Endocrine Abstracts

Background: Pheochromocytoma (PHEO) and paraganglioma (PGL) of abdominal origin secrete catecholamines which are metabolized to metanephrines. Head-and-neck paraganglioma (HNPGL) are considered as non secretary tumors.Objectives: To find the utility of plasma free metanephrine (MN), normetanephrine (NMN) and 3-methxytyramine (3-MT) for the differential diagnosis PHEO/PGL after excluding MEN2 and VHL patients.Methods: A total of 79 ...

Cadaveric islet transplantation can cure diabetes, however scarcity of donor islets means that this approach cannot be used for large-scale treatment. An alternative source of insulin-producing β-cells or whole islets would be step-wise in vitro differentiation from either human embryonic stem cells or induced pluripotent stem cells, which could be transplanted directly into patients. However, ‘β-like’ cells generated from existing differentiation ...

Background: Aortic sized index (ASI) defined as aortic dimensions/body surface area (BSA), has been proposed as a method of identifying aortic dilatation in Turner syndrome. A recent paper reported centile charts of aortic dimensions across for BSA using echocardiogram in 451 children and adults with TS allowing for calculation of Z scores.1Methods: We report Z scores for aortic root adjusted for BSA from clinical echocardiog...

Background: In 2007, the Turner syndrome (TS) consensus study group developed an international guideline for clinical care of girls and women with TS. Given emerging concerns of long term cardiovascular complications, the consensus recommends that cardiac MRI should be performed when girls are old enough to tolerate the procedure or at the time of transition and to be repeated at least every 5–10 years.Method: We conducted a survey ...

Aims: The aim of this study is to evaluate sitting height (SH) and leg length (LL) in girls with Turner syndrome.Methods: Retrospective study of SH and LL SDS, using SH–LL SDS (~0 in a proportionate child) as a measure of disproportion in 76 girls with Turner syndrome. Eligible girls were aged at least 4 years, had not started recombinant GH, and had no other chronic disease. 40 girls with measurements prior to pubertal induction and at adult height...

Background: Pheochromocytoma (PHEO) and paraganglioma (PGL) are the tumors of adrenal medulla and extra adrenal ganglia respectively. Most of these PHEO/PGL are benign and may become malignant, if remain undiagnosed/untreated for a longer time. Vaginal PGL are extremely rare. There is not much published literature on vaginal PGL.Objective: To carry out biochemical and genetic analysis of the patient with MIBG negative malignant vaginal PGL.<p class="...

Enzymatic 5′- and 5-deiodination represent key pathways for local and systemic and local activation and inactivation of iodothyronines and iodothyronamines. Expression of the three respective deiodinases (DIO) is regulated by thyroid parameters, local and systemic inflammatory status as well as by selenium supply. In addition, the DIO isoenzymes are intended but also unanticipated target proteins of pharmacological, nutritive and environmental substances that might inter...

Growth hormone treatment (GHT) is used to improve height, and potentially quality of life (QOL), in children with abnormal growth patterns. Previous QOL research suggests children with acquired growth hormone deficiency (AGHD) benefit more from GHT than those treated for other conditions.The aim was to determine child and parent reported QOL change over 1 year depending on GHT and diagnosis.One hundred and twenty-two children (mean...

Objective: To assess prevalence, clinical features, and follow-up of renal/urological malformations in patients with Turner syndrome (TS).Methods: The medical records of 182 patients with TS born between 1970 and 2013 were retrospectively reviewed.Results: Twenty-one girls (11.5%) were identified with renal/urological anomalies: 15 (71%) horseshoe kidney (HSK), 1 (4.7%) malrotation, 2 (9.5%) single kidney, and 1 (4.7%) duplex colle...

Aim: To review trends in bisphosphonate use in children with secondary osteoporosis attending a Tertiary Paediatric Endocrine Unit (2004–July 2015).Methods: Data were gathered from a combination of a clinical and pharmacy database. Results reported as median (range).Results: A total of 42 children (19M) commenced on bisphosphonates treatment over the 11-year period, median age 11.8 years (3.3–18.4). 19 were on pamidronate...