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In this study, 64% of children aged 7-12 years with sickle cell disease were found to have a parent-reported behavior problem, and 50% met the criteria for a Diagnostic and Statistical Manual of Mental Disorders (3rd ed.) diagnosis based on a structural clinical interview of the child. Internalizing types of behavior problems and diagnoses were the most(More)
OBJECTIVE Brain magnetic resonance imaging (MRI) and neuropsychological evaluations were conducted to determine whether neuroradiographic evidence of infarct in children with sickle cell disease between ages 6 and 12 years would result in impairment in cognitive and academic functioning. METHOD AND DESIGN Children enrolled in the Cooperative Study of(More)
BACKGROUND & AIMS The bile salt export pump (BSEP) is the major bile salt transporter in the liver canalicular membrane. Our aim was to determine the affinity of the human BSEP for bile salts and identify inhibitors. METHODS Human BSEP was expressed in insect cells. Adenosine triphosphatase (ATPase) assays were performed, and bile salt transport studies(More)
Examined pain coping strategies in 72 children and adolescents with sickle cell disease and their parents using the Coping Strategies Questionnaire, and found that pain coping strategies could be reliably assessed. Children high on Negative Thinking and Passive Adherence were less active, required more health care services, and were more psychologically(More)
Assessed the psychological adjustment of 78 mothers of children and adolescents (7-17 years of age) with sickle cell disease. Support was provided for a transactional stress and coping model in delineating the processes associated with maternal adjustment. In particular, poor maternal adjustment was associated with use of palliative coping methods and high(More)
The CALICE collaboration is studying the design of high performance electromagnetic and hadronic calorimeters for future International Linear Collider detectors. For the electromagnetic calorimeter, the current baseline choice is a high granularity sampling calorimeter with tungsten as absorber and silicon detectors as sensitive material. A " physics(More)
Elucidating genetic causes of cholestasis has proved to be important in understanding the physiology and pathophysiology of the liver. Here we show that protein-truncating mutations in the tight junction protein 2 gene (TJP2) cause failure of protein localization and disruption of tight-junction structure, leading to severe cholestatic liver disease. These(More)
The progressive familial intrahepatic cholestases (PFIC) are a group of inherited disorders with severe cholestatic liver disease from early infancy. A subgroup characterized by normal serum cholesterol and gamma-glutamyltranspeptidase (gammaGT) levels is genetically heterogeneous with loci on chromosomes 2q (PFIC2) and 18q. The phenotype of the(More)
BACKGROUND & AIMS Progressive familial intrahepatic cholestasis (PFIC), an inherited liver disease of childhood, is characterized by cholestasis and either normal or increased serum gamma-glutamyltransferase activity. Patients with normal gamma-glutamyltransferase activity have mutations of the FIC1 locus on chromosome 18q21 or mutations of the BSEP gene on(More)
Found 60% of children 7-12 years old with cystic fibrosis to have a parent-reported behavior problem and 62% met the criteria for a DSM-III diagnosis based on a structured clinical interview with the child. Mixed internal and external behavior problem patterns and diagnoses of anxiety and oppositional disorder were most frequent. Support was provided for(More)