A community-based long-term follow up of women undergoing obstetric fistula repair in rural Ethiopia

Objectives  To assess urinary and reproductive health and quality of life following surgical repair of obstetric fistula.
Design  Follow-up study.
Setting  A newly established fistula clinic (2004) at Gimbie Adventist Hospital, a 71-bedded district general hospital in West Wollega Zone, in rural Western Ethiopia.
Population  Thirty-eight women (86%) of 44 who had undergone fistula repair were identified in their community.
Methods  Community-based structured interviews 14–28 months following fistula repair, using a customised questionnaire addressing urinary health, reproductive health and quality of life.
Main outcome measures  Urinary health at follow up was assessed as completely dry, stress or urge incontinence, or fistula. King's Health Questionnaire was modified and used for the quality-of-life assessment.
Results  At follow up, 21 women (57%) were completely dry, 13 (35%) suffered from stress or urge incontinence and three (8%) had a persistent fistula. Surgery improved quality of life and facilitated social reintegration to a level comparable to that experienced before fistula development for both women who were dry and those with residual incontinence ( P  =   0.001). For women still suffering from fistula no change was seen ( P  =   0.1). Four women became pregnant following their surgery, among which there was one maternal death, three stillbirths and one re-occurrence of fistula.
Conclusion  Community-based, long-term follow up after fistula repair succeeded in Western rural Ethiopia. Despite one-third still suffering stress or urge incontinence, the women reported improved quality of life and social reintegration after fistula closure.     

Combined hepatocellular-cholangiocarcinoma: A clinicopathological study

Combined hepatocellular-cholangiocarcinoma (HCC-CC) is an uncommon form of primary liver cancer having features of both hepatocellular and biliary epithelial differentiation. We reviewed 21 cases of this tumour diagnosed between 1972 and 1996 (patient age range 16–79 years; mean patient age 49.7 years; 18 male and three female patients). Histologically, the majority (n= 18) of tumours were ‘mixed’ tumours, in which areas of hepatocellular and biliary epithelial differentiation were intimately mixed within the same tumours. Two patients had separate tumours in which discrete nodules of HCC and CC occurred in the same livers. One patient had a ‘fibrolamellar’ tumour that histologically simulated the fibrolamellar variant of HCC, but some of the tumour cells were mucin-producing cells. Of the 21 cases, mucin was demonstrable in 16 and, in the few mucin-negative tumours, electron microscopic studies confirmed the presence of the dual differentiation. The tumours frequently exhibited an invasive character with frequent venous permeation, direct invasion into adjacent liver parenchyma and tumour microsatellite formation, similar to that of ordinary HCC. Histological evidence of cirrhosis or chronic hepatitis was present in 77.8% of patients and 75% of patients were hepatitis B surface antigen positive. Raised serum α-fetoprotein (AFP) levels (above 300 ng/mL) were present in 61.5% of patients and AFP was detected immunohistochemically in 55% of tumours. The overall survival times of patients with HCC-CC were short. In conclusion, HCC-CC showed clinical and pathological features more akin to those of ordinary HCC than to CC.     

ACR-NEMA digital imaging and communications standards: minimum requirements

The purposes, implications, and history of development of the American College of Radiology-National Electrical Manufacturers Association (ACR-NEMA) Digital Imaging and Communication Standard and its contents are briefly described, and the minimum requirements of the ACR-NEMA Digital Imaging and Communication Standard are described with a concise introduction of each layer. The usefulness, validity, current status, and future development of the standard are also discussed.     

Thin layer chromatography and counter-current analysis in porphyrias

The porphyrin metabolism of 100 patients with porphyria and 351 of their relatives has been studied. Thin layer chromatography of methyl esters of the urinary porphyrin was undertaken in sixty-six patients with different types of porphyria, and forty-five relatives, seventeen patients with hepatic cirrhosis, three patients with lead poisoning and twenty normal control subjects. This investigation was also made on the stools of thirty-six patients with porphyria, and then of their relatives. Countercurrent analysis of the bile of nine selected patients with porphyria was also undertaken. The results provide some evidence that symptomatic hepatic porphyria may be familial. Thin layer chromatography was decisive in the characterization of a new type of porphyria described recently by the authors (hepato-erythrocytic porphyria). The counter-current examination of the bile showed the absence of the 'S 411' porphyrin in all the nine cases investigated.     

In vivo development of dendritic orientation in wild-type and mislocalized retinal ganglion cells

Background

The mammalian amygdala is composed of two primary functional subdivisions, classified according to whether the major output projection of each nucleus is excitatory or inhibitory. The posterior dorsal and ventral subdivisions of the medial amygdala, which primarily contain inhibitory output neurons, modulate specific aspects of innate socio-sexual and aggressive behaviors. However, the development of the neuronal diversity of this complex and important structure remains to be fully elucidated.

Results

Using a combination of genetic fate-mapping and loss-of-function analyses, we examined the contribution and function of Sonic hedgehog (Shh)-expressing and Shh-responsive (Nkx2-1 ⁺ and Gli1 ⁺) neurons in the medial amygdala. Specifically, we found that Shh- and Nkx2-1-lineage cells contribute differentially to the dorsal and ventral subdivisions of the postnatal medial amygdala. These Shh- and Nkx2-1-lineage neurons express overlapping and non-overlapping inhibitory neuronal markers, such as Calbindin, FoxP2, nNOS and Somatostatin, revealing diverse fate contributions in discrete medial amygdala nuclear subdivisions. Electrophysiological analysis of the Shh-derived neurons additionally reveals an important functional diversity within this lineage in the medial amygdala. Moreover, inducible Gli1 ^CreER(T2) temporal fate mapping shows that early-generated progenitors that respond to Shh signaling also contribute to medial amygdala neuronal diversity. Lastly, analysis of Nkx2-1 mutant mice demonstrates a genetic requirement for Nkx2-1 in inhibitory neuronal specification in the medial amygdala distinct from the requirement for Nkx2-1 in cerebral cortical development.

Conclusions

Taken together, these data reveal a differential contribution of Shh-expressing and Shh-responding cells to medial amygdala neuronal diversity as well as the function of Nkx2-1 in the development of this important limbic system structure.     

The role of breast self-examination in early breast cancer detection (results of the 5-years USSR/WHO randomized study in Leningrad)

A randomized population-based study has been carried out since 1985 in Leningrad in order to evaluate the efficacy of breast self-examination (BSE) in early breast cancer detection. The population under study covers 120,310 women aged 40–64 years with no history of breast cancer.About half of these women were exposed to BSE training (60,221) and 60,098 women constituted the control group. BSE teaching was carried out on a person-to-person basis and each patient received the BSE calendar. ME education sessions resulted in a higher frequency of visits to specialists with complaints about pathology of the breast, a higher rate of referral to a specialized institution for an examination, and a higher number of excision biopsies due to a benign lesion (RR = 1.5; 95% C.I. = 1.1 – 1.9) as compared with the control group. As a result of examination, 190 breast cancer patients in the BSE group and 192 patients in the control group were detected. Comparisons of patients from both groups with regard to the size of primary tumor and the incidence of metastatic lesion in the regional lymph nodes showed no differences. The study is ongoing and all cases of breast cancer in the BSE group will be registered up to 1994 and followed-up to 1999; information will then be available on the impact of BSE upon breast cancer mortality.Corresponding author.     

20-year experience on prenatal diagnosis in a reference university medical genetics center in Turkey

Background/aimAlthough cutting edge procedures such as cell-free fetal DNA isolation from maternal blood are now available, invasive prenatal tests are still being used extensively for prenatal diagnosis. The study aims to evaluate the demographic data, indications, and cytogenetic results of 9297 results of patients who underwent prenatal invasive testing for genetic analysis that were referred for the last 20 years in a University Medical Genetics Center.Materials and methodsThe records of 8363 amniocenteses, 626 chorionic villus, and 308 cordocenteses samples were retrospectively evaluated and analyzed regarding referral reasons, indications and their cytogenetic results. The total numbers and the percentages of each group were recorded; Chi-square and logistic regression analyses were performed to give the statistical likelihood of different events. ResultsThe number of referrals decreased significantly after 2009. Risk of having trisomy 21 as well as trisomy 13 and 18 significantly increased in parallel with advanced maternal age. When the 21–25 age group was compared to the older age groups in terms of having a trisomy 21 pregnancy, the risk doubled in the 36–40, 5 times higher in 41–45 and 10-fold in 46–50 age groups. No significant linear correlation between maternal serum screening test results and trisomy 21 was found, however the difference between the pregnancies whom cut-off value above and below 1/250 in maternal serum screening test were significant.ConclusionThese data have provided useful information on the frequency of referrals to the reference genetics department, and the feasibility of genetic services. By reviewing the indications and their corresponding results, we can offer invaluable insights that will be useful in genetic counseling and also in the development of more effective genetic strategies.     

Imaging of follicular dendritic cell tumours of the liver

Follicular dendritic cell tumour of the liver is a recently recognized entity. To date, only two cases have been described, both in the pathology literature. Histologically, it resembles an inflammatory pseudotumour and immunohistochemical and ultrastructural studies are required for its diagnosis. The ultrasound, computed tomography and angiographic features of two cases of follicular dendritic cell tumour of the liver are described in detail. One of the patients had multiple recurrences of this tumour. The imaging features are very similar to those of hepatocellular carcinoma. As follicular dendritic cell tumour is considered to be of low-grade malignant potential, in contrast to the dismal prognosis for hepatocellular carcinoma, it is important to be able to accurately distinguish between the two types of tumour prior to initiating definitive therapy.