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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

Survey and clinical considerations of gender identity in lower primary school children

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World Journal of Psychiatry 2024年第1期14卷 36-43页

作者： Ya-Lin Zhang Hong-Mei Zhang Jing-Xia Xu Qi-Ying Zhou He Wang Xiao-Cheng Pan Department of pediatrics Hangzhou Ninth People’s HospitalHangzhou 311225Zhejiang ProvinceChina Department of Nursing Hangzhou Children’s HospitalHangzhou 310014Zhejiang ProvinceChina Pediatric Intensive Care Unit Hangzhou Children’s HospitalHangzhou 310014Zhejiang ProvinceChina Out-patient infection Hangzhou Children’s HospitalHangzhou 310014Zhejiang ProvinceChina Traditional Chinese Medicine ward Hangzhou Children’s HospitalHangzhou 310014Zhejiang ProvinceChina

BACKGROUND Gender consciousness directly affects the development of gender identity,which is a continuous and lifelong ***,hospitalization is a part of many children's lives and has an impact on their gender *** To investigate the current situation of gender identity in lower primary school children by conducting a survey of 202 hospitalized children in the lower grades and to provide a theoretical basis and foundation for the cultivation of gender identity and medical treatment of children based on the *** study aims to inspire clinical medical staff to scientifically and reasonably arrange hospital wards for lower primary school children and pay attention to gender protection during the medical treatment process and to help children shape a unified and clear gender identity,which will enable them to better integrate into society and promote their personality *** The gender consciousness scale for elementary and middle school students was RESULTS Gender identity was already present in lower primary school *** children's gender roles and gender equality consciousness were strong,exceeding the critical value,but their gender characteristics,gender identity,and gender ideal consciousness were *** aged 6 had the weakest gender identity,and girls had significantly stronger gender identity than *** Gender identity is already present in lower primary school children,providing a basis and inspiration for the cultivation of gender identity and medical treatment of lower primary school *** medical staff should be aware of and understand these results and should scientifically and reasonably arrange hospital wards for lower primary school children.

关键词： School-age children Gender consciousness Gender identity hospitalization Gender weakening

Juvenile Systemic Sclerosis: About 9 Cases

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Open Journal of pediatrics 2024年第2期14卷 320-326页

作者： Kaoutar Danaoui Houda Nassih Khadija Oujennane Rabiy El Qadiry Aicha Bourrahouat Said Amal Imane Ait Sab B Pediatric ward Mohammed VI University Hospital Center Marrakesh Morocco Dermatology ward Mohammed VI University Hospital Center Marrakesh Morocco

Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, hospital Center in Marrakech, Morocco.

关键词： Scleroderma Systemic Sclerosis Children Pediatric

Enhancing pediatric asthma management through multifaceted health education

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World Journal of Clinical Cases 2024年第19期12卷 3708-3716页

作者： Guang-Qun Lin Zu-Qiong Ye En-Lian Song Yu-Nan Lin Department of pediatrics First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina Day Surgery ward First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina Anesthesia&Operation Center First Affiliated Hospital of Guangxi Medical UniversityNanning 530021Guangxi Zhuang Autonomous RegionChina

BACKGROUND Pediatric asthma is a significant public health issue that impacts the quality of life of children *** management approaches focus on symptom control and medication adherence but often overlook the comprehensive educational needs of patients and their families.A multifaceted health education approach may offer a more holistic strategy in managing pediatric asthma,especially in outpatient *** To evaluate the efficacy of a comprehensive health education strategy in improving disease management,medication adherence,and quality of life among children with asthma in outpatient *** In total,100 pediatric patients with severe asthma were enrolled from January 2021 to November 2022 and randomly allocated to a control group(n=50)or an observation group(n=50).The control group received standard nursing care,including basic nursing interventions and health education upon *** contrast,the observation group was exposed to a broad spectrum of health education methodologies,including internet-based hospital systems,social media channels,one-on-one verbal education,informational brochures,slide present ations,telephone check-ins,animated videos,and illustrated health education *** on asthma management knowledge,symptom control,quality of life[***’s Respiratory Questionnaire(SGRQ)],treatment adherence,and nursing satisfaction were collected and *** The scores of the observation group in knowledge areas,such as medication,home care,disease understanding,symptom management,prevention strategies,and nutritional guidance,were significantly higher than those of the control group(P<0.05).In addition,the observation group exhibited greater symptom control,improved quality of life based on their SGRQ scores,and higher treatment adherence post-intervention(P<0.05).Nursing satisfaction was also rated higher in the observation group across all evaluated areas(P<0.05).CONCLUSION Implementing a diversified hea

关键词： Multifaceted health education mode pediatrics Outpatient service Bronchial asthma

Epidemiological and Clinical Profile and Immediate Outcome of Neonates Transferred from the Maternity Unit to the Neonatology Unit of the CSREF in Commune V of Bamako District, Mali

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Open Journal of pediatrics 2024年第3期14卷 547-557页

作者： Maimouna Kante Ya Traoré Balilé Harber Beydari Mamadou Traoré Issiaka Koné Diéneba Sacko Maimouna Haidara Korotoumou Bamba Assitan Farota Abdoulaye Kassogué Mohamed Diarra Dramane Touré Abdoul Salam Diarra Leyla Maïga Dicko Fatoumata Paediatric ward CSR&#233f CV Bamako Mali Paediatric ward of Nianankoro Fomba hospital S&#233gou Mali Paediatric ward of Kalaban CoroCSREF Bamako Mali Pediatric Department of the Mopti Reference Health Center Mopti Mali National Centre for Scientific and Technological Research Bamako Mali Paediatrics Department Mohamed VI Perinatal Clinic Bamako Mali Department of Paediatrics Gabriel Tour&#233 University Hospital Bamako Mali

Introduction: Neonatal mortality remains a major public health concern. According to the World Health Organisation (WHO), the number of newborns dying each year has fallen from 5 million in 1990 to 2.4 million in 2019. The aim of our study was to describe the main morbid conditions and their lethality in newborns admitted to the paediatric ward of the CSREF in Commune V. Methodology: We conducted a 12-month prospective descriptive and analytical study from August 2020 to July 2021. Results: During the 12 months of the study, out of 2654 neonates admitted to hospital, 216 met our inclusion criteria, i.e. 8%. Newborns admitted in the first few hours of life represented 99.54% of cases. Full-term newborns accounted for 65.74% of the total. Forty-eight percent of newborns had hypothermia on admission. The most common diagnosis was asphyxia in 55.56% of cases, followed by neonatal infection in 27.78% and prematurity in 10.65%. Death was recorded in 22.2% of cases. Asphyxia was the main cause of neonatal death, followed by prematurity in our context. Conclusion: Improving the prognosis of newborn babies will require a thorough understanding of neonatal pathologies and the implementation of a pre- and perinatal prevention policy.

关键词： Newborns Morbidity Mortality

Low-dose immune tolerance induction for severe hemophilia A inhibitor patients: Immunosuppressants are generally not necessary for inhibitor-titer below 200 BU/mL

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儿科学研究(英文) 2024年第2期08卷 91-100页

作者： Li Zhengping Sun Jie Li Zekun Chen Zhenping Liu Guoqing Yao Wanru Cheng Xiaoling Li Gang Zhen Yingzi Ai Di Zhou Yaohan Mao Qianqian Poon Man-Chiu Wu Runhui Hemophilia Comprehensive Care Center Hematology Department Hematology Center Beijing Key Laboratory of Pediatric Hematology-Oncology Key Laboratory of Major Diseases in Children National Key Discipline of Pediatrics (Capital Medical University) Ministry of Education Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China Hematologic Disease Laboratory Hematology Department Hematology Center Beijing Key Laboratory of Pediatric Hematology-Oncology Key Laboratory of Major Diseases in Children National Key Discipline of Pediatrics (Capital Medical University) Ministry of Education Beijing Pediatric Research Institute Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China Department of Pharmacy Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China Departments of Medicine Pediatrics and Oncology University of Calgary Cumming School of Medicine Calgary Canada

Importance: It remained unclear that the efficacy comparison between low-dose immune tolerance induction (LD-ITI) incorporating immunosuppressants (IS) when severe hemophilia A (SHA) patients had inhibitor-titer ≥200 Bethesda Units (BU)/mL (LD-ITI-IS200 regimen) and LD-ITI combining with IS when SHA patients had inhibitor-titer ≥40 BU/mL (LD-ITI-IS40 regimen).Objective: To compare the efficacy of the LD-ITI-IS200 regimen with that of the LD-ITI-IS40 regimen for SHA patients with high-titer ***: A prospective cohort study on patients receiving LD-ITI-IS200 compared to those receiving LD-ITI-IS40 from January 2021 to December 2023. Both received LD-ITI [FVIII 50 IU/kg every other day]. IS (rituximab+ prednisone) was added when peak inhibitor tier ≥200 BU/mL in the LD-ITI-IS200 regimen and ≥40 BU/mL in the LD-ITI-IS40 regimen. Success is defined as a negative inhibitor plus FVIII recovery ≥66% of the ***: We enrolled 30 patients on LD-ITI-IS200 and 64 patients on LD-ITI-IS40, with similar baseline clinical characteristics. A lower IS-use rate was discovered in the LD-ITI-IS200 regimen compared to the LD-ITI-IS40 regimen (30.0% vs. 62.5%). The two regimens (LD-ITI-IS200 vs. LD-ITI-IS40) had similar success rate (70.0% vs. 79.7%), median time to success (9.4 vs. 10.6 months), and annualized bleeding rate during ITI (3.7 vs. 2.8). The cost to success was lower for LD-ITI-IS200 than for LD-ITI-IS40 (2107 vs. 3256 US Dollar/kg). Among patients with peak inhibitor-titer 40-199 BU/mL, 10 non-IS-using (on LD-ITI-IS200 regimen) and 28 IS-using (on LD-ITI-IS40 regimen) had similar success rates (70.0% vs. 78.6%) and time to success (9.0 vs. 8.8 months).Interpretation: In LD-ITI, IS are not necessary for inhibitor titer <200 BU/mL.

关键词： Hemophilia A High-titer inhibitor Immune tolerance induction Immunosuppressant Low-dose

Attitudes and Practices of Lumbar Puncture among Students, Interns, and Residents of the Pediatric Department of the Mohammed VI University hospital Center of Oujda, Morocco

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Open Journal of pediatrics 2024年第3期14卷 598-608页

作者： Hasnae Elhaddadi Ayyad Ghanam Hind Zahiri Amal Hamami Aziza Elouali Abdeladim Babakhouya Maria Rkain Department of pediatrics University Hospital Center Mohammed VI Oujda Morocco Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco

Introduction: Even though lumbar puncture (LP) represents an important tool in the diagnosis of certain neurological diseases, this procedure is little practiced by our students. We aimed to evaluate the attitudes and practices of students, interns, and residents about LP, and to assess their feelings about how this procedure is taught. Materials and Methods: We conducted a cross-sectional study of 160 participants, using an anonymous questionnaire, to evaluate the attitudes and practices of students, interns and residents in the pediatrics department concerning lumbar puncture. Results: Half of the participants had never performed LP, usually because of the risk involved or lack of confidence, while 20% had performed it more than 4 times. None of the participants had learned to perform the procedure through simulation sessions, while (42%) had learned it under the supervision of a senior physician and had not been able to perform it successfully the first time. Most participants inform the patient about the procedure before performing it. Only 44% of participants acknowledged that LP can be performed under local anesthetic. The sitting position (60%) was by far the most commonly used. Most LPs were performed for diagnostic purposes. Most participants stated that the pediatric ward and the pediatric emergency department are among the departments that perform LP most frequently, and that they would be interested in taking part in simulated lumbar puncture sessions in children in the future. Conclusion: The results of our study show that LP is perceived by students as a risky procedure that is difficult to perform. Teachers should reconsider how this technical procedure is taught, by integrating simulation on mannequins into student training.

关键词： Lumbar Puncture Learning Healthcare Simulation Stress Clinical Competency

Bithalamic Ischemic Stroke in Sickle Cell Patient at the Kamenge University hospital in Burundi: Case Report

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Open Journal of Medical Imaging 2024年第2期14卷 72-78页

作者： Sébastien Manirakiza Daniel Nduwayo Patrice Barasukana Chantal Murekatete Jean Bosco Bizimana Alice Ndayishimiye Léopold Nzisabira Hélène Bukuru Department of Radiology Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of Internal Medicine Neurology Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of Surgery Neurosurgery Kamenge University Hospital Center University of Burundi Bujumbura Burundi Department of pediatrics Kamenge University Hospital Center University of Burundi Bujumbura Burundi

Aim: To describe the clinical and radiological diagnostic for a sickle cell patient who got a bithalamic vasculature accident. Observation: This was a 4-year-old female child admitted to the pediatric ward for convulsive seizures in a feverish context. The physical examination did not find signs of meningeal irritation. The effective workup including thick gout, lumbar puncture, complete blood count, and C reactive protein was normal. An electrocencephalogram was not performed due to lack of availability of the device. Based on clinical data and the fact that Burundi is an area of high malaria prevalence, antimalarial and anticonvulsant treatment has been started. An alteration of consciousness (the Glasgow score of 7) had motivated a CT scan. Bithalamic hypodensities in favor of a vascular accident have been demonstrated. Etiological investigation revealed homozygous sickle cell disease ss during hemoglobin electrophoresis. The final evolution has been characterized by a return to normal consciousness. Conclusion: The complexity of the clinical picture following occlusion of Percherons artery makes it difficult to diagnose. Sickle cell disease may favour the occurrence of ischaemic lesions. Cerebral imaging enables the diagnosis to be made with certainty and avoids delays in treatment.

关键词： Stroke Bithalamic Infarction Sickel Cell Percheron Artery

Ketogenic diet therapy in children with epilepsy caused by SLC2A1 mutations:a single-center single-arm retrospective study

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World Journal of pediatrics 2024年第5期20卷 517-524页

作者： Ying-Yan Wang Yun-Qing Zhou Li-Juan Luo Cui-Jin Wang Nan Shen Hao Li Ji-Wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical Research ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective study assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by SLC2A1 genetic mutations and glucose transporter type 1 deficiency *** Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an SLC2A1 genetic mutation on whole-exome *** analyzed the patients’convulsions and treatment with antiepileptic *** patients were followed up at different time periods after ketogenic diet *** Six patients with SLC2A1 mutations were included in this *** patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their *** were then treated with a ketogenic diet for at least four *** analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet *** patients were seizure-free within a month of receiving the diet *** patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this *** antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment *** severe adverse events occurred during the *** Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by SLC2A1 ***,patients with glucose transporter type 1 deficiency syndrome caused by SLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： Epilepsy Glucose transporter type 1 Ketogenic diet SLC2A1