Transition of Care from Childhood to Adulthood: Congenital Adrenal Hyperplasia.

Transition of Care from Childhood to Adulthood: Congenital Adrenal Hyperplasia. Endocr Dev. 2018 Jun 08;33:17-33 Authors: Bachelot A Abstract 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 e...
Source: Endocrine Development - Category: Endocrinology Authors: Tags: Endocr Dev Source Type: research

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CONCLUSIONS: The identification of inflammatory biomarkers released by adipose tissue, in the pathogenesis of EC, could be useful in improving diagnostic accuracy, identifying targets of therapy, suggesting useful lifestyle behaviors. A deeper knowledge of the genetic background of alterations in inflammatory pathway genes could better define the population exposed to a higher susceptibility to EC due to genetic polymorphisms. Future studies are needed to better understand this field. PMID: 30338797 [PubMed - in process]
Source: European Review for Medical and Pharmacological Sciences - Category: Drugs & Pharmacology Tags: Eur Rev Med Pharmacol Sci Source Type: research
CONCLUSIONS: Prenatal exercise is safe and beneficial for the fetus. Maternal exercise was associated with reduced odds of macrosomia (abnormally large babies) and was not associated with neonatal complications or adverse childhood outcomes. PMID: 30337465 [PubMed - in process]
Source: British Journal of Sports Medicine - Category: Sports Medicine Authors: Tags: Br J Sports Med Source Type: research
ConclusionsOur data provide evidence that moderate weight loss attenuates systemic inflammation and IR and promotes the amelioration of ER stress and mitochondrial dysfunction, increasing the expression of chaperones, SIRT1 and antioxidant GPX1.Graphical abstract
Source: Molecular Metabolism - Category: Endocrinology Source Type: research
ConclusionAltogether, we have identified several genes that act within the CNS to regulate energy homeostasis. One of these, Dgk, acts within the insulin-producing cells to regulate the secretion of dILPs and energy homeostasis in Drosophila.Graphical abstract
Source: Molecular Metabolism - Category: Endocrinology Source Type: research
ConclusionsThis study shows that reduction of CTRP3 concentrations is likely to bring a concomitant increase in risk of diabetes in obese and normal body weight young adults. Decrease in CTRP3 concentration may have an essential role in the pathophysiology of metabolic disorders concomitant to obesity.
Source: Diabetes and Metabolic Syndrome: Clinical Research and Reviews - Category: Endocrinology Source Type: research
In conclusion, Ffar1 expression is markedly down-regulated under diabetic conditions which is accompanied by decreased expression of Pdx1. Furthermore, it is likely that Pdx1 is a regulator of Ffar1 expression in β-cells. PMID: 30333365 [PubMed - as supplied by publisher]
Source: Endocrine Journal - Category: Endocrinology Tags: Endocr J Source Type: research
This study was conducted in Turkey as an empirical study in randomized, controlled, and time series design for the purpose of evaluating the effects of the Diet-Exercise trainings with creative drama method provided to overweight/obese teenagers. METHODS: This study was designed to highlight the effect of interactive education provided to slightly overweight/obese teenagers through creative drama on modifiable factors that caused obesity. Seventy-six overweight/obese teenagers studying at sixth and seventh grades of upper primary education were included in the study (38 participants in the Study Group and 38 participan...
Source: Childhood Obesity - Category: Eating Disorders & Weight Management Tags: Child Obes Source Type: research
Authors: Choi JH, Yoo HW Abstract Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of 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, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial iss...
Source: Korean Journal of Pediatrics - Category: Pediatrics Tags: Korean J Pediatr Source Type: research
DiscussionPolycystic ovary syndrome (PCOS) affects 6-8% of reproductive-age women making it the most common endocrinopathy in this age group. There is no consensus on the specific diagnostic criteria for PCOS in adolescents as many of the characteristics overlap with normal adolescent physiology. However, patients should have evidence of hyperandrogenism, oligo- or amenorrhea, and potentially polycystic ovaries. PCOS has a genetic component although a specific gene has not been identified. Incidence of PCOS is 20-40% for a woman with a family history. Hyperandrogenism Androgen levels change during puberty therefore actual ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Abstract Nonclassic congenital adrenal hyperplasia (NCAH) is one of the most frequent autosomal recessive disorders in man with a prevalence ranging from 0.1 % in Caucasians up to a few percent in certain ethnic groups. Most cases are never diagnosed due to very mild symptoms, misdiagnosing as polycystic ovary syndrome, or ignorance. In contrast to classic CAH, patients with NCAH present with mild partial cortisol insufficiency and hyperandrogenism and will survive without any treatment. Undiagnosed NCAH may result in infertility, miscarriages, oligomenorrhea, hirsutism, acne, premature pubarche, testicular a...
Source: Endocrine - Category: Endocrinology Source Type: research
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