Journal of nephrology
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Journal of nephrology · Nov 1998
ReviewPathophysiology and management of thrombotic microangiopathies.
Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are syndromes of microangiopathic hemolytic anemia, and thrombocytopenia in which endothelial dysfunction appears to be an important factor in the sequence of events leading to microvascular thrombosis. They are termed thrombotic microangiopathies (TMA). Differentiation of the several primary forms of TMA is crucial to predict disease outcome and to establish the most appropriate therapeutic approach. ⋯ The outcome is usually poor. In summary, a general consensus has been achieved that therapies (i.e. plasma exchange or infusion) aimed at stopping the microangiopathic process should always be tried in TTP and in adult and/or atypical forms of HUS to minimize the risk of death or long-term sequela. This approach is seldom effective in secondary forms whose outcome mainly depends on the prognosis of the underlying condition, and is not risk-effective in typical childhood HUS, that usually recovers spontaneously.
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Journal of nephrology · Mar 1998
ReviewGenotype-phenotype correlations in normotensive patients with primary renal tubular hypokalemic metabolic alkalosis.
Among the different forms of hereditary renal tubulopathies associated with hypokalemia, metabolic alkalosis and normotension, two main types of disorders have been identified: Gitelman disease, which appears to be a homogeneous post-Henle's loop disorder, and Bartter syndrome, a heterogeneous Henle loop disorder. A specific gene has been found responsible for Gitelman disease, encoding the thiazide-sensitive Na-Cl cotransporter (TSC) of the distal convoluted tubule. From a phenotypic point of view the characteristic findings of this disease are hypocalciuria, hypomagnesemia and tetanic crises appearing during childhood or later. ⋯ However, these three genes do not explain all the patients with Bartter syndrome which unlike Gitelman disease, appears to be a very heterogeneous disorder. Clearance studies, especially if done during furosemide and/or hydrochlorothiazide administration, have been helpful in identifying the site of tubular involvement. Considering both phenotypic and genotypic data, we propose a clinical-pathophysiological and molecular approach to diagnose the different tubulopathies associated with hypokalemic metabolic alkalosis.
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Journal of nephrology · Nov 1997
Comparative StudyCannulation of the internal jugular vein: comparison of the classic Seldinger technique and an ultrasound guided method.
Central venous catheterization allows immediate and easy vascular access for hemodialysis. Accidental arterial puncture is the most frequent complication of central vein cannulation and may occur in up to 8% of cases with the classic Seldinger procedure. We compared the Seldinger technique which implies manual localization of the vascular access, and an ultrasound guided technique, to assess whether the latter is an improvement on the Seldinger procedure.