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Acute Thoracic syndrome in children: Epidemiological, Diagnostic and Evolutionary Aspects at the Albert Royer national children’s hospital in Dakar senegal

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Open Journal of Pediatrics 2023年第6期13卷 763-773页

作者： Guillaye Diagne Papa souleye sow Khadim Bop Maimouna sow Idrissa Demba Ba Albert Royer national children’s hospital Dakar Senegal

Acute chest syndrome (ACs) is a serious pulmonary complication of sickle cell disease. It is estimated to be responsible for a quarter of deaths in the pediatric sickle cell population. In senegal, there are not enough pediatric studies in this area. The objective of our study was to determine the epidemiological, diagnostic and evolutionary characteristics of ATs at the Albert Royer national children’s hospital (CHNEAR) in Dakar. This was a retrospective study in patients hospitalized at CHNEAR for ATs from January 1, 2021 to March 31, 2022. We included patients hospitalized and diagnosed with ATs. We had collected 102 patients, i.e. a hospital incidence of 2.96%. The average age of the children was 9 years old;the sex ratio was 1.04. The main symptoms on admission were hypoxemia (97.06%), chest pain (77.45%), dyspnea (77.45%) and fever (65.69%). 52.94% of patients had an associated vaso-occlusive crisis (VOC). The chest x-ray was abnormal in 92 patients, a rate of 90.20% and showed images of pneumonia (71%);bronchitis (17.65%) and pleurisy (0.98%). None of the children benefited from a pulmonary ultrasound. The treatment associated with analgesics (100%), broad-spectrum antibiotics (100%), oxygen therapy (100%), hydration (95.09%), transfusion (73.53%), non-ventilation invasive (6.86%), intubation (2.94%) and beta 2 mimetics (12.75%). No patient benefited from incentive spirometry. Almost all of the patients 95.10% (n = 97) had a favorable clinical evolution. However, five children (4.90%) had an unfavorable outcome including one case of complication such as stroke (0.98%) and four (4) cases of death. The average hospital stay was 8 days. ATs is common in children with sickle cell disease in senegal and its etiologies seem to be dominated by infectious causes in our context.

关键词： Acute Chest syndrome sickle Cell Disease children senegal

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Pneumothorax in children: Epidemiological Diagnostic and Evolutionary Aspects at the Albert Royer national children’s hospital in Dakar

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Open Journal of Pediatrics 2023年第5期13卷 663-668页

作者： Guillaye Diagne Khadim Bop Papa souleye sow Mohamed Mbodj serigne Tawa Ndiaye Idrissa Demba Ba Department of Pediatric Pulmonology Albert Royer National Children’s Hospital of Dakar Dakar Senegal

Pneumothorax is defined by the presence of air between the parietal pleura and the visceral pleura. Most of the child’s pneumothorax is traumatic. The spontaneous pneumothorax (Ps) of the child is rare, and often associated with a favoring factor. When it is large, it can affect the life expectancy. The objective of our study was to determine the epidemiological, diagnostic and evolutionary aspects of pneumothorax in children aged 0 to 15 years, hospitalized at the Albert Royer national children’s hospital in Dakar. This is a retrospective descriptive study of patient records followed at the Albert Royer national children’s hospital (CHNEAR) for the management of a pneumothorax during the period from 2020 to 2022. During our study, 15 patients were hospitalized for pneumothorax, with an average annual incidence of 7.5 cases per year. The sex ratio (H/F) was 2.03. The average age was 3.7 years. Chest pain was present in 27% of patients, dyspnea was found in 93%, and cough in 87% of patients. All patients underwent chest radiography and the location of the pneumothorax was predominant on the right in 60% of cases. Thoracic drainage was performed in 93.3% of patients associated with etiological treatment. The average length of hospitalization was 27.8 days with a cure rate of 86.7% and a recurrence rate of 13.3%.

关键词： Pneumothorax Drainage children Dakar

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Pulmonary Arterial Hypertension in Congenital Heart Disease with Left-to-Right shunt: According to a Retrospective study at Albert Royer’s national children’s hospital Center

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Open Journal of Pediatrics 2023年第5期13卷 649-656页

作者： Aliou Mar Coundoul Khadim Bop Abdou Aziz Faye Amadou Lamine Fall Idrissa Demba Ba Papa Mactar Faye Department of Cardiology Albert Royer’s National Children’s Hospital Dakar Senegal Pediatric Ward Abass Ndao Hospital Center Dakar Senegal

Pulmonary arterial hypertension PAH is a progressive disease characterized by an increase in pulmonary arterial pressure and resistance. It often complicates congenital heart disease with a left-to-right shunt. The objective of this study is to evaluate the evolutionary profile of the pulmonary pressures of children with congenital heart disease with left-to-right shunt and to identify the factors favoring an evolution towards PAH. This is a retrospective, descriptive and analytical study over a period of four years and six months (from January 1, 2016 to June 30, 2020) at the Center national d’Enfant Albert Royer in Dakar on a series of 87 cases. The hospital frequency was 3.98 per thousand, and the incidence of PAH was 9.44%. The sex ratio of 0.74. The average age was 44.76 months. The average time between the onset of symptoms and the diagnosis of heart disease is 78.81 days. Dyspnea was found in 70.11% of cases, the burst of pulmonary B2 was found in 55%. Global heart failure was noted in 39.08% of cases. Cardiomegaly was found in 89.66% and pulmonary hypervascularization was found in 57.72% of cases. CIV was present in 54.02% of cases, followed by PCA in 21.14% and CAVc in 18.39% of cases. furosemide was used in 97.70% of cases and Captopril in 74.71% of cases. sildenafil was used in 10.34% of patients, and 12.64% of patients benefited from surgical management. Eisenmenger syndrome was found in 12.64% of patients. PAH in these heart diseases is a formidable and frequent complication, due to an increase in precapillary flow. The major improvement in mortality and morbidity is early surgical management, in the first months of life, to prevent pulmonary vascular disease.

关键词： PAH Left-to-Right shunt Einsenmenger syndrome

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Enhanced autophagic clearance of amyloid-βvia histone deacetylase 6-mediated V-ATPase assembly and lysosomal acidification protects against Alzheimer's disease in vitro and in vivo

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Neural Regeneration Research 2025年第9期20卷 2633-2644页

作者： Zhimin Long Chuanhua Ge Yueyang Zhao Yuanjie Liu Qinghua Zeng Qing Tang Zhifang Dong Guiqiong He Institute of Neuroscience School of Basic Medical SciencesChongqing Medical UniversityChongqingChina Department of Anatomy Chongqing Medical UniversityChongqingChina Department of Physiology Chongqing Medical UniversityChongqingChina Pediatric Research Institute Children’s Hospital of Chongqing Medical UniversityChongqingChina Ministry of Education Key Laboratory of Child Development and Disorders Children’s Hospital of Chongqing Medical UniversityChongqingChina national Clinical Research Center for Child Health and Disorders Children’s Hospital of Chongqing Medical UniversityChongqingChina Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders Children’s Hospital of Chongqing Medical UniversityChongqingChina

Recent studies have suggested that abnormal acidification of lysosomes induces autophagic accumulation of amyloid-βin neurons,which is a key step in senile plaque ***,resto ring normal lysosomal function and rebalancing lysosomal acidification in neurons in the brain may be a new treatment strategy for Alzheimer's *** acetylation/deacetylation plays a central role in lysosomal ***,we show that inhibiting the classic microtubule deacetylase histone deacetylase 6 with an histone deacetylase 6 shRNA or thehistone deacetylase 6 inhibitor valproic acid promoted lysosomal reacidification by modulating V-ATPase assembly in Alzheimer's *** rthermore,we found that treatment with valproic acid markedly enhanced *** clearance of amyloid-βaggregates,and ameliorated cognitive deficits in a mouse model of Alzheimer's *** findings demonstrate a previously unknown neuroprotective mechanism in Alzheimer's disease,in which histone deacetylase 6 inhibition by valproic acid increases V-ATPase assembly and lysosomal acidification.

关键词： Alzheimer's disease amyloid-β APP/Ps1 mice autophagy cognitive impairment histone deacetylase 6 lysosomal acidification microtubule acetylation valproic acid V-ATPase

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Infantile spinal Muscular Atrophy at the Albert Royer national children’s hospital Center in Dakar

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Open Journal of Pediatrics 2024年第3期14卷 514-521页

作者： Guillaye Diagne Khadim Bop sofiatou Dieye Arame Faye Fatou Ly Amadou Lamine Fall Pediatrics Ward of Pikine hospital Pikine Senegal Pé diatrics Ward of Albert Royer National Hospital Dakar Senegal

Introduction: Infantile spinal muscular atrophy (IsA) is an autosomal recessive disease caused by primary degeneration of cells in the anterior horn of the spinal cord, leading to muscle weakness and hypotonia. Its incidence is estimated at 1 in 6000 births worldwide. In Africa, particularly in senegal, there are few studies interested on this pathology. We therefore deemed this study necessary, which set itself the objective of describing the diagnostic, therapeutic and progressive aspects of infantile spinal muscular atrophy at the Albert Royer national children’s hospital Center in Dakar (CHNEAR). Methodology: We conducted a retrospective descriptive study over a period of two (2) years from December 2020 to December 2022. Included were all hospitalized patients in whom the diagnosis of spinal muscular atrophy was made with or without genetic confirmation. The data were collected on a pre-established form then entered and analyzed with the following software: Excel 2013 and R version 4.1.3. Results: During our study period, 2100 children were hospitalized, the annual incidence was 0.76%. The average age of our patients was 9 ± 9 months with a range of 3 months to 32 months and the median was 6.5 months. The sex ratio was 7. The notion of family consanguinity was found in 62.5% of cases and the notion of IsA in the family in 25% of cases. Hypotonia and respiratory distress were found at the forefront in equal proportions (50% of cases). Electromyogram (EMG) was performed in 3 patients (37.5%). symptomatic medical treatment was administered in 100% of patients, 04 patients had benefited from respiratory physiotherapy, i.e. 50% of cases, and genetic counseling was carried out in one patient (12.5%). The evolution was immediately favorable in 2 patients or 25% of cases, unfavorable in 75% of cases with a death rate of 50% and the average age of death was 5.5 months ± 1 with extremes ranging from 3 to 7 months. Conclusion: The number of Infantile spinal muscular atrop

关键词： spinal Muscular Atrophy Child Hypotonia Dakar

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FUBP3 mediates the amyloid-β-induced neuronal NLRP3 expression

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Neural Regeneration Research 2025年第7期20卷 2068-2083页

作者： Jing Yao Yuan Li Xi Liu Wenping Liang Yu Li Liyong Wu Zhe Wang Weihong song The national Clinical Research Center for Geriatric Disease Xuanwu HospitalCapital Medical UniversityBeijingChina Department of Neurology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina Department of Neurosurgery Xuanwu HospitalCapital Medical UniversityBeijingChina Department of Pediatrics The Second Affiliated Hospital and Yuying Children’s HospitalKey Laboratory of Alzheimer’s Disease of Zhejiang ProvinceInstitute of AgingWenzhou Medical UniversityWenzhouZhejiang ProvinceChina Oujiang Laboratory(Zhejiang Laboratory for Regenerative Medicine Visionand Brain Health)WenzhouZhejiang ProvinceChina

Alzheimer's disease is characterized by deposition of amyloid-β,which forms extracellular neuritic plaques,and accumulation of hyperphosphorylated tau,which aggregates to form intraneuronal neurofibrillary tangles,in the *** NLRP3 inflammasome may play a role in the transition from amyloid-βdeposition to tau phosphorylation and *** NLRP3 is primarily found in brain microglia,and tau is predominantly located in neurons,it has been suggested that NLRP3 expressed by microglia indirectly triggers tau phosphorylation by upregulating the expression of pro-inflammatory ***,we found that neurons also express NLRP3 in vitro and in vivo,and that neuronal NLRP3 regulates tau *** biochemical methods,we mapped the minimal NLRP3 promoter and identified FUBP3 as a transcription factor regulating NLRP3 expression in *** primary neurons and the neuroblastoma cell line Neuro2A,FUBP3 is required for endogenous NLRP3 expression and tau phosphorylation only when amyloid-βis *** the brains of aged wild-type mice and a mouse model of Alzheimer's disease,FUBP3 expression was markedly increased in cortical *** analysis suggested that FUBP3 plays a role in neuron-mediated immune *** also found that FUBP3 trimmed the 5′end of DNA fragments that it bound,implying that FUBP3 functions in stress-induced *** findings suggest that neuronal NLRP3 may be more directly involved in the amyloid-β-to–phospho-tau transition than microglial NLRP3,and that amyloid-βfundamentally alters the regulatory mechanism of NLRP3 expression in *** that FUBP3 was only expressed at low levels in young wild-type mice and was strongly upregulated in the brains of aged mice and Alzheimer's disease mice,FUBP3 could be a safe therapeutic target for preventing Alzheimer's disease progression.

关键词： 5′end trimming Alzheimer's disease amyloid-beta amyloid-β-dependent transcription FUBP3 inflammasome inflammation neuron NLRP3 tau transcription factor

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Morbidity and Mortality in the Emergency Department of the Albert Royer national children’s hospital Center Dakar: Prospective study from January 1 to April 30, 2020

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Journal of Biosciences and Medicines 2024年第11期12卷 137-159页

作者： Hilaire Lisimo Abwa Halladin Mansoj Alioune Thiogane Mf Faye Feza Muhemerie Yvette Kpakpo Nyange Patient Kapambu Joel Djunguluka Erick Albert Kombo Uele University Clinics Department of Pediatrics Faculty of Medicine University of Uele Isiro Democratic Republic of the Congo Royer national childrens hospital Center Dakar Senegal hospital General of Watsa Watsa Democratic Republic of the Congo

Introduction: Infant and child morbidity and mortality constitute a public health problem in Africa, particularly in senegal. The objective of this work was to help identify the determinants this morbidity and mortality. Materials and Methods: A prospective study, over a 4-month period (January to April 2020). All patients aged 0 to 15 years, hospitalized for emergency reasons in the Albert Royer emergency department, were included. Mortality was analyzed according to sociodemographic data, the patient’s itinerary, transfer procedures, availability of emergency medications, diagnosis made during hospitalization and causes of death. The data were collected based on a protocol in an established file and analyzed by sPss (statistical Package for science social) software. Version 18. Results: 225 children in emergency situations were included. The age of our patients is between 0 and 15 years (minimum 0.23 months, maximum 191 months, average 47 months and standard deviation 54). Thus, emergencies represented 57.98% of the 388 hospitalizations. seventy percent of patients were less than 60 months old with a male predominance (sex ratio 1.16), 2.22% were newborns and 27.11% were aged between 60 and 191 months). A total of 79 (35.1%) were transferred and only 57.4% received care before transfer. A total of 12 deaths were reported, representing a lethality of 5.3%. Only young age (less than 59 months, a mortality rate of 91.7%), cardiac decompensations, severe sepsis, and neonatal conditions were more associated with mortality. The majority of our deceased patients came from families with a low socio-economic level (83.3%). Conclusion: Pediatric emergencies are frequent and responsible for lethality and require the necessary efforts, particularly through parent education, the creation of emergency outpatient care units and, above all, the strengthening of the technical platform and therapeutic means.

关键词： Morbidity Mortality Emergencies Infant and Juvenile

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FK506 contributes to peripheral nerve regeneration by inhibiting neuroinflammatory responses and promoting neuron survival

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Neural Regeneration Research 2025年第7期20卷 2108-2115页

作者： Yuhui Kou Zongxue Jin Yusong Yuan Bo Ma Wenyong Xie Na Han Department of Trauma and Orthopedics Peking University People’s HospitalBeijingChina national Center for Trauma Medicine Peking University People’s HospitalBeijingChina Key Laboratory of Trauma and Neural Regeneration(Peking University) Ministry of EducationBeijingChina Trauma Medicine Center Peking University People’s HospitalBeijingChina Department of Orthopedics China-Japan Friendship HospitalBeijingChina

FK506(Tacrolimus)is a systemic immunosuppressant approved by the *** and Drug ***506 has been shown to promote peripheral nerve regeneration,however,its precise mechanism of action and its pathways remain *** this study,we established a rat model of sciatic nerve injury and found that FK506 improved the morphology of the injured sciatic nerve,increased the numbers of motor and sensory neurons,reduced inflammatory responses,markedly improved the conduction function of the injured nerve,and promoted motor function *** findings suggest that FK506 promotes peripheral nerve structure recovery and functional regeneration by reducing the intensity of inflammation after neuronal injury and increasing the number of surviving neurons.

关键词： FK506 inflammation motor neurons nerve regeneration neuron peripheral nerve injury sensory neurons

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Impacts of Nutlin-3a and exercise on murine double minute 2-enriched glioma treatment

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Neural Regeneration Research 2025年第4期20卷 1135-1152页

作者： Yisheng Chen Zhongcheng Fan Zhiwen Luo Xueran Kang Renwen Wan Fangqi Li Weiwei Lin Zhihua Han Beijie Qi Jinrong Lin Yaying sun Jiebin Huang Yuzhen Xu shiyi Chen Department of sport Medicine Huashan HospitalFudan UniversityShanghaiChina Department of Orthopedic surgery Hainan Province Clinical Medical CenterHaikou Affiliated Hospital of Central South University Xiangya School of MedicineHaikouHainan ProvinceChina Department of Basic Medicine Shanghai Jiao Tong University School of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Neurosurgery Second Affiliated Hospital of Zhejiang University School of MedicineZhejiang UniversityHangzhouZhejiang ProvinceChina Department of Orthopedics Shanghai General HospitalSchool of Medicine Shanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Children’s Hospital of Fudan UniversityNational Children’s Medical CenterShanghaiChina Department of Rehabilitation The Second Affiliated Hospital of Shandong First Medical UniversityTaianShandong ProvinceChina

Recent research has demonstrated the impact of physical activity on the prognosis of glioma patients,with evidence suggesting exercise may reduce mortality risks and aid neural *** role of the small ubiquitin-like modifier(sUMO)protein,especially post-exercise,in cancer progression,is gaining attention,as are the potential anti-cancer effects of *** used machine learning to create the exercise and sUMO-related gene signature(EsLRs).This signature shows how physical activity might help improve the outlook for low-grade glioma and other *** demonstrated the prognostic and immunotherapeutic significance of EsLRs markers,specifically highlighting how murine double minute 2(MDM2),a component of the EsLRs,can be targeted by *** underscores the intricate relationship between natural compounds such as nutlin-3 and immune *** comprehensive CRIsPR screening,we validated the effects of specific EsLRs genes on low-grade glioma *** also revealed insights into the effectiveness of Nutlin-3a as a potent MDM2 inhibitor through molecular docking and dynamic ***-3a inhibited glioma cell proliferation and activated the p53 *** efficacy decreased with MDM2 overexpression,and this was reversed by Nutlin-3a or *** using a low-grade glioma mouse model highlighted the effect of physical activity on oxidative stress and molecular pathway ***,both physical exercise and Nutlin-3a administration improved physical function in mice bearing tumors derived from MDM2-overexpressing *** results suggest the potential for Nutlin-3a,an MDM2 inhibitor,with physical exercise as a therapeutic approach for glioma *** research also supports the use of natural products for therapy and sheds light on the interaction of exercise,natural products,and immune regulation in cancer treatment.

关键词： exercise and sUMO-related gene signatures(EsLRs) glioblastoma management low-grade glioma natural bioactives neural regeneration physical exercise

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AAV2-PDE6B restores retinal structure and function in the retinal degeneration 10 mouse model of retinitis pigmentosa by promoting phototransduction and inhibiting apoptosis

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Neural Regeneration Research 2025年第8期20卷 2408-2419页

作者： Ruiqi Qiu Mingzhu Yang Xiuxiu Jin Jingyang Liu Weiping Wang Xiaoli Zhang Jinfeng Han Bo Lei Henan Eye hospital Henan Provincial People’s HospitalPeople’s Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina Eye Institute Henan Academy of Innovations in Medical ScienceZhengzhouHenan ProvinceChina Branch of national Clinical Research Center for Ocular Disease Henan Provincial People’s HospitalZhengzhouHenan ProvinceChina Academy of Medical science Zhengzhou UniversityZhengzhouHenan ProvinceChina

Retinitis pigmentosa is a group of inherited diseases that lead to retinal degeneration and photoreceptor cell ***,there is no effective treatment for retinitis pigmentosa caused by PDE6B ***-associated virus(AAV)-mediated gene therapy is a promising strategy for treating retinitis *** aim of this study was to explore the molecular mechanisms by which AAV2-PDE6B rescues retinal *** do this,we injected retinal degeneration 10(rd10)mice subretinally with AAV2-PDE6B and assessed the therapeutic effects on retinal function and structure using dark-and light-adapted electroretinogram,optical coherence tomography,and ***-independent acquisition-mass spectrometry-based proteomic analysis was conducted to investigate protein expression levels and pathway enrichment,and the results from this analysis were verified by real-time polymerase chain reaction and western ***2-PDE6B injection significantly upregulated PDE6βexpression,preserved electroretinogram responses,and preserved outer nuclear layer thickness in rd10 *** expressed proteins between wild-type and rd10 mice were closely related to visual perception,and treating rd10 mice with AAV2-PDE6B restored differentially expressed protein expression to levels similar to those seen in wild-type *** Encyclopedia of Genes and Genome analysis showed that the differentially expressed proteins whose expression was most significantly altered by AAV2-PDE6B injection were enriched in phototransduction ***,the phototransductionrelated proteins Pde6α,Rom1,Rho,Aldh1a1,and Rbp1 exhibited opposite expression patterns in rd10 mice with or without AAV2-PDE6B ***,Bax/Bcl-2,p-ERK/ERK,and p-c-Fos/c-Fos expression levels decreased in rd10 mice following AAV2-PDE6B *** data suggest that AAV2-PDE6B-mediated gene therapy promotes phototransduction and inhibits apoptosis by inhibiting the ERK signaling pathway and upr

关键词： apoptosis AAV2-PDE6B ERK1/2 gene therapy phototransduction proteomics rd10 retinitis pigmentosa

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