Chronic Diarrhea Due to Eosinophilic Enterocolitis With Coexisting Eosinophilic Esophagitis.

Eosinophilic gastrointestinal disorders (EGIDs) are a spectrum of disorders including eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. We report a case of EGID involving the esophagus, small intestine, and large intestine simultaneously. A 38-year-old male patient presented with chronic diarrhea, abdominal pain, and unquantified weight loss for the last two months, which not improving with routine empirical treatment. Endoscopy revealed erosions in the stomach, duodenum, terminal ileum, and proximal colon. Biopsy revealed eosinophilic infiltration in the esophagus, terminal ileum, and proximal colon. Contrast-enhanced CT showed multiple skip areas of short- and long-segment circumferential mural thickening with enhancement in the jejunum and ileal loops, causing mild luminal narrowing with pelvic ascites, indicating involvement of muscular and probably serosal layer to a lesser degree (absence of obstructive symptoms with minimal ascites) along with predominant mucosal involvement (responsible for clinical symptoms). The patient was treated with elimination diet, systemic corticosteroids, and montelukast. Diarrheal episodes decreased, and the treatment was shifted to oral budesonide. We believe it to be one of the first reports to show a simultaneous involvement of the esophagus, small intestine, and large intestine, along with mucosal and mural involvement. It strengthens the fact that a common underlying pathogenesis causes EGIDs and an underlying muscular layer involvement in patients with predominant mucosal disease.

Obesity Correlates With Glomerulomegaly But Is Not Associated With Kidney Dysfunction Early After Donation.

BACKGROUND: Body mass index (BMI) is a convenient measure used to assess obesity and is used to select candidates for kidney donation. Glomerulomegaly is an early indicator of obesity-related kidney disease. Whether obesity assessment by BMI best reflects underlying glomerulomegaly and is predictive of adverse changes in renal function postdonation is unclear. METHODS: We performed a retrospective study on a cohort of 1065 living donors at the Mayo Clinic in Rochester; obesity measures by BMI and by computed tomography were compared between 20 donors with largest to 20 donors with the smallest glomerular volumes (on implantation biopsy). In addition, the change in kidney function postdonation (mean 7 months) was compared across BMI groups (<25, 25-29, 30-34, ≥35 kg/m2) in about 500 donors. RESULTS: We observed that larger glomerular volume was more strongly associated with BMI per standard deviation (SD) (odds ratio [OR] =5.0, P = 0.002) than waist circumference/height2 per SD (OR = 3.9, P = 0.02), visceral fat/height2 per SD (OR = 2.4, P = 0.02), subcutaneous fat/height2 per SD (OR = 2.0, P = 0.06), renal hilar fat/height2 per SD (OR = 1.6, P = 0.19), or peri/pararenal fat/height2 per SD (OR = 1.5, P = 0.23). Postdonation changes in glomerular filtration rate, blood pressure, and albuminuria were similar across BMI categories. CONCLUSIONS: The BMI outperforms various computed tomography measures of abdominal fat in detecting obesity-related glomerulomegaly. Despite this strong association with glomerulomegaly, short-term renal function outcomes are similar across BMI categories. Long-term follow-up is required to definitively define the impact of obesity on kidney function after donation.

Automated assessment of renal cortical surface roughness from computerized tomography images and its association with age.

RATIONALE AND OBJECTIVES: Nephrosclerosis occurs with aging and is characterized by increased kidney subcapsular surface irregularities at autopsy. Assessments of cortical roughness in vivo could provide an important measure of nephrosclerosis. The purpose of this study was to develop and validate an image-processing algorithm for quantifying renal cortical surface roughness in vivo and determine its association with age. MATERIALS AND METHODS: Renal cortical surface roughness was measured on contrast-enhanced abdominal computed tomography (CT) images of potential living kidney donors. A roughness index was calculated based on geometric curvature of each kidney from three-dimensional images and compared to visual observation scores. Cortical roughness was compared between the oldest and youngest donors, and its interaction with cortical volume and age assessed. RESULTS: The developed quantitative roughness index identified significant differences in kidneys with visual surface roughness scores of 0 (minimal), 1 (mild), and 2 (moderate; P < .001) in a random sample of 200 potential kidney donors. Cortical roughness was significantly higher in the 94 oldest (64-75 years) versus 91 youngest (18-25 years) potential kidney donors (P < .001). Lower cortical volume was associated with older age but not with roughness (r = -0.03, P = .75). The association of oldest age group with roughness (odds ratio [OR] = 1.8 per standard deviation [SD] of roughness index) remained significant after adjustment for total cortex volume (OR = 2.0 per SD of roughness index). CONCLUSIONS: A new algorithm to measure renal cortical surface roughness from CT scans detected rougher surface in older compared to younger kidneys, independent of cortical volume loss. This novel index may allow quantitative evaluation of nephrosclerosis in vivo using contrast-enhanced CT.

Age, kidney function, and risk factors associate differently with cortical and medullary volumes of the kidney.

The kidney atrophies in patients with advanced chronic kidney disease (CKD) but factors influencing kidney size in normal adults are less clear. To help define this, we measured kidney volumes on contrast-enhanced computed tomographic images from 1344 potential kidney donors (aged 18-75 years). Cortical volume per body surface area progressively declined in both genders with increased age. Statistically, this was primarily dependent on the age-related decline in glomerular filtration rate (GFR). Independent predictors of increased cortical volume per body surface area were male gender, increased GFR, increased 24-h urine albumin, current smoker, and decreased high-density lipid cholesterol. Medullary volume per body surface area increased with age in men, while it increased with age in women until the age of 50 years followed by a subsequent decline. Independent predictors of increased medullary volume per body surface area were older age, male gender, increased GFR, increased 24-h urine albumin, increased serum glucose, and decreased serum uric acid. Thus, while cortical volume declines with age along the same biological pathway as the age-related decline in GFR, albuminuria and some risk factors are actually associated with increased cortical or medullary volume among relatively healthy adults. Underlying hypertrophy or atrophy of different nephron regions may explain these findings.

Disrupted thalamocortical connectivity in PSP: a resting-state fMRI, DTI, and VBM study.

Progressive supranuclear palsy (PSP) is associated with pathological changes along the dentatorubrothalamic tract and in premotor cortex. We aimed to assess whether functional neural connectivity is disrupted along this pathway in PSP, and to determine how functional changes relate to changes in structure and diffusion. Eighteen probable PSP subjects and 18 controls had resting-state (task-free) fMRI, diffusion tensor imaging and structural MRI. Functional connectivity was assessed between thalamus and the rest of the brain, and within the basal ganglia, salience and default mode networks (DMN). Patterns of atrophy were assessed using voxel-based morphometry, and patterns of white matter tract degeneration were assessed using tract-based spatial statistics. Reduced in-phase functional connectivity was observed between the thalamus and premotor cortex including supplemental motor area (SMA), striatum, thalamus and cerebellum in PSP. Reduced connectivity in premotor cortex, striatum and thalamus were observed in the basal ganglia network and DMN, with subcortical salience network reductions. Tract degeneration was observed between cerebellum and thalamus and in superior longitudinal fasciculus, with grey matter loss in frontal lobe, premotor cortex, SMA and caudate nucleus. SMA functional connectivity correlated with SMA volume and measures of cognitive and motor dysfunction, while thalamic connectivity correlated with degeneration of superior cerebellar peduncles. PSP is therefore associated with disrupted thalamocortical connectivity that is associated with degeneration of the dentatorubrothalamic tract and the presence of cortical atrophy.

Clinical correlates of white matter tract degeneration in progressive supranuclear palsy.

OBJECTIVES: To use diffusion tensor imaging to assess white matter tract degeneration in progressive supranuclear palsy (PSP) and to investigate correlates between tract integrity and clinical measures. DESIGN: Case-control study. SETTING: Tertiary care medical center. PATIENTS/PARTICIPANTS: Twenty patients with probable PSP and 20 age- and sex-matched healthy controls were enrolled. All patients with PSP underwent standardized clinical testing, including the Frontal Behavioral Inventory and Frontal Assessment Battery to assess behavioral change, the PSP Rating Scale to measure disease severity, the Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (parts II and III) to measure motor function, and the PSP Saccadic Impairment Scale to measure eye movement abnormalities. METHODS: Fractional anisotropy and mean diffusivity were measured using region of interest analysis and tract-based spatial statistics. RESULTS: Compared with controls, abnormal diffusivity was observed predominantly in the superior cerebellar peduncles, body of the corpus callosum, inferior longitudinal fasciculus, and superior longitudinal fasciculus in patients with PSP. Fractional anisotropy values in the superior cerebellar peduncles correlated with disease severity (r = -0.59, P = .006), inferior longitudinal fasciculus correlated with motor function (r = -0.51, P = .02), and superior longitudinal fasciculus correlated with severity of saccadic impairments (r = -0.45, P = .047). CONCLUSIONS: The results of this study demonstrate that PSP is associated with degeneration of the brainstem, association, and commissural fibers and that this degeneration likely plays an important role in clinical dysfunction.

Effect of APOE ε4 status on intrinsic network connectivity in cognitively normal elderly subjects.

OBJECTIVE: To examine default mode and salience network functional connectivity as a function of APOE ε4 status in a group of cognitively normal age-, sex-, and education-matched older adults. DESIGN: Case-control study. SUBJECTS: Fifty-six cognitively normal APOE ε4 carriers and 56 age-, sex- and education-matched cognitively normal APOE ε4 noncarriers. MAIN OUTCOME MEASURE: Alterations in in-phase default mode and salience network connectivity in APOE ε4 carriers compared with APOE ε4 noncarriers ranging from 63 to 91 years of age. RESULTS: A posterior cingulate seed revealed decreased in-phase connectivity in regions of the posterior default mode network that included the left inferior parietal lobe, left middle temporal gyrus, and bilateral anterior temporal lobes in the ε4 carriers relative to APOE ε4 noncarriers. An anterior cingulate seed showed greater in-phase connectivity in the salience network including the cingulate gyrus, medial prefrontal cortex, bilateral insular cortex, striatum, and thalamus in APOE ε4 carriers vs noncarriers. There were no groupwise differences in brain anatomy. CONCLUSIONS: The observation of functional alterations in default mode and salience network connectivity in the absence of structural changes between APOE ε4 carriers and noncarriers suggests that alterations in connectivity may have the potential to serve as an early biomarker.