An Atypical Cause of a Child Limp: A Gorham-Stout Disease with a Vanishing Hip.

Gorham-Stout disease (GSD) is a rare bone disease characterized by an abnormal proliferation of endothelial-lined vessels and destruction of the affected bone. As it affects commonly children and young adults, it is associated with significant morbidity and mortality. To date, there is no established treatment strategy for GSD. We report through this observation a rare case of GSD in a child located in the hip and the iliac crest.

Development of the paediatric society of the African league against rheumatism (PAFLAR) JIA registry and clinical profile of JIA in Africa from the PAFLAR JIA registry.

BACKGROUND: The spectrum of Juvenile Idiopathic Arthritis (JIA) in Africa is still largely unknown. We thus set out to illustrate how we set up the PAFLAR JIA registry and describe the clinical profile of Juvenile Idiopathic Arthritis across various regions in Africa. METHODS: We carried out a retrospective observational cohort study where collaborators were trained on use of the existing PAFLAR REDCAP database to enter data for the JIA patients currently under their care capturing their epidemiological data, clinical features, laboratory investigations, diagnosis and therapy at initial diagnosis. Descriptive statistics including means, standard deviations, medians, interquartile ranges (IQR) for continuous variables and proportions for categorical variables were calculated as appropriate. Tests for difference between groups were performed between categorical variables using Pearson's chi-square or Fisher's exact tests. All analyses were performed using SPSS version 22 software. RESULTS: We enrolled 302 patients, 58.6% (177 of 302) of whom were female. The median age of disease onset was 7 years (range 3-11 years) and the median age at diagnosis was 8.5 years (range 5-12 years). The median duration delay in diagnosis was 6 months (range 1-20.8 months). The JIA categories included Systemic JIA 18.9% (57), Oligoarticular JIA 19.2% (83), Polyarticular RF + ve 5% (15), Polyarticular RF-ve 17.9% (54), Enthesitis Related Arthritis (ERA) 18.2% (55), Psoriatic Arthritis 7% (21) and undifferentiated JIA 5.6% (17). As regards treatment the commonest therapies were NSAID therapy at 31.1%, synthetic DMARDs at 18.1%, synthetic DMARDs combined with NSAIDs at 17.5% and steroid therapy at 9.6%. Biological DMARDs accounted for 2.3% of therapies offered to our patients at diagnosis. The average JADAS score was 10.3 (range 4.8-18.2) and the average CHAQ score was 1.3 (range 0.7-2.0). CONCLUSION: Our study highlights strategies involved in setting up a Pan-African paediatric rheumatology registry that embraces our broad diversity and the vast spectrum of JIA in Africa while comparing the various therapies available to our patients. The PAFLAR JIA registry strives to ensure a comprehensive representation of the diverse healthcare landscapes within the continent. Further longitudinal observation studies are required to ascertain the long-term outcomes of our patients and ultimately help inform policy to create a more favorable health ecosystem to support the healthcare needs of JIA patients in Africa.

Does sacroiliitis is a mandatory criterion for enthesitis-related arthritis diagnosis?

INTRODUCTION AND OBJECTIVES: Magnetic resonance imaging (MRI) sensitivity and specificity seem to be less studied in enthesitis-related arthritis (ERA). We aimed to determine the ability of sacroiliac MRI to diagnose ERA patients. MATERIALS AND METHODS: We conducted a retrospective study including 44 patients with juvenile idiopathic arthritis (JIA). Each patient had a sacroiliac joint MRI. We divided patients into two groups: G1 patients with ERA and G2 patients with non-ERA subtype. RESULTS: ERA was noted in 61% of the cases. Sacroiliac joints were painful in 15 patients (34%). MRI was normal in 25 patients (57%) (G1:11 versus G2:14) and showed bone marrow edema in the sacroiliac joints in 19 patients (34%) (G1=16 versus G2=3, p=0.005). Sacroiliac joints MRI's sensitivity and specificity in the ERA diagnosis were 61.54% and 82.35%, respectively. Positive and negative predictive values were 84.21% and 58.33%, respectively. Furthermore, sacroiliac joint pain in the clinical examination was able to predict sacroiliac bone edema in MRI with an odds ratio of 6.8 (95% CI 1.68-28.09; p=0.006). CONCLUSION: Our study showed that sacroiliac joint MRI has good specificity and positive predictive value in the diagnosis of ERA patients among JIA patients. This underlines the usefulness of sacroiliac joint MRI in the early diagnosis of ERA patients.

Pediatric and adult osteoporosis: a contrasting mirror.

Pediatric osteoporosis (PO) is a condition that is currently gaining recognition. Due to the lack of official definitions over the past few decades, the exact incidence of PO is unknown. The research does not provide a specific prevalence of PO in different world regions. However, this is expected to change with the latest 2019 guidelines proposed by the International Society of Clinical Densitometry. Although adult osteoporosis (AO) has been postulated a pediatric disease because its manifestation in adulthood is a result of the bone mass acquired during childhood, differences between PO and AO should be acknowledged. AO is defined as low bone density; however, PO is diagnosed based on existing evidence of bone fragility (vertebral fractures, pathological fractures). This is particularly relevant because unlike in adults, evidence is lacking regarding the association between low bone density and fracture risk in children. The enhanced capacity of pediatric bone for reshaping and remodeling after fracture is another difference between the two entities. This contrast has therapeutic implications because medication-free bone reconstitution is possible under certain conditions; thus, background therapy is not always recommended. In this narrative review, differences between PO and AO in definition, assessment, and medical approach were investigated.

[Clinical examination of the child's foot]. / Examen clinique du pied de l'enfant.

CLINICAL EXAMINATION OF THE CHILD'S FOOT. The clinical examination of the child's foot is part of the musculoskeletal examination and requires a good knowledge of morphological and physiological features of foot during growth. This article describes the stages of the clinical assessment of the child's foot from walking age onwards, as well as the main disorders to be investigated. The examination consists of a complete physical and musculoskeletal assessment, followed by the foot examination. The latter includes a static examination with inspection of bare feet, followed with a podoscope evaluation, a dynamic examination including an overall analysis of walking, and finally an examination of the footwear. The main static disorders to investigate are the flat foot, defined by the collapse of the internal arch of the foot and considered physiological until the age of 5 or 6, and the Pes Cavus, defined by the accentuation of the plantar arch. A thorough examination, followed by a synthetic clinical reasoning, is necessary to guide the diagnostic and therapeutic management of some disorders.

EXAMEN CLINIQUE DU PIED DE L'ENFANT. L'examen clinique du pied de l'enfant fait partie de l'examen ostéoarticulaire et requiert une bonne connaissance des particularités morphologiques et physiologiques du pied, tout au long de la croissance. Cet article décrit les étapes de l'examen clinique du pied de l'enfant à partir de l'âge de la marche, ainsi que les principales anomalies à rechercher. L'examen est constitué d'un examen physique et ostéoarticulaire complet, précédant celui du pied. Ce dernier comporte un examen statique incluant une inspection des pieds nus en position couchée puis en charge sur un podoscope, un examen dynamique avec analyse globale de la marche, et enfin un examen de la chaussure. Les principaux troubles statiques à rechercher sont le pied plat, défini par l'affaissement de l'arche interne du pied et considéré comme physiologique jusqu'à l'âge de 5 ou 6 ans, et le pied creux, défini par l'accentuation de la voûte plantaire. Un examen complet, suivi d'un raisonnement clinique synthétique, est nécessaire afin de guider la prise en charge diagnostique et thérapeutique devant certaines anomalies.

Enthesitis-related arthritis: monitoring and specific tools

Abstract Objective: In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. Sources: The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020. Summary of finding: Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems. Conclusion: Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.