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Flashcards in Dr. Newman Stuff Deck (21)
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1

What are the two most important parts of the workup for child pts. with Lymphadenopathy?

History and Physical

2

When is a CXR/Chest CT indicated? What is the best way to look at LN's?

- indicated in pts. with swollen LNs and respiratory problems

- excisional biopsy is the best way to look at LNs

3

What is the most common cause of pneumonia and bacterial otitis media in children?

Strep Pneumoniae

4

What is the most common tumor-like lesion of infancy and what Syndrome is it associated with?

- Hemangioma (Cavernous or Capillary)

- Sturge Weber Syndrome
- facial port wine stain
- developmental delay/intellectual disability

- lesion usually regresses with age but stains are faulry permanent

5

What is a Neuroblastoma?

- tumor of sympathetic ganglia and adrenal medulla

- most common extracranial tumor of childhood (18 mos)

- produce excessive catecholamines (inc. VMA/HVA in the urine) and potential "Blueberry Muffin Baby"

6

What is a Wilm's Tumor and what is a major congenital malformation it is associated with?

- most common primary renal tumor of childhood (2-5 yo)

- Beckwith-Wiedemann Syndrome
- macroglossia, organ enlargement, omphalocele

7

What is the most common malignancy in kids (< 10 yo) and adolescents (15-19 yo)?

kids = LEUKEMIA

adolescents = HODGKINS LYMPHOMA (EBV)

8

Diamond Blackfan Anemia

- congenital pure RBC aplasia that presents in infancy

- causes inc. apoptosis in erythroid precursors

- macrocytic with dec. reticulocyte counts

9

Fanconi Anemia

- most common inherited form of aplastic anemia (inc. chromosome fragility)

- increased apoptosis in bone marrow that progresses to pancytopenia (inc. AML, brain tumor, Wilms)

- may be 10 yo old before symptoms present

10

What is the Mentzer Index?

- distinguishes Iron Deficiency Anemia from Beta Thalassemia

MCV/RBC
- < 13 = IDA
- > 13 = Thalassemia

11

How is the Absolute Neutrophil Count calculated and what are the ranges for Mild, Moderate, and Severe neutropenia?

(% neutrophils + % bands) x WBC / 100

Mild = 1000-15000
Moderate 500-1000
Severe < 500

12

What is Kostmann Syndrome? What is its inheritance?

- impaired myeloid differentiation via arrest of neutrophil precursors

- life-threatening pyrogenic infections in early life

- auto recessive (inc. AML risk)

13

What is Cyclic Neutropenia? What is its inheritance?

- stem cell regulatory defects resulting in defective maturation

- cyclic fever, oral ulcers, recurrent bacterial infections

- auto dominant

14

What is Schwachmann-Diamond Syndrome? What is its inheritance?

- defective neutrophil mobility, migration, chemotaxis

- neutropenia, exocrine pancreas insufficiency, skeletal abnormalities

- auto recessive (inc. MDS or leukemia)

15

What is Leukocyte Adhesion Deficiency? What is its inheritance?

- delayed umbilical cord separation (> 3 wks), recurrent infection W/O pus, poor wound healing

- auto recessive

16

What is Hyper-immunoglobulin E Syndrome (Job Syndrome)? What is its inheritance?

- severe eczema, recurrent bacterial skin infections, recurrent pulmonary infections

- inc. IgE/eosinophilia, dec. neutrophil chemotaxis

- auto dominant (inc. Hodgkins)

17

What is Chediak-Higashi Syndrome? What is its inheritance?

- partial albinism, neuropathies, pyrogenic infections

- defects in degranulation, ineffective granulopoiesis

- auto recessive

18

What is Chronic Granulomatous Disease? What is its inheritance?

- recurrent purulent infections with catalase positive organisms

- defective oxidative metabolism, no superoxide

- x-linked recessive

19

What is Wiscott-Aldrich Syndrome? What is its inheritance?

- recurrent sino-pulmonary/ear infections, severe atopic dermatitis, bleeding due to thrombocytopenia

- x-linked recessive

20

What is Immune Thrombocytopenic Purpura?

- anti-platelet Abs usually following viral infections 1-3 weeks after (50%)

- platelets below 20,000 with other cells normal (LARGE platelets)

- will normally self resolve; PT/PTT normal; can use prednisone if severe enough

21

What is Kasabach-Merritt phenomenon?

- thrombocytopenia and hypo-fibrinogenemia associated with GIANT HEMANGIOMA