title: Medical procedures and outcomes of Japanese patients with trisomy 18 or trisomy 13: analysis of a nationwide administrative database of hospitalized patients.
abstract: The choices of aggressive treatment for trisomy 18 (T18) and trisomy 13 (T13) remain controversial. Here, we describe the current medical procedures and outcomes of patients with T18 and T13 from a nationwide administrative database of hospitalized patients in Japan. We used the database to identify eligible patients with T18 (n = 438) and T13 (n = 133) who were first admitted to one of 200 hospitals between July 2010 and March 2013. Patients were divided into admission at day <7 (early neonatal) and admission at day ≥7 (late neonatal and post neonatal) groups, and we described the medical intervention and status at discharge for each group. In the day <7 groups, surgical interventions were performed for 56 (19.9%) T18 patients and 22 (34.4%) T13 patients, including pulmonary artery banding, and procedures for esophageal atresia and omphalocele. None received intracardiac surgery. The rate of patients discharged to home was higher in the day ≥7 groups than the day <7 groups (T18: 72.6 vs. 38.8%; T13: 73.9 vs. 21.9%, respectively). Our data show that a substantial number of patients with trisomy received surgery and were then discharged home, but, of these, a considerable number required home medical care. This included home oxygen therapy, home mechanical ventilation, and tube feeding. These findings will be useful to clinicians or families who care for patients with T18 and T13.
authors.affiliation: Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Kazue
authors.initials: K
authors.lastname: Ishitsuka
authors.affiliation: Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Matsui
authors.affiliation: Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Michihata
authors.affiliation: Department of Health Policy and Informatics, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Kiyohide
authors.initials: K
authors.lastname: Fushimi
authors.affiliation: Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tomoo
authors.initials: T
authors.lastname: Nakamura
authors.affiliation: Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Yasunaga
mesh_terms: D025063:Chromosome Disorders; D002882:Chromosomes, Human, Pair 13; D002887:Chromosomes, Human, Pair 18; D003628:Database Management Systems; D005260:Female; D006760:Hospitalization; D006801:Humans; D007231:Infant, Newborn; D007297:Inpatients; D007564:Japan; D008297:Male; D014314:Trisomy; D000073839:Trisomy 13 Syndrome; D000073842:Trisomy 18 Syndrome
keywords: congenital malformation; neonatal intensive care; survival; trisomy13; trisomy18