- Email: [email protected]
6. Pediatric ESRD
Q
Q u i c k l y t a ke me up into the bright child of yo u r m i n d . E.E. CUMMINGS, The Enormous Room
mSIX PEDIATrIC ESrD
American Journal of Kidney Diseases, Vol 38, No 4, Suppl 3 (October), 2001: pp S107–S116
06 pediatrics ajkd '01.p65
107
7/20/01, 8:04 AM
S 1 0 8
2 0 01 A T L A S
O F
I
ncident rates of ESRD in children have risen two to three percent over the past decade, and the odds of developing ESRD are as much as 2.2 times greater in some states than in others (fig 6.2). The primary causes of ESRD in pediatric patients are glomerulonephritis and cystic/congenital/hereditary diseases. Boys have a higher likelihood of renal failure from these latter causes than do girls (fig 6.4).
E S R D
I N
T H E
U N I T E D
received a transplant. Some of these disparities may be partially explained by the availability of organs for renal transplants, as demonstrated in Chapter Seven (figs 7.1 and 7.3). Since ESRD incidence rates are two to three times higher for black children than for whites, the organ donation rates from the general population would need to be at a comparable rate for there to be a similar proportion of transplants. The ability to match an organ immunologically may also be different in black children compared to whites.
The modalities for treatment of pediatric ESRD vary with patient age and particularly with race (figs 6.9– Most young patients with a first 10). White pediatric patients are more renal transplant receive that translikely to receive a renal transplant plant from a living donor, a pattern than patients in other racial groups, opposite to that in the adult and non-whites are twice as likely to population. Children with be on hemodialysis as whites. After a repeat renal transt 1999 two years on ESRD treatment black plant, in con1997-revAlen6 P 1,21 children have an almost equal chance trast, ent 1 d i c n of being treated with either dialysis 2,58 96 nt I 529 9 2 1 1 or transplant whereas more 4,8 1994 evAle 2 423 7 r ,26 P 8,74 than 60% of white 0 1 nt 84 e 8 d i 6 Inc 502 6 children 2,4 1,61 93 lent 53 9 1 4 , 1 4 have 2 21.0 199 evA 45 1
Pr 1,165 ent 8 d i c In 493 2,25 s t 8 n 0 3,7 Cou 380
785 4 1,64
8,22
9.1 7.0 4 1 .2 26.5
42.4 81.1 .1 144
21.3 4 0 1 4 7, 42.0 0686 8.4 n 9 3 76.9 tio 57.7 1,42 opulA .6 14 .2 0 1 19 144 Table 6.1 ion p 13.4 l 19 1 l . i 5 0 1 4 Incident & prevalent counts & rates .4 r m 8.3 8 e 2 p s 66.8 For patients younger than ten there was little RAte 6.8 3.7 4 3 variation in incident and prevalent rates per million 1 5 . 012 population through the 1990s. For older pediatric patients, in 5- 9 26.3 4 contrast, prevalent rates have risen, possibly a reflection of improved 1 0 1 survival after transplantation (see Chapter Seven). 19 15-
06 pediatrics ajkd '01.p65
108
S TAT E S
are more likely to receive a cadaveric organ (fig 6.11). Pediatric patients younger than five have a 93% survival rate after transplantation in contrast to a 69% rate for children of the same age on dialysis (fig 6.12). Other pediatric age groups have five-year survivals on dialysis of greater than 81% (fig 6.12). Hospitalizations for children are directly related to age (figs 6.15–17) and are dominated by infectious complications. Although the number of prevalent deaths is low, female children are generally at a greater risk than males regardless of modality (figs 6.18–20). The reasons for these differences have not been explored, but may be related to a higher degree of anemia in girls than in boys. At initiation of dialysis children have lower hematocrits than adults (fig 2.39), with female children having the lowest levels. These findings are present even though children are the most likely to receive EPO before ESRD, as documented on the Medical Evidence form 2728 (fig 2.25). These associations suggest that girls may be iron deficient to a greater degree than boys or adults. Also, the prolonged and more severe anemia may predispose these children to a greater risk of myocardial hypertrophy and subsequent fibrosis, which could place them at risk for arrhythmic disorders. Further investigations are needed to more completely understand the pathobiology of these patients.
7/20/01, 8:04 AM
C H A P T ER 6 P E D I A T R I C
E S R D S 1 0 9
15-19 30
25
RAte per million populAtion
0-4
20
10-14
15 5-9 10
5 1990
199 1
1992
1993
1994
1995
1996
1997
1998
1999
Figure 6.1 Incident rates, by age
per million population, adjusted for gender & race
Incident rates of ESRD for pediatric patients aged 0–4 increased slightly from 1990 to 1999, as did the rates for children aged 10–14.
Figure 6.2 Incident rates, ages 0–19 per million population, 1995–1999 combined, adjusted for age, gender, & race, by state
15.7+ (20.3) 14.3 to <15.7 13.2 to <14.3 11.6 to <13.2 below 11.6 (9.3)
Included in tHis cHApter
The mean incident rate varies by 118% between the lowest and highest quintiles.
♦ A graph of incident rates, and graphs of patient distribution by modality, primary diagnosis, gender, race, and
age ♦ Graphs showing treatment modality two years following the onset of ESRD ♦ A graph of the number of first and repeat transplants, and Kaplan-Meier survival curves ♦ Graphs of hospital admissions for infection, by modality, age, and gender ♦ Graphs of causes of death by gender and race
06 pediatrics ajkd '01.p65
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7/20/01, 8:04 AM
S 1 1 0
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.3 Incident rates, by race, age, & gender 1997–1999 combined, unadjusted 70 60 Rate per million population
Black pediatric patients across all age groups have higher incident rates of end-stage renal disease compared to patients in other racial groups, with the highest incident rates among pediatric patients occurring in blacks aged 15–19.
Ages 0-4
Ages 5-9
Ages 10-14
Ages 15-19
Male Female
50 40 30 20 10 0 White Black
N Am Asian
White Black
N Am Asian
White Black
N Am Asian
White Black
N Am Asian
Figure 6.4 Number of patients within primary diagnosis group, by gender: dialysis 900
incident patients aged 0–19, 1995–1999 combined
Male Female
700 Number of patients
Glomerulonephritis and cystic/ hereditary/congenital diseases are the most frequent primary diagnoses in the pediatric ESRD population, with both occurring more in boys than girls.
800
600 500 400 300 200 100 0 Diabetes
Glomerulonephritis (GN)
Secondary GN/ vasculitis
Interstitial nephritis
Hypertension Cystic/hereditary/ congenital
Neoplasm/ cancer
Glomerulonephritis (GN)
Secondary GN/ vasculitis
Interstitial nephritis
Hypertension Cystic/hereditary/ congenital
Neoplasm/ cancer
Figure 6.5 Number of patients within primary diagnosis group, by gender: transplant 320
incident patients aged 0–19, 1995–1999 combined
280
Male Female
240 Number of patients
In the pediatric patients who receive a transplant as their initial modality, cystic/hereditary/ congenital diseases are the primary cause of ESRD, and occur in more than twice as many boys as girls.
200 160 120 80 40 0 Diabetes
06 pediatrics ajkd '01.p65
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C H A P T ER 6 P E D I A T R I C
E S R D S 1 1 1 Figure 6.6 Distribution of primary diagnosis within racial group: dialysis
Percent of patients with diagnosis
60
Diabetes Glomerulonephritis Secondary GN/vasculitis Interstitial nephritis/pyelonephritis
50
incident patients aged 0–19, 1995–1999 combined
Hypertension Cystic/hereditary/congenital Neoplasms/cancer
Glomerulonephritis is the most frequent primary diagnosis in black, Native American, and Asian pediatric patients. Cystic/ hereditary/congenital diseases occur most often in young white and Native American patients, while Asian children are almost twice as likely than children of other races to have a primary diagnosis of secondary glomerulonephritis/vasculitis.
40 30 20 10 0 White
Black
Native American
Asian
Figure 6.7 Distribution of primary diagnosis within racial group: transplant
Percent of patients with diagnosis
70
incident patients aged 0–19, 1995–1999 combined
Hypertension Cystic/hereditary/congenital Neoplasms/tumors
Diabetes Glomerulonephritis Secondary GN/vasculitis Interstitial nephritis/pyelonephritis
80
Cystic/hereditary/congenital disease is by far the most frequent diagnosis among pediatric patients who receive a transplant at the beginning of ESRD. No Native American or Asian patients with diabetes, secondary glomerulonephritis/ vasculitis, interstitial nephritis/ pyelonephritis, or neoplasms/ tumors were reported.
60 50 40 30 20 10 0 White
Black
Native American
Asian
Figure 6.8 Gender distribution within primary diagnosis group incident patients aged 0–19, 1995–1999 combined
Percent of patients with diagnosis
80
Dialysis
Transplant
Diabetes, glomerulonephritis, secondary glomerulonephritis, interstitial nephritis/pyelonephritis, hypertension, cystic/hereditary/congenital diseases, and neoplasms/tumors are abbreviated along the axis.
Male Female
70 60 50 40 30 20 10 0 DM
06 pediatrics ajkd '01.p65
GN
SGN
111
Inst.
HTN
Cyst.
Neopl.
DM
GN
SGN
Inst.
HTN
7/20/01, 8:04 AM
Cyst.
Neopl.
Distribution within primary diagnosis categories differs greatly between the modalities. Girls account for 61% of diabetic children on dialysis, and 68% of those with secondary glomerulonephritis. Among transplant patients, however, boys account for 75% of diabetics, and the population with secondary glomerulonephritis is split evenly between the genders.
S 1 1 2
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.9 Treatment modality two years following ESRD onset, within age group 100
incident patients aged 0–19, 1995–1997 combined
Hemodialysis Peritoneal dialysis Transplant Death
90 80 70
Percent of patients
At two years following the onset of ESRD, transplant is by far the most frequent modality among all pediatric age groups. Among patients aged 5–9, 72% have received a transplant; the number is 47%, however, in patients aged 15–19, and 36% of patients in this age group are on hemodialysis two years after beginning ESRD.
60 50 40 30 20 10 0 0-4
5-9
10-14
15-19
Black
Native American
Asian
Figure 6.10 Treatment modality two years following ESRD onset, within race group 100
incident patients aged 0–19, 1995–1997 combined
Hemodialysis Peritoneal dialysis Transplant Death
90 80 70
Percent of patients
White children are most likely to have received a transplant by the end of their second year of ESRD, followed by Native Americans. The percentage of black and Asian children with a transplant at two years is only slightly higher than that of their peers on hemodialysis.
60 50 40 30 20 10 0 White
Figure 6.11 Total first & repeat transplants, by donor source incident patients aged 0–19
First transplantation rates among pediatric patients have been relatively steady for transplants from living donors, while the rate of cadaveric transplants declined from 1994 to 1998. Both rates increased in 1999. The number of living unrelated transplants in pediatric patients is extremely small, and reduced organ availability limits the number of repeat transplants in these patients.
06 pediatrics ajkd '01.p65
500
First transplants
150
450
Repeat transplants
Living (365, 22.7%) 125
400 Number of transplants
Includes patients not eligible for Medicare enrollment. The number of transplants in 1994, and the percent change between 1994 and 1999, are indicated next to the lines.
350
100
Cadaver (380, -5.5%)
Cadaver (109, -24.8%)
300 75
250 200
50
150
Living (28, -3.6%)
100
25
50 0 1994
112
1995
1996
1997
1998
1999
1994
1995
1996
7/20/01, 8:04 AM
1997
1998
1999
C H A P T ER 6 P E D I A T R I C
Dialysis
Figure 6.12 Kaplan-Meier five-year patient survival, by age
First transplant
incident dialysis & transplant patients, 1993–1994 combined
100
By five years after the start of ESRD, 69% of children whose ESRD began between birth and age 4 are still alive on dialysis, compared to 82% of children in other age groups. For patients who receive their first transplant, survival is best for those aged 10–14 years and lowest for those aged 0–4 years. All age groups, however, have over 93% survival at five years.
Percent surviving
90
80
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
70
E S R D S 1 1 3
60 0
12
24
36
48
60
0
12
24
36
48
60
Months of survival
100
White
Figure 6.13 Kaplan-Meier five-year patient survival after first transplantation, by race & gender: cadaveric transplants
Black
1993–1994 combined
Percent surviving
Because of the small number of patients, data are not shown for Native American or Asian patients.
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
90
The lowest five-year survival probabilities for cadaveric transplants occur in black and male patients aged 0–4. Overall, children aged 10–14 have the highest probabilities of survival across racial and gender groups.
80
Female
Male 100
90
80 0
12
24
36
48
60
0
12
24
36
48
60
Months of survival
100
White
Figure 6.14 Kaplan-Meier five-year patient survival after first transplantation, by race & gender: living donor transplants
Black
1993–1994 combined
Percent surviving
Because of the small number of patients, data are not shown for Native American or Asian patients.
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
90
Survival probabilities for pediatric patients receiving transplants from living donors are similar across age groups for whites and for males.
80 100
Male
Female
90
80 0
12
24
36
48
60
0
12
24
36
48
Months of survival
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60
S 1 1 4 2 0 0 1 A T L A S O F E S R D I N T H E U N I T E D S TAT E S Figure 6.15 Admissions for infection (overall), by age, gender, & time on ESRD: hemodialysis
The rates of admissions for infection vary little between the genders. Among age groups, however, rates are highest for children younger than ten years of age who have had ESRD for less than one year, and for children aged 5–9 who have had ESRD less than five years.
140 Age Admissions per 100 patient years at risk
incident & prevalent hemodialysis patients,1997– 1999 combined
Gender < 1 year 1 to < 2 years 2 to <5 years 5+ years
120 100 80 60 40 20 0 0-4
Figure 6.16 Admissions for infection (overall), by age, gender, & time on ESRD: peritoneal dialysis
In all age and gender groups except ages 5–9, pediatric patients on peritoneal dialysis have higher rates of admission for infection than their counterparts on hemodialysis. Rates are highest for the youngest patients (ages 0–4), and for children aged 5–9 who have had ESRD for more than five years. Females have slightly higher admission rates than males, and these rates increase with longer patient vintages.
Male
Female
Male
Female
Male
Female
Gender
100 80 60 40 20 0 0-4
5-9
10-14
15-19
80 Age Admissions per 100 patient years at risk
Transplant-related admissions for infection in pediatric patients are the lowest within all modalities, at about half of those of the other dialytic therapies. Age is a significant factor, with transplant patients younger than ten having higher infectious hospitalization rates than their older counterparts.
15-19
< 1 year 1 to < 2 years 2 to <5 years 5+ years
120
Figure 6.17 Admissions for infection (overall), by age, gender, & time on ESRD: transplant incident & prevalent transplant patients, 1997–1999 combined
10-14
140 Age Admissions per 100 patient years at risk
incident & prevalent peritoneal dialysis patients, 1997–1999 combined
5-9
Gender
70 < 1 year 1 to < 2 years 2 to <5 years 5+ years
60 50 40 30 20 10 0 0-4
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5-9
10-14
15-19
7/20/01, 8:04 AM
C H A P T ER 6 P E D I A T R I C
E S R D
S 1 1 5
Figure 6.18 Causes of death, by gender: hemodialysis prevalent patients aged 0–19, 1997–1999 combined
Deaths per 1,000 patient years at risk
14 12
Male Female
The “other known” category accounts for the highest number of deaths in pediatric patients on hemodialysis. Death rates are higher for females than for males in this category, “cardiac other,” and infectious death.
10 8 6 4 2 0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.19 Causes of death, by gender: peritoneal dialysis
Deaths per 1,000 patient years at risk
20
prevalent patients aged 0–19, 1997–1999 combined
Male Female
Female patients on peritoneal dialysis have a more than fourfold difference in rates of death due to cardiac arrest compared to their male counterparts.They also have higher rates of death due to infection and to other cardiac and other known causes.
16
12
8
4
0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.20 Causes of death, by gender: transplant prevalent patients aged 0–19, 1997–1999 combined
Deaths per 1,000 patient years at risk
4 Male Female 3
2
1
0
06 pediatrics ajkd '01.p65
While mortality rates for pediatric patients with transplants are low, girls again have higher rates than boys in many categories. Males do have higher rates of mortality due to infection and other known causes.
Cardiac arrest
115
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
7/20/01, 8:04 AM
Unknown
Transplant centers frequently do not receive notification about the deaths of patients who are followed by the primary care doctors. Because graft survival is longest in pediatric patients, causes of death for these patients are frequently unreported.
S 1 1 6
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.21 Causes of death, by race: hemodialysis prevalent patients aged 0–19, 1997–1999 combined
The rate of mortality due to cardiac arrest is almost three times higher among black pediatric patients on hemodialysis than in their white counterparts. White patients, however, have slightly higher rates of death due to infection and to other cardiac and other known causes.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
15 White Black 12
9
6
3
0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.22 Causes of death, by race: peritoneal dialysis prevalent patients aged 0–19, 1997–1999 combined
In contrast to rates in the hemodialysis population, rates of mortality due to cardiac arrest show little difference between the races for pediatric patients on peritoneal dialysis. Mortality due to infection or to other known causes is more frequent among black patients, while deaths related to cerebrovascular disease or to other cardiac events occur more often in white patients.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
15 White Black 12
9
6
3
0 Cardiac arrest
Figure 6.23 Causes of death, by race: transplant prevalent patients aged 0–19, 1997–1999 combined
Black patients have higher mortality rates on transplant for all causes of death except “other known;” in several categories (cardiac other, cerebrovascular disease, and infection) rates for blacks are more than twice as high as those for whites.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
4 White Black 3
2
1
0 Cardiac arrest
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Q u i c k l y t a ke me up into the bright child of yo u r m i n d . E.E. CUMMINGS, The Enormous Room
mSIX PEDIATrIC ESrD
American Journal of Kidney Diseases, Vol 38, No 4, Suppl 3 (October), 2001: pp S107–S116
06 pediatrics ajkd '01.p65
107
7/20/01, 8:04 AM
S 1 0 8
2 0 01 A T L A S
O F
I
ncident rates of ESRD in children have risen two to three percent over the past decade, and the odds of developing ESRD are as much as 2.2 times greater in some states than in others (fig 6.2). The primary causes of ESRD in pediatric patients are glomerulonephritis and cystic/congenital/hereditary diseases. Boys have a higher likelihood of renal failure from these latter causes than do girls (fig 6.4).
E S R D
I N
T H E
U N I T E D
received a transplant. Some of these disparities may be partially explained by the availability of organs for renal transplants, as demonstrated in Chapter Seven (figs 7.1 and 7.3). Since ESRD incidence rates are two to three times higher for black children than for whites, the organ donation rates from the general population would need to be at a comparable rate for there to be a similar proportion of transplants. The ability to match an organ immunologically may also be different in black children compared to whites.
The modalities for treatment of pediatric ESRD vary with patient age and particularly with race (figs 6.9– Most young patients with a first 10). White pediatric patients are more renal transplant receive that translikely to receive a renal transplant plant from a living donor, a pattern than patients in other racial groups, opposite to that in the adult and non-whites are twice as likely to population. Children with be on hemodialysis as whites. After a repeat renal transt 1999 two years on ESRD treatment black plant, in con1997-revAlen6 P 1,21 children have an almost equal chance trast, ent 1 d i c n of being treated with either dialysis 2,58 96 nt I 529 9 2 1 1 or transplant whereas more 4,8 1994 evAle 2 423 7 r ,26 P 8,74 than 60% of white 0 1 nt 84 e 8 d i 6 Inc 502 6 children 2,4 1,61 93 lent 53 9 1 4 , 1 4 have 2 21.0 199 evA 45 1
Pr 1,165 ent 8 d i c In 493 2,25 s t 8 n 0 3,7 Cou 380
785 4 1,64
8,22
9.1 7.0 4 1 .2 26.5
42.4 81.1 .1 144
21.3 4 0 1 4 7, 42.0 0686 8.4 n 9 3 76.9 tio 57.7 1,42 opulA .6 14 .2 0 1 19 144 Table 6.1 ion p 13.4 l 19 1 l . i 5 0 1 4 Incident & prevalent counts & rates .4 r m 8.3 8 e 2 p s 66.8 For patients younger than ten there was little RAte 6.8 3.7 4 3 variation in incident and prevalent rates per million 1 5 . 012 population through the 1990s. For older pediatric patients, in 5- 9 26.3 4 contrast, prevalent rates have risen, possibly a reflection of improved 1 0 1 survival after transplantation (see Chapter Seven). 19 15-
06 pediatrics ajkd '01.p65
108
S TAT E S
are more likely to receive a cadaveric organ (fig 6.11). Pediatric patients younger than five have a 93% survival rate after transplantation in contrast to a 69% rate for children of the same age on dialysis (fig 6.12). Other pediatric age groups have five-year survivals on dialysis of greater than 81% (fig 6.12). Hospitalizations for children are directly related to age (figs 6.15–17) and are dominated by infectious complications. Although the number of prevalent deaths is low, female children are generally at a greater risk than males regardless of modality (figs 6.18–20). The reasons for these differences have not been explored, but may be related to a higher degree of anemia in girls than in boys. At initiation of dialysis children have lower hematocrits than adults (fig 2.39), with female children having the lowest levels. These findings are present even though children are the most likely to receive EPO before ESRD, as documented on the Medical Evidence form 2728 (fig 2.25). These associations suggest that girls may be iron deficient to a greater degree than boys or adults. Also, the prolonged and more severe anemia may predispose these children to a greater risk of myocardial hypertrophy and subsequent fibrosis, which could place them at risk for arrhythmic disorders. Further investigations are needed to more completely understand the pathobiology of these patients.
7/20/01, 8:04 AM
C H A P T ER 6 P E D I A T R I C
E S R D S 1 0 9
15-19 30
25
RAte per million populAtion
0-4
20
10-14
15 5-9 10
5 1990
199 1
1992
1993
1994
1995
1996
1997
1998
1999
Figure 6.1 Incident rates, by age
per million population, adjusted for gender & race
Incident rates of ESRD for pediatric patients aged 0–4 increased slightly from 1990 to 1999, as did the rates for children aged 10–14.
Figure 6.2 Incident rates, ages 0–19 per million population, 1995–1999 combined, adjusted for age, gender, & race, by state
15.7+ (20.3) 14.3 to <15.7 13.2 to <14.3 11.6 to <13.2 below 11.6 (9.3)
Included in tHis cHApter
The mean incident rate varies by 118% between the lowest and highest quintiles.
♦ A graph of incident rates, and graphs of patient distribution by modality, primary diagnosis, gender, race, and
age ♦ Graphs showing treatment modality two years following the onset of ESRD ♦ A graph of the number of first and repeat transplants, and Kaplan-Meier survival curves ♦ Graphs of hospital admissions for infection, by modality, age, and gender ♦ Graphs of causes of death by gender and race
06 pediatrics ajkd '01.p65
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7/20/01, 8:04 AM
S 1 1 0
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.3 Incident rates, by race, age, & gender 1997–1999 combined, unadjusted 70 60 Rate per million population
Black pediatric patients across all age groups have higher incident rates of end-stage renal disease compared to patients in other racial groups, with the highest incident rates among pediatric patients occurring in blacks aged 15–19.
Ages 0-4
Ages 5-9
Ages 10-14
Ages 15-19
Male Female
50 40 30 20 10 0 White Black
N Am Asian
White Black
N Am Asian
White Black
N Am Asian
White Black
N Am Asian
Figure 6.4 Number of patients within primary diagnosis group, by gender: dialysis 900
incident patients aged 0–19, 1995–1999 combined
Male Female
700 Number of patients
Glomerulonephritis and cystic/ hereditary/congenital diseases are the most frequent primary diagnoses in the pediatric ESRD population, with both occurring more in boys than girls.
800
600 500 400 300 200 100 0 Diabetes
Glomerulonephritis (GN)
Secondary GN/ vasculitis
Interstitial nephritis
Hypertension Cystic/hereditary/ congenital
Neoplasm/ cancer
Glomerulonephritis (GN)
Secondary GN/ vasculitis
Interstitial nephritis
Hypertension Cystic/hereditary/ congenital
Neoplasm/ cancer
Figure 6.5 Number of patients within primary diagnosis group, by gender: transplant 320
incident patients aged 0–19, 1995–1999 combined
280
Male Female
240 Number of patients
In the pediatric patients who receive a transplant as their initial modality, cystic/hereditary/ congenital diseases are the primary cause of ESRD, and occur in more than twice as many boys as girls.
200 160 120 80 40 0 Diabetes
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C H A P T ER 6 P E D I A T R I C
E S R D S 1 1 1 Figure 6.6 Distribution of primary diagnosis within racial group: dialysis
Percent of patients with diagnosis
60
Diabetes Glomerulonephritis Secondary GN/vasculitis Interstitial nephritis/pyelonephritis
50
incident patients aged 0–19, 1995–1999 combined
Hypertension Cystic/hereditary/congenital Neoplasms/cancer
Glomerulonephritis is the most frequent primary diagnosis in black, Native American, and Asian pediatric patients. Cystic/ hereditary/congenital diseases occur most often in young white and Native American patients, while Asian children are almost twice as likely than children of other races to have a primary diagnosis of secondary glomerulonephritis/vasculitis.
40 30 20 10 0 White
Black
Native American
Asian
Figure 6.7 Distribution of primary diagnosis within racial group: transplant
Percent of patients with diagnosis
70
incident patients aged 0–19, 1995–1999 combined
Hypertension Cystic/hereditary/congenital Neoplasms/tumors
Diabetes Glomerulonephritis Secondary GN/vasculitis Interstitial nephritis/pyelonephritis
80
Cystic/hereditary/congenital disease is by far the most frequent diagnosis among pediatric patients who receive a transplant at the beginning of ESRD. No Native American or Asian patients with diabetes, secondary glomerulonephritis/ vasculitis, interstitial nephritis/ pyelonephritis, or neoplasms/ tumors were reported.
60 50 40 30 20 10 0 White
Black
Native American
Asian
Figure 6.8 Gender distribution within primary diagnosis group incident patients aged 0–19, 1995–1999 combined
Percent of patients with diagnosis
80
Dialysis
Transplant
Diabetes, glomerulonephritis, secondary glomerulonephritis, interstitial nephritis/pyelonephritis, hypertension, cystic/hereditary/congenital diseases, and neoplasms/tumors are abbreviated along the axis.
Male Female
70 60 50 40 30 20 10 0 DM
06 pediatrics ajkd '01.p65
GN
SGN
111
Inst.
HTN
Cyst.
Neopl.
DM
GN
SGN
Inst.
HTN
7/20/01, 8:04 AM
Cyst.
Neopl.
Distribution within primary diagnosis categories differs greatly between the modalities. Girls account for 61% of diabetic children on dialysis, and 68% of those with secondary glomerulonephritis. Among transplant patients, however, boys account for 75% of diabetics, and the population with secondary glomerulonephritis is split evenly between the genders.
S 1 1 2
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.9 Treatment modality two years following ESRD onset, within age group 100
incident patients aged 0–19, 1995–1997 combined
Hemodialysis Peritoneal dialysis Transplant Death
90 80 70
Percent of patients
At two years following the onset of ESRD, transplant is by far the most frequent modality among all pediatric age groups. Among patients aged 5–9, 72% have received a transplant; the number is 47%, however, in patients aged 15–19, and 36% of patients in this age group are on hemodialysis two years after beginning ESRD.
60 50 40 30 20 10 0 0-4
5-9
10-14
15-19
Black
Native American
Asian
Figure 6.10 Treatment modality two years following ESRD onset, within race group 100
incident patients aged 0–19, 1995–1997 combined
Hemodialysis Peritoneal dialysis Transplant Death
90 80 70
Percent of patients
White children are most likely to have received a transplant by the end of their second year of ESRD, followed by Native Americans. The percentage of black and Asian children with a transplant at two years is only slightly higher than that of their peers on hemodialysis.
60 50 40 30 20 10 0 White
Figure 6.11 Total first & repeat transplants, by donor source incident patients aged 0–19
First transplantation rates among pediatric patients have been relatively steady for transplants from living donors, while the rate of cadaveric transplants declined from 1994 to 1998. Both rates increased in 1999. The number of living unrelated transplants in pediatric patients is extremely small, and reduced organ availability limits the number of repeat transplants in these patients.
06 pediatrics ajkd '01.p65
500
First transplants
150
450
Repeat transplants
Living (365, 22.7%) 125
400 Number of transplants
Includes patients not eligible for Medicare enrollment. The number of transplants in 1994, and the percent change between 1994 and 1999, are indicated next to the lines.
350
100
Cadaver (380, -5.5%)
Cadaver (109, -24.8%)
300 75
250 200
50
150
Living (28, -3.6%)
100
25
50 0 1994
112
1995
1996
1997
1998
1999
1994
1995
1996
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1997
1998
1999
C H A P T ER 6 P E D I A T R I C
Dialysis
Figure 6.12 Kaplan-Meier five-year patient survival, by age
First transplant
incident dialysis & transplant patients, 1993–1994 combined
100
By five years after the start of ESRD, 69% of children whose ESRD began between birth and age 4 are still alive on dialysis, compared to 82% of children in other age groups. For patients who receive their first transplant, survival is best for those aged 10–14 years and lowest for those aged 0–4 years. All age groups, however, have over 93% survival at five years.
Percent surviving
90
80
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
70
E S R D S 1 1 3
60 0
12
24
36
48
60
0
12
24
36
48
60
Months of survival
100
White
Figure 6.13 Kaplan-Meier five-year patient survival after first transplantation, by race & gender: cadaveric transplants
Black
1993–1994 combined
Percent surviving
Because of the small number of patients, data are not shown for Native American or Asian patients.
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
90
The lowest five-year survival probabilities for cadaveric transplants occur in black and male patients aged 0–4. Overall, children aged 10–14 have the highest probabilities of survival across racial and gender groups.
80
Female
Male 100
90
80 0
12
24
36
48
60
0
12
24
36
48
60
Months of survival
100
White
Figure 6.14 Kaplan-Meier five-year patient survival after first transplantation, by race & gender: living donor transplants
Black
1993–1994 combined
Percent surviving
Because of the small number of patients, data are not shown for Native American or Asian patients.
Ages 0-4 Ages 5-9 Ages 10-14 Ages 15-19
90
Survival probabilities for pediatric patients receiving transplants from living donors are similar across age groups for whites and for males.
80 100
Male
Female
90
80 0
12
24
36
48
60
0
12
24
36
48
Months of survival
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113
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60
S 1 1 4 2 0 0 1 A T L A S O F E S R D I N T H E U N I T E D S TAT E S Figure 6.15 Admissions for infection (overall), by age, gender, & time on ESRD: hemodialysis
The rates of admissions for infection vary little between the genders. Among age groups, however, rates are highest for children younger than ten years of age who have had ESRD for less than one year, and for children aged 5–9 who have had ESRD less than five years.
140 Age Admissions per 100 patient years at risk
incident & prevalent hemodialysis patients,1997– 1999 combined
Gender < 1 year 1 to < 2 years 2 to <5 years 5+ years
120 100 80 60 40 20 0 0-4
Figure 6.16 Admissions for infection (overall), by age, gender, & time on ESRD: peritoneal dialysis
In all age and gender groups except ages 5–9, pediatric patients on peritoneal dialysis have higher rates of admission for infection than their counterparts on hemodialysis. Rates are highest for the youngest patients (ages 0–4), and for children aged 5–9 who have had ESRD for more than five years. Females have slightly higher admission rates than males, and these rates increase with longer patient vintages.
Male
Female
Male
Female
Male
Female
Gender
100 80 60 40 20 0 0-4
5-9
10-14
15-19
80 Age Admissions per 100 patient years at risk
Transplant-related admissions for infection in pediatric patients are the lowest within all modalities, at about half of those of the other dialytic therapies. Age is a significant factor, with transplant patients younger than ten having higher infectious hospitalization rates than their older counterparts.
15-19
< 1 year 1 to < 2 years 2 to <5 years 5+ years
120
Figure 6.17 Admissions for infection (overall), by age, gender, & time on ESRD: transplant incident & prevalent transplant patients, 1997–1999 combined
10-14
140 Age Admissions per 100 patient years at risk
incident & prevalent peritoneal dialysis patients, 1997–1999 combined
5-9
Gender
70 < 1 year 1 to < 2 years 2 to <5 years 5+ years
60 50 40 30 20 10 0 0-4
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114
5-9
10-14
15-19
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C H A P T ER 6 P E D I A T R I C
E S R D
S 1 1 5
Figure 6.18 Causes of death, by gender: hemodialysis prevalent patients aged 0–19, 1997–1999 combined
Deaths per 1,000 patient years at risk
14 12
Male Female
The “other known” category accounts for the highest number of deaths in pediatric patients on hemodialysis. Death rates are higher for females than for males in this category, “cardiac other,” and infectious death.
10 8 6 4 2 0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.19 Causes of death, by gender: peritoneal dialysis
Deaths per 1,000 patient years at risk
20
prevalent patients aged 0–19, 1997–1999 combined
Male Female
Female patients on peritoneal dialysis have a more than fourfold difference in rates of death due to cardiac arrest compared to their male counterparts.They also have higher rates of death due to infection and to other cardiac and other known causes.
16
12
8
4
0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.20 Causes of death, by gender: transplant prevalent patients aged 0–19, 1997–1999 combined
Deaths per 1,000 patient years at risk
4 Male Female 3
2
1
0
06 pediatrics ajkd '01.p65
While mortality rates for pediatric patients with transplants are low, girls again have higher rates than boys in many categories. Males do have higher rates of mortality due to infection and other known causes.
Cardiac arrest
115
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
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Unknown
Transplant centers frequently do not receive notification about the deaths of patients who are followed by the primary care doctors. Because graft survival is longest in pediatric patients, causes of death for these patients are frequently unreported.
S 1 1 6
2 0 01 A T L A S
O F
E S R D
I N
T H E
U N I T E D
S TAT E S
Figure 6.21 Causes of death, by race: hemodialysis prevalent patients aged 0–19, 1997–1999 combined
The rate of mortality due to cardiac arrest is almost three times higher among black pediatric patients on hemodialysis than in their white counterparts. White patients, however, have slightly higher rates of death due to infection and to other cardiac and other known causes.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
15 White Black 12
9
6
3
0 Cardiac arrest
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Cardiac other
Cerebrovascular disease
Infection
Malignancy
Other known
Unknown
Figure 6.22 Causes of death, by race: peritoneal dialysis prevalent patients aged 0–19, 1997–1999 combined
In contrast to rates in the hemodialysis population, rates of mortality due to cardiac arrest show little difference between the races for pediatric patients on peritoneal dialysis. Mortality due to infection or to other known causes is more frequent among black patients, while deaths related to cerebrovascular disease or to other cardiac events occur more often in white patients.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
15 White Black 12
9
6
3
0 Cardiac arrest
Figure 6.23 Causes of death, by race: transplant prevalent patients aged 0–19, 1997–1999 combined
Black patients have higher mortality rates on transplant for all causes of death except “other known;” in several categories (cardiac other, cerebrovascular disease, and infection) rates for blacks are more than twice as high as those for whites.
Deaths per 1,000 patient years at risk
Because of the small number of patients, data are not shown for Native American or Asian patients.
4 White Black 3
2
1
0 Cardiac arrest
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