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An unusual case of hypereosinophilic syndrome and disseminated intravascular coagulation

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Chinese Medical Journal 2005年第18期118卷 1582-1584页

作者： YEUNG Tok-fai LAU Siu-wah WONG Kin Department of Medicine and Geriatrics Our Lady of Maryknoll Hospital Hong Kong ChinaDepartment of Medicine and Geriatrics Our Lady of Maryknoll Hospital Hong Kong China Department of Medicine and Geriatrics Our Lady of Maryknoll Hospital Hong Kong China

Idiopathic hypereosinophilic syndrome （HES） is a heterogeneous disorder characterized by hypereosinophilia and organ damage which affects men more commonly than women （9：1）. The disease can be detected incidentally, but more often presents either insidiously or acutely with cardiac, neurological, cutaneous, pulmonary or gastro- intestinal manifestations, with cardiac involvement being a major cause of morbidity and morality in patients. 1.2 Although a major mechanism of tissue damage in HES is due to thromboembolism,3 fullblown disseminated intravascular coagulation （DIC） is apparently rare. This paper describes a man who presented acutely with life-threatening DIC complicating HES and its management, the first Chinese case reported in the literature.

关键词： hypereosinophilic syndrome·disseminated intravascular syndrome·low molecular weight heparin

Deficiency of platelet-derived growth factor receptor-α-positive cells in Hirschsprung's disease colon

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World Journal of Gastroenterology 2016年第12期22卷 3335-3340页

作者： Anne-Marie O'Donnell David Coyle Prem Puri National Children's Research Centre Our Lady's Children's Hospital Crumlin

AIM: To investigate whether the expression of platelet-derived growth factor receptor-α-positive (PDGFRα+)-cells is altered in Hirschsprung’s disease (HD).METHODS: HD tissue specimens (n = 10) were collected at the time of pull-through surgery, while colonic control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 10). Immunolabelling of PDGFRα+-cells was visualized using confocal microscopy to assess the distribution of these cells, while Western blot analysis was undertaken to quantify PDGFRα protein ***: Confocal microscopy revealed PDGFRα+-cells within the mucosa, myenteric plexus and smooth muscle in normal controls, with a marked reduction in PDGFRα+-cells in the HD specimens. Western blotting revealed high levels of PDGFRα protein expression in normal controls, while there was a striking decrease in PDGFRα protein expression in the HD ***: These findings suggest that the altered distribution of PDGFRα+-cells in both the aganglionic and ganglionic HD bowel may contribute to the motility dysfunction in HD.

关键词： Platelet-derived growth factor receptor alpha Hirschsprung’ s disease Gastrointestinal motility Aganglionosis Myenteric plexus

Decreased expression of hyperpolarisation-activated cyclic nucleotide-gated channel 3 in Hirschsprung's disease

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World Journal of Gastroenterology 2015年第18期21卷 5635-5640页

作者： Anne Marie O’Donnell David Coyle Prem Puri National Children’s Research Centre our lady’s Children’s hospital Cnmlin Dublin 12 Ireland

AIM: To determine if hyperpolarisation-activated nucleotide-gated(HCN) channels exist in human colon, and to investigate the expression of HCN channels in Hirschsprung's ***:We investigated HCN1,HCN2,HCN3 and HCN4 protein expression in pull-through specimens from patients with Hirschsprung’s disease(HSCR,n=10)using the proximal-most ganglionic segment and distalmost aganglionic segment,as well as in healthy control specimens obtained at the time of sigmoid colostomy closure in children who had undergone anorectoplasty for imperforate anus(n=10).Fluorescent immunohistochemistry was performed to assess protein distribution,which was then visualized using confocal ***:No HCN1 channel expression was observed in any of the tissues *** HCN2 and HCN4proteins were found to be equally expressed in the aganglionic and ganglionic bowel in HSCR and ***3 channel expression was found to be markedly decreased in the aganglionic colon vs ganglionic colon and ***2-4 channels were seen to be expressed within neurons of the myenteric and submucosal plexus of the ganglionic bowel and normal controls,and also co-localised to interstitial cells of Cajal in all tissues ***:We demonstrate HCN channel expression in human colon for the first *** HCN3expression in aganglionic bowel suggests its potential role in HSCR pathophysiology.

关键词： Hyperpolarisation-activated nucleotide-gated Interstitial cells of Cajal Hirschsprung’s Pacemaker Ih current

Tunneled peritoneal catheters for the palliative therapy of malignant ascites

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Family Medicine and Community Health 2013年第1期1卷 17-22页

作者： YVONNE RYAN KAREN LYONS JOHN HANSEN AISLING O’GORMAN North Eastern General Practice Training Scheme Department of Radiology Beaumont HospitalDublin our lady of Lourdes hospital DroghedaCo.Louth

Objective:Malignant ascites presents difficult management issues in the palliative care population and results in distressing symptoms and recurrent hospital *** palliative care services in our lady of Lourdes hospital have been using radiologically guided tunneled peritoneal catheters for the palliative therapy of troublesome malignant *** conducted a review of this management ***:There were 10 catheters inserted in 9 patients between May 2007 and June 2011 in patients with malignant ***:There were no procedural related mortalities or major *** catheter life was 98.9 *** catheter re-hospitalization rate was 1.55 times per *** patient survival post insertion was 140 *** eight percent of patients achieved death at ***:We conclude that this is a safe and useful strategy in the management of malignant ascites in a palliative care population.

关键词： Tunneled peritoneal catheter Palliative therapy Malignant ascites

Musculoskeletal Manifestations of Atypical Mycobacterium: Case Reports

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Surgical Science 2014年第1期5卷 25-27页

作者： Mujeeeb Rohilla Khalid Khan Husnain Raza Department of Orthopaedics Our Lady of Lourdes Hospital Drogheda Ireland our lady of Lourdes hospital Drogheda Ireland

Musculoskeletal manifestations of atypical Mycobacterium are very rare and they can be easily missed by the junior doctors. We are presenting two cases of atypical Mycobacterium: the first one was infected by cleaning a fish tank and the other was immune-compromised patients. Both the cases were treated with broad spectrum antiboitics initially, with no results, unless the specimen cultures were obtained surgically and the specific cultures were asked. The treatment was started after obtaining the culture results. Both the patients were started on antituberculous treatment for 3 - 6 months with good functional results.

关键词： Musculoskeletal Atypical Mycobacterium Antiboitics

Co-morbidity,not age predicts adverse outcome in clostridium difficile colitis

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World Journal of Gastroenterology 2000年第2期6卷 198-201页

作者： TS Dharmarajan M Sipalay R Shyamsundar EP Norkus CS Pitchumoni Divisions of Geriatrics our lady of Mercy Medical Center BronxNew YorkUSA Department of Biomedical Research Our Lady of Mercy Medical CenterBronxNew YorkUSA Divisions of Gastroenterology our lady of Mercy Medical Center BronxNew YorkUSA

AIM To examine whether age alone or co-morbidity is a risk factor for death in olderadults who developed Clostridium difficile(Cd)colitis during *** A retrospective,observationalstudy design was performed in our lady ofMercy Medical Center,a 650-bed,urban,community-based,university-affiliated teachinghospital.121 patients with a positive diagnosisof Cd colitis Caged 23-97 years)were studied,and data pertinent to demographic variables,medical history,co-morbidity,physicalexamination,and laboratory results *** was examined as a continuousvariable and stratified into Age1(80 years).However,age,per se,had no effect onmortality.A history of cardiac disease(P=0.036),recurrent or refractory infection>4weeks(P=0.007),low serum total protein(P=0.034),low serum albumin(P=0.001),antibiotic use >4 weeks(P<0.010),use of over 4 antibiotics(P=0.026),and use of certainclasses of antibiotics(P=0.035-0.004)werepredictive of *** was stronglypredicted by the use of penicillin-like antibioticsplus clindamycin,in the presence ofhypoalbuminemia,refractory sepsis,andcardiac disease(P=0.00005).CONCLUSION Cd colitis is common in the ***,unlike co-morbidity,age alonedoes not affect the clinical outcome(survival vsdeath).

关键词： clostridium difficile colitis/old age comorbidity/old age diarrhea colitis/therapy physical examination

Pigeon chest: comparative analysis of surgical techniques in minimal access repair of pectus carinatum (MARPC)

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World Journal of Pediatrics 2018年第1期14卷 18-25页

作者： Ancuta Muntean Ionica Stoica Amulya K.Saxena Department of Pediatric Surgery Chelsea Children’sHospitalChelsea and Westminster Hospital NHSFoundation TrustImperial College London369FulhamRoadLondon SW109NHUK Department of P aediatric Surgery Our Lady’s Children’sHospitalCrumlinDublinIreland

Background After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. Data sources Literature was reviewed on PubMed with the terms 'pectus carinatum', 'minimal access repair', 'thoracos-copy' and 'children'. Results Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15.46 years met the inclusion criteria. Success rate of corrections wasn = 125, about 89% in cumulative reports, with seven articles reporting 100%. The complication rate was 39.28%. Since the pectus bar is placed over the sternum and has a large contact area, skin irritation was the most frequent morbidity (n = 20, 14.28%). However, within the complication group (n = 55), wire breakage (n = 21, 38.18%) and bar displacement (n = 10, 18.18%) were the most frequent complications. Twenty-two (15.71%) patients required a second procedure. Recurrences have been reported in four of twelve techniques. There were no lethal outcomes. Conclusions MARPC techniques are not standardized, as MIRPE are, so comparative analysis is difficult as the only common denominator is minimal access. Surgical morbidity is high in MARPC and affects > 2/3rd patients with about 15% requiring surgery for complication management.

关键词： Children Complications Minimal access surgery Pectus carinatum

Development and validation of a novel PCR-RFLP based method for the detection of 3 primary mitochondrial mutations in Leber's hereditary optic neuropathy patients

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Eye and Vision 2015年第1期2卷 170-176页

作者： Siobhan Eustace Ryan Fergus Ryan David Barton Veronica O’Dwyer Derek Neylan National Optometry Centre Dublin Institute of TechnologyKevin StreetDublin 8Ireland School of Biological Sciences Dublin Institute of TechnologyKevin StreetDublin 8Ireland Centre for Medical Genetics Our Lady’s Hospital for Sick ChildrenCrumlinDublin 12Ireland

Background:Leber’s Hereditary Optic Neuropathy(LHON;MIM 535000)is one of the most commonly inherited optic neuropathies and it results in significant visual morbidity among young adults with a peak age of onset between the ages of 15–*** worldwide incidence of LHON is approximately 1 in 31,000.95%of LHON patients will have one of 3 primary mitochondrial mutations,G3460A(A52T of ND1),G11778A(R340H of ND4)and T14484C(M64V of ND6).There is incomplete penetrance and a marked gender bias in the development of visual morbidity with approximately 50%of male carriers and 10%of female carriers developing optic *** recovery can occur but is dependent on the mutation present with the highest level of visual recovery seen in patients who have the T14484C *** 3 primary mutations are typically identified by individual end-point PCR-restriction fragment length polymorphism(RFLP)or individual targeted bi-directional Sanger sequencing *** purpose of this study was to design a simple multiplex PCR-RFLP that could detect these 3 primary LHON mutations in one ***:PCR primers were designed to incorporate a MaeIII restriction site in the presence of 3460A and 14484C mutations with the 11778A mutation naturally incorporating a MaeIII site.A multiplex PCR-RFLP assay was developed to detect the 3 common mutations in a single *** LHON controls based on the mitochondrial genome harbouring the 3 common mutations were synthesized and cloned into plasmids to act as reliable assay *** from previously tested patients and the synthetic LHON controls were subjected to the multiplex PCR-RFLP *** RFLP products were detected by agarose gel ***:The novel PCR-RFLP assay accurately detects the 3 primary mutations both in patient DNA and in synthesized DNA control samples with a simple visual mutation detection *** synthesized DNA was demonstrated to be a robust control for the detection of LHON Mutations

关键词： LHON Mitochondrial mutations Mutation detection Visual morbidity Multiplex PCR

HIV Prevalence amongst Pregnant Women Clients Attending Antenatal Clinic at the Faith Alive Foundation and PMTCT Centre, Jos Plateau State

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World Journal of AIDS 2016年第2期6卷 59-64页

作者： Anyaka Charles Oyebode Tinuade Musa Jonah Isichei Mercy Anyaka Ifechi Isichei Christian Department of Obstetrics and Gynaecology University of Jos/Jos University Teaching Hospital Jos Nigeria Faith Alive Foundation and hospital Jos Nigeria Department of Surgery University of Jos/Jos University Teaching Hospital Jos Nigeria our lady of apostles hospital Jos Nigeria Department of Chemical Pathology University of Jos/Jos University Teaching Hospital Jos Nigeria

Introduction: HIV infection and AIDS is a public health problem worldwide, particularly affecting the populace in resource challenged setting like sub-saharan Africa. Women of reproductive age are mostly affected and infected with the HIV disease. Methodology: A cross-sectional study of 216 randomly selected women that booked for antenatal care at the Faith Alive Foundation and PMTCT centre Jos between 1st July to 31st December, 2014 was carried out. Information regarding age, parity, gestational age at booking, educational status and HIV sero-status of the clients was analysed. Screening test was conducted in a serial two step approval using determine and UNIGOLD as the confirmatory test, while STAT-PAK was the tie-beaker with discordant result as per the national algorithm. Positive samples were confirmed by western blot method. Result: A total of 18 women out of the 216 women studied were positive giving a sero-prevalence rate of 8.3%. The highest sero-prevalent rate was amongst 25 - 29 years age group (31.5%). Women of parity 1 - 4 constituted the highest group of 62.9%. Majority of the women 65.7% booked in the second trimester while 26.9% booked in the 3rd trimester. Most of the women 47.2% had secondary school education while only 5.6% of them had no form of education. Conclusion: HIV infection prevalence rate among antenatal attendants at Faith Alive Foundation and PMTCT centre Jos is still high. Multisectorial approach and intervention strategies should be further scaled up for the prevention of vertical transmission of the virus. However, it is worth noting that it is mainly an HIV/AIDS hospital which should have affected the outcome.

关键词： HIV Prevalence Antenatal Clinic Faith Alive Foundation and PMTCT Centre Jos Plateau State