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SBL 1 > Pediatric Cases HYHO > Flashcards

Pediatric Cases HYHO Flashcards

1
Q

When does the physiologic NADIR for hemoglobin in infants occur?

A

2 months of age

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2
Q

What does anemia do to the oxygen dissociation curve?

A

shifts it to the right because the concentration of 2,3DPG increases within the RBC

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3
Q

Why is a slower developing anemia better than a rapidly developing one?

A

The body can compensate better if it develops slowly, a rapid developing anemia results in dramatic symptoms

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4
Q

What does a low retic count indicate?

A

inadequate bone marrow response due to bone marrow failure or ineffective erythropoiesis

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5
Q

What does a high retic count indicate?

A

hemolysis of RBC, sequestration, or blood loss

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6
Q

What is Diamond Blackfan syndrome?

A

Congenital pure red cell aplasia that presents in infancy with increased apoptosis in erythroid precursors and macrocytic anemia with a low retic count

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7
Q

What is the most common inherited form of aplastic anemia?

A

Fanconi

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8
Q

Describe fanconi anemia.

A

Macrocytic anemia with a low retic count, leukopenia, thrombocytopenia and increased apoptosis in the bone marrow that can progress to pancytopenia. May not present until 10 yo

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9
Q

In toddlers what is the most common microcytic hypochromic anemia? How will they present?

A

iron deficiency caused by dietary deficiency pale, big on drinking cows milk You will see target cells on peripheral smear, iron ferritin and iron saturation are all low and transferrin is elevated

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10
Q

What is the Mentzer index?

A

Useful in distinguishing mild IDA from beta thalassemia trait in peds patients with a mild microcytic anemia

  • >13 IDA is more likely
  • <13 Thalassemia more likely
  • = 13 indeterminate
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11
Q

How do you calculate the ANC when given a CBC?

A

ANC= (%neutrophils + %bands) x (WBC) / 100

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12
Q

What is classified as a mild, moderate, and severe neutropenia?

A
  • mild: 1000-1500
  • Moderate: 500-1000
  • Severe: <500
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13
Q

What class of drugs are most associtated with neutropenia?

A

Chemotherapeutics

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14
Q

Describe Kostmann syndrome

A
  • Life threatening pyogenic infections early in life
  • Imparied myeloid differentiation caused by maturational arrest of neutrophil precursors
  • ANC <200
  • AR
  • Increased risk AML
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15
Q

Describe Cyclic Neutropenia. (Red on handout)

A
  • Cyclic fever, oral ulcers, gingivitis, periodontal disease, recurrent bacterial infections
  • Stem cell regulatory defect resutling in defective maturation
  • ANC <200 for 3-7 days every 15-35 days
  • No increased risk for malignancy
  • AD or sporadic
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16
Q

Describe Schwachman diamond syndrome.

A
  • Triad of neutropenia, exocrine pancreas insufficiency, skeletal abormalities
  • defects in neutrophil mobility, migration and chemotaxis in addition to neutropenia
  • AR
  • increased risk for MDS or leukemia
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17
Q

Fanconi Anemia (Red on handout)

A
  • Bone marrow failure syndrome with all cells lines affected
  • GU and skeletal anomalies
  • Increased chromosome fragility
  • AR usually seen in first 10 yrs of life
  • classic ex of congenital pancytopenia/bone marrow failure/aplastic anemia
  • Increased risk for AML, brain tumors, and Wilms tumor
18
Q

Leukocyte adhesion deficiency

A
  • Very rare
  • Results in delayed separation of umbilical cord and recurrent severe bacterial fungal infections without pus
  • poor wound healing
  • Neutrophils have diminished adhesion to surfaces
  • Autosomal recessive
  • No risk of malignancy
19
Q

Chediak Higashi syndrome?

A
  • partial oculocutaneous albinism, neuropathies, and recurrent pyogenic infections
  • Defects are in chemotaxis and ddegranulation resulting inineffective granulopoiesis
  • Very rare AR
  • Fatal by 20 yo
  • no risk malignancy
20
Q

Chronic granulomatous disease (red in handout)

A
  • Recurrent prulent infections with fungal or bacterial catalase positive organisms
    • starts in infancy and you seen inflamatory granulomas
  • Defect is in oxidative metabolism and there is abssent superoxide
  • X linked recessive
  • no risk of malignancy
21
Q

What is the number one cause of death by disease in kids in the US?

A
  • Brain cancer followed by leukemia
  • overall #1 death is accidents
22
Q

What is the most common malignancy in 15-19 yo and infectious agents implicated with its development?

A

Hodgkin Lymphoma and HHV-6, CMV, and EBV are implicated with its development

23
Q

What are Reed Sterngburg cells indicitave of?

A

HL

24
Q

What are B signs used in staging of HL?

A
  • Unexplained fever >39
  • Weight loss >10% over 6 months
  • Drenching night sweats
25
Q

In a patient with lymphadenopath, what are the indications for a chest x ray?

A
  • any patient with persistent unexplained lymphadenopathy unassociated with an obviouos underlying inflammatory or infectious process
  • Any patient with persistenet LAD and accompanyting respiratory symptoms
  • Lymphoma prefers the mediastinum
26
Q

Most common type of lymphoma in adolescents and kids?

A

non hodgkin

27
Q

Wiscott Aldrich syndrome presisposes the patient to the development of what kind of cancer?

A
  • B cell lymphomas
28
Q

What is the triad of symptoms for Wiscott Aldrich syndrome?

A
  • X linked recesive
  • Recurrent sino pulmonary and ear infections
  • Severe atopic dermatitis
  • Bleeding secondary to significant thrombocytopenia
29
Q

What accounts for the greatest percentage of childhood malignancies? (___ malignancies are the #1 cause of death, but #1 cause of malignancy is ___.)

A
  • Leukemias
  • Brain malignancies are the #1 cause of death, but #1 cause of malignancy is leukemia
30
Q

Down syndrome is associated with what leukemias?

A

ALL and AML

31
Q
A
32
Q

Should you administer corticosteroids before doing a bone marrow biopsy as an immediate treatment?

A

No, they can alter the appearance of the bone marrow a they modulate immune responses

33
Q

What are the DDx for a child who appears well with moderate to severe thrombocytopenia and an otherwise normal CBC?

A
  • ITP
  • Medication
  • Undiagnosed portal hypertension
  • Early aplastic process (rare, such as fanconi anemia)
34
Q

Young boy comes to your office complaining about an itchy rash on his hands and arms. His mom informs you that his main complaint however is his headache with sinus pressure. She believes this is his 6th sinus infection in 3 months. What is the underlying cause of his issues?

A

Wiscott-Aldrich Syndrome

35
Q

How will a CBC look in a patient with Immune thrombocytopenic purpura?

A
  • Platelet count is below 20,000 and other cell lines are normal (typically)
  • If other cell lines affefcted consider bone marrow aspirate
36
Q

What platelet disorder followsa viral infection by 1-3 weeks?

A

Immune thrombocytopenic purpura

37
Q

What is Kaswabach Merritt phenomenon?

A
  • Thrombocytopenia and hypo-fibrinogenemia associated with giant hemangioma and assoc. intravascular coagulation
  • Rare
38
Q

Red flag symptoms in patients with thrombocytopenia?

A
  • Evidence of pancytopenia
  • Raised LDH
  • Assoc. new renal impairment
39
Q

Acute vs Chronic ITP

A

Acute:

  • Occurs 2-6 yo
  • infection common 1-3 prior
  • <20,000 platelet
  • Eosinophila/lymphocytosis common
  • lasts 2-6 weeks rarely longer
  • spontaneous remission occurs in 80%

Chronic:

  • occurs 20-40 yo
  • unusual to have prior infection
  • 30,000-80,000 platelet
  • Rare eosinophila/lymphocytosis
  • Months or years duration
  • Uncommon for spontaneous remission
40
Q

Classic triad for HUS?

A
  • Microangiopathic hemolytic anemia
  • Thrombocytopenia
  • Acute renal damage/failure
41
Q

What causes most cases of HUS in US?

A

E.coli O157:H7 which produces the shiga toxin causing bloody diarrhea

42
Q

Prognosis of HUS?

A
  • Blood is always in the urine, but if protein is as well it is a worse outcome

SBL 1 (8 decks)

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