bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Variability After Partial External Biliary Diversion in Familial Intrahepatic Cholestasis 1 Deficiency
Authors: Squires James E. , Celik Neslihan , Morris Amy , Soltys Kyle , Mazariegos George , Shneider Benjamin , Squires Robert H. 
Published: December 2016

ABSTRACTObjectives:Familial intrahepatic cholestasis 1 (FIC1) **deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease.Methods:We performed a single‐center, retrospective review of genetically confirmed FIC1 deficient patients who received PEBD. Clinical outcomes after PEBD were cholestasis, pruritus, fat‐soluble vitamin supplementation, growth, and markers of disease progression that included splenomegaly and aspartate aminotransferase‐to‐platelet ratio index.Results:Eight patients with FIC1 disease and PEBD were included. Mean follow‐up was 32 months (range 15–65 months). After PEBD, total bilirubin was <2 mg/dL in all patients at 8 months after surgery, but 7 of 8 subsequently experienced a total of 15 recurrent cholestatic events. Subjective assessments of pruritus demonstrated improvement, but itching exacerbation occurred during cholestatic episodes. High‐dose fat‐soluble vitamin supplementation persisted, with increases needed during cholestatic episodes. Weight z scores improved (−3.4 to −1.65, P < 0.01). Splenomegaly did not worsen or develop and 1 patient developed an aminotransferase‐to‐platelet ratio index score of >0.7 suggesting development of fibrosis 24 months after PEBD.Conclusions:Clinical variability is evident among genetically defined FIC1 deficient patients after PEBD, even among those with identical mutations. Recurrent, self‐limited episodes of cholestasis and pruritus are reminiscent of the benign recurrent intrahepatic cholestasis phenotype. Despite diversion of bile from the intestinal lumen, weight gain improved while fat‐soluble vitamin requirements persisted. Significant progression of liver disease was not evident during follow‐up.
Capillary Flow Rates in the Duodenum of Pediatric Ulcerative Colitis Patients Are Increased and Unrelated to Inflammation
Authors: Zaidi Deenaz , Churchill Lucas , Huynh Hien Q. , Carroll Matthew W. , Persad Rabin , Wine Eytan 
Published: December 2016

ABSTRACTBackground and Aim:Inflammatory bowel diseases (IBD), including Crohn disease (CD) and ulcerative colitis (UC), are chronic pediatric disorders. Changes in vasculature are described in IBD, but these could be secondary to inflammation and the role in pathogenesis is poorly understood. Assessing circulatory changes in typically unaffected sites in IBD (eg, duodenum), when inflammation is absent, can identify vascular changes associated with pathogenesis. The aim of the study is to measure capillary flow rates in duodenal mucosa using probe‐based confocal laser endomicroscopy (pCLE) during endoscopy in children with IBD.Methods:Images of villi with visible blood vessels obtained using pCLE were captured as video sequences. Capillary flow rate (mm/s) was calculated by dividing the distance travelled by blood cells by the duration of the sequence. Flow rates were correlated with various clinical parameters.Results:Forty‐five patients (22 non‐IBD, 14 CD, 9 UC) were included in the study. Duodenal capillary flow rates were significantly higher in UC patients (0.75 ± 0.07 mm/s) as compared to non‐IBD (0.57 ± 0.03) and CD (0.65 ± 0.04). There was no correlation between serum hemoglobin and albumin, disease activity indices, serum inflammatory markers, and capillary flow rates in patients.Conclusions:This pilot study shows, for the first time, increased capillary blood flow in the duodenum of UC patients that was unrelated to inflammatory markers or disease activity. Thus, early vascular changes can be assessed using pCLE during endoscopy.
Diverting Ileostomy for the Treatment of Severe, Refractory, Pediatric Inflammatory Bowel Disease
Authors: Maxwell Elizabeth C. , Dawany Noor , Baldassano Robert N. , Mamula Petar , Mattei Peter , Albenberg Lindsey , Kelsen Judith R. 
Published: December 2016

ABSTRACTObjectives:Diverting ileostomy is used as a temporizing therapy in patients with perianal Crohn disease; however, little data exist regarding its use for colonic disease. The primary aim of the present study was to determine the role of diversion in severe refractory colonic inflammatory bowel disease (IBD) in a pediatric population.Methods:Retrospective study of patients who underwent diverting ileostomy at The Children's Hospital of Philadelphia from 2000 to 2014 for the management of severe, refractory colonic IBD. Clinical variables were compared in the 1 year before ileostomy and 1 year after diversion. Surgical and disease outcomes including changes in diagnosis were reviewed through 2015.Results:Twenty‐four patients underwent diverting ileostomy for refractory colonic disease. Initial diagnoses were Crohn disease in 10 (42%), ulcerative colitis in 1 (4%), and IBD‐unclassified in 13 patients (54%). Comparing data before and after surgery, there were statistically significant improvements in height and weight velocities, height velocity z score, blood transfusion requirement, hemoglobin, and hospitalization rates. Chronic steroid use decreased from 71% to 22%. At the conclusion of the study, 10 patients had undergone subsequent colectomy, 7 had successful bowel reanastomosis, and 7 remain diverted. Seven patients (29%) had a change in diagnosis. There were 13 surgical complications in 7 subjects, including prolapse reduction, stoma revision, and resection of ischemic bowel.Conclusions:In pediatric patients with refractory colonic IBD, diverting ileostomy can be a successful intervention to induce clinical stability. Importantly, diversion is a steroid‐sparing therapy and allows additional time to clarify the diagnosis.
Nutritional Practices and Growth in Premature Infants After Surgical Necrotizing Enterocolitis
Authors: Lin Grace C. , Robinson Daniel T. , Olsen Steven , Reber Kristina M. , Moallem Mohannad , DiGeronimo Robert , Mulroy Cecilia , Datta Ankur , Murthy Karna 
Published: December 2016

ABSTRACTObjective:The aim of the study was to describe the nutritional provisions received by infants with surgical necrotizing enterocolitis (NEC) and the associated effects on short‐term growth.Methods:Through the Children's Hospitals Neonatal Database, we identified infants born ⩽32 weeks’ gestation with surgical NEC from 5 regional neonatal intensive care units for 4 years. Excluded infants had isolated intestinal perforation and died <14 days postoperatively. Infants were stratified by their median parenteral protein dose (low [LP] or high [HP] protein) for the first postoperative week. The primary outcome was postoperative weight growth velocity. Growth (weight, length, and head circumference [HC]) was measured and the effects related to protein dose were estimated using multivariable analyses.Results:There were 103 infants included; the median parenteral protein dose received was 3.27 g · kg−1 · day−1 (LP: 2.80 g · kg−1 · day−1; HP: 3.87 g · kg−1 · day−1). Postoperative weight (11.5 ± 6.5 g · kg−1 · day−1) and linear growth (0.9 ± 0.2 cm/wk) were similar regardless of dose (P > 0.3 between groups for weight and length). Unadjusted and independent associations were identified with HC changes and HP dose (β = 0.1 cm/wk, P = 0.03) after adjusting for gestational age, the presence of severe bronchopulmonary dysplasia, short bowel syndrome, blood stream infection, severe intraventricular hemorrhage, small for gestational age, and calorie intake. Eventual nonsurvivors received 18% less protein and 14% fewer calories over the first postoperative month.Conclusions:Postoperative protein doses in infants with surgical NEC appear related to increases in HC. The influence of postoperative nutritional support on risk of adverse outcomes deserves further attention.
Autophagy in Hepatocytes in Infants With Alpha‐1 ATD and Different Liver Disease Outcomes
Authors: Czarnowska Elżbieta , Bakuła Agnieszka , Bierła Joanna B. , Niderla‐Bielińska Justyna , Sowińska Agnieszka , Cukrowska Bożena , Cielecka‐Kuszyk Joanna , Socha Piotr 
Published: December 2016

ABSTRACTObjectives:It is unclear whether a distinct activity of pathways removing the antitrypsin (AT) protein in Alpha‐1‐Antitrypsin Deficiency (α1ATD) are associated with an unfavorable predisposition to liver disease in the future. The aim of this study was to determine whether liverspecific activity of AT protein disposal occurs at infancy in α1ATD with PiZZ phenotype (ATZ).Methods:Liver samples of 17 infants with unfavorable ATZ outcome (Group I, n = 8, median age = 0.35 year) and good outcome (Group II, n = 9, 0.17 year), and 9 with biliary atresia (BA, median age = 0.17 year) as control, were enrolled. For each subject were investigated autophagy activity by mRNA, protein expression (Calnexin, Beclin‐1, p62, and Parkin), and hepatocyte ultrastructure with morphometric analyses.Results:No significant differences in gene expression in the liver of infants were found between the 2 ATZ groups. Although a correlation between patients’ age and protein expression was observed, the ATZ groups differed Parkin immunohistochemical expression. Moreover, the hepatocytes in ATZ infants with unfavorable outcome were characterized by low Parkin expression and the presence of isolated mitophagosoms and numerous enlarged mitochondria. The mentioned findings differed in patients with BA.Conclusions:Thus, mentioned specific features occurring at infancy may suggest association with poor liver outcome. Parkin low expression could have a potential for disease prognosis and treatment; however, further studies in a greater number of patients are needed.
Study of Carnitine/Acylcarnitine and Amino Acid Profile in Children and Adults With Acute Liver Failure
Authors: Sood Vikrant , Rawat Dinesh , Khanna Rajeev , Sharma Shvetank , Gupta Prem K. , Alam Seema , Sarin Shiv Kumar 
Published: December 2016

ABSTRACTObjectives:Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and amino acid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and amino acid profile in ALF.Methods:A prospective study was performed including all patients with ALF, and detailed evaluation including metabolic testing was done.Results:A total of 55 patients (33 pediatric and 22 adult patients) were included in the study. Three patients (a 1‐year 6‐month‐old child, a 13‐year‐old adolescent, and a 21‐year‐old adult, ie, 5.5% of all) were identified for the study with underlying metabolic etiology, that is, carnitine palmitoyl transferase‐1 deficiency, based on the abnormal carnitine/acylcarnitine profile. Almost three‐fourths of patients (78%) had evidence of serum hyperaminoacidemia. Thirty‐one patients (56%) had evidence of abnormal carnitine/acylcarnitine profile with predominant abnormality being low free carnitine (C0). Higher levels of serum tyrosine (P = 0.002) and lower levels of serum C0 (P = 0.032) in children and higher levels of serum phenyalanine (P = 0.047) in adults predicted poor outcome (death/liver transplant) on univariate analysis.Conclusions:FAODs are not uncommon in ALF with a suggested prevalence of approximately 5.5%. FAODs can cause ALF or modify the natural course of ALF caused by other etiologies. Serum hyperaminoacidemia and low serum free carnitine may predict poor outcome in patients with acute liver failure.
Longitudinal Study of Posttraumatic Stress Disorder in the Community
Authors: Pejuskovic Bojana , Lecic-Tosevski Dusica , Toskovic Oliver 
Published: December 2016

Abstract
Posttraumatic stress disorder (PTSD) is a multicausal phenomenon and a final end point of the combination of a number of potential causes. Our study aimed to examine potential risk and recovery factors of PTSD in general adult population at 1-year follow-up period. The sample consisted of 640 subjects in the initial phase, chosen by random walk technique in five regions of the country, and 100 in the follow-up. The assessment has been carried out by the following instruments: Mini-International Neuropsychiatric Interview, Life Stressor Checklist-Revised, Brief Symptom Inventory, and Manchester Short Assessment of Quality of Life Scale. Older age, low education, and lower monthly income are potential risk factors for current PTSD, as well as decreased quality of life, psychiatric comorbidity, and higher personal distress. Urban population, higher quality of life, smaller number of stressors, and lower personal distress contributed to recovery of PTSD. It is essential to know the risk and resilience factors that contribute to the development and recovery of PTSD, which is important for prevention and treatment of this disorder.
Healing Through Words: Jerome Frank and Psychotherapy at Johns Hopkins
Authors: Jamison Kay Redfield 
Published: December 2016

Abstract
This lecture, given to celebrate the centenary of the founding of the Henry Phipps Psychiatric Service at Johns Hopkins, focuses on the contributions of Jerome Frank to the understanding and practice of psychotherapy. It gives an overview of Professor Frank's ideas and work, puts them into the context of the history of psychotherapy research at Johns Hopkins, and discusses psychotherapy and demoralization in bipolar illness.
Role of Survival Time and Injury Severity in Fatal Pulmonary Fat Embolism
Authors: Jarmer Juliane , Ampanozi Garyfalia , Thali Michael J. , Bolliger Stephan A. 
Published: December 2016

Abstract
Pulmonary fat embolism (PFE) is frequent in blunt trauma and may occasionally lead to death. A correlation between fracture grade and severity and PFE grade has been described before, but no correlation between PFE and survival time, fat crushing extent, fat crush grade, or number of body regions with fractures could be noted in this small study. To further examine this, we decided to examine the aforementioned points in a far larger study group.
Autopsy protocols of 188 nonresuscitated fatalities with blunt trauma and without right heart injury, which underwent whole body dissection, were retrospectively reviewed concerning the presence and the severity of PFE, injuries, survival time, age, sex, and the body mass index.
The fracture grade, the fracture severity, and the number of the fractured regions correlated very well with the grade of PFE, but the crushed regions, crush grade, and crush severity did not. We observed a time correlation between survival time and PFE only in the sense that very rapid deaths were often PFE negative. High-grade PFE was observed most often in patients having died less than 6 hours after the incident, and PFE grades of 2 or more were occasionally noted even after 48 hours.
Exploring the Experiences of Women and Health Care Workers in the Context of PMTCT Option B Plus in Malawi
Authors: Cataldo Fabian , Chiwaula Levison , Nkhata Misheck , van Lettow Monique , Kasende Florence , Rosenberg Nora E. , Tweya Hannock , Sampathkumar Veena , Hosseinipour Mina , Schouten Erik , Kapito-Tembo Atupele , Eliya Michael , Chimbwandira Frank , Phiri Sam ,   for the PURE Malawi Consortium
Published: December 2016

Introduction:
Malawi has embarked on a “test-and-treat” approach to prevent mother-to-child transmission (PMTCT) of HIV, known as “Option B+,” offering all HIV-infected pregnant and breastfeeding women lifelong antiretroviral therapy (ART) regardless of CD4 count or clinical stage. A cross-sectional qualitative study was conducted to explore early experiences surrounding “Option B+” for patients and health care workers (HCWs) in Malawi.

Methods:
Study participants were purposively selected across 6 health facilities in 3 regional health zones in Malawi. Semi-structured interviews were conducted with women enrolled in “Option B+” (n = 24), and focus group discussions were conducted with HCWs providing Option B+ services (n = 6 groups of 8 HCWs). Data were analyzed using a qualitative thematic coding framework.

Results:
Patients and HCWs identified the lack of male involvement as a barrier to retention in care and expressed concerns at the rapidity of the test-and-treat process, which makes it difficult for patients to “digest” a positive diagnosis before starting ART. Fear regarding the breach of privacy and confidentiality were also identified as contributing to loss to follow-up of women initiated under the Option B+. Disclosure remains a difficult process within families and couples. Lifelong ART was also perceived as an opportunity to plan future pregnancies.

Conclusions:
As “Option B+” continues to be rolled out, novel interventions to support and retain women into care must be implemented. These include providing space, time, and support to accept a diagnosis before starting ART, engaging partners and families, and addressing the need for peer support and confidentiality.
Hepatic Histology and Morphometric Measurements in Idiopathic Extrahepatic Portal Vein Thrombosis in Children, Correlated to Clinical Outcome of Meso-Rex Bypass
Authors: Tantemsapya Niramol , Superina Riccardo , Wang Deli , Kronauer Grace , Whitington Peter F. , Melin-Aldana Hector 
Published: December 2016

Objective:
The aim of this study was to correlate clinical, histologic, and morphometric features of the liver in children with extrahepatic portal vein thrombosis (EHPVT), with surgical outcome after Meso-Rex bypass (MRB).

Background:
Idiopathic EHPVT, a significant cause of portal hypertension, is surgically corrected by MRB. Correlation of histologic and morphometric features of the liver with outcome has not been reported in children.

Methods:
We retrospectively reviewed clinical and intraoperative data of 45 children with idiopathic EHPVT. Liver samples were obtained at the time of MRB. Morphometric measurements of portal tract structures were performed and correlated with surgical outcome. Median follow-up was 3.65 years after surgery (range 1.5 to 10 years).

Results:
Thirty-seven (82.2%) children had successful MRB. There was no association between age, sex, and suture material with surgical outcome. Average patient age was higher in patients with postoperative complications (P = NS). Portal fibrosis, bridging, parenchymal nodules, portal inflammation, hepatocellular swelling, steatosis, dilatation of portal lymphatics, and periductal fibrosis did not show a significant difference between the 2 groups. Portal vein and bile duct area index were significantly smaller in the unsuccessful group (P = 0.004 and 0.003, respectively). A portal vein area index <0.08 had a lower chance of successful surgical outcome. Hepatic artery area index was not significantly different. Measured intraoperative portal blood inflow was the only significant clinical factor affecting surgical outcome (P = 0.0003).

Conclusions:
Low portal vein area index and intraoperative portal blood inflow may be negative prognostic factors for MRB outcome in children with idiopathic EHPVT. Average patient age was higher, although not statistically significant, in patients with postoperative complications.
Quantitative EEG After Brain Stimulation and Cognitive Training in Alzheimer Disease
Authors: Gandelman-Marton Revital , Aichenbaum Sergio , Dobronevsky Evgenya , Khaigrekht Michael , Rabey Jose M. 
Published: December 2016

Purpose:
Medications are the currently accepted symptomatic treatment of Alzheimer disease (AD), but their impact on delaying the progression of cognitive deficits and functional impairment is limited. The authors aimed to explore long-term electrophysiological effects of repetitive transcranial magnetic stimulation interlaced with cognitive training on quantitative electroencephalography (EEG) in patients with AD.

Methods:
Quantitative EEG was assessed on non–repetitive transcranial magnetic stimulation interlaced with cognitive training treatment days before treatment and after each treatment phase in seven patients with mild AD.

Results:
After 4.5 months (54 sessions) of treatment, a significant increase of delta activity over the temporal region was found compared with pretreatment values. Nonsignificant increases of the log EEG power were found for alpha band over the frontal and temporal regions, beta band over the frontal region, theta band over the frontal, temporal, and parieto-occipital regions, and delta band over the frontal and parieto-occipital regions. Nonsignificant decreases were found for alpha over the parieto-occipital region, and for beta over the temporal and parieto-occipital regions. A positive correlation was found between log alpha power over the frontal and temporal regions at 6 weeks and Mini–Mental State Examination (MMSE) scores at 6 weeks and 4.5 months, and between log alpha power over the parieto-occipital regions and MMSE scores at 6 weeks. A negative correlation was found between log alpha power over the frontal and temporal regions at 6 weeks and baseline Alzheimer's Disease Assessment Scale-cognitive subscale scores.

Conclusions:
Repetitive transcranial magnetic stimulation interlaced with cognitive training has long-term effects on quantitative EEG in patients with mild AD. Further research on the quantitative EEG long-term effects of transcranial magnetic stimulation interlaced with cognitive training is required to confirm the authors' data.
A Multiparametric Alarm Criterion for Motor Evoked Potential Monitoring During Spine Deformity Surgery
Authors: Segura Martín J. , Talarico María E. , Noel Mariano A. 
Published: December 2016

Purpose:
This is a pilot study to compare changes in the amplitude, area below the curve, number of phases, duration, and latency of the intraoperative transcranial motor evoked potentials (TcMEP) for early detection of impending spinal cord injury. An empirical ratio calculated by a combination of the above-mentioned parameters was also assessed.

Methods:
Intraoperative TcMEP recordings from five patients presenting with neuromuscular kyphoscoliosis, idiopathic scoliosis, achondroplasia and lumbar kyphosis, congenital kyphosis, and achondroplasia with cervical instability were reviewed. Anesthesia was sustained with propofol or sevoflurane plus remifentanil, no muscle relaxants were used after intubation. The TcMEPs to multipulse electrical stimulation were recorded from upper-limb and lower-limb muscles. To be worthy of analyses, changes should include TcMEP disappearance or amplitude decrease >−65% during surgical/force maneuverings.

Results:
Transient TcMEP changes related to surgical/force maneuvering were observed in all five patients consisting of a decrease in the empirical ratio value (>−95%), followed in magnitude by a drop in the area below the curve, amplitude (>−80%), duration, and number of phases, whereas latency increased. Changes returned to baseline when maneuverings were reverted. No hemodynamic/anesthetic factors were present during these events. After surgeries, no new neurological deficits were detected.

Conclusions:
An empirical ratio accounting for all TcMEP parameters seems to drop more than the amplitude during an intraoperative event. As few cases were analyzed, further studies in larger series of patients will be necessary to assess empirical ratio sensitivity/specificity and to determine whether this may be a useful monitoring warning criterion not leading to unnecessary interference with surgical treatment.