Clinical burden of liver disease from hemochromatosis at an academic medical center.

Hereditary hemochromatosis (HH) can cause cirrhosis and hepatocellular carcinoma (HCC), but the frequency of these complications is controversial. To address this question, we reviewed the experience with HH at an academic medical center that is the sole liver transplantation center in a state with a population that is >90% Caucasian. The records of all subjects with International Classification of Diseases, Ninth Revision, code 275, "disorders of iron metabolism" seen at the University of Iowa Hospitals and Clinics between January 1, 2004 and December 31, 2014 were reviewed, and C282Y homozygotes and C282Y/H63D compound heterozygotes were identified.

Safety and accuracy of Ga-DOTATOC PET/CT in children and young adults with solid tumors.

Ga-DOTA-tyr3-Octreotide (Ga-DOTATOC) PET/CT has been shown to have high accuracy in adults with neuroendocrine tumors, however has not been studied in pediatric patients. This study evaluated the safety and accuracy of Ga-DOTATOC PET/CT in children and young adults with solid tumors that express somatostatin receptor type 2. A series of three prospective, IRB approved, clinical trials evaluating safety and efficacy of Ga-DOTATOC PET/CT were conducted for subjects aged 6 months to 90 years.

Corrigendum: The Impact of Aerobic Exercise on Fronto-Parietal Network Connectivity and Its Relation to Mobility: An Exploratory Analysis of a 6-Month Randomized Controlled Trial.

[This corrects the article on p. 344 in vol. 11, PMID: 28713255.

Fitting the Fractional Polynomial Model to Non-Gaussian Longitudinal Data.

As in cross sectional studies, longitudinal studies involve non-Gaussian data such as binomial, Poisson, gamma, and inverse-Gaussian distributions, and multivariate exponential families. A number of statistical tools have thus been developed to deal with non-Gaussian longitudinal data, including analytic techniques to estimate parameters in both fixed and random effects models. However, as yet growth modeling with non-Gaussian data is somewhat limited when considering the transformed expectation of the response via a linear predictor as a functional form of explanatory variables.

Active Experiencing Training Improves Episodic Memory Recall in Older Adults.

Active experiencing (AE) is an intervention aimed at attenuating cognitive declines with mindfulness training via an immersive acting program, and has produced promising results in older adults with limited formal education. Yet, the cognitive mechanism(s) of intervention benefits and generalizability of gains across cognitive domains in the course of healthy aging is unclear. We addressed these issues in an intervention trial of older adults ( = 179; mean age = 69.

How Cells Can Control Their Size by Pumping Ions.

The ability of all cells to set and regulate their size is a fundamental aspect of cellular physiology. It has been known for sometime but not widely so, that size stability in animal cells is dependent upon the operation of the sodium pump, through the so-called pump-leak mechanism (Tosteson and Hoffman, 1960). Impermeant molecules in cells establish an unstable osmotic condition, the Donnan effect, which is counteracted by the operation of the sodium pump, creating an asymmetry in the distribution of Na and K staving off water inundation.

Beta Adrenergic Regulation of Intrapulmonary Arteriovenous Anastomoses in Intact Rat and Isolated Rat Lungs.

Intrapulmonary arteriovenous anastomoses (IPAVA) allow large diameter particles of venous origin to bypass the pulmonary capillary bed and embolize the systemic arterial circulation. IPAVA have been routinely observed in healthy humans with exercise, hypoxia, and catecholamine infusion, but the mechanism by which they are recruited is not well-defined. We hypothesized that beta-adrenergic receptor stimulation recruits IPAVA and that receptor blockade would limit hypoxia-induced IPAVA recruitment.

Intracochlear Recordings of Acoustically and Electrically Evoked Potentials in Nucleus Hybrid L24 Cochlear Implant Users and Their Relationship to Speech Perception.

The Hybrid cochlear implant (CI) has been developed for individuals with high frequency hearing loss who retain good low frequency hearing. Outcomes have been encouraging but individual variability is high; the health of the cochlea and the auditory nerve may be important factors driving outcomes. Electrically evoked compound action potentials (ECAPs) reflect the response of the auditory nerve to electrical stimulation while electrocochleography (ECochG) reflects the response of the cochlear hair cells and auditory nerve to acoustic stimulation.

Spiral Ganglion Neuron Projection Development to the Hindbrain in Mice Lacking Peripheral and/or Central Target Differentiation.

We investigate the importance of the degree of peripheral or central target differentiation for mouse auditory afferent navigation to the organ of Corti and auditory nuclei in three different mouse models: first, a mouse in which the differentiation of hair cells, but not central auditory nuclei neurons is compromised ( ); second, a mouse in which hair cell defects are combined with a delayed defect in central auditory nuclei neurons ( ), and third, a mouse in which both hair cells and central auditory nuclei are absent (). Our results show that neither differentiated peripheral nor the central target cells of inner ear afferents are needed (hair cells, cochlear nucleus neurons) for segregation of vestibular and cochlear afferents within the hindbrain and some degree of base to apex segregation of cochlear afferents. These data suggest that inner ear spiral ganglion neuron processes may predominantly rely on temporally and spatially distinct molecular cues in the region of the targets rather than interaction with differentiated target cells for a crude topological organization.

Intracranial Electrophysiology of Auditory Selective Attention Associated with Speech Classification Tasks.

Auditory selective attention paradigms are powerful tools for elucidating the various stages of speech processing. This study examined electrocorticographic activation during target detection tasks within and beyond auditory cortex. Subjects were nine neurosurgical patients undergoing chronic invasive monitoring for treatment of medically refractory epilepsy.

An Open-Source Label Atlas Correction Tool and Preliminary Results on Huntingtons Disease Whole-Brain MRI Atlases.

The creation of high-quality medical imaging reference atlas datasets with consistent dense anatomical region labels is a challenging task. Reference atlases have many uses in medical image applications and are essential components of atlas-based segmentation tools commonly used for producing personalized anatomical measurements for individual subjects. The process of manual identification of anatomical regions by experts is regarded as a so-called gold standard; however, it is usually impractical because of the labor-intensive costs.

Probing Functional Changes in Exocyst Configuration with Monoclonal Antibodies.

Spatial regulation of exocytosis relies on the exocyst, a hetero-octameric protein complex that tethers vesicles to fusion sites at the plasma membrane. Nevertheless, our understanding of mechanisms regulating exocyst assembly/disassembly, localization, and function are incomplete. Here, we have exploited a panel of anti-Sec6 monoclonal antibodies (mAbs) to probe possible configurational changes accompanying transitions in exocyst function in epithelial MDCK cells.

Limited Wegener's Granulomatosis Presenting with an Isolated Abduction Deficit.

Wegener's granulomatosis often affects the orbit, typically presenting with painful proptosis. The authors describe a 14 year-old girl, with limited Wegener's granulomatosis, who initially presented with an isolated painless abduction deficit that spontaneously resolved over several weeks. She subsequently developed painful proptosis and diplopia, followed by facial and oral nodules.