Procollagen-Proline Dioxygenase

前胶原 - 脯氨酸双加氧酶
  • 文章类型: Observational Study
    背景:多年来,在乳房中使用网状物的普及程度各不相同。它描述得很好,然而,它在美容乳房手术中的利用率却很低。聚-4-羟基丁二酸(P4HB;GalaFLEX,Galatea,列克星敦,MA)最近被描述为支持不良组织质量的有用辅助手段,具有积极的早期结果和低并发症发生率。
    目的:作者试图确定PH4B在美容乳房手术中的结果和适应症范围,以证明其在乳房固定术中的长期疗效。
    方法:首先,我们进行了一项观察性研究,将术中包括P4HB技术标准化.进行摄影测量以确定术后3个月和1年下极下降和下极拉伸的程度,以观察随时间的变化。其次,探索了其他适应症,包括纠正次生缺陷,如下位错位和症状。
    结果:乳房固定术研究的结果非常令人鼓舞,与以前发表在文献中的结果相当,确认乳房固定术和植入乳房固定术的下极随时间的持续稳定性。此外,GalaFLEX已成功用于纠正许多次要缺陷。
    结论:P4HB用途广泛,易于使用,并发症发生率低。结果导致了实践的变化,所有常规乳腺固定术常规掺入GalaFLEX。它还取代了在美容乳房手术中使用无细胞真皮基质。
    The popularity of utilizing mesh in the breast has varied over the years. It is well described, and yet there has been poor uptake of its utilization in aesthetic breast surgery. Poly-4-huydroxybutarate (P4HB; GalaFLEX, Galatea, Lexington, MA) has recently been described as a useful adjunct in supporting poor tissue quality with positive early outcomes and low complication rates.
    The authors sought to determine the outcome and range of indications of PH4B in aesthetic breast surgery to document its effectiveness in assisting with long-term outcomes in mastopexy.
    Firstly, an observational study was undertaken standardizing mastopexy techniques with P4HB included in the procedure. Photographic measurements were taken to determine the extent of lower pole descent and lower pole stretch at 3 months and 1 year postoperative to observe change over time. Secondly, other indications were explored, including the correction of secondary defects such as inferior malposition and symmastia.
    The results of the mastopexy study were highly encouraging and comparable with those previously published in the literature, confirming sustained stability of the lower pole over time for mastopexies and implant mastopexies. In addition, the GalaFLEX was successfully employed in correcting many secondary defects.
    P4HB is extremely versatile and easy to utilize and has low complication rates. The results have led to a change in practice, with routine incorporation of GalaFLEX for all routine mastopexies. It has also replaced the utilization of acellular dermal matrices in aesthetic breast surgery.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    BACKGROUND: Cole-Carpenter syndrome-1 (CLCRP1) is an independent osteogenesis imperfect (OI)-like disorder that manifests as bone fragility, craniosynostosis, ocular proptosis, hydrocephalus, and distinctive facial features. Only 2 types of mutation sites in the P4HB and CRTAP genes have been reported.
    METHODS: A 14-month-old Chinese girl presented with prominent ocular proptosis, frontal bossing, craniosynostosis, plump anterior fontanel, growth retardation, osteopenia, and distinctive facial features that were strikingly similar to those in the original 2 cases.
    METHODS: Whole-exome sequencing revealed a novel deletion variation in exons 5 to 8 of the P4HB gene, which was found to be heterozygous using fluorogenic quantitative-polymerase chain reaction.
    CONCLUSIONS: This de novo deletion mutation in exons 5 to 8 of the P4HB gene advances our understanding of CLCRP1, expands the mutation spectrum of P4HB, and diversifies the cases reported for this condition.
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