Dinesh Rakheja

Summary

Affiliation: University of Texas Southwestern Medical Center
Country: USA

Publications

  1. pmc A zebrafish transgenic model of Ewing's sarcoma reveals conserved mediators of EWS-FLI1 tumorigenesis
    Stefanie W Leacock
    Department of Pediatrics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 8534, USA
    Dis Model Mech 5:95-106. 2012
  2. 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
  3. ncbi request reprint Infantile cystinosis
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, TX, USA
    Arch Pathol Lab Med 129:126-7. 2005
  4. ncbi request reprint Extrapleural benign solitary fibrous tumor in the shoulder of a 9-year-old girl: case report and review of the literature
    Dinesh Rakheja
    Department of Pathology, MC 9073, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Pediatr Dev Pathol 7:653-60. 2004
  5. ncbi request reprint Pathologic quiz case: sphenoid sinus mass in a 12-year-old girl. Infrasellar adamantinomatous craniopharyngioma
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, Dallas, USA
    Arch Pathol Lab Med 129:e73-4. 2005
  6. ncbi request reprint Renal metanephric adenoma with previously unreported cytogenetic abnormalities: case report and review of the literature
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, 1935 Motor Street, Dallas, TX 75235, USA
    Pediatr Dev Pathol 8:218-23. 2005
  7. doi request reprint A subset of cranial fasciitis is associated with dysregulation of the Wnt/beta-catenin pathway
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, UT Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Mod Pathol 21:1330-6. 2008
  8. ncbi request reprint Thyroid enlargement, hyperthyroidism, and antibodies to thyroglobulin and thyroid peroxidase in an 11-year-old girl
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas and University of Texas Southwestern Medical Center, Dallas, TX 75235, USA
    Arch Pathol Lab Med 129:e159-61. 2005
  9. 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
  10. ncbi request reprint Increased ratio of saturated to unsaturated C18 fatty acids in colonic adenocarcinoma: implications for cryotherapy and lipid raft function
    Dinesh Rakheja
    Department of Pathology, MC 9073, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Med Hypotheses 65:1120-3. 2005

Collaborators

Detail Information

Publications74

  1. pmc A zebrafish transgenic model of Ewing's sarcoma reveals conserved mediators of EWS-FLI1 tumorigenesis
    Stefanie W Leacock
    Department of Pediatrics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 8534, USA
    Dis Model Mech 5:95-106. 2012
    ..These results indicate that functional targets of EWS-FLI1 that mediate tumorigenesis are conserved from zebrafish to human and provide a novel context in which to study the function of this fusion oncogene...
  2. 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
    ....
  3. ncbi request reprint Infantile cystinosis
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, TX, USA
    Arch Pathol Lab Med 129:126-7. 2005
  4. ncbi request reprint Extrapleural benign solitary fibrous tumor in the shoulder of a 9-year-old girl: case report and review of the literature
    Dinesh Rakheja
    Department of Pathology, MC 9073, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Pediatr Dev Pathol 7:653-60. 2004
    ..In addition, cytogenetic analysis revealed a karyotype unreported in this neoplasm: 46,XX,der(4)t(4;9)(q31.1;q34), del(9)(p22p24),der(9)t(4;9)(q31.1;q34)ins(9;?)(q34;?) (17 cells)/46,XX (3 cells)...
  5. ncbi request reprint Pathologic quiz case: sphenoid sinus mass in a 12-year-old girl. Infrasellar adamantinomatous craniopharyngioma
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, Dallas, USA
    Arch Pathol Lab Med 129:e73-4. 2005
  6. ncbi request reprint Renal metanephric adenoma with previously unreported cytogenetic abnormalities: case report and review of the literature
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, 1935 Motor Street, Dallas, TX 75235, USA
    Pediatr Dev Pathol 8:218-23. 2005
    ..The involvement of the chromosome region 9p24 is particularly interesting because of the recent identification of a tumor suppressor gene, KANK (kidney ankyrin repeat-containing protein), at this locus...
  7. doi request reprint A subset of cranial fasciitis is associated with dysregulation of the Wnt/beta-catenin pathway
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, UT Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Mod Pathol 21:1330-6. 2008
    ..Occasional cases of cranial fasciitis may be associated with FAP and serve as an early indicator of this disease, information that would be important in the early diagnosis of FAP in patients without a family history of polyposis...
  8. ncbi request reprint Thyroid enlargement, hyperthyroidism, and antibodies to thyroglobulin and thyroid peroxidase in an 11-year-old girl
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas and University of Texas Southwestern Medical Center, Dallas, TX 75235, USA
    Arch Pathol Lab Med 129:e159-61. 2005
  9. 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...
  10. ncbi request reprint Increased ratio of saturated to unsaturated C18 fatty acids in colonic adenocarcinoma: implications for cryotherapy and lipid raft function
    Dinesh Rakheja
    Department of Pathology, MC 9073, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Med Hypotheses 65:1120-3. 2005
    ..Dietary or therapeutic interventions targeting lipid rafts may thus be an option for cancer treatment...
  11. ncbi request reprint Pediatric renal cell carcinomas with Xp11.2 rearrangements are immunoreactive for hMLH1 and hMSH2 proteins
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center and University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Pediatr Dev Pathol 8:615-20. 2005
    ..Our study demonstrates that inactivation of the DNA mismatch repair genes hMLH1 and hMSH2 does not appear to play a role in the tumorigenesis of pediatric renal cell carcinomas with Xp11.2 rearrangements...
  12. 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
    ....
  13. 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...
  14. 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...
  15. ncbi request reprint Translocation (4;19)(q35;q13.1)-associated primitive round cell sarcoma: report of a case and review of the literature
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center, and UT Southwestern Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 11:239-44. 2008
    ..Recognition of the histopathologic and cytogenetic features of this entity is necessary to avoid its misdiagnosis as ES/PNET, especially in small biopsy samples...
  16. 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...
  17. ncbi request reprint Immunohistochemical expression of beta-catenin in solitary fibrous tumors
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Arch Pathol Lab Med 129:776-9. 2005
    ..Among mesenchymal tumors, aberrant nuclear localization of beta-catenin is seen in desmoid-type fibromatoses but has not been described for solitary fibrous tumors that may mimic the former lesions, especially in small biopsy samples...
  18. 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
  19. ncbi request reprint Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney
    Dinesh Rakheja
    Department of Pathology, MC 9073, Children s Medical Center of Dallas and the University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA
    Cancer Genet Cytogenet 154:175-9. 2004
    ..A clear cell sarcoma from the kidney of a 12-month-old male child manifested a balanced translocation, t(10;17)(q22;p13). This is the second report of this cytogenetic abnormality in renal clear cell sarcoma...
  20. ncbi request reprint Pathologic quiz case: a woman with right facial nerve paralysis. Endolymphatic sac tumor associated with von Hippel-Lindau disease
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Tex 75390 9073, USA
    Arch Pathol Lab Med 127:1387-8. 2003
  21. ncbi request reprint CLN3P, the Batten disease protein, localizes to membrane lipid rafts (detergent-resistant membranes)
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas and the University of Texas Southwestern Medical Center, Dallas, TX, USA
    Biochem Biophys Res Commun 317:988-91. 2004
    ..In this study, we demonstrate that CLN3P resides on membrane lipid raft domains (detergent-resistant membranes) and provide important new data towards possible functions of the protein...
  22. ncbi request reprint Multilocular thymic cyst associated with mature mediastinal teratoma: a report of 2 cases
    Dinesh Rakheja
    Department of Pathology, The University of Texas Southwestern Medical Center, DallasTex 75235, USA
    Arch Pathol Lab Med 128:227-8. 2004
    ..We describe 2 cases (in a 12-year-old girl and an 11-year-old boy) of acquired thymic cysts occurring in association with cystic teratomas, an association that to our knowledge has not been described previously in the literature...
  23. ncbi request reprint Phosphorylated histone H3, Ki-67, p21, fatty acid synthase, and cleaved caspase-3 expression in benign and atypical granular cell tumors
    Payal Kapur
    Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Arch Pathol Lab Med 131:57-64. 2007
    ..It has been suggested that a GCT be classified as atypical when 1 or 2 of these features are seen and as malignant when 3 or more of these are present. In our practice, we do not classify GCTs as malignant in the absence of metastasis...
  24. 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
    J Urol 188:1500-5. 2012
    ..Thus, we correlated preoperative computerized tomography and pathological lymph node findings to establish a radiological criterion for pathological lymph node enlargement...
  25. 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 Pediatric Urology, Children s Medical Center of Dallas, Dallas, Texas Division of Urologic Oncology, University of Texas Southwestern Medical Center, Dallas, Texas
    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...
  26. 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...
  27. doi request reprint Activated NOTCH2 is overexpressed in hepatoblastomas: an immunohistochemical study
    Jason B Litten
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA
    Pediatr Dev Pathol 14:378-83. 2011
    ..In the hepatoblastoma sinusoidal vasculature, we saw NOTCH3 and NOTCH1 expression. These observations have potential implications with regard to therapeutic targeting of the NOTCH signaling pathway in hepatoblastomas...
  28. 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...
  29. 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...
  30. ncbi request reprint "Proximal-type" and classic epithelioid sarcomas represent a clinicopathologic continuum: case report
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, 1935 Motor Street, Dallas, TX 75235, USA
    Pediatr Dev Pathol 8:105-14. 2005
    ..Based on the morphologic and cytogenetic findings in this case, we suggest that the proximal-type and the classic epithelioid sarcomas are not distinct entities but represent a continuum...
  31. ncbi request reprint Dysmorphic villi mimicking partial mole in a case with del(18)(q21)
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas, 1935 Motor Street, Dallas, TX 75235, USA
    Pediatr Dev Pathol 7:546-8. 2004
    ..These histopathologic changes have been previously described in numerical chromosomal aberrations like triploidy, tetraploidy, and trisomies, but not in structural chromosomal abnormalities...
  32. 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...
  33. 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...
  34. 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...
  35. 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...
  36. 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...
  37. 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...
  38. ncbi request reprint Absence of expression of SMARCB1/INI1 in malignant rhabdoid tumors of the central nervous system, kidneys and soft tissue: an immunohistochemical study with implications for diagnosis
    Ellen Sigauke
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Mod Pathol 19:717-25. 2006
    ..Immunohistochemistry to assess expression of SMARCB1/INI1 may be useful in the diagnosis of rhabdoid tumors of the CNS, kidneys and soft tissue...
  39. doi request reprint Atypical and malignant hidradenomas: a histological and immunohistochemical study
    Rosalynn M Nazarian
    Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
    Mod Pathol 22:600-10. 2009
    ..A tumor with Ki-67>11% and/or phosphorylated histone H3>0.7% would likely be a malignant rather than an atypical hidradenoma. The infrequent Her2/neu overexpression in hidradenocarcinoma suggests its limited therapeutic role...
  40. ncbi request reprint Hepatic mesenchymal hamartoma with translocation involving chromosome band 19q13.4: a recurrent abnormality
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center of Dallas and the University of Texas Southwestern Medical Center, 1935 Motor Street, Dallas, TX 75235, USA
    Cancer Genet Cytogenet 153:60-3. 2004
    ..4). This is the fifth description of a cytogenetic abnormality in mesenchymal hamartoma and is similar to the four cases reported previously in that one of the breakpoints involved chromosome band 19q13.4...
  41. 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...
  42. ncbi request reprint Primary mucinous adenocarcinoma of the thymus: a case report and review of the literature
    Payal Kapur
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Arch Pathol Lab Med 130:201-4. 2006
    ..Morphologic and immunophenotypic similarities to mucinous adenocarcinomas of the gastrointestinal tract can pose diagnostic challenges for surgical pathologists, especially in small biopsy specimens...
  43. ncbi request reprint Fatty acid synthase expression in cutaneous melanocytic neoplasms
    Payal Kapur
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Mod Pathol 18:1107-12. 2005
    ..This may represent persistence of or regression to a fetal phenotype since normal fetal tissues are known to express high levels of fatty acid synthase...
  44. 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...
  45. doi request reprint Intratumoral expression level of epidermal growth factor receptor and cytokeratin 5/6 is significantly associated with nodal and distant metastases in patients with basal-like triple-negative breast carcinoma
    Lisa M Sutton
    Dept of Pathology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390 9073, USA
    Am J Clin Pathol 134:782-7. 2010
    ..Furthermore, EGFR targeted therapy may be potentially useful in the treatment of basal-like TN breast cancer...
  46. ncbi request reprint Characterization of inflammation in syphilitic villitis and in villitis of unknown etiology
    Payal Kapur
    Department of Pathology, The University of Texas Southwestern Medical School, Dallas, TX, USA
    Pediatr Dev Pathol 7:453-8; discussion 421. 2004
    ..These data indicate that the immune response in both syphilitic villitis and VUE is similar, raising the possibility of a similar immunopathogenetic pathway...
  47. 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...
  48. 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...
  49. ncbi request reprint Rosette formation within a proliferative nodule of an atypical combined melanocytic nevus in an adult
    Mai P Hoang
    Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, 75390 9073, USA
    Am J Dermatopathol 25:35-9. 2003
    ..We also retrospectively examined 78 congenital nevi of 65 pediatric patients at our institution. Rosette formation was not seen in any of these cases...
  50. doi request reprint The aging kidney
    Xin J Zhou
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
    Kidney Int 74:710-20. 2008
    ..This review summarizes the structural and functional changes of the aging kidney and highlights the advances made in our understanding of the renal aging process...
  51. 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...
  52. 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
    ....
  53. pmc DNA methylation analysis reveals distinct methylation signatures in pediatric germ cell tumors
    James F Amatruda
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    BMC Cancer 13:313. 2013
    ..Aberrant DNA methylation is a prominent feature of many cancers, and may be especially relevant in germ cell tumors (GCTs) due to the extensive epigenetic reprogramming that occurs in the germ line during normal development...
  54. 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...
  55. pmc Mutation in the type IB bone morphogenetic protein receptor Alk6b impairs germ-cell differentiation and causes germ-cell tumors in zebrafish
    Joanie C Neumann
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Proc Natl Acad Sci U S A 108:13153-8. 2011
    ..These results indicate a germ-cell-autonomous role for BMP signal transduction in germ-cell differentiation, and highlight the importance of the BMP pathway in human GCTs...
  56. doi request reprint A novel SOX9 mutation, 972delC, causes 46,XY sex-reversed campomelic dysplasia with nephrocalcinosis, urolithiasis, and dysgerminoma
    Nicholas G Cost
    Department of Urology, University of Texas Southwestern Medical Center and Children s Medical Center at Dallas, Dallas, TX 75390 9110, USA
    J Pediatr Surg 44:451-4. 2009
    ..This first reported case of dysgerminoma in a sex-reversed patient with CD who also had urolithiasis stresses the importance of prophylactic gonadectomy and urologic evaluations in this susceptible population...
  57. ncbi request reprint Helicobacter heilmannii infection in a child
    Dennis C Wooten
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Tex 75235, USA
    Arch Pathol Lab Med 128:1461-2. 2004
  58. ncbi request reprint Pathologic quiz case: laryngeal lesion in an elderly man. Verrucous carcinoma of the larynx
    Payal Kapur
    Department of Pathology, University of Texas Southwestern Medical Center, Dallas 75390, USA
    Arch Pathol Lab Med 129:115-6. 2005
  59. 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
  60. ncbi request reprint Extrahepatic biliary atresia demonstrates abnormal persistence of HES1 protein in neonatal biliary epithelium: an immunohistochemical study
    Dinesh Rakheja
    Department of Pathology, Children s Medical Center and University of Texas Southwestern Medical Center, Dallas, USA
    Pediatr Dev Pathol 9:98-102. 2006
    ..Further studies should be performed to evaluate the role of HES1 immunohistochemistry as a diagnostic tool in extrahepatic biliary atresia...
  61. ncbi request reprint Pathologic quiz case. Cystic and solid ovarian tumor in a 43-year-old woman
    Dinesh Rakheja
    Department of Pathology, University of Texas Southwestern Medcial Center, Dallas, USA
    Arch Pathol Lab Med 126:1123-4. 2002
  62. ncbi request reprint Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas Children's Medical Center
    Payal Kapur
    Department of Pathology, Children s Medical Center Dallas, Texas, USA
    J Am Acad Dermatol 56:290-4. 2007
    ....
  63. 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...
  64. 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...
  65. ncbi request reprint Pathologic quiz case: upper aerodigestive tract mass in a 90-year-old woman
    Dinesh Rakheja
    Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Arch Pathol Lab Med 127:e279-80. 2003
  66. ncbi request reprint Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review
    Ellen Sigauke
    Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    Lab Invest 83:1543-54. 2003
    ..We review the clinical features, the diagnostic protocol including expanded neonatal screening, the treatment, and the biochemical and molecular basis of CPT II deficiency...
  67. ncbi request reprint Intratubular embryonal carcinoma
    Dinesh Rakheja
    Division of Anatomic Pathology, The University of Texas Southwestern Medical Center, Dallas, TX 75390 9073, USA
    Arch Pathol Lab Med 126:487-90. 2002
    ..It was negative for cytokeratin 20, p21, and alpha-fetoprotein. These findings are strongly supportive of the opinion that intratubular embryonal carcinoma is the precursor of invasive embryonal carcinoma...
  68. doi request reprint Unsuspected, disseminated coccidioidomycosis without maternofetal morbidity diagnosed by placental examination: case report and review of the literature
    Christina A Arnold
    Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas 75390 9073, USA
    Clin Infect Dis 46:e119-23. 2008
    ....
  69. doi request reprint Establishment and characterization of a cancer cell line derived from an aggressive childhood liver tumor
    Tina T L Chen
    Hamon Center for Therapeutic Oncology Research, University of Texas Southwestern Medical Center, Dallas, Texas, USA
    Pediatr Blood Cancer 53:1040-7. 2009
    ..The scarcity of adequate cell models has limited our understanding of this tumor. Here we describe and characterize a new human liver tumor cell line, Hep293TT, derived from an aggressive childhood hepatoblastoma...
  70. 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
  71. ncbi request reprint Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: further evidence that CLN3P has anti-apoptotic properties
    Srinivas B Narayan
    Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Mol Genet Metab 88:178-83. 2006
    ..CLN3P significantly increased the survival rate of the SH-SY5Y cells in this system. This study provides additional evidence that the function of CLN3P is related to preventing neuronal apoptosis...
  72. ncbi request reprint CLN3P, the Batten's disease protein, is a novel palmitoyl-protein Delta-9 desaturase
    Srinivas B Narayan
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Ann Neurol 60:570-7. 2006
    ..Although the gene for the disorder was cloned more than a decade ago, the function of the encoded protein, CLN3P, has not been defined thus far...
  73. 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...
  74. pmc Targeting the apoptotic machinery in pancreatic cancers using small-molecule antagonists of the X-linked inhibitor of apoptosis protein
    Collins A Karikari
    The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins University School of Medicine, CRB2 Room 345, 1550 Orleans Street, Baltimore, MD 21231, USA
    Mol Cancer Ther 6:957-66. 2007
    ..These preclinical results suggest that targeting of the apoptotic machinery in pancreatic cancers with XAntags is a promising therapeutic option that warrants further evaluation...