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Pectus carinatum

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https://www.readbyqxmd.com/read/29092017/the-effect-on-cardiopulmonary-function-after-thoracoplasty-in-pectus-carinatum-a-systematic-literature-review
#1
Stephan Sigl, Barbara Del Frari, Carina Harasser, Anton H Schwabegger
OBJECTIVES: Creating an aesthetically appealing result using thoracoplasty, especially when correcting extensive deformities, but only causing low morbidity, is challenging. The frequency of thoracoplasties in cases of pectus carinatum (PC) has increased due to improved experience and modified surgical techniques, resulting in low morbidity and low complication rates. The indications for surgical treatment are still controversial and, in most cases, remain aesthetic or psychological rather than physiological...
October 30, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29027531/-costal-cartilage-structural-and-functional-changes-in-children-with-a-funnel-or-keeled-chest
#2
A V Kurkov, A B Shekhter, V S Paukov
Congenital chest wall deformities (CCWDs) in children are severe diseases leading to cosmetic defects and diseases of the respiratory and cardiovascular systems. The most common of these deformities are funnel-shaped (pectus excavatum, FD) and keeled (pectus carinatum, KD) ones. The pathogenesis of CCWDs and the role of costal cartilage structural and functional changes in their pathogenesis have now been not well studied, which makes it difficult to elaborate pathogenetic approaches to correcting these diseases...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28946165/a-single-center-experience-with-dynamic-compression-bracing-for-children-with-pectus-carinatum
#3
Ashwini Suresh Poola, Amy L Pierce, Beth A Orrick, Shawn David St Peter, Charles L Snyder, David Juang, Pablo Aguayo, Jason D Fraser, George W Holcomb
No abstract text is available yet for this article.
September 25, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28893434/biallelic-mutations-in-szt2-cause-a-discernible-clinical-entity-with-epilepsy-developmental-delay-macrocephaly-and-a-dysmorphic-corpus-callosum
#4
Yuji Nakamura, Yasuko Togawa, Yusuke Okuno, Hideki Muramatsu, Kazuhiko Nakabayashi, Yoko Kuroki, Daisuke Ieda, Ikumi Hori, Yutaka Negishi, Takao Togawa, Ayako Hattori, Seiji Kojima, Shinji Saitoh
Mutations in SZT2 were first reported in 2013 as a cause of early-onset epileptic encephalopathy. Because only five reports have been published to date, the clinical features associated with SZT2 remain unclear. We herein report an additional patient with biallelic mutations in SZT2. The proband, a four-year-old girl, showed developmental delay and seizures from two years of age. Her seizures were not intractable and readily controlled by valproate. She showed mildly dysmorphic facies with macrocephaly, high forehead, and hypertelorism, and also had pectus carinatum...
September 8, 2017: Brain & Development
https://www.readbyqxmd.com/read/28893382/pain-and-sensory-disturbances-following-surgical-repair-of-pectus-carinatum
#5
Marie Veje Knudsen, Hans K Pilegaard, Kasper Grosen
BACKGROUND/PURPOSE: The purpose of this study was to assess the characteristics of persistent postoperative pain and sensory disturbances following surgical repair of pectus carinatum. METHODS: Using a prospective observational design, 28 patients were assessed before, 6 weeks and 6 months after a modified Ravitch operation for pectus carinatum. Postoperative pain was assessed using the Short Form McGill Pain Questionnaire. Sensory testing was conducted to detect brush-evoked allodynia and pinprick hyperalgesia...
August 25, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28857530/-surgery-in-times-of-crisis-conservative-treatment-of-pectus-carinatum-by-static-corset
#6
J V Redondo Sedano, M D Delgado Muñoz, M E Martí Carrera, A Gómez Fraile
INTRODUCTION: Dynamic compression system is the elective treatment for chondrogladiolar pectus carinatum. Nevertheless, its high cost poses a problem for its prescription in places where it is not subsidized. This article analyzes the experience of the Paediatric Plastic Surgery Service at a third grade hospital in the treatment of this deformity with a static compression system. MATERIALS AND METHODS: The study presents a descriptive, retrospective analysis of 30 patients with pectus carinatum treated with a static compression system...
April 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28757834/anesthesia-for-minimally-invasive-chest-wall-reconstructive-surgeries-our-experience-and-review-of-literature
#7
REVIEW
Shagun Bhatia Shah, Uma Hariharan, Ajay Kumar Bhargava, Laleng M Darlong
Minimal access procedures have revolutionized the field of surgery and opened newer challenges for the anesthesiologists. Pectus carinatum or pigeon chest is an uncommon chest wall deformity characterized by a protruding breast bone (sternum) and ribs caused by an overgrowth of the costal cartilages. It can cause a multitude of problems, including severe pain from an intercostal neuropathy, respiratory dysfunction, and psychologic issues from the cosmetic disfigurement. Pulmonary function indices, namely, forced expiratory volume over 1 s, forced vital capacity, vital capacity, and total lung capacity are markedly compromised in pectus excavatum...
July 2017: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/28738883/recurrent-c-g1636a-p-g546s-mutation-of-col2a1-in-a-chinese-family-with-skeletal-dysplasia-and-different-metaphyseal-changes-a-case-report
#8
Jing Chen, Xiaomin Ma, Yulin Zhou, Guimei Li, Qiwei Guo
BACKGROUND: Mutations in the COL2A1 gene cause type II collagenopathies characterized by skeletal dysplasia with a wide spectrum of phenotypic severity. Most COL2A1 mutations located in the triple-helical region, and the glycine to bulky amino acid substitutions (e.g., glycine to serine) in the Gly-X-Y repeat were identified frequently. However, the same COL2A1 mutations are associated with different phenotypes and the genotype-phenotype relationship is still poorly understood. Therefore, the studies of more patients about the recurrent mutations in COL2A1 will be needed for further research to provide more comprehensive clinical and genetic data...
July 24, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28714634/-pectus-excavatumn-and-carinatum-in-children-and-adolescents-what-to-say-what-to-do
#9
Pierre Lascombes, Isabelle Ruchonnet-Métrailler, Maurice Beghetti, Armand Bottani, James Wilde
In children presenting with a pectus excavatum (PE) or pectus carinatum (PC) an underlying syndrome including Marfan's syndrome needs to be excluded. In adolescents, severe chest wall deformities may cause cardiac or respiratory problems but most commonly they have a psychological impact. The conservative treatment is a Vacuum Bell for PE, and a Dynamic Compression System for PC ; they play an increasing role in young patients. These devices need to be worn multiple hours per day for several months for an optimal result...
February 15, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28618768/psychosocial-functioning-in-pediatric-patients-with-pectus-excavatum-and-pectus-carinatum
#10
Aliye Tuğba Bahadir, Pınar Kuru Bektaşoğlu, Aylin Çakiroğlu Eser, Ceyda Afacan, Mustafa Yüksel
BACKGROUND/AIM: Pectus excavatum and pectus carinatum are the most commonly seen anterior chest wall deformities. Recent studies reveal that minimal invasive repair of pectus deformities improves the quality of life. Our aim is to assess the psychosocial functioning and sociodemographic characteristics of pediatric patients with pectus deformities and evaluate the differences between patients operated on with minimal invasive repair techniques and nonoperated patients. MATERIALS AND METHODS:  Thirty-two patients with pectus deformities who were operated on 6 months or more before and 31 nonoperated patients participated in the study...
June 12, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28499711/does-an-external-chest-wall-measurement-correlate-with-a-ct-based-measurement-in-patients-with-chest-wall-deformities
#11
Franziska Ewert, Julia Syed, Sonja Wagner, Manuel Besendoerfer, Roman T Carbon, Stefan Schulz-Drost
BACKGROUND: Measurements in chest wall deformities are typically conducted using a thorax caliper or a CT scan of the chest wall. This paper focuses on the possible correlation between these two methods to validate the reliability of the thorax caliper, minimize radiation exposure, and limit the usage of expensive imaging techniques. METHODS: We evaluated 95 consecutive patients (77 pectus excavatum (PE), 17 pectus carinatum (PC), 1 mixed deformity) who received surgical correction of the anterior chest wall...
April 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28363366/minimal-invasive-repair-of-pectus-excavatum-and-carinatum
#12
REVIEW
Hans Pilegaard, Peter Bjørn Licht
Minimal invasive surgery has become the gold standard for surgical repair of pectus excavatum. The procedure can be performed as fast-track surgery and cosmetic results are excellent. In addition, cardiac performance improves after correction. With increased awareness on the Internet, the number of patients who seek help continues to rise, primarily for cosmetic reasons. Pectus carinatum is much less frequent than pectus excavatum. Over the past decade surgery has largely been replaced by compression techniques that use a brace, and cosmetic results are good...
May 2017: Thoracic Surgery Clinics
https://www.readbyqxmd.com/read/28274516/the-dynamic-compression-brace-for-pectus-carinatum-intermediate-results-in-286-patients
#13
Sjoerd A de Beer, Martin Gritter, Justin R de Jong, Ernst L W van Heurn
BACKGROUND: Dynamic brace compression is a novel treatment for patients with pectus carinatum. The dynamic compression system contains a device to measure the flexibility of the thoracic wall and regulate the pressure of the brace. METHODS: Patients referred to our pediatric surgical center were screened for treatment with the dynamic compression brace. Patients with a pressure of initial correction (PIC) of 10.0 pounds per square inch or less were offered treatment with the brace...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28196662/a-less-intensive-bracing-protocol-for-pectus-carinatum
#14
George Wahba, Ahmed Nasr, Marcos Bettolli
OBJECTIVES: Despite the widespread use of bracing to correct Pectus carinatum (PC) there is no consensus in the number of hours per day patients are instructed to wear the brace. In our practice, we use a less rigorous protocol of 8-12h/day. We sought to evaluate our results and those in the literature to determine whether more intensive usage is necessary. STUDY DESIGN: We reviewed the outcomes of patients with PC treated at our institution between 2012 and 2015...
November 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28044041/visual-diagnosis-a-child-with-presumed-reactive-airway-disease-pectus-carinatum-and-aortic-root-dilation
#15
Aamir Jeewa, Shaine A Morris, William J Dreyer, Iki Adachi, Susan W Denfield, E Dean McKenzie
No abstract text is available yet for this article.
January 2017: Pediatrics in Review
https://www.readbyqxmd.com/read/27979613/mucopolysaccharidosis-iva-and-glycosaminoglycans
#16
REVIEW
Shaukat Khan, Carlos J Alméciga-Díaz, Kazuki Sawamoto, William G Mackenzie, Mary C Theroux, Christian Pizarro, Robert W Mason, Tadao Orii, Shunji Tomatsu
Mucopolysaccharidosis IVA (MPS IVA; Morquio A: OMIM 253000) is a lysosomal storage disease with an autosomal recessive trait caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to accumulation of specific glycosaminoglycans (GAGs): chondroitin-6-sulfate (C6S) and keratan sulfate (KS). C6S and KS are mainly produced in the cartilage. Therefore, the undegraded substrates are stored primarily in cartilage and in its extracellular matrix (ECM), leading to a direct impact on cartilage and bone development, and successive systemic skeletal dysplasia...
January 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27942072/pectus-carinatum-repair-in-an-adolescent-with-hyperhomocysteinaemia-anaesthetic-implications
#17
Prachi Kar, Suresh Kumar Chintha, Padmaja Durga, Ramachandran Gopinath
No abstract text is available yet for this article.
November 2016: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/27836367/success-and-duration-of-dynamic-bracing-for-pectus-carinatum-a-four-year-prospective-study
#18
Sherif Emil, Marika Sévigny, Kathleen Montpetit, Robert Baird, Jean-Martin Laberge, Jade Goyette, Ian Finlay, Guylaine Courchesne
BACKGROUND: This study sought to establish factors that can prognosticate outcomes of bracing for pectus carinatum (PC). METHODS: Prospective data were collected on all patients enrolled in a dynamic bracing protocol from July 2011 to July 2015. Pressure of correction (POC) was measured at initiation of treatment, and pressure of treatment (POT) was measured pre- and post-adjustment at every follow-up visit. Univariate and Cox regression analysis tested the following possible determinants of success and bracing duration: age, sex, symmetry, POC, and POT drop during the first two follow-up visits...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27825548/is-the-abramson-technique-effective-in-pectus-carinatum-repair
#19
Ozgur Katrancioglu, Yucel Akkas, Sule Karadayi, Ekber Sahin, Melih Kaptanoğlu
BACKGROUND: The minimally invasive pectus carinatum (PC) surgery described by Abramson has been performed in many centers. We have been using this technique since 2011. This article describes our experience with PC correction. METHODS: Between 2011 and 2016, 32 patients at our institution underwent minimally invasive repair of a PC deformity. All patients presented with cosmetic complaints. The deformity involved the lower sternum (all had chondrogladiolar type PC), and three patients had asymmetrical deformities...
November 4, 2016: Asian Journal of Surgery
https://www.readbyqxmd.com/read/27761411/30-months-follow-up-of-an-early-enzyme-replacement-therapy-in-a-severe-morquio-a-patient-about-one-case
#20
J Do Cao, A Wiedemann, T Quinaux, S F Battaglia-Hsu, L Mainard, R Froissart, C Bonnemains, S Ragot, B Leheup, P Journeau, F Feillet
Patients under 5 years were not evaluated in the phase-3 study for enzyme replacement therapy (ERT) in MPS IV A. Here we describe the evolution of a severe Morquio A pediatric patient who was diagnosed at 19 months old and treated by ERT at 21 months old for the next 30 months. Applying the standard ERT protocol on this very young patient appeared to reduce his urinary excretion of glycosaminoglycans (GAGs); the improvements in both the 6 minute-walk test (6MWT) and the stair climb test, however, were no different than those reported in the nature history study...
December 2016: Molecular Genetics and Metabolism Reports
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