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PPOM1 Block 4 > Lecture 162 > Flashcards

Lecture 162 Flashcards

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1
Q

At what hemoglobin levels do clinical features in children typically appear?

A

Hb < 8-9g/dL

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2
Q

What signs suggest something more than just iron deficiency anemia?

A

Bone pain, homlysis, jaundice, splenomegaly, lymphadenopathy, systemic complaints

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3
Q

A decline in Hb that occurs during the first 6-8 weeks of life

A

Physiologic anemia of infancy

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4
Q

What is the pathophysiology of physiologic anemia of infancy?

A

Onset of respiration at brith downregulates EPO

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5
Q

What is the typical course for physiologic anemia of infancy?

A

Self-limited and self-correcting

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6
Q

Transient suppression of RBC production in children, leading to reticulocytopenia and normocytic anemia

A

Transient erythroblastopenia of childhood (TEC)

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7
Q

What precipitates TEC?

A

Viral illness, 2-3 months prior

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8
Q

What is the course of TEC?

A

No treatment, self-limiting if no red flags are present

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9
Q

Excessive cow’s milk intake in pediatric patients can cause ____

A

Iron deficiency anemia (IDA)

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10
Q

What labs should be run when IDA is suspected?

A

Lead, CBC, iron studies

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11
Q

During iron replacement therapy, during what time frame does replacement of intracellular iron enzymes, subjective improvement, and increases serum iron take place?

A

12-24 hours

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12
Q

During iron replacement therapy, during what time frame does an initial bone marrow response and erythroid hyperplasia take place?

A

36 to 48 hours

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13
Q

During iron replacement therapy, during what time frame does reticulocytosis take place?

A

48-72 hours

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14
Q

During iron replacement therapy, during what time frame does increase in Hb levels, MCV, and ferritin take place?

A

1-3 months

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15
Q

An inherited disorder of flobin chain synthesis, resulting in microcytic anemia

A

Thalassemia

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16
Q

Deletion of 1 to 4 alpha-globin genes

A

Alpha thalassemia

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17
Q

Mutations in beta-globin genes

A

Beta-thalassemia

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18
Q

What clinical spectrum of thalassemia is often asymptomatic with mild microcytic anemia?

A

Minor (trait) thalassemia

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19
Q

What clinical spectrum of thalassemia displays moderate anemia and may require occasional transfusions?

A

Intermedia thalassemia

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20
Q

What clinical spectrum of thalassemia idisplays severe anemia, is transfusion dependent, and can lead to skeletal deformities?

A

Major thalassemia (Cooley’s anemia)

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21
Q

Autosomal recessive disorder involving a G6V point mutation resulting in hemoglobin S

A

Sickle cell anemia (SCA)

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22
Q

In a sickle cell blood smear, what is seen aside from sickled cells?

A

Howell-Jolly bodies and target cells

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23
Q

Why does SCD typically present around 3-6 months of age?

A

That’s when protective fetal hemoglobin (HbF) level decline

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24
Q

New pulmonary infiltrate on chest x-ray of an SCD patient accompanied by respiratory symptoms

A

Acute chest syndrome (ACS)

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25
Dactylitis, AKA
Hand-Foot syndrome
26
Sickled cells block splenic outflow, leading to rapid pooling of blood in the spleen in SCD
Acute splenic sequestration crisis
27
Aplastic crisis in SCD is often triggered by ____
Parovirus B19 infection
28
Why does Parovirus B19 infection is SCD cause an aplstic crisis?
Causes a temporary arrest of RBC production
29
Kay lab finding in aplastic crisis
Very low reticulocyte count
30
How is SCD treated?
Hydroxyurea to increase HbF production
31
What factor deficiency causes hemophilia A?
Factor VIII
32
What factor deficiency causes hemophilia B?
Factor IX deficiency
33
What is the inheritance pattern of hemophilia A & B?
X-linked recessive
34
What cascade is responsible for inflammation, vasodilation, permeability, and pain?
Kinin cascade
35
What system is responsible for breaking down fibrin and turning plasminogen to plasmic?
Fibrinolytic system
36
VKDB
Vitamin K deficient bleeding
37
A bleeding disorder in newborns caused by vitamin K deficiency, leading to impaired coagulation
Hemorrhagic disease of the newborn
38
Causes of low vitamin K in newborns
Poor placental transfer, sterile gut, and immature liver
39
What drugs are risk factors for hemorrhagic disease of the newborn?
Anticonvulsants or tuberculosis drugs
40
What are the typical PT, PTT, and platelet values in VKDB?
Prolonged PT, normal PTT, and normal platelets
41
Anti-platelet autoantibodies form and bind to the platelet surface, following a viral illness 11-4 weeks prior
Immune thrombocytopenic purpura (ITP)
42
What is the main clinical presentation of ITP?
Petechiae (small, non-blanching macular rash) and purpura on the skin and mucous membranes
43
What is the main laboratory finding in ITP?
Trombocytopenia
44
How is ITP typically treated?
Most cases self-limited and resolve spontaneously, IVIG is a possible treatment
45
What is the first sign of hemophilia?
Severe or persistent bleeding after cicumcision
46
What is a hallmark sign of hemophilia?
Hemarthrosis (bleeding into joints)
47
What is a typical complaint of patients with hemathrosis?
Warm, tingling sensation in the joint
48
What complication of hemophilia can lead to large blood accumulation causing vague groin pain?
Iliopsoas muscle hemorrhage
49
What are the typical PT, PTT, and platelet values in hemophilia?
Normal PT, prolonged PTT, and normal platelets
50
What is desmopressin (DDAVP) used to treat?
Hemophilia A
51
What is desmopressin's MOA?
Promotes Factor VIII release
52
What is the MOA of emicizumab?
Monoclonal antibody that bridges Factor IXa and factor X, mimicking function of Factor VIIIa
53
What is emicizumab used to treat?
Hemophilia A
54
What factor aids in platelet adhesion and protects factor VIII from degradation?
von Willebrand factor (vWF)
55
What is the inheritance pattern of vWD?
Autosomal dominant (AD)
56
How is vWD diagnosed?
vWF antigen and ristocetin cofactor activity
57
What are the typical PT, PTT, and bleeding time values in vWD?
Normal PT, increased PTT, increased bleeding time
58
How is vWD treated?
Desmopressin, FFP
59
ALL
Acute lymphoblastic leukemia
60
AML
Acute myelogenous leukemia
61
CML
Chronic myelogenous leukemia
62
Juvenile myelomonocytic leukemia
JMML
63
What cell type is most commonly implicated in pediatric ALL?
B-lymphoblastic
64
What is the peak incidence of AML?
15-19 years old
65
What childood leukemia presents with "blueberry muffin" appearance? (purplish skin nodules from dermal infiltration by leukemic cells)
AML
66
What type of cells are present in a peripheral smear of pediatric acute leukemia?
Blasts (immature white blood cells)
67
Auer rods and MPO positive are markers for ____
AML
68
TdT positive is a marker for ____
ALL
69
What leukemia treatment complication results in hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia?
Tumor lysis syndrome (TLS)
70
Pediatric leukemia treatment can cause hyperleukocytosis, leading to what complication?
White thrombi of leukocyte aggregates
71
What complication is common in T-ALL?
Superior vena cava syndrome due to anterior mediastinal mass
72
Tumor derived fom primordial crest cells of the sympathetic nervous system
Neuroblastoma
73
Median age at diagnosis of neuroblastoma?
22 months
74
What is the most common site for neuroblastomas?
Abdomen/adrenal gland
75
Periorbital metastases in neuroblastoma can cause what clinical signs?
Ecchymoses (raccoon eyes) and proptosis (bulging eyes)
76
Rapid, uncontrollable jerking eye movements, myoclonic jerks of the body, ataxia, and cognitive dysfunction associagted with neuroblastoma
Opsoclonus-myoclonus-ataxia syndrome (OMAS)
77
OMAS is a paraneoplastic syndrome associated with ____
Neuroblastoma
78
What paraneoplastic syndrome associated with neuroblastoma causes profound secretory diarrhea?
Vasoactive intestinal peptide (VIP) secretion
79
What paraneoplastic syndrome associated with neuroblastoma can cause sweating and hypertension?
Catecholamine secretion
80
Small, round blue cells and Homer-Wright rosettes are histological findings consistent with what neoplasm?
Neuroblastoma
81
Elevated HVA and VMA are catecholamine metabolites seen in what neoplasm?
Neuroblastoma
82
Wilms tumor, AKA
Nephroblastoma
83
Peack incidence of nephroblastoma
2-3 years
84
What is the hallmark symptom of nephroblastoma?
Asymptomatic abdominal mass
85
What is typical of the positioning of abdominal nephroblastoma masses??
Does not cross the midline
86
A triphasic pattern with blastemal, stromal, and epithelial cells histological findings consistent with what neoplasm?
Nephroblastoma
87
Mutations in the RB1 tumor suppressor gene is associated what neoplasm?
Retinoblastoma
88
What is leukocoria?
White pupillary reflex
89
Small, round blue cell with rosette formation (Flexnew-Wintersteiner rosettes) are histological findings consistent with what neoplasm?
Retinoblastoma
90
Malignant neoplasm of primitive neuroectodermal cells, primarily affecting bone but also soft tissue
Ewing sarcoma
91
t(11;22) translocation involving the EWSR1 gene and the FLI1 gene is associated with what neoplasm?
Ewing sarcoma
92
What type of bone pain is characteristic of Ewing sarcoma?
Bone pain that is worse at night
93
What can Ewing's sarcoma be mistaken for?
Sports injury
94
What do "onion skinning" or "moth-eaten" radiograph findings indicate?
Ewing's sarcoma
95
What histological findings are typical in Ewing's sarcoma?
Sheets of primitive, small, round blue cells
96
Malignant bone neoplasm arising from osteoblasts
Osteosarcoma
97
What is the highest risk period for osteosarcoma?
Adolescent growth spurt
98
What neoplasm is associated with mutations in RB1 and TP53
Osteosarcoma
99
Pathologic fracture is common in what pediatric neoplasm?
Osteosarcoma
100
What location is typically affected by osteosarcoma?
Metaphysis of long bones
101
Biopsy showing pleomorphic, osteoid-producing cells is indicative of what neoplasm?
Osteosarcoma
102
What neoplasms presents as a "sunburst pattern" or Codman's triangle on radiographs?
Osteosarcoma

PPOM1 Block 4 (44 decks)

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