Stenosing ureteritis in Henoch–Schönlein purpura: Report of two cases

Stenosing ureteritis (SU), a rare complication of Henoch–Schönlein purpura (HSP), typically presents with severe symptoms. We report the cases of two HSP patients presenting with gross hematuria, blood clotting, and colicky flank pain, followed by purpura on the lower extremities. Early‐stage ultrasonography indicated hydronephrosis, thickened renal pelvic mucous membrane, and ureteral dilatation (UD), suggesting HSP complicated with SU. After early SU treatment with prednisolone, kidney function, thickened renal pelvic mucous membrane, and UD progressively normalized and the pain gradually disappeared. Regular ultrasonography of HSP patients from the onset of gross hematuria can be useful to detect early SU and facilitate conservative therapy with prednisolone. Diagnosis of SU can be easily missed by assuming HSP nephritis, particularly owing to the non‐specific symptoms. Common characteristics as well as treatment methods and prognosis of SU are given in the literature review.     

免疫性血小板减少性紫癜合并IgA肾病

目的：认识免疫性血小板减少性紫癜（ITP）合并IgA肾病这一新的临床病症，并对其可能的机制进行探讨。方法：回顾分析3例ITP合并IgA肾病患者的临床病理特征及治疗后的转归。结果：3例患者均经骨髓穿刺确诊为ITP，肾活检确认为IgA肾病。经激素等治疗后，ITP和IgA肾病均可得到缓解。结论：ITP可以合并IgA肾病。IgA肾病的发生可以继发于IPT，也不排除二者均源于骨髓干细胞功能异常的可能性。     

糖皮质激素治疗慢性特发性血小板减少性紫癜疗效相关因素分析

目的：探讨影响糖皮质激素治疗慢性特发性血小板减少性紫癜疗效的有关因素。方法：采用Logistic回归对74例经糖皮质激素治疗的慢性特发性血小板减少性紫癜患者性别、年龄、是否并发除皮肤出血以外较严重的出血表现、起病至开始治疗时间、是否有脾脏肿大、是否并发肝炎病毒感染、治疗前血小板计数、骨髓巨核细胞数8项因素进行多因素分析。结果：起病至开始治疗时间，是否并发肝炎病毒感染，骨髓巨核细胞数是影响糖皮质激素治疗慢性特发性血小板减少性紫癜疗效的相关因素。结论：分析患者治疗前各项可能影响疗效的相关因素，对于指导慢性特发性血小板减少性紫癜治疗方案的选择，尤其是合理掌握糖皮质激素的使用时间有重要意义。     

Chylothorax in Henoch-Schonlein purpura: a case report and review of the literature

Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While renal abnormalities in HSP are common, the classic pulmonary manifestations, such as hemorrhage and pneumonitis, are thought to be infrequent. Subclinical pulmonary manifestations, including diffusion defects and radiographic anomalies, seem to be quite frequent in patients with HSP but are not commonly reported. Other respiratory manifestations include pleural effusion and chylothorax, but these are rarely mentioned in the literature. Chylothorax was only reported once in an adult patient with HSP in whom the mechanism of formation was demonstrated to be secondary to transdiaphragmatic passage of chylous fluid from the peritoneal cavity. Here we describe an 8-year-old girl with HSP, nephrotic syndrome, and chylothorax, and we report the results of a review of the literature regarding respiratory complications in HSP. The present case is the first pediatric patient reported with HSP and chylothorax. The therapeutic measures utilized were effective in resolving her edema, ascites, and chylothorax, and we advocate the use of these measures as first-line therapy in future patients with similar complications from HSP.     

尿微量蛋白和尿酶测定在过敏性紫癜患儿早期肾损伤中的临床意义

目的探讨尿微量清蛋白(MA)和尿酶测定在过敏性紫癜患儿早期肾损伤中的临床意义。方法选取120例过敏性紫癜患者作为研究组,过敏性紫癜患儿根据有无肾功损害分为早期肾损伤组和无损伤组,另选取同期该院行健康查体的40例健康儿童作为健康对照组,分析比较各组间尿MA和尿酶水平。结果研究组患者的MA为(17.5±3.8)mg/L、转铁蛋白(TRF)为(0.56±0.34)mg/L、β2-微球蛋白为(β2-MG)为(0.35±0.07)mg/L、N-乙酰-B-D-氨基-葡萄糖苷酶(NAG)为(18.6±5.1)U/L,显著高于健康对照组,差异有统计学意义(P0.05);早期肾损伤组MA(42.1±21.3)mg/L、TRF(1.65±0.54)mg/L、β2-MG(0.43±0.06)mg/L、NAG(23.1±5.12)U/L,显著高于无损伤组,差异有统计学意义(P0.05)。结论尿MA和尿酶检测可以作为监测过敏性紫癜患儿早期肾损伤的指标,有利于早期发现、及时干预,减少并发症的发生。     

幽门螺杆菌感染与儿童过敏性紫癜相关性的 Meta分析

目的：综合分析幽门螺杆菌（Hp）感染与儿童过敏性紫癜（HSP）的关系。方法通过检索 PubMed、Medline、Em‐base、万方数据库、中国期刊全文数据库（CNKI）、中国生物医学数据库（CBM ）及维普（VIP）等数据库,检索时间均为建库开始至2014年,全面收集公开发表的关于Hp感染与儿童 HSP关系的病例对照研究资料,采用 Meta分析方法对所有文献进行综合分析,并计算综合的优势比（odds ratio ,OR）值和95％可信区间（confidence interval ,CI）。结果共检索到相关文献68篇,最终纳入符合条件的文献13篇。结果显示,HSP组与对照组为：OR（95％ CI）为3.17（2.53～3.97）,合并效应量的检验结果Z＝10.06,P＜0.00001;腹型HSP与非腹型HSP组为：OR（95％ CI）为3.43（1.83～6.43）,合并效应量的检验结果Z＝3.85, P＝0.0001。结论 Hp感染与儿童 HSP的发病密切相关,尤其是腹型 HSP。     

Protein C deficiency

Summary. Severe protein C deficiency (i.e. protein C activity <1 IU dL^?1) is a rare autosomal recessive disorder that usually presents in the neonatal period with purpura fulminans (PF) and severe disseminated intravascular coagulation (DIC), often with concomitant venous thromboembolism (VTE). Recurrent thrombotic episodes (PF, DIC, or VTE) are common. Homozygotes and compound heterozygotes often possess a similar phenotype of severe protein C deficiency. Mild (i.e. simple heterozygous) protein C deficiency, by contrast, is often asymptomatic but may involve recurrent VTE episodes, most often triggered by clinical risk factors. The coagulopathy in protein C deficiency is caused by impaired inactivation of factors Va and VIIIa by activated protein C after the propagation phase of coagulation activation. Mutational analysis of symptomatic patients shows a wide range of genetic mutations. Management of acute thrombotic events in severe protein C deficiency typically requires replacement with protein C concentrate while maintaining therapeutic anticoagulation; protein C replacement is also used for prevention of these complications around surgery. Long‐term management in severe protein C deficiency involves anticoagulation with or without a protein C replacement regimen. Although many patients with severe protein C deficiency are born with evidence of in utero thrombosis and experience multiple further events, intensive treatment and monitoring can enable these individuals to thrive. Further research is needed to better delineate optimal preventive and therapeutic strategies.     

Clinico-pathological association of Henoch-Schoenlein purpura nephritis and IgA nephropathy in children

Objective: Henoch-Schonlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are similar syndromes. We aimed to determine whether the crescent formation/immunocomplex in glomeruli is associated with the differences of the biochemical indexes between HSPN and IgAN. Methods: We investigated the medical records of 137 HSPN cases and 41 IgAN cases from January 2009 to April 2014 in Nanjing Children’s Hospital of Nanjing Medical University. The clinical and pathological data were analyzed and compared between HSPN and IgAN. Results: HSPN patients had markedly higher levels of blood white blood cell (WBC), hemoglobulin (Hb) and platelet (PLT), lower levels of hematuria, blood nitrogen (BUN) and C4 compared with IgAN cases. Crescents formation and C3 deposition in the kidney did not affect these differences. Significantly lower levels of hematuria, blood IgG, IgM and C4 in HSPN compared with IgAN cases were observed among patients with IgG deposition. Markedly higher levels of WBC and Hb, lower levels of hematuria, creatinine (Cr), C4 in HSPN compared with IgAN cases were observed among patients with IgM deposition. No marked differences of the biochemical indexes were noted between HSPN and IgAN cases among patients with C1q deposition. Markedly higher levels of WBC and Hb, lower level of blood C4 in HSPN compared with IgAN cases were observed among patients with fibrogen deposition. Conclusions: The different levels of biochemical indexes at presentation between HSPN and IgAN may be associated with the deposition of IgG, IgM, C1q and fibrogen in the kidney.     

儿童过敏性紫癜102例临床分析

目的：总结过敏性紫癜(HSP)患儿的临床特征及肾脏损害相关因素。方法回顾性分析102例过敏性紫癜患儿的临床资料,总结其临床特征,并比较过敏性紫癜性肾炎(HSPN)与非HSPN患儿临床资料及生化指标的差异。结果儿童过敏性紫癜以7~10岁学龄期儿童最常见,占44.1%,秋冬季节发病率最高,占70.6%,最常见的诱因为感染,占52.9%,临床表现以双下肢及臀部皮疹、腹部症状、关节痛常见;本组有31.4%的患儿出现紫癜性肾炎,HSPN组与HSPN组患儿在年龄、反复皮疹、纤维蛋白原(FIB)、总胆固醇(TC)方面比较差异均有统计学意义(P<0.05)。结论过敏性紫癜是儿童常见病,临床表现典型,年龄大、皮疹反复发作及高凝、高胆固醇是患儿发生HSPN的危险因素。