bluuebunny/crossref_metadata_2025 huggingface dataset:
Embolic MCA Stroke Treated With tPA and TICI-3 Mechanical Thrombectomy Complicated by Hemiballismus: A Case Study and Literature Review
Authors: Tatum Peter S , Kornbluth Joshua , Soroka Andrew
Published: November 2021

This report examines the etiology of hemiballistic movements that began 24 hours after a 63-year-old male with vascular risk factors received tissue plasminogen activator (tPa) and thrombolysis in cerebral ischemia 3 (TICI3) thrombectomy for a left middle cerebral artery (MCA) ischemic stroke. The clinical course was reviewed from an admission at a large academic institution where assessments included physical exams, head and neck computed tomography angiography (CTA), and head magnetic resonance imaging (MRI) without contrast. The patient’s initial physical exam was consistent with a left MCA syndrome and included a National Institute of Health Stroke Scale (NIHSS) of 20. CTA showed an embolic M2 occlusion. After tPA and TICI 3 thrombectomy, NIHSS improved to 3 for dysarthria, facial weakness, and language deficits. MRI showed left insular diffusion restriction. New right-sided hemiballistic movements began 24 hours after treatment. At his six-week follow-up outpatient appointment, the movements were no longer present, and his neurologic exam was unremarkable, including an NIHSS of zero. No prior cases of hemiballism have been reported as a likely complication of treatment with tPa and thrombectomy. The globus pallidus is the suspected origin of the ballistic movements either from a decreased insular signal or embolic event during treatment. As stroke interventions improve, the susceptibility of certain tissues to brief ischemic events during treatment must be assessed.
Development of a Critical Care Air Transport Team “Go Bag”: Slimming Down ICU Capability to Augment Pararescue or Civilian Contract Medical Evacuation Personnel
Authors: Nowadly Craig D , Foley Kyle E , Davis Maxwell L , Hebert Erik J , Corey Gabe A
Published: November 2021

Introduction

Critical Care Air Transport Team (CCATT) is a three-person United States Air Force (USAF) medical asset, typically providing intercontinental medical evacuation on large military aircraft. The CCATT equipment Allowance Standard (AS) weighs approximately 272 kg (600 lbs). In austere locations, CCATT teams may augment contract medical evacuation (CME) personnel or Pararescue (PJ) in small aircraft with limited space for medical equipment. It was unknown what deployed PJ and CME carry within their packouts. We sought to design a packout or “Go Bag,” weighing less than 22.7 kg (50 lbs) and sourced from the CCATT AS, that a CCATT member could use to complement CME or PJ equipment to provide a higher level of care while limiting redundancy.

Materials and Methods

Equipment lists were obtained from a CME and PJs from two separate USAF squadrons. The equipment lists were combined to provide a reference for development of a CCATT Go Bag. Three members of a deployed CCATT team independently generated a list of necessary equipment from the CCATT AS. The list was peer reviewed by a separate, deployed CCATT team.

Results

A Go Bag was developed with the supplies and equipment necessary for video laryngoscopy, ventilation, invasive pressure monitoring, basic laboratory capability, chest tube placement, ultrasound, and advanced pharmacologic interventions. The Go Bag weighed 18.3 kg (40.4 lbs). A separate respiratory bag weighing 1.1 kg (2.4 lbs) was attached directly to a ventilator. Intravenous pumps and cardiac monitoring equipment were notable ICU equipment excluded from the Go Bag.

Conclusion

Major components of the CCATT AS can be reduced into a Go Bag and accompanying Ventilator Accessory Bag. This may benefit CCATT teams required to augment PJs or CME in small aircraft during prolonged field care scenarios.
Vela pulsar: single pulses analysis with machine learning techniques
Authors: Lousto Carlos O , Missel Ryan , Prajapati Harshkumar , Sosa Fiscella Valentina , Armengol Federico G López , Gyawali Prashnna Kumar , Wang Linwei , Cahill Nathan D , Combi Luciano , Palacio Santiago del , Combi Jorge A , Gancio Guillermo , García Federico , Gutiérrez Eduardo M , Hauscarriaga Fernando
Published: November 2021

We study individual pulses of Vela (PSR B0833−45/J0835−4510) from daily observations of over 3 h (around 120 000 pulses per observation), performed simultaneously with the two radio telescopes at the Argentine Institute of Radioastronomy. We select four days of observations in 2021 January to March and study their statistical properties with machine learning techniques. We first use Density-Based Spatial Clustering of Applications with Noise clustering techniques, associating pulses mainly by amplitudes, and find a correlation between higher amplitudes and earlier arrival times. We also find a weaker (polarization dependent) correlation with the mean width of the pulses. We identify clusters of the so-called mini-giant pulses, with ∼10 times the average pulse amplitude. We then perform an independent study, with Self-Organizing Maps (SOM) clustering techniques. We use Variational AutoEncoder (VAE) reconstruction of the pulses to separate them clearly from the noise and select one of the days of observation to train VAE and apply it to the rest of the observations. We use SOM to determine four clusters of pulses per day per radio telescope and conclude that our main results are robust and self-consistent. These results support models for emitting regions at different heights (separated each by roughly a hundred km) in the pulsar magnetosphere. We also model the pulses amplitude distribution with interstellar scintillation patterns at the inter-pulses time-scale finding a characterizing exponent nISS ∼ 7–10. In the appendices, we discuss independent checks of hardware systematics with the simultaneous use of the two radio telescopes in different one-polarization/two-polarizations configurations. We also provide a detailed analysis of the processes of radio-interferences cleaning and individual pulse folding.
Brexit and UK higher education
Authors: Mayhew Ken
Published: November 2021

The Brexit vote in 2016 caused consternation in higher education circles. Financial and reputational questions were raised concerning: the number of students from EU countries coming to study in the UK; the ability of the sector to retain and recruit staff from the EU; the threats to research funding; and the ability of UK students to study abroad. This paper tracks developments since then. Much remains uncertain and the picture has been complicated by the impact of Covid-19. Though enrolments of EU citizens for the 2021–22 academic year have fallen dramatically, this has been more than offset by larger numbers of UK entrants and entrants from non-EU countries. The main immediate threat is to research funding, partly because of restricted access to EU programmes and partly because of constrained government spending. The longer-term threat is to the sector’s relationship with European academia.
P011 Radiological pattern in juvenile onset Spondylarthritis differ from the adult onset spondylarthritis
Authors: Ferjani Hanene , Yasmine Makhlouf , Maatallah Kaouther , Ben Nessib Dorra , Triki Wafa , Kaffel Dhia , Hamdi Wafa
Published: November 2021

Background

Spondylarthritis (SpA) is a chronic inflammatory disease of the axial spine that may be affect the peripheral joints. SpA occurs predominantly in adulthood: Adult onset Ankylosing Spondylitis (AoAS). However, it can occur earlier in childhood (≤16 years old), also termed as juvenile onset Ankylosing Spondylitis (JoAS). In the second group, delay in diagnosis may lead to further structural damage. The aim of our study was to compare the differences in radiographic features between JoAS and AoAS.

Methods

We conducted a retrospective study in our department of rheumatology of Kassab Institute of orthopedics, including patients diagnosed with SpA according to the ASAS criteria (≥17 years at symptom onset) or to the ILAR criteria (≤16 years at symptom onset). Were not included AS patients whose disease onset was ≥ 45 years. Sociodemographic as well as disease characteristics were recorded. The lateral cervical and lumbar spine radiographs were used to assess structural damage by the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) and The Bath Ankylosing Radiologic Index (BASRI). The total score of mSASSS and BASRI vary between 0–72 and 0–16 respectively with higher scores indicating evolved structural damage. Radiographic features were then compared between AoAS and JoAS. The level of significance was fixed for a P < 0.05.

Results

Of 140 AS patients, 40 had JoAS and 100 had AoAS. The average age at disease onset was 12.4 ± 3 [8–16] and 25.4 ± 10.1 [17–42] respectively (P < 0.001). The disease duration was 10.6 years [1–44]. The average current age was 25.3 ± 10.2 [9–59] and 32.5 ± 7.5 [18–46] (P < 0.001). The JoAS group showed a more frequent onset with peripheral joint involvement than the AoAS group (80% vs 50%,P = 0.001). Similarly, hip involvement was more frequent among JoAS patients (57.7% vs 25%, P = 0.000). There was no significant differences between the two groups regarding the mean mSASSS score, the total BASRI score and the BASRI score for the sacroiliac joints (P = 0.9, P = 0.49, P = 0.06 respectively). Similarly, there was no differences in the mean spine BASRI score between the two groups, although there was a trend of higher scores among AoAS patients (3.2 vs 3.9, P = 0.15 respectively). On the contrary, JoAS patients had a significantly higher BASRI score for the hip than AoAS patients (2.4 vs 1.3, P = 0.000).

Conclusion

Our study showed that JoAS patients are more likely to suffer from early hip damage than AoAS patients. The spine BASRI was similar between the two groups despite the shorter disease duration in the juvenile group. This highlights the need for screening for hip involvement and for a closer monitoring in this subset of children.
P012 An HLA Typing for every JIA?
Authors: Boutrid N , Rahmoune H , Boutrid H , Bioud B , Madani M , Amrane M
Published: November 2021

Background

Among the genetic susceptibility factors of Juvenile Idiopathic Arthritis (JIA), the HLA class I and II genes are the most frequently involved. These HLA genes are extensively investigated, highlighting the role of self/non-self-balance in auto-immune and auto-inflammatory diseases.

Methods

We carried out a retrospective study of HLA class I and/or class II genes (HLA B and HLA DR genes) of 12 children.

Genotyping was performed through microlymphocytotoxicity for class I (HLA B5 and B27) and through indirect immunofluorescence and PCR for class II (HLA DR4)

Results

The summarized results depict:

    4 patients have the HLA B27 +

    3 patients have the HLA B5+

    1 patient had the HLA DR

JIA comprises a broad spectrum influenced by both genetic and environmental factors. The International League of Rheumatology Associations (ILAR) has defined seven categories of JIA consisting of a myriad of pediatric auto-immune and auto-inflammatory diseases.

The HLA genomic region is also associated with a wide range of auto-immune diseases, encoding the HLA-DR, HLA-DQ, and HLA-DP proteins involved in the peptide’s presentations to HLA -class II-restricted CD4 + helper T cells. These mechanisms are involved in the pathogenesis of various diseases such as rheumatoid arthritis.

Juvenile arthritis has also been associated with a single nucleotide polymorphism in the regulatory region of the interleukin-6 gene, close to the HLA coding region.

Conclusion

Genetic exploration of the HLA system in JIA, more accessible and less expensive than other targeted genetic typing, can be a helpful tool. Its usage ought to be encouraged and expanded in clinical practice.
P014 Use of prosthetic surgery in Juvenile Idiopathic Arthritis
Authors: Miladi S , El Mabrouk Y , Fazaa A , Souabni L , Ouenniche K , Kassab S , Chekili S , Ben Abdelghani K , Laatar A
Published: November 2021

Background

Juvenile Idiopathic Arthritis (JIA) is the most common inflammatory rheumatism in childhood characterized by destructive potential. The involvement of weight-bearing joints constitutes an evolutionary turning point in the course of the disease, which may be responsible for a major functional handicap. In this context, arthroplasty constitutes a therapeutic alternative. Through this study, we aimed to determine the prevalence of the prosthetic surgery and its benefits in children with JIA.

Methods

This is a retrospective study of JIA patients. The diagnosis of JIA was selected according to ILAR criteria. All the patients included were followed for a minimum of 1 year. The parameters collected were: pain VAS, HAQ, Lequesnealgofunctional index (AFI), disease activity scores and mobility of the operated joint before and 6 months after surgery.

Results

Forty -four JIA patients were followed over an average period of 14 years (minimum of 1 year). The mean age of disease onset was 9 years [1.5–16 years]. Fifteen cases of coxitis (34%) and 4 cases of destruction of the knees (9%) were noted after an average course of the disease of 8 years [6 months–19 years]. Radiologically, coxofemoral ankylosis is reported in 6 cases, acetabular protrusion in 2 others and total destruction of the knee with misalignment in 2 cases. Eight patients required hip replacement surgery and 2 knee cases. In all cases, the hip prostheses were bilateral. Only one knee prosthesis was placed. The JIA subtype was rheumatoid factor seronegative polyarthritis in 5 cases and enthesitis related arthritis in 3 cases. Six months after surgery, the average AFI dropped from 15.4–4.5; the pain VAS from 73 mm to 50 mm and the HAQ from 2.5–1.06. In the polyarticular JIA, the DAS28 composite score decreased from 4.02–3.8 and in ERA patients the BASDAI decreased from 6.5–4.25 while BASFI remained stable. Recovery of joint mobility was partial in only one case. No immediate or late postoperative complications were noted.

Conclusion

Prosthetic surgery provided improvement in disease activity and recovery of gait pattern in children with severe JIA. However, the decision of an arthroplasty remains delicate in children, especially since this intervention can compromise the stature growth.
P015 Imaging findings in cervical spine in patients with Juvenile Idiopathic Arthritis in Tunisia
Authors: Bettaieb Hiba , Ferjani Hanene , Maatallah Kaouther , Ben Nessib Dorra , Triki Wafa , Kaffel Dhia , Hamdi Wafa
Published: November 2021

Background

Juvenile Idiopathic Arthritis (JIA) is a chronic disease characterized by prolonged synovial inflammation that may cause structural joint damage. However, little is known about cervical spine involvement in JIA. The main objective of this study is to describe radiological findings of the cervical spine in patients with JIA.

Methods

We conduct a retrospective monocentric study. All JIA patients were included (ILAR criteria). Sociodemographic, JIA subtype, and clinical characteristics were collected. Disease activity at JIA diagnosis was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis-related enthesitis form. Cervical spine radiographs including anteroposterior and lateral with flexion views were analyzed. A p-value < 0.05 was considered significant.

Results

We included 25 patients (16 girls and 9 boys) diagnosed with JIA with a mean age at disease onset of 9.9 ± 3.9 [3–16]. The median disease duration was 36 months (IQR 25–75%; 30–84). The JIA subtypes were in decreasing order of frequency: Enthesitis-related Arthritis (n = 9), Oligoarticular (n = 6), Polyarticular RF- (n = 4), Polyarticular RF + (n = 2), Systemic (n = 2), Psoriatic Arthritis (n = 1), and Undifferentiated (n = 1).

Median ESR and CRP were 17 mm/h [2–98] and 15.4 mg/l [0–56] respectively. The Median BASDAI score was 2.8 [1–6.3]. Median JADAS10 score was 5.3 [0–20]. Four patients (16%) were on long-term corticosteroid therapy.

Five patients (20 %) have a cervical spine involvement with the following subtypes: Polyarticular (n = 2), enthesitisenthesitis-related arthritis (n = 2), and systemic (n = 1). Clinical manifestations were neck pain (n = 3) and limited range of motion (n = 4). Neurological examination noted brisk deep tendon reflexes (n = 6), positive Babinski reflex (n = 1) and positive Hoffmann reflex (n = 2). No patient had a neurological deficit.

The conventional radiographs of the cervical spine showed: loss of cervical lordosis (n = 2), diastasis C1-C2 (n = 3), erosion of the odontoid process (n = 1), and anterior ankylosis (n = 3). Subsequent cervical spine MRI confirmed these findings and showed pannus at the craniocervical junction in one case and block vertebra of C6-C7 in another case. Atlanto-axial subluxation was anteroposterior in 3 patients and rotatory in one.

Conclusion

Cervical spine involvement is frequent and underestimated in JIA, and its radiological features are various. Hence, regular radiographic monitoring of the cervical spine is required to prevent the development of this complication.
P017 Complications of Juvenile Idiopathic Arthritis
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

Background

Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease characterized by onset before the age of 16. This term encompasses several disease categories, each of which has distinct methods of presentation, clinical signs, and prognosis. The study aimed to determine JIA complications in 51 patients.

Methods

A cross-sectional study including patients diagnosed with JIA according to ILAR criteria was conducted for 26 years [1995– 2021]. Epidemiological, clinical, therapeutic, and evolutive aspects were noted.

Results

Twenty-nine males and 22 females were included. The mean age of the disease onset was 7.6 years [1,5–16]. The mean age of patients at the time of the study was 23.29 years [9–45]. Polyarticular and seronegative form was the most frequent (34.5%). Other subtypes diagnosed were systemic (25%), enthesitis-related arthritis (21.2%), oligoarticular (12.5%), and seropositive polyarticular (5.8%).

Standard X-Ray imaging showed articular damage in 50% of the cases. Hip arthritis was observed in 32% and surgery was needed in 16.9%. One patient presented with atlantoaxial subluxation. Growth retardation was noted in 28.6%. Cardiac manifestations were seen in 3 patients (pericarditis = 2, myocarditis = 1), uveitis in 3 cases, renal manifestation (extra membranous glomerulonephritis) in one patient with polyarticular form. One patient was diagnosed with multiple sclerosis.

Small doses of corticosteroids were prescribed in 71.7%. Methotrexate was prescribed in 70.5% (interrupted for adverse effects in 3 patients), sulfasalazine in 30.6%, hydroxychloroquine in 5.7%, leflunomide in 15.4%. bDMARDs were needed in 16 patients: 14 patients received TNF alpha inhibitors, rituximab was prescribed for one patient with a polyarticular form, and tocilizumab in a patient with a systemic form.

A switch of bDMARDs was conducted in 10 patients: for inefficiency in 4 cases and adverse effects in other 4 cases. Three patients developed uveitis under Etanercept, septicemia under Adalimumab, an allergic reaction, and depression under Infliximab. One patient died from a convulsive seizure at the age of 9.

Conclusion

The presence of complications is an additional burden to JIA patients. A multidisciplinary approach is required for the management of these complications.
P018 Functional impairment in enthesitis related arthritis patients
Authors: Ferjani Hanene , Bettaieb Hiba , Ben Ammar Lobna , Maatallah Kaouther , Ben Nessib Dorra , Triki Wafa , Kaffal Dhia , Hamdi Wafa
Published: November 2021

Background

Enthesitis related arthritis (ERA) is a subgroup of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis and predominately lower limb arthritis and can affect sacroiliac joint and spine. Recent studies showed that ERA is associated with worse physical status and poorer quality of life (1). The main objective of this study was to compare the aspects of functional status in patients (ERA) and patients with spondyloarthritis (SpA).

Methods

A retrospective monocentric study was carried out on patients with ERA (ILAR criteria) or SpA (ASAS Criteria). Demographic data and clinical characteristics were obtained from medical records. Disease activity was evaluated by: erythrocyte sedimentation rate (ESR), C-reactive protein rate (CRP) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Functional impairment was evaluated by Bath Ankylosing Spondylitis Functional Index (BASFI) and Ankylosing Spondylitis Quality of life Questionnaire (ASQoL). Global well-being was assessed by Bath Ankylosing Spondylitis Global Index (BASGI). Population was divided into two groups: group 1 (G1) stands for ERA patients and group 2 (G2) stands for SpA patients. P < 0.05 was considered statistically significant.

Results

A total of 174 patients (40 ERA and 134 SpA) were enrolled. Mean age at disease onset was 12.4 ± 3 years in G1 and 27.8 ± 8 years in G2. Male to female sex ratio was 5.6 in G1 and 3.7 in G2.

Morning stiffness (>60 min) was reported by 37.5% of G1 and 49.3%. G1 patients had longer morning stiffness than G2 (61 [0–90] min vs 30 [0–240] min; P = 0.58). Multiple nocturnal awakenings were reported by 45% of G1 patients and 58.2% of G2 patients. Median BASDAI score was 4.9 [1–44] in G1 and 4.5 in G2 [0–10] (P = 0.48). Median BASGI score was 6 [1.5–9.5] in G1 and 6 [0–10] in G2 (P = 0.58). Median ESR was 35 mm/h [8–90] in G1 and 35 mm/h [2–125] in G2. Median CRP was 18.2 mg/l [1–70] in G1 and 13 mg/l [3–180] in G2. The assessment of functional status revealed that G1 patients had higher BASFI scores than G2 patients (5.2 vs 4.5). The association between G1 and BASFI was statistically significant (P = 0.05). Median ASQoL was 12 [2–17] in G1 and 9 [0–18] in G2. No link was noted between G1 and ASQoL score (P = 0.152).

Conclusion

Our study showed that ERA was associated with higher BASFI scores in comparison with SpA. Treat-to target strategies are mandatory in order to optimize the functional status of children with ERA.
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

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 >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

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

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

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 < 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

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

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 (> 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

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

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

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

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
P085 Chronic recurrent multifocal osteomyelitis: about 2 cases
Authors: Miladi Saoussen , Makhlouf Yasmine , Fazaa Alia , Sellami Mariem , Ouenniche Kmar , Souabni Leila , Kassab Selma , Chekili Selma , Ben Abdelghani Kawther , Laatar Ahmed
Published: November 2021

Background

Chronic recurrent multifocal osteomyelitis (CRMO) also known as aseptic osteomyelitis is a rare auto-inflammatory disease with an incidence estimated at 4/100 000 population [1]. The aim of our work was to report two cases of CRMO that illustrate challenges in the diagnosis of this rare disease.

Method

We report the case of two patients diagnosed with CRMO. Clinical, biological and radiological data as well as disease outcomes were described. We also collected data about treatment modalities.

Results

Two patients aged of 7 and 10 years respectively, without any notable pathological history, presented recurrent episodes multifocal painful swelling of limbs. In the first case, the symptoms concerned the left ankle and knee as well as the left hip, all associated with lameness and an altered general condition, with neither fever nor skin manifestations. In the second case, the swelling involved the right shoulder, right hip and the left ankle. There was no elevated CRP or ESR in any of patients. Immunological status (RF, anti-CCP, AAN) as well as the HLA-B27 antigen test were negative. In the first patient, standard radiographs showed lytic lesions of the proximal metaphysis of the tibia, the greater trochanter and the left lateral malleolus. MRI of the pelvis, knee, and sternum of the first patient revealed edematous involvement of the left greater trochanter, the right ilium, the proximal metaphyseal region of the tibia and the right edge of the sternum, whereas in the second patient, a whole-body MRI showed inflammatory signs over the left greater trochanter, the insertion of the gluteus medius and obturator externus, right trochanteric bursitis and oedema of the entire right ilium. In the first patient, bone scintigraphy showed intense uptake of radioisotopes in the left ilium, the 7 th right costo-vertebral junction, the trochanteric mass, the upper end of the tibia and the lower end of the left fibula. Bone biopsy showed bone remodeling in both cases without evidence of infection or tumor. The diagnosis of CRMO was retained, supported by the prompt response to NSAIDs and short-term corticosteroid therapy. However, the second patient presented, 8 years later, pain in the sterno-clavicular joint as well as the right hip. A relapse of the disease was confirmed by MRI. Therapeutic escalation with zoledronic acid 0.025 mg/kg intravenous infusion every six months allowed the resolution of the symptoms.

Conclusion

These observations illustrated a rare disorder in children, characterized by lytic lesions predominantly in the metaphysis of long bones. Bone scintigraphy allowed an early assessment of disease extension and histological examination ruled out a malignant tumor and an infection. The first-line treatment is anti-inflammatory drugs. In case of failure, bisphosphonates seem to be effective.