Henoch Schönlein purpura and amebiasis

Abstract The pathogenesis of Henoch Schonlein purpura (HSP) is unknown but is believed to result from an immune complex reaction to various antigenic stimuli, such as infectious agents. However, its association with Entamoeba histolytica has not been reported before. We present an 11 -year-old boy with HSP, confirmed by the demonstration of leukocytoclastic vasculitis from skin and diffuse endocapillary proliferative glomerulonephritis, together with immunoglobulin A and complement component C3 deposition from renal biopsies. Cysts and trophozoites of Entamoeba histolytica were detected from the stool of the patient at the same time and disappeared after the treatment with metranidasole. The temporal association of these two disorders is either coincidental or due to a causal relationship between them.     

Serum hepatocyte growth factor levels in Henoch–Schönlein purpura

Abstract Background : Henoch–Schönlein purpura (HSP) is the most common form of vasculitis in children. The potential role of hepatocyte growth factor (HGF) in acute immune-mediated vasculitis has not been elucidated. Methods : Serum HGF levels were determined in patients with HSP. Results : In patients with acute-phase HSP, mean (~SD) serum HGF levels were 0.32~0.14 ng/mL and were significantly higher than those in the control group (0.11~0.10 ng/mL). This elevation of serum HGF levels recovered to control levels in parallel with improvement of the clinical symptoms. Conclusions : It is suggested that elevation of serum HGF levels in patients with acute-phase HSP may reflect endothelial cell damage or dysfunction in HSP.     

Clinical impact of altered immunoglobulin levels in Henoch–Schönlein purpura

Background:  The aim of the present study was the identification of immunological features, present at the time of diagnosis, that would predict the severity of Henoch–Schönlein purpura and its outcome.
Methods:  A cohort study was carried out in a tertiary pediatric hospital of 69 children with Henoch–Schönlein purpura, in whom serum complement components C3, C4 and IgA, IgM, IgG were repeatedly determined.
Results:  During the acute phase of the disease in 54/69 patients (78.3%) immunological imbalances were observed. In 24/54 cases (44.4%) certain complications involving the kidneys and the gastrointestinal tract were noted as opposed to in 3/15 children (20%) without immunologic abnormalities. In 50/69 children (72.5%), elevated serum IgA was detected and 16 of them (32%) developed renal involvement while only 1/19 children (5.3%) with normal IgA concentration had renal involvement. Considering separately the group of 9/69 children (13%) with increased IgM and those with normal IgM levels (53/69; 76.8%), irrespective of IgA and IgG concentration, we found a comparable percentage of children who had both renal and intestinal involvement without, however, developing severe complications, which were exclusively seen in patients with increased IgA (5/7 children) and reduced IgM levels. Serum C3 fraction was elevated in 26 children (37.7%) and in 73% of cases it was associated with increased serum IgA values.
Conclusion:  Renal involvement was seen in 32% of children with increased IgA values. Most importantly, elevated IgA concentration along with reduced IgM levels was associated with higher prevalence of severe complications.     

Erythrocyte superoxide dismutase activity and plasma malondialdehyde levels in children with Henoch Schönlein purpura

Reactive oxygen molecules (ROM) have been suggested to contribute to many pathological conditions including vasculitides and renal diseases. In the present study we measured the activity of superoxide dismutase (SOD) as an antioxidant enzyme in red blood cells and the level of malondialdehyde (MDA), which is a product and an indicator of lipid peroxidation, in the plasma of 16 children (7M, 9F) with Henoch Schönlein purpura (HSP) at the onset of the disease (SOD 1 and MDA 1) and at the remission period (SOD 2 and MDA 2). The results were compared with the results of 17 healthy children studied as a control group. There was no significant difference for SOD activities between the patients in each period and the control group ( p > 0:05). There was a statistically significant difference between MDA 1 and MDA 2 levels ( p < 0:01), each of which were also significantly different from the MDA levels of control group ( p < 0:001 and p < 0:01, respectively). The effect of ROMs on different clinical conditions of HSP was also examined and lipid peroxidation was found to be increased more in patients with renal involvement. It is concluded that oxidant stress especially lipid peroxidation plays an important role in the pathogenesis of HSP and in development of renal injury.     

Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment

Aim:  To identify risk factors for a child with Henoch-Schönlein purpura (HSP) either to develop nephritis (HSPN) or to contract progressive course and to obtain the currently available evidence on the efficacy of treatment options in both preventing and treating the established renal disease.
Method:  Review of the literature published over the last two decades.
Results:  Persistent or recurrent purpura, severe abdominal symptoms and an older age proved as the most significant risk factors for later HSPN. The risks of long-term renal impairment are the highest in children having at presentation nephritic/nephrotic syndrome and/or more than 50% of glomeruli occupied by large crescents or sclerosing lesions. Randomized controlled trials (RCT) do not support short course prednisone at presentation of HSP in preventing persistent renal disease. Many uncontrolled studies using various treatment regimens have reported outcomes considered better than expected. However, the data from RCTs are sparse and no treatment options for the established renal disease can be currently recommended based on RCTs.
Conclusion:  Severity and/or duration of extrarenal HSP symptoms and an older age are the most significant risk factors for developing HSPN, whereas clinical and histological severity at HSPN onset are in general predictive of a long-term renal impairment. The existing evidence does not support of short course prednisone in preventing persistent renal disease. A well-designed RCTs are needed in children with moderately severe or rapidly progressive (crescentic) HSPN.     

Henoch-Schönlein purpura with hypocomplementemia in children

BACKGROUND: The clinical course and prognosis of Henoch-Schonlein purpura (HSP) associated with hypocomplementemia are not clear. METHODS: The clinical findings of 10 children with HSP and hypocomplementemia were studied. RESULTS: Purpuric rash in all patients, abdominal pain in five, and arthralgia in nine were noted. The findings in HSP were not different from others with HSP. In eight patients, infection preceded hypocomplementemia. Serum levels of CH50, C3 or C4 were depressed variously. Complement levels returned to normal within 5 weeks in all patients. Antistreptolysin-O (ASO) titer was elevated in all patients and nephritis occurred in eight patients. Six patients had generalized edema and hypertension. Macroscopic hematuria occurred in two patients and heavy proteinuria in five patients. One patient was diagnosed as having poststreptococcal acute glomerulonephritis (PSAGN) combined with HSP nephritis according to renal biopsy findings. In three of eight patients with nephritis, abnormal urinary findings continued for more than 1 year. CONCLUSIONS: Hypocomplementemia in children with HSP was transient and was not related to severity of HSP. Incidences of elevated ASO titer and nephritis were high. The nephritis resembled PSAGN during the acute stage and long-term clinical courses varied. These findings suggest PSAGN may be associated with HSP nephritis.     

双重血浆置换联合激素与免疫抑制剂治疗儿童重症紫癜性肾炎的临床效果

目的 探讨双重血浆置换（DFPP）联合甲泼尼龙（MP）、环磷酰胺（CTX）双冲击疗法治疗儿童重症紫癜性肾炎（HSPN）的临床疗效与安全性。方法 将2014年1月至2018年3月收治的60例重症HSPN患儿随机分为观察组和对照组,每组30例。在常规治疗基础上,对照组予MP+CTX双冲击治疗,观察组在对照组基础上联合采用DFPP治疗,共3个疗程。治疗3个疗程后,比较两组24 h尿蛋白定量、尿系列微量蛋白含量、肾功能指标、不良反应及临床疗效。结果 治疗3个疗程后,观察组24 h尿蛋白定量、尿白蛋白、尿免疫球蛋白G、尿β2微球蛋白、血肌酐及血尿素氮的下降幅度均显著高于对照组（P < 0.05）。治疗结束后,观察组完全缓解患儿达缓解的时间明显短于对照组（P < 0.05）。两组均未发生出血性膀胱炎、血小板下降、溶血等严重不良反应,两组总体不良反应发生率差异无统计学意义（P > 0.05）。结论 DFPP联合MP+CTX双冲击治疗儿童重症HSPN较单纯MP+CTX冲击治疗能进一步减轻肾脏损害,提高临床疗效,且未加重不良反应的发生。     

Ranitidine administration in Henoch-Schönlein vasculitis

In this study, we discuss 12 patients with gastrointestinal (GI) bleeding who were diagnosed as having Henoch Schoenlein vasculitis (HSV) in Dr Behçet Uz Children's Hospital, Izmir, between January 1991 and January 1992. Seven male and five female patients were included in the study. Their ages ranged between 6–14 years. The patients were separated into two identical groups and were given ranitidine or a placebo. Both groups were followed up for abdominal pain and GI bleeding. In the group administered ranitidine the duration and severity of abdominal pain and gastrointestinal bleeding decreased significantly as compared to the group taking placebo (P <0.05). No side effects of ranitidine were observed. As a result, it was concluded that ranitidine could be used to treat HSV with GI symptoms.     

Clinicopathologic correlations of Henoch–Schönlein nephritis in Turkish children

Abstract Background: In this study, 66 patients with Henoch–Schönlein nephritis (HSN) were investigated clinicopathologically. Methods: The patients were classified according to their initial presentation, histologic findings, recurrences of purpura, type of treatment and clinical outcome. Logistic regression analysis was performed. Results: Sixty-eight percent of patients were hospitalized with mild renal disease. Most patients were evaluated as class I and II according to light microscopy. In addition to IgA deposition alone, 33% of patients showed IgA+C3 and 27% had IgA+IgG+C₃ depositions. After the follow-up period of 3.3 years, 15 patients had minor urinary findings, 4 had active renal disease and 1 had renal insufficiency. Recurrences occurred in 37.9% of patients and 37.1% of patients with recurrences had persistent pathologic findings. Symptomatic treatment was given to 51.5% of patients, while 27.2% were given corticotherapy. Conclusions: Clinical presentation was found to be correlated with outcome. Recurrence of the disease and the type of the treatment also affected the outcome. It was also thought that mesangial IgG and C₃ depositions may have a role in the pathogenesis of renal damage in HSN.     

内皮细胞微粒在过敏性紫癜患儿中的表达及意义

目的 探讨内皮细胞微粒（EMPs）在过敏性紫癜患儿中的表达及意义。方法 100例初治过敏性紫癜患儿分为紫癜性肾炎组（HSPN组,40例）和非肾炎组（60例）,并以30例健康体检儿童为对照组。采用流式细胞术或ELISA方法检测各组血清EMPs、Th17及IL-17的表达或含量。结果 Th17及IL-17在HSP的肾炎组和非肾炎组均高于对照组,以HSPN组最高,差异有统计学意义（P < 0.05）。HSP肾炎组和非肾炎组的EMPs水平较对照组升高,以HSPN组最高,差异有统计学意义（P < 0.05）。紫癜性肾炎组的Th17、IL-17水平与EMPs水平呈正相关（r=0.830、0.644,P < 0.05）。结论 EMPs在过敏性紫癜的发病机制中起一定作用,EMPs升高可能是过敏性紫癜患儿肾脏受累的原因之一。     

Carbamazepine-induced thrombocytopenia and leucopenia complicated by Henoch-Schönlein purpura symptoms

A rare case of carbamazepine-induced leucopenia and thrombocytopenia complicated by Henoch-Schönlein purpura (HSP) symptoms is presented. Laboratory findings suggested that leucopenia and thrombocytopenia could be due to bone marrow suppression and HSP symptoms to an allergic reaction to carbamazepine. To the best of our knowledge this is the first report that carbamazepine may cause haematological disorders associated with symptoms of HSP by different mechanisms at the same time in the same patient.     

从IgA血管炎角度分析儿童IgA肾病和紫癜性肾炎的临床病理

目的从IgA血管炎角度分析器官特异性IgA血管炎(IgA肾病)与系统性IgA血管炎(紫癜性肾炎)临床和病理的差异。方法回顾性分析1993年6月至2014年11月住院的IgA肾病和紫癜性肾炎患儿的临床、病理和转归。结果 IgA肾病患儿405例,男256例、女149例,男女比例为1.7:1,平均发病年龄(10.2±2.8)岁;临床类型以肾病综合征最多,占31.6%,其次为血尿和蛋白尿型占27.9%。紫癜性肾炎患儿548例,男329例、女219例,男女比例为1.5:1,平均发病年龄(10.2±3.1)岁,临床类型以血尿和蛋白尿型最多,占60.9%,肾病综合征占21.4%。未发现IgA肾病因病情加重或反复进展至系统性血管炎的病例。结论 IgA肾病和过敏性紫癜的起病诱因、发病年龄、临床表现多有一致或重叠,二者在发病机制上也有一定共性,但IgA肾病更可能还有肾脏局部免疫紊乱等参与发病。     

Henoch–Schönlein purpura in children

Henoch–Schönlein purpura is the most common systemic vasculitis of childhood. In the majority of children, the outcome of Henoch–Schönlein purpura is excellent with spontaneous resolution of symptoms and signs. However, a small subset of patients will develop long‐term sequelae in the form of chronic kidney disease. While the clinical presentation and diagnosis of Henoch–Schönlein purpura is straightforward, treatment of Henoch–Schönlein purpura nephritis and long‐term renal outcomes of more severely affected children are less certain. This review article gives a general overview of Henoch–Schönlein purpura with emphasis on recently published information, including the new classification of childhood vasculitis, insights into pathogenesis of Henoch–Schönlein purpura and a summary of various treatments of established Henoch–Schönlein purpura nephritis.