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Nature Reviews. Disease Primers Apr 2018Haemochromatosis is defined as systemic iron overload of genetic origin, caused by a reduction in the concentration of the iron regulatory hormone hepcidin, or a... (Review)
Review
Haemochromatosis is defined as systemic iron overload of genetic origin, caused by a reduction in the concentration of the iron regulatory hormone hepcidin, or a reduction in hepcidin-ferroportin binding. Hepcidin regulates the activity of ferroportin, which is the only identified cellular iron exporter. The most common form of haemochromatosis is due to homozygous mutations (specifically, the C282Y mutation) in HFE, which encodes hereditary haemochromatosis protein. Non-HFE forms of haemochromatosis due to mutations in HAMP, HJV or TFR2 are much rarer. Mutations in SLC40A1 (also known as FPN1; encoding ferroportin) that prevent hepcidin-ferroportin binding also cause haemochromatosis. Cellular iron excess in HFE and non-HFE forms of haemochromatosis is caused by increased concentrations of plasma iron, which can lead to the accumulation of iron in parenchymal cells, particularly hepatocytes, pancreatic cells and cardiomyocytes. Diagnosis is noninvasive and includes clinical examination, assessment of plasma iron parameters, imaging and genetic testing. The mainstay therapy is phlebotomy, although iron chelation can be used in some patients. Hepcidin supplementation might be an innovative future approach.
Topics: Cation Transport Proteins; Chelation Therapy; Hemochromatosis; Humans; Iron; Magnetic Resonance Imaging; Mass Screening; Phlebotomy; Polymorphism, Genetic; Quality of Life
PubMed: 29620054
DOI: 10.1038/nrdp.2018.16 -
Journal of Acupuncture and Meridian... Jun 2018Cupping therapy is an ancient traditional and complementary medicine practice. Recently, there is growing evidence of its potential benefits in the treatment of... (Review)
Review
Cupping therapy is an ancient traditional and complementary medicine practice. Recently, there is growing evidence of its potential benefits in the treatment of pain-related diseases. This article gives an overview of cupping therapy practice. Furthermore, this article suggests a new classification of cupping therapy sets, a new classification of cupping therapy adverse events, and an updated classification of cupping therapy types.
Topics: Acupuncture Therapy; Bloodletting; Humans; Medicine, Chinese Traditional
PubMed: 29436369
DOI: 10.1016/j.jams.2018.02.001 -
American Family Physician Jun 2021Polycythemia vera is one of three stem-cell-derived myeloid malignancies commonly known as myeloproliferative neoplasms. It is characterized by erythrocytosis, often... (Review)
Review
Polycythemia vera is one of three stem-cell-derived myeloid malignancies commonly known as myeloproliferative neoplasms. It is characterized by erythrocytosis, often with associated leukocytosis and thrombocytosis. It has a significant negative impact on overall mortality and morbidity in the form of arterial and venous clots, symptoms of fatigue and pruritus, and conversion to leukemia and myelofibrosis. The World Health Organization's major diagnostic criteria include an elevated hemoglobin or hematocrit level, abnormal results on bone marrow biopsy, and presence of the Janus kinase 2 genetic mutation, which is present in approximately 98% of cases. The only minor criterion is a subnormal erythropoietin level, which helps differentiate polycythemia vera from common causes of secondary erythrocytosis such as smoking, sleep apnea, and testosterone use. First-line treatments, such as low-dose aspirin and goal-directed phlebotomy to a hematocrit level of less than 45% to reduce thrombotic events, improve quality of life and prolong survival. When indicated, cytoreductive therapy, primarily with hydroxyurea, can be added with consideration of second-line agents such as pegylated interferon-alfa, busulfan, and ruxolitinib, depending on the clinical scenario. Smoking cessation and cardiometabolic disease are modifiable risk factors that should be addressed to reduce the risk of thrombosis. Currently, no medications have been shown to cure the disease or to reduce the risk of conversion to leukemia and myelofibrosis.
Topics: Antineoplastic Agents; Fibrinolytic Agents; Genetic Markers; Humans; Hydroxyurea; Janus Kinase 2; Mutation; Phlebotomy; Polycythemia Vera
PubMed: 34060791
DOI: No ID Found -
Archives of Pathology & Laboratory... Jun 2022Laboratory directors are tasked with staffing laboratories in a manner that provides adequate services and maintains economic sustainability.
CONTEXT.—
Laboratory directors are tasked with staffing laboratories in a manner that provides adequate services and maintains economic sustainability.
OBJECTIVE.—
To determine the national normative rates of phlebotomy staffing and the types of laboratory operational characteristics that may be associated with the magnitude of those staffing levels.
DESIGN.—
Study participants provided data on inpatient and outpatient phlebotomy sites, including the numbers of patients receiving phlebotomy services, phlebotomy staff, and billable tests. From these data, we calculated performance indicators including the numbers of phlebotomies/phlebotomy full-time equivalent staff, outpatient phlebotomy visits/full-time equivalent staff, and average outpatient phlebotomy wait times. Participants also completed a survey of their laboratory phlebotomy practices.
RESULTS.—
This study was conducted during the third quarter of 2017. Forty-two institutions participated in this study, providing eligible results for 40 selected inpatient sites and 70 selected outpatient sites. The ratios for all performance indicators spanned between 3.3- and 142-fold. The median average outpatient phlebotomy wait time was 8 minutes. None of the performance indicators were associated with the practice variables that we chose to test.
CONCLUSIONS.—
The distribution of phlebotomy staffing performance indicators among the laboratories participating in this study varied widely, even among those groups performing similar volumes of tests.
Topics: Humans; Laboratories; Phlebotomy; Surveys and Questionnaires; Workforce
PubMed: 34784416
DOI: 10.5858/arpa.2021-0158-CP -
American Journal of Hematology Mar 2021Pregnancy in the context of myeloproliferative neoplasms (MPN) poses unique fetal and maternal challenges. Current literature in this regard mostly involves essential... (Review)
Review
Pregnancy in the context of myeloproliferative neoplasms (MPN) poses unique fetal and maternal challenges. Current literature in this regard mostly involves essential thrombocythemia (ET) and less so polycythemia vera (PV) or myelofibrosis. In ET, live birth rate is estimated at 70% with first trimester fetal loss (˜ 30%) as the major complication. Risk of pregnancy-associated complications is higher in PV, thus mandating a more aggressive treatment approach. Herein, we appraise the relevant literature, share our own experience and propose management recommendations. Aspirin therapy may offer protection against fetal loss; however the additive benefit of systemic anticoagulation or cytoreductive therapy, in the absence of high risk disease, is unclear. We recommend cytoreductive therapy in the form of interferon alpha in all high risk and select low-risk ET and PV patients with history of recurrent fetal loss, prominent splenomegaly or suboptimal hematocrit control with phlebotomy. In addition, all women with PV should maintain strict hematocrit control <45% with the aid of phlebotomy. Systemic anticoagulation with low molecular weight heparin is advised in patients with history of venous thrombosis. Further clarification awaits prospective clinical trials that implement risk adapted therapeutic interventions.
Topics: Abortion, Habitual; Abortion, Spontaneous; Anticoagulants; Aspirin; Combined Modality Therapy; Female; Heparin, Low-Molecular-Weight; Humans; Infant, Low Birth Weight; Infant, Newborn; Interferon-alpha; Live Birth; Multicenter Studies as Topic; Mutation; Myeloproliferative Disorders; Phlebotomy; Platelet Count; Practice Guidelines as Topic; Preconception Care; Pregnancy; Pregnancy Complications; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Prenatal Care; Puerperal Disorders; Retrospective Studies; Thrombophilia; Venous Thrombosis
PubMed: 33296529
DOI: 10.1002/ajh.26067 -
The Cochrane Database of Systematic... Dec 2015Infant acute pain and distress is commonplace. Infancy is a period of exponential development. Unrelieved pain and distress can have implications across the lifespan. ... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Infant acute pain and distress is commonplace. Infancy is a period of exponential development. Unrelieved pain and distress can have implications across the lifespan. This is an update of a previously published review in the Cochrane Database of Systematic Reviews, Issue 10 2011 entitled 'Non-pharmacological management of infant and young child procedural pain'.
OBJECTIVES
To assess the efficacy of non-pharmacological interventions for infant and child (up to three years) acute pain, excluding kangaroo care, and music. Analyses were run separately for infant age (preterm, neonate, older) and pain response (pain reactivity, immediate pain regulation).
SEARCH METHODS
For this update, we searched the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library (Issue 2 of 12, 2015), MEDLINE-Ovid platform (March 2015), EMBASE-OVID platform (April 2011 to March 2015), PsycINFO-OVID platform (April 2011 to February 2015), and CINAHL-EBSCO platform (April 2011 to March 2015). We also searched reference lists and contacted researchers via electronic list-serves. New studies were incorporated into the review. We refined search strategies with a Cochrane-affiliated librarian. For this update, nine articles from the original 2011 review pertaining to Kangaroo Care were excluded, but 21 additional studies were added.
SELECTION CRITERIA
Participants included infants from birth to three years. Only randomised controlled trials (RCTs) or RCT cross-overs that had a no-treatment control comparison were eligible for inclusion in the analyses. However, when the additive effects of a non-pharmacological intervention could be assessed, these studies were also included. We examined studies that met all inclusion criteria except for study design (e.g. had an active control) to qualitatively contextualize results. There were 63 included articles in the current update.
DATA COLLECTION AND ANALYSIS
Study quality ratings and risk of bias were based on the Cochrane Risk of Bias Tool and GRADE approach. We analysed the standardized mean difference (SMD) using the generic inverse variance method.
MAIN RESULTS
Sixty-three studies, with 4905 participants, were analysed. The most commonly studied acute procedures were heel-sticks (32 studies) and needles (17 studies). The largest SMD for treatment improvement over control conditions on pain reactivity were: non-nutritive sucking-related interventions (neonate: SMD -1.20, 95% CI -2.01 to -0.38) and swaddling/facilitated tucking (preterm: SMD -0.89; 95% CI -1.37 to -0.40). For immediate pain regulation, the largest SMDs were: non-nutritive sucking-related interventions (preterm: SMD -0.43; 95% CI -0.63 to -0.23; neonate: SMD -0.90; 95% CI -1.54 to -0.25; older infant: SMD -1.34; 95% CI -2.14 to -0.54), swaddling/facilitated tucking (preterm: SMD -0.71; 95% CI -1.00 to -0.43), and rocking/holding (neonate: SMD -0.75; 95% CI -1.20 to -0.30). Fifty two of our 63 trials did not report adverse events. The presence of significant heterogeneity limited our confidence in the findings for certain analyses, as did the preponderance of very low quality evidence.
AUTHORS' CONCLUSIONS
There is evidence that different non-pharmacological interventions can be used with preterms, neonates, and older infants to significantly manage pain behaviors associated with acutely painful procedures. The most established evidence was for non-nutritive sucking, swaddling/facilitated tucking, and rocking/holding. All analyses reflected that more research is needed to bolster our confidence in the direction of the findings. There are significant gaps in the existing literature on non-pharmacological management of acute pain in infancy.
Topics: Acute Disease; Acute Pain; Child, Preschool; Heel; Humans; Immunization; Infant; Infant Care; Infant, Newborn; Infant, Premature; Needles; Pain Management; Phlebotomy; Punctures; Randomized Controlled Trials as Topic; Sucking Behavior
PubMed: 26630545
DOI: 10.1002/14651858.CD006275.pub3 -
Current Opinion in Hematology Mar 2023Development of hepcidin therapeutics has been a ground-breaking discovery in restoring iron homeostasis in several haematological disorders. The hepcidin mimetic,... (Review)
Review
PURPOSE OF REVIEW
Development of hepcidin therapeutics has been a ground-breaking discovery in restoring iron homeostasis in several haematological disorders. The hepcidin mimetic, rusfertide, is in late-stage clinical development for treating polycythemia vera patients with a global phase 3 trial [NCT05210790] currently underway. Rusfertide serves as the first possible noncytoreductive therapeutic option to maintain haematocrit control and avoid phlebotomy in polycythemia vera patients. In this comprehensive review, we discuss the pathobiology of dysregulated iron metabolism in polycythemia vera, provide the rationale for targeting the hepcidin-ferroportin axis and elaborate on the preclinical and clinical trial evidence supporting the role of hepcidin mimetics in polycythemia vera.
RECENT FINDINGS
Recently, updated results from two phase 2 clinical trials [NCT04057040 & NCT04767802] of rusfertide (PTG300) demonstrate that the drug is highly effective in eliminating the need for therapeutic phlebotomies, normalizing haematological parameters, repleting iron stores and relieving constitutional symptoms in patients with polycythemia vera. In light of these findings, additional hepcidin mimetic agents are also being evaluated in polycythemia vera patients.
SUMMARY
Hepcidin agonists essentially serve as a 'chemical phlebotomy' and are poised to vastly improve the quality of life for phlebotomy requiring polycythemia vera patients.
Topics: Humans; Polycythemia Vera; Polycythemia; Hepcidins; Quality of Life; Iron Deficiencies; Phlebotomy; Iron
PubMed: 36728649
DOI: 10.1097/MOH.0000000000000747 -
The Cochrane Database of Systematic... Feb 2017Skin-to-skin care (SSC), often referred to as 'kangaroo care' (KC) due to its similarity with marsupial behaviour of ventral maternal-infant contact, is one... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Skin-to-skin care (SSC), often referred to as 'kangaroo care' (KC) due to its similarity with marsupial behaviour of ventral maternal-infant contact, is one non-pharmacological intervention for pain control in infants.
OBJECTIVES
The primary objectives were to determine the effect of SSC alone on pain from medical or nursing procedures in neonates compared to no intervention, sucrose or other analgesics, or additions to simple SSC such as rocking; and to determine the effects of the amount of SSC (duration in minutes), method of administration (e.g. who provided the SSC) of SSC in reducing pain from medical or nursing procedures in neonatesThe secondary objectives were to determine the safety of SSC care for relieving procedural pain in infants; and to compare the SSC effect in different postmenstrual age subgroups of infants.
SEARCH METHODS
For this update, we used the standard search strategy of the Cochrane Neonatal Review group to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1); MEDLINE via PubMed (1966 to 25 February 2016); Embase (1980 to 25 February 2016); and CINAHL (1982 to 25 February 2016). We also searched clinical trials' databases, conference proceedings, and the reference lists of retrieved articles for randomized controlled trials and quasi-randomized trials.
SELECTION CRITERIA
Studies with randomisation or quasi-randomisation, double- or single-blinded, involving term infants (≥ 37 completed weeks' postmenstrual age (PMA) to a maximum of 44 weeks' PMA and preterm infants (< 37 completed weeks PMA) receiving SSC for painful procedures conducted by healthcare professionals.
DATA COLLECTION AND ANALYSIS
The main outcome measures were physiological or behavioural pain indicators and composite pain scores. A mean difference (MD) with 95% confidence interval (CI) using a fixed-effect model was reported for continuous outcome measures. We included variations on type of tissue-damaging procedure, provider of care, and duration of SSC.
MAIN RESULTS
Twenty-five studies (n = 2001 infants) were included. Nineteen studies (n = 1065) used heel lance as the painful procedure, one study combined venepuncture and heel stick (n = 50), three used intramuscular injection (n = 776), one used 'vaccination' (n = 60), and one used tape removal (n = 50). The studies were generally strong and had low or uncertain risk of bias. Blinding of the intervention was not possible, making them subject to high risk, depending on the method of scoring outcomes.Seventeen studies (n = 810) compared SSC to a no-treatment control. Although 15 studies measured heart rate during painful procedures, data from only five studies (n = 161) could be combined for a mean difference (MD) of -10.78 beats per minute (95% CI -13.63 to -7.93) favouring SSC. Meta-analysis of four studies (n = 120) showed no difference in heart rate following the painful procedure (MD 0.08, 95% CI -4.39 to 4.55). Two studies (n = 38) reported heart rate variability with no significant differences. Two studies (n = 101) in a meta-analysis on oxygen saturation at 30 and 60 seconds following the painful procedure did not show a difference. Duration of crying meta-analysis was performed on four studies (n = 133): two (n = 33) investigated response to heel lance (MD = -34.16, 95% CI -42.86 to -25.45), and two (n = 100) following IM injection (MD = -8.83, 95% CI -14.63 to -3.02), favouring SSC. Five studies, one consisting of two substudies (n = 267), used the Premature Infant Pain Profile (PIPP) as a primary outcome, which favoured SCC at 30 seconds (MD -3.21, 95% CI -3.94 to -2.47), at 60 seconds (3 studies; n = 156) (MD -1.64, 95% CI -2.86 to -0.43), and at 90 seconds (n = 156) (MD -1.28, 95% CI -2.53 to -0.04); but at 120 seconds there was no difference (n = 156) (MD 0.07, 95% CI -1.11 to 1.25). No studies on return of heart rate to baseline level, cortisol levels, and facial actions could be combined for meta-analysis findings.Eight studies compared SSC to another intervention with or without a no-treatment control. Two cross-over studies (n = 80) compared mother versus other provider (father, another female) on PIPP scores at 30, 60, 90, and 120 seconds with no significant difference. When SSC was compared to other interventions, there were not enough similar studies to pool results in an analysis. One study compared SSC (n = 640) with and without dextrose and found that the combination was most effective and that SSC alone was more effective than dextrose alone. Similarly, in another study SSC was more effective than oral glucose for heart rate (n = 95). SSC either in combination with breastfeeding or alone was favoured over a no-treatment control, but not different to breastfeeding. One study compared SSC alone and in combination with both sucrose and breastfeeding on heart rate (HR), NIPS scores, and crying time (n = 127). The combinations were more effective than SSC alone for NIPS and crying. Expressed breast milk was compared to SSC in one study (n = 50) and found both equally effective on PIPP scores. There were not enough participants with similar outcomes and painful procedures to compare age groups or duration of SSC. No adverse events were reported in any of the studies.
AUTHORS' CONCLUSIONS
SSC appears to be effective as measured by composite pain indicators with both physiological and behavioural indicators and, independently, using heart rate and crying time; and safe for a single painful procedure. Purely behavioural indicators tended to favour SSC but with facial actions there is greater possibility of observers not being blinded. Physiological indicators were mixed although the common measure of heart rate favoured SSC. Two studies compared mother-providers to others, with non-significant results. There was more heterogeneity in the studies with behavioural or composite outcomes. There is a need for replication studies that use similar, clearly defined outcomes. Studies examining optimal duration of SSC, gestational age groups, repeated use, and long-term effects of SSC are needed. Of interest would be to study synergistic effects of SSC with other interventions.
Topics: Breast Feeding; Heart Rate; Humans; Hydrocortisone; Infant, Newborn; Infant, Premature; Injections, Intramuscular; Kangaroo-Mother Care Method; Oxygen Consumption; Pain Management; Phlebotomy; Punctures; Randomized Controlled Trials as Topic; Saliva; Term Birth
PubMed: 28205208
DOI: 10.1002/14651858.CD008435.pub3 -
Diagnosis (Berlin, Germany) Mar 2019It has been well documented over recent years that the preanalytical phase is a leading contributor to errors in the total testing process (TTP). There has however been... (Review)
Review
It has been well documented over recent years that the preanalytical phase is a leading contributor to errors in the total testing process (TTP). There has however been great progress made in recent years due to the exponential growth of working groups specialising in the field. Patient safety is clearly at the forefront of any healthcare system and any reduction in errors at any stage will improve patient safety. Venous blood collection is a key step in the TTP, and here we review the key errors that occur in venous phlebotomy process and summarise the evidence around their significance to patient safety. Recent studies have identified that patient identification and tube labelling are the steps that carry the highest risk with regard to patient safety. Other studies have shown that in 16.1% of cases, patient identification is incorrectly performed and that 56% of patient identification errors are due to poor labelling practice. We recommend that patient identification must be done using open questions and ideally three separate pieces of information. Labelling of the tube or linking the identity of the patient to the tube label electronically must be done in the presence of the patient whether it is before or after sampling. Combined this will minimise any chance of patient misidentification.
Topics: Humans; Patient Identification Systems; Patient Safety; Phlebotomy; Pre-Analytical Phase; Quality Control; Veins
PubMed: 30315735
DOI: 10.1515/dx-2018-0042 -
JAMA Network Open Feb 2023Venipuncture is one of the most painful and distressing procedures experienced by pediatric patients. Emerging evidence suggests that providing procedural information... (Randomized Controlled Trial)
Randomized Controlled Trial
IMPORTANCE
Venipuncture is one of the most painful and distressing procedures experienced by pediatric patients. Emerging evidence suggests that providing procedural information and distraction using immersive virtual reality (IVR) may reduce pain and anxiety among children undergoing needle-related procedures.
OBJECTIVES
To examine the effects of IVR on reducing the pain, anxiety, and stress experienced by pediatric patients undergoing venipuncture.
DESIGN, SETTING, AND PARTICIPANTS
This 2-group randomized clinical trial recruited pediatric patients aged 4 to 12 years undergoing venipuncture from a public hospital in Hong Kong between January 2019 and January 2020. Data were analyzed from March to May 2022.
INTERVENTIONS
Participants were randomly allocated to an intervention (an age-appropriate IVR intervention offering distraction and procedural information) or a control (standard care only) group.
MAIN OUTCOMES AND MEASURES
The primary outcome was child-reported pain. Secondary outcomes included child-reported anxiety, heart rate, salivary cortisol, length of procedure, and satisfaction of health care professionals with the procedure (rated on a 40 point scale, with higher scores indicating greater satisfaction). Outcomes were assessed 10 minutes before, during, immediately after, and 30 minutes after the procedure.
RESULTS
A total of 149 pediatric patients were recruited, with 86 female patients (57.7%) and 66 patients (44.3%) diagnosed with fever. Compared with the 74 participants in the control group (mean [SD] age, 7.21 [2.49] years), the 75 participants in the IVR group (mean [SD] age, 7.21 [2.43] years) reported significantly less pain (β = -0.78; 95% CI, -1.21 to -0.35; P < .001) and anxiety (β = -0.41; 95% CI, -0.76 to -0.05; P = .03) immediately after the intervention. Health care professional satisfaction in the IVR group (mean [SD] score, 34.5 [4.5]) was significantly higher than that in the control group (mean [SD] score, 32.9 [4.0]; P = .03). Moreover, the length of venipuncture procedure in the IVR group (mean [SD] duration, 4.43 [3.47] minutes) was significantly shorter than that in the control group (mean [SD] duration, 6.56 [7.39] minutes; P = .03).
CONCLUSIONS AND RELEVANCE
In this randomized clinical trial, integrating procedural information and distraction in an IVR intervention for pediatric patients undergoing venipuncture significantly improved pain and anxiety in the IVR group compared with the control group. The results shed light on the global trends of research on IVR and its clinical development as an intervention for other painful and stressful medical procedures.
TRIAL REGISTRATION
Chinese Clinical Trial Registry identifier: ChiCTR1800018817.
Topics: Child; Humans; Female; Phlebotomy; Virtual Reality Exposure Therapy; Pain, Procedural; Pain; Anxiety
PubMed: 36795410
DOI: 10.1001/jamanetworkopen.2023.0001