Herlyn–Werner–Wünderlich syndrome: A case series / Síndrome de Herlyn-Werner-Wünderlich: una serie de casos

Introduction: Herlyn–Werner–Wünderlich syndrome is a uterine malformation characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Clinical findings: The manifestation of the disease is widely diverse; it is usually diagnosed after menarche, with dysmenorrhea and abnormal uterine bleeding; it is also associated with infertility. Main diagnosis: Four clinical cases, their diagnosis are reported here. Therapeutic interventions and results: The treatment and results of these four patients are described here. Conclusion: When studying uterine malformation it is important to consider this rare disease to avoid possible complications and giving the patient a correct diagnose and treatment. The hysteroscopy resection of the longitudinal vaginal septum in those symptomatic patients with hematocolpos should be considered as a good option for treatment.(AU)

Introducción: El síndrome de Herlyn-Werner-Wünderlich es una malformación uterina que asocia útero didelfo, hemivagina obstruida total o parcialmente y agenesia renal ipsilateral. Hallazgos clínicos: La clínica que presenta este síndrome es muy diversa; se suele diagnosticar después de la menarquia cursando con dismenorrea y sangrado uterino anómalo; así mismo se asocia a infertilidad. Diagnósticos principales: Se presentan a continuación 4 casos clínicos, su diagnóstico y tratamiento mediante diversas técnicas. Intervenciones terapéuticas y resultados: Se describen en este manuscrito los tratamientos aplicados a estas pacientes y sus resultados. Conclusión: Ante el hallazgo de una malformación uterina es importante tener en cuenta esta entidad infrecuente, para evitar posibles complicaciones y proporcionar a la paciente un diagnóstico y tratamiento correctos. La resección histeroscópica del tabique vaginal longitudinal en aquellas pacientes sintomáticas con hematocolpos debe ser considerada como una buena opción de tratamiento.(AU)

Searching for a prognostic index in lupus nephritis.

BACKGROUND: Currently we do not have an ideal biomarker in lupus nephritis (LN) that should help us to identify those patients with SLE at risk of developing LN or to determine those patients at risk of renal progression. We aimed to evaluate the development of a prognostic index for LN, through the evaluation of clinical, analytical and histological factors used in a cohort of lupus. We have proposed to determine which factors, 6 months after the diagnosis of LN, could help us to define which patients will have a worse evolution of the disease and may be, more aggressive treatment and closer follow-up. METHODS: A retrospective study to identify prognostic factors was carried out. We have included patients over 18 years of age with a clinical diagnosis of systemic lupus erythematosus (SLE) and kidney involvement confirmed by biopsy, who are followed up in our centre during the last 20 years. A multi-step statistical approach will be used in order to obtain a limited set of parameters, optimally selected and weighted, that show a satisfactory ability to discriminate between patients with different levels of prognosis. RESULTS: We analysed 92 patients with LN, although only 73 have been able to be classified according to whether or not they have presented poor renal evolution. The age of onset (44 vs. 32; p = 0.024), the value of serum creatinine (1.41 vs. 1.04; p = 0.041), greater frequency of thrombocytopenia (30 vs. 7%; p = 0.038), higher score in the renal chronicity index (2.47 vs. 1.04; p = 0.015), proliferative histological type (100%) and higher frequency of interstitial fibrosis (67 vs. 32%; p = 0.017) and tubular atrophy (67 vs. 32%; p = 0.018) was observed between two groups. The multivariate analysis allowed us to select the best predictive model for poor outcome at 6 months based on different adjustment and discrimination parameters. CONCLUSION: We have developed a prognostic index of poor renal evolution in patients with LN that combines demographic, clinical, analytical and histopathological factors, easy to use in routine clinical practice and that could be an effective tool in the early detection and management.

Distribution patterns, carbon sources and niche partitioning in cave shrimps (Atyidae: Typhlatya).

Cave shrimps of the Typhlatya genus are common and widespread in fresh, brackish and marine groundwater throughout the Yucatan Peninsula (Mexico). These species are ideal models to test niche partitioning within sympatric species in oligotrophic systems. Nevertheless, their food sources remain unidentified, and despite their frequency and functional importance, distribution and abundance patterns of these species within caves have not been fully recognized. Here, we describe the abundance of three Typhlatya species in different temporal and spatial scales, investigate changes in water conditions, and potential sources of carbon as an indication of food origin. Species composition and abundance varied markedly in space and time revealing patterns that differed from one system to another and in relation to environmental parameters. Isotope analysis showed that each species reflects a particular δ13C and Δ14C fingerprint, suggesting they feed in different proportions from the available carbon sources. Overall, our findings suggest a niche partitioning of habitat and feeding sources amongst the three Typhlatya species investigated, where environmental characteristics and physiological differences could play an important role governing their distribution patterns.

FAK phosphorylation plays a central role in thrombin-induced RPE cell migration.

The migration of retinal pigment epithelial (RPE) cells is an important step in various pathologic conditions including subretinal neovascularization (SRN), proliferative vitreoretinopathy (PVR) and, importantly, as a consequence of retinal surgery. Therefore, the elucidation of the mechanisms underlying RPE trans-differentiation and migration is essential for devising effective treatments aimed to the prevention of these disorders. A common event in these pathologies is the alteration of the blood-retina barrier (BRB), which allows the interaction of RPE cells with thrombin, a pro-inflammatory protease contained in serum. Our previous work has demonstrated that thrombin induces RPE cell cytoskeletal remodeling and migration, hallmark processes in the development of PVR; however, the molecular mechanisms involved are still unclear. Cell migration requires the disassembly of focal adhesions induced by Focal Adhesion Kinase (FAK) phosphorylation, together with the formation of actin stress fibers. The aim of the present work was to identify thrombin-activated signaling pathways leading to FAK phosphorylation and to determine FAK participation in thrombin-induced RPE cell migration. Results demonstrate that the activation of PAR1 by thrombin induces FAK autophosphorylation at Y397 and the subsequent phosphorylation of Y576/577 within the activation loop. FAK phosphorylation was shown to be under the control of c/nPKC and PI3K/PKC-ζ, as well as by Rho/ROCK, since the inhibition of these pathways prevented thrombin-induced FAK phosphorylation and the consequent disassembly of focal adhesions, in parallel to FAK-dependent actin stress fiber formation and RPE cell migration. These findings demonstrate, for the first time, that thrombin stimulation of RPE cell transformation and migration are regulated by FAK tyrosine phosphorylation. Thus, targeting FAK phosphorylation may provide a strategical basis for PVR treatment.

Inflamación y síndrome metabólico por resistencia a la insulina-leptina en pacientes con psoriasis / Inflammation and metabolic syndrome due to insulin-leptin resistance in psoriatic patients

Resumen La psoriasis es una enfermedad inflamatoria crónica que se distingue por hiperproliferación recidivante de la piel, de causa y patogénesis multifactoriales. Afecta aproximadamente a 1-3% de la población en general. Durante los últimos diez años, en diversos estudios se ha encontrado que los pacientes con psoriasis tienen prevalencia elevada de factores de riesgo cardiovascular. Asimismo, esos estudios también sugieren una relación entre el síndrome metabólico y la psoriasis. El síndrome metabólico comprende una serie de condiciones fisiopatológicas relacionadas principalmente con aspectos metabólicos, como la resistencia y señalización a la insulina y leptina (Sx Met_RI-Lep). Es un grupo de factores de riesgo que incluye obesidad central, dislipidemia aterogénica, hipertensión arterial sistémica e intolerancia a la glucosa. Su importancia se debe a que duplica el riesgo de enfermedad cardiovascular y diabetes mellitus tipo 2, y aumenta la mortalidad incluso más que sus componentes por separado. La prevalencia mundial del síndrome varía en función del país y de los criterios utilizados para medirla; varía entre 15 y 47% de la población general. Las prevalencias publicadas también varían de acuerdo con las organizaciones que las emiten; tal es el caso de los criterios de la Organización Mundial de la Salud (OMS), que indican que la prevalencia en México es de 14%, y al aplicar los criterios ATP-III asciende a 27%. Existen, por tanto, 6.7 y 14.3 millones de mexicanos afectados, según los criterios de la Organización Mundial de la Salud y los ATP-III, respectivamente. La asociación de la psoriasis con otras enfermedades sistémicas podría deberse a diversas causas, como predisposición genética, factores ambientales (tabaco, alcohol, vida sedentaria) o tratamientos sistémicos prescritos. Cada vez son más los estudios que relacionan el síndrome metabólico por resistencia a la insulina-leptina con la psoriasis. En esta revisión bibliográfica se pretende sintetizar los mecanismos fisiopatológicos compartidos por ambos padecimientos, así como insistir en el cribado de los factores de riesgo cardiovasculares y del síndrome metabólico para prevenir o mejorar el tratamiento y pronóstico de los pacientes con psoriasis.

Abstract Psoriasis is a chronic inflammatory disease characterized by relapsing skin hyperproliferation, with multifactorial pathogenesis and causes. It affects approximately 1% to 3% of the general population. During the past ten years, several studies have found that patients with psoriasis have a high prevalence of cardiovascular risk factors. Likewise, such studies also suggest a link between metabolic syndrome and psoriasis. Metabolic syndrome comprises a number of pathophysiologic conditions that mainly involves the metabolic aspects concerning insulin-leptin resistance and signaling (Sx Met_RI-Lep). It is a group of risk factors including central obesity, atherogenic dyslipidemia, systemic hypertension and glucose intolerance. Its importance is due to its presence doubles the risk of cardiovascular disease, type 2 diabetes mellitus and increases mortality higher than its separate components. The worldwide prevalence of the syndrome varies depending on the country and the criteria used; between 15% and 47% of the general population. The prevalence published also vary according to the organizations that issue; such is the case of the criteria of the World Health Organization (WHO) to handle a prevalence in Mexico of 14% and applying the criteria ATP-III rises to 27%, so there are 6.7 and 14.3 million Mexicans affected, according to the criteria of the World Health Organization and the ATP-III, respectively. The association of psoriasis with other systemic diseases may be due to various causes such as genetic predisposition, environmental factors (smoking, alcohol, sedentary lifestyle) or be influenced by systemic treatments used against psoriasis. More and more studies link the Sx Met_RI-Lep with psoriasis. This paper summarizes the pathophysiological mechanisms shared by both pathologies, as well as emphasizes screening for cardiovascular risk factors and metabolic syndrome to improve treatment and prognosis of psoriasis.

Necrosis avascular de la cabeza femoral tras el uso del arnés de Pavlik / Femoral head avascular necrosis after the use of Pavlik harness

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Extended-release tacrolimus therapy in de novo kidney transplant recipients: single-center experience.

BACKGROUND: Available data for extended-release tacrolimus (Tac) except in clinical trials are limited. OBJECTIVE: To describe our initial experience with once-daily Tac in combination with corticosteroids and mycophenolate mofetil therapy in patients undergoing de novo renal transplantation. PATIENTS AND METHODS: In this retrospective, observational, single-center study, data were obtained for 49 adult recipients treated with extended-release Tac and 30 patients treated with standard-release Tac (control group). Mean (SD) follow-up in the 2 groups was 3.5 (2.5) months and 4.0 (2.6) months, respectively. The primary characteristics were comparable between the groups. RESULTS: The acute rejection rate in the extended-release group was 10%, and 13% in the standard-release group. Patient and graft survival rates were 98% and 96% vs 100% and 90%, respectively. Renal function in the 2 groups was comparable: serum creatinine concentration 1.3 (0.2) mg/dL vs 1.45 (0.4) mg/dL. At day 14 posttransplantation, Tac doses were 0.17 mg/kg/d vs 0.14 mg/kg/d, and blood concentrations were 9.0 ng/mL vs 14.0 ng/mL. In recipients older than 60 years, lower dosages of Tac resulted in blood concentrations similar to those in younger patients, with less variation in dosage. CONCLUSIONS: Short-term experience with extended-release Tac therapy in de novo renal recipients confirms its efficacy and safety. Adjusting blood concentrations in the immediate posttransplantation period is less difficult with extended-release Tac compared with the twice-daily formulation.

Enfermedad renal ateroembólica: un análisis de los factores clínicos y terapéuticos que influyen en su evolución / Atheroembolic renal disease: analysis of clinical and therapeutic factors that influence its progression

El embolismo de colesterol es una enfermedad causada por la suelta de cristales de colesterol desde las placas arterioscleróticas ulceradas de la aorta. Esta suelta puede ocurrir de forma espontánea o más frecuentemente tras procedimientos vasculares invasivos o tras tratamientos anticoagulantes o fibrinolíticos. Entre 1989 y 2005, en tres hospitales españoles, se diagnosticaron 45 casos de embolismo renal de colesterol. El diagnóstico fue confirmado mediante biopsia de cualquier órgano afectado o hallazgos típicos en el fondo de ojo. La mayoría de los pacientes eran varones (93,3%), ancianos (el 55,7% era mayor de 70 años), fumadores (91,1%), hipertensos (95,6%) y con varios factores de riesgo cardiovascular. Todos los pacientes presentaron un fracaso renal agudo en el momento del diagnóstico. La creatinina media al inicio fue de 4,3 ± 2,4 mg/dl. El fracaso renal agudo se acompañó frecuentemente de eosinofilia (64,4%) y lesiones cutáneas (57,7%). El 20% de los casos ocurrieron espontáneamente y el 46,7% tras manipulación endovascular (cateterismo/arteriografía); tan sólo un 8,9% ocurrió tras cambios en la anticoagulación. Tras un seguimiento de 12 ± 16,3 meses, el 55,6% (25) de los pacientes requerían diálisis crónica y un 64,4% (29) había fallecido, ocho de ellos tras haber entrado en diálisis crónica. Se observó una recuperación parcial de función renal en 9 pacientes (20%), que presentaban una creatinina media al final del seguimiento de 3 ± 1,7 mg/dl. La comorbilidad cardiovascular y la gravedad clínica del embolismo de colesterol no tuvieron impacto sobre la supervivencia renal o del individuo. La supervivencia renal (Kaplan-Meier) fue mayor en los casos de ateroembolismo espontáneo que en los iatrogénicos. 15 de los 45 pacientes recibieron esteroides. En los tratados se observó una mayor incidencia de fallecimientos (73,3% frente a 60%) y un menor porcentaje de recuperación de función renal (13,3% frente a 23%), aunque sin diferencias estadísticamente significativas. El tiempo medio de evolución a la diálisis fue significativamente más corto entre los tratados con esteroides (p = 0,017). El uso de estatinas no se asoció con una mejoría en el pronóstico renal o vital del individuo. En conclusión, la enfermedad renal ateroembólica constituye un tipo de fracaso renal agudo con unas características clínicas muy determinadas. La supervivencia renal y del paciente es mala, pero existe un porcentaje significativo de recuperaciones espontáneas de la función renal. La supervivencia renal fue significativamente mejor en los casos espontáneos y no observamos efectos beneficiosos del tratamiento esteroideo (AU)

Cholesterol embolism is a disease caused by distal showering of cholesterol crystal released from disintegration of arterial atheromatous plaques. It may occur spontaneously or more often after invasive vascular procedures or thrombolytic/anticoagulant agents. Forty five cases were diagnosed between 1989 and 2005 in three Spanish hospitals. The diagnosis was confirmed by histology or diagnostic ophthalmoscopic findings. The majority were male (93.3%), elder (55.5% were older than 70 years), smoker (91.1%), had hypertension (95.6%), with high prevalence of cardiovascular risk factors. At the time of diagnosis all patients presented acute renal failure. Mean serum creatinine at diagnosis was 4.3± 2.4mg/dl. The acute renal failure was accompanied with eosinophilia (64.4%) and cutanous lesions (57.7%). 20% of cases occur spontaneously and 46.7% after endovascular manipulation (coronary angiography/arteriography) and only 8% after changes in anticoagulant treatment. After a follow-up of 12 ± 16.3 months the 55.6% of patients need chronic dialysis, 64.4% died, 8 of them after the beginning of dialysis. Nine patients recovered renal function, with a mean creatinine of 3 ± 1.7 mg/dl at the end of follow-up. The cardiovascular comorbididy and the clinical severity of the embolism don´t have impact in the renal or patient survival. Renal survival (Kaplan-Mier) were better in spontaneous than in iatrogenic cholesterol embolism. Fifteen of 45 patients were treated with steroids. In treated patients we observed a high incidence of death (73.3% versus 60%) and fewer recovery of renal function (13.3% versus 23%), without statistical significance. The mean time to dialysis was shorter in treatment patients (p= 0.017). Statins treatment was not associated with outcome (renal or individual). In summary, atheroembolic renal disease represents an acute renal failure with special characteristics. Renal and individual outcome is poor, but some patients have spontaneous recovery of renal function. Renal survival was significantly better in spontaneous disease. We don´t observe beneficial effect of steroid treatment (AU)

[The antiproteinuric effect of the blockage of the renin-angiotensin-aldosterone system (RAAS) in obese patients. Which treatment option is the most effective? ]. / Efecto antiproteinúrico del bloqueo del sistema renina-angiotensina-aldosterona (SRAA) en los pacientes obesos. ¿Cuál es la opción terapéutica más eficaz?

BACKGROUND: Obesity increases the risk of proteinuria and chronic renal insufficiency and hastens the progression of renal diseases. Increased activity of renin-angiotensin-aldosterone system and elevated levels of aldosterone are common in obese patients. No studies have compared the efficacy of the currently available antiproteinuric strategies (ACE inhibitors -ACEI-, angiotensin receptor blockers -ARB-, aldosterone antagonists) in obese patients with proteinuric renal diseases. METHODS: Single centre, prospective, randomized study. Twelve obese patients (body mass index > 30 Kg/m2) with proteinuria > 0.5 g/24 h were selected from our outpatient renal clinic. Patients were consecutively treated during 6 weeks with an ACEI (lisinopril 20 mg/day), combined therapy ACEI+ARB (lisinopril 10 mg/day + candesartan 16 mg/day) and eplerenone (25 mg/day) in random order. A drug washout period of 6 weeks was established between the different treatment periods. The primary outcome point was the change in 24-h proteinuria at the end of each treatment period and the number of patients showing a proteinuria reduction greater than 25% of baseline. RESULTS: The reduction in proteinuria induced by lisinopril (11.3+/-34.8%) was not statistically significant with respect to baseline, whereas that of lisinopril plus candesartan (26.9+/-30.6%) and eplerenone (28.4+/-31.6%) showed a statistically significant difference both with respect to baseline values and to lisinopril group. The number of patients who showed a greater than 25% proteinuria reduction was significantly higher with eplerenone (67%) and lisinopril+candesartan (67%) than with lisinopril (25%). CONCLUSIONS: Monotherapy with an aldosterone antagonist and combination therapy with ACEI+ARB were more effective than ACEI monotherapy to reduce proteinuria in obese patients with proteinuric renal diseases.

Efecto antiproteinúrico del bloqueo del sistema renina-angiotensina-aldosterona (SRAA) en los pacientes obesos. ¿Cuál es la opción terapéutica más eficaz? / No disponible

Introducción: La obesidad aumenta el riesgo de proteinuria e insuficiencia renal crónica, y acelera la progresión de enferme- dades renales. En los pacientes obesos existe un aumento de la actividad del sistema renina-angiotensina-aldosterona (SRAA) y de los niveles de aldosterona. Ningún estudio ha comparado la eficacia de las diferentes estrategias antiproteinúricas actual- mente disponibles (inhibidores de la enzima convertidora de la angiotensina [IECA], antagonistas de los receptores de la an- giotensina [ARA], antagonistas de la aldosterona) en pacientes obesos con nefropatías proteinúricas. Métodos: Es un estudio prospectivo y aleatorizado, realizado en un único centro. Fue- ron seleccionados doce pacientes obesos (índice de masa cor- poral >30 kg/m 2 ), con proteinuria >0,5 g/24 h, de nuestras con- sultas de Nefrología. Los pacientes fueron tratados consecutivamente durante seis semanas y en orden aleatorio con un IECA (lisinopril 20 mg/día), una terapia combinada con IECA más ARA (lisinopril 10 mg/día más candesartán 16 mg/día) y eplerenona (25 mg/día). Se estableció un período de lavado de seis semanas entre los diferentes períodos de tratamiento. El objetivo principal del estudio fue el cambio en la proteinu- ria de 24 h al final de cada período de tratamiento y el núme- ro de pacientes que mostraban una reducción de la proteinu- ria superior al 25% con respecto al valor basal. Resultados: La reducción de la proteinuria obtenida por lisinopril (11,3 ± 34,8%) no fue estadísticamente significativa con respecto al va- lor basal, mientras que la reducción con lisinopril y candesar- tán (26,9 ± 30,6%) y eplerenona (28,4 ± 31,6%) mostró una di- ferencia estadísticamente significativa frente a sus valores basales (comparación intragrupo) y frente al grupo de lisino- pril (comparación entre grupos). El número de pacientes que mostraron una reducción mayor al 25% de la proteinuria fue significativamente mayor con eplerenona (67%) y lisinopril + candesartán (67%) que con lisinopril (25%). Conclusiones: La monoterapia con antagonistas de la aldosterona (eplerenona) y la terapia de combinación con IECA + ARA fueron más efectivos que los IECA en monoterapia para reducir la proteinuria en pacientes obesos con diferentes tipos de nefropatías crónicas proteinúricas (AU)

Background: Obesity increases the risk of proteinuria and chronic renal insufficiency and hastens the progression of renal diseases. Increased activity of renin-angiotensin-aldosterone system and elevated levels of aldosterone are common in obese patients. No studies have compared the efficacy of the currently available antiproteinuric strategies (ACE inhibitors –ACEI-, angiotensin receptor blockers –ARB-, aldosterone antagonists) in obese patients with proteinuric renal diseases. Methods: Single centre, prospective, randomized study. Twelve obese patients (body mass index >30 Kg/m 2 ) with proteinuria >0.5 g/24 h were selected from our outpatient renal clinic. Patients were consecutively treated during 6 weeks with an ACEI (lisinopril 20 mg/day), combined therapy ACEI + ARB (lisinopril 10 mg/day + candesartán 16 mg/day) and eplerenone (25 mg/day) in random order. A drug washout period of 6 weeks was established between the different treatment periods. The primary outcome point was the change in 24-h proteinuria at the end of each treatment period and the number of patients showing a proteinuria reduction greater than 25% of baseline. Results: The reduction in proteinuria induced by lisinopril (11.3 ± 34.8%) was not statistically significant with respect to baseline, whereas that of lisinopril plus candesartán (26.9 ± 30.6%) and eplerenone (28.4 ± 31.6%) showed a statistically significant difference both with respect to baseline values and to lisinopril group. The number of patients who showed a greater than 25% proteinuria reduction was significantly higher with eplerenone (67%) and lisinopril+candesartán (67%) than with lisinopril (25%). Conclusions: Monotherapy with an aldosterone antagonist and combination therapy with ACEI + ARB were more effective than ACEI monotherapy to reduce proteinuria in obese patients with proteinuric renal diseases (AU)

Human platelet-specific antigens frequencies in the Argentinean population.

The frequencies of several human platelet antigens (HPAs) vary between different populations and are a major determinant for the prevalence of HPA alloimmunization and its clinical associated entities. The aim of this study was to characterize the allele frequencies of seven HPA systems in two different ethnic groups from the Argentinean city of Rosario, the major population and a minority Amerindian group recently arrived from the north of the country, the Tobas. A total of 192 healthy unrelated individuals from blood donors and hospital staff from the Hospital Italiano Garibaldi and 27 unrelated Toba Amerindians were genotyped for HPA-1, -2, -3, -4, -5, -6 and -15 systems by polymerase chain reaction with sequence specific primers (PCR-SSP). The present data showed that the distribution of the HPA alleles among Argentineans from Rosario is quite similar to that reported among Europeans. The frequencies seen in Tobas, although limited by the small number of aboriginal samples studied, are similar to those reported for other Amerindians populations. Statistically significant differences were found for the genotype distribution of HPA-1, -3, -5 and -15 between both groups, indicating important differences in the potential risk of HPA alloimmunization associated to transfusion and pregnancy. The study of these polymorphisms represents the first step in the elucidation of pathological conditions that are underdiagnosed in our population. It allowed us to establish a panel of characterized blood donors necessary for the serological work out and as a source for compatible platelets transfusion.

HNA-1a, HNA-1b and HNA-1c gene frequencies in Argentineans.

Human neutrophil antigens (HNA) are polymorphic structures located in the neutrophil membrane. The neutrophil-specific antigens HNA-1a (NA1), 1b (NA2) and 1c (SH) are well-recognized allotypic forms of FcgammaRIIIb and the most frequent targets of neutrophil alloantibodies. The aim of this study was to determine the gene frequencies of the neutrophil-specific antigens belonging to the HNA-1 system in blood donors and Toba Amerindians from Rosario, Argentina. Two hundred and eighteen unrelated healthy Argentinean blood donors and Toba Amerindians from Rosario were typed for HNA-1a, HNA-1b and HNA-1c using polymerase chain reaction with sequence-specific primers. For the Argentinean blood donors, the HNA-1a and HNA-1b gene frequencies were 0.44 and 0.56 and for the Amerindians Toba were 0.77 and 0.23, respectively. The HNA-1c antigen is present in 4.7% (gene frequency=0.023) of the blood donors but in none of the Amerindian individuals. The present data showed that the HNA-1 allele frequencies in the major population and the Toba Amerindians from Rosario are similar to those described in European and others distant Amerindians populations, respectively.

¿Cuándo y cómo tratar a los pacientes con glomerulonefritis membranosa? / When and how to treat patients with membranous glomerulonephritis?

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Influencia de la prediabetes en el pronóstico cardiovascular del paciente hipertenso / Influence of prediabetes in the cardiovascular prognosis of the hypertensive patient

Las diversas características de los pacientes que presentan un riesgo elevado de desarrollar diabetes mellitus han sido descritas de forma reciente, siendo las principales una concentración de glucosa sérica más elevada, índice de masa corporal aumentado, presión arterial sistólica elevada, una cifra de colesterol HDL bajo y la presencia de tratamiento antihipertensivo previo. Sin embargo, se sabe poco respecto al pronóstico a largo plazo de este grupo de pacientes, también denominados «prediabéticos». El estado prediabético ha sido definido por la presencia de intolerancia hidrocarbonada o glucemia anómala en ayunas. Las evidencias acumuladas sugieren que los individuos con hiperglucemia en rango no diabético (prediabéticos) presentan riesgo aumentado de enfermedades cardiovasculares. Esta revisión analiza la necesidad de reconocer de forma precoz a los pacientes hipertensos prediabéticos para desarrollar estrategias de protección cardiovascular y de esta forma disminuir las consecuencias de la precipitación del desarrollo de diabetes y sus efectos deletéreos cardiovasculares y renales

The different characteristics of patients who have an elevated risk of developing diabetes mellitus have recently been described, the main ones being higher serum glucose concentration, increased body mass index, elevated systolic blood pressure, low HDL-cholesterol value and presence of previous antihypertensive treatment. However, little is known about the long term prognosis of this group of patients, also called "prediabetics". The prediabetic state has been defined by the presence of hydrocarbonate intolerance of abnormal fasting glycemia. The accumulated evidence suggests that individuals with hyperglycemia in the non-diabetic range (prediabetics) have increased risk of cardiovascular diseases. This review analyzes the need to recognize prediabetic hypertensive patients early to develop cardiovascular protection strategies and, in this way, to decrease the consequences of the precipitation of the development of diabetes and its cardiovascular and renal harmful effects

The molecular genetics of Marfan syndrome and related disorders.

Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular systems, is caused by mutations in the gene for fibrillin-1 (FBN1). The leading cause of premature death in untreated individuals with MFS is acute aortic dissection, which often follows a period of progressive dilatation of the ascending aorta. Recent research on the molecular physiology of fibrillin and the pathophysiology of MFS and related disorders has changed our understanding of this disorder by demonstrating changes in growth factor signalling and in matrix-cell interactions. The purpose of this review is to provide a comprehensive overview of recent advances in the molecular biology of fibrillin and fibrillin-rich microfibrils. Mutations in FBN1 and other genes found in MFS and related disorders will be discussed, and novel concepts concerning the complex and multiple mechanisms of the pathogenesis of MFS will be explained.

[Tansitional vesical cell carcinoma metastatizing to the sphenoid sinus]. / Metástasis esfenoidal de un carcinoma de células trancisionales de la vejiga.

Secundary tumors of the paranasal sinus are very uncommon with only one hundred cases reported in the literature up to 2001. The commonest site of the primary tumor is the kidney. The maxillary sinus is most often involved. The Sphenoid sinus is the rarest site. We report a rare case of metastasis to the sphenoid sinces from a transitional cell bladder tumor in a 69-year-old man who died after treatment with chemotherapy and we also review the liteature.

Metástasis esfenoidal de un carcinoma de células trancisionales de la vejiga / Transitional vesical cell carcinoma matastazing to the sphenoid sinus

Las metástasis en los senos paranasales son extremadamente raras. En el año 2001 apenas existían 100 casos publicados en distintas revisiones. Cuando ocurren, el tumor primario asienta con mayor frecuencia en el riñón. El seno maxilar es el más frecuentemente afectado, el esfenoidal el más raro. Presentamos un raro caso de metástasis en el seno esfenoidal de un carcinoma de células trancisionales de la vejiga

Secundary tumors of the paranasal sinus are very uncommon with only one hundred cases reported in the literature up to 2001. The commonest site of the primary tumor is the kidney. The maxillary sinus is most often involved. The Sphenoid sinus is the rarest site. We report a rare case of metastasis to the sphenoid sinces from a transitional cell bladder tumor in a 69 years old men, who died after treatment with chemotherapy and we also review the liteature

[Effect of short-term nonsteroidal antiinflammatory therapy on mean blood pressure. A prospective, randomized clinical trial]. / Efecto del tratamiento a corto plazo con antiinflamatorios no esteroideos en la presión arterial media. Un ensayo clínico aleatorizado.

Our objective was to evaluate the effect of short-term therapy with nonsteroidal antiinflammatory drugs (NSAID) on mean blood pressure (MBP). Two hundred thirty-three consecutive patients (185 women, 54 men; 53.9±13.5 years) requiring NSAID therapy were prospectively randomized to: indomethacin (21 patients), naproxen (33 patients), diclofenac (40 patients), sulindac (42 patients), meloxicam (35 patients) and celecoxib (40 patients) for 8.5±2 days. BP was measured at the beginning and end of follow-up using a Kenz OS22 monitor. At the end of follow-up, 211 (88%) patients were still available for evaluation. A slight posttreatment decrease in MBP (-1.4±9.0 mm Hg, 95%CI: -2.7 to -0.2) was found. No significant differences in posttreatment changes in MBP were found among the distinct NSAIDs evaluated. Fifty-five patients (23%; 95% CI: 18-29) showed a posttreatment increase in MBP of>5 mm Hg. This increase was not associated with hypertension, old age, or any specific NSAID. We conclude that, overall, shortterm NSAID therapy does not seem to have a clinically significant effect on MAP. However, 23% of patients undergoing this therapy showed an increase in MBP that, if persistent, could lead to a higher risk of cardiovascular disease. Further research is needed to identify whether this outcome persists in long-term NSAID therapy.