Combination of chelation therapy:
For the
purpose of iron trapping, it is possible that a combination of different types
of chelators, such as LI plus desferoxamine, may chelate iron more successfully
than a single drug. Deferiprone was administered orally three times daily, in
combination with 8 h of nightly subcutaneous desfemoxamine. A 24-129% increase
in iron excretion using the combination was found, compared to desferrioxamine
alone (Hoffbrand et al., 1998). Significant reduction in serum
ferritin levels and in cardiac iron stores measured by MRI and improvement of
left ventricular shortening fraction are reported using combination therapy,
without unexpected toxicity (Kattamis et al., 2006).
·
Splenectomy:
Indications for splenectomy
include:
-
A
persistent increase in blood transfusion requirements by 5 % or more over
initial needs for over 6 months.
-
Yearly
packed cell transfusion requirements in excess of 250 ml / kg / year.
However,
especially in children under 4 years of age, the risk from the development of
sudden, overwhelming infection appears to be much greater following splenectomy
than in older children. In thalassemia major, this risk seems to be much higher
than following splenectomy for more benign conditions. The pneumococcus has
been the most frequent organism involved and for this reason daily prophylactic
administration of penicillin or sulfisoxazole has been recommended for a
younger child four year or two after splenectomy. Raised anti-streptolysin
titers have also been reported and it has been suggested that benign
pericarditis occurring post-splenectomy is attributable to such streptococcal
infections. Serum immunoglobulin levels for IgG and IgA have been found to be
increased after splenectomy. Patients planned to undergo this operation should
be immunized with polyvalent pneumococcal, meningococcal
and hemophilus influenza vaccines (Model et al, 2001). Intensive family
education should be provided regarding the need for immediate medical attention
if patient develops high fever (El-Alfy and El Sayed 2004).
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