Aquifer - Cardiovascular and Hematology (Part 1) Flashcards
(151 cards)
1
Q
List the steps in the cardiac examination.
A
- Assess color of skin and mucous membranes
- Precordial activity
- Heart sounds (S1 and S2, physiologic split of S2, possible gallop (S3 or S4), and clicks)
- Murmurs, (clicks, rubs)
(History - poor feeding, diaphoresis, FTT, family h/o CHD)
(Vitals)
(Inpsection for dysmorphism, cyanosis, clubbing)
(Distal pulses)
2
Q
What is the most common cyanotic heart defect? What are the other cyanotic congenital heart defects?
A
Tetralogy of Fallot
Truncus arteriosus, transposition of the great arteries, tricuspid atresia, and total anomalous pulmonary venous return
3
Q
What does a hyperactive precordium indicate?
A
Increased workload
4
Q
True or false - gallops are uncommonly heard in infants and children.
A
True
5
Q
What is a holosystolic murmur?
A
Begins with S1 (not after it), during isovolumic contraction
6
Q
List 3 causes of holosystolic murmurs.
A
VSD, mitral regurgitation, and tricuspid regurgitation
7
Q
What is an ejection murmur?
A
Systolic murmur that does not start until after S1 (delay to the onset of ejection)
8
Q
List 2 causes of ejection murmurs.
A
Aortic and pulmonic valve stenosis
9
Q
What type of murmur is always pathologic?
A
Diastolic murmur
10
Q
What causes a continuous murmur?
A
PDA
11
Q
How are murmurs graded (intensity)?
A
I - faint and easily missed
II - obvious
III - loud
IV - associated with a thrill
(III and IV are likely pathologic and should be evaluated by a cardiologist)
(1/6 - faint, not heard in all positions, no thrill)
(2/6 - soft, heard in all positions, no thrill)
(3/6 - loud, no thrill)
(4/6 - loud with palpable thrill)
(5/6 - heard with stethoscope partially off chest, thrill)
(6/6 - heard with stethoscope off the chest - thrill)
12
Q
Define hepatomegaly in infancy. What can cause a false impression of hepatomegaly?
A
Liver edge palpated >1 cm below the right costal margin; hyperexpansion of the lungs
13
Q
DDx - hepatomegaly in infancy?
A
CHF, congenital infections, inborn errors of metabolism, anemias, and tumors (less commonly)
14
Q
What are the classic findings of CHF in an infant a few weeks after birth?
A
Dyspnea with feedings Diaphoresis Poor growth Active precordium Hepatomegaly Tachycardia
15
Q
Describe the pathogenesis of CHF in infants.
A
Inefficient circulation, whether due to poor cardiac function, increased myocardial demand, or shunt lesions (most common in infants) leads to adrenergic activation. This increases metabolic demands that contribute to poor weight gain, as well as diaphoresis with any activity, including feeding.
16
Q
Which signs of CHF seen in adults are not frequently seen in children?
A
Rales, JVD, and peripheral edema
17
Q
List the 4 heart defects that can present with a murmur and signs of CHF in infancy.
A
- VSD
- Severe aortic stenosis
- Coarctation of the aorta
- Large patent ductus arteriosus
(Most cyanotic heart defects present with cyanosis rather than progressive CHF)
18
Q
How is CHF treated in infants?
A
- First line/most common: Furosemide (Lasix) - diuretic that counteracts the fluid retention caused by activation of RAAS.
- Digoxin: some studies show improvement in infants with CHF due to VSD (mechanism is unclear, as infants with VSD do not have impaired contractlity)
- Enalapril (ACEIs): reduces afterload to decrease systemic vascular resistance and promote forward flow of blood from the left ventricle rather than through the VSD to the pulmonary vasculature
These medications control symptoms and help the child grow - there are no medications to encourage closure of the VSD.
Consider fortifying expressed breast milk or formula to provide greater caloric density.
19
Q
What is the most common cause of a murmur in children? Give a specific statistic.
A
Innocent murmur; occurs in 70-80% of otherwise healthy patients as some point during childhood, particularly between 3-7 years of age. It is by definition normal and is not due to any heart abnormality.
20
Q
What is the most commonly heard innocent murmur? Describe its quality and location.
A
Still’s murmur; Musical or vibratory, heard best at LLSB or in supine position (low-pitched and louder)
(Usually found between 3-6 years, thought to be due to turbulence in LV outflow or vibration of fibrous tituse bands crossing LV lumen)
(Typically grade II-III, midsystolic, LLSB, vibratory, decreases with standing, increases with fever, exercise, anemia)
21
Q
List important questions to consider when deciding if a murmur is innocent.
A
Is the child otherwise well?
Is the precordial activity normal?
Is the second heart sound normally split?
Is the murmur less than or equal to grade II/VI?Is the oxygen saturation normal?
22
Q
Which structural heart defects may present later than infancy?
A
ASD (3-5 years of age)
Coarctation of the aorta (infancy or any age, as it tends to be progressive)
Bicuspid aortic valve
23
Q
What are the findings of an ASD?
A
Systolic ejection murmur, widely split, fixed S2 (best way to distinguish from an innocent murmur)
24
Q
What causes the systolic ejection murmur in an ASD?
A
Increased flow across a normal pulmonary valve
25
What are the findings of coarctation of the aorta?
Murmur (may present medial to the left scapula), hypertension in the upper extremities, discrepancy between upper and lower extremity blood pressures (poor femoral pulses)
26
What are the findings of a VSD?
Holosystolic murmur beginning with S1 with a blowing quality
27
What are the findings of aortic stenosis?
Systolic ejection murmur, radiating to the neck (occasionally a thrill in the jugular notch), with an early systolic click
28
What are the findings of pulmonic stenosis?
Prominent and harsh systolic ejection murmur just after S1, radiating to the lung fields, with an early systolic click
29
What are the findings of a PDA?
Continuous (a bit louder in systole) machine-like murmur, bounding pulses due to a widened pulse pressure
30
What are the findings of tetralogy of fallot?
Systolic ejection murmur radiating to the lung fields (RVOT obstruction)
31
What are the findings of a bicuspid aortic valve?
No murmur if the valve is not stenotic or regurgitant
Early systolic click made by the abnormal valve when it opens (shortly after the first heart sound)
Subtle exam finding
32
Congenital heart defects occur in approximately what percent of newborns?
1%
33
What are the leading cause of early mortality from congenital anomalies?
Congenital CV malformations
34
Isolated VSD accounts for what percent of all congenital heart defects?
15-20%
35
What are the 4 components of Tetralogy?
1. VSD
2. RVOT obstruction
3. Overriding aorta
4. RVH
Causes cyanosis through obstruction of the pulmonary artery and R to L shunting through the VSD; progressive, worsens over time
36
What is the most common heart defect presenting with cyanosis in the newborn period (despite having a lower overall incidence than Tetralogy)?
Transposition of the great vessels (requires urgent intervention)
37
List the 3 general methods for evaluating congenital heart defects.
1. EKG
2. CXR
3. Echo
38
What is seen on EKG with congenital heart defects?
Abnormal EKG demonstrating chamber enlargement; findings not specific to a gien defect
39
What are the typical EKG findings in an infant with a large VSD?
Prominent, biventricular forces (high voltage QRS complexes in leads V1 and V2) suggesting both LV volume overload and RV pressure overload
40
Compare the classic findings on EKG of and large, moderate, and small VSD.
Large -
RVH due to RV pressure overload (pulmonary hypertension) + an upright T wave in V1 (another RVH sign)
Medium - LVH due to LV overload (VSD causes left-heart dilation by increased pulmonary blood flow returning to the left heart, VSD shunt occurs in systole when the right ventricle is also contracting. The right ventricle does not fill with the extra volume and dilate, as the VSD flow is immediately ejected into the pulmonary arteries)
Small - normal
41
True or false - interpreting the EKG in children and infants is complicated by the fact that the normal voltage is very age-dependent.
True; in general, newborns/young infants have a more right ventricular voltage and a more rightward axis (in fetal circulation, the lungs do not contribute to ventilation and the resultant pulmonary vascular resistance is elevated, leading to thickening of the right ventricle)
42
What is seen on CXR to evaluate heart defects?
Heart size and prominence of pulmonary vascular markings
Hallmark of L to R shunt - cardiomegaly, increased pulmonary vascular markings, pulmonary edema
43
When should children be admitted for congenital heart disease?
Children in shock
Severity of illness
Tempo with which the symptoms are evolving
Respiratory effort, lethargy/decreased level of alertness, feeding difficulty, cyanosis
44
Define a VSD.
Any persistent communication between the ventricles occurring in isolation or as part of a more complex defect
45
The ventricular septum is composed of what three distinct structures?
1. Inlet septum (embryologic endocardial cushion)
2. Outlet septum (embryologic conotruncus)
3. Muscular septum (embryologic trabecular septum)
46
Describe the formation of a VSD.
The fusion point of the three structures of the ventricular septum is the membranous septum. VSDs occur due to either a lack of tissue (endocardial cushino defect resulting in an inlet VSD) or a lack of fusion at the septum (resulting in a perimembranous defect)
47
Describe the pathophysiology of a VSD.
L to R shunt during ventricular systole causes increased pulmonary blood flow, increased pulmonary venous return, and resultant left ventricular volume overload
48
When does VSD typically present?
Several days to weeks of age (related to the magnitude of the shunt, which is determined by the size of the defect and the pulmonary vascular resistance)
49
Prognosis of VSD?
Tend to diminish in size with time, spontaneous closure of ~75% of small defects and 25-50% of all defects
50
Why does the VSD murmur not present right away?
Newborns have elevated pulmonary vascular resistance. When this is nearly equal to the systemic vascular resistance, blood does not shunt through the VSD. When the pulmonary vascular resistance drops at a few days to weeks of age, this changes.
51
What is Eisenmenger's syndrome?
Outcome of a patient with unrepaired VSd due to pulmonary vascular obstruction in response to high pressure and high flow. Shunting through the VSD will shift to R to L. The patient will develop cyanosis, progressing to polycythemia, heart failure, and death. Most patients live into their 20s but with a very impaired quality of life.
52
When does Eisenmenger's syndrome develop?
After 6 months of age
53
When should VSD closure be performed?
Large defect + pulmonary hypertension, before 6 months of age
54
Qualities of an innocent vs. pathologic murmur
Innocent - systolic, ejection, soft/vibratory, grade 1-2/6, normal S1/S2, no extra sounds, louder supine
Pathologic - diastolic, holosystolic, harsh, 3+/6 grade, abnormal split S2, extra sounds (click), louder with standing
55
List 5 mechanisms of petechiae and purpura.
1. Trauma
2. Platelet deficiency or dysfunction (e.g., immune-mediated thrombocytopenia, bone marrow infiltration or suppression, malignancy)
3. Coagulation abnormalities (e.g. hereditary or acquired clotting-factor deficiencies)
4. Vascular fragility (e.g., immune-mediated vasculitis)
5. Combinations of the above (e.g., infection causing coagulation abnormalities, vascular fragility, platelet consumption_)
56
DDx - bruising and leg pain (9)
1. Coagulation disorder
2. Henoch-Schnolein purpura (HSP)
3. Idiopathic thrombocytopenic purpura (ITP)
4. Leukemia
5. Viral infection
6. Bacterial endocarditis
7. Meningococcal septicemia
8. Rocky Mountain spotted fever (RMSF)
9. Systemic lupus erythematosus (SLE)
57
How does a coagulation disorder present?
May present with petechiae or superficial bruising, but more often presents with easy bruising in deep tissues or hemarthrosis (painful bleeding into joints)
Family history and/or personal history of bleeding (e.g., after trauma, immunizations, circumcision, dental work)
58
How are bleeding disorders (hemophilias, von Willebrand's disease) characterized?
Easy bruising in response to minor trauma. Spontaneous superficial bruising is less common
59
What is HSP (aka anaphlyactoid purpura)?
Self-limited Ig-A mediated, small vessel vasculitis that typically involves the skin, GI tract, joints, and kidneys
60
How does HSP present?
Hallmark: non-thrombocytopenic purpura; rash tends to involve lower extremities
"Otherwise well-appearing child with bruising and leg pain)
1/3 of children have renal involvement (typically hematuria), less common <2 years
Arthritis or arthralgia due to periarticular vasculitis (mainly of knees and ankles) in 75% of children
Colicky abdominal pain in 65%, 50% may develop intestinal bleeding
Recent URI (66%)
NOT associated with splenomegaly
61
How does ITP present?
Often with asymptomatic, thrombocytopenic purpura and petechiae, non-specific URI preceding >50% of the time
NOT associated with splenomegaly
62
How does leukemia present?
Constitutional symptoms such as fever, malaise, and weight loss
Bone pain is also common in children resulting from infiltration of the bone marrow by the malignant cell
Petechiae can be caused by thrombocytopenia due to bone marrow replacement by malignant cells
Splenomegaly and lymphadenopathy
63
How does viral infection present when causing a petechiael rash?
Some viruses such as enteroviruses may present with a petechial rash
Children usually have a low-grade fever
Other constitutional complains may be present or absent
Prominent coughing and/or vomiting can also cause petechiae, generally above the nipple line
64
How does bacterial endocarditis present?
Fever (usually present but may be low-grade), fatigue, weight loss, petechial rash
Bruising is not characteristic
65
How does meningococcal septicemia present?
Petechiae and purpura
While the early stages may have only mild symptoms, by the time the hemorrhagic rash appears, patients are usually very ill appearing and require emergent care
Fever is usually present
66
How does RMSF present?
Rash is often petechial, starts on the extremities before moving centrally
Fever is a hallmark
67
How does SLE present?
Variable rash
More common in older children and in girls
Often presents with constitutional symptoms such as fever and malaise
68
Lab evaluation of suspected HSP?
1. Platelet count (non-thrombocytopenic [HSP] vs. thrombocytopenic [ITP or leukemia] purpura)
2. Urinarlysis (determine if renal involvement via hematuria/proteinuria)
3. BUN/Cr (if hematuria or proteinuria is present, needed to determine extent of renal disease)
69
What is the most commonly diagnosed form of vasculitis in children? M vs. F?
HSP (~50% of cases)
M>F
70
Hallmark of HSP?
Non-thrombocytopenia purpura
71
Pathologic finding in HSP?
Leukocytoclastic vasculitis with IgA deposition
72
Complications of HSP?
~5% progress to chronic renal failure. Fewer than 1% will develop ESRD.
Although considered a benign childhood disease, it occasionally requires hospitalization for management of severe abdominal pain, GI bleeding, intussusception, and renal involvement
73
Treatment of HSP?
Systematic reviews have not demonstrated a consistent benefit for corticosteroids in preventing serious renal involvement
Early corticosteroids in hospitalized children may confer benefits in reducing GI manifestations
74
Pathophysiology of ITP?
Caused by the binding of an antiplatelet antibody to the platelet surface, leading to removal and destruction of platelets in the spleen an dliver
75
Most common cause of isolated thrombocytopenia in otherwise healthy children?
ITP
76
ITP vs. HSP - epidemiology (M vs. F, peak incidence age, # annually)
ITP: M=F, 2-5 years (range 2-10 years), ~5/100,000
HSP: M>F (2:1), 4-6 years (range 2-17 years), ~10/100,000
77
ITP vs. HSP - pathophysiology and history of URI?
ITP: anti-platelet antibody binds to platelet surface leading to removal and destruction of platelets in the spleen and liver; most cases follow a non-specific viral illness
HSP: underlying mechanism unknown; suspected to represent an Ig-A dominated immune response to infection or other triggers. 50% of cases follow viral/bacterial URIs
78
Clinical features of ITP vs. HSP
ITP: superficial bleeding into the skin (petechiae and bruising), some have evidence of mucosal bleeding; other symptoms/physical findings are generally absent. No HSM on exam.
HSP: skin lesions begin as erythmatous macules or urticarial wheals that evolve into petechiae and palpable purpura. Rash is symmetrically distributed in gravity-dependent o pressure-sensitive areas (lower extremities, elbows), young patients more likely to have involvement of face/upper extremities, colicky diffuse or periumbilical abdominal pain (50-75%), arthritis/arthralgia (40-75%), renal disease (20-50%)
79
ITP vs. HSP - lab findings
ITP - thrombocytopenia (usually <20k) with normal WBC and HgB
HSP - normal platelet count; may have hematuria/proteinuria/affected BUN/Cr, may have blood in stool, coagulation studies should be normal
80
ITP vs. HSP - natural history
ITP: most do not have significant bleeding (3% have severe epistaxis or other mucous membrane hemorrhage), most concerning complication is intracranial hemorrhage (0.1-0.5% of cases)
HSP: all will eventually have skin findings, abdominal pain/joint involvement may precede the rash, recurrence rate is about 30% (symptoms may return weeks to many months after the first episode)
81
ITP vs. HSP - Rx
ITP - observation, oral corticosteroids, IVIg, Rhogam
HSP - no specific therapy, supportive care for symptoms of arthralgia or stomach pain, observation for complications
82
Define shock.
Inadequate delivery of substrates and oxygen to meet the metabolic needs of tissues.
83
Describe the pathophysiology of shock.
As cells are starved of oxygen and substrate, they can no longer sustain metabolism. Eventually, cellular metabolism is no longer able to generate enough energy to power the components of cellular homeostasis, leading to disruption of cell-membrane ionic pumps. The cell swells, the cell membrane breaks down, and cell death occurs.
84
Describe the compensatory mechanisms of shock in children.
Children can maintain a normal BP until they are in profound shock. They have excellent compensatory mechanisms to maintain tissue perfusion that include:
- Tachycardia - because CO is dependent on both SV and HR, the body tries to maintain CO when SV decreases by increasing HR
- Increased heart contractility - results in more complete emptying of ventricles
- Increased venous tones - results in more blood return to the heart
- Tachypnea - attempts to compensate for the metabolic acidosis caused by decreased oxygen perfusion of the tissues and cells
Therefore in early shock you will find the following: elevated HR and RR, peripheral blood vessel constriction (cool, clammy extremities and delayed capillary refill time), and decreased peripheral pulses (due to vasoconstriction and decreased SV). This can lead to difficulty obtaining a pulse ox measurement.
85
Because children compensate so well in early shock ___ is a late sign of shock.
Hypotension
86
List the 4 types of shock.
1. Distributive (includes neurogenic and anaphylactic, some authors include septic in this category as well)
2. Hypovolemic
3. Cardiogenic
4. Septic
87
What causes distributive shock?
Intravascular hypovolemia from vasodilation, increased capillary permeability, and third-space fluid losses
88
What is the most common type of shock worldwide?
Hypovolemic
89
What causes hypovolemic shock?
Inadequate fluid intake to compensate for fluid output (e.g., vomiting, diarrhea, hemorrhage)
90
What causes cardiogenic shock in children (albeit rare)?
Severe congenital heart disease, dysrhythmias, cardiomyopathy, and tamponade
91
What causes septic shock?
Results when infectious organisms release toxins that affect fluid distribution and cardiac output
92
How do patients in septic shock present?
Compensated or "warm" shock, with warm extremities, bounding pulses, tachycardia, tachypnea, adequate urination, mild metabolic acidosis
(second most common cause in children)
93
What is the primary consideration in managing shock?
Maintaining perfusion - intravascular volume replacement is the priority
94
How should fluid replacement be administered in shock?
Isotonic solutions, NOT hypotonic solutions, rate of infusion as fast as possible, even in patients who have a relative contraindication, such as patients with meningitis
General guidelines recommend giving repeated boluses of isotonic fluids up to a total of 60 mL/kg, then starting inotropic support (e.g. with dopamine) if perfusion remains inadequate
95
What is the ideal approach to vascular access in managing a patient in shock?
Place the largest bore catheter possible in the largest, most accessible vein. Peripheral venous access is always the first attempt.
96
In an adolescent or adult patient, placement of a ___ line would be appropriate if peripheral access cannot be established. List common options.
Central venous; femoral, subclavian, or internal jugular
97
In shock, if a peripheral IV line cannot be placed within 90 seconds, what is the fastest way to get access?
Intraosseous line inserted into the marrow cavity of a long bone
98
What technique is often used as a last resort if peripheral venous access, central venous access, and intraosseous access are unavailable or unsuccessful?
Venous cutdown technique on the saphenous vein
99
Why does infusion by IO work?
IO infusion is possible because the veins that drain the medullary sinuses in the bone marrow do not collapse in children with shock or hypovolemia.
100
Blood draining from the bone marrow of the tibia enters the general circulation via the ___ vein, and branches of the ___ vein drain the medullary sinuses of the distal femur.
Popliteal; femoral
101
Discuss the pathophysiology of sickle cell disease.
Mutation: substitution of valine for glutamic acid at the 6th amino acid position of the Hgb molecule
Leads to the formation of polymers of Hgb when Hgb becomes deoxygenated
These polymers lead to deformation of the RBC into sickle cells, which have increased adherence and block blood flow in the microvasculature, which leads to local tissue hypoxia, pain, and tissue damage.
Abnormal Hgb induces hemolysis of the RBCs leading to chronic anemia with an elevation of the reticulocyte count
102
Predominant hemoglobin at birth?
Hemoglobin F (fetal)
103
What always appears first in hemoglobin nomenclature of the newborn?
F (predominant), followed by other Hgb in order of concentration
104
What is Hgb FA?
Fetal + normal adult
105
What is Hgb FAS?
Baby is a carrier of one abnormal hemoglobin gene (S) -> benign sickle cell trait
106
What is Hgb FAC?
Baby is a carrier of one abnormal hemoglobin g (C) -> benign hemoglobin C trait
107
What is Hgb FS?
Both of the baby's hemoglobin genes have mutations for Hgb S
108
What is the most common hemoglobin pattern causing SCD?
FS
109
What is Hgb FSA?
Sickle cell beta thalassemia, meaning one of the globin genes has a mutation for S and the other has a mutation for beta thalassemia (which produces no or little hemoglobin)
110
What is Hgb FSC?
Sickle cell hemoglobin C disease - one gene has the S mutation, one gene has the C mutation
111
List 2 common procedures for young individuals with SCD.
1. Tonsillectomy
| 2. Cholecystectomy
112
Why is a tonsillectomy common in SCD?
Lymphoidal-tissue hypertrophy involving Waldeyer's ring is common in children with SCD, possibly related to desaturation of hemoglobin and increased risk of sickling. Excessive snoring and OSA may be observed.
Tonsillectomy with adenoidectomy will improve OSA in most patients.
113
Why is a cholecystectomy common in SCD?
Bilirubin gallstones occur frequently in all patients with hemolytic anemias, including SCD due to the increased release of Hgb during breakdown of abnormal RBCs.
114
Who should have a cholecystectomy in the setting of SCD?
Patients who are symptomatic, NOT patients who have evidence of gallstones on screening U/S
115
Why is penicillin given to children until ages 5-6 with SCD prophylactically?
To prevent infections that can lead to sepsis (decreases risk of mortality from overwhelming sepsis)
116
Why are children with SCD at greatly increased risk for sepsis?
Decreased splenic function leads to decreased resistance to infection with encapsulated organisms (S. pneumoniae, Hib, N. meningitidis)
117
List the goals for the comprehensive visit for SCD.
1. How the family deals with the various complications that can occur
2. Frequency of painful or other vaso-occulsive problems
3. How the family accesses health care
4. How the family is dealing with a chronic illness
118
List # expected complications for children with SCD.
1. Jaundice (hemolysis)
2. Anemia (can cause some fatigue, can sometimes be more severe due to myelosuppression from infections such as parvovirus or from hypersplenism when the spleen enlarges and traps blodo cells)
3. Stroke (increased risk, 10% by the age of 15 years)
4. Respiratory problems (lungs are a site of occasional sickling problems, can have pneumonia because of increased tendency to infection or acute chest syndrome as a result of vaso-occlusion in the lung parenchyma)
119
Rx - acute chest syndrome
Medical emergency requiring supplemental oxygen and transfusion therapy
120
What is a useful screening tool for predicting the risk of SCD patients for stroke?
Transcranial doppler studies to evaluate cerebral blood flow (recommendd in children 2-15 years)
121
Immunization recommendations for children with SCD?
Hib and 13-valent pneumococcal conjugate vaccine (Prevnar 13) - 2, 4, and 6 months
23-valent pneumococcal polysaccharide vaccine - 2 and 5 years
Meningococcal conjugate at 2 years, booster 3-5 years later
Influenza annually
122
Why are vaccine conjugated for infants?
Children under 2 years have a suboptimal immune response to purely polysaccharide vaccines. Conjugation allows better antibody production.
123
Sickle cell gene mutation gene frequency among African Americans living in the US?
7-10%
124
Inheritance pattern of SCD?
AR
125
Impairment of growth is common in children in SCD. Why?
Combination of chronic anemia, poor nutrition, painful crises, endocrine dysfunction, poor pulmonary function
126
Important signs in the physical exam in a patient with SCD?
1. Splenic enlargement (common during first few years of life, becomes progressively fibrotic, by 4-6 years, no longer palpable)
2. Sclera (icterus?03
3. Neurologic exam (signs of potential stroke)
127
Massive enlargement or rapid change in size of the spleen can indicate what?
Splenic sequestration crisis - life-threatening complication that occurs when blood pools in the spleen, leading to severe anemia and shock
128
Baseline Hgb in SCD?
Children with SCD frequently have baseline Hgb between 6-9 g/dL
129
List reasons to seek emergency care for a patient with SCD.
1. Fever (medical emergency, sometimes the only sign of serious infection)
2. Splenic enlargement (sequestration crisis)
3. Slurred speech (stroke, exchange transfusion may be indicated)
4. Chest pain (+tachypnea - acute chest syndrome)
5. Rapid breathing (ACS)
6. Increased pallor (anemia, non-emergent unless with signs of splenic sequestration, increased hemolysis, etc.)
7. increased jaundice (baseline jaundice is typical, marked increase indicates increased hemolysis and need for transfusion)
8. Priapasm (sickling in penile arteries)
130
What is an aplastic crisis?
Temporary inhibition of erythryoid production frequently associated with viral illness, particularly Parvovirus B19
131
In a child with sickle cell anemia, cough and breathing difficulty may indicate?
Pneumonia
Intrapulmonary sickling
Pulmonary fat embolism
All = ACS
132
In ACS, what three problems must be addressed rapidly and appropriately?
Possible infection
Pulmonary/respiratory complaints
Pain
133
DDx - Fever, Respiratory Distress, and Chest Pain in a child with SCD
1. ACS
2. Pericarditis
3. CHF
4. Rib infarction
5. Sepsis
134
What is one of the most common causes of death in patients with SCD?
ACS
135
Clinical features of ACS?
Fever, cough, chest pain (can lead to difficulty with expansion of the lower lung and result in atelectasis), SOB, decreased oxygenation
136
Clinical features of pericarditis?
Tachypnea and fever
| Uncommonly causes chest pain
137
Clinical features of CHF?
Can occur in patients with SCD and chronic anemia
Tachypnea (common sign)
Usually does not cause chest pain on its own
138
CXR findings of ACS
Findings may be due either to fat embolism or to vaso-occlusion of pulmonary vasculature
Include multilobular infiltrates (more commonly lower and middle), effusions, atelectasis
It is always difficult to distinguish whether an infiltrate represents an infectoius process or atelectasis from infarction/pulmonary fat embolusm
139
CXR findings of pericarditis
Effusion and infiltrate
140
CXR findings of CHF
Lower lobe infiltrates and cardiomegaly
141
CXR findings of rib infarction
No pulmonary infiltrate, pleural effusion is possibe
142
CXR findings of sepsis
Cardiomegaly only if patient in cardiogenic shock (cardiomegaly may be patient's baseline)
143
Patients with SCD frequently demonstrate what CBC findings in response to stress?
Exaggerated eukocytosis and thrombocytosis
144
Rx - painful crises +/- fever or pulmunary event
IV fluids, NSAIDs, IV narcotics
145
Hydration of children with SCD?
Vigorously hydrate children with SCD because dehydration may contribute to intracellular Hgb polymerization and RBC sickling
With ACS, however, we must be careful to avoid being too vigorous and cause pulmonary edema
146
What is atelectais?
A collapsed or airless segment of lung due to obstruction with distal collapse of the alveoli
147
What is a serious risk in a child with chest pain?
Atelectasis
148
How can atelectasis be prevented?
Encourage deep breathing, often through the use of assistive devices like an incentive spirometer
149
Rx ACS?
RBC transfusion is the only way to directly reduce or reverse the sickling process causing the ACS
150
When is RBC transfusion recommended in ACS?
```
Fall in Hgb from baseline
Increasing RR
Worsening chest symptoms
Declining O2 sats
Progressive infiltrates on CXR
```
151
What is the only known curative therapy for SCD?
HSC transplantation
