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1.
Arch. argent. pediatr ; 117(4): 363-367, ago. 2019. ilus, tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1054937

ABSTRACT

La glomerulonefritis rápidamente progresiva de etiología posinfecciosa es rara en la infancia, con una prevalencia estimada del 1-3 %. La mayoría debuta como insuficiencia renal aguda y su tratamiento se basa en el uso de corticoides y ciclofosfamida. Si se realiza diagnóstico precoz, el 70 % presenta una recuperación temprana de la función renal. En los últimos años, se han descrito "glomerulopatías por C3", que presentan características que se superponen. Son útiles, en el diagnóstico diferencial, la inmunofluorescencia y la determinación del factor nefrítico. Se presenta un varón de 4 años que acude por fiebre y cuadro respiratorio. Se observa microhematuria, proteinuria, descenso de filtrado glomerular y descenso de C3, y se sospecha glomerulonefritis aguda. Se realiza una biopsia, cuya microscopía óptica muestra la presencia de semilunas epiteliales, y la electrónica, depósitos subepiteliales en forma de joroba, por lo que se diagnostica glomerulonefritis rápidamente progresiva de etiología posinfecciosa.


Postinfectious glomerulonephritis is rarely presented as rapidly progressive glomerulonephritis in children; the prevalence is approximately 1-3 %. Most children have acute onset of renal failure; initial treatment involves corticosteroids and immunosuppressive therapy. Early diagnosis improves prognosis. In recent years, an entity known as "C3 glomerulopathies" has been described, presenting characteristics that overlap. In the differential diagnosis, the immunofluorescence and the determination of the nephritic factor are useful. We report a 4-year-old boy with fever, respiratory symptoms and hyporexia. Microhematuria, proteinuria, decline in glomerular filtration and depressed C3 were found. Acute glomerulonephritis was suspected. Renal biopsy showed crescent formation, immunofluorescence staining for C3 and subepithelial humps. Therefore, postinfectious glomerulonephritis with crescent formations was diagnosed.


Subject(s)
Humans , Male , Child, Preschool , Glomerulonephritis/diagnosis , Pediatrics , Adrenal Cortex Hormones/therapeutic use , Glomerulonephritis/drug therapy , Immunosuppressive Agents/therapeutic use
2.
J. bras. nefrol ; 41(2): 293-295, Apr.-June 2019. graf
Article in English | LILACS | ID: biblio-1012528

ABSTRACT

ABSTRACT Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.


RESUMO A glomerulonefrite (GN) pauci-imune é mais comum em idosos em comparação com crianças, e a etiologia ainda não é completamente compreendida. A positividade do anticorpo citoplasmático antineutrófilo (ANCA) ocorre em 80% dos pacientes. Relatamos o caso de uma menina de 7 anos de idade que apresentou mal-estar e creatinina discretamente elevada, diagnosticada como glomerulonefrite rapidamente progressiva pauci-imune associada a ANCA com crescentes em 20 dos 25 glomérulos (80%). Destes 20 crescentes, 12 eram celulares, 4 fibrocelulares e 4 globalmente escleróticos. Ela não apresentava púrpura, artrite ou sintomas sistêmicos e respondeu bem ao tratamento imunossupressor inicial, apesar da histopatologia relativamente grave. A paciente recebeu três pulsos de metilprednisolona intravenosa (30 mg/kg em dias alternados) inicialmente e continuou com ciclofosfamida (2 mg/kg por dia) por via oral durante 3 meses com prednisona (1 mg/kg por dia). Em um mês, a remissão foi alcançada com creatinina sérica normal e a prednisona foi gradualmente reduzida. O caso desta criança com uma doença pediátrica relativamente rara enfatiza a importância do tratamento imunossupressor precoce e agressivo em pacientes com GN rapidamente progressiva renal associada à ANCA, mesmo com uma apresentação clínica leve. Como em nossa paciente, os achados clínicos e laboratoriais podem nem sempre refletir exatamente a gravidade da histopatologia renal e, assim, a biópsia renal é obrigatória nessas crianças para orientar a conduta clínica e auxiliar no prognóstico.


Subject(s)
Humans , Child , Antibodies, Antineutrophil Cytoplasmic/blood , Glomerulonephritis/diagnosis , Glomerulonephritis/blood , Kidney/pathology , Biopsy , Methylprednisolone/administration & dosage , Methylprednisolone/therapeutic use , Prednisone/administration & dosage , Prednisone/therapeutic use , Follow-Up Studies , Treatment Outcome , Creatinine/blood , Cyclophosphamide/administration & dosage , Cyclophosphamide/therapeutic use , Glomerulonephritis/drug therapy , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/therapeutic use , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents/therapeutic use
3.
Med. Afr. noire (En ligne) ; 66(6): 323-332, 2019.
Article in French | AIM, AIM | ID: biblio-1266334

ABSTRACT

Introduction : La Glomérulo-Néphrite Aiguë Post-infectieuse (GNAP) reste fréquente chez l'enfant dans les pays en développement. Au Burkina Faso, il existe très peu de données sur le sujet. Le but de ce travail était de contribuer à travers les données fournies par l'étude, à l'amélioration de la prise en charge de la maladie en milieu pédiatrique au Burkina Faso. Patients et méthode : Il s'est agi d'une étude rétrospective descriptive sur la période du 1er janvier 2008 au 31 décembre 2017 (10 ans). Ont été inclus dans l'étude les enfants âgés de 0 à 15 ans hospitalisés dans le service de pédiatrie médicale du Centre Hospitalier Universitaire Pédiatrique Charles De Gaulle (CHUP-CDG) de Ouagadougou pour glomérulonéphrite aiguë post-infectieuse pendant notre période d'étude. Les données collectées ont été traitées sur micro-ordinateur à l'aide du logiciel SPSS dans sa version 20. Résultats : Cinquante-huit patients ont été inclus durant la période d'étude, soit 1,6% des hospitalisations. L'âge moyen était de 5,8 ± 2,9 ans avec des extrêmes de 20 mois et 13 ans. La tranche d'âge la plus concernée était celle de 5 à 12 ans. La prédominance était masculine (65,5%). Les enfants étaient dans la majorité des cas issus de familles à niveau socioéconomique défavorable. Un syndrome œdémateux était retrouvé chez la plupart des patients à l'admission. La tension artérielle a été mesurée chez 44 patients soit 75,86% des cas. Les moyennes des pressions artérielles systolique et diastolique étaient respectivement de 113,18 ± 23 mm Hg (extrêmes = 80 et 190) et 70,82 ± 18 mm Hg (extrême = 40 et 120). Sur le plan paraclinique nous avons noté des ASLO positifs dans 40,4% des cas. Un seul patient avait une protéinurie > 50 mg/kg/24h. La créatininémie moyenne était de 125,6 ± 197,1 µmol/L avec des extrêmes de 17 et 1830 µmol/L. Le traitement était essentiellement symptomatique. La durée moyenne d'hospitalisation était 12,03 ± 8,6 avec des extrêmes de 0 et 47 jours. L'évolution était favorable dans 79,31% des cas. Conclusion : La GNAPI reste relativement fréquente au CHUP-CDG de Ouagadougou probablement à cause des conditions de vie et d'hygiène précaires. L'évolution est généralement favorable mais des complications peuvent survenir


Subject(s)
Burkina Faso , Child , Glomerulonephritis/drug therapy , Glomerulonephritis/nursing , Patients , Pediatrics
4.
Medisan ; 21(8)ago. 2017.
Article in Spanish | LILACS | ID: biblio-894649

ABSTRACT

Se describe el caso clínico de una paciente de 47 años de edad con antecedentes de síndrome linfoproliferativo, que fue ingresada en la sala de Hematología del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, y luego se le trasladó al Servicio de Nefrología por presentar dolor poliarticular, fiebre, edemas, disminución de la diuresis y aumento progresivo de la creatinina plasmática. El cuadro clínico y los exámenes complementarios permitieron diagnosticar una glomerulonefritis rápidamente progresiva como consecuencia de una nefritis lúpica de clase IV. La paciente fue tratada con prednisona y ciclofosfamida, y mostró una evolución satisfactoria sin necesidad de recurrir a tratamiento hemodialítico


The case report of a 47 years patient is described with a history of lymphoproliferative syndrome who was admitted at the Hematology room of Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba, and then she was transferred to the Nephrology Service due to a polyarticular pain, fever, edemas, decrease of diuresis and progressive increase of the plasmatic creatinine. The clinical pattern and the lab tests allowed to diagnose a quickly progressive glomerulonephritis as consequence of a lupus nephritis class IV. The patient was treated with prednisone and cyclophosphamide, and she showed a satisfactory clinical course without the necessity of using hemodyalisis treatment


Subject(s)
Humans , Female , Middle Aged , Lupus Nephritis/diagnosis , Autoimmune Lymphoproliferative Syndrome , Glomerulonephritis/drug therapy , Lupus Erythematosus, Systemic , Secondary Care , Prednisone/therapeutic use , Cyclophosphamide/therapeutic use , Renal Insufficiency
5.
Med. infant ; 23(2): 90-95, junio 2016. tab, ilus
Article in Spanish | LILACS | ID: biblio-882206

ABSTRACT

La glomerulonefritis rápidamente progresiva (GNRP) es una entidad caracterizada por una brusca y progresiva declinación de la función renal y por la presencia en la biopsia renal de proliferación celular extra capilar (semilunas) que ocupan el espacio de Bowmans. Nosotros analizamos en forma retrospectiva 37 niños con diagnóstico de GNRP (50% o más de los glomérulos con semilunas) asistidos en esta institución durante los últimos 20 años. El propósito fue evaluar la presentación clínica e histopatológica, etiología, evolución y factores de mal pronóstico. La edad media al diagnóstico fue de 11 ± 3,5 años. Los síntomas de presentación fueron: hematuria 100% de los casos (hematuria macroscópica 56%); hipertensión arterial 92%; proteinuria 88%; síndrome nefrótico 57%. Fue necesaria diálisis al ingreso en el 64,1% de los casos. Las biopsias renales fueron realizadas a 38 ± 26 días desde el comienzo de los síntomas. El porcentaje de glomérulos que presentaron semilunas fue del 81,4%; las mismas fueron epiteliales en el 28,3% de los casos; fibroepiteliales en el 21,8% y fibrosas en el 31,3%. En el 75,8% de las biopsias se encontró fibrosis intersticial y atrofia tubular moderada y/o severa. La inmunofluorescencia no mostro depósitos de complejos inmunes (GN pauci-inmune) en el 40,6% de las biopsias, mostró depósitos granulares de complejos inmunes en el 48,6% y depósitos lineales de anticuerpos anti membrana basal glomerular (Goodpasture´s) en el 10,8%. El tratamiento fue iniciado a 36 ± 32 días desde el comienzo de los síntomas. Todos los pacientes recibieron tratamiento de sostén; en 29 de ellos se indicaron además esteroides y ciclofosfamida, y en 5 solo esteroides. El tiempo medio de seguimiento fue de 4,6 ± 3,9 años. La sobrevida de los pacientes al final del seguimiento fue del 87% (IC95% 55-97%) y la sobrevida del órgano fue del 17% (IC95% 7-38%). Por análisis multivariado encontramos que la fibrosis intersticial y atrofia tubular moderada y/o severa fue el único factor que se relacionó con pérdida del órgano (OR: 14,6 IC95%2,6-80) p= 0,001. Nuestros resultados muestran que la GNRP en niños es una entidad con pobre pronóstico en relación a la función renal. El factor de peor pronóstico que puede llevar a la pérdida del órgano es el compromiso túbulo-intersticial (AU)


Rapidly progressive glomerulonephritis (RPGN) is characterized by a sudden and progressive decrease of kidney function and extra-capillary cell proliferation (crescents) occupying the Bowman's space on the biopsy. We retrospectively analysed 37 children with RPGN (50% or more of glomeruli with crescents) seen at our institution over the past 20 years. The purpose of the study was to evaluate clinical and histopathological presentation, etiology, outcome, and factors of poor prognosis. Mean age at diagnosis was 11 ± 3.5 years. Presenting symptoms were: hematuria in 100% of the cases (macroscopic hematuria 56%); arterial hypertension in 92%; proteinuria in 88%; and nephrotic syndrome in 57%. Dialysis was necessary on admission in 64.1% of the cases. Kidney biopsies were performed at 38 ± 26 days after symptom onset. The percentage of glomeruli that presented crescents was 81.4%; they were epithelial in 28.3% of the cases, fibroepithelial in 21.8%, and fibrous in 31.3%. In 75.8% of the biopsies interstitial fibrosis and moderate and/or severe tubular atrophy was found. Immunofluorescence techniques did not show immune complex deposits (pauci-immune GN) in 40.6% of the biopsies. Granular deposits of immune complexes were found in 48.6% and linear anti-glomerular basement membrane deposits (Goodpasture´s) in 10.8%. Treatment was started 36 ± 32 days after symptom onset. All patients received support treatment; in 29 steroids and cyclophosphamide were also indicated, and in 5 steroids only. Mean time of follow-up was 4.6 ± 3.9 years. Patient survival at the end of follow-up was 87% (95%CI 55-97%) and organ survival was 17% (95%CI 7-38%). On multivariate analysis we found that interstitial fibrosis and moderate and/or severe tubular atrophy was the only factor related to organ loss (OR: 14.6; 95%CI 2.6-80) p= 0.001). Our results show that RPGN in children has a poor prognosis regarding kidney function. Tubulo-interstitial involvement is the factor of poor prognosis that may lead to organ loss (AU)


Subject(s)
Humans , Infant , Child, Preschool , Cell Proliferation , Disease Progression , Glomerulonephritis/drug therapy , Glomerulonephritis/etiology , Glomerulonephritis/pathology , Prognosis , Cohort Studies , Retrospective Studies
6.
Säo Paulo med. j ; 133(1): 43-50, Jan-Fev/2015. tab, graf
Article in English | LILACS | ID: lil-733008

ABSTRACT

CONTEXT AND OBJECTIVE: Glomerular disease registries are increasing all around the world. The aim of this study was to evaluate the clinical characteristics and treatment response among patients with glomerular diseases followed up in a tertiary hospital in Brazil. DESIGN AND SETTING: Analytical cross-sectional study; tertiary-level public hospital. METHODS: This study included patients with glomerular diseases followed up at a tertiary hospital in Fortaleza, northeastern Brazil. Clinical and laboratory data on each patient were registered. The response to specific treatment was evaluated after 3, 6 and 12 months. RESULTS: The study included 168 patients of mean age 37 ± 14 years. The most prevalent glomerular diseases were focal segmental glomerulosclerosis FSGS] (19.6%), minimal change disease MCD] (17.9%), membranous nephropathy MN] (16.7%) and lupus nephritis LN] (11.9%). The main clinical presentations were nephrotic proteinuria (67.3%) and renal insufficiency (17.9%). The mean proteinuria value decreased after the treatment began. Regarding 24-hour proteinuria on admission, there was no significant difference between patients with a good response and those with no response (7,448 ± 5,056 versus 6,448 ± 4,251 mg/24 h, P = 0.29). The glomerular disease with the highest remission rate was MCD (92%). Absence ...


CONTEXTO E OBJETIVO: Registros de glomerulopatias estão aumentando em todo o mundo. O objetivo deste estudo é avaliar as características clínicas e a resposta do tratamento de pacientes com glomerulopatias acompanhados em um hospital terciário no Brasil. TIPO DE ESTUDO E LOCAL: Estudo transversal analítico. Hospital público terciário. MÉTODOS: O estudo incluiu pacientes com glomerulopatias acompanhados em um hospital terciário de Fortaleza, Ceará, Brasil. Foi realizado registro dos dados clínicos e laboratoriais para cada paciente. A resposta ao tratamento específico foi avaliada após 3, 6 e 12 meses. RESULTADOS: Foram incluídos 168 pacientes, com média de idade de 37 ± 14 anos. A glomerulopatia mais prevalente foi a glomerulosclerose segmentar e focal GESF] (19,6%), seguida pela doença de lesão mínima DLM] (17,9%), nefropatia membranosa NM] (16,7%) e nefrite lúpica NL] (11,9%). As principais manifestações clínicas foram proteinúria nefrótica (67,3%) e insuficiência renal (17,9%). A média dos valores de proteinúria diminuiu após o início do tratamento. Com relação à proteinúria de 24 horas na admissão, não houve diferença significativa entre os pacientes com boa resposta ao tratamento ...


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Adrenal Cortex Hormones/therapeutic use , Glomerulonephritis/drug therapy , Glomerulosclerosis, Focal Segmental/drug therapy , Brazil/epidemiology , Cross-Sectional Studies , Cyclosporine/therapeutic use , Follow-Up Studies , Glomerulonephritis/epidemiology , Glomerulonephritis/pathology , Glomerulosclerosis, Focal Segmental/epidemiology , Glomerulosclerosis, Focal Segmental/pathology , Prevalence , Prognosis , Proteinuria/blood , Remission, Spontaneous , Renal Insufficiency/complications , Time Factors , Treatment Outcome , Tertiary Care Centers/statistics & numerical data
7.
Article in English | WPRIM | ID: wpr-212602

ABSTRACT

Deregulation of soluble apoptosis stimulating fragment (sFas) plays an important role in glomerulonephritis (GN). The study assed the influence of immunosuppressive treatment on serum and urine sFas in patients with proliferative (PGN) and non-proliferative (NPGN) GN, and evaluated the potential of sFas measurements in predicting outcomes. Eighty-four patients with GN (45 males and 39 females) were included. Serum concentration (ng/mL) and urinary excretion (ng/mg of urinary creatinine) of sFas were measured before and after the treatment. After 12 months of therapy with steroids and cyclophosphamide, patients were divided into two subgroups according to the treatment results: Responders (R) and Non-Responders (NR). The sFas urinary excretion was reduced after treatment in both PGN and NPGN (from 17.12 +/- 15 to 5.3 +/- 4.2, P = 0.008 and from 10.11 +/- 6.1 to 3.4 +/- 3.0, P = 0.039; respectively) whereas the sFas serum concentration remained unchanged. In PGN, pre-treatment urinary sFas concentration was significantly lower in the Responders than in Non-Responders (2.3 +/- 3.1 vs 19.4 +/- 14.1, P = 0.003), and was lower still than in both R (P = 0.044) and NR (P = 0.042) subgroups with NPGN. The immunosuppressive treatment reduced sFas urinary excretion in proliferative and non-proliferative GN and results suggest that the lower urinary sFas may be linked with favorable therapy outcomes in patients with PGN.


Subject(s)
Adult , fas Receptor/blood , Cyclophosphamide/therapeutic use , Female , Glomerulonephritis/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Steroids/therapeutic use , Treatment Outcome
8.
Article in English | WPRIM | ID: wpr-25349

ABSTRACT

Interleukin-1 receptor antagonist (IL-1ra), tumor necrosis factor soluble receptors (sTNF-R) type I and II, and regulated upon activation, normal T-cell expressed and secreted (RANTES) play an important role in the modulation of primary glomerulonephritis (GN) course. The aim of the study was to assess whether pre-treatment measurements of IL-1ra, sTNF-R, and RANTES assessed conjointly may be useful as predicting factors in patients with GN. In 84 patients (45 males and 39 female) serum concentration (pg/mL) and urinary excretion (pg/mgCr) of cytokines were measured. After 12 months of therapy with steroids and cyclophosphamide the patients were divided into two subgroups: Responders (R) and Non-Responders (NR) according to the treatment results. The urinary IL-1ra, TNF-RI and RII were significantly higher in R than NR (1,732 vs 646 with P < 0.001, 13.1 vs 6.3 with P = 0.005, and 33.6 vs 14.4 with P = 0.012). The urinary RANTES excretion was increased in NR (79.6 vs 28.5; P < 0.001). The multivariable analysis showed that if conjointly assessed, only urinary IL-1ra, TNF-R I and R II, RANTES with 85% probability pointed the feature remission (R). In conclusion, the urinary excretion of IL-1ra, TNF-R I and R II, and RANTES examined conjointly are effective in predicting favorable response to immunosuppressive treatment in patients with GN.


Subject(s)
Adult , Cyclophosphamide/therapeutic use , Female , Glomerulonephritis/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Interleukin 1 Receptor Antagonist Protein/analysis , Lymphocyte Activation , Male , Middle Aged , Multivariate Analysis , Predictive Value of Tests , Receptors, Tumor Necrosis Factor, Type I/analysis , Receptors, Tumor Necrosis Factor, Type II/analysis , Steroids/therapeutic use , T-Lymphocytes/immunology
9.
Medicina (B.Aires) ; 67(2): 151-155, 2007. ilus
Article in Spanish | LILACS | ID: lil-480614

ABSTRACT

La paquimeningitis hipertrófica es una enfermedad poco frecuente caracterizada por engrosamiento de la duramadre. Presentamos una paciente con esta enfermedad que se manifestó con cefalea crónica y en la que concomitantemente se evidenció una glomerulonefritis necrotizante extracapilar pauciinmune asociada a anticuerpos anticitoplasma de neutrófilos de patrón perinuclear (ANCA-P). El diagnóstico se estableció por resonancia nuclear magnética. Recibió tratamiento inmunosupresor con prednisona y ciclofosfamida con evolución favorable.


Hypertrophic pachymeningitis is a very unusual disease, the main characteristic of which is thickening of the dura mater. We describe a patient who started this illness showing chronic headache and pauci-immune necrotizing extracapillary perinuclear antineutrophil cytoplasmic antibody (P-ANCA) associated glomerulonephritis. The diagnosis was made by brain magnetic resonance image. She received immunosuppressant therapy with prednisonel and cyclophosphamide with clinical improvement.


Subject(s)
Humans , Female , Adult , Antibodies, Antineutrophil Cytoplasmic/blood , Glomerulonephritis/etiology , Meningitis/etiology , Vasculitis/complications , Anti-Inflammatory Agents/therapeutic use , Cyclophosphamide , Glomerulonephritis/diagnosis , Glomerulonephritis/drug therapy , Headache/etiology , Immunosuppressive Agents/therapeutic use , Magnetic Resonance Imaging , Meningitis/diagnosis , Meningitis/drug therapy , Prednisolone/therapeutic use , Vasculitis/drug therapy , Vasculitis/immunology
10.
Article in English | IMSEAR | ID: sea-45067

ABSTRACT

OBJECTIVE: To examine the effectiveness of pulse methylprednisolone in children with nephrotic primary focal segmental glomerulosclerosis. MATERIAL AND METHOD: Medical records of children, who were treated with a pulse methylprednisolone regimen for nephrotic syndrome resulting from primary focal segmental glomerulosclerosis between 1987 and 2005, were retrospectively reviewed. The age, gender, urine protein, serum creatinine, and glomerular filtration rate at the onset of nephrotic syndrome were recorded. Urine protein, serum creatinine, glomerular filtration rate, and percentile of height before and after methylprednisolone treatment were compared. RESULTS: There were six patients (4 male, 2 female) in the present report. The mean age at onset was 9.5 +/- 2.2 years. Hypertension was noted in four patients and mild renal insufficiency in three. All patients had nephrotic-ranged proteinuria at onset and they were initially treated with prednisolone. Two were steroid-dependent and four were steroid-resistant. All of the steroid resistant cases were also resistant to oral cyclophosphamide. After methylprednisolone treatment, remission of proteinuria was noted in five patients (83%) (2 complete, 3 partial). Mean duration to remission was 20.8 weeks. There were no significant changes in serum creatinine (p = 0.43), GFR (p = 0.78) and percentile of height before and after treatment. No hypertension or cardiac arrhythmia was detected during methylprednisolone administration. The follow-up period after completion of the methylprednisolone regimen was 19.5 +/- 15.2 months (range 4-36 months). The clinical course of five patients with remission was characterized by sustained remission in three patients. Two patients relapsed at 2 and 8 months after treatment. CONCLUSION: Methylprednisolone was effective and safe in treating nephrotic children with primary focal segmental glomerulosclerosis. There was a high incidence of relapse shortly after the cessation of treatment. However, a larger number of patients and longer period of follow-up are needed to confirm this conclusion.


Subject(s)
Child , Female , Glomerulonephritis/drug therapy , Glucocorticoids/administration & dosage , Humans , Infusions, Intravenous , Male , Methylprednisolone/administration & dosage , Nephrotic Syndrome/drug therapy , Recurrence , Treatment Outcome
11.
Article in English | IMSEAR | ID: sea-42937

ABSTRACT

Hepatitis B virus (HBV)-associated glomerulonephropathy (HBV-GN) has been increasingly reported, especially in adults. In the present study, the authors investigated the clinical and histopathology features of patients who suspected HBV-GN in 24 patients and age ranging from 23 to 74 years (mean 43 years). Asymptomatic hematuria was the most common presentation (54%); followed by edema and hypertension at equal percentages of 50%. The nephrotic syndrome was presented in 43%, the nephrito-nephrotic syndrome in 3.5%. Clinically suspected rapidly progressive GN was found in 14%. Renal insufficiency was determined in 30%. The most common pathologic finding was IgA nephropathy (IgAN 29%), followed by membranous nephropathy (21%), focal segmental glomerulo sclerosis (FSGS 11%), membranoproliferative GN (11%), post-infectious GN (11%). Liver disease activity also tended to be mild or had no symptoms of hepatitis. The authors remission rates both complete and partial were 75% (higher than the usual report), notwithstanding treatment. The authors achieved a sustained complete remission in half of the patients (3 in 6 cases) treated with steroid alone and 2 out of 7 cases (28.6%) treated with anti-viral therapy. Spontaneous remission was demonstrated in 2 (1 with IgAN + FSGS, and 1 with post infectious GN) out of 6 patients (33.3%). None of the patients in both treatment groups turned to ESRD that occurred in 2 cases receiving non-specific treatment. Of note, all of the patients who received anti-viral therapy or corticosteroid and had complete follow up were in remission either complete or partial.


Subject(s)
Adult , Aged , Antiviral Agents/therapeutic use , Biopsy , Female , Glomerulonephritis/drug therapy , Glucocorticoids/therapeutic use , Hematuria/physiopathology , Hepatitis B/complications , Humans , Male , Middle Aged , Nephrotic Syndrome/drug therapy , Prednisolone/therapeutic use , Renal Insufficiency/drug therapy , Thailand
12.
Article in English | IMSEAR | ID: sea-38846

ABSTRACT

OBJECTIVE: Treatment of primary nephrotic syndrome (NS) with steroid and cyclophosphamide may be unsuccessful and have much toxicity. Therefore, the authors evaluated the outcome of mycophenolate mofetil (MMF) treatment in these patients. MATERIAL AND METHOD: Fourteen primary NS patients who had failure to steroid and/or cyclophosphamide therapy were treated by MMF for at least 3 months as alternative treatment. Median +/- SD (range) of urine protein to creatinine index (UPCI), serum albumin, serum creatinine, serum cholesterol and dose of prednisolone at the start, 1mo, 3 mo, 6mo, and 12 mo after MMF therapy were compared. RESULTS: In the study group, the mean of UPCI decreased significantly from 2.79 +/- 8.1 to 1.81 +/- 1.54 (p = 0.02) at 12 months after the MMF therapy with no significant change in the mean serum creatinine from 1.14 +/- 0.45 to 1.27 +/- 0.67 mg/dL. The mean serum albumin increased significantly from 2.87 +/- 0.56 to 3.46 +/- 0.76 g/dL (p = 0.05) and the mean of prednisolone dosage decreased significantly from 34.64 +/- 19.16 to 11.11 +/- 8.58 mg/day (p = 0.004). For patients with IgM Nephropathy (IgMN), one of three steroid dependent patients presented with improved renal function. One of two patients with focal segmental glomerulosclerosis (FSGS) had a complete remission and one of two patients with IgA nephropathy (IgAN) had improved renal function with partial remission. CONCLUSION: MMF therapy in NS patients with primary glomerular disease was well tolerated and safe. These efficacies can improve NS, stabilize renal function, and achieve steroid withdrawal.


Subject(s)
Adult , Cyclophosphamide/adverse effects , Female , Follow-Up Studies , Glomerulonephritis/drug therapy , Humans , Immunosuppressive Agents/adverse effects , Kidney Glomerulus , Male , Mycophenolic Acid/analogs & derivatives , Nephrotic Syndrome/drug therapy , Retrospective Studies , Steroids/adverse effects , Substance Withdrawal Syndrome/etiology
13.
Article in English | IMSEAR | ID: sea-46383

ABSTRACT

A 19 years old, male patient presented with symptoms of smoky urine for 2 weeks, puffiness of face and diminished urine output for 3 weeks associated with occasional lower abdominal and flank pain. Patient's history, clinical findings and available investigations were strongly suggestive of Idiopathic Rapidly Progressive Glomerulonephritis. The patient showed excellent response to glucocorticoid and cytotoxic agent.


Subject(s)
Adrenal Cortex Hormones/administration & dosage , Adult , Cyclophosphamide/administration & dosage , Drug Therapy, Combination , Glomerulonephritis/drug therapy , Humans , Male , Methylprednisolone/administration & dosage
14.
Rev. méd. Minas Gerais ; 11(2): 97-103, abr.-jun. 2001. ilus, tab
Article in Portuguese | LILACS | ID: lil-587216

ABSTRACT

Os autores apresentam um estudo de revisão sobre a importância de se definir a resposta imune nefritogênica segundo o predomínio de atividade do linfócito T auxiliar 1 (LTh1) ou T auxiliar 2 (L1h2), salientando a estratégia usada para tal definição. Nas glomerulonefrites (GNs) cujos estudos de imuno-histologia detectaram depósitos de imunoglobulinas tipo IgG1 ou IgG2 ou efetores da resposta de hipersensibilidade tardia (RHT), como macrófagos, células T, interleucina-2 (IL-2), gama-interferon (IFN-y) e fibrina haveria um predomínio de atividade de LTh1, correspondendo principalmente às GNs crescénticas (anti-membrana basal glomerular e "pauciimune") e GN membranoproliferativa. Naquelas em que foram detectadas imunoglobulinas tipo IgE e IgG4 ou IL-4, IL-10 e IL-13, haveria um predomínio de atividade de LTh2, correspondendo às GNs membranosa e lesão glomerular mínima. A nefropatia IgA e a nefrite lúpica apresentariam padrão de resposta imune heterogênea tipo LTh1/L1h2. Embora tais características não estejam estudadas para todas as doenças glomerulares, elas auxiliariam no entendimento da resposta imune nefritogênica, possibilitando estabelecer novas estratégias terapêuticas para controlar a evolução da doença glomerular.


This review covers the importance of defining the nephritogenic immune response through the predominance of T helper cells 1 (Thl) or T helper cells 2 (Th2). In glomerulonephritis (GN) such as crescentic GN (anti glomerular basement membrane and pauci-immune) and membrane-proliferative GN, a frequent finding is glomerular deposition of immunoglobulin IgG1 or IgG2 or elements of delayed type hypersensitivity reaction (DTH) such as macrophages, T cells, interleukin-2 (IL-2), gama interferon (IFN-7), and tibrin suggesting an immune response driven by Th 1 . In cases of membranous or minimal changes GNs, thedeposition of immunoglobulins of IgE and IgG4 classes or the finding of IL-4, II.-10 and 1L-13 indicate a predominance of Th2 response. In IgA nephropathy and lupus nephritis the pattern of the immune response was heterogeneous with the elements of Thl /Th2 rype. Although we still don't have caracterized the immune response for all the GNs, it is becoming clear that a better understanding of its pathogenetic mechanisms will allow the stablishment of new therapeutic strategies in the management of GNs.


Subject(s)
Humans , Cytokines/therapeutic use , Glomerulonephritis/drug therapy , T-Lymphocytes , Immunoglobulin A , Immunoglobulin E
15.
Rev. cuba. pediatr ; 72(3): 194-202, jul.-sept. 2000.
Article in Spanish | LILACS | ID: lil-295643

ABSTRACT

Se realizó una revisión bibliográfica. La bibliografía correspondiente a los últimos años se consultó con el objetivo de profundizar en el estudio de la nefropatía IgA y se revisaron las características patogénicas y clinicopatogénicas de la enfermedad, así como su abordaje terapéutico. La nefropatía IgA se reconoce como la causa más común de enfermedad glomerular y fueron la hematuria recurrente y la presencia de depósitos de IgA en el mesangio, los signos clínicos y patológicos más característicos de esta enfermedad. La nefropatía IgA no es una afección benigna, su evolución es crónica y progresiva. La proteinuria es un indicador de mal pronóstico y no existe un tratamiento específico eficaz para ella por lo que constituye un reto para el futuro


Subject(s)
Child , Glomerulonephritis, IGA/pathology , Glomerulonephritis/drug therapy
16.
Asian Pac J Allergy Immunol ; 1999 Dec; 17(4): 281-7
Article in English | IMSEAR | ID: sea-36475

ABSTRACT

The impact of vasculitis as a cause of primary rapidly progressive crescentic glomerulonephritis (RPGN) was examined in patients with Thai ethnic by antineutrophil cytoplasmic antibody (ANCA) test. Thirty patients found in a six years study period were included. Patients' mean age was 34.8+/-16.4 years. Mean crescent score was 86.2+/-22.9%. ANCA proved positive in fifteen patients. This helps to differentiate vasculitis associated (ANCA positive) from nonvasculitis (ANCA negative) RPGN. Incidence of immune complex type RPGN (46.6%) is higher than the Caucasians while the incidence of antiglomerular basement membrane antibody (anti-GBM disease) is much lower. More vasculitis patients were treated with cyclophosphamide (n = 11) than the nonvasculitis group (n = 2). Mean renal survival time of ANCA and non-ANCA associated patients were 26.69 and 14.16 months, respectively. Renal survival of all patients is significantly worse if associated with a high entry creatinine (>6 mg/dl). Our results show that vasculitis associated RPGN is not an uncommon disease in the Thai population and can be recognized initially by ANCA test.


Subject(s)
Adult , Antibodies, Antineutrophil Cytoplasmic/analysis , Cyclophosphamide/therapeutic use , Diagnosis, Differential , Disease Progression , Female , Fluorescent Antibody Technique, Indirect , Follow-Up Studies , Glomerulonephritis/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Male , Survival Analysis , Thailand , Vasculitis/immunology
17.
Rev. méd. Chile ; 127(8): 970-6, ago. 1999. ilus, tab
Article in Spanish | LILACS | ID: lil-253165

ABSTRACT

Anti neutrophil cytoplasmic antibodies are associated to vasculitis and crescentic glomerulonephritis in adults. However, this association has been seldom reported in children. We report two girls aged 12 and 15 years old with ANCA + glomerulonephritis. Both were subjected to a percutaneous kidney biopsy. One girl had to enter a chronic hemodialysis program. The other patient recovered her renal function and after 12 months of treatment with steroids and cyclophosphamide microscopic hematuria and proteinuria persist but with normal kidney function. ANCA should be measured in children with vasculitis and glomerulonephritis


Subject(s)
Humans , Female , Adolescent , Antibodies, Antineutrophil Cytoplasmic , Glomerulonephritis/diagnosis , Antibodies, Antineutrophil Cytoplasmic/metabolism , Glomerulonephritis/complications , Glomerulonephritis/drug therapy , Glomerulonephritis/etiology , Renal Dialysis , Methylprednisolone/therapeutic use , Prednisone/therapeutic use , Renal Insufficiency/etiology , Renal Insufficiency/therapy
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