bluuebunny/crossref_metadata_2025_split huggingface dataset:
P036 Juvenile Idiopathic Arthritis in adulthood: outcomes of oligo and polyarticular forms?
Authors: Saoussen Miladi , Yasmine Makhlouf , Alia Fazaa , Mariem Sellami , Kmar Ouenniche , Leila Souabni , Selma Kassab , Selma Chekili , Kawther Ben Abdelghani , Ahmed Laatar 
Published: November 2021

Abstract

Background
Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic disease of childhood that can cause major physical disability, impairing patients' ability to lead a normal adult life. The objective of this study was to assess the outcomes of oligo- and polyarticular JIA in adulthood as well as the progression to authentic rheumatoid arthritis (RA).

Methods
We conducted a retrospective study including adult patients with JIA (diagnosed according to the International League of Associations for Rheumatology [ILAR]). Data collected included age, gender, disease characteristics (JIA subtype, disease duration). The progression to an authentic RA was determined by checking the response to the ACR/EULAR 2010 classification criteria.

Results
Twenty-eight patients were included in this study. There was a female predominance with a sex ratio of 1.5. The mean age of disease onset was 6 years and 2 months [2–17]. The mean age at inclusion was 29.5 years [18–64]. The frequency of each JIA subtype was as follows: polyarticular with RF + (n = 14), polyarticular with RF- (n = 9), oligoarthritis (n = 5). The polyarticular forms: RF+ and RF- evolved into genuine seropositive and seronegative RA in 71.4% and 66.7% of cases, respectively.
Among the oligoarticular JIA subtype, an extension of the disease to a seronegative RA was noted in one patient (20%). Hip involvement was noted in 20% of patients. Carpal tunnel syndrome and elbow arthritis were found in 28.5% and 14.3% of patients, respectively. Regarding treatment modalities and at the time of recruitment, 36.3% of RAs were treated with Methotrexate, and 33% were on biological treatment. Ten percent of the patients dropped out of school because of disease flares and deformities.

Conclusion
Our study showed oligo- and polyarticular JIA evolves in most of the cases towards an authentic RA causing deformities and handicaps. Earlier management is necessary to allow a better transition to adulthood.
P079 Multi-systemic inflammatory syndrome in children: skin tropism
Authors: Guedri Rahma , Glai Mariem , Fitouri Zohra , Ben Becher Saayda 
Published: November 2021

Abstract

Background
Multisystem inflammatory syndrome in children (MIS-C) is a severe immune-mediated syndrome that occurs after COVID-19 infection. It mainly affects children and presents several clinical aspects. The cutaneous and mucous signs are very much part of the diagnostic criteria. The aimisto describe the clinical and evolutionary aspects of the muco-cutaneous signs during MIS-C

Patients and methods
It was a descriptive retrospective study conducted over a period of 9 months (March 1, 2020 - December 31, 2020) including children admitted to COVID isolation unit with suspicion of MIS-C syndrome.

Results
We included 17 patients. The average age was 7 years (1–11 years). The sex ratio was 2.2 (11 boys and 6 girls). There was o history of dermatological disease has been reported. Fever was present in all patients. Mucosal signs were present in 13 patients. Conjunctival hyperemia without purulent secretions was noted in 12 patients. Conjunctivitis was bilateral in 11 cases. A rash was found in 10 cases. It was macular (6 cases), maculopapular (2 cases) and vesicular cluster in one 1 case. It was itchy in 2 cases. It was located in the limbs (7 cases), thorax and/or abdomen (5 cases), pelvis (4 cases), palms and/or soles (3 cases) and the face in one case. Cheilitis was found in 6 cases and stomatitis or glossitis in 5 cases. oEdema of the extremities was present in three patients and oedema of the face was noted in one patient. All children received intravenous immunoglobulin therapy combined with high-dose corticosteroids and acetylsalicylic acid at anti-aggregating doses. The course was marked by the disappearance of the muco-cutaneous signs without recurrence in all cases.

Conclusion
Mucocutaneous involvement is a characteristic manifestation of multisystem inflammatory syndrome. It is one of the various diagnostic criteria for this syndrome.
P084 Recurrent fever in children: why not a mevalonate kinase deficiency?
Authors: Ouahbi K , Gacem O , Boukandoura F , Benmekhmouf H , Hamadouche N , Hadjyoucef N , Chouiki H , Mehaba N , Abba K , Mansouri N , Achir M , Ladj M S 
Published: November 2021

Abstract

Background
Mevalonate kinase deficiency (MKD) is a rare autosomal recessive auto inflammatory disease. The clinical spectrum of this disease is a continuum ranging from the moderate form of Hyper-IgD syndrome (HIDS) to lethal forms of mevalonic aciduria (MA). An autoinflammatory disease should be considered in children with recurrent fever of unexplained origin.

Case report
M, 28 months old boy from a non-consanguineous marriage of Algerian parents, presented since the age of two months’ episodes of unexplained fever resistant to antibiotics and requiring several hospitalizations. Family history found that the mother and a maternal uncle experienced an unexplained recurrent fever. Personal history found episodes of fever lasting in average 6 days, with no obvious cause and recurring every 15–20 days; associated with bilateral cervical adenopathy, mouth ulcers, arthralgia, abdominal pain with vomiting and diarrhea. Clinical examination confirmed the fever with temperature of 38.7°, Chills and irritability, Multiple cervical adenopathy oral aphthae, tongue of geographical aspect, cervical pain and large joint arthralgia, tender abdomen with no organomegaly. Biologic workups found major inflammatory syndrome with hyper leucocytosis at 14X103/mm3 predominantly polynuclear, high CRP level, high ESR at 110 mm. Blood cultures and viral serologies were negative. Immunoelectrophoresis found elevated IgA, IgG and IgM with a normal IgD level. Chest X-ray and abdominal ultrasound were normal. The diagnosis of MKD in its moderate form was supported by elevated urinary mevalonic acid excretion to 5.1 mmol/mol during the febrile episode and subsequently confirmed by a 2nd contributory urine assay during another febrile episode.

Conclusion
MKD is a rare disease. The clinical spectrum of this condition is variable and could benefit of an effective treatment. Our patient developed a moderate form with a better long-term prognosis.
P091 Osteoid osteoma of the trapezoid bone: a diagnostic pitfall of a wrist chronic monoarthritis
Authors: Rym Fakhfakh , Baccouche Khadija , Elamri Nejla , Laataoui Sadok , Zegaloui Hela , Bouajina Elyes 
Published: November 2021

Abstract

Background
Osteoid osteoma is a benign bone tumor representing ∼10% of all benign bone tumors. Although osteoid osteoma of the long bones is usual, the location in the carpus, especially, in the trapezoid bone is rare [1,2]. The Objective is toreport a case of osteoid osteoma of the trapezoid bone with extension to the adjacent second metacarpal bone that was missed diagnosis at initial presentation.

Methods
We describe the clinical, radiological features and outcomes after surgical resection.

Results
A 24-year-old right-handed female, presented with a three-year history of pain at the left wrist, particularly, in the radial-side. She didn’t report any trauma to the wrist. The pain increased over time. She couldn’t use her hand in daily activities and could hardly sleep because of increasing pain at night. Before being referred to our department, she consulted a surgeon. She was treated with oral anti-inflammatory drugs, steroids, several intraarticular steroid injections without relieving pain. The Magnetic resonance imaging (MRI) showed bone marrow oedema in the trapezium and the trapezoid bone, synovial effusion, and tenosynovitis of the flexors of the thumb. A synovectomy was performed and the histopathology revealed non-specific chronic inflammation.
One year later in our department, examination showed wrist swelling, tenderness and pain on palpation. The erythrocyte sedimentation rate was 12 mm/h and the mean C-reactive protein was 2 mg/l. Autoantibodies testing were negative. Initial radiography was read as normal. Initially, Naproxen (550 mg/day) and Morphine (60 mg/day) were prescribed with partially relieving pain. Scintigraphy showed increased uptake of 99mTc in the radial-side of the left wrist. Then, a bone tumor was suspected. The Computed tomography (CT) images revealed a well-demarcated sclerotic nidus surrounded by a halo of radiolucent osteoid tissue, consistent with an osteoid osteoma of the trapezoid bone and the adjacent second metacarpal bone. Surgical resection of the osteoid osteoma was performed. Histopathological examination confirmed the diagnosis. The patient reported complete pain relief since her surgery. She had no recurrence to date.

Conclusion
Osteoid osteoma should be in the differential diagnosis list of chronic wrist pain/arthritis. In this case, careful attention is necessary for patients unresponsive to treatment with long-lasting symptoms. If radiographs are inconclusive, CT or MRI should be considered to better visualize the nidus of osteoid osteoma in the small bones of the hand.
P095 Camptodactyly, arthropathy, coxa-vara, pericarditis syndrome: a case report
Authors: Ferhat A , Mechid F , Rahmoune A , Ifticene M A , Benaziez R , Aboura R , Dahou-Makhloufi C 
Published: November 2021

Abstract

Background
Camptodactyly, arthropathy, coxa vara, pericarditis (CACP) syndrome is a rare genetic disorder with autosomal recessive transmission including camptodactyly, synovial hyperplasia-related arthropathy, progressive coxa vara deformity and non-inflammatory pericarditis. We report the observation of a case.

Observation
S.Y aged 8.5 years, from a consanguineous marriage, presented with a chronic arthropathy affecting wrists, knees and ankles that had been evolving for 6 years and currently, elbows are also affected. The history includes surgery for claw deformity of the hands at the age of 3. No similar cases in the family.
Osteoarticular examination showed symmetrical swelling of the elbows, wrists (Fig. 1),knees (Fig. 2) and ankles (Fig. 3.The affected joints were neither red, painful nor warm on palpation, with normal mobility.
The onset of symptoms could not be determined due to the indolence of the condition and the rest of the clinical examination was unremarkable.

Discussion
CACP syndrome is a more common condition in the Middle East and North Africa, with about 20 cases reported worldwide. This syndrome is still poorly understood and is often confused with juvenile idiopathic arthritis. It should be suspected in the presence of any congenital claw deformity of the hands (camptodactyly) with a chronic non-inflammatory arthropathy, which are constant signs. Coxa-vara and pericarditis (found respectively in 60 and 30% of cases) should be systematically sought. The consanguinity reported in the literature is also present in our case. However, there are no similar cases in siblings. The diagnosis is important, thus avoiding the initiation of unnecessary treatments such as corticosteroids, DMARDs or biotherapy.

Biology
no inflammatory syndrome, FAN negatives.

Standard X-ray of the pelvis
presence of a coxa-vara on the right

Joint ultrasound
common tenosynovitis of the extensors of the fingers, effusion with synovial hypertrophy without a Doppler catch of the elbows, knees and ankles
Myelogram without abnormality.
Cardiac ultrasound without pericarditis.
The diagnosis of CACP was made in view of: a history of camptodactyly, chronic non-inflammatory arthropathy and coxa-vara.
Analgesic treatment was instituted in case of pain, with cardiac monitoring by ultrasound every 6 months.

Conclusion
CACP syndrome is a rare disease often confused with juvenile idiopathic arthritis. Congenital camptodactyly and non-inflammatory arthropathy are very evocative of the diagnosis. The absence of similar cases in siblings makes our observation special.
O002. Factors facilitating the self- and shared-management of Juvenile Idiopathic Arthritis
Authors: Stones Simon 
Published: November 2021

Abstract

Background
Juvenile idiopathic arthritis (JIA) requires some form of lifelong management, with at least one third of children symptomatic in adulthood. Therefore, empowering children to competently self-manage their health and wellbeing across the lifecourse is logical, while supporting families in their shared-management role during childhood. However, there was a limited theoretical basis to the self- and shared-management of JIA across the lifecourse. The objective is to explore the factors facilitating the self- and shared-management of JIA using a realist approach to evaluation.

Methods
Guided by the Individual and Family Self-management Theory, a three-stage realist approach to evaluation was undertaken: 1) initial JIA self- and shared-management question theories were elicited from literature reviews and stakeholder insights [1]; 2) seven initial question theories were tested using teacher-learner cycle interviews with 20 participants; 3) findings were analysed using a theory-driven approach to thematic analysis, using deductive, inductive, and retroductive reasoning to extend or refute the initial question theories, in order to identify demi-regularities in the data.

Results
Six refined JIA self- and shared-management question theories emerged: 1) meaningful and bespoke self-management support across the life course for children and young people with JIA; 2) recognised and valued shared-management support for the families of children and young people with JIA, with autonomy in mind; 3) individual healthcare plans as a shared management communication tool to facilitate optimal management of JIA; 4) consistent recognition, value, and encourage of self- and shared-management support from the paediatric rheumatology multi-disciplinary team and associated professionals; 5) child, young-person, and family-focused paediatric rheumatology care and support services across the lifecourse; and 6) bespoke and inclusive approaches by education providers to enable children and young people with JIA to feel safe, supported, and able to fulfil their potential.

Conclusion
There is an increasing recognition of the importance of self- and shared-management of JIA and other paediatric-onset chronic conditions. However, there is a lack of an overall, cohesive approach to self- and shared-management between healthcare providers, education providers, and patient/parent organisations. The findings from this study illuminate the factors facilitating JIA self- and shared-management at individual, interpersonal, institutional and infrastructural levels, bearing relevance to individuals and organisations involved in caring for, and supporting children with JIA and their families.
Protestant Missionaries Are Associated With Reduced Community Cohesion
Authors: Hager Anselm 
Published: November 2021

AbstractDo Protestant missionaries affect community cohesion? This study puts forth two mechanisms that link missionaries to trusting, cooperative community life: pro-social preferences and social networks. On the one hand, Protestant missionaries espouse charity, and they establish regular venues of social interaction. On the other hand, Protestant missionaries propagate an individualist faith, and they provide an identity along which communities may separate. The effect of Protestant missionaries on community cohesion is thus unclear. To make headway on these conflicting theoretical predictions, we study variation in missionary activity in southeastern Peru. We document that villages with Protestant missions show lower levels of community cohesion compared to non-missionized, Catholic villages. We point to weakened networks as the most likely causal channel and show that effect sizes are particularly large among Pentecostal missionaries.
Devaluing Women’s Lives through Law: Familyism Ideologies in Abortion and Violence against Women Laws in El Salvador
Authors: Diossa-Jiménez Leydy , Menjívar Cecilia 
Published: November 2021

AbstractEl Salvador’s violence against women (VAW) and antiabortion laws present optimal empirical ground to examine the intersection of familyism ideology, laws, and the state relevant beyond this case. Analyzing legal documents, content of laws, and newspapers, we juxtapose these two laws that have followed different applications within the same socio-legal context and historical time, legal reasoning, and juridical structure to identify a common thread: the control of women’s bodies and devaluation of women’s lives enshrined in the legal system. “Familyism” ideology embedded in the law prioritizes family over women’s rights where social class emerges as a central factor. The analysis centers the state, as it interacts with and responds to pressures from the international community and domestic political forces to create, align, and implement antiabortion and VAW laws while devaluing gender ideologies that seek to protect women. In sum, both laws prioritize family at the expense of women’s rights and lives, especially poor and socially disadvantaged women.
Dynamic Risk Prediction for Hospital-Acquired Pressure Injury in Adult Critical Care Patients
Authors: Shui Amy M. , Kim Phillip , Aribindi Vamsi , Huang Chiung-Yu , Kim Mi-Ok , Rangarajan Sachin , Schorger Kaelan , Aldrich J. Matthew , Lee Hanmin 
Published: November 2021

IMPORTANCE:
Accurately measuring the risk of pressure injury remains the most important step for effective prevention and intervention. Time-dependent risk factors for pressure injury development in the adult intensive care unit setting are not well understood.

OBJECTIVES:
To develop and validate a dynamic risk prediction model to estimate the risk of developing a hospital-acquired pressure injury among adult ICU patients.

DESIGN:
ICU admission data were split into training and validation sets. With death as a competing event, both static and dynamic Fine-Gray models were developed to predict hospital-acquired pressure injury development less than 24, 72, and 168 hours postadmission. Model performance was evaluated using Wolbers’ concordance index, Brier score, net reclassification improvement, and integrated discrimination improvement.

SETTING AND PARTICIPANTS:
We performed a retrospective cohort study of ICU patients in a tertiary care hospital located in San Francisco, CA, from November 2013 to August 2017.

MAIN OUTCOMES AND MEASURES:
Data were extracted from electronic medical records of 18,019 ICU patients (age ≥ 18 yr; 21,220 encounters). Record of hospital-acquired pressure injury data was captured in our institution’s incident reporting system. The information is periodically reviewed by our wound care team. Presence of hospital-acquired pressure injury during an encounter and hospital-acquired pressure injury diagnosis date were provided.

RESULTS:
The dynamic model predicting hospital-acquired pressure injury more than 24 hours postadmission, including predictors age, body mass index, lactate serum, Braden scale score, and use of vasopressor and antifungal medications, had adequate discrimination ability within 6 days from time of prediction (c = 0.73). All dynamic models produced more accurate risk estimates than static models within 26 days postadmission. There were no significant differences in Brier scores between dynamic and static models.

CONCLUSIONS AND RELEVANCE:
A dynamic risk prediction model predicting hospital-acquired pressure injury development less than 24 hours postadmission in ICU patients for up to 7 days postadmission was developed and validated using a large dataset of clinical variables readily available in the electronic medical record.
Low-level viremia in nucleoside analog-treated chronic hepatitis B patients
Authors: Zhang Qian , Cai Da-Chuan , Hu Peng , Ren Hong 
Published: November 2021

Abstract
Low-level viremia (LLV) was defined as persistent or intermittent episodes of detectable hepatitis B virus (HBV) DNA (<2000 IU/mL, detection limit of 10 IU/mL) after 48 weeks of antiviral treatment. Effective antiviral therapies for chronic hepatitis B (CHB) patients, such as entecavir (ETV), tenofovir disoproxil fumarate (TDF), and tenofovir alafenamide (TAF), have been shown to inhibit the replication of HBV DNA and prevent liver-related complications. However, even with long-term antiviral therapy, there are still a number of patients with persistent or intermittent LLV. At present, the research on LLV to address whether adversely affect the clinical outcome is limited, and the follow-up treatment for these patients is open to question. At the same time, the mechanism of LLV is not clear. In this review, we summarize the incidence of LLV, the association between LLV and long-term outcomes, possible mechanisms, and management strategies in these patient populations.
Efficacy and safety of a parylene-coated occluder for atrial septal defect: a prospective, multi-center, randomized controlled clinical trial
Authors: Yang Kai , Lyu Jian-Hua , Hu Hai-Bo , Wu Wen-Hui , Zhang Ge-Jun , Jin Jing-Lin , Liu Yu-Hao , Feng Yuan , Wang Li-Ming , Zhao Shi-Hua , Jiang Shi-Liang , Li Wei , Pan Xiang-Bin 
Published: November 2021

Abstract

Background:
Nitinol-containing devices are widely used in clinical practice. However, there are concerns about nickel release after nitinol-containing device implantation. This study aimed to compare the efficacy and safety of a parylene-coated occluder vs. a traditional nitinol-containing device for atrial septal defect (ASD).

Methods:
One-hundred-and-eight patients with ASD were prospectively enrolled and randomly assigned to either the trial group to receive a parylene-coated occluder (n = 54) or the control group to receive a traditional occluder (n = 54). The plugging success rate at 6 months after device implantation and the pre- and post-implantation serum nickel levels were compared between the two groups. A non-inferiority design was used to prove that the therapeutic effect of the parylene-coated device was non-inferior to that of the traditional device. The Cochran–Mantel–Haenszel chi-squared test with adjustment for central effects was used for the comparison between groups.

Results:
At 6 months after implantation, successful ASD closure was achieved in 52 of 53 patients (98.11%) in both the trial and control groups (95% confidence interval (CI): [−4.90, 5.16]) based on per-protocol set analysis. The absolute value of the lower limit of the 95% CI was 4.90%, which was less than the specified non-inferiority margin of 8%. No deaths or severe complications occurred during 6 months of follow-up. The serum nickel levels were significantly increased at 2 weeks and reached the maximum value at 1 month after implantation in the control group (P < 0.05 vs. baseline). In the trial group, there was no significant difference in the serum nickel level before vs. after device implantation (P > 0.05).

Conclusions:
The efficacy of a parylene-coated ASD occluder is non-inferior to that of a traditional uncoated ASD occluder. The parylene-coated occluder prevents nickel release after device implantation and may be an alternative for ASD, especially in patients with a nickel allergy.