Page last updated: 2024-11-05

thalidomide and Neurofibromatosis 1

thalidomide has been researched along with Neurofibromatosis 1 in 3 studies

Thalidomide: A piperidinyl isoindole originally introduced as a non-barbiturate hypnotic, but withdrawn from the market due to teratogenic effects. It has been reintroduced and used for a number of immunological and inflammatory disorders. Thalidomide displays immunosuppressive and anti-angiogenic activity. It inhibits release of TUMOR NECROSIS FACTOR-ALPHA from monocytes, and modulates other cytokine action.
thalidomide : A racemate comprising equimolar amounts of R- and S-thalidomide.
2-(2,6-dioxopiperidin-3-yl)-1H-isoindole-1,3(2H)-dione : A dicarboximide that is isoindole-1,3(2H)-dione in which the hydrogen attached to the nitrogen is substituted by a 2,6-dioxopiperidin-3-yl group.

Neurofibromatosis 1: An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).

Research Excerpts

ExcerptRelevanceReference
"An open-label phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF)."9.10Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1. ( Cohen, BH; Gupta, A; Packer, RJ; Phillips, PC; Ruggieri, P, 2003)
"An open-label phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF)."5.10Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1. ( Cohen, BH; Gupta, A; Packer, RJ; Phillips, PC; Ruggieri, P, 2003)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19901 (33.33)18.7374
1990's0 (0.00)18.2507
2000's1 (33.33)29.6817
2010's0 (0.00)24.3611
2020's1 (33.33)2.80

Authors

AuthorsStudies
Suresh, KV1
Xu, AL1
Groves, ML1
Sponseller, PD1
Gupta, A1
Cohen, BH1
Ruggieri, P1
Packer, RJ1
Phillips, PC1
Achs, R1
Harper, RG1

Reviews

2 reviews available for thalidomide and Neurofibromatosis 1

ArticleYear
Spinal screening, malignancy, medical therapy, and surgical correction of deformity in pediatric patients with neurofibromatosis type 1: a systematic review.
    Journal of pediatric orthopedics. Part B, 2022, Nov-01, Volume: 31, Issue:6

    Topics: Child; Humans; Kyphosis; Mitogen-Activated Protein Kinase Kinases; Neurofibromatosis 1; Sirolimus; S

2022
Dermatoglyphics.
    American journal of obstetrics and gynecology, 1968, Aug-01, Volume: 101, Issue:7

    Topics: Abnormalities, Drug-Induced; Anemia, Sickle Cell; Anencephaly; Cerebral Palsy; Chromosome Aberration

1968

Trials

1 trial available for thalidomide and Neurofibromatosis 1

ArticleYear
Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1.
    Neurology, 2003, Jan-14, Volume: 60, Issue:1

    Topics: Adolescent; Adult; Angiogenesis Inhibitors; Child; Cohort Studies; Dermatologic Agents; Dose-Respons

2003