* Corresponding Author;
Address: AbuzarChildren'sMedicalCenter,AhvazJundishapourUniversityofMedicalSciences,Ahvaz,Iran
E-mail:
©2009byCenterofExcellenceforPediatrics,Children’sMedicalCenter,TehranUniversityofMedicalSciences, Allrightsreserved.


ChronicKidneyDiseaseinSouthwesternIranianChildren
AliAhmadzadeh*
1
,MD;EhsanValavi
1
,MD;MehrnazZangenehKamali
1
,MD;
AzinAhmadzadeh
1
,MD
1. DepartmentofPediatrics,AhvazUniversityofMedicalSciences,Ahvaz,IRIran
Received:Sep03,2008;FinalRevision:Dec01,2008;Accepted:Jan23,2009
Abstract
Objective:TheaimofthestudywastodeterminetheetiologyofChronicKidneyDisease(CKD)
among children attending the pediatric nephrology service at Abuzar children's hospital in
Ahvazcity,thereferralcenterinSouthwestofIran.
Methods: We reviewed the records of 139 children, diagnosed to have CKD over a 10‐year
period.CKDwasdefinedaglomerularfiltrationrate(GFR)below 60 ml/1.73 m2/min
persistingformorethan3months.
Findings:Among139children81(58%)weremales.ThemeanageatdiagnosisofCKDinthe
patients was 4.2 (±3.6) years. Mean level ofserum creatinine at presentation was 1.9 (±1.4)
mg/dl. The mean GFR at presentation was 33.5 (±15.4) ml/1.73m2/min while 22% of the

CKDinchildrenistheresultofheterogeneous
diseases of the kidney and urinary tract that
rangefromcommoncongenitalmalformations
of the urinary tract, to rare inborn errors of
metabolismthataffectkidneyfunction
[1]
.CKD
is an irreversible condition that eventually
progressestoendstage renaldisease(ESRD).
Itisanimportantcauseofmorbidityand
mortalityinchildrenworldwide
[2,3]
.
The causes of CKD vary from one
geographicareatoanotherduetogeneticand
environmental factors. Some of these causes
are preventable while in others, appropriate
medical treatment and interventions may
retardtheprogressionofthedisease
[2]
.Inthe
absenceofanationalregistry,thereispaucity
of information regarding the etiology of CKD
in children from Iran
[4]
. An understanding of
thecausesofCKDisimportantasitmayguide
the distribution of limited resources towards
its prevention. The aim of the present study
wastodetermine retrospectivelytheetiology

lessthan3months;(ii)hisorherinformation
wasincomplete.
Theetiologicalclassificationof CKDwasas
follows: Chronic glomerulonephritis (CGN)
was defined clinically by irreversibility and
histologicallybyobsolescenceandsclerosis
[6]
.
Hypertention was defined if the blood
pressure (systolic or diastolic) levels were
measuredabove95
th
percentile+5mmHgfor
gender, age and height
[7]
. Growth retardation
or failure to thrive (FTT) referred to growth
lessthanthethirdorfifthpercentileorchange
ingrowththathascrossedtwomajorgrowth
percentiles in a short time
[8]
. The underlying
cause of CKD was considered to be reflux
nephropathyinthepresenceofscarredkidney
(irregular renal outline) demonstrated by
ultrasonoraphy, intravenous pyelography or
radionuclideandeitherofthefollowing:(a)
primary vesicoureteric reflux (VUR)
demonstrated on voiding cystouretrography
(VCUG) or radionuclide cystography, and (b)

causeofCRFcouldnotbeidentifiedwere
classifiedasunknownetiology.
Findings
A total of 139 children were included in the
study over a 10‐year period. There were 81
(58.2%) males and 58 (47.8%) females. The
mean age at presentation of CKD was 4.2
(±3.6)years(range3monthsto16years).93
(66.9%) children were below 5, 27 (19.4%)
between 6 and 10, and19 (13.7%) above 11
years old. The details of patients in different
etiological groups and subgroups of each
diseasearesummarizedintable1.
Obstructive uropathy was found in 15
(10.8%) patients. Boys were affected more
commonly (70%)thangirls. The mean ageat
presentationwas14months.Failuretothrive
Table1:EtiologyofchronickidneydiseaseatdifferentagesinSouth‐WesternIranianchildren
Etiology 5m3yr 610yr 1116yr Total(%)

Congenitalurologicmalformations:
Refluxnephropathy
Obstructiveuropathy
Aplastic/hypoplastic/dysplastickidney

Prunebellysyndrome
55
24
10
20

‐
‐
9(6.5)
4(2.9)
4(2.9)
1(0.7)
Hereditarynephropathies:
Infantilecystinosis
Polycystickidneydisease
Diffusemesangialsclerosis
Primaryhyperoxaluria
Juvenilenephronophthisis
Tyrosinemia
BardetBiedlsyndrome
AlportSyndrome
18
7
4
4
2
‐
1
‐
‐
4
2
1
‐
‐
1

1
‐
‐
4
3
‐
1
6(4.3)
4(2.9)
1(0.7)
1(0.7)
Otherrenaldiseases:
Urolithiasis
DistalRTA*(Nephrocalcinosis/stones)

Renalcorticalnecrosis(norecovery)
Chronicinterstitialnephritis
Renaltumor
10
2
5
3
‐
‐
5
3
‐
‐
1
1

1.4%ofcases.
Twenty‐four (23%) patients had reflux
nephropathy. The mean age at presentation
was 4.6 years. Hypertension was present in
25.6%. Twenty‐four patients (45 renal units)
hadasymmetricalrenalscarringwithahistory
ofurinarytractinfectionsinthepast.
CGNwasdiagnosedin9patients(6girls,3
boys). The mean age at presentation was 7.6
years.Thediagnosiswasestablishedonrenal
biopsy. Focal segmental glomerulo‐sclerosis
and membranoproliferative glomerulo‐
nephritis were found each in 4 (2.9%) and
crescent glomerulonephritis in one kidney.
Lupus nephritis was seen in 4 patients. IgA
nephropathywasnotfound.
Renal dysplasia was found in 22 (15.8%)
cases, presented at mean age of 5 months.
These children were more stunted than the
others. Infantile cystinosis was found in 9
(6.5%), polycystic kidneydiseasein5(3.6%)
and diffuse mesangial sclerosis in 4 (2.9%)
cases. These were the commonest causes of
hereditary nephropathies. Alport syndrome,
nephronophthisis andBardet‐Biedlsyndrome
werefoundeachinonecase.
All the patients received standard
treatment for CKD, including dietary
modulation,calciumcarbonate,activevitamin
D analogues, iron and multivitamin

CKDisamedicalprobleminpediatric
populationinsouth‐westofIran(Khuzestan
province) and its neighboring Arab countries
like Kuwait
[11]
andSaudiArabia
[12]
. Genetic
factors associated with consanguinity are
important factors leading to a high incidence
of hereditary diseases and congenital
malformations. It is well‐known that
consanguinity is a common practice in
Khuzestan province and most neighboring
Arabcountries.
Thepreciseincidence ofCKD inIranis not
known.Theageatpresentationwiththe
feature of CKD was lower than in India (8
years),andhigher(4.2years)ascomparedto
reports from developed countries (74%
higher than 5 years), suggesting delayed
detection and referral of patients
[10,13]
. The
etiologyofCKDvariesindifferentpartsofthe
world. Hereditary disorders are common in
regions where the frequency of
consanguineous marriages is high
[4,12]
.

Dateofstudy
1
999 2001 2001 2003 2005 2007
Congenitalurologicmalformations:
Refluxnephropathy
Obstructiveuropathy
Aplasi/hypoplasia/dysplasia
Prunebellysyndrome
55.1
7.2
20.2
25.5
2.2
40
5.4
16.1
15.8
2.7
47
25.9
13.8
7.2
0.6
57.9
16.7
31.8
4.9
61.9
14
29.2

Infantilepolycystickidneydisease
Congenitalnephroticsyndrome/DMS

Juvenilenephronophthisis
Alportsyndrome
Infantilecystinosis
17.6
0.4
1.8
6.9
5.3
1.2
2
13.3
0.6
2.8
2.6
2.8
2.4
2.1
21.1
2.4
3
1.8
2.4
2.4
6.6
7.5
3.6
‐

3.6
0.6
2.4
0.6
‐
0.9
1.6
‐
3.5
1.1
2.3
‐
4.3
2.9
0.7
0.7
Miscellaneous:
Kidneytumors
Ischemic/Vascular
Others
9
1.6
4.5
2.8
12.6
0.6
1.7
10.3
9.6
‐

. In
thisstudyrefluxnephropathyandobstructive
uropathyweremorecommoninmalesthanin
girls.
Posteriorurethralvalvewasthemost
common cause (86.6%) of urinary tract
obstructionaccountingfor13(9.3%) ofcases
ofCKDwhichwassomewhatlessthanin
Indian(14.7%)andotherstudies
 [10,17,18]
.
These conditions together contribute to 23‐
34%ofCKDcasesindevelopedcountries.Itis
proposedthatadeclineintheproportionof

152

ChronicKidneyDiseaseinIranianChildren;

A

Ahmadzadeh,

etal
patientswithrefluxnephropathyischieflydue
to prompt detection and management of
urinary tract infections, followed by careful
screeningforunderlyinganomalies.Screening
of urinary tract anomalies by antenatal
ultrasonography is likely to detect significant

onsetofCKDandtubulardysfunction
(acidosis)ininfancy
[21]
.
Significant advances have been made in
understanding various renal replacement
measures, which have enabled provision of
better care. Both chronic peritoneal dialysis
andhemodialysisalongwithothersupportive
measures can ensurelongevityandimproved
qualityoflifeinpatientsofESRD.
However, chronic dialysis is, in the long‐
term, not able to achieve homeostasis and
growthinchildren.So,kidneytransplan‐tation
is considered the standard therapy for
children with ESRD. Since prolonged dialysis
isassociatedwithmultiplecomplications,itis
usuallyadvised,childrenwithESRDto
undergo kidney transplantation as early as
possible. Pre‐emptive kidney transplantation,
without prior dialysis is also encouraged in
children.
Conclusion
AmajorityofcasesofCKDinourregionaredue
toobstructiveuropathyandrefluxnephropathy
and may be preventable. Renal dysplasia is
common in infants and toddlers, while CGN
accountsformorecasesofCRFinolderchildren
and adolescents. The majority of patients are
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