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486
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N. V. Bergasa
https://t.me/medicina_free
487© The Author(s), under exclusive license to Springer-Verlag London Ltd., part
of Springer Nature 2022
N. V. Bergasa (ed.), Clinical Cases in Hepatology,
https://doi.org/10.1007/978-1-4471-4715-2
A
Abdominal pain, 7
Aceruloplasminemia, 361
Acetaminophen, 435
Acute liver failure, 377, 378
Adjuvant therapy, 148
Afferent and efferent nerve bers, 3
Alcohol abuse, 11
Alcohol induced liver disease, 255
alcohol withdrawal, 280
alcoholic hepatitis, treatment for, 281–284
associated conditions, 266–271
clinical manifestations of, 265, 266
delirium tremens (DTs), 281
epidemiology of, 260, 261
etiology of, 256
genetics of, 256–259
histology, 274–276, 278
laboratory and radiological
examinations, 272–274
natural history of, 261–265
treatment for, 278–280
Alcohol withdrawal, 280
Alcoholic cirrhosis, 276
Alcoholic hepatitis, 275, 276
treatment for, 281–284
Alcoholic neuropathy, 268
Alfa fetoprotein (AFP), 401
Alfapump® transfers ascites, 460
All-trans retinoic acid (ATRA), 145
Alpha 1 antitrypsin (A1AT) deciency, 325,
326, 328
associated conditions, 335, 336
clinical manifestations of, 333–334
characterization, 326
epidemiology, 330, 331
genetics, 328–330
hepatocellular carcinoma, 335
histology of, 334, 335
laboratory tests, 334
liver transplantation in, 337
natural history of, 331–333
plasma concentration, 328
polymerization, 327
treatment, 336
Alpha interferon therapy, 182
Amoxicillin-clavulanate induced DILI, 414
Angiotensin converting enzyme (ACE)
system, 103
Anion exchanger 2 (AE2), 48, 49
Antigen presenting cells (APCs), 46
Antinuclear antibodies (ANA), 43, 44
Antituberculous therapy, 414
Anti-tumor necrosis factor-alpha
rituximab, 111
Apoceruloplasmin, 373
Apoptosis, 314
Apotransferrin, 343
Ascites, 17
Alfapump® transfers ascites, 460
capillarization, 456
characterization of, 456
complication of, 455
hepatic sinusoids, 455, 456
management of, 458, 459
NSAIDs, 460
pathogenesis of, 456
pathophysiology, 455
refractory ascites, 459
serum albumin ascites gradient, 457
Index
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488
Ascites (cont.)
sinusoidal hypertension, 456
splanchnic vasodilatation, 455
survival of, 455
TIPS shunts, 459, 460
Aspartate transaminase to platelets ratio index
(APRI), 21
Asterixis, 20
ATP-binding cassette (ABC) transporters, 137
Autoantibodies, 93–94
Autoimmune hepatitis (AIH)
absolute indications, 102
autoantibodies, 93–94
characterization, 98
in children, 112, 113
clinical cases, 85, 86
clinical manifestations, 91, 92
denition, 86
diagnostic criteria, 96, 99, 100
epigenetics, 91
follow up period, 98
genetics, 90, 91
hepatocellular carcinoma, 111
histology, 86, 94–96, 98
history, 97
interpretation of scores, 99
laboratory ndings, 97
liver transplantation, 113, 114
lymphocytes inltrates, 95
overlap syndromes, 101, 102
pathogenetics, 87
CD4+CD25+FOXP3+ regulatory T
cells (Treg), 88
chemokines, 89
environmental factors, 89
FOXP3, expression of, 87
hypergammaglobulinemia, 87
mediate immune tolerance, 87
molecular mimicry, 88, 89
M2BPGi, serum levels of, 87
natural killer (NK) cells, 88
regulatory T cells (Tregs), 87, 88
serum CD4+ CD 25+ Tregs cells, 87
T cell immunoglobulin mucin-3, 88
physical examination, 92, 97
plasma cells, 95
relative indications, 102
scoring system, 99
treatment
adrenal suppression, 105
anti-CD20 rituximab, 111
anti-tumor necrosis factor-alpha
rituximab, 111
budesonide, 108, 109
calcineurin inhibitors, 109
combination therapy, 104, 105
continuation of therapy, 104
with corticosteroids, 103
corticosteroids therapy, 104, 106
cyclosporin, 109
discontinuation of therapy, 107, 108
with brosis, 103
galectins, 103
grade 3 encephalopathy, 111
hypoxanthine phosphoribosyl
transferase, 107
immunosuppression therapy, 107
maintenance therapy, 106
MELD score, 111
mTOR inhibitor sirolimus, 111
mycophenolate mofetil, 110
with prednisone, 104
serum transaminase activity, 106
standard therapy, 110
tacrolimus, 109
thiopurine methyltransferase, 107
with and without cirrhosis, 103
xanthine oxidase, 107
type 1, 93
type 2, 93
Autonomic nervous system, 3
Autophagy, 48
Autoreactive T cells, 45
B
B cells and plasma cells, 46
Baclofen, 279
Bacterial cholangitis, 146
Bacterial infection, 264, 461
Ballooned hepatocytes, 275
Balloon-occluded retrograde transvenous
obliteration (BRTO), 455
Bariatric surgery, 315, 316
Basedowiana-Haban cirrhosis, 12
Bile salt export protein (BSEP), 421
Biliary epithelial cell (BECS), 3, 29, 46, 47
apoptotic, 49
glutathionylation, 50
senescence, 48, 49
Bone disease, 268
Bone morphogenic proteins (BMP)
receptors, 344
Branched-chain 2 oxoacid dehydrogenase
complex (BCOADC), 28
Bronze diabetes, 350
BTT1023 (Timolumab), 146
Buttery sign, 14
Index
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489
C
Caput medusa, 17
Cardiomyopathy, 467
Cardiovascular disease, 308
C-C receptor 7 programmed cell death 1
(CCR7 PD-1) chemokine, 89
CD4(+)CD25(+) regulatory T-cell activity, 209
chronic hepatitis C virus, 168, 170
CD4+ T cells, 209, 210
chronic hepatitis C virus, 168
CD4+CD25+FOXP3+ regulatory T cells
(Treg), 88
CD4+CD25 high regulatory T cells (Tregs), 45
CD56
+
CD3
–
NK cells, 208
CD56
+
CD3
+
NT cells, 208
CD8+ T cells, 209, 210
chronic hepatitis C virus, 168–170
cDNA library, 134
Cellular senescence, 135
Cenicriviroc, 146, 314
Ceruloplasmin deciency, 343
Chelators, 359
Chemokines, 89
Cholangiocarcinoma (CCA), 141–143,
148, 391
Choledocholithiasis, 147
Cholelithiasis, 37, 61, 147, 473
Cholestasis, 37, 131
complication, 443
denition, 443
Cholestatic liver injury, 432
Cholestyramine, 436, 444, 445
Chronic hepatitis B virus (C-HBV)
budding process, 205
cccDNA formation, 205
characteristics of, 214, 216–219
circulating viral particles, 203, 204
clinical cases, 203
clinical manifestations, 213
coinfection with HIV, HCV, AND delta
hepatitis virion, 226
core antigen, 204
differential diagnosis, 221
direct repeats, 204
epidemiology, 212
ESCRT system, 205
extrahepatic manifestations, 213, 214
genetics
CD4+ T cells, 209, 210
CD4+CD25+ regulatory T cells, 209
CD56
+
CD3
–
NK cells, 208
CD56
+
CD3
+
NT cells, 208
CD8+ T cells, 209, 210
chromosome 21q22, 212
in cytokines, 211
ex-vivo studies, 208
genotype and genomic mutations, 211
GWAS methodology, 211
HLA-DPA1 and HLA-DPB1
genes, 212
host’s MHC II composition, 211
host’s immune system and
characteristics, 206
IFN-AR2, 212
IFN-λ1, 206
IL-10RB, 212
in vitro studies, 206, 208
increased TNF- alpha production, 208
IRF3, 207
Mannose binding lectin, 212
natural killer (NK) cell activation,
206, 207
RIG-I functions, 207
TLR-2 pathway, 208
TNF-alpha, 211
tumor necrosis factor-related apoptosis-
inducing ligand, 207
type I IFN receptor gene, 212
with chronicity, 212
genomic DNA synthesis, 205
hepatocellular carcinoma, 240–242
identication of, 203
laboratory ndings and serology, 220
liver histology, 221, 222, 226
liver transplantation, 239, 240
natural history, 222–225
NTCP, 204
open reading frames, 204
pegylated interferon, 232
phases, 215
Pol-linked terminal redundant
sequence, 205
relaxed circular, 204
replication, 205
robust immunological response, 204
treatment, 229
active investigation area, 238
anti core with or without detected HBV
DNA, 229
antiviral medications, 229
antiviral therapy, 234
combination therapy, 234, 236, 237
discontinuation of therapy, 237
entecavir, 234
indications for, 228, 230, 231
interferon, 229
interferon alpha, 231
lamivudine, 233, 234, 236
Index
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490
Chronic hepatitis B virus (C-HBV) (cont.)
lamivudine therapy, 229
oral antiviral therapy, 229
oral medications, 233
oral nucleosides/nucleotides
analogues, 237
oral therapy, 237
Peginterferon alfa-2a, 231
pegylated interferon alpha, 236
TAF, 236
tenofovir, 234, 235
virological and clinical relapse
rates, 238
viral pregenomic RNAs and P product, 205
Chronic hepatitis C virus (C-HCV)
clinical cases, 163, 164
clinical manifestations, 173
diagnosis, 181
differential diagnosis, 175
epidemiology, 170, 171
etiology, 163, 164
extrahepatic manifestations
adrenal tumors, 180, 181
cardiomyopathy, 177
cryoglobulinemia, 177–179
diabetes mellitus type 2, 178
hypertension, 181
lichen planus, 180
lymphoma, 178
nephropathy, 180
porphyria cutanea tarda, 178, 179
sicca syndrome, 180
Sjogren’s syndrome, 180
xerophthalmia, 180
xerostomia, 180
genetics, 166
CD4(+)CD25(+) regulatory T cells,
168, 170
CD4+T cells, 168
CD56(dim) NK cells, 167
CD8+ T cells, 168–170
characteristics, 165
CXCR3(+)CD56Bright NK cells, 167
haplotypes, 165
HLA-C-binding KIR(+) NK cells, 166
IL-10, 170
natural killer (NK) cells, 166
polymorphism, 166
T cell mediated cytotoxic
mechanisms, 168
histology, 175–177
life cycle, 182, 183
liver histology, 175
liver transplantation, 188, 189
natural history, 171–173
physical examination, 173–175
treatment
alpha interferon therapy, 182
combination therapy, 185
cryoglobulinemia, 186, 187
daclatasvir, 184
dasabuvir, 185
direct-acting antiviral agents, 182
elbasvir, 184
Harvoni®, 184
ledipasvir, 184, 185
lichen planus, 188
nephropathy, 187
NS5B inhibitors, 185
ombitasvir, 184
pegylated interferon, 182
pibrentasvir, 184, 185
porphyria cutanea tarda, 187
ribavirin, 182
sicca syndrome, 186
simeprevir, 184
sofosbuvir, 184, 185
sustained virological response, 181
triple therapy, 182–184
velpatasvir, 184, 185
xerophthalmia, 188
xerostomia, 188
Chronic hepatitis delta virus (HDV) infection
clinical presentation of coinfection, 227
denition, 227
hepatic decompensation and HCC,
risk of, 227
liver transplantation, 239, 240
mortality rate, 228
probability of chronicity, 227
structure of, 227
treatment, 238, 239
Chronic liver disease, 418, 419
Chronic nonsuppurative destructive cholangitis
(CNSDC), 28
Cilofexor, 145
Cirrhosis, 269, 277, 335, 400
complication, 452
denition, 452
in WD, 378
Cirrhotic cardiomyopathy, 467
Clobrate, 55
Contrast enhanced ultrasound (CEUS), 400
Copper homeostasis, 372, 373
Core antigen, 204
Covalently closed circular DNA
(cccDNA), 205
CREST syndrome, 37
Index
https://t.me/medicina_free
491
Cryoglobulinemia
extrahepatic manifestations, 177–179
treatment, 186, 187
CXCR3(+)CD56Bright NK cells, 167
Cystadenocarcinomas, 404
Cystic echinococcosis, 394
Cystic brosis, 12
Cytokeratins, 142
D
Damage-associated molecular patterns
(DAMPs), 308
Dane particle, 204
Deferasirox, 359
Delirium tremens (DTs), 281
Delta hepatitis
prevalence, 227
serology of, 203
Diabetes, 350
Differentially methylated regions
(DMRs), 374
Direct-acting antiviral agents (DAA), 182
Direct repeats (RC), 204
Disulram, 278
Dominant biliary strictures, 147
Double-negative (DN)-T cells, 46
D-penicillamine (DPA), 371, 381, 383
Drug-induced hepatitis, 96
Drug induced liver injury (DILI), 411, 412
associated conditions, 435
clinical manifestations, 424
clinical phenotypes, 425–431
epidemiology, 421, 422
etiology, 412, 413
imaging studies, 432, 433
laboratory examination, 412, 432
liver histology, 433
natural history, 433–435
prevention of, 436, 437
risk factors for
age, 416
alcohol, 417
chronic liver disease, 418, 419
comorbidities, 418
drug dependent risk factors, 419
drug interactions, 420
drug metabolism, 419, 420
gender, 416
genetics, 413–415
hepatobiliary transporters,
inhibition of, 421
lipophilicity, 420
mitochondrial toxicity, 420, 421
pregnancy, 417
race and ethnicity, 417
suspected drug induced liver injury, patient
with, 422–424
treatment for, 435, 436
Drug interactions, 420
Drug metabolism, 419, 420
Drug rash with eosinophilia and systemic
symptoms (DRESS syndrome), 435
Dry gland syndrome, 35, 43, 50
Dysbiosis, 136, 137
E
Echinococcosis, 393
Edema, 20
Emperipolesis, 96
End stage liver disease (ESLD), 335
Endoscopic retrograde cholangiogram
pancreatography (ERCP), 40
Endoscopic variceal ligation, 453
Endosomal-sorting-complex-required-for- -
transport (ESCRT) system, 205
Enzyme-linked immunosorbent assay, 298
ER‐associated degradation (ERAD), 327
Erythrocytapheresis, 358, 359
Ethnicity, 417
Euchromatic histone-lysine-methyltransferase
2 (EHMT2), 212
Extracellular iron sensors, 345
F
Fat malabsorption, 470
Fatigue, 6, 57, 130, 451, 452
Ferritin, 272, 342, 358
Ferroportin disease, 345, 361
Fibrogenic mechanisms, 96
Fibrolamellar carcinoma (FLC), 401, 402
Fibroscan®, 379
Fibrosis, 357
Fibrosure, 21
Fibrotest-Actitest, 21
Flucloxacillin induced DILI, 414
Focal nodular hyperplasia (FNH), 398, 399
Follicular helper T cells (Tfh), 89
Fractalkine (CX3C), 47
G
GABAergic neurotransmission, 258
Galactose elimination capacity (GEC)
test, 264
Galectins, 103
Index
https://t.me/medicina_free
492
Gamma glutamyl transpeptidase (GGTP), 392
Gastroesophageal varices, 62, 454
Gastrointestinal bleeding, 8
Genetic iron overload conditions, treatment
for, 361
Genetic polymorphisms, 90
Genome-wide association studies (GWAS),
127, 129, 211, 257, 347, 414
Giant hemangioma, 397–398
GLOBE score, 56
Glucagon-like peptide 1 (GLP-1), 313
Glutathionylation, 49
Glycogenated bodies, 306
Granulomatosis with polyangiitis (GPA), 336
Granulomatous hepatitis, 432
Gynecomastia, 472
H
Hemochromatosis, 341
charcterization, 341
classication of, 342
clinical manifestations of, 349–353
epidemiology, 347, 348
genetic iron overload conditions, treatment
for, 361
genetics, 346, 347
hepcidin, 344–346
hepcidin-ferroportin disease,
pharmacological treatment of, 361
histology, 355, 356
iron homeostasis, 342–344
iron mediated injury, 346
laboratory test for, 353–355
liver transplantation, 360
natural history of, 348, 349
treatment, 356–360
Hemojuvelin (HJV), 344
Hepatic angiosarcoma, 402, 403
Hepatic blood ow, 2
Hepatic cysts, 393, 394
Hepatic encephalopathy (HE), 20
altered serotonin neurotransmission, 465
ammonia, 465
classication, 465
denition, 464
electroencephalagram, 466
false neurotransmitters, 465
gut dysbiosis, 465
inammation, 465
liver disease or portosystemic shunt, 465
liver transplantation, 467
manganese neurotoxicity, 465
non-pharmacological therapy, 467
pathogenesis, 464
pathological brain ndings, 464
patient evaluation, 466
pharmacological therapy, 466
physical examination, 466
treatment, 466
Hepatic epithelioid hemangioendothelioma
(HEHE), 403
Hepatic hemangiomata, 397, 398
Hepatic hydrothorax, 460
Hepatic iron index (HII), 357
Hepatic metabolism, 420
Hepatic mucinous cystic neoplasms, 404
Hepatic osteodystrophy, 268
Hepatic sinusoids, 2, 455, 456
Hepatic stellate cells, 3
Hepatic synthetic dysfunction, 63–65
Hepatic triglyceride content (HTGC), 296
Hepatitis C, 419
Hepatobiliary transporters, inhibition of, 421
Hepatocellular adenomata (HCA), 399, 400
Hepatocellular carcinoma (HCC), 111, 335,
384, 400, 401
in chronic HBV, 240–242
Hepatocellular carcinoma (HHC), 62
Hepatocellular injury, 432
Hepatopulmonary syndrome (HPS),
16, 467–469
Hepatorenal syndrome (HRS), 463
Hepatotoxicity, 412, 413
Hepatoxicity, 420
Hepcidin deciency, 341, 344–346, 361
Hepcidin-ferroportin disease, pharmacological
treatment of, 361
Homeostasis, 326
Humoral immune response, 42
Hyperbilirubinemia, 131, 272
Hypercholesterolemia, 38
Hyperpigmentation, 9
Hypervolemic hyponatremia, 461
Hypogonadotropic hypogonadism, 351
Hyposmia, 8
Hypovolemic hyponatremia, 461
Hypoxanthine phosphoribosyl transferase, 107
I
Idiosyncratic DILI, 417
Idiosyncratic drug reactions, 414
IFN-regulatory factor-3 (IRF3), 207
Immediate discontinuation, 435
Immunosuppression therapy, 107
Index
https://t.me/medicina_free
493
Impaired fatty acid oxidation, 300
Inammation in NAFLD, 308
Inammatory bowel disease (IBD), 124, 135,
147, 149
Interface hepatitis, 94
Interferon alpha, 231
Interleukin 8, 275
Intestinal dysbiosis, 89, 269
Intrahepatic cholangiocarcinoma (ICC), 402
Iron deposition in hepatocytes, 273
Iron homeostasis, 342–344
Iron mediated injury, 346
Iron metabolism, 278
Isoniazid hepatotoxicity, 416
Itch, see Pruritus
J
Jaundice, 6
Juvenile hemochromatosis, 345, 361
K
Kayser-Fleischer (KF) ring, 376
Killer immunoglobulin-like receptors
(KIRs), 127
Kupffer cells, 2
L
Lamivudine therapy, 229
Langerhans cells (LCs), 46
Leg ulcers, 10
Leipzig score, 380
Lichen planus
extrahepatic manifestations, 180
treatment, 188
Ligamentum venosum, 1
Lipid metabolism, 37
Lipophilicity, 420
Lipotoxicity, 297, 298, 300
Liver
abscesses, 394–397
anatomy of, 1
biopsy, 21, 22
cancer, 400
function, 3
hepatic blood ow, 2
histology, 2, 3
lymphatics, 2
organization, 3
transplantation, 113, 114
in A1AT deciency, 337
in hemochromatosis, 360
in PSC, 140, 141, 148, 149
in Wilson’s disease, 384, 385
tumors, 391
brolamellar carcinoma (FLC),
401, 402
FNH, 398, 399
HEHE, 403
hepatic angiosarcoma, 402, 403
hepatic cysts, 393, 394
hepatic hemangiomata, 397, 398
hepatic mucinous cystic
neoplasms, 404
hepatocellular adenomata (HCA),
399, 400
hepatocellular carcinoma (HCC),
400, 401
ICC, 402
liver abscesses, 394–397
polycystic liver disease (PCLD),
392, 393
simple liver cysts, 392
Lobular inammatory inltrates, 95
Loop-sheet polymerization, 329
Lower extremity edema, 20
Lymphocytic phlebitis, 276
Lysophosphatidylinositol acyltransferase 1
(LPIAT1), 303
Lysosomal dysfunction, 300
M
Macrophages, 300, 343
Magnetic radiographic
cholangiopancreatography
(MRCP), 40
Magnetic resonance cholangiography
(MRC), 131
Magnetic resonance elastography, 307
Magnetic resonance imaging (MRI), 379,
392, 398
Major histocompatibility complex
(MHC), 90
Malignant liver tumors, 11
Mallory Denck bodies, 305
Mammalian target of rapamycin (mTOR)
inhibitor sirolimus, 111
MBOAT7, 303
Megamitochondria, 275
Metabolic zonation, 3
Metacestode, 394
METAVIR score system, 172
Methotrexate, 418
Index
https://t.me/medicina_free
494
Methylenetetrahydrofolate reductase gene
(MTHFR), 374
Methylprednisolone, 283
MicroRNAs (miRNAs), 300
Microvesicular and macrovesicular
steatosis, 262
Mitochondrial abnormalities, 378
Mitochondrial antiviral signaling (MAVS)
protein, 206
Mitochondrial toxicity, 420, 421
Model for end stage liver disease (MELD)
score, 64, 111
Molecular mimicry theory, 41, 88, 89
Mucosal associated invariant T cells
(MAIT), 270
Muscle cramps, 464
N
N-acetyltransferases, 414
NAFLD activity score (NAS), 306
Naltrexone, 279
NARCAN nasal spray, 448
Natural killer (NK) T cells, 48, 88
Nephropathy
extrahepatic manifestations, 180
treatment, 187
New generation sequencing (NGS), 413
Non parenchymal liver cells, 2
Non suppurative destructive
cholangitis, 45
Nonalcoholic fatty liver disease
(NAFLD), 304
associated disease, 308, 309
bariatric surgery, 315, 316
clinical manifestations of, 304, 305
denition of, 295
epidemiology of, 296, 297
etiology of, 297–301
genetics of, 301, 303
inammation in, 308
lean, 310, 311
liver histology of, 305–308
natural history of, 305
treatment for, 311–315
Nonalcoholic steatohepatitis (NASH), 295
Non-cirrhotic portal hypertension, 452
Non-hepatic disorders, 37
Nonsteroidal anti-inammatory drugs
(NSAIDs), 460
Novosphingobium aromaticivorans, 29
24-norursodeoxycholic acid
(norUDCA), 144
O
Obesity, 11
Obeticholic acid (OCA), 28, 53, 54, 145, 313
Open reading frames (ORFs), 204
Opioid-receptor mediated effect, 446
Oral antiviral therapy, 229
Orthodeoxia, 468
Osteopenia, 39, 269
Osteoporosis, 39, 58–60, 269, 352
Overlap syndromes, 101, 102
Oxidative stress, 298
2 oxo-glutarate dehydrogenase complex
(OGDC), 28
P
Panniculitis, 335
“Paper money” skin, 15
Pathogen-associated molecular patterns
(PAMPs), 308
Pathogenetic mechanism, 95
Patient approach, liver disease
clinical investigation, 20, 21
comorbidities, hepatic manifestations
of, 11, 12
extremities, 19, 20
family history, 12
liver biopsy, 21, 22
past medical and surgical history, 12
physical examination
abdomen, 17, 18
cardiovascular system, 16
fetor hepaticus, 13
glands and lymph nodes, 15, 16
mucous membranes, 14
nails, 15
neuropsychiatric alterations, 20
respiratory system, 16
skin, 13, 14
review of systems, 12, 13
risks identication, 11
symptoms of
abdominal pain, 7
bone pain and fractures, 9
chills and rigors, 10
dark brown urine, 10
dyspnea, 8
fatigue, 6
gastrointestinal bleeding, 8
hyperpigmentation, 9
jaundice, 6
leg ulcers, 10
muscle cramps, 11
Index
https://t.me/medicina_free
495
pain, 9
personality changes, 8
poor appetite, 7
pruritus or itch, 6, 7
sexual dysfunction and disinterest, 10
sleep disturbances, 8
stool characteristics, 8
taste abnormalities, 7, 8
vision disturbances, 9
weight loss, 7
Peginterferon alfa-2a, 231
Periodic Acid-Schiff (PAS) positive
granules, 278
Peripheral neuropathy, 9
Periportal plasma cells, 95
Perivenular brosis, 274
Perls’ Prussian blue stain, 355
Phlebotomy, 356, 357, 361
Plasma cell inltration, 95
Plasma cells, 94
Pol-linked terminal redundant
sequence, 205
Polyarteritis nodosa, 213
Polycystic liver disease (PCLD), 392, 393
Polycystic ovarian syndrome (PCOS), 309
Portal hypertension, 61, 62
balloon tamponade, 454
blood ow diversion, 453
classication, 452
denition, 452
endoscopic variceal ligation, 453
gastroesophageal varices, 454
non-cirrhotic, 452
pharmacological therapy, 453, 454
portal pressure gradient, 452
proton pump inhibitors, 454
screening and management, 453
treatment, 187
upper gastrointestinal endoscopy, 453
Portopulmonary hypertension (PoPH)
beta blockers, 470
clinical manifestations, 469
denition, 469
Doppler transthoracic echocardiogram, 470
drugs, 470
histology, 469
liver transplantation, 470
pathogenesis, 470
PPARα receptor, 313
PPARα’s gene expression, 299
PPARγ, 299
Prednisolone, 281, 283
Prednisone, 283
Primary biliary cholangitis (PBC)
associated conditions, 37–39
buttery sign, 39
cellular immunology
anion exchanger 2, 48, 49
antigen presenting cells, 46
autophagy, 48
autoreactive T cells, 45
B cells and plasma cells, 46
biliary epithelial cells, 46, 47
CD4+CD25high regulatory T cells, 45
CD8 Tregs cells, 45
double-negative (DN)-T cells, 46
fundamental pathogenetic process, 50
glutathionylation, 49
Langerhans cells, 46
loss of self tolerance, 45
natural killer (NK) T cells, 48
PDC-E2 specic CD4+ cells, 45
peripheral CD 8+ cells, 49
T cell anergy, 46
T cells, 50
Toll like receptors, 48
clinical features, 28
clinical manifestations, 34, 35
complexes, 28
complications
cholelithiasis, 61
fatigue, 57
hepatic synthetic dysfunction, 63–65
hepatocellular carcinoma, 62
osteoporosis, 58–60
portal hypertension, 61, 62
pruritus, 56, 57
sicca symptoms, 57, 58
xanthelasmata, 61
xanthomata, 61
contraindications, 28
denitive diagnosis, 27, 28
diagnosis of, 27
epidemiology, 33, 34
epigenetics, 32
etiology, 29–31
orid bile duct lesion, 28
genetics, 31
histology, 44, 45
inner lipoyl domain, 28
natural history, 28, 36
patient history, 27
physical examination, 39, 40
prevalence and incidence, 33
radiographic studies, 40
serological hallmark of, 28
Index
https://t.me/medicina_free
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