Derm/Rheum/Ortho Flashcards
1
Q
What are the characteristics of growing pains?
A
Age 3-10 years Bilateral leg pain Occurs at night Normal physical exam Normal lab tests Normal imaging
2
Q
What are the pain amplification syndromes?
A
Generalized- Fibromyaglia Localized- Reflex sympathetic dys -Autonomic changes, swelling acute, atrophic cold extremities chronic Girls>Boys
3
Q
What are the HIP causes of limp by age?
A
2-6 Transient synovitis 4-10 Legg Calve Perthes 10-14 Slipped Capital Femoral epiphysis
4
Q
What are the cardinal features of septic arthritis?
A
Fever Severe pain Swelling Redness SURGICAL EMERGENCY:Joint aspirate, culture antibiotics
5
Q
What is the differential diagnosis for monoarthritis?
A
Trauma/Mechnical/Orthopedic Infection (Acute-Septic/OM, Chronic- TB/Lyme) Inflammatory (JIA, FMF) Tumor Hemarthrosis
6
Q
What is the differential diagnosis for polyarthritis?
A
Inflammatory (JIA/SLE/Vasculitis) Infection Malignancy Mechnical Pain syndrome
7
Q
What are the major criteria for rheumatic fever?
A
SPACE Subcutaneous nodules Pancarditis Arthritis, migratory/poly Chorea Erythema marginatum
8
Q
What are the minor criteria for rheumatic fever?
A
Fever Arthralgia increase ESR, CRP Prolonged PR
9
Q
What are the diagnostic criteria for rheumatic fever?
A
2 major OR 1 mahor and 2 minor PLUS evidence of GAS infection
10
Q
What is the treatment for rheumatic fever?
A
Pencillin for 10 days ASA for arthritis ASA/steriod for carditis Prophylactic penicillin for at least 5 years or until 21 Lifelong for carditis
11
Q
What are antinuclear antibodies?`
A
Autoantibodies against nuclear antigens Low titres of ANA present in up to 30% of healthly people Persistent high titres present in CTD Specific antibodies only if ANA + and disease other than JIA suspected
12
Q
What is rheumatoid factor?
A
IgM autoantibody reacts to Fc portion of IgG antibodies
13
Q
What is oligoarthritis JIA
A
Girls >Boys Arhtirits max 4 joints in first 6 months Asymmetric joint involvement Knee most common Uveitis 18%, Extended OA 30-50% ANA + in 70% Consequences: malalignment of joints, muscle atrophy, growth distubrances, delay of motor development
14
Q
Uveitis
A
Most common in oligoarticular JIA Associated with ANA + Complications:irregular pupil, glaucoma, cataract, visual loss
15
Q
What is polyarthritis JIA
A
Symmetric joint involvement Small and large joints Slowly progressive Uveitis 5-10%
16
Q
Enthesis associated arthritis
A
BOYS Assymetric joint involvemen Enthesitis 50-60% HLA B27 70-90%
17
Q
Psoriatic arthritis
A
Search for hidden psoriasis Arthritis plus 2 of the following: Nail pits Dactylitis Family history of psoriasis
18
Q
Systemic JIA
A
>2weeks fo spiking fevers Arthritis + At least one of: Rash Generalized LN HSM Serositis
19
Q
What is macrophage activation syndrome?
A
Hemophagocytic lymphohistiocytosis Masssive release of proinflammatory cytokines Accumulation of marcophages in liver, spleen, LN, CNS Drop in cell lines, high Ferritin, lipids
20
Q
Which is TRUE: 1. Uveitis is a complication of psoriatic arthritis 2. Majority of polyarticular JIA cases are RF positive 3. ANA are typically positive in systemic HIA 4. Uveitis in JIA usually presents with a nonexudative conjuctivities
A
- Uveitis is a complication of psoriatic arthritis
21
Q
What is the approach to therapy in JIA?
A
NSAID/Joint injection for oligo JIA NSAID then MTX for poly HIA then biologics NSAIDS then corisone and or MTX for sJIA then biologics
22
Q
What are the side effects to treatment in JIA
A
CBC/Transaminases/Creatinine Increased risk for infections No live vaccines with MTX and biologics Monitor for TB with biologics Varicella can be severe No sulfa antibiotics with MTX
23
Q
Familial Mediterranean Fever
A
Fever, brief episodes (1-3 days) Painful polyserositis,peritonitis, pleuritis, arthritis Amyloidosis PPX with colchicine
24
Q
PFAPA
A
Periodic fever, aphtous stomatitis, pharyngitis, adenitis Regularly reccuring fevers with early age of onset Symptoms in ABSENCE of upper respiratory tract infection with at least one of: aphtous stomatitis, pharyngitis, adenitis ASx in between
25
SLE criteria
SOAP BRAIN MD Serositis Oral ulcers Arthritis Photosensitivity Blood disorders Renal involvement Antinuclear antibodies Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies) Neurologic disorder Malar rash Discoid rash
26
Lab findings in SLE?
ANA+ ESR elevated Low complement Increased LFTs, CK, etc
27
Dermatomyositis
Symmetric prox muscle weakness Cutaneous changes: heliotrope or Gottrons papules Elevated muscle enzymes
28
Helpful clues for Kawasaki?
Peeling in the perineal area Aseptic meningitis Anterior uveitis Hydrops of the GB Sterile pyuria Lung involvement Diarrhea in 25% GGT elevation
29
Intoeing by age
Infant: Metatarsus adductus Toddler: INternal tibial torsion Child: INcreased femoral anteversion
30
Scoliosis treatment by angle
50 Surgery
31
When to refer scoliosis
Atypical curves Skeletally immature and \>20 Skeletally immature and progressive curve Rapid progreen Skeletally mature and \>45
32
Septic arthritis vs. transient synovitis
Temp \>38 Refusal to wt bear WBC \>12 ESR \>40
33
Transient synovitis
Painful limp Limited ROM Fever Preceeding viral illness Noraml XR Elevated inflam markers
34
DDH risk factors
First Born Female Family history Frank Breech LeFt
35
DDH treatment
Early: Pavlik harness Late: Surgical
36
Legg Calves Perthes Disease
4-8 Y Boys Hyperactive Limp Decreased ROM
37
Hemangioma of infancy
4-10% F\>M RF: Prem, LBW, Multiples Not present at birth Proliferative in first few weeks until 3-12M, Usually complete by 5 months Regression at 18 M until 9-10Y
38
When do you worry about a hemangioma?
Obstruction: periorbital Midline lessions Large segmental on face Ulceration Multiple
39
What doe PHACES stand for?
Posterior fossa Hemangioma Arterial Cardiac Eye Sternal
40
Hemangioma treatment
95% require no treatment Propranolol if needed
41
Stork bite
Nevus Simplex 40% of newborns Usually fade
42
When do you worry about capillary malformations
V1 distrubtion (Sturge-weber\_ Lumbosacral
43
When do you worry about nevi?
ABCD Assymetry Border regularity Colour variety D
44
NF criteria?
2/7 \>5 CALM prepuberty Family History Axillary freckling Specific bony abN 2 NF Optic glioma Lisch nodules
45
Vitiligo
Autoimmune dlo Depigmented, sharply marginated patches No scale Strong topical steriods
46
Tinea Versicolour
Acute, recurrent Hypopigmented, fine white scale Antigungal shampoo daily Topical antifungals
47
Tinea captis
African-americans Culture PO therapy: Terbinafine
48
Pityriasis rosea
Self-limiting scaly eruption ?Viral Herald patch then Christmas tree pattern
49
Comedonal acne
Retinoid or BP
50
Inflammatory acne
BP OR Topical antibiotic + BP
51
Moderate acne
Oral antibiotics Hormonal therapy
52
Mastocytomas
Collection of mast cells below the skin Red and inflamed Stroked: Urticate: Dariers sign
53
Erythema Toxicum Neonatorum
Red macules, papules and pustules Palms and soles not involved Between 24-48 hours of age Lesions last 1-2 days, new crops qfew days
54
Transient neonatal pustule melanosis
3 lesions: 1. Pustules with no erythema 2. Ruptured pustules with peripheral scale 3. Hyperpigmented macules Term, dark skinned Lesions always at birth
55
Stages of incontinentia pigmenti
X linked Dominant Stage 1: Erythema, vesicles, pustules Stage 2: Verrucous, hyperkaterotsis Stage 3: Hyperpigmentation Stage 4: HYpopigmentation, atorphy
56
Incontinentia pigmenti
Linear vesicles on a red base Midlines respected Ocular associations
57
What is acrodermatitis enteropathica?
Zinc deficiency AR Impaired zinc excreiton in breast milk Diarrhea, skin eruption
58
Subcutaneous fat necrosis
Nodules/plaques in the neonatal period Hard, indurated, not warm Usually self-limited Obstetrical x 2 Hypercalcemia
59
Most common causes of serious drug eruptions?
Penicillins, AED, sulpha
60
What is the most common cause of erythema multiforme?
HSV infection
61
What are the clinical features of erythema multiforme? -most commonly appears in what age group? -what is the prognosis of EM? -treatment?
1. Classic TARGET lesions with erythematous border -can also look like macules, papules, vesicles, bullae, URTICARIA 2. Abrupt, symmetric eruption of rash usually on extensor upper extremities with SPARSE lesions on the face, trunk and legs 3. Oral lesions only with SPARING of other mucous membranes (ie. genital, resp, etc.) \*\*Most commonly occurs between 10-40 yo -prognosis: lesions typicaly resolve without sequelae in approx 2 weeks; does NOT progress to SJS -treatment: supportive care with topical emollients, systemic antihistamines, NSAIDs to provide symptomatic relief -do NOT use steroids as could increase HSV replication and make EM more frequent/continuous -MAY give prophylactic oral acyclovir x 6 mo if recurrent episodes of HSV-associated EM
62
What are possible causes of SJS? (2)
1. Infection: most common cause is MYCOPLASMA 2. Drugs: NSAIDs, sulfonamides, antibiotics, anticonvulsants
63
What are the clinical features of SJS? -percentage of body surface area involvement
1. Erythematous macules that rapidly develop central necrosis to form VESICLES, BULLAE and DESQUAMATION on face, trunk, extremities (widespread) 2. TWO OR MORE MUCOSAL SURFACES involved: eyes, oral cavity, upper airway or esophagus, GI tract or anogenital mucosa 3. Lots of pain from mucosal ulceration but skin tenderness is minimal to absent
64
What are main differences between erythema multiforme and SJS? (2)
1. Location of rash: -EM: usually on extensor surfaces of upper extremities, occasionally on legs; rarely on face and trunk -SJS: more widespread involving face, trunk and extremities 2. Mucosal membrane involvement: -EM: usually only involves mouth -SJS: involves TWO OR MORE mucosal surfaces (eyes, oral vacity, upper airway, esophagus, GI tract or anogenital mucosa)
65
What are the main differences between SJS and TEN? (2)
1. BSA involvement of skin necrolysis: -SJS 30% BSA 2. Pain of skin lesions: -SJS: only mucosal membrane pain from ulceration, minimal to NO skin tenderness -TEN: lots of pain from skin lesions
66
What are possible complications of SJS?
1. Corneal ulceration 2. Anterior uveitis 3. Bronchitis 4. Pneumonitis 5. Myocarditis 6. Hepatitis 7. ATN 8. Bacterial superinfection and sepsis 9. High insensible fluid loss 10. Urogenital strictures in long term
67
What are possible lab abnormalities seen in SJS?
1. Leukocytosis 2. Elevated ESR 3. Increased liver enzymes 4. Decreased serum albumin
68
What is the management of SJS?
SUPPORTIVE overall! 1. Stop possible offending drugs asap 2. Ophtho consult to assess for corneal scarring 3. PAIN management for oral lesions (morphine, mouth wash, etc.) 4. Monitor vaginal lesions to prevent vaginal stricture/fusion 5. IV fluids if insensible fluid losses from bullous eruption or desquamation \*\*\*\*no evidence for use of steroids! \*\*\*Can consider IVIG in early disease
69
What are the clinical features of TEN? (4) -what is a classic sign on exam of TEN? -complications? -etiology? -treatment?
\*\*\*\>30% BSA involvement in necrolysis 1. Widespread blister formation and morbilliform or confluent erythema ASSOCIATED WITH SKIN TENDERNESS! 2. NO NO NO target lesions!!!!!! 3. Sudden onset and generalization within 24-48 hrs 4. Full thickness epidermal necrosis! 5. Usually involves multiple mucous membranes (3 or more) \*\*\*usually have prodrome of fever, malaise, diffuse erythema -classic exam finding: NIKOLSKY SIGN (gentle pressure on the skin causes desquamation of the bullae) -complications: strictures, fluid loss, sepsis, conjunctival scarring -etiology: same as SJS (mycoplasma, sulfonamides, antibiotics, anticonvulsants, NSAIDs) -treatment: supportive. NO STEROIDS. Consider IVIG
70
What are the two fungi that cause tinea capitis? -how do you contract this fungi? -treatment?
1. Trichophyton 2. Microsporum -contact with person, animal, or soil -treatment: 1. Oral therapy is mandatory if kerion is present = terbinafine PO (lamisil) or itraconazole x 2-4 weeks
71
What kind of rashes can develop from methotrexate?
1. Stevens Johnson 2. Erythema multiforme 3. Photosensitivity 4. Hypo- or hyper-pigmentation
72
What is your differential diagnosis for midline neck mass? (3) -what about neck mass in anterior triangle?
1. Thyroglossal cyst 2. Dermoid cyst 3. Laryngocele -treatment: amoxi-clav if PO (oral anaerobes and skin bugs) or cefuroxime + clinda if IV Neck masses in the anterior triangle: 1. Cervical lymphadenitis 2. Branchial cleft cyst 3. Dermoid cyst 4. Teratoma
73
What are clinical features of herpetic whitlow? -treatment?
Usually seen in children who suck their thumb -vesicles look pus-filled but do NOT I&D because then you're spreading the HSV everywhere -can get regional lymph ndoes -resolves in ~3 weeks -treatment: PO acyclovir
74
A child with diffuse atopic dermatitis has been compliant with steroid treatment but is not improving. What is your next line agent?
Topical tacrolimus: calcineurin inhibitor -for kids \> 2 yo who have failed topical steroids -if they fail to respond to topical tacrolimus: can try cyclosporine
75
How long is the treatment with Accutane (isotretinoin)? -recurrence risk? -side effects? -monitoring while on treatment?
-Treatment course is 5 months: 40% cure rate, 40% will require topical or oral maintenance, 20% will relapse and need repeat course Side effects: 1. Teratogenecity 2. Increased triglycerides and cholesterol 3. Dry mucous membranes 4. Major depression with suicide risk BUT no case reports of actual suicide cases Monitoring: 1. Monthly pregnancy tests 2. Counsel re: two forms of birth control 3. Monthly lipid profile 4. Monthly liver enzymes
76
A patient presents with nail pitting. What is your differential diagnosis? (3)
1. Psoriasis 2. Alopecia areata 3. Reactive arthritis
77
How do you contract molluscum contagiosum? -affects which two populations the most? -description of rash -the child develops pustules around the lesion. What do you do? -management?
Contraction: contact with lesions or fomites Two populations: 1. school age children 2. Immunosuppressed Central umbilication, pearly papules, can be anywhere on the body Pustules: don't treat as this is an immunological reaction! Treatment: 1. Cantharidin 2. Monitoring (resolves with time) 3. Curettage
78
What are the clinical features of incontinentia pigmenti? -mode of inheritance?
Rare, X-linked (lethal in males, so females are ones you will), ectodermal disorder 1. Derm: -erythematous, LINEAR streaks, plaques of vesicles, then become dry on limbs and trunk -follow lines of blaschkow -increased eosinophils in rash and serum -presents in neonatal period 2. Ocular: neovascularization, strabismus, cataracts, optic atrophy 3. CNS: seizures, spasticity, etc. 4. Dental anomalies: late dentition, hypodontia, conical teeth 5. Alopecia on top of head 6. Nail dystrophy (ridging and pitting) 7. Skeletal defects
79
What is the treatment for scabies? -what are contraindications to first line treatment?
5% permethrin x 8-14 hrs if \> 6 yo, 6-8 hrs if
80
A patient presents with vesicles after sun exposure and hypopigmented flar scars with mild trauma. What is the most likely medication that causes this rash?
Pseudoporphyria: small hypopigmented depressed scars after mild trauma or after spontaneous vesicular lesions = caused by NSAIDs
81
What organ is most commonly affected by hemangiomas?
Liver
82
What are complications of hemangiomas?
1. Bleeding 2. Airway obstruction 3. Ulceration 4. Kasabach Meritt resulting in high output cardiac failure 5. Association with other defects (PHACES, midline hemangiomas may be associated with other midline defects)
83
What are the treatment modalities for psoriasis?
1. Topical corticosteroids 1st line 2. Topical vitamin D analogues: calcipotriene 3. Topical retinoids 4. Topical tar 5. Phototherapy 6. PO cyclosporine 7. PO Methotrexate 8. PO biologics
84
What are treatment options for pustular acne?
Moderate acne: 1. Topical retinoids + benzoyl peroxide 2. Topical retinoids, benzoyl peroxide, topical antibiotics Severe: 1. Oral antibiotic + topical retinoid + benzoyl peroxide + topical antibiotics
85
What are the clinical features of pityriasis rosea? -most common cause? -treatment?
-most often caused by HHV-6 or HHV-7 -see herald patch preceding the eruption, then systemic eruption follows 5-7 days later in Christmas tree (skin cleavage lines) -lasts up to 12 weeks -treatment: antihistamine or topical steroids
86
How is poison ivy spread? -is it spread from fluid from burst vesicles?
Fluid from ruptured -antigen retained on skin under fingernails and on clothes can initiate new plaques of dermatitis if you don't wash with soap and water -can also be on live or dead leaves, can be on animal fur
87
What is the characteristic rash of poison ivy?
LINEAR VESICLES with excoriation, itchy and spreads where scratches
88
What age is the peak incidence of erythema toxicum? -what type of cells do you see inside?
2nd day of life -should disappear by 2nd week of life -see eosinophils
89
What type of cell do you see in neonatal pustular melanosis? -how long does it last?
PMNs! -last several months
90
What is the most common infection associated with id reactions?
Tinea pedis
91
What are risk factors for development of hemangiomas? -3 types of hemangiomas? -natural progression? -treatment?
1. Prem 2. Low birth weight 3. Multiple gestation -think SMALL babies = might be because of ?hypoxic injury leading to vascular proliferation but no one knows for sure -3 types: 1. Superficial: strawberry hemangioma = papular or plaque 2. Deep: sits under the skin 3. Mixed: components of both -natural progression: grows until 4-5 mo of age, then starts to plateau and regresses until 9-10 yo. 50% of kids have lasting skin changes Treatment: 90% require no active management -10% require treatment IF vision or airway threatening or risk of significant negative cosmetic outcome -use propranolol at standard therapy (avoid in asthma, diabetes, PHACES because of cardiac defects)
92
What are the differences between capillary malformation and hemangioma?
Capillary malformation: present at birth, grow with the child and persist Hemangioma: usually not present right at birth, rapid growth, then resolves
93
What are the ABCDEs of moles?
Acquired Nevi: this tells you when to worry! Asymmetry Border: irregular Color: multiple colors, asymmetrical colors Diameter \> 6 mm Evolution/changing
94
Which of the following hypopigmented lesions is scaly? a. vitiligo b. tinea versicolor c. tuberous sclerosis d. post-inflammatory hypopigmentation -clinical features of tinea versicolor? -treatment?
Tinea versicolor! Malassessia furfur infection = see a fine scale over the rash. Also, the malassessia LIKES sebum/sebaceous glands! Will thus see tinea versicolor in older children/adolescents in areas of sebaceous glands (think where you get acne) -need to rub the rash to elicit the scale -treatment: antifungal shampoo daily x 1-2 wks, then 1x q3 mo to prevent recurrence -vitiligo: no scale, SHARPLY demaRCATED -post-inflammatory: no scale, poorly marginated
95
A patient has salmon colored think scaling plaques on the trunk in the shape of a Christmas tree. What is your diagnosis? -management?
Pityriasis rosea = self limited condition thought to be caused by a virus. -herald patch = salmon colored patch surrounded by darker rim and fine scale -management: reassurance - rash will self-resolve in several weeks
96
When are pimples precocious?
Acne vulgaris that begins before age 7 years should warrant further investigation
97
What other topical acne products should not be used in combination?
The combination of topical tretinoin plus BPO applied at the same time is known to cause oxidation and inactivity of the tretinoin.
98
Midline Lumbosacral Lesions Associated with Occult Spinal Dysraphism or Tethered Cord
1.Sacral pits (particularly with lateral deviation of the gluteal cleft) 2. Hairy patches 3. Appendages (skin tag or tail) 4.Sacral lipoma 5. Vascular lesions (hemangioma, port wine stain, telangiectasias) 6. Pigmentation variants (hyperpigmentation, including lentigo and melanocytic nevus, and hypopigmentation) 7. Aplasia cutis congenita
99
What are the common causes of acute urticaria in children?
• Infection (viral and bacterial are the most frequent, but fungal pathogens may also cause urticaria) • Infestation (parasites) • Ingestion (medication and foods) • Injections or infusions (immunizations, blood products, and antibiotics) • Inhalation (allergens such as pollens and molds)
100
What is impetigo?
Impetigo is a superficial skin infection that is caused by Staphylococcus aureus or group A streptococcus. Bullous impetigo is usually caused by S. aureus.
101
Dermographism
Dermographism (dermatographism) occurs when susceptible skin is stroked firmly with a pointed object.
102
Describe the five key battle plans to treat atopic dermatitis
Reduce pruritus. Hydrate the skin. Reduce inflammation. Control infection Avoid irritants.
103
SSSS vs TEN
104
In children suspected of having an anterior shoulder dislocation, which nerves should you check are intact?
Axillary nerve: deltoid contraction and sensory patch over deltoid
Musculocutaneous nerve: biceps contraction and sensory patch on lateral forearm
105
What are the indications for open reduction?
NO CAST
N - non-union
O - open fracture
C - compromise of neurovasculature
A - intra-articular fracture
S - Salter Harris type 3, 4, 5
T - polytrauma
106
In anterior shoulder dislocation, what tests can you perform to confirm the diagnosis?
1. Apprehension test: abduct and externally rotate the arm and should see apprehension in the patient's face since it re-creates a feeling of anterior dislocation
2. Relocation test: posteriorly direct a force during the apprehension test to relieve apprehension
107
What are two classic features of systemic-onset JIA?
1. High-spiking fevers once or twice daily at similar times
2. EVANESCENT (comes and goes in a few hours), salmon-colored, macular rash (usually on extremities)
108
What are two conditions to rule out in a child with fever and limb pain?
1. Osteomyelitis
2. Septic arthritis
109
ifferential diagnosis for fever and limb pain?
Reactive:
-arthritis
-transient synovitis
Infections:
-Lyme disease
-Viral
Malignancy
Inflammatory
110
Where does bacteria come from in septic arthritis?
Penetrating
Contiguous: from adjacent infection
Hematogenous: more blood vessels in growth plates and thus bacteria can enter there
50% of neonates with osteomyelitis will also have a septic joint because of spread through blood vessels
111
Top common causes of septic arthritis? (3)
In neonates?
In sickle cell?
In teenagers?
1. Staph aureus
2. Strep pneumonia
3. Non Group A strep
4. Kingella Kingae
In neonates: GBS and gram negatives
Sickle cell: Salmonella
Teenagers: gonorrhea
112
What percentage of children with septic arthritis have fever?
Clinical features of septic arthritis?
-70%
Acute, monoarthritis, erythema, warmth swelling, intense pain with passive movement
113
Septic arthritis in the hip: clinical feature?
Hip is a deep joint, will not see effusion, erythema but will still have intense pain, pain on passive movement
114
What is the diagnostic test of joint to diagnose septic arthritis?
What percentage of children with septic arthritis will have a positive blood culture?
- Arthrocentesis (aspiration of joint) is diagnostic intervention of choice
- WBC \> 50 x 10^9
- Gram stain positive 50% of time
- Culture positive 70% of time
Percentage of children with culture positive septic arthritis who will have positive blood culture: 50%
115
Antibiotic choice for osteomyelitis/septic arthritis:
- neonate
- 1-3 month old
- child
- teenager
- sickle cell
- Neonate: Cloxacillin and Gentamicin
- 1-3 month old: Cefuroxime (because of more gram negatives)
- Child: Cefazolin
- Teenager: ceftriaxone OR cefixime + azithromycin
- Sickle cell: cefotaxime (salmonella coverage)
116
Pseudomonas coverage in nail penetrating injury?
Ceftazidime or Pip Tazo
117
What are useful tests with high sensitivity for osteomyelitis?
```
Bone scan (but high exposure to radiation)
MRI
```
118
How long to treat with IV antibiotics in osteomyelitis/septic arthritis?
Until fever resolves \> 24 hrs, pain completely resolved (may need up to 4-6 weeks); then switch to PO abx x 2-3 wks
-monitor CRP and ESR for improvement
119
What is the prognosis of transient synovitis?
More common in boys or girls?
Resolution within 7-10 days
More common in boys
120
Features of transient synovitis vs. septic arthritis?
Septic arthritis: will not want to walk, looks unwell, lots of pain, fever, elevated ESR/CRP, elevated WBC, positive gram stain or culture of joint fluid, key is MARKED RESISTANCE TO MOBILITY AND SWELLING/WARMTH OF JOINT
Transient synovitis: will still be active but limping, looks well, can elicit range of motion in joint if done gently, can have low fever or afebrile, normal WBC, mild pain usually, normal or mildly elevated ESR/CRP
\*\*\*\*Remember that transient synovitis is a diagnosis of exclusion
121
What are the clinical features of PAPA syndrome? (3)
- mode of inheritance?
- main treatment principles?
1. Pyogenic Arthritis (sterile with high neutrophils)
2. Pyoderma gangrenosum (skin ulcerations)
3. Acne (severe cystic)
-considered a hereditary periodic fever syndrome but fever is not a major symptom
-can often be mistaken for multiple episodes of septic arthritis but cultures will be negative
-mode of inheritance: autosomal dominant
Main treatment principles:
1. Acne control with tetracycline or accutane
2. High dose NSAIDs: indomethacin TID
3. Prednisone pulse therapy for acute flare-ups
122
What are the laboratory findings in Kawasaki Disease? (8)
1. Anemia
2. Elevated platelets in 2nd week of illness (thrombocytosis = think inflammatory condition, acute phase reactant)
3. Elevated ESR & CRP
4. Elevated transaminases
5. Sterile pyuria
6. Elevated GGT
7. Hypoalbuminemia
8. Leukocytosis with neutrophilia and left shift
123
What is the Jones Criteria?
-what are 3 circumstances where diagnosis can be made without strict adherence to Jones criteria?
Diagnostic criteria for acute rheumatic fever
- need 2/5 major criteria OR 1/5 major and 2/4 minor criteria PLUS evidence of recent GAS infection (positive throat culture or rapid strep antigen test or elevated or increasing ASOT)
- Major criteria: SPACE
1. Subcutaneous nodules on extensor surfaces of tendons near bony prominences
2. Pancarditis (endocarditis universal finding, sometimes has myocarditis or pericarditis)
3. Arthritis (poly, migrating, large joints = 75% of cases, most common feature)
4. Chorea
5. Erythema marginatum
- minor criteria: FLAP
1. Fever
2. Lab findings: elevated ESR, CRP
3. Arthralgia
4. Prolonged PR interval
3 circumstances where diagnosis of ARF can be made without Jones criteria:
1. Chorea (may occur as the only manifestation)
2. Indolent carditis
3. Recurrent episodes
124
What are the cardiac consequences of having acute rheumatic fever?
Occurs in 50-60% of cases. 70% of patients recover with no residual heart disease.
- if a patient presents with carditis during the initial episode, then have risk for recurrence with increased risk of permanent heart damage with each recurrence
- see mitral regurgitation and aortic insufficiency in the acute phase
- see valvular disease (mitral or aortic stenosis) in chronic phase
125
What are the features of erythema marginatum? What condition is it seen in?
Seen in acute rheumatic fever
- erythematous, macular lesions with pale centers
- non pruritic
- occurs on trunk and extremities, NOT on the face!!!
- can be accentuated by warming the skin
126
What is the timing of development of acute rheumatic fever in relation to when the GAS infection occurred?
2-4 wks after GAS pharyngitis
- 1/3 of patients with ARF have no history of antecedent pharyngitis
- only 10-20% of throat culture or rapid strep antigen tests will be positive at the time of presentation of ARF; this is why ASOT is needed!
127
What antibody tests should be performed to diagnose recent GAS infection in a patient with suspected ARF?
1. ASOT
2. Anti-DNase B
3. Antihyaluronidase
\*\*\*Only 80-85% of patients with ARF will have elevated ASOT at presentation whereas 95-100% will have elevation of one of the antibodies if all 3 are tested
128
What is the treatment for acute rheumatic fever? (5)
1. Penicillin V PO x 10 days to eradicate GAS from upper respiratory tract
2. Long-term antibiotic prophylaxis to prevent recurrence (and thus cardiac complications): Pen V BID OR Pen G IM monthly until 21 yo or for life if they had carditis
3. Salicylates: only once diagnosis has been confirmed! ASA = 100 mg/kg/day div q6h x 5 days, then 75 mg/kg/day div q6h x 4 wks
4. Steroids IF there is carditis or congestive heart failure
5. Phenobarb PO for chorea
129
What are the characteristic features of Sydenham's chorea in acute rheumatic fever?
-when does it typically present in relation to GAS infection?
Emotional lability, incoordination, poor school performance, uncontrollable movements, facial grimacing, milkmaid's grip (irregular contractions of the muscles of the hands while squeezing the examiner's fingers like milking a teet), darting movements of the tongue with protrusion
- these are exacerbated by stress
- can present months after GAS infection
- in a patient with a long latent period from GAS infection, antibody levels may have declined to normal
130
What is the most sensitive imaging test to rule out septic arthritis of the hip?
Ultrasound = can detect joint effusion and fluid collection, especially in the hip joint whereas plain xrays are normal in \> 50% of cases of hip septic arthritis
131
Septic arthritis of which joint is a surgical emergency?
Hip septic arthritis = needs debridement in OR because of the vulnerability of the blood supply to the head of the femur
132
What is the most common cause of hip pain in a child?
Acute transient synovitis = diagnosis of exclusion!
133
What is the treatment for kawasaki disease?
-what about for patients who remain febrile after 1st course of treatment?
1. IVIG 2 g/kg x 1 dose and high dose aspirin (80-100 mg/kg/day div q6h) as soon as possible after diagnosis (ideally within 10 days of fever onset)
- IVIG and ASA together decrease risk of coronary artery disease
2. When child has been afebrile \> 48 hrs, then decrease dose of aspirin to 3-5 mg/kg/day single dose) x 6-8 wks until repeat ECHO and can be discontinued if ECHO is normal
\*\*\*\*If patient is still febrile despite 1 dose of IVIG and starting ASA, this is defined as IVIG-resistant KD (15% of patients)
- increased risk for coronary artery abnormalities
1. Give 2nd dose of IVIG at 2 g/kg
2. Consider IV methylprednisolone pulse therapy or cyclophosphamide or plasmapheresis
134
What are the diagnostic criteria of systemic JIA?
Requires all of the following:
1. Arthritis affecting 1 or more joints \> 6 weeks
2. Fever of at least 2 weeks duration that is daily
3. Accompanied with one of more of the following:
1. Hepato +/- splenomegaly
2. Generalized lymphadenopathy
3. Evanescent rash
4. Serositis
\*\*\*\*Arthritis may develop LATER (ie. within one year of fever) and is usually oligoarticular (knees, wrists, ankles) but can involve cervical spine and hip
135
What are predisposing factors to osteosarcoma? (4)
-where is the most common site of metastasis?
1. Radiation exposure
2. Hx of retinoblastoma
3. Benign bone conditions can morph into osteosarcmoa
4. Fibrous dysplasia
- most common site of metastasis: lungs!
136
What is the best confirmatory test for juvenile dermatomyositis?
-other tests?
EMG = denervation & myopathy
- other tests:
1. Muscle biopsy = perifascicular atrophy
2. CK can be normal or high
3. ANA: 80% can be positive
137
What are the clinical features of juvenile dermatomyositis? (6)
- 3 tests to confirm?
- initial treatment?
1. Rash: heliotrope rash, gottron's papules, shawl sign, raynaud's phenomenon = most commonly presents with this
2. Proximal muscle weakness: gradual, fatigue, decreased ADLs, positive Gower's sign, difficulty swallowing/dysphagia
3. Cardiac: dilated cardiomyopathy
4. Resp: interstitial lung disease
5. Arthritis
6. Nailbed changes diagnosed via capillaroscopy
- tests to confirm diagnosis: EMG (best test), CK/LDH/Aldolase/AST, muscle biopsy
- initial treatment: steroids plus or minus methotrexate, PFTs, PT, OT
138
What is the diagnostic criteria for enthesitis-related arthritis?
\*\*\*enthesitis: inflammation where ligament or tendon attaches to bone
- Arthritis AND enthesitis OR enthesitis or arthritis with at least 2 of the following:
1. Presence of SI joint tenderness and/or back pain
2. Onset of arthritis in a male \> 6 yo
3. Acute uveitis
4. Family history of ankylosing spondylitis, uveitis, sacroileitis with IBD
5. HLA-B27 positive
139
What are the diagnostic criteria for SLE?
- what do you see in complement level in SLE?
- what lab markers are used to monitor disease activity (2)?
SOAP BRAIN MD
-remember that these are for adults and have not been validated in children so pediatric patients may be diagnosed with SLE without meeting diagnostic criteria
Need 4/11:
1. Serositis (pleuritis or pericarditis)
2. Oral ulcers (usually painless)
3. Arthritis (non erosive)
4. Photosensitivity
5. Blood disorders: autoimmune hemolytic anemia, lymphopenia, thrombocytopenia
6. Renal nephritis: proteinuria or cellular casts
7. ANA positive
8. Immunologic: anti-dsDNA (increases clotting risk), anti-Smith, anti-ro, anti-la, antiphospholipid antibodies
9. Neurologic encephalopathy (seizures and/or psychosis)
10. Malar rash: butterfly rash sparing nasolabial folds
11. Discoid rash
\*\*\*Complement is LOW in SLE
-C3 and C4 are used to monitor disease activity = levels fall during a flare and return to normal after appropriate treatment
140
What are clinical features of osteogenesis imperfecta? (5)
-what is the diagnostic test for OI?
1. Blue sclera
2. Wormian sutures: extra bones in between sutures
3. Cortical bone thinning
4. Dentinogenesis imperfecta
5. Triangular facies
6. Late onset hearing loss in 50% of children
- diagnostic test: fibroblast testing or DNA testing
141
What are 3 ways that maternal SLE can affect neonate?
1. Rash
2. Congenital heart block
3. Thrombocytopenia
142
What are 3 long term orthopedic complications of JIA?
1. Leg length discrepancy
2. Joint deformity
3. Flexion contracture
143
What are physical exam findings of ankylosing spondylitis? (4)
1. Loss of normal lumbar lordosis
2. Positive Schober test (decreased forward flexion)
3. Anterior uveitis
4. Enthesitis (ie. patellar tendon and achilles tendon)
144
What is the pathophysiology behind acute phase reactants?
- which lab markers increase in acute phase response? (11)
- which lab markers decrease in acute phase response? (3)
Acute phase reactants: plasma proteins produced by the liver that change production during acute phase of inflammation (mediated by cytokines)
- increased lab markers:
1. CRP
2. ESR
3. Complement proteins
4. Fibrinogen
5. Ferritin
6. Ceruloplasmin
7. Haptoglobin
8. G-CSF
9. IL-1 receptor antagonist
10. Serum amyloid
11. Coag factors
- decreased lab markers:
1. Albumin
2. Transferrin
3. IGF-1
145
A patient with JIA has a positive ANA. What 3 things does this put them at risk for?
1. Earlier disease onset
2. Asymmetric arthritis
3. Uveitis
146
What level is considered a positive ANA?
-when should specific antibodies (ie. anti-dsDNA) be ordered?
\>1:160
-specific antibodies should only be ordered if ANA is positive AND a disease other than JIA is suspected (ie. SLE)
147
What 4 antibodies are SPECIFIC for SLE?
-what antibody is SENSITIVE for SLE?
- most sensitive antibody: ANA (if negative, very unlikely to have SLE
- most specific:
1. anti-dsDNA
2. anti-Ro
3. anti-La
4. anti-smith
148
What specific auto-antibodies (3) can often be seen with juvenile dermatomyositis?
1. Anti-Jo1
2. Anti-SRP
3. Anti-Mi-2
149
What specific group of auto-antibodies are associated with increased risk of thrombosis?
-two examples of these?
Antiphospholipid antibodies
- this is why people with antiphospholipid antibody syndrome are at increased risk of clots and pregnancy loss secondary to clots
- ex. lupus anticoagulant, anticardiolipin
150
How useful is rheumatoid factor (RF) in diagnosis of JIA?
-implication for prognosis?
Not useful at all!
- present in 85% of adults with rheumatoid arthritis but only 5-10% of children with JIA
- helpful in classification and prognosis of JIA but should NOT be used as screening test since arthritis is a clinical diagnosis
- RF positive JIA = higher risk of aggressive joint disease with erosions and functional disability
- children with RF negative polyarthritis are typically younger and have a much better prognosis
151
Which disease is associated with a positive c-ANCA?
Wegener granulomatosis
"C Wegener!"
152
Which diseases are associated with positive p-ANCA? (5)
1. Microscopic polyangitis
2. Churg-Strauss syndrome
3. Ulcerative colitis
4. Primary sclerosing cholangitis
5. SLE
153
Which conditions is associated with positive HLA-B27? (5)
1. JIA: enthesitis related and psoriatic arthritis
2. IBD
3. Isolated acute anterior uveitis
4. Reactive arthritis
5. Ankylosing spondylitis
154
A patient presents to you with leg pain that occurs solely at night. What is your differential diagnosis? (3)
1. Malignancy: osteosarcoma, Ewing sarcoma
2. Osteoid osteoma (benign)
3. Growing pains
155
A patient presents to you with dactylitis. What is your differential diagnosis? (3)
1. Sickle cell anemia
2. JIA (ie. enthesitis related, psoriatic arthritis)
3. Trauma
156
What are the 7 types of JIA?
-definition of JIA (2 criteria)?
JIA: needs to begin before the 16th birthday and persists for \> 6 weeks!!!
1. Oligoarthritis
2. Polyarthritis (RF negative)
3. Polyarthritis (RF positive)
4. Enthesitis related arthritis
5. Systemic JIA
6. Psoriatic arthritis
7. Undifferentiated arthritis
157
What is the most common subtype of JIA?
-most frequent joints involved? (4)
Oligoarthritis
- most frequent joints involved:
1. Knees
2. Ankles
3. Wrists
4. Elbows
158
What is the definition of oligoarthritis?
-two subcategories?
Definition: arthritis affecting 1-4 joints during first 6 months of disease
- subcategories:
1. Persistent: no more than 4 joints affected throughout disease course
2. Extended: more than 4 joints affected after first 6 months of disease
159
A young female patient of yours oligoarticular JIA with positive ANA. What does she have a high risk of developing?
-what is the chance that a patient with oligoarticular JIA will have a positive ANA?
Asymptomatic uveitis
- need ophtho consult
- positive ANA is found in 70% of patients with oligoarticular JIA
160
What is the diagnostic criteria for RF-negative-polyarthritis?
-what about RF-positive polyarthritis?
RF-negative polyarthritis:
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. RF testing negative
RF-positive polyarthritis:
1. Arthritis affecting 5 or more joints during first 6 months of disease
2. 2 or more positive tests for RF at least 3 months apart during first 6 months of disease
161
Which population is usually affected by RF positive polyarthritis?
Adolescent girls = this is essentially adult-type rheumatoid arthritis presenting in a child or adolescent
-usually involve the PIP and MCP joints
162
What is the triad of Felty's syndrome?
1. Rheumatoid arthritis
2. Neutropenia
3. Splenomegaly
163
A patient has high spiking fevers daily for the last 2 weeks. What is your differential diagnosis? (5)
-workup?
1. Infection
2. Kawasaki disease
3. Periodic fever syndromes
4. Malignancy (especially leukemia and lymphoma)
5. Systemic JIA
Work-up:
1. Full infectious workup
2. Bone marrow aspirate PRIOR to starting corticosteroid treatment for query JIA
164
What are the most common sites of involvement in enthesitis-related arthritis? (4) Insertion sites of:
Insertion sites of:
1. Achilles tendon
2. Plantar fascia
3. Patellar tendon
4. Quadriceps tendon
- can also see dactylitis, tarsitis
165
What is the diagnostic criteria for psoriatic arthritis?
-what is the clinical hallmark of this condition?
1. Arthritis AND psoriasis
OR
2. Arthritis with at least 2 of the following:
-dactylitis (clinical hallmark!!!)
-nail-pitting or onycholysis
-psoarisis in a first degree relative
166
What are the general goals of therapy for treatment of JIA? (4)
- what is the first line treatment of JIA?
- what subspecialties (2) should be consulted for all patients diagnosed with JIA?
1. Eliminate inflammation
2. Prevent joint damage
3. Promote normal growth and function
4. Minimize medication toxicity
- first line treatment: NSAIDs
- two consultants:
1. Rheumatology
2. Ophthamology to rule out uveitis (ESPECIALLY those with oligoarthritis and positive ANA)
167
Which population of JIA patients are at highest risk for developing uveitis?
ANA positive oligoarthritis
168
What is the algorithm for treatment of oligoarticular JIA?
Try NSAID --\> if no remission, try intraarticular corticosteroid injection --\> if no remission, consider methotrexate or other second line agent
169
What is the algorithm for treatment of polyarticular JIA?
Remember that this is more severe and causes worse functional impairment since it's multiple joints so you're more aggressive!
Try NSAIDs or intraarticular corticosteroid injections --\> no response --\> start second line agent right away (methotrexate, leflunomide or sulfasalazine) --\> if no response, optimize second line agent and consider IAC or low dose PO steroids as bridging therapy --\> if no response, consider biologic anti-TNF therapy
\*\*\*May need to start a second line agent as part of initial therapy for children with severe polyarthritis
170
What is the algorithm for treatment of systemic JIA?
Try NSAIDs first OR for severe disease, consider starting corticosteroids or biologic agent (anti-IL-1 or anti-IL-6) right away
-if tried NSAIDs and no response --\> systemic corticosteroids --\> if no response --\> add biologic anti-IL-1 or anti-IL-6 therapy
\*\*\*\*note that you do not use methotrexate/sulfasalazine/etc.
171
What are the treatment options for SLE?
Overall: use minimum required treatment to maintain clinical and laboratory quiescence. More aggressive treatment needed for severe organ involvement
1. Hydroxychloroquine: standard therapy for SLE; decreases frequency and severity of disease flares
2. Corticosteroids: often used in initial therapy for SLE (especially for severe lupus nephritis, hematologic crisis, CNS disease)
3. Azathioprine: used for hematologic and renal manifestations
4. Mycophenolate mofetil: used for hematologic, renal and CNS manifestations
5. Cyclophosphamide: used for severe renal and CNS manifestations
6. Rituximab: used for resistant thrombocytopenia
172
What are possible long term complications of SLE?
1. Death from infection, renal, CNS, cardiac or pulmonary disease
2. Early onset coronary artery disease from vasculitis
3. Bone disease --\> osteopenia, avascular necrosis from steroid use
4. Malignancy from use of immunosuppressants/biologics
173
What is the underlying pathophysiology of neonatal lupus erythematosus?
- in a mother with the specific autoantibodies, what is the incidence of NLE in their infants?
- what is the risk of recurrence for future pregnancies if one child has been affected?
Transplacental passage of maternal autoantibodies (anti-Ro and anti-La) = remember that mothers of infants with NLE MAY have SLE but also may be healthy with no known autoimmune disease
- incidence: 1-2% of children with mothers with anti-ro or anti-la antibodies
- risk of recurrence: 16%
174
What are the clinical features of NLE?
1. Cardiac: complete congenital heart block (associated with non-immune hydrops)
2. Annular, erythematous papulosquamous rash with fine scale and central clearing
- usually photosensitive
- can be present at birth or develop within first few weeks of life
- predilection for face and scalp
- self-resolving
3. Hematologic: thrombocytopenia (most common), neutropenia and anemia less common
4. Hepatic involvement: cholestatic hepatitis with elevated liver enzymes, hepatomegaly
5. Neurologic: macrocephaly, hydrocephalus, spastic paraparesis, vasculopathy
175
What is the treatment for NLE
If fetal bradycardia is found during pregnancy, need fetal ECHO and may require treatment with Dex +/- sympathomimetics
-neonates with complete heart block may need pacemaker
176
What are types of:
- large vessel vasculitis (2)
- medium vessel vasculitis (2)
- small vessel vasculitis (4)
Large vessel vasculitis:
1. giant cell arteritis (older adults)
2. takayasu arteritis
Medium vessel vasculitis:
1. Kawasaki
2. Polyarteritis nodosa
Small vessel vasculitis:
1. Wegener's granulomatosis
2. Churg-Strauss
3. Microscopic polyangitis
4. HSP
177
What are the clinical features of takayasu arteritis?
- diagnostic criteria?
- treatment?
Large vessel vasculitis involving the aorta and its branches (thoracic, abdominal, carotid)
-initially presents as non-specific inflammatory illness with fever, then evolves into chronic, fibrotic phase with signs and symptoms of chronic vascular insufficiency (pulse deficit, claudication, BP discrepancy, bruits)
\*\*\*Diagnostic criteria:
Needs:
1. Angiographic abnormalities (MRI) of aorta or its main branches (aneurysm/diltation, narrowing or thickened arterial wall)
AND
1 of the following:
1. Pulse deficit or claudication
2. Discrepancy of 4 limb BP \> 10 mm Hg
3. Bruits
4. Hypertension
5. Acute phase reactants elevated (ESR or CRP)
-treatment: corticosteroids plus second line agent: cyclophosphamide, methotrexate, etc.
178
What is the diagnostic criteria for:
- incomplete KD
- atypical KD
- alternate way to make diagnosis for KD aside from the 5 or more days of fever + 4/5 features
Incomplete KD: if 5 or more days of fever with 2-3 features instead of 4
-commonly seen in infants who are at higher risk of coronary artery involvement
Atypical KD: if KD with unusual manifestation (eg. renal failure)
Alternate way to diagnose KD: in presence of fever AND coronary artery involvement on echo,
179
What is the usual age group for KD?
-what is the pathophysiology?
3 mo-5 yo
-small vessel vasculitis with predilection for coronary arteries; thought to be triggered by infectious agent
180
What are less common clinical manifestations of KD (ie. clinical features other than those in diagnostic criteria)? (8)
1. Irritability (aseptic meningitis)
2. Arthritis
3. Sterile pyuria from urethritis
4. Gastroenteritis (abdo pain, vomiting, diarrhea)
5. Uveitis
6. Gallbladder hydrops
7. Myocarditis/pericarditis
8. MAS
181
What is the most worrisome complication of KD and when does this occur?
- risk factors for developing complication? (6)
- if untreated, what is the chance of developing this complication? What does the risk change to if treated?
Coronary artery aneurysms = occur 6-8 wks after acute illness
- risk factors:
1. Males
2. Infants 9 yo
3. Prolonged fever
4. Asian or hispanic ethnicity
5. Thrombocytopenia
6. Hyponatremia
- if untreated: 25% will develop coronary artery aneurysms
- if treated (IVIG given within 10 days): decreased to 4%
182
What is the treatment of KD with myocarditis?
-what about KD with large coronary aneurysm?
KD with myocarditis: add corticosteroids right away to first dose IVIG
-KD with large coronary aneurysm: abciximab (glycoprotein IIb/IIIa receptor inhibitor) in acute phase and long term antiplatelet therapy
183
What is the diagnostic criteria for Wegener Granulomatosis?
\*\*\*Overall, think small vessel vasculitis primarily affecting upper airway, lungs, and kidneys!!!!
Need 3/6:
1. Histopathology showing granulomatous inflammation within wall of artery
2. Upper airway involvement: chronic purulent or bloody nasal discharge, recurrent epistaxis, nasal septum perforation, saddle nose deformity, chronic sinus inflammation
3. Laryngo-tracheo-bronchial involvement: subglottic, tracheal or bronchial stenosis
4. Pulmonary involvement: nodules, cavities or pulmonary infiltrates (SOB, chronic cough, hemoptysis/alveolar hemorrhage)
5. ANCA positive
6. Renal involvement: proteinuria, elevated Cr, hematuria
184
What is the treatment for Wegener granulomatosis? (2)
Initial therapy: Corticosteroids and cyclophosphamide
Maintenance therapy: methotrexate, azathioprin, mycophenolate and low dose corticosteroids
185
What is the diagnostic criteria for HSP?
Need:
Palpable purpura or petechiae with lower limb predominance
AND
1 or more of the following:
1. Diffuse colicky abdominal pain with acute onset (can include intussusception, GI bleed) = 50-75% of patients
2. Skin biopsy showing IgA deposits in leukocytoclastic vasculitis OR kidney biopsy showing proliferative glomerulonephritis with IgA deposits (THIS IS REQUIRED IF PURPURA IS IN ATYPICAL DISTRIBUTION)
3. Arthritis or arthralgia of acute onset (75% of patients, usually knees/ankles with edema)
4. Renal involvement: proteinuria, hematuria impaired renal function
186
What is the most common vasculitis in children?
HSP
187
What does the prognosis of HSP depend on?
Depends on severity of nephritis (worse prognosis with nephrotic syndrome)
-end stage renal disease occurs in 1-3% of patients
188
What is the diagnostic criteria for Behcet disea
Needs:
1. Recurrent oral ulcers
AND
2 or more of the following:
-recurrent genital ulcers
-eye lesions (uveitis)
-skin lesions (erythema nodosum, pseudo vasculitis, etc.)
-Pathergy (skin papule 2 mm or more in size developing 24-48 hrs after insertion of 20-25 gauge needle into the skin)
189
What are the diagnostic criteria for juvenile dermatomyositis? (5)
- what can be seen on capillaroscopy?
- what organ systems aside from MSK/neuro are involved?
1. Symmetrical proximal muscle weakness
2. Characteristic skin changes: gottron's papules on dorsal surface of the knuckles, heliotrope rash over the eyelids
3. Elevated muscle enzymes (CK, AST, LDH, aldolase)
4. Abnormal EMG demonstrating denervation and myopathy
5. Abnormal muscle biopsy demonstrating necrosis and inflammation
\*\*\*\*capillaroscopy: looks at changes in the nail fold capillaries (tortuosity, dilatation, dropout)
Other organ systems:
1. Cardiac: cardiomyopathy (rare)
2. Interstitial lung disease
3. GI tract symptoms: ulceration, perforation
4. Arthritis
190
What are important questions to ask on clinical history to elicit proximal muscle weakness?
- Difficulty getting up from sitting or lying, difficulty climbing stairs, frequent falls
- difficulty swallowing, choking on foods = weakness of palate and cricopharyngeal muscles
191
What is the treatment for juvenile dermatomyositis?
-what are the 2 best predictors of longer time to remission?
1. Supportive: nutrition, physiotherapy, sunscreen for photosensitive rash
2. Medications: -steroids 1-2 mg/kg/day with slow taper
- methotrexate
- if refractory or resistant: IVIG, cyclosporine, or rituximab
- 2 predictors for longer time to remission:
1. Nail fold abnormalities
2. Persistence of rash
192
What is CREST syndrome?
-most important complication?
Aka Limited systemic sclerosis
1. Calcinosis
2. Raynaud phenomenon
3. Esophageal dysmotility
4. Sclerodactyly
5. Telangiectasias
- most important complication: future development of pulmonary hypertension
193
What are the precipitants of raynaud phenomenon?
- causes?
- treatment?
Precipiants: cold, physical or emotional stress, caffeine, smoking
Causes:
1. Primary: no underlying etiology
2. Secondary: due to underlying autoimmune diseases (scleroderma, mixed connective tissue disease, SLE, etc.), drugs, toxins, polycythemia
Treatment:
- avoid precipitants
- may need nifedipine (peripheral vasodilator)
194
A patient presents to you with isolated Raynaud phenomenon. What are the 2 best predictive factors for future development of autoimmune diseases?
1. Positive ANA
2. Abnormal nail fold vasculature
195
What is Morphea?
Group of disorders with skin and subdermal connective tissue changes due to excessive accumulation of collagen
196
What is the most common form of localized scleroderma inpediatric population?
Linear scleroderma: characterized by one or more linear streaks (following dermatomal distribution) extending over upper or lower extremity
- can involve face or scalp (en coup de sabre), usually forehead
- Parry-Romberg syndrome: progressive hemi-facial atrophy, disfiguring
197
What is the minimum duration of fever to be called fever of unknown origin?
Fever needs to last \> 14 days with standard investigations not resulting in a clear diagnosis
198
What 3 autoimmune conditions can be complicated by MAS?
1. Systemic JIA
2. SLE
3. Kawasaki disease
199
What are the clinical features of familial mediterranean fever?
- hallmark?
- treatment?
1. Fever episodes x 1-3 days q4-8 wks (very predictable)
2. CLINICAL HALLMARK = serositis (peritonitis, pleuritis, synovitis)
3. erysipelas-like rash on shins and dorsum of feet
4. MSK: monoarthrtis, myalgia
- treatment: colchicine
200
What are the clinical features of TRAPS?
-treatment?
TNF-receptor associated periodic syndrome
1. Long duration of fevers lasting 3-4 weeks and occur at irregular intervals
2. Migrating erythematous, maculopapular rash
3. Severe migratory myalgias with rash, arthralgias
4. Conjunctivitis, periorbital edema
5. Severe abdo pain
- treatment: unproven therapy; steroids provide symptomatic relief but do not change frequency
201
What are the clinical features of PFAPA?
-treatment?
Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis
-usually starts before 5 yo and is self-limited
Clinical features:
1. Episodes of high fever that occur with regular periodicity q4-6w
2. Fever usually up to 5 days
3. non scarring aphthous ulcers, pharyngitis, cervical adenitis with negative throat cultures
-treatment: single dose of corticosteroid stops symptoms but really, no consensus regarding treatment
202
What are complications of uncontrolled uveitis? (3)
1. Cataracts
2. Glaucoma
3. Vision loss
203
What is the usual age of onset of growing pains in children?
- clinical features?
- treatment?
4-10 yo
- deep aching cramping pain in bilateral thighs or calves, usually at night, often awaken from sleep
- treatment: improves with gentle massage, heat and/or analgesia with acetaminophen
- symptoms disappear by morning
- normal physical exam
204
What is the diagnostic criteria for fibromyalgia?
-treatment?
hi
205
How does colchicine work in FMF?
Binds to microtubules to prevent activation, proliferation and functioning of inflammatory cells
206
What are examples of DMARDs? (7)
DMARDs = disease-modifying anti-rheumatic drug
1. Azathioprine
2. Cyclophosphamide
3. Cyclosporine
4. Hydroxychloroquine
5. Methotrexate
6. Mycophenolate mofetil
7. Sulfasalazine
207
What monitoring needs to occur when patients are on hydroxychloroquine?
Eye exams q6mo to assess for retinal deposits (retinal toxicity)
208
What are the two most specific tests for diagnosing SLE?
1. anti-ds dna
2. anti-smith
209
What are clinical features of serum sickness?
-tx?
Immune complex mediated hypersensitivity vasculitis = type 3 hypersensitivity reaction, occurs 1-3 wks after exposure to agent (usually an antibiotic)
- clinical features: fever, urticaria, looks unwell, malaise, lymphadenopathy, arthritis, GI s/s
- dx: decreased C3/C4, increased ESR, decreased platelets
- tx: supportive care, especially with benadryl and NSAIDs. Steroids if severe symptoms
210
A patient with JIA or SLE suddenly becomes extremely unwell with fever, splenomegaly, and pancytopenia. What is the most worrisome possible diagnosis?
Macrophage activation syndrome
211
most common benign tumor in childhood?
nonossifying fibroma.
212
Ricketts bone finding
* Femoral and tibial bowing
* Delayed suture and fontanel closure
* Pectus carinatum or “pigeon breast” (anterior protrusion of the sternum)
* Frontal thickening and bossing of the forehead
* Defective tooth enamel
* Harrison groove (a rim of rib indentation at the insertion of the diaphragm)
* Widened physes at wrists and ankles
* “Rachitic rosary” (enlarged costochondral junctions)
213
McCune-Albright syndrome is associated with what skeletal abnormalities?
Polyostotic fibrous dysplasia
214
Is in-toeing a problem?
The majority of cases of in-toeing are not pathologic problems.
215
A 15-year-old with tibial pain (worse at night and relieved by nonsteroidal anti-inflammatory drugs) has a small lytic area surrounded by reactive bone formation on x-ray. What is the likely diagnosis?
Osteoid osteoma, a benign bone-forming tumor
216
Ortho emergencies
1. Open fracture
2. Impending compartment syndrome
3. Dislocation of major joints
4. Septic arthritis
5. Arterial injury
217
What is the most common congenital foot abnormality?
Metatarsus adductus
forefoot is turned toward the midline as a result of adduction of the metatarsal bones at the tarsometatarsal joints.
Most cases are mild and flexible, with the foot easily straightened by passive stretching.
218
How is metatarsus adductus treated?
a program of passive stretching is in order
219
How is clubfoot distinguished from severe metatarsus adductus?
If the ankle can be dorsiflexed to neutral or beyond, metatarsus is the most likely diagnosis.
220
How are clubfeet treated?
Most clubfeet respond well to serial casting using the Ponseti method.
221
What is LCP disease?
is a disorder of the femoral head of unknown etiology that is characterized by ischemic necrosis, collapse, and subsequent repair
222
Reverse tailor position or “W” position
This is femoral anteversion (or medial femoral torsion), which is a common cause of in-toeing in younger children. The child is demonstrating the reverse tailor, or “W” position, which is a sign of the internally rotated hip.
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What symptoms do children with slipped capital femoral epiphysis (SCFE) have?
progressive displacement of the hip with external rotation of the femur on the epiphyseal growth plate. The patient has intermittent or constant hip, thigh, or knee pain that has often been present for weeks or months
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What systemic conditions are associated with SCFE?
hypothyroidism, panhypopituitarism, hypogonadism, rickets, and irradiation.
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