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1.
Medicine (Baltimore) ; 98(41): e17566, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31593141

RESUMO

RATIONALE: Coexistence of Fabry disease and IgM nephropathy is rare. The varying severity and unapparent clinical manifestation of Fabry disease makes it difficult to recognize when coexisting with another more prevalent cause of nephropathy requiring electron microscopy and genetic testing to confirm their coexistence. PATIENT CONCERNS: A 54-year-old female presented with proteinuria without any clinical signs or family history of Fabry disease. DIAGNOSES: Immunostaining of the renal biopsy identified mesangial IgM deposition diagnosing it as IgM nephropathy. The light microscopy indicated prominent vacuolization of podocytes. Further examination of toluidine blue stained semi-thin sections and electron microscopy revealed blue bodies and myelin bodies in the cytoplasm of podocytes, respectively. Mutation analysis detected missense mutation establishing the diagnosis of coexisting Fabry disease. INTERVENTIONS: The patient was treated with angiotensin-converting enzyme inhibitors. Enzyme replacement therapy was not administered due to financial constraints. OUTCOMES: After 2 months of treatment the patient demonstrated urine protein to creatinine ratio of 0.21 g/g. LESSONS: Identifying coexistence of Fabry disease with other nephropathy requires meticulous pathologic investigations including electron microscopy especially when Fabry disease presents with atypical phenotype.


Assuntos
Doença de Fabry/complicações , Glomerulonefrite/diagnóstico , Imunoglobulina M/imunologia , Podócitos/ultraestrutura , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Terapia de Reposição de Enzimas/economia , Doença de Fabry/diagnóstico , Doença de Fabry/genética , Doença de Fabry/patologia , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/patologia , Humanos , Microscopia Eletrônica/métodos , Pessoa de Meia-Idade , Mutação de Sentido Incorreto/genética , Podócitos/patologia , Proteinúria/diagnóstico , Proteinúria/etiologia , Resultado do Tratamento
2.
Zhonghua Nei Ke Za Zhi ; 58(10): 758-762, 2019 Oct 01.
Artigo em Chinês | MEDLINE | ID: mdl-31594174

RESUMO

Objective: To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods: PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results: A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 µmol/L) including creatinine>140 µmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t(2)=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ(2)=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ(2)=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ(2)=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ(2)=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion: Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.


Assuntos
Arterite/diagnóstico , Nefropatias/etiologia , Rim/fisiopatologia , Poliarterite Nodosa/diagnóstico , Adulto , Idoso , Ciclofosfamida/uso terapêutico , Gastroenteropatias , Glomerulonefrite/diagnóstico , Glucocorticoides/uso terapêutico , Humanos , Infarto , Nefropatias/fisiopatologia , Pessoa de Meia-Idade , Poliarterite Nodosa/complicações , Poliarterite Nodosa/tratamento farmacológico , Adulto Jovem
3.
Internist (Berl) ; 60(11): 1201-1208, 2019 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-31511906

RESUMO

This article reports about a 73-year-old woman of Bosnian descent who presented with acute renal failure. A renal biopsy was diagnostic for a postinfect necrotizing and extracapillary proliferative glomerulonephritis. The patient reported a febrile infection fever 2 weeks previously. The diagnostics did not reveal any indications of an ongoing infection. The glomerulonephritis responded to treatment with systemic steroids. The patient was readmitted to hospital 6 weeeks later in a severely ill condition. A gastric biopsy revealed a Strongyloides stercoralis infestation. Due to the systemic steroid therapy the patient had developed a so-called hyperinfection syndrome and died despite treatment on the intensive care unit. This case illustrates the need for awareness of this rare parasitosis, particularly in patients from endemic areas. A likely causal relationship with the glomerulonephritis is discussed and an overview of the diagnostics, course of the disease and treatment of this parasitosis is given.


Assuntos
Lesão Renal Aguda/etiologia , Glomerulonefrite/tratamento farmacológico , Prednisolona/efeitos adversos , Esteroides/efeitos adversos , Strongyloides stercoralis/isolamento & purificação , Estrongiloidíase/diagnóstico , Idoso , Animais , Antiparasitários/uso terapêutico , Evolução Fatal , Feminino , Glomerulonefrite/diagnóstico , Humanos , Ivermectina/uso terapêutico , Prednisolona/uso terapêutico , Esteroides/uso terapêutico , Estômago/microbiologia , Estômago/patologia , Estrongiloidíase/complicações , Estrongiloidíase/tratamento farmacológico
4.
J Korean Med Sci ; 34(24): e173, 2019 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-31222986

RESUMO

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Glomerulonefrite/diagnóstico , Hemorragia/diagnóstico , Pneumopatias/diagnóstico , Penicilamina/efeitos adversos , Adolescente , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/etiologia , Feminino , Degeneração Hepatolenticular/tratamento farmacológico , Humanos , Rim/patologia , Penicilamina/uso terapêutico , Tomografia Computadorizada por Raios X
5.
Klin Lab Diagn ; 64(5): 287-293, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31185152

RESUMO

The paper presents data on the study of the content of cytokines (IL-1ß, RAIL-1ß, IL-2, IL-4, IL-10, IL-17A, TNF-, IFN-γ) in the morning urine using enzyme immunoassay in healthy individuals (n = 20) and in patients with acute glomerulonephritis (n = 93). The determination of cytokine levels in patients was carried out in the debut of the disease and 12 months after the onset of the disease. The obtained indicators of cytokine content in the urine are presented as absolute values in pg/ml and creatinine-normalized values calculated by the formula: cytokine level (pg/ml) / urine creatinine (µmol/ml). The study was made of changes in the content of cytokines in the urine of patients with glomerulonephritis with respect to a group of healthy individuals, as well as the dynamics of the content of cytokines in the urine during the 12-month observation period. The results of the study showed that the absolute values of cytokines in urine can distort the true picture of the cytokine profile of urine in renal pathology. Normalized values of the predominant number of pro- and anti-inflammatory cytokines (IL-1ß, IL-2, IL-8, IL-10, IL-17A and TNF-α) in patients with glomerulonephritis were significantly higher than the corresponding indicators of healthy individuals. The normalized values of cytokines were shown to be as more sensitive indicators than absolute values in the course of analyzing differences in the cytokine profile in patients with glomerulonephritis, depending on chronic and acute course of the disease. These indicators influenced the outcome of glomerulonephritis, assessed, as a rule, 12 months after the onset of the disease. Thus, the low levels of IL-1ß, IL-8 and IL-17А detected in the debut of the disease in combination with the high level of RAIL-1ß determined the chronization of glomerulonephritis. So, the creatinine-normalized cytokine levels in the urine expand the possibilities of using the evaluation of the cytokine profile of urine to establish changes in the cytokine content in the urine in renal pathology and predict the chronization of glomerulonephritis.


Assuntos
Citocinas/urina , Glomerulonefrite/diagnóstico , Rim/fisiopatologia , Creatinina , Glomerulonefrite/urina , Voluntários Saudáveis , Humanos , Técnicas Imunoenzimáticas
6.
J Med Life ; 12(1): 49-55, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31123525

RESUMO

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-negative Rapidly Progressive Glomerulonephritis (RPGN) is a severe form of autoimmune renal injury with a bleak prognosis. A 60-year-old Indian woman was treated with classical homeopathy for ANCA-negative RPGN, and after one year of treatment, serum creatinine and other parameters indicating renal injury dropped steadily despite the withdrawal of immunosuppressive drugs; renal dialysis, which was conducted twice a week initially, was made rarer and stopped after one year. Classical homeopathy may be considered a potential therapeutic modality in severe pathologies. Controlled studies are required to establish further the extent to which classical homeopathy may relieve patients from procedures such as dialysis that cause considerable physical and economic discomfort.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/metabolismo , Progressão da Doença , Glomerulonefrite/imunologia , Glomerulonefrite/patologia , Homeopatia , Medicina de Precisão , Insuficiência Renal/complicações , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/diagnóstico , Humanos , Pessoa de Meia-Idade , Diálise Renal
7.
Int J Mol Sci ; 20(9)2019 May 05.
Artigo em Inglês | MEDLINE | ID: mdl-31060307

RESUMO

Glomerular hematuria is a cardinal symptom of renal disease. Glomerular hematuria may be classified as microhematuria or macrohematuria according to the number of red blood cells in urine. Recent evidence suggests a pathological role of persistent glomerular microhematuria in the progression of renal disease. Moreover, gross hematuria, or macrohematuria, promotes acute kidney injury (AKI), with subsequent impairment of renal function in a high proportion of patients. In this pathological context, hemoglobin, heme, or iron released from red blood cells in the urinary space may cause direct tubular cell injury, oxidative stress, pro-inflammatory cytokine production, and further monocyte/macrophage recruitment. The aim of this manuscript is to review the role of glomerular hematuria in kidney injury, the role of inflammation as cause and consequence of glomerular hematuria, and to discuss novel therapies to combat hematuria.


Assuntos
Glomerulonefrite/complicações , Glomerulonefrite/fisiopatologia , Hematúria/etiologia , Hematúria/urina , Glomérulos Renais/fisiopatologia , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/fisiopatologia , Animais , Biomarcadores , Glomerulonefrite/diagnóstico , Glomerulonefrite/etiologia , Hematúria/diagnóstico , Humanos , Rim/patologia , Rim/fisiopatologia , Estresse Oxidativo , Fenótipo , Prognóstico
9.
Med. clín (Ed. impr.) ; 152(9): 361-367, mayo 2019. ilus, graf, tab
Artigo em Espanhol | IBECS | ID: ibc-183662

RESUMO

La glomerulopatía colapsante (GC) es una entidad poco frecuente como enfermedad glomerular. A pesar de ser considerada como una variante de la glomeruloesclerosis segmentaria y focal, lo cierto es que las lesiones podocitarias presentan rasgos diferenciales respecto a la glomeruloesclerosis segmentaria y focal típica, aspecto que le ha otorgado su clasificación como un tipo de podocitopatía. En la GC la lesión podocitaria se caracteriza típicamente por una desdiferenciación fenotípica, reflejada por la pérdida de la expresión de marcadores podocitarios maduros. La GC puede ser una enfermedad primaria o puede estar asociada a diversos factores causales que desarrollan una entidad histopatológica común. La expresividad clínica de la GC a menudo se caracteriza por la presencia de un síndrome nefrótico y un rápido deterioro de la funcional renal superior a otras variantes de la glomeruloesclerosis segmentaria y focal. El pronóstico de los pacientes afectados por una GC es una rápida progresión hacia la insuficiencia renal terminal con mala respuesta al tratamiento


Collapsing glomerulopathy (CG) is a rare entity as a glomerular disease. Although it has been considered as a variant of focal segmental glomerulosclerosis, the fact is that the podocyte lesions show different features with respect to the typical focal segmental glomerulosclerosis, an aspect that has been attributed to a type of podocytopathy. In CG, the podocyte lesion is typically characterised by a dysregulated podocyte phenotype, reflected by the loss of expression of mature podocyte markers. CG can be a primary disease or it can be associated with several causal factors that develop a common histopathological entity. The clinical expressiveness of CG is often characterised by the presence of a nephrotic syndrome and a rapid deterioration of the renal function than other variants of the focal segmental glomerulosclerosis. The prognosis of these patients is a rapid progression towards end-stage renal disease with poor response to treatment


Assuntos
Humanos , Glomerulonefrite/classificação , Glomerulonefrite/diagnóstico , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/etiologia , Diagnóstico Diferencial , Prognóstico
10.
Am J Case Rep ; 20: 542-547, 2019 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-30996221

RESUMO

BACKGROUND Point-of-care ultrasound (POCUS) is performed at the bedside by a healthcare professional who is directly caring for the patient. Subacute infective endocarditis can be challenging to diagnose, as patients often present with non-specific symptoms. The modified Duke criteria include echocardiographic findings as a major criterion, but the role of POCUS has not been established. This report is of a case of infective endocarditis diagnosed using POCUS. CASE REPORT A 57-year-old man was admitted to hospital with a presumptive diagnosis of rapidly progressive glomerulonephritis secondary to vasculitis associated with a non-specific rheumatologic condition that had developed during the previous three months. Several specialist physicians had previously examined him. On hospital admission, POCUS was performed by the internal medicine physician, which showed mitral valve endocarditis resulting in a change in clinical management from steroid therapy to antibiotic therapy. Blood cultures were performed, which grew Streptococcus mutans. CONCLUSIONS To our knowledge, this is the first reported case of infective endocarditis diagnosed by an internist using POCUS in a patient admitted to hospital with an alternative diagnosis and management plan in place. This case highlights the potential role of POCUS in the acute hospital setting and supports the need for studies to compare the diagnostic performance of POCUS with transthoracic echocardiography for the detection of valvular vegetations. POCUS may be considered for patients with a possible diagnosis of infective endocarditis that cannot be excluded using the modified Duke criteria, potentially resulting in earlier diagnosis and management, with an improved clinical outcome.


Assuntos
Antibacterianos/uso terapêutico , Ecocardiografia Doppler/métodos , Endocardite Bacteriana/diagnóstico por imagem , Estenose da Valva Mitral/tratamento farmacológico , Sistemas Automatizados de Assistência Junto ao Leito , Acidente Vascular Cerebral/etiologia , Tratamento Conservador , Serviço Hospitalar de Emergência , Endocardite Bacteriana/complicações , Seguimentos , Glomerulonefrite/complicações , Glomerulonefrite/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/microbiologia , Multimorbidade , Doenças Reumáticas/complicações , Doenças Reumáticas/diagnóstico , Medição de Risco , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/tratamento farmacológico , Streptococcus mutans/isolamento & purificação , Acidente Vascular Cerebral/terapia , Resultado do Tratamento
11.
Biomed Res Int ; 2019: 6869179, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31032355

RESUMO

Purpose: To identify the epidemiology and pathological types of kidney diseases and their changes during the past decade, in a population from Northeast China. Methods: We retrospectively analysed clinical and renal pathological data from 4910 patients who received renal biopsies in the Second Hospital of Jilin University from 2008 to 2017. Results: Males received more renal biopsies than females (p < 0.001). The average age (p < 0.001) and percentage of elderly patients (p < 0.001) increased over time. The pathological types were primary glomerulonephritis (PGN, 73.2%), secondary glomerulonephritis (SGN, 23.7%), tubular-interstitial nephropathy (TIN, 2.8%), and hereditary nephropathy (HN, 0.3%). The most common forms of PGN were membranous nephropathy (MN, 37.2%) and IgA nephropathy (IgAN, 29.9%). Over time, the prevalence of IgAN decreased, but the prevalence of MN increased. MN was more common in middle-aged and elderly patients, but IgAN was most common in young adults. Analysis of SGN data indicated that lupus nephritis (LN, 34.0%), Henoch-Schönlein purpura glomerulonephritis (HSPN, 17.9%), and diabetic nephropathy (DN, 11.7%) were the most common forms. Over time, the prevalence of DN (p = 0.003), hypertension-associated renal damage (p = 0.005), and systemic vasculitis-associated nephritis (SVARD, p < 0.001) increased, but the prevalence of HSPN (p < 0.001) and hepatitis B virus-associated glomerulonephritis (HBV-GN, p = 0.001) decreased. Nephrotic syndrome was the main clinical manifestation of PGN. Conclusion: From 2008 to 2017, renal biopsies were increasingly performed in the elderly. There were notable changes in the epidemiology and pathological types of kidney disease among renal biopsy patients at our centre.


Assuntos
Biópsia , Glomerulonefrite/epidemiologia , Hipertensão Renal/epidemiologia , Nefrite Hereditária/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Nefropatias Diabéticas/diagnóstico , Nefropatias Diabéticas/epidemiologia , Nefropatias Diabéticas/patologia , Feminino , Glomerulonefrite/diagnóstico , Glomerulonefrite/patologia , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/epidemiologia , Glomerulonefrite por IGA/patologia , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/epidemiologia , Glomerulonefrite Membranosa/patologia , Humanos , Hipertensão Renal/diagnóstico , Hipertensão Renal/patologia , Rim/patologia , Masculino , Pessoa de Meia-Idade , Nefrite Hereditária/diagnóstico , Nefrite Hereditária/patologia , Adulto Jovem
12.
BMJ Case Rep ; 12(4)2019 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-31028048

RESUMO

Vasculitis presents several diagnostic challenges. Herein, we present a clinical case of a 71- year old woman, observed in our emergency department due to asthenia, vomiting and persistent cough. The patient had a history of progressive renal failure and anaemia over the last years. On physical examinations, fine pulmonary crackles were detected and laboratory test showed haemoglobin 69 g/L, creatinine 4 mg/dL, potassium 6.3 mmol/L, positive antineutrophil cytoplasm antibody (ANCA), with proteins and dimorphic erythrocytes in the urinary sediment. CT analysis of the thorax revealed patchy ground glass haziness, likely due to diffuse alveolar haemorrhage. Pulmonary-renal syndrome was assumed, and induction therapy was initiated. She was discharged after 33 days of hospitalisation. On the following months, ANCA titres remained undetectable, but minor recovery of renal function was observed, requiring haemodialysis. Indeed, the use of aggressive induction therapy at early stage dramatically improve prognosis, maintenance of disease remission may be difficult, as relapse is frequent.


Assuntos
Ciclofosfamida/uso terapêutico , Glomerulonefrite/diagnóstico , Hemorragia/diagnóstico , Imunossupressores/uso terapêutico , Pneumopatias/diagnóstico , Plasmaferese/métodos , Idoso , Progressão da Doença , Feminino , Glomerulonefrite/fisiopatologia , Glomerulonefrite/terapia , Hemorragia/fisiopatologia , Hemorragia/terapia , Humanos , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Radiografia Torácica , Resultado do Tratamento
13.
Am J Case Rep ; 20: 508-510, 2019 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-30978177

RESUMO

BACKGROUND Glomerulonephritis (GN) associated with post staphylococcus infection (PSIGN) and high serum immunoglobulin A (IgA) has been reported recently. Patients with GN after infection with underlying IgA nephropathy create a challenge to determine the etiology of GN. Therefore, treatment should be accordingly, with steroids used if the IgA nephropathy flare-up is determined to be the etiology. The aim of this case report was to shed light on the difference between PSIGN and IgA nephropathy flare-ups in patients with a history of IgA nephropathy, and how to treat patient cases accordingly. CASE REPORT An 81-year-old male presented to our Emergency Department complaining of increasing pain, swelling, and redness of his left knee since 2 days ago. He had a history of recent methicillin sensitive Staphylococcus aureus (MSSA) left knee arthroplasty infection that was treated with cefazolin, and he had a history of IgA nephropathy diagnosed 1 year ago. CONCLUSIONS In our patient case, renal biopsy studies were not enough to differentiate between PSIGN and IgA nephropathy flare-ups, thus, clinical presentation was important. PSIGN was found to have a delayed onset compared to IgA nephropathy. Lower serum complement 3 (C3) level, heavier proteinuria, and acute renal failure are common with PSIGN compared to IgA nephropathy. Identifying the etiology and treating our patient accordingly with immunosuppressive therapy had a positive impact on the patient, restoring renal function without further damage.


Assuntos
Glomerulonefrite por IGA/diagnóstico , Infecções Estafilocócicas/complicações , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Cefazolina/uso terapêutico , Diagnóstico Diferencial , Glomerulonefrite/diagnóstico , Humanos , Masculino , Infecções Estafilocócicas/tratamento farmacológico , Staphylococcus aureus
14.
Nefrología (Madrid) ; 39(2): 198-201, mar.-abr. 2019. tab, ilus
Artigo em Espanhol | IBECS | ID: ibc-181328

RESUMO

La gammapatía monoclonal de significado renal incluye todas las enfermedades renales causadas por una inmunoglobulina monoclonal secretada por un clon de célula B no maligno. Por definición, los pacientes con gammapatía monoclonal de significado renal no cumplen criterios de mieloma múltiple y la alteración hematológica es generalmente considerada gammapatía monoclonal de significado incierto. No obstante, la dolencia que pueden causar a nivel renal puede ser importante, requiriendo un tratamiento específico. El espectro de la gammapatía monoclonal de significado renal es amplio, incluyendo una entidad reciente como la nefropatía C3. El desarrollo de una nefropatía C3 en el contexto de una gammapatía monoclonal de significado renal tras el trasplante renal no es frecuente y hasta el momento ha sido poco descrita. A continuación presentamos 3 casos de nefropatía C3 asociados a una gammapatía monoclonal de aparición de novo tras el trasplante renal


Monoclonal gammopathy of renal significance includes all renal disorders caused by a monoclonal immunoglobulin secreted by a non-malignant B-cell clone. Patients with MGRS do not, by definition, meet criteria for multiple myeloma, with haematological disorders generally considered to be monoclonal gammopathy of undetermined significance. Nevertheless, the renal involvement can be serious and require specific treatment. Monoclonal gammopathy of renal significance is associated with a wide spectrum of disorders, including the recently discovered C3 glomerulopathy. Development of C3 glomerulopathy in the context of monoclonal gammopathy of renal significance after kidney transplantation is uncommon and very few cases have been published to date. We report on three cases of C3 glomerulopathy in the context of de novo monoclonal gammopathy after kidney transplantation


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Paraproteinemias/etiologia , Nefropatias/complicações , Insuficiência Renal Crônica/genética , Transplante de Rim/métodos , Glomerulonefrite/diagnóstico , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Imunossupressão/métodos , Rituximab/administração & dosagem , Biópsia , Diagnóstico Precoce
15.
Internist (Berl) ; 60(5): 478-484, 2019 05.
Artigo em Alemão | MEDLINE | ID: mdl-30868251

RESUMO

The rapid progressive glomerulonephritis is an emergency case. Renal function is rapidly lost within weeks or a few months (rarely within days) due to necrotizing extracapillary proliferative crescentic glomerulonephritis. Early diagnosis and treatment improve prognosis, as the best prognostic marker is creatinine when treatment is initiated. Three classes can be distinguished by immunofluorescence in histology. Firstly, there are no or only few immunoglobulins found (anti-neutrophil cytoplasmic antibody [ANCA]-associated vasculitis). Secondly, there is linear immunofluorescence due to antibodies against the glomerular basement membrane (anti-glomerular basement membrane [GBM] disease or Goodpasture syndrome); and thirdly, there is a granular pattern of immunoglobulin deposition (for example systemic lupus erythematosus [SLE], Schoenlein-Henoch purpura or cryoglobulinaemia). The immunosuppressive repertoire has improved (such as induction therapy in ANCA-associated vasculitis with lower total steroid dose, cyclophosphamide pulses or rituximab). New treatment approaches are on their way and the prognosis regarding life expectancy and renal function has improved. There are still challenging questions to answer like treatment duration and markers of recurrence.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Autoanticorpos/sangue , Glomerulonefrite/diagnóstico , Glomerulonefrite/imunologia , Rim/patologia , Doença Antimembrana Basal Glomerular , Ciclofosfamida/uso terapêutico , Humanos , Imunossupressores/uso terapêutico
16.
Pol Merkur Lekarski ; 46(273): 115-121, 2019 Mar 28.
Artigo em Polonês | MEDLINE | ID: mdl-30912519

RESUMO

Acute poststreptococcal glomerulonephritis (APSGN) is a complication of infection with group A beta-hemolytic streptococcus. The disease manifests as microscopic/gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly self-limiting course. AIM: The aim of study was the analysis of clinical course of APSGN in period of increased incidence in the first half of 2018. MATERIALS AND METHODS: We analyzed following parameters in children hospitalized due to APSGN in January-June 2018: age, sex, anthropometric parameters, preceding infection, clinical signs, renal function, biochemical and immunological tests (including antristreptolysins (ASO) and complement), urinalysis, renal ultrasonography, and treatment. The incidence of APSGN in years 2007-2018 was analyzed. RESULTS: We found 11 children (6 boys, 5 girls) aged 5.01±2.44 years. The disease was preceded by pharyngitis in 8, skin infection in 1 with latent period 16.40±5.77 days. Clinical symptoms were: gross hematuria in 8, edema in 6, hypertension in 5, renal function impairment 6, and hyperkalemia in 5; all patients had lowered C3 complement factor; ASO was elevated in all patients except for a boy with skin infection. During hospitalization clinical symptoms resolved in all children; significant elevation in GFR (p=0.018) and C3 (p=0.034), and decrease in proteinuria (p=0.039) were observed. Four patients with abnormal ultrasonographic kidney image were characterized by worse kidney function (p=0.018), higher potassium concentration (p=0.052), higher proteinuria (p=0.073) and erythrocyturia (p=0.015) than remaining children. In follow-up (after 142,00±89,20days) all children had normal renal function and blood pressure, 1 patient had proteinuria, and 4 had erythrocyturia. CONCLUSIONS: In most cases APSGN is characterized by rapid resolution of symptoms and good prognosis, but patients require periodic follow-up visits. Abnormal initial ultrasonographic kidney image may be a marker of worse clinical course of APSGN.


Assuntos
Glomerulonefrite , Infecções Estreptocócicas , Doença Aguda , Criança , Pré-Escolar , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/diagnóstico , Glomerulonefrite/tratamento farmacológico , Humanos , Rim/microbiologia , Masculino , Proteinúria/etiologia , Infecções Estreptocócicas/complicações , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/tratamento farmacológico
17.
Pediatr. aten. prim ; 21(81): 31-35, ene.-mar. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-184525

RESUMO

En los últimos años ha aumentado el número de niños que viajan con sus familias, ya sea por turismo o por regreso a su país de origen. Este hecho supone un incremento de las consultas de este tipo de pacientes en los servicios de urgencias, lo que abre el abanico de posibilidades diagnósticas. La incidencia de la glomerulonefritis posinfecciosa ha disminuido considerablemente en las últimas décadas. En nuestro medio, la faringoamigdalitis estreptocócica continúa siendo la principal causa; sin embargo, en niños procedentes de países menos desarrollados, se debe tener en cuenta que las infecciones cutáneas también representan un origen frecuente de esta patología. Se presenta el caso de un niño de 11 años que acudió al servicio de urgencias por lesiones cutáneas y hematuria


In recent years, the number of children travelling with their families has increased, due to tourism or to return to their origin country. This fact supposes an increase of medical consultations of this type of patients in the emergency department, which opens the spectrum of possible diagnoses. The incidence of postinfectious glomerulonephritis has considerably decreased in recent decades. In our environment, streptococcal pharyngotonsillitis remains the main cause; however, in children from less-developed countries, it must be kept in mind that cutaneous infections also represent a frequent origin of this pathology. We present the case of an 11-year-old boy who came to the emergency department because of skin lesions and hematuria


Assuntos
Humanos , Masculino , Criança , Hematúria/etiologia , Pioderma/etiologia , Glomerulonefrite/diagnóstico , Infecções Estreptocócicas/diagnóstico , Streptococcus pyogenes/patogenicidade , Doença Relacionada a Viagens , Doenças Transmissíveis Importadas/diagnóstico , Antibacterianos/uso terapêutico
18.
Neth J Med ; 77(1): 10-18, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30774098

RESUMO

C3 glomerulopathy is a rare renal disease that has been distinguished as a renal disease for about 10 years. It is caused by an excessive activation of the alternative complement pathway in the circulation, which leads to deposition of complement factor C3 in glomeruli. It is diagnosed based on clinical presentation, histological patterns in a kidney biopsy and tests of the complement pathways. It can closely resemble immune complexmediated glomerulonephritis and postinfectious glomerulonephritis. Renal failure develops in up to half of all patients within 10 years after presentation. A curative treatment is not available. Treatment relies on renoprotective measures, occasional immunosuppressive medication and experimental novel complement inhibitors. Because the disease is rare, its care and cure are concentrated in centers of expertise. Here we provide an overview of the state-ofthe-art diagnosis and treatment of C3 glomerulopathy in a center of expertise in the Netherlands.


Assuntos
Ativação do Complemento/fisiologia , Complemento C3/metabolismo , Glomerulonefrite/diagnóstico , Glomerulonefrite/tratamento farmacológico , Glomérulos Renais/metabolismo , Via Alternativa do Complemento/imunologia , Via Alternativa do Complemento/fisiologia , Glomerulonefrite/imunologia , Humanos , Rim/patologia , Glomérulos Renais/patologia
19.
Medicine (Baltimore) ; 98(7): e14557, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30762802

RESUMO

RATIONALE: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (PCD). The three entities can cause renal lesions via different mechanisms and, however, they have not been reported in a single patient with renal lesion. PATIENT CONCERNS: Here, we describe a patient with half-year fatigue and 40-day nausea and vomiting. Laboratory workup displayed increased serum creatinine, proteinuria, and mild microscopic hematuria. Serological tests were positive for anti-nuclear antibody (titer 1:100), anti-GBM antibodies (not quantified), and myeloperoxidase (MPO)-ANCA (228 RU/ml). Serum immunofixation electrophoresis found monoclonal immunoglobulin (MIg) G κ-light chain in the serum. Renal biopsy displayed crescentic formation in glomerule by microscopy and staining for liner IgG (+), sparse C3 (+-) and light chain (κ and λ) (+-) by immunofluorescence. The bone marrow examination indicated basically normal myelogram and sporadic plasma cells positive for CD38, CD138 staining, and κ light-chain restriction. DIAGNOSIS: Crescentic glomerulonephritis and MGUS. INTERVENTIONS: The patient was treated with plasmapheresis, pulse methylprednisolone therapy in combination with cyclophosphamide. OUTCOMES: The patient still became hemodialysis-dependent. LESSONS: The present study discusses, to the best of our knowledge, first case of crescentic glomerulonephritis seropositive for ANCA anti-GBM antibody in MGUS. The rare concurrence highlights it as a clinical concern.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Autoanticorpos/imunologia , Glomerulonefrite/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Glomerulonefrite/diagnóstico , Glomerulonefrite/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/imunologia
20.
Rev Med Liege ; 74(1): 15-19, 2019 Jan.
Artigo em Francês | MEDLINE | ID: mdl-30680968

RESUMO

Diabetic nephropathy is a complication of diabetes that affects 25-40 % of diabetic patients. This complication is usually associated with other microangiopathic disorders. We describe a case of a patient suffering from type 2 diabetes with proteinuria, and no signs of retinopathy. This case allows us to discuss and review different etiologies of proteinuria in diabetic patients, particularly in patients who don't suffer from retinopathy.


Assuntos
Carcinoma de Células Escamosas/diagnóstico , Glomerulonefrite/etiologia , Neoplasias Pulmonares/diagnóstico , Idoso , Diabetes Mellitus Tipo 2/complicações , Evolução Fatal , Glomerulonefrite/diagnóstico , Humanos , Masculino , Proteinúria/etiologia
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