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1.
Medicine (Baltimore). 2018 Sep;97(38):e12291. doi: 10.1097/MD.0000000000012291.

Castleman disease mimicking systemic lupus erythematosus: A case report.

Author information

1
Key Laboratory of Rheumatology and Clinical Immunology, Department of Rheumatology and Clinical Immunology.
2
Department of Gastroenterology.
3
Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Abstract

RATIONALE:

Castleman disease (CD) is a nonclonal lymphoproliferative disorder sometimes manifested systemic inflammatory symptoms. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized with multi-system involvement as well as broad spectrum of serum autoantibodies. When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred.

PATIENT CONCERNS:

A 46-year-old man suffered from chronic fever, nephrotic syndrome, acute kidney injury, anemia, thrombocytopenia and serositis, as well as hypocomplementemia and negative anti-nuclear antibody.

DIAGNOSES:

Meeting the classification criteria for SLE, the patient was diagnosed as SLE at first. The renal biopsy showed that he had endocapillary proliferative glomerulonephritis with negative immunofluorescence. Finally, he was diagnosed with CD after lymph nodes biopsy.

INTERVENTIONS:

The patient was treated with oral prednisone (50 mg daily) but got poor response. After being proved to have CD, he was treated with CHOP chemotherapy.

OUTCOMES:

His condition was controlled by CHOP chemotherapy. After six course of chemotherapy, the proteinuria disappeared.

LESSONS:

If patients, even qualified by classification criteria of SLE, had negative autoantibody or unsatisfied response to the standard treatment, the original diagnosis should be suspected. The biopsy may be help to identify the final criminals, such as CD.

PMID:
30235674
PMCID:
PMC6160051
DOI:
10.1097/MD.0000000000012291
[Indexed for MEDLINE]
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2.
BMC Ophthalmol. 2018 Sep 3;18(1):234. doi: 10.1186/s12886-018-0883-2.

Disseminated Nocardiosis with subretinal abscess in a patient with nephrotic syndrome-a case report.

Author information

1
Department of Ophthalmology, The 4th People's Hospital of Shenyang, Shenyang, Liaoning, People's Republic of China.
2
Departments of Gastroenterology, The First Affiliated Hospital of China Medical University, Shenyang, Liaoning, People's Republic of China. zgydxh_sj@163.com.

Abstract

BACKGROUND:

Nocardia infection is uncommon in clinical practice, with most cases occuring as the result of opportunistic infection in immunocompromsed patients. Here, we report a case of disseminated nocardiosis with subretinal abscess in a patient with nephrotic syndrome, and whom is receiving immunosuppressive therapy.

CASE PRESENTATION:

A 58-year-old male presented with decreased vision in his left eye, without redness or floaters, which had persisted for three days. The patient had previously been diagnosed with membranous nephropathy, and as such, had received systemic corticosteroid therapy for four months. Further, the patient had developed pneumonia three weeks prior to this presentation. The ocular lesion appeared as a creamy-white subretinal abscess, with overlying retinal hemorrhages. Subsequent administration of three intravitreal injections of vancomycin and ceftazidime ultimately led to eradication of the intraocular infection, however, two months later, the patient developed a brain abcess. Pathogens isolated from the blood were subsequently identified as Nocardia. The patient was successfully treated via systemic administration of imipenem and trimethoprim-sulfamethoxazole.

CONCLUSIONS:

Clinicians should be aware of the possibility of Nocardia infections within all immunocompromised patients, as well as the tendency of this infection to disseminate--particularly in the brain. The early detection of Nocardia infections and prolonged treatment of the proper antibiotics may significantly improve the prognosis of this life-threatening infection.

KEYWORDS:

Endogenous endophthalmitis; Immunosuppression; Nephrotic syndrome; Nocardiosis; Subretinal abscess

PMID:
30176830
PMCID:
PMC6122714
DOI:
10.1186/s12886-018-0883-2
[Indexed for MEDLINE]
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3.
Indian Pediatr. 2018 Jul 15;55(7):605-607.

Favorable Renal Outcome of Japanese Children with Severe IgA Nephropathy With Nephrotic Syndrome.

Author information

1
Division of Nephrology, Saitama Children's Medical Center, Saitama-city, Japan. Correspondence to: Dr Shuichira Fujinaga, Division of Nephrology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama-city Saitama 330-8777, Japan. f_shuich@d2.dion.ne.jp.
2
Division of Nephrology, Saitama Children's Medical Center, Saitama-city, Japan.

Abstract

BACKGROUND:

Nephrotic syndrome is a rare but severe feature of IgA nephropathy.

CASE CHARACTERISTICS:

Nine Japanese children with severe IgA nephropathy with nephrotic syndrome.

INTERVENTION:

All received low-dose intravenous methylprednisolone (IVMP) within five weeks after the disease onset. Eight out of nine patients achieved resolution of proteinuria without severe adverse events.

MESSAGE:

Early low-dose intravenous methylprednisolone may be safe and effective for children with severe IgA nephropathy with nephrotic syndrome.

PMID:
30129546
[Indexed for MEDLINE]
4.
Medicine (Baltimore). 2018 Aug;97(32):e11608. doi: 10.1097/MD.0000000000011608.

Clinicopathological features of atypical membranous nephropathy with unknown etiology in adult Chinese patients.

Author information

1
Department of Nephrology, Peking University People's Hospital, Beijing, China.

Abstract

Membranous nephropathy is typically classified as idiopathic and secondary, but nowadays the number of atypical membranous nephropathy (aMN) is increasing, many of which cannot determine its etiology in China. In this study, we compared the clinical and pathological characteristics of idiopathic membranous nephropathy (iMN) with aMN with unknown etiology from a single center in China.We retrospectively reviewed the clinical data of 577 patients with iMN and aMN at Peking University People's Hospital from January 2006 to December 2015 over a 10-year period, and analyzed their clinical and pathological characteristics. The level of serum phospholipase A2 receptors (PLA2R) antibody was detected in 106 iMN and 162 aMN patients.There were 278 iMN patients and 299 aMN patients who were included into this study in 3210 cases of renal biopsy during a 10-year period in our hospital. The average age of patients with iMN was significantly older than those with aMN (54.77 ± 13.01 vs 47.13 ± 16.16, P < .001). Around 75 patients (27%) were smokers in iMN patients, and 111 patients (37.1%) in aMN patients (P = .009). The mainly clinical manifestation of these 2 groups was nephrotic syndrome (61.5% in iMN group vs 58.4% in aMN group), but there were more patients accompanied with nephritis syndrome in aMN group than iMN group (17.1% vs 6.1%, P < .001). The immunofluorescence of renal biopsy showed "full house" in aMN group; and IgG subclass of the glomeruli demonstrated IgG4 (90.4%) was commonest in iMN group, but IgG1 (94.6%) in aMN group. 51 (48.1%) patients with iMN were detected positive PLA2R antibody in their serum, and 93 (57.4%) in aMN patients (P = .168). The patients with positive PLA2R antibody had higher positive rate of microscopic hematuria and urinary protein, lower albumin.The aMN patients are younger, higher smoking rate, its main clinical manifestation is nephrotic syndrome, but more of them accompanied with nephritis syndrome than those in iMN patients. Serum PLA2R antibody could not distinguish aMN from iMN. aMN could be a special glomerular disease in China, and need a further research on a larger scale.

PMID:
30095619
PMCID:
PMC6133607
DOI:
10.1097/MD.0000000000011608
[Indexed for MEDLINE]
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5.
Medicine (Baltimore). 2018 Jul;97(29):e11598. doi: 10.1097/MD.0000000000011598.

Adjuvant treatment with Yupingfeng formula for primary nephrotic syndrome in children: A PRISMA systematic review and meta-analysis of randomized controlled trials.

Author information

1
Department of Pediatrics, Peking University First Hospital, Beijing, China.

Abstract

BACKGROUND:

Yupingfeng formula (YPFF) has been prescribed as adjuvant treatment for pediatric patients with primary nephrotic syndrome (PNS) in China for years. However, the efficacy and adverse effects of these formulations are controversial. A systematic review and meta-analysis of randomized controlled trials (RCTs) were performed to evaluate the benefits and harms of YPFF in treating PNS in children.

METHODS:

The MEDLINE, EMBASE, Cochrane Library, CNKI, VIP, WanFang, and CBM databases were searched for RCTs comparing therapies with and without YPFF for PNS from inception to May 13, 2017. Relative risk (RR) and 95% confidence intervals (CI) were expressed for dichotomous outcomes, and weighted mean difference (WMD) with 95% CI for continuous outcomes. Cochrane collaboration tool was used to evaluate the risk of bias of methodologies.

RESULTS:

Eight studies with 538 participants were identified. Treatment with YPFF significantly increased serum levels of IgA (WMD, 0.48, 95% CI, 0.40-0.56, P < .001), IgG (WMD, 3.36, 95% CI, 2.61-4.12, P < .001), CD4 T-lymphocytes (WMD, 3.35, 95% CI, 2.26-4.43, P < .001), but decreased the level of CD8 T-lymphocytes (WMD, -3.38, 95% CI -5.48 to -1.28, P = .002). YPFF also increased the rates of complete remission (RR: 1.35, 95% CI, 1.09-1.67, P = .005), and decreased the rates of relapse (RR: 0.57, 95% CI, 0.45-0.71, P < .001), and infection (RR: 0.72, 95% CI 0.62-0.83, P < .001). There was no significant difference in the level of IgM between the groups (WMD, 0.12, 95% CI -0.11-0.35, P = .322).

CONCLUSIONS:

YPFF could improve total remission rate and decrease the frequency of relapse and infection rate. The beneficial influence of YPFF may be associated with its immunomodulatory effects. More high-quality studies with larger sample sizes are needed to further identify its efficacy and safety.

PMID:
30024564
PMCID:
PMC6086467
DOI:
10.1097/MD.0000000000011598
[Indexed for MEDLINE]
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6.
Medicine (Baltimore). 2018 Jul;97(29):e11495. doi: 10.1097/MD.0000000000011495.

Nephrotic syndrome with acute pulmonary embolism in young adults: Two case reports.

Author information

1
Department of Cardiology, The First Hospital of Jilin University, Changchun, China.

Abstract

INTRODUCTION:

Pulmonary embolism (PE) is often misdiagnosed, or the diagnosis is delayed because of its diverse clinical manifestations, it may even remain asymptomatic until sudden death. Most risk factors are not associated with young people, and there is a paucity of literature regarding PE in children and young adults.

CASE PRESENTATION:

Patient 1 who died was diagnosed with nephrotic syndrome more than 10 years before. He presented to a clinic with gradually worsening dyspnea, which was initially misdiagnosed as myocarditis. Patient 2 presented with sudden shortness of breath after treatment for nephrotic syndrome. His PE was quickly diagnosed, allowing prompt initiation of anticoagulant therapy. At follow-up 30 days after hospital discharge, his symptoms had disappeared, and his abnormal laboratory results had returned to almost normal.

CONCLUSION:

The diagnosis and treatment of the above 2 patients suggest that the possible occurrence of PE in a young person with nephrotic syndrome should not be ignored. The early diagnosis and delayed diagnosis will have different clinical outcomes.

PMID:
30024529
PMCID:
PMC6086462
DOI:
10.1097/MD.0000000000011495
[Indexed for MEDLINE]
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7.
Niger J Clin Pract. 2018 Jul;21(7):945-948. doi: 10.4103/njcp.njcp_422_17.

Cerebral arterial thrombosis in a child with nephrotic syndrome.

Author information

1
Department of Pediatric Nephrology, Firat University School of Medicine, Elazig, Turkey.
2
Department of Pediatric Neurology, Firat University School of Medicine, Elazig, Turkey.
3
Department of Pediatrics, Firat University School of Medicine, Elazig, Turkey.

Abstract

Nephrotic syndrome (NS) in childhood may be associated with thromboembolic complications, mainly in venous origin. However, arterial thrombosis may also be seen as a very rare and life-threatening complication. Herein, we described a case of steroid-resistant NS who did not respond to full-dose steroid treatment for 8 weeks and was complicated by neurological findings. The renal biopsy was consistent with focal segmental glomerulosclerosis. His cerebral magnetic resonance angiography showed the sudden termination of M3 branch of the left middle cerebral artery which corresponded with subacute infarction in the left frontoparietotemporal area. Thrombosis panel yielded the results of hyperhomocysteinemia (46.1 μmol/L, range: 5-15 μmol/L) and heterozygous methylene tetrahydrofolate reductase mutation (C677T, A1298C). After that, the patient was given medical therapy including anticoagulant treatment. Improvement in the neurological outcome was determined on the 1st month of follow-up examinations.

KEYWORDS:

Arterial thrombosis; cerebral infarction; children; nephrotic syndrome; treatment

PMID:
29984731
DOI:
10.4103/njcp.njcp_422_17
[Indexed for MEDLINE]
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8.
Medicine (Baltimore). 2018 Jul;97(27):e11282. doi: 10.1097/MD.0000000000011282.

A PRISMA-compliant systematic review and network meta-analysis on the efficacy between different regimens based on Tripterygium wilfordii Hook F in patients with primary nephrotic syndrome.

Author information

1
Gansu University of Traditional Chinese Medicine, Lanzhou, China.

Abstract

BACKGROUND:

The present study aims to comprehensively determine the efficacy of different therapy regimens based on Tripterygium wilfordii Hook F (TwHF) for patients with primary nephrotic syndrome (PNS) using network meta-analysis method.

METHODS:

Seven electronic databases were searched to identify randomized controlled trials (RCTs) that compared the differences between different therapy regimens based on TwHF for patients with PNS. The risk of bias in included RCTs was evaluated according to the Cochrane Handbook version 5.2.0. Network meta-analysis was performed to compare different regimens. Primary outcomes were complete remission rate and total remission rate. The secondary outcomes were hr urinary protein excretion, serum albumin, serum creatinine, and urea nitrogen. Data analysis was performed using R software.

RESULTS:

A total of 40 studies involving 2846 patients with PNS were included. Compared with prednisone, the improvement in total remission rate and complete remission rate was associated with TwHF alone (odds ratio [OR] = 4.80, 95% credible intervals [CrI]: 2.20-10.00; OR = 6.30, 95% CrI: 2.90-13.00, respectively), TwHF+prednisone (OR = 2.10, 95% CrI: 1.30-3.50; OR = 2.40, 95% CrI: 1.50-3.80, respectively), TwHF+CPA (OR = 12.00, 95% CrI: 1.10-150.00; OR = 16.00, 95% CrI: 1.60-170.00, respectively), and TwHF+Cyclosporine A (OR = 28.00, 95% CrI: 3.20-250.00; OR = 35.00, 95% CrI: 4.50-270.00, respectively). Compared with TwHF alone, TwHF+prednisone showed less benefit in improving total remission rate and complete remission rate (OR = 0.44, 95% CrI: 0.21-0.91; OR = 0.38, 95% CrI: 0.19-0.77, respectively). TwHF alone, TwHF+prednisone could significantly reduce hr urinary protein excretion (MD = -0.69, 95% CrI: -1.30 to -0.14; MD = -1.00, 95% CrI: -1.90 to -0.14, respectively) and increase serum albumin (MD = 5.90, 95% CrI: 2.50-9.30; MD = 3.40, 95% CrI: 1.30-5.50, respectively) when compared to prednisone alone. TwHF alone showed significant reduction in serum creatinine when compared to CPA (MD = -19.00, 95% CrI: -37.00 to -0.56).

CONCLUSIONS:

TwHF alone, the addition TwHF to prednisone showed more benefit in improving total and complete remission rate, hr urinary protein excretion, serum albumin, and serum creatinine.

PMID:
29979395
PMCID:
PMC6076150
DOI:
10.1097/MD.0000000000011282
[Indexed for MEDLINE]
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9.
Afr Health Sci. 2017 Dec;17(4):1130-1136. doi: 10.4314/ahs.v17i4.22.

Idiopathic nephrotic syndrome in South African children.

Author information

1
Division of Paediatric Nephrology, Department of Paediatrics and Child Health, Charlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.
2
Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
3
Department of Paediatrics, Faculty of Clinical Sciences, College of Health Sciences, Bayero University, Kano, Nigeria.

Abstract

Background:

Different histo-pathological types and treatment response patterns of Idiopathic nephrotic syndrome (INS) have been associated with differences in ethnicity and geographical location.

Objective:

To provide an update on the steroid response and renal histo-pathological pattern in children treated for INS.

Method:

Medical records of children with INS treated at the Charlotte Maxeke Johannesburg Academic Hospital were reviewed.

Results:

Mean age was 5.3 years ± 2.8. The majority (68.1%) of the 163 children were of the black racial group. The highest rate of INS was seen in the 2-6 year age group (71.2%). The black racial group had the highest rate (42/111; 37.8%) of focal segmental glomerulosclerosis (FSGS), and the white race had the highest rate (9/14; 64.3%) of minimal change disease (MCD). Ninety four (57.7%) patients were steroid sensitive (SSNS) while 69 patients (42.3%) were steroid resistant (SRNS). Minimal change disease was the most common histo-pathological type seen in SSNS (60%), while FSGS was the most common observed in patients who had SRNS (65.2%).

Conclusion:

There appears to be a higher rate of FSGS in all the racial groups, and also a higher rate of MCD in the black race group, when compared to previous reports.

KEYWORDS:

Idiopathic; children; nephrotic syndrome

PMID:
29937885
PMCID:
PMC5870266
DOI:
10.4314/ahs.v17i4.22
[Indexed for MEDLINE]
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10.
Lancet. 2018 Jul 7;392(10141):61-74. doi: 10.1016/S0140-6736(18)30536-1. Epub 2018 Jun 14.

Idiopathic nephrotic syndrome in children.

Author information

1
Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
2
Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
3
Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada; Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, Toronto, ON, Canada; University Health Network, Toronto, ON, Canada; Dalla Lana School of Public Health, and Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada. Electronic address: rulan.parekh@sickkids.ca.

Erratum in

Abstract

The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast majority of patients who are steroid responsive; however, the disease can run a frequently relapsing course, necessitating the need for alternative immunosuppressive agents. Infection and venous thromboembolism are the main complications of NS with also increased risk of acute kidney injury. Prognosis in terms of long-term kidney outcome overall is excellent for steroid-responsive disease, and steroid resistance is an important determinant of future risk of chronic or end-stage kidney disease.

PMID:
29910038
DOI:
10.1016/S0140-6736(18)30536-1
[Indexed for MEDLINE]
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11.
Biomed Res Int. 2018 May 15;2018:5647120. doi: 10.1155/2018/5647120. eCollection 2018.

γδT Cells Exacerbate Podocyte Injury via the CD28/B7-1-Phosphor-SRC Kinase Pathway.

Chen W1,2, Wu Y1,2, Zhang G2,3, Wang M2,3, Yang H2,3, Li Q1,2.

Author information

1
Key Laboratory of the Ministry of Education, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Chongqing Key Laboratory of Child Infection and Immunity, Children's Hospital of Chongqing Medical University, Chongqing, China.
2
Children's Hospital of Chongqing Medical University, No. 136 Second Zhongshan Road, Yuzhong District, Chongqing 400014, China.
3
Department of Nephrology, Children's Hospital of Chongqing Medical University, Chongqing, China.

Abstract

Primary nephrotic syndrome (PNS) is a devastating pediatric disorder. However, its mechanism remains unclear. Previous studies detected B7-1 in podocytes; meanwhile, γδT cells play pivotal roles in immune diseases. Therefore, this study aimed to assess whether and how γδT cells impact podocytes via the CD28/B7-1 pathway. WT and TCRδ-/- mice were assessed. LPS was used to induce nephropathy. Total γδT and CD28+γδT cells were quantitated in mouse spleen and kidney samples. B7-1 and phosphor-SRC levels in the kidney were detected as well. In vitro, γδT cells from the mouse spleen were cocultured with mouse podocytes, and apoptosis rate and phosphor-SRC expression in podocytes were assessed. Compared with control mice, WT mice with LPS nephropathy showed increased amounts of γδT cells in the kidney. Kidney injury was alleviated in TCRδ-/- mice. Meanwhile, B7-1 and phosphor-SRC levels were increased in the kidney from WT mice with LPS nephropathy. CD28+γδT cells were decreased, indicating CD28 may play a role in LPS nephropathy. Immunofluorescence colocalization analysis revealed a tight association of γδT cells with B7-1 in the kidney. High B7-1 expression was detected in podocytes treated with LPS. Podocytes cocultured with γδT cells showed higher phosphor-SRC and apoptosis rate than other cell groups. Furthermore, CD28/B7-1 blockage with CTLA4-Ig in vitro relieved podocyte injury. γδT cells exacerbate podocyte injury via CD28/B7-1 signaling, with downstream involvement of phosphor-SRC. The CD28/B7-1 blocker CTLA4-Ig prevented progressive podocyte injury, providing a potential therapeutic tool for PNS.

PMID:
29862277
PMCID:
PMC5976931
DOI:
10.1155/2018/5647120
[Indexed for MEDLINE]
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12.
Nutrients. 2018 May 27;10(6). pii: E677. doi: 10.3390/nu10060677.

Longitudinal Study of the Role of Epidermal Growth Factor on the Fractional Excretion of Magnesium in Children: Effect of Calcineurin Inhibitors.

Author information

1
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. kristien.ledeganck@uantwerp.be.
2
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. charlotte.anne@student.uantwerpen.be.
3
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. amandine.demonie@student.uantwerpen.be.
4
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. sarang.meybosch@student.uantwerpen.be.
5
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. gert.verpooten@uantwerpen.be.
6
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. marleen.vinckx@uantwerpen.be.
7
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. Koen.vanhoeck@uza.be.
8
Department of Pediatric Nephrology, Antwerp University Hospital, Wilrijkstraat 10, 2650 Edegem, Belgium. Koen.vanhoeck@uza.be.
9
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. annelies.vaneyck@uantwerpen.be.
10
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. benedicte.dewinter@uantwerpen.be.
11
Laboratory of Experimental Medicine and Pediatrics, University of Antwerp, Universiteitsplein 1, T3.34, 2610 Antwerp, Belgium. Dominique.trouet@uza.be.
12
Department of Pediatric Nephrology, Antwerp University Hospital, Wilrijkstraat 10, 2650 Edegem, Belgium. Dominique.trouet@uza.be.

Abstract

BACKGROUND:

It was shown in animal models and adults that the epidermal growth factor (EGF) is involved in the pathophysiology of calcineurin inhibitor (CNI) induced renal magnesium loss. In children, however, the exact mechanism remains unclear, which was set as the purpose of the present study.

METHODS:

Children with nephrotic syndrome and renal transplant children treated with CNI (n = 50) and non-CNI treated children (n = 46) were included in this study. Urine and serum samples were collected at three time points to determine magnesium, creatinine, and EGF. The magnesium intake was calculated from a food frequency questionnaire.

RESULTS:

Serum Mg2+ and urinary EGF/creatinine were significantly lower in the CNI treated children, with significantly more CNI-treated children developing hypomagnesaemia. In the latter patients, the fractional excretion of magnesium (FE Mg2+) was significantly higher. Urinary EGF, age, renal function, and serum magnesium were independent predictors of the FE Mg2+. Only 29% of the children reached the recommended daily intake of magnesium. The magnesium intake did not differ between hypomagnesemic and normomagnesemic patients and was not a predictor of the FE Mg2+.

CONCLUSIONS:

In CNI-treated children who developed hypomagnesemia, the FE Mg2+ was increased. The urinary EGF concentration, age, and renal function are independent predictors of the FE Mg2+.

KEYWORDS:

calcineurin inhibitor; children; epidermal growth factor; fractional excretion; kidney transplantation; magnesium; magnesium intake; nephrotic syndrome

PMID:
29861470
PMCID:
PMC6024309
DOI:
10.3390/nu10060677
[Indexed for MEDLINE]
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13.
Medicine (Baltimore). 2018 Jun;97(22):e10556. doi: 10.1097/MD.0000000000010556.

Minimal change disease related to rifampicin presenting with acute renal failure during treatment for latent tuberculosis infection: A case report.

Author information

1
Department of Internal Medicine, Yeungnam University Medical Center.
2
Department of Pathology, Yeungnam University Medical Center, Daegu, Republic of Korea.

Abstract

RATIONALE:

The standard drugs used to treat tuberculosis are rifampicin and isoniazid. These agents are usually safe and inexpensive for short-term use in treatment of latent tuberculosis infection, but sometimes cause adverse renal effects, including minimal change disease (MCD).

PATIENT CONCERNS:

Here, we report a 51-year-old woman with latent tuberculosis infection who developed nephrotic syndrome during treatment with rifampicin and isoniazid for 25 days.

DIAGNOSES:

Renal biopsy findings were compatible with MCD, and she had no relevant medical history and was not taking other medications. A diagnosis of anti-tuberculosis drug- induced MCD was made. This is the first report of acute renal failure due to rifampicin and/or isoniazid-induced MCD.

INTERVENTIONS:

After cessation of rifampicin and isoniazid, however, acute renal failure progressed and she was treated with temporary dialysis and oral prednisolone.

OUTCOMES:

The patient achieved complete remission after cessation of rifampicin and isoniazid with steroid therapy.

LESSONS:

This case demonstrates that rifampicin and/or isoniazid can cause nephrotic syndrome with acute renal failure during the first months of continuous latent tuberculosis therapy. Therefore, renal function and proteinuria should be monitored carefully in all patients taking rifampicin and isoniazid, especially during the first few months of therapy.

PMID:
29851774
DOI:
10.1097/MD.0000000000010556
[Indexed for MEDLINE]
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14.
J Coll Physicians Surg Pak. 2018 Jun;28(6):436-439. doi: 10.29271/jcpsp.2018.06.436.

Histopathological Spectrum and Short-Term Outcome of Treatment with Cyclophosphamide in Relapsing Steroid-Sensitive Nephrotic Syndrome.

Author information

1
Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Karachi.
2
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi.

Abstract

OBJECTIVE:

To determine the short-term outcome of cyclophosphamide (CPO) course in children with relapsing steroid sensitive nephrotic syndrome (SSNS) with different histopathological lesions.

STUDY DESIGN:

Descriptive, observational study.

PLACE AND DURATION OF STUDY:

Pediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2012 to December 2014.

METHODOLOGY:

All children with relapsing steroid-sensitive nephrotic syndrome, who underwent renal biopsy and received cyclophosphamide therapy, were included and followed up for 2 years. Histopathological features in renal biopsy, duration of treatment, duration of complete remission and complication frequency was noted.

RESULTS:

Of the total 74 patients, 47 (63.5%) were males and 27 (36.5%) females. Median age with Interquartile range (IQR) at presentation was 5 years (4-7 years). Minimal change disease (MCD) was the most common histopathological diagnosis (n=54, 73%) followed by focal segmental glomerulosclerosis (FSGS) (n=13, 17.5%), mesangioproliferative glomerulonephritis(MesPGN) (n=6, 8.1%), IgA nephropathy (n=1, 1.4%). The median number of glomeruli included in each biopsy sample was 15. The median duration of treatment with CPO was 11 weeks (9 to 13 weeks), whereas the median duration of complete remission post-therapy was 13 months (7-23 months). A median timeframe of 17 months (13-24.2 months) lapsed between establishing the diagnosis of NS and initiating CPO treatment. Leucopenia was noted in six (8.1%) patients.

CONCLUSION:

The short-term outcome of relapsing SSNS can be improved with CPO and steroids, with minimum short-term side effects.

PMID:
29848418
DOI:
10.29271/jcpsp.2018.06.436
[Indexed for MEDLINE]
16.
J Vis Exp. 2018 May 6;(135). doi: 10.3791/57642.

Induction of Nephrotic Syndrome in Mice by Retrobulbar Injection of Doxorubicin and Prevention of Volume Retention by Sustained Release Aprotinin.

Author information

1
Department of Internal Medicine, Division of Endocrinology, Diabetology, Vascular Disease, Nephrology and Clinical Chemistry, University Hospital Tübingen; Institute of Diabetes Research and Metabolic Diseases (IDM) of the Helmholtz Center Munich, University Tübingen; German Center for Diabetes Research (DZD), University Tübingen.
2
Department of Internal Medicine, Division of Endocrinology, Diabetology, Vascular Disease, Nephrology and Clinical Chemistry, University Hospital Tübingen; Institute of Diabetes Research and Metabolic Diseases (IDM) of the Helmholtz Center Munich, University Tübingen; German Center for Diabetes Research (DZD), University Tübingen; Ferruh.Artunc@med.uni-tuebingen.de.

Abstract

Nephrotic syndrome is the most extreme manifestation of proteinuric kidney disease and characterized by heavy proteinuria, hypoalbuminemia, and edema due to sodium retention and hyperlipidemia. To study the pathophysiology of this syndrome, rodent models have been developed based on the injection of toxic substances such as doxorubicin causing podocyte damage. In mice, only few strains are susceptible to this model. In wildtype 129S1/SvImJ mice, the administration of doxorubicin by rapid intravenous injection to the retrobulbar sinus induces experimental nephrotic syndrome that features all the symptoms of human disease including sodium retention and edema. After the onset of proteinuria, mice exhibit increased urinary serine protease activity that leads to the activation of the epithelial sodium channel (ENaC) and sodium retention. Pharmacological inhibition of urinary serine proteases by the treatment with sustained release aprotinin abrogates ENaC activation and prevents sodium retention. This model is ideal to study the pathophysiology of proteasuria, i.e., the excretion of active serine proteases that cause ENaC activation by the proteolysis of its γ-subunit. This can be regarded as the primary mechanism of ENaC activation and sodium retention in proteinuric kidney disease.

PMID:
29782000
DOI:
10.3791/57642
[Indexed for MEDLINE]
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17.
Pol Merkur Lekarski. 2018 Apr 23;44(262):192-195.

[Mycophenolate mofetil (MMF) as the first choice immunosuppressive drug in treatment of steroid-dependent nephrotic syndrome in children].

[Article in Polish]

Author information

1
Department of Pediatric Nephrology and Hypertension CMUJ in Cracow, Poland.

Abstract

Most children diagnosed with nephrotic syndrome show favourable response to corticosteroid therapy, nonetheless 30% of patients have frequent relapses or a steroid-dependent course of disease. Cyclophosphamide, cyclosporin A or MMF are being used in treatment of steroid-dependent nephrotic syndrome in search of a drug with highest long-term effectiveness and least amount of side effects.

AIM:

The aim of study was to assess of the efficacy of MMF as the first choice immunosuppressive drug in children with nephrotic syndrome after determining a steroid-dependency.

MATERIALS AND METHODS:

23 children with steroid-dependent nephrotic syndrome were enrolled in the study. Mean age at disease onset was 3.8 years. Mean disease duration time before introducing MMF was 21.3 months. Mean treatment time with MMF was 23.6 months. Patients previously treated with immunosuppressive drug, except for prednisone were excluded from the study.

RESULTS:

Per year of treatment with MMF 56,5% of patients had not more than 1 relapse of the disease, 5 patients had more than 1, but less than 2 relapses. After the mean time of 23.6 months MMF treatment was discontinued in 15 patients (62,5%). 11 patients (48%) from that group significantly benefited from treatment in the form of no further relapses, defer of steroid-dependence or the possibility to reduce the dose of corticosteroids to minimal.

CONCLUSIONS:

MMF has advantage over cyclophosphamide and calcineurin inhibitors in reference to side effect profile, especially glomerular filtration markers, hypertension and frequent drug dependency. Treatment with MMF is effective in maintaining long-term remission and enables the reduction of cumulative steroid dose. Regarding nearly 50% of patients with benefits after MMF treatment and good treatment tolerance, it seems justified to introduce MMF as the first choice immunosuppressive drug in patients with steroiddependent nephrotic syndrome.

KEYWORDS:

immunosuppressive treatment; mycophenolate mofetil; nephrotic syndrome in children; steroid-dependence

PMID:
29775447
[Indexed for MEDLINE]
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18.
Pol Merkur Lekarski. 2018 Apr 23;44(262):183-187.

The clinical pattern of nephrotic syndrome in children has no effect on the concentration of soluble urokinase receptor (suPAR) in serum and urine.

Author information

1
The Children's Memorial Health Institute, Warsaw, Poland: Department of Biochemistry, Radioimmunology and Experimental Medicine.
2
The Children's Memorial Health Institute, Warsaw, Poland: Department of Nephrology, Kidney Transplantation and Hypertension.

Abstract

Concentration of soluble urokinase receptor (suPAR) was regarded as viable marker to differentiate the focal segmental glomerulosclerosis (FSGS) from other glomerulopathies and also as predictive parameter for progression of renal disease.

AIM:

The aim of this study was to evaluate serum and urine (s)(u)suPAR concentration in steroid-sensitive and steroid-resistant nephrotic children treated with different (double and triple-drug) regimens.

MATERIALS AND METHODS:

Overall 43 children were evaluated including 14 patients with steroid-resistant nephrotic syndrome (SRNS) aged 9±6 years and 29 with steroid-sensitive nephrotic syndrome (SSNS) aged 9±5 years, as well as control group (n=59). The concentration of suPAR was measured with ELISA kit (R∧D Systems Inc.).

RESULTS:

There was no difference in serum suPAR level between SRNS (6404, range: 4613-9575 pg/mL) and SSNS (5745, range: 4666-8246 pg/mL) patients, and also in urinary suPAR: SRNS (2877, range: 847- 19121 pg/mL) and SSNS (2854, range: 328-7434 pg/mL), respectively. There was no statistically significant difference in serum biomarker concentrations between patients with severe course of the disease, in combination therapy, with three drugs: CsA + MMF + Pred (5968, range: 4613-9575 pg/mL) in comparison with patients receiving double therapy: CsA + Pred or MMF + Pred (5449, range: 4666-6623 pg/mL, 5905, range: 5102-6730 pg/mL, respectively). SuPAR concentration in the urine of patients treated with Pred + MMF was lower (1493, range: 328-4444 pg/mL) than in patients receiving Pred + CsA (3193, range: 629-7434 pg/mL), as well as lower than in patients with triple combination of drugs (3318, range: 448-5570 pg/mL), however the difference was not statistically significant.

CONCLUSIONS:

Serum and urine concentration of suPAR did not different between different clinical patterns of nephrotic syndrome in children, regardless the immunosuppressive treatment used.

KEYWORDS:

children; nephrotic syndrome; soluble urokinase-type plasminogen activator receptor

PMID:
29775445
[Indexed for MEDLINE]
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19.
Gene. 2018 Jul 20;664:12-21. doi: 10.1016/j.gene.2018.04.067. Epub 2018 Apr 25.

Knockdown of NUP160 inhibits cell proliferation, induces apoptosis, autophagy and cell migration, and alters the expression and localization of podocyte associated molecules in mouse podocytes.

Author information

1
Department of Pediatrics, Fuzhou Dongfang Hospital, Second Military Medical University, Fuzhou 350025, Fujian, China; The Military Hospital of 92435 Unit of the People's Liberation Army, Ningde 352103, Fujian, China.
2
Department of Pediatrics, Dongfang Hospital, Xiamen University, Fuzhou 350025, Fujian, China.
3
Department of Pediatrics, Fuzhou Dongfang Hospital, Second Military Medical University, Fuzhou 350025, Fujian, China.
4
Department of Pediatrics, Fuzhou Dongfang Hospital, Second Military Medical University, Fuzhou 350025, Fujian, China; Department of Pediatrics, Dongfang Hospital, Xiamen University, Fuzhou 350025, Fujian, China; Department of Pediatrics, Fuzhou Clinical Medical College, Fujian Medical University, Fuzhou 350025, Fujian, China. Electronic address: zihuayu@vip.sina.com.

Abstract

Genetic mutations in dozens of monogenic genes can lead to serious podocyte dysfunction, which is a major cause of steroid-resistant nephrotic syndrome (SRNS). The NUP160 gene is expressed in both human kidney and mouse kidney. However, whether knockdown of NUP160 impairs podocytes has not yet been established. Therefore, we knocked down NUP160 by targeted short hairpin RNA (shRNA) in conditionally immortalized mouse podocytes and observed the effect of NUP160 knockdown on the proliferation, apoptosis, autophagy and cell migration of podocytes. We also investigated the effect of NUP160 knockdown on the expression and localization of podocyte associated molecules, such as nephrin, podocin, CD2AP and α-actinin-4. The knockdown of NUP160 significantly inhibited the proliferation of podocytes by decreasing the expression of both cyclin D1 and CDK4, increasing the expression of p27, and inducing S phase arrest. The knockdown of NUP160 promoted the apoptosis and autophagy of podocytes, and enhanced cell migration. The knockdown of NUP160 decreased the expression of nephrin, podocin and CD2AP in podocytes, and increased the expression of α-actinin-4. The knockdown of NUP160 also altered the subcellular localization of nephrin, podocin and CD2AP in podocytes. These results suggest that the knockdown of NUP160 impairs mouse podocytes, i.e. inhibiting cell proliferation, inducing apoptosis, autophagy and cell migration of mouse podocytes, and altering the expression and localization of podocyte associated molecules, including nephrin, podocin, CD2AP and α-actinin-4.

KEYWORDS:

NUP160; Nucleoporin; Podocyte; Podocyte associated molecules

PMID:
29704630
DOI:
10.1016/j.gene.2018.04.067
[Indexed for MEDLINE]
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20.
Kidney Int. 2018 May;93(5):1043-1044. doi: 10.1016/j.kint.2018.01.020.

Global glomerulosclerosis in primary nephrotic syndrome: including age as a variable to predict renal outcomes.

Author information

1
Kidney Diseases Branch, NIDDK, National Institutes of Health, Bethesda, Maryland, USA. Electronic address: jbkopp@nih.gov.

Abstract

With normal aging, there are increased numbers of globally sclerotic glomeruli. Global glomerulosclerosis is also a feature of chronic kidney disease. In this issue, Hommos and colleagues provide data on the fraction of globally sclerotic glomeruli in healthy kidney donor biopsies. This provides reference for evaluation of whether isolated global glomerulosclerosis is evidence of pathology.

PMID:
29680021
DOI:
10.1016/j.kint.2018.01.020
[Indexed for MEDLINE]
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