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A Review of Literature on Mrkh Syndrome Mrkh综合征的文献综述
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/2980f
Nidhi Jain, J. Kamra
Primary amenorrhea is described as the inability to reach menarche until the age of 14 in the lack of normal secondary sexual characteristics, or until the age of 16 in the absence of secondary sexual characteristics. Gonadal pathology is the most common cause of primary amenorrhea, followed by MRKH syndrome. MRKH syndrome is an uncommon congenital condition characterised by aplasia of the uterus and vaginal wall. It happens when the Mullerian duct fails to grow properly.It occurs once every 4500 female births. Primary amenorrhea affects mostly girls. It is defined by the presence of normal secondary sexual features, a normal 46 XX genotype, normal ovarian function in the majority of cases, and a missing or undeveloped uterus and upper part (2/3) of vagina. It is divided into two types: type A is an isolated condition, and type B is linked to various renal, skeletal, and cardiac anomalies. Psychological counselling and vaginoplasty are among the options for treatment. Vaginoplasty can be performed using a variety of non-surgical and surgical methods. The authors present a review of the literature on the embryology, etiopathogenesis, work-up, and therapy of MRKH syndrome.
原发性闭经被描述为直到14岁才出现月经初潮,并且没有正常的第二性征,或者直到16岁才出现第二性征。性腺病理是原发性闭经最常见的原因,其次是MRKH综合征。MRKH综合征是一种罕见的先天性疾病,其特征是子宫和阴道壁发育不全。当缪勒管不能正常生长时就会发生。每4500名女性分娩一次。原发性闭经主要影响女孩。它的定义是存在正常的第二性特征,正常的46 XX基因型,大多数情况下卵巢功能正常,子宫和阴道上部(2/3)缺失或未发育。它分为两种类型:A型是一种孤立的疾病,B型与各种肾脏、骨骼和心脏异常有关。心理咨询和阴道成形术是治疗的选择之一。阴道成形术可采用多种非手术和手术方法。作者现对MRKH综合征的胚胎学、发病机制、检查和治疗方面的文献进行综述。
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引用次数: 0
Study of Fibroepithelial Lesions of Breast at a tertiary Care Centre- an Audit, Clinicopathological APPROACH and Comparison with WHO Grading 三级保健中心乳腺纤维上皮病变的研究——审计、临床病理方法和与WHO分级的比较
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/3127f
Deepti Yadav, Mukta Meel, D. Hemrajani, A. Mittal, K. Mathur
Background: Mammary fibroepithelial lesions cover a wide range of malignancies, from indolent fibroadenoma to the potentially fatal phyllodes tumor (PT). The morphological assessment criteria used for classification are typically difficult to apply, and there is no consensus on what constitutes a sufficient resection margin. The current study objectives were to investigate the histopathological spectrum of fibroepithelial lesions of the breast at a tertiary care centre, to stratify and classify various fibroepithelial lesions into fibroadenomas and PT, and to reclassify all confirmed cases of PT seen during the study period using standard histopathological WHO criteria. Methods: Between January 2016 and August 2019, records and slides of fibroepithelial lesions of the breast obtained at the department were retrieved and reviewed. Results: During this time, 891 fibroepithelial lesions of the breast were discovered. There were 826 (92.7%) cases of fibroadenoma and its variations, 34 (3.8%) cases of fibroadenomatoid mastopathy, and 31 (3.5%) cases of PT. Eight (25.8%) of all PT were borderline, four (12.9%) were malignant, and the remaining (61.3%) were benign. Conclusions: Fibroepithelial tumours of the breast are a diverse group of lesions that range in severity from benign fibroadenoma to malignant PT. Various subtypes have overlapping histologic characteristics, and transformation and progression to a more malignant phenotype are possible.  As there are significant clinical disparities among subtypes, precise pathologic categorization is critical for proper management. Despite the fact that some immunohistochemical markers may be helpful in this differential diagnosis, histomorphology is still the gold standard.
背景:乳腺纤维上皮病变涵盖范围广泛的恶性肿瘤,从惰性纤维腺瘤到潜在致命的叶状瘤(PT)。用于分类的形态学评估标准通常难以应用,并且对于什么构成足够的切除边缘没有共识。目前的研究目的是调查三级保健中心的乳腺纤维上皮病变的组织病理学谱,将各种纤维上皮病变分层和分类为纤维腺瘤和PT,并使用标准的组织病理学WHO标准对研究期间看到的所有确诊的PT病例进行重新分类。方法:对2016年1月至2019年8月在该科获得的乳腺纤维上皮病变的记录和切片进行检索和复习。结果:本组共发现乳腺纤维上皮病变891例。纤维腺瘤及其变异826例(92.7%),纤维腺瘤样乳腺病34例(3.8%),PT 31例(3.5%),其中交界性PT 8例(25.8%),恶性PT 4例(12.9%),良性PT 61.3%。结论:乳腺纤维上皮肿瘤是一组不同的病变,其严重程度从良性纤维腺瘤到恶性PT不等。各种亚型具有重叠的组织学特征,并且有可能转化和进展为更恶性的表型。由于不同亚型之间存在明显的临床差异,精确的病理分类对于适当的治疗至关重要。尽管一些免疫组织化学标记物可能有助于这种鉴别诊断,但组织形态学仍然是金标准。
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引用次数: 0
A Review on Brain Metastases Prognostic Scoring Systems 脑转移瘤预后评分系统综述
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/11258d
E. Topkan, A. Kucuk, D. Sezen, S. Senyurek, Eyub Yasar Aydemir, N. Durankus, Y. Bolukbasi, B. Pehlivan, U. Selek
Brain metastases (BMs) represent the most common intracranial tumors, and nearly 25% of all cancer patients are diagnosed with this poor prognostic disease condition somewhere during their treatment course. Depending on the potential patient's general well-being status, the main prevailing treatment options typically incorporate palliative radiotherapy, chemotherapy, targeted agents, immunotherapy, best supportive care, and less frequently surgery in select patients. Past investigations demonstrated the probability to stratify BM patients into particular prognostic gatherings according to the credible combinations of multiple patients- and tumor-related features: the prognostic scoring systems. Such frameworks may serve usefully in the accurate prediction of survival outcomes and the appropriate selection of the best-fit treatment elective. In this present review, we intended to survey the advantages and disadvantages of the broadly recognized on-the-spot prognostic scoring systems for BMs and their clinical merits.
脑转移瘤(BMs)是最常见的颅内肿瘤,近25%的癌症患者在治疗过程中被诊断为这种预后不良的疾病。根据潜在患者的总体健康状况,主要的主流治疗方案通常包括姑息放疗、化疗、靶向药物、免疫治疗、最佳支持性护理和少数患者的手术。过去的研究表明,根据多个患者和肿瘤相关特征的可信组合,有可能将BM患者分层为特定的预后集合:预后评分系统。这样的框架可能有助于准确预测生存结果和适当选择最适合的治疗方案。在本综述中,我们旨在调查广泛认可的脑转移现场预后评分系统的优缺点及其临床价值。
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引用次数: 0
Varied Uses of Bone Allograft in Plastic and Reconstructive Surgery: A Case Series of 13 Patients 同种异体骨移植在整形和重建手术中的不同用途:13例患者的病例系列
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/3149f
Vikas Singh, S. Dang
Background: The availability of safe, clinically useful, and cost-effective bone allografts have led to modifications in surgical therapy, as well as a rise in demand for bone allografts grafts for use in plastic surgery reconstruction.  They are an appealing alternative to bone autograft because their supply is less limited, they allow structural restoration of the skeleton, and their surfaces promote bone formation. Case Series: We provide a case series of 13 patients who were treated with freeze-dried bone allografts. The FDA and the Bone Banks both advised that all grafts meet certain parameters. The objective evaluation of graft volume persistence was achieved using radiography, whereas the subjective estimation of graft volume was obtained through patient response throughout a one-year period of follow-up. Objective of this study was to establish the clinical use of bone allografts in various reconstructive surgeries and compare its utility with bone autografts.   Conclusion: Bone allografts can be used in any type of bone surgery, from minor defects to major bone loss following tumour resection. Without donor-site morbidity, freeze-dried allograft bone is a safe and equal alternative to bone autograft.
背景:安全、临床有用、经济实惠的同种异体骨移植的可用性导致了手术治疗的改变,以及用于整形外科重建的同种异体骨移植的需求的增加。它们是一种有吸引力的自体骨移植替代品,因为它们的供应不那么有限,它们允许骨骼的结构修复,它们的表面促进骨形成。病例系列:我们提供了13例接受同种异体冻干骨移植治疗的患者的病例系列。FDA和骨库都建议所有的移植物都要符合一定的参数。对移植物体积持久性的客观评价是通过x线摄影实现的,而对移植物体积的主观估计是通过患者在一年随访期间的反应获得的。本研究的目的是建立异体骨移植在各种重建手术中的临床应用,并比较其与自体骨移植的效用。结论:同种异体骨移植可用于任何类型的骨手术,从肿瘤切除后的小缺损到大骨丢失。冻干同种异体骨是一种安全、平等的替代自体骨移植的方法。
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引用次数: 1
A Rare Case Report of Acute T-cell Lymphoblastic Leukaemia Presenting with Cutaneous Involvement in a Child 一例罕见的急性t细胞淋巴细胞白血病患儿表现为皮肤受累
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/10757d
Lohit kumar Kalita, C. Kalita, P. Gogoi, U. Sharma
Primary cutaneous involvement in T-cell lymphoblastic leukemia is rare in childhood. We present a case of 6-year- old girl admitted to our hospital because of multiple skin lesions. She was looked pale and weak. Generalized lymphadenopathy was present. Complete blood count revealed 216,000/mm3 white blood cell count. Peripheral blood smear showed 80% lymphoblasts. Bone marrow aspiration revealed 96% blastic cells with immunophenotype and morphological characteristics of acute lymphoblastic leukemia (T-ALL) which was confirmed by flowcytometry. ALL BFM -95 remission induction treatment protocol was started. Skin lesion remained same after two month of the cytotoxic therapy. The symptoms became more aggressive and she died after 4 months of treatment.
原发性皮肤受累的t细胞淋巴细胞白血病是罕见的儿童。我们报告一例六岁女童因多发性皮肤病变而入院。她脸色苍白,身体虚弱。全身性淋巴结病变。全血细胞计数显示白细胞计数216,000/mm3。外周血涂片示淋巴母细胞80%。骨髓穿刺显示96%的母细胞具有急性淋巴细胞白血病(T-ALL)的免疫表型和形态学特征,流式细胞术证实了这一点。开始ALL BFM -95缓解诱导治疗方案。细胞毒治疗2个月后,皮肤病变保持不变。症状变得更加严重,她在治疗4个月后死亡。
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引用次数: 0
A Case Report on Urothelial Carcinoma in the Ureteral Stump of a Nefrectomized Patient by Kidney Exclusion 肾排除术后输尿管残端尿路上皮癌1例报告
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/3196f
J. Campos, Plinio H. F. Leandro, L. D. O. Lima, C. Lima, L. Aquino, Marcos F. H. Rocha
Upper urinary tract urothelial carcinoma is a rare and possibly fatal disease. It sometimes affects the ureter, and it's even unusual in the ureteral stump of a patient who has had a nephrectomy for benign disease.The example of a male patient who underwent total left nephrectomy due to renal exclusion is described here. He was diagnosed with a urethral tumour of the ureteral stump after three years due to an episode of hematuria, and he had ureterectomy and bladder cuff excision. The ureteral stump being involved by a urothelial tumour is an incredibly rare occurrence that is usually linked with a poor prognosis due to the delay in detection caused by atypical presentation. Hematuria is the most prevalent symptom. Because there is no renal unit and hence no hydronephrosis, pain is uncommon.Although rare, primary tumours in the ureteral stump should be remembered, and a high level of diagnostic suspicion should be maintained to avoid diagnosis in the advanced stages. Our objective with this paper was to report a rare presentation of a urothelial carcinoma and to alert physicians.
上尿路尿路上皮癌是一种罕见且可能致命的疾病。它有时会影响输尿管,甚至在因良性疾病而行肾切除术的病人的输尿管残端也不常见。一例男性患者因肾排斥而行全左肾切除术。三年后,由于血尿发作,他被诊断为输尿管残端尿道肿瘤,他接受了输尿管切除术和膀胱袖口切除术。输尿管残端被尿路上皮肿瘤累及是一种非常罕见的情况,通常由于非典型表现导致的检测延迟而导致预后不良。血尿是最常见的症状。由于没有肾单元,因此没有肾积水,疼痛不常见。输尿管残端原发肿瘤虽然罕见,但应记住,并保持高度的诊断怀疑,以避免在晚期诊断。我们这篇论文的目的是报告一个罕见的尿路上皮癌的表现,并提醒医生。
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引用次数: 0
Determination of External Ear Indices by Digital Photometry among Adult Population 数字测光法测定成人外耳指数
Pub Date : 2021-08-02 DOI: 10.9734/bpi/nfmmr/v1/3213f
Swati S. More, Vaishali Mane, Aruna Y. Yadav
Background: External ear is a defining feature of the face as it contributes to facial aesthetics by its appearance and symmetry. Its shape and size is influenced by age, sex and ethnic origin. Features of auricle had long been recognized as an important anthropological variable for studying racial variability and for identifying few genetic abnormalities at an early stage of life. Out of all available methods to study external ear indices, digital photometry is most convenient and useful method. Objectives: To study external ear indices using digital photometry among adult population. Methods: The present cross sectional study consisted of 500 adult subjects between 20 to 30 years of age. Patients with malignancies, previous surgery or trauma to the earlobe, or congenital earlobe anomalies were excluded. Ear features were then captured using a digital camera mounted on stand. Various soft tissue landmarks were tagged on the photo and the various tagged points were connected on the photo. Different parameters on right and left ear were measured. The indices like auricular, lobular and conchal were computed. The measurements were statistically analyzed by calculating their mean and standard deviations. Results: There were 250 women and 250 men. Oval shape of auricle was more common both in males as well as in females. Auricular and conchal index in males as well as females on right and left side were statistically insignificant. These indices were significantly different in both genders. Lobular index in males on right and left side was statistically not significant, but was significant in females. Gender wise lobular index on right side was statistically significant. Conclusion: The result of the present study also can be used in the field of Forensic Science for excluding criminals.  Ear Biometrics is a promising new passive approach to Human Identification system used for screening people. This knowledge will be useful in designing a new identification tool-‘Ear Biometrics’
背景:外耳是面部的一个决定性特征,因为它的外观和对称性有助于面部美学。它的形状和大小受年龄、性别和民族血统的影响。长期以来,耳廓特征一直被认为是研究种族差异和识别生命早期少数遗传异常的重要人类学变量。在所有可用的研究外耳指数的方法中,数字测光法是最方便和有用的方法。目的:应用数字光度法研究成人外耳指数。方法:本横断面研究包括500名年龄在20至30岁之间的成人受试者。排除有恶性肿瘤、既往耳垂手术或外伤、先天性耳垂异常的患者。然后用安装在支架上的数码相机捕捉耳朵的特征。在照片上标记各种软组织地标,并在照片上连接各种标记点。测量了左右耳的不同参数。计算耳廓、小叶、耳廓等指标。通过计算其平均值和标准差对测量结果进行统计分析。结果:女性250例,男性250例。卵形耳廓在男性和女性中都更为常见。男性、女性左右侧耳廓、耳道指数差异均无统计学意义。这些指标在两性间存在显著差异。男性左右侧小叶指数差异无统计学意义,但女性有统计学意义。右侧小叶指数性别差异有统计学意义。结论:本研究结果可用于法医学领域排除罪犯。耳部生物识别技术是一种很有前途的被动的人体识别技术。这些知识将有助于设计一种新的识别工具——“耳朵生物识别”。
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引用次数: 0
Serum Zinc Level Estimation- Comparision between Normal Control and in Leprosy Patients: Clinical Presentations 血清锌水平的估计-比较正常对照和麻风病患者:临床表现
Pub Date : 2014-02-01 DOI: 10.9734/bpi/nfmmr/v1/11351d
Pramila Jain, V. Khare, Ashish Koshti, R. Malik, Bhawna Bhimte
Leprosy is a chronic infectious disease caused by Mycobacterium leprae. It principally affects the cooler parts of the body, mainly skin and peripheral nerves. Leprosy involves wide range of biochemical as well as immunological changes in the body including trace elements such as zinc, copper and magnesium. After iron, zinc is the second most abundant trace element in the body. This study has been undertaken to correlate with clinical presentations wherever possible and to investigate the level of serum zinc in leprosy. Study included 63 newly diagnosed leprosy. Cases were further distributed according to clinical subtypes and were compared to controls. Venous blood samples were collected and estimation of serum zinc level has been done with serum. Maximum patients detected were of Intermediate Leprosy (26.9) where as lesser (1.58)were of Histoid type. Pure tuberculoid and Indeterminate leprosy group showed minimum decrease and pure lepromatous and Erythema nodosum leprosum group showed maximum decrease in Serum Zinc level i.e. leprosy patients with increased bacterial load have decreased serum zinc levels. This suggests that there could be a correlation of serum zinc levels and the bacillary load.
麻风病是一种由麻风分枝杆菌引起的慢性传染病。它主要影响身体较冷的部位,主要是皮肤和周围神经。麻风病涉及体内广泛的生化和免疫变化,包括锌、铜和镁等微量元素。锌是人体中仅次于铁的第二丰富的微量元素。本研究的目的是尽可能与临床表现相联系,并调查麻风病患者血清锌水平。研究对象包括63名新诊断的麻风病患者。病例进一步根据临床亚型进行分布,并与对照组进行比较。采集静脉血,用血清测定血清锌水平。检测到的患者中,中度麻风最多(26.9例),组织型麻风较少(1.58例)。纯结核性和不确定型麻风组血清锌水平下降最小,纯麻风性和结节性红斑麻风组血清锌水平下降最大,即细菌负荷增加的麻风患者血清锌水平下降。这表明血清锌水平与细菌负荷可能存在相关性。
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引用次数: 2
期刊
New Frontiers in Medicine and Medical Research Vol. 1
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