Unclassified Osteochondrodystrophies with Extreme Platyspondyly (Report of two cases)

Two girls with different unclassified bone dysplasias characterised by severe, generalised platyspondyly are reported. In view of unrewar ding biochemical investigations the clinico-radiographic examinations remain the most im portant method of evaluation in this group of diseases.     

Metatropic Dwarfism and its Variants (Report of six cases)

Six cases of METATROPIC DWARFISM or its VARIANTS–four which are the first Australian cases and one which is the first Indonesian case–are reported. Four of the cases had characteristic clinical and radiographic appearances whereas two, who died in early infancy, showed some variability in radiographic features. In one of the variants microscopy showed histologic features consistent with metatropic dwarfism. Microscopic, ultrastructural and biochemical studies of cartilage should be performed in all the cases of bone dysplasias in which the radiographic diagnosis is unknown or uncertain.     

骨性纤维发育异常4例临床病理分析

目的探讨骨性纤维发育异常这一新病种的临床、放射学和病理特征。方法对4例骨性纤维发育异常患者的临床资料进行分析,并与10例骨纤维结构不良者进行对照分析。结果4例骨性纤维发育异常患者中3例行病灶清除＋自体植骨术,1例仅行病灶切除＋碘酊烧灼骨腔内壁。随访3年,其中1例术后2年复发。结论骨性纤维发育异常其临床表现、放射学和病理学改变均有其自身固有的特点,不应与骨纤维结构不良和长骨骨化性纤维瘤相混淆。     

Dominant Craniometaphyseal Dysplasia — A Family Study Over Five Generations

A two month old male infant being investigated for nasal obstruction was noted to have the typical facies and radiological changes of craniometaphyseal dysplasia. Investigation of the family detected 9 individuals in 4 generations with radiological evidence of craniometaphyseal dysplasia. Their ages ranged from 2 months to 70 years. Three presented with nasal obstruction, one with facial nerve dysfunction and three had developed deafness in or before their third decade. Two were asymptomatic. One family member, now dead, was known to have had early onset of deafness but had not been examined or X-rayed. All of the affected individuals were noted to have had the typical facies in childhood. Characteristic radiological findings in the cranium and long bones were present to a varying degree and were most prominent in those presenting in infancy.     

Cranio-Metaphyseal Dysplasia (Report of 3 cases - two infants and one adult)

Two infants were investigated because of facial nerve palsy. Clinical examination suggested the diagnosis of cranio-metaphyseal dysplasia and radiographic examination was typical of this. The mother of one of the infants was affected along with three relatives. This bone dysplasia is characterised by sclerosis of the skull and abnormal modelling of the long bones often heralded by neurological complication. The radiographic features are typical.     

Rhizomelic Bone Dysplasia with Club-like Femora (Case report and confirmation of a syndrome)

A newborn with rhizomelic bone dysplasia with club-like femora is reported. This is the fourth case Of this easily recognizable, recently reported, congenital bone disease. Maroteaux et al recently reported two patients with rhizomelic bone dysplasia and club-like femora as a distinctive new bone dysplasia. Their observation was confirmed by Gugliantini et al (2) who reported another case. This paper reports a fourth patient with this easily recognisable disorder.     

Bilateral Glenoid Dysplasia (Report of 8 Cases)

8 cases of isolated glenoid hypoplasia, 6 of them among children are reported. Although it is a benign finding patients with this anomaly should abstain from excessive sport activities and heavy manual work.     

Spondylo - Epiphyseal Dysplasia Ribbing - Fairbank Type Report of Three Cases

Spondyloepiphyseal Dysplasia Ribbing-Fairbank type in three members of a family are described. A fourth member of the family was also probably affected. The importance of the radiographic examination for the recognition of the disease is stressed and the radiographic differential diagnosis is reviewed.     

Inter- and Intra-Observer Variability in Diagnosis of Oral Dysplasia

Background: Oral potentially malignant disorders (OPMDs) are lesions from which malignancy is more likely to develop that from other tissues. The potential for malignant transformation of OPMDs is estimated by determining the degree of dysplastic changes in the epithelium. Dysplasia grading has been criticized for lack of reproducibility and poor predictive value but is still considered the gold standard for diagnosing OPMDs. Since grading of dysplasia is based on architectural and cytological changes, there can be considerable inter- and intra-observer variability due to subjective impressions. This aim in this study was to assess the degree of agreement between two pathologists grading dysplasia in the same patients and review the existing grading system. Materials and Methods: In this hospital-based cross-sectional study, 100 patients with clinically diagnosed OPMDs were subjected to biopsy followed by histopathological examination. The slides were examined by two pathologists using WHO and binary systems of classification and both were blinded to the clinical and each other’s histological diagnosis. For statistical analysis the Chi square test was applied. Results: Statistical analysis showed poor inter-observer variability with P values of 0.8 using the WHO classification and 0.3 using the binary classification. Conclusion: Our study provides evidence that the existing systems for grading dysplasia are not competent to rule out subjectivity. There is a need for a classification system that can overcome this drawback.     

Cranio-Diaphyseal Dysplasia: Report of a Case

Cranio-diapyseal dysplasia is one of the rarest of the well differentiated sclerotic bone disorders. This is the first case of this rare syndrome reported in the Australian literature.     

食管上皮增生细胞学显微测量分级的图像分析

应用图像分析技术检测50例食管拉网涂片中经光镜测量协诊的食管鳞状上皮正常、轻度增生、重增Ⅰ级、重增Ⅱ级和近癌细胞的核面积、平均卡规直径及最大和最小直径四项参数。结果表明:各项参数均值随着增生级别增高逐级增加(P<0.01),判别回代符合率为84％,各级增生细胞核增大的程度与分级判别标准一致。证实在食管上皮增生细胞学分级诊断时应用显微测量定量方法经光镜测微目尺测算核的算术平均直径代表核面积进行增生细胞的分级是可行的。     

额眶部骨纤维结构不良30例：三球法定位视神经管及临床效果评估

目的 研究额眶部骨纤维结构不良患者视神经管减压的适应证,以及术中视神经管定位和减压的方法。方法 收集我科骨纤维异常增殖病例共30例,术前进行充分的影像学评估,有症状者进行手术,包括眶部成型术和视神经管减压术。手术采用额颞硬膜外入路,如有凸眼则行经眶上缘入路。术中有6例进行了CT和MRI融合导航,协助寻找视神经的眶口或者颅口。术中使用三球法行视神经管减压,确认视神经位置。结果 视神经管减压30例,其中1例失明。患者视力和视野有不同程度改善,手术后凸眼消失或者改善。眶顶采用正常内板重建13例,钛板重建5例,未重建9例,2例用增生的碎骨铺于眶顶,但其中复发1例,表现为5年后再次出现眼球突出,而视力无下降。结论 额眶部骨纤维结构不良患者应积极手术治疗,视力下降应选择视神经管减压,合并面部畸形的同时做颅面部成型手术和眶减压术;硬膜外入路从眶口或者颅口定位视神经,结合三球法,可以做到安全且彻底的视神经管减压。     

胃粘膜灶性异型增生的形态研究

３９６例活检胃粘膜炎症性病变中１９．９％找见异型增生灶，其由单个至１０多个细胞构成，可向管腔内或外突起。其检出率为糜烂性胃炎的８．０％，浅表性胃炎的１０．９％，萎缩性胃炎的３８．４％，慢性溃疡的１８．９％。灶性异型增生细胞ＰＣＮＡ阳性表达６１．３％；ＡｇＮＯＲ计数３．８１～８．７２个，提示ＰＣＮＡ和ＡｇＮＯＲ检测可帮助判断胃粘膜灶性异型增生。     

Spondylo-Costal Dysplasia — Severe and Moderate Types (Report of 8 Cases)

Eight cases of spondylo-costal dysplasia, three of severe and five of moderate types are described. In the author's opinion spondylocostal dysplasia represents a group of genetically different diseases characterised radiologically by malsegmentation of the spine and dysplastic/hypoplastic changes of the ribs. Spondylo-costal dysplasia is a relatively common finding in severe forms of myelomenuigocoele.     

Accuracy of Visual Inspection with Acetic Acid (VIA) for Early Detection of Cervical Dysplasia in Tehran,Iran

Objective: To evaluate the accuracy of visual inspection with 5% acetic acid (VIA) when used to detect cervical ‍cancer and its precursors. ‍Methods: The study population included women attended Family Planning and Gynecological Clinic in Bagher ‍Abad Health Center and Mirza Koochak Khan Hospital for regular cervical screening tests. After obtaining informed ‍consent from each woman, VIA was performed. One hundred with a positive VIA test and 100 women with a ‍negative VIA test were randomly selected for this study. Cytology and colposcopy examination were performed for ‍all 200 cases and cervical biopsies were conducted for those individuals showing abnormal colposcopic findings. ‍Results: Nine cases in VIA-positive group and two cases in VIA-negative group had an abnormal cytology. Ninety ‍five women in the VIA-positive group and 25 in the VIA-negative group had abnormal colposcopic findings. From ‍biopsy examination, 67 (71%) of cases in the VIA-positive group and 3 (12%) cases in the VIA-negative group had ‍a final diagnosis of dysplasia. Among biopsied samples, only 7 cases of VIA-positive group showed abnormal result ‍and the remaining were normal. Based on these results, VIA test sensitivity and specificity were 95.7% and 44.0% ‍respectively, while they were 10% and 92% for cytology tests. ‍Conclusions: The results of this study indicate that although VIA is a sensitive screening test for detection of ‍cervical dysplasia, it can not be used by itself. Applying VIA along with Pap smears helps to detect a higher number ‍of cases with cancer precursor lesions.     

双侧乳腺癌（附14例报告）

自１９７２年至１９８９年，共收治乳腺癌９８４例。其中双侧性乳腺癌１４例（１．４％），均为女性。同时性５例（０．５％），异时性９例（０．９％），间隔时间为１０个月至１５年８个月，平均为５年８个月。年龄在３０～６４岁，平均４３．４岁。全部病例两侧乳癌病灶均经病理证实。作者认为对单侧乳癌治疗后患者应长期密切随访，对高危患者在对侧乳腺出现可疑病灶时应及时做活检。双侧乳腺癌的预后与肿瘤大小、腋窝淋巴结受累情况、治疗是否及时、正确，两侧病灶同时或异时发生及间隔时间的长短等因素有关。双侧乳腺癌经及时、积极、合理治疗后，预后并不差于单侧乳腺癌。     

骨纤维结构不良的流行病学与临床分析

目的 分析单中心30年来收治的骨纤维结构不良患者的流行病学特征和临床特点。方法 我院在1991年到2020年共有744例病理明确诊断为骨纤维结构不良的患者住院接受外科治疗,所有患者的流行病学特征包括年龄、性别、部位,临床特征包括发或多发、是否发生病理骨折及恶变情况进行分析。结果 744例患者共有1 183处病变,患者入院时平均年龄31.1±13.5岁,男女比例接近1:1。最常见的部位是下肢916处（77.4%）,其次是上肢106处（9.0%）。下肢最常见的部位是股骨645处（54.5%）和胫骨224处（18.9%）。多发占25.4%,单发占74.6%。就诊时发生病理骨折的患者163例（13.8%）。发生恶变的患者有6例（0.8%）,平均年龄40.5岁（32~47）岁,平均恶变时间为7.7年。结论 骨纤维结构不良是一组罕见的良性骨肿瘤,具有典型的流行病学特征和临床特点。     

p16蛋白在前列腺癌及非典型增生中的表达

目的　研究p16蛋白在前列腺癌及非典型增生中的表达。方法　采用免疫组织化学 (ABC)法 ,对 40例前列腺癌及 3 0例前列腺非典型增生进行p16蛋白的免疫组化检测。结果　 40例前列腺癌中有 18例为阳性反应 ( 4 5 % ) ,3 0例前列腺非典型增生中 7例为阳性反应 ( 2 3 % ) ,p16蛋白在前列腺癌中的表达明显高于前列腺非典型增生。结论　p16基因在前列腺癌的发生发展中发挥重要作用。     

嗜铬细胞瘤的诊断问题：附14例分析

本文分析经手术、病理确诊嗜铬细胞瘤14例,其中2例恶性,肿瘤在肾上腺者11例,肾上腺外者3例,主要表现为高血压和代谢紊乱,呈阵发性高血压11例,持续性高血压3例。阵发性高血压的发作日及持续性高血压者VMA均增高。儿茶酚胺的测定有一定意义,肾上腺区B超检查可为首选定位诊断方法,临床高度怀疑属肾上腺嗜铬细胞者,CT检查有较高的诊断价值。本文就嗜铬细胞瘤的诊断进展略加讨论。