Sjogren-Larsson Syndrome [Sjögrenův-Larssonův syndrom]

topical
4
Terms

Sjoegrenův-Larssonův syndrom
Sjögren-Larssonův syndrom

 

Congenital Icthyosis Mental Retardation Spasticity Syndrome
FALDH Deficiency
Fatty Alcohol:NAD+ Oxidoreductase Deficiency
Fatty Aldehyde Dehydrogenase Deficiency
Fatty Aldehyde Dehydrogenase Deficiency Disease
Ichthyosis Oligophrenia Syndrome
Ichthyosis, Spastic Neurologic Disorder, and Oligophrenia
Sjögren-Larsson Syndrome

Persistent link   https://www.medvik.cz/link/D016111
Definition

An autosomal recessive neurocutaneous disorder characterized by severe ichthyosis MENTAL RETARDATION; SPASTIC PARAPLEGIA; and congenital ICHTHYOSIS. It is caused by mutation of gene encoding microsomal fatty ALDEHYDE DEHYDROGENASE leading to defect in fatty alcohol metabolism.

Annotation
do not confuse with SJOGREN'S SYNDROME; in titles & translations use diacritic: Sjögren-Larsson
DUI
D016111 MeSH Browser
CUI
M0024610
Previous indexing
Ichthyosis (1966-1990)
History note
1991; use ICHTHYOSIS 1984-1990
Public note
1991; see ICHTHYOSIS 1984-1990

Allowable subheadings

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

C Diseases
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities 439
C16.131 Congenital Abnormalities 1 753
C16.131.831 Skin Abnormalities 51
C16.131.831.512 Ichthyosis 62
C16.131.831.512.400 Ichthyosiform Erythroderma, Congenital 8
C16.131.831.512.408 Ichthyosis Bullosa of Siemens 3
C16.131.831.512.410 Ichthyosis Vulgaris 5
C16.131.831.512.420 Ichthyosis, X-Linked 9
C16.131.831.512.723 Sjogren-Larsson Syndrome 4
C16.320 Genetic Diseases, Inborn 1 215
C16.320.565 Metabolism, Inborn Errors 714
C16.320.565.398 Lipid Metabolism, Inborn Errors 154
C16.320.565.398.641 Lipidoses 35
C16.320.565.398.641.201 Cholesterol Ester Storage Disease 13
C16.320.565.398.641.509 Neuronal Ceroid-Lipofuscinoses 29
C16.320.565.398.641.723 Sjogren-Larsson Syndrome 4
C16.320.565.398.641.803 Sphingolipidoses 12
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
C16.320.850.746 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
C16.614 Infant, Newborn, Diseases 1 950
C16.614.492 Ichthyosis 62
C16.614.492.400 Ichthyosiform Erythroderma, Congenital 8
C16.614.492.410 Ichthyosis Bullosa of Siemens 3
C16.614.492.420 Ichthyosis, X-Linked 9
C16.614.492.723 Sjogren-Larsson Syndrome 4
C17 Skin and Connective Tissue Diseases 32
C17.800 Skin Diseases 2 801
C17.800.428 Keratosis 133
C17.800.428.333 Ichthyosis 62
C17.800.428.333.250 Ichthyosiform Erythroderma, Congenital 8
C17.800.428.333.330 Ichthyosis Bullosa of Siemens 3
C17.800.428.333.410 Ichthyosis Vulgaris 5
C17.800.428.333.420 Ichthyosis, X-Linked 9
C17.800.428.333.723 Sjogren-Larsson Syndrome 4
C17.800.804 Skin Abnormalities 51
C17.800.804.512 Ichthyosis 62
C17.800.804.512.400 Ichthyosiform Erythroderma, Congenital 8
C17.800.804.512.405 Ichthyosis Bullosa of Siemens 3
C17.800.804.512.410 Ichthyosis Vulgaris 5
C17.800.804.512.420 Ichthyosis, X-Linked 9
C17.800.804.512.723 Sjogren-Larsson Syndrome 4
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
C17.800.827.750 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
C18.452.584 Lipid Metabolism Disorders 99
C18.452.584.563 Lipid Metabolism, Inborn Errors 154
C18.452.584.563.641 Lipidoses 35
C18.452.584.563.641.201 Cholesterol Ester Storage Disease 13
C18.452.584.563.641.509 Neuronal Ceroid-Lipofuscinoses 29
C18.452.584.563.641.723 Sjogren-Larsson Syndrome 4
C18.452.584.563.641.803 Sphingolipidoses 12
C18.452.648 Metabolism, Inborn Errors 714
C18.452.648.398 Lipid Metabolism, Inborn Errors 154
C18.452.648.398.641 Lipidoses 35
C18.452.648.398.641.201 Cholesterol Ester Storage Disease 13
C18.452.648.398.641.509 Neuronal Ceroid-Lipofuscinoses 29
C18.452.648.398.641.723 Sjogren-Larsson Syndrome 4
C18.452.648.398.641.803 Sphingolipidoses 12

Sjogren-Larsson-like syndrome Disease MeSH Browser

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