Flashcards in Dr. Newman Stuff Deck (21)
What are the two most important parts of the workup for child pts. with Lymphadenopathy?
History and Physical
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
What is the most common cause of pneumonia and bacterial otitis media in children?
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
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"
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
What is the most common malignancy in kids (< 10 yo) and adolescents (15-19 yo)?
kids = LEUKEMIA
adolescents = HODGKINS LYMPHOMA (EBV)
Diamond Blackfan Anemia
- congenital pure RBC aplasia that presents in infancy
- causes inc. apoptosis in erythroid precursors
- macrocytic with dec. reticulocyte counts
- 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
What is the Mentzer Index?
- distinguishes Iron Deficiency Anemia from Beta Thalassemia
- < 13 = IDA
- > 13 = Thalassemia
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
Severe < 500
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)
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
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)
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
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)
What is Chediak-Higashi Syndrome? What is its inheritance?
- partial albinism, neuropathies, pyrogenic infections
- defects in degranulation, ineffective granulopoiesis
- auto recessive
What is Chronic Granulomatous Disease? What is its inheritance?
- recurrent purulent infections with catalase positive organisms
- defective oxidative metabolism, no superoxide
- x-linked recessive
What is Wiscott-Aldrich Syndrome? What is its inheritance?
- recurrent sino-pulmonary/ear infections, severe atopic dermatitis, bleeding due to thrombocytopenia
- x-linked recessive
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