ONKI3 | Medical Subject Headings


Hierarchy:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Lipid Metabolism, Inborn Errors ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Lysosomal Storage Diseases ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Infant, Newborn, Diseases ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Lipid Metabolism Disorders ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Lipid Metabolism, Inborn Errors ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Lysosomal Storage Diseases ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease 28 coordinate concepts ∟ Amniotic Band Syndrome \| ∟ Anemia, Neonatal \| ∟ Asphyxia Neonatorum \| ∟ Birth Injuries \| ∟ Cystic Fibrosis \| ∟ Epilepsy, Benign Neonatal \| ∟ Erythroblastosis, Fetal \| ∟ Hemorrhagic Disease of Newborn \| ∟ Hernia, Umbilical \| ∟ Hydrocephalus \| ∟ Hydrophthalmos \| ∟ Hyperbilirubinemia, Neonatal \| ∟ Hyperostosis, Cortical, Congenital \| ∟ Ichthyosis \| ∟ Infant, Premature, Diseases \| ∟ Meconium Aspiration Syndrome \| ∟ Mobius Syndrome \| ∟ Neonatal Abstinence Syndrome \| ∟ Nystagmus, Congenital \| ∟ Ophthalmia Neonatorum \| ∟ Persistent Fetal Circulation Syndrome \| ∟ Persistent Hyperinsulinemia Hypoglycemia of Infancy \| ∟ Rothmund-Thomson Syndrome \| ∟ Sclerema Neonatorum \| ∟ Severe Combined Immunodeficiency \| ∟ Syphilis, Congenital \| ∟ Thanatophoric Dysplasia \| ∟ Toxoplasmosis, Congenital X
Broader terms:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Lipid Metabolism, Inborn Errors ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Metabolism, Inborn Errors ∟ Lysosomal Storage Diseases ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Infant, Newborn, Diseases ∟ Wolman Disease Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Lipid Metabolism Disorders ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Lipid Metabolism, Inborn Errors ∟ Lipidoses ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease Medical Subject Headings (MeSH) ∟ Nutritional and Metabolic Diseases ∟ Metabolic Diseases ∟ Metabolism, Inborn Errors ∟ Lysosomal Storage Diseases ∟ Cholesterol Ester Storage Disease ∟ Wolman Disease Cholesterol Ester Storage Disease > Lipidoses > Lipid Metabolism, Inborn Errors > Metabolism, Inborn Errors > Genetic Diseases, Inborn > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Cholesterol Ester Storage Disease > Lysosomal Storage Diseases > Metabolism, Inborn Errors > Genetic Diseases, Inborn > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Infant, Newborn, Diseases > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Cholesterol Ester Storage Disease > Lipidoses > Lipid Metabolism Disorders > Metabolic Diseases > Nutritional and Metabolic Diseases > Medical Subject Headings (MeSH) Cholesterol Ester Storage Disease > Lipidoses > Lipid Metabolism, Inborn Errors > Metabolism, Inborn Errors > Metabolic Diseases > Nutritional and Metabolic Diseases > Medical Subject Headings (MeSH) Cholesterol Ester Storage Disease > Lysosomal Storage Diseases > Metabolism, Inborn Errors > Metabolic Diseases > Nutritional and Metabolic Diseases > Medical Subject Headings (MeSH) X
History note:	Cholesterol Esters (1978-1988) \| Lipase (1966-1988) \| Lipid Metabolism, Inborn Errors (1966-1988) \| Lipoidosis (1966-1988) \| Xanthomatosis (1966-1988)X
historyNote*:	89 X
publicMeSHNote*:	89 X
Scope note:	The severe infantile form of inherited lysosomal lipid storage diseases due to deficiency of acid lipase (CHOLESTEROL ESTERASE). It is characterized by the accumulation of neutral lipids, particularly CHOLESTEROL ESTERS in leukocytes, fibroblasts, and hepatocytes. It is also known as Wolman's xanthomatosis and is an allelic variant of CHOLESTEROL ESTER STORAGE DISEASE. X
activeMeSHYear*:	2007X
dateCreated*:	1988-05-12X
dateEstablished*:	1989-01-01X
dateRevised*:	2006-07-05X
recordAuthorizer*:	sjnX
recordMaintainer*:	lktX
recordOriginator*:	EJBX
Type:	Concept X
Coordinate terms:	Amniotic Band Syndrome \| Anemia, Neonatal \| Asphyxia Neonatorum \| Birth Injuries \| Cystic Fibrosis \| Epilepsy, Benign Neonatal \| Erythroblastosis, Fetal \| Hemorrhagic Disease of Newborn \| Hernia, Umbilical \| Hydrocephalus \| Hydrophthalmos \| Hyperbilirubinemia, Neonatal \| Hyperostosis, Cortical, Congenital \| Ichthyosis \| Infant, Premature, Diseases \| Meconium Aspiration Syndrome \| Mobius Syndrome \| Neonatal Abstinence Syndrome \| Nystagmus, Congenital \| Ophthalmia Neonatorum \| Persistent Fetal Circulation Syndrome \| Persistent Hyperinsulinemia Hypoglycemia of Infancy \| Rothmund-Thomson Syndrome \| Sclerema Neonatorum \| Severe Combined Immunodeficiency \| Syphilis, Congenital \| Thanatophoric Dysplasia \| Toxoplasmosis, Congenital X
URI:	X
Labels and equivalent concepts:	Wolmanin tauti (fi) Wolmans sjukdom (sv) Xanthomatosis, Wolman's (en, replaced) Xanthomatosis, Familial (en, replaced) Wolman's Disease (en, replaced) Liposomal Acid Lipase Deficiency, Wolman Type (en, replaced) Morbus Wolman (la-FI, replaced) X
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