hrp0097p2-210 | Diabetes and Insulin | ESPE2023

Idiopathic Chronic Calcific Pancreatitis (ICCP) presenting with fibro calculus pancreatic diabetes (FCPD) - a rare case in a 9-year-old Sri Lankan boy.

Arsadh Muhammadh , Kahandawa Shyaminda , Suntharesan Jananie

Introduction: Chronic pancreatitis is defined as recurrent or persistent pancreatitis, which will result in irreversible morphological change in pancreatic structure leading to pancreatic exocrine and endocrine insufficiency. ICCP is a rare condition and only few cases in adults have been reported in Sri Lanka. Prevalence of ICCP is highly variable in Asian and western countries. We present a rare case of FCPD in a 9 a years old boy.<str...

hrp0095p2-102 | Fat, Metabolism and Obesity | ESPE2022

Long term effect of metformin therapy in children and young people with obesity - A single center experience

Suntharesan Jananie , Pickles Rosanna , John Reed , Apperley Louise , Senniappan Senthil

Introduction: The prevalence of childhood obesity is increasing worldwide, and the management remains a challenge. The mainstay of management is lifestyle modification but if this is not successful, pharmacological agents are considered although the options in children and young people are quite limited. Metformin has been used as an adjunct therapy alongside lifestyle modification in children and young people with obesity. Some studies have shown it to be eff...

hrp0092p3-304 | Late Breaking Abstracts | ESPE2019

Congenital Craniopharyngioma - A Rare Case of Congenital Hypopituitarism

Suntharesan Jananie , Atapattu Navoda , Prematilake Dilusha , Hashim Raihana , Gunasekara Buddhi

Introduction: Hypopituitarism leads to one or more pituitary hormones deficiency. Hypopituitarism can be congenital or acquired. The incidence of congenital hypopituitarism is between 1 in 4000 and 1in 10 000 live births. Children with congenital hypopituitarism may present with hypoglycemia, hyponatremia, shock, micro phallus in males, and later present with growth failure. It can be due to congenital or acquired causes. Adamantinomatous type craniopharingiom...

hrp0092p3-321 | Late Breaking Abstracts | ESPE2019

Hydrometrocolpos Due to Congenital Adrenal Hyperplasia – A Rare Cause of Bladder Outflow Tract Obstruction in a Female Child

Suntharesan Jananie , Atapattu Navoda , Gunasekara Buddhi , De silva Dimarsha

Introduction: Hydrometrocolpos (HMC) develops in a female child as a result of a vaginal outflow tract obstruction and accumulation of secretions. HMC can have associated with other malformations or associated syndromes. Imperforated hymen, vaginal atresia, persistent urogenital sinus, and cloacal malformation, are the common causes for HMC. Congenital adrenal hyperplasia causing androgen exposure during the fetal life leads to varying degree of ambiguous geni...

hrp0097p1-227 | Bone, Growth Plate and Mineral Metabolism | ESPE2023

Parathyroid Adenoma in a 12-year- old Child: A Case with Unusual Presentation

Arsadh Muhammadh , Mahaliyana Dileepa , Manathunga Rasitha , Kahandawa Shyaminda , Suntharesan Jananie

Introduction: Acute pancreatitis (AP) is rarely seen in children and is typically not associated with common aetiologies seen in adults. AP secondary to hypercalcaemia due to primary hyperparathyroidism (PHPT) is very rare. The annual incidence of PHPT in adults is 30/100,000 with female predominance and 2–5/100,000 in children regardless of sex. We present a rare presentation of PHPT presenting with AP in a 12-year-old boy.<strong...

hrp0092p3-291 | Late Breaking Abstracts | ESPE2019

Rare Causes for Paediatric Virilizing Tumors

Suntharesan Jananie , Atapattu Navoda , Prematilake Dilusha , Hashim Raihana , Gunasekara Buddhi , De silva Dimarsha

Introduction: Excessive androgen secretion from gonads, adrenal gland and tumors arising from germ cells lead to gonadotropin independent precocious puberty in male and virilization in females. Rapid progression of symptoms with peripheral precocity need urgent evaluation to identify the underlying etiology. We report three cases of malignancy with excess androgen secretion within a year in a tertiary care children hospital in Sri Lanka....

hrp0089p3-p058 | Bone, Growth Plate &amp; Mineral Metabolism P3 | ESPE2018

Severe Neonatal Hypercalcemia: A Challenging Case

Hashim Raihana , Prematilake Dilusha , Gunasekara Buddi , Suntharesan Jananie , Kollurage Udeni , Atapattu Navoda

Neonatal severe hyperparathyroidism (NSHPT) is a rare autosomal recessive disorder due to inactivating mutations of calcium-sensing receptor. These receptors are vital in calcium homeostasis and are expressed in a number of tissues such as parathyroid glands, renal tubules and bone. Homozygous mutations lead to severe hypercalcemia and life-threatening bone demineralization if untreated. A neonate born to 2nd degree consanguineous parents presented with poor feeding...

hrp0094p2-418 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

46 XX, SRY negative testicular DSD presenting with VACTERL Syndrome

Suntharesan Jananie , Gunasekara Budhi , Lakmini Chamila , De Silva Uamd , Navoda Atapattu

Introduction: Sex determining region Y (SRY gene) located in chromosome Yp11.3, initiate gonadal differentiation into testes, which subsequently leads to development of Wolffian duct, male external genitalia, and regression of Mullerian duct. Not only SRY, SOX9, FGF9, DAX1, WT1, RSPO1, and SOX10 contribute for gonadal differentiation. Imbalance of expression of above genes in the absence of SRY leads to 46, XX testicular DSD. We describe a case of 46...

hrp0097p1-73 | Fat, Metabolism and Obesity | ESPE2023

Insulin as a treatment modality for familial chylomicronemia syndrome in resource-limited settings- A Case series

Jebaseeli Hoole Thabitha , Suntharesan Jananie , Jayasundara Imalka , Siriwardne Dinendra , Naotunna Chamidri , Jasinghe Eresha , Atapattu Navoda

Introduction: Familial chylomicronemia syndrome (FCS) is an extremely rare monogenic disease with a prevalence of 1-2:1,000,000. Defects in lipoprotein lipase (LPL) are the main cause. Recurrent acute pancreatitis is a life-threatening complication of FCS. Insulin therapy is known to be a mode of treatment for hypertriglyceridemia.Case series: We present four children with genetically confirmed LPL deficiency followed up...

hrp0095p2-211 | Multisystem Endocrine Disorders | ESPE2022

McCune Albright Syndrome in children- Case series from a Tertiary Care Centre- Sri Lanka

Siriwardhane Dinendra , Atapattu Navoda , Naotunna Chamidri , Hashim Raihana , Premathilake Dilusha , Gunasekara Buddhi , Suntharesan Jananie , De Silva Dimarsha , Lakmini Chamila , Gamage Senani

McCune Albright syndrome (MAS) is rare with a prevalence of 1 in 100,000 to 1 in 1,000,000, characterized by the triad of monostotic/polyostotic fibrous dysplasia (FD), café au lait skin pigmentation, and hyperfunctioning endocrinopathies caused by somatic activating mutations of the GNAS1 gene encoding the α subunit of guanine nucleotide-binding protein. Here we are reporting three cases of MAS who are actively being followed up in a leading Children’s Hosp...