Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

Bello-Márquez, Diana Carolina; Nieto­-Ríos, John Fredy; Higuita, Lina María Serna; Gonzalez-Vergara, Alfonso Jose

doi:10.1590/2175-8239-jbn-2020-0050

Cited by 3 publications

(3 citation statements)

References 18 publications

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“…Bello-Marquez et al reported a case of aHUS complicated with NS, in which heterozygous CFI mutation and CFHR deletion were identified (Bello-Marquez et al 2021). This report supports the possibility that heterozygous CFI mutation and CFHR3-CFHR1 deletion seen in our case may have been jointly involved in the development of TMA.…”

Section: Accepted Manuscriptsupporting

confidence: 86%

An Infant Case of Streptococcus Pneumoniae-Associated Thrombotic Microangiopathy with Heterozygous CFI Mutation and CFHR3-CFHR1 Deletion

Matsumoto

Ikezumi

Kondoh

et al. 2022

Tohoku J. Exp. Med.

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Section: Accepted Manuscriptsupporting

confidence: 86%

An Infant Case of Streptococcus Pneumoniae-Associated Thrombotic Microangiopathy with Heterozygous CFI Mutation and CFHR3-CFHR1 Deletion

Matsumoto

Ikezumi

Kondoh

et al. 2022

Tohoku J. Exp. Med.

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“…Efficacy criteria [7]: complete remission: children with clinical symptoms disappeared after 3 months of treatment, renal function returned to normal, 24-h urine protein quantification less than 0.3 g, and blood creatinine and eGFR returned to normal or remained stable; partial remission: children with clinical symptoms disappeared after 3 months of treatment, renal function returned to normal, 24-h urine protein quantification between 0.3 and 1.0 g, and the blood creatinine and EGFR returned to normal or remained stable; no remission: children with no improvement in clinical symptoms after 3 months of treatment, kidney function still significantly abnormal or even continue to deteriorate, 24-h urine protein quantification more than 1.0 g, and blood creatinine and eGFR showed no change or were even worse than before. Total effective rate � complete remission rate + partial remission rate.…”

Section: Observed Indicatorsmentioning

confidence: 99%

Study on the Effect of Combination of Prednisone and Vitamin D in the Treatment of Primary Nephrotic Syndrome in Children

Zhou¹,

Kong

2021

Journal of Healthcare Engineering

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Objective. To study the effect of prednisone combined with vitamin D in the treatment of primary nephrotic syndrome in children. Method. 73 cases of primary nephrotic syndrome admitted to the nephrology department of our hospital were randomly selected and retrospectively analyzed. 36 cases were treated with prednisone as the control group, and 37 cases were treated with prednisone combined with vitamin D as the observation group. The efficacy was compared after 3 months of continuous treatment. Result. After 3 months of treatment, the blood calcium of the observation group was higher than that of the control group, PTH was lower than that of the control group, and 25-(OH)2D3 and 1,25-(OH)2D3 were higher than those of the control group ( P < 0.05 ). After 1, 2, and 3 months of treatment in the observation group, Scr and 24-h urine protein quantification were lower than those in the control group and eGFR was higher than that in the control group ( P < 0.05 ). CD4+ and CD4+/CD8+ were lower in the observation group than in the control group after 3 months of treatment ( P < 0.05 ). The serum sTfR and TGF-β1 levels were lower in the observation group than in the control group after 3 months of treatment ( P < 0.05 ). The total effective rate of the observation group was 83.78% after 3 months of combined treatment with prednisone and vitamin D, which was significantly higher than the total effective rate of the control group of 61.11% ( P < 0.05 ). The incidence of nausea and vomiting, heartburn, headache, dry cough, hypercalcemia, and constipation during treatment in the observation group was not statistically different from that in the control group ( P > 0.05 ). Conclusion. Combined treatment of primary nephrotic syndrome in children with prednisone and vitamin D can more significantly improve the level of clinical indicators, improve renal function and immune function, and obtain more satisfactory efficacy, without significantly affecting the safety of treatment.

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“…NS can be assigned into three types, including congenital, primary, and secondary, among which primary nephrotic syndrome (PNS) is the most common, accounting for about 90% of all children's NS [2]. According to US statistics, the annual incidence of PNS in children is (2-4)/ 100,000, and the prevalence is 16/100,000 [3,4]. Recent statistics have reported that PNS can account for about 20.0% of the total number of hospitalized children with urinary tract diseases in the same period [5].…”

Section: Introductionmentioning

confidence: 99%

Systematic Review of the Efficacy and Safety of Traditional Chinese Medicine Granules Associated with Hormone When Treating Primary Nephrotic Syndrome in Children

Zhang¹,

Chen²

2022

Contrast Media & Molecular Imaging

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Objective. To systematically evaluate the efficacy and safety of traditional Chinese medicine (TCM) granules associated with hormones when treating primary nephrotic syndrome (PNS) in children. Methods. Search online databases such as PubMed, EMBASE, ScienceDirect, Cochrane Library, China Knowledge Network Database (CNKI), China VIP Database, Wanfang Database, and China Biomedical Literature Database (CBM) to search for information on the use of hormone-related Chinese medicine granules in the treatment of children with PNS controlled trials. Retrieval time was limited to the period from the date the database was established to the present. Separately, two researchers gathered the data. Statistical software RevMan5.4 was adopted to estimate bias risk in accordance with the Cochrane Handbook 5.3 standard. Results. Finally, 7 articles were selected with a total sample size of 487 cases. The infection rate, recurrence rate, and adverse reaction rate after treatment were analyzed by meta-analysis. The infection rate and recurrence rate in the study group were notably lower, and the difference was statistically significant (P < 0.05). However, the incidence of adverse reactions exhibited not notably different (P > 0.05). The levels of albumin and blood cholesterol after treatment indicated no statistical difference between the levels (P > 0.05). Meta-analysis was performed on the time to negative urine protein and the time to edema subsidence after treatment. The urine protein negative time and edema subsidence time of the study group were shorter after treatment, but the difference exhibited not notable (P > 0.05). Meta-analysis was performed on the dosage of glucocorticoids after treatment. The dosage of glucocorticoid in the study group was notably lower, and the difference was statistically significant (P < 0.05). The levels of T lymphocytes after treatment were analyzed by meta. T lymphoid level in the study group was notably better after treatment, and the difference was statistically significant (P < 0.05). Further subgroup analysis indicated that the levels of CD3+ and CD4+ in the study group were higher after treatment (P < 0.05), and there exhibited no statistical difference in the levels of CD8+, CD4/CD8+, and CD19 (P > 0.05). Immunoglobulin levels in the study group after treatment were notably better, and the difference was statistically significant (P < 0.05). Further subgroup analysis indicated that the levels of IgA, IgM, and IgG in the study group were notably higher after treatment, and the difference was statistically significant (P < 0.05). Conclusion. Huai Qi Huang can reduce the recurrence rate of PNS children and the incidence of infection and the dosage of prednisone. A long-term application can improve the cellular and humoral immune function of children with PNS. It has high treatment safety and has no notable effect on plasma cholesterol levels, so it is suitable for clinical application.

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Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

Cited by 3 publications

References 18 publications

An Infant Case of <i>Streptococcus Pneumoniae</i>-Associated Thrombotic Microangiopathy with Heterozygous <i>CFI</i> Mutation and <i>CFHR3-CFHR1</i> Deletion

An Infant Case of <i>Streptococcus Pneumoniae</i>-Associated Thrombotic Microangiopathy with Heterozygous <i>CFI</i> Mutation and <i>CFHR3-CFHR1</i> Deletion

Study on the Effect of Combination of Prednisone and Vitamin D in the Treatment of Primary Nephrotic Syndrome in Children

Systematic Review of the Efficacy and Safety of Traditional Chinese Medicine Granules Associated with Hormone When Treating Primary Nephrotic Syndrome in Children

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