Very severe hypertriglyceridemia complicating pediatric acute lymphoblastic leukemia treatment: a call for management guidelines.

OBJECTIVES: Severe and very severe hypertriglyceridemia although rare within the pediatric population occur more often among oncology patients, secondary to chemotherapeutic agents. Currently there exists minimal literature to guide management of severe hypertriglyceridemia among pediatric patients. Very-low-fat dietary restriction should be considered over nil per os (NPO) for initial management of severe hypertriglyceridemia in stable pediatric patients. Pediatricians caring for oncology patients must consider chylomicronemia as a potential etiology for presenting symptoms. Pediatric severe hypertriglyceridemia management guidelines are needed as pediatricians must currently rely on anecdotal experiences for management decisions. CASE PRESENTATION: Three children receiving treatment for acute lymphoblastic leukemia required hospitalization for very severe hypertriglyceridemia. Management varied among the cases but included: NPO or very-low-fat diet, insulin, intravenous fluids, fibrates, and omega-3 fatty acids. CONCLUSIONS: These cases suggest that pediatric severe hypertriglyceridemia management, in the absence of pancreatitis should allow a very-low-fat diet initially rather than NPO followed by pharmacologic therapies.

Ethical and Analytic Challenges With Genomic Sequencing of Relapsed Hematologic Malignancies Following Allogeneic Hematopoietic Stem-Cell Transplantation.

The implementation of precision medicine and next-generation sequencing technologies in the field of oncology is a novel approach being more widely studied and used in cases of high-risk primary and recurrent malignancies. Leukemias are the second most common cause of cancer-related mortality in children and the sixth most in adults. Relapsed leukemia represents a major component of the population that may benefit from genomic sequencing. However, ethical and analytic challenges arise when considering sequencing of biologic samples obtained from patients with relapsed leukemia following allogeneic hematopoietic stem-cell transplantation. Blood from the recipient after transplantation would include donor-derived cells and thus, genomic sequencing of recipient blood will interrogate the donor germline in addition to the somatic genetic profile of the leukemia cells and the recipient germline. This is a situation for which the donor will not have typically provided consent and may be particularly problematic if actionable secondary or incidental findings related to the donor are uncovered. We present the challenges raised in this scenario and provide strategies to mitigate this risk.

Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review.

PURPOSE: Postural orthostatic tachycardia syndrome has been recognized for decades, but treatment is largely based on anecdotal experience and expert opinion. Pharmacologic treatment is inconsistent and unstandardized. We did a systematic review to identify controlled studies from which informed treatment decisions can be made. METHOD: Through a standard systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we identified all English-language studies of a medication treatment for postural orthostatic tachycardia syndrome that included a comparison or control group and followed outcomes for at least 1 week of treatment. RESULTS: A total of 626 studies were identified by the search criteria, and 8, involving a total of 499 patients, met the criteria. No studies were adequately similar to allow for meta-analysis. Of the identified 8 studies, 2 were randomized controlled trials and 4 had been subjected to peer review. In individual studies, there was some favorable effect with fludrocortisone, beta blockers, midodrine, and selective serotonin reuptake inhibitors. CONCLUSION: There is a paucity of high-quality data about effectiveness of medication in the treatment of postural orthostatic tachycardia syndrome. Nonetheless, 2 randomized trials and 6 other reports show some favorable effects of medication.

Awareness of an obstetric population about environmental tobacco smoking.

BACKGROUND AND OBJECTIVES: The reported rate of women's smoking is typically low. However, many pregnant women are exposed to environmental tobacco smoke (ETS), which could affect their own health and the health of their growing fetus. The aim of this study was to estimate the magnitude of the problem of exposure to ETS and assess the awareness of postpartum women to ETS and its possible effects. DESIGNS AND SETTINGS: This was a cross-sectional study conducted on 1182 postpartum women at a university hospital in Riyadh, Saudi Arabia, between 1(st) January and 30(th) June, 2012. MATERIALS AND METHODS: A structured questionnaire was used for data collection. Factors associated with the level of understanding of the possible effects of ETS exposure were analyzed. RESULTS: The majority of the participating women knew that exposure to ETS had adverse effects on maternal and fetal health (>80%), but their knowledge of the specific effects on fetal health was limited. The level of mothers' education was found to be associated with better knowledge of effects on mother and fetal health (P < 0.01). CONCLUSION: This study revealed that pregnant women in our sample had limited knowledge of the specific effects of ETS on fetal health. This shortcoming in knowledge needs to be addressed by improving health.

A cervix penetrating the posterior bladder wall: case report.

Clinical manifestations of vesicouterine fistulas, a rare complication of cesarean section, include amenorrhea and cyclic hematuria (menouria) without urinary incontinence, a triad collectively known as "Youssef's syndrome." Fistulas affecting the uterus usually reside above the isthmus or at the cervix and have a distinct morphology composed of granulation tissue, chronic inflammatory cells, and fibrous tissue. We present a case of Youssef's syndrome where the patient's entire cervix penetrated into the posterior bladder wall rather than the two organs connecting via a discrete fistulous tract.