Wolman disease (Q65100): Difference between revisions

@@ description / pt-br / description / pt-br @@
+A doença de Wolman representa a manifestação mais grave da deficiência de lipase ácida lisossômica pode se manifestar no feto (hepatomegalia, ascite, glândulas adrenais calcificadas) ou, mais comumente, nas primeiras semanas de vida com distensão abdominal e hepatoesplenomegalia grave ou mesmo maciça (que pode ocorrer no período neonatal) e às vezes ascite e glândulas adrenais calcificadas.
@@ description / en / description / en @@
+Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the first weeks of life with abdominal distension and major or even massive hepatosplenomegaly (which can occur in the neonatal period) and sometimes ascites and calcified adrenal glands.
@@ Property / Canonical URI @@
+https://id.who.int/icd/entity/520367511
@@ Property / Canonical URI: https://id.who.int/icd/entity/520367511 / rank @@
+Normal rank
@@ Property / CURIE @@
+CID11:ID_520367511
@@ Property / CURIE: CID11:ID_520367511 / rank @@
+Normal rank
@@ Property / Canary Token @@
+dki-india-ID_520367511
@@ Property / Canary Token: dki-india-ID_520367511 / rank @@
+Normal rank
@@ Property / Verification Status @@
+Concluído
@@ Property / Verification Status: Concluído / rank @@
+Normal rank
@@ Property / Knowledge Architect @@
+https://pauloleads.com.br/cases-publicos/
+Normal rank
@@ Property / Collection date @@
+August 2026Timestamp +2026-08-13T00:00:00Z
Timezone +00:00
Calendar Gregorian
Precision 1 day
Before 0
After 0
-Timestamp
++2026-08-13T00:00:00Z
-Timezone
++00:00
-Calendar
+Gregorian
-Precision
+day
 Before
 After
@@ Property / Collection date: 13 August 2026 / rank @@
+Normal rank

Latest revision as of 12:49, 14 August 2026

Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the first weeks of life with abdominal distension and major or even massive hepatosplenomegaly (which can occur in the neonatal period) and sometimes ascites and calcified adrenal glands.

Language	Label	Description	Also known as
default for all languages	ID_520367511
English	Wolman disease	Wolman disease represents the most severe manifestation of lysosomal acid lipase deficiency and can present in the fetus (hepatomegaly, ascites, calcified adrenal glands), or more typically in the first weeks of life with abdominal distension and major or even massive hepatosplenomegaly (which can occur in the neonatal period) and sometimes ascites and calcified adrenal glands.