Publications by authors named "Jose R Ramirez"

8 Publications

  • Page 1 of 1

Hybrid single-port cholecystectomy in children.

JSLS 2012 Jul-Sep;16(3):401-5

Cleveland Clinic Foundation, Department of Pediatric Surgery, Pediatric Institute & Children's Hospital, Cleveland, OH 44195, USA.

Background And Objectives: Multiple single-port or single-incision techniques have been successfully implemented for laparoscopic cholecystectomy in adults and children. These techniques require either a large multichannel port or a larger skin incision to accommodate multiple ports or instruments. Inspired by a first generation single-port instrument, we developed a safe and effective technique for a single-port laparoscopic cholecystectomy with virtually scarless results.

Methods: Over a 14-mo period, 20 patients (19 females, 1 male) underwent the hybrid single-port cholecystectomy. A straight 10-mm Storz telescope with inbuilt 6-mm working channel in combination with 2 portless 2.3-mm percutaneous graspers was used. The dissection is carried out with 43-cm bariatric length instruments. The cystic artery and duct are sealed with WECK Hem-o-lok clips or the Harmonic scalpel.

Results: Range (mean) age: 7.7 y to 19.5 y (15.5), BMI: 11.6kg/m(2) to 42.3kg/m(2) (27), operative duration 48 min to 120 min (79), postoperative length of stay: 5 h to 78 h (24).

Diagnosis: 13 patients cholecystolithiasis, 7 patients biliary dyskinesia. Conversion to conventional 4-port cholecystectomy was required in 2 patients. No intra- or postoperative complications occurred.

Conclusion: The hybrid single-port technique is easy to master. It provides traditional anatomical exposure and allows application of conventional laparoscopic principles.
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http://dx.doi.org/10.4293/108680812X13427982377148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3535812PMC
February 2013

Nipple-areola complex evaluation in long pedicled breast reductions with real-time fluorescent videoangiography.

Plast Reconstr Surg 2011 Aug;128(2):585-586

Department of Plastic Surgery; Cleveland Clinic Florida; Weston, Fla. (Brunworth, Samson, Newman) Department of General Surgery; Cleveland Clinic; Cleveland, Ohio (Ramirez).

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http://dx.doi.org/10.1097/PRS.0b013e31821e71f6DOI Listing
August 2011

Heat shock proteins (HSP70 and HSP27) as markers of epithelial dysplasia in oral leukoplakia.

Am J Dermatopathol 2006 Oct;28(5):417-22

Stomatology Department, School of Medicine and Dentistry, University of Santiago de Compostela, Spain.

Heat shock proteins (HSPs) play a significant role in cell proliferation, differentiation, and oncogenesis. HSP70 and HSP27 are constitutively and gradually expressed in a broad range of normal tissues and neoplasms, and their expression has been assessed as markers for oral epithelial dysplasia. The study involved 43 patients with oral leukoplakia (OL): 23 were categorized as nondysplastic and 20 as dysplastic OLs. Immunohistochemistry was carried out with the monoclonal antibodies HSP70 and HSP27. The presence of epithelial dysplasia and its histologic grading was evaluated according to the World Health Organization classification: mild, moderate, and severe squamous epithelial dysplasia. Expression of HSPs within the epithelium was also evaluated. The difference in the percentage of HSP70 positive nuclei in nondysplastic and dysplastic OL reached statistical significance(Equation is included in full-text article.)95% confidence interval = 17.74-43.82; P = 0.000). None of the 43 specimens analyzed showed positive nuclear immunostaining for anti-HSP27 antibody. No significant difference for HSP27 cytoplasmic expression could be identified between OL with or without epithelial dysplasia(Equation is included in full-text article.)95% confidence interval = 0.44-3.95; P = 0.89). It is concluded that the nuclear HSP70 immunoexpression could be an objective marker for the presence of the epithelial dysplasia in OL.
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http://dx.doi.org/10.1097/01.dad.0000211509.44865.bbDOI Listing
October 2006

Mutation study of Spanish patients with hereditary hemorrhagic telangiectasia and expression analysis of Endoglin and ALK1.

Hum Mutat 2006 Mar;27(3):295

Centro de Investigaciones Biologicas, Madrid, Spain.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant and age-dependent vascular disorder originated by mutations in Endoglin (ENG) or activin receptor-like kinase-1 (ALK1, ACVRL1) genes. The first large series HHT analysis in Spanish population has identified mutations in 17 unrelated families. Ten different mutations in ALK1 and six in ENG genes were found. Six unrelated families had a mutation in ENG gene, four representing new mutations, p.Y258fs, pV323fs, p.F279fs (c.834_837del CTTC), and p.F279fsdupC. Eleven unrelated families harboured mutations in ALK1; ten were new mutations identified as p.H328P, p.R145fs, p.G68C, p.A377T, p.H297R, p.M376T, p.C36Y, p.H328P, p.T82del and p.R47P. Overall, ALK1 mutations (HHT2) were predominant over ENG mutations (HHT1), in agreement with data reported for other Mediterranean countries (France, Italy), but at variance with Northern Europe or North America. Endoglin expression in HHT1 or HHT2 activated monocytes and blood outgrowth endothelial cells (BOECs) from older patients was well below the theoretical 50% level expected from the HHT1 haploinsufficiency model, suggesting that the pathogenic endoglin haploinsufficiency leading to the HHT phenotype is age-dependent. Interestingly, ALK1 protein levels of HHT BOECs in some missense ALK1 mutants were similar to controls. In vitro expression of these ALK1 constructs suggests that, in addition to the haploinsufficiency model, certain ALK1 mutants may inhibit the function of the wild type allele.
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http://dx.doi.org/10.1002/humu.9413DOI Listing
March 2006

Mutation analysis in Spanish patients with hereditary hemorrhagic telangiectasia: deficient endoglin up-regulation in activated monocytes.

Clin Chem 2004 Nov 16;50(11):2003-11. Epub 2004 Sep 16.

Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain.

Background: Mutations in the endoglin (ENG) or ALK1 genes are responsible for hereditary hemorrhagic telangiectasia types 1 and 2 (HHT1 and HHT2), respectively, a dominant vascular dysplasia caused by haploinsufficiency. No formal mutation studies of patients with HHT have been conducted in Spain.

Methods: ENG and ALK1 mutation analyses were carried out in 13 Spanish HHT patients diagnosed according to the Curacao criteria. Because endoglin is up-regulated at the cell surface during the monocyte-macrophage transition, endoglin concentrations in activated monocytes were determined by immunofluorescence flow cytometry in a systematic analysis. As controls, 40 non-HHT volunteers were studied for up-regulation of endoglin in activated monocytes.

Results: The mutation responsible for HHT was identified in eight patients belonging to two unrelated families. One of the families has a nonsense mutation in exon 4 (c.511C>T; R171X) of the ENG gene, and accordingly the disorder was identified as HHT1. The other family has a missense mutation affecting exon 8 (c.1120C>T; R374W) of the ALK1 gene, and hence is a HHT2 family. Interestingly, endoglin up-regulation was deficient in activated monocytes of both HHT1 and HHT2 patients compared with controls. By contrast, endoglin up-regulation was age-independent in control donors across a broad range of ages. The extent of endoglin up-regulation in activated monocytes was most diminished in those patients with the most severe symptoms.

Conclusions: Endoglin up-regulation in activated monocytes is impaired in HHT1 and HHT2 patients and is age-dependent in both HHT types. Endoglin expression may predict the clinical severity of HHT.
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http://dx.doi.org/10.1373/clinchem.2004.035287DOI Listing
November 2004

Lumican is down-regulated in cells expressing endoglin. Evidence for an inverse correlationship between Endoglin and Lumican expression.

Matrix Biol 2004 Jan;22(7):561-72

Centro de Investigaciones Biológicas (CSIC), Ramiro de Maeztu, 9 Madrid, Spain.

Endoglin (CD105) is a homodimeric membrane glycoprotein, which acts as a TGF-beta coreceptor in the vasculature and plays an important role in cardiovascular development and vascular remodelling. To isolate putative genes regulated by endoglin expression, a PCR-based RNA fingerprinting technique was carried out. Myoblasts stably transfected with endoglin showed a decrease in the expression of lumican both at the RNA and protein levels. Lumican is a proteoglycan of the extracellular matrix, belonging to the SLRP (Small Leucine-Rich Repeat Proteoglycans) family. Lumican down-regulation by endoglin appeared to be controlled, at least in part, at the transcriptional level, as indicated by RT-PCR, and transient transfection experiments using a lumican promoter reporter based vector. This inverse correlation between endoglin and lumican expression was substantiated by immunohistochemical staining of vessels from human tissues. Thus, cells belonging to the high endothelia, such as tonsil, express a large amount of endoglin, and the lumican content of their matrix is considerably reduced. Conversely, in resting endothelia, such as that of large vessels, the expression of endoglin is reduced whereas the amount of lumican is greatly increased. The inverse regulation in the expression of endoglin and lumican was also evident after TGF-beta treatments since endoglin was up-regulated, whereas lumican was down-regulated by this cytokine. This report describes for the first time a relationship between endoglin and lumican expression.
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http://dx.doi.org/10.1016/j.matbio.2003.11.006DOI Listing
January 2004

A cross-talk between hypoxia and TGF-beta orchestrates erythropoietin gene regulation through SP1 and Smads.

J Mol Biol 2004 Feb;336(1):9-24

Centro de Investigaciones Biológicas, CSIC Ramiro de Maeztu, 9, Madrid 28040, Spain.

Erythropoietin (Epo) is the humoral regulator of red blood-cell production. Low oxygen tension increases the Epo levels by enhancing transcription, through the hypoxia-inducible factor (HIF)-1, a transcriptional modulator in oxygen-regulated gene expression. In the present work, a cooperative interaction between hypoxia, mediated by the HIF-1 complex, and transforming growth factor-beta (TGF-beta), mediated by Smad3/4, was revealed in the Epo gene. This cooperation is due to physical interaction between Smad3/4 and HIF-1alpha. The Smad3/4 binding site is located within the 3' Epo enhancer, downstream from the HRE consensus, and immediately adjacent to the orphan hepatic nuclear factor receptor (HNF-4). HNF-4 is interacting also with Smad3 and the HIF-1 complex, to potentiate further the cooperative effect between both factors. Moreover, Sp1 has been identified as the factor binding the promoter necessary for the full hypoxia inducibility of the EPO gene. However, this full induction is achieved only if the TGF-beta pathway is mediating a cross-talk between promoter (Sp1) and enhancer (HIF-1alpha) regions through Smad3. We show that Sp1 binding to the proximal promoter is relevant for Epo transcription, and contributes to the Epo induction by hypoxia. A functional cooperation among the transcription factors mediating hypoxia (HIF-1, Sp1), the TGF-beta pathway (Smad3/4), and tissue-specific HNF-4 is proposed for the regulation of the Epo gene. In this model, the physical contact between the upstream promoter and the 3' downstream enhancer is mediated by Sp1 and Smad3 factors, and would occur upon bending of the DNA intervening sequences. Thus, Sp1 would reinforce the promoter/enhancer contact, while Smad3 would stabilize the multifactorial complex by interacting with HIF-1/Sp1/HNF-4 and the coactivator CBP/p300. This model may be extended to other genes where collaboration between TGF-beta and hypoxia takes place.
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http://dx.doi.org/10.1016/j.jmb.2003.12.023DOI Listing
February 2004

Transcriptional activation of endoglin and transforming growth factor-beta signaling components by cooperative interaction between Sp1 and KLF6: their potential role in the response to vascular injury.

Blood 2002 Dec;100(12):4001-10

Centro de Investigaciones Biológicas, Consejo Superior Investigaciones Cientificas (CSIC), Madrid, Spain.

Endoglin is an endothelial membrane glycoprotein involved in cardiovascular morphogenesis and vascular remodeling. It associates with transforming growth factor-beta (TGF-beta) signaling receptors to bind TGF-beta family members, forming a functional receptor complex. Arterial injury leads to up-regulation of endoglin, but the underlying regulatory events are unknown. The transcription factor KLF6, an immediate-early response gene induced in endothelial cells during vascular injury, transactivates TGF-beta, TGF-beta signaling receptors, and TGF-beta-stimulated genes. KLF6 and, subsequently, endoglin were colocalized to vascular endothelium (ie, expressed in the same cell type) following carotid balloon injury in rats. After endothelial denudation, KLF6 was induced and translocated to the nucleus; this was followed 6 hours later by increased endoglin expression. Transient overexpression of KLF6, but not Egr-1, stimulated endogenous endoglin mRNA and transactivated the endoglin promoter. This transactivation was dependent on a GC-rich tract required for basal activity of the endoglin promoter driven by the related GC box binding protein, Sp1. In cells lacking Sp1 and KLF6, transfected KLF6 and Sp1 cooperatively transactivated the endoglin promoter and those of collagen alpha1(I), urokinase-type plasminogen activator, TGF-beta1, and TGF-beta receptor type 1. Direct physical interaction between Sp1 and KLF6 was documented by coimmunoprecipitation, pull-down experiments, and the GAL4 one-hybrid system, mapping the KLF6 interaction to the C-terminal domain of Sp1. These data provide evidence that injury-induced KLF6 and preexisting Sp1 may cooperate in regulating the expression of endoglin and related members of the TGF-beta signaling complex in vascular repair.
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http://dx.doi.org/10.1182/blood.V100.12.4001DOI Listing
December 2002