A rare cause of hydrops fetalis in two Gaucher disease type 2 patients with a novel mutation

Metabolic Brain Disease - Gaucher disease type 2 is the most progressive and the rarest form of Gaucher disease, defined as the acute neuronopathic type. We presented two GD2 patients who died...     

Factors influencing the survival of patients with cancer of the stomach

During the period 1969 through 1973, 332 male and 135 female patients diagnosed with stomach cancer were registered with the Cross Cancer Institute in Edmonton, Alberta. These patients were followed up to December 31, 1981. Therefore, we were able to observe eight year survival times for all patients. Rates for one to eight years of survival were calculated using the Kaplan-Meier method. The results showed poor survival experience for male and female patients, similar to survival reported in other countries. Stomach cancer in Alberta belongs to the ten most common cancers and a search for factors influencing survival is therefore important. A large number of environmental factors and factors pertaining to the patients' lifestyle, as well as clinical factors related to stage of disease and treatment were examined using Cox's proportional hazard model. Surgical treatment and the presence of metastases were found to be important determinants of survival for both sexes. In males, increased age and British ethnicity were associated with poor survival prospects. Relative hazards of dying are presented for various combinations of factors.     

Monitoring adverse events following immunization with a new conjugate vaccine against group A meningococcus in Niger, September 2010

Introduction

MenAfriVac is a new conjugate vaccine against Neisseria meningitidis serogroup A, the major cause of meningitis outbreaks in sub-Saharan Africa. In Niger, the MenAfriVac introduction campaign was conducted in the District of Filingue, during September 2010, targeting 392,211 individuals aged 1–29 years. We set up an enhanced spontaneous surveillance system to monitor adverse events following immunization (AEFI) during the campaign period and 42 days thereafter.

Methods

All the 33 health centres of the district have been designated as surveillance units, which reported AEFIs on a daily basis to the health district headquarters. Health care workers were instructed to screen patients presenting with predefined conditions of interest and patients spontaneously presenting at units or at vaccination posts with complaints after vaccination. Cases were classified as serious (resulting in death, hospitalization or long-term disability) or minor. A National Expert Committee was established to determine if serious cases were causally associated with the vaccine.

Results

In total, 356,532 vaccine doses were administered. During 61 days of monitoring, 82 suspected AEFIs were reported: 16 severe and 66 minor. The cumulative incidence was of 23.0 per 100,000 doses. Among severe cases, 14 were classified as coincidences, one urticaria complicated by respiratory distress was classified as a probable vaccine reaction, and one death was unclassifiable because post-mortem information was unavailable. The number of units that reported at least one case was 19/33 (57.6%).

Conclusions

Although these results are limited by underreporting of cases, we did not identify safety concerns with MenAfriVac. The lessons learned from this experience should be used to reinforce the national pharmacovigilance system in Niger to make it complaint with international standards. In order to do so, we recommend using a lighter system for routine; and conducting regular training and supervisory activities to increase its acceptance among local health workers.     

Rheologic investigation of electrode gels

Nine electrode gels have been investigated 6 of them are used in hospital pharmacies of this country. The results were compared to properties of two gels from abroad, the recommended electrode gel has been developed on this basis. Character of formed gel structure has been studied by flow curves and structural forces have been numerically characterized by determining the parameters of the function made linear. By determination of electric conductivity the proposed Carbopol gel has been found suitable not only to supersonic tests but also to ECG and may be to EEG.     

Small bowel obstruction at a Filshie clip applicator port site

Laparoscopic Filshie clip sterilisation remains a common method of permanent female contraception. Worldwide, approximately 190 million couples use tubal occlusion (United Nations world population monitoring. United Nations, 2002). Trocar site incisional hernia has been reported as a complication of laparoscopic surgery where a 10-mm port was employed (Tonouchi et al. Arch Surg 139(11):1248–1256, 2004). It is common practice to repair port sites of 10 mm or more to prevent herniation. Port sites of 5 mm are not routinely repaired by most surgeons because it is thought that such iatrogenic fascial defects are not large enough to predispose to hernia (Reardon et al. J Laparoendosc Adv Surg Tech A 9(6):523–525, 1999). We report a rare case of early Filshie clip applicator port site intestinal obstruction following laparoscopic sterilisation. The mechanism of hernia formation and a preventive strategy are discussed.     

Geographic areas with extremely high mortality correlations for cancers in the province of Alberta, Canada

Age-adjusted mortality rates, calculated by the direct method and based on the 1971 census-population of Alberta, for 24 male and 26 female cancer sites, from 29 census sub-divisions of the province of Alberta, collected over the period 1961 through 1981, were employed in this study. A total of 50,374 cases of cancer mortality were utilized. Correlations between pairs of cancer sites across census sub-divisions were calculated and cancer sites noted that varied little in mortality rates over Alberta's geographic areas. Significant correlations of mortalities (p less than 0.01) were observed between 5 pairs of male cancer and 18 pairs of female cancer sites. Risk factors common to correlating pairs are discussed. Similarities of our results with other studies are pointed out. The geographical distribution of extreme mortality correlations is analyzed and possible risk factors are discussed.     

The benefit of myomectomy in women aged 40 years and above: Experience in an urban teaching hospital in Nigeria

Background:

Abdominal myomectomy remains the mainstay of surgical management of uterine fibroids in our environment. However, its benefit in women aged 40 years and above remains debatable.

Materials and Methods:

An 11-year prospective study was conducted involving 98 women, aged 40 years and above, who had abdominal myomectomy for the treatment of uterine fibroid at the University of Maiduguri Teaching Hospital, Maiduguri. They were followed up regularly for 1–6 years to detect conception, resolution of symptoms and obstetrics performance. Data were analyzed using SPSS version 13.

Results:

The mean age of the patients was 42.6±2.9 years and 77 (78.6%) of them were nulliparous. Lower abdominal swelling was the commonest clinical presentation and the mean uterine size was 18.6±8.5 weeks. Infertility with uterine fibroids was the indication for myomectomy in majority of the cases [48 (48.9%)], while pregnancy complications accounted for 11.2% (11) of the cases Fertility restoration was 10.4% among the infertile patients. There was complete resolution of symptoms in 35.9% of those who required symptomatic relief, and term pregnancies were recorded in 72.7% of patients with pregnancy complications.

Conclusion:

Myomectomy is the recommended treatment of uterine fibroids in women aged 40 years and above with infertility and who wish to become pregnant. If there is no need for further fertility preservation, hysterectomy should be offered.