日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 36(2): 121-127 (2020)
doi:10.9794/jspccs.36.121

ReviewReview

ラット胎仔胸腺欠損,大動脈弓離断,血管輪の胸部断面像Cross-Sectional Morphology of Fetal Absent Thymus, Interrupted Aortic Arch, and Vascular Ring in Rats

東京女子医科大学循環器小児科Department of Pediatric Cardiology, Tokyo Women’s Medical University ◇ Tokyo, Japan

発行日:2020年6月1日Published: June 1, 2020
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Bis-diamineはラットに強力なteratogenで高率にヒト染色体22q11欠失症候群類似の先天性心疾患と胸腺欠損を生じるが,作用機序の詳細は不明である.Bis-diamine 200 mgを妊娠9日と10日のラット40匹に胃内注入し,満期21日目に胎仔を全身急速凍結法,凍結ミクロトーム,実体顕微鏡(Wild M400)を用いて0.5 mm毎の胸部横断面を連続写眞で記録した.330胎仔に括弧内の率で次の先天性心疾患が生じた.各種のFallot四徴症(68%),総動脈幹残遺(18%),大動脈弓離断(2%)などである.これらの心疾患には胸腺欠損(70%)乃至低形成(30%),右側大動脈弓,鎖骨下動脈起始異常,血管輪などが合併した.前著ではこれらの胎生期先天性心疾患の生体内断面図を染色体2q11.2欠失症候群の胎児心エコーモデル図譜として提示したが,胸腺低形成,血管輪と大動脈弓離断について紙面の都合で図示不充分であったので,ここに追加提示する.

Bis-diamine is a potent teratogen in rats which induces conotruncal diseases and thymic hypoplasia similar to those seen in chromosome 22q11 deletion syndrome in humans. The precise mechanism of the cardiac teratogenic effects of bis-diamine is unknown. Bis-diamine was administered to 40 pregnant rats on the 10th day of gestation at a dose of 200 mg. Following cervical dislocation of these rats on the 21st day of gestation, the fetuses were delivered by Cesarean section and were then frozen immediately in acetone cooled to −76°C with dry ice. Each frozen fetus thorax was cut transversely and the section surface was serially photographed with a stereoscopic microscope (Wild M400 Photomacroscope) every 500 microns. The following cardiac diseases were recorded in 330 fetuses: tetralogy of Fallot (16%), tetralogy of Fallot with absent pulmonary valve (14%), tetralogy of Fallot with pulmonary valvular and infundibular atresia (38%), truncus arteriosus (10%), ventricular septal defect (3%), interrupted aortic arch type B (2%), and atrioventricular septal defect (2%). These diseases are animal models of fetal echocardiographic diagnoses representing congenital heart diseases associated with chromosome 22q11.2 deletion syndrome, and the cross-sectional morphologies represent a graphical review of these condition. These are additional graphs of hypoplastic or absent thymus, interrupted aortic arch, and vascular ring.

Key words: interrupted aortic arch; vascular ring; fetal echocardiography; chromosome 22q11.2 deletion syndrome; absent thymus

This page was created on 2020-05-21T14:37:17.237+09:00
This page was last modified on 2020-06-18T10:34:13.000+09:00


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