bluuebunny/crossref_metadata_2025_split huggingface dataset:
P019 Adulthood of juvenile idiopathic arthritis patients: professional outcome
Authors: Miladi S , Bouzid S , Fazaa A , Souabni L , Sellami M , Ouenniche K , Kassab S , Chekili S , Ben Abdelghani K , Laatar A 
Published: November 2021

Abstract

Background
Juvenile idiopathic arthritis (JIA) is a heterogeneous group of rheumatic diseases characterized by onset before the age of 16. Since the disease starts at an early age, it may lead to socio-professional difficulties in adulthood for JIA patients. This study aimed to describe the professional outcome of a series of 20 JIA patients.

Methods
A cross-sectional study including patients aged 20 years and more and fulfilling the ILAR criteria for the diagnosis of JIA was carried out. Telephone interviews were conducted. The responders answered questions about family status, current occupation, working h, eventual workplace adjustments, and sick leave frequency.

Results
Twenty patients answered the questionary; 14 males and 6 females. The mean age of the disease onset was 8 years. The mean age of patients at the time of the study was 24.27 years [20–36]. Polyarticular form was the most frequent (10 cases). Other subtypes diagnosed were systemic (4 cases), enthesitis-related arthritis (5 cases), oligoarticular (one case). Hip arthritis was observed in 8 patients and surgical intervention was needed in three. Eight patients were treated with csDMARDSs and 12 with bDMARDs.
Three of our patients were married (aged 24, 34, and 36). Five were still studying: 4 had good grades without absenteeism. However, one patient needed special aid to go to school and had a higher absence rate. Five other patients were full-time college students without absenteeism. Four patients were college graduates. Among them, two were searching for a job for &gt;6 months. The other two were full-time administrative workers with no absenteeism. One patient did need workplace adjustments. Six patients could not work because of their physical disabilities.

Conclusion
According to our results, a quarter of our patients could not access professional life. Disease activity and hip destruction are the two main factors causing JIA patients to miss out on important personal and professional opportunities.
P021 Children living with Juvenile Idiopathic Arthritis: their views about online resources and social media
Authors: Miri Sirine , Triki Wafa , Maatallah Kaouther , Hanene Ferjani , Ben Nessib Dorra , Kaffel Dhia , Hamdi Wafa 
Published: November 2021

Abstract

Background
Adolescents and children nowadays are quite accustomed to using the Internet for personal, social, and educational purposes. The purpose of our study was to determine the point of view of children with juvenile idiopathic arthritis (JIA) regarding the impact of social media on their lives.

Methods
We interviewed 23 children and adolescents, diagnosed with JIA according to the classification criteria of the International League of Associations for Rheumatology (ILAR).

Results
The mean age of our group was 16 years, with a sex ratio of 3. Ninety-seven of our participants confirmed that they’re using social media, for a mean of 3 h per day. YouTube and Facebook came back as the first two preferred platforms: 82.6% and 69.6% respectively, followed by Instagram 30.4%, Tiktok 21.7, Snapchat 17.4% and Whatsapp 8.7%. Seventeen percent of children viewed these platforms as a positive and helpful in dealing with JIA, specially taking their mind of pain, dealing with the stress resulting from the lack of mobility and facilitating interactions with others. Interestingly, none of the participants considered online resources as a place to interact with health professionals or have access to information about JIA.

Conclusions
According to their perceptions, children with JIA can benefit from an online resource, which helps them cope with some aspects of their disease, especially taking their mind of pain. Therefore, if used properly by health professionals, these online sources may be a great opportunity to deliver simplified and useful medical information for children with JIA.
P034 Macrophage activation syndrome in Juvenile Idiopathic Arthritis
Authors: Guedri Rahma , Glaimeriem  , Fitouri Zohra , Ben Becher Saayda , Hamza Bechir 
Published: November 2021

Abstract

Objectives
To describe the epidemiological, clinical-biological, and therapeutic characteristics of children with systemic JIA complicated with a macrophage activation syndrome (MAS).

Methods
It was a retrospective study, including children with JIA followed at the pediatric rheumatology unit of the children's hospital in Tunis for 22 years (January 1999 to December 2020), and presented MAS during their follow-up.

Results
We included 40 patients with JIA. Nineteen children (47.5%) presented MAS during the disease. They are 11 boys and 8 girls, with a sex ratio of 1.3. The mean age was 5.31 years (range: 0.66–10.83 years). The circumstances of the occurrence were variable. MAS was inaugural in 10 patients. Three of our patients presented MAS twice. MAS was definitive with clinical and biological markers in 12 cases and only biological in 7 cases. Seventeen of our patients were treated with intravenous Corticosteroids. Seven Childs among them received, in combination, Immunoglobulins (IG). We prescribed a biological treatment in 3 patients. We have mourned only one death.

Conclusion
MAS is a severe complication of JIA and is the leading cause of death.
P040 Effect of parental smoking on Juvenile Idiopathic Arthritis activity
Authors: Hajji H , Ben Nessib D , Maatallah K , Ferjani H , Triki W , Kaffel D , Hamdi W 
Published: November 2021

Abstract

Background
Juvenile idiopathic arthritis (JIA) is an heterogeneous group of autoimmune diseases considered as the main cause of chronic arthritis among children and teenagers. This group of children is thought to be more vulnerable because of chronic inflammation and long-term immunosuppressive therapy. Passive smoking, which is a major health problem, is likely to be particularly harmful for these children whose immunity is already compromised by their disease. The aim of our study was to assess the effect of parental smoking on disease activity in children with JIA

Methods
A monocentric cross-sectional study of patients with JIA according to the criteria of the International League Against Rheumatism (ILAR) was conducted in the rheumatology department of Kassab Institute. The following clinical and biological data were collected: sex, age, disease duration, BASDAI activity score, functional impairment assessed by the BASFI score, biological inflammation assessed by the erythrocyte sedimentation rate (ESR) and the C-reactive protein (CRP). All parents were contacted and smoking data were recorded: parent smoking, pack-years history, smoking in an indoor or outdoor space, impact of passive smoking on disease activity. We considered any child with at least one smoking parent and living in the same house to be exposed to parental passive smoking.
Patients were divided into two groups: group 1 (G1) including patients who are not exposed to parental smoking and group 2 (G2) including patients exposed to smoking by one or both parents. Data were analyzed using SPSS (Statistical Package for the Social Sciences) version 24 software. The significance level was set at a p-value &lt; 0.05.

Results
Twenty-three patients were included: 15 girls and 8 boys. Sex ratio M/F was 0.53. The mean age was 16.5 + 8.1 years [8–40]. The mean disease duration was 4.9 + 4.9 years [0.15]. The mean ESR was 21.5 + 27.1 mm and the mean CRP was 4.7 + 7.1 mg/l. Nine patients had coxitis, including 4 who were exposed to passive smoking.
Passive smoking noted in 11 patients (47.8%), including 6 boys and 5 girls. In 63.6% of the cases, this exposure occurred indoor in the family home. The number of cigarettes smoked per day was 11.7 + 8.4 [1,20]. Among the exposed patients, 36.4% considered that this exposure was an exacerbating factor of their disease. Clinical and biological data were comparable between the 2 groups as shown in table 1.

Conclusion
Although the adverse effect of maternal smoking during pregnancy on the risk of developing JIA in the child has been demonstrated, few studies have investigated the effect of parental smoking once the disease is established. Our study did not find a statistically significant association between smoking exposure and high disease activity or poor prognosis. However, further studies including a larger number of patients are needed to assess the potential effect of parental smoking on the prognosis of JIA.
P048 Juvenile systemic lupus erythematosus, what specificities?
Authors: Okka Kamelia , Belghazi M , Dehimi A , Benarab Z , Bouabdallah S , Bioud B 
Published: November 2021

Abstract

Background
Juvenile systemic lupus erythematosus (J-SLE) is a chronic autoimmune disease characterized by multi-visceral involvement with an unpredictable prognosis. The diagnosis is usually made in young women between the ages of 20 and 40, however, it can set in at any age and will be classified as juvenile (LESj) when it begins before the age of 16.We report the epidemiological, clinical, therapeutic and evolutionary characteristics of a retrospective series carried out at the level of the pediatric center—CHU de Sétif comprising 13 girls and one boy.

Methods
The mean age of onset is 12 years and 3 months, the mean time to diagnosis is 7 months. The clinical picture is made of e reached articulaire skin and e fever in 86% respectively 57% and 57% of cases, followed by kidney disease in 57% of cases. Cardiac involvement pulmon area ophtalmologiqu e is referred to in low percentages. The blood reached logic of étectée on blood counts in 85% of patient e s i and the syndrome nflammatoire was almost constant. A positive titer of antinuclear antibodies and anti- AD Nn is objectified, as well as a reduction in the level of complement. The anti-GP 2 and anti- cardio lopine antibodies are positive in 57% of cases. Has the present hue kidney in 42% of cases. A single case of overlap syndrome with dermatomyositis has been reported. As for the neurological form, only one adolescent presented it. With a single case of familial lupus and a single case of Rhupus.

Results
The diagnosis is based on the American College of Rheumatology (ACR) 1982 classification revised in 1997 and the new SLICC “Systemic Lupus International Collaborating Clinics” criteria.
The clinical characteristics of our series are consistent with the overall data in the literature with a predominance of cutaneous and joint involvement. with however some specific characteristics which are individualized by a more advanced age of onset, of 13 years on average in our study vs 10 years and 12 years, the rarity of the familial forms (1 case), a lower percentage of renal damage (42% vs 63% and 80%). The therapeutic management was based on corticosteroids and Hydroxychloroquine in most cases, the use of immunosuppressants was reserved for x severe.

Conclusion
Lupus is an autoimmune disease with protean clinical manifestations, the prognosis of which is dominated by renal, neurological and thrombotic damage. Corticosteroid treatments and immunosuppressants markedly improved the vital prognosis.
P056 Acute rheumatic fever: still a hot topic
Authors: Belamri D J , Boustil I , Chériet H , Bouslama F , Zoubir S , Bouchair N 
Published: November 2021

Abstract

Background
Acute rheumatic fever (ARF) still a very common condition and constitutes a real public health problem in the developing countries including Algeria. The objective is to assess the epidemiological and clinical profile and the disease course of ARF in our region and estimate the incidence of cardiac complications.

Method
The authors report the results of a retrospective study of 52 cases of ARF in children, hospitalized and treated at the Sainte Therese Clinic of the CHU Annaba for a period of 6 years. The diagnosis was made according to Jones criteria. Information was obtained from hospital records.

Results
The disease mainly concerned the age group of 4–10 years (52%), the sex ratio was 0.86 (28 girls, and 24 boys). The maximum number of hospitalizations is observed during the rainfall period (October-March). 54% of patients had untreated angina. Clinically, fever was noted in 23 patients, polyarthritis in third of cases and a heart murmur in half of patients. Doppler-coupled echocardiography founded carditis in 73% of patients: Endocarditis was noted in all of these cases and the mitral insufficiency was the dominant anatomo-clinical form. The number of infra-clinical carditis was considerable (10/38 cases of carditis). Biologically, accelerated ESR (&gt; 50 mm/H1) was observed in 65% of cases, high CRP level and hyper leucocytosis were noted in 38 and 31 patients, respectively. ASLOs were high in 85% of cases. The outcomes were favorable in the majority of cases. Nevertheless 5 relapses were recorded, including 3 complicated by severe carditis.

Conclusion
ARF still frequent in our region. Cardiac complications are always frequent and constitute a major factor of morbidity. Our results illustrate the absolute need for a rigorous application of the national prevention program, the main objective of which is to reduce the prevalence of rheumatic heart disease.
P060 Childhood uveitis: epidemiological, clinical and etiological profile, about 52 cases
Authors: Sakhi A , Chahir R , Elkettani A , Bouayed K 
Published: November 2021

Abstract

Background
Childhood uveitis is a rare, often serious condition, and accounts for 2% to13% of all uveitis. It is characterized by an asymptomatic onset delaying the diagnosis and exposing a high risk of complications. The etiological profile of childhood uveitis seems to be different from that of adults. The objective of our work is to analyze the epidemiological, clinical, etiological, therapeutic and disease course characteristics of pediatric uveitis.

Method
This retrospective study, carried out in a tertiary care center, involved a number of 87 eyes in 52 children under the age of 16, collected over a period extending between January 2015 and September 2019 with a minimum follow-up of 6 months.

Results
The patients mean age was 8.15 years with a sex ratio of 1.47 and a mean consultation delay of 6.7 months. Uveitis was bilateral in 67.3% of cases. Pan-uveitis predominated, followed by intermediate, anterior and posterior uveitis in 50%, 21%, 17% and 12% of cases, respectively. Uveitis was idiopathic in 67.3% of cases. Inflammatory uveitis was the most frequent aetiology with 5 cases of Behçet's disease, 2 cases of juvenile idiopathic arthritis (JIA) and 2 cases of TINU syndrome. Ophthalmologic complications were dominated by iridocrystalline synechiae in 13.5%, retinal detachment in 10.4%, cataract in 5.4% and macular oedema in 2.7%. Corticosteroid therapy was administered to all children, combined with immunosuppressive therapy in 48% of cases (azathioprine, methotrexate). The use of anti-TNF alpha was justified in 11 patients. The disease course was marked by complete remission in 11 children and partial remission in 15 patients, 26 children presented relapses of their uveitis with satisfactory final visual acuity in 73% of cases.

Conclusion
Our results show the predominance of panuveitis, probably due to diagnostic delay. Idiopathic uveitis, as described in the literature, and inflammatory causes of Behçet's disease are at the top of the list of etiologies. Pediatric uveitis is a particular entity, which requires regular monitoring by a team of experts in order to avoid eye complications and amblyopia in the youngest.
P065 A case of fibrodysplasia ossificans progressiva in Kenya
Authors: Mwende Aura , Kooliyath Athul , Samia Pauline , Mutiso Kavulani , Sagoo Ravjit , Okiro Patricia , Mugambi Stanley , Migowa Angela 
Published: November 2021

Abstract

Background
Fibrodysplasia Ossificans Progressiva (FOP) is an extremely rare and disabling disorder affecting 1 in 2 million individuals worldwide. It is caused by mutations in bone morphogenetic protein which leads to extra-skeletal ossification of soft tissues in a characteristic cranio-caudal pattern. Hallux valgus, episodic flares and progressive functional disability are characteristic features. The cause of death is often cardio-respiratory failure following thoracic insufficiency. The objective of our case study is to describe a rare but interesting case of fibrodysplasia ossificans progressiva (FOP) in an African setting.

Methods
This was a retrospective case review

Results
Two-year-old boy presented with painful firm masses of bony consistency over the axilla, neck, occiput and the forehead for seven months. He developed progressive inability to lift the arms, flex his neck and had a characteristic stiff posture while walking. The masses had a waxing and waning nature lasting about 4–5 days but with no complete resolution. He was previously treated with antibiotics for lymphadenitis which was diagnosed by biopsy of the neck mass at a different health facility. On examinationhe had bony masses on the posterior aspect of the neck tender on palpation and attached to the underlying subcutaneous tissue. Similar masses were found on his anterior and posterior chest wall measuring 1 cm by 1 cm anteriorly and larger masses- measuring 5 cm by 5 cm posteriorly. He had bilateral hallux valgus and both elbows held in fixed flexion. His fingers had varying degrees of fixed flexion. The lower limbs were unaffected.
Laboratory evaluation within normal ranges.

extensive dystrophic soft tissue calcification around both elbows outlining the joint capsules.

dystrophic soft tissue calcification in the bilateral axillae

extensive “sheet like” soft tissue dystrophic calcifications overlying the posterior elements of the vertebral column separate from the vertebrae.

Histology showed muscle replacement by hyaline cartilage and bone with intervening fibromyxoid stroma.

Discussion & Conclusions
This case is meant to raise awareness of this rare disease across Africa. The misdiagnosis of FOP approaches 90% of cases worldwide. Definitive genetic testing of FOP is now available and can now confirm a diagnosis before the appearance of heterotopic ossifications. Clinical suspicion of FOP early in life on the basis of malformed great toes can lead to early clinical diagnosis. Current management focuses on early diagnosis, avoidance of trauma and optimization of function. A short course of oral steroids is beneficial in prevention of flares.
P066 Orthopedic complications of osteogenesis imperfecta
Authors: Ferdaous Blel , Haifa Hachfi , Mouna Brahem , Younes Mouhamed 
Published: November 2021

Abstract

Background
Osteogenesis imperfecta (OI) is an inherited connective tissue disorder including various skeletal manifestations. Bone fragility, vertebral body malformation and length lower limbs inequality are often responsible for orthopedic complications. The aim of our study was to determine orthopedic complications of OI and to specify management modalities in rheumatology.

Methods
We conduct a retrospective study based on the files of children referred to the rheumatology departement over the last 15 years, for treatment of OI. The evaluation was clinical and radiological. Clinical examination included joint and spine assessment. An X-ray of the spine and painful joints associated with a quantitative densitometry were requested.

Results
Five patients were collected, their mean age was 9 years. All patients had peripheral fracture. Joint assessment revealed a flessum of the elbow and a reduction of the motion of hip in 40% of cases. Scoliosis was noted also in 40% of cases. The average of spine Z –score was -3, 4. All children had benefited from oral calcium and vitamin D supplementation associated with cyclic intravenous bisphosphonates and an adapted rehabilitation protocol. Surgical management was often needed in 2 cases.

Discussion and Conclusion
Orthopedic complications during OI are essentially the consequence of lower bone mineral density which would cause peripheral or vertebral fracture. Indeed, some other factors like length inequality, pelvic obliquity, ligamentous laxity and inter-vertebral disc abnormalities could be involved in scoliotic progression. The management of these skeletal complications must be early and multidisciplinary in order to improve the prognosis and the quality of life of the patient
P078 Multi-system inflammatory syndrome associated with SARS-CoV-2 in children: clinical and therapeutic characteristics
Authors: Masmi Nacera 
Published: November 2021

Abstract

Background
The COVID-19 pandemic was associated with the emergence of a new disease resembling the Kawasaki disease (kDa). That was primarily reported in children of western countries strongly affected by SARS-CoV-2. This syndrome was called Pediatric Multi-System Inflammatory Syndrome (PIMS or MIS-C), and has affected thousands of children, adolescents and young adults throughout the world since the alert launched by the National Health Service of the United Kingdom at the end of April 2020. This work is a literature review of data focusing on the clinical and prognostic characteristics of the MIS-C, the links between Covid-19 and MIS-C, and current therapeutic recommendations.

Material and methods
We carried out research of the studies recently published in the electronic bibliographic databases (Google Scholar, PubMed, Science direct, EmConsults, Research Gate, Medline, Cochrane, Trip).

Results
25 studies were selected, involving 3015 MIS-C patients from different countries (France, United Kingdom, Italy, Germany, Spain, Brazil, Mexico, Chile, United States, India, Pakistan, Iran). Fever and gastrointestinal disorders were the most frequent clinical manifestations, followed by cardiovascular and respiratory symptom. Biological markers of inflammation and SARS-CoV-2 serology were assessed in all studies. Characteristics similar to Kawasaki disease, and toxic shock syndrome were reported in all studies. Complications such as coronary artery aneurysm; cardiac insufficiency, renal insufficiency, respiratory distress were noted as well as a significant rate of admission to intensive care units. Finally, the mortality rate was low in the majority of studies. The treatment adopted in the majority of the studies was an association of intravenous immunoglobulins and corticosteroids.

Conclusion
Although the fact that the exact pathogenesis of MIS-C remains unknown, post-infectious immune deregulation induced by the SARS-CoV-2 virus appears to be the trigger of MIS-C. MIS- C has been described as a new and serious disease entity with multi-organ involvement and a wide range of signs ranging from fever and gastrointestinal symptoms to myocardial damage, shock, and the development of coronary artery aneurysms.

Keywords
COVID-19, Multi-System Inflammatory Syndrome (MIS-C), Kawasaki Disease (kDa), Child