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- Disease Information
- Disease Comparison
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Disease Processes ▼
- Auto Immune
- Vascular-Arteriosclerosis
- Biochemical
- Congenital-developmental
- Deficiency
- Degenrative-Necrosis
- Electromagnetic-Physics
- Eponymic
- Functional-Physiologic
- Hereditofamilial
- Iatrogenic
- Idiopathic
- Infected Organ-Abcess
- Infectious agent
- Inflammatory-Granulomatous
- Metabolic-Storage
- Neoplastic
- Poison Agent
- Poisoned Organ
- Radiation-Xray-trauma
- Mental
- Structural-Anatomic-Foreign body
- Surgical Procedure-Complication
- Trauma
- Use-age-Atrophic
- Endocrine-Vegetative
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Major Organs-Systems ▼
- Systemic
- Pediatric
- Nervous & Sensory System (Neurology)
- Cardiovascular System
- Respiratory (Pulmonary) System
- Gastro-Intestinal (Digestive) System
- Urinary System
- Dermatologic System
- Endocrine System
- Immune System
- Musculoskeletal System
- Genital Reproductive System
- Hematopoietic System (Hematology)
- Lymphatic System
- Tissue/Cells/Organelles
Disease Information for Wolman Disease
- Clinical Manifestations
- Signs & Symptoms
- Abdominal distension/protrusion/sign
- Failure to Thrive
- Failure to Thrive Child
- Failure to thrive/infant sign
- Fatty food intolerance
- Flatus/gas foul smelling
- Foul stool
- Projectile vomiting
- Relentless vomiting/retching/purging
- Stool Bulky/greasy/frothy/malodorous
- Hepatosplenomegaly
- Jaundice/Yellow skin and eyes
- Massive Hepatomegaly
- Palpable Liver/Hepatomegaly
- Hypotonia
- Development Motor Skills (Milestones) Delayed
- Splenomegaly
- Clinical Presentation & Variations
- Presentation/Death in infancy
- Disease Progression
- Course/Chronic disorder
- Course/Chronic only
- Course/Fulminant/Precipitous
- Course/Lethal
- Course/Prognosis bad/usually
- Course/Relapsing
- Lethal Potential
- Onset/Infancy 3-4 Months
- Prognosis/Childhood-congenital early death usual
- Demographics & Risk Factors
- Ethnic or Racial Factors
- Iran population/ethnic
- Jewish population
- Sephardic Jewish population
- Population Group
- Child
- Infant
- Population/Pediatrics population
- Sex & Age Groups
- Population/Child
- Population/Child-Infant Only
- Population/Children/all
- Population/Infant
- Laboratory Tests
- Abnormal Lab Findings (Non Measured)
- Chorionic Villous Sampling Abnormalities
- Cultured fibroblast enzyme assay abnormal (Lab)
- Vacuolated lymphocytes/Lab
- Abnormal Lab Findings - Decreased
- Hematocrit (Lab)
- Hemoglobin (Lab)
- Iron, serum (Lab)
- RBC/Red Blood Count (Lab)
- Abnormal Lab Findings - Increased
- Cholesterol (Lab)
- STOOL Fat Sudan stain
- Diagnostic Test Results
- Pathology
- BX/Skin biopsy/Abnormal
- Cultured Fibroblasts/Lysosomal Acid Lipase Defect
- Bone marrow/Foam cells
- Bone Marrow/Sea-blue histiocytes/Pathology
- PATH/Fat Deposition Viscera
- X-RAY
- Xray/Adrenal calcifications/bilateral/KUB
- Xray/Adrenal calcifications/Diffuse punctate
- Xray/Abdominal calcifications/ABD
- Xray/Abdominal film abnormalities/ABD/KUB
- Associated Diseases & Rule outs
- Associated Disease & Complications
- Adrenocortical insufficiency
- Anemia
- Arteriosclerosis
- Cachexia/inanition/wasting
- Cirrhosis
- Death Newborn/Perinatal
- Death/Unanticipated
- Hepatic failure
- Hypercholesterolemia
- Malabsorption/Steatorrhea syndrome
- Malnutrition/Starvation
- Steatorrhea
- Wolman Disease
- Disease Mechanism & Classification
- Class
- CLASS/Pediatric disorders (ex)
- Pathophysiology
- Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
- Pathophysiology/Lysosome storage disorder (ex)
- Pathophysiology/Neutral lipid storage disease/all tissues
- Pathophysiology/Storage/glycerides/cholesteryl esters
- Pathophysiology/Fat Malabsorption
- Pathophysiology/Fat soluble Vitamin Malabsorption
- Process
- PROCESS/Autosomal recessive disorder (ex)
- PROCESS/Hereditofamilial (category)
- PROCESS/INCIDENCE/Extremely rare disease
- PROCESS/Lipidosis/storage disorder (ex)
- PROCESS/Metabolic/storage disorder (category)
- PROCESS/Storage disorder (ex)
- Synonyms
- Synonym
- Acid esterase deficiency, Acid lipase deficiency, Deficiency of cholesterol esterase and triacylglycerol lipase, Disease Wolman, Disease Wolman's, Familial visceral xanthomatosis, Familial Xanthomatoses, Familial Xanthomatosis, Familial xanthomatosis with adrenal involvement, LIPA deficiency, Liposomal Acid Lipase Deficiency Wolman Type, Lysosomal acid lipase deficien, Lysosomal acid lipase deficiency, Primary familial xanthomatosis, Primary familial xanthomatosis with adrenal calcification, Wolman, WOLMAN DIS, Wolman disease, Wolman xanthomatosis, WOLMANS DIS, Wolmans Disease, Wolman's disease, Wolman's disease (disorder), Wolman's or triglyceride storage Type III disease, Wolman's Xanthomatosis, Xanthomatoses Familial, Xanthomatosis Familial, Xanthomatosis familial (disorder), Xanthomatosis Wolman, Xanthomatosis Wolmans, Xanthomatosis Wolman's, Synonym/Acid Cholesteryl Hydrolase Deficiency
- Treatment
- Drug Therapy - Indication
- RX/Directed Enzyme Replacement
- RX/No effective/definitive treatment yet available
- SX/Bone marrow transplant
- SX/Stem cell transplant/allogenic
- SX/Stem-cell transplant
- Surgical Procedures or Treatments
- TX/Cord Blood Transplant
- Other Treatments
- TX/Supportive/Symptomatic treatment
- Definition
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The severe infantile form of inherited lysosomal lipid storage diseases, due to deficiency of acid lipase. It results in accumulation of neutral lipids, particularly cholesterol esters, within cells (particularly leukocytes, fibroblasts, and liver cells). An allelic variant of CHOLESTEROL ESTER STORAGE DISEASE, it is also known as Wolman's xanthomatosis.
(Edit)
- External Links Related to Wolman Disease
- Wikipedia
- Merck
- Images
- PubMed (National Library of Medicine)
- NGC (National Guideline Clearinghouse)
- Medscape (eMedicine)
- Harrison's Online (accessmedicine)
- NEJM (The New England Journal of Medicine)
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