大动脉炎致双侧肾动脉狭窄、高血压心脏病猝死一例

患儿,女、13岁,因双膝关节痛5个月,2月前心动过速、心脏增大在外院以“风心、心包炎”收治,用青霉素、强的松、地高辛等50余天,关节痛缓解,心率仍快,双颊渐出现对称性红斑,一周来出现浮肿、气急、高血压,于1984年5月4日转我院。以往健、无发热、咽痛、关节痛、心悸、盗扦和过敏史。家族史无特殊。检查:一般情况尚可,发育营养中等,体温36.5℃、呼吸36次/分,血压左上肢134/100,右上肢138/106,左下肢108/88,右下肢138/106mmHg,面部不肿,双颊有对称性蝶形红     

肾胚胎细胞瘤致肾素增高并发高血压

该文报告1例肾胚胎细胞瘤伴严重高血压时肾素增高,经肿瘤免疫组织化学证实在血管周围有含肾素细胞。病例男,10个月,厌食及全身不适2周。查体:患儿烦燥不安,血压33.3/25.9kPa,脉搏100次/分,左腹巨大不规则包块。生化分析:Na~+ 131mmol/L,K~+ 3.7mmol/L,尿素9.5mmol/L,静脉肾盂造影和CT 证实包块来自左肾。成神经细胞瘤标志的羟甲基扁桃酸和香草扁桃酸分别为16和24μmol/L 肌酸酐(正常)。开始静脉用柳胺心定和肼苯哒嗪,但未降压,故在入院后第9日行剖腹探查术,用开普通降压效果     

双侧多发性周围肾动脉狭窄所致高血压症1例报告

小儿高血压症中约80%为继发性,其中肾血管性占6～13%。肾动脉的肌纤维发育不良为最多见的病因,他所造成的狭窄在肾动脉的主干部为多。本文报告1例比较少见的双侧周围肾动脉多发性狭窄引起的高血压。病例7岁,女。出生前、出生时无异常。2岁7个月时发现心脏有杂音。3岁2个月时心导管检查诊断为Seller'sⅡ度二尖瓣返流和高血压症。3岁7个月时作肾图、肾闪烁图可见右肾血流降低,疑诊肾血管性高血压。但腹主动脉造影无异常。7岁时为确诊而入院。入院时检查:心率60次/分,律整。BP164/104mmHg。心尖部听到LevineⅢ度收缩期杂音,腹部无血管杂音,肝脾未触及。周围血、B U N、总胆固醇正常。尿常规检查正常。血、尿儿苯酚胺正常,周围     

儿童肾动脉纤维肌发育不良致难治性高血压1例报告

目的探讨儿童肾动脉纤维肌发育不良(FMD)致难治性高血压的临床特点、诊断和治疗。方法回顾分析1例FMD致难治性高血压患儿的临床资料。结果患儿,男,4岁起病,以血压明显升高为突出表现,同时伴血钾降低,肾素、血管紧张素明显升高,经血管彩色超声和胸腹增强CT加血管重建,诊断为肾血管性高血压。经口服多种降压药效果欠佳,行肾血管造影后诊断为FMD所致难治性高血压。因不宜手术,调整降压方案,血压趋于稳定。连续随访8年,每年调整降压方案并动态评估血压波动及靶器官受损情况。结论儿童FMD所致肾血管性高血压初期可无特异性表现,首选药物治疗,并可考虑外科治疗。     

儿童先天性肾发育不全致高血压6例

目的 报道6例肾发育不全所致肾血管性高血压。方法回顾13年间共收治6例，女4例，男2例，均行发育不全的肾脏切除治疗。结果术中见肾脏小，肾动脉全程狭窄，直径0．1～0．2cm。病理检查示肾实质薄，肾小球数目减少，肾动脉壁未见炎性细胞浸润，无纤维肉芽组织增生。术后血压下降，随访50天～1年，血压均恢复正常，1例患儿术后8个月因合并大动脉炎致对侧肾动脉狭窄，血压回升。结论肾发育不全致高血压，手术切除患肾，效果良好。     

儿童先天性肾发育不全致高血压6例

目的 探讨肾发育不全所致肾血管性高血压的治疗。方法 回顾性分析13年间收治的6例患儿的诊断与手术治疗。结果 6例患儿均行手术治疗，术中见肾脏小，肾动脉全程狭窄，直径0．1—0．2cm，遂将发育不全的肾脏切除。病理检查示肾实质薄，肾小球数目减少，肾动脉壁未见炎性细胞浸润，无纤维肉芽组织增生。术后血压下降，随访50d—1年，血压均恢复正常，1例患儿术后8个月因合并大动脉炎致对侧肾动脉狭窄，血压回升。结论 肾发育不全致高血压，手术切除患肾，效果良好。     

乳头皲裂致咽血综合征长期延误诊断一例

患儿，男，26天，足月顺产，母乳喂养，无家族性出血倾向史。因间歇性呕血、黑便10余天于1989年4月11日2：20拟：“新生儿自然出血症”收住儿科。查体：足月新生儿貌，无明显贫血貌，全身皮肤无出血点，四肢关节无肿胀，前卤平软，巩膜无黄染，口腔粘膜无出血及破溃，颈软，两肺无罗音，心率：120次／分，律齐、无杂音，腹手软，肝脾不大，无腹壁静脉怒张，NS（－）。血常规：Hb：13g、WBC：11300／mm3、N：28％、L：60％、M：4％、E：2％、BPC：22万／mm3，大便隐血：（），凝血酶原时间：12秒（奎氏法），钡剂胃肠镜检：（－）。患…     

双主动脉弓致气管狭窄一例

患儿,男,4个月.因喘鸣4个月,咳嗽5 d入院.患儿出生后一直有喘鸣,家长未予重视,未治疗.患儿入院前5 d无明显诱因出现咳嗽,呈阵发性,较剧烈,伴痰鸣、气促,无发绀,无发热.在我院门诊予头孢噻肟、地塞米松等治疗,但效果不佳.入院时体检:体温正常,R 38次/min,P 130次/min,神志清,精神可,营养发育可,呼吸稍促,轻度三凹征,无发绀,咽部充血,双肺呼吸音粗,可闻及哮鸣音及痰鸣音,心音有力,心律齐,心前区未及明显病理性杂音,腹平软,肝肋下约1.5 cm,质软边锐,脾脏肋下未及,四肢肌张力正常,脑膜刺激征阴性.     

双主动脉弓致气管狭窄一例

患儿,男,4个月.因喘鸣4个月,咳嗽5 d入院.患儿出生后一直有喘鸣,家长未予重视,未治疗.患儿入院前5 d无明显诱因出现咳嗽,呈阵发性,较剧烈,伴痰鸣、气促,无发绀,无发热.在我院门诊予头孢噻肟、地塞米松等治疗,但效果不佳.入院时体检:体温正常,R 38次/min,P 130次/min,神志清,精神可,营养发育可,呼吸稍促,轻度三凹征,无发绀,咽部充血,双肺呼吸音粗,可闻及哮鸣音及痰鸣音,心音有力,心律齐,心前区未及明显病理性杂音,腹平软,肝肋下约1.5 cm,质软边锐,脾脏肋下未及,四肢肌张力正常,脑膜刺激征阴性.     

胃粘膜迷路致食管狭窄一例

胃粘膜迷路致食管狭窄一例曾骐患儿：男，４岁。因生后反复呕吐，进行性加重而入院。患儿生后偶有呕吐，逐渐加重，１岁时不能进半流质。在当地医院按“胃炎”治疗，每服碱性药后症状缓解，近３年来呕吐渐频繁，呕吐物偶带咖啡色，且服碱性药后不缓解。无服强酸、强碱史。...     

Polycythaemia and hypertension caused by renal artery stenosis

A girl with failure to thrive and a haemoglobin of 140 g/l at 1.3 years died from a brain haemorrhage 2.5 years later. Renal artery stenosis had caused severe, chronic hypertension and increased erythropoietin secretion (haemoglobin 182 g/l). Blood pressure should be measured in all unwell children, including those failing to thrive.     

Polycythaemia and hypertension caused by renal artery stenosis.

A girl with failure to thrive and a haemoglobin of 140 g/l at 1.3 years died from a brain haemorrhage 2.5 years later. Renal artery stenosis had caused severe, chronic hypertension and increased erythropoietin secretion (haemoglobin 182 g/l). Blood pressure should be measured in all unwell children, including those failing to thrive.     

Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography

Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patient with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.     

Kawasaki disease complicated by renal artery stenosis

We report the case of a child who developed severe renovascular hypertension six months after acute Kawasaki disease. The hypertension was well controlled with enalapril, but there was a gradual decrease in function of the affected kidney. The lesion, an ostial stenosis of the right main renal artery, was not amenable to percutaneous balloon angioplasty, so was treated with bypass surgery. Vasculitis is an important cause of renovascular hypertension in children. This case highlights the importance of regular blood pressure monitoring in children with a history of systemic vasculitis.     

Kawasaki disease complicated by renal artery stenosis.

We report the case of a child who developed severe renovascular hypertension six months after acute Kawasaki disease. The hypertension was well controlled with enalapril, but there was a gradual decrease in function of the affected kidney. The lesion, an ostial stenosis of the right main renal artery, was not amenable to percutaneous balloon angioplasty, so was treated with bypass surgery. Vasculitis is an important cause of renovascular hypertension in children. This case highlights the importance of regular blood pressure monitoring in children with a history of systemic vasculitis.     

Polyarteritis nodosa with renal artery stenosis

Renal angiographic signs in Polyarteritis Nodosa (PAN) vary from aneursyms of medium and small vessels, perfusion defects and delayed emptying of renal arteries. These vascular changes are usually responsible for the hypertension. In this case study stenosis of a main renal artery, an unusual finding in classical PAN, is believed to be the cause of hypertension. Hence renal angiography is essential to define the renal vascular changes and confirm the cause of hypertension.     

先天性肺静脉狭窄的诊断和治疗

目的探讨先天性肺静脉狭窄的诊断和治疗.方法应用心导管和心血管造影、超声心动图及磁共振确定诊断.结果5例中管状狭窄4例,隔膜性狭窄1例;5例狭窄部位均在肺静脉与左心房连接处.三支肺静脉狭窄1例,二支肺静脉狭窄2例,一支肺静脉狭窄2例.合并其他先天性心脏病4例.结论早期发生原因难以解释的严重肺动脉高压的先天性心脏病,应考虑合并肺静脉狭窄.心导管和心血管造影及超声检查可明确诊断.外科手术疗效尚不满意,需继续研究探讨.