CAJ | 학술논문

目的探讨儿童假性巴特综合征(Pseudo-Bartter syndrome,PBS)的临床特征及实验室检查特点,提高临床医师对本病的认识.方法收取首都医科大学附属北京儿童医院2008年11月至2013年9月收治的PBS患儿9例,对患儿的临床表现、辅助检查及治疗等相关临床资料进行回顾性分析.应用SPSS 19.0软件分析数据.结果 1.临床特征:本组病例共9例,其中女4例,男5例;就诊年龄4个月～8岁8个月.引起PBS的原因以呼吸道感染引起的消化道症状(如腹泻、呕吐)(7/9例)最为多见.6例患儿无明显临床表现,治疗原发病过程中发现低钾血症.2.实验室检查:均存在低钾低氯血症、不同程度的代谢性碱中毒及肾素、血管紧张素及醛固酮升高.3.治疗:在病因控制治疗、补钾对症治疗或吲哚美辛治疗[1 mg/(kg·d),分3次口服]后,患儿均达到临床好转且血电解质均恢复正常.在停补钾对症治疗后,血电解质仍正常,随访期限5～7d.结论 1.在中国儿童中,引起PBS最常见的原因以呼吸道感染所致的呕吐、腹泻等最为多见.2.儿童PBS除了病因相关的临床表现外,多无明显临床表现,常于实验室检查中偶然发现低钾血症.3.儿童PBS实验室检查表现为低钾低氯血症、代谢性碱中毒,其肾素、血管紧张素及醛固酮水平均明显升高.4.儿童PBS经祛除病因及补钾对症治疗,通常2～4d电解质恢复正常.
목적 탐토인동가성파특종합정(Pseudo-Bartter syndrome,PBS)적림상특정급실험실검사특점,제고림상의사대본병적인식.방법 수취수도의과대학부속북경인동의원2008년11월지2013년9월수치적PBS환인9례,대환인적림상표현、보조검사급치료등상관림상자료진행회고성분석.응용SPSS 19.0연건분석수거.결과 1.림상특정:본조병례공9례,기중녀4례,남5례;취진년령4개월～8세8개월.인기PBS적원인이호흡도감염인기적소화도증상(여복사、구토)(7/9례)최위다견.6례환인무명현림상표현,치료원발병과정중발현저갑혈증.2.실험실검사:균존재저갑저록혈증、불동정도적대사성감중독급신소、혈관긴장소급철고동승고.3.치료:재병인공제치료、보갑대증치료혹신타미신치료[1 mg/(kg·d),분3차구복]후,환인균체도림상호전차혈전해질균회복정상.재정보갑대증치료후,혈전해질잉정상,수방기한5～7d.결론 1.재중국인동중,인기PBS최상견적원인이호흡도감염소치적구토、복사등최위다견.2.인동PBS제료병인상관적림상표현외,다무명현림상표현,상우실험실검사중우연발현저갑혈증.3.인동PBS실험실검사표현위저갑저록혈증、대사성감중독,기신소、혈관긴장소급철고동수평균명현승고.4.인동PBS경거제병인급보갑대증치료,통상2～4d전해질회복정상.
Objective To analyze the clinical characteristics of children's Pseudo-Bartter syndrome(PBS) in order to enhance physician's understanding of the disease.Methods Nine children with PBS who were admitted into Beijing Children's Hospital from Nov.2008 to Sep.2013 were selected as research subjects.A retrospective study was carried out with the clinical data and the outcome of treatment.Results 1.Clinical characteristics:there were 9 cases in this group including 5 male and 4 female.The patients' age ranged from 4 months to 8 years 8 months.The most common cause of children's PBS was gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection (7/9 cases).Six patients had no striking clinical manifestations,and hypokalemia was found in the treatment of primary disease.2.Laboratory tests:All of the children in this group had hypokalemia and metabolic alkalosis in varying degrees.The activation of renin,angiotensin and aldosterone system increased.3.Therapy:all children were treated by giving potassium supplemental treatment or indomethacin therapy [1 mg/(kg · d),3 times orally].After treatment,all cases achieved clinical improvement and normal blood electrolytes.All patients' blood electrolytes remained normal for 5 to 7 days after stopping treatment.Conclusions 1.In China,the most common cause of children's PBS is gastrointestinal symptoms(such as diarrhea and vomiting) induced by respiratory tract infection.2.Except for clinical manifestations related to causes,patients have no significant clinical manifestations.Hypokalemia can be found in the treatment of primary disease.3.The biochemical results show low blood potassium chloride with metabolic alkalosis.In PBS renin,angiotensin and aldosterone concentration in blood are all elevated.4.Treatment of children's PBS mainly includes etiological treatment and electrolyte supplement therapy.The treatment effectiveness is good after etiological treatment and potassium supplement treatment.In the condition of controlling etiology and potassium supplementation,electrolytes mas return to normal in 2-4 days.