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. 2025 Jul 1;26:334. doi: 10.1186/s12882-025-04269-y

Global, regional, and national burden of congenital anomalies of the kidney and urinary tract from 1990 to 2021, with projections to 2036: a systematic analysis of the global burden of disease study 2021

Ting Chen 1, Jia Wei 1, Qiang Shu 2, Xiang Yan 1,✉
PMCID: PMC12219625  PMID: 40597805

Abstract

Background

Congenital anomalies of the kidney and urinary tract (CAKUT) represents an important global health challenge. However, the emerging analysis of the burden of CAKUT is limited and outdated. This study aimed to evaluate the global burden and temporal trends of CAKUT across 204 countries and territories from 1990 to 2021.

Methods

Data on the prevalence, incidence, mortality, and disability-adjusted life years (DALYs) of CAKUT from 1990 to 2021 were obtained from the Global Burden of Disease Study 2021. Temporal trends were analyzed using Joinpoint regression. The association between CAKUT burden and the socio-demographic index (SDI) was examined via Spearman correlation analysis. Decomposition analysis was used to estimate the effects of population growth, aging, and epidemiological changes on overall change of CAKUT burdens. The Bayesian age–period–cohort model was utilized to predict the CAKUT burden through 2036.

Results

From 1990 to 2021, the global prevalence of CAKUT increased by 21.50%, reaching 6.34 million (95% uncertainty interval [UI]: 5.07, 7.90), with a modest rise of 3.96% in the age-standardized prevalence rate (ASPR). In contrast, the incidence, mortality, and DALYs of CAKUT declined, with the age-standardized incidence rate (ASIR) decreasing by 2.86%, the age-standardized mortality rate (ASMR) by 20.00%, and the age-standardized DALY rate (ASDR) by 18.46%. In 2021, Southern Sub-Saharan Africa exhibited the highest ASPR and ASIR, while Central Latin America recorded the highest ASMR, and Southern Latin America had the highest ASDR. Furthermore, ASPR, ASIR, ASMR, and ASDR were all negatively correlated with SDI. Decomposition analysis revealed that population growth drove the increase in CAKUT prevalence. Projections to 2036 suggest further increases in ASPR and ASIR, whereas ASMR and ASDR are expected to decline.

Conclusion

Despite reductions in ASIR, ASMR, and ASDR, the increasing ASPR underscores the persistent global burden of CAKUT. Targeted interventions are urgently needed, particularly in high-burden regions such as Southern Sub-Saharan Africa.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12882-025-04269-y.

Keywords: CAKUT, Global burden of disease, Forecasting, Prevalence, Incidence, Mortality, Disability-adjusted life years

Authors and affiliations.

Introduction

Congenital anomalies of the kidney and urinary tract (CAKUT) encompass a large variety of malformations that are caused by impaired embryonic development of the genitourinary tract [1, 2]. The majority of CAKUTs can be diagnosed early in life, with a broad spectrum of manifestations ranging from mild urinary tract structural anomalies to severe renal dysfunction, even leading to end-stage renal disease (ESRD) [3]. CAKUT represents the main cause of pediatric chronic kidney disease (CKD), accounting for 40–50% of pediatric CKD cases [4, 5]. Given the increasing global burden of CKD, which is projected to become the fifth leading cause of death by 2040 [6], a thorough understanding of its upstream etiologies, such as CAKUT, is imperative.

Recent literature has highlighted important limitations in the current understanding of the burden of CAKUT. Many published studies are based on single-center experiences or national-level hospital data, and often lack methodological standardization [7–10]. The Global Burden of Disease (GBD) study offers a robust and standardized framework for evaluating disease burden across 204 countries and territories over time. It provides an invaluable data repository for assessing the health impacts of a plethora of diseases and injuries [11]. Through this resource, researchers can evaluate key health metrics such as prevalence, incidence, mortality, and disability-adjusted life years (DALYs) associated with CAKUT. These data are crucial for understanding the disease’s impact on both global and regional scales, offering insights into disparities across countries, regions, and socioeconomic groups [12, 13]. Two recent studies utilizing GBD 2019 data provided valuable insights into the global burden of CAKUT [14, 15]. However, these analyses had several limitations. They were based on the earlier GBD 2019 dataset rather than the most up-to-date GBD 2021 estimates. Furthermore, one of the studies focused exclusively on individuals aged 0–24 years, without capturing the burden across the full life course. Importantly, neither study included projections of future disease burden, which limits their utility for long-term health planning and policy development.

This study aims to fill critical gaps by providing a comprehensive assessment of the global, regional, and national burden of CAKUT via estimates from the Global Burden of Disease Study 2021 (GBD 2021). We will analyze the prevalence, incidence, mortality, and DALYs associated with CAKUT while also projecting trends up to 2036. Through rigorous data analysis and future-oriented modeling, this research seeks to offer a more nuanced understanding of CAKUT, contributing valuable insights for clinicians and public health policymakers [16, 17].

Methods

Data source

Our study utilized data from the GBD 2021 study, which is the largest and most comprehensive global observational epidemiologic survey to date. The GBD 2021 study provides annual disease burden estimates of 371 diseases and injuries associated with 88 risk factors in 204 countries and territories from 1990 to 2021 [11, 18]. The detailed methodology used for GBD 2021 has been described previously [11, 13, 18]. We focused on four key metrics associated with CAKUT: prevalence, incidence, mortality, and DALYs. The estimates of prevalence and incidence for CAKUT are produced using DisMod-MR 2.1 (Disease Modelling Meta-Regression; version 2.1), a Bayesian meta-regression tool developed by the GBD team, through an analytical cascade process. The estimates of mortality for CAKUT are generated using the Cause of Death Ensemble Model (CODEm). DALYs, a summary measure of overall health loss, were calculated by adding years of life lost (YLLs) and years lived with disability (YLDs) attributable to CAKUT. YLLs are calculated by multiplying the number of deaths associated with CAKUT by the remaining life expectancy at the time of death, based on a standard life expectancy. YLDs are calculated by multiplying the number of cases with a certain health outcome by the weight of the specified disability [12, 13, 18]. The data were accessed from the Global Health Data Exchange (GHDx, http://ghdx.healthdata.org) and include annual statistics disaggregated by age, sex, and geographic location. To describe the burden of CAKUT in different geographic units, our analysis included 204 countries and territories, which were grouped into 21 GBD regions based on geographic proximity and further aggregated into seven super-regions according to similarities in cause-of-death patterns, as defined by the GBD framework. Additionally, all countries were categorized into five socio-demographic index (SDI) quintiles to assess the global burden and trends of CAKUT.

Definition of CAKUT

The spectrum of CAKUT is broad and is collectively reported under urogenital congenital anomalies in the GBD 2021 dataset. In this study, CAKUT encompasses congenital anomalies of the genital and urinary systems present at birth, including malformations of the collecting system, ureter, bladder, and kidney; bladder exstrophy and epispadias; hypospadias; ambiguous or indeterminate sex; and other genital malformations, as defined by the GBD [11].

SDI

The SDI, developed by GBD researchers, is a composite indicator used to evaluate the socio-economic development of regions. It combines income per capita, average educational attainment, and fertility rates into a single metric that ranges from 0 (worst) to 1 (best). Based on SDI scores, regions are classified into five quintiles: low (0–0.465816), low-middle (0.465816–0.618829), middle (0.618829–0.711975), high-middle (0.711975–0.810296), and high (0.810296–1) [11].

Data analysis

Our data analysis commenced with an exploratory overview of the dataset’s structure, calculating counts and rates for key indicators such as the prevalence, incidence, mortality, and DALYs associated with CAKUT at the global, regional, and national levels. In accordance with the GBD framework, 95% uncertainty intervals (UIs) were computed for all final estimates as the 2.5th and 97.5th percentiles values of 500 or 1000 draws [11, 12].

The estimated annual percentage change (EAPC) is widely used to quantify the temporal trend of rates over a specified time interval. A regression line was fitted to the natural logarithm of the rates, expressed as ln (rate) = α + β × (calendar year) + ε. And EAPC was calculated as 100 × (exp (β) − 1), with corresponding 95% confidence intervals (CIs) derived from the linear regression model. The EAPC is considered statistically significant if the 95% CIs do not include zero.

We utilized Joinpoint regression software (version 5.2.0) to identify significant changes in data trends over time, thereby distinguishing genuine shifts in trends from random variability. This method uses statistical criteria to determine the minimum number of linear segments required to describe a trend. The software calculates the annual percent change (APC) for each segment, as well as the average annual percent change (AAPC) over the entire study period, along with the corresponding 95% CIs, allowing us to assess the direction, magnitude, and significance of the observed trends. The APC and AAPC are considered statistically significant if the 95% CIs do not include zero.

The correlation between the SDI and CAKUT indicators was examined via Spearman correlation analysis, which helped us assess the strength and direction of their linear relationships across various geographic locations.

We utilized decomposition analysis by population growth, aging, and epidemiological changes to better understand the explanatory factors driving changes in the key indicators of CAKUT between 1990 and 2021 [19].

To project future trends in CAKUT through 2036, we applied the Bayesian age-period-cohort (BAPC) model. This approach allowed us to estimate future patterns while accounting for the effects of age, period, and cohort factors [20–22].

All the statistical analyses were executed via R version 4.3.3.

Results

Global trends

Between 1990 and 2021, the global prevalence of CAKUT increased from 5,221,076 (95% UI: 4188738, 6516684) to 6,343,413 (95% UI: 5069614, 7900494) cases. The age-standardized prevalence rate (ASPR) rose from 86.34 (95% UI: 69.25, 107.63) per 100,000 individuals to 89.76 (95% UI: 71.86, 111.25) per 100,000 individuals. Conversely, the global incidence of CAKUT decreased from 1,167,139 (95% UI: 887687, 1554170) to 1,094,236 (95% UI: 823143, 1438205) cases, with the age-standardized incidence rate (ASIR) showing a slight decrease from 18.21 (95% UI: 13.85, 24.25) per 100,000 individuals to 17.69 (95% UI: 13.31, 23.25) per 100,000 individuals. The global number of deaths due to CAKUT significantly decreased from 9679 (95% UI: 6857, 15280) to 7495 (95% UI: 5175, 11712). Similarly, the age-standardized mortality rate (ASMR) decreased from 0.15 (95% UI: 0.11, 0.24) per 100,000 individuals to 0.12 (95% UI: 0.08, 0.19) per 100,000 individuals. The global DALYs attributable to CAKUT also showed a significant decrease, decreasing from 1,043,302 (95% UI: 775258, 1567968) to 882,185 (95% UI: 659007, 1258015). This trend was reflected in the age-standardized DALY rate (ASDR), which declined from 16.63 (95% UI: 12.38, 24.89) per 100,000 individuals to 13.56 (95% UI: 10.08, 19.57) per 100,000 individuals (Fig. 1; Table 1).

Fig. 1.

Fig. 1

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Trends in global numbers and ASRs of CAKUT prevalence (a), incidence (b), deaths (c), and DALYs (d) among different sexes, from 1990 to 2021. ASRs, age-standardized rates; ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate; DALYs, disability-adjusted life years

Table 1.

The global numbers and ASRs of prevalence, incidence, deaths, and dalys for CAKUT

Measure All ages cases (1990) All ages cases (2021) Age-standardized rates per 100 000 people (2021)
Total Male Female Total Male Female Total Male Female
n (95% UI) n (95% UI) n (95% UI) n (95% UI) n (95% UI) n (95% UI) n (95% UI) n (95% UI) n (95% UI)
Prevalence 5,221,076 (4188738,6516684) 2,353,311 (1868263,2952129) 2,867,765 (2309980,3541559) 6,343,413 (5069614,7900494) 2,954,601 (2329695,3707274) 3,388,812 (2718970,4195504) 89.76 (71.86,111.25) 81.73 (64.92,102.21) 98.19 (79.39,121.36)
Incidence 1,167,139 (887687,1554170) 568,360 (431650,757081) 598,779 (454511,800617) 1,094,236 (823143,1438205) 551,828 (413326,727785) 542,408 (407967,716770) 17.69 (13.31,23.25) 17.25 (12.92,22.75) 18.16 (13.66,24)
Deaths 9679 (6857,15280) 5461 (3236,9077) 4218 (1843,8233) 7495 (5175,11712) 4894 (2978,8226) 2601 (1601,4343) 0.12 (0.08,0.19) 0.15 (0.09,0.25) 0.08 (0.05,0.14)
DALYs 1,043,302 (775258,1567968) 569,971 (360494,900971) 473,331 (264352,819503) 882,185 (659007,1258015) 535,992 (368094,835885) 346,193 (237705,517654) 13.56 (10.08,19.57) 16.12 (10.97,25.46) 10.82 (7.41,16.49)
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DALYs, disability-adjusted life years

An in-depth analysis using APC and AAPC revealed diverse trends for CAKUT. ASPR demonstrated an overall increasing trend, as indicated by an AAPC of 0.13% (95% CI: 0.11%, 0.15%) (Supplementary Table 1). Notably, the period from 2005 to 2009 experienced the most pronounced decline, with an APC of -0.21% (95% CI: -0.28%, -0.14%). Conversely, the most significant increase in ASPR was observed from 2019 to 2021, with an APC of 1.09% (95% CI: 0.95%, 1.23%) (Supplementary Fig. 1a and Supplementary Table 2). ASIR exhibited a biphasic trend, initially decreasing from 1990 to 2016 before experiencing a subsequent increase. The rebound in ASIR was particularly evident from 2016 to 2021, with an APC of 1.67% (95% CI: 1.47%, 1.88%) (Supplementary Fig. 1b and Supplementary Table 2). The ASMR showed a consistent downward trend, reflected by an AAPC of -0.87% (95% CI: -0.96%, -0.77%) (Supplementary Table 1). The most substantial decrease in the ASMR was recorded between 2019 and 2021, with an APC of -2.90% (95% CI: -4.07%, -1.71%) (Supplementary Fig. 2a and Supplementary Table 2). Similarly, the ASDR exhibited a significant downward trend, with an AAPC of -0.63% (95% CI: -0.68%, -0.59%) (Supplementary Table 1). The most pronounced reduction in the ASDR was observed over the period from 2013 to 2021, with an APC of -1.49% (95% CI: -1.61%, -1.37%) (Supplementary Fig. 2b and Supplementary Table 2).

Regional trends in 21 GBD regions

At the level of 21 GBD regions in 2021, Southern Sub-Saharan Africa exhibited the highest ASPR and ASIR, reaching 164.25 (95% UI: 129.29, 206.02) and 28.41 (95% UI: 21.04, 38.86), respectively. The highest ASMR was observed in Central Latin America, at 0.27 (95% UI: 0.19, 0.35), while Southern Latin America recorded the highest ASDR at 26.21 (95% UI: 15.84, 35.22). Notably, East Asia and Southeast Asia had the lowest ASPR, ASIR, ASMR, and ASDR among all 21 GBD regions (Table 2).

Table 2.

The numbers and ASRs of prevalence, incidence, deaths, and dalys for CAKUT across the 21 GBD region in 2021

Location Prevalence Incidence Deaths DALYs
Cases (95%UI) ASPR (95%UI) Cases (95%UI) ASIR (95%UI) Cases (95%UI) ASMR (95%UI) Cases (95%UI) ASDR (95%UI)
Southeast Asia, East Asia, and Oceania
East Asia 486,107 (390781,597275) 51.5 (41.37,63.64) 60,774 (44055,82158) 11.02 (7.99,14.89) 133 (54,209) 0.01 (0.00,0.03) 24,665 (15895,37561) 2.77 (1.65,4.35)
Southeast Asia 297,511 (233395,375698) 48.11 (37.7,60.46) 53,439 (39490,71738) 9.91 (7.32,13.3) 169 (100,333) 0.03 (0.02,0.06) 25,045 (16437,39485) 4.27 (2.79,6.85)
Oceania 15,036 (11777,19232) 85.83 (67.29,110.21) 3565 (2675,4848) 17.36 (13.03,23.61) 20 (4,55) 0.10 (0.02,0.27) 2361 (854,5397) 12.15 (4.68,26.94)
Central Europe, Eastern Europe, and Central Asia
Central Asia 88,449 (70461,108127) 90.9 (72.32,111.45) 17,674 (12773,23656) 17.99 (13.01,24.09) 52 (36,76) 0.05 (0.04,0.08) 7413 (5500,10303) 7.56 (5.60,10.52)
Central Europe 54,734 (44018,66750) 80.9 (65.11,98.93) 6855 (5097,8941) 13.62 (10.13,17.77) 38 (20,52) 0.07 (0.04,0.10) 5279 (3324,7232) 9.27 (5.70,12.77)
Eastern Europe 167,491 (135239,204944) 127.49 (102.38,155.45) 18,731 (13470,25167) 21.72 (15.62,29.18) 58 (38,98) 0.05 (0.03,0.08) 9945 (7037,13900) 8.08 (5.55,11.55)
High-income
High-income Asia Pacific 98,358 (79789,121119) 107.25 (86.3,132.07) 9832 (7473,12990) 17.21 (13.08,22.73) 35 (21,58) 0.06 (0.04,0.09) 6705 (4572,9286) 9.06 (6.25,12.41)
Australasia 18,134 (14724,21927) 84.92 (68.82,102.91) 2759 (1985,3751) 16.04 (11.54,21.81) 13 (9,27) 0.07 (0.04,0.15) 1638 (1154,2890) 8.49 (5.94,15.64)
Western Europe 207,749 (170498,250401) 78.66 (64.92,95.24) 27,549 (22277,33949) 13.98 (11.30,17.22) 165 (104,217) 0.08 (0.05,0.10) 20,875 (14896,27276) 9.32 (6.49,12.15)
Southern Latin America 57,171 (45680,70151) 106.11 (85.69,130.22) 6746 (5059,8890) 18.12 (13.59,23.88) 95 (54,132) 0.25 (0.14,0.35) 10,449 (6443,13917) 26.21 (15.84,35.22)
High-income North America 155,033 (126063,186131) 62.51 (50.64,75.45) 26,095 (19243,34853) 13.39 (9.87,17.88) 258 (179,341) 0.13 (0.09,0.17) 27,252 (19660,34052) 13.10 (9.36,16.59)
Latin America and Caribbean
Caribbean 28,196 (22799,33983) 67.48 (54.40,81.58) 4909 (3625,6578) 12.87 (9.50,17.25) 52 (28,118) 0.13 (0.07,0.3) 5343 (3225,10928) 13.56 (8.12,27.83)
Andean Latin America 38,156 (31238,46157) 59.05 (48.15,71.57) 7318 (5491,9654) 12.31 (9.24,16.24) 83 (46,146) 0.14 (0.08,0.24) 8368 (5062,14204) 13.78 (8.30,23.53)
Central Latin America 187,651 (154501,223340) 82.33 (67.56,97.96) 28,541 (21122,37196) 15.23 (11.27,19.85) 581 (405,743) 0.27 (0.19,0.35) 51,542 (37553,65316) 24.61 (17.78,31.68)
Tropical Latin America 105,221 (85036,126193) 55.63 (44.71,67.14) 20,205 (15186,26827) 12.22 (9.19,16.23) 380 (196,509) 0.22 (0.12,0.30) 36,217 (19833,47961) 21.41 (11.62,28.43)
North Africa and Middle East
North Africa and Middle East 639,153 (507974,803575) 100.25 (79.82,126.38) 86,214 (65312,111016) 15.07 (11.42,19.40) 1363 (852,2157) 0.23 (0.15,0.37) 146,040 (98315,216102) 24.73 (16.63,36.79)
South Asia
South Asia 1,996,136 (1555342,2568175) 111.97 (87.39,143.43) 338,108 (247792,456233) 22.33 (16.37,30.14) 2439 (1060,4871) 0.16 (0.07,0.32) 293,670 (158751,518527) 18.42 (9.73,33.33)
Sub-Saharan Africa
Central Sub-Saharan Africa 239,113 (185957,305249) 126.65 (99.95,161.45) 52,943 (39030,72252) 24.77 (18.26,33.80) 160 (59,454) 0.08 (0.03,0.22) 23,162 (12297,47502) 11.57 (6.24,23.19)
Eastern Sub-Saharan Africa 649,125 (509242,819187) 111.03 (87.81,139.30) 137,987 (103037,183612) 21.03 (15.70,27.99) 473 (195,1173) 0.07 (0.03,0.18) 65,637 (36824,124935) 10.62 (6.08,19.79)
Southern Sub-Saharan Africa 137,330 (107872,172735) 164.25 (129.29,206.02) 22,210 (16443,30376) 28.41 (21.04,38.86) 44 (22,73) 0.06 (0.03,0.09) 8950 (6073,12801) 10.97 (7.51,15.64)
Western Sub-Saharan Africa 677,557 (538492,841523) 96.20 (76.79,119.34) 161,783 (120590,213250) 18.99 (14.15,25.03) 885 (447,2123) 0.11 (0.06,0.26) 101,631 (61457,207289) 12.94 (7.97,25.49)
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ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate; DALYs, disability-adjusted life years

Between 1990 and 2021, the EAPCs exhibited significant variation across the 21 GBD regions. In terms of the ASPR, 11 GBD regions showed a positive EAPC, with Andean Latin America and Oceania recording the highest EAPC values at 0.58% (95% CI: 0.56%, 0.59%) and 0.49% (95% CI: 0.44%, 0.53%), respectively. Conversely, Eastern Europe recorded the most substantial decline, with an EAPC of ASPR at -0.43% (95% CI: -0.46%, -0.39%). For the ASIR, only five GBD regions exhibited a positive EAPC. Andean Latin America and Oceania again showed the most pronounced increases, with EAPC of ASIR at 0.77% (95% CI: 0.67%, 0.86%) and 0.52% (95% CI: 0.47%, 0.58%), respectively. In contrast, the lowest EAPC of ASIR was observed in High-income Asia Pacific, at -1.22% (95% CI: -1.45%, -0.99%). The trends for the ASMR and the ASDR followed a similar pattern. Only seven regions—Oceania, Tropical Latin America, Central Latin America, Andean Latin America, Southern Sub-Saharan Africa, Western Sub-Saharan Africa, and Southern Latin America—showed positive EAPC values, whereas the remaining 14 GBD regions exhibited negative trends. Eastern Europe recorded the most significant declines, with EAPC values of -3.66% (95% CI: -3.97%, -3.36%) for ASMR and − 2.57% (95% CI: -2.75%, -2.39%) for ASDR (Fig. 2 and Supplementary Table 3).

Fig. 2.

Fig. 2

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The EAPCs of ASPR (a), ASIR (b), ASMR (c), and ASDR (d) for CAKUT across the 21 GBD regions, from 1990 to 2021. Red indicates positive EAPC values and blue indicates negative EAPC values. EAPC, estimated annual percentage change; ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate

Regional trends by the SDI

In 2021, CAKUT prevalence was highest in the low-middle SDI region, with 987,898 (95% UI: 768661, 1274289) cases in males and 1,018,236 (95% UI: 807992, 1282828) in females. The high SDI region had the lowest prevalence, with 228,956 (95% UI: 184013, 281819) cases in males and 302,979 (95% UI: 250027, 360979) in females. In terms of incidence, the low-middle SDI region had the most cases (183421 [95% UI: 135901, 245776] males and 176231 [95% UI: 131100, 235270] females), whereas the high SDI region had the fewest cases (33805 [95% UI: 25729, 43301] males and 37430 [95% UI: 28801,48000] females). Mortality was also highest in the low-middle SDI region, with 1871 (95% UI: 844, 3642) male deaths and 946 (95% UI: 498, 1665) female deaths. DALYs followed a similar trend, with the largest burden in low-middle SDI regions (Fig. 3 and Supplementary Table 4).

Fig. 3.

Fig. 3

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The prevalence (a), ASPR (b), incidence (c), ASIR (d), deaths (e), ASMR (f), DALYs (g) and ASDR (h) for CAKUT among different sexes, stratified by SDI, in 2021. ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate; SDI, socio-demographic index

On the other end of the spectrum, the low SDI region exhibited the highest ASPR, with rates of 100.36 (95% UI: 79.19, 126.15) for males and 109.75 (95% UI: 86.13, 137.70) for females. Interestingly, a notable sex-based disparity in ASPR was observed predominantly in high-middle SDI regions, where the rates were 60.01 (95% UI: 48.06, 74.17) for males and 98.76 (95% UI: 79.82, 120.94) for females. Similarly, the low SDI region recorded the highest ASIR, reaching 19.67 (95% UI: 14.70, 26.10) in males and 19.94 (95% UI: 14.87, 26.47) in females. Furthermore, with respect to mortality, the low-middle SDI region had the highest ASMR for both sexes, with 0.19 (95% UI: 0.09, 0.38) for males and 0.10 (95% UI: 0.05, 0.18) for females. The high-middle SDI region had the lowest ASMR, with rates of 0.09 (95% UI: 0.06, 0.13) for males and 0.05 (95% UI: 0.03, 0.08) for females. Finally, the ASDR was also highest in the low-middle SDI region, where males had an ASDR of 20.83 (95% UI: 11.07, 37.61), and females had an ASDR of 12.97 (95% UI: 8.20, 21.29). And the high-middle SDI region also had the lowest ASDR, at 9.84 (95% UI: 6.84, 13.52) for males and 8.06 (95% UI: 5.70, 11.42) for females (Fig. 3 and Supplementary Table 5).

Between 1990 and 2021, the EAPCs showed substantial variation across five SDI regions. Regarding the ASPR, the most notable increase was observed in middle SDI region, with an EAPC of 0.16% (95% CI: 0.13%, 0.19%). In contrast, high-middle SDI region experienced the most significant decline in ASPR, with an EAPC of -0.16% (95% CI: -0.18%, -0.14%). For all five SDI regions, the ASIR, ASMR, and ASDR exhibited decreasing trends. Among them, the most pronounced reduction in ASIR occurred in high SDI region, with an EAPC of -0.57% (95% CI: -0.74%, -0.40%). The most significant declines in ASMR and ASDR were observed in high-middle SDI region, with EAPCs of -2.27% (95% CI: -2.46%, -2.08%) and − 1.81% (95% CI: -1.96%, -1.67%), respectively (Supplementary Fig. 3 and Supplementary Table 6).

National trends

In 2021, there was marked variation in the burden of CAKUT across 204 countries and territories. Botswana and South Africa recorded the highest ASPR at 166.81 (95% UI: 129.40, 208.57) and 165.63 (95% UI: 131.10, 206.68), respectively, whereas Indonesia and the Philippines had the lowest rates at 43.90 (95% UI: 34.28, 54.79) and 47.08 (95% UI: 37.19, 59.14), respectively. Concerning ASIR, South Africa and Singapore led with 30.99 (95% UI: 22.65, 41.90) and 30.33 (95% UI: 21.23, 42.66), whereas Paraguay and Ireland had the lowest ASIR at 8.08 (95% UI: 5.50, 11.13) and 8.48 (95% UI: 6.59, 10.97), respectively. Kuwait and Sudan reported the highest ASMR and ASDR, with Kuwait’s ASMR at 0.46 (95% UI: 0.26, 0.65) and ASDR at 46.24 (95% UI: 29.05, 63.20), whereas Sudan’s ASMR was 0.43 (95% UI: 0.22, 0.88) and the ASDR at 42.7 (95% UI: 23.02, 82.83) (Fig. 4 and Supplementary Table 7).

Fig. 4.

Fig. 4

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The ASPR (a), ASIR (b), ASMR (c), and ASDR (d) of CAKUT across 204 countries and territories in 2021. ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate

Correlations of age-standardized rates (ASRs) with the SDI

When the CAKUT metrics across 21 GBD regions were investigated, a nuanced correlation with the SDI emerged. Notably, there was a significant negative correlation between the ASPR and SDI (R = -0.25, P < 0.01), as well as between the ASIR and SDI (R = -0.38, P < 0.01). Conversely, the ASMR exhibited a negligible and nonsignificant slight negative association with the SDI (R = -0.05, P = 0.21). Additionally, a marginally significant weak negative correlation was found between the ASDR and SDI (R = -0.09, P = 0.01) (Fig. 5). Similarly, a broader analysis across 204 countries and territories confirmed significant negative correlations between the SDI and ASPR (R = -0.27, P < 0.01), ASIR (R = -0.35, P < 0.01), ASMR (R = -0.19, P < 0.01), and ASDR (R = -0.24, P < 0.01) (Supplementary Fig. 4).

Fig. 5.

Fig. 5

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Spearman correlation analysis between the SDI and ASPR (a), ASIR (b), ASMR (c), and ASDR (d) for CAKUT across 21 GBD regional levels from 1990 to 2021. ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate; SDI, socio-demographic index

Drivers of CAKUT epidemiology: population growth, aging, and epidemiologic changes

The decomposition analysis was performed across different genders and various SDI regions (Figs. 6 and 7). It is evident that between 1990 and 2021, population growth was the predominant contributor to the increase in CAKUT prevalence across both genders. Aging was identified as the primary driver behind the decline in CAKUT incidence. Epidemiological changes were the main factor responsible for the reduction in deaths, particularly among females. The synergistic effects of aging and epidemiological changes contributed to the decrease in CAKUT DALYs (Fig. 6).

Fig. 6.

Fig. 6

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Decomposition analysis of CAKUT prevalence (a), incidence (b), deaths (c), and DALYs (d) from 1990 to 2021, by sex. The black dot represents the overall change value of population growth, aging, and epidemiological change. DALYs, disability-adjusted life years

Fig. 7.

Fig. 7

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Decomposition analysis of CAKUT prevalence (a), incidence (b), deaths (c), and DALYs (d) from 1990 to 2021, by SDI. The black dot represents the overall change value of population growth, aging, and epidemiological change. DALYs, disability-adjusted life years

In the context of various SDI regions, population growth was the chief contributor to the increase in CAKUT prevalence, incidence, deaths, and DALYs within low SDI regions. Aging served as the principal cause of the decline in CAKUT incidence in other regions. Furthermore, epidemiological changes were the dominant factors leading to the marked reduction in deaths and DALYs in the high-middle and high SDI regions (Fig. 7).

BAPC prediction of CAKUT burden

From 2022 to 2036, the prevalence of CAKUT is predicted to continue increasing, a trend also reflected in the ASPR. Regarding incidence, the ASIR steadily declined from 1990 to 2019 but rebounded between 2019 and 2021. BAPC projections suggest that the incidence numbers and ASIR will continue to rise through 2036 (Fig. 8).

Fig. 8.

Fig. 8

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Projected numbers and ASRs of prevalence (a), incidence (b), deaths (c), and DALYs (d) for CAKUT among different sexes, from 1990 to 2036 based on the BAPC model. ASPR, age-standardized prevalence rate; ASIR, age-standardized incidence rate; ASMR, age-standardized mortality rate; ASDR, age-standardized disability-adjusted life year rate; DALYs, disability-adjusted life years

In terms of mortality and DALYs related to CAKUT, BAPC projections indicate that from 2022 to 2036, both male and female mortality, along with the ASMR, will continue to decline. Similarly, DALYs and ASDR for both sexes are expected to decrease steadily over the same period (Fig. 8).

Discussion

From 1990 to 2021, the global prevalence of CAKUT cases increased by 21.50%, reaching 6.34 million cases, whereas the ASPR experienced a modest rise of 3.96%. In contrast, the absolute incidence decreased by 6.25%, accompanied by a 2.86% decrease in the ASIR. Mortality dropped by 22.56%, and the ASMR decreased by 20.00%. Similarly, DALYs declined by 15.44%, alongside an 18.46% reduction in the ASDR. In 2021, Southern Sub-Saharan Africa recorded the highest ASPR and ASIR, while Central Latin America recorded the highest ASMR, and Southern Latin America had the highest ASDR. Additionally, ASPR, ASIR, ASMR, and ASDR were all negatively correlated with SDI. Decomposition analysis identified population growth as the primary contributor to the rising CAKUT prevalence. Furthermore, BAPC projections indicate that from 2022 to 2036, ASPR and ASIR will continue to increase, while ASMR and ASDR are expected to decline.

The 21.50% increase in global CAKUT cases from 1990 to 2021, alongside a modest 3.96% increase in ASPR. Decomposition analysis revealed that population growth was the main contributor to this increase, whereas epidemiological changes mitigated this increase. This suggests that improvements in public health and advancements in healthcare, such as the development of ultrasound diagnostics [23, 24], have helped curb the increase in CAKUT prevalence. Decomposition analysis showed that ASMR and ASDR declined in both sexes, primarily due to epidemiological improvements. The APC for ASMR was − 1.69% from 2013 to 2019, which accelerated to -2.90% between 2019 and 2021. Similarly, the APC for DALYs was − 1.49% from 2013 to 2021. These trends highlight the positive impact of healthcare interventions [25–29], such as early fetal interventions, in improving outcomes for patients with fetal hydronephrosis [25]. More recently, advances in robot-assisted surgery have further contributed to shorter recovery times and better postoperative outcomes in CAKUT patients [30–32]. Despite these advancements, CAKUT remains a major cause of pediatric renal failure, which in severe cases necessitates kidney replacement therapy (KRT) for survival [33, 34]. However, the high costs and limited availability of KRT pose significant challenges, particularly in resource-limited settings such as low SDI regions and remote areas [34, 35]. Therefore, developing cost-effective KRT options is essential to enhancing its accessibility, particularly in underserved regions.

Across the five SDI regions, the low and low-middle SDI regions presented the higher ASPR, ASIR, ASMR, and ASDR. The relationship between the CAKUT burden and SDI is notably negative, particularly regarding CAKUT prevalence and incidence, emphasizing the positive impact of higher SDI levels in reducing these rates. Regions with higher SDI levels likely benefit from advances in early diagnosis, treatment, and surgical techniques [24, 36]. Previous study has shown that the use of multiple ultrasound features improves prenatal CAKUT assessments [24], and the combination of whole-exome sequencing (WES) with karyotype analysis and copy number variation (CNV) has enhanced prenatal diagnosis, particularly for high-risk fetuses with inconclusive ultrasound results [37]. Early diagnosis of severe CAKUT during pregnancy, along with timely pregnancy termination, also contributes to reducing incidence rates [38]. A deeper understanding of CAKUT epidemiological risk factors, coupled with the implementation of targeted interventions, has played a crucial role in decreasing CAKUT prevalence and incidence [7, 39–42]. Furthermore, proactive healthcare interventions have been shown to significantly lower CAKUT-related mortality and DALYs [43]. Minimally invasive surgeries, including laparoscopic and robot-assisted procedures, have become widely adopted for CAKUT treatment, especially in developed countries [44, 45]. In contrast, financial constraints, limited healthcare budgets, and a lack of training programs hinder the implementation of robot-assisted surgeries in lower SDI regions [46]. Interestingly, the CAKUT ASMR in low SDI regions was found to be comparable to that in high SDI regions. This unexpected finding may be attributed to incomplete or non-existent civil registration and vital statistics systems in low SDI regions [47], leading to potential underestimation or misclassification of CAKUT-related deaths.

Notably, our findings are consistent with other studies based on GBD 2019 data [14, 15], showing that females have lower ASMR and ASDR compared to males. This disparity may be partly attributed to the renoprotective effects of estrogen, which has been shown to slow the progression of kidney disease [48]. Furthermore, males tend to progress to ESRD earlier, possibly due to greater exposure to adverse factors such as physical and psychological stress, higher rates of smoking, and less favorable nutritional profiles [48, 49]. Additionally, the disease spectrum of CAKUT differs by sex. Severe anomalies such as posterior urethral valves, which are exclusive to males and associated with early-onset renal dysfunction, may further contribute to the higher ASMR and ASDR observed in males [50, 51].

The observed sex-based difference in ASPR within high-middle SDI regions may be attributed to several factors. First, certain CAKUT subtypes—such as ureteral duplication, ectopic ureter, and ureterocele—have been reported to occur more frequently in females, potentially contributing to this disparity [52, 53]. Second, greater healthcare accessibility and utilization in high-middle SDI regions may lead to more frequent diagnostic evaluations among female patients, thereby increasing the likelihood of detection. Cultural, genetic, or biological differences in disease presentation or care-seeking behavior between sexes may also play a role. However, further investigation is warranted to fully elucidate these patterns.

Some countries, such as Russia, demonstrated relatively high ASPR and ASIR of CAKUT, while maintaining comparatively low ASMR and ASDR. This pattern may be attributed to early detection and early intervention. The widespread implementation of prenatal and neonatal screening programs in these settings likely contributes to increased ASPR and ASIR [54–56]. In parallel, timely access to surgical intervention and nephrological care may effectively reduce ASMR and ASDR.

BAPC projections reveal that over the next 15 years, CAKUT-related mortality and DALYs, along with their ASRs, are expected to continue to decline, reflecting advancements in CAKUT management, surgical techniques, and medical treatments. However, CAKUT prevalence and incidence, as well as their respective ASPR and ASIR, are projected to rise steadily over the next 15 years, indicating that CAKUT will remain a significant public health challenge. The rebound in ASIR, which began in 2019, is expected to persist through 2036, highlighting the need for further investigation into CAKUT etiology to identify more effective preventive strategies.

These findings have important public health implications, particularly for regions where the burden of CAKUT remains high, such as Southern Sub-Saharan Africa, Central Latin America, and other low SDI regions. Expanding access to routine prenatal and neonatal ultrasound screening could facilitate early diagnosis and timely intervention [24, 36]. Increased investment in healthcare infrastructure to increase the availability of surgical services and KRT could contribute to reductions in CAKUT-related mortality and DALYs [25, 26, 30–32]. Multidisciplinary care involving nephrologists, urologists, nurses, dietitians, and social workers may further enhance clinical management and improve long-term outcomes in individuals with CAKUT [50]. Additionally, family planning initiatives in high-burden regions could be instrumental in alleviating its impact [57]. Overall, CAKUT remains a global public health issue that requires enhanced cooperation among countries, the sharing of experiences, and a collective response to common challenges.

Our study also has some inevitable limitations. First, as a descriptive study, GBD analysis cannot establish causal relationships, and its reliance on raw data that vary in quality across regions may introduce biases or inaccuracies [11]. Second, the GBD 2021 study grouped all CAKUT cases into a single category of urogenital congenital anomalies, preventing a more detailed analysis of different CAKUT subtypes. Despite these limitations, the GBD 2021 study remains a valuable resource for understanding the current disease burden and comparing global CAKUT trends.

Conclusions

In summary, this study demonstrates that from 1990 to 2021, CAKUT prevalence rose globally, while incidence, mortality, and DALYs declined. By 2036, CAKUT prevalence and incidence are expected to rise, highlighting the need for global collaboration to enhance screening, prevention, and treatment, particularly in high-burden regions such as Southern Sub-Saharan Africa, Central Latin America, and Southern Latin America.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary Material 1 (4.6MB, docx)

Acknowledgements

Thanks to the Institute for Health Metrics and Evaluation (IHME), and the Global Burden of Disease study collaborations.

Abbreviations

AAPC

Average annual percentage change

APC

Annual percentage change

ASDR

Age-standardized DALY rate

ASIR

Age-standardized incidence rate

ASMR

Age-standardized mortality rate

ASPR

Age-standardized prevalence rate

ASRs

Age-standardized rates

BAPC

Bayesian age-period-cohort

CAKUT

Congenital anomalies of the kidney and urinary tract

CI

Confidence interval

CKD

Chronic kidney disease

CNV

Copy number variation

CODEm

Cause of Death Ensemble Model

DALYs

Disability-adjusted life years

DisMod-MR 2.1

Disease Modelling Meta-Regression; version 2.1

EAPC

Estimated annual percentage change

ESRD

End-stage renal disease

GBD

Global Burden of Disease

GHDx

Global Health Data Exchange

SDI

Socio-demographic index

UI

Uncertainty interval

WES

Whole-exome sequencing

YLDs

Years lived with disability

YLLs

Years of life lost

Author contributions

T.C. conceptualized the study, curated data, conducted formal analysis, developed the methodology, performed visualization, and wrote the original draft. J.W. contributed to data curation, validation, and manuscript review & editing. Q.S. provided conceptualization, supervision, and manuscript review & editing. X.Y. supervised the study and contributed to manuscript review & editing.

Funding

Not applicable.

Data availability

The data from this study can be accessed openly through the GBD 2021 online database, as outlined in the Methods section.

Declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Not applicable.

Competing interests

The authors declare no competing interests.

Footnotes

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Supplementary Materials

Supplementary Material 1 (4.6MB, docx)

Data Availability Statement

The data from this study can be accessed openly through the GBD 2021 online database, as outlined in the Methods section.

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