Bariatric surgery has been effective in treating type 2 diabetes mellitus (T2DM); it has not been used frequently in obese patients with type 1 diabetes mellitus (T1DM). This is the first case series reporting on the effect of bariatric surgery on diabetes control in adolescents with T1DM. Patient A is a 19-year-old obese man with T1DM who underwent vertical sleeve gastrectomy. At 12 months after surgery he demonstrated 28% reduction in BMI. His daily total insulin requirement had decreased; however, hemoglobin A1c remained primarily unchanged at 8.8%. Patient B is a 13-year-old obese girl with an initial clinical diagnosis of T2DM controlled on only metformin. She underwent Roux-en-Y gastric bypass; at 1 month after surgery she presented in diabetic ketoacidosis and was found to have positive islet cell antibodies, which were also present before surgery. Her diagnosis was revised to T1DM, and she was started on insulin. By 28 months after surgery her BMI had decreased by 42%. Since initiation of insulin, her daily total insulin requirement had decreased, but hemoglobin A1c had significantly worsened from 6.3% to 10%. We found that despite significant weight loss, improvements in cardiovascular risk factors (dyslipidemia and obstructive sleep apnea), and quality of life in our patients, bariatric surgery does not necessarily lead to improved glycemic control of T1DM. Patients with T1DM have ongoing dependency on exogenous insulin, and optimal glycemic control still depends on patient compliance with diabetes care. Pediatrics 2013;132:e1031-e1034 Dr Chuang conceptualized the case series and drafted the initial manuscript; Dr Zeller interpreted the quality of life measurements used in the case series, wrote the interpretations for the manuscript, and reviewed and revised the manuscript; Dr Inge helped obtain data used in the case series and critically reviewed and revised the manuscript; Dr Crimmins help conceptualize and design the outline for the case series and critically reviewed and revised the manuscript; and all authors approved the final manuscript as submitted.

Background17β-Hydroxysteroid dehydrogenase type-3 (17βHSD-3) deficiency is a rare cause of 46,XY disorders of sex development. The enzyme converts androstenedione to testosterone, necessary for masculinization of male genitalia in utero. 17βHSD-3 deficiency is frequently diagnosed late, at puberty, following virilization, with consequent female-to-male gender reassignment in 39-64%. The decision for sex of rearing is difficult, especially if diagnosed in early childhood. Consensus guidelines are equivocal or support male gender assignment. Long-term outcomes data to guide decisions are also lacking; however, in the few cases of early diagnosis and orchiectomy, female gender retention appears more likely.We report two patients with 17βHSD-3 deficiency, who presented at unusual ages, in whom female gender was chosen. We performed a focused literature review and summary of gender outcomes in 17βHSD-3 deficiency following early orchiectomy.CasesPatient A was a phenotypic female who presented at one year of age with bilateral inguinal hernias and external female genitalia. Testes were identified at surgery. The karyotype was 46,XY. She was initially diagnosed with complete androgen insensitivity syndrome; however, androgen receptor mutation analysis was negative. Human chorionic gonadotropin stimulation yielded a low testosterone: androstenedione ratio (0.6, normal >0.8). Genetic testing demonstrated compound heterozygosity for two known mutations of the HSD17B3 gene. She underwent bilateral orchiectomy at two years of age.Patient B was born with female genitalia and virilized at 13 years of age. She did not seek evaluation until 22 years of age. Her karyotype was 46,XY. She had bilateral inguinal testes and low testosterone: androstenedione ratio (0.3). HSD17B3 gene sequencing showed her to be a compound heterozygote for two known mutations. She identified herself as female and underwent bilateral orchiectomy and estrogen replacement therapy.ConclusionsThese two patients highlight the complexities of diagnosis and management in 17βHSD-3 deficiency. Although existing data are limited, early orchiectomy is likely to result in retention of female gender identity, avoiding the complications related to virilization in adolescence. As such, it is important to pursue a definitive diagnosis to guide clinical decisions, and to have the support and long term follow up with an inter-disciplinary disorders of sex development team.

Untreated congenital hypothyroidism (CH) leads to intellectual disabilities. Prompt diagnosis by newborn screening (NBS) leading to early and adequate treatment results in grossly normal neurocognitive outcomes in adulthood. However, NBS for hypothyroidism is not yet established in all countries globally. Seventy percent of neonates worldwide do not undergo NBS. The initial treatment of CH is levothyroxine, 10 to 15 mcg/kg daily. The goals of treatment are to maintain consistent euthyroidism with normal thyroid-stimulating hormone and free thyroxine in the upper half of the age-specific reference range during the first 3 years of life. Controversy remains regarding detection of thyroid dysfunction and optimal management of special populations, including preterm or low-birth weight infants and infants with transient or mild CH, trisomy 21, or central hypothyroidism. Newborn screening alone is not sufficient to prevent adverse outcomes from CH in a pediatric population. In addition to NBS, the management of CH requires timely confirmation of the diagnosis, accurate interpretation of thyroid function testing, effective treatment, and consistent follow-up. Physicians need to consider hypothyroidism in the face of clinical symptoms, even if NBS thyroid test results are normal. When clinical symptoms and signs of hypothyroidism are present (such as large posterior fontanelle, large tongue, umbilical hernia, prolonged jaundice, constipation, lethargy, and/or hypothermia), measurement of serum thyroid-stimulating hormone and free thyroxine is indicated, regardless of NBS results.

<b><i>Introduction:</i></b> The standard workup of thyroid nodules concerning for malignancy includes fine-needle aspiration (FNA). In 2015, the American Thyroid Association (ATA) guidelines for the management of pediatric thyroid nodules recommended that all nodules with a Bethesda III cytology undergo surgical resection. <b><i>Objectives:</i></b> To correlate a Bethesda III cytology with histologic and clinical outcomes to determine the relevance of the ATA recommendations, and to evaluate whether Thyroid Imaging Reporting and Data System (TI-RADS) scoring could identify Bethesda III nodules at a lower risk of malignancy. <b><i>Methods:</i></b> A retrospective chart review of patients who had undergone thyroid nodule FNA from 2008 to 2018 was performed. Malignancy rates were determined for each Bethesda category. The reference standard was histopathology or 2-year follow-up of imaging outcomes for nonoperative cases. Ultrasound exams of Bethesda III nodules were reviewed and TI-RADS scores assigned. <b><i>Results:</i></b> A total of 143 FNA samples from 128 patients were identified. The mean age was 14.9 years (range 7–22). Twenty-two (15%) of the FNA samples were Bethesda III; the malignancy rate was 38%. A TI-RADS score was assigned in 20 of the 22 Bethesda III nodules. ROC analysis found an optimal cut-off for malignancy prediction of ≥7 points (risk category TR5). The negative predictive value was 85.7% (95% CI 35.9–99.6) and the positive predictive value was 83.3% (95% CI 57.2–98.2). <b><i>Conclusion:</i></b> Although, at baseline, thyroid nodules with a Bethesda III classification carry a moderate risk of malignancy in the pediatric population, TI-RADS scoring can identify nodules with a lower risk within this group. If validated by larger studies, this can inform decision making and reduce unneeded surgery.

Context Gastric bypass (GB) increases postprandial glucose excursion, which in turn can predispose to the late complication of hypoglycemia. Diagnosis remains challenging and requires documentation of symptoms associated with low glucose, and relief of symptom when glucose is normalized (Whipple’s triad). Objective To compare the yield of mixed meal test (MMT) and continuous glucose monitoring system (CGMS) in detecting hypoglycemia after gastric bypass surgery (GB). Setting The study was conducted at General Clinical Research Unit, Cincinnati Children’s Hospital (Cincinnati, OH, United States). Methods Glucose profiles were evaluated in 15 patients with documented recurrent clinical hypoglycemia after GB, 8 matched asymptomatic GB subjects, and 9 healthy weight-matched non-operated controls using MMT in a control setting and CGMS under free-living conditions. Results Patients with prior GB had larger glucose variability during both MMT and CGMS when compared to non-surgical controls regardless of their hypoglycemic status. Sensitivity (71 vs. 47 %) and specificity (100 vs. 88 %) of MMT in detecting hypoglycemia was superior to CGMS. Conclusions Our findings indicate that a fixed carbohydrate ingestion during MMT is a more reliable test to diagnose GB-related hypoglycemia compared to CGMS during free-living state.