Medication-induced hyperglycemia is a frequently encountered clinical problem in children. The intent of this review of medications that cause hyperglycemia and their mechanisms of action is to help guide clinicians in prevention, screening and management of pediatric drug-induced hyperglycemia. We conducted a thorough literature review in PubMed and Cochrane libraries from inception to July 2019. Although many pharmacotherapies that have been associated with hyperglycemia in adults are also used in children, pediatric-specific data on medication-induced hyperglycemia are scarce. The mechanisms of hyperglycemia may involve β cell destruction, decreased insulin secretion and/or sensitivity, and excessive glucose influx. While some medications (eg, glucocorticoids, L-asparaginase, tacrolimus) are markedly associated with high risk of hyperglycemia, the association is less clear in others (eg, clonidine, hormonal contraceptives, amiodarone). In addition to the drug and its dose, patient characteristics, such as obesity or family history of diabetes, affect a child’s risk of developing hyperglycemia. Identification of pediatric patients with increased risk of developing hyperglycemia, creating strategies for risk reduction, and treating hyperglycemia in a timely manner may improve patient outcomes.
Background. McCune-Albright syndrome (MAS) is characterized by hyperpigmented macules, endocrinopathies, and fibrous dysplasia. Hyperthyroidism is the second most common endocrinopathy in MAS and its management is challenging, particularly among infants and toddlers. Traditionally, young infants have been treated with antithyroid medications, but remission is likely and these medications have severe side effects and affect the control of other endocrinopathies. Thus, it is reasonable to consider permanent treatment options at an earlier age. In this article, we performed a retrospective chart review and describe 3 children who underwent thyroidectomy at an early age due to complex presentation. Case Descriptions. Case 1 was a female patient who underwent bilateral adrenalectomy due to adrenal hyperplasia and subsequently underwent thyroidectomy at 5 months of age due to unremitting hyperthyroidism with fibrous dysplasia, multiple fractures, and ovarian cysts with vaginal bleeding. Case 2 was a 20-month-old female on methimazole who acquired influenza A, precipitating a thyroid storm, and subsequently developed central precocious puberty. Case 3 was a 4-year-old female who underwent thyroidectomy because of unremitting hyperthyroidism after methimazole cessation due to declining neutrophils. All 3 children experienced no complications from thyroidectomy. Conclusions. Early thyroidectomy by an experienced surgeon is an option for managing MAS-associated hyperthyroidism, even in very young patients, with excellent results.
The birth of a child with a disorder of sexual development (DSD) is challenging from multiple perspectives, particularly that of sex assignment. Patients, parents, and health care professionals who care for patients with DSDs understand that the physical, biochemical, and mental characteristics of these patients better fit a model of fluidity. Yet, sex continues to be predominantly binary, and the decision that families make early on often has medical, social, and legal implications. Until recently, all US birth certificates listed either male or female sex. However, in December 2016, the state of New York issued the nation's first intersex birth certificate to a 55-year-old individual who was born with a DSD consisting of male chromosomes, female external genitalia, and mixed internal genitalia. 1 This legal landmark challenges the traditional legal framework of the binary division of sex. Therefore, we are taking this opportunity to discuss the fluidity of sex assignment and how this unprecedented event may provide an opportunity to improve the care of patients with a DSD. Historical PersPective Multiple terms and classification systems have been used to describe patients with DSDs. The classification systems were initially based on anatomy and were modified over time as the chromosomal basis of sex, gonadal characteristics, and sexual differentiation were better understood. However, the subclassifications had the qualifiers of male or female, implying the sex to be assigned. 2 It was not until 2006 that the Pediatric Endocrine Society and the European Society of Paediatric Endocrinology revised the nomenclature into a classification system in which the diagnosis did not determine gender assignment, acknowledging that gender is complex and not determined by a single criterion. The resulting international Consensus Statement on the Management of Intersex Disorders, also known as the Chicago Consensus Statement, recommended the use of the term DSD to describe any condition in which sex-related chromosomes, molecules, and/or internal organs do not develop in a way that could be classified as male or female. It also recommended against the use of terms such as male/female, hermaphrodite, and pseudohermaphrodite because these terms can be confusing and may carry negative connotations (Table 1). 3, 4
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