不同类型卵巢过度刺激综合征对妊娠结局的影响

目的:分析卵巢过度刺激综合征(OHSS)发病早晚、病情轻重、病程长短对妊娠结局的影响。方法:对于行IVF-ET而发生中、重度OHSS的132例患者(早发型83例,晚发型49例;中度34例,重度98例;短病程103例,长病程29例;妊娠72例)临床资料进行回顾性分析。结果:①OHSS患者妊娠丢失与双胎、OHSS发病早晚、病情轻重、病程长短无显著性相关(P>0.05),但在晚发型妊娠丢失比率高于早发型、重度比率高于中度、病程长者比率高于病程短者。②早产与双胎有显著性相关(P<0.01),与OHSS发病早晚、病情轻重、病程长短无显著性相关(P>0.05)。③低体重儿与双胎有显著性相关(P<0.01),与OHSS发病早晚、病情轻重、病程长短无显著性相关(P>0.05)。结论:OHSS患者的流产比率与发病早晚、病情轻重、病程长短也无显著性相关,但在发病晚、病情重、病程长者比率略高;低体重儿、早产与双胎有显著性相关,与发病早晚、病情轻重、病程长短无相关性。     

Paget''s disease of the vulva: clinicopathologic study of type 1 cases treated at a single institution

The aim of this study was to evaluate clinicopathologic characteristics of primary cutaneous Paget's disease of the vulva. Between 1986 and 2005, 22 patients with primary cutaneous Paget's disease of the vulva (type 1) were treated at Tohoku University Hospital. Medical records were reviewed for pathologic diagnosis, patient age, associated neoplasms, type(s) of eczema, symptom duration, treatment, surgical procedures, recurrence, and length of follow-up. Patient age ranged from 51 to 85 years (median 71.5 years). Median duration of symptoms was 24 months (range 2-60 months). Type 1a (intraepithelial) Paget's disease accounted for 18 patients, with 3 type 1b (invasive) cases and 1 type 1c (intraepithelial disease with underlying adenocarcinoma) case. Mean length of follow-up was 53.7 months, and median follow-up was 49 months (range 6-199 months). Only two patients had an associated internal malignancy: T-cell leukemia and breast cancer. Mapping biopsy was performed in 14 of the 18 type 1a cases. All patients were free of disease at the surgical margins and are alive without recurrence. The four patients with type 1b or 1c disease had lymph node metastases. Two has died of disease, and two are alive with no recurrence. The rate of secondary malignancy seems to be low in primary cutaneous Paget's disease of the vulva. Mapping biopsy with careful examination of characteristic skin surface may be useful for surgery of type 1a cases. Inguinal lymphadenectomy is recommended in cases with question of invasion or known underlying adenocarcinoma.     

Experimental group B streptococcal infection in the rhesus monkey. I. Disease production in the neonate.

Group B streptococci (GBS) are responsible for serious infections of newborn infants. An experimental model for GBS infection was developed in the newborn rhesus monkey in order to obtain more information concerning the pathogenesis of such infections. A series of 29 newborn monkeys were inoculated with either type Ic or type III GBS or sterile broth. Fatal neonatal meningitis without associated pneumonia was produced consistently following intracerebral inoculation with either type Ic or type III; intracerebral inoculation with sterile broth produced no apparent disease. Variable disease production followed intravenous or intra-amniotic GBS inoculation, and clinical manifestations ranged from no apparent disease to fatal meningitis and pneumonia. This monkey model may be useful for further investigation of treatment and prevention of neonatal GBS infection.     

First-trimester fetal nuchal translucency and inherited metabolic disorders

OBJECTIVES: To assess the association between inherited metabolic disorders and nuchal translucency (NT) measurements. METHODS: The NT measurements obtained from 66 fetuses at high risk for metabolic diseases prior to chorionic villus sampling (CVS) were retrospectively analysed. RESULTS: NT was found to be within the normal range in all of the 13 affected fetuses, which included three with Gaucher disease, two with glycogenosis type II, two with mucopolysaccharidosis type I and six others with Krabbe disease, metachromatic leukodystrophy, mucopolysaccharidosis type II, Niemann-Pick A disease, Pelizaeus-Merzbacher disease and sialidosis, respectively. An increased nuchal thickness was found only in one fetus affected with trisomy 21 but not affected with mucopolysaccharidosis type II. CONCLUSION: NT appears to have a limited role in identifying affected fetuses in pregnancies at high risk for inherited metabolic disorders. NT may be normal in early pregnancy even for fetuses affected with conditions known to be associated with non-immune hydrops fetalis.     

Fetal presentation of Morquio disease type A.

A fetus with mucopolysaccharidosis type IV A (Morquio type A) is described. The family had one affected child exhibiting symptoms of classical Morquio A disease, and late in the subsequent pregnancy prenatal diagnosis was requested. At 23 weeks' gestation, moderate ascites was detected by detailed ultrasound scan and keratan sulphate was found in the amniotic fluid. The pregnancy was terminated by prostaglandin induction and the diagnosis of mucopolysaccharidosis type IV A was confirmed by demonstration of a deficiency of N-acetylgalactosamine-6-sulphate (GalNac-6-S) sulphatase in cultured amniotic cells and in post-mortem fibroblast cultures. The activities of beta-galactosidase and arylsulphatase A were normal, ruling out Morquio disease type B and multiple sulphatase deficiency. These results indicate that mucopolysaccharidosis IV A (a disease that predominantly affects the skeletal system) may produce ascites in the fetus to such an extent that it can be detected by ultrasound.     

Operative technique and results of subtotal splenectomy for Gaucher disease

Patients with type 1 (adult form) Gaucher disease complicated by massive splenomegaly and hypersplenism have previously been treated by total splenectomy. Subtotal splenectomy in Gaucher disease will retain splenic tissue which may protect the patient from the risk of overwhelming sepsis. Removal of at least 85 per cent of the splenic mass is necessary to eliminate hypersplenism and to decrease splenic size. The surgical technique and outcome of four patients undergoing subtotal splenectomy for massive splenomegaly due to type 1 Gaucher disease are discussed.     

Second-look laparotomy; factors affecting the results and the prognosis

OBJECTIVE: To define the factors which might effect the results of second-look laparotomy (SLL) and the prognosis of patients with negative SLL. METHODS: Fourteen (42.4%) of the 33 patients who underwent SLL for primary epithelial ovarian cancer had residual disease (positive SLL), while 19 (57.6%) of them had no evidence of disease (negative SLL); 13 (68.4%) of the 19 patients who were followed without any intervention, still had no evidence of disease, while 6 (31.6%) of them had clinical recurrences. The age of the patients, histopathological type, stage and grade of the cancers, type of chemotherapy and the effectiveness of the cytoreductive surgery were presumed as prognosticators and were compared in each group of patients. RESULTS: The age of the patients, histopathological type of the carcinomas and the type of combined chemotherapy were similar in patients with negative or positive SLL and those with recurrences or no evidence of disease during the follow-up after negative SLL (p>0.05). However, the groups of patients with negative SLL and those with no evidence of disease during the follow-up period had lower grade or stage of cancers and less frequent sub-optimal cytoreduction than the groups of patients with positive SLL and those with recurrences during the follow-up, respectively (p<0.05). CONCLUSION: SLL could be selectively performed in patients with high grade or high stage ovarian carcinoma or in those patients treated by sub-optimal cytoreduction.     

Pregnancy in Gaucher disease

An 18-year old woman with type I Gaucher disease and two uncomplicated pregnancies is described. Although she experienced one miscarriage and pregnancy was associated with exaggeration of the clinical symptoms, leading to the diagnosis of the disorder, both her 2nd and 3rd pregnancies were uneventful and deterioration of her clinical situation was not observed. The issue of criteria for risk assessment in pregnancy of type I Gaucher disease patients is addressed.     

Validity of a rheumatic disease diagnosis in the Medical Birth Registry of Norway

BACKGROUND: In our population-based study of pregnancy outcome in women with rheumatic disease we based our assessment on the Medical Birth Registry of Norway (MBRN). We evaluated the MBRN as a source of data for such epidemiologic research by assessing the validity of a diagnosis of rheumatic disease in the MBRN against a gold standard. The validity may also be interpreted as a quality indicator, reflecting an obstetrician's attention to rheumatic diseases in pregnancy. METHODS: Using the mother's national identification number the MBRN was linked with local hospital databases (gold standard), which contained data of mothers with rheumatic disease. The sensitivity of the MBRN was calculated as the proportion of all cases registered locally with a diagnosis of rheumatic disease notified to the MBRN. The correctness of type differentiation was calculated as the proportion of all cases notified to the MBRN that was correct with respect to the type of rheumatic disease. RESULTS: Among 169 mothers, 149 had a diagnosis in the MBRN, representing a sensitivity of 88.2%. Altogether, 97.3% of the diagnoses (145/149) were correct with respect to the type of rheumatic disease. CONCLUSION: Taken in to consideration the limitations of the study, namely the small numbers studied, we assume a rather high validity of rheumatic disease diagnoses in the MBRN, probably reflecting a high level of attention in the obstetric care for these patients.     

The influence of cytoreductive surgery on recurrence-free interval and survival in small-volume stage III epithelial ovarian cancer: a Gynecologic Oncology Group study.

Gynecologic Oncology Group Protocol 52, a randomized trial of cisplatin and cyclophosphamide with or without doxorubicin in "optimal" Stage III epithelial ovarian cancer, failed to demonstrate a significant difference in the outcome in 349 evaluable patients. Additional review of the records was carried out to determine the influence of cytoreductive surgery on survival. Since eligibility for the study was the presence of residual cancer of 1 cm or less, the influence of cytoreductive surgery could be evaluated by comparing outcome in patients presenting with large-volume extrapelvic disease, but who were cytoreduced to small-volume disease. Factors evaluated were age, cell type, grade, size, and location of disease at exploration, size, and location of residual disease after cytoreduction, number of residual nodules, ascites, type of surgery, blood loss, and hospital days. Univariate analysis revealed that age, size of residual disease, mucinous or clear cell histologic type, histologic grade, and number of residual lesions were significant prognostic factors. By univariate analysis patients found to have extrapelvic disease of 1 cm or less had a better recurrence-free interval and survival than those patients with large-volume disease who were cytoreduced to disease of 1 cm or less. Multivariate analysis revealed that older age, histologic grades 2 and 3, and 20 or more residual lesions were unfavorable. The volume of initial extrapelvic disease remained significant when gross disease was present in the omentum and in other extrapelvic sites. This study failed to prove the hypothesis that initial cytoreductive surgery would allow a patient presenting with large-volume ovarian cancer to have the same chance for survival as a patient found to have small-volume disease. Factors other than cytoreductive surgery are important in predicting survival.     

Latent polyglandular autoimmune syndrome type 2 case diagnosed during a shock manifestation

There are many types of polyglandular autoimmune syndrome (PAS). PAS type 2 is the most common type among adults. For PAS type 2 (PAS-2) diagnosis, detection of Addison's disease with autoimmune thyroid disease and/or type 1 diabetes mellitus are required. Premature ovarian insufficiency, pernicious anemia, vitiligo, alopecia, myasthenia gravis, celiac disease and autoimmune diabetes insipidus may be comorbidities of this condition. Contrary to the common belief, latent PAS is more common than the manifest forms. Here, we present a PAS-2 case diagnosed via adrenal crisis. At the time of diagnosis, the case was observed to have thyroid, adrenal and ovarian involvement. Therefore, PAS-2 and possible immunologic disorders were discussed.     

1例新生儿先天性H型气管食管瘘诊断逐渐明确的过程

气管食管瘘系由于先天性胚胎发育异常形成气管与食管间由瘘道相连通的一种疾病。新生儿可出现进食呛咳和反流。本例为V型:占0.5%～6.5%,食管通畅,但有斜行瘘管向前上方与气管相通,又称H型。由于H型用橡皮导管插入食管时不会遇阻,所以本病诊断困难。     

Two pathogenetic types of endometrial carcinoma

The author presents a hypothesis that the complex of endocrine and metabolic disturbances arising long before the development of endometrial carcinoma determines the biological peculiarities of the tumor, its clinical course, and the prognosis of the disease. On the basis of a prospective study of 366 patients with endometrial carcinoma, the author postulates that there are two different pathogenetic types of endometrial carcinoma. The first pathogenetic type of the disease arises in women with obesity, hyperlipidemia, and signs of hyperestrogenism: anovulatory uterine bleeding, infertility, late onset of the menopause, and hyperplasia of the stroma of the ovaries and endometrium. The second pathogenetic type of the disease arises in women who have no signs stated above or these signs are not clearly defined. The frequency of the first pathogenetic type in the studied group of women was 65%, whereas the frequency of the second type was 35%. The peculiarities outlined above which are characteristic of the first pathogenetic type of the disease determine the development of highly and moderately differentiated tumors (82.3% G1 and G2), superficial invasion of the myometrium (69.4%), high sensitivity to progestogens (80.2%), and favorable prognosis (85.6% 5-year survival rate). In patients who have the second pathogenetic type of endometrial cancer when endocrine and metabolic disturbances are absent or occult, poorly differentiated tumors arise (62.5% G3), a tendency to deep invasion of tumor into the myometrium is observed (65.7%); high frequency of metastatic spread into the pelvic lymph nodes (27.8%); decrease of sensitivity to progestogens (42.5%); and doubtful prognosis (58.8% 5-year survival rate) are noted.     

Congenital ichthyosis in severe type II Gaucher disease with a homozygous null mutation

This paper describes a neonate with type II Gaucher disease. The phenotype was unusually severe with congenital ichthyosis, hepatosplenomegaly, muscular hypotonia, myoclonus and respiratory failure. Electron microscopy of the skin revealed lamellar body contents in the stratum corneum interstices, appearances considered to be typical of type II Gaucher disease. The baby died from respiratory failure 1 month postpartum having made no neurological progress. Molecular analysis identified a previously not reported homozygous null mutation, c.1505G→A of the β-glucocerebrosidase gene.     

Congenital dilatation of the bile duct in 100 instances and its relationship with anomalous junction.

Congenital dilatation of the bile duct (CDBD) or choledochal cyst has been demonstrated to be associated with an anomalous junction of the pancreaticobiliary ductal system. Multifarious clinical signs and symptoms of CDBD have been shown to be closely related with the presence of this anomalous junction. In the present study, 100 instances of CDBD treated surgically at our institutions during a 30 year period were classified into two types according to the morphologic features of dilatation of the bile duct; there were 77 instances of the cystic type and 23 of the cylindric type. Morphologic features of the lesion, clinical signs and symptoms and laboratory findings in these 100 instances were clinically analyzed. In almost all of the patients who were less than one year of age, the disease was of the cystic type and patients presented with either a palpable mass or jaundice as the main symptom. In patients more than one year of age, the disease was of either the cystic or cylindric type. A history of episodes of characteristic abdominal pain accompanied by elevated levels of serum amylase was present in 70 of the patients with the cystic type of disease and in all of the patients with the cylindric type. Histologic sections from the patients showed glandular formation with chronic inflammation, possibly a result of refluxed activated pancreatic juice; in contrast, histologic sections from the remaining patients of all ages showed only thickening of the fibrous layer. Thus, such variable morphologic features and clinical signs and symptoms in CDBD are highly dependent on two factors--the age at onset and the reflux of pancreatic juice into the bile duct through the common channel.     

Sexually transmitted papillomaviral infections. I. The anatomic distribution and pathologic grade of neoplastic lesions associated with different viral types

Multiple colposcopic biopsy specimens were collected from 160 women, with sampling of principal cervical and vulvar lesions as well as secondary areas of either minor acetowhitening or normal epithelium. Papillomaviral deoxyribonucleic acid was detected by Southern blot hybridization in 197 (90%) of the 218 principal biopsy specimens and 93 (46%) of 198 secondary biopsy specimens. Although different papillomaviruses were found at different sites in 31 women, only six of 416 specimens contained multiple types within the same sample. Specific viral types were associated with specific disease patterns. Only one of 80 type 6 or 11 infections had a diagnosis greater than cervical intraepithelial neoplasia, grade 2. In contrast, 42 of 48 (90%) biopsy specimens of cervical intraepithelial neoplasia, grade 3, or invasive cancer contained type 16, 18, or 31. Nonetheless, 12 of 124 (10%) cases of condyloma and cervical intraepithelial neoplasia, grade 1, were associated with types 16, 18, and 31 infections. Of 58 women with multicentric disease, 46 had positive hybridizations for both cervical and vulvar lesions (32 showing the same type in both samples and 14 showing different viruses). Differing patterns of papillomavirus-induced disease arise partly from the predilection of specific viral types for certain anatomic sites and partly through variations in host response. Detection of viral deoxyribonucleic acid in 46% of the secondary biopsy specimens suggests that disease expression may represent focal breakdown of host surveillance within a field of latent papillomaviral infection.     

普通型外阴上皮内瘤变19例临床病理学研究

目的 探讨按照国际外阴疾病学会(ISSVD)2004年新分类标准对原外阴上皮内瘤变(VIN)Ⅱ级及以上级别以及鲍温病、鲍温样丘疹病的病理切片进行重新判读的结果及其手术治疗效果.方法 调阅北京协和医院1999年至2006年入院手术的全部VIN Ⅱ及以上级别患者、鲍温病及鲍温样丘疹病患者共19例的手术病理切片,请病理医师根据ISSVD 2004新分类标准重新阅片分类,并总结其临床随访结果.结果根据ISSVD 2004分类标准,所有19例患者的病理诊断均符合普通型VIN.进一步区分亚型,惟一1例鲍温病患者的两处病灶中,1处符合疣型VIN,另1处符合基底细胞型VIN,其余患者的病灶均符合疣型VIN.患者初次手术切除的治愈率为89%(17/19);术后随访中有2例出现复发,但再次手术治疗效果好,复发病灶的病理均符合疣型VIN.结论原VIN Ⅱ及以上级别病变以及鲍温病、鲍温样丘疹病按ISSVD 2004分类标准以普通型VIN(疣型)为主,新的分类标准使VIN的病理诊断明显简化.手术切除治疗普通型VIN效果满意,但术后密切随访十分必要.     

小儿先天性心脏病体外循环术后毛细血管渗漏综合征的影响因素

目的探讨小儿先天性心脏病体外循环术后发生毛细血管渗漏综合征(capillary leak syndrome，CLS)的相关影响因素,为预防和控制CLS的发生提供依据。 方法收集2004 01—2005 12重庆医科大学附属儿童医院310例先天性心脏病体外循环(CPB)术后患儿的临床资料，对可能引起CLS发生的因素进行单因素分析和多元Logistic逐步回归分析，确定出与CLS发病相关的独立影响因素。 结果体外循环术后CLS的发生率为16.78%，病死率为19.23%。多元Logistic回归分析显示体外循环时间、心脏畸形的种类(单纯型/复杂型)、CPB术中最低温度、年龄是CLS发生的独立影响因素。 结论患儿的体外循环时间、心脏畸形的种类、术中最低温度、年龄是CLS发生的独立影响因素。     

44例围产儿肺透明膜病的临床和病理研究

目的探讨围产儿肺透明膜病的临床意义、发病机理及其与缺氧缺血性脑病的关系。方法在分析常规;临床和病理资料的基础上,用尸肺灌洗液进行表面活性物质测定。结果病理检查显示肺组织广泛不张及透明膜形成,肺泡Ⅱ型上皮细胞受损及呼吸膜断裂。88％的患儿继发缺血性脑病。结论肺发育不成熟,肺泡Ⅱ型上皮细胞受损,肺表面活性物质缺乏,呼吸膜断裂是本病的主要原因。     

A comparison of adenocarcinoma and squamous cell carcinoma of the cervix

A comparison and survival analysis of 203 patients (21%) with adenocarcinoma and 756 (79%) with squamous cell cancer of the cervix for the time period 1970-1985 is reported. The mean number of new cases of adenocarcinoma (N = 12) remained the same, while squamous cell cases decreased from 57 to 32. The clinical features were compared; oral contraceptive usage, node status, diabetes, and symptoms of bleeding were not related to cell type. Nulliparity was more frequent in patients with adenocarcinoma, whereas obesity and smoking were more frequent in patients with squamous cell cancer. Survival in stage I was significantly influenced by the cell type. Patients with stage I squamous cell disease had a 90% 5-year survival, compared with 60% for adenocarcinoma (P less than .0001). Other features that influenced survival included node status (P = .001), poor differentiation of tumor histology (P = .001), diabetes (P = .001), and Papanicolaou smear interval (P = .001). Patients undergoing radical hysterectomy were analyzed separately, and adenocarcinoma cell type significantly influenced survival (P = .0008). Patients with stage II squamous cell disease had a 62% survival, compared with 47% for adenocarcinoma (P = .01); patients with stage III squamous cell disease had a 36% survival, compared with 8% for adenocarcinoma (P = .002). The percentage of adenocarcinoma has increased because of the decreased number of patients with squamous cell disease. Stage-for-stage survival is significantly decreased for patients with adenocarcinoma as opposed to squamous cell disease.