Peds: Block 3 Flashcards
Define Hypochromic, Microcytic anemia
What diseases does this include?
Dec Hgb production
Chronic Dz
Lead
Iron deficiency
Thalassemia
Define Normocytic Anemia
What issues can cause macrocytic anemia?
Systemic illnesses impairing marrow RBC synthesis
B12/Folic acid deficiency*
Trisomy 21
Hypothyroid
Newborn
What are Sxs of acute anemia and which one is especially seen in infants?
What Sxs may be seen in Chronic Anemia
Murmur Exercise Intolerance Tachy HA Excessive sleeping*
Fatigue Irritable Poor feeding Syncope
Minimal tachy/flow murmurs
What findings dictate if a PT needs a transfusion or not?
What are the MC presenting S/Sxs of anemia
CV and functional impairment, not Hgb level
Pallor Jaundice Splenomegaly
PT w/ petechiae, purpura, deeper bleeds indicate a generalized ?
Infiltrative d/o or systemic illness induced anemia may present w/ ? PE findings?
Hemorrhage
Hepatosplenomegaly
Lymphadenopathy
What part of the CBC help differentiate the cause of the issue?
How are anemia categorized?
What does the RDW do?
WBC/Platelet count
RDW and Peripheral smear
Measures variation in RBC sizes/indicates abnormal sizes
Dec RBC production, norm RDW can be caused by ?
What are the etiologies of Macrocytic, Hypo Micro, Target cell and Schizocyte cells?
Marrow failure (pancytopenia)
Anemia, chronic dz (microcytic)
Transient erythroblastopenia
Macro: B12/Folic deficiency
Hypo Micro: Fe deficiency
Target: HbCC Dz
Schizocytes: hemolytic-uremic syndrome
Inc RBC destruction, high RDW can be due to ?
Peripheral smears asses ? parts of blood work?
G6PD deficiency
Sickle cell
Spherocytosis
RBC, WBC, Platelet morphology, Fe and Lead levels
Determines if anemia is due to erythroid line or marrow elements
What does it mean if a reticulocyte count is elevated, normal or dec?
This measurement can indicate severity of ?
E: normal response to blood loss
N: dec/ineffective production
D: body hasn’t reacted to blood loss yet, Ab mediated destruction or intrinsic marrow dz
Hemolysis
What lab results would be seen in Fe deficient anemia as a presumptive dx?
Breast fed babies need to receive Fe supplementation starting at ? age?
CBC w/ microcytic anemia
High RDW, Red RBC
Norm WBC, Platelet
4mon
Start Fe foods @ 6mon
How long are Fe Deficient anemias Tx?
48-72hrs: reticulocytes inc and peak in 5-7 days
4-30 days: inc in Hb, MCV, ferritin
1-3mon: replenished stores
Continue Tx for 2-3mon after values normalize
How is normocytic anemia suspected dx made and how is it confirmed?
What are the common DDx for Macrocytic Anemias
Hx to Dx of dec production
Peripheral smear- dx for destruction
Hypo BAFFLE Hypothyroid B12 deficiency Aplastic anemia Folate deficiency Fanconi Liver dz ETOH
What labs are ordered to work up macrocytic anemia
What is done for Tx and for how long?
B12 LFTs Folate TSH/Thyroid
B12/Folate replacement
CBC improvement <1wk
Tx liver/thyroid issue
Aplastic anemia is most often ?
How is it Dx?
What is the criteria for a severe Dz Dx?
How is it Tx?
Idiopathic
Marrow biopsy
Reticulocyte count <50K
Platelets <20K
Neutrophil <500
Hematopoietic stem cell transplantation
ImmSupp therapy
Define Fanconi Anemia
How does it present
How is Fanconi anemia Tx?
Autosomal recessive form of aplastic anemia causing abnormal DNA repair, effects all cell lines
High MCV and Hgb F
Cafe au Lait spots Horseshoe/one kidney Absent thumbs Microcephaly Pancytopenia
Stem cell transplant
Androgenic therapy
CCS
What are the 6 types of hemolytic anemias
I RIMES
Intrinsic- thalassemia, sickle
RBC membrane defect- spherocytosis
Isolated site- vasculitis
Mechanical
Extrinsic- immune mediated
Systemic d/o- DIC, malignancy
What will be seen in lab results of hemolysis
Define Schistocytes seen in hemolytic anemia
What are two possible findings seen during this issue
Inc bilirubin/serum Hgb
Dec haptoglobin
Broken RBCs
Hemoglobinuria
HyperKalemia
Define Thalassemia
Normal Hgb is made of ?
Insufficient Hgb production from dec A/B-globulin production
HgA= A2B2
Four A genes= 4 A proteins
Two B genes= 2 B proteins
What are the 4 versions of Alpha Thalassemia
One= silent carrier; normal CBC/ASx; detected w/ genetics
Two= a-Thalassemia trait; microcytic, dec Hgb/Hct
Three= Hgb H Dz, microcytic anemia/mild hemolysis, not transfusion dependent
Four: Bart Hgb/Hydrops fetalis
Define Beta Thalassemia
One: thalassemia minor (heterozygous)- mild macrocytic anemia
Two: thalassemia intermeida (compound heterozygous) moderate hemolysis, splenomegaly, mod/sev anemia (not transfusions dependent)
Define Thalassemia Major
Cooley’s Anemia
2 gene deletion- homozygous
Severe hemolysis, ineffective erythropoiesis, inc Hgb F
Transfusion dependent, frequent crisis/hemachromatosis
Reqs stem cell transplantation
Define Sickle cell anemia
Define Sickle Cell Dz
HbSS homozygous
HbS >50% of Hgb
Heterozygotes w/ one B-globulin includes sickle cell mutation and 2nd: Hbc, Beta-thalassemia, HbD
What do PTs w/ Sickle Cell and a fever need?
By 5yrs they are functionally asplenic and vulnerable to what 3 microbes?
Medical emergency
+6mon- splenic dysfunction
HIB
N Meningitidis
Strep Pneumo
Sickle Cell PTs are at increased risk of infections by ? bacteria?
If PT w/ Sickle presents w/ osteomyelitis, suspect ? microbes?
PT w/ aplastic crisis, suspect ? microbe?
Encapsulated: Strep Pneumo, HiB, N Meningitidis
Salmonella
Staph A
Parvovirus B19
Sickle Cell PTs most often have avascular necrosis in ? location?
Define Dactylitis
What Sx is seen in males w/ sickle cell?
Femoral head
First manifestation of pain in kids, swelling of hands/feet
Priapism w/ stuttering
What regression of mile stones could be seen in Sickle PTs?
Define Pain Crisis in Sickle PTs
Enuresis
MC vasoocclusive event
2-7 days of arm/leg pain
Tx: fluids, pain, O2
What other adverse effects can occur due to Sickle Cell?
Define Acute Chest Syndrome in Sickle PTs
PHTN, Enuresis, Pain, Stroke
Occlusive crisis of lungs
CXR= new infiltrates
Tx: pain fluid transfusion O2 ABX dilators
What meds are used for Sickle Tx?
What meds can be used for prophylaxis
What procedure can be curative for sickle cell PTs?
Hydroxyurea at 9mon old- inc Hgb F production
Dec occlusive events
PO PCN at Dx
Vaccines- HiB, Influenza, HBV
Folate
Hematopoietic stem cell transplant from sibling donor
What unique lab finding is indicative of G6PD?
What is the clinical presentation of G6PD deficiency?
What Hx piece can precipitate a hemloysis presentation?
Heinz Bodies- RBC membrane fragility leads to hemolysis, phagocytosis by macrophages to make Bite Cells
Hemoglobinuria Anemia Jaundice
Serious infection
G6PD PTs that ingest ? compounds lead to issues
How is G6PD Dx?
Fava beans Naphthalene (mothballs) Dapson Quinidine Primaquine ASA Infection Nitrofurantoin Sulfa drugs
Enzyme measurement, low NADPH/G6PD levels
Thrombocytopenia means platelet count below ?
Platelet levels below ? are not an issue unless surgery/major trauma occurs
Levels below ? have PTs at risk for spontaneous bleeds
<150K
> 80K
<20K
How does the most common childhood bleeding d/o present?
What type of d/o is this
ITP, 1-4wks post viral infection
Petechiae epistaxis purpura
IgG/IgM coats platelets causing destruction in spleen
What would be seen in lab results of ITP?
ITP Tx is not needed if platelets counts are above ?
Severe thrombocytopenia
Normal results elsewhere
> 30K, 80% resolve in 6mon
What med is used for ITP Tx?
What is the definitive Tx of Chronic ITP lasting more than 6mon?
IVIG, Prednisone
IV anti-D in Rh pos PTs
Splenectomy
Define Wiskott-Aldrich Syndrome
What are the three characteristics of this dz
X-linked cytoskeletal protein defected lymphocytes/platelets
Thrombocytopenia
Hypogammaglobinemia- defected Ab production due to defected lymphocytes
Eczema
How is Wiskott Syndrome Dx and Tx
What is the difference between Hemophila A and B
Peripheral smear- small platelets
Splenectomy improves thrombocytopenia
A: Factor 8 deficiency
B: Factor 9 deficiency
How is Hemophilia Dx?
What is used for Tx?
What is the most common congenital bleeding D/o
Coagulation studies: prolonged PTT, normal when mixed w/ normal serum
Desmopressin- increases Factor 8, vWF in Hem A
Recombinant Factor 8/9
Von Willebrand dz
What are the function of vWF in the blood?
What are the subtypes of this dz?
Bridges platelets and collagen
and protects Factor 8 from being removed from circulation
1: dec production, autosomal dom is MC
2: normal production, defective vWF (dyspoteinemia)
3: no production, rare
How is vWF measured by the lab during Dx studies?
What is done for Tx?
Ristocetin- ABX that induces vWF to bind to platelets
Desmopressin- Type 1 and 2
vWF concentration-Type 3
Define Henoch-Schonlein Purpura
Half of these presentations follow a ?
MC childhood systemic vasculitis before 6y/o
Inflamed small vessels w/ Leukocyte infiltrate, Hemorrhage, Ischemia
URI
What is a adverse issue from Henoch Purpura
What will be seen on PE?
What findings can be found but are less common?
IgA deposition= glomerulonephritis in adults
Palpable purpura* below waist
Arthralgia- no weight bearing
Abdominal pain- precedes rash
Renal involvement
Edema: Calves Scalp Feet Genitals
Encephalopathy, Pancreatitis, Orchitis
What peds hematological dz needs baseline blood work at Dx
What is the work up for this dz?
HSP
Platelet count- normal
WBC, ESR/CRP- elevated
Creatinine UA BUN
Stool occult
What criteria are needed to Dx HSP?
What education piece needs to happen after Dx?
2 of 4: Palpable purpura Bowel angina Diagnostic biopsy Pediatric age <20y/o
Monophasic x 3-4wks
Rash can last 1yr
Arthritis not permanent
How is HSP Tx
What adverse outcomes are possible?
What is the prognosis of this Dz?
Monitor renal function
NSAIDs
Rash wax/wane x 12mon
Arthritis
Abnormal GI paristalsis
Excellent, rare renal failure
Define Kawasakis
What are the 3 phases
Vasculitis of unknown etiology
Arterial inflammation w/ aneurysm formation
2nd MC childhood vasculitis
Acute: Fever >40*C
Conjunctival erythema
Cracked lips/red tongue
Cervical lymphadenopathy
Subacute: Desquamination, Coronary artery aneurysm
Convalescent: from Sx resolution until ESR normalizes
What is the number 1 Sx of Kawasakis?
This can present similar to Scarlet Fever, how is it different?
Fever +5 days
Pos throat culture
No conjunctiva/lip findings
What f/u study needs to be done in Kawasaki PTs?
How is it Tx
Echo for coronary aneurysm @ 2-3wks and 6-8wks
IVIG*- to prevent aneurysms
ASA
Childhood malignancy are not associated to ? factors
What are warning signs of Ped Cancer?
Tobacco Alcohol Diet Sun
F/Ns/WL
Lymphadenopathy >4wks
Atraumatic limp
HA- AM, vomit, wakes at night
Peds with fevers due to CA can present as what type?
What two labs are best when working up a possible CA case?
Fever of Unknown Origin
CBC w/ diff
Peripheral blood smear
What lab results would be seen in Peds w/ fast growing tumors?
What is the exception to surgical resection of cancer tumors?
Inc LDH and Uric acid
Lymphoma- almost all are chemosensitive
What are the two methods of using chemo for CA Tx?
What is a medical emergency presentation of a PT w/ fever?
Adjuvant- chemo after surgical removal
Neoadjuvant- chemo while tumor is present
Sepsis- presents w/ severe neutropenia
When is Tumor Lysis Syndrome commonly seen
What are the 5 types of oncology emergencies?
Leukemia/lymphoma Tx
Sepsis- neutropenia TLS- K, PO4, uric acid release Anemia/Thrombocytopenia Inc ICP Airway obstruction
What are 5 rare but common infections seen in Peds undergoing CA Tx?
What is the most common and possible only presenting Sx?
Crypto meningitis
HSV
Aspergillosis
P Jiroveci- atypical pneumo
Fever
Peds w/ CA w/ ? two Sxs are admitted
What is the most common childhood cancer?
Fever + neutropenia
Leukemia
What are the 3 sub-types of leukemia seen in Peds?
What would be seen on lab results for Leukemia PTs?
ALL- 2-3y/o males
AML- neonate/adolescence
CML
JMML
Immature blast cells
Anemia
Thrombocytopenia
DDx of leukemia
What leukemia DDx is unique to the Trisomy 21 population that carries a certain risk?
MyPEC
Mycobacteria Pertussis EBV CMV
Transient Myeloproliferative D/o
30% chance of ALL or AML
How is leukemia definitively Dx?
What are the two types of ALL?
Flow cytometry and cytochemical stain patterns
T 12,21: more favorable, MC
T 9,22: less common, poor prognosis
What is the 3rd MC malignancy in children?
When is the MC new Dx?
Lymphoma
15-19y/o
Although not proven, what etiology is linked to lymphoma?
EBV
Hodgkin- MC lymphoma
Non-Hodgkin- male Caucasian that inc w/ age
What are the 3 sub-types of Non-Hogkin lymphoma?
When treating PTs w/ Non-Hodgkin’s what two adverse outcomes need to be monitored for?
B cell: sporadic (US) endemic (Africa)
T cell- lymphoblastic
Large cell- T or B cell origin
Superior mediastinal syndrome
TLS
What are B-Sxs of Hodgkins?
What findings will be present on PE?
Poor prognosis
Fever >38*C x 3 days
Drenched night sweats
>10% weight loss in 6mon of Dx
Cervical/Supraclavicular adenopathy
What lab result finding is indicative of Hodgkin Lymphoma?
What use do bone marrow biopsies have?
Reed Sternberg cells
Dz staging
25% or more are blasts, Dz is classified as acute leukemia
What is the prognosis of Hodgkins?
How is Non-Hodgkins Tx?
90% w/ chemo/rad
Chemo, rarely surgery/radiation used
What is the most common type of brain tumor?
What is the 2nd MC?
Astrocytoma- posterior fossa w/ good prognosis
Medulloblastoma- cerebellar vermis w/ variable prognosis
Never do an LP before ? due to herniation risk
What are 3 unique complications to Tx of Peds brain tumors?
Neuroimaging
Cerebellar mutism syndrome- dec speech/behavior changes
Posterior fossa syndrome- HA, aseptic meningitis
Somnolence syndrome- excessive fatigue/sleep
What is the MC solid neoplasm outside the CNS of kids?
Where are these neoplasms able to grow?
Neuroblastoma- made of neural crest cells from adrenal medulla and SNS formation
Anywhere SNS is found
Half in adrenal glands
Half in paraspinal ganglia
What is the MC presenting Sx of neuroblastoma development?
How else can these present?
Abdomen mass/pain
Horners
Paraneoplastic syndrome: sweating, diarrhea, opsoclonus/myoclonus
What labs are ordered when working a suspected neuroblastoma up?
What is a Wilms Tumor?
VMA/HMA UA for catecholamines
Nephroblastoma- MC primary malignant renal tumor of kids and 2nd MC abdominal tumor of kids
WIlms Tumors are associated w/ ? other anomalies
WIlms causes PT to have inc risk of developing ? syndrome?
Retardation
Aniridia
Genitourinary malformation
Beckwith Wiedmann- Macroglossal, Umbilical herniation, Omphalocele
What is the MC presenting Sx of a nephroblastoma
How is it Tx
This has a good prognosis as long as ? is not present
Abdominal mass w/ pain
Surgery, chemo
Rad if advanced stage
Anaplasia
What is the MC soft tissue sarcoma in kids?
What are the two MC bone tumors?
Rhabdomyosarcoma of skeletal muscle- small round and blue cell tumors
Osteosarcoma
Ewing- unless PT is <10y/o, then is more common
Osteosarcomas have a ? appearance on x-rays
Ewing Sarcomas MC affect what 2 bones?
Osteosarcoma MC affect ? bones?
Star burst
Femur, Pelvis
Distal femur, Proximal tibia, Prox humerus
How do Rhabdo and Ewing differ from Osteosarcoma on microscopy?
What words are used to describe x-rays of Ewing Sarcoma?
R/E: small round blue
O: osteoid substance
Onion skin
Moth eaten
What cytogenic analysis result is Dx of Ewing?
What are the two forms of Rhabdomyosarcoma and the variants
T 11,22= Ewings
Embryonal- young PT w/ head/neck/GU tumor
Alveolar- older PT w/ trunk/extremity tumor (t2,13 or t 1,13)
How are Rhabomyosarcoma, Osteosarcoma and Ewing sarcoma Tx?
Rhabdo- surgery w/ post-op chemo/rad
Osteo: neoadjuvant chemo then surgery and adjuvant chemo
Ewing- radiation sensitive
Define Consciousness and what controls it
Define Arousal and what controls it
Awareness of self and environment, controlled by cerebral cortex
Initiates and maintains consciousness, controlled by reticular activating system from mid pons to mid-brain/thalamus
Define Lethargic
Define Obtunded
Difficulty maintaining aroused state
Dec arousal but responsive to stimuli
Define Stupor
Define Coma
Responds only to pain stimuli
Unresponsive conciousness, dyfunction of hemispheres bilaterally, brainstem or both
What are the MC causes of comas
What is the MC cause of long term morbidity in PTs w/ depressed consciousness
Seizure Head trauma Hypoxia-ischemia Infection Toxin
Hypoxia
Two-thirds of Peds who have syncope have ?
What are the 4 types of injuries that can occur during a TBI
Anoxic seizures
Skull Fx
Intracranial hemorrhage
Cerebral concussion
Cerebral contusion
Define Concussion
What Sxs can be present?
TBI from rapid/short neurologic impairment that self-resolves
Somatic- HA (MC)
Emotion- personality change
Cognitive- slows reaction time, confusion
LoC, Amnesia, Drowsiness
What adverse finding can accompany a concussion and correlate to the severity of the injury?
What is the name of the issue that develops when PTs suffers multiple concussions especially in ? period of time
Amnesia- retrograde (events leading to) or anterograde (no new memory)
Second Impact Syndrome
First 10 days after a concussion
Normally w/ a concussion, there are no ? deficits
What is the preferred imaging?
Focal neurological
Normal CT
If seizures occur due to a concussion, what time frames indicate an excellent, good or poor prognosis?
How long does it usually take for a concussion to resolve?
At injury= excellent
7 days: edema/contusion= good
After 7 days from glial scarring- lead to epilepsy
Sxs <72hrs
Resolve 7-10 days
What happens during Second Impact Syndrome
What are the protocol steps for returning to sports after a concussion
Repeat concussion during healing phase leading to rapid increase of ICP
24hrs between each:
1: complete rest
2: light aerobic, no resistance
3: sport specific exercise
4: resistance, drills
5: full contact after clearance
6: game play
Define Post Concussive Syndrome
What are the two primary HAs seen in Peds?
Altered mental capacity of sleep, school or thinking post concussion
Migraine
Tension
What are the secondary HAs seen in Peds?
What is the most recurrent pattern of Primary HAs?
Associated w/ minor illness
May be 1st Sx of serious conditions
Tension
What are the red flags of Peds HA that warrant imaging?
Worst HA of life- hemorrhage
Morning HA- tumor
Wakes at night
Chronic/progressive- most ominous pattern)
Change due to position- pseudotumor/migraine
Recurrent morning vomiting
No FamHx
Almost all Peds w/ migraines will have ? in their Hx
What imaging modality is preferred when working a Peds PT up for HAs
FamHx of migraines
MRI w/ or w/out contrast
What PT education piece goes to PTs when Tx HAs?
What other class of drug can be used and when are they c/i?
NSAID/ASA no more than 2-3x/wk to avoid rebound HAs
Triptans- SRAs
C/i- focal neuro deficit w/ migraine
Basilar migraine sign/syncope
Since there are no FDA approved migraine prophylaxis drugs, what drugs can be used and when
What drug is C/i in Peds w/ Hx of migraine w/ aura?
If >1 HA/wk: Propranolol Ami/Amitriptyline Valproic acid/Topiramate Verapamil Cyproheptadine (antihistamine)
OCPs
Define Epilepsy
What are the 4 types and what type are not classified here
Recurrent unprovoked seizures
Focal/partial- from one region of cortex
Unclassified
General- from both hemispheres simultaneously
Unknown
Febrile- separate category
Define Generalized seizure and the 3 types
Define Focal seizure and the 2 types
Begin diffusely
Febrile, Absence (petite), Tonic Clonic (grand)
Formerly partial seizures
Simple- no altered consciousness
Complex- altered consciousness
Imbalances in what 4 E+ can lead to seizures
What do PTs retain during focal seizures and what meds are used for these PTs?
Glucose Na Ca Mg
Consciousness preserved
Oxcarbezapine
Levetiracetem
What is different in Complex Partial Seizure
What two things may be seen during absence seizures and what med is different in this Tx?
Altered LoC
Automatisms- unconscious actions of drool, pupil dilation or color changes
Eye lid flutter/eye roll up
Hyperventilation
Ethosuximide
What is seen in PTs w/ Generalized Motor Seizures
What can be done as first aid for seizures?
Bilateral or Focal to Bilateral tonic-clonic
Incontinence
Postictal period
Place on side
Clear mouth if open
Loosen clothes/jewelry
Extend head
What is the MC type of seizure between 6mon-5y/o
What are the two types?
Febrile
Simple: <15min and once/24hrs
Complex/Atypical: >15min, repeats w/in 24hrs or PT has pre-existing neuro issues
Febrile status epilepticus >30min
What med is used for Febrile Seizure management
What two drugs are not used?
What lab result needs to be checked?
Rectal Diazepam
Daily anticonvulsant
Antipyretic
Fe deficiency
What is the myth associated w/ fevers and seizures?
What is the truth?
Higher temp is associated w/ increased chance of seizure
How rapidly body inc temp correlates to likelihood of seizure
Define Status Epilepticus
What meds can be used in the immediate setting?
What labs are ordered?
Neuro emergency due to seizure lasting >30min w/out return of consciousness
D/M/Lorazepam
Glucose CBC, Platelet BMP Anticonvulsant levels Toxicology
What issues of weakness/hypotonia are seen in the anterior horn, peripheral, junctions or muscle cells?
Anterior: spinal muscular atrophy, poliomyelitis
Peripheral: Guillain Barre, Charcot Marie, Tick paralysis
Junction: M Gravis, Botulism
Muscle: dystrophies
What are the three types of Spinal Muscular Atrophy
1- Wernig Hoffman Dz: severe infant dz leading to death before 2y/o
2- Kugleberg Welander Syndrome: MC; late infantile Dz but PTs reach adulthood
3: chronic, juvenile form w/ normal life expectancy
What would be seen in PTs w/ Spinal Muscular Atrophy
What is the mainstay of management to prevent death
Proximal atrophy/weakness
No DTRs
Tongue fasciculations
Respiratory infections
Polio invades and destroys ? part of the nervous system?
This is an endemic in what 4 countries
Anterior horns
Pakistan Afghanistan Nigeria India
What type of vaccine is used for Polio in the USA?
Guillain Barre Syndrome is AKA and MC cause of ?
IPV
Acute Inflammatory Demyelinating Polyradidiculoneuropathy
Acute flaccid paralysis in kids
Guillain Barre usually follows ?
What are the characteristic features of this Dz
10 days post respiratory/GI infection of M Pneumonia, C Jejuni
Areflexia
Flaccid
Symmetrical ascending weakness
How is Guillain Barre Tx
Charcot Marie Dz affects ? nerves first?
IVIG
Plasma exchange or ImmSuppression if IVIG fails/Dz progresses quickly
Motor then sensation and autonomic
What is the MC form of Charcot Marie and where does it affect the most on the body
What kind of deformity does this create?
Type 1A, Peroneal/Tibial nerves
Pes Cavus deformity
What is done for Charcot Marie Tx
Tick saliva toxin is similar to ? and it’s pattern mimics?
Supportive foot bracing
Physical therapy
Botulism
Guillain Barre
What are the 3 types of M Gravis?
What muscles are affected?
Juvenile
Transient neonatal- have maternal anti-AChR
Congenital- genetic mutation of junctional components
Abs block ACh receptors causing fatigue in striated muscles
How is MGravis Dx
What can be done for management
Tensilon/Edrophonium test causes improved ptosis/strength
Pyridostigmine- ACh inhibitors
Thymectomy- dec Abs
PO steroids/ImmSupp drugs
Severe- plasmaphoresis and respiratory support
What two foods are not given to infants under 12mon due to risk of botulism?
What are the first Sxs if poisoning has occurred
How is it Tx?
Honey
Canned foods
Constipation
Poor feeding
IVIG, respiratory/support care
How are muscular dystrophies characterized
What causes the weakness in these Pts?
Myofiber degeneration and replacement w/ fibrotic tissue
Dystrophin
What are the two types of muscular dystrophy
Which one is ASx in infancy
Duchenne- x-linked
Beckers
Duchennes- delayed milestones or toe walkers
What can be seen on PE of a PT w/ Duchennes
What ages are arms and legs useless?
Gower sign- climbing up own body to stand
Pseudohypertrophy of calves due to thigh atrophy
Arm weakness at 6y/o
Wheelchair at 12y/o
Define Prader Willi Syndrome
What does this syndrome cause
Deletion of paternal chromosome 15q11q13
Neonatal hypotonia causing poor feeding and FTT
What lab results would be elevated in muscular dystrophy work ups
What Dzs would cause an abnormal CSF result
CK
Polio, Guillan Barre
How are muscle dystrophies Dx
Brain and skin share what common embryonic origin?
Muscle biopsy show inc CT in muscles
Ectoderm
What is Neurofibromatosis Type 1
NF 1 gene encodes neurofibromin which makes these fibromas made up of ?
von Reckling Dz
Autosomal dominant on Chrom 17
Schwann cells
What is the cardinal feature of Neurofibromatosis Type 1
How is NF Type 2 different?
Cafe au Lait spots
Axilla/Inguinal freckles
Iris hamartoma (Lisch nodules)
Auto Dom mutation of Chrom 22 which codes for merlin
What would be seen on PE of NF Type 2
This Dz commonly affects ? CN
Posterior/Cortical cataracts
No axillary freckles
CN8
What are the two types of Tuberous Sclerosis mutations
PT w/ this Dz present w/ ?
Chrom 9- makes hamartin
Chrom 21- makes tuberin
Hamartomas
Tuberous Sclerosis is a common cause of ?
What are the cardinal features?
Infantile spasms
Mental retardation
Epilepsy
Facial angiofibromas (adenoma sebaceum)
What can be seen on PE of Tuberous Sclerosis
What malignant manifestation can occur?
Cerebral cortex tubers
Adenoma sebaceum
Ash leaf spots
Shagreen patches on lumbar/glutes
Renal angiomyolipomas- MC cause of death
How is Tuberous Sclerosis Dx and Tx
Define Sturge Weber Syndrome
Imaging/Skin biopsy
Anti-seizure meds
Angiomas overlying cerebral cortex associated w/ port-wine stains on trigeminal nerve and glaucoma
What is the MC presenting feature of Sturge Weber Syndrome
Hos is this syndrome Tx?
Seizures
Laser surgery for nevus flammeus
Anti-convulsants
Hemispherectomy- difficult seizures
Define Rachischisis
What are the 3 different forms
Spina bifida occulta
Meningocele- open meninges
Meningomyelocele- open meninges/cord
Myeloscisis- open skin/cord
What are the 2 types of hydrocephalus
How is this Tx?
Communicating w/ subarachnoid
Non-communicating
Ventriculoperitoneal shunt
Define Arnold Chiari Malformation Type 1
What do these PTs present complaining of?
Cerebellar tonsils protrude down through formen magnum
HA, neck pain, Inc urination urgency
Define Arnold Chiari Type 2`
Define Dandy Walker Malformation
Hydrocephalus w/ myelomeningocele anomaly of hind brain due to elongated 4th ventricle
Cystic expansion of 4th ventricle in posterior fossa
How do 90% of PTs w/ Dandy Walker present?
Define Craniosynostosis
Hydrocephalus
Premature closure of suture, Tx w/ surgery
Pseudotumor Cerebri is AKA ?
What are the 3 origins of this issue?
Idiopathic Intracranial HTN
Idiopathic
Meds: TCA, Vit A, OCP
Thyroid, Addison
What is a predisposing factor of Pseudotumor Cerebri
How is it Tx
Overweight
Azetazolamide/diuretic
Topiramate
CCS
What are the REM sleep stages
How much sleep do Infants/Peds need?
NREM 1-3: lightest sleep to deep/slow wave
REM: awake like sleep pattern
Infant: 16-18hrs/day
1y/o: 10-11 w/ 2-3hrs of nap
12y/o: 9-10hrs
How much sleep do bottle/breast fed babies sleep
When does the ability to self soothe develop?
Bottle: 2-5hrs
Breast: 1-3hrs
First 12wks of life
When do night time fears develop?
What age group who has cosleeping pattern is associated w/ sleep problems
1-2yrs old
3-5yrs old
Define sleep onset association subtype
Define Limi-setting subtype
Infant/kids w/ frequent awakenings in new/unfamiliar surroundings
Pre-school/older kids w/ bed time resistance due to night time fears
When are parasomnias MC?
When is sleep walking more common?
Preschool kids
2x higher >5y/o w/ Hx of night terrors
10x higher if FamHx
What is the difference of timing between night terrors and night mares?
What causes circadian rhythm d/o
Terror: First 1/3
Mares: last 1/3
Delayed sleep phase
Although rarely warranted, what two meds can be used for Peds w/ sleep d/o
Kids should be able to walk w/ normal gait and walk in tandem by ? age
Melatonin
Clonidine
6y/o
How are DTRs graded
When should babinski be gone?
0: absent
1: trace
2: normal
3: exaggerated
4: clonus
12-18mon
What microbes are more likely to cause UTIs?
What are the two types?
E Coli Klebsiella Proteus Enterococcus Pseudomonas
Pyelonephritis
Cystitis
When do UTI prevalence peak?
What microbes can cause cystitis?
Infancy
Toilet training
Onset of sexual activity
E Coli
Adenovirus- MC in boys
What is the MC serious bacterial infection in kids <24mon?
What UA findings are Dx for UTIs
Pyelonephritis
Bacteria/Pyuria +50K
Leukocyte esterase AND nitrite: 99% specific for UTI
How are UA samples gathered for suspected UTIs?
What UA result is indicative of sample contamination?
Clean catch if toilet trained
Catheter if 2-24mon
> 3-5 epithelial cells
What test is the gold standard for UTI Dx
What results are Dx?
UA culture
> 50K one pathogen + pyuria
100K in older kids/adolescents
What parenteral ABX are used for UTIs
How long are they used for infant/adolescents
Ceftriaxone
Cefotaxime
Cefepime
Neonate: 10-14 days
Older: 7-14 days
What ABX can be used PO for UTIs?
When are renal/bladder US conducted when investigating UTIs?
Cephalosporin
Amoxicillin
Nitrofuratoin- no febrile UTIs
Trimethoprim
1st UTI in infant or non-toilet trained kid
When is a VCUG ordered for Peds UTIs?
What is the MC cause of this reflux?
1st UTI in 2-24mon kid w/ abnormal US or,
Recurrent febrile UTIs w/ normal US
Congenital ureterovesical junction incompetence
What are the 5 grades of VUR dx tests?
Which ones are less likely to resolve by them self?
1: reflux into ureter
2: reflux into renal calyx, no dilation
3: dilation
4: dilation, no angle of fornices
5: loss of papillary impression in most calyces
Bilateral 3 or 4-5
What is the key feature of Nephrotic Syndrome
What can happen during this issue
Proteinuria >3.5g/24hrs
Protein:creatinine ratio > 2
Hypoalbuminemia <2.5
Hyperlipidemia >200
Hyperlipidemia
Inc infection risk
Hypercoagulability
Edema: eye, LE
Minimal Change Nephrotic Syndrom
Does not progress to renal failure Edema above eyes Inc BUN Normal complement levels Remission after 8wks of steroid- 90%
No HTN
Focal Segmental Glomerulosclerosis
Associated w/ HIV, Sickle, Heroine use, Reflux nephropathy
Remission: 15-20%
Membranoproliferative Glomerulonephritis Type 1
Membranoproliferative Glomerulonephritis Type 2
Hematuria
Low complement
Normal complement
What is the MC presenting issue of nephrotic syndrome
Define Transient/Postural proteinuria
Sudden onset of dependent pitting edema/ascites
Transient: post work out
Postural: significant proteinuria when upright
MC in tall, thin PTs
Not associated w/ renal Dz
What would be seen in Tubular proteinuria
Define Fanconi Syndrome
LMW protein in urine
Tubular proteinuria
Tubular E+ wasting
Glycosuria
Define Glomerular Proteinuria
When is this usually seen?
Large and small weight proteins in urine
Post Strep + Lupus
Most Peds under 13y/o will have ? nephrotic syndrome
How is the edema in these PTs Tx?
Steroid responsive NS
May start steroid Tx w/out renal biopsy
Loop diuretics
Salt restriction
PTs w/ nephrotic syndrome are at increased risk for bacteremia/peritonitis from ? microbes
If these PTs are responding to steroid therapy, they have little risk of developing ?
Strep pneumo
E coli
Klebsiella
Renal failure
What are the cardinal features of glomerular injury in glomerulonephritis?
What can cause factitious hematuria
HTN Oliguria Proteinuria Edema Renal insufficiency
Rifampin
Urate crystals
Beets
What is the MC chronic glomerulonephritis
These PTs present w/ what other current issue
IgA Nephropathy- Bergers Nephropathy
URI/GI infection
Define Hereditary Nephritis
What PE finding is pathgnomonic for Dx
Alport syndrome
X-chromosome mutation of Type 4 collagen, causes abnormal basement membrane leading to ASx hematuria
Anterior Lenticonus in the eye
Males w/ ASx hematuria need to have ? test done
How does post-strep glomerulonephritis present
Audiogram- sensorinueral hearing loss
Post GAS infection
Sudden onset gross hematuria, HTN and renal dysfunction
What is the MC type of hemolytic uremic syndrome
What 2 meds are not used during supportive Tx?
Prodromal diarrheal illness from contaminated meat/produce/water w/ E Coli O157:H7
ABX
Antidiarrheals
What is the MC inherited kidney dz
What other issues will be seen in these PTs?
Auto Dominant Polycystic kidney Dz, Polycystin 1 or 2 defects
Cerebral aneurysm
Liver, spleen, brain, pancreatic cysts
Auto Recessive form of PKD is defective in ?
What type of kidneys are Turners Syndrome likely to have
Fibrocystin
Horse shoe
Unilateral Renal Agenesis is MC to be seen ?
This is associated w/ ? issues
Diabetic mothers
Black mothers
VUR, VACTERL, Turner’s
Bilateral renal agenesis is associated w/
What are the 3 DDx of testicular torsions?
Potter syndrome- flat face, clubfoot, pulmonary hypoplasia
Epididymo-orchitis: abnormal UA, + Prehns
Appendiceal torsion: blue dot on scrotum
Incarcerated hernia- palpable hernia w/ valsalva
Cryptorchidism is more common in ? PTs
Tx w/ ? procedure prior to ? age rarely causes testis tumor
Spontaneous descent is rare after ? age
Premature
Orchidopexy <2y/o
4-6mon
Define hydrocele
What are the two types
Fluid collection in tunic vaginalis
Communicate: w/ peritoneal space
Non-Comm: MC; processus vaginalis obliterated
When are hydroceles referred to Urology?
Define Hypospadias
Non-Comm: 18mon
Comm: smallest in AM, associated w/ inguinal hernia, refer
Failure of ventral urethral folds to fuse
Hypospadias commonly also present w/ ? issue
When is surgical Tx preferred?
What must be told to the parents?
Undescended testes
Inguinal hernia
6-12mon, but before 18mon
No circumcision until correction surgery
What type of enuresis has a strong FamHx link?
What can be the cause of Secondary enuresis?
Primary nocturnal
Constipation UTI DM/DI
Due to development of the same time, eye issues can be related w/ ? other issues
What are neonatal surgery red flags?
Kidneys
Polyhydramnios
Delayed meconium
Abdominal distension
Perinatal vomiting
What causes infant vomit to be bilous or non-bilous
What is the MC type of tracheoesophageal fistula
Bilous= distal to ligament of triaz Non= proximal to ligament
Esophageal atresia w/ distal TEF
What causes polyhydramnios
How do they present
Fetus w/ atresia can’t swallow amniotic fluid
Drooling, Bubbling, Single umbilical artery
If T/E fistula is suspected, what is avoided after birth?
T/Es have the strongest association w/ VACTERLs which stand for?
First feeding
Vertebral anomaly Anal atresia Cardiac anomaly TEF Renal anomaly Limb anomaly
How are TEFs Dx
Failure to pass OG cath
CXR w/ tube in pouch of esophagus
Gastrografin swallow study
What is the #1 cause of intestinal obstruction before 3mon of age?
What kind of issue is this and when is it MC?
Pyloric stenosis
Hypertrophy + spasm= obstruction prox to LoT
MC in male and first born
How do PTs w/ pyloric stenosis present?
What will be seen on labs?
Non-bilous projectile vomit w/ FTT
Hypo calorie, potassium
Metabolic alkalosis
Elevated BUN
What is seen on PE of Pyloric Stenosis
What is seen on US and barium imaging?
Olive sign
LUQ peristaltic waves
Elongated thick pylorus
String sign
How are Pyloric Stenosis PTs managed?
What is the name of the Tx procedure
NS bolus
D5 w/ K+ to Tx alkalosis and make the candidate for surgery
Pyloromyotomy
What are the 4 parts of the diaphragm that fuse during development?
Where is it innervated from?
Pleuroperitoneal Muscular Septum Dorsal
Wk 6: thoracic
Wk 8: 1st lumbar
Congenital diaphragmatic hernia is usually on ? side causing defected development of ?
This defect leaves a hole in the diaphragm where?
L side
Pleuroperitoneal fusion
Posterolateral
During a congenital diaphragmatic hernia, bowels herniate into the thoracic cavity through ? foramen?
This deformity causes what findings seen on PE?
How is it Dx?
Bochdalek foramen
Scaphoid abdomen
Bowel sounds in L chest
X-ray
What is a common type of ventral hernia?
What are the predisposing risk factors for this development?
What tissues can be contained here?
Umbilical hernia- contents still covered by subcutaneous tissue and skin
African American
Low birth weight
Omentum, small intestine
When are umbilical hernias Tx w/ surgery?
How do PTs w/ intestinal malrotation/volvulus present?
After 5y/o
Sx/Strangulation
Larger after 2y/o
Obstruction is distal to LoT= bilious vomiting in 1st mon of life
What can cause vomiting in PTs w/ volvulus if there’s no malrotation?
What would be seen on a barium study?
Ladd’s Bands
Cecum in RUQ
Upper GI cork screw affect
While correcting a malrotation/volvulus, what prophylactic procedure is done?
Intestinal atresias are associated w/ what abnormalities?
Appendectomy
Trisomy
Rotations
Annular pancreas
Meconium ileus w/ CF
Infant w/ Dx of atresia needs to have / test?
How do intestinal atresias present?
CF
Polyhydramnios Failure to feed Bilious vomit Abdominal distension Jaundice
What two conditions present w/ non-bilious vomit?
What is seen on imaging of PTs w/ intestinal atresia?
TEF
Pyloric stenosis
Duodenal atresia= Double bubble sign
Distal atresia- dilated/air filled bowel
Define Gastroschisis
What is thought to be the cause?
Split/open stomach, split in abdominal wall w/out affecting umbilicus
Absence of omphalomesenteric artery
Why are gastrochisis PTs hard to Tx w/ surgery?
What causes an omphalocele?
Intestines are exposed to amniotic fluids, develop thick covering
Muscle/skin growth dysfunction, bowel remains in umbilical ring and covered
No amniotic irritation
Omphaloceles are associated w/ ? issues
Meckels contain ? tissues
These are remnants of ? duct
Cardiac
Beckwith Syndrome
Gastric, Pancreatic
Vitelline/omphalomesenteric
Once discovering a anorectal malformation, what is the next step?
Why is this step important?
MRI of lumbrosacral spine
Tethered cord
What type of issue is Hirschsprungs Dz
What part of the colon is usually involved
Absent motility and functional obstruction
Rectosigmoid
How do most Hirschsprung PTs present?
What PTs usually have NEC?
No stool passing in first 24hrs of life
Premature: <1500g or <34wks
What are the two types of lead points that can result in intussusception
What is an associated cause of this?
Lyphoid hyperplasia- peyers patches
Abnormal anatomy- ileocecal valve/Meckels
Rotavirus or old immunization
How do intussusceptions present?
What can be seen on PE?
Paroxysmal abdomen cramping
Current jelly stool
Sausage shaped mass in RUQ
What test is therapeutic and Dx of intussusceptions?
What can potentially cause a blocked appendix?
Air/Barium enema
Fecalith
Lymphoid hyperplasia
Neoplasms
What PE landmark is used to assess for appendicitis?
What is the alvardo rule for scoring?
McBurney’s: 2/3 distance between umbilicus to ASIS
Migrating pain Anorexia N/V Rebound tenderness Fever WBC shift >75%
RLQ tenderness
Leukocytosis >100K
What image is more accurate for identifying appendicitis
After Dx of NEC, what is the next step?
CT w/ contrast
Stop enteral feedings
Who is usually the reported perpetrator of abuse?
Who is more likely to cause serious injury?
Mother
Father/maternal boy friend
What is the MC type of emotional abuse?
What is the MC type of abuse overall?
Verbal
Neglect
What types of Fxs are suspicious for abuse?
What color bruise by what day of healing
Metaphyseal
Scapular
Vertebral
Red 0-2 Blue/purple 2-5 Green 5-7 Yellow 7-10 Brown 14-28 Hgb Bilveriden Bilirubin
How can the age of a Fx be estimated?
What is the shaken baby trifecta
7-14: callus
14-21: loss of Fx line
21-42: callus inc w/ density
Subdermal hematoma
Retinal hemorrhage
Brain swelling
What 3 STDs in kids are almost always due to abuse?
What PE finding can be seen in neglect abuse?
G/C/Syphilis
Type 1 growth deficiency- normal head/length, low weight
What are the top 5 unintentional injuries in kids aged 9-18
What does a Primary Assessment include?
MVC Drowning Burn Falls Toxins
ABCDE
Airway Breathing
Circulation Deformity Environment/exposure
What adjuncts are available during a primary assessment?
Max, min and intubation scores on GCS
Pulse ox O2 Labs/Rads IV VS
15, 3, less than 8
GCS section mnemonic
ESSPN- 4
Eye Spont Sound Pain No
VOCAIN- 5
Verbal Orient Confuse Appropriate Incomp Nil
MY OLD BEN- 6
Obey Localize Draws Bends Extend Nil
What types of fluids are used in Peds resuscitation?
What combo needs to be avoided?
Isotonic- NS, LR
LR w/ pRBCs= hemolysis
What is the primary cause of cardiopulmonary arrest in kids?
What level is the definition of hypoxemia and hypercarbic
Respiratory arrest
PaO2 <60mm
PaCO2 >50mm
What are the early and late signs of hypoxic respiratory failure
5 types of shock
Tachy/Tachy
Cyanosis/AMS
Hypovolemic Distributive Dissociative Obstructive Cardiogenic
What is the MC type of shock in kids?
Define Distributive shock
Acute hypovolemic
MC caused by sepsis
Adequate volume, maldistributed blood flow
Define Cardiogenic shock
Define Obstructive shock
Dec heart contractility
Mechanical obstruction to ventricular filling/outflow
MC type of trauma in Ped
What’s the 2nd MC cause of trauma death?
What’s the 3rd cause?
Head
Thoracic trauma- rib fx, contusion, pneumothorax
Abdomen trauma
What abdominal injury causes severe bleeding?
MC location of Fxs
Liver
Physeal: radius, tibia, fibula
SALTR
MC type of Fx
Separated Above Lower Through Rammed
Complete- spiral, transverse, comminuted, oblique
Define Greenstick Fx
Buckle Fx AKA and typically occur ?
Fx on tension side
Bend deformity on compression side
Torus, metaphysis
What is a Gymnast’s wrist
Little Leaguers elbow
Distal radial physis Fx
Medial humerus epicondyle apophysitis
Legg Calve Dz is associated w/ ?
What is the characteristic presentation
Favtor V Leiden
Child w/ atraumatic painless limp
Define ALTE
What are the MC causes
Acute Life Threatening Event characterized by apnea, color change, limpness and choking
GERD
Laryngospasms
What is the 3rd leading cause of injury related death in kids 1-9y/o
How is this injury classified
Burns
Percent
Location
Association w/ injuries
Depth
What does underlying dermis look like after debrisment?
How are deep partial thickness burns classified?
Painful
Erythematous
Wet
Blanches
2nd degree- involved epidermis and deeper dermis
How are 4th degree burns classified
Burns disrupt what 3 skin functions
All of 3rd w/ fascia, muscle or bone
Heat regulation
Fluid preservation
Infection barrier
Burns cause a release of ?
Smoke inhalation may be associated w/ ?
Cytokines
Mediators inc permeability, dec plasma volume, dec CO
CO toxicity
What does initial management of burns involve
Nutritional support is needed due to managing ?
20ml/kg LR bolus
Titrate to urine output of >1mL/kg/hr
Hyermetabolic state
What meds are used for burn wound care?
What two Txs are avoided when Tx poisonings?
Silver sulfadiazine
Polymyxin B/Bacitracin
Syrup of Ipecac
Gastric lavages
What does MUDPILES stand for
Methanol Uremia DKA Paraldehyde Isoniazid Lactic acidosis Ethanol Salicylates
Triad of narcotic poisoning
What is a common source of Fe poisoning
Miosis
Dec mental status
Respiratory depression
Prenatal Fe tabs
How does Fe poisoning present
What is the antidote and side effects of its use?
Hemorrhagic gastroenteritis
HOTN
Heptatitis
Deferoxamine
Not in renal failure
Predispose to Yersinia sepsis
What is an early and late sign of salicylate poisoning
How can urine be forced into alkalinization
Tachypnea, respiratory alkalosis
Severe anion gap, metabolic acidosis
IV HCO3 and D5W
How does lead poisoning present
How is it Tx
Alopecia
Seizure
Coma
Basophilic stippling
Edetate Ca disodium
Dimercaprol* peanut allergy
Succimer
How does carbon monoxide poisoning present
Methanol poisoning leads to ?
Ethylene glycol poisoning leads to ?
Flu like Sxs
Cherry red
Groups of people
Optic/renal edema
Renal/CNS toxicity
Both methanol and ethylene glycol poisoning present w/ ?
How is it Tx
Metabolic acidosis
D5W
Fomepizole
Thiamine and B6 (ethylene)
Folic acid (methanol)
How does organophosphate poisoning present
How is this Tx
SLUDGE
Salivation Lacrimation Urination Defecation Gastroenteritis Emesis
Atropine
Pralidoxime
When is urticaria acute or chronic
How is angioedema different and caused
+ or - 6wks
Below dermis
Mast cells, IgE mediated
How is anaphylactoid different from anaphylaxis
When is this seen
Not IgE mediated
C3a and C5a anaphylatoxins
Serum sickness after blood transfusions
Complications of SCFE
What type of shock is called Warm shock
Chondrolysis
Avascular necrosis
Distributive
What type of shock can cause JVD in kids
Use caution when pushing fluids in ? type of shock?
Cardiogenic
Cardiogenic
