Dinesh Rakheja

Summary

Publications

  1. ncbi request reprint Long-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical review
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Lab Invest 82:815-24. 2002
  2. ncbi request reprint Biochemical abnormality associated with Smith-Lemli-Opitz syndrome in an infant with features of Rutledge multiple congenital anomaly syndrome confirms that the latter is a variant of the former
    Dinesh Rakheja
    Department of Pathology, Mail Code 9073, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Pediatr Dev Pathol 6:270-7. 2003
  3. ncbi request reprint A severely affected female infant with x-linked dominant chondrodysplasia punctata: a case report and a brief review of the literature
    Dinesh Rakheja
    Department of Pathology, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390 9073, USA
    Pediatr Dev Pathol 10:142-8. 2007
  4. doi request reprint Correlation between preoperative staging computerized tomography and pathological findings after nodal sampling in children with Wilms tumor
    Jessica D Lubahn
    Division of Pediatric Urology, Children s Medical Center of Dallas and University of Texas Southwestern Medical Center, Dallas, Texas 45229, USA
    J Urol 188:1500-4. 2012
  5. doi request reprint Pathological review of Wilms tumor nephrectomy specimens and potential implications for nephron sparing surgery in Wilms tumor
    Nicholas G Cost
    Division of Urologic Oncology, University of Texas Southwestern Medical Center, Dallas, Texas 45229, USA
    J Urol 188:1506-10. 2012
  6. ncbi request reprint Single institution experience with Tru-Cut renal mass biopsy for diagnosing Wilms tumor
    Nicholas G Cost
    Division of Pediatric Urology, Children s Medical Center of Dallas, Dallas, Texas, USA
    Urol J 10:780-3. 2013
  7. pmc Glucose metabolism via the pentose phosphate pathway, glycolysis and Krebs cycle in an orthotopic mouse model of human brain tumors
    Isaac Marin-Valencia
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA
    NMR Biomed 25:1177-86. 2012
  8. doi request reprint Immunohistochemical expression of GLUT1 and its correlation with unfavorable histology and TP53 codon 72 polymorphism in Wilms tumors
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 15:286-92. 2012
  9. doi request reprint Immunohistochemical expression of embryonic stem cell markers in malignant rhabdoid tumors
    Jeremy Deisch
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Pediatr Dev Pathol 14:353-9. 2011
  10. doi request reprint Isocitrate dehydrogenase 1/2 mutational analyses and 2-hydroxyglutarate measurements in Wilms tumors
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center and UT Southwestern Medical Center, Dallas, Texas 75390, USA
    Pediatr Blood Cancer 56:379-83. 2011

Collaborators

Detail Information

Publications32

  1. ncbi request reprint Long-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical review
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Lab Invest 82:815-24. 2002
    ..In this review we discuss the biochemical and molecular basis, clinical features, diagnosis, and management of long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency...
  2. ncbi request reprint Biochemical abnormality associated with Smith-Lemli-Opitz syndrome in an infant with features of Rutledge multiple congenital anomaly syndrome confirms that the latter is a variant of the former
    Dinesh Rakheja
    Department of Pathology, Mail Code 9073, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Pediatr Dev Pathol 6:270-7. 2003
    ..The biochemical data showing an excess of 7-dehydrocholesterol and low cholesterol in the liver tissue of our case supports this viewpoint...
  3. ncbi request reprint A severely affected female infant with x-linked dominant chondrodysplasia punctata: a case report and a brief review of the literature
    Dinesh Rakheja
    Department of Pathology, The University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390 9073, USA
    Pediatr Dev Pathol 10:142-8. 2007
    ..We present this case as an illustration of an unusually severe manifestation of this disorder in a female, with additional unusual features including lack of skin manifestations and apparent bilateral symmetry of the skeletal findings...
  4. doi request reprint Correlation between preoperative staging computerized tomography and pathological findings after nodal sampling in children with Wilms tumor
    Jessica D Lubahn
    Division of Pediatric Urology, Children s Medical Center of Dallas and University of Texas Southwestern Medical Center, Dallas, Texas 45229, USA
    J Urol 188:1500-4. 2012
    ..Thus, we correlated preoperative computerized tomography and pathological lymph node findings to establish a radiological criterion for pathological lymph node enlargement...
  5. doi request reprint Pathological review of Wilms tumor nephrectomy specimens and potential implications for nephron sparing surgery in Wilms tumor
    Nicholas G Cost
    Division of Urologic Oncology, University of Texas Southwestern Medical Center, Dallas, Texas 45229, USA
    J Urol 188:1506-10. 2012
    ..With the increasing use of nephron sparing surgery in Wilms tumor, we reviewed pathological features from Wilms tumor radical nephrectomy specimens to determine the potential efficacy of a nephron sparing approach...
  6. ncbi request reprint Single institution experience with Tru-Cut renal mass biopsy for diagnosing Wilms tumor
    Nicholas G Cost
    Division of Pediatric Urology, Children s Medical Center of Dallas, Dallas, Texas, USA
    Urol J 10:780-3. 2013
    ..To evaluate the efficacy of needle biopsy for diagnosing Wilms tumor (WT) before chemotherapy...
  7. pmc Glucose metabolism via the pentose phosphate pathway, glycolysis and Krebs cycle in an orthotopic mouse model of human brain tumors
    Isaac Marin-Valencia
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA
    NMR Biomed 25:1177-86. 2012
    ..Taken together, these data demonstrate that (13) C-labeled glucose can be used in orthotopic mouse models to study tumor metabolism in vivo and to ascertain new metabolic targets for cancer diagnosis and therapy...
  8. doi request reprint Immunohistochemical expression of GLUT1 and its correlation with unfavorable histology and TP53 codon 72 polymorphism in Wilms tumors
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 15:286-92. 2012
    ....
  9. doi request reprint Immunohistochemical expression of embryonic stem cell markers in malignant rhabdoid tumors
    Jeremy Deisch
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Pediatr Dev Pathol 14:353-9. 2011
    ..No tumor showed any significant OCT3/4, NANOG, or TCL1 expression. Our results suggest that malignant rhabdoid tumors may arise from and/or share features with embryonic stem cells or germ cells...
  10. doi request reprint Isocitrate dehydrogenase 1/2 mutational analyses and 2-hydroxyglutarate measurements in Wilms tumors
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center and UT Southwestern Medical Center, Dallas, Texas 75390, USA
    Pediatr Blood Cancer 56:379-83. 2011
    ....
  11. pmc 2-hydroxyglutarate detection by magnetic resonance spectroscopy in IDH-mutated patients with gliomas
    Changho Choi
    Advanced Imaging Research Center, University of Texas Southwestern Medical Center, Dallas, Texas, USA
    Nat Med 18:624-9. 2012
    ..Detection of 2HG correlated with mutations in IDH1 or IDH2 and with increased levels of D-2HG by mass spectrometry of the resected tumors. Noninvasive detection of 2HG may prove to be a valuable diagnostic and prognostic biomarker...
  12. ncbi request reprint Precholesterol sterols accumulate in lipid rafts of patients with Smith-Lemli-Opitz syndrome and X-linked dominant chondrodysplasia punctata
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 11:128-32. 2008
    ..We suggest that such alterations in the lipid raft sterol environment may affect the biology of cells and the development of fetuses with cholesterol biosynthetic disorders...
  13. doi request reprint Papillary thyroid carcinoma shows elevated levels of 2-hydroxyglutarate
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Tumour Biol 32:325-33. 2011
    ..Further studies should clarify if elevated 2-hydroxyglutarate in PTC may be useful as cancer biomarker and evaluate the role of 2-hydroxyglutarate in cancer biology...
  14. doi request reprint Immunoexpression of SALL4 in Wilms tumors and developing kidney
    Jeremy Deisch
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Pathol Oncol Res 17:639-44. 2011
    ..The absence of SALL4 staining in nephrogenic rests, the presumed precursors of Wilms tumors, is intriguing and suggests that Wilms tumors have a pluripotency quality that may be lacking in nephrogenic rests...
  15. doi request reprint IDH mutations in acute myeloid leukemia
    Dinesh Rakheja
    Department of Pathology, The University of Texas Southwestern Medical Center and Children s Medical Center, Dallas, TX, USA
    Hum Pathol 43:1541-51. 2012
    ..These data suggest that IDH1/2 mutations constitute a distinct mutational class in acute myeloid leukemia, which affects the epigenetic state, an important consideration for the development of therapeutic agents...
  16. doi request reprint Oncologic outcomes of partial versus radical nephrectomy for unilateral Wilms tumor
    Nicholas G Cost
    Division of Pediatric Urology, University of Texas, Dallas, TX, USA
    Pediatr Blood Cancer 58:898-904. 2012
    ..Based on recent data demonstrating that renal preserving surgery decreases the likelihood of chronic renal disease and associated co-morbidities, we analyzed oncologic outcomes of patients after partial nephrectomy (PN) for UWT...
  17. ncbi request reprint The function of CLN3P, the Batten disease protein
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical Center and Children s Medical Center, MC 9073, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Mol Genet Metab 93:269-74. 2008
  18. doi request reprint A potential role for targeted therapy in a subset of metastasizing adnexal carcinomas
    Dora Dias-Santagata
    Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA
    Mod Pathol 24:974-82. 2011
    ..Targeted therapy including PI3K pathway inhibitors might be a potential treatment for rare cases of adnexal carcinomas with metastases...
  19. doi request reprint Immunohistochemical expression of neural cell adhesion molecule in Wilms tumors, nephrogenic rests, and fetal and postnatal renal cortices
    Payal Kapur
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 14:16-9. 2011
    ..It is not known if these persistent NCAM-positive interstitial cells might give rise to adult Wilms tumors and cases of pediatric Wilms tumors not associated with nephrogenic rests...
  20. doi request reprint Fetal akinesia deformation sequence and neuroaxonal dystrophy without PLA2G6 mutation
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 13:492-6. 2010
    ..In addition, placental examination showed α-fetoprotein-positive, eosinophilic, globular inclusions in the cytoplasm of a few villous macrophages. The significance of this novel histologic finding is unclear...
  21. pmc IL-21 promotes the production of anti-DNA IgG but is dispensable for kidney damage in lyn-/- mice
    Toni Gutierrez
    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390 8884, USA
    Eur J Immunol 43:382-93. 2013
    ..These studies identify a specific role for IL-21 in the class switching of anti-DNA B cells and demonstrate that neither IL-21 nor anti-DNA IgG is required for kidney damage in lyn(-/-) mice...
  22. ncbi request reprint Juvenile neuronal ceroid-lipofuscinosis (Batten disease): a brief review and update
    Dinesh Rakheja
    Department of Pathology, UT Southwestern Medical Center and Children s Medical Center, Dallas, TX 75390, USA
    Curr Mol Med 7:603-8. 2007
    ..In this review, following an introduction to the neuronal ceroid-lipofuscinoses, we provide a brief overview and an update of the most recent research in JNCL, specifically that related to the function of CLN3P...
  23. doi request reprint Proliferative nodules arising within congenital melanocytic nevi: a histologic, immunohistochemical, and molecular analyses of 43 cases
    Pushkar A Phadke
    Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA
    Am J Surg Pathol 35:656-69. 2011
    ..Although numerous mutations are detected in the samples, the diagnostic use of molecular analysis in this regard is limited...
  24. ncbi request reprint Isocitrate dehydrogenase 1/2 mutational analyses and 2-hydroxyglutarate measurements in Wilms tumors
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center and UT Southwestern Medical Center, Dallas, Texas
    Pediatr Blood Cancer 56:379-83. 2011
    ....
  25. pmc Oxidation of alpha-ketoglutarate is required for reductive carboxylation in cancer cells with mitochondrial defects
    Andrew R Mullen
    Children s Medical Center Research Institute, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390 8502, USA
    Cell Rep 7:1679-90. 2014
    ..The data demonstrate that reductive carboxylation requires bidirectional AKG metabolism along oxidative and reductive pathways, with the oxidative pathway producing reducing equivalents used to operate IDH in reverse. ..
  26. doi request reprint Embryonal rhabdomyosarcoma of the auricle in a child
    Emily Crozier
    Department of Otolaryngology Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas 75390 9035, USA
    Pediatr Int 54:945-7. 2012
    ..The final diagnosis was embryonal rhabdomyosarcoma. Although rare, otolaryngologists, pediatricians, and radiologists need to consider rhabdomyosarcoma in the differential diagnosis of auricle mass in children...
  27. doi request reprint Wilms tumor in a child with L-2-hydroxyglutaric aciduria
    Robert E Rogers
    1Department of Pathology, Children s Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 13:408-11. 2010
    ..This observation potentially widens the tumor spectrum in this metabolic disorder and may lead to further insight into the relationship between L-2-hydroxyglutaric acid and cellular transformation...
  28. doi request reprint Highly active antiretroviral therapy does not affect mitochondrial beta-oxidation of fatty acids: an in vitro study in fibroblasts
    Yasmeen M Butt
    Department of Pathology, Children s Medical Center, USA
    Pediatr Dev Pathol 11:35-8. 2008
    ..Therefore, an altered fatty acid oxidation may not be the mechanism for the reported increased risk of preeclampsia in HIV-infected pregnant women on HAART...
  29. ncbi request reprint Pediatric pathology: a case report highlighting its singular niche
    Dinesh Rakheja
    University of Texas Southwestern Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 6:110-1. 2003
  30. ncbi request reprint Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical School, 5323 Harry Hines Boulevard, Dallas, TX 75390 9073, USA
    Pediatr Dev Pathol 6:88-93. 2003
    ..It is possible that the CFTR gene may play a previously unrecognized role in CNS development. Alternatively, this CNS abnormality may be acquired due to the metabolic and electrolyte imbalances that characteristically occur in CF...
  31. ncbi request reprint Hydrolethalus syndrome, in contrast to Smith-Lemli-Opitz syndrome, is not due to a defect in post-squalene cholesterol biosynthesis: a case report
    Dinesh Rakheja
    Am J Med Genet A 129:212-3. 2004
  32. ncbi request reprint Human placenta metabolizes fatty acids: implications for fetal fatty acid oxidation disorders and maternal liver diseases
    Prem Shekhawat
    Departments of Pediatric, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Am J Physiol Endocrinol Metab 284:E1098-105. 2003
    ..Thus human placenta derives energy from fatty acid oxidation, providing a potential explanation for the association of fetal fatty acid oxidation disorders with maternal liver diseases in pregnancy...