Fabry''s disease

Fifteen hemizygotes and 30 heterozygotes have been diagnosed since our investigations of Fabry's disease were started 10 years ago. They belong mainly to three Danish families. Genetic counseling and prenatal diagnoses have been performed, and in vitro studies of cultured fibroblasts and endothelial cells have been made with special reference to enzyme therapy.     

Microsurgical anatomy of VII and VIII cranial nerves and related arteries in the cerebellopontine angle

Summary The relationships of VII and VIII cranial nerves and related arteries are reviewed in 26 preparations by microdissection techniques. These vessels may be grouped in large (AICA, PICA), medium (LA, SA, CSA, RPI) and small calibre (vasa nervorum, radicullar and medullar branches). The importance of these structures in acoustic neuroma surgery, vestibular neurectomy and cross-compression syndromes is discussed. Vascular loops and elongated arteries are normal structures present at birth.This work was supported by a grant from the AJ Roemmers Foundation     

序列解剖层片的全局和局部色彩校正

本研究提出了一种全局和局部相结合的序列解剖层片的色彩校正算法。利用数字人层片（中国女婴一号）上提供的彩色灰度卡对相邻层片间各颜色通道的色彩差异进行全局校正，以消除解剖数据集沿垂直方向的光照变化；再利用线性校正模型和差值图像各通道（R、G、B）的灰度局部直方图，对解剖数据集进行局部色彩差异校正。重建结果表明，经过全局和局部色彩校正后，重建视图中的明暗条纹基本消失，说明层片间颜色的不连续现象得到了校正。     

Study of the carrying angle of the human elbow joint in full extension: a morphometric analysis

This study measured the carrying angle of the elbow joint in full extension in 600 students, using the supplementary angle to that between the longitudinal axis of the arm and that of the forearm. The mean carrying angle was 12.88°±5.92: 10.97°±4.27 in men and 15.07°±4.95 in women. The carrying angle changes with skeletal growth and maturity. The angle is always greater on the side of the dominant hand. We confirmed the inverse relationship between the carrying angle and the intertrochanteric diameter. Also, the type of constitution influences the value of the carrying angle, especially in women.     

Length and moment arm of human leg muscles as a function of knee and hip-joint angles

Summary Lengths of muscle tendon complexes of the quadriceps femoris muscle and some of its heads, biceps femoris and gastrocnemius muscles, were measured for six limbs of human cadavers as a function of knee and hip-joint angles. Length-angle curves were fitted using second degree polynomials. Using these polynomials the relationships between knee and hip-joint angles and moment arms were calculated. The effect of changing the hip angle on the biceps femoris muscle length is much larger than that of changing the knee angle. For the rectus femoris muscle the reverse was found. The moment arm of the biceps femoris muscle was found to remain constant throughout the whole range of knee flexion as was the case for the medial part of the vastus medialis muscle. Changes in the length of the lateral part of the vastus medialis muscle as well as the medial part of the vastus lateralis muscle are very similar to those of vastus intermedius muscle to which they are adjacent, while those changes in the length of the medial part of the vastus medialis muscle and the lateral part of the vastus lateralis muscle, which are similar to each other, differ substantially from those of the vastus intermedius muscle. Application of the results to jumping showed that bi-articular rectus femoris and biceps femoris muscles, which are antagonists, both contract eccentrically early in the push off phase and concentrically in last part of this phase.     

Approximately 80% of Japanese osteoarthritic patients fall out of the safety range in restricted kinematically-aligned total knee arthroplasty in an analysis of preoperative long-leg radiograms

BackgroundRestricted kinematically-aligned total knee arthroplasty (KA-TKA) is a reasonable modification to avoid the alignment outlier that may cause implant failure. However, despite a noted high incidence of constitutional varus in Japanese individuals, there has been no investigation into how many knees require the restriction in restricted KA-TKA (RKA-TKA) among Japanese patients. Therefore, we conducted a study using preoperative long-leg radiograms.MethodsWe studied long-leg radiographs of 228 knees in 114 consecutive patients. The numbers of knees within the safety range and their corrective osteotomy angle in the restriction algorithms advocated by Almaawi et al. (2017) and MacDessi et al. (2020) were evaluated.ResultsAccording to the algorithms used by Almaawi et al. and MacDessi et al., out of 228 knees, 46 (20%) and 39 (17%) fell within the safety range, respectively. The mean correction angles of the hip-knee-ankle angle, lateral distal femoral angle and medial proximal tibial angle were 2.8 ± 3.4°, 0.4 ± 1.4° and 2.4 ± 2.8° in the algorithm used by Almaawi et al., while they were −4.9 ± 4.7°, 1.1 ± 2.5° and −6.0 ± 3.4° in the algorithm used by MacDessi et al. Most of the knees needed to be restricted in order to perform RKA-TKA, regardless of the algorithm used.ConclusionsBased on a preoperative analysis of long-leg radiograms in a Japanese population, most knees fall out of the safety range in RKA-TKA. Surgeons must consider whether to allow component outlier or to perform corrective osteotomy that likely requires soft tissue release.     

老年转子间骨折角型钢板治疗失误和并发症分析

目的分析角型钢板治疗股骨转子间骨折中出现的各种失误和并发症,并提出预防措施。方法1990年10月以来共治疗股骨转子间骨折68例,逐例分析治疗失误的原因。结果术中操作失误致股骨外侧皮质崩裂2例;钢板刀口穿破股骨头进入髋臼1例,术后切口感染1例,共8例出现并发症,发生率14．7％。结论角型钢板治疗股骨转子间骨折效果好,但不应忽视可能发生的各种失误和并发症。     

三维节段脊柱内固定系统在脊柱矫形术中的应用

目的评价三维节段脊柱内固定系统在不同脊柱畸形中的应用效果.方法应用三维节段脊柱内固定系统CD、TSRH等治疗脊柱畸形26例,其中特发性侧凸19例,先天性侧凸2例,后凸畸形5例,均以后路矫形自体骨植骨融合.结果所有病例经6～27个月随访,平均10.4个月.侧凸冠状面畸形平均矫正率为61.4%;矢状面后凸术后平均32.5°;旋转畸形平均矫正Ⅰ度,顶椎最大偏距平均矫正率为65.1%;后凸畸形平均矫正率为59.2%.所有病例无脱钩、断棍、感染、骨折以及脊髓神经损伤.结论三维节段脊柱内固定系统不仅可以完成畸形的三维矫正,而且矫正可靠,稳定性高,术后可不用石膏固定.     

Alignment in Medial Fixed-Bearing Unicompartmental Knee Arthroplasty: The Limb Has a Leg Up on the Component

BackgroundTo assess how implant alignment affects unicompartmental knee arthroplasty (UKA) outcome, we compared tibial component alignment of well-functioning UKAs against 2 groups of failed UKAs, revised for progression of lateral compartment arthritis (“Progression”) and aseptic loosening (“Loosening”).MethodsWe identified 37 revisions for Progression and 61 revisions for Loosening from our prospective institutional database of 3351 medial fixed-bearing UKAs performed since 2000. Revision cohorts were matched on age, gender, body mass index, and postoperative range of motion with “Successful” unrevised UKAs with minimum 10-year follow-up and Knee Society Score ≥70. Tibial component coronal (TCA) and sagittal (TSA) plane alignment was measured on postoperative radiographs. Limb alignment was quantified by hip-knee-ankle (HKA) angle on long-leg radiographs. In addition to directly comparing groups, a multivariate logistic regression examined how limb and component alignments were associated with UKA revision.ResultsIn the Progression group, component alignment was similar to the matched successes (TCA 3.6° ± 3.5° varus vs 5.1° ± 3.5° varus, P = .07; TSA 8.4° ± 4.4° vs 8.8° ± 3.6°, P = .67), whereas HKA angle was significantly more valgus (0.3° ± 3.6° valgus vs 4.4° ± 2.6° varus, P < .001). Loosening group component alignment was also similar to the matched successes (TCA 6.1° ± 3.7° varus vs 5.9° ± 3.1° varus, P = .72; TSA 8.4° ± 4.6° vs 8.1° ± 3.9°, P = .68), and HKA was significantly more varus (6.1° ± 3.1° varus vs 4.0° ± 2.7° varus, P < .001). Using a multivariate logistic regression, HKA angle was the most significant factor associated with revision (P < .001).ConclusionIn this population of revised UKAs and long-term successes, limb alignment was a more important determinant of outcome than tibial component alignment.Level of EvidenceLevel III case-control study.