bluuebunny/crossref_metadata_2025_split huggingface dataset:
E17 Juvenile idiopathic arthritis: are girls less lucky?
Authors: Tbini Houssem , Rahmouni Safa , Rekik Sonia , Ammou Ahlem Ben , Zouaoui Khaoula , Boussaid Soumaya , Sahli Hela , Elleuch Mohammed 
Published: August 2023

Abstract

Introduction
Juvenile idiopathic arthritis (JIA) is one of the most common chronic diseases in children. ∼3 million children and young adults worldwide have JIA, with higher prevalence rates in girls.

Objectives
Our objective was to assess the relationship between gender and disease characteristics.

Methods
A retrospective study including adults with long-standing JIA according to International League of Associations of Rheumatology (ILAR) criteria over a 28-year period from 1994 to 2022. We collected clinical, biological, and radiological disease characteristics from medical records. These parameters were compared according to gender.

Results
We have included 29 patients (17 women and 12 men). The mean age was 35.69 ± 11.72 [18–61] years. Symptoms began at an average age of 11.10 ± 4.25 [2–16] years and have been evolving since 24.48 ± 12.76 [1–47] years. The average diagnostic delay was 52.96 ± 95.97 [0–336] months.
The mean body mass index (BMI) was 21.20 ± 4.88 [14.17–27.55] kg/m2, 8 patients had a normal BMI. Growth delay was noted in 3 cases.
Sixteen patients (55.2%) had a polyarticular form. The presence of rheumatoid factor (RF), ACPA and anti-nuclear antibodies was noted in 12, 7 and 5 cases, respectively.
At least one extra-articular manifestation was noted in 16 patients: ophthalmologic (n = 6), skin (n = 4), cardiac (n = 4), pulmonary (n = 3) and renal (n = 2) involvement. Osteoporosis was noted in 7 cases.
Mean C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and haemoglobin value level 42.74 ± 63.37 [2–218] mg/l, 69.67 ± 36.23 [36–108] mm, and 11.31 ± 2.10 [6.5–15] g/dl. An elevation of inflammation markers was noted in 19 cases. Anaemia was noted in 20 cases.
Bone erosions were noted in 18 cases. Hip and cervical spine involvement were noted in 14 and 4 cases, respectively.
Our comparative study showed that rheumatoid factor positivity was significantly associated with female gender (73.3% vs 16.7%; P = 0.018). No other significant gender differences were identified when comparing the rest of the clinical, biological, and radiological variables.
The correlation study concluded the following correlations of gender with haemoglobin level (r = -0.574; P = 0.001), rheumatoid factor positivity (r = 0.517; P = 0.016) and JIA subform (r = -0.482; P = 0.008).

Conclusion
A relationship of gender to rheumatoid factor positivity, haemoglobin level, and JIA subform was identified in our study. Larger-scale longitudinal studies would better investigate the influence of gender on JIA.
E19 Gamma-glutamyl transpeptidase level is correlated with disease activity in oligoarticular and polyarticular Juvenile idiopathic arthritis
Authors: Dhia Siwar Ben , Ferjani Hanene , Nessib Dorra Ben , Rabhi Emna , Kaffel Dhia , Maatallah Kaouther , Hamdi Wafa 
Published: August 2023

Abstract

Introduction
Gamma-glutamyl transpeptidase (GGT) level was found to be correlated with disease activity indexes in rheumatoid arthritis [1]. This association was not yet investigated in children with oligoarticular and polyarticular Juvenile idiopathic arthritis (JIA).

Objectives
The present study aims to investigate the correlation between GGT level and disease-related parameters in oligoarticular and polyarticular JIA.

Methods
We performed a retrospective study including patients with oligoarticular and polyarticular JIA responding to the 2001 International League of Associations for Rheumatology (ILAR) classification.
Sociodemographic, disease-related parameters (age of onset, disease duration, patient global assessment (PGA), Pain Visual Analogue Scale (VAS), C-Reactive protein (CRP) level, Erythrocyte Sedimentation Rate (ESR), disease activity assessed using the Juvenile Arthritis Disease Activity Score (JADAS) based on ESR), and GGT level were collected. Continuous variables were expressed with median and Interquartile range (IQR). Spearman correlation was used to assess the association between GGT levels and disease-related data.

Results
Eleven patients were included with a median age of 12 (IQR: 10–15) years and 8 females. Median disease duration was 4 (IQR: 4–7) years and median age of onset was 8 (IQR: 5–10) years. Five patients had oligoarticular JIA, 3 had a polyarticular with positive Rheumatoid Factor (RF) form, 2 had a polyarticular with negative RF form and one had an undifferentiated JIA. Median JADAS was 3.6 (IQR: 0–9). Median ESR, CRP, and GGT levels were 14 (10–19) mm/H, 1.8 (2–6.4) mg/l, and 14 (12–16) IU/l, respectively. A strong positive correlation was found between GGT level and JADAS (r = 0.749, P = 0.008). Level of CRP and ESR were not correlated with GGT level. Besides, patient global assessment and pain VAS were positively correlated with GGT level (r = 0.670, P = 0.049; r = 0.814, P = 0.014, respectively). No correlation was found between GGT level and disease duration and number of tender or swollen joints.

Conclusion
The GGT level was significantly higher in patients with more active disease, higher pain, and global assessment. This suggests that GGT level can be used as a marker of disease activity as in JIA.

References
1 Vergneault H, Vandebeuque E, Codullo V, Allanore Y, Avouac J. Disease Activity Score-28 using γGT permits a dual evaluation of joint activity and cardiovascular risk. J Rheumatol 2020.
E24 Does juvenile onset impact the serum levels of bone-forming biomarkers in adult patients with Spondyloarthritis?
Authors: Maatallah Kaouther 
Published: August 2023

Abstract

Introduction
The Wnt signalling pathway regulates bone metabolism. Dickkopf1 (DKK-1) and Sclerostin are potent inhibitors of this pathway, and their dysfunction would be responsible for bone formation phenomena in patients with spondyloarthritis (SpA). Osteoprotegerin (OPG), a potent inhibitor of osteoclasts, regulated by the Wnt pathway, has also been implicated in this bone ossification phenomenon. We aimed to assess the impact of juvenile-onset on the serum levels of DKK-1, sclerostin, and OPG in adult with spondyloarthritis.

Methods
It was a cross-sectional case control study including patients with SpA (meeting ASAS criteria). Data collected included sociodemographic and disease characteristics. Biomarker assays were performed using an ELISA technique.

Results
Among one hundred and nine included patients, seven patients had a juvenile onset of the disease (six males, one female). The mean age of disease onset was 12.8 ± 4.3 [5–16] years. The mean duration of SpA at inclusion was 15 ± 6 years [4 – 21]. Two (28.6%) patients had an axial form, and five (71.4%) had an axial and peripheral form. Three patients (42.9%) had entheseal involvement. The means of serum levels of DKK-1 and sclerostin in patients with juvenile-onset SpA and healthy controls were (132 ± 81 vs 122 ± 113; P = 0.439) and (27 ± 15 vs 39 ± 49; P = 0.985) respectively.
The analysis did not show significant differences in serum levels of DKK-1 (P = 0.293), sclerostin (P = 0.428), and OPG (P = 0.504) between patients with SpA with juvenile onset and adult SpA.

Conclusion
In our study the juvenile onset of the disease did not correlate with the inhibitors of the Wnt signalling pathway and OPG in patients with SpA.
E43 Multisystem inflammatory syndrome in children: an experience from Batna –Algeria
Authors: Hadef Djohra , Zerguine Halima , Bouderdaben Leila , Bechah Soumia , Kahlane Ibtissam , Benbouzid Nourelhouda , Bensacia Norhane , Brahmi Saida 
Published: August 2023

Abstract

Introduction
Multisystem Inflammatory Syndrome in Children (MIS-C) is a new phenomenon reported worldwide with temporal association with Covid-19. MIS-C is defined as a clinically serious condition requiring hospitalization with fever, multi-system organ dysfunction, inflammatory biomarkers increase.

Objectives
The aim of this study was to describe the demographic, clinical features, therapeutic aspects, complications, and outcomes of patients with Multisystem Inflammatory Syndrome in Children (MIS-C) in our region.

Methods
A retrospective descriptive study was conducted in the COVID-19 area of the Department of Pediatrics of the university hospital center (CHU Benflis Touhami Batna) over a three-year period from March 2019 to March 2022. Demographic and clinical data were collected.

Results
As at April 01, 2022, a total of 24 patients were hospitalized for MIS-C in our COVID-19 area of the Department of Pediatrics. The mean age was 5 years [3 months to 15 years]; 58% were male. The most common presenting symptoms were fever (88%), rash (60%), conjunctival injection (56%). 45% had gastrointestinal symptoms and 33% had respiratory symptoms. Elevated levels of C-reactive protein, d-dimer, and procalcitonin were found in 80%, 63%, and 50% of the patients, respectively. For treatment, intravenous immunoglobulin was used in 11 (46%), corticosteroids in 18 (75%). All patients were discharged home.

Conclusion
the incidence of MIS-C among children infected with SARS-CoV-2 is unclear as children often present with mild symptoms of Covid-19. This serious disease condition requires more surveillance and study.
Disclosure statement: None declared.
E46 Viruses reactivating each other’s!
Authors: Guedri R , Glai M , Jelassi A , Fitouri Z , Becher S Ben 
Published: August 2023

Abstract

Introduction
COVID-19 is known to cause an immunocompromised state. This condition was associated with reactivation of quiescent viruses.

Objectives
Describe a case of HHV-6 reactivation in a patient diagnosed with MIS-C syndrome.

Methods
The diagnosis of MIS-C syndrome associated to SARS-CoV-2 was done by following the criteria established by the World Health Organization (WHO). The reactivation of HHV6 was confirmed by positivity of RT-PCR in blood and presence of IgG in serology.

Results
A four years old girl, with no medical history who presented to emergency room with high fever associated with conjunctival injection, cheilitis, cervical adenopathy and rash since four days. She developed a generalized tonic-clonic seizure then a vasoplegic shock two days later. Thus, she was admitted in ICU. Clinical examination on admission showed polypnea, a low blood oxygen level, tachycardia, a low blood pressure (28/15mmhg), a hepatomegaly, a maculopapular rash in pelvic region and a bilateral eyelid swelling. Biology showed lymphopenia (660 109/l), hepatic cytolysis, low prothrombin test (19%), an acute renal failure, a biological inflammatory syndrome with an elevated C-reactive protein (274 mg/l), an elevated ferritin, increased d-dimers (1µg/ml) and fibrinogen level which suggest activation of the coagulation cascade with a prothrombotic state and elevated lactatemia (6.4 mmol/l). The chest X-ray was normal. She had a low ejection fraction (39%) showed on echocardiogram. A rapid SARS-CoV-2 test was negative but serology was positive. Since MIS-C syndrome was not known to give severe hepatitis, other diagnoses was proposed. RT-PCR in serum associated to RT-PCR in cutaneous lesion’s biopsy was positive for HHV6. The hepatitis A serology was negative. She has an anterior immunity to varicella-zoster (VZV). She was intubated. She received vasoactive medications, intravenous immunoglobulin (IVIG) and steroids. Course was marked by hemolytic anaemia, which was associated to immunoglobulin (IVIG). Our patient become rapidly afebrile. Vital signs and laboratory parameters of inflammation, CRP, lymphopenia, markers of cardiac injury, transaminases and renal function parameters all normalized. On day 28 of hospital stay, she was discharged with full dose of steroids and low dose of aspirin.

Conclusion
HHV6 reactivation needs a state of immunosuppression and was reported with acquired immunodeficiency syndrome (AIDS) and a variety of lymphoproliferative disorders. It leads to fever, cutaneous eruption as well as more serious complication such as hepatitis, which is the case with our patient.

Ethics
No conflicts of interest.
E49 Behçet's disease associated with Warkany syndrome: a case report
Authors: Hadef Djohra , Zerguine Halima , Behamma Hayette , Benbouzid Nourhane , Rougui Mohamed Fateh 
Published: August 2023

Abstract

Background
Behçet's disease is a rare chronic, relapsing, multisystem vasculitis characterized by mucocutaneous lesions, articular, ocular, vascular, and central nervous system manifestations, of which pediatric forms may be syndromic. Warkany syndrome is a rare genetic anomaly defined by trisomy 8, characterized clinically by facial dysmorphia, mild intellectual disability, and skeletal anomalies.

Aim
We report the case of a 9-year-old boy diagnosed with Behçet's disease and whose examination revealed an associated Warkany syndrome.

Observation
the boy was from a non-consanguineous marriage, correctly vaccinated, with a history of congenital dislocation of the hip operated at the age of 3 years and obstruction of the lacrimal duct operated at the age of 6 years, who presented with chronic bilateral synechial pan uveitis, recurrent oral aphthosis (&gt;3 times a year), multiple dysostosis with chronic diffuse arthralgias: hip, knees, shoulders. The clinical examination reveals a facial dysmorphia (elongated skull, high and prominent forehead, hypertelorism, sunken eyes, wide nose with anteverted nostrils, micrognathia with eversion of the lower lip, large ears with a thick rim), organomegaly (hepatosplenomegaly, large kidneys), a moderate intellectual deficit and deep palmar and plantar folds Auditory evoked potential finds bilateral deafness. Inflammatory workup was positive, HLAb51 typing was positive. MPS enzyme assay is normal. Genetic study: not done due to lack of means.

Conclusion
Behçet's disease in children can be associated with other genetic syndromes, it remains to be seen if it is a simple fortuitous association.
E64 Clinical, radiological, and etiological profile of hip pain in children
Authors: Taboubi F , Lassoued Ferjani H , Ben Nessib D , Belhaj W , Kaffel D , Maatallah K , Hamdi W 
Published: August 2023

Abstract

Background
Hip pain is a frequent complaint in the pediatric population, in orthopedics, and in rheumatology. In children, the clinical features of hip pain vary widely according to age. This clinical variability represents a diagnosis challenge for clinicians. This study aimed to identify the clinical, paraclinical, and etiological spectrum of hip pain in children.

Methods
A retrospective, descriptive study was conducted between January 2017 and December 2020. We included children aged under 16 years who were hospitalized in the rheumatology and/or pediatric orthopedics department at Mohamed Kassab orthopedics institute for non-traumatic hip pathology. Clinical, biological, and radiological data were collected from recorded medical files. Missed anamnestic data were collected from parents by telephone call.

Results
We included 81 children. Male predominance was noted (48 boys and 33 girls). The mean age was 9.9 ± 4.4 years with extremes from 1.3–16 years. Limping was the presenting feature in 84.4% of children, followed by pain (76.5%) and refusal to bear weight (12.3%). The mean time of onset to presentation was 8.8 ± 21.3 months. The mean diagnosis delay was 3.5 ± 15.3 months with extremes from 0 to 10 years. Fifty-four (66.7%) children visited their physician at least once before diagnosis. In clinical examination, fever was found in 12 children, and local inflammatory signs in 2 children. Leg length discrepancy was noted in 13 children, hip joint pain in 47 children, and restricted hip movement in 58 children. Elevation of erythrocyte sedimentation rate was noted in (23/28) of children and elevation of C reactive protein in (14/33) of children. An elevated leucocyte count was present in (10/79) patients. Radiographs showed anomalies in 76.5% of cases and Ultrasonography in 90.9% of cases. The final diagnoses of our sample were: slipped capital femoral epiphysis (37%), juvenile idiopathic arthritis (18.5%), hip dysplasia (14.8%), septic arthritis (12.3%), transient synovitis (8.6%) and tumors (4.9%). The chirurgical approach was conducted in 71.6% of patients. Most children (72.5%) had benign outcomes.

Conclusion
The diagnosis of pediatric hip pain should always follow a standard algorithm. This starts with taking a detailed history from the patient, if possible, and parents and conducting a thorough clinical examination; imaging and/or blood tests may follow if necessary.

Ethics
The authors declare that they have no conflict of interest.
E68 Kawasaki Disease before and during COVID-19
Authors: Guedri R , Jelassi A , Meriem Glai , Hrizi H , Essaddam L , Fitouri Z , Ben Becher S 
Published: August 2023

Abstract

Introduction
Kawasaki disease is a febrile systemic vasculitis of children, which is suspected to be triggered by respiratory viral infections. We further note, with the outbreak of COVID 19, an increase in incidence with a change in the profile of Kawasaki disease.

Objective
To describe the epidemiological, clinical and evolutionary characteristics of children managed for Kawasaki or Kawasaki-Like syndrome before and during the Covid pandemic19.

Materials and Methods
This is a retrospective, descriptive study conducted in the PUC department of the Béchir Hamza children's hospital in Tunis, over a period of 05 years from 1 January 2017–31 December 2021.

Results
We collected 30 cases of patients hospitalized with Kawasaki disease, between January 2017 and December 2021: 11 cases with Kawasaki syndrome before COVID-19; and 19 cases with Kawasaki syndrome after COVID-19. The mean age of diagnosis was 42 months before the pandemic vs 81 months after the pandemic. The sex ratio was 0.5 before the pandemic vs 0.7 after the pandemic. Viral disease was reported in 0 cases before the pandemic vs 4 cases with confirmed COVID contacts after the pandemic. Fever was the main symptom in both periods, with a mean maximum temperature of 39.7°C before the pandemic vs 39.5°C after the pandemic. The delay of fever from diagnosis was 7.8 days before the pandemic vs 5 days after the pandemic. Cheilitis was reported in 10 cases before the pandemic vs 16 case after the pandemic. Palmoplantarerythema was reported in 1 case before the pandemic vs 7 cases after the pandemic. Scaling was found in 8 cases before the pandemic vs 2 cases after the pandemic. The rash was reported in 9 cases mainly purpuric before the pandemic vs 17 cases after the pandemic, maculopapular. Abdominal involvement was found in 2 cases before the pandemic vs 14 cases after the pandemic. Clinical cardiac involvement was found in 2 cases before the pandemic vs 3 cases after the pandemic. The TTE was pathological in 2 cases before the pandemic, with coronary aneurysm vs 7 cases after the pandemic, with heart failure. All cases received immunoglobulin with a mean time to apyrexia of 26 h before the pandemic vs 25 h after the pandemic. The mean time to hospitalization was 8 days before the pandemic vs 7.3 days after the pandemic. No deaths occurred.

Conclusion
The change in the profile and incidence of Kawasaki disease following the emergence of COVID-19 must be taken into account in order to manage an influx of patients with severe Kawasaki disease.
Management of postpartum perineal wound complications
Authors: Saad Jaber , Painter Caitlyn 
Published: August 2023

Purpose of review
Perineal wound complications occur in up to 25% of postpartum patients. Wound complications are most common after obstetric anal sphincter injuries (OASIS) but can occur after any laceration. It is imperative that any provider caring for postpartum patients understand the best evidence-based practices to recognize and manage these complications. We present a review of the available literature on the management of postpartum perineal wound complications.

Recent findings
There is a paucity of new publications on the management of postpartum perineal wound complications, despite an increased emphasis on postpartum recovery in women's health. The role of topical estrogen in healing of perineal wounds was investigated in a pilot study, demonstrating that granulation tissue does express estrogen receptors, and the use of estrogen increases cell proliferation. Progression of perineal wound healing by secondary intention was evaluated in an observational study. Wound healing was delayed in 30% of women, with the initial wound area, perimeter, bacterial colonization, and OASIS being associated with delayed healing.

Summary
Evidence based practices on timing of follow-up, addressing wound care and analgesia, administrating antibiotics, timing secondary repair, and surgical technique all play a role in optimizing recovery and reducing morbidity in patients with postpartum perineal wound complications.
P2RX7 gene knockout or antagonism reduces angiotensin II-induced hypertension, vascular injury and immune cell activation
Authors: Shokoples Brandon G. , Berillo Olga , Comeau Kevin , Chen Hao Yu , Higaki Akinori , Caillon Antoine , Ferreira Nathanne S. , Engert James C. , Thanassoulis George , Paradis Pierre , Schiffrin Ernesto L. 
Published: August 2023

Objective:
Extracellular ATP is elevated in hypertensive mice and humans and may trigger immune activation through the purinergic receptor P2X7 (P2RX7) causing interleukin-1β production and T-cell activation and memory T-cell development. Furthermore, P2RX7 single nucleotide polymorphisms (SNP) are associated with hypertension. We hypothesized that P2RX7 activation contributes to hypertension and cardiovascular injury by promoting immune activation.

Methods:
Male wild-type and P2rx7

–/–
mice were infused or not with angiotensin II (AngII) for 14 days. A second group of AngII-infused wild-type mice were co-infused with the P2RX7 antagonist AZ10606120 or vehicle. BP was monitored by telemetry. Cardiac and mesenteric artery function and remodeling were assessed using ultrasound and pressure myography, respectively. T cells were profiled in thoracic aorta/perivascular adipose tissue by flow cytometry. Associations between SNPs within 50 kb of P2RX7 transcription, and BP or hypertension were modeled in 384 653 UK Biobank participants.

Results:

P2rx7 inactivation attenuated AngII-induced SBP elevation, and mesenteric artery dysfunction and remodeling. This was associated with decreased perivascular infiltration of activated and effector memory T-cell subsets. Surprisingly, P2rx7 knockout exaggerated AngII-induced cardiac dysfunction and remodeling. Treatment with a P2RX7 antagonist reduced BP elevation, preserved mesenteric artery function and reduced activated and effector memory T cell perivascular infiltration without adversely affecting cardiac function and remodeling in AngII-infused mice. Three P2RX7 SNPs were associated with increased odds of DBP elevation.

Conclusion:
P2RX7 may represent a target for attenuating BP elevation and associated vascular damage by decreasing immune activation.