We measured cerebrospinal fluid (CSF) hypocretin‐1 levels in 11 patients with narcolepsy–cataplexy, five with narcolepsy without cataplexy and 12 with idiopathic hypersomnia (IHS). All patients were Japanese. As reported in Caucasian patients, undetectable or very low hypocretin‐1 levels were observed in most (9 out of 11) Japanese narcolepsy–cataplexy patients. Our hypocretin‐deficient narcoleptics included three prepubertal cases within few months after the disease onset. All nine hypocretin‐deficient patients were human leuckocyte antigen (HLA) DR2 positive, while two who had normal CSF hypocretin‐1 levels were HLA DR2 negative. In contrast, none of the narcolepsy without cataplexy and IHS subjects had undetectable low levels. Low CSF hypocretin‐1 is therefore very specific for HLA DR2 positive narcolepsy–cataplexy, and the deficiency is likely to be established at the early stage of the disease.
The impact of surfactant therapy on chronic lung disease remains uncertain. During the past decade (1982–91), over 300 babies with respiratory distress syndrome (RDS) weighing 501–2,500 g at birth were consecutively treated with surfactant‐TA at our neonatal intensive care unit. Data on 95 RDS babies treated in the first 5 year period (Period 1, 1982–86) were compared with those on 158 RDS babies treated in the second 5 year period (Period 2, 1987–91). Overall respiratory improvement was better in Period 2 than in Period 1. In Period 2, surfactant therapy converted 98% of the babies with moderate/severe RDS to those with ‘near normal’ lung by 72 hr post‐treatment. In Period 2, 95% of the surfactant‐treated babies weighing 501–1,750 g at birth survived, 97% of which required no supplemental oxygen at 40 weeks corrected gestational age. Increased survival rate in the surfactant‐treated babies during the past decade has not been followed by a parallel increase in chronic lung disease. The severity of the initial pulmonary disease per se was not the significant risk factor for chronic lung disease. Several other variables affecting the response to surfactant therapy and outcome have been identified by stepwise logistic regression analysis and include factors related to perinatal events such as birth asphyxia and infection, and other complications of prematurity.
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