著者

Ryohei Kobayashi Mari Hasegawa Chiharu Kawaguchi Naoko Ishikawa Kiyotaka Tomiwa Midori Shima Keiji Nogami

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.30, no.1, pp.19-26, 2021 (Released:2021-01-05)

参考文献数

27

被引用文献数

9

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Selenium, one of the essential trace minerals, is present in vivo in form of selenoproteins. Iodothyronine deiodinase, a selenoprotein, is involved in the activation and inactivation of thyroid hormone. Therefore, patients with selenium deficiency may present changes in thyroid hormone levels due to inhibition of T4 to T3 conversion; however, this assumption is still under debate. In the present study, we retrospectively investigated the thyroid function in 22 patients with selenium deficiency. Thyroid stimulating hormone (TSH) and free T4 (FT4) levels were increased in 3 (14%) and 5 (23%) patients, respectively, and free T3 (FT3) levels were decreased in 6 (27%) patients. The FT4/FT3 ratio was significantly higher in patients with selenium deficiency than that in the control group. There appeared to be a positive correlation between the decreased rate of selenium levels and FT4/FT3 ratio, thereby indicating that patients with severe selenium deficiency also exhibited abnormal thyroid hormone levels. Furthermore, when selenium was supplemented in seven patients with abnormal thyroid hormone levels, the TSH, FT4, and FT4/FT3 ratio were significantly decreased and FT3 levels were increased. Collectively, patients with selenium deficiency could present the characteristics of not only low FT3 but also high FT4 and FT4/FT3 ratio.

著者

Tsutomu Ogata Toshiaki Tanaka Masayo Kagami

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.16, no.4, pp.85-87, 2007 (Released:2007-11-17)

参考文献数

6

被引用文献数

7 16

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In 1990, we proposed the equations to calculate target height (TH) and target range (TR) for Japanese, taking account of the positive height secular trend observed over the last ~100 years. However, height difference between generations appears to have become small or negligible in contemporary Japanese populations. Thus, we re-analyzed the Japanese height data, and revised the equations for TH and TR for contemporary Japanese children as follows (cm): Boys, TH = {PH + (MH + 13)} 2, TR = TH ± 9; and Girls, TH = {(PH - 13) + MH} 2; TR = TH ± 8, where PH indicates paternal height and MH maternal height.

著者

Keisuke Nagasaki Akie Nakamura Takeru Yamauchi Hotaka Kamasaki Yosuke Hara Junko Kanno Satomi Koyama Yoshiaki Ohtsu Ikuko Takahashi Shigeru Suzuki Kenichi Kashimada Toshihiro Tajima

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.30, no.2, pp.79-84, 2021 (Released:2021-04-03)

参考文献数

16

被引用文献数

2

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Atrophic autoimmune thyroiditis (AAT) is a type of autoimmune hypothyroidism without goiter. TSH receptor-blocking antibodies (TSBAb) are involved in its etiology in adults. Reportedly, this disease is extremely rare in children. In this study, we aimed to investigate the prevalence of TSBAb during AAT onset in children using a commercially available cell-based bioassay TSAb kit. We conducted a multicenter retrospective observational study. We collected data of patients with AAT who were < 15 yr old, enrolled in a collaborative research group, and diagnosed since July 2003. AAT was defined as acquired autoimmune hypothyroidism without thyroid enlargement. Eighteen patients (including 15 females) whose TSH receptor antibody (TRAb) or TSBAb levels were measured within a year from the initial visit were included. The median age at diagnosis was 9.3 years, and the estimated time between onset and diagnosis was 2.6 yr. The positive rate for either TSBAb or TRAb was 38.8% (95% confidence interval: 18.3–59.5%). There were no significant differences in age, the estimated time between onset and diagnosis, and FT4 levels at diagnosis between the TSBAb-positive and -negative groups. Unlike previous reports, we showed that the prevalence of TSBAb-positivity in childhood-onset AATs is not rare, as in adults.

著者

Rohi Shima Kentaro Sawano Nao Shibata Hiromi Nyuzuki Sunao Sasaki Hidetoshi Sato Yohei Ogawa Yuki Abe Keisuke Nagasaki Akihiko Saitoh

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.29, no.3, pp.105-110, 2020 (Released:2020-07-11)

参考文献数

13

被引用文献数

2

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Newborn screening (NBS) can detect 21-hydroxylase deficiency (21-OHD), allowing for early treatment initiation. However, many patients present with adrenal crises or hyponatremia at their first visit. Age (in days) of hyponatremia development in infants with salt-wasting (SW)-type 21-OHD remains unclear. Therefore, we determined the earliest age of hyponatremia diagnosis in this retrospective observational study using medical records of 40 patients with classic 21-OHD in Niigata Prefecture, Japan, from April 1989 to March 2019. We determined the earliest diagnosis of hyponatremia (serum sodium levels < 130 mEq/L) and created a sodium decrease rate model to estimate hyponatremia development age. Of 23 patients with SW-type 21-OHD, 10 (43.5%) were identified during NBS; the earliest case to present with hyponatremia was at day 7. Serum sodium levels were significantly and negatively correlated with age in days, and hyponatremia was estimated to develop at 6.6 d after birth. Genotype or serum 17-hydroxyprogesterone levels were not associated with sodium decrease rate. Thus, hyponatremia development age is earlier (within 7 d) than the previously described time-point (10–14 d) in infants with SW-type 21-OHD. Efforts to reduce the time lag from obtaining results to consultation may be required in patients with high 17-hydroxyprogesterone levels on NBS.

著者

Tomohiro Saito Katsuhiko Tachibana Akira Shimatsu Noriyuki Katsumata Naomi Hizuka Kenji Fujieda Susumu Yokoya Toshiaki Tanaka

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.15, no.3, pp.79-84, 2006 (Released:2006-08-02)

参考文献数

8

被引用文献数

1 3

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Accurate and reliable determination of blood growth hormone level is essential in the diagnosis and treatment of short stature children. However, measured levels differed considerably among measurement kits available in Japan until 2003. Therefore, standardization of the measured values was attempted by measuring growth hormone levels in a sample of healthy adult individuals every year using the different kits. A standardization equation was developed for each kit through linear structural relationship with the mean values of the used kits and measured values in each kit as random variables. A Pearson's correlation coefficient between the mean values of all kits and the measured values from each kit was also obtained. Sources for the marked discrepancies amongst the measured values in the different kits were also explored. The obtained values for slopes and intercepts in the equations varied considerably, but the standard values obtained from these equations after the measured values for each kit were transformed into standard values served well as the standard. The standard solutions in the respective measurement kits were found to be the source of variability in the measured values among the kits.

著者

Toshimi Michigami Yasuhisa Ohata Makoto Fujiwara Hiroshi Mochizuki Masanori Adachi Taichi Kitaoka Takuo Kubota Hideaki Sawai Noriyuki Namba Kosei Hasegawa Ikuma Fujiwara Keiichi Ozono

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.29, no.1, pp.9-24, 2020 (Released:2020-01-09)

参考文献数

48

被引用文献数

28

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Hypophosphatasia (HPP) is a rare bone disease caused by inactivating mutations in the ALPL gene, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have varied clinical manifestations and are classified based on the age of onset and severity. Recently, enzyme replacement therapy using bone-targeted recombinant alkaline phosphatase (ALP) has been developed, leading to improvement in the prognosis of patients with life-threatening HPP. Considering these recent advances, clinical practice guidelines have been generated to provide physicians with guides for standard medical care for HPP and to support their clinical decisions. A task force was convened for this purpose, and twenty-one clinical questions (CQs) were formulated, addressing the issues of clinical manifestations and diagnosis (7 CQs) and those of management and treatment (14 CQs). A systematic literature search was conducted using PubMed/MEDLINE, and evidence-based recommendations were developed. The guidelines have been modified according to the evaluations and suggestions from the Clinical Guideline Committee of The Japanese Society for Pediatric Endocrinology (JSPE) and public comments obtained from the members of the JSPE and a Japanese HPP patient group, and then approved by the Board of Councils of the JSPE. We anticipate that the guidelines will be revised regularly and updated.

著者

Eiji Kawasaki

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.23, no.4, pp.99-105, 2014 (Released:2014-11-06)

参考文献数

19

被引用文献数

26 95

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Type 1 diabetes (T1D) is an organ-specific autoimmune disease caused by the autoimmune response against pancreatic β cells. T1D is often complicated with other autoimmune diseases, and anti-islet autoantibodies precede the clinical onset of disease. The most common coexisting organ-specific autoimmune disease in patients with T1D is autoimmune thyroid disease, and its frequency is estimated at > 90% among patients with T1D and autoimmune diseases. The prevalence of anti-thyroid antibodies in children with T1D at disease onset is about 20% and is particularly common in girls. Furthermore, patients with anti-thyroid antibodies are 18 times more likely to develop thyroid disease than patients without anti-thyroid antibodies. Therefore, for early detection of autoimmune thyroid disease in children with T1D, measurement of anti-thyroid antibodies and TSH at T1D onset and in yearly intervals after the age of 12 yr is recommended. Anti-islet autoantibodies are predictive and diagnostic markers for T1D. The most frequently detected autoantibodies in Japanese patients are GAD autoantibodies (~80%) followed by IA-2 autoantibodies (~60%), insulin autoantibodies (~55%) and ZnT8 autoantibodies (~50%). In a combined analysis, 94% of Japanese patients with T1D can be defined as having type 1A diabetes. Furthermore, autoantibodies to ZnT8 and IA-2 are associated with childhood-onset and acute-onset patients. Thus, it is important to develop a diagnostic strategy for patients with type 1A diabetes in consideration of the age or mode of disease onset.

著者

Aman B Pulungan Carine de Beaufort Amajida F Ratnasari Helena A Puteri Laura Lewis-Watts Zulfiqar A Bhutta

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.32, no.3, pp.137-146, 2023 (Released:2023-06-24)

参考文献数

35

被引用文献数

1

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A decade since the discovery of insulin, the increasing prevalence of type 1 diabetes mellitus (T1DM) has underscored the prevailing inequalities in the provision of essential care for T1DM worldwide. However, the details on the availability of insulin types and associated medical devices remain unclear. A cross-sectional electronic survey was distributed across a global network of pediatric societies under the umbrella of the International Pediatric Association (IPA). Access to and availability of pediatric diabetes care were investigated using standardized questions. Responses from 25 of 132 pediatric societies across six regions were included. Pediatric endocrinologists typically manage T1DM together with pediatricians or adult endocrinologists. Nonetheless, 24% of the respondents reported pediatricians to be the sole healthcare professionals. According to the respondents, the patients were either partially or completely responsible for payments of insulin (40%), A1C (24%), C-peptide (28%), and antibody testing for diagnosis (28%). Government support is generally available for insulin, but this was merely 20% for insulin pumps and 12% for continuous glucose monitors. There are considerable disparities in the access, availability, and affordability of diabetes testing, medications, and support between countries with significant out-of-pocket payments for care. Country- and region-specific improvements to national programs are necessary to achieve optimal pediatric diabetes care globally.

著者

Aysun Ata Arzu Jalilova Tarık Kırkgöz Hafize Işıklar Günay Demir Yasemin Atik Altınok Behzat Özkan Ayşin Zeytinlioğlu Şükran Darcan Samim Özen Damla Gökşen

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.31, no.1, pp.33-37, 2022 (Released:2022-01-01)

参考文献数

21

被引用文献数

17

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The novel coronavirus disease (COVID-19) has emerged as a global pandemic. This was a prospective, case-control study conducted in Izmir, Turkey. The aim of this study was to assess the relationship between COVID-19 and new-onset T1DM. We included pediatric patients (aged 6 mo–18 yr) with new-onset type-1 diabetes mellitus (T1DM) diagnosed during the COVID-19 pandemic, between April 2020 and January 2021. Polymerase chain reaction was used to diagnose COVID-19 after hospital admission. An enzyme-linked immunoassay for IgM and IgG against SARS-CoV-2 was performed after the diagnosis was confirmed. In the control group, the blood antibody test was conducted as close as possible to the time of the T1DM patient referral. A total of 118 participants were included in the study, comprising 57 (48%) patients with new-onset T1DM and 61 (52%) healthy controls. Of the 57 patients, 36 (63.2%) presented with DKA, 17 (29.7%) with diabetic ketosis, and four (7%) incidentally. The SARS-CoV-2 antibody test was positive in five (8.7%) patients with T1DM and six (10%) controls. The rate of positivity did not differ between the two groups (p = 0.901). It was not possible to demonstrate a clear association between SARS-CoV-2 infection and new-onset T1DM. Whether SARS-CoV-2 increases susceptibility to diabetes by triggering islet cell autoimmunity and affects the timing of overt diabetes in patients with existing autoimmunity should be studied in large cohorts.

著者

I Wayan Eka Satriawibawa I Made Arimbawa Ketut Ariawati Ida Bagus Gede Suparyatha I Gusti Ngurah Sanjaya Putra I Nyoman Budi Hartawan

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.31, no.4, pp.242-249, 2022 (Released:2022-10-01)

参考文献数

34

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Although mainly affected by the blood glucose levels, the level of HbA1c could be influenced by other important factors, such as an iron deficiency, which is commonly found in children with type 1 diabetes mellitus (T1DM). However, a clinical judgment could not be established, as previous studies still reported conflicting results and lack of data regarding Indonesia. We aimed to evaluate the correlation between the serum iron and HbA1c levels in children with T1DM. This single-center cross-sectional study was conducted from February to October 2020 at Sanglah Hospital, Bali, Indonesia. Patients aged 1–18 yr were included in this study. The HbA1c and serum iron levels were evaluated in the blood samples. Spearman and partial correlation analyses were used to analyze the correlations between variables. The statistical significance was set at P < 0.05. Thirty-three subjects were analyzed, with a mean age of 11.24 ± 3.76 yr. Low serum iron and poor glycemic index were found in 54.5% and 69.7% of the subjects, respectively. Spearman correlation analysis revealed a low negative correlation between the serum iron and HbA1c levels (Spearman’s rho = –0.376, P = 0.031). A partial correlation showed a moderate negative correlation (r = –0.473, P = 0.013) after adjusting for confounding variables. This study found a moderate negative correlation between the serum iron and HbA1c level in children and adolescents with T1DM.

著者

Tsuyoshi Isojima Noriko Kato Yoshiya Ito Susumu Kanzaki Mitsunori Murata

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.25, no.2, pp.71-76, 2016 (Released:2016-04-28)

参考文献数

8

被引用文献数

45 98

著者

Keiichi Ozono Toshimi Michigami Noriyuki Namba Shigeo Nakajima Takehisa Yamamoto

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.15, no.4, pp.129-135, 2006 (Released:2006-11-03)

参考文献数

45

被引用文献数

3 3

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Serum phosphate levels are regulated in both calcium-dependent and -independent fashions. Active vitamin D increases while PTH decreases serum phosphate levels in association with the elevation of serum calcium. On the other hand, a calcium-independent phosphaturic factor, historically called phosphatonin is believed to exert a physiological function based on findings in hereditary and tumor-induced diseases characterized by hypophosphatemia with normocalcemia. Among them, autosomal dominant hypophosphatemic rickets (ADHR) has contributed greatly to its elucidation because the gene responsible for ADHR encodes fibroblast growth factor 23 (FGF23) that has been found to have a phosphaturic effect. In addition, FGF23 has been proved to be involved in most cases of oncogenic osteomalacia and X-linked hypophosphatemic rickets that are also characterized by hypophosphatemia and normocalcemia. Moreover, familial tumoral calcinosis, which represents the metabolic mirror image of hypophosphatemic conditions, is caused by a loss-of-function mutation in the FGF23 gene in some patients. Very recently, hereditary hypophosphatemic rickets with hypercalciuria has been found to be caused by mutations in the SLC34A1 gene which encodes a type of sodium phosphate cotransporter. These findings may provide new strategies for treating patients with abnormal phosphate metabolism.

著者

Kenji Ohyama Tamiko Watanabe Aiko Muramatsu

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.12, no.2, pp.105-107, 2003 (Released:2004-01-10)

参考文献数

6

被引用文献数

1

著者

Takuo Kubota Masanori Adachi Taichi Kitaoka Kosei Hasegawa Yasuhisa Ohata Makoto Fujiwara Toshimi Michigami Hiroshi Mochizuki Keiichi Ozono

出版者

The Japanese Society for Pediatric Endocrinology

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.29, no.1, pp.25-42, 2020 (Released:2020-01-09)

参考文献数

79

被引用文献数

19

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Achondroplasia (ACH) is a skeletal dysplasia that presents with limb shortening, short stature, and characteristic facial configuration. ACH is caused by mutations of the FGFR3 gene, leading to constantly activated FGFR3 and activation of its downstream intracellular signaling pathway. This results in the suppression of chondrocyte differentiation and proliferation, which in turn impairs endochondral ossification and causes short-limb short stature. ACH also causes characteristic clinical symptoms, including foramen magnum narrowing, ventricular enlargement, sleep apnea, upper airway stenosis, otitis media, a narrow thorax, spinal canal stenosis, spinal kyphosis, and deformities of the lower extremities. Although outside Japan, papers on health supervision are available, they are based on reports and questionnaire survey results. Considering the scarcity of high levels of evidence and clinical guidelines for patients with ACH, clinical practical guidelines have been developed to assist both healthcare professionals and patients in making appropriate decisions in specific clinical situations. Eleven clinical questions were established and a systematic literature search was conducted using PubMed/MEDLINE. Evidence-based recommendations were developed, and the guidelines describe the recommendations related to the clinical management of ACH. We anticipate that these clinical practice guidelines for ACH will be useful for healthcare professionals and patients alike.