hrp0094p2-214 | Fat, metabolism and obesity | ESPE2021

A study on the Complications associated with Severe Obesity in Children and Young People

Apperley Louise , Senniappan Senthil ,

Introduction: The complications of obesity are well recognised and documented in the adult population. There is little data available that reports the complications of childhood obesity seen in clinical practice. The aim of our study is to investigate the different types of complications seen in children and young people (CYP) with obesity.Methods: 125 CYP aged 0-18 years who had a body mass index (BMI) standard deviatio...

hrp0092p1-113 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Delayed Puberty in A 16-Year-Old Male Associated with Gamma Aminobutyric Acid Capsule Supplements

Blackburn James , Senniappan Senthil , Ahmed Syed Harris

Background: Delayed puberty is defined as the absence of physical signs of puberty 2 to 2.5 standard deviations greater than the mean and affects 2% of the adolescent population. We present a male patient aged 16, presenting with delayed puberty. On direct questioning the patient revealed he had been taking regular Gamma-Aminobutyric Acid (GABA). These supplements appeared to suppress the hypothalamic-pituitary-gonadal (HPG) axis.<st...

hrp0092p2-236 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Isolated Premature Menarche into Two Siblings with Neurofibromatosis Type 1

Blackburn James , Didi Mohammed , Senniappan Senthil

Background: Neurofibromatosis type 1(NF1) is an autosomal dominant genetic disorder caused by NF1 mutation. The condition is typified by the development of benign and malignant tumours in both the central nervous system and peripheral tissues. We present two siblings diagnosed with NF1 who presented at an early age with isolated menarche.Case Presentation: Sibling 1 – A 7-year-old patient w...

hrp0089p2-p385 | Thyroid P2 | ESPE2018

Thyroid Hormone Receptor β (THRB) Mutation: Two New Cases of Heterozygous Mutation with Significant Family History

Blackburn James , Senniappan Senthil , Giri Dinesh

Introduction: Resistance to thyroid hormone (RTH) is a relatively uncommon disorder that is usually associated with mutations in thyroid hormone receptor (THR) beta, although rarely THR alpha mutations have been described. RTH beta (RTHB) is often inherited in an autosomal dominant pattern. We describe two cases of RTHB to highlight the significant variations in both clinical presentation and family history.Case 1: A 2 year old patient ...

hrp0094p1-113 | Fat, Metabolism and Obesity B | ESPE2021

Are you considering Idiopathic Intracranial Hypertension when evaluating a patient with obesity?

Apperley Louise , Kumar Ram , Senniappan Senthil ,

Introduction: Obesity is a recognised risk factor for raised intracranial pressure in the adult population but is still under-recognised in children and young people. The pathophysiology of raised intracranial pressure in relation to obesity remains unclear. The aim of our study is to investigate the association between idiopathic intracranial hypertension (IIH) and obesity in children and young people.Method: Patients d...

hrp0094p2-377 | Pituitary, neuroendocrinology and puberty | ESPE2021

Use of Tolvaptan in a child with SIADH post pituitary surgery

Lohiya Nikhil , Didi Mohamed , Senniappan Senthil ,

Introduction: Post-operative management of fluid and electrolyte imbalance after surgery for pituitary or suprasellar tumors could be challenging. Post-operative course could include diabetes insipidus (DI) (transient or permanent) or a classical triphasic response (initial phase of DI followed by a second phase of transient SIADH and third and final phase of permanent DI). Mainstay of management of SIADH in these patients involves fluid restriction. At times,...

hrp0089p3-p062 | Bone, Growth Plate &amp; Mineral Metabolism P3 | ESPE2018

Idiopathic Juvenile Osteoporosis: Common Symptoms in an Uncommon Condition

Uppal Saurabh , Senniappan Senthil , Dharmaraj Poonam , Hughes David

Introduction: Osteoporosis in children and young people can be primary due to Osteogenesis Imperfecta (OI) or secondary to chronic disease. We report 2 patients with Idiopathic Juvenile Osteoporosis (IJO), a rare primary osteoporotic disorder.Case 1: A 12 year old boy presented with 12 months of lower back pain and stiffness, against a background of chronic pain in knees, wrist and ankles. There was no history of fractures or systemic disease. Examinatio...

hrp0089p2-p190 | Fetal, Neonatal Endocrinology and Metabolism P2 | ESPE2018

Atypical Hepatoblastoma and Wilm’s Tumour in an Infant with Beckwith-Wiedemann Syndrome and Diazoxide Resistant Congenital Hyperinsulinism

Uppal Saurabh , Senniappan Senthil , Didi Mohammad , Hayden James

Introduction: Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that could be associated with embryonal tumours. Genotype based categorisation of patients enables better screening strategies. We present a patient with BWS who developed atypical congenital hepatoblastoma and atypical Wilms tumour in infancy.Case Report: A 2 day old infant was referred with recurrent hypoglycaemia and high intravenous glucose requirement [>12 mg/kg/min]. He ...

hrp0089p3-p178 | Fetal, Neonatal Endocrinology and Metabolism P3 | ESPE2018

Congenital Hyperinsulinism in Children with Beckwith-Wiedemann Syndrome

Gubaeva Diliara , Melikyan Maria , Didi Mohammed , Senniappan Senthil

Introduction: Beckwith-Wiedemann syndrome (BWS) is a multisystem imprinting disorder. Approximately 50% of patients with BWS develop congenital hyperinsulinism (CHI). In this report, we describe the main clinical features in a group of patients with BWS and CHI.Study: Clinical and laboratory data was collected from all patients with BWS under the care of endocrine units at Alder Hey Children’s Hospital (Liverpool, UK) and Endocrine Research Centre (...

hrp0082p3-d1-876 | Perinatal and Neonatal Endocrinology | ESPE2014

Biochemical Studies in Patients with Hyperinsulinaemic Hypoglycaemia

Otaibi Hessah Al , Senniappan Senthil , Alam Syeda , Hussain Khalid

Background: Hyperinsulinaemic hypoglycaemia (HH) is characterized by the dysregulated secretion of insulin from the pancreatic β-cell. It is a major cause of severe and persistent hypoglycaemia in the newborn period. The rapid diagnosis and avoidance of recurrent episodes of hypoglycaemia are vital in preventing brain damage.Objective and hypotheses: To assess if the serum insulin measured at the time of hypoglycaemia in neonates with HH could be co...