Paediatric Case Paediatric Case PresentationPresentation

Natasha Quader Natasha Quader ST1ST1

September 2008September 2008

Patient CDPatient CD

8 year old girl8 year old girl Normally fit and wellNormally fit and well

PC – PyrexiaPC – Pyrexia

- Lethargy- Lethargy

- Groin and hip pain- Groin and hip pain

History of Presenting History of Presenting ComplaintComplaint

7/52 ago had pyrexia for 1 week followed by a cough → viral 7/52 ago had pyrexia for 1 week followed by a cough → viral URTI URTI

Intermittent pyrexia - mainly nocturnal spikesIntermittent pyrexia - mainly nocturnal spikes

C/o hip and groin pain. C/o hip and groin pain. - Present on waking up in the morning- Present on waking up in the morning - Pain waking her up during sleeping- Pain waking her up during sleeping - Developed difficulty in walking, pain on weight bearing- Developed difficulty in walking, pain on weight bearing

Increasingly lethargic – sleeping during the day and missing Increasingly lethargic – sleeping during the day and missing school school

Generally unwell, Generally unwell, ↓ appetite, ↓ appetite, irritable and cryingirritable and crying

PMHPMH

PMH PMH - Laryngomalacia during infancy- Laryngomalacia during infancy - RTA in Feb ’07 – back injury resolved- RTA in Feb ’07 – back injury resolved

SH – no recent travel abroadSH – no recent travel abroad

Immunisations up-to-dateImmunisations up-to-date

DxH – nil, NKDADxH – nil, NKDA

FH – Maternal grandmother with RAFH – Maternal grandmother with RA

Examination 1Examination 1 Alert, communicatingAlert, communicating PR – 80 regularPR – 80 regular Temp – Temp – 38 38 °C°C RR-18RR-18

Bilateral cervical Bilateral cervical lymphadenopathylymphadenopathy

Throat – NADThroat – NAD Ears – wax bilaterallyEars – wax bilaterally No rashesNo rashes

HS I + II + OHS I + II + O Chest: ClearChest: Clear

AbdomenAbdomen

Inguinal lymphadenopathy

Lower abdominal tenderness

Examination 2Examination 2CNSCNS CN – NAD. PEARLCN – NAD. PEARL Lower limbs bilaterallyLower limbs bilaterally - tone normal- tone normal - power 4/5 - power 4/5 - reflexes symmetrical- reflexes symmetrical Co-ordination – intactCo-ordination – intact Romberg’s negativeRomberg’s negative Antalgic gaitAntalgic gait Slight waddling and holding R. Slight waddling and holding R.

inguinal regioninguinal region

MSKMSK No hip/knee joint No hip/knee joint

swellingswelling Tenderness over AIS Tenderness over AIS

and crest bilaterally and crest bilaterally Hips - Hips - ↓ active ROM↓ active ROM

Positive Examination Positive Examination FindingsFindings

PyrexicPyrexic

Lower abdominal tendernessLower abdominal tenderness

Bilateral inguinal lymphadenopathyBilateral inguinal lymphadenopathy

Bilateral hip tenderness and restricted Bilateral hip tenderness and restricted ROMROM

Antalgic gaitAntalgic gait

Differential DiagnosisDifferential Diagnosis

Septic hipSeptic hip Infection – viral, bacterial, Lyme diseaseInfection – viral, bacterial, Lyme disease Juvenile arthritisJuvenile arthritis Malignancy – leukaemia, neuroblastomaMalignancy – leukaemia, neuroblastoma PerthesPerthes Transient irritable hipTransient irritable hip

Infection ScreenInfection Screen MSU – NADMSU – NAD Blood cultures – no growthBlood cultures – no growth

EBV Igm – negativeEBV Igm – negative Toxopl Igm – negativeToxopl Igm – negative ASOT – normalASOT – normal Rubella Igm – negativeRubella Igm – negative Parovirus Igm – negativeParovirus Igm – negative

Bilateral Hip X-rayBilateral Hip X-ray

ImagingImaging

USS abdo and hips – no fluid in hip jointsUSS abdo and hips – no fluid in hip joints

MRI abdo/pelvis - No evidence of avascular MRI abdo/pelvis - No evidence of avascular necrosisnecrosis

Blood Test ResultsBlood Test ResultsInitialInitial

GPGP7 weeks7 weeks

laterlater

8 weeks8 weeks

In -patientIn -patient9 weeks9 weeks

In-In-patientpatient

10 weeks10 weeks

OPAOPA14 weeks14 weeks

OPAOPA

HbHb 9.99.9 9.79.7 9.79.7 10.210.2 10.210.2 11.111.1WCCWCC 6.36.3 5.55.5 4.24.2 6.16.1 6.16.1 4.94.9PltPlt 418418 354354 338338 516516 516516 295295NeutNeut 2.202.20LympLymp 2.702.70

CRPCRP 99 1313 <1<1 <1<1ESRESR 9898 9090 105105 104104 104104 1414

Follow UpFollow Up

Follow up 10/7 after discharged from HospitalFollow up 10/7 after discharged from Hospital

Symptoms much improvedSymptoms much improved

- no complaints of hip pain on weight bearing- no complaints of hip pain on weight bearing

- no pain during sleep- no pain during sleep

- no pyrexia for 5/7- no pyrexia for 5/7

- analgesia reduced from QDS to ON- analgesia reduced from QDS to ON

- generally more mobile, active and cheerful- generally more mobile, active and cheerful

Follow up ResultsFollow up Results On examinationOn examination - discomfort on extreme range of full - discomfort on extreme range of full

abduction of R. hipabduction of R. hip - Tearful on jumping on 2 feet- Tearful on jumping on 2 feet

Repeat bloods: ESR remains high – 104Repeat bloods: ESR remains high – 104 Anti nuclear antibody positiveAnti nuclear antibody positive

Referral → RheumatologistReferral → Rheumatologist → → OphthalmologistOphthalmologist

Juvenile ArthritisJuvenile Arthritis

Is a group of conditions Is a group of conditions

in which there is chronic arthritis in which there is chronic arthritis

lasting lasting more than 6 weeksmore than 6 weeks, ,

presenting presenting

before 16 years of agebefore 16 years of age

ClassificationsClassifications

Systemic illness (Still’s disease) - 20%Systemic illness (Still’s disease) - 20%

Rh factor negative polyarticular - 25%Rh factor negative polyarticular - 25%

Rh factor positive polyarticular - 5%Rh factor positive polyarticular - 5%

Pauciarticular arthritis assoc with ANAPauciarticular arthritis assoc with ANA & chronic uveitis - 30-35%& chronic uveitis - 30-35%

Pauciarticular arthritis assoc with Pauciarticular arthritis assoc with spondylitis and HLA B27 - 10-15%spondylitis and HLA B27 - 10-15%

AetiologyAetiology

Autoimmune disease in which the cause of Autoimmune disease in which the cause of arthritis is largely unknownarthritis is largely unknown

A possible viral aetiology has been considered for A possible viral aetiology has been considered for a variety of arthritis conditions and clustering of a variety of arthritis conditions and clustering of patient’s following viral epidemics patient’s following viral epidemics

Viral infections that have noted include mumps, Viral infections that have noted include mumps, rubella and Parvovirus B19rubella and Parvovirus B19

Pauciarticular ArthritisPauciarticular Arthritis Female > MaleFemale > Male Involvement of up to 4 or fewer joints – typically larger joints Involvement of up to 4 or fewer joints – typically larger joints

such as the kneesuch as the knee

3 subtypes:3 subtypes: 1) The early onset form (< 5 years of age), involves most 1) The early onset form (< 5 years of age), involves most

frequently knee joints frequently knee joints

2) The late onset form ( > 9 years of age) most frequently 2) The late onset form ( > 9 years of age) most frequently involves hips with/without sacroilitisinvolves hips with/without sacroilitis

3) Develops at any age with an asymmetrical 3) Develops at any age with an asymmetrical

oligoarthritis, dactylitis and psoriatic manifestationoligoarthritis, dactylitis and psoriatic manifestation

Is a large association with Anti Nuclear Is a large association with Anti Nuclear Antibodies between 40-75%Antibodies between 40-75%

Frequently associated with chronic anterior Frequently associated with chronic anterior uveitis (can be uveitis (can be asymptomaticasymptomatic or asso pain, or asso pain, light sensitivity and redness)light sensitivity and redness)

Regular opthalmological screening is Regular opthalmological screening is indicatedindicated

Pauciarticular Arthritis 2Pauciarticular Arthritis 2

ComplicationsComplications Chronic anterior uveitisChronic anterior uveitis – if poorly controlled may – if poorly controlled may

result in permanent eye damage, including blindnessresult in permanent eye damage, including blindness

Flexion contactures of the jointFlexion contactures of the joint – joint held in the – joint held in the most comfortable position, thereby minimising intra-most comfortable position, thereby minimising intra-articular pressure. Chronic disease can lead to joint articular pressure. Chronic disease can lead to joint destruction and need for joint replacementdestruction and need for joint replacement

Growth failureGrowth failure – anorexia, chronic disease and – anorexia, chronic disease and steroid therapysteroid therapy

AmyloidosisAmyloidosis – rare complication causing proteinuria – rare complication causing proteinuria and renal failureand renal failure

Management 1Management 1 Multidisciplinary approach is required for Multidisciplinary approach is required for

optimal Rx: optimal Rx: - relieve pain- relieve pain - preserve joint function- preserve joint function - maintain normal growth and- maintain normal growth and psycho – social developmentpsycho – social development

Physiotherapist & Occupational TherapistsPhysiotherapist & Occupational Therapists - strengthen muscles & keep joints flexible - strengthen muscles & keep joints flexible - encourage normal limb development- encourage normal limb development - maintain function and prevent deformities- maintain function and prevent deformities - develop exercise programs- develop exercise programs

Management 2Management 2

Paediatricians, Rheumatologist and NursesPaediatricians, Rheumatologist and Nurses

- educating child and family- educating child and family

- medical management- medical management

CounsellorCounsellor

– – for the child and their family to reduce for the child and their family to reduce anxiety and share management of diseaseanxiety and share management of disease

Medical ManagementMedical Management

NSAIDS – aspirin, ibuprofen, diclofenacNSAIDS – aspirin, ibuprofen, diclofenac

DMARDS (Disease modifying anti rheumatic DMARDS (Disease modifying anti rheumatic drugs)drugs)

- under rheumatologist’s supervision- under rheumatologist’s supervision - hydroxychloroquine, penicillamine, methotrexate- hydroxychloroquine, penicillamine, methotrexate

Intra-articular corticosteroid injectionsIntra-articular corticosteroid injections

Corticosteroids Corticosteroids – – severe systemic involvement &/or for eyes Rxsevere systemic involvement &/or for eyes Rx TNF alpha blockers TNF alpha blockers – – block the immune protein TNF (inflammatory agent in block the immune protein TNF (inflammatory agent in

arthritis)arthritis)

PrognosisPrognosis

Symptoms usually go away after a few Symptoms usually go away after a few yearsyears

There are usually no further recurrencesThere are usually no further recurrences

Some children may have longer lasting Some children may have longer lasting involvementinvolvement

ReferencesReferences Laura Quarte et al. Juvenile idiopathic arthritis: An update Laura Quarte et al. Juvenile idiopathic arthritis: An update

on clinical and therapeutic approaches. Ann Ital Med Int on clinical and therapeutic approaches. Ann Ital Med Int 2005; 20: 211-2172005; 20: 211-217

Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis http://www.emedicine.com/ped/topic1749.htmhttp://www.emedicine.com/ped/topic1749.htm

Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis http://www.emedicinehealth.com/juvenile_rheumatoihttp://www.emedicinehealth.com/juvenile_rheumatoid_arthritis/article_em.htmd_arthritis/article_em.htm

Arthritis in childrenArthritis in children

http://www.medicinenet.com/juvenile_arthritis/article.htmhttp://www.medicinenet.com/juvenile_arthritis/article.htm