Dr. Newman Stuff Flashcards
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?
Strep Pneumoniae
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
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
What is the Mentzer Index?
- distinguishes Iron Deficiency Anemia from Beta Thalassemia
MCV/RBC
- < 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
Moderate 500-1000
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
What is Kasabach-Merritt phenomenon?
- thrombocytopenia and hypo-fibrinogenemia associated with GIANT HEMANGIOMA
