New insights into epidemiological data and impact of the COVID-19 pandemic on IgA vasculitis in children and adults: a French nationwide cohort.

IgA vasculitis (IgAV) is a small size vasculitis for which epidemiologic data are strikingly lacking, especially about the adult form. Additionally, the COVID-19 pandemic seems to have profoundly modified the incidence of this disease. Here, we aimed to establish some relevant epidemiological data in both pediatric and adult IgAV. We performed an observational study using a national database called "BNDMR" on IgAV, which gathers patients managed in the French network of experts on rare diseases. We primarily performed descriptive statistics over the 2010-2022 period. Then, we compared the North-South geographical areas, the seasonality, and the impact of COVID-19 with that of other patients reported in the same centers. We collected data from 1988 IgAV patients. The sex ratio was 1.57 for adults and 1.05 for children. The annual incidence in 2021 was 0.06 for 100,000 adults and 0.50 for 100,000 children. Compared with other diseases reported into the BNDMR, IgAV was more common in the South than in the North of France (OR 4.88 [4.17-5.74] in adults and OR 1.51 [1.35-1.68] in children). IgAV was also observed more frequently in winter and autumn. Strikingly, we observed a decrease in incidence during the COVID-19 pandemic period in children (OR 0.62 [0.47-0.81]). Our study provides both new insights and confirmations of IgAV epidemiological data: winter and autumn seasonality, more pronounced male predominance in adults, decreasing incidence of pediatric IgAV during the COVID-19 pandemic and increasing incidence in the South of France.

Towards better indications for kidney biopsy in adult IgA vasculitis: a clinical-laboratory and pathology correlation study.

BACKGROUND: Indications for kidney biopsy in adult IgA vasculitis (IgAV) remain debated and there are very few studies on this subject. The aim of this study was to establish a correlation between renal histological and clinical-laboratory data. METHODS: A retrospective multicenter study was conducted using three databases from French hospitals, gathered between 1977 and 2020. The study included 294 adult patients with IgAV who had undergone kidney biopsy assessed according to the prognostic "Pillebout classification". Different statistical models were used to test the correlations between histological and clinical-laboratory data: Cochran Armitage, ANOVA, Kruskal-Wallis and logistic regression. RESULTS: The patients were primarily men (64%), with a mean age of 52 years. The main organs and tissues involved were: dermatological 100%, digestive 48% and rheumatological 61%. All had features of kidney involvement. The median serum creatinine was 96 µmol/L serum albumin 35 g/L, and C-reactive protein 28 mg/L. Of the patients, 86% (n = 254) had hematuria and median proteinuria was 1.8 g/day. The only statistically significant correlation between the pathological stages and the clinical-laboratory data was the presence of hematuria (p = 0.03, 66% class I to 92% class IV). In multivariate analysis, only albuminemia was associated with extracapillary proliferation (p = 0.02; OR 0.94) and only age was associated with stages 3-4 (p = 0.03; OR 1.02). CONCLUSION: Our study suggests that there is no strict baseline correlation between renal pathology and clinical-laboratory data. Given the current knowledge, it seems relevant to recommend a kidney biopsy in the presence of significant and persistent proteinuria or unexplained kidney function decline.

Efficacy of montelukast sodium combined with methylprednisolone in the treatment of allergic purpura in children and its effects on inflammatory factors and immune function / 中国基层医药

Objective:To investigate the efficacy of montelukast sodium combined with methylprednisolone in the treatment of pediatric allergic purpura and its effects on inflammatory factors and immune function.Methods:A total of 94 children with allergic purpura who received treatment in Taizhou Women and Children's Hospital and Taizhou Hospital Medical Center (Group) Enze Hospital from March 2019 to March 2021 were included in this study. They were randomly divided into observation and control groups ( n = 47/group). The control group was treated with methylprednisolone. The observation group was treated with montelukast sodium combined with methylprednisolone. The course of treatment was 2 weeks in both groups. Efficacy and changes in inflammatory factors and immune function post-treatment relative to those pre-treatment were compared between the two groups. Results:Total response rate in the observation group [93.62% (44/47)] was significantly higher than that in the control group [74.47% (35/47), Z = 2.15, P < 0.05)]. After treatment, interleukin (IL-4), IL-6, and IL-18 levels in each group were significantly decreased compared with those before treatment ( tobservation group = 21.19, 22.26, 27.20, tcontrol group = 11.10, 13.21, 14.86, all P < 0.05). After treatment, IL-4, IL-6, and IL-8 levels in the observation group were (48.98 ± 5.21) ng/L, (34.10 ± 6.42) ng/L, and (53.29 ± 5.67) ng/L, respectively, which were significantly lower than (65.38 ± 7.08) ng/L, (47.83 ± 4.71) ng/L, (67.83 ± 7.10) ng/L in the control group ( t = 12.79, 11.82, 10.97, all P < 0.05). After treatment, CD3 +, CD4 +, and CD4 +/CD8 + in each group were significantly increased compared with those before treatment ( tobservation group = 14.27, 14.41, 17.61, tcontrol group = 6.90, 5.12, 7.40, all P < 0.05). After treatment, CD3 +, CD4 +, and CD4 +/CD8 + in the observation group were (68.94 ± 2.89)%, (39.94 ± 2.15)%, and (1.79 ± 0.13), respectively, which were significantly higher than (63.86 ± 3.28)%, (35.65 ± 2.31)%, and (1.53 ± 0.16) in the control group ( t = 7.96, 9.32, 8.64, all P < 0.05). After treatment, serum IgG and IgM levels in each group were significantly decreased compared with those before treatment ( tobservation group = 21.00, 7.99, tcontrol group = 8.38, 5.76, both P < 0.05). After treatment, serum IgG and IgM levels in the observation group were (1.43 ± 0.19) g/L and (9.74 ± 0.78) g/L, respectively, which were significantly lower than (1.95 ± 0.37) g/L and (10.89 ± 0.85) g/L in the control group ( t = 8.57, 6.83, both P < 0.05). Conclusion:Montelukast sodium combined with methylprednisolone is highly effective on allergic purpura in children. The combined therapy can reduce inflammatory responses and improve immune function in children.

Serum Syndecan-1 levels in patients with immunoglobulin A vasculitis in children

Abstract Objective The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. Methods Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. Results Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. Conclusions SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.

Factors associated with surgical treatment in pediatric intussusception.

BACKGROUND: The study aimed to identify factors related to the need for surgical treatment of intussusception in pediatric patients. METHODS: The medical charts of 106 patients diagnosed with intussusception and treated at the Imam Khomeini Medical Center in Ahvaz city between September 2019 and October 2020 were retrospectively reviewed. Patients were compared in terms of risk factor groups treated with surgery (12 pediatric patients) and nonsurgical methods (92 pediatric patients). Size of intussusception, free fluid in the abdomen, and currant jelly stool were compared between the groups. RESULTS: The mean age in the group treated with surgery was significantly higher (p = 0.01). The duration of symptoms in patients treated with surgery was significantly higher (p = 0.033). The size of intussusception in the surgical treatment group was significantly larger than in the nonsurgical recovery group (p = 0.042). The rates of presence of free fluid in the abdomen and currant jelly stool were significantly higher in patients treated with surgery (p = 0.001 and p = 0.004, respectively). CONCLUSION: Age > 1 year, duration of symptoms > 24 h, currant jelly stool, intussusception > 3.5 cm, and free peritoneal fluid are factors associated with surgical treatment of intussusception in children.

Serum Syndecan-1 levels in patients with immunoglobulin A vasculitis in children.

OBJECTIVE: The aim of this study was to evaluate the serum Syndecan-1 (SDC-1) levels in patients with immunoglobulin-A vasculitis (IgAV) in children and its relation with gastrointestinal involvements. METHODS: Sixty-eight children with IgAV and 48 healthy children were enrolled in this cross-sectional study. Clinical and related laboratory data were collected from a computerized hospital database. Serum SDC-1 was collected on admission prior to treatment. RESULTS: Forty-eight patients fully met the IgAV diagnostic criteria at admission (IgAV group), 20 patients with rash only and diagnosed IgAV during hospitalization (Purpura group). In IgAV group, 30 patients with gastrointestinal involvements (IgAV-GI group) and 18 patients without gastrointestinal involvements (IgAV-NGI group). SDC-1 serum levels were significantly higher in the IgAV group (86.37 ng/mL (IQR 59.16-117.14 ng/mL)) than in the controls (20.37 ng/mL (IQR 15.52-26.45 ng/mL)) and the Purpura group (32.66 ng/mL (IQR 14.87-49.89 ng/mL)). Additionally, SDC-1 (OR = 1.08) was independently associated with IgAV with a cut-off value (sensitivity and specificity) of 66.55 ng/mL (68.8%, 95.0%), and the area under the curve was 0.908. The serum SDC-1 levels of the IgAV-GI group (106.92 ± 50.12 ng/mL) were significantly higher than those in the IgAV-NGI group (67.52 ± 17.59 ng/mL). Logistic regression analysis showed that SDC-1 (OR = 1.03) was independently associated with IgAV-GI with a cut-off value of 89.39 ng/mL. CONCLUSIONS: SDC-1 serum levels may mirror vascular endothelium injury and mucosal damage in IgAV. Its applicability as a surrogate biomarker in IgAV remains to be determined.

Renal biopsy in children with IgA vasculitis / Biópsia renal em crianças com vasculite por IgA

Abstract Introduction Henoch-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. Methods Biopsies 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. Results Of 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). Conclusion Cyclosporin-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.

Resumo Introdução A nefrite da púrpura de Henoch-Schönlein (NPHS) é definida como púrpura de Henoch-Schönlein com envolvimento renal, incluindo hematúria e/ou proteinúria. O objetivo deste estudo foi avaliar os dados de pacientes com NPHS que foram submetidos à biópsia renal e comparar os principais parâmetros clínicos e laboratoriais que podem afetar os achados da biópsia renal, os protocolos de tratamento e o desfecho de curto e longo prazo desses pacientes. Métodos Foram avaliadas retrospectivamente biópsias realizadas em 72 pacientes com NPHS entre Janeiro de 2007 e Janeiro de 2017. Eles foram divididos em dois grupos de acordo com a classificação de biópsia renal do Estudo Internacional de Doenças Renais em Crianças. O desfecho renal, parâmetros clínicos e laboratoriais, protocolos de tratamento e desfechos foram comparados entre os grupos. Foi avaliado o acompanhamento de pacientes de curto e longo prazo. Resultados De 72 pacientes, 47 eram homens (65,3%) e 44 (61,1%) tinham ≤10 anos de idade. A razão neutrófilo-linfócito foi encontrada mais alta em pacientes com envolvimento escrotal (p=0,042). O desfecho desfavorável de curto prazo foi significativamente maior em pacientes com envolvimento escrotal (p=0,038). Constatou-se que pacientes com hipertensão e diminuição da depuração de creatinina apresentaram desfechos mais desfavoráveis no acompanhamento de longo prazo (p=0,029, p=0,040). Conclusão A ciclosporina-A e a ciclofosfamida podem ser eficazes em pacientes com NPHS não responsivos a esteroides. Pacientes com envolvimento escrotal, diminuição da depuração de creatinina e hipertensão devem ser monitorados de perto para sequelas de NPHS.

Diagnostic value of combined detection of urine and plasma kidney injury markers in children with Henoch-Sch?nlein purpura nephritis / 中华检验医学杂志

Objective:To investigate the clinical significance and the diagnostic value of detecting kidney injury biomarkers in urine and serum of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:A total of 216 children with untreated HSPN, who were admitted in Beijing Children′s Hospital of Capital Medical University from January 2018 to December 2019, were recruited in this retrospective study. Two hundred and sixteen healthy children were selected as the healthy control group. We determined the levels of six biomarkers of kidney injury, including transferrin (TRF), immunoglobulin (IgG), microalbumin (mAlb), alpha-1 microglobulin (α1-MG), N-acetyl-β-D-glucosaminidase (NAG) in urine and cystatin C (CysC) in serum. The data from the two groups were analyzed, the diagnostic value of each biomarker was evaluated and a logistic regression model for the diagnosis of HSPN was established. In addition, 60 children with HSPN, who were admitted to our hospital from November 2021 to February 2022 and 60 healthy children, who underwent healthy check up in the same period were included to validate the diagnostic performance of the established logistic model. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of each biomarker.Results:The urine levels of TRF, IgG, mAlb, α1-MG and NAG and the serum level of CysC were significantly higher in the HSPN group than those in healthy control group (all P<0.05). The area under the ROC curve (AUC) of TRF, IgG, mAlb, α1-MG, NAG and the serum levels of CysC was 0.749, 0.719, 0.810, 0.648, 0.828 and 0.790 (all P<0.05). Logistics regression analysis showed that IgG, mAlb and TRF were the three diagnostic determinants of HSPN ( OR=1.083, 1.105, 1.704,all P<0.001), and the AUC was 0.916 of the established logistic model based on these three biomarkers. The sensitivity was 87.4% and the specificity reached 96.2%. The logistic model was validated by independent cohorts, and the AUC was 0.973, the sensitivity was 95.0% and the specificity was 98.3%. Conclusions:The levels of urine TRF, IgG, mAlb, α1-MG, NAG and serum CysC were higher in children with HSPN. The established logistic regression model based on three biomarkers including IgG, mAlb and TRF in this study has satisfactory clinical value in diagnosing HSPN in children.

IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship? / Vasculite por IgA (púrpura de Henoch-Schönlein), nefrite e psoríase em uma criança: existe relação?

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

Henoch Schonlein purpura in pediatric patients with inflammatory bowel disease: A case series.

Retrospective chart review was conducted to identify the clinical features of Henoch Schonlein purpura (HSP) in five children with inflammatory bowel disease (IBD). All five children, four of which were on anti-TNF therapies, experienced the onset of HSP after their IBD diagnosis. HSP averaged 20.8 months in duration. The patients in our cohort, particularly those on anti-TNF therapy for inflammatory bowel disease, experienced chronic and recurrent courses of HSP.

New susceptible locus, rs9428555, is associated with pediatric-onset immunoglobulin A nephropathy and immunoglobulin A vasculitis in Koreans.

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is one of the most common primary forms of glomerulonephritis, while IgA vasculitis (IgAV) is the most common systemic vasculitis in children. OBJECTIVE: Herein we aimed to uncover single nucleotide polymorphism (SNP) markers associated with these two related diseases by applying association tests and Sanger sequencing. METHODS: Within the discovery stage, genomic DNA in blood samples from 101 enrolled patients were genotyped by the Korean Biobank Array. Association tests were performed with 397 Korean reference genomes. In the validation stage, 26 independent samples were genotyped by Sanger sequencing. RESULTS: Four SNPs were identified (P < 5 × 10-8) in the discovery stage. To determine whether the genotypes determined by SNP array were accurate, additional genotyping via Sanger sequencing was performed. As a result, only one SNP, rs9428555, was properly genotyped. In the validation stage, the minor allele (A > G) was found in as many as 15 out of 26 samples (minor allele frequency = 0.288), even though this minor allele is rare in East Asians (< 3%). CONCLUSIONS: We found rs9428555 as a novel susceptible locus associated with the development of both IgAN and IgAV in Koreans. Though we cannot conclude rs9428555 is the unique susceptible locus of IgAN and IgAV, it is likely a good marker as the minor allele of this SNP occurred much more often in the patient group here versus within East Asians as a whole.

Nefropatía por inmunoglobulina A en pediatría, desenlaces clínicos con diferentes esquemas de manejo / IgA nephropathy in pediatric patients, clinical outcomes with three types of management

Resumen Introducción: la nefropatía por inmunoglobulina A (NIgA) es la enfermedad glomerular más común en el mundo. En Colombia, el 11-22 % de las glomerulonefritis primarias en niños corresponden a NIgA y de estos casos, el 30 % progresa a enfermedad renal terminal. Objetivo: describir las características paraclínicas e histopatológicas de la NIgA, así como los resultados clínicos según tres tipos de tratamiento en pacientes pediátricos con esta enfermedad atendidos en un hospital de alta complejidad del suroccidente colombiano. Materiales y métodos: estudio retrospectivo realizado en pacientes pediátricos de entre 1 mes y 18 años de edad con diagnóstico de NIgA. Las variables categóricas se presentaron como proporciones y las continuas con medianas y rango intercuartílico. Se usó la prueba de Fisher para comparar los tres esquemas de tratamiento. Resultados: se incluyeron 58 pacientes pediátricos atendidos entre 1996 y 2013. La media de edad al inicio de síntomas fue 7,5±4,2 años y al momento de la biopsia renal, 10±3,8 años. El 77,6 % de los pacientes presentó hematuria microscópica y el 27,6 %, macroscópica. Además, el 81 % tuvo proteinuria, siendo severa el 29 %. Histológicamente, el 10 % se clasificó como grado I, el 62 % como grado II, el 21 % como grado III y el 7 % como grado IV. Tres pacientes requirieron diálisis y dos, trasplante renal. Los esquemas terapéuticos evaluados fueron: solo prednisona (n=20, 34,5 %), prednisona y mofetil micofenolato (MMF) (n=13, 22,4 %) y sin prednisona ni MMF (n=25, 43,1 %). La diferencia en la presencia de hematuria entre los grupos fue significativa (p>0,001), siendo más frecuente en el grupo sin prednisona ni MMF (68 %). No hubo diferencia entre los grupos de proteinuria, hipertensión arterial y valor de creatinina. La mediana de años entre la biopsia renal y el ultimo control fue de 4 años (RIC 1-7). La supervivencia de la función renal fue del 89,1 % a los 5 años. Conclusión: la NIgA amerita reconocimiento temprano y seguimiento estricto, ya que puede tener desenlaces ominosos como enfermedad renal crónica.

Abstract Introduction: IgA nephropathy (IgAN) is the most common glomerular disease in the world, in Colombia belongs to 11-22 % of primary glomerulonephritis in pediatric patients. Of these, 30 % progress to chronic kidney disease. Materials and methods : It is a retrospective descriptive study. We used median and IRQ for continuous variables, and proportions for categorical variables, Fisher test to compare clinical outcomes. Results: Between 1996 to 2013 58 patients were diagnosed. The mean age at symptoms onset was 7.5 years (SD±4.2) and at the time of renal biopsy was 10 years (SD±3.8). At diagnosis, 77.6 % of the patients showed microscopic hematuria, 27.6 % gross hematuria and 81 % proteinuria, classified as severe in 29 %. Three patients required dialysis and two needed kidney transplant. Three groups with different therapeutic regimens were evaluated: first group only prednisone 34.5 % (n = 20), second group prednisone and mycophenolate mofetil (MMF) 22.4 % (n = 13) and third group without prednisone neither MMF 43.1 % (n = 25). The difference in the presence of hematuria among the groups was significant (p> 0.001), being more frequent in the group without prednisone neither MMF (68 %). There were no significant differences in proteinuria, hypertension or creatinine among the groups. The median of years between the renal biopsy and the last control was 4 years RI 1-7. At five years, the renal function survival probability (GFR >90 ml/min/1.73m2) was 89.1 %. Conclusion: IgAN needs early recognition and strict follow-up, since it may have ominous outcomes.

Vasculitis IgA en el adulto. Informe de caso / IgA vasculitis in adults. Case report

RESUMEN La vasculitis IgA, también conocida como púrpura de Schönlein-Henoch, es una vasculitis leucocitoclástica que involucra pequeños vasos con depósito de inmunocomplejos IgA. Puede abarcar piel, articulaciones, riñones y tracto gastrointestinal. Su presentación en adultos es rara, y las formas clínicas suelen ser más agresivas. Es objetivo del presente trabajo describir el curso y evolución de vasculitis IgA, en un paciente de 59 años, con púrpuras en miembros inferiores y tronco, hematuria macroscópica, y edema de miembros inferiores. Los complementarios mostraron creatininas elevadas, proteinuria de rango nefrótico, elevación de la IgA y anticuerpos contra el citoplasma de los neutrófilos negativos. Se descartaron causas neoplásicas. El estudio anatomo-patológico del riñón concluyó una vasculitis IgA.

ABSTRACT IgA vasculitis, also known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis that involves small vessels with deposition of IgA immune complexes. It can include skin, joints, kidneys, and gastrointestinal tract. Its presentation in adults is rare, and the clinical forms are usually more aggressive. The objective of this study is to describe the course and evolution of IgA vasculitis, in a 59-years-old patient, with purples in the lower limbs and trunk, macroscopic hematuria, and lower limb edema. The complementary ones showed elevated creatinines, nephrotic range proteinuria, elevated IgA and negative antibodies against the cytoplasm of neutrophils. Neoplastic causes were dismissed. The anatomical-pathological study of the kidney concluded IgA vasculitis.

Vasculitis por IgA (Púrpura de Henoch Schönlein) en un paciente pediátrico con COVID-19 y Estrongiloidiasis / IgA (Henoch Schönlein Purpura) Vasculitis In A Pediatric Patient With COVID-19 And Strongyloidiasis

La vasculitis es una enfermedad rara en los niños, siendo la Vasculitis por IgA su presentación más frecuente. Una condición que se ha asociado al desarrollo de vasculitis es la invasión del endotelio vascular por el Strongyloides stercoralis en casos de hiperinfestación. Otra condición aún poco investigada, es la probable asociación de los procesos tipo vasculitis por IgA con la infección por SARS-CoV-2 y el COVID-19 propiamente. Presentamos el caso de una paciente de cuatro años que cursó con lesiones purpúricas palpables a predominio de miembros inferiores, dolor abdominal agudo, y episodios de hemorragia digestiva alta. Inicialmente catalogado como un posible dengue grave y leptospirosis, pero que clínica y laboratorialmente se asoció a un cuadro de vasculitis por IgA. Fue SARS-CoV-2 IgM e IgG: Reactivo. Y tuvo coproparasitológico en el que se identificó al Strongyloides stercoralis. La sintomatología remitió tras la administración de corticoterapia y la evolución fue favorable.

Vasculitis is a rare disease in children, with IgA Vasculitis being its most common presentation. One condition that has been associated with the development of vasculitis is the invasion of the vascular endothelium by Strongyloides stercoralis in cases of hyperinfestation. Another condition that is not yet under-researched is the likely association of IgA vasculitis-like processes with SARS-CoV-2 and COVID-19 infection itself. It is presented the case of a four-year-old patient who healed with palpable purplish lesions to lower limb predominance, acute abdominal pain, and episodes of high digestive hemorrhage. Initially listed as a possible severe dengue and leptospirosis, but clinically and laboratorially associated with IgA vasculitis. It was SARS-CoV-2 IgM and IgG: Reactive. And in parasitological study was identified Strongyloides stercoralis. Symptomatology subsided after administration of corticotherapy and the evolution was favorable.

Vasculitis por IGA (púrpura de henoch schönlein) en un paciente pediátrico con COVID-19 y estrongiloidiasis. Reporte de caso

La vasculitis es una enfermedad rara en los niños, siendo la Vasculitis por IgA su presentación más frecuente. Una condición aún poco investigada, es la probable asociación de los procesos tipo vasculitis por IgA con la infección por SARS-CoV-2. Se presenta el caso de una paciente de cuatro años que cursó con lesiones purpúricas palpables a predominio de miembros inferiores, dolor abdominal agudo, y episodios de hemorragia digestiva alta. Inicialmente catalogado como un posible dengue grave y leptospirosis, pero que clínica y laboratorialmente se asoció a un cuadro de vasculitis por IgA. Fue SARS-CoV-2 IgM e IgG: Reactivo. Y tuvo coproparasitológico en el que se identificó al Strongyloides stercoralis. La sintomatología remitió tras la administración de corticoterapia y la evolución fue favorable. Como conclusión, se expuso un caso infrecuente en la población pediátrica, probablemente asociado a los efectos y daños aún desconocidos de la COVID-19 en la actual pandemia.

Vasculitis is a rare disease in children, with IgA Vasculitis being its most common presentation. One condition that is not yet under-researched is the likely association of IgA vasculitis-like processes with SARS-CoV-2 infection. It is presented the case of a four-year-old patient who healed with palpable purplish lesions to lower limb predominance, acute abdominal pain, and episodes of high digestive hemorrhage. Initially listed as a possible severe dengue and leptospirosis, but clinically and laboratorially associated with IgA vasculitis. It was SARS-CoV-2 IgM and IgG: Reactive. And in parasitological study was identified Strongyloides stercoralis. Symptomatology subsided after administration of corticotherapy and the evolution was favorable. In conclusion, it was presented a rare case in the pediatric population, probably associated with the still unknown effects and damage of COVID-19 in the current pandemic.

Clinical study on Jiedu-Huaban Decoction combined with montelukast sodium chewable tablets in the treatment of children with Henoch Schonlein purpura and blood heat syndrome / 国际中医中药杂志

Objective:To evaluate the efficacy of Jiedu-Huaban Decoction combined with montelukast sodium chewable tablets in the treatment of children with henoch schonlein purpura (HSP). Methods:A total of 80 children with HSP and blood heat syndrome who met the inclusion criteria, from January 2017 to December 2019, were randomly divided into two groups by random number table method, 40 in each group. The control group took montelukast sodium chewable tablets at night, and the study group took Jiedu-Huaban Decoction on the basis of the control group. Both groups were treated for 2 weeks. The disappearance time of gastrointestinal disease, skin purpura, kidney disease, joint swelling and pain were observed. The improvement score of skin purpura was evaluated before and after treatment. The serum levels of IL-6, IL-4, interferon-γ (IFN-γ) and TNF-α were detected by ELISA, and the levels of IgG, IgA and IgM. The T lymphocyte subsets (CD4 + and CD8 +) were measured by nephelometry, and the CD4 +/CD8 +values were calculated. The clinical efficacy was evaluated. Results:The total effective rate was 87.5% (35/40) in the study group and 67.5% (27/40) in the control group, with significant difference between the two groups ( χ2 =4.588, P=0.032). The disappearance time of gastrointestinal disease, skin purpura, kidney disease and joint swelling and pain in the study group were significantly earlier than those in the control group ( t=7.802, 12.167, 7.309, 9.365, all Ps<0.001). After treatment, the serum levels of IL-6, IL-4, IFN-γ and TNF-α in the study group were significantly lower than those in the control group ( t=9.319, 6.738, 8.221, 6.553, all Ps<0.001). The improvement score of skin purpura at 1 week after treatment (2.75 ± 0.69 vs. 3.92 ± 0.83, t=6.856) and 2 weeks after treatment (0.41 ± 0.15 vs. 1.55 ± 0.37, t=18.095) in the study group were significantly lower than those in the control group ( P<0.01). After treatment, the level of IgG, CD4 +, CD4 +/CD8 + in the study group were significantly higher than those in the control group ( t=5.160, 4.558, 3.442, all Ps<0.01), the level of IgA, IgM, CD8 + in the study group were significantly lower than those in the control group ( t=2.614, 6.712, 5.468, all Ps< 0.05). During the treatment, the incidence of adverse reactions in the control group was 17.5% (7/40), and that of the study group was 15.0% (6/40), wherer there was no statistical difference between the two groups ( χ2=0.092, P=0.762). Conclusion:Jiedu-Huaban Decoction combined with montelukast sodium chewable tablets can improve the clinical symptoms of children with HSP and blood heat syndrome, reduce the body inflammatory reaction, improve immunity, with good safety.

Bulluos Henoch-Schönlein Purpura in pediatrics. Case report / Púrpura Henoch-Schönlein bullosa en pediatría. Reporte de caso

Abstract Introduction: Henoch Schönlein purpura (HSP) is the most common type of vasculitis in childhood. HSP affects small blood vessels, and it rarely leads to serious complications such as bullous small vessel vasculitis, as it occurred in the case presented here. Case presentation: 5-year-old male who was brought to a primary healthcare center due to having arthralgia and purple skin lesions on his lower limbs. After the patient was diagnosed with HSP, he developed bullous lesions, so he was hospitalized and analgesic and topical management was started. During his hospital stay, the patient's renal function was monitored, and since he did not experience other complications, he was discharged. Conclusion: The available literature on HSP suggests that its cutaneous bullous manifestation rarely occurs in pediatric population and that, unlike normal HSP cases, it is not always associated with renal and/or gastrointestinal involvement. However, regardless of the dermatological severity of this type of vasculitis, the function of the gastrointestinal and renal systems must be always monitored in these patients.

Resumen Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación. Presentación del caso. Paciente masculino de cinco años que fue traído a un centro de atención primaria con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta. Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.

Effect of Qishen-Xiaodian Decoction combined with laser acupoint irradiation on recurrence oxidative stress and renal function oin children with henoch schonlein purpura / 国际中医中药杂志

Objective@#To explore the effect of Qishen-Xiaodian Decoction combined with laser acupoint irradiation on oxidative stress and renal function on children with recurrent henoch schonlein purpura (HSP).@*Methods@#A total of 120 children with recurrent HSP in the dermatology department clinic of Hospital of Xinle City from January 2015 to November 2017 were divided into two groups according to the random number table method, with 60 cases in each group. The control group took loratadine, Troxerutin and vitamin C orally, while the treatment group took Chinese medicine combined with laser acupoint irradiation based onthe control group. Drugs were taken orally for 4 weeks, laser acupoint irradiation treatment for 2 weeks. The level of glutathione peroxidase (GSH-Px) in plasma was detected by ELISA, the levels of MDA and SOD in plasma were detected by thiobarbituric acid colorimetry and hydroxylamine method, the level of IgG, micro albumin (Alb), β2-microglobulin (β2-MG) in urine were detected by radioimmunoassay, and the clinical effect was evaluated. The recurrence was recorded.@*Results@#After treatment, the plasma GSH-Px (90.45 ± 15.36 μmol/L vs. 81.62 ± 13.68 μmol/L, t=3.318), SOD (99.64 ± 18.66 IU/ml vs. 84.21 ± 16.73 IU/ml, t=4.769) in the treatment group were significantly higher than the control group (P<0.01); the MDA (5.58 ± 1.31 μmol/L vs. 4.37 ± 1.36 μmol/L, t=4.964) was significantly lower than the control group (P<0.01); the ratio of urine IgG (3.48 ± 0.95 mg/L vs. 6.56 ± 1.47 mg/L, t=13.630), Alb (7.80 ± 2.94 mg/L vs. 12.73 ± 4.26 mg/L, t=7.378), β2-MG (4.02 ± 1.61 mg/L vs. 6.95 ± 2.10 mg/L, t=8.577) were significantly lower than those of the control group (P<0.01). The total effective rate was 95.0% (57/60) in the treatment group and 80.0% (48/60) in the control group. There was statistically significant difference between two groups (χ2=6.171, P=0.013). Follow-up of 6 and 12 months, the recurrence rate of the treatment group were significantly lower than that of the control group (χ2 value were 4.931, 4.574, P<0.05).@*Conclusions@#Qishen-Xiaodian Decoction combined with laser acupoint irradiation can improve the oxidative stress of children with recurrent HSP, protect the renal function, improve the clinical efficacy and reduce the recurrence rate.

Púrpura de Shönlein-Henoch en el curso de trombocitopenia inmune primaria crónica. Presentación de un caso / Shönlein-Henoch Purpura in the Course of Chronic Primary Inmmune Thrombocytopenia. Presentation of a Case

Resumen La púrpura de Shönlein-Henoch es una vasculitis de la infancia caracterizada por una púrpura palpable no trombocitopénica y manifestaciones sistémicas, acompañadas de dolor abdominal, sangrado gastrointestinal, glomerulonefritis, artritis y artralgias. Está relacionada con patologías desencadenantes como las infecciones y las picaduras de insectos. Se presenta el caso de un niño de 4 años de edad, que presentaba cuadros alérgicos de rinitis y urticaria. Se le había diagnosticado una trombocitopenia inmune primaria de evolución crónica. A pesar del empleo de diferentes alternativas de tratamiento, recurrió con trombocitopenia muy severa, asociada a cuadro de púrpura y lesiones maculo-papulares y urticarianas. Los complementarios realizados, a excepción del conteo de plaquetas, estuvieron en rangos normales. La evolución fue satisfactoria en un periodo de tres semanas. Se presenta el caso porque es una forma atípica de esta enfermedad.

Abstract Shönlein-Henoch purpura is a common vasculatis in childhood, characterized by a non-trombocytopenic palpable purpura and systemic manifestations, accompanied by abdominal pain, gastro-intestinal bleeding, glomerulonephritis, arthritis and arthralgias. It is the most common of diseases due to disturbances in the vascular component in children. It is related to triggering pathologies as infections and insect bites. A case of a 4 year old boy is presented who presented allergic episodes of rhinitis and urticaria. He had been diagnosed with an chronic primary inmmune thrombocytopenia. In spite of the different treatment approaches, the patient had a severe thrombocytopenia, associated to purpura and macolu-papular lesions and uticaria. Lab tests, with the exception of platelet count, were within the normal limits. The progress was satisfactory in a three week period.

Study of peripheral blood T lymphocyte subsets CD45RA+ and CD45RO+ in children withHenoch-Schonlein purpura / 中国基层医药

Objective@#To observe the change and clinical significance of the peripheral blood T-lymphocytes CD45RA+ and CD45RO+ in children with Henoch-Schonlein purpura (HSP) under different clinical classification.@*Methods@#From October 2015 to July 2017, the clinical data of 80 children with HSP in the Affiliated Hospital of North Sichuan Medical College were retrospectively analyzed.According to the clinical classification, they were divided into three groups: skin involvement group (35 cases), abdominal type group (36 cases), and renal type group (9 cases). Another 80 healthy children were selected as the control group.The changes and clinical significance of peripheral blood T-lymphocytes CD45RA+ , CD45RO+ and CD45RA+ /CD45RO+ ratio were analyzed.@*Results@#Among the 80 children, 40 cases were male and 40 cases were female, with age of (7.2±2.3)years old.The CD45RA+ and CD45RO+ rates in the HSP group were (13.19±7.09)%, (12.07±3.46)%, respectively, which were significantly lower than those in the control group [(23.26±6.01)%, (21.74±3.46)%], the differences were statistically significant (t=9.69, 16.42, all P<0.05). The CD45RA+ ratio, CD45RA+ count and CD45RA+ /CD45RO+ ratio of the kidney type group were (8.02±3.63)%, (2.19±0.33)/μL, (-0.28±0.19), respectively, which were significantly lower than those of the skin group [(15.74±7.71)%, (2.55±0.33)/μL, (0.27±0.12)], the differences were statistically significant(t=3.085, 2.709, 4.013, all P<0.05). The ratio of CD45RA+ /CD45RO+ in the abdominal group was significantly lower than that in the skin group[(-0.07±0.27)vs.(0.27±0.12), t=2.989, P<0.05].@*Conclusion@#Peripheral blood T lymphocyte subsets CD45RA+ and CD45RO+ may play a role in the generation and development of immune function changes in children with HSP, and the decrease of CD45RA+ T cells may be related to the risk of HSP nephritis.