There appears to be an increased risk of developing diabetes for persons with Turner syndrome. The exact reasons for this are enigmatic, as there have been very few studies. This lack of knowledge makes it difficult to know how best to screen for and help prevent diabetes in this population. To help address this, pediatric endocrine fellow Dr. Pinnaro has initiated a study of blood levels levels in persons with Turner syndrome who do not have diabetes. The initial results from this study suggest a greater degree of atypical glucose levels in those with Turner syndrome as compared to controls. This past weekend, Dr. Pinnaro would have presented these initial results at the national Pediatric Endocrine Society meeting in Texas (cancelled due to COVID-19). Her faculty mentors for this study were Drs. Katie Larson Ode and Andrew Norris.
We most commonly associate thyroid eye disease as occurring in the context of Graves disease. However, the same immunologic processes that drive Graves thyroid eye disease can also occur in the context of hypothyroidism. Dr. Alex Tuttle woudl have just presented such a case this weekend at the annual Pediatric Endocrine Society meeting originally planned to occur in Texas. His presentation was entitled: “Active Thyroid Eye Disease in a Pediatric Patient with Hypothyroidism”. This serves as a reminder that it is important for even otherwise routine cases of hypothyroidism to receive expert care. Dr. Tuttle is completing his pediatric residency at the University of Iowa this year. We are thrilled that on July 1 he will join our division as a pediatric endocrine fellow. On this case report, he was mentored by pediatric thyroid expert Dr. Liuska Pesce.
Dr. Katie Larson Ode has been named director of the Clinical Core component of the University of Iowa’s Center for Gene Therapy of Cystic Fibrosis. The NIH grant funding for this project was just renewed. Her core will support maintenance and growth of a Cystic Fibrosis biobank which will obtain/provide clinical samples from/to researchers in cystic fibrosis for translational projects. Congratulations to Dr. Larson Ode on your excellent work.
By invitation, Dr. Lauren Kanner has been installed as the Workshops Co-Chair on the Program Committee for the North American Society for Pediatric and Adolescent Gynecology (NASPAG) 2021 Annual Clinical & Research Meeting. In this role, she will help plan and set the agenda for the workshops to occur at this national meeting. She also is now the pediatric endocrine liason for the Fellow Research Consortium of NASPAG. She remains a member of the NASPAG education committee, a role she has maintained since April 2018. Thanks to Dr. Kanner for her hard work in this important area.
As the COVID-19 pandemic began impacting the region in March 2020, it quickly became apparent that the pandemic would impact our Division of Pediatric Endocrinology & Diabetes ability to provide healthcare and would adversely impact many of the children and families for whom we provide care. In response to this arising situation, the Leona M. And Harry B. Helmsley Charitable Trust issued a call for grant proposals aimed at providing local solutions relating to type 1 diabetes care. Dr. Tansey answered this call, writing a proposal to aid with delivery of healthcare for those with type 1 diabetes cared for by our clinic. The goal of the Helmsley program is to “improve the lives of all people living with type 1 diabetes (T1D). Working closely with key players across the T1D ecosystem – patients, physicians, caregivers, researchers, government agencies, funders, pharmaceutical companies, device makers, insurers, and community organizations – we seek to improve care and ultimately prevent the disease.” I am pleased to announce that the proposal created by Dr. Tansey has been approved and funded, as of today. The funds will help our team provide services to our patients with type 1 diabetes, through improved telemedicine education opportunities, and will help provide services to those whom have been directly affected by COVID-19. My deepest gratitude to Dr. Tansey for taking the initiative and rapidly helping answer the challenges imposed by the COVID-19 pandemic.
Andrew Norris, MD PhD
Director, Pediatric Endocrinology & Diabetes
University of Iowa Stead Family Children’s Hospital
I am excited to report that the Iowa newborn screening program will begin using age-adjusted TSH cut-offs starting March 30, 2020. The purpose is to better screen newborns for congenital hypothyroidism, which is a condition that if untreated leads to profound deficiencies in brain development and body growth. The cutoff changes are expected to improve both the specificity and sensitivity of the screen. Otherwise, in general, there will be no change in workflow as involves endocrinologists and primary care providers. Only the cutoffs are changing. Despite these improvements, the newborn screen remains only a screening test and should not be used as a quantitative test to examine an infant in whom you suspect a thyroid abnormality.
“with the advent of techniques to strengthen brain regions, such as transcranial magnetic stimulation, might this type of research help guide possible interventions?” –Andrew Norris
Dr. Tsalikian and collaborators across the country have been studying brain cognitive function in children with and without type 1 diabetes. In a study just published in PLOS Medicine, they report interesting differences. They used functional magnetic imaging resonance (fMRI) to measure activation in various brain locations while the children were given tasks. Compared to children without diabetes, those with type 1 diabetes exhibited two differences. One of the observed changes was impaired control of a region towards the back of the brain and this impairment typically leads to diminished task performance. In contrast, there was enhanced activation of a region towards the front of the brain involved in executive control. It appears that these two changes balanced each other, in that the two groups had similar task performance. Simply put, it appears that the brains of children with type 1 diabetes are able to compensate for impairments presumably induced by long-term exposure to high blood sugars. More study is needed to understand this latter point in particular, for example would the pattern normalize if the blood sugars were held to the normal range during the study? Furthermore, more study is needed to understand the broader implications of this work, for example might these or related changes contribute to the increased risk of depression in persons with diabetes? Finally, with the advent of techniques to strengthen brain regions, such as transcranial magnetic stimulation, might this type of research help delineate important interventions? Also involved in the study from our Division were Dr. Tansey, Julie Coffey, Joanne Cabbage, Sara Salamati, and Rachel Bisbee.
Dr. Pinnaro, pediatric endocrine fellow, has published her recent research that finds several candidate genes which may modify the phenotype of 22q11.2 syndrome. This genetic syndrome can cause congenital structural heart disease, failure of the parathyroid glands to properly form, and immunodeficiency. Although the effects of the syndrome vary from person to person, the reasons for this variability is unknown. Thus, Dr. Pinnaro set out to understand whether other genetic regions might be the reason. Her findings show that various genes might indeed contribute. She is the lead author on the work which is published in the January 2020 issue of Molecular Genetics & Genomic Medicine. Congratulations to Dr. Pinnaro for the results of her hard work in this area.
Children, adolescents, and young adults with neuromuscular disorders are at risk for various endocrine complications. For example, Duchenne muscular dystrophy is associated with risk of various endocrine conditions including poor bone health, adrenal insufficiency, obesity, pubertal delay, and short stature. To help with these issues, in 2019, Dr. Vanessa Curtis joined the multidisciplinary Neuromuscular team at the University of Iowa to provide endocrine care to patients in this clinic. This week, Dr. Curtis attended the Parent Project MD meeting in San Diego to further her skills in this emerging area of medicine.
We are pleased to report that Dr. Liuska Pesce, one of our pediatric endocrine faculty, has been selected to co-chair & then chair the Pediatric Endocrinology Self-assessment Program for the Endocrine Society in 2020 & 2021. As part of this group, she helped publish Pediatric ESAP 2019-2020, ESAP Special Edition: Historical Perspectives for Today’s Clinician, and Pediatric ESAP 2017- 2018.