Study of Adrenal Functions using ACTH stimulation test in Egyptian children with Sickle Cell Anemia: Correlation with Iron Overload.

  • Adel A Hagag Mail Pediatrics1 and Clinical Pathology Departments, Faculty of Medicine, Tanta University, Egypt
  • Mohamed S El-Farargy Departments, Faculty of Medicine, Tanta University, Egypt
  • Amany M Abo El-Enein Departments, Faculty of Medicine, Tanta University, Egypt.
Adrenal functions, Iron overload, Sickle cell anemia


Background: Sickle-cell anemia is characterized by defective hemoglobin synthesis with production of sickle hemoglobin. Sickle red blood cells become deformed and rigid with difficulty to pass through narrow capillaries and frequent clotting and thrombosis leading to repetitive vascular occlusions and progressive organ damage. We conducted this work to study some adrenal functions using ACTH stimulation test in Egyptian children with sickle cell anemia in correlation with iron overload.
Methods: This study was conducted on 60 children with sickle cell anemia who were attendants of Hematology unit, Pediatric department, Tanta university hospital in the period from April 2012 to May 2014 including 34 males and 26 females with their age ranging from 5-17 years and main age value of 13±2.9 in comparison with 30 healthy children matched for age and sex as a control group. For all patients the following were done: Complete blood count, Hb electrophoresis, serum ferritin, iron, iron binding capacity, sodium and potassium, random blood glucose, thyroid functions , morning basal cortisol levels and adrenocorticotrophic hormone stimulation test.
Results : There was significantly lower basal morning cortisol in patients than controls (mean value in patients were 8.78±3.53 ug/dl compared with 11.79±2.32 ug/dl in control group with p value of 0.021). No significant differences in adrenocorticotrophic hormone stimulation test between patients and controls were detected. (Mean value in patients were 23.078±3.709 ug/dl compared with 24.492±5.006 ug/dl in control group with p value of 0.389). Significant negative correlation was found between serum cortisol and ferritin in patients group (r= 0.625 and p value =0.003)
There was significantly lower basal morning cortisol in sickle-cell anemia patients with significant negative correlation with iron overload so regular follow up to adrenal functions to detect any adrenal impairment, as SCD patients are vulnerable to adrenal hypo function, is recommended.


Aghaa M, Eid AF, Sallam M. Sickle cell anemia: Imaging from head to toe. Egyptian Journal of Radiology and Nuclear Medicine. 2013; 44 (3): 547–561.

Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet 2010 Dec; 376(9757):2018-2031. doi: 10.1016/S0140-6736(10)61029-X. Epub. 2010 Dec 3.

Cresio A, Zilda B. Endocrine disorders in sickle cell disease. Current pediatric reviews J. 2011; 7(1):68-72.

Makino J, Ndzengue A, Adekolujo S, et al. High Prevalence of adrenal insufficiency in patients with sickle cell disease. Exp Clin Endocrinol Diabetes. 2013 Jan; 121(1):32-6. doi: 10.1055/s-0032-1330036. Epub. 2013 Jan 17.

Monica C. Strategy of blood safety in Africa region. In: Tito A and Lewis SM (eds). District laboratory practice in tropical countries, 2nd edition. Cambridge low price Egyptian edition 2005; chapter 4, P. 268-378.

Schneider RG, Hightower B, Hosty TS, et al. Abnormal hemoglobins in a quarter million people. Blood 1976 Nov; 84(5): 629-637.

Bishop NL, Fudy EP, Schoeff L. Priniciples and correlations In: Harris N. and Winter W (eds.) Clinical chemistry, 5th edition, Lippinocott Williams and Wilkins, Philadelphia. 2000; 180-220.

Muntzel M, Hannedouche T, Lacour B, et al. Effect of erythropoietin on hematocrit and blood pressure in normotensive and hypertensive rats. J Am Soc Nephrol. 1992 Aug; 3(2):182-7.

Beard J L. Iron Biology in immune function, muscle metabolism and neuronal functioning. J Nutr. 2001 Feb; 131:568-580.

Stewart PM, Krone NP. The adrenal cortex. In: Kronenberg HM, Melmed S, Polonsky KS, Larsen PR, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Saunders Elsevier.2011: chap 15.

Dorin RI, Qualls CR, Crapo LM. Diagnosis of adrenal insufficiency. Ann Intern Med.2003 Aug; 139 (3): 194– 204.

Bablok W, Passing H, Bender R, et al. A general regression procedure for method transformation. Application of linear regression procedures for method comparison studies in clinical chemistry, Part III. J Clin Chem Clin Biochem. 1988 Nov; 26 (11): 783-790.

Patra PK, Khodiar PK. Study of serum ferritin, iron,total iron binding capacity in sickle cell disease. Journal of Advance Researches. 2012; 4(4):340-344.

Akinbami AA, Dosunmu AO, Adediran AA, et al. Serum ferritin levels in adults with sickle cell disease in Lagos, Nigeria. J Blood Med. 2013 May; 4:59-63. doi: 10.2147/JBM.S42212. Print 2013.

Pandey S, Sharma A, Dahia S, et al. Biochemical indicator of sickle cell disease, preliminary report from India. Indian J Clin Biochem 2012 Apr; 27(2):191-5. doi: 10.1007/s12291-011-0162-y. Epub. 2011 Sep 25.

Mohapatra MK. Type 1 diabetes mellitus in homozygous sickle cell anemia. Journal Assoc Physicians India. 2005 Oct; 53:895-896.

Smiley D, Dagogo-Jack S, Umpierrez G. Therapy insight: Metabolic and endocrine disorders in sickle cell disease. Nat Clin Pract Endocrinol Metab. 2008 Feb; 4(2):102-109. doi: 10.1038/ncpendmet0702

Soliman AT, Darwish A, Mohammed SH, et al. Circulating growth hormone, Insulin-like growth factor, and free thyroxin, GH response to clonidine provocation and CT scanning of hypothalamic-pituitary area in children with sickle cell disease. Journal Trop Pediatric. 1995; 41(5):285-9.

Lawrence Soloman MD. Adrenal insufficiency as a possible contributor to pain in adults with sickle cell disease. Journal of Pain and Symptom Management disease. 2012; 43(2):379.

Saad ST, Saad MJ. Normal cortisol secretion in sickle cell anemia. Trop Geogr Med. 1992 Jan; 44(1-2): 86–88.

Rhodes M, Akohoue SA, Shankar SM, et al. Growth Patterns in Children with Sickle Cell Anemia during Puberty. Pediatr Blood Cancer. 2009 Oct; 53(4): 635-641. doi: 10.1002/pbc.22137.

Ozen S, Unal S, Erçetin N, et al. Frequency and risk factors of endocrine complications in Turkish children and adolescents with sickle cell anemia. Turkish Journal Hematology. 2013 Mar; 30(1): 25-31. doi: 10.4274/tjh.2012.0001. Epub 2013 Mar 5.

El-Sarraf NA, Sulaiman AM, Mansour H. Endocrine disorder in patients with sickle cell anemia. Benha Medical Journal. 2009; 26(2): 467-468.

How to Cite
A Hagag A, S El-Farargy M, M Abo El-Enein A. Study of Adrenal Functions using ACTH stimulation test in Egyptian children with Sickle Cell Anemia: Correlation with Iron Overload. Int J Hematol Oncol Stem Cell Res. 9(2):60-66.