今日の臨床サポート

Turner症候群(小児科)

著者: 長谷川行洋 東京都立小児総合医療センター 内分泌代謝科

監修: 五十嵐隆 国立成育医療研究センター

著者校正/監修レビュー済:2022/11/09
参考ガイドライン:
  1. International Turner Syndrome Consensus Group:Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting
  1. European Society for Paediatric Endocrinology Turner Syndrome Working Group:Optimal Pubertal Induction in Girls with Turner Syndrome Using Either Oral or Transdermal Estradiol: A Proposed Modern Strategy
  1. Endo-European Reference Network:Pubertal induction and transition to adult sex hormone replacement in patients with congenital pituitary or gonadal reproductive hormone deficiency: an Endo-ERN clinical practice guideline
患者向け説明資料

概要・推奨   

  1. ターナー症候群はもっとも多い染色体異常症のひとつであり、女性2000名に一人程度の頻度である。
  1. 本症では、低身長、思春期遅発がもってもよく見られる症状である。循環器、耳鼻科などの合併症もしられ、全身の診療が求められる疾患単位である。
  1. 小児期に診断される症例では生涯にわたる医療的経過観察を要する。
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薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
長谷川行洋 : 特に申告事項無し[2022年]
監修:五十嵐隆 : 特に申告事項無し[2022年]

改訂のポイント:
  1. 定期レビューを行い、薬剤と参考文献について加筆した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. ターナー(Turner)症候群は、低身長、思春期発達の遅れから診断されることが多いX染色体あるいはその短腕の欠失などを特徴とする染色体異常の1つで、女性2,000人に1人以上の頻度である。
  1. 年齢ごとに疑われる徴候は異なり、新生児・乳児では身長・体重増加不良に加え、手足の浮腫(リンパ管浮腫)、翼状頚、心雑音(大動脈縮窄症に代表される心疾患)がきっかけとなり診断が疑われる。
 
手背の浮腫

左の手背にリンパ管浮腫がみられる

出典

img1:  著者提供
 
 
 
  1. 幼児期、学童期(思春期年齢前)では、低身長がきっかけとなり診断が疑われる。
  1. 思春期年齢では、思春期が発来しない、あるいは進行しないこと(原発性性腺機能低下症の合併)がきっかけとなり診断が疑われる。
  1. 低身長に対しては成長ホルモンの治療が有効であることは確立している。無治療と比べて+5~8cm程度の最終身長改善が期待できる。性腺機能低下症に関しては女性ホルモン補充が行われる。
  1. 生命予後に関する事項として、大動脈拡張、大動脈解離の合併が知られている。10歳代からの合併が知られ、20歳代以上での死亡例が存在する。
問診・診察のポイント  
  1. 年齢ごとに診断のきっかけになる徴候、症状がおよそ分かれるので、そのことを頭に入れて問診、診察を行う。低身長以外にほとんど何も診察での陽性所見がみられない本症患児が存在するため、−2SD以下の低身長女児では一度は染色体検査により本症を除外することを考慮する。
  1. 新生児、乳児早期のリンパ管浮腫、翼状頚はともに他の染色体異常症(ダウン症など)でもみられ得る徴候である。

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文献 

Tsuyoshi Isojima, Susumu Yokoya, Junko Ito, Yasuhiro Naiki, Reiko Horikawa, Toshiaki Tanaka
Proposal of new auxological standards for Japanese girls with turner syndrome.
Clin Pediatr Endocrinol. 2010 Jul;19(3):69-82. doi: 10.1297/cpe.19.69. Epub 2010 Aug 31.
Abstract/Text We recently published new reference growth charts for Japanese girls with Turner syndrome (TS) based on the cross-sectional data of 1,447 subjects beyond the secular trend of growth in Japan. This study was undertaken for their validation and, if necessary, modification before general application. For validation, 24 subjects who had data both at younger (≤5 yr) and older ages (≥13 yr) were used. We analyzed the concordance/discordance of their height standard deviation score (SDS) defined by the charts between the two age periods. For modification, the LMS method was used with 5,772 longitudinal measurements obtained both from the previously analyzed subjects and 118 newly recruited subjects who had been followed up at the National Center for Child Health and Development or Toranomon Hospital. Significant and critical discordance (mean difference, 1.95 SDS; 95% confidence interval (CI), 1.53-2.36; p<0.0001) was detected in height SDS. This prompted us to perform the modifications. A similar analysis using the modified charts revealed no significant discordance (mean difference, 0.27 SDS; 95%CI: -0.17 - 0.71; p=0.22). They seem more adequate for clinical applications for girls with TS born after 1970. New auxological standards for Japanese girls with TS were proposed.

PMID 23926382
Keiko Aso, Shinobu Koto, Asako Higuchi, Daisuke Ariyasu, Masako Izawa, Junko Miyamoto Igaki, Yukihiro Hasegawa
Serum FSH level below 10 mIU/mL at twelve years old is an index of spontaneous and cyclical menstruation in Turner syndrome.
Endocr J. 2010;57(10):909-13. doi: 10.1507/endocrj.k10e-092. Epub 2010 Aug 24.
Abstract/Text The gonadal function of patients with Turner syndrome (TS) is variable. Individuals with mosaicism characterized by 45,X/46,XX or 45,X/47,XXX are more likely to experience spontaneous menarche compared with other karyotypes. Prepubertal gonadotropins of TS patients with spontaneous menarche are reportedly normal or significantly lower than those of patients with induced menarche. The present study investigated an index of spontaneous and cyclical menstruation at 10-12 years old in TS. Subjects comprised 50 patients with TS, divided into three groups: Group A (n=7), with spontaneous menarche before 16 years old and regular menstruation for at least 1 year and 6 months; Group B (n=6), with irregular menstruation since menarche leading to secondary amenorrhea despite spontaneous menarche before 16 years old; and Group C (n=37), without spontaneous breast budding before 14 years old or without spontaneous menarche before 16 years old. Karyotype, LH and FSH concentrations at 10 and 12 years old were analyzed retrospectively. Spontaneous and cyclical menstruation was more frequently observed in TS with mosaicism characterized by 45,X/46,XX or 45,X/47,XXX than in TS with other karyotypes, as previously described. Spontaneous and cyclical menstruation in TS was observed when serum FSH level was <10 mIU/mL at 12 years old, suggesting this FSH level as an index of spontaneous and cyclical menstruation in TS.

PMID 20798475
Claus H Gravholt, Niels H Andersen, Gerard S Conway, Olaf M Dekkers, Mitchell E Geffner, Karen O Klein, Angela E Lin, Nelly Mauras, Charmian A Quigley, Karen Rubin, David E Sandberg, Theo C J Sas, Michael Silberbach, Viveca Söderström-Anttila, Kirstine Stochholm, Janielle A van Alfen-van derVelden, Joachim Woelfle, Philippe F Backeljauw, International Turner Syndrome Consensus Group
Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting.
Eur J Endocrinol. 2017 Sep;177(3):G1-G70. doi: 10.1530/EJE-17-0430.
Abstract/Text Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016. Prior to this meeting, five groups each addressed important areas in TS care: 1) diagnostic and genetic issues, 2) growth and development during childhood and adolescence, 3) congenital and acquired cardiovascular disease, 4) transition and adult care, and 5) other comorbidities and neurocognitive issues. These groups produced proposals for the present guidelines. Additionally, four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society for Endocrinology and the Pediatric Endocrine Society, in collaboration with The European Society for Pediatric Endocrinology, The Endocrine Society, European Society of Human Reproduction and Embryology, The American Heart Association, The Society for Endocrinology, and the European Society of Cardiology. The guideline has been formally endorsed by the European Society for Endocrinology, the Pediatric Endocrine Society, the European Society for Pediatric Endocrinology, the European Society of Human Reproduction and Embryology and the Endocrine Society. Advocacy groups appointed representatives who participated in pre-meeting discussions and in the consensus meeting.

© 2017 European Society of Endocrinology.
PMID 28705803
Jane Marjoribanks, Cindy Farquhar, Helen Roberts, Anne Lethaby, Jasmine Lee
Long-term hormone therapy for perimenopausal and postmenopausal women.
Cochrane Database Syst Rev. 2017 Jan 17;1:CD004143. doi: 10.1002/14651858.CD004143.pub5. Epub 2017 Jan 17.
Abstract/Text BACKGROUND: Hormone therapy (HT) is widely provided for control of menopausal symptoms and has been used for the management and prevention of cardiovascular disease, osteoporosis and dementia in older women. This is an updated version of a Cochrane review first published in 2005. OBJECTIVES: To assess effects of long-term HT (at least 1 year's duration) on mortality, cardiovascular outcomes, cancer, gallbladder disease, fracture and cognition in perimenopausal and postmenopausal women during and after cessation of treatment. SEARCH METHODS: We searched the following databases to September 2016: Cochrane Gynaecology and Fertility Group Trials Register, Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase and PsycINFO. We searched the registers of ongoing trials and reference lists provided in previous studies and systematic reviews. SELECTION CRITERIA: We included randomised double-blinded studies of HT versus placebo, taken for at least 1 year by perimenopausal or postmenopausal women. HT included oestrogens, with or without progestogens, via the oral, transdermal, subcutaneous or intranasal route. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies, assessed risk of bias and extracted data. We calculated risk ratios (RRs) for dichotomous data and mean differences (MDs) for continuous data, along with 95% confidence intervals (CIs). We assessed the quality of the evidence by using GRADE methods. MAIN RESULTS: We included 22 studies involving 43,637 women. We derived nearly 70% of the data from two well-conducted studies (HERS 1998; WHI 1998). Most participants were postmenopausal American women with at least some degree of comorbidity, and mean participant age in most studies was over 60 years. None of the studies focused on perimenopausal women.In relatively healthy postmenopausal women (i.e. generally fit, without overt disease), combined continuous HT increased the risk of a coronary event (after 1 year's use: from 2 per 1000 to between 3 and 7 per 1000), venous thromboembolism (after 1 year's use: from 2 per 1000 to between 4 and 11 per 1000), stroke (after 3 years' use: from 6 per 1000 to between 6 and 12 per 1000), breast cancer (after 5.6 years' use: from 19 per 1000 to between 20 and 30 per 1000), gallbladder disease (after 5.6 years' use: from 27 per 1000 to between 38 and 60 per 1000) and death from lung cancer (after 5.6 years' use plus 2.4 years' additional follow-up: from 5 per 1000 to between 6 and 13 per 1000).Oestrogen-only HT increased the risk of venous thromboembolism (after 1 to 2 years' use: from 2 per 1000 to 2 to 10 per 1000; after 7 years' use: from 16 per 1000 to 16 to 28 per 1000), stroke (after 7 years' use: from 24 per 1000 to between 25 and 40 per 1000) and gallbladder disease (after 7 years' use: from 27 per 1000 to between 38 and 60 per 1000) but reduced the risk of breast cancer (after 7 years' use: from 25 per 1000 to between 15 and 25 per 1000) and clinical fracture (after 7 years' use: from 141 per 1000 to between 92 and 113 per 1000) and did not increase the risk of coronary events at any follow-up time.Women over 65 years of age who were relatively healthy and taking continuous combined HT showed an increase in the incidence of dementia (after 4 years' use: from 9 per 1000 to 11 to 30 per 1000). Among women with cardiovascular disease, use of combined continuous HT significantly increased the risk of venous thromboembolism (at 1 year's use: from 3 per 1000 to between 3 and 29 per 1000). Women taking HT had a significantly decreased incidence of fracture with long-term use.Risk of fracture was the only outcome for which strong evidence showed clinical benefit derived from HT (after 5.6 years' use of combined HT: from 111 per 1000 to between 79 and 96 per 1000; after 7.1 years' use of oestrogen-only HT: from 141 per 1000 to between 92 and 113 per 1000). Researchers found no strong evidence that HT has a clinically meaningful impact on the incidence of colorectal cancer.One trial analysed subgroups of 2839 relatively healthy women 50 to 59 years of age who were taking combined continuous HT and 1637 who were taking oestrogen-only HT versus similar-sized placebo groups. The only significantly increased risk reported was for venous thromboembolism in women taking combined continuous HT: Their absolute risk remained low, at less than 1/500. However, other differences in risk cannot be excluded, as this study was not designed to have the power to detect differences between groups of women within 10 years of menopause.For most studies, risk of bias was low in most domains. The overall quality of evidence for the main comparisons was moderate. The main limitation in the quality of evidence was that only about 30% of women were 50 to 59 years old at baseline, which is the age at which women are most likely to consider HT for vasomotor symptoms. AUTHORS' CONCLUSIONS: Women with intolerable menopausal symptoms may wish to weigh the benefits of symptom relief against the small absolute risk of harm arising from short-term use of low-dose HT, provided they do not have specific contraindications. HT may be unsuitable for some women, including those at increased risk of cardiovascular disease, increased risk of thromboembolic disease (such as those with obesity or a history of venous thrombosis) or increased risk of some types of cancer (such as breast cancer, in women with a uterus). The risk of endometrial cancer among women with a uterus taking oestrogen-only HT is well documented.HT is not indicated for primary or secondary prevention of cardiovascular disease or dementia, nor for prevention of deterioration of cognitive function in postmenopausal women. Although HT is considered effective for the prevention of postmenopausal osteoporosis, it is generally recommended as an option only for women at significant risk for whom non-oestrogen therapies are unsuitable. Data are insufficient for assessment of the risk of long-term HT use in perimenopausal women and in postmenopausal women younger than 50 years of age.

PMID 28093732
Malcolm Donaldson, Berit Kriström, Carina Ankarberg-Lindgren, Siska Verlinde, Janiëlle van Alfen-van der Velden, Aneta Gawlik, Marleen M H J van Gelder, Theo Sas, on behalf of the European Society for Paediatric Endocrinology Turner Syndrome Working Group
Optimal Pubertal Induction in Girls with Turner Syndrome Using Either Oral or Transdermal Estradiol: A Proposed Modern Strategy.
Horm Res Paediatr. 2019;91(3):153-163. doi: 10.1159/000500050. Epub 2019 Jun 5.
Abstract/Text BACKGROUND: Most girls with Turner syndrome (TS) require pubertal induction with estrogen, followed by long term replacement. However, no adequately powered prospective studies comparing transdermal with oral 17β-estradiol administration exist. This reflects the difficulty of securing funding to study a rare condition with relatively low morbidity/mortality when competing against conditions such as cancer and vascular disease. Protocol Consensus: The TS Working Group of the European Society for Paediatric Endocrinology (ESPE) has agreed to both a 3-year oral and a 3-year transdermal regimen for pubertal induction. Prerequisites include suitable 17β-estradiol tablets and matrix patches to allow the delivery of incremental doses based on body weight. Study Proposal: An international prospective cohort study with single centre analysis is proposed in which clinicians and families are invited to choose either of the agreed regimens, usually starting at 11 years. We hypothesise that pubertal induction with transdermal estradiol will result in better outcomes for some key parameters. The primary outcome measure chosen is height gain during the induction period.
ANALYSIS: Assessment of the demographics and drop-out rates of patients choosing either oral or transdermal preparations; and appropriate analysis of outcomes including pubertal height gain, final height, liver enzyme and lipid profile, adherence/acceptability, cardiovascular health, including systolic and diastolic blood pressure and aortic root diameter and bone health.
CONCLUSION: The proposed model of prospective data collection according to internationally agreed protocols aims to break the current impasse in obtaining evidence-based management for TS and could be applied to other rare paediatric endocrine conditions.

© 2019 S. Karger AG, Basel.
PMID 31167218
Jens Bollerslev, Lars Rejnmark, Alexandra Zahn, Ansgar Heck, N M Appelman-Dijkstra, Luis Cardoso, Fadil M Hannan, Filomena Cetani, Tanja Sikjær, Anna Maria Formenti, Sigridur Björnsdottir, Camilla Schalin-Jantti, Zhanna Belaya, Fraser Wilson Gibb, Bruno Lapauw, Karin Amrein, Corinna Wicke, Corinna Grasemann, Michael Krebs, Eeva M Ryhänen, Ozer Makay, Salvatore Minisola, Sebastien Gaujoux, Jean-Philippe Bertocchio, Zaki K Hassan-Smith, Agnès Linglart, Elizabeth M Winter, Martina Kollmann, Hans-Georg Zmierczak, Elena Tsourdi, Stefan Pilz, Heide Siggelkow, Neil J Gittoes, Claudio Marcocci, Peter Kamenicky, 2021 PARAT Working Group
European Expert Consensus on Practical Management of Specific Aspects of Parathyroid Disorders in Adults and in Pregnancy: Recommendations of the ESE Educational Program of Parathyroid Disorders.
Eur J Endocrinol. 2022 Feb 1;186(2):R33-R63. doi: 10.1530/EJE-21-1044. Epub 2022 Jan 13.
Abstract/Text This European expert consensus statement provides recommendations for the diagnosis and management of primary hyperparathyroidism (PHPT), chronic hypoparathyroidism in adults (HypoPT), and parathyroid disorders in relation to pregnancy and lactation. Specified areas of interest and unmet needs identified by experts at the second ESE Educational Program of Parathyroid Disorders (PARAT) in 2019, were discussed during two virtual workshops in 2021, and subsequently developed by working groups with interest in the specified areas. PHPT is a common endocrine disease. However, its differential diagnosing to familial hypocalciuric hypercalcemia (FHH), the definition and clinical course of normocalcemic PHPT, and the optimal management of its recurrence after surgery represent areas of uncertainty requiring clarifications. HypoPT is an orphan disease characterized by low calcium concentrations due to insufficient PTH secretion, most often secondary to neck surgery. Prevention and prediction of surgical injury to the parathyroid glands are essential to limit the disease-related burden. Long-term treatment modalities including the place for PTH replacement therapy and the optimal biochemical monitoring and imaging surveillance for complications to treatment in chronic HypoPT, need to be refined. The physiological changes in calcium metabolism occurring during pregnancy and lactation modify the clinical presentation and management of parathyroid disorders in these periods of life. Modern interdisciplinary approaches to PHPT and HypoPT in pregnant and lactating women and their newborns children are proposed. The recommendations on clinical management presented here will serve as background for further educational material aimed for a broader clinical audience, and were developed with focus on endocrinologists in training.

PMID 34863037
Yukihiro Hasegawa, Tomoyo Itonaga, Kento Ikegawa, Satsuki Nishigaki, Masanobu Kawai, Eri Koga, Hideya Sakakibara, Judith L Ross
Ultra-low-dose estrogen therapy for female hypogonadism.
Clin Pediatr Endocrinol. 2020;29(2):49-53. doi: 10.1297/cpe.29.49. Epub 2020 Apr 16.
Abstract/Text In females, endogenous estrogen secretion increases gradually before pubertal development. The benefits of low-dose estrogen therapy in patients with Turner syndrome were originally discussed by Ross et al. and Quigley et al. These seminal studies used ethinyl estradiol (EE2), starting at a dose of 25 ng/kg/d. We hypothesized that the initial dosage of estrogen could be titrated to more closely mimic physiological increments of endogenous estrogen. Therefore, our recent study initiated EE2 treatment at a dosage of 1-2 ng/kg/d, an ultra-low-dose estrogen therapy in pediatric patients with Turner syndrome. The ultra-low-dose estrogen therapy in this syndrome produced a good final height outcome but achieved suboptimal bone mineral density (BMD). In the present review, we have explained our findings to clarify the merits and demerits of this new therapy and to promote further discussion and research. This type of ultra-low-dose estrogen therapy, initiated at an early age, could be ideal for estrogen replacement in female patients with hypogonadism, such as Turner syndrome.

2020©The Japanese Society for Pediatric Endocrinology.
PMID 32313372
David K Stephure, Canadian Growth Hormone Advisory Committee
Impact of growth hormone supplementation on adult height in turner syndrome: results of the Canadian randomized controlled trial.
J Clin Endocrinol Metab. 2005 Jun;90(6):3360-6. doi: 10.1210/jc.2004-2187. Epub 2005 Mar 22.
Abstract/Text BACKGROUND: A randomized, controlled trial of GH supplementation to adult height in girls with short stature due to Turner syndrome was conducted in Canada. We report results in subjects who completed the protocol and subjects who participated in follow-up.
METHODS: One hundred fifty-four girls with Turner syndrome, aged 7-13 yr, were randomly assigned to one of two groups: 1) GH by sc injection six times per week (0.30 mg/kg.wk), and 2) control (C), no GH treatment. Both cohorts received standardized sex steroid replacement starting at a chronological age of 13 yr. Subjects were followed until protocol completion, defined as height velocity less than 2 cm/yr and bone age 14 yr or greater. A subsequent protocol addendum requested follow-up safety and efficacy assessment in all patients at least 1 yr after the last core protocol visit.
RESULTS: One hundred four patients completed the study (61 GH, 43 C), and 50 withdrew (15 GH, 35 C). At protocol completion, mean heights were 147.5 +/- 6.1 (GH) and 141.0 +/- 5.4 cm (C), respectively (P < 0.001). Of those who completed the protocol, 59 (40 GH, 19 C) had height data at least 1 yr after protocol completion; in that group, mean heights were 149.0 +/- 6.4 (GH) and 142.2 +/- 6.6 cm (C), respectively (P < 0.001). At protocol completion and follow-up, the mean height gain due to GH, estimated by analysis of covariance, was +7.2 cm (confidence interval 6.0, 8.4) and +7.3 cm (confidence interval 5.4, 9.2), respectively (both P < 0.001).
CONCLUSIONS: This is the first evidence from a randomized, controlled trial to adult height that GH supplementation with induction of puberty at a near physiological age increases the adult height of girls with Turner syndrome.

PMID 15784709

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