Henoch-Schönlein Purpura Nephritis Research Articles

Clinical Observation on 50 Cases of Hematuria Caused by Henoch-Schonlein Purpura Nephritis Treated by Nuohua Zizhu Tablets

Clinical Observation on 50 Cases of Hematuria Caused by Henoch-Schonlein Purpura Nephritis Treated by Nuohua Zizhu Tablets

Immunoglobulin A Vasculitis After a Not So Innocuous Wasp Bite

Background: Immunoglobulin A vasculitis (IgAV), previously known as Henoch–Schonlein purpura (HSP), is the most common vasculitis in children. Previous studies have identified various triggers of IgAV, with infections being the most common. We present herein a 9-year-old girl who developed IgAV with nephritis following a wasp sting. Clinical Description: A 9-year-old girl presented to us with a history of wasp sting 7 days ago, followed by the appearance of reddish, raised rashes over the back of her lower limbs, which later spread all over the body. She also developed edema over the face, abdomen, and lower limbs along with pain abdomen. On examination, she was afebrile, was normotensive, and had periorbital edema and bilateral pedal edema with multiple discrete palpable, nonblanching purpura predominantly over the extensor surfaces of the lower and upper extremities and the trunk. Abdominal examination revealed no tenderness. Complete blood counts, blood urea, serum creatinine, and liver function tests were normal. Urinalysis showed microscopic hematuria and nephrotic range proteinuria. Skin biopsy of the lesions showed evidence of IgA vasculitis. Renal biopsy was suggestive of HSP nephritis class 3. Management and Outcome: She was managed with oral corticosteroids, mycophenolate mofetil, and enalapril and had remission of proteinuria. The renal function tests and blood pressure continue to be normal. Conclusion: Few case reports exist of IgAV precipitated by insect bites; however, we could not find any previous reports of IgAV with nephritis following a wasp sting in children. This report adds to existing knowledge regarding precipitating factors for IgAV in children.

A predictive system for Henoch-Schönlein purpura nephritis established by multivariate analysis plus nomogram model in Chinese hospitalized children: A retrospective cohort study

To the Editor: Renal involvement is recognized as a predominant contributor to the long-term prognosis of Henoch-Schönlein purpura (HSP) and affects 38.5% of patients after a mean follow up of 73.9 months.1 Several possible risk factors for predicting HSP nephritis (HSPN) have been suggested. In this study, we established a predictive system for HSPN in hospitalized children in China using multivariate analysis plus a nomogram model.

Use of renin-angiotensin system inhibitors as initial therapy in children with Henoch-Schönlein purpura nephritis of moderate severity.

Cases of Henoch-Schönlein purpura nephritis (HSPN) with moderate severity were demonstrated to achieve good prognosis after treatment with renin-angiotensin system (RAS) inhibitors. However, some patients required additional treatment for recurrence after remission. This study aimed to clarify the effect of RAS inhibitors in HSPN cases with moderate severity, including the proportion of cases with recurrence and their response to additional treatment. Among 126 patients diagnosed with HSPN between 1996 and 2019, 71 patients with clinicopathologically diagnosed HSPN of moderate severity, defined as ISKDC grade II-IIIa and serum albumin ≥ 2.5 g/dL, were investigated. Proteinuria became negative after RAS inhibitor treatment alone in all 71 cases. However, 16 (22.5%) had recurrence. Eleven recurrent cases achieved negative proteinuria again following additional treatment. At the last follow-up (median 46.5 months; IQR, 23.2-98.2), 5 patients had persistent mild proteinuria; no patients had estimated glomerular filtration rate < 90 mL/min/1.73 m2. The pathological findings in all recurrent cases were ISKDC grade IIIa. The 16 recurrent cases had significantly higher proportions of glomeruli with global/segmental sclerosis (25.0 vs. 0%, P < 0.001) and tubular atrophy/interstitial fibrosis (37.5 vs. 12.7%, P =0.0 24) than 55 cases without recurrence. Japanese childhood HSPN cases with moderate severity had good outcomes without need for corticosteroids or immunosuppressants, when prescribed RAS inhibitor treatment. Even in recurrent cases, abnormal proteinuria was transient, and prognosis was excellent. A higher resolution version of the Graphical abstract is available as Supplementary information.

Analysis of children with Henoch–Schonlein purpura secondary to infection

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis, infection is the most essential inducement. We hypothesized that infection could impact the blood routine characteristics and/or outcome of vasculitis. Thus, we aim to find the most common infectious agent in HSP patients and identify convenient indicators to predict renal involvement in HSP patients with infection. We conducted a retrospective study of 208 HSP children and 98 healthy children. Clinical parameters were compared in those cases. A total of 68.75% of patients were infected with various pathogens. The mean platelet volume (MPV) (P < 0.02) was lower in HSP patients with infection than patients without infection. Mycoplasma pneumoniae (MP) infection accounted for the largest proportion (45.77%). MPV in HSP nephritis (HSPN) group was lower than in HSP patients (excluded renal involvement) in patients with MP infection. Logistic regression analysis found that age and MPV were risk factors for the occurrence of MP-infected HSPN. The receiver operating characteristic curve (ROC) analysis showed that the combination of MPV with the onset age at the optimal cut-off point had 81% sensitivity in predicting whether HSP patients with MP infection would develop into HSPN. Our research revealed that MP was the most commonly infected pathogen of children's HSP. MPV was an essential predictor of nephritis in HSP patients with MP infection. This discovery can prompt clinical treatments as well as reduce costs. Key Points • Mycoplasma pneumoniae (MP) accounts for the largest proportion in HSP children with infection. • MPV can be used as a predictor for the development of MP-triggered HSP to HSPN.

Renal biopsy in children with IgA vasculitis.

IntroductionHenoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients.MethodsBiopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated.ResultsOf 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040).ConclusionCyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.

Correlations of Leukotriene B4 and 25-Hydroxyvitamin D3 Levels with Disease Severity in Children with Henoch-Schonlein Purpura.

The aim was to explore the correlations of leukotriene B4 (LTB4) and 25-hydroxyvitamin D3 [25(OH)D3] levels with disease severity in children with Henoch-Schonlein purpura (HSP). A total of 260 HSP children admitted from January 2017 to December 2018 were selected, and 60 healthy children physically examined in the same period were enrolled as controls. The results of general laboratory tests and expression levels of leukotriene B4 (LTB4) and 25-hydroxyvitamin D3 [25(OH)D3] were compared. The correlations of LTB4 and 25(OH)D3 with immunoglobulin A (IgA) were statistically analyzed, and the expression levels of LTB4 and 25(OH)D3 were compared between Henoch-Schonlein purpura nephritis (HSPN) group and non-Henoch-Schonlein purpura nephritis (NHSPN) group. The optimal cutoff values of LTB4 and 25(OH)D3 to predict HSP were analyzed by receiver operating characteristic (ROC) curves, based on which they were divided into low-, intermediate-, and high-risk groups. The length of hospital stay and recurrence rate within 6 months were compared. The levels of white blood cell count, platelets, C-reactive protein, IgA, IgM, IgE, complement C3, and LTB4 were significantly higher and 25(OH)D3 was lower in HSP group than those in control group (p < 0.05). IgA, LTB4, and 25(OH)D3 levels were independent risk factors for HSP (p < 0.05). LTB4 was positively correlated with IgA (p < 0.05), and 25(OH)D3 was negatively correlated with IgA (p < 0.05). LTB4 level was significantly higher and 25(OH)D3 level was lower in HSPN group than those in NHSPN group (p < 0.05). The optimal cutoff values of LTB4 and 25(OH)D3 were 27.82 pg/mL and 22.10 ng/mL, respectively, the length of hospital stay gradually increased in low-, intermediate-, and high-risk groups, and the recurrence rates within 6 months were 14.2%, 31.5%, and 39.6%, respectively (p < 0.05). LTB4 level increases and 25(OH)D3 level decreases in children with HSP, and the expression levels are significantly correlated with disease severity, suggesting predictive values for prognosis.

Clinicopathological Spectrum of Henoch-Schönlein Purpura Vasculitis: An Experience from a Tertiary Care Center.

Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with multiorgan involvement. Renal involvement is the key factor predicting morbidity. We have aimed to analyze the clinicopathological spectrum of HSP vasculitis and HSP nephritis to assess the risk factors associated with kidney involvement. This retrospective study was performed in the department of pathology with collaboration of department of dermatology and department of nephrology of a tertiary care center. All clinical details along with biopsy findings were retrieved. Starting materials of the study were cases of leukocytoclastic vasculitis with only perivascular IgA deposit of more than ++ in the absence of other immunoglobulin and trace complements. To investigate the possible factors that are influential on the development of biopsy-proven HSP nephritis, we divided the whole study population in two groups -group 1: with and group 2: without biopsy-proven nephritis. One-way analysis of variance was carried out during comparative analysis between two groups using IBM SPSS statistics software, version 19 and MedCalc software, version 12.3.0.0. HSP vasculitis comprised 11.6% (n = 19) of total cutaneous vasculitis in 2 years (164 cases) with a mean age of 13.52 ± 8.10 (range: 4-33 years). Three cases developed de novo kidney disease (15.79%). A correlation analysis revealed that predictors were seasonal variation (P = 0.018), severe gastrointestinal involvement (P = 0.03), and subcutaneous edema (P = 0.005). Various clinical and laboratory parameters were associated with renal consequences. Occult nephritis was the most common presentation with crescent as a constant histopathological feature.

Dermatologic Manifestations of Systemic Diseases in Childhood

Dermatologic Manifestations of Systemic Diseases in Childhood

Chinese patent herbal medicine Huaiqihuang for Henoch-Schonlein purpura nephritis in children: a systematic review of randomized controlled trials

BackgroundHenoch-Schönlein purpura nephritis (HSPN) is listed as the most common secondary glomerular diseases among children. Approximately 15 to 20% of children eventually could develop into chronic renal failure. Chinese patent herbal medicine Huaiqihuang (HQH) has been widely used in children with HSPN. This study aimed to evaluate the effectiveness and safety of HQH for HSPN in children, so as to provide evidence for clinical use.MethodsRandomized controlled trials (RCTs) on HQH for HSPN in children were searched in eight Chinese and English databases from their inception to December 2020. We included children with HSPN received HQH combined with conventional medicine. Cochrane “Risk of bias” tool was used to assess methodological quality, and “Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach” to summarize the certainty of evidence for main findings. Effect estimates were presented as risk ratio (RR), mean difference (MD) or standardized mean difference (SMD) with 95% confidence interval (CI) in meta-analyses using RevMan 5.3. Data not suitable for statistical pooling were synthesized qualitatively.ResultsIn total seven RCTs were identified. Compared with conventional medicine alone, HQH plus conventional medicine showed the better effect in improving clinical cure rate (RR 1.58; 95%CI 1.17 to 2.14; n = 6) and total effective rate (RR 1.34; 1.16 to 1.54; n = 6); reducing urine sediment erythrocyte count (MD -9.23; − 10.76 to − 7.69; n = 3) and urine β2 micro-globulin level (MD -0.09; − 0.12 to − 0.06; n = 2). No serious adverse event was recorded in all included trials.ConclusionsLimited evidence showed HQH combined with conventional medicine had a beneficial effect for children with HSPN, and the side effects were mild. HQH may be a promising complementary therapy. However, long term follow-up, high quality and multicenter RCTs are required to confirm the findings.

A Case of Henoch-Schonlein Purpura Nephritis in a Limited Resource Setting

Henoch-Schonlein Purpura (HSP) is the commonest vasculitis in children, and the outcome depends on renal involvement or occurrence of HSP nephritis (HSPN). Hence, a meticulous diagnosis, treatment and regular follow-up plan is needed for a better prognosis. A case of 12 - year - old girl showing HSPN symptoms only eight weeks after the first onset of HSP is reported. Symptoms of HSPN might appear later, hence emphasising the importance of close monitoring in a patient of HSP. There is a lack of clear consensus about the management of HSPN until now, and it becomes a challenge especially in a limited resource setting like ours. In our case, the patient was treated with corticosteroids.

The Efficacy of Tripterygium Glycosides Combined with LMWH in Treatment of HSPN in Children.

Objective This study aimed to explore the clinical efficacy and relevant mechanism of Tripterygium glycosides combined with low molecular weight heparin calcium (LMWH) in the treatment of Henoch–Schönlein purpura nephritis (HSPN) in children. Methods 64 cases of children patients with HSPN treated at Qilu Hospital (Qingdao) from January 2015 to May 2020 were selected and randomly divided into the control group and the observation group and 32 cases in each group. Conventional medical treatment was applied in the two groups, besides which the control group was given LMWH while the observation group was given Tripterygium glycosides based on the control group. The clinical efficacy and the indexes of clinical symptoms of the two groups were compared. Immune globulin level, fibrinogen content (FIB), prothrombin time (PT), platelet level (PLT), and activated partial thromboplastin time (APTT) level of the two groups were compared before and after the treatment. Results The total effective rate in the observation group was significantly higher than that of the control group, and the recurrence rate in the observation group was lower than that in the control group. After treatment, urine red blood cell count and 24 h urine protein were obviously better than those of the control group. There was no statistically significant difference in PT between the two groups of children before and after treatment. The levels of PLT and FIB in the two groups of patients after treatment were significantly lower than before treatment, and the PLT levels in the observation group were lower than those in the control group. Conclusion The combination of Tripterygium glycosides and LMWH had good clinical effects in the treatment of children with HSPN, and it could improve the clinical symptoms, the mechanism of which might be related to the increase of PT, a decrease of PLT, and the improvement of coagulation function.

Diagnostic efficacy and influence factors of urinary protein/creatinine ratio replacing 24-h urine protein as an evaluator of proteinuria in children.

The aim was to investigate the diagnostic efficacy of urinary protein/creatinine ratio (UPCR) and factors influencing its substitutability of 24-h urine protein (24hUP) in children with proteinuria. A total of 356 children were recruited, including 149 with non-nephrotic-range proteinuria and 207 with nephrotic-range proteinuria which were further divided into Henoch-Schönlein purpura nephritis (HSPN), lupus nephritis (LN), and primary nephrotic syndrome (PNS). The urine protein and creatinine were measured by routine methods. Bland-Altman analysis was used to test the agreement. Spearman correlation was performed to evaluate the relevance. The receiver operating characteristic curve was used to analyze the diagnostic efficacy of UPCR. Bland-Altman analysis showed there was an excellent agreement between UPCR and 24hUP in each group. Correlations between UPCR and 24hUP were strong in 356 children (r = 0.869) and in the non-nephrotic-range proteinuria group (r = 0.806), but moderate in nephrotic-range proteinuria group (r = 0.586). With the increase of nephrotic-range proteinuria, the correlations between UPCR and 24hUP were decreased further, however, after UPCR was adjusted by 24-h urine creatinine (24hUCr), the correlation coefficient was improved (r = 0.682). In three subgroups with nephrotic-range proteinuria, high correlation coefficient (r = 0.731) was observed in HSPN, but not in LN (r = 0.552) and PNS (r = 0.563). The sensitivity and specificity of UPCR for diagnosing nephrotic-range proteinuria were 89.9 % and 92.2%. UPCR is competent in evaluating proteinuria. The degree of proteinuria, 24hUCr and the underlying pathological types of renal disease may be the important influencing factors in the correlation between UPCR and 24hUP in children with nephrotic-range proteinuria.

Renal Outcomes of Childhood IgA Nephropathy and Henoch Schönlein Purpura Nephritis

Background: Henoch-Schönlein purpura nephritis (HSPN) is considered the systemic form of IgA nephropathy (IgAN). However, differing clinicopathological features and renal outcomes of children with IgAN and HSPN have been reported in some studies.&#x0D; Methods: This study retrospectively reviewed children with IgAN and HSPN younger than 18 years, between January 2004 and December 2015. The clinicopathological characteristics at diagnosis and the renal outcomes after at least 1 year of follow-up were compared between the two groups.&#x0D; Results: A total of 54 children, comprising 21 with IgAN and 33 with HSPN, were recruited. The children with HSPN were younger than the children with IgAN. Gross hematuria and nephritic syndrome at the initial presentation were more common in children with IgAN. Regarding the pathological findings, IgAN had greater chronicity than HSPN. After a median follow-up period from first presentation to renal outcomes measurement of 4.0 years (1.3-12.2) in children with IgAN and 4.2 years (1.1-11.4) in children with HSPN, the renal outcomes were better in the latter group. The incidence of chronic kidney disease (CKD) was 28.6% in children with IgAN and 6.1% in children with HSPN (p = 0.02). Complete recovery was observed more frequently in children with HSPN than in children with IgAN (57.1% in IgAN vs. 87.9% in HSPN, p = 0.01).&#x0D; Conclusions: Childhood IgAN has greater chronicity and worse renal outcomes than childhood HSPN, with a lower rate of complete recovery and a higher frequency of CKD. We recommend long-term follow-up for CKD in children with IgAN.

A POSSIBLE RELATIONSHIP BETWEEN SERUM HOMOCYSTEINE LEVEL AND IgA NEPHROPATHY IN CHILDREN

Objective: The evidences from experimental and epidemiological studies suggests that elevated serum homocysteine levels may lead to renal injury and may be a significant risk factor for the development of chronic kidney disease. The aim of this study was to investigate a possible relationship between serum homocysteine level and crescent formation in children with IgA nephropathy and Henoch-Schonlein purpura nephritis. Material and Methods: A total of 31 patients diagnosed as biopsy proven IgA nephropathy and Henoch-Schonlein purpura nephritis and idiopathic crescentic glomerulonephritis in three Pediatric Nephrology centers within the last five years and 25 healthy controls were enrolled in the study. Results: Homocysteine levels of patients were higher than the upper limit of normal value and also higher than the controls (p=0.0001). There was no significant difference between the patients with or without crescent formation regarding homocysteine levels (p&gt;0.05). Presence or severity of proteinuria was not related to homocysteine levels (p&gt;0.05). Conclusion: Serum homocysteine levels are elevated in patients with IgA nephropathy and Henoch-Schonlein purpura nephritis.&nbsp;Our results suggest that elevated serum homocysteine levels may be related to segmental glomerulosclerosis in these patient groups.

Variations of tongue coating microbiota in children with Henoch-Schönlein purpura nephritis

BackgroundVariations in the oral microbiota have been significantly correlated with the progress of autoimmune diseases, such as immunoglobulin A nephropathy and Henoch-Schönlein purpura (HSP). However, there is no report outlining the character of tongue coating microbiota variations in children with Henoch-Schönlein purpura nephritis (HSPN). MethodA total of 20 children with HSPN and 14 healthy controls were recruited for this research. Tongue coating samples of two groups were collected for 16S rRNA gene sequencing. The diversity, principal component analysis (PCA), nonmetric multidimensional scaling (NMDS), partial least squares discriminant analysis (PLS-DA), and linear discriminant analysis (LDA) effect size (LEfSe) were performed. Microbial function was assessed using the PICRUST. ResultsThe ACE and Chao indices were greatly lower in the HSPN group than in the HG (P = 0.001). The Shannon and Simpson indices were dramatically reduced in children with HSPN compared with those in the healthy controls (P = 0.005). Bacteroidales, Selenomonadales, Lactobacillales, Fusobacteriales, Neisseriales, and Actinomycetales composed more than 80% of all sequences, while Bacteroidales was the most generous order in both groups. PCA, NMDS and PLS-DA showed a marked difference between the control and HSPN groups. LEfSe analysis showed alteration of tongue coating microbiota in the HSPN group. There were 30 metabolic functions significantly differed between the two groups. ConclusionsChildren with HSPN have substantially various tongue coating microbiota compared to healthy controls. Even though this research does not indicate causality, it is beneficial to enhance the possibility for coming microbial-based treatments to enhance the clinical effects of HSPN in children.

Leflunomide therapy for IgA vasculitis with nephritis in children

BackgroundHenoch-Schönlein purpura (HSP), also called IgA vasculitis, is a systemic vasculitis characterized by deposits of immunoglobulin A in blood vessels. Renal impairment of these patients is the main determinant of prognosis. The optimal treatment of HSP nephritis (HSPN) in children remains controversial, but many clinicians administer an immunosuppressive agent with a corticosteroid. A previous study reported that leflunomide (LEF) with a corticosteroid was effective for adult patients with HSPN and nephrotic proteinuria. However, data on this treatment in pediatric patients is limited.MethodsWe described our experience at a single center on the use of LEF in 5 pediatric patients who had IgA vasculitis with proteinuria that was nearly 50 mg/kg (nephrotic range) and remained high despite administration of intravenous steroid, and biopsy-proven nephritis. All patients had class II to IIIb lesions based on the International Study of Kidney Disease in Children (ISKDC).ResultsWe successfully treated all 5 children who had IgA vasculitis with nephritis using LEF with a corticosteroid. Four patients achieved a complete remission of proteinuria, and 1 patient had significantly reduced proteinuria. The children received LEF for 6 months to 12 months, and none of them had severe adverse events.ConclusionsTo our knowledge, this is the first case series to report successful treatment of pediatric HSPN with LEF in combination with a corticosteroid.

To biopsy or not to biopsy: Henoch-Schönlein nephritis in children, a 5-year follow-up study.

The prognosis of Henoch-Schönlein purpura (HSP), IgA vasculitis, depends on kidney involvement. There is no consensus on the initiation of treatment for HSP nephritis (HSPN). Some centres start treatment before performing a kidney biopsy (KB) while in others, treatment is dictated by the importance of the clinical, biological and histological signs. The aim of this study was to evaluate which of these two approaches is associated with a better kidney outcome at 5-year follow-up. This multicentre, retrospective, nonrandomised study included children treated for HSPN between 2006 and 2010 in a French paediatric nephrology unit. One group had an early KB at diagnosis (before starting treatment or in the 15 following days). In the second group, initial treatment was decided without performing a KB. Among the 107 children included, 63.5% had an early KB at diagnosis. Follow-up at 5 years was completed in 44 children (28 KB at diagnosis, 16 no KB at diagnosis). Median urine protein/creatinine at 5 years was 2.5 mg/mmol in the early biopsy diagnosis group and 12.5 mg/mmol in the non-biopsy group. An antiproteinuric treatment was given, at 5 years, to 35.7% of the early biopsy at diagnosis children and in 62.5% of the non-biopsied children. Children with early KB at diagnosis seem to have a better renal outcome at 5 years compared to those without an early biopsy at diagnosis or biopsied later. However, this is a small patient cohort and data are missing. Further work is needed to build consensual guidelines on the management of HSPN in children.

Etiology and Morphological Pattern in Rapidly Progressive Glomerulonephritis

Introduction: Rapidly progressive glomerulonephritis (RPGN) is a type of GN disease of the kidney. It is clinically characterized by a rapid decrease in the glomerular filtration rate (GFR) of at least 50% over a short period, from a few days to 3 months. The main pathologic finding is extensive glomerular crescent formation. The present study was conducted to recognize the morphological pattern in RPGN, underlying causes, and the outcome of RPGN in children. Aim of the study: To assess the histopathology of Rapidly Progressive Glomerulonephritis (RPGN), as RPGN can cause rapid irreversible damage to renal glomeruli in the form of crescents. Methods: This study was done on 34 RPGN children at the department of pediatric Nephrology in Dhaka Shishu (Children) Hospital over the period of June 2017 to December 2019. Result: The etiology of RPGN in this study showed, HSP nephritis was the commonest (23.5%) followed by IgA nephropathy (21.9%) and postinfectious glomerulonephritis (17.64%). Hematuria, proteinuria, and edema were present in almost all cases. The proportion of glomeruli displaying cellular crescent was 21%, fibrous crescent 7.1%, and fibrocellular crescent was 35% respectively. In terms of outcome, 61.7% of patients exhibited total recovery of renal function, 14.7% of patients were lost during follow-up, 5.8% were dialysis dependant, 5.8% patients developed chronic kidney disease and 11.7% died during hospitalization. Conclusion: RPGN is one of the important causes of unexplained acute kidney injury (AKI). Rapid and irreversible loss of renal function and remarkable mortality consider the need for awareness and development of a specific registry for Pediatric RPGN in hospitals.

HSP Nephritis in Children, Clinical Behavior, Renal Involvement and Remedy

Introduction: Childhood HSP is a systemic vasculitis of diverse clinical features with a favorable prognosis but renal involvement in HSP can cause chronic kidney disease (CKD) in children which is preventable in most cases if diagnosed and referred early. Aim of the study: To determine the occurrence ratio of HSP in children, its clinical behavior, renal involvement, and possible remedies. Methods: Medical records of 31 HSP patients were evaluated in the Nephrology and Rheumatology department of Dhaka Shishu (Children) Hospital from July 2017 to December 2019. Epidemiological data, clinical and laboratory findings were reviewed. The treatment for each patient was recorded including supportive care antihypertensive, ACE inhibitor, and immunosuppressive drug. Result: Most children affected during winter and spring (29%). Mean duration of disease 21.19 ±11.54 days. The mean age was 8.63±2.53 yrs. The mean age of renal involvement was 9.5 years. Male-female ratio 5.7:2. Almost all children (96.8%) presented with a rash. Abdominal pain was noted in 74.19% of patients. Joint pain was reported in 51.6% of patients. Renal involvement was presented in 58% of patients commonly in the form of proteinuria (58.6%) and hematuria (48.4%). 9 (29%) patients developed acute kidney injury (AKI). Occasional complications include convulsion, intestinal obstruction, septicemia, and pneumonia. Lab analysis showed thrombocytosis in 54% of patients, leukocytosis in 61% of patients, hypo-albuminemia in 58% of patients. C3 low in 29% patients. A kidney biopsy was done in 10 patients. IgA deposition and mesangial cell proliferation were the most common pathology. Regarding treatment, 12 patients were treated with methylprednisolone and MMF. 8 patients go into remission after taking only oral prednisolone. 6 patients took ACE inhibitor and 2 patients need dialysis. The majority of children (90.3%) achieved remission, though 2 patients died during admission. Conclusion: Present literature .....