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Phenotypical Aspects of a Familial Syndromic Retinitis Pigmentosa

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Open Journal of Ophthalmology 2024年第2期14卷 168-173页

作者： Irma Eneida Dos Santos Joseph Matar Mass Ndiaye Mariama Diambone Badji Alioune Abdoulaye Ndongo Gerauld Akpo jean pierre Diagne Gabriel Karold Mendy Mouhamed Attye Aboubacry Sadikh Sow Elhadji Amadou Ba Paule Aida Ndoye Roth Ophthalmology Department of Aristide Le Dantec Hospital Dakar Senegal Pediatric Department of Aristide Le Dantec Hospital Dakar Senegal Radiological Imaging Department of Aristide Le Dantec Hospital Dakar Senegal Ophthalmology Department of Abass Ndao Hospital Dakar Senegal

Aim: To report a familial case of syndromic retinitis pigmentosa identified at Aristide Le Dantec Hospital in Dakar and to describe their clinical characteristics ophthalmic. Observation: We report a sibling group of nine children, four died at a young age from unknown causes. Three children were affected by retinitis pigmentosa, two cases were syndromic. A history of nyctalopia was found in all three affected children. The mean age of onset of decreased visual acuity was 6.6 years. Patient 1 affected by syndromic retinitis pigmentosa had an extraocular sign of cystic dilation of the main bile duct. Patient 2 had myoclonic epilepsy, psychomotor retardation, and the molar tooth sign on cerebral MRI (highly suggestive of Joubert syndrome). The third child had isolated retinitis pigmentosa. Ophthalmological examinations (fundus examination, electroretinogram, and visual evoked potentials) and pediatric examinations in the remaining two children were normal. Discussion and Conclusion: Retinitis pigmentosa is a rare degenerative disease that can be associated with several other malformations, highlighting the importance of screening for associated conditions. It presents a grim functional prognosis and a life prognosis dependent on extraocular manifestations. Molecular biology (karyotyping, next-generation sequencing) could have identified the implicated genes and allowed for a formal diagnosis and genetic counseling.

关键词： Retinitis Pigmentosa Syndromic Heredity Ciliopathy

Retinoblastoma: The Situation in Burkina Faso over Ten Years

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Open Journal of Ophthalmology 2024年第3期14卷 175-195页

作者： Amadou Ouattara Anicet Corneille Beremwidougou Paté Sankara Mariam Traore Dolo Tierinyê Armand Meda Windinmanégdé pierre Djiguimde Rolande Kabore Chantal Gabrielle Bouda Gertrude Augustine Meda Hien Jerome Sanou jean Wenceslas Diallo Ahgbatouhabeba Zabsonre Ahnoux Ophthalmology Department Regional University Hospital of Ouahigouya Ouahigouya Burkina Faso Regional University Hospital of Ouahigouya Ouahigouya Burkina Faso National Center for the Fight against Blindness Ouagadougou Burkina Faso University Hospital of Sour Sanou Bobo-Dioulasso Burkina Faso Nouna District Hospital Nouna Burkina Faso University Hospital of Bogodogo Ouagadougou Burkina Faso University Hospital of Yalgado Ouedraogo Ouagadougou Burkina Faso

Context: In Burkina Faso, there is only one retinoblastoma treatment center located in the capital. Nowadays, the treatment of retinoblastoma has benefited from the contribution of scientific progress. Objective: The aim was to take stock of the situation of retinoblastoma in the pediatric oncology department from January 1, 2010 to December 31, 2019. Materials and Methods: This was a descriptive cross-sectional study with retrospective data collection over a 10-year period, based on records of patients admitted to pediatric oncology department of CHU-YO. Data were analysed using CS Pro version 7.2 software. Categorical variables were compared using Pearsons Chi-square test at the 5% significance level. Overall survival was estimated using the Kaplan-Meier method. Operational definitions were used for lost to follow-up, consultation and diagnosis delays. Results: We collected a total of 204 cases in 10 years, i.e. an annual average of 20.4 cases/year. The mean age at diagnosis was 37.5 months for unilateral cases and 26.4 months for bilateral cases. Male predominance was noted, with a sex ratio of 1.31. The majority of patients came from disadvantaged backgrounds (72% farming fathers and 91% housewives). Clinically, leukocoria and exophthalmos were the main presenting features. The average time to consultation was long (8.73 months) and unilateral localization was predominantly unilateral at 77%. In terms of treatment, 102 patients were eligible for curative treatment and 80 for palliative treatment. The prognosis was poor, with 41% death and numerous cases of lost to follow-up (18%). Overall survival was estimated at 32%. The factor associated with the lethality of retiniblastoma was the extension of the tumor to other organs. Conclusion: Recognition of the early clinical signs of retinoblastoma can anticipate the occurrence of this cancer. Health professionals should be encouraged to perform the Buckner test every time they come into contact with children aged 0 t

关键词： Retinoblastoma Prognosis Pediatric Oncology CHU-YO Burkina Faso

Contribution of High Sensible C-Reactive Protein (hsCRP) in the Assessment of the Risk of Onset of Type 2 Diabetes and Its Cardiovascular Complications

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Journal of Diabetes Mellitus 2024年第2期14卷 95-107页

作者： Maïmouna Touré Léopold Ngor Sène jean pierre Diagne Demba Diédhiou Souleymane Thiam Abdou Khadir Sow Mor Diaw Aïssatou Seck Salimata Diagne Houndjo Abdoulaye Ba Abdoulaye Samb Fatou Diallo-Agne Modou Oumy Kane Laboratoire de Physiologie et d’ Explorations Fonctionnelles Physiologiques Facult&#233 de M&#233decine de Pharmacie et d&#8217OdontoStomatologie (FMPOS) Universit&#233 Cheikh Anta Diop (UCAD) Dakar S&#233n&#233gal IRL3189 &#8220Environnement Sant&#233 Soci&#233t&#233&#8221 CNRS CNRST Bamako-UCAD Facult&#233 de M&#233decine de Pharmacie et d&#8217OdontoStomatologie (FMPOS) Universit&#233 Cheikh Anta Diop (UCAD) Dakar S&#233n&#233gal Laboratoire Privé de Biologie General Kaolack S&#233n&#233gal Centre Hospitalier Universitaire (CHU) Abasse Ndao Universit&#233 Cheikh Anta Diop (UCAD) Dakar S&#233n&#233gal Laboratoire de Biochimie et de Biologie molé culaire Facult&#233 de M&#233decine de Pharmacie et d&#8217OdontoStomatologie (FMPOS) Universit&#233 Cheikh Anta Diop (UCAD) Dakar S&#233n&#233gal Laboratoire de Physiologie Pharmaceutique Facult&#233 de M&#233decine de Pharmacie et d'OdontoStomatologie (FMPOS) Universit&#233 Cheikh Anta Diop (UCAD) Dakar S&#233n&#233gal

Background: Type 2 diabetes is a metabolic disease characterized by chronic hyperglycemia. The latter plays an essential role in inflammation by stimulating the production of pro-inflammatory cytokines or by increasing their secretion by an oxidative mechanism. These cytokines control the hepatic synthesis of an inflammatory protein called C-Reactive Protein (CRP). High or average values of CRP would have a predictive value of cardiovascular diseases and type 2 diabetes. The relationship between low CRP values and the risk of developing type 2 diabetes and its cardiovascular complications is not sufficiently studied. The hsCRP could serve as a predictive biomarker of risk of onset, follow-up and prognosis for type 2 diabetes and its cardiovascular complications. To answer this problem, we conducted this study, the aim of which was to study the predictive role of hsCRP in the risk of occurrence of type 2 diabetes and its cardiovascular complications. Materials and Methods: This is a prospective and cross-sectional case-control study involving 200 participants including 100 control women and 100 women with type 2 diabetes (mean age was respectively 49.89 years ± 8.26 & 51.92 years ± 7.18;p = 0.066). The interviews were conducted on the basis of a questionnaire. Physical examination collected biometric data and cardiovascular constants. The biochemical parameters such as hsCRP were analyzed by an automated Abbott device. Results: We noted that hsCRP was significantly higher in type 2 diabetic subjects compared to control subjects (p (rho = 0.40, p systolic blood pressure (rho = 0.30, p = 0.003), diastolic blood pressure (rho = 0.28, p = 0.006), total body fat (rho = 0.48, p Conclusion: hsCRP has a prognostic value in the evaluation of cardiovascular risk. It seems to play an important role in the pathogenesis of type 2 diabetes mellitus and its cardiovascular complications. It could thus be considered as a biomarker for the screening, monitoring and prognosis of type 2

关键词： hsCRP Biomarker Inflammation Type 2 Diabetes

Evidence of a Light-Sensing Role for Folate in Arabidopsis Cryptochrome Blue-Light Receptors

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Molecular Plant 2008年第1期1卷 68-74页

作者： Nathalie Hoang jean-pierre Bouly Margaret Ahmad Universite Paris Vl Casier 156 4 Place Jussieu 75005 Paris France Penn State University 25 Yearsley Mill Road Media PA 19063 USA

Arabidopsis cryptochromes cry1 and cry2 are blue-light signalling molecules with significant structural similarity to photolyases-a class of blue-light-sensing DNA repair enzymes. Like photolyases, purified plant cryptochromes have been shown to bind both flavin and pterin chromophores. The flavin functions as a light sensor and undergoes reduction in response to blue light that initiates the signalling cascade. However, the role of the pterin in plant cryptochromes has until now been unknown. Here, we show that the action spectrum for light-dependent degradation of cry2 has a significant peak of activity at 380 nm, consistent with absorption by a pterin cofactor. We further show that cry1 protein expressed in living insect ceils responds with greater sensitivity to 380 nm light than to 450 nm, consistent with a light-harvesting antenna pigment that transfers excitation energy to the oxidized flavin of cry1. The pterin biosynthesis inhibitor DHAP selectively reduces cryptochrome responsivity at 380 nm but not 450 nm blue light in these cell cultures, indicating that the antenna pigment is a folate cofactor similar to that of photolyases.

关键词： a Light-Sensing Role for Folate Arabidopsis Cryptochrome Blue-Light Receptors

Pozzolanic reactivity of silico-aluminous fly ash

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Particuology 2008年第2期6卷 93-98页

作者： pierre Adamiec jean-Charles Benezet Ali Benhassaine Ecole des Mines d'Alès 6 Avenue de Clavières30319 ALES CedexFrance

For some years it has been possible to control the particle size of fly ashes, by-products of thermal power stations. Incorporating these very fine particles （obtained by grinding and/or pneumatic selection） improves the physical-mechanical characteristics of mortars and concretes. In this study, we measured the lime consumption of the various fractions （granulometric and densimetric） and identified by X-ray diffraction the neoformed phases by the pozzolanic reaction, to show that it is not sufficient to simply define the pozzolanicity of products based on lime consumption since it does not take into account the nature of the phases formed. The size of the particles used in the test samples also has a determining effect on the quantity of lime consumed. Before comparing results, it is necessary to ensure that the size of the particles （of the global ash and its constituents） be the same. Two distinct neoformed ohases appear： CSH in the largest granular fractions （d〉 40 μm） and C3AH6 in the smaller fractions.

关键词： Fly ash Pozzolanic reaction Particle size

Relation between silico-aluminous fly ash and its coal of origin

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Particuology 2008年第2期6卷 85-92页

作者： jean-Charles Benezet pierre Adamiec Ali Benhassaine Ecole des Mines d'Alès 6 Avenue de Clavieres30319 ALES CedexFrance

Fly ashes are typical complex solids which incorporate at the same time intrinsic properties derived from the layers （various mineralogical and dimensional spectra） and major transformations generated during prior processing. To use fly ashes in various applications, it is necessary to characterise them completely. The first research to date carried out on silico-aluminous fly ashes in order to characterise them physically, morphologically, chemically and mineralogically, resulted in the recognition that they are relatively simple materials. In the present study, a silico-aluminous fly ash coming from the power station of Albi （France） was selected. Heat treatment at 450 and 1200℃ together with coal simulated the treatment undergone by coal in the power station in order to mimic real coal residue. In conclusion, the diversity of the particles contained in fly ash could only be exolained by the relation existing between the fly ash and its coal of origin.

关键词： Fly ash Selection Thermal treatment

Useful zero friction simulations for assessing MBS codes Pascal’s formula giving wheelsets frequency for zero wheel-rail friction

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Theoretical & Applied Mechanics Letters 2022年第3期12卷 145-155页

作者： jean pierre Pascal Pascal Coding Rue d’ErquyPleneuf-Val-Andre F-22370France

Present study provides a simple analytical formula,the“Klingel-like formula”or“Pascal’s Formula”that can be used as a reference to test some results of existing railway codes and specifically those using rigid *** develops properly the 3D Newton-Euler equations governing the 6 degrees of freedom(DoF)of unsuspended loaded wheelsets in case of zero wheel-rail friction and constant ***,by solving numerically these equations,we got pendulum like harmonic oscillations of which the calculated angular frequency is used for assessing the accuracy of the proposed formula so that it can in turn be used as a fast practical target for testing multi-body system(MBS)railway *** to the harmonic property of these pendulum-like oscillations,the squareω2 of their angular frequency can be made in the form of a ratio K/M where K depends on the wheelset geometry and load and M on its *** on K and M are useful to understand wheelsets *** analytical formula is derived from the first order writing of full trigonometric Newton-Euler equations by setting zero elastic wheel-rail penetration and by assuming small *** trigonometric equations are numerically solved to assess that the formula providesω2 inside a 1%accuracy for usual wheelsets *** decreasing the conicity down to 1×10^(−4) rad,the relative formula accuracy is under 3×10^(−5).In order to test the formula reliability for rigid contact formulations,the stiffness of elastic contacts can be increased up to practical rigidity(Hertz stiffness×1000).

关键词： Unsuspended wheelsets Klingel formula Rigid contact Harmonic oscillation Multi body system railway codes Code validation

Ocean-based climate actions recommended by academicians from Europe and China

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Science China Earth Sciences 2022年第8期65卷 1612-1614页

作者： jean-pierre GATTUSO Nianzhi JIAO Laboratoire d’Océanographie de Villefranche CNRS-Sorbonne University-IddriParis75006France Carbon Neutral Innovation Reaserch Center Xiamen UniversityXiamen361005China

Ocean acidification,deoxygenation and sea level Rise impacted by global warming intertwined with anthropogenic activities have resulted in tremendous ecological losses at species,diversity and ecosystem levels,leading... 详细信息

关键词： warming climate consequences

Efficacy and tolerability of chitin-glucan combined with simethicone(GASTRAP^(■)DIRECT)in irritable bowel syndrome:A prospective,open-label,multicenter study

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World Journal of Gastrointestinal Pharmacology and Therapeutics 2024年第3期15卷 10-22页

作者： Nathalie Talbodec Pauline Le Roy Peggy Fournier Benoit Lesage Elodie Lepoutre François Castex jean Michel Godchaux Lionel Vandeville Benjamin Bismuth Xavier Lesage Pauline Bayart Michael Genin Christel Rousseaux Veronique Maquet Salvatore Modica pierre Desreumaux Caroline Valibouze Department of Gastroenterology Hôpital privéLe BoisLille 59000France Department of Gastroenterology Hôpital privéde Villeneuve d’AscqVilleneuve d’Ascq 59650France Univ.Lille CHU LilleULR 2694–METRICSÉvaluation des Technologies de Santéet des Pratiques MédicalesLille 59000France Development of Intestinal Biotech 1 Avenue Oscar LambretLille 59045France KitoZyme SA Parc Industriel des hauts Sarts Zone 2Rue de MilmortHerstal 4040Belgium BiOkuris A Parc Industriel des hauts Sarts Zone 2Rue de MilmortHerstal 4040Belgium Department of Hepato-Gastroenterology Lille University HospitalLille 59000France U1286-INFINITE Institute for Translational Research in InflammationUniv.LilleInsermCHU LilleLille 59000France Department of Digestive Surgery and Transplantation Lille University HospitalLille 59037France

BACKGROUND Irritable bowel syndrome(IBS),defined according to the Rome IV diagnostic criteria,is a chronic functional gastrointestinal disorder characterized by recurrent abdominal pain related to altered bowel ***-line recommended treatments are limited to combining drugs targeting predominant symptoms,particularly pain(antispasmodics),constipation(laxatives),and diarrhea(loperamide),yielding only a limited therapeutic ***^(■)DIRECT is a class IIa medical formulation composed of a combination of chitin-glucan and simethicone indicated for the symptomatic treatment of gas-related gastrointestinal disorders by combining different mechanisms of *** To evaluate the efficacy,tolerability,and safety of 4-week GASTRAP^(■)DIRECT treatment in patients with *** In this prospective,multicenter,open-label trial,120 patients with IBS received three sticks of GASTRAP^(■)DIRECT(1.5 g/d of chitin-glucan and 0.75 mg/d of simethicone)per day for 4 *** primary endpoint was the responder rate,defined as the number of patients whose abdominal pain score decreased by≥30%from baseline to week(W)*** analysis was performed using the per-protocol *** symptoms,impact of global symptoms on daily life,change in stool consistency,and improvement in defecatory disorders were *** Overall,100 patients were *** W4,67%(95%CI:57-75)showed improvement in abdominal pain(score:5.8±2.4 vs 2.9±2.0,P<0.0001).Similar improvements were observed for bloating[8.0±1.7 vs 4.7±2.9,P<0.0001;60%(95%CI:50-70)responders],abdominal distension[7.2±2.1 vs 4.4±3.1,P<0.0001;53%(95%CI:43-63)responders],and impact of global symptoms on daily life[7.1±2.0 vs 4.6±2.9,P<0.0001;54%(95%CI:44-64)responders].Stool consistency improved in most patients(90%and 57%for patients with liquid and hard stools,respectively).Overall,42%of patients with defecatory disorders reported very much/considerable improvements by *** severe adverse event occurred,and tolerabil

关键词： Chitin-glucan Irritable bowel syndrome Abdominal pain Flatulence Defecatory disorders Stool consistency Natural non-pharmacological treatment