Peds Flashcards
** Peds joint pain differential **
PRIME BONE PAIN
-P: pharm: serum sickness, DLE
-R: rheum: JIA, SLE, SS, SSc, MCTD, vasculitis, DM, sarcoid
-I: infxn: bacterial (OM, diskitis, septic, rheumatic fever, ReA, lyme), viral (hep b/c, hiv, parvo, EBV, herpes, rubella)
-M: metabolic/genetic: marfan
-E: episodic: autoinflammatory (FMF, HIDS, TRAPS, CAPS, BLAU, PAPA, DIRA)
-B: blood/heme: SS, hemophilia
-O: ortho: osgood schlatter (apophysitis of tibial tubercle), Perthes, SCFE
-N: neoplastic: leuk/lymphoma, sarcoma, osteoma, osteochondroma, mets, neuroblastoma
-E: endocrine: hypothyroid, hypercortisol, DM, ricketts
-P: CRPS, fibro
-A: accidental/trauma
-I: inflamm: IBD
N: normal variant (growing pain), hypermobility
** DDX Fever & polyarthritis **
Polyarticular JIA (RF+, RF-)
-Systemic JIA, SLE, MCTD
-ERA, Juvenile PsA, ReA
-Sjogrens
-Sarcoidosis
-Rarely Lyme disease
-Acute rheumatic fever (ARF), Post Strep arthritis
-Endocarditis
-Parvo, EBV
-Sickle cell disease
-Leukemia/lymphoma
-Serum sickness
Organic vs nonorganic joint pain in Peds
Day+night (vs only night)
-Wkend + vacay (vs only school days)
-Interrupts play (vs can carrying normal activities)
-In joint (vs between joints)
-Limbs/refuses to walk (vs bizarre gait)
-Description fits anatomic explanation (vs illogical or dramatic)
-Other signs of systemic illness (vs isolated pain in othewise well)
DDx acute monoarthritis peds
Early rheum dz: oligoarticular JIA, ERA
-Infxn: septic, ReA, Lyme
-Ca: Leuk, neuroblastoma,
-Hemophilia
-Trauma
-Gout
DDx chronic monoarthritis peds
JIA: ERA, juvenile PsA, oligoarticular
-Sarcoid
-Infxn: TB, lyme
-Hemarthrosis: PVNS, hemophilia hemangioma
-Noninflamm: lymphangioma, synovial chondromatosis, lipomatosis arborescens
DDx chronic polyarthritis peds
-Polyarticular JIA, Sjogren’s
- ERA, Juvenile PsA
-SLE, MCTD
-Inxn: rheum fever, ReA, Lyme,
-Sarcoid
-Psueodorheumatoid chondrodyspasia
-Mucopolysacch
Synovial fluid findings
– SLE
– ReA
– Tb
– Septic arthritis
SLE: LE cells
-ReA: Reiter cells
-TB arthritis: acid fast bacteria (shld do biopsy)
-Septic arthritis: low glucose, bacteria, >75% PMN, 50k-300k WBC
Measuring leg length in Peds
ASIS to medial malleolus
When to suspect leukemia causing polyarthritis
High ESR
-LOW platelet
-High LDH, uric acid
-Abnormal smear
Cancer with MSK manifestations Peds
Leukemia
-Neuroblastoma
-Ewing sarcoma (monoarticular)
-Lymphoma
Hip pain ddx Peds
Cancer: osteoid osteoma, ewing sarcoma, leukemia, neuroblastoma
-Infxn: Septic arthritis, OM, rheumatic fever
-Autoimmune: JIA (ERA, JAS), ReA,
-Trauma, Fracture, AVN
-SCFE, Perthes, Protrusio acetabili
Back pain ddx peds
-Acute diskitis (Viral, Staph, Enterobacter, Moraxella)
-Disk herniation
-Spondylolysis +/- spondylolisthesis
-Juvenile discogenic disease
-Cancer (mets, primary bone tumor, leukemia)
** Main types of JIA**
-sJIA
-Oligoarthritis
-Polyarthritis (RF+ and RF-)
-PsA
-ERA/JAS (juvenile spondyloarthropathy)
-Undifferentiated arthritis
** JIA pathogenesis - interleukins involved**
-IL1
-IL6
-IL18
DDx for migratory arthralgia
-Rheumatic fever
-Poststep arthritis
-Gonococcal arthritis
-Lyme
Rashes specific to juvenile arthritis
Erythema marginatum = rheumatic fever
-Lower extremity purpura = HSP (IgA vasculitis)
-Evanescent salmon pink macules = sJIA
** sJIA (aka AOSD) clinical characteristics**
Arthritis in 1+ joints AND quotidian fever x 2+ wks and 1 of:
-Lymphadenopathy
-Evanescent Rash
-Hepato / splenomegaly
-Serositis (pleural, pericardial, peritoneal)
** Cytokines involved in sJIA**
IL 1, 6, 18 , TNF
**DDx sJIA **
-SLE, JDM, KD, AAV, PAN
-Sarcoid
-Autoinflammatory: FMF, cryopyrin assoc’d periodic syndromes, TNF-R assoc’d periodic ever syndrome
Infxn (any causing quotidian fever): endocarditis, bartonella, brucellosis, mycoplasma, rheumatic fever, TB
-Malignancy (solid/heme)
MAS manifestations
Fevers, LN
-CNS: sz, coma, ataxia, PRES
-Rash
-Hypotension
-ARDS
-Liver dysfcn (jaundice), HSM
-Cytopenias, Bleeding, bruising, purpura
-Renal dysfunction
MAS pathophys
Abnormal immune response to infection or autoantigen → exaggerated inflamm response
-Continual expansion of T lymphocytes and macrophages → increased proinflammatory cytokines, IL1/6
MAS causes
-Genetics
-Rheum: SLE, JIA, Kawasaki, DM, APS, MCTD
-Cancer
-Infxn: eg EBV
-Idiopathic
MAS Ix
Cytopenias, DIC (prolonged PT/PTT)
-High TG, Ferritin, soluble IL2R
-Liver dysfunction → transaminitis, prolonged PT/PTT, LOWER ESR and fibrinogen
-LP: CSF inflammatory
-Bone marrow Bx (hemophagocytosis)
-MRI brain
-Viral/Autoimmune serologies
MAS Biopsy
Macrophages show hemophagocytosis in BM, LN, or liver
HLH/MAS Tx
GC
-IVIG
-CNI
-Etoposide (chemo)
-Anakinra (Toci if IL1 failure or contraindication)
-PLEX
-Ruxolitinib (JAKi)
-Ritux
-Treat virus/bacteria/cancer
** MAS vs sJIA**
MAS not JIA:
– Cytopenia
– Hemophagocytosis on biopsy
– HyperTG
– CNS symptoms
– Liver dysfunction
– Hypofibrinogen and ESR
– Prolonged PT/PTT
– Bleeding, purpura
– Resp distress: ARDS
sJIA Tx
Not active:
– SJC<4 = NSAID or IA steroids x1mo → antiIL1/6/TNF, abatacept if refractory
– SJC>4 = MTX/LFN x3mo → biologic as above
-
-Mild active (physician global <5):
– NSAID → GC, anakinra if activity >1mo
-
-Moderate Active:
– Anakinra → toci/canakinumab if refractory @ 1mo;
-OR
– GC → Anakinra/Toci/canakinumab if refractory @ 2wks (+MTX/LN if persistent)
** Outcome measure for JIA **
Childhood HAQ (CHAQ)
-For longitudinal studies and clinic ax
-JAQQ for prospective studies
** Desirable features of outcome measures for JIA**
Practical, easy to use
-Short completion time by child/parent
-Measures social/psychological fcn
-Measures pain
-Reliable, valid, responsive
-Adaptable for international use
Oligoarticular JIA manifestations
1-4 joints during 1st 6mo of disease
-Asymmetric, knee >ankle > wrist (RARE hip/back)
-Chronic nongranulamtous anterior uveitis (asymptomatic) in ANA+ and girls <7yo
Oligoarticular JIA subtypes
Persistent : never more than 4 joints involved
-Extended: more than 4 joints ater 1st 5 mo
Oligoarticular JIA labs
ANA+
-RF-
-Normal WBC
-Mild inflamm markers
Anterior uveitis in JIA
-types and presentation
- complications and screening
Acute: Spondyloarthropathy with HLAB27; pain and redness with few complications and not requiring screening
Chronic: Oligoarticular JIA with ANA; ASYMPTOMATIC → cataract, glaumcoma, band keratopathy REQUIRES SLIT LAMP SCREEN
Poorly localized leg pain sufficient to interrupt sleep and cause a limp, must rule out:
Leukemia
-Lymphoma
-Neuroblastoma
-Osteosarcoma
Oligoarticular JIA Tx
NSAIDs and IA steroids → MTX → TNFi
Polyarticular JIA RF+ vs RF- subgroup differences
RF- : insidious/progressive, asymmetric larger joints, TMJ, chronic anterior uveitis, ANA+ (or ANA- symmetrical polyarthritis)
-RF+: faster onset, symmetric small joints (RA deformities), MORE joints, nodules, vasculitis, felty, RA lung dz, ANA+, ACPA+
RF- polyarticular JIA subgroups
ANA+ girls <7yo: oligoarticular JIA but more joints in 1st 6mo, high risk chronic uveitis
-ANA- children >7-9yo: RA like (~RF+ subgroup) w/ symmetric polyarthritis
Differentiate RF-/ANA- and RF+ polyarticular JIA from adult RA
Peds:
– More hip, shoulder, C spine, DIP than adults
– Fusion of bones, micrognathia, fusion of C spine apophyseal joints
Polyarticular JIA Tx
Low dz = NSAID
-Mod-High dz = DMARD (MTX, SSZ, LFN)
-Biologic (TNF, Toci, abatacept, ritux) if refractory, TNFi monotherapy or in combo w/ MTX
Juvenile PsA classsifcation criteria
<16yo w/ dz duration > 6 weeks:
-Psoriasis + Arthritis OR
-Psoriasis OR Arthritis with 2 of:
-Dactylitis
-Enthesitis
-Onycholysis or pitting (minimum 2 pits on 1+ nail)
-FamHx Psoriasis
-*excludes: sJIA, RF+, 1st deg fam hx of seroneg/ERA/anterior uveitis, HLAB27+ w/ arthritis >6yo
** ERA classification **
Child <16yo
-Arthritis (>6wks) AND Enthesitis
-OR
-Arthritis OR Enthesitis + 2 of following
-And 2 of:
-Inflamm back pain
-HLAB27+
-ACUTE anterior uveitis
-1st deg Fam Hx w/ seroneg (PEAR), ERA, uveitis
-*exclusion: personal or fam hx of PsO , positive RF, or known sJIA
** What 3 clinical features apart from sacroiliitis and enthesitis would differentiate ERA from seronegative polyarthritis on one hand and from psoriatic arthritis on the other hand?**
Seroneg
– IBD
– ??
-
-PsA
– Dactylitis
– Nail pitting
– Psoriasis
– Fam Hx of PsO
ERA Tx
NSAIDs
-IA GC
-SSZ
-MTX
-TNFi if axial or refractory
** Diff between child and adult SLE **
Child SLE:
MORE active at presentation & over time
-MORE likely to have ACTIVE renal dz
-MORE intensive drug therapy (HIGHER daily steroid dose)
-MORE severe disease phenotype & damage
-MORE malar rash, chorea
Child SLE Tx
HCQ (5mg/kg)
-Pred (1-2mg/kg bc higher metab)
-IV methylpred (30mg/kg; max 1g)
-Aza (1-2mg/kg divided BID)
-CYC (1-2mg/kg; Eurolupus 500mg/m2 q2wks x6)
-MMF 1200mg/m2 divided BID (max 1g BID)
-Cyclosporin 6mg/kg divided BID (caution HTN, renal dz)
Child SLE Tx indication PLEX
Life-threatening/ Tx-resistant
- Refractory nephritis
- DAH
- NPSLE eg transverse myelitis
- TTP
- CAPS
- Cryo
Child SLE Tx indication IVIG
CAPS
-MAS
-Immune hemolytic anemia/ thombocytopenia
Neonatal lupus manifesetations
Photosensitive rash (discoid/subacute cutaneous lupus rash)
-Hepatic dysfcn
-Cytopenia (Neut, Plt)
-Heart block (btwn 18-28 wks)
-Fetal hydrops (cardiomyopathy)
Neonatal lupus pathophysiology
Transplacental passage of maternal anti-Ro/La IgG AB
NLE heart block screening
Weekly pulsed doppler echo from 16-28 wks
NLE heart block Tx
1st / 2nd degree or pericardial effusion = fluorinated steroids eg Dex or betamethasone
-3rd deg: pacemaker
NLE heart block prevention
HCQ at 6wks GA if previous CHB child or positive Ro/LA
Sjogren’s DDx in children
-Benign recurrent parotid swelling
-Acquired immunodeficiency
-Sarcoid
-IgG4
-TB, Mumps
-Lymphoma
MC SVV childhood vs MC vasculitis
HSP = MC SVV
-KD = MC vasculitis
**HSP manifestations and duration **
Purpura, +1 of:
– Colicky abdo pain,
– Arthritis
– GN (proteinuria, RBC casts, hematuria)
– Testicular swelling/edema
-1-4 months
-Recur in 1/3
-1% ESRD (higher in adults)
**HSP criteria **
Classification
-MANDATORY palpable purpura/petechia WITHOUT thrombocytopenia/coagulopathy, plus at least ONE:
-
– Acute diffuse abdominal pain
– Acute arthritis or arthralgia
– Kidney involvement (any proteinuria, or hematuria) and IgA deposition
– Any biopsy sample showing LCV, or proliferative GN with predominant IgA deposition
HSP pathophys
After URTI, AAV, Crohns’ = inflamm at mucosal surface → IgA (role in mucosal immunity) aggregates with IgG to form complexes that deposit in tissue → SV LCV
HSP labs
Elevated serum IgA
-Normal complements
-Proteinuria
** HSP Tx **
Tx infxn
-Most = supportive care
-Purpura: dapsone
-Arthritis: Tylenol > NSAID if GIB
-Abdo pain or bleeding: prednisone 1mg/kg
-Nephritis:
– GC (Pulsed if proteinuria >1g/d, nephrotic syndrome or >50% crescents) w/ MMF, Ritux, CNI
– ACEi
–+/-PLEX
-Recurrent hospitalizations: B cell depletion (ritux)
HSP dx
Skin bx shows LCV w/ predominantly IgA vessel wall deposition
HSP poor prog features
-Melena
-Persistent rash
-Hematuria w/ proteinuria >1g/d
-Nephrosis / renal insuff
Enzyme deficiency in childhood PAN
DADA2 (adenosine deaminase 2 deficiency)
DADA2 manifestations
Vasculitis, ie:
-Fever
-Livedo reticularis
-hemorragic/ischemic strokes
-PNS involvement eg neuropathy
-HSM,LN
-immunodeficiency
DADA2 labs
Hypogammablobulin
-Cytopenias
DADA2 pathophys
ADA2 = growth factor in endothelial and hematopoeitic cell development,
-Induces monocyte prolif and macrophage differentiation (more M1 proinflammatory macrophages)
DADA2 Tx
Anti TNF
-GC,AZA, CNI, CYC, MTX, MMF variable success.
-Hematopoeitic stem cell transplant if treatment resistant
JDM manifestations
Muscle weakness: proximal, palate, swallowing → choking/cough/aspiration
-Rash: heliotrope, gottrons, telangectasia, capillary dropout/dilatation
-Abdo pain, GIB, intestinal vasculitis/bowel perf
-Arthralgias/arthritis
-Decreased DLCO
JDM biopsy results
Inflammation and/or fiber necrosis
-Perifascicular atrophy
-SVV
JDM EMG
Spontaneous fibrillation
-Increased insertional activity
-Small muscle unit action potential
JDM diff from adult DM/PM
PM rare in childhood
-Rare malignancy in children
Chronic sequelae JDM
NXP2: Subcutaneous Calcinosis
-TIF1: Lipodystrophy → metabolic syndrome, insulin resistance, hirsutism, clitoroemgaly, acanthosis nigricans
Child CTD infxn mimicks
Acute rheumatic fever
-Parvovirus, EBV
-Humoral/combined immunodeficiency
-Lyme
-HIV
Kawasaki Disease (KD) diagnostic criteria
-1. Conjunctivitis (bilateral, bulbar, nonexudative)
-2. Oral changes (erythema, fissuring, cracking, bleeding, oropharyngeal erythema w/o exudate, strawberry tongue)
-3. Cervical LN (anterior cervical triangle, unilateral)
-4. Rash (multiforme, diffuse maculopapular, erythoderma, urticarial,
-5. Extremity changes (erythema & desquamation palms/soles)
KD investigations
Elevated ESR/CRP, WBC >15, Plt >450
-Anemia
-Alb <3
-ALT up
-Urine WBC >10/HPF
-Echo
KD organ systems
MSK
-Cardio
-Ocular
-Resp
-Gi
-Nervous system
-Skin
-Heme
KD MSK manifestations
Oligoarthritis
KD Cardiac manifestations
Myopericarditis
-Aortic root enlargement
-Medium artery aneurysm
-Shock
KD Resp manifestations
Cough
-Hoarseness
KD GI manifestations
N/V/D/Abdo pain
-Pancreatitis
-Hepatitis
-Gallbladder hydrops
KD GU manifestations
Urethritis
-Sterile pyuria
KD Ocular manifestations
Anterior uveitis
KD Skin manifestations
Inflamm at previous BCG immunization site
Erythematous mouth and pharynx, strawberry tongue or red, cracked lips
Polymorphous exanthem (morbilliform, maculopapular, or scarlatiniform)
Swelling of hands and feet with erythema of the palms and soles
KD Heme manifestations
MAS
KD Echo findings, what is considered positive
-Positive if Z score >2.5 OR
-3 of the following:
– LV dysfunction
– Mitral regurg
– Pericardial effusion
– Z score between 2-2.5
KD DDx
-Staph/strep toxin mediated dz (scarlet fever, toxic shock)
– Viral: measles, adeno/enterovirus, EBV
– D hypersensitivity: SJS
– Rheum: sJIA
– Other: ricketts, leptospirosis, mercury poison
KD Tx
IVIG 2g/kg (w/i 10d onset prevents aneurysm)
-+ASA (30-50 or 80-100mg/kg in 4 divided doses)
-ASA→ 3-5mg/kg once fever resolved; discontinue after 6 wks when labs normal
-Refractory = add infliximab, or steroids
Refractory KD Tx
2nd dose IVIG
-Infliximab (5mg/kg)
-High dose pulsed steroids (20-30mg/kg)
-Anakinra, Cyclosporine, PLEX
KD FU
No coronary artery lesion:
-Echo @ 1-2wks, 6wks (can DC ASA if normal), and 1 yr after DC
-Then q5y stress test w/ lipid profile
** 3 ways in which pediatric patients with APL differ from adult patients with APLAS.**
Children
– Higher rates of LAC and anticardiolipin IgM
– More peripheral DVT and brain clots (CVST, stroke) bc Higher assoc w/ SLE
– Less RF like DLPD, HTN, smoking, E2
– CAPS as 1st presentation is more common
– More likely infxn as trigger
– Avoid ASA given risk of bleeding and Reyes syndrome
**14-year-old boy presents with a 2 week history of fever, weight loss, ankle swelling, tender nodules on calves, and a cough.
-What is the most likely diagnosis?
-List 4 tests to confirm your diagnosis.
-
Sarcoid
-CBC, CXR, ESR/CRP, ANA, Blood Cx, ASOT, Throat culture, Biopsy, Ca
** List 6 other diagnoses for hypermobility?**
-EDS (classic, hypermobile, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis types)
–Loeys-Dietz Syndrome (Aortic aneurysm with arterial tortuosity, hypertelorism, bifid uvula or cleft palate)
–Marfan syndrome (marfanoid habitus, arachnodactyly, arm span/heigh ratio >1.05, ectopia lentis, aortic dilatation or aneurysm, pectus)]\
–Osteogenesis imperfecta (blue sclera, bone fragility, SNHL) – can have overlap with EDS
–Downs
–Turners
–Arterial Tortuosity Syndrome (tortuosity and elongation of large and medium-size steries with propensity towards aneurysm formation, soft/velvety/hyperextensible skin, dysmorphic facial features, abdominal hernias)
–Lateral Meningocele Syndrome (widespread spinal lateral meningoceles, facial dysmorphism, MSK pain)
-
**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-a) What is the diagnosis: **
ALPS – autoimmune lymphoproliferative syndrome (Canale–Smith syndrome) -
-Lymphadenopathy,
-Hepatosplenomegaly,
-Autoimmune hemolytic anemia
-Thrombocytopenia
**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-b) Name 3 characteristic genetic features **
Fas germline (autosomal dominant) or somatic mutation
-Fas ligand germline mutation
-Caspase germline mutation
-
**A 6 year old boy is referred to you because of hepatosplenomegaly and lymphadenopathy. He has had these present since age 3 years. He has had splenectomy for persistent thrombocytopenia. Several female family members have had splenectomies.
-c) Name 3 clinical features apart from thrombocytopenia and mechanism for each **
Lymphadenopathy and HSM: lymphoid cell proliferation due to Fas abnormality.
-Autoimmune hemolytic anemia and neutropenia: (+DAT )
-HL, NHL: overactive lymphoid cells.
Gaucher clinical features
Lysosomal storage disease causing infiltration and accumulation of lipid laden macrophages in bone, lung, organs →
-AVN
-ILD
-HSM
**What is the differential diagnosis of Stroke in young patient **
SLE, APS
-Takayasu’s arteritis, PACNS, PAN, GPA
-TB, syphilis, VZV, fungal, meningitis
-IE, PFO
-Sickle cell, Prot C/S deficiency, Factor V leiden
-Moya moya, FMD, Dissection (EDS, Marfan)
-CADASIL, DADA2
**Marfan clinical characteristics **
-Lens dislocation, Myopia
-Aortic root dilatation, MV prolapse
-Pectus carinatum/excavatum
-Pneumothorax
-Wrist/thumb sign
-Hindfoot deformity
-Protrusio acetabuli
-Scoliosis, thoracolumbar kyphoscoliosis
-Skin striae
** 6 systemic physical signs of Marfan syndrome?**
Tall, long extremities
-Lower upper/lower body ratio
-Low fat
-Pectus carinatum/excavatum
-Scoliosis
-Pes planus (flat feet
-Long narrow face
-Retrognathia
-Striae
