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OBJECTIVES: To evaluate the accuracy of greyscale ultrasound (US) and colour Doppler detecting placenta accreta spectrum (PAS) based on the newly recommended International Federation of Obstetrics and Gynaecology (FIGO) grading system. METHODS: This prospective study was conducted on women diagnosed with placenta previa or low-lying placenta involving the anterior uterine wall and associated with PAS as identified by the US. Transabdominal and transvaginal greyscale US was performed on admission between 34 and 36 weeks of gestation and compared to clinical grading and histopathological examination after cesarean hysterectomy. RESULTS: In total, 36 pregnant females who underwent a cesarean hysterectomy due to placenta previa complicated by PAS were included in this study. All patients had a history of previous cesarean deliveries, ranging from 1 to 5 deliveries. The US has an overall sensitivity of 33%, 55%, and 84.62%, and specificity of 100%, 75%, and 60% in detecting the 3 degrees of PAS, respectively. US cannot differentiate between the different subtypes of PAS grade 3 (a, b, and c). CONCLUSION: The overall US evaluation was highly significant in predicting the FIGO diagnosis of PAS; however, all ultrasonographic signs were equally relevant in diagnosing grade 1 and/or 2 PAS and were inaccurate in differing the subtypes of PAS grade 3.
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Placenta Acreta , Placenta Prévia , Gravidez , Feminino , Humanos , Placenta Acreta/diagnóstico por imagem , Ultrassonografia Pré-Natal , Estudos Prospectivos , Ultrassonografia , Estudos Retrospectivos , PlacentaRESUMO
BACKGROUND: Determining the role of epigenetics in systemic juvenile idiopathic arthritis (SJIA) provides an opportunity to explore previously unrecognized disease pathways and new therapeutic targets. AIM: We aimed to identify the clinical significance of microRNAs (miRNA-26a, miRNA-223) in SJIA. MATERIALS AND METHODS: This cross-sectional study was conducted on a group of children with SJIA attending to pediatric rheumatology clinic, at Mansoura University Children's Hospital (MUCH) from December 2021 to November 2022. Patient demographics, and clinical, and laboratory data were collected with the measurement of microRNAs by quantitative real-time PCR. The Mann-Whitney, Kruskal-Wallis, and Spearman correlation tests were used for variable comparison and correlations, besides the receiver operating characteristic (ROC) curve for microRNAs disease activity and treatment non-response discrimination. RESULTS: Forty patients were included in the study. On comparison of miRNA-26a, and miRNA-223 levels to the clinical, assessment measures, and laboratory features, miRNA-26a was statistically higher in cases with systemic manifestations versus those without. Similarly, it was higher in children who did not fulfill the Wallace criteria for inactive disease and the American College of Rheumatology (ACR) 70 criteria for treatment response. Meanwhile, miRNA-223 was not statistically different between cases regarding the studied parameters. The best cut-off value for systemic juvenile arthritis disease activity score-10 (sJADAS-10) and the ability of miRNA-26a, and miRNA-223 to discriminate disease activity and treatment non-response were determined by the (ROC) curve. CONCLUSION: The significant association of miRNA-26a with SJIA features points out that this molecule may be preferentially assessed in SJIA disease activity and treatment non-response discrimination.
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Artrite Juvenil , Epigênese Genética , MicroRNAs , Fenótipo , Humanos , Artrite Juvenil/genética , Artrite Juvenil/diagnóstico , Artrite Juvenil/terapia , Criança , Feminino , Estudos Transversais , Masculino , MicroRNAs/genética , Pré-Escolar , Adolescente , Resultado do Tratamento , Antirreumáticos/uso terapêuticoRESUMO
PURPOSE: Pediatric uveitis poses unique challenges, characterized by difficulties in performing comprehensive examinations, potential delays in diagnosis, and a heightened risk of ocular complications. This study evaluate the etiologic and clinical characteristics of uveitis in children presenting to the Mansoura Ophthalmic Center, Mansoura, Egypt. METHODS: A cross-sectional observational study was undertaken involving children diagnosed with uveitis attending the uveitis outpatient clinic at Mansoura University Ophthalmic Center. Comprehensive clinical evaluations were carried out, including detailed history taking and exhaustive ophthalmological examinations. Whenever deemed necessary, Spectral Domain Optical Coherence Tomography (OCT) and Fluorescein Fundus Angiography (FFA) were utilized to secure retinal images. An extensive systemic evaluation was also conducted to discern the diverse causes of uveitis among the participants. RESULTS: The cohort comprised 63 children, impacting 97 eyes. Bilateral involvement was seen in 54% of cases, with a male predominance of 58.7%. The predominant etiologies of uveitis were presumed trematode-induced (36.7%), Juvenile Idiopathic Arthritis (JIA) accounting for 28.6%, and in 12.7% of cases, the cause remained undetermined. Anterior uveitis emerged as the primary presentation in 79.4% of cases. Regarding visual loss, cataract was the leading cause at 56.4%, followed by vitritis at 38.4%, and macular edema at 20.5%. CONCLUSION: Anterior uveitis was the most frequent presentation in our pediatric cohort. Despite the challenges, the majority of children with uveitis exhibited no significant visual impairment, with most causes of visual loss being reversible.
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BACKGROUND: Measurement of the circulating levels of long-non-coding RNAs (lncRNAs) in lupus nephritis (LN) patients could dramatically explore more insights about the disease pathogenesis. Hence, we aimed to quantify the level of expression of CTC-471J1.2 and NeST in LN patients and to correlate it with the disease activity. METHOD: This case-control study was conducted on a group of children with juvenile LN attending to Mansoura University Children's Hospital (MUCH). Demographics, clinical, and laboratory findings were collected besides the measurement of lncRNAs by quantitative real-time PCR. RESULTS: The expression level of lncRNAs-CTC-471J1.2 was significantly down-regulated in children with active LN versus inactive cases or controls. In contrast, the NeST was significantly up-regulated in active LN cases. A significant correlation was found between CTC-471J1.2 expression and LN activity parameters. Additionally, both lncRNAs showed a reasonable sensitivity and specificity in differentiation of active LN. A regression analysis model revealed that CTC-471J1.2 and NeST were independent predictors of active nephritis. CONCLUSION: The expression level of circulatory lncRNAs-CTC-471J1.2 and NeST can be used as sensitive and specific biomarkers for active LN. Furthermore, both could serve as predictors for nephritis activity.
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Nefrite Lúpica , RNA Longo não Codificante , Nefrite Lúpica/genética , Nefrite Lúpica/sangue , Humanos , RNA Longo não Codificante/genética , RNA Longo não Codificante/sangue , Estudos de Casos e Controles , Feminino , Criança , Masculino , Fatores de Risco , Adolescente , Epigênese Genética , Biomarcadores/sangue , Biomarcadores/metabolismoRESUMO
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.
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Artrite Juvenil , Sistema de Registros , Humanos , Feminino , Masculino , Artrite Juvenil/epidemiologia , Artrite Juvenil/tratamento farmacológico , Artrite Juvenil/diagnóstico , Criança , Estudos Retrospectivos , Pré-Escolar , África/epidemiologia , Antirreumáticos/uso terapêutico , Sociedades Médicas , ReumatologiaRESUMO
Background and Objectives: Rheumatoid factor (RF) and anti-cyclic citrullinated protein (anti-CCP) have been used to improve the diagnosis and prognosis of rheumatoid arthritis (RA). However, their association with RA disease phenotypes, individually and in combination, is not well studied. The aim of the study was to compare patients' and disease characteristics, activity and severity in double seronegative (DNRA), single seropositive RF, single seropositive anti-CCP and double seropositive (DPRA) patients. Methods: Adults subjects with RA from Egyptian College of Rheumatology (ECR) database who had RF and anti-CCP results available were included. Demographic, clinical features, disease activity score 28 (DAS28), Health Assessment Questionnaire (HAQ) and laboratory data were collected and compared among different RA groups. Results: 5268 RA patients with mean age of 44.9±11.6 years, and 4477 (85%) were females. 2900 (55%) had DPRA, 892 (16.9%) had single positive RF, 597 (11.3%) had single positive anti-CCP while 879 (16.7%) had DNRA. Patients with DPRA had significantly high percentage of metabolic syndrome (19.3%, P < 0.001), and functional impairment using HAQ (P = 0.01). Older age (RRR [relative risk ratio]: 1.03, 95%CI: 1.0, 1.0, P = 0.029), greater DAS28 (RRR: 1.51, 95%CI: 1.2, 1.9, P < 0.001), higher steroid use (RRR: 2.4, 95%CI: 1.36, 4.25, P = 0.002) were at higher risk of DPRA while longer disease duration (RRR: 1.08, 95%CI: 1.01, 1.16, P = 0.017) and fibromyalgia syndrome (RRR: 2.54, 95%CI: 1.10, 5.88, P = 0.028) were associated with higher odds of single positive RF status. Conclusion: Dual antibody-positive status has higher disease activity and severity, and higher chance of development of metabolic syndrome; highlighting the implicated role of inflammation, atherogenesis and cardiovascular disease risk in RA.
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OBJECTIVE: To explore the different menstrual and pubertal abnormalities in adolescent females with systemic autoimmune rheumatic diseases (ARD). METHODS: The study included adolescent girls aged 13-18 years with juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (JSLE), and juvenile dermatomyositis (JDM) classified according to their international classification criteria. Data were collected from our patients' files and interpreted with respect to the demographic, clinical, disease assessment parameters, medications used, and the hormonal profile. The aspects of puberty and menstruation were assessed by a gynaecologist with ultrasound evaluation as well. The girls were classified according to their menstrual pattern into those with regular cycles versus abnormal ones. The subgroups were compared and significant variables entered into a logistic regression model to detect the independent predictors. RESULTS: Twenty-one girls with JSLE were included, besides 23 JIA and 8 JDM cases. Ten patients with JSLE (47.6%) had menstrual abnormalities, whereas only four JIA (17.4%) and 1 JDM girls had these alterations without significant difference between the three groups. The median of the SLICC/ACR damage index was statistically higher in JSLE with abnormal menstrual cycles, similarly were the cumulative steroid dose and puberty onset. No difference was observed between JIA or JDM subgroups concerning the disease parameters, hormonal profile, ultrasound assessment or the treatment lines. The most significant predictor for menstrual abnormalities in JSLE was the SLICC/ACR damage index. CONCLUSION: Menstrual abnormalities is a common disturbance among adolescent girls with ARDs. The SLICC/ACR damage index is the main determinant for menstrual abnormalities rather than the cumulative steroid use or disease duration in JSLE.
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Artrite Juvenil , Dermatomiosite , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Feminino , Humanos , Adolescente , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/diagnóstico , Dermatomiosite/complicações , Puberdade , Esteroides , Doenças Reumáticas/complicaçõesRESUMO
BACKGROUND: About 25-50% of multisystem inflammatory syndrome in children (MIS-C) patients meet the criteria for diagnosis of Kawasaki disease (KD). The differentiation of both conditions is so challenging on clinical practice as the management of both is time dependant and precise diagnosis is fundamental. METHOD: Data were collected from children < 18 years old hospitalized with MIS-C or KD. Patient demographics, clinical, and laboratory data were compared, and a discrimination score was created to assist in clinical differentiation. RESULTS: 72 patients with MIS-C and 18 with KD were included in the study. Patients with MIS-C had a higher prevalence of abdominal pain (p = 0.02), vomiting (p = 0.03), and cervical lymphadenopathy (p = 0.02) compared with KD cases. MIS-C patients had higher liver enzymes (aspartate aminotransferase (AST) (p = 0.04), alanine aminotransferase (ALT) (p = 0.03), serum creatinine (p = 0.03), and lower platelet count nadir (p = 0.02) than KD. Four variables were detected in the regression analysis model, and the independent predictors were utilized to generate a scoring model that distinguished MIS-C from KD with an area under the curve of 0.70. CONCLUSION: This study constructed a prediction model for differentiation of MIS-C from KD based on clinical and laboratory profiles. This model will be valuable to guide clinicians in the treatment decisions. Key Points ⢠Children with MIS-C are more likely to have gastrointestinal symptoms, cervical lymphadenopathy, and respiratory involvement than KD patients. ⢠Elevated liver enzymes and lower platelet count are more pronounced laboratory findings in MIS-C than KD. ⢠This study constructed a prediction model for differentiation of MIS-C from KD based on clinical and laboratory profiles. This model will be valuable to guide clinicians in the treatment decisions.
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COVID-19 , Síndrome de Linfonodos Mucocutâneos , Criança , Humanos , Adolescente , SARS-CoV-2 , Estudos de Coortes , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , COVID-19/epidemiologiaRESUMO
OBJECTIVE: Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR. METHODS: Consenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants. RESULTS: One hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P < 0.001). The CDAS significantly decreased after initiating second-line treatments from median 12.0 (IQR 8.0-15.5) to 5.0 (IQR 3.0-12.0; P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event. CONCLUSION: The CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER.
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Pesquisa Comparativa da Efetividade , Osteomielite , Criança , Adulto Jovem , Humanos , Estudos de Viabilidade , Estudos Prospectivos , Osteomielite/tratamento farmacológico , Osteomielite/patologia , Doença CrônicaRESUMO
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may present with some systemic lupus erythematosus (SLE) manifestations intermingled with Kawasaki disease features. These emerging presentations were dubbed under the umbrella term 'multisystem inflammatory syndrome in children (MIS-C)'. A one and half-year-old girl, admitted to Mansoura University Children's Hospital (MUCH) with fever, bad general condition, vomiting, widespread maculopapular, vasculitic rash, hands and feet oedema, oral ulceration, arthralgia and lymphadenopathy. Moreover, bicytopenia, positive antinuclear, anti-double-stranded DNA antibodies and low C3 qualified her as a case of juvenile SLE. Despite the child received the initial therapy of immunosuppressive medication, her general condition deteriorated with fever persistence and rash exacerbation. At that time, the skin of her hands and feet started to peel. Thus, an expanded study for other alternatives was obligatory; SARS-CoV-2 infection testing revealed positive IgG serology, and retesting for lupus autoantibodies turned negative. HRCT chest showed bilateral basal consolidation with ground-glass appearance. Furthermore, Echo exhibited coronary artery dilation with thrombus inside. This evolution raised the concern for COVID-related MIS-C syndrome. This report provides a model of COVID-19 heterogeneity with protean immune-related manifestations. This case has a unique presentation that necessities its description, in order to provide a nidus for future studies in this new entity.
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COVID-19 , Exantema , Lúpus Eritematoso Sistêmico , Anticorpos Antivirais , Autoanticorpos , COVID-19/complicações , COVID-19/diagnóstico , Criança , DNA , Exantema/etiologia , Feminino , Febre , Humanos , Imunoglobulina G , Lactente , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnósticoRESUMO
To describe a unique presentation of fever and bilateral bicipital synovial cysts in young female with systemic juvenile idiopathic arthritis (SJIA). A previous healthy 5-year-old female presented with spiking fever and sudden painless bilateral swelling on the flexor aspect of the upper arm. Ultrasonography confirmed a cystic structure with multiple septae, thickening and tenosynovitis of bicipital tendon but without Doppler signals inside or shoulder synovitis, enlightens the term of non-articular synovitis. Thorough analysis of clinical manifestations, disease assessment measures, laboratory findings and imaging of arm swellings eventually disclosed unusual case of SJIA. Systemic juvenile idiopathic arthritis should be considered in case of spiking fever with bicipital synovial cysts. Ultrasound is a useful diagnostic tool in this condition.