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

Related Links:

Purpose Bariatric surgery is considered as an effective therapeutic strategy for weight loss in severe obesity. Remission of type 2 diabetes is often achieved after the surgery. We investigated whether increase in self-reported habitual physical activity associates with improved skeletal muscle insulin sensitivity and reduction of fat depots after bariatric surgery. Methods We assessed self-reported habitual physical activity using Baecke questionnaire in 18 diabetic and 28 nondiabetic patients with morbid obesity (median age, 46 yr; body mass index, 42.0 kg·m−2) before and 6 months after bariatric surger...
Source: Medicine and Science in Sports and Exercise - Category: Sports Medicine Tags: CLINICAL SCIENCES Source Type: research
Conclusions Substantial variability in response to standardized exercise was observed for change in both WC and body weight after accounting for the variability not attributed to exercise. Potential determinants of the interindividual variability in response to exercise remain unclear.
Source: Medicine and Science in Sports and Exercise - Category: Sports Medicine Tags: APPLIED SCIENCES Source Type: research
CONCLUSIONSFuture research is needed to determine whether obese women seeking abortions are being referred despite evidence that they do not require hospital ‐based care. If obese women are suffering delays because of referral, strategies to help overcome delay should also be explored.
Source: The Guttmacher Institute - Category: International Medicine & Public Health Authors: Source Type: news
We describe an adolescent with severe obesity, iron deficiency, poor iron absorption, and their impact on decision-making for surgical treatment of her severe obesity. We present a case of a 15-year-old female with a BMI of 50 kg/m2 who was referred for bariatric surgery requesting a RYGB. During her preoperative evaluation, mild iron deficiency anemia was identified after noting a low hemoglobin concentration and mean corpuscular volume. Endoscopic biopsy revealed mild chronic gastritis. Iron supplementation over a 9-month period did not improve her anemia preoperatively and an oral iron challenge demonstrated inadequat...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
ConclusionsThis study showed that the occurrence of OSA in patients with MG is associated with male sex and obesity, which is in accordance with the normal population. Moreover, PSG data showed a high prevalence of supine dominant OSA in MG patients with OSA.
Source: Neurological Sciences - Category: Neurology Source Type: research
ConclusionIn this Hispanic population undergoing the nutrition transition, total red meat intake may have an impact on MetS. Based on the relatively low consumption of red meat in Costa Rica compared to other Westernized countries, we hypothesize that a “threshold effect” may exist for unprocessed and processed red meat.
Source: European Journal of Nutrition - Category: Nutrition Source Type: research
AbstractPurposeVitamin D regulates adipokine production in vitro; however, clinical trials have been inconclusive. We conducted secondary analyses of a randomized controlled trial to examine whether vitamin D supplementation improves adipokine concentrations in overweight/obese and vitamin D-deficient adults.MethodsSixty-five individuals with a BMI  ≥ 25 kg/m2 and 25-hydroxyvitamin D (25(OH)D)  ≤ 50 nmol/L were randomized to oral cholecalciferol (100,000 IU single bolus followed by 4,000 IU daily) or matching placebo for 16 weeks. We measured BMI, waist-to-hip ratio, % ...
Source: European Journal of Nutrition - Category: Nutrition 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
More News: Cardiology | Cardiovascular | Child Development | Children | Congenital Adrenal Hyperplasia | Eating Disorders & Weight Management | Education | Endocrinology | Health Management | Heart | Hormone Replacement Therapy | Hormones | Infertility | Obesity | Pediatrics | Psychiatry | Reproduction Medicine | Universities & Medical Training | Urology & Nephrology