中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2014年
5期
362-367
,共6页
刘金荣%姚春美%徐保平%沈文彬%周春菊%段晓岷%周锦%安然%王维
劉金榮%姚春美%徐保平%瀋文彬%週春菊%段曉岷%週錦%安然%王維
류금영%요춘미%서보평%침문빈%주춘국%단효민%주금%안연%왕유
乳糜胸%淋巴管畸形%放射性核素显像
乳糜胸%淋巴管畸形%放射性覈素顯像
유미흉%림파관기형%방사성핵소현상
Chylothorax%Lymphatic abnormalities%Radionuclide imaging
目的 探讨2例原发性淋巴管发育异常导致乳糜胸患儿的临床特点和诊治过程.方法 对首都医科大学附属北京儿童医院呼吸二科收治的经核素淋巴管显影扫描明确诊断的2例原发性淋巴管发育异常患儿的临床资料进行回顾性分析,并进行相关文献复习.结果 例1男,2岁7个月,因“呼吸急促43 d,发热、咽痛5d”入院.发病前曾有外伤史.体格检查:呼吸28～30次/min,左侧胸廓较右侧稍膨隆,左下肺呼吸音减弱,双肺未闻及哕音.胸腔积液暗红色(静置后分为2层:上层为乳白色液体,底层为血性液体),白细胞9 000×106/L,单核细胞0.9,多核细胞0.1;甘油三酯12.37 mmol/L.胸部增强CT示左侧胸腔积液、心包积液.核素淋巴管显影扫描示原发性淋巴管发育异常(胸导管颈胸段显影增宽紊乱,双侧静脉角持续增宽显影,提示胸导管畸形,双侧静脉角引流并出口梗阻).例2男,9岁6个月,因“咳嗽24d,间断发热、呕吐、腹痛19 d,双下肢浮肿4d”入院.体格检查:眼睑略水肿,腹稍膨隆,腹痛拒按,双下肢凹陷性水肿,阴囊水肿明显.血清白蛋白14 g/L.肺炎支原体抗体1:320.胸腔积液乳白色,白细胞10×106/L,甘油三酯0.82 mmol/L;腹腔积液乳白色,白细胞74×106/L,单核细胞0.78,多核细胞数0.22,甘油三酯1.01 mmol/L.胸腔积液、腹腔积液乳糜试验均阳性.胸部X线示右下肺少许斑片状阴影,左下心影后略致密,右侧胸腔积液.腹部B超提示胃黏膜巨大肥厚症、大量腹腔积液.胃镜提示胃黏膜巨大肥厚症？核素淋巴管显影扫描示原发性淋巴管发育异常(胸腹腔放射性轻度增高,10 min可见胸导管末段及左静脉角增宽显影).结论 原发性淋巴管发育异常可见于儿童,常因呼吸道感染就诊而发现原发病,可导致多浆膜腔积液(乳糜胸、乳糜性心包积液、乳糜腹).浆膜腔积液可为血性或乳白色,白细胞升高,以淋巴细胞为主,甘油三酯常高于1.0 mmol/L.核素淋巴管显影扫描可以协助诊断本病.
目的 探討2例原髮性淋巴管髮育異常導緻乳糜胸患兒的臨床特點和診治過程.方法 對首都醫科大學附屬北京兒童醫院呼吸二科收治的經覈素淋巴管顯影掃描明確診斷的2例原髮性淋巴管髮育異常患兒的臨床資料進行迴顧性分析,併進行相關文獻複習.結果 例1男,2歲7箇月,因“呼吸急促43 d,髮熱、嚥痛5d”入院.髮病前曾有外傷史.體格檢查:呼吸28～30次/min,左側胸廓較右側稍膨隆,左下肺呼吸音減弱,雙肺未聞及噦音.胸腔積液暗紅色(靜置後分為2層:上層為乳白色液體,底層為血性液體),白細胞9 000×106/L,單覈細胞0.9,多覈細胞0.1;甘油三酯12.37 mmol/L.胸部增彊CT示左側胸腔積液、心包積液.覈素淋巴管顯影掃描示原髮性淋巴管髮育異常(胸導管頸胸段顯影增寬紊亂,雙側靜脈角持續增寬顯影,提示胸導管畸形,雙側靜脈角引流併齣口梗阻).例2男,9歲6箇月,因“咳嗽24d,間斷髮熱、嘔吐、腹痛19 d,雙下肢浮腫4d”入院.體格檢查:眼瞼略水腫,腹稍膨隆,腹痛拒按,雙下肢凹陷性水腫,陰囊水腫明顯.血清白蛋白14 g/L.肺炎支原體抗體1:320.胸腔積液乳白色,白細胞10×106/L,甘油三酯0.82 mmol/L;腹腔積液乳白色,白細胞74×106/L,單覈細胞0.78,多覈細胞數0.22,甘油三酯1.01 mmol/L.胸腔積液、腹腔積液乳糜試驗均暘性.胸部X線示右下肺少許斑片狀陰影,左下心影後略緻密,右側胸腔積液.腹部B超提示胃黏膜巨大肥厚癥、大量腹腔積液.胃鏡提示胃黏膜巨大肥厚癥？覈素淋巴管顯影掃描示原髮性淋巴管髮育異常(胸腹腔放射性輕度增高,10 min可見胸導管末段及左靜脈角增寬顯影).結論 原髮性淋巴管髮育異常可見于兒童,常因呼吸道感染就診而髮現原髮病,可導緻多漿膜腔積液(乳糜胸、乳糜性心包積液、乳糜腹).漿膜腔積液可為血性或乳白色,白細胞升高,以淋巴細胞為主,甘油三酯常高于1.0 mmol/L.覈素淋巴管顯影掃描可以協助診斷本病.
목적 탐토2례원발성림파관발육이상도치유미흉환인적림상특점화진치과정.방법 대수도의과대학부속북경인동의원호흡이과수치적경핵소림파관현영소묘명학진단적2례원발성림파관발육이상환인적림상자료진행회고성분석,병진행상관문헌복습.결과 례1남,2세7개월,인“호흡급촉43 d,발열、인통5d”입원.발병전증유외상사.체격검사:호흡28～30차/min,좌측흉곽교우측초팽륭,좌하폐호흡음감약,쌍폐미문급홰음.흉강적액암홍색(정치후분위2층:상층위유백색액체,저층위혈성액체),백세포9 000×106/L,단핵세포0.9,다핵세포0.1;감유삼지12.37 mmol/L.흉부증강CT시좌측흉강적액、심포적액.핵소림파관현영소묘시원발성림파관발육이상(흉도관경흉단현영증관문란,쌍측정맥각지속증관현영,제시흉도관기형,쌍측정맥각인류병출구경조).례2남,9세6개월,인“해수24d,간단발열、구토、복통19 d,쌍하지부종4d”입원.체격검사:안검략수종,복초팽륭,복통거안,쌍하지요함성수종,음낭수종명현.혈청백단백14 g/L.폐염지원체항체1:320.흉강적액유백색,백세포10×106/L,감유삼지0.82 mmol/L;복강적액유백색,백세포74×106/L,단핵세포0.78,다핵세포수0.22,감유삼지1.01 mmol/L.흉강적액、복강적액유미시험균양성.흉부X선시우하폐소허반편상음영,좌하심영후략치밀,우측흉강적액.복부B초제시위점막거대비후증、대량복강적액.위경제시위점막거대비후증？핵소림파관현영소묘시원발성림파관발육이상(흉복강방사성경도증고,10 min가견흉도관말단급좌정맥각증관현영).결론 원발성림파관발육이상가견우인동,상인호흡도감염취진이발현원발병,가도치다장막강적액(유미흉、유미성심포적액、유미복).장막강적액가위혈성혹유백색,백세포승고,이림파세포위주,감유삼지상고우1.0 mmol/L.핵소림파관현영소묘가이협조진단본병.
Objective To analyze the clinical characteristics and diagnosis of 2 cases with chylothorax due to primary lymphatic dysplasia and to elevate pediatrician's recognition level for this disease.Method Clinical manifestations of the children were retrospectively analyzed.Primary lymphatic dysplasia was diagnosed by lymphoscintigraphy.Result The first patient was a male aged 2-year-7-month who presented with a history of tachypnea for 43 days,fever and sore throat for 5 days at the early stage of the illness.He had a history of external injury before his illness.Physical examination showed his left chest bulging and left side diminished breath sound.His pleural effusion showed dark red(It was divided into two layers after standing,the upper layer turned into milky white,and the lower turned into hemorrhagic liquid).White blood cell (WBC) count was 9 000 × 106/L,mononuclear cell was 0.9,polykaryocytes was 0.1,triglyceride was 12.37 mmol/L in the pleural effusion.Contrast-enhanced lung CT (revascularization)showed pericardial effusion and a massive left sided pleural effusion.The second patient was a male aged 9 years and 6 months,who presented with a history of cough for 24 days,intermittent fever,vomiting,abdominal pain for 19 days,and edema of lower limbs for 4 days.Physical examination showed edema in both eyelids,lower extremities and scrotum.The level of albumin was 14 g/L and the titer of Mycoplasma pneumoniae IgM was 1:320 in the serum.His hydrothorax pleural effusion showed milk white.White blood cell (WBC) count was 74 × 106/L,mononuclear cell was 0.78,polykaryocytes was 0.22,triglyceride was 1.01 mmol/L in the pleural effusion.Chyle test showed positive in his pleural effusion and seroperitoneum.High-resolution CT of the lung revealed bilateral interstitial and parenchymal infiltration and both sided pleural effusion.Abdominal ultrasound showed giant hypertrophy of the gastric mucosa and massive ascites.Gastroscopy showed giant hypertrophy of the gastric mucosa.Lymphoscintigraphy revealed primary lymphatic dysplasia in both children.Conclusion Primary lymphatic dysplasia might occure in children and result in dropsy of serous cavity (chylothorax,chylopericardium,chylous ascites).Dropsy of serous cavity showed bloody or milk white.WBC count might elevate with lymphocyte increasing mostly,triglyceride was often higher than 1.0 mmol/L in dropsy of serous cavity.Primary lymphatic dysplasia can be diagnosed by lymphoscintigraphy.
