中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2013年
1期
21-28
,共8页
汪吉梅%程国强%钱蓓倩%孟永勤
汪吉梅%程國彊%錢蓓倩%孟永勤
왕길매%정국강%전배천%맹영근
红细胞增多症%双胎输血综合征%双胎疾病%大脑中动脉%双胎贫血红细胞增多序列症
紅細胞增多癥%雙胎輸血綜閤徵%雙胎疾病%大腦中動脈%雙胎貧血紅細胞增多序列癥
홍세포증다증%쌍태수혈종합정%쌍태질병%대뇌중동맥%쌍태빈혈홍세포증다서렬증
Polycythemia%Transfusion%Fetofetal diseases in twins%Middle cerebral artery%Twin anemia-polycythemia sequence
目的 探讨双胎贫血红细胞增多序列症的临床表现、产前特点、诊断标准及分级、治疗及近期预后等,以提高临床医生对本病的认识.方法 报道1例双胎贫血红细胞增多序列症患儿的临床资料,并对近5年来已报道的15例进行文献复习.结果 双胎贫血红细胞增多序列症的报道有增多趋势.产前表现:有13/16例是自然发生的,有3/16例是发生在激光治疗术后的.16例均无羊水量差异.超声多普勒大脑中动脉峰值流速(MCA-PSV)在供血胎大于1.5 MoM的有11/16例,3/16例未测；在受血胎小于1.0 MoM的有10/16例,3/16例未测.有胎儿水肿的有6/16例.宫内干预:未治疗的9/16例,宫内输血的4/16例,胎儿镜下激光凝固胎盘吻合血管的4/16例,脐带阻塞选择性灭胎的2/16例,1/16例受血儿给予宫内血液稀释治疗.生后表现:贫血儿平均血红蛋白浓度为95 g/L,多血质儿为208g/L,胎胎间血红蛋白差异大于80 g/L的有10/16例,2/16例小于80 g/L(宫内激光治疗后).5/16例胎胎间网织红细胞比率大于1.7,1/16例小于1.7(宫内激光治疗后),8/16例未提供网织红细胞比率(使用的是网织红细胞绝对计数).10/16例胎盘彩色染料灌注有直径小于1 mm的动静脉吻合支存在,2/16例胎盘无残存的血管吻合支(宫内激光治疗后),2/16例未提供胎盘灌注结果.生后治疗:9/16例供血儿有贫血表现,其中有6/16例予输血治疗；6/16例受血儿有高黏滞血症表现,给予部分换血治疗.11/16例新生儿期神经发育正常,1/16例双胎颅脑超声持续脑室周围白质高回声,本例随访至纠正胎龄3个月时神经发育正常,继续随访中.结论 双胎贫血红细胞增多序列症是胎胎输血综合征的一个特殊类型:单绒毛膜双羊膜囊双胎,产前无明显羊水量差异,出生体重相差不大,但血红蛋白相差明显且网织红细胞比率增高.对于单绒毛膜双羊膜囊双胎,产前除胎儿生长经线及羊水量测定外,应常规监测大脑中动脉峰值流速,以便筛查出需要宫内干预的病例,以期减少死亡率及改善预后.生后认真评估治疗,并需长期随访其运动及智能发育情况.
目的 探討雙胎貧血紅細胞增多序列癥的臨床錶現、產前特點、診斷標準及分級、治療及近期預後等,以提高臨床醫生對本病的認識.方法 報道1例雙胎貧血紅細胞增多序列癥患兒的臨床資料,併對近5年來已報道的15例進行文獻複習.結果 雙胎貧血紅細胞增多序列癥的報道有增多趨勢.產前錶現:有13/16例是自然髮生的,有3/16例是髮生在激光治療術後的.16例均無羊水量差異.超聲多普勒大腦中動脈峰值流速(MCA-PSV)在供血胎大于1.5 MoM的有11/16例,3/16例未測；在受血胎小于1.0 MoM的有10/16例,3/16例未測.有胎兒水腫的有6/16例.宮內榦預:未治療的9/16例,宮內輸血的4/16例,胎兒鏡下激光凝固胎盤吻閤血管的4/16例,臍帶阻塞選擇性滅胎的2/16例,1/16例受血兒給予宮內血液稀釋治療.生後錶現:貧血兒平均血紅蛋白濃度為95 g/L,多血質兒為208g/L,胎胎間血紅蛋白差異大于80 g/L的有10/16例,2/16例小于80 g/L(宮內激光治療後).5/16例胎胎間網織紅細胞比率大于1.7,1/16例小于1.7(宮內激光治療後),8/16例未提供網織紅細胞比率(使用的是網織紅細胞絕對計數).10/16例胎盤綵色染料灌註有直徑小于1 mm的動靜脈吻閤支存在,2/16例胎盤無殘存的血管吻閤支(宮內激光治療後),2/16例未提供胎盤灌註結果.生後治療:9/16例供血兒有貧血錶現,其中有6/16例予輸血治療；6/16例受血兒有高黏滯血癥錶現,給予部分換血治療.11/16例新生兒期神經髮育正常,1/16例雙胎顱腦超聲持續腦室週圍白質高迴聲,本例隨訪至糾正胎齡3箇月時神經髮育正常,繼續隨訪中.結論 雙胎貧血紅細胞增多序列癥是胎胎輸血綜閤徵的一箇特殊類型:單絨毛膜雙羊膜囊雙胎,產前無明顯羊水量差異,齣生體重相差不大,但血紅蛋白相差明顯且網織紅細胞比率增高.對于單絨毛膜雙羊膜囊雙胎,產前除胎兒生長經線及羊水量測定外,應常規鑑測大腦中動脈峰值流速,以便篩查齣需要宮內榦預的病例,以期減少死亡率及改善預後.生後認真評估治療,併需長期隨訪其運動及智能髮育情況.
목적 탐토쌍태빈혈홍세포증다서렬증적림상표현、산전특점、진단표준급분급、치료급근기예후등,이제고림상의생대본병적인식.방법 보도1례쌍태빈혈홍세포증다서렬증환인적림상자료,병대근5년래이보도적15례진행문헌복습.결과 쌍태빈혈홍세포증다서렬증적보도유증다추세.산전표현:유13/16례시자연발생적,유3/16례시발생재격광치료술후적.16례균무양수량차이.초성다보륵대뇌중동맥봉치류속(MCA-PSV)재공혈태대우1.5 MoM적유11/16례,3/16례미측；재수혈태소우1.0 MoM적유10/16례,3/16례미측.유태인수종적유6/16례.궁내간예:미치료적9/16례,궁내수혈적4/16례,태인경하격광응고태반문합혈관적4/16례,제대조새선택성멸태적2/16례,1/16례수혈인급여궁내혈액희석치료.생후표현:빈혈인평균혈홍단백농도위95 g/L,다혈질인위208g/L,태태간혈홍단백차이대우80 g/L적유10/16례,2/16례소우80 g/L(궁내격광치료후).5/16례태태간망직홍세포비솔대우1.7,1/16례소우1.7(궁내격광치료후),8/16례미제공망직홍세포비솔(사용적시망직홍세포절대계수).10/16례태반채색염료관주유직경소우1 mm적동정맥문합지존재,2/16례태반무잔존적혈관문합지(궁내격광치료후),2/16례미제공태반관주결과.생후치료:9/16례공혈인유빈혈표현,기중유6/16례여수혈치료；6/16례수혈인유고점체혈증표현,급여부분환혈치료.11/16례신생인기신경발육정상,1/16례쌍태로뇌초성지속뇌실주위백질고회성,본례수방지규정태령3개월시신경발육정상,계속수방중.결론 쌍태빈혈홍세포증다서렬증시태태수혈종합정적일개특수류형:단융모막쌍양막낭쌍태,산전무명현양수량차이,출생체중상차불대,단혈홍단백상차명현차망직홍세포비솔증고.대우단융모막쌍양막낭쌍태,산전제태인생장경선급양수량측정외,응상규감측대뇌중동맥봉치류속,이편사사출수요궁내간예적병례,이기감소사망솔급개선예후.생후인진평고치료,병수장기수방기운동급지능발육정황.
Objective To summarize and review the clinical characteristics including clinical features,prenatal characteristics,diagnosis,treatments and short-term outcomes of the twin anemiapolycythemia sequence (TAPS) to improve the recognition of the disease.Method The clinical data of one case with twin anemia-polycythemia sequence and the reports of 15 cases seen in the past 5 years were reviewed and analyzed.Result There was an increasing number of reports of cases with TAPS.Prenatal manifestation:among the 16 cases,TAPS occurred in 13 cases naturally and in 3 cases occurred after laser treatment.Amniotic fluid volume showed no significant difference in 16 cases.Middle cerebral artery peak systolic velocity (MCA-PSV) ＞ 1.5 multiples of the median (MoM) in the donor were 11/16 cases and 3/16 cases were not tested.MCA-PSV ＜ 1.0 MoM in the recipient were seen in 10/16 cases and in 3/16 cases MCA-PSV was not tested.Hydrops fetalis was found in 6/16 cases.Intrauterine intervention:intrauterine blood transfusion was performed in 4/16 cases,fetoscopic laser occlusion of chorioangiopagus vessels was performed in 4/16 cases,umbilical cord occlusion selective feticide was done in 2/16 cases and intrauterine hemodilution in the recipient was performed in 1/16 case.Postnatal manifestation:average hemoglobin concentration in the anemic neonate was 95 g/L and in the polycythemic one was 208 g/L,intertwin Hb difference was ＞ 80 g/L in 10/16 cases and ＜80 g/L in 2/16 cases (after intrauterine laser treatment).Intertwin reticulocyte count ratio was ＞ 1.7 in 5/16 cases and ＜ 1.7 in 1/16 case (after intrauterine laser treatment).Postnatal treatment:9/16 cases of donor had anemia,among them,6/16 cases were given blood transfusions,6/16 cases of recipient with hyperviscosity underwent partial exchange transfusions.Neurodevelopmental follow-up during neonatal period was normal in 11/16 cases,in our case,neurodevelopmental follow-up at the corrected gestational age 3 months was normal.Conclusion TAPS is a new atypical form of twin-twin transfusion syndrome (TTTS) that presents as a large intertwin hemoglobin difference with one twin developing anemia and the other developing polycythemia,without oligohydramniospolyhydramnios sequence that is required for the diagnosis of TTTS.We suggest that routine doppler studies and MCA-PSV measurements should be performed during each follow-up visit in all uncomplicated monochorionic twin pregnancies,in order to find out the cases required intrauterine intervention to decrease neonatal mortality rates and improve the prognosis.
