Effect of urine screening for school children with pediatric renal disease: A quantitative evaluation of children with IgA nephropathy.

ABSTRACT. Primary IgA nephropathy (IgAN) is characterized by recurrent episodes of macroscopic hematuria accompanied by upper respiratory tract infections or persistent asymptomatic microscopic hematuria with or without proteinuria. IgAN may involve one or more members of a family. Three generations of a cohort of 110 patients with biopsy-proven IgAN, living in Southern Italy, were checked for urinalysis, and the relative risk (RR) of developing the disease was evaluated. A total of 19 unrelated familial, 37 suspected, and 54 sporadic cases of IgAN were identified. Renal survival was estimated by the Kaplan-Meier method for censored data and compared by use of the log-rank test. More than 50% of the patients with IgAN clustered in kindred with more than two probably affected relatives. In 19 unrelated IgAN families, 8 had single-generation (SG) and 11 multigenerational (MG) involvement showing a prevalent vertical transmission of the trait. The RR was 16 times higher in first-degree relatives (odds ratio [OR], 16.4; 95% confidence interval [CI], 5.7 to 47.8; P < 0.0001) and >2 times higher, even if NS, in second-degree relatives (OR, 2.4; 95 % CI, 0.7 to 7.9; P = 0.145). The clinical and histologic picture of familial and sporadic IgAN appeared to be similar. The 20-yr renal survival rate from the apparent onset of the disease was significantly poorer in patients with familial (41%) than in patients with sporadic (94%) IgAN (P = 0.003). Furthermore, 15-yr renal survival from the time of renal biopsy was significantly worse in familial IgAN (P = 0.02); end-stage renal disease was present in 64% of familial and only in 8% of patients with sporadic IgAN. Finally, renal survival was significantly worse in patients belonging to families with SG rather than with MG involvement (P = 0.03). These data show, for the first time, that familial IgAN may be considered a nonbenign disease that occurs frequently in first-degree relatives. Familial IgAN has a poorer outcome than sporadic IgAN. Therefore, an accurate family history and urinalysis in all family members is urgently recommended in clinical practice. This procedure might avoid late referral of subjects with persistent and underestimated urinary abnormalities and late diagnosis of the disease.

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P0114BENEFITS AND CHALLENGES OF A RENAL BIOPSY REGISTRY AND NETWORK - FCGG EXPERIENCE

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0114 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Wim Laurens ◽

Johan De Meester ◽

Ben Sprangers ◽

Steven Van Laecke ◽

Dendooven Amélie ◽

...

Keyword(s):

Renal Disease ◽

Renal Biopsy ◽

Iga Nephropathy ◽

Clinical Care ◽

Adult Population ◽

Age Groups ◽

Time Frame ◽

Renal Pathology ◽

Renal Diseases ◽

Renal Biopsies

Abstract Background and Aims End December 2016, a renal biopsy network in the Flemish region (Belgium) was founded as a collaboration between the renal pathologists and nephrologists. This FCGG network introduced a uniform renal biopsy request form, a well-structured report form of the renal pathology examination and a comprehensive list of renal pathology diagnoses. Method Following informed consent [99.5%] and in compliance with GDPR, the registration of the renal biopsies consists of basic categorical renal data, structured renal pathology information and the final clinical renal disease. Results In 2017 and 2018, 1385 renal biopsies were registered – 10.5 per million inhabitants per year; in addition, 28 patients had a repeat biopsy in the same time frame (2%). Of the pediatric patients ( age < 18 years; N=92; 6.6%), 23 had IgA nephropathy, 20 a minimal change disease and 15 another type of glomerulonephritis. The biopsy was reported as normal or non-diagnostic in 15 patients (16%) – the majority was clinically considered as glomerulonephritis. The spectrum of the adult population was quite similar across gender and age groups: 56% glomerulonephritis [= IgA nephropathy [19%] + FSGS [8%] + pauci-immune glomerulonephritis [7%] + other GN [22%] ), 10% tubulo-interstitial nephritis, 7% acute tubular necrosis [ATN], 7% diabetes mellitus, and 7% nephroangiosclerosis. Exceptions are pauci-immune glomerulonephritis as the most important renal disease in women aged 65 years and older, and lupus nephritis as the second most important glomerulonephritis in women aged 18-44 years. Only a small percentage of adult renal biopsies yielded no result (7%), clinically interpreted as glomerulonephritis in 50% of the cases. Conclusion The FCGG network has provided a more intense collaboration between renal pathologists and nephrologists mainly by standardizing the renal biopsy reading and reporting across all centers. More precise estimates of the prevalent renal diseases were provided for the first time; however, in order to get full information, renal diseases diagnosed by other techniques ( serology, genetic analysis ) should also be collected in the future. Efforts will be done to coordinate the clinical care of renal diseases, particularly the more rare renal diseases, and to offer access to new therapeutic molecules or new schemes, through this super-regional network.

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Treatment effects of Chinese medicine (Yi-Qi-Qing-Jie herbal compound) combined with immunosuppression therapies in IgA nephropathy patients with high-risk of end-stage renal disease (TCM-WINE): study protocol for a randomized controlled trial

Trials ◽

10.1186/s13063-019-3989-9 ◽

2020 ◽

Vol 21 (1) ◽

Cited By ~ 4

Author(s):

Shen Li ◽

Jin-pu Li

Keyword(s):

High Risk ◽

Renal Disease ◽

Iga Nephropathy ◽

End Stage Renal Disease ◽

Controlled Trial ◽

Eligible Patient ◽

Study Phase ◽

Double Blind ◽

Stage Renal Disease ◽

End Stage

Abstract Background IgA nephropathy (IgAN) is the most common glomerular disease worldwide. It has a high incidence in Asians and is more likely to progress to end-stage renal disease (ESRD). For high-risk IgAN, which is clinically characterized by massive proteinuria and renal dysfunction, however, there has been no international consensus on treatment options. Compared with other developed countries, IgAN patients in China are often found to have severe kidney function loss at initial diagnosis. Yi-Qi-Qing-Jie formula (YQF; a compound recipe of Chinese medicinal herbs) has shown potential renal protection in our previous clinical studies. To further confirm the efficacy and safety of YQF in the treatment of high-risk IgAN, we have designed a prospective double-blind randomized placebo-controlled trial. Methods/design The TCM-WINE study is a single-center, prospective, double-blind randomized placebo-controlled trial. We plan to randomize 60 participants with biopsy-proven IgAN to a YQF combined group (YQF compound combined with prednisolone, and cyclophosphamide if necessary) or an immunosuppression group (placebo-YQF combined with prednisolone, and cyclophosphamide if necessary). The two groups will enter a 48-week in-trial treatment phase and receive post-trial follow-up until study completion (3 years). All patients will receive optimal supportive care. The primary composite outcome is defined as the first occurrence of a 40% decrease in estimated glomerular filtration rate (eGFR) from the baseline lasting for 3 months, initiating continuous renal replacement treatment, or death due to chronic kidney disease (CKD) during the 3-year study phase. The secondary endpoint events are defined as the mean annual eGFR decline rate (eGFR slope, ml/min per 1.73 m2 per year), which is calculated by the eGFR regression curve for each eligible patient, and proteinuria remission (prescribed as proteinuria < 0.5 g/day) at weeks 24, 36, and 48 during the in-trial phase. The remission rate of symptoms and inflammation status will be evaluated at week 48. Safety monitoring and assessment will be undertaken during the study. Discussion The TCM-WINE study will evaluate the effects and safety of YQF combined therapy compared with immunosuppression monotherapy on the basis of the optimal supportive treatment in high-risk IgAN. The evidence from this study will provide a novel, effective, and safe Chinese characteristic therapy for high-risk IgAN patients. Trial registration ClinicalTrials.gov, NCT03418779. Registered on 18 June 2018.

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FAMILIAL IgA NEPHROPATHY: A STUDY OF RENAL DISEASE IN AN AUSTRALIAN ABORIGINAL FAMILY

Australian and New Zealand Journal of Medicine ◽

10.1111/j.1445-5994.1987.tb05045.x ◽

1987 ◽

Vol 17 (1) ◽

pp. 27-33 ◽

Cited By ~ 33

Author(s):

P. J. O'CONNELL ◽

L. S. IBELS ◽

M. A. THOMAS ◽

M. HARRIS ◽

R. P. ECKSTEIN

Keyword(s):

Renal Disease ◽

Iga Nephropathy ◽

Australian Aboriginal

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End Stage Renal Disease caused by IgA Nephropathy - One Case Report -

Journal of Physiology & Pathology in Korean Medicine ◽

10.15188/kjopp.2013.12.27.6.823 ◽

2013 ◽

Keyword(s):

Case Report ◽

Renal Disease ◽

Iga Nephropathy ◽

End Stage Renal Disease ◽

Stage Renal Disease ◽

End Stage

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Spectrum of Biopsy Proven Glomerular Disease in Children at Kanti Children’s Hospital

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v34i3.11162 ◽

2015 ◽

Vol 34 (3) ◽

pp. 225-229 ◽

Cited By ~ 1

Author(s):

N Khatun ◽

KP Bista ◽

C Mahaseth

Keyword(s):

Nephrotic Syndrome ◽

Lupus Nephritis ◽

Renal Disease ◽

Iga Nephropathy ◽

Kidney Biopsy ◽

Demographic Data ◽

Glomerular Disease ◽

Children's Hospital ◽

Steroid Dependent ◽

Children’S Hospital

Introduction: Glomerular disease is one of the most common forms of renal disease and can have many different clinical presentations. However there is variation in the prevalence in the type of glomerular disease according to geographical location and race of population. The aim of the study was to find the overall distribution pattern of glomerular disease based on renal biopsies. Material and Methods: The medical records of all children who underwent kidney biopsy (n=29) between January 2012-june 2014 were analyzed. In this retrospective study we review children from Kanti Children’s Hospital, Nephrology Department. Demographic data including age, sex and indication of kidney biopsy as well as complication of the procedures were recorded. Result: A total number of thirty one biopsies were done. Two children were excluded from the study due to inadequate tissue and artifacts defects in preservative. The remaining twenty nine biopsies were included in the study and analyzed. Renal disease was found nearly equal in both male and female (51.72% vs. 48.27%).Mean age was 10.95±3.30 years. Maximum number of biopsies (75.86%) was performed between 10- 15 years. The most common indication of the kidney biopsy was nephrotic syndrome (steroid resistant nephrotic syndrome and steroid dependent nephrotic syndrome, 31.02% followed by lupus nephritis 27.58%.The most common glomerular disease were focal segmental glomerulosclerosis and lupus nephritis both contribute 27.58%. Among lupus nephritis class III was found more common (44.44%) in children. IgA nephropathy was also contribute (17.24%) in all glomerular disease. Regarding the kidney biopsy complication gross hematuria was observed in 6.89% of children which was self-limited. Conclusion: FSGS and lupus nephritis is becoming the most common glomerular disease in children attending the Kanti Children Hospital. IgA nephropathy was also contribute the glomerular disease in the children. J Nepal Paediatr Soc 2014;34(3):225-229 DOI: http://dx.doi.org/10.3126/jnps.v34i3.11162

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