Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed...Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed with mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS) by biopsy and failed to respond to a 3-month treatment with prednisone (1 mg/kg·d), were randomly divided into 2 groups (TAC + TW Group and TW Group). Initially TAC + TW group took TAC 0.05mg/(kg·d) 2 h after meal at 12 h interval. The plasma TAC level was examined after 3 days and was kept at 1.5 - 4 ng·ml;meanwhile, TW was given at 60 mg/d before meal. TW group only took TW (60 mg/d). The efficacy, adverse reactions and plasma TAC levels were observed in each group. Results: 1) Totally 20 SRNS patients completed the trial, 11 of TAC + TW Group and 9 of TW Group. There is no statistical difference between the two groups in terms of age, gender, duration since onset of the disease, blood pressure, 24 h UPQ, serum albumin, creatinine, cholesterol, triglyceride, FBG, kidney pathological categories, time of taking prednisone etc.;2) Urine protein started to decrease after 1 month treatment in both of TAC + TW and TW groups. By the 12th month of treatment, TAC + TW group showed 8 cases of complete remission (72.7%), 2 cases of partial remission (18.2%) and 1 case of no improvement (9.1%), while those of TW groups were 2 (22.2%), 4 (44.5%) and 3 (33.3%), respectively;3) With treatment, the TAC + TW Group patients’ plasma protein was significantly higher than that of pretreatment stage and recovered to normal level after 6 month of treatment. However, there was no significant plasma protein increase in TW Group. No obvious changes were observed on serum creatinine level of patients of both the two groups;4) The incidence of adverse reactions was not significantly different between the two groups. Conclusion: TAC + TW reduced proteinuria of SRNS patients, increased clinical remission rate and was tolerant to SRNS patients. We conclude that TAC + TW treatment is an effective way to treat patients with SRNS.展开更多
BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).Howev...BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).However,the characteristics of nephrotic syndrome(NS)caused by CRB2 mutations have not been described.CASE SUMMARY We report a novel compound heterozygous mutation of the CRB2 gene in two siblings with SRNS.The two siblings had edema,proteinuria,hypoproteinemia and hyperlipidemia.Both their father and mother had normal phenotypes(no history of NS).Whole exon sequencing(WES)of the family showed a novel compound heterozygous mutation,c.2290(exon 8)C>T and c.3613(exon 12)G>A.Glucocorticoid therapy(methylprednisolone pulse therapy or oral prednisone)and immunosuppressive agents(tacrolimus)had no effect.During a 3-year follow-up after genetic diagnosis by WES,proteinuria persisted,but the patient was healthy.CONCLUSION CRB2 mutations related to SRNS often occur in exons 7,10,and 12.Clinical manifestations of SRNS caused by CRB2 mutations are often less severe than in other forms of SRNS.展开更多
Objective:?Although several NPHS2 gene mutations and polymorphisms were described and?associated with clinical manifestation of steroid-resistant nephrotic syndrome (SRNS),?the?occurrence of these genetic abnormalitie...Objective:?Although several NPHS2 gene mutations and polymorphisms were described and?associated with clinical manifestation of steroid-resistant nephrotic syndrome (SRNS),?the?occurrence of these genetic abnormalities or variants appeared?to be influenced by race and ethnic group. We have investigated?probable mutations and variants in NPHS2 gene involved in SRNS and their association with clinical manifestations. Methods: We examined 28 children?with primary SRNS?whovisited?the pediatric nephrology division of 10 teaching hospitals in Indonesia. Molecular genetic studies of the NPHS2 gene were?conducted through screenings?for the exon 1, exon 2, and exon 8. The mutational analysis of NPHS2 was performed by DNA sequencing.?Fisher’s Exact Test was used to determine?the?correlation?between?NPHS2 polymorphisms and clinical manifestations.Results:?Seven?females (25%) and 21 males (75%)?participated in the study.?The mean age of the subjects with 95% CI is: 7.6 (6.1 - 9.0) years while the mean age at onset of disease with 95% CI is: 5.4 (3.9 - 7.0) years. Sixteen patients (57.14%) were younger than 6 years at the onset of disease. Seventeen (60.7%) subjects had normal eGFR, while 11 (39.3%) had chronic renal insufficiency. The mean eGFR of the subjects with 95% CI is: 111.4 (87.7 - 135.1) ml/min/1.73 m2. The mean systolic blood pressure with 95% CI is: 117.0 (108.9 - 125.1) mmHg and the mean diastolic blood pressure with 95% CI is: 77.0 (70.3 - 83.7) mmHg.?We identified 6 NPHS2polymorphisms,?i.e.?g.-52G>T, c.101A>G, g.-117C>T, c.288C>T, c.954C>T, and c.1038A>G and no mutation?was found. There was?no correlation?between?NPHS2 polymorphisms and clinical manifestations (p > 0.05). Conclusion: The?results demonstrate no mutation of NPHS2 gene, and the 6 NPHS2 gene polymorphisms that were identified have no correlation with the clinical manifestation in Indonesian children with SRNS.展开更多
BACKGROUND Nephritic syndrome(NiS)is a major indicator of serious renal diseases necessitating kidney biopsies for histopathological evaluations,but due to the lack of comprehensive reviews in the literature,the curre...BACKGROUND Nephritic syndrome(NiS)is a major indicator of serious renal diseases necessitating kidney biopsies for histopathological evaluations,but due to the lack of comprehensive reviews in the literature,the current understanding of the syndrome and its significance is limited.AIM To collect all the evidence retrievable from the literature on the diagnoses made on the renal biopsies performed for NiS as the indication to the procedure.METHODS A literature search was conducted to find studies reporting final diagnoses on renal biopsies in NiS patients.Data were pooled and analyzed with stratifications on age and regions.Meta-analyzes were performed using Stata v.9.RESULTS Overall,26414 NiS patients from the total number of 96738 kidney biopsy diagnoses reported by 47 studies from 23 countries from all continents(except sub-Saharan Africa)were found and analyzed.NiS was the indication for renal biopsy in 21%of the patient populations across the reviewed studies.Immunoglobulin A(IgA)nephropathy was the single most frequent diagnosis in these patients(approximately 38%)followed by lupus nephritis(approximately 8%)and Henoch Schönlein purpura(approximately 7%).IgA nephropathy was the most frequent diagnosis reported for the NiS patients from the East Asia,comprising half of all the cases,and least prevalent in South Asia.Considering the age subgroups,adult(vs pediatric or elderly)patients were by far the most likely age group to be diagnosed with the IgA nephropathy.A myriad of such regional and age disparities have been found and reported.CONCLUSION As the indication for renal biopsy,NiS represents a very distinctive epidemiology of final renal disease diagnoses compared to the other major syndromes.展开更多
Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conduct...Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.展开更多
In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,dia...In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.展开更多
Background Tacrolimus,a calcineurin inhibitor,is recommended by the recent guidelines from the Kidney Disease Improving Global Outcomes Group as the first-line treatment for steroid-resistant nephrotic syndrome(SRNS),...Background Tacrolimus,a calcineurin inhibitor,is recommended by the recent guidelines from the Kidney Disease Improving Global Outcomes Group as the first-line treatment for steroid-resistant nephrotic syndrome(SRNS),but its clinical application in China is still limited.We investigated the efficacy and safety of tacrolimus combined with low-dose corticosteroids in a population of Chinese children with SRNS.Methods In this prospective non-randomized,non-controlled study,Chinese children with SRNS who failed the previous full-dose prednisone treatment were given tacrolimus(0.1 mg/kg/day)and low-dose prednisone(0.25-0.50 mg/kg/day).We compared the overall remission rate(ORR)and adverse events in the follow-up period with this therapeutic regimen.Results A total of 76 children were enrolled into the study with an average follow-up period of 18±6 months(maximum 36 months).ORR achieved by the first,third,and sixth months was 94.7%,94.7%,and 96.0%,respectively.All patients who attained an initial tacrolimus trough concentration(FK506C0)>6 ng/mL(60.3%)achieved remission.The relative risk of relapse at FK506C0<3 ng/mL compared to 3-6 ng/mL,6-9 ng/mL,and 9-12 ng/mL was 2.3,3.2,and 16.9,respectively.During the follow-up period,adverse effects that had been previously reported were rare.Conclusions Combination of tacrolimus and low-dose prednisone was safe and effective for the treatment of children with SRNS,with high remission rates observed as early as the first month.Relapses were infrequent,but tended to increase significantly with decreases in FK506C0.展开更多
Acute post-streptococcal glomerulonephritis(APSGN)is the major cause of acute glomerulonephritis among children,especially in low-and middle-income countries.APSGN commonly occurs following pharyngitis due to the acti...Acute post-streptococcal glomerulonephritis(APSGN)is the major cause of acute glomerulonephritis among children,especially in low-and middle-income countries.APSGN commonly occurs following pharyngitis due to the activation of antibodies and complements proteins against streptococcal antigens through the immune-complex-mediated mechanism.APSGN can be presented as acute nep-hritic syndrome,nephrotic syndrome,and rapidly progressive glomerulonephritis,or it may be subclinical.The management of APSGN is mainly supportive in nature with fluid restriction,anti-hypertensives,diuretics,and renal replacement therapy with dialysis,when necessary,as the disease is self-limiting.Congestive heart failure,pulmonary edema,and severe hypertension-induced encephalopathy might occur during the acute phase of APSGN due to hypervolemia.APSGN generally has a favorable prognosis with only a small percentage of patients with persistent urinary abnormalities,persistent hypertension,and chronic kidney disease after the acute episode of APSGN.Decreased complement levels,increased C-reactive protein,and hypoalbuminemia are associated with disease severity.Crescent formations on renal biopsy and renal insufficiency on presentation may be the predictors of disease severity and poor outcomes in APSGN in children.展开更多
Objective:To understand the psychological characteristics of nephrotic syndrome.Methods:The personality characteristics,intelligence and social adaptive behavior(SAB) of 67 moderate to severe nephrotic syndrome aged 7...Objective:To understand the psychological characteristics of nephrotic syndrome.Methods:The personality characteristics,intelligence and social adaptive behavior(SAB) of 67 moderate to severe nephrotic syndrome aged 7～14 years were measured by means of EPQ,CBCL,CRT and SAB scales.Results:The EPQ-N score of nephritic syndrome group was higher than that of control group( P <0.001).The occurrence rate of behavior problem was 31%.The abnormal behavior in boys was assault,while in girls was social flinch.The mean IQ in nephritic syndrome group was 105±13 and there was no significant difference between the two groups( P >0.05).The nephritic group got a significantly lower social/self direction factor score than the control group.Conclusion:The results showed that nephritic syndrome possessed personality characteristics of neuroticism and obvious behavior problems;and their social adaptive behavior,particularly social/self-direction factor,was significantly lower than that of normal children.展开更多
文摘Objective: To observe the clinical efficacy and safety of low dose tacrolimus (TAC) combined with tripterygium (TW) in treatment of steroid resistant nephritic syndrome (SRNS). Method: The patients, who were diagnosed with mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS) by biopsy and failed to respond to a 3-month treatment with prednisone (1 mg/kg·d), were randomly divided into 2 groups (TAC + TW Group and TW Group). Initially TAC + TW group took TAC 0.05mg/(kg·d) 2 h after meal at 12 h interval. The plasma TAC level was examined after 3 days and was kept at 1.5 - 4 ng·ml;meanwhile, TW was given at 60 mg/d before meal. TW group only took TW (60 mg/d). The efficacy, adverse reactions and plasma TAC levels were observed in each group. Results: 1) Totally 20 SRNS patients completed the trial, 11 of TAC + TW Group and 9 of TW Group. There is no statistical difference between the two groups in terms of age, gender, duration since onset of the disease, blood pressure, 24 h UPQ, serum albumin, creatinine, cholesterol, triglyceride, FBG, kidney pathological categories, time of taking prednisone etc.;2) Urine protein started to decrease after 1 month treatment in both of TAC + TW and TW groups. By the 12th month of treatment, TAC + TW group showed 8 cases of complete remission (72.7%), 2 cases of partial remission (18.2%) and 1 case of no improvement (9.1%), while those of TW groups were 2 (22.2%), 4 (44.5%) and 3 (33.3%), respectively;3) With treatment, the TAC + TW Group patients’ plasma protein was significantly higher than that of pretreatment stage and recovered to normal level after 6 month of treatment. However, there was no significant plasma protein increase in TW Group. No obvious changes were observed on serum creatinine level of patients of both the two groups;4) The incidence of adverse reactions was not significantly different between the two groups. Conclusion: TAC + TW reduced proteinuria of SRNS patients, increased clinical remission rate and was tolerant to SRNS patients. We conclude that TAC + TW treatment is an effective way to treat patients with SRNS.
文摘BACKGROUND Crumbs homolog 2(CRB2)is a recently discovered gene that is closely related to the maintenance of normal polarity in podocytes;mutations can directly lead to steroid-resistant nephrotic syndrome(SRNS).However,the characteristics of nephrotic syndrome(NS)caused by CRB2 mutations have not been described.CASE SUMMARY We report a novel compound heterozygous mutation of the CRB2 gene in two siblings with SRNS.The two siblings had edema,proteinuria,hypoproteinemia and hyperlipidemia.Both their father and mother had normal phenotypes(no history of NS).Whole exon sequencing(WES)of the family showed a novel compound heterozygous mutation,c.2290(exon 8)C>T and c.3613(exon 12)G>A.Glucocorticoid therapy(methylprednisolone pulse therapy or oral prednisone)and immunosuppressive agents(tacrolimus)had no effect.During a 3-year follow-up after genetic diagnosis by WES,proteinuria persisted,but the patient was healthy.CONCLUSION CRB2 mutations related to SRNS often occur in exons 7,10,and 12.Clinical manifestations of SRNS caused by CRB2 mutations are often less severe than in other forms of SRNS.
文摘Objective:?Although several NPHS2 gene mutations and polymorphisms were described and?associated with clinical manifestation of steroid-resistant nephrotic syndrome (SRNS),?the?occurrence of these genetic abnormalities or variants appeared?to be influenced by race and ethnic group. We have investigated?probable mutations and variants in NPHS2 gene involved in SRNS and their association with clinical manifestations. Methods: We examined 28 children?with primary SRNS?whovisited?the pediatric nephrology division of 10 teaching hospitals in Indonesia. Molecular genetic studies of the NPHS2 gene were?conducted through screenings?for the exon 1, exon 2, and exon 8. The mutational analysis of NPHS2 was performed by DNA sequencing.?Fisher’s Exact Test was used to determine?the?correlation?between?NPHS2 polymorphisms and clinical manifestations.Results:?Seven?females (25%) and 21 males (75%)?participated in the study.?The mean age of the subjects with 95% CI is: 7.6 (6.1 - 9.0) years while the mean age at onset of disease with 95% CI is: 5.4 (3.9 - 7.0) years. Sixteen patients (57.14%) were younger than 6 years at the onset of disease. Seventeen (60.7%) subjects had normal eGFR, while 11 (39.3%) had chronic renal insufficiency. The mean eGFR of the subjects with 95% CI is: 111.4 (87.7 - 135.1) ml/min/1.73 m2. The mean systolic blood pressure with 95% CI is: 117.0 (108.9 - 125.1) mmHg and the mean diastolic blood pressure with 95% CI is: 77.0 (70.3 - 83.7) mmHg.?We identified 6 NPHS2polymorphisms,?i.e.?g.-52G>T, c.101A>G, g.-117C>T, c.288C>T, c.954C>T, and c.1038A>G and no mutation?was found. There was?no correlation?between?NPHS2 polymorphisms and clinical manifestations (p > 0.05). Conclusion: The?results demonstrate no mutation of NPHS2 gene, and the 6 NPHS2 gene polymorphisms that were identified have no correlation with the clinical manifestation in Indonesian children with SRNS.
文摘BACKGROUND Nephritic syndrome(NiS)is a major indicator of serious renal diseases necessitating kidney biopsies for histopathological evaluations,but due to the lack of comprehensive reviews in the literature,the current understanding of the syndrome and its significance is limited.AIM To collect all the evidence retrievable from the literature on the diagnoses made on the renal biopsies performed for NiS as the indication to the procedure.METHODS A literature search was conducted to find studies reporting final diagnoses on renal biopsies in NiS patients.Data were pooled and analyzed with stratifications on age and regions.Meta-analyzes were performed using Stata v.9.RESULTS Overall,26414 NiS patients from the total number of 96738 kidney biopsy diagnoses reported by 47 studies from 23 countries from all continents(except sub-Saharan Africa)were found and analyzed.NiS was the indication for renal biopsy in 21%of the patient populations across the reviewed studies.Immunoglobulin A(IgA)nephropathy was the single most frequent diagnosis in these patients(approximately 38%)followed by lupus nephritis(approximately 8%)and Henoch Schönlein purpura(approximately 7%).IgA nephropathy was the most frequent diagnosis reported for the NiS patients from the East Asia,comprising half of all the cases,and least prevalent in South Asia.Considering the age subgroups,adult(vs pediatric or elderly)patients were by far the most likely age group to be diagnosed with the IgA nephropathy.A myriad of such regional and age disparities have been found and reported.CONCLUSION As the indication for renal biopsy,NiS represents a very distinctive epidemiology of final renal disease diagnoses compared to the other major syndromes.
文摘Introduction: There is currently little information in the literature on the spectrum of histopathologic patterns in children presenting with idiopathic steroid-resistant nephrotic syndrome (iSRNS) in Iran. We conducted to compare the histopathologic distribution of different subtypes’ glomerular morphologic patterns in iSRNS and the clinical and biochemical parameters at the time of diagnosis and outcome of patients after immunosuppressive therapy. Material and Methods: This cross sectional study was done in two hundred children, aged 1 - 15 years, who were diagnosed for iSRNS and no response to 4 weeks of standard prednisone therapy (60 mg/m<sup>2</sup>/day) referred to nephropathology Department of Emam Reza hospital between 2005 and 2013. Demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy reports. We discussed histopathologic diagnosis and outcome of iSRNS after initial therapy in patients separately. This study investigated prognostic effects of histopathologic pattern on outcome of iSRNS. Results: The study included 200 children with iSRNS: 141 (70.5%) were males and 59 (29.5%) females, with male-to-female ratio of 2.4:1. The mean age was 7.23 ± 4.37 years (range: 1 - 15 years). Upon pathologic investigation of iSRNS cases, focal segmental glomerulosclerosis (NOS subtype) was the first, with a highest prevalence at a rate of 102/200 (51%) and MGN was the last, at a rate of 7/200 (3.5%). Children with iSRNS secondary to MCD are more likely to achieve remission and have better long term prognostic value (P 0.00). Focal segmental glomerulosclerosis (FSGS) (Tip and Collapse subtypes) is more likely to have worse outcome in response to immunosuppressive therapy (P 0.04). Conclusions: This study defines the true spectrum of clinicohistopathology patterns underlying iSRNS in children in Northwest of Iran. Also this study shows that the response to cyclosporine can be correlated with the underlying histopathology patterns which have been earned by adequate renal biopsy.
文摘In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.
文摘Background Tacrolimus,a calcineurin inhibitor,is recommended by the recent guidelines from the Kidney Disease Improving Global Outcomes Group as the first-line treatment for steroid-resistant nephrotic syndrome(SRNS),but its clinical application in China is still limited.We investigated the efficacy and safety of tacrolimus combined with low-dose corticosteroids in a population of Chinese children with SRNS.Methods In this prospective non-randomized,non-controlled study,Chinese children with SRNS who failed the previous full-dose prednisone treatment were given tacrolimus(0.1 mg/kg/day)and low-dose prednisone(0.25-0.50 mg/kg/day).We compared the overall remission rate(ORR)and adverse events in the follow-up period with this therapeutic regimen.Results A total of 76 children were enrolled into the study with an average follow-up period of 18±6 months(maximum 36 months).ORR achieved by the first,third,and sixth months was 94.7%,94.7%,and 96.0%,respectively.All patients who attained an initial tacrolimus trough concentration(FK506C0)>6 ng/mL(60.3%)achieved remission.The relative risk of relapse at FK506C0<3 ng/mL compared to 3-6 ng/mL,6-9 ng/mL,and 9-12 ng/mL was 2.3,3.2,and 16.9,respectively.During the follow-up period,adverse effects that had been previously reported were rare.Conclusions Combination of tacrolimus and low-dose prednisone was safe and effective for the treatment of children with SRNS,with high remission rates observed as early as the first month.Relapses were infrequent,but tended to increase significantly with decreases in FK506C0.
文摘Acute post-streptococcal glomerulonephritis(APSGN)is the major cause of acute glomerulonephritis among children,especially in low-and middle-income countries.APSGN commonly occurs following pharyngitis due to the activation of antibodies and complements proteins against streptococcal antigens through the immune-complex-mediated mechanism.APSGN can be presented as acute nep-hritic syndrome,nephrotic syndrome,and rapidly progressive glomerulonephritis,or it may be subclinical.The management of APSGN is mainly supportive in nature with fluid restriction,anti-hypertensives,diuretics,and renal replacement therapy with dialysis,when necessary,as the disease is self-limiting.Congestive heart failure,pulmonary edema,and severe hypertension-induced encephalopathy might occur during the acute phase of APSGN due to hypervolemia.APSGN generally has a favorable prognosis with only a small percentage of patients with persistent urinary abnormalities,persistent hypertension,and chronic kidney disease after the acute episode of APSGN.Decreased complement levels,increased C-reactive protein,and hypoalbuminemia are associated with disease severity.Crescent formations on renal biopsy and renal insufficiency on presentation may be the predictors of disease severity and poor outcomes in APSGN in children.
文摘Objective:To understand the psychological characteristics of nephrotic syndrome.Methods:The personality characteristics,intelligence and social adaptive behavior(SAB) of 67 moderate to severe nephrotic syndrome aged 7～14 years were measured by means of EPQ,CBCL,CRT and SAB scales.Results:The EPQ-N score of nephritic syndrome group was higher than that of control group( P <0.001).The occurrence rate of behavior problem was 31%.The abnormal behavior in boys was assault,while in girls was social flinch.The mean IQ in nephritic syndrome group was 105±13 and there was no significant difference between the two groups( P >0.05).The nephritic group got a significantly lower social/self direction factor score than the control group.Conclusion:The results showed that nephritic syndrome possessed personality characteristics of neuroticism and obvious behavior problems;and their social adaptive behavior,particularly social/self-direction factor,was significantly lower than that of normal children.
