ILAs Flashcards
Management of juvenile infant arthritis?
-NSAIDs, such as ibuprofen
-Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
-DMARDs- methotrexate, sulfasalazine and leflunomide
-Biologics -TNF inhibitors etanercept, infliximab and adalimumab
What is still disease
-a subtype of Juvenile idiopathic arthritis
-which is systemic and can affect joints and organs
Signs of stills disease (systemic arthritis) (8)
-Subtle salmon-pink rash
-High swinging fevers
-Enlarged lymph nodes
-Weight loss
-Joint inflammation and pain
-Splenomegaly
-Muscle pain
-Pleuritis and pericarditis
Complication of Systemic JIA?
-macrophage activation syndrome
What is macrophage activation syndrome?
-severe activation of the immune system and inflammatory response
Investigation that will suggest Macrophage activation syndrome?
Low ESR
Key NON INFECTIVE differentials of a high grade fever for 5+ days? (4)
-Kawasaki disease
-stills’s disease
-rheumatic fever
-leukemia
5 Subtypes of JIA?
-Systemic JIA
-Polyarticular JIA
-Oligoarticular JIA
-Enthesitis related arthritis
-Juvenile psoriatic arthritis
Macrophage activation syndrome presentation
-stills disease presentation
PLUS
-aneamia
-DIC
-thrombocytopenia
-non blanching rash
What is polyarticular arthritis?
-Idiopathic inflammatory arthritis in 5 joints or more
-tends tot be symmetrical
What is oligoarticular JIA?
- idiopathic inflammatory arthritis, involving 4 joints or less
-tends to affect larger joints like hips and knees
What presentation is commonly associated with Oligoarticular JIA?
Anterior uveitis
Which group is e the sites related arthritis more common in
Males over 6 years
- equivalent to seronegative spondyloarthropathies such as Ankylosing spondylitis, psoriatic arthritis and reactive arthritis
What is an enthesis?
Point at which the tendon of a muscle inserts into a bone
What is Enthesitis-Related Arthritis?
Inflammation of the insertion point of a tendon to bone
causes of enthesitis?
-traumatic stress e.g during repetitive sport
-autoimmune inflammation
What is commonly associated with Enthesitis related arthritis?
Anterior uveitis
Common sites of inflammation in enthesitis related arthritis ?
-Interphalangeal joints in the hand
-Wrist
-Over the greater trochanter
-Quadriceps insertion at the ASIS
-Quadriceps and patella tendon insertion
-Base of achilles, at the calcaneus
-Metatarsal heads on the base of the foot
Signs of juvenile psoriatic arthritis?
-Plaques of psoriasis
-Pitting of the nails (nail pitting)
-Onycholysis, separation of the nail from the nail bed
-Dactylitis, inflammation of the full finger
-Enthesitis
What is Patent ductus arteriosus?
-when the ductus arteriosus fails to close completely within the first 2-3 weeks of life
Risk factors for PDA
-PREMATURITY
-rubella infection during pregnancy to the mother
-FHx
Presentation of PDA (5)
-SOB
-Difficulty feeding
-Poor weight gain
-Lower respiratory tract infections
-tachypnoea
Pathophysiology of PDA?
-pressure in AORTA is greater than that in pulmonary vessels
-causes L to R shunt
-increases pulmonary vessel pressure (pulmonary HTN)
-leads to RHS overload and hypertrophy
-Can then lead to LVH
What heart sound in PDA?
Continuous Crescendo-descrecendo murmur
- can obscure the second heart sound
How to diagnose PDA
-ECHO
- can be used in conjunction with Doppler to assess L to R shunt
Managment of PDA?
-monitor until 1 year old using ECHO
-NSAIDs may be used to encourage the closure of PDA
-trans catheterisation
-surgically close
What disease do the newborn blood spot test test for?
-congenital hypothyroidism
-sickle cell
-cystic fibrosis
-phenylketonuria
-beta thalassaemia
-MCAD deficiency
What is involved in the septic screen
- blood cultures
- bloods - FBC, U+E, glucose
- lactate
- LP considered
- CXR
- urine sample
