Misc.

2017

Importance of the multisystem follow-up in patients with tuberous sclerosis complex

NO TO HATTATSU
  • Yoshinaga Harumi
  • ,
  • Oka Makio
  • ,
  • Akiyama Tomoyuki
  • ,
  • Endoh Fumika
  • ,
  • Akiyama Mari
  • ,
  • Hayashi Yumiko
  • ,
  • Shibata Takashi
  • ,
  • Hanaoka Yoshiyuki
  • ,
  • Kobayashi Katsuhiro

Volume
49
Number
1
First page
5
Last page
9
Language
Japanese
Publishing type
DOI
10.11251/ojjscn.49.5
Publisher
The Japanese Society of Child Neurology

<p>  Objective: Tuberous sclerosis complex (TSC) is a multisystem disorder characterized by the formation of hamartoma in multiple organ systems of the body. However, without a well-established cooperative system involving related departments, some organ lesions might be overlooked until symptoms appear or even until the disorder progresses. Therefore, the purpose of this study is to investigate the current status of follow-ups in the TSC patients in the Department of Child Neurology at Okayama University Medical Hospital. Methods: We performed a retrospective chart review of 38 patients with TSC who visited our hospital at least twice between January 2005 and December 2014. Patients were between 3 years and 48 years of age at their latest visit. We divided the patients into a child group and an adult group, and investigated the patients' follow-up data while focusing on the various multiorgan systems. Results: The follow-ups were well conducted in the child group in terms of every organ. In the adult group, neuroimaging tests were unsatisfactorily performed. The kidney has not been examined in seven patients more than five years even though these patients all had kidney lesions. The lung was not been examined in 7 out of 14 female patients over 18 years of age who are most at risk for lymphangioleiomyomatosis (LAM). In 12 out of 18 child patients, echocardiograms were performed every few years, while electrocardiograms to assess underlying conduction defects were rarely performed in either age group. Conclusions: In Europe, guidelines for the management of TSC have been well established. However, in our hospital, the multiorgan system follow-up is not satisfactorily performed especially in adult patients. We decided the establishment of a TSC board in our hospital for the management of this multiorgan disorder.</p>

Link information
DOI
https://doi.org/10.11251/ojjscn.49.5
CiNii Articles
http://ci.nii.ac.jp/naid/130005606834
URL
http://search.jamas.or.jp/link/ui/2017196531
ID information
  • DOI : 10.11251/ojjscn.49.5
  • CiNii Articles ID : 130005606834

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