人类颞骨火棉胶切片中线粒体DNA的扩增及重组测序

目的应用分子生物学技术建立人类颞骨火棉胶切片中的ＤＮＡ分析方法。方法采用多聚酶链反应（ｐｏｌｙｍｅｒａｓｅｃｈａｉｎｒｅａｃｔｉｏｎ，ＰＣＲ）配合不同的引物、扩增方式及酶切技术检测长期保存的７例（９侧）颞骨火棉胶切片中微量线粒体ＤＮＡ的片段缺失及点突变，ｐＧＥＭＴ载体进行扩增片段的重组与克隆。结果９侧颞骨ＤＮＡ提取液中均可见正常的１３５ｂｐ扩增片段，巢式ＰＣＲ检出其中２例生前患老年聋者（各查１侧）有线粒体ＤＮＡ大片段缺失，测序结果证实扩增的准确性。结论分子生物学技术在颞骨切片研究中的应用对于提高其回顾性研究水平有重要意义，目前可在耳科疾病的研究中发现相关的基因突变或病原体。     

上颌骨掀翻复位术在良性涉颅鼻腔鼻窦肿瘤中的应用

目的探讨涉颅良性鼻腔、鼻窦肿瘤的手术治疗方法。方法报道６例鼻腔、鼻窦良性肿瘤侵及颅底的病例，其中骨化纤维瘤３例，骨瘤１例，骨软骨瘤１例，骨巨细胞瘤（Ｉ～Ｉ级）１例，均采用１侧上颌骨掀翻复位术及颅面联合进路手术切除肿瘤，全部切除肿瘤４例，近次全切除２例。结果无手术并发症，术后随访２～３年，５例无异常，１例骨化纤维瘤术后２年复发。结论上颌骨掀翻术是一种较好的治疗涉颅良性鼻腔、鼻窦肿瘤的手术方法。     

Mastoid size and otitis media with effusion: Question of correlation

Objectives/Hypothesis: To determine the incidence of otitis media (OME) with effusion on histologic examination in temporal bones with mastoid cavities reduced by the fenestration procedure for otosclerosis. Study Design: Temporal bone histologic study. Methods: Light-microscopic examination of serially sectioned temporal bones. Results: The incidence of otitis media with effusion in temporal bones with prior fenestration operation was not any more frequent than the control group of temporal bones with surgically unaltered mastoid cavity. Conclusions: There is no increased incidence of otitis media with effusion in temporal bones with prior fenestration operation.     

Effect of central precocious puberty and gonadotropin-releasing hormone analogue treatment on peak bone mass and final height in females

To evaluate the effect of central precocious puberty (CPP) and its treatment with gonadotropin-releasing hormone (GnRH) analogues on final height and peak bone mass (PBM), we measured lumbar bone mineral density (BMD) in 23 girls at final height. Patients were distributed in two groups. Group 1: 14 patients with progressive CPP were treated with GnRH analogues; seven patients received buserelin (1600 μg/daily), subsequently switched to depot triptorelin (60 μg/kg/26–28 days); seven patients were treated with depot triptorelin (60 μg/kg/26–28 days); mean age of treatment was 6.2 years (range 2.7–7.8 years); the treatment was discontinued at the mean age of 10.1 years (range 8.7–11.3 years); final height was reached at the mean age 13.4 years (range 12.0–14.9 years). Group 2: 9 patients (mean age 6.5 years, range 4.8–7.7 years) with a slowly progressing variant of CPP were followed without treatment; final height was reached at the mean␣age␣13.6 years (range 12.5–14.8 years). Lumbar BMD (L₂-L₄ by dual energy X-ray␣absorptiometry) was measured in all patients at final height. In group 1, final height␣(158.9 ± 5.4 cm) was significantly greater than the pre-treatment predicted height (153.5 ± 7.2 cm, P < 0.001), but significantly lower than mid-parental height (163.2 ± 6.2 cm, P < 0.005). Subdividing the girls of group 1 according to the bone age at discontinuation of therapy (i.e. ≤11.5 years, n = 5, or ≥12.0 years, n = 9), the former patients had a final height significantly higher than the latter (163.7 ± 3.9 cm vs 156.5 ± 4.6 cm, P < 0.02). In group 2, final height (161.8 ± 4.6 cm) was similar to the pre-treatment predicted height (163.1 ± 6.2 cm, P = NS) and was not significantly different from mid-parental height (161.0 ± 5.9 cm). BMD values (group 1: 1.11 ± 0.14 g/cm², group 2: 1.22 ± 0.08 g/cm²) were not significantly different from those of a control group (1.18 ± 0.10 g/cm²; n = 20, age 16.3–20.5 years) and the patients' mothers (group 1: 1.16 ± 0.07 g/cm², n = 11, age 32.9–45.1 years; group 2: 1.20 ± 0.08 g/cm², n = 7, age 33.5–46.5 years). In group 1, the girls who stopped therapy at a bone age ≤11.5 years had significantly higher BMD (1.22 ± 0.10 g/cm²) compared to those who discontinued therapy at a bone age ≥12.0 years (1.04 ± 0.12 g/cm², P < 0.05). Conclusion In girls with progressive CPP, long-term treatment with GnRH analogues improves final height. A subset of patients with CPP does not require treatment because good statural outcome (slowly progressing variant). In CPP, the abnormal onset of puberty and the long-term GnRH analogue treatment do not impair the achievement of PBM. In GnRH treated patients, the discontinuation of therapy at an appropriate bone age for pubertal onset may improve both final height and PBM. Received: 5 June 1997 / Accepted in revised form 21 November 1997     

Durable remission of locally advanced breast cancer with multimodality management

We treated 20 women with locally advanced breast cancer between January 1991 and September 1996, The treatment regimen included 4 cycles of intensive doxorubicin (30 mg/m2/ d on 3 consecutive days every 2 weeks with G-CSF support), followed by appropriate surgery, followed by high dose therapy with cyclophosphamide, carboplatin and thiotepa (STAMP V, CTCb). Of the 20 patients, seven presented with inflammatory breast cancer, three with Stage HIB, seven with stage IIIA, one with multifocal Stage IIB and two with Stage IV M1 (ipsilateral supraclavicular lymph node involvement) (including one who had an inflammatory primary) disease. Six patients had not undergone mastectomy at the time of entering the protocol. These six received the doxorubicin in a neoadjuvant fashion and were thus evaluable for tumor response. The remaining 14 received doxorubicin as adjuvant therapy prior to intensification and transplantation. All patients underwent local-regional radiation therapy and were placed on oral tamoxifen. Doxorubicin was well tolerated in this schedule with ali but three patients receiving all their cycles on schedule. Both BM and PBPC were easily collected after this regimen and, when reinfused, resulted in the prompt recovery of granulocytes (median 11 days to 500 absolute granulocyte count) and platelets (median 13 days to 20000 platelets). The six patients who received doxorubicin prior to mastectomy all had major clinical responses, but were found to have microscopic focii of breast cancer in the mastectomy specimens. The overall treatment was well tolerated with the exception of one treatment-related death (5%). The overall and relapse free survival are 70% and 58% respectively with a median follow-up of 40 months (range 12–74 months). When the Stage IV patients are censored, the relapse-free survival rate is 69%. In the bone marrow transplant phase of treatment, the major non-hematologic toxicities were stomatitis (70%) and anorexia requiring parental nutrition (75%).     

Cerebral venous sinus thrombosis: a clinical study of 23 cases

Objective To describe the etiologies, clinical features and diagnosis of cerebral venous sinus thrombosis.
Methods We reviewed the records of 23 patients admitted with a documented diagnosis of c erebral venous sinus thrombosis from 1991 through 1999 in the Beijing Tiantan Hospital.
Results Infection was the major condition associated with cerebral venous sinus thrombos is. Pseudotumor cerebri syndrome was the most common manifestation, while hemip legia, seizure and unconsciousness may occur alone or in association. The diagn ostic sensitivity of computed tomography (CT), magnetic resonance imaging (MRI) and digital subtract angiography (DSA) were 59%, 86% and 100%, respectively. Th e sensitivity of MRI with magnetic resonance angiography (MRA) reached 96%.
Conclusion As the clinical findings were found to be nonspecific, CT combined with DSA, or MRI with MRA turns out to be valuable for the early diagnosis of cerebral venous sinus thrombosis.     

骨巨细胞瘤AgNOR和DNA的定量研究

 采用AgNOR染色和流式细胞术分别对50例和30例骨巨细胞瘤（GCT）进行研究。AgNOR计数Ⅰ级7．49±1．57，Ⅱ级9．47±1．08，Ⅲ级10．67±0．37。Ⅰ级与Ⅱ级，Ⅱ级与Ⅲ级之间皆有显著性差异，Ⅱ级与Ⅲ级之间无显著性差异。FCM分析结果表明，DNA异倍体率在各级GCT之间有显著差异，各级GCT的S期百分比无显著性差异。整倍体和异倍体AgNOR计数有显著性差异。GCT复发浸润与倍体水平及AgNOR计数有关。     

Bone marrow metastasis in astrocytic gliomata

With the increasing survival time of many pediatric patients with malignancies, unexpected symptoms or signs require diligent search for rare complications or second cancers related to the disease or treatment. We recently encountered a patient with extensive glioblastoma multiforme who developed pancytopenia six months after completion of treatment with craniospinal radiation and chemotherapy with etoposide and cyclophosphamide. Bone marrow aspirate and biopsy confirmed bone marrow metastasis from the brain tumor. He showed good partial remission with chemotherapy with carmustine and cis-platinum as demonstrated by serial bone marrow aspirate for cytology and cytogenetics and enjoyed good quality of life for eight months. 14 other patients with astrocytic glioma, two of whom are children, are reported in the literature to have diffuse bone marrow metastasis. Therefore, in patients with malignant astrocytic tumor, bone marrow metastasis, though not common, should be considered when bone pain or cytopenias occur, especially when prolonged.     

骨嗜酸性肉芽肿临床病理分析

作者回顾性分析２６例骨嗜酸性肉芽肿的病理肉眼镜了改变，着重探讨其组织病理特点，指出本病性质属良性非肿瘤性病变，认为骨嗜酸性肉芽肿的病理分期有利于病理诊断及指导临床治疗，并提出病理活检在该病的诊断中有重要意义。     

脑疝复位天幕切开与常规手术治疗重型脑外伤脑疝的临床对照研究

目的　比较脑疝复位天幕裂孔切开与常规去骨瓣手术治疗重型脑外伤引起脑挫裂伤脑水肿、颅内血肿出现脑疝晚期患者的手术效果。方法　共收治重型脑外伤脑疝晚期患者5 6例,分为2组:脑疝复位组31例;常规手术组2 5例。患者术前GCS计分均为3～5分、双瞳孔散大或有不同程度散大,所有患者都经头颅CT扫描确定脑损伤情况。结果　患者术后存活者随访1a ,脑疝复位组:恢复良好/中残1 0例、重残/长期昏迷7例、死亡1 4例(45 %) ;常规手术组:恢复良好/中残1例、重残/长期昏迷3例、死亡2 1例(84 %) (P <0 .0 5 )。脑疝复位组术后大脑后动脉梗死、应激性溃疡、脑积水发生率明显低于常规手术组(P均<0 .0 5 )。结论　脑疝复位天幕裂孔切开治疗重型脑外伤引起脑挫裂伤脑水肿、颅内血肿出现脑疝晚期患者疗效明显优于常规去骨瓣手术。