Factors related to home health-care transition in trisomy 13

被引:2
作者
Kitase, Yuma [1 ]
Hayakawa, Masahiro [1 ]
Kondo, Taiki [1 ]
Saito, Akiko [1 ]
Tachibana, Takashi [2 ]
Oshiro, Makoto [2 ]
Ieda, Kuniko [3 ]
Kato, Eiko [3 ]
Kato, Yuichi [4 ]
Hattori, Tetsuo [4 ]
Hayashi, Seiji [5 ]
Ito, Masatoki [6 ]
Hyodo, Reina [6 ]
Muramatsu, Yukako [7 ]
Sato, Yoshiaki [1 ]
机构
[1] Nagoya Univ Hosp, Div Neonatol, Ctr Maternal Neonatal Care, Nagoya, Aichi, Japan
[2] Japanese Red Cross Nagoya Daiichi Hosp, Dept Neonatol, Nagoya, Aichi, Japan
[3] Tosei Gen Hosp, Dept Pediat, Alchi, Japan
[4] Anjo Kosei Hosp, Dept Pediat, Alchi, Japan
[5] Okazaki City Hosp, Dept Pediat, Alchi, Japan
[6] Ogaki Municipal Hosp, Dept Pediat 2, Ogaki, Japan
[7] Cent Hosp, Aichi Human Serv Ctr, Dept Pediat, Alchi, Japan
关键词
congenital heart defects; gestational age; home care; occipitofrontal circumference; trisomy; 13; PATAU SYNDROME; NATURAL-HISTORY; SURVIVAL; MANAGEMENT; CHILDREN;
D O I
10.1002/ajmg.a.38371
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Trisomy 13 (T13) is accompanied by severe complications, and it can be challenging to achieve long-term survival without aggressive treatment. However, recently, some patients with T13 have been receiving home care. We conducted this study to investigate factors related to home health-care transition for patients with T13.We studied 28 patients with T13 born between January 2000 and December 2014. We retrospectively compared nine home care transition patients (the home care group) and 19 patients that died during hospitalization (the discharge at death group). The median gestational age of the patients was 36.6 weeks, with a median birth weight of 2,047g. Currently, three patients (11%) have survived, and 25 (89%) have died. The home care group exhibited a significantly longer gestational age (38.9 vs. 36.3 weeks, p=0.039) and significantly larger occipitofrontal circumference Z score (-0.04 vs. -0.09, p=0.019). Congenital heart defects (CHD) was more frequent in the discharge at death group, with six patients in the home care group and 18 patients in the discharge at death group (67% vs. 95%, p=0.047), respectively. Survival time was significantly longer in the home care group than in the discharge at death group (171 vs. 19 days, p=0.012). This study has shown that gestational age, occipitofrontal circumference Z score at birth, and the presence of CHD are helpful prognostic factors for determining treatment strategy in patients with T13.
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收藏
页码:2635 / 2640
页数:6
相关论文
共 16 条
[1]   Shared Decision Making and the Pathways Approach in the Prenatal and Postnatal Management of the Trisomy 13 and Trisomy 18 Syndromes [J].
Andrews, Sasha E. ;
Downey, Ann G. ;
Showalter, David Scott ;
Fitzgerald, Heather ;
Showalter, Vivian P. ;
Carey, John C. ;
Hulac, Peter .
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS, 2016, 172 (03) :257-263
[2]   NATURAL-HISTORY OF TRISOMY-18 AND TRISOMY-13 .2. PSYCHOMOTOR DEVELOPMENT [J].
BATY, BJ ;
JORDE, LB ;
BLACKBURN, BL ;
CAREY, JC .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1994, 49 (02) :189-194
[3]   NATURAL-HISTORY OF TRISOMY-18 AND TRISOMY-13 .1. GROWTH, PHYSICAL ASSESSMENT, MEDICAL HISTORIES, SURVIVAL, AND RECURRENCE RISK [J].
BATY, BJ ;
BLACKBURN, BL ;
CAREY, JC .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1994, 49 (02) :175-188
[4]   Birth History, Physical Characteristics, and Medical Conditions in Long-Term Survivors With Full Trisomy 13 [J].
Bruns, Deborah .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2011, 155A (11) :2634-2640
[5]  
Carey J.C., 2005, Management of Genetic Syndromes, V2nd ed., P555
[6]  
Carey JC, 2010, Management of Genetic Syndromes, VThird, P807, DOI [10.1002/9780470893159.ch54, DOI 10.1002/9780470893159.CH54]
[7]   Variable expressivity in Patau syndrome is not all related to trisomy 13 mosaicism [J].
Hsu, Hui-Fang ;
Hou, Jia-Woei .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2007, 143A (15) :1739-1748
[8]   Changes in fetal prevalence and outcome for trisomies 13 and 18: a population-based study over 23 years [J].
Irving, Claire ;
Richmond, Sam ;
Wren, Christoper ;
Longster, Caitlin ;
Embleton, Nicholas D. .
JOURNAL OF MATERNAL-FETAL & NEONATAL MEDICINE, 2011, 24 (01) :137-141
[9]   Parental Hopes, Interventions, and Survival of Neonates With Trisomy 13 and Trisomy 18 [J].
Janvier, Annie ;
Farlow, Barbara ;
Barrington, Keith J. .
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS, 2016, 172 (03) :279-287
[10]   Procedures in the 1st Year of Life for Children With Trisomy 13 and Trisomy 18, a 25-Year, Single-Center Review [J].
Josephsen, Justin B. ;
Armbrecht, Eric S. ;
Braddock, Stephen R. ;
Cibulskis, Catherine C. .
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS, 2016, 172 (03) :264-271