Hepatomegaly and splenomegaly, not elsewhere classified (R16)
Clinical Information
Gaucher Disease - An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement.
Idiopathic Noncirrhotic Portal Hypertension - Portal hypertension without known risk factors for hypertension, e.g., HEPATIC CIRRHOSIS and SCHISTOSOMIASIS. Idiopathic Noncirrhotic Portal Hypertension is most often associated with pathology in the PORTAL SYSTEM vasculature.
Splenomegaly - Enlargement of the spleen.
Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00–R99)
Symptoms and signs involving the digestive system and abdomen (R10-R19)
R16 Hepatomegaly and splenomegaly, not elsewhere classified
- R16.0 Hepatomegaly, not elsewhere classified
- R16.1 Splenomegaly, not elsewhere classified
- R16.2 Hepatomegaly with splenomegaly, not elsewhere classified
Hepatomegaly and splenomegaly, not elsewhere classified (R16)