Miracle-dz/UniFAR-FTUs huggingface dataset:
The Long‐Term Quality of Life of Living Kidney Donors: A Multicenter Cohort Study

Previous studies that described the long‐term quality of life of living kidney donors were conducted in single centers, and lacked data on a healthy nondonor comparison group. We conducted a retrospective cohort study to compare the quality of life of 203 kidney donors with 104 healthy nondonor controls using validated scales (including the SF36, 15D and feeling thermometer) and author‐developed questions. Participants were recruited from nine transplant centers in Canada, Scotland and Australia. Outcomes were assessed a median of 5.5 years after the time of transplantation (lower and upper quartiles of 3.8 and 8.4 years, respectively). 15D scores (scale of 0 to 1) were high and similar between donors and nondonors (mean 0.93 (standard deviation (SD) 0.09) and 0.94 (SD 0.06), p = 0.55), and were not different when results were adjusted for several prognostic characteristics (p = 0.55). On other scales and author‐developed questions, groups performed similarly. Donors to recipients who had an adverse outcome (death, graft failure) had similar quality of life scores as those donors where the recipient did well. Our findings are reassuring for the practice of living transplantation. Those who donate a kidney in centers that use routine pretransplant donor evaluation have good long‐term quality of life.
Perceiving nonverbal behavior: Neural correlates of processing movement fluency and contingency in dyadic interactions

Despite the fact that nonverbal dyadic social interactions are abundant in the environment, the neural mechanisms underlying their processing are not yet fully understood. Research in the field of social neuroscience has suggested that two neural networks appear to be involved in social understanding: (1) the action observation network (AON) and (2) the social neural network (SNN). The aim of this study was to determine the differential contributions of the AON and the SNN to the processing of nonverbal behavior as observed in dyadic social interactions. To this end, we used short computer animation sequences displaying dyadic social interactions between two virtual characters and systematically manipulated two key features of movement activity, which are known to influence the perception of meaning in nonverbal stimuli: (1) movement fluency and (2) contingency of movement patterns. A group of 21 male participants rated the “naturalness” of the observed scenes on a four‐point scale while undergoing fMRI. Behavioral results showed that both fluency and contingency significantly influenced the “naturalness” experience of the presented animations. Neurally, the AON was preferentially engaged when processing contingent movement patterns, but did not discriminate between different degrees of movement fluency. In contrast, regions of the SNN were engaged more strongly when observing dyads with disturbed movement fluency. In conclusion, while the AON is involved in the general processing of contingent social actions, irrespective of their kinematic properties, the SNN is preferentially recruited when atypical kinematic properties prompt inferences about the agents' intentions. Hum Brain Mapp 35:1362–1378, 2014. © 2013 Wiley Periodicals, Inc.
Improved Survival with Recent Post‐Transplant Lymphoproliferative Disorder (PTLD) in Children with Kidney Transplants

Post‐transplant lymphoproliferative disorder (PTLD) has been associated with high mortality, but recent anecdotal survival appeared better. From 1988 to 2010, the NAPRTCS registry had 235 registered PTLD cases. We sent a special 25‐point questionnaire study to the NAPRTCS centers with the most recent 150 cases to obtain additional follow‐up data not collected in the master registry, our objective being to determine the recent outcomes after PTLD and determine prognostic factors. We received 92 completed responses, in which only 12 (13%) deaths were reported, 2 from nonmedical causes, 10 with a functioning graft. Kaplan–Meier‐calculated patient survival was 90.6% at 1 year and 87.4% at 3, 4 and 5 years post‐PTLD. Graft survival post‐PTLD was 81.8% at 1 year, 68.0% at 3 years and 65.0% at 5 years. Seven patients received a retransplant after PTLD, with no PTLD recurrence reported. Using all 235 PTLD cases, the covariates associated with better patient survival were more recent year of PTLD diagnosis (adjusted hazard ratio AHR 0.86, p < 0.001), and with worse survival were late PTLD (AHR 1.98, p = 0.0176) and patient age above 13 at PTLD (AHR 3.43, p value 0.022). In children with kidney transplants, patient survival has improved with more recent PTLDs.
Ghrelin and Obestatin Levels in Children With Failure to Thrive and Obesity

Objectives: Ghrelin and obestatin are 2 gastric hormones with opposite effects on food intake and body weight. We investigated plasma ghrelin and obestatin in children with failure to thrive (FTT) and obesity as compared with age-matched controls. Methods: A total of 63 children were included in the study: 13 with FTT, 17 with obesity, and 33 age-matched controls. Children fasted for at least 8 hours before specimen collection. Both hormones were measured using commercially available enzyme immunoassay kits. Results: Ghrelin and obestatin levels in children with FTT were not significantly different from that of the age-matched controls (P >0.05). In children with obesity, the total ghrelin levels were significantly lower (P = 0.0003) and the obestatin levels significantly higher (P = 0.029) compared with those in controls. In the control group, the fasting ghrelin level was significantly higher in the younger (<3 years) than in the older children (>3 years; P = 0.0004). Obestatin levels correlated positively with weight-for-age percentiles in the obese group (P = 0.011) and negatively in the control group >3 years (P = 0.019). Conclusions: Compared with the levels in age-matched controls, fasting ghrelin and obestatin levels did not differ significantly in children with FTT. In the children with obesity, the decreased ghrelin and increased obestatin levels suggest a possible adaptive process to positive energy balance. Ghrelin had pronounced age-related changes, and obestatin was associated with the weight status. This may suggest that these 2 hormones use different mechanisms to regulate energy balance and weight.
Dietary Fiber Mixture in Pediatric Patients With Controlled Chronic Constipation

Objectives: The aim of the study was to test the clinical efficacy and effect on colonic transit time (CTT) of a dietary fiber mixture given to children with controlled chronic constipation (CC) after the withdrawal of stool softeners and enemas. Methods: This randomized, placebo-controlled, double-blind clinical trial involved 54 patients aged 4 to 12 years and had CC that was controlled by the use of low-dose stool softeners. The use of these softeners was discontinued when the patients were admitted to the clinical trial. The patients were randomized into 2 groups for the 4-week study period. One group received a dietary fiber mixture and the other group received a placebo (maltodextrin). The primary outcome was therapeutic failure (oral stool softeners or enemas was required to prescribe during the trial). Secondary outcomes included defecation frequency, stool consistency (measured using the Bristol Stool Form Scale), and CTT. Results: Therapeutic failure was observed in 34.6% (9/26) of the patients in the dietary fiber mixture group and in 35.7% (10/28) in the control group (P = 0.933). The mean increase in daily bowel movements was 0.53 in the dietary fiber mixture group and 0.23 in the control group (P = 0.014). The patients in the dietary fiber mixture group (60.0%) passed nonhardened stools more frequently than did those in the control group (16.7%, P = 0.003). The CTT was similar for both groups. Conclusions: The fiber mixture did not prevent the suspension of stool softeners or lead to reduced CTT; however, the mixture promoted an increased frequency of defecation and an improvement in the stool consistency.
Postconcussional disorder and PTSD symptoms of military‐related traumatic brain injury associated with compromised neurocircuitry

Traumatic brain injury (TBI) is a common combat injury, often through explosive blast, and produces heterogeneous brain changes due to various mechanisms of injury. It is unclear whether the vulnerability of white matter differs between blast and impact injury, and the consequences of microstructural changes on neuropsychological function are poorly understood in military TBI patients. Diffusion tensor imaging (DTI) techniques were used to assess the neurocircuitry in 37 US service members (29 mild, 7 moderate, 1 severe; 17 blast and 20 nonblast), who sustained a TBI while deployed, compared to 14 nondeployed, military controls. High‐dimensional deformable registration of MRI diffusion tensor data was followed by fiber tracking and tract‐specific analysis along with region‐of‐interest analysis. DTI results were examined in relation to post‐concussion and post‐traumatic stress disorder (PTSD) symptoms. The most prominent white matter microstructural injury for both blast and nonblast patients was in the frontal fibers within the fronto‐striatal (corona radiata, internal capsule) and fronto‐limbic circuits (fornix, cingulum), the fronto‐parieto‐occipital association fibers, in brainstem fibers, and in callosal fibers. Subcortical superior‐inferiorly oriented tracts were more vulnerable to blast injury than nonblast injury, while direct impact force had more detrimental effects on anterior‐posteriorly oriented tracts, which tended to cause heterogeneous left and right hemispheric asymmetries of white matter connectivity. The tractography using diffusion anisotropy deficits revealed the cortico‐striatal‐thalamic‐cerebellar‐cortical (CSTCC) networks, where increased post‐concussion and PTSD symptoms were associated with low fractional anisotropy in the major nodes of compromised CSTCC neurocircuitry, and the consequences on cognitive function were explored as well. Hum Brain Mapp 35:2652–2673, 2014. © 2013 Wiley Periodicals, Inc.
Successful Gastrostomy Tube Weaning Program Using an Intensive Multidisciplinary Team Approach

Objectives: The present study evaluated the effectiveness of a multidisciplinary intensive inpatient model for gastrostomy tube (GT) weaning. Methods: A retrospective chart review was completed on 30 GT-dependent children, ages 3.9 (±1.4) years, admitted to the inpatient feeding program (length of stay 19 days) from May 2009 to December 2011. Administered GT calories were decreased on admission by an average of 73% from home regimen. Patients were offered 3 meals and 2 to 3 snacks/day, including 3 intensive feeding therapy sessions (Monday to Friday), along with psychosocial support, nutrition guidance, and behavioral therapy. Daily calorie counts and weights were recorded. Patients returned for a postdischarge feeding evaluation at an average of 4 months and a clinic visit at 1 year. Data were analyzed using paired samples t tests. Results: Before admission, patients received 69% (±25) of goal calories by GT and 22% (±19) of goal calories orally. During admission, average caloric intake by mouth as a percentage of goal increased during the course of weeks 1, 2, and 3 (68%, 77%, and 82%, respectively), with a statistically significant increase between weeks 1 and 2 (P = 0.001) and 1 and 3 (P = 0.011). At discharge, 90% had discontinued GT feedings. Average percent weight change during admission was 0.2% (±4). At 1 year follow-up, 83% remained successfully off GT feedings. Conclusions: Children who are GT dependent can be weaned off GT feedings during a 3-week admission using a multidisciplinary feeding model. The therapeutic gains were maintained at 1 year postdischarge.
Lipid peroxidation in type 2 diabetes mellitus

AIM: The link between hyperglycemia, enhanced freeradical activity, and the complications of diabetes is unknown. The purpose of this study is to evaluate the levels of malondialdehyde (MDA) measured as thiobarbituric acid-reactive substances (TBARS), an index of lipid peroxidation, in patients of type 2 diabetes without complications, and compare with normal subjects of the same population. METHODOLOGY: We recruited 60 type 2 diabetic subjects without complications and with poor metabolic control and 60 age-matched controls with good metabolic control. Levels of glucose, total cholesterol, HbA 1C , and MDA as TBARS were determined. RESULTS: Diabetic patients had higher levels of blood glucose (P<0.001), HbA 1C (P<0.001), and MDA (P<0.001) than control subjects. The total cholesterol of the control subjects and diabetic patients did not differ. There was no correlation between the family history in diabetics and elevation in either HbA or 1C MDA levels. CONCLUSION: Increased levels of MDA may be a useful marker of oxidative stress. The enhanced lipid peroxidation leads to an increase in free-radical activity in type 2 diabetics. This increase in free-radical activity in type 2 diabetes mellitus along with insulin resistance can lead to activation of stress-sensitive pathways, which may play an important role in the complications of diabetes.
Role of Pediatric Endoscopic Retrograde Cholangiopancreatography in an Era Stressing Less-Invasive Imaging Modalities

Background:The significance of pediatric endoscopic retrograde cholangiopancreatography (ERCP) according to age or disease variation is inconclusive. This study aimed to evaluate the usefulness of pediatric ERCP in diagnosing or treating small children with pancreaticobiliary disorders, including choledochal cyst (CC) and biliary atresia (BA). Methods:From 1980 to 2011, 235 ERCPs were performed in 220 pediatric patients (median age, 2 years) at our institution. Underlying pathology was CC in 92 patients (3 years), BA in 62 patients (55 days), and others in 66 patients. Success and complication rates, and ERCP findings were retrospectively analyzed. Results:The overall success rate was 96%. Rates for individual pathologies were 99% for CC, 92% for BA, and 96% for others. The success rate was 100% among children >3 years, but 92% when limited to infants. Post-ERCP hyperamylasemia and duodenal perforation occurred in 9% and 0.4% of cases, respectively. Regarding preoperative evaluation of the pancreaticobiliary system in CC, ERCP clearly delineated pancreaticobiliary maljunction (79%) and the pancreatic duct (94%), whereas it visualized the common bile duct and intrahepatic bile duct at relatively low rates (77% and 33%, respectively). ERCP successfully identified 16 cases (18%) with non-BA of 90 patients with suspected BA. Moreover, ERCP demonstrated only pancreatic duct in 70% of all BA. Conclusions:ERCP among children including infants with CC and BA can be performed with fairly satisfactory results. Although pediatric ERCP can also help describe the pancreaticobiliary system in detail, its indication should be deliberately considered when anatomical information from less-invasive imaging modalities is insufficient.
Liver Enzyme Elevations Within 3 Months of Diagnosis of Inflammatory Bowel Disease and Likelihood of Liver Disease

Background: Inflammatory bowel disease–associated liver diseases (IBD-LDs) include autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and an overlap syndrome. Prospective unbiased multicenter data regarding the frequency of IBD-LD in patients with pediatric inflammatory bowel disease (IBD) are lacking. We examined early alanine aminotransferase (ALT) and &ggr;-glutamyl transpeptidase (GGT) elevations in children diagnosed as having IBD and assessed the likelihood of IBD-LD. Methods: Data collected from the prospective observational Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry enrolling children of age <16 years within 30 days of diagnosis. AIH, PSC, and overlap syndrome were diagnosed using local institutional criteria. Results: A total of 1569 subjects had liver enzymes available. Of the total, 757 had both ALT and GGT, 800 had ALT only (no GGT), and 12 had GGT only (no ALT). Overall, 29 of 1569 patients (1.8%) had IBD-LD. IBD-LD was diagnosed in 1 of 661 (0.15%) of patients with both ALT and GGT ⩽ 50 IU/L compared with 21 of 42 (50%) of patients with both ALT and GGT > 50 (odds ratio 660, P < 0.0001). Of the 29 patients with IBD-LD, 21 had PSC, 2 had AIH, and 6 had overlap syndrome. IBD-LD was more common in patients with ulcerative colitis and IBD-unclassified (indeterminate colitis) than in those with Crohn disease (4% vs 0.8%, respectively, P < 0.001). Conclusions: Elevation of both ALT and GGT within 90 days after the diagnosis of IBD is associated with a markedly increased likelihood of IBD-LD. Both ALT and GGT levels should be measured in all of the pediatric patients newly diagnosed as having IBD.
Investigation of morphometric variability of subthalamic nucleus, red nucleus, and substantia nigra in advanced Parkinson's disease patients using automatic segmentation and PCA‐based analysis

Subthalamic nucleus (STN) deep brain stimulation (DBS) is an effective surgical therapy to treat Parkinson's disease (PD). Conventional methods employ standard atlas coordinates to target the STN, which, along with the adjacent red nucleus (RN) and substantia nigra (SN), are not well visualized on conventional T1w MRIs. However, the positions and sizes of the nuclei may be more variable than the standard atlas, thus making the pre‐surgical plans inaccurate. We investigated the morphometric variability of the STN, RN and SN by using label‐fusion segmentation results from 3T high resolution T2w MRIs of 33 advanced PD patients. In addition to comparing the size and position measurements of the cohort to the Talairach atlas, principal component analysis (PCA) was performed to acquire more intuitive and detailed perspectives of the measured variability. Lastly, the potential correlation between the variability shown by PCA results and the clinical scores was explored. Hum Brain Mapp 35:4330–4344, 2014. © 2014 Wiley Periodicals, Inc.
Brain systems mediating voice identity processing in blind humans

Blind people rely more on vocal cues when they recognize a person's identity than sighted people. Indeed, a number of studies have reported better voice recognition skills in blind than in sighted adults. The present functional magnetic resonance imaging study investigated changes in the functional organization of neural systems involved in voice identity processing following congenital blindness. A group of congenitally blind individuals and matched sighted control participants were tested in a priming paradigm, in which two voice stimuli (S1, S2) were subsequently presented. The prime (S1) and the target (S2) were either from the same speaker (person‐congruent voices) or from two different speakers (person‐incongruent voices). Participants had to classify the S2 as either a old or a young person. Person‐incongruent voices (S2) compared with person‐congruent voices elicited an increased activation in the right anterior fusiform gyrus in congenitally blind individuals but not in matched sighted control participants. In contrast, only matched sighted controls showed a higher activation in response to person‐incongruent compared with person‐congruent voices (S2) in the right posterior superior temporal sulcus. These results provide evidence for crossmodal plastic changes of the person identification system in the brain after visual deprivation. Hum Brain Mapp 35:4607–4619, 2014. © 2014 Wiley Periodicals, Inc.
Microvillous Atrophy: Atypical Presentations

Objectives: Microvillous inclusion disease (MVID) is a cause of intractable diarrhea in infancy. In its classic form, the disease is characterized by a severe persistent watery diarrhea starting within the first days of life. Parenteral nutrition and small bowel transplantation are the only known treatments for the affected children. Histologically, periodic acid-Schiff (PAS) staining shows accumulation of periodic acid-Schiff–positive staining material along the apical pole of enterocytes, whereas transmission electron microscopy exhibits microvillus inclusion bodies within the cytoplasm of enterocytes with rarefied and shortened microvilli and secretory granules. The objective of this work was to explore clinical, morphological, and genetic findings in cases of MVID with unusual presentations. Methods: Clinical, histological, and genetic findings are reported for 8 cases of MVID with atypical presentation. Results: The diarrhea started after several months in 3 cases. It was usually less abundant and 3 patients were weaned off parenteral nutrition. None required intestinal transplantation. Three patients experienced malformations, dysmorphy, sensory disabilities, and severe mental retardation. One had a hydrocephaly. Three patients had a cholestasis with low &ggr;-glutamyl transferase levels. Light microscopy showed histological abnormalities consistent with MVID in all of the cases, but the lesions were sometimes focal or delayed. Transmission electron microscopy retrieved some criteria of MVID in 4 patients. Finally, 6 patients were homozygotes or compound heterozygotes for MYO5B mutations. Conclusions: This study extends the spectrum of MVID to less severe clinical presentations.
Influences of Breast Milk Composition on Gastric Emptying in Preterm Infants

Objectives: The aim of the present study was to determine whether specific biochemical and energy concentrations influence gastric emptying of unfortified and fortified mother's own milk (MOM) in stable preterm infants, and whether gastric emptying differs between feeds of unfortified MOM and feeds fortified with S-26 or FM 85 human milk fortifier (HMF) when infants are fed the same volume under similar conditions. Influences of infant gestation, age, and weight, and feed characteristics were also explored. Methods: Stomach volumes of 25 paired unfortified and fortified MOM feeds were monitored prefeed and postfeed delivery and at 30-minute intervals thereafter. For each feed, MOM samples were analyzed to determine concentrations of total protein, casein, whey, carbohydrate, lactose, fat, and energy. Fortified feed compositions were calculated by adding fortifier biochemical and energy concentrations to unfortified MOM concentrations. Ultrasound images were used to calculate infant stomach volumes. Statistical comparisons were made of paired stomach volume measurements. Results: Higher feed concentrations of casein were associated with faster gastric emptying during feed delivery (P = 0.007). When compared with unfortified MOM, S-26 fortified feeds emptied similarly, whereas FM 85 fortified feeds emptied more slowly both during feed delivery and during the postprandial period (P = 0.002, <0.001, respectively). Gastric emptying was slower for 2-hourly feeds compared with that for 3-hourly feeds (P = 0.003) and in supine position compared with that in prone (P = 0.001). Conclusions: Breast milk composition influences gastric emptying in stable preterm infants, with feeds of higher casein concentration emptying faster during feeding than otherwise equivalent feeds, and FM 85 fortified MOM emptying more slowly than unfortified MOM.