curs rahitism+malnutritie
DESCRIPTION
curs pediatrieTRANSCRIPT
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RAHITISMUL CARENTIALRAHITISMUL CARENTIAL
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Ioana Ardeleanu
DefinitieDefinitie
•• Boala metabolica generala Boala metabolica generala caracterizata printrcaracterizata printr--o o tulburare tulburare de de mineralizare mineralizare a a osului osului in in perioada perioada de de crestere rapidacrestere rapida
•• Cauza Cauza –– carenta carenta de de vitamina vitamina DDcare care perturba metabolismul perturba metabolismul fosfofosfo--calciccalcic
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Ioana Ardeleanu
Vitamina Vitamina DD•• Surse Surse
–– transformarea provitamineitransformarea provitaminei D din D din tegument sub tegument sub actiunea razeloractiunea razelor UVUV
–– AlimentaraAlimentara ((ficatficat, , galbenus ougalbenus ou, , pestepeste, , carne carne grasagrasa, , untunt) ) –– nu acopera nevoilenu acopera nevoile de de vitvit D in D in sezon recesezon rece
•• FormeForme–– ergocalciferol ergocalciferol ((vit vit D2)D2)–– colecalciferolcolecalciferol ((vit vit D3)D3)
•• Absorbtie intestinala Absorbtie intestinala in in prezenta prezenta ac.ac.biliaribiliari((liposolubilaliposolubila))-->>hidroxilare hidroxilare hepaticahepatica--> > hidroxilare renalahidroxilare renala-->>metaboliti activi metaboliti activi cu act. cu act. antirahiticaantirahitica
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Ioana Ardeleanu
Factori favorizantiFactori favorizanti
•• Aplicare incorectaAplicare incorecta//incompleta incompleta a a profilaxiei profilaxiei cu cu vit vit DD
•• Insorire insuficienta Insorire insuficienta ((anotimp anotimp recerece))
•• Poluare intensaPoluare intensa•• Alimentatie dezechilibrata Alimentatie dezechilibrata
((exces exces de de fainoasefainoase))•• PrematuritatePrematuritate, , gemelaritategemelaritate
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Ioana Ardeleanu
Factori favorizantiFactori favorizanti
Determina rahitism vitaminoD rezistentDetermina rahitism vitaminoD rezistent•• Sindroame Sindroame de de malabsorbtie malabsorbtie ((fibroza fibroza
chisticachistica, , celiachieceliachie))•• Insuficienta renala cronicaInsuficienta renala cronica•• Atrezie cai biliare extrahepaticeAtrezie cai biliare extrahepatice•• Acidoze renale tubulareAcidoze renale tubulare•• Corticoterapie indelungataCorticoterapie indelungata•• TratamenteTratamente cu cu anticonvulsivanteanticonvulsivante
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Ioana Ardeleanu
Mecanism Mecanism de de actiune actiune vitvit.D.D
•• Intestin Intestin –– Creste absorbtia Creste absorbtia CaCa–– Creste permeabilitatea celulelor Creste permeabilitatea celulelor
intestinale intestinale pt Capt Ca•• RinichiRinichi
–– Creste reabsorbtia tubulara Creste reabsorbtia tubulara Ca, P, Ca, P, aaaa•• Os Os
–– Creste mineralizarea matricei proteice Creste mineralizarea matricei proteice a a osuluiosului
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Ioana Ardeleanu
•• Absorbtia intestinala Absorbtia intestinala a Ca e a Ca e favorizata favorizata dede–– Raport Raport Ca/P=2/1Ca/P=2/1–– Mediu Mediu acidacid–– Prezenta lactozeiPrezenta lactozei–– Prezenta vit Prezenta vit D D
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Ioana Ardeleanu
Carenta Carenta de de vitvit.D.D
•• Scade absorbtia intestinala Scade absorbtia intestinala CaCa-->>hipoCahipoCa-->>creste secretia creste secretia de PTHde PTH-->>ØØcreste absorbtia intestinala creste absorbtia intestinala CaCaØØscade reabsorbtia renala scade reabsorbtia renala PPØØscoatescoate Ca din Ca din osos-->>os rahiticos rahitic ((fara duritate fara duritate –– se se
deformeaza usordeformeaza usor) )
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Ioana Ardeleanu
Manifestari cliniceManifestari clinice
1.1.Semne osoaseSemne osoase–– Leziuni simetriceLeziuni simetrice, , nedureroasenedureroaseCutie Cutie cranianacranianaØØCraniotabes parietoCraniotabes parieto--occipitaloccipitalØØBose frontaleBose frontale//parietale parietale
proeminenteproeminenteØØFA FA largalarga>8 >8 luniluni//deschisa deschisa >18 >18 luniluniØØPlagiocefaliePlagiocefalie//macrocraniemacrocranie
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Ioana Ardeleanu
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Ioana Ardeleanu
ToraceToraceØØMatanii costaleMatanii costale (la (la nivelul jonctiunii nivelul jonctiunii
condrocostalecondrocostale))ØØSant submamar Sant submamar Harrison (Harrison (pe linia pe linia de de
insertieinsertie a a diafragmuluidiafragmului))ØØTorace largit Torace largit la la bazebazeØØStern Stern infundatinfundat//proeminent proeminent MembreMembreØØBratari metafizareBratari metafizareØØGenu valgumGenu valgum//genu varumgenu varumØØFracturi spontane indoloreFracturi spontane indolore
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Ioana Ardeleanu
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Ioana Ardeleanu
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Ioana Ardeleanu
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Ioana Ardeleanu
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Ioana Ardeleanu
•• Coloana vertebralaColoana vertebrala–– Cifoza dorsalaCifoza dorsala//lombaralombara–– Coxa varaCoxa vara
•• Deformarea bazinuluiDeformarea bazinului
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Ioana Ardeleanu
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Ioana Ardeleanu
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Ioana Ardeleanu
Manifestari cliniceManifestari clinice2.2.Semne musculoSemne musculo--ligamentareligamentareØØHipotonie muscularaHipotonie muscularaØØHiperlaxitate ligamentaraHiperlaxitate ligamentara3.3.Manifestari neuroManifestari neuro--muscularemusculareØØHiperexcitabilitate neuroHiperexcitabilitate neuro--musculara musculara
((semn Chwosteksemn Chwostek+)+)ØØStridorStridor//larigospasmlarigospasmØØConvulsii hipocalcemice Convulsii hipocalcemice ((risc risc
imediatimediat//sechelesechele))
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Ioana Ardeleanu
4.4.Semne asociateSemne asociateIntarziere Intarziere in in eruptia dentaraeruptia dentara, , distrofii distrofii
dentaredentareSusceptibilitate crescuta Susceptibilitate crescuta la la infectii infectii
((plamanul rahiticplamanul rahitic –– modificari anatomicemodificari anatomicesisi functionalefunctionale cece agraveazaagraveaza evolutiaevolutiaafectiunilorafectiunilor respiratoriirespiratorii))
Paloare Paloare ((anemie carentiala asociataanemie carentiala asociata))HSMG (HSMG (prin hipotonie muscularaprin hipotonie musculara))Transpiratii abundenteTranspiratii abundente, predominant , predominant
cefalicecefalice
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Ioana Ardeleanu
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Ioana Ardeleanu
Semne radiologiceSemne radiologice
PatognomonicePatognomonice
ØØ Largire metafize oase lungiLargire metafize oase lungi
ØØ Spiculi lateraliSpiculi laterali
ØØDemineralizare osoasa Demineralizare osoasa ((radiotransparenteradiotransparente))
ØØ Intarziere Intarziere in in aparitia nucleilor aparitia nucleilor de de cresterecrestere
ØØ Fracturi Fracturi in in os patologicos patologic
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Ioana Ardeleanu
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Ioana Ardeleanu
ParaclinicParaclinic
•• Faza Faza I I –– Ca Ca scazutscazut,,–– P normal, P normal, –– FA FA normale normale / / usor crescuteusor crescute
•• Faza Faza IIII–– Ca normalCa normal–– P P scazutscazut–– FA FA crescutecrescute
•• Faza Faza IIIIII–– Ca Ca scazutscazut–– P P scazutscazut–– FA FA mult crescutemult crescute
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Ioana Ardeleanu
ProfilaxieProfilaxie
AntenatalAntenatal–– Alimentatie echilibrata Alimentatie echilibrata ((surse naturale surse naturale
de Ca de Ca si vit si vit D)D)
–– Expunere Expunere la la soaresoare
–– Administrare vitAdministrare vit D D mai mai ales in ales in ultimele ultimele luni luni de de sarcina sarcina (500(500--1000U/1000U/zizi, , popo))
!!! CI !!! CI parenteralparenteral
–– Evitare nasteri Evitare nasteri prematurepremature
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Ioana Ardeleanu
ProfilaxieProfilaxie
PostnatalPostnatal
•• Alimentatie naturalaAlimentatie naturala/ / formule formule cu cu vitvit DD
•• Diversificare corectaDiversificare corecta
•• ExpunereExpunere la la aer si soareaer si soare, , exercitii exercitii fizicefizice
•• Locuinte salubreLocuinte salubre, , aerisiteaerisite
•• Cura heliomarinaCura heliomarina>1an>1an
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Ioana Ardeleanu
ProfilaxieProfilaxie
Suplimentarea Suplimentarea cu cu vitamina vitamina DD din din ziua ziua 7 la 18 7 la 18 luni luni zilniczilnic, , apoi apoi in in lunile lunile cu R cu R pana pana la 15la 15--18 18 aniani
•• Doza Doza 500500--1000U/1000U/zizi popo•• Se Se opreste opreste
–– Pe perioada imobilizarii gipsatePe perioada imobilizarii gipsate–– 15 15 zile inaintezile inainte, in , in timpul si timpul si 15 15 zile dupa cura zile dupa cura
heliomarinaheliomarina–– La La initiere tratament initiere tratament cu cu hhhh..tiroidienitiroidieniAport suplimentar Aport suplimentar de Ca de Ca doar doar la la
prematuriprematuri//copii ce primesc copii ce primesc <400 ml <400 ml laptelapte//zizi!!! !!! Forme injectabile doar Forme injectabile doar la la grupe grupe
populationale defavorizatepopulationale defavorizate
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Ioana Ardeleanu
TratamentTratament
ObiectiveObiective::•• PrevenirePrevenire//corectare deformari corectare deformari
osoaseosoase•• PrevenirePrevenire//corectare hipocalcemiecorectare hipocalcemie•• Asigurare crestere si dezvoltare Asigurare crestere si dezvoltare
normelenormele•• Evitarea efectelor Evitarea efectelor adverse ale adverse ale
hipervitaminozeiDhipervitaminozeiD
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Ioana Ardeleanu
TratamentTratament
•• Corectare alimentatieCorectare alimentatie•• Modificare regim Modificare regim de de viataviata•• Vitamina Vitamina D 2000D 2000--4000U/4000U/zizi popo, 6, 6--8 8
saptamanisaptamani•• Ca 500Ca 500--1000mg/1000mg/zizi popo
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Ioana Ardeleanu
Efecte tratamentEfecte tratament
•• Ameliorare semne clinice Ameliorare semne clinice in 2in 2--4 4 saptamanisaptamani
•• Normalizare biochimica Normalizare biochimica in 2in 2--4 4 saptamnisaptamni
•• Ameliorare radiologica Ameliorare radiologica in 3in 3--6 6 saptamani saptamani ((aparitia aparitia ““linieiliniei de de doliudoliu””--de de calcificare distalacalcificare distala))
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Ioana Ardeleanu
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Ioana Ardeleanu
EvolutieEvolutie
•• Vindecare fara secheleVindecare fara sechele/cu defect in /cu defect in remanierea osoasa remanierea osoasa ((incurbare incurbare diafizaradiafizara, , macrocraniemacrocranie, , nanismnanism) ) ––necesita uneori corectie ortopedicanecesita uneori corectie ortopedica
•• Absenta semnelor Absenta semnelor de de normalizare normalizare clinicaclinica, , biochimicabiochimica, , radiologica dupa radiologica dupa 4 4 saptamani saptamani de de tratament corect tratament corect rahitism vitaminoDrahitism vitaminoD--rezistentrezistent
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Ioana Ardeleanu
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Ioana Ardeleanu
Hipervitaminoza Hipervitaminoza DD
•• Dupa practicarea profilaxiei pe scara largaDupa practicarea profilaxiei pe scara larga/ / imbogatire formule lapte imbogatire formule lapte cu cu vit vit DD
Clinic Clinic •• anorexie severaanorexie severa, , rebelarebela//varsaturivarsaturi•• ConstipatieConstipatie•• HipotonieHipotonie•• PaloarePaloare•• PoliuriePoliurie•• PolidipsiePolidipsieComplicatiiComplicatii: : calcificari renalecalcificari renale, , osteosclerozaosteoscleroza
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Ioana Ardeleanu
HipervitaminozaHipervitaminoza DD
ParaclinicParaclinic•• hipercalcemiahipercalcemia•• hipercalciuriehipercalciurie•• proteinurieproteinurie•• retentie azotataretentie azotataTratamentTratament•• oprire administrare vit oprire administrare vit DD•• scadere aport scadere aport CaCa•• perfuzii diureticeperfuzii diuretice•• chelatorichelatori de Cade Ca•• corticoterapiecorticoterapie
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MalnutritiaMalnutritia
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Ioana Ardeleanu
DefinitieDefinitie
•• Tulburare cronica Tulburare cronica de de nutritie nutritie sugar sugar si si copil miccopil mic, , caracterizata prin caracterizata prin incetinireaincetinirea//oprirea cresterii oprirea cresterii pe pe o o perioada mai perioada mai mare de 1 mare de 1 lunaluna, cu o , cu o abatere abatere de 2 DS /deficit>10% de 2 DS /deficit>10% fatafata de de normalul varsteinormalul varstei
•• Cauza majora Cauza majora de de mortalitatemortalitate/ / morbiditatemorbiditate
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Ioana Ardeleanu
CauzeCauze
•• PrimaraPrimara–– Carente alimentare Carente alimentare -- aport alimentar aport alimentar
insuficient cantitativinsuficient cantitativ ((hipogalactiehipogalactie) ) //calitativcalitativ ((diversificarediversificare incorectaincorecta))
•• Secundara Secundara ((conditii patologice ce conditii patologice ce impiedica aportulimpiedica aportul//cresc cresc catabolismulcatabolismul))–– InfectiiInfectii acute acute recurenterecurente//cronicecronice, ,
enteraleenterale//parenteraleparenterale
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Ioana Ardeleanu
CauzeCauze
–– Afectiuni psihosomaticeAfectiuni psihosomatice ((anorexie anorexie nervoasanervoasa, , encefalopatiiencefalopatii cu cu tulburaritulburari de de deglutitiedeglutitie, , boli cronice boli cronice renalerenale, , cardiacecardiace, , sindroamesindroame de de malabsorbtiemalabsorbtie))
–– Carente psihosocialeCarente psihosociale ((deprivare deprivare maternamaterna, , hospitalismhospitalism, , neglijareneglijare, , conditii precareconditii precare de de microclimatmicroclimat))
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Ioana Ardeleanu
Factori favorizantiFactori favorizanti
•• Greutate Greutate mica la mica la nasterenastere•• Varsta Varsta mica de debutmica de debut•• Conditii Conditii sociosocio--economice economice
nefavorabilenefavorabile
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Ioana Ardeleanu
PatogeniePatogenie
•• Aport exogen redusAport exogen redus-->metabolism de >metabolism de inanitieinanitie--consumul glucozei consumul glucozei din din rezerve rezerve de de glicogenglicogen ++economisirea economisirea proteinelor proteinelor ((scade excretia scade excretia de de ureeuree))
•• Dupa epuizare glicogenDupa epuizare glicogen--> > formare formare glucoza glucoza din din aa aa (din (din distructia distructia proteinelor musculareproteinelor musculare) )
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Ioana Ardeleanu
Clasificare clinicaClasificare clinica1.MPC 1.MPC usoara si medie usoara si medie ((grgr I I si si II)II)•• Curba ponderala stationaraCurba ponderala stationara/ /
descrescatoaredescrescatoare in in treptetrepte•• IP=0,9IP=0,9--0,6; IN=0,90,6; IN=0,9--0,70,7•• Continua Continua cresterea staturalacresterea staturala•• Disparitie treptata tesut adipos Disparitie treptata tesut adipos
(abdomen(abdomen--toracetorace--membremembre))•• Scadere progresiva Scadere progresiva tonus musculartonus muscular•• Tegumente palideTegumente palide
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Ioana Ardeleanu
Clasificare clinicaClasificare clinica2.MPC 2.MPC severa severa ((grgr III)III)•• Curba ponderala descrescatore continuuCurba ponderala descrescatore continuu•• IP<0,6; IN<0,7IP<0,6; IN<0,7•• Talie afectataTalie afectata•• Tesut adipos Tesut adipos absent (absent (inclusiv bula Bichatinclusiv bula Bichat) ) ––
facies facies simian, simian, privire privire vievie•• Tegumente Tegumente cu cu tulburari troficetulburari trofice, , zbarcitezbarcite, ,
fese fese in in ““punga punga de de tabactabac””•• Hipotonie musculara Hipotonie musculara –– hiporeactivhiporeactiv, ,
abdomen de abdomen de ““batracianbatracian””
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Ioana Ardeleanu
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Ioana Ardeleanu
Clasificare clinicaClasificare clinica
2.MPC 2.MPC severasevera ((grgr III)III)•• Alterare aparare antiinfectioasaAlterare aparare antiinfectioasa::
–– Receptivitate crescutaReceptivitate crescuta la la infectiiinfectii–– Reactivitate scazutaReactivitate scazuta ((absenta absenta
febreifebrei))•• Scaderea tolerantei Scaderea tolerantei digestivedigestive--> >
diaree ce agraveaza deficituldiaree ce agraveaza deficitul!!! !!! DistroficulDistroficul are are varsta greutatiivarsta greutatii
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Ioana Ardeleanu
Clasificare clinicaClasificare clinica
3.MP (kwashiorkor)3.MP (kwashiorkor)•• Aport proteic scazutAport proteic scazut, , aport aport
caloric normal (caloric normal (lipide si glucidelipide si glucide))•• Edeme hipoproteiceEdeme hipoproteice•• HMG (HMG (steatozasteatoza))•• AnemieAnemie•• Modificari tegumente si fanereModificari tegumente si fanere
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Ioana Ardeleanu
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Ioana Ardeleanu
ProfilaxieProfilaxie
•• Alimentatie naturala Alimentatie naturala 6 6 luniluni//utilizare utilizare formule corespunzatoare varstei si formule corespunzatoare varstei si greutatiigreutatii
•• Diversificare corectaDiversificare corecta•• Imunizari corecteImunizari corecte•• Tratamentul corect Tratamentul corect al al infectiilorinfectiilor•• Asanare conditii necorespunzatoare Asanare conditii necorespunzatoare
de de mediumediu
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Ioana Ardeleanu
Tratament Tratament de de urgentaurgenta
CorectareCorectare: : •• dezechilibredezechilibre HEHE•• Hipoproteinemie severa Hipoproteinemie severa
((albuminaalbumina, plasma), plasma)•• HipoglicemieHipoglicemie•• Anemie severa Anemie severa (ME, (ME, sangesange))Tratamentul infectiilor asociateTratamentul infectiilor asociate
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Ioana Ardeleanu
Tratament recuperatorTratament recuperator
•• Dieta hipercalorica Dieta hipercalorica (200 kcal/kg/(200 kcal/kg/zizi) ) si si hiperproteica hiperproteica (4g/kg/(4g/kg/zizi) cu 35) cu 35--40 kcal 40 kcal la 1g la 1g proteinaproteina
! ! Regim hiperproteic Regim hiperproteic (>6g/kg/(>6g/kg/zizi) :) :–– HiperamoniemieHiperamoniemie–– Crestere uree sanguina Crestere uree sanguina –– suprasolicitare suprasolicitare
renalarenala–– Acidoza metabolicaAcidoza metabolica–– Deshidratare Deshidratare
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Ioana Ardeleanu
TratamentTratament•• Proteine Proteine (8(8--10% din 10% din aport aport caloric) caloric) ––
initial din initial din soia soia / / hidrolizathidrolizat de de cazeinacazeina, , apoi apoi cu cu valoare biologica valoare biologica maremare
•• Lipide Lipide (50% din (50% din aport aport caloric) caloric) –– initial initial preparatepreparate partial partial degresatedegresate, MCT in , MCT in steatoreesteatoree
•• Glucide Glucide (40(40--42% din 42% din aport aport caloric) caloric) ––initial initial monozaharide monozaharide ((glucozaglucoza, , fructozafructoza))
•• Aport optim Aport optim de de vitamine si mineralevitamine si minerale
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Ioana Ardeleanu
Eficienta tratamentuluiEficienta tratamentului
•• Normalizare scaune si tranzit Normalizare scaune si tranzit intestinalintestinal
•• Curba ponderala ascendenta Curba ponderala ascendenta dupa dupa 22--3 3 saptamani saptamani de la de la normalizare scaunenormalizare scaune
•• Ameliorare Ameliorare status status imunologicimunologic