Clinical and pathological analysis of 41 children with lupus nephritis / 中华实用儿科临床杂志

Objective To analyze the correlation between clinical characteristics and pathological findings lupus nephritis (LN) in children,together with the correlation of the renal vascular lesion respectively with the glomerular lesion and tubulointerstitial lesion.Methods Forty-one cases of LN in children diagnosed by percutaneous renal biopsy from Jan.2008 to Sep.2012 in Pediatrics of the Second Xiangya Hospital of Central South University were collected.Clinical manifestations and the lab findings of the blood and urine of all the patients were analyzed,and all the frozen sections were evaluated according to the standard of ISN/RPS2003 for LN.The glomerular lesion and tubalointerstitial lesion separately were also evaluated,respectively.The wall thickening/outer diameter ratio,intima thickening/outer diameter ratio and medial thickening/outer diameter ratio of the arterioles were measured.Results The percentage of clinical manifestation with LN increased coupled with the degree of pathological damage.In the different stages of pathological damage,both the glomerular lesion and tubulointerstitial lesion were getting much more serious along with the progressiveness of pathological damage,and what's more,they had a positive correlation(r =0.959,P ＜ 0.05).The wall thickness/outer diameter ratio in all cases was greater than 0.5,in dicating the thickness of vessel wall.Conclusion Renal vascular lesion really existed and is characterized by the progressive loss of integrity of the intima and the medial thickening.

Application of stem cell transplantation in kidney diseases / 中国当代儿科杂志

This paper summarizes the current literature on the potential therapeutic role of stem cell transplantation for kidney injury and repair and focuses on the choice of types of stem cells, the method of transplantation, and the mechanisms of stem cell homing to injured renal tissues and its protective effects. The application of umbilical cord mesenchymal stem cells (UC-MSCs) shows wide prospects, but the approach and optimal dose of cell transplantation are under intensive investigation. Signals that regulate stem cell homing to injured renal tissues may be related to chemokines or factors released in the target site. Several studies have pointed out that paracrine and endocrine of stem cells are the most likely mechanism of action in the injured nephron. Many questions remain unanswered but stem cell-based therapy is a promising new strategy for acute and chronic kidney diseases.

Relationship between renal Th1/Th2 ratio and renal microvascular injury in children with Henoch-Sch-nlein purpura nephritis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To explore possible correlations between renal Th1/Th2 ratio and renal microvascular injury in children with Henoch-Sch-nlein purpura nephritis (HSPN).</p><p><b>METHODS</b>Thirty-two children with HSPN were enrolled. They were classified into four groups by renal pathology: HSPN class II (n=8), HSPN class IIIa (n=7), HSPN class IIIb (n=10) and HSPN class IV/V (n=7). Five patients undergoing nephrectomy due to trauma were used as the controls. INFγ, IL-4 and CD34 in the renal tissues were measured by immunohistochemical analysis. INFγ was used as a marker of Th1, IL-4 was used as a marker of Th2 and CD34 was used as a marker of microvessel. The renal microvessel density was evaluated according to the Weidner standard. The relationships among the local Th1/Th2 ratio, renal pathological grade, microvessel score and microvessel density were studied.</p><p><b>RESULTS</b>Immunohistochemical analysis showed a lower expression of INFγ and a higher expression of IL-4 in the HSPN groups than in the control group. The local Th1/Th2 ratio in the HSPN groups decreased and correlated significantly with the renal pathological grade. There were significant differences among four HSPN subgroups (P<0.05). Compared with the control group, the renal microvessel density in the HSPN class II and class IIIa groups increased significantly (P<0.05), but it decreased in the HSPN class IV/V group (P<0.05). The renal microvessel scores in the HSPN class IIIa, class IIIb and class IV/V groups increased significantly compared with those in the control and the HSPN classⅡ. The increased renal microvessel scores were associated with more severe renal pathological changes. A negative correlation was found between the local Th1/Th2 ratio and the microvessel density in kidneys (r=-0.921, P<0.01).</p><p><b>CONCLUSIONS</b>The decrease of Th1/Th2 ratio in kidneys might be responsible for renal microvascular injury in children with HSPN.</p>

Pathological features and clinical manifestations in 313 children with nephropathy under 6 / 中南大学学报(医学版)

OBJECTIVE@#To explore the relationship between pathological features and clinical manifestations in children with nephropathy under 6 years old.@*METHODS@#Renal biopsy by rapid percutaneous puncturation was performed on 313 children under 6 who were all diagnosed clinically as kidney diseases of 14 different kinds. The specimens were divided into 3 parts for microscope, electron microscope and immuno fluorescence examination respectively and processed by HE, PAS, PASM, and Masson staining. Immunofluorescence was used to detect the deposition of IgG, IgM, IgA, C3, C4, C1q, and Fb in the renal tissues. Additional examinations were done to detect HBs-Ag, HBeAg and HBcAg deposition in some cases with positive serum HBs-Ag. Altogether 290 of the specimens (290/313, 92.65%) were examined by electron microscope.@*RESULTS@#All the renal biopsy performances were successful. The clinical manifestations comprised of persistent haematuria (32.92%, 103/313), idiopathic nephritic syndrome (26.1%, 82/313), acute nephritic syndrome (20.14%, 63/313), Henoch Schonlein purpura nephritis (8.32%, 26/313), HBV-nephritis (4.79%, 15/313), and isolated proteinuria (2.56%, 8/313). The main pathological patterns of glomerular disease were identified as mesangial proliferation (51.75%, 162/313), IgM nephropathy (8.31%,26/313), minor and minimal change (7.99%, 25/313), IgA nephropathy (7.35%, 23/313), endocapillary proliferative glomerulonephritis (5.11%, 16/313), focus segmental glomerulosclerosis (4.47%, 14/313), thin basement membrane nephropathy (4.47%, 14/313), and membrane nephropathy (4.47%, 14/313). Alport syndrome, congenital nephrotic syndrome, and thin basement membrane nephropathy can be diagnosed by electron microscope, white IgA nephropathy, IgM nephropathy and C1q nephropathy by immunopathology.@*CONCLUSION@#Similar clinical manifestations may differ in the pathology and the clinical features of one pathological diagnosis may vary greatly. Renal biopsy is of great help to the diagnosis, treatment and the prognosis evaluation for children with nephropathy under 6. Electron microscopes also play an important role in the diagnosis of nephropathy.

Assessment of mycophenolate mofetil for treatment of frequently relapsing nephrotic syndrome in children / 中南大学学报(医学版)

OBJECTIVE@#To investigate the efficacy and adverse effect of mycophenolate mofetil (MMF) in the treatment of frequently relapsing nephrotic syndrome in children.@*METHODS@#The study population consisted of 37 children (24 simple nephrotic syndrome and 13 nephritis-type syndrome) suffering from frequently relapsing nephrotic syndrome. Patients received 20-30 mg/(kg d) of MMF in conjunction with 1 mg/(kg d) prednisone for 3-6 months.@*RESULTS@#Out of 24 patients suffered from simple nephrotic syndrome, 17 patients (70.8%) with complete relief, 4 patients (16.7%) with partial relief and 3 patients (12.5%) with non-relief, whereas out of 13 patients suffered from nephritis-type syndrome 6 patients (46.2%) with complete relief, 3 patients (23.1%) with partial relief and 4 patients (30.7%) with non-relief. Eight patients with Minimal Change Disease (MCD) achieved complete relief. Of 23 patients with Mesangial Proliferative Glomerulonephritis (MsPGN) or Membranoproliferative Glomerulonephritis (MPGN), complete relief was observed in 17 patients (73.9%), partial relief in 4 patients (17.4%) and non-relief in 2 patients.@*CONCLUSION@#These Results suggest that MMF has better efficacy against simple renal disease than against nephritis-type syndrome, and MMF may be more suitable for the treatment of frequently relapsing nephrotic syndrome characterized by proliferative lesions.

Glucocorticoid administration in steroid sensitive nephritic syndrome: a meta-analysis / 中南大学学报(医学版)

OBJECTIVE@#To evaluate the benefits and toxicities of different corticosteroid regimes in preventing relapse in children with steroid sensitive nephrotic syndrome (SSNS).@*METHODS@#MEDLINE (Jan. 1963-Mar. 2007), elsevier (Jan. 1997-Aug. 2006), OVID databank (Jan. 1993-Aug. 2006), Springer databank (Jan. 1994-March 2007), the Cochrane Controlled Trials Register (Cochrane Library, Issue Feb. 2006), Cochrane Renal Group Specialised Register (Jul. 2006), EMBASE (Jan. 1980-Mar. 2007) and CNKI (Jan. 1994-Mar. 2007) etc, were searched by the terms primary nephrotic syndrome, glucocorticoid, corticosteroid, steroid, prednisone, methylprednisolone, dexamethasone and children etc for the human clinical trials about glucocorticoid (GC) administration in primary nephrotic syndrome (PNS) (aged 3 months to 18 years), controlled or semi-controlled ones, including unpublished documents from scientific meetings and theses, and similar documents listed in the references of the above documents were also included. All the studies were evaluated strictly according to Jadad Standard, and the Meta-analysis were adopted. Review manager 4.2 software was used to analyze the data. The odds ratio was calculated for the relapse rate and side effect from the initial episode to the end of follow-up between the patients treated with corticosteroids and the controls.@*RESULTS@#Totally 12 trials with 868 subjects meeting the criteria were included in this review. A Meta-analysis of 7 trials, which compared between 2 months of prednisone and 3 months or more in the first episode, showed that longer treatment duration significantly reduced the risk of relapse at 12-24 months (RR=0.70,95% CI:0.60-0.89),without an increase of side effect. There was a negative linear relationship between the duration of treatment and risk of relapse (r2 =0.66, P=0.05).@*CONCLUSION@#(1) Children in their first episode of SSNS should be treated for at least 3 months of GC. The therapeutic effect of patients in the primary nephrotic syndrome treated with GC for 12 weeks was prior to that for 8 weeks, compared with that in the controls. It could reduce the relapse rate of half year, the longer treatment duration in the NS patients at the first relapse was, the lower relapse risk was.(2) Compared with alternative GC administration, standard GC administration can reduce the side effects; Course more than 1 year of GC administration can reduce the 2-year relapse rate. Hence in children who relapse frequently, multicentre, well-designed experiments should be adopted.

Clinicopathologic characteristics of 1,316 children with renal disease / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics of childhood renal diseases.</p><p><b>METHODS</b>A retrospective analysis of 1316 renal biopsies performed over the past 20 years was performed.</p><p><b>RESULTS</b>Of the 1316 patients, 383 (29.09% ) were diagnosed as nephrotic syndrome, 291 (22.00%) as acute nephritis syndrome, 224 (17.21%) as isolated hematuria, 209(15.87%) as purpura nephritis, and 96 (7.30% ) as hepatitis B virus-associated nephritis . Mesangial proliferation was the most common pathological change (756 cases; 57.45%), followed by IgA nephropathy (113 cases; 8.59%), endothelial capillary proliferation(112 cases; 8.51%), membranous nephropathy (66 cases; 5.02%), and various minor and minimal changes (59 cases; 4.48%). Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by electronic microscopy. IgA, IgM and C1q nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases (915 cases); secondary glomerular disease was noted in 26.14% (344 cases). Of the 915 cases of primary glomerular disease, 375 (41.0%) had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common (209/344; 60.8%).</p><p><b>CONCLUSIONS</b>Primiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.</p>

Evidence-based therapy of nephrotic edema / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the evidence-based therapy of edema in nephrotic syndrome by analyzing the literatures systematically.</p><p><b>METHODS</b>The literatures related to the treatment of nephrotic edema were retrieved from the following: Chinese Biological Medicine Database (CBM-disk), Chinese Journals Full-text Database (CNKI, 1994-2006), Chinese Technological Periodicals Database (VIP, 1989-2006), Chinese Evidence Biological Medicine/Cochrane Central Database (CEBM/CCD), Cochrane Library Database, MEDLINE (1966-2006), EMBASE (1975-2006), MEDLARS, SCI (1985-2006) and OVID by electron and craft search with the following key words: nephrotic syndrome, edema, recalcitrant edema, refractory edema or resistant nephrotic edema, and treatment, diuretic therapy or human albumin treatment. The relevant literatures on randomized controlled trials (RCT) that met the criteria were statistically analyzed by the Coorporative network software RevMan 4.2.</p><p><b>RESULTS</b>A total of 113 articles were searched (60 in Chinese and 53 in English), of which 12 were RCT. Three of the 12 articles were included for Meta analysis. Meta analysis showed that dextran-40 together with furosemide was effective for nephrotic edema. Human albumin solution could be used in nephrotic edema patients with coexistent severe hypoalbuminemia. A combination of diuretics by intravenous drip infusion was effective for diuretic-resistant nephrotic edema.</p><p><b>CONCLUSIONS</b>The treatment for nephrotic edema should be individualized. The evidence of treatment of nephrotic edema has not been fully elucidated. Further multicentre, large sample, and randomized controlled trials are needed.</p>