LAST PEDS! Flashcards
Define Epilepsy
recurrent, UNPROVOKED seizures
SZR that arises from one region of the brain cortex
Focal/ partial SZR
SZR that arises form both hemispheres of the brain
Generalized
4 categories of SZR
Focal
Generalized
Unknown onset
Unclassified
A SZR that begins diffusely think
Generalized
Tonic clonic, Absence, Febrile
Difference between simple and complex focal SZRs
Simple: no altered consciousness
Complex: altered consciousness
MC cause of SZRs in kids
Idiopathic is 75% of all cases
Tx for Generlizedd Motor SZRs
oxcarbazepine or levetiracetem
How to DDX tics vs SZR
Tics are partially under voluntary control
Focal SZRs are preceded by..
Aura prodrome
What are automatisms
unconscious actions seen in focal complex SZRs
Automatic movements commonly of mouth or extremities
Age of onset for Absence SZRs
Around 5-8 yrs old
A 5 yr old haas a brief period of eye flutter and upward eye rolling
Think what SZR D/o? And what is the treatment?
Absence SZR
Treat with ethosuximide
Do absence SZRs present with post ictal phases
NO!
->immediate resumption of activity
MC cause of SZR from age 6months to 5 yrs
Febrile SZR
What is the DDX for simple febrile SZR compared to Complex/Atypical Febrile SZR
Simple: Tonic/Clonic lasts less than 15 min and only occurs x1 in 24 hours
Complex/Atypical: Focal S/s lasts longer than 15 min, recurres several times in 24 hours and/or child has preexisting nuero d/o
What should be ruled out in SZR evaluation
need to rule out meningitis, encephalitis, brain abscess
If focal neurologic signs or papilledema → CT before LP
MRI is imaging modality of choice for detecting brain lesions in subacute setting (in the ED -> CT)
Febrile SZR MGMT
USUALLY NO INTERVENTION NEEDED
High risk pt: Rectal Diazepam to abort SZR >5min
Daily AntiSZR medication NOT needed unless at risk for epilepsy
Do Antipyretic’s prevent Febrile SZRs
Antipyretics during febrile illnesses does NOT prevent febrile seizures
SZR for 30 min or more…
Status Epilepticus
Status Epilepticus MGMT
ABCs
ECG + O2/Pulse Ox
IV access -> Benzo
LABS:
Glucose, BMP, Therapeutic Rx level, Toxicology, CBC+ platelets and Differential
Proper disposition for Status Epilepticus MGMT
PICU for monitoring
Head aches in the morning
Think
Tumor
Headache Red Flags
“Worst headache of my life” → subarachnoid hemorrhage
Morning headache → tumor
Pain awakens child at night
Chronic, progressive headaches (most ominous headache pattern)
Abnormal/focal findings on neuro exam
Changes w/ body position
Recurrent vomiting (especially in morning)
No previous family history
=GET IMAGING!
A younger pt iwth a bilateral, bifrontal headache
Think
Migraine
Migraine Headache
Common recurrent headache in peds
Focal, bilateral and bifrontal
Seeking dark quiet rooms
N./V, pallor, photophobia, phonophobia
+/- Aura
If a kid presents with headache but no FMHx of headaches
Think
A worse secondary cause
Because 90% have a 1st or 2nd degree relative with recurrent headaches
DDX for 2nd Headaches
Head trauma Viral illness Sinusitis Medication overuse headaches Increased ICP—mass, vascular malformation, pseudotumor cerebri
Headache that is worse when lying down or early in the AM
Think
Secondary cause to headache
After a CT w/u for headache what is the next step
If CT negative → obtain a lumbar puncture → measure opening pressure & evaluate for RBCs, WBCs, protein, glucose, or xanthochromia
What is the best way to find posterior fossa lesions
Brain MRI with/without gadolinium contrast (study of choice)
1st line Tx for Migraines
Acetaminophen or NSAIDs (ibuprofen or naproxen)
Adjunct therapies: hydration & antiemetics
2nd line: Triptan agents
What are the contra/I for using triptans for migraines
Focal neurologic deficits with migraines
Basilar migraine signs (syncope) → stroke risk
What drug can you not give to peds with migraine with aura
NO OCPs!
When to start migraine prophylaxis
If more than 1 disabling headache per week
What is pseudo tumor cerebri
AKA idiopathic intracranial hypertension
Elevated ICP with normal brain imaging
S/s : Diplopia, abducens palsy, transient visual obscurations, papilledema
MC Cause: idiopathic
Tx for Idiopathic Intracranial hypertension
Untreated? permanent visual field loss!
Treatment: acetazolamide (or other diuretics), topiramate, or corticosteroids
(Also wt loss and remove any triggering medications)
Retrograde vs Anterograde amnesia
Retrograde amnesia: inability to recall events leading up to trauma
Anterograde amnesia: inability to form new memories after trauma
Variable duration: length of amnesia typically correlates w/ severity of trauma
Highest timeline risk for concurrent concussions
Increased in the 1st 10 days after concussion occurs
Prognosis of SZR with concussions
At time of injury: due to cortical stimulation → excellent prognosis
Within 7 days: typically due to localized edema or contusion → good prognosis
After 7 days: attributed to glial scarring → leads to epilepsy
Role of CT in concussion MGMT
CT not used to diagnose concussion, but rather to rule-out serious traumatic brain injury (i.e., hemorrhage)
What is second impact syndrome
Acute, sometimes fatal brain swelling occurring when a second concussion is sustained before complete recovery from a previous concussion
May cause rapid increase in ICP which is impossible to control
3 deaths every 2 years
Return to play guidelines for concussion
- No activity
- Light aerobic
- Sports specific excercise
- Non contact sports play
- Full contact practice
- Game play
Each stage should be a minimum of 24 hours without s/s to move to the next stage
Most at risk for Post concussive syndrome
Risk factors: younger age, level of play, ongoing clinical symptom, chronic neurobehavioral impairments, catastrophic outcomes (i.e., subdural hematoma)
S/s: Hallucinations, Dizziness, HA, Unclear thinking, sleep d/o
Can last weeks and effect school performance
Refer to NEURO!
Stage IV sleep….
REM (rapid eye movement)
Active, awake-like EEG pattern & muscle atonia
Active sleep: REM sleep in neonates
Who sleeps longer, bottle fed or breastfeed babies
Bottle-fed babies generally sleep for longer periods (2-5 hr bouts) than breastfed babies (1-3 hr).Sleep periods are separated by 1-2 hr awake.
Should peds less than 1 years old
Share the bed
American Academy of Pediatrics issued a revised recommendation in 2016 advocating against bed-sharing in the 1st yr of life, instead encouraging proximate but separate sleeping surfaces for mother and infant for at least the 1st 6 mo and preferably 1st yr of life.
Best sleeping position for babies
On back, firm mattress, do not use pillows or comforters
NML sleep patterns for infants 2-12 months old
great individual variability
At what age does the capacity to self soothe start?
capacity to self-soothe begins to develop in the 1st 12 wk of life and is a reflection of both neurodevelopmental maturation and learning.
NML nap time for toddlers
Naps decrease from 2 to 1 nap at average age of 18 mo.
When do nighttime fears start to develop
1-2 years old
Have a good night routine to abate this
Should preschoolers cosleep
Persistent cosleeping tends to be highly associated with sleep problems in this age-group.
Sleep hygiene for 6-12 years old
School and behavior problems may be related to sleep problems.
Avoid media and electronics at bed time
What is sleep onset associated insomina
child awakens under conditions different from those experienced as they fell asleep and cannot return to sleep independently
Leads to sleep d/o
What is limit-setting subtype of insomina
Bedtime resistance or refusal due to a caregiver’s unwillingness or inability to enforce bedtime rules & expectations
Nighttime fears → also common cause of bedtime refusal
When do night terrors occur
When do nightmares occurs
Terrors: 1st 1/3 of sleep
Nightmares: last 1/3 of sleep
Circadian rhythm disorders in adolescence
Exaggerated delayed sleep phase
Leads to inability to arouse in the mornings & failure to meet sleep requirements
Attempt to recoup lost sleep on weekends
Leads to decreased cognition and emotional regulation
What is the most common cause of flaccid paralysis in peds
Guillian-Barre
Peds presents with Areflexia, flaccidity, & symmetrical ascending weakness
Think
Guillian Barre syndrome
Tx for Guillian Barre
Early stages → admit to hospital for observation & respiratory support if needed
Treatment: IVIG; plasma exchange & immunosuppressive drugs if IVIG fails or rapidly progressive disease
A pt presents with constipation and poor suck reflex that progresses to hypotonia and weakness with a decreased gag reflex
Think
Botulism 2/2 Canned foods or Honey
Tx: IVIG and RR support
X linked muscular dystrophy MC in boys that presents with Toe walking, wide broad lordonic stance
Think
Dúchennes
What is Gower sign
Clumsiness and easy fatigability, then weakness of muscles
Seen in dúchennes
Prognosis for Duchenne Muscual dystrophy
Arm weakness by age 6
Wheelchair bound by age 12
What enzyme is elevated in muscular dystrophies
CK
Cafe au lait spots are assoc with
Neurofibromas type 1
Dx criteria for Neurofibromatosis type 1
Need 2 or more of following:
-Café au lait spots (≥6)
- >5 mm (prepubescent child)
-Axillary or inguinal region freckling
-2+ Lisch nodules (iris hamartoma)
-2+ neurofibromas
-Optic gliomas
-Relative (1st degree) with NF by criteria
-Osseous lesions
(sphenoid dysplasia or long bone abnormalities)
Need cranial imaging to r/o neoplasms
Complications of Neurofibromatosis type 1
Learning disabilities, scoliosis, seizures, cerebrovascular abnormalities
Other tumors possible: optic nerve gliomas, brain & spinal cord astrocytomas, malignant peripheral nerve tumors, sarcomas
What is the finding that really tells you that its Neurofibromatosis type II
NF2 gene codes for merlin
Cataracts common
NO axillary freckling, Occasionally Café-au-lait
Mutation of chromosome 9
-TSC1 gene makes hamartin
Mutation of chromosome 21
-TSC2 gene makes tuberin
Think
Tuberous Sclerosis
Hamartomas =
Tuberous sclerosis
An pt presents with facial angiofibromas (adenoma sebaceum), intellectual delay, epilepsy
Think
Tuberous Sclerosis
3 types of skin manifestations of Tuberous Sclerosis
Adenoma sebaceum
(facial angiofibromas): small, red nodules over nose & cheeks confused w/ acne
Ash leaf spots: hypomelanotic areas of skin
Shagreen patches: elevated, rough plaques of lumbosacral/gluteal areas
What is the most common cause of adult death 2/2 tuberous sclerosis
Renal manifestations:
->Renal angiomyolipomas: can have malignant transformation
most common cause of death in adults)
Sturgeon Weber syndrome
Sporadic (not inherited)
Abnormal leptomeningeal blood vessels (angiomas) overlying cerebral cortex associated with:
->Ipsilateral facial port-wine stain (nevus flammeus) involving ophthalmic division of trigeminal nerve (forehead & upper eyelid)
+Glaucoma
+SZRs (MC presentation)
What is the tx for unilateral disease w/ difficult to control seizures
hemispherectomy
What is the foramen ovale
Opening between right & left atrium allowing for a right to left shunt
Allows oxygenated blood to bypass fetal lungs
What is the ductus arteriosus
Opening between the pulmonary artery & aorta allowing for a right to left shunt
Prostaglandin E occasionally used to maintain patency when a cyanotic lesion also present
What is the ductus venosus
Opening between the umbilical vein & inferior vena cava allowing oxygenated blood from the placenta to bypass the liver & flow to the heart
A baby that sweats during feeding
Think
Congenital Heart Defects
Fixed s2 split=
Atrial Septal Defect
Is an S4 sound ever NML
S4 = Poor diastolic function
Never normal!
Decreased ventricular compliance
Clicks in the heart =
valvular abnormality or dilated great vessel
What is the most common symptomatic arrhythmia
SVT is most common symptomatic arrhythmia
Responds to vagal maneuvers or adenosine
1st test to order in the W/u for murmurs or HDz
CXR r/o pulm involvement
What findings in chest pain lead to a cardio referal
Hx of chest pain w/ syncope, exertion, palpitations or acute onset w/ fever → cardiac etiology
DDX for kids with complete hr block
Congenital Complete Heart Block
->Associated w/ maternal collagen vascular disease (i.e., systemic lupus erythematous or Sjogren syndrome) or congenital heart disease
Acquired complete heart block:
_>most often occurs secondary to cardiac surgery
Other causes: infection, inflammation, or drugs
MC congenital heart defect
VSD
<3 mm = asymptomatic
3-5 mm = moderate symptoms
>5 mm = CHF & FTT
Tx options for VSD
~One third close spontaneously
Initial treatment
(moderate-large VSDs):
diuretics (± digoxin)
& afterload reduction
Poor growth, failure to thrive (FTT) or pulmonary HTN despite treatment → closure required (usually surgical)
When do you do Tx correction for ASD
If significant shunt still present around age 3 → closure recommended:
Many closed via catheter closure devices
Otherwise, surgical closure
A pt that presents with “Bounding” pulses w/ widened pulse pressure
Continuous machine-like murmur at LUSB
Think
PDA
Tx for PDA
Indomethacin IV
->Most effective in premature infants
May cause transient renal insufficiency
Cardiac catheter closure:
Coil embolization or PDA closure device
Click that is at the LUSB that radiates to the back
Think
Pulm Stenosis
Click that happens at the RUSB and radiates to the neck think
AS
Tx for stenotic valves
Balloon valvuloplasty (more effective in pulmonary than aortic stenosis)
Surgery:
If ballon valvuloplasty fails OR subvalvular pulmonic stenosis
Pt presents with Femoral pulses weaker & more delayed than R radial pulse
BP in legs < BP in arms
Think
Coart
Grade I-II systolic murmur at LUSB w/ radiation to L upper back, next to scapula
Think
Coart
Rib notching in older peds think
Coart
Components of tetralogy
Overriding aorta (into the RV)
Pulmonary stenosis
VSD
RVH
Tx for Tetralogy
PGE is indicated if infants are cyanotic at birth
Surgical repair
(Tet spells are an indication to proceed)
SBE Prophylaxis (until 6 mos s/p surgery or indefinitely due to residual VSD)
Boot shaped heart
Think
Tetralogy
Egg on a string on CXR =
Transpo
Any cyanotic baby should get..
PGE 1 to maintain a PDA
Define Ebsteins anomaly
Tricuspid valve leaflet(s) malformed & partly attached to RV endocardium & fibrous tricuspid valve annulus
Leads to an enlarged RA and Abn Tricuspid valve / regurg
RA enlargement → ↑ RA pressures causing R → L shunt through foramen ovale (leading to PFO) → cyanosis (deoxygenated blood by-passing lungs)
Globular cardiomegaly, decreased pulmonary vascular markings, box-shaped heart
Think
Ebsteins anomaly
Rhematic HDz effects what valves
Mitral, aortic, or pulmonic insufficiency or stenosis
Lab test for rheumatic fever
antistreptolysin O titer (streptococcal antibody test)
Major Cx for Rhematic HDz
Polyarthritis Carditits Chorea (syndham chorea) Erythema Marginatum Sub Q nodules
MGMT for Rheumatic HDz
Consult Cardio
-> PCN G/ Amoxicillin
(Allergic; Erythro)
- > ASA/ NSAIDs
- > Steroids (severe)
Long term: PCN prophylaxis q 28 days
(If VHDz then for life)
MC Cause of Pericarditis
VIRAL VIRAL VIRAL
MC Causes of infectious endocarditis
Principal cause in children w/ congenital defect w/out prior surgery: streptococcus Viridans
Important causes in children s/p cardiac surgery & children w/ prosthetic cardiac & endovascular materials:
Staphylococcus aureus & coagulase-negative staphylococci
Risk fxs for infective endocarditis
Prosethic valves
Dental procedures
REcent surgeries
Or Neonate central lines
Fever, malaise, & weight loss
+ Tachycardia & new/changed heart murmur
Think
Infective endocarditis
Roths spots
Splinter hemorrhages
Oslers nodes
And Janeway lesions
Think
Infective endocarditis
Tx for endocarditis
Get blood Cx
Infective carditis by viridans streptococci → monotherapy penicillin G for 4 wks
If medical treatment unsuccessful → surgery
Prophylaxis Rx for prevention of Endocarditis
Recommended regimen: Oral amoxicillin 50mg/kg (maximum dose: 2g) 30-60 mins before procedure
Alternative regimen: β-lactam allergy → clindamycin or azithromycin
Primary vs Secondary HTN
Primary (Essential) HTN:
Most common cause of HTN in adolescents
More likely in obese children
Secondary HTN:
Younger age
More severely elevated BP
Renal disease (most common cause in children)
Mc cause of 2nd HTN in peds
Renal disease (most common cause in children)
MGMT for Ped HTN
2 BP averaged together
Pre-HTN (90-95)—repeat BP in 6 months and education
Stage 1 HTN (95-99)—repeat BP in 1-2 weeks (average BP over all 3 visits) + education
Stage 2 HTN (>99)—evaluate and/or + refer for treatment within 1 week + education
When to evaluate for 2ndary causes of HTN
Especially if BMI <85th percentile
BP >140/100
1st line tx for peds HTN
Most common 1st line:
Calcium channel blockers
Angiotensin-converting enzyme inhibitors (ACEI)
Other 1st line:
Angiotensin receptor blockers (ARB)
β-blockers
Diuretics
When do we screen for Hgb/Hct
At 12 months
DDX MCV <80
Iron deficiency, Thalassemias, Lead
Anemia DDx for MCV 80-100
Associated w/systemic illness that impairs adequate marrow synthesis of RBCs
Anemia DDX with MCV above 100
Vitamin B12 & folic acid deficiencies
Ex: Hypothyroid, Trisomy 21, Newborns
What dictates transfusion threshold for anemia
PRBC transfusion dictated by cardiovascular & functional impairment more than hemoglobin level
Most common S/s for Anemias
Hemolysis:
Pallor, jaundice (most common signs) & splenomegaly
Coagulopathy or platelet abnormalities:
Petechiae, purpura, & deeper bleeding (i.e., generalized hemorrhage)
A peds pt with dementia, ataxia, or neuropathy
What type of anemia?
Vit b12 def.
DDX for elevated RDW
Suspect increased RBC destruction
- > G6PD deficiency
- > Sickle cell
- > Spherocytosis
Anemia with a NML RDW
Think
RDW normal? Suspect decreased RBC production
- > Transient erythroblastopenia of ->childhood
- > Anemia of chronic disease
- > Bone marrow failure (pancytopenia)
High Retic # + bleeding
Look at
GI/GU cause
High Retic # + hemolysis
Think
Immune vs non immune cause of anemia
DDX for Microcytic anemia
FLATS
(Fe) Iron Lead Anemia of Chronic Dz Thalassemia Sideroblastic Anemia
MC nutritional deisorder in the world
IRON def.
2/2:
Cow’s milk: 9-24mo (inadequate iron intake) Blood loss Menses Intestinal Epistaxis Undernutrition (Worldwide)
CBC microcytic anemia
High RDW
Reduced RBC
Think
Iron Def,.
How long do we treat for Iron Def ANEMIA
3 months ! To replenish iron stores
DDX for Macrocytic anemia
Common DDX (“FLAHB”) Folate deficiency/Fanconi Liver disease Alcohol/Aplastic anemia Hypothyroidism B12 deficiency
MC Cause Aplastic anemia
Often idiopathic
Dx with Bone Bx
Tx with Stem cells
↑ Bilirubin
↑ Free (aka serum) hemoglobin
↓ Haptoglobin
Schistocytes
Think
Hemolysis
1 alpha gene mutation =
1 gene mutation: “Silent” carrier thalassemia
Completely asymptomatic → normal CBC
Only detectable through genetic studies
2 alpha chain defects=
2 gene mutations: α-Thalassemia trait
Mild microcytic anemia
Detectable through mildly decreased Hgb & Hct
3 gene thalassemia defects
3 gene mutations: “Hgb H disease”
Microcytic anemia & mild hemolysis
Not transfusion-dependent
4 gene thalassemia defects
4 gene mutations:
Bart Hgb/Hydrops fetalis (γ4)
Severe anemia, intrauterine anasarca from congestive heart failure, death in utero or at birth
1 beta mutation =
1 gene mutation:
Thalassemia minor (heterozygous)
Mild microcytic anemia
2 beta gene mutations =
2 gene mutations:
Thalassemia intermedia
(compound heterozygous)
Moderate hemolysis, splenomegaly, moderately severe anemia
(not transfusion dependent)
High Hgb A2 (α2δ2) & Hgb F (α2γ2)
2 beta gene deletion=
2 gene deletion: Homozygous
Severe hemolysis, ineffective erythropoiesis, transfusion dependent, hepatosplenomegaly
Frequent anemic crises requiring regular transfusions by 2 mos
High Hgb F (α2γ2)
→ due to lack of nml Hgb A (α2β2)
Iron overload (hemochromatosis) common
Fever in a kid with sickle cell =
Medical emergency!
Onset of spleen disfunction in peds with Sickle Cell
As early as 6mo abnormal immune function due to splenic dysfunction
By 5yr most with functional asplenia
When would be the most likely agent in a sickle cell pt with osteomyelitis
Salmonella or Staph aureus
If a pt presents in an Aplastic crisis
Think
Parvovirus B19
What is often the first manifestation of plenipotentiary failure in kids
Dactylitis-> often first manifestation of pain infants/children
Symmetic or unilateral swelling of hands and/or feet
A pt presents with Symmetic or unilateral swelling of hands and/or feet
Often the first S/s of Sickle Cell in peds
Stroke in a pediatric pt
Think
Sickle Cell Sequestration
What is the most common vascular event of sickle cell
Pain crisis
-Last 2-7 days
-Pain usually localizes to arm/leg long bones
(Femur → possible femoral head avascular necrosis)
Treatment: fluids, analgesics
(narcotics & NSAIDs), O2
What is Acute Chest Syndrome in Sickle Cell Pts
Vasoocclusive crisis w/in lungs
On CXR= new infiltrate
Associated w/ infection & infarction
1st chest pain → w/in few hrs, cough, increasing RR & HR, hypoxia, & progressive respiratory distress
PE: decreased breath sounds & dullness to chest percussion
Treatment: O2, fluids, analgesics, antibiotics, bronchodilators, incentive spirometry, & RBC transfusion
Sickle cell pts with a new infiltrate on CXR
Think
Acute Chest Syndrome
S/s 1st chest pain → w/in few hrs, cough, increasing RR & HR, hypoxia, & progressive respiratory distress
PE: decreased breath sounds & dullness to chest percussion
Tx for Acute Chest Syndrome
O2 fluids analgesics antibiotics bronchodilator incentive spirometry & RBC transfusion
MGMT for sickle Cell
Hydroxyurea (start at 9mo old)
-Increases production of Hgb F (α2γ2)
(Which normally decreases at 9mo)
-Decreases number & severity of vasoocculusive events
Or
Hematopoietic stem cell transplantation
-If HLA matched sibling donor used → curative
Sickle Cell Preventative MGMT approach
Daily prophylactic oral penicillin begun at diagnosis
Vaccinations (against pneumococcus, H. influenzae type b, hepatitis B virus, & influenza virus)
Folate supplementation
Heme/Onc referral
Heinz bodies=
G6PD deficiency
What are the oxidizing agents that G6PD pts should avoid
Fava beans sulfa drugs ASA primaquine nitrofurantoin
Exposure to mothballs (naphthalene)
Also Serious infection can precipitate hemolysis
Define Thrombocytopenia
Thrombocytopenia: platelet counts <150,000/mm3
Platelet counts >80,000 /mm3
→ no bleeding risk unless surgery or major trauma
Platelet counts <20,000/mm3
→ risk for spontaneous bleeding
Difference between DVT and Arterial Clots in pts with hemostatic Dz
Deep venous thrombi
-Warm, swollen (distended), tender, PURPLISH discolored extremities or organs
Arterial clots
- Acute, painful, PALE, & poorly perfused extremities
- Internal organ involvement: signs & symptoms of infarction
MC childhood bleeding D/o
ITP
Young children → 1-4 wks after viral infection w/ abrupt onset of petechiae, purpura, & epistaxis
ITP tx for pts with #>30,000
monitor; unlikely to treat
~80% resolve w/in 6 mos
Treatment does not affect long-term outcome but does increase platelets in short-term
ITP tx for pts with #<10,000
-IVIG (intravenous immunoglobulin)
1 g/kg/d x1-2 ds
-Prednisone 2-4 mg/kg/d x2 wks
Splenectomy only for life-threatening bleeding
Chronic ITP: lasts ≥6 months
- Rule out secondary causes (SLE, HIV)
- Splenectomy for definitive treatment (70-80% remission)
Fx VIII deficiency
Hemophilia A
Fx IX deficiency
Hemophilia B
A crawling peds pt presents with Bleeding with minor trauma, spontaneous bleeding, hemarthrosis (bleeding into joints/muscle)
Think
hemophilia A or B
W/u for Hemophilia
PTT prolonged
PT and Bleeding Time usually normal
PTT corrects to normal when sample mixed with normal serum
Does Desmopressin help Hemophilia A or B ?
Mild or moderate hemophilia A only (no effect on factor IX)
Increases factor VIII & vWF production
What is the most common CONGENITAL bleeding d/o
VWF dz
Function of vWF
Bridge between platelets & subendothelial collagen
Binds & protects factor VIII from rapid clearance from circulation
Type 1, 2 , and 3 vWF Dz
Type 1: Decreased production of vWF
Autosomal dominant & most common
Type 2: Normal production but defective vWF (dysproteinemia)
Type 3: No production
Rare
Lab for vWF Dz
vWF quantity is measured and then function is measured with ristocetin*
Tx for vWF Dz
Desmopressin (DDAVP) effective for type 1 & 2
vWF concentrate available for type 3
Define Henoch Schonlein Purpura (HSP)
Vasculitis of unknown etiology
Most common childhood systemic vasculitis
Ages 3-15 yrs (most before age 6)
More frequent in winter
50% of cases follow URI’s
More frequent during winter months
Characterized by inflammation of SMALL blood vessels w/:
- Leukocytic infiltration of tissue
- Hemorrhage
- Ischemia
- IgA immune complex deposition →
Child presents with palpable purpura, arthralgia, arthritis, and abdominal pain
NO FEVER
Think
HSP
Dx criteria for HSP
Palpable purpura
Bowel Angina
Dx Bx -> IgA in the vessel wall
And being <20
What is the post complication of HSP
Rash resolves in a year
Increased risk of GI involvement → temporary abnormal peristalsis → intussusception risk!!
May be followed by complete obstruction or infarction w/ bowel perforation
Evaluate for intussusception if recent HSP history w/ acute abdominal pain, obstipation, or diarrhea
Tx for HSP presentation
Supportive ->Follow renal function NSAIDS for arthritis Steroids for GI disease & nephritis → controversial If severe, hospitalize
Vasculitis of unknown etiology
Small to medium-sized arterial inflammation w/ aneurysm formation
Think
Kawasakis
Greatest risk complication of Kawasaki’s
Hematoma around the heart
/ Coronary artery aneurysm
Child with a fever for 5 days!!
Think
Kawasakis
High fever x 5 days + strawberry tongue/ mucosal changes and cervical lymphadenopathy
Think
Kawasakis
Kawaksi treatment should be followed until…
until ESR normalizes
Pediatric presents with morbilliform rash, conjunctivitis and red chapped lips
Think
Kawasakis
Cx to Dx Kawaskis
Fever x 5 days Bilateral conjunctivitis Mucus involvement Desquamation Polymorphous rash on the trunk Cervical Lymphadenopathy
Tx approach to Kawasakis
echocardiogram at diagnosis, 2-3 wks in & again at 6-8 wks
Treatment
IVIG (mainstay of therapy)
Goal: coronary aneurysm prevention
+Aspirin
-> High dose first then reduce dose
Risk of Reye syndrome low & acceptable because other NSAIDs are not as effective
Define Tumor Lysis Syndrome
Seen in Cancer emergencies (2/2 chemo)
Rapid cellular lysis leads to potassium, phosphate, & uric acid released into circulation
Manifests as arrhythmias, cardiac arrest, gout, uric acid nephropathy
Common in: leukemia & lymphoma treatment
Usual infections seen in cancer pts
Central catheter or port infections
Atypical pneumonia
(Pneumocystis jiroveci)
Aspergillosis
Human herpes virus infections
(severe)
Cryptococcal meningitis
Fever in a cancer pt =
Emergency!!! ADMIT
Treat early & aggressively w/ broad spectrum antibiotics
Immature blast cells
Think
Leukemia
W/u for myeloproliferative d/o with pts with trisomy 21
Transient myeloproliferative disorder (unique to patients with Trisomy 21)
CBC: Elevated WBC w/ peripheral blasts, anemia, thrombocytopenia
Bone marrow aspirate: very minor marrow infiltration (key to differentiating from leukemia)
Suggestive agent of Lymphoma
Unknown etiology, but evidence suggests Epstein-Barr virus (EBV) may play a causal role
Bimodal distribution of Hodgkin’s
Peaks 15-35yo and again age >50
Non Hodgkin lymphoma
Males > females
Almost always diffuse, highly malignant, & little differentiation
2 types of B cell Non Hodgkin’s
B cell (Burkitt lymphoma/small noncleaved cell) Two forms: -sporadic form (North America) -endemic form (Africa; strong EBV association)
A child presents with Painless, nontender, firm,rubbery cervical & supraclavicular adenopathy
Think
Hodgkin’s
Reed Sternberg Cells =
Hodgkin’s
MC type of Brian tumor
Astrocytomas: Most common Usually found in posterior fossa Often low grade Usually good prognosis
Should you do LP prior to CT/ MRI?
Never perform an LP prior to neuroimaging if tumor is a concern—herniation risk
What is the Most common childhood solid neoplasm outside CNS
Neuroblastoma
At age 22 months
Derived from neural crest cells
What is paraneoplastic syndrome in pts with neuroblastoma
Profuse sweating
Secretory diarrhea
Opsoclonus/myoclonus (dancing eyes & dancing feet)
90% of pts with neuroblastomas will have what lab finding
90% will have positive urine catecholamines
(vanillylmandelic acid; homovanillic acid): eval with urine catecholamines test
MC primary malignant renal tumor
Nephroblastoma= Wilms Tumor
What is WAGR
WAGR (Wilms tumor, Aniridia, Genitourinary malformation, Range of developmental delays) syndrome → germline deletion at chromosome 11p
What is Beckwith Wiedemann Syndrome
Macroglossia,
Umbilical hernia
And omphalocele
With Nephroblastoma increased risk
Abdominal mass + painless hematuria
Think
Nephroblastoma
Most common soft tissue sarcoma
Rhabdomyosarcoma
Most common primary malignant bone tumor in chlidren
Osteosarcoma
Most common bone sarcoma in children less than 10
Ewing sarcoma
What pts are at an increased risk for soft tissue sarcomas
Pts with Li-fraumeni and Neurofibromatosis
Pts with Heriditary retinoblastoma are at an increased risk of what cancer
increased osteosarcoma risk
Small round blue cell tumors on microscopy
Think
Rhabdomyosarcoma
MC;
GU (24%)
Head & neck (25%)
Extremities (19%)
Orbit (9%)
ALSO Ewing Sarcoma
A peds with pain and mass at the epiphysis with osteoid substance in the joint
Think
Osteosarcoma
Starburst pattern on XR
Think
Osteosarcoma
Onio skin/ Moth eaten appearance on XR
Think
Ewing Sarcoma
How can you DDx Ewing on Anaylsis
Immunohistochemical & cytogenic analysis differentiates rhabdo & Ewing
Ewing: t(11;22)
Tx option for Rhabdomyosarcoma
Surgical resection w/ POST OP chemo & radiation depending on stage & site
Tx for osteosarcoma
Neoadjuvant chemotherapy followed by surgery & adjuvant chemotherapy
Tx for Ewing sarcoma
Ewing radiation sensitive
4 distinct phases of DM type I
- Preclinical B-cell autoimmunity with progressive defect of insulin secretion
- Onset clinical diabetes
- Transient remission honeymoon period
- Established diabetes during which there may occur acute and/or chronic complications
Tx for T1DM vs T2DM
T1: life long insulin
T2: metformin and life style
How do we eval the honeymoon period for DM
Measure residual function with c-peptide
Glucose > 300, pH <7.3,
bicarbonate <15
Think
DKA
What intervention should you NOT do in pts with DKA
DO NOT DO NOT DO NOT Bolus them!!
Rapid decreases in serum glucose levels >100 mg/dL/hr
Leads to
Cerebral edema
T1 DM Goals in peds
A1C
:Age <6: HgbA1c = 7.5-8.5%
Age 6-13: HgbA1c = <8%
Age 13-18: HgbA1c = <7.5%
Blood Glucose:
Children <5 yrs: 80-200 mg/dL
School-age children (5-15): 80-150 mg/dL
Adolescents (age 16+): 70-120 mg/dL
Definitve Dx for Hashimotos
TPO abs
An infant that presents with poor feeding, vomiting, lethargy, and convulsions with no response to glucose WITHOUT A FEVER
Think
Inborn error of metabolism
PKU
Define PKU
Defect in hydroxylation of phenylalanine to form tyrosine
Primarily affects the brain
Autosomal Recessive
Appears completely NML that then develops into profound developmental delays
How to check for PKU
Positive Newborn Screen ->check quantitative plasma amino acid analysis (positive: phenylalanine >360µM or 6mg/dL)
MGMT for PKU
Low Phenylalanine diet to maintain plasma phenylalanine levels between 120-360 mMol/L throughout life (frequent labs)
Define Galactosemia
Autosomal Recessive
Galactose-1-phosphate uridyltransferase deficiency
When a neonate is fed milk -> -Liver failure (hyperbilirubinemia, disorders of coagulation, hypoglycemia) -Renal tubular dysfunction (acidosis, glycosuria, aminoaciduria) -Cataracts
Need rapid neonatal screening test turnaround time ->affected infants may die in 1st week of life
MGMT for galatosemia
Tx: eliminate dietary galactose!
Learning disorders may persist despite dietary compliance
Premature ovarian failure usually develop despite treatment
What infections are pts with galactosemia at risk for
Increased risk for severe neonatal Escherichia coli
S/s of Addisons
Hyperpigmentation (tan color to skin) Salt craving Postural hypotension Fasting hypoglycemia Anorexia Weakness Shock can occur during severe illness
Lab findings for Addisons
Cortisol
-Subnormal at baseline and also low with ACTH-stimulation
Other labs: BMP—hyponatremia, hyperkalemia, elevated plasma renin (indicate mineralocorticoid deficiency)
MGMT for Addisons
Hydrocortisone (10-15 mg/m2/24 hours)
- Baseline daily dosing
- Stress dosing = three times daily dosing or IM hydrocortisone
- Dose titrated to allow a normal growth rate
Mineralocorticoid deficiency treated with fludrocortisone
-Monitor with plasma renin, Na+, K+ levels
A male pt that presents with normal genitalia
Salt loser: present within 2 weeks in adrenal crisis
Non salt loser: present at 6mo with androgen side effects on exam but NO testicular enlargement
Think
CAH
Refer to Endo
Cushing Syndrome
Progressive central or generalized obesity Marked failure of longitudinal growth Hirsutism Weakness Nuchal fat pad Acne Striae Hypertension Hyperpigmentation (if ACTH elevated)
Neonatal Surgical Rep Flags
Maternal Polyhydramnios
-Various causes—surgical concern if inability of fetus to swallow/digest amniotic fluid causing “back-up” of fluid
Delayed meconium passage
Abdominal distention
-Obstruction?
Perinatal infant vomiting
- Bilious or non-bilious?
- Can indicate location of pathology
NML development of the tracheal and esophageal tissue
Tracheal and esophageal tissue develop in close proximity at 4-6 weeks gestation
A fistula represents dysgenesis (abnormal development) of this tissue
Peds with a single umbilical artery are at an increased risk of…
Tracheo-esophageal fistula
What does VACTERL mean
VACTERL association:
- Vertebral anomalies (70%)
- Anal atresia (imperforate anus) (50%)
- Cardiac anomalies (30%)
- TEF (transesophageal fistula) (70%)
- Renal anomalies (50%)
- Limb anomalies (polydactyly, forearm defects, absent thumbs, syndactyly) (70%)
Dx for Fistulas of the esophagus
Gastrografin swallow
Methylene blue challenge
What is the number 1 cause of GI obstruction before age 3 months old
Pyloric Stenosis
2-6 week-old
Post-prandial, progressive nonbilious* “PROJECTILE” vomiting
Initially ravenously hungry, then lethargic as malnutrition/dehydration progresses
Think
Pyloric stenosis
Olive shaped mass in the abdomen
Think
Pyloric stenosis
Pyloric stenosis on a Lab
Hypochloremic, hypokalemic metabolic alkalosis
String Sign on upper barium study
Pyloric stenosis
Prognosis of umbilical hernias
Typically 1-5 cm in size
> 2 cm = less likely to close on their own
SRGRY criteria for Umbilical hernias
Hernia persists past 5 years of age
Painful
Becomes strangulated
Increasing in size after age of 1-2 yrs
Pt presents with BILIOUS vomiting
Think
Intestinal Malrotation & Volvulus
Corkscrew effect on upper GI studies thing
Intestinal Malrotation & Volvulus
MC congenital d/o assoc with Intestinal atresia
Trisomy 21 (30%) Malrotation (25%) Annular pancreas (20%) Meconium ileus w/ cystic fibrosis Check for CF if infant has atresia
DOuble bubble sign on CXR
Think
intestinal atresia
Define Gastrochisis
Definition “split or open stomach”
-Misnomer as actually abdominal wall that is split
Linear abdominal wall defect
- Lateral to the median plane of the anterior abdominal wall
- More often on right side
Does not involve umbilicus
MGMT for Gastrochisis
Surgical correction requires → return of normal bowel function often slow
Prolonged parenteral nutrition (TPN) often required
DDx of ompahlocele and Gastrochisis
Omph: bowel remains in the umbilical cord & covered by peritoneum & amniotic membranes
Rule of 2s of meckels
The rule of 2’s: 2% of the population 2 ectopic mucosae -Gastric/Pancreatic Presents by 2 years Within 2 feet of the cecum 2 inches long
MASSIVE painless GI bleeding
Think
Meckles Diverticulum
Any evidence on imoperforate anus
Needs what follow up image
All imperforate anus require MRI of the lumbosacral spinal cord
high incidence of tethered spinal cord
When should you do a DRE in pediatrics
HIRSHPRUNGS
MGMT for necrotizing enterocoliitis
Stop enteral feedings → start Total Parenteral Nutrition (TPN), GI decompression w/ NG, Fluid & electrolyte replacement
Broad spectrum abx
Surgery: laparotomy w/ excision of affected bowel
Intussusception is associated with what viral infection
Associated w/ rotavirus infection & old rotavirus immunization*
Pt presents with “Currant jelly” stools: mixture of mucus, sloughed mucosa, & blood
Think
Intussusception
Donut sign on US
Think
Intussusception
MGMT for intussusception
Pneumatic or contrast (i.e., barium) enema under fluoroscopy
Diagnostic & therapeutic
Pneumatic preferred: NO barium peritonitis if bowel perforates & allows for subsequent radiologic studies
Most common surgical emergency in children
Appendicitis
ALVARADOS Score
Score 1 point for each:
- Migration of pain to RLQ
- Anorexia
- Nausea/vomiting
- Rebound pain
- Fever (at least 37.3° C)
- WBC shift >75% neutrophils
Score 2 points for each:
- RLQ tenderness
- Leukocytosis >10,000/µL
Score < 4 Unlikely
Score > 7 Likely
Primary cause of arrest in peds
Respiratory arrest, not cardiac, is the primary cause of cardiopulmonary arrest in children (contrast to adults)
define dissociative shock
O2 not appropriately bound or released from hemoglobin
Causes:
Carbon monoxide poisoning
Methemoglobinemia
Clinical presentation: Tachycardia Tachypnea Alterations in mental status Cardiovascular collapse
Cervical spine radiographs are not sufficient to rule out a spinal cord injury → due to immature vertebral column:
Allow stretching of the cord or nerve roots w/ no radiologic abnormality (Spinal Cord Injury Without Radiologic Abnormality [SCIWORA])
If suspect SCIWORA=
Get MRI
MGMT of BRUE
Tests for RSV & pertussis (for respiratory infections)
Barium swallow or pH probe study (for GERD)
High risk patients: Admit to hospital for12-24 hr cardiorespiratory monitoring
Full workup
Antidote for iron poisoning
Deferoxamine
Treatment for Lead poisoning
Edetate calcium disodium (EDTA)
Dimercaprol (BAL = British Anti-Lewisite)
Prepared in peanut oil → do not use in patients w/ peanut allergy
Succimer (DMSA = dimercaptosuccinic acid ) → few toxic effect
S/s of lead poisoning
Insidious onset: weakness, lethargy, ataxia, growth delays, school problems Alopecia Gum lines Seizures Coma (if severe) Hypochromic microcytic anemia Basophilic stippling
S/s of methanol poisoning
optic papillitis & retinal edema
MGMT for Methanol/ Ethylene Poisoning
Treatment:
10% ethanol & D5W
Fomepizole (Antizol)
- Alcohol dehydrogenase inhibitor
- 8,000 times more affinity than ethanol*
Supplementation
- Thiamine & B6 (for ethylene glycol)
- Folic acid (for methanol)
