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Diffuse St Depression With St Elevation Avr In Acute Coronary Syndrome And Its Association With Significant Left Main Or Three Vessel Coronary Artery Disease And Its Confounders. 急性冠脉综合征的弥漫性St降伴St升高及其与显著左主干或三支冠状动脉疾病及其混杂因素的关系
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-10590
Salman Ahmed, Shehzad Khatti, Ghazanfar Ali Shah, Naveedullah Khan, Lajpat Rai, Mohammad Zeb Khan, Syed Abdul Bari, Naveen Roy

Background: : Global ST depression in 8 or more leads along with ST elevation in aVR has been considered as hallmark of widespread sub-endocardial ischemia. It has been associated with left main (LM) stem or three vessel disease (3VD). But different studies have shown different results. We collected data from patients to see association of these ECG changes with significant LM stem disease and/or significant (3VD).

Methods: TIt was a prospective observational study performed at tertiary care cardiac center. All patients with acute coronary syndrome (ACS) having global ST depression and ST Elevation in aVR (that is ST depression of at least 0.5 mv in ≥8 leads along with ST elevation in aVR of at least 0.5 mv) and have undergone coronary angiogram were included.

Results: Our study included 404 patients with above mentioned ECG findings. We observed significant LM stem or significant 3VD in 67% (n=274), 3VD in 55% (n=222) and significant LM stem in only 29% (n=118). Risk factors like diabetes, hypertension and smoking increase probability of these ECG changes up to 40.4%, 32.1% and 33.3% for significant LM stem disease and 62.7%, 57.1% and 57.5% for significant 3VD. Magnitude of ST elevation in aVR leads ≥1 mm increase sensitivity for LM stem disease 35% and for 3VD up to 60.4% and TIMI score ≥4 up to 36.7% for significant LM stem disease and 62.5% for significant 3VD.

Conclusions: : Global ST depression along with ST elevation in aVR in patients with ACS has low probability for significant LM stem intermediate probability for significant 3VD. Factors like presence of diabetes, hypertension, smoking, magnitude of ST elevation in aVR, and TIMI score improves its diagnostic yield.

背景:aVR中8个或更多导联的整体ST段压低和ST段抬高被认为是广泛的心内膜下缺血的标志。它与左主干(LM)或三血管疾病(3VD)有关。但不同的研究显示出不同的结果。我们收集了患者的数据,以了解这些心电图变化与显著的LM干细胞疾病和/或显著的(3VD)之间的关系。方法:TIt是一项在三级护理心脏中心进行的前瞻性观察性研究。纳入所有急性冠状动脉综合征(ACS)患者,这些患者在aVR中出现整体ST段压低和ST段抬高(即在≥8个导联中ST段压低至少0.5 mv,aVR中ST段抬高至少0.5 mv),并接受了冠状动脉造影。结果:我们的研究包括404例有上述心电图表现的患者。我们在67%(n=274)、55%(n=222)和仅29%(n=118)中观察到显著的LM干或显著的3VD。糖尿病、高血压和吸烟等危险因素使这些心电图变化的概率在显著的LM干细胞疾病中分别增加40.4%、32.1%和33.3%,在显著的3VD中分别增加62.7%、57.1%和57.5%。aVR导联ST段抬高幅度≥1mm,对LM干细胞疾病的敏感性增加35%,对3VD的敏感性增加60.4%,TIMI评分≥4,对重大LM干细胞病变的敏感性增加36.7%,对重大3VD的灵敏度增加62.5%。结论:ACS患者aVR中整体ST段压低伴ST段抬高发生LM的概率较低,发生3VD的概率中等。糖尿病、高血压、吸烟、aVR中ST段抬高程度和TIMI评分等因素可提高其诊断率。
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引用次数: 0
Marked Acanthocytosis Associated With Klipple Trenaunay Syndrom. 与Klipple Trenaunay综合征相关的显著棘细胞增多症。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11307
Syeda Mah Ali, Naila Raza, Eraj Aftab, Mahnoor Faisal Mohammad

Klipple-Trenaunay syndrome (KTS) is an extremely rare congenital vascular disorder with poorly defined incidence and prevalence. We report a case of a patient who presented after road traffic accident with primary complaints of poor wound healing and persistent bleeding from wound site. Discernible presence of arteriovenous malformation and skin hypertrophy since birth lead to the diagnosis of Klipple-Trenaunay syndrome (KTS). There was an incidental finding of acanthocytosis on peripheral film of blood which remained elevated even after clinical improvement of the patient. This case report highlights a close association of marked acanthocytosis of red blood cells and Klipple-Trenaunay syndrome.

kliple - trenaunay综合征(KTS)是一种极其罕见的先天性血管疾病,发病率和患病率不明确。我们报告一个病例的病人谁提出后道路交通事故的主要投诉,伤口愈合不良和持续出血的伤口部位。自出生以来明显存在的动静脉畸形和皮肤肥大导致Klipple-Trenaunay综合征(KTS)的诊断。偶然发现外周血膜棘细胞增多症,即使在患者临床好转后仍升高。本病例报告强调了红细胞棘细胞增多症与Klipple-Trenaunay综合征的密切联系。
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引用次数: 0
Efficacy Of Sub-Tenon's Block In Paediatric Strabismus Surgery In Terms Of Reduction In Oculocardiac Reflex. 腱膜下阻滞在小儿斜视手术中减少眼球反射的功效
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11512
Noor Ul Hussain, Muhammad Naeem Khan

Background: Strabismus is a common disease entity in paediatric age group usually requiring surgical intervention under general anaesthesia and Oculocardiac reflex is the most dangerous complications intraoperatively. Various anaesthetic options have been evaluated to mitigate this complication. The objective of this study was to assess the efficacy of sub-tenon's block in paediatric strabismus surgery in terms of reduction in oculocardiac reflex.

Methods: This prospective Randomized control trial was conducted at Department of ophthalmology, MTI, Hayatabad Medical complex, Peshawar over a period of six months from 1st July to 31st December 2021. A total of 124, were equally divided in subtenon's group (Group A) and placebo group (Group B). Intraoperatively, patients were assessed for Bradycardia and development of OCR. Data including demographics, intraoperative BP, HR and OCR development were noted and analysed with SPSS version 22.

Results: The total number of patients were 124, 62 in each group with a mean age of 9.45±1.61. Sixty-six (53.22%) patients were males and 58 (46.87%) patients were females. At 10-, 20- and 30-minutes interval, the SBP and DBP has no significant difference. At 10, 20, 30 minutes interval, the HR differed significantly (79.33±7.36 vs 66.65±6.83 (p˂0.05), 79.78±7.63 vs 66.57±7.06 (p˂0.05), 79.80±7.78 vs 66.52±7.01 (p˂0.05), respectively. Intraoperative OCR was recorded in 13 (21%) patients in sub-tenon's (Group A) versus 56 (90.30%) patients in placebo (Group B) with statistically significant difference between the two (p<0.05).

Conclusion: Sub-tenon's injection of bupivacaine reduces the incidence of Bradycardia and OCR in squint surgery patients after general anaesthesia induction and usage is recommended in routine.

背景:斜视是儿科常见疾病,通常需要在全身麻醉下进行手术治疗,而眼球反射是术中最危险的并发症。为减轻这一并发症,已对各种麻醉方案进行了评估。本研究的目的是评估腱膜下阻滞在小儿斜视手术中减少眼心反射的疗效:这项前瞻性随机对照试验于 2021 年 7 月 1 日至 12 月 31 日在白沙瓦哈亚塔巴德医疗中心 MTI 眼科进行,为期 6 个月。共有 124 名患者被平均分为亚腱膜组(A 组)和安慰剂组(B 组)。术中对患者的心动过缓和 OCR 发展情况进行评估。数据包括人口统计学、术中血压、心率和 OCR 发展情况,并用 SPSS 22 版进行分析:患者总数为 124 人,每组 62 人,平均年龄(9.45±1.61)岁。66例(53.22%)患者为男性,58例(46.87%)患者为女性。间隔 10、20 和 30 分钟时,SBP 和 DBP 无显著差异。在 10 分钟、20 分钟和 30 分钟间隔内,心率有明显差异(分别为 79.33±7.36 vs 66.65±6.83 (p˂0.05)、79.78±7.63 vs 66.57±7.06 (p˂0.05)、79.80±7.78 vs 66.52±7.01 (p˂0.05))。13(21%)名腱膜下注射患者(A 组)与 56(90.30%)名安慰剂患者(B 组)术中记录到 OCR,二者之间存在显著统计学差异(p 结论:腱膜下注射布比卡因可降低全身麻醉诱导后斜视手术患者心动过缓和 OCR 的发生率,建议常规使用。
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引用次数: 0
Vaginal Cuff Dehiscence After Total Laparoscopic Hysterectomy: Prospective Comparison Of Two Types Of Suturing Techniques. 腹腔镜全子宫切除术后阴道袖裂:两种缝合技术的前瞻性比较。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-9828
Samina Saleem Dojki, Alia Bano, Saliha Kanwal

Background: To compare the frequency of vaginal cuff dehiscence after total laparoscopic hysterectomy between two different suturing techniques. Place and duration of study: The study was conducted at three centers; postgraduate tertiary care hospital, university affiliated hospital and private multidisciplinary hospital. The studied duration was from January 2019 to June 2020.

Methods: All patients with indication of total laparoscopic hysterectomy during the study period were included. These were randomly divided in to two groups A and B. Group A was performed upon the conventional interrupted figure of 8 vault suturing and group B with continuous, running, double layered suturing. Keeping the demographics almost same the frequency of a known but rare complication of vaginal cuff dehiscence (VCD) was determined.

Results: A total of 195 patients were enrolled. Of these 87 were in group A and 108 in group B. The results were unequivocal as only one patient had the said complication.

Conclusions: The morbid complication has no relation with the technique of vault suturing.

背景:比较两种不同缝合技术在腹腔镜全子宫切除术后阴道袖带破裂的发生率。研究地点和时间:研究在三个中心进行;研究生三级医院、大学附属医院和私立多学科医院。研究时间为2019年1月至2020年6月。方法:纳入研究期间所有有腹腔镜全子宫切除术指征的患者。随机分为A、B两组,A组采用常规间断式8孔缝合,B组采用连续式、连续式、双层缝合。保持人口统计学几乎相同的频率,一个已知的,但罕见的并发症阴道袖带破裂(VCD)被确定。结果:共纳入195例患者。其中A组87例,b组108例,结果明确,只有1例患者出现上述并发症。结论:畸形并发症与拱顶缝合技术无关。
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引用次数: 1
Comparison Of Chula Formula With Standard 21/23 Rule For Estimation Of Orotracheal Tube Depth In Intubated Adult Patients: A Sindh Institute Of Urology And Transplantation Experience. Chula公式与21/23标准估计成人气管插管深度的比较:信德省泌尿外科和移植研究所的经验。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11237
Ramesh Kumar, Fakhir Raza Haidri, Bushra Zafar

Background: The proper estimation of depth of the orotracheal tube (OTT) in intubated patients is difficult. Several methods have been developed for proper estimation of the depth of OTT. The purpose of this study was to compare two commonly used formulae (21/23 rule and Chula formula) for proper estimation of depth of OTT in our Pakistani population.

Methods: In this randomized interventional study, we included 74 adult patients. The study was conducted in the Intensive care unit of a tertiary care hospital in Karachi, Pakistan, from October 2021 to April 2022. Patients were intubated using either the 21/23 rule (OTT was fixed at 21 cm in females and 23 cm in males from the right incisor) or the Chula formula (OTT was fixed at the right incisor according to the height based formula, {(height in cm/10)+4}). The distance between the carina and the OTT tip was measured using the digital chest x-ray with a PACS software.

Results: A total of 74 patients were intubated in which 32 were intubated using 21/23 rule and 42 were intubated using the Chula formula. Four female patients in 21/23 rule group encountered unsafe distance between the carina and the tip of the OTT (i.e., <2 cm) while no such complications were reported in Chula formula group (p-value 0.031).

Conclusions: Chula formula was safe method for OTT placement in our study. Further studies with larger sample size are needed to assess the safety and efficacy of Chula formula for Pakistani Population.

背景:正确估计气管插管患者的气管插管深度是一个困难的问题。目前已经发展了几种正确估计OTT深度的方法。本研究的目的是比较两种常用的公式(21/23规则和Chula公式),以正确估计我们巴基斯坦人群的OTT深度。方法:在这项随机介入研究中,我们纳入了74名成年患者。该研究于2021年10月至2022年4月在巴基斯坦卡拉奇一家三级护理医院的重症监护室进行。患者插管采用21/23规则(OTT固定在距右切牙21 cm和23 cm处)或Chula公式(OTT固定在距右切牙处,根据身高公式{(身高厘米/10)+4})。使用PACS软件进行数字胸片测量隆突与OTT尖端之间的距离。结果:共74例患者插管,其中32例采用21/23规则插管,42例采用丘拉公式插管。21/23规则组有4例女性患者出现隆突与OTT尖端距离不安全(即:结论:Chula配方在本研究中是一种安全的OTT放置方法。需要进一步开展更大样本量的研究来评估Chula配方对巴基斯坦人群的安全性和有效性。
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引用次数: 0
Sessile Pelvic Osteochondroma: A Rare Case That Required Abdominal Wall Reconstruction After Excision. 骨盆无底性骨软骨瘤:一例手术切除后需重建腹壁的罕见病例。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11151
Mehroze Zamir, Nasir Ahmed, Faizan Iqbal, Syed Wajahat Kamal

Osteochondroma are the most common benign tumours of the bone presenting in the young age group. Commonly found at the metaphysis of the long bones and mostly pedunculated; atypical locations and sessile appearances have also been described in literature. Due to the risk of conversion to malignant chondrosarcoma, the recommended treatment of these lesions is complete excision. We encountered a similar sessile growth in the pelvic region of a 21-year-old male with complaints of pain and swelling. After thorough investigation, excisional biopsy was performed and abdominal wall repair was reinforced with a polypropylene mesh. Careful evaluation, adequate investigations and meticulous surgical treatment can avoid potential problems in managing these tumours.

骨软骨瘤是最常见的良性肿瘤的骨骼呈现在年轻的年龄组。常见于长骨的干骺端并且大部分有花序梗;非典型的位置和无梗的外观也被描述在文献中。由于有转化为恶性软骨肉瘤的危险,这些病变的推荐治疗是完全切除。我们遇到了一个类似的无根生长在骨盆区域的21岁男性与疼痛和肿胀主诉。彻底检查后,行切除活检,并用聚丙烯网片加固腹壁修复。仔细的评估,充分的调查和细致的手术治疗可以避免潜在的问题在管理这些肿瘤。
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引用次数: 0
Frequency Of Stone Clearance After Transurethral Fragmentation Of Large Urinary Bladder Calculi Using Pneumatic Swiss Lithoclast. 气压瑞士碎石对经尿道大膀胱结石碎裂后结石清除频率的影响。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-10910
Shawana Asad, Bilawal Gul, Mir Jalal-Ud-Din, Sher Ali Khan, Rabeeha Bashir, Hina Rafaqat

Background: Vesical calculi refer to stones in the urinary bladder. The causes of bladder stones include bladder outlet obstruction, neurogenic voiding dysfunction, infection, or foreign bodies. Very rarely, these vesical calculi may reach very large sizes and the largest dimension can sometimes reach 13 centimetres.

Methods: This descriptive cross-sectional study was conducted from 1ST May 2019 to 31st October, 2019 at Institute of Kidney Diseases, Urology Department, Hayatabad Peshawar. 164 patients with vesical stone were included in study. Ultrasound-KUB was used for diagnosis of vesical stone and after informed consent, and they underwent transurethral nephroscopic lithotripsy via the pneumatic Swiss Lithoclast.

Results: Frequency of stone clearance was 96.34%. No statistically significant association of stone clearance was observed with age, gender, number of stones or max dimension of largest stone in the bladder (p>0.05).

Conclusions: Transurethral nephroscopic pneumatic lithotripsy via pneumatic Swiss Lithoclast is safe and effective procedure for treatment of large vesical stones. However, this being the first such study in adults, more data is needed to confirm these findings.

背景:膀胱结石是指膀胱内的结石。膀胱结石的病因包括膀胱出口梗阻、神经源性排尿功能障碍、感染或异物。极少数情况下,这些膀胱结石可达到非常大的尺寸,最大的尺寸有时可达到13厘米。方法:本描述性横断面研究于2019年5月1日至2019年10月31日在白沙瓦哈亚塔巴德泌尿科肾脏疾病研究所进行,纳入164例膀胱结石患者。超声- kub用于诊断膀胱结石,经知情同意后,经尿道肾镜气压瑞士碎石机进行碎石术。结果:结石清除率为96.34%。结石清除与年龄、性别、结石数量、膀胱最大结石最大尺寸无统计学意义(p < 0.05)。结论:经尿道肾镜气压碎石是治疗大膀胱结石安全有效的方法。然而,这是第一次在成人中进行这样的研究,需要更多的数据来证实这些发现。
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引用次数: 0
Association Of Low Gfr And Non-Alcoholic Hepatosteatosis On Fibroscan Of Liver. 肝纤维扫描显示低Gfr与非酒精性肝纤维化的关系
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11051
Khurram Baqai, Mahvesh Mahmud, Maryam Anwar, Shaukat Ali, Nida Hussain, Ambreen Wasim

Background: Chronic kidney disease (CKD) and non-alcoholic fatty liver disease (NAFLD) are becoming a health concern, owing to their increasing incidence and prevalence. Both entities are linked to poor outcomes and increased costs, hence greatly impacting the healthcare system and economy. Therefore, it is imperative to establish the link between the two, so as to prevent disease progression and complications.

Methods: The study was an observational retrospective study done in Karachi, from November 2021 to May 2022. It was conducted on 255 patients who were diagnosed with NAFLD, and the presence of CKD was then determined by calculating their GFRs.

Results: Out of the 255 patients diagnosed with hepatosteatosis, 76% had a normal GFR, 20% had a mild decrease and 4% were noted to have a moderate reduction in their GFR. When cross-tabulated with CAP score, it was found that 28% had S1 grade steatosis, out of which 85% had a normal GFR, 13% had a mild decrease and 2% had a moderate decrease in GFR. 22% had S2 grade steatosis, out of which 76% had a normal GFR, 18% had a mild decrease and 6% had a moderate reduction in GFR. 50% patients had S3 grade steatosis, out of which 70% had a normal GFR, 25% had a mild decrease and 5% had a moderate reduction in GFR.

Conclusions: There is an association present between NAFLD and the development of low GFR. Therefore, it is important that patients diagnosed with NAFLD are regularly screened for CKD, so as to prevent its development and complications.

背景:慢性肾脏疾病(CKD)和非酒精性脂肪性肝病(NAFLD)正成为一个健康问题,由于其发病率和患病率的增加。这两个实体都与不良结果和成本增加有关,因此对医疗保健系统和经济产生了极大影响。因此,必须建立两者之间的联系,以防止疾病的进展和并发症。方法:该研究是一项观察性回顾性研究,于2021年11月至2022年5月在卡拉奇进行。该研究对255名被诊断为NAFLD的患者进行了研究,然后通过计算他们的gfr来确定CKD的存在。结果:在255例被诊断为肝纤维化的患者中,76%的患者GFR正常,20%的患者GFR轻度下降,4%的患者GFR中度下降。当与CAP评分交叉表时,发现28%为S1级脂肪变性,其中85%的GFR正常,13%的GFR轻度下降,2%的GFR中度下降。22%为S2级脂肪变性,其中76%的患者GFR正常,18%的患者GFR轻度降低,6%的患者GFR中度降低。50%的患者为S3级脂肪变性,其中70%的患者GFR正常,25%的患者GFR轻度降低,5%的患者GFR中度降低。结论:NAFLD与低GFR之间存在关联。因此,诊断为NAFLD的患者定期筛查CKD,以预防其发展和并发症是很重要的。
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引用次数: 0
Ganglion Impar Block For Chronic Coccydynia. 神经节阻滞治疗慢性尾骨痛。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11092
Sara Haider Malik, Khaleel Ahmad, Liaquat Ali

Background: Coccydynia is also known as coccygodynia or coccygeal neuralgia. Coccyx is a triangular bone located within the vertebral column. The cause of coccydynia is unknown in the literature; however, it is found to be common among obese individuals, particularly females. The probability of women having coccydynia is five times higher than among men, which may result from larger pressure during pregnancy and child delivery. It is treated well with ganglion impar block. The aim of our study was to assess pain relief after Ganglion Impair Block with subsequent improvement in quality of life.

Methods: A single-arm study was done in the department of Pain Medicine, Fauji Foundation Hospital, Rawalpindi from July 2021 to June 2022. A total of 50 patients with ≥3 months' coccygeal pain of either gender aged between 20-60 years unresponsive to analgesics & anti-inflammatory medications with no laboratory abnormalities were included. Fluoroscopic guided trans-sacro‑coccygeal ganglion impair block was done with alcohol neurolysis. The patients were observed for 1 hour in the recovery room to record post-intervention complications that may occur like hypotension, bradycardia, signs, and symptoms of cardiotoxicity or neurotoxicity etc. and evaluated for pain scores on the basis of the numeric rating scale (NRS). Data collected was analyzed using the statistical package for social scientists (SPSS) version 21. The quantitative data, i.e., age and NRS score were analyzed using mean and standard deviation and compared between pre and post-intervention periods.

Results: The data from 50 patients who completed the follow-up period were used for analysis. The average age of the patients was 42.9±8.39 years, with a range of 38-60 years. Based on the data obtained, 30% of the patients experienced trauma (that is falling on the coccyx region). The mean score from NRS before intervention was 7.80±0.16 which decreased to 0.96±0.35, respectively, and this difference was statistically significant (p-value, <0.001).

Conclusions: Ganglion Impar neurolysis is highly effective in the treatment of chronic coccydynia.

背景:尾骨痛也被称为尾骨痛或尾骨神经痛。尾骨是位于脊柱内的三角形骨。尾骨痛的病因在文献中尚不清楚;然而,它在肥胖人群中很常见,尤其是女性。女性患尾骨痛的概率是男性的五倍,这可能是由于怀孕和分娩时压力较大。神经节阻滞治疗效果良好。我们研究的目的是评估神经节损伤阻滞后疼痛的缓解以及随后生活质量的改善。方法:于2021年7月至2022年6月在拉瓦尔品第福吉基础医院疼痛内科进行单臂研究。共纳入50例尾骨疼痛≥3个月的患者,年龄在20-60岁之间,男女不限,对镇痛和抗炎药物无反应,无实验室异常。透视引导下经骶尾骨神经节损伤阻滞采用酒精神经松解术。患者在康复室观察1小时,记录干预后可能出现的低血压、心动过缓、心脏毒性或神经毒性的体征和症状等并发症,并根据数字评定量表(NRS)进行疼痛评分。收集的数据使用社会科学家统计软件包(SPSS)第21版进行分析。采用均数和标准差对年龄和NRS评分等定量数据进行分析,并对干预前后进行比较。结果:对50例完成随访期的患者资料进行分析。患者平均年龄42.9±8.39岁,年龄范围38 ~ 60岁。根据获得的数据,30%的患者经历了创伤(即落在尾骨区域)。干预前NRS评分均值为7.80±0.16,干预前NRS评分均值为0.96±0.35,干预前NRS评分均值降至0.96±0.35,差异有统计学意义(p值)。结论:神经节Impar神经松解术治疗慢性尾骨痛疗效显著。
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引用次数: 0
Proteus Syndrome: A Rare Disease Of Disproportionate And Asymmetric Overgrowth Of Connective Tissue. 变形肌综合征:一种罕见的结缔组织不成比例和不对称过度生长的疾病。
Q3 Medicine Pub Date : 2023-02-01 DOI: 10.55519/JAMC-01-11210
Fahad Qaisar, Nauman Ismat Butt, Muhammad Sohail Ajmal Ghoauri, Madiha Azhar, Muhammad Umair Javed, Abdul Samad

Proteus syndrome is a rare disease manifested by progressive segmental overgrowth involving the skeletal, Cutaneous, subcutaneous, and nervous systems. We report the case of a 24-year-old female who was born with no obvious abnormality at birth. From the age of 1 year, she developed asymmetric enlargement of her left upper limb and bilateral lower limbs leading to enlargement of the right-hand phalanges with radial deviation, enlargement of the right big toe, lateral deviation of left foot, and discrepancy in the length of lower extremities and kyphoscoliosis. She had become bed-bound for the last few years due to increasing disability. She was diagnosed with Proteus syndrome based on clinical features of progressive course, mosaic distribution, and sporadic occurrence of the lesions.

变形肌综合征是一种罕见的疾病,表现为累及骨骼、皮肤、皮下和神经系统的进行性节段性过度生长。我们报告一例24岁的女性,出生时没有明显的异常。从1岁开始,患者出现左上肢和双下肢不对称肿大,导致右指骨肿大,伴桡骨偏移,右大脚趾肿大,左脚外侧偏移,下肢长度不一致,并发脊柱后凸。由于残疾日益严重,过去几年她一直卧床不起。根据病程进展、马赛克分布和病灶散发的临床特征,诊断为变形肌综合征。
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引用次数: 1
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Journal of Ayub Medical College, Abbottabad : JAMC
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