肾病综合征并发颅内静脉窦血栓五例

目的 探讨肾病综合征患儿并发颅内静脉窦血栓的诊断,及应用尿激酶与低分子肝素等联合治疗效果.方法 采用尿激酶与低分子肝素等联合治疗.(1)尿激酶:初剂量2000～4000 U/(kg·d),首剂冲击量20 000～40 000 U在15～30 min内滴完,而后将余量用输液泵均匀地泵入,第2天起2000 U/(kg·d)用输液泵均匀地泵入,1个疗程3～7 d.治疗期间每周测定3次凝血酶时间(TT)、激活的部分凝血致活酶时间(APTT),应控制TT和APTT应小于2倍延长的范围内,特别注意有无出血情况.(2)低分子肝素:每次100～120 AXaIU/kg,每天1～2次,腹部皮下注射,2周1疗程.(3)抗血小板凝聚药物:长期口服双嘧达莫3～5 mg/(kg·d),每天2～3次,疗程3个月.结果 5例肾病综合征并发颅内静脉窦血栓,经过尿激酶、低分子肝素与双嘧达莫等联合治疗,1个月后患儿临床症状均消失;复查血浆黏度1个月恢复正常,部分凝血活酶时间、血浆凝血酶原时间、纤维蛋白原降解产物1～2个月后恢复至正常,头颅CT或MRI检查1～3个月后静脉血栓消失,示颅内静脉窦血栓完全再通;随访无复发病例.结论 早期应用尿激酶抗纤溶、低分子肝紊抗凝及抗血小板凝聚药物综合治疗,其溶栓疗效较好.对儿童肾病综合征并发颅内静脉窦血栓的早期诊断与防治具有一定的推广价值.     

Neonatal cerebral venous thrombosis

It is postulated that a vigorous host inflammatory response in the cystic fibrosis lung contributes to lung injury. Tumour necrosis factor-alpha (TNF-alpha) may play a part in that process and in the generation of leukotrienes. Therefore, the relationships between sputum TNF-alpha, leukotriene concentration, and lung function abnormalities in 16 children with cystic fibrosis were investigated. Each subject provided sputum samples and performed spirometry. TNF-alpha was measured by enzyme linked immunosorbent assay; individual leukotrienes were separated using high performance liquid chromatography and quantified by radioimmunoassay. The geometric mean concentration of TNF-alpha was 129.7 pg/ml and 95% confidence interval 48.2 to 348.3. Mean (SEM) leukotriene B4 (LTB4) was 97.8 (22.9) pmol/g and total cysteinyl leukotrienes were 60.9 (14.8) pmol/g. Mean (SD) forced expiratory volume in one second (FEV1) of the group was 53 (15)% of predicted and forced vital capacity (FVC) was 65 (14)% of predicted. There was a significant positive correlation between TNF-alpha and both LTB4 and the total cysteinyl leukotriene sputum content. An inverse relationship existed between TNF-alpha and FEV1 and FVC. Moreover, a negative correlation was observed between sputum LTB4 and FEV1 and FVC. These results suggest that TNF-alpha and the leukotrienes may participate in the airways inflammation and airflow obstruction observed in cystic fibrosis subjects and support the hypothesis that TNF-alpha upregulates the 5-lipoxygenase pathway in vivo.     

Neonatal cerebral venous thrombosis coexisting with bilateral adrenal hemorrhage

We report a case of severe perinatal asphyxia with both cerebral venous thrombosis and adrenal hemorrhage who survived with severe sequela including multicystic encephalomalasia, acquired microcephaly and blindness. Hematological investigations showed normal levels of anticardiolipin antibodies, protein C and S levels and activity, antithrombin III levels. Factor V Leiden mutation was negative. The adrenal hemorrhage resolved within three months with glucocorticoid therapy, the cerebral venous thrombosis resolved within two months without treatment. The literature on neonatal cerebral venous thrombosis is also reviewed.     

儿童颅内静脉窦血栓形成抗凝治疗的队列研究

目的 评估抗凝治疗是否可以改善患儿颅内静脉窦血栓形成(cerebral venous sinus thrombosis,CVST)的临床症状和长期生存质量.方法 资料选取2006年6月至2013年6月收治的符合标准的CVST患儿58例(1个月～16岁).其中,接受抗凝治疗(低分子肝素、华法林、尿激酶)38例,为抗凝治疗组(抗凝组);行非抗凝对症治疗20例,为非抗凝治疗组(非抗凝组).回顾性分析评价两组血栓复发率、抗凝相关出血率及长期预后情况.长期生存质量应用改良Rankin评分进行评估.结果 治疗后出现抗凝治疗相关出血2例(5.3％).复发5例(8.6％)中,抗凝组1例,非抗凝组4例,组间比较,差异有统计学意义(P=0.023).死亡5例(8.6％)中,抗凝组1例,非抗凝组4例,抗凝治疗同单纯对症治疗相比降低病死率25％～30％.长期随访45例(不包括死亡5例及失访8例)临床长期预后不良6例(13.3％),整体预后不良(包括死亡5例和长期预后不良6例)11例(22％).影响长期预后因素中足月儿预后较好(风险比0.12,P=0.045)、颅内病灶广泛预后较差(风险比15.16,P=0.042)和抗凝治疗为主要减少预后不良发生率因素(风险比0.007,P=0.024);起病初期颅高压发生的相关因素中,新生儿窒息史患儿颅高压发生率低(风险比0.35,P=0.025)、颅内病灶广泛发生率高(风险比8.73,P=0.048)和发病时间较短治疗及时的患儿发生颅高压率较低(风险比0.89,P =0.003).同时,抗凝治疗对于亚急性期颅高压控制有一定帮助(P=0.048).结论 对于儿童CVST(包括轻度颅内出血的CVST患儿)抗凝治疗是安全有效的治疗方法,可有效降低其复发率,延长生存时间和改善长期生存质量.     

CT of cerebral venous sinus thrombosis in a child with homocystinuria

We describe a case of cerebral venous sinus thrombosis in a child with homocystinuria. We present both the classic CT findings of cerebral sinus thrombosis, and also the first report of the CT visualization of transdural venous collateral circulation.     

Treatment of neonatal aortic thrombosis with urokinase

We describe the successful treatment of a neonate with severe aortic thrombosis using systemic urokinase. Current knowledge regarding the treatment of this condition, including fibrinolytic therapy, is reviewed.     

儿童原发性肾病综合征合并颅内静脉血栓4例病例报告

目的 通过对原发性肾病综合征合并颅内静脉血栓患儿行临床分析,探讨儿童NS合并颅内静脉血栓早期诊断和治疗的可行方案。方法 纳入2012年1月至2015年9月在上海市儿童医院住院的原发性肾病综合征且经头颅CT和（或）MRI确诊的颅内静脉血栓患儿,对其临床症状、实验室指标、影像学检查结果、疗效及预后进行分析。结果 4例原发性肾病综合征合并颅内静脉血栓患儿进入分析,均为男性,年龄5岁4个月至11岁4个月,出现颅内静脉血栓时间距原发性肾病综合征起病时间为1个月至7年余。4例颅内静脉血栓发病时均有神经精神系统症状,查体均未见神经系统阳性体征。3例在颅内静脉血栓发病期间D-二聚体、纤维蛋白原降解产物（FDP）均升高,抗凝血酶Ⅲ（AT-Ⅲ）下降;确诊后D-二聚体、FDP较前继续升高; 4例血清白蛋白明显降低,总胆固醇明显升高。4例出现颅内静脉血栓临床症状当日或次日均行头颅MRI增强+MRV+MRA检查,3例为左侧乙状静脉窦血栓,1例为脑栓塞。明确颅内静脉血栓诊断后,3例予尿激酶溶栓,低分子肝素钙和双嘧达莫抗凝治疗;1例脑栓塞患儿予对症和抗凝治疗;4例症状均明显改善。3例出院后6~12个月随访头颅MRI增强+MRV显示颅内异常信号均有不同程度吸收。结论 儿童原发性肾病综合征合并 颅内静脉血栓易发生于左侧乙状静脉窦。在原发性肾病综合征病程中如出现神经精神系统症状时,应及时行头颅MRI相关序列检查,有助于颅内静脉血栓早期诊断;早期积极溶栓治疗预后良好。     

肾病综合征合并颅内静脉窦血栓致失明1例报告

目的探讨肾病综合征(NS)合并颅内静脉窦血栓(CVST)致失明患儿的临床表现、治疗方案及其预后。方法分析1例NS合并CVST患儿的临床特点及诊治经过,并复习和总结国内外有关儿童CVST治疗相关研究的最新进展。结果患儿在NS标准化抗凝治疗过程中突然出现抽搐等癫发作症状,并有复视、双眼视力下降、失明,头颅磁共振及脑血管造影检查(MRV)示CVST形成,肝素化抗凝治疗效果不佳,后予尿激酶溶栓治疗1个月,复查MRV示颅内血栓已完全溶解。目前国际上针对CVST患儿已采取标准化抗凝治疗方案,并随时监测凝血功能如活化部分凝血活酶时间(APTT)、国际标准化比值(INR)等预防出血风险。结论临床考虑CVST的患儿需早期及时完善神经影像学检查,在排除出血风险后应立即予以抗凝或溶栓治疗,并按疗程使用3~6个月。     

Neonatal inferior vena cava and renal venous thrombosis treated by thrombectomy and nephrectomy.

Neonatal inferior vena cava and renal venous thrombosis with obstruction was diagnosed clinically and confirmed by ultrasonography. Successful thrombectomy and nephrectomy were performed at 40 hours of age. Thrombus with obstruction occluding the inferior vena cava favours immediate surgery.     

Deep cerebral venous thrombosis in thalamo-ventricular hemorrhage of the term newborn

Unilateral thalamic bleeding with associated intraventricular hemorrhage is reported in three full-term neonates. The first presented within 48 hours from birth with early onset streptococcal meningitis, persistent pulmonary hypertension, tonic seizures and a tense fontanelle. The second presented 6 days after birth with irritability, opisthotonus, a tense fontanelle and tonic seizures. The third was admitted three days after birth with seizures and a tense fontanelle. In the latter two infants NMR and CT imaging documented thrombosed superficial and deep cerebral veins. The etiopathogenesis of intracranial venous thrombosis in the neonate is diverse: asphyxia, dehydration, polycythemia, sepsis-meningitis and difficult delivery are the main causes. In one of our patients jugular vein compression by the collar of a negative-pressure ventilation chamber probably initiated the intracranial events. More than half of the survivors sustain severe neurological impairment.     

Cerebral venous thrombosis during diabetic ketoacidosis.

Neurological deterioration during an episode of diabetic ketoacidosis is usually assumed to be caused by cerebral oedema. We present a case of cerebral venous sinus thrombosis presenting in a similar manner, also associated with severe iron deficiency anaemia. Computed tomography scanning provided the correct diagnosis and allowed institution of anticoagulation with improvement in neurological outcome. Neuroimaging should always be performed in suspected cerebral oedema associated with diabetic ketoacidosis in order to exclude other pathologies.     

Continuous infusion of low-dose urokinase in the treatment of central venous catheter thrombosis in infants and children

Thrombotic occlusion is a frequent complication associated with the use of central venous catheters. The purpose of this study was to evaluate the efficacy of a continuous infusion of low-dose urokinase (200 U/kg/h) in clearing catheters that had not cleared after two bolus doses of urokinase in a pediatric oncology population. Fifty-eight incidents of catheter-related occlusions (49 Hickman-type catheters/nine implantable ports) as documented by radiographic dye study occurred in 227 pediatric oncology patients with 254 central venous catheters during a 1-year period. Fourteen of 58 catheters failed to clear after two bolus instillations of urokinase (5,000 U and 10,000 U). Thirteen catheters were treated for 24 hours with urokinase, 200 U/kg/h, and one catheter with urokinase, 100 U/kg/h for 24 hours. Twelve catheters were used for study. Coagulation studies were monitored preinfusion, 12 hours into the infusion, and postinfusion. Patency was reestablished in 11/12 catheters (92%) with a mean infusion time of 28.7 hours. No coagulation abnormalities or clinical bleeding associated with the urokinase infusion occurred. Only one patient exhibited a prolonged partial thromboplastin time (greater than 150 seconds); this was associated with a heparin effect. These data indicate that low-dose urokinase may be a safe and effective means to clear occluded central venous catheters in children.     

Central alveolar hypoventilation syndrome and cerebral venous thrombosis: fortuitous association?]

Central hypoventilation syndrome is defined as the failure of automatic control of breathing. Secondary central hypoventilation syndrome should distinguish from congenital central hypoventilation syndrome by brainstem abnormalities, place of respiratory control. CASE REPORTS: We report two clinical cases characterized by late onset central hypoventilation syndrome (three years--six months, and five years old): in the first case the diagnosis was made after general anesthesia and the second one presented with acute nocturnal comatose state. Neuroradiologic investigations showed bilateral cerebral sinus veinous thrombosis without any brainstem lesions. Moreover these children had severe behavior disorders: psychomotor instability, alterations of social relations, language dysfunction, and neurocognitive deficit. This symptomatology seems independent from central hypoventilation syndrome and cerebral venous thrombosis. CONCLUSION: Late onset central hypoventilation syndrome may be associated with cerebral venous thrombosis. Ischemia of central chemoreceptors or integration of their informations could be one of mechanism.     

Prophylaxis with urokinase in pediatric oncology patients with central venous catheters

This study evaluated the effects of urokinase in the prevention of central venous catheter (CVC)-related complications in children with malignancy. Fifteen patients with 16 CVCs (study group A) received an intraluminal application of urokinase (10,000 IU in each catheter lumen for 4 h) once a week. They were monitored prospectively with quantitative blood cultures and ultrasonography (color Doppler ultrasound of the great veins and echocardiography). The rate of complications was compared with that of 15 children with 19 CVCs without thromboprophylaxis, treated the previous year (control group B). The authors found a significantly lower incidence of CVC dysfunction (3/16 versus 13/19), no major thrombosis, fewer CVC-related bacteremias (2/16 versus 8/19), and a higher salvage of CVCs (1/16 versus 5/19 CVC removals due to persistent bacteremia) in the thromboprophylaxis group. Asymptomatic thrombosis rate was also lower (7/16 cases in group A versus 9/11 in group B when sonography was performed). No hemorrhagic complications were noted. Thromboprophylaxis with urokinase seems a safe and effective measure for reducing the rate of CVC-related complications.     

Severe Mycoplasma pneumoniae-associated mucositis treated with immunoglobulins

Mycoplasma pneumoniae-associated mucositis (MPAM), previously labelled as atypical Stevens-Johnson syndrome (SJS), SJS with minimal or no skin manifestations, is a rare non-respiratory manifestation of Mycoplasma pneumoniae infection. The nineteen cases described so far in children and young adults were characterized by a high male gender prevalence (16/19) and a good response to appropriate antibiotic treatment and supportive care in the majority of patients. We describe a case of MPAM in a previously healthy girl, who improved after a 0.5 g/kg daily dose of intravenous immunoglobulins (IVIG) for four consecutive days, after traditional therapy had failed. CONCLUSION: The successful treatment with IVIG described in this report suggests that, where appropriate antibiotic and supportive therapy fails to improve the clinical course of severe MPAM, IVIG treatment is worth considering.     

Prolonged bacteremia with catheter-related central venous thrombosis

Infection of a central venous thrombus is a serious but rarely recognized complication of the use of central venous catheters in children. We report the cases of seven children with persistent bacteremia or fungemia in which central venous thrombosis was demonstrated by ultrasonography after removal of the catheter. All patients had signs and symptoms of infection, but only one had clinical evidence of central venous stasis. Bacteremia persisted from 6 to 35 days. Infection did not resolve in any patient prior to catheter removal, and five patients had positive blood cultures for 5 or more days after removal of the catheter. Six patients, including all who survived, were treated parenterally with antibiotics for more than 28 days. Two patients died; neither death was directly attributable to infection. Central venous thrombosis should be suspected in patients with persistent catheter-related bacteremia. Optimal treatment of this problem is not yet known.     

颅内静脉窦血栓

报道1例颅内全静脉窦血栓病例,探讨颅内静脉窦血栓的临床特征和诊治方法。8岁男童,因阵发性头痛,呕吐50d,复视2d就诊,经头颅MRI及磁共振静脉成像(MRV)确诊为颅内全静脉窦血栓。经降颅压、静脉滴注尿激酶和激素治疗,临床症状消失,继续口服华法令治疗。头痛、视乳头水肿是颅内静脉窦血栓的主要表现,头颅MRI和MRV是诊断的主要依据。