bŦ nered|tary d|sorders of the metabo||sm of sugars and fats

Metabo||sm of sugars

Inborn errors of metabo||sm of ga|actose
Ŵ CalacLosemla (galacLose lncrease ln serum) can be caused by defecLs ln Lhese L Ŵ galacLoseŴ1ŴphosphaLeŴurldylLransferázyţ
urldyldlfosfáLgalakLosaŴ4Ŵeplmerázyţ galacLoklnase
C|ass|c galacLosemla
Ŵ A8 serlous lllnessţ lncldence of 1ť 30 000
Ŵ Cause Ŵ lack of Ga|Ŵ1ŴÞŴur|dy|transferázy Ŵ meLabollzes CalŴ1ŴÞ
Ŵ ÞaLhogenesls Ŵ CalŴ1ŴÞ accumulaLes ln Lhe llverţ kldneys and bralnţ eye lens
alLernaLlve rouLe ls meLabollsed Lo galacLlLolţ whlch ls Loxlc
Ŵ Cllnlcal plcLure Ŵ sympLoms begln beLween 4 and 9Ŧdnem
Ŵ Vom|t|ngţ hepatomega|yţ progress|ve [aund|ceţ |ethargyţ or se|zures
Ŵ 8emlnlscenL of acuLe sepLlc dlsease wlLh hepaLlc and renal fallure
Ŵ uevelops ln unLreaLed cerebral edemaţ ofLen bllaLeral caLaracL
Ŵ lor nnÞP Lhe sympLoms appear only afLer Lransferrlng Lo Lhe mllk dleL
Ŵ ulagnosls Ŵ deLecLlon of lncreased concenLraLlons of urlnary galacLlLol and galŴ1ŴÞ ln Lhe era
Ŵ ls always necessary Lo conflrm Lhe enzymaLlc and molecular level
Ŵ 1herapy Ŵ when Lhey hear Lhe suspecL |mmed|ate|y d|scont|nu|ng m||k d|et
Ŵ When Lhe dlagnosls ls lndlcaLed bezlakLózová llfelong dleL
Ŵ Þrognosls Ŵ may noL be favorable aL Lhe Llme nor recognlzedţ because Lhe chlld has been exposed Lo galacLoseţ lnLrauLerlne
Ŵ MosL ofLen arlse Ŵ speech dlsorder and Lhe emergence of glrls hypergonadoLrofnlho hypogonadlsm

Inborn errors of metabo||sm of fructose

nered|tary fructose |nto|erance
Ŵ A8 serlous dlseaseţ Lhe lncldence of 1ť40 000
Ŵ L lacks frucLoseŴ6ŴÞ ln llver aldolasa
Ŵ lrucLoseŴ6ŴÞ accumulaLes ln Lhe llver and cause compeLlLlve lnhlblLlon of phosphorylase
and prevenLs spllLLlng of glycogen lnLo glucoseţ whlch causes severe hypoglycemla
Ŵ Cllnlcal slgns Ŵ appear shorLly afLer Lhe lnfanLs began Lo be glven sugar
Ŵ SympLoms are vlrLually ldenLlcal wlLh classlcal galacLosaemlaţ [usL mlsslng caLaracL
Ŵ 1olerance LesL frucLose kl Ŵ could resulL ln severe hypoglycaemlaţ shock and deaLh
Ŵ ulagnosls Ŵ deLecLlon muLaLlon A149Þ aldolase 8 gene
Ŵ 1reaLmenL Ŵ CompleLe e||m|nat|on of fructose from the d|et
Ŵ Lven when Lhe prognosls ls uncerLaln adherence Lo dleL

8en|gn fruktosur|e
Ŵ ueflclL frukLoklnázy
Ŵ lrucLose absorbed ln Lhe lnLesLlne can be no organlsm used meLabollcally and excreLed ln Lhe urlne wlLhouL cllnlcal sympLoms

Inborn errors of metabo||sm of g|ycogen Ŵ g|ycogen storage
Ŵ Are due Lo meLabollc dlsorders LhaL resulL ln elLher abnormal sLrucLure or abnormal glycogen conLenL ln Llssues
Clycogen sLorage dlsease Lype 0
Ŵ Aglykogenózaţ lack glykogensynLeLasy enzyme ln Lhe llver (noL muscleţ leukocyLes and enLerocyLes)
Ŵ Llver glycogen ls reduced below 2Ʒ normal
Ŵ Cllnlcal plcLure Ŵ sLocks wlLh severe hypoglycemla cramps Ŵ lead Lo braln damage and menLal reLardaLlon
Ŵ Cccur malnly ln Lhe mornlngţ afLer overnlghL fasLlngţ are accompanled by keLonemla
Ŵ Clucose observed afLer prolonged hyperglycemla and lncreased serum lacLaLe (llver glycogen does noLţ Lhey do lL lacLaLe)
Ŵ urgenL dlagnosls ls essenLlal Lo Lhe survlval of Lhe chlld Ŵ eplsodes of hypoglycemla can be prevenLed by frequenL admlnlsLraLlon of foods
rlch ln proLeln

G|ycogen storage d|sease type Ia
Ŵ von G|erekƌs dlseaseţ hepaLorenal glycogenosls
Ŵ uefecL ln glucoseŴ6ŴphosphaLase (glc6Þ converLs Lo CLC)ţ Lhe A8 gene ls 17ŦchrŦ
Ŵ Cllnlcal plcLure Ŵ sLarLlng ln lnfancyţ progresslve hepaLomegaly and fasLlng hypoglycaemlc convulslons
Ŵ uurlng febrlle condlLlons are more frequenL hypoglycemla and lacLlc acldosls ls accompanled wlLh breaLhlng kussmaulovym
Ŵ CharacLerlsLlc facles Ŵ ƍuoll laceƍ Ŵ dollƌs face
Ŵ 1he body adapLs Lo hypoglycemla Ŵ lnsulln secreLlon decreasesţ lL acLlvaLes llpase ln adlpose Llssue occurs hyperllpoproLelnemla lj lj
lncreased Lhelr breaklng down formed keLone bodlesţ whlch LogeLher wlLh lacLaLe acldosls conLrlbuLe Lo
Ŵ Clucagon does noL lncrease glucose buL lacLaLe
Ŵ Slowlng growLh and puberLy ls behlnd schedule
Ŵ ln adulLhood Ŵ may occur xanLhomasţ renal fallure wlLh hyperLenslon and gouLţ llver adenomas
Ŵ LaboraLory Ŵ fasLlng hypoglycemla (frequenL only ln lnfanLs and Loddlers)
Ŵ PyperlacLaLemla and hyperllpldemlaţ whlch blocks Lhe excreLlon of urlc acld and hyperurlcemla makes
Ŵ Sono Ŵ nefromegalle and hepaLomegalyţ llver adenomas can be
Ŵ Llver blopsy Ŵ Worn sLeaLosls and glycogen
Ŵ 1herapy Ŵ Lhe alm ls Lo prevenL sLaLes of severe hypoglycemla and Mac
Ŵ uleL Lherapy Ŵ frequenL use of dleL wlLh resLrlcLlon of anlmal faLţ lacLoseţ sucrose and frucLose
Ŵ We pay Lhe calorlc needs mosLly sLarches and malLodexLrlns
Ŵ lrom Loddler age are served afLer every mealţ corn sLarch
Ŵ 1he nlghL ls sulLable conLlnuous nasogasLrlc Lube feedlng and Lo 30Ʒ of dally lncome broughL ln Lhe nlghL
Ŵ AcuLe meLabollc dlsrupLlon assoclaLed wlLh lacLlc acldosls durlng lnfecLlon musL be admlnlsLered lnglucose
Ŵ Þrognosls Ŵ ln chlldhood ls goodţ ln adulLs aL rlsk of developlng hepaLlcţ renal and cardlovascular compllcaLlons

G|ycogen Storage D|sease 1ype Ib
Ŵ 1ransporL defecL CLCŴ6ŴÞ
Ŵ ls cllnlcally lndlsLlngulshable from laţ buL frequenL neuLropenla and consequenLly aphLhous sLomaLlLls and ulceraLlon of Lhe lnLesLlnes

G|ycogen Storage D|sease 1ype II
Ŵ Þompe d|seaseţ generallzed glycogenosls
Ŵ Pas Lwo forms Ŵ lla affecLs lnfanLs (enzymopaLhles) llb older chlldren and adulLs (enzymopenle)
Ŵ 1he fallure of lysosomes of llverţ muscle and hearL Ŵ Lhey accumulaLe glycogen
Ŵ Clycogen accumulaLes ln Lhe cyLoplasm of Lhe muscle (lncludlng myocardlum)
Ŵ ÞrenaLal dlagnosls Ŵ perhaps flndlng abnormal lysosomes ln amnlocyLes
Ŵ A classlcal form of dlsease ls always faLal Ŵ lL ls Lhe slze afLer blrLh hearL border
Ŵ uurlng Lhe weeks and monLhs Lo become fully hypoLonlc Ŵ weak sucklngţ breaLhlng superflclally
Ŵ Þronounced cardlomegalyţ LCC aL hlgh Þţ ÞC and shorLened gear fallure
Ŵ SllghLly enlarged llver
Ŵ Consclousness ls noL vlolaLedţ nor lnLellecL
Ŵ lrequenL asplraLlon pneumonla wlLh aLelecLaslsţ around 2 years of deaLh from resplraLory fallure
Ŵ LffecLlve Lherapy ls noL avallable
Ŵ AdulL forms Ŵ muscle weakness occurs laLer
Ŵ SomeLlmes does noL shorLen llfe expecLancyţ sedenLary occupaLlon allows
Ŵ CLhers dle around 30 and 40 year
Ŵ ls less cardlomegalyţ normal LCCţ arrhyLhmla ofLen
Ŵ ulagnosls Ŵ blopsy of Lhe skln Ŵ under an elecLron mlcroscope cerLlflcaLe abnormal lysosomes

G|ycogen storage d|sease type III
Ŵ Cor| dlseaseţ lorbes dlsease
Ŵ A8ţ lmpalred glycogen branchlng enzyme ellmlnaLlng Lhe
Ŵ 8areţ a slmllar plcLure of a CSu lţ mllder

G|ycogen storage d|sease type IV
Ŵ Andersen dlsease
Ŵ A rare A8ţ has reporLed abouL 10 casesţ branchlng enzyme dlsorder
Ŵ Clycogen ln hepaLocyLes ls a long chaln wlLhouL branchlng (amylopecLln)

Inborn errors of metabo||sm Mucopo|ysacchar|de
Ŵ 1hese dlsorders can be classlfled lnLo groups of lysosomal dlsorders
Ŵ Þlay an especlally lmporLanL role Lhese mucopolysaccharldes Ŵ heparansulfáLţ and keraLansulfáL dermaLansulfáL
Ŵ 8ecause Lhey are a parL of mucopolysaccharldes blnders are characLerlzed by bone changes (dysosLosls mulLlplex)ţ ls affecLed CnS (menLal
reLardaLlon)ţ as well as kvSţ llverţ spleenţ sklnţ [olnLs and Lendons
Ŵ Are A8 (wlLh PunLer syndrome (MÞS ll) ls Lhe x8
Ŵ ulagnosls of MÞS ls based on Lhe phenoLype of Lhe chlldţ ldenLlLy CAC excreLlon ln Lhe urlneţ xŴray flndlng dysosLoslsţ enzymaLlc
examlnaLlons
Ŵ Þrognosls ls generally favorableţ many dylng ln preschool or school age
Ŵ ÞaLlenLs wlLh mllder forms may llve Lo adulLhood
Ŵ 1herapy Ŵ excepL for MÞS l ls a sympLomaLlc

nur|er syndrome (MÞS I)
Ŵ AccumulaLlon of dermaLan sulfaLe and keraLan sulfaLe ln Llssues and excreLlon ln Lhe urlne
Ŵ lL ls Lhe mosL severe MÞS
Ŵ uurlng Lhe flrsL year Ŵ usually flnd only delayed psychomoLor developmenLţ we can reveal hepaLosplenomegalyţ kyphosls accenLţ raLLllng
breaLhlng
Ŵ AfLer 1 ln developlng Lhe sympLoms Ŵ Lhere ls a Lyplcal face Ŵ gargololdnl (remlnds gargoyles)ţ macrocephalyţ promlnenL forehead wlLh
dollchocefalleţ Lhe nose ls broad and flaL wlLh a deep rooLţ macroglossla
Ŵ 1here are corneal opaclLlesţ psychomoLor speeds up Lo second yearţ Lhe paLlenL ceases Lo be moblle
Ŵ ulsablllLy valvesţ kMÞ lnsuffŦ
Ŵ ueaLh usually ln adolescence
Ŵ 1herapy Ŵ can be LreaLed by bone marrow LransplanLaLlon

Congen|ta| d|sorders of ||p|d metabo||sm

D|sorders of ĒŴox|dat|on of fatty ac|ds
Ŵ 8eLaŴoxldaLlon plays an lmporLanL role ln ensurlng Lhe energy needs ln perlods of fasLlng
Ŵ lL ls Lhe dlrecL source of energy for hearL and muscle Llssue and Lhe source of keLone bodles for CnS
Ŵ ls abouL 20 known dlsorders are A8 Ŵ Lhe mosL common one Ŵ MCAu (MedlumŴchaln acylŴCoA dehydrogenaseŴ) and LCPAu (LongŴchaln 3Ŵ
CPŴacylŴCoA dehydrogenase)
Ŵ Cllnlcal plcLure Ŵ an early chlldhood manlfesL aLLacks of 8eyeŴllke syndrome Ŵ an acuLe dlsLurbance of consclousnessţ selzuresţ
hepaLomegalyţ or SluS
Ŵ 8uL may begln laLer ln adulLhoodţ chronlc muscle weakness or kMÞ
Ŵ LaboraLory LesLs Ŵ ln Lhe acuLe condlLlon ls ofLen hypokeLoacldoLlcká hypoglycemlaţ mlld hypoamonnemleţ lncreased amlnoLransferase
Ŵ ln Lhe blood are decreased levels of free and LoLal carnlLlne
Ŵ lor Lhe MCAu dlcarboxyllc acldurla
Ŵ lor LCPAu ls a 3ŴCPŴdlcarboxyllc acldurla
Ŵ lncreased echogenlclLy of llver sLeaLosls
Ŵ lor LCPAu ls a load and fever ln Ck and urlne myoglobln
Ŵ ulagnosls Ŵ based on an examlnaLlon of Lhe parameLers of beLaŴoxldaLlon ln lsolaLed lymphocyLes ln comblnaLlon wlLh enzymaLlc and
molecular meLhods
Ŵ 1herapy Ŵ ls dlfferenL from fallureţ Lo prevenL sLarvaLlon ln a chlld
Ŵ lrequenL dleL wlLh resLrlcLlon of faL and one or Lwo porLlons of food aL nlghL
Ŵ lor MCAu Ŵ enrlch Lhe malLodexLrlns and sLarches
Ŵ lor LCPAu Ŵ nuLrlLlon wlLh faL resLrlcLlonţ enrlch M1C oll (whlch ls noL buL glve Lhe MCAu)
Ŵ WlLh fever Ŵ a hlgher lnLake of sweeLened beveragesţ and Llmely admlnlsLraLlon of CLC
Ŵ 1he lmporLance of carnlLlne ls dlspuLed
Ŵ Þrognosls Ŵ chlldren wlLh unrecognlzed syndrome ofLen dle ln lnfancy under Lhe plcLure of SluS or 8eyeŴllke syndrome
Ŵ LCPAu may laLer compllcaLe Lhe emergence of reLlnlLls plgmenLosa and kMÞ
Ŵ ÞaLlenLs ldenLlfled by early dlagnosls and dleLary LreaLmenL have longŴLerm cllnlcal problemsţ buL Lhe quesLlon of longŴLerm survlval sLlll
remalns open

D|sorders of p|asma ||poprote|ns
Ŵ Cne of Lhe mosL common meLabollc dlsorders
Ŵ SlgnlflcanLly lncrease Lhe rlsk of developlng Cv dlsease wlLh premaLure occurrence of A1 and lM
Ŵ Culdance for consulLaLlon we wlll deLermlne Chol and 1AC
Ŵ lor more accuraLe dlagnosls ls necessary Lo collaboraLe wlLh speclallzed deparLmenLs

nypo||poprote|nóm|e
Abeta||poprote|nem|a
Ŵ 8areţ A8ţ compleLely lacklng llpoproLeln parLlcles conLalnlng Apo8 (chylomlkraţ vLuL)
Ŵ 1oLal Chol and 1AC levels are low
Ŵ PeLerozygoLes are easlly
Ŵ PomozygoLes Ŵ ln lnfancy Ŵ faL malabsorpLlonţ sLeaLorrheaţ reLarded growLh
Ŵ LaLer Lhere ls reLlnlLls plgmenLosaţ and cerebellar aLaxla
Ŵ ls Lyplcal akanLocyLóza (a sLrange hornllke era)
Ŵ ueflclency of faLŴsoluble vlLamlnsţ lmpalred corLlsol
Ŵ Llplds accumulaLe ln Lhe lnLesLlnal eplLhellum Ŵ vakuoallzace
Ŵ 8ody lacklng essenLlal Mk (llnolelc)
Ŵ 1herapy Ŵ admlnlsLraLlon of 1AC wlLh medlumŴchaln (MC1)ţ whlch are absorbed from Lhe lnLesLlne dlrecLly lnLo Lhe porLal clrculaLlon and
vlLŦ ln faLs

Ana|fa||poprote|nóm|e (1ang|erská d|sease)
Ŵ 8educed levels of PuL and ApoAŴlţ and lower LuL and LoLal Cholţ PuL noL only are ApoCll lj vLuL
Ŵ Cllnlcal plcLure Ŵ lL occurs ln lnfancyţ condlLlonal on Chol esLer accumulaLlon ln Llssues
Ŵ Lnlarged yellowlsh Lonsllsţ hepaLosplenomegalyţ and lnfllLraLlon of Lhe cornea
Ŵ Plgher lncldence of A1

nyper||poprote|naem|a
lamlllal comblned hyperllpoproLelnemla
Ŵ lrequenLlyţ 1ť200
Ŵ ApproxlmaLely 1 / 3 of Lhe affecLed famlly members reporLed hlgher Cholţ 1 / 3 of 1AC and 1 / 3 boLh
Ŵ LLlopaLhogenesls Ŵ plays a role ln lncreased formaLlon of vLuL
Ŵ 1herapy Ŵ sLrlcL dleLţ focuslng malnly on Lhe measuremenL of cholesLerol
Ŵ lor chlldren wlLh hyper1AC we musL reduce lnLake of sweeLs

nyperapobeta||poprote|nóm|e
Ŵ lncrease ln Apo8 componenLs Ŵ LuL
Ŵ Levels of LoLal Chol and 1AC are normal

Iam|||a| hypercho|estero|em|a
Ŵ lallure of Lhe sLrucLure or fce LuL recepLor
Ŵ Au prevalence ls 1ť300
Ŵ Plgher lncldence for Arabs and !ewsţ were lower ln blacks
Ŵ 1ypes of dlsorders LuL8 Ŵ mosLly mlsslngţ or ls slowly LransporLed from LC Lo CAţ or recepLors ls enoughţ buL do noL make LuLţ or blnd buL
can noL be zanoilLţ and also glven Apo83300
Ŵ LuL ls noL absorbedţ accumulaLes ln Lhe body
Ŵ 1he maln resulL Ŵ early developmenL of coronary arLery A1
Ŵ 1he flrsL aLLack follows Lhe dlsabled men ls around 40 year Lo Lhe 60Lh ls affecLed by 83Ʒ (women abouL 10 more)
Ŵ 1endon xanLhomas (nodular enlargemenL of Lendons) Ŵ can occur as early as puberLy
Ŵ ueposlLs ln sofL Llssues (xanLhelesmaLa) Ŵ such as Lhe eyellds or cornea (arcus corneae) ln adulLhood
Ŵ 1oLal Chol ls above 6Ŧ3 mmol / l
Ŵ A sLrlcL dleL wlll reduce Cholţ buL usually normallze
Ŵ lor chlldren over 6 years served cholecysLamln (resln) Ŵ dose noL depend on age buL on Lhe degree of cholesLerolemlaţ ls well LoleraLed
even aL hlgh doses
Ŵ Can cause consLlpaLlonţ someLlmes vlLamln deflclenclesŦ soluble ln faLs
Ŵ CLher llpld lowerlng ls only sulLable for Lhe LreaLmenL of adulLs
Ŵ ln homozygous formţ Lhe prognosls ls poor Ŵ lM a few years of llfe
Iam|||a| hypera|fa||poprote|nóm|e
Ŵ Plgh PuL syndrome assoclaLed wlLh longevlLy

Inborn errors of metabo||sm of t|ssue ||p|ds Ŵ ||p|dos|s
Ŵ 1he resulL of lncompleLe enzymaLlc equlpmenL lysosomes
Ŵ usually accumulaLe glycosphlngollplds
Ŵ lf Lhey accumulaLe ouLslde Lhe CnSţ occurs malnly hepaLosplenomegaly (Caucher)
Ŵ 8uL lf Lhey accumulaLe only ln Lhe CnSţ no hepaLosplenomegalyţ and Lhe lmage remlnds degeneraLlve braln dlseases (1ayŴSachs)
Ŵ When Lhey accumulaLe ln Lhe CnS and perlphery Ŵ boLh (nelmannŴÞlck)
Ŵ 1reaLmenL of Lhese dlseases ls unsuccessfulţ poor prognosls

Gaucher d|sease
Ŵ 8educed acLlvlLy of ĒŴglucocerebrosldaseţ Lhere ls a charglng lysosomal glucosylceramlde ln cells of orlgln makrofágoveho
Ŵ A8
Ŵ Cllnlcal plcLure Ŵ accordlng Lo Lhe course ls dlvlded lnLo Lhree Lypes
Ŵ nonŴneuronopaLhlc Lype l Ŵ Lhe mosL commonţ progresslve splenomegalyţ hepaLomegalyţ moderaLeţ paln ln Lhe long bonesţ speeches
splenlsmu wlLh anemlaţ leukopenla and LhrombocyLopenla wlLh bleedlng manlfesLaLlons ln Lhe skln and subcuLaneous Llssue
Ŵ AcuLe neuronopaLhlc Lype ll Ŵ a speech ln lnfanLs Ŵ fallure Lo Lhrlveţ sLrablsmus or nysLagmusţ progresslve hepaLosplenomegalyţ CnS
lnvolvemenL wlLh Lhe arresL of psychomoLor developmenL and oplsLhoLonus
Ŵ SubacuLe neuronopaLhlc Lype lll Ŵ beglns ln Loddlers and preschoolţ affecLlng Lhe CnSţ buL ls slower Lhan Lype ll
Ŵ LaboraLory Ŵ dlfferenL manlfesLaLlons of pancyLopenla
Ŵ lncreased acLlvlLy chlLoLrlosodázyţ acld phosphaLase and amlnoLranferáz
Ŵ ln Lhe bone marrow are Caucher cellsŦ
Ŵ 1he skeleLal dlsablllLles Ŵ paLhologlc fracLuresţ verLebral compresslonţ expanslon of dlsLŦ ends of Lhe femurs and Llbla
Ŵ 1he presence of osLeonecrosls sclnLl
Ŵ 1herapy Ŵ LreaLmenL of cholce for Lype l and lll ls prepared recomblnanL enzyme Ŵ Ceresyme

N|emannŴÞ|ck d|sease
Ŵ A heLerogeneous group of dlseases leadlng Lo excesslve charglng lyzosomálnlmu sflngomyellnuţ A8
Ŵ Cllnlcal plcLure Ŵ already ln lnfanLs Ŵ fallure Lo Lhrlveţ hepaLosplenomegaly and lymphadenopaLhyţ ln Lhe flrsL weeks of prolonged [aundlceţ
gradually progresslve neurodegeneraLlve dlsorders Ŵ psychomoLor reLardaLlonţ spasLlc kvadrupareza
Ŵ ulagnosls Ŵ charglng sfyngomyellnu hlsLopaLhologlcal flndlngs and oLher llplds ln cells penovlLychŦ ln Lhe marrow (nlemannŴÞlck cellsŦ)
Ŵ lrequenL ls Lhe presence of a cherry sLaln Lhe reLlna
Ŵ 1herapy Ŵ bone marrow LransplanLaLlon (only for Lype 8ţ vlsceral moderaLe dlfflculLy)








cŦ Loss of appet|te

Ŵ Causes Ŵ organ|c and |norgan|c
lnfecLlonţ nSZ
Cncologyţ
8raln 1umors
MeLabollc defecLs (aclds fall lnLo cenLers for vomlLlngţ whlch ls ouLslde Lhe PL8)
Anorexla nervosaţ bullmla
8umlnaLlonsţ
plcaţ
8egurglLaLlon

Nonorgan|c |oss of appet|te
Ŵ 1hlnk of Lhe psychologlcal orlgln Lo Lhe excluslon of organlc dlsorders
Ŵ MosL common ln Loddlers and preschool age
Ŵ SCS vlllages and orphanages only very rarely Ŵ lLƌs a quesLlon of Lhe relaLlonshlp of parenLs Lo Lhe chlld
Ŵ ?ou need Lo make parenLs undersLand Lhelr own aLLlLudesť
Ŵ 1he amounL of food Lhe chlld has recelved a greaL psychologlcal lmporLance for parenLs
Ŵ lL ls proof of lLs vlLallLyţ healLh
Ŵ lL ls conflrmaLlon of Lhe success of parenLs as educaLors
Ŵ lL ls for Lhem a source of deep saLlsfacLlon
Ŵ Cn Lhe conLraryţ lf a chlld refuses food
Ŵ leellngs of frusLraLlonţ lnsecurlLyţ anxleLyţ frusLraLlon wlLh parenLs lj parenLs Lhen Lend Lo use ƍvlolenceƍ agalnsL Lhe chlldţ requlre
medlcal lnLervenLlon
Ŵ Such behavlor leads Lo a chlld even sLronger flxaLlon re[ecLlng aLLlLudes
Ŵ 1he chlld LhaL Lhelr behavlor ln facL domlnaLes Lhe whole famlly ls aL Lhe cenLer of aLLenLlon
Ŵ lf Lhe parenLs accepL Lhls lnLerpreLaLlonţ and Lake furLher advlce and can be furLher addressed
Ŵ noL all of lL can have dlfferenL subconsclous defense mechanlsms
Ŵ We lead Lhe parenLs Lo domlnaLe Lhelr efforLs Lo nurLure a chlld accordlng Lo Lhelr wlshes
Ŵ 1herapy Ŵ food should be LasLyţ [ulcyţ wellŴdecoraLed
Ŵ We do noL compleLely conform Lo Lhe LasLes of Lhe chlldţ buL noL Lo worry hlm very much Lo meeL
Ŵ Cn a plaLe of food Lo be so much a chlld can really eaL
Ŵ 8efore Lhe chlld can sLlll remove a small porLlon of food
LaLlng dlsordersť anorexlaţ anorexlaţ blnge eaLlngţ vomlLlngţ mornlng

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