小儿结节性多动脉炎的诊断标准

结节性多动脉炎(PAN)是累及中、小动脉的血管炎综合征,临床表现不一,难与其他风湿病、血管炎综合征区别。该文对确诊为 PAN 病例的临床及预后进行回顾性研究。方法 1979～1991年诊治31例 PAN,其中8例     

儿童期系统性血管炎

血管炎在组织学上定义为血管壁存在纤维素样坏死和炎症，按照主要受累血管的大小可分为大血管炎如大动脉炎；中血管炎如结节性多动脉炎，川崎病；小血管炎如过敏性紫癜，抗中性粒细胞胞浆抗体相关性血管炎等。儿童系统性血管炎包括一组异质性疾病，病因不清，发病机制、组织学、临床表现及预后各不相同。川崎病，过敏性紫癜主要见于儿童期，近年来对其病因、发病机制、治疗和预后进行了大量研究；而小血管炎中韦格纳肉芽肿病、显微镜下多血管炎和变应性肉芽肿性血管炎(Churg-Strauss综合征)可发生于任何年龄，儿童少见，临床资料有限、不为多数儿科医生昕认识、而早期诊断，选择恰当的治疗方法可避免或延缓不可逆损害的发生，使病人的长期生存率得到改善。     

儿童期系统性血管炎

血管炎在组织学上定义为血管壁存在纤维素样坏死和炎症,按照主要受累血管的大小可分为大血管炎如大动脉炎;中血管炎如结节性多动脉炎,川崎病;小血管炎如过敏性紫癜,抗中性粒细胞胞浆抗体相关性血管炎等。儿童系统性血管炎包括一组异质性疾病,病因不清,发病机制、组织学、临床表现及预后各不相同。川崎病,过敏性紫癜主要见于儿童期,近年来对其病因、发病机制、治疗和预后进行了大量研究;而小血管炎中韦格纳肉芽肿病、显微镜下多血管炎和变应性肉芽肿性血管炎(Churg Str ss综合征)可发生于任何年龄,儿童少见,临床资料有限,不为多数儿科医生所认识,而早期诊断,选择恰当的治疗方法可避免或延缓不可逆损害的发生,使病人的长期生存率得到改善。     

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。     

小儿胰母细胞瘤一例

患儿:男,9岁,因发现上腹部包块并偶感疼痛1年入院。腹部包块逐渐增大,脐上可触及。患儿无发热、畏寒;无恶心、呕吐;无腹泻、腹胀等其他不适。检查:腹部平坦,无腹壁静脉曲张,脐上可触及约11cm×10cm质韧包块,边界欠清楚,表面不光滑,轻度压痛,活动度差,未闻及血管杂音。CT扫描提示胰腺占位性病变,约10cm×9cm,包块与整个胰腺分界不清。彩色B超提示包块表面有动脉频谱,血管丰富。入院诊断为:胰腺肿瘤。行剖腹探查术,术中探查:胰头、胰体可触及大结节肿物,长约12cm,色黄白,胰腺增宽、增厚,仅尾部约2~3cm质地正常。肠系膜血管起始部可及多个淋…     

儿童颈内动脉颅外段巨大梭形动脉瘤一例

患儿：女，13岁。因颈部包块1年，快速增大2个月入院。患儿于入院前1年无意间发现左颈部无痛搏动性包块，2个月前包块突然明显增大，无头痛、头昏、晕厥等。既往无颈部外伤、感染等病史，无家族史。检查：发育正常，营养中等。左颈部近下颌角处可触及一个3．5cm×6cm大小包块，随脉搏搏动，无震颤，听诊无血管杂音。神经系统检查无异常。实验室检查：血常规、梅毒血清学、细菌学检查无异常。     

儿童结节性多动脉炎1例

结节性多动脉炎(polyarteritis nodosa,PAN)是一种少见疾病,平均发病年龄大于40岁。该病主要累及中小肌性动脉,以血管中层改变最为明显,属于自身免疫性疾病。因可损伤各个组织器官的血管,所以临床表现差异很大,常见有发热、皮疹和受累器官功能障碍等[1]。本病在儿童期发病更少见,吉林大学第一医院于2010年收治了1例PAN患儿。现报道如下。     

新生儿处女膜闭锁并子宫阴道积液一例

患儿女,因"宫内B超提示下腹部包块"生后转入我科。患儿母孕32周时B超发现胎儿下腹部包块,怀疑肛门闭锁,孕40周B超仍提示下腹部包块,同时提示左肾中-重度积水,行剖宫产分娩。入院查体:左下腹触及8cm×8cm包块,质中,哭闹或腹腔压力增高时阴道口可见肿物突出,表面为肉色且光滑,边缘相对清楚,安静后回缩,肛门通畅。生后出现腹胀,经禁食、通便后好转,开奶顺利,无呕吐,大小便正常。入院后B超提示下腹探及一囊性包块,约8.7cm×4.5cm,内回声不均,见散在漂浮点,子宫及附件显示不清,双肾盂积水;腹部CT回报:(1)考虑先天性子宫阴道积液,无孔处女膜闭锁可能性     

新生儿脑实质内中枢神经细胞瘤一例

患儿:男,17d.足肉顺产,无出生窒息史,因生后2d无明显诱因下出现抽搐,喷射样呕吐,到当地医院就诊,给予内科治疗后,症状无改善,到我院就诊.检查:神志清,反应可,头颅无畸形,前囟直径2cm,略饱满,双瞳孔等大等圆,对光反射灵敏,颈软,无抵抗,四肢肌力肌张力正常,病理反射未引出.术前MR:左颞叶可见一不规则混杂信号包块,T1W周围可见环状高信号,中心呈片状低信号;T2W呈不均匀高信号;DWl周边可见环状高信号,中心呈片状低信号.周围脑白质可见T1W低信号、T2W高信号水肿.增强后肿块边缘稍强化,内部强化不明显,左顶脑膜强化明显.左侧脑室受压变小,右侧脑室变大.MRA:左侧大脑前、中动脉较右侧稍细.MR诊断:左侧颞叶不均质包块,巨大血肿可能,肿瘤待排.     

Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.     

Renal‐limited necrotizing granulomatous vasculitis in a pediatric patient

A 10‐year‐old girl presented with mild proteinuria and hypertension. Laboratory data indicated slightly elevated serum creatinine (0.67 mg/dL) and elevated serum IgG (2111 mg/dL). On renal arteriography mild stenosis over the entire length of the right renal artery and irregular stenosis of the interlobar arteries in the right kidney were seen. She was diagnosed with renovascular hypertension, and received conventional anti‐hypertensive therapy, but did not respond to them. The right kidney had atrophy and dysfunction on technetium‐99m‐labeled dimercaptosuccinic acid renal scintigraphy, and was therefore resected. Histopathology of the kidney indicated severe necrotizing granulomatous vasculitis affecting the arteries from the renal hilus to the interlobar area. After nephrectomy plus steroid pulse therapy, blood pressure and urinary protein returned to normal. To our knowledge, this is the first report of necrotizing granulomatous vasculitis limited to the medium‐sized renal arteries.     

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.     

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium‐sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium‐sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14‐year‐old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis‐related PAN facilitates early diagnosis, and instigation of appropriate treatment.     

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.     

"STRESS" POLYCYTHAEMIA AND PERIPHERAL FACIAL PALSY COMPLICATIONS OF SEVERE HYPERTENSION

Abstract. An 11-month-old boy had an episode of generalized convulsions followed by a right peripheral facial palsy, which resolved gradually within 3 weeks. Three months later he had another similar episode of convulsions followed by a left peripheral facial palsy. On both occasions it was found that he had polycythaemia. A careful physical examination discovered that the child had severe hypertension. Extensive laboratory investigations did not reveal a cause for his hypertension. Haematologic investigations showed that the polycythaemia was due to a contracted plasma volume as a result of the hypertension. The peripheral facial palsy most probably was due to a blood clot in the facial canal, below the origin of the nerve to m. stapedius, as audiograms were normal and lacrimation preserved. Control of the hypertension resulted in resolution of the facial palsy within 4 weeks and normal haematocrit readings within 6 weeks. It should be stressed that every patient with peripheral facial palsy should be examined for hypertension.     

"Stress" polycythaemia and peripheral facial palsy complications of severe hypertension.

An 11-month-old boy had an episode of generalized convulsions followed by a right peripheral facial palsy, which resolved gradually within 3 weeks. Three months later he had another similar episode of convulsions followed by a left peripheral facial palsy. On both occasions it was found that he had polycythaemia. A careful physical examination discovered that the child had severe hypertension. Extensive laboratory investigations did not reveal a cause for his hypertension. Haematologic investigations showed that the polycythaemia was due to a contracted plasma volume as a result of the hypertension. The peripheral facial palsy most probably was due to a blood clot in the facial canal, below the origin of the nerve to m. stapedius, as audiograms were normal and lacrimation preserved. Control of the hypertension resulted in resolution of the facial palsy within 4 weeks and normal haematocrit readings within 6 weeks. It should be stressed that every patient with peripheral facial palsy should be examined for hypertension.     

Intrarenal aneurysm of the renal artery in children

Two children with hypertension had intrarenal aneurysms in the renal artery. In both children, renovascular hypertension was documented by renal vein renin assay showing elevated renin secretion from the involved kidney and suppression of renin secretion from the contralateral kidney. Transcatheter embolization of a segmental artery cured the hypertension in one case. A nephrectomy performed in the other case, because of multiple intrarenal aneurysms, was curative. Intrarenal renal artery aneurysms are an uncommon cause of renovascular hypertension in children. The aneurysms may be mycotic or congenital in origin, or secondary to diffuse vasculitis. Although spontaneous resolution of the aneurysms may occur when due to vasculitis, treatment of most aneurysms is usually necessary. Transcatheter embolization of solitary aneurysms is an attractive alternative to partial nephrectomy, since it allows maximal preservation of renal parenchyma with minimal morbidity.