DEPARTMENT OF PEDIATRICS

SACRED HEART HOSPITAL

CEBU CITY

A case of C.K., 4 y.o , male, child, Filipino, A case of C.K., 4 y.o , male, child, Filipino, Roman Catholic , from Tisa, Cebu City, Roman Catholic , from Tisa, Cebu City, admitted for the first time at SHH due to fever admitted for the first time at SHH due to fever and joint pain.and joint pain.

Prenatal HistoryPrenatal History

Prenatal check up at 4 months AOG Prenatal check up at 4 months AOG Regular , by OB Gyne , no maternal illness noted Regular , by OB Gyne , no maternal illness noted

at time of pregnancyat time of pregnancy Multivitamins and Ferrous Sulfate was taken with Multivitamins and Ferrous Sulfate was taken with

good compliancegood compliance Delivered via NSD , good cry , no complications Delivered via NSD , good cry , no complications

Nutritional HistoryNutritional History

Breastfeeding from birth until 3 monthsBreastfeeding from birth until 3 months Formula Feeding: (Nestogen) 3 months to 1y.o. Formula Feeding: (Nestogen) 3 months to 1y.o.

and 2 months (1:1 dilution) and 2 months (1:1 dilution) semi solid foods at 4 monthssemi solid foods at 4 months Solid foods at 6 monthsSolid foods at 6 months

ImmunizationsImmunizations

BCGBCG DPT/OPV 3 dosesDPT/OPV 3 doses Hep B 3 dosesHep B 3 doses MeaslesMeasles

Developmental HistoryDevelopmental History 2months: social smile2months: social smile 4 months : roll over4 months : roll over 7 months: sit with support , recognize familiar 7 months: sit with support , recognize familiar

faces faces 1 y.o.: stands alone1 y.o.: stands alone 2 y.o.: runs well 2 y.o.: runs well

Past Medical History / Family Past Medical History / Family HistoryHistory

No previous hospitalizationNo previous hospitalization No surgical procedure doneNo surgical procedure done ( - ) DM( - ) DM ( - ) HPN( - ) HPN ( - ) Cancer( - ) Cancer ( -) Asthma( -) Asthma

Personal & Social History Personal & Social History

Kindergarten-2 pupilKindergarten-2 pupil Lives with his parentsLives with his parents Playful , cooperativePlayful , cooperative No other member of the family noted to No other member of the family noted to

have the same health problemhave the same health problem

HPI:HPI:

2 weeks PTA , fever ( 38 – 39c ).2 weeks PTA , fever ( 38 – 39c ). Relieved by Paracetamol (AD:14.2 MKd). Relieved by Paracetamol (AD:14.2 MKd). Associated with pain on swallowingAssociated with pain on swallowing No consult done. Condition was tolerated. No consult done. Condition was tolerated. Fever subsided after 3 days. Fever subsided after 3 days.

1 week PTA , fever recurred, associated with 1 week PTA , fever recurred, associated with minimal swelling of the ankle then on the next minimal swelling of the ankle then on the next day on the knee. day on the knee.

Limitation of movement of the area was notedLimitation of movement of the area was noted Consult to a private physician, Ibuprofen P.O. Consult to a private physician, Ibuprofen P.O.

(AD:11.3) was given with relief. (AD:11.3) was given with relief.

Recurrence of swelling and pain of the joint Recurrence of swelling and pain of the joint prompted consult at CCMC prompted consult at CCMC

CBC showed leukocytosisCBC showed leukocytosis Urinalysis showed unremarkable resultUrinalysis showed unremarkable result ASO showed elevated resultASO showed elevated result advised admission but opted to transfer hereadvised admission but opted to transfer here

Physical ExamPhysical Exam

Conscious , coherent , cooperative , NIRDConscious , coherent , cooperative , NIRD

Vital signs:Vital signs:

BP: 90/60 HR: 98 bpm BP: 90/60 HR: 98 bpm

RR: 28 cpm Temp: 37.1 cRR: 28 cpm Temp: 37.1 c

Wt: 17.6 kgsWt: 17.6 kgs

SkinSkin : warm , good turgor , no rashes : warm , good turgor , no rashes HEENTHEENT : anicteric sclera , pinkish palpebral : anicteric sclera , pinkish palpebral

conjunctiva , no discharges, moist lips and conjunctiva , no discharges, moist lips and tongue , no tonsillopharyngeal congestion , no tongue , no tonsillopharyngeal congestion , no lymph node enlargementlymph node enlargement

Chest and LungsChest and Lungs : Equal chest expansion, no : Equal chest expansion, no retractions , clear breath sounds retractions , clear breath sounds

HeartHeart : Normal rate , regular rhythm , apex : Normal rate , regular rhythm , apex beat at 5beat at 5thth intercostal space midclavicular line , intercostal space midclavicular line , distinct heart sound, no murmurdistinct heart sound, no murmur

AbdomenAbdomen : flat , normoactive bowel sounds , : flat , normoactive bowel sounds , soft , non tender , no organomegalysoft , non tender , no organomegaly

GenitaliaGenitalia : grossly male , no lesions , no : grossly male , no lesions , no discharges discharges

RectalRectal : patent , no lesions, no mass : patent , no lesions, no mass ExtremitiesExtremities : swelling of both ankles noted, : swelling of both ankles noted,

with redness of the swollen area, with slight with redness of the swollen area, with slight limitation of movement, strong pulses, CRT < limitation of movement, strong pulses, CRT < 2 sec 2 sec

Neurologic Exam : Neurologic Exam :

Conscious , coherent , cooperative , not Conscious , coherent , cooperative , not irritableirritableMotor : 5/5 , full resistance with gravityMotor : 5/5 , full resistance with gravity

no atrophy , non spastic , non no atrophy , non spastic , non flaccid , with spontaneous flaccid , with spontaneous movement movement

Sensory : intact , ( + ) response to pain and Sensory : intact , ( + ) response to pain and touchtouch

Cranial NervesCranial Nerves

CN 1 intact CN 1 intact

CN 2 PERL CN 2 PERL

CN 3,4,6 EOM no limitationCN 3,4,6 EOM no limitation

CN 5 + corneal reflexCN 5 + corneal reflex

CN 7 no facial asymmetryCN 7 no facial asymmetry

CN 8 able to hear spoken wordsCN 8 able to hear spoken words

CN 9 , 10 uvula at midline , + gag CN 9 , 10 uvula at midline , + gag reflexreflex

CN 11 able to shrug shoulders , turns CN 11 able to shrug shoulders , turns head from side to sidehead from side to side

CN 12 tongue at midline CN 12 tongue at midline

DTR : 2 +DTR : 2 +

( - ) ( - ) BabinskiBabinski reflex reflex

( - ) nystagmus ( - ) nystagmus

no ankle clonusno ankle clonus

Meningeals : ( - ) nuchal rigidity Meningeals : ( - ) nuchal rigidity

Impression : Rheumatic Fever vs. JRAImpression : Rheumatic Fever vs. JRA

Salient FeaturesSalient Features

4 years old4 years old Fever 2 weeks PTAFever 2 weeks PTA swelling of the joints at ankle and kneeswelling of the joints at ankle and knee Redness of the swollen jointsRedness of the swollen joints slight limitation of movement of jointsslight limitation of movement of joints Leukocytosis(CBC)Leukocytosis(CBC) ASO showed elevated resultASO showed elevated result ESR was also elevatedESR was also elevated

Differential DiagnosisDifferential Diagnosis

1) Rheumatic fever1) Rheumatic fever

RULE IN: RULE IN: 1.1. Joint tendernessJoint tenderness2.2. Fever 2 weeks PTAFever 2 weeks PTA3.3. Migratory polyarthritisMigratory polyarthritis4.4. RashRash was noted near the swollen joints was noted near the swollen joints5.5. Leukocytosis(CBC)Leukocytosis(CBC)6.6. ASO showed elevated resultASO showed elevated result7.7. Elevated ESRElevated ESR

1) Rheumatic fever1) Rheumatic fever

RULE OUT: cannot be totally ruled outRULE OUT: cannot be totally ruled out

2) Post-streptococcal Reactive 2) Post-streptococcal Reactive ArthritisArthritis

RULE IN:RULE IN:

1.1. Had history of Had history of feverfever

2.2. May May follow infectionfollow infection with either group A or with either group A or group G group G StreptococcusStreptococcus

3.3. arthralgiaarthralgia and joint swelling are transient, and joint swelling are transient, usually lasting usually lasting less than 6 wkless than 6 wk..

4.4. Leukocytosis(CBC)Leukocytosis(CBC)

5.5. ASO showed elevated resultASO showed elevated result

2) Post-streptococcal Reactive 2) Post-streptococcal Reactive ArthritisArthritis

RULE OUT:RULE OUT:

1.1. Patient had migrating polyarthritis Patient had migrating polyarthritis

2.2. diagnosis of reactive postinfectious arthritis diagnosis of reactive postinfectious arthritis is usually established by is usually established by exclusion only after exclusion only after the arthritis has resolvedthe arthritis has resolved

3.3. No urethritis and conjunctivitis notedNo urethritis and conjunctivitis noted

3) JUVENILE RHEUMATOID ARTHRITIS3) JUVENILE RHEUMATOID ARTHRITIS

RULE IN:RULE IN:

1.1. Age at onset <16 yr Age at onset <16 yr (4yo)(4yo)

2.2. ArthritisArthritis in one or more joints in one or more joints

3.3. predominantly affects the joints of the lower predominantly affects the joints of the lower extremities, such as the extremities, such as the knees and ankleknees and ankle

4.4. Had history of Had history of feverfever

5.5. Elevated (ESR)Elevated (ESR)

3) JUVENILE RHEUMATOID ARTHRITIS3) JUVENILE RHEUMATOID ARTHRITIS

RULE OUT:RULE OUT:

1.1. Duration of disease 6 wk or longer Duration of disease 6 wk or longer (2 weeks (2 weeks duration only)duration only)

2.2. Arthritis of JRA is Arthritis of JRA is not migratorynot migratory

3.3. No evanescent erythematous, macular rashNo evanescent erythematous, macular rash most commonly over the trunk and proximal most commonly over the trunk and proximal extremities notedextremities noted

4) Henoch-Schönlein Purpura4) Henoch-Schönlein Purpura

RULE IN:RULE IN:

1.1. follows an follows an upper respiratory tract infectionupper respiratory tract infection

2.2. more frequent in children than adults ( more frequent in children than adults ( 2–8 yo.)2–8 yo.)

3.3. had had elevated (ASO) antibodieselevated (ASO) antibodies, - group A , - group A StreptococcusStreptococcus

4.4. Low-grade Low-grade feverfever

5.5. ArthritisArthritis localized to the localized to the knees and anklesknees and ankles and and appears to be appears to be concomitant with edemaconcomitant with edema

6.6. Elevated (ESR)Elevated (ESR)

4) Henoch-Schönlein Purpura4) Henoch-Schönlein Purpura

RULE OUT:RULE OUT:

1.1. No pinkish maculopapular rashNo pinkish maculopapular rash that initially that initially blanch on pressureblanch on pressure and progress to and progress to petechiae petechiae or palpable purpura notedor palpable purpura noted

2.2. No intermittent abdominal painNo intermittent abdominal pain that is often that is often colicky in nature notedcolicky in nature noted

3.3. Arthritis is not migratoryArthritis is not migratory

Course in the WardCourse in the Ward On AdmissionOn Admission

IVF at Maintenance rateIVF at Maintenance rate Ibuprofen q 6 hours ( AD 9 MKd )Ibuprofen q 6 hours ( AD 9 MKd )LabsLabs

CBC : Wbc 10CBC : Wbc 10 Neu 61.4 %Neu 61.4 %

Lym 28.9 %Lym 28.9 % Hgb 9.85 (11.5 – 15.5)Hgb 9.85 (11.5 – 15.5)

Hct 29.1 (35 – 45%)Hct 29.1 (35 – 45%) Plt 327Plt 327

Urinalysis : Urinalysis :

Sp gr: 1.005Sp gr: 1.005

Ph 6.0Ph 6.0

WBC 0-1WBC 0-1

RBC 0-1RBC 0-1

Epithelial cells – rareEpithelial cells – rare

Bacteria – fewBacteria – few

Mucus threads – few Mucus threads – few

Amorphous urates - fewAmorphous urates - few

ESR : Result ESR : Result 125 mm125 mm

reference range : 0 -15reference range : 0 -15

ASO : Result ASO : Result 400 IU/ml400 IU/ml

reference range :< 200reference range :< 200

CXR: later revealed CXR: later revealed Right Basal Right Basal Pneumonitis Pneumonitis

11StSt Hospital Day Hospital Day

BP 90/60 HR 94 RR 28 BP 90/60 HR 94 RR 28 Temp 37.8 – 38cTemp 37.8 – 38c Referred to Pedia Cardiologist:Referred to Pedia Cardiologist: Impression : Post – streptococcal reactive Impression : Post – streptococcal reactive

arthritisarthritis Suggested: anti inflammatory dose of aspirinSuggested: anti inflammatory dose of aspirin Aspirin 80 mg q 8hrs ( AD 54.5 mkd )Aspirin 80 mg q 8hrs ( AD 54.5 mkd )

22NdNd Hospital Day Hospital Day

BP: 90/60 HR: 96 BP: 90/60 HR: 96

RR: 26 RR: 26 Temp: 38 - 39cTemp: 38 - 39c

- no swelling of joints (ankle) noted- no swelling of joints (ankle) noted

- Phenoxymethylpenicillin ( AD 50 mkd ) - Phenoxymethylpenicillin ( AD 50 mkd )

- Referred to Rheumatologist - Referred to Rheumatologist

33RDRD Hospital Day Hospital Day

BP 90/60 HR 96 BP 90/60 HR 96

RR 26 RR 26 temp 36.5 - 38ctemp 36.5 - 38c Pedia Cardio out of servicePedia Cardio out of service

44THTH Hospital Day Hospital Day

BP 90/ 60 HR 98 RR 28BP 90/ 60 HR 98 RR 28 Temp:36.4 – Temp:36.4 – 38.138.1

Seen by an Adult Rheumatologist:Seen by an Adult Rheumatologist: Impression : Post – streptococcal arthritisImpression : Post – streptococcal arthritis Suggested: continue phenoxymethylpenicillinSuggested: continue phenoxymethylpenicillin Aspirin at 50 – 100 mkd for 2 weeksAspirin at 50 – 100 mkd for 2 weeks Repeat CBC , ESR and ASO after 2 weeks Repeat CBC , ESR and ASO after 2 weeks

55THTH Hospital Day Hospital Day

Discharged ImprovedDischarged Improved THM : PhenoxymethylpenicillinTHM : Phenoxymethylpenicillin

Aspirin ( 54.5 mkd ) for 13 days Aspirin ( 54.5 mkd ) for 13 days moremore

Follow up at AP’s clinic after 2 weeks (CBC, Follow up at AP’s clinic after 2 weeks (CBC, ESR, ASOT )ESR, ASOT )

Final Diagnosis:Final Diagnosis:

Rheumatic Fever:Rheumatic Fever: Is a multisystem inflammatory disease that occurs as a

delayed sequel to a group A streptococcal infection :• Throat• Skin

• Results in inflammation of:• Heart - Carditis• Skin - Erythema marginatum• Brain - Chorea• Joints - Polyarthritis / arthralgia

most often occurs in children between 5 and most often occurs in children between 5 and 15 years.15 years.

Epidemiologic risk factors include:Epidemiologic risk factors include:

1.1. Lower standards of living (crowding)Lower standards of living (crowding)

2.2. More common among socially and More common among socially and economically disadvantaged populations.economically disadvantaged populations.

Pathogenesis:Pathogenesis:

1.1. cytotoxicity theorycytotoxicity theory

GAS produces several enzymes that are GAS produces several enzymes that are cytotoxic for mammalian cardiac cells. For cytotoxic for mammalian cardiac cells. For example, streptolysin O has a direct example, streptolysin O has a direct cytotoxic effect on mammalian cells in tissue cytotoxic effect on mammalian cells in tissue culture.culture.

2.2. immunologic theoryimmunologic theory

clinical similarity of acute rheumatic fever to clinical similarity of acute rheumatic fever to other illnesses produced by other illnesses produced by immunopathogenic processes and by the immunopathogenic processes and by the latent period between the group A latent period between the group A streptococcal infection and the acute streptococcal infection and the acute rheumatic fever. rheumatic fever.

DiagnosisDiagnosis

Revised Jones Criteria for Acute Rheumatic Revised Jones Criteria for Acute Rheumatic Fever:Fever:

Major Criteria:Major Criteria: PolyarthritisPolyarthritis

Minor Criteria:Minor Criteria: ArthralgiaArthralgia FeverFever Leukocytosis/ elevated ESRLeukocytosis/ elevated ESR

TreatmentTreatment

two necessary therapeutic approaches to two necessary therapeutic approaches to patients with acute rheumatic fever: patients with acute rheumatic fever:

1.1. anti-streptococcal antibiotic therapyanti-streptococcal antibiotic therapy

2.2. therapy for the clinical manifestations of the therapy for the clinical manifestations of the diseasedisease

BedrestBedrest Close monitoring for evidence of carditisClose monitoring for evidence of carditis Antibiotic Therapy:Antibiotic Therapy: 10 days Oral Penicillin/ Erythromycin10 days Oral Penicillin/ Erythromycin Single Dose IM Benzathine PenicillinSingle Dose IM Benzathine Penicillin

Anti-inflammatory TherapyAnti-inflammatory Therapy

Migratory polyarthritis + carditis ( w/o Migratory polyarthritis + carditis ( w/o cardiomegaly or CHF):cardiomegaly or CHF):

- Aspirin (100 mg/kg/day) QID PO for 3-5 days, - Aspirin (100 mg/kg/day) QID PO for 3-5 days, followed by 75mg/kg/day QID PO for 4 followed by 75mg/kg/day QID PO for 4 weeksweeks

Carditis + cardiomegaly/ CHF:Carditis + cardiomegaly/ CHF:- Prednisone 2 mg/kg/day QID for 2-3 weeks Prednisone 2 mg/kg/day QID for 2-3 weeks

followed by a tapering of the dose that reduces followed by a tapering of the dose that reduces the dose by 5 mg/24 hrs every 2-3 daysthe dose by 5 mg/24 hrs every 2-3 days

- At the beginning of tapering of prednisone At the beginning of tapering of prednisone dose, Aspirin should be started at 75mg/kg/day dose, Aspirin should be started at 75mg/kg/day QID for 6 weeks QID for 6 weeks

Sydenham’s Chorea – not seen in our patient Sydenham’s Chorea – not seen in our patient

- - Phenobarbital Phenobarbital 16-32 mg/kg, q6 – 8 hrs PO16-32 mg/kg, q6 – 8 hrs PO

- - HaloperidolHaloperidol > if Phenobarbital is not > if Phenobarbital is not effectiveeffective

> 0.01 - 0.03 mg/kg/24hrs > 0.01 - 0.03 mg/kg/24hrs BID POBID PO

- - ChlorpromazineChlorpromazine > 0.5 mg/kg q 4 -6 hrs PO > 0.5 mg/kg q 4 -6 hrs PO

Complications:Complications:

Arthritis and choreaArthritis and chorea of resolve completely of resolve completely without sequelae. without sequelae.

Long-term sequelae are usually limited to the Long-term sequelae are usually limited to the heartheart

Infective EndocarditisInfective Endocarditis: those w/ cardiac : those w/ cardiac valvular disease due to RF are at increased risk valvular disease due to RF are at increased risk during episodes of transient bacteremia.during episodes of transient bacteremia.

PreventionPrevention

PRIMARY PREVENTION.PRIMARY PREVENTION.Antibiotic therapy instituted before the 9th day Antibiotic therapy instituted before the 9th day of symptoms of acute group A streptococcal of symptoms of acute group A streptococcal pharyngitis - highly effective in preventing pharyngitis - highly effective in preventing first attacks of acute rheumatic fever from that first attacks of acute rheumatic fever from that episode. episode.

Secondary preventionSecondary prevention

To prevent acute GAS pharyngitis in patients at To prevent acute GAS pharyngitis in patients at substantial risk of recurrent acute rheumatic feversubstantial risk of recurrent acute rheumatic fever

No carditis in initial episode of acute rheumatic fever No carditis in initial episode of acute rheumatic fever have low risk of carditis with recurrences. have low risk of carditis with recurrences.

Antibiotic prophylaxis may be discontinued when Antibiotic prophylaxis may be discontinued when they reach their early 20s and after at least 5 yr have they reach their early 20s and after at least 5 yr have elapsed since their last episode of acute rheumatic elapsed since their last episode of acute rheumatic feverfever

Secondary PreventionSecondary Prevention

Regimen of choice:Regimen of choice: Single IM of Single IM of Benzathine penicillin GBenzathine penicillin G (1.2 (1.2

million IU) every 4 wkmillion IU) every 4 wk Compliant Pt: Compliant Pt: Penicillin V BIDPenicillin V BID POPO and and

Sulfadiazine OD POSulfadiazine OD PO Pt allergic to penicillin and sulfonamides: Pt allergic to penicillin and sulfonamides:

erythromycinerythromycin BID may be used BID may be used