儿童肾脏疾病合并可逆性后部脑病综合征6 例临床分析

目的探讨儿童肾脏疾病合并可逆性后部脑病综合征(PRES)的临床特点。方法回顾分析2015-2018年确诊的6例肾脏疾病合并PRES患儿的临床资料,并复习相关文献。结果 6例患儿中男4例、女2例,年龄3～14岁。原发病分别为肾病综合征2例、紫癜性肾炎2例、过敏性紫癜1例、狼疮性肾炎1例,均有激素及免疫抑制剂应用史。临床表现均为急性起病,抽搐、意识丧失、血压升高,还有精神行为异常、呕吐、头痛、视物障碍表现。影像学表现均以可逆性白质异常病变为特征,多位于半球后部白质。5例患儿恢复至发病前状态,1例死亡。结论肾脏疾病应用激素及免疫抑制剂的患儿易合并PRES,一般预后良好,但并非完全可逆。     

系统性红斑狼疮合并血栓性血小板减少性紫癜1 例分析

目的探讨系统性红斑狼疮(SLE)合并血栓性血小板减少性紫癜(TTP)的临床特点、诊断和治疗。方法回顾分析1例重症SLE合并TTP患儿的临床资料,并复习相关文献。结果患儿,女,13岁,以肾病综合征起病,诊断重症SLE、狼疮性肾炎。初治好转后再现头痛、血小板减少、贫血、急性肾损伤及发热。血涂片破碎红细胞2%,诊断合并TTP。经激素冲击、血浆置换、免疫抑制剂等综合性治疗后病情缓解。结论 SLE合并TTP较为少见,病死率高,早期识别、合理治疗可改善预后。     

儿童系统性红斑狼疮继发抗磷脂综合征及血栓性微血管病1例并文献复习

目的　探讨儿童系统性红斑狼疮（SLE）继发抗磷脂综合征（APS）及血栓性微血管病（TMA）的临床特点。方法　回顾2019-10-29收入重庆医科大学附属儿童医院风湿免疫科的1例SLE继发APS及TMA患儿的临床资料,并结合文献复习对该类疾病临床特征进行分析。结果　患儿女,12岁,病初全身水肿、尿少、皮肤溃疡伴感染,有血小板减少及贫血,肾功能损害,抗核抗体（ANA）及抗心磷脂抗体（aCL）阳性,肾脏病理示弥漫球性增生性狼疮性肾炎Ⅳ-G（A）伴TMA,明确诊断为SLE继发APS及TMA,使用血浆置换、甲基泼尼松龙、环磷酰胺、丙种球蛋白、血液透析滤过及抗凝、抗感染等治疗,患儿放弃治疗后死亡。结合国内外报道13例儿童SLE继发APS患者以及本例患儿资料,14例患儿中,女11例,男3例;14例患儿均发生血栓事件,静脉血栓9例,动脉血栓2例,微血管血栓3例;血小板减少11例,溶血性贫血7例,狼疮性肾炎4例,其中弥漫性增生性狼疮性肾炎Ⅳ2例,膜性肾病2例,1例（本例）抗磷脂综合征肾病,表现为TMA;14例加用激素和（或）免疫抑制剂治疗,10例患儿抗凝治疗,13例患儿病情好转随访无血栓事件复发,仅本例患儿因放弃治疗后死亡,死亡原因肾功能衰竭。结论　SLE合并APS患儿病情重,早期免疫抑制及抗凝治疗可有效缓解病情,血栓事件复发率低,但继发TMA时治疗效果及预后差,病死率高。     

儿童系统性红斑狼疮合并糖尿病4例及远期随访

目的 分析儿童系统性红斑狼疮(JSLE)合并糖尿病(DM)的临床特征并文献复习。方法 收集首都儿科研究所附属儿童医院2002至2008年收治的4例JSLE合并DM患儿的临床资料,总结其临床表现、治疗和预后。结果 4例女性患儿,3例12岁,1例8岁。4例患儿风湿免疫病相关自身抗体阳性,均确诊为JSLE;4例患儿多次查血糖升高,符合DM诊断;2/4例行胰岛素刺激试验和糖耐量试验,1例提示1型糖尿病可能性大,1例2型糖尿病可能性大;3/4例DM发病急剧,出现DM酮症酸中毒的临床表现。2例确诊为甲状腺功能减低。4例患儿均予糖皮质激素或添加免疫抑制剂治疗,狼疮活动均能缓解。1例患儿失访;3例患儿分别随访1、2和5年,其中2例长期应用胰岛素治疗,1例JSLE活动期予胰岛素治疗,病情稳定后通过控制饮食维持血糖正常;血糖均控制满意,生长发育正常。结论 DM可能JSLE的一种表现。糖皮质激素可能不是引起DM的主要原因。在治疗JSLE合并DM时,糖皮质激素是主要的治疗药物。JSLE患儿应及早预防DM的发生,除监测血糖和尿糖外,还应检测胰岛素自身抗体和谷氨酸脱羧酶抗体。     

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目的总结对系统性红斑狼疮(SLE)并发乳糜性腹水的诊断及治疗经验。方法报道中山大学附属第一医院儿科首例SLE并发乳糜性腹水患儿的诊治经过并进行文献复习。结果患儿诊断为SLE、狼疮性肾炎(LN)、溶血性贫血。LN表现为肾病综合征型,肾穿刺活检示LNV+III(A/C),伴有持续严重腹水,腹水检查提示为乳糜性腹水,先后给予甲泼尼龙(MP)、环磷酰胺(CTX)冲击及饮食治疗,狼疮指标好转,腹水消退。查阅相关文献,其发病机制可能与SLE对淋巴网状系统的影响,低蛋白血症致肠壁水肿,淋巴管通透性增加,脂质代谢异常等因素有关。结论 SLE并发乳糜性腹水属罕见病例,对SLE合并难治性腹水,建议行腹腔穿刺腹水检查排除乳糜腹。治疗原则是以原发病变的治疗为主,配合饮食控制等保守治疗。     

儿童肾脏疾病合并后部可逆性脑病综合征临床特点（附4例报告）

目的　提高对儿童肾脏疾病合并后部可逆性脑病综合征（PRES）的临床表现、影像学特征和治疗策略的认识。方法　回顾性分析2010年7月至2018年6月首都儿科研究所附属儿童医院肾脏内科收治的4例肾脏疾病合并PRES患儿的临床资料及影像学结果，结合文献分析其临床特点。结果　患儿原发病分别为急性链球菌感染后肾小球肾炎1例、肾病综合征2例、非典型溶血尿毒综合征1例，起病前有血压控制不良或细胞毒性药物使用史，以抽搐为首发症状，伴有头痛、头晕、视物模糊、血压急剧增高。典型头颅磁共振成像（MRI）示双侧大脑顶枕叶皮质或皮质下白质片状长T1T2 信号，对称或不对称，严重者可累及侧脑室旁、小脑、脑干。4例PRES经过及时降血压和对症治疗后，1～2 d症状缓解，2 ～3周影像学异常完全消失。结论　高血压、药物引起的血管源性脑水肿是发生儿童肾脏疾病合并PRES的主要原因，PRES的诊断依赖于头颅MRI的特征性改变，及时有效的降压和对症治疗可在短期内有效控制病情，治疗中应积极控制高血压，及时停用或减量诱发PRES的细胞毒性药物。     

儿童肾脏疾病合并后部可逆性脑病综合征临床特点（附4例报告）

目的　提高对儿童肾脏疾病合并后部可逆性脑病综合征（PRES）的临床表现、影像学特征和治疗策略的认识。方法　回顾性分析2010年7月至2018年6月首都儿科研究所附属儿童医院肾脏内科收治的4例肾脏疾病合并PRES患儿的临床资料及影像学结果，结合文献分析其临床特点。结果　患儿原发病分别为急性链球菌感染后肾小球肾炎1例、肾病综合征2例、非典型溶血尿毒综合征1例，起病前有血压控制不良或细胞毒性药物使用史，以抽搐为首发症状，伴有头痛、头晕、视物模糊、血压急剧增高。典型头颅磁共振成像（MRI）示双侧大脑顶枕叶皮质或皮质下白质片状长T1T2 信号，对称或不对称，严重者可累及侧脑室旁、小脑、脑干。4例PRES经过及时降血压和对症治疗后，1～2 d症状缓解，2 ～3周影像学异常完全消失。结论　高血压、药物引起的血管源性脑水肿是发生儿童肾脏疾病合并PRES的主要原因，PRES的诊断依赖于头颅MRI的特征性改变，及时有效的降压和对症治疗可在短期内有效控制病情，治疗中应积极控制高血压，及时停用或减量诱发PRES的细胞毒性药物。     

儿童系统性红斑狼疮合并肺高压15例临床分析

目的探讨系统性红斑狼疮(SLE)合并肺高压(PH)患儿的临床和实验室检查特点、诊断治疗及预后。方法选取住院诊断SLE合并PH(SLE-PH)的儿童患者15例,对其临床症状、实验室检查、超声心动图特点、SLE病情活动指标和治疗转归等进行回顾性分析。结果 15例SLE-PH患儿中,从SLE确诊到PH诊断的间隔时间中位数为0.1年(范围:0~6.5年)。除PH相关症状外,40%的患儿合并雷诺现象。反映SLE疾病活动度的指标(如补体C3、C4、ESR水平及抗ds DNA阳性率)在PH轻-中度组与重度组间比较差异无统计学意义。13例患儿接受糖皮质激素及免疫抑制剂治疗,2例同时接受PH靶向药物等治疗。诊断PH后,中位随访时间8.0年(范围:0.5~18.1年),期间2例患儿死亡,其心功能为Ⅲ~Ⅳ级;余13例病情平稳。结论雷诺现象是SLE-PH患儿常见的临床表现。PH轻重程度与SLE疾病活动度无明显关联,应重视SLE患儿肺动脉压筛查。早期诊断、早期治疗有利于改善患儿预后。     

儿童系统性红斑狼疮外周血淋巴细胞亚群变化的临床意义

目的探讨儿童系统性红斑狼疮(SLE)患者外周血淋巴细胞亚群测定的临床意义。方法采用直接四色标记免疫荧光染色法,流式细胞仪检测24例患儿(其中活动期11例,非活动期13例)和20例健康儿童组外周血的淋巴细胞亚群,对比两组检测结果。结果与对照组及非活动期比较,活动期SLE患儿CD3+、CD4+、自然杀伤细胞百分率及CD4+/CD8+比值明显降低;B细胞百分率明显升高;活动期SLE患儿CD8+细胞百分率明显高于对照组,差异均有统计学意义(P<0.05)。结论儿童SLE细胞免疫功能存在异常,淋巴细胞亚群的测定可作为诊断活动期与非活动期SLE患儿一项有价值的参考指标。     

婴幼儿期肾病综合征临床特点分析

目的　探讨婴幼儿期原发性肾病综合征 (简称婴幼儿肾病 )的临床特点 ;分析婴幼儿肾病临床特点、免疫功能、病理分型和糖皮质激素 (简称激素 )疗效的关系。方法　对 31例婴幼儿肾病患儿进行临床观察 ;进行体液免疫和细胞免疫功能测定 ;14例接受肾穿刺活检 ;31例均采用激素中长程疗法 ,18例予以免疫抑制剂如环磷酰胺 (CTX)等联合治疗。结果　婴幼儿肾病临床以肾炎型肾病为主 ;体液免疫和细胞免疫功能下降 ;病理以非微小病变型为主 ;约 6 0 %患儿对激素治疗不敏感 ,需用激素与免疫抑制剂联合治疗。结论　婴幼儿肾病具有与其它儿童肾病综合征不同的特点 ,应当引起临床重视。     

儿童可逆性后部脑病综合征2例报告并文献复习

目的提高对儿童可逆性后部脑病综合征(RPES)的认识。方法回顾性分析首都儿科研究所收治的2例可逆性后部脑病综合征患儿的临床资料,并结合文献进行分析。结果2例患儿原发病分别为系统性红斑狼疮和肾病综合征,均在病程中突然出现头痛、视觉异常、意识障碍、高血压和抽搐等症状。头颅MRI显示双侧大脑顶、颞、枕叶皮层或皮层下片状长T1/T2信号。经过及时降血压和对症治疗后,短期内症状很快缓解,2～3周内影像学异常完全消失。结论RPES的发病机制是多方面的,急剧增高的血压可能是发生RPES的主要原因之一,及时有效的降压治疗可使病情在短期内逆转。     

MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

Background

Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.

Objective

We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.

Materials and methods

We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.

Results

Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.

Conclusion

MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.     

Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.     

Posterior reversible encephalopathy syndrome after kidney transplantation in pediatric recipients: Two cases

PRES is a neuro‐clinical and radiological syndrome that can result as a consequence of several different conditions including hypertension, fluid overload, and immunosuppressive treatment. Herein, we report two children who received kidney and combined liver–kidney transplantation as treatment for renal hypodysplasia associated with bilateral vesico‐ureteral reflux and methylmalonic acidemia, respectively. Early after surgery (seven and 10 days), both patients presented with hypertension and seizures. The patients' immunosuppressive regimen included steroid and calcineurin inhibitors (tacrolimus and cyclosporine, respectively) and basiliximab and one with anti‐IL2 receptor. In both cases, the imaging strongly supported the diagnosis of PRES. In details, the CT scan showed hypodensities in the posterior areas of the brain, and brain MRI demonstrated parieto‐occipital alterations indicative of vasogenic edema. Treatment with calcineurin inhibitors was temporally discontinued and restarted at lower dosage; arterial hypertension was treated with Ca‐channel blockers. Both children fully recovered without any neurological sequels. In conclusion, in children undergoing solid organ transplantation, who develop neurological symptoms PRES, should be carefully considered in the differential diagnosis and once the diagnosis is ruled in, we recommend strict arterial blood pressure control and adjustment or withholding of calcineurin inhibitor therapy should be considered based upon blood levels.     

Posterior Reversible Encephalopathy Syndrome in a Child with Meningitis Presenting with Transient Visual Loss and Hypertension

Posterior reversible encephalopathy syndrome (PRES) is a clinico- radiological syndrome characterized clinically by headache, seizures, visual disturbances, vomiting and radiologically by predominant posterior leukoencephalopathy. The authors report an 11-y-old boy with meningitis presenting with transient hypertension and visual loss and MRI finding compatible with the diagnosis of PRES and complete reversal of symptomatology within 10 d. A wide plethora of tests carried out failed to identify the cause of transient hypertension with a probability of this being due to transient autonomic dysfunction.     

Posterior reversible encephalopathy syndrome in children with cancer

PURPOSE: To identify predisposing factors, radiologic features, and clinical outcome of posterior reversible leucoencephalopathy (PRES) in children receiving cancer treatment. METHODS: We identified 11 patients (7 female) who had radiological and clinical features consistent with PRES and were treated for cancer at St. Jude Children's Research Hospital between January 1995 and January 2005. Clinical and radiographic data were abstracted from their records. RESULTS: The average age at the time of PRES onset was 10.4 years. Primary diagnoses were acute leukemia (n = 8), non-Hodgkin lymphoma (n = 2), and Ewing sarcoma (n = 1). PRES occurred in 8 patients during the induction phase of treatment, and all 11 patients had hypertension (5 chronically). Seizure activity was proximate to cytarabine and tacrolimus administration in three patients and further seizures occurred with re-administration of these medications in two patients. Coagulation and chemistry studies were normal. Concurrent brain magnetic resonance imaging (MRI) demonstrated T2 signal abnormalities in all 11 patients, restricted diffusion in 4, and hemorrhage in 3. Follow-up MRI showed chronic changes consistent with a previous hemorrhage in three and evidence of prior parenchymal ischemia in one. Three patients developed epilepsy and remain on chronic anticonvulsant therapy. CONCLUSIONS: PRES is an increasingly recognized complication of pediatric cancer treatment. Risk factors for PRES in pediatric cancer patients include hypertension (not necessarily acute), remission induction chemotherapy, and administration of tacrolimus. MR images often show atypical findings, some of which are irreversible. A significant number of patients develop epilepsy despite clinical and radiographic evidence of recovery.     

Posterior reversible encephalopathy syndrome and hemorrhage associated with tacrolimus in a pediatric heart transplantation recipient

Posterior reversible encephalopathy syndrome (PRES) is a disorder characterized by gray and white matter abnormalities in the temporal, parietal, and occipital lobes of the brain. Its etiology has been attributed to renal failure, immunosuppressive drugs such as cyclosporine and tacrolimus, and other potential entities leading to acute hypertension. Clinical findings include headaches, altered mental status, seizures, visual changes, and focal neurologic deficits. We report the case of a child who developed PRES with intracerebral and subarachnoid hemorrhages associated with tacrolimus exposure 10 days after heart transplantation for restrictive cardiomyopathy. The patient initially presented with complex partial seizures, headache, agitation, and hypertension. Head MRI was suggestive of PRES along with intracerebral and subarachnoid hemorrhages. Tacrolimus was discontinued and blood pressure was controlled. The patient's encephalopathy resolved, but he has had ongoing neurologic symptoms secondary to hemorrhage. Generally, PRES is less common in children than in the adult population and is a rare complication of calcineurin inhibitors (CNI). Presentation with secondary hemorrhage also can occur. In children receiving CNIs presenting with new neurologic symptoms, PRES should be considered as prompt discontinuation of the offending agent can induce resolution of symptoms. Children can develop hemorrhage in the context of PRES, leading to increased morbidity.     

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??Objective The aim of this study was to summarize the clinical features in children with neuropsychiatric systemic lupus erythematosus??NPSLE?? and to investigate the related risk factors of in systemic lupus erythematosus??SLE?? patients with NPSLE. Methods The clinical data of 14 children with NPSLE were retrospectively analyzed. The indicators including the age?? sex?? clinical manifestations?? laboratory tests were compared with 31 randomly selected SLE patients with non-NPSLE manifestations by univariate and multivariate analysis. Results The main clinical manifestations in children with NPSLE were headache??50%????lateral type motor disorder??50%????mental disorders??35.7%????epilepsy??35.7%????involuntary movement ??14.29%????blurred vision??14.29%?? etc. 13 patients had abnormalities in brain MRI. Univariate analysis showed that children with oral ulcers??blood lymphopenia??thrombocytopenia??elevated CRP??anticardiolipin antibodies positive??antineutrophil cytoplasmic antibody positive??anti-u1-RNP antibody positive??urine red blood cells increased??elevated serum K+ elevated??blood urea nitrogen??elevated serum creatinine were the risk factors with statistical significance. Further non-selective multivariate regression analysis showed that only the anticardiolipin antibody positive?? elevated CRP?? elevated serum creatinine into the multiple regression equation?? a positive correlation. Conclusion Headache?? movement disorders?? mental disorders and seizures were the most common symptoms in children with NPSLE. The brain MRI is a sensitive indicator of checking NPSLE. Anticardiolipin antibody positive?? elevated CRP and elevated serum creatinine were the most important risk factor for SLE children with NPSLE.