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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

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2
Q

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

A

Leukemia

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3
Q

how is leukemia classified

A

the cell line affected and level where differentiation has been interrupted

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4
Q

what inherited conditions predispose patients to ALL

A

Fanconi anemia

Trisomy 21

Ataxia telangiectasis

klinefelter syndrome

shwachman-diamond syndrome

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5
Q

genetic predisposition for CML

A

Associated with chromosomal translocation known as Philadelphia chromosome

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6
Q

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

A

CML

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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

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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

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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
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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

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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)

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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

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13
Q

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

A

WBC>100,000mm3
or tumor lysis syndrome

PICU

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14
Q

Leukemia and hyperleukocytosis is what?

A

WBC >100,000mm3

results in increased blood viscosity

Medical emergency

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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)

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16
Q

3 phases of ALL treatment

A

Induction

consolidation

maintenance

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17
Q

chemotherapy treatment for ALL lasts how long and depends on ?

A

2-3 years depending on risk and gender

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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

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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

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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

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21
Q

side effects of chemo and radiation

A 
n/v
alopecia
mucositis
anorexia
pancytopenia
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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

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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)

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24
Q

Leukemia physical exam findings

A

pallor
petechiae
hepatosplenomegaly
adenopathy

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25
signs and symptoms of CNS involvement for leukemia
``` headache papilledema retinal hemorrhages cranial nerve palsies coma ```
26
signs/symptoms of hyperleukocytosis
fever, bone pain, night sweats, priapism, malaise, splenomegaly causing abd pain
27
chronic phase of CML is defined as
less than 10% blasts in the bone marrow and high levels of myeloid cells and precursors
28
Accelerated phase of CML is defined as
10-19 % blasts in the bone marrow, organomegaly, leukocytosis, and abnormal platelet counts and functioning
29
what is ordered in leukemia to look at patient's risk of developing Acute Tumor lysis syndrome (ATLS)
metabolic studies
30
what is ordered in leukemia to evaluate for the presence of a mediastinal mass and why
chest x ray it can compromise the pediatric pt airway
31
what is ordered to assess CNS involvement in leukemia
LP
32
life threatening complications that need to be identified in leukemia
``` Sepsis DIC ATLS (acute tumor lysis syndrome) hyperleukocytosis leukostasis ```
33
what should platelet count be prior to LP
>80,000 cells mm3 (generally)
34
malignancy arises from lymph node or lymph tissue
lymphoma
35
3rd most common type of pediatric cancer
lymphoma
36
4 sub types of hodgkins lymphoma
lymphocyte predominant nodular sclerosing mixed cellularity lymphocyte depleted
37
what virus is believed to have an association with Hodkins lymphoma
Ebstein-Barr virus
38
what population is at greater risk for Hodkins lymphoma
children with immunodeficiencies
39
3 main types of non-Hodkins lymphoma
lymphoblastic lymphoma mature B cell lymphoma Anaplastic large cell lymphoma
40
clinical presentation of Hodkins lymphoma
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
41
Non-Hodkins lymphoma clinical presentation
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
42
diagnostic for lymphoma
CT staging - measuring tumor/mass and identifying spread bone scans and bone marrow aspirate LP - if CNS symptoms PET scan Biopsy
43
neoplasm from the sympathetic nervous system, most common extracranial solid tumor of childhood
neuroblastoma
44
neuroblastomas are most frequently diagnosed during
infancy
45
neuroblastoma presentation
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
46
diagnostics for neuroblastoma
``` 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
47
what population for neuroblastoma could present emergently and require immediate evaluation for
Patients with orbital, mediastinal, spinal cord, or extensive abdominal disease
48
most common abdominal tumor of childhood
Wilms tumor
49
Beckwith-wiedemann syndrome puts pt more at risk for what onc
Wilms tumor
50
WAGR syndrome puts pt more at risk for what onc
Wilms tumor
51
most common presenting sign of wilms tumor
asymptomatic abd mass
52
clinical presentation for wilms tumor
asymptomatic abd mass HTN (due to renal artery compression and possibly increased renin production gross hematuria fever constipation Rare: hypotension, anemia
53
diagnostic for wilms tumor
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
54
inflammation or destruction of mucosal cells of the oral cavity and throughout the GI tract
mucositis
55
clinical presentation of mucositis
mouth sores and/or ulcerations from esophagus to rectum ``` severe pain anorexia dehydration bleeding infection ```
56
how to manage mucositis
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
57
when is hyperleukocytosis typically seen
new-onset leukemic patients
58
what oncologic process are the complications for hyperleukocytosis more significant
AML
59
what is the most common complication of hyperleukocytosis
stroke
60
``` fever lethargy mental status changes headache seizure stroke coma dyspnea tachypnea hypoxemia resp failure acidosis cor pulmonale hemorrhage DIC Renal failure ```
clinical presentation for hyperleukocytosis
61
diagnostics for hyperleukocytosis
WBC >100,000 CXR - diffuse interstitial infiltrates Head CT - high risk of intracranial hemorrhage
62
management of hyperleukocytosis
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)
63
claw sign on imaging
(the appearance of the mass wrapping around the kidney on imaging) - Wilms tumor
64
what happens in sepsis in oncology
vasodilation myocardial dysfunction leading to multiorgan system dysfunction and third spacing of fluid as a result of capillary leak.
65
what happens in superior vena cava syndrome
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
66
clinical presentation of superior vena cava syndrome
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
67
diagnostic eval for superior vena cava syndrome
chest radiograph chest CT US
68
management for superior vena cava syndrome
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
69
oncological emergency associated with initial chemotherapy for leukemias, lymphomas and other tumors with high growth rates. Can affect neurological, pulmonary, cardiac and renal function
Tumor Lysis syndrome
70
what is released massively in tumor lysis syndrome
large amounts of potassium, phosphate and uric acid
71
when does tumor lysis syndrome most commonly occur
within 6-48 hours and up to 7 days after initiation of chemotherapy
72
symptoms of tumor lysis syndrome
``` 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
73
diagnostic eval for tumor lysis syndrome
CMP
74
management of tumor lysis syndrome
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
75
A potentially life threatening disorder defined by the triad of neutropenia, abdominal pain and fever
Typhlitis (neutropenic enterocolitis)
76
what is the problem in Typhlitis
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
77
clinical presentation for Typhlitis
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
78
diagnostic for Typhlitis
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
79
management for Typhlitis
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
80
what measures oxygen carrying potential
Hemoglobin
81
what measures mass of RBC
hematocrit
82
Life span of RBC
approx 120 days (varies with age and gender)
83
What shows us RBC production
Reticulocyte count (measures bone marrow response to anemia)
84
what part of blood are mediators of inflammation
WBC
85
medical term for decreased WBC
leukopenia
86
medical term for elevated WBC
leukocytosis
87
what granulocyte is primary infectious response
segmented neutrophils
88
what granulocyte is a mature neutrophil
Polys
89
immature neutrophils
bands
90
granulocyte seen in allergic response
Eosinophils
91
agranulocytes involved in immune response
T cell and B cell lymphocytes
92
Agranulocyte produced by bone marrow, process foreign antigens through phagocytosis
Monocytes
93
indicator of pt ability to fight infection? what number is concerning
ANC (absolute neutrophil count) - <500 is concerning
94
formula for ANC
Total #WBCs x (%polys+%bands)
95
life span of platelets
5-9 days. Transfused platelets have a shorter life span
96
average concentration of hemoglobin of a given volume of PRBC,
MCHC - mean corpuscular hemoglobin concentration
97
MCHC - mean corpuscular hemoglobin concentration formula
dividing the hemoglobin by the hematocrit
98
average quantity of hemoglobin
MCH (mean corpuscular hemoglobin)
99
index of the size of RBC
MCV
100
abnormal WBCs
blasts
101
what lab study indicates the time for plasma to clot in the presence of thromboplastin (extrinsic pathway), altered in vit k deficiency
Prothrombin time (PT)
102
what lab study measures conversion of fibrinogen to fibrin - last step in the clotting process
Thrombin Time (TT)
103
activated partial thromboplastin time is most accurate if results are obtained within ___ hours of specimen collection
4
104
iron is stored in tissue as
ferritin
105
what lab shows percentage of transferrin saturated with iron showing the capacity of blood to transport iron
Transferrin saturation
106
what lab measures reserve capacity of transferrin
Total iron binding capacity (TIBC)
107
isoenzyme used as a marker of tissue breakdown or damage and hemolysis
LDH
108
product of metabolic breakdown of purine nucleotides. Some cancers will cause this to elevate which can contribute to renal failure
Uric acid
109
Advantages of PET scan
ability to up and down stage disease and evaluate response to chemotherapy
110
disadvantages of PET scan
results are not specific to a cancer diagnosis. significant radiation lower sensitivity in brain and lungs
111
A PET scan provides information about
metabolic activity and the proliferative potential of a residual tumor
112
clinical indications of PET
baseline staging disease response f/u for disease surveillance
113
fuses metabolic activity with anatomical structures
PET-CT
114
what is best at detecting bone marrow and bony sites of disease in rhabdomyosarcoma? PET CT or Bone Scan
PET-CT
115
Pediatric challenges in PET
need for sedation pregnancy screening potential artifact caused by normal physiologic uptake
116
what is critical to have for a PET scan to ensure accurate imaging measurements
Correct weight
117
in bone marrow aspiration immature cells are ____ in hyperplastic conditions such as leukemia
increased
118
What other condition may show atypical cells increased on bone marrow aspiration
vit B12 or folate deficiency myelodysplastic syndromes Drug toxins infectious problems
119
what is bone marrow aspiration used for
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
120
causes of anemia
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
121
transient erythroblastopenia of childhood
transient red cell aplasia -> typically follows viral illness with anemia in the range of 6-8mg/dL but can be lower and reticulocytopenia
122
symptoms of anemia
``` weakness fatigue confusion palpitations pallor tachycardia flow murmur diminished peripheral pulses jaundice ```
123
diagnostic anemia
decrease in HCT and HGB
124
decrease in HCT and HGB increased retic count low MCV
hemoglobinopathies (such as thalassemia syndromes)
125
decrease in HCT and HGB increased retic count normal MCV
membrane enzyme or immune disorders microangiopathic anemias DIC infection-induced hemolysis chronic blood loss
126
decrease in HCT and HGB low, normal or slightly elevated retic low MCV think...
iron def anemia lead tox thalassemia trait sideroblastic anemia anemia of chronic disease
127
decreased in HCT and HGB low normal or slightly elevated retics high MCV think...
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
128
anemia with hypovolemic shock, how to treat?
volume expansion with packed RBC
129
life threatening disease of bone marrow failure resulting in decreased production of hematopoietic stem cells that results in peripheral pancytopenia and bone marrow aplasia
aplastic anemia
130
clinical presentation of aplastic anemia
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 ```
131
diagnostic for aplastic anemia
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
132
management of aplastic anemia
transfusion of RBC and platelets ABX Bone marrow transplant immunosuppressive therapy if unable to receive a BMT
133
A rare congenital hypoplastic anemia resulting in constitutional bone marrow failure
Diamond-Blackfan Anemia
134
mutation for Diamond Blackfan is on Chromosome
19
135
clinical presentation of Diamond Blackfan anemia
``` 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 ```
136
diagnostics for Diamond Blackfan anemia
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
137
Management for Diamond Blackfan Anemia
Corticosteroids (glucocorticoids improve erythropoiesis and 60-70% achieve transfusion independence) frequent blood transfusion BMT in some cases
138
what teams do you need involved for Diamond Blackfan Anemia pt
Hematology BMT Endocrinology
139
AKA defibrination syndrome
DIC (Disseminated intravascular coagulation)
140
AKA consumptive coagulopathy
DIC (Disseminated intravascular coagulation)
141
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
DIC (Disseminated intravascular coagulation)
142
most common cause of DIC
infection
143
what type of sepsis is most commonly associated with DIC
gram neg sepsis
144
causes of DIC
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
145
what is DIC
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
146
headache altered LOC Bleeding disproportionate bruising
DIC
147
findings in DIC
diffuse bleeding petechiae ecchymosis purpura hematoma gingival bleeding and epistaxis hematuria hematemesis melena intrahepatic hemorrhage s/s shock thrombosis cool mottled skin poor perfussion
148
diagnostic of DIC
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
149
Management of DIC
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
150
a disease of the microcirculation is characterized by hemolytic anemia, thrombocytopenia and acute renal failure. occurs most frequently in children younger than 4
Hemolytic uremic syndrome
151
most common cause of ARF
Hemolytic uremic syndrome
152
etiology of Hemolytic uremic syndrome
contamination of water, meats, fruits and vegetables with infectious bacteria E.Coli 0157:H7 is most common of post-diarrheal (D+) HUS
153
Peak season for Hemolytic uremic syndrome
summer
154
most common etiology of post-diarrheal (D+) HUS
E coli 0157:H7
155
When do you see D+ HUS
previously healthy children who have had recent gastroenteritis
156
Bacteria that cause HUS
E. Coli 0157:H7 infection (Shiga toxin; most common cause), shigella dysenteriae, citrobacter freundii and other subtypes of E coli (Also shiga todxdin)
157
what is worse D-HUS or D+ HUS
D-HUS is atypical and more severe
158
what type of HUS may have a familial link and may also begin in the neonatal period
D- HUS
159
causative factors of HUS
Inherited factor H deficiency -> inhibits complement activation membrane cofactor protein mutations streptococcus pneumoniae infection meds - cyclosporine and Tacrolimus
160
incubation period of D+HUS
3-5 days
161
abdominal pain watery, non-bloody diarrhea fever weakness lethargy iritability
Symptoms of D+ HUS
162
Progression to hemorrhagic colitis occurs __ to __ days after onset of diarrhea for D+HUS
5-7
163
clinical findings in D+ HUS
``` pallor petechiae ecchymoses hematuria oliguria azotemia HTN ``` may progress to anuria, hepatomegaly splenomegaly hematemesis edema tremor and seizures
164
HUS diagnosis
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
165
Management of D+HUS
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
166
long term follow up care of D+HUS and D- HUS
monitor BP and urinalysis proteinuria, decreased GFR, HTN may recur up to 1 yr later
167
D-HUS treatment
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
168
Children with cervical masses can exhibit symptoms of ____ syndrome, including ____
Horner Syndrome unilateral ptosis myosis anhidrosis
169
most common sites for metastatic disease in neuroblastoma are
``` lymph nodes liver bone bone marrow skin ``` rare for lung or brain
170
Cairo and Bishop definitions of tumor lysis syndrome
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
171
chvostek sign
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
172
Trousseau Sign
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
173
what does allopurinol do
blocks the conversion of hypoxanthine and xanthine to uric acid - prevents future uric acid production but does not effect what is already there
174
Allopurinol dosing
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
what does Rasburicase do
metabolizes uric acid to water soluble allantoin
176
dosing for Rasburicase
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
contraindication for Rasburicase
Pt with G6PD deficiency bc hydrogen peroxide (a byproduct of uric acid metabolism) can trigger hemolysis
178
important to note for Rasburicase (lab draws)
Rasburicase can remain active out of the body....when you are doing a serum uric acid...keep blood on ice until processed
179
treatment for hyperkalemia in the setting of tumor lysis syndrome
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
normal potassium level
3.4-5.3 mEq/L
181
treatment for hyperphosphatemia in the setting of TLS
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
dosing for Aluminum hydroxide
prevents phosphorous absorption in GI tract orally 50-150 mg/kg/dose q 6 hours
183
hypocalcemia treatment in the setting of TLS
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
Benadmustine (traditional cytotoxic agent) when administered concurrently with allopurinol
can result in serious complications such as SJS and Toxic Epidermal Necrolysis
185
most pediatric oncologists define survivorship when the patient
is 5 years post diagnosis and min of 2 yrs free of cancer treatment
186
phosphorous normal range
4.5-6.5mg/dL
187
normal calcium range
8.8-10.8mg/dL
188
normal uric acid
2-6.2mg/dL
189
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
B-Thalassemia
190
what are the 3 phenotypes of B-Thalassemia
Thalassemia minor B Thalassemia intermedia Thalassemia major
191
what B Thalassemia phenotype: | patients are clinically asymptomatic but have specific hematologic findings
Thalassemia minor
192
what B thalassemia phenotype? symptoms can range in severity
B-Thalassemia intermedia
193
what B thalassemia phenotype? severe transfusion dependent anemia
Thalassemia major
194
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
B thalassemia
195
one of the most common autosomal recessive disorders in the world High prevalence among individuals of ___, ____, and ____ descent
B Thalassemia Mediterranean African Southeast asian
196
which B thalassemia phenotype is usually diagnosed during infancy
B-thalassemia major
197
presentation of pt with B thalassemia
``` FTT pallor irritability diarrhea abdominal enlargement (hepatosplenomegaly) jaundice ```
198
diagnosis of B thalassemia
lab data to confirm
199
B thalassemia major is also called
Cooley Anemia
200
thalassemia that occurs when a gene or genes r/t the A globin protein are missing or changed (mutated)
A-Thalassemia major
201
B Thalassemia is very common in....
Italy and Greece Mediterranean middle eastern Asian
202
what chromosome is involved in B thalassemia
11
203
B Thalassemia | Heterozygous is which phenotype
B thalassemia minor
204
B thalassemia | Homozygous is which phenotype
can be | B thalassemia intermedia or major
205
B thalassemia is what type of anemia
Microcytic anemia
206
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
B thalassemia minor
207
in B thalassemia major where is the problem?
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
B-thalassemia intermedia has a co-inheritance with
A thal trait
209
in B thalassemia intermedia you have minor qualitative defects in
B globin
210
typical features for Thalassemia
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
findings of thalassemia
``` hepatomegaly splenomegaly enlarged kidneys with dilated renal tubules dark urine cardiac abnormalities delayed sexual development ```
212
Hypochromic, microcytic anemia with decreased MCV, basophilic stippling and presence of Hgb A may have hyperuricemia
Thalassemia
213
primary treatment for thalassemia
blood transfusion and folate replacement BMT from a matched sibling donor is the best chance for cure
214
complications for thalassemia
iron overload from transfusions congestive heart failure early death
215
A thalassemia trait
one locus deletion (AA, A-) | pt is asymptomatic
216
A thalassemia minor
2 loci deleted (can be A-, A-) or (AA, --) pt symptomatic with low MCV
217
A thalassemia intermedia
3 loci deleted (A, -, --) | moderate to severe hemolytic anemia
218
4 loci deleted no effective erythropoiesis hydrops fetalis (baby dies en utero)
A thalassemia major
219
most accurate test to diagnose a thalassemia
genetic studies
220
A thalassemia intermedia needs what?
blood transfusions
221
According to Virchow's triad concept, thrombosis is caused by a disruption in this triad:
changes in vessel wall alteration in blood flow increased coagulability of the blood
222
acquired risk factors for thrombosis
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
antiphospholipid syndrome
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
inherited risk factor for thrombosis
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
Venous thrombosis in neck vessels can result in
superior vena cava syndrome
226
what is a cerebral sinus venous thrombosis s/s?
thrombus deep veins of the head persistent headache blurred vision neurologic signs seizures
227
what type of thrombosis is associated with nephrotic syndrome and what symptom?
renal vein thrombosis generalized edema
228
Portal vein thrombosis causes
splenomegaly with thrombocytopenia and anemia. Esophageal varices can result
229
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
May-Thurner Syndrome
230
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
Paget-Schroetter syndrome
231
what electrolyte abnormality increases risk for thombi
hypercalcemia
232
the single greatest risk factor for arterial or venous thrombosis in infants and children is
the presence of a CVC
233
what pediatric population is most at risk for developing a DVT or PE
infant less than 1 year and adolescents
234
in infants less than one year of age, the majority of non CVC related thrombotic cases are from
RVT (renal vein thrombosis)
235
most commonly used diagnostic for DVT is
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
diagnostic for PE
``` CT angio (Computed tomography angiography) is the study of choice ``` Ventilation -perfusion lung scan (VQ scan) are still occasionally used
237
anticoagulant therapy in pt with arterial disease
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
inheritance pattern for Von willebrand disease (vWD)
autosomal dominant
239
a plasma protein composed of multimers that cause platelet adhesion to initiate a "platelet plug" and act as a carrier protein for factor VIII.
vWF (von Willebrand factor)
240
which vWF is a qualitative protein dysfunction of circulating vWF
vWD type 2 (further divided into 2A, 2B, 2M, 2N)
241
which vWf are caused by an insufficient quantity of vWF
types 1 and 3
242
what is the most common inherited bleeding disorder
vWD
243
what disease process reduces the circulating level of vWF in plasma
hypothyroidism
244
what factors can increase circulating level of vWF in plasma
``` contraceptives exercise stress inflammation pregnancy ```
245
what history should put vWF in your mind
history of easy bruising frequent epistaxis heavy menstrual bleeding or bleeding after a surgical/dental procedure
246
diagnosis of Von Willebrand
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
If the RCo activity and the vWF antigen differ by more than 30% there is evidence of
protein dysfunction (type 2 vWD)
248
what lab measures vWF function
RCo activity
249
what lab measures vWF quantity
vWF antigen
250
first line treatment for mild bleeding episodes in pt with type 1 vWD
IV or IN DDAVP (desmopressin acetate - stimulates release of VWF and factor VIII
251
DDAVP side effect
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
in vWD, RCo and Factor VIII goal for minor surgical procedures
>=30 maintain for 1-5 days post procedure
253
In vWD, RCo and Factor VIII goal for more invasive procedures, aim for
>=50 maintain for 1-5 days post procedure
254
In vWD, RCo and Factor VIII goal for neurosurgery, abdominal surgery or other major surgery, aim for
>=100 maintain for 1-5 days post procedure
255
for vWD what is the most common presenting symptom in women treat>=?
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
how are antifibrinolytics used in vWD
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
all pt diagnosed with vWD should have what done for baseline levels
serum iron and ferritin levels to assess for iron deficiency
258
what type of vWD is the most severe, requiring a more comprehensive maintenance plan
type 3
259
Any pt who received clotting factor concentrates prior to when should have additional screening
1985 | Hep B and C and HIV
260
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
Fresh Frozen Plasma (FFP)
261
what blood product is responsible for hemostasis with resulting thrombus formation.
platelets
262
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
Cryoprecipitate
263
blood product used to increased oxygen circulation and delivery
RBCs
264
threshold for transfusion in children
Hb 7gm/dl
265
at what Hb point is the "do not go past, must transfuse number?
Hb <5 gm/dL because the risk of morbidity and mortality is increased
266
what is the recommended transfusion volume in children for PRBCs
10-15 ml/kg which will raise the Hb concentration 2-3 gm/dL or HCT by 6-9%
267
All RBC transfusions should be completed within ___ hours after removal from a controlled temp storage
4
268
in clinically stable children what is the platelet threshold
10,000 uL
269
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
50,000uL | 100,000uL
270
platelet transfusions are not indicated in a pt with what? unless what
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
how are platelets stored
at room temp
272
Platelets should be given at
1unit per every 10kg weight 10-15ml/kg at the rate of 10-20ml/kg/hr expect a 50,000 uL rise
273
FFP should be given at
10-15ml/kg/transfusion
274
Cryoprecipitate should be given at
1 unit per every 10kg pt weight expect rise of 60-100mg/dL
275
To calculate PRBC transfusion dose
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
____ is possible when children drink large volumes of milk
anemia
277
Stored PRBC's lack _____ content, so children who receive any type of multiple transfusion require?
calcium additional calcium
278
PRBCs stored > __ days are associated with higher ___ concentrations
5 days | potassium
279
Based on illness severity, infants < ___ months of age will not require cross matching
4 mos in critical situations it can be used for any child
280
Estimated circulating blood volume is based on ?
average calculation of 80ml/kg adults are 70mL/kg
281
Massive transfusion complications include
thrombocytopenia hypocalcemia coagulation factor depletion hyperkalemia increased levels of lactic acid (acid base disorders) hypothermia altered or decreased oxygen delivery to tissues
282
what is the shelf life of platelets
7 days
283
FFP contains clotting factors
II, V, VIII, IX, X and XI, fibrinogen, albumin, protein C, Protein S, antithrombin, TF pathway inhibitor and added anticoagulants
284
FFP can be stored for up to
1 year
285
After thawing, FFP should be used within
24 hours
286
therapeutic dose of FFP
10-15ml/kg
287
FFP can be used for emergent reversal of
vit K antagonist
288
FFP is given within 24 hours of
ECMO or Cardiopulmonary bypass
289
cryoprecipitate transfusion is indicated in children whose fibrinogen levels are less than
100mg/dL
290
cryoprecipitate dosing
one unit per 5-10kg of weight increase 50-100mg/dl
291
Each unit of RBCs contains how much iron
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
___ to ___ PRBC transfusions can saturate the transferrin
10-20
293
in children iron accumulation in the anterior pituitary gland will produce
systemic endocrine disturbances (delayed sexual maturation and growth failure
294
what imaging can evaluate for cardiac iron overload
modified MRI
295
what type of therapy may be required in iron overload s/t chronic transfusions
iron chelation therapy (Deferoxamine - IV agent) (Deferasirox -oral agent)
296
washed blood products reduce risk of
inflammatory markers
297
for chronically transfused patients administer units < =
21 days old
298
what 3 systems are usually involved in GVHD
skin mucosal cells (oral and GI tract) liver
299
what type of transplants carry higher risk for GVHD
allogenic
300
what risk factors raise risk for GVHD
source of stem cells allogenic transplants higher risk - degree of HLA matching female cells for a male pt increased donor age
301
how is GVHD measured in skin
Rash staged grade I with less than 25% of BSA -> Grade IV - bullae and desquamation
302
how is GVHD measured in the liver
total bilirubin
303
how is GVHD measured in GI tract
determined by volume of diarrhea
304
a form of hypervolemia that occurs secondary to rapidly administered transfusions due to high osmotic load of blood products
TACO
305
who is at increased risk for TACO (transfusion Associated circulatory overload
pt with cardiac disease and | infants
306
international society of blood transfusion guidelines for TACO advocate looking for 2 + of what symptoms
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
treatment for TACO
supportive diuretics oxygen positive pressure ventilation if needed
308
s/s of hemolytic transfusion reaction
``` fever chills headache nausea vomiting chest or back pain hypotension tachycardia tachypnea hemoglobinuria ```
309
a delayed Hemolytic transfusion reaction can occur up to how long after a transfusion
4 weeks
310
symptoms of delayed Hemolytic transfusion reaction
can be asymptomatic or fever fatigue jaundice
311
Children with sickle cell anemia can present with a ______ as a symptom of delayed HTR
vaso-occlusive crisis
312
febrile nonhemolytic transfusion reactions are characterized by a rise in temp >
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
anaphylaxis to blood transfusions -what blood should be sent
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
second leading cause of death among transfusion related fatalities
TRAILI - Transfusion related acute lung injury
315
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
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
most common form of SCD
Sickle cell anemia (HbSS)
317
most severe form of SCD
Sickle cell anemia (HbSS)
318
asymptomatic carrier of SCD
Sickle cell trait
319
what type of genetic disorder is SCD
autosomal recessive inherited disorder
320
how is SCD identified
newborn screen
321
confirmatory SCD testing
hemoglobin electrophoresis
322
prenatal diagnosis for SCD
amniocentesis | chorionic villus sampling
323
the only cure for sickle cell anemia is
BMT
324
what is the only disease modifying med to treat SCD
Hydroxyurea which increases fetal hemoglobin levels resulting in decreased incidence of complications
325
supportive care for SCD
initiation of PCN prophylaxis by 2 mos of age and continued to at least 5 yrs old
326
additional vaccines for SCD
ie)pneumococcal and meningococcal given at 2 and 5 yrs old
327
infants and young children in the first year of life who have SCD often present with
Dactylitis - pain and swelling of the hands and feet they may refuse to bear weight, be irritable or have a fever
328
SCD higher risk for what?
stroke
329
what is acute chest syndrome in a SCD pt
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
what is one of the leading causes of death in children with SCD
splenic sequestration
331
s/s splenic sequestration
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
SCD are particularly susceptible to
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
treatment for Acute chest syndrome
ABX supplemental Oxygen exchange transfusion if having severe resp distress or failure
334
Most children with SCD will have a dysfunctional ____ for the first year of life
spleen
335
repeated ischemia of spleen will cause autosplenectomy and atrophy by age
5 - leads to massive enlargement due to trapping of sickled RBCs
336
what is the most common cause of death in children with SCD
infection
337
individuals with SCD are at higher risk and should be screened for
HTN retinopathy risk for stroke ages 2-16 should be screened annually retinopathy screen start at age 10
338
pregnancy concerns for SCD
preterm delivery stillbirth maternal mortality severe fetal anemia
339
an acquired autoimmune disorder that results in destruction of platelets. presents in healthy children (peaks at ages 2-6).
Immune Thrombocytopenic Purpura in children < 12 yrs, it is self limiting and resolves within 6 months
340
ITP is frequently precipitated by
viral illness
341
IgG, IgA or IgM autoantibodies coat the platelets and they are destroyed in the spleen with resulting splenic sequestration
ITP
342
``` bruising and petechiae epistaxis GI Bleeding Hematuria Menorrhagia spontaneous bleeding from mucous membranes and gingiva ```
clinical presentation of ITP *intracranial bleeding and splenomegaly are possible
343
diagnostic of ITP
<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
Management or ITP
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
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)
Henoch-Schonlein Purpura
346
what is the most serious complication of HSP
renal
347
what usually precipitates HSP
Most commonly - Group A streptococcus URI medication environmental trigger
348
IgA complexes are deposited in the small vessels of the renal glomeruli, skin and GI tract causing petechiae, purpura, GI Bleeding and glomerulonephritis
HSP
349
tetrad of symptoms for HSP
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
diagnostics for HSP
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
management of HSP
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
what is the first sign of HIT
venous thrombosis
353
management of HIT
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
absence of Hb A on electrophoresis and microcytic anemia
B thalassemia
355
RLQ pain and fever in an immunocompromise pt usually indicates
Typhlitis not appendicitis
356
Starburst mass on x ray to the proximal tibia
osteosarcoma
357
moth eaten or onion skin appearance of prox tibia on xray
Ewing sarcoma
358
non invasive test that can be ordered to confirm a suspicion of neuroblastoma is
urine for HVA and VMA (catecholamines) (24 hour or random urine specimen)
359
tachycardia pallor flow murmur what are you thinking
acute anemia get a CBC
360
jaundice associated with anemia is caused by what
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
how should children receiving anticoagulation with unfractionated heparin (ie) Enoxaparin) be monitored
Anti-XA with goal of 0.5
362
what type of sickle cell disease are more commonly affected by vascular necrosis
HbSC have milder disease mild anemia or normal hb levels
363
Congenital hemihypertrophy is a finding associated with what congenital disease and have a higher risk of developing a Wilms tumor
Beckwith-Weidemann Syndrome (BWS)
364
first step in transfusion reaction
stop transfusion and administer NS
365
what type of abx associated with methemglobinemia
sulfonamide antibiotics
366
cyanotic pt with spo2 100 % that does not improve with Oxygen is a hallmark sign for what?
methemglobinemia
367
what test provides the most accurate diagnosis of methemoglobinemia
Spectrophotometric assay
368
what is next step for methemoglobinemia after causative agent is removed
Methylene blue is treatment of choice in children who are symtomatic
369
preferred anticoagulant for DVT in history of HIT
Bivalirudin (direct thrombin inhibitor)
370
type 1 von willebrand disease is inherited as a _____ disorder
autosomal
371
diagnostic indications for LP
meningitis autoimmune processes viral encephalopathies Guillain -Barre Multiple Sclerois Subarachnoid Hemorrhage Cancer grading (test for CNS involvement)
372
Therapeutic indications for LP
Pseudotumor cerebri Intrathecal chemo Intrathecal abx spinal anesthesia
373
contraindications for LP
impending herniation Inappropriate hgb, platelets or coags anticoagulation localized infection over proposed needle insertion site trauma to lumbar spine/full spine precautions
374
Complications for LP
``` CSF leak spinal headache pain bleeding/hematoma herniation ```
375
tests for CSF
culture/gram stain glucose protein cell count/diff
376
what goes in tube 1 for CSF
culture/gram stain
377
what goes in tube 2 and 3 for CSF collection
glucose, protein or other tests
378
What goes in tube 4 for CSF collection
CSF cell count/diff
379
for lateral recumbent position where do hips need to be for an LP
perpendicular to bed
380
what vertebral space for LP
L3-L4 or L4-L5
381
where is your landmark for LP
feel for top edge of iliac crest and move directly downward from there to the spine
382
where should your bevel of needle be when performing an LP
perpendicular to spinal canal
383
in lateral recumbent position for an LP where should your bevel of needle be facing
up to ceiling
384
in sitting position, where should your bevel of needle be facing
outward towards side
385
how much CSF should be in each collection tube
0.5-1mL
386
what is approx max of removal for CSF
10 ml , can do more with larger kids if needed to make a diagnosis
387
post LP instructions
lay flat for 4-6 hours to prevent CSF leak and headache
388
airway pediatric anatomic considerations
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
pediatrics vs adult The narrowest portion of the pediatric airway is the
cricoid ring vs adult which is the vocal cord
390
for child > 2 what might be helpful to use in an intubation
a folded towel under the occiput
391
what FiO2 does Non rebreather offer and how much flow is required
12-15 l flow offers FiO2 approx 0.85 as opposed to room air believed to be 0.22-0.5
392
Pre-oxygenate for how long prior to first intubation attempt
2-3 min
393
what does preoxygenation actually do
washes out nitrogen contained in lungs after breathing room air -> gives us a larger alveolar oxygen reservoir
394
intubation steps
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
how to verify placement of ET tube
listen for breath sounds capnography improvement in 02 sates and color of pt chest x ray
396
you intubated a pt and are listening for breath sounds you hear gurgling over epigastric region
may indicate the tube is in the esophagus
397
ET tube tip should be located where
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
how do you know if your capnography is showing a false pos
it causes a permanent color change in the device
399
the capnography color is violet with
greater O2 exposure
400
the capnography color is yellow with
CO2 exposure
401
why do you consider Atropine in children less than 1 yr for RSI
reduces chance of bradycardia from airway manipulation
402
Larynx is the most heavily innervated sensory structure in the body (Vagus nerve -CN X). Stimulation of larynx in intubation can do what?
activate the parasympathetic nervous system causing bradycardia, bronchial vasoconstriction and increased ICP
403
why is lidocaine used in RSI
given to blunt the cough reflex and bronchospasm caused by intubation best used to control ICPs when intubating an TBI
404
what meds used in RSI to render a pt unconscious and prepare for paralysis
Fentanyl/Versed
405
what is used in RSI for neuromuscular blockade (paralysis)
Rocuronium | Vecuronium
406
RSI med used for emergence reactions
Ketamine
407
RSI med can cause adrenal suppression
Etomidate
408
RSI med with a very long list of warnings/precautions
Succinylcholine
409
formula to select ET tube size
16+ age in years all divided by 4
410
depth of insertion for ET tube
length = ET tube size x 3
411
level of sedation? purposeful responses to verbal or tactile stimulation
moderate sedation
412
level of sedation? Purposeful response after repeated or painful stimulation
deep sedation
413
level of sedation unarousable even with painful stimulation
general anesthesia
414
what levels of sedation may require airway
deep is maybe often is general anesthesia
415
what level of sedation can affect cardiac function
general anesthesia
416
WHO pain ladder
+/- NSAIDS during all steps Step 1 - nonopioid analgesics + adjuvants step 2 - weak opioids +/-adjuvants step 3 - strong opioids +/- adjuvants
417
Benzodiazepines (-pam, -lam) good and bad ie) Midazolam
effects - induce anterograde amnesia anticonvulsant properties muscle relaxant properties physiological dependence resp depression hypotension paradoxical excitation
418
Midazolam vs Lorazepam
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
Propofol has what kind of properties
Anesthetic (used in OR) with sedative and amnesic properties at low doses (ICU)
420
onset of action duration of action where is it metabolized dosing Propofol
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
adverse effects of propofol
``` resp and cardio depression hypotension bradycardia injection site pain triglycerides due to lipid emulsion formulation ```
422
alpha 2 agonist sedation, analgesia and sympatholytic effects preservation of resp drive
Dexmetomidine continuous infusion 0,2-0.7mcg/kg/hr
423
state behavioral scale
-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
tools for measuring quality and depth of sedation in ICU pts
``` 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
what opioid analgesic can cause chest wall rigidity and is 100 times more potent than morphine?
Fentanyl (0.5-4mcg/kg/hr for contin)
426
morphine continuous dosing
0.01-0.3 mcg/kg/hr
427
what opioid analgesic is a good option in renal dysfunction and why
Hydromorphone - no active metabolites
428
When to avoid NSAIDS in what type of dysfunction or failure
avoid in renal dysfunction/failure
429
max duration of ketorolac
5 days
430
dosing for ibuprofen
10mg/kg/dose q 4-6 hours | max 40mg/kg/day

Critical Care I (27 decks)

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