Free testosterone level in patients with homozygous beta thalassemia on regular transfusions regimen
Keywords:
homozygous beta thalassemia, regular transfusions, iron overload, free testosterone level
Abstract
Background Patients with homozygous beta thalassemia requireregular transfusions which will lead to iron deposition in tissues
including testicles.
Objective This study aimed to evaluate testicular function in syn-
thesizing testosterone by measuring free testosterone level. The
correlation between free testosterone level and transferrin satura-
tion was evaluated.
Methods This was a cross sectional study. Sampling was done
consecutively. Free testosterone level was measured by radioim-
munoassay in 20 homozygous beta thalassemic patients receiv-
ing regular transfusions and compared to 20 healthy subjects.
Results Fourteen out of 20 patients showed free testosterone level
below the reference range. Out of 20 thalassemic patients, one
patient’s serum was not enough for transferrin saturation determi-
nation. Among the 19 patients, 18 had transferrin saturation above
55%, 1 less than 55%, while all healthy subjects had normal trans-
ferrin saturation. A significant difference was found between the
two groups, both in the free testosterone level (p=0.001) and trans-
ferrin saturation (p<0.001). A very weak correlation was found be-
tween free testosterone level and transferrin saturation (r=-0.215).
Conclusion We concluded that there might be a relationship be-
tween iron overload and testicular endocrine function in patients
with homozygous beta thalassemia receiving regular transfusions
References
1. Weatherall DJ. Genetic disorders of the haemoglobin.
In: Hoffbrand AV, Lewis SM, Tuddenham EGD, edi-
tors. Postgraduate Haematology. 4 th ed. Oxford:
Butterworth-Heinemann; 1999. p. 91-119.
2. Olivieri NF. The β-Thalassemias. N Engl J Med
1999;341:99-109.
3. Chatterjee R, Katz M. Reversible hypogonadotrophic
hypogonadism in sexually infantile male thalassaemic
patients with transfusional iron overload. Clin
Endocrinol 2000;53:33-8.
4. McKenzie SB. Anemias caused by abnormalities in
globin biosynthesis. In: McKenzie SB. Textbook of
Hematology. 2 nd ed. Baltimore: Williams and Wilkins;
1996. p.147-78.
5. Italian Working group on endocrine complications in
non-endocrine diseases. Thalassemia and endocrino-
pathies: Multicenter study of 3092 patients. In: Ando
S, Brancati C, editors. Endocrine Disorders in Thalas-
semia. Berlin: Springer-Verlag; 1995. p. 91-3
6. Guyton AC. Reproductive and hormonal functions of the
male. In: Guyton AC editor. Textbook of Medical Physiol-
ogy. 8 th ed. Philadelphia: WB Saunders; 1991. p. 885-98.
7. Coat-A-Count Free Testosterone [leaflet]. Diagnostic
Products Corporation, Los Angeles, 1999.
8. CON6 [leaflet]. Diagnostic Products Corporation, Los
Angeles, 1999
9. Saunders DB, Trapp GR. Estimating and comparing
means. In: Basic Clinical Biostatistics. Canada:
Appleton-Lange; 1994. p. 896-909.
10. Piomelli S. The management of patients with Cooley’s
anemia: Transfusions and splenectomy. Semin Hematol
1995;32:262-8.
11. Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A,
Di Gregorio F, Gamberini MR, et al. Survival and dis-
ease complications in thalassemia major. Ann NY Acad
Sci 1998;850:227-31.
12. Hershko C, Link G, Cabantchik I. Pathophysiology of
iron overload. Ann NY Acad Sci 1998;850:191-201.
13. Weatherall DJ, Clegg JB. Genetic disorders of hemo-
globin. Semin Hematol 1999;36(Suppl 7):24-37.
14. Sherwood RA, Pippard MJ, Peters TJ. Iron homeosta-
sis and the assessment of iron status. Ann Clin Biochem
1998;35:693-708.
15. Powell LW, George K, McDonnell SM, Kowdley KV. Di-
agnosis of hemochromatosis. Ann Intern Med
1998;129:925-31.
16. Kattamis CA, Kattamis AC. Management of
thalassemias: growth and development, hormone sub-
stitution, vitamin supplementation, and vaccination.
Semin Hematol 1995;32:269-79.
17. Braunstein GD. Testes. In: Greenspan FG, editor. Ba-
sic and Clinical Endocrinology. 3 rd ed. London:
Appleton-Lange; 1991. p.407-41.
18. Wilke TJ, Utley DJ. Total testosterone, free-androgen in-
dex, calculated free testosterone, and free testosterone
by analog RIA compared in hirsute women and in other-
wise-normal women with altered binding of sex-hormone-
binding globulin. Clin Chem 1987;33:1372-5.
19. Caglayan S, Ozata M, Osizik G, Turan M, Bolu E,
Oktenli C, et al. Plasma melatonin concentration be-
fore and during testosterone replacement in
Klinefelter’s Syndrome: relation to hepatic indolamine
metabolism and sympathoadrenal activity. J Clin
Endocrinol Metab 2001;86:738-43.
20. Kasa-Vubu JZ, Kelch RP. Precocious and delayed pu-
berty: diagnosis and treatment. In: DeGroot LJ, Besser
M, Burger HG, Jameson JL, Loriaux DL, Marshall JC,
et al, editors. Endocrinology. 3 rd ed. Philadelphia: WB
Saunders Company; 1997. p. 1953-77.
21. Wirawan R, Kusnandar S, Suherli A, Gatot D. Renal
impairment in β-thalassemia major patients receiving
repeated blood transfusion. Med J Indones In press
2003.
22. Cheng RW, Reed MJ, James VHT. Plasma free testosterone: equi-
librium dialysis vs direct radioimmunoassay. Clin Chem
1986;32:1411.
23. Lewis SM. Quality assurance in haematology. WHO/
Lab/ 98.4
24. Van den Beld AW, de Jong FH, Grobbee DE, Pols HAP,
Lamberts SW. Measures of bioavailable serum testoster-
one and estradiol in their relationships with muscle
strength, bone density and body composition in eld-
erly men. J Clin Endocrinol Metab 2000;85:3276-82.
25. Garde AH, Hansen AM, Skovgaard LT, Christensen JM.
Seasonal and biological variation of blood concentra-
tions of total cholesterol, dehydroepiandrosterone sul-
fate, hemoglobin A 1c , IgA, prolactin, and free testoster-
one in healthy women. Clin Chem 2000;46:551-9 .
26. Stites DP, Rodgers RPC. Clinical laboratory methods
for detection of antigens and antibodies. In: Stites DP,
Terr, AI, editors. Basic and Clinical Immunology. 6 th ed.
London: Appleton & Lange; 1987. p. 241-85.
27. Winters SJ, Kelley DE, Goodpaster B. The analog free
testosterone assay: are the results in men clinically use-
ful? Clin Chem 1998;44:2178-82.
28. Roesli T. Gangguan fungsi hati pada penderita thalas-
semia b mayor: telaah khusus hubungan dengan
saturasi transferin dan infeksi virus hepatitis hepatitis
B & C [thesis]. Jakarta: Departments of Clinical Pa-
thology, Medical School, University of Indonesia; 2001.
In: Hoffbrand AV, Lewis SM, Tuddenham EGD, edi-
tors. Postgraduate Haematology. 4 th ed. Oxford:
Butterworth-Heinemann; 1999. p. 91-119.
2. Olivieri NF. The β-Thalassemias. N Engl J Med
1999;341:99-109.
3. Chatterjee R, Katz M. Reversible hypogonadotrophic
hypogonadism in sexually infantile male thalassaemic
patients with transfusional iron overload. Clin
Endocrinol 2000;53:33-8.
4. McKenzie SB. Anemias caused by abnormalities in
globin biosynthesis. In: McKenzie SB. Textbook of
Hematology. 2 nd ed. Baltimore: Williams and Wilkins;
1996. p.147-78.
5. Italian Working group on endocrine complications in
non-endocrine diseases. Thalassemia and endocrino-
pathies: Multicenter study of 3092 patients. In: Ando
S, Brancati C, editors. Endocrine Disorders in Thalas-
semia. Berlin: Springer-Verlag; 1995. p. 91-3
6. Guyton AC. Reproductive and hormonal functions of the
male. In: Guyton AC editor. Textbook of Medical Physiol-
ogy. 8 th ed. Philadelphia: WB Saunders; 1991. p. 885-98.
7. Coat-A-Count Free Testosterone [leaflet]. Diagnostic
Products Corporation, Los Angeles, 1999.
8. CON6 [leaflet]. Diagnostic Products Corporation, Los
Angeles, 1999
9. Saunders DB, Trapp GR. Estimating and comparing
means. In: Basic Clinical Biostatistics. Canada:
Appleton-Lange; 1994. p. 896-909.
10. Piomelli S. The management of patients with Cooley’s
anemia: Transfusions and splenectomy. Semin Hematol
1995;32:262-8.
11. Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A,
Di Gregorio F, Gamberini MR, et al. Survival and dis-
ease complications in thalassemia major. Ann NY Acad
Sci 1998;850:227-31.
12. Hershko C, Link G, Cabantchik I. Pathophysiology of
iron overload. Ann NY Acad Sci 1998;850:191-201.
13. Weatherall DJ, Clegg JB. Genetic disorders of hemo-
globin. Semin Hematol 1999;36(Suppl 7):24-37.
14. Sherwood RA, Pippard MJ, Peters TJ. Iron homeosta-
sis and the assessment of iron status. Ann Clin Biochem
1998;35:693-708.
15. Powell LW, George K, McDonnell SM, Kowdley KV. Di-
agnosis of hemochromatosis. Ann Intern Med
1998;129:925-31.
16. Kattamis CA, Kattamis AC. Management of
thalassemias: growth and development, hormone sub-
stitution, vitamin supplementation, and vaccination.
Semin Hematol 1995;32:269-79.
17. Braunstein GD. Testes. In: Greenspan FG, editor. Ba-
sic and Clinical Endocrinology. 3 rd ed. London:
Appleton-Lange; 1991. p.407-41.
18. Wilke TJ, Utley DJ. Total testosterone, free-androgen in-
dex, calculated free testosterone, and free testosterone
by analog RIA compared in hirsute women and in other-
wise-normal women with altered binding of sex-hormone-
binding globulin. Clin Chem 1987;33:1372-5.
19. Caglayan S, Ozata M, Osizik G, Turan M, Bolu E,
Oktenli C, et al. Plasma melatonin concentration be-
fore and during testosterone replacement in
Klinefelter’s Syndrome: relation to hepatic indolamine
metabolism and sympathoadrenal activity. J Clin
Endocrinol Metab 2001;86:738-43.
20. Kasa-Vubu JZ, Kelch RP. Precocious and delayed pu-
berty: diagnosis and treatment. In: DeGroot LJ, Besser
M, Burger HG, Jameson JL, Loriaux DL, Marshall JC,
et al, editors. Endocrinology. 3 rd ed. Philadelphia: WB
Saunders Company; 1997. p. 1953-77.
21. Wirawan R, Kusnandar S, Suherli A, Gatot D. Renal
impairment in β-thalassemia major patients receiving
repeated blood transfusion. Med J Indones In press
2003.
22. Cheng RW, Reed MJ, James VHT. Plasma free testosterone: equi-
librium dialysis vs direct radioimmunoassay. Clin Chem
1986;32:1411.
23. Lewis SM. Quality assurance in haematology. WHO/
Lab/ 98.4
24. Van den Beld AW, de Jong FH, Grobbee DE, Pols HAP,
Lamberts SW. Measures of bioavailable serum testoster-
one and estradiol in their relationships with muscle
strength, bone density and body composition in eld-
erly men. J Clin Endocrinol Metab 2000;85:3276-82.
25. Garde AH, Hansen AM, Skovgaard LT, Christensen JM.
Seasonal and biological variation of blood concentra-
tions of total cholesterol, dehydroepiandrosterone sul-
fate, hemoglobin A 1c , IgA, prolactin, and free testoster-
one in healthy women. Clin Chem 2000;46:551-9 .
26. Stites DP, Rodgers RPC. Clinical laboratory methods
for detection of antigens and antibodies. In: Stites DP,
Terr, AI, editors. Basic and Clinical Immunology. 6 th ed.
London: Appleton & Lange; 1987. p. 241-85.
27. Winters SJ, Kelley DE, Goodpaster B. The analog free
testosterone assay: are the results in men clinically use-
ful? Clin Chem 1998;44:2178-82.
28. Roesli T. Gangguan fungsi hati pada penderita thalas-
semia b mayor: telaah khusus hubungan dengan
saturasi transferin dan infeksi virus hepatitis hepatitis
B & C [thesis]. Jakarta: Departments of Clinical Pa-
thology, Medical School, University of Indonesia; 2001.
Published
2016-10-10
How to Cite
1.
Wirawan R, Santosa E, Setiawan L, Munthe B, Astrawinata D. Free testosterone level in patients with homozygous beta thalassemia on regular transfusions regimen. PI [Internet]. 10Oct.2016 [cited 24Nov.2024];44(2):73-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/740
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Received 2016-09-29
Accepted 2016-09-29
Published 2016-10-10
Accepted 2016-09-29
Published 2016-10-10
