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https://www.readbyqxmd.com/read/29931283/location-of-tef-at-the-carina-as-an-indicator-of-long-gap-c-type-esophageal-atresia
#1
A Koivusalo, J Suominen, R Rintala, M Pakarinen
We analyzed retrospectively the outcomes in long gap Gross type C esophageal atresia. We hypothesized that outcomes in type C (long gap) atresia differ from type C (normal gap) and be similar with outcomes in Gross type A and B esophageal atresia. Location of the distal tracheoesophageal fistula (TEF) at the carina was chosen as the hallmark of type C atresia (long gap). We compared the type of esophageal repair, major reoperations for anastomotic complications and gastroesophageal reflux, and long-term mucosal changes between type C (normal gap), type C (long gap), and type A/B...
June 19, 2018: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/29930182/unilateral-pulmonary-vein-atresia-diagnostic-dilemma-unfolded-on-imaging
#2
Basavaraj Biradar, Arun Sharma, Amarinder Singh Malhi, Sanjeev Kumar
Unilateral pulmonary vein atresia is a rare entity, usually congenital in origin. It is thought to result from failure of incorporation of common pulmonary vein to left atrium. Patients often present with recurrent chest infections and haemoptysis during infancy or early childhood. Associated anomalies are commonly present in these cases. Pulmonary angiography is generally used for definitive diagnosis; however, characteristic imaging findings on latest multislice CT can be virtually diagnostic.
June 21, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29927863/endoscopic-esophageal-vacuum-therapy-a-novel-therapy-for-esophageal-perforations-in-pediatric-patients
#3
Michael A Manfredi, Susannah J Clark, Steven J Staffa, Peter D Ngo, C Jason Smithers, Thomas E Hamilton, Russell W Jennings
BACKGROUND: Esophageal perforation is a potentially life-threatening problem if not quickly diagnosed and treated appropriately. Negative pressure wound therapy (NPWT), commercially known as V.A.C.® therapy, was developed in the early 1990 s and is now standard of care for chronic surface wounds, ulcers, and burns. Adapting vacuum sponge therapy for use intraluminally for perforations of the esophagus was first reported in 2008. We report the first pediatric experience on a customized esophageal vacuum assisted closure (EVAC) device for closure of esophageal perforations...
June 20, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29925723/regenerative-therapy-for-patients-with-congenital-heart-disease
#4
Naritaka Kimura
Congenital heart disease (CHD) is the most common birth defect, affecting 1 in 100 babies. Among CHDs, single ventricle (SV) physiologies, such as hypoplastic left heart syndrome and tricuspid atresia, are particularly severe conditions that require multiple palliative surgeries, including the Fontan procedure. Although the management strategies for SV patients have markedly improved, the prevalence of ventricular dysfunction continues to increase over time, especially after the Fontan procedure. At present, the final treatment for SV patients who develop heart failure is heart transplantation; however, transplantation is difficult to achieve because of severe donor shortages...
June 19, 2018: Keio Journal of Medicine
https://www.readbyqxmd.com/read/29923290/mycotoxin-zearalenone-induced-gonadal-impairment-and-altered-gene-expression-in-the-hypothalamic-pituitary-gonadal-axis-of-adult-female-zebrafish-danio-rerio
#5
Sellamani Muthulakshmi, Pourmohammadi Fallah Hamideh, Hamid R Habibi, Kannan Maharajan, Krishna Kadirvelu, Venkataramana Mudili
In the present study, we aimed to assess the adverse effects of zearalenone (ZEA) at environmentally relevant concentrations (0.5, 1, 5 and 10 μg l-1 ) on hypothalamic-pituitary-gonadal axis associated reproductive function using zebrafish model. ZEA was exposed to female zebrafish for 21 days to assess growth indices such as condition factor, hepatosomatic index, gonadosomatic index and caspase 3 activity. Further, expression of estrogen receptor (ER) α and CYP19a1b genes in the brain, ERα and vitellogenin (Vtg) genes in the liver and follicle-stimulating hormone receptor, luteinizing hormone receptor, ERα, steroidogenic acute regulatory protein, 3β-hydroxysteroid dehydrogenase (HSD), 17-βHSD and CYP19a1 genes in the ovary were also investigated...
June 19, 2018: Journal of Applied Toxicology: JAT
https://www.readbyqxmd.com/read/29921470/ttc7a-mutation-must-be-considered-in-patients-with-repeated-intestinal-atresia-associated-with-early-inflammatory-bowel-disease-two-new-case-reports-and-a-literature-review
#6
J Fayard, S Collardeau, Y Bertrand, M-P Cordier, C Malcus, R Dubois, P-Y Mure, G de Saint Basile, T Louazon, B Rohmer, A Lachaux, R Duclaux, N Peretti
TTC7A mutations cause multiple neonatal intestinal atresias with early inflammatory bowel disease and severe combined immunodeficiency. There are no treatment protocols for this rare disease. Two new cases are described for which radical early treatment measures - total enterectomy, home parenteral nutrition, immunoglobulin therapy and intravenous antibiotic prophylaxis - have allowed both patients to develop optimally.
June 16, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29920636/quality-of-life-in-parents-of-children-born-with-esophageal-atresia
#7
Stefanie Witt, Michaela Dellenmark-Blom, Jens Dingemann, Carmen Dingemann, Benno M Ure, Barbara Gomez, Monika Bullinger, Julia Quitmann
INTRODUCTION:  For parents of chronically ill children, the experiences of caregiving are challenged by increased demands and restrictions imposed by their child's disease. Therefore, this study aims to investigate the quality of life (QoL) in parents of children born with esophageal atresia (EA) and to explore associated factors. METHODS:  Parents of children (2-17 years) with EA recruited from two German pediatric hospitals participated in this cross-sectional study about QoL in EA...
June 19, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29915925/analysis-of-risk-factors-for-morbidity-in-children-undergoing-the-kasai-procedure-for-biliary-atresia
#8
Alejandro V Garcia, Mitchell R Ladd, Todd Crawford, Katherine Culbreath, Oswald Tetteh, Samuel M Alaish, Emily F Boss, Daniel S Rhee
OBJECTIVE: To evaluate the perioperative risk factors for 30-day complications of the Kasai procedure in a large, cross-institutional, modern dataset. STUDY DESIGN: The 2012-2015 National Surgical Quality Improvement Program Pediatric database was used to identify patients undergoing the Kasai procedure. Patients' characteristics were compared by perioperative blood transfusions and 30-day outcomes, including complications, reoperations, and readmissions. Multivariable logistic regression was used to identify risk factors predictive of outcomes...
June 18, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29914284/congenital-heart-defects-the-10-year-experience-at-a-single-center
#9
Emine Aydin, Ebru Aypar, Ahmet Oktem, Ozgur Ozyuncu, Murat Yurdakok, Murat Guvener, Metin Demircin, M Sinan Beksac
OBJECTIVE: We aimed to evaluate congenital heart disease (CHD) cases according to EUROCAT subgroup classification that were diagnosed during the prenatal period in our center. METHODS: CHDs that were prenatally diagnosed using ultrasonography and confirmed by fetal echocardiography were reviewed over a 10-year period. Subgroup classification was finalized at the post-partum period in terms of the EUROCAT guide 1.3. Congenital heart defect subtypes and obstetric outcomes (gestational week at delivery, birth weight, gender, extracardiac structural abnormalities, karyotype results if performed) were analyzed...
June 18, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29912286/a-new-classification-of-the-germinal-vesicle-gv-chromatin-configurations-in-pig-oocytes
#10
Liu-Zhu Pan, Shuai Zhu, Min Zhang, Ming-Jiu Sun, Juan Lin, Fei Chen, Jing-He Tan
Reported classifications of germinal vesicle (GV) chromatin configurations in pig oocytes were not done by uniform standards and they were not well correlated with oocyte competence. In this study, GV-chromatin of pig oocytes was classified into non-surrounded nucleolus (NSN), surrounded nucleolus (SN), partly NSN (pNSN) and SN (pSN), prematurely-condensed NSN (cNSN), pNSN (cpNSN) and pSN (cpSN), and early diakinesis (ED) patterns. During in vitro maturation in 199-medium, NSN oocytes from 1-2 mm follicles went consecutively through pNSN, pSN, cpSN and ED before undergoing GV breakdown, and chromatin in some SN oocytes from 3-6 mm follicles re-decondensed into a RDC configuration...
June 14, 2018: Biology of Reproduction
https://www.readbyqxmd.com/read/29909817/technical-factors-are-associated-with-complications-and-repeat-intervention-in-neonates-undergoing-transcatheter-right-ventricular-decompression-for-pulmonary-atresia-and-intact-ventricular-septum-results-from-the-congenital-catheterisation-research-collaborative
#11
Christopher J Petit, Athar M Qureshi, Andrew C Glatz, Michael S Kelleman, Courtney E McCracken, R Allen Ligon, Namrita Mozumdar, Wendy Whiteside, Asra Khan, Bryan H Goldstein
BACKGROUND: Transcatheter right ventricle decompression in neonates with pulmonary atresia and intact ventricular septum is technically challenging, with risk of cardiac perforation and death. Further, despite successful right ventricle decompression, re-intervention on the pulmonary valve is common. The association between technical factors during right ventricle decompression and the risks of complications and re-intervention are not well described. METHODS: This is a multicentre retrospective study among the participating centres of the Congenital Catheterization Research Collaborative...
June 18, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29909604/upregulation-of-high-mobility-group-box-1-may-contribute-to-the-pathogenesis-of-biliary-atresia
#12
Chun Jing Ye, Jiang Wang, Yi Fan Yang, Zhen Shen, Gong Chen, Yan Lei Huang, Yi Jie Zheng, Rui Dong, Shan Zheng
OBJECTIVES:  Biliary atresia (BA) is a progressive inflammatory obstructive cholangiopathy in infants. High mobility group box 1 (HMGB1) is known to play an important role as a late mediator of inflammation. However, it is not clear whether HMGB1 levels are of clinical significance in patients with BA. The aim of this study was to determine correlations between serum HMGB1 levels and the clinicopathologic features of BA. MATERIALS AND METHODS:  Serum samples were collected from 19 infants with BA, 7 infants with anicteric choledochal cysts (CC) and normal liver function, and 8 healthy controls...
June 17, 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29909011/gene-expression-profile-of-tlr7-signaling-pathway-in-the-liver-of-rhesus-rotavirus-induced-murine-biliary-atresia
#13
Yue Wu, Tingzheng Liu, Yuhang Yuan, Zhibo Zhang
PURPOSE: To identify genes potentially involved in the pathogenesis of bile duct obstruction in biliary atresia (BA). METHODS: We used rhesus rotavirus (RRV) Balb/c mouse BA model to study BA. Liver and serum samples were harvested from BA and normal control (NC) groups at 1, 3, 5, 7, 10 and 14 days postinoculation. Serum total bilirubin (STB) and conjugated bilirubin (CB) were measured. Livers of each group at day 7 were used for a genome-wide expression analysis...
June 14, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29907720/intestinal-atresia-in-suckler-calves
#14
(no author information available yet)
No abstract text is available yet for this article.
June 16, 2018: Veterinary Record
https://www.readbyqxmd.com/read/29907662/expression-of-atresia-biomarkers-in-granulosa-cells-after-ovarian-stimulation-in-heifers
#15
David A Landry, Lia Rossi-Perazza, Simon Lafontaine, Marc-Andre Sirard
The use of younger gamete donors in dairy cattle genetic selection programs significantly accelerates genetic gains by decreasing the interval between generations. Ovarian stimulation and the practice of follicle-stimulating hormone (FSH) withdrawal, also known as coasting, are intensively used in pre-pubertal heifers without detrimental effects on subsequent reproductive performance but generally with lower embryo yields. However, recent data from embryo transfer programs showed similar embryo yields in younger and sexually mature animals but with a significant difference in the coasting period...
June 15, 2018: Reproduction: the Official Journal of the Society for the Study of Fertility
https://www.readbyqxmd.com/read/29907216/liquid-chromatography-mass-spectroscopy-in-the-diagnosis-of-biliary-atresia-in-children-with-hyperbilirubinemia
#16
Jamie Golden, Jessica A Zagory, Michael Fenlon, Catherine J Goodhue, Yi Xiao, Xiaowei Fu, Kasper S Wang, Christopher P Gayer
BACKGROUND: Biliary atresia (BA) is difficult to distinguish from other causes of cholestasis. We evaluated the use of liquid chromatography-mass spectroscopy (LC-MS) and bile acid profiles in the rapid, noninvasive diagnosis of BA. MATERIALS AND METHODS: Following Institutional Animal Care and Use Committee and Institutional Review Board approval, we used LC-MS to measure 26 bile acids in serum and stool samples from experimental models of BA and in urine, stool, and serum samples from non-cholestatic and cholestatic human infants...
August 2018: Journal of Surgical Research
https://www.readbyqxmd.com/read/29905609/double-supercharged-jejunal-interposition-for-late-salvage-of-long-gap-esophageal-atresia
#17
Ingrid M Ganske, Joseph M Firriolo, Laura C Nuzzi, Oren Ganor, Thomas E Hamilton, C Jason Smithers, Russell W Jennings, Joseph Upton, Brian I Labow, Amir H Taghinia
BACKGROUND: A variety of surgical techniques exist to manage long-gap esophageal atresia (LGEA), including gastric pull-up (GPU), colonic interposition (CI), jejunal interposition (JI), and distraction lengthening. Salvage reconstruction for late failure of any conduit type is a complex surgical problem fraught with technical difficulty and significant risk. Jejunal interposition can be used as a salvage procedure in the management of LGEA. However, the opposing requirements of conduit length and adequate perfusion make the procedure technically challenging...
June 13, 2018: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/29904725/serum-level-of-interleukin-13-receptor-alpha-2-in-infants-with-biliary-atresia-is-it-of-value
#18
Nermin Adawy, Hanaa El-Araby, Alif Allam, Soha Elshenawy, Mohammed Khedr, Yasmine Ibrahim, Haidy M Zakaria
Aim of the study: We aimed to assess the utility of serum level IL-13Rα2 receptors as a non-invasive marker for early diagnosis of biliary atresia (BA) and selection of BA patients indicated for Kasai portoenterostomy. Material and methods: The study included 60 infants with neonatal cholestasis in three groups; early BA group ( n = 20), delayed BA group ( n = 20) and non-BA cholestasis group ( n = 20). A fourth group of 20 healthy neonates ( n = 20) served as controls...
June 2018: Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29904493/esophageal-lung-with-rare-associated-vascular-and-anorectal-malformations
#19
Ali Alsaadi, Hamdi A Alsufiani, Mohammad D Allugmani, Altaf Hussain Gora
Esophageal lung is a rare communicating bronchopulmonary foregut malformation in which the main bronchus arising from the trachea is absent. The affected lung is usually hypoplastic and aerated via an anomalous airway originating from the esophagus. Other anomalies such as esophageal atresia with tracheoesophageal fistula or VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities) association can co-exist. The initial radiographic findings may be normal, but subsequent imaging usually shows progressive and recurrent lung collapse, probably because of recurrent aspiration through the anomalous airway and poor compliance of the affected lung during breathing...
April 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29904486/cystic-biliary-atresia-a-distinct-clinical-entity-that-may-mimic-choledochal-cyst
#20
Gary R Schooler, Alisha Mavis
Cystic biliary atresia (CBA) is a relatively uncommon but clinically significant variant of biliary atresia. The presence of a cyst in the hepatic hilum on imaging in an infant with cholestasis supports the diagnosis of CBA, but can also be seen in patients with a choledochal cyst-the main differential diagnosis in patients with CBA. The reported case outlines the clinical presentation and imaging findings in a patient with surgically confirmed and treated CBA and emphasizes the importance of distinguishing CBA from choledochal cyst at diagnostic imaging given the disparate timing and type of surgical treatment necessary for successful management of these distinct entities...
April 2018: Radiology Case Reports
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