Surg Neurol Int 2017 18;8:147. Epub 2017 Jul 18.

Department of Neurosurgery, Hospital Córdoba, Córdoba, Argentina.

Background: Cutaneomeningospinal angiomatosis or Cobb syndrome is a rare, not well understood phacomatosis that features metameric cutaneous and spinal arteriovenous malformations (AVMs). The first case was described in Boston in 1915, and since then, few more cases have been reported in the English literature. No case was found to be from Argentina. Read More

Childs Nerv Syst 2017 Jul 10. Epub 2017 Jul 10.

Division of Pediatric Neurosurgery, Department of Neurological Surgery, Saint Louis University School of Medicine, Saint Louis, MO, USA.

Background: The management of concomitant scoliosis and tethered cord syndrome in the non-spina bifida pediatric population is challenging. In the present study, we evaluate the efficacy of different treatment modalities and propose a treatment plan for the management of affected patients.

Methods: A systematic literature review was conducted by querying the MEDLINE, PubMed, Cochrane, EMBASE, Scopus, and Web of Science databases for papers published between January 1996 and June 2016 and reporting on concomitant scoliosis and tethered cord. Read More

Neuroscience 2017 Sep 4;358:261-268. Epub 2017 Jul 4.

Institute of Neuroscience and Psychology, University of Glasgow, United Kingdom. Electronic address:

Rett syndrome (RTT) is a neurological disorder characterized by motor and cognitive impairment, autonomic dysfunction and a loss of purposeful hand skills. In the majority of cases, typical RTT is caused by de novo mutations in the X-linked gene, MECP2. Alterations in the structure and function of neurons within the central nervous system of RTT patients and Mecp2-null mouse models are well established. Read More

Eur Spine J 2017 Jun 26. Epub 2017 Jun 26.

Department of Orthopaedic Surgery, Hospital for Joint Diseases, New York University Langone Medical Center, 301 East 17th Street, New York, NY, 10003, USA.

Objective: The aim of this study is to present our technique for a large focal correction of a partially flexible dropped head deformity through combined anterior and posterior osteotomies, as well as anterior soft tissue releases.

Methods: One patient with dropped head deformity underwent an anterior and posterior osteotomy with anterior soft tissue release.

Results: The patient recovered well, with postoperative radiographs demonstrating significant improvement in coronal and sagittal alignment. Read More

Diabetologia 2017 Jun 8. Epub 2017 Jun 8.

Institute for Molecular Medicine Finland (FIMM), Nordic EMBL Partnership for Molecular Medicine, University of Helsinki, Helsinki, Finland.

Aims/hypothesis: The aims of this study were to evaluate systematically the predictive power of comprehensive metabolomics profiles in predicting the future risk of type 2 diabetes, and to identify a panel of the most predictive metabolic markers.

Methods: We applied an unbiased systems medicine approach to mine metabolite combinations that provide added value in predicting the future incidence of type 2 diabetes beyond known risk factors. We performed mass spectrometry-based targeted, as well as global untargeted, metabolomics, measuring a total of 568 metabolites, in a Finnish cohort of 543 non-diabetic individuals from the Botnia Prospective Study, which included 146 individuals who progressed to type 2 diabetes by the end of a 10 year follow-up period. Read More

J Neurosurg Sci 2017 May 31. Epub 2017 May 31.

IRCCS Istituto Ortopedico Galeazzi, Milan, Italy.

Background: The aim of this study was to determine the effects of magnetically controlled growing rods surgery (MCGRS) of the scoliosis on pulmonary function in children with neuromuscular scoliosis.

Methods: Seven patients, 85.7% female (mean ± SD age: 6. Read More

Mol Ther Methods Clin Dev 2017 Jun 22;5:180-190. Epub 2017 Apr 22.

Institute of Neuroscience and Psychology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow G12 8QQ, UK.

Rett syndrome (RTT), caused by loss-of-function mutations in the MECP2 gene, is a neurological disorder characterized by severe impairment of motor and cognitive functions. The aim of this study was to investigate the impact of vector design, dosage, and delivery route on the efficacy and safety of gene augmentation therapy in mouse models of RTT. Our results show that AAV-mediated delivery of MECP2 to Mecp2 null mice by systemic administration, and utilizing a minimal endogenous promoter, was associated with a narrow therapeutic window and resulted in liver toxicity at higher doses. Read More

Mol Ther Methods Clin Dev 2017 Jun 19;5:106-115. Epub 2017 Apr 19.

University of North Carolina (UNC) Gene Therapy Center, Chapel Hill, NC 27599, USA.

Intravenous administration of adeno-associated virus serotype 9 (AAV9)/hMECP2 has been shown to extend the lifespan of Mecp2(-/y) mice, but this delivery route induces liver toxicity in wild-type (WT) mice. To reduce peripheral transgene expression, we explored the safety and efficacy of AAV9/hMECP2 injected into the cisterna magna (ICM). AAV9/hMECP2 (1 × 10(12) viral genomes [vg]; ICM) extended Mecp2(-/y) survival but aggravated hindlimb clasping and abnormal gait phenotypes. Read More

Clin Spine Surg 2017 May;30(4):E448-E453

*Department of Orthopedics, The First Affiliated Hospital of Zhengzhou University †Spinal Deformity Center of Henan Province, Zhengzhou, China.

Study Design: This is a retrospective study.

Objectives: The treatment of severe spinal deformities is still a difficult surgical challenge. It is generally significant to identify the related complications performed to a consecutive series of patients with severe spinal deformity and managed by 1-stage posterior spinal osteotomy. Read More

Acta Neurochir (Wien) 2017 Jun 22;159(6):1129-1135. Epub 2017 Apr 22.

Department of Orthopaedic Surgery, Biomedical Research Institute, Pusan National University School of Medicine, 179 Gudeok-ro, Seo-gu, Busan, 602-739, South Korea.

Background: Several different techniques exist to treat degenerative lumbar foraminal stenosis. Failure to adequately decompress the lumbar foramen may lead to failed back surgery syndrome. However, wide decompression often causes spinal instabilities or may require an additional fusion surgery. Read More

Anaesthesia 2017 Jul 13;72(7):879-888. Epub 2017 Apr 13.

Department of Anaesthesiology and Critical Care, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Optimal immunological homoeostasis determines the long-term recovery of patients in the postoperative period. The functional adaptability of monocytes plays a pivotal role in adjusting the host's response to an insult, immunostasis and long-term health, and may help to determine successful recovery. We undertook a longitudinal analysis of the functional adaptability of monocytes in 20 patients undergoing heart surgery with cardiopulmonary bypass, as a model of severe stress. Read More

Orthop Traumatol Surg Res 2017 Jun 1;103(4):549-557. Epub 2017 Apr 1.

The MSK Lab, Charing Cross Campus, laboratory Block, London W6 8RP Imperial College, London, United Kingdom.

Relationship between hip pathoanatomy and symptomatic FAI has been reported to be weak. This is explained by the reciprocal interaction between proximal femur and acetabular anatomies, but potentially also by the individual spine-hip relations (SHR). The key-answer for a complete understanding of the pathophysiology of FAI might lie in the comprehension of the SHRs, which have not yet been fully addressed. Read More

Orthop Traumatol Surg Res 2017 Jun 1;103(4):559-568. Epub 2017 Apr 1.

London hip unit, 30, Devonshire St, W1G 6PU London, UK.

Sagittal pelvic kinematics along with spino-pelvic angular parameters have recently been studied by numerous investigators for their effect on total hip replacement (THR) clinical outcomes, but many issue of spine-hip relations (SHR) are currently unexplored. Therefore, our review aims at clarifying the following questions: is there any evidence of a relationship between articular impingement/dislocation risk in primary THR and (1) certain sagittal pelvic kinematics patterns, (2) pelvic incidence, and (3) types of SHRs? A systematic review of the existing literature utilising PubMed and Google search engines was performed in January 2017. Only clinical or computational studies published in peer-reviewed journals over the last five years in either English or French were reviewed. Read More

Pediatr Neurol 2017 May 7;70:20-25. Epub 2017 Feb 7.

University of Alabama at Birmingham, School of Medicine, Birmingham, Alabama; University of Alabama at Birmingham, Civitan International Research Center, Birmingham, Alabama. Electronic address:

Background: Scoliosis is prominent in Rett syndrome (RTT). Following the prior report from the US Natural History Study, the onset and progression of severe scoliosis (≥40° Cobb angle) and surgery were examined regarding functional capabilities and specific genotypes, addressing the hypothesis that abnormal muscle tone, poor oral feeding, puberty, and delays or absence of sitting balance and ambulation may be responsible for greater risk in RTT.

Methods: The multicenter RTT Natural History Study gathered longitudinal data for classic RTT, including mutation type, scoliosis, muscle tone, sitting, ambulation, hand function, and feeding. Read More

Eur Spine J 2017 Mar 16. Epub 2017 Mar 16.

School of Medicine, University of Bari Aldo Moro, AOU Consorziale Policlinico, Department of Basic Medical Sciences, Neuroscience and Sense Organs, Orthopaedic, Trauma and Spine Unit, Piazza Giulio Cesare 11, 70100, Bari, Italy.

Purpose: The study of the interrelation between hip and spine disorders is gaining increasing importance in the last years, but the link between Hip Osteoarthritis (HOA) and Low Back Pain (LBP) remains still unclear. Aim of the study is to assess the relationship between Femoral Neck Anteversion (FNA), LBP, and spinopelvic parameters in patients undergoing Total Hip Replacement (THR) for unilateral severe primary HOA.

Materials And Methods: 91 patients were recruited. Read More

Eur Spine J 2017 Jul 9;26(7):1862-1870. Epub 2017 Mar 9.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, 88 Jiefang Road, Hangzhou, 310009, China.

Background: Postural deformities in the coronal plane were frequent and disabling complications of PD, which reduces the quality of life of patients. This study aimed to garner greater attention to the Parkinson disease (PD)-related postural trunk deviations in the coronal plane by exploring a method for diagnosis because of the lack of any uniform diagnostic criteria and epidemiological studies. It also aimed to provide correlation data in the Chinese PD patients. Read More

Acta Ortop Mex 2016 Jul-Aug;30(4):196-200

Servicio de Ortopedia, Hospital General Xoco, Av. México Coyoacán S/N, Benito Juárez, Gral Anaya, 03340 , Ciudad de México, México.

Background: The non-lethal variant of the Escobar or multiple pterygium syndrome is an entity of autosomal recessive inheritance linked to the X chromosome; it is characterized by multiple pterygia (hence its name) located mainly in the neck (95%) and armpits (55%), as well as other orthopedic malformations such as a vertical talus, congenital hip dislocation, and congenital scoliosis.

Objective: To present an optional surgical technique for the management of severe spinal deformities.

Case Report: Twelve-year-old female diagnosed with Escobar syndrome with severe scoliosis which conditions malformations of the chest with lung involvement, producing mechanical ventilatory restriction and increasing the risk of severe lower respiratory tract infection. Read More

Am J Physiol Endocrinol Metab 2017 May 28;312(5):E429-E436. Epub 2017 Feb 28.

CNR Institute of Clinical Physiology, Pisa, Italy.

While hyperthyroidism and hypothyroidism cause dysglycemia, the relationship between thyroid hormone levels within the normal range and insulin resistance (IR) is unclear. In 940 participants with strictly normal serum concentrations of free triiodothyronine (fT3), free thyroxine (fT4), and thyroid-stimulating hormone (TSH) followed up for 3 yr, we measured insulin sensitivity (by the insulin clamp technique) and 35 circulating metabolites. At baseline, across quartiles of increasing fT3 levels (or fT3/fT4 ratio) most features of IR emerged [i. Read More

Redox Biol 2017 Aug 14;12:121-128. Epub 2017 Feb 14.

Linus Pauling Institute, Oregon State University, Corvallis, OR 97331, United States. Electronic address:

Electric conductivity in plasma is the balance between oxidized and reduced molecules (static Oxidation-Reduction Potential, sORP) and the amount of readily oxidizable molecules (capacity ORP, cORP). Adults with metabolic syndrome (MetS) have increased inflammation, dyslipidemia and oxidative stress; therefore, participants with MetS were hypothesized to have higher plasma sORP and lower cORP than those measures in healthy adults. Heparin-anticoagulated plasma from healthy and age- and gender-matched individuals with MetS (BMI: 22. Read More

Eur Spine J 2017 Jun 8;26(6):1595-1599. Epub 2017 Feb 8.

Department of Orthopaedic Surgery, G05.228, University Medical Center Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands.

Purpose: Primary ciliary dyskinesia (PCD) is a respiratory syndrome in which 'random' organ orientation can occur; with approximately 46% of patients developing situs inversus totalis at organogenesis. The aim of this study was to explore the relationship between organ anatomy and curve convexity by studying the prevalence and convexity of idiopathic scoliosis in PCD patients with and without situs inversus.

Methods: Chest radiographs of PCD patients were systematically screened for existence of significant lateral spinal deviation using the Cobb angle. Read More

Hum Mol Genet 2016 10;25(20):4389-4404

Institute of Neuroscience and Psychology, College of Medical, Veterinary & Life Sciences, University of Glasgow, Glasgow, UK.

Rett syndrome (RTT) is a severe genetic disorder resulting from mutations in the X-linked MECP2 gene. MeCP2 protein is highly expressed in the nervous system and deficiency in the mouse central nervous system alone recapitulates many features of the disorder. This suggests that RTT is primarily a neurological disorder, although the protein is reportedly widely expressed throughout the body. Read More

Nat Rev Neurol 2017 Jan 9;13(1):37-51. Epub 2016 Dec 9.

Telethon Kids Institute, 100 Roberts Road, Subiaco, Perth, Western Australia 6008, Australia.

In the 50 years since Andreas Rett first described the syndrome that came to bear his name, and is now known to be caused by a mutation in the methyl-CpG-binding protein 2 (MECP2) gene, a compelling blend of astute clinical observations and clinical and laboratory research has substantially enhanced our understanding of this rare disorder. Here, we document the contributions of the early pioneers in Rett syndrome (RTT) research, and describe the evolution of knowledge in terms of diagnostic criteria, clinical variation, and the interplay with other Rett-related disorders. We provide a synthesis of what is known about the neurobiology of MeCP2, considering the lessons learned from both cell and animal models, and how they might inform future clinical trials. Read More

Spine (Phila Pa 1976) 2016 Oct;41(20):1600-1605

*Department of Neurosurgery, University of California San Diego, San Diego, California †Department of Orthopedics and Scoliosis Center, Rady Children's Hospital, San Diego, California ‡Department of Pediatric Neurosurgery, Rady Children's Hospital, San Diego, California §Department of Orthopedic Surgery, University of California San Diego, San Diego, California.

Study Design: This was a retrospective study of patients with Chiari I (CM I) and Chiari II (CM II) malformations, tethered cord syndrome, and syringomyelia examining the effect of neurosurgery on scoliosis.

Objective: The aim of this study was to determine the factors affecting spinal deformity progression in patients with diseases of the neural axis following neurosurgical management.

Summary Of Background Data: There have been attempts to explain which factors influence the spinal deformity in diseases of the neural axis with varying results. Read More

Neurochem Int 2016 Nov 10;100:110-119. Epub 2016 Sep 10.

Institute of Neuroscience and Psychology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, G12 8QQ, UK. Electronic address:

To quantify the full range of tryptophan metabolites along the kynurenine pathway, a liquid chromatography - tandem mass spectrometry method was developed and used to analyse brain extracts of rodents treated with the kynurenine-3-mono-oxygenase (KMO) inhibitor Ro61-8048 during pregnancy. There were significant increases in the levels of kynurenine, kynurenic acid, anthranilic acid and 3-hydroxy-kynurenine (3-HK) in the maternal brain after 5 h but not 24 h, while the embryos exhibited high levels of kynurenine, kynurenic acid and anthranilic acid after 5 h which were maintained at 24 h post-treatment. At 24 h there was also a strong trend to an increase in quinolinic acid levels (P = 0. Read More

J Psychosom Res 2016 Sep 19;88:42-7. Epub 2016 Jul 19.

Department of Psychology, University of Bath, Claverton Down, Bath, UK. Electronic address:

Objective: Psychological Flexibility (PF) is a relatively new concept in physical health. It can be defined as an overarching process of being able to accept the presence of wanted/unwanted experiences, choosing whether to change or persist in behaviour in response to those experiences. Associations between processes of PF and quality of life (QoL) have been found in long-term health conditions such as chronic pain, PF has not yet been applied to Chronic Fatigue Syndrome/Myalgic Encephalomyelitis (CFS/ME). Read More

Medicine (Baltimore) 2016 Jun;95(26):e3775

Department of Orthopedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Guillain-Barré syndrome (GBS) is an acute autoimmune inflammatory demyelinating polyneuropathy that mostly affects the peripheral nervous system. Little is reported about spinal deformity associated with GBS. This study aims to present a case of scoliosis occurring in the setting of GBS. Read More

Braz J Anesthesiol 2016 Jul-Aug;66(4):408-13. Epub 2014 Nov 27.

Anesthesiology Department, Hospital Santa Maria, Lisbon, Portugal.

Background: Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. Read More

Scoliosis Spinal Disord 2016 2;11. Epub 2016 Mar 2.

Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitazato, Minami, Sagamihara, Kanagawa 252-0374 Japan.

Background: Jeune syndrome (asphyxiating thoracic dystrophy) is an autosomal recessive disorder with constriction and narrowing of the thorax. To our knowledge, there are no reports regarding spinal deformity and correction in Jeune syndrome. Herein, we report two cases of spinal correction in patients with Jeune syndrome, and review the literature. Read More

Rev Bras Anestesiol 2016 Jul-Aug;66(4):408-13. Epub 2016 May 4.

Departamento de Anestesiologia, Hospital Santa Maria, Lisboa, Portugal.

Background: Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. Read More

Eur Spine J 2016 Oct 3;25(10):3288-3293. Epub 2016 May 3.

Department of Orthopedics, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Zhongshan Road 321, Nanjing, 210008, China.

Purpose: To investigate surgical outcomes and complications of scoliosis associated with Marfan syndrome.

Methods: Inclusion criteria were patients who were 10-20 years of age, had a diagnosis of Marfan syndrome by the Ghent nosology, had scoliosis and had undergone spinal fusion, and had at least 2 years of postoperative follow-up. The medical records of all patients were reviewed for age at the time of surgery, surgical procedures performed, instrumentation type, estimated blood loss (EBL) during surgery, operation time and complications related to surgery. Read More

Eur Spine J 2016 Oct 12;25(10):3301-3307. Epub 2016 Apr 12.

Department of Orthopaedics, All India Institute of Medical Sciences (AIIMS), Ansari Nagar, New Delhi, 110029, India.

Objective: To evaluate clinical and radiological outcomes of growing rod (GR) in the management of Early Onset Scoliosis (EOS) with intraspinal anomalies.

Background Data: The effect of repeated distractions following GR, in the presence of intraspinal anomalies has not been studied.

Methods: During 2007-2012, 46 patients underwent fusionless surgery. Read More

Rev Sci Tech 2015 Dec;34(3):961-75

A quantitative model was developed to estimate the likelihood of an incursion of porcine reproductive and respiratory syndrome virus (PRRSV) into New Zealand through the importation of fresh consumer-ready cuts of pig meat. A sensitivity analysis of all the inputs used in this model illustrated the importance of correctly modelling the available 'dose-response' data, and a mechanistic Beta-Poisson model was shown to be the most appropriate method for this in the authors' assessment. The output of this model predicts an average of approximately 1,200 years between PRRSV introductions resulting in primary infections in New Zealand. Read More

Indian J Orthop 2016 Jan-Feb;50(1):80-6

Department of Neurology, Celal Bayar University School of Medicine, Turkey.

Background: Tethered cord syndrome (TCS) is a progressive clinical entity that arises from abnormal spinal cord tension. Scoliosis may be a unique symptom in TCS. The aim of this study is to investigate prognosis after releasing the filum terminale in scoliosis due to TCS with/without findings in magnetic resonance imaging (MRI) and to draw attention to the importance of somatosensorial evoked potentials (SSEP) on the differential diagnosis of idiopathic scoliosis versus scoliosis due to TCS with normal appearance of filum terminale and conus medullaris. Read More

PLoS Negl Trop Dis 2016 Mar 2;10(3):e0004530. Epub 2016 Mar 2.

Nevada Center for Infectious Disease Forecasting, University of Nevada, Reno, Nevada, United States of America.

Introduction: Reports of high rates of primary microcephaly and Guillain-Barré syndrome associated with Zika virus infection in French Polynesia and Brazil have raised concerns that the virus circulating in these regions is a rapidly developing neuropathic, teratogenic, emerging infectious public health threat. There are no licensed medical countermeasures (vaccines, therapies or preventive drugs) available for Zika virus infection and disease. The Pan American Health Organization (PAHO) predicts that Zika virus will continue to spread and eventually reach all countries and territories in the Americas with endemic Aedes mosquitoes. Read More

Global Spine J 2016 Feb 5;6(1):7-13. Epub 2015 Jun 5.

Center for Cervical Spine, Washington University Orthopedics, Washington University in St. Louis, Saint Louis, Missouri, United States.

Study Design Retrospective study. Objective Cervical scoliosis is a rare condition that can arise from various etiologies. Few reports on the surgical management of cervical scoliosis exist. Read More

J Craniovertebr Junction Spine 2015 Oct-Dec;6(4):200-5

Department of Orthopaedics and Traumatology, Spine Surgery and Arthroplasty Clinic, Metin Sabanci Baltalimani Bone Disease Education and Research Hospital, Istanbul, Turkey.

Aims: In the treatment of thoracic insufficiency syndrome, the main aim is to maintain spinal and thoracic growth in order to continue respiratory functions. Vertical expandable prosthetic titanium rib (VEPTR) device application is a method of choice especially in the congenital cases with a thoracic deformity. In our study, we evaluated the effect of VEPTR on growth in congenital scoliosis. Read More

Spine (Phila Pa 1976) 2016 Jun;41(12):1022-7

Institute of Orthopaedics and Musculoskeletal Science, University College London, the Royal National Orthopaedic Hospital, Stanmore, UK.

Study Design: A retrospective study.

Objective: Rate of neurological injuries is widely reported for spinal deformity surgery. However, few have included the influence of the subtypes and severity of the deformity, or anterior versus posterior corrections. Read More

Spine (Phila Pa 1976) 2016 May;41(10):856-63

*Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia; School of Physiotherapy and Exercise Science, Curtin University, Perth, WA, Australia †Department of Orthopaedics, Royal Children's Hospital, Melbourne, VIC, Australia ‡Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia §Disciplines of Genetic Medicine and Paediatrics and Child Health, The University of Sydney, Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, NSW, Australia ¶Discipline of Paediatrics and Child Health, The University of Sydney, The Children's Hospital at Westmead, Sydney, NSW, Australia; The Sydney Children's Hospitals Network (Westmead), Sydney, NSW, Australia ||Department of Radiology, Princess Margaret Hospital for Children, Perth, WA, Australia **Paediatric Spine Research Group, Queensland University of Technology and Mater Health Services, Brisbane, QLD, Australia ††Department of Surgery, University of Queensland, Brisbane, QLD, Australia ‡‡Department of Orthopaedics, The Children's Hospital at Westmead, Sydney, NSW, Australia §§Discipline of Orthopaedics and Trauma, University of Adelaide, Adelaide, SA, Australia; Department of Orthopaedic Surgery, Women's and Children's Hospital, Adelaide, SA, Australia.

Study Design: Population-based longitudinal observational study.

Objective: To describe the prevalence of scoliosis in Rett syndrome, structural characteristics and progression, taking into account the influences of age, genotype, and ambulatory status.

Summary Of Background Data: Scoliosis is the most common orthopedic comorbidity in Rett syndrome yet very little is known about its natural history and influencing factors such as age, genotype, and ambulatory status. Read More

Dev Med Child Neurol 2016 Jun 11;58(6):632-8. Epub 2015 Dec 11.

Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.

Aim: Scoliosis is a common comorbidity in Rett syndrome and spinal fusion may be recommended if severe. We investigated the impact of spinal fusion on survival and risk of severe lower respiratory tract infection in Rett syndrome.

Method: Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry, and from the Australian Institute of Health and Welfare National Death Index database. Read More

Nature 2015 Oct;526(7573):331-2

Institute of Neuroscience and Psychology, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow G12 8QQ, UK.

Int Wound J 2016 Oct 1;13(5):1009-10. Epub 2015 Oct 1.

Cancer Research Center, Semnan University of Medical Sciences, Semnan, Iran.

Cobb syndrome (Cutaneomeningospinal Angiomatosis) is a rare segmental neurocutaneous syndrome associated with metameric cutaneous and spinal cord arteriovenous malformations (AVMs). In this syndrome, capillary malformation or angiokeratoma-like lesions are formed in a dermatomal distribution, with an AVM in the corresponding segment of the spinal cord. The spinal cord lesions can cause neurological disorder and paraplegia, which typically develop during young adulthood. Read More

Spine (Phila Pa 1976) 2016 Jan;41(1):46-52

*Vanderbilt Orthopaedic Institute, Medical Center East, Nashville, TN †Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN.

Study Design: Retrospective chart and radiographic review.

Objective: To evaluate spinopelvic fixation technical advancements for the treatment of neuromuscular scoliosis.

Summary Of Background Data: Implants for vertebral and pelvic fixation have evolved without data demonstrating the benefit for neuromuscular scoliosis. Read More

Ann Neurosci 2015 Jul;22(3):191-3

Department of Medicine, Medical College and Hospital, Kolkata.

Cobb syndrome is an exceedingly rare clinical condition defined by the presence of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We report the case of a 14-year-old boy who presented with sudden onset paraplegia. Physical examination showed port-wine stains over buttock and thigh. Read More

Pediatr Neurosurg 2015 6;50(3):133-44. Epub 2015 Jun 6.

Department of Neurosurgery, Diyarbakır Bismil State Hospital, Diyarbakır, Turkey.

Purpose: Defective posterior spinal arch and paraspinal musculature lead to progressive kyphosis in patients with myelomeningocele. Kyphosis decreases the patients' functional status and quality of life. To correct or prevent further deterioration, different surgical techniques have been introduced. Read More

J Orthop Surg Res 2015 May 21;10:73. Epub 2015 May 21.

Department of Orthopedics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1 shuai fu yuan, wang fu jing street, Dong Cheng District, Beijing, 100730, People's Republic of China.

Background: The X-ray films of the patients with Marfan syndrome scoliosis (MSS) look like those with adolescent idiopathic scoliosis (AIS). In literature, there are many reports on the correction results of AIS, while there are a few studies focused on the difference of the correction results between MSS and AIS. This study aims to analyze whether there are differences of posterior correction surgery in MSS and AIS. Read More

Am J Respir Crit Care Med 2015 Apr;191(7):728-30

1 University of North Carolina Chapel Hill, North Carolina.

Ann Thorac Surg 2015 Mar;99(3):753-4

Washington University School of Medicine, 1600 Glenarm Place, Apt 3002, Denver, CO 80202. Electronic address:

Clin Toxicol (Phila) 2015 Mar 11;53(3):185-7. Epub 2015 Feb 11.

Department of Emergency Medicine, Upstate New York Poison Center, SUNY Upstate Medical University , Syracuse, NY , USA.

Unlabelled: Metaxalone has only recently been associated with serotonin syndrome. The mechanism of action of this centrally acting muscle relaxant is unknown; however, the observation of serotonin syndrome in patients with metaxalone overdose suggests a role in the serotonergic pathway.

Case Report: (Case 1) A 29-year-old woman with overdose of metaxalone presented to the emergency department with altered mental status, seizure-like activity, hyperthermia, rigidity in the lower extremities, myoclonus, and hyperreflexia. Read More

J Pediatr Orthop 2015 Dec;35(8):783-97

*Department of Orthopaedics, College of Medicine, University of Texas Health Science Center at San Antonio (UTHSCSA) †Spinal & Thoracic Treatment and Research (STTAR) Center, Children's Hospital of San Antonio §Pediatric, Orthopaedic, & Scoliosis Center of South Texas, San Antonio ‡University of Texas Medical School at Houston, Houston, TX ∥Division of Orthopaedics, Children's Hospital of Philadelphia, Philadelphia, PA.

Background: Jeune syndrome (JS) often results in lethal thoracic insufficiency syndrome. Since 1991, vertical expandable prosthetic titanium rib Dynamic PosteroLateral Expansion Thoracoplasty was used at our institution for treatment of JS. This study assesses the safety and efficacy of this procedure. Read More

Crit Care Med 2015 Apr;43(4):808-15

1Ross Tilley Burn Centre, Sunnybrook Health Sciences Centre, Division of Plastic Surgery, Department of Surgery, University of Toronto, Toronto, ON, Canada. 2Programme in Trauma, Emergency, and Critical Care, Sunnybrook Health Sciences Centre, Toronto, ON, Canada. 3Department of Critical Care Medicine, Sunnybrook Health Sciences Centre, Toronto, ON, Canada. 4Shriners Hospitals for Children, Galveston, TX. 5Department of Surgery, University of Texas Medical Branch, Galveston, TX. 6Sealy Center for Molecular Medicine and the Institute for Translational Science, University of Texas Medical Branch, Galveston, TX. 7Department of Surgery, Loyola University Stritch School of Medicine, Maywood, IL. 8Department of Surgery, University of Washington School of Medicine, Harborview Medical Center, Seattle, WA. 9Department of Surgery, University of Texas Southwestern Medical School, Dallas, TX. 10Department of Surgery, Massachusetts General Hospital, Shriners Hospital for Children, and Harvard Medical School, Boston, MA.

Objective: Characterizing burn sizes that are associated with an increased risk of mortality and morbidity is critical because it would allow identifying patients who might derive the greatest benefit from individualized, experimental, or innovative therapies. Although scores have been established to predict mortality, few data addressing other outcomes exist. The objective of this study was to determine burn sizes that are associated with increased mortality and morbidity after burn. Read More