Views: 1,399 | Downloads:
39
| Responses: 0
XML | Respond to this article | Alert & updates | Request permissions | Email to a friend |
Original ArticleOpen Access
Linear Growth in Homozygous 8-Thalassemia and 8-Thalassemia/ Hemoblogin E Patients Under Different Treatment Regimens
Viprakasit V ,
Tanphaichitr V ,
Mahasandana C ,
Assteerawatt A ,
Suwantol L ,
Veerakul G ,
Kankirawatana S ,
Pungamritt P ,
Suvatte V
The effects on linear growth and development among thalassemic patients under diffe-
rent treatment regimens were compared. Twelve homozygous
~-thalassemia
(homozygous
~-thai)
and 36
~-thalassemia!Hb
E
(~-thal/Hb
E) were studied longitudinally between 1977 and 1998.
Eighteen cases (10 homozygous
~-thai
and 8
~-thal/Hb
E) received hypertransfusion with iron
chelation by desferrioxamine. Another 30 cases (2 homozygous
~-thai
and 28
~-thal/Hb
E) were
given a low transfusion (depending on their clinical requirement). Their heights were measured
serially and are presented as a standard deviation score (SDS). There was no significant difference
in initial basic hematological data and ferritin levels between either group. However, the hyper-
transfused group, seemed to be clinically more severely affected than the other group as evidenced
by early age at initial transfusion, the early onset of anemia and diagnosis and also their large
acquired iron load after a period of transfusion.
The average height SDS of the hypertransfused patients was within the
50'h
percentile เธ‘ 1
SD during the first decade of life in both sexes and both genotypes. Whereas, in patients who were
transfused infrequently, the SDS was always below the -I SD and decreased gradually. In severe
~-thal/Hb
E cases, their growth SDS showed no difference from those with homozygous
~-thai.
Normal linear growth in those with homozygous
~-thai
and severe
~-thal/Hb
E was only
seen in the group that underwent hypertransfusion and this regimen contributed to normal growth
during the first ten years of life. However, adequate iron chelation and hormonal treatment in these
patients were also required in order to achieve normal adult height.
Key word : Linear Growth, Homozygous
~-thalassemia, ~-thalassemia/Hb
E, Blood Transfusion,
Iron Chelation
rent treatment regimens were compared. Twelve homozygous
~-thalassemia
(homozygous
~-thai)
and 36
~-thalassemia!Hb
E
(~-thal/Hb
E) were studied longitudinally between 1977 and 1998.
Eighteen cases (10 homozygous
~-thai
and 8
~-thal/Hb
E) received hypertransfusion with iron
chelation by desferrioxamine. Another 30 cases (2 homozygous
~-thai
and 28
~-thal/Hb
E) were
given a low transfusion (depending on their clinical requirement). Their heights were measured
serially and are presented as a standard deviation score (SDS). There was no significant difference
in initial basic hematological data and ferritin levels between either group. However, the hyper-
transfused group, seemed to be clinically more severely affected than the other group as evidenced
by early age at initial transfusion, the early onset of anemia and diagnosis and also their large
acquired iron load after a period of transfusion.
The average height SDS of the hypertransfused patients was within the
50'h
percentile เธ‘ 1
SD during the first decade of life in both sexes and both genotypes. Whereas, in patients who were
transfused infrequently, the SDS was always below the -I SD and decreased gradually. In severe
~-thal/Hb
E cases, their growth SDS showed no difference from those with homozygous
~-thai.
Normal linear growth in those with homozygous
~-thai
and severe
~-thal/Hb
E was only
seen in the group that underwent hypertransfusion and this regimen contributed to normal growth
during the first ten years of life. However, adequate iron chelation and hormonal treatment in these
patients were also required in order to achieve normal adult height.
Key word : Linear Growth, Homozygous
~-thalassemia, ~-thalassemia/Hb
E, Blood Transfusion,
Iron Chelation
Download:
PDF