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https://www.readbyqxmd.com/read/28324045/ring-finger-protein-6-mediates-androgen-induced-granulosa-cell-proliferation-and-follicle-growth-via-modulation-of-androgen-receptor-signaling
#1
Jung Jin Lim, Chae Young Han, Dong Ryul Lee, Benjamin K Tsang
The destiny of the ovarian follicle (growth or atresia) is tightly regulated by actions and interactions of endocrine, paracrine and autocrine factors. Although androgens are known to be important in the regulation of folliculogenesis, whether they facilitate or suppress follicular growth has been controversial and the mechanisms involved are not fully understood. Moreover, the role and regulation of its receptor (AR) in mediating androgen signaling during follicular development is not clear. Here, we report that the active androgen dihydrotestosterone (DHT) up-regulates the expression of AR and its E3 ligase Ring Finger Protein 6 (RNF6), increasing site-specific AR polyubiquitination and AR transcriptional activity for sKit-L expression in preantral follicle growth...
January 30, 2017: Endocrinology
https://www.readbyqxmd.com/read/28319568/predictive-value-of-intraoperative-troponin-i-elevation-in-pediatric-living-donor-liver-transplant-recipients-with-biliary-atresia
#2
Mingwei Sheng, Yuanbang Lin, Yiqi Weng, Rubin Xu, Ying Sun, Wenli Yu, Hongyin Du
BACKGROUND: Pediatric living donor liver transplantation is associated with slight alteration in cardiac enzymes without ongoing acute cardiac injury, but available information about the significance of these changes is limited. The aims of this study were to analyze the link between the anomalies of intraoperative serum cardiac troponin I (cTnI) and acute lung injury during the first week after liver transplantation. METHODS: In this retrospective study, 123 children suffered from biliary atresia were enrolled...
March 18, 2017: Transplantation
https://www.readbyqxmd.com/read/28319315/maxillofacial-features-and-systemic-malformations-in-expanded-spectrum-hemifacial-microsomia
#3
Noah Cohen, Erica Cohen, Alberto Gaiero, Silvia Zecca, Graziella Fichera, Federica Baldi, Joseph Felix Giordanetto, Jacques Marie Mercier, Amnon Cohen
Hemifacial microsomia (HFM) is a rare, multisystemic congenital disease with estimated frequency of 1/26370 births in Europe. Most cases are sporadic and caused by unilateral abnormal morphogenesis of the first and second pharyngeal arches. The aim of this study is to define the types and frequency of maxillofacial and systemic malformations in HFM patients. This is a case series study of patients with HFM evaluated at a single institution. Data were acquired through history, physical examination, photographs, diagnostic radiology, and laboratory and analyzed by the FileMakerPro database on 95 patients (54F; 41M) of which 89 met the inclusion criteria...
March 20, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28318598/pediatric-eating-assessment-tool-10-as-an-indicator-to-predict-aspiration-in-children-with-esophageal-atresia
#4
Tutku Soyer, Sule Yalcin, Selen Serel Arslan, Numan Demir, Feridun Cahit Tanyel
AIM: Airway aspiration is a common problem in children with esophageal atresia (EA). Pediatric Eating Assessment Tool-10 (pEAT-10) is a self-administered questionnaire to evaluate dysphagia symptoms in children. A prospective study was performed to evaluate the validity of pEAT-10 to predict aspiration in children with EA. METHODS: Patients with EA were evaluated for age, sex, type of atresia, presence of associated anomalies, type of esophageal repair, time of definitive treatment, and the beginning of oral feeding...
March 14, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28315283/effect-of-swallowing-rehabilitation-protocol-on-swallowing-function-in-patients-with-esophageal-atresia-and-or-tracheoesophageal-fistula
#5
Selen Serel Arslan, Tutku Soyer, Numan Demir, Sule Yalcın, Ayşe Karaduman, Ibrahim Karnak, Feridun Cahit Tanyel
Aim The aim of this study was to evaluate the results of Swallowing Rehabilitation Protocol (SRP) on swallowing function (SF) of esophageal atresia and tracheoesophageal fistula (EA-TEF) patients with pharyngeal swallowing disorder. Materials and Methods In this study, 24 children with EA-TEF who had deglutitive and respiratory problems were grouped into either study (n = 12) or control group (n = 12) by basic randomization. Study group received the SRP including neuromuscular electrical stimulation, thermal tactile stimulation, and hyolaryngeal mobilization...
March 17, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28314387/twin-pregnancy-complicated-by-esophageal-atresia-duodenal-atresia-gastric-perforation-and-hypoplastic-left-heart-structures-in-one-twin-a-case-report-and-review-of-the-literature
#6
Mohamad K Abou Chaar, Mariana L Meyers, Bethany D Tucker, Henry L Galan, Kenneth W Liechty, Timothy M Crombleholme, Ahmed I Marwan
BACKGROUND: The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy...
March 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28304404/interactions-between-th1-cells-and-tregs-affect-regulation-of-hepatic-fibrosis-in-biliary-atresia-through-the-ifn-%C3%AE-stat1-pathway
#7
Jie Wen, Ying Zhou, Jun Wang, Jie Chen, Wenbo Yan, Jin Wu, Junkai Yan, Kejun Zhou, Yongtao Xiao, Yang Wang, Qiang Xia, Wei Cai
Regulatory T cells (Tregs) and CD4(+) T helper (Th) cells have important roles in bile duct injury of biliary atresia (BA). However, their impacts on liver fibrosis are undefined. Between 2013 and 2016, 146 patients with various stages of BA were enrolled in this study. Peripheral blood, liver biopsy and lymph node samples were collected. Flow cytometry, magnetic cell sorting and immunostaining were used to characterize lymphocytes from BA patients. Deficiency of Tregs was observed along with increased Th1, Th2 and Th17 frequencies in the peripheral blood and livers of BA patients...
March 17, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28303723/decellularised-material-as-scaffolds-for-tissue-engineering-studies-in-long-gap-oesophageal-atresia
#8
Esmond Lee, Anna Milan, Luca Urbani, Paolo De Coppi, Mark W Lowdell
Oesophageal atresia refers to an anomaly in foetal development in which the oesophagus terminates in a blind end. Whilst surgical correction is achievable in most patients, when a long gap is present it still represents a major challenge associated with higher morbidity and mortality. In this context, tissue engineering could represent a successful alternative to restore oesophageal function and structure. Naturally derived biomaterials made of decellularised tissues retain native extracellular matrix architecture and composition, providing a suitable bed for the anchorage and growth of relevant cell types...
March 17, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28300528/functional-near-tricuspid-atresia-in-a-patient-with-absent-pulmonary-valve-and-an-intact-ventricular-septum
#9
Yasunobu Miki, Toshikatsu Tanaka, Yoshihiro Oshima
Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock-Taussig shunt at 11 days of age...
August 30, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28296740/diagnosis-and-treatment-of-communicating-bronchopulmonary-foregut-malformation-report-of-two-cases-and-review-of-the-literature
#10
Hongxia Ren, Liqiong Duan, Baohong Zhao, Xiaoxia Wu, Hongyi Zhang, Caixia Liu
RATIONALE: Communicating bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory systems. To our best knowledge, most cases of CBPFM reported in the literature were in infancy or adulthood and CBPFM in infantile is even rarer with a high case-fatality rate partly due to misdiagnosis. PATIENT CONCERNS: We presented 2 cases of neonatal CBPFM. A 11-hour male newborn was admitted because of moaning for 7 hours, and a 1-day male newborn was referred to us with profuse foams, choking on breast-milk feeding and facial cyanosis...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28293976/evolution-of-hybrid-interventions-for-congenital-heart-disease
#11
Hitesh Agrawal, Wail Alkashkari, Damien Kenny
Surgical and transcatheter interventions have seen a tremendous evolution in last three decades. Hybrid technology combines the achievements of both disciplines to bring substantial hemodynamic benefit to patients with congenital heart disease (CHD) in a minimally invasive manner. This collaboration between surgeons and interventionalists will continue to grow as the technology evolves to meet the demands of CHD patients, potentially avoiding cardiopulmonary bypass and vascular access complications as well as optimizing immediate technical outcomes with exit angiography...
March 15, 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28293742/the-accumulation-of-regulatory-t-cells-in-the-hepatic-hilar-lymph-nodes-in-biliary-atresia
#12
Naoya Sakamoto, Toshihiro Muraji, Haruo Ohtani, Kouji Masumoto
PURPOSE: A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA...
March 14, 2017: Surgery Today
https://www.readbyqxmd.com/read/28293001/-congenital-bronchial-atresia-requiring-differentiation-from-intralobar-sequestration
#13
Yusuke Tanaka, Isao Matsumoto, Daisuke Saito, Syuhei Yoshida, Seiichi Kakegawa, Masaya Tamura, Hirofumi Takemura, Kaori Yoshimura, Hiroko Ikeda
We reported a case of bronchial atresia requiring differentiation from the intralobar sequestration. A 42-year-old man was referred to our institution with suspicion of intralobar sequestration, based on a 3-dimensional computed tomography (CT) angiography that showed abnormal blood vessels from the right inferior phrenic artery flowing into the right lower lobe. CT revealed a lesion between S9 and S10 wherein there were refluxed blood vessels from A9 without an accompanying bronchus, with polycysts and emphysematous changes...
March 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28292964/longitudinal-evaluation-of-growth-in-oesophageal-atresia-patients-up-to-12%C3%A2-years
#14
Floor W T Vergouwe, Marjolein Spoel, Nicole W G van Beelen, Saskia J Gischler, René M H Wijnen, Joost van Rosmalen, Hanneke IJsselstijn
OBJECTIVE: Previous studies reported diminished growth after oesophageal atresia (OA) repair. We evaluated long-term follow-up data on growth. METHODS: Longitudinal cohort study up to 12 years. Patients with OA, born 1999-2013, who participated in a longitudinal follow-up programme were included. Children with genetic syndromes associated with growth disorders were excluded. SD scores (SDS) for height-for-age (HFA), weight-for-height (WFH) and distance-to-target-height were calculated for routine visits (0...
March 14, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/28291300/-possible-relation-between-antenatal-venlafaxine-use-and-vacterl-association-in-a-newborn-a-case-report
#15
Muammer Özgür Çevik, Mustafa Çelik, İbrahim Hakan Bucak, Behice Han Almış, Mehmet Turğut
Major depressive disorder is common during antenatal period and many women are prescribed antidepressant drugs despite no antidepressant can be regarded as definitely safe in pregnancy. Previous studies have suggested links between gestational use of selective serotonin reuptake inhibitors (SSRI) or serotonin and norepinephrine reuptake inhibitors (SNRI) and certain birth defects. VACTERL association is a rare group of congenital malformations which were observed to occur together more often than would be expected by chance...
2017: Türk Psikiyatri Dergisi, Turkish Journal of Psychiatry
https://www.readbyqxmd.com/read/28289870/the-effects-of-choanal-atresia-on-development-of-the-paranasal-sinuses-and-turbinates
#16
Suat Terzi, Engin Dursun, Fatma Beyazal Çeliker, Metin Çeliker, Mehmet Beyazal, Abdulkadir Özgür, Emel Çadallı Tatar, Mehmet Hakan Korkmaz
PURPOSE: The objective of this study was to evaluate the volume of paranasal sinuses (PNS) and turbinate in patients with unilateral choanal atresia (CA). MATERIALS AND METHOD: Computed tomography images of PNS in 11 individuals with unilateral CA were evaluated retrospectively. Mucosal thickness and volume of the maxillary, frontal and sphenoidal sinuses were determined, in addition to the volume of the middle and inferior turbinate. The unaffected nasal side of patients was used as a control group for the measurements...
March 13, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28289704/preferential-tnf%C3%AE-signaling-via-tnfr2-regulates-epithelial-injury-and-duct-obstruction-in-experimental-biliary-atresia
#17
Pranavkumar Shivakumar, Tatsuki Mizuochi, Reena Mourya, Sridevi Gutta, Li Yang, Zhenhua Luo, Jorge A Bezerra
Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNFA and its receptors TNFR1 and TNFR2 in human livers and found an increased expression of the receptors at the time of diagnosis...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28286566/gastrointestinal-manifestations-of-mitochondrial-disorders-a-systematic-review
#18
REVIEW
Josef Finsterer, Marlies Frank
Mitochondrial disorders (MIDs) due to respiratory-chain defects or nonrespiratory chain defects are usually multisystem conditions [mitochondrial multiorgan disorder syndrome (MIMODS)] affecting the central nervous system (CNS), peripheral nervous system, eyes, ears, endocrine organs, heart, kidneys, bone marrow, lungs, arteries, and also the intestinal tract. Frequent gastrointestinal (GI) manifestations of MIDs include poor appetite, gastroesophageal sphincter dysfunction, constipation, dysphagia, vomiting, gastroparesis, GI pseudo-obstruction, diarrhea, or pancreatitis and hepatopathy...
January 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28283556/utilization-of-reflex-testing-for-direct-bilirubin-in-the-early-recognition-of-biliary-atresia
#19
Leo Lam, Samarina Musaad, Campbell Kyle, Stephen Mouat
BACKGROUND: Delayed diagnosis of biliary atresia is an important cause of pediatric end-stage liver failure and liver transplantation. We sought to determine whether direct bilirubin is underutilized by retrospectively reviewing patients with biliary atresia. Further, we aimed to determine the role of reflex testing for direct bilirubin in patients suspected for jaundice. METHODS: The time intervals between total bilirubin and direct bilirubin measurements were retrospectively reviewed in patients with biliary atresia...
March 10, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28283284/mri-in-the-evaluation-of-obstructive-reproductive-tract-anomalies-in-paediatric-patients
#20
H Zhang, H Qu, G Ning, B Cheng, F Jia, X Li, X Chen
AIM: To outline the anatomical variations of obstructive reproductive tract anomalies (ORTA) using magnetic resonance imaging (MRI) and its role in preoperative evaluation. MATERIALS AND METHODS: MRI and treatment of 21 paediatric patients with ORTA were reviewed and analysed. MRI findings were correlated with ultrasound and surgical findings. RESULTS: Patients presented in two distinct ways: primary amenorrhoea with cyclic pelvic pain, or progressive dysmenorrhoea...
March 7, 2017: Clinical Radiology
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