Pediatric Behçet's disease with cardiac valvular lesions: A case-based review.

Due to the insidious nature of pediatric cardiac Behçet's disease (BD), misdiagnosis or missed diagnosis occurred frequently. We described a female pediatric patient with BD with cardiac valvular involvement diagnosed at the age of 4 years with clinical symptoms, including aphthous ulcers, fever, perianal ulcers, and erythema nodosum, as well as significantly elevated inflammatory markers. Echocardiography revealed that previously absent aortic valve lesions developed later and gradually worsened. After being diagnosed with BD with cardiovascular involvement, the patient was treated with glucocorticoids, immunosuppressants, biologics, diuretics, and aortic valvuloplasty. At the time of the follow up, the patient was stable. A review of 13 publications was conducted, including 14 cases of cardiac involvement in pediatric BD.

The quality of life in Chinese juvenile idiopathic arthritis patients: psychometric properties of the pediatric quality of life inventor generic core scales and rheumatology module.

BACKGROUND: Juvenile idiopathic arthritis (JIA) may seriously affects patients' quality of life (QoL), but it was rarely focused and studied in China, so we explore JIA children's QoL using Chinese version of the PedsQL4.0 Generic Core and PedsQL3.0 Rheumatology Module scale, and analyzed the psychometric properties of these two Scales among Chinese JIA children. METHODS: We recruited 180 JIA patients from Children's Hospital Affiliated to Capital Institute of Pediatrics and Hebei Yanda Hospital from July 2018 to August 2019. The questionnaires include information related on JIA, PedsQL4.0 generic core and PedsQL3.0 Rheumatology Module scales. According to the disease type, onset age of and course of JIA, we divided them into different groups, then compared the QoL status among different groups. Moreover, we analyzed the reliability and validity of these two scales in these 180 JIA children. RESULTS: The mean score of PedsQL4.0 generic core scale on these 180 patients was 82.85 ± 14.82, for these in active period was 72.05 ± 15.29, in remission period was 89.77 ± 9.23; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 77.05 ± 19.11, 84.33 ± 12.46 and 87.12 ± 10.23. The mean score of PedsQL3.0 Rheumatology Module scale on 180 patients was 91.22 ± 9.45, for these in active period was 84.70 ± 11.37, in remission period was 95.43 ± 4.48; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 89.41 ± 11.54, 89.38 ± 10.08 and 93.71 ± 6.92. In the PedsQL 4.0 Generic Core scale, the α coefficients of total scale and almost every dimension are all greater than 0.8 except for the school activity dimension of 0.589; the correlation coefficients of 22 items' scores (total 23 items) with the scores of dimensions they belong to are greater than 0.5 (maximum value is 0.864), and the other one is 0.406. In PedsQL3.0 Rheumatology Module scale, except for the treatment and worry dimensions of 0.652 and 0.635, the α coefficients of other dimensions and the total scale are all greater than 0.7; the correlation coefficients of all items' score were greater than 0.5 (the maximum is 0.933, the minimum is 0.515). CONCLUSIONS: The QoL of Chinese JIA children is worse than their healthy peers, these in active period and diagnosed as systemic type were undergoing worst quality of life. The reliability and validity of PedsQL 4.0 Generic Core and PedsQL3.0 Rheumatology Module scale in Chinese JIA children are satisfactory, and can be used in clinical and scientific researches.

Reports of three cases with the initial presentation of mesenteric vasculitis in children with system lupus erythematous.

We reviewed three cases of systemic lupus erythematosus (SLE) in children with mesenteric vasculitis (LMV) as initial presentation and analysed their clinical characteristics to improve the understanding of this disease. Three patients with SLE were admitted to our hospital and initially presented with gastrointestinal symptoms. We retrospectively analysed their clinical data, including clinical presentations, laboratory results, images and short- and long-term treatment outcomes. (1) All three children were school-age girls. The patients were presented to our hospital with vomiting and abdominal pain as initial symptoms. The patients also had urinary symptoms, including proteinuria in three cases, ureteropelvic dilatation in two cases and hydronephrosis in one case. (2) The patients had various positive autoantibodies and a low complement level. Two of the patients had blood system involvement, and one had central nervous system symptoms. (3) All of the patients had active SLE (SLEDAI-2K score ≥ 5 points and moderate to severe degree 10-24). (4) Abdominal CT scans with contrast showed the 'target sign' of the intestinal wall in case 1, a slightly thickened intestinal wall and blurry mesentery in case 2, and the 'comb sign' of the margin mesenteric blood vessels in case 3. (5) All three patients responded promptly to steroid therapy. The patients' symptoms improved rapidly after treatment. LMV is a rare SLE complication. The lack of comprehensive understanding of LMV's clinical presentation makes it considerably challenging to diagnose. LMV is also a serious complication of SLE that is often accompanied by concurrent damage to other organs. LMV often occurs with active SLE but responds rapidly to glucocorticoid therapy. Therefore, in order to make early diagnosis and treatment, we suggest checking autoantibodies and abdominal CT scans with contrast when children present with gastrointestinal symptoms and the involvement of other organs, especially the urinary system.

[Treatment of refractory rheumatism among preschool children with autologous peripheral blood hematopoietic stem cell transplantation].

OBJECTIVE: To investigate the feasibility and safety of autologous peripheral blood hematopoietic stem cell transplantation (auto-PBHSCT) and its therapeutic effect on refractory rheumatism among preschool children. METHODS: Three boys with juvenile rheumatoid arthritis (JRA), juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM) respectively, 3 to 6 years old with the mean age of 5 years with 3.5 to 22 months course of disease with 14 months on average, received auto-PBHSCT. Their conditions were so severe that conventional therapy failed to control the diseases. The changes of both clinical manifestations and immunologic indexes were observed before and after transplantation with long term following up at specialty clinic of rheumatism. RESULT: The time when neutrophil count >or= 0.5 x 10(9)/L in the 3 children was days +9, +13 and +11 respectively, that of platelet count >or= 20 x 10(9)/L was days +14, +18 and +13 respectively. The cellular immune function remained abnormal with CD4 cells at a low level and CD4/CD8 being inverted. As to the JDM child, the skin rash had disappeared and his muscle tone was improved to grade 5 within one month after the transplantation. The EMG and serum creatase level returned to normal and muscle MRI findings were improved greatly within 2 months after the transplantation. As to the JSLE child, skin rash and proteinuria had disappeared, MRI of brain showed that the pathological changes had been absorbed and EEG returned to normal 3 months after the transplantation, all the autoantibodies turned to negative within 8 months after transplantation. As to the JRA child, the arthritis had been improved remarkably within 3 weeks after auto-PBHSCT. There was no swelling of joints nor movement limitation 3 months post transplantation. The steroids and immunosuppressive drugs were discontinued post transplantation. Cushing syndrome disappeared. Their body heights increased by 10 to 15 cm in the past 18 months, and they all returned to school. There was no relapse during follow-up periods of 25 - 27 months. CONCLUSION: The therapy with auto-PBHSCT for refractory rheumatism among preschool children was remarkably effective in a short-term, yet the safety and long-term effect still need to be further studied.