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COMPLICATIONS OF THALASSEMIA :forbidden: :warning: :!!: (ENDOCRINE…
COMPLICATIONS OF THALASSEMIA
:forbidden: :warning: :!!:
BONE COMPLICATION
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PATHOPHYSIOLOGY
These factors
can cause bone disorder and development of
osteopenia
and
osteoporosis
:
:check: iron overload
:check: over chelation
:check: inadequate transfusion
:check: endocrine problem
The symptoms
:
:check: bone fractures
:check: pain
:check: growth retardation
:check: skeletal deformities
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MONITORING
Annual evaluation
:
(evaluate and measure)
:unlock: calcium metabolism and serum calcium
:unlock: parathyroid function
:unlock: PTH level
:unlock: Initial bone-density assessment
(performed age 8 years and annually thereafter)
ENDOCRINE COMPLICATION
HYPOPARATHYROIDISM
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PATHOPHYSIOLOGY
Iron deposition at parathyroid glands or suppression of parathyroid secretion resulting from increased hematopoiesis secondary to the chronic anemia.
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MONITORING
:unlock: Evaluate annually with serum calcium, PTH, and 25-hydroxy vitamin D screening
SHORT STATURE & GROWTH FAILURE
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PATHOPHYSIOLOGY
Few factors causing growth failure;
:check: transfusional iron overload
:check: hypogonadism shown to be associated with short stature
:check:malnutrition
:check: GH deficiency
:check: chelation toxicity
:check: chronic anemia
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MONITORING
:unlock: Growth assessment
:unlock: endocrine screening
:unlock: GH secretion assessment
:unlock: Dietary assessment
:unlock: Insulin sensitivity and glucose tolerance
:unlock: Laboratory tests
:unlock: growth and
development (Children)
DELAYED PUBERTY &HYPOGONADISM
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MONITORING
:unlock: Serum LH, FSH
:unlock: Estradiol
:unlock: Testosterone
:unlock: Ultrasound
:unlock: screening, hormonal assessment and bone age
(Girls & Boys)
:unlock: secondary hypogonadism, impotence or infertility (Adult)
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PATHOPHYSIOLOGY
Iron deposition in the pituitary gonadotrophic cells ; leads to
disruption of gonadotropin (LH and FSH)
HYPOTHYROIDISM
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MONITORING
:unlock: TSH & free T4 annually
(12 years or after 3 years of transfusion)
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PATHOPHYSIOLOGY
:check: Late consequence of iron deposition in the thyroid gland causes
parenchymal fibrosis
DIABETES MELLITUS
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MONITORING
:unlock: screening with OGTT and fructosamine annually (non-diabetic pt)
:unlock: measurement of random plasma glucose and fructosamine (hyperglycaemia pt)
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PATHOPHYSIOLOGY
:check: Iron overload damages pancreatic β-cells, reducing insulin secretion
:check: insulin resistance secondary to liver disease
:check: hepatitis C virus infection affecting glucose metabolism
LIVER
COMPLICATION
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PATHOPHYSIOLOGY
contributory factors
:check: hepatic toxicity
:check: viral hepatitis
:check: drug toxicity
:check: biliary disease
Clinical presentation
:check: obstructive jaundice
:check: hypertension
:check: hepatic insufficiency
:check: acute and
chronichepatitis
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MONITORING
monitor every 3 months
:
:unlock: Liver enzymes
(AST, ALT, ALP)
:unlock: bilirubin
monitor annually
:
:unlock: Viral serology including HepBsAg, anti-HepB sAb, & anti-Hep
C Ab
CARDIAC + IRON OVERLOAD
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PATHOPHYSIOLOGY
Free iron accumulates
when body is unable to store excess iron
, The irons participates in the formation of reactive radicals, causing denaturation of proteins and membrane damage.
Cardiac iron loading occurs when the
heart is exposed to high circulating non-transferrin bound iron species
for long periods of time.
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MONITORING
Cardiac MRI
Provides consistency in detecting pre-clinical changes in ejection fraction and measures the effectiveness of chelation.
:unlock: Low risk - every 24 months
:unlock: Standard risk - every 12 months
:unlock: High risk - every 6 months
Annual Evaluation
:unlock: Echocardiogram assessment of systolic and diastolic function & pulmonary artery pressure
:unlock: Electrocardiogram monitoring for abnormal baseline ECG
If suspected arrhythmia
:unlock: Holter monitoring
:unlock: Monitor serum ferritin concentration every 2-3 months.
:unlock: Liver iron concentration (LIC) measured on ultrasound-guided liver biopsy.
:unlock: MRI based techniques to determine organ iron load non-invasively.
INFECTIONS
HEPATITIS C
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PATHOPHYSIOLOGY
Hepatitis C (HCV) is spread through contact with an infected person's blood - which may be present because of genital sores or cuts or menstruation.
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MONITORING
:unlock:
Liver biopsy every 6 months
for thalassaemia patients with liver cirrhosis. It is useful in assessing severity of liver damage, provides information on prognosis and adds information on response to treatment
:unlock:
Anti-HCV antibody (ELISA)
, this is indicative of exposure to
the virus
HEPATITIS B
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PATHOPHYSIOLOGY
Hepatitis B virus (HBV) may occasionally be transmitted through transfusion of blood units that are hepatitis B surface antigen (HBsAg) negative but HBV DNA positive.
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MONITORING
Every 3 months
:unlock: HBeAg
:unlock: HBV-DNA
Monitor
ALT and HBV-DNA
level monthly for 3 months to detect early relapse
HIV
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PATHOPHYSIOLOGY
CD4 + depletion
due to
:check: Direct cytotoxic effects of HIV replication
:check: Cell-mediated immune cytotoxicity
:check: Thymic damage that impairs lymphocyte production
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MONITORING
Every 6 months
:unlock: HIV serology
:unlock: HIV Antibody’ test