Abstract | BACKGROUND: METHODS: Six patients with ataxia telangiectasia received betamethasone at 0.02 mg/kg/day for two years. After cessation of betamethasone, the patients were observed for two additional years. Neurological assessments were performed, and adverse effects were monitored every three months throughout the four-year study period. RESULTS: Transient improvement of neurological symptom was observed in five of the six patients. However, after two years betamethasone treatment, only one of the six patients showed a slight improvement in the neurological score, one patient showed no change, and the neurological scores of the remaining four patients deteriorated. After the cessation of betamethasone treatment, neurological symptoms worsened in all patients. As an adverse effect of betamethasone, transient adrenal dysfunction was observed in all cases. CONCLUSIONS: Although these findings are in agreement with previous studies suggesting that short-term betamethasone treatment transiently benefits patients with ataxia telangiectasia, the long-term benefits and risks should be carefully considered.
|
Authors | Setsuko Hasegawa, Satoko Kumada, Naoyuki Tanuma, Atsumi Tsuji-Hosokawa, Ayako Kashimada, Tomoko Mizuno, Kengo Moriyama, Yuji Sugawara, Ikuko Shirai, Yohane Miyata, Hiroya Nishida, Hideaki Mashimo, Takeshi Hasegawa, Takatoshi Hosokawa, Hiroaki Hisakawa, Mitsugu Uematsu, Akio Fujine, Rie Miyata, Hiroshi Sakuma, Kenichi Kashimada, Kohsuke Imai, Tomohiro Morio, Masaharu Hayashi, Shuki Mizutani, Masatoshi Takagi |
Journal | Pediatric neurology
(Pediatr Neurol)
Vol. 100
Pg. 60-66
(11 2019)
ISSN: 1873-5150 [Electronic] United States |
PMID | 31272782
(Publication Type: Clinical Trial, Phase II, Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
|
Copyright | Copyright © 2019 Elsevier Inc. All rights reserved. |
Chemical References |
- Glucocorticoids
- Betamethasone
|
Topics |
- Adolescent
- Adrenal Gland Diseases
(chemically induced)
- Ataxia Telangiectasia
(drug therapy)
- Betamethasone
(administration & dosage, adverse effects, pharmacology)
- Child
- Drug-Related Side Effects and Adverse Reactions
- Female
- Glucocorticoids
(administration & dosage, adverse effects, pharmacology)
- Humans
- Longitudinal Studies
- Male
- Outcome Assessment, Health Care
- Peripheral Nervous System Diseases
(chemically induced)
|
|
Join CureHunter, for free Research Interface BASIC access!
Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease.
Find out why thousands of doctors, pharma researchers and patient activists
around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!
|