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Transcript of Jaundice, diarrhoea and other gastroenterological · Jaundice, diarrhoea and other...
Jaundice, diarrhoea and other gastroenterological symptoms in childhood AGNIESZKA WEGNER
KL INIKA NEUROLOGI I DZIECIĘCEJ
WARSZAWSKI UNIWERSYTET MEDYCZNY
VomiJng 1. Vomi%ngisthemeansbywhichtheuppergastrointes%naltractridsitselfofitscontents
whenalmostanypartoftheuppertractbecomesexcessivelyirritated,overdistended,orevenoverexcitable.Vomi%ngisasymptom,presen%ngcomplaintinmul%tudeofdisorders.Rangefromgastrointes%nalpathologytodiseaseindistantorgan(o%%smediaorintracraniallesion)
2. Nausea:Theunpleasantsensa%onoftheimminentneedtovomit,usuallyreferredtothethroatorepigastrium;asensa%onthatmayormaynotul%matelyleadtotheactofvomi%ng
3. Regurgita%on:Theactbywhichfoodisbroughtbackintothemouthwithouttheabdominalanddiaphragma%cmuscularac%vitythatcharacterizesvomi%ng.
VomiJng- causes Infant Child Adolescent
Common • Gastroenteri%s• Gastroesophageal
reflux• Overfeeding• Anatomic
obstruc%on• Systemicinfec%on• Pertussissyndrome• O%%smedia• Pyloricstenosis
• Gastroenteri%s• Gastri%s• Reflux(GERD)• Systemicinfec%on• Pertussissyndrome• Sinusi%s• O%%smedia
• Gastroenteri%s• GERD• Gastri%s• Inflammatorybowel
disease• Appendici%s• Systemicinfec%on• Sinusi%s• Migraine• Pregnancy
VomiJng- causes Infant Child Adolescent
• Rare • CAH• Inbornerrorofmetabolism• Braintumor(increasedintracranialpressure)• Subduralhemorrhage• Foodpoisoning• Renaltubularacidosis
• Reyesyndrome• Hepatitis• Pepticulcer• Pancreatitis• Braintumor• IncreasedICP• Middleeardisease• Achalasia• CyclicvomitingEsophagealstricture• Duodenalhematoma• Inbornerrorofmetabolism
• Reyesyndrome• Hepatitis• Pepticulcer• Pancreatitis• Braintumor• IncreasedICP• Middleeardisease• Cyclicvomiting• Biliarycolic• Renalcolic
Interview q Ageofthepa3entq Dura3on/Frequency
q Onset
q Associatedwithfoodintakeü instantly:esophagealobstruc%onü ATerawhile:stomachorduodenalobstruc%on
q Nature(projec3le/nonprojec3le)
q Colorandcontentsü Nondigestedfood:proximalobstruc%onü Semidigestedfood:distalobstruc%onü Billouscontent:distalto2ndpartofduodenumü Fecalmaterial:obstruc%onatthelargeintes%ne
q Associatedsymptomsü Fever/AbdominalPain/Diarrhea/cons%pa%on/dysphagia
q Respiratory–cough,chestdiscomfort
q Urinary–dysuria,hematuria
q CNS–irritability,alteredsensorium,drowsy,necks%ffness,headache,visualdisturbance
q PastmedicalhistoryAnyknownmedicalillnesssuchasmetabolicinbornerror,cerebral
palsy,downsyndrome,neurologicaldeficit
q Drugandallergyhistory
q Birthhistory
q Nutri%onalhistoryRecentlychangeintocowmilk/foodallerrgy/typeoffood
q OtherrelevanthistoryRecentea%ngoutside,recenttravelling,familymemberorfriendsin
schoolhavesimilarillness
VomiJng- examinaJon q Generalcondi%on§ Comparisonofpa%ent’sweightbeforeandaTeronsetofillness
q Hydra%onstatus§ Sunkenfrontanelle§ Eyessunkenandtearless§ Drymucousmembrane§ Prolongedcapillaryrefill%me§ Reducedskinturgor§ Tachycardia,tachypnea
q AbdominalExamina%on◦ Distension,Visibleperistalsis,Tenderness,hepatospelnomegaly,abdominalmasses,Bowelsounds
q CNSExamina%on Power,Tone,reflexes Changesinvisionq RespiratoryExamina%on,Earexamina%on Rehydrateaccordingly-encourageoralintake Correctelectrolyteimbalances Treataccordingtotheunderlyingcause
Jaundice Jaundiceisayellowishpigmenta%onoftheskin,theconjunc%valmembranesoverthesclerae(whitesoftheeyes),andothermucousmembranescausedbyhighbloodbilirubinlevels.
q Thecondi%onforthecrea%onofjaundiceisanincreasedbilirubinserum.
q Normallevelsofbilirubininthebloodserumrangesfrom0.3-1.0mg/dl,withnearly90%ofconjugatedbilirubin.
q Bilirubiniscreatedinliver,spleenandbonemarrowasaproductofthecatabolismof:
q hemoglobinfromerythrocytes80-85%
q othercombina%onsofcompoundscontainingheme/myoglobin,cytochromes,especiallyCP-450,otherenzymescontainingheme
q hemoglobinderivedfromineffec%veerythropoiesis/maturingerythrocytes/
ClassificaJon of jaundices Liverplaysamailroleinbilirubinmetabolism.Therearethreestagesofbilirubinmetabolisminliver:1. uptakeofbilirubin2. conjuga%onwithglucuronicandsulfericacid3. excre%onofconjugatedbilirubinintobile
Classifica%onofthejaundice Pre-hepa%c/hemoly%c-Intrinsicdefectsinredbloodcellsorextrinsiccausesexternaltoredbloodcells Hepa%c/hepatocellular–liverdisease Post-Hepa%c/cholesta%c-obstruc%onofbiliarypassage
HemoliJc jaundice Ø Structuralerythrocytesanomalies-defectsinmembrane/sicklecellanemia/,enzymopa%e,hemoglobinopathies,thalassemia
Ø extrinsiccauseshaemolysis
- immunological/auto-,izoan%bodies/
- chemical/intoxica%onPb,Cu,drugs/
- mechanical/valvularprosthesis/
- infec%on/bacterialsespis/- Hipersplenizm,metabolicaldisorders/uricemia/
DiagnosisØ FamilyhistorY,drugs,
Ø weakness,worseexercisetolerance
Ø normalurine,darkstools,strawyellowdiscolora%onoftheskin
Ø tachycardia,hypersplenism
Ø predominanceofindirectbilirubin/unbound/
Ø anemia,re%culocytosis
Ø amarkedincreaseinLDH,aslightincreaseintransaminases
Ø lackofserologicalmarkersofviralinfec%ons
Unconjugated bilirubin predominance Ø Increasedproduc%on- Intra-andextravascularhemolysis- Ineffec%veerythropoiesis
Ø Impaireduptakebilirubinbyhepatocytes- Sepsis,drugs,long-termhunger,hyperthyroidism
Ø Imapiredconjuga%onofbilirubin- Gilbert'ssyndrome,Crigler-Najjarsyndrome,neonataljaundice
Jaundice caused by congenital enzymaJc defects of hepatocytes Ø GilbertSyndromeØ abnormalbindingofbilirubinwithglucuronideacid–deficiencyofUDPG
Ø Familycharacter,thepredominanceofindirectbilirubin,normallevelsofliverenzymes,noevidenceofhemolysis,bilirubinrarelyexceeds5mg/dl
Ø Crigler-NajjarSyndromeØ bilirubinmetabolismdefectassociatedwithdeficiencyofUDPG
Ø TypeI-childrendieinthefirst2yearsoflifeØ TypeII-normallevelsofenzymes,<20mg/dlbilirubin
Ø predominanceofindirectbilirubin
Ø Dubin-JohnsonSyndromeØ secre%ondisorderassociatedbilirubinpolegallhepatocytes
Ø familycharacter,thepredominaneofconjugatedbilirubin,
Ø bilinubinuria,thepresenceofthedyeinhepatocytesmelanin-like
Ø RotorSyndromeØ VarietyofDubin-Johnsonsyndrome,theabsenceofthedyedepositsinhepatocytes
Ø NewbornsjaundiceØ immaturityofenzyma%chepatocytes-deficiencyUDPGtransferase
HepaJc jaundice- causes Ø Infec%onØ viral:HAV,HBV,HCV,HDV,HEV,EBV,TORCH,Echo,Parvo,
Ø listeriosis
Ø ToxicØ drugs-cytosta%cs,paracetamol,TPN
Ø Chemicalcompounds-carbontetrachloride
Ø mushrooms-Amannitaphalloides
Ø Alcohol
Ø MetabolicalØ Wilsondisease-disordersofCumetabolism
Ø hemochromatosis-disordersofFemetabolism
Ø alfa-1antytrypsindeficiency
Ø Galactozemia,fructozemia,glycogenosisIV,tyrosinemia,cys%cfibrosis
Ø Autoimmunological/AIH/Ø AIHtypI,IIa,IIb,III
Ø Primarybilliarycirrhosis/PBC/
Ø Primarysclerosingcholangi%s/PSC/
Ø other-Alagille’asyndrome,Aegenessydrome
Diagnosis Ø interview-surgery,bloodtransfusion,alcohol,drugs,chemicalsØ darkurine,stooldiscolora%onØ Cutaneous/vascularspiders,palmarerythema,purpuraflaw/Ø enlargedliverandspleenØ predominanceofdirectbilirubin/connected/Ø asignificantincreaseinthelevelofALT,ASTinserumlesspronouncedFA,GGTØ leukopeniaandthrombocytopenia/hypersplenism/Ø decreaseinalbumin,gammaglobulinincrease
Ø serologicalmarkersofinfec%onHAV,HBV,HCV,HDV,CMV,EBV
Ø autoan%bodiesANA,AMA,ANCA,LKM,SLA
Ø reduc%oninthelevelofceruloplasmin,
Ø highcopperconcentra%onsinserumanddailyurinecollec%on
Ø elevatedlevelsofFeØ Elevetadurobilinogeninurine,bilirubinuria
Jaundice extracellular- cholestasis Congenitalobstruc%onofextrahepa%croadsżółciowych-biliaryatresia Cystofcommonbileduct
Narrowingofthebileducts Urolithiasisand/orspontaneousperfora%onofthecommonbileduct
Diagnosis Ø interview-symptomsofbiliarycholelithiasis,itching,weightloss
Ø darkurine,stooldiscolora%onØ enlargementofthegallbladder,Chełmońskisymtome,liverenlarged,hard,withunevenshore
Ø predominanceofdirectbilirubin/related
Ø asignificantincreaseinGGT,FA,smallALT,AST
Ø leukocytosis,increasedESR,anemia
Ø lackofserologicalmarkersofhepa%%sinfec%on
Ø Abdominalultrasound-thelackofgallbladder
Ø Biopsywątroby-to3weeksoflife->GOLDSTANDARDDIAGNOSISbiliaryatresia
Ø ERCP
Ø CT,MR
Ø PTC-percutaneouscholangiografia
Abdominal pain Abdominalpaininchildrencanbeasymptomofpsychosocialstress,asinadultstensionheadacheintheoccipitalregion.
Childrenusuallylocalizedpainaroundthenavel. Division• acute• chronic• recurrent
Acute abdominal pain q mayhaveamildstart,thengraduallythepainincreases
q themomentofhisappearancecanbeappliedtoaspecificdate
q painwakesthechildorpreventssleep,interruptsplayq itisnon-recurring
q rarelysubsideswithouttreatment
q oTenaccompaniedbyothersymptoms:nausea,vomi%ng,diarrhea,fever,lackofappe%te
q thepa%entisanxious,unwillingnesstoinves%gate
q posi%onwithcurledlegs-protectsthestomach,decreasingmuscletensionofthestomach
Acute abdominal pain- causes SURGICAL
appendeci%s
intussep%on
cystorovariantorsion
NON-SURGICAL
cons%pa%on
gastroenteri%s
ovulatorypain
simula%on
urinarytractinfec%on
haemoly%curaemicsyndrome
hepa%%s
Chronic abdominal pain q prevelence20-30%ofchildren5-14q restrictsthenormalac%vityofthechild-only1/10pa%entsregularlygotoschool
q to9yearsofageoccurswithequalfrequencyinboysandingirls,olderchildrenmoreoTeningirls
q maybealsorelapsing
q theremaylastforseveraldaysorweeks
q individualepisodeslastupto3hours,morethanhalfofchildrenabout1hour
q seizuresusuallyoccurindrovesq paininterfereswithfallingasleep,butdoesnotraiseachildq inmostchildrendis%nctperiodswithoutabdominalpain,butoccasionallythepaincanbecon%nuous
q Itcanbeassociatedwithothersymptoms:nausea,vomi%ng,diarrhea,headache,dizziness,fa%gue
The causes of chronic abdominal pain q Themostcommoncause-func%onalabdominalpain
q Infec%on-yersiniosis,giardiasis,H.pyloriinfec%on,UTIq cons%pa%onq Foodalergyq Foodintolerance-disaccharidoses-fructose,lactose,sucroseq celiacdiseaseq Anatomicalchanges-ovariancyst,pancreasdivided,gastrointes%nalileus/subileus
q Inflammatoryboweldisease-ulcera%vecoli%s,Crohn'sdisease
q Epilepsy,migraine
q Drugs:NSAIDs,steroids,ironprepara%ons
Chronic abdominal pain FUNCTIONALABDOMINALPAIN
propergrowth lackoflocalizedsymptoms
painspilled painaroundthenavel
ORGANICABDOMINALPAIN
painradia%ngtotheback,chest,hips,associatedwiththeintakeoffoodorflushing
painawakensthechildfromsleep
painlocated,limited
inhibi%onofbodyweightgain
Othersymptoms-fever,vomi%ng,jointpain,gastrointes%nalbleeding,painaroundlumbararea,changesontheskinaroundtheanusoranalfissure,analincon%nence,abdominaltumors,hepatosplenomegaly
Abdominal pain- diagnosJcs Laboratorytests:bloodcount,ESR,CRP,ALT,AST,amylase,crea%nine
Urinetest
Stooltest:culture,viruses,occultblood,parasites,H.pylorian%geninstooltestELISA-Giardiaintes%nalis
abdominalultrasound
Diarrhoea- diagnosis Thehigherthannormalnumberofstoolsperday(oTenmorethentwice),orchangeinthenatureofstool(theyarewateryorsemi-liquid),orthechild'sstoolscontainmucus,bloodorsputum
WHO-acondi%oninwhichachild<2yearsofage,ar%ficiallyfedormixedhas
threeormoreliquidorsemi-liquidstoolsper24hours
or 1abnormalstoolhavingbloodormucusorpuswithin24hours
Diarrhoea- classificaJon Divisionduetodura%on:◦ Acutediarrhea<14days(usually3-5days)◦ Prolongeddiarrhoea14-30days◦ Chronicdiarrhea>30days(>14days)
Thedivisionbecauseofthee%ology◦ Noninfec%ousdiarrhea◦ Infec%ousdiarrhea,fever,bloodinthestool,nauseaand/orvomi%ng,◦ an%bio%cs
Thedivisionduetothemechanism◦ Osmo%cdiarrheacausedbyintes%nalinabilitytoabsorbexcessiveamountsofsubstancesthatretainwater,therebyincreasingstoolvolume(eg.lactoseintolerance)
◦ Secretorydiarrheacausedbyac%vesecre%onintotheintes%neoflargeamountsofsalt,waterandothersubstances(infec%onagents,laxa%ves)
Acute diarrhoea- causes Infec%onsenteral->viruses,bacteria,exotoxins,protozoa Infec%onsoutsidethegastrologicaltract->UTI,o%%smedia,pneumonia
Surgicaldiseases->appendici%s,intussuscep%on,obstruc%on Drugs:->An%bio%cs
Viral diarrhoea HRV(Rhinoviruses)ofgroupA,BandC◦ infec%onthroughoral-faecal◦ infec%oniscomprisedofsmallintes%ne◦ shortincuba%onperiod(1-3days)◦ oTenexcessivevomi%ng(1-2days)◦ Acutewaterydiarrhea(usually2-7days)◦ oTenfever◦ 20-40%-rhini%ssymptomsofupperrespiratorytract
◦ Diagnosis:ELISAoralatexagglu%na%onassay
Adenoviruses◦ tendencytoprolongthediarrhea◦ OTenaccompaniedbysymptomsofupperrespiratorytractinfec%ons
◦ clinicalcourseisusuallymild◦ severedehydra%on,acidosis-inthecaseofseverevomi%ngand/ordiarrheawithoutpropertreatment
Rotavirus◦ Themaincauseofseverediarrheainyoungchildren
◦ Europe:-autumn-winterseason◦ Childrenaged6-24months◦ Oralanddroplet◦ OTenasnosocomialinfec%onsandepidemics◦ vaccina%on
Bacterial diarrhoea Thecourseisself-limi%ng!
Waterystoolswithmucus,pusorblood
Thetendencytothreading(Salmonella,Shigella,Yersinia,Campylobacter,EPEC)
Cancallsep%cemiaandsystemicinfec%ons(Salmonella,Yersinia)
Traveler'sdiarrhea(ETEC,Shigella,Salmonella,Campylobacterjejuni,Vibriocholerae,Cryptosporidiumparvum,Giardiaintes%nalis,Entamoebahistoly%ca)
ETEC EnterocytotoxicalE.coli;ETEC,Shiga-liketoxinproducingE.coli;verotoxin-producingE.coli◦ Produc%onverocytotoxins(VT1,VT-2)◦ Clinically:bloodydiarrhea,usuallywithoutfever◦ E.coliO157:H7-afrequentcauseofbloodydiarrheaindevelopedcountries◦ undercookedorundercookedbeef,hamburgers,drinkingwater,unpasteurizedmilk,yogurt,vegetables
◦ Lackofsensi%vitytochemotherapy
◦ Complica%on:Hemoly%curemicsyndrome(upto10%infectedwithE.coliO157:H7
Salmonella q Infec%onofatyphoidcancausebacteremiaandfocusofinfec%onintheGItract(bones,joints,CNS)-S.typhi,S.paratyphiA,B,C
q gastroenteri%s(salmonellosis)-S.enteri%dis,S.typhimurium,S.agonae
q asymptoma%ccarriers
q onsetusually<6yearsofageq oralinfec%on->contaminatedwater,food(icecream,mayonnaise)
q fever,nauseaq many,plen%fulsmellystools(mucus,pus,blood)
q Theywereusuallyself-limited
q sheddinginfecesisusuallyapprox.3weeks.q an%bio%cspromotesprolongedcarriage
Clostridium difficile q ToxinA-enterotoxinsimilartothetoxinsofV.cholerae
q ToxinB-cytotoxinq Approx.50-70%ofnewborns;20-50%<1r.ż.;approx.3%ofadults-contaminatedwithC.difficile
q SymptomsaTeran%bio%cstherapy:clindamycin,ampicillin,amoxicillin,cephalosporins
q Thebroadspectrumofclinicalsymptoms:◦ milddiarrheawithwatery-brownstools◦ severehemorrhagicdiarrhea◦ pseudomembranouscoli%s
q posi%vebacteriologicalexamina%oncannotbethebasisforthediagnosis!
q goldstandard-cellculture,demonstratethecytotoxicityofC.difficiletoxinAandB
Giardia intesJnalis q themostcommoncauseofparasi%cdiarrhea
q developedcountriesapprox.2-5%ofthepopula%on,developingcountriesapprox.20%
q Itisintheformtrofozoitandcysts
q oralinfec%on->intakeofwaterorproductscontainingcysts
q mileagevaries(asymptoma%ccarriers,acuteself-limiteddiarrhea,chronicdiarrheawithmalabsorp%on
q fecesorduodenalcontentstothepresenceofcystsortrophozoites
q ELISAtestforGiardiaintes%nalisantygen
Laboratory test Specificdiagnos%ctestsimmunosorbentassay(ELISA)orlatexagglu%na%on-detectedinasampleofstoolan%gens:HRVgroupA,adenovirus,rotavirus
Inmostchildrenwithoutimmunodeficiencymicrobiologicaldiagnosisitisnotnecessary-doesnotchangethebasicprinciplesoftreatment
Indica%onsformicrobiologicalexamina%on:◦ Bloodydiarrhea(Salmonella,Shigella,Campylobacter,E.coliO157:H7,Yersiniaenteroli%ca,Clostridiumdifficile-toxinAandB)
◦ Diarrheawithveryseveredehydra%on≥10%orseriouscondi%on◦ Prolongeddiarrhea(>10-14days)◦ Alargenumberofleukocytesinthestool(>5hpf)◦ Immunosuppressioninchild◦ epidemiologicalconsidera%ons◦ Suspectedcholera
Diarrhoea treatment Theresultofdiarrhoea-dehydra%on Lossofwaterandelectrolytesinthefaeces(Na,K,Cl,HCO3)
Thedegreeofdehydra%onisdefinedasapercentageofweightloss
Comparisonofcurrentweightandbeforethediarrhoeaonset
Primarytreatmentofacutediarrheaisoralirriga%on(DPN)orintravenous(dependingontheextentofdehydra%on)andfeeding(earlyrealimenta%on)
GRADE(weighloss)
GENERALCONDITI
ONEYEBALLS TEAR
S
MOUTH AND
TONGUE
THIRST SKINFOLD
Noorsmall(<5%)
Calm, concious
Propertension + Wet Normal Straighte
nupfast
Intermediate
(5-10%)restless
Sunken ,
dark circles- Dry
drinksgreedilythirsty
Straigtensupslowly
Severe(>10%)
Drowsy
or unconsci
ous , limp
Moresunken - Verydry
Drinks
poorly or unable to
drink independ
ently
Straighte
ns up very slowl
Acute diarrhoea treatment Oralfluids-prepara%onswithalowercontentofsodium(60mmol/l)andalowerosmolarity(245mmol/l)
Smec%te(Smecta)-reducesthedura%onofdiarrheaWed.17-24hrs.,noeffectonfaecaloutput
Probio%cs-LactobacillusrhamnosusGG,Saccharomycesboulardishortensthedura%onofacutediarrheaininfantsandyoungchildrenapprox.for24hours.Efficiencyisstrainspecific.Moreefficientinviraldiarrhea(diarrheainthee%ologyHRV)andinearlyuse.
Notrecommended:
• drugsabsorbingbacterialtoxins(ac%vatedcharcoal).• drugsinhibi%ngperistalsisp.p.(Eg.Loperamide)
• an%secre%ondrugs(bismuthsubsalicylate)
• Torehydratenotuseacommerciallyavailableclearfruitjuices,ordrinkslike"cola"
AnJbioJcs in diarrhoea Inmostcases,acuteinfec%ousdiarrhoea(viralandbacterial)resolvesspontaneouslyanddoesnotrequirean%microbialagents
Indicatedan%bio%ctherapyandmodifica%onoftreatmentaTercollec%ngstoolculturewhen:
fever(>380C)andatleastoneofthefollowingsymptoms:
->Bloodydiarrhea
->Largenumberofleukocytesand/orlactoferrininthestool
Chronic diarrhoea- causes Frequentcausesofchronicdiarrhoea◦ post-infec%ousdiarrhoea,toddler’sdiarrhoea,foodallergy,coeliacdisease,cys%cfibrosis,disaccharideintolerance
Rarecausesofchronicdiarrhea◦ diarrhoeachloride,diarrhoeasodium,congenitalatrophyofmicrovilli,epithelialdysplasia,autoimmunediarrhoea
Food allergy Themostcommonallergens
Childhood-90%offoodallergiesarecausedbyproteinsofcow'smilk,eggs,peanuts,wheat
Teens,youngadultsandlaterage-85%peanuts,fish,shellfish
Allergytocow'smilkproteins
Formulafeeding:1,9-4,4%
Breast-feeding:0.5%
Food allergy- causes Diges%vetract◦ Vomi%ng(approx.25-75%)◦ Acutediarrhea◦ Chronicdiarrhea(approx.25-75%)◦ Lossofbodymass◦ Enteropathy-malabsorp%on◦ Irondeficiencyanemia◦ Abdominalpain,abdominalcolic(babies)◦ Coli%s,proctocoli%s◦ gastroenteropathyeosinophilic◦ cons%pa%on
Skin Respiratorysystem
Food allergy DIAGNOSISq Interviewq Oralfodchallenge
• open• blind• double-blind
q skintestsq TotalIgEandspecific
TREATMENT
q Elimina%ondiet
q Proteinhydrolysates(Bebilonpep%,Nutramigen)
q Dietselementary(Bebilonamino,Neocate)
q Soyformula
Lactose intolerance Primarylactosedeficiency–veryrare!Secondarylactasedeficiency• acuteandchronicgastrointes%nalinfec%ons• foodalergy• celiacdisease• an%bio%corchemotherapy• immunedisorders• malnutri%on
Hypolactasiaadulttype◦ InPolandapprox.35%
Coeliac disease Celiacdiseaseisimmune-mediated,causedbygluten,andthegluten-likeprolamins.
This disease affects people with a gene%c predisposi%on, and is characterized by clinicalsymptomsglutenodependant,enteropathyandan%bodiesspecificforcoeliacdisease:
• %ssuetransglutaminasean%bodies–tTGA• endomysialan%bodies–EMA• deamidatedgliadinpep%de–DGP• histocompa%bilityan%gensHLA-DQ2orDQ8
Coeliac disease- classificaJon 1. Theclassicformofthedisease
2. Hiddenrefractoryanemiaandrecurrentshortstaturedelayedpubertyandinfer%lityenamelhypoplasiarecurrentaphthousinmouth
3. Latent4. Thepoten%al(onlyHLA)
Coeliac disease – classical form Arich,smelling,fa{ystools Inhibi%onofweightgainordecrease Growthretarda%on Childsad,apathe%c,irritable Lipoatrophy Alargeprotrudingbelly,thinlegs
Toddler’s diarrhoea Themostcommoncauseofchronicdiarrhoea!
Painlesspassingthreeormoreunformedstoolsforatleast4weeks
Onsetbetween6a36m.ż.
Stoolsonlyduringtheday%me
Nodisturbancesofweightgain(ifsufficientcaloricintake)
Children◦ treatmentisnotnecessary◦ increasingthedoseoffatandreducedamountsofjuices
Parents◦ explainthenatureofthedisease◦ persuadingparentsaboutthebenignnatureofthedisease
ConsJpaJon Defeca%onoccursless1per3daysand/orthebigeffortsisnecceserybecausehard,thoughstoolconsistencyinchildrenaTerinfancy.
Func%onalcons%pa%onaccordingtoRomeIIICriteria:
*≤2stools/week
*≥1episodeoffecalinconinenec/week
*fecalmassesinrectalampulla
*ininterview:reten%onbehaviours,hard,toughstools,largediameterofthestools
*Diagnosis-2ormoresymptomsoccursatleastfor2months
Organic causes of the consJpaJon q Surgical:• Hirschsprungdisease• Anusandrectaldiseases• Acuteabdomen
q Metabological• dehydrata%on• Cys%cfibrosis(meconiumobstruc%on)• Hipothyroidism,adrenalinsufficiency• Hipercalcemia
q neuromuscular• hypotensionabdominalmuscles(Downsyndrome,MPD)• musculardystrophy• spinalinjury(tumors,spinabifida)• Congenitalmuscularlimpness
q ATerdrugsintake• narco%cs• an%depressants• psychotropmedicines• vincris%ne
q Psychiatric:anorexianervosa,depression
q Inolderinfantsexclusivelybreas~ed
ConsJpaJon INTERVIEWq Childageq Dura%on%meofilnessq Frequencyofbowelmovement,stoolsconsistency,effortduringdefeca%on,bloodinstools,fecalincon%nenceq TimeaTerbirthwhenmeconiumappearsq drugsq VitD3supplementa%onq dietq Otherdiseases
Physicalexamina%onq assessmentofnutri%onalstatusq abdominaldistensionq palpa%onassessmentoffaecalimpac%onintheleTandsome%mesrightiliacfossa%meq rectalexamina%on-evalua%onvoltageofsphincter,thepresenceoffecalmassesinarectum
AddiJonal tests q Ultrasoundofabdominalcavityandthyroidq electrolytesq Thyroidhormonesq Sweatchloridetestq Vitamin25OHD3q Anorectalmanometryq enemaq rectoscopyq transit%meq Rectalbiopsy:histologicalexamina%onandhistochemical
Treatment q Coloncleansingfromdefaul%ngfecalmasses
q Drugs:laxa%ves,drugsaffec%nggastrointes%nalmo%lity
q High-fiberdietq Increasedphysicalac%vity
q Psychologicalexamina%on
q Surgicaltreatment
Literature Ø Pediatria,Kawalec,Grenda,PZWL,Warszawa2013
Ø IllustratedtextbookofPediatrics,4thEdi%onØ EuropeanSocietyforPediatricGastroenterology,Hepatology,andNutri%onGuidelinesfor
theDiagnosisofCoeliacDisease.JPGNVolume54,Number1,January2012
Ø DrossmanDA.Thefunc%onalgastrointes%naldisordersandtheRomeIIIprocess.Gastroenterology.2006;130:1377-90.
Ø SummaryandRecommenda%ons:Classifica%onofGastrointes%nalManifesta%onsDuetoImmunologicReac%onstoFoodsinInfantsandYoungChildrenSampson,HughA.;Anderson,JohnA.JPGN,2000,30,S87-S94
Ø Google.com
Ø Slideshare.net