Endocrine Complication in

Thalassemia patient
.

..

autosomal recessive
globin chain

( developing
country )

free-radical formation peroxidation
membrane mitochondria, lysosome
sarcolemma

20 30


hypogonadotropic hypogonadism impaired glucose
tolerance
hypothyroidism
hypoparathyroidism

1861

50

(delayed puberty),
hypogonadism
amenorrhea (secondary
hypogonadism).
4-10 ,
, ,

. 10
18,
10 6.31

1.

(hemochromatosis)

 (iron overload)

, ,
, glomerulosa
, 2,
(
)
(non-invasive)2,
magnetic
resonance imaging (MRI),

,

(fibrosis),

,

,
.
ferritin
. ferritin
2000 ./.
.
2.

(puberty)

3
3

10.5 /.

8.5 /.
4,5

8 /.
.

(growth hormone, GH) ,
(impaored glucose tolerance) ,

gonadotropin prolactin

 ferritin

3.

,
-

desferrioxamine

,
.
-

(adrenalitis)
HIV
cytomegalovirus,
ketoconazole.

(adrenal insufficiency) .

1.

(short stature)


9 10

multifactorial6,7
chronic anemia
chronic hypoxia, chronic liver desease,
, , chelating
agent , , endocrine-pathies
(hypogonadism, delayed puberty, hypo-thyrodism)
Growth Hormone Insulinlike growth factor (IGF-1)
0-10
Hb
9 g/dl

6,8

desferioxamine 9,10

2-5 desferioxamine9,11,12

DNA, Fibroblast proliferation, collagen

desferioxamine 50 ./.

(short trunk)
(normal)
15-40%

 ( 1)
( 2)

Heshmat 200613
158

(short stature) 62
Growth hormone
insulin like growth factor 1

Growth hormone insulin like growth factor
1 38 42
ferritin
2000 /
01
1
(p<0.001)
14

55 49
-2SD
10 40
-2SD
11

Intervention15
1.

6
2.

(GH)
growth

curve,

3.

Growth Hormone,

deferoxamine

15
1.

Recombinant human
Growth Hormone ( rhGH )
GH 0.6 0.9
iu/kg/wk GH

GH resistance
GH
GH

insulin sensitivity glucose tolerance

2. Zine Sulphate

3. Desferioxamine
risk/benefit Desferioxamine

2.

(Delayed Puberty and

Hypogonadism)

16 435
32.5
hypogonadotropic hypogonadism
hypothalamus

primary gonadol failure

 Delayed Puberty
13-14 (breast
enlargement or testicular enlargement)
Hypogonadotrophic Hypogonagism

Arrested puberty

1

6 8 .
Tanner stage 3

Hypogonadism
15-16

Secondary hypogonadism

hypogonadism
hypothalamus
impotence ejaculation
secondary amenorrhea
hot flush,
17

Hypogonadotrophic
Hypogonadism phenotype
18-20

18

 Hypogonatotrophic Hypogonadism
gonadotroph cell

adipose tissue leptin

/21

Heshmat13 2006
158
hypogonadism 69
hypogonadism
14 FSH LH
hypogonadism
ferritin
2000 /
0-1

1
(p<0.001)
Investigation22
1. 10
1

13 (breast development)
14 (testicular
enlargement)
2. hypogonadism
testicular enlargement (
4 .)
breast development 16

3. hypogonadism
,
testosterone estradiol
DHEA-S
4. secondary hypoganadism ,
impotence infertility

22

hypothalamo-pituitary-gonadal axis

hypogonadism
depottestosterone 25-5. .


6 100 mg
1 1
14
ethiyl estradiol 2.5 5 mcg 6
1

ethinyl estradiol 5 10
mcg 12
(breakthrough uterine bleeding)
medroxy progesterone acetate

5 . 12
estrogen-progesterone
patche
3. Hypothyroidism
granule follicular
epithelium macrophage interstitium
fibrosis primary
hypothyroidism secondary

preclinical, mild overt hypothyroidism

pericardial effusion

thyroid-stimulating hormone (TSH)
T4 free T4 antibody
thyrolobulin
8823 alteration

thyroglobulin membrane

homogeneous echo pattern capsule
electrocardiogram (ECG) prolonged
QT interval pericardial effusion ECG
Voltage secondary hypothyroidism serum T4
TSH thyrotropinreleasing hormone (TRH) stimulation
Hypothyroidism & Cardiac dysfunction


adenosine triphosphatase

hypothyroidism
cardiac output 24
peripheral
vascular resistance
hypothyroidism peripheral vascular
resistance tissue perfusion oxygen utilization

,

bradycardia, hypertension, nonpitting edema pleural
pericardial effusion
dyslipidemia Cholesterol LDL

De Sanctis 200823

50 hypothyriod
arrhythmia
4

L-thyroxine

Hypothyroidism & hypogonadism

Subclinical hypothyroidism
gonad dysfunction


,

Treatment

overt hypothyroidism sodium-L-thyroxine
25 2-3
100 mcg/m2/day
mild hypothyroidism
preclinical hypothyroidism

Biondi Cooper
200825

L-thyroxine
Bauer 26 thyroid hormone

Trokoudes 200627
thyroxine hormone
subclinical hypothyroidism ovulatory
dysfunction
Kallistheni28
2011
52 FT4
(p < 0.001) combine
chelation treatment ( DFO and DFP ) TSH
(p < 0.001)
mono chelation
treatment
Intervention
1.

TSH 1
12

2.

hypothyroidism
thyroid hormone replacement

4. Hypoparathyroidism
hypoparathyroidism

hypoparathyroidism
1.2 1429 16-20
( 18.7)

induce reabsorption
hematopoiesis
hypoparathyroidism
tetany, seizures, carpopedal
spasm paresthesi30
mild parathesia
tetany

, genu valgum

1,25-dihydroxy
24

(osteoporosis) trabecule
codfishdeformities
analogue
Calcitriol 0.25-1.0
2
3

 24 3


phosphate binder
tetany

60 /

turnover
resorptive phase neoformation phase
31 bone density
-2.5 SD
hypertransfusion

(hormonal
replacement therapy,HRT) resorption
bisphosphonate 31
Hamidieh 29 2009
130
hypoparathyroidism
14.6 serum ferritin
(p=0.95)
hypoparathyroidism cardiac
diastolic dysfunction
hypoparathyroidism
(p=0.04)
hypoparathyroidism abnormal
glucose metabolism

 hypoparathyroidism
(p=0.03)
Nicholas 32 2006
243
hypoparathyroidism 14.6
serum parathyroid hormone
calcium (total and ionized form)

(p=0.03)
phosphorus

(p=0.007)
hypoparathyroidism abnormal glucose
metabolism (p<0.05) heart dysfunction
hypoparathyroidism
(p<0.05)
Intervention
1. 12

parathyroid
hormone
5.Impaired glucose tolerance and diabetes
Impaired glucose tolerance (IGT)
10
IGT 11-24
4.710.5 33,34
IGT 2

Beta-

cell ,
, ,
, , blood transfusion
, serum ferritin,
35-36

blood transfusion 10

2:5 37
interstitial cell collagen
microcirculation
supply

impaired glucose tolerance
ketoacidosis IGT
(intensive chelation therapy)
IGT


fructosamine
glycosylated hemoglobin
hemolysis glycosylation

threshold
hypogonadism
hypothyroidism life expectancy

acidosis
thromboembolism

IGT


10
glucose tolerance test
Jimmy 38
transfusion dependent beta-thalassemia
89 19.5

8.5
abnormal glucose tolerance transfusion
dependent beta-thalassemia serumm ferritin
hepatitis C infection
Mehrvar 39
437
5.4

Intervention
1. fasting plasma glucose test
1
75-g2-h OGTT
40
2.
insulin resistance glucose intolerance 41


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