Efficacy and safety of mycophenolate mofetil versus cyclophosphamide in the treatment of Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria in children: a prospective randomized controlled trial / 中国当代儿科杂志

OBJECTIVE@#To study the efficacy and safety of mycophenolate mofetil (MMF) versus cyclophosphamide (CTX) in the treatment of children with Henoch-Schönlein purpura nephritis (HSPN) and nephrotic-range proteinuria.@*METHODS@#A prospective clinical trial was conducted in 68 pediatric patients who were admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics and who were diagnosed with HSPN and nephrotic-range proteinuria from August 2016 to November 2019. The patients were randomly divided into two groups:MMF treatment (@*RESULTS@#At months 3, 6, and 12 of treatment, there was no significant difference in the complete remission rate and the response rate between the MMF treament and CTX treatment groups (@*CONCLUSIONS@#MMF and CTX have similar efficacy and safety in the treatment of HSPN children with nephrotic-range proteinuria.

Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Clinical features and outcomes of diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis in children

OBJECTIVE: To investigate the outcomes of childhood diffuse endocapillary proliferation Henoch-Schönlein purpura nephritis (DEP-HSPN) in response to early diagnosis and prompt treatment. METHODS: Eleven cases of DEP-HSPN in children were investigated in comparison to HSPN without diffuse endocapillary proliferation (non-DEP-HSPN). RESULTS: DEP-HSPN had a higher prevalence of nephrotic syndrome but a lower prevalence of hematuria compared to non-DEP-HSPN. IgA, IgG and IgM antibody deposition was found in DEP-HSPN by histopathological examination. Proteinuria cleared in all 11 cases through treatment with steroids and/or immunosuppressive drugs. However, half of the DEP-HSPN patients continuously had hematuria after treatment. CONCLUSION: The early diagnosis and prompt initiation of immunosuppressive treatment based on renal biopsy are important for achieving favorable outcomes.

Nefronia en pediatría: dentro del espectro de las infecciones urinarias. Serie clínica y revisión de la literatura / Nephronia in pediatrics: part of the spectrum of upper urinary tract infections. Clinical cases and review of the literature

Nephronia or focal acute nephritis corresponds to a localized inflammatory non-liquefactive kidney infection which may involve parenchyma of one or more renal lobes. It has been suggested that nephronia is part of the spectrum of upper urinary tract infections between acute pyelonephritis and renal abscess. It is associated with a prolonged clinical course, higher levels of inflammatory markers and an increased risk of renal scarring, compared to pyelonephritis. Ultrasound plays a useful role. Nephronia is an under-diagnosed condition, thus, clinical suspicion is important for early diagnosis and appropriate treatment. We present three paediatric cases, and a review of the literature.

La nefronia o nefritis focal aguda es una inflamación renal de etiología bacteriana sin licuefacción que típicamente involucra el parénquima de uno o más lóbulos renales. Dentro del espectro de las infecciones del tracto urinario, se ha planteado que podría ser una entidad intermedia entre la pielonefritis aguda y el absceso renal. Se asocia a un curso clínico más prolongado, mayores marcadores inflamatorios y mayor riesgo de cicatrices renales, al compararla con la pielonefritis. La ecografía renal juega un rol útil en el diagnóstico. Es una entidad subdiagnosticada; por lo que su sospecha es relevante para un diagnóstico oportuno y un tratamiento adecuado. Se presentan tres casos clínicos pediátricos y una revisión del tema.

Anti-inflammatory effects of royal jelly on ethylene glycol induced renal inflammation in rats

ABSTRACT Objective: In this study, anti-inflammatory effects of Royal Jelly were investigated by inducing renal inflammation in rats with the use of ethylene glycol. For this purpose, the calcium oxalate urolithiasis model was obtained by feeding rats with ethylene glycol in drinking water. Materials and Methods: The rats were divided in five study groups. The 1st group was determined as the control group. The rats in the 2nd group received ethylene glycol (1%) in drinking water. The rats in the 3rd group were daily fed with Royal Jelly by using oral gavage. The 4th group was determined as the preventive group and the rats were fed with ethylene glycol (1%) in drinking water while receiving Royal Jelly via oral gavage. The 5th group was determined as the therapeutic group and received ethylene glycol in drinking water during the first 2 weeks of the study and Royal Jelly via oral gavage during the last 2 weeks of the study. Results: At the end of the study, proinflammatory/anti-inflammatory cytokines, TNF-α, IL-1β and IL-18 levels in blood and renal tissue samples from the rats used in the application were measured. Conclusion: The results have shown that ethylene glycol does induce inflammation and renal damage. This can cause the formation of reactive oxygen species. Royal Jelly is also considered to have anti-inflammatory effects due to its possible antiradical and antioxidative effects. It can have positive effects on both the prevention of urolithiasis and possible inflammation during the existing urolithiasis and support the medical treatment.

Nefritis aguda focal: caso clínico / Focal acute nephritis: a case report

Focal acute nephritis (FAN) or acute lobar nephronia is a rare clinical picture characterized by an infection localized in the kidney, with an inflammatory mass without liquefaction. Of variable clinical manifestations, diagnosis is achieved through CT scanning. Histologically, unlike acute pyelonephritis, it does not present a diffuse infíltrate. Objective: Case report of FAN in a pediatric patient. Case Report: Ten year old male complaining of abdominal pain, presents painful percussion in the right lumbar fossa. Urinary analysis and culture were negative, renal sonogram was negative. Abdominal CT sean showed múltiple hypodense renal foci. He responded well to cephotaxim, being discharged in the third day of hospitalization with completion of treatment as outpatient. Differential diagnosis with Acute Pyelonephritis is very important, as it requires controlled and opportune treatment to prevent renal absceses. Diagnosis of this pathology must be pursued despite a normal UA.

La nefritis aguda focal o nefronia lobar aguda constituye un cuadro poco común caracterizado por una infección localizada en el riñon, la que corresponde a una masa inflamatoria sin licuefacción. Posee una clínica variable, siendo la tomograña computada (TAC) la prueba más sensible y específica para el diagnóstico de esta enfermedad. Esta patología se diferencia histológicamente de la pielonefritis aguda por no presentar un infiltrado inflamatorio difuso. Objetivo: presentar un caso de nefronia aguda multifocal en un paciente pediátrico. Caso clínico: Escolar de 10 años que consultó por dolor abdominal, al examen destacaba la presencia de percusión dolorosa en fosa lumbar. Los exámenes de orina y urocultivo fueron negativos. Al ingreso no se detectó cambios renales ecográficamente evidenciables. Se realizó un TAC de abdomen que mostraba múltiples focos renales hipodensos. Respondió favorablemente a terapia antibiótica con cefotaxima siendo dado de alta al tercer día, completando terapia en forma ambulatoria. La diferenciación de este cuadro de otros procesos renales como la pielonefritis aguda (PNA) es muy importante, ya que precisa un tratamiento oportuno y controlado por el riesgo de evolucionar a absceso renal. El diagnóstico de esta patología debe ser buscado a pesar de contar con exámenes de orina negativos.

Radiation nephritis: anti-inflammatory effect of dexamethasone in adult Wistar rats (Rattus norvegicus) / Nefritis por radiación: efecto anti-inflamatorio de la dexametasona en ratas Wistar adultas (Rattus norvegicus)

The anti-inflammatory effect of dexamethasone on the irradiated kidneys of adult Wistar rats (Rattus norvegicus) was studied. Eighteen adult Wistar rats were, after acclimatization, randomly divided into 3 groups of 6 animals per group. The control group had normal saline, receiving neither drugs nor radiation. The second group received normal saline and radiation. The third group received pretreatment with dexamethasone at 1mg/kg body weight/day for 2 days followed by radiation. Radiation was delivered to the animals as a single fraction of 2.5 Gy of gamma rays from Cobalt-60 source, using an AECL Theatron 780-C Teletherapy machine. After exposure to the different interventions, the animals were sacrificed on the 14th post-irradiation day and the kidneys dissected out from each animal. The renal tissues were subjected to histological processing, and then studied using an eyepiece objective ruler calibrated with a 2mm stage micrometer for histomorphometric studies. The result of the study showed that all irradiated animals suffered weight loss by the 14th day post-irradiation (p<0.05) irrespective of the additional treatment with dexamethasone and this was statistically significant. Histomorphometry showed that the maximum width of the glomerular capsule was significantly greater in the radiation groups than in the control at p<0.05. The maximal glomerular diameter was significantly greater in irradiated animals compared with the control animals at p<0.05. The outcome of this study showed that the intraperitoneal administration of dexamethasone at 1mg/kg body weight/day for 2 days prior to treatment with irradiation did not prevent weight loss nor ameliorate the swelling of the nephrons resulting from the effect of radiation injury to the Wistar rat.

Fue estudiado el efecto anti-inflamatorio de la dexametasona en riñones irradiados de 18 ratas Wistar adultas (Rattus norvegicus). Luego de la aclimatización, aleatoriamente se dividieron en 3 grupos de 6 animales por grupo. El grupo control recibió una solución salina normal, sin recibir drogas ni radiación. El segundo grupo recibió solución salina normal y radiación. El tercer grupo recibió tratamiento previo con dexametasona con 1 mg / kg de peso corporal / día, durante 2 días, seguido de radiación. Los animales fueron expuestos a radiación con una fracción independiente de 2.5 Gy de rayos gamma por una fuente de Cobalto-60, usando una máquina de teleterapia AECL Theatron 780-C. Después de la exposición a las diferentes intervenciones, los animales fueron sacrificados el día 14 post-irradiación y los riñones de cada uno de los animales fueron disecados. Los tejidos renales fueron sometidos a procesamiento histológico, y luego se estudiaron utilizando un objetivo ocular milimetrado calibrado a 2mm para el estudio histomorfométrico. Se demostró que todos los animales irradiados sufrieron pérdida de peso 14 días después de ésta (p <0.05), independientemente de los tratamientos adicionales con dexametasona , siendo estadísticamente significativo. La histomorfometría mostró que el ancho máximo de la cápsula glomerular fue significativamente mayor en los grupos irradiados que en el control en p <0.05. El diámetro máximo del glomérulo fue significativamente mayor en los animales irradiados en comparación con los animales control p <0.05. Los resultados de este estudio mostraron que la administración intraperitoneal, de 1 mg / kg de peso corporal / día durante 2 días, de dexametasona antes de comenzar el tratamiento con irradiación, no impide la pérdida de peso ni permite aliviar el edema de los nefrones, injuria producto de la radiación a las Ratas Wistar.

young girl with acute lobar nephronia

We report a young girl with acute lobar nephronia. She presented with signs and symptoms suggestive of a febrile urinary tract infection. Sonography, radioisotope study and enhanced computed tomography scan confirmed the diagnosis of acute lobar nephronia. She was treated with intravenous antibiotics for two weeks followed by long-term prophylaxis antibacterial therapy. Despite clinical recovery, follow-up computed tomography scams and isotope studies showed residual renal scarring of the affected kidney

Individual or combined effects of enalapril and verapamil on chronic cyclosporine nephrotoxicity in rats

Previous studies have demonstrated that enalapril and verapamil seem to attenuate the cyclosporine nephrotoxicity. However, the mechanisms have not been completely understood, especially on molecular events. The aim of this study was to examine the effect of individual or combined treatment on osteopontin, TGF-beta, endothelin-1 and procollagen alpha 1(I) mRNA expressions. Enalapril (50 mg/L in drinking water) and verapamil (0.5 mg/kg/day, subcutaneously), alone or in combination, were administered to rats with chronic cyclosporine nephrotoxicity (cyclosporine, 25 mg/kg/day, subcutaneously) (n = 5 each). Five rats treated with olive oil vehicle were used as control. After 4 weeks, biochemical parameters were measured, and renal cortical mRNA levels were evaluated by Northern blot analysis. Cyclosporine reduced renal creatinine clearance significantly and induced renal cortical osteopontin, TGF-beta, endothelin-1 and procollagen alpha 1(I) gene expressions around 13.5 +/- 1.3, 2.4 +/- 0.2, 1.5 +/- 0.1, 1.9 +/- 0.1 folds, respectively. Individual treatment with enalapril or verapamil significantly suppressed the osteopontin and TGF-beta mRNA expression, but not endothelin-1 and procollagen alpha 1(I). Combined treatment also inhibited the osteopontin and TGF-beta mRNA expression but there was no difference between combined and individual treatment. In conclusion, enalapril or verapamil significantly blunted the cyclosporine-induced osteopontin and TGF-beta gene expressions. However, combined treatment did not show any additive effect.

Nephrotoxic nephritis in rats: treatment with heparin, cyclophosphamide and betamethasone

Ratos foram inoculados com 0,8 ml/100 g de coelho anti membrana basal de rato de título 1/160. Um grupo (T) foi tratado com heparina, ciclofosfamida e betametasona; o outro (NT) näo recebeu tratamento. Foram feitas as seguintes observaçöes: a) nos animais do grupo T a proteinuria diminuiu a partir do 14§ dia; b) os clearances de creatinina foram menores nos animais do grupo T; c) as lesöes histológicas foram semelhantes em intesidade em ambos os grupos, mas os animais do grupo T apresentaram lesöes periglomerulares, intracapsulares e peritubulares näo observados no grupo NT; d) os depósitos de anticorpos autólogos foram menores no grupo T, sendo a deposiçäo de fibrina semalhante em ambos; e) 100% dos animais do grupo NT sobreviveram enquanto a mortalidades dos animais do grupo T foi 40%. Conclue-se que a associaçäo das drogas, além de seus efeitos secundários e da alta mortalidade näo melhorou a nefrite nefrotóxica do rato e contribuiu para o aparecimento de lesöes adicionais que diminuíram a funçäo renal