Clinical Pediatric Endocrinology
Online ISSN : 1347-7358
Print ISSN : 0918-5739
ISSN-L : 0918-5739
Volume 27, Issue 1
Displaying 1-7 of 7 articles from this issue
Review
  • Tatsuhiko Urakami
    2018 Volume 27 Issue 1 Pages 1-8
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    The principal treatment for children and adolescents with type 2 diabetes is dietary and exercise management. However, the blood glucose levels of some patients receiving this treatment fail to improve; thus, pharmacological treatment is eventually required. The pathophysiology of type 2 diabetes in pediatric patients appears to be similar to that in adults; thus, the range of antidiabetic drugs used in adults is likely to be effective in pediatric patients as well. However, in the majority of countries, including Japan, only metformin, glimepiride, and insulin have been approved for use in pediatric patients. Indeed, the evidence for the usefulness of antidiabetic drugs other than metformin and insulin in children and adolescents is limited at this time. Therefore, the efficacy and safety of various antidiabetic drugs, including DPP-4 inhibitors, GLP-1 receptor agonists, and SGLT2 inhibitors, which are used in adult patients, should be evaluated in the pediatric population in a large number of centers worldwide. In addition, it is critical that researchers and clinicians establish treatment guidelines for children and adolescents with type 2 diabetes in all racial groups worldwide.

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Original Article
  • Takako Takeuchi, Yuko Yoto, Akira Ishii, Takeshi Tsugawa, Masaki Yamam ...
    2018 Volume 27 Issue 1 Pages 9-18
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    We present a 4-yr-old boy with adrenocortical carcinoma (ACC), diagnosed due to the appearance of gynecomastia as the presenting symptom. Six months prior to admission, an acute growth spurt along with the development of bilateral breast swelling was observed. He did not present any features of virilization, including enlargement of the testes, increase in testis volume, and penis size. Laboratory investigations showed gonadotropin-independent hypergonadism, with low LH/ FSH levels and elevated estradiol/testosterone levels. Abdominal computed tomography revealed a large heterogeneous mass adjacent to the right kidney and below the liver. Pathological investigations of the biopsy specimen demonstrated that the tumor was an ACC. Pre- and post-operative combination chemotherapy with mitotane was administered and surgical resection was carried out. Post-surgery, the elevated estradiol/testosterone concentrations reverted to within the reference range. Urinary steroid profile and tissue concentration analysis of estradiol and testosterone indicated the presence of estrogen in the ACC tissue. An investigation for TP53 gene aberrations revealed the presence of a germline point mutation in exon 4 (c.215C>G (p.Pro72Arg)). In ACC, the most common symptom is virilization, and feminization, characterized by gynecomastia, is very rare. However, a diagnostic possibility of ACC should be considered when we encounter patients who have developed gynecomastia without the influence of causative factors such as obesity or puberty, and do not present with the typical signs of virilization.

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  • Yuichi Nakagawa, Toshiki Nakanishi, Eiichiro Satake, Rie Matsushita, H ...
    2018 Volume 27 Issue 1 Pages 19-29
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    The purpose of this study was to clarify the degree of early postnatal growth by birthweight and detect early predictive factors for pediatric obesity. Body mass index (BMI) and degree of obesity were examined in children in the fourth year of elementary school and second year of junior high school. Their BMI at birth and three years of age were also examined. Based on birthweight, participants were divided into three groups: low (< 2500 g), middle (2500–3500 g), and high (> 3500 g). Furthermore, according to the degree of obesity, they were divided into two groups: obese (20% ≤) and non-obese (>20%). The change of BMI from birth to three years of age (ΔBMI) showed a strong inverse relationship with birthweight and was significantly different among the three birthweight groups (low > middle > high). The ΔBMI and BMI at three years of age were higher in obese than in non-obese children and showed significant positive correlations with the degree of obesity. Early postnatal growth might be determined by birthweight and was higher in obese than in non-obese children. The ΔBMI from birth to three years of age and BMI at age of three years could be predictive factors for pediatric obesity.

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  • Nozomi Matsuda, Atsuko Taki, Atsumi Tsuji, Keisuke Nakajima, Kei Takas ...
    2018 Volume 27 Issue 1 Pages 31-38
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    Factors affecting growth and development in extremely low birth weight infants (ELBWIs) born small for gestational age (SGA) have not been precisely elucidated. We performed a retrospective analysis of ELBWIs born SGA who were treated in the neonatal intensive care unit of Kawaguchi Municipal Medical Centre, Japan. A total 244 ELBWIs were born from 2003 to 2010, and 31 were born with weight and height below the 10th percentile for their gestational age. Among the 31 ELBWIs born SGA, we excluded 9 who died before they reached 3 yr of age or who had severe developmental retardation. A total of 16 patients (weight, 510–998 g; GA, 28w0d–32w5d) who were followed until age 3 yr were eligible for our study. At age 3 yr, 94% and 88% of ELBWIs were above the −2 standard deviation (SD) for height and weight, respectively. A history of mechanical ventilation was associated with height. The average score of the full developmental quotient (DQ) was 85, and 63% (10/16) of ELBWIs scored more than 85. Lower Apgar score (≤ 7) was a risk factor for lower DQ scores in motor development and full development. Our study revealed that most ELBWIs born SGA were more than −2 SD below the mean for height and body weight.

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Case Report
  • Seiichi Tomotaki, Tetsuo Naramura, Junko Hanakawa, Katsuaki Toyoshima, ...
    2018 Volume 27 Issue 1 Pages 39-43
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    Infants with an ileostomy can be at high risk of hypoglycemia because of inadequate nutritional intake; however, there are no reports investigating blood glucose (BG) in infants with ileostomy. We experienced a case of an extremely low birth weight infant who was born at 24 wk of gestation and weighted 623 g. He received an ileostomy because of an intestinal perforation. After the ileostomy, he had recurrent hypoglycemia. Continuous glucose monitoring showed fluctuation of BG levels (postprandial BG elevations and subsequent declines) and non-fasting hypoglycemia, which were undetectable with intermittent fasting BG measurement. The fluctuation of BG levels and non-fasting hypoglycemia improved after closure of the ileostomy. Patients with ileostomy may present with hypoglycemia that is undetectable with intermittent fasting BG measurement. In this case, continuous glucose monitoring was very useful for detecting fluctuation of BG levels and hypoglycemic episodes. Therefore, we recommend that continuous glucose monitoring be performed in infants with an ileostomy to confirm whether they have hypoglycemia or a fluctuation in BG levels. Further studies on the postprandial dynamics of various hormones in infants with ileostomy are required.

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  • Toshihiko Mori, Eri Nishino, Tomomi Jitsukawa, Emiko Hoshino, Satoshi ...
    2018 Volume 27 Issue 1 Pages 45-51
    Published: 2018
    Released on J-STAGE: January 30, 2018
    JOURNAL OPEN ACCESS

    We describe the case of a short-statured 12-yr-old boy who developed a Chiari type 1 malformation associated with central sleep apnea after administration of high-dose GH therapy, which he had been receiving since the age of 10 yr and 4 mo. He responded well to GH therapy, and his height increased by 18.8 cm in 2 yr. At 12 yr and 4 mo of age, his mother reported that he had developed sleep apnea during the previous year and it had worsened over a month prior to presentation at our hospital. Otolaryngological examination did not reveal tonsillar or adenoidal hypertrophy. Polysomnography demonstrated severe central sleep apnea with an apnea-hypopnea index of 46.5/h. Sagittal T1-weighted magnetic resonance imaging (MRI) demonstrated herniation of the cerebellar tonsils 15 mm below the foramen magnum into the cervical spinal cord. Continuous positive airway pressure therapy initiated prior to performing neurosurgery was ineffective. Following uncomplicated foramen magnum decompression, his breathing pattern during sleep returned to normal. Sagittal MRI examination should be considered in patients who develop sleep apnea during/following administration of GH therapy.

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