Memorize Flashcards
What is age of consent for sex?
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
Birth control - OCP
Absolute CI
s/e
Also depo s/e
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
Diagnostic criteria for Anorexia nervosa and hospital admission criteria
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)
diagnostic criteria for Bulimia
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)
Define 1 key characteristic for age -
EARLY adolescence
MID adolescence
LATE adolescence
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
which tanner stage does
1) testis grow
2) scrotum enlarges
3) phallus length increase
4) scrotum darkens
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
for females which tanner stage does
1) breast bud
3) breast tissue beyonf areola
3) secondary mound
4) hair not on thigh only vagina
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
what are four things to rule out before diagnosing PUBERTAL gynecomastia in males?
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)
What are indications for tubes
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
with TM perforation what is false
1) most heal iwthin 6 weeks
2) you cannot use ciprodex
3) repaired at 10yo
you CAN use ciprodex drops and should be used.
12 yo draining left ear for one year - what is likely diagnosis
1) cholesteatoma
b) AOME
c) Otitis externa
d) perforated AOM
cholesteatoma - unilateral foul smell
persistant and recurrent and responds to ototopicals but recurs by months
(intracranial complications in 0.36%)
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
branchial oto rental sx
(AD - would also be in parents)
monidni is inner ear issue with 1.5 coils instead of 2
what are the ABCDs of hearing loss
50% acquired
50% inherited
- connexin 26 most cmoon genetic AR cause , transmembrane protein that recyclkes K
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
13yo M wiht severe chronic epistaxis with normal bleeding tests 1- rpt tests 2- transfuse prophylactic 3- refer to ENT d- start steroids
REFER - need to rule out juvenile nasal angiofibroma
secondary indications of OSA include?
enuresis ADHD daytime somnelence FTT cor pulonale
name 6 causes of stridor organized:
supraglottic
subglottic
and tracheal
laryngomalacia, epiglottitis
subglocttic- stenosis, hemangioma, croup
tracheal- tracheomalacia, FB, complete tracheal ring, TEF
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
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
what are 4 absolute indications for T and A
and 4 elective ones
risk of re bleed 7-10 days after seperation of eschar
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
name tumors of the necl
small round blue cell tumor - lymphoma,r habdo, ewing
thyroid carcinoma
neuroblastoma
langerhand cell histiocytosis
Marfan disease associated with which heart defect
MVP (mid systolic click), dilation of ascending aorita
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
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
which long QTc associateed with hearing loss
Jerve Diel Nielson
treatment is with beta blocker
cannot interpret QTc if have WPW or bundle branch block
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%
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.
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
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
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
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
what are the four innocent murmurs
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)
classic places for these murmurs to be heard?
ASD AS PDA PS VSD
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
what are two genetic conditions that cause LQTS and their associated findings
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)
name four causes of acquired LQTS w workup hx/physical ecg serial parental ecg holter echo (exercise test u have to watch them after)
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
WPW - three findings on ecg
two outcomes possible
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
SVT presentation
ECG findings
Tx
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
match these conditions with associated cardiac abn
t18-edward t13-patau VACTERK turner FAS digeorge william alagile marfan
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 dilationname 5 duct dependant lesions
transpostion of GA with intact septum PS PA AS TOF severe coactation hypoplastic L heart
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)
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
Diagnosis and treatment of Tet spell
most common cyanotic CHD
repaoir best at 4-6mo
still at risk of cardiac death, arrythmias, RV dysfunction
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
presentation timing of heart lesions
NEWBORN
6 weeks when PVR drops
LATER
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)
Managemnet of HLHS
Longterm complications arrythmias protein losing enteropathy cyanosis for AVMs and collatorals plastic bronchitis lymphangectasia
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
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
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
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
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
what are risk factors for pHTN >25mmHg (normal 8-20)
MAS, pulm hypoplasia, early onset sepsis, hypoglycemia, RDS, polycythemia, matenral NSAID
treatment oxygen intubate inhaled ntiric anticoag CCB phosphodiesterase inhibitor (sildenafil) transplant
differnetial for pulsus paradoxus (change in BP by more hten 10 with inspiration (SBP)
OSA chronic pericarditis cardiac tamponade tension pneumo asthma pregnancy
Rheumatic fever
fidings and secondary treatment to prevent
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
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
first week its HLHS
severe AS
coarctation
2-6w: VSD, AVSD, PDA (NOT ASD)
adolescemt: DCM, myocarditis, tachycaridas
name six causes of primary amenorrhea (14 without breast development or 16 with breast development)
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
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
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
Describe how anovulation works with physiology and H
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.
effects good and bad of estrogen and progesterone
Prevents ovulation, thickens cervical mucus, inhospitable endometrium. blocks sperm penetration
reduces ovarian and endometrial cancer
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
with RAPE
investigations and treatment
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
STI- investigation, sx, treatmebt
BV
Trichomonas
Candida
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
differential for red lesion on labia
1 -chancre (syphillis) tropenoma pallidum 2- chancroid 3- herpes 4- trauma 5- HPV
6 A’s of smokiing cessation\
can use patch plus CBT
ask about smoke status advise to quit assess readiness assist with effort arrange fu anticipate risk of use in adolescent
what are side effects of androgen and steroids
some athletes also use GH: causes insulin resistnace, hyperglycemia, DM, Na retention, cardiomegaly, carpal tunner
hepatotosixc, gynecomastia, erythrocystosis, premature fusion of epiphseal, infxn, virilization in women
6 things for adolescent transition u can do
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
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
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
Proteinuria
Diff btwn nephrotic and nephritic
remember proteinuria predicts CKD progression in children
Nephrotic: proteinuria (>40mg/m2/hour) or 250mg/mmol, edema, hypoalbumin<25, high TG
Nephritic is PHAROH
proteinuria, hematuria, azotemia, RBC cast, oliguria, HTN
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
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
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
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)
Differnetial for GROSS hematuria Glomerular: deformed red cells, casts, acanthocyte Interstital: Malignancy Tract-
Glomerular: nephritis (post strep, IgA, HSP) or alport, benign familial hematuria
Interstital: pyelno
Malignanc: wilms
Tract- UTI, stones, FB
post strep glomerulonephritis
order of resolvement of
HTN, Proteinuria, C3, hematuria
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)
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
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
Diagnosis of SLE
4/11
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
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
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
post transplant kidney monitor
CMV
EBV
opportunistic infxn
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
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
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
Prune Belly syndrome TRIAD?
associations?
(renal dysplasias in general assocaited with reflux, PUV and spina bifida)
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)
HUS - triad? - investigations - risk factors for dveloping prognosis
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
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
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
Causes of Hypernatremia
Hypervolemic (Na excess)
Euvolemic (water deficit)
Hypovalemic (water and Na deficit)
Symptoms - thirst, irritabile, vomit, restless, lethargy, weak, coma
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)
Causes of hyponatremia
symptoms, nausea, letahrgy, seizure, headache
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
HyperK causes
(remmeber RTA 4 has hyper K)
HypoK causes
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
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
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
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
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
with ARF - causes,
lyte abnormalities? K, Ca, phosphate
management
if its CKD - supplement Ca, get phosphate binders (CaC03) and restrict phospahte. need epo
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
workup for HTN and organ damage workup
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.
RF for renal vein thrombosis
common form in neonates not assoc with vascular catheter
RF-
prem, asphyxia, dehydration, polycy, CHD, maternal diabietes
present with flank mass, hematuria, low plt
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
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
proximal vs distal RTA
think of it when its non anion gap hyperchloremic metabolic acidosis
remember type 4 has hyperkalemia
=with aldoesterone deficeincy
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
name causes of HYPOnatermic, HYPOchloremic, HYPOkalemic metabolic alkalosis
lasix use
PS
CF
BArter
indications for dialysis
uremia wiht sx severe lyte derangements high ammonia drug- salcicyalte,e thylene glyclol, methaonl, isopronaol, fluid overload
causes of wilms tumor
NOT li fraumeni (AD, leukemia, p54, breash scarcoma and leukemia)
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
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
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
Tuberous sclerosis
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
Diff btwn homocystien and marfan
MARFAN - AD, superior ectopic lentis, no hyperoacgulable, tx with Losartan to dec TGF beta (prevent aneurysm)
Homocysteine- AR, hypercoagulable, inferior ectopic lentis
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
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
Monitoring in BWS
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
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
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
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
incontinentia pigmenti
RETT syndroime- mainly FEMALES
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
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
Expansion of trinucleotide rpt mutation includes fragile X, what are the rpt and some findings in premutation
Other conditions: Huntington, Freidrecih ataxia, Myotonic dystrophy
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
MATCH these with upslanting or downslanting or narrow palpebral fissures
T21, prader willy Marfan FAS William Difeorge Sotos Lennox Gatuaut\ Ehler dan
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
Fetal efects of maternal drugs:
1) tobacco
2) cocaine
3) matenral insulin
4) phenytoin
5) alcohol
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
what is the most common trisomy 16 21 13 18
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
Management t21
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
High risk ALL stratification name 5 things
age <1, >10 wbc >50 000 hypoploidy cns positive disease mrd
William syndrome
7p deletion
perfect pitch and musical abilities
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
what does CHARGE AND VACTERL stand for
think CHARGE is heart and above (no renal stuff)
coloboma, heart effect, atresia choanael, retarded, genital, ear
vacterl
vertebrae , anus imperforate, cardiac, tef, renal, limb
AR disorders
smith lemli optiz -cholesterol metabolism issue 7DHC
short stature, microcephalic, mod/severe disability, syndactyl of 2-3 toes, geniterlalie may be ambiguas in male
dmd - x linked recessive
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
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
cmv waardenberg - also synophyrns and hirshcpsring turner down syndrome haddad syndrome
ehler danloe
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
random conditions to know
mccune albright
abetalipoporteiniema
bardat biedel
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
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
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
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
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
pulse oximtery
R hand and either foot 24-36 hours
a pass is >95% with less then 3% diff
Whats a fail?
<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
HSP diagnosis
small vessel IgA vasculitis
normal or HIGH platelet mild anemia, WBC elevated low albumin elevated markers Cr may be higher elevated IgA
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%
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)
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
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
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
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
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
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
treatiwht 10 days pen
need prop pen for 5 years or 21 years old - whatevr is later
if carditis - life long treatment with prop
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
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
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.
cyclophosphamide - skin hyperpig, hemmorhagic cystitis, BM suppresion, vomitting, alocpecia
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
anti ro and anti la ab skin - discoid rash brain -hydrocephalus heart block - hepatitis panyctopenia, stippling og epiphysis
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
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
granulomatosis with polyangiitis
GPA
this is small vesssle disease
C- ANCA
necrotizing granulomatous
treat with steroids, Cyclophosphamide, imuran, retux MTX
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
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
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
You see a biopsy with non caseating epithelioid granulomatous lesions - whats teh diagnosis
sarcoid - children<4 for early onset triad rash, arthritis and uveitis
bilateral hilar lymphadenopathy
differential for uveitis
IBD SLE JIA CMV/HSV bartonella - unilateral syphillis TB sarcoidosis
differential for erythema multiforme
mycoplasma ebv cmv NSAID sulfonamide hsv HIV IBD SARCOID
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
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
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
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
name four causes for congenital scoliosis and acquired scoliosis
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
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
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
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
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
RF for DDH
L hip more common
neonate- barlow and ortalani
untreated - scoliosis, limb length inequality, arhtritis, hyperlordosis if bilateal, ipsilateral knee pain
first born, female, breech, family hx, any tight intrauterine space - oligo, large weight, twins, swaddling tight-acquired by bringing hips together
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
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
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
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
Diff and similarities
btwn SCFE and Legg perthes
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
RF for AVN
steroids alcohol sickle cell SLE trauma, antiphospholipid ab gaucher disease (peroxisomal/lysosomal d/o)
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)
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
osteo - usuall staph with
neonate - GBS, <5yo kingella, sickle salmonella and bartonella in pelvis and verebrae
usually femur then tibia, usually long bone
tx ancef +/- clinda or vanco
if sickler then cefotax and clinda/vanco with 3-6w
Differential of varous
Rickett findings- craniotabes, frotnal bossing,r achitic rosary, widening of wrists and ankles (cupping metaphysis)
1) physiological: RF obese, fmhx, early walker, resolve 2yo
2) Metabolic bone disease- Ricketts
3) blount disease - medial grwoth plate is diseased-progressive
age it shows, exam, resolving
metatarsus adductus
internal tibial torsion
increased femoral anteversion
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
four exam findings of talipes equino cavo varous
CAVE
TEV- talipes equinas varous
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
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)
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
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
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
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)
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)
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
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
when do primitirve reflex go and which one comes on at 4-5 mo and 9-10 month
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)
when to image a spinal dysraphism
myelomeningocele - aperta worst form need surgery, vs tethered is when spinal cord goes to L2
> 25mm from anal verge or larger then 5mm, mass or lipoma hairy patch, dermal sinus, vascular lesions
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
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
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
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
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
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)
abscence seizures 3hz spike peak 6yo treat with ethosuximide or valproic and usu resolve by adolescnece, common to hv psych or beh comorbidities
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
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)
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
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
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
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
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
sturge weber - angiomatosis of leptomengingis, causes glacuoma nad port wine stian of v1v2 of CN5, also get seizures, hemo paresis, headache. dev disbality, hemianopsia
PHACES post fossa hemagnioma arterial anoalies coarct aorta eye abn sternal cleft/supraumbical raphe
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
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
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
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
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
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
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: 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
sturge weber - angiomatosis of leptomengingis, causes glacuoma nad port wine stian of v1v2 of CN5, also get seizures, hemo paresis, headache. dev disbality, hemianopsia
PHACES post fossa hemagnioma arterial anoalies coarct aorta eye abn sternal cleft/supraumbical raphe
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
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
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
septo optic dysplasia
- optic nerve dysplasia
- absent septum pellucidum
-agenesis of corpus callsum
-migration anomalies
-encephalomalacia
posteiror pit ectopia
mri and endo eval
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
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
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
adrenoleukodystrophy- neurodegeneratve disoder
sphingolipidoses can also have defecting cellular membranes due to problem of lipid substrates - ie) krabbe diesease
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
dandi walker similar to chiari 2 but expansion of 4th centricle and needs a vp shunt
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)
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
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
septo optic dyspalsia
- optic nerve dysplasia
- absent septum pellucidum
-agenesis of corpus callsum
-migration anomalies
-encephalomalacia
posteiror pit ectopia
mri and endo eval
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\
vital capacity is what you breathe in and out regularly - which is your TLC without the residual volume
lungs are grown by 8yo
wht is differential for obstructive lung path
versus
restrictive
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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)
bronchiolitis risk factors for severe disease <3 mo PID prem <35w hemodynamic sig cardioresp disease
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
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)
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
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
causes of pulm hemm-- GPA pulmonary hemosidorosis coagulopathy - vwb sle HSP trauma bronhecistati FB PHENYTOIN
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
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
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
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
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?
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
duke criteria for endocarditis BE FEVEER 2 major or 1 majro 3 minor or 5 minor
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
Jervell and Lange-Nielsen SNHL AR AND LONG QT
autosomal dominant form, the Romano-Ward syndrome, has a purely cardiac phenotype.
nasal prongs - help with humidfying airway helps with mucus clearanace and also creates increased fxnal residual capacity , from distending pressures
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
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
duke criteria for endocarditis BE FEEVER 2 major or 1 majro 3 minor or 5 minor
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
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
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
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
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
GCS
sections and what they mean?
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
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
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)
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
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
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
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
miosis - think opiates, cholinergics (insecticides/organophosphates), clonidine, olanzpine, sedatives,
mydriasis - anticholingic, sympathomimetic (cocaine, pcp), opiate withdrawal
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
MUDPILES CAT -met acidosis elevated ag
methanol, uremia, dka, proponyl alcohol, isoniazid,iron, lactic acidosis, ethalyne glcol, salicilaytes, co and cyanide, alcohol ketoacidosis, tylenol
HYPOGLYCEMIA IS HOBBIES
- hypoglycemic (insulin/sulfonyrea), quinine, beta blocker, insulin, ethanol, salcylates a late effect
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
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
ethylene glycol
- has high osmolar gap at first and then becomes high anion gap as its metabolized
methanol - permanent blindness
- treatment fomepazole
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
-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
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
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
Cholinergic- DUMBELS -
organophoshate/pesticides
diaphoretic,urination, miosis, brady, bronchospasm, emesis, lacrimation, salivation and seizures- atropine and pralidoxime
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
RF FOR polycythmiea
-IDM, placentail insuff, TTTS reciepeint, POSTDATES, delayed cord clamp, neonatal grave/hypothyroidism
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
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
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
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
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
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
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
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
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
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
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
indications for BMA
PANCYTOPENIA AND LEUKOCYTOSIS blasts on smear unexplained sign depression more then 1 periperhline leukoerythroblastic changes on smear unexplaine dlad or hsm ant mediastinal mass
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
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
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)
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
associations of conditions with leukemia
name 8
associations qith LYMPHOMA
think PID
WAS, AT, congenital hypogam, HIV
post solid organ tx
pid - AT, SCID WAS diamond blackfan and schachman diamond paroxysml noctunal hemoglobinura nf1 ds kosman syndrome /scn li fraumeni bloom syndrome
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
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
high risk ALL 1- wbc>50 2- age <1, or >10 3- cns disease 4- unfavourable cytogeneitcs 5- hypodiploid
higher risk NBL n myc amplifiation older 18 months hypodiploid lack of cell differetiation
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
worrisome lymph node >2 cm chronic generalized supraclavicular associated with type b features hard non- tender
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
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
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
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
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
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
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
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)
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
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
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
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
acute vision loss ddx-
retinal detachment
hyphema, uveitis, acute closer glacumoa, papilledema, optic nerve ischemia/inflm/infxn
glaucoma- buphthalmos, tearing , photohphobia, blepheropsasms
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
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
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
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
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
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,
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
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
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
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
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
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
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
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
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
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
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
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.
HSV
stays in trigeminal ganglion
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)
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
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
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
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
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
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
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
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
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
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
traveller workup vision and hearing cbc diff lft renal serology cxr and urine and o and p dentitition vaccine catch ups
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
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)
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
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
- 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
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
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
hiv - heterosex most common transmission for females
- hyper gam, dec cd4 and no ab responses to vaccines
cns involved in children more then adults
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
functioanl diarreha
painless passage of 3+ stools large unformed durng waking hours for 4+ weeks, onset in infancy or preschool years and no FTT
osmotic causes
- lactase defiiciency, glucose galatcose malabsoprtion, lactulose >100msoms
causes of acute diarrhea
infant
achild
acolsencet
infant - gastro, systemic infxn, abx, overfeeding
child - gastro, food posion, abx, HUS, intuss, toxic ingestions
adol - gasto, food, abx, hyperthyroidi, appendicitis
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
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
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
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
neonatal hypothyroidism findings
- macrocephaly
- enlarged/wide fontanelle
- macroglossia
- umbilical hernia
- brady
- constipation
- jaundice
- goiter
- absent distal femoral epiphysis
- lymphedema of extremties
- hoarse cry
neontal hyperthyrodism
- HSM
- tachy
- flushed
- exopthalmus
- goiter
- HTN
- craniosynostosis
- iugr
- hypolglycemia ,
- chf
fasd features - upturn nose midface hypplasia - short palpebreal fissure smooth filtrum thin upper lip - septal cardiac defets
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
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
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
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
lbw primary cause 705 of death <2500g
black smoking
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
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
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
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
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
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
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
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
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
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
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
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
premature infants respod to hypoxemia with APNEA
APNEA is not a risk factor for apnea
apnea resolves by 36w
can give caffeien which increases minute ventilation, diaphragmatic activity, dec periodic breathing and hypoxic depression of breathing
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
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
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
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
NEC
xray- thickened loops, pneumoperitoneum, pneumatosis itestinalis, intrahepatic portal gas
amo gent flagyl
npo
rf- cardiac lesions, prem, enterla feeds, abn bateria
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
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
aps 1 APECED aire mutation candida hypoparathy addison hypothy
aps2 thyroid dm1 addison NOT HYPOPARATH
hyperbili long term effect - only uncong is neurotoxic
SNHL
dyskinetic CP
upward gaze CP
intellectual impairment
UGT1A1
glucuronosyltransferase 1-1
1% in terms compared to adut
asians have mutations i this
HIGH RISK FACTOR SEVERE BILI
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
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
cryo has fibrinogeon factor 8 and 13, vwf
warfarin blocks vit k
heparin blocks antithrombin 3
treat dic with ffp and platelet
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
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
HARDUP- hyperalimination, acetazolamide, rta, diarrhea, ureto sigmoid fistula pancreatitc fistula
NON ANION GAP ACIDOSIS
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
b6 - pyridoxine - treatment for <18mo seizures INH therapy in babe homocystinruia
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
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
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
isolated hepatogmegaly-
GSD - van gieke, pompe
sphingolipidosis - ALSO splenomgeg -NORMAL INTELLIGENGENCE- niemen pick, gaucher, can get supranuclear gaze (like kids with bili issues)
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
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
'2 of mekel 2yo 2inch 2ft from IC 2% 2x more in male 2 tissue - oanc and stomach
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
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
testicular torsion signs
SHARN
swelling, hard, absent crem, riding high, n/v
swelling and testis hard msot worrisome
bell clapper prerdisposis - spermatifc cord torsion
dsd - karyotpype 17ohp AND ULTRASOUND
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
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
buproprion - can cause seizures at higher dose and inc risk of psychosis
depression
self- worthless
env;t harsh
future - hopeless
anxiety
self- inaequate
envt scary
future - unrepredictable
b1 deficiency - thiamine
wet beri beri cardiomyotpahy
dry beri beri encephalopathy - nystagmus think wernicke
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
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
niacin vit d3 deficiency
pellegra
dermatitis - sun distrubtion, diarrhea, dementia and death
mainly wher they eat corn
if too much get itching and irritation, myopathy
function of liver
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
liver failure is 1.5 inr with encep or 1.9 wihtout encephalopathy
if have tylenol can be upto 6
function inr ptt albumoin glu ammonia
fredireichs ataxia
lower motor neuron abn, absent reflexes, progressive ataxia, normal congition, ggg reperats
occult causes of pain for colic
torniwute, corneal abrasion, oste, #, om iuti,oral ulcer insect
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
with ID need 3 criteria
- adaptive function dec
- intellectual function dec
- <18yo
mild - can still marry and have kids
mod - need support,housing and emplyiment support
severe- lvfe skill in school shortened life expectnacty
lower gi bleed fissure most common cmpa NEC hsirsprung enteropathy swallowed maternal blood meckel - 2% have complications
older kids think infectious meckels hus hsp polyps ibd
if unwell - hemorrhagic gastritis or esophageal varices
thumb
1-schwachmon diamond - bifid thumb, panc insuff
2- rubenstein taybi - broad thumbs, short stature
3- fanconi no thumb
4- dba - three thumb
4 meds causign pancreattis
azithaiprine
asparginase
vpa 6mo
need 2/3 , sx peak 24h
- abdo pain suggstive pancreatis
- amylase 3x
- iamging findings sufgestive
watch for complicatio- absess pseudocyst, thrombosis, shock, hypoca arf, ileus,
function if liver
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
treatment of parasomnia vs night mare and differences
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.
derm
epidermis
- stratum corneum is the most superficial -and barrier to microbes irritants and transpidermal water loss
neonates have normal skin - funcyional not normal
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
5 causes of erythema nodosum
IBD SLE scaroid TB lymphoma EBV
5 fcauses of erythema multiforme
hsv, mycoplasma
adeno, tb, ebv,
meds - NSAID, sulfonamide, hiv, anti arrytmic
name for causes of photosensitivity
dermaotoses- sle amd jdm
exogenous- tetracycline, nsaid, retinoids
pseudoprophyria- bullous phodermatsosi with no abn in porphyrin metabolism - meds NAPROXEN most common cause
what are patients with congenital melnocytic nevi at risk for
medium and large ones >15 and 20cm
malignant melaonma - usus remove at adolescent
when to biopsy a skin lesion
manage niv with skin sun block, avoiding sun, skin exam, phtoography abcde
= bleeding, under nail, growing asymetry border irregular concering variable color diameter is >6mm evolution and changing
mongolion spots or slate grey nevus is associated with?
RARELY
melanoma, cleft palate issues, and spinal meingieal tumor
causes of cafe au lait
mccune albright russsel silver noonan WAGR fanconi leopard syndrome nf1
name 5 phakomatos disease
ocular neuro cutaneosu
nf1 tubrous at sturge weber von hippel landau incontentia pigmenta
molluscums treatment
its a virus poxvirus
elf limited 9 months
ry cantharidin but not on face
imiquimod (can also use for warts, or salicyilic acid)
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”:
Streptococcus pneumoniae Neisseria meningitidis Klebsiella pnemoniae Haemophilus influenzae Salmonella typhi Cryptococcus neoformans Pseudomanas aeruginosa
metabolic disease with liver failure
worst -OTC
MCAD
causes of neutropenia
- organic acidemia
- cyclic
- schwachman diamonond
- congenital neuropenia kostmann
- viral
- autoimm neutropenia of childhood
- chediak higashi sx
cgd has hyper gam,penumonia and osteo, colitis, gingiitis
stah aureus
serratia
aspergillus
salmonella
for gvhd need
1- histocompatible diff btwn gradt donor and recipeijnt
2- immunocompoentn cells in graft responding to host
3 host being incompentent preventing rejection
