Saudi J Kidney Dis Transpl 2008;19(2):206-209

Saudi Journal
of Kidney Diseases
and Transplantation
2008 Saudi Center for Organ Transplantation




Original Article


Comparative Evaluation of Renal Findings in Beta-Thalassemia Major
and Intermedia

Derakhshan Ali
1
, Karimi Mehran
2
, Abdolkarim Ghadimi Moghaddam
3

Shiraz Nephro-Urology Research Center
1
, Hematology Oncology Research Center
2
, and
Department of Pediatrics
3
, Nemazee Hospital, Shiraz University of Medical Sciences,
Shiraz-Iran

ABSTRACT. Thalassemia is a systematic disease in which the renal involvement has not yet been
scrupulously studied. In a cross-sectional study, the renal findings of 50 cases of thalassemia intermedia
(group 1) were compared to 58 patients with thalassemia major (group 2). Blood urea nitrogen, serum
creatinine, uric acid, calcium, phosphorus, urinalysis, and ultrasonographical findings were evaluated.
Mean age was 18 3.0 in group 1 and 17 3.5 years in group 2. The mean of serum ferritin levels was
871 81.8 ng/ml in group 1 vs. 3503 201 ng/ml in thalassemia major (p < 0.05). Ninety-two percent of
the patients in group 1 were on hydroxyurea at the time of evaluation. Serum uric acid was significantly
higher in group 1 than group 2 patients (5.74 2.95 vs. 4.12 0.9 mg/dl, p < 0.05). Microscopic
hematuria (red blood cell in high power field of urine microscopy 5) was observed among 19 children
(17.6%); 17 of them were in group 1. In contrast, children with thalassemia major had significantly
higher serum creatinine (0.89 0.18 vs. 0.59 0.37 mg/dl, p < 0.05) and blood urea nitrogen values
(12.14 5.58 vs. 13.85 3.54 mg/dl, p < 0.05). We conclude that significant renal involvement is not a
frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and
microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Microscopic
hematuria in thalassemia intermedia might be related to either hypercalciuria or hyperuricosuria.

Keywords: Thalassemia, Renal, Hyperuricosuria, Hypercalciuria, Hematuria, Hyperuricemia

Introduction

Thalassemia was first described by Dr.
Cooley in 1925.
1
It is more prevalent in the
Mediterranean Region, North and West
Africa, the Middle East and some other
parts of the world.
2
About 150 million people
worldwide carry beta-thalassemia gene. High
frequency of thalassemia gene is encountered
in Sardinia (11-34%), Greece (5-15%), and
Reprint request and correspondence to:

Ali Derakhshan, M.D.
Associate Professor of Pediatrics
and Pediatric Nephrology
Shiraz Nephro-Urology Research Center
Nemazee Hospital
Shiraz University of Medical Sciences
Shiraz-Iran
E-mail: derakhsh@sums.ac.ir
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Renal findings in beta-thalasemia 207
Iran (4-10%).
3-5
Thalassemia is the most common type of
genetic abnormality in the world. According
to our health ministry data, there are 20,000
registered thalassemia patients in Iran, of
whom health care has a large burden on
health budget and family economics.
Despite advances in chelating therapy, some
major complications of thalassemia are
inevitable. A large number of studies have
been performed on different complications of
thalassemia, however, there are a few studies
on the renal involvement in this disease.
6-8

We aim in this study to evaluate the renal
manifestations in patients with thalassemia
major and intermedia.

Materials and Methods

We randomly selected 50 thalassemia inter-
media children, who were under regular
follow-up of a hematologist and did not
receive regular transfusions (group 1), and
compared their renal findings with 58
thalassemia major children who were on
regular blood transfusion and chelation
therapy (group 2). Thalassemia was diag-
nosed in the study patients by complete
blood count and hemoglobin electrophoresis.
Children with diabetes and major cardiac
complications were excluded from the study.
The study protocol and laboratory evalua-
tions were discussed with the patients and
their parents and a written consent was
obtained.
The medical records were reviewed meti-
culously, and a complete physical exami-
nation was performed. Repeated investigations
included hemoglobin, urinalysis, serum
calcium, phosphate, blood urea nitrogen
(BUN), creatinine, serum ferritin, and abdo-
minal ultrasonography. All the laboratory
investigations were performed in the same
reference laboratory, and all ultrasono-
graphical evaluations were performed by the
same expert ultrasonographist.

Statistical analysis

Statistical analysis was performed by SPSS
11.5 software and T-test. P <0.05 was
considered significant.

Results

Mean age was 18 3 years (range, 4-24)
in group 1 and 17 3.5 years (range, 3-24)
in group 2. Age at 1
st
blood transfusion or
diagnosis was 3.24 3.38 and 1.6 1.31
years in group1 and 2, respectively. Children
in thalassemia major group received more
regular infusions of desferrioxamine than
thalassemia intermedia group (72.5% vs.
10%, p < 0.05). Hemoglobin level was
9.81 1.13 and 9.11 0.83 g/dL in group
1 and 2, respectively. Serum ferritin level
was significantly higher in thalassemia
major children (3503 201 vs. 871 818
ng/ml; p < 0.05). Ninety-two percent of
thalassemia intermedia children were on
hydroxyurea at the time of evaluation.
Serum uric acid was significantly higher
in group 1 patients than group 2 (5.74 2.95
vs. 4.12 0.9 mg/dL, p < 0.05). Calcium
and phosphorus levels were higher in group 1
than group 2 (9.08 1.03 and 4 0.53
mg/dL vs. 8.69 0.99 and 3.46 0.51
mg/dl, respectively, p < 0.05). In contrast,
group 2 patients revealed significantly higher
serum creatinine and BUN levels than
group 1 (0.89 0.18 vs. 0.59 0.37 mg/dL,
and 12.1 5.58 vs. 13.9 3.54 mg/dL,
respectively, p < 0.05).
Microscopic hematuria (red blood cell in
high power field of urine microscopy 5)
was observed among 19 (17.6%) children,
of whom 17 patients had thalassemia
intermedia. Pyuria was also more common
in group 1 than group 2 (12% vs. 5%, P: not
significant). On the other hand, hemoglo-
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208 Derakhshan A, Mehran K, Moghaddam AG
binuria was observed in 10% of group 2
patients, but in none of the patients in
group 1.
The mean renal size was slightly larger in
group 1 than group 2, but was not statis-
tically significant. A small renal stone of
nonclinical importance was noticed in 2
children in group 1 and 1 child in group 2.

Discussion

Blood transfusion is life saving for
patients with thalassemia major, but loads
body with excess iron that may result in
hemosiderosis and its complications, inclu-
ding multiple endocrinopathies, cardiomyo-
pathy, and hepatic failure. Survival of patients
has greatly improved following the intro-
duction of desferrioxamine and regular
iron chelation when serum ferritin level is
maintained below 2,000 g/L.
9
High serum
ferritin level in thalassemia major group in
this study was related to higher frequencies
of blood transfusion and suboptimal use of
desferrioxamine mostly due to poor
compliance.
Although renal involvement could occur
at tubular level in thalassemia major
6-8

and minor,
10
significant renal findings are
not common. Renal failure is a terminal event
in thalassemia major and is usually secondary
to heart failure and/or hepatic failure. Acute
renal failure following deferoxamine over-
dose or hemolysis has been reported.
11-13

There are numerous reports on complica-
tions of -thalassemia in different organs,
14-18

but a few on renal complications.
6-8,10
The
results of this study compared the renal
findings in children with thalassemia major
and thalassemia intermedia. Children with
thalassemia major had higher BUN and
creatinine levels possibly due to higher
iron deposition in their kidneys. Calcium and
phosphate levels was lower in thalassemia
major, which could be related either to liver
or parathyroid involvement. However, the
simultaneous low phosphorus level in our
patients could not be explained by hypo-
parathyroidism. We found significantly higher
levels of uric acid in thalassemia intermedia
group, which was predictable due to the
higher cellular turnover secondary to the
use of hydroxyurea.
19
An important finding in our study was the
significant number of children in thalassemia
intermedia group who revealed microscopic
hematuria. This finding might be related to
either hyperuricosuria and hypercalciuria.
However, further studies are required to
confirm this speculation.
We conclude that significant renal involve-
ment is not a frequent complication in
children and young adults suffering from
thalassemia. Hyperuricemia and microscopic
hematuria are more common in thalassemia
intermedia than thalassemia major. Micros-
copic hematuria in thalassemia intermedia
might be related to either.

Acknowledgement

The authors wish to thank the vice chancellor
for research for his financial support and
also Dr. Hamed Jalaeian for his editorial
assistance.

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