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Gene Review

FND  -  frontonasal dysplasia

Homo sapiens

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Disease relevance of FND

  • One hundred forty-two patients with previously untreated stage IV indolent lymphoma were evaluable (73 on FND; 69 on ATT) [1].
  • ATT was associated with a substantially higher rate of grade 3-4 toxicities than FND [1].
  • To our knowledge, multiple pericallosal lipomas in association with FND have not been described before [2].
  • Fourteen patients (19%) developed Grade 3-4 infections, the majority during FND therapy with neutropenia and/or accompanied by CD4 counts < 200/microL [3].
  • After the administration of the FND regimen, thrombocytopenia developed, and the platelet count did not recover to previous levels [4].
 

Psychiatry related information on FND

 

High impact information on FND

 

Chemical compound and disease context of FND

 

Biological context of FND

 

Anatomical context of FND

  • Pericallosal lipomas in cases of FND are of the tubulonodular type; they have been reported only in relation to the anterior part of the corpus callosum [2].
  • FND is an active regimen for the treatment of indolent B- and T-cell malignancies in Chinese patients, with results comparable with Western patients with similar indolent lymphomas [15].
  • Lymph node treatment protocol comprised 51 functional ipsilateral neck dissections (FNDs), 9 bilateral FNDs, 1 radical ipsilateral dissection, and 1 radical ipsilateral and contralateral FND [16].
  • Excessive medial orbital wall protrusion (mean, 145% of normal in FND and 177% in CFND), shortened zygomatic arch lengths (mean, 94% of normal in FND and 91% in CFND), and reduced cephalic lengths (mean, 96% of normal in FND and 83% in CFND) were all observed [17].
  • Frontonasal dysplasia, Poland anomaly and unilateral hypoplasia of lower limb: report on a male patient [18].
 

Associations of FND with chemical compounds

  • In a separate trial, FM plus dexamethasone (Decadron; Merck and Co., Inc.; Whitehouse Station, NJ, http://www.merck.com), FND, plus concurrent rituximab produced a CR rate of 92% [19].
  • Acute treatment with methotrexate results in frontonasal dysplasia (median facial clefts) [20].
  • Verapamil and nifedipine partially block [Ca2+]i influx by as much as 50% suggesting the slow Ca2+ (voltage independent) channels are partially responsible for the increased [Ca2+]i seen following FNDP [21].
 

Other interactions of FND

 

Analytical, diagnostic and therapeutic context of FND

References

  1. Fludarabine, mitoxantrone, dexamethasone (FND) compared with an alternating triple therapy (ATT) regimen in patients with stage IV indolent lymphoma. Tsimberidou, A.M., McLaughlin, P., Younes, A., Rodriguez, M.A., Hagemeister, F.B., Sarris, A., Romaguera, J., Hess, M., Smith, T.L., Yang, Y., Ayala, A., Preti, A., Lee, M.S., Cabanillas, F. Blood (2002) [Pubmed]
  2. Multiple pericallosal lipomas in two siblings with frontonasal dysplasia. Alzoum, M.A., Alorainy, I.A., Al Husain, M., Al Ruhaimi, K. AJNR. American journal of neuroradiology. (2002) [Pubmed]
  3. Immunosuppression and infectious complications in patients with stage IV indolent lymphoma treated with a fludarabine, mitoxantrone, and dexamethasone regimen. Tsimberidou, A.M., Younes, A., Romaguera, J., Hagemeister, F.B., Rodriguez, M.A., Feng, L., Ayala, A., Smith, T.L., Cabanillas, F., McLaughlin, P. Cancer (2005) [Pubmed]
  4. Successful treatment of lymphoma with fludarabine combined with rituximab after immune thrombocytopenia induced by fludarabine. Tsutsumi, Y., Kanamori, H., Minami, H., Musashi, M., Fukushima, A., Ehira, N., Yamato, H., Obara, S., Ogura, N., Tanaka, J., Asaka, M., Imamura, M., Masauzi, N. Ann. Hematol. (2005) [Pubmed]
  5. The syndrome of frontonasal dysplasia, spastic paraplegia, mental retardation and blindness: a case report with CT scan findings and review of literature. Dubey, S.P., Garap, J.P. Int. J. Pediatr. Otorhinolaryngol. (2000) [Pubmed]
  6. The origin of EFNB1 mutations in craniofrontonasal syndrome: frequent somatic mosaicism and explanation of the paucity of carrier males. Twigg, S.R., Matsumoto, K., Kidd, A.M., Goriely, A., Taylor, I.B., Fisher, R.B., Hoogeboom, A.J., Mathijssen, I.M., Lourenco, M.T., Morton, J.E., Sweeney, E., Wilson, L.C., Brunner, H.G., Mulliken, J.B., Wall, S.A., Wilkie, A.O. Am. J. Hum. Genet. (2006) [Pubmed]
  7. Mutations of ephrin-B1 (EFNB1), a marker of tissue boundary formation, cause craniofrontonasal syndrome. Twigg, S.R., Kan, R., Babbs, C., Bochukova, E.G., Robertson, S.P., Wall, S.A., Morriss-Kay, G.M., Wilkie, A.O. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  8. Myelodysplasia and acute myeloid leukemia following therapy for indolent lymphoma with fludarabine, mitoxantrone, and dexamethasone (FND) plus rituximab and interferon alpha. McLaughlin, P., Estey, E., Glassman, A., Romaguera, J., Samaniego, F., Ayala, A., Hayes, K., Maddox, A.M., Preti, H.A., Hagemeister, F.B. Blood (2005) [Pubmed]
  9. Fludarabine, mitoxantrone, and dexamethasone: an effective new regimen for indolent lymphoma. McLaughlin, P., Hagemeister, F.B., Romaguera, J.E., Sarris, A.H., Pate, O., Younes, A., Swan, F., Keating, M., Cabanillas, F. J. Clin. Oncol. (1996) [Pubmed]
  10. Syndromes with cephaloceles. Cohen, M.M., Lemire, R.J. Teratology (1982) [Pubmed]
  11. Phase I study of the combination of fludarabine, mitoxantrone, and dexamethasone in low-grade lymphoma. McLaughlin, P., Hagemeister, F.B., Swan, F., Cabanillas, F., Pate, O., Romaguera, J.E., Rodriguez, M.A., Redman, J.R., Keating, M. J. Clin. Oncol. (1994) [Pubmed]
  12. The spectrum of frontonasal dysplasia in an inbred pedigree. Moreno Fuenmayor, H. Clin. Genet. (1980) [Pubmed]
  13. Low-grade non-Hodgkin's lymphoma--development of a new effective combination regimen (fludarabine, mitoxantrone and dexamethasone; FND). Keating, M.J., McLaughlin, P., Cabanillas, F. European journal of cancer care. (1997) [Pubmed]
  14. Frontonasal dysplasia, coronal craniosynostosis, pre- and postaxial polydactyly and split nails: a new autosomal dominant mutant with reduced penetrance and variable expression? Kwee, M.L., Lindhout, D. Clin. Genet. (1983) [Pubmed]
  15. Fludarabine, mitoxantrone and dexamethasone in the treatment of indolent B- and T-cell lymphoid malignancies in Chinese patients. Ma, S.Y., Au, W.Y., Chim, C.S., Lie, A.K., Lam, C.C., Tse, E., Leung, A.Y., Liang, R., Kwong, Y.L. Br. J. Haematol. (2004) [Pubmed]
  16. Supracricoid laryngectomy with cricohyoidopexy (CHP) in the treatment of laryngeal cancer: a functional and oncologic experience. de Vincentiis, M., Minni, A., Gallo, A. Laryngoscope (1996) [Pubmed]
  17. Frontonasal and craniofrontonasal dysplasia: preoperative quantitative description of the cranio-orbito-zygomatic region based on computed and conventional tomography. Moffat, S.M., Posnick, J.C., Pron, G.E., Armstrong, D.C. The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. (1994) [Pubmed]
  18. Frontonasal dysplasia, Poland anomaly and unilateral hypoplasia of lower limb: report on a male patient. Guion-Almeida, M.L., da Silva Lopes, V.L. Clin. Dysmorphol. (2003) [Pubmed]
  19. The role of mitoxantrone in the treatment of indolent lymphomas. Hagemeister, F., Cabanillas, F., Coleman, M., Gregory, S.A., Zinzani, P.L. Oncologist (2005) [Pubmed]
  20. Teratogens and craniofacial malformations: relationships to cell death. Sulik, K.K., Cook, C.S., Webster, W.S. Development (1988) [Pubmed]
  21. Alteration in Ca2+ homeostasis by a trauma peptide. Hoyt, D.B., Ozkan, A.N., Frevert, J., Junger, W.G., Loomis, W.H. J. Surg. Res. (1991) [Pubmed]
  22. Syndromal frontonasal dysostosis in a child with a complex translocation involving chromosomes 3, 7, and 11. Stevens, C.A., Qumsiyeh, M.B. Am. J. Med. Genet. (1995) [Pubmed]
  23. Severe acrocallosal syndrome or acromelic frontonasal dysplasia? Verloes, A. Am. J. Med. Genet. (1994) [Pubmed]
  24. Frontonasal dysplasia in the Klippel-Feil syndrome: a new associated malformation. Fragoso, R., Cid-García, A., Hernández, A., Nazará, Z., Cantú, J.M. Clin. Genet. (1982) [Pubmed]
  25. Aggressive subcutaneous panniculitis-like T-cell lymphoma: complete remission with fludarabine, mitoxantrone and dexamethasone. Au, W.Y., Ng, W.M., Choy, C., Kwong, Y.L. Br. J. Dermatol. (2000) [Pubmed]
  26. Development of targeted therapies for B-cell non-hodgkin lymphoma and multiple myeloma. Hernandez-Ilizaliturri, F.J., Gowda, A., Czuczman, M.S. Clinical advances in hematology & oncology : H&O (2004) [Pubmed]
  27. Prenatal diagnosis of frontonasal dysplasia (median cleft syndrome). Frattarelli, J.L., Boley, T.J., Miller, R.A. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. (1996) [Pubmed]
  28. Angular analysis of corpus callosum in 18 patients with frontonasal dysplasia. Giffoni, S.D., Gonçalves, V.M., Zanardi, V.A., Lopes, V.L. Arquivos de neuro-psiquiatria. (2004) [Pubmed]
  29. Bilateral perysilvian polymicrogyria in Chiari I malformation. Spalice, A., Parisi, P., Mastrangelo, M., De Luca, F., Verrotti, A., Iannetti, P. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery (2006) [Pubmed]
 
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