Materiały źródłowe

• #1 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Nephrotic syndrome is an important chronic disease in children. The estimated annual incidence of nephrotic syndrome in healthy children is two to seven new cases per 100,000 children less than 18 years of age. It is more common in boys than girls at younger ages, but once adolescence is reached, there is no significant difference between genders. Increased incidence and more severe diseases are seen in African American and Hispanic populations. […] Diabetic nephropathy associated with nephrotic syndrome is most common, with an estimated rate of around 50 cases per million population. In the pediatric population, nephrotic syndrome could occur at a rate of 20 cases per million. […] In India and Turkey, biopsy results in children with nephrotic syndrome have revealed similar histology types compared to what would be expected in Western countries. In Pakistani adult patients with nephrotic syndrome, the histological patterns of kidney biopsies are similar to those seen in western countries.

• #2 Nephrotic Syndrome Epidemiology

  https://www.researchandmarkets.com/report/global-nephrotic-syndrome-drug-market?srsltid=AfmBOorKrmF9fRw7yhT4Vxqwu95DvHr9ht7Zd5jmVec8rcI563az_xO3

  The analyst projects that among the total diagnosed prevalent cases of Nephrotic Syndrome in 7MM was around 786 thousand and approximately 34% of cases were from the US. As per our estimations, in 2022, the EU4 and the UK accounted for nearly 367 thousand diagnosed prevalent cases of Nephrotic Syndrome. […] The total diagnosed prevalent cases of Nephrotic Syndrome in Japan, in 2022 were approximately 151 thousand cases. These cases are expected to change during the study period. […] As per estimations, the total diagnosed prevalent cases of Nephrotic Syndrome in the 7MM were approximately 786 thousand in 2022, which are projected to increase during the forecast period due to rising population with obesity and diabetes. […] The analysis in 2022 revealed that the total diagnosed prevalent cases of nephrotic syndrome in the US stood at approximately 267 thousand. Among these cases, primary glomerulonephropathies contributed to a substantial portion, accounting for around 214 thousand cases, while secondary glomerulonephropathies contributed to approximately 53 thousand cases. These numbers are anticipated to escalate during the forecast period from 2023 to 2034, indicating a growing health concern that necessitates attention and resources.

• #3 Pediatric Nephrotic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/982920-overview

  In the United States, the reported annual incidence of nephrotic syndrome is 2-7 cases per 100,000 children younger than 16 years. The cumulative prevalence is approximately 16 cases per 100,000 individuals. The International Study of Kidney Disease in Children (ISKDC) found that 76.6% of children with INS had MCNS on kidney biopsy findings, with 7% of cases associated with FSGS. […] A study from New Zealand found the incidence of nephrotic syndrome to be almost 20 cases per million children under age 15 years. In specific populations, such as those of Finnish or Mennonite origin, congenital nephrotic syndrome may occur in 1 in 10,000 or 1 in 500 births, respectively. […] Some studies have suggested a change in the histopathology of INS over the past few decades, although the overall incidence of INS has remained stable. The frequency of FSGS associated with INS appears to be increasing. A review of the literature suggested a 2-fold increase in the incidence of FSGS in recent decades. However, another study found no evidence of an increasing incidence of FSGS.

• #4 Nephrotic Syndrome: Symptoms and Treatment | Doctor

  https://patient.info/doctor/nephrotic-syndrome-pro

  In children, the average worldwide incidence of nephrotic syndrome is 2-16.9 per 100,000. There is significant variability in both incidence and steroid responsiveness among various ethnic groups. […] In adults, the annual incidence of nephrotic syndrome in adults is 3 per 100,000. […] In adults, 80-90% of cases of nephrotic syndrome are idiopathic. Focal segmental glomerulosclerosis (FSGS) and membranous nephropathy are the most common primary causes. Type 2 diabetes mellitus and systemic lupus erythematosus (SLE) are the most common secondary causes. […] Minimal change disease accounts for 10-25% of cases of nephrotic syndrome in adults and 70-90% of cases in children.

• #5 Nephrotic syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Nephrotic_syndrome_epidemiology_and_demographics

  Idiopathic nephrotic syndrome has an incidence of 2-7 cases per 100,000 and a prevalence of 16 cases per 100,000. […] Nephrotic syndrome may affect children and adults alike. There is no age or ethnic predominance. The prevalence of nephrotic syndrome in children has a 2 to 1 male to female ratio. […] In total, the incidence of nephrotic syndrome is the same for adults and for children. Idiopathic nephrotic syndrome has an incidence of 2-7 cases per 100,000 and a prevalence of 16 cases per 100,000. […] According to observational studies, the prevalence of nephrotic syndrome in children has a 2 to 1 male to female ratio. In adults, however, the prevalence is the same in both genders.

• #6 Nephrotic Syndrome | 5-Minute Pediatric Consult

  https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/617242/all/Nephrotic_Syndrome?q=Ascites

  Minimal change nephrotic syndrome (MCNS) is the most frequent cause of NS in younger children: […] Occurs mainly between 2 and 8 years of age, with a peak at 3 years. […] Boys are more commonly affected than girls (3:2). […] Focal segmental glomerulosclerosis (FSGS) is the 2nd most frequent cause of NS in childhood: […] Children with FSGS are more likely than children with MCNS to have steroid-resistant nephrotic syndrome (SRNS). […] Less common than MCNS and FSGS are congenital NS (3 months) and infantile NS (3 to 12 months). […] 2 to 7 per 100,000 in children 16 years. […] 16 cases per 100,000 in children 16 years. […] African American and Hispanic children have a higher prevalence of FSGS than Caucasian and Asian children.

• #7 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Nephrotic-Syndrome-Epidemiology.aspx

  Nephrotic syndrome is quite rare but has an important role to play in the development of kidney disease. In adults, the incidence of the condition is approximately 3 cases per 100,000 per year. The incidence of minimal change disease in Caucasian children is reported to be 2 per 100,000. […] Higher rates of nephrotic syndrome have been reported in children of Arabian and Asian racial origins, in contrast to Caucasian children.

• #8 Diagnosis and Management of Nephrotic Syndrome in Adults | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0315/p479.html

  Nephrotic syndrome (NS) consists of peripheral edema, heavy proteinuria, and hypoalbuminemia, often with hyperlipidemia. […] The annual incidence of NS in adults is three per 100,000 persons. Approximately 80% to 90% of NS cases in adults are idiopathic. Membranous nephropathy is the most common cause in whites, and focal segmental glomerulosclerosis is most common in blacks; each of these disorders accounts for approximately 30% to 35% of NS cases in adults. Minimal change disease and immunoglobulin A nephropathy each account for approximately 15% of cases. The remaining 10% of cases are secondary to an underlying medical condition.

• #9

  https://step2.medbullets.com/renal/120696/nephrotic-syndrome

  Epidemiology […] Incidence […] annually there are 3 cases per 100,000 adults

• #10 Twenty-four-Year Trends in Incidence and Mortality of Nephrotic Syndrome: A Population-Based Cohort Study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36730008/

  With the increasing prevalence of risk factors for nephrotic syndrome, updated epidemiologic data on the syndrome are needed. […] We examined its age- and sex-specific incidence, histopathology, and mortality over 24 years. […] We computed overall, and age- and sex-specific, incidence rates of nephrotic syndrome, 1- and 5-year mortality by calendar period, and 1-year hazard ratios (HRs) of death using Cox models. […] Incidence was highest in men and increased with age to 11.77 per 100,000 person-years (95% confidence interval [CI]: 10.21-13.32) in men aged 80+ years, and 6.56 per 100,000 person-years (95% CI: 5.71-7.41) in women aged 80+ years. […] Incidence of nephrotic syndrome increased from 3.35 per 100,000 person-years (95% CI: 3.12-3.58) in 1995-2000 to 4.30 per 100,000 person-years (95% CI: 4.05-4.54) in 2013-2018.

• #11

  https://journals.lww.com/epidem/fulltext/2023/05000/twenty_four_year_trends_in_incidence_and_mortality.15.aspx

  With the increasing prevalence of risk factors for nephrotic syndrome, updated epidemiologic data on the syndrome are needed. We examined its age- and sex-specific incidence, histopathology, and mortality over 24 years. […] We identified 3,970 adults with first-time nephrotic syndrome diagnosis. Incidence was highest in men and increased with age to 11.77 per 100,000 person-years (95% confidence interval [CI]: 10.2113.32) in men aged 80+ years, and 6.56 per 100,000 person-years (95% CI: 5.717.41) in women aged 80+ years. Incidence of nephrotic syndrome increased from 3.35 per 100,000 person-years (95% CI: 3.123.58) in 19952000 to 4.30 per 100,000 person-years (95% CI: 4.054.54) in 20132018. […] During 19952018, the incidence of recorded adult nephrotic syndrome increased slightly, and the adjusted mortality of nephrotic syndrome decreased markedly. Whether these findings reflect changes in epidemiology or awareness and coding of nephrotic syndrome, remains to be clarified.

• #12 Nephrotic syndrome – GPnotebook

  https://gpnotebook.com/pages/ophthalmology/nephrotic-syndrome

  more common in males than females (ratio 3:2) […] conditions such as focal segmental glomerulosclerosis (FSGS) and mesangiocapillary glomerulosclerosis (MCGN) account for the remaining 20% of cases of NS […] MCD accounts for only 10 to 25 percent of cases of nephrotic syndrome in adults […] adults tend to respond more slowly, with more than 25 percent of responders taking three to four months or longer to undergo complete remission […] approximately 50 to 75 percent of glucocorticoid-responsive adults will have a relapse, and frequent relapses occur in 10 to 25 percent […] steroid-dependence is seen in 25 to 30 percent.

• #13 Nephrotic Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/244631-overview

  Nephrotic syndrome may affect adults and children of both sexes and of any race. It may occur in typical form, or in association with nephritic syndrome. The figure below shows the incidence per million population of important causes of nephrotic syndrome. Diabetic nephropathy with nephrotic syndrome is most common, at an estimated rate of at least 50 cases per million population. In children, nephrotic syndrome may occur at a rate of 20 cases per million children. […] Biopsy studies in children with nephrotic syndrome have shown similar types of histology in India and Turkey, compared with what one would expect in Western countries. In Pakistani adults with nephrotic syndrome, the spectrum of histologies of kidney biopsies is similar to that seen in western countries. […] Because diabetes is a major cause of nephrotic syndrome, American Indians, Hispanics, and African Americans have a higher incidence of nephrotic syndrome than do white persons. HIV nephropathy is a complication of HIV infection that is unusual in whites; it is seen with greater frequency in African Americans, because of their much greater prevalence of the ApoL1 risk alleles. Focal glomerulosclerosis appears to be overrepresented as a cause of nephrotic syndrome in African-American as compared with white children. […] There is a male predominance in the occurrence of nephrotic syndrome, as for chronic kidney disease in general. This male overrepresentation is also seen in paraneoplastic membranous nephropathy. But lupus nephritis affects mostly women.

• #14 Nephrotic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470444/

  Because diabetes mellitus is one of the major causes of nephrotic syndrome, American Indians, African Americans, and Hispanics have an increased incidence of nephrotic syndrome than White persons. HIV-associated nephropathy is a consequence of HIV infection that is uncommon in Whites; however, it is frequently seen in African Americans because of their greater prevalence of the ApoL1 alleles. Focal glomerulosclerosis seems to be overrepresented as one of the causes of nephrotic syndrome in African-Americans as opposed to White children.

• #15 Emerging Nephrotic Syndrome Treatments: A Glimpse Ahead

  https://www.delveinsight.com/blog/emerging-therapies-for-nephrotic-syndrome-treatment

  Nephrotic syndrome is a clinical condition characterized by significant protein in the urine (exceeding 40 mg/m2/h), leading to low levels of albumin (below 30 g/L), which in turn causes high levels of lipids, swelling, and a range of associated complications. […] The epidemiological assessment of nephrotic syndrome plays a pivotal role in understanding the prevalence, distribution, and impact of this renal disorder. To comprehensively evaluate the landscape of nephrotic syndrome, assumptions, and rationales are employed to delineate the primary and secondary glomeruli nephropathies contributing to the overall burden of the syndrome. […] As per DelveInsights estimations, the total nephrotic syndrome diagnosed prevalent cases in the 7MM were approximately 786K in 2022 and are projected to increase by 2034. DelveInsights analyst projects that among the total diagnosed prevalent cases of Nephrotic Syndrome in 7MM approximately 34% of cases were from the US. As per our estimations, in 2022, the EU4 and the UK accounted for nearly 367K diagnosed prevalent cases of nephrotic syndrome.

• #16 Nephrotic Syndrome Epidemiology

  https://www.researchandmarkets.com/report/global-nephrotic-syndrome-drug-market?srsltid=AfmBOorKrmF9fRw7yhT4Vxqwu95DvHr9ht7Zd5jmVec8rcI563az_xO3

  In EU4 and the UK, the diagnosed prevalent cases of nephrotic syndrome were estimated to be around 367 thousand in 2022. Among these regions, Germany emerged with the highest number of diagnosed prevalent cases, totaling approximately 98 thousand. Following closely behind, the UK reported around 87 thousand cases, indicating a considerable burden within these healthcare systems. Conversely, Spain reported the least number of cases among the EU4 and the UK, with approximately 48 thousand cases. […] In Japan, a notable breakdown of nephrotic syndrome cases in 2022 revealed approximately 120 thousand cases attributed to primary glomerulonephropathies and around 30 thousand cases attributed to secondary glomerulonephropathies.

• #17 Nephrotic Syndrome: Symptoms and Treatment | Doctor

  https://patient.info/doctor/nephrotic-syndrome-pro

  In children, the average worldwide incidence of nephrotic syndrome is 2-16.9 per 100,000. There is significant variability in both incidence and steroid responsiveness among various ethnic groups. […] In adults, the annual incidence of nephrotic syndrome in adults is 3 per 100,000. […] In adults, 80-90% of cases of nephrotic syndrome are idiopathic. Focal segmental glomerulosclerosis (FSGS) and membranous nephropathy are the most common primary causes. Type 2 diabetes mellitus and systemic lupus erythematosus (SLE) are the most common secondary causes. […] Minimal change disease accounts for 10-25% of cases of nephrotic syndrome in adults and 70-90% of cases in children.

• #18 Nephrotic Syndrome Research with ARREST-NEPHROSIS

  https://delta4.ai/arrest-nephrosis/

  Nephrotic Syndrome affects millions globally, presenting a significant health burden with its hallmark symptoms of severe proteinuria and edema. […] Globally, Nephrotic Syndrome affects millions, striking both adults and children, albeit with varying underlying causes and incidences. […] The impact of Nephrotic Syndrome extends beyond the physical symptoms. Living with this condition can affect mental health, lead to financial strain due to the cost of treatment and care, and disrupt social and professional life. […] Registries collect data on the incidence and prevalence of diseases, which is essential for public health monitoring, understanding disease patterns, and planning healthcare services. […] For conditions like Nephrotic Syndrome, the collaboration between biobanks and registries accelerates the pace of discovery and the translation of research findings into tangible medical interventions, ultimately enhancing patient care and outcomes.

• #19 Nephrotic Syndrome Research with ARREST-NEPHROSIS

  https://delta4.ai/arrest-nephrosis/

  ARREST-NEPHROSIS aims to provide a real-world snapshot of Nephrotic Syndrome, offering invaluable insights into its management and the efficacy of existing therapies. […] ARREST-NEPHROSIS serves as a vital resource for researchers and pharmaceutical companies, providing access to a wealth of data and biological samples that can accelerate the development of new treatments and facilitate patient recruitment for clinical trials.

• #20 NephCure Kidney Network for Patients with Nephrotic Syndrome – Phase I | PCORI

  https://www.pcori.org/research-results/2013/nephcure-kidney-network-patients-nephrotic-syndrome-phase-i

  Focal segmental glomerulosclerosis (FSGS), minimal change disease (MCD), and membranous nephropathy (MN) collectively referred to as primary nephrotic syndrome are rare but serious kidney diseases that pose substantial physical, psychological, and financial burdens and often lead to major medical complications including kidney failure requiring dialysis. […] Our collaborative group will develop the NephCure Kidney Network (NKN) as a Patient-Powered Research Network (PPRN) to serve as a resource for comparative effectiveness research to improve care and outcomes for these patients. […] Our team is composed of specialists in adult and pediatric clinical nephrology, clinical research in nephrotic syndrome, epidemiology, patient advocacy, and data management from Arbor Research Collaborative for Health (Arbor Research), the NephCure Foundation (NephCure), and the University of Michigan (U-M).

• #21

  https://link.springer.com/article/10.1007/s10157-015-1216-x

  MCNS, focal segmental glomerulosclerosis (FSGS), MN, and membranoproliferative glomerulonephritis are known to relapse frequently. However, a wide range of relapse rates was reported in previous articles; thus, prospective follow-up surveys such as the Japanese Nephrotic Syndrome Cohort Study (JNSCS) are expected to provide precise rates. […] Various complications develop in patients with nephrotic syndrome. Although cohort studies performed abroad revealed a high incidence of cardiovascular events, the actual state in Japan seems to be different. Treatment with glucocorticoids and/or immunosuppressants, and nephrotic syndrome itself, often make patients susceptible to infection, the true rate of which remains to be determined. Reports from abroad also highlighted a high incidence of thromboembolic events. Furthermore, the westernized lifestyle makes the Japanese population more susceptible to thrombosis and therefore should receive research attention. Malignant tumors have been considered a common complication in patients with nephrotic syndrome. However, according to recent surveys, the co-occurrence rate of malignant tumors with nephrotic syndrome seems relatively low in Asian countries such as Japan and China compared with that in Western countries. Acute renal failure is another representative complication in patients with nephrotic syndrome, especially in the elderly.

• #22

  https://link.springer.com/article/10.1007/s10157-015-1216-x

  The researchers of the Committee for the Standardization of Renal Pathological Diagnosis and the Working Group for the Renal Biopsy Database of the Japanese Society of Nephrology had set up the J-RBR/J-KDR (Japan Renal Biopsy and Kidney Disease Registry) since 2007, and the epidemiology of nephrotic syndrome in Japan was gradually revealed. In the analysis of cases registered to the J-RBR until the end of 2010, primary glomerular disease was the most frequently occurring glomerular disease and diabetic nephropathy was the most frequent among the secondary glomerular diseases. The total cases of membranous nephropathy (MN) and minimal change nephrotic syndrome (MCNS) were close to 80 % among the primary glomerular diseases. In the analysis of nephrotic syndrome patients aged 65 years, the ratios of diabetic nephropathy and amyloid nephropathy were highest, next to primary glomerular disease.

• #23 Population-Based Epidemiology, Management and Outcomes of Nephrotic Syndrome in Adults and Children: The Kaiser Permanente Nephrotic Syndrome Cohort Study – Kaiser Permanente Division of Research

  https://divisionofresearch.kaiserpermanente.org/studies/population-based-epidemiology-management-and-outcomes-of-nephrotic-syndrome-in-adults-and-children-the-kaiser-permanente-nephrotic-syndrome-cohort-study/

  The aims of this study are to: 1) Assemble a cohort of KPNC adult and pediatric members with nephrotic syndrome and examine temporal trends in the epidemiology of nephrotic syndrome in adults and children between 1996 and 2012, including classification of potential causes. […] 2) Examine temporal trends in the evaluation and management of nephrotic syndrome in adults and children, overall and in targeted subgroups. […] 3) Assess predictors of developing nephrotic syndrome in adults and children, overall and by presumptive cause. […] 4) Characterize the short- and longer-term prognosis of nephrotic syndrome in adults vs. children with regards to renal and cardiovascular outcomes.

• #24

  https://link.springer.com/article/10.1007/s00467-023-06006-9

  A total of 128 and 324 cases of first onset INS were reported in the Netherlands and Paris area, respectively, corresponding to an annual incidence of 1.21 and 2.58 per 100,000 children/year. […] Incidence before and during the pandemic did not differ. When schools were closed, incidence was lower in both regions: 0.53 vs. 1.31 (p=0.017) in the Netherlands and 0.94 vs. 2.63 (p=0.049) in the Paris area. […] The results of this observational study in the Netherlands and the Paris area in France show that incidence of INS was lower when schools were closed and lockdown measures were at their maximum. There was no difference between the period before and during the Covid-19 pandemic in both countries. […] Our findings, combined with data on viral infections from the literature, support our hypothesis that the onset of INS is potentially driven by a (viral) infection. This is based on several observations. First, incidence in both regions was lower when schools were closed.

• #25

  https://link.springer.com/article/10.1007/s00467-023-06006-9

  This study has some limitations. Although new cases were prospectively reported to the Dutch study team, the additional survey identified ten more first onset INS patients. […] In conclusion, our results suggest a link between INS onset and viral infections in children, although COVID-19 does not appear to be a specific trigger. A significant decrease in incidence was observed in periods when child-to-child interaction was minimised by closing schools and thereby reducing viral transmission in two independent European cohorts.

• #26 Twenty-four-Year Trends in Incidence and Mortality of Nephrotic Syndrome: A Population-Based Cohort Study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/36730008/

  Over time, 1-year mortality of nephrotic syndrome was stable at 13%-16%, but HR of death was 0.54 (95% CI: 0.42-0.69), adjusted for age, sex, and comorbidities, in 2013-2018 compared with 1995-2000. […] During 1995-2018, the incidence of recorded adult nephrotic syndrome increased slightly, and the adjusted mortality of nephrotic syndrome decreased markedly.

• #27

  https://journals.lww.com/cjasn/fulltext/2012/03000/epidemiology_and_pathophysiology_of_nephrotic.21.aspx

  After infections, thromboembolism is considered by many experts to be the most significant life-threatening complication of nephrotic syndrome. […] Children (2.8%) are less likely than adults (26.7%) with nephrotic syndrome to develop thromboembolism. However, infants and children aged 12 years are at much greater risk. […] Membranous histologic changes increase thromboembolic risk at all ages; in particular, adults with membranous nephropathy have the highest reported risk (37.0%) and children with membranous histology have a rate (25%) that approaches the overall adult rate. […] There is inadequate evidence to support routine prophylactic therapy. […] Future hemostatic research in nephrotic syndrome should focus on identifying cohorts at highest risk for thrombosis through the use of clinical markers and biomarkers as well as searching for molecular targets to correct the prothrombotic pathophysiology of this disease.

• #28 Systemic Complications In Childhood Nephrotic Syndrome | IJNRD

  https://www.dovepress.com/disease-associated-systemic-complications-in-childhood-nephrotic-syndr-peer-reviewed-fulltext-article-IJNRD

  Nephrotic syndrome (NS) is one of the most common childhood kidney diseases. […] The prevalence of childhood NS worldwide is approximately 16 cases per 100,000 children, with an incidence of two to seven per 100,000 children. […] Complications of childhood NS are divided into two categories: disease-associated complications and drug-associated complications. […] However, complications especially disease-associated ones in pediatric patients with NS are still limited. […] The aim of the study was to systematically assess disease-associated complications in children with NS to better understand how they impact outcomes. […] This systematic review shows that the disease-associated systemic complications in childhood NS include cardiovascular complications, infections, thyroid complications, kidney complications, and poor oral health condition, ie, caries and enamel hypoplasia.

• #29 Systemic Complications In Childhood Nephrotic Syndrome | IJNRD

  https://www.dovepress.com/disease-associated-systemic-complications-in-childhood-nephrotic-syndr-peer-reviewed-fulltext-article-IJNRD

  Complications in pediatric patients with NS include cardiovascular complications linked to atherosclerosis and thromboembolism, infections (both minor and major), thyroid-hormone complications, especially hypothyroidism, kidney complications involving AKI, oral health complications due to defects in enamel, and periodontal disease. […] These complications need the attention of health-care providers to prevent any worsening of patients condition.

• #30 Orphanet: Idiopathic steroid-resistant nephrotic syndrome

  https://www.orpha.net/en/disease/detail/567548

  The annual incidence of idiopathic nephrotic syndrome is estimated at 1/34,000 children, although this varies according to ethnicity or the country of origin. […] Steroid-resistance is described in 2.1-27.3% of children with idiopathic nephrotic syndrome, with annual incidence estimated at 1/390,000. […] In most regions, there is a male predominance, although reported ratios vary between 1.0 and 3.8.

• #31 Clinical Patterns and Renal Survival of Nephrotic Syndrome in Childhood: A Single-Center Study (1980-2006)

  https://openurologyandnephrologyjournal.com/VOLUME/3/PAGE/8/FULLTEXT/

  The nephrotic syndrome (NS) is a relatively rare but severe form of glomerulopathy in children. The estimated global annual incidence rate of NS is 1.9 – 2.6 cases per 100000 children. […] However, steroid resistance (SRNS) has been associated with adverse outcome in terms of potential development of stage 3 of chronic kidney disease (CKD3). […] The aim of the present study was to investigate and compare the clinical, epidemiological, histological patterns and treatment response among the Ukrainian children with NS during two consecutive time periods (1980-2000 and 2001-2006) besides the assessment of prognostic factors that modify the risk of development of End Stage Renal Disease (ESRD) in this part of population. […] The CKD3 rate is one of the crucial characteristics of the NS treatment effectiveness. A significant decrease in the percentage of CKD3 outcomes was found in the entire cohort over the years 2001-2006 in contrast to the years 1980-2000 (11.2% vs 17.6%, p=0.008). […] The most important risk factor of the unfavorable prognosis of NS was the unresponsiveness to PRED.

• #32 Childhood Nephrotic Syndrome in Africa: Epidemiology, Treatment Trends, and Outcomes

  https://ouci.dntb.gov.ua/en/works/4OnPNbW7/

  Childhood nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function. […] Nephrotic syndrome (NS) remains the most common presentation of glomerular diseases in children. […] The objective of our study was to describe the characteristics and outcomes of NS in children from three major hospitals in one of the world’s poorest countries, Chad. […] In Chad, childhood idiopathic nephrotic syndrome predominantly affects young males; steroid sensitivity is as high as 95%, and in the long-term, 80% of patients achieve remission with normal renal function.

• #33 eCommons@AKU

  https://ecommons.aku.edu/eastafrica_fhs_mc_intern_med/403/

  Childhood nephrotic syndrome, if left untreated, leads to progressive kidney disease or death. We quantified the prevalence of steroid-sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome, and histological types as the epidemiology of nephrotic syndrome in Africa remains unknown, yet impacts outcomes. […] Few African countries reported on the prevalence of childhood nephrotic syndrome. Steroid-sensitive disease is more common than steroid-resistant disease although prevalence of steroid-resistant nephrotic syndrome is higher than reported globally. Scarcity of data in Africa prevents appropriate healthcare resource allocation to diagnose and treat this treatable childhood kidney disease to prevent poor health outcomes.

• #34 Nephrotic Syndrome in Adults | NeST | Nephrotic Syndrome Trust

  https://nstrust.co.uk/who-we-are/nephrotic-syndrome-in-adults

  Nephrotic syndrome in adults is rare (it effects approximately 1 person per 30,000 people per year) but it is an important type of kidney problem and there are many people with nephrotic syndrome seen in kidney outpatient clinics. […] All patients with nephrotic syndrome would usually be seen regularly in a hospital nephrology outpatient clinic where they would be monitored by blood and urine tests. […] Nephrotic syndrome is sometimes divided into primary and secondary nephrotic syndrome. Primary nephrotic syndrome is caused by diseases which specifically effect the kidneys and the glomeruli in particular. […] Nephrotic syndrome affects around 10,000 people in the UK.

• #35 G386 Surveillance biopsies of tacrolimus effect in childhood nephrotic syndrome | Archives of Disease in Childhood

  https://adc.bmj.com/content/101/Suppl_1/A224

  Objectives Tacrolimus is increasingly advocated as a steroid sparing agent in the management of nephrotic syndrome. As concerns exist about long term nephrotoxicity, interval renal biopsies have been advocated to detect early changes. Associations between histological changes and patient factors are not well established. […] Methods Single centre review of surveillance renal histology of children who received tacrolimus to treat nephrotic syndrome between 20022015. […] Results 25 children (16 male, 1 non-caucasian, 21 minimal change, 4 FSGS) commenced tacrolimus at median age 4.0 years (range 1.48.9). 9/25(35%) of first biopsy taken after median duration 4.7 years (range 1.9 to 6.9) demonstrated features of calcineurin inhibitor (CNI) nephrotoxicity. […] Conclusion Significant number of children on tacrolimus showed histological nephrotoxicity after a short duration of therapy. In this small single centre experience, toxicity correlated with tacrolimus level rather than duration. A high number relapsed shortly following dose reduction. We suggest maintaining tacrolimus levels 5.5 g/L to minimise nephrotoxicity.

• #36

  https://scholars.duke.edu/publication/1507072

  Background: Childhood nephrotic syndrome, if left untreated, leads to progressive kidney disease or death. […] We quantified the prevalence of steroid-sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome, and histological types as the epidemiology of nephrotic syndrome in Africa remains unknown, yet impacts outcomes. […] Few African countries reported on the prevalence of childhood nephrotic syndrome. […] Scarcity of data in Africa prevents appropriate healthcare resource allocation to diagnose and treat this treatable childhood kidney disease to prevent poor health outcomes.

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