Severe renal failure and thrombotic microangiopathy induced by malignant hypertension successfully treated with spironolactone.

Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0.4mg/dL, haptoglobin≤10mg/dL, platelet count=59,000/µL and schistocytes on peripheral smear. He required initiation of hemodialysis. Additionally, we considered that the possible cause of TMA was malignant hypertension according to the presence of hypertensive retinopathy and thrombocytopenia which remitted only with blood pressure control, hence, plasmapheresis was not given. Renal function did not improve and the patient remained chronic hemodialysis. Intensive therapy for hypertension with a combination of antihypertensive drugs including spironolactone successfully lowered his blood pressure without developing hyperkalemia.

[Transforming growth factor beta (TGF-beta) in Gaucher's disease. Preliminary results in a group of patients and their carrier and non-carrier relatives]. / Transforming growth factor beta (TGF-beta) en la enfermedad de Gaucher. Resultados preliminares en un grupo de enfermos y familiares portadores y no portadores.

BACKGROUND: The poor phenotype/genotype correlation in Gaucher's disease makes difficult therapy-decision-making and prevention of complications. Gaucher's cells and tissue fibrosis are the earliest findings of the disease. Transforming growth factor ss (TGF-beta1) is the key cytokine involved in the regulation of tissular scarring and fibrosis. The aim of the study was to ascertain if there are differences in plasma TGF-beta1 between Gaucher's disease patients, carriers and non-carriers healthy people and whether there is any correlation between plasma TGF-beta1 and clinical phenotype among patients. PATIENTS AND METHOD: Plasma TGF-beta1 was measured in 11 patients with Gaucher's disease, 12 carriers and 10 healthy people. Patients were further evaluated to know their liver and spleen size, bone involvement, hemoglobin, leukocyte and platelet count and the Zimran's severity score index (SSI). Plasma concentration of TGF-beta1 was determined by RIA phenotypic sandwich antibodies assay and quantified by a colorimetric procedure. Sensitivity was 25 pg/ml and specificity (cross reactivity) < 5% with beta2-TGF and beta3-TGF. STATISTICS: ANOVA and T-test were applied for mean comparisons and subgroup analyses. RESULTS: Plasma TGF-beta1 values were increased in Gaucher's disease patients (98.4 [91.4] pg/ml) over carriers (47.2 [21,7] pg/ml; p = 0.04) and healthy relatives (40.8 [9.8] pg/ml; p = 0.02). No differences in patients subgroups, with regard to SSI or bone involvement, were observed. CONCLUSIONS: Plasma TGF-beta1 levels are increased in this group of patients with Gaucher's disease. Since there is no correlation between the plasma values and the phenotypic expression, TGF-beta1 could merely be a marker of macrophage activation.

[Rate of protein catabolism as an indicator of protein ingestion in uremic patients on hemodialysis]. / Tasa de catabolismo proteico como indicador de la ingesta proteica en pacientes urémicos en hemodiálisis.

Protein catabolic rate (PCR) has been proposed as a indirect index of dietary protein intake, but its sensitivity has been discussed. In a group of 140 chronic uremic patients undergoing maintenance hemodialysis, we evaluated the nutritional status (body mas index: BMI, triceps skinfold thickness: TST, arm muscle circumference: AMC, serum total proteins, albumin and lymphocytes) and its relation with dietary survey and PCR levels. PCR was correlated positively with Kt/V (p: 0.0001, r: 0.45) and with seric albumin (p: 0.01, r: 0.22), whereas dietary protein intake by dietary survey (g/Prot/Kg/day) was correlated strongly with anthropometric measurements like BMI, AMC (p: 0.0001) and less with Kt/V (p: 0.01), but not with serum albumin. PCR was correlated with dietary survey results: g Prot/Kg/day (p: 0.04, r: 0.18) and Kcal/Kg/day (p: 0.03, r:0.2). The results suggest that nutritional parameters with slow evolution as anthropometric measurements could be related with usual dietary intake (dietary survey), whereas serum albumin (that vary early with recent changes of dietary intake) could be related better with a biochemical index like PCR, in these way both determinations are complementary.