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Impact of virtual reality on the perception of procedural pain in paediatric oncology. 虚拟现实对儿科肿瘤手术疼痛感的影响。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4934
Elhem Jbebli, Yosra Jbeli, Rawia Ben Farhat, Samar Rhayem, Sarra Ben Ahmed, Rym Amdouni, Faten Fedhila Ben Ayed, Monia Khemiri

Introduction: Virtual reality is being used more and more in the healthcare field, particularly during treatment. In the context of pain management, the question arises as to the effectiveness of using virtual reality during care in reducing the perception of procedural pain.

Aim: To study the impact of using virtual reality on the perception of procedural pain and on the course of care in pediatric oncology.

Methods: A quasi-experimental, matched-case-control study conducted at the pediatric oncology unit of the Tunis Children's Hospital over a three-month period. Each child had two nursing care : with and without the use of virtual reality.

Results: Thirty-two children were enrolled. The sex ratio was 0.88. The edia nage was 96 ± 49 months. The care provided was peripheral venous line insertion (53%), blood sampling (32%) and port-a-catheter puncture (15%). The use of virtual reality significantly reduced heart rate (p<0.0001), respiratory rate (p<0.0001) and pain perception (p<0.0001). Workload was reduced, as evidenced by a drop in the duration of nursing care (p<0.0001) and in the number of people needed to carry out care (p<0.0001).

Conclusions: Virtual reality is a good non-pharmacological alternative for procedural pain control in a pediatric oncology unit.

导言:虚拟现实技术在医疗保健领域的应用越来越广泛,尤其是在治疗过程中。目的:研究虚拟现实技术对儿科肿瘤治疗过程中疼痛感的影响以及对治疗过程的影响:在突尼斯儿童医院儿科肿瘤科进行了一项为期三个月的准实验、匹配病例对照研究。结果:32 名儿童参加了研究:结果:32 名儿童参加了研究。性别比为 0.88。年龄为 96 ± 49 个月。提供的护理包括外周静脉置管(53%)、抽血(32%)和导管穿刺(15%)。使用虚拟现实技术大大降低了心率(p结论:虚拟现实技术是儿科肿瘤科控制手术疼痛的一种良好的非药物替代方法。
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引用次数: 0
Incidence and risk factors of venous thromboembolism in patients with amyotrophic lateral sclerosis: a systematic review and meta-analysis. 肌萎缩侧索硬化症患者静脉血栓栓塞症的发病率和风险因素:系统回顾和荟萃分析。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.5154
Abdullah Ashraf Hamad, Ibraheem M Alkhawaldeh, Abdallah Abbas, Ahmed Elaraby, Mostafa Meshref

Aims: This systematic review and meta-analysis aimed to determine the annual incidence rate of venous thromboembolism (VTE) and identify risk factors of VTE in amyotrophic lateral sclerosis (ALS) patients.

Methods: A comprehensive search of three databases was conducted up to April 8, 2024, to identify longitudinal studies reporting VTE incidence in ALS patients. The included studies were either prospective or retrospective, following up with ALS patients. Quality assessment was performed using the NIH tool for observational cohort studies. Meta-analysis was conducted using Open Meta Analyst, employing a random-effect model. Subgroup, Meta-regression, and sensitivity analyses were also carried out.

Results: Our analysis included eight studies comprising a total of 26,758 ALS patients that met the inclusion criteria. The pooled annual incidence of VTE across all studies was found to be 22 cases per 1,000 person-year (95% CI = 18 to 27). Subgroup analysis revealed that the annual incidence of VTE in males was 19 cases per 1,000 person-year (95% CI = 15 to 22), while in females, it was 20 cases per 1,000 person-year (95% CI = 16 to 25). Leave-one-out analysis demonstrated that the incidence ranged from 21 to 28 cases per 1,000 person-year when excluding each study individually. Meta-regression analysis did not find a significant association between age and the risk of VTE (P = 0.079). Based on the included studies, risk factors of VTE in ALS patients included a history of VTE, non-invasive ventilation, immobility, and decreased functional status.

Conclusion: Patients with ALS face a higher risk of developing VTE compared to individuals of the same age. These findings underscore the importance of implementing preventive measures and closely monitoring VTE in ALS patients.

目的:本系统综述和荟萃分析旨在确定肌萎缩侧索硬化症(ALS)患者静脉血栓栓塞症(VTE)的年发病率,并识别VTE的风险因素:对截至 2024 年 4 月 8 日的三个数据库进行了全面检索,以确定报告 ALS 患者 VTE 发生率的纵向研究。纳入的研究均为前瞻性或回顾性研究,对 ALS 患者进行随访。采用 NIH 观察性队列研究工具进行质量评估。采用随机效应模型,使用 Open Meta Analyst 进行 Meta 分析。此外,还进行了分组分析、元回归分析和敏感性分析:我们的分析纳入了八项研究,共有 26758 名 ALS 患者符合纳入标准。所有研究汇总的 VTE 年发病率为每千人年 22 例(95% CI = 18 至 27)。亚组分析显示,男性的 VTE 年发病率为每千人年 19 例(95% CI = 15 至 22),女性为每千人年 20 例(95% CI = 16 至 25)。剔除分析表明,当剔除每项研究时,发病率为每千人年 21 至 28 例。元回归分析未发现年龄与 VTE 风险之间存在显著关联(P = 0.079)。根据纳入的研究,ALS 患者发生 VTE 的风险因素包括 VTE 病史、无创通气、行动不便和功能状态下降:结论:与同龄人相比,ALS 患者发生 VTE 的风险更高。这些发现强调了对 ALS 患者实施预防措施和密切监测 VTE 的重要性。
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引用次数: 0
GAIA therapeutic farm: Prospective study on multidisciplinary care efficacy. GAIA 治疗农场:多学科护理疗效的前瞻性研究。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4959
Soumaya Halayem, Faten Chennoufi, Uta Ouali, Yosr Lajmi, Raja Moumni, Asma Bouden, Leila Guesmi

Introduction: The therapeutic farm "GAIA" in Sidi Thabet is a non-profit organization created in 2009, situated in the North-Western suburbs of the capital Tunis/Tunisia. Its main aims are the day-care for and the socio-professional integration of children and young adults between age 6 and 30 with diverse types of disability through multiple activities, such as pet assisted therapy and horticulture.

Aim: to assess the potential benefits of therapeutic on-farm management.

Methods: We conducted a prospective study including 50 children and young adults aged 7 to 30 years with intellectual disabilities and/or autism spectrum disorders. Study participants were evaluated before inclusion and after 10 months of care using a self-established questionnaire collecting socio-demographic and clinical variables, an evaluation of competences, and the Child Behavior Checklist (CBCL).

Results: Mean sub scores (externalization/internalization) of CBCL decreased significantly (p<0.002). Competences were significantly improved in the following domains: understanding of verbal language, gross and fine motor skills, body hygiene, spatial and temporal orientation, preschool and school learning achievements and social integration.

Conclusions: These positive initial results should encourage the further development of this initiative with support of the community.

简介西迪塔贝特(Sidi Thabet)"GAIA "治疗农场是一个非营利组织,成立于 2009 年,位于突尼斯首都西北郊。其主要目标是通过宠物辅助治疗和园艺等多种活动,为 6 至 30 岁患有不同类型残疾的儿童和年轻人提供日托服务,并帮助他们融入社会职业生活:我们开展了一项前瞻性研究,研究对象包括 50 名 7 至 30 岁患有智力障碍和/或自闭症谱系障碍的儿童和青少年。研究参与者在入组前和接受护理 10 个月后接受了评估,评估使用的是一份自制的调查问卷,其中收集了社会人口学和临床变量、能力评估以及儿童行为检查表(CBCL):结果:儿童行为检查表(CBCL)的平均分(外化/内化)明显下降(p):这些积极的初步结果应鼓励在社区的支持下进一步发展这一举措。
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引用次数: 0
Prevention of perioperative anxiety: Interest of audiovisual distraction. 预防围手术期焦虑:视听分散的兴趣
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4468
Faten Haddad, Sirine Rais, Hajer Arfaoui, Emna Kammoun, Skander Naimi, M'hamed Sami Mebazaa

Introduction: Audiovisual distraction (AVD) is one of the non-pharmacological means of anxiety prevention. However, few studies have evaluated its perioperative effect in adults.

Aim: To evaluate the contribution of audio-visual distraction in the prevention of perioperative anxiety in adult patients proposed for surgery under spinal anesthesia.

Methods: This was a prospective randomized controlled study lasting three months from July 1, 2021. We included ASA I to III over 18 years patients, proposed for visceral or orthopedic surgery under spinal anesthesia. We didn't include patients operated in lateral or prone positions or with a history of anxiety disorders or communication difficulties. 90 patients were randomized into two groups: a control group T and a group A who had an AVD throughout the operation. The primary outcome was anxiety as assessed by the visual analogue scale (VAS).

Results: Demographic characteristics, Amsterdam preoperative anxiety and information scale (APAIS) and pain levels were comparable between the two groups. During the surgery, 22 (48.9 %) patients in group T versus 3 (6.7 %) in group A required Midazolam (p < 0.001). Anxiety assessed by VAS at the end of the act was lower in group A (p < 0.001). The pain levels also were lower in patients who received AVD (p = 0.004). A patient satisfaction score (Iowa Satisfaction with Anesthesia Scale French version) ≥ 5.4 was more frequently observed in group A (p < 0.001).

Conclusion: AVD reduced perioperative anxiety and decreased the use of midazolam during surgery performed under spinal anesthesia.

简介视听分散法(AVD)是预防焦虑的非药物疗法之一。目的:评估视听分散法在预防拟接受脊髓麻醉手术的成年患者围术期焦虑中的作用:这是一项前瞻性随机对照研究,从 2021 年 7 月 1 日开始,为期三个月。研究对象包括 ASA I 至 III 级、年满 18 岁、拟在脊髓麻醉下进行内脏或骨科手术的患者。我们不包括侧卧位或俯卧位手术的患者,也不包括有焦虑症或交流障碍病史的患者。90名患者被随机分为两组:对照组T和A组,A组在整个手术过程中都有AVD。主要结果是通过视觉模拟量表(VAS)评估焦虑程度:两组的人口统计学特征、阿姆斯特丹术前焦虑和信息量表(APAIS)以及疼痛程度相当。在手术过程中,T 组有 22 名患者(48.9%)需要服用咪达唑仑,而 A 组只有 3 名患者(6.7%)(P < 0.001)。根据 VAS 评估,A 组患者在手术结束时的焦虑程度较低(P < 0.001)。接受 AVD 的患者疼痛程度也较低(p = 0.004)。A组患者的满意度评分(爱荷华麻醉满意度量表法文版)≥5.4的比例更高(p < 0.001):结论:在脊髓麻醉下进行手术时,AVD 可减少围术期焦虑,并减少咪达唑仑的使用。
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引用次数: 0
Atypical presentation of systemic lupus erythematosus in a 16-year-old boy: Case report. 一名 16 岁男孩系统性红斑狼疮的非典型表现:病例报告。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.5065
Hela Sahbani, Marwa Bahri, Emna Azza, Yosr Ben Abdennebi, Lamia Aissaoui

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, characterized by a clear female predominance and a major clinical polymorphism. Some initial presentations, particularly in children and adolescents are exceptional. Our objective was to report an unusual initial presentation of SLE in a male adolescent.

Observation: We reported the case of a 16-year- old tunisian boy who was referred to the hematology emergency department because of a hemorrhagic syndrome. The evolution was marked by pancytopenia, prolonged fever and an anasarca with recurrent cardiac tamponade. Explorations concluded to SLE. The patient was started on pulse therapy of dexamethasone followed by immunoglobulin. He was also provided with pericardiocentesis with a favorable outcome.

Conclusion: SLE is characterized by clinical polymorphism, that's why diagnosis and management of complications could be difficult. SLE should be considered in differential diagnosis of many disorders, such as pancytopenia and cardiac tamponade.

导言系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,其特点是女性患者明显占多数,临床表现多态。有些患者,尤其是儿童和青少年患者的初始症状非常特殊。我们的目的是报告一名男性青少年系统性红斑狼疮不寻常的初始表现:我们报告的病例是一名 16 岁的突尼斯男孩,他因出血性综合征被转到血液科急诊。该病例的特征是泛发热、长期发热和伴有反复心脏填塞的哮喘。检查结果为系统性红斑狼疮。患者开始接受地塞米松脉冲治疗,随后注射免疫球蛋白。他还接受了心包穿刺术,结果良好:结论:系统性红斑狼疮的特点是临床多态性,因此并发症的诊断和处理可能会很困难。在许多疾病的鉴别诊断中都应考虑系统性红斑狼疮,如泛发性心肌炎和心脏填塞。
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引用次数: 0
Epidemiology of intestinal parasitosis in schoolchildren in the Moughatâa of Riyadh (Nouakchott). 利雅得 Moughatâa(努瓦克肖特)学龄儿童肠道寄生虫病流行病学。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.5044
Ousmane Ba, Ousmane Sy, Abbah Mohamed Mbareck, Sidya Mohamed Issa, Mamadou Fofana, Wafi Sidi Baba

Introduction: Intestinal parasitoses are very common in school and in Mauritania few external studies address the issue. Several risk factors are known for intestinal parasitosis, including fecal danger, promiscuity and contaminated food Objective: To describe the epidemiology of intestinal parasitosis in school-age children in the Moughatâa (department) of Riyadh in Nouakchott (Mauritania) and the associated risk factors.

Methods: This was a descriptive cross-sectional study lasting three months, at the level of a random sample of pupils enrolled in five primary schools in the Moughatâa of Riyadh, as well as the foods sold in front of the establishments included. The data was collected through an anamnestic and clinical observation grid, as well as stool and food samples exposed in front of the schools included. The parasitological examination of the stools was carried out at the National Institute of Public Health Research (INRSP) in Nouakchott, by the methods of modified Ritchie and Kato-Katz. For food, a microbiological analysis looking for fecal colibacilli is carried out.

Results: The study population was composed of 337 students with a sex ratio of 0.76. The prevalence of parasitosis in the study population was 46% (95% CI [40.75%-51.33%]): Boys (48.6%), 95% CI [40.66%-56.67%]); Girls (43.9%), 95% CI [37.13%-51.07%]. The spectrum of these parasitic intestinal diseases was dominated by protozoa (51.6%) and helminthiasis represented 10.4% and characterized by mono parasitism (31.8%). The food sold in front of the schools studied was contaminated with coli bacteria, in 54.5% of cases.

Conclusion: The high prevalence of intestinal parasitosis in the primary schools studied and the contamination of food sold to students, require the strengthening of the strategy to fight against diseases of fecal peril and school and public hygiene, in the Moughatâa of Riyadh (Nouakchott).

导言:肠道寄生虫病在学校非常常见,而在毛里塔尼亚,很少有外部研究涉及这一问题。肠道寄生虫病有几个已知的危险因素,包括粪便危险、乱交和受污染的食物:描述努瓦克肖特(毛里塔尼亚)利雅得 Moughatâa(省)学龄儿童肠道寄生虫病的流行病学及其相关风险因素:这是一项为期三个月的描述性横断面研究,随机抽取了利雅得 Moughatâa 五所小学的在校学生以及学校门口出售的食品。数据是通过肛诊和临床观察网格以及在所含学校门口暴露的粪便和食物样本收集的。粪便的寄生虫学检查在努瓦克肖特国家公共卫生研究所(INRSP)进行,采用改良里奇和卡托-卡茨的方法。对食物则进行了微生物分析,以寻找粪便中的大肠杆菌:研究对象包括 337 名学生,男女比例为 0.76。研究人群中寄生虫病的发病率为 46%(95% CI [40.75%-51.33%]):男生(48.6%),95% CI [40.66%-56.67%]);女生(43.9%),95% CI [37.13%-51.07%]。这些寄生虫性肠道疾病中,原虫病占 51.6%,蠕虫病占 10.4%,单寄生虫病占 31.8%。在所研究的学校门口出售的食品中,54.5%受到大肠杆菌污染:结论:在所研究的小学中,肠道寄生虫病的发病率很高,出售给学生的食物也受到污染,因此需要在利雅得(努瓦克肖特)的 Moughatâa 加强防治粪便危害疾病的战略以及学校和公共卫生。
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引用次数: 0
Papillary optical coherence tomography angiography in optic neuropathies. 视神经病变中的乳头状光学相干断层血管造影。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4986
Dhouha Nefzi, Lotfi Chaabani, Arij Jouini, Nesrine Abroug, Imen Ksiaa, Moncef Khairallah

Aim: to describe and analyze peripapillary microvascular changes in eyes with optic neuropathies using optical coherence tomography angiography (OCTA).

Methods: In this cross-sectional observational study, 63 eyes of 48 patients with different causes of optic neuropathy and 36 healthy control eyes were evaluated by optical coherence tomography (OCT) and OCTA (Topcon DRI OCT; Triton) between January 2018 and December 2018 at the Fattouma Bourguiba ophthalmology department in Monastir.

Results: Vascular changes were more frequent in acute non arteritic anterior ischemic optic neuropathy (NAION) than in optic neuritis. Eyes with optic atrophy had capillary rarefaction corresponding to the area of thinning of the peripapillary optic fiber layer (RNFL). The peripapillary vascular density (PVD) was lower (p=0.036) in case of optic atrophy compared to normal eyes. For non arteritic ischemic optic neuropathy and for chronic retrobulbar optic neuritis (NORB), a positive correlation between visual acuity and PVD was observed (p<0.05). An inversely proportional correlation between the PVD and the duration of evolution was noted. In the multivariate study, high blood pressure was a systemic factor correlated with peripapillary microvascular damages.

Conclusion: OCTA showed a reduction in PVD and greater vascular changes in acute non-arteritic ischemic optic neuropathy than in edematous optic neuritis. At the atrophic stage, the PVD was reduced. Hypertension was an independent factor correlated with peripapillary microvascular damages.

目的:使用光学相干断层血管成像(OCTA)描述和分析视神经病变患者眼球周围微血管的变化:在这项横断面观察研究中,2018年1月至2018年12月期间,莫纳斯提尔的法图马-布尔吉巴眼科通过光学相干断层扫描(OCT)和OCTA(拓普康DRI OCT;Triton)对48名不同病因的视神经病变患者和36名健康对照眼的63只眼睛进行了评估:与视神经炎相比,急性非动脉炎性前部缺血性视神经病变(NAION)的血管病变更为常见。视神经萎缩患者的毛细血管稀疏,与毛细血管周围视纤维层(RNFL)变薄的区域相对应。与正常眼相比,视神经萎缩患者的毛细血管密度(PVD)更低(P=0.036)。非动脉缺血性视神经病变和慢性球后视神经炎(NORB)的视力与 PVD 呈正相关(P=0.036):与水肿性视神经炎相比,OCTA 显示急性非动脉缺血性视神经病变的 PVD 有所减少,血管变化更大。在萎缩阶段,PVD 有所减少。高血压是与毛细血管周围微血管损伤相关的一个独立因素。
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引用次数: 0
Knowledge and perception of asthma among parents of children with asthma. 哮喘患儿家长对哮喘的认识和看法。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4856
Fatma Khalsi, Hajer Mansouri, Soumaya Kbaier, Imen Belhadj, Khedija Boussetta

Introduction: Parents' knowledge is an integral part of healthcare quality, impacting treatment adherence, patient loyalty and healthcare utilisation in pediatric asthma. Parental knowledge is particularly crucial as parents influence decision-making for their child's healthcare.

Aim: To assess parents' knowledge and perceptions of their children's asthma and to identify areas for weakness in therapeutic education.

Methods: This is transversal survey study based on validated and translated self-administered questionnaire (Arabic asthma knowledge questionnaire AAKQ). The study was conducted in 2 departments: pediatric primary care department at Elomrane and pediatric pneumology outpatient clinics at Bechir Hamza children's hospital of Tunis during 6 months (March2022- October2022).

Results: One hundred forty-four parents were involved in the study. Most of the participants were mothers. The mean age was 38 years. Only 27,0% had higher education and smoking in parents was observed in 58% of cases. Our results showed moderate knowledge in 67,9% of participants. The mean of the total knowledge score was 57,7/85. Forty-six per cent of parents had poor knowledge for the first category of the questionnaire. The second category was the most successful part where only 14% had poor knowledge. The mean score of the third category was 13,5/20 with 35,4% of parents having poor knowledge. The mean of total knowledge was found to be higher among participants with higher education (P=0,000), when the gender of the child is masculine (P=0,038), when the parents were asked in Hospital of children of Tunis (P=0,003) and when there is a history of asthma in the siblings (P=0,05). No difference in caregiver's knowledge was based on their age or gender or history of asthma, on the age of the child, his hospitalization for asthma, his asthma control level or his compliance with medication.

Conclusion: Knowledge of parents and their children suffering from asthma is acceptable but insufficient. An approach focusing on improving patient education skills is needed to help families understand and accept the disease and achieve optimal control.

导言:家长的知识是医疗质量不可或缺的一部分,影响着儿科哮喘的治疗依从性、患者忠诚度和医疗利用率。目的:评估家长对子女哮喘的了解和看法,找出治疗教育的薄弱环节:这是一项横向调查研究,以经过验证和翻译的自填式问卷(阿拉伯语哮喘知识问卷 AAKQ)为基础。研究在两个部门进行:埃洛姆兰的儿科初级保健部门和突尼斯贝希尔-哈姆扎儿童医院的儿科肺病门诊,为期6个月(2022年3月至2022年10月):144名家长参与了研究。大多数参与者是母亲。平均年龄为 38 岁。只有 27.0% 的人受过高等教育,58% 的父母吸烟。我们的结果显示,67.9%的参与者具有中等知识水平。知识总分的平均值为 57.7/85。46%的家长对问卷中的第一类知识知之甚少。第二类是最成功的部分,仅有 14% 的家长对其了解较少。第三类的平均分是 13.5/20,35.4%的家长知识贫乏。研究发现,受教育程度较高(P=0,000)、儿童性别为男性(P=0,038)、父母在突尼斯儿童医院接受询问(P=0,003)以及兄弟姐妹有哮喘病史(P=0,05)的参与者的总知识平均值较高。照顾者对哮喘知识的了解与他们的年龄、性别或哮喘病史、孩子的年龄、因哮喘住院治疗的情况、哮喘控制水平或用药依从性没有关系:结论:哮喘患者的父母及其子女对哮喘的了解是可以接受的,但还不够。结论:哮喘患者的父母及其子女对哮喘的了解是可以接受的,但还不够。需要采取一种侧重于提高患者教育技能的方法,帮助家庭了解和接受这种疾病,并达到最佳的控制效果。
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引用次数: 0
Phenotypes of 126 Moroccan HIES patients according to NIH Score. 根据 NIH 评分得出的 126 名摩洛哥 HIES 患者的表型。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.5148
Ilham Fadil, Ibtihal Benhsaien, Jalila El Bakkouri, Leila Jeddane, Noufissa Benaajiba, Noureddine Rada, Mohamed Hbibi, Naïma Amenzoui, Sara Ben Miloud, Mustapha Hida, Mohammed Bouskraoui, Fatima-Zahra El Fetoiki, Fouzia Hali, Soumiya Chiheb, Brahim Admou, Jean Laurent Casanova, Anne Puel, Bertrand Boisson, Vivien Beziat, Fatima Ailal, Ahmed Aziz Bousfiha

Introduction: Hyper-IgE syndrome is a group of inborn errors of immunity, some of which are syndromic, characterized clinically by the classic triad of chronic eczema, cutaneous and/or pulmonary staphylococcal infections and high serum IgE concentrations (> 2000 IU/ml or > 10 x normal for age).

Aim: We report here the clinical and immunological aspects of Moroccan patients presenting probable or possible HIES according to NIH-HIES score.

Methods: This retrospective study covers the period from 1998 to 2023 and includes Moroccan patients with a clinical presentation suggestive of HIES (skin and/or pulmonary infections, eczema, high IgE levels) and an NIH score ≥ 20. We attempted to classify the patients phenotypically according to the 2022 IUIS IEI Expert Committee classification.

Results: Median age at symptom onset was 0.5 years and median age at diagnosis was 5.5 years. The main clinical signs were eczema (66%), skin abscesses (32.5%), pneumonia (32.5%), otitis (20%), mucocutaneous candidiasis (19%), diarrhea (12%), facial dysmorphism (10.3%), lymphadenopathy (9.5%), bronchial dilation (8%), pneumatoceles (8%), conjunctivitis (7.1%), rhinitis (6.3%), psychomotor delay (5.6%), pathological fractures (4%), retention of deciduous teeth (4%), cognitive delay (3.2%).

Conclusion: This is the first clinical description of a cohort of Moroccan patients presenting HIES according to NIH criteria. Phenotype can sometimes orient towards identification of the mutated gene, but the overlapping clinical signs make molecular analysis necessary for genetic counseling and appropriate treatment.

导言:高IgE综合征是一组先天性免疫错误,其中一些是综合征,临床特征是慢性湿疹、皮肤和/或肺部葡萄球菌感染以及高血清IgE浓度(> 2000 IU/ml或>正常年龄的10倍)的典型三联征:这项回顾性研究的时间跨度为 1998 年至 2023 年,研究对象包括临床表现提示 HIES(皮肤和/或肺部感染、湿疹、高 IgE 水平)且 NIH 评分≥ 20 分的摩洛哥患者。我们试图根据 2022 年 IUIS IEI 专家委员会的分类对患者进行表型分类:中位发病年龄为 0.5 岁,中位确诊年龄为 5.5 岁。主要临床表现为湿疹(66%)、皮肤脓肿(32.5%)、肺炎(32.5%)、耳炎(20%)、皮肤粘膜念珠菌病(19%)、腹泻(12%)、面部畸形(10.3%)、淋巴结病(9.5%)、支气管扩张(8%)、气胸(8%)、结膜炎(7.1%)、鼻炎(6.3%)、精神运动发育迟缓(5.6%)、病理性骨折(4%)、乳牙滞留(4%)、认知发育迟缓(3.2%):这是首次根据美国国立卫生研究院的标准对摩洛哥 HIES 患者进行临床描述。表型有时可引导突变基因的鉴定,但临床症状的重叠使分子分析成为遗传咨询和适当治疗的必要条件。
{"title":"Phenotypes of 126 Moroccan HIES patients according to NIH Score.","authors":"Ilham Fadil, Ibtihal Benhsaien, Jalila El Bakkouri, Leila Jeddane, Noufissa Benaajiba, Noureddine Rada, Mohamed Hbibi, Naïma Amenzoui, Sara Ben Miloud, Mustapha Hida, Mohammed Bouskraoui, Fatima-Zahra El Fetoiki, Fouzia Hali, Soumiya Chiheb, Brahim Admou, Jean Laurent Casanova, Anne Puel, Bertrand Boisson, Vivien Beziat, Fatima Ailal, Ahmed Aziz Bousfiha","doi":"10.62438/tunismed.v102i10.5148","DOIUrl":"10.62438/tunismed.v102i10.5148","url":null,"abstract":"<p><strong>Introduction: </strong>Hyper-IgE syndrome is a group of inborn errors of immunity, some of which are syndromic, characterized clinically by the classic triad of chronic eczema, cutaneous and/or pulmonary staphylococcal infections and high serum IgE concentrations (> 2000 IU/ml or > 10 x normal for age).</p><p><strong>Aim: </strong>We report here the clinical and immunological aspects of Moroccan patients presenting probable or possible HIES according to NIH-HIES score.</p><p><strong>Methods: </strong>This retrospective study covers the period from 1998 to 2023 and includes Moroccan patients with a clinical presentation suggestive of HIES (skin and/or pulmonary infections, eczema, high IgE levels) and an NIH score ≥ 20. We attempted to classify the patients phenotypically according to the 2022 IUIS IEI Expert Committee classification.</p><p><strong>Results: </strong>Median age at symptom onset was 0.5 years and median age at diagnosis was 5.5 years. The main clinical signs were eczema (66%), skin abscesses (32.5%), pneumonia (32.5%), otitis (20%), mucocutaneous candidiasis (19%), diarrhea (12%), facial dysmorphism (10.3%), lymphadenopathy (9.5%), bronchial dilation (8%), pneumatoceles (8%), conjunctivitis (7.1%), rhinitis (6.3%), psychomotor delay (5.6%), pathological fractures (4%), retention of deciduous teeth (4%), cognitive delay (3.2%).</p><p><strong>Conclusion: </strong>This is the first clinical description of a cohort of Moroccan patients presenting HIES according to NIH criteria. Phenotype can sometimes orient towards identification of the mutated gene, but the overlapping clinical signs make molecular analysis necessary for genetic counseling and appropriate treatment.</p>","PeriodicalId":38818,"journal":{"name":"Tunisie Medicale","volume":"102 10","pages":"696-701"},"PeriodicalIF":0.0,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11574375/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142509777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Changes in echocardiographic parameters after hemodialysis session in a North African pediatric population with end-stage renal disease and without known heart disease. 患有终末期肾病且无已知心脏病的北非儿童血液透析后超声心动图参数的变化。
Q3 Medicine Pub Date : 2024-10-05 DOI: 10.62438/tunismed.v102i10.4967
Faten El Ayech Boudiche, Malek Elarbi, Selim Boudiche, Taha Sayari, Hakim Ben Jemaa, Ahmed Chetoui, Habib Ben Ahmed, Wejdene Ouechtati, Emna Allouche, Tahar Gargah, Leila Bezdah

Introduction: Children undergoing long-term hemodialysis (HD) face a reduction in life expectancy mostly due to cardiovascular mortality. Effects of HD on cardiac function have not been fully elucidated in pediatric population.

Aim: This study aimed to assess HD session impact on cardiac function in pediatric patients using conventional and strain echocardiography.

Methods: We performed a prospective, comparative study of echocardiographic parameters before and after single HD session in a chronic HD pediatric population. We enrolled between the 1st and 30th September 2023, all consecutive patients with end-stage renal disease (ESRD) aged up to 18 years old on maintenance HD three times weekly for at least three months. All patients underwent conventional and left ventricular (LV) longitudinal strain echocardiography in a window of 30-60 minutes before and after HD.

Results: 23 patients, 14.8 ± 2.1 years old and 47.8% male, were enrolled. Reductions in body weight and blood pressure were observed after HD, whereas heart rate increased. Significant decrease in LV and left atrial diameters and volumes after HD session were observed. Mitral peak E velocity, as well as average E/e' were significantly lower after HD. Although LV ejection fraction was unchanged, global longitudinal strain for LV was significantly reduced after dialysis (-17.3 ± 3.0% vs. -14.9 ± 2.4%, p=4.10-8).

Conclusion: Patent deterioration in LV systolic function following HD was identified by speckle tracking echocardiography (STE). STE has the potential to unmask early myocardial dysfunction even when there is no evident alteration in conventional systolic function parameters in children with ESRD.

导言:长期接受血液透析(HD)的儿童面临着预期寿命缩短的问题,这主要是由于心血管疾病导致的死亡率。本研究旨在使用常规和应变超声心动图评估血液透析对儿童患者心脏功能的影响:我们在慢性 HD 儿科人群中开展了一项前瞻性、单次 HD 治疗前后超声心动图参数比较研究。我们在 2023 年 9 月 1 日至 30 日期间连续招募了所有年龄在 18 岁以下的终末期肾病(ESRD)患者,这些患者每周接受三次维持性 HD 治疗,至少持续三个月。所有患者均在血液透析前后 30-60 分钟内接受了常规和左心室纵向应变超声心动图检查。HD 后体重和血压下降,心率上升。HD治疗后,观察到左心室和左心房的直径和容积明显缩小。二尖瓣峰值 E 速度和平均 E/e' 在 HD 后明显降低。虽然左心室射血分数没有变化,但透析后左心室整体纵向应变明显降低(-17.3 ± 3.0% vs. -14.9 ± 2.4%,P=4.10-8):结论:斑点追踪超声心动图(STE)可发现透析后左心室收缩功能的早期恶化。即使ESRD患儿的常规收缩功能参数没有明显改变,STE也有可能揭示早期心肌功能障碍。
{"title":"Changes in echocardiographic parameters after hemodialysis session in a North African pediatric population with end-stage renal disease and without known heart disease.","authors":"Faten El Ayech Boudiche, Malek Elarbi, Selim Boudiche, Taha Sayari, Hakim Ben Jemaa, Ahmed Chetoui, Habib Ben Ahmed, Wejdene Ouechtati, Emna Allouche, Tahar Gargah, Leila Bezdah","doi":"10.62438/tunismed.v102i10.4967","DOIUrl":"10.62438/tunismed.v102i10.4967","url":null,"abstract":"<p><strong>Introduction: </strong>Children undergoing long-term hemodialysis (HD) face a reduction in life expectancy mostly due to cardiovascular mortality. Effects of HD on cardiac function have not been fully elucidated in pediatric population.</p><p><strong>Aim: </strong>This study aimed to assess HD session impact on cardiac function in pediatric patients using conventional and strain echocardiography.</p><p><strong>Methods: </strong>We performed a prospective, comparative study of echocardiographic parameters before and after single HD session in a chronic HD pediatric population. We enrolled between the 1st and 30th September 2023, all consecutive patients with end-stage renal disease (ESRD) aged up to 18 years old on maintenance HD three times weekly for at least three months. All patients underwent conventional and left ventricular (LV) longitudinal strain echocardiography in a window of 30-60 minutes before and after HD.</p><p><strong>Results: </strong>23 patients, 14.8 ± 2.1 years old and 47.8% male, were enrolled. Reductions in body weight and blood pressure were observed after HD, whereas heart rate increased. Significant decrease in LV and left atrial diameters and volumes after HD session were observed. Mitral peak E velocity, as well as average E/e' were significantly lower after HD. Although LV ejection fraction was unchanged, global longitudinal strain for LV was significantly reduced after dialysis (-17.3 ± 3.0% vs. -14.9 ± 2.4%, p=4.10-8).</p><p><strong>Conclusion: </strong>Patent deterioration in LV systolic function following HD was identified by speckle tracking echocardiography (STE). STE has the potential to unmask early myocardial dysfunction even when there is no evident alteration in conventional systolic function parameters in children with ESRD.</p>","PeriodicalId":38818,"journal":{"name":"Tunisie Medicale","volume":"102 10","pages":"628-634"},"PeriodicalIF":0.0,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11574370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142509863","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Tunisie Medicale
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