Clinical characteristics and prognosis of 12 cases of lupus nephritis complicated with thrombotic microangiopathy / 中国当代儿科杂志

OBJECTIVES@#To investigate the clinical characteristics, pathological features, treatment regimen, and prognosis of children with lupus nephritis (LN) and thrombotic microangiopathy (TMA), as well as the treatment outcome of these children and the clinical and pathological differences between LN children with TMA and those without TMA.@*METHODS@#A retrospective analysis was conducted on 12 children with LN and TMA (TMA group) who were admitted to the Department of Nephrology, Children's Hospital of Nanjing Medical University, from December 2010 to December 2021. Twenty-four LN children without TMA who underwent renal biopsy during the same period were included as the non-TMA group. The two groups were compared in terms of clinical manifestations, laboratory examination results, and pathological results.@*RESULTS@#Among the 12 children with TMA, 8 (67%) had hypertension and 3 (25%) progressed to stage 5 chronic kidney disease. Compared with the non-TMA group, the TMA group had more severe tubulointerstitial damage, a higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score at onset, and higher cholesterol levels (P<0.05). There were no significant differences between the two groups in the percentage of crescent bodies and the levels of hemoglobin and platelets (P>0.05).@*CONCLUSIONS@#There is a higher proportion of individuals with hypertension among the children with LN and TMA, as well as more severe tubulointerstitial damage. These children have a higher SLEDAI score and a higher cholesterol level.

Clinical and immunological features of lupus nephritis in children: retrospective analysis of 40 cases / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To analyze the clinical and immunological features of children with lupus nephritis (LN).</p><p><b>METHODS</b>Chart records of 40 (4 male and 36 female) LN children who were admitted consecutively between January, 2005 and December, 2010 were reviewed. The baseline demographic, pathological and immunological data were analyzed.</p><p><b>RESULTS</b>In the 40 LN patients analyzed, the mean age of the disease onset was 10.6 ± 2.6 (range from 2.6 to 14.3) years, and 35 cases (88%) were school-age children. Proteinuria was detected in all 40 cases, including nephrotic-range proteinuria in 12 (30%) cases, and isolated proteinuria in 9 (22%) cases. Twenty-six (65%) patients had varying degrees of hematuria. Acute nephritis was the most common sub-type, accounting for 47% of the total cases. Among the 39 cases undergoing renal biopsy, 3 were unclassified and the remaining 36 were classified, respectively, as type IV LN (50%, 18 cases), type II LN (22%, 8 cases). In the histopathologcally classified case, 100% were antinuclear antibody-positive, 61% were anti-dsDNA-positive, and 89% showed varying degrees of decrease in serum C3 and C4 concentrations. Following treatment for 6 months, a high LN remission rate (95%) was achieved; the acute renal activity index remained higher in IV, V+III and V+IV subtypes than in other subtypes, while the chronic index and the degree of tubulointerstitial damage were not different between histopathological subtypes.</p><p><b>CONCLUSIONS</b>The clinical manifestations of LN children are diverse. Clinically, acute nephritis is the most common form of LN in children. Histopathologically, type IV is the most frequent subtype of LN. Early treatment may result in significant disease remission.</p>

Effects of core proteoglycan on the transdifferentiation of human renal tubular epithelial cell induced by transforming growth factor-beta1 in vitro / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the effects of core proteoglycan on the transdifferentiation of human renal tubular epithelial cell induced by transforming growth factor beta1 (TGF-beta1) in vitro.</p><p><b>METHOD</b>The cultured HK-2 cells were divided into six groups: A. negative control group; B. 10 ng/ml TGF-beta1 group; C. 10 ng/ml core proteoglycan treated group; D. 100 ng/ml core proteoglycan treated group; E. 10 ng/ml TGF-beta1 + 10 ng/ml core proteoglycan group; F. 10 ng/ml TGF-beta1 + 100 ng/ml core proteoglycan group. The changes in configuration of HK-2 cells were inspected 48 hours after adding the stimulating factor. At the same time, changes in mRNA of keratin, alpha-smooth muscle actin, vimentin were analyzed.</p><p><b>RESULTS</b>Compared with group A, group B showed great changes in the morphology of cells, most cells converted into spindle shape, like fibroblast; groups E and F, especially group F showed significantly reduced spindle shape cells. Compared with group A, groups C and D had no significant changes in morphology of cells Compared with 10 ng/ml TGF-beta1 group and negative control, the mRNA expression of alpha-smooth muscle actin and vimentin had significant increase, but that of keratin reduced (P < 0.05). However, after combined treatment with TGF-beta1 and core proteoglycan, alpha-smooth muscle actin and vimentin expression were reduced significantly, while expression of keratin was up-regulated. Single core proteoglycan treated group and negative control group had no dramatic differences (P > 0.05).</p><p><b>CONCLUSION</b>TGF-beta1 can induce the transdifferentiation of human renal tubular epithelial cell and core proteoglycan has some inhibitory effect on transdifferentiation of human renal tubular epithelial cell induced by TGF-beta1 in vitro.</p>