Three Rwandan Children With Massive Splenomegaly and Epstein-Barr Virus-associated Lymphoproliferative Disorders: Case Presentations and the Literature Review.

This report describes 3 Rwandan children with massive splenomegaly and pancytopenia who underwent splenectomy. Each was diagnosed with Epstein-Barr virus-associated lymphoproliferative disorder (EBV LPD) based on lymphocyte morphology, lymphocyte immunophenotype, and the results of EBV in situ hybridization studies. The differential diagnosis of splenomegaly, with a special emphasis on the sub-Saharan African context, is discussed along with EBV and associated disorders. These cases serve as a call to consider EBV LPD in the differential diagnosis of splenomegaly in children in whom common causes have been ruled out.

Patient- and Family-Centered Care as an approach to reducing disparities in asthma outcomes in urban African American children: A review of the literature.

We thank Cathy Eames (Director, Library Services, Detroit Medical Center) for valuable input and assistance with the search strategy. Funding for this research was supported by a grant from Children's Hospital of Michigan Research Foundation (Principal Investigator: Terrance L. Albrecht, Ph.D.). BACKGROUND: Patient- and family-centered care (PFCC) has the potential to address disparities in access and quality of healthcare for African American pediatric asthma patients by accommodating and responding to the individual needs of patients and families. STUDY OBJECTIVES: To identify and evaluate research on the impact of family-provider interventions that reflect elements of PFCC on reducing disparities in the provision, access, quality, and use of healthcare services for African American pediatric asthma patients. METHODS: Electronic searches were conducted using PubMed, CINAHL, and Psyclnfo databases. Inclusion criteria were peer-reviewed, English-language articles on family-provider interventions that (a) reflected one or more elements of PFCC and (b) addressed healthcare disparities in urban African American pediatric asthma patients (≤18years). RESULTS: Thirteen interventions or programs were identified and reviewed. Designs included randomized clinical trials, controlled clinical trials, pre- and post-interventions, and program evaluations. CONCLUSIONS: Few interventions were identified as explicitly providing PFCC in a pediatric asthma context, possibly because of a lack of consensus on what constitutes PFCC in practice. Some studies have demonstrated that PFCC improves satisfaction and communication during clinical interactions. More empirical research is needed to understand whether PFCC interventions reduce care disparities and improve the provision, access, and quality of asthma healthcare for urban African American children. ELECTRONIC DATABASES USED: PubMed, CINAHL, and Psyclnfo ABBREVIATIONS: AA-African American: CCT-Controlled clinical trial; ED-Emergency Department; ETS-Environmental tobacco smoke; FCC- Family Centered Care; PFCC-Patient and Family Centered Care; RCT- Randomized, controlled trial.

The importance of surgical staging in women with uterine serous carcinoma: experience in a single institution reveals a survival benefit.

OBJECTIVE: To assess the appropriate extent of surgical staging in women with clinically early stage uterine serous carcinoma (USC). METHODS: We conducted a single-institution retrospective cohort study of all women with USC between 2007 and 2012. Treatment practices, outcomes, and factors affecting survival were analyzed using univariate and multivariate analysis. RESULTS: Eighty-four patients were identified, 76 of whom were included in the analysis. Preoperative pathology correctly identified USC in 73.3% of cases. Surgical stage distribution was 44.7% stage I, 7.9% stage II, 31.6% stage III, and 15.8% stage IV. Women thought to have early stage disease preoperatively encompassed 84.2% (64) of the cohort. Fifty-two (81.3%) of these women with clinically early stage disease had complete surgical staging. Thirty-four (53.1%) were determined to have surgical stage I, and the remaining 30 (46.9%) had occult advanced stage disease. Median follow-up was 43.2 months. Univariate analysis found a significant increase in progression-free survival and overall survival for women with clinically early stage disease with positive lymphovascular space invasion (P < 0.001 and P = 0.002, respectively), positive peritoneal cytology (P = 0.022 and P = 0.04, respectively), early stage (P < 0.001 and P = 0.004, respectively), and elevated serum CA125 at diagnosis (P = 0.003 and P = 0.001, respectively). On multivariate analysis, early stage (hazard ratio [HR] 9.87; 95% CI 2.79 to 34.92, P < 0.001) and complete surgical staging (HR 2.96; 95% CI 1.05 to 8.37, P = 0.040) were associated with prolonged progression-free survival, while overall survival was not affected by complete surgical staging (HR 1.92; 95% CI 0.64 to 5.76, P = 0.79). CONCLUSION: Complete surgical staging prolongs the progression-free survival of women with clinical early-stage uterine serous cancer. Although this does not extend to overall survival, this enables patients to have an improved quality of life with a longer interval without the burden of disease.

Objectif : Déterminer l'ampleur adéquate de la stadification chirurgicale chez les femmes qui présentent un carcinome séreux de l'utérus (CSU) de stade clinique précoce. Méthodes : Nous avons mené une étude de cohorte rétrospective portant sur toutes les femmes qui ont présenté un CSU entre 2007 et 2012 au sein d'un seul établissement. Les pratiques de traitement, les issues et les facteurs affectant la survie ont été étudiés au moyen d'analyses univariées et multivariées. Résultats : Quatre-vingt-quatre patientes ont été identifiées, 76 desquelles ont été admises à l'analyse. L'analyse pathologique préopératoire a correctement identifié le CSU dans 73,3 % des cas. La distribution des stades chirurgicaux était la suivante : stade I, 44,7 %; stade II, 7,9 %; stade III, 31,6 %; et stade IV, 15,8 %. Les femmes qui, avant l'opération, semblaient présenter une maladie de stade précoce représentaient 84,2 % (64) de la cohorte. Cinquante-deux (81,3 %) de ces femmes présentant une maladie de stade clinique précoce ont subi une stadification chirurgicale complète. Il a été déterminé que 34 (53,1 %) de ces 64 femmes présentaient un stade chirurgical I, tandis que les 30 autres (46,9 %) présentaient une maladie occulte de stade avancé. Le suivi médian a été de 43,2 mois. L'analyse univariée a constaté une hausse significative des taux de survie sans progression et de survie globale chez les femmes connaissant une maladie de stade clinique précoce qui avaient obtenu des résultats positifs en ce qui concerne l'invasion de l'espace lymphovasculaire (P < 0,001 et P = 0,002, respectivement), qui avaient obtenu des résultats positifs dans le cadre de la cytologie péritonéale (P = 0,022 et P = 0,04, respectivement), qui présentaient un stade précoce (P < 0,001 et P = 0,004, respectivement) et chez lesquelles un taux sérique élevé de CA125 avait été constaté au moment du diagnostic (P = 0,003 et P = 0,001, respectivement). Dans le cadre de l'analyse multivariée, la présence d'un stade précoce (rapport des risques instantanés [RRI], 9,87; IC à 95 %, 2,79 - 34,92, P < 0,001) et la tenue d'une stadification chirurgicale complète (RRI, 2,96; IC à 95 %, 1,05 - 8,37, P = 0,040) ont été associées à une prolongation de la survie sans progression, tandis que la survie globale n'a pas été affectée par la tenue d'une stadification chirurgicale complète (RRI, 1,92; IC à 95 %, 0,64 - 5,76, P = 0,79). Conclusion : La tenue d'une stadification chirurgicale complète prolonge la survie sans progression des femmes qui présentent un carcinome séreux de l'utérus de stade clinique précoce. Bien que cette intervention n'exerce pas d'effets sur la survie globale, elle permet aux patientes de connaître une amélioration de leur qualité de vie (prolongation de l'intervalle dans le cadre duquel les patientes n'ont pas à vivre avec le fardeau de la maladie).

A rare case of NUT midline carcinoma.

► NUT midline carcinoma is a rare and aggressive cancer arising in midline structures and is of squamous cell lineage. ► Diagnosis by use of molecular tests is possible with clinical suspicion. ► NUT midline carcinoma is refractory to conventional treatments, but understanding of molecular alterations may lead to an effective therapy.

Choosing the right patient: planning for laparotomy or laparoscopy in the patient with endometrial cancer.

OBJECTIVE: Endometrial cancer remains the most commonly diagnosed gynaecologic cancer in North America. The staging and initial treatment of endometrial cancer involves surgery. Laparoscopic surgery is increasingly used as an alternative to laparotomy. Patient selection for laparoscopy can be optimized by examining factors involved in both the choice of surgical approach and the ultimate procedure performed. We wished to identify factors that might be barriers to laparoscopic surgery in women with endometrial cancer who had surgery performed by the gynaecologic oncology group at the Capital District Health Authority (CDHA) in Halifax, Nova Scotia. METHODS: We conducted a retrospective review of the records of women with endometrial cancer, histologically confirmed preoperatively between 2005 and 2007, who underwent surgery at the CDHA. RESULTS: Between 2005 and 2007 in Nova Scotia, 428 cases of endometrial cancer were diagnosed, and 289 women with a preoperative diagnosis of endometrial cancer underwent surgery at the CDHA. Of these, 66.1% (191/289) underwent a planned laparotomy, and 33.9% (98/289) had a planned laparoscopy. The proportion of attempted laparoscopies increased from 21.9% to 57.1% (P = 0.002) over time, while there was no change in the conversion rate (P = 0.23). Patients with abnormal findings on pelvic examination were more likely to have a laparotomy (RR = 1.5; 95% CI 1.34 to 1.68). Independent predictors of laparoscopic conversion to laparotomy were age 75 years or over (P = 0.03) and non-endometrioid histology (P = 0.002). CONCLUSION: Our data identify age and non-endometrioid histology as independent factors for conversion of surgery for endometrial cancer from laparoscopy to laparotomy. With this information we can optimize patient selection for laparoscopic surgery. Patients undergoing a conversion to laparotomy do not have a significant increase in surgery time or perioperative morbidity.

Ovarian fibromas in pediatric patients with basal cell nevus (Gorlin) syndrome.

BACKGROUND: Gorlin syndrome is a rare genetic condition consisting of multiple basal cell nevi associated with other entities such as medulloblastoma, skeletal abnormalities, and ovarian fibromas. CASE: A 15-year-old girl presented with abdominal discomfort. Magnetic resonance imaging showed multiple bilateral solid adnexal masses, the largest measuring 5.5 cm × 6.1 cm × 5.6 cm. At laparoscopy, 10 ovarian fibromas, ranging from 3 mm to 7 cm in size, were removed from each ovary. Concurrent with her gynecologic course, she was found to have maxillary sinus cysts and multiple basal cell nevi. The patient's history was also significant for a medulloblastoma as an infant. Given this constellation of findings, a diagnosis of Gorlin syndrome was made. CONCLUSION: The development of ovarian fibromas in the pediatric population is rare. When diagnosed, the possibility of Gorlin syndrome must be considered. Furthermore, females with Gorlin syndrome would benefit from regular gynecologic surveillance.

Phorbol ester treatment of human myometrial cells suppresses expression of oxytocin receptor through a mechanism that does not involve activator protein-1.

Oxytocin (OT) is a potent uterine agonist. Its receptor (OTR) is a G protein-coupled receptor that is downregulated by prolonged exposure to OT. We hypothesized that activation of PKC mediated this OT-induced decrease in OTR expression. Diminished PKC activity in late pregnancy could underlie the increased expression of uterine OTR preceding labor onset. Using cell cultures of transformed human uterine myocytes, we determined the effects of PKC agonists and antagonists on the expression of OTR. We also explored the effects of overexpression of activator protein-1 (AP-1, a mediator of many PKC- and phorbol ester-induced effects) using adenoviral expression vectors for the AP-1 subunits c-Jun and c-Fos. Stimulation of PKC using the phorbol ester 12-O-tetradecanoylphorbol 13-acetate caused a rapid, significant (P < or = 0.05) increase in c-Jun and c-Fos concentrations but a significant decrease in mRNA for OTR within 6 h followed by a significant decrease in OT binding by 24 h. Adenoviral infection of the cells with expression vectors for c-Jun and c-Fos increased the AP-1 subunits but had no effect on OTR expression. Furthermore, there were no changes in c-Fos or c-Jun levels in human intrauterine tissues around the time of labor onset, as measured by Western analyses. We conclude that phorbol ester treatment decreases OTR expression, likely through a mechanism that does not involve AP-1.