Ferrer Castillo M.T.,Divisions of Nephrology |
Jimenez Crespo B.,Divisions of Endocrinology |
Dominguez Quintero M.L.,Juan Ramon Jimenez Hospital |
Gonzalez Fuentes C.,Juan Ramon Jimenez Hospital
Neonatology | Year: 2013
A neonate affected by a novel inactivating mutation in the calcium-sensing receptor (CASR) gene is presented. This mutation is homozygously inherited and has not been previously described. A deletion in exon 5 (c.1392-1404del13) of the gene causes a loss of function of the receptor, which results in neonatal severe hyperparathyroidism and an ensuing extreme hypercalcemia. In a case of homozygosis of the CASR gene, the use of cinacalcet is the second reported calcimimetic treatment attempt and the first treatment attempt prior to surgery. However, because of the type of mutation, parathyroid surgery was necessary at 4 months of age after therapeutic failure. Because there are multiple mutations that affect the CASR gene in different ways, treatment with cinacalcet as an alternative to surgery may be valuable in homozygous cases that are caused by different mutations than the reported case. Copyright © 2013 S. Karger AG, Basel.
De La Portilla F.,University of Seville |
Rada R.,Juan Ramon Jimenez Hospital |
Jimenez-Rodriguez R.,University of Seville |
Diaz-Pavon J.M.,University of Seville |
Sanchez-Gil J.M.,University of Seville
Diseases of the Colon and Rectum | Year: 2011
BACKGROUND: The treatment of anal fistulas using plugs is a very promising method because of its simplicity and ability to be carried out on an ambulatory basis. If unsuccessful, it does not compromise subsequent alternative surgical techniques and/or products. However, success rates are variable. OBJECTIVE: This pilot study was designed to investigate the safety and effectiveness of a new synthetic plug in the treatment of transsphincteric anal fistulas. DESIGN: This was a prospective observational study. SETTING: Patients were treated at 2 colorectal surgery centers in Spain (Seville and Huelva). INTERVENTIONS: Anal fistulas were treated with a fistula plug made of bioabsorbable polymers (67% polyglycolide, 33% trimethylene carbonate). PATIENTS: Starting in January 2009, consecutive adult patients with transsphincteric anal fistulas were evaluated. MAIN OUTCOME MEASURES: Outcomemeasures included rates of successful fistula closure, complications, and continence (Jorge-Wexner incontinence score), assessed postoperatively at 1 week and again at 1, 3, 6, and 12 months. Healing was determined by clinical examination by a surgeon blinded for the intervention. RESULTS: A total of 19 patients (18 men, 1 woman) with transsphincteric anal fistulas were included in the study. The median age was 49 (range, 33- 65) years. Of these patients, 12 presented with fistula relapse. The median time from onset of symptoms to surgery was 12 (range, 6 -120) months. Three patients had previously placed setons. The follow-up duration was 12 months. Relapse occurred in 16 patients (with a perianal abscess in 1), and successful closure was observed in 3 patients (15.8%). LIMITATIONS: The number of patients was small, and time was needed for the learning curve of the technique. CONCLUSIONS: This study indicates that the new synthetic plug is safe, but the fistula closure rate was low. Randomized studies are needed to further determine the role of the bioabsorbable synthetic plug in the management of anal fistulas. © The ASCRS 2011.
Munoz M.,University of Seville |
Gonzalez-Ortega A.,University of Seville |
Salinas-Martin M.V.,Juan Ramon Jimenez Hospital |
Caranza A.,University of Seville |
And 3 more authors.
International Journal of Oncology | Year: 2014
The substance P (SP)/neurokinin (NK)-1 receptor system plays an important role in the development of cancer. No in-depth studies of the involvement of this system in breast cancer (BC) have been carried out, and the action exerted by the drug aprepitant on BC cells is currently unknown. We show the involvement of this system in human BC cell lines: i) these cells express mRNA for the NK-1 receptor; ii) they overexpress NK-1 receptors; iii) the NK-1 receptor is involved in their viability; iv) SP induces their proliferation; v) NK-1 receptor antagonists block SP-induced mitogen stimulation of these cells; vi) the specific antitumor action of such antagonists on these cells occurs through the NK-1 receptor; and vii) BC cell death is due to apoptosis. We also found NK-1 receptors and SP in all human BC samples studied. The NK-1 receptor may be a promising target in the treatment of BC and NK-1 receptor antagonists could be candidates as a new antitumor drug in the treatment of BC.
Ramos-Font C.,Juan Ramon Jimenez Hospital |
Gomez-Rio M.,Virgen Of Las Nieves Hospital |
Rodriguez-Fernandez A.,Virgen Of Las Nieves Hospital |
Jimenez-Heffernan A.,Juan Ramon Jimenez Hospital |
And 2 more authors.
Journal of Surgical Oncology | Year: 2014
Purpose To assess the value of FDG-PET/CT in the evaluation of gallbladder carcinomas (GBC). Methods A prospective cohort of patients with suspicion of or confirmed GBC was studied with FDG-PET/CT. Diagnostic accuracy parameters were calculated in comparison with pathology and/or the clinical course of patients. Clinical impact of PET/CT imaging was estimated. Results Forty-nine patients were enrolled (34 malignant tumors, 15 benign lesions; 37 staging, 12 restaging). Overall diagnostic accuracy was 95.9% for the diagnosis of the primary lesion, 85.7% for lymph node involvement and 95.9% for metastatic disease. Mean SUVmax in malignant gallbladder lesions was 7.92 ± 6.25 Analysis of ROC curves showed a SUVmax cut-off value of 3.62 for malignancy (S: 78.1%; Sp: 88.2%). Diagnostic accuracy in the restaging group reached 100%. FDG-PET/CT changed the management of 22.4% of the population. Comments Diagnosis of malignancy or benignity of suspicious gallbladder lesions is accurately made with FDG PET/CT, allowing a precise staging of GBC due to its ability to identify unsuspected metastatic disease. SUVmax has a complementary role in addition to visual analysis. J. Surg. Oncol. 2014 109:218-224. © 2013 Wiley Periodicals, Inc.
Munoz-Beamud F.,Juan Ramon Jimenez Hospital |
Isenberg D.A.,University College London
Clinical and Experimental Rheumatology | Year: 2013
Objective To assess the efficacy and safety of B-lymphocyte depletion therapy (BCDT) utilising rituximab in refractory idiopathic inflammatory myositis. Methods We retrospectively evaluated 16 adult patients with active dermatomyositis (DM) or polymyositis (PM) who received 1 gram rituximab intravenous infusions two weeks apart after failing to respond to conventional therapy. The clinical and biochemical response were analysed by the Myositis Intention to Treat index (MITAX) and the serum creatine kinase (CK) levels at baseline and 6 and 12 months after treatment. The primary efficacy outcome was 20% improvement in the MITAX index and 30% reduction in CK. Results Eight patients responded to treatment and achieved both the MITAX and CK levels objectives within 6 months of rituximab therapy. Five out of these 8 responders remained clinically stable at 12 months and CK levels were still reduced or normalised. Of note, 4 patients who did not respond were re-assessed and had their diagnoses corrected. All patients showed adequate B cell depletion (BCD) with re-population occurring for a 15.4 months average (range 3-42 months). Those simultaneously treated with cyclophosphamide achieved more long-lasting depletion (average 18.6 months). Conclusion The heterogeneous clinical and serological characteristics of patients diagnosed with IIM probably explain why some, but not all patients respond to rituximab. Myositis overlap and anti-synthetase syndromes seem to respond better than other patient subsets. © Clinical and Experimental Rheumatology 2013.
Berger M.,Virgen Del Rocio Childrens Hospital |
Berger M.,Ludwig Maximilians University of Munich |
Neth O.,Virgen Del Rocio Childrens Hospital |
Ilmer M.,University of Texas Health Science Center at Houston |
And 6 more authors.
Journal of Hepatology | Year: 2014
Background & Aims Multidrug resistance presents a major problem in hepatoblastoma (HB), and new anti-tumor strategies are desperately needed. The substance P (SP)/neurokinin-1 receptor (NK1R) complex has been discovered to be pivotal in the development of a variety of human cancers, and NK1R antagonists, such as the clinical drug aprepitant, are promising future anticancer agents. Yet, the role of the SP/NK1R complex as a potential anticancer target in HB is unknown. Methods Human HB cell lines HepT1, HepG2, and HuH6, human tumor samples from 17 children with HB as well as mice xenografted with human HB cell line HuH6 were analyzed regarding the SP/NK1R complex as a potential new anti-tumor target in HB. Results Therapeutic targeting with the NK1R antagonists aprepitant, L-733,060, and L-732,138 led to growth inhibition and apoptosis in HepT1, HepG2, and HuH6 cells in a dose-dependent manner. Intriguingly, HB cells predominantly expressed the truncated splice variant of NK1R. Human fibroblasts showed only dismal NK1R expression and were significantly more resistant. Stimulation of HB cells with SP, NK1R's natural ligand, caused increased growth rates and abrogated the anti-proliferative effect of NK1R antagonists. Expression analysis of 17 human HB samples confirmed the clinical relevance of NK1R. Most importantly, oral treatment of a HuH6 xenograft mouse model with 80 mg/kg/day aprepitant for 24 days resulted in a striking reduction of tumor growth, as evidenced by reduced tumor volume and weight, lowered tumor-specific alpha-fetoprotein (AFP) serum levels, and decreased number of Ki-67 positive cells. Furthermore, aprepitant treatment inhibited in vivo angiogenesis. Conclusions For the first time, we describe the NK1R in its truncated splice variant as a potent target in human HB and an inhibitory effect in vivo and in vitro by NK1R antagonists. Therefore, NK1R antagonists should be considered promising new candidates for innovative therapeutic strategies against HB. © 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
Rosety-Rodriguez M.,University of Cádiz |
Camacho A.,Juan Ramon Jimenez Hospital |
Rosety I.,University of Cádiz |
Fornieles G.,University of Cádiz |
And 5 more authors.
Archives of Physical Medicine and Rehabilitation | Year: 2014
Objective To ascertain the effect of arm cranking exercise on improving plasma levels of inflammatory cytokines and adipokines in untrained adults with chronic spinal cord injury (SCI). Design Longitudinal study. Setting Community-based supervised intervention. Participants Men (N=17) with complete SCI at or below T5 volunteered for this study. Participants were randomly allocated to the intervention (n=9) or control group (n=8) using a concealed method. Intervention A 12-week arm cranking exercise program of 3 sessions per week consisted of warm-up (10-15min), arm crank (20-30min; increasing 2min and 30s every 3wk) at a moderate work intensity of 50% to 65% of heart rate reserve (starting at 50% and increasing 5% every 3wk), and cool-down (5-10min). Main Outcome Measures Plasma levels of leptin, adiponectin, plasminogen activator inhibitor-1, tumor necrosis factor-alpha, and interleukin-6 were determined. Furthermore, physical fitness (maximum oxygen consumption [V̇O 2max]) and body composition (anthropometric index, waist circumference, and body mass index) were also assessed. Results Plasma levels of leptin, tumor necrosis factor-alpha, and interleukin-6 were significantly decreased after the completion of the training program. Similarly, the anthropometric index and waist circumference were diminished too. A moderate correlation was found between leptin and the anthropometric index. Finally, V̇O2max was significantly increased, suggesting an improvement of physical fitness in the intervention group. No changes were found in the control group. Conclusions Arm cranking exercise improved low-grade systemic inflammation by decreasing plasma levels of inflammatory cytokines. Furthermore, it also reduced plasma leptin levels. Long-term, well-conducted studies are still required to determine whether these changes may improve clinical outcomes of adults with chronic SCI. © 2014 by the American Congress of Rehabilitation Medicine.
Barba-Pichardo R.,Juan Ramon Jimenez Hospital |
Morina-Vazquez P.,Juan Ramon Jimenez Hospital |
Fernandez-Gomez J.M.,Juan Ramon Jimenez Hospital |
Venegas-Gamero J.,Juan Ramon Jimenez Hospital |
Herrera-Carranza M.,Juan Ramon Jimenez Hospital
Europace | Year: 2010
Aims Right ventricular apical pacing can have deleterious effects and the His bundle has been widely reported to be an alternative site. This paper presents our experience with permanent His-bundle pacing (HBP).Methods and resultsPatients referred for pacemaker implants (regardless of block type) were screened to determine if temporary HBP corrected conduction dysfunctions (threshold ≤2.5 V for 1 ms) and provided infra-Hisian 1:1 conduction of at least 120 s/m. Of the 182 patients selected, HBP corrected conduction dysfunctions in 133 (73) patients, 42 (32) of whom were rejected for the permanent procedure due to high thresholds. His-bundle lead implantation was attempted in the remaining 91 patients and was successful in 59 (65 of all attempts, 44 of all possible cases).ConclusionIn some patients, permanent HBP may be an alternative to right ventricular apical pacing. © 2009 The Author.
Morina-Vazquez P.,Juan Ramon Jimenez Hospital |
Roa-Garrido J.,Juan Ramon Jimenez Hospital |
Fernandez-Gomez J.M.,Juan Ramon Jimenez Hospital |
Venegas-Gamero J.,Juan Ramon Jimenez Hospital |
And 2 more authors.
PACE - Pacing and Clinical Electrophysiology | Year: 2013
Background Biventricular pacing through the coronary sinus (CS) is effective for the treatment of patients with heart failure and left bundle-branch block. However, this approach is not always feasible. Although surgical epicardial lead implantation is an alternative, the technique may be deleterious in some patients. Thus, direct left ventricular (LV) endocardial pacing under local anesthesia may be an option. Objective We describe our technique and analyze the results of direct LV endocardial pacing. Method Fourteen patients with failed resynchronization via CS (April 2006-September 2011) were selected. Using a femoral approach, we performed transseptal puncture and LV mapping, then fixed the active lead where the longest electrical delay was observed; the generator was placed in the anterior thigh. For resynchronization, eight patients with a device previously implanted through the upper veins received a single-chamber generator that was set to the VVT mode to sense the subclavian pacing spike. Six patients received a complete femoral resynchronization system with either a defibrillator or pacemaker. Patients were followed for 6-54 months. Results The LV lead was successfully implanted in all cases. Two patients experienced excessive bleeding and two died during follow-up. All except one improved at least one New York Heart Association class and experienced improved left ventricle ejection fraction. One patient with recurrent episodes of ventricular fibrillation was asymptomatic. Conclusion Direct LV endocardial pacing is safe and may be a less risky, more efficient alternative than surgical epicardial lead implantation for resynchronization via CS. ©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.
Transradial occlusion of a large intercostal branch of a left internal mammary artery graft with the novel amplatzer vascular plug 4 using a 4 French diagnostic catheter: Treatment of coronary steal phenomenon
Sanghvi A.B.,Juan Ramon Jimenez Hospital |
Diaz Fernandez J.F.,Juan Ramon Jimenez Hospital |
Gomez Menchero A.E.,Juan Ramon Jimenez Hospital
Journal of Invasive Cardiology | Year: 2011
Sidebranches of the left internal mammary artery that are not ligated at the time of coronary artery bypass surgery can cause coronary steal syndrome, resulting in angina. Several isolated case reports have demonstrated successful resolution of this steal syndrome after transcatheter embolization of these branches. Here, we describe successful occlusion of such a sidebranch using an Amplatzer vascular plug via the transradial route with a 4 Fr Judkins Right coronary artery diagnostic catheter.