High-risk pregnancies in Diamond-Blackfan anemia: a survey of 64 pregnancies from the French and German registries

We reviewed 64 pregnancies in 26 women with Diamond-Blackfan anemia (DBA) included in the French and German DBA registries. Complications were seen in 42 pregnancies (66%) and included abortion, pre-eclampsia, in utero fetal death, intrauterine growth retardation, retroplacental hematoma, pre-term delivery and fetal malformations. Of the 34 children (53%) born alive, 13 had DBA. No correlations were found between pregnancy outcome and features of either maternal or child DBA. Pregnancies in DBA-affected women are at high risk, especially for complications likely to be of vascular-placental origin. Careful monitoring with prevention of severe anemia and early introduction of aspirin is suggested.     

Sustained clinical response of large hepatocellular carcinoma after chemoembolization with pirarubicin, amiodarone and Lipiodol

Although chemoembolization is known to be an effective palliative treatment in hepatocellular carcinoma, it has a limited effect in large tumors. We report the case of a patient with a large hepatocellular carcinoma of the left liver who had a significant and sustained clinical response after six sessions of chemoembolization with a pirarubicin/amiodarone/lipiodol emulsion. Pirarubicin is an anthracycline which penetrates faster than doxorubicin into cancer cells. Amiodarone is a multidrug resistance inhibitor. Polysorbate 80, an excipient of injectable amiodarone stabilizes the anthracycline/lipiodol emulsion. The clinical efficacy of this new formulation could be evaluated in a phase II clinical trial.     

MIP-1alpha, MIP-1beta, RANTES, and ATAC/lymphotactin function together with IFN-gamma as type 1 cytokines

We analyzed for the first time the expression of chemokines in subpopulations of the murine immune system at the single-cell level. We demonstrate in vitro and in a model of murine listeriosis that macrophage inflammatory protein (MIP)-1alpha, MIP-1beta, regulated on activation normal T cell expressed and secreted (RANTES), and activation-induced, T cell-derived, and chemokine-related cytokine (ATAC)/lymphotactin are cosecreted to a high degree with IFN-gamma by activated individual natural killer (NK), CD8(+) T, and CD4(+) T helper 1 (Th1) cells. Functionally, ATAC and the CC chemokines cooperate with IFN-gamma in the up-regulation of CD40, IL-12, and tumor necrosis factor-alpha, molecules playing a central role in the effector phase of macrophages. Our data indicate that (i) MIP-1alpha, MIP-1beta, RANTES, and ATAC are not only chemoattractants but also coactivators of macrophages, (ii) MIP-1alpha, MIP-1beta, RANTES, and ATAC constitute together with IFN-gamma a group of "type 1 cytokines," and (iii) these cytokines act together as a functional unit that is used by NK cells in the innate phase and then "handed over" to CD8(+) T cells in the antigen-specific phase of the immune defense, thus bridging the two components of a Th1 immune reaction.     

Lung volumes: measurement, clinical use, and coding

Measurement of lung volumes is an integral part of complete pulmonary function testing. Some lung volumes can be measured during spirometry; however, measurement of the residual volume (RV), functional residual capacity (FRC), and total lung capacity (TLC) requires special techniques. FRC is typically measured by one of three methods. Body plethysmography uses Boyle's Law to determine lung volumes, whereas inert gas dilution and nitrogen washout use dilution properties of gases. After determination of FRC, expiratory reserve volume and inspiratory vital capacity are measured, which allows the calculation of the RV and TLC. Lung volumes are commonly used for the diagnosis of restriction. In obstructive lung disease, they are used to assess for hyperinflation. Changes in lung volumes can also be seen in a number of other clinical conditions. Reimbursement for measurement of lung volumes requires knowledge of current procedural terminology (CPT) codes, relevant indications, and an appropriate level of physician supervision. Because of recent efforts to eliminate payment inefficiencies, the 10 previous CPT codes for lung volumes, airway resistance, and diffusing capacity have been bundled into four new CPT codes.From the Division of Pulmonary, Allergy and Critical Care, Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.Correspondence to: Judd D. Flesch, MD, Division of Pulmonary, Allergy and Critical Care, Perelman School of Medicine at the University of Pennsylvania, 8 Maloney Bldg, 3400 Spruce St, Philadelphia, PA 19104; e-mail: judd.flesch@uphs.upenn.eduFinancial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.     

Quality Improvement Initiative to Reduce Nighttime Noise in a Transplantation and Cellular Therapy Unit

Sleep is an essential biologic function vital for physiologic rest, healing, and emotional well-being. Sleep disruption is commonly seen in patients and caregivers with lengthy hospital stays such as patients undergoing hematopoietic stem cell transplantation and cellular therapy (TCT). Sleep disruption can lead to increased stress and fatigue, affecting caregivers’ ability to support their loved one. The global aim of our quality improvement initiative was to improve sleep quality in TCT patients and caregivers. The smart aim of our project was to decrease nighttime hallway noise from 47 dB to 43 dB and decrease the number of overnight noise peaks greater than 60 dB from 865 to 432 in 6 months. Through a cross-sectional quantitative and qualitative evaluation of sleep we had previously identified poor sleep quality, and with a cross-sectional focus group analysis of patients, caregivers, and medical staff we identified the factors associated with poor sleep. Hallway noise was a major factor. A simplified failure mode analysis identified 4 main key drivers; unobtrusive nighttime cleaning process, nighttime awareness maintenance system, quiet nighttime nursing system, and reliable nighttime awareness system. Several plan-do-study-act interventions took place and were adopted. From January to June 2018 the overnight mean decibel level decreased from 47 dB to 44 dB (6% reduction). Overnight noise spikes above 60 dB decreased from a mean of 865 spikes to a mean of 463 spikes (46% reduction). With a quality improvement initiative, we identified the causes of hallway nighttime hospital unit noise that negatively impact sleep and through a team-based approach performed interventions that successfully mitigated these factors.