Congenital-type juvenile xanthogranuloma: A case series and literature review.

BACKGROUND/OBJECTIVES: Congenital juvenile xanthogranulomas are infrequently described in the medical literature. We report three previously unpublished cases and systematically review the literature to better characterize this variant. METHODS: We surveyed English-language articles indexed in MEDLINE (1951-March 2017) and EMBASE (1974-March 2017) for cases of congenital-onset juvenile xanthogranulomas confirmed on histopathology. Cases were divided into two categories: cutaneous only or cutaneous with systemic involvement. RESULTS: We identified 31 cases of congenital juvenile xanthogranulomas involving only the skin and 16 cases with systemic involvement. Congenital juvenile xanthogranulomas involving only the skin were large (> 3 cm), presented with various clinical morphologies, and showed signs of regression by 1 year of age. Atypical clinical presentations included exophytic tumors, infiltrative plaques, agminated plaques, and subcutaneous tumors. Complications included ulceration and anetodermic scarring. Infants with congenital cutaneous juvenile xanthogranulomas who also had systemic involvement typically had multiple cutaneous tumors and hepatic involvement and showed signs of spontaneous regression independent of treatment. CONCLUSIONS: The medical literature supports that congenital juvenile xanthogranulomas behave in a fashion similar to that of juvenile xanthogranulomas of infancy or childhood. Congenital cutaneous juvenile xanthogranulomas with or without systemic involvement spontaneously regress. The varied clinical presentations in the skin may lead to misdiagnosis, inappropriate examination, and unnecessary treatments. Infants with multiple congenital cutaneous juvenile xanthogranulomas should be evaluated for systemic involvement, with a particular focus on the liver, because 72.2% of these children were found to have hepatic juvenile xanthogranulomas.

Medication dyes as a source of drug allergy.

Excipients are defined as inert substances added to a drug or food to confer a suitable consistency, appearance, or form. They may be added for bulk, to change dissolution or the kinetics of absorption, to improve stability, to influence palatability, or to create a distinctive appearance. The last function may depend heavily on the use of coloring agents, especially when there are multiple dosages (such as with warfarin), and dose confusion may result in profound complications. While described as inert, excipients have been associated with triggering immunological reactions, although this is almost never considered in common practice when patients have reactions to medications, even when they appear to react to many different and distinct drugs. We have found a cohort of 11 patients with chronic, unexplained pruritic skin disorders that have responded to medication changes centered around avoidance of coloring agents, particularly FD&C Blue No. 1 (bright blue) and Blue No. 2 (indigo carmine). We believe that reactions to agents that color medications and foods may be more common than previously appreciated and that recognition of this phenomenon may provide therapeutic alternatives to patients with intractable pruritic disorders.

Sex bias in cardiovascular testing: the contribution of patient preference.

STUDY OBJECTIVE: Women with potential acute coronary syndromes are less likely to receive cardiac catheterization or revascularization than men. We hypothesize that this may be due to different diagnostic test preferences of female and male patients. METHODS: We conducted a cohort study at 4 emergency departments enrolling patients who presented with symptoms of potential acute coronary syndromes. After hearing the potential benefits and harms of each test, subjects completed a 21-item survey assessing their preference for noninvasive testing versus cardiac catheterization. Based on hypothetical test results, similar questions about medical versus interventional management were asked. Subjects were also queried about likelihood of following physician recommendation for each test or intervention. Actual 30-day testing and interventions were recorded. The main outcome was patient preference about each procedure and the likelihood of patient saying they would accept the physician recommendation. RESULTS: One thousand eighty patients enrolled; 652 (60%) were admitted to the hospital. With regard to diagnostic test preference, both women and men preferred stress test to catheterization (women 58% versus men 52%; difference 6% [95% confidence interval {CI} -0.06% to 12%]), and the proportion of women and men who would accept the physician recommendation for stress tests was similar (85% for both); however, the stated acceptance rate for cardiac catheterization was lower for women (65% versus 75%; difference -10% [95% CI -15% to -4%]). Women were 6% less likely (67% versus 73%; 95% CI for difference 12% to 0.5%) to accept percutaneous coronary intervention over medical therapy and 7% less likely (61% versus 68%; 95% CI for difference -13% to 1%) to desire coronary artery bypass grafting over medical therapy. The survey results are consistent with the patients' clinical course. During the initial hospitalization, women were less likely to receive diagnostic testing of any type (38% versus 45%; difference -7%; 95% CI for the difference -13% to -1.5%) and cardiac catheterization (10% versus 17%; difference -7% [95% CI -11% to -2%]). Revascularization was infrequent in both groups (4% versus 6%; difference -2% [95% CI -5% to 0.6%]). CONCLUSION: Although women and men had similar preferences about cardiac diagnostic tests and treatment options, women were less likely than men to say they would accept the physician recommendation for any intervention. Patient preference may partially explain the disparity in cardiovascular testing between women and men.

The decubitus ulcer: facts and controversies.

Defining the decubitus ulcer proves as difficult as agreeing on a name for the condition. Causes include pressure over bony prominences, shearing force, destruction of skin, and compromised blood flow. Evidence is emerging of the importance of ischemia as a primary causative agent, rather than pressure, which needs further investigation. Scales, staging, and treatment and prevention guidelines should be used with caution due to their arbitrary implementation and lack of evidence-based support. Unfortunately, much of the research and expert opinion developed by the government and touted as regulation lacks appropriate strength-of-evidence. Although decubitus ulcers should be prevented and treated to the best of our abilities, recognizing the possibility that the skin, like any other organ in the body, may fail is crucial.

Observed behaviors of subjects during informed consent for an emergency department study.

STUDY OBJECTIVE: To determine emergency department patients' behaviors during informed consent for an intimate partner violence survey. METHODS: We conducted a cross-sectional study during administration of informed consent. Research assistants recorded whether informed consent was read, time spent reading it, whether questions were asked, and whether the patients took a copy of the form that was handed to them. Results are reported as percentage of frequency of occurrence. RESULTS: Of 1,609 patients approached for the intimate partner violence study, 1,312 (82%) patients participated. After verbal description of the study, 53% of patients read the informed consent but only 13% spent more than 2 minutes doing so. Only 20% of patients asked questions and less than half (49%) accepted a copy of the form when it was handed to them. CONCLUSION: Patients who participated in an intimate partner violence study did not spend a lot of time reading the consent document, asked few questions, and did not take the copy of the consent form with them. Future studies of the current consent process should determine whether it provides adequate human subjects protections in a manner desired by the patient.

Combining Thrombolysis in Myocardial Infarction risk score and clear-cut alternative diagnosis for chest pain risk stratification.

OBJECTIVE: The Thrombolysis in Myocardial Infarction (TIMI) risk score is a validated risk stratification tool useful in patients with definite and potential acute coronary syndromes (ACS) but does not identify patients safe for discharge from the emergency department (ED). Likewise, the use of a clear-cut alternative noncardiac diagnosis risk stratifies patients but does not identify a group safe for discharge. We hypothesized that the presence of an alternative diagnosis in patients with a TIMI risk score less than 2 might identify a cohort of patients safe for ED discharge. METHODS: In prospective cohort study, we enrolled ED patients with potential ACS. Data included demographics, medical history, components of the TIMI risk score, and whether the treating physician ascribed the condition to an alternative noncardiac diagnosis. Investigators followed the patients through the hospital course, and 30-day follow-up was done. The main outcome was 30-day death, myocardial infarction, or revascularization. RESULTS: A total of 3169 patients were enrolled (mean age, 53.6+/-14 years; 45% men; 67% black). There were 991 patients (31%) with an alternative diagnosis, 980 patients with a TIMI risk score of 0, and 828 with a TIMI score of 1. At low levels of TIMI risk (<3), adding in a clinical impression of an alternative diagnosis did not reduce risk; at higher levels of TIMI risk, it did. The incidence of 30-day death, myocardial infarction, or revascularization for patients with a clinical impression of an alternative diagnosis and a TIMI score of 0 was 2.9% (95% confidence interval, 1.6%-5.0%). CONCLUSIONS: The TIMI risk score stratifies patients with and without an alternative diagnosis. Unfortunately, patients with both a low TIMI risk score and a clinical impression of an alternative noncardiac diagnosis still have a risk of 30-day adverse events that is not low enough to allow safe discharge from the ED.