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Peds: Block 3 Flashcards

1
Q

Define Hypochromic, Microcytic anemia

What diseases does this include?

A

Dec Hgb production

Chronic Dz
Lead
Iron deficiency
Thalassemia

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2
Q

Define Normocytic Anemia

What issues can cause macrocytic anemia?

A

Systemic illnesses impairing marrow RBC synthesis

B12/Folic acid deficiency*
Trisomy 21
Hypothyroid
Newborn

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3
Q

What are Sxs of acute anemia and which one is especially seen in infants?

What Sxs may be seen in Chronic Anemia

A

Murmur Exercise Intolerance Tachy HA Excessive sleeping*
Fatigue Irritable Poor feeding Syncope

Minimal tachy/flow murmurs

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4
Q

What findings dictate if a PT needs a transfusion or not?

What are the MC presenting S/Sxs of anemia

A

CV and functional impairment, not Hgb level

Pallor Jaundice Splenomegaly

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5
Q

PT w/ petechiae, purpura, deeper bleeds indicate a generalized ?

Infiltrative d/o or systemic illness induced anemia may present w/ ? PE findings?

A

Hemorrhage

Hepatosplenomegaly
Lymphadenopathy

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6
Q

What part of the CBC help differentiate the cause of the issue?

How are anemia categorized?

What does the RDW do?

A

WBC/Platelet count

RDW and Peripheral smear

Measures variation in RBC sizes/indicates abnormal sizes

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7
Q

Dec RBC production, norm RDW can be caused by ?

What are the etiologies of Macrocytic, Hypo Micro, Target cell and Schizocyte cells?

A

Marrow failure (pancytopenia)
Anemia, chronic dz (microcytic)
Transient erythroblastopenia

Macro: B12/Folic deficiency
Hypo Micro: Fe deficiency
Target: HbCC Dz
Schizocytes: hemolytic-uremic syndrome

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8
Q

Inc RBC destruction, high RDW can be due to ?

Peripheral smears asses ? parts of blood work?

A

G6PD deficiency
Sickle cell
Spherocytosis

RBC, WBC, Platelet morphology, Fe and Lead levels
Determines if anemia is due to erythroid line or marrow elements

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9
Q

What does it mean if a reticulocyte count is elevated, normal or dec?

This measurement can indicate severity of ?

A

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

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10
Q

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?

A

CBC w/ microcytic anemia
High RDW, Red RBC
Norm WBC, Platelet

4mon
Start Fe foods @ 6mon

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11
Q

How long are Fe Deficient anemias Tx?

A

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

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12
Q

How is normocytic anemia suspected dx made and how is it confirmed?

What are the common DDx for Macrocytic Anemias

A

Hx to Dx of dec production
Peripheral smear- dx for destruction

Hypo BAFFLE
Hypothyroid
B12 deficiency
Aplastic anemia
Folate deficiency
Fanconi
Liver dz
ETOH
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13
Q

What labs are ordered to work up macrocytic anemia

What is done for Tx and for how long?

A

B12 LFTs Folate TSH/Thyroid

B12/Folate replacement
CBC improvement <1wk
Tx liver/thyroid issue

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14
Q

Aplastic anemia is most often ?

How is it Dx?

What is the criteria for a severe Dz Dx?

How is it Tx?

A

Idiopathic

Marrow biopsy

Reticulocyte count <50K
Platelets <20K
Neutrophil <500

Hematopoietic stem cell transplantation
ImmSupp therapy

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15
Q

Define Fanconi Anemia

How does it present

How is Fanconi anemia Tx?

A

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

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16
Q

What are the 6 types of hemolytic anemias

A

I RIMES
Intrinsic- thalassemia, sickle

RBC membrane defect- spherocytosis

Isolated site- vasculitis

Mechanical

Extrinsic- immune mediated

Systemic d/o- DIC, malignancy

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17
Q

What will be seen in lab results of hemolysis

Define Schistocytes seen in hemolytic anemia

What are two possible findings seen during this issue

A

Inc bilirubin/serum Hgb
Dec haptoglobin

Broken RBCs

Hemoglobinuria
HyperKalemia

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18
Q

Define Thalassemia

Normal Hgb is made of ?

A

Insufficient Hgb production from dec A/B-globulin production

HgA= A2B2
Four A genes= 4 A proteins
Two B genes= 2 B proteins

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19
Q

What are the 4 versions of Alpha Thalassemia

A

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

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20
Q

Define Beta Thalassemia

A

One: thalassemia minor (heterozygous)- mild macrocytic anemia

Two: thalassemia intermeida (compound heterozygous) moderate hemolysis, splenomegaly, mod/sev anemia (not transfusions dependent)

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21
Q

Define Thalassemia Major

A

Cooley’s Anemia
2 gene deletion- homozygous
Severe hemolysis, ineffective erythropoiesis, inc Hgb F
Transfusion dependent, frequent crisis/hemachromatosis
Reqs stem cell transplantation

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22
Q

Define Sickle cell anemia

Define Sickle Cell Dz

A

HbSS homozygous
HbS >50% of Hgb

Heterozygotes w/ one B-globulin includes sickle cell mutation and 2nd: Hbc, Beta-thalassemia, HbD

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23
Q

What do PTs w/ Sickle Cell and a fever need?

By 5yrs they are functionally asplenic and vulnerable to what 3 microbes?

A

Medical emergency
+6mon- splenic dysfunction

HIB
N Meningitidis
Strep Pneumo

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24
Q

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?

A

Encapsulated: Strep Pneumo, HiB, N Meningitidis

Salmonella
Staph A

Parvovirus B19

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25
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
26
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
27
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
28
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
29
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
30
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
31
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
32
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
33
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
34
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
35
# 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
36
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
37
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
38
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
39
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
40
# 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
41
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
42
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
43
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
44
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
45
# 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
46
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
47
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
48
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
49
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
50
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
51
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
52
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 ```
53
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
54
Peds w/ CA w/ ? two Sxs are admitted What is the most common childhood cancer?
Fever + neutropenia Leukemia
55
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
56
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
57
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
58
What is the 3rd MC malignancy in children? When is the MC new Dx?
Lymphoma 15-19y/o
59
Although not proven, what etiology is linked to lymphoma?
EBV Hodgkin- MC lymphoma Non-Hodgkin- male Caucasian that inc w/ age
60
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
61
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
62
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
63
What is the prognosis of Hodgkins? How is Non-Hodgkins Tx?
90% w/ chemo/rad Chemo, rarely surgery/radiation used
64
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
65
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
66
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
67
What is the MC presenting Sx of neuroblastoma development? How else can these present?
Abdomen mass/pain Horners Paraneoplastic syndrome: sweating, diarrhea, opsoclonus/myoclonus
68
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
69
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
70
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
71
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
72
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
73
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
74
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)
75
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
76
# 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
77
# Define Lethargic Define Obtunded
Difficulty maintaining aroused state Dec arousal but responsive to stimuli
78
# Define Stupor Define Coma
Responds only to pain stimuli Unresponsive conciousness, dyfunction of hemispheres bilaterally, brainstem or both
79
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
80
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
81
# 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
82
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
83
Normally w/ a concussion, there are no ? deficits What is the preferred imaging?
Focal neurological Normal CT
84
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
85
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
86
# 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
87
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
88
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
89
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
90
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
91
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
92
# 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
93
# 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
94
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
95
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
96
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
97
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
98
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
99
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
100
# 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 ```
101
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
102
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
103
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
104
Polio invades and destroys ? part of the nervous system? This is an endemic in what 4 countries
Anterior horns Pakistan Afghanistan Nigeria India
105
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
106
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
107
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
108
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
109
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
110
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
111
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
112
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
113
How are muscular dystrophies characterized What causes the weakness in these Pts?
Myofiber degeneration and replacement w/ fibrotic tissue Dystrophin
114
What are the two types of muscular dystrophy Which one is ASx in infancy
Duchenne- x-linked Beckers Duchennes- delayed milestones or toe walkers
115
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
116
# Define Prader Willi Syndrome What does this syndrome cause
Deletion of paternal chromosome 15q11q13 Neonatal hypotonia causing poor feeding and FTT
117
What lab results would be elevated in muscular dystrophy work ups What Dzs would cause an abnormal CSF result
CK Polio, Guillan Barre
118
How are muscle dystrophies Dx Brain and skin share what common embryonic origin?
Muscle biopsy show inc CT in muscles Ectoderm
119
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
120
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
121
What would be seen on PE of NF Type 2 This Dz commonly affects ? CN
Posterior/Cortical cataracts No axillary freckles CN8
122
What are the two types of Tuberous Sclerosis mutations PT w/ this Dz present w/ ?
Chrom 9- makes hamartin Chrom 21- makes tuberin Hamartomas
123
Tuberous Sclerosis is a common cause of ? What are the cardinal features?
Infantile spasms Mental retardation Epilepsy Facial angiofibromas (adenoma sebaceum)
124
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
125
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
126
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
127
# Define Rachischisis What are the 3 different forms
Spina bifida occulta Meningocele- open meninges Meningomyelocele- open meninges/cord Myeloscisis- open skin/cord
128
What are the 2 types of hydrocephalus How is this Tx?
Communicating w/ subarachnoid Non-communicating Ventriculoperitoneal shunt
129
# 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
130
# 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
131
How do 90% of PTs w/ Dandy Walker present? Define Craniosynostosis
Hydrocephalus Premature closure of suture, Tx w/ surgery
132
Pseudotumor Cerebri is AKA ? What are the 3 origins of this issue?
Idiopathic Intracranial HTN Idiopathic Meds: TCA, Vit A, OCP Thyroid, Addison
133
What is a predisposing factor of Pseudotumor Cerebri How is it Tx
Overweight Azetazolamide/diuretic Topiramate CCS
134
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
135
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
136
When do night time fears develop? What age group who has cosleeping pattern is associated w/ sleep problems
1-2yrs old 3-5yrs old
137
# 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
138
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
139
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
140
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
141
How are DTRs graded When should babinski be gone?
0: absent 1: trace 2: normal 3: exaggerated 4: clonus 12-18mon
142
What microbes are more likely to cause UTIs? What are the two types?
``` E Coli Klebsiella Proteus Enterococcus Pseudomonas ``` Pyelonephritis Cystitis
143
When do UTI prevalence peak? What microbes can cause cystitis?
Infancy Toilet training Onset of sexual activity E Coli Adenovirus- MC in boys
144
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
145
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
146
What test is the gold standard for UTI Dx What results are Dx?
UA culture >50K one pathogen + pyuria >100K in older kids/adolescents
147
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
148
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
149
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
150
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
151
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
152
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
153
Focal Segmental Glomerulosclerosis
Associated w/ HIV, Sickle, Heroine use, Reflux nephropathy | Remission: 15-20%
154
Membranoproliferative Glomerulonephritis Type 1 Membranoproliferative Glomerulonephritis Type 2
Hematuria Low complement Normal complement
155
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
156
What would be seen in Tubular proteinuria Define Fanconi Syndrome
LMW protein in urine Tubular proteinuria Tubular E+ wasting Glycosuria
157
# Define Glomerular Proteinuria When is this usually seen?
Large and small weight proteins in urine Post Strep + Lupus
158
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
159
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
160
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
161
What is the MC chronic glomerulonephritis These PTs present w/ what other current issue
IgA Nephropathy- Bergers Nephropathy URI/GI infection
162
# 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
163
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
164
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
165
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
166
Auto Recessive form of PKD is defective in ? What type of kidneys are Turners Syndrome likely to have
Fibrocystin Horse shoe
167
Unilateral Renal Agenesis is MC to be seen ? This is associated w/ ? issues
Diabetic mothers Black mothers VUR, VACTERL, Turner's
168
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
169
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
170
# Define hydrocele What are the two types
Fluid collection in tunic vaginalis Communicate: w/ peritoneal space Non-Comm: MC; processus vaginalis obliterated
171
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
172
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
173
What type of enuresis has a strong FamHx link? What can be the cause of Secondary enuresis?
Primary nocturnal Constipation UTI DM/DI
174
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
175
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
176
What causes polyhydramnios How do they present
Fetus w/ atresia can't swallow amniotic fluid Drooling, Bubbling, Single umbilical artery
177
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 ```
178
How are TEFs Dx
Failure to pass OG cath CXR w/ tube in pouch of esophagus Gastrografin swallow study
179
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
180
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
181
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
182
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
183
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
184
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
185
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
186
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
187
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
188
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
189
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
190
Infant w/ Dx of atresia needs to have / test? How do intestinal atresias present?
CF ``` Polyhydramnios Failure to feed Bilious vomit Abdominal distension Jaundice ```
191
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
192
# Define Gastroschisis What is thought to be the cause?
Split/open stomach, split in abdominal wall w/out affecting umbilicus Absence of omphalomesenteric artery
193
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
194
Omphaloceles are associated w/ ? issues Meckels contain ? tissues These are remnants of ? duct
Cardiac Beckwith Syndrome Gastric, Pancreatic Vitelline/omphalomesenteric
195
Once discovering a anorectal malformation, what is the next step? Why is this step important?
MRI of lumbrosacral spine Tethered cord
196
What type of issue is Hirschsprungs Dz What part of the colon is usually involved
Absent motility and functional obstruction Rectosigmoid
197
How do most Hirschsprung PTs present? What PTs usually have NEC?
No stool passing in first 24hrs of life Premature: <1500g or <34wks
198
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
199
How do intussusceptions present? What can be seen on PE?
Paroxysmal abdomen cramping Current jelly stool Sausage shaped mass in RUQ
200
What test is therapeutic and Dx of intussusceptions? What can potentially cause a blocked appendix?
Air/Barium enema Fecalith Lymphoid hyperplasia Neoplasms
201
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
202
What image is more accurate for identifying appendicitis After Dx of NEC, what is the next step?
CT w/ contrast Stop enteral feedings
203
Who is usually the reported perpetrator of abuse? Who is more likely to cause serious injury?
Mother Father/maternal boy friend
204
What is the MC type of emotional abuse? What is the MC type of abuse overall?
Verbal Neglect
205
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 ```
206
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
207
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
208
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
209
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
210
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
211
What types of fluids are used in Peds resuscitation? What combo needs to be avoided?
Isotonic- NS, LR LR w/ pRBCs= hemolysis
212
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
213
What are the early and late signs of hypoxic respiratory failure 5 types of shock
Tachy/Tachy Cyanosis/AMS ``` Hypovolemic Distributive Dissociative Obstructive Cardiogenic ```
214
What is the MC type of shock in kids? Define Distributive shock
Acute hypovolemic MC caused by sepsis Adequate volume, maldistributed blood flow
215
# Define Cardiogenic shock Define Obstructive shock
Dec heart contractility Mechanical obstruction to ventricular filling/outflow
216
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
217
What abdominal injury causes severe bleeding? MC location of Fxs
Liver Physeal: radius, tibia, fibula
218
SALTR MC type of Fx
Separated Above Lower Through Rammed Complete- spiral, transverse, comminuted, oblique
219
# Define Greenstick Fx Buckle Fx AKA and typically occur ?
Fx on tension side Bend deformity on compression side Torus, metaphysis
220
What is a Gymnast's wrist Little Leaguers elbow
Distal radial physis Fx Medial humerus epicondyle apophysitis
221
Legg Calve Dz is associated w/ ? What is the characteristic presentation
Favtor V Leiden Child w/ atraumatic painless limp
222
# Define ALTE What are the MC causes
Acute Life Threatening Event characterized by apnea, color change, limpness and choking GERD Laryngospasms
223
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
224
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
225
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
226
Burns cause a release of ? Smoke inhalation may be associated w/ ?
Cytokines Mediators inc permeability, dec plasma volume, dec CO CO toxicity
227
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
228
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
229
What does MUDPILES stand for
``` Methanol Uremia DKA Paraldehyde Isoniazid Lactic acidosis Ethanol Salicylates ```
230
Triad of narcotic poisoning What is a common source of Fe poisoning
Miosis Dec mental status Respiratory depression Prenatal Fe tabs
231
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
232
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
233
How does lead poisoning present How is it Tx
Alopecia Seizure Coma Basophilic stippling Edetate Ca disodium Dimercaprol* peanut allergy Succimer
234
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
235
Both methanol and ethylene glycol poisoning present w/ ? How is it Tx
Metabolic acidosis D5W Fomepizole Thiamine and B6 (ethylene) Folic acid (methanol)
236
How does organophosphate poisoning present How is this Tx
SLUDGE Salivation Lacrimation Urination Defecation Gastroenteritis Emesis Atropine Pralidoxime
237
When is urticaria acute or chronic How is angioedema different and caused
+ or - 6wks Below dermis Mast cells, IgE mediated
238
How is anaphylactoid different from anaphylaxis When is this seen
Not IgE mediated C3a and C5a anaphylatoxins Serum sickness after blood transfusions
239
Complications of SCFE What type of shock is called Warm shock
Chondrolysis Avascular necrosis Distributive
240
What type of shock can cause JVD in kids Use caution when pushing fluids in ? type of shock?
Cardiogenic Cardiogenic

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