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Pediatric Endocrinology Reviews (PER) is the most respected international peer reviewed journal in Pediatric Diabetes, Nutrition Metabolism and Genetics. Hypothyriodism, Hyperthyriodism, Glycemic Management for Children with Diabetes Glucose Monitoring Adrenal Insufficiency Turner Syndrome Late Adolescence Klinefelter Syndrome Children with Short Stature and Growth Failure: Heightism Type 1 Diabetes in Children Growth Hormone Treatment for GHD Insulin-like Growth Factor-I Growth Hormone Deficiency SGA Children with Short Stature Receiving GH Treatment Hypothalamic Obesity Adolescent Gynecomastia Hematospermia in Adolescents Gain-of-Function CDKN1C Mutations Craniopharyngioma Succinate-Dehydrogenase Deficient Paragangliomas/Pheochromocytomas Adrenal Steroidogenesis: Impact on Gonadal Function Focal Congenital Hyperinsulinism (CHI)  Longevity Hormone Klotho Pediatric Congenital Hypothyroid Lysosomal Storage Diseases Juvenile NCL (CLN3 Disease) GM1 and GM2 Gangliosidoses Types A and B Niemann-Pick Disease CLN2 Disease (Classic Late Infantile Neuronal Ceroid Lipofuscinosis) Krabbe Disease Fucosidosis Nuclear Factor Kappa B (NF-κB) in Growth Plate Chondrogenesis Persistent Müllerian Duct Syndrome LHX4 Gene Alterations Stunted Growth 45,X/46,XY Gonadal Dysgenesis Thyroid Hemiagenesis Nutrimetabolomics and Adipocitokines Chromosomal Microarray Analysis (CMA) Chromosomal microarray, Copy Number Variant (CNV), Prenatal, Amniocentesis, Comparative genomic hybridization, SNP array, Diagnosis, Clinical Abreviations: aCGH – array-based comparative genomic hybridization, ASD – autism spectrum disorder, BAC – bacterial artificial chromosome, CHD – congenital heart disease, CMA – chromosomal microarray analysis, CNV – copy number variant, CVS – chorionic villus sampling, DD – developmental delay, DNA – deoxyribonucleic acid, FISH – fluorescent in situ hybridization, GABA - gammaaminobutyric acid, ID – intellectual disability, LOH – loss of heterozygosity, NGS – next generation sequencing, NIPT – noninvasive prenatal testing, NOS – not otherwise specified, PGD - preimplantation genetic diagnosis, SNP – single nucleotide polymorphism, VUS – variant of unclear clinical significance Central precocious puberty, Traumatic brain injury, Pathophysiology Nephrolithiasis, Nephrocalcinosis, Hypercalciuria, Hyperoxaluria, Hypouricemia, Cystinuria, Genetics 

Volume 13.2

December 2015

 

Insulin-Like Growth Factor-I is a Marker for the Nutritional State

Colin P Hawkes, MD , Adda Grimberg, MD

 

Abstract

Measurement of the serum concentration of insulin-like growth factor-I (IGF-I) is generally used as a screening investigation for disorders of the growth hormone (GH)/IGF-I axis in children and adolescents with short stature. IGF-I concentration is sensitive to short-term and chronic alterations in the nutritional state, and the interpretation of IGF-I measurements requires knowledge of the child’s nutritional status. In this review, we summarize the effects of nutrition on the GH/IGF-I axis, and review the clinical implications of these interactions throughout childhood, both in under-nutrition and over-nutrition.

 

Primary Dysmenorrhea in Adolescents: Prevalence, Impact and Recent Knowledge

Vincenzo De Sanctis, MD, Ashraf Soliman, MD, Sergio Bernasconi, MD, Luigi Bianchin4, MD, Gianni Bona, MD, Mauro Bozzola, MD, Fabio Buzi, MD, Carlo De Sanctis, MD, Giorgio Tonini9, MD, Franco Rigon1, MD, Egle Perissinotto, ScD

 

Abstract

Background and Objectives: Dysmenorrhea is commonly categorized into two types; primary and secondary. Primary dysmenorrhea (PD) is the focus of this review. PD is defined as painful menses with cramping sensation in the lower abdomen that is often accompanied by other symptoms, such as sweating, headache, nausea, vomiting, diarrhea, and tremulousness. All these symptoms occur just before or during the menses in women with normal pelvic anatomy.

In adolescents the prevalence of PD varies between 16% and 93%, with severe pain perceived in 2% to 29% of the studied girls. Several studies suggest that severe menstrual pain is associated with absenteeism from school or work and limitation of other daily activities. One-third to one-half of females with PD are missing school or work at least once per cycle, and more frequently in 5% to 14% of them. The wide variation in the prevalence rates may be attributed to the use of selected groups of subjects. Many risk factors are associated with increased severity of dysmenorrhea including earlier age at menarche, long menstrual periods, heavy menstrual flow, smoking and positive family history. Young women using oral contraceptive pills (OCP) report less severe dysmenorrhea.

The considerably high prevalence of dysmenorrhea among adolescents verified that this condition is a significant public health problem that requires great attention.

Summary of Main Results: Many methodological problems are encountered during quantifying and grading severity of pain related to dysmenorrhea. Quantifying and assessment tools depend on women’s self-reporting with potential bias. There is a scarcity of longitudinal studies on the natural history of dysmenorrhea as well as the possible effects of many modifiable risk factors. In addition, the duration of follow-up in the available studies is relatively short. Therefore, several aspects are still open for research.

Medical treatment for dysmenorrhea includes anti-inflammatory drugs (NSAIDs), OCP or surgical intervention. The efficacy of conventional treatments using NSAIDs and OCP is high. However, failure rate may reach up to 20% to 25%, besides the occurrence of drug-associated adverse effects. Only 6% of adolescents receive medical advice to treat dysmenorrhea while 70% practice self-management. Unfortunately, some girls even abuse these medications (non-therapeutic high doses) for quick pain relief.

The persistence of dysmenorrhea despite the use of OCP and/or NSAIDs drugs is a strong indicator of an organic pelvic disease. This condition mandates an appropriate referral to a gynecologist with proper laparoscopic diagnosis of endometriosis and/or other pelvic diseases.

Conclusions: Dysmenorrhea is an important health problem for adolescents, school and occupational as well as practitioners that adversely affects the daily activities and quality of life for adolescent women. The accurate prevalence of dysmenorrhea is difficult to establish due to the variety of diagnostic criteria and the subjective nature of the symptoms. In adolescents, moderate to severe dysmenorrhea that affects lifestyle and does not respond to medical treatment requires professional attention and proper diagnosis of possible underlying pelvic disease. Therefore, adolescent care providers should be more knowledgeable and actively involved in the care of dysmenorrhea.

 

Ref: Ped. Endocrinol. Rev. 2015;13(2):512-520

Key words: Dysmenorrhea, Adolescents, Epidemiology, Health problems

 

Insulin-Like Growth Factor Binding Proteins-an Update

Leon A Bach, MB, BS, PhD, FRACP

 

Abstract

The insulin-like growth factor (IGF) system is essential for normal growth and development, and its perturbation is implicated in a number of diseases. IGF activity is finely regulated by a family of six high-affinity IGF binding proteins (IGFBPs). IGFBPs usually inhibit IGF actions but may enhance them under certain conditions. Additionally, IGFBPs bind non-IGF ligands in the extracellular space, cell membrane, cytoplasm and nucleus, thereby modulating cell proliferation, survival and migration in an IGF-independent manner. IGFBP activity is regulated by transcriptional mechanisms as well as by post-translational modifications and proteolysis. Understanding the balance between the various actions of IGFBPs in vivo may lead to novel insights into disease processes and possible IGFBP-based therapeutics.

Ref: Ped. Endocrinol. Rev. 2015;13(2):521-530

Key words: Insulin-like growth factor, Binding protein, Regulation, Cellular actions, Protein structure

 

The Effects of Electromagnetic Field on the Endocrine System in Children and Adolescents

Özlem Sangün, MD, Bumin Dündar, MD, Selçuk Çömlekçi, PhD, Attila Büyükgebiz, MD

 

Abstract

Children are exposed to various kind of non-ionizan radiation in their daily life involuntarily. The potential sensitivity of developing organism to the effects of radiofrequency (RF) signals, the higher estimated specific absorption rate (SAR) values of children and greater lifetime cumulative risk raised the scientific interest for children’s vulnerability to electromagnetic fields (EMFs).

In modern societies, children are being exposed to EMFs in very early ages. There are many researches in scientific literature investigating the alterations of biological parameters in living organisms after EMFs. Although the international guidelines did not report definite, convincing data about the causality, there are unignorable amount of studies indicating the increased risk of cancer, hematologic effects and cognitive impairment. Although they are less in amount; growing number of studies reveal the impacts on metabolism and endocrine function. Reproductive system and growth look like the most challenging fields. However there are also some concerns on detrimental effects of EMFs on thyroid functions, adrenal hormones, glucose homeostasis and melatonin levels.

It is not easy to conduct a study investigating the effects of EMFs on a fetus or child due to ethical issues. Hence, the studies are usually performed on virtual models or animals. Although the results are conflicting and cannot be totally matched with humans; there is growing evidence to distress us about the threats of EMF on children.

Ref: Ped. Endocrinol. Rev. 2015;13(2):531-545

Key words: Electromagnetic field, Endocrine system, Child, Adolescent

 

Nutrimetabolomics and Adipocitokines in the “Great Obstetrical Syndromes”

Anna De Magistris, MD, Flaminia Cesare Marincola, Researcher, Vassilios Fanos, MD, Angelica Dessì, MD

 

Abstract

In neonatal medicine, nutritional research is focusing more and more on thrifty phenotype effects, in order to understand and prevent the development of long-term diseases. Ischemic placental disease which brings together Gestational diabete, Preeclamptic Toxemia, and Intrauterine Growth Restriction, the “Great Obstetrical Syndromes" (GOS), originates from sugar and lipid metabolism. Adipokines and metabolomics can be valuable tools for the diagnosis of obstetrical syndromes and addressing nutrition. Inappropriate nutrition, even in the first periods of life, can accelerate the development of chronic metabolic diseases, especially in the pediatric age. The purpose of this review is firstly to critically examine the information provided by the studies of metabolomics on GOS's and better understand their origin. Secondly, it reflects on the IUGR metabolism and on applications of metabolomics in nutrition and its “nutrimetabolomic” effects and then to discuss the principles that guide nutrition of IUGR children in the light of these.

Ref: Ped. Endocrinol. Rev. 2015;13(2):546-558

Key words: IUGR, GDM, PET, Adipocytokines, Nutrimetabolomics, Obstetrical syndromes