广州天河区2001-2010年传染病流行概况分析

目的探讨10年来传染病发生趋势,为制定防控措施提供参考依据。方法采用描述性流行病学方法对本辖区2001-2010年间各类法定传染病疫情报告资料进行统计学分析。结果 10年来共报告法定传染病31种93 566例,其中甲类1种7例,乙类22种49289例,丙类8种44 270例。10年间传染病构成比主要以肠道传染病和呼吸道传染病为主。虫媒和自然疫源性疾病构成比波动较小。结论由于本辖区人口构成比较复杂,流动人口较多,规范性预防接种难以实现,经常有输入性传染病病例的发生,导致传染病防控难度加大。需要政府加大对传染病防控机构人力和财力的投入。     

Cemento-ossifying fibroma of the maxilla

Abstract

Despite the fact that cemento-ossifying fibromas of the maxilla may be quite large and locally aggressive, en-bloc excision is achieved by gentle blunt dissection, with the whole tumour mass peeled out from the adjacent structures. Until recently different fibro-osseous tumours that contained cementum were classified together as “cementomas”. In 1992 The World Health Organization adopted a new classification that included these fibromas as benign osseous tumours. While such tumours of the mandible are common, those of the maxilla are rare. They are growth products of periodontal membrane remnant. The triggering mechanism in the formation of cementum outside the periodontal membrane remains unclear. We present a 35-year-old woman who had a giant expanding lobular mass in the right maxilla of 5 years duration. She had visual disturbances and nasal obstruction, and was treated successfully by surgical en-bloc resection of the tumour through an infraorbital transverse incision. The differential diagnosis included fibrous dysplasia, osteoid osteoma, osteoblastoma, chronic sclerosing osteomyelitis, ameloblastoma, squamous cell carcinoma of the maxillary sinus, calcifying epithelial odontogenic tumour (Pindborg tumour) and calcifying odontogenic cyst (Gorlin cyst). Histopathological examination confirmed a cemento-ossifying fibroma.     

Dermal pleomorphic liposarcoma resembling pleomorphic fibroma: report of a case and review of the literature

Pleomorphic liposarcoma (PLPS) is a rare, high‐grade sarcoma defined by the presence of pleomorphic lipoblasts. Constituting 5% of all liposarcomas, PLPS usually arises in deep soft tissues of the extremities, with rare occurrences in the dermis and subcutis. We describe a unique case of an 85‐year‐old Caucasian gentleman with a 1 year history of a pedunculated, pink, non‐tender papule on the dorsum of his left arm, measuring 1.0 cm in maximum dimension. Biopsy revealed a dermal collection of atypical epithelioid and spindle cells superimposed on a sclerotic background, resembling a pleomorphic fibroma on low power. On high power, a central focus of discrete adipocytic differentiation with pleomorphic lipoblasts was present. Tumor cells were positive for S‐100 and negative for desmin, actin, CD68, keratin, MART‐1 and CD34. Clinicopathologic findings were consistent with PLPS and the diagnosis was made. PLPS is rarely localized to the dermis and one with low power features resembling a pleomorphic fibroma has not been previously described in the literature.     

Behavioural phenotype of a patient with a de novo 1.2 Mb chromosome 4q25 microdeletion

A female patient, 20 years of age, is reported with a history characterized by developmental and psychomotor delay, and during grammar-school period increasing learning problems, ritualistic behaviours and social withdrawal. Subsequently, challenging and autistic-like behaviours became prominent. The patient showed mild facial dysmorphisms, long thin fingers with bilateral mild short V metacarpals, and hyperlaxity of the joints. Neuropsychiatric examination disclosed obsessive, ritualistic behaviours and vague ideas of reference. Neuropsychological assessment demonstrated mild intellectual disability, mental inflexibility and incongruent affect. MRI-scanning of the brain showed no relevant abnormalities. Genome wide SNP array analysis revealed a 1.2 Mb de novo interstitial microdeletion in 4q25 comprising 11 genes, that was considered to be causative for the developmental delay, perseverative cognitive phenotype and dysmorphisms.To the authors knowledge, this is the first report of a de novo 4q25 microdeletion that presents with a specific behavioural phenotype.     

Whipple disease: a 15-year retrospective study on 36 patients with positive polymerase chain reaction for Tropheryma whipplei

Our institution has performed microbiological diagnosis of Tropheryma whipplei since 2001, initially with a PCR targeting 16S rRNA before the development of a quantitative PCR in 2012. Here we report the clinical characteristics of a cohort of patients suffering from Whipple disease (WD) and evaluate the impact of these molecular techniques. Patients with a positive PCR for T. whipplei between 2001 and 2016 were retrospectively collected from microbiological databases. Two infectious diseases specialists reviewed their medical records and classified them as definite WD, probable WD or carriage of T. whipplei without disease. A total of 1153 samples were tested for T. whipplei; 76 samples taken from 36 patients were positive. Fifteen were considered as presenting a definite WD, seven as a probable WD and 14 as carriers. Median age was 56.4 years (extremes, 6.6–76.1). Median time from symptoms to diagnosis was 3 years (2.5 months to 13.3 years). About 60% were immunosuppressed. The most frequent clinical presentations were joint pain (16/22), weight loss (15/22) and/or digestive tract disorder (15/22); 41% had neurological manifestations, 32% pulmonary involvement and 32% lymphadenopathies. Bacterial load in faeces or saliva were 88 425 copies/mL (IQR 6175-292 725) in definite and probable WD and 311 copies/mL (IQR 253–2090) in carriers, respectively. We observed a 90% PPV above 32 200 copies/mL in faeces. WD is a chronic multisystemic disease with frequent pulmonary involvement. Underlying immunodeficiency is commonly observed leading to more complex clinical presentation. Positive T. whipplei PCR in both stool and saliva has a high positive predictive value. Moreover, patients with WD present higher bacterial load in faeces with a threshold of >32 200 copies/mL predicting ongoing infection.     

512例胆管细胞癌的临床及预后特点

目的： 胆管细胞癌(cholangiocarcinoma，CCA)恶性程度较高，总体预后较差，初诊时分期较晚，治疗手 段有效率欠佳。本研究探索影响CCA发生、发展的临床特点及预后相关因素，以期为CCA的早期诊断及临床治疗提 供潜在的手段。方法： 回顾性分析中南大学湘雅二医院2002 年以来经病理确诊且临床资料完善的512 例CCA患者的 病历资料。利用Kaplan-Meier 法绘制生存曲线、log-rank 进行单因素分析，多元Cox回归法对有意义的变量进行多因 素分析。结果： CCA发病率≤60 岁者高于>60 岁者(61.13% vs 38.87%)，男性略高于女性(52.54% vs 47.46%)，糖类抗 原19-9(carbohydrate antigen 19-9，CA19-9)≥35 μg/L 者占66.21%，病理分期III 和IV 期患者居多(分别占49.22%和 17.58%)。单因素分析发现ALB，ALP，CA19-9 等因素与预后相关，多因素Cox 生存分析发现ALP，CA19-9，肿瘤 最大直径等是CCA预后独立影响因素。结论：CCA发病率≤60 岁人群较高，初诊时分期较晚，CA19-9 是较为敏感的 实验室指标。ALP，CA19-9，肿瘤最大直径，合并子瘤，肝硬化及TNM分期是影响CCA预后的独立因素。     

Ten‐year retrospective clinicohistological study of cutaneous lupus erythematosus in Korea

An understanding of the differences in clinical manifestations and laboratory abnormalities between subtypes of cutaneous lupus erythematosus (CLE) is still lacking. The purpose of this study was to analyze demographic, clinical and histological features of CLE according to three main presentation subsets: acute (ACLE), subacute (SCLE) and chronic (CCLE). A 10‐year retrospective analysis was performed on data from patients who were diagnosed with CLE between March 2005 and September 2015 in a Korean tertiary referral dermatology clinic. We compared demographic data and clinical and histological findings between three different CLE groups. An overall sample of 220 patients with CLE consisted of 67 patients with ACLE, 25 patients with SCLE and 135 patients with CCLE. Patients with CCLE regardless of systemic lupus erythematosus (SLE) presence had lower prevalence of anemia, urinary abnormalities and elevated erythrocyte sedimentation rate. Furthermore, CCLE patients who only had skin lesions showed lower female predominance, lower extracutaneous manifestation, fewer laboratory and immunological abnormalities including low antinuclear antibody titers and the lowest positivity for C3, C4 and anti‐dsDNA, anti‐Ro, anti‐Sm and anti‐RNP antibodies, and more prominent perieccrine inflammation and dermal fibrosis in histological findings. Considering distinct cutaneous manifestations of LE, a comprehensive awareness of each CLE subtype is important for achieving a favorable prognosis through appropriate diagnosis and management. This study provides comparative clinical and histological profiles of patients with different CLE subtypes in Korea.     

Inflammatory features of melasma lesions in Asian skin

Melasma is triggered by various factors including ultraviolet radiation and estrogen; however, its pathogenesis is unclear. To investigate the inflammatory features of melasma lesions as triggers for this disorder, 197 women with melasma who attended Asan Medical Center and Kangskin Clinic, Seoul, from June 2011 to October 2011 completed a questionnaire concerning triggering or aggravating factors. These cases were divided into “non‐inflammatory” and “inflammatory” groups. Skin biopsies and immunostaining for CD68, CD117, and leukocyte common antigen (LCA) were performed in the lesional and peri‐lesional skin of ten cases in the non‐inflammatory group and nine cases in the inflammatory group. Among the 197 subjects (mean age, 41.5 years; mean age of melasma onset, 33.8 years), 50 patients (25.4%) were categorized into the inflammatory group. This group comprised cases that had inflammatory symptoms and events that triggered the melasma lesions. The lesional dermis contained more CD68⁺ melanophages, CD117⁺ mast cells, and LCA⁺ leukocytes in the inflammatory group than in the non‐inflammatory group. Inflammatory clinical features and an increased number of inflammatory cells in the lesion may be involved in the development of melasma in Asian skin.