Sex Steroid Replacement Therapy in Female Hypogonadism from Childhood to Young Adulthood.
Abstract
The overall goal of pubertal sex hormone replacement therapy (HRT) in girls is not only about development of secondary sexual characteristics, but also to establish an adult endocrine and metabolic milieu, as well as adult cognitive function. Estradiol (E2) is the first choice for HRT compared to ethinyl estradiol (EE2). E2 is the most potent endogenous estrogen in the circulation, with established levels during spontaneous puberty. Transdermal E2, compared to oral administration, is the first choice to start pubertal HRT. Transdermal application avoids liver exposure to supraphysiologic estrogen ...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Norjavaara E, Ankarberg-Lindgren C, Kriström B Tags: Endocr Dev Source Type: research
Gonadotropin-Releasing Hormone Agonist Treatment in Sexual Precocity.
Abstract
Depot gonadotropin-releasing hormone (GnRH) analogs represent the first-line therapy in sexual precocity due to central precocious puberty. GnRH analogs desensitize the pituitary and account for the suppression of luteinizing hormone and follicle-stimulating hormone leading to a decrease of sex steroid levels. The conventional indications are central puberty starting before the age of 8 years in girls and 9 years in boys. These indications can be extended to difficult conditions with poor adult height prognosis or marked psychosocial impact. This includes children after irradiation, international ...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Pienkowski C, Tauber M Tags: Endocr Dev Source Type: research
Treatment of Peripheral Precocious Puberty.
Abstract
There are many etiologies of peripheral precocious puberty (PPP) with diverse manifestations resulting from exposure to androgens, estrogens, or both. The clinical presentation depends on the underlying process and may be acute or gradual. The primary goals of therapy are to halt pubertal development and restore sex steroids to prepubertal values. Attenuation of linear growth velocity and rate of skeletal maturation in order to maximize height potential are additional considerations for many patients. McCune-Albright syndrome (MAS) and familial male-limited precocious puberty (FMPP) represent rare...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Schoelwer M, Eugster EA Tags: Endocr Dev Source Type: research
Consultation for Disordered Puberty: What Do Adolescent Medicine Patients Teach Us?
Abstract
The period of adolescence is not only marked by important growth and pubertal events, but is also characterized by important psychosocial changes driven by a search for autonomy and the construction of one's identity. It can thus be easily understood that puberty disorders interfere heavily with these process, requiring from the endocrinologist not only medical knowledge, but also a great deal of emotional and psychological skills. They must progressively move from an educational approach that heavily involves the parents to one of shared information and decision making that places the young patie...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Michaud PA, Ambresin AE Tags: Endocr Dev Source Type: research
Toward Automation of Insulin Delivery - Management Solutions for Type 1 Diabetes.
Abstract
In the past decade, the field of type 1 diabetes was characterized by the efforts to integrate technology into the daily management of diabetes. Automated insulin delivery systems have emerged followed by the improvements in technology of pumps and sensors and automated close-loop systems that were developed around the world for overnight as well as for day and night use. Initially, these closed-loop systems were tested clinically in research centers, then at diabetes camps or hotels, and recently at patients' homes. The systems were tested in a wide range of populations of patients with type 1 di...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Nimri R, Phillip M Tags: Endocr Dev Source Type: research
Islet Transplantation in Pediatric Patients: Current Indications and Future Perspectives.
Abstract
The first islet transplantation in diabetes mellitus was performed more than 20 years ago. Since then, clinical results have progressively improved. Nowadays, islet transplantation can be considered a real therapeutic option after pancreatectomy for painful chronic pancreatitis (autotransplantation) and in selected adult patients affected by type 1 diabetes mellitus (allotransplantation). Better results are mainly due to the advances in the standardization of islet isolation and purification procedures as well as in the pharmacological treatment of recipients. Anti-inflammatory treatments facilita...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Bertuzzi F, Antonioli B, Tosca MC, Galuzzi M, Bonomo M, Marazzi M, Colussi G Tags: Endocr Dev Source Type: research
Glucagon-Like Peptide-1 Receptor Agonist Treatment for Pediatric Obesity.
Abstract
Obesity is a complex and retractable disease for which effective and durable treatments are elusive. Successful treatment of severe obesity with lifestyle modification therapy alone is highly unlikely, particularly for adolescents. Pharmacotherapy, if appropriately prescribed, can be an effective tool to use in conjunction with lifestyle modification therapy to achieve better weight loss outcomes. Only a few obesity medications have been evaluated in children and adolescents with results suggesting modest efficacy. However, a new pipeline of obesity drugs has been recently approved for use among a...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Kelly AS Tags: Endocr Dev Source Type: research
Docosahexaenoic Acid and Its Role in G-Protein-Coupled Receptor 120 Activation in Children Affected by Nonalcoholic Fatty Liver Disease.
Abstract
Nonalcoholic fatty liver disease (NAFLD) is one of the most important causes of chronic liver disease in children and adults. Recently, therapeutic supplementation with docosahexaenoic acid (DHA) showed an anti-inflammatory and insulin-sensitizing effect in children with NAFLD. The anti-inflammatory effects of DHA depend on its ability to alter phospholipid composition of the cell membrane, to disrupt lipid rafts and to hamper the transcriptional activity of nuclear factor-x03BA;B that controls the expression of proinflammatory cytokines. These effects of DHA are due to the interaction with the G-...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Della Corte C, Mosca A, Ionata A, Nobili V Tags: Endocr Dev Source Type: research
Noninvasive Prenatal Diagnosis of Congenital Adrenal Hyperplasia.
We describe the utilization of cell-free fetal DNA in mothers carrying at-risk fetuses as early as 6 gestational weeks by targeted massively parallel sequencing of the genomic region including and flanking the CYP21A2 gene. Our highly personalized and innovative approach should permit the diagnosis of CAH before genital development begins, therefore restricting the purposeful administration of dexamethasone to mothers carrying affected females.
PMID: 26683339 [PubMed - in process] (Source: Endocrine Development)
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Khattab A, Yuen T, Sun L, Yau M, Barhan A, Zaidi M, Lo YM, New MI Tags: Endocr Dev Source Type: research
Recent Advances in Hydrocortisone Replacement Treatment.
Abstract
Since the first use of cortisone in patients around 65 years ago, the use of synthetic glucocorticoids has made a crucial impact on the treatment of several diseases in medicine. Although significant reductions in morbidity and mortality have occurred in patients suffering from cortisol deficiency, conventional hydrocortisone replacement treatment is still inadequate. A major limitation is that it fails to replace cortisol in a physiological manner. Cortisol has a distinct circadian rhythm and acts as a secondary messenger synchronizing the central to peripheral clocks, hence playing a key role in...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Mallappa A, Debono M Tags: Endocr Dev Source Type: research
Experience with the Histrelin Implant in Pediatric Patients.
Abstract
The histrelin implant has emerged as a therapeutic option for the treatment of central precocious puberty that has been favorably received by patients and providers. Inserted subcutaneously, the 50-mg implant provides continuous release of the potent gonadotropin-releasing hormone analog (GnRHa) histrelin. Profound suppression of the hypothalamic-pituitary-gonadal (HPG) axis occurs within 1 month of its placement resulting in pubertal arrest, attenuation of skeletal advancement and a progressive increase in predicted adult height. Although marketed for annual use, suppression lasting 2 years from ...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Eugster EA Tags: Endocr Dev Source Type: research
Different Medications for Hypogonadotropic Hypogonadism.
Abstract
Delayed puberty (DP) in boys is the lack of sexual maturation at a chronological age of 14 years. Several conditions induce DP and they can be classified into reversible and irreversible causes. The most common cause of DP is constitutional delay of puberty (CDP; 63%), followed by DPs due to functional hypogonadotropic hypogonadism (HH; 20%), congenital isolated HH (9%) and hypergonadotropic hypogonadism (7%). A correct diagnosis, although often difficult, is pivotal for choosing the most adequate therapy. In CDP boys, expectant management can be an option. However, patient's psychological distres...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Rastrelli G, Vignozzi L, Maggi M Tags: Endocr Dev Source Type: research
Long-Acting Growth Hormone: An Update.
Abstract
After the introduction of recombinant human growth hormone (rhGH) in 1985, a myriad of children and adults have benefited from its growth-promoting and metabolic effects. Nowadays, current therapeutic regimens rely on daily subcutaneous GH injections that could be burdensome and inconvenient to pediatric patients. As expected with any long-term parenteral pharmacological treatment, these daily regimens may promote nonadherence, poor compliance, treatment abandonment and/or suboptimal clinical outcomes. In order to improve patient and caregiver acceptance of proposed regimens, simplified dosing sch...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Saenger PH, Mejia-Corletto J Tags: Endocr Dev Source Type: research
C-Type Natriuretic Peptide Analog as Therapy for Achondroplasia.
Abstract
Fibroblast growth factor receptor 3 (FGFR3) is an important regulator of bone formation. Gain-of-function mutations in the FGFR3 gene result in chondrodysplasias which include achondroplasia (ACH), the most common form of dwarfism, in which skull, appendicular and axial skeletons are affected. The skeletal phenotype of patients with ACH showed defective proliferation and differentiation of the chondrocytes in the growth plate cartilage. Both endochondral and membranous ossification processes are disrupted during development. At cellular level, Fgfr3 mutations induce increased phosphorylation of th...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Legeai-Mallet L Tags: Endocr Dev Source Type: research
Therapeutic Neuroendocrine Agonist and Antagonist Analogs of Hypothalamic Neuropeptides as Modulators of the Hypothalamic-Pituitary-Gonadal Axis.
Abstract
Reproductive hormones play a role at all stages of life and affect most tissues of the body. Gonadotropin-releasing hormone (GnRH) synthesized in the hypothalamus stimulates the secretion of gonadotropins which in turn stimulate gonadal sex hormone production and gamete formation. This hypothalamic-pituitary-gonadal (HPG) axis has, therefore, been the target for the development of numerous drugs which regulate it at various points. These include sex steroid agonists and antagonists, inhibitors of sex steroid biosynthesis, and GnRH agonists and antagonists, which have found extensive applications i...
Source: Endocrine Development - December 21, 2015 Category: Endocrinology Authors: Newton CL, Anderson RC, Millar RP Tags: Endocr Dev Source Type: research
