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Objective The aim of this study was to investigate serum levels of soluble B-cell maturation antigen (sBCMA) in childhood-onset systemic lupus erythematous (cSLE) patients with renal involvement, and to elucidate their association with clinical characteristics. Methods 116 cases of cSLE patients with renal involvement (84 females and 32 males; median age 11.6 (10.1, 12.9) years) hospitalized in Department of Pediatric Nephrology and Rheumatology, the First Affiliated Hospital, Sun Yat-sen University and 31 healthy controls (HCs) were enrolled. Serum concentrations of sBCMA were determined using enzyme-linked immunosorbent assay (ELISA). Clinical and laboratory information of cSLE patients were retrospectively analyzed. Results Serum sBCMA levels were significantly increased in primary cSLE when compared with treated cSLE patients and HCs, whereas there was no significant difference between treated cSLE patients and HCs. Patients with high disease activity displayed higher serum sBCMA levels compared with those with no or mild to moderate disease activity. Positive correlation was observed between serum sBCMA levels and systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), antinuclear antibody titers, anti-double-stranded DNA titers, erythrocyte sedimentation rate, and immunoglobulin G levels, while sBCMA levels were negatively correlated with blood white blood cell count, hemoglobin, platelet count, complement C3 and C4 levels. Serum sBCMA levels decreased as disease ameliorated after treatments among 11 cases with follow-up examinations. Conclusions In cSLE patients with renal involvement, serum sBCMA levels correlated significantly with disease activity, immunological, and hematological parameters, but not with renal parameters. Our results suggest the potential and significance of serum sBCMA as a biomarker in cSLE patients.

Background: Identifying the arterial variation of the lateral circumflex femoral artery (LCFA) is a vital step in planning surgical and radiological approach. The aim of the study was to evaluate the variations and discuss the clinical correlates of the LCFA. Materials and methods: Fifty eight adult cadavers (male 45, female 13) with 115 usable sides were used to assess and classify the origin and branches of the LCFA. Also its external diameter, distance from mid-inguinal ligament to sites of origin from the profunda femoris artery or femoral arteries. Results: There were seven types of LCFA variations in this sample. We classified them as types A to G, of which type A was normal, that is, the one showing a single LCFA arising from the profunda femoris artery. Nearly 50.43% of the sample had type B–G variations, each having 13, 10, 23, 4, 4, and 3 cases, accounting for 11.30%, 8.70%, 20.00%, 3.48%, 3.48%, and 2.61%, respectively. Conclusions: There are many variant types in the LCFA. To avoid iatrogenic injuries, clinicians must have a sound understanding of the variation types of this important blood vessel.

IgA nephropathy (IgAN) is often chronically progressive and commonly accompanied by dyslipidemia. However, the intrinsic relationship between dyslipidemia and IgAN remains to be elucidated. This study aimed to investigate the impact of different types of dyslipidemia on clinical and pathological characteristics in children with IgAN.In our retrospective cohort study from January 2006 to January 2021, 276 children with IgAN were ultimately included in the baseline analysis, and 169 were included in the follow-up analysis. The clinical and pathological features of different types of dyslipidemia and their effect on kidney prognosis were analyzed.Children in the dyslipidemia group had more severe clinical characteristics (higher blood urea nitrogen, serum uric acid, and 24-h proteinuria; higher proportion of hypertension; and lower serum albumin and estimated glomerular filtration rate) and pathological changes (higher proportion of Lee grades IV-V and E1, S1, and C2 in MEST-C). Furthermore, the clinical and pathological characteristics were worse in the mixed hyperlipidemia group. Multivariate logistic analysis showed that hypertension, steroid treatment, lower serum albumin, severe proteinuria, and segmental glomerulosclerosis were independent risk factors for dyslipidemia in children with IgAN. The Kaplan-Meier analysis revealed that the probability of kidney survival in children with dyslipidemia was lower than that in those without dyslipidemia, with a median follow-up of 5.9 years.Children with IgAN and dyslipidemia, especially mixed hyperlipidemia, are prone to more severe clinical and pathological changes. Our study provides further insight into dyslipidemia as a potential risk factor in children with IgAN. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background: This study aimed to summarize the clinicopathological features and prognostic risk factors of primary antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in children.Methods: Clinical and prognostic data for children admitted to our center with AAV between September 2003 and September 2020 were studied retrospectively. The incidence and risk factors of end-stage renal disease (ESRD) were calculated and analyzed.Results: Thirty-four children were enrolled; 28 were female, with a median onset age of 10 years. Except for one case negative for ANCA, the other 33 patients were diagnosed with microscopic polyangiitis (MPA). The most frequently involved organ was the kidney (100.0%), followed by the lungs (58.8%) and heart (50.0%). Twenty children (58.8%) progressed to ESRD with a median course of 3 months, and they were more likely to present respiratory and cardiovascular system involvement than were the non-ESRD group (P < 0.05). Patients in the ESRD group also had a higher serum creatinine level, 24-h protein excretion, Pediatric Vasculitis Activity Score (PVAS), and a lower level of estimated glomerular filtration rate (eGFR), hemoglobin, and complement C3 than had those in the non-ESRD group (P < 0.05). The main pathological manifestations were crescentic and sclerotic classes in the ESRD group and focal class in the non-ESRD group. After 6 months of induction therapy, 90.0% of cases achieved complete or partial remission. The multivariate logistic regression model showed that baseline eGFR < 60 ml/min/1.73 m2 was an independent risk factor for progressing to ESRD (OR = 0.016, 95% CI = 0.001~0.412, P = 0.012).Conclusions: AAV in children usually occurs in teenage girls, and the most commonly involved organ is the kidney, of which hematuria is the most common symptom, followed by proteinuria, abnormal renal function (eGFR < 90 ml/min/1.73 m2), etc. The primary type of AAV is MPA. Nearly 60% of patients progressed to ESRD with a median course of 3 months. Baseline eGFR < 60 ml/min/1.73 m2 is an independent risk factor for ESRD progression in AAV children.

Background: The aim of the study was to probe the morphological features of the proximal segment (V1) of vertebral artery (VA) in a sample of Chinese cadavers. Materials and methods: The origin, course and outer diameter at origin of the pre-vertebral part of the VAs were evaluated in 119 adult cadavers. Results: It was found that 94.12% of the VAs originated from the subclavian arteries, bilaterally. The variant origins were present in 5.88% of the cadavers and all originated directly from the arch of the aorta. All the variations were observed on the left side of male cadavers. The average outer diameters at origin of the normal and variation groups were 4.35 ± 1.00 mm and 4.82 ± ± 1.42 mm, respectively, p = 0.035. In the normal group, but not in the variation group, the average diameter in the males was significantly larger than that in the females (4.50 ± 0.99 mm, 3.92 ± 0.92 mm, respectively, p = 0.000). In addition, only 5 cadavers in the normal group had hypoplastic VAs (4.20%, 4 males, 3 right-sided). Vertebral artery dominance (VAD) was present in 91 (69 males) out of 112 cadavers and more common on the left (n = 48). In addition, 3 cadavers satisfied conditions for coexistence of VAD and vertebral artery hypoplasia. All 7 cadavers in the variation group exhibited VAD, which was more common on the right side (n = 5). Conclusions: The morphologic variations and frequencies described above have implications for the early prevention, abnormal anatomy detection, accurate diagnosis, safe surgery and endovascular treatment of cardiovascular and neurological disease.

Objectives The aim of this study is to determine the incidence and explore the types of aortic arch branch variations found in our cadavers. Methods The types and incidence of aortic branch variations in 120 cadavers were analysed after careful dissection. Results One hundred and six of 120 cadavers had normal aortic arch branches and gave rise to usual branches, namely the brachiocephalic trunk, the left common carotid artery and the left subclavian artery. The remaining 14 cadavers had 2 basic types of branch variations, thus accounting for an incidence of 11.67%. A total of 9 aortic arches emitted 4 branches; the brachiocephalic trunk, the left common carotid artery, the left vertebral artery and the left subclavian artery (incidence 7.5%). The second subgroup of 5 cadavers also emitted 4 aortic branches: the right common carotid artery, the left common carotid artery, the left subclavian artery and the right subclavian artery (incidence 4.16%). In this group, the right subclavian artery sprung as a distal branch of the aortic arch (descending), thus making a vascular ring that takes a superoposterior course round the back of the trachea and the oesophagus to reach the right side. There was a single cadaver, different from the other 4 aortic branches of the second group which had a common origin for the common carotid arteries, while the left subclavian artery and distally placed right subclavian artery were present. We did not observe any Kommerell's aortic diverticula. Conclusions The variations of aortic arch branching are complex and diverse due to varied possible alterations in the embryological processes. There is an imperative need for further research on these variations to elucidate the possible relationships with clinical diagnostic or surgical events.