scholarly journals Osteoprotegerin in Childhood Nephrotic Syndrome: Relation To Glucocorticoids, Parathyroid Hormone And Markers of Bone Metabolism

GEGET ◽  
2005 ◽  
Vol 5 (1) ◽  
pp. 63-76
Author(s):  
Gehan Mostafa ◽  
Nadia Nagui
Keyword(s):  
Nephrotic Syndrome ◽  
Parathyroid Hormone ◽  
Bone Metabolism ◽  
Childhood Nephrotic Syndrome

scholarly journals Assessment of the Concentration of Bone Metabolism Markers: Sclerostin and FGF-23 in Children with Idiopathic Nephrotic Syndrome Treated with Glucocorticosteroids

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Agnieszka Pukajło-Marczyk ◽  
Anna Jakubowska ◽  
Agnieszka Bargenda-Lange ◽  
Katarzyna Kiliś-Pstrusińska ◽  
Danuta Zwolińska
Keyword(s):  
Nephrotic Syndrome ◽  
Parathyroid Hormone ◽  
Bone Metabolism ◽  
Idiopathic Nephrotic Syndrome ◽  
Fibroblast Growth Factor 23 ◽  
Control Group ◽  
Healthy Children ◽  
Positive Correlation ◽  
Long Term Treatment ◽  
Fgf 23

Recurring nature of idiopathic nephrotic syndrome (INS) and steroid dependence imply a long-term treatment with glucocorticosteroids (GCSs), which increases the risk of bone metabolism disorders. The search for new markers of that process is essential. The aims of this study were to assess the concentrations of sclerostin (Scl) and fibroblast growth factor-23 (FGF-23) in the plasma of children with INS and compare Scl and FGF-23 to existing markers of bone metabolism, mainly parathyroid hormone (PTH). The study involved 70 children, 50 with INS and 20 healthy children. Patients with INS were divided into 4 groups depending on the number of relapses and applied therapy. Significantly higher concentrations of FGF-23 and Scl were found in all patient groups with INS compared to the control group, and increase in the concentrations of examined parameters depending on the number of NS relapses was showed. In patients from the group with numerous relapses, higher concentrations of FGF-23 and Scl in the relapse phase than those in the remission phase were found. We observed positive correlation in these proteins with parathyroid hormone. Positive correlation of FGF-23 and Scl in the examined group was noted. Children having relapsing INS treated with steroids have higher levels of Scl and FGF-23 that can indicate the bone metabolism disorders. The significance of these observations requires further research.

scholarly journals Steroid treatment for the first episode of childhood nephrotic syndrome: comparison of the 8 and 12 weeks regimen using an individual patient data meta-analysis

2021 ◽  
Author(s):  
Anne M. Schijvens ◽  
Nynke Teeninga ◽  
Eiske M. Dorresteijn ◽  
Steven Teerenstra ◽  
Nicholas J. Webb ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Individual Patient Data ◽  
Meta Analysis ◽  
Steroid Treatment ◽  
First Episode ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
First Relapse ◽  
Steroid Regimen

AbstractSteroids are the cornerstone of the treatment of childhood nephrotic syndrome. The optimal duration for the first episode remains a matter of debate. The aim of this study is to determine whether the 8 weeks International Study of Kidney Disease in Children (ISKDC) regimen is equally effective as the 12 weeks steroid regimen from the German society of pediatric nephrology (Arbeitsgemeinschaft für Pädiatrische Nephrologie [APN]). An individual patient data (IPD) meta-analysis of randomized controlled trials reporting on prednisolone treatment for a first episode of childhood nephrotic syndrome was conducted. European trials aimed at investigating the ISKDC and/or APN steroid regimen were selected. The lead investigators of the selected trials were requested to provide the IPD of the specific treatment groups. Four trials included European cohorts using dosing schedules according to the regimens studied. IPD of two trials were available. A significant difference was found in time to first relapse after cessation of steroid treatment between the 8 and 12 weeks treatment group with a median time to relapse of 29 and 63 days, respectively. Moreover, relapse rate ratios during total follow-up were 51% higher for the 8 weeks regimen. Finally, younger children have a significantly lower time to first relapse and frequently relapsing nephrotic syndrome.Conclusions: The results of this IPD meta-analysis suggest that the 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen. Moreover, this study highlights the importance of using uniform definitions to enable accurate comparison and interpretation of trial results.Trial registration: Registration number: CRD42020199244, date of registration 16-08-2020 What is Known:• Steroids are the cornerstone of the treatment of childhood nephrotic syndrome, however the optimal duration for the first episode remains a matter of debate.• Currently, the 8 weeks ISKDC protocol and 12 weeks APN protocol are among the most frequently used protocols in Europe. What is New:• The 8 weeks steroid regimen for a first episode of steroid-sensitive nephrotic syndrome may not be equally effective as the 12 weeks steroid regimen for the treatment of a first episode of nephrotic syndrome.• Younger children have a significantly shorter time to first relapse and time to frequent relapsing nephrotic syndrome.

scholarly journals SLPI is a critical mediator that controls PTH-induced bone formation

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Akito Morimoto ◽  
Junichi Kikuta ◽  
Keizo Nishikawa ◽  
Takao Sudo ◽  
Maki Uenaka ◽  
...  
Keyword(s):  
Parathyroid Hormone ◽  
Bone Resorption ◽  
Bone Formation ◽  
Protease Inhibitor ◽  
Bone Metabolism ◽  
Serine Protease ◽  
Serine Protease Inhibitor ◽  
Secretory Leukocyte Protease Inhibitor ◽  
Bone Imaging ◽  
Genetic Ablation

AbstractOsteoclastic bone resorption and osteoblastic bone formation/replenishment are closely coupled in bone metabolism. Anabolic parathyroid hormone (PTH), which is commonly used for treating osteoporosis, shifts the balance from osteoclastic to osteoblastic, although it is unclear how these cells are coordinately regulated by PTH. Here, we identify a serine protease inhibitor, secretory leukocyte protease inhibitor (SLPI), as a critical mediator that is involved in the PTH-mediated shift to the osteoblastic phase. Slpi is highly upregulated in osteoblasts by PTH, while genetic ablation of Slpi severely impairs PTH-induced bone formation. Slpi induction in osteoblasts enhances its differentiation, and increases osteoblast–osteoclast contact, thereby suppressing osteoclastic function. Intravital bone imaging reveals that the PTH-mediated association between osteoblasts and osteoclasts is disrupted in the absence of SLPI. Collectively, these results demonstrate that SLPI regulates the communication between osteoblasts and osteoclasts to promote PTH-induced bone anabolism.

scholarly journals Management of idiopathic childhood nephrotic syndrome in sub-Saharan Africa: Ibadan consensus statement

2021 ◽  
Vol 99 (1) ◽  
pp. 59-67 ◽  
Author(s):  
Christopher Esezobor ◽  
Adebowale D. Ademola ◽  
Adewale E. Adetunji ◽  
Emmanuel A. Anigilaje ◽  
Anthony Batte ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Consensus Statement ◽  
Sub Saharan Africa ◽  
Childhood Nephrotic Syndrome ◽  
Sub Saharan

Haemostatic measurements in childhood nephrotic syndrome

1991 ◽  
Vol 150 (5) ◽  
pp. 374-378 ◽  
Author(s):  
A. T. H. Elidrissy ◽  
M. B. Abdurrahman ◽  
H. M. Bahakim ◽  
M. D. Jones ◽  
A. M. A. Gader
Keyword(s):  
Nephrotic Syndrome ◽  
Childhood Nephrotic Syndrome

scholarly journals Twenty-year follow-up of protective immunity of the Oka strain of live varicella vaccine recipients with childhood nephrotic syndrome

1994 ◽  
Vol 7 (2) ◽  
pp. 213-216
Author(s):  
Shigeru Minowa ◽  
Sachiko Takeichi ◽  
Yoshizo Asano ◽  
Takehiko Yasaki ◽  
Kazuo Tsuzuki ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Protective Immunity ◽  
Varicella Vaccine ◽  
Childhood Nephrotic Syndrome

scholarly journals Practice patterns and outcomes of repository corticotropin injection (Acthar® Gel) use in childhood nephrotic syndrome: A study of the North American Pediatric Renal Trials and collaborative studies and the Pediatric Nephrology Research Consortium

2021 ◽  
Vol 5 (2) ◽  
pp. 067-076
Author(s):  
Faizan Mohammed K ◽  
McCracken Courtney ◽  
Lieberman Kenneth ◽  
Leong Traci ◽  
Benfield Mark R
Keyword(s):  
Nephrotic Syndrome ◽  
Demographic Data ◽  
Pediatric Nephrology ◽  
Drug Regimen ◽  
Disease Response ◽  
Childhood Nephrotic Syndrome ◽  
The North ◽  
Set Up ◽  
Steroid Responsiveness ◽  
Active Disease

Objective: We set up a U.S. registry to examine prescription patterns and patient outcomes of repository corticotropin injection (Acthar® Gel) for childhood nephrotic syndrome. Methods: 18 participating U.S. pediatric centers performed retrospective review and prospective observation of patients < 21 years old with nephrotic syndrome treated with Acthar Gel. We captured baseline characteristics, drug regimen and duration, and disease response following treatment. Results: 46 patients, enrolled from 2015 to 2020 were included. 27 (58.7%) were male. 18 patients (39.1%) had a diagnosis of minimal change followed by focal segmental glomerulosclerosis in 16 patients (34.7%). Median age at start of treatment was 12.5 years (IQR 8.5-17.4) compared to 5.3 years at diagnosis (IQR 2.7-10.5 years). 52% were resistant to corticosteroids. The most common Acthar Gel regimen was 80IU twice a week with a median duration of 199 days (IQR 88-365). Among 37 patients with active disease, 18 (49%) were able to achieve partial or complete remission, though all patients that had a positive response were on other immunosuppressants concomitantly. Conclusion: We report the findings of the largest registry cohort of pediatric patients in the U.S. treated with Acthar Gel for clinically challenging cases of nephrotic syndrome. Acthar Gel was successful in inducing remission in approximately half of the patients with active disease at time of treatment. No predictors of response with respect to demographic data, age at start of Acthar Gel therapy, etiology of nephrotic syndrome, presence or absence of comorbidities, or steroid responsiveness was noted.

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