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Developmental dysplasia

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https://read.qxmd.com/read/30865145/relationship-between-torticollis-and-gastroesophageal-reflux-disorder-in-infants
#1
Deborah Bercik, Susan Diemer, Stephanie Westrick, Sarah Worley, Ryan Suder
PURPOSE: Although pediatric physical therapists may evaluate and treat infants with both congenital muscular torticollis (CMT) and gastroesophageal reflux disorder (GERD), a literature review found only 1 article suggesting a connection between these diagnoses. This study investigates a correlation between CMT and GERD. METHODS: A retrospective chart review spanning 5 years including 2519 infants younger than 12 months examined the correlation between CMT, GERD, and other comorbidities including developmental dysplasia of the hip, oligohydramnios, multiparity, and breech positioning...
March 8, 2019: Pediatric Physical Therapy
https://read.qxmd.com/read/30863765/application-of-computer-assisted-navigation-in-treating-congenital-maxillomandibular-syngnathia-a-case-report
#2
Li-Qin Lin, Shan-Shan Bai, Min Wei
BACKGROUND: Congenital maxillomandibular syngnathia is an extremely rare disorder characterized by craniofacial malformations and inability to open the mouth adequately, which leads to problems with feeding, swallowing, and breathing as well as temporomandibular joint ankylosis. The main goal of the surgery is to release the ankylosis, establish functioning mandible, and prevent re-fusion. However, surgical procedures for this disease are rarely reported. CASE SUMMARY: Here, we report a 7-mo-old girl with bilateral maxillomandibular syngnathia...
March 6, 2019: World Journal of Clinical Cases
https://read.qxmd.com/read/30857951/high-hip-center-reduces-the-dynamic-hip-range-of-motion-and-increases-the-hip-load-a-gait-analysis-study-in-hip-arthroplasty-patients-with-unilateral-developmental-dysplasia
#3
Bedri Karaismailoglu, Fahri Erdogan, Gokhan Kaynak
BACKGROUND: Long-term favorable clinical outcomes of anatomical or high hip center techniques in total hip arthroplasty (THA) are reported in patients with developmental dysplasia of the hip (DDH). However, there is little information about the effect of the hip center location on gait characteristics. The purpose of this study was to compare these surgical techniques with gait analysis, analyze the effect of the hip rotation center location on gait parameters, and discuss the possible problems that may arise...
February 18, 2019: Journal of Arthroplasty
https://read.qxmd.com/read/30856249/complex-patterns-of-gabaergic-neuronal-deficiency-and-type-2-potassium-chloride-cotransporter-immaturity-in-human-focal-cortical-dysplasia
#4
Pengcheng Han, Cynthia T Welsh, Michael T Smith, Robert E Schmidt, Steven L Carroll
Focal cortical dysplasia (FCD) is a common histopathologic finding in cortical specimens resected for refractory epilepsy. GABAergic neuronal abnormalities and K-Cl cotransporter type 2 (KCC2) immaturity may be contributing factors for FCD-related epilepsy. We examined surgical specimens from 12 cases diagnosed with FCD, and brain tissues without developmental abnormality obtained from 6 autopsy cases. We found that GABAergic neuronal density was abnormal in FCD with 2 distinct patterns. In 7 of 12 (58%) FCD subjects, the GABAergic neuron density in dysplastic regions and in neighboring nondysplastic regions was equally reduced, hence we call this a "broad pattern...
March 11, 2019: Journal of Neuropathology and Experimental Neurology
https://read.qxmd.com/read/30844524/cyst-peritoneal-shunt-for-the-treatment-of-a-progressive-intracerebral-cyst-associated-with-asns-mutation-case-report-and-literature-review
#5
Bo Chen, Wenchen Li, Xiaodan Wang, Kexin Chen, Xinyu Hong
BACKGROUND: Congenital microcephaly could result from a gene mutation. Asparagine synthetase deficiency, which is caused by ASNS mutation, is a rare autosomal recessive neurometabolic disorder. It is characterized by severe developmental delay, congenital microcephaly, and seizures. CASE DESCRIPTION: Here we present the first report on a progressive intracerebral cyst associated with ASNS mutation, which caused neurodevelopmental dysplasia. ASNS mutation was confirmed by whole exome sequencing and is the most likely reason for the neurodevelopmental dysplasia, which results in microcephaly, refractory seizures, congenital visual impairment...
March 4, 2019: World Neurosurgery
https://read.qxmd.com/read/30840573/morphological-abnormalities-of-the-femur-in-the-dysplastic-hip-relation-between-femur-en-acetabulum
#6
Peter Mahieu, Takehito Hananouchi, Nobuyuki Watanabe, Peter Claes, Hao Li, Emmanuel Audenaert
To apply cutting edge geometry processing techniques and statistical shape modelling to perform a quali-tative and quantitive evaluation of femoral deformity in developmental hip dysplasia and to describe its relation to the amount of acetabular coverage in full 3D. An observational case-control study consisting of 40 right dysplastic cases compared to 43 normal hips, was designed. All subjects were Asian females with an average age of 53.9 years. The right femurs were scanned using computed tomography, followed by 3D reconstruction for statistical shape modelling...
September 2018: Acta Orthopaedica Belgica
https://read.qxmd.com/read/30839476/intermediate-to-long-term-results-of-femoral-neck-lengthening-morscher-osteotomy
#7
Mark Eidelman, Doron Keshet, Scott Nelson, Noam Bor
BACKGROUND: Femoral neck lengthening and transfer of the greater trochanter were introduced by Morscher as treatment for patients with coxa breva and overgrowth of the greater trochanter. In this study we evaluated intermediate and long-term results of this operation. METHODS: We reviewed clinical and radiographic results of 18 patients (20 hips) who were treated by Morscher osteotomy. Ten patients had Perthes disease, 4 had developmental dysplasia of the hip, and 4 had avascular necrosis of the hip...
April 2019: Journal of Pediatric Orthopedics
https://read.qxmd.com/read/30839361/oligohydramnios-should-it-be-considered-a-risk-factor-for-developmental-dysplasia-of-the-hip
#8
Dimitrios Manoukian, Andreas Rehm
Breech, family history, first born and female sex are the main risk factors described for developmental dysplasia of the hip (DDH). Foot abnormalities and oligohydramnios have also been listed. Recent studies have discredited torticollis, multiple gestation pregnancy, mode of delivery and prematurity as risk factors. Definition of oligohydramnios in the literature is inconsistent. Our aim was to investigate the term oligohydramnios and evaluate whether it should be considered a risk factor for DDH. All live births in our institution between 2001 and 2014 were included...
March 4, 2019: Journal of Pediatric Orthopedics. Part B
https://read.qxmd.com/read/30839114/gender-and-disease-severity-determine-proximal-femoral-morphology-in-developmental-dysplasia-of-the-hip
#9
Oliver R Boughton, Keisuke Uemura, Kazunori Tamura, Masaki Takao, Hidetoshi Hamada, Justin P Cobb, Nobuhiko Sugano
In this computed tomography (CT) morphological study we describe the way the proximal femoral morphology differs with worsening degrees of developmental dysplasia of the hip (DDH) and describe gender differences in patients with DDH. 49 male patients with DDH were matched with 49 females with DDH, using age and the Crowe classification of DDH severity. The femoral length, anteversion, neck-shaft angle, offset, neck length, canal-calcar ratio, canal flare index, lateral center-edge angle, alpha angle, pelvic tilt and pelvic incidence were measured for each patient on their pre-operative CT scans, prior to total hip arthroplasty surgery...
March 6, 2019: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://read.qxmd.com/read/30838928/the-space-time-continuum-of-cortical-dysplasia
#10
Laura A Jansen
Somatic Mutations Activating the mTOR Pathway in Dorsal Telencephalic Progenitors Cause a Continuum of Cortical Dysplasias D'Gama AM, Woodworth MB, Hossain AA, Bizzotto S, Hatem NE, LaCoursiere CM, Najm I, Ying Z, Yang E, Barkovich AJ, Kwiatkowski DJ, Vinters HV, Madsen JR, Mathern GW, Blümcke I, Poduri A, Walsh CA. Cell Rep. 2017;21:3754-3766. Focal cortical dysplasia (FCD) and hemimegalencephaly (HME) are epileptogenic neurodevelopmental malformations caused by mutations in mTOR pathway genes. Deep sequencing of these genes in FCD/HME brain tissue identified an etiology in 27 (41%) of 66 cases...
January 2019: Epilepsy Currents
https://read.qxmd.com/read/30821590/corticosterone-rather-than-ethanol-epigenetic-programmed-testicular-dysplasia-caused-by-prenatal-ethanol-exposure-in-male-offspring-rats
#11
Min Liu, Qi Zhang, Linguo Pei, Yunfei Zou, Guanghui Chen, Hui Wang
Prenatal ethanol exposure (PEE) could affect offspring's testicular development. This study aimed to illuminate its intrauterine origin and the programming mechanism caused by PEE. Pregnant Wistar rats were given ethanol (4 g/kg.d) by gavage administration during gestational days (GD) 9-20. Serum samples and testes of male offspring rats were collected on GD20, postnatal week (PW) 6, and PW12. We found that PEE induced testicular morphological abnormality, low serum testosterone levels, expressive suppression of 3β-hydroxysteroid dehydrogenase (3β-HSD), and low acetylation levels of histone 3 lysine 14 (H3K14ac) of 3β-HSD before and after birth...
March 1, 2019: Epigenetics: Official Journal of the DNA Methylation Society
https://read.qxmd.com/read/30820422/bilateral-nonsyndromic-dentigerous-cyst-in-a-10-year-old-child-a-case-report-and-literature-review
#12
Bhawna Pant, Karla Carvalho, Anita Dhupar, Anita Spadigam
Dentigerous cysts are the most common developmental cysts of the jaws, accounting for approximately 20% of all jaw cysts. These cysts are usually unilateral, surrounding the crowns of impacted mandibular third molars. Bilateral dentigerous cysts have been associated with syndromes such as cleidocranial dysplasia and Maroteaux-Lamy syndrome. Nonsyndromic bilateral dentigerous cysts are extremely rare, particularly in the mixed dentition. Seventeen such cases have been reported till date. This article reports the eighteenth case of bilateral dentigerous cysts involving permanent maxillary canines in a 10-year-old boy...
January 2019: International Journal of Applied and Basic Medical Research
https://read.qxmd.com/read/30813797/what-is-the-incidence-of-late-detection-of-developmental-dysplasia-of-the-hip-in-england
#13
C Broadhurst, A M L Rhodes, P Harper, D C Perry, N M P Clarke, A Aarvold
AIMS: The aim of this study was to establish the incidence of developmental dysplasia of the hip (DDH) diagnosed after one-year of age in England, stratified by age, gender, year, and region of diagnosis. PATIENTS AND METHODS: A descriptive observational study was performed by linking primary and secondary care information from two independent national databases of routinely collected data: the United Kingdom Clinical Practice Research Datalink and Hospital Episode Statistics...
March 2019: Bone & Joint Journal
https://read.qxmd.com/read/30813782/femoral-morphology-asymmetry-in-hip-dysplasia-makes-radiological-leg-length-measurement-inaccurate
#14
K Tamura, M Takao, H Hamada, W Ando, T Sakai, N Sugano
AIMS: The aim of this study was to examine whether hips with unilateral osteoarthritis (OA) secondary to developmental dysplasia of the hip (DDH) have significant asymmetry in femoral length, and to determine potential related factors. PATIENTS AND METHODS: We enrolled 90 patients (82 female, eight male) with DDH showing unilateral OA changes, and 43 healthy volunteers (26 female, 17 male) as controls. The mean age was 61.8 years (39 to 93) for the DDH groups, and 71...
March 2019: Bone & Joint Journal
https://read.qxmd.com/read/30807510/spica-mri-predictors-for-epiphyseal-osteonecrosis-after-closed-reduction-treatment-of-dysplasia-of-the-hip
#15
Lee S Haruno, J Herman Kan, Michael J Rivlin, Scott B Rosenfeld, Erica K Schallert, Huirong Zhu, Vinitha R Shenava
Spica MRI with intravenous gadolinium contrast after closed reduction for developmental dysplasia of the hip (DDH) helps to determine successful reduction and attempts to identify patients at risk for epiphyseal osteonecrosis. The objective of our study was to evaluate spica MRI predictors for epiphyseal osteonecrosis after closed reduction. Retrospective study for all patients undergoing closed reduction for DDH followed by gadolinium-enhanced spica MRI between July 2011 and November 2014. Patient demographics and clinical follow-up through 2017, including the development of epiphyseal osteonecrosis and need for reintervention after the initial reduction, were recorded...
February 22, 2019: Journal of Pediatric Orthopedics. Part B
https://read.qxmd.com/read/30803943/-the-role-of-actin-cytoskeleton-in-regulating-the-deployment-process-of-mouse-cardiac-second-heart-field-progenitor-cells
#16
Zhong Ying Liu, Xia Huang, Zi Yi Li, Zi Hao Yang, Bai Yin Yuan
The vertebrate heart tube originates from cardiogenic mesodermal cells in the early embryo, and subsequently elongates by progressive addition of second heart field (SHF) progenitor cells from adjacent pharyngeal mesoderm and splanchnic mesoderm. Insufficient addition of SHF cells to the heart tube causes the failure of maximal elongation of the heart tube, which results in a series of developmental defects including the most common congenital birth defects, such as right ventricular dysplasia and outflow tract septation, and alignment anomalies...
February 20, 2019: Yi Chuan, Hereditas
https://read.qxmd.com/read/30777621/results-of-200-consecutive-ceramic-on-ceramic-cementless-hip-arthroplasties-in-patients-up-to-50-years-of-age-a-5-24-years-of-follow-up-study
#17
Giuseppe Solarino, Luigi Zagra, Andrea Piazzolla, Arcangelo Morizio, Giovanni Vicenti, Biagio Moretti
BACKGROUND: Ceramic-on-ceramic (CoC) bearings due to their mechanical properties can be used in primary total hip arthroplasty (THA) especially in young patients requiring a long-lasting implant. The goal of this study is to analyze the results of a series of 200 consecutive CoC THAs in patients aged 50 years or less at the time of surgery. METHODS: A retrospective study was conducted on the first 200 consecutive CoC arthroplasties performed using the direct lateral approach on 105 females and 81 males (14 bilateral cases) with an average age of 44...
January 31, 2019: Journal of Arthroplasty
https://read.qxmd.com/read/30765927/late-acetabular-dysplasia-after-successful-treatment-for-developmental-dysplasia-of-the-hip-using-the-pavlik-method-a-systematic-literature-review
#18
REVIEW
K Aaron Shaw, Colleen M Moreland, Dana Olszewski, Tim Schrader
The Pavlik method is the most common method used for treatment of developmental dysplasia of the hip (DDH). Late acetabular dysplasia despite successful treatment, however, has had varied reporting. A systematic review was performed, investigating the long-term outcomes of DDH treated with the Pavlik method. Seventeen studies met inclusionary criteria, including 6029 hips treated with an average of 5.29 years follow-up. Radiographic evidence of late dysplasia was present in 280 hips, with 109 hips requiring additional surgery...
January 2019: Journal of Orthopaedics
https://read.qxmd.com/read/30762594/hip-arthrography-complicated-by-air-embolism-in-infants-a-report-of-2-cases
#19
Courtney Bell, Nattaly Greene, Meredith Kato, Rachel Goldstein, Scott Yang
CASE: We report 2 cases of infants with developmental dysplasia of the hip who underwent arthrography of the hip with use of air for structure identification, which resulted in a presumed air embolism and deep oxygen desaturation. This led to the hypothesis that there is an increased potential of air embolism in the pediatric population given the vascular anatomy of the hip. CONCLUSION: These 2 cases document the important and not well-known complication of air embolism during air arthrography, which resulted in a change of practice for the authors...
February 13, 2019: JBJS Case Connector
https://read.qxmd.com/read/30761426/cementless-hip-arthroplasty-and-transverse-shortening-femoral-osteotomy-with-the-s-rom-stem-for-crowe-type-iv-developmental-dysplasia
#20
Libor Necas, Maros Hrubina, Marian Melisik, Zoltan Cibula, Michal Chmurny, Matej Daniel, Boris Steno
INTRODUCTION: The aim of this study has been to present outcomes after cementless arthroplasty for developmental dysplasia Crowe type IV of the hip, with transverse subtrochanteric shortening osteotomy and using the S-ROM stem. METHODS: We evaluated radiographs, functional scores and complications in a consecutive series of 23 patients (28 hips) with high dislocation of the hip. The average age of patients at surgery was 49.9 (range 22-68) years. The operations were performed between 2007 and 2013...
February 13, 2019: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
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