ABSTRACT
BACKGROUND: Multi-transfused thalassemia major (TM) patients frequently develop severe endocrine complications, mainly due to iron overload, anemia, and chronic liver disease, which require prompt diagnosis, treatment and follow-up by specialists. The most common endocrine complication documented is hypogonadotropic hypogonadism which increases with age and associated comorbidities. It is thus important for physicians to have a clear understanding of the pathophysiology and management of this disorder. Also to be aware of the side effects, contraindications and monitoring of sex steroid therapy. In this paper, practical ICET-A recommendations for the management of hypogonadism in adult females with TM are addressed. METHODS: In March 2015, the Coordinator of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) conducted a two-step survey to assess the attitudes and practices of doctors in the ICET-A network taking care of adult female TM patients with hypogonadism. They were clinically characterized by the absence of pubertal development or discontinuation or regression of the maturation of secondary sex characteristics, and biochemically by persistent low FSH, LH and estradiol levels. Recently a supplementary survey on adult female hypogonadism in TM was undertaken within the ICET-A network. RESULTS: The completed questionnaires were returned by 16 of 27 specialists (59.2%) following 590 female TM patients over the age of 18 years; 315 patients (53.3%) had hypogonadism, and only 245 (74.6%) were on hormone replacement therapy (HRT). Contraceptive oral pills (COC) were the first treatment choice in 11 centers (68.7%). A wide range of COCs was used with different progestin contents. In general, the patients' compliance to treatment was reported as good in 81.2 % of centers. The frequency of required tests for follow-up HRT, in addition to the regular check-up for thalassemia, was variable in the participating centers. CONCLUSIONS: Doctors taking care of TM patients should have sound knowledge of the pathophysiology of hypogonadism in adult females with TM. They should know the potential effects of HRT including advantages and disadvantages of estrogen and progestins. Moreover, they should keep in consideration the emotional needs of these patients dreaming of attaining a full pubertal development.
ABSTRACT
In a review of 109 cases reported in the literature, including our own experience with two successful right laparoscopic adrenalectomies performed in a 3-year old girl for androgen-secreting adenoma and in a 9-year-old male for pheochromocytoma, we analysed the indications, surgical techniques and results of video-assisted (laparoscopic or retroperitoneoscopic) adrenalectomy in children. The indications are no different from those for traditional surgery. It seems that there are no age or tumour size limits for a well-trained surgical team. The best endoscopic approach needs to be more clearly defined. Experience shows that laparoscopy is undoubtly preferred for right adrenalectomy (95.2% of cases), while left adrenalectomy has been performed by retroperitoneoscopy in 30% of cases. Considering the conversion rate of laparoscopy vs retroperitoneoscopy (12.5% vs 28.5%), right laparoscopic vs right retroperitoneoscopic adrenalectomy (4.7% vs 100%) and left laparoscopic vs left retroperitoneoscopic adrenalectomy (5% vs 16.6%) and on the basis of our experience in adults, we recommend laparoscopic adrenalectomy via a transperitoneal route in 45-degree flank decubitus for both right and left adrenal lesions. However, we think that the best surgical result can be achieved if the paediatric and adult surgeon collaborate with their different experience and expertise. As a technical point, we would like to stress that because of the child's small peritoneal cavity, trocar placement must be lower than in adults. Lastly, we suggest the use of new technological devices such as the Ultracision Harmonic Scalpel, which was a critical factor in our two successful right laparoscopic adrenalectomies.
