ESPE Abstracts

Background: In Japan, diabetic screening for school children using urinalysis has been performed to detect diabetes mellitus during childhood throughout the country. However, inadequate follow-up system after urinary screening caused several problems. The rate of participation in workup examination still remains less than 30%. Also, accurate annual incidences of children with type 1 diabetes mellitus (T1DM) and type 2 diabetes mellitus (T2DM) detected by screening of urine glu...

Background: Appropriate management for hyperglycemia is essential in preterm infants, because hyperglycemia increase the risk for intracranial hemorrhage, sepsis, retinopathy of prematurity, impairing long outcome to mortality and morbidity. In general, transient neonatal hyperglycemia is frequently observed during glucose infusion therapy, and it may not require interventions other than reducing glucose infusion. On the other hand, extremely preterm infants (EPIs GA <28 w...

Background: Prader–Willi syndrome (PWS) patients have been followed in our country in different ways but without a reference center. In January 2015, we started a PWS reference center in Sao Paulo University to promote a better care for patients and families and to support them with a multidisciplinary team, including pediatric endocrinologist, dietician, nurses, neurologist specialized in sleep disorders and otorhinolaringologist.Method: Forty-two ...

Objective and hypotheses: To describe the morphological characteristics of patients with Prader-Willi Syndrome (SPW) who have been followed in a Pediatric Endocrinology Outpatient Clinic.Method: We performed a retrospective study on 51 patients evaluating the age of diagnosis, genetic mutation, use of GH (rhGH), age of beginning of follow-up, and z-score of weight, height and BMI. Data on their first and latest visit to our clinic were compared....

Background: Obesity in Prader-Willi Syndrome has peculiar features associated to reduced lean body mass which could confer different metabolic characteristics.Objective and hypotheses: The aim of this study is to describe and to compare the metabolic profile in obese patients and obese Prader-Willi syndrome patients (OPWS) followed in a Pediatric Endocrinology outpatient clinic.Method: 45 obese and 22 OPWS patients between 8 and 20...

Background: While existing guidelines recommend long-term follow-up of childhood cancer survivors (CCS), transitional care among pediatric and adult endocrinologists has not been established in Japan.Objective and Hypotheses: To know the present situation and to cultivate a better understanding, we had conducted a nation-wide survey targeting Japanese pediatric endocrinologists.Method</stro...

Background: While existing guidelines recommend long-term follow-up of childhood cancer survivors (CCS), their fertility has not been clarified in Japan.Objective and hypotheses: To address this issue, we organized a working panel to compile evidence from CCSs. The team consisted of various medical specialists in foundation hospitals.Method: We had conducted the questionnaire surveys targeting pediatric endocrinologists regarding r...

Background: Mixed gonadal dysgenesis (MGD) is a heterogeneous group of gonadal, chromosomal and phenotypic abnormalities. The diagnosis is based on the presence of testicular tissue and streak.Objective and hypotheses: Casuistics description of patients with MGD in our hospital.Method: A retrospective analysis of medical records.Results: We studied 15 patients. The age at first visit ranged from two weeks to ...

Background: In X-linked hypophosphatemia (XLH), abnormally elevated serum fibroblast growth factor 23 (FGF23) results in low renal maximum threshold for phosphate reabsorption (TmP/GFR), low serum phosphorus (Pi), inappropriately normal 1,25-dihydroxyvitamin D (1,25(OH)2D) and development of rachitic deformities.Methods: Up to four s.c. KRN23 doses were given every 28 days to 28 adults with XLH according to a dose-escalation algorithm (0.05&#1...

Background: In X-linked Hypophosphatemia (XLH), abnormally elevated serum Fibroblast Growth Factor 23 (FGF23) results in low renal maximum threshold for phosphate reabsorption (TmP/GFR), low serum phosphorus (Pi), inappropriately normal 1,25-dihydroxyvitamin D (1,25(OH)2D) and development of rachitic deformities.Methods: Up to four SC KRN23 doses were given every 28 days to 28 adults with XLH (26 completed) according to a dose-escalation algorithm (0.05&...