Rheumatology Flashcards
(260 cards)
1
Q
What is Juvenile Idiopathic Arthritis (JIA) and how is it defined?
A
Juvenile Idiopathic Arthritis (JIA) is a group of heterogeneous autoimmune inflammatory joint disorders in children under 16 years old, lasting for more than 6 weeks. It is a diagnosis of exclusion and encompasses several subtypes, each with unique clinical and prognostic features. Inflammatory cytokines (e.g., IL-6, TNF-α) drive joint inflammation, synovial hypertrophy, and damage.
2
Q
What are the key clinical symptoms of JIA in children?
A
Children with JIA often present with early morning stiffness lasting >1 hour, joint pain that improves with movement, and swelling. These features reflect inflammatory pathology. Functional impairment includes difficulty with walking, writing, or dressing, and the child may appear fatigued or reluctant to play.
3
Q
What are the ILAR classification subtypes of JIA?
A
The ILAR classification includes: 1) Oligoarticular JIA, 2) Polyarticular JIA (RF-positive or negative), 3) Systemic JIA, 4) Psoriatic JIA, 5) Enthesitis-Related Arthritis, and 6) Undifferentiated JIA. This classification aids in prognosis and guides treatment escalation.
4
Q
What are the features of oligoarticular JIA?
A
Oligoarticular JIA is the most common subtype. It typically affects large joints asymmetrically (e.g., knees, ankles), predominantly in girls under 6 years old. ANA positivity is common and correlates with a high risk of chronic anterior uveitis, necessitating regular ophthalmologic screening.
5
Q
What are the features of polyarticular JIA?
A
Polyarticular JIA affects 5 or more joints within the first 6 months. RF-negative cases are milder, while RF-positive disease resembles adult RA and includes symmetric small joint arthritis, often with anti-CCP positivity. RF+ disease is more aggressive and often requires early DMARDs or biologics.
6
Q
What defines Systemic JIA (Still’s disease)?
A
Systemic JIA (Still’s disease) presents with quotidian fever (spikes once daily, returning to normal), evanescent salmon-pink rash, lymphadenopathy, hepatosplenomegaly, and serositis. Arthritis may appear later. Elevated ferritin, leukocytosis, and thrombocytosis are hallmark lab findings.
7
Q
What is Enthesitis-Related Arthritis (ERA)?
A
Enthesitis-Related Arthritis affects older boys (>8 years), particularly HLA-B27 positive individuals. It involves entheses (e.g., Achilles tendon), sacroiliac joints, and lower limb joints. There’s a risk of progression to juvenile ankylosing spondylitis and axial disease.
8
Q
What characterizes Psoriatic JIA?
A
Psoriatic JIA may present with arthritis preceding skin manifestations. Hallmarks include dactylitis (sausage digits), nail pitting, and asymmetric joint involvement. A family history of psoriasis supports the diagnosis, especially if the rash is not yet present.
9
Q
What infections should be ruled out before diagnosing JIA?
A
Before diagnosing JIA, rule out infections (septic arthritis, TB, viral arthritis), malignancies (leukemia), and trauma. Septic arthritis, in particular, is a pediatric emergency and must be excluded by joint aspiration and culture in any acutely swollen joint.
10
Q
What differentiates JIA from septic arthritis?
A
Septic arthritis often presents with a hot, swollen joint, fever, and refusal to bear weight. Synovial fluid analysis typically reveals turbid fluid with >50,000 WBC/mm³, neutrophil predominance, and low glucose. Empiric IV antibiotics and surgical drainage are needed.
11
Q
What labs support JIA diagnosis?
A
Laboratory work-up includes CBC (normocytic anemia, leukocytosis), inflammatory markers (ESR, CRP), ANA (uveitis risk), RF and anti-CCP (prognosis), and HLA-B27 in suspected ERA. Hypoalbuminemia and thrombocytosis are markers of systemic inflammation.
12
Q
How does ANA positivity affect JIA management?
A
ANA positivity is especially important in oligoarticular JIA, predicting higher risk for asymptomatic chronic anterior uveitis. These patients require slit-lamp exams every 3 months during active disease and less frequently in remission.
13
Q
What imaging is useful in JIA?
A
X-rays show periarticular osteopenia, joint space narrowing, and erosions in late disease. MRI detects synovitis, effusion, and bone marrow edema early. Ultrasound can guide joint aspiration and detect subclinical synovitis.
14
Q
What is the first-line treatment for mild JIA?
A
NSAIDs like naproxen and ibuprofen are first-line for mild disease. They offer symptomatic relief but do not halt disease progression. Gastroprotection may be needed with long-term use. Response helps differentiate inflammatory vs mechanical pain.
15
Q
When is Methotrexate used in JIA?
A
Methotrexate (MTX) is the DMARD of choice for moderate to severe JIA. Given weekly (oral or SC), it can take 4–6 weeks to show benefit. Liver enzymes and CBC should be monitored regularly. Folic acid reduces side effects like mouth ulcers and cytopenias.
16
Q
When are biologics used in JIA?
A
Biologic agents (e.g., etanercept, adalimumab, tocilizumab) are used for refractory disease. Anti-TNF agents are most commonly used. Screen for latent TB and hepatitis B/C before starting. They reduce flares and prevent joint destruction.
17
Q
What is the role of physical therapy in JIA?
A
Physical therapy is crucial to maintain joint mobility and strength. It also prevents contractures and muscle atrophy. Occupational therapy helps with activities of daily living. Psychological support addresses the impact of chronic illness.
18
Q
Why is eye screening important in JIA?
A
Slit-lamp exams are required every 3–6 months depending on ANA status and age. Chronic anterior uveitis may be asymptomatic until complications like band keratopathy or cataracts occur. Early detection preserves vision.
19
Q
What complications may arise from untreated JIA?
A
Complications include joint deformity, growth abnormalities (due to inflammation or steroid use), osteoporosis, delayed puberty, and psychosocial issues. Early aggressive treatment improves prognosis and prevents disability.
20
Q
What is Macrophage Activation Syndrome (MAS)?
A
Macrophage Activation Syndrome (MAS) is a life-threatening complication of systemic JIA. It resembles Hemophagocytic Lymphohistiocytosis (HLH) and presents with high ferritin (>10,000), pancytopenia, ↑ LFTs, DIC, and multiorgan failure. It requires urgent high-dose steroids or biologics.
21
Q
What is Rheumatic Fever (RF) and how is it defined?
A
Rheumatic Fever (RF) is an autoimmune inflammatory disease triggered by untreated group A β-hemolytic Streptococcus (GAS) pharyngitis. It affects the heart, joints, skin, and central nervous system, typically 2–4 weeks after infection. Molecular mimicry between streptococcal M-protein and host tissues initiates inflammation.
22
Q
What causes RF and what is its pathogenesis?
A
Cause:
Rheumatic fever is caused by an autoimmune response following untreated Group A Streptococcal (GAS) pharyngitis, especially from rheumatogenic M protein strains.
Pathogenesis:
Molecular mimicry: Streptococcal antigens resemble host tissues → immune cross-reaction.
Autoimmune inflammation: Affects heart, joints, brain, and skin.
Cardiac involvement: Formation of Aschoff bodies, leading to pancarditis and valvular damage (especially mitral valve).
Mediators: T cells, antibodies, and cytokines (e.g., IL-1, TNF-α) drive tissue damage.
23
Q
What are the major Jones criteria for RF diagnosis?What are the major Jones criteria for RF diagnosis?
A
Major Jones Criteria for Rheumatic Fever Diagnosis (Revised Jones Criteria, 2015 AHA):
These are the major manifestations used to diagnose acute rheumatic fever:
Major Jones Criteria:
- Carditis (clinical or subclinical via echocardiography)
May involve pericarditis, myocarditis, or valvulitis (especially mitral or aortic)
- Polyarthritis
Migratory, large joint arthritis (knees, ankles, elbows)
- Chorea (Sydenham chorea)
Involuntary, purposeless movements; emotional lability; hypotonia
- Erythema marginatum
Non-pruritic, pink macular rash with central clearing; trunk/proximal limbs
- Subcutaneous nodules
Firm, painless nodules over extensor surfaces or bony prominences
Note:
Diagnosis of RF requires evidence of preceding GAS infection plus:
2 major criteria, or
1 major + 2 minor criteria
24
Q
What are the minor Jones criteria for RF diagnosis?
A
- Fever
≥38.5°C (high-risk populations)
≥38.2°C (low-risk populations) - Arthralgia
Polyarthralgia (low-risk)
Monoarthralgia (accepted in high-risk) - Elevated acute phase reactants
ESR ≥60 mm/hr (≥30 mm/hr in high-risk)
CRP ≥3.0 mg/dL - Prolonged PR interval on ECG
Age-adjusted
Only valid in the absence of carditis
25
What is required for a diagnosis of acute rheumatic fever (RF) using the Jones Criteria (AHA 2015)?
Diagnosis requires evidence of a preceding Group A Streptococcal (GAS) infection plus:
Either
2 Major criteria,
OR
1 Major + 2 Minor criteria
Evidence of preceding GAS infection includes:
Positive throat culture for GAS
Positive rapid antigen test for GAS
Elevated or rising streptococcal antibody titer (e.g., ASO or anti-DNase B)
Recent scarlet fever
Note:
In high-risk populations, modified criteria (like monoarthritis or monoarthralgia) may be accepted.
Recurrent RF may be diagnosed with less stringent combinations if there’s existing rheumatic heart disease.
26
What are the clinical signs of carditis in RF?
1. New murmur(s):
Mitral regurgitation (most common)
Aortic regurgitation
2. Tachycardia out of proportion to fever
3. Pericardial rub or pericardial effusion
4. Cardiomegaly (seen on chest X-ray)
5. Signs of heart failure:
Dyspnea, orthopnea
Hepatomegaly
Peripheral edema
Elevated jugular venous pressure (JVP)
6. Prolonged PR interval (may also be seen in minor criteria)
7. Subclinical carditis:
Detected only by echocardiography (e.g., valvulitis without audible murmur)
27
Describe the arthritis seen in RF?
1. Type:
Migratory polyarthritis (classic)
Involves large joints (knees, ankles, elbows, wrists)
2. Pattern:
One joint becomes inflamed, then resolves as another joint is affected
Asymmetric involvement is common
3. Onset:
Appears early in the course of RF (within first few weeks after sore throat)
4. Characteristics:
Painful, red, swollen, and warm joints
Very responsive to NSAIDs or salicylates
Leaves no permanent joint damage
5. Modified Criteria (in high-risk populations):
Monoarthritis or polyarthralgia may be accepted
28
What is Sydenham chorea and how does it present?
Sydenham chorea is a neurological manifestation of acute rheumatic fever, caused by autoimmune inflammation of the basal ganglia.
Presentation:
1. Involuntary, purposeless, jerky movements
Affects face, hands, and feet
Often worsens with stress, disappears during sleep
2. Muscle hypotonia (floppy limbs)
3. Emotional lability
Irritability, inappropriate laughing or crying
4. Dysarthria (slurred speech)
5. Milkmaid’s grip
Inability to maintain a steady hand grip
6. Delayed onset
Occurs weeks to months after GAS pharyngitis
May appear alone without other major RF features
7. Self-limited course
Usually resolves over weeks to months, but may recur
29
What are subcutaneous nodules in RF?
Subcutaneous nodules are painless, firm lumps found under the skin, associated with severe carditis in rheumatic fever.
Characteristics:
1. Location:
Over extensor surfaces: elbows, knees, wrists, Achilles tendon, occiput, and spinous processes
2. Size and texture:
Small (0.5–2 cm), firm, non-tender, and freely movable under the skin
3. Course:
Usually transient (last days to weeks)
Often appear late in the course of illness
4. Association:
Strongly linked to carditis (may indicate severe or active cardiac involvement)
30
Describe erythema marginatum seen in RF?
Erythema marginatum is a non-pruritic, transient rash seen in rheumatic fever, classified as a major Jones criterion.
Presentation:
1. Appearance:
Pink/red macules with central clearing and serpiginous (wavy) margins
Often evanescent – may come and go spontaneously
2. Location:
Primarily on trunk and proximal limbs
Spares the face
3. Onset and course:
Often occurs early in the illness
May be subtle and easily missed
4. Associated with:
Usually seen in patients with carditis
31
How can recent streptococcal infection be confirmed in RF?
Rising or elevated antistreptolysin-O (ASO) titre (≥ 2-fold rise or > 200 Todd units in children)\n- Elevated anti-DNase B titre (especially useful if ASO is low)\n- Streptozyme test (screening agglutination for multiple anti-streptococcal antibodies)\n- Positive throat culture or rapid antigen detection test for group A β-hemolytic streptococcus (GAS)\n- Recent documented positive rapid strep test/throat culture in the preceding weeks"
32
What are common lab findings in RF?
Elevated acute-phase reactants:
ESR (erythrocyte sedimentation rate)
CRP (C-reactive protein)
Leukocytosis (especially neutrophilic)
Prolonged PR interval on ECG (reflecting carditis)
Evidence of recent streptococcal infection:
Elevated ASO (antistreptolysin O) titer
Elevated anti-DNase B
Mild to moderate anemia
In carditis: elevated cardiac enzymes or echocardiographic findings consistent with valvulitis or myocarditis.
33
How is arthritis in RF treated?
First-line treatment:
Aspirin (salicylates): High-dose aspirin (60–100 mg/kg/day in divided doses) for 1–2 weeks, then tapered over 4–6 weeks.
Alternative NSAIDs:
Naproxen (preferred in some guidelines due to better GI tolerance)
Steroids:
Not routinely used for arthritis alone
Reserved for severe carditis or if NSAIDs are contraindicated
Supportive care:
Rest during the acute phase
Monitor for response within 48–72 hours
Note: The arthritis is highly responsive to NSAIDs and typically non-destructive.
34
When are corticosteroids used in RF?
Indications for corticosteroids in RF include:
Severe carditis with heart failure
Poor response to salicylates/NSAIDs
Pericarditis with effusion or tamponade risk
Occasionally in severe systemic symptoms unresponsive to NSAIDs
Common regimen:
Prednisolone 1–2 mg/kg/day orally
Gradually tapered over 2–3 weeks after clinical improvement
Often followed by a short course of NSAIDs (e.g., aspirin) to prevent rebound inflammation
Note: Corticosteroids are not used for isolated arthritis or mild carditis.
35
How is Sydenham chorea managed?
General Approach:
Ensure patient safety: protect from falls, limit physical activity if chorea is severe
Provide psychosocial support, as symptoms are often distressing and prolonged
Pharmacologic Treatment:
First-line agents:
Valproic acid: preferred due to efficacy and favorable side-effect profile
Carbamazepine: alternative if valproic acid not tolerated
Neuroleptics (if needed):
Haloperidol or Risperidone (effective but risk of extrapyramidal symptoms)
Used in severe, functionally impairing cases
Corticosteroids (e.g., Prednisolone 1–2 mg/kg/day):
Consider in severe or refractory cases
Especially if carditis coexists or autoimmune pathogenesis is suspected
May shorten disease duration and reduce severity
Non-Pharmacologic Measures:
Encourage rest, reduce stress
Monitor academic and social impact (especially in adolescents)
Duration and Prognosis:
Typically self-limited: resolves over 2–3 months, but may last up to a year
Recurrence possible during stress, pregnancy (chorea gravidarum), or reinfection
Essential adjunct:
Long-term benzathine penicillin prophylaxis to prevent recurrence of streptococcal infection and further cardiac damage
36
What is the prognosis of RF?
Good prognosis if no carditis or mild involvement
Poorer outcome if moderate/severe carditis due to risk of chronic rheumatic heart disease (RHD)
Chorea usually self-limited; resolves in weeks to months
Recurrence is common without long-term penicillin prophylaxis
Prognosis improves significantly with early treatment and regular secondary prophylaxis
37
What is primary prevention of RF?
Goal: Prevent the first episode of RF by treating Group A Streptococcal (GAS) infections early.
Target: Children 5–15 years with sore throat or skin infections.
Drug of Choice:
Benzathine Penicillin G IM
<27 kg: 600,000 units IM single dose
≥27 kg: 1.2 million units IM single dose
Penicillin Allergy:
Erythromycin 10 days or Azithromycin 5 days
Timing: Within 9 days of symptom onset
Sudan Strategy:
Simplified diagnosis at primary care level
Free penicillin programs
Community education campaigns
This approach helps reduce RF and its complication: Rheumatic Heart Disease (RHD).
38
What is secondary prevention of RF?
Goal: Stop new streptococcal infections in anyone who has already had RF, thus preventing further heart-valve damage and progression to rheumatic heart disease.
Core therapy: Long-acting benzathine penicillin G (1.2 million U IM, or 600 000 U if < 27 kg) given every 3–4 weeks; in very high-risk settings such as Sudan, every 3 weeks is preferred.
If penicillin-allergic: Oral erythromycin (250 mg twice daily) or sulfadiazine (0.5 g daily if < 30 kg; 1 g daily if ≥ 30 kg).
39
How long should secondary prophylaxis be continued?
How long to continue:
• No carditis: at least 5 years after the last attack and until age 21 (whichever is longer).
• Carditis, no residual valve disease: 10 years after the last attack or until age 21.
• Carditis with residual valve disease: ≥ 10 years and often to age 40—or lifelong if risk remains high.
Sudan context: Prophylaxis is delivered through primary-care clinics; adherence is reinforced by community health workers because missed injections sharply raise recurrence risk.
40
How is carditis monitored in RF?
Follow-up includes regular cardiology visits, echocardiograms, and strict adherence to secondary prophylaxis to prevent recurrent rheumatic fever and progression of valve damage.
41
What is Henoch-Schönlein Purpura (HSP) and how is it defined?
Henoch-Schönlein Purpura (HSP), now termed IgA vasculitis, is a small-vessel immune complex-mediated vasculitis. It is the most common vasculitis in children and typically follows a respiratory infection. It involves deposition of IgA-containing immune complexes in vessel walls.
42
What is the pathogenesis of HSP?
IgA vasculitis predominantly affects small vessels of the skin, gastrointestinal tract, kidneys, and joints. The vasculitis causes increased permeability and inflammation, leading to purpura, arthritis, abdominal pain, and renal manifestations.
43
What are the four hallmark features of HSP?
The classical tetrad of HSP includes: 1) palpable purpura without thrombocytopenia, 2) arthritis/arthralgia, 3) abdominal pain, and 4) renal involvement (hematuria/proteinuria). Skin findings are often the first manifestation.
44
What is characteristic about the rash in HSP?
Palpable purpura are raised, non-blanching lesions due to leukocytoclastic vasculitis. They are most commonly seen on the lower limbs and buttocks, sparing the trunk. Lesions may be symmetric and do not disappear with pressure.
45
Which joints are typically involved in HSP?
Joint involvement occurs in 75% of cases, usually affecting large joints (knees, ankles). Symptoms include pain, swelling, and limited motion. The arthritis is transient and non-deforming, with no joint destruction.
46
What are the typical GI manifestations of HSP?
Gastrointestinal involvement may include colicky abdominal pain, nausea, vomiting, and GI bleeding (melena or hematochezia). Intussusception is the most serious complication, especially in younger children.
47
How does renal involvement present in HSP?
Renal manifestations range from isolated microscopic hematuria to nephritic or nephrotic syndrome. Severe involvement may lead to crescentic glomerulonephritis and long-term renal impairment.
48
What are important differential diagnoses for HSP?
Important differential diagnoses include immune thrombocytopenic purpura (ITP), meningococcemia, systemic lupus erythematosus (SLE), leukemia, and thrombotic thrombocytopenic purpura (TTP).
49
How is HSP differentiated from ITP?
HSP differs from ITP by the presence of normal platelet counts, multisystem involvement, and palpable purpura. ITP typically presents with isolated thrombocytopenia and bleeding from mucosal sites.
50
What investigations support the diagnosis of HSP?
Laboratory findings in HSP are often non-specific but may include elevated ESR and CRP. Platelet counts and coagulation profiles are normal. Serum IgA may be elevated in up to 50% of cases.
51
What does skin biopsy show in HSP?
Skin or renal biopsy (if performed) shows leukocytoclastic vasculitis with IgA deposition in vessel walls. Immunofluorescence confirms IgA as the dominant immunoglobulin deposited.
52
What is the role of serum IgA in HSP?
Abdominal ultrasound can reveal bowel wall thickening, free fluid, and in rare cases, intussusception or signs of gastrointestinal bleeding. It is preferred in children with severe abdominal symptoms.
53
What imaging helps assess GI symptoms in HSP?
Abdominal ultrasound can reveal bowel wall thickening, free fluid, and in rare cases, intussusception or signs of gastrointestinal bleeding. It is preferred in children with severe abdominal symptoms.
54
How is mild HSP managed?
Management of mild HSP is supportive: adequate hydration, rest, and analgesia (paracetamol or NSAIDs). Most cases resolve within 4 weeks without sequelae.
55
When are corticosteroids used in HSP?
Corticosteroids are indicated for severe abdominal pain, GI bleeding, orchitis, or significant renal involvement. Prednisone 1–2 mg/kg/day is typically used for a short course.
56
What is the prognosis of HSP?
Immunosuppressants (e.g., azathioprine, cyclophosphamide) or biologics may be considered for severe renal disease, especially in crescentic glomerulonephritis unresponsive to steroids.
57
What is the follow-up for HSP?
Prognosis is generally excellent, with most children recovering fully. However, persistent hematuria or proteinuria at 6 months predicts worse renal outcomes.
58
Can HSP recur?
Recurrence occurs in up to 30% of cases, usually milder and self-limiting. Recurrent flares may follow infections, cold exposure, or allergens.
59
What are possible complications of HSP?
Complications include intussusception, GI hemorrhage, renal failure, orchitis, and rarely CNS vasculitis. Early recognition and follow-up minimize these risks.
60
How is severe renal disease in HSP managed?
Follow-up includes regular blood pressure measurement and urinalysis for 6–12 months. Persistent proteinuria or hypertension warrants nephrology referral.
61
What is Kawasaki Disease (KD) and how is it defined?
Kawasaki Disease (KD) is an acute, self-limited vasculitis of medium-sized arteries, primarily affecting children under 5 years. It has a predilection for coronary artery involvement, making it the leading cause of acquired heart disease in children in developed countries.
62
What is Kawasaki Disease?
KD is classified as a medium-vessel vasculitis, with inflammation affecting the coronary arteries, skin, mucous membranes, lymph nodes, and conjunctiva. Without treatment, up to 25% develop coronary artery aneurysms.
63
What is the diagnostic criteria for Kawasaki Disease?
Diagnosis of classic KD requires ≥5 days of fever plus ≥4 of 5 principal features: (1) bilateral non-purulent conjunctivitis, (2) oropharyngeal changes, (3) peripheral extremity changes, (4) rash, (5) cervical lymphadenopathy. Diagnosis can be made earlier if coronary abnormalities are evident.
64
What are the mucosal changes seen in Kawasaki Disease?
Oral changes include erythematous, cracked lips, strawberry tongue, and diffuse erythema of the oropharynx. Mucosal involvement is prominent and may resemble viral illness or scarlet fever.
65
What eye findings are typical in Kawasaki Disease?
The conjunctivitis in KD is bilateral, non-purulent, and limbic-sparing. It typically appears within the first 4 days of fever and resolves without scarring.
66
What are the extremity findings in Kawasaki Disease?
Extremity changes include erythema of palms and soles, edema in the acute phase, and periungual desquamation (peeling) in the subacute phase (typically after 2 weeks).
67
Describe the rash seen in Kawasaki Disease?
The rash in KD is polymorphous, non-vesicular, and non-bullous. It may be maculopapular, urticarial, or resemble scarlet fever. Perineal desquamation is a specific but less common finding.
68
What type of lymphadenopathy is seen in Kawasaki Disease?
Lymphadenopathy is usually unilateral, >1.5 cm, and cervical. It is the least consistent feature and often the last to appear.
69
What lab findings support the diagnosis of Kawasaki Disease?
Laboratory findings include leukocytosis with neutrophilia, normocytic anemia, thrombocytosis (after day 7), elevated ESR/CRP, elevated transaminases, and sterile pyuria. ANA and RF are negative.
70
What is incomplete (atypical) Kawasaki Disease?
Echocardiography is essential to assess for coronary artery aneurysms, pericardial effusion, myocarditis, or valvular regurgitation. Serial echocardiograms are recommended at diagnosis, 2 weeks, and 6–8 weeks.
71
What infections can mimic Kawasaki Disease?
Incomplete KD should be considered in children with prolonged fever and fewer than 4 classic signs. CRP ≥3 mg/dL and ESR ≥40 mm/h support the diagnosis. Echocardiogram may show coronary changes.
72
What are the phases of Kawasaki Disease?
Differential diagnosis includes viral infections (EBV, adenovirus, measles), scarlet fever, SLE, Stevens-Johnson syndrome, and toxic shock syndrome. Features like hepatomegaly, arthritis, or thrombocytopenia may suggest alternatives.
73
What are the major complications of untreated Kawasaki Disease?
The main treatment for KD is IVIG (2 g/kg) given as a single infusion over 10–12 hours, ideally within 10 days of fever onset. It significantly reduces the risk of coronary artery aneurysms.
74
When should echocardiography be performed in Kawasaki Disease?
High-dose aspirin (30–50 mg/kg/day) is given in the acute phase for anti-inflammatory effect. Once afebrile, low-dose aspirin (3–5 mg/kg/day) continues for antiplatelet effect until normalization of coronary arteries.
75
What is the first-line treatment for Kawasaki Disease?
Children with persistent or recurrent fever 36 hours after IVIG may receive a second IVIG dose. If refractory, corticosteroids or biologics (e.g., infliximab) may be used.
76
What is the role of aspirin in Kawasaki Disease?
Coronary aneurysms are classified by Z-scores (body surface area-adjusted diameter). Z-score ≥2.5 defines aneurysm; >10 or absolute >8 mm is giant aneurysm with high thrombosis risk.
77
What should be done if fever persists after IVIG in Kawasaki Disease?
Prognosis is excellent with early treatment. Coronary abnormalities regress in 50–75% of patients. Giant aneurysms may persist and require long-term cardiology follow-up and anticoagulation.
78
What are the echocardiographic signs of coronary artery involvement?
Influenza and varicella vaccination should be delayed for 11 months after IVIG due to impaired vaccine response. Live vaccines may be ineffective in this window.
79
What is the prognosis for Kawasaki Disease with early treatment?
Long-term follow-up includes repeat echocardiograms, ECGs, and cardiology visits, particularly if coronary involvement is noted. Lipid profiles may also be monitored for cardiovascular risk.
80
What are the follow-up recommendations for Kawasaki Disease?
High-risk patients (e.g., infants <6 months, prolonged fever, anemia, high CRP) are more likely to develop coronary complications and may benefit from adjunctive corticosteroids during initial treatment.
81
What is Takayasu Arteritis (TA) and how is it defined?
Takayasu Arteritis (TA) is a chronic granulomatous large-vessel vasculitis affecting the aorta and its major branches. It predominantly affects adolescent girls and young women, especially in Asia. It leads to stenosis, occlusion, or aneurysms in major arteries.
82
What age group and sex is most commonly affected by TA?
TA is considered a large-vessel vasculitis and is characterized histologically by granulomatous inflammation of the vessel wall with giant cells. The disease evolves in phases—early systemic (inflammatory) and late occlusive (pulseless) phases.
83
What are the systemic (early) symptoms of Takayasu Arteritis?
It typically presents in young females under 40 years with systemic symptoms (fever, weight loss, fatigue), followed by vascular symptoms like diminished or absent pulses, limb claudication, and hypertension.
84
What are the vascular (late) symptoms of TA?
Classic signs include diminished or absent peripheral pulses, blood pressure discrepancies (>10 mmHg between arms), and bruits over large vessels (carotid, subclavian, abdominal aorta).
85
What are key physical exam findings in TA?
Hypertension is a common presentation, often due to renal artery stenosis. It can be severe and lead to hypertensive encephalopathy or retinopathy.
86
What are the neurologic manifestations of TA?
Neurological symptoms may include headache, dizziness, syncope, and visual disturbances due to cerebral ischemia from carotid or vertebral artery involvement.
87
What is the ACR classification criteria for TA?
Pulmonary artery involvement may lead to pulmonary hypertension, dyspnea, or hemoptysis. Coronary artery involvement can lead to angina or myocardial infarction.
88
Which arteries are commonly affected in TA?
ACR diagnostic criteria for TA include: age <40 years, claudication, decreased brachial pulse, BP difference >10 mmHg, bruit over subclavian/aorta, and arteriographic abnormalities. ≥3 criteria yield high sensitivity and specificity.
89
What are important differentials for TA?
Differential diagnoses include giant cell arteritis (age >50), atherosclerosis, fibromuscular dysplasia, congenital aortic coarctation, and neurofibromatosis (vascular type).
90
How is Takayasu Arteritis diagnosed?
Lab findings are non-specific: elevated ESR and CRP during active inflammation, normocytic anemia, and leukocytosis. ANA and ANCA are usually negative.
91
What laboratory findings are seen in TA?
MRI angiography and CT angiography are the preferred imaging modalities to visualize arterial wall thickening, luminal stenosis, aneurysms, and occlusions. PET-CT is helpful in assessing disease activity.
92
What imaging modalities are used in TA?
Conventional angiography remains the gold standard for detecting arterial lesions but is invasive. Doppler ultrasonography is useful for superficial arteries (e.g., carotids).
93
How does Doppler ultrasound help in TA?
Treatment includes high-dose corticosteroids (e.g., prednisone 1 mg/kg/day) to induce remission. Tapering is done over months based on response and inflammatory markers.
94
What is the role of PET-CT in TA?
Immunosuppressive agents (methotrexate, azathioprine, mycophenolate) or biologics (tocilizumab, infliximab) are added in steroid-resistant cases or as steroid-sparing agents.
95
How is active Takayasu Arteritis treated?
Surgical or endovascular interventions (angioplasty, stenting, bypass) are indicated for severe stenosis, critical ischemia, or uncontrolled hypertension but should be done during inactive disease.
96
What steroid-sparing agents are used in TA?
Long-term complications include vascular stenosis, aortic aneurysms, ischemic stroke, vision loss, and heart failure. Relapses are common and require monitoring.
97
When is surgery or angioplasty indicated in TA?
Monitoring includes serial ESR/CRP, blood pressure measurements, pulse checks, and periodic vascular imaging. PET-CT is useful in detecting subclinical inflammation.
98
What are the complications of untreated TA?
Disease activity indices (e.g., NIH criteria, Indian Takayasu Activity Score) and imaging guide treatment decisions and disease monitoring.
99
How is disease activity monitored in TA?
Prognosis depends on early detection, control of inflammation, and prevention of vascular damage. With treatment, 5-year survival exceeds 90%, but morbidity from vascular lesions persists.
100
What is the long-term prognosis in TA?
Multidisciplinary care with rheumatologists, vascular surgeons, radiologists, and cardiologists is essential for comprehensive management and long-term follow-up.
101
What is Polyarteritis Nodosa (PAN) and how is it defined?
Polyarteritis Nodosa (PAN) is a necrotizing vasculitis of medium-sized muscular arteries, leading to microaneurysms, thrombosis, infarction, and organ ischemia. It typically spares small vessels and the lungs.
102
Which vessels are primarily affected in PAN?
PAN predominantly affects adults but may also present in older children and adolescents. The disease involves renal, gastrointestinal, neurologic, musculoskeletal, and cutaneous systems.
103
What are the systemic symptoms seen in PAN?
The pathogenesis involves immune complex deposition and transmural inflammation of medium-sized arteries, often associated with hepatitis B virus in 10–30% of cases.
104
What skin manifestations are common in PAN?
Systemic features include fever, weight loss, malaise, myalgias, and fatigue. These nonspecific symptoms often precede localized organ manifestations.
105
What neurological findings are typical in PAN?
Cutaneous signs include livedo reticularis, painful subcutaneous nodules (especially along medium arteries), and non-healing ulcers. Digital ischemia and gangrene may occur in severe cases.
106
What are the renal manifestations of PAN?
Renal involvement manifests as renovascular hypertension, microscopic hematuria, proteinuria, and renal infarction. Unlike small vessel vasculitis, PAN does not cause glomerulonephritis.
107
How does PAN affect the gastrointestinal system?
GI symptoms result from mesenteric ischemia and include abdominal pain (especially postprandial), nausea, vomiting, and GI bleeding. Intestinal perforation is a serious complication.
108
What are key cardiovascular features of PAN?
Peripheral neuropathy is common in PAN, typically presenting as mononeuritis multiplex. This results in asymmetric motor and sensory deficits (e.g., wrist or foot drop).
109
What are important differential diagnoses of PAN?
Testicular pain and tenderness may occur due to testicular artery vasculitis. This symptom is highly suggestive of PAN and rarely seen in other vasculitides.
110
How does PAN differ from Microscopic Polyangiitis?
PAN should be distinguished from Microscopic Polyangiitis (MPA), which affects small vessels, is usually ANCA-positive, and often includes pulmonary capillaritis and glomerulonephritis.
111
What is the association between PAN and hepatitis B?
Laboratory findings in PAN are non-specific: elevated ESR/CRP, anemia of chronic disease, leukocytosis, and thrombocytosis. ANCA is typically negative. Hepatitis B serologies should be checked.
112
What laboratory findings support PAN diagnosis?
Tissue biopsy of affected skin, nerve, or muscle reveals transmural inflammation of muscular arteries with fibrinoid necrosis and inflammatory infiltrates.
113
What imaging is useful in PAN diagnosis?
Angiography (CT/MR/conventional) may show microaneurysms, segmental stenosis, occlusions, or 'beading' of renal or mesenteric arteries—hallmarks of PAN.
114
How is biopsy used in diagnosing PAN?
Diagnosis of PAN requires clinical suspicion, supported by biopsy and/or angiographic findings. ACR criteria include weight loss, livedo, testicular pain, mononeuritis, diastolic HTN, elevated BUN/Cr, HBV, biopsy, and arteriographic abnormality.
115
What is the standard treatment for idiopathic PAN?
Treatment includes high-dose corticosteroids (prednisone 1 mg/kg/day) for induction. In severe or life-threatening disease, cyclophosphamide is added.
116
How is PAN related to hepatitis B treated?
In hepatitis B-associated PAN, treatment includes antivirals (e.g., tenofovir), short course corticosteroids, and plasmapheresis to remove immune complexes.
117
When is cyclophosphamide indicated in PAN?
Other immunosuppressants like azathioprine or methotrexate may be used for maintenance therapy or steroid-sparing in non-severe disease.
118
What complications may arise from untreated PAN?
Untreated PAN can result in severe morbidity and mortality from renal failure, bowel infarction, stroke, or cardiac ischemia. Prognosis improves significantly with immunosuppressive therapy.
119
How is disease activity monitored in PAN?
Five-factor score (FFS) helps predict prognosis and guide treatment. Poor prognostic factors include renal insufficiency, proteinuria, GI involvement, CNS disease, and cardiomyopathy.
120
What is the long-term prognosis for PAN?
Close follow-up with labs (CBC, ESR/CRP, renal function) and periodic imaging is essential to monitor treatment response and detect relapses early.
121
What is Developmental Dysplasia of the Hip (DDH)?
Developmental Dysplasia of the Hip (DDH) is a spectrum of disorders characterized by abnormal development of the hip joint, including subluxation, dislocation, or instability. It results from failure of normal hip joint development during fetal life or infancy.
122
What are the major risk factors for DDH?
Major risk factors include female sex, breech presentation, family history of DDH, firstborn status, oligohydramnios, and swaddling with the hips in extension and adduction.
123
Why is DDH more common in females?
Estrogen-induced ligamentous laxity and intrauterine crowding (e.g., in breech position or twins) are major pathophysiological contributors. Breech babies cannot achieve hip flexion and abduction in utero.
124
What are the early clinical signs of DDH in newborns?
Clinical signs in newborns include hip instability detected by positive Barlow and Ortolani maneuvers. The Barlow test dislocates an unstable hip; the Ortolani test reduces it with a palpable 'clunk'.
125
What is the Ortolani maneuver?
In infants >3 months, signs include limited hip abduction, apparent limb shortening, asymmetric thigh/gluteal folds, and positive Galeazzi sign (unequal knee height in flexed hips).
126
What is the Barlow maneuver?
In toddlers, delayed walking, limping, toe-walking, and lumbar lordosis may suggest undiagnosed DDH. Painless limping is a common presenting feature.
127
What are late clinical signs of DDH in infants and toddlers?
Hip held in flexion and abduction reduces intra-articular pressure and improves joint stability in affected neonates.
128
What imaging modality is preferred in neonates under 6 months for DDH?
Hip ultrasound is the imaging modality of choice in infants under 6 months. It assesses femoral head position and acetabular development using Graf classification.
129
What imaging is preferred after 6 months of age for DDH?
Plain pelvic X-rays (AP view) are preferred after 6 months once femoral head ossification centers appear. Assess for acetabular angle and Shenton's line integrity.
130
What is the role of the Galeazzi test in DDH?
The acetabular index (>30° is abnormal) and disrupted Shenton’s line are key radiologic signs of DDH. Femoral head lateralization may also be visible.
131
How is DDH managed in newborns (<6 months)?
The Pavlik harness is first-line treatment for infants <6 months. It holds hips in flexion and abduction while allowing movement. Compliance and monitoring are essential.
132
What is the management of DDH in infants 6–18 months?
Failure of Pavlik harness or late presentation (>6 months) may require closed reduction under anesthesia followed by spica cast immobilization.
133
What is the treatment for DDH in children older than 18 months?
Open surgical reduction is indicated in children >18 months or if closed reduction fails. Procedures include adductor tenotomy and femoral/pelvic osteotomies.
134
What are complications of untreated DDH?
Complications of untreated DDH include early osteoarthritis, chronic limp, Trendelenburg gait, and leg length discrepancy. Hip joint remodeling potential declines with age.
135
When is screening for DDH recommended?
All newborns should be screened by physical examination. High-risk infants (breech, family history, female) should have targeted hip ultrasound at 4–6 weeks even if physical exam is normal.
136
What is the acetabular index and its significance in DDH?
The goal of treatment is to ensure concentric reduction and stable development of the hip joint, preventing long-term disability and degenerative changes.
137
What is the role of swaddling in DDH?
Regular follow-up with orthopedic evaluation and serial imaging is essential until skeletal maturity to ensure normal acetabular development and joint congruence.
138
How effective is the Pavlik harness in DDH?
Avoid tight swaddling that forces hips into adduction and extension, as this increases DDH risk. Promote safe swaddling with hips in flexion and abduction.
139
Why is early detection of DDH important?
Prognosis is excellent with early detection and treatment. Over 95% of infants treated before 6 months achieve normal hip development without residual issues.
140
What specialists are involved in DDH care?
Multidisciplinary team involvement includes pediatricians, orthopedic surgeons, physical therapists, and radiologists for diagnosis, management, and follow-up.
141
What is Transient Synovitis (TS) of the Hip?
Transient Synovitis (TS) is a self-limited, non-infectious inflammation of the hip joint synovium. It is the most common cause of acute hip pain and limping in children aged 3–8 years.
142
What is the typical age group affected by TS?
TS typically affects healthy children following a viral upper respiratory infection. It may represent a post-viral immune reaction with synovial irritation and effusion.
143
What are common triggers of TS?
Children with TS present with sudden onset limping or refusal to bear weight. Hip, groin, or medial thigh pain is common. The child appears well and usually afebrile or has only a low-grade fever.
144
What are the classic clinical features of TS?
The affected hip is often held in flexion, abduction, and external rotation to minimize intracapsular pressure. Pain is exacerbated by internal rotation or full extension.
145
How is the hip held in TS?
On examination, range of motion may be mildly reduced, especially internal rotation. There is no warmth or redness over the joint. Unlike septic arthritis, systemic signs are absent.
146
Is the child systemically ill in TS?
Differentiating TS from septic arthritis is critical. Kocher criteria help: (1) non–weight-bearing, (2) fever >38.5°C, (3) ESR >40 mm/h, (4) WBC >12,000. ≥2 criteria suggest septic arthritis.
147
How does TS differ from septic arthritis?
Laboratory findings in TS are usually normal or show mild elevation in ESR/CRP. WBC count is normal. No leukocytosis or high inflammatory markers typical of septic arthritis.
148
What scoring system helps differentiate TS from septic arthritis?
Ultrasound of the hip may show a small, non-septated joint effusion. However, ultrasound cannot distinguish between TS and septic arthritis without clinical correlation.
149
What investigations are used in TS?
X-rays are typically normal in TS but help rule out Legg-Calvé-Perthes disease, fracture, or SCFE. MRI is rarely needed but may be used in persistent or atypical cases.
150
What is the role of ultrasound in TS?
Management is conservative: bed rest, reduced activity, NSAIDs (e.g., ibuprofen), and close monitoring. Most cases resolve within 3–10 days without sequelae.
151
How is TS managed?
Symptoms usually resolve completely within 1–2 weeks. Return to full activity is allowed once pain and limp have resolved. No long-term treatment is needed.
152
When should a child with suspected TS be reevaluated?
Red flags for reconsideration of diagnosis include persistent pain beyond 2 weeks, recurrence, systemic illness, or hip stiffness. Consider Perthes or JIA in such cases.
153
What is the natural course of TS?
Unlike septic arthritis, TS does not cause joint destruction or need surgical drainage. Failure to respond to NSAIDs within 48–72 hours warrants reevaluation.
154
Can TS recur?
TS is more common in boys and usually unilateral. Bilateral TS is rare and should prompt evaluation for systemic diseases or rheumatologic conditions.
155
What is the prognosis of TS?
Recurrence is seen in ~15% of cases, usually mild and self-limiting. Repeat episodes may occur with viral illnesses and often require the same conservative approach.
156
What are key features distinguishing TS from Legg-Calvé-Perthes Disease?
No specific test confirms TS; it is a diagnosis of exclusion. Careful clinical assessment and short-term follow-up are key to safe management.
157
What are key features distinguishing TS from juvenile idiopathic arthritis?
Complications are rare. Misdiagnosis (e.g., missing septic arthritis or Perthes disease) is the main concern, underscoring the need for vigilance and follow-up.
158
Why is early differentiation from septic arthritis critical?
Children with hip effusion and a well appearance should be followed closely. Daily reassessment of pain, fever, and mobility is essential in early stages.
159
What is the role of MRI in evaluating TS?
Perthes disease differs by age (older child), insidious onset, persistent limp, and radiographic changes. TS is more acute and resolves fully.
160
Who should be involved in follow-up of unresolved or atypical cases?
Parental reassurance, appropriate education, and follow-up plans are important to prevent anxiety and ensure early detection of complications or evolving diagnoses.
161
What is Legg-Calvé-Perthes Disease (LCPD)?
Legg-Calvé-Perthes Disease (LCPD) is idiopathic avascular necrosis of the femoral head epiphysis in children. It results from temporary disruption of blood supply to the capital femoral epiphysis, leading to bone death and remodeling over time.
162
What age and sex are most commonly affected by LCPD?
LCPD typically affects children aged 4–10 years and is more common in boys (4:1 ratio). It is often unilateral and more common in children with low birth weight or delayed skeletal maturity.
163
What is the typical clinical presentation of LCPD?
Risk factors include male sex, low socioeconomic status, passive smoking exposure, coagulation disorders (e.g., factor V Leiden), and mechanical stress on immature femoral heads.
164
Why is pain referred to the knee in LCPD?
LCPD presents with a painless limp or antalgic gait, sometimes associated with mild hip, groin, or referred knee pain. The onset is insidious and not associated with fever or trauma.
165
What are common gait abnormalities seen in LCPD?
On physical exam, there is limited range of motion, especially hip abduction and internal rotation. A positive Trendelenburg sign and leg length discrepancy may be seen in late stages.
166
What are important physical exam findings in LCPD?
Plain radiographs (AP and frog-leg lateral views) are the initial imaging modality. Early stages show sclerosis of the femoral head, later progressing to fragmentation, reossification, and remodeling.
167
What imaging modality is first-line in LCPD?
MRI is more sensitive in early LCPD and may detect subchondral fractures and ischemic changes before X-ray abnormalities appear. Bone scans can also show decreased uptake in early disease.
168
What is the role of MRI in LCPD?
The disease is staged radiologically: Initial (ischemia), Fragmentation, Reossification, and Healed stages. Classification systems include Catterall and Herring lateral pillar classifications.
169
What are the radiographic stages of LCPD?
Differential diagnoses include transient synovitis (acute onset, normal X-ray), SCFE (adolescents, obesity, acute or chronic pain), and septic arthritis (febrile, acute pain, toxic appearance).
170
How is LCPD differentiated from SCFE?
Management depends on age, severity, and stage. Younger children (<6 years) with mild disease may be treated conservatively with NSAIDs, activity restriction, and physical therapy.
171
How is LCPD differentiated from transient synovitis?
Goals of treatment are to preserve femoral head sphericity and maintain range of motion to prevent early-onset osteoarthritis. Weightbearing may be limited in active phases.
172
What conservative treatments are used for mild LCPD?
Orthoses (abduction braces) may be used in select cases to maintain femoral head containment within the acetabulum during remodeling.
173
What is the goal of treatment in LCPD?
Surgical options (e.g., femoral or pelvic osteotomy) are indicated for older children (>8 years), poor lateral pillar support, or failed conservative treatment.
174
When is surgical treatment considered in LCPD?
Long-term complications include femoroacetabular impingement, hip stiffness, leg length discrepancy, and premature osteoarthritis. Outcomes are worse with late diagnosis or severe involvement.
175
What are the outcomes of LCPD with appropriate treatment?
LCPD should be followed every 3–6 months with clinical exams and serial radiographs until reossification is complete. MRI may be used to monitor healing in complex cases.
176
What long-term complications may arise from LCPD?
Children with bilateral involvement, which is rare (~10% cases), require more aggressive follow-up and consideration of systemic or metabolic causes.
177
What is the role of bone scans in LCPD?
Physical therapy focuses on strengthening, range of motion, and avoidance of joint contractures. Swimming and non-impact sports are often encouraged.
178
What classification is used to assess LCPD severity?
Education of families regarding disease course, expected recovery, and need for long-term monitoring is vital for adherence and outcomes.
179
What is the prognosis for LCPD diagnosed before age 6?
Prognosis is best in children <6 years with full epiphyseal coverage. Poor prognosis correlates with lateral pillar collapse and older age at presentation.
180
Who manages LCPD and how often should follow-up be done?
Multidisciplinary management includes orthopedic surgeons, physiotherapists, and radiologists to optimize individualized treatment plans.
181
What is Slipped Capital Femoral Epiphysis (SCFE)?
Slipped Capital Femoral Epiphysis (SCFE) is a Salter-Harris type I fracture through the proximal femoral growth plate, leading to displacement of the femoral head relative to the neck. It is the most common adolescent hip disorder.
182
What age and population are most affected by SCFE?
SCFE typically occurs in adolescents aged 10–16 years during periods of rapid growth. It is more common in boys, African-Americans, and children with obesity or endocrinopathies (e.g., hypothyroidism, GH deficiency).
183
What are the hallmark symptoms of SCFE?
Risk factors include obesity, male sex, family history, radiation therapy, and endocrine abnormalities such as hypothyroidism, renal osteodystrophy, or panhypopituitarism.
184
Why is SCFE sometimes misdiagnosed as knee pathology?
Patients present with progressive limp and hip, groin, or referred knee pain. Chronic slips have insidious onset, while acute slips occur after trauma or exertion.
185
What is the most sensitive clinical sign of SCFE?
On examination, the affected limb is often held in external rotation. There is limitation of internal rotation, flexion, and abduction. Drehmann’s sign is positive (obligate external rotation during hip flexion).
186
What is the Drehmann sign?
Plain radiographs (AP and frog-leg lateral views) confirm diagnosis. The lateral view is most sensitive. Look for posterior-inferior displacement of the epiphysis, widened physis, or a step-off at the metaphysis.
187
What are key physical exam findings in SCFE?
The Klein line (a line along the superior femoral neck) normally intersects the epiphysis; failure to do so is suggestive of SCFE.
188
How is SCFE classified radiologically?
SCFE is classified as stable (able to bear weight) or unstable (non-weight-bearing). Unstable slips have a higher risk of avascular necrosis and are orthopedic emergencies.
189
What imaging views are essential for SCFE diagnosis?
Degree of slippage is graded by the Southwick angle or classified as mild (<30°), moderate (30–50°), or severe (>50°). Severity guides surgical approach.
190
What does the 'Klein line' indicate in SCFE?
Immediate management includes non-weight-bearing with crutches or stretcher and urgent orthopedic referral. Delay in treatment increases risk of AVN and chondrolysis.
191
What are the risk factors for SCFE?
Definitive treatment is surgical in-situ fixation with a single cannulated screw across the physis. This stabilizes the slip and halts further displacement.
192
What is the standard treatment for SCFE?
Prophylactic fixation of the contralateral hip may be considered in high-risk patients (e.g., endocrinopathy, young age, bilateral symptoms, open physis).
193
When is prophylactic pinning of the contralateral hip considered in SCFE?
Chondrolysis is a complication marked by cartilage necrosis and joint space narrowing. It presents with stiffness and pain and may occur post-op or with delayed treatment.
194
What are the major complications of untreated or delayed SCFE?
Avascular necrosis (AVN) is the most feared complication, especially in unstable SCFE. Risk is higher with attempted reduction or delayed fixation.
195
How is stable vs unstable SCFE defined?
Post-operative care includes partial weight-bearing once healing is evident and physical therapy to restore motion. Long-term monitoring is essential for joint remodeling.
196
What is chondrolysis and its relevance in SCFE?
SCFE may lead to femoroacetabular impingement, limb length discrepancy, and early-onset osteoarthritis if not managed appropriately.
197
What conditions should be considered in the differential diagnosis of SCFE?
Differential diagnoses include LCPD (younger age, gradual onset), transient synovitis (mild symptoms, resolves quickly), and septic arthritis (fever, toxic appearance).
198
What is the long-term prognosis in SCFE?
Long-term follow-up with periodic imaging is required to assess for physeal closure, femoral head remodeling, and development of degenerative changes.
199
What follow-up is necessary after SCFE surgery?
Education on early signs and importance of rapid orthopedic referral is crucial, especially for primary care providers encountering limping adolescents.
200
Who manages SCFE cases?
Multidisciplinary care includes pediatric orthopedics, radiology, endocrinology (if underlying disorder is present), and physical therapy for rehabilitation.
201
What is Acute Hematogenous Osteomyelitis?
Acute hematogenous osteomyelitis is a bacterial infection of the bone acquired through bloodstream seeding. It most commonly affects the metaphysis of long bones in children due to their rich vascular supply.
202
What is the most common causative organism in osteomyelitis?
It primarily occurs in children under 10 years, especially boys. The most frequently affected sites include the femur, tibia, and humerus. In neonates, the infection may spread to adjacent joints due to transphyseal vessels.
203
What are key risk factors for osteomyelitis in children?
The most common pathogen in healthy children is Staphylococcus aureus. In sickle cell disease, Salmonella species are also common. In neonates, consider Group B Streptococcus and Gram-negative enteric bacteria.
204
Why are children more prone to hematogenous osteomyelitis?
Children typically present with localized bone pain, warmth, swelling, tenderness, fever, and refusal to bear weight or use the affected limb. Systemic signs like malaise and irritability may also be present.
205
What are hallmark clinical features of acute osteomyelitis?
Neonates and infants may have subtle signs: pseudoparalysis, poor feeding, fever, or excessive crying with movement. They may develop joint involvement due to transphyseal spread.
206
What bones are most commonly involved in children with osteomyelitis?
Initial investigations include CBC (elevated WBC), ESR and CRP (usually elevated), and blood cultures (positive in 40–60% of cases). Serial CRP is useful for monitoring treatment response.
207
How does osteomyelitis present in infants differently?
X-rays may show soft tissue swelling initially; bony changes (periosteal reaction, lucency) appear after 7–10 days. They are useful for baseline and follow-up.
208
What lab tests support a diagnosis of osteomyelitis?
MRI is the imaging modality of choice for early diagnosis. It shows marrow edema, abscesses, and involvement of adjacent structures. Gadolinium contrast enhances detection of phlegmon or sequestra.
209
What is the first-line imaging for suspected osteomyelitis?
Ultrasound can detect subperiosteal fluid collection and guide aspiration, especially in younger children. It is also useful to exclude adjacent septic arthritis.
210
What is the role of ultrasound in osteomyelitis?
Definitive diagnosis is made by culture of bone aspirate or biopsy. This also guides targeted antibiotic therapy. Blood cultures may help if aspiration is not feasible.
211
What is the definitive test for osteomyelitis?
Empiric IV antibiotics should be started immediately after blood cultures. Choices include cloxacillin, cefazolin, or vancomycin depending on MRSA risk. Modify based on culture results.
212
When should MRI be preferred over X-ray in osteomyelitis?
In neonates, empirical coverage includes ampicillin + gentamicin or cefotaxime. In sickle cell patients, cover for Salmonella with ceftriaxone or fluoroquinolones.
213
How is osteomyelitis treated initially?
Duration of treatment is typically 3–6 weeks. Initial IV therapy for 7–14 days is followed by oral antibiotics once clinical and lab parameters improve.
214
What is the typical duration of antibiotic therapy for osteomyelitis?
Indications for surgical drainage include large abscess, poor response to antibiotics, or confirmed subperiosteal/intraosseous collection. Biopsy may also be needed if diagnosis is uncertain.
215
What antibiotics are preferred in sickle cell patients with osteomyelitis?
Complications of untreated or delayed osteomyelitis include chronic osteomyelitis, growth plate damage, pathological fractures, limb length discrepancy, and joint destruction.
216
When is surgical intervention required in osteomyelitis?
Chronic osteomyelitis is characterized by sequestrum formation, sinus tracts, and poor antibiotic penetration. It may require long-term therapy and debridement.
217
What are potential complications of untreated or delayed osteomyelitis?
Follow-up includes regular clinical assessment, inflammatory markers (especially CRP), and imaging as needed. ESR may remain elevated longer than CRP.
218
What is the role of CRP in monitoring treatment of osteomyelitis?
Differential diagnoses include bone tumors (e.g., Ewing sarcoma), trauma, leukemia, transient synovitis, and septic arthritis. Imaging and labs help differentiate.
219
What is the prognosis of acute osteomyelitis with early treatment?
Multidisciplinary care involves pediatrics, orthopedic surgeons, infectious disease specialists, radiologists, and physiotherapists for comprehensive management.
220
Which specialists are typically involved in managing osteomyelitis?
Prognosis is excellent with early diagnosis and treatment. Most children recover fully without sequelae. Delays increase the risk of chronic complications.
221
What is Septic Arthritis (SA)?
Septic arthritis is a bacterial infection of the joint space, usually monoarticular, that leads to rapid cartilage destruction and joint damage. It is a pediatric emergency requiring urgent diagnosis and treatment.
222
What are the most common causative organisms in pediatric SA?
In children, the hip, knee, and ankle are the most commonly affected joints. Infants and toddlers are at highest risk due to rich metaphyseal blood supply and immature immune response.
223
What are key risk factors for septic arthritis?
The most common causative organisms are Staphylococcus aureus (MSSA/MRSA), Streptococcus species (especially Group A Strep), and Kingella kingae in toddlers. In sickle cell disease, Salmonella species should be considered.
224
What are the hallmark clinical features of septic arthritis?
Neonates may be infected with Group B Streptococcus, E. coli, and other Gram-negative bacilli. In immunocompromised children, atypical organisms may occur.
225
Which joints are most commonly affected in children?
Children present with acute onset of joint pain, swelling, warmth, erythema, and inability to move the joint. The affected limb is often held in a position of comfort. Systemic features include fever and irritability.
226
What are Kocher's criteria for septic arthritis of the hip?
In septic arthritis of the hip, the leg may be held in flexion, abduction, and external rotation. Infants may present with pseudoparalysis and refusal to feed or move.
227
What laboratory tests support the diagnosis of SA?
Kocher criteria help differentiate septic arthritis from transient synovitis: 1) fever >38.5°C, 2) non–weight-bearing, 3) ESR >40 mm/h, 4) WBC >12,000. ≥3 criteria significantly increase the likelihood of septic arthritis.
228
What imaging studies are helpful in diagnosing SA?
Investigations include CBC (leukocytosis), ESR, CRP, and blood cultures (positive in ~50%). CRP is useful for monitoring response to therapy. Procalcitonin may aid in differentiating from non-bacterial causes.
229
What is the gold standard for diagnosing septic arthritis?
Joint aspiration is diagnostic. Synovial fluid analysis shows turbid fluid with WBC >50,000/mm³ (mostly neutrophils), low glucose, high protein, and positive Gram stain/culture.
230
What pathogens should be suspected in neonates with SA?
X-rays may show joint space widening or soft tissue swelling. They are mainly used to rule out fractures or bone lesions. Ultrasound helps detect joint effusion, especially in the hip.
231
What is the initial management of SA?
MRI is useful for assessing adjacent osteomyelitis, soft tissue extension, and early joint destruction. It is particularly useful if aspiration is equivocal or diagnosis is uncertain.
232
What antibiotics are used empirically in SA for children >3 months?
Empirical IV antibiotics should be started after aspiration. Choices include cloxacillin, cefazolin, or vancomycin (if MRSA suspected). In neonates, use cefotaxime + vancomycin.
233
When is surgical drainage indicated in SA?
In sickle cell patients or immunocompromised children, use ceftriaxone or a fluoroquinolone to cover Salmonella and Gram-negative organisms.
234
How long is antibiotic therapy continued for SA?
Surgical drainage is indicated in the hip, shoulder, or if aspiration is inadequate. Options include arthrotomy or arthroscopic irrigation and debridement.
235
What is the role of CRP in SA management?
IV antibiotics are continued for 1–2 weeks, followed by oral therapy for a total of 3–4 weeks based on clinical improvement and lab normalization.
236
What are complications of delayed or untreated SA?
Untreated or delayed treatment can lead to cartilage destruction, growth plate injury, limb length discrepancy, and chronic joint deformity.
237
How does SA differ from transient synovitis?
Differential diagnoses include transient synovitis, JIA flare, reactive arthritis, trauma, leukemia, and osteomyelitis. Careful history and targeted imaging are key.
238
How is joint fluid in SA different from other arthritides?
CRP and ESR should be trended every 48–72 hours to monitor response. Lack of improvement should prompt repeat imaging or reconsideration of diagnosis.
239
What long-term follow-up is required for children with SA?
Multidisciplinary care with orthopedics, infectious disease, and physiotherapy ensures effective treatment, rehabilitation, and prevention of long-term disability.
240
What specialists manage pediatric septic arthritis?
Prognosis is excellent with early treatment. Delayed intervention increases risk of permanent joint damage and functional impairment.
241
What is Systemic Lupus Erythematosus (SLE)?
Pediatric SLE is a chronic multisystem autoimmune disorder characterized by the production of autoantibodies and immune complex deposition, causing widespread inflammation. It often presents more aggressively than adult-onset SLE.
242
What age and sex are most affected by pediatric SLE?
It typically affects girls >9 years of age and shows a female predominance post-puberty. Early onset is associated with more severe renal and CNS involvement.
243
What are the common constitutional symptoms of pediatric SLE?
The most common presenting symptoms are fever, fatigue, weight loss, arthralgia, and rash. Organ-specific involvement varies widely but often includes the skin, kidneys, joints, and hematologic systems.
244
What are the characteristic skin manifestations of SLE?
Cutaneous findings include malar (butterfly) rash, discoid lesions, photosensitivity, oral/nasal ulcers (usually painless), and alopecia. These findings may be misdiagnosed as eczema or dermatitis.
245
What musculoskeletal symptoms are seen in pediatric SLE?
Musculoskeletal symptoms include non-erosive arthritis or arthralgia, often symmetric, affecting small joints. Morning stiffness and functional limitations are common.
246
What renal manifestations occur in pediatric SLE?
Renal involvement may range from asymptomatic hematuria/proteinuria to nephritic or nephrotic syndrome. Lupus nephritis is a major cause of morbidity and is classified I–VI via renal biopsy.
247
How is lupus nephritis classified?
CNS involvement may include seizures, psychosis, cognitive dysfunction, mood changes, or transverse myelitis. These manifestations can occur at diagnosis or as the disease progresses.
248
What hematologic abnormalities are common in pediatric SLE?
Hematologic abnormalities include autoimmune hemolytic anemia, leukopenia, thrombocytopenia, and lymphopenia. These can mimic leukemia or aplastic anemia.
249
What neurological manifestations are seen in pediatric SLE?
Cardiopulmonary manifestations include pericarditis, pleuritis, myocarditis, pulmonary hypertension, and Libman-Sacks endocarditis. Chest pain and dyspnea are common complaints.
250
What are key cardiovascular complications in SLE?
Gastrointestinal manifestations include hepatosplenomegaly, pancreatitis, serositis, and mesenteric vasculitis. These may be confused with infection or inflammatory bowel disease.
251
What laboratory markers are specific for diagnosing SLE?
Diagnostic workup includes ANA (sensitive but not specific), anti-dsDNA (specific and correlates with renal disease), anti-Sm (highly specific), anti-RNP, and antiphospholipid antibodies.
252
What are common screening antibodies in suspected SLE?
Complement levels (C3, C4) are usually low during active disease. Persistent hypocomplementemia indicates ongoing immune complex activity, particularly in lupus nephritis.
253
How is complement level affected in active SLE?
ESR is usually elevated during flares. CRP may remain normal unless there is concurrent infection. CBC may show anemia, leukopenia, or thrombocytopenia.
254
What diagnostic criteria are used for pediatric SLE?
The 2019 EULAR/ACR classification requires a positive ANA and a weighted scoring of clinical and immunological features. A total score ≥10 is needed for diagnosis.
255
What drugs are used in the management of pediatric SLE?
First-line therapy includes hydroxychloroquine for all patients (unless contraindicated), as it reduces flares and long-term damage. Regular ophthalmologic exams are required due to retinal toxicity.
256
What are the indications for using cyclophosphamide in pediatric SLE?
Systemic corticosteroids (e.g., prednisone) are used for moderate-to-severe flares. Pulse methylprednisolone is used for life-threatening organ involvement.
257
What is the role of hydroxychloroquine in pediatric SLE?
Immunosuppressants like mycophenolate mofetil (preferred for nephritis), azathioprine, cyclophosphamide (for CNS/nephritis), and biologics (e.g., rituximab) are added based on severity and organ involvement.
258
What are key differences between pediatric and adult SLE?
Infection prevention is essential, especially during immunosuppression. Live vaccines are contraindicated during high-dose steroid therapy. Pneumococcal and influenza vaccines are recommended.
259
What complications can arise from pediatric SLE?
Long-term monitoring includes renal function, proteinuria, BP, complement levels, anti-dsDNA titers, CBC, and disease activity scores (e.g., SLEDAI). Early detection of flares improves outcomes.
260
How is disease activity monitored in pediatric SLE?
Multidisciplinary care includes pediatric rheumatology, nephrology, neurology, cardiology, ophthalmology, and mental health support to manage disease activity, treatment side effects, and quality of life.
