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- Lysosomal_acid_lipase_deficiency abstract "Lysosomal acid lipase deficiency (or LAL deficiency) happens when the body does not produce enough active lysosomal acid lipase (LAL or LIPA) enzyme. This enzyme plays an important role in breaking down fatty material (cholesteryl esters and triglycerides) in the body. Infants, children and adults that suffer from LAL Deficiency experience a range of serious health problems. The lack of the LAL enzyme can lead to a build-up of fatty material in a number of body organs including the liver, spleen, gut, in the wall of blood vessels and other important organs.Very low levels of the LAL enzyme lead to early onset LAL Deficiency, sometimes called Wolman disease after the physician who first described it. Early onset LAL Deficiency typically affects infants in the first year of life. The accumulation of fat in the walls of the gut in early onset disease leads to serious digestive problems including malabsorption, a condition in which the gut fails to absorb nutrients and calories from food. Because of these digestive complications, affected infants usually fail to grow and gain weight at the expected rate for their age (failure to thrive). As the disease progresses, other complications develop including increasing liver dysfunction or liver failure. Very few infants with Wolman disease survive beyond the first year of life.Late onset LAL Deficiency is sometimes called Cholesteryl ester storage disease (CESD) and can affect children and adults. The deficiency of the LAL enzyme leads to a build-up of fat in the liver, spleen and other parts of the body. The build-up of fat in the liver and spleen can cause many problems including:Swelling of the liver (‘fatty’ liver or hepatomegaly)Abnormal liver tests (high AST or ALT)Scarring of the liver (liver fibrosis or cirrhosis)Swelling of the spleen (splenomegaly)Infants, children and adults are at risk for significant health problems and premature death from complications due to LAL Deficiency.Lysosomes are found in the body’s cells and play an important role in digesting nutrients and other materials. Lysosomal Storage Disorders (LSDs) are inherited conditions in which one or more of the enzymes in lysosomes is missing or not functioning effectively. When this happens, materials that would normally be broken down by the lysosome accumulate and this disturbs normal cell function.".
- Lysosomal_acid_lipase_deficiency icd10 "E75.5".
- Lysosomal_acid_lipase_deficiency icd9 "272.7".
- Lysosomal_acid_lipase_deficiency omim "278000".
- Lysosomal_acid_lipase_deficiency wikiPageExternalLink www.rarediseases.org.
- Lysosomal_acid_lipase_deficiency wikiPageExternalLink www.hideandseek.org.
- Lysosomal_acid_lipase_deficiency wikiPageExternalLink www.lalsolace.org.
- Lysosomal_acid_lipase_deficiency wikiPageExternalLink 278000.
- Lysosomal_acid_lipase_deficiency wikiPageExternalLink 613497.
- Lysosomal_acid_lipase_deficiency wikiPageID "30896077".
- Lysosomal_acid_lipase_deficiency wikiPageLength "7683".
- Lysosomal_acid_lipase_deficiency wikiPageOutDegree "45".
- Lysosomal_acid_lipase_deficiency wikiPageRevisionID "694635080".
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Abdominal_distension.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Blood_vessel.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Calorie.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Category:Articles_created_via_the_Article_Wizard.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Category:Autosomal_recessive_disorders.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Category:Lipid_storage_disorders.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Category:Rare_diseases.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Cholesterol.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Cholesteryl_ester.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Cholesteryl_ester_storage_disease.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Chronic_liver_disease.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Cirrhosis.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Death.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Diagnosis.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Diarrhea.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Enzyme.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Failure_to_thrive.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Fat.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Gastrointestinal_tract.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Gene.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Genetic_disorder.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Genetic_sequencing_analysis.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Hepatomegaly.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Lipid.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Liver.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Liver_failure.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Low-density_lipoprotein.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Lysosomal_lipase.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Lysosomal_storage_disease.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Lysosome.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Malabsorption.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Nutrient.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Pregnancy.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Risk_factor.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Sebelipase_alfa.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Spleen.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Splenomegaly.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Symptom.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Triglyceride.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLink Wolman_disease.
- Lysosomal_acid_lipase_deficiency wikiPageWikiLinkText "Lysosomal acid lipase deficiency".
- Lysosomal_acid_lipase_deficiency wikiPageWikiLinkText "lysosomal acid lipase deficiency".
- Lysosomal_acid_lipase_deficiency diseasesdb "31220".
- Lysosomal_acid_lipase_deficiency icd "272.7".
- Lysosomal_acid_lipase_deficiency icd "E75.5".
- Lysosomal_acid_lipase_deficiency name "Lysosomal acid lipase deficiency".
- Lysosomal_acid_lipase_deficiency omim "278000".
- Lysosomal_acid_lipase_deficiency wikiPageUsesTemplate Template:Citation_needed.
- Lysosomal_acid_lipase_deficiency wikiPageUsesTemplate Template:Infobox_disease.
- Lysosomal_acid_lipase_deficiency wikiPageUsesTemplate Template:Lipid_storage_disorders.
- Lysosomal_acid_lipase_deficiency wikiPageUsesTemplate Template:No_footnotes.
- Lysosomal_acid_lipase_deficiency wikiPageUsesTemplate Template:Reflist.
- Lysosomal_acid_lipase_deficiency subject Category:Articles_created_via_the_Article_Wizard.
- Lysosomal_acid_lipase_deficiency subject Category:Autosomal_recessive_disorders.
- Lysosomal_acid_lipase_deficiency subject Category:Lipid_storage_disorders.
- Lysosomal_acid_lipase_deficiency subject Category:Rare_diseases.
- Lysosomal_acid_lipase_deficiency type Disease.
- Lysosomal_acid_lipase_deficiency type Disorder.
- Lysosomal_acid_lipase_deficiency type Redirect.
- Lysosomal_acid_lipase_deficiency type Thing.
- Lysosomal_acid_lipase_deficiency type Q12136.
- Lysosomal_acid_lipase_deficiency comment "Lysosomal acid lipase deficiency (or LAL deficiency) happens when the body does not produce enough active lysosomal acid lipase (LAL or LIPA) enzyme. This enzyme plays an important role in breaking down fatty material (cholesteryl esters and triglycerides) in the body. Infants, children and adults that suffer from LAL Deficiency experience a range of serious health problems.".
- Lysosomal_acid_lipase_deficiency label "Lysosomal acid lipase deficiency".
- Lysosomal_acid_lipase_deficiency sameAs Q6710283.
- Lysosomal_acid_lipase_deficiency sameAs Deficyt_lizosomalnej_kwaśnej_lipazy.
- Lysosomal_acid_lipase_deficiency sameAs m.0gg559p.
- Lysosomal_acid_lipase_deficiency sameAs Q6710283.
- Lysosomal_acid_lipase_deficiency wasDerivedFrom Lysosomal_acid_lipase_deficiency?oldid=694635080.
- Lysosomal_acid_lipase_deficiency isPrimaryTopicOf Lysosomal_acid_lipase_deficiency.
- Lysosomal_acid_lipase_deficiency name "Lysosomal acid lipase deficiency".