Abstract
Objective: End stage Renal
Disease is a rare condition in children. It is usually the result of slowly progressive
irreversible kidney damage. The aim of
this study is to determine the causes, outcome and describe the demographic
features of all children with end stage renal disease on regular dialysis at
Queen Rania Al-Abdullah II Hospital of Paediatrics. This is the referral centre for all pediatric
subspecialties in Jordan.
Methods: A retrospective review of all computerized
data for all children with end stage renal disease who were on regular dialysis
at Queen Rania Hospital during the period January 2006 to April 2011 were done. The data was reviewed regarding gender, age at the initiation of dialysis, mode
of dialysis and primary disease. The outcome of these children was also
followed and reviewed.
Results: Out of 98 children who were on regular dialysis at Queen Rania
Hospital, 90 (92%) were
on hemodialysis and eight (8%) were on peritoneal dialysis, 53 (54%) were
female, 45 (46%) were male. The mean age at the initiation of dialysis was 8.2±2.3
years;
the
commonest cause of end stage renal disease was congenital anomalies of the
kidney and urinary tract which occurred in 34 (34.5%) children. However unknown causes were found in four
cases (4%). Thirty-nine children (39.5%) were transplanted and 25 (25.5%)
children died while they were on dialysis.
Conclusions: The most common
cause of end stage renal disease at our center was congenital anomalies of the
kidney and urinary tract, which is a preventable cause when detected early. The
increased percentage of renal transplants observed among patients with end
stage renal disease on regular dialysis at our centre is promising as it offers
the best choice of renal replacement therapy.
Key words: End
stage renal disease, Haemodialysis, Peritoneal dialysis, Regular dialysis
JRMS September
2013; 20(3): 33-36 /DOI: 10.12816/0001038
Introduction
End stage renal disease (ESRD) is a devastating chronic health problem
in children, for which there is no lifelong cure, not even after
kidney transplantation.(1) It occurs in five to 10
children per million each year.(2) Fifty years ago, all
children with ESRD died.(1) Now, almost all such children
are treated with dialysis or renal transplantation.(3) The first pediatric dialysis programs were
started in the 1960s and the first pediatric renal transplants took place in
the 1970s.(3) Awareness of
the cause of ESRD helps the nephrologists anticipate problems during renal
replacement therapy (RRT) and plan preventive measures for the community.(4)
Unfortunately in most of the
developing world, patients with renal disease present late because of the lack
of proper follow up and can only be evaluated while they are undergoing
dialysis or enlisted for renal transplantation.(5) In the USA
incidence and prevalence of ESRD is expected to increase by 44 and 85%,
respectively from 2000 to 2015 and incidence and prevalence rates per million
population by 32 and 70%.(6)
There is limited data on pediatric
ESRD in Jordan due to the lack of a national registry system. However, the
incidence of pediatric ESRD was estimated in 2005 to be 7.5 patients per
million children.(7) While the incidence and prevalence of chronic
kidney disease among Jordanian children was estimated in 2002 to be 10.7 and 51
patients per million children, respectively.(8)
The aim of this study was to determine the causes, outcome and describe the demographic
features of all children with ESRD who were on regular dialysis for the last 5
years at Queen Rania Al-Abdullah II
Hospital for Paediatrics.
Methods
This is a retrospective review
of all the computerized data of 98 children with ESRD at Queen Rania Al-Abdullah
II Hospital of Paediatrics (QRH) during the period from January 2006 to April
2011. These data were reviewed regarding gender, age at the initiation of dialysis,
mode of dialysis, either haemodialysis (HD) or peritoneal dialysis (PD). The
outcome of those children were followed and reviewed. Patients who under went urgent dialysis for
acute renal failure were excluded from this study. Data were analyzed using
SPSS 10 (statistical package for social studies); results were presented in
number, percent, mean and standard deviation (SD).
Results
There were a total of 98 children with ESRD on regular dialysis at (QRH)
during the period between January 2006 to April 2011. Fifty-three (54%) were
female and 45 (46%) were male. The mean age at the time of dialysis initiation
was 8.2 ± 2.3 years. Ninety (92%)
children were on HD, while eight (8%) children were on PD. The outcome of patients with ESRD from the
study group is shown in Table I. The most common cause of ESRD
was congenital anomalies of the
kidney and urinary tract which occurred in 34 (34.5%) children. However,
glomerulopathy and hereditary nephropathy was found in 29 (29.6%) and 27 (27.9%)
children, respectively. Multisystem disease like those with renal amyloidosis
(one patient secondary to Hyper IgD syndrome, the other secondary to FMF),
Hemolytic uremic syndrome (Diarrhea positive-D+) and Henoch Schonlein Purpura
nephritis occurred in four (4%) children. Four cases had no identifiable causes
and accounted for 4% of the total. The etiology of ESRD among our study is described
in Table II.
Discussion
The underlying causes of ESRD are different in children than those in
adults. Diabetic nephropathy and hypertension, predominant causes of ESRD in
adults, are rare causes of ESRD in childhood.(9) The
predominance of congenital anomalies of the kidney and urinary tract and
glomerulopathy are the main causes of ESRD in children and are similarly
observed in other studies.(7,9) However, other studies(10-13) reported congenital and
hereditary reasons as the main cause of ESRD in children. In the United States,
the most common primary renal diseases found in chronic kidney disease are
glomerulonephritis followed by cystic, hereditary and congenital diseases.
Diabetes is rare.(14) Congenital anomalies of the kidney and
urinary tract account for the majority of preventable causes of ESRD in our
study which included reflux nephropathy, neurogenic bladder which occurred in
10.2% and 16.3%, respectively, and is comparable to other studies(7)
which reported it in 11.9% and 14.3% respectively. In the Arab world, genetic
factors associated with consanguinity are important factors leading to a high
percentage of hereditary diseases and congenital malformations, for which
primary oxalosis, polycystic kidney disease, juvenile nephronophthesis, Bartter
syndome, Alport syndrome which appears to cause ESRD in our study in a
considerable percentage which is comparable to other studies.(15)
In the present study, ESRD was more common in females
than males, in contrast to other studies.(7) The mean age at the time of dialysis
initiation in our study was comparable to what has been reported in other
studies.(7,16)
Table I: Outcome of ESRD patients among the study group
Out Come
|
Number
|
%
|
Transplanted
Dialysis
Died
|
39
34
25
|
39.5
35
25.5
|
Table II: Etiology of ESRD among the study group
Etiology
|
Number
|
%
|
CAKUT*
-Neurogenic
bladder
-Reflux
nephropathy
-Obstructve
nephropathy
-Hypoplastic
/Dysplastic kidney
|
34
16
10
4
4
|
34.5
16.3
10.2
4.0
4.0
|
Glomerulopathy
-Focal segmental
glomerulosclerosis
-Mesangiocapillary
GN**
-Rapidly progressive GN
|
29
20
5
4
|
29.6
20.4
5.2
4.0
|
Hereditary
nephropathy
-Polycystic
kidney disease
-Primary oxalosis
-Juvenile
nephronophthesis
-Alport syndrome
-Bartter syndrome
-Bardet Biedel syndrome
-Cystinosis
|
27
9
9
5
1
1
1
1
|
27.9
9.35
9.35
5.2
1.0
1.0
1.0
1.0
|
Multi system disease
-Hemolytic uremic
syndrome
-Henoch Schonlein
purpura
-Amyloidosis
|
4
1
1
2
|
4.0
1.0
1.0
2.0
|
Unknown causes
|
4
|
4.0
|
*Congenital Anomalies of Kidney
and Urinary Tract **Glomerulonephritis
The mean age at the
time of dialysis initiation was 11.1 and 12.1 years, respectively. In our centre,
HD is the primary mode of dialysis, accounting for 92% of children in this
study, while PD is done for less than 8% of the children for reasons related to
parental choice and technical reasons. In some European countries, HD was often
preferred for children over the age of five years.(17) In contrast, PD is offered to younger
children, especially under the age of two years or weighing less than 10 kilograms.(18)
Nevertheless, the 2005 USRDS annual report noted a re-emergence of HD as the
modality of choice over PD for which now in United States more than two thirds
of children receive HD rather than PD.(16) Despite
improvement in long-term survival of patients with ESRD, mortality rate among
children requiring RRT remains substantially higher than those among children
without ESRD.(3) Transplantation remains the major modifiable
factor in improving the long term survival of children with ESRD.(3,16,19)
In the Royal Medical Services of Jordan
the first pediatric renal transplant was performed in April 2003. Sacca et
al.(7) reported that 30% of children were transplanted
during the period between January 2001 to December 2005 which is a low
percentage when compared to the western world where more than 66% of children
are transplanted.(16) In our study, 39% of children on
regular dialysis were transplanted, and this appears to be a good percentage, when taking into
consideration that we excluded pre-emptive transplants from our study, and the
high percentages of patients with oxalosis which requires a combined liver and
kidney transplantation, an option not yet available in our center.
Conclusion
The most common causes of ESRD in
our centre were the congenital anomalies of the kidney and urinary tract, which
is a preventable cause if detected early.
Increasing percentage of renal transplantation among patients with ESRD
on regular dialysis in our centre is promising as it offers the best choice of
RRT.
The establishment of a Renal Registry in Jordan would be helpful as it would
improve organization in our health care system.
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