Abstract

This translational research proposal addresses the epidemiology, biology and treatment of children with the related preleukemic conditions juvenile chronic myelogenous leukemia and bone marrow monosomy 7 syndrome (JCML/Mo7). These disorders share a number of major clinical features that suggest they are related pathogenically; however, the precise relationship between them is uncertain. Treatment results in children with JCML/Mo7 have been dismal even with allogeneic bone marrow transplantation. This proposal is based upon recent epidemiological findings linking specific obstetric and parental occupational risk factors with the development of myeloid leukemia during infancy; upon the observation that some children with JCML have show clinical responses to the differentiating agent cis retinoic acid; and upon molecular genetic and biochemical studies that implicate deregulated signaling through the p21 ras family of proteins in the pathogenesis of these malignant myeloid disorders. We will test the hypothesis that JCML/Mo7 are associated with genetic and acquired risk factors which correlate with specific molecular and cytogenetic alterations in patient bone marrows, and that these molecular markers will be of prognostic value and can be used to measure responses to treatment.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
5M01RR001271-17
Application #
6280855
Study Section
Project Start
1997-12-01
Project End
1998-11-30
Budget Start
1997-10-01
Budget End
1998-09-30
Support Year
17
Fiscal Year
1998
Total Cost
Indirect Cost

  Institution

Name
University of California San Francisco
Department
Type
DUNS #
073133571
City
San Francisco
State
CA
Country
United States
Zip Code
94143

  Publications

Peyvandi, Shabnam; Kim, Hosung; Lau, Joanne et al. (2018) The association between cardiac physiology, acquired brain injury, and postnatal brain growth in critical congenital heart disease. J Thorac Cardiovasc Surg 155:291-300.e3
Peyvandi, Shabnam; Chau, Vann; Guo, Ting et al. (2018) Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease. J Am Coll Cardiol 71:1986-1996
Jacobson, Denise L; Lindsey, Jane C; Coull, Brent A et al. (2018) The Association of Fat and Lean Tissue With Whole Body and Spine Bone Mineral Density Is Modified by HIV Status and Sex in Children and Youth. Pediatr Infect Dis J 37:71-77
Wahlstrom, Justin; Patel, Kiran; Eckhert, Erik et al. (2017) Transplacental maternal engraftment and posttransplantation graft-versus-host disease in children with severe combined immunodeficiency. J Allergy Clin Immunol 139:628-633.e10
Ward, Shan L; Turpin, Autumn; Spicer, Aaron C et al. (2017) Long-Term Pulmonary Function and Quality of Life in Children After Acute Respiratory Distress Syndrome: A Feasibility Investigation. Pediatr Crit Care Med 18:e48-e55
Ruan, Alexandra; Tobin, Nicole H; Mulligan, Kathleen et al. (2016) Brief Report: Macrophage Activation in HIV-Infected Adolescent Males Contributes to Differential Bone Loss by Sex: Adolescent Trials Network Study 021. J Acquir Immune Defic Syndr 72:372-5
Desikan, Rahul S; Barkovich, A James (2016) Malformations of cortical development. Ann Neurol 80:797-810
Peyvandi, Shabnam; De Santiago, Veronica; Chakkarapani, Elavazhagan et al. (2016) Association of Prenatal Diagnosis of Critical Congenital Heart Disease With Postnatal Brain Development and the Risk of Brain Injury. JAMA Pediatr 170:e154450
Harmatz, Paul R; Mengel, Karl Eugen; Giugliani, Roberto et al. (2015) Longitudinal analysis of endurance and respiratory function from a natural history study of Morquio A syndrome. Mol Genet Metab 114:186-94
Aweeka, F T; Hu, C; Huang, L et al. (2015) Alteration in cytochrome P450 3A4 activity as measured by a urine cortisol assay in HIV-1-infected pregnant women and relationship to antiretroviral pharmacokinetics. HIV Med 16:176-83

Showing the most recent 10 out of 502 publications