Thyroid Storm Caused by Hyperemesis Gravidarum.

Background: Transient thyrotoxicosis has been documented in the setting of hyperemesis gravidarum (HG) with elevated human chorionic gonadotropin (hCG) levels. Thyroid storm in pregnancy is rarer and typically associated with autoimmune hyperthyroidism. We described thyroid storm in a primigravid 18-year-old patient due to hCG level elevation secondary to HG, which resolved in the second trimester of pregnancy. Case Report: Our patient presented with vomiting, hyperthyroidism, and cardiac and renal dysfunction at 16 weeks' gestation. She was clinically found to have a thyroid storm, with undetectable thyroid-stimulating hormone (TSH) and a free thyroxine level of >6.99 ng/dL. The hCG level was elevated at 246 030 mIU/L (9040-56 451 mIU/L). She was treated with methimazole, saturated solution potassium iodide, and propranolol. Because thyroid autoantibodies were absent, thyroid ultrasound yielded normal results, and thyroid function testing results rapidly improved as the hCG level decreased, the medications were tapered and ultimately discontinued by day 10 of hospitalization. The thyroid function remained normal after discharge. Discussion: Because hCG and TSH have identical alfa subunits and similar beta subunits, hCG can bind to the TSH receptor and stimulate thyroxine production. The hCG level peaks at around 8-14 weeks of gestation, correlating with decreased TSH levels in this same time frame. This case emphasizes the relevant physiology and importance of timely and thorough evaluation to determine the appropriate management, prognosis, and follow-up for patients with thyroid storm in the setting of HG. Conclusion: Although transient thyrotoxicosis is documented in patients with HG, thyroid storm is rare, and our case illustrates a severe example of these comorbidities.

Severity of non-alcoholic steatohepatitis is not linked to testosterone concentration in patients with type 2 diabetes.

BACKGROUND: Hypogonadism is reported to occur in non-alcoholic fatty liver disease (NAFLD), but earlier studies used low-sensitivity diagnostic techniques (CT, ultrasound), for NAFLD diagnosis. We hypothesized that if hypogonadism was due to NAFLD, and not solely attributable to underlying obesity/diabetes, it would be more severe in the presence of steatohepatitis (NASH). To examine the influence of liver disease on testosterone in males with type 2 diabetes mellitus (T2DM), we used gold-standard liver imaging with MR-spectroscopy (1H-MRS), and performed liver biopsies to grade/stage the NAFLD. METHODS: In this cross-sectional study, we measured in 175 males with T2DM total and free testosterone, markers of insulin resistance, and intrahepatic triglyceride content (IHTG) by 1H-MRS. Those with NAFLD on imaging underwent a liver biopsy. RESULTS: Total testosterone was higher in the group without NAFLD ("No-NAFLD"; n = 48) compared to isolated steatosis (IS; n = 62) or NASH (n = 65) (385 ± 116 vs. 339 ± 143 vs. 335 ± 127 ng/ml, ptrend 0.03). Testosterone was also lower in obese vs. non-obese subjects in both the No-NAFLD and IS groups (p = 0.06 and p = 0.11, respectively), but not in obese vs. non-obese patients with NASH (p = 0.81). IHTG was independently associated with total testosterone (ß = -4.8, p = 0.004). None of the liver histology characteristics were associated with lower testosterone. CONCLUSIONS: NAFLD is linked to lower total testosterone in patients with T2DM, but likely given a common soil of insulin resistance/obesity and not from the severity of liver necroinflammation or fibrosis. Nevertheless, clinicians should consider screening patients with T2DM and NAFLD for hypogonadism.

Real-World Screening for Retinopathy in Youth With Type 1 Diabetes Using a Nonmydriatic Fundus Camera.

OBJECTIVE: To assess the use of a portable retinal camera in diabetic retinopathy (DR) screening in multiple settings and the presence of associated risk factors among children, adolescents, and young adults with type 1 diabetes. DESIGN AND METHODS: Five hundred youth with type 1 diabetes of at least 1 year's duration were recruited from clinics, diabetes camp, and a diabetes conference and underwent retinal imaging using a nonmydriatic fundus camera. Retinal characterization was performed remotely by a licensed ophthalmologist. Risk factors for DR development were evaluated by a patient-reported questionnaire and medical chart review. RESULTS: Of the 500 recruited subjects aged 9-26 years (mean 14.9, SD 3.8), 10 cases of DR were identified (nine mild and one moderate nonproliferative DR) with 100% of images of gradable quality. The prevalence of DR was 2.04% (95% CI 0.78-3.29), at an average age of 20.2 years, with the youngest affected subject being 17.1 years of age. The rate of DR was higher, at 6.5%, with diabetes duration >10 years (95% CI 0.86-12.12, P = 0.0002). In subjects with DR, the average duration of diabetes was 12.1 years (SD 4.6, range 6.2-20.0), and in a subgroup of clinic-only subjects (n = 114), elevated blood pressure in the year before screening was associated with DR (P = 0.0068). CONCLUSION: This study in a large cohort of subjects with type 1 diabetes demonstrates that older adolescents and young adults (>17 years) with longer disease duration (>6 years) are at risk for DR development, and screening using a portable retinal camera is feasible in clinics and other locations. Recent elevated blood pressure was a risk factor in an analyzed subgroup.

Hypercalcemia from hypervitaminosis A in a child with autism.

Objectives Vitamin A is essential for normal cellular physiology and is often taken as a dietary supplement. Hypervitaminosis A can lead to hypercalcemia by increasing osteoclasts and subsequent bone resporption. Dietary supplements including vitamin A are new popular treatment stategies for autism. Case presentation We report a five-year old boy with autism spectrum disorder presenting with severe abdominal pain and bilateral lower extremity pain, who was found to have persistent hypercalcemia due to hypervitaminosis A. The patient ingested over 700 times the recommended intake of Vitamin A per day for age. Retention of vitamin A in the liver and adipose tissue causes toxic levels of retinoids and hypercalcemia. Conclusions Acute treatment included intravenous rehydration, furosemide, and calcitonin. Pamidronate was the definitive treatment for hypercalcemia from hypervitaminosis A due to its osteoclast inhibition and long biologic half-life. Parents should be counseled on risks of toxicity and absence of evidence showing benefits of vitamin A therapy for autism.

Finding treatable genetic obesity: strategies for success.

PURPOSE OF REVIEW: Genetic obesity is responsible for up to 7% of severe childhood obesity. Although current Pediatric Endocrine Society guidelines recommend assessment of children with early-onset morbid obesity and hyperphagia for underlying genetic disorders, a vast majority of patients are not being appropriately screened for genetic obesity syndromes. RECENT FINDINGS: With advances in genetic testing, more genetic causes of obesity are being identified. Treatments are likely to be individualized, depending on the cause of the obesity, and must be targeted at addressing the underlying cause. Investigational therapies include melanocortin-4 receptor antagonists, oxytocin and medications targeting the endocannabinoid system. SUMMARY: Improved identification of patients with genetic obesity syndromes will lead to development of new treatments and personalized management of these diseases.

Clinical and Histologic Characterization of Nonalcoholic Steatohepatitis in African American Patients.

OBJECTIVE: There has been a widespread misconception among physicians that African Americans are protected from developing nonalcoholic steatohepatitis (NASH). However, a formal histologic and metabolic comparison against well-matched Caucasians has never been performed. RESEARCH DESIGN AND METHODS: Sixty-seven African American patients were matched 2:1 to Caucasians (n = 134) for age, sex, BMI, hemoglobin A1c, and prevalence of type 2 diabetes mellitus (T2DM). Screening for NASH included measurement of intrahepatic triglyceride content by proton MRS (1H-MRS), followed by a liver biopsy if patients had hepatic steatosis. Insulin resistance was estimated during an oral glucose tolerance test using the Matsuda Index. RESULTS: Compared with Caucasians, African American patients had a lower intrahepatic triglyceride content (mean ± SD 6.1 ± 6.8% vs. 9.4 ± 7.5%, P = 0.007) and the presence of nonalcoholic fatty liver disease (NAFLD) was less common (25.0% vs. 51.9%, P = 0.003). However, prevalence of NASH was not different between ethnicities in patients with NAFLD (57.1% vs. 73.3%, P = 0.12). Moreover, they showed similar severity in each of the individual histologic parameters (inflammation, ballooning, and fibrosis). Among patients with NAFLD, insulin resistance was similar between both ethnic groups (Matsuda Index: 3.3 ± 1.8 vs. 3.1 ± 1.9, P = 0.61; adipose tissue insulin resistance [Adipo-IR] index: 5.7 ± 4.6 vs. 6.4 ± 4.7 mmol/L ⋅ µU/mL, P = 0.53) but appeared to be worse in African American versus Caucasian patients without NAFLD (Matsuda Index: 4.9 ± 3.6 vs. 7.0 ± 4.9, P = 0.11; Adipo-IR: 3.9 ± 2.8 vs. 2.7 ± 2.3 mmol/L ⋅ µU/mL, P = 0.06). African American patients also had lower plasma triglycerides and higher HDL cholesterol, independent of the severity of intrahepatic triglyceride. CONCLUSIONS: Although African Americans have lower intrahepatic triglyceride accumulation, once NAFLD develops, NASH occurs as frequently, and as severe, as in Caucasian patients. Therefore, African Americans with NAFLD should be screened for NASH with the same degree of clinical resolve as in Caucasian patients.

Symptomatic Floor-of-Mouth Swelling with Neck Extension in a 14-Year-Old Girl.

A plunging ranula is a soft-tissue mass stemming from a mucous extravasation cyst of the sublingual gland which can herniate through the mylohyoid muscle. We describe a case in which a 14-year-old girl presented with a rapidly expanding mass on the floor of her mouth affecting her ability to swallow and speak and causing tracheal compression. The patient was initially managed conservatively with antibiotics and steroids; however, the mass continued to expand necessitating emergent bedside incision and drainage and subsequent surgical intervention. The pathophysiology and management options for ranulas are also discussed herein.