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Renal thrombotic microangiopathy(TMA) is an uncommon vascular complication of severe hypertension.Until now,its clinical-pathological characteristics and renal survival have been unclear.Twenty-one patients with biopsy-proven renal TMA and with severe or malignant hypertension were retrospectively studied.All the patients exhibited severe hypertension,with systolic blood pressure at 200-280 mmHg and diastolic pressure at 110-180 mmHg.No patients had hemorrhagic manifestations.Elevated lactate dehydrogenase and thrombocytopenia were found in 6 and 5 patients,respectively.Significant proteinuria(> 3 g/day) was present in 2 patients and microscopic hematuria in 18 patients.All patients presented with renal insufficiency(creatinine 3.1 +/-2.1 mg/dL).The level of von Willebrand factor:antigen(vWF:Ag) in patients was not significantly higher than that in the healthy subjects,while the ADAMTS13(a disintegrin and metalloprotease,with thrombospondin-1-like domains) activity was not significantly lower than that in the healthy subjects.Renal histology showed a TMA involving preglomerular arterioles and/or interlobular arteries character-ized by fibrin deposits and vascular wall sclerosis.Fibrin glomerular microthrombi were not observed in these patients.Four patients required hemodialysis upon admission for severe acute renal failure.On follow-up,3 patients had recovered normal renal function and 14 had mild renal insufficiency(creatinine 1.8 +/-0.3 mg/dL),while 4 patients still required persistent hemodialysis.In conclusion,compared with patients having hemolytic uremic syndrome/thrombocytopenic purpura,our patients showed a low incidence of throm-bocytopenia and better renal outcome.
Renal thrombotic microangiopathy (TMA) is an uncommon vascular complication of severe hypertension. Unite now, its clinical-pathological characteristics and renal survival have been unclear.Twenty-one patients with biopsy-proven renal TMA and with severe or malignant hypertension were retrospectively studied. All the patients showed severe hypertension, with systolic blood pressure at 200-280 mmHg and diastolic pressure at 110-180 mmHg. No patients had hemorrhagic manifestations. Elevated lactate dehydrogenase and thrombocytopenia were found in 6 and 5 patients, respectively. Significant proteinuria (> 3 g / day) was present in 2 patients and microscopic hematuria in 18 patients. All patients presented with renal insufficiency (creatinine 3.1 +/- 2.1 mg / dL). The level of von Willebrand factor: antigen (vWF: Ag) in patients was not significantly higher than that in the healthy subjects, while the ADAMTS13 (a disintegrin and metalloprotease, with thrombospondin-1-like domains) activity was not significantl y lower than that in the healthy subjects. Renal histology showed a TMA involved preglomerular arterioles and / or interlobular arteries character-ized by fibrin deposits and vascular wall sclerosis. Fibrin glomerular microthrombi were not observed in these patients. Four patients required hemodialysis upon admission for severe acute renal failure. One follow-up, 3 patients had recovered normal renal function and 14 had mild renal insufficiency (creatinine 1.8 +/- 0.3 mg / dL), while 4 patients still required persistent he modification in. conclusion, compared with patients having hemolytic uremic syndrome / thrombocytopenic purpura, our patients showed a low incidence of throm-bocytopenia and better renal outcome.