulled toward good side, desnt effect feeding, no intervnetion can have heart/resp/msk/gu but if normal exam no workup needed

Answer 149

differnetial for esotropia - cn6 palsy, tumor. trauma from basal skull #, inc icp. get horizantal diplopia when looking towards the paralytic rectus muscle r/o myesthenia gravis

Question 150

findings of syndeham chorea (lysch nyhan too), in huntington AS ADULT but as kid have dystinia and ataxia and parkinsonism - hyperkinetic, issues at rest but worst with intention, 1 month after ifxn - spooning, emotional lability, darting tonge, ballismus, milkmaid grip, low tone, normal sensation, pronator sign and choreic hand

Answer 150

tourette diagnosis and treatment ``` 1- tic motor and verbal everyday for a year - change over time 2. 18yo or older 3. recorded and witness 4. no tic free for 3 months treat tic- clonidine (alpha2 adrenergic agonist) ocd- ssri adhd stimulands life long max in adolescent ```

Question 151

TSC tuberous sclerosis diagnosis with A LA GRASS HUT 2 major, or 1 maj, 2 minor AD - tuberous and hamartin gene IS as baby assoc with renal carcinoma nad brain tumor, autsim, seizurs, cardiac failure, autism do mri q1-3y, renal mri/us 1-3y, neurodev teting grade 1 optho yearly htn and gfr yearly

Answer 151

minor: cerebral white matter radialmline, ginvigial fibroma, rential achromatic, dentla pitting, confetti skin, non renal hamrartoma, bone cysts, renal cysts adenoma sebacium lymphangioleiomyomatosis of liung ash leaf spot ``` giant cell subepyndmal astrocytoma rhabdomyoma heart angiomyolipoma kidney shagreen patch subependymal nodules hamartoma of retina ungal fibroma tubers ```

Question 152

sturge weber - angiomatosis of leptomengingis, causes glacuoma nad port wine stian of v1v2 of CN5, also get seizures, hemo paresis, headache. dev disbality, hemianopsia

Answer 152

``` PHACES post fossa hemagnioma arterial anoalies coarct aorta eye abn sternal cleft/supraumbical raphe ```

Question 153

bells palsy features and treatent usu 2./3 ant tonge cant taste ramsay hunt is when TRIAD facial paralysis, ear pain and vescile - treat with acyclovir too mobieus is congentail 6,7 palsy also cn7 bilateral palsy pahtopneumonic for lyme disease

Answer 153

cn7 palsy, does nto spare foreherad bugs: hsv,vzv, ebv, lyme, mycoplasma and mumps 2w post infection STEROIDS 1MG/KG/D x1 w then taper, and can give qacylovir, METHYLCELLULOSE drops in eye to protect against keratitis 90% recover sponatneous can also happen post delivery, give artiifical tears

Question 154

findings with lamboid craniosynostosis - ridging affected suture- ispialteral occipitomastoid bossing, posterior ear displacement with metopic- u get traingle shaped head, with hypotelorsim and recessed lateral orbits repair 8-12mo

Answer 154

septo optic dysplasia - optic nerve dysplasia - absent septum pellucidum -agenesis of corpus callsum -migration anomalies -encephalomalacia posteiror pit ectopia mri and endo eval

Question 155

findings of syndeham chorea (lysch nyhan too) - hyperkinetic, issues at rest but worst with intention, 1 month after ifxn - spooning, emotional labitliy, darting tonge, ballismus, milkmaid grip, low tone, normal sensation, pronator sign and choreic hand

Answer 155

tourette diagnosis and treatment ``` 1- tic motor and verbal everyday for a year - change over time 2, 18yo or older 3. recorded and witness 4. no tic free for 3 months treat tic- clonidine (alpha2 adrenergic agonist) ocd- ssri adhd stimulands life long max in adolescent ```

Question 156

patient presetning with ? diagnosis 1- 4-8 yo with learning disability and beh problem, diagnosed as adhd, and then progressive decline, blindiness, quardiparesis and adrenal insufficeincy

Answer 156

adrenoleukodystrophy- neurodegeneratve disoder sphingolipidoses can also have defecting cellular membranes due to problem of lipid substrates - ie) krabbe diesease

Question 157

diff btwn chiari 1 and 2 - cerebellar tonil through foramen magnum chiari 1 - no hydropcephalus (caused nf1, rickets, craniocynysotosis) also has reccurent headche, urinary freq, tinnits, central sleep apnea and lower limb spacisity chiari 2 has progressive hydrocephalus and paralysis below defect

Answer 157

dandi walker similar to chiari 2 but expansion of 4th centricle and needs a vp shunt

Question 158

RF for brachial plexus injury and the types 1) LGA, GDM, shoulder dystocia, prolonged labour and insturmentation usu resolve b 4w, 25% more permament, if not gone refer to brachial plexus team c3-5 diaphram, phrenic nere c5-t1- brachial plexus, shoulder inneration, upper arm, forearm and hand t1- sympathetic fibre, horner (ptosis, miosis and anhidrosis)

Answer 158

erbs palsy c5-7 ;HAND AND WRIST PRESERVED, adducted uper arm and internal rotation with forearm extended (weak deltoid, infraosinatus and bicep) klumpke palsy-c8-T1: rare with absent grasph and claw line wiht intact bicep paralyszied hand and horner total injury- WORST and horner

Question 159

findings with lamboid craniosynostosis - ridging affected suture- ispialteral occipitomastoid bossing, posterior ear displacement with metopic- u get traingle shaped head, with hypotelorsim and recessed lateral orbits repair 8-12mo

Answer 159

septo optic dyspalsia - optic nerve dysplasia - absent septum pellucidum -agenesis of corpus callsum -migration anomalies -encephalomalacia posteiror pit ectopia mri and endo eval

Question 160

functional residual capacity = residual volume (what is left after you breathe out) and expiratory reserve volume anything that DECREASES COMPLIANCE will decreased fxnal residual capacity hence- no surfactant, edema, fibrosis all dec FRC frc increased in asthma due to gas trapping\

Answer 160

vital capacity is what you breathe in and out regularly - which is your TLC without the residual volume lungs are grown by 8yo

Question 161

wht is differential for obstructive lung path versus restrictive

Answer 161

obstructive - CF, asthma, BO restrictive - obesity, scoliosis, PF, ILD, neuromuscular - remember its all low fev1,tlc,vc,fef25/75, flow cruve, whats normaL is fev1/fvc, rv, rv/tlc

Question 162

lights criteria think exudate high protein and LDH protein in fluid >3g/DL ldh . in fluid >2/3 upper limit protein/serum ratio >0.5 ldh/serum ratio >0.6 for transduate is just LOWER THEN for the values

Answer 162

``` diagnosis of asthma in preschooler 1- documentation of airflow obstruction preferred by a physician 2-reversibility of obstruction (with saba, or with 3 mo ICS, or with parent report 3- no otiher cause ``` if older then 6 then 1) less then lower limit for fev1/fvc based on age, height, sex and ethnicity (bp need sex age and height) <0.8 and saba response 12% or 2) inc peak expiratoy flow of 20% post contolller therapy 3) meth challenge pc20<4 or 15%dec fev1 with exercise nitric oxide cannot be used

Question 163

asthma control target . SPEND BFP ``` school absence none physical activity not limited exacerbations none night time sx <1/week daytime sx <4 per week ``` beta agonimst use less>4/w fev1>90% OF BEST Pef diurnal chage <10

Answer 163

lack of control for asthma is the opposite AND ALSO fev1<80%, day sx>2d/w need steroids 2x/year reduction of lung growth or loss of fxn RF FOR PERSISTANT ASTHMA -parental asthma, allergy, male, LBW, tobacco smoking, wheeze outside of colds, lrti, chorinated pool, dec lung fxn at birth RF FOR MORTALITY 1) icu admission or intubatin 2) resp dsitress, 2+ hospitlizations in the year, LBW, inc PEf diurnal variations, use of >2 canisters of SABA 3) male, LBW, non white/black, sensitivity to alternria ENVT - allergen exposure, smoke, air pollution, urbanenvt, low SES, crowding, mother <2yo, mom not highschool educatied, SA, psych issues, inadequate medd care

Question 164

Asthma med management - check control, spirometry, inhaler technique, adhenrecen, trigger, comorbidities 1-5yo LOW dose ICS = 100-125, medium 200 6-12yo - saba, then low dose ICS upto 200mcg/d - then medium dose (upto 400mcg per day) then add LABA or LRTA >12yo, saba, then low dose ICS upto 250, then LABA or LTRA, then LTRA and increase to med dose upto 500mcg laba - formoterol and salmetorol combo - advair (flutic and salmet) symbicort (budesoninde and formoterol) zenhale (formotorol and momethasone) ipatroprium - antichoilnergic, bronchodlate increase fev1 by 10% if added, best within 4 hours LTRA (block cysteinyl-LTI receptor) monteleukast - modest broncholate, dec mucus, dec vascular perm, and Eo recruitment

Answer 164

s/e and mechanism SABA - bronchodilate via smooth muscle relaxation, inc mucus clearance, dec vascular permeability and edema tachy, hypokal, tremor LABA always with steroid-can cause tachyphylaxis (salmeterol and formoterol - this one works wihtn 2min) steroids - dec cytokine production and infl cascase, dec mediator release of macrophag and Eo, inc b2 receptor expression, inhibitit Eo and Lymph production (remember alvesco, fluticasone and qvar same dosing BUT pulmicort just double it) s/e- hoarseness, thrush contact hypersensitive, adrneal supp, lung fxn, glaucoma, loss of 1cm, osteoporosis/penia worst case omalizubmab- watch for ANAPHYLAXIS must do in office MDI - helps with delivery of med, decrease cordination and minimize s/e (breath regularly for 5-10breaths or 30sec

Question 165

CF - delta F508 mutation EARLY findings- hyperechoic bowel on u/s, jaundice, delayed meconium passage, edema, hypoalbumin, acrodermattis enteropathic, hemm disease of newborn SKIN- nasal polyp RESP - chronic infxn, pneumothroax, ABPA, bronchiectasis, pft obstrructive first then restrictive GI - gerd, intussuceptin, DIOS, meconium ileus, rectal prolapse GU - thick cervical plug, azotemia, other- jaundice, clubbing, ADEK def finding, hyponatremic, hypochloremic alkalosis

Answer 165

bugs - pseudomonas, b cepacia, staph aures, aspergillus, stentotrophomonas, achromobacter - tx tobra and ceftaz (or pip taz, cefipime, meropenem) diagnos prenata- NBS, trypsinogen or clinkcal feautres, or CF sibs or POSITIVE NBS elevated 2 sweat chlorde, or 2 mutations or abn potential diff measurements of nasal GOLD STD diagnosis i sweat test, >60meq/L diagnositc FP - think endo and skin stuff - eczema, ectodermal dysplasia, low albumin, AN, CAH, AI, g6pd. DI, metabolic, FN - technique, dilution, malnutrtion, hyponatremia, edema and hypoalbumin

Question 166

CF treatment 1- resp - bronchodilator, human recomgibant dna, hypertonic saline, chest physio, acute execerbation tx x2w, if pseudomonas add azithro and inhaled tobra ongoing 2- gi, high fat and high energy wiht pancreatic enxymes and ADEK

Answer 166

complications 1- chest= hemoptysis, atelectasis,pneumothroax, abpa (ige and spt), resp failure, ntm, core pulmonale, ftt live till 53- better if BMI improved wiht maintained fev1 remember PCD similar but NO RECC AOM, CILIARY ISSUE, atlectasis on xray, stuffy and rhinitisz, 505 kartagener, EM of ciliar diagnostic

Question 167

htn guidelines 6-13yo stage 1 : 95-95+12 (130-138/80-89) stage 2: over 95+12 or 140/90 13yo and above just have the # value old guidelines were stage 1-95-99+5 stage 2 >99+5

Answer 167

OSA RF anamtomical - choanal atresia, ant nasal stenosis, deviated nasal septum, adeniotonsialr hypertohy, macroglossia, cleft palate repair, mandibular hypopoaspla, achrondoplasia, storage diseases , midface hypoplasia like t21, apert, crozon DAY sx= dry mouth, mouth breathing, hyponasal speech, moody, daytime sleepy, hyperactive, school issues, morning headache diagnose- overnight polysomohgram . to look at apnea hypopnea index which is <1,5 normal if 12yo and adolesent <5 complications-FTT, pHTN, cor pulomale, sleepy, depression, learning probvlems first line is T AND A

Question 168

central hypoventilation primary - congenital central hypoventilation, arnold chiari secondary - tmor, infarct, medications, dec muscles can be considered they have hirsprung rule out sedocndary cause before BIPAP

Answer 168

ARDS 1- sx within one week if insult 2- bilat opacities like pulm edema (not nodules or effusions) 3- not due to cardiac over load 4-mod to severe oxygenation . PaO2/FI02 (mild is 200mmhg, cpap 5, mod, 100-200, severe <100)

Question 169

``` bronchiolitis risk factors for severe disease <3 mo PID prem <35w hemodynamic sig cardioresp disease ```

Answer 169

admission criteria . bronchiolits Signs of severe respiratory distress (eg, indrawing, grunting, RR >70/min) Supplemental O2 required to keep saturations >90% Dehydration or history of poor fluid intake Cyanosis or history of apnea Infant at high risk for severe disease (Table 4) Family unable to cope

Question 170

RSV PROP indication - start nov/dec- 4 months 1) sig CHD OR CLD (need oxygen at 36w) who are <12mo at start of season and need diuretics, oxygen etc. if >12 mo, only if needed oxygen in the last 3 momnths 2_<30 weeker whose <6mo (not necessary) 3- 36 weeker who <6 mo and needed air transportaion to come, or inuit populations which high amount of rsv in population BUT CLD/CHD is priority 3- <24 mo if severe immunodef or admitted for prev severe pulm dsiease (mmunodeficiencies, Down syndrome, cystic fibrosis, upper airway obstruction or a chronic pulmonary disease other than CLD should not routinely be offered palivizumab)

Answer 170

pleurodynia - borholm disease group b coxacie cause and ehcho virus with vesicular stomatisi and rash pamsl and soles - with PAROXYSMAL spasm of msucle with fever - malaise, headache and myalgia that com eafte rsudden onset of fever and spaomodic pleutric pain, aggravated by mvoemet or valsava, can have a friction rub

Question 171

causes of bronchiectasis - abn dilation of bronchial tree with causes chronic obstructive lung disease - CF, PCD, PID, ABPA,post infxn (adeno, measels) and congential - hflu, staph and pseudomonas - clubbing high resolution ct - tram trackline (diamater on airway stays same rather then getting smaller) signet ring (airway bigger then vascukar can get central bronchiectasis wiht BO- caused by adeno, measysels, sjs, post ttarnpolsant, hypersensivity pneumonitis, aspiration pnuemonia

Answer 171

``` causes of pulm hemm-- GPA pulmonary hemosidorosis coagulopathy - vwb sle HSP trauma bronhecistati FB PHENYTOIN ```

Question 172

ENDOTRACH INTUBATION SIZE TUBE and what you need AGE+4/4 and minus 0.5 if cuffed, SOAP ME - suction oxygen, airway millder balde and ETT, pharm - atropine, ketamine, succ/rocc, monitoring eqt DOPE= displacement obsturction ptx eqpt

Answer 172

proper CPR push hard and fast - 1/3 of diameter (1.5 in infant, 2 in child inches) - 100 per min, allow full recoil hard surface, no interruption, rotate 2 min ventilate 8-10 breath per min 15:2 two rescuer, and alone 30:2 SHOCK SHOCK EPI SHOCK AMIADRONE SHOCK start at 2j then its 4J epi dose is 0.01mg/kg 1/10 000 q3-5min

Question 173

remember the 5'h 5't of reversible causes for cardio resp failure 5h: hypoexemia, hypothermia, hypoK/hyper k, hypoglycemia, h (acidemia) 5t- thrombosis, tamponade, tension ptx, toxins

Answer 173

what causes hypoxemic resp failure (Pa02/fi02 <200) 1, vq mistmatch, r to l shunt, red pi02, diffusion limitation hypoventilation hypercarbic resp failure - due to dec min ventilation or inc dead space - like drugs, neuromuscuslar issues, chest wall injury, upper airway edema, abdo distension MIXED is asthma

Question 174

invasive ventilation - prefer cuff, unless under 8yo. need to use this vs high flow if dec LOC, need airway protection, or have ay nasal obstruction you set the PEEP.PIP, fi02, rate, pressure or volume (5cc/kg) TO INCREASE OXYGENATION? TO GET RID OF C02?

Answer 174

TO INCREASE OXYGENATION - increase the fi02, inc the PEEP and the inspiratory time TO GET RID OF C02 - increase minute ventilation (TV or RESP RATE), expiratory time or sedation only increase PIP if obstructive picture, and decrease it if pneumothorax

Question 175

``` duke criteria for endocarditis BE FEVEER 2 major or 1 majro 3 minor or 5 minor ```

Answer 175

1- positive bcx- with 12h apart, typical strep viridans, HACEK, staph 2- endocarditis on echo, or new murmur MINOR fever, echo fidndings, Evidence with immune stuff (janeway and oslder) evidence with hemorhage, risk factor of drug use or heart condition

Question 176

Jervell and Lange-Nielsen SNHL AR AND LONG QT autosomal dominant form, the Romano-Ward syndrome, has a purely cardiac phenotype.

Answer 176

nasal prongs - help with humidfying airway helps with mucus clearanace and also creates increased fxnal residual capacity , from distending pressures

Question 177

invasive ventilation - prefer cuff, unless under 8yo. need to use this vs high flow if dec LOC, need airway protection, or have ay nasal obstruction you set the PEEP.PIP, fi02, rate, pressure or volume (5cc/kg) TO INCREASE OXYGENATION? TO GET RID OF C02 remembver child cant change their SV only HR to alter CO

Answer 177

TO INCREASE OXYGENATION - increase the fi02, inc the PEEP and the inspiratory time TO GET RID OF C02 - increase minute ventilation (TV or RESP RATE), expiratory time or sedation only increase PIP if obstructive picture, and decrease it if pneumothorax

Question 178

``` duke criteria for endocarditis BE FEEVER 2 major or 1 majro 3 minor or 5 minor ```

Answer 178

1- positive bcx- with 12h apart, typical strep viridans, HACEK, staph 2- endocarditis on echo, or new murmur MINOR fever, echo findings, Evidence with immune stuff (janeway and oslder) evidence with hemorhage, risk factor of drug use or heart condition

Question 179

``` diagnostic for SIRS NEED 2/5 hr>2 std above temp 38.5, or 36 less rr>2sd leukocyte abn if <1yo then bradycardia <10th ```

Answer 179

you have cardiac dysfunction if the bp stays low or need vasoactive drugs to keep bp up or if end organ stuff : acdosis, high lactate,. oliguria. prolonged cap refill, core-peripheral temp big doff of 3 deg

Question 180

management of shock - 1) IV access/IO - start fluids isotonic 60ml/kg and keep going 2) get 2nd PIV if have then start ionotrope, intubate with ketamine and atropine 3) 15min, if still shock, start dopamine (or epi) get IV access TARGET Scv02 70% and normal MAP-CVP - AT 60MIN = try hydrocortisone for adrenal insuff 4) try diff ionotrope based on clinical scenario of shcok and BP 5) if refractory to all then r/o pericardial eff, PTX, intraabdo pressure

Answer 180

1- cold shock with N BP - Epi (0.05mcg/kg/min) 2- cold shock and low BP - Epi, and if not working NE 3- warm shock and low BP- nor epi ``` contraindication to ketamine - hypersensitive -Inc HTN Psychosis <3mo ``` ``` succinylcholine malignant hyperthermia skeletal muscle myopathies burns musce trauma ```

Question 181

GCS | sections and what they mean?

Answer 181

E - 1-4 sponatneous eye, eye to voice, eye to pain, no eye V- 1-5: oriented, confussed, inapp words, incomp sounds/moan, no sounds M - 1-6: follow command, localize to pain, withdraw pain, flexion pain, extension pain

Question 182

duration of submersion most important factor for drowning, dont need abx as expect it but AVOID hyperthermia , usually PEA, injury worst at 3-5min attempt d fib x3 before 30deg, ensure warm 32-34 deg before giving up observe in ED 6-8 hour as can have pulm edema after

Answer 182

resus worst factors 1- submersion more then 25 min (<5min good) - resus more then 25 min (if <10min good) - good outcoe if awake in ED, GCS>6 good outcome - gcs 5 or less - seizures stay - comatose recover 24-72 hours, bad if still coma 72h neuro status in 48-72 hours imp (learn to swim by age 4yo)

Question 183

hypothermia - <30 deg, CPR 3 shock ((if vt, give max shock x1) , no drug - DONT RESUS: IF snow in airway, if thorax is frozen and cant do cpr, if avalanche under snow 35min, or lethal injury, TERMINATE CPR IF K=12 children higher risk due to low subcut fat, high bsa, limited thermogenic if ELECTRICAL INJURY- induce alkaline diuresis to avoid myoglobinuria and renal damage

Answer 183

burns - oxygen sats OVERESTIMATED (looks at oxyhem vs deoxy using light abs) - thermal injury, chemical injury, systemic poisoning CO and cyanide - signs to intubate: soot nostril, singe nostil hair, blister lip, stridor, hypoxemia, carbenaceous sputum tx- CO poisoning is fi02 and hyperbaric (cherry red lips, n/v, seizure, dec LOC) tx of CYANIDE POISONING - they have lactic acidosis and declinin paC02, get CO oximetry and treat with 100% fi02 and sodium thiosulfate + hydroxycobalamin

Question 184

burns 1st deg- painful, dry, superficial, red, no scar,. no blisters, blanches and bleeds 2nd deg - painful, wet blisters, epidermis and dermis, mottled, consider same fluid loss as 3rd deg 3rd deg- down to subcut fat, bone or muscle, , dry leathery eschar, , white and wax, no bleeding, no pain BSA- torso 40%, hand is 1% ' if mgt at outpatient- no tetanus or abx use BACITRACIN dressing and apply aquacel dressing, change daily, wash with lukewarm water, dont break blister, debride when it breaks on its own

Answer 184

``` reasons to admit for burns - hands/face/feet/perineum/genitalia or joints involved - BSA>15% - 3rd deg - electrical or chemical burns - inhalation - NAI suspected - pregnancy - underlying medical cond'n, or other injuries like # ``` mgt: ABCDE PAIN, parkland 4cc RL x kg x tbsa% >15%= first half over 8 hours then rest over 16h, if <5yo add d5, keep temp 28-33deg, measure gas and hbC0 level, NG put in start feed by 48h 1.5x, monitor u/o, wrap and debride, PAIN CONTROL MORPHINE

Question 185

miosis - think opiates, cholinergics (insecticides/organophosphates), clonidine, olanzpine, sedatives, mydriasis - anticholingic, sympathomimetic (cocaine, pcp), opiate withdrawal

Answer 185

1 can kill you - methyl salicylates (wintergreen), tca, camphor remmeber alcohol can cause elevated ELEVATED OSMOLAR GAP normal 0-5 (2na+glu+urea) n= osmole N = 275

Question 186

MUDPILES CAT -met acidosis elevated ag methanol, uremia, dka, proponyl alcohol, isoniazid,iron, lactic acidosis, ethalyne glcol, salicilaytes, co and cyanide, alcohol ketoacidosis, tylenol

Answer 186

HYPOGLYCEMIA IS HOBBIES | - hypoglycemic (insulin/sulfonyrea), quinine, beta blocker, insulin, ethanol, salcylates a late effect

Question 187

TCA- ANTICHOL, Alpha antagonism - block fast Na channels - give sodium bicarb if- death due to hypotension, get prolonged qtc>100, r wave in avr, or ventricullar dysrthmia worst at 6 hours, activated charchoal within 2 hr if seizure DO NOT USE ANYTHING EXCEPT BENZO

Answer 187

SSRI - sedation tachy , seizures qtc prolong, serotonin sx- altered LOC, autonomic instability, neuromusc hyperactive- cryoheptadine tx- keep cool, if prolonged qtc then give sodium bicarb ANTIPSYCHOTICS- ANTI dopimingergic- get NMS- FARM- fever autonomic dys reg, rigidity, mental status change - hypotension, give dantrolene, bromocriptine and diaz

Question 188

ethylene glycol - has high osmolar gap at first and then becomes high anion gap as its metabolized methanol - permanent blindness - treatment fomepazole

Answer 188

isopropanol alcohol does not cause anion gap -= hypotension, coma and resp arrest and KETOSIS camphor - vix, pneumatoceles- do cxr 2-3 w later, can cause death, no activated charcoal

Question 189

-tylenol/acetaminophen four phases 1. prophylactic/0-24h- anorexia, n/v, malaise but normal LABS except tyl level 2. hepatic injury/24-48h: resolution, RUQ pain, high lfts, INR 3. acute liver failure d3-5- coagulpathy. liver failur, multiorgan failure, death vs recovery 4. 4d-2w- resoultion ,clinical recovery, and histological recovery

Answer 189

ASA/SALICIYLATES- metabolin acidosis anion gap high, initially resp ALKALOSIS hyper then hypo glycemia, hypoK, hyperthermia, urine alkalinzaition Iron - >500ug/dl drawn4-6h - deferoxamine + WBI (4 stages, 1st stage, no ileus, bloody diarrhea3rd stage at 12-36h hepatoxic and shock, with death and ARDS, 4th is stricture) toxic 60mg/kg, death at 200mg/kg

Question 190

general toxicity management - never use ipecac or gastric lavage - cant use charcoal for: HC, HEAVY METALS, PHAILS- pesticide, HC, acid and alkali, alcohol, ileus/iron, lithium can use for tca upto 2 hours

Answer 190

Cholinergic- DUMBELS - organophoshate/pesticides diaphoretic,urination, miosis, brady, bronchospasm, emesis, lacrimation, salivation and seizures- atropine and pralidoxime

Question 191

NEXUS CRITERIA FOR CLEARING C SPINE >8yo, awake and gcs 15 clinically 1- no focal tenderness midline on c spine 2- no intox 3- no focal findings 4- normal alertnss 5- no distracting injury 6- normal flexion and extension and rotate 45 deg both sides get XRAY three views if want them odontoid and axial CT if - xray normal but high clin suspcion, # seen on xray, or xrays inadeqate xray lateral- 4 lines:anterior vert, post vert, facet and spinolaminar line

Answer 191

how do kids differ in trauma 1- pliable chest wall absorbs trauma so more internal trauma possible even if normal exam 2- medistanum mobile 3- rib fratures r less common 4- less protection from ribs/muscle and pelvis 5- tension pneumotx can occur at lower pressure and mor rapid also with lap belt injury - hyperflexion cause chance fracture, post vert # and ant tension failure, or compression which is tear or avulsion of mesentary. rupture of the small bowel. thrombosis of iliac

Question 192

compartment sx - Ps, pallor, paresthesias, pain, pulseless, paralysis SALTER - straight (widened growth plate), above, lower, through, crush growth plates more at risk of injury, heal more rapid

Answer 192

supracondylar fracture = ant humeral line and radial capitellar line is abnormal , can hv montegga where ulna affected or galeazzi where distal radial # extension injury - check for neurovasc status 1- perfusion and pulse, thumbs up (radial), ok sign is median nerve and ulnar is crossing of finger

Question 193

duct dependant lesions, can present in first 2 weeks when ductus closes- - TGA - severe hypoexmia firt born WITH NO MURMUR - coarct aorta, AS, interrupted aortic arch, hypoplastic left heart - remeber with hypoxia test except pa02 to rise >100 if its pulmonary

Answer 193

methemogbloneia - oxidation of ferrous fe2+ to ferric 3+ and dec oxygen binding and letting go capacity cause - neonate dehydration, diarreaha, nitrate from well wter, dapsone, local anesthetics, pyridium dapsone= treats dermatitis herpetiformis - celiac rash have cyanosis and oxygen does not help - chocolate brown blood and can have hypothermia tachypnic and tachy mottling and cns depression. metabolic acidosis and seizures treat with METHYLINE BLUE

Question 194

metabolic emergencies - w/u ammonia, lactate, gas and serum amino acids, urine: ketones, reducing substances and organic acids - urea cycle defect: RESPIRATORY ALKALOSIS with high ammonia and bulging fontanelle, heptaomegaly hypotonia coma

Answer 194

DKA - small 7-10cc bolus over an hour - insulin at 1 hr 0.1 - add glucose if under 15, d5, or d10 if under 10 metabolic acidosis due to ketosis and dehydration leading to lactic acidosis give K if k<5.5 and bvoiding well trying to drop glu by 5 an hour worried abotu cerebral edema- RF Na slow to rise with treatment, needing bicarb, high urea at presentation, greater hypocapniea

Question 195

bites - dog - pasturalla multicida - cat- pasturella multicida - human - eikenalla corodons tetanus if its been 5 years hand wounds primary closure, give rabies proop upto 10 days, clean well, ensure neurovasc intact, amox clav prop abx

Answer 195

eye trauma - penetrting injury: teardrop pupil, 360 subconjuctivial hem, hyphema - risk of rebleed of hypema at 5-7d, inc glacuoma risk - sheifl eye, give antiemetics CORNEAL ABRASION- photophobia, fb sensation and tearing can use flurosecein drops and green on the cornea no tx, pressure patch, abx cream or polysporin

Question 196

bites -need tetatnus and ig if <3 doses in dirty wound, need only tetatnus if more hten 3 doses, 5 years if dirty or 10 if clean DONT CLOSE if; cat or human (unless its face), hand or foot, >12h, puncture wound human - hepb virus too, antibiotic if ON HAND, worry about eikenella corodoens cat - dont close, need prop, pasturella multicida dog- can close, no prop, rabies needed (can watch dog for 10d if domestic and if ok then no need) prop is amox clav x 5d - generally need it if you close it, if they are immunocompromised, if it is near a joint, on face/genitalia/hand, crush injury, deep puncture

Answer 196

indication for urgent FB removal 1- sharp object in esophagous, disk button battery, fb with resp sx, in eso>24h, multiple magnets AP wide circle for esopagnous

Question 197

microcytic anemia low retic - TAILS and copper high retic- thalaesmia MENTZER index MCV/RBC - <13 thal, >13 iron def normoctyic low retic- TEC, chronic disease, endocrinopathy, renal failure high retic- ab, maha, membranopathy and enzymopathy and sickle cell macro cytic low retic- folate, b12, diamond blackfan, fanconi, t21, hypothroidim high retic- active hemolysis

Answer 197

basophillic stiplling - lead or thal basket cell - g6pd pencil cell - iron def (screen 9mo term, 3 mo preterm if mom breastfeedng wihtout suppl or not iron fortified form) remmeber stored iron in ferritin fe2+, then in transferrin its fe3+, hepcidin is form stored in liver signs of iron def - (inc risk prem, breatfeed exlsuve past 6mo, excess milk, go loss, menst bleed, kidney disease)- koilonychia, cheilosis, pica low iron, low ferritn, inc transferring, high tibc.saturation tx 4-6mg/kg/f on empty stomach, avoid milk, take with vit c, check 2 w treatment - enusre complaince/blood loss not there if low ferritin, hgb rec 6 mo sometimes get consitpation, stiained teeth and dark stools - retic by 48h, hgb 4-30d,, normal ferritn 3 mo

Question 198

microcytic anemia low retic - TAILS and copper high retic- thalaesmia MENTZER index MCV/RBC - <13 thal, >13 iron def normoctyic low retic- TEC, chronic disease, endocrinopathy, renal failure high retic- ab, maha, membranopathy and enzymopathy and sickle cell macro cytic low retic- folate, b12, diamond blackfan, fanconi, t21, hypothroidim high retic- active hemolysis

Answer 198

basophillic stiplling - lead or thal basket cell - g6pd pencil cell - iron def (screen 9mo term, 3 mo preterm if mom breastfeedng wihtout suppl or not iron fortified form) remmeber stored iron in ferritin fe2+, then in transferrin its fe3+ signs of iron def - (inc risk prem, breatfeed exlsuve past 6mo, excess milk, go loss, menst bleed, kidney disease)- koilonychia, cheilosis, pica low iron, low ferritn, inc transferring, high tibc.saturation tx 4-6mg/kg/f on empty stomach, avoid milk, take with vit c, check 2 w treatment - enusre complaince/blood loss not there if low ferritin, hgb rec 6 mo sometimes get consitpation, stiained teeth and dark stools - retic by 48h, hgb 4-30d,, normal ferritn 3 mo

Question 199

DBA - only anemia, nromoctyic usu diangosed before 1yo, have anomalies like long thumgs, craniofascial, cardiac , optho absent radial pulse nad high risk of malignancy- treatment with transufsion adn chelation and BMT is curative vs tec- resolves on it own usally 1yo and older, can have neutropenia

Answer 199

THAL mentzer index < 13 , RDW NORMAL (high in IDA), mcv VERY LOW, plt normal with minor/trait - have bb0 or b+b - microctosis and target cell with basophilic stippling, with mild to no anemia - no tx. with beta intermediate they have no normal beta, so b+b0 or b+b+ - HSM, bone changes, iron overload, transfusion needed, hgb F 70% and HGBA2 (which is alpha:gama) major - cooley anemia - iron overlaod, severe anemia, expansion of bone marrow cavitiy, splenomegly, path fractures, trat with transfusion and chelation take out splene IRON OVERLOAD- DM, cirrhosis, heart failure, nrozne skin, endocrine changes- death usu heart failure d/t iron overload

Question 200

on smear - stippling, target cells, microcyTosis, hypochromia hgb bart is 4 gammas, they die in utero hgb f is 2alpha, 2 gamma hgba2 2 alpha 2 delta for alpha 3 copies is silent carrier traisis aa/-- or a-/a-have low mcv, hypochormia, low or normal hgb H wiht normal electrophoresis - hgb H PREP may be positive have normal RDW with RBC increased and low mcv low mentzer<13 with hgb h disease --/-a moderate anemia and hpochormia microcytosis target cell and heinz body, splenomegaly and jaundice

Answer 200

hgb SS dont transfuse for VOC INDICATIONS- aplastic crisis, splenic seq, preop for high risk surery , stroke if low hgb, chronically to prevent stroke by keeping hgb s under 30% exchange- for severe ACS, STROKE, preop soemtimes transfusion risk- allimmunization, iron overload, usually 1 yr later, infxn, penicllin at 3 month and unknown when to stop, prevnar and pneuvomvax transcrianall dopllers until 16 start at 2yo till 16 yo end organ damage parental education HGB SC- higher risk of retinoatjy

Question 201

g6pd nadph depletion xlinked recessive oxidative stress causes heinz bodies - diff types type b- normal a variant - unstable african american meditterian variant - chronic hemolysis and potentialiy life threantening avoid - splenecomty, sulfa, septra, nitro, antimalariasl, dapsone, fava beans, rasbirucase and ASA spherocytosis - confirm with osmotic fragility test- neg DAT, gt gallstones and suspectible to aplastic crisis, treat with ssplenectomy - try to wait till 5yo

Answer 201

AIHA - usually post infxn or drug or sle, alps drug also can form hapen on rbc 3 types cold- 1mg, c3 positive dat warm- igg extravascular, igg positive dat biphasic treat with least incompatible transufsion, steroids ivig and retux, 80% recover spontaneously. best severity predictor of jaundice- CORD HEMOGLOBIN

Question 202

NON IMMUNE CAUSES of hemolytic anemia - rbc trapped by fibrin in a vessel - shear stress HUS DIC TTP kasabach merritt - murjmur over liver- ennlarging kaposiform hemangioendothelioma - painful and tense with drop of platelet, low fibrongen and microangiopahty HUS - anemia, thromboytopenia na drneal - ecoli 0157:h7 toxin shiga like ttp- maha, low plt, renal impairment, cns and fever - due to acquired ADAMTS13

Answer 202

fanconi - xm breakage -<10yo, ar, no thumbs, shor tstature, cafe au lait, horseshoe kidney, microephaly- inc risk of malignancy- leukemia, mds, aml, solid tumor, tc BMT, consider androgenic therapy schwachman diamond - ribosome issue - fat malabs and neutropenia, get short flared rib, bifid thumb, dental anomloies, neurocog isuees- test fat malabs on 72h fecal fat, bm shows fat infilitration and low b cell, MDS LEUKEMIA, tx ADEK, oanc enzyme and monitor cell count g-csf if neutropenia diamond blackfan - only red cell, treatment with steroids, can have congenital craniofacial anomalies, triphalagnial thumb, no radial pulse, inc malignancy risk, <1yo usu diangosis

Question 203

RF FOR polycythmiea | -IDM, placentail insuff, TTTS reciepeint, POSTDATES, delayed cord clamp, neonatal grave/hypothyroidism

Answer 203

creation of a clot 1- vascular injury, endothelial collegen change 2- activation vwf, binds plt with glycoprotein 1b 3- plt release ADP, get more plt 4-coag cascade, makes THROMBIN, fibrinogen becomS FIBRIN 5- factor 13 ativates and crosslinking fibrin makes stable clot thrombin acctivates protein C and converts plasmiogen into plasmin which causes thombolysis (breakdown of fibrin) antithombin 3 inactivates THROMBIN and 9-11 protein c and s - inactivate 5, and 8 - VIT K depnedant

Question 204

against coagulation / break down clot thrombin - activates protein C and plasminogen to plasmin antithrombin 3 breask down thrombin and f9-13 plasmin, breaks down fibrin protein c and se - inactivate 5 and 8 w.u - cbc inr ptt, factor levels, pfa 100 - plt fxn - if abn vwd, kiver, heriditary or acquired plt issue

Answer 204

thrombytopenia dec production - TAR wiht normal thumb, amegakaryocytotic thrombocytopenia- no other abn, usually progresses to aplastic anemia, TORCH, EBV HIV MEASELS destruction NAIT -allimmune - HPA1a antibodies, tx 1year dp abma- if severe anemi or neutropenia, lad, type b sx,., or hsm

Question 205

ITP management dont have splenomegaly watch and wait if just basic bleed- petichae bruises or gum bleed/menerrhagia if have plt <30 PLUS another issue like wf, or using nsaid or active lifestyle, consider ivig/antid if need immediate or steroids if not urgent if BAD BLEED, like epistasxis not stopping, gi bleed, jt bleed or intracranial bleed, may need splenegomaly then do steroids, ivig and antiD and plt trfn tell them avoid contat<30, avoid nsaid, BMA if concerning features

Answer 205

platelets - small WAS - xlinked, low plt., low gam, eczema big platelets = bernard soulier syndrome deficitient in glycoprotein 1b glanzmann thrombasthenia - deficieny 2b and 3 a CLOTTING CASCADE- intrinsic PTT = 8,9,11,12 factor 12 only effects ptt extrinsic INR = 7, tissue factor COMMON 1,2(promthrombin) 5,10, 1=fibrinogen neonates have less vit K DEPENDANT and have high factor 8

Question 206

hemophilia is a- 8 or b-9 affects ptt (intrisic = 8,9,11,12), need factor assay <1% spontaneous bleeding, hermatrhrosis SEVERE AND INFANCY., THINK IVH, vit k needle, circumscision moderate 1-5% need moderate trauma to get issue still give them vit k - hold pressure TREATMENT= for hemophilia A that s mild to moderate u can give DDAVP which increases factor 8x4 transamic acid or amminocaproic acid inhibitor of fibrinolysis useful for oral or mentstrual bleedings goal - to prevent orthopedic issues (chronic arthropathy, fractures, compartment sx_ AVOID NSAID OR IM injections with vaccine give orophylaxis and then subcut with lots of pressure side note- cryopreceipitate has fibrnogen f13, vwf, 8

Answer 206

VWF - 1% AD bridges collagen and platelets and prevents FACTOR 8 . from being broken down quickly if very severe can mimic hemophilia A type 1 is partial and most common type 2 is quality issue - no ddavp type 3 ABSOLUTE def and severe labs - vwf antigen, ristocetin cofactor assay (FUNCTIONAL), cbc, pfa (elevateD) coags N, factor 8 low, blood group - O is worst if normal number and low ristocetin - type 2 treatment with DDAVP = watch for thirst and hyponatremia humate p is second line if not enough avoid asa and advil avoid contact sportjs tranexmacid acid for mucocutnaeous bleeds

Question 207

``` differential for neutropenia -b12 and folate deficiency - von gierke gsd1b - fanconi schwanmon diamond - scn - cyclic neutropia - AD sporadic and do 3x/week x 6 week to show cycling ```

Answer 207

transfusion reaction 1) stop it, iv open, confirm product and assess, dat - TACO - resp distress, diuresis oxygen - TRALI - FEVER and resp distress- neutrphil from lung - supportive - anaphylaxis - iga def epi - ***acute hemotlyic reaction - mistake, hydrdate and diuresis - febrile non hemolytic- symptomatic from wbcs not leukoreduced -transufsion sepsis urticarial reactin- give anthisatmine

Question 208

leukoreduce - reduce hla allimmunization, reduce cmv, reduce febrile non hemoltyic rxn - give if chronic trfn, surgery, may be transplanted irradiate for gvhd - prems, pid and cancer hiv is 1/8-12 million in blood product,hcv 1-5/million, hbv1/1million

Answer 208

``` splenectomy - chronic itp,rupture,hypersplenism give amox prop vaccinate prevnar 13, penxumvax at 2,7yo mening at 2mo then q5y give malaria prop ``` populations at risk of moya moya -NF1, sickle cell, tubrous, sle, turner, marfan, t21

Question 209

indications for BMA

Answer 209

``` PANCYTOPENIA AND LEUKOCYTOSIS blasts on smear unexplained sign depression more then 1 periperhline leukoerythroblastic changes on smear unexplaine dlad or hsm ant mediastinal mass ```

Question 210

side effects of med vincritsine- peirpherl neuropathy - constipation, alopecia - bad intrathecal, hyponatremia cyclpohphamide- hem cystitis, diarrhea, alopecia, siadh hyponetremia cisplatin- renal damage , ototoxic doxyrubicin, cardiac, damage, typhlitis bleomycin causes pulm fibrosis 6mp - pancreatitis, pulmonary fibrosis, renal toxi, hyperpigment, hypoglycemia, Asparaginase can be associated with allergic reactions, coagulopathies, acute pancreatitis, and increased liver transaminases, Asparaginase induces a hypercoagulable state that can result in catastrophic thrombosis

Answer 210

TLS- high phoshate, high urate, high k, high cr, LOW calcium at risk if host has renal issue, leukemia, lymphoma, bulky abdo disease, burkitts, high wbc treat with urate - allopurinal, rasbirucase high phoshpate - aluminum hydration and monitor 1.5x maintenance

Question 211

other emergencies of cancer 1- f&n 2- mediastinal mass 3- spinal cord compression (neuroblastoma,s arcoma) 4-inc ICP -cn 6 palsy 5- hyperleukocytosis WBC>100- death r/t resp failure and ich hydrate abx call icu dsluggsih and clotting, vareful with prbc transfusion due to inc it more 5- hyperCa(bone tumor) hyponat(siadh)

Answer 211

PTH Causes activation vit d from 25 to 1-25 using the 1 ALPHA hydroxylase in kidney reabsorbs cal and phosphate from gut using active vitamin D reabs ca from kidney, STOP reabs phopshate from kidney inc ca and phosphate breakdown from bone

Question 212

associations of conditions with leukemia name 8 associations qith LYMPHOMA think PID WAS, AT, congenital hypogam, HIV post solid organ tx

Answer 212

``` pid - AT, SCID WAS diamond blackfan and schachman diamond paroxysml noctunal hemoglobinura nf1 ds kosman syndrome /scn li fraumeni bloom syndrome ```

Question 213

sicke cell fever must admit criteria (outpatient ceftriaxone and d/c with follwo up., do bcx) if <2yo, hgb 2 sd below baseline, hemodynamic instability, temp over 40, wbc>3- or <5, indwelling line, prev bactermia , family concerns, signs of other sickle crisis

Answer 213

burkitts can present with appendicitis or intuss as 80% have abdo mass, endemic -ebv driven in africa, jaw mass sporadic-buth norht american type b sx, cough, lad if >25% blasts its ALL smear REED STERNBERG cells pft and echo pre chemo

Question 214

``` high risk ALL 1- wbc>50 2- age <1, or >10 3- cns disease 4- unfavourable cytogeneitcs 5- hypodiploid ```

Answer 214

``` higher risk NBL n myc amplifiation older 18 months hypodiploid lack of cell differetiation ```

Question 215

Febrile seizures recur in 30% of those experiencing a first episode, 50% after 2 or more episodes, and 50% if <1 year old at onset. Several factors affect recurrence rate: MAJOR factors Age < 1yo Duration of fever <24 hours (short onset between fever and seizure) Fever 38-89 degrees Celsius (seizure occurred at lower temperature) MINOR factors Family history of febrile seizures Family history of epilepsy Complex febrile seizure Male gender Lower sodium at time of presentation

Answer 215

``` worrisome lymph node >2 cm chronic generalized supraclavicular associated with type b features hard non- tender ```

Question 216

inc risk of brain tumor li fraumeni, nf1 nf2 tubrous von hipper lindau most common are cerebellar 1- medulloblastoma -this is non glioma - its embryological 2- cerebellar astrocytoma (glioma) Glioma less sensitive to chemo treatment complicatins (dex, chemo and rads) cerebellar mustim syndrome - 25%, irritbile apathy and cerebellar dysfunction for hours-days post surgery somnolence syndrome - is self limited fatigue after radiation posterior fossa sx- h/a, asepctic mengitis for weeks post op

Answer 216

supratentorial = present with weakness focal, sensory, seizures, LOCALIZING SIGNS, no increased ICP, hyperreflexic, hemiparesis or hemi sensory loss usu infant or adolescent infratentorial - includes cerebellum and brainstem usally CHILDREN 1-cerebellum-more ataxia, and increased ICP (cn6 palsy) 2-brainstem usualyl children-head tilt, cranial neurpathy like dysphagia, facial weakness,) ataxia, long tract signs perinaud syndrome - pineal region- paresis iof upward gaze, dont act to light, eye lid retracted, nystagmu, can accomodate

Question 217

neuroblatoma presentation -periorbital ecchymosis, spinal cord compression, horner syndrome, opsonclonus mycoclonus, VIP induced diarrhea ``` 4s- great prognosis <18mo, bluish subuct nodules, hsm, bone marrow, inv urine HVA/VMA axr stage with CT need histology, n mtc, ploidy ```

Answer 217

wilms tumor - assoc with 3-5yo at risk WAGR - wilms anirdia, GU ab, retarded beckwith weidemann, ultrasound q3mo till 8 Denys Drash - renal disease, pseudohermoprodtie, wilms, hemi hypertorphpy horsehoe kidney 2x risk ofwilsm 11p deletion can get cardiomyopathy, htn, renal insuff, infertlity, lung and liver dysfunction hepatpblastoma risk- beckwith weimdna, familial adenomatous polyposis, prem, hemihypertrophu, increased AFP

Question 218

soft tissue cancer- 1- rhabdomyoscarma - M>F usually 2-6yo osteosarcoma- DISTAL FEMUR, common in adolescents or proximal tibia lytic lesions with calcifications, mets to lung EWING usualyl ribs and flatbones and elvis or in long bone diaphysis. can be asscoaited with soft tissue mass

Answer 218

retinoblastoma can be assoc with rb1 mutation do orbital ultrasound can have leukemia or secndary glaucoma screen first degree relatives if its hereditary inc risk of osteosarcoma, sarcoma and melanoma amaurosis- profound and total loss of vision

Question 219

amblyopia - decrease in visual acuity due to lack of clear image projection - absence of ocular disease (inc risk in prem, sga, fmhx, neurodev delay) - unilateral if more then 2 lines off from good eye, or bilateral if both eyes visual acuitiy under 20/40 cause by strabismus - most common refractive deprivation ametropic - astigmatism causing inability o focus on far objects anisometropic- unequal focus causing blurry in one eye

Answer 219

test for it by: FIXATION REFLEX- each eye occluded and ensure target moving - when both eyes uncovered, they dont maintain fixation with amblopic eye OBJECTION TO OCCLUSION VERTICAL PRISM TEST: prism in fornt of R eye and if eye moves upward to view image if over 3yo do visual acuity, with optotypes allen figure cards if cant use SNELLEN or tumbling E but allen overestimates refer if visual acuity 20/40 at 3-5yo, or worst then 20/30 visual acuity >2 line btwn eyes strabusmus - abn ocular ralignment, abnormal red reflex, asym of vision, unilaterla ptosis or lesion that threaten visual axis (hemangioma)

Question 220

anisocoria 20% its normal if issue with dilation in dark - thats horner if issue with constrition in bright light- CN3 palsy - larger pupil is abnormal (diff, WAGR- horner, cn3 palsy) leukocoria differenital - retinoblastoma - cataract - persistant fetal vasculature- micropthalmic - coats disease- exudative retonopathy - rop - larval granulomatosis

Answer 220

``` cataract 1/3 inherited,1/3 genetic,1/3 idipathic drug - steroid autoimm- JIA uveitis genetic - t21 infxn= RUBELLA, neonatal varicella metabolic- GALACTOSEMIA trauma radiation (<1yo wiht RB get 2ndary tumor) ``` sequale - glacuoma, ambylopia, strabismnus, retinal detachment

Question 221

with contacts-worry about pseudomonas keratitis, tx with topical fluoqinoilones worry abut conjucnctivitis IF - accompanying with headache, photohobia, fb sensiation, ciliary flush, fixed pupil, reduction of visual acitvitiy rememebr keratoconjunctivitis cna be caused by adeno- with cehmosis and photopgbia if its PSEUDOmembranous- strep and staph and clhamydia if its true membranous and stuck to eye- DIPTHERIA- raw when removing

Answer 221

bacterial conjunctivitis - organisms stpah, s pneumo, m catarrhalis, h flu erythrmycin drop QID x5-7days respond within 48hours and refer if not better by then wiht nacrolacrima duct obstruction- sx at 6w due to failed canalization of epithelim to make a duct -tear overlfow, reflex mucoid material, maceration of srrounding skin with infection- acute - urgent iotho or if dacryocystocele - refer treatment is massage if no pus if it is give topical abx, and if persistants ater 1yo then refer

Question 222

acute vision loss ddx- retinal detachment hyphema, uveitis, acute closer glacumoa, papilledema, optic nerve ischemia/inflm/infxn

Answer 222

glaucoma- buphthalmos, tearing , photohphobia, blepheropsasms

Question 223

orbital cellulitis- stpah/mrsa, strep, h flu posterior to orbital septum from sinu sinfxn due to thinner walls, porous bones and open suture lines and larger vascular foramina - ct orbits tx iv vanco and third gen ceph consider flaygyl if anareobe, RAPD, vision loss, vision loss CVST preseptal - eye white, no proptosis, no rom or rapd or edemea, well rhabdomyosacroma- have proptosis but NO PAIN and limited eom, Cchild well and area cool

Answer 223

uveitis - anterior is iris and ciliary body posterior is the choroid NOT RED - complications glaucoma, cataract, synecheia, cloudly ca on cornea antierior is autoimm KD, JIA, SJS, akn spon - get ciliary flushing, pain, photophobia, lacrimation psoteiror is toxo, rubella, hiv, tb parasites and both is behchets and lyme , present with NO REDNESS, but floaters, dec acuity tx for non infectious - steroid local drops and dilate drops to precent synechiae

Question 224

``` ROP stage 1 demarcated stage 2- ridge stage 3 extraretinal fibrovasc tissue 4- subtotal retinal detachment 5- retinal deatcmehnt ``` prog- myopia, anisometropia, strbaismus, amvylopia, nystagmus tx- cryo and laser

Answer 224

hyphema - anterior chamber so cornea to iris tx- elevate head of bed, sheiflw itout patch, immobilize wiht cyclplegic drops like atropine -, topical steroids, AVOID nsaid risk of reblee at 2-5 days riskier if 50% hyphema, if inc BP, asa use or elevated ocular pressures loss of vision and flaocma positentioal

Question 225

how to dfferintate ptosis from horner vs cn3 palsy horner- miosis, SMALL ptosis as muller muscle, anhyrsosis, r/o neuroblastoma and do mri contrast of head neck hest and abdo and urine hva/vma- topical lidocaine to tests cn3 ptosis- BIG ptsosis as its levator muscle affected, u get mydriasis adn eye downand out, workup mri/mra dont forget myesthenia gravis on differential

Answer 225

rando facts cystinosis- eye finding blephersomapsm, watering and photophobia due to cysteine deposoits, and can lead to hemm retinopathy - DONT GET CATARACT or glaucoma t21 nystagmus, catarat, glacoma, refractive error, strabismus,

Question 226

FUO - fever 2w, NYD despite investigation at 3 outpatient visits or 3 days in hopital can be nosocomial, neutropenic or hiv assoc first line bw - cbcd, lfts, esr/crp, blood cultures, ana RF, mono, ebv, cmv serologies

Answer 226

``` others to consider- viral = cmv, ebv hep and hiv bacterial - tb brucellosis salmonealla cat scratch. lyme. lepto gungal histo and blasto parasite malaria and toxo ``` hence malaria smears, tst, echo imaging, bma, hiv

Question 227

bugs the next age groups are prone too neonate 1-3 mo if >3 mo then hib, strep pneum, meningocooccus and non typhi salmonella, do only urine and bcx

Answer 227

neonate- gbs, listeria, e coli -amp/gent or amo cefotax if there is mec at birth- think about listeria if PREM 1-3 months = gbs, listeria, ecoli ALSO strep pneumo, h flu, neissiera, and salmonella - if the >4w meet low rik crtieria can be trated with abx as an outpatient -->wbc 5-15, wel appear. no prem, no focus, urine <10wbc hpf, diarrhea <5 wbc/hpf, normal cxr if resp signs

Question 228

reccurent fever PFAPA 3d, q3-6w, one dose pred, can do cimitedine, tonsillectomy, cholcicine fmf - AR mefv gene , perotintis, 1-3days, treat with colchicine get erysipelas rash, peritnits, arthritis, pericarditis

Answer 228

cervical adenitits - acute bilat: cmv, ebv, adeno, entero - acute unilat: staph strep (gas) - bilateral chronic, hiv, ebv, cmv, toxo - chronic unilateral- atypical mycobacterium (NTM- violaceous and pus), bartoenella henselae, TB, tularemia with bartonella- can get cns disease u shud treat with rifampin or doxy, if just lymphadenitis can give azithro to shorten the course , get perinaud oculoglandular wx, hhepatosplenic ganuloma, eneceophlopathy and neuro retinitis

Question 229

measels finding 1- stimson line- eye inflammation 2- koplik spots Contagious 2 d before and 4 d after rash gone cough coryza and conjuncitivits LARGE DROPLET rash decendes down body high fever toxic, spares palm and sole complications- myocarditis, encephalomyelitis, subacute sclerosis panencephalitis 8-10y later, OM, mesenteric lymphadenitis, giant cell pneumonia treamtnet with high dose vitamin A suppl if high risk: PID, vit A DEF, impaired abs or malnutrion or developing country, prevent with vaccine- cant give if steroids x14d, or chemo in last 3 months give immunoglob if within 6d, vaccine if 3d of exposure

Answer 229

rubella - forscheimer spots on palate petichae contagnious 2d befor eand 7 days afte rrashm their prodrome is not huge - post auricular node tenderness and rash x3days paresthesia and tendinitis common, can get low plt, good prognnosis congenitla rubella sx= iugr, deafness, cataract, PDA,bluberry miuffin rash REMEMEBR roseala 6th disease assoc with hhv6/7, rare complication is encephalitis, fever 3d then rash, by 2-3yo 100% have igg to hhv6/7, causes 1/3 febrile seizures REMEMBER WITH parvo , once rash there usually not contagious anymore, prodrome 7-10before rash

Question 230

chicken pox VZV incubate 14d, prodrome 1 day fever malaise anorexia then tear drop vesicles on erytjematous base, new crop 3-4d, 100-500 lesiosn that are itchy can be in MM, latent stay in dorsal root ganglia contagious 2 day before rash and 7 day after onset with reactivation of zoster, stays in the dermatome manage- cool bath, no asa, antiviral if (1-13yo pulm cor cutaneous disease, kids on steroids, kids on ASA) NOT PREGNANT WOMAN COMPLOICATIONS- varicella pneumonia, any itis -myocarditis, pnacreatitis, reye symdrome, CEERBEELLAR ATAXIA, ramsay hunt sydnrome, GBS

Answer 230

GROUP A STREP Scarlet fever need to be second exposure wiht prodome being 2-4 d of sore throat and fever get strawberry tonge, pastia line, maculopapular rash treat to prevent RHEUMATIC FEVER can treat within 9 days complications - parapharyngeal abscess, ARF, glomerulonephitis amox firstline or penicillin erythro if allergic

Question 231

skin stuff - hot tub folliculitis is psuedomonas aerignosa within 2 days, no treatment goes away in 1-2 w if its impetigo -bullous is staph -non bullous and honey crusted can be staph or strep = if strep increased risk of Gn but not RF, STILL TREAT to prvent sptead, out of school till on abx 24h complication - ecthyma (ulcer) need to admit cellulitis- if face/hand/perineum, if erysipelas and with lymphangitis

Answer 231

nec fasc treatment if GAS - PEN + CLINDA + IVIG (usually vzv mediated) if staph - clox and clinda abscess- drain and send for cx <1mo iv abx, unless <1cm can do oral clinda outpatient 1-3mo - septra po >3mo - observe post drain, only abx if dont improve, unless theres cellulitis then do septra and keflex RF lose ­skin-to-skin contact, openings in the skin such as cuts or abrasions, contaminated items and surfaces, crowded living conditions and poor hygiene. Aboriginal populations, athletes, daycare attendees, military recruits, intravenous drug users, men who have sex with men, and prisoners, but many infected children have no risk factors.

Question 232

HSV | stays in trigeminal ganglion

Answer 232

buttwhole stuff perianal dermatitis GAS- pain with poo and pain and itcht - give oral pen pinworms -enterobiasis give mebendazole once and rpt in 2 weeks (wash ahnds, clip nails, wash bedding, educate family)

Question 233

CMV - rare to cause death congenital infection mainly 85% asymptomatic - of those 15% wull have sequalea of hearingloss 15% with symptoms, 50% have permanent sequlae - chorioretitnis, thrombycortpenia, iugr, perveintricular calficiation, snhl, alt, heptogemagly best test IS CMV DNA by pcr in 14d of life urine or saliva treat if cns, snhl, chorio WIHT 6 MONTHS OF VALGANCYLCOVIR monitor cbc and cr if mild - ie, low platelet, case by case

Answer 233

rubella triad - cataract, pda, snhl bluberry rash, celery stalk boney lucency permanent- =peripheral pulm stenosis, micropthalmia if exposed in pregnacy send for igm, discuss possible termination TOXOCPLASMOSIS- hydropeph, cerberal calcification and chorioretinitis (early disease - 1/2nd trimester) if third trimeser can be asx other then chorioretitniris causing vision loss at 3-4yo check igg, igm iga serum pcr of csf, blood and urine (Csf shows very high protein) placental pathology and eye exam treatment 12 months - pyrimethamine, sulfadiazine and leucovorin x12 months - check neutrpphils , steorids for eye disease, vp shunt

Question 234

SYPHILIS bone deminserliation, periositis, rash on palms and soles and desquamate, pseudoparalysis, uveitis, glaucoma SNUFFLES, Late- saddle nose, hutchinson teeth, higoumenakiss sign SNHL test non trepomenol - VDRL, RPR titre over 1:16 active treponemal - TPPA or CLIA, its positive or neg- its the SCREEN PERFECT WORLD- mama treated approrpiately with penicillin, done a month before delivery, and drop of RPR by 4 ) then just do serologies trepenomal and non treponemal tsts on baby and just clinically follow the baby

Answer 234

``` IF HOWEVER 1- not 4 x rpr drop 2- mother not done tx by 1 month before 3- mom didnt get pencillin 4- infant symptoms of syphillis 5- mom not treated 6- mmom relapsed on treatment ``` THEN FULL EVALUATION while baby on pen G X14 DAYS 1- serologies treponomel and non treponemal 2- LP and then again at 6 mo 3- CBC LFTS 4- optho and ent 5- skeletal survey 6- direct detection with cord or nasal discharge or placenta

Question 235

varicella ONLY TIME to seperate mom and baby if if mom has current vzv - cicratical scar, limb hypoplasia, microphtlamia, cataract, chorio, horner, gerd, duodenal stenosis, microcolon, iugr moms exposed face to face more then 5 min or more then 15 min indoor contact PROPHLYAXIS 1. give vzig wihtin 10 days if igg neg 2 vzig to babe if mom gets it 5 days before or 2. days after delivery 3. or if <28 w <1000g 4. or if >28w without immunity in mom

Answer 235

zika virus its a FLAVA virus clinical signs- microcephaly, collapsed skull, contracture, thin cerbrebral cortices, subcortical calfication with maccular scarring and focal pigmentary retinal mottling Approach: child 2016 born with sx + ventricomeg+ maternal travel/paternal to zika area. Discuss w/ ID ZIKV ser+ blood/urine for ZIKV PCR- mom and baby+ exposure time on req Placenta save u/s and mri of baby head non urgent Approach if normal infant with mother having exposure 1. MOM-ZIKV serology + (if exposure within 4w, blood and urine ZIKV PCR) → if NEG then no zika 2. MOM-If serology +/int or PCR + --- baby normal head and exam → ZIKV IgM and blood urine for ZIKV PCR and u/s baby head, if ALL normal - consider audiologic follow up till 6 yo for late hearing loss --- baby microceph head and exam → ZIKV IgM and blood urine for ZIKV PCR and MRI baby head - if normal then as above, if normal or MILD abnormal then audio/optho exam

Question 236

fever in returning traveller - three killers - neisseria, ,malaria and typhoid - emergencies are above- include dengue hemorrhagic fevers initial test- cbc diff, lft blood culture, malaria smears thick and thin x3, serology - mv, ebv, hepatitis, hiv, dengue, brucellosis, strongyloidosis, cxr, tb urine culture stool for c&s and o&p

Answer 236

salmonella typhi treat if culture is positive if non typhoid dont have to ttreat unless <3mo old, immuncomp or have GI issues VACCINE PREVENTABLE meningoccous, yellow fever, typhoid, hep a and b hep b get gianni crosti, polyarteritis, GN aplastic anemia

Question 237

malaria p. falciparum most common. sx within 2 months thick and thin smears x3 seperated 12 hours apart sx- cant walk or sit up, changed loc, convusltion, resp failure. ards, dic jaundice, macroscopic hemaglobinur,a, paraxoysms of fever/chills, severe anemia , can get seizures, fevers can have periodicity labs - acidosis, renal impairment, high lactate, hyperparasites ADMIT if faciparum if mild - malarone x3d po severe ARTESUNATE IV

Answer 237

typhoid - aka salmonella typhi india and south asia gram negative rod RELATIVE BRADY, abdo pain, rose spots , hsm course week 1- fever and brady 2 abdo pain and rose spot 3 hsm and intestinal bleeding with 2ndary bacteremia has a vaccine 50-70% effective treatment with ceftriaxone if severe and cipro if typical disease giardia treatmebt metrondiazonle 5days and avoid lactose containign foods

Question 238

``` traveller workup vision and hearing cbc diff lft renal serology cxr and urine and o and p dentitition vaccine catch ups ```

Answer 238

for brain abscess -its ceftriaxone and clox and flagyl if theres a vp shunt then cefrixone and vanco sinusitis - amox clav orbital cellulitis- vanco and ceft and flagyl

Question 239

toxic shock syndrome staph arueus or GAS - empiric therapy is clox AND CLINDA - if GAS then pen and clinda and ivig need major cirteira due to tsst1 enterototxoin acute fever, hypotension, rash (minor 3 - mm, liver, renal, muscle, cns, low plt)

Answer 239

uti - po abx, amox clav or septra need iv if toxic or immunocomp = amp and gent for 7-10 days RENAL us needed if febrile under 2yo

Question 240

osteomyelitis age - and treatment neonate - staph, GBS, gonorrhea treatment is clox and tobra 1-3 mo - staph strep pneimp, h influenze - cefotax and cloxacillin child - staph aureus, strep pneumo, GAS, kingella - clox or cefaz remember westnile virus can have aspectic meningits, enceph and acute flaccid paralysis , its from mosquito and late summer and fall worst

Answer 240

- ebv high risk of splenic rupture is within 3 weeks - can return to sport if at 50% low impact if 1- resolution of symptoms 2- normaliation of. lab markers 3- resolved splenomegaly - confirm ideal by u/s 4- resolved of complications like enlarged tonisls - ebna, ea, and vca most useful for diagnostics monospot over 5yo VCA IGM and EA IGG tell u active infection and EBNA NEG if VCA IGM/EA IGG NEG and EBNA positive its a PAST INFECTION

Question 241

isoniazide - hepatotoxicity, peripheral neuropathy rifampin - hepatoxic, memory impairment, turns body orange pyrazinaimide, hepatox, gi upset ethambutol - optic neuropathy, color blindness, need monthly ete exam, renal and hepatic and heme tsts these r treatments for tb PIRE

Answer 241

q fever- c. burnetti . from farm and inahling infetious aerosal and contaminated food aspiratioN FARM= Q FEVER Influenxa like - acute and self limited pericarditis, hepatitis, rhabdo and HUS like hsm if near lake think legionellosis- or polluted water

Question 242

hiv - heterosex most common transmission for females - hyper gam, dec cd4 and no ab responses to vaccines cns involved in children more then adults

Answer 242

``` contraindications to vaccines 1- anaphylaxis 2- pid and live vaccine 3- intuss secondary to rota 4- live attenuated vaccine and severe asthma 5- influenza vaccine if got GBS within 6w of getting vaccine 6- mmrv in pt with active tb 7- moderate to SEVERE ILLNESS ```

Question 243

pertussis - disease and vaccine dont confer immunity - three stages - catarral URTI, for a week second stage is paroxysmal upto 6 weeks - cough and post tussive emesis convalenscent after 2 weeks and it starts to improve - can see cxr perihilar infiltrateios and lymphocytosis -macrolide to decrease SPREAD but no chagne in the severity or cough synptoms azitrhy x 5 days admit if <3months - tend to have apnea, any child with complications , 3-6 mo with sevre paryxosyms 1% mortality prevent wiht vaccination, reporting, post exposure vaccines, boosters

Answer 243

varicella vaccine with immunosuppresion give 4 weeks before intiaition and have to wait 3 months after intiation - unless its high dose steroids then wait one month - if cancer - its contraaindiacated but can give in AL . if 12 months in remision and wbc 1.2 or highter, no rads, and chemo can be withheld one week before and one week after vzig - if exposured indication ---pregnant, pid, newborn if mom had chicken pox 5 d before or 2 days agter hosp prem >28w where mom doesnt have varicella or if prem <28w bw 1000g regardless of mother hdx

Question 244

vaccines of mmr and varicella affected by ivig wait 8 months if 300-400mg/kg wait 10 months for 1g/kg wait 11 months for 2g/kg

Answer 244

``` rabies if domestic can monitor for 10 days if not then rabies ig and vaccine esp if bat skunk, fox, racoon or othe rcarnivore notify public health ``` for tteatnus vaccinate if less then 3 doses or its more then 5 year since vaccine for dirsty wound, plus IG

Question 245

items that must be removed from the esophagous 1- sharp and large items or supraabsorpbent polymer 2- compromising the airway, compressing trrachea 3- blocking the esophagous, patient can handle their secretions 4- button/disc battery 5- item is in esophagous 24 hours

Answer 245

``` reasons for stomach removal 1- large item more then 5cm 2- magnets 2 or more 4- button battery there within 48 hours 3- persistent past 4 weeks ```

Question 246

rome criteria for dyspepsia 1x/week for 2 moths 1- upper abdo pain persistnat or recurrent 2- not relieved by defecation 3- no evidence of MAIN -metabolic, anatomic, inflammatory or neiplastic

Answer 246

``` rome criteria for constipation need two of the follwoign 1x/week for 2 months TIPPOR less then 2 stools per week inconteincnce pain with defacation postural retention obstructing hte toilet bowl rectal diameter is extended and large ```

Question 247

abdo migraine 2x over 12 months 1- severe or paroxysmal abdominal pain thats there for an hour perimunbilical 2- well in btwn for weeks to months 3- having 2 of 6: nausea headache pallor vomitting photophobia anorexia 4- not explained by main 5 - intefere with activity

Answer 247

functioanl diarreah - 6 mo to 36 mo - during waking hours - more then 3 soft stools per day - more then 4 weeks - no FTT colic - crying and discomfort with no apparent cause - 3x/week for 3 hours or longer - no ftt

Question 248

factors increasing GERD - due to LES transient relqaxation, obesity, asthma, large vol meals, resp effot, straining most gone by 1 year old, peak is 4 months

Answer 248

clinical sigs - regurg, arching, hoarseess, vocal cord noduels can diagnose wiht ph monitoring, usually its clinical, upper gi, endoscopy for erosive esophagitis can treat with thickened feeds add 1 tbsp rice creeal to 1 ounce formula, hypoallergenic diet, avoid acidic food, weight loss if obese, small vol more frequetly position prone and upright, elevate head when sleeping, and hold infant 20min upright post feeds, can trial omeprazole

Question 249

h pylori thnk in family has gastric ca, or MALT, or iron def anemia refractory to treatment, NOT FUNCTIOANL abdo PAIN treat with triples omep, amox, clarithro, x14d, daignosis is via upper endoscopy with bipsy and uclture tract treatment response with urea breath test and or stool antigen makrers 4 weeks after abx stopped

Answer 249

rf for ulcer - fmhx, drugs, spesis, hypotension, h pylori infection peptic ulcer gastric if pain with eating, and weight loss and acid blockers help durodenal if pain after meal, eating releies pain and awakens patient at night

Question 250

diarrhea if more then 10ml/kg/d of stool or 200g over 24 for teerns in less 14 d OSMOTIC diarrhea- lactose in tolerance, sugar from toddlers

Answer 250

osmotic diarrhea gets better with fasting secretory diarrhea - like congenital sodium, or chloride or tufting enteropathy can also cause diarreha or micorvillus inclusion disease or neuroendocrine tumors, cholerheic diarrhea (from TI removal and not abs bile acids), <100mosm/kg in the stool

Question 251

functioanl diarreha painless passage of 3+ stools large unformed durng waking hours for 4+ weeks, onset in infancy or preschool years and no FTT

Answer 251

osmotic causes | - lactase defiiciency, glucose galatcose malabsoprtion, lactulose >100msoms

Question 252

causes of acute diarrhea infant achild acolsencet

Answer 252

infant - gastro, systemic infxn, abx, overfeeding child - gastro, food posion, abx, HUS, intuss, toxic ingestions adol - gasto, food, abx, hyperthyroidi, appendicitis

Question 253

gastroo bugs and tx campylobacter - RA GBS, from pets, farms, poultry 10 days , erythromycin ecoli o157 h7 undergooked beef, check for shiga, can e associated hus, so monitor renal ABX PROMOTE HUS there is Etec - non hemmorhagic can treat in severe cases wiht septra b cereus is the one from fried rice and refridge meats

Answer 253

listeria is from soft cheese and unpasturatized milk and deli meats and hot dogs- during pregnancy can elad to premature stillbirth and babe can get mengingits- if mo mhas invase disease giv septra salmonbella- treat iny typhi and paratyphi - amp gent or septra shigella causes painful defecation- usus contaminated food or water- fecal oral, like salads, can try septra yesersinia is appedicitis like, can havescarlatinjiform rash, undercooked pork tofu , can get erythema nodosum and ra, Ttreat with septra or doxy

Question 254

IBD- no induction with Azathioprine/6-MP, no maintenance with steroids uc- panca neg, asca positive, transmulra, granuloma, fistula and absess, perianal disease, also thiknk of bechets labs - left, crp esr, low hgb, high plt, cd has patchy ulcers, cobblestoning

Answer 254

UC = NO granuloma or transmural doisease, limited to mucosa, have hematochzie and pus passage, no perianal disease or IC invovemnt - also uc get adneo carcioma based on extent and duration of disease or presnece of PSC, 4-8% per decade of disease, p ANCA positive, ASCA neg

Question 255

neonatal hypothyroidism findings - macrocephaly - enlarged/wide fontanelle - macroglossia - umbilical hernia - brady - constipation - jaundice - goiter - absent distal femoral epiphysis - lymphedema of extremties - hoarse cry

Answer 255

neontal hyperthyrodism - HSM - tachy - flushed - exopthalmus - goiter - HTN - craniosynostosis - iugr - hypolglycemia , - chf

Question 256

``` fasd features - upturn nose midface hypplasia - short palpebreal fissure smooth filtrum thin upper lip - septal cardiac defets ```

Answer 256

``` IDM baby findings hypoglycemia macrosomic birth asphyia increased hcm small left colon syndrome hypo Ca and hypo MG rds bpd polycythemia caudal regression polyhydramnios ``` - HAVE LESS surfactant production

Question 257

oligohdyramnois <500 polyhydamnios .>2000 trisomy 21 have high beta hcg and inhibin a on IPS first trimster pappa a and nuchale 2nd trimester is MSAFP, BHCG, INHIBIN A AND ESTRIOL

Answer 257

NAS signs of sweat, sneeze mottle, poor feeding, diarrhea, inability to sleep methaodne sx day 4 DONT USE NALOXONE dilatin - fetal hydantoin syndrome - cleft lip, short nose, depressed brdige,hyperteolirsm, digit and nail hyplplasia iugr - no ecg findings

Question 258

warfarin - nasal hypolplasia, depressed nasal brige, stippled bones lithium- ebstein anomaly, arrythmia, DI, seizure VPA - ntd, cardiac, thin finger PB- ceft lip, cardiac, hemm disease of newborn

Answer 258

lbw primary cause 705 of death <2500g | black smoking

Question 259

``` iugr babies adaption from genetic and envt factors - fetal - rubella, infxn, chromosme, placental insuffiency maternal - htn, poor nutirition etoh smoking ``` babe sx- temp low,. hypoglycemia,MAS, hyperinsulin, polyechtemia, , hpoxemia, dec glu stores u can be iugr but not sga - which is placental insuff of parents that LGA

Answer 259

surivival and BAD/SEVERE neural outcome for 22w = 18%, 31% 23: 41 AND 17 24 =67 AND 21 25=80% SURVIVAL AND 14% have severe NDD but most have 70% mild if less then 22 w or 24w3 <350g then dont resus 23-25 individufalized if 25 has other issue 25 plus u shud active resius

Question 260

fhr NST normal is there re more then 2 accel within 20min and inc of 15bpm lasting 15 sec nromal variabiltiy is 6-15bpm and if low think of tazchy sleeping distress dec cerebral oxygen decells - early is head ompression, variable is umbilical cord compression late = is uteroplacental insffuciency - checkc fetal acidosis on scalp

Answer 260

ttts - mono di most common mono mono can too (high risk cord entanglement) - the recipeint does worst - both can get hydrops hgb diff of 50, 20% of weight diff fetal pa02 35

Question 261

fetal circulation ductus venosis brings back oxygenaded blood to IVC from placenta goes into RA, most then shunt to LA - through FO - then to LV THEN AORta- most oxygen to brain, then go down to body and umbilical artey small from RA goes to RV THEN through pda back to aorta mainly, small amount to the lungs

Answer 261

TRANSITIONIN - cut the UA - that increases sytemic resistance - baby breathes and cries, this increasea teh resistnace on pulmonary side pfo closes as la pressure>ra pressure pda closs when arterial oxygen rise usually 1 day renal failure if cr >133

Question 262

``` apgar activitiy tone pulse 100 grimace cry/irritible appearance or color resp ``` if baby cold can get pulm hemm, pink skin color, apnea, brady acidosis

Answer 262

erbs palsy- C5-C7 = WAITER TIP - intenral shoulder rortation, adduction shiulder, elbow extension forearm pronation, preserved grasp and wrist and finger flexed klympe is c8-t1 clawlike and may have horner, paralysis of uppe rarm better then if its lower arm total injury c5-t1 worst prognosis , usu need surgeryt

Question 263

workup for neonatal seizure hie - 12-24h ivh 1-3d, prterm hypogly 1-2h or 24h glu, na, ca, mg, bili culture csf and blood head imaging eeg not seisure if suppresible, or can be provoked, vitals are stdable, eeg normal and lack of eye involvement/deviationam

Answer 263

NAME 4 ways to try and prevent IVH and 4 risk factors gr 1 germinal matrix, 2 is in ventricle but not enlarged, 3 venticule enlarged, 4- blood into cortex its bleed in subepyndymal germinal matrix bleed antental steroids, indometh, delayed cord clamp rf- pda, pntx, mechanical ventlaition, asphyxia, heart failure, bicarb therapy longterm can lead to hydrocephalus, infarcts and PVL

Question 264

RDS RF - prem, gdm, immature l/s ratio, fetal distress, cold c section white male xray shows ground glass appearence with air broncho grams and dec lung volumes - mechanical ventilaiton makes it worst goal to maitain pa02 at 60-70 ph >7.25 BPD oxygen at 36w PMA xray spnge like, atlectastis, cysts and overdistended 26w - 50% need oxygen at 36w r heart failure phtn, hypercapniea, bronchoconstriction and fluid retention

Answer 264

``` TTN RF - male macrosminia, csection without labour fmhx of asthma gdm twin pregnancy ``` xray shows perihilar streaking, fluid in fissure, bilteral infliltates HYPERinflation can observe for 2 hours THEN investigate if not better . - r/p pmeumonia cxr cbc diff anti bx amp and gen d/c when rr under 60x12h NO ROLE FOR EPI NEB OR LASIX resolves by 72 hour to 5 days

Question 265

premature infants respod to hypoxemia with APNEA APNEA is not a risk factor for apnea apnea resolves by 36w

Answer 265

can give caffeien which increases minute ventilation, diaphragmatic activity, dec periodic breathing and hypoxic depression of breathing

Question 266

CCAM VS sequestration CCAM - Commnicates wiht bronch/tracheal tree, blood supply from pulomary styem, cxry shows shift away from the lesion sequestration - no comm with bronch, and blood supply from aorta , usu LLL

Answer 266

with congenital lobar emphyesema may be LUL and may be due to cartilagenous defect, causes ++ shift and atelecatsis of opp side diaphram flat due to hyperinflation\ CDH- occut within 8-10w can have cns, atresia and cv and intestinal issues t21.18 and turner inc risk scaphoid abdo, chest wall diapmeter inc, BS in cest and displaces cardiac impluse put cxr and ng tube, intubate keep pip less then 25 to avoid barotramauma na daim 85% sat - permissive hypercapnea ph goal 7.3 or above

Question 267

with TEF avoid ETT type c most common where distal trach attached to esophagus E is where they are paralllel and just have connection A noting is connected b distal stomach is not connected

Answer 267

``` htn neonate most common causes 1- uac thrombous 2- cld 3- renal disease - mdkd,pckd, tubrious ``` PDA 2-4 d of life presentation with heart failure treatment with indo, direutics and fluid restriction - CI to indo/nsaid = bleeding, low plt <50, serum cr 180/oliguric

Question 268

NEC xray- thickened loops, pneumoperitoneum, pneumatosis itestinalis, intrahepatic portal gas amo gent flagyl npo rf- cardiac lesions, prem, enterla feeds, abn bateria

Answer 268

hirshsprung associations waardenberg - check hearing too, whitlock smith lemli optz -dyndactyl 2-3 toe, agen corpis colloseum - ALSO GET gastroschisis heddad - central hypoVentiliation down syndrome CHH bardet biedel- obesity, retinitis pigmentosa, polydactyly, hypogonadism, and renal failure

Question 269

5 long term side effects of gastrschisis bad gut, - leading to short gut syndrome ``` 1- diarrhea 2- bacterial overgrowth 3- renal stones 4- ftt 5- intenstinal hypelrplasia ```

Answer 269

``` aps 1 APECED aire mutation candida hypoparathy addison hypothy ``` ``` aps2 thyroid dm1 addison NOT HYPOPARATH ```

Question 270

hyperbili long term effect - only uncong is neurotoxic SNHL dyskinetic CP upward gaze CP intellectual impairment

Answer 270

UGT1A1 glucuronosyltransferase 1-1 1% in terms compared to adut asians have mutations i this HIGH RISK FACTOR SEVERE BILI

Question 271

``` RF - for HYPERINSULINSM 1 SGA 2 hie 3 prem 4 IUGR 5 pre eclampsia ``` tend to have high inyslun kow ketone sand low ffa glucagon inc glu by 2mmol

Answer 271

delayed cord clamping - theres 20-30ml/kg of blood for prem it prevents IVH, nec and anemia for term prevents iron def anemia it can cause polycythemia and jaundice inc PVR nad hypoxeia and improved GFR

Question 272

cryo has fibrinogeon factor 8 and 13, vwf

Answer 272

warfarin blocks vit k heparin blocks antithrombin 3 treat dic with ffp and platelet

Question 273

within 8 days of being in hosp - get nosocomial infxn as neonate, thhink more CONS, staph aureus and gram nef- treat with vanco and aminoglycoside

Answer 273

polycystic kidney disease RECESSIVE IS BAD- get oligo, large cysts wiuth iver involvement and can have HTN and hypoplasia ad COMMON - NOT BIRTH findigns adn can have cysts in liver pancreas and spleen multicystic dysplastic kidney - abdo mass

Question 274

HARDUP- hyperalimination, acetazolamide, rta, diarrhea, ureto sigmoid fistula pancreatitc fistula NON ANION GAP ACIDOSIS

Answer 274

``` PKU- enzyme PAH or BH4 nt working and binds to bbb transporters mousey smeol prominent maxilla enamel hypoplasia seizure, athetosis ``` CONFIRM with phenylalanine do bh4 loadin test phe>360 treat with restriction of phenlalanine

Question 275

``` b6 - pyridoxine - treatment for <18mo seizures INH therapy in babe homocystinruia ```

Answer 275

MSUD - leucine enceph - 3 aa essential - lecuine isolecuine and valine can present at 2-3 days FENCING AND BICYCLING, hypertonia and hypotonia enceoh, cerberal edema can get acrodermatitis enteropathica like rash diet low in branched chain AA

Question 276

organic adidemia - NEUTROPENIA, HYPOGLYCEMIA, high anion gap high ketones acylcarnitine and UOA is important present in first month usually, esp when ill IVA- smelly feet, low plt,low neutrophil, give glycine, carnitine MMA- b12 def, dystonia due to globus pallidus stroke, hepatomegaly, optic nerve atrophy, pancreatitis glutaric acidemia biotinidase deficiency and holocarboxylase deficiency - aloepcia, pid, metabolic acidosis, seizures hypotonia

Answer 276

propionic acid - increased proprionic acid symptoms within first month present 1w-1month with dehdyatioon , constipation vomitting hypotonia DESTRUCTION of basal ganglia eleavted c3 on acycarnitine - measure enzyme in fibroblasts and treat with fagyl give l carnitine and biotin guarded outcome

Question 277

isolated hepatogmegaly- GSD - van gieke, pompe sphingolipidosis - ALSO splenomgeg -NORMAL INTELLIGENGENCE- niemen pick, gaucher, can get supranuclear gaze (like kids with bili issues)

Answer 277

``` sphingolipidoises include GANGLIOSIDOSES AR- check for regression at 8 motnsh - MACULAR CHERRY RED SPOT tay sach and sanhoff look for b hexosaminidase a and b ```

Question 278

epigastic hernia - need srugeyr dont usually resolve umbiilcial resolve by 5yo - but if mrie then 1.5cm less likely or if underlying condtion like bws, no point of strap dressing, can repair if increasing size

Answer 278

``` '2 of mekel 2yo 2inch 2ft from IC 2% 2x more in male 2 tissue - oanc and stomach ```

Question 279

AP grading - 7-9mild = rpt in 3 weeks post natal moderate 9-15mm rpt in 3 days post natal severe 15mm rpt in 24 hours post natal

Answer 279

unilateral ageneisis f the kidney = GDM and african americans most common contact spots okay turner VACTERL are associations year BP and urnialysis and make sure on ultrasound it sgrowing normal duplex collecting systems dont need investigation unless theres dilation

Question 280

testicular torsion signs SHARN swelling, hard, absent crem, riding high, n/v swelling and testis hard msot worrisome bell clapper prerdisposis - spermatifc cord torsion

Answer 280

dsd - karyotpype 17ohp AND ULTRASOUND

Question 281

differential for psych MAAPSSB RULE OUT cbc lytes renal lft, tsh drug screen urine and serum other to cosnider is extended lytes, albumin vit b12, rbc folate, prolactin lipid profile paroxeitine has highest potential for discontiinuition syndrome - dizxy, nausea paresthesia tremor anxiety flu

Answer 281

``` MOOOD ANXIETY ADJUSTMENT PSYCHOSIS SUSBTANCE SOMATIC BEHAVIOUR ``` side effect of antiudepressanat-sexuadysfunction, nausea, insomnia, headache and gi, dont stop abruptly except for fluoexetine anxiety improves 1-2w, depression 4-6w

Question 282

buproprion - can cause seizures at higher dose and inc risk of psychosis

Answer 282

depression self- worthless env;t harsh future - hopeless anxiety self- inaequate envt scary future - unrepredictable

Question 283

b1 deficiency - thiamine

Answer 283

wet beri beri cardiomyotpahy | dry beri beri encephalopathy - nystagmus think wernicke

Question 284

PLE treat with mct and protein forumla avoid - long chain check for elevated alpha 1 antitripsin and low igg hypaoalbumin, edema, lymmphopenia can get from gastritis, celiac, cmpa, intestinal lymphageniectasia secondary intestinal - from chf and post fontan

Answer 284

four condn assoicated with celiac turner, t1dm, down, iga def williams barley, rye oats and wheat non gi manifestations dermatitis herpetiformis, hepattitis, , arthritis, epilepsy with occipital calcifcation, ostepenia, shrot stature, dental enamel erosions villous atrophy, hyperplasia of crypts and abn surface epithelium + FULL remision when withdraw gluten

Question 285

niacin vit d3 deficiency

Answer 285

pellegra dermatitis - sun distrubtion, diarrhea, dementia and death mainly wher they eat corn if too much get itching and irritation, myopathy

Question 286

function of liver

Answer 286

vit k clotting factor syntehsis production of plasma proteins like albumin and fibronigen energy metabolism of ch and fats storage of adek bilirrubin metaboism drug metabolism and detox immunologic

Question 287

liver failure is 1.5 inr with encep or 1.9 wihtout encephalopathy if have tylenol can be upto 6

Answer 287

function inr ptt albumoin glu ammonia

Question 288

fredireichs ataxia | lower motor neuron abn, absent reflexes, progressive ataxia, normal congition, ggg reperats

Answer 288

occult causes of pain for colic | torniwute, corneal abrasion, oste, #, om iuti,oral ulcer insect

Question 289

what is the dsm 5 for developmental coordination disorder 1, achivement of motor skills as expected for age 2. sig inteferes wtih ADL and impact school play 3. began early in developemnt not bettr explained by another path condition assoc with adhd, ld, asd

Answer 289

with ID need 3 criteria 1. adaptive function dec 2. intellectual function dec 3. <18yo mild - can still marry and have kids mod - need support,housing and emplyiment support severe- lvfe skill in school shortened life expectnacty

Question 290

``` lower gi bleed fissure most common cmpa NEC hsirsprung enteropathy swallowed maternal blood meckel - 2% have complications ```

Answer 290

``` older kids think infectious meckels hus hsp polyps ibd ``` if unwell - hemorrhagic gastritis or esophageal varices

Question 291

thumb 1-schwachmon diamond - bifid thumb, panc insuff 2- rubenstein taybi - broad thumbs, short stature 3- fanconi no thumb 4- dba - three thumb

Answer 291

4 meds causign pancreattis azithaiprine asparginase vpa 6mo need 2/3 , sx peak 24h 1. abdo pain suggstive pancreatis 2. amylase 3x 3. iamging findings sufgestive watch for complicatio- absess pseudocyst, thrombosis, shock, hypoca arf, ileus,

Question 292

function if liver

Answer 292

vit k clotting factor syntehsis production of plasma proteins like albumin and fibronigen energy metabolism of ch and fats storage of adek bilirrubin metaboism drug metabolism and detox immunologic

Question 293

treatment of parasomnia vs night mare and differences

Answer 293

parasominia, usually 1/3 of night in NREM, its non arousalbe and they cant remember it, there is a family hisotry - treat if >3 per week byw aking them up before ALSO ensure safety - if sleep walking and bz are last line Night mares are REM and later on in sleep and can remmeber and no family and rousable, vivid.

Question 294

derm epidermis - stratum corneum is the most superficial -and barrier to microbes irritants and transpidermal water loss neonates have normal skin - funcyional not normal

Answer 294

children heal more quickly no evidence for adding oil to bath emolliants have lipids if need for dry skin 1g covers 10x10 -if its ointment increase that by 10% - usually on damp skin except - calcineurin inh or toxic

Question 295

5 causes of erythema nodosum

Answer 295

``` IBD SLE scaroid TB lymphoma EBV ```

Question 296

5 fcauses of erythema multiforme

Answer 296

hsv, mycoplasma adeno, tb, ebv, meds - NSAID, sulfonamide, hiv, anti arrytmic

Question 297

name for causes of photosensitivity

Answer 297

dermaotoses- sle amd jdm exogenous- tetracycline, nsaid, retinoids pseudoprophyria- bullous phodermatsosi with no abn in porphyrin metabolism - meds NAPROXEN most common cause

Question 298

what are patients with congenital melnocytic nevi at risk for

Answer 298

medium and large ones >15 and 20cm | malignant melaonma - usus remove at adolescent

Question 299

when to biopsy a skin lesion | manage niv with skin sun block, avoiding sun, skin exam, phtoography abcde

Answer 299

``` = bleeding, under nail, growing asymetry border irregular concering variable color diameter is >6mm evolution and changing ```

Question 300

mongolion spots or slate grey nevus is associated with?

Answer 300

RARELY | melanoma, cleft palate issues, and spinal meingieal tumor

Question 301

causes of cafe au lait

Answer 301

``` mccune albright russsel silver noonan WAGR fanconi leopard syndrome nf1 ```

Question 302

name 5 phakomatos disease | ocular neuro cutaneosu

Answer 302

``` nf1 tubrous at sturge weber von hippel landau incontentia pigmenta ```

Question 303

molluscums treatment

Answer 303

its a virus poxvirus elf limited 9 months ry cantharidin but not on face imiquimod (can also use for warts, or salicyilic acid)

Question 304

What's in a Mnemonic: encapsulated organisms One of the most high yield mnemonics in microbiology is the one for encapulated organisms: "Some Nasty Killers Have Some Capsule Protection":

Answer 304

``` Streptococcus pneumoniae Neisseria meningitidis Klebsiella pnemoniae Haemophilus influenzae Salmonella typhi Cryptococcus neoformans Pseudomanas aeruginosa ```

Question 305

metabolic disease with liver failure

Answer 305

worst -OTC | MCAD

Question 306

causes of neutropenia

Answer 306

1. organic acidemia 2. cyclic 3. schwachman diamonond 4. congenital neuropenia kostmann 5. viral 6. autoimm neutropenia of childhood 7. chediak higashi sx

Question 307

cgd has hyper gam,penumonia and osteo, colitis, gingiitis

Answer 307

stah aureus serratia aspergillus salmonella

Question 308

for gvhd need

Answer 308

1- histocompatible diff btwn gradt donor and recipeijnt 2- immunocompoentn cells in graft responding to host 3 host being incompentent preventing rejection