ESPE Abstracts

Background: Multiple Endocrine Neoplasia type 2A (MEN2A) is an autosomal dominant disorder characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and parathyroid hyperplasia. MTC shows near complete penetration and its presentation and prognosis highly depend on RET proto-oncogene mutation. To ensure the favorable prognosis in patients with MEN2A, planning surgical intervention for MTC at appropriate timing is important. We herein report a Japanese girl ...

It is noted that patients with heart disease (HD) are likely to develop abnormal glucose metabolism and this metabolic deterioration usually occurs after middle age. However, we recently had two patients with congenital HD, who developed type 2 diabetes mellitus (DM) in their adolescence.The first patient underwent Fontan operation at 2 years of age for single ventricle and pulmonary atresia. His paternal grandfather had type 2 DM. He noticed polydipsia and polyuria at 15 year...

Context: Maternal Graves’ disease (GD) may lead to not only thyrotoxicosis but also hypothyroidism in neonates. We describe a case of transient central hypothyroidism (CH) due to undiagnosed maternal GD.Case presentation: A 4-month old Japanese girl visited our hospital for the treatment of hypothyroidism. She was born after 37-week gestation, weighting 2320g. She developed hypoglycemia and admitted to the prior hospital. She exhibited hypothyroxine...

Background: Congenital hyperinsulinaemic hypoglycaemia (CHI) can cause various degrees of hypoglycaemia in infancy. In mild CHI, unnoticeable recurrent hypoglycaemia may cause deterioration of central neurological functions in patients. We report a case of mild CHI that presented with developmental delay without any previous hypoglycaemic events.Case presentation: An 18-month-old Japanese girl was admitted to our hospital with seizures and unconsciousnes...

Objective: The aim of this study was to reveal the increase of BMI during later infant related to skeletal maturation in prepubertal obese boys.Subjects and Methods: The subjects were 63 Japanese 10-years old obese boys. Height and weight were measured. Bone age (BA) of left hand-wrist radiographs was assessed using RUS score of the Japanese-standardized Tanner-Whitehouse 2 method. Weight and length or height at birth, 1.5, 3 and 6 years old were obtaine...

Background: In initial treatment of DKA, volume expansion should begin with 0.9% saline to restore the peripheral circulation. The use of large amounts of chloride-rich/bicarbonate-free fluids may cause the rapid development of hyperchloremic metabolic acidosis, which is described in ISPAD Clinical Practice Consensus Guidelines 2018.The severity of DKA, defined by pH, HCO3- Base Excess (BE), is one of the factor...