Rotational Shift of Proximal Carpal Row and it's Correlation with Global Wrist Joint Laxity

Introduction: As the wrist moves to ulnar deviation, theproximal carpal row undergoes radial translation, dorsalrotation and supination. Similarly, the radial deviationinvolves ulnar translation of the proximal carpal row, its volarrotation and pronation. These combined movements of theproximal carpal row are called rotational shift of the carpus.Study aimed to quantify the rotational shift of the proximalcarpal row during ulnar or radial deviation of the wrist.Material and methods: The proposed study was a prospectivestudy conducted in the Department of Orthopedics, RMCH,Bareilly, UP comprising 100 healthy volunteers with equal sexration who never had any symptoms pertaining to their wristjoint. PA and lateral radiograph were obtained in full radialand ulnar deviation. A custom-made positioning device wasused to ensure proper placement of the hand and wrist duringthe examination.Results: In the present study, we observed that theradioscaphoid angle in radiation deviation varied from 50to 85 degree with mean of 68.96 degree. Radioulnate anglein radial deviation varied from -2 to 45 degree with mean of17.79 degree. Radioscaphoid angle in ulnar deviation variedfrom 10 to 70 degree with mean of 39.97 degree. Radioulnateangle in ulnar deviation varied from -3 to -38 degree withmean of -17.15 degree.Conclusion: Within the limitations of the present study, itcan be concluded that the ulnar deviation of the wrist is seento cause radial translation ad dorsal rotation of the proximalcarpal row. Similarly, the radial deviation was seen to causeulnar translation and volar rotation of the proximal carpal row.

Rare Cases of PLOD1-Related Kyphoscoliotic Ehlers-Danlos Syndrome in a Korean Family Identified by Next Generation Sequencing

Kyphoscoliotic Ehlers-Danlos syndrome (kEDS) is an autosomal recessive connective tissue disorder characterized by muscular hypotonia, hyperextensible skin, skin fragility, joint hypermobility, and progressive kyphoscoliosis. The disorder results from a deficiency of the enzyme collagen lysyl hydroxylase 1 due to mutations in the gene PLOD1. We describe the rare cases of kEDS in Korean siblings with two novel compound heterozygous variants, c.926_934del (p.Leu309_Leu311del) and c.2170_2172del (p.Phe724del) in the PLOD1 gene. They had congenital hypotonia, joint laxity, skin hyperextensibility, Marfanoid habitus, high myopia and atrophic scarring. The younger sibling had an early-onset progressive kyphoscoliosis, while the older sibling showed mild scoliosis during childhood. Intrafamilial variability of the clinical severity and age of kyphoscoliosis onset observed in our cases.

Short-term effectiveness of arthroscopically capsular vertical mattress suturing for shoulder recurrent anterior dislocation combined with joint laxity / 中国修复重建外科杂志

Objective: To evaluate the short-term effectiveness of arthroscopically capsular vertical mattress suturing for shoulder recurrent anterior dislocation combined with joint laxity. Methods: A retrospective analysis was performed on 6 shoulder recurrent anterior dislocation patients combined with joint laxity treated with arthroscopically capsular vertical mattress suturing between January 2017 and December 2018. There were 5 males and 1 female with an average age of 20.8 years (range, 19-24 years). The number of shoulder dislocation was 3-18 times, with an average of 9.5 times. The disease duration ranged from 2 to 60 months, with an average of 25.3 months. The preoperative Beighton score was 4-7, with an average of 5.8; the Instability Severity Index Score (ISIS) was 2-5, with an average of 3.5. There were 5 cases of simple Bankart injury and 1 case of bony Bankart injury. The range of motion of shoulder joint (including active flexion and lifting, external rotation, abduction and external rotation, and internal ratation) was recorded before operation and at last follow-up; Oxford shoulder instability score, Rowe shoulder instability score, and Simple Shoulder Test (SST) score were used to evaluate shoulder joint function before operation, at 6 months after operation, and at last follow-up, and complications were recorded. Results: All patients were followed up 16-28 months (mean, 19.3 months). During the follow-up, all patients had satisfactory motor function, and no re-dislocation and postoperative neurovascular complications occurred. At last follow-up, the activities of active external rotation and abduction and external rotation were significantly improved when compared with those before operation ( P0.05). The Oxford shoulder instability score, Rowe shoulder instability score, and SST score at 6 months after operation and at last follow-up were significantly improved when compared with those before operation ( P0.05). Conclusion: The treatment of shoulder recurrent anterior dislocation combined with joint laxity by arthroscopically vertical matress suturing can achieve good short-term effectiveness.

Arterial Tortuosity Syndrome in a Neonate

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.

Síndrome de Morquio, enfermedad de interés para la odontopediatría: presentación de un caso / Morquio syndrome, a disease of interest for pediatric dentistry: case report

El Síndrome de Morquio es un trastorno de almacenamiento de mucopolisacáridos se caracteriza principalmente por estatura corta y afectación ósea grave, pero el coeficiente intelectual es normal. La prevalencia es rara se estima que afecta a uno de cada 200.000 nacimientos hombres y mujeres por igual. La MPS IV A y B son enfermedades autosómicas recesivas con esto queremos decir que ambos progenitores son portadores del mismo gen afectado el cual se encuentra alterado produciendo así una deficiencia en la producción de la enzima. Las manifestaciones esqueléticas en esta displasia son retardo en el crecimiento, hipoplasia del odontoides, cifosis toracolumbar, displasia de cadera, genu valgo, manchas cutáneas y laxitud articular, en cuando a cuestiones dentales tenemos: el esmalte es delgado, rugoso e hipoplásico afectando dientes deciduos como permanentes. Se presenta el caso de un paciente masculino de 8 años 3/12 presentando MPS el cual requiere un protocolo de rehabilitación lo cual se realiza en el área de odontopedriatría del Hospital del Niño DIF.

Morquio syndrome is a mucopolysaccharide storage disorder is mainly characterized by short stature, severe bone involvement, but IQ is normal. The prevalence is rare is estimated to affect one in every 200,000 births men and women alike. The MPS IV A and B are autosomal recessive diseases with this we mean that both parents are carriers of the same gene affected which is altered thus producing a deficiency in the production of the enzyme. The skeletal manifestations in this dysplasia are growth retardation, hypoplasia of the odontoid, thoracolumbar kyphosis, hip dysplasia, genu valgus, skin blemishes and joint laxity, then dental issues are: the enamel is thin rugged and hypoplastic affecting deciduous theeth as permanent. The case of a male patient presenting eight years 3/12 MPS which requires a rehabilitation protocol which is done in the dental area of Hospital del Niño DIF is presented.

Relación entre hiperlaxitud articular, dismetría de miembros inferiores y control postural con los trastornos posturales / Relationship between joint hypermobility, lower limb asymmetry and postural control with postural abnormalities

Objetivos: Determinar si existe relación entre hiperlaxitud articular, dismetría de miembros inferiores y la estabilidad ocontrol postural en bipedestación con los trastornos posturales en adolescentes. Material y métodos: Estudio relacional transversal,realizado en el Instituto Nacional de Rehabilitación “Dra. Adriana Rebaza Flores” Amistad Perú-Japón,Chorrillos-Perú. Participaron todos los estudiantes del 5º año de secundaria de un colegio privado de la ciudad deLima. La hiperlaxitud articular (HA) se evaluó con el score de Beighton; la dismetría de miembros inferiores (DMI),con medición en ortoradiografía; el control postural (CP) con posturografía estática usando una plataforma ISTFOOTWORK; el apoyo plantar (pie plano y pie cavo) con baro-podometría; la escoliosis e hiperlordosis lumbar conmedición radiográfica del ángulo de COBB y de lordosis...

Objectives: To determine an association between joint hypermobility, lower limb asymmetry and postural control withpostural abnormalities in adolescents. Methods: Cross-sectional study conducted at the National Rehabilitation Institute“Dra. Adriana Rebaza Flores” Amistad Perú-Japón, Chorrillos-Perú. All students of the 5th year of a private secondaryschool in Lima participated in the study. Joint hypermobility (JH) was assessed with the Beighton´score; lower limbasymmetry (LLA) was evaluated with X-ray; postural control (PC) was evaluated with static posturography using ISTFOOTWORK platform; plantar surface (flat foot and cavus foot) was evaluated with baro-podometry; scoliosis andlumbar hyperlordosis were measured with X-ray measuring the COBB angle...

Cutaneous asthenia (Ehlers-Danlos syndrome) in a Korean short-haired cat

A 1-year-old Korean domestic short-haired cat presented with skin hyperextensibility and a severely macerated wound on the skin of the dorsal part of the neck. Diagnostic studies including histopathology and skin extensibility index revealed congenital cutaneous asthenia (Ehlers-Danlos syndrome). In this cat, the skin wounds and defects were successfully managed with standard wound management and cosmetic surgery. Although skin hyperextensibility is persistent, the cat has lived well without other complications to date. To the best of our knowledge, this is the first report of cutaneous asthenia in a cat in Korea.

Distribution and expression of PDGF-A in the ligamentum teres of children with developmental dislocation of the hip / 中国矫形外科杂志

[Objective] This study examined the distribution and the expression of platelet-derived growth factor-A (PDGF-A) in the ligamentum teres of children with developmental dislocation of the hip (DDH) and normal persons in order to investigate the roles of PDGF-A in hip joint laxity. [Methods]There were six pairs of joint laxity of children with DDH and normal children (control group) matched to gender and age. Immunohistochemistry (SP method) method was used to detect the location and distribution of the PDGF-A in the ligamentum teres,and to semi-quantify their content.Semiquantitative RT-PCR method was used to detect the PDGF-A in the ligamentum teres at mRNA level. The quantitative analysis of the PDGF-A was performed by professional image software and the results were analyzed with standard statistical methods.[Results]High density expression was observed in the synovial layer with fibroblast regularly arranged parallel to the joint surface in the ligamentum teres. PDGF-A distribution was decreased in the fibrous layer of the ligamentum teres. There were significant differences in the percentage of positive fibroblast and gray-scale density in the fibrous layer between DDH group and control group (P

THE EFFECT OF DIRECT KICKING FORCE ON ANTERIOR TIBIAL TRANSLATION FROM SOCCER BALL IN HEALTHY KNEES / 体力科学

Anterior cruciate ligament (ACL) is the first restraint against anterior tibial translation (ATT) in the knee joint. Previous studies suggested that knee joint laxity might be increased by the repeated stress imposed on the knee joint. The purpose of this study was to demonstrate the effect of the repeated soccer ball kicking actions on the ATT in healthy knees. Forty female collegiate students volunteered for this study. KT-2000 knee arthrometer was used to measure ATT before and after the task. The findings demonstrated that the repeated kicking actions had no significant effect on the ATT in healthy knees. The maximum knee extension angle on the striking side was greater than the supporting side (p<0.05). Significant correlations between mean anterior translation and maximum knee extension angle were observed on both sides (p<0.05). The kicking force on a soccer ball resulted in no increase in the knee laxity.

Expression of COLlal and COL3al in the ligamentum teres of developmental dislocation of the hip / 中国矫形外科杂志

[Objective]To investigate the expression of collagen type I and III at mRNA and protein level in the ligamentum teres of developmental dislocation of the hip(DDH) and the normal hip. [Method]There were 6 pairs joint laxity of patients of DDH group and normal control group with paired control of same sex and age.Semiquantity RT-PCR method was used to detect the COLlal and COL3al in the ligamentum teres at mRNA level. Western-Blot method was used to detect the COLlal and COL3al in the ligamentum teres at protein level. The quantitative analysis of the COLlal and COL3al were performed by professional image software and the results were analyzed with standard statistical methods.[Result]At both mRNA and protein level COLlal expression were decreased in the DDH group compared to the control group(P0.05).[Conclusion]The decreased collagen I expression at mRNA and protein level in the ligamentum teres of the children with DDH may lead to hip joint laxity.Hip joint laxity in DDH may be independent to the content of collagen III.

RELATIONSHIP BETWEEN MAJOR JOINT INJURY AND GENERAL JOINT LAXITY IN COLLEGIATE AMERICAN FOOTBALL PLAYERS / 体力科学

A preparticipation examination for athletes should be performed to prevent severe athletic injury by examining physical characteristics related to various injuries. The general joint laxity test has been included among the check-up items considering as relating factor of joint injury. Authors investigated the relationship between the score of general joint laxity for freshmen examination and the incidence of major joint injury, during the succeeding 5 athletic seasons, in eighty-seven collegiate American football players.<BR>Significant higher scores were observed in athletes affected by shoulder injury, and slightly higher scores in those affected by knee joint injury. But, the scores were similar between athletes with and without elbow or ankle injury.<BR>The degree of laxity was associated with shoulder and knee joint injury in a comparison of incidence of these injuries among tight, medium and lax groups.<BR>Authors concluded that general joint laxity is related to the incidence of shoulder and knee joint injury.

Injuries of water polo players - In special regard to the knee joint / 体力科学

102 water polo players were interviewd about the injuries caused by water polo. 13% of the subjects had knee pain at least once a year. 73% of which were chronic cases. For 24 players, clinical examinations, including X-ray examination, A-P knee laxity measurement, isokinetic strength measurement of knee muscles, and photo analysis of the vertical egg beater kick motion were performed to elucidate the cause of knee pain.<BR>The summary of the results were as follows;<BR>1. The most common site of knee pain was the medial portion of the knee joint.<BR>2. Protrusion of the medial femoral epicondyle and sharpning of the tibial intercondylar eminentia as well as spur formation of the superior pole of the patella were observed in most subjects.<BR>3. Injured subjects had stiff knees and weak knee extensor muscles.<BR>4. The vertical egg beater kick motion must be the main factor of the painful knee.

INJURIES OF WATER POLO PLAYERS / 体力科学

102 water polo players were interviewd about the injuries caused by water polo. 13% of the subjects had knee pain at least once a year. 73% of which were chronic cases. For 24 players, clinical examinations, including X-ray examination, A-P knee laxity measurement, isokinetic strength measurement of knee muscles, and photo analysis of the vertical egg beater kick motion were performed to elucidate the cause of knee pain.<BR>The summary of the results were as follows;<BR>1. The most common site of knee pain was the medial portion of the knee joint.<BR>2. Protrusion of the medial femoral epicondyle and sharpning of the tibial intercondylar eminentia as well as spur formation of the superior pole of the patella were observed in most subjects.<BR>3. Injured subjects had stiff knees and weak knee extensor muscles.<BR>4. The vertical egg beater kick motion must be the main factor of the painful knee.