Transfusion Management of Patients with IgA Deficiency and Anti-IgA during Liver Transplantation

Severe anaphylactic or allergic reactions may occur during blood transfusion to patients who are IgA-deficient and have anti-IgA in their blood, particularly those with class-specific antibodies. These patients are a particular challenge to the hospital transfusion service when large volumes of blood components are required for transfusion support, as in liver transplantation. We have successfully provided blood components for 3 such patients undergoing liver transplantation. Red cells were washed manually or by automated technique. Platelets were washed manually. All plasma was from IgA-deficient donors. One patient's entire plasma requirements were supplied by autologous plasmapheresis. Serial determinations of IgA levels and anti-IgA titers in 1 patient demonstrated an abrupt fall in anti-IgA with the appearance of barely detectable amounts of IgA during the surgery. IgA-containing plasma cells were demonstrated in the biopsies of liver homografts of 2 patients following transplantation. IgA deficiency with anti-IgA can be successfully managed during liver transplantation with advance planning.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/74856/1/j.1423-0410.1992.tb01229.x.pd

Evaluation and treatment of resistant hypertension

Hypertension is a major cause and contributor to stroke, heart and kidney disease. Despite the development of an arsenal of medication to treat hypertension over the past half-century, adequate treatment continues to be a major problem in the United States. The Third National Health and Nutrition Examination Survey (NHANES-III) shows that only 29% of hypertensive patients reach a blood pressure less than 140/90 mm Hg. Resistant hypertension is defined as a blood pressure greater than 140/90 mm Hg despite a rational combination of three or more blood pressure medications including a diuretic. The prevalence of true resistant hypertension in hypertension clinics is only about 11-13%. Higher prevalence rates are evident in populations with evidence of end-organ disease such as cardiac or renal disease where lower blood pressure targets have now been established. Ascertaining the possible cause(s) for resistant hypertension is a challenge to all clinicians, but critical in eventual determination of a therapeutic solution. The following review will hopefully help guide clinicians in their discernment of causes and potential treatments for resistant hypertension. The diagnosis and treatment of the more common secondary causes will be described and treatment options for patients with resistant hypertension are discussed. Newer options, some still under clinical investigation, will be described and their future utility will be discussed. (Cardiol J 2007; 14: 329-339

Are Parent Activation and Health Literacy Distinct Concepts? A Study in Low Income Urban Populations

Patient activation (the knowledge, confidence, willingness, and skills to manage one’s healthcare) and health literacy have well-established associations with health and healthcare outcomes in adults. However, little is known about parent activation on behalf of children and its relation to health literacy. Our objective was to examine relations between parent activation, health literacy, and parent-provider relationship quality. We surveyed 316 Spanish- or English-speaking parents of publicly-insured patients of a general pediatrics clinic. Surveys included the Parent-Patient Activation Measure (P-PAM), the Newest Vital Sign (NVS), and parent-provider relationship measures. We used chi-square analyses and logistic regression to explore associations stratified by survey language. Spanish-speaking parents had significantly lower levels of both parent activation and health literacy compared with English-speaking parents (

The Role of Corticothalamic Projections (Prelimbic Cortex to Nucleus Reuniens) in Working Memory

Working memory (WM) is the ability to store information for short periods of time and is used to execute tasks WM has been understood to work via the medial prefrontal cortex (mPFC) and dorsal hippocampus (dHPC), but they do not directly project to each other The nucleus reuniens of the thalamus (Re) is a “middle man” between the mPFC and dHPC There are projections between the prelimbic cortex (PrL) and Re that may be used during WM To test the connection of the PrL to Re, a delayed nonmatch to position (DNMTP) task was performe

Diagnostyka i leczenie nadciśnienia opornego

Nadciśnienie tętnicze jest jedną z głównych przyczyn udarów mózgu, chorób serca i nerek. Mimo że w ciągu ostatniego półwiecza opracowano wiele leków hipotensyjnych, odpowiednie leczenie nadciśnienia pozostaje dużym problemem. W badaniu NHANES III wykazano, że tylko u 29% pacjentów z nadciśnieniem tętniczym w Stanach Zjednoczonych uzyskuje się ciśnienie mniejsze niż 140/90 mm Hg. Nadciśnienie oporne definiuje się jako ciśnienie utrzymujące się na poziomie powyżej 140/90 mm Hg pomimo stosowania racjonalnego połączenia 3 lub więcej leków hipotensyjnych, w tym diuretyku. Częstość występowania rzeczywistego nadciśnienia opornego w specjalistycznych ośrodkach leczenia nadciśnienia tętniczego wynosi jedynie około 11-13%. Większą częstość występowania stwierdza się w populacjach osób z powikłaniami narządowymi, takimi jak choroby serca lub nerek, u których przyjęto obecnie niższe docelowe wartości ciśnienia. Określenie możliwej przyczyny (lub przyczyn) nadciśnienia opornego jest wyzwaniem dla wszystkich klinicystów, ale ma zasadnicze znaczenie w ostatecznym wyborze rozwiązania terapeutycznego. Celem niniejszej pracy jest ułatwienie klinicystom wykrywania przyczyn i określania potencjalnych sposobów terapii nadciśnienia opornego. Przedstawiono diagnostykę i leczenie częstszych postaci wtórnego nadciśnienia, a także możliwości terapii nadciśnienia opornego. Uwzględniono również nowsze metody, z których część jest wciąż w fazie badań klinicznych i omówiono ich przydatność w przyszłości. (Folia Cardiologica Excerpta 2007; 2: 511-523

A patient with glycogen storage disease type Ib presenting with acute myeloid leukemia (AML) bearing monosomy 7 and translocation t(3;8)(q26;q24) after 14 years of treatment with granulocyte colony-stimulating factor (G-CSF): A case report

<p>Abstract</p> <p>Introduction</p> <p>Glycogen storage disease type Ib is an autosomal recessive transmitted disorder of glycogen metabolism caused by mutations in the glucose-6-phosphate translocase gene on chromosome 11q23 and leads to disturbed glycogenolysis as well as gluconeogenesis. Besides hepatomegaly, growth retardation, hypoglycemia, hyperlactatemia, hyperuricemia and hyperlipidemia, patients suffer from neutropenia associated with functional defects predisposing for severe infections. In order to attenuate these complications, long-term treatment with granulocyte colony-stimulating factor is common but this is associated with an increased risk for acute myeloid leukemia or myelodysplastic syndromes in patients with inherited bone marrow failures such as severe congenital neutropenia. Onset of these myeloid malignancies is linked to cytogenetic aberrations involving chromosome 7. In addition, granulocyte colony-stimulating factor is known to stimulate proliferation of monosomy 7 cells <it>in vitro</it>. To our knowledge, we report for the first time a case report of a patient with glycogen storage disease type Ib, who developed acute myeloid leukemia with a classical monosomy 7 and acute myeloid leukemia-associated translocation t(3;8)(q26;q24) after 14 years of continuous treatment with granulocyte colony-stimulating factor.</p> <p>Case presentation</p> <p>A 28-year-old Turkish man with glycogen storage disease type Ib was admitted to our department because of dyspnea and increasing fatigue. He also presented with gum bleeding, bone pain in his legs, night sweats, recurrent episodes of fever with temperatures up to 39°C and hepatosplenomegaly.</p> <p>A blood count taken on the day of admission showed pancytopenia and a differential count displayed 30% blasts. A bone marrow biopsy was taken which showed a hypercellular marrow with dysplastic features of all three cell lines, while blast count was 20%. Classical cytogenetic analyses as well as fluorescence in situ hybridization showed a monosomy 7 with a translocation t(3;8)(q26;q24). Based on these findings, the diagnosis of acute myeloid leukemia was made.</p> <p>Conclusion</p> <p>Our observations suggest that bone marrow examinations including cytogenetic analysis should be carried out on a regular basis in patients with glycogen storage disease type Ib who are on long-term treatment with granulocyte colony-stimulating factor for severe neutropenia, since this treatment might also contribute to an increased risk for acute myeloid leukemia or myelodysplastic syndromes.</p

Icodextrin Versus Glucose Solutions for the Once-Daily Long Dwell in Peritoneal Dialysis: An Enriched Systematic Review and Meta-analysis of Randomized Controlled Trials

Rationale & Objective The efficacy and safety of icodextrin versus glucose-only peritoneal dialysis (PD) regimens is unclear. The aim of this study was to compare once-daily long-dwell icodextrin versus glucose among patients with kidney failure undergoing PD. Study Design Systematic review of randomized controlled trials (RCTs), enriched with unpublished data from investigator-initiated and industry-sponsored studies. Setting & Study Populations Individuals with kidney failure receiving regular PD treatment enrolled in clinical trials of dialysate composition. Selection Criteria for Studies Medline, Embase, CENTRAL, Ichushi Web, 10 Chinese databases, clinical trials registries, conference proceedings, and citation lists from inception to November 2018. Further data were obtained from principal investigators and industry clinical study reports. Data Extraction 2 independent reviewers selected studies and extracted data using a prespecified extraction instrument. Analytic Approach Qualitative synthesis of demographics, measurement scales, and outcomes. Quantitative synthesis with Mantel-Haenszel risk ratios (RRs), Peto odds ratios (ORs), or (standardized) mean differences (MDs). Risk of bias of included studies at the outcome level was assessed using the Cochrane risk-of-bias tool for RCTs. Results 19 RCTs that enrolled 1,693 participants were meta-analyzed. Ultrafiltration was improved with icodextrin (medium-term MD, 208.92 [95% CI, 99.69-318.14] mL/24 h; high certainty of evidence), reflected also by fewer episodes of fluid overload (RR, 0.43 [95% CI, 0.24-0.78]; high certainty). Icodextrin-containing PD probably decreased mortality risk compared to glucose-only PD (Peto OR, 0.49 [95% CI, 0.24-1.00]; moderate certainty). Despite evidence of lower peritoneal glucose absorption with icodextrin-containing PD (medium-term MD, −40.84 [95% CI, −48.09 to −33.59] g/long dwell; high certainty), this did not directly translate to changes in fasting plasma glucose (−0.50 [95% CI, −1.19 to 0.18] mmol/L; low certainty) and hemoglobin A1c levels (−0.14% [95% CI, −0.34% to 0.05%]; high certainty). Safety outcomes and residual kidney function were similar in both groups; health-related quality-of-life and pain scores were inconclusive. Limitations Trial quality was variable. The follow-up period was heterogeneous, with a paucity of assessments over the long term. Mortality results are based on just 32 events and were not corroborated using time-to-event analysis of individual patient data. Conclusions Icodextrin for once-daily long-dwell PD has clinical benefit for some patients, including those not meeting ultrafiltration targets and at risk for fluid overload. Future research into patient-centered outcomes and cost-effectiveness associated with icodextrin is needed

IPSS-independent prognostic value of plasma CXCL10, IL-7 and IL-6 levels in myelodysplastic syndromes

Recent studies suggest a powerful prognostic value for plasma cytokine levels in primary myelofibrosis (interleukin (IL)-2R, IL-8, IL-12, IL-15 and C–X–C motif chemokine 10 (CXCL10)) and large-cell lymphoma (IL-2R, IL-8, IL-10, IL-12, CXCL9 and CXCL10). To examine the possibility of a similar phenomenon in myelodysplastic syndromes (MDS), we used multiplex enzyme-linked immunosorbent assay to measure 30 plasma cytokines in 78 patients with primary MDS. Compared with normal controls (n=35), the levels of 19 cytokines were significantly altered. Multivariable analysis identified increased levels of CXCL10 (P<0.01), IL-7 (P=0.02) and IL-6 (P=0.07) as predictors of shortened survival; the survival association remained significant when the Cox model was adjusted for the International Prognostic Scoring System, age, transfusion-need or thrombocytopenia. MDS patients with normal plasma levels of CXCL10, IL-7 and IL-6 lived significantly longer (median survival 76 months) than those with elevated levels of at least one of the three cytokines (median survival 25 months) (P<0.01). Increased levels of IL-6 were associated with inferior leukemia-free survival, independent of other prognostic factors (P=0.01). Comparison of plasma cytokines between MDS (n=78) and primary myelofibrosis (n=127) revealed a significantly different pattern of abnormalities. These observations reinforce the concept of distinct and prognostically relevant plasma cytokine signatures in hematological malignancies