Associations of Raynaud's Phenomenon in Juvenile Onset Systemic Lupus Erythematosus

INTRODUCTION: Raynaud's phenomenon (RP) is associated with mild disease in adults with systemic lupus erythematosus (SLE). Although RP is more frequently reported in juvenile onset SLE (jSLE) clinical associations were not studied. We aimed to investigate whether the presence of RP is associated with clinical and serological features at onset and outcomes in children with SLE. METHODS: Medical charts of children diagnosed with jSLE were reviewed. The presence of RP was evaluated by the presence of patients reporting symptoms, and patients were compared according to the presence of RP. RESULTS: Among the 52 included patients, 13 (25.0%) displayed RP. The clinical and laboratory features did not significantly differ according to the presence of RP. However, positive anti-dsDNA (76.9% vs. 35.8%, p = 0.02), anti-RNP (53.8% vs. 17.9%, p = 0.02), and anti-centromere (23.0% vs. 2.5% p = 0.04) antibodies were more frequently observed in patients with RP than in those without. RP was not found to be associated with initial disease severity, flare rate, remission, or damage accrual. DISCUSSION AND CONCLUSION: RP was associated with certain serological features. However, RP was not strongly associated with clinical features. Moreover, neither disease severity nor disease outcomes differed according to the presence of RP in children with SLE

COVID-19 in pediatric nephrology centers in Turkey

Background/aim: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. Materials and methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. Results: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10–15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. Conclusion: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients’ susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage

Endoscopic Findings of Children with Familial Mediterranean Fever

Purpose: Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by periodic fever, synovitis and serositis. Patients may be admitted to gastroenterology units due to gastrointestinal symptoms. In this study; we aimed to analyze endoscopic findings and diagnostic utility of endoscopic procedure in children with FMF

Neonatal ulcerative colitis associated with Familial Mediterranean fever: a case report

Neonatal inflammatory bowel disease (IBD) is a subclass of very early onset IBD that includes children younger than 1 month. It is characterized by more colonic involvement and monogenetic etiology, resistance to classical anti-inflammatory/immunomodulatory treatments and associated with colitis in first-degree family members. Herein we report a 3 month-old girl who was admitted with bloody diarrhea since 10 days of age. Her symptoms persist despite diet elimination. She was diagnosed with neonatal ulcerative colitis (UC) based on clinical, laboratory and histopathological examination. But, she was unresponsive to the immunosuppressive therapy. On the follow-up, she was hospitalized for the high fever two times. Genetic analysis revealed homozygote M694 V mutation. Bloody diarrhea and other clinical findings were improved after colchicine therapy. Neonatal UC associated with Familial Mediterranean fever is an extremely rare condition and to the best of our knowledge our case is the first case in literature. Early diagnosis autoinflammatory disease may prevent complications related to unnecessary immunosuppressive drug usage and the risk of development of amyloidosis associated with autoinflammatory disorders

Prevalence of celiac disease in children with joint hypermobility.

Introduction: Generalized joint hypermobility is a clinical feature that is associated with excessive joint laxity, which can occur alone or with various inherited disorders. The term of benign joint hypermobility or joint hypermobility is used when the presence of musculoskeletal symptoms in subjects with generalized joint hypermobility in the absence of demonstrable systemic rheumatic diseases. In recent studies, it was shown that there is a strong relationship between structural and functional gastrointestinal disorders and joint hypermobility. We aimed to analyze the prevalence of celiac disease in a group patient with joint hypermobility. Patients and methods: The study included the 2 groups of children (i) Group 1; patients with joint hypermobility that were followed in pediatric rheumatology outpatient clinic (n=131). (ii) Group 2; healthy children without known chronic diseases (n=995). Demographic features, clinical findings, accompanying symptoms and anthropometric measurements of all patients were recorded. All cases were screened for celiac disease by serological marker and histopathological examinations if serological marker was positive. Results: There was no difference between two groups for age, gender, presence of malnutrition and accompanying symptoms (p>0.05). Serology positivity of anti-tissue transglutaminase IgA >20 RU/ml was found in seven patients with joint hypermobility. After histopathological examinations, asymptomatic celiac disease was detected in one (n=1, 0.9%) and potential celiac disease in six patients (n=6, 5.3%). There were six (0.6%) patients with positive serology in the control group. Celiac serology positivity and potential celiac disease were higher in patients with joint hypermobility (6.2%, vs. 0.6%, OR: 10.9, 95% CI: 3.6-33, p < 0.001 and 5.3%, vs. 0.4%, OR: 13.9, 95% CI: 3.6-50, p < 0.001, respectively), but no significant difference was found on the prevalence of asymptomatic celiac disease (0.9%, vs. 0.2%, OR: 4.4, p=0.22). Conclusion: Our study shows the increased prevalence of potential celiac disease in patients with joint hypermobility. Serological screening of celiac disease is recommended for to rule out organic problems in the presence gastrointestinal symptoms in patients with BJH