bluuebunny/crossref_metadata_2025_split huggingface dataset:
Starch Malabsorption in Infants
Authors: Shulman Robert J. 
Published: May 2018

ABSTRACTBased on the developmental physiology of pancreatic amylase production, starch digestion in young infants was anticipated to be compromised whenever compared with that in older infants and toddlers. This appears to be the case, but with great variability among infants to digest starch. Evidence points to the importance of maltase‐glucoamylase in young infants and its effect on starch digestion. These observations have critical importance for recommendations regarding the feeding of starch‐containing foods to young infants.
Structural Studies of the Intestinal α‐Glucosidases, Maltase‐glucoamylase and Sucrase‐isomaltase
Authors: Rose David R. , Chaudet Marcia M. , Jones Kyra 
Published: May 2018

ABSTRACTObjectives:Maltase‐glucoamylase and sucrase‐isomaltase are enzymes in the brush‐border membrane of the small intestinal lumen responsible for the breakdown of postamylase starch polysaccharides to release monomeric glucose. As such, they are critical players in healthy nutrition and their malfunction can lead to severe disorders.Methods:This review covers investigations of the structures and functions of these enzymes.Results:Each consists of 2 enzyme domains of the glycoside hydrolase family GH31 classification, yet with somewhat differing enzymatic properties.Conclusions:Crystallographic structures of 3 of the domains have been published. Insights into substrate binding and specificity will be discussed, along with future lines of inquiry related to the enzymes’ roles in disease and potential avenues for therapeutics.
Starch Tolerance and the Short Bowel
Authors: de Laffolie Jan , Naim Hassan Y. , Rudloff Silvia , Zimmer Klaus‐Peter 
Published: May 2018

ABSTRACTShort bowel syndrome with intestinal failure is a rare disease with a massive impairment in quality of life, requiring a multidisciplinary team approach to medical, surgical, and nutritional therapy. Current pharmacological and surgical therapeutic options are limited; an important cornerstone is enteral and parenteral nutrition. The changed physiology of carbohydrate digestion plays a major role in the adaptation process and can be a target for specific enteral nutrition interventions. An important prognostic factor is the preservation of at least portions of the colon in continuity with small bowel. This strategy has to include an evaluation of the anatomical situation and small bowel absorptive capacity, adaptation processes, and luminal microbiota including its fermentative properties. Starch is probably the most important complex carbohydrate in short bowel syndrome nutrition, because it is absorbed or fermented almost completely. Benefits of supplementation with complex carbohydrates include improved adaptive processes, positive trophic effects on the mucosa and its hormonal response, longer transit time, and possibly a faster time to wean from parenteral nutrition, but supplementation advice needs to weigh carefully the risks and benefits, especially considering bacterial overgrowth, osmotic load, and D‐lactate acidosis.
Molecular Regulations of Mucosal Maltase Expressions
Authors: Goda Toshinao , Honma Kazue 
Published: May 2018

ABSTRACTTwo major α‐glucosidase (maltase) genes, sucrase‐isomaltase (SI) and maltase‐glucoamylase (MGAM), respectively, are expressed in the small intestine. In this review, we have summarized whether jejunal expression of these maltase genes is regulated by dietary manipulations, which may affect carbohydrate availability from the luminal side, through changes in the binding of transcription factors and/or histone code on these genes. Studies using a model of mice fed either a low‐starch or a high‐starch diet for 7 days, found the mRNA levels of SI, MGAM, and Na+‐glucose cotransporter (SGLT1) genes in the jejunum to be increased in parallel by feeding a high‐starch diet. Chromatin immunoprecipitation assays, using jejunal tissue of mice and rats fed a high‐starch diet, revealed that the diet increased the acetylations of histones H3 and H4, bindings of coactivators, including general control of amino acid synthesis (GCN5) and the transcriptional factors, including caudal‐related homeobox 2 (CDX2), and hepatocyte nuclear factor 1 (HNF1), not only in the promoter/enhancer regions, but also in the transcribed regions of SI and MGAM genes. Feeding rats a diet rich in resistant starch led to a concomitant reduction of mRNA levels of the MGAM gene and histone H3 modifications (acetylations and di‐/tri‐methylations) in the jejunum. These data suggest that a signal elicited by available glucose in the jejunal mucosa is associated with SI and MGAM gene expressions through a histone code, such as acetylation and di‐/tri‐methylations of histone H3 in the promoter/enhancer and transcribed regions of SI and MGAM genes.
Quality and Safety in Health Care, Part XLI
Authors: Harolds Jay A. 
Published: May 2018

Abstract
The IMPACT Registry is a repository of information for heart catheterizations for congenital heart disease regardless of age and also the catheterizations for acquired heart disease in children. The registry collects information on outcomes, provides quality improvement opportunities for participants, provides reports to participants comparing their results with national results, compares the volume of catheter work done at an institution with the frequency of adverse events, and provides information that may be helpful in evaluating the use of medical devices and treatment options.
Quality and Safety in Health Care, Part XL
Authors: Harolds Jay A. 
Published: May 2018

Abstract
Percutaneous coronary interventions (PCI) are done for a variety of indications. Data from the CathPCI Registry is highly useful to evaluate the outcomes from performing a PCI procedure in unusual circumstances, such as in cases of chronic total coronary artery occlusion, cardiogenic shock after a myocardial infarction, syncopal patients, asymptomatic patients, and before surgery that is noncardiac. Registry information is also valuable in evaluating the efficacy of coronary stents. This article summarizes some of the information available on these subjects.
Acute Pancreatitis Following Peptide Receptor Radionuclide Therapy
Authors: Karfis Ioannis , Marin Gwennaëlle , Machiels Godelieve , Hendlisz Alain , Flamen Patrick 
Published: May 2018

Abstract
A 54-year-old man with grade 2 rectal neuroendocrine tumor and hepatic, pancreatic, and bone metastases was treated with 177Lu-DOTATATE as second-line therapy, after failure of somatostatin analogues. Two weeks after the first injection, he presented at the emergency department with acute pancreatitis. We hypothesized that this unusual adverse event, never been reported so far, was the result of acute tumor irradiation after PRRT, leading to peritumoral inflammation and edema with obstruction of an accessory pancreatic duct.
Growth Plate Suppression in an Adolescent Patient With Acute Lymphoblastic Leukemia After Treatment
Authors: Tiktinsky Ekaterina , Levin Daniel , Lantsberg Sophia , Kapelushnik Joseph 
Published: May 2018

Abstract
Osteotoxic effect is a common adverse effect of chemotherapy for childhood acute lymphoblastic leukemia. The pathophysiology of impaired bone growth is multifactorial and can affect both osteoblast and osteoclast function. Significant contribution in affecting skeletal metabolism belongs to high-dose corticosteroid treatment. We present the case of a 12-year-old adolescent girl who was treated for high-risk pro-B acute lymphoblastic leukemia. The Tc-MDP bone scan, as a sensitive indicator of osteoblastic activity, shows that growth plate inhibition after intensive treatment may be temporary and reversible.
Diagnostic Performance of 18F-Fluciclovine in Detection of Prostate Cancer Bone Metastases
Authors: Chau Albert , Gardiner Peter , Colletti Patrick M. , Jadvar Hossein 
Published: May 2018

Purpose

18F-fluciclovine is a synthetic amino acid radiotracer that has recently been approved in Europe and the United States for PET imaging in men with biochemical recurrence (BCR) of prostate cancer after prior definitive treatment. Accurate identification of the sites of disease in patients presenting with BCR of prostate cancer is important in determining the appropriate treatment. Bone is the most frequent site of metastatic disease in patients with prostate cancer.

Methods
We conducted a comprehensive review of the available preclinical and clinical data on the diagnostic performance of 18F-fluciclovine PET/CT in an attempt to draw practical and general conclusions on the utility and limitations of 18F-fluciclovine PET/CT in localization of osseous metastatic disease in prostate cancer.

Results
The cumulative preclinical data and results of some retrospective and 2 prospective clinical studies suggest that 18F-fluciclovine can detect early bone marrow involvement in patients with BCR of prostate cancer and negative prior bone-specific imaging findings.

Conclusions

18F-fluciclovine PET/CT seems to offer useful information for early detection of bone metastases in men with BCR of prostate cancer. Additional investigations will be needed to compare the diagnostic performance of 18F-fluciclovine PET/CT to other standard and novel imaging methods in initial staging, BCR, and castrate-resistant phases of disease.
Primary Congenital Lymphedema
Authors: Mrhac Lubomir , Bukovansky Kamil , Kafkova Jana , Kraft Otakar 
Published: May 2018

Abstract
We present the case of a 26-year-old man with congenital primary lymphedema. A lymphedema of the right upper limb appeared at the age of 2 years, followed by edemas of the right lower limb and of the right part of the head and neck. A lymphedema of the left lower limb began 5 years ago, and finally an edema of the external genitalia began 2 years ago. Lymphoscintigraphy of the lower limbs and pelvic/abdominal region (including SPECT/ldCT) revealed interesting abnormalities.
Preoperative Analysis of Venous Anatomy Before Deep Inferior Epigastric Perforator Free-Flap Breast Reconstruction Using Ferumoxytol-enhanced Magnetic Resonance Angiography
Authors: Dortch John , Forte Antonio J. , Bolan Candice , Kandel Pujan , Perdikis Galen 
Published: May 2018

Background
Venous congestion after deep inferior epigastric artery perforator (DIEP) flap breast reconstruction is a complication that may be partially attributable to variations in venous abdominal wall anatomy. In previous work, we have shown that ferumoxytol may be used as a bloodpool contrast agent to perform high-resolution venous imaging. Our current aim was to use this technology to perform a detailed analysis of the venous anatomy among patients undergoing DIEP flap breast reconstruction.

Methods
All patients undergoing DIEP flap reconstruction with preoperative ferumoxytol-enhanced magnetic resonance angiography (FE-MRA) were retrospectively reviewed. A detailed anatomic analysis of each abdominal wall on FE-MRAwas performed before review of operative findings. Statistical analysis was used to determine venous characteristics associated with superficial inferior epigastric vein (SIEV) augmentation.

Results
From 2012 to 2016, 59 patients underwent preoperative FE-MRA. This resulted in imaging for 118 hemiabdomen and 99 flaps. Superficial-deep communication was identified in 117 of 118 hemiabdomen. Fifty (93%) of 59 patients had greater than 1-mm venous communication of the superficial system across midline. Reconstructed breasts were based on dominant medial row perforators in 82 (83%) of 99 flaps. The mean diameters of the SIEVand dominant venous perforator were 3.8 and 2.8mm, respectively. Anatomic characteristics associated with SIEVaugmentation included SIEVdiameter (P = 0.01), dominant perforator diameter (P = 0.04), and the ratio between these 2 variables (P = 0.001).

Conclusions
Ferumoxytol-enhanced magnetic resonance angiography provides excellent imaging of the venous system. Anatomic characteristics such as the diameter of the SIEVand the diameter of the dominant perforator may be useful in determining which flaps require venous augmentation using the SIEV.
Primary Premaxillary Ostectomy and Setback
Authors: Farhadi Rana , Wallace Robert D. 
Published: May 2018

Objective
This study aimed to analyze our outcomes of primary premaxillary ostectomy and setback combined with lip adhesion as the first stage in repair of the bilateral cleft lip with fly-away premaxilla.

Methods
This retrospective study included all patients who required primary premaxillary setback by a single surgeon to achieve successful lip repair between 2011 and 2017 at a tertiary children's hospital. All patients had bilateral cleft lip and complete cleft palate with significant premaxillary protrusion. All patients either had failed presurgical orthodontic manipulation or were thought to be poor candidates because of the rigidity of the premaxilla on clinical examination. Patient data were obtained from review of medical records and photographs. Surgical technique is described.

Results
Eleven patients with bilateral cleft lip, all of whom also had complete cleft palate, underwent primary premaxillary setback and lip adhesion at an average age of 8.2 months. The mean follow-up was 1.9 years (range, 139 days to 4.5 years). All patients subsequently completed formal lip repair, of which 10 have undergone palatoplasty. Serial photography documented stable premaxillary position and successful lip repair within the follow-up time frame. No patient had postoperative premaxillary instability or vascular compromise. One patient had dehiscence of the lip adhesion requiring reoperation.

Conclusions
Primary premaxillary ostectomy and setback allows for successful staged formal lip repair in patients with bilateral cleft lip and palate who have failed presurgical infant orthopedics. Further research is required regarding longterm follow-up with respect to midfacial growth.
Endoscopically Assisted Modified Caldwell-Luc Approach to Enucleation of Dentigerous Cyst With Ectopic Tooth From the Maxillary Sinus
Authors: Liau Isaac , Lynch Nicholas , Hearn Brenton , Cheng Andrew 
Published: May 2018

Abstract
Dentigerous cysts in the craniomaxillofacial complex are a benign pathology commonly associated with ectopically placed teeth. Owing to slow and asymptomatic growth, they often attain considerable size before diagnosis that may compromise adjacent anatomical structures. Various techniques for removal of dentigerous cysts from the maxillary sinus have been described. This case report demonstrates a hybrid endoscopically assisted modification of the Caldwell-Luc approach to provide the technical advantages of both techniques
Maternal Folic Acid Supplementation and the Risk of Oral Clefts in Offspring
Authors: Jahanbin Arezoo , Shadkam Elaheh , Miri Hamid Heidarian , Shirazi Alireza Sarraf , Abtahi Mostafa 
Published: May 2018

Introduction:
There is controversial evidence from the literature regarding the protective effect of folic acid supplementation during pregnancy against orofacial clefts. The authors undertook this meta-analysis to assess whether folate supplementation during pregnancy can reduce the risk of nonsyndromic cleft lip with or without cleft palate (CL/P) and cleft palate only (CPO) in infants.

Methods:
Eligible articles were identified by searching databases, including PubMed, Medline, Scopus, ISI (Web of Knowledge) to September 2017. A meta-analysis was performed to evaluate the effects of maternal supplementation on oral clefts. Odds ratios (ORs) and 95% confidence intervals (CIs) were pooled using Stata software. Publication bias was assessed by the Begg and Egger test. (Registration ID: CRD42018083922)

Results:
Out of the 1630 articles found in the authors’ initial literature searches, 6 cohort studies, and 31 case-control studies were included in the authors’ final meta-analysis. The results of the main analysis revealed that maternal folate supplementation was associated with a modest but statically significant decreased risk of all cleft subtypes (OR = 0.69, 95% CI: 0.60, 0.78). Folic acid intake alone was inversely associated with CL/P (OR = 0.73, 95% CI: 0.62–0.85,) but to a lesser extent than CPO (OR = 0.75, 95% CI = 053–1.04). Multivitamin intake had a significant protective effect for CL/P (OR = 0.65 95% CI = 0.55–0.80) as well as CPO (OR = 0.69, 95% CI = 0.53–0.90).

Conclusions:
Our results indicate that maternal supplementation in early pregnancy reduces the risk of nonsyndromic CL/P and CPO in infants. These data can serve to reassure women planning a pregnancy to consume multivitamins during the periconception period to protect against oral clefts.
Nodular Tenosynovitis of the Temporomandibular Joint: An Entity Not Yet Described
Authors: Cariati Paolo , Garcia-Martin Miguel , Perez de Perceval Tara Miguel , Roman Ramos Maria , Garcia Medina Blas 
Published: May 2018

Abstract
Nodular tenosynovitis usually affects the hands and it represents a benign pathology with locally aggressive behavior. Its etiology could be related to chronic inflammatory processes such as trauma, metabolic disturbance, and joint diseases. Histopathological analysis is required for a diagnosis of certainty and surgery represents the treatment of choice. There are no cases in the literature that describe a nodular tenosynovitis affecting the temporomandibular joint (TMJ) The main aim of the present report therefore, is to describe this unusual case and to show the utility of arthroscopic procedures in managing intra-articular tumors of the TMJ.
Rare Location for Pilonidal Sinus: the Nasal Dorsum
Authors: Kars Ayhan , Atalay Fatma , Kilic Korhan , Bingöl Fatih , Can Yusuf 
Published: May 2018

AbstractPilonidal sinuses are recurrent chronic inflammatory lesions which may occur due to penetration of hair particles into skin. Herein, the authors report a pilonidal sinus case that is unusually seen on nasal dorsum and totally excised with the open technique rhinoplasty method. A 20-year-old male patient was admitted to the authors’ outpatient clinic with complaints of dysmorphism and discharge from nasal dorsum. Physical examination revealed a swelling in nasal dorsum and hair-containing fistula. Excision was performed with an open rhinoplasty approach. Histo-pathology examination revealed pilonidal sinus. While pilonidal sinus is usually located in sacro-coccygeal region, it may also be seen in atypical localizations like nasal dorsum. Although the prediagnosis of a hair-containing lesion usually includes dermoid cyst, pilonidal sinus should also be considered and histo-pathological examination should certainly be performed. It is a problematic condition when it is symptomatic; however, management and treatment of the disease is easy when correct diagnosis is made.
Treatment of Craniosynostosis: The Impact of Hospital Surgical Volume on Cost, Resource Utilization, and Outcomes
Authors: Chattha Anmol , Bucknor Alexandra , Curiel Daniel A. , Ultee Klaas H.J. , Afshar Salim , Lin Samuel J. 
Published: May 2018

Abstract
The authors aim to quantify the impact of hospital volume of craniosynostosis surgery on inpatient complications and resource utilization using national data. Children <12 months with nonsyndromic craniosynostosis who underwent surgery in 2012 at academic hospitals in the United States were identified from the Kids’ Inpatient Database (KID) developed by the Healthcare Cost and Utilization Project (HCUP). Hospital craniosynostosis surgery volume was stratified into tertiles based on total annual hospital cases: low volume (LV, 1–13), intermediate volume (IV, 14–34), and high volume (HV, ≥35). Outcomes of interest include major complications, blood transfusion, charges, and length of stay (LOS). In 2012, 154 hospitals performed 1617 total craniosynostosis surgeries. Of these 580 cases (35.8%) were LV, 549 cases (33.9%) were IV, and 488 cases (30.2%) were HV. There was no difference in major complications between hospital volume tertiles (4.3% LV; 3.8% IV; 3.1% HV; P = 0.487). The highest blood transfusion rates were seen at LV hospitals (47.8% LV; 33.9% IV; 26.2%; P < 0.001). Hospital charges were lowest at HV hospitals ($55,839) compared with IV hospitals ($65,624; P < 0.001) and LV hospitals ($62,325; P = 0.005). Mean LOS was shortest at HV hospitals (2.96 days) compared with LV hospitals (3.31 days; P = 0.001); however, there was no difference when compared with IV hospitals (3.07 days; P = 0.282). Hospital case volume may be an important associative factor of blood transfusion rates, LOS, and hospital charges; however, there is no difference in complication rates. These results may be used to guide quality improvement within the surgical management of craniosynostosis.
Alzheimer's disease neuroimaging
Authors: Whitwell Jennifer L. 
Published: May 2018

Purpose of review
The aim of this study was to discuss the contribution of neuroimaging studies to our understanding of Alzheimer's disease. We now have the capability of measuring both tau and beta-amyloid (Aβ) proteins in the brain, which together with more traditional neuroimaging modalities, has led the field to focus on using neuroimaging to better characterize disease mechanisms underlying Alzheimer's disease.

Recent findings
Studies have utilized tau and Aβ PET, as well as [18F]fluorodeoxyglucose PET, and structural and functional MRI, to investigate the following topics: phenotypic variability in Alzheimer's disease , including how neuroimaging findings are related to clinical phenotype and age; multimodality analyses to investigate the relationships between different neuroimaging modalities and what that teaches us about disease mechanisms; disease staging by assessing neuroimaging changes in the very earliest phases of the disease in cognitively normal individuals and individuals carrying an autosomal dominant Alzheimer's disease mutation; and influence of other comorbidities and proteins to the disease process.

Summary
The findings shed light on the role of tau and Aβ, as well as age and other comorbidities, in the neurodegenerative process in Alzheimer's disease. This knowledge will be crucial in the development of better disease biomarkers and targeted therapeutic approaches.
A web bench for analysis and prediction of oncological status from proteomics data of urine samples
Authors: Bhalla Sherry , Chaudhary Kumardeep , Gautam Ankur , Sharma Suresh , Raghava Gajendra P. S. 
Published: May 2018

AbstractUrine-based cancer biomarkers offer numerous advantages over the other biomarkers and play a crucial role in cancer management. In this study, an attempt has been made to develop proteomics-based prediction models to discriminate patients of oncological disorders related to urinary tract and healthy controls from their urine samples. The dataset used in this study was obtained from human urinary peptide database that contains urine proteomics data of 1525 oncological and 1503 healthy controls with the spectral intensity of 5605 peptides. First, we identified peptide spectra using various feature selection techniques, which display different intensity and occurrence in oncological samples and healthy controls. Based on selected 173 peptide-based biomarkers, we developed models for predicting oncological samples and achieved maximum accuracy of 91.94% with 0.84 MCC. Prediction models were also developed based on spectral intensities with known peptide sequences. We also quantitated the amount of protein in a sample based on intensities of its fragments/peptides and developed prediction models based on protein expression. It was observed that certain proteins and their peptides such as fragments of collagen protein are more abundant in oncological samples. Based on this study, we also developed a web bench, CancerUBM, for mining proteomics data, which is freely available athttp://webs.iiitd.edu.in/raghava/cancerubm/.