Porphyrias, Hepatic [jaterní porfyrie]

topical
27
Terms

hepatická porfyrie
porfyrie hepatální
porfyrie jaterní

 

Hepatic Porphyria
Porphyria, Hepatic

Persistent link   https://www.medvik.cz/link/D017094
Definition

A group of metabolic diseases due to deficiency of one of a number of LIVER enzymes in the biosynthetic pathway of HEME. They are characterized by the accumulation and increased excretion of PORPHYRINS or its precursors. Clinical features include neurological symptoms (PORPHYRIA, ACUTE INTERMITTENT), cutaneous lesions due to photosensitivity (PORPHYRIA CUTANEA TARDA), or both (HEREDITARY COPROPORPHYRIA). Hepatic porphyrias can be hereditary or acquired as a result of toxicity to the hepatic tissues.

Annotation
do not confuse with PORPHYRIA, ERYTHROHEPATIC
DUI
D017094 MeSH Browser
CUI
M0025973
Previous indexing
Porphyria (1966-1992)
History note
2005 (1993)
Public note
2005; see PORPHYRIA, HEPATIC 1993-2004

Allowable subheadings

BL
blood
CF
cerebrospinal fluid
CI
chemically induced 1
CL
classification 1
CO
complications 2
CN
congenital
DI
diagnosis 9
DG
diagnostic imaging
DH
diet therapy
DT
drug therapy 3
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology 4
GE
genetics 7
HI
history
IM
immunology
ME
metabolism 1
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology 2
PP
physiopathology 3
PC
prevention & control
PX
psychology
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy 5
UR
urine 1
VE
veterinary
VI
virology

C Diseases
C06 Digestive System Diseases 674
C06.552 Liver Diseases 3 026
C06.552.074 alpha 1-Antitrypsin Deficiency 86
C06.552.100 Chemical and Drug Induced Liver Injury 270
C06.552.150 Cholestasis, Intrahepatic 163
C06.552.241 Fatty Liver 374
C06.552.270 Focal Nodular Hyperplasia 18
C06.552.289 Hepatic Infarction
C06.552.308 Hepatic Insufficiency 39
C06.552.347 Budd-Chiari Syndrome 45
C06.552.360 Hepatic Veno-Occlusive Disease 23
C06.552.380 Hepatitis 734
C06.552.413 Hepatolenticular Degeneration 243
C06.552.416 Hepatomegaly 116
C06.552.455 Hepatopulmonary Syndrome 8
C06.552.465 Hepatorenal Syndrome 91
C06.552.494 Hypertension, Portal 648
C06.552.597 Liver Abscess 52
C06.552.630 Liver Cirrhosis 1 541
C06.552.645 Liver Diseases, Alcoholic 94
C06.552.664 Liver Diseases, Parasitic 56
C06.552.697 Liver Neoplasms 1 664
C06.552.802 Peliosis Hepatis
C06.552.830 Porphyrias, Hepatic 27
C06.552.830.074 Coproporphyria, Hereditary 2
C06.552.830.100 Porphyria Cutanea Tarda 69
C06.552.830.150 Porphyria, Acute Intermittent 43
C06.552.830.437 Porphyria, Hepatoerythropoietic 7
C06.552.830.625 Porphyria, Variegate 4
C06.552.830.812 Protoporphyria, Erythropoietic 8
C06.552.933 Tuberculosis, Hepatic 9
C06.552.970 Zellweger Syndrome 10
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities 439
C16.320 Genetic Diseases, Inborn 1 215
C16.320.850 Skin Diseases, Genetic 89
C16.320.850.080 Albinism 17
C16.320.850.180 Cutis Laxa 10
C16.320.850.190 Darier Disease 26
C16.320.850.210 Dermatitis, Atopic 1 454
C16.320.850.235 Dyskeratosis Congenita 9
C16.320.850.250 Ectodermal Dysplasia 33
C16.320.850.260 Ehlers-Danlos Syndrome 51
C16.320.850.275 Epidermolysis Bullosa 108
C16.320.850.337 Erythrokeratodermia Variabilis 1
C16.320.850.368 Hyaline Fibromatosis Syndrome 1
C16.320.850.400 Ichthyosiform Erythroderma, Congenital 8
C16.320.850.402 Ichthyosis Bullosa of Siemens 3
C16.320.850.405 Ichthyosis Vulgaris 5
C16.320.850.408 Ichthyosis, X-Linked 9
C16.320.850.420 Incontinentia Pigmenti 19
C16.320.850.475 Keratoderma, Palmoplantar 15
C16.320.850.542 Leukokeratosis, Hereditary Mucosal 1
C16.320.850.595 Lipoid Proteinosis of Urbach and Wiethe 5
C16.320.850.647 Monilethrix 1
C16.320.850.673 Netherton Syndrome 2
C16.320.850.700 Pemphigus, Benign Familial 11
C16.320.850.730 Porokeratosis 11
C16.320.850.738 Porphyria, Erythropoietic 11
C16.320.850.742 Porphyrias, Hepatic 27
C16.320.850.742.074 Coproporphyria, Hereditary 2
C16.320.850.742.150 Porphyria, Acute Intermittent 43
C16.320.850.742.250 Porphyria Cutanea Tarda 69
C16.320.850.742.437 Porphyria, Hepatoerythropoietic 7
C16.320.850.742.625 Porphyria, Variegate 4
C16.320.850.742.812 Protoporphyria, Erythropoietic 8
C16.320.850.746 Prolidase Deficiency
C16.320.850.750 Pseudoxanthoma Elasticum 13
C16.320.850.765 Rothmund-Thomson Syndrome 8
C16.320.850.820 Sjogren-Larsson Syndrome 4
C16.320.850.895 Trichothiodystrophy Syndromes 1
C16.320.850.970 Xeroderma Pigmentosum 25
C17 Skin and Connective Tissue Diseases 32
C17.800 Skin Diseases 2 801
C17.800.827 Skin Diseases, Genetic 89
C17.800.827.080 Albinism 17
C17.800.827.180 Cutis Laxa 10
C17.800.827.190 Darier Disease 26
C17.800.827.210 Dermatitis, Atopic 1 454
C17.800.827.235 Dyskeratosis Congenita 9
C17.800.827.250 Ectodermal Dysplasia 33
C17.800.827.260 Ehlers-Danlos Syndrome 51
C17.800.827.275 Epidermolysis Bullosa 108
C17.800.827.337 Erythrokeratodermia Variabilis 1
C17.800.827.368 Hereditary Autoinflammatory Diseases 47
C17.800.827.384 Hyaline Fibromatosis Syndrome 1
C17.800.827.400 Ichthyosiform Erythroderma, Congenital 8
C17.800.827.403 Ichthyosis Bullosa of Siemens 3
C17.800.827.405 Ichthyosis Vulgaris 5
C17.800.827.408 Ichthyosis, X-Linked 9
C17.800.827.420 Incontinentia Pigmenti 19
C17.800.827.475 Keratoderma, Palmoplantar 15
C17.800.827.595 Leukokeratosis, Hereditary Mucosal 1
C17.800.827.602 Monilethrix 1
C17.800.827.610 Muir-Torre Syndrome 10
C17.800.827.655 Netherton Syndrome 2
C17.800.827.700 Pemphigus, Benign Familial 11
C17.800.827.730 Porokeratosis 11
C17.800.827.738 Porphyria, Erythropoietic 11
C17.800.827.742 Porphyrias, Hepatic 27
C17.800.827.742.074 Coproporphyria, Hereditary 2
C17.800.827.742.150 Porphyria, Acute Intermittent 43
C17.800.827.742.250 Porphyria Cutanea Tarda 69
C17.800.827.742.437 Porphyria, Hepatoerythropoietic 7
C17.800.827.742.625 Porphyria, Variegate 4
C17.800.827.742.812 Protoporphyria, Erythropoietic 8
C17.800.827.750 Pseudoxanthoma Elasticum 13
C17.800.827.775 Rothmund-Thomson Syndrome 8
C17.800.827.820 Sjogren-Larsson Syndrome 4
C17.800.827.895 Trichothiodystrophy Syndromes 1
C17.800.827.970 Xeroderma Pigmentosum 25
C18 Nutritional and Metabolic Diseases 172
C18.452 Metabolic Diseases 1 200
C18.452.811 Porphyrias 252
C18.452.811.250 Porphyria, Erythropoietic 11
C18.452.811.400 Porphyrias, Hepatic 27
C18.452.811.400.074 Coproporphyria, Hereditary 2
C18.452.811.400.150 Porphyria, Acute Intermittent 43
C18.452.811.400.250 Porphyria Cutanea Tarda 69
C18.452.811.400.437 Porphyria, Hepatoerythropoietic 7
C18.452.811.400.625 Porphyria, Variegate 4
C18.452.811.400.812 Protoporphyria, Erythropoietic 8

Porphyria, Acute Hepatic Disease MeSH Browser

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