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Lip and palatal cleft

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https://www.readbyqxmd.com/read/28526461/-anesthesia-in-a-child-operated-for-cleft-lip-associated-with-patau-s-syndrome
#1
Manoj Kamal, Don Varghese, Jeet Bhagde, Geeta Singariya, Annie Miju Simon, Amar Singh
Patients with Patau's syndrome (Trisomy 13) have multiple craniofacial, cardiac, neurological and renal anomalies with very less life expectancy. Among craniofacial anomalies cleft lip and palate are common. These craniofacial and cardiac anomalies present difficulties with anesthesia. We therefore describe the anesthetic management in the case of a Trisomy 13 child for operated for cleft lip at 10 months of age.
May 16, 2017: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/28524264/maternal-occupational-physical-activity-and-risk-for-orofacial-clefts
#2
A J Agopian, Jihye Kim, Peter H Langlois, Laura Lee, Lawrence W Whitehead, Elaine Symanski, Michele L Herdt, George L Delclos
OBJECTIVES: To perform a case-control study of maternal occupational physical activity and risk for orofacial clefts in Texas during 1999-2009. METHODS: We used logistic regression to assess 14 measures of physical activity estimated from a job exposure matrix, using the maternal occupation reported on the birth certificate, among 887 children with cleft lip with or without cleft palate (CLP), 436 children with cleft palate only (CP), and 1932 controls. RESULTS: After adjusting for several potential confounders, seven measures of physical activity (as a categorical and/or continuous variable) were significantly associated with CLP, CP, or both...
May 19, 2017: American Journal of Industrial Medicine
https://www.readbyqxmd.com/read/28513979/identification-of-a-de-novo-variant-in-chuk-in-a-patient-with-an-eec-aec-syndrome-like-phenotype-and-hypogammaglobulinemia
#3
Kriti D Khandelwal, Charlotte W Ockeloen, Hanka Venselaar, Cécile Boulanger, Bénédicte Brichard, Etienne Sokal, Rolph Pfundt, Tuula Rinne, Ellen van Beusekom, Marjon Bloemen, Gerrit Vriend, Nicole Revencu, Carine E L Carels, Hans van Bokhoven, Huiqing Zhou
The cardinal features of Ectrodactyly, Ectodermal dysplasia, Cleft lip/palate (EEC), and Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndromes are ectodermal dysplasia (ED), orofacial clefting, and limb anomalies. EEC and AEC are caused by heterozygous mutations in the transcription factor p63 encoded by TP63. Here, we report a patient with an EEC/AEC syndrome-like phenotype, including ankyloblepharon, ED, cleft palate, ectrodactyly, syndactyly, additional hypogammaglobulinemia, and growth delay...
May 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28512138/factors-predicting-parent-anxiety-around-infant-and-toddler-postoperative-and-pain
#4
Rebecca E Rosenberg, Rachael A Clark, Patricia Chibbaro, H Rhodes Hambrick, Jean-Marie Bruzzese, Chris Feudtner, Alan Mendelsohn
BACKGROUND AND OBJECTIVES: Understanding of parent anxiety and its effect on infant postoperative pain is limited. We sought to identify psychological factors associated with preoperative anxiety for parents of infants and toddlers undergoing elective surgery and to determine whether parent anxiety is associated with child postoperative pain. METHODS: This was a prospective cohort study of consecutively eligible patients aged ≤18 months undergoing craniofacial surgery and their parents...
May 16, 2017: Hospital Pediatrics
https://www.readbyqxmd.com/read/28507866/establishing-content-validity-of-the-cleft-q-a-new-patient-reported-outcome-instrument-for-cleft-lip-palate
#5
Elena Tsangaris, Karen W Y Wong Riff, Tim Goodacre, Christopher R Forrest, Marieke Dreise, Jonathan Sykes, Tristan de Chalain, Karen Harman, Aisling O'Mahony, Andrea L Pusic, Lehana Thabane, Achilleas Thoma, Anne F Klassen
BACKGROUND: The CLEFT-Q is a new patient-reported outcome instrument designed to measure outcomes that matter to patients. The aim of this qualitative study was to establish content validity of the CLEFT-Q in patients who differ by age and culture. METHODS: Patients aged between 6 and 29 years were recruited from plastic surgery clinics in Canada, India, Ireland, the Philippines, the Netherlands and the United States. Healthcare providers and other experts participated in a focus group or provided individual feedback...
April 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28496230/invasive-cervical-resorption-and-the-oro-facial-cleft-patient-a-review-and-case-series
#6
A O'Mahony, C McNamara, A Ireland, J Sandy, J Puryer
Introduction Invasive cervical resorption (ICR) has an unknown aetiology, yet it exhibits very aggressive behaviour compared with typical external root resorption, posing a high risk of tooth loss.Aim To investigate the number of patients at the Dublin Cleft Prosthodontic Department with an oro-facial cleft who experienced ICR and to identify any possible aetiological factors.Materials and method A retrospective investigation of all oro-facial cleft patients treated at the Dublin Cleft Prosthodontic Department, St James's Hospital, Dublin...
May 12, 2017: British Dental Journal
https://www.readbyqxmd.com/read/28489962/development-and-validation-of-a-screening-procedure-to-identify-speech-language-delay-in-toddlers-with-cleft-palate
#7
Line Dahl Jørgensen, Elisabeth Willadsen
The purpose of this study was to develop and validate a clinically useful speech-language screening procedure for young children with cleft palate ± cleft lip (CP) to identify those in need of speech-language intervention. Twenty-two children with CP were assigned to a +/- need for intervention conditions based on assessment of consonant inventory using a real-time listening procedure in combination with parent-reported expressive vocabulary. These measures allowed evaluation of early speech-language skills found to correlate significantly with later speech-language performance in longitudinal studies of children with CP...
May 10, 2017: Clinical Linguistics & Phonetics
https://www.readbyqxmd.com/read/28488057/the-impact-of-rapid-maxillary-expansion-on-maxillary-first-molar-root-morphology-of-cleft-subjects
#8
Lucas Cardinal, Gabriela da Rosa Zimermann, Fausto Medeiros Mendes, Ildeu Andrade, Dauro Douglas Oliveira, Gladys Cristina Dominguez
OBJECTIVES: The aim of this prospective cohort study was to determine the effects of rapid maxillary expansion (RME) on the first molar roots of cleft lip and palate subjects along different root development stages. MATERIALS AND METHODS: Thirty participants with unilateral cleft lip and palate were divided into three groups (n = 10), according to the type of expander used: Hyrax, iMini and Fan-type. A cone beam CT scan was performed before (T1) and 3 months after stabilization of the appliance (T2)...
May 9, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28486694/differential-microrna-expression-in-cultured-palatal-fibroblasts-from-infants-with-cleft-palate-and-controls
#9
Christian Schoen, Jeffrey C Glennon, Shaghayegh Abghari, Marjon Bloemen, Armaz Aschrafi, Carine E L Carels, Johannes W Von den Hoff
Background: The role of microRNAs (miRNAs) in animal models of palatogenesis has been shown, but only limited research has been carried out in humans. To date, no miRNA expression study on tissues or cells from cleft palate patients has been published. We compared miRNA expression in palatal fibroblasts from cleft palate patients and age-matched controls. Material and Methods: Cultured palatal fibroblasts from 10 non-syndromic cleft lip and palate patients (nsCLP; mean age: 18 ± 2 months), 5 non-syndromic cleft palate only patients (nsCPO; mean age: 17 ± 2 months), and 10 controls (mean age: 24 ± 5 months) were analysed with next-generation small RNA sequencing...
May 9, 2017: European Journal of Orthodontics
https://www.readbyqxmd.com/read/28485378/phf8-loss-confers-resistance-to-depression-like-and-anxiety-like-behaviors-in-mice
#10
Ryan M Walsh, Erica Y Shen, Rosemary C Bagot, Anthony Anselmo, Yan Jiang, Behnam Javidfar, Gregory J Wojtkiewicz, Jennifer Cloutier, John W Chen, Ruslan Sadreyev, Eric J Nestler, Schahram Akbarian, Konrad Hochedlinger
PHF8 is a histone demethylase with specificity for repressive modifications. While mutations of PHF8 have been associated with cognitive defects and cleft lip/palate, its role in mammalian development and physiology remains unexplored. Here, we have generated a Phf8 knockout allele in mice to examine the consequences of Phf8 loss for development and behaviour. Phf8 deficient mice neither display obvious developmental defects nor signs of cognitive impairment. However, we report a striking resiliency to stress-induced anxiety- and depression-like behaviour on loss of Phf8...
May 9, 2017: Nature Communications
https://www.readbyqxmd.com/read/28483218/hearing-thresholds-and-ventilation-tube-treatment-in-children-with-unilateral-cleft-lip-and-palate
#11
Birgitta Tengroth, Christina Hederstierna, Erik Neovius, Traci Flynn
OBJECTIVE: Children with cleft lip and palate have a high prevalence of otitis media with effusion (OME) which is often associated with a fluctuating, conductive hearing loss in the low and mid-frequencies and a risk for permanent hearing loss in the higher frequencies. Although common, there is no consensus on the treatment of OME with ventilation tubes. The aim of this study is to document if the risk for permanent hearing loss and acquired cholesteatoma increases due to treatment with ventilation tubes (VT treatments) during childhood in a group of children with cleft lip and palate...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28481036/generation-and-characterization-of-a-conditional-allele-of-interferon-regulatory-factor-6
#12
Arianna L Smith, Youssef A Kousa, Akira Kinoshita, Kate Fodor, Baoli Yang, Brian C Schutte
Interferon Regulatory Factor 6 (IRF6) is a critical regulator of differentiation, proliferation and migration of keratinocytes. Mutations in IRF6 cause two autosomal dominant disorders characterized by cleft lip with or without cleft palate. In addition, DNA variation in IRF6 confers significant risk for non-syndromic cleft lip and palate. IRF6 is also implicated in adult onset development and disease processes, including mammary gland development and squamous cell carcinoma. Mice homozygous for a null allele of Irf6 die shortly after birth due to severe skin, limb, and craniofacial defects, thus impeding the study of gene function after birth...
May 8, 2017: Genesis: the Journal of Genetics and Development
https://www.readbyqxmd.com/read/28480845/risk-factors-of-cleft-lip-and-palate-in-georgia
#13
S Chincharadze, Z Vadachkoria, I Mchedlishvili
A case-control study was conducted to reveal risk factors for cleft lip and palate. The main group consisted of 41mothers of infants with orofacial clefts (cleft lip and/or palate) who were born in Tbilisi maternity houses during 2015-2016. The control group was composed of 61 mothers who have given birth to healthy babies in the same maternity houses. Information on possible risk factors was collected using a special form. In order to evaluate a role of these risk factors, bivariate analysis was conducted at the first stage of the study...
March 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28474057/-biomechanical-effect-of-2-anchorages-on-maxillary-protraction-with-cleft-lip-and-palate-a-finite-element-analysis
#14
Han Han, Ya-Fen Xu, Yu-Feng Duan, Yong-Hua Lei
PURPOSE: To establish cranio-maxillary complex finite element models (FEMs) in cleft lip and palate (CLP) patients with miniplate and tooth-borne anchorage, and to investigate the biomechanical effects on maxillary protraction. METHODS: The protracting process was simulated on FEMs established from a male patient's cone beam CT (CBCT) data to analyze the displacement and stress from different appliances. RESULTS: Four FEMs of unilateral cleft lip and palate (UCLP) or bilateral cleft lip and palate (BCLP) patients' cranio-maxillary complex with MA and TA were established...
February 2017: Shanghai Kou Qiang Yi Xue, Shanghai Journal of Stomatology
https://www.readbyqxmd.com/read/28472982/comparative-safety-of-anti-epileptic-drugs-during-pregnancy-a-systematic-review-and-network-meta-analysis-of-congenital-malformations-and-prenatal-outcomes
#15
Areti Angeliki Veroniki, Elise Cogo, Patricia Rios, Sharon E Straus, Yaron Finkelstein, Ryan Kealey, Emily Reynen, Charlene Soobiah, Kednapa Thavorn, Brian Hutton, Brenda R Hemmelgarn, Fatemeh Yazdi, Jennifer D'Souza, Heather MacDonald, Andrea C Tricco
BACKGROUND: Pregnant women with epilepsy frequently experience seizures related to pregnancy complications and are often prescribed anti-epileptic drugs (AEDs) to manage their symptoms. However, less is known about the comparative safety of AED exposure in utero. We aimed to compare the risk of congenital malformations (CMs) and prenatal outcomes of AEDs in infants/children who were exposed to AEDs in utero through a systematic review and Bayesian random-effects network meta-analysis...
May 5, 2017: BMC Medicine
https://www.readbyqxmd.com/read/28472275/dental-age-comparison-in-patients-born-with-unilateral-cleft-lip-and-palate-to-a-control-sample-using-demirjian-and-willems-methods
#16
Nabeel Almotairy, Marie Pegelow
Objectives: To determine and compare the differences in dental age (DA) and chronological age (CA) of Demirjian and Willems methods in 9- to 11-year-old Caucasian boys and girls born with non-syndromic unilateral cleft lip and palate (NSUCLP) to an age-matched control group. Analysis of the results is undertaken to determine whether there are differences between gender, groups, and methods. Materials and methods: Dental panoramic tomograph (DPT) of 108 children with NSUCLP aged from 8...
May 2, 2017: European Journal of Orthodontics
https://www.readbyqxmd.com/read/28471735/clinical-finding-and-management-of-12-orofacial-clefts-cases-with-nevoid-basal-cell-carcinoma-syndrome
#17
Lee Mui Lee, Lian Ma, Tie Jun Li
Objective  To study the phenotype and overview the clinical management on Cleft Lip and/or Palate (CL/P) with Nevoid Basal Cell Carcinoma Syndrome (NBCCS) patients in Peking University School and Hospital of Stomatology. Design  Case series study. Main Outcome Measures  To describe the clinical phenotype of 12 CL/P with NBCCS patients who fulfilled the diagnostic criteria as well as to explore clinical management. Results  Seven cases (7/12, 58.33%) were classified as bilateral complete cleft lip and palate (BCCLP)...
May 2017: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/28471211/arithmetical-calculation-and-related-neuropsychological-skills-in-subjects-with-isolated-oral-clefts
#18
Jon W Goodwin, Amy L Conrad, Timothy Ansley, Peg Nopoulos
OBJECTIVE: The current study examined whether the arithmetical calculation skills of children, adolescents, and young adults with isolated cleft of the lip and/or palate (iCL/P) differ significantly from unaffected control participants. Comparisons of potential neuropsychological predictors of arithmetical calculation were also conducted to determine whether these variables differ significantly for participants with iCL/P. METHOD: Participants (N = 176; 93 iCL/P and 83 Control) ranged in age from 7 to 26 years old...
May 4, 2017: Neuropsychology
https://www.readbyqxmd.com/read/28468208/popliteal-pterygium-syndrome-with-syngnathia
#19
Andrea Sisti, Nicola Freda, Alessandro Giacomina, Gian Luca Gatti
Popliteal pterygium syndrome is a condition characterized by skin webs on the popliteal fossa, which may impair mobility unless surgically repaired. Affected individuals may also have syndactyly on the fingers and/or toes. Most people with this disorder present cleft lip and cleft palate and they can have syngnathia, that is a congenital adhesion between maxilla and mandible by fibrous bands, which affects the opening of the mouth. The case that we report is about a 2-month-old male, who presented skin webs bilaterally on the popliteal fossa, syndactyly between the IV and the V toe of the right foot and between the III and the IV toe of the left foot, and genital malformations...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28462671/the-impact-of-tympanostomy-tubes-on-speech-and-language-development-in-children-with-cleft-palate
#20
Amber D Shaffer, Matthew D Ford, Sukgi S Choi, Noel Jabbour
Objective Describe the impact of hearing loss, tympanostomy tube placement before palatoplasty, and number of tubes received on speech outcomes in children with cleft palate. Study Design Case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods Records from 737 children born between April 2005 and April 2015 who underwent palatoplasty at a tertiary children's hospital were reviewed. Exclusion criteria were cleft repair at an outside hospital, intact secondary palate, absence of postpalatoplasty speech evaluation, sensorineural or mixed hearing loss, no tubes, first tubes after palatoplasty, or first clinic after 12 months of age...
April 1, 2017: Otolaryngology—Head and Neck Surgery
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