Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics final > Rheumatic diseases > Flashcards

Rheumatic diseases Flashcards

1
Q

Juvenile idiopathic arthritis characteristics (2)

A

chronic arthritis in:
1. one or more joints
2. for at least 6 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Subtypes of JIA (4)

A

1) oligoarticular,
2) polyarticular,
3) systemic,
4) enthesitis-associated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Clinical findings Oligoarticular form (5)

A
  • 4 or less joints
  • medium and large joints
  • often asymmetric
  • may develop leg length discrepancy in which the involved leg is longer
  • mild and painless synovitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Ophthalmologic screening

A

Routine ophthalmologic screening with slit-lamp examination must be performed at 3-month intervals if the antinuclear antibody (ANA) test is positive, and at 6-month intervals the ANA test is negative, for at least 4 years after the onset of arthritis, as this is the period of highest risk.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Clinical findings Polyarticular form

A
  • 5 or more joints
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Clinical findings Systemic form (6)

A
  • low grade fever, fatigue, rheumatoid nodules and anaemia
  • other systemic features: hepatosplenomegaly, lymphadenopathy, leukocytosis, serositis
  • further divided into RF positive and RF negative
  • systemic form is called Still disease
  • both small and large joints are affected
  • classic feature is high fever 39-40 C 1-2x per day
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Clinical findings Entesitis-associated arthritis

A
  • most common in males above 10
  • lower extremity large joints
  • hallmark is inflammation of tendineous insertions (enthesopathy)
  • psoriasis, nail pitting, dactylitis (sausage digit)
  • there is no specific lab test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Imaging arthritis (3)

A

In the early stages of the disease, only soft tissue swelling and possibly periarticular osteoporosis may be seen.

MRI + gadolinium - early joint damage and synovitis

Later in disease, plain films may show joint space narrowing and erosive changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Treatment objectives (4)

A

to restore function,
relieve pain,
maintain joint motion prevent damage to cartilage and bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Medication

A
  • NSAIDs: naproxen (10mg/kg 2xd), ibuprofen (10mg/kg 3-4xd)
  • Disease-modifying and biologic agents: methotrexate (5-10mg wk or 1mg/kg/wk), etanercept, infliximab, adalimumab
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Systemic lupus erythematosus (2)

A
  • prototype of immune complex diseases
  • its pathogenesis is related to the formation of antibody-antigen complexes that exist in the circulation and deposit in the involved tissues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Which 2 immunological molecules may initiate SLE?

A
  • autoreactive T lymphocytes
  • unregulated B lymphocytes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Symptoms and signs of SLE (11)

A
  1. malar rash (butterfly rash)
  2. discoid rash (annular)
  3. photosensitivity
  4. mucous membrane ulcers
  5. arthritis
  6. serositis
  7. renal abnormalities (proteinuria >0.5 g/day)
  8. neurologic abnormalities
  9. blood count abnormalities
  10. positive ANA
  11. autoantibodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Lab findings in SLE (6)

A
  1. CBC abnormalities: leukopenia, anemia, thrombocytopenia
  2. electrolyte disturbances
  3. raised ESR
  4. proteinuria, hematuria
  5. immune complex disease: C3 and C4 are low
  6. ANA test positive in almost 100%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment of SLE (7)

A
  • prednisone
  • methylprednisone 30 mg/kg (max 1000mg) daily for 3 days, then 2mg/kg
  • hydroxychloroquine 5-7 mg/kg/d orally
  • NSAIDs
  • If disease control is inadequate with prednisone paring agent, such as mycophenolate mofetil, azathioprine, and cyclophosphamide, should be added
  • rituximab
  • aspirin in patients with antiphospholipid antibodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Some of the toxicities of SLE treatment

A

growth failure
osteoporosis
Cushing’s syndrome
infections with high doses of steroids
liver damage and bone marrow suppression with azathioprine
hydroxychloroquine retinal damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Vasculitis definition

A

The vasculitides are a group of conditions that involve inflammation of blood vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Most and least common forms of vasculitis in children (4)

A

Most common:
- Henoch-Schonlein Purpura
- Kawasaki disease

Least common:
- granulomatosis with polyangiitis
- microscopic polyangiitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Signs and symptoms of vasulitis

A
  • fever, fatigue, weight loss, inflammation
  • GWP form: nephritis and lung involvement
  • PAN: skin lesions
  • MPA: pulmonary-renal syndrome
20
Q

Lab findings in vasculitis

A
  • elevated inflammation markrs
  • elevated renal function tests
  • abnormal urinary sedimentation
  • anemia
  • ANCA bodies
  • c-ANCA bodies (specific for GPA)
  • p-ANCA (specific for HSP)
21
Q

Biopsy and angiogram findings

A

Biopsy
in patients with GPA - necrotizing granulomatous vasculitis of involved areas will confirm the presence of vasculitis in small vessels
in patients with MPA and small and medium arteries in PAN.

Angiogram
shows inflammation of affected blood vessels

22
Q

Treatment vasculitis

A
  • corticosteroids
  • methylprednisone 30mg/kg per dose daily for 3 days, then 2 mg/kg/d prednisone
  • cyclophospamide
  • methotrexate
  • azatioprine
  • rituximab
23
Q

Constitutional aplastic anemia (Fanconi anemia)

A

Number 1 inherited (autosomal recessive) bone marrow failure syndrome, characterised by defective DNA repair

24
Q

Some cllinical findings in Fanconi anemia

A
  • thrombocytopenia (purpura, petechie, bleeding)
  • neutropenia
  • anemia
  • congenital anomalies
  • hyper and hypo skin pigmentation
  • microcephaly
  • strabismus etc.
25
Q

Lab findings in Fanconi anemia (7)

A
  • thrombocytopenia
  • leukopenia
  • later anemia
  • macrocytosis
  • anisocytosis
  • elevation in fetal hemoglobin levels
  • bone marrow hypoplasia/aplasia
26
Q

Confirmation of diagnosis of Fanconi anemia is done by (2)

A

The diagnosis is confirmed by the demonstration of an increased number of chromosome breaks and rearrangements in peripheral blood lymphocytes!

27
Q

Complications in Fanconi anemia (4)

A
  • thrombocytopenia
  • neutropenia
  • endocrine dysfunction
  • risk for malignancies (especially acute non-lymphocytic leukaemia)
28
Q

Treatment of Fanconi anemia (3)

A
  • parenteral broad-spectrum antibiotics
  • transfusions (be caregul of refractory thrombocytopenia due to alloimmunization in platelet transfusion)
  • immunomodulation (antithymocyte globulin, cyclosporine, tacrolimus)
29
Q

Some of the causes of anemia in children (4)

A
  • nutrition
  • haemolytic disease
  • ethnicity (G6PD enzyme)
  • congenital anomalies
30
Q

Definition of nutritional anemia

A

Iron deficiency (ID) and iron-deficiency anemia (IDA) ID is defined as a state in which there is insufficient iron to maintain normal physiologic functions such that iron stores (serum ferritin or bone marrow iron content) are reduced.

31
Q

Normal term infants have enough iron stores to prevent ID for first _______ months

A

4 months

premature infants have reduced stores

32
Q

Lab tests for IDA (3)

A

There is no single measurement that will document the iron status;
recommended tests include serum ferritin and C-reactive protein or reticulocyte hemoglobin concentration.

33
Q

Recommended supplement conc. of iron

A

6 mg/kg/d in 3 divided daily doses

34
Q

Neutropenia definition

A

Neutropenia is an absolute neutrophil (granulocyte) count of less than 1500/μL in childhood, or less than 1100/μL between ages 1 month and 2 years.

35
Q

Causes of neutropenia (5)

A
  • absent/defective myeloid stem cells
  • ineffective/supressed myeloid maturation
  • decreased production of hematopoietic cytokines
  • decreased marrow release
  • increased neutrophil apoptosis etc.
36
Q

Signs and symptoms of neutropenia (4)

A
  • acute severe bacterial/fungal infection
  • septicemia, cellulitis, abscess, pneumonia
  • chills, fever, malaise
  • spleen and liver are NOT enlarged
37
Q

Most common pathogens in neutropenia (2)

A

Staphylococcus aureus and gram-negative bacteria

38
Q

Lab findings in neutropenia

A
  • absent neutrophils in peripheral blood smear
  • normal monocytes and lymphocytes
  • marked reduction in myeloid cells
  • bone marrow aspiration and biopsy are most important for myelopoiesis
39
Q

Treatment of neutropenia (4)

A
  • infection treatment
  • recombinant G-CSF
  • immunisation
  • hematopoietic stem cell transplant
40
Q

Bleeding disorders types (4)

A
  • quantitative/qualitative abnormalities of platelets
  • quantitative/qualitative abnormalities of plasma procoagulant factors
  • vascular abnormalities
  • accelerated fibrinolysis
41
Q

Lab testing in bleeding disorders (5)

A
  1. Prothrombin time (PT) to assess clotting function of factors VII, X, V, II, and fibrinogen.
  2. Activated partial thromboplastin time (aPTT) to assess clotting function of high-molecular-weight kininogen, prekallikrein, XII, XI, IX, VIII, X, V, II, and fibrinogen.
  3. Platelet count and size determined as part of a CBC.
  4. Platelet functional assessment by platelet function analyzer- 100 (PFA-100), template bleeding time, or whole blood platelet aggregometry.
  5. Fibrinogen functional level by clotting assay.
42
Q

Lab testing in bleeding disorders (5)

A
  1. Prothrombin time (PT) to assess clotting function of factors VII, X, V, II, and fibrinogen.
  2. Activated partial thromboplastin time (aPTT) to assess clotting function of high-molecular-weight kininogen, prekallikrein, XII, XI, IX, VIII, X, V, II, and fibrinogen.
  3. Platelet count and size determined as part of a CBC.
  4. Platelet functional assessment by platelet function analyzer- 100 (PFA-100), template bleeding time, or whole blood platelet aggregometry.
  5. Fibrinogen functional level by clotting assay.
43
Q

Acute idiopathic thrombocytopenic purpura (ITP) (4)

A
  • most common bleeding disorder in children 2-5 years
  • often with viral infection (rubella, varicella, measels ,parvovirus etc.)
  • most patients have spontaneous recovery
  • spleen plays a dominant role in disease by forming the platelet cross-reactive antibodies
44
Q

Symptoms and signs of ITP (3)

A
  • acute, multiple petechie and ecchymoses
  • epistaxis
  • concurrent infection with EBV or CMV
45
Q

Lab findings in ITP (3)

A
  • The platelet count: reduced (< 50,000/μL and often < 10,000/μL), and platelets are of larger size on peripheral blood smear
  • number of megakaryocytes increased
  • PT and aPTT are normal
46
Q

What are most important risk factors for haemorrhage in ITP (2)?

A

The most important risk factors for hemorrhage are a platelet count less than 10,000/μL and mean platelet volume less than 8 fL.

47
Q

Treatment of ITP (5)

A
  • avoid aspirin and NSAIDs
  • avoid platelet transfusion, unless really needed
  • corticosteroids (1-2 mg/kg/d for 2-3 weeks, max 60-80 mg/d)
  • IV immunoglobulin
  • splenectomy (ITP for a year)

Pediatrics final (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions