Emerging Mechanisms of GH-Induced Lipolysis and Insulin Resistance.
Authors: Sharma VM Abstract Growth hormone (GH) is a pleiotropic hormone that coordinates an array of physiological processes including growth and metabolism. GH promotes anabolic action in all tissues except adipose, where it catabolizes stored fat to release energy for the promotion of growth in other tissues. However, chronic stimulation of lipolysis by GH results in an increased flux of free fatty acids (FFAs) into systemic circulation. Hence, a sustained release of high levels of GH contributes significantly to the development of insulin resistance by antagonizing the anti-lipolytic action of insulin....
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

FGF23 and Associated Disorders of Phosphate Wasting.
Authors: Gohil A, Imel EA Abstract Fibroblast growth factor 23 (FGF23), one of the endocrine fibroblast growth factors, is a principal regulator in the maintenance of serum phosphorus concentration. Binding to its cofactor αKlotho and a fibroblast growth factor receptor is essential for its activity. Its regulation and interaction with other factors in the bone-parathyroid-kidney axis is complex. FGF23 reduces serum phosphorus concentration through decreased reabsorption of phosphorus in the kidney and by decreasing 1,25 dihydroxyvitamin D (1,25(OH)2D) concentrations. Various FGF23-mediated disorders of r...
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Are We Aware that Hyperphosphatemia Affects Mortality and Morbidity as much as Hypophosphatemia in Pediatric Intensive Care Patients?
CONCLUSION: In our study, we identified that hyperphosphatemia is a serious problem as hypophosphatemia for patients who admitted to the PICU. Patients identified to be hyperphosphatemic on admission had a significantly higher PELOD score. The significant difference of hyperphosphatemia in terms of PELOD score is one of the important points shown in our study. It should not be forgotten that like hypophosphatemia, hyperphosphatemia may cause serious problems in pediatric intensive care patients. PMID: 31599134 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Deficiency and Excessive Sleepiness: A Case Report and Review of the Literature.
Authors: Gohil A, Eugster E Abstract The somatotropic axis is intricately involved in normal sleep, as evidenced by the fact that hypothalamic growth hormone-releasing hormone (GHRH) has sleep promoting effects and pituitary growth hormone (GH) release is strongly associated with slow-wave sleep (SWS). Abnormalities in the somatotropic axis, such as GH deficiency of hypothalamic or pituitary origin, result in an alteration of normal sleep patterns which may explain the fatigue reported in these individuals. Sleep disorders such as narcolepsy, in which individuals abnormally enter rapid eye movement (REM) s...
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Letter to the Editor: Central Nervous System Complications in Diabetic Ketoacidosis.
Authors: Baszyńska-Wilk M, Wysocka-Mincewicz M, Wajda-Cuszlag M, Świercz A, Szalecki M PMID: 31599136 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: 2019 Annual Meeting of the Pediatric Endocrine Society: Selected Highlights Baltimore, MD, April 26-29, 2019.
Authors: Guiffre D, Kim G, Gupta MB PMID: 31599137 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 12, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: Testicular Volume Development along Ages: Evaluation by Different Methods.
Authors: De Sanctis V, Soliman AT, Di Maio S, Millimaggi G, Kattamis C Abstract In the last five decades an increasing number of studies and clinical reports demonstrated the importance of testicular volume assessment in pediatric and adolescent population. Reliable and accurate determination of testicular volume (TV) through infancy and adolescence is of great importance for assessing normal pubertal development to diagnose disturbances in development and to suspect certain genetic and endocrine diseases. Various approaches are available for the assessment of TV, including orchidometry, rulers, callipers,...
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Karyotype - Phenotype Associations in Patients with Turner Syndrome.
This study was part of the European multicentre dsd-LIFE study. We evaluated the associations between different karyotypes of TS patients and age at diagnosis, Turner stigmata, cardiac/renal involvement and gonadal function. Information was available for 328 TS patients. Participants had a monosomy 45,X (46%), mosaicism 45,X/46,XX (10%), karyotype with isochromosome (18%), or other karyotype (26%). The clinical signs of TS were the most severe in patients with monosomy 45,X and the least severe in patients with mosaicism 45,X/46,XX. Patients with isochromosome and y-material showed an intermediate phenotype. Despite the mo...
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Sleep Disorders and Obesity in Childhood: A New Component in Solving Obesity.
Authors: Atar M, Pirgon Ö, Buyukgebiz A Abstract Sleep disorders have been widely reported in obese individuals. Previous studies have shown that together with an increase in obesity prevalence, so does sleep duration in children and adolescents decrease. By contributing to energy imbalances, hormonal changes occurring with reduced sleep quality may cause weight gain and obesity. Current evidence shows that short sleep duration has effects on body weight and weight gain. Compared to individuals sleeping for a normal duration, insulin sensitivity is lower in those who sleep less. Lack of sleep increases th...
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Hormones and their Structural and Functional Effects on the Brain: How Can We Change our Practice Moving Forward?
The objectives of this paper are to: 1) Enhance understanding of the uncertainties in the decision-making process regarding hormonal interventions to treat patients with DSD. 2) Recognize that the effects of hormonal interventions might require a consent process similar to that applied for surgical procedures. 3) Emphasize the need to establish treatment algorithms that could form the basis of a standard of care for this patient population. PMID: 31245940 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Height SDS Changes ( ΔhSDS) in Healthy Children from Birth to 18 Years, with Correction Factors for Measurement Intervals of Less than One Year.
Height SDS Changes (ΔhSDS) in Healthy Children from Birth to 18 Years, with Correction Factors for Measurement Intervals of Less than One Year. Pediatr Endocrinol Rev. 2019 Jun;16(4):457-467 Authors: Hermanussen M, Mumm R, Rintisch A, Tutkuviene J, Suchomlinov A, Joubert K, Longas AF, Scheffler C Abstract BACKGROUND: Growth is volatile and non-linear. Assessing the instantaneous speed of growth (momentary height velocity) depends on the precision and the number of measurements and the duration of the observation period. Measurements at short intervals reflect both the non-lineari...
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: 2019 Annual Meeting of the Endocrine Society New Orleans, LA (March 23-26, 2019)Selected Highlights.
Authors: Agarwal S, Seagroves A, Bakhach M, Jindal I PMID: 31245942 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - June 29, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

The History of Noonan Syndrome.
Authors: Miller BS Abstract Early in her career, Jacqueline Noonan, a pediatric cardiologist, recognized that a number of children with valvular pulmonary stenosis had similar facial features. Dr. Noonan reported the clinical characteristics of this condition including short stature, hypertelorism, ptosis, mild mental retardation, undescended testes, and skeletal malformations. Further characterization of Noonan Syndrome led to the development of clinical criteria for the diagnosis of the condition. Identification of the first genetic cause of Noonan Syndrome, mutation of ptpn11 was reported in 2001. Multi...
Source: Pediatric Endocrinology Reviews - May 24, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Clinical Manifestations of Noonan Syndrome and Related Disorders.
Authors: Breilyn MS, Mehta L Abstract Noonan syndrome represents a heterogeneous group of genetic disorders caused by mutations in genes of the RAS/MAPK pathway. Related syndromes include cardiofaciocutaneous syndrome, Noonan syndrome with multiple lentigines and Costello syndrome. The common phenotypic features of Noonan syndrome include facial dysmorphisms, short stature, congenital heart defects and genitourinary abnormalities. These and other findings as well as features of related disorders are discussed. In addition we briefly review clinical diagnosis and prenatal findings of these syndromes and gen...
Source: Pediatric Endocrinology Reviews - May 24, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Molecular Genetics of Noonan Syndrome and RASopathies.
Authors: Liao J, Mehta L Abstract The RAS/MAPK signaling pathway plays an essential role in development and tumorigenesis by regulating cell proliferation, differentiation, apoptosis, migration, and metabolism. Therefore, it is not surprising that germline mutations in genes encoding components or regulators of this signaling pathway cause numerous human genetic conditions, including Noonan syndrome and related disorders. The term "RASopathies" has been used to describe these disorders collectively due to their common underlying RAS/MAPK pathway dysregulation and overlapping clinical features. Taken togeth...
Source: Pediatric Endocrinology Reviews - May 24, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research