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SINDROM DE MALABSORBTIE I. maldigestie 1. boli hepato-biliare 2. boli pancreatice - sinteza, eliminare redusa saruri bililare - sinteza redusa enzime

Malabsorbtie - Curs

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Page 1: Malabsorbtie - Curs

SINDROM DE MALABSORBTIE

I. maldigestie

1. boli hepato-biliare

2. boli pancreatice

- sinteza, eliminare redusa saruri bililare

- sinteza redusa enzime

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3. postgastrectomie

rezervor gastric mic

eliminare rapida de alimente

stimulare isuficienta secretina, CCK

aciditate redusa

supracrestere bacteriana

steatoreeENZIME

ANTIBIOTICE

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SINDROM DE MALABSORBTIE

II. malabsorbtie

1. Enteropatii- enzimatice

- inflamatorii, sprue tropical

- enterita radica

- obstructii limfatice

- enterita eozinofile

3. Sindrom supracrestere bacteriana

2. Suprfata intestinala redusa- rezectii intestinale, etc

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LOCUL DE ABSORBTIE

Jejun

- Fe, Ca, apa, vit. hidrosolubile

Ileon

- Vit B 12, AA, saruri biliare

Colon

- apa, electroliti

- monogliceride, ac grasi, AA

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Vitamina A, D, E, K- vitamina A - esterisicare, absorbtie

Apa, Na

- vitamina D, E, K ?

- absorbtie pe intreg intestinul: paracelular,

transcelularCalciul

- absorbtie activa: 1- 12 dihidrocholecalciferol

Fierul

- absorbtie dependenta de: - aciditatea gastrica

- receptori transferinici - feritina intestinala

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Proteinele

Proteoliza:- di- and tripeptides, AA - stomac, intestin subtire

- pepsina si tripsina

- pepsinogenul – pepsina

activate de pepsina la pH 5

- tripsinogenul – tripsinaactivare prin enterokinaza

-

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Anomalii genetice rare:

1) Deficienta de enterokinaza - absenta enzimei ce converteste tripsinogen la tripsina :

- diaree, intarziere crestere, retardare, hipoproteinemie;

2) Sindrom Hartnup defect in transportul AA neutrii

- rash “pellagra-like” rash and simptome neuropsihice

3) Cystinuria - defect in transportul AA dibazici

- asociat cu litiaza renala sipancreatita cronica

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Deficienta de dizaharidaze

• intoleranta la lactoza - deficit de lactaza, insuficienta transformare a lactozei in glucoza si galactoza

efect osmotic LAPTE

balonare durere abdominala diaree

scaun acid

fermentarea lactozei

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• intoleranta la lactoza

Congenitala - 5-10% caucazieni

80-90% Africa, Asia

Dobandita - enterite ( bacteriene, virale,

parazitare)

- RUH

- fibroza chistica

- abetalipoproteinemia

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Simptome intoleranta la lactoza

1. Cantitatea de lactoza din dieta

2. Rata de evacuare gastrica

3. Timpul de tranzit intestinal

Antibioterapia poate accentua simptomele

restrictia de lapte si derivate reduce simptomele in 70% cazuri

Tratament

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- Malabsorbtia de fructoza – fructoza - absorbita “ marginea in perie” prin proteina transportoare, GLUT 5

- diaree la sorbitol- indulcitor la diabetici

Malabsorptia glucoza-galactoza

- absenta congenitala a SGLT

- diaree la hidrati ce contin glucoza, galactoza, dar nu fructoza

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Lipide

Lipide dieta: 120 – 150 g/zi: AgLL

• faza intraluminala:

- emulsionare, lipoliza trigliceridelor,

stomac – lipaza gastrica la pH 4,5 - 6 jejun proximal – lipaza pancreatica la pH > 7

(colipaza faciliteaza actiunea lipazei)

(lipoliza efeicienta la 5% activitatea maximala a lipazei)

AG, monogliceride, glicerol

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AB primari : acid cholic si chenodeoxycholic - circulatie enterohepatica – reglata de alfa hidroxilaza: degradare colesterol/ sinteza AB

AB secunadri :deoxycholic acid si lithocholic

- 500 mg AB sintetizat in ficat/zi conjugati cu taurina sau glycina

Colon : hidroliza AB conjugati -

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Functiile AB:

1) promoveaza flux bilar

2) solubilizeaza colesterol si phospholipide in bila prin formare de micelii mixte

3) emulsioneaza lipide dietetice – formarea de micelii absorbite in intestinul proximal

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• Steatoree:

• steatoree pancreatica

- pancreatita cronica, fibroza chistica

• gastrinom – inactivare pH acid a lipazei panceratice

• reducerea sintezei si eliminarii AB

- sinteza <AB - ciroza- eliminare <AB - ciroza biliara

• reducerea reabsorbtiei AB - B Chron, rezectii jejun, sclerodermie

• Steatoree:

• steatoree pancreatica

- pancreatita cronica, fibroza chistica

• gastrinom – inactivare pH acid a lipazei panceratice

• reducerea sintezei si eliminarii AB

- sinteza <AB - ciroza- eliminare <AB - ciroza biliara

• reducerea reabsorbtiei AB - B Chron, rezectii jejun, sclerodermie

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SINDROM DE MALABSORBTIE

- deficit ponderal - casexie

- edeme - “tip hipoproteinemic” ascita

- tulburari de crestere

- cheilite, glosite, stomatite ( Vit B2 , B 6)

- eczeme, purpura, hipecheratoza

Manifestari clinice

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SINDROM DE MALABSORBTIE

- sindrom hemoragipar - IP>

- retinopatie - deficit vit A,

- osteoporoza

- anemie

- hTA, retentie azotata

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Diagnostic paraclinic

- Diagnostic malabsorbtie

- Diagnosticul bolii digestive

- examen radiologic

- examen endoscopic, bioptic

- teste biologice

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DIAGNOSTICUL DE MALABSORBTIE

TESTE ABSORBTIE

• absorbtia grasimi

grasimi in scaun < 6-7g/zi

• absorbtie glucide

•- absorbtia D xilozei 25g per os

5 ore excretie urinara > 4,5g/zi

absorbtia vit B12 >8% excretie urinara in 24 ore

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DIAGNOSTICUL DE MALABSORBTIE

TESTE RESPIRATIE

H2 ( 50 g lactoza) H+ CO2

xilozei (C14)

trioleina (C14)

CO2

CO2 3,5% din doza administrata

14

14

14

• absorbtia glucide

• absorbtia lipide

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Absorbtia cobalaminei poate fi anormala:

1. Anemia pernicioasa

2. Pancreatita cronica – defect de desfacere B12 de complaxul ligand R - cobalamina

50%pacientii cu pancreatitia cronica au test Schilling anormal corectat cu enzime pancreatice

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3. Achlorhydria – reduce desfacerea B12 de proteine 1/3 din cei 60 ani au tulburari de absorbtie B12 incapacitatea de a desface cobalamina din alimente ; nu au defect in absorbtia B12 cristalina

4. Sindrom supracrestere bacteriana – consum vit. B12

5. Ileon – ileita terminala

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Test Schilling

Excretie urinara 58Co-labeled cobalamin

1 mg cobalamin administerata im 1 h dupa ingestia de 58Co-cobalamin

-Test Schilling anormal 10% excretie in 24 h

- anemia pernicioasa, pancreatita cronica, SSB, boli ileon

- corectia cu enzime pancretice- corectia cu antibiotice 5 zile

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BIOPSIA DE MUCOAS INTESTINALA

LEZIUNI DIFUZE SPECIFICE

1. Boala Wipple's - macrofages (PAS)-positive in lamina propria, bacili microscopi electronica 2. Abetalipoproteinemia - lipide postprandial, dispar dupa perioada prelungita de post fara grasimi 3. Deficienta de imunoglobuline - plasmocite reduse in lamina propria; mucoasa normala, plata – atrofie viloasa + Giardia lamblia, Giardia trophozoites

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Mucoasa intestinala normala

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LEZIUNI SPECIFICE:

4. Limfom malignan 5. Lymphangiectasia - limfatice dilatate in submucosa,

lamina propria 6.Gastroenterita eosinophilica - infiltarat eosinophilic

in lamina propria, cu sau fara eosinofilie.

5. Boala Crohn6. Amyloidoza intestinala – rosu de Congo7. Diareea persoanelor imunodeficiente (HIV):

Cryptosporidium, Isospora belli, cytomegalovirus, Mycobacterium avium intracellulare, G. lamblia.

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LEZIUNI NESPECIFICE DIFUZE:

- Celiac sprue - Tropical sprue - similar cu sprue celiac dupa

expunere tropicala, subtropicala

- nu raspunde la restrictie de gluten- raspunde la antibiotica si folati

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DIAGNOSTICUL DE MALABSORBTIE

Culturi bacteriene din jejun < 103/ml

III. Determinari serice

Fe, Zn, Mg

IP, albuminemie

vit A

Pierderea intestinala de albumina I125, Cr 51

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ENTEROPATIA GLUTENICA

• intoleranta la gluten

(Sprue celiac)

• malabsorbtie + structura anormala intestinala• anticorpi antigliadinici,

antireticulinici, antiendomisiali

Enteropatii enzimatice

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ENTEROPATIA GLUTENICA

• factor de mediu:

- gluten

(Sprue celiac)

• factor imunologic:

- anticorpi antigliadinici, antireticulinici,

antiendomisiali

- limfocite T sensibilizate la gliadina mediaza injuria tisulara

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ENTEROPATIA GLUTENICA

• factor genetic:

- 95% din pacientii cu sprue celiac - HLA-DQ2 allele

-10% rude de gard I

(Sprue celiac)

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ENTEROPATIA GLUTENICA

gluten

efect toxic epiteliul intestinal

gliadina insuficienta hidroliza

HLA DR8, DQW2

- anticorpi antigliadinici

- posibil adenvirus Ad12

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Date clinice

- balonare -

- diaree - steatoree

- sindrom de malabsorbtie

- in relatie cu alimentatia ce contine grau

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Date paraclinice

- anticorpi antigliadinici

- anticorpi IgA antiendomiosiali

- biopsia de jejun:

- vili <

- cripte >

- infiltrat inflamator submucoasa

- epiteliu cuboid

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Sprue celiac netratat

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Sprue celiac netratat

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Sprue celiac tratat

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Complicatii

- limfoame

- sprue collagenous

Boli asociate

- dermatita herpetiforma

- ulceratii

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Tratament

- DIETA: fara gluten

- corticoterapie - forme severe

- 6 mrcaptopurina - forme rezistente

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• G. lamblia, Yersinia enterocolitica, C. difficile, Cryptosporidium parvum, Cyclospora cayetanensis

• Sprue tropical

• dieree cronica

• mucoasa anormala- nespecifica

• tratament – tetraciclina, folati

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• Sindrom intestin scurt

• asociaza

• absorbtie cerscuta de oxalati, litiaza oxalica• litiaza biliara

• tratament- reducere lipide, lactoza

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SINDROM SUPRACRESTERE BACTERIANA

- Cauze:

- diverticuli 1.Anomalii structurale

- stricturi - enterite inflamatorii

- sindrom ansa aferenta

- fistule gastro - colice, gastro-ileale, etc

2. Anomalii motilitate

- hipotiroidism

- diabet

- vagotomie

3. Diverse- hipogamaglobulinemie

- rezectii gastrice, gastrite anacide, etc

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- deconjugarea saruri biliare, cresterea reabsorbtiei ac biliari liberi

scaderea concentratiei intestinale saruri biliare

- consum vit B12

- tetraciclina, claritromicina, metronidazol, ciprofloxacin

- Supracresterea bacteriana E. coli, Bacteroides,

- Tratament

- steatoree

- anemie macrocitara

- deficit de dizaharidaze

- leziuni epiteliu intestinal

corectie prin antibioeterapie

diagnostic: T

Schilling + antibioeterapie

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ENTERITA RADICA

- vili scurti

- epiteliu megalocitar

- infiltrat inflamator in lamina propria

ENTERITA REGIONALA

- absorbtia sarurilor biliare scazuta

- supracrestere bacteriana

- suprafata de absorbtie scazuta ( rezectii, fistule)

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ENTERITA CU EOZINOFILIE

- “ alergie alimentara”

- eozinofilie, Ig E crescute

- infiltrat eozinofilic in mucoasa

- hipoalbuminemie

- steatoree medie

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ENTEROPATIA PIERDERE PROTEINE

10 - 20% pierdere enterica de proteine

Pierderea enterica de proteine:

1. inflamatii, ulceratii

2. cresterea presunii limfatice

- boli granulomatoase

- boli neoplazice

3. Limfangiectazia intestinala

4. Obstructia limfatica

5.Gastropatia hipertrofica Menetrier

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ENTEROPATIA PIERDERE PROTEINE

- hipoproteinemie, limfopenie

- edeme

- pierdere intestinala – albumine marcate

- alfa1 antitripsina

Page 48: Malabsorbtie - Curs

LIMFANGIECTAZIA INTESTINALA

- hipoproteinemie, hipoalbuminemie

- edeme

- limfocitopenie, Ig scazute

- malabsorbtie cu steatoree

- endoscopie digestiva: efuziuni chiloase in duoden

AP: limfatice jejunale dilatate

Tratament

- grasimi reduse, trigliceride lant mediu

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Limfangiectazia intstinala

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OBSTRUCTIA LIMFATICA

1. Boala Whipple

- Diagnostic

- biopsie intestin

- infiltrat macrofagic in lamina propria + granule PAS pozitive

- actinomicete: Tropherima whippelii

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Boala Wipple

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OBSTRUCTIA LIMFATICA 1. Boala Whipple

- diaree, balonare

- subfebrilitate

- artralgii

- adenomegalie

- hiperpigmentatere cutanata

- manifestari SNC

- Manifestari clince

- sulfametoazol - trimetopin, 1 an

- Tratament

Page 53: Malabsorbtie - Curs

LIMFOM INTESTINAL

- dureri abdominale

- subfebrilitate

- balonare

- infiltrat limfocitar in lamina proproa

- Ig A (lant ) >

- Manifestari clince

- Biopsie intestin

- Biologic

Page 54: Malabsorbtie - Curs

COLONUL IRITABIL

- 10-22%

- 25-50% patologia gastrointestinala

- F/B > 2/1

- cea mai comuna boala gastrointestinala

- Perturbare functionala cu tulburari motorii si secretorii, in afara

anomaliilor structurale, inflamatorii, enzimatice

- Incidenta

- Definitie

Page 55: Malabsorbtie - Curs

COLONUL IRITABIL

- disfunctie psihosociala

tulbuari de personalitate

- Etiopatogenie

- disfunctie sistem nervos autonom digestiv

depresie, atac de panica

somatizare

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COLONUL IRITABIL

- Criterii Manning

>3 luni de simptome: continuee,

recurente

- Diagnostic

- durere, disconfort abdominal indepartat prin defecatie

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- modificari in frecventa si consistenta scaunelor, cu cel putin 2

din urmatoarele in 1/4 din timp:

- > 3 scaune/zi cel putin 3 saptamani

- consistenta moale apoasa

- mucus in scaun

- distensie sau senzatie de distensie abdominala

- perioade de constipatie

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- simptome excluse:

rectoragii, melena

febra

diaree, durere nocturna

pierdere ponderala

- se vor exclude:

intoleranta la lactoza

parazitozele intestinale

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- Examene de laborator:

irigoscopie - normala, colon spastic

colonoscopie - normala

ex. coproparazitologic - negativ

functie tiroidiana - normala- Tratament:

- pacient implicat in decizii terapeutice

- dieta: evita alimente fermentate, cofeina, sorbitol

cu fibre lungi in constipatie

Page 60: Malabsorbtie - Curs

MEGACOLON

Colon gigant cu disfunctie de tranzit si constipatie cronica

Megacolon: cronic

acut

MEGACOLON AGANGLIONIC - boala Hirsprung

- congenital - boala genetica heterogena

- insuficienta migrare a celulelor crestei neurale in colonul distal

- zona aganglionica subdimensionata cu dilatatie supraiacenta

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- Manifestari clinice

- debut in copilarie

- constipatie cronica

- ampula rectala goala

- sfincter anal normal

- ingustare rectosigmidiana, dilatatie supraiacenta

- Irigoscopie

- Biopsie - absenta ganglionilor rectosigmoidieni

- Tratament

- colon normal anastomozat cu rectul

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MEGACOLON DOBANDIT

- mixedem

- amiloidoza

- constipatie

- tact rectal: rect plin cu fecale

- etiologic

- sclerodermie

- narcotice cronic

- boli neurologice

Tratament

Manifestari clinice