Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lipoprotein-X (LP-X) in the serum of infants with persistent jaundice is indicative of cholestasis. In early infancy biliary atresia and biliary agenesis are the most common cause of cholestasis, whereas neonatal hepatitis is a less frequent cause of cholestasis. The authors introduced and described the qualitative and quantitative methods of LP-X determination for diagnostic purposes. LP-X estimations were carried out in 9 children with persistent jaundice. LP-X was found to be present in 4 infants-in 2 with complete absence of extrahepatic biliary tracts, in 1 with extrahepatic biliary atresia and in 1 with hypoplastic extrahepatic biliary tract. LP-X was also found in a 5 year old boy with mechanical occlusion of bile ducts caused by a malignant tumor ( rhabdomyoblastoma ), and in 3 year old girl with inborn enzymatic liver dysfunction. In this case LP-X concentration was estimated before and after 3 week course of cholestyramine, after which there was a 35% decrease in the LP-X concentration. In a 4 month old child LP-X was not found in spite of the absence of extra and intrahepatic biliary tracts. This finding may be explained by the far advanced hepatic cirrhosis. The authors stress the importance of introducing of LP-X estimation in the differential diagnosis of jaundice in early infancy.
 ...
PMID:[Lipoprotein X (LP-X) in the differential diagnosis of cholestasis in children, with special reference to biliary atresia]. 26 31

The purpose of this study was to assess the diagnostic value of magnetic resonance cholangiopancreatography (MRCP) in studying the anatomy, sites, and causes of obstructive jaundice. From September 1994 to May 1996 three-dimensional MRCP was performed on 31 patients with abdominal pain and obstructive jaundice with a fast spin-echo T2-weighted pulse sequence. The images were reconstructed using maximal intensity projection, AVERAGE and SURFACE algorithm processing techniques at a graphics workstation. All the reconstructed images were compared with those obtained using conventional cholangiographic techniques, such as endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography and drainage, and intraoperative cholangiography. The patients' diagnoses included choledochal cyst (13), cholangiocarcinoma (five), choledocholithiasis (four), pancreatic head carcinoma (three), rhabdomyosarcoma (one), papillary Vater carcinoma (one), recurrent gastric carcinoma (one), ascaris (one), and biliary atresia (two). Extrahepatic biliary dilatation was present in all 13 patients with choledochal cyst; the pancreatic ducts and their entrance level to the common bile duct were observed in eight of these patients. The level of obstruction in patients with cholangiocarcinoma was well documented but the biliary tract of one patient with biliary atresia was not identified by MRCP. In one patient with biliary rhabdomyosarcoma, MRCP clearly delineated the extrabiliary extension of the tumor. In a patient with ascaris in the common bile duct an increase in signal intensity inside the digestive tract of the worm denoted fluid in its gut. Lithiasis was shown in all of the four patients with choledocholithiasis. Thus, MRCP is a useful tool in the assessment of biliary tract obstruction and its causes, and is a valuable addition to ultrasonography.
 ...
PMID:Three-dimensional magnetic resonance cholangiopancreatography for evaluation of obstructive jaundice. 929 Feb 67

Hypertrophic osteoarthropathy (HOA) is characterized by digital clubbing, long bone periosteal reaction, and polyarthralgias. Primary familial HOA is very rare and is not associated with underlying disorders and has a good prognosis. Secondary pediatric nonneoplastic HOA is associated with cystic fibrosis, congenital heart disease, biliary atresia, and inflammatory bowel disease. Secondary neoplastic HOA may be associated with intra or extrathoracic tumors.A 5-year-old girl was admitted to our hospital for an abdominal mass, digital clubbing, and diffuse articular pain. The bone scan revealed symmetrical tracer uptake in the long bones. Upper and lower extremity x-rays were diagnostic for HOA. Paraneoplastic HOA in childhood accounts for not more than 12% of HOA paitents. HOA has been reported in 2 other cases of rhabdomyosarcoma.
 ...
PMID:Rhabdomyosarcoma associated hypertrophic osteoarthropathy in a child: detection by bone scintigraphy. 1935 78