Saint Georges Hospital

London, United Kingdom

Saint Georges Hospital

London, United Kingdom
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Aoun M.,Saint Georges Hospital | Aoun M.,Saint - Joseph University | Makki M.,American University of Beirut | Azar H.,Saint - Joseph University | And 2 more authors.
BMC Nephrology | Year: 2017

Background: Vascular calcifications are highly prevalent in hemodialysis patients. Dephosphorylated-uncarboxylated MGP (dp-ucMGP) was found to increase in vitamin K-deficient patients and may be associated with vascular calcifications. Supplementation of hemodialysis patients with vitamin K2 (menaquinone-7) has been studied in Europe with a maximum 61% drop of dp-ucMGP levels. The aim of this study is to assess first the drop of dp-ucMGP in an Eastern Mediterranean cohort after vitamin K2 treatment and second the correlation between baseline dp-ucMGP and vascular calcification score. Methods: This is a prospective, pre-post intervention clinical trial involving 50 hemodialysis patients who received daily 360 μg of menaquinone-7 for 4 weeks. At baseline they were assessed for plasma dp-ucMGP levels and vascular calcification scores (AC-24) as well as for other demographic, clinical and biological variables. Dp-ucMGP levels were measured a second time at 4 weeks. Results: At baseline, dp-ucMGP levels were extremely elevated with a median of 3179.15 (1825.25; 4339.50) pM and correlated significantly with AC-24 (Spearman’s rho = 0.43, P = 0.002). Using a bivariate regression analysis, the association between dp-ucMGP levels and AC-24 was most significant when comparing dp-ucMGP levels less than 1000 to those more than 1000 pM (P = 0.02). Dp-ucMGP levels higher than 5000 pM were significantly associated with females, patients with recent fracture and patients with lower serum albumin (respectively P = 0.02, 0.004 and 0.046). The average drop of dp-ucMGP at 4 weeks of treatment was found to be 86% with diabetics having the lowest drop rate (P = 0.01). Conclusion: Vitamin K deficiency, as assessed by high dp-ucMGP levels, is profound in hemodialysis patients from the Eastern Mediterranean region and it is significantly correlated with vascular calcifications. Daily 360 μg of menaquinone-7, given for 4 weeks, effectively reduces dp-ucMGP in this population. Future studies are needed to assess the changes in vascular calcifications in hemodialysis patients treated with vitamin K2 over a longer follow-up period. Trial registration: The clinical trial was registered on clinicaltrials.gov (Identification number NCT02876354 , on August 11, 2016). © 2017 The Author(s).


Bornholdt D.,University of Marburg | Atkinson T.P.,Childrens Hospital | Bouadjar B.,CHU of Bab El Oued | Catteau B.,Service de Dermatologie et de Pediatrie | And 20 more authors.
Human Mutation | Year: 2013

Missense mutations affecting membrane-bound transcription factor protease site 2 (MBTPS2) have been associated with Ichthyosis Follicularis with Atrichia and Photophobia (IFAP) syndrome with or without BRESHECK syndrome, with keratosis follicularis spinulosa decalvans, and Olmsted syndrome. This metalloprotease activates, by intramembranous trimming in conjunction with the protease MBTPS1, regulatory factors involved in sterol control of transcription and in cellular stress response. In this study, 11 different MBTPS2 missense mutations detected in patients from 13 unrelated families were correlated with the clinical phenotype, with their effect on cellular growth in media without lipids, and their potential role for sterol control of transcription. Seven variants were novel [c.774C>G (p.I258M); c.758G>C (p.G253A); c.686T>C (p.F229S); c.1427T>C (p.L476S); c.1430A>T (p.D477V); c.1499G>A (p.G500D); c.1538T>C (p.L513P)], four had previously been reported in unrelated sibships [c.261G>A (p.M87I); c.1286G>A (p.R429H); c.1424T>C (p.F475S); c.1523A>G (p.N508S)]. In the enzyme, the mutations cluster in transmembrane domains. Amino-acid exchanges near the active site are more detrimental to functionality of the enzyme and, clinically, associated with more severe phenotypes. In male patients, a genotype-phenotype correlation begins to emerge, linking the site of the mutation in MBTPS2 with the clinical outcome described as IFAP syndrome with or without BRESHECK syndrome, keratosis follicularis spinulosa decalvans, X-linked, Olmsted syndrome, or possibly further X-linked traits with an oculocutaneous component. Missense mutations affecting membrane-bound transcription factor protease site 2 (MBTPS2) cluster in transmembrane domains. Amino-acid exchanges near the active site are more detrimental to functionality of the enzyme and, clinically, associated with more severe phenotypes. In male patients, a genotype-phenotype correlation begins to emerge, linking the site of the mutation in MBTPS2 with the clinical outcome described as IFAP syndrome with or without BRESHECK syndrome, keratosis follicularis spinulosa decalvans, X-linked, or the X-linked form of Olmsted Syndrome. © 2013 Wiley Periodicals, Inc.


PubMed | Karolinska Institutet, University of Genoa, University of Ferrara, Saint Georges Hospital and 5 more.
Type: | Journal: Circulation | Year: 2016

-Prenatal diagnosis of Coarctation of the Aorta (CoA) is still challenging and affected by high rates of false positive diagnoses. The aim of this study was to ascertain the strength of association and to quantify the diagnostic accuracy of different ultrasound signs in predicting CoA prenatally.-Medline, Embase, CINAHL and Cochrane databases were searched. Random-effect and HSROC model meta-analyses were used to analyse the data.-794 articles were identified and 12 (922 fetuses at risk for CoA) were included. Mean mitral valve diameter z-score was lower (p<0.001) and the mean tricuspid valve diameter z-score was higher in fetuses with CoA compared to those without CoA (p=0.01). Mean Aortic valve diameter z-score was lower in fetuses with CoA compared to normal fetuses (p= <0.001), but the ascending aorta diameter, expressed as z-score or mm, was similar between groups (p= 0.07 and 0.47, respectively). Mean aortic isthmus diameter z-scores measured either in sagittal (p= 0.02) or in three-vessel trachea view (p<0.001) were lower in fetuses with CoA. Conversely, the mean pulmonary artery diameter z-score, the right/left ventricular and pulmonary artery/ascending aorta diameter ratios were higher (p<0.001, p=0.02 and p=0.02, respectively) in fetuses with CoA compared to controls, while aortic isthmus/arterial duct diameter ratio was lower in fetuses with CoA compared to those without CoA (p<0.001). The presence of coarctation shelf and aortic arch hypoplasia were more common in fetuses with CoA compared to controls (OR: 26.0, 95% CI 4.42-153, p<0.001 and OR: 38.2, 95% CI 3.01-486, p=0.005), while persistent left superior vena cava (p= 0.85), ventricular septal defect (p= 0.12) and bicuspid aortic valve (p= 0.14) did not carry an increased risk for this anomaly. Multi-parametric diagnostic models integrating different ultrasound signs for the detection of CoA were associated with an increased detection rate.-The detection rate of CoA may improve when a multiple criteria-prediction model is adopted. Further large multicenter studies sharing the same imaging protocols are needed in order to develop objective models for risk assessment in these fetuses.


Irani C.,Allergy Immunology Section | Karam M.,Allergy Immunology Section | Baz Z.,Saint Georges Hospital | Maatouk H.,Hammoud Hospital | Zaitoun F.,American University of Beirut
Revue Francaise d'Allergologie | Year: 2013

Background: Pollen monitoring is essential in the case of pollen-related allergic diseases. It may guide physicians and patients towards better prevention strategies, diagnosis, and treatment. Methods: One hundred and twenty four patients suffering from rhinoconjunctivitis and/or asthma from different cities around the country, and consulting our allergy clinic between January 2008 and December 2008, were reviewed in a randomized, retrospective study. Skin prick-tests to a battery of perennial or seasonal allergens were performed to all patients during their visit. An airborne pollen investigation was performed in different cities around the country during this period. The Rhinoconjunctivitis Quality of Life (RQLQ) and the Asthma Quality of Life (AQLQ) questionnaires were used to assess the severity of symptoms. Results of the symptoms evaluation were then correlated to the peaks and total concentrations of airborne pollens. Results: The airborne pollen sampling showed that Cupressaceae species were the dominant pollen persisting almost at all seasons. A tremendous surge in pollen count was noted in March at all stations with remarkably high level of pollen averaging about 400 pollen/m3/day in the one station. Pollens such as olive, and grass were noted as the spring developed. Urticaceae most likely of the Parietaria species, the pollen specific to the Mediterranean area was noted starting in the spring at all stations. The incidence of exacerbations of rhinoconjunctivitis or asthma was highest during the spring season with a frequency of 58.87%. There was another lower peak in the fall with a frequency of 23.3%. A total of 15.32% of patients were allergic to Cupressaceae; 73.68% of them experienced symptoms during spring and 52.63% during fall. A total of 17.75% were allergic to grass; 59.1% of these patients suffered of symptoms during spring and 36.36% during the fall. A total of 24.2% were allergic to Parietaria; 76.67% of these patients endured symptoms during spring and 43.3% during fall. A total of 16.2% were allergic to olive; 70% of these patients experienced symptoms during spring and 30% during fall. A total of 24.2% were allergic to mites; 36.67% of these patients experienced symptoms during spring and 30% during fall. Conclusion: There was a good correlation between the peaks of the pollen count and the exacerbations of rhinoconjunctivitis and asthma symptoms. To our knowledge, this is the first study of clinical correlation with pollen count done in Lebanon. Continued monitoring and further studies will confirm our data, and support in better diagnosis and treatment of pollen-related allergic diseases. © 2013 Elsevier Masson SAS.


Background: The purpose of this study was to determine the diagnostic test accuracy of ultrasound for the detection of lateral epicondylitis. Methods: An electronic search of databases registering published (MEDLINE, EMBASE, CINAHL, AMED, Cochrane Library, ScienceDirect) and unpublished literature was conducted to January 2013. All diagnostic accuracy studies that compared the accuracy of ultrasound (index test) with a reference standard for lateral epicondylitis were included. The methodological quality of each of the studies was appraised using the QUADAS tool. When appropriate the pooled sensitivity and specificity analysis was conducted. Results: Ten studies investigating 711 participants and 1077 elbows were included in this review. Ultrasound had variable sensitivity and specificity (sensitivity: 64-100%; specificity: 36-100%). The available literature had modest methodological quality, and was limited in terms of sample sizes and blinding between index and reference test results. Conclusions: There is evidence to support the use of ultrasound in the detection of lateral epicondylitis. However its accuracy appears to be highly dependent on numerous variables such as operator experience, equipment and stage of pathology. Judgment should be used when considering the benefit of ultrasound for use in clinical practice. Further research assessing variables such a transducer frequency independently is specifically warranted. Level of evidence: Level II. © 2014 Elsevier Masson SAS.


Latham S.K.,Saint Georges Hospital | Smith T.O.,University of East Anglia
Orthopaedics and Traumatology: Surgery and Research | Year: 2014

Background: The purpose of this study was to determine the diagnostic test accuracy of ultrasound for the detection of lateral epicondylitis. Methods: An electronic search of databases registering published (MEDLINE, EMBASE, CINAHL, AMED, Cochrane Library, ScienceDirect) and unpublished literature was conducted to January 2013. All diagnostic accuracy studies that compared the accuracy of ultrasound (index test) with a reference standard for lateral epicondylitis were included. The methodological quality of each of the studies was appraised using the QUADAS tool. When appropriate, the pooled sensitivity and specificity analysis was conducted. Results: Ten studies investigating 711 participants and 1077 elbows were included in this review. Ultrasound had variable sensitivity and specificity (sensitivity: 64%-100%; specificity: 36%-100%). The available literature had modest methodological quality, and was limited in terms of sample sizes and blinding between index and reference test results. Conclusions: There is evidence to support the use of ultrasound in the detection of lateral epicondylitis. However, its accuracy appears to be highly dependent on numerous variables, such as operator experience, equipment and stage of pathology. Judgement should be used when considering the benefit of ultrasound for use in clinical practice. Further research assessing variables such a transducer frequency independently is specifically warranted. Level of evidence: Level II. © 2014 Elsevier Masson SAS.


Megarbane H.,Saint Georges Hospital | Megarbane A.,Saint - Joseph University
Orphanet Journal of Rare Diseases | Year: 2011

The IFAP syndrome is a rare X-linked genetic disorder reported in nearly 40 patients. It is characterized by the triad of Ichthyosis Follicularis, Alopecia, and Photophobia from birth. Other features such as short stature, intellectual disability, and seizures may develop in the first few years of life. Skin histopathology is non-specific and consists of dilated hair follicles with keratin plugs extending above the surface of the skin, decreased or absent sebaceous glands, and decreased desmosomes in number and size. The disorder results from mutations in the MBTPS2 gene that impairs cholesterol homeostasis and the ability to cope with endoplasmic reticulum stress. Follicular hyperkeratosis can be treated using topical keratolytics, emollients and urea preparations. A moderate response to acitretin therapy has been noted in some patients. Intensive lubrication of the ocular surface is essential. Life expectancy in patients with IFAP syndrome can vary from death in the neonatal period to normal surviving. Cardiopulmonary complications remain the major cause of death. © 2011 Mégarbané and Mégarbané; licensee BioMed Central Ltd.


Riachy M.,Hotel Dieu Of France Hospital | Najem S.,Hotel Dieu Of France Hospital | Iskandar M.,Hotel Dieu Of France Hospital | Choucair J.,Hotel Dieu Of France Hospital | And 2 more authors.
Sleep and Breathing | Year: 2016

Purpose: Obstructive sleep apnea (OSA) is associated with a range of neurocognitive and cardiovascular complications, leading to a compromised quality of life. Continuous positive airway pressure (CPAP) is the gold standard therapeutic intervention for this disorder, yet patient compliance remains essential to its success. This study aimed at identifying the determinants of short-term and long-term CPAP adherence in a group of Lebanese patients. Methods: This cross-sectional study, conducted at the Sleep Center of Hôtel-Dieu de France hospital in Lebanon, included patients diagnosed with OSA and treated with CPAP for at least one night between June 2008 and January 2015. Adherence was assessed based on patients’ self-report. Results: The study enrolled 138 patients. Adherence rate was 70.3 % at 6 months and around 50 % after 5 years. Cox models showed that use of nasal masks (HR 0.48, p = 0.048) and higher oxygen desaturation index at baseline (HR 0.98 p = 0.048) were associated with a better short-term CPAP adherence. Patients who bought their devices (HR 0.18, p < 0.001), those who were frequently contacted by the CPAP provider (HR 0.85, p 0.024), and those who reported improvement on treatment (HR 0.36, p < 0.001) were significantly more likely to remain adherent on the long term. Conversely, individuals on a diet plan tended to interrupt the treatment earlier (HR 4.85, p 0.039). Conclusion: The present findings should be further explored in order to tailor interventions to the specific needs of different patient profiles and secure an improved CPAP adherence. © 2016 Springer-Verlag Berlin Heidelberg


Aoun J.,Lebanese University | Saleh N.,Lebanese University | Waked M.,Saint Georges Hospital | Salame J.,Lebanese University | Salameh P.,Lebanese University
Journal of Epidemiology and Global Health | Year: 2013

Background: Lung cancer is one of the most prevalent types of cancers. However, there are no epidemiological studies concerning lung cancer and its risk factors in Lebanon. This study was carried out to determine the association between lung cancer and its most common risk factors in a sample of the Lebanese population. Methods: A hospital-based case-control study was conducted. Patients were recruited in a tertiary health care center. A questionnaire in Arabic was designed to assess the possible risk factors for lung cancer. Results: For females, cigarette smoking (ORa. =. 9.76) and using fuel for heating (ORa. =. 9.12) were found to be the main risk factors for lung cancer; for males, cigarette smoking (ORa. =. 156.98), living near an electricity generator (ORa. =. 13.26), consuming low quantities of fruits and vegetables (ORa. =. 10.54) and a family history of cancer (ORa. =. 8.75) were associated with lung cancer. Waterpipe smoking was significantly correlated with lung cancer in the bivariate analysis. Conclusion: In this pilot study, it was found that in addition to smoking, outdoor and indoor pollution factors were potential risk factors of lung cancer. Additional studies would be necessary to confirm these findings. © 2013 Ministry of Health, Saudi Arabia.


PubMed | Hotel Dieu Of France Hospital and Saint Georges Hospital
Type: | Journal: Sleep & breathing = Schlaf & Atmung | Year: 2016

Obstructive sleep apnea (OSA) is associated with a range of neurocognitive and cardiovascular complications, leading to a compromised quality of life. Continuous positive airway pressure (CPAP) is the gold standard therapeutic intervention for this disorder, yet patient compliance remains essential to its success. This study aimed at identifying the determinants of short-term and long-term CPAP adherence in a group of Lebanese patients.This cross-sectional study, conducted at the Sleep Center of Htel-Dieu de France hospital in Lebanon, included patients diagnosed with OSA and treated with CPAP for at least one night between June 2008 and January 2015. Adherence was assessed based on patients self-report.The study enrolled 138 patients. Adherence rate was 70.3% at 6months and around 50% after 5years. Cox models showed that use of nasal masks (HR 0.48, p = 0.048) and higher oxygen desaturation index at baseline (HR 0.98 p = 0.048) were associated with a better short-term CPAP adherence. Patients who bought their devices (HR 0.18, p<0.001), those who were frequently contacted by the CPAP provider (HR 0.85, p 0.024), and those who reported improvement on treatment (HR 0.36, p<0.001) were significantly more likely to remain adherent on the long term. Conversely, individuals on a diet plan tended to interrupt the treatment earlier (HR 4.85, p 0.039).The present findings should be further explored in order to tailor interventions to the specific needs of different patient profiles and secure an improved CPAP adherence.

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