Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Critical Care I > Test 2 Roadmap questions > Flashcards

Test 2 Roadmap questions Flashcards

1
Q

3 most common types of pediatric cancer

most common is?

A

ALL (acute lymphoblastic leukemia)

AML (acute myelogenous leukemia)

Chronic myelogenous leukemia (CML)

Most common is ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

cancer of the blood and blood forming organs such as the bone marrow, lymph nodes and spleen

A

Leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

how is leukemia classified

A

the cell line affected and level where differentiation has been interrupted

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what inherited conditions predispose patients to ALL

A

Fanconi anemia

Trisomy 21

Ataxia telangiectasis

klinefelter syndrome

shwachman-diamond syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

genetic predisposition for CML

A

Associated with chromosomal translocation known as Philadelphia chromosome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

initial phase is “chronic” and associated with Leukocytosis, mild anemia and thrombocytosis

A

CML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

May progress from the chronic phase after a variable amount of time to the accelerated phase and ____ _____ which resembles acute _____

A

Blast crisis

resembles acute leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are blast cells

A

there is a dysregulation of hematopoietic development secondary to genetic abnormalities; stem cells that are malignant do not differentiate or mature properly. These cells are called blasts and accumulate in the marrow and possibly some solid organs (thymus, liver, spleen, kidneys, CNSO and cause impairment or failure of bone marrow function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

clinical presentation of ALL (can vary)

A 
Fevers
fatigue
anorexia
weight loss
nonspecific or bone pain
infections that do not resolve
persistent lymphadenopathy
WBC may be elevated; very high WBC may cause leukostasis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Clinical presentation of AML

A

Tend to appear more ill than patients with ALL

cytopenias (reduction in the number of mature blood cells) more significant

Severe anemia ->can lead to heart failure

ecchymoses

petechiae

epistaxis and other bleeding (ie: gum bleeding)

collection of tumor cells called chloromas, may present as masses most commonly located in the orbital or periorbital areas - may see ptosis or s/s consistent with spinal cord compression

leukemia cutis (small, colorless to blue/purple nodules under the skin - sign of leukemic infiltration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

CML clinical presentation

A

often asymptomatic
diagnosed incidentally
identified by abnormal CBC

non-specific symptoms'
fever
fatigue
weight loss
LUQ pain

pt in blast crisis will resemble those symptomatic with ALL or AML

can present with symptoms stemming from hyperleukocytosis (fever, bone pain, night sweats, priapism, malaise, splenomegaly causing abd pain)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Leukemia diagnostics

A

CBC with anemia

thrombocytopenia

leucopenia/leukocytosis

Blasts present on peripheral blood smear

Bone marrow aspirate is diagnostic when blasts comprise >25% of the marrow - marrow is evaluated for morphology, immunophenotyping, immunohistochemical stains and cytogenic abnormalities

Metabolic studies for kidney and liver function and to look for complications such as tumor lysis syndrome

LP - look for CNS involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Leukemia diagnosis with elevated WBC or hyperleukocytosis means? where to admit

A

WBC>100,000mm3
or tumor lysis syndrome

PICU

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Leukemia and hyperleukocytosis is what?

A

WBC >100,000mm3

results in increased blood viscosity

Medical emergency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

treatment for hyperleukocytosis

A

aggressive hydration

correction of metabolic disturbances

prevention of tumor lysis syndrome

may require leukopheresis or exchange transfusion

recombinant urate oxidase (rasburicase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

3 phases of ALL treatment

A

Induction

consolidation

maintenance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

chemotherapy treatment for ALL lasts how long and depends on ?

A

2-3 years depending on risk and gender

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

CNS therapy and ALL

A

required either for prophylaxis or treatment due to risk for CNS relapse and failure of systemic chemotherapy to penetrate the blood brain barrier adequately

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

AML treatment

A

short intense periods of multi- agent chemotherapy -> causes prolonged marrow hypoplasia and immunosuppression (may need Hematopoietic stem cell transplant (HSCT) from an allogenic donor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

CML treatment

A

current therapy is tyrosine kinase inhibitor (imatinib mesylate) while in chronic phase.

only curative is HSCT (hematopoietic stem cell transplant from an allogeneic donor) which is indicated if the pt does not tolerate or fails to achieve or maintain a remission with imatinib mesylate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

side effects of chemo and radiation

A 
n/v
alopecia
mucositis
anorexia
pancytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what places leukemia pt esp at high risk for infections and serious complications of infections

A

leukopenia, specifically neutropenia combined with a central venous catheter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

children with Noonan syndrome and neurofibromatosis type 1 are at particular risk of developing a specific type of AML known as

A

juvenile myelomonocytic leukemia (JMML)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Leukemia physical exam findings

A

pallor
petechiae
hepatosplenomegaly
adenopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

signs and symptoms of CNS involvement for leukemia

A 
headache
papilledema
retinal hemorrhages 
cranial nerve palsies
coma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

signs/symptoms of hyperleukocytosis

A

fever, bone pain, night sweats, priapism, malaise, splenomegaly causing abd pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

chronic phase of CML is defined as

A

less than 10% blasts in the bone marrow and high levels of myeloid cells and precursors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Accelerated phase of CML is defined as

A

10-19 % blasts in the bone marrow, organomegaly, leukocytosis, and abnormal platelet counts and functioning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what is ordered in leukemia to look at patient’s risk of developing Acute Tumor lysis syndrome (ATLS)

A

metabolic studies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

what is ordered in leukemia to evaluate for the presence of a mediastinal mass and why

A

chest x ray

it can compromise the pediatric pt airway

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what is ordered to assess CNS involvement in leukemia

A

LP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

life threatening complications that need to be identified in leukemia

A 
Sepsis
DIC
ATLS (acute tumor lysis syndrome)
hyperleukocytosis
leukostasis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what should platelet count be prior to LP

A

> 80,000 cells mm3 (generally)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

malignancy arises from lymph node or lymph tissue

A

lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

3rd most common type of pediatric cancer

A

lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

4 sub types of hodgkins lymphoma

A

lymphocyte predominant

nodular sclerosing

mixed cellularity

lymphocyte depleted

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

what virus is believed to have an association with Hodkins lymphoma

A

Ebstein-Barr virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what population is at greater risk for Hodkins lymphoma

A

children with immunodeficiencies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

3 main types of non-Hodkins lymphoma

A

lymphoblastic lymphoma

mature B cell lymphoma

Anaplastic large cell lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

clinical presentation of Hodkins lymphoma

A

lymphadenopathy of 1+ nodes

fatigue

anorexia

weight loss

pruritis

“B” symptoms include
unexplained fevers >38C (100.4)
unexplained weight loss of at least 10% of body weight in 6 mos
Drenching night sweats

Superior vena cava syndrome may result from significant mediastinal adenopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Non-Hodkins lymphoma clinical presentation

A

lymphadenopathy

if abd is involved may have a palpable mass - with n/v, abd distension, hepatosplenomegaly, change in bowel habits, hematochezia

mediastinal involvement may present as superior vena cava syndrome

facial/neck swelling

snoring

dysphagia

chest pain

Systemic symptoms
fatigue
fever
malaise
weight loss
anorexia
night sweats

pancytopenia and related complications if bone marrow is involved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

diagnostic for lymphoma

A

CT

staging - measuring tumor/mass and identifying spread

bone scans and bone marrow aspirate

LP - if CNS symptoms

PET scan

Biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

neoplasm from the sympathetic nervous system, most common extracranial solid tumor of childhood

A

neuroblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

neuroblastomas are most frequently diagnosed during

A

infancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

neuroblastoma presentation

A

primary mass (thoracic, cervical or pelvic mass) -chest abd or pelvis

abdominal mass
constipation
refusing to walk

if they have bone marrow metastases you may see anemia, thrombocytopenia and neutropenia
-with metastatic disease may have bone pain, malaise, fever, racoon eyes (periorbital ecchymosis), “blueberry muffin spots” (metastasis to subcutaneous tissue)

abd distension/tenderness
hepatomegaly
distal edema

tumors of the upper thoracic spine can cause mechanical obstruction and superior vena cava sydrome

horner syndrome may be noted in pt with cervical masses

HTN may be present

bone marrow infiltration may cause fatigue, weakness, increased infections, pallor, bruising

infiltration of periorbital bones may cause proptosis and periorbital ecchymosis

Opsoclonus - myoclonus ataxia (OMA) - 50% seen with this have neuroblastoma - manifests as myoclonic jerking and random eye movements with or without cerebellar ataxia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

diagnostics for neuroblastoma

A 
Labs:
CBC
Complete chemistry 
Serum ferritin
LFT
Urine (catecholamine metabolites - vanillylmandelic acid (VMA) and homovanillic acid (HVA)

bilat bone marrow aspirate and biopsy

elevated (catecholamine metabolites - vanillylmandelic acid (VMA) and homovanillic acid (HVA)
in urine

Imaging: CT, MRI, meta-iodobenzylguanidine (MIBG) scintiscan - nuclear medicine study

Biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

what population for neuroblastoma could present emergently and require immediate evaluation for

A

Patients with orbital, mediastinal, spinal cord, or extensive abdominal disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

most common abdominal tumor of childhood

A

Wilms tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Beckwith-wiedemann syndrome puts pt more at risk for what onc

A

Wilms tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

WAGR syndrome puts pt more at risk for what onc

A

Wilms tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

most common presenting sign of wilms tumor

A

asymptomatic abd mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

clinical presentation for wilms tumor

A

asymptomatic abd mass

HTN (due to renal artery compression and possibly increased renin production

gross hematuria

fever

constipation

Rare: hypotension, anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

diagnostic for wilms tumor

A

ON physical exam - large palpable flank mass - be gentle not to disrupt the capsule

evaluate for features of congenital predisposition syndrome (aniridia, hemihypertrophy, macroglossia, urogenital malformations)

US

CT abd and chest

Labs - CBC, chemistry (renal function tests, electrolytes, calcium)

Urinalysis

Coagulation (preoperative)

Testing for 1P and 16q deletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

inflammation or destruction of mucosal cells of the oral cavity and throughout the GI tract

A

mucositis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

clinical presentation of mucositis

A

mouth sores and/or ulcerations from esophagus to rectum

severe pain
anorexia
dehydration
bleeding 
infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

how to manage mucositis

A

diligent oral care (decrease severity)

mouth rinses such as sodium chloride (prevent infections)

Pain management - lidocaine mouth rinses, opioids - may require PCA

Usually resolves with return of bone marrow activity or with engraftment following HSCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

when is hyperleukocytosis typically seen

A

new-onset leukemic patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

what oncologic process are the complications for hyperleukocytosis more significant

A

AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

what is the most common complication of hyperleukocytosis

A

stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q 
fever
lethargy
mental status changes
headache
seizure
stroke
coma
dyspnea
tachypnea
hypoxemia
resp failure
acidosis
cor pulmonale
hemorrhage
DIC
Renal failure
A

clinical presentation for hyperleukocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

diagnostics for hyperleukocytosis

A

WBC >100,000
CXR - diffuse interstitial infiltrates
Head CT - high risk of intracranial hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

management of hyperleukocytosis

A

goal is rapid reduction in number of circulating WBCs - exchange transfusion, leukopheresis, cytotoxic therapy initiation

Hydration - 2-4 x maintenance, avoid diuretics

Allopurinol, rasburicase

Serial electrolytes, BUN, uric acid

Transfuse platelets, treat coagulopathy, avoid PRBCs (goal <10g/dL)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

claw sign on imaging

A

(the appearance of the mass wrapping around the kidney on imaging) - Wilms tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

what happens in sepsis in oncology

A

vasodilation
myocardial dysfunction leading to multiorgan system dysfunction and third spacing of fluid as a result of capillary leak.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

what happens in superior vena cava syndrome

A

SVC is a low pressure vessel that can become easily compressed

this can be from compression or thrombosis formation

results in increased venous pressure and decreased cardiac output

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

clinical presentation of superior vena cava syndrome

A

cough
fever
dyspnea
chest pain

edema/engorgement of the face, neck and upper torso, dilation of the superficial veins, cyanosis or plethora, stridor, dyspnea, anxiety

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

diagnostic eval for superior vena cava syndrome

A

chest radiograph
chest CT
US

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

management for superior vena cava syndrome

A

manage airway by relief of obstructive process or underlying cause

emergent mgmt - oxygen, noninvasive ventilation, heliox therapy, imaging, minimizing pressures by addressing source of compression

elevate HOB may provide some relief

diuretic therapy in some cases

steroids in some cases for malignancy or tumor

if etiology is clot, anticoagulation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

oncological emergency associated with initial chemotherapy for leukemias, lymphomas and other tumors with high growth rates. Can affect neurological, pulmonary, cardiac and renal function

A

Tumor Lysis syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

what is released massively in tumor lysis syndrome

A

large amounts of potassium, phosphate and uric acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

when does tumor lysis syndrome most commonly occur

A

within 6-48 hours and up to 7 days after initiation of chemotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

symptoms of tumor lysis syndrome

A 
anorexia
cardiac arrhythmias
heart failure
edema
fluid overload
hematuria
lethargy
muscle cramps
n/v/d
oliguria
seizures
syncope
muscle cramps
tetany
sudden death

can be asymptomatic but with
Hyperkalemia
hyperphosphatemia
Hyperuricemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

diagnostic eval for tumor lysis syndrome

A

CMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

management of tumor lysis syndrome

A

Prevention is key
Aggressive hydration - 2-3 L/m2/day to enhance uric acid and phosphate excretion, diuretics

urine specific gravity and pH

strict I and O

Allopurinol, Rasburicase

Serial electrolyte monitoring

Dialysis if oliguria, azotemia, dangerously high potassium or phosphorus levels or refractory hyperuricemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

A potentially life threatening disorder defined by the triad of neutropenia, abdominal pain and fever

A

Typhlitis (neutropenic enterocolitis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

what is the problem in Typhlitis

A

most commonly inflammation of the cecum, but may also be in the ascending and proximal colon. Inflammation can progress rapidly to gangrene or bowel perforation

mucosal wall thickening, ischemia, ulceration, hemorrhage and possible perforation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

clinical presentation for Typhlitis

A

Fever and neutropenia (ANC <500 cells/uL)

Elevated CRP

Abd pain (RLQ) with distended abd

Peritoneal signs (guarding, abd wall rigidity, rebound tenderness)

n/v/d

diarrhea may be bloody

poor appetite

range in bowel sounds from high pitched to diminished or absent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

diagnostic for Typhlitis

A

CT with PO and IV contrast (preferred) - may demonstrate colonic wall thickening, mesenteric fat stranding secondary to inflammation, submucosal bowel wall edema, paralytic ileus, pneumatosis)

plain abd film - may show RL soft tissue mass, paralytic ileus - “thumbprinting” due to bowel wall edema, bowel edema or intraluminal gas

US - nonspecific findings such as thickening of bowel wall, intraluminal fluid or pericecal fluid

labs - CBC, coags, blood and stool cultures, chemistries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

management for Typhlitis

A

broad spectrum abx with both gram pos, gram neg and aerobic coverage

bowel rest 
abd decompression (ng tube)

pain management

Granulocyte colony stimulating factor

IV fluids for hydration, parenteral nutrition

if pneumatosis, pneumoperitoneum or pericolic fluid are noted on studies - urgent surgical eval

persistent GI bleeding - surgical eval

Avoid or caution with anticholinergics, antidiarrheal and narcotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

what measures oxygen carrying potential

A

Hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

what measures mass of RBC

A

hematocrit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

Life span of RBC

A

approx 120 days (varies with age and gender)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

What shows us RBC production

A

Reticulocyte count (measures bone marrow response to anemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

what part of blood are mediators of inflammation

A

WBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

medical term for decreased WBC

A

leukopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

medical term for elevated WBC

A

leukocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

what granulocyte is primary infectious response

A

segmented neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

what granulocyte is a mature neutrophil

A

Polys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

immature neutrophils

A

bands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

granulocyte seen in allergic response

A

Eosinophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

agranulocytes involved in immune response

A

T cell and B cell lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
92
Q

Agranulocyte produced by bone marrow, process foreign antigens through phagocytosis

A

Monocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
93
Q

indicator of pt ability to fight infection? what number is concerning

A

ANC (absolute neutrophil count) - <500 is concerning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
94
Q

formula for ANC

A

Total #WBCs x (%polys+%bands)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
95
Q

life span of platelets

A

5-9 days. Transfused platelets have a shorter life span

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
96
Q

average concentration of hemoglobin of a given volume of PRBC,

A

MCHC - mean corpuscular hemoglobin concentration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
97
Q

MCHC - mean corpuscular hemoglobin concentration formula

A

dividing the hemoglobin by the hematocrit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
98
Q

average quantity of hemoglobin

A

MCH (mean corpuscular hemoglobin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
99
Q

index of the size of RBC

A

MCV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
100
Q

abnormal WBCs

A

blasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
101
Q

what lab study indicates the time for plasma to clot in the presence of thromboplastin (extrinsic pathway), altered in vit k deficiency

A

Prothrombin time (PT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
102
Q

what lab study measures conversion of fibrinogen to fibrin - last step in the clotting process

A

Thrombin Time (TT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
103
Q

activated partial thromboplastin time is most accurate if results are obtained within ___ hours of specimen collection

A

4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
104
Q

iron is stored in tissue as

A

ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
105
Q

what lab shows percentage of transferrin saturated with iron showing the capacity of blood to transport iron

A

Transferrin saturation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
106
Q

what lab measures reserve capacity of transferrin

A

Total iron binding capacity (TIBC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
107
Q

isoenzyme used as a marker of tissue breakdown or damage and hemolysis

A

LDH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
108
Q

product of metabolic breakdown of purine nucleotides. Some cancers will cause this to elevate which can contribute to renal failure

A

Uric acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
109
Q

Advantages of PET scan

A

ability to up and down stage disease and evaluate response to chemotherapy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
110
Q

disadvantages of PET scan

A

results are not specific to a cancer diagnosis.

significant radiation

lower sensitivity in brain and lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
111
Q

A PET scan provides information about

A

metabolic activity and the proliferative potential of a residual tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
112
Q

clinical indications of PET

A

baseline staging
disease response
f/u for disease surveillance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
113
Q

fuses metabolic activity with anatomical structures

A

PET-CT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
114
Q

what is best at detecting bone marrow and bony sites of disease in rhabdomyosarcoma?

PET CT or Bone Scan

A

PET-CT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
115
Q

Pediatric challenges in PET

A

need for sedation

pregnancy screening

potential artifact caused by normal physiologic uptake

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
116
Q

what is critical to have for a PET scan to ensure accurate imaging measurements

A

Correct weight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
117
Q

in bone marrow aspiration immature cells are ____ in hyperplastic conditions such as leukemia

A

increased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
118
Q

What other condition may show atypical cells increased on bone marrow aspiration

A

vit B12 or folate deficiency

myelodysplastic syndromes

Drug toxins

infectious problems

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
119
Q

what is bone marrow aspiration used for

A

staging and eval for abnormal peripheral blood findings such as inherited bone marrow failure syndromes, microbiologic cultures in fevers of unknown origin and for f/u of minimal residual disease (MRD) after chemo or BMT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
120
Q

causes of anemia

A

autoimmune hemolytic anemia

hemoglobinopathies

membrane and enzyme defects

drug associated hemolytic anemias

Disseminated intravascular coagulation (DIC)

Hemolytic uremic syndrome (HUS)

Excessive blood loss (hemorrhage)

Deficient red cell production (ineffective hematopoiesis) -> nonnutritional disorders of hemoglobin synthesis, thalassemia syndromes, lead poisoning, iron deficiency, chronic inflammatory diseases, chronic infections, chronic renal disease, hyper/hypothyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
121
Q

transient erythroblastopenia of childhood

A

transient red cell aplasia -> typically follows viral illness with anemia in the range of 6-8mg/dL but can be lower and reticulocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
122
Q

symptoms of anemia

A 
weakness
fatigue
confusion
palpitations
pallor
tachycardia
flow murmur
diminished peripheral pulses
jaundice
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
123
Q

diagnostic anemia

A

decrease in HCT and HGB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
124
Q

decrease in HCT and HGB

increased retic count

low MCV

A

hemoglobinopathies (such as thalassemia syndromes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
125
Q

decrease in HCT and HGB

increased retic count

normal MCV

A

membrane enzyme or immune disorders

microangiopathic anemias

DIC

infection-induced hemolysis

chronic blood loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
126
Q

decrease in HCT and HGB

low, normal or slightly elevated retic

low MCV

think…

A

iron def anemia

lead tox

thalassemia trait

sideroblastic anemia

anemia of chronic disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
127
Q

decreased in HCT and HGB

low normal or slightly elevated retics

high MCV

think…

A

congenital hypoplastic or aplastic anemia

acquired hypoplastic or aplastic anemia (malignancies)

aplastic crisis with underlying hemolytic anemia (HbSS)

megoblastic anemia (folate or B12 def)

immune disorders

Hypersplenism

anemia of chronic disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
128
Q

anemia with hypovolemic shock, how to treat?

A

volume expansion with packed RBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
129
Q

life threatening disease of bone marrow failure resulting in decreased production of hematopoietic stem cells that results in peripheral pancytopenia and bone marrow aplasia

A

aplastic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
130
Q

clinical presentation of aplastic anemia

A

History:
mucosal/gingival bleeding

headaches

fatigue

easy bruising

rash

fever

mucosal ulcerations

recurrent viral infections

symptoms: (depends on level of pancytopenia)
pallor
tachycardia
petechial rash
purpura
ecchymoses
jaundice
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
131
Q

diagnostic for aplastic anemia

A

decrease in HGB, WBC, platelet count

reduction in or absence of the absolute number of reticulocytes

peripheral blood smear - no abnormal cells

reduction or absence of hematopoietic elements from bone marrow aspirate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
132
Q

management of aplastic anemia

A

transfusion of RBC and platelets

ABX

Bone marrow transplant

immunosuppressive therapy if unable to receive a BMT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
133
Q

A rare congenital hypoplastic anemia resulting in constitutional bone marrow failure

A

Diamond-Blackfan Anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
134
Q

mutation for Diamond Blackfan is on Chromosome

A

19

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
135
Q

clinical presentation of Diamond Blackfan anemia

A 
symptoms:
pallor
fatigue
irritability
syncope
dyspnea during feeding
physical exam
irregular heartbeat
hypotonia
short stature
evidence of FTT
associated physical defects
Craniofacial  such as cleft lip/palate
hands (thumb abnormality)
upper limbs
cardiac
genitourinary
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
136
Q

diagnostics for Diamond Blackfan anemia

A

CBC, adenosine deaminase activity and bone marrrwo biopsy

Profound macrocytic anemia

WBC and platelet gen normal

Reticulocytopenia

increased % of HGB F for age

Elevated erythrocyte adenosine deaminase activity

Decreased or absent erythroid precursors in bone marrow aspirate

Mutation in RPS19

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
137
Q

Management for Diamond Blackfan Anemia

A

Corticosteroids (glucocorticoids improve erythropoiesis and 60-70% achieve transfusion independence)

frequent blood transfusion

BMT in some cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
138
Q

what teams do you need involved for Diamond Blackfan Anemia pt

A

Hematology

BMT

Endocrinology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
139
Q

AKA defibrination syndrome

A

DIC (Disseminated intravascular coagulation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
140
Q

AKA consumptive coagulopathy

A

DIC (Disseminated intravascular coagulation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
141
Q

life threatening complication of systemic or localized tissue injury causing a disturbance of the normal coagulation cascade that results in uncontrolled intravascular coagulation coupled with the consumption of coagulation factors and platelets which trigger concurrent thrombosis and hemorrhage

A

DIC (Disseminated intravascular coagulation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
142
Q

most common cause of DIC

A

infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
143
Q

what type of sepsis is most commonly associated with DIC

A

gram neg sepsis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
144
Q

causes of DIC

A

infection - Sepsis, viral processes, fungal infection, severe pancreatitis

Trauma - penetrating brain injury, burns, multiple trauma

hematologic malignancies - Hemolytic processes

Acute resp distress syndrome

Obstetrical complication

necrotizing enterocolitis

Extra corporeal membrane oxygenation

Graft vs host disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
145
Q

what is DIC

A

concurrent acceleration of the clotting cascade and the fibrinolytic system causing simultaneous hemorrhage and microvascular clotting

consumption of platelets - thrombocytopenia

intravascular thrombosis, purpura, petechiae, end organ ischemia and infarction

hemorrhage secondary to:
-depletion of coagulation factors and platelets
-prolonged PT
Prolonged activated PTT

multiple organ system failure s/t microinfarction, tissue ischemia and necrosis

shock

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
146
Q

headache

altered LOC

Bleeding

disproportionate bruising

A

DIC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
147
Q

findings in DIC

A

diffuse bleeding

petechiae

ecchymosis

purpura

hematoma

gingival bleeding and epistaxis

hematuria

hematemesis

melena

intrahepatic hemorrhage

s/s shock

thrombosis

cool mottled skin

poor perfussion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
148
Q

diagnostic of DIC

A

prolonged PT
Prolonged activated PTT
increased INR

decreased fibrinogen and platelet count

schistocytes (fragmented RBCs) on CBC smear

Elevated fibrin split product

elevated d dimer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
149
Q

Management of DIC

A

supportive therapy

monitor vitals, CVP, oxygen sat

administer oxygen PRN

ABX - for infectious etiology

correct acidosis and shock

administer vit k as indicated

Coags
CBC
acid base balance

Blood product admin and replacement

cryoprecipitate (provides fibrinogen, factor VIII and von willebrand factor

consider anticoagulation (heparin) - contraversial

Antithrombin III - an A2 globulin that inhibits coagulation

consider Aprotinin - slows fibrinolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
150
Q

a disease of the microcirculation is characterized by hemolytic anemia, thrombocytopenia and acute renal failure. occurs most frequently in children younger than 4

A

Hemolytic uremic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
151
Q

most common cause of ARF

A

Hemolytic uremic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
152
Q

etiology of Hemolytic uremic syndrome

A

contamination of water, meats, fruits and vegetables with infectious bacteria

E.Coli 0157:H7 is most common of post-diarrheal (D+) HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
153
Q

Peak season for Hemolytic uremic syndrome

A

summer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
154
Q

most common etiology of post-diarrheal (D+) HUS

A

E coli 0157:H7

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
155
Q

When do you see D+ HUS

A

previously healthy children who have had recent gastroenteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
156
Q

Bacteria that cause HUS

A

E. Coli 0157:H7 infection (Shiga toxin; most common cause), shigella dysenteriae, citrobacter freundii and other subtypes of E coli (Also shiga todxdin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
157
Q

what is worse D-HUS or D+ HUS

A

D-HUS is atypical and more severe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
158
Q

what type of HUS may have a familial link and may also begin in the neonatal period

A

D- HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
159
Q

causative factors of HUS

A

Inherited factor H deficiency -> inhibits complement activation

membrane cofactor protein mutations

streptococcus pneumoniae infection

meds - cyclosporine and Tacrolimus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
160
Q

incubation period of D+HUS

A

3-5 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
161
Q

abdominal pain

watery, non-bloody diarrhea

fever

weakness

lethargy

iritability

A

Symptoms of D+ HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
162
Q

Progression to hemorrhagic colitis occurs __ to __ days after onset of diarrhea for D+HUS

A

5-7

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
163
Q

clinical findings in D+ HUS

A 
pallor 
petechiae
ecchymoses
hematuria
oliguria
azotemia
HTN

may progress to anuria, hepatomegaly
splenomegaly
hematemesis
edema

tremor and seizures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
164
Q

HUS diagnosis

A

pt history

microangiopathic hemolytic anemia

thrombocytopenia

ARF

Retics

Abnormal RBC morphology

Schistocytes, burr, and helmet cells on smear; fragmented erythrocytes

Anemia - decreased plasma haptoglobin

Thrombocytopenia

Leukocytosis is common

Coags often normal

Stool cultures often positive for E.Coli 0157:H7 or other toxin producing bacteria (not always detected)

Serum ELISA testing should be done at diagnosis and repeated 2 weeks later to determine presence of antibodies to Shiga toxin E.Coli serotypes

Elevated BUN, serum creatinine, bilirubin, potassium

Coombs neg

microscopic hematuria, proteinuria and casts on urinalysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
165
Q

Management of D+HUS

A

supportive therapy

No ABX - may stimulate bacteria to release more toxins

Dialysis

Correct electrolyte imbalances, azotemia, manage fluid overload

nutrition (renal protective diet)

correct anemia - most require PRBC transfusion

Control HTN - oral Calcium channel blocker - nifedipine)
IV Calcium channel blocker ie) nicardipine or nitroprusside

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
166
Q

long term follow up care of D+HUS and D- HUS

A

monitor BP and urinalysis

proteinuria, decreased GFR, HTN may recur up to 1 yr later

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
167
Q

D-HUS treatment

A

Plasmapheresis - consider for pt with factor H deficiency
may limit renal involvement temporarily but does not prevent progression to ESRD and has not been shown to prevent recurrence of D-HUS

kidney transplantation if recurrence persists

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
168
Q

Children with cervical masses can exhibit symptoms of ____ syndrome, including ____

A

Horner Syndrome

unilateral ptosis
myosis
anhidrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
169
Q

most common sites for metastatic disease in neuroblastoma are

A 
lymph nodes
liver
bone
bone marrow
skin

rare for lung or brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
170
Q

Cairo and Bishop definitions of tumor lysis syndrome

A

lab tumor lysis syndrome:
requires 2 or more of the following criteria within a 24 hour time period occurs between 3 days before and 7 days after initiating chemo

uric acid >8mg/dL or 25% increase from baseline

Potassium > 6.0mEq/L or 25% increase from baseline

Phosphorous > or equal 6.5mg/dL in children or 25% increase from baseline

Calcium < or equal to 7.0mg/dL or 25% increase form baseline

Clinical tumor lysis syndrome

requires Laboratory tumor lysis syndrome plus one of the following
Cardiac arrhythmia or death
seizure
Creatinine >1.5 times the upper limit of normal for age and sex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
171
Q

chvostek sign

A

indicates hypocalcemia

tap on the facial nerve located anterior to the ear lobe and below the zygomatic arch.

A positive sign is twitching or contraction of the facial muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
172
Q

Trousseau Sign

A

hypocalcemia

inflate a bp cuff above systolic pressure on the upper arm for several minutes.

A positive sign is a carpopedal spasm - wrist/metacarpophalangeal/thumb flexion and hyperextension of the fingers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
173
Q

what does allopurinol do

A

blocks the conversion of hypoxanthine and xanthine to uric acid - prevents future uric acid production but does not effect what is already there

174
Q

Allopurinol dosing

A

50-100 mg/m2/dose q 8 hours (max 300 mg/m2/day)

or 10mg/kg/day divided q 8 hours (max 800mg/day)

175
Q

what does Rasburicase do

A

metabolizes uric acid to water soluble allantoin

176
Q

dosing for Rasburicase

A

given as a 30 min infusion to high risk children and pt with symptomatic TLS

0.2mg/kg/day

can be used for 3-7 days

177
Q

contraindication for Rasburicase

A

Pt with G6PD deficiency bc hydrogen peroxide (a byproduct of uric acid metabolism) can trigger hemolysis

178
Q

important to note for Rasburicase (lab draws)

A

Rasburicase can remain active out of the body….when you are doing a serum uric acid…keep blood on ice until processed

179
Q

treatment for hyperkalemia in the setting of tumor lysis syndrome

A

avoid external sources of potassium

monitor for life threatening arrhythmias

verify level of serum potassium on ECG ( peaked T waves, P wave widening/flattening, PR prolongation)

repeat lab to make sure it was not due to hemolysis

if pt is asymptomatic - potassium binding resins can be given such as Kayexalate

diuretics can be used to increase kidney excretion of potassium

if life threatening arrhythmia - IV calcium chloride or gluconate to stabilize myocardium
or insulin (increase cell uptake of potassium)
and glucose (treats resultant hypoglycemia)

nebulized albuterol - promotes potassium to move intracellularly)

180
Q

normal potassium level

A

3.4-5.3 mEq/L

181
Q

treatment for hyperphosphatemia in the setting of TLS

A

minimizing dietary phosphorous

IV hydration and phosphate binding agents (sevelamer carbonate)

Aluminum hydroxide prevents phosphorus absorption in the GI tract

dialysis can be indicated for severe, symptomatic hyperphosphatemia

182
Q

dosing for Aluminum hydroxide

A

prevents phosphorous absorption in GI tract

orally 50-150 mg/kg/dose q 6 hours

183
Q

hypocalcemia treatment in the setting of TLS

A

if asymptomatic, no therapy is indicated bc of risk of calcium phosphate precipitation and subsequent potential for AKI

symptomatic ->calcium chloride or gluconate

dialysis is indicated for refractory or life threatening electrolyte derangements
(peritoneal not recommended for TLS due to inadequate clearance of uric acid with this modality)

184
Q

Benadmustine (traditional cytotoxic agent) when administered concurrently with allopurinol

A

can result in serious complications such as SJS and Toxic Epidermal Necrolysis

185
Q

most pediatric oncologists define survivorship when the patient

A

is 5 years post diagnosis and min of 2 yrs free of cancer treatment

186
Q

phosphorous normal range

A

4.5-6.5mg/dL

187
Q

normal calcium range

A

8.8-10.8mg/dL

188
Q

normal uric acid

A

2-6.2mg/dL

189
Q

result of decreased or absent production of one or more Hb B-globulin chains, which results in a relative excess number of A globin chains and ineffective erythropoiesis, chronic hemolytic anemia and iron overload

A

B-Thalassemia

190
Q

what are the 3 phenotypes of B-Thalassemia

A

Thalassemia minor

B Thalassemia intermedia

Thalassemia major

191
Q

what B Thalassemia phenotype:

patients are clinically asymptomatic but have specific hematologic findings

A

Thalassemia minor

192
Q

what B thalassemia phenotype?

symptoms can range in severity

A

B-Thalassemia intermedia

193
Q

what B thalassemia phenotype?

severe transfusion dependent anemia

A

Thalassemia major

194
Q

A decrease in Hb production causes hypochromia and microcytosis. Grossly abnormal RBC shapes may also be noted, including the presence of target cells, tear-drop cells (dacrocytes), fragmented forms, echinocytes and presence of RBC inclusions in the peripheral blood

A

B thalassemia

195
Q

one of the most common autosomal recessive disorders in the world

High prevalence among individuals of ___, ____, and ____ descent

A

B Thalassemia

Mediterranean
African
Southeast asian

196
Q

which B thalassemia phenotype is usually diagnosed during infancy

A

B-thalassemia major

197
Q

presentation of pt with B thalassemia

A 
FTT
pallor
irritability
diarrhea
abdominal enlargement (hepatosplenomegaly)
jaundice
198
Q

diagnosis of B thalassemia

A

lab data to confirm

199
Q

B thalassemia major is also called

A

Cooley Anemia

200
Q

thalassemia that occurs when a gene or genes r/t the A globin protein are missing or changed (mutated)

A

A-Thalassemia major

201
Q

B Thalassemia is very common in….

A

Italy and Greece

Mediterranean
middle eastern
Asian

202
Q

what chromosome is involved in B thalassemia

A

11

203
Q

B Thalassemia

Heterozygous is which phenotype

A

B thalassemia minor

204
Q

B thalassemia

Homozygous is which phenotype

A

can be

B thalassemia intermedia or major

205
Q

B thalassemia is what type of anemia

A

Microcytic anemia

206
Q

increase in microcytic RBCs
low hgb, low hct
iron normal
RBC are a little high

Hb electrophoresis will be abnormal showing
increase in HbA2 and increase in HbF

A

B thalassemia minor

207
Q

in B thalassemia major where is the problem?

A

no B globin production
alpha globin will precipitate will form hemotetramers - in the marrow they will die (ineffective erythropoiesis)
macrophages in the spleen will kill them ->hemolytic anemia-kidney will produce EPO ->lead to erythroid hyperplasia - medullary erythropoiesis and extramedullary hematopoiesis ->hepatosplenomegaly

medullary cavity in the skull will enlarge (on x ray it will have a hair-on end appearance also called crew cut skull)

maxilla will enlarge - chipmunk facies

we will give them blood - they will become transfusion dependent

leads to iron overload

also leads to unconjugated hyperbili (jaundice)

208
Q

B-thalassemia intermedia has a co-inheritance with

A

A thal trait

209
Q

in B thalassemia intermedia you have minor qualitative defects in

A

B globin

210
Q

typical features for Thalassemia

A

chipmunk facies with prominent frontal bossing

delayed pneumatization of the sinuses

marked overgrowth of maxillae

bones and ribs become “box like” premature fusion of epiphyses and thinning of the cortex of the bone

211
Q

findings of thalassemia

A 
hepatomegaly
splenomegaly
enlarged kidneys with dilated renal tubules
dark urine
cardiac abnormalities
delayed sexual development
212
Q

Hypochromic, microcytic anemia with decreased MCV, basophilic stippling and presence of Hgb A

may have hyperuricemia

A

Thalassemia

213
Q

primary treatment for thalassemia

A

blood transfusion and folate replacement

BMT from a matched sibling donor is the best chance for cure

214
Q

complications for thalassemia

A

iron overload from transfusions

congestive heart failure

early death

215
Q

A thalassemia trait

A

one locus deletion (AA, A-)

pt is asymptomatic

216
Q

A thalassemia minor

A

2 loci deleted (can be A-, A-) or (AA, –)

pt symptomatic with low MCV

217
Q

A thalassemia intermedia

A

3 loci deleted (A, -, –)

moderate to severe hemolytic anemia

218
Q

4 loci deleted
no effective erythropoiesis

hydrops fetalis (baby dies en utero)

A

A thalassemia major

219
Q

most accurate test to diagnose a thalassemia

A

genetic studies

220
Q

A thalassemia intermedia needs what?

A

blood transfusions

221
Q

According to Virchow’s triad concept, thrombosis is caused by a disruption in this triad:

A

changes in vessel wall

alteration in blood flow

increased coagulability of the blood

222
Q

acquired risk factors for thrombosis

A

obesity

smoking

cancer

medications including L-asparginase and estrogen based hormones

increased risk in pregnancy r/t elevations of procoagulant factors and relative deficiency of anticoagulant factors

trauma - can lead to vessel damage and compounded by venous stasis secondary to bed rest during recovery

223
Q

antiphospholipid syndrome

A

presence of antiphospholipid antibodies on 2 occasions separated by 12 weeks and a thrombotic event

significant increased risk for recurrent thrombosis

consider indefinite anticoagulation therapy to prevent repeat thrombotic events

224
Q

inherited risk factor for thrombosis

A

Factor V Leiden mutation is the most common inherited thrombophilia

Prothrombin gene mutation (more common in Caucasians)

protein C def
Protein S def is rare

Elevated factor VIII - an acute phase reactant is increased by stresses to system (trauma or surgery)

225
Q

Venous thrombosis in neck vessels can result in

A

superior vena cava syndrome

226
Q

what is a cerebral sinus venous thrombosis

s/s?

A

thrombus deep veins of the head

persistent headache
blurred vision
neurologic signs
seizures

227
Q

what type of thrombosis is associated with nephrotic syndrome and what symptom?

A

renal vein thrombosis

generalized edema

228
Q

Portal vein thrombosis causes

A

splenomegaly with thrombocytopenia and anemia. Esophageal varices can result

229
Q

vascular anomaly in the pelvis in which the R common iliac artery compresses the L common iliac vein - predisposing them to L Lower extremity DVT

A

May-Thurner Syndrome

230
Q

upper extremity DVT that results from venous thoracic outlet syndrome - which the axillary and subclavian veins are compressed at their exit site into the chest.

Thrombosis triggered by repetitive overhead arm motion (baseball pitching) that exacerbates the compression

A

Paget-Schroetter syndrome

231
Q

what electrolyte abnormality increases risk for thombi

A

hypercalcemia

232
Q

the single greatest risk factor for arterial or venous thrombosis in infants and children is

A

the presence of a CVC

233
Q

what pediatric population is most at risk for developing a DVT or PE

A

infant less than 1 year and adolescents

234
Q

in infants less than one year of age, the majority of non CVC related thrombotic cases are from

A

RVT (renal vein thrombosis)

235
Q

most commonly used diagnostic for DVT is

A

doppler US

elevated d-dimer (a neg d-dimer excludes the diagnosis of a thrombus)
-other things can cause an elevated d-dimer so this test is sensitive but not specific

236
Q

diagnostic for PE

A 
CT angio (Computed tomography angiography)
is the study of choice

Ventilation -perfusion lung scan (VQ scan) are still occasionally used

237
Q

anticoagulant therapy in pt with arterial disease

A

unfractionated heparin or LMWH plus aspirin or a GpIIIb/IIa inhibitor (clopidogrel)

Unfractionated heparin is given as a continuous infusion (initial bolus at 50u/kg, maintenance dose is 10-15u/kg/hr for 7-10 days titrated to goal aPTT of 60-90seconds)

as pt is weaned off heparin, pt should simultaneously be started on warfarin…
loading dose 0.2mg/kg with max of 10mg

dose is titrated with goal of maintaining the INR 2-3

usual maintenance dose is 0.1mg/kg/day by mouth with goal INR of 2-3.

continue therapy for 4-6 weeks to prevent recurrence of thromboses and until the risk factors have resolved or stabilized, imaging confirms resolution, and D-dimers have normalized.

if using LMWH (becoming standard bc eliminates need for frequent monitoring)
dose is 1.5mg/kg SQ q 12 hours in infants 2 mos and younger, 1mg/kg Sq q 12 hours in children greater than 2 mos

for prophylaxis
0.75mg/kg and 0.5mg/kg once a day

238
Q

inheritance pattern for Von willebrand disease (vWD)

A

autosomal dominant

239
Q

a plasma protein composed of multimers that cause platelet adhesion to initiate a “platelet plug” and act as a carrier protein for factor VIII.

A

vWF (von Willebrand factor)

240
Q

which vWF is a qualitative protein dysfunction of circulating vWF

A

vWD type 2 (further divided into 2A, 2B, 2M, 2N)

241
Q

which vWf are caused by an insufficient quantity of vWF

A

types 1 and 3

242
Q

what is the most common inherited bleeding disorder

A

vWD

243
Q

what disease process reduces the circulating level of vWF in plasma

A

hypothyroidism

244
Q

what factors can increase circulating level of vWF in plasma

A 
contraceptives
exercise
stress
inflammation
pregnancy
245
Q

what history should put vWF in your mind

A

history of easy bruising
frequent epistaxis
heavy menstrual bleeding
or bleeding after a surgical/dental procedure

246
Q

diagnosis of Von Willebrand

A

The Von Willebrand protein levels may vary so repeat lab test may be needed for diagnosis

CBC - normal, microcytic anemia, thrombocytopenia

aPTT - normal or prolonged

bleeding time - normal to slightly prolonged

ristocetin cofactor (RCo) - low

vWF antigen - low

Factor VIII level - low or normal

family history
bleeding symptoms
labs

Normal RCo and vWF antigen normally range from 50-200 IU/dL.

247
Q

If the RCo activity and the vWF antigen differ by more than 30% there is evidence of

A

protein dysfunction (type 2 vWD)

248
Q

what lab measures vWF function

A

RCo activity

249
Q

what lab measures vWF quantity

A

vWF antigen

250
Q

first line treatment for mild bleeding episodes in pt with type 1 vWD

A

IV or IN DDAVP (desmopressin acetate - stimulates release of VWF and factor VIII

251
Q

DDAVP side effect

A

fluid retention similar to SIADH - pay attention to urine output and water intake

if unable to take DDAVP - replacement with vWF concentrate (Humate-P) should be considered

252
Q

in vWD, RCo and Factor VIII goal for minor surgical procedures

A

> =30

maintain for 1-5 days post procedure

253
Q

In vWD, RCo and Factor VIII goal for more invasive procedures, aim for

A

> =50

maintain for 1-5 days post procedure

254
Q

In vWD, RCo and Factor VIII goal for neurosurgery, abdominal surgery or other major surgery, aim for

A

> =100

maintain for 1-5 days post procedure

255
Q

for vWD what is the most common presenting symptom in women

treat>=?

A

menorrhagia

estrogen containing contraceptives

DDAVP and the administration of antifibrinolytic agents during the first few days of the menstrual cycle have both shown to decrease menstrual blood flow
(Tranexamic acid - Lysteda - approved for menorrhagia)

256
Q

how are antifibrinolytics used in vWD

A

managing recurrent bleeding in vWD but do not stop active bleeding - slows the breakdown of clots to prevent re-bleeding

Aminocaproic acid (Amicar) - contraindicated in hematuria

257
Q

all pt diagnosed with vWD should have what done for baseline levels

A

serum iron and ferritin levels to assess for iron deficiency

258
Q

what type of vWD is the most severe, requiring a more comprehensive maintenance plan

A

type 3

259
Q

Any pt who received clotting factor concentrates prior to when should have additional screening

A

1985

Hep B and C and HIV

260
Q

what type of blood product is extracted from whole blood and contains coagulation, fibrolytic and complement systems that assist in the restoration of coagulation disorders such as DIC

A

Fresh Frozen Plasma (FFP)

261
Q

what blood product is responsible for hemostasis with resulting thrombus formation.

A

platelets

262
Q

what blood product is obtained by centrifuging plasma and removing the precipitate. used to replace low fibrinogen levels and when certain factors are not available for treatment of coagulation disorders

A

Cryoprecipitate

263
Q

blood product used to increased oxygen circulation and delivery

A

RBCs

264
Q

threshold for transfusion in children

A

Hb 7gm/dl

265
Q

at what Hb point is the “do not go past, must transfuse number?

A

Hb <5 gm/dL because the risk of morbidity and mortality is increased

266
Q

what is the recommended transfusion volume in children for PRBCs

A

10-15 ml/kg

which will raise the Hb concentration 2-3 gm/dL or HCT by 6-9%

267
Q

All RBC transfusions should be completed within ___ hours after removal from a controlled temp storage

A

4

268
Q

in clinically stable children what is the platelet threshold

A

10,000 uL

269
Q

in a pt who is actively bleeding or expected to receive invasive procedures, a platelet transfusion may be given to support hemostasis with a target of at least _____ uL for minor procedures or _____uL for CNS bleeding

A

50,000uL

100,000uL

270
Q

platelet transfusions are not indicated in a pt with what? unless what

A

platelet consumption disorders such as thrombotic thrombocytopenia purpura (TTP), Heparin induced thrombocytopenia (HIT) or Hemolytic Uremic syndrome (HUS) but should be used in severe life threatening hemorrhage

271
Q

how are platelets stored

A

at room temp

272
Q

Platelets should be given at

A

1unit per every 10kg weight

10-15ml/kg at the rate of 10-20ml/kg/hr

expect a 50,000 uL rise

273
Q

FFP should be given at

A

10-15ml/kg/transfusion

274
Q

Cryoprecipitate should be given at

A

1 unit per every 10kg pt weight

expect rise of 60-100mg/dL

275
Q

To calculate PRBC transfusion dose

A

what is the target or goal for the HCT?

volume or PREC required
(mLs) = HCT (d) -HCT (i) x TBV
all divided by HCT target

HCT (d) is desired HCT
HCT (i) is initial HCT
TBV is (infant-100mL/kg, child - 80ml/kg, adult is 65ml/kg)

276
Q

____ is possible when children drink large volumes of milk

A

anemia

277
Q

Stored PRBC’s lack _____ content, so children who receive any type of multiple transfusion require?

A

calcium

additional calcium

278
Q

PRBCs stored > __ days are associated with higher ___ concentrations

A

5 days

potassium

279
Q

Based on illness severity, infants < ___ months of age will not require cross matching

A

4 mos

in critical situations it can be used for any child

280
Q

Estimated circulating blood volume is based on ?

A

average calculation of 80ml/kg

adults are 70mL/kg

281
Q

Massive transfusion complications include

A

thrombocytopenia
hypocalcemia
coagulation factor depletion
hyperkalemia
increased levels of lactic acid (acid base disorders)
hypothermia
altered or decreased oxygen delivery to tissues

282
Q

what is the shelf life of platelets

A

7 days

283
Q

FFP contains clotting factors

A

II, V, VIII, IX, X and XI, fibrinogen, albumin, protein C, Protein S, antithrombin, TF pathway inhibitor and added anticoagulants

284
Q

FFP can be stored for up to

A

1 year

285
Q

After thawing, FFP should be used within

A

24 hours

286
Q

therapeutic dose of FFP

A

10-15ml/kg

287
Q

FFP can be used for emergent reversal of

A

vit K antagonist

288
Q

FFP is given within 24 hours of

A

ECMO or Cardiopulmonary bypass

289
Q

cryoprecipitate transfusion is indicated in children whose fibrinogen levels are less than

A

100mg/dL

290
Q

cryoprecipitate dosing

A

one unit per 5-10kg of weight

increase 50-100mg/dl

291
Q

Each unit of RBCs contains how much iron

A

200-250mg iron - with repeated transfusions saturating the patient’s transferrin -> can lead to nontransferrin bound iron ->cause organ damage (liver and heart disease)

292
Q

___ to ___ PRBC transfusions can saturate the transferrin

A

10-20

293
Q

in children iron accumulation in the anterior pituitary gland will produce

A

systemic endocrine disturbances (delayed sexual maturation and growth failure

294
Q

what imaging can evaluate for cardiac iron overload

A

modified MRI

295
Q

what type of therapy may be required in iron overload s/t chronic transfusions

A

iron chelation therapy
(Deferoxamine - IV agent)
(Deferasirox -oral agent)

296
Q

washed blood products reduce risk of

A

inflammatory markers

297
Q

for chronically transfused patients administer units < =

A

21 days old

298
Q

what 3 systems are usually involved in GVHD

A

skin
mucosal cells (oral and GI tract)
liver

299
Q

what type of transplants carry higher risk for GVHD

A

allogenic

300
Q

what risk factors raise risk for GVHD

A

source of stem cells

allogenic transplants higher risk - degree of HLA matching

female cells for a male pt

increased donor age

301
Q

how is GVHD measured in skin

A

Rash staged
grade I with less than 25% of BSA ->
Grade IV - bullae and desquamation

302
Q

how is GVHD measured in the liver

A

total bilirubin

303
Q

how is GVHD measured in GI tract

A

determined by volume of diarrhea

304
Q

a form of hypervolemia that occurs secondary to rapidly administered transfusions due to high osmotic load of blood products

A

TACO

305
Q

who is at increased risk for TACO (transfusion Associated circulatory overload

A

pt with cardiac disease and

infants

306
Q

international society of blood transfusion guidelines for TACO advocate looking for 2 + of what symptoms

A

1) evidence of pulmonary edema
2) evidence for cardiovascular changes to include tachycardia, hypertension, JVD, peripheral edema
3) positive fluid balance or increase in pt weight
4) elevation in Brain natriuretic peptide (BNP)

307
Q

treatment for TACO

A

supportive
diuretics
oxygen
positive pressure ventilation if needed

308
Q

s/s of hemolytic transfusion reaction

A 
fever
chills
headache
nausea
vomiting
chest or back pain
hypotension
tachycardia
tachypnea
hemoglobinuria
309
Q

a delayed Hemolytic transfusion reaction can occur up to how long after a transfusion

A

4 weeks

310
Q

symptoms of delayed Hemolytic transfusion reaction

A

can be asymptomatic or

fever
fatigue
jaundice

311
Q

Children with sickle cell anemia can present with a ______ as a symptom of delayed HTR

A

vaso-occlusive crisis

312
Q

febrile nonhemolytic transfusion reactions are characterized by a rise in temp >

A

1 degree C or 1.8 degree F during or shortly following a transfusion and is a diagnosis of exclusion

associated symptoms
chills
rigors
vomiting
flushing
anxiety
headache
313
Q

anaphylaxis to blood transfusions -what blood should be sent

A

supportive care and epi

send a IgA level and a anti-IgA antibody level because with severe IgA def can receive washed RBCs, platelets and IgA neg donor FFP if transfusions are needed in future

314
Q

second leading cause of death among transfusion related fatalities

A

TRAILI - Transfusion related acute lung injury

315
Q

activation of either donor or recipient neutrophils that cause an inflammatory cascade and sequestration of neutrophils within the pulmonary vasculature ->leads to endothelial injury and pulmonary edema

A

TRAILI - development of acute onset hypoxemia and bilat infiltrates within 6 hours of transfusion without a cardiac cause for pulmonary edema -> treat with supportive care

316
Q

most common form of SCD

A

Sickle cell anemia (HbSS)

317
Q

most severe form of SCD

A

Sickle cell anemia (HbSS)

318
Q

asymptomatic carrier of SCD

A

Sickle cell trait

319
Q

what type of genetic disorder is SCD

A

autosomal recessive inherited disorder

320
Q

how is SCD identified

A

newborn screen

321
Q

confirmatory SCD testing

A

hemoglobin electrophoresis

322
Q

prenatal diagnosis for SCD

A

amniocentesis

chorionic villus sampling

323
Q

the only cure for sickle cell anemia is

A

BMT

324
Q

what is the only disease modifying med to treat SCD

A

Hydroxyurea which increases fetal hemoglobin levels resulting in decreased incidence of complications

325
Q

supportive care for SCD

A

initiation of PCN prophylaxis by 2 mos of age and continued to at least 5 yrs old

326
Q

additional vaccines for SCD

A

ie)pneumococcal and meningococcal given at 2 and 5 yrs old

327
Q

infants and young children in the first year of life who have SCD often present with

A

Dactylitis - pain and swelling of the hands and feet

they may refuse to bear weight, be irritable or have a fever

328
Q

SCD higher risk for what?

A

stroke

329
Q

what is acute chest syndrome in a SCD pt

A

characterized by presence of chest pain, fever and resp symptoms accompanied by a new pulmonary infiltrate on chest x ray from ischemia and/or infarction of a lung segment

330
Q

what is one of the leading causes of death in children with SCD

A

splenic sequestration

331
Q

s/s splenic sequestration

A

distended abdomen
L sided abd pain
vomiting
shock

profound hypotension with cardiac decompensation may be present along with Hb concentrations as low as 2gm/dl

can be as young as 8 weeks old

332
Q

SCD are particularly susceptible to

A

infection due to reduced or absent splenic fx

high risk for septicemia and meningitis due to encapsulated bacteria such as Strep pneumonia, Neisseria meningitidis, and Haemophilus influenza

normally a pt with SCD can compensate for decreased red cells survival by ramping up bone marrow output 6-8 fold but any temp reduction in bone marrow activity due to incurrent viral or bacterial infection can cause the Hb and HCT to fall precipitously -> aplastic crisis (s/s extreme pallor and fatigue but no jaundice, lab show severe anemia without reticulocytosis)

333
Q

treatment for Acute chest syndrome

A

ABX
supplemental Oxygen
exchange transfusion if having severe resp distress or failure

334
Q

Most children with SCD will have a dysfunctional ____ for the first year of life

A

spleen

335
Q

repeated ischemia of spleen will cause autosplenectomy and atrophy by age

A

5 - leads to massive enlargement due to trapping of sickled RBCs

336
Q

what is the most common cause of death in children with SCD

A

infection

337
Q

individuals with SCD are at higher risk and should be screened for

A

HTN
retinopathy
risk for stroke

ages 2-16 should be screened annually

retinopathy screen start at age 10

338
Q

pregnancy concerns for SCD

A

preterm delivery
stillbirth
maternal mortality
severe fetal anemia

339
Q

an acquired autoimmune disorder that results in destruction of platelets. presents in healthy children (peaks at ages 2-6).

A

Immune Thrombocytopenic Purpura

in children < 12 yrs, it is self limiting and resolves within 6 months

340
Q

ITP is frequently precipitated by

A

viral illness

341
Q

IgG, IgA or IgM autoantibodies coat the platelets and they are destroyed in the spleen with resulting splenic sequestration

A

ITP

342
Q 
bruising and petechiae
epistaxis
GI Bleeding
Hematuria
Menorrhagia
spontaneous bleeding from mucous membranes and gingiva
A

clinical presentation of ITP

*intracranial bleeding and splenomegaly are possible

343
Q

diagnostic of ITP

A

<100,000 platelet count

large platelets on smear

PT and PTT normal

fibrinogen normal

fibrin degradation products normal

Diagnosis may be confirmed by bone marrow aspiration (normocellular result with elevated megakaryocytes)

344
Q

Management or ITP

A

Acute ITP (less than 6 mos) goal is to restore the platelet count

oral corticosteroids (weeks to months)

IV gamma globulin (IVIG) or Anti D immunoglobulin (WinRho-D).

recurrent monitoring of platelet counts guide therapy

Avoid NSAIDS and ASA

for Chronic IPT - regular administration of IVIG (WinRho-D)

splenectomy

345
Q

An acute systemic, immune complex small vessel vasculitis, that is self limiting and usually resolves about 4 weeks (peak age affecting more males is between 2-8)

A

Henoch-Schonlein Purpura

346
Q

what is the most serious complication of HSP

A

renal

347
Q

what usually precipitates HSP

A

Most commonly - Group A streptococcus

URI
medication
environmental trigger

348
Q

IgA complexes are deposited in the small vessels of the renal glomeruli, skin and GI tract causing petechiae, purpura, GI Bleeding and glomerulonephritis

A

HSP

349
Q

tetrad of symptoms for HSP

A

Rash: nonpruritic, erythematous papules or wheals that progress to petechiae and nonblanching, palpable, purpuric lesions >10mm diameter (trunk is usually spared and lesions fade over 10-12 days

Polyarthralgias - pain, swelling, decreased ROM. most frequently lower extremities

Bowel angina - diffuse, colicky abdominal pain with melena and vomiting (70 % of pt)

Renal symptoms with hematuria, proteinuria, and HTN (20-60%) weeks to mths after initial presentation

350
Q

diagnostics for HSP

A

clinical features
evaluate renal function

platelets normal or elevated
BUN and creatinine may be elevated
normal coags
immune antibody pannel (presence of IgA antibodies in the blood skin or glomerili

urinalysis for blood and protein

351
Q

management of HSP

A

rest and activity limitations

manage systemic complications - NSAIDS

oral prednisone - if there is kidney involvement

if results in severe kidney disease - may require plasma exchange, high dose IVIG or immunosuppressant agents

long term mgmt of HTN may be required

352
Q

what is the first sign of HIT

A

venous thrombosis

353
Q

management of HIT

A

removal of heparin and LMWH

if ongoing anticoagulation is needed, use vit K antagonist or direct thrombin inhibitor

Platelet recovery and disappearance of antibodies can take weeks once heparin is discontinued

354
Q

absence of Hb A on electrophoresis and microcytic anemia

A

B thalassemia

355
Q

RLQ pain and fever in an immunocompromise pt usually indicates

A

Typhlitis not appendicitis

356
Q

Starburst mass on x ray to the proximal tibia

A

osteosarcoma

357
Q

moth eaten or onion skin appearance of prox tibia on xray

A

Ewing sarcoma

358
Q

non invasive test that can be ordered to confirm a suspicion of neuroblastoma is

A

urine for HVA and VMA (catecholamines) (24 hour or random urine specimen)

359
Q

tachycardia

pallor

flow murmur

what are you thinking

A

acute anemia

get a CBC

360
Q

jaundice associated with anemia is caused by what

A

increased red cell destruction (hemolysis) ->results in unconjugated hyperbilirubinemia s/t the livers inability to conjugate and remove the destroyed RBCs as quickly as they are being hemolyzed

361
Q

how should children receiving anticoagulation with unfractionated heparin (ie) Enoxaparin) be monitored

A

Anti-XA with goal of 0.5

362
Q

what type of sickle cell disease are more commonly affected by vascular necrosis

A

HbSC

have milder disease
mild anemia or normal hb levels

363
Q

Congenital hemihypertrophy is a finding associated with what congenital disease and have a higher risk of developing a Wilms tumor

A

Beckwith-Weidemann Syndrome (BWS)

364
Q

first step in transfusion reaction

A

stop transfusion and administer NS

365
Q

what type of abx associated with methemglobinemia

A

sulfonamide antibiotics

366
Q

cyanotic pt with spo2 100 % that does not improve with Oxygen is a hallmark sign for what?

A

methemglobinemia

367
Q

what test provides the most accurate diagnosis of methemoglobinemia

A

Spectrophotometric assay

368
Q

what is next step for methemoglobinemia after causative agent is removed

A

Methylene blue is treatment of choice in children who are symtomatic

369
Q

preferred anticoagulant for DVT in history of HIT

A

Bivalirudin (direct thrombin inhibitor)

370
Q

type 1 von willebrand disease is inherited as a _____ disorder

A

autosomal

371
Q

diagnostic indications for LP

A

meningitis

autoimmune processes

viral encephalopathies

Guillain -Barre

Multiple Sclerois

Subarachnoid Hemorrhage

Cancer grading (test for CNS involvement)

372
Q

Therapeutic indications for LP

A

Pseudotumor cerebri

Intrathecal chemo

Intrathecal abx

spinal anesthesia

373
Q

contraindications for LP

A

impending herniation
Inappropriate hgb, platelets or coags

anticoagulation

localized infection over proposed needle insertion site

trauma to lumbar spine/full spine precautions

374
Q

Complications for LP

A 
CSF leak
spinal headache
pain
bleeding/hematoma
herniation
375
Q

tests for CSF

A

culture/gram stain

glucose

protein

cell count/diff

376
Q

what goes in tube 1 for CSF

A

culture/gram stain

377
Q

what goes in tube 2 and 3 for CSF collection

A

glucose, protein or other tests

378
Q

What goes in tube 4 for CSF collection

A

CSF cell count/diff

379
Q

for lateral recumbent position where do hips need to be for an LP

A

perpendicular to bed

380
Q

what vertebral space for LP

A

L3-L4 or L4-L5

381
Q

where is your landmark for LP

A

feel for top edge of iliac crest and move directly downward from there to the spine

382
Q

where should your bevel of needle be when performing an LP

A

perpendicular to spinal canal

383
Q

in lateral recumbent position for an LP where should your bevel of needle be facing

A

up to ceiling

384
Q

in sitting position, where should your bevel of needle be facing

A

outward towards side

385
Q

how much CSF should be in each collection tube

A

0.5-1mL

386
Q

what is approx max of removal for CSF

A

10 ml , can do more with larger kids if needed to make a diagnosis

387
Q

post LP instructions

A

lay flat for 4-6 hours to prevent CSF leak and headache

388
Q

airway pediatric anatomic considerations

A

large head ->increased neck flexion and obstruction

smaller nostrils, narrow airways and larger tongue _> increased airway resistance

lower muscle tone

more horizonal epiglottis

shorter trachea ->increased risk of R mainstem intubation

389
Q

pediatrics vs adult

The narrowest portion of the pediatric airway is the

A

cricoid ring

vs adult which is the vocal cord

390
Q

for child > 2 what might be helpful to use in an intubation

A

a folded towel under the occiput

391
Q

what FiO2 does Non rebreather offer and how much flow is required

A

12-15 l flow

offers FiO2 approx 0.85 as opposed to room air believed to be 0.22-0.5

392
Q

Pre-oxygenate for how long prior to first intubation attempt

A

2-3 min

393
Q

what does preoxygenation actually do

A

washes out nitrogen contained in lungs after breathing room air -> gives us a larger alveolar oxygen reservoir

394
Q

intubation steps

A

hold laryngoscope in LEFT hand with blade extended
- Using RIGHT hand, take index finger and thumb in a scissor motion and open the
patients mouth (like opening grandma’s coin purse!)
-sweep the blade into the mouth right-to-center to move tongue
-Advance blade while also gently lifting hand/wrist/forearm in fixed upward motion
motion with ~60 degree angle (the trajectory of the angle should be in line with
laryngoscope handle). Prevent rocking motion of the wrist and careful not to damage
teeth.
-at glottic opening with vocal cords visualized, advance ET tube along the RIGHT
side of the mouth toward the opening until the first set of black markings on the tube
disappear. Keep your eyes on the airway at all times……your RT/RN at the head of the
bed will hand you ETT, suction, remove stylet, etc…
-Hold ET tube firmly until initial confirmations are complete and tube is secured with
tape.

395
Q

how to verify placement of ET tube

A

listen for breath sounds

capnography

improvement in 02 sates and color of pt

chest x ray

396
Q

you intubated a pt and are listening for breath sounds

you hear gurgling over epigastric region

A

may indicate the tube is in the esophagus

397
Q

ET tube tip should be located where

A

mid trachea

can vary with age so if placed accurately it should lie between the clavicles

for adults or large teens approx 5 cm above carina

398
Q

how do you know if your capnography is showing a false pos

A

it causes a permanent color change in the device

399
Q

the capnography color is violet with

A

greater O2 exposure

400
Q

the capnography color is yellow with

A

CO2 exposure

401
Q

why do you consider Atropine in children less than 1 yr for RSI

A

reduces chance of bradycardia from airway manipulation

402
Q

Larynx is the most heavily innervated sensory structure in the body (Vagus nerve -CN X). Stimulation of larynx in intubation can do what?

A

activate the parasympathetic nervous system causing bradycardia, bronchial vasoconstriction and increased ICP

403
Q

why is lidocaine used in RSI

A

given to blunt the cough reflex and bronchospasm caused by intubation

best used to control ICPs when intubating an TBI

404
Q

what meds used in RSI to render a pt unconscious and prepare for paralysis

A

Fentanyl/Versed

405
Q

what is used in RSI for neuromuscular blockade (paralysis)

A

Rocuronium

Vecuronium

406
Q

RSI med used for emergence reactions

A

Ketamine

407
Q

RSI med can cause adrenal suppression

A

Etomidate

408
Q

RSI med with a very long list of warnings/precautions

A

Succinylcholine

409
Q

formula to select ET tube size

A

16+ age in years

all divided by 4

410
Q

depth of insertion for ET tube

A

length = ET tube size x 3

411
Q

level of sedation?

purposeful responses to verbal or tactile stimulation

A

moderate sedation

412
Q

level of sedation?

Purposeful response after repeated or painful stimulation

A

deep sedation

413
Q

level of sedation

unarousable even with painful stimulation

A

general anesthesia

414
Q

what levels of sedation may require airway

A

deep is maybe

often is general anesthesia

415
Q

what level of sedation can affect cardiac function

A

general anesthesia

416
Q

WHO pain ladder

A

+/- NSAIDS during all steps

Step 1 - nonopioid analgesics + adjuvants

step 2 - weak opioids
+/-adjuvants

step 3 - strong opioids
+/- adjuvants

417
Q

Benzodiazepines (-pam, -lam)

good and bad

ie) Midazolam

A

effects - induce anterograde amnesia
anticonvulsant properties
muscle relaxant properties

physiological dependence
resp depression
hypotension
paradoxical excitation

418
Q

Midazolam vs Lorazepam

A

Midazolam

  • rapid onset so good for acute agitation
  • active metabolite so prolonged sedation may occur
  • contin infusion 0.01-0.05mg/kg/hr

Lorazepam -

  • crosses BBB more slowly
  • slower onset and longer Duration of action
  • no active metabolite so sedation more predictable

IV intermittent - 0.05-0.1mg/kg/dose q 4-6 hours

419
Q

Propofol has what kind of properties

A

Anesthetic (used in OR) with sedative and amnesic properties at low doses (ICU)

420
Q

onset of action
duration of action
where is it metabolized
dosing

Propofol

A

1-2 min
3-10 min from IV bolus
metabolized in liver without active metabolites

IV intermittent 1-2mg/kg/dose

continuous 5-50mcg/kg/min

421
Q

adverse effects of propofol

A 
resp and cardio depression
hypotension
bradycardia
injection site pain
triglycerides due to lipid emulsion formulation
422
Q

alpha 2 agonist sedation, analgesia and sympatholytic effects

preservation of resp drive

A

Dexmetomidine

continuous infusion 0,2-0.7mcg/kg/hr

423
Q

state behavioral scale

A

-3 unresponsive
-2 responsive to noxious stimuli
-1 responsive to gentle touch or voice
0 awake and able to calm
+1 restless and difficult to calm
+2 agitated

424
Q

tools for measuring quality and depth of sedation in ICU pts

A 
Richmond Agitation Sedation Score (RASS)
\+4 combative
\+3 very agitated
\+2 agitated
\+1 restless
0 alert and calm
-1 drowsy
-2 light sedation
-3 moderate sedation
-4 deep sedation
-5 unarousable

Sedation-Agitation Score(SAS)

425
Q

what opioid analgesic can cause chest wall rigidity and is 100 times more potent than morphine?

A

Fentanyl (0.5-4mcg/kg/hr for contin)

426
Q

morphine continuous dosing

A

0.01-0.3 mcg/kg/hr

427
Q

what opioid analgesic is a good option in renal dysfunction and why

A

Hydromorphone - no active metabolites

428
Q

When to avoid NSAIDS in what type of dysfunction or failure

A

avoid in renal dysfunction/failure

429
Q

max duration of ketorolac

A

5 days

430
Q

dosing for ibuprofen

A

10mg/kg/dose q 4-6 hours

max 40mg/kg/day

Critical Care I (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions