hrp0092p2-190 | Growth and Syndromes (to include Turner Syndrome) | ESPE2019

Growth Hormone Therapy in Patients with Noonan Syndrome

Apperley Louise , Ramakrishnan Renuka , Dharmaraj Poonam , Das Urmi , Didi Mohammed , Blair Jo , Senniappan Senthil

Introduction: Noonan Syndrome (NS) is an autosomal dominant condition that affects 1 in 1000 to 2500 live births and is associated with short stature. Studies have shown that height velocity (HV) improved significantly with the use of growth hormone (GH) in the first year of treatment and sustained for the second year. We aimed to look at the effects of GH therapy in our cohort of patients with NS.Method: In this retrosp...

hrp0089p1-p163 | Growth & Syndromes P1 | ESPE2018

The Association between Growth Hormone Dose and Short-Term Height Outcomes in a Large Cohort of Paediatric Patients with Turner Syndrome: Real-World Data from the NordiNet® International Outcome Study (IOS) and ANSWER Program

Blair Jo , Rohrer Tilman R. , Tonnes Pedersen Birgitte , Roehrich Sebastian , Backeljauw Philippe

Objectives: The recently updated clinical practice guidelines for Turner syndrome (TS) recommend a growth hormone (GH) dose of 45–50 μg/kg/day, increasing to 68 μg/kg/day in case adult height potential is substantially compromised (1). Real-world data on the modifiable factors impacting near-adult height in GH-treated TS patients are limited, but short-term responsiveness to GH has been suggested as one factor (2). We, therefore, analysed the impact of GH dose o...

hrp0089p2-p252 | Growth & Syndromes P2 | ESPE2018

Final Results of NordiNet® International Outcome Study: Key Outcomes in Paediatric Patients

Polak Michel , Blair Jo , Rohrer Tilman R. , Pietropoli Alberto , Tonnes Pedersen Birgitte , Savendahl Lars

Background: NordiNet® International Outcome Study ([IOS]; NCT00960128), a non-interventional study (2006–2016), assessed the effectiveness and safety of real-world treatment with Norditropin®. Outcomes were assessed in children with growth hormone deficiency (GHD), born small for gestational age (SGA), Turner syndrome (TS), chronic renal disease (CRD), idiopathic short stature (ISS), Noonan syndrome (NS) and Prader-Willi syndrome (PWS)....

hrp0089p1-p198 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

A National UK Guideline for Managing Pituitary Adenomas in Children and Young People Under 19 Years Developed According to the AGREE II Framework

Blair Jo , Korbonits Marta , Ronaldson Amy , Dang Mary N , Spoudeas Helen

Pituitary adenomas are usually benign tumours arising from the hormone-secreting cells of the anterior pituitary gland. These adenomas can result in excess hormone secretion and the development of characteristic syndromes, such as Cushing’s disease, acromegaly and hyperprolactinaemia, and/or mass effects on surrounding vital structures causing for example visual disturbances and pituitary hormone deficiencies. In children and young people under 19 years (CYP), the managem...

hrp0086p2-p51 | Adrenal P2 | ESPE2016

Bone Health Index in Children and Adolescents with Congenital Adrenal Hyperplasia

Alsaffar Hussain , Davies Rosie , Reed John , Das Urmi , Senniappan Senthil , Didi Mohammed , Blair Jo

Introduction: Patients with congenital adrenal hyperplasia (CAH) require life long glucocorticoid (GC) therapy. In CAH, the adverse effect of GC on bone health (BH) may be counteracted by the effect of modest elevations in adrenal androgens.Aim: To examine relationships between BH index (BHI) SDS, calculated by BoneXpert on bone age (BA) x-rays, BA, hydrocortisone (HC) dose (mg/m2 per day), and mean 17-hydroxyprogesterone (17-OHP) concentratio...

hrp0086p1-p371 | Gonads & DSD P1 | ESPE2016

Androgen Profile Differs to Adults in Adolescent Girls with Polycystic Ovary Syndrome

Frerichs Carley , Das Urmi , Garden Ann , Williams Cara , Dharmaraj Poonam , Didi Mohammed , Ramakrishnan Renuka , Blair Jo

Background: Diagnostic criteria for polycystic ovarian syndrome (PCOS) are well established in the adult population, but may not be appropriate for adolescent girls. Clinical and/or biochemical hyperandrogenism is one important diagnostic criterion. Screening for hyperandrogenism is often restricted to measurements of testosterone in adult practice. It was our impression that this strategy would under-diagnose PCOS in our adolescent population.Objective ...

hrp0082p2-d3-480 | Hypoglycaemia | ESPE2014

The Role of Plasma C-Peptide Concentration in the Diagnosis of Congenital Hyperinsulinism

Green Julie , Giri Dinesh , Gangadharan Arundoss , Blair Jo , Dharmaraj Poonam , Das Urmi , Senniappan Senthil , Didi Mohammed

Background: The hallmark of congenital hyperinsulinism (CHI) is the demonstration of detectable plasma insulin during hypoglycaemia. Insulin can be undetectable in a significant proportion of patients with CHI. Plasma samples for insulin requires rapid and careful handling for reliable results. There is little published data on the value of C-peptide in the diagnosis of CHI.Objective and hypotheses: To assess the usefulness of C-peptide in the diagnosis ...

hrp0086p2-p840 | Syndromes: Mechanisms and Management P2 | ESPE2016

Testosterone Therapy Improves the First Year Height Velocity in Adolescent Boys with Constitutional Delay of Growth and Puberty

Giri Dinesh , Patil Prashant , Blair Jo , Das Urmi , Ramakrishnan Renuka , Dharmaraj Poonam , Didi Mohammed , Senniappan Senthil

Background: Constitutional Delay of Growth and Puberty (CDGP) is a transient state of hypogonadotropic hypogonadism associated with prolongation of childhood phase of growth, delayed bone age and pubertal delay. CDGP can cause significant psychological stress and anxiety in adolescent boys. Although testosterone usage in this group has not been shown to affect the final adult height, the effect on the first year height velocity is not widely reported.Obj...

hrp0086p2-p773 | Pituitary and Neuroendocrinology P2 | ESPE2016

Primary Thirst Defect is a Rare But Important Complication Following Surgery for Hypothalamic Hamartoma and Intractable Epilepsy

Giri Dinesh , Blair Jo , Das Urmi , Dharmaraj Poonam , Senniappan Senthil , Malluci Connor , Benedetta Pettorini , Pizer Barry , Burns Sasha , Didi Mohammed

Background: Diabetes insipidus (DI) is a well-recognized post neuro surgical complication arising after hypothalamic-pituitary surgery. DI occurring in the post-operative period can be transient happening within 24–48 hour of surgery, secondary to trauma to the connections between the magnocellular bodies and the nerve terminals in the posterior pituitary, or to axonal shock from disturbances in the vascular supply to the pituitary stalk and posterior pituitary.<p cla...

hrp0086p2-p683 | Growth P2 | ESPE2016

Bone Health Index: A Potential Discriminator between Growth Hormone Deficiency and Constitutional Delay in Growth and Puberty in Adolescent Children

Patil Prashant , Dharmaraj Poonam , Povall Ann , Abernethy Laurence , Das Urmi , Didi Mohommed , Ramkrishnan Renuka , Senniappan Senthil , Blair Jo

Background: Constitutional delay in growth and puberty (CDGP), the most common cause of short stature in children, is a transient state of delayed growth, skeletal maturation and attenuated pubertal growth spurt. It is not always easy to differentiate from GH deficiency (GHD) even with robust clinical and auxological assessment, measurement of IGF1 and bone age evaluation Bone health index (BHI) is a quantitative measure of bone health calculated from a hand and wrist X-ray us...