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1.
Am J Kidney Dis ; 74(2): 224-238, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30929851

RESUMEN

RATIONALE & OBJECTIVE: Primary membranoproliferative glomerulonephritis (MPGN) is a rare glomerulopathy characterized by complement dysregulation. MPGN progresses rapidly to kidney failure when it is associated with nephrotic syndrome. We assessed the effects of C5 convertase blockade in patients with MPGN and terminal complement activation. STUDY DESIGN: Prospective off-on-off-on open-label clinical trial. SETTING & PARTICIPANTS: Consenting patients with immune complex-mediated MPGN (n=6) or C3 glomerulonephritis (n=4) with sC5b-9 (serum complement membrane attack complex) plasma levels>1,000ng/mL and 24-hour proteinuria with protein excretion>3.5g identified from the Italian Registry of MPGN and followed up at the Istituto di Ricerche Farmacologiche Mario Negri IRCCS (Bergamo, Italy) between March 4, 2014, and January 7, 2015. INTERVENTION: Anti-C5 monoclonal antibody eculizumab administered during 2 sequential 48-week treatment periods separated by one 12-week washout period. OUTCOMES: Primary outcome was change in 24-hour proteinuria (median of 3 consecutive measurements) at 24 and 48 weeks. RESULTS: Median proteinuria decreased from protein excretion of 6.03 (interquartile range [IQR], 4.8-12.4) g/d at baseline to 3.74 (IQR, 3.2-4.4) g/d at 24 weeks (P=0.01) and to 5.06 (IQR, 3.1-5.8) g/d (P=0.006) at 48 weeks of treatment, recovered toward baseline during the washout period, and did not significantly decrease thereafter. Hypoalbuminemia, dyslipidemia, and glomerular sieving function improved during the first treatment period. 3 patients achieved partial remission of nephrotic syndrome and all had undetectable C3 nephritic factors before treatment. Mean measured glomerular filtration rate was 69.7±35.2 versus 87.4±55.1 and 75.8±42.7 versus 76.6±44.1mL/min/1.73m2 at the start versus the end of the first and second treatment periods, respectively, among all 10 study participants. Unlike C3, sC5b-9 plasma levels normalized during both treatment periods and recovered toward baseline during the washout in all patients. LIMITATIONS: Single-arm design, small sample size. CONCLUSIONS: Eculizumab blunted terminal complement activation in all patients with immune complex-mediated MPGN or C3 glomerulonephritis and nephrotic syndrome, but persistently reduced proteinuria in just a subgroup. TRIAL REGISTRATION: Registered in the EU Clinical Trials Register with study no. 2013-003826-10.


Asunto(s)
Anticuerpos Monoclonales Humanizados/farmacología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Activación de Complemento/efectos de los fármacos , Convertasas de Complemento C3-C5/antagonistas & inhibidores , Inactivadores del Complemento/farmacología , Inactivadores del Complemento/uso terapéutico , Glomerulonefritis Membranoproliferativa/tratamiento farmacológico , Glomerulonefritis Membranoproliferativa/inmunología , Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Estudios Prospectivos , Adulto Joven
2.
G Ital Nefrol ; 29(5): 616-20, 2012.
Artículo en Italiano | MEDLINE | ID: mdl-23117741

RESUMEN

Uremia associated with anticoagulant therapy is a high risk factor for bleeding complications in patients undergoing hemodialysis. We report a case of intrarenal hematoma arising in a uremic patient treated with warfarin. The hematoma was rapidly diagnosed by ultrasonography of the abdomen and treated with embolization. Our experience confirms that the availability of an ultrasound facility within the renal unit allows better assessment of our patients, also in the management of the most fearsome and rare complications. Moreover, it strengthens the evidence that uremic patients are at high risk of bleeding complications when treated with oral anticoagulants.


Asunto(s)
Anticoagulantes/efectos adversos , Hematoma/inducido químicamente , Hematoma/diagnóstico por imagen , Enfermedades Renales/inducido químicamente , Enfermedades Renales/diagnóstico por imagen , Diálisis Renal , Anciano , Diagnóstico Precoz , Humanos , Masculino , Ultrasonografía
3.
G Ital Nefrol ; 33(4)2016.
Artículo en Italiano | MEDLINE | ID: mdl-27545629

RESUMEN

Karyomegalic interstitial nephritis (KIN) is a rare disease entity that was first described by Burry in 1974. The prevalence of this disease is less than 1% and its pathogenesis is unclear. KIN is characterized by chronic tubulointerstitial nephritis associated with enlarged tubular epithelial cell nuclei, which leads to progressive decline of renal function. The disease has no known treatment. Here, we report on a 50-year-old female patient who presented with asymptomatic progressive decline of renal function. Renal biopsy demonstrated chronic tubulointerstitial nephritis with markedly enlarged and hyperchromic nuclei of tubule epithelial cells the hallmark of karyomegalic nephritis. Clinical and pathologic findings of this case are discussed in light of the available literature.


Asunto(s)
Núcleo Celular/patología , Nefritis Intersticial/patología , Enfermedad Crónica , Femenino , Humanos , Persona de Mediana Edad
4.
G Ital Nefrol ; 32(1)2015.
Artículo en Italiano | MEDLINE | ID: mdl-25774589

RESUMEN

Percutaneous ultrasound-guided renal biopsy (RB) is the gold standard for diagnosis of renal diseases. The standard procedure involves biopsy in the prone position (PP) for the native kidneys. In high risk patients, transjugular and laparoscopic RB have been proposed. In patients suffering from obesity or respiratory diseases, the RB of the native kidney in the supine anterolateral position (SALP) represents an alternative to these invasive and expensive methods. We illustrate the technique of execution of RB in the lateral position (LP) on native kidneys. The procedure is safe, effective and has reduced the path travelled by the needle biopsy compared with PP and SALP.


Asunto(s)
Biopsia con Aguja/métodos , Enfermedades Renales/patología , Riñón/patología , Obesidad , Posicionamiento del Paciente/métodos , Ultrasonografía Intervencional , Análisis de Varianza , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estadísticas no Paramétricas
5.
G Ital Nefrol ; 31(1)2014.
Artículo en Italiano | MEDLINE | ID: mdl-24671842

RESUMEN

Goodpasture's disease (GD) is an uncommon and severe autoimmune disorder caused by circulating autoantibodies directed against the glomerular basement membrane cross-reacting with the alveolar basement membrane. GD is clinically characterized by rapidly progressive glomerulonephritis, often associated with pulmonary hemorrhage representing a nephrological emergency. We present the clinical features of 9 cases, diagnosed in 1997-2012, in our Renal Unit. Contrary to previous reports, we found a predominance of GD in females and we observed unusual clinical patterns, such as the association with renal vein thrombosis in a pregnant patient, thrombosis of the pulmonary arteries and a late isolated recurrence of alveolitis. In dialysis-dependent patients, renal transplantation can represent an available treatment option.


Asunto(s)
Enfermedad por Anticuerpos Antimembrana Basal Glomerular , Adulto , Anciano , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/terapia , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
6.
G Ital Nefrol ; 31(4)2014.
Artículo en Italiano | MEDLINE | ID: mdl-25098459

RESUMEN

Percutaneous ultrasound-guided renal biopsy is the gold standard for diagnosis and treatment of renal diseases. Recently, many studies strongly support the role of renal biopsy for the management of small renal mass. The experience of the operator is crucial in reducing the incidence of major complications. The use of simulators can accelerate the learning curve in those individuals who train in renal biopsy. We describe four simple and affordable phantoms for renal biopsy. The first two simulators were constructed by a porcine kidney wrapped in perirenal fat or covered by a flap of abdominal skin. The third simulator was constructed by embedding a porcine kidney in a turkey breast and olives to simulate the presence of small tumors. For the fourth model, we used the loin of a pork. Given the encouraging results of our in vitro study, we believe that simulators allow trainees to familiarize themselves with the handling of the equipment in an environment that is risk-free when compared to the clinical scenario.


Asunto(s)
Endosonografía , Biopsia Guiada por Imagen , Neoplasias Renales/patología , Riñón/diagnóstico por imagen , Riñón/patología , Animales , Modelos Biológicos , Porcinos , Turquía
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