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Hereditary haemoglobinopathies are the most common group of monogenic hereditary diseases in the world. Erythrocytes in sickle form, cellular expression of polymerization of deoxygenated HbS, cause intermittent vascular obstruction, leading to tissue ischaemia and consequent chronic damage in organs and endocrine glands. Objective: The evaluation of the growth pattern and pubertal development of a group of patients with sickle-cell anaemia (SCA) from childhood to adulthood. Methods: Thirty patients with SCA between the ages of 10 and 23 years were evaluated in a prospective longitudinal study at three points in time (Te1: 2005; Te2: 2010 and Te3: 2015) and compared with controls. Anthropometric, pubertal and hormonal evaluations were carried out. Age- and gender-specific Z-scores for weight, height and BMI (body mass index) were calculated according to the reference growth standards. Results: Thirty patients with SCA (mean age = 13.93 years) were evaluated at Te1 and 26 patients (mean age = 25.08 years) at Te3. The SCA group lower showed Z-scores for weight (p = 0.0002), height (p = 0.0184) and BMI (p = 0.0011) than the control group at Te1. At Te3, there was no difference in height, but weight (p = <0.0001) and BMI (p = <0.0001) were lower in the SCA group. Men showed greater weight commitment than women at the three study times (Te1: p = 0.0340, Te2: p = 0.0426 and Te3: p = 0.0387) and lower BMI in Te3 (p = 0.0155) in the SCA group. There was a significant increase in weight when comparing Te1 with Te3 (p = 0.0009) and in height when comparing Te1 with Te2 (p = 0.0292) and with Te3 (p = 0.0003) in the SCA group. There was a significant increase in weight when comparing Te1 and Te3 (p = 0.0009) and in height when comparing Te1 and Te2 (p = 0.0292) and Te3 (p = 0.0003) in the SCA group. At Te1, 14 cases and 2 controls were prepubertal. Bone age was delayed in 12 patients. Age at menarche was delayed and lower in the SCA group (mean = 15 years). Five patients had gestated, but no patient had experienced fatherhood. At Te1, TSH levels were higher (p = 0.0080) and T3 levels were lower (p = 0.0020) in the SCA group. At Te3, LH and FSH levels were higher in men with SCA (p = 0.0014; p; 0.0002). IGF-I levels were lower in cases both at Te1 (p = 0.0002) and at Te3 (p = 0.0032). Conclusions: Patients with SCA showed growth impairment and pubertal delay compared with healthy controls. However, albeit belatedly, they reached normal sexual maturation and height in adulthood. Women with SCA showed no fertility problems. The findings highlight the need to investigate the intention of paternity and fertility among men with SCA.

Growth and puberty in a prospective cohort of patients with sickle-cell anaemia: an assessment over ten years abstract