Dr. Newman Stuff

Question 1

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

Answer 1

History and Physical

Question 2

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

Answer 2

• indicated in pts. with swollen LNs and respiratory problems
• excisional biopsy is the best way to look at LNs

Question 3

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

Answer 3

Strep Pneumoniae

Question 4

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

Answer 4

• 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

Question 5

What is a Neuroblastoma?

Answer 5

• 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”

Question 6

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

Answer 6

• most common primary renal tumor of childhood (2-5 yo)
• Beckwith-Wiedemann Syndrome
  • macroglossia, organ enlargement, omphalocele

Question 7

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

Answer 7

kids = LEUKEMIA

adolescents = HODGKINS LYMPHOMA (EBV)

Question 8

Diamond Blackfan Anemia

Answer 8

• congenital pure RBC aplasia that presents in infancy
• causes inc. apoptosis in erythroid precursors
• macrocytic with dec. reticulocyte counts

Question 9

Fanconi Anemia

Answer 9

• 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

Question 10

What is the Mentzer Index?

Answer 10

• distinguishes Iron Deficiency Anemia from Beta Thalassemia

MCV/RBC

• < 13 = IDA
• > 13 = Thalassemia

Question 11

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

Answer 11

(% neutrophils + % bands) x WBC / 100

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

Question 12

What is Kostmann Syndrome? What is its inheritance?

Answer 12

• impaired myeloid differentiation via arrest of neutrophil precursors
• life-threatening pyrogenic infections in early life
• auto recessive (inc. AML risk)

Question 13

What is Cyclic Neutropenia? What is its inheritance?

Answer 13

• stem cell regulatory defects resulting in defective maturation
• cyclic fever, oral ulcers, recurrent bacterial infections
• auto dominant

Question 14

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

Answer 14

• defective neutrophil mobility, migration, chemotaxis
• neutropenia, exocrine pancreas insufficiency, skeletal abnormalities
• auto recessive (inc. MDS or leukemia)

Question 15

What is Leukocyte Adhesion Deficiency? What is its inheritance?

Answer 15

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

Question 16

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

Answer 16

• severe eczema, recurrent bacterial skin infections, recurrent pulmonary infections
• inc. IgE/eosinophilia, dec. neutrophil chemotaxis
• auto dominant (inc. Hodgkins)

Question 17

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

Answer 17

• partial albinism, neuropathies, pyrogenic infections
• defects in degranulation, ineffective granulopoiesis
• auto recessive

Question 18

What is Chronic Granulomatous Disease? What is its inheritance?

Answer 18

• recurrent purulent infections with catalase positive organisms
• defective oxidative metabolism, no superoxide
• x-linked recessive

Question 19

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

Answer 19

• recurrent sino-pulmonary/ear infections, severe atopic dermatitis, bleeding due to thrombocytopenia
• x-linked recessive

Question 20

What is Immune Thrombocytopenic Purpura?

Answer 20

• 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

Question 21

What is Kasabach-Merritt phenomenon?

Answer 21

• thrombocytopenia and hypo-fibrinogenemia associated with GIANT HEMANGIOMA