ABSTRACT
La glomerulopatía colapsante (GC) constituye una variedad de la glomeruloesclerosis focal y segmentaria. Afecta tanto a la población adulta (40%) como a la infantil (20%); presentándose con mayor frecuencia en hombres jóvenes y afrodescendientes. Clínicamente se presenta como un síndrome nefrótico, con niveles elevados de úrea y creatinina. Se presenta el caso de paciente femenino de 22 años, quien acude por presentar fiebre, edema matutino en miembros inferiores, e intolerancia oral de 9 días de evolución. Al examen físico: Hipertensión arterial y ascitis. La GC es una entidad poco diagnosticada, que progresa rápidamente a insuficiencia renal terminal a pesar de recibir cualquier tratamiento sistémico descrito hasta la actualidad, por lo que amerita mayor investigación en el ámbito terapéutico(AU)
Collapsing glomerulopathy (GC) is a variety of focal segmental glomerulosclerosis. It affects both adult population (40%) and children (20%); it occurs most often in young people, male and of African descent. Clinically it is presented as a nephrotic syndrome, with high levels of urea and creatinine serum. There is insufficient evidence regarding the treatment of this entity, so that steroids and immunosuppressants are used at high doses. We present the case of a 22-year old female, who presented fever, edema in the lower limbs and oral intolerance of 9 days of evolution. Physical examination showed: high blood pressure and ascitis. This nephropathy is an underdiagnosed entity rapidly progressing to kidney failure despite receiving any systemic treatment described until now, so it merits further research in the therapeutic field(AU)
Subject(s)
Humans , Female , Adult , Urea/analysis , Adrenal Cortex Hormones/therapeutic use , Creatinine/analysis , Glomerulonephritis/pathology , Nephrotic Syndrome , Internal Medicine , Kidney Failure, ChronicABSTRACT
La glomerulonefritis colapsante es una enfermedad poco frecuente que puede estar asociada a distintas causas, una de ellas son las enfermedades autoinmunes y dentro de estas el lupus eritematoso sistémico (LES). De manera frecuente se presenta con un cuadro de severas alteraciones renales que tienden a progresar la enfermedad renal terminal, con escasa respuesta a los tratamientos. Se presenta un caso de glomerulonefritis colapsante asociado a lupus eritematoso sistémico que tuvo una respuesta completa al tratamiento de inducción con la combinación de glucocorticoides, antimaláricos y mofetil micofenolato iniciado precozmente por el diagnóstico temprano realizado por biopsia renal(AU)
Collapsing glomerulonefritis is a slightly frequent disease that can be associated to different causes. Autoimmune diseases are part of those, and inside these, the systemic lupus erythematosus (SLE). It frequently appears with manifestations of severe renal alterations that tend to develop the renal terminal disease, with scanty response to the treatments. It is presented a case of collapsing glomerulonefritis associated to systemic lupus erythematosus that had a complete response to the treatment of induction with the combination of glucocorticoids, antimalarials and mycophenolate mofetil used prematurely after the early diagnosis performed by renal biopsy(AU)
Subject(s)
Humans , Biopsy/methods , Glomerulonephritis/etiology , Glucocorticoids/therapeutic use , Lupus Erythematosus, Systemic , Mycophenolic Acid/therapeutic use , Antimalarials/therapeutic useABSTRACT
Collapsing glomerulopathy (CG) is a recently described form of focal segmental glomerulosclerosis (FSGS) which was defined by renal pathology findings. CG is characterized by severe proteinuria and rapid progressive decline of renal function clinically. We experienced one case of CG in renal allograft. 24 year-old male recipient was admitted for evaluation of proteinuria (5.08 g/day) and increment of serum creatinine level at post-transplant 150 days. The graft biopsy was taken and the pathology specimen demonstrated a typical characteristics of CG such as glomerular capillary collapse, visceral epithelial hypercellularity, deposition of immunoglobulin/C3 and variable degree of tubulointerstitial injury. The patient was negative for HIV infection before transplantation and at the time of biopsy diagnosis. No specific treatment for CG was performed. The patient progressed to the graft failure and returned to hemodialysis 84 days after biopsy. In conclusion, recognition of CG by graft biopsy is important because it is a lesion with a high risk for rapid progression to graft failure.
Subject(s)
Humans , Male , Young Adult , Allografts , Biopsy , Capillaries , Creatinine , Diagnosis , Glomerulosclerosis, Focal Segmental , HIV Infections , Pathology , Proteinuria , Renal Dialysis , TransplantsABSTRACT
Collapsing glomerulopathy is recently described as the disease which causes rapidly progressive renal failure. Clinically, the disease starts with constitutional symptoms, and then nephrotic syndrome with marked proteinuria and hypertension follows. Eventually the disease rapidly progresses to the ESRD within several weeks to months. Its typical renal biopsy findings are extensive glomerular capillary collapse, visceral epithelial cell hypertrophy and hyperplasia, and variable degree of tubulointerstitial inflammation. Such findings closely resemble those of HIV associated nephropathy(HIVAN) except endothelial inclusion body in HIVAN. Here we present, for the first time in Korea, a 16 yrs-old female patient with ESRD in a normal sized kidney. Nephrotic syndrome and hypertension were also accompanied. Serologically, she had no evidence of HIV infection. Though her symptom duration was somewhat shorter than that previously reported, her renal biopsy findings were those of collapsing glomerulopathy as previously described. Her renal function did not return to normal, and now she is on regular hemodialysis, waiting for renal transplantation.