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Amigo M.-C.,Medical Center | Goycochea-Robles M.V.,Hospital General Regional 1 | Medina G.,Centro Medico Nacional la Raza | Barragan-Garfias J.A.,Hospital General Of Zona 48 | And 2 more authors.
Lupus | Year: 2015

Introduction: In antiphospholipid syndrome (APS), certain principal manifestations are associated with a worse prognosis and organ damage. Objective: The objective of this paper is to describe the development and initial content, criterion and construct validity of a disease-specific cumulative damage index in patients with thrombotic APS (DIAPS). Methods: Through expert panel agreement, 47 items were considered to reflect damage in APS. This preliminary version of the DIAPS was submitted to four local and international clinical and research experts in APS who ranked each item according to severity. A Delphi exercise resulted in a final 37 item instrument. In the second phase, a cross-sectional study was conducted applying the DIAPS in patients included in a multicenter electronic registry of patients with APS. Quality of life related to health status was evaluated with the EuroQol for construct validation. An a Cronbach and correlation with the EuroQol scale were calculated with SPSS 20.0 (p<0.05). Results: We evaluated the DIAPS in 156 patients, 77% female, with a mean age at diagnosis 34.7±5.5 years. A total of 69% had primary APS. Common comorbidities included obesity, depression and dyslipidemia. The most frequent manifestations resulting in sequelae were deep venous thrombosis and ischemic stroke. Blindness, retinal occlusive vessel disease, myocardial infarction, cardiac valve requiring replacement, mesenteric thrombosis, and renal insufficiency also occurred. Homogeneity: a Cronbach 0.619. DIAPS items correlated with EuroQol domains with the exception of pulmonary, renal, gastrointestinal, and endocrine systems. Conclusion: This study demonstrates content, criterion and construct validity of a new physician-reported instrument to assess the DIAPS. In addition, the DIAPS correlated with the EuroQol. © The Author(s), 2015.


PubMed | Hospital Universitario Clementino Fraga Filho, Nantes University Hospital Center, University of Strasbourg, Detska Fakultna Nemocnica and 31 more.
Type: Journal Article | Journal: Lancet (London, England) | Year: 2016

Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis.We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedmans test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960.Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35.5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0.0228). Median time to clinical remission was 41.9 months in patients assigned prednisone plus methotrexate but was not observable in the other two treatment groups (2.45 fold [95% CI 1.2-5.0] increase with prednisone plus methotrexate; p=0.012). Median time to treatment failure was 16.7 months in patients allocated prednisone, 53.3 months in those assigned prednisone plus ciclosporin, but was not observable in patients randomised to prednisone plus methotrexate (1.95 fold [95% CI 1.20-3.15] increase with prednisone; p=0.009). Median time to prednisone discontinuation was 35.8 months with prednisone alone compared with 29.4-29.7 months in the combination groups (p=0.002). A significantly greater proportion of patients assigned prednisone plus ciclosporin had adverse events, affecting the skin and subcutaneous tissues, gastrointestinal system, and general disorders. Infections and infestations were significantly increased in patients assigned prednisone plus ciclosporin and prednisone plus methotrexate. No patients died during the study.Combined treatment with prednisone and either ciclosporin or methotrexate was more effective than prednisone alone. The safety profile and steroid-sparing effect favoured the combination of prednisone plus methotrexate.Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).


Santos-Franco J.A.,Centro Medico Nacional La Raza
Revista médica del Instituto Mexicano del Seguro Social | Year: 2013

epidermoid neoplasm (EN) accounts for 1 % of whole intracranial neoplasms. Usually, it is found at the cerebello-pontine angle and the location in the fourth ventricle (FV) is rare. The aim was to report two cases of EN of the FV. case 1: a female 22 year old presented with an intense headache with a history of 3 months. At the hospital entry, symptoms and signs of high intracranial pressure were found. Tomography images showed hydrocephalus with high pressure in the FV. She was treated with a shunt from ventricular to peritoneal cavity. After that an encapsulated neoplasm was drawn. It had a pearled aspect. The histology report showed an EN originating in the FV. Case 2: a female 44 year old with a history of five years of dizziness; three years before admission she presented intermittent diplopia and disophagia. At the hospital admission the patient presented paresis of the 6th and 7th cranial nerve. The tomography and the magnetic resonance studies showed a mass in the FV. The neoplasm was extirpated. the EN of the FV is an infrequent benign lesion. Magnetic resonance is the standard diagnostic study, but it could lead to confusion with neurocisticercosis. The extirpation and the treatment of the hydrocephalus are indicated.


to determine the relation between IL6, IL10 and TNFa serum levels in a cohort of patients with type 2 diabetes (T2D) and severe soft tissue infections (STI), with severity and mortality factors. A. comparative and transversal, study with 15 adult patients, any gender, with T2D and STI were done. A T2D control group of 20 patients without STI was included. Apache II Score, glycemia and by ELISA, IL6, IL10 and TNFa, were determined. in all patients, it was a correlation at beginning between glycemia and IL6 (r = 0.67, IC 95 % 0.24-0.88), as soon as glycemia and Apache II, (r = 0.59, IC 95 % 0.11-0.83). although IL6 was very usefulness, it is not a routine test in clinical laboratory and it is expensive, but in medical practice, it could be possible to evaluate these patients with Apache II Score and glycemia. However, in STI, the values of IL6 and IL10 were highly significant. It is likely that IL6 is a marker of poor outcome.


Benitez-Arvizu G.,Centro Medico Nacional La Raza
Revista médica del Instituto Mexicano del Seguro Social | Year: 2010

To preserve the umbilical cord blood (UCB), the volume containing the cells must be reduced before freezing, but the quality, quantity and functionality of the cells has to be preserved during this procedure. The aim is to compare the performance of two different techniques for volume reduction. A semiautomatic system and an automatic system were compared as two different UCB volume reduction techniques. Total nucleated cell (TNC) counts and viability were measured before and after volume reduction. The CD34+ cell counts also were measured. Seventy units of cord blood cells (UCB) were collected. Thirty-three volume reduction procedures were performed by semiautomatic system and thirty-seven by automatic system. The volume recovered and the CD34+ count in both techniques was similar, although the viability differed slightly (1% higher by Optipress II). The semiautomatic and automatic techniques are suitable to reduce volumes of UCB units.


Pineda-Galindo L.F.,Centro Medico Nacional la Raza | Moranchel-Garcia L.,Centro Medico Nacional la Raza
Medicina Interna de Mexico | Year: 2015

Mucopolysaccharidosis type I (MPS I) is the prototype lysosomal storage disease. It is characterized by a deficiency of the enzyme a-L-iduronidase, resulting in the accumulation of glycosaminoglycans in different tissues and organs with varying severity and three clinical presentations according to severiry. We report the case of a 19-year-old male patient with a confirmed diagnosis of MPS I and enzymatic treatment with a favorable clinical response. The objective of this report is to describe the clinical picture, course and treatment of MPS I, and the role of the internist in disease monitoring and management of complications.


BACKGROUND: In diabetic patients with chronic renal failure (CRF) treated with dialysis, the diastolic and systolic left ventricular dysfunction is frequent. The aim was to assess by echocardiography the prevalence of diastolic and systolic ventricular dysfunction in diabetic patients with CRF treated with continuous ambulatory peritoneal dialysis (CAPD). METHODS: Sixty diabetic patients with CRF in CAPD were studied. The mean age was 54.5 +/- 12 years (27-78 years). The left ventricular filling pattern (LVFP) as a diastolic function parameter and left ventricular ejection fraction (LVEF) as a systolic function parameter were measured by transthoracic echocardiography. Descriptive statistical analysis was used. RESULTS: 27 (45 %) patients were women and 33 (55 %) were men. In 55 (91.7 %) left ventricular concentric hypertrophy was observed. Fifty-two patients (86.7 %) showed LVFP type I; three (5 %) had the type II; two (3.3 %) showed pseudonormal pattern and three (5 %) had a normal LVFP. The LVEF was 0.63 +/- 0.09 (CI = 0.41-0.82). Forty nine (81.7 %) patients had LVEF equal or greater than 0.55. CONCLUSIONS: The prevalence of diastolic left ventricular dysfunction was 95 % and the prevalence of systolic left ventricular dysfunction was 18.3%.


Pulido-Diaz N.,Centro Medico Nacional La Raza
Revista médica del Instituto Mexicano del Seguro Social | Year: 2012

Toxic epidermal necrolysis and Stevens Johnson syndrome are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. We report a case of 43 year old female with 75 % body surface area affected, treated with intravenous immunoglobulin who presented favorable outcomes.


Garcia-Chavez J.,Centro Medico Nacional La Raza
Revista médica del Instituto Mexicano del Seguro Social | Year: 2011

We can estimated the probability of developing thromboembolic disease and consequently prescribed antithrombotic measures to patients undergoing surgery. The anticoagulants carry the risk of abnormal bleeding, which can sometimes be fatal. Recently, this concept changed with the development of new drugs that retain their antithrombotic activity but decrease their anticoagulant effect; other advantages are: route of administration, predictable bioavailability (generally do not require monitoring), and little interaction with food and other drugs. The most representative are direct factor Xa inhibitors like apixaban and rivaroxaban as well as factor IIa inhibitors, such as dabigatran. They had been evaluated in patients undergoing hip or knee surgery in comparison with low molecular weight heparins; in general they had better results in efficacy and similar results in safety. Trials are now underway to evaluate their use in other surgical and nonsurgical environments. Today, the surgical patient is older and has comorbidities, such as atrial fibrillation, prosthetic valves, diabetes mellitus, hypertension, and other chronic diseases. These patients need be protected from thrombosis with low bleeding risk. New antithrombotic drugs offer a margin of safety and maintain efficacy; therefore, they constitute an advantage over classical anticoagulant drugs. We highlight concepts related with the need for thromboprophylaxis and the new antithrombotic medication in a surgical context.


Franco-Ornelas S.,Centro Medico Nacional La Raza
Revista médica del Instituto Mexicano del Seguro Social | Year: 2010

The lysosomal storage diseases (LSD) are a group of entities with a meaningful organic affectation profile and important morbidity-mortality rates, which considerably affect the patients' quality of life. At present, new LSD are regularly described because their physiopathological mechanism is recognized and they are susceptible to be treated with enzyme replacement therapy. During 2009, a cross-disciplinary group of Mexican experts on the Gaucher's disease gathered to develop diagnosis and treatment guidelines. This document presents the approach and recommendations of Mexican experts, according to the demography, resources, and epidemiologic reality in Mexico, a country with over 100 million inhabitants.

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