School of Medicine Bachelor of Medicine; Bachelor of Surgery
Paediatrics 2 Case Write-Up Name: Charlotte Ee Sze Lyn Lyn Student ID: 0313464 Date of Postin: 14!11!16"#3!11!16 14!11!16"#3!11!16 Case $rite"u% num&er: 1 Item History including patient data Physical Examination Diagnosis/Diferential Diagnosis Investigation anagement Discussion including evidence!"ased medicine #E$% and re&erences Total
Name of Lecturer:
Date:
History Taking denti!cation "ata Name
: SNA
R/N
: SB00047407
Age
: 12 years and 11 months old
Gender
: Female
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Marks Allocated 20 20 15 10 15
Marks Awarded Awarded
20
100 Signature:
Ethnicity
: alay
!iti"enshi#
: alaysian
Address
: $ay% &aras
'ate o( admission
: 24th No)em*er 201+
'ate o( cler,ing
: 2-th No)em*er 201+
So%r So%rce ce o( hist history ory
: $at $atie ient nt.. #at #atien ient tss (iles (iles and and #atie #atient nts moth mother er
Chief Co#plaint he #atient SNA is a 1 year old alay girl ho is a ,non case o( steroid de#endent ne#hrotic syndrome ith (re3%ent rela#ses She as re(erred to 5os#ital S%ngai B%loh and #resents ith a chie( com#laint #erior*ital selling (or 2 days (olloed *y general *ody selling
History of Presenting llness he #atient as #re)io%sly ell %ntil years ago in 6cto*er 201 hen she de)elo#ed grad%al grad%al onset o( generali"ed selling selling (or 2 days he selling started at the #erior*ital #erior*ital area then to (acial #%((iness and #rogressed to the a*domen then to the lim*s $rior to de)elo#ing these sym#toms. she had #receding diarrhoea. (e)er and co%gh hich lasted (or a ee, #rior to admission he (e)er as initially meas%red *y the mothers cr%de to%ch and she also noticed SNA had a decrease in acti)ity SNAs mother cannot remem*er the doc%mented tem#erat%re at the time *%t (eels the (e)er as lo grade he (e)er as contin%o%s in nat%re here as no di%rnal )ariation o( the tem#erat%re and there ere no chills or rigors here is no ecessi)e seating or night seating '%ring this #eriod o( illness. the #atient a##eared to *e less acti)e *%t is a*le to do daily dai ly acti)ities here as a s no loss o( a##etite and no eight loss he #atients co%gh as non8#rod%cti)e ith no di%rnal )ariation he co%gh had intermittent short *o%ts o( co%ghing itho%t any o*)io%s triggers here as no s#ecial characteristic o( the co%gh and the mother cannot remem*er i( there as di%rnal )ariation SNA also s%((ered (rom diarrhoea in hich she had loose stool (or increased (re3%ency o( de(ecation 5oe)er. the #atient and her mother cannot remem*er m%ch else a*o%t the diarrhoea here is no recollection o( the stool colo%r. consistency or amo%nt *%t it as not *lood or m%c%s stained A(ter de)elo#ing generali"ed selling. SNAs mother r%shed her to 5os#ital S%ngai B%loh and a(ter the doctors assessment as diagnosed ith ne#hrotic syndrome and conse3%ently started on oral steroids to hich she as res#onsi)e to She achie)ed remission (or 2 years 5oe)er 2 years later. in Fe*r%ary 201-. my #atient had her (irst rela#se in hich (olloing an %##er %##er res#irat res#iratory ory tract tract in(ecti in(ection. on. she de)elo de)elo#ed #ed general generali"ed i"ed sellin selling g She achie) achie)ed ed remission a(ter *eing reind%ced ith oral #rednisolone +0mg/m2/day and com#leted her maintenance oral #rednisolone 940mg/m2/day 5oe)er as they ere ta#ering don the
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dose she had another rela#se 5er mother co%ld not remem*er the eact dosage at hich she de)elo#ed the rela#se 5er second rela#se as in ay 201-. this time she had a rela#se (olloing an e#isode o( ac%te gastroenteritis he rela#se as (o%nd *y *rea,thro%gh #rotein%ria on home di#stic, monitoring here it as more than 2; (or consec%ti)e days She had also de)elo#ed (acial #%((iness She as admitted (or 10 days and as gi)en oral anti*iotics. a di%retic and #rednisolone She achie)ed remission a(ter *eing reind%ced ith oral #rednisolone +0mg/m2/day and com#leted her maintenance oral #rednisolone 940mg/m2/day 6nce again as they ere ta#ering don the dose she had another rela#se 5er mother co%ld not remem*er the eact dosage at hich she de)elo#ed the rela#se 5er third and (o%rth rela#ses ere in A%g%st and 6cto*er 201+ res#ecti)ely hese e#isodes also occ%rred a(ter a #receding %##er res#iratory tract in(ection and she res#onded steroid treatment as she did ith the #re)io%s 2 e#isodes A(ter her third rela#se. she achie)ed remission in only days 5oe)er she s%*se3%ently de(a%lted (ollo %# and her maintenance dose o( steroids A(ter her (o%rth rela#se. the mother did not ta#er don the #ills and contin%ed gi)ing high dose #rednisolone itho%t ta#ering don %ntil the #ills ran o%t *eca%se she had r%n o%t o( %rine di#stic,s and co%ld not a((ord more at the time d%e to (inancial constraints 5oe)er my. #atient did not ha)e any loer *ac, #ain. a*domen #ain. se)ere )omiting and diarrhea. loss o( conscio%sness or other sym#toms s%ggesti)e o( an Addisonian crisis SNAs (i(th rela#se as in arch 201+ At the time. she once again #resented ith generali"ed edema associated ith (e)er and diarrhoea (or days 5er %rine di#stic, readings had *een 4; (or days She had had sic, contact ith her si*ling #rior to de)elo#ing the sym#toms She achie)ed remission a(ter *eing reind%ced ith oral #rednisolone +0mg/m2/day and com#leted her maintenance oral #rednisolone 940mg/m2/day *e(ore grad%ally ta#ering don the medication he sith rela#se as in ay 201+. the se)enth in A%g%st 201+. the eighth in Se#tem*er 201+ and the ninth in 6cto*er 201+ hey ere all similar to the #re)io%s e#isodes ith each e#isode *eing #receded *y either an %##er res#iratory tract in(ection or ac%te gastroenteritis and characteri"ed *y either *y *rea,thro%gh #rotein%ria hich *ecomes #ersistent #rotein%ria or generali"ed selling hich started aro%nd the eyes and (acial #%((iness She achie)ed remission a(ter *eing reind%ced ith oral #rednisolone +0mg/m2/day *%t o%ld rela#se either hile on her maintenance oral #rednisolone 940mg/m2/day or hile ta#ering don (%rther his c%rrent admission is my #atients 10 th rela#se
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hich as her %s%al he stool as neither *lood nor m%c%s stained 2 days *e(ore admission. she also de)elo#ed s%dden #erior*ital selling hich she noticed on a,ing %# hich #rogressed to (acial #%((iness. then. a*dominal and la*ial selling. then selling o( the lim*s At the time. my #atient as on a dose o( -mg oral #rednisolone E6' =#on de)elo#ing the selling. my #atient did a %rine di#stic, test hich re)ealed 4; #rotein%ria She as then *ro%ght to the emergency de#artment hro%gho%t these years there has *een no re#ort o( (%llness and ro%nding o( the (ace 9so8 called >moon (acies>. added (at on *ac, o( nec, 9so8called >*%((alo h%m#>. no easy *r%ising o( the s,in. no stretch mar,s 9striae. no ecessi)e eight gain. most mar,ed in the a*dominal region. no ecess hair groth. no m%scle ea,ness. no hy#ertension or hy#erglycaemia or other sym#toms s%ggesting corticosteroid toicity here as also ne)er any tea colo%red %rine tho%gh at times her %rine did a##ear clo%dy y #atient also denies ha)ing a (acial rash in the distri*%tion o( her chee,s and nasal *ridge es#ecially a(ter e#os%re to s%nlight. ?oint #ain. *alding. oral %lcers or ?oint selling
Syste#atic $n%uiry Systemic re)ie as not signi(icant 6ther than the sym#toms mentioned a*o)e. the (olloing negati)es ere elicited: !entral ner)o%s system Res#iratory system
A*sence o( ea,ness. (aint. *eha)io%ral changes and sei"%res A*sence o( chest #ain. shortness o( *reath. tachy#nea or noticea*le chest recession
!ardio)asc%lar system
A*sence o( #al#itations. synco#e and e)ident oedema
Gastrointestinal system Genito%rinary system
No consti#ation A*sence o( change in (re3%ency o( mict%rition. hesitancy.
%sc%los,eletal system
haemat%ria. and dys%ria A*sence o( ?oint #ain. mo)ement limitations and *r%ising
Past Medical History here is no #ast medical history other than that mentioned as #art o( the history o( #resenting illness 9,indly re(er a*o)e
Birth History he mother had o*tained reg%lar antenatal care ith *oo,ing done at aro%nd 1+ ee,s #eriod o( gestation and (re3%ent %ltraso%nd scans done ith no signi(icant (indings She too, s%##lements reg%larly She recei)ed imm%nisation against all signi(icant in(ections Screening as also done on the #regnant mother *%t the res%lt is insigni(icant '%ring #regnancy. the mother did not s%((er (rom any maternal illness s%ch as chic,en#o. r%*ella and too#lasmosis he #regnant mother as not e#osed to dr%gs or alcohol *%t as
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e#osed to #assi)e smo,ing he mother did not s%((er (rom any #regnancy com#lication s%ch as #regnancy ind%ced hy#ertension or gestational dia*etes he mothers age d%ring the deli)ery o( NSB is 1@ years old he #atient as *orn *y at term ith a *irth eight o( 2,g he deli)ery method is s#ontaneo%s )aginal deli)ery ith no inter)ention 9(orce#s or )ento%se done he *a*y cried immediately a(ter deli)ery here as no need (or N
##unisation History he imm%nisation is %# to age ith the last )accination *eing at the time here the #atient as 1 years old =n(ort%nately. the mother is %na*le to recall the name o( the )accine gi)en *%t according to that alaysian #aediatric #rotocol it as #ro*a*ly the 5$ )accine he #atient has not ta,en any additional )accines s%ch as the #ne%mococcal or chic,en #o )accine
&eeding History he #atient as ecl%si)ely *reast(ed (or a*o%t + months Form%lated mil, as ne)er %sed eaning as started hen the #atient as + months old B%t s%##lementary *reast(eeding as contin%ed %ntil the #atient as + years old he #atient no cons%mes an ad%lt diet o( solid (ood here as no (eeding di((ic%lty *y the mother A ty#ical diet consists o( rice. meat and )egeta*les he #atient is not a #ic,y eater
"e'elop#ental history he de)elo#ment o( the #atient is a##ro#riate to her age he mother re#orts that the #atient #er(orms ell at school and gets along ell ith her si*lings and #eers here is no re#ort o( #eer #ress%re or si*ling ri)alry y #atient has *een in the (irst class o( her school since Standard and #er(ormed reasona*ly ell in her =$SR9As2Bs des#ite (re3%ent a*sences (rom school9d%e to her (re3%ent admissions Gross motor: he #atient ass a*le al, itho%t s%##ort *y the age o( 10 months Vision and fine motor: he #atient as a*le to hold a #encil and and scri**le *y the age o( 1@ months Hearing, speech and language: the #atient co%ld s#ea, 84 meaning(%l ords *y the age o( C810 months Social and emotional: She co%ld dress and (eed hersel( *y the time she as 2 years old She as toilet8trained *y the age o( 1@ months She has ne)er had any di((ic%lty (orming (riendshi#s ith other children
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"rug History and (llergic History he #atient is on (re3%ent i( not constant steroid %se 5oe)er. there ha)e *een no signs o( steroid toicity She is c%rrently on high dose oral steroids9+0mg/m2/day and a #ro#hylactic dose o( oral #enicillin She does not ta,e any other dr%gs. s%##lements or traditional medicine here is no history o( dr%g or (ood allergy
&a#ily History he #atient is the oldest child among 4 si*lings o( a non8consang%ineo%s marriage he #atient has 2 yo%nger sisters o( ages 10 and - res#ecti)ely and a yo%nger *rother ho is @ years old 5er *rother as *orn ith cle(t li# *%t a(ter s%rgery has no made a (%ll reco)ery 5er si*lings are all c%rrently are healthy y #atients (ather as a hea)y smo,er and had %nderlying dia*etes and hy#ertension 5e #assed aay recently at the age o( 2 d%e to a myocardial in(raction 5e as a manager at Sya*as Sdn Bhd *e(ore his %ntimely demise !%rrently. my #atients mother ho is 2 years old ith no %nderlying diseases is the sole *readinner o( the (amily SNAs maternal a%nt s%((ered (rom ne#hrotic syndrome as a child *%t reco)ered *y adolescence Another maternal a%nt had cer)ical cancer *%t is no %nder (%ll remission SNAs #aternal %ncle is a ,non case o( SDE ith renal in)ol)ement here is no other record o( hereditary diseases s%ch as coronary heart disease and asthma in the (amily 'other( 31
)*+,E-( 3#
SNA. 1
Sister. 10
Brother. @
.e 'ale
1st eneration
)emale De/eased #nd eneration ' (aylors )niversity 201* +rite!up
Sister . -
Social History y #atients (ather is deceased ma,ing NSAs the mother the sole *readinner o( the (amily 5er highest le)el o( ed%cation as Form 2 She has her on (ood stall and earns aro%nd 00 ringgit #er day *%t her *%siness is constantly interr%#ted *y her da%ghters (re3%ent admission *eca%se she ill choose to ta,e care o( her rather than go to or, hen her da%ghter de)elo#s sym#toms 5oe)er. the (amily is c%rrently co#ing reasona*ly ell (inancially *eca%se NSAs (ather had li(e ins%rance hich they claimed and also *eca%se $ERES6 gi)es them a certain amo%nt each month 5oe)er. my #atients mother orries a*o%t (%rthering her childrens ed%cation a(ter they (inish high school SNAs (ather %sed to smo,e hea)ily *%t did not cons%me alcohol NSAs mother has ne)er smo,ed nor cons%med alcohol he #atient stays ith her n%clear (amily at $ay% &aras in a 3%arters ho%se here are residents in the ho%se here are no indos in the ho%se so it is a )ery st%((y and d%sty en)ironment hey ha)e 17 cats that ander in and o%t o( the ho%se hey do not li)e in a deng%e #rone area he other children are c%rrently le(t at home to (end (or themsel)es hile the mother is aay *eca%se the mother (eels they are old eno%gh to loo, a(ter each other She #re#ares their meals in ad)ance *e(ore going to her da%ghter in the hos#ital and ret%rns home *y the e)ening to loo, a(ter them he mother re#orts that the #atient #er(orms ell at school and gets along ell ith her si*lings and #eers here is no re#ort o( #eer #ress%re or si*ling ri)alry y #atient has *een in the (irst class o( her school since Standard and #er(ormed reasona*ly ell in her =$SR9As2Bs *%t she (eels that she co%ld ha)e done *etter i( she did not ha)e to *e (re3%ent a*sent (rom school 9d%e to her (re3%ent admissions She #lays s#orts o(ten ith her (riends (rom school and is acti)e in etrac%rric%lar acti)ities
Physical $)a#ination *eneral e)a#ination
6n ins#ection. the #atient is sitting com(orta*ly on the *ed tal,ing to her mother he #atient is alert and res#onsi)e here is mar,ed (acial #%((iness and #rominent #erior*ital oedema seen
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#ercentile and *elo the -th #ercentile res#ecti)ely 9re(er *elo 5er height is not %# to the normal standard tho%gh her eight (alls in the normal range (or her age he #atient is not in any a##arent #ain res#iratory distress here is an identi(ication tag on her right rist here is a meas%ring c%# (or the #atients %rine *%t it is not (illed at this #oint o( time here is also an in#%t o%t#%t chart hich shos the #atient is not olig%ric *%t there is c%rrently #ositi)e *alance here is e)idence o( *%((alo h%m#. acne. *r%ises. hirs%tism or striae that can *e seen here is no *%tter(ly rash or alo#ecia seen
(nthropo#etric Measure#ents 5eight : 1401cm 'ry eight : 40C ,g !%rrent eight: 4C ,g B< : 20@ ,g/m2 A centile groth chart (or her age and gender as #lotted. as shon *elo She eighed 40C,g. hich as #lotted to *e *eteen -0th and 2-th #ercentile. hile her height as meas%red to *e 1401 cm. hich as #lotted to *e *elo the -th #ercentile 5er B< as 20@,g/m2 hich is #lotted to *e *eteen the 7- th and -0th #ercentile
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6n eamination o( the hands.
he #alms are arm and #in, here is no #allor in #alm !a#illary re(illing time is less than 2 second No le%,onychia or ,oilonychias No (inger cl%**ing. No #eri#heral cyanosis. No signs o( in(ecti)e endocarditis li,e 6slers nodes. s#linter haemorrhages. &aneay lesions $%lse as @0 *eats #er min%te good )ol%me reg%lar rhythm 9a #oint against hy#o)olemic shoc,
Eamination o( the (ace
here is o*)io%s (acial #%((iness and #erior*ital oedema No #resence o( con?%ncti)al #allor No yelloish discolo%ration o( the sclera No *%tter(ly rash is seen here are no mo%th %lcers seen ong%e is not coated and she loo,s ell hydrated 6ral hygiene is good here is an in(lammation o( the throat or the tonsils No central cyanosis No halitosis as noted
Eamination o( the nec, Both the carotid #%lses ere reg%larly reg%lar and o( good #%lse )ol%me here is no selling in the region o( the thyroid here as no cer)ical lym#hadeno#athy
Eamination o( the loer lim*s
here as *ilateral #edal edema %# to the le)el o( %##er thigh No scars s%ggesti)e o( im#etigo or other in(ections No %lcers or gangrene No signs o( lim* ischaemia s%ch as cold etremities. loss o( hair. shiny s,in. #igmentation
Eamination o( the lym#h nodes
no lym#h nodes enlargements are noted
Vital Signs
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Blood #ress%re $%lse rate Res#iratory rate em#erat%re
: 10@/@0 mm 5g : @0 *eats #er min%te : 20 *reaths #er min%te : 7 !
All her )ital signs are ithin the normal range (or her age he #%lse rate is reg%larly reg%lar and o( good )ol%me here is no radio8radio or radio8(emoral delay he )ital signs do not s%ggest the #resence o( hy#o)olemic shoc, Assessment of dehydration
he #atient is a*le to #ass %rine reg%larly here are no s%n,en eyes he m%c%s mem*rane is moist as the tong%e a##ears to *e ell hydrated he #atient does not e#erience increased thirst Eamination o( )ital signs do not re)eal any sign o( dehydration s%ch as hy#otension. tachy#nea. ea, and tready #%lse 5ence. < o%ld li,e to commit that my #atient SNA is ell hydrated
+espiratory e)a#ination ith ho grossly edemato%s NSA a##eared. < as concerned ith the #ossi*ility o( #%lmonary edema *%t this as r%led o%t on eamination Inspection : !hest all o( the #atient mo)es symmetrically =sage o( accessory m%scle to *reath is not noted No )isi*le #%lsation or hea)e o)er the chest all he chest all is elli#soidal in sha#e here is no #ect%s eca)at%m or #ect%s carinat%m Palpation: No de)iation o( tracheal #osition !hest all e#ansion is symmetrical ocal tactile (remit%s as normal Percussion : $erc%ssion re)eals a resonant note at all l%ng (ields ece#t in the region o( cardiac and he#atic d%llness Auscultation: Breathing so%nd is normal
Cardio'ascular e)a#ination Inspection : No signi(icant (indings No ele)ated ?%g%lar )eno%s #ress%re 5e#ato?%g%lar re(le as negati)e No )isi*le #%lsation or hea)e o)er the chest all No sign o( #recordial *%lge A#e *eat is not )isi*le Palpation: A#e *eat is not dis#laced ie it is at the -th intercostal s#ace at ?%st medial to the midcla)ic%lar line No #al#a*le thrill is (elt De(t #arasternal hea)e is not noted No tenderness is (elt at right hy#ochondri%m %#on dee# #al#ation Auscultation: S1 and S2 heart so%nd can *e heard clearly ith normal intensity S and S4 heart so%nd cannot *e heard here is no m%rm%r
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*astrointestinal e)a#ination Inspection : he a*domen as distended symmetrically he %m*ilic%s as centrally #laced and in)erted here is a hy#o#igmmented area at the le(t l%m*ar area and a mole ?%st medial to it here is another mile ?%st *elo the %m*ilic%s All regions o( the a*domen mo)ed e3%ally ith res#iration and there ere no rashes. distended )eins. s%rgical scars. )isi*le #eristalsis. or )isi*le #%lsations seen Palpation: he a*domen as so(t and non8tender on s%#er(icial #al#ation. hile on dee# #al#ation the li)er and s#leen ere not #al#a*le Both the ,idneys ere not #al#a*le +here ere no other masses (elt Percussion : Shi(ting d%llness and (l%id thrill ere *oth noted to *e #ositi)e. indicating the #resence o( ascites Di)er s#an as C cm hich as normal (or her age S#lenomegaly not elicited as there as resonance in ra%*es s#ace Renal #%nch as %nremar,a*le Auscultation: Boel so%nd is normal at a*o%t + #er min%te here is no aortic or renal *r%it No )eno%s h%m co%ld *e a%sc%ltated
,er'ous Syste# $)a#ination $atient as (%lly conscio%s and orientated to time. #lace and #erson 5is higher (%nctions 9Dang%age. and memory ere intact !ranial Ner)es: All cranial ner)es ere normal otor system: Normal Sensory system: Normal
Su##ary y #atient is a 1 year old (emale child ho #resented ith s%dden #erior*ital selling hich #rogressed to (acial #%((iness. then. a*dominal and la*ial selling. then selling o( the lim*s associated ith 4; #rotein%ria the sym#toms ere #receded *y a one ee, history o( (e)er and diarrhoea She as diagnosed ith ne#hrotic syndrome at the age o( 10 *%t has no (eat%res s%ggesti)e o( secondary ca%ses o( ne#hrotic syndrome s%ch as SDE and c%rrentl y she has (re3%ent rela#ses and is steroid de#endent $ositi)e #hysical (indings incl%de #rominent #erior*ital oedema and (acial #%((iness. #edal oedema %# to le)el o( %##er thigh. a distended a*domen. #ositi)e shi(ting d%llness and (l%id thrill
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Pro'isional "iagnosis Steroid "ependent relapses
,ephrotic
Syndro#e
ith
fre%uent
Fa)o%ra*le #oints that #oint toards the diagnosis o( ne#hrotic syndrome are: •
here is edema hich initially de)elo#s aro%nd the eyes then s#read #rogressi)ely
•
donards to the rest o( the (ace. the a*domen. the genitals. then the arms and legs '%e the edema. there may *e associated increase in eight hich is seen in my
• • • • •
#atient9a @1 increase in *ody eight since the onset o( edema he #resence o( ascites as e)idenced *y #ositi)e shi(ting d%llness and (l%id thrill he #resence o( #itting #edal edema =rine di#stic, as 4; (or #rotein on admission !lo%dy a##earance o( %rine he (act that she is a ,non case o( ne#hrotic syndrome
5oe)er. there are also #oints against this diagnosis. namely: • •
here as no re#ort o( (rothy %rine he #atient as 10 years old hen she as initially diagnosed hich is rare as a )ast ma?ority o( #atients ith ne#hrotic syndrome ha)e minimal change disease hich is ty#ical in a yo%nger age gro%#98- years old hoe)er this can *e e#lained as a #ossi*le case o( a secondary ne#hrotic syndrome #artic%larly o( concern in my #atient *eca%se o( the (act o( my #atients age. gender and (amily history. systemic l%#%s ne#hritis ca%sing ne#hrotic syndrome 5oe)er. she ehi*its no sign o( this disease
$oints (or the diagnosis o( Steroid 'e#endent Ne#hrotic Syndrome ith (re3%ent rela#ses •
he #atient has had many rela#ses9more than rela#ses 4 in a 12 month #eriod hich
•
(its the criteria (or (re3%ent rela#ser She has had more than 2 consec%ti)e rela#ses occ%rring d%ring a steroid ta#er hich (its the criteria o( a steroid de#endent ne#hrottic syndrome
"i.erential diagnosis Steroid +esistant ,ephrotic Syndro#e Fa)o%ra*le #oints that #oint to ne#hrotic syndrome are: •
here is edema hich initially de)elo#ed aro%nd the eyes then s#read #rogressi)ely
•
donards to the rest o( the (ace. the a*domen. the genitals. then the arms and legs hich is ty#ical o( ne#hrotic syndrome '%e the edema. there may *e associated increase in eight hich is seen in my
• •
#atient9a @1 increase in *ody eight since the onset o( edema he #resence o( ascites as e)idenced *y #ositi)e shi(ting d%llness and (l%id thrill he #resence o( #itting #edal edema
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• • •
=rine di#stic, as 4; (or #rotein on admission !lo%dy a##earance o( %rine he (act that she is a ,non case o( ne#hrotic syndrome
5oe)er. there are also #oints against the diagnosis o( ne#hrotic syndrome. namely: • •
here as no re#ort o( (rothy %rine he #atient as 10 years old hen she as initially diagnosed hich is rare as a )ast ma?ority o( #atients ith ne#hrotic syndrome ha)e minimal change disease hich is ty#ical in a yo%nger age gro%# 98- years old hoe)er this can *e e#lained as a #ossi*le case o( a secondary ne#hrotic syndrome #artic%larly o( concern in my #atient *eca%se o( the (act o( my #atients age. gender and (amily history. systemic l%#%s ne#hritis ca%sing ne#hrotic syndrome 5oe)er. she ehi*its no sign o( this disease
$oints against steroid resistant ne#hrotic syndrome: •
y #atient NSA has alays *een res#onsi)e to steroid thera#y that is there ill *e a
•
res#onse to the initial 4 ee,s treatment ith #rednisolone at +0mg/m2/day hich ill not ha##en i( she is steroid resistant She ill %s%ally ha)e a rela#se once she is ta#ering don the steroids hich is more indicati)e o( *eing steroid de#endent
Syste#ic /upus $rythe#atosus 0/upus ,ephritis1 $oints in (a)o%r o( this diagnosis are: •
A ne onset ,idney disease can *e a #resenting (eat%re o( childhood onset systemic
• •
l%#%s erythematos%s SDE is more #re)alent in (emales more than C0H o( cases occ%r in omen here is edema hich initially de)elo#s aro%nd the eyes then s#read #rogressi)ely
•
donards to the rest o( the (ace. the a*domen. the genitals. then the arms and legs '%e the edema. there is an increase in eight hich is seen in my #atient9a @1
• • • •
increase in *ody eight since the onset o( edema he #resence o( ascites as e)idenced *y #ositi)e shi(ting d%llness and (l%id thrill he #resence o( #itting #edal edema =rine di#stic, as #ositi)e (or #rotein%ria on admission i)e s #osit Familial histor
$oints against the disease are: •
• • • •
$atient does not ha)e any other signs or sym#toms s%ggesti)e o( this diagnosis s%ch as (atig%e. ?oint #ain. malar rash. and (e)er A*sence o( hemat%ria A*sence o( olig%ria y #atient is normotensi)e Edema is not %s%ally as se)ere as it is in a child ith ne#hrotic syndrome
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•
$rotein%ria as 4;
Post-Streptococcal *lo#erulonephritis $oints in (a)o%r o( this diagnosis are: •
here is edema hich initially de)elo#s aro%nd the eyes then s#read #rogressi)ely
•
donards to the rest o( the (ace. the a*domen. the genitals. then the arms and legs '%e the edema. there may *e associated increase in eight hich is seen in my
• • • •
•
#atient he #resence o( ascites as e)idenced *y #ositi)e shi(ting d%llness and (l%id thrill he #resence o( #itting #edal edema $resence o( #rotein%ria any o( the e#isodes o( selling are %s%ally #receded *y a %##er res#iratory tract in(ection
$oints against the diagnosis are: • • •
• •
A*sence o( hemat%ria A*sence o( olig%ria y #atient is normotensi)e Dac, o( im#etigo scars 9Gro%# A B8hemolytic Stre#tococc%s8commonest ca%se o( #ost8stre#tococcal glomer%lone#hritis Edema is not %s%ally as se)ere as it is in a child ith ne#hrotic syndrome $rotein%ria as 4;
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n'estigation Bloods &ull Blood Count
Hemoglobin White blood cell Hematocrit !V !H !H! "#W Platelet
Value 12 1@40 7C 2C+ +0 142 @1
Normal range 911081+0g/dl 940811010 C/l 9408-0H 97+08C+0(l 927080#g 9208+0g/l 91181+H 91-084-010 C/l
Interpretation Normal
E)al%ation: here is an increase in total hite *lood cell hich may *e e#lained *y the (act my #atient had a history o( ha)ing ac%te gastroenteritis *e(ore de)elo#ing #erior*ital selling 5oe)er. it is im#ortant to ,ee# in mind that this can *e indicati)e o( a #ossi*le glomer%lone#hritis 6therise. all the #arameters are normal
/i'er &unction Test his test is done to e)al%ate #rotein le)els #artic%larly the al*%min le)el hich ill *e mar,edly red%ced. ty#ically I2-g/D
otal $rotein Glo*%lin Al*%min / Glo*%lin Ratio otal Bilir%*in Alanine ransaminase 9SG$ Al*%min Al,aline $hos#hatase
Value 00 g/D 4 g/D 0C
Normal range 9+48@g/D 92@84g/D 0@820
Interpretation Do 5igh Normal
@ Jmol/D C =/D
9821%mol/D 908--=/D
Normal Normal
+ g/D 1- =/D
9-8-0g/D 94081-0=/D
Do Normal
E)al%ation: here is hy#oal*%minemia that is ser%m al*%min is I2-g/D hich is one o( the (o%r diagnostic criteria o( ne#hrotic syndrome here is also red%ced total #rotein hich is
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#ro*a*ly attri*%ta*le to the lo ser%m al*%min he other #arameters are ithin the normal range
+enal &unction Test o assess ,idney (%nction and to detect any a*normalities or #ossi*le ,idney (ail%re hich 9altho%gh highly %nli,ely in this case. may *e a di((erential diagnosis (or anarsarca and also to detect any electrolyte a*normalities his test can also r%le o%t glomer%lone#hritis *y con(irming the a*sence o( a"otemia as ell as l%#%s ne#hritis hich #resents ith increased %rea and creatinine
$rea Sodium Potassium !hloride !reatinine
Value 41C 44 110 44
Normal range 9-8+-mmol/l 91-814-mmol/l 9-8-0mmol/l 9C@8107mmol/D 9+08120%mol/l
Interpretation Normal Normal Normal 5igh Do
E)al%ation: here is increased chloride hich may *e d%e to dehydration here is also a lo creatinine hich may *e d%e to the (act my #atient is c%rrently in a state o( hy#o#roteinemia he other #arameters are normal here is no a"otemia or increased creatinine hich is a #oint against l%#%s ne#hritis and glomer%lone#hritis
Seru# Co#ple#ent his test is done to ecl%de #ost8stre#tococcal glomer%lone#hritis 9lo ! normal !4 and SDE 9lo ! and !4
! !4 E)al%ation: Ser%m ! and !4 le)els are normal
Value 10+ g/D 024 g/D
(nti-streptolysin Titer A high AS6 le)el signi(ies recent stre#tococcal in(ection hich is s%ggesti)e o( #ost8 stre#tococcal glomer%lone#hritis Value AS6 100 %nits/mD E)al%ation: AS6 is negati)e9I200%nit/mD $ost stre#tococcal glomer%lone#hritis in %nli,ely
(ntinuclear (nti3ody4 anti-as",( he history my #atient #resents ith that is her age at initial #resentation. her gender and her #ositi)e (amily history has me s%s#icio%s o( SDE hich co%ld either ca%se secondary ne#hrotic syndrome or l%#%s ne#hritis h%s. < o%ld li,e to screen (or SDE %sing this test Assessment: his as done *y 5os#ital S%ngai B%loh earlier this year in Se#tem*er d%ring a #re)io%s admission (or one o( her rela#ses
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'ate:10/C/201+8 ANA is negati)e he res%lt as negati)e at the time. hoe)er < o%ld li,e to do the test again to r%le o%t the #ossi*ility o( a (alse negati)e
Body &luids Urinalysis4U&$M$ his is %sed to detect #rotein%ria his is to r%le o%t glomer%lone#hritis as the #resence o( RB! casts is almost #athognomonic o( glomer%lone#hritis his test can also detect microsco#ic hemat%ria hich ill *e %se(%l (or diagnosis ne#hritic syndrome Res%lts: 'i#stic, done in the re)ealed #rotein ithin the range o( 2; to 4; (or consec%ti)e days hich s%##orts the diagnosis o( ne#hrotic syndrome
Urine protein5 creatinine ratio his is to o*tain e)idence o( #rotein%ria A single s#ot %rine collection is m%ch easier to o*tain than a 24 ho%r %rine sam#le tho%gh in this case my #atient is old eno%gh to *e a*le to do so E)al%tion: he %rine #rotein: creatinine ratio as in the ne#hrotic range o( #rotein%ria ie K200 mg/mmol
26 hour urine protein his is to o*tain e)idence o( #rotein%ria E)al%tion: $rotein%ria o( K40 mg/m2/day as recorded ith s%##orts the diagnosis o( ne#hrotic syndrome
(dditional in'estigations •
6ther la*oratory tests associated ith the #resence o( in(lammation. s%ch as
•
erythrocyte sedimentation rate 9ESR and/or !8reacti)e #rotein 9!R$ may also *e %sed to e)al%ate a #erson (or SDE A li#id #ro(ile can *e done as ne#hrotic syndrome #resents ith
•
hy#ercholesterolaemia his test as not done in my #atient d%ring her c%rrent admission Renal *io#sy o%ld *e diagnostic o( a #rimary or idio#athic ne#hrotic syndrome *%t it is mainly indicated hen one is #ro)en to *e steroid resistant hich is not the case in my #atient
Working "iagnosis
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Steroid 'e#endent Ne#hrotic Syndrome ith (re3%ent rela#ses as chosen as the or,ing diagnosis *eca%se it most closely (its the descri#tion gi)en in the history and the (indings on #hysical eamination that is the edema that generally *egins aro%nd the eyes and (ace *e(ore #rogressing donards among other things
Manage#ent *eneral Manage#ent 1 $atient is admitted in ard @! (or monitoring and (%rther in)estigations to con(irm this is a rela#se rather than a di((erent disease entirely and to ecl%de ca%ses o( secondary ne#hrotic syndrome 2 A normal #rotein diet ith ade3%ate calories is recommended A lo salt diet is ad)ocated hen child has edema No (l%id restriction as done as it is not recommended ece#t in chronic edemato%s states 4 entilate on room air - Strict in#%t8o%t#%t9<6 charting and ne#hrotic charting ith *lood #ress%re monitoring 28ho%rly + 'aily =rine FEE and eight meas%rement are ordered 7 $atient as ad)ised on ho to do a 24 ho%r %rine collection
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@ $atients mother is co%nseled once again a*o%t #rednisolone treatment. indication o( steroid treatment. *ene(its and ris,s o( steroid treatment on #atients c%rrent illness other is also co%nseled regarding the need o( daily =FEE C $enicillin -00 mg B' 9K 12 years is recommended at diagnosis and d%ring rela#ses. #artic%larly in the #resence o( gross edema 10 Reind%ction o( oral #rednisolone 11 $atients general condition and )ital signs are monitored e)ery 4 ho%rs to assess haemodynamic stat%s and to r%le o%t com#lications li,e a 5y#o)olaemia * 5y#er)olaemia c hrom*oem*olism d $eritonitis
Patient Progress +eport 26477478 "ay 7 of ad#ission "ay 9 of illness
$rogress: y #atient a##eared grossly edemato%s ith #rominent #erior*ital edema. (acial #%((iness. la*ial selling. clinical ascites and #edal edema *%t hoe)er as otherise ell and com(orta*le at rest here is no sign o( shortness o( *reath. a*dominal #ain. tachycardia. cool #eri#heries or other signs o( com#lications !%rrently. my #atient is no longer (e*rile *%t still has diarrhea9loose stool 84 times a day =rine di#tic, as 4; edications #rescri*ed: • •
$atient is started ith oral #rednisolone 7-mg 6' 9+0mg/m2/day $ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B'
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$atient as gi)en 6RS sachets and ta%ght ho to dil%te them
2:477478 "ay 2 of ad#ission "ay of illness
$rogress: $atient is not yet im#ro)ing clinically #erior*ital edema. (acial #%((iness. la*ial selling. ascites and #edal edema has not im#ro)ed and in (act is orsening =rine di#stic, maintained at 4; 5oe)er. NSA is alert. cheer(%l and acti)e $atients )ital signs are sta*le she is normotensi)e. a(e*rile. no tachycardic or tachy#nic he decision is made to add on (%rosemide to red%ce the edema altho%gh di%retics9according to the $aediatric $rotocols For alaysian 5os#itals is not necessary in steroid res#onsi)e ne#hrotic syndrome he #atient is to *e monitored closely (or signs o( hy#o)olemia d%ring the %se o( (%rosemide $atient re#orts (re3%ency o( de(ecation has red%ced edications #rescri*ed: • • • •
6ral #rednisolone 7-mg 6' 9+0mg/m2/day $ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B' Additional 6RS sachets < (%rosemide 20mg/2ml
28477478 "ay < of ad#ission "ay 7= of illness
'es#ite adding (%rosemide. %atient is still not im#ro)ing clinically the degree #erior*ital edema. (acial #%((iness. la*ial selling. ascites and #edal edema is %nchanged and mother noticed that #atients %rine o%t#%t decreased mar,edly 5oe)er. #atient still a##ears com(orta*le. her )itals are sta*le and her diarrhea has resol)ed
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6ral #rednisolone 7-mg 6' 9+0mg/m2/day
•
$ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B'
•
< (%rosemide 20mg/2ml
•
5%man al*%min 20H in?ection 100ml
2>477478-<=477478 "ay 6-> of ad#ission "ay 77-76 of illness
$atient as sitched to ard 7A *eca%se she as relati)ely sta*le des#ite shoing little im#ro)ement he al*%min in(%sion as contin%ed o)er a #eriod o( 4 days and s%*se3%ently. the #atients condition im#ro)ed her #erior*ital edema and (acial #%((iness decreased greatly. ascites as still clinically e)ident *y shi(ting d%llness and (l%id thrill *%t #edal edema only as %# to the le)el o( the ,nee =rine o%t#%t has increased '%ring this 4 day #eriod. oral s#ironolactone as added to co%nteract the #otassi%m8losing e((ect o( (%rosemide 9a loo# di%retic as s#ironolactone is a #otassi%m8s#aring di%retic Also. < (%rosemide as sitched to oral9syr%# (%rosemide edications #rescri*ed: •
a*let #rednisolone 7-mg 6' 9+0mg/m2/day
•
$ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B'
•
Syr%# (%rosemide 1mg/ml 92-mg 'S
•
5%man al*%min 20H in?ection 100ml
•
Syr%# s#ironolactone 1mg/ml92-mg 'S
7472478 "ay 9 of ad#ission "ay 7: of illness A(ter the al*%min in(%sion. #atients condition contin%es to im#ro)e $erior*ital edema and (acial #%((iness decreased greatly. ascites as still clinically e)ident *y shi(ting d%llness *%t (l%id thrill as negati)e *y day 7 o( admission and #edal edema only as %# to the le)el o( the ,nee 5er )itals ere normal and there ere no signs o( com#lications =rine di#stic, is no 2; and c%rrent eight is 4--,g
edications #rescri*ed:
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a*let #rednisolone 7-mg 6' 9+0mg/m2/day
•
$ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B'
•
Syr%# (%rosemide 1mg/ml 92-mg 'S
•
5%man al*%min 20H in?ection 100ml
•
Syr%# s#ironolactone 1mg/ml92-mg 'S
24724780Present "ay1 "ay of ad#ission "ay 78 of illness
y #atients condition contin%es to im#ro)e $erior*ital edema and (acial #%((iness decreased. ascites and #edal edema ha)e decreased 5er )itals ere normal and there ere no signs o( com#lications =rine di#stic, is no 2; and c%rrent eight is 44C ,g edications #rescri*ed: •
a*let #rednisolone 7-mg 6' 9+0mg/m2/day
•
$ro#hylactic anti*iotic is gi)en: 6ral #enicillin -00mg B'
•
Syr%# (%rosemide 1mg/ml 92-mg 'S
•
5%man al*%min 20H in?ection 100ml
•
Syr%# s#ironolactone 1mg/ml92-mg 'S
At this #oint o( time. my #atient cannot *e discharged yet 5oe)er %#on discharge s#ecial considerations to *e gi)en are: •
o do home %rine al*%min monitoring: once daily di#sti testing o( the (irst morning
•
%rine s#ecimen he #atient is ad)ised to cons%lt the doctor i( al*%min%ria L 2; (or consec%ti)e days. or o%t o( 7 days
•
regardless o( the %rine di#sti res%lt !hildren on systemic corticosteroids or other imm%nos%##ressi)e agents sho%ld *e
•
ad)ised and ca%tioned a*o%t contact ith chic,en#o and measles. and i( e#osed sho%ld *e treated li,e any imm%nocom#romised child ho has come into contact ith these diseases Regarding imm%ni"ation. hile the child is on corticosteroid treatment and ithin + ee,s a(ter its cessation. only ,illed )accines may sa(ely *e administered to the child
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and li)e )accines can *e gi)en only + ee,s a(ter cessation o( corticosteroid thera#y 5oe)er. it is im#ortant to ad)ocate that *oth #ne%mococcal and )aricella "oster )accine *e administered to all children ith ne#hrotic syndrome <( #ossi*le. gi)e hen the child is in remission y #atient sho%ld also *e co%nseled a*o%t ac%te adrenal crisis
$'idence-Based Medicine 71Ho e.ecti'e are steroids in the treat#ent of fre%uently relapsing nephrotic syndro#e? "o alternati'e treat#ents ha'e a 3etter success rate? =# to @0H o( children ith idio#athic ne#hrotic syndrome res#ond to corticosteroids. ith a com#lete remission occ%rring ithin 0 days A##roimately one third o( these #atients are c%red a(ter the co%rse o( corticosteroids Another 10820H o( #atients. e#erience rela#ses se)eral months a(ter sto##ing the treatment and a c%re ta,es #lace a(ter three or (o%r e#isodes. hich res#ond to a standard co%rse o( corticosteroids 5oe)er. %# to 40 to -0H o( #atients e#erience (re3%ent rela#ses either as soon as steroid thera#y is sto##ed 9(re3%ent rela#sers or hen the dosage o( steroids is decreased 9steroid de#endent 91 here are many (actors may ca%se the rela#se o( #rimary ne#hrotic syndrome. incl%ding too short steroid treatment #eriod. ra#id ta#ering o(( the dose o( #rednisone. in(ections. etc he most im#ortant (actor is too short #rednisone treatment #eriod A st%dy *y ang et al ado#ted #rolonged #rednisone treatment #l%s tri#terysi%m gl%cosides thera#y9a traditional !hinese medication res%lts shoed that children d%ring the last three months o( #rednisolone thera#y adding tri#terysi%n gl%cosides hel#ed to strengthen the treatment and red%ce the re8 rela#se92 5oe)er. st%dies *y a,eda et al that cyclo#hos#hamide8controlled #atients ha)e more sta*le long8term remission com#ared ith tri#terysi%m gl%cosides gro%# 9 5oe)er it is o( note that tri#terysi%m gl%cosides has less side e((ects com#ared to cyclo#hos#hamide he (irst second line dr%g is %s%ally cyclo#hos#hamide and indeed it is shon that this dr%g may *e o( #artic%lar %se in children ith (re3%ent rela#ses as in a st%dy *y Shohet et al. a gro%# o( #atients recei)ed cyclo#hos#hamide (or -+ days in a single daily dose in order to #rolong the length o( remission and it as shon that the #ercentage o( #atients ho contin%ed in remission at the end o( the 1st. 2nd and -th years as greater in the (re3%ent8 rela#ser gro%# 9- Another treatment s%ggestion is #%lse intra)eno%s cyclo#hos#hamide thera#y in (re3%ently rela#sing ne#hrotic syndrome. hoe)er there it co%ld not *e #ro)en to ha)e a greater e((icacy than the con)entional oral steroid treatment94. 10 Some #hysicians ad)ocate the e((ecti)eness o( al,ylating agents in the treatment o( (re3%ent rela#sers. in a certain #atient ho had o)er 100 rela#ses a short cycle o( chloram*%cil 902 mg/,g/day (or @ ee,s as the dr%g that *ro%ght a*o%t a lasting com#lete remission911
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Finally. a st%dy *y B !ammas et al shoed that long8term e((icacy o( cyclo#hos#hamide in steroid8res#onsi)e ne#hrotic s yndrome is disa##ointing9+ Met another st%dy s%##orted the s%#eriority o( steroids as a (irst line o( treatment in #atients ith ne#hrotic syndrome *e they (re3%ent rela#ses or not *y rea((irming that that tho%gh le)amisole may *e considered an alternati)e (or cyclos#orin as a (irst second8line agent (or #atients it co%ld not re#lace steroids97
21 "oes 3eing a fre%uently relapsing patient #ean that they ha'e a orse prognosis? he longterm #rognosis o( idio#athic ne#hrotic syndrome is (a)o%ra*le
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+eferences 1 Nia%det $ Dong8erm 6%tcome o( !hildren ith Steroid8Sensiti)e
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11 !arreno A he #atient ith o)er 100 rela#ses o( minimal change ne#hrotic syndrome: #rolonged com#lete remission a(ter chloram*%cil treatment Ne#hrology 'ialysis rans#lantation 20001-9+:C228a8C2 12 5a?i %hammad
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