Thelansis

  Wolman disease, an intense manifestation of lysosomal acid lipase deficiency, is characterized by swift and extensive accumulation of lipids in organs and tissues. It manifests with notable symptoms during the neonatal or infantile phase, including significant hepatosplenomegaly, liver failure, steatorrhea-associated diarrhea, and vomiting. Although the disease can also emerge prenatally with indications like hepatomegaly, ascites, and calcified adrenal glands, its more common onset occurs within the initial weeks of life. This phase is marked by abdominal distension, substantial hepatosplenomegaly (which can even manifest in the neonatal period), and intermittent ascites. A notable radiographic sign is the presence of calcified adrenal glands. Children affected by this condition experience notable gastrointestinal disturbances, such as vomiting and diarrhea accompanied by steatorrhea. These symptoms can result in abrupt cessation of weight gain and gradual deterioration of psy...