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Transitions in Care from Pediatric to Adult Health Care Providers: Ongoing Challenges and Opportunities for Young Persons with Diabetes. 从儿科到成人医疗保健提供者的护理转变:对年轻糖尿病患者的持续挑战和机遇。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487866
Katharine Garvey, Lori Laffel

Adolescence and young adulthood are times of multiple developmental changes, including physiological, social, emotional, cognitive, and behavioral transformations. The adolescent or young adult living with type 1 or type 2 diabetes must navigate the vicissitudes of these developmental stages while managing the rigors and self-care demands of these conditions. Diabetes in children is managed by adults, mainly by parents. As the child matures, diabetes management tasks transition from parents to the developing teen. This transition in care is a process that generally begins in early adolescence and culminates when the older teen successfully accepts and manages diabetes self-care tasks. Along with the transitions in diabetes management tasks, older teens and young adults must be prepared for transfer from the pediatric diabetes care team to an adult-focused health care team. Numerous publications have described the challenges associated with both the process of transition and the act of transfer. Lack of preparation during transition followed by unsuccessful transfer often results in gaps in diabetes care exceeding 6 months, deterioration in glycemic control, increase in emergency room use and hospitalization, and emergence of diabetes complications among older teens and young adults. There is need for ongoing research internationally to address these deficiencies in order to improve the short- and long-term health of young persons with diabetes.

青春期和青年期是多种发育变化的时期,包括生理、社会、情感、认知和行为转变。患有1型或2型糖尿病的青少年或年轻人必须在处理这些条件的严峻性和自我保健需求的同时,驾驭这些发展阶段的变迁。儿童糖尿病由成年人管理,主要由父母管理。随着孩子的成熟,糖尿病管理的任务从父母转移到发展中的青少年。这种护理的过渡过程通常从青春期早期开始,当年龄较大的青少年成功地接受并管理糖尿病自我护理任务时达到高潮。随着糖尿病管理任务的转变,年龄较大的青少年和年轻人必须准备好从儿科糖尿病护理小组转移到以成人为中心的卫生保健小组。许多出版物描述了与过渡过程和转移行为有关的挑战。在转诊期间缺乏准备,随后转诊不成功,往往导致糖尿病护理间隔超过6个月,血糖控制恶化,急诊室使用率和住院率增加,并在年龄较大的青少年和年轻人中出现糖尿病并发症。国际上需要进行持续的研究,以解决这些不足,以改善青年糖尿病患者的短期和长期健康。
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引用次数: 10
Transition of Care from Childhood to Adulthood: Turner Syndrome. 从童年到成年的照顾过渡:特纳综合症。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487524
Gerard Conway

Girls with Turner syndrome (TS) require special consideration during transition from childhood to adult care. During the transition years, treatment for short stature will be completed and sexual development induced in parallel with the peer group. The timing of sexual development may have later repercussions with respect to psychosocial development and partnership status. Late presentation of TS, which is so common, can result in additional difficulties with the transition process.

患有特纳综合征(TS)的女孩在从童年到成人护理的过渡期间需要特别考虑。在过渡期间,将完成对身材矮小的治疗,并与同龄群体平行诱导性发育。性发育的时间可能会对以后的社会心理发展和伙伴关系状况产生影响。很常见的TS延迟呈现可能会导致过渡过程中的额外困难。
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引用次数: 2
Transition of Care from Childhood to Adulthood: Congenital Hypogonadotropic Hypogonadism. 从儿童期到成年期护理的过渡:先天性促性腺功能减退症。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487527
Andrew A Dwyer, Nelly Pitteloud

Passage from childhood to adult life involves biological changes culminating in full reproductive capacity as well as psychosocial development. For patients with congenital hypogonadotropic hypogonadism (CHH), this can be an emotionally challenging time as their pubertal failure results in striking physical differences from their peers. CHH is difficult to differentiate from common disorders of puberty such as constitutional delay of growth and puberty. As such, delays in diagnosis are frequent, and it is a common source of stress and frustration for these adolescents. While effective treatments are available for inducing puberty and attaining fertility is possible in most cases, patients may find it difficult to cope with living with CHH. A critical issue for adolescents with CHH is the risk for being lost to follow-up during the transition from pediatric-centered care to adult care. This article will review the state of the art in diagnosis and treatment of patients with CHH with a particular focus on supporting an effective transition from pediatric-centered care to adult-oriented endocrine services. A synthesis of best practices is offered to help guide clinicians in supporting patients and families during this challenging period of care.

从童年到成年的过程涉及生理变化,最终达到充分生殖能力和社会心理发展。对于先天性促性腺功能减退症(CHH)患者来说,这可能是一个情感上具有挑战性的时期,因为他们的青春期失败导致他们与同龄人的身体差异显著。CHH很难与常见的青春期疾病区分,如体质性生长迟缓和青春期。因此,延误诊断是经常发生的,这是这些青少年压力和沮丧的常见来源。虽然在大多数情况下,有效的治疗方法可以诱导青春期和获得生育能力,但患者可能会发现很难应对CHH的生活。青少年CHH的一个关键问题是在从以儿科为中心的护理过渡到成人护理期间失去随访的风险。本文将回顾CHH患者的诊断和治疗的最新进展,特别侧重于支持从以儿科为中心的护理到以成人为导向的内分泌服务的有效过渡。提供最佳实践的综合,以帮助指导临床医生在这一具有挑战性的护理期间支持患者和家属。
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引用次数: 10
"Child-Adult" Transition - Adolescence: When Illness Appears…. “儿童-成人”过渡-青春期:当疾病出现....
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487522
Sabine Malivoir, Karinne Gueniche

Considering the patient's transition from child to adolescent to adult and its psychological aspects in endocrinology and diabetology, it is necessary to recall the child's psycho-affective development when he enters adolescence. Indeed, adolescence appears paradigmatic of the "child-adult" transition; it is a specific and decisive psychic process which allows the child to become an adult, that is to say autonomous and subject to his desire. In our paper, we study the resonance of a chronic disease in young people; type 1 diabetes is chosen.

考虑到患者从儿童到青少年再到成人的转变及其在内分泌学和糖尿病学上的心理方面,有必要回顾儿童进入青春期时的心理情感发展。事实上,青春期似乎是“儿童-成人”过渡的典范;这是一个特定的和决定性的心理过程,它使孩子成为一个成年人,也就是说,自主和服从他的欲望。在我们的论文中,我们研究了年轻人慢性疾病的共振;选择1型糖尿病。
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引用次数: 1
Transition in Pediatric and Adolescent Hypogonadal Girls: Gynecological Aspects, Estrogen Replacement Therapy, and Contraception. 儿童和青春期性腺功能低下女孩的转变:妇科方面,雌激素替代疗法和避孕。
Pub Date : 2018-01-01 Epub Date: 2018-06-12 DOI: 10.1159/000487529
Anette Tønnes Pedersen, Line Cleemann, Katharina M Main, Anders Juul

Hypogonadism may be suspected if puberty is delayed. Pubertal delay may be caused by a normal physiological variant, by primary ovarian insufficiency (Turner syndrome), or reflect congenital hypogonadotropic hypogonadism (HH; genetic) or acquired HH (brain lesions). Any underlying chronic disease like inflammatory bowel disease, celiac disease, malnutrition (anorexia or orthorexia), or excessive physical activity may also result in functional HH. Thus, girls with delayed puberty should be evaluated for an underlying pathology before any treatment, including oral contraception, is initiated. Estrogen replacement is important and natural 17β-estradiol, preferably transdermally, is the preferred choice, whereas the oral route can be used as an alternative depending on patient preference and compliance. Sexual activity is often delayed in the hypogonadal adolescent girl. In the adolescent hypogonadal girl, hormone replacement therapy (HRT) most likely has been initiated at the time she becomes sexually active. If a risk of unwanted pregnancy cannot be ruled out, there is a need to consider contraception. This consideration does not contradict the principles of HRT but can be included as a part of the substitution, e.g. oral contraceptives containing 17β-estradiol or a progestogen intrauterine device combined with continuous 17β-estradiol (transdermal or oral).

如果青春期推迟,可能会怀疑性腺功能减退。青春期延迟可能是由正常生理变异、原发性卵巢功能不全(特纳综合征)或先天性促性腺功能低下(HH;遗传)或获得性HH(脑部病变)。任何潜在的慢性疾病,如炎症性肠病、乳糜泻、营养不良(厌食症或正食症)或过度的身体活动也可能导致功能性HH。因此,青春期延迟的女孩应该在任何治疗(包括口服避孕药)开始之前,对潜在的病理进行评估。雌激素替代是重要的,天然的17β-雌二醇,最好是经皮,是首选,而口服途径可以作为一种选择,这取决于患者的偏好和依从性。性腺功能低下的青春期女孩的性活动往往推迟。在青春期性腺功能低下的女孩中,激素替代疗法(HRT)很可能在她变得性活跃的时候就开始了。如果不能排除意外怀孕的风险,就需要考虑避孕。这种考虑并不与激素替代疗法的原则相抵触,但可以作为替代的一部分,例如含有17β-雌二醇的口服避孕药或结合连续17β-雌二醇的孕激素宫内节育器(透皮或口服)。
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引用次数: 4
Fertility Preservation in Endocrine Disorders during Transition for Girls. 女孩过渡期内分泌失调的生育能力保存。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487530
Julie Bénard, Nathalie Sermondade, Michaël Grynberg

Recent advances in fertility preservation (FP) techniques have led to a wide spread of indications. FP should now be discussed not only for young girls having to receive gonadotoxic treatments for cancer, but also for those with genetic or endocrine disorders, as well as benign ovarian diseases at risk of premature ovarian insufficiency. For premenarchal girls, ovarian tissue cryopreservation is still the only available technique. Oocyte cryopreservation after ovarian stimulation could be offered to postmenarchal girls. Whenever possible, the parents and the young patient should be informed of the benefits to be expected, as well as of the risks and limits of FP for children.

生育保存技术的最新进展导致了适应症的广泛传播。现在不仅应该对接受促性腺毒素治疗的年轻女孩讨论计划生育,而且应该对那些患有遗传或内分泌失调以及有卵巢早衰风险的良性卵巢疾病的女孩讨论计划生育。对于月经前的女孩,卵巢组织冷冻保存仍然是唯一可用的技术。卵巢刺激后卵母细胞冷冻保存可用于绝经后少女。在可能的情况下,应告知父母和年轻患者预期的益处,以及计划生育对儿童的风险和限制。
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引用次数: 2
Transition of Care from Childhood to Adulthood: Congenital Adrenal Hyperplasia. 从童年到成年护理的过渡:先天性肾上腺增生。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487523
Anne Bachelot

Deficiency of the 21-hydroxylase enzyme is the most common form of congenital adrenal hyperplasia (CAH), accounting for more than 95% of the cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, impaired bone mineral density, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and practitioners. In adulthood, the aims of the medical treatment are to substitute cortisol and, when necessary, aldosterone deficiency, to ensure normal fertility, and to avoid the long-term consequences of glucocorticoid use on bone, metabolism, and cardiovascular risk. Recent data suggest that poor health status is likely to begin in adolescence and persist into adulthood, highlighting the importance of this time period in a patient's endocrine care. During transition from pediatric to adult specific care, a shift in treatment goals is thus needed. Successful transition from pediatric to adult health care requires a regular follow-up of patients by a multidisciplinary team including pediatric endocrinologists, urologists, gynecologists, psychiatrists, and adult endocrinologists. All of this could be included in a specific therapeutic education program regarding transition and/or CAH.

21-羟化酶缺乏是先天性肾上腺增生(CAH)最常见的形式,占95%以上的病例。随着新生儿筛查和激素替代疗法的出现,大多数患有CAH的儿童存活到成年。患有CAH的青少年和成人会出现许多并发症,包括身材矮小、肥胖、不孕、骨密度受损和生活质量下降。从儿科过渡到成人护理和管理的长期并发症是具有挑战性的患者和从业人员。在成年期,医学治疗的目的是替代皮质醇,必要时替代醛固酮缺乏,以确保正常的生育能力,并避免糖皮质激素对骨骼、代谢和心血管风险的长期影响。最近的数据表明,健康状况不佳可能从青春期开始,并持续到成年,这突出了这一时期对患者内分泌护理的重要性。在从儿童到成人的特殊护理过渡期间,治疗目标的转变是必要的。从儿科到成人医疗保健的成功过渡需要由包括儿科内分泌学家、泌尿科医生、妇科医生、精神科医生和成人内分泌学家在内的多学科团队对患者进行定期随访。所有这些都可以包含在关于过渡和/或CAH的特定治疗教育计划中。
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引用次数: 14
Fertility Preservation in Klinefelter Syndrome Patients during the Transition Period. Klinefelter综合征患者过渡期生育能力的保存。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487531
Nathalie Rives, Aurélie Rives, Christine Rondanino, Mireille Castanet, Ariane Cuny, Louis Sibert

Spermatozoa have occasionally been identified in ejaculate of adult Klinefelter syndrome (KS) patients but very exceptionally in KS adolescents. Spermatozoa can also be retrieved in testicular tissue of KS adolescents. The testis may also harbor spermatogonia and noncompletely differentiated germ cells. Neither clinical features nor hormonal parameters could predict germ cell recovery in KS adults or adolescents. No predictive factors can actually demonstrate that early diagnosis of KS would allow increasing the chance of sperm retrieval even if it has been suggested that semen quality may decline with age in KS patients. Leydig cell dysfunction may also be another factor that might affect the spermatogenesis process in XXY adolescents. Fertility preservation might be preferentially proposed in KS adolescents when semen sampling is possible, when the patient is able to consider alternative options to become a father, and to accept germ cell retrieval failure. However, precocious diagnosis of KS has also to be considered because it might not solely improve the possibility of fertility preservation after the onset of puberty, but also the medical care and the quality of life of these patients.

精子偶尔在成人Klinefelter综合征(KS)患者的射精中被发现,但在KS青少年中非常罕见。精子也可以在KS青少年的睾丸组织中恢复。睾丸也可能含有精原细胞和未完全分化的生殖细胞。无论是临床特征还是激素参数都不能预测KS成人或青少年的生殖细胞恢复。没有预测因素可以证明早期诊断KS会增加精子恢复的机会,即使有证据表明KS患者的精液质量可能随着年龄的增长而下降。间质细胞功能障碍也可能是影响XXY青少年精子发生过程的另一个因素。当精液取样是可能的,当患者能够考虑成为父亲的其他选择,并且接受生殖细胞回收失败时,生育能力保存可能优先建议给KS青少年。然而,对KS的过早诊断也需要考虑,因为它不仅可以提高青春期开始后保留生育能力的可能性,而且还可以提高这些患者的医疗护理和生活质量。
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引用次数: 7
Challenges of the Transition from Pediatric Care to Care of Adults: "Say Goodbye, Say Hello". 从儿科护理过渡到成人护理的挑战:“说再见,说你好”。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487521
Philippe Touraine, Michel Polak

Transition has been defined as "the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems." We will here describe the challenges of such a process: challenges coming from the pediatrician, from the adolescent, linked to the disease itself, and those from the parents. We will outline how to overcome those fears and challenges to provide a successful transition process. A key factor to underline that process is that a relationship based on confidence should be established between the pediatrician and the physician for adults, in order for that relationship, based on trust, to be the basis for the transfer of the adolescent from the pediatric system of care to the adult one.

过渡被定义为“患有慢性身体和医疗疾病的青少年和年轻人有目的、有计划地从以儿童为中心的卫生保健系统转向以成人为导向的卫生保健系统。”我们将在这里描述这个过程中的挑战:来自儿科医生的挑战,来自青少年的挑战,与疾病本身有关,以及来自父母的挑战。我们将概述如何克服这些恐惧和挑战,以提供一个成功的过渡进程。强调这一过程的一个关键因素是,应该在儿科医生和成人医生之间建立一种基于信任的关系,以便使这种基于信任的关系成为将青少年从儿科护理系统转移到成人护理系统的基础。
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引用次数: 7
Growth Hormone Deficiency in the Transition Age. 过渡年龄的生长激素缺乏。
Pub Date : 2018-01-01 Epub Date: 2018-06-08 DOI: 10.1159/000487525
Sandro Loche, Natascia Di Iorgi, Giuseppa Patti, Serena Noli, Marta Giaccardi, Irene Olivieri, Anastasia Ibba, Mohamad Maghnie

Growth hormone (GH) is essential not only for normal growth during childhood, but also for the acquisition of bone mass and muscle strength in both sexes. This process is completed after the achievement of adult height in the phase of transition from adolescence to adulthood. Adolescents with childhood onset GH deficiency (GHD) show reduction of bone mineral density, decrease in lean body mass, increase in fat mass, and deterioration of the lipid profile. For this reason, continuation of GH replacement therapy in the transition age is recommended in all patients with a confirmed diagnosis of GHD. To confirm the diagnosis of GHD, GH treatment should be discontinued for at least 1 month after the attainment of adult height, and the patient should be re-evaluated for GH secretion. Current guidelines indicate that retesting is not required for those with a transcription factor mutation, more than 3 pituitary hormone deficits, or isolated GHD associated with an identified mutation. The key predictors of persistent GHD are its severity, the presence of additional pituitary hormone deficits, low insulin-like growth factor I (IGF-I) concentration, and the presence of structural hypothalamic-pituitary abnormalities Treatment should be initiated with a low dose (0.2-0.5 mg/day s.c.) and then adjusted according to IGF-I concentrations.

生长激素(GH)不仅对儿童时期的正常生长至关重要,而且对两性骨量和肌肉力量的获得也至关重要。这个过程是在青春期到成年期的过渡阶段达到成人身高后完成的。儿童期发生生长激素缺乏症(GHD)的青少年表现为骨密度降低,瘦体重减少,脂肪量增加,脂质谱恶化。因此,建议所有确诊为GHD的患者在过渡年龄继续接受生长激素替代治疗。为确认GHD的诊断,应在达到成人身高后至少1个月停止GH治疗,并重新评估患者的GH分泌情况。目前的指南指出,对于转录因子突变、超过3种垂体激素缺陷或与确定突变相关的孤立性GHD,不需要重新检测。持续性GHD的关键预测因素是其严重程度、是否存在额外的垂体激素缺陷、低胰岛素样生长因子I (IGF-I)浓度以及是否存在结构性下丘脑-垂体异常。治疗应以低剂量(0.2-0.5 mg/d s.c)开始,然后根据IGF-I浓度进行调整。
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引用次数: 11
期刊
Endocrine development
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