Coats plus syndrome (cerebroretinal microangiopathy with calcifications and cysts‐1): A case report

We present a 6‐year‐old girl with skin hyperpigmentation, leukoplakia, and onychodystrophy, the classic mucocutaneous triad usually associated with dyskeratosis congenita. The patient also had premature graying of the hair, bone marrow failure, hepatitis, exudative retinopathy, osteopenia with multiple long bone fractures, and intracranial calcifications and brain cysts. Coats plus syndrome is a rare disease with a clinical and genetic overlap with dyskeratosis congenita. This disease is reviewed, with a focus on the pathogenesis of the genetic anomalies and its background as a telomere biology disorder.     

Effect of the menstrual cycle and oral contraceptives on aromatase and cyclooxygenase-2 expression in adenomyosis.

OBJECTIVES: To determine whether aromatase expression in the eutopic endometrium and adenomyotic foci is affected by previous use of oral contraceptives containing gestodene, and to determine whether changes in cyclooxygenase-2 (COX-2) expression occur in adenomyosis during the menstrual cycle. PATIENT AND METHODS: This was a retrospective cohort study carried out in paraffin-embedded endometrial tissue obtained from patients with a histological diagnosis of adenomyosis obtained during the proliferative (n = 25) and luteal (n = 10) phases of the menstrual cycle and following the use of continuous oral contraception with gestodene/ethinyl estradiol (n = 7). COX-2 and aromatase expression were measured in both eutopic endometrium and adenomyotic foci using immunohistochemical methods. RESULTS: Aromatase expression was detected in 80% of the endometrial slices by immunohistochemistry. In positive cases, aromatase was mainly detected in the stromal cells of the eutopic endometrium, whereas in the adenomyotic foci this expression was negative in the majority of the cases. Oral contraceptives containing gestodene, on the other hand, were effective in suppressing aromatase expression in both eutopic and ectopic endometrium. COX-2 expression was detected by immunohistochemistry in the glandular epithelium of both eutopic endometrium and adenomyotic foci and there were no significant changes in its intensity throughout the menstrual cycle. CONCLUSION: Aromatase expression in the eutopic endometrium and adenomyotic foci is suppressed by oral contraceptives containing gestodene. Increased aromatase activity may be responsible for the persistent COX-2 expression during the luteal phase.     

高效液相色谱法测定人血浆中阿莫西林浓度及药代动力学

高效液相色谱法测定人血浆中阿莫西林浓度及药代动力学谭力周继红罗楠袁倚盛（南京军区南京总医院中心仪器分析科，南京２１０００１）阿莫西林（ａｍｏｘｉｃｉｌｉｎ）为β内酰胺类抗生素，其抗菌谱广，口服受食物影响小，对大多数病人耐受性良好，因而在临床上得以广泛...     

Primary papillary serous cystadenocarcinoma of broad ligament

A rare case of an advanced primary broad ligament carcinoma is discussed, with a review of the literature regarding its incidence, presentation and management. This patient showed a complete response to adjuvant cisplatin-based chemotherapy following panhysterectomy and is presently without any evidence of disease, 15 months after completion of her treatment.     

Isolation of Malassezia spp. from cerumen of wild felids.

The objective of this study was to determine the presence of different species of the genus Malassezia in the healthy external auditory canal of wild felids maintained in captivity. One hundred and thirty-two adult animals (264 samples of cerumen), 77 males (58.3%) and 55 females (41.7%), were studied: large felids (55 animals) - 26 lions (Panthera leo), 13 tigers (Panthera tigris), 6 leopards (Panthera pardus), 6 jaguars (Panthera onca), 2 cheetahs (Acinonyx jubatus), 2 pumas (Puma concolor); small felids (77 animals) - 29 tiger cats (Leopardus tigrinus), 19 jaguarundis (Herpailurus yagouaroundi), 10 margays (Leopardus wiedii), 9 pampas cats (Oncifelis colocolo), 6 geoffroy's cats (Oncifelis geoffroyi), and 4 servals (Leptailurus serval). Samples were obtained by the introduction of a sterile swab into the ear canal after cleaning the auricle with an alcohol-ether solution. The swabs were seeded onto Petri dishes containing modified Mycosel agar and sterile olive oil was added to the surface of the medium before specimen seeding. The plates were incubated at 35oC for two weeks. The isolates were analyzed regarding macro-and micromorphology and identified through catalase tests and growth on Tween 20, 40, 60 and 80. Malassezia spp. were isolated from 58 of the felids studied (43.9%) and from 102 samples of cerumen (38.6%). Malassezia sympodialis was isolated exclusively in large felids (33 animals-56.9%), and Malassezia pachydermatis exclusively in smaller varieties (25 animals - 43.1%). The incidence of fungi was higher in lions, with yeast being isolated in 25 of 26 animals (96.2%). Forty-eight strains (47.1%) were isolated from the right ear canal and 54 (52.9%) from the left. Although M. pachydermatis is the species considered a member of the microbiota of the mammalian external ear canal these results suggest that M. sympodialis participates in the microbiota of large felids.     

Propene polymerization with δ-TiCl3/AlCl(C2H5)2 and δ-TiCl3/Al(C2H5)3 catalyst systems, 1. Effect of external donors

The effect of ethyl benzoate (EB), diisobutyl phthalate (DIBP), dibutyl ether (DBE) and triethoxy(phenyl)silane (EPS) as third components on the propene polymerization with the catalyst systems δ-TiCl₃/AlCl(C₂H₅)₂ and δ-TiCl₃/Al(C₂H₅)₃ was investigated. The influence of external donors on the isotacticity, catalyst activity and average molecular weight (M _v) was tested. If external donors are employed, M _v decreases, the insoluble fraction in boiling isooctane increases and the catalyst activity is strongly influenced by the mole ratio external donor/TiCl₃. The results indicate that all external donors employed have the same qualitative effect on catalytic active centers.     

Choledochoenterostomy with an anti-reflux mechanism

A new technique of choledochoenterostomy was devised to solve some of the problems of enterobiliary anastomosis with a normal calibre. The distal extremity of the common bile duct is completely surrounded by the bowel mucosa to a length of 3 cm after seromyectomy of a bowel wall rectangle of 4 × 1 cm. Experimental studies in rats and dogs demonstrated that this procedure prevents the risks of anastomotic disruption and functions like a mechanical unidirectional valve, which has great efficacy in stopping enterobiliary reflux. Studies in ten patients with obstructive jaundice with an extrahepatic biliary dilation less than 1.2 cm diameter submitted to this procedure Confirmed the experimental results. All patients were asymptomatic, without jaundice and with normalization of the liver enzymes after 2 months. The permeability of the valvular anastomosis studied by cholangiography, the HIDA ^99mTc test and manometry was quite similar to other classical biliary-enteric anastomosis. In contrast, anti-reflux efficacy was only demonstrated in patients with a valvular anastomosis.     

The HIV prevalence among pregnant women in the Amsterdam region (1988–1991)

The objective of the study was to monitor the HIV prevalence in the years 1988–1991 among pregnant women in the Amsterdam region, visitors to an abortion clinic and 3 outpatient infertility clinics. All women attending these clinics were asked to participate in the study on a voluntary basis and were tested with informed consent. The women were questioned about risk-bearing behaviour of themselves and their sexual partner(s). In the period 1988–1991, of the 23,827 eligible pregnant women, 22,165 women participated (93.0%). Twenty-seven women were found to be positive for HIV antibodies (0.12%, 95% CI: 0.08%–0.17%), of whom twenty belonged to a known HIV risk group or had a partner who belonged to one of these groups and 7 women had no known HIV risk. Seventeen of the 27 women had a foreign nationality. The annual HIV prevalence among pregnant women was: 1988: 0.28%; 1989: 0.10%; 1990: 0.10%; 1991: 0.11%. In the years 1990 and 1991, of the 1,128 eligible women visiting the abortion clinic 953 (84.5%) were tested. Eleven women were HIV-seropositive (1.15%, 95% CI: 0.6%–2.0%), of whom 9 were from an AIDS endemic region, 1 woman had a partner from this region and 1 woman had no known HIV risk. Four African women had HIV-2 antibodies. At the 3 outpatient infertility clinics 1 woman was found to be HIV-positive (0.13%; 95% CI: 0.02–0.9). She had no other risk than a partner from an AIDS endemic area. In the Amsterdam region there was a steady and low HIV prevalence (0.1%) among pregnant women through the years 1988–1991. The prevalence in the abortion clinic was ten times higher. The program was able to detect possible high risk groups within the population. Migration and travelling can play an important role in the spread of HIV in the general heterosexual population.     

Impact of HIV antibody testing on changes in sexual behavior among homosexual men in The Netherlands.

Between October 1984 and May 1986, 746 homosexual men, living in and around Amsterdam, The Netherlands, were surveyed at three consecutive six months periods regarding their sexual behavior. At the start of the study all subjects, of whom 234 (31 per cent) were HIV-Ab seropositive, were informed about their HIV antibody status. Seropositives initially reported more sexual partners than seronegatives; they also showed a greater reduction in the number of sexual partners and the number of partners with whom all forms of sexual practices were performed than did seronegatives. In both groups subjects were more likely to terminate orogenital intercourse than anogenital intercourse and masturbation.     

44Sacral extracorporeal magnetic stimulation is an effective treatment for pelvic floor dyssynergia; Comparative study with biofeedback therapy

Background: Recent development of extracorporeal magnetic stimulation (ECMS) which uses current‐changing magnetic fields allows the induction of electrical stimulation in the desired deep tissue. Recent study showed the sacral nerve stimulation reduces corticoanal excitability that may play a functional role in anal continence mechanisms. Preliminary study shows that ECMS of sacral nerve can modify pelvic floor function and expel rectal balloon in patients with pelvic floor dyssynergia (PFD). Aims: To evaluate the effect of ECMS compared with biofeedback therapy (BF) in patients with PFD. Methods and Materials: Thirty‐eight patients who fulfilled Rome II criteria for PFD by colon transit time and anorectal function tests, were randomly treated with 8 sessions of ECMS (2/weeks; n = 19) at prone position or BF (2/weeks; n = 19) at sitting position. Stimulation parameters were set at 50–80% of maximum intensity, 10 and 50 Hz frequency, 3 s burst length with 3 and 6 s off using arm‐typed stimulator (BioCom‐1000, Mcube Co., Korea). Symptom scores for constipation with/without anorectal function test were repeatedly measured after each treatment. Response was defined as 50% or more decreased symptom score after treatment (partial response: 30–50%, poor: <30%). Results: Fifteen patients (age 49.1 ± 13.4 years, mean ± SD; 4 men) completed 8 session of BF and 14 patients (54.5 ± 17.6 years, 3 men) completed 8 session of ECMS. Four patients of BF group discontinued treatment due to unsatisfactory therapeutic effect (n = 1) and withdrew consent (n = 3) and 5 patients of ECMS group discontinued treatment because of same reasons (n = 1, 4). Total symptom scores were significantly decreased after treatment of 8 session in both treatment groups (13.4 ± 6.6 vs. 4.3 ± 4.0 for BF, p = 0.009; 14.9 ± 5.6 vs. 3.4 ± 4.0 for ECMS, p < 0.001). Bowel movements per week were also significantly increased after treatment in both groups (median 2 vs. 7 for BF, p = 0.035; median 2 vs. 7 for ECMS, p = 0.008). Thirteen out of 15 patients showed response in BF group and 12 out of 14 showed good response in ECMS group. No adverse effects in both groups. Conclusions: ECMS is as effective as BF for the treatment of PFD. Long‐term effect of ECMS for the patients with pelvic floor dyssynergia need to be evaluated in the near future.