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