2024-
2025
Hepatologie en
beningne aandoeningen
van de pancreas
PROF DR LALEMAN
JOZEFIEN CEULEMANS
,Lever-, gal-, en pancreasaandoeningen: inleiding + acute leverziekten .............................. 6
Inleiding ................................................................................................................................... 6
Anatomie lever .............................................................................................................................................. 6
Galwegen .................................................................................................................................................. 6
Fysiologie lever .............................................................................................................................................. 6
Synthese.................................................................................................................................................... 7
Afbraak...................................................................................................................................................... 7
Stockeren .................................................................................................................................................. 7
Immunologie ............................................................................................................................................. 7
Functie van gal .......................................................................................................................................... 8
Hoe schade inschatten en evalueren ............................................................................................................ 8
Klinisch onderzoek .................................................................................................................................... 8
Lever-testen .............................................................................................................................................. 8
Afwijkende levertesten ........................................................................................................................... 10
Technische onderzoeken/interventies ................................................................................................... 10
Acute hepatitis ....................................................................................................................... 11
Definitie ....................................................................................................................................................... 11
Kliniek .......................................................................................................................................................... 12
Algemene biochemische kenmerken .......................................................................................................... 12
Etiologie ....................................................................................................................................................... 12
Specifieke vormen ....................................................................................................................................... 12
Infectieus – virale hepatitis ..................................................................................................................... 12
Niet-virale hepatitis ................................................................................................................................ 16
Specifieke vormen/ diagnostiek .................................................................................................................. 17
Toxisch leverlijden .................................................................................................................................. 17
Andere .................................................................................................................................................... 18
Complicatie: acuut leverfalen ...................................................................................................................... 18
Definitie acuut leverfalen ....................................................................................................................... 18
Criteria lever tx ....................................................................................................................................... 19
Chronische leverziekten .................................................................................................. 19
Definitie en relevantie ............................................................................................................ 19
Definitie ....................................................................................................................................................... 19
Relevantie .................................................................................................................................................... 19
Schade/fibrose evalueren ....................................................................................................... 19
Kliniek.................................................................................................................................... 20
Etiologie - DD/........................................................................................................................ 20
Bespreking specifieke vormen ................................................................................................ 20
Infectieuze vormen ...................................................................................................................................... 20
Hepatitis B............................................................................................................................................... 20
Chronische hepatitis D ............................................................................................................................ 22
Chronische hepatitis C ............................................................................................................................ 23
Chronische hepatitis E ............................................................................................................................ 23
Chronisch auto-immune hepatitis ............................................................................................................... 23
Definitie en pathofysiologie .................................................................................................................... 23
Kliniek ..................................................................................................................................................... 23
Diagnose ................................................................................................................................................. 24
Behandeling en prognose ....................................................................................................................... 24
Cholestatische leverziekten .................................................................................................... 24
Vocabularium .............................................................................................................................................. 24
Onstaan ................................................................................................................................................... 25
Primaire biliaire cholangitis ......................................................................................................................... 25
1
, Definitie en pathogenese ........................................................................................................................ 25
Symptomatologie .................................................................................................................................... 26
Epidemiologie en natuurlijk verloop ....................................................................................................... 26
Klinisch beeld .......................................................................................................................................... 26
Diagnose ................................................................................................................................................. 26
Prognose ................................................................................................................................................. 27
Behandeling ............................................................................................................................................ 27
Primaire scleroserende cholangitis ............................................................................................................. 28
Definitie en pathogenese ........................................................................................................................ 28
Kliniek ..................................................................................................................................................... 28
Complicaties............................................................................................................................................ 28
Diagnose ................................................................................................................................................. 29
Behandeling ............................................................................................................................................ 29
IgG4- geassocieerde cholangiopathie .......................................................................................................... 29
Metabole aandoeningen......................................................................................................... 29
Hemochromatose ........................................................................................................................................ 29
Definitie .................................................................................................................................................. 29
Fe metabolisme ...................................................................................................................................... 29
Pathofysiologie ....................................................................................................................................... 30
Epidemiologie ......................................................................................................................................... 30
Klinisch beeld .......................................................................................................................................... 30
Diagnose ................................................................................................................................................. 30
Behandeling ............................................................................................................................................ 31
Screening ................................................................................................................................................ 31
Ziekte van Wilson ........................................................................................................................................ 31
Cu metabolisme ...................................................................................................................................... 31
Diagnose en behandeling ....................................................................................................................... 32
Alfa-1-antitrypsine (A1AT) ........................................................................................................................... 32
Normale rol ............................................................................................................................................. 32
Genotype bepaalt fenotype .................................................................................................................... 32
Diagnose ................................................................................................................................................. 32
Behandeling ............................................................................................................................................ 32
Zeldzame ziekte of weesziekten .................................................................................................................. 33
Porfyrie ................................................................................................................................................... 33
Steatotische leveraandoeningen ............................................................................................. 33
Definitie ....................................................................................................................................................... 33
MASLD ......................................................................................................................................................... 33
Definitie .................................................................................................................................................. 33
Epidemiologie ......................................................................................................................................... 33
Pathogenese ........................................................................................................................................... 33
Kliniek ..................................................................................................................................................... 34
Diagnose ................................................................................................................................................. 34
Behandeling ............................................................................................................................................ 35
ALD: alcohol-associated liver disease .......................................................................................................... 35
Definitie .................................................................................................................................................. 35
Alcohol use disorder: AUD ...................................................................................................................... 36
Epidemiologie ......................................................................................................................................... 36
Pathofysiologie ....................................................................................................................................... 36
Bevorderende factoren schade .............................................................................................................. 37
Klinisch spectrum .................................................................................................................................... 37
Behandeling ............................................................................................................................................ 37
Alcoholische steatohepatitis (ASH) ......................................................................................................... 38
MetALD ........................................................................................................................................................ 39
Specific Etilogie SLD ..................................................................................................................................... 39
Vasculaire aandoeningen van de lever ............................................................................ 40
2
, Ischemische hepatitis ............................................................................................................. 40
Definitie ....................................................................................................................................................... 40
Congestieve hepatopathie ...................................................................................................................... 40
Vena porta thrombose ........................................................................................................... 41
Definitie ....................................................................................................................................................... 41
Etiologie ....................................................................................................................................................... 41
Kliniek .......................................................................................................................................................... 41
Behandeling ................................................................................................................................................. 42
Primair voor trombose ............................................................................................................................ 42
Complicaties............................................................................................................................................ 42
Portale biliopathie ....................................................................................................................................... 42
Syndroom van Budd-Chiari (BCS) ............................................................................................ 42
Definitie ....................................................................................................................................................... 42
Kliniek .......................................................................................................................................................... 42
Oorzaken ..................................................................................................................................................... 43
Pathofysiologie ............................................................................................................................................ 43
Diagnose ...................................................................................................................................................... 43
Behandeling ................................................................................................................................................. 44
Sinusoidaal obstructie syndroom ............................................................................................ 44
Definitie ....................................................................................................................................................... 44
Ziekte van Rendu-Osler-Weber ............................................................................................... 44
Kliniek .......................................................................................................................................................... 45
Lever in HHT ............................................................................................................................................ 45
Behandeling ................................................................................................................................................. 45
Porto-Sinusoidal vascular disorder (PSVD)............................................................................... 45
Cirrose ............................................................................................................................ 46
Definitie en relevantie ............................................................................................................ 46
Pathogenese - Cirrogenese ..................................................................................................... 46
Natural history ....................................................................................................................... 47
Prognose & scores .................................................................................................................. 47
Cirrose “(h)erkennen” ............................................................................................................ 48
Kliniek .......................................................................................................................................................... 48
Technische onderzoeken ............................................................................................................................. 48
Algemene aanpak cirrose ....................................................................................................... 48
Complicaties gerelateerd aan portale hypertensie ................................................................... 49
Gastro-oesofageale varices ..................................................................................................................... 50
Ascites .......................................................................................................................................................... 53
Hepatopulmonaal syndroom en hypertensie .............................................................................................. 56
Complicaties gerelateerd aan leverinsufficiëntie ..................................................................... 56
Hepatische encephalopathie ....................................................................................................................... 56
Klinisch beeld .......................................................................................................................................... 56
Classificatie ............................................................................................................................................. 56
Aanpak .................................................................................................................................................... 58
Behandeling ............................................................................................................................................ 58
Complicatie: Acuut op chronisch leverfalen (ACLF) .................................................................. 59
Pathofysiologie ............................................................................................................................................ 59
Huidig beeld ................................................................................................................................................. 59
3
2025
Hepatologie en
beningne aandoeningen
van de pancreas
PROF DR LALEMAN
JOZEFIEN CEULEMANS
,Lever-, gal-, en pancreasaandoeningen: inleiding + acute leverziekten .............................. 6
Inleiding ................................................................................................................................... 6
Anatomie lever .............................................................................................................................................. 6
Galwegen .................................................................................................................................................. 6
Fysiologie lever .............................................................................................................................................. 6
Synthese.................................................................................................................................................... 7
Afbraak...................................................................................................................................................... 7
Stockeren .................................................................................................................................................. 7
Immunologie ............................................................................................................................................. 7
Functie van gal .......................................................................................................................................... 8
Hoe schade inschatten en evalueren ............................................................................................................ 8
Klinisch onderzoek .................................................................................................................................... 8
Lever-testen .............................................................................................................................................. 8
Afwijkende levertesten ........................................................................................................................... 10
Technische onderzoeken/interventies ................................................................................................... 10
Acute hepatitis ....................................................................................................................... 11
Definitie ....................................................................................................................................................... 11
Kliniek .......................................................................................................................................................... 12
Algemene biochemische kenmerken .......................................................................................................... 12
Etiologie ....................................................................................................................................................... 12
Specifieke vormen ....................................................................................................................................... 12
Infectieus – virale hepatitis ..................................................................................................................... 12
Niet-virale hepatitis ................................................................................................................................ 16
Specifieke vormen/ diagnostiek .................................................................................................................. 17
Toxisch leverlijden .................................................................................................................................. 17
Andere .................................................................................................................................................... 18
Complicatie: acuut leverfalen ...................................................................................................................... 18
Definitie acuut leverfalen ....................................................................................................................... 18
Criteria lever tx ....................................................................................................................................... 19
Chronische leverziekten .................................................................................................. 19
Definitie en relevantie ............................................................................................................ 19
Definitie ....................................................................................................................................................... 19
Relevantie .................................................................................................................................................... 19
Schade/fibrose evalueren ....................................................................................................... 19
Kliniek.................................................................................................................................... 20
Etiologie - DD/........................................................................................................................ 20
Bespreking specifieke vormen ................................................................................................ 20
Infectieuze vormen ...................................................................................................................................... 20
Hepatitis B............................................................................................................................................... 20
Chronische hepatitis D ............................................................................................................................ 22
Chronische hepatitis C ............................................................................................................................ 23
Chronische hepatitis E ............................................................................................................................ 23
Chronisch auto-immune hepatitis ............................................................................................................... 23
Definitie en pathofysiologie .................................................................................................................... 23
Kliniek ..................................................................................................................................................... 23
Diagnose ................................................................................................................................................. 24
Behandeling en prognose ....................................................................................................................... 24
Cholestatische leverziekten .................................................................................................... 24
Vocabularium .............................................................................................................................................. 24
Onstaan ................................................................................................................................................... 25
Primaire biliaire cholangitis ......................................................................................................................... 25
1
, Definitie en pathogenese ........................................................................................................................ 25
Symptomatologie .................................................................................................................................... 26
Epidemiologie en natuurlijk verloop ....................................................................................................... 26
Klinisch beeld .......................................................................................................................................... 26
Diagnose ................................................................................................................................................. 26
Prognose ................................................................................................................................................. 27
Behandeling ............................................................................................................................................ 27
Primaire scleroserende cholangitis ............................................................................................................. 28
Definitie en pathogenese ........................................................................................................................ 28
Kliniek ..................................................................................................................................................... 28
Complicaties............................................................................................................................................ 28
Diagnose ................................................................................................................................................. 29
Behandeling ............................................................................................................................................ 29
IgG4- geassocieerde cholangiopathie .......................................................................................................... 29
Metabole aandoeningen......................................................................................................... 29
Hemochromatose ........................................................................................................................................ 29
Definitie .................................................................................................................................................. 29
Fe metabolisme ...................................................................................................................................... 29
Pathofysiologie ....................................................................................................................................... 30
Epidemiologie ......................................................................................................................................... 30
Klinisch beeld .......................................................................................................................................... 30
Diagnose ................................................................................................................................................. 30
Behandeling ............................................................................................................................................ 31
Screening ................................................................................................................................................ 31
Ziekte van Wilson ........................................................................................................................................ 31
Cu metabolisme ...................................................................................................................................... 31
Diagnose en behandeling ....................................................................................................................... 32
Alfa-1-antitrypsine (A1AT) ........................................................................................................................... 32
Normale rol ............................................................................................................................................. 32
Genotype bepaalt fenotype .................................................................................................................... 32
Diagnose ................................................................................................................................................. 32
Behandeling ............................................................................................................................................ 32
Zeldzame ziekte of weesziekten .................................................................................................................. 33
Porfyrie ................................................................................................................................................... 33
Steatotische leveraandoeningen ............................................................................................. 33
Definitie ....................................................................................................................................................... 33
MASLD ......................................................................................................................................................... 33
Definitie .................................................................................................................................................. 33
Epidemiologie ......................................................................................................................................... 33
Pathogenese ........................................................................................................................................... 33
Kliniek ..................................................................................................................................................... 34
Diagnose ................................................................................................................................................. 34
Behandeling ............................................................................................................................................ 35
ALD: alcohol-associated liver disease .......................................................................................................... 35
Definitie .................................................................................................................................................. 35
Alcohol use disorder: AUD ...................................................................................................................... 36
Epidemiologie ......................................................................................................................................... 36
Pathofysiologie ....................................................................................................................................... 36
Bevorderende factoren schade .............................................................................................................. 37
Klinisch spectrum .................................................................................................................................... 37
Behandeling ............................................................................................................................................ 37
Alcoholische steatohepatitis (ASH) ......................................................................................................... 38
MetALD ........................................................................................................................................................ 39
Specific Etilogie SLD ..................................................................................................................................... 39
Vasculaire aandoeningen van de lever ............................................................................ 40
2
, Ischemische hepatitis ............................................................................................................. 40
Definitie ....................................................................................................................................................... 40
Congestieve hepatopathie ...................................................................................................................... 40
Vena porta thrombose ........................................................................................................... 41
Definitie ....................................................................................................................................................... 41
Etiologie ....................................................................................................................................................... 41
Kliniek .......................................................................................................................................................... 41
Behandeling ................................................................................................................................................. 42
Primair voor trombose ............................................................................................................................ 42
Complicaties............................................................................................................................................ 42
Portale biliopathie ....................................................................................................................................... 42
Syndroom van Budd-Chiari (BCS) ............................................................................................ 42
Definitie ....................................................................................................................................................... 42
Kliniek .......................................................................................................................................................... 42
Oorzaken ..................................................................................................................................................... 43
Pathofysiologie ............................................................................................................................................ 43
Diagnose ...................................................................................................................................................... 43
Behandeling ................................................................................................................................................. 44
Sinusoidaal obstructie syndroom ............................................................................................ 44
Definitie ....................................................................................................................................................... 44
Ziekte van Rendu-Osler-Weber ............................................................................................... 44
Kliniek .......................................................................................................................................................... 45
Lever in HHT ............................................................................................................................................ 45
Behandeling ................................................................................................................................................. 45
Porto-Sinusoidal vascular disorder (PSVD)............................................................................... 45
Cirrose ............................................................................................................................ 46
Definitie en relevantie ............................................................................................................ 46
Pathogenese - Cirrogenese ..................................................................................................... 46
Natural history ....................................................................................................................... 47
Prognose & scores .................................................................................................................. 47
Cirrose “(h)erkennen” ............................................................................................................ 48
Kliniek .......................................................................................................................................................... 48
Technische onderzoeken ............................................................................................................................. 48
Algemene aanpak cirrose ....................................................................................................... 48
Complicaties gerelateerd aan portale hypertensie ................................................................... 49
Gastro-oesofageale varices ..................................................................................................................... 50
Ascites .......................................................................................................................................................... 53
Hepatopulmonaal syndroom en hypertensie .............................................................................................. 56
Complicaties gerelateerd aan leverinsufficiëntie ..................................................................... 56
Hepatische encephalopathie ....................................................................................................................... 56
Klinisch beeld .......................................................................................................................................... 56
Classificatie ............................................................................................................................................. 56
Aanpak .................................................................................................................................................... 58
Behandeling ............................................................................................................................................ 58
Complicatie: Acuut op chronisch leverfalen (ACLF) .................................................................. 59
Pathofysiologie ............................................................................................................................................ 59
Huidig beeld ................................................................................................................................................. 59
3
