著者

Masanori Minagawa

出版者

日本小児内分泌学会

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.14, no.Supplement23, pp.S23_9-S23_16, 2005 (Released:2005-08-27)

参考文献数

5

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Pseudohypoparathyroidism (PHP) is composed of PHP-Ia and PHP-Ib. The former is characterized by multiple hormone resistance including parathyroid hormone associated with Albright hereditary osteodystrophy (AHO); but in the latter, AHO is not complicated. In the natural course of PHP-Ia, the time of onset, the degree of hypocalcemia and associated symptoms vary among patients. Since the symptoms of AHO, including brachydactyly, are often ambiguous, the criteria for clinical differential diagnosis between these two types are required. In our 10 cases of sporadic PHP-Ib, no patient had subcutaneous calcification. Currently, however, genetic analysis on the DNA methylation is necessary for the definite diagnosis. All of the 10 cases of sporadic PHP-Ib had complete methylation in NESP55 as well as complete demethylation in the AS and 1A regions of the GNAS gene, while the region of the abnormal methylation in XL were varied among the patients. There was no clear correlation among skewed X-inactivation, abnormal DNA methylation pattern, clinical phenotypes and other complications.

著者

Chatelain Pierre G. Claris Olivier Nicolino Marc

出版者

日本小児内分泌学会

雑誌

Clinical Pediatric Endocrinology

巻号頁・発行日

vol.3, no.5, pp.27-34, 1994

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Intra-uterine growth retardation (IUGR) and Silver-Russell syndrome (SRS) are born short for gestational age and many of them do not show post-natal catch-up growth Although post-natal catch-up growth frequently occurs, significant number of both IUGR and SRS newborns often fail to catch up. This may result in very short adult height (more than 2 standard deviations (SD) below the mean) in up to 20% of these patients, in association with poor school and social performance.<BR>In order to try to induce catch-up growth and hopefully increase final height, attempts have been made in clinical trial to treat these growth hormone (GH) sufficient patients with human GH.<BR>Using the Kabi Pharmacia International Growth Study patient data base, a group of 106 IUGR (83 males and 23 females) and 45 SRS with persistent post-natal growth failure were studied. IUGR clinical characteristics included a birth length and weight more than 2 SD below the mean for gestational age. Height deficit at start of the GH treatment was -3.0 standard deviation scores (SDS) for chronological age (CA), at a median CA of 8.7 years with a median bone age (BA) of 7.0 years. Mean parental heights were 166 cm for fathers and 153 cm for mothers. Median dose of recombinant human GH (rhGH) in 106 IUGR patients was 0.5 IU/kg/week, given at a median frequency of 5 injections per week. The median height SDS for CA at the start and at 1, 2 and 3 years of hGH treatment were -3.0, -2.5, -2.1 and -1.9 respectively.<BR>45 patients presented with SRS. Their median CA and BA at start of treatment were 6.7 and 3.2 years respectively. Mean parental heights were 167.5 cm for fathers and 160 for mothers. Median dose of rhGH in these 45 SRS was 0.7 IU/kg/week, with a median frequency of 6 injections per week. Median height SDS of SRS patients at start, 1, 2 and 3 years of hGH treatment were -3.5, -2.9, -2.8 and -2.2 respectively. One difference between these IUGR and SRS groups is that SRS patients tended to be born of normal height parents, in contrast to IUGR with persistent post-natal growth failure. In these two groups, exogenous GH treatment tended to induce catch up growth, although long term follow up is sufficient to allow conclusions to be draw regarding these patients' final adult height.

著者

Ogata Tsutomu Tanaka Toshiaki Kagami Masayo

出版者

日本小児内分泌学会

雑誌

Clinical pediatric endocrinology (ISSN:09185739)

巻号頁・発行日

vol.16, no.4, pp.85-87, 2007-10

被引用文献数

10 16 4

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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

Megumi Miyakawa

出版者

日本小児内分泌学会

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.23, no.3, pp.73-82, 2014 (Released:2014-08-06)

参考文献数

18

被引用文献数

2 8

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It has been more than three years since the unprecedentedly massive earthquake and tsunami struck eastern Japan on March 11, 2011, and the large accident occurred at the Fukushima Daiichi Nuclear Power Plant. To investigate the influence of radiation exposure, thyroid ultrasonography has been provided preliminarily for 360,000 children who lived in Fukushima Prefecture at the time of the accident. As of September 2013, 59 children had been diagnosed with thyroid cancer by fine-needle aspiration cytology, and 34 children had been treated surgically and ultimately diagnosed with papillary thyroid cancer. Here, I would like to describe the characteristics of pediatric thyroid cancer and typical thyroid images obtained by ultrasonography.

著者

Tatsuhiko Urakami Shigeo Morimoto Yoshikazu Nitadori Kensuke Harada Misao Owada Teruo Kitagawa

出版者

日本小児内分泌学会

雑誌

Clinical Pediatric Endocrinology (ISSN:09185739)

巻号頁・発行日

vol.16, no.2, pp.53-58, 2007 (Released:2007-05-17)

参考文献数

18

被引用文献数

2 2

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This study evaluated recent changes in the annual incidence of childhood type 2 diabetes in the Tokyo metropolitan area. From 1974 to 2004, a total of 236 students were diagnosed as having type 2 diabetes by the urine glucose screening program at school. The overall incidence of type 2 diabetes was 2.55/100,000. Overall, 83.9% of students with diabetes were obese; junior high school students had a significantly higher incidence than primary school students (0.75 vs. 6.27/100,000). The annual incidences over the 5-yr periods from 1974-2004 were 1.73, 3.23, 3.05, 2.90, 2.70 and 1.41/100,000, respectively. The incidences in 1974-1980 and 2001-2004 were significantly lower than those in 1981-1985, 1986-1990 and 1991-1995, because primary school students in 1974-1980 had a significantly lower incidence (0.27/100,000), and junior high school students in 2001-2004 had a somewhat lower incidence (3.66/100,000) than during 1981-2000. In recent years, Japanese children's lifestyle and eating habits have gradually improved, and this may have contributed to the trend toward decrease in the incidence of type 2 diabetes in 2001-2004 in the Tokyo metropolitan area.