Shoichiro Izawa, Kazuhisa Matsumoto, Kanako Kadowaki, Toshifumi Nakamura, Norio Wada, Takuyuki Katabami, Yoichi Nozato, Ryuji Okamoto, Takamasa Ichijo, Daisuke Taura, Masanori Murakami, Miki Kakutani, Takashi Yoneda, Minemori Watanabe, Yutaka Takahashi, Shintaro Okamura, Katsutoshi Takahashi, Hiroki Kobayashi, Mitsuhide Naruse, Kenichi Yokota, Masakatsu Sone, the JPAS-II Study Group
Recommendation from the Editor
Congenital nephrogenic diabetes insipidus (CNDI) is a rare genetic disorder characterized by polyuria, growth retardation, and dehydration. Japanese guidelines indicate that DDAVP is effective in some cases when used in combination with conventional therapies. However, until now, no studies have investigated the CNDI patients who were responsive to DDAVP. In this issue, Dr Kento Ikegawa in Tokyo Metropolitan Children’s Medical Center and his colleagues aimed to clarify the use, efficacy, and safety, and characteristics of the patients who were responsive to DDAVP. They clearly demonstrated that DDAVP is effective for CNDI with residual V2R function and the water deprivation test with vasopressin injection and genetic testing may be useful in predicting responsiveness to DDAVP. Our editorial team is confident that this excellent report provides readers with the latest knowledge on the treatment of CNDI with DDAVP.