Disfunción tiroidea y lipídica asociada a bexaroteno en el linfoma cutáneo de células T / Thyroid and lipidic dysfunction associated with bexarotene in cutaneous T-cell lymphoma

Fundamento y objetivo: El bexaroteno es un rexinoide sintético selectivo del receptor X aprobado para el tratamiento sistémico del linfoma cutáneo de células T. Durante el tratamiento es muy frecuente la aparición de hipotiroidismo e hiperlipidemia mixta grave, siendo ambos efectos adversos reversibles y dependientes de la dosis. La elevación del colesterol LDL y los triglicéridos (hasta unos niveles aumentados que incluso se han asociado a pancreatitis en algunos casos) está ampliamente descrita (al igual que sucede con otros retinoides), siendo el descenso del colesterol HDL menos conocido. Revisamos nuestra experiencia con el uso de bexaroteno. Material y métodos: Se presenta una serie de 3 casos de pacientes tratados con bexaroteno en los que, además de sufrir los efectos adversos bien conocidos, se observó un grave descenso del colesterol HDL. Resultados: Los 3 pacientes estudiados presentaron complicaciones metabólicas en forma de hipotiroidismo central e hiperlipidemia mixta grave, con especial énfasis en el marcado descenso que experimentaron nuestros pacientes (descenso medio > 83%) en las cifras de colesterol HDL. El tratamiento hipolipidemiante y hormonal sustitutivo con levotiroxina dio lugar a una mejoría de los parámetros, sin llegar a alcanzarse los objetivos. Conclusiones: El bexaroteno produce, como efectos secundarios predecibles, una hiperlipidemia mixta grave con descenso marcado de los niveles de colesterol HDL e hipotiroidismo central, los cuales son reversibles y dependientes de la dosis. Se incluye una reflexión sobre los posibles mecanismos etológicos e implicaciones de este fenómeno (AU)

Background and objective: Bexarotene is a synthetic selective X receptor rexinoide approved for the systemic treatment of cutaneous T-cell lymphoma. During treatment is very frequent the occurrence of hypothyroidism and severe mixed hyperlipidemia, both are reversibles and dose-dependent adverse events. Increase of triglycerides and LDL-cholesterol level (up to even higher levels have been associated with pancreatitis in some cases) is widely described (as is the case with other retinoids) but decrease in HDL-cholesterol is poored know. We review our experience with the use of bexarotene. Material and methods: We present a serie of 3 clinical report of patients treated with bexarotene in whose, in addition to these well-known adverse event, a serious lowering of HDL-cholesterol was observed. Results: The 3 patients studied had metabolic complications like central hypothyroidism and severe mixed hyperlipidemia; with special emphasis on the sharp fall (mean decrease > 83%) in the HDL-cholesterol level. Cholesterol lowering medication and substitutive hormonal replacement with levotiroxine resulted in an improvement of the biochimical parameters without reaching the correct goals. Conclusions: Bexarotene produce as predictable side effects severe mixed hyperlipidemia with marked decrease in HDL-cholesterol levels and central hypothyroidism, being the both reversible and dose-dependent. A reflection on the possible aetiological mechanisms and implications of this phenomenon are included (AU)

[Thyroid and lipidic dysfunction associated with bexarotene in cutaneous T-cell lymphoma]. / Disfunción tiroidea y lipídica asociada a bexaroteno en el linfoma cutáneo de células T.

BACKGROUND AND OBJECTIVE: Bexarotene is a synthetic selective X receptor rexinoide approved for the systemic treatment of cutaneous T-cell lymphoma. During treatment is very frequent the occurrence of hypothyroidism and severe mixed hyperlipidemia, both are reversibles and dose-dependent adverse events. Increase of triglycerides and LDL-cholesterol level (up to even higher levels have been associated with pancreatitis in some cases) is widely described (as is the case with other retinoids) but decrease in HDL-cholesterol is poored know. We review our experience with the use of bexarotene. MATERIAL AND METHODS: We present a serie of 3 clinical report of patients treated with bexarotene in whose, in addition to these well-known adverse event, a serious lowering of HDL-cholesterol was observed. RESULTS: The 3 patients studied had metabolic complications like central hypothyroidism and severe mixed hyperlipidemia; with special emphasis on the sharp fall (mean decrease>83%) in the HDL-cholesterol level. Cholesterol lowering medication and substitutive hormonal replacement with levotiroxine resulted in an improvement of the biochimical parameters without reaching the correct goals. CONCLUSIONS: Bexarotene produce as predictable side effects severe mixed hyperlipidemia with marked decrease in HDL-cholesterol levels and central hypothyroidism, being the both reversible and dose-dependent. A reflection on the possible aetiological mechanisms and implications of this phenomenon are included.

Abnormal levels of antioxidant defenses in a large sample of patients with hypertensive disorders of pregnancy.

Increased levels of oxidative stress have been demonstrated in Preeclampsia in previous studies, but this finding has not been established in other hypertensive disorders in pregnancy (HDP). We measured different markers of lipid peroxidation and antioxidant defenses by spectrophotometry or enzymoimmunoanalysis in 339 pregnant women: 85 with gestational hypertension (GH), 88 chronic hypertension (CH), 104 Preeclampsia and 62 healthy pregnant control women (PCW). Lower activity of superoxide dismutase and higher levels of catalase were found in GH, CH and preeclampsia compared with PCW (964.4±116.5, 970.0±120.4, 971.2±137.5 and 1063.4±133.7 U g(-1) Hb, P<0.001; and 313.0±71.7, 292.2±45.3, 297.1±47.2, 215.5±26.2 U mg(-1) Hb, P<0.0001; respectively). Regarding the glutathione REDOX cycle, we found the following in GH, CH and preeclampsia compared with PCW: a decrease in its reduced form (2.6±0.6, 2.7±0.8, 2.7±0.9, 3.3±1.3 µmol l(-1), P<0.003), a parallel increase in the oxidized form (185.6±68.9, 194.7±75.0, 184.3±78.3, 85.1±27.5 µmol l(-1), P<0.0001) and an increment in glutathione peroxidase (85.9±22.0, 86.4±20.9, 82.1±23.5 and 77.2±19.7 U g(-1) Hb, P<0.04) and glutathione reductase (6384.3±1261.9, 6724.6±1154.1, 6287.9±1399.9 and 6044.4±1208.4 mU g(-1) Hb, P<0.01, respectively). Nitrites/nitrates were higher in patients with preeclampsia than in PCW (31.50±15.08, 26.80±8.39 µmol l(-1), P<0.002). Although malondialdehyde and oxidized-LDL levels were similar among groups, free fatty acids were increased in every HDP (GH 514.6±194.6, CH 501.3±197.4, preeclampsia 555.2±230.1 µmol l(-1)) compared with PCW (351.4±146.1 µmol l(-1)), P<0.0001. Our results show an oxidation/reduction imbalance with an increase in oxidative stress coupled with a decreased capacity of antioxidant systems, not only in preeclampsia but also in every HDP.

[Clinical and biological characteristics of a group of 54 pregnant women with HELLP syndrome]. / Características clinicobiológicas de un grupo de 54 gestantes con síndrome HELLP.

BACKGROUND AND OBJECTIVE: The HELLP syndrome is a rare form of preeclampsia with a variable presentation with substantial maternal and perinatal morbidity and mortality. The aim of this study was to determine its clinical and biological characteristics in our hospital. PATIENTS AND METHOD: An observational study of all cases of confirmed HELLP syndrome from 1999 to 2002 was carried out. RESULTS: There were 54 cases of HELLP syndrome. Mean maternal age was 30.06 (16-41) years. 57.4% were primiparous. Mean gestational age at diagnosis of HELLP syndrome was 31.75 (20-41) weeks. Symptoms and signs were dominated by the digestive ones. Hypertension was observed in all cases. 77.8% pregnancies were delivered by caesarean section. Mean birth weight was 1674.52 g (150-3800 g). There were 12 perinatal deaths and one maternal death. CONCLUSIONS: HELLP syndrome is an uncommon but potentially serious complication of pregnancy which is associated with an increased risk of adverse maternal and fetal outcomes. Management should be multidisciplinary and based on strict control by maternal and fetal symptoms.

Características clinicobiológicas de un grupo de 54 gestantes con síndrome HELLP / Clinical and biological characteristics of a group of 54 pregnant women with HELLP syndrome

FUNDAMENTO Y OBJETIVO: El síndrome HELLP es una forma rara de preeclampsia de presentación variable que asocia un incremento de la morbilidad y mortalidad materna y perinatal. El objetivo de este estudio fue determinar las características clínicas y biológicas de los casos asistidos en nuestro hospital. PACIENTES Y MÉTODO: Estudio observacional de todas las pacientes con síndrome HELLP atendidas en un solo centro entre 1999 y 2002. RESULTADOS: Hubo 54 casos de síndrome HELLP. La edad materna fue de 30,06 años (extremos, 16-41) y el 57,4 por ciento eran primíparas. La edad gestacional al diagnóstico fue de 31,75 semanas (extremos, 20-41). Los síntomas y signos fueron predominantemente digestivos. La presencia de hipertensión fue común en todos los casos. En el 77,8 por ciento de los casos fue necesario practicar una cesárea. El peso medio de los recién nacidos fue de 1.674,52 g (150-3.800 g). Hubo 12 muertes perinatales y una muerte materna. CONCLUSIONES: Este síndrome es una complicación rara pero potencialmente grave del embarazo que asocia un riesgo aumentado de problemas maternos y fetales. Por consiguiente, su manejo debe ser multidisciplinario y basado en un control estricto de la clínica materna y fetal (AU)

Enteropatía proteinorreica asociada a cirugía de Fontan. Presentación de un caso / Protein losing enteropathy in association with Fontan operation. Report of a case

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[Glucose effectiveness and components of the metabolic syndrome in recently diagnosed hypertensive patients]. / Efectividad de la glucosa y componentes del síndrome metabólico en pacientes con hipertensión arterial esencial de diagnóstico reciente.

BACKGROUND: Glucose effectiveness (SG) is a parameter that indicates the glucose ability to clearing itself from the plasma independently of insulin's action. Our purpose was to analyze the cluster characteristics associated with the metabolic syndrome in a group of non-obese, recent-onset hypertensives and to test if there was a correlation with SG and the effectiveness of glucose at basal insulin point (GEZI). PATIENTS AND METHOD: We studied 36 patients with mild hypertension with normal basal glucose levels. We determined plasma lipid subfractions, apolipoproteins and urate levels. An intravenous glucose tolerance test (TTGI) and minimal model analysis according to Bergman was performed and SG, GEZI and insulin sensitivity (SI) were calculated. RESULTS: Patients with lower SG and GEZI had higher levels of total triglycerides (Tg) (r = 0.42; p = 0.01 and r = 0.48; p = 0.002, respectively) and triglycerides bind to VLDL (Tg-VLDL) (r = 0.40; p < 0.01 and r = 0.49; p = 0.002, respectively). When the cluster of metabolic syndrome was analyzed, SG was inversely related to uric acid levels and to the waist-hip index. However, SI was only related to the uric acid levels (r = 0.38; p = 0.01). CONCLUSIONS: In non-obese, recently diagnosed hypertensive patients, the SG parameter seems to be an early marker for the development of metabolic syndrome.