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Ethnic Differences in Childhood Nephrotic Syndrome.

Chanchlani R, Parekh RS - Front Pediatr (2016)

Bottom Line: For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease.Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes.In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Nephrology, Hospital for Sick Children, Toronto, ON, Canada; Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, Toronto, ON, Canada; Division of Pediatric Nephrology, McMaster Children's Hospital, Hamilton, ON, Canada.

ABSTRACT
Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2-16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid-resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease.

No MeSH data available.


Related in: MedlinePlus

(A) Incidence of childhood nephrotic syndrome per 100,000 persons by ethnicity reported from 1946 to 2014. (B) Variability of steroid responsiveness by ethnicity among children with nephrotic syndrome in reported studies from 1986 to 2014.
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Figure 1: (A) Incidence of childhood nephrotic syndrome per 100,000 persons by ethnicity reported from 1946 to 2014. (B) Variability of steroid responsiveness by ethnicity among children with nephrotic syndrome in reported studies from 1986 to 2014.

Mentions: Based on our review of the literature, the average incidence of nephrotic syndrome is 4.7 (range 1.15–16.9) per 100,000 persons in studies reported from 1946 to 2014, and the proportion with steroid resistance is 12.4% (range 2.1–27.3%) from 1986 to 2014 (Figures 1A,B). Hence, there is a considerable variation in disease burden by country of origin and steroid responsiveness, suggesting the potential role of ethnicity in susceptibility to disease (8–23).


Ethnic Differences in Childhood Nephrotic Syndrome.

Chanchlani R, Parekh RS - Front Pediatr (2016)

(A) Incidence of childhood nephrotic syndrome per 100,000 persons by ethnicity reported from 1946 to 2014. (B) Variability of steroid responsiveness by ethnicity among children with nephrotic syndrome in reported studies from 1986 to 2014.
© Copyright Policy
Related In: Results  -  Collection

License
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getmorefigures.php?uid=PMC4835686&req=5

Figure 1: (A) Incidence of childhood nephrotic syndrome per 100,000 persons by ethnicity reported from 1946 to 2014. (B) Variability of steroid responsiveness by ethnicity among children with nephrotic syndrome in reported studies from 1986 to 2014.
Mentions: Based on our review of the literature, the average incidence of nephrotic syndrome is 4.7 (range 1.15–16.9) per 100,000 persons in studies reported from 1946 to 2014, and the proportion with steroid resistance is 12.4% (range 2.1–27.3%) from 1986 to 2014 (Figures 1A,B). Hence, there is a considerable variation in disease burden by country of origin and steroid responsiveness, suggesting the potential role of ethnicity in susceptibility to disease (8–23).

Bottom Line: For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease.Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes.In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Nephrology, Hospital for Sick Children, Toronto, ON, Canada; Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, Toronto, ON, Canada; Division of Pediatric Nephrology, McMaster Children's Hospital, Hamilton, ON, Canada.

ABSTRACT
Nephrotic syndrome is a common glomerular disease in children with significant variability in both incidence and steroid responsiveness among various ethnic groups. The average incidence of nephrotic syndrome is 2-16.9 per 100,000 children worldwide. Understanding the variability by ethnicity may point to potential factors leading to nephrotic syndrome, which remains elusive, and may highlight factors accounting for differences in medication response. The emerging role of genetic factors associated with steroid responsive and steroid-resistant forms of nephrotic syndrome within an ethnic group can provide insight into potential biological mechanisms leading to disease. For example, among African-Americans, the risk variants in APOL1 are associated with a more than 10-fold increase in risk of focal segmental glomerulosclerosis and high-risk carriers have a twofold greater risk of progression to end-stage renal disease. Ongoing collaborative studies should consider capturing data on self-reported ethnicity to understand differences in incidence and outcomes. In the future, the availability of whole-genome data will provide an excellent opportunity for new clinical and translational research in childhood nephrotic syndrome and lead to a better understanding of the disease.

No MeSH data available.


Related in: MedlinePlus