hrp0098p1-79 | Multisystem Endocrinology | ESPE2024

Clinical Characteristics and Management Outcomes in Girls with McCune-Albright Syndrome: An Experience from a Tertiary Center

Dejkhamron Prapai , Unachak Kevalee , Wejaphikul Karn

Background: The clinical manifestations of McCune-Albright syndrome (MAS) are diverse, typically including fibrous dysplasia (FD), café au lait spots, and endocrine hyperfunction, most commonly gonadotropin-independent precocious puberty (GIPP). Management of endocrinopathies in MAS varies by the affected organs. Treatment options for GIPP include clinical observation, aromatase inhibitors (AIs), ketoconazole, medroxyprogesterone acetate, selective estr...

hrp0098p1-75 | Multisystem Endocrinology | ESPE2024

Endocrine Complications in Childhood Primary Brain Tumor Survivors: A Single Center Study

Chooprasertsuk Nuanpan , Wejaphikul Karn , Dejkhamron Prapai , Wiwattanadittakul Natrujee

Background: Primary brain tumors are the most prevalent solid tumors among children. Both the tumor itself and therapeutic interventions can lead to endocrine complications and metabolic consequences in survivors. However, current information on these complications in childhood brain tumor survivors (CBTS) in Thailand is limited.Objectives: This study aims to evaluate the prevalence of endocrine and metabolic complicatio...

hrp0098p1-119 | Bone, Growth Plate and Mineral Metabolism 2 | ESPE2024

Craniometaphyseal Dysplasia Mimicking Infantile Osteopetrosis: A Case of Skeletal Hyperostosis and Calcipenic Rickets

Wejaphikul Karn , Dejkhamron Prapai , Klangjorhor Jeerawan , Tengsujaritkul Maliwan

Background: Mutations in the ANKH gene affect the cellular transportation of the bone mineralization inhibitor, inorganic pyrophosphate (PPi). Loss-of-function mutations in this gene result in craniometaphyseal dysplasia (CMD), a rare disorder characterized by bone hyperostosis, particularly in the craniofacial bones, and metaphyseal widening of long bones. CMD patients experience impaired transport of PPi to extracellular compartment, leading to exce...

hrp0098p2-236 | Pituitary, Neuroendocrinology and Puberty | ESPE2024

Achieving normal near final height in girls with idiopathic central precocious puberty and early puberty following gonadotropin-releasing hormone analog treatment: Predictors

Puttawong Dolrutai , Dejkhamron Prapai , Wejaphikul Karn , Phinyo Phichayut

Background: Central precocious puberty (CPP) and early puberty (EP) are characterized by premature breast development. Girls with CPP and EP who experience rapid pubertal progression are treated with gonadotropin-releasing hormone analog (GnRHa) to halt pubertal advancement and optimize height potential. However, studies on factors impacting near-final height (NFH) for CPP and EP in Asian girls remain scarce. We aim ed to explore the factors associated with pr...

hrp0082p1-d2-80 | Diabetes (1) | ESPE2014

Methylmalonic Acidemia (MMA) with Unusual Presentation Mimicking Diabetic Ketoacidosis

Dejkhamron Prapai , Wejaphikul Karn , Katanyuwong Kamornwan , Unachak Kevalee , Wattanasirichaigoon Duangrurdee , Tanpaiboon Pranoot

Background: Hyperglycemic ketoacidosis is an acute, life threatening condition requiring early etiologic recognition and management to prevent serious morbidity/mortality. The most common cause is diabetic ketoacidosis (DKA). Organic acidaemias (OAs) are inheritable metabolic disorders caused by defects in protein metabolism resulting in acid accumulation. Patients with metabolic decompensation usually present with lactic and/or ketoacidosis, with/without hypoglycemia. Hypergl...

hrp0097p1-36 | Diabetes and Insulin | ESPE2023

Incidence of Newly Diagnosed Childhood Diabetes and Severity at Onset Between Pre-Pandemic and Pandemic COVID-19 Eras in Northern Thailand

Sinthuprasith Pattharaporn , Wejaphikul Karn , Puttawong Dolrutai , Tang-Ngam Hataitip , Sanrattana Naphatsorn , Unachak Kevalee , Dejkhamron Prapai

Background: The incidence rate (IR) of childhood diabetes is increasing globally. These upward trends were also found in Thailand. However, newly diagnosed childhood diabetes incidences are not updated, especially in Northern Thailand. There are possibilities that COVID-19 pandemic affects the development of acute diabetes after infection and the severity of the first clinical presentation of childhood diabetes. However, data are also conflicting regarding the...

hrp0097p1-591 | Thyroid | ESPE2023

Health-Related Quality of Life in Patients Diagnosed with Childhood Primary Hyperthyroidism

Pitupan Pakanut , Dejkhamron Prapai , Boonchooduang Nonglak , Puttawong Dolrutai , Wongsa Danil , Unachak Kevalee , Wejaphikul Karn

Background: Hyperthyroidism is a common condition in general pediatric practice. Several adult studies show hyperthyroidism affects health-related quality of life (HRQoL). However, information regarding HRQoL in the pediatric population is limited.Objectives: To evaluate HRQoL and psychosocial functioning of patients diagnosed with childhood primary hyperthyroidism compared with healthy controls and to identify the possi...

hrp0097p2-171 | Bone, Growth Plate and Mineral Metabolism | ESPE2023

An Infantile Hypophosphatasia with Novel Mutation in ALPL Gene: A Case Report

Wejaphikul Karn , Sinthuprasith Pattharaphorn , Fanhchaksai Kanda , Kangsuwan Supakanya , Damrongmanee Alisara , Kittisakmontri Kulnipa , Puttawong Dolrutai , Unachak Kevalee , Dejkhamron Prapai

Background: Hypophosphatasia (HPP) is a rare inherited disease of bone metabolism caused by inactivating mutations in the ALPL gene encoding tissue non-specific alkaline phosphatase (TNSAP). Infantile HPP is characterized by early onset abnormal skeletal mineralization with hypercalcemia and low alkaline phosphatase (ALP). It has been rarely reported from Thailand, resulting in limited disease awareness. We reported an infantile HPP Thai patient who p...