Test 2 Roadmap questions Flashcards
3 most common types of pediatric cancer
most common is?
ALL (acute lymphoblastic leukemia)
AML (acute myelogenous leukemia)
Chronic myelogenous leukemia (CML)
Most common is ALL
cancer of the blood and blood forming organs such as the bone marrow, lymph nodes and spleen
Leukemia
how is leukemia classified
the cell line affected and level where differentiation has been interrupted
what inherited conditions predispose patients to ALL
Fanconi anemia
Trisomy 21
Ataxia telangiectasis
klinefelter syndrome
shwachman-diamond syndrome
genetic predisposition for CML
Associated with chromosomal translocation known as Philadelphia chromosome
initial phase is “chronic” and associated with Leukocytosis, mild anemia and thrombocytosis
CML
May progress from the chronic phase after a variable amount of time to the accelerated phase and ____ _____ which resembles acute _____
Blast crisis
resembles acute leukemia
what are blast cells
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
clinical presentation of ALL (can vary)
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
Clinical presentation of AML
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
CML clinical presentation
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)
Leukemia diagnostics
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
Leukemia diagnosis with elevated WBC or hyperleukocytosis means? where to admit
WBC>100,000mm3
or tumor lysis syndrome
PICU
Leukemia and hyperleukocytosis is what?
WBC >100,000mm3
results in increased blood viscosity
Medical emergency
treatment for hyperleukocytosis
aggressive hydration
correction of metabolic disturbances
prevention of tumor lysis syndrome
may require leukopheresis or exchange transfusion
recombinant urate oxidase (rasburicase)
3 phases of ALL treatment
Induction
consolidation
maintenance
chemotherapy treatment for ALL lasts how long and depends on ?
2-3 years depending on risk and gender
CNS therapy and ALL
required either for prophylaxis or treatment due to risk for CNS relapse and failure of systemic chemotherapy to penetrate the blood brain barrier adequately
AML treatment
short intense periods of multi- agent chemotherapy -> causes prolonged marrow hypoplasia and immunosuppression (may need Hematopoietic stem cell transplant (HSCT) from an allogenic donor
CML treatment
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
side effects of chemo and radiation
n/v alopecia mucositis anorexia pancytopenia
what places leukemia pt esp at high risk for infections and serious complications of infections
leukopenia, specifically neutropenia combined with a central venous catheter
children with Noonan syndrome and neurofibromatosis type 1 are at particular risk of developing a specific type of AML known as
juvenile myelomonocytic leukemia (JMML)
Leukemia physical exam findings
pallor
petechiae
hepatosplenomegaly
adenopathy
signs and symptoms of CNS involvement for leukemia
headache papilledema retinal hemorrhages cranial nerve palsies coma
signs/symptoms of hyperleukocytosis
fever, bone pain, night sweats, priapism, malaise, splenomegaly causing abd pain
chronic phase of CML is defined as
less than 10% blasts in the bone marrow and high levels of myeloid cells and precursors
Accelerated phase of CML is defined as
10-19 % blasts in the bone marrow, organomegaly, leukocytosis, and abnormal platelet counts and functioning
what is ordered in leukemia to look at patient’s risk of developing Acute Tumor lysis syndrome (ATLS)
metabolic studies
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
what is ordered to assess CNS involvement in leukemia
LP
life threatening complications that need to be identified in leukemia
Sepsis DIC ATLS (acute tumor lysis syndrome) hyperleukocytosis leukostasis
what should platelet count be prior to LP
> 80,000 cells mm3 (generally)
malignancy arises from lymph node or lymph tissue
lymphoma
3rd most common type of pediatric cancer
lymphoma
4 sub types of hodgkins lymphoma
lymphocyte predominant
nodular sclerosing
mixed cellularity
lymphocyte depleted
what virus is believed to have an association with Hodkins lymphoma
Ebstein-Barr virus
what population is at greater risk for Hodkins lymphoma
children with immunodeficiencies
3 main types of non-Hodkins lymphoma
lymphoblastic lymphoma
mature B cell lymphoma
Anaplastic large cell lymphoma
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
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
diagnostic for lymphoma
CT
staging - measuring tumor/mass and identifying spread
bone scans and bone marrow aspirate
LP - if CNS symptoms
PET scan
Biopsy
neoplasm from the sympathetic nervous system, most common extracranial solid tumor of childhood
neuroblastoma
neuroblastomas are most frequently diagnosed during
infancy
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
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
what population for neuroblastoma could present emergently and require immediate evaluation for
Patients with orbital, mediastinal, spinal cord, or extensive abdominal disease
most common abdominal tumor of childhood
Wilms tumor
Beckwith-wiedemann syndrome puts pt more at risk for what onc
Wilms tumor
WAGR syndrome puts pt more at risk for what onc
Wilms tumor
most common presenting sign of wilms tumor
asymptomatic abd mass
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
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
inflammation or destruction of mucosal cells of the oral cavity and throughout the GI tract
mucositis
clinical presentation of mucositis
mouth sores and/or ulcerations from esophagus to rectum
severe pain anorexia dehydration bleeding infection
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
when is hyperleukocytosis typically seen
new-onset leukemic patients
what oncologic process are the complications for hyperleukocytosis more significant
AML
what is the most common complication of hyperleukocytosis
stroke
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
diagnostics for hyperleukocytosis
WBC >100,000
CXR - diffuse interstitial infiltrates
Head CT - high risk of intracranial hemorrhage
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)
claw sign on imaging
(the appearance of the mass wrapping around the kidney on imaging) - Wilms tumor
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.
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
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
diagnostic eval for superior vena cava syndrome
chest radiograph
chest CT
US
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
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
what is released massively in tumor lysis syndrome
large amounts of potassium, phosphate and uric acid
when does tumor lysis syndrome most commonly occur
within 6-48 hours and up to 7 days after initiation of chemotherapy
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
diagnostic eval for tumor lysis syndrome
CMP
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
A potentially life threatening disorder defined by the triad of neutropenia, abdominal pain and fever
Typhlitis (neutropenic enterocolitis)
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
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
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
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
what measures oxygen carrying potential
Hemoglobin
what measures mass of RBC
hematocrit
Life span of RBC
approx 120 days (varies with age and gender)
What shows us RBC production
Reticulocyte count (measures bone marrow response to anemia)
what part of blood are mediators of inflammation
WBC
medical term for decreased WBC
leukopenia
medical term for elevated WBC
leukocytosis
what granulocyte is primary infectious response
segmented neutrophils
what granulocyte is a mature neutrophil
Polys
immature neutrophils
bands
granulocyte seen in allergic response
Eosinophils
agranulocytes involved in immune response
T cell and B cell lymphocytes
Agranulocyte produced by bone marrow, process foreign antigens through phagocytosis
Monocytes
indicator of pt ability to fight infection? what number is concerning
ANC (absolute neutrophil count) - <500 is concerning
formula for ANC
Total #WBCs x (%polys+%bands)
life span of platelets
5-9 days. Transfused platelets have a shorter life span
average concentration of hemoglobin of a given volume of PRBC,
MCHC - mean corpuscular hemoglobin concentration
MCHC - mean corpuscular hemoglobin concentration formula
dividing the hemoglobin by the hematocrit
average quantity of hemoglobin
MCH (mean corpuscular hemoglobin)
index of the size of RBC
MCV
abnormal WBCs
blasts
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)
what lab study measures conversion of fibrinogen to fibrin - last step in the clotting process
Thrombin Time (TT)
activated partial thromboplastin time is most accurate if results are obtained within ___ hours of specimen collection
4
iron is stored in tissue as
ferritin
what lab shows percentage of transferrin saturated with iron showing the capacity of blood to transport iron
Transferrin saturation
what lab measures reserve capacity of transferrin
Total iron binding capacity (TIBC)
isoenzyme used as a marker of tissue breakdown or damage and hemolysis
LDH
product of metabolic breakdown of purine nucleotides. Some cancers will cause this to elevate which can contribute to renal failure
Uric acid
Advantages of PET scan
ability to up and down stage disease and evaluate response to chemotherapy
disadvantages of PET scan
results are not specific to a cancer diagnosis.
significant radiation
lower sensitivity in brain and lungs
A PET scan provides information about
metabolic activity and the proliferative potential of a residual tumor
clinical indications of PET
baseline staging
disease response
f/u for disease surveillance
fuses metabolic activity with anatomical structures
PET-CT
what is best at detecting bone marrow and bony sites of disease in rhabdomyosarcoma?
PET CT or Bone Scan
PET-CT
Pediatric challenges in PET
need for sedation
pregnancy screening
potential artifact caused by normal physiologic uptake
what is critical to have for a PET scan to ensure accurate imaging measurements
Correct weight
in bone marrow aspiration immature cells are ____ in hyperplastic conditions such as leukemia
increased
What other condition may show atypical cells increased on bone marrow aspiration
vit B12 or folate deficiency
myelodysplastic syndromes
Drug toxins
infectious problems
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
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
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
symptoms of anemia
weakness fatigue confusion palpitations pallor tachycardia flow murmur diminished peripheral pulses jaundice
diagnostic anemia
decrease in HCT and HGB
decrease in HCT and HGB
increased retic count
low MCV
hemoglobinopathies (such as thalassemia syndromes)
decrease in HCT and HGB
increased retic count
normal MCV
membrane enzyme or immune disorders
microangiopathic anemias
DIC
infection-induced hemolysis
chronic blood loss
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
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
anemia with hypovolemic shock, how to treat?
volume expansion with packed RBC
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
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
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
management of aplastic anemia
transfusion of RBC and platelets
ABX
Bone marrow transplant
immunosuppressive therapy if unable to receive a BMT
A rare congenital hypoplastic anemia resulting in constitutional bone marrow failure
Diamond-Blackfan Anemia
mutation for Diamond Blackfan is on Chromosome
19
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
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
Management for Diamond Blackfan Anemia
Corticosteroids (glucocorticoids improve erythropoiesis and 60-70% achieve transfusion independence)
frequent blood transfusion
BMT in some cases
what teams do you need involved for Diamond Blackfan Anemia pt
Hematology
BMT
Endocrinology
AKA defibrination syndrome
DIC (Disseminated intravascular coagulation)
AKA consumptive coagulopathy
DIC (Disseminated intravascular coagulation)
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)
most common cause of DIC
infection
what type of sepsis is most commonly associated with DIC
gram neg sepsis
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
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
headache
altered LOC
Bleeding
disproportionate bruising
DIC
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
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
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
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
most common cause of ARF
Hemolytic uremic syndrome
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
Peak season for Hemolytic uremic syndrome
summer
most common etiology of post-diarrheal (D+) HUS
E coli 0157:H7
When do you see D+ HUS
previously healthy children who have had recent gastroenteritis
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)
what is worse D-HUS or D+ HUS
D-HUS is atypical and more severe
what type of HUS may have a familial link and may also begin in the neonatal period
D- HUS
causative factors of HUS
Inherited factor H deficiency -> inhibits complement activation
membrane cofactor protein mutations
streptococcus pneumoniae infection
meds - cyclosporine and Tacrolimus
incubation period of D+HUS
3-5 days
abdominal pain
watery, non-bloody diarrhea
fever
weakness
lethargy
iritability
Symptoms of D+ HUS
Progression to hemorrhagic colitis occurs __ to __ days after onset of diarrhea for D+HUS
5-7
clinical findings in D+ HUS
pallor petechiae ecchymoses hematuria oliguria azotemia HTN
may progress to anuria, hepatomegaly
splenomegaly
hematemesis
edema
tremor and seizures
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
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
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
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
Children with cervical masses can exhibit symptoms of ____ syndrome, including ____
Horner Syndrome
unilateral ptosis
myosis
anhidrosis
most common sites for metastatic disease in neuroblastoma are
lymph nodes liver bone bone marrow skin
rare for lung or brain
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
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
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
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
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)
what does Rasburicase do
metabolizes uric acid to water soluble allantoin
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
contraindication for Rasburicase
Pt with G6PD deficiency bc hydrogen peroxide (a byproduct of uric acid metabolism) can trigger hemolysis
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
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)
normal potassium level
3.4-5.3 mEq/L
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
dosing for Aluminum hydroxide
prevents phosphorous absorption in GI tract
orally 50-150 mg/kg/dose q 6 hours
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)
Benadmustine (traditional cytotoxic agent) when administered concurrently with allopurinol
can result in serious complications such as SJS and Toxic Epidermal Necrolysis
most pediatric oncologists define survivorship when the patient
is 5 years post diagnosis and min of 2 yrs free of cancer treatment
phosphorous normal range
4.5-6.5mg/dL
normal calcium range
8.8-10.8mg/dL
normal uric acid
2-6.2mg/dL
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
what are the 3 phenotypes of B-Thalassemia
Thalassemia minor
B Thalassemia intermedia
Thalassemia major
what B Thalassemia phenotype:
patients are clinically asymptomatic but have specific hematologic findings
Thalassemia minor
what B thalassemia phenotype?
symptoms can range in severity
B-Thalassemia intermedia
what B thalassemia phenotype?
severe transfusion dependent anemia
Thalassemia major
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
one of the most common autosomal recessive disorders in the world
High prevalence among individuals of ___, ____, and ____ descent
B Thalassemia
Mediterranean
African
Southeast asian
which B thalassemia phenotype is usually diagnosed during infancy
B-thalassemia major
presentation of pt with B thalassemia
FTT pallor irritability diarrhea abdominal enlargement (hepatosplenomegaly) jaundice
diagnosis of B thalassemia
lab data to confirm
B thalassemia major is also called
Cooley Anemia
thalassemia that occurs when a gene or genes r/t the A globin protein are missing or changed (mutated)
A-Thalassemia major
B Thalassemia is very common in….
Italy and Greece
Mediterranean
middle eastern
Asian
what chromosome is involved in B thalassemia
11
B Thalassemia
Heterozygous is which phenotype
B thalassemia minor
B thalassemia
Homozygous is which phenotype
can be
B thalassemia intermedia or major
B thalassemia is what type of anemia
Microcytic anemia
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
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)
B-thalassemia intermedia has a co-inheritance with
A thal trait
in B thalassemia intermedia you have minor qualitative defects in
B globin
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
findings of thalassemia
hepatomegaly splenomegaly enlarged kidneys with dilated renal tubules dark urine cardiac abnormalities delayed sexual development
Hypochromic, microcytic anemia with decreased MCV, basophilic stippling and presence of Hgb A
may have hyperuricemia
Thalassemia
primary treatment for thalassemia
blood transfusion and folate replacement
BMT from a matched sibling donor is the best chance for cure
complications for thalassemia
iron overload from transfusions
congestive heart failure
early death
A thalassemia trait
one locus deletion (AA, A-)
pt is asymptomatic
A thalassemia minor
2 loci deleted (can be A-, A-) or (AA, –)
pt symptomatic with low MCV
A thalassemia intermedia
3 loci deleted (A, -, –)
moderate to severe hemolytic anemia
4 loci deleted
no effective erythropoiesis
hydrops fetalis (baby dies en utero)
A thalassemia major
most accurate test to diagnose a thalassemia
genetic studies
A thalassemia intermedia needs what?
blood transfusions
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
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
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
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)
Venous thrombosis in neck vessels can result in
superior vena cava syndrome
what is a cerebral sinus venous thrombosis
s/s?
thrombus deep veins of the head
persistent headache
blurred vision
neurologic signs
seizures
what type of thrombosis is associated with nephrotic syndrome and what symptom?
renal vein thrombosis
generalized edema
Portal vein thrombosis causes
splenomegaly with thrombocytopenia and anemia. Esophageal varices can result
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
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
what electrolyte abnormality increases risk for thombi
hypercalcemia
the single greatest risk factor for arterial or venous thrombosis in infants and children is
the presence of a CVC
what pediatric population is most at risk for developing a DVT or PE
infant less than 1 year and adolescents
in infants less than one year of age, the majority of non CVC related thrombotic cases are from
RVT (renal vein thrombosis)
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
diagnostic for PE
CT angio (Computed tomography angiography) is the study of choice
Ventilation -perfusion lung scan (VQ scan) are still occasionally used
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
inheritance pattern for Von willebrand disease (vWD)
autosomal dominant
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)
which vWF is a qualitative protein dysfunction of circulating vWF
vWD type 2 (further divided into 2A, 2B, 2M, 2N)
which vWf are caused by an insufficient quantity of vWF
types 1 and 3
what is the most common inherited bleeding disorder
vWD
what disease process reduces the circulating level of vWF in plasma
hypothyroidism
what factors can increase circulating level of vWF in plasma
contraceptives exercise stress inflammation pregnancy
what history should put vWF in your mind
history of easy bruising
frequent epistaxis
heavy menstrual bleeding
or bleeding after a surgical/dental procedure
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.
If the RCo activity and the vWF antigen differ by more than 30% there is evidence of
protein dysfunction (type 2 vWD)
what lab measures vWF function
RCo activity
what lab measures vWF quantity
vWF antigen
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
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
in vWD, RCo and Factor VIII goal for minor surgical procedures
> =30
maintain for 1-5 days post procedure
In vWD, RCo and Factor VIII goal for more invasive procedures, aim for
> =50
maintain for 1-5 days post procedure
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
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)
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
all pt diagnosed with vWD should have what done for baseline levels
serum iron and ferritin levels to assess for iron deficiency
what type of vWD is the most severe, requiring a more comprehensive maintenance plan
type 3
Any pt who received clotting factor concentrates prior to when should have additional screening
1985
Hep B and C and HIV
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)
what blood product is responsible for hemostasis with resulting thrombus formation.
platelets
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
blood product used to increased oxygen circulation and delivery
RBCs
threshold for transfusion in children
Hb 7gm/dl
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
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%
All RBC transfusions should be completed within ___ hours after removal from a controlled temp storage
4
in clinically stable children what is the platelet threshold
10,000 uL
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
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
how are platelets stored
at room temp
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
FFP should be given at
10-15ml/kg/transfusion
Cryoprecipitate should be given at
1 unit per every 10kg pt weight
expect rise of 60-100mg/dL
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)
____ is possible when children drink large volumes of milk
anemia
Stored PRBC’s lack _____ content, so children who receive any type of multiple transfusion require?
calcium
additional calcium
PRBCs stored > __ days are associated with higher ___ concentrations
5 days
potassium
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
Estimated circulating blood volume is based on ?
average calculation of 80ml/kg
adults are 70mL/kg
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
what is the shelf life of platelets
7 days
FFP contains clotting factors
II, V, VIII, IX, X and XI, fibrinogen, albumin, protein C, Protein S, antithrombin, TF pathway inhibitor and added anticoagulants
FFP can be stored for up to
1 year
After thawing, FFP should be used within
24 hours
therapeutic dose of FFP
10-15ml/kg
FFP can be used for emergent reversal of
vit K antagonist
FFP is given within 24 hours of
ECMO or Cardiopulmonary bypass
cryoprecipitate transfusion is indicated in children whose fibrinogen levels are less than
100mg/dL
cryoprecipitate dosing
one unit per 5-10kg of weight
increase 50-100mg/dl
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)
___ to ___ PRBC transfusions can saturate the transferrin
10-20
in children iron accumulation in the anterior pituitary gland will produce
systemic endocrine disturbances (delayed sexual maturation and growth failure
what imaging can evaluate for cardiac iron overload
modified MRI
what type of therapy may be required in iron overload s/t chronic transfusions
iron chelation therapy
(Deferoxamine - IV agent)
(Deferasirox -oral agent)
washed blood products reduce risk of
inflammatory markers
for chronically transfused patients administer units < =
21 days old
what 3 systems are usually involved in GVHD
skin
mucosal cells (oral and GI tract)
liver
what type of transplants carry higher risk for GVHD
allogenic
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
how is GVHD measured in skin
Rash staged
grade I with less than 25% of BSA ->
Grade IV - bullae and desquamation
how is GVHD measured in the liver
total bilirubin
how is GVHD measured in GI tract
determined by volume of diarrhea
a form of hypervolemia that occurs secondary to rapidly administered transfusions due to high osmotic load of blood products
TACO
who is at increased risk for TACO (transfusion Associated circulatory overload
pt with cardiac disease and
infants
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)
treatment for TACO
supportive
diuretics
oxygen
positive pressure ventilation if needed
s/s of hemolytic transfusion reaction
fever chills headache nausea vomiting chest or back pain hypotension tachycardia tachypnea hemoglobinuria
a delayed Hemolytic transfusion reaction can occur up to how long after a transfusion
4 weeks
symptoms of delayed Hemolytic transfusion reaction
can be asymptomatic or
fever
fatigue
jaundice
Children with sickle cell anemia can present with a ______ as a symptom of delayed HTR
vaso-occlusive crisis
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
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
second leading cause of death among transfusion related fatalities
TRAILI - Transfusion related acute lung injury
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
most common form of SCD
Sickle cell anemia (HbSS)
most severe form of SCD
Sickle cell anemia (HbSS)
asymptomatic carrier of SCD
Sickle cell trait
what type of genetic disorder is SCD
autosomal recessive inherited disorder
how is SCD identified
newborn screen
confirmatory SCD testing
hemoglobin electrophoresis
prenatal diagnosis for SCD
amniocentesis
chorionic villus sampling
the only cure for sickle cell anemia is
BMT
what is the only disease modifying med to treat SCD
Hydroxyurea which increases fetal hemoglobin levels resulting in decreased incidence of complications
supportive care for SCD
initiation of PCN prophylaxis by 2 mos of age and continued to at least 5 yrs old
additional vaccines for SCD
ie)pneumococcal and meningococcal given at 2 and 5 yrs old
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
SCD higher risk for what?
stroke
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
what is one of the leading causes of death in children with SCD
splenic sequestration
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
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)
treatment for Acute chest syndrome
ABX
supplemental Oxygen
exchange transfusion if having severe resp distress or failure
Most children with SCD will have a dysfunctional ____ for the first year of life
spleen
repeated ischemia of spleen will cause autosplenectomy and atrophy by age
5 - leads to massive enlargement due to trapping of sickled RBCs
what is the most common cause of death in children with SCD
infection
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
pregnancy concerns for SCD
preterm delivery
stillbirth
maternal mortality
severe fetal anemia
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
ITP is frequently precipitated by
viral illness
IgG, IgA or IgM autoantibodies coat the platelets and they are destroyed in the spleen with resulting splenic sequestration
ITP
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
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)
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
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
what is the most serious complication of HSP
renal
what usually precipitates HSP
Most commonly - Group A streptococcus
URI
medication
environmental trigger
IgA complexes are deposited in the small vessels of the renal glomeruli, skin and GI tract causing petechiae, purpura, GI Bleeding and glomerulonephritis
HSP
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
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
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
what is the first sign of HIT
venous thrombosis
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
absence of Hb A on electrophoresis and microcytic anemia
B thalassemia
RLQ pain and fever in an immunocompromise pt usually indicates
Typhlitis not appendicitis
Starburst mass on x ray to the proximal tibia
osteosarcoma
moth eaten or onion skin appearance of prox tibia on xray
Ewing sarcoma
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)
tachycardia
pallor
flow murmur
what are you thinking
acute anemia
get a CBC
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
how should children receiving anticoagulation with unfractionated heparin (ie) Enoxaparin) be monitored
Anti-XA with goal of 0.5
what type of sickle cell disease are more commonly affected by vascular necrosis
HbSC
have milder disease
mild anemia or normal hb levels
Congenital hemihypertrophy is a finding associated with what congenital disease and have a higher risk of developing a Wilms tumor
Beckwith-Weidemann Syndrome (BWS)
first step in transfusion reaction
stop transfusion and administer NS
what type of abx associated with methemglobinemia
sulfonamide antibiotics
cyanotic pt with spo2 100 % that does not improve with Oxygen is a hallmark sign for what?
methemglobinemia
what test provides the most accurate diagnosis of methemoglobinemia
Spectrophotometric assay
what is next step for methemoglobinemia after causative agent is removed
Methylene blue is treatment of choice in children who are symtomatic
preferred anticoagulant for DVT in history of HIT
Bivalirudin (direct thrombin inhibitor)
type 1 von willebrand disease is inherited as a _____ disorder
autosomal
diagnostic indications for LP
meningitis
autoimmune processes
viral encephalopathies
Guillain -Barre
Multiple Sclerois
Subarachnoid Hemorrhage
Cancer grading (test for CNS involvement)
Therapeutic indications for LP
Pseudotumor cerebri
Intrathecal chemo
Intrathecal abx
spinal anesthesia
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
Complications for LP
CSF leak spinal headache pain bleeding/hematoma herniation
tests for CSF
culture/gram stain
glucose
protein
cell count/diff
what goes in tube 1 for CSF
culture/gram stain
what goes in tube 2 and 3 for CSF collection
glucose, protein or other tests
What goes in tube 4 for CSF collection
CSF cell count/diff
for lateral recumbent position where do hips need to be for an LP
perpendicular to bed
what vertebral space for LP
L3-L4 or L4-L5
where is your landmark for LP
feel for top edge of iliac crest and move directly downward from there to the spine
where should your bevel of needle be when performing an LP
perpendicular to spinal canal
in lateral recumbent position for an LP where should your bevel of needle be facing
up to ceiling
in sitting position, where should your bevel of needle be facing
outward towards side
how much CSF should be in each collection tube
0.5-1mL
what is approx max of removal for CSF
10 ml , can do more with larger kids if needed to make a diagnosis
post LP instructions
lay flat for 4-6 hours to prevent CSF leak and headache
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
pediatrics vs adult
The narrowest portion of the pediatric airway is the
cricoid ring
vs adult which is the vocal cord
for child > 2 what might be helpful to use in an intubation
a folded towel under the occiput
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
Pre-oxygenate for how long prior to first intubation attempt
2-3 min
what does preoxygenation actually do
washes out nitrogen contained in lungs after breathing room air -> gives us a larger alveolar oxygen reservoir
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.
how to verify placement of ET tube
listen for breath sounds
capnography
improvement in 02 sates and color of pt
chest x ray
you intubated a pt and are listening for breath sounds
you hear gurgling over epigastric region
may indicate the tube is in the esophagus
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
how do you know if your capnography is showing a false pos
it causes a permanent color change in the device
the capnography color is violet with
greater O2 exposure
the capnography color is yellow with
CO2 exposure
why do you consider Atropine in children less than 1 yr for RSI
reduces chance of bradycardia from airway manipulation
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
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
what meds used in RSI to render a pt unconscious and prepare for paralysis
Fentanyl/Versed
what is used in RSI for neuromuscular blockade (paralysis)
Rocuronium
Vecuronium
RSI med used for emergence reactions
Ketamine
RSI med can cause adrenal suppression
Etomidate
RSI med with a very long list of warnings/precautions
Succinylcholine
formula to select ET tube size
16+ age in years
all divided by 4
depth of insertion for ET tube
length = ET tube size x 3
level of sedation?
purposeful responses to verbal or tactile stimulation
moderate sedation
level of sedation?
Purposeful response after repeated or painful stimulation
deep sedation
level of sedation
unarousable even with painful stimulation
general anesthesia
what levels of sedation may require airway
deep is maybe
often is general anesthesia
what level of sedation can affect cardiac function
general anesthesia
WHO pain ladder
+/- NSAIDS during all steps
Step 1 - nonopioid analgesics + adjuvants
step 2 - weak opioids
+/-adjuvants
step 3 - strong opioids
+/- adjuvants
Benzodiazepines (-pam, -lam)
good and bad
ie) Midazolam
effects - induce anterograde amnesia
anticonvulsant properties
muscle relaxant properties
physiological dependence
resp depression
hypotension
paradoxical excitation
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
Propofol has what kind of properties
Anesthetic (used in OR) with sedative and amnesic properties at low doses (ICU)
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
adverse effects of propofol
resp and cardio depression hypotension bradycardia injection site pain triglycerides due to lipid emulsion formulation
alpha 2 agonist sedation, analgesia and sympatholytic effects
preservation of resp drive
Dexmetomidine
continuous infusion 0,2-0.7mcg/kg/hr
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
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)
what opioid analgesic can cause chest wall rigidity and is 100 times more potent than morphine?
Fentanyl (0.5-4mcg/kg/hr for contin)
morphine continuous dosing
0.01-0.3 mcg/kg/hr
what opioid analgesic is a good option in renal dysfunction and why
Hydromorphone - no active metabolites
When to avoid NSAIDS in what type of dysfunction or failure
avoid in renal dysfunction/failure
max duration of ketorolac
5 days
dosing for ibuprofen
10mg/kg/dose q 4-6 hours
max 40mg/kg/day
