bluuebunny/crossref_metadata_2025_split huggingface dataset:
Facilitating innovation diffusion in social networks using dynamic norms
Authors: Zino Lorenzo , Ye Mengbin , Cao Ming 
Published: October 2022

AbstractDynamic norms have recently emerged as a powerful method to encourage individuals to adopt an innovation by highlighting a growing trend in its uptake. However, there have been no concrete attempts to understand how this individual-level mechanism might shape the collective population behavior. Here, we develop a framework to examine this by encapsulating dynamic norms within a game-theoretic mathematical model for innovation diffusion. Specifically, we extend a network coordination game by incorporating a probabilistic mechanism where an individual adopts the action with growing popularity, instead of the standard best-response update rule; the probability of such an event captures the population’s “sensitivity” to dynamic norms. Theoretical analysis reveals that sensitivity to dynamic norms is key to facilitating social diffusion. Small increases in sensitivity reduces the advantage of the innovation over status quo or the number of initial innovators required to unlock diffusion, while a sufficiently large sensitivity alone guarantees diffusion.
12 Hip involvement in juvenile idiopathic arthritis: frequency and associated factors
Authors: Tbini Houssem , Rahmouni Safa , Boussaid Soumaya , Ammou Ahlem Ben , Jemmali Samia , Rekik Sonia , Zouaoui Khaoula , Sahli Hela , Elleuch Mohammed 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is a pediatric rheumatic disease with several subgroups. The hip is frequently affected. The frequency of this involvement can reach 50% especially in the severe and destructive forms. Arthroplasty may be indicated in advanced cases.

Objectives
To assess the frequency and associated factors with hip involvement in (JIA).

Methods
We conducted a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). Demographic, clinical, biological, and radiographic data were collected. These parameters were compared according to the presence or absence of hip involvement.

Results
A total of 29 Patients were enrolled (12 men and 17 women), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years.
Sixteen patients had a polyarticular form. Mean CRP values were 42.74 ± 63.37 [2–218] mg/l, a biological inflammatory syndrome was present in 19 cases. Rheumatoid factor, ACPA and anti-nuclear antibodies were observed in 12, 7 and 5 cases respectively. At least one extra-articular manifestation was noted in 16 cases.
Hip involvement was noted in 14 patients (48.3%). It was bilateral in 64.3% of cases (n = 9). Twenty-three hips were affected in total (56.9%). Hip involvement was diagnosed 14.5 ± 9.37 [1–28] years after disease onset. Arthroplasty was performed on 10 hips with a delay of 201.60 ± 104.75 [108–348] months between diagnosis of JIA and surgery.
Hip involvement was associated with male gender in our study (75% vs 29.4%; p = 0.016). On the other hand, our study showed that age, age at onset, diagnostic delay, symptoms duration, smoking, BMI, extra-articular manifestations, CRP, rheumatoid factor, antinuclear antibodies, ACPA and erosive character were not associated with hip involvement.

Conclusion
Our study showed that hip involvement is common in JIA, mainly in male patients. It usually occurs late in the disease course. Since hip involvement is a cause of disability and functional impairment, it should be assessed regularly.
14 Juvenile idiopathic arthritis: osteoporosis and fractures
Authors: Ammou Ahlem Ben , Rahmouni Safa , Boussaid Soumaya , Tbini Houssem , Jemmali Samia , Rekik Sonia , Zouaoui Khaoula , Sahli Hela , Elleuch Mohammed 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is the most common inflammatory arthritis in children. Chronic inflammation, as well as the various treatments used during JIA increase the risk of occurrence of osteoporosis (OP) and fractures.

Objectives
The objective of this work was to determine the frequency of OP in JIA and to investigate the factors associated with its occurrence.

Methods
This was a retrospective study including adults with long-standing JIA according to the International League of Associations for Rheumatology (ILAR) criteria over a period of 28 years (1994–2022). We collected sociodemographic and anthropometric parameters, clinical data, results of biological assessments, bone densitometry results and data on prescribed therapies. We compared these variables according to the bone densitometry profile to assess the factors associated with OP in JIA.

Results
There were 29 patients (17 females and 12 males), the mean age was 35.69 ± 11.72 [18–61] years. The mean age of disease onset was 11.10 ± 4.25 [2–16] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months. The average disease duration was 24.48 ± 12.76 [1–47] years. Three patients were smokers. The mean BMI was 21.20 ± 4.88 [14.17–27.55] kg/m2, and 4 patients had a BMI ≥ 25 kg/m².
The polyarticular form was the most frequent, noted in 55.2% of cases (n = 16). NSAIDs, corticosteroids and methotrexate were prescribed in 62.1%, 69% and 79.3% of cases respectively.
OP was found in 24.1% of cases (n = 7). Mean T-score values at the lumbar and femoral sites were −2.20 ± 1.22 SD and −2.31 ± 0.97 SD, respectively. Six patients suffered at least one fracture.
Patients with OP had lower BMI than patients with normal bone density (17.02 ± 2.46 vs 23.91 ± 5.42; p = 0.046). Furthermore, absence of rheumatoid factor was associated with OP in our study (83.3% vs 16.7%; p = 0.008).
On the other hand, the following parameters were not associated with the occurrence of OP in our study: age, gender, smoking, age at onset and duration of progression of JIA, BMI, extra-articular manifestations, CRP, antinuclear antibodies, ACPA and erosive character. Regarding the treatment received (NSAIDs, corticosteroids and methotrexate), no difference was found between patients.

Conclusion
Osteoporosis in JIA is common and has a prognostic impact. It must be systematically screened throughout the follow-up. In our study, OP was associated with the absence of rheumatoid factor and was more frequent in patients with low BMI.
23 Predictive factors of hip involvement in juvenile idiopathic arthritis
Authors: Rania Ben Aissa , Hanene Ferjani , Mariam Moalla , Dorra Ben Nessib , Wafa Triki , Kaouther Maatallah , Dhia Kaffel , Wafa Hamdi 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Hip involvement is a common problem in JIA patients and is associated with functional disability and poor outcomes. Intensive therapy is required to avoid replacement surgery. With few studies in the literature, the predictive factors of hip involvement are still unknown.

Objectives
To identify the clinical, biological characteristic of patients with hip involvement and determine the risk factors associated.

Methods
A cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR). The recorded data included sociodemographic features, disease characteristics (subtype disease, duration, and juvenile arthritis disease activity score (JADAS) as well as treatment modalities. Regarding coxitis, we collected radiographs, ultrasound (US), and magnetic resonance imaging (MRI) of the hip when performed. Coxitis was defined by clinical (limited range of motion) and/or radiographic findings (destruction, synovitis, bone marrow oedema).

Results
Thirty-five patients (20 females) with a median age of 12 years (5–18) and disease duration 3 years (0.25–15) were recruited. The patient's distribution of JIA subtypes were oligoarticular (n = 13), enthesitis-related arthritis (n = 9), polyarticular (n = 4) (negative rheumatoid factor in 3 patients), undifferentiated (n = 4), psoriasis-arthritis (n = 4) and systemic-onset (n = 1). ESR and CRP median values were 15 mm/h (0–63) and 2 mg/l (0–47); respectively. DMARDS was admitted to 16 patients Sixteen patients [Methotrexate (n = 10), biological agent (n = 3), biological agent and methotrexate (n = 2), salazopyrine (n = 1)].
Sixteen patients (45.71%) developed coxitis (radiographic (n = 8), MRI (n = 5) and US findings (n = 3)) with limited range of movement in eight (50%) and radiological evidence of hip damage in 10 (62.5%). Hip involvement was associated with a longer disease duration (p = 0.051). JADAS of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significant (p = 0.565). Higher CRP and ESR values were found in patients with coxitis (mean 8.39 mg/l vs 6.83 mg/l, 22.29 mm/h vs 16.37 mm/h respectively) but not significant (p = 0.718, p = 0.287 respectively). No associations were found between hip involvement and BMI (p = 0.233), age-onset (p = 0.496), JIA subtype (p = 0.509) and sex (p = 0.767).

Conclusion
Our study shows that long disease duration exposes to a higher risk of hip involvement in children with JIA. Active disease and biological inflammatory syndrome could be associated risk factors. Studies with larger sample size are needed to draw definite causal associations.
26 Kawasaki’s disease vs systemic juvenile idiopathic arthritis, a diagnostic dilemma
Authors: Thakkar Monali , Migowa Angela 
Published: October 2022

Abstract

Background
Both these conditions are separate inflammatory conditions whose clinical presentation overlap and thus make their distinction difficult. Each has a criterion that allows diagnosis but since features overlap, diagnoses may be missed.
Recent research also shows that both disease processes have similar underlying pathogenesis and involve interleukin 1; which have now raised the question of whether both these disease processes are distinct entities or conditions of a similar spectrum.

Methods
Medical records of from the inpatient admission at current and referring hospitals which included clinical history and examination, laboratory and radiological findings.

Results
13-year-old boy referred from Isiolo county with complains of unremitting fevers for 9 days with associated hip pains. Was initially treated for malaria, and started on broad spectrum antibiotics and antivirals. On further examination, was noted to have cracked lips, strawberry tongue, non-suppurative conjuctivitis and an anterior chest wall wheal like rash. Examination also revealed a left sided non tender submandibular lymph node. Initial laboratory investigations showed neutrophilia of 12.89, thrombocytosis of 431, CRP of 364 and normal joint radiographs. An ECHO done showed left ventricular enlargement with corresponding mild Mitral regurgitation. Blood cultures showed no organisms. Autoimmune markers and tropical fever markers were normal as well. He was then placed on prednisolone challenge and reported to be fever free for the first time in 12 days.

Discussion and conclusion
A persistent fever unremitting with parenteral antipyretics can have a multitude of differentials. In a tropical nation the likelihood of an infectious cause is more common than other aetiologies. Hence, it should always be investigated for thoroughly. In the case highlighted above, malaria, TB, osteomyelitis, septic arthritis and other infectious causes were investigated. Childhood malignancy can also present with the same. But of note is autoimmune conditions need to be considered. With history of arthritic like pain, quotidian pattern of fevers, evanescent salmon coloured chest wall rash and cervical lymphadenopathy, a diagnosis of Systemic Juvenile Arthritis needs to be considered. Often this may be overlooked or misinterpreted as Kawasaki’s; which is a close differential; or Still’s disease. There is a criterion for SJIA by the International League of Associations for Rheumatology, however, there can be an incomplete presentation as well. This adds on to the diagnostic challenge and hence, SJIA may remain undiagnosed for a long time.
28 Osteoarticular manifestations simulating JIA in acute leukemia: case report and literature review
Authors: Benmouffok N , Boudouaa F. Z , Tari S , Nemmar R , Boulainine A , Rouabeh S , Ouatah S , Zeroual Z 
Published: October 2022

Abstract

Background
The osteoarticular manifestations are proven during hematological malignancies in children, they can be the revealing symptom of the disease, and they may simulate a full clinical picture of Juvenile Idiopathic Arthritis (JIA), especially if no blasts are found in blood smear. Some therapies are common to both diseases, which commonly delay diagnosis for a couple of months and worsens the prognosis of these malignancies. We report three observations of patients treated for JIA which turned out to be acute leukaemia.

Clinical caises
The first patient is a 7-year-old male followed in pediatric rheumatology consultation for a monoarticular onset JIA. Since the patient did not respond to indomethacin, a bone marrow examination (BME) was performed before corticosteroid therapy (CST), no blasts were found. The patient received 01 month of CST which led to the amendment of clinical and biological signs, however, joint pain and bicytopenia reappeared after we started CST reduction. Blood and blood marrow smear were performed, leading to the diagnosis of acute B-lymphoblastic leukaemia.
The second patient is a 4 years old male admitted for exploration of polyarthritis with cervical lymphadenopathy, the initial assessment allowed the diagnosis of JIA with polyarticular onset. Indomethacin is prescribed, without any improvement. Radiological assessment was performed, as well as a complete blood count (CBC) which found neutropenia with lymphopenia, and blasts were discovered in the second BME.
The third patient is a 6-year-old female admitted for exploration of arthralgia and pathological fractures. Radiological signs in favor of leukaemia and normochromic aregenerative normocytic anaemia were revealed, allowing the diagnosis of ALL B to be made after several consultations in rheumatology.

Discussion
Joint damage during leukaemia, called leukaemic arthritis, is frequent, complicating 12% to65% of pediatric leukaemia, most often ALL. It is linked to specific damage by leukaemic infiltration of the synovium or less likely to a synovial reaction to adjacent periosteal or capsular infiltration. In JIA there are incomplete or atypical forms, the differential diagnoses to be sought are numerous and depend on age, personal and family anamnesis and clinical, biological, hematological and radiological signs. The dread of a haematological malignancy must constantly be sought and excluded before the use of CST, which is one of the therapeutic weapons in ALL, thus causing the blasts to disappear and delaying the diagnosis.

Conclusion
The diagnosis of JIA must be made with caution, raise the possibility of haematological malignancy in children in the absence of response to usual treatments, in the presence of systemic signs such as fever or a persistent biological syndrome or atypical radiological lesions, hence the correct analysis of the CBC and the blood and marrow smears is clearly important.
29 Juvenile idiopathic arthritis and osteogenesis imperfecta: an exceptional association
Authors: Emna Hannech , Soumaya Boussaid , Sonia Rekik , Samia Jemmali , Safa Rahmouni , Houda Ajlani , Hela Sehli , Mohamed Elleuch 
Published: October 2022

Abstract

Background
Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. Osteogenesis imperfecta (OI) is an autosomal dominant inherited disease defined by bone fragility due to abnormal collagen synthesis. It affects the entire skeleton, predisposing the patient to fractures.
We report an uncommon association between osteogenesis imperfecta and juvenile idiopathic arthritis.

Case presentation
A 15-year-old child presented to our rheumatology department with medical history of recurrent bone fractures due to low-energy trauma since the age of five years. The patient was born from a consanguineous marriage. He has been diagnosed with a polyarticular JIA since the age of eight years-old. The initial clinical presentation was a symmetric, cumulative, large and small joint polyarthritis. The rheumatoid factor was negative. No other autoantibodies were detected.
The patient was treated initially with methotrexate which was inefficient. He developed joint deformities. Then, biologic therapy was associated with a good response.
Clinical examination on entry showed severe joint deformities touching elbows, wrists and interphalangeal joints. Arms and thighs were curved and an unequal leg length was noted. There was no arthritis. The patient has bluish sclera. Neurological and dental examination was normal. There was no hearing loss. Laboratory findings showed a normal C-reactive protein. Calcium and phosphate serum levels were within normal ranges. On radiological investigations, radiographs showed excessive trabecular bone transparency, cortical bone thinning and incomplete bone fractures. Bone densitometry revealed a Z-score of the lumbar spine of -4.2 SD.
According to clinical, biological, and radiological investigations, the diagnosis of JIA associated with OI was confirmed clinically. Treatment with Pamidronate was initiated intravenously.

Conclusion
We reported an exceptional association between JIA and OI which occurred in a young Tunisian patient. We emphasize that this association should be considered when severe joints and extremities deformities occur.
Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this abstract.
Acknowledgements: declared none.
40 Arthralgia and celiac disease
Authors: Boutrid N , Rahmoune H , Bioud B , Amrane M 
Published: October 2022

Abstract

Background
In a large cohort of 2125 children followed in rheumatology, 2% had celiac disease (CD) (about 3 times the rate in the general population). Inversely, CD was reported in up to 2–3% of children with juvenile idiopathic arthritis

Objectives
Reporting joint damage such as arthrlagia in a celiac pediatric cohort

Material and method
We collected 58 celiac children and reported those with arthralgia and studied their clinical aspects

Results
Arthralgia was reported in 10/58 children (i.e. 17.24%) of the entire cohort studied (n = 58), including 09 children with positive HLA DQ2/DQ8 typing, potentially with an autoimmune background. The arthralgia was not accompanied by arthritis, and had an apyretic, labile and transient character. Only one case of episodic spinal pain was reported in the history

Conclusion
More than a sixth of the pediatric celiac cohort presents with arthralgia. These joint pains can be caused by vitamin D deficiency, osteoporosis, associated juvenile idiopathic arthritis (JIA), in particular HIV-positive. They can also be isolated and respond to the gluten-free diet.
54 Kawasaki disease: experience in a general paediatric department of the CHU de Annaba Algeria
Authors: Belamri D. J , Sehab H , Bouglouf S , Boutalbi N , Boustil L , Bouchair N 
Published: October 2022

Abstract

Background
Kawasaki disease (adeno-cutaneous-mucosal syndrome) is an acute febrile systemic vasculitis, affecting with predilection infants. Of unknown aetiology, its diagnosis is based on clinical criteria validated by the CDC and updated by the American Heart Association. Its prognosis is linked to the presence of coronary complications. Its prevalence in Algeria remains unknown, the increase in the number of cases in recent years has raised questions about its situation in our department.

Objective
To study the epidemiological, diagnostic, therapeutic, biological and evolutionary aspects of this disease.

Materials and methods
The authors report the results of a retrospective study of 2 years and 4 months, concerning 15 cases of Kawasaki disease hospitalized and treated in the pediatric department of the University Hospital of Annaba. The information was obtained from the hospitalization records.

Results
15 cases of Kawasaki disease were identified during the study period. Almost half of the patients were between 2 and 5 years of age, with an average age of 26.3 months (range: 11 months-9 years). A clear male predominance was noted (SR: 2). The reasons for hospitalization were dominated by fever (73%), skin involvement (66%) and stomatitis (40%). The clinical examination showed the frequency of the classical criteria of the disease with almost constant fever (100%), polymorphous skin rash 93.33%, oral-pharyngeal involvement 93.33%, involvement of hands and feet 40%, conjunctivitis 53.33% and adenopathies 46.67%. Biologically, the blood count showed anaemia (93.33%), hyperleukocytosis (66.67%) and hyperplateletosis (46.67%); an accelerated blood count in all our patients and an elevated CRP in 86.67% of cases. Doppler ultrasound performed in all patients returned normal in 73.33% of cases and found 2 cases of mitral insufficiency and 1 case of 8 mm coronary aneurysm. All our patients were treated with human immunoglobulins (2 g/kg IV) associated with aspirin 80–100 mg/kg/d in 4 doses. The evolution was favorable for all our patients.

Conclusion
Kawasaki disease is a pediatric vasculitis whose severity is related to coronary complications, which are prevented by early administration of IG and aspirin. It is therefore imperative to think of the disease in front of a prolonged fever associated with cutaneous and mucosal clinical signs and a biological inflammatory syndrome.
60 Childhood Behçet disease: clinical features, management and outcome of 13 cases
Authors: El Qadiry R , Nassih H , Bourrahouat A , Ait Sab I 
Published: October 2022

Abstract

Background
Behçet disease is a rare vasculitis that involves medium and small calibre arteries. Multisystem involvement is possible. Few are the papers that provide information about its clinical course, and outcome in the pediatric population. Also, the management is mainly based on adult guidelines.

Objectives
To shed light on the clinical course and outcome of juvenile Behçet disease.

Methods
A single center retrospective descriptive study of 13 children with Bechet disease. The mean follow-up period was for 3 years.

Results
We had 8 boys and 5 girls. Four of our patients were consanguineous. The mean age was 9 years. The mean interval between onset and diagnosis was 3 months. All of our patients had recurrent aphthous stomatitis with a median of 6 episodes per year. The other symptoms at diagnosis were concomitant genital ulcers in 4 cases, pseudo-folliculitis in 7 cases, urticarial rush in 4 cases, severe headache in 2 patients, stroke in one patient, prolonged and/or recurrent fever in 9 patients, uveitis in 4 cases, episcleritis in one case, arthritis in 5 cases, pulmonary hemorrhage in one case, and pyoderma gangrenosum in one case. The pathergy test was performed in all the patients, and was positive in 3 of them. Diagnosis was based on the International Criteria for Behcet's Disease. Acute phase reactants (ESR and CRP) were high in all patients. Meanwhile, granulocytosis was found in 10 patients. HLA 51 was positive in 8 patients, from whom three were siblings. All our patients were on steroids, five had colchicine, seven had azathioprine, two had methotrexate, two had cyclophosphamide, three had etanercept and one had tocilizumab. Complete remission was obtained in 8 cases in a mean time of 15 months, while five children had partial remission, and one boy lost sight of his left eye after a severe retinitis.

Conclusion
Behcet’s disease can cause severe sequalae if not diagnosed and treated on time. More studies are needed to establish management guidelines for pediatric population.