bluuebunny/crossref_metadata_2025 huggingface dataset:
07 Predictor 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

Background

Juvenile idiopathic arthritis (JIA) is one of the commonest rheumatic diseases 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 joint replacement surgery. Little studied in the literature, the predictors of hip involvement are still unknown.

Objectives

Our study aims to identify the clinical, biological characteristic of patients with hip involvement and determine the associated risk factors.

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 of 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), psoriatic-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. Sixteen patients were under DMARDs (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 eight (50%) presenting limited range of motion and 10 (62.5%) developing radiological evidence of hip damage. Hip involvement was associated with a longer disease duration (p = 0.051). JADAS score value of patients with coxitis was higher (Mean 8.35 vs 7.05) but not significantly (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 significantly (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), nor 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 sizes are needed to draw definite causal associations.
08 Juvenile idiopathic arthritis and corticosteroid-induced osteonecrosis of the femoral head
Authors: Benaziez F , Remilaoui A , Bahaz N , Yettou S , Dahou Makhloufi C
Published: October 2022

Background

Juvenile idiopathic arthritis (JIA) is a group of inflammatory joint diseases that begin before the age of 16. Depending on the clinical form of JIA, medical treatment may involve long-term high-dose corticosteroid therapy, which may lead to aseptic femoral head osteonecrosis of patients.

We report three observations of aseptic osteonecrosis of the femoral head in patients followed for JIA.

1st observation: Mr I.A, aged 19 years, presented with ankylosing spondylitis with peripheral involvement, with a juvenile onset, for which he had received a high dose of corticosteroids at an average of 1.5 mg/kg/day since the age of 15 months on an intermittent basis. Clinically, the patient had a height of 1.65, a right limp when walking and limited movement of the right hip with a Lequesne algofunctional index of 10.5 (significant disability). MRI of the pelvis showed aseptic osteonecrosis of the right femoral head (ONATF), Arlet and Ficat stage 3.

Observation 2: Mr A.B, 27-year-old, had systemic JIA since the age of 12. He received corticosteroid therapy at an average dose of 1 mg/kg/day intermittently. Clinically the patient presents with signs of cortisone impregnation (a cushingoid face, purple stretch marks, thin skin) and bilateral cataract. The patient walks with the help of a crutch. The examination of the hips revealed a limitation of mobility of the left hip with a Lequesne algo-functional index of 18.5. Bone densitometry showed a decrease in bone density. Standard radiographs noted a left ONATF, Arlet and Ficat stage 4 with L4 and L5 vertebral fractures.

3rd Observation: Mr B.M, 26-year-old, followed for systemic JIA since the age of 14. He was treated with high dose corticosteroids at a rate of 1 mg/kg/day. The clinical examination revealed unevenness of the lower limbs, a left limp when walking, limited internal rotation and abduction of the right hip. The patient also had multiple inflammatory acne lesions and a bilateral cataract. Bone densitometry showed a decrease in bone density. Standard radiography showed bilateral ONATF, stage 3 on the right and 4 on the left according to Arlet and Ficat's classification, which required the placement of a double total hip replacement.

Conclusion

Corticosteroid therapy is one of the main risk factors for non-traumatic aseptic osteonecrosis. Our three patients presented with ONAT with major disability following long-term corticosteroid therapy. The efficacy of corticosteroids is well established but they should be used with caution to avoid side effects
09 Validation of the francophone version of pGALS with the Delphi approach
Authors: Hanen Ferjani , Emna Rabhi , Lilia Loucif , Djohra Hadef , Rawdha Tekaya , Sami Slimani , Wafa Hamdi , Foster Helen
Published: October 2022

Background

The pediatric Gait, Arms, Legs, and Spine (pGALS) is a practical quick musculoskeletal assessment to increase awareness of the joint disease, facilitate early recognition of joint problems, and prompt referral to specialist teams to optimize clinical outcomes. pGALS has been shown to be practical and useful, with excellent acceptability by children and their parents. Its use was limited in French-speaking countries because of the lack of the francophone version.

Objectives

To describe the steps of the translation of the pGALS francophone version using the Delphi approach.

Methods

Delphi method is the consensus-building method, providing the consensual opinion of the experts. For each translated item of the pGALS, the experts assessed the relevance using a scale ranging from 1 to 9 (not relevant-completely relevant). Then median was calculated giving for each item the position of the group: disagree (if the median < 3), equivocal (median between 4–6) and agreement (median >7). The degree of the convergence with the group was assessed to clarify this result: the group’s opinion is consensual if 70% of the responses were within the range of the median; otherwise, it’s “not consensual”. For the no consensual and no relevant item, the experts propose a comment to reformulate the sentence.

Results

Three native speakers were invited to translate the English form of the pGALS into the francophone language. The different propositions were mixed in a consensual way by a children’s musculoskeletal specialist. The version was validated according to the Delphi method. Six experts (pediatricians and rheumatologists) from different French-speaking countries were interviewed during 3 rounds by electronic survey individually and anonymously. After each round: the median, consensus, and comments of every item are collected and a meeting with experts was held to analyze the results. During the first meeting, we were consensual and we had an agreement on 82% of the items (28 items were validated, and 6 items were reformulated). Then the form was reformulated using the results of the preliminary rounds: opinions of the experts and their proposals during the last meeting). We were in agreement and we validated the remaining six-item during the second meeting. In the last round, we obtain a consensual version of pGALS.

Conclusion

Our approach contributed to the consensual translation of the francophone version of pGALS. This tool is now ready to be used as a basic clinical skill. More research is mandatory to assess its sensibility and specificity in screening musculoskeletal disorders.
18 Does overweight enhance foot disorders in patients with juvenile idiopathic arthritis?
Authors: Dghaies H. Ferjani , Moalla M , Ben Nessib D , Triki W , Maatallah K , Kaffel Dh , Hamdi W
Published: October 2022

Background

Juvenile idiopathic arthritis (JIA) is chronic inflammatory arthritis of childhood that may result in persistent and disabling foot impairments. Foot disorders are common in JIA with a prevalence estimated at over 90%. Many factors can worsen foot disorders in these patients. The influence of weight on foot disorders is poorly studied.

Objectives

To examine associations between weight and foot disorders in patients with JIA.

Methods

Patients with a diagnosis of JIA, based on the International League of Associations for Rheumatology (ILAR) criteria were included. Age, gender, weight, characteristics of the disease and of foot pain were noted. A specialized podiatric examination was performed.

Results

Thirty-two patients were included. The mean age was 12.2 ± 2.9 [5–18]. Forty-three percent of the patients were boys (n = 14). The mean age of disease onset was 8.5 ± 3.9 [3–15]. Only one patient had a triggering factor (elbow fracture). The most common type of JIA was oligoarthritis in 12 cases, then enthesitis-related in 8 cases, polyarthritis without rheumatoid factor in 3 cases, polyarthritis with positive rheumatoid factor in 1 case, psoriatic arthritis in 3 cases, systemic arthritis in 1 case and undifferentiated arthritis in 4 cases.

The mean weight was 43.5 kg [17–98]. Only 28% (n = 9) of the children had a normal weight for their age, 41% (n = 13) of them had a low weight for their age and 31% (n = 10) of them had overweight. Ten patients had foot pain: hindfoot pain in 5 patients, midfoot pain in 3 patients, and forefoot pain in 2 patients. Foot deformities were found in 78.1% of the patients (n = 27): flat foot in 39% (n = 13) of the patients and pes cavus in 39% (n = 13).

There was no association between overweight and foot pain (p = 0,14) or hindfoot pain (p = 0.08). However, overweight was associated with a forefoot (p = 0.01) and midfoot (p = 0.01) pain. Overweight was not associated with foot deformities (p = 0.1).

Conclusion

This study showed that weight is associated with foot pain especially forefoot and midfoot pain. A healthy lifestyle and a normal weight are fundamental to preventing foot pain at an early age in patients with JIA and improving their quality of life.
22 Monitoring of juvenile idiopathic arthritis: don’t miss the foot deformities!
Authors: Kharrat L , Ferjani H , Moalla M , Triki W , Ben Nessib D , Maatallah K , Kaffel D , Hamdi W
Published: October 2022

Background

Foot deformities seem to be frequent in children with Juvenile Idiopathic Arthritis (JIA) [1,2]. These deformities can deeply affect the child’s activity and alter his quality of life.

Objectives

To study the association between disease activity and foot deformities in JIA patients.

Methods

We conducted a cross-sectional study including patients meeting the International League of Associations for Rheumatology (ILAR) 2001 criteria for JIA. For each patient, we collected the following data: age, disease duration, tender joint count (TJC), swollen joint count (SJC), Patient Global Assessment (PGA), Visual Analogic Scale (VAS), and therapeutic management. Disease activity was assessed using the Juvenile Arthritis Disease Activity score (JADAS). Foot deformities were assessed using an optical podoscope. C-reactive protein (CRP) and Erythrocyte sedimentation rate (ERS) levels were measured. Statistical analysis was performed using SPSS software.

Results

We included 35 patients. Forty-three percent of the patients were boys (n = 15). The mean age was 12.2 ± 3.61 years. The mean disease duration was 4.1 ± 3.29 years. The mean PGA and the mean VAS were 3.4 ± 3.02 and 3.37 ± 2.92, respectively. The mean TJC and the mean SJC were 1.48 ± 1.69 and 0.61 ± 0.77, respectively. The mean CRP and ESR were 7.51 ± 11.85 mg/l and 18.88 ± 15.53 mm, respectively. Twenty-four patients were under non-steroidal anti-inflammatory drugs (69%), 12 patients were under methotrexate (34%), and 5 patients were under TNFα inhibitor (14%).

The mean JADAS was 7.58 ± 6.3. Seventeen percent of the patients had the inactive disease (n = 6). Foot deformities were found in 80% of the patients (n = 28). They were flatfoot in 40% (n = 14) and pes cavus in 46% (n = 16). These deformities were bilateral in 18 cases (51%). Hallux valgus was present in 14% of the cases (n = 5). Foot deformities were associated to a higher PGA (4.04 ± 3.01 vs 0.86 ± 1.2, p< 10–3), VAS (3.93 ± 2.94 vs 1.14 ± 1.46, p = 0.022), CRP level (8.84 ± 13.1 vs 2.79 ± 2.5 mg/l, p = 0.039), and higher JADAS (9.12 ± 6.25 vs 2.08 ± 1.93, p< 10–3).

Conclusion

Our study showed that foot deformities are common in JIA. Interestingly, these deformities are associated with a higher CRP level and a higher disease activity. These results suggest that an early screening of foot deformities is advisable in patients with active disease.

References

1. Truckenbrodt H, Häfner R, von Altenbockum C. Functional joint analysis of the foot in juvenile chronic arthritis. Clin Exp Rheumatol. 1994; 12 Suppl 10: S91-96.

2. Gschwend N, Ivosevic-Radovanovic D. [The child’s foot in juvenile polyarthritis (cP)]. Orthopade. 1986; 15(3):212–9.
30 Oligoarticular juvenile idiopathic arthritis-associated uveitis
Authors: Guedri Rahma
Published: October 2022

Background

Oligoarticular juvenile idiopathic arthritis (JIA) is a rare inflammatory disease that occur in children under the age of 16. JIA associated uveitis is the most frequent extra-articular manifestation. The uveitis can be sight-threatening and may be burdened with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stages.

Aim

The purpose of our survey is to report the incidence of uveitis in oligoarticular JIA and to determine the potential risk factors for the occurrence of uveitis in children with oligoarticular JIA.

Results

We retrospectively evaluated 70 consecutive pediatric patients with oligoarticular JIA in the emergency and outpatient paediatric departement at the children's hospital in Tunisia between January 2005 and December 2021. A total of 12 cases (17.1%) with oligoarticular JIA associated uveitis where identified. The incidence of uveitis in these children was 0.7 cases per year.

The uveitis had occurred before the joint manifestations in only one patient. The average time between the onset of symptoms and the occurrence of uveitis was 1.7 years with a maximum time interval of 5 years. Management of JIA-associated uveitis involved use of topical agents in half of our patients and systemic agents in the other half.

JIA-associated uveitis has led to ocular complications such as cataracts (n = 5), glaucoma (n = 6), anterior/posterior synechiae (n = 8), and ultimately a visual impairment and blindness (n = 1). Eleven of the twelve patients with uveitis retained acceptable visual acuity.

The presence of antinuclear antibody (ANA) has been identified as a risk factor for the occurrence of uveitis (p = 0.03).

Conclusion

Collateral damage of oligoarticular JIA include growth failure, muscle atrophy and intraocular damage. The main challenge during the management of oligoarticular JIA is the early detection of uveitis. Close cooperation between the rheumatologist and the ophthalmologist is essential to optimize outcome.
32 Methotrexate in oligoarticular juvenile idiopathic arthritis: adverse effects and outcome
Authors: Guedri R , Dghaies C , Hrizi H , Fraj H , Rebhi M , Essaddam L , Dahmouni M , Abdelaali A , Fitouri Z , Ben Becher S
Published: October 2022

Background

Methotrexate (MTX) is the anti-rheumatic drug of choice in juvenile idiopathic arthritis (JIA). Its adverse effects such as intolerance occur frequently, potentially hindering its efficacy

Objective

To describe the frequency of MTX intolerance and its various adverse effects (AE).

Results

A retrospective study was conducted on the AEs associated with MTX therapy in children diagnosed with oligoarticular juvenile idiopathic arthritis followed-up at the paediatric emergency and outpatient department of the children's hospital in Tunisia between January 2005 and December 2021. Among 95 cases, only 70 patients were included into the study, of whom 55 (78.5%) were girls. The median age at diagnosis was 5.9 years. Of these, 39/70 (55.7%) had required the use of MTX. The use of MTX was early (within the first 6 months after diagnosis) in 61.5% of cases. The initial median weekly dose was 11.4 mg/m². MTX intolerance was noted in 35.8% of cases. Management of intolerance included change in the dose, education and counselling. Adverse events led to MTX withdrawal in only one patient (2.5%).

The adverse effects noted during the follow-up were mainly transient increased plasma transaminases (17.9%), gastrointestinal symptoms (10.2%) and cytopenia (7.6%). Impairment of kidney function and behavioural problems were not noted in any of our patients.

The medium duration of the treatment was 3.8 years. The evolution was marked by the occurrence of a relapse of the disease after discontinuation of MTX in 14/33 cases (42.4%). The addition of biotherapy was necessary in 10/39 cases (25.6%). Only 7% of children have persistent joint stiffness or deformity.

Conclusion

In JIA, it is important to start effective treatment early to avoid long-term sequelae, such as joint damage. MTX adverse effects such as MTX intolerance occur frequently, potentially hindering its efficacy. To avoid inefficacy, the physician should be timely aware of these adverse events.
33 Oligoarticular juvenile idiopathic arthritis-associated uveitis
Authors: Dghaies C , Hrizi H , Guedri R , Rebhi M , Fraj H , Essaddam L , Dahmouni M , Abdelaali A , Fitouri Z , Ben Becher S
Published: October 2022

Background

Oligoarticular juvenile idiopathic arthritis (JIA) is a rare inflammatory disease that occur in children under the age of 16. JIA associated uveitis is the most frequent extra-articular manifestation. The uveitis can be sight-threatening and may be associated with disabling morbidity. The uveitis seen in JIA is chronic anterior uveitis which is always asymptomatic in the initial stage.

Objective

To report the incidence of uveitis in oligoarticular JIA and to determine the potential risk factors for the occurrence of uveitis in children with oligoarticular JIA.

Results

We retrospectively evaluated 95 consecutive pediatric patients with oligoarticular JIA in the paediatric emergency and outpatient department of the children's hospital in Tunisia between January 2005 and December 2021. 70 patients were included in this study. A total of 12 cases (17.1%) with oligoarticular JIA associated uveitis where identified. The incidence of uveitis in these children was 0.7 cases per year.

The uveitis had occurred before the joint manifestations in only one patient. The average time between the onset of symptoms and the occurrence of uveitis was 1.7 years with a maximum time interval of 5 years. Management of JIA-associated uveitis involved use of topical agents in half of our patients and systemic agents in the other half.

JIA-associated uveitis has led to ocular complications such as cataracts (n = 5), glaucoma (n = 6), anterior/posterior synechiae (n = 8), and ultimately a visual impairment and blindness (n = 1). Eleven of the twelve patients with uveitis had acceptable visual acuity. The presence of antinuclear antibody (ANA) was identified as a risk factor for the occurrence of uveitis (p = 0.03).

Conclusion

Collateral damage of oligoarticular JIA include growth failure, muscle atrophy and intraocular damage. The main challenge during the management of oligoarticular JIA is the early detection of uveitis. Multidisciplinary management by the rheumatologist and the ophthalmologist is essential to optimize outcome.
36 Juvenile idiopathic arthritis-associated uveitis: a study of 69 cases
Authors: Hadef Djohra , Slimani Samy , Khamari Mohamed Choukri , Mekaoussi Walid , Belot Alexandre , Quartier Pierre
Published: October 2022

Background

Juvenile Idiopathic Arthritis (JIA) is the most common rheumatic disease association with uveitis in children. Uveitis is a significant cause of visual morbidity in children with JIA. Geographical variations in the incidence of uveitis in JIA have been reported around the world.

Objectives

The aim of this study was to determine the prevalence of JIA associated uveitis and its features in Batna -Algeria- and to compare the findings with other JIA populations worldwide.

Methods

A multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA medications, JIA status at the time of enrolment and the latest follow-up.

Results

Sixty-nine cases of JIA were included. The female to male ratio was 1.8 :1. The median age at diagnosis was 9 years (range 1–16). At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34(49.3%) oligoarthritis, 9(13%) rheumatoid factor (RF) negative polyarticular arthritis, 8(11.6%) rheumatoid factor (RF) positive polyarticular arthritis, 6(8.7%) systemic onset JIA, 6(8.7%) enthesitis-related arthritis, 3 (4.3%) psoriatic arthritis, and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status. Sixty-three patients (91.3%) have benefited from the slit lamp examination. Uveitis was found in 5 patients (7.2%). three of these patients presented with oligoarticular JIA and two presented Polyarticular JIA. There was male predominance (4 boys and 1 girl). All these patients were ANA negative.

Conclusion

The observed phenotypic variabilities in JIA associated uveitis underscore the existence of true diversities in disease characteristics across geographical areas. Prospective multicentre studies are necessary to better identify the JIA associated uveitis peculiarities in our country.

Disclosure of Interest

None declared
52 Use of corticosteroids in childhood systemic lupus erythematosus: experience from a pediatric rheumatology center
Authors: Gacem Ourida , Abba Kahina , Mansouri Nawal , Ayad Nadia , Zeroual Zoulikha , Arrada Zakia , Achir Moussa , Ladj Mohamed Samir
Published: October 2022

Background

Childhood-onset Systemic Lupus Erythematosus (cSLE) is a rare but severe multisystem autoimmune/inflammatory disease that can affect any organ system and cause significant damage, disability and/or death. Corticosteroids are one of the important therapeutic weapons for childhood Systemic Lupus Erythematosus (SLE). Prednisone doses are increased during flare-ups of lupus disease, and then progressively reduced to reach a threshold dose in order to avoid the harmful effects of this treatment in growing children. The oral route is by far the most used over prolonged periods, however corticosteroid boluses are recommended in the treatment of severe acute attacks of the disease.

Objective

Assess the use of corticosteroids in childhood systemic lupus erythematosus (SLE) and the risk of steroid-related damage.

Methods

Patients with childhood -onset SLE (n = 83) treated at paediatric rheumatology centre were followed for 36 months. Information on medication use, disease activity as measured by SLEDAI was collected.

Results

Eighty-three (83) patients were studied. Female: male ratio was 1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 (±3,88_ and 11, 3 (±3,62) years. Corticosteroids were used in 92% of cases in oral form and on 37% of cases as a bolus of high dose intravenous methylprednisolone. The indication of corticoids bolus was significantly related to severe forms (p = 0,001), high disease activity (p = 0, 00008), and macrophage activation Syndrome (p = 0, 0006). The doses varied between 0.5–1 g/1.73m2. The mean initial disease activity in these patients was 30 ± 10, while that found after taking the treatment was 12.9 ± 15. The decrease was statistically significant (p = 0, 000001). More than half of our patients were on high dose oral corticosteroids (2 mg/kg/d) and only 1/5 of the patients benefited from a dose of (1 mg/kg/day). The use of corticosteroids has been associated with significant side effects, including growth delay (22%), cushingoid obesity (58%), stretch marks (49%), osteoporosis (33%), femoral head osteonecrosis (1%), infections (54%). The association of certain side effects with a high dose of oral corticosteroids (2 mg/kg/day) was significant in this case cushingoid obesity (p = 0, 0003), stretch marks (p = 0.014).

Conclusion

Corticosteroids are the mainstay of therapy for prompt control of acute disease related inflammation in patients with SLE; they remain the first-line treatment for significant lupus manifestations, especially severe forms and life-threatening situations Higher dosage and longer duration of therapy, which are common in SLE, result in greater risk of adverse effects. Their use wisely is the guarantee of a better response and the reduction of deleterious effect.
53 Nakajo-Nishimura syndrome: the first African case
Authors: Ghodbane Nacif Eddine , Mecibah Ali , Haddouche Assia , Zerguine Halima , Akakba Zineb , Slimani Samy
Published: October 2022

Nakajo-Nishimura syndrome is a hereditary autoinflammatory disorder caused by an autosomal recessive homozygous mutation of the PSMB8 gene, which encodes the immunoproteasome subunit beta 5i. The clinical manifestations of NNS are mainly pernio-like skin rashes, nodular erythema, lipodystrophy, clubbed fingers, remittent fever, hepatosplenomegaly, and basal ganglia calcifications. Here we are reporting a case of NNS in an 11-year-old girl, who lives in eastern Algeria, born from a first-degree consanguineous marriage, she presented with erythematous patches on her face and her back, nodular erythema on her neck, swollen and painful fingers with acrocyanosis and recurrent fever that mainly occurred in cold weather. The patient received long-term treatment with low-dose glucocorticoids, along with immunomodulatory drugs (hydroxychloroquine with methotrexate), partial improvement clinically and biologically was observed. Colchicine was added to her treatment, with increased prednisone doses when she recently developed an AA amyloidosis. Our patient was diagnosed clinically with NNS because she exhibited six of the eight characteristics. To the best of our knowledge, this is the first case of NNS in Africa.
70 Comparative study between PIMS and Kawasaki disease
Authors: Jalal M , Sakhi A , Ansari G. Benbrahim , Aboufaris H , Bouayed K , Ameayou S , Hassoun S , Nani S
Published: October 2022

Background

Kawasaki disease (kDa) is a multisystemic vasculitis affecting medium and small vessels with a predilection for the coronary arteries. It appears to be very similar to the pediatric multisystemic inflammatory syndrome (PIMS), which is a post-infectious inflammatory condition occurring after SARS-COV2 infection. Although these two entities have clinical and biological similarities, marked differences are identified. The aim of this study is to compare the two conditions in order to highlight the specific criteria of each of these related entities and to describe their demographic, clinical, biological and therapeutic characteristics in our context.

Method

Single center prospective observational study from January 2021 to March 2022 was conducted, comparing children admitted for PIMS on the basis of persistent fever, inflammatory syndrome, and positive COVID-19 IgG serology to patients hospitalized for kDa according to American Heart Association criteria.

Results

42 cases were recruited, among them 24 PIMS and 18 kDa, with a male predominance. The average age was 5 years for PIMS and 2 years and 7 months for kDa. All had a persistant fever with an average duration of 8 days as reason for consultation. Conjunctivitis was found in 88% of kDa vs 75% of PIMS and cheilitis in 94% of kDa vs 75% of PIMS. Skin rash, extremity involvement, cervical adenopathy were reported in both pathologies with respective percentages of 62.5%, 17.4% and 25% for PIMS vs 61.1%, 33.3% and 33.3% for kDa. Abdominal pain was reported in 54.2% of PIMS patients vs 5.6% of kDa. All patients had an inflammatory syndrome. The mean sedimentation rate was 67 mm at the first h in PIMS and 99 mm at the first h in kDa. The mean CRP was 147 mg/l in PIMS vs 132 mg/l in kDa. Lymphopenia was predominant in PIMS with 33% vs 11% in kDa. Cardiac enzymes were higher in PIMS with 29% of myocarditis and 15% of coronary dilatation vs 16.7% of coronary involvement in kDa. All PIMS patients received immunoglobulin infusion IV-IG, corticosteroid therapy, and an antiplatelet agent, whereas patients with kDa received IV-IG and acetyl salicylic acid in anti-inflammatory doses. Apyrexia was obtained at day 1 of treatment in the majority of patients, whereas 5.6% of kDa had persistent aneurysm.

Conclusion

PIMS cases predominated over kDa cases during this pandemic period. The distinction between the two entities could be difficult given the clinico-biological similarities. Abdominal pain was significantly more frequent in PIMS patients, whereas lymphopenia and myocarditis were not. The prognosis was better in PIMS.
71 Estimation of incidence of MIS-C in Cape Town, South Africa
Authors: Butters Claire , Abrahams Deepthi , Stander Raphaella , Spraklen Timothy , Facey-Thomas Heidi , Zühlke Liesl , Rabie Helena , Scott Christiaan , Webb Kate
Published: October 2022

Background

Multisystem inflammatory syndrome is a severe manifestation of SARS-CoV-2 in children. The incidence of MIS-C after SARS-CoV-2 infection is poorly understood. There are very few cohorts describing MIS-C in Africa despite MIS-C being more common in Black children worldwide.

Methods

A cohort of children with MIS-C and healthy children was recruited from May 2020 to May 2021 from the two main paediatric hospitals in Cape Town, South Africa. Clinical and demographic data were collected, and serum was tested for SARS-CoV-2 antibodies. The incidence of MIS-C was calculated using an estimation of population exposure from seroprevalence in the healthy group. Summary data, non-parametric comparisons and logistic regression analyses were performed.

Results

Sixty-eight children with MIS-C were recruited with a median age of 7 years and 97 healthy children were recruited with a 30% seroprevalence. The estimated incidence of MIS-C was 22/100 000 SARS-COV-2 infections in children under 14 years old in the city at that time. Black children were over-represented in the MIS-C group (62% vs 37%, p = 0.002). The most common clinical features in MIS-C were fever (100%), tachycardia (98.5%), rash (85.3%), conjunctivitis (77.9%), abdominal pain (60.3%) and hypotension (60.3%). Median levels of haemoglobin, sodium, CRP, ferritin, cardiac (pro-BNP, trop-T) and coagulation markers (D-dimer and fibrinogen), neutrophil and white cell count were markedly deranged in MIS-C. Cardiac, pulmonary, central nervous and renal organ systems were involved in 71%, 29.4%, 27.9% and 27.9% respectively. Ninety-four point one per cent patient received intravenous immune globulin, 64.7% received methylprednisolone and 61.7% received both. ICU admission was required in 39.7% patient while 38.2% required inotropic support, 38.2% required oxygen therapy, 11.8% required invasive ventilation and 6% required peritoneal dialysis. The median hospital stay duration was 7 days with no deaths.

Conclusion

The lack of reports from Southern Africa does not reflect a lack of cases of MIS-C. The clinical manifestations and outcomes of MIS-C in this region highlight the need for improved surveillance, reporting and data to inform diagnosis and treatment.

Implications

To our knowledge, these are the first data on MIS-C in Africa. This shows that children in Africa are indeed presenting with MIS-C which will increase surveillance around the continent.
80 Case series of juvenile scleroderma at Aga Khan University Hospital, Nairobi
Authors: Nzisa Irene , Mercy Chemutai , Migowa Angela
Published: October 2022

Background

Juvenile scleroderma includes a range of conditions presenting with skin fibrosis. It’s classified into two major categories, localized and systemic. Juvenile localized scleroderma (morphea) is limited to the skin and forms majority of pediatric presentations (>95%). Though rare, juvenile systemic sclerosis involves the skin and other internal organs and is associated with a poorer prognosis.

Objective

To characterize the clinical profile of two cases of scleroderma at Aga Khan university hospital, Nairobi.

Methods

Retrospective review of medical records of two patients with Juvenile scleroderma managed at Aga Khan university hospital, Nairobi.

Results

The first patient was a 7-year-old boy presenting with joint pains for 2 months, restricted activities and limping. He also had a non-itchy lesion on the right thigh for about 6 years. On examination, there was an 8 by 18 cm, flat, shiny skin lesion with ill-defined margins and skin thickening around it. There were 2 punched out scars from previous skin biopsy sites with latest biopsy showing atrophic epidermis, dermal collagen homogenization with thick sclerosed fibers in keeping with morphea. Pulmonary function tests revealed mild restrictive lung disease. There were no other systems involved on further evaluation. He was started on weekly methotrexate, daily prednisone, daily folic acid and omeprazole to be followed up in clinic.

The second case is a 5-year-old girl who presented with 3-year history of inability to walk. Physical examination revealed areas of skin hypopigmentation and hyperpigmentation on the neck and back with active lesions on the left flank and posterior right leg. She had left lower limb atrophy and extreme wasting of thigh and calf muscles, a contracture of the left knee, ankle and limb length discrepancy. Her investigations revealed positive rheumatoid factor, positive antinuclear antibody (ANA), microcytic hypochromic anaemia and skin biopsy with features of morphea. She received six courses of high dose methyl prednisone over six months, weekly methotrexate, daily folic acid, daily prednisone that was tapered off gradually, calcium supplementation and iron supplementation. In addition, she received isoniazid for tuberculosis prophylaxis with pyridoxine. Her management was multidisciplinary involving orthopedics, physiotherapy, occupational therapy, and dieticians. She developed a new skin lesion on the left forearm about five months after initiation of treatment but no worsening of symptoms or other new lesions thereafter.

Conclusion

Juvenile scleroderma though rare has a high risk of misdiagnosis with delay in initiation of treatment. It is associated with significant morbidity and early diagnosis, treatment and follow up is imperative for good outcomes.
90 Nakajo-Nishimura syndrome: a case report
Authors: Halima Zerguine , Hadef Djohra , Soltani Hanane , Zerrouki Younes , Ouazani Sana , Belkacemi Hafidha , Challa Safia , Slimani Samy
Published: October 2022

Introduction

Nakajo-Nishimura is a rare autosomal recessive inherited autoinflammatory condition that has been described mainly in the Japanese population, but very similar syndromes, such as Candle syndrome, have been reported in Western populations. There is no gold standard treatment for this condition. This is a case report of a 12-year-old female who most likely has Nakajo-Nishimura syndrome.

Methods and results

A 12-year-old female (born in 2010), from Batna (Algeria), was born of a first-degree consanguineous marriage, and the second of two children. She had no relevant past medical history. She was referred on account of dermatology symptoms, occurring very early in life, with papules and distal purplish erythema located in fingers and toes associated with an acrocyanosis. Subsequently, diffuse arthralgias appeared, exacerbated by cold, with a delay in walking, around the age of 3 years.

Clinical examination revealed a thread-like appearance of the fingers with shortening of the 5th digit, and small erythematous lesions, without sclerosis or Raynaud's disease. There was no sign of synovitis or joint deformities. Hepatosplenomegaly with dental dysgenesis and delayed puberty were noted. The laboratory tests showed an inflammatory syndrome—ESR (Erythrocyte Sedimentation Rate) of 100 mm the first h, positive CRP (C-reactive protein), hyper alpha and gamma globulinaemia, and inflammatory anaemia with thrombocytopaenia. The genetic work-up is currently in progress.

The treatment was Hydroxychloroquine and steroids. After the onset of a nephrotic syndrome, Mycophenolate mofetil (MMF) was added. The renal biopsy found a renal amyloidosis. The patient was in chronic renal failure stage 4 requiring hemodialysis.

Conclusion

Extremely rare syndrome described in 30 patients in the literature and whose treatment remains symptomatic and does not prevent complications.

Disclosure of Interest

None declared
Kurstakin Triggers Multicellular Behaviors in Bacillus cereus AR156 and Enhances Disease Control Efficacy Against Rice Sheath Blight
Authors: Yu Yi-Yang , Zhang Yi-Yuan , Wang Ting , Huang Tao-Xiang , Tang Shu-Ya , Jin Yu , Mi Dan-Dan , Zheng Ying , Niu Dong-Dong , Guo Jian-Hua , Jiang Chun-Hao
Published: October 2022

Kurstakin is the latest discovered family of lipopeptides secreted by Bacillus spp. In this study, the effects of kurstakin on the direct antagonism, multicellularity, and disease control ability of Bacillus cereus AR156 were explored. An insertion mutation in the nonribosomal peptide synthase responsible for kurstakin synthesis led to a significant reduction of antagonistic ability of AR156 against the plant-pathogenic fungi Rhizoctonia solani, Ascochyta citrullina, Fusarium graminearum, and F. oxysporum f. sp. cubense. The loss of kurstakin synthesis ability significantly impaired the swarming motility of AR156 and reduced biofilm formation and amyloid protein accumulation. Although the loss of kurstakin synthesis ability did not reduce the competitiveness of AR156 under laboratory conditions, the colonization and environmental adaptability of the mutant was significantly weaker than that of wild-type AR156 on rice leaves. The cell surface of wild-type AR156 colonizing the leaf surface was covered by a thick biofilm matrix under a scanning electron microscope, but not the mutant. The colonization ability on rice roots and control efficacy against rice sheath blight disease of the mutant were also impaired. Thus, kurstakin participates in the control of plant diseases by B. cereus AR156 through directly inhibiting the growth of pathogenic fungi and improving long-term environmental adaptability and colonization of AR156 on the host surface by triggering multicellularity. This study explored the multiple functions of kurstakin in plant disease control by B. cereus.
Within-Season Shift in Fungicide Sensitivity Profiles of Pseudoperonospora cubensis Populations in Response to Chemical Control
Authors: Kikway Isaack , Keinath Anthony P. , Ojiambo Peter S.
Published: October 2022

Cucurbit downy mildew, caused by Pseudoperonospora cubensis, is an important disease affecting cucurbits worldwide. Chemical control is an effective method for disease control but P. cubensis has a high risk for developing resistance to fungicides. Alternating fungicides with different modes of action is recommended to avoid an increase of resistant subpopulations. Thus, this study was conducted to establish shifts in the sensitivity profiles of P. cubensis isolates during the growing season, wherein chlorothalonil was applied in alternation with either cymoxanil, fluopicolide, or propamocarb in field experiments conducted from 2018 to 2020 at Rocky Mount, NC and in 2018 and 2020 at Charleston, SC. The sensitivity of baseline isolates sampled early in the season or exposed isolates sampled late in the season to these single-site fungicides was determined using a detached-leaf assay, where tested isolates were classified as sensitive or resistant based on the relative disease severity. Based on the Kruskal-Wallis test, the distribution profile of relative disease severity among baseline and exposed isolates was significantly different where chlorothalonil was alternated with fluopicolide (χ2 = 10.82; P = 0.001) but not with cymoxanil (χ2 = 1.39; P = 0.238) or propamocarb (χ2 = 2.37; P = 0.412). Although there was a directional selection toward resistance for isolates sampled from plots that were treated with fluopicolide or propamocarb alternated with chlorothalonil during a growing season, a significant shift in fungicide sensitivity distribution based on combined data were observed for fluopicolide (χ2 = 8.25; P = 0.004) but not propamocarb (χ2 = 1.05; P = 0.461). Baseline and exposed isolates sampled from the cymoxanil-treated plots were all resistant to this fungicide and there was no significant shift in their fungicide sensitivity profile during a growing season (χ2 = 0.06; P = 1.000). These results indicate that a shift toward reduced sensitivity in P. cubensis can occur during a growing season and the efficacy of fluopicolide is likely to decrease as the frequency of the less sensitive subpopulations increases during a production season. The resultant effect on disease severity and selection of an insensitive subpopulation may accelerate the development of resistance to propamocarb in the southeastern United States.
Diversity in MD–PhD Programs and Factors Affecting Admission and Completion Among Minoritized Groups: A Scoping Review
Authors: Cavanagh Alice , Owais Sawayra , Syed Saad A. , Zhang Ali , Van Lieshout Ryan J.
Published: October 2022

Purpose

To improve awareness of diversity in MD–PhD program applicants, matriculants, and graduates; facilitators and barriers to matriculation and/or completion among minoritized groups; and the effects of research experience programs on admissions processes aimed to increase representation of minoritized groups in MD–PhD programs.

Method

The authors conducted a scoping review, searching EMBASE, MEDLINE, PsycINFO, CINAHL, and Web of Science through December 21, 2021, for studies that contained data on the characteristics of MD–PhD learners and initiatives aimed to make the clinician–scientist trainee population more diverse. They excluded studies that had no primary data, were unavailable in English, and were not peer-reviewed.

Results

Of 4,369 articles identified, 16 met inclusion criteria. Studies conceptualized diversity inconsistently, including as sex/gender disparities (n = 11), race/ethnicity underrepresentation (n = 9), disability (n = 2), first-generation student (n = 1), visible minority (n = 1), Indigenous population (n = 1), and economic/social disadvantage (n = 1). Potential barriers to entering or continuing in an MD–PhD program among women and underrepresented ethnic minorities included the long program duration and lack of mentorship; potential facilitators included the flexibility of the dual-degree program. Limited data on high school, undergraduate, and postbaccalaureate research experience programs targeting underrepresented minorities suggest that they may help facilitate admission into MD–PhD programs.

Conclusions

The findings of this scoping review suggest that the diversity of MD–PhD students has been conceptualized in unitary, inconsistent terms, without addressing how different dimensions of diversity may intersect and impact MD–PhD admissions. Future studies should be explicit and intentional in defining “diversity” as it relates to their research questions, explore the impact of intersectionality, and systematically identify and address causal facilitators and barriers of entry to and completion of MD–PhD programs among minoritized groups.
Thoracic radiotherapy may improve the outcome of extensive stage small cell lung carcinoma patients treated with first-line immunotherapy plus chemotherapy
Authors: Wu Jia-Jun , Huang Jing-Wen , Hsu Kuo-Hsuan , Huang Yen-Hsiang , Chen Kun-Chieh , Tseng Jeng-Sen , Yang Tsung-Ying , Chang Gee-Chen
Published: October 2022

Objective

Immunotherapy plus etoposide and platinum (EP)-based chemotherapy is the standard of care for patients with extensive stage–small cell lung carcinoma (ES-SCLC). In the era of immunotherapy, the role of thoracic radiotherapy for ES-SCLC remains unclear.

Methods

We retrospectively included ES-SCLC patients treated with first-line EP-based chemotherapy plus atezolizumab or durvalumab at Taichung Veterans General Hospital to evaluate the prognostic role and safety of thoracic radiotherapy.

Results

A total of 22 patients were included. The median age was 64 years and most of them were male and smokers. Sixteen patients (72.7%) received durvalumab, while the other 6 patients (27.3%) underwent atezolizumab treatment. Among these patients, 11 (50.0%) had a history of thoracic radiotherapy. There was no significant difference in baseline characteristics between patients with and without thoracic radiotherapy. In the overall population, the objective response rate to immunotherapy plus chemotherapy was 73.7%. The progression-free survival and overall survival were 6.0 months (95% CI: 4.0–7.9) and 13.8 months (95% CI: 8.0–19.6), respectively. The overall survival was significantly longer in patients with thoracic radiotherapy (not-reached [NR] [95% CI NR-NR] vs. 9.6 months [95% CI 2.5–16.6]), respectively (P value by log-rank test <0.001). Both multivariate analysis and subgroup analysis specifically comparing patients with consolidative thoracic radiotherapy and patients with clinical benefits to systemic therapy who did not undergo thoracic radiotherapy indicated that thoracic radiotherapy improved survival.

Conclusion

The real-world efficacy of EP-based chemotherapy plus atezolizumab or durvalumab was comparable with that of clinical trials. Thoracic radiotherapy may improve the outcome of ES-SCLC.