[Long-term outcome of type V lupus membranous glomerulonephritis]. / Curso clínico de la nefropatía membranosa lúpica pura.

BACKGROUND: The long-term outcome of the pure form of WHO type V lupus membranous glomerulonephritis is apparently more benign than that of other forms of lupus glomerulonephritis. However 12% of such patients progress to terminal renal failure. The presence of proteinuria may be an indication of cytotoxic agents. AIM: To study the clinical long-term outcome of WHO type V lupus membranous glomerulonephritis. MATERIAL AND METHODS: A retrospective analysis of all kidney biopsies of a University Pathology Department, with the diagnosis of WHO type V lupus membranous glomerulonephritis. Review of medical records of patients with the disease and one clinical assessment of all living patients. RESULTS: Between 1973 and 2000, 703 kidney biopsies were done to patients with systemic lupus erythematosus. Of these, 40 were membranous glomerulonephritis and in 33 patients (28 women, age range 6-71 years), data on the evolution and survival was obtained. Nineteen had type Va and the rest type Vb nephritis. Two presented with renal failure and 11 with proteinuria over 3.5 g/24 h. The median follow-up since the renal biopsy was 63 months (range 1-316). At the end of follow-up, four had a creatinine clearance of less then 15 ml/h and four a clearance between 15 and 29 ml/h (one of these received a renal allograft). Eleven (33%) patients had died, mostly due to infections. Life expectancy at five years with a creatinine clearance over 15 ml/h was 75%. Bad prognostic factors were an elevated creatinine clearance over 15 ml/h was 75%. Bad prognostic factors were an elevated creatinine and high blood pressure at the moment of the biopsy. CONCLUSIONS: The clinical outcome of these patients was bad. Twelve percent reached a stage of terminal renal failure. This is in contrast with the 3% progression to a similar stage of proliferative glomerulonephritis treated with i.v. cyclophosphamide. New therapies for this condition must be sought.

Curso clínico de la nefropatía membranosa lúpica pura / Long-Term outcome of type V lupus membranous glomerulonephritis

Background: The long-term outcome of the pure form of WHO type V lupus membranous glomerulonephritis is apparently more benign than that of other forms of lupus glomerulonephritis. However 12percent of such patients progress to terminal renal failure. The presence of proteinuria may be an indication of cytotoxic agents. Aim: To study the clinical long-term outcome of WHO type V lupus membranous glomerulonephritis. Material and methods: A retrospective analysis of all kidney biopsies of a University Pathology Department, with the diagnosis of WHO type V lupus membranous glomerulonephritis. Review of medical records of patients with the disease and one clinical assessment of all living patients. Results: Between 1973 and 2000, 703 kidney biopsies were done to patients with systemic lupus erythematosus. Of these, 40 were membranous glomerulonephritis and in 33 patients (28 women, age range 6-71 years), data on the evolution and survival was obtained. Nineteen had type Va and the rest type Vb nephritis. Two presented with renal failure and 11 with proteinuria over 3.5 g/24h. The median follow-up since the renal biopsy was 63 months (range 1-316). At the end of follow-up, four had a creatinine clearance of less then 15 ml/h and four a clearance between 15 and 29 ml/h (one of these received a renal allograft). Eleven (33percent) patients had died, mostly due to infections. Life expectancy at five years with a creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine clearance over 15 ml/h was 75percent. Bad prognostic factors were an elevated creatinine and high blood pressure at the moment of the biopsy. Conclusions: The clinical outcome of these patients was bad. Twelve percent reached a stage of terminal renal failure. This is in contrast with the 3percent progression to a similar stage of proliferative glomerulonephritis treated with i.v. cyclophosphamide. New therapies for this condition must be sought.

Nefritis intersticial aguda y lamotrigina / Acute interstitial nephritis associated to lamotrigine use: report of one case

Acute interstitial nephritis is a mononuclear and sterile inflammation of the renal interstice caused by drugs, infections or immune phenomena. The clinical presentation is characterized by the triad of rash, fever and eosinophilia. We report a 32 years old man, in treatment with lamotrigine for depression, admitted to the hospital due to fever, abdominal pain, jaundice, cutaneus rash and malaise. Due to an oliguric renal failure of acute onset, a renal biopsy was done. The pathological study showed a granulomatous acute interstitial nephritis. He was started on hemodialysis and treated with cessation of the drug and corticosteroids, with complete recovery of the renal function (Rev Méd Chile 2004; 132: 742-6).

Insuficiencia renal y respiratoria en una embarazada con esclerosis sistémica progresiva: caso anatomo-clínico / Renal and respiratory failure in a pregnant woman with progressive systemic sclerosis: anatomical clinical case

Pregnancy in patients with systemic sclerosis may predispose to a fast progression of the disease. We report a woman with systemic sclerosis and a multiple visceral involvement that during the third trimester of pregnancy developed a respiratory failure caused by interstitial fibrosis complicated with alveolar hemorrhage, bronchopneumonia and respiratory distress syndrome and a renal failure. On admission, she was subjected to a cesarean section, delivering a 1205 g newborn. In spite of intensive care support, the multisystemic failure became unmanageable and the patient died 15 days after admission. Literature review, although sometimes controversial, indicates that pregnancy is a situation with definitive death risk for patients with systemic sclerosis, that requires a close follow up

Nefropatía por IgA (enfermedad de Berger): experiencia en 78 casos / Ig-A nephropathy

We studied 78 patients with a diagnosis of IgA nephropathy. Renal biopsy was indicated in 69 patients by the presence of macroscopic hematuria (52%), microhematuria or renal failure (14%) or hephritic syndrome (1%). Nine were healthy subjects being studied as live kidney donors. An association with IgG and/or IgM was present in 92% of patients. Serum IgA was elevated in 36% of patients. Hypertension was present in 30% and decreased renal function in 29%. Patients with serum creatinine above 1,5 mg/dl tended to be older (33.8 vs 28.7 years) and to have hypertension (52% vs 19%). among 25 pátients followed for more than 12 months renal function remained stable in 44%, deteriorated in 20% and 36% developed renal failure. the latter was associated to older age, hypertension, abscense of macroscopic hematuria and nephrotic syndrome. The 9 live donors had no clinical manifestations of renal disease. Thus, IgA nephropathy is a highly variable clinical manifestations and its prognosis. An asymptomatic course is demonstrated in some subjects

Nefrosis lipoidea en la infancia: evolución y correlación anatomoclínica / Lipoid nephrosis in children. Development and anatomoclinical correlation

Se presentan 34 pacientes; 23 hombres, con diagnóstico de nefrosis lipoidea (NL) confirmado con biopsia renal. El promedio de seguimiento fue 6,8 años (rango 2,2 a 14 años); 97% de pacientes respondieron satisfactoriamente a los esteroides, a los inmunosupresores o ambos. Durante la actividad de la enfermedad 4,7% de los episodios se complicaron con infecciones graves; 94% de los pacientes estaban en remisión a los 5 años de iniciada la enfermedad. Las lesiones morfológicas renales varían con el tiempo de evolución (x 39 días en casos con desaparición completa de los pedicelos, 11 meses en 14 casos con desaparición parcelar y 20 meses en 15 casos con zonas de desaparición segmentaria alternando con otras con pedicelos normales). No se observaron diferencias significativas entre 2 biopsias en cinco pacientes dependientes (n = 4) o resistentes (n = 1) a los corticosteroides a quienes se practicaron biopsias renales. No hubo mortalidad en esta serie ni deterioro de la función renal

Glomerulonefritis asociada a derivación ventriculoatrial / Glomerulonephritis associated with atrial ventricle derivation

Se presentan 5 pacientes con glomerulonefritis asociada a derivación ventriculoatrial. La edad al diagnóstico fluctuó entre 5 y 12 años, tres eran varones. En todos la derivación fue secundaria a hidrocefalia. El intervalo entre la colocación de la derivación y la nefropatía varió entre 1 año 6 meses y 5 años. Todos los casos tuvieron fiebre, anemia y proteinuria de grado variable. Hematuria microscópica se observó en 4 pacientes. Tres niños desarrollaron un síndrome nefrótico y otros 2 insuficiencia renal. El Staphylococcus epidermidis fue el agente aislado con frecuencia. La biopsia renal mostró en todos los casos una glomerulonefritis mesangiocapilar. En 4 pacientes el tratamiento consistió en antibióticos y retiro de la derivación. Dos fallecieron a causa de la septicemia. Tres pacientes están sanos con función renal normal. Se destaca la evolución favorable en relación al retiro de la derivación, que detiene la bacteremia, suprime el factor inmunológico y conduce a la mejoría de la enfermedad

Linfangiectasia pulmonar congénita / Congenital pulmonary lymphangiectasis

Presentamos el caso de un recién nacido con linfangiectasia pulmonar congénita no asociada a otras malformaciones congénitas, que falleció dentro de las primeras 24 horas de vida, destacando los aspectos clínicos, radiológicos y anatomopatológicos

Trasplante renal / Renal transplantation

Se describe en este artículo los cambios morfológicos más característicos que aparecen en el injerto renal en relación a causas que ocasionan falla renal como: mala preservación; rechazo; otras nefropatías (recurrente, de novo, etc); complicaciones médicas y/o quirúrgicas como infecciones, vasculares, urológicas y tóxicas

Pielonefritis xantogranulomatosa / Xantogranulomatous pielonephritis

La pielonefritis xantogranulomatosa (PXG) es una lesión inflamatoria crónica del riñon poco frecuente, relacionada con la obstrucción renal crónica y la infección urinaria recurrente. Puede simular cualquier cuadro inflamatorio o neoplásico haciendo difícil su diagnóstico preoperatorio. Hay pocos casos descritos en la literatura y en el medio nacional no encontramos información al respecto. Presentamos una serie de 17 pacientes tratados en nuestro servicio en un período de 20 años