Doege-Potter Syndrome: A Solitary Fibrous Tumor Causing Non-Islet Cell Tumor Hypoglycemia.

Doege-Potter syndrome occurs when incompletely processed insulin-like growth factor 2 (IGF-2), also known as big IGF-2, is produced by a solitary fibrous tumor (SFT) and results in non-islet cell tumor hypoglycemia (NICTH). We discuss here the case of a 66-year-old male who presented with a 2-week history of increasing confusion and a serum glucose of 34 mg/dL. The patient's symptoms immediately improved with dextrose. The patient did not use insulin, serum sulfonylurea screen was negative, and testing for adrenal insufficiency was unremarkable. Outpatient laboratory evaluation revealed a serum glucose of 48 mg/dL along with low insulin, C-peptide, and proinsulin levels. Further work-up showed an IGF-2 to IGF-1 ratio of 38:1. A ratio greater than 10:1 is diagnostic of NICTH. Imaging demonstrated a 21-cm mass in the lower abdomen and pelvis. The patient underwent surgical resection. The hypoglycemia resolved immediately postoperatively. Surgical pathology revealed a malignant SFT. In NICTH, big IGF-2 forms a complex that is biologically active and saturates the insulin and IGF receptors, resulting in refractory hypoglycemia. Although glucocorticoids can mitigate hypoglycemia, complete surgical resection is the only definitive treatment of NICTH. This case highlights the importance of maintaining a broad differential for seemingly simple hypoglycemia.

Regional variations in medical trainee diet and nutrition counseling competencies: Machine learning-augmented propensity score analysis of a prospective multi-site cohort study.

BACKGROUND: Medical professionals and students are inadequately trained to respond to rising global obesity and nutrition-related chronic disease epidemics, primarily focusing on cardiovascular disease. Yet, there are no multi-site studies testing evidence-based nutrition education for medical students in preventive cardiology, let alone establishing student dietary and competency patterns. METHODS: Cooking for Health Optimization with Patients (CHOP; NIH NCT03443635) was the first multi-national cohort study using hands-on cooking and nutrition education as preventive cardiology, monitoring and improving student diets and competencies in patient nutrition education. Propensity-score adjusted multivariable regression was augmented by 43 supervised machine learning algorithms to assess students outcomes from UT Health versus the remaining study sites. RESULTS: 3,248 medical trainees from 20 medical centers and colleges met study criteria from 1 August 2012 to 31 December 2017 with 60 (1.49%) being from UTHealth. Compared to the other study sites, trainees from UTHealth were more likely to consume vegetables daily (OR 1.82, 95%CI 1.04-3.17, p=0.035), strongly agree that nutrition assessment should be routine clinical practice (OR 2.43, 95%CI 1.45-4.05, p=0.001), and that providers can improve patients' health with nutrition education (OR 1.73, 95%CI 1.03-2.91, p=0.038). UTHealth trainees were more likely to have mastered 12 of the 25 competency topics, with the top three being moderate alcohol intake (OR 1.74, 95%CI 0.97-3.11, p=0.062), dietary fats (OR 1.26, 95%CI 0.57-2.80, p=0.568), and calories (OR 1.26, 95%CI 0.70-2.28, p=0.446). CONCLUSION: This machine learning-augmented causal inference analysis provides the first results that compare medical students nationally in their diets and competencies in nutrition education, highlighting the results from UTHealth. Additional studies are required to determine which factors in the hands-on cooking and nutrition curriculum for UTHealth and other sites produce optimal student - and, eventually, preventive cardiology - outcomes when they educate patients in those classes.

Deletion of ghrelin prevents aging-associated obesity and muscle dysfunction without affecting longevity.

During aging, decreases in energy expenditure and locomotor activity lead to body weight and fat gain. Aging is also associated with decreases in muscle strength and endurance leading to functional decline. Here, we show that lifelong deletion of ghrelin prevents development of obesity associated with aging by modulating food intake and energy expenditure. Ghrelin deletion also attenuated the decrease in phosphorylated adenosine monophosphate-activated protein kinase (pAMPK) and downstream mediators in muscle, and increased the number of type IIa (fatigue resistant, oxidative) muscle fibers, preventing the decline in muscle strength and endurance seen with aging. Longevity was not affected by ghrelin deletion. Treatment of old mice with pharmacologic doses of ghrelin increased food intake, body weight, and muscle strength in both ghrelin wild-type and knockout mice. These findings highlight the relevance of ghrelin during aging and identify a novel AMPK-dependent mechanism for ghrelin action in muscle.

Relevance of low testosterone to non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease (NAFLD) is a condition where there is excess accumulation of triglycerides in the liver in the absence of excess alcohol consumption. It ranges from simple steatosis to non-alcoholic steatohepatitis (NASH), which can progress to fibrosis, cirrhosis, and hepatocellular carcinoma (HCC). NAFLD, one of the most common causes of chronic liver disease in Western populations, is the hepatic component of the metabolic syndrome (MetS) and is associated with increased visceral adipose tissue (VAT), insulin resistance, and dyslipidemia. Studies have also shown that testosterone deficiency is associated with increased VAT and insulin resistance in males while hyperandrogenemia has been associated with increased risk of insulin resistance and VAT in females. Thus, the aims of this review are to discuss the available experimental and epidemiological data evaluating the association between testosterone and NAFLD, to discuss the potential clinical relevance of these data, and to identify gaps in the literature.

Comprehensive diagnostic battery for evaluating sensorineural hearing loss in children.

OBJECTIVE: Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child. STUDY DESIGN: Retrospective review. SETTING: Tertiary care university hospital. PATIENTS: A total of 270 children referred for severe to profound SNHL between January 2002 and June 2009. INTERVENTIONS: Results of the following were reviewed: magnetic resonance imaging, computed tomography, renal ultrasound, electrocardiography, fluorescent treponemal antibody absorption test, connexin 26 sequencing, genetic consultation, and ophthalmologic consultation. MAIN OUTCOME MEASURE: Diagnostic yield of each test was determined. RESULTS: Each diagnostic test or consultation was completed by at least 95% of patients for whom it was ordered. Magnetic resonance imaging revealed abnormalities explaining SNHL in 24% of patients. Computed tomography showed inner ear anomalies in 18% of patients. Biallelic connexin 26 mutations were found in 15%. Renal ultrasound found anomalies in 4% of patients. Electrocardiography found 1% of patients with prolonged QT intervals. Fluorescent treponemal antibody absorption test result was positive in 0.5%. Genetic consultation found a genetic cause for hearing loss in 25%. Ophthalmologic consultation found abnormalities associated with hearing loss in 8%. CONCLUSION: Diagnostic radiologic imaging is the highest yielding test for evaluating children with SNHL. Connexin 26 sequencing identifies a nearly nonoverlapping subset of children compared with imaging. Specialty consultations, particularly from a clinical geneticist, can improve diagnostic yield. Other tests, although of lower diagnostic yield for SNHL, can identify important diseases that significantly affect patient health.

Characteristics of malfunctioning channels in pediatric cochlear implants.

OBJECTIVES/HYPOTHESIS: To examine the characteristics of pediatric cochlear implant channel malfunction preceding device failure. STUDY DESIGN: : Retrospective review. METHODS: All pediatric patients who underwent cochlear implantation at a tertiary academic medical center were reviewed regarding device type, reason for replacement, time to replacement, and timing and pattern of channel faults in failed versus nonfailed devices. RESULTS: Between 1993 and 2008, 264 pediatric cochlear implantations were performed. With an average 894-day follow-up, the replacement rate was 9.5% (25/264). Reasons for replacement were device failure (6.4%), medical/surgical failure (2.3%), and obsolescence (0.8%). Replacement rates were comparable among Advanced Bionics (13.3%), Cochlear Corporation (6.3%), and MED-EL (10.3%) devices. Fifty-two cochlear implants developed at least one channel fault, and 13 eventually progressed to failure requiring replacement. MED-EL devices comprised 12 of these 13 failures. At the 12-month follow-up interval, one, three, and five channel faults predicted 40%, 75%, and 100% probabilities of eventual electrode failure, respectively. Channels destined to fail demonstrated small, yet statistically significant, impedance elevations 12 months before failure and large elevations 3 months before failure. CONCLUSIONS: Replacement of cochlear implants in pediatric patients is common and is due to device malfunction about one half of the time. Earlier initial channel fault, earlier subsequent channel faults, adjacent channel faults, and a greater total number of channel faults were associated with the need for replacement surgery. Elevations in a channel's impedance should raise the concern for an impending failure. These predictors can help the cochlear implant team when considering surgery to replace the device.