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Pectus carinatum

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https://www.readbyqxmd.com/read/29890445/evidence-of-aortic-dissection-and-marfan-syndrome-in-a-mummy-from-the-capuchin-catacombs-of-palermo-sicily
#1
Stephanie Panzer, Randall C Thompson, Klaus Hergan, Albert R Zink, Dario Piombino-Mascali
The authors report on the assessment of an anthropogenic mummy of a young man from the Capuchin Catacombs of Palermo, Sicily, tentatively dated from the mid- to late 19th century AD. The mummy was investigated by full-body CT examination. CT images clearly showed aortic dissection classified as Stanford-A. Due to the relation of aortic dissection to inherited connective tissue diseases in young people, such as Marfan syndrome, conspicuous and pathological findings possibly indicating the presence of underlying Marfan syndrome were assessed...
June 8, 2018: International Journal of Paleopathology
https://www.readbyqxmd.com/read/29878177/disturbingly-high-fracture-rate-of-stratos-bars-in-pectus-corrections
#2
Nagarajan Muthialu, Denise McIntyre, Neil McIntosh, Julie Plumridge, Martin J Elliott
OBJECTIVES: Several procedures have been described to correct pectus excavatum and carinatum. We have used a modified Ravitch procedure (STRATOS titanium bars) for patients who were unsuitable for the minimally invasive Nuss procedure. The operation produced excellent cosmetic results, but we have noted several fractures and displacements of the STRATOS bars. METHODS: We reviewed all our STRATOS patients since first use in 2009 until 2014. We collated the following data of each patient: diagnosis, severity of pectus deformity, comorbidity, previous operations for pectus, age at bar implantation, the number of bars implanted and duration of implantation...
June 5, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29866441/bilateral-single-shot-erector-spinae-plane-block-for-pectus-excavatum-and-pectus-carinatum-surgery-in-2-pediatric-patients
#3
M A Nardiello, M Herlitz
Numerous publications have emerged on the application of erector spinae plane block in adult population. There are few reports of the use of this block in pediatric patients. The objective is to report 2 cases of adolescents, one diagnosed with pectus excavatum and the other one with pectus carinatum undergoing reconstructive surgery in which a bilateral single-shot erector spinae plane block was performed as an analgesic technique. The block was performed before surgery under general anesthesia in lateral decubitus position, guided by ultrasound using 20ml of 0...
June 1, 2018: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/29798996/-surgical-treatment-of-pectus-carinatum-in-children
#4
N S Narkhodzhaev, I Zh Turmetov, A K Karabekov
AIM: To improve the results of surgical treatment of pectus carinatum. MATERIAL AND METHODS: The work included 47 pectus carinatum patients aged 3-15 years for the period from 2000 to 2015. All patients were divided into 2 groups depending on surgical approach: control group - 23 (48.94%) children who underwent thoracoplasty by M.M. Ravitsh procedure with the use of device for pectus carinatum repair of our design; main group - 24 (51.06%) patients where our method of thoracoplasty based on changes of deformed ribs biomechanics was used...
2018: Khirurgiia
https://www.readbyqxmd.com/read/29779902/molecular-genetics-and-metabolism-special-edition-diagnosis-diagnosis-and-prognosis-of-mucopolysaccharidosis-iva
#5
REVIEW
Hira Peracha, Kazuki Sawamoto, Lauren Averill, Heidi Kecskemethy, Mary Theroux, Mihir Thacker, Kyoko Nagao, Christian Pizarro, William Mackenzie, Hironori Kobayashi, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji Orii, Tadao Orii, Toshiyuki Fukao, Shunji Tomatsu
Mucopolysaccharidosis IVA (MPS IVA, Morquio A syndrome) is an autosomal recessive disorder caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to the accumulation of specific glycosaminoglycans (GAGs), chondroitin-6-sulfate (C6S) and keratan sulfate (KS), which are mainly synthesized in the cartilage. Therefore, the substrates are stored primarily in the cartilage and its extracellular matrix (ECM), leading to a direct impact on bone development and successive systemic skeletal spondylepiphyseal dysplasia...
May 15, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29766672/ribcage-deformity-and-the-altered-breathing-pattern-in-children-with-osteogenesis-imperfecta
#6
Antonella LoMauro, Paolo Fraschini, Simona Pochintesta, Marianna Romei, Maria G D'Angelo, Andrea Aliverti
AIM: Osteogenesis Imperfecta (OI) is a genetic disease characterized by bones fragility and progressive deformity. Life expectancy is reduced in the non-lethal most severe type III form before the age of 10 years. The main cause of death in OI is respiratory insufficiency resulting from impaired thoracic function worsened by ribcage deformity and scoliosis. METHODS: We used opto-electronic plethysmography to study chest geometry, the ventilatory, and the thoraco-abdominal pattern at rest in supine position in children younger than 10 years...
May 15, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29744653/minimal-cosmetic-revision-required-after-minimally-invasive-pectus-repair
#7
Brittany L Murphy, Nimesh D Naik, Penny L Roskos, Amy E Glasgow, Christopher R Moir, Elizabeth B Habermann, Denise B Klinkner
BACKGROUND: Despite surgical correction procedures for pectus deformities, remaining cosmetic asymmetry may have significant psychological effects. We sought to evaluate factors associated with plastic surgery (PS) consultation and procedures for these deformities at an academic institution. METHODS: We reviewed patients aged 0-21 diagnosed with a pectus excavatum or carinatum deformity at our institution between January 2001 and October 2016. Pectus diagnoses were identified by ICD-9/ICD-10 codes and surgical repair by CPT codes; patients receiving PS consultation were identified by clinical note service codes...
May 9, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29628596/subarachnoid-block-a-safe-choice-for-cesarean-section-in-neurofibromatosis-patient-with-severe-kyphoscoliosis
#8
Ibrahim Demir, Ebru Tarıkçı Kılıç, Mehmet Salim Akdemir
Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuroectodermal hereditary disorder, in which spinal skeletal deformities are one of the manifestations. Pectus carinatum, temporomandibular joint dysfunction, and kyphoscoliosis can be seen with this genetic disorder which can lead to cardiorespiratory system problems and can cause difficulty in airway management. We present the anesthetic management of a NF1 patient with a sharp thoracolumbar dystrophic kyphoscoliosis who have been posted for emergency cesarean section as a result of fetal distress...
January 2018: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/29627275/growth-impairment-in-mucopolysaccharidoses
#9
REVIEW
Melodie Melbouci, Robert W Mason, Yasuyuki Suzuki, Toshiyuki Fukao, Tadao Orii, Shunji Tomatsu
Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that affect regulation of glycosaminoglycan (GAG) processing. In MPS, the lysosomes cannot efficiently break down GAGs, and the specific GAGs accumulated depend on the type of MPS. The level of impairment of breakdown varies between patients, making this one of the many factors that lead to a range of clinical presentations even in the same type of MPS. These clinical presentations usually involve skeletal dysplasia, in which the most common feature is bone growth impairment and successive short stature...
May 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29525272/bracing-of-pectus-carinatum-a-quantitative-analysis
#10
Tomasz Bugajski, Kartikeya Murari, Steven Lopushinsky, Marc Schneider, Janet Ronsky
BACKGROUND/PURPOSE: Primary treatment of pectus carinatum (PC) is performed with an external brace that compresses the protrusion. Patients are 'prescribed' a brace tightening force. However, no visual guides exist to display this force magnitude. The purpose of this study was to determine the repeatability of patients in applying their prescribed force over time and to determine whether the protrusion stiffness influences the patient-applied forces and the protrusion correction rate...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29500788/pigeon-chest-comparative-analysis-of-surgical-techniques-in-minimal-access-repair-of-pectus-carinatum-marpc
#11
REVIEW
Ancuta Muntean, Ionica Stoica, Amulya K Saxena
BACKGROUND: After minimally invasive repair for pectus excavatum (MIRPE), similar procedures for pectus carinatum were developed. This study aimed to analyse the various published techniques of minimal access repair for pectus carinatum (MARPC) and compare the outcomes. DATA SOURCES: Literature was reviewed on PubMed with the terms "pectus carinatum", "minimal access repair", "thoracoscopy" and "children". RESULTS: Twelve MARPC techniques that included 13 articles and 140 patients with mean age 15...
February 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29357992/force-required-for-correcting-the-deformity-of-pectus-carinatum-and-related-multivariate-analysis
#12
Chenghao Chen, Qi Zeng, Zhongzhi Li, Na Zhang, Jie Yu
OBJECTIVE: To measure the force required for correcting pectus carinatum to the desired position and investigate the correlations of the required force with patients' gender, age, deformity type, severity and body mass index (BMI). METHODS: A total of 125 patients with pectus carinatum were enrolled in the study from August 2013 to August 2016. Their gender, age, deformity type, severity and BMI were recorded. A chest wall compressor was used to measure the force required for correcting the chest wall deformity...
December 24, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29326877/natural-history-of-morquio-a-patient-with-tracheal-obstruction-from-birth-to-death
#13
Caitlin Doherty, Lauren W Averill, Mary Theroux, William G Mackenzie, Christian Pizarro, Robert W Mason, Shunji Tomatsu
Morquio A syndrome (mucopolysaccharidosis IVA, MPS IVA) is a lysosomal storage disease caused by a deficiency of N -acetylgalactosamine-6-sulfate sulfatase, resulting in systemic accumulation of the partially degraded glycosaminoglycans (GAGs), keratan sulfate and chondroitin-6-sulfate. The accumulation of these GAGs leads to distinguishing features as skeletal dysplasia with disproportionate dwarfism, short neck, kyphoscoliosis, pectus carinatum, tracheal obstruction, coxa valga, genu valgum, and joint laxity...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325984/minimally-invasive-repair-of-pectus-carinatum
#14
Mustafa Yuksel, Tunc Lacin, Nezih Onur Ermerak, Esra Yamansavci Sirzai, Bihter Sayan
BACKGROUND: The second most common deformity of the anterior chest wall, pectus carinatum, is a diverse deformity that has been largely managed using open techniques. This study reviews clinical experience with a newly designed bar for minimally invasive repair of pectus carinatum. METHODS: We reviewed the records of all patients recorded in our Chest Wall Deformities Clinical Database. Between January 2006 and November 2016, minimally invasive repair of pectus carinatum was performed in 172 patients...
March 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29092017/the-effect-on-cardiopulmonary-function-after-thoracoplasty-in-pectus-carinatum-a-systematic-literature-review
#15
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...
March 1, 2018: 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
#16
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
#17
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
OBJECTIVE:  Bracing for pectus carinatum (PC) has emerged as an alternative to surgical correction. However, predictive factors for bracing remain poorly understood, as much of the data have been reported from small series. MATERIALS AND METHODS:  We reviewed a prospective dataset in patients with PC who underwent dynamic compression bracing (DCB) from July 2011 to July 2016. Bracing was initiated in patients > 10 years of age with a significant PC and desire for bracing...
February 2018: 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
#18
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...
February 2018: Brain & Development
https://www.readbyqxmd.com/read/28893382/pain-and-sensory-disturbances-following-surgical-repair-of-pectus-carinatum
#19
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...
April 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28857530/-surgery-in-times-of-crisis-conservative-treatment-of-pectus-carinatum-by-static-corset
#20
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
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