Meeting Report: Updates in Diagnosis and Management of Hyperinsulinism and Neonatal Hypoglycemia: Highlights from the Fourth International Hyperinsulinism Symposium.
Authors: Lord K, De León DD Abstract Hypoglycemia remains a significant cause of morbidity in infants and children. Up to 50% of children with hypoglycemic disorders suffer from neurodevelopmental deficits, as a consequence of delays in the diagnosis and inadequate treatment. Recent advances in the field have resulted in new therapies and improved outcomes. To review these advances and have a dialogue regarding controversies in the field, the Fourth International Hyperinsulinism Symposium, sponsored by the Children's Hospital of Philadelphia was held in Philadelphia, Pennsylvania on September 5-6, 2019. T...
Source: Pediatric Endocrinology Reviews - August 4, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

The Discovery and Structure of Human Insulin.
Authors: Zimmerman C, Forlenza G, Schatz D Abstract The isolation and purification of insulin nearly 100 years ago has been one of the most ground-breaking discoveries in the history of medicine. Subsequent determination of the specific structure of human insulin has permitted further developments and modifications of the formulations of insulin to allow improved quality of care and quality of life for patients with diabetes. In this paper, we will review insulin structure and biosynthesis, treatment and prognosis of type 1 diabetes prior to insulin therapy, experimentation leading to the discovery of insu...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Eli Lilly and Company Insulins - A Century of Innovation.
Authors: Malone JK, Anderson JHJ, Wolpert HA, Ilag LL, Frank BH, De Felippis MR, Paavola CD, Orr AL, Beals JM Abstract Eli Lilly and Company has played a pivotal role in the development of insulin products since its discovery in 1921. Through their dedication to pharmaceutical innovation, Josiah K. Lilly Sr. and George HA Clowes, in close collaborations with the University of Toronto, made insulin commercially available in 1923. Other innovations include the development and commercialization of the first biosynthetic human insulin, a rapid-acting insulin analog and analog mixtures. Lilly has advanced the f...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Nearly a Century of Insulin at Sanofi: Looking Back Over the Decades of Production and Development.
Authors: Bosnyak Z, Korn M, Bielohuby M, Wohlfart P, Tennagels N Abstract Almost a century ago, the first insulin was produced by Banting, Best, MacLeod and Collip in Toronto, thereby enabling life-saving treatment for people with diabetes. Since then, there have been many advancements in insulin production and development of new insulin analogues. In this article, we reflect on the rich heritage of Sanofi and its predecessor, Hoechst, in insulin production and development, from being one of the first companies to produce insulin in Europe in 1923, to modern-day insulin analogues and integrated care soluti...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Decision Support Systems for Insulin Treatment Adjustment in People with Type 1 Diabetes.
Authors: Nimri R Abstract For people with type 1 diabetes, achieving optimal glycemic control requires use of intensive insulin therapy. To achieve this goal individuals are required to become proficient in accurately determining the appropriate amount of insulin needed to address a variety of situations throughout the day while considering numerous influencing factors. They also need to perform multiple tasks a day such as counting carbohydrates to accurately determine the required premeal bolus. There is also a need to periodically adjust insulin dosing as insulin sensitivity varies considerably over tim...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Insulin Adverse Events.
Authors: Ludvigsson J Abstract The negative consequencies of diabetes treatment are traditionally regarded as caused by a disastrous treatment rather than adverse events of the insulin preparations. However, hypoglycemia, changes at the injection site (lipatrophy, lipoma), insulin allergy, obesity and increased risk of certain forms of cancer can easily be regarded as adverse events of the drug, and needle-phobia, psychological problems, increased risk of suicide are adverse events related to insulin and its administration. Also macroangiopathy and even microangiopathy to some extent can be regarded as adv...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Insulin: A Growth Hormone and Potential Oncogene.
Authors: Laron Z, Werner H Abstract Both in vitro and in vivo experimental studies proved that insulin has an important anabolic role. This physiological function of insulin is reflected in its well documented involvement in protein metabolism and in acceleration of cell proliferation. Support for a growth promoting action of insulin is further provided by clinical studies that revealed that children with hypoinsulinemia have a decreased growth rate whereas, on the other hand, children with hyperinsulinemia have an accelerated growth. While it was initially assumed that the growth activities of insulin are...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Update on Worldwide Trends in Occurrence of Childhood Type 1 Diabetes in 2020.
Authors: Tuomilehto J, Ogle GD, Lund-Blix NA, Stene LC Abstract Epidemiological data on pediatric type 1 diabetes (T1D), mainly incidence, have become increasingly available since the second half of the 20th century. Comparative incidence data across populations were only obtained since the 1980s. The 2019 IDF Atlas provides T1D incidence, prevalence and mortality estimates for children < 15 years for all 211 countries, but actual data were available for only 94 countries (only 3 low-income). The estimated prevalent cases were 600,900 and incident cases 98,200. Incidence remains highest in Finland (60/1...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Global Inequality in Type 1 Diabetes: a Comparison of Switzerland and Low-and Middle-Income Countries.
Authors: Marque NA, Lazo-Porras M, Schwitzgebel V, Castellsague M, Cimarelli G, Dirlewanger M, Klee P, Perrenoud L, Beran D Abstract Globally it is estimated that over 1 million children and adolescents have Type 1 diabetes with large variations in incidence between different contexts. Health systems need to provide a variety of elements to ensure appropriate diabetes care, such as service delivery; healthcare workforce; information; medical products and technologies; financing and leadership and governance. Describing these elements between Geneva, Switzerland, a high-income country with high spending on ...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

History of Insulin Treatment in Children and Adolescents with Diabetes in Japan.
Authors: Urakami T Abstract For the increasing number of type 1 diabetes in Japan, after 1960, a greater supply of insulin was required, accordingly the availability of insulin gradually improved, The National Health Insurance approved self-injections of insulin at home in 1981. Afterwards, intensive insulin treatment with short-acting insulin and intermediate-acting insulin became widely used. Recombinant rapid-acting insulin analog was introduced in 1986 and long-acting insulin analog was introduced in 2003. In recent years, basal-bolus insulin regimens using these insulin analogs have become popular in ...
Source: Pediatric Endocrinology Reviews - March 27, 2020 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

For Debate: Programing of the Autoimmune Diabetogenic Response in the Thymus during Fetal and Perinatal Life.
Authors: Geenen V, Hober D PMID: 31763800 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - November 27, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Osteoclasts in Health and Disease.
Authors: Lerner UH Abstract Osteoclasts are multinucleated, giant cells originating from myeloid hematopoetic stem cells. These are the only cells in nature which can resorb bone. Differentiation of mononucleated osteoclast progenitor cells requires stimulation with M-CSF (macrophage colony-stimulating factor) for the cells to proliferate and survive and with RANKL (receptor activator of nuclear factor kappa B ligand) for differentiation along the osteoclastic lineage to cells which eventually fuse to the mature, multinucleated osteoclasts. Therefore, most hormones and cytokines stimulating osteoclastogene...
Source: Pediatric Endocrinology Reviews - November 27, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Pediatric Cushing Syndrome; an Overview.
Authors: Tatsi C, Stratakis CA Abstract Endogenous Cushing syndrome (CS) is a rare entity in the pediatric population. Patients usually present with height deceleration and continuous weight gain, along with other classic stigmata of CS, such as acne, striae, hirsutism and others. The diagnosis of CS involves documentation of abnormal cortisol secretion with measurement of urinary free cortisol and midnight serum or salivary cortisol, and the use of the 1mg dexamethasone suppression test. After confirmation of CS, localization of the source of hypercortisolemia, involves differentiation of ACTH-dependent v...
Source: Pediatric Endocrinology Reviews - November 27, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Endocrine Management of Ovotesticular DSD, an Index Case and Review of the Literature.
Authors: Kilberg MJ, McLoughlin M, Pyle LC, Vogiatzi MG Abstract Ovotesticular Differences in Sexual Development (OT-DSD) is a rare subset of DSD with great phenotypic variability characterized by the presence of both testicular and ovarian tissue in the same individual. Here, we describe the case of 46,XX, SRY-negative baby with ambiguous genitalia and ovotestis discovered during laparoscopy. As the family decided on female gender of rearing, the testicular component of the ovotestis was removed while the ovarian component was preserved. Stemming from this case, we review the clinical presentation of OT-D...
Source: Pediatric Endocrinology Reviews - November 27, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research

Primary School Performance of Girls with Turner Syndrome: A Transcultural Assessment.
CONCLUSION: Excluding results in Mathematics, which showed pronounced worsening in relation to age in comparison with unaffected controls, girls with TS performed similarly to their controls. PMID: 31763804 [PubMed - in process] (Source: Pediatric Endocrinology Reviews)
Source: Pediatric Endocrinology Reviews - November 27, 2019 Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research