Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a neonate with two very rare anomalies: primary chylopericardium and diffuse hypoplasia of the thoraco-abdominal aorta. The presentation on the 16th post-natal day was with dyspnoea and refusal to feed. The initial clinical examination revealed hepatomegaly and weak femoral pulses. 2D ultrasound gave a diagnosis of a large compressive pericardial effusion combined with moderate hypoplasia of the aortic isthmus. Emergency pericardial drainage removed 80ml of chylous liquid. Following prolonged parenteral nutrition, the pericardial effusion stabilised. A mechanism of diffuse vascular disease affecting the aorta and the lymphatic system is suggested.
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PMID:[Unusual association of chylopericardium and aortic hypoplasia in a neonate]. 1596 13

Hepatic sinusoidal obstruction syndrome (HSOS) is a hepatic vascular disease presenting with abdominal distension, pain in the hepatic region, ascites, jaundice, and hepatomegaly. In China, this disease is often associated with the oral intake of plants that contain pyrrolidine alkaloids. The existing guidelines are limited to HSOS associated with hematopoietic stem cell transplantation in Western countries. The Hepatobiliary Diseases Committee of the Chinese Society of Gastroenterology convened an expert consensus conference on the diagnosis and treatment of PA-HSOS to evaluate current research in China and abroad. The "Nanjing criteria" developed by the committee to diagnose PA-HSOS include a confirmed history of PA-containing plant use and (i) abdominal distention and/or pain in the hepatic region, hepatomegaly, and ascites; (ii) elevation of serum total bilirubin or abnormal laboratory liver tests; (iii) evidence on enhanced computed tomography or magnetic resonance imaging; or (iv) pathological evidence that rules out other known causes of liver injury. Supportive symptomatic treatment, anticoagulant therapy, and placement of a transjugular intrahepatic portosystemic shunt for patients who do not respond to medical treatment are effective for the treatment of PA-HSOS. The benefits of glucocorticoids and prostaglandin E1 in PA-HSOS are not clear.
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PMID:Expert consensus on the clinical management of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome. 3066 84