CAJ | 학술논문

目的探讨新生儿MN溶血病的临床特点及诊治经验,更好地预防治疗本病.方法报道新生儿MN溶血病1例,并对国内近20年来已报告的21例进行文献复习.结果新生儿MN溶血病的报道有增多趋势.有4/22例发生于第1胎,男:女=10:8;患儿血型19/21例为MN、2/21例为M,其母血型10/21例为N、11/21例为NN;直接抗人球蛋白试验7/18例为阳性、4/18例为弱阳性、7/18例为阴性,抗体释放试验13/16例为阳性、3/16例为阴性,游离抗体试验17/17例为阳性;母抗体类型:21/21例IgG抗-M均(+),10/21例IgM抗M亦(+);患儿抗体类型:22/22例均为IgG抗M(+);患儿均有不同程度的贫血、黄疸;11/15例在出生24 h内出现黄疸;4/13例直接胆红素也明显增高;出现黄疸后,多数给予了光疗;4/15例给予了大剂量静脉注射免疫球蛋白;8/22例实施了换血治疗;死亡3/22例、治愈19/22例.结论抗-M抗体引起的新生儿溶血病其病情轻重相差较大,重者甚至死胎或者需要换血.对于反复死胎、流产、胎儿水肿、严重贫血或多年不育的患者,在排除了其他原因及新生儿ABO、Rh溶血病后,应查MN血型及其抗体进一步明确本病.有效的光疗、效果显著的大剂量静脉注射免疫球蛋白、金属卟啉类药物等治疗,已经明显减少了需要换血的患儿数量.换血多采用全自动周围血管双管同步换血法.
목적 탐토신생인MN용혈병적림상특점급진치경험,경호지예방치료본병.방법 보도신생인MN용혈병1례,병대국내근20년래이보고적21례진행문헌복습.결과 신생인MN용혈병적보도유증다추세.유4/22례발생우제1태,남:녀=10:8;환인혈형19/21례위MN、2/21례위M,기모혈형10/21례위N、11/21례위NN;직접항인구단백시험7/18례위양성、4/18례위약양성、7/18례위음성,항체석방시험13/16례위양성、3/16례위음성,유리항체시험17/17례위양성;모항체류형:21/21례IgG항-M균(+),10/21례IgM항M역(+);환인항체류형:22/22례균위IgG항M(+);환인균유불동정도적빈혈、황달;11/15례재출생24 h내출현황달;4/13례직접담홍소야명현증고;출현황달후,다수급여료광료;4/15례급여료대제량정맥주사면역구단백;8/22례실시료환혈치료;사망3/22례、치유19/22례.결론 항-M항체인기적신생인용혈병기병정경중상차교대,중자심지사태혹자수요환혈.대우반복사태、유산、태인수종、엄중빈혈혹다년불육적환자,재배제료기타원인급신생인ABO、Rh용혈병후,응사MN혈형급기항체진일보명학본병.유효적광료、효과현저적대제량정맥주사면역구단백、금속계람류약물등치료,이경명현감소료수요환혈적환인수량.환혈다채용전자동주위혈관쌍관동보환혈법.
Objective To analyze and summarize clinical manifestation of hemolytic disease of the newborn (HDN) due to anti-M. Methods Data of one case of HDN due to anti-M and the reports of 21 cases seen in the past 20 years at the home country were reviewed and analyzed. Results There was an increasing number of reports of cases with HDN due to anti-M. Among the 22 cases, four were the first fetus. Of 18 infants, ten were male, and eight were female. The blood group was MN in 19/21 infants, and was M in 2/21 infants. The blood group was N in 10/21 mothers, and was NN in 11/21 mothers. Among the 18 infants, the direct antiglobulin test of 7 infants were positive, of 4 infants were dubiously positive, and of 7 infants was negative. Among the 16 infants, the antibody release test of 13 infants was positive, and of 3 infants were negative. Among 17 infants, the free antibody test of all was positive. Among the 21 mothers, the anti-M of IgG were positive in all mothers, and along with IgM in 11 mothers. The anti-M of IgG was positive in all infants. Mild or severe anemia and icterus were found in all cases. Among the 15 cases, jaundice was evident on the 1st day of life in 11 cases. Among 13 cases, marked elevation of both indirect-and direct-reacting bilirubin levels was reported in 4 cases. Phototherapy was applied when jaundice became evident. High-dose intravenous immunoglobulin was given to 4/15 cases. Exchange transfusion were performed in 8 of 22 cases. Three cases died, and 19 cases were cured. Conclusion HDN of varying degrees of severity has been reported in association with anti-M and can even lead to intrauterine deaths or requiting treatment with exchange transfusion. If the mother has a history of prior intrauterine deaths, abortion, hydrops fetalis, severe fetal anemia or infertile, MN blood group and anti-M antibodies should be tested after excluding the possibility of other causes and HDN due to ABO or Rh blood group incompatibility.As the efficacy of phototherapy increases, the role of exchange transfusion in acute management is rapidly decreasing. High-dose intravenous immunoglobulin and/or intramuscular metalloporphyrins may further reduce the need for exchange transfusion. The exchange transfusion may be performed through peripheral arterial (drawn out) and venous (infused in) lines.