For Debate: When is Selenium Deficiency Suspected and When is Its Measurement Indicated?
Authors: Kawai M Abstract Selenium (Se) is an essential trace element involved in numerous biological processes including the antioxidant defense system and thyroid hormone metabolism. Since the content of Se in the body is highly dependent on that in the environment, Se deficiency rarely occurs in individuals living in areas rich in Se; with the exception of preterm infants and patients nourished exclusively with parenteral and enteral nutrition (PN and EN) without Se supplementation. Severe Se deficiency causes increases in T4 levels associated with decreases in T3 levels due to the blockage of the conversion of ...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Genetic Tumor Syndromes with Endocrine Involvement: A Compendium and an Update.
Authors: Kushchayeva Y, Lightbourne M, Lodish M, Stratakis CA Abstract Many hereditary and sporadic tumor and other syndromes are associated with endocrine functional and or structural abnormalities. The last few decades have yielded advancements in the field with improvements in diagnostic testing, screening guidelines and novel treatment options. In general, endocrine functional abnormalities and neoplasms share an early age of onset. There remains room for improvement as limited literature exists regarding clinical course, prognosis, and screening for earlier cancer detection. This should allow for more timely i...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Apparent Mineralocorticoid Excess in the Pediatric Population: Report of a Novel Pathogenic Variant of the 11 β-HSD2 Gene and Systematic Review of the Literature.
Apparent Mineralocorticoid Excess in the Pediatric Population: Report of a Novel Pathogenic Variant of the 11β-HSD2 Gene and Systematic Review of the Literature. Pediatr Endocrinol Rev. 2019 Mar;16(3):335-358 Authors: Adamidis A, Cantas-Orsdemir S, Tsirka A, Abbott MA, Visintainer P, Tonyushkina K Abstract Apparent mineralocorticoid excess (AME) is a rare inherited disorder caused by pathogenic variants in the 11β-HSD2 gene resulting in a deficiency of the 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) enzyme catalyzing the conversion of cortisol to its inactive metabolite, cort...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Enhanced Understanding of the Natural History of Pre-Type 1 Diabetes: Fundamental to Prevention.
Authors: Bruggeman BS, Schatz DA Abstract Due to well-designed studies of birth cohorts and at-risk individuals, our understanding of the natural history of pre- and early type 1 diabetes (T1D) has advanced considerably over the past decade. Genetic risk scores can predict with increasing precision and accuracy who is at risk for T1D, and early staging based upon islet autoantibody status allows for improved mechanistic and natural history studies as well as improved clinical trial design. A growing number of children are being diagnosed with islet autoimmunity prior to the onset of symptoms, and confusion remains ...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

To Freeze or Not to Freeze? An Update on Fertility Preservation In Females with Turner Syndrome.
Conclusion The efficacy of fertility preservation procedures in females with TS is still unknown. Future studies with focus on efficacy, safety and long-term follow-up are desperately needed. PMID: 30888127 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: The Role of Beliefs and Perception on Body Size. Proceedings of the 26th Aschauer Soiree, Held at Aschauhof, Altenhof, Germany, May 26th, 2018.
Authors: Hermanussen M, Pulungan AB, Scheffler C, Mumm R, Rogol AD, Pop R, Swanson JM, Sonuga-Barke E, Reimann A, Siniarska-Wolanska A, Musalek M, Bogin B, Boldsen JL, Tassenaar PGV, Groth D, Liu YC, Meigen C, Quanjer B, Thompson K, Özer BK, Bryl E, Mamrot P, Hanć T, Koziel S, Söderhäll J, Gomula A, Banik SD, Roelants M, Veldre G, Lieberman LS, Sievert LL Abstract Thirty-one scientists met at Aschauhof, Germany to discuss the role of beliefs and self-perception on body size. In view of apparent growth stimulatory effects of dominance within the social group that is observed in social mammals, they d...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: The Equality Project on Endocrine Complications in Thalassemia: Selected Highlights from the First Turkish Congress, Antalya, 10th-11th December 2018.
Authors: Canatan D, De Sanctis V, Corrons JV, Gorar S, Turkkahraman D, Baran RT, Kurtoglu E, Aslan V, Kupesiz FT, Tamburaci Uslu ZD, Ozdemir Z, Erinekci O Abstract Major difficulties reported by endocrinologists /pediatricians/ hematologists in the care of thalassemic patients with endocrine complications were: lack of facilities, correct interpretation of tests, unfamiliarity with medical treatment and the cost of diagnostics and therapeutics. Therefore, there is a felt need to educate and train more endocrinologists/pediatricians/hematologists in this field in order to optimise growth and prevent endocrine compli...
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: Report on the 9th International Congress of the Growth Hormone Research and IGF Societies, September 14-17, 2018, in Seattle, Washington, USA.
Authors: Roberts CT, Yuen KC PMID: 30888130 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - March 21, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: Paediatric T1DM: DKA is Still a Problem.
CONCLUSION: The frequency of DKA although stable, remains high and is associated with a worse evolution of the disease. PMID: 30556656 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

National Cooperative Growth Study: 25 Years of Growth Hormone Data, Insights, and Lessons for Future Registries.
CONCLUSIONS: Twenty-five years of monitoring GH use through the NCGS yielded extensive insight into the utility of GH in various underlying etiologies. Demographic disparities were clear and became evident by analyzing data collected through the registry. PMID: 30556657 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Hypothyroidism in Young Children Following Exposure to Iodinated Contrast Media: An Observational Study and a Review of the Literature.
Authors: Rosenberg V, Michel A, Chodick G, Cheng Y, Palkowitsch P, Koren G, Shalev V Abstract While it is well documented that exposure to iodinated contrast media (ICM) can interfere with thyroid function in adults, much less is known about the incidence and risk factors associated with ICM induced hypothyroidism in young children. Using a computerized database we identified 843 children who were exposed to ICM between 1998 and 2015. The incidence rate of ICM induced hypothyroidism per 1000 person-years was 9.66 (95% CI: 4.17-19.04). When compared to the rest of the cohort, children with hypothyroidism were more l...
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Narcolepsy, Precocious Puberty and Obesity in the Pediatric Population: a Literature Review.
Authors: Maia Palhano AC, Kim LJ, Moreira GA, Santos Coelho FM, Tufik S, Levy Andersen M Abstract Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic and hypnopompic hallucinations. The onset of the symptoms usually occurs in childhood, and previous studies have reported an association between narcolepsy and other endocrine diseases in the pediatric population, such as obesity and precocious puberty. The incidence of overweight or obesity ranges from 25% to 74% in patients with narcolepsy type I, while precocious puberty is present in 17% of childr...
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Ellis-Van Creveld Syndrome and Dandy-Walker Malformation: An Uncommon Association.
We report a case of an 11-year-old Moroccan boy with EVC syndrome and Dandy-Walker malformation. To our knowledge, this association has been previously reported in 3 patients in the literature. PMID: 30556660 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: 2018 Annual Meeting of the Pediatric Endocrine Society, Toronto, Canada, May 5-8, 2018, Selected Highlights.
Authors: Ryabets-Lienhard A, Akhtar S, Monzavi R, Austin J Abstract PMID: 30556661 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - December 19, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Discovery and Structure.
Authors: Buchman M, Bell S, Kopchick JJ Abstract The purpose of this review is to describe and document the discovery of growth hormone (GH) and various activities associated with it. Crucial to this discourse will be a chronicle of results related to the structure of GH. Many individuals were instrumental in the early and current work. Throughout the review we present glimpses into their scientific lives as it affects the evolution of GH's story. We realize that we have not presented a comprehensive review of GH's history and its current and future status, and apologize for the omission of many individuals who con...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

The Era of Cadaveric Pituitary Extracted Human Growth Hormone (1958-1985):Biological and Clinical Aspects.
Authors: Laron Z Abstract The first patient treated with cadaveric pituitary GH (hGH) was reported in 1958. Subsequently, collection of cadaveric pituitaries started in many countries and several centers extracted the hormone using one of two methods: a. Acetone preservation and extraction with hot glacial acetic acid (Rabin method) b. Collection in distilled water, freezing and extraction on columns yielding several pituitary hormones including hGH (Wilhelmi method). The purified extracts of hGH were found to have metabolic and growth stimulating activity but the limited amounts permitted the treatment only of chi...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Gene Sequence and Production of Recombinant MetGH/hGH.
Authors: Parks JS Abstract Recombinant human growth hormones were the products of a revolution in biotechnology that took place in the San Francisco Bay area of California in the 1970's. A combination of Herb Boyer's restriction enzymes with Stanley Cohen's bacterial plasmids provided the power to select and amplify virtually any gene. The complementary personalities and talents of Herb Boyer and Robert Swanson led to formation of Genentech and with it the development of a product that overcame the limitations of scarcity and the risks of slow virus contamination inherent in extracted pituitary growth hormone. The ...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Standardization of Growth Hormone and Insulin-like Growth Factor-I Measurement.
Authors: Katsumata N Abstract Determination of serum levels of GH and IGF-I is crucial for the diagnosis and treatment of GH deficiency and disorders related to GH excess such as acromegaly and pituitary gigantism. However, significant discrepancies in measured GH values among the methods were observed around the world. In Japan, the Study Committee for GH and Its Related Factors of The Foundation for Growth Science standardized GH values measured with various commercially available GH assay kits by creating formulas to adjust them to their averages. The committee also established reference values for IGF-I in Japa...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Regulatory Role for Growth Hormone in Statural Growth: IGF-Dependent and IGF-Independent Effects on Growth Plate Chondrogenesis and Longitudinal Bone Growth.
Authors: De Luca F Abstract It was initially thought that the growth-promoting effects of GH were exclusively mediated by liver-derived Insulin-like Growth Factor-I (IGF-I). Subsequent studies demonstrated that GH promotes IGF-I synthesis and activity in other organs and in the growth plate. GH has also IGF-I-independent growth-promoting effects. In Igf1 null mice, high circulating GH levels may be responsible for normal chondrocyte proliferation. Furthermore, tibial growth is reduced more in Ghr null mice than in Igf1 null mice, while the body of mice lacking both Ghr and Igf1 is smaller than that of mice lacking ...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Genetic Mutations in the GH/IGF Axis.
Authors: Domené S, Domené HM Abstract The GH/IGF axis plays an important role in the control of pre and postnatal growth. At least 48 monogenic defects have been described affecting the production, secretion, and action of GH and IGFs. Molecular defects of the GH/IGF axis resulting in short stature were arbitrarily classified into 4 groups: 1. Combined pituitary hormone deficiency (CPHD) (a. syndromic CPHD and b. non-syndromic CPHD), 2. Isolated GH deficiency (IGHD), 3. GH insensitivity, and 4. IGF-I insensitivity. Genetic diagnosis is obtained in about 30-40% of children with growth retardation, seve...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Pediatric Growth Hormone Deficiency (GHD) in the Recombinant Human GH (rhGH) Era.
Authors: Ranke MB Abstract During the phase of using hGH extracted from pituitaries (pit hGH) - 1958-1985 - fundamental experience related to the diagnosis and treatment was accumulated. However, since recombinant hGH (rhGH) had become available diagnosis and treatment of GHD were conducted world-wide in a more standardized way. Treatment with rhGH was also accompanied by documentations in large international pharmaco-epidemiological surveys, which provided new insight. Despite of this development the treatment of children and adolescents with GHD raises still issues related to the most effective and efficacious as...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Adult Growth Hormone Deficiency: from Transition to Senescence.
Authors: Jørgensen JO, Hermansen K, Stochholm K, Juul A Abstract The acute metabolic actions of hGH were discovered in GH-deficient adults (GHDA) 60 years ago and placebo controlled trials of prolonged rhGH replacement therapy appeared 30 years after. Untreated GHDA causes excess morbidity and mortality from cardiovascular disease and the clinical features include fatigue, reduced aerobic exercise capacity, abdominal obesity, reduced lean body mass, osteopenia, and elevated levels of circulating cardiovascular risk biomarkers. Several of these abnormalities normalize with GH replacement. Frequent side effect...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Therapy for Turner Syndrome.
Authors: Blunden C, Nasomyont N, Backeljauw P Abstract Growth failure is nearly universal in individuals with Turner syndrome (TS). It is a consequence of haploinsufficiency of the short stature homeobox gene located on the short arm of the X chromosome (SHOX). Without treatment, individuals with TS are expected to be on average 20 cm shorter than unaffected adult females. Short stature is cited by patients as one of their biggest burdens and may have an adverse impact on psychosocial well-being, pubertal timing, and ability to complete a variety of daily living activities. The routine use of recombinant human grow...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Treatment for Prader-Willi Syndrome.
Authors: Tauber M, Diene G, Molinas C Abstract The European Marketing Authorization for recombinant human growth hormone (rhGH) in children with Prader-Willi syndrome was the first indication for metabolic and body composition effects in children. In the US it is indicated for short stature associated with PWS. Recombinant hGH is the first treatment for the PWS population and radically changed the care of these children by facilitating access to physicians who prescribe rhGH, mainly paediatric endocrinologists, and manage the organization of multidisciplinary care. Recombinant hGH treatment improved linear growth, ...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Treatment for Patients with Noonan Syndrome.
Authors: Gaete X, Rodríguez F, Cassorla F Abstract Noonan syndrome (NS) is a genetic disorder, which can present clinically with a variable phenotype. Proportional post natal short stature is a common manifestation of NS, with the majority of affected patients having an adult height below the third percentile. Some investigators have reported minor abnormalities in GH secretion and/or action, suggesting that recombinant growth hormone (rhGH) therapy may be useful for the treatment of their short stature. Our review of the literature regarding rhGH therapy in children with NS indicates that this therapy impro...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Treatment for Short Children Born Small for Gestational Age.
Authors: de Andre Cardoso-Demartini A, Malaquias AC, da Silva Boguszewski MC Abstract Despite the difficulty to define born small for gestational age (SGA), being SGA has been associated with a higher risk of short stature, early-onset and rapid progression of puberty, neurocognitive dysfunctions, alterations in body composition, bone density, glucose and lipid metabolism and increased risk for cardiovascular diseases later in life. The majority of children born SGA experience spontaneous catch-up growth during the first years of life. For those who remain with short stature, treatment with recombinant human growth...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Treatment for Idiopathic Short Stature.
Authors: Cutfield WS, Albert BB Abstract ISS is the commonest cause of short stature and poor growth and is arbitrarily defined as a height
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Growth Hormone Treatment for Achondroplasia.
Authors: Yorifuji T, Higuchi S, Kawakita R Abstract Achondroplasia (ACH) is the most common form of skeletal dysplasia causing rhizomelic, short-limb short stature. Short- and long-term clinical trials have been conducted with rhGH, with similar results across these studies. At supraphysiological dose of GH, height gain of 1-1.5 SDS on the population curve was observed during the first 1-3 years, which was then followed by a smaller increase in growth rate persisting for 5-6 years. These studies led to the approval of rhGH for ACH in Japan where rhGH has been used for 20 years at 0.05 mg/kg/day. Although the availa...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Psychosocial Aspects of Short Stature and rhGH Treatment: Implicit Trends over 60+ Years.
Authors: Gardner M, Scerbak T, Sandberg DE Abstract Between 1958 and today, advances in research and the clinical management of short stature with GH have occurred. Initially, limited supply of pituitary-derived hGH led to strict criteria for diagnosing GH deficiency and tightly controlled treatment protocols. With the advent of biosynthetic GH, the supply has increased, the number of indications for treatment has grown, and the focus of intervention changed from hormone replacement to treatment of short stature. Improved psychosocial adaptation is an underlying, albeit largely unspoken and inadequately researched,...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

rhGH Abuse for Sports Performance.
Authors: Rogol AD Abstract Doping is at least as old as the ancient Olympics. Substances taken to improve athletic performance ranged from stimulants to hallucinogenic plant substances, but more recently include anabolic agents. Recombinant human growth hormone, rhGH, is one agent with a relatively short history of use, but few data to unequivocally show that it actually improves performance. However, rhGH has therapeutic use for those GH deficient and the concept of a therapeutic use exemption for those with documented deficiency is outlined along with doping control methods. The athlete's biological passport, a d...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Monitoring rhGH Safety: rhGH Registries, SAGhE and Future Needs.
Authors: Miller BS, Rosenfeld RG Abstract The safety of growth hormone (GH) therapy in children has been studied extensively. The identification of Creutzfeldt-Jacob disease in individuals who received pituitary-derived GH led to heightened surveillance for safety issues related to recombinant human GH (rhGH). An excellent safety profile of rhGH has been demonstrated in large Phase IV registries comprising> 600,000 patient-years of rhGH exposure and long-term safety cohorts of adults treated with GH as children. Increased mortality risk has been reported but eliminated when corrected for small size at birth. Inc...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Long-Acting Growth Hormone Preparations in the Treatment of Children.
Authors: Lal RA, Hoffman AR Abstract Human growth hormone (hGH), which had been in use since 1958, was supplanted by recombinant human growth hormone (rhGH) in 1985 for those with growth hormone deficiency (GHD). Adherence to daily subcutaneous growth hormone is challenging for patients. Thus, several companies have pursued the creation of long acting rhGH. These agents can be divided broadly into depot formulations, PEGylated formulations, pro-drug formulations, non-covalent albumin binding GH and GH fusion proteins. Nutropin Depot is the only long acting rhGH ever approved by the U.S. Food and Drug Administration...
Source: Pediatric Endocrinology Reviews - November 1, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: The Significance of Etiologic Diagnosis in Neonates with Overgrowth Syndromes. Lesson Learned from the Simpson-Golabi-Behmel Syndrome.
Authors: Plachý L, Elblová L, Neuman V, Fencl F, Bláhová K, Straňák Z, Lebl J, Průhová Š Abstract Overgrowth syndromes are rare genetic disorders characterized by excessive pre- and postnatal growth accompanied by dysmorphic features and developmental disorders. In addition to other health hazards, the life expectancy of affected children may be compromised due to an increased risk of developing tumors. To demonstrate the need for early recognition, correct diagnostic evaluation and adequate follow-up, we present a family with recurrent Simpson-Golabi-Behmel syndro...
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Is There a Difference between Ultrasonographic (US) Uterine Changes of Oral Versus Transdermal (TD) 17 β Estradiol (17β E2) in Girls with Turner Syndrome (TS)? Own Experience and Literature Review.
CONCLUSIONS: According to our experience, in a group of TS patients randomized to oral vs TD 17β E2 and monitored with trans-abdominal US, both groups achieved similar increases in uterine size comparable to normal women. To confirm our observation a larger sample and a longer evaluation period is needed. PMID: 30371036 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Challenges in Prenatal Treatment with Dexamethasone.
Authors: McCann-Crosby B, Placencia FX, Adeyemi-Fowode O, Dietrich J, Franciskovich R, Gunn S, Axelrad M, Tu D, Mann D, Karaviti L, Sutton VR Abstract Classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency causes elevated androgen levels, which can lead to virilization of female external genitalia. Prenatal dexamethasone treatment has been shown to be effective in preventing virilization of external genitalia when started prior to 7-9 weeks of gestation in females with classic CAH. However, CAH cannot be diagnosed prenatally until the end of the first trimester. Treating pregnant women with a...
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

The Management of Permanent Primary Hypoparathyroidism in Children and Adolescents: A Complex Task.
Authors: De Sanctis V, Soliman AT, Di Maio S, Kattamis C Abstract Management of hypoparathyroidism (hypoPT), depends on the etiology and the severity of hypocalcemia. Treatment goals include control of hypocalcemic symptoms preserving serum calcium in the low-normal range and phosphate in the high normal range. While correction of serum calcium to low-normal range does not fully correct mineral and bone metabolism it may be associated with increased risk of complications such as nephrolithiasis, nephrocalcinosis and soft tissue calcifications. Therefore, it is imperative to find out ways to individualize treatment ...
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Clinical Perspectives of Mitochondrial Disorders.
Authors: Finsterer J Abstract Mitochondrial disorders are increasingly recognised world-wide and represent a diagnostic and therapeutic challenge. This is due to the peculiarities of mitochondrial genetics and the extreme genotypic and phenotypic heterogeneity of these disorders. Traditional time-consuming and expensive diagnostic steps are increasingly replaced by first-line genetic approaches. Despite recent advances in the treatment and prevention of mitochondrial disorders, therapeutic approaches are still limited mainly to non-invasive or invasive symptomatic measures. PMID: 30371039 [PubMed - in process] ...
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: 2018 Annual Meeting of the Endocrine Society, Chicago IL (March 17-20, 2018), Selected Highlights.
Authors: Vidmar A, Ali SA, Chao L Abstract PMID: 30371040 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: The 51st Annual Meeting of the Japanese Society for Pediatric Endocrinology (JSPE), Osaka, Japan, September 28th-30th, 2017.
Authors: Urakami T Abstract PMID: 30371041 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - October 31, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: Personalized Health Care: As Exemplified by Home Sodium Measurements in a Child with Central Diabetes Insipidus and Impaired Thirst Perception.
CONCLUSION: This case is an example of personalized health care and has led to better self-reliance and quality of life. PMID: 29806747 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Review of Current Care Models for Transgender Youth and Application to the Development of a Multidisciplinary Clinic - The Seattle Children's Hospital Experience.
We describe our experience at Seattle Children's Hospital in the development of a multidisciplinary Gender Clinic which incorporates the expertise of social work, mental health professionals, pediatric endocrinology, adolescent medicine, and bioethics. Other institutions may build from our experience, with the ultimate goal of further decreasing health disparities for young transgender patients. PMID: 29806748 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

The Effects of Diuretics on Mineral and Bone Metabolism.
Authors: Alon US Abstract The effects of diuretics on water and electrolyte metabolism are well-established, but less known to the clinician are their effects on bone and mineral metabolism, and in particular on that of calcium homeostasis. In general, and clinically most relevant, diuretics acting at the thick ascending limb of the loop of Henle cause loss of calcium into the urine, thus making them a useful tool in treating hypercalcemia. However the hypercalciuria caused by loop diuretics may lead to the development of urolithiasis and nephrocalcinosis, as well as secondary hyperparathyroidism and bone disease. ...
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Gonadotropin-Releasing Hormone (GnRHa) Therapy for Central Precocious Puberty (CPP): Review of Nuances in Assessment of Height, Hormonal Suppression, Psychosocial Issues, and Weight Gain, with Patient Examples.
Authors: Klein KO, Lee PA Abstract This review suggests a central theme: that the treatment of each patient presenting with evidence consistent with central precocious puberty (CPP) needs to be individualized. This pertains to multiple factors relating to growth and growth potential, monitoring patients on treatment with gonadotropin-releasing hormone analogue (GnRHa), evaluating psychological issues with CPP and therapy, and concerns about weight gain during GnRHa therapy. Individual cases are presented. New data on adult height and rate of bone age advance are included. GnRHa treatment is effective in improving a...
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Thyroid Dimensions Using Handheld Point-of-Care (bedside) Ultrasound Scan of the Thyroid Gland in Neonates in Port Harcourt and a Review of Literature.
CONCLUSION: Handheld point-of-care (bedside) US is useful in determining thyroid gland dimensions in newborn babies and the volume measurements obtained thus far are comparable to those from Glasgow. PMID: 29806751 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Meeting Report: Growth and Social Environment. Proceedings of the 25th Aschauer Soiree, held at Krobielowice, Poland, November 18th 2017.
Authors: Kozie S, Scheffler C, Tutkuviene J, Jakimaviciene EM, Mumm R, Barbieri D, Godina E, El-Shabrawi M, Elhusseini M, Musalek M, Pruszkowska-Przybylska P, El Dash HH, Safar HH, Lehmann A, Swanson J, Bogin B, Liu YC, Groth D, Kirchengast S, Siniarska A, Nieczuja-Dwojacka J, Králík M, Satake T, Hanć T, Roelants M, Hermanussen M Abstract Twenty-two scientists met at Krobielowice, Poland, to discuss the impact of the social environment, spatial proximity, migration, poverty, but also psychological factors such as body perception and satisfaction, and social stressors such as elite sports, and teenage...
Source: Pediatric Endocrinology Reviews - May 30, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

In memoriam: Teruo Kitagawa, MD (1926-2017).
Authors: Urakami T PMID: 29493123 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - March 3, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: Combination Growth Hormone and Insulin-Like Growth Factor-I Therapy for Childhood Growth Disorders: Prime Time or Too Much Dime?
Authors: Geffner ME Abstract Although metabolic outcomes may be aided by dual therapy with GI and IGF-I, the one published study of the combination approach to treat children with non-GH-deficient short stature showed only a meager additional height response compared to that achieved with GH alone. PMID: 29493124 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - March 3, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Genetics of Primary Congenital Hypothyroidism.
Authors: Zwaveling-Soonawala N, van Trotsenburg P Abstract Congenital hypothyroidism (CH) is one of the most common preventable forms of mental retardation and since the implementation of neonatal screening programs in the mid-1970s, early detection and treatment have proven to be very successful in preventing brain damage. CH may be of thyroidal (= primary) or of hypothalamic-pituitary (= central) origin. Primary CH may be due to abnormal thyroid gland formation (dysgenesis) or defective thyroid hormone syntheses by a structurally normal gland (dyshormonogenesis). While thyroid dysgenesis is the most common form o...
Source: Pediatric Endocrinology Reviews - March 3, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Prolactin - Not Only a "Milk Hormone" Prolactin - Growth Hormone Relationships with Emphasis on Cancer.
Prolactin - Not Only a "Milk Hormone" Prolactin - Growth Hormone Relationships with Emphasis on Cancer. Pediatr Endocrinol Rev. 2018 Mar;15(3):216-222 Authors: Farfel A, Werner H, Laron Z Abstract Prolactin (PRL) is a hormone secreted by lactotrophic cells in the anterior pituitary gland and its main function is the stimulation of lactogenesis. Research in recent years has revealed that PRL is also related to cancer development and plays a role in autoimmune diseases. PRL and Growth Hormone (GH) belong to the same cytokine family, both are, at least in part, secreted by the same somatomammotr...
Source: Pediatric Endocrinology Reviews - March 3, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Options for Fertility Preservation in Children.
Authors: Javed A, Khan Z, Pittock ST, Jensen JR Abstract Fertility preservation therapies can conserve future reproductive potential for persons facing serious medical diagnoses. With cure rates for childhood cancer reaching almost 80%, quality-of-life concerns for long-term survivors, including future parenting, are becoming more pertinent. Late effects of childhood cancer can be divided into physical, social, psychological, and spiritual domains. Potential loss of fertility threatens the well-being of these children in all these domains. Providers often hesitate to discuss fertility preservation with the patients...
Source: Pediatric Endocrinology Reviews - March 3, 2018 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research