OBJECTIVETo analyze the clinical and immunological features of children with lupus nephritis (LN).

METHODSChart 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.

RESULTSIn 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.

CONCLUSIONSThe 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.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Lupus Nephritis ; drug therapy ; immunology ; pathology ; Male ; Retrospective Studies

Objective: To explore the short-term efficacy and safety of dapagliflozin in children with hereditary proteinuric kidney disease. Methods: This was a prospective cohort study. From August 2020 to December 2021, 23 children with hereditary kidney disease from Children's Hospital of Fudan University were enrolled. Patients received dapagliflozin 5 mg/d (weight≤30 kg) or initial dose 5 mg/d for 1 week, then 10 mg/d (weight>30 kg) and the dose of angiotensin converting enzyme inhibitors was stable during treatment. Clinical data including demographic parameters, primary diagnosis, estimated glomerular filtration rate (eGFR), 24 h proteinuria and characteristics in the follow-up were collected. The primary outcome was the change in 24 h proteinuria at 12 (±2) weeks, secondary outcomes included changes of 24 h proteinuria at 24 (±2) weeks, eGFR at both 12 (±2) and 24 (±2) weeks. The data were analysed by using mixed linear model. Results: Totally 23 patients were enrolled, including 16 males and 7 females. The age was (10.8±2.9) years. The primary diseases were Alport syndrome (12 cases), Dent disease (5 cases), proteinuria (4 cases), and focal segmental glomerulosclerosis (2 cases) respectively. Primary outcome showed that 24 h proteinuria decreased from baseline at 12 (±2) weeks during treatment (1.75 (1.46, 2.20) vs. 1.84 (1.14, 2.54) g/m², P<0.05). Secondary outcomes showed that there was no significant difference in 24 h urine protein at 24 (±2) weeks (P>0.05). eGFR decreased slightly at 12 (±2) weeks ((107±21) vs. (112±28) ml/(min·1.73m²), P<0.05), and there was no significant difference at 24 (±2) weeks (P>0.05). Serum albumin increased at 12 (±2) and 24 (±2) weeks following the treatment ((39±8) vs. (37±8) g/L, (38±7) vs. (37±8) g/L, both P<0.05). No hypoglycemia event was reported during the treatment. Conclusion: The dapagliflozin had therapeutic effects on decreasing proteinuria and increasing serum albumin in short-term treatment in children with hereditary proteinuric kidney disease, no hypoglycemia or serious adverse events were observed.
Female ; Male ; Humans ; Child ; Adolescent ; Prospective Studies ; Nephritis, Hereditary ; Proteinuria/drug therapy* ; Serum Albumin

OBJECTIVE: To study the clinical effect and mechanism of total glucosides of paeony (TGP) in the adjuvant therapy for children with Henoch-Schönlein purpura nephritis (HSPN). METHODS: Sixty-four HSPN children with moderate proteinuria were divided into a TGP treatment group ( RESULTS: Compared with the healthy children before treatment, the children with HSPN had higher proportion of Tfh cells and expression levels of IL-21 and IL-4 ( CONCLUSIONS TGP has a marked clinical effect in the treatment of HSPN and can reduce the inflammatory response of the kidney and exert a protective effect on the kidney by inhibiting the proliferation of Tfh cells and downregulating the expression of IL-21 and IL-4 in plasma.
Child ; Glucosides/therapeutic use* ; Humans ; Nephritis ; Paeonia ; Prospective Studies ; Purpura, Schoenlein-Henoch/drug therapy*

Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease entity usually occurring in children. In the present study a case of TINU syndrome in an elderly patient is described and relevant literature is reviewed. A 61-year-old man presented with bilateral flank pain, urinary frequency, and foamy urine. A kidney ultrasonography revealed an increase in kidney parenchyma echogenicity. Following a kidney biopsy, the patient was diagnosed with acute tubulointerstitial nephritis. An ophthalmology examination initially performed for floater symptoms, revealed anterior uveitis in both eyes. Acute tubulointerstitial nephritis and anterior uveitis in both eyes responded to treatment with oral prednisolone, furosemide, carvedilol, and a topical steroid. TINU syndrome can occur in the elderly and should be part of the differential diagnosis when seeing a patient who has uveitis in association with renal disease; any therapy should be managed by both an internist and an ophthalmologist.
Biopsy ; Diagnosis, Differential ; Glucocorticoids/therapeutic use ; Humans ; Male ; Middle Aged ; Nephritis, Interstitial/*diagnosis/drug therapy ; Uveitis/*diagnosis/drug therapy

OBJECTIVETo systematically evaluate the clinical effects of cyclosporine A (CsA) and tacrolimus, which are calcineurin inhibitors, on lupus nephritis.

METHODIn this study, the clinical trials on treatment of lupus nephritis with cyclosporine A and tacrolimus published until May 2010 were searched at www.guideline.gov, www.nice.org.uk, mdm.ca/cpgsnew/cpgs/index.asp, www.show.scot.nhs.uk, www.nzgg.org.nz, www.eguid elines.co.uk, www.gin.net, Cochrane library, EMBASE, MEDLINE, Wanfang database, Chinese Journal full-text Database, Chongqing Weipu Database by using the methods of Cochrane systematic review. At the same time the information from related journals, professional data and network were hand-searched. The homogeneous evaluation was performed by meta-analysis.Statistical analysis of clinical data was performed by using RevMan 4.2 software provided by the Cochrane Collaboration.

RESULTA total of 214 reports were found, while only 7 randomized controlled trials met the inclusion criteria, 4 of them were on the treatment with CsA (treatment group) and cyclosporine (CTX) group (control group), and 3 of them were the on treatment with FK506 (treatment group) and CTX group (control group). There were 148 reports in the treatment of CsA and CTX group, while 185 reports in the treatment of FK506 and CTX group. Both CsA and tacrolimus group could decrease daily urinary protein. Tacrolimus group was good at reducing daily urinary protein as compared with CTX group, and the difference was statistically significant (Z = 2.8, P = 0.005), but there was no significant difference between CsA and CTX groups (Z = 1.08, P = 0.28). Tacrolimus group was good at complete remission as compared with CTX group (Z = 3.64, P = 0.0003), partial remission was similar in both groups (Z = 0.53, P = 0.6), and tacrolimus group was good at total remission (Z = 2.2, P = 0.03). There was no significant difference between CsA and CTX group in side effect within a short period, while FK506 had less side-effect than CTX group.

CONCLUSIONCompared with the treatment with CTX, tacrolimus was good at reducing daily urinary protein. CsA and CTX were similar in reducing daily urinary protein in the treatment of lupus nephritis. Tacrolimus resulted in better total remission than CTX and had less side effect. CsA and CTX groups were similar in side effect. On the whole, calcineurin inhibitor could significantly decrease daily urinary protein, and tacrolimus was better in treatment and had less side-effect than CTX. However, large scale, multicenter, well-designed clinical trials should be adopted to further confirm the conclusions.

Calcineurin Inhibitors ; Cyclosporine ; therapeutic use ; Enzyme Inhibitors ; therapeutic use ; Humans ; Lupus Nephritis ; drug therapy ; Tacrolimus ; therapeutic use

OBJECTIVETo observe the therapeutic effect of a drug pair of Radix Astragali and Rehmanniae Radix combined with glucocorticoid (GC) in treating lupus nephritis (LN) patients and its influence on some experimental indices.

METHODSTotally 52 LN patients were randomly assigned to the treatment group (treated by routine Western medicine and a drug pair of Radix Astragali and Rehmanniae Radix, 25 cases) and the control group (treated by Western medicine, mainly by GC and cyclophosphamide, 27 cases). All patients received 6-month therapy. The GC dosage, the withdrawal and reduction dosage of GC, clinical efficacy, systemic lupus erythematosus disease activity index (SLEDAI) score, adverse reactions, and laboratory indicators were recorded.

RESULTS(1) All patients got relieved to some degree with the dosage of GC reduced. The total withdrawal and reduction dosage of GC was slightly higher in the treatment group than in the control group [(50.23 +/- 12.43) mg vs (48.76 +/- 13.61) mg, P > 0.05]. Besides, the prednisone dosage in the treatment group was lower than that in the control group, but without statistical difference (P > 0.05). The ratio of patients in need of adding prednisone for aggravating disease was 24.0%, significantly lower than that in the control group (44.44%, P < 0.05). (2) There was no statistical difference in the SLEDAI score, inflammatory indicators, liver and renal functions, blood electrolytes, blood glucose, blood and urine routines between the two groups (P > 0.05). The 24-h urinary protein count was (1.06 +/- 0.22) g/L in the treatment group, obviously lower than that in the control group (1.43 +/- 0.55 g/L, P < 0.05). (3) There was no statistical difference in the incidence rate of infection, gastrointestinal hemorrhage, psychoneuroses, Cushing's syndrome, cardiovascular anomalies, and femoral head necrosis between the two groups (P > 0.05). But the incidence of adverse reactions such as insomnia, tidal fever, spontaneous sweat, and obesity was less in the treatment group than in the control group (P < 0.05).

CONCLUSIONSUsing a drug pair of Radix Astragali and Rehmanniae Radix combined with GC in treating LN could withdraw the dosage of GC and relieve symptoms it induced. It was advantageous in reducing the dosage of GC and stabilizing patients' conditions.

Adolescent ; Adult ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Lupus Nephritis ; drug therapy ; Treatment Outcome ; Young Adult

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs, and lupus nephritis (LN) is the most common renal complication of SLE. Belimumab is a fully humanized monoclonal antibody that can reduce the number of B cells, thereby reducing the formation of autoantibodies. Belimumab can improve SLE response index and SLE disease activity score and delay the progression of LN in both adults and children and thus plays an important role in the treatment of SLE and LN. This article reviews related research reports of belimumab used in the treatment of children and adults with SLE in China and overseas and analyzes the efficacy and safety of belimumab in pediatric patients, in order to provide a reference for the clinical application of belimumab in children with SLE.
Antibodies, Monoclonal, Humanized ; Child ; Humans ; Immunosuppressive Agents ; Kidney ; Lupus Erythematosus, Systemic/drug therapy* ; Lupus Nephritis ; Treatment Outcome

OBJECTIVETo investigate retrospectively the efficacy of cyclosporine A (CsA) in the treatment of membranous lupus nephropathy (MLN).

METHODSTwenty-four patients with systemic lupus erythematosus (SLE) and biopsy-proven MLN were treated with CsA in combination with prednisone. CsA was given at a starting dosage of 5 mg x kg(-1) x d(-1) for 3 months, with a 1 mg x kg(-1) x d(-1) reduction every month and then maintained at a dosage of 2 mg x kg(-1) x d(-1). The dosage of oral prednisone differed from person to person according to levels of extra-renal activity. Clinical efficacy and adverse reactions were retrospectively analyzed. Complete remission was defined as having a urinary proteinuria level (Upr) of < 0.4 g/d, and normal serum albumin and serum creatinine (SCr) levels, without SLE activity. Partial remission was defined as having a UPr decrement > 50% of baseline value and a serum albumin value of 30 - 35 g/L, without SLE activity. No response was defined as having a Upr decrement < 50% of baseline value and > 2.0 g/d, or as a deterioration of renal function, or as having active SLE.

RESULTSOne patient could no longer undergo follow-up, and the other 23 patients were treated with CsA and followed up for 6 - 36 months (mean 16.8 +/- 8.4 months). The mean starting dosage of CsA was 4.7 +/- 0.5) mg x kg(-1) x d(-1) and the trough level of the whole blood CsA was 248 +/- 110) micro g/L. Twelve patients (52.2%) achieved complete remission, 10 patients (43.3%) achieved partial remission after CsA treatment, and one patient showed no response. At different CsA treatment timepoint, the complete remission rates were 17.4% (3rd month), 21.7% (6th month), 40% (12th month), 88.9% (18th month) and 100% (24th month) respectively. SCr elevation, when within a normal limit was not observed in most patients during early CsA administration, and at the end of the follow-up all the patients had a normal SCr. Relapse occurred in 33.3% of the patients after withdrawing CsA for 4 - 24 months. No chronic CsA renal toxicity was observed in 4 patients who had a repeat renal biopsy after CsA treatment for 6 - 24 months.

CONCLUSIONSCsA could be regarded as an effective therapy for patients with membranous lupus nephropathy, but its adverse effects, especially its nephrotoxicity, should be carefully monitored during CsA treatment.

Adolescent ; Adult ; Cyclosporine ; administration & dosage ; therapeutic use ; Female ; Glomerulonephritis, Membranous ; drug therapy ; Humans ; Lupus Nephritis ; drug therapy ; Male ; Prednisone ; administration & dosage ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo observe the therapeutic effect of total glucosides of paeony (TGP) on lupus nephritis (LN) in MRL/lpr mice.

METHODSMRL/lpr mice with lupus nephritis were randomized into model group and TGP group. The urinary protein content was detected using Coomassie brilliant blue, and the serum levels of IgG anti-double-stranded DNA (dsDNA) antibodies and antinuclear antibodies (ANA) were measured by enzyme-linked immunosorbent assay (ELISA). The changes in the renal pathology were examined microscopically, and the spleen and thymus were weighed to calculate the spleen and thymus indexes.

RESULTSAt 15 and 30 days after TGP administration, the urinary protein content in the TGP group was significantly lower than that in the model group (P<0.05). TGP treatment significantly lowered the serum levels of anti-dsDNA antibodies and ANA and the weight and index of spleen (P<0.05), resulting also in lessened renal pathology at 30 days after the administration. Compared to those before TGP treatment, the urinary protein content and the levels of anti-dsDNA antibodies and ANA decreased significantly at 15 and 30 days after TGP administration (P<0.05), while in the model group, the level of anti-dsDNA increased significantly without obvious changes in urinary protein content or ANA. At 30 days after TGP administration, the urinary protein content was significantly lowered in the TGP group as compared to that at 15 days (P<0.05), but the antibodies showed no significant changes.

CONCLUSIONTGP can reduce urinary protein content and serum levels of anti-dsDNA antibodies and ANA, and lessen renal pathology in MRL/lpr mice with lupus nephritis, suggesting its therapeutic effect on lupus nephritis.

Animals ; Antibodies, Antinuclear ; blood ; Autoantibodies ; blood ; DNA ; immunology ; Female ; Glucosides ; pharmacology ; Lupus Nephritis ; blood ; drug therapy ; urine ; Male ; Mice ; Mice, Inbred MRL lpr ; Paeonia ; chemistry ; Proteinuria ; drug therapy

Child ; Child, Preschool ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Interleukin-6 ; urine ; Male ; Nephritis ; drug therapy ; urine ; Purpura, Schoenlein-Henoch ; drug therapy ; urine ; Tablets