Bartter症候群に属するテタニーの兄妹例

DEVELOPMENT OF TETANY IN SIBLINGS SUFFERED FROM BARTTER'S SYNDROME

　抄録　テタニーの基礎障害に関しては，低Ca血症以外には判然としないものが多い。今回，われわれはBartter症候群に属するテタニーの兄妹例を経験した。Bartter症候群は，それに関する知識の普及に伴ってそれ程珍しいものではなくなくなりつつあるが，家族性出現例（劣性遺伝を示唆する）も多く，テタニーはその主な臨床症状の一つで，血清Ca正常性テタニーに属する。未だ一般には注目されていないようであるが，テタニーの基礎障害の一つとして本症候群を加えるべきであろう。なお妹には軽い激越うつ病と過敏で小児的な性格傾向を認め，前者はスピロノラクトンによる低K血症の改善と平行して，テタニーと共に寛解した。低Ca血症ないし副甲状腺機能低下症の場合と同様，本症候群の文献例にも精神障害の出現が散見され，むしろそれが臨床症状の前景を占める場合もある。

The present authors observed and treated a siblings case of normocalcemic tetany, which is considered as belonging to Bartter's syndrome. As far as we know, there are a number of fami-lial cases of tetany in literature, but none of them spreads over more than two generations, so that the tetany appears to be recessive in hereditary characters including our patients. Both of them presented tetanic seizures in the course of Bartter's syndrome and they were regarded as one of vari-ous manifestations of the syndrome. In other words, the Bartter's syndrome or the hypopotas-semia should be one of the fundamental disorders for developing tetanic symptom.

The tetanic symptoms became extinct during the treatment with spironolactone against hypopotas-semia. Of the two patients, younger sister had shown an agitated depression developed on her childish and oversensitive personality, but the depression was improved in parallel to the reco- very from tetany and hypopotassemia. Therefore, it appears to be certain that the patients would have some premorbid deviation of personality traits, where symptomatic psychoses could be at-tributed, in the case of Bartter's syndrome.

Generally speaking the psychic disorders, such as personality deviation and psychotic episode, seem to by very important symptoms in patient with Bartter's syndrome as well as in patient with hypocalcemia or hypoparathyroidism.