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1.
A case of a rare form of Sanfilippo disease, mucopolysaccharidosis type III D is presented. The cause of the disease is a deficit of N-acetylglycosamine-6-sulfate sulfatase. Differences in clinical course and symptoms with type A and B Sanfilippo disease are shown (later presentation of symptoms, milder course, lack of distinct psychomotor regression and differences in characteristic phenotypic traits, such as facial features, joint contracture, tall height). It is suggested that type III D mucopolysaccharidosis be taken into account in the differentiation of mental retardation syndromes with hyperactivity.  相似文献   

2.
We have analyzed 60 low-grade cervical squamous intraepithelial lesions for low- and high-risk human papillomaviruses (HPVs) and for numerical abnormalities of chromosomes 1, 3, 11, 17, and 18 and the X chromosome. Eleven of 33 lesions infected with high-risk HPVs (HPV 16, 18, 30, 31, 33, 35, 39, 45, 51, 52, 56, 58, and 66) but none of 24 lesions infected with low-risk HPVs (HPV 6, 11, 42, 43, and 44) and none of 15 normal cervices showed basal cell tetrasomy of all six chromosomes in the HPV-infected areas. These changes were not HPV type specific and were not present in all lesions infected with the same HPV type. The presence of basal cell tetrasomy in lesions infected with high- but not low-risk HPVs suggests that induction of chromosome instability may be one mechanism underlying the biological differences between these viral types.  相似文献   

3.
BACKGROUND: We performed a cost-benefit analysis of a protocol for studying patients with squamous intraepithelial lesions (SIL) on Papanicolaou smears to determine whether it compared favorably with resources spent on other health programs for screening and treatment. METHODS: During a 3-year period, 424 patients with dysplastic Papanicolaou smears were examined, studied by biopsy, and treated. We calculated costs based on a model protocol and derived a cost per year of life saved for preventing death from invasive cervical carcinoma. A sensitivity analysis was performed on selected assumptions of the analysis. RESULTS: The marginal, or incremental, cost of colposcopic evaluation and treatment of Papanicolaou smears with low-grade SIL, high-grade SIL (moderate), and high-grade SIL (severe), depending on assumptions, ranged from $406 to $5746, $160 to $2263, and $85 to $1197 per year of life saved, respectively. Depending on the assumption of the rate of Papanicolaou smears with SIL in the screened population being 1.8%, 5.1%, or 11.5%, the estimated total cost of screening and treating the referral base was $1.3 million, $538,126, and $307,037, respectively. This results in the average cost per year of life saved to screen and treat low-grade SIL, high-grade SIL (moderate), and high-grade SIL (severe) to be $1105 to $68,909, $375 to $21,673, and $177 to $8831, respectively. CONCLUSIONS: Both marginal cost and average screening costs of evaluating and treating abnormal Papanicolaou smears by the protocol described in this article compare favorably with costs per year of life saved for other health care screening and treatment strategies for many assumptions. The marginal cost to perform colposcopy on patients with a Papanicolaou smear with low-grade SIL is so low that it is relatively a very effective strategy.  相似文献   

4.
OBJECTIVE: To analyze follow-up data on atypical squamous cells of undetermined significance (ASCUS) based on morphologic characteristics. STUDY DESIGN: Five years of follow-up was obtained on a cohort of 437 consecutive patients from 1986 who had initial diagnoses of ASCUS, with a further categorization of type based on the maturity of the atypical cells. All such categorizations were made on the basis of specific cytologic criteria. Follow-up cytology and/or biopsy was available on 366 patients. RESULTS: During the follow-up period, 40 patients (13.5%) with ASCUS were diagnosed as having a squamous intraepithelial lesion (SIL); 15 (5%) were interpreted as high grade. When stratified by type of ASCUS based on cellular maturity, the following association/progression rates were noted: mature ASCUS, 10%; metaplastic ASCUS, 24%; and immature metaplastic ASCUS, 42%. In metaplastic and immature metaplastic ASCUS cases, high grade SIL accounted for 42% and 60% of those subsequently diagnosed with a squamous intraepithelial lesion, respectively, versus 30% for mature ASCUS. CONCLUSION: With well-defined and consistent criteria for the diagnosis of the variety of "squamous atypias," a stratification of risk of progression to or association with SIL can be made. When features of metaplasia and immature metaplasia are noted in the cells of ASCUS, patients were observed to be at increasingly greater risk for the detection of SIL; those cases were proportionately more likely to be high grade.  相似文献   

5.
The validity of human papillomavirus (HPV) detection using the hybrid capture assay (HCA) was compared with the polymerase chain reaction (PCR) in 38 patients with high-grade squamous intraepithelial lesions (HSILs). HCA and PCR showed 84% agreement for HPV detection. HCA missed a significant higher proportion of HSIL compared with PCR (21% vs. 5%; P = .04). Thus, the sensitivity of HCA should be increased before this test can be recommended for HSIL.  相似文献   

6.
Clinical studies report a low incidence of intestinal side effects with transdermally administered fentanyl (TTS-fentanyl) in comparison with oral morphine. To support these clinical data, analgesic and intestinal effects of both opioids were compared in rats. After subcutaneous injection, analgesia in the tail withdrawal reaction test was obtained at a peak effect dose of 0.032 mg/kg with fentanyl and 8.0 mg/kg with morphine. This analgesic dose exceeded the ED50 for inhibition of castor oil-induced diarrhea only slightly (1.1 x) in the case of fentanyl (0.028 mg/kg) but markedly (36 x) in the case of morphine (0.22 mg/kg). To reverse completely the antidiarrheal effect of equivalent analgesic doses of the opioids (their ED50S for analgesia lasting 2 hours), much more naloxone was required in the case of morphine (5.4 mg/kg) than in the case of fentanyl (0.19 mg/kg). After oral administration, the difference between both opioids was less pronounced. Analgesia was obtained at 0.85 mg/kg with fentanyl and 32 mg/kg with morphine. This analgesic dose only slightly (1.7 x) exceeded the antidiarrheal dose in the case of fentanyl (0.49 mg/kg) but significantly (6.2 x) in the case of morphine (5.2 mg/ kg). To reverse completely the antidiarrheal effect of equivalent analgesic oral doses of the opioids (their ED50S for analgesia lasting 2 hours), more naloxone was required in the case of morphine (11 mg/kg) than in the case of fentanyl (2.0 mg/kg). Rapid penetration of fentanyl into the brain is thought to be responsible for small dissociation between the analgesic and intestinal effect of this lipophilic opioid. The present data provide preclinical evidence to support the relatively low incidence of intestinal side effects observed clinically with the use of TTS-fentanyl in comparison with orally administered morphine.  相似文献   

7.
OBJECTIVE: The aim of this investigation was to study three groups of women presenting with possible HPV-infection with regard to HPV-types and cervical dysplasia. METHODS: Eighty women were included. Eighteen of them were present partners to men with condylomas, 20 had clinical vulvar HPV-lesions and 42 were referred due to an abnormal PAP-smear. Samples for HPV-analysis by PCR-technique were taken from the vulva, the portio and the cervical canal. A universal HPV-primer as well as specific primers for HPV 6/11, 16, 18, 31, and 33 were utilized. PAP-smears were taken as well as biopsies from cervix/portio. RESULTS: Seventy-eight percent had HPV-DNA identified. Sixty-seven percent of those with HPV 16 and/or 18 had dysplasia verified by histopathology and 50% of those with 31 and/or 33. Twenty of 21 women with dysplasia had HPV 16, 18, 31 and/or 33 identified. One woman with dysplasia was HPV-negative. Histopathologically verified CIN were diagnosed in all groups investigated. Women referred for suspicion of CIN significantly more often had HPV detected at the cervix/portio. HPV 6/11 was mostly found in women with condylomas. Apart from this the occurrence of the different HPV types were alike in the three groups. CONCLUSION: Infection with HPV is a process and the usefulness of different diagnostic methods seems to depend on when during the course of the disease they are used. HPV-findings in women with dysplasia were all associated with oncogenic virus-types. High-risk virus was often found simultaneously with low-risk virus indicating a covariation in the acquisition of the different HPV-types.  相似文献   

8.
Two hundred and twenty one women at high risk for HIV (intravenous drug users and/or those with infected partners) were investigated, through a self-filled questionnaire and gynaecological examination, to define the relationship between genital Human Papilloma Virus (HPV) infections, preneoplastic cervical intraepithelial lesions (CIN) and behavioural risk factors. In the 121 HIV positive women, 58 (47%) had HPV lesions at colposcopic and/or cytologic examination and, out of these 58, 23 (40%) had CIN 1, CIN 2 or CIN 3. Six out of the 16 cases with CIN 1 and CIN 2 (37%) followed-up showed a rapid progression of the lesion to CIN 3; in 3 women the interval was 6 months, in the other 3 about 12 months. Only 5 (7%) of the remaining 66 women without HPV lesions had a CIN lesion, with an obviously significant difference on comparison with HPV positive subjects. Sixty two women out of the 121 (52%) had a previous diagnosis of condylomata. In the 100 HIV negative women, 23 (23%) had HPV lesions and, among these 23, 6 (26%) had CIN 1, CIN 2 or CIN 3; 1 of them had rapid progression from CIN 1 to CIN 3 within a year. Only 5 (3%) without HPV infection showed any kind of CIN. 33 women out of 100 (33%) had a previous clinical history of condylomata. Our findings strongly suggest that HIV infection is associated with HPV lesions and that cervical cytological abnormalities develop in this situation. There is a need for short interval cytological and colposcopic follow-up for women at high risk of HIV infection.  相似文献   

9.
We report on three patients who presented with a cerebellar medulloblastoma at age 48, 53, and 59 years. Histopathology showed typical features of medulloblastoma, in one case with marked neuronal differentiation. In addition, all neoplasms contained focal accumulations of mature fat cells. Immunoreactivity of adipocytes for S-100 protein, neuron-specific enolase, synaptophysin, microtubule-associated protein-2, and glial fibrillary acidic protein and the lack of immunoreactivity to type IV collagen suggest lipomatous differentiation of neoplastic primitive neuroectodermal cells rather than an admixture of mesenchymal elements. Mitotic activity was low and the growth faction, as determined by the MIB-1 labeling index, was less than 5%. All patients are alive with a recurrence-free interval ranging from 3.5 to 12 years. These three patients and five similar previously reported cases all fit into the concept of the lipomatous medulloblastoma as a new clinicopathological entity characterized by (a) typical features of a cerebellar medulloblastoma with advanced neuronal differentiation, (b) areas of lipomatous differentiation, (c) low proliferative potential, (d) manifestation in adults (mean age, 50 years), and (e) apparent favorable clinical prognosis.  相似文献   

10.
The cytologic features of squamous cell carcinoma in situ with endocervical gland involvement have been described in cervical smears. We evaluated the presence of two types of cellular fragments in 43 cervical smears of high grade squamous intraepithelial lesions (HGSIL) to assess their ability to predict glandular involvement by HGSIL in subsequent cone biopsies. An endocervical brush was used to obtain all endocervical specimens. Of 16 cases without glandular involvement, fragments were present in 13 smears. Of 27 cases with glandular involvement, fragments were absent in 11 smears. No statistical association was identified between the presence of abnormal cellular fragments on cervical smears of HGSIL and endocervical gland involvement on cone biopsies.  相似文献   

11.
BACKGROUND: Squamous intraepithelial lesions (SILs) of the cervix are associated with human immunodeficiency virus (HIV) infection, but multiple risk factors must be considered in this context. The authors performed a cross-sectional study to assess the prevalence of and the factors associated with SILs and invasive cervical carcinoma (ICC). METHODS: In Abidjan, C?te d'Ivoire, women were recruited from three outpatient gynecology clinics and screened for both cervical disease and HIV infection. A CD4 cell count was performed for HIV-infected women. RESULTS: A total of 2198 women were included in the study. The prevalence of HIV infection was 21.7%. Of the 2170 women who underwent a cervical screening, 254 (11.7%) presented with a dysplasia or neoplasia: 7.6% had low grade SILs (LSILs), 3.3% had high grade SILs (HSILs), and 0.8% had ICCs. In multivariate analyses, factors associated with these lesions were as follows: for LSILs, HIV-1 seropositivity, age <24 years, parity >1, consultation for genital infection, and no use of oral contraception in the past; for HSILs, HIV-1 seropositivity, chewing tobacco use, low educational level, and parity >1; and for ICCs, age >33 years, parity >3, and illiteracy. In women infected with HIV-1, the prevalence of LSILs increased with a decrease in CD4 cell count, whereas this relation was not found among patients with HSILs. ICCs were linked to HIV-2 infection, but not to HIV-1 infection, in univariate analysis. CONCLUSIONS: In Africa, the prevalence of SILs is high. The factors associated with precancerous and cancerous lesions are different. Cancers in women infected with HIV-1 often may not reach the invasive stage. These findings could have implications for cervical screening programs in the future.  相似文献   

12.
13.
OBJECTIVE: To evaluate the long-term outcomes after treatment of cervical intraepithelial neoplasia (CIN) in women infected with the human immunodeficiency virus (HIV). METHODS: Human immunodeficiency virus-infected and HIV-negative women treated for CIN by ablation or excision were followed-up prospectively by cytology and colposcopy for periods of up to 73 months. RESULTS: Among 127 HIV-infected CIN patients, 62% developed recurrent CIN by 36 months after treatment, compared with 18% of the 193 HIV-negative CIN patients. Recurrence rates reached 87% in 41 HIV-infected women with CD4 counts less than 200 cells/mm3. Progression to higher-grade neoplasia, including one invasive cancer, occurred by 36 months in 25% of HIV-infected and 2% of HIV-negative women. After adjusting for age, CIN severity, and treatment type, predictors of recurrence included HIV infection (rate ratio 4.4), and, in HIV-positive women, low CD4 count (rate ratio 2.2). In patients treated by excision, predictors of recurrence included HIV infection (rate ratio 2.0) and residual CIN after treatment (rate ratio 2.7). After a second treatment,a second CIN recurrence developed in 14 of 33 HIV-infected and in one of 17 HIV-negative women. After a third treatment, three of six HIV-infected women developed a third recurrence. With long-term follow-up, 45% of treated HIV-infected CIN patients had chronic condylomatous changes in the cervix compared with 5% of HIV-negative women. CONCLUSION: In HIV-infected women, CIN may recur despite multiple treatments, and chronic condylomatous changes are common. Innovative therapies for controlling CIN in HIV-infected women are needed.  相似文献   

14.
Among micro-organisms infecting vagina whose dominant genus is GV anaerobic bacteria are often present. There are reports that GV infections of vagina and uterine cervix, apart from their well-known negative role in obstetrics practice can play a role in carcinogenic processes of uterine cervix. The aim of the study was to assess characteristic colposcopy images of cervix in women with Gardnerella vaginalis infection. The research was carried out on 1180 women hospitalised in the period of 14 months. Many observations lead to conclusions that pathognomic clinic feature of Gardnerella vaginalis infection of uterine cervix is visible in colposcope presence of clean, translucent mucus in external cervical os and opaque vaginal contents in the rear vaginal vault. High hydrogen ion concentration in vaginal contents (pH 6.0 and over) correlates with positive "fishy odour test" of this contents. Gardnerella vaginalis infection of vagina and uterine cervix concerns women with existing erosion-type changes of uterine cervix. Visible in colposcopic test restless and "spotted" images visible after Schiller test are pathognomic colposcopic features of Gardnerella vaginalis infection.  相似文献   

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17.
Performance on five behavioral tasks was assessed post-operatively in Macaca fascicularis monkeys prepared with bilateral lesions of the entorhinal cortex (E group). Three of the tasks were also readministered 9-14 months after surgery. Initial learning of the delayed nonmatching-to-sample (DNMS) task was impaired in the E animals relative to unoperated control monkeys. On the delay portion of DNMS, the performance of E animals was nearly at control levels at short delays (up to 60 sec) but was impaired at 10 min and 40 min retention intervals. On the retest of DNMS, the E animals performed normally at all retention intervals. The E animals were unimpaired on the four other memory tasks. Neuroanatomical studies revealed a significant transverse expansion of the terminal field of the perirhinal cortical projection in the CA1 region of the hippocampus. Compared to unlesioned, anatomical control monkeys, the transverse length of the perirhinal terminal field in CA1 increased approximately 70% in the E monkeys. Although this was a striking morphological alteration, it is not known whether the sprouting of this projection influenced the behavioral recovery. The results of these studies suggest that the entorhinal cortex may normally participate in the learning and performance of tasks that are dependent on the medial temporal lobe memory system. However, recovery of normal DNMS performance demonstrates that the entorhinal cortex is not, by itself, essential for learning and performance of such tasks.  相似文献   

18.
Despite the development and widespread implementation of Basic Life Support and Advanced Cardiac Life Support, the percentage of patients who survive in-hospital cardiac arrest has remained stable at approximately 15%. Although survival rates may approach 90% in coronary care units, survival rates plummet outside of these units. The lower survival rates for cardiac arrest that occur outside of the coronary care unit may relate to the time elapsed between the onset of ventricular fibrillation and first defibrillation. The advent of automated external defibrillators has made it possible to decrease the time elapsed before first defibrillation in non-critical care areas of the hospital. First responders need only recognize that the patient is unresponsive, apneic, and pulseless before attaching and activating the automated external defibrillator. Our research shows that, as part of Basic Life Support training, non-critical care nurses can learn to use the device and can retain the knowledge and skill over time. Establishing an in-hospital automated external defibrillator program requires commitment from administration, physicians, and nursing personnel. Critical care practitioners should be aware of this technology and the literature that supports its safety and effectiveness when used by non-critical care first responders. Critical care nurses are in a unique position to effect changes that will decrease the time between the onset of cardiac arrest and first defibrillation.  相似文献   

19.
To evaluate the biological significance of esophageal squamous cell carcinoma that is associated with contiguous intraepithelial carcinoma, we analyzed 95 patients with operated esophageal carcinoma. Of these 95 patients, eight had in situ carcinoma. Among 87 cases in which the tumo had invaded more deeply than the lamina propria, there were 42 cases (48.3%) of contiguous intraepithelial carcinoma associated with the main tumor. The biological characteristics (proliferative activity of cells, as revealed by immunostaining with the Ki-67 monoclonal antibody) of 45 tumors without contiguous intraepithelial carcinoma (group A) were compared with those of 42 tumors with contiguous intraepithelial carcinoma (group B). The more advanced was the main lesion, the lower was the incidence of contiguous intraepithelial carcinoma. The mean Ki-67 score of the main tumors in group A was 51.6% and that of the main tumors in group B was 45.9%. The mean Ki-67 score of the main tumors in group B was very similar to that of the contiguous intraepithelial carcinomas that were associated with the main tumors (44.4%, P = 0.682). Furthermore, the mean Ki-67 score of contiguous intraepithelial carcinomas associated with main tumors was very similar to that of carcinomas in situ (41.2%, P = 0.529). From our results, it is suggested that tumors with high proliferative activity may be assumed to grow rapidly and, as a result, the region of intraepithelial carcinoma may develop into an invasive tumor. By contrast, tumors with low proliferative activity may grow slowly and, in such cases, the carcinoma may remain in the epithelium around the invasive tumor.  相似文献   

20.
A working model for the histogenesis of carcinoma of the uterine cervix is summarized in Table I. The inclusion of squamous metaplasia in this chart does not imply that this reaction falls into the spectrum of cervical neoplasia or is necessarily an antecedent to neoplasia. It does simply imply that the carcinogenic event or events apparently occur in or involve an epithelium that is indistinguishable from squamous metaplasia. The chart intentionally implies that the lesions mentioned are not separate diseases but arbitrary points in the spectrum of cervical neoplasia. It must be emphasized that one stage does not necessarily progress to the next and that at any stage up to indisputable cancer the changes may regress, persist or progress. The careful evaluation of histologic material from the uterine cervix will permit the pathologist to exclude those epithelial abnormalities which are not a part of the spectrum of cervical neoplasia and allow him or her to place the patient with cervical neoplasia at the proper stage in the development of the process. With this information the clinician can then intelligently plan appropriate therapy.  相似文献   

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