Amniotic band syndrome (ABS) was first described by Montgomery in Montgomery (1832). It is a poorly known congenital malformation due to strangulation of the organs by an amniotic fibrous band. Several parts of the body can be affected: for instance, skull, face, neck, trunk and musculoskeletal system. It generally associates three types of anomalies namely, amputations, deformities, and malformations. There are two genuine theories covering this syndrome; the Intrinsic Theory associating the syndrome to a germline defect and the Purely Mechanical Extrinsic Theory related to the amniotic band. These theories have thoroughly tried to explain the disease and the organ involvement (Goldfarb et al., 2009). In the current study, we report a rare case of an open fracture of both leg bones with amniotic disease in a 10-day-old neonate who underwent surgical treatment. In our case, it is a surgical emergency where we try to explain its physiopathology and show how to operate it. We discuss likewise the appropriateness of using the expressions “leg fracture” and “congenital pseudarthrosis of the leg”. Finally, we describe a revised classification by Hall (1982) and Weinzweig (1994) of ABS incorporating a stage with bone involvement.

Background

Pediatric Rheumatology (PR) is a young specialty that has not yet gained momentum in Algeria. The aim of this study is the assessment of the perception of PR by pediatric residents in Algeria and the current practice of this young sub-specialty in our country.

Methods

Descriptive cross-sectional study evaluating the perception of PR by pediatric residents was created on Google forms and distributed via email and Facebook to be taken as a self-administered online survey.

The survey is composed of three parts: socio-demographic characteristics of the participant, current practice of PR and perspectives of PR training.

Results

Seventy-seven residents responded to the online questionnaire, with an average age of 30.40 ± 3.13 years and 65% of participants being female. Participation included different regions of Algeria (Batna, Annaba, Setif, Constantine, Algiers and Oran) and different levels of training (from the first year to the fifth year of residency)

Ninety-two percent (92%) of participants had managed PR consultations, with a frequency of once a week in 75.3% of cases and twice a week in 15.6% of cases. In 81% of cases, it was not the initial presentation. The patients were mainly referred by a general practitioner or a general pediatrician. The Management of these patients was provided by a general pediatrician in 69.4% of cases and only in 37.7% by a specialized pediatrician. In 71.4% of cases, there was no pediatric rheumatologist in the establishment where the resident was being trained.

The level of knowledge in PR was admitted to be poor by 59.7% of the respondents. Seventy-five percent of the residents wanted to have rotations dedicated to this sub-specialty during their training. Seventy percent thought that PR is equally as important to know as the other subspecialties in pediatrics (pediatric endocrinology, pediatric pneumology ...). Interest in further PR training was expressed by 80.5% of participants, given the chance.

Conclusion

This survey reinforces the perception that PR training needs to be improved in Algeria, especially as the number of PR consultations is on the rise.

The creation of specialized training in PR is becoming a necessity.

Background

Gourgerot-Sjögren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjögren Syndrome

Methods and results

A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal.

The patient was diagnosed with primary Gougerot-Sjögren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs.

Conclusion

Our case is particular in that primary Gougerot-Sjögren Syndrome is rare in children. Routine follow- up with this patient is important to determine whether it is indeed pGSS with pediatric onset or GSS secondary to another autoimmune disease.

Background

Pediatric Rheumatology is an orphan specialty in Africa which is gradually gaining importance across the continent.

Main body

This commentary discusses the current state of affairs in the sphere of Pediatric Rheumatology across Africa and offers practical strategies to navigate the challenges encountered in research, models of care, education and training. We outline the establishment, opportunities of growth and achievements of the Pediatric Society of the African League Against Rheumatism (PAFLAR).

Conclusion

This commentary lays the foundation for establishment of a formidable framework and development of partnerships for the prosperity of Pediatric Rheumatology in Africa and beyond.

Juvenile Idiopathic Arthritis (JIA) is a group of chronic heterogenous disorders that manifests as joint inflammation in patients aged <16 years. Globally, approximately 3 million children and young adults are suffering from JIA with prevalence rates consistently higher in girls. The region of Africa and Middle East constitute a diverse group of ethnicities, socioeconomic conditions, and climates which influence the prevalence of JIA. There are only a few studies published on epidemiology of JIA in the region. There is an evident paucity of adequate and latest data from the region. This review summarizes the available data on the prevalence of JIA and its subtypes in Africa and Middle East and discusses unmet needs for patients in this region. A total of 8 journal publications were identified concerning epidemiology and 42 articles describing JIA subtypes from Africa and Middle East were included. The prevalence of JIA in Africa and Middle East was observed to be towards the lower range of the global estimate. We observed that the most prevalent subtype in the region was oligoarticular arthritis. The incidence of uveitis and anti-nuclear antibody (ANA) positivity were found to be lower as compared to the incidence from other regions. There is a huge unmet medical need in the region for reliable epidemiological data, disease awareness, having regional and local treatment guidelines and timely diagnosis. Paucity of the pediatric rheumatologists and economic disparities also contribute to the challenges regarding the management of JIA.

Objectives

To identify the changes in rheumatology service delivery across the five regions of Africa from the impact of the COVID-19 pandemic.

Methods

The COVID-19 African Rheumatology Study Group created an online survey consisting of 40 questions relating to the current practices and experiences of rheumatologists across Africa. The CHERRIES checklist for reporting results of internet e-surveys was adhered to.

Results

A total of 554 completed responses were received from 20 countries, which include six in Northern Africa, six in West Africa, four in Southern Africa, three in East Africa and one in Central Africa. Consultant grade rheumatologists constituted 436 (78.7%) of respondents with a mean of 14.5 ± 10.3 years of experience. A total of 77 (13.9%) rheumatologists avoided starting a new biologic. Face-to-face clinics with the use of some personal protective equipment continued to be held in only 293 (52.9%) rheumatologists’ practices. Teleconsultation modalities found usage as follows: telephone in 335 (60.5%), WhatsApp in 241 (43.5%), emails in 90 (16.3%) and video calls in 53 (9.6%). Physical examinations were mostly reduced in 295 (53.3%) or done with personal protective equipment in 128 (23.1%) practices. Only 316 (57.0%) reported that the national rheumatology society in their country had produced any recommendation around COVID-19 while only 73 (13.2%) confirmed the availability of a national rheumatology COVID-19 registry in their country.

Conclusion

COVID-19 has shifted daily rheumatology practices across Africa to more virtual consultations and regional disparities are more apparent in the availability of local protocols and registries.

The new coronavirus infection COVID-19 has quickly become a global health emergency. Mortality is principally due to severe Acute Respiratory Distress Syndrome (ARDS) which relays only on supportive treatment. Numerous pathological, clinical and laboratory findings rise the similarity between moderate to severe COVID-19 and haemophagocytic lymphohistiocytosis (HLH). Etoposide-based protocol including dexametasone is the standard of care for secondary HLH. The protocol has been successfully used in HLHs that are secondary to EBV and H1N1 infections by inducing complete response and prolonged survival. These observations prompt to consider this cytotoxic therapy in HLH associated to moderately severe to severe forms of COVID-19.