-
1.
The first Chinese case of unstable Hemoglobin Santa Ana detected by capillary electrophoresis: a case report and literature review.
Du, L, Qin, D, Wang, J, Yao, C, Zhu, J, Guo, H, Yuan, T, Liang, J, Yin, A
Hematology (Amsterdam, Netherlands). 2022;(1):258-262
Abstract
Hemoglobin Santa Ana [β88(F4)Leu→Pro (CTG > CCG) HBB: c.266T > C] is an unstable hemoglobin variant characterized by a substitution of the amino acid leucine by proline at the 88th position of the β-globin chain. We for the first time identified this hemoglobin variant in a Chinese patient by capillary electrophoresis (CE). The proband was an 8-year-old boy with chronic anemia, brown urine and splenomegaly. He had been affected by moderate anemia, twice approaching a severe degree, that was attributed to infection. The CE result revealed an abnormal hemoglobin peak at electrophoretic zone 4 that correspond to the hemoglobin Santa Ana peak, and a CTG > CCG mutation at codon 88 of the β-globin gene was confirmed by DNA sequencing. To avoid misdiagnosis and genetic risks, a literature review of other unstable hemoglobins that migrate similarly to the hemoglobin Santa Ana was performed. Our findings indicate that hemoglobin Santa Ana can be clearly separated by CE, with accurate diagnosis depending on molecular analysis. This information will be useful for providing appropriate genetic counselling and for prenatal diagnosis.
-
2.
Cytopenias among patients with rheumatic diseases using methotrexate: a meta-analysis of randomized controlled clinical trials.
Vanni, KMM, Lyu, H, Solomon, DH
Rheumatology (Oxford, England). 2020;(4):709-717
-
-
Free full text
-
Abstract
OBJECTIVE To conduct a systematic literature review and meta-analysis to estimate the incidence of anaemia, leucopoenia, neutropenia and thrombocytopenia associated with MTX plus folic acid among patients with rheumatic diseases. METHODS We searched MEDLINE, PubMed and EMBASE through August 2016 for all randomized controlled clinical trials with a MTX monotherapy arm. We excluded randomized controlled clinical trials for cancer and included only double-blind studies that reported on haematologic adverse events. Studies were excluded if patients did not receive folic acid or leucovorin supplementation. Full text articles were assessed by two independent reviewers. Incidence estimates were calculated using random-effects models. RESULTS Of 1601 studies identified, 30 (1.87%) were included, representing 3858 patients; all had RA. Seventeen trials reported on anaemia (n = 2032), 17 reported on leucopoenia (n = 2220), 16 reported on neutropenia (n = 2202) and 12 reported on thrombocytopenia (n = 1507). The incidence for any anaemia was 2.55% (95% CI 0.60-5.47%), any leucopoenia 1.17% (95% CI 0.16-2.80%), any neutropenia 1.77% (95% CI 0.33-4.00%), and any thrombocytopenia 0.19% (95% CI 0.00-0.86%). Four cases of severe anaemia were reported, as defined by authors, along with three cases of severe neutropenia. No cases of severe leucopoenia, severe thrombocytopenia or pancytopenia were reported. CONCLUSION Cytopenias are an uncommon side effect of low-dose MTX with folic acid supplementation among RA patients. Further research is needed to reach a more precise estimate.
-
3.
Transfusion-related Gut Injury and Necrotizing Enterocolitis.
Rose, AT, Saroha, V, Patel, RM
Clinics in perinatology. 2020;(2):399-412
-
-
Free full text
-
Abstract
Necrotizing enterocolitis (NEC) accounts for 10% of deaths in neonatal intensive care units. Several causal mechanisms are likely to lead to a final common disease phenotype. This article summarizes recent data on NEC following red blood cell (RBC) transfusion, with a focus on the most recent literature and ongoing trials. It highlights potential mechanisms from preclinical and human physiologic studies. It also discusses the role of feeding during RBC transfusion and the risk of NEC. Ongoing randomized trials will provide important data on how liberal or conservative approaches to RBC transfusion influence the risk of NEC.
-
4.
Periprocedural anemia management in severe aortic stenosis patients undergoing transcatheter aortic valve implantation.
Cammalleri, V, Muscoli, S, Versaci, F, Romeo, F
Journal of cardiology. 2020;(2):117-123
Abstract
Preoperative anemia is a common finding in patients with severe aortic valve stenosis undergoing transcatheter aortic valve implantation (TAVI), and it has been shown to be associated with high mortality. The present review provides an overview of current management strategies of perioperative anemia in TAVI patients, including red blood cell transfusion, blood conservative protocol, iron supplementation, and erythropoietin administration. The goal is to recognize the treatable causes of anemia and treat them, in order to reduce transfusions and improve the outcome.
-
5.
Atrophic glossitis: Etiology, serum autoantibodies, anemia, hematinic deficiencies, hyperhomocysteinemia, and management.
Chiang, CP, Chang, JY, Wang, YP, Wu, YH, Wu, YC, Sun, A
Journal of the Formosan Medical Association = Taiwan yi zhi. 2020;(4):774-780
Abstract
Atrophic glossitis (AG) is characterized by the partial or complete absence of filiform papillae on the dorsal surface of the tongue. AG may reflect the significant deficiencies of some major nutrients including riboflavin, niacin, pyridoxine, vitamin B12, folic acid, iron, zinc, and vitamin E. Moreover, protein-calorie malnutrition, candidiasis, Helicobacter pylori colonization, xerostomia, and diabetes mellitus are also the etiologies of AG. Our previous study found the serum gastric parietal cell antibody (GPCA), thyroglobulin antibody (TGA), and thyroid microsomal antibody (TMA) positivities in 26.7%, 28.4%, and 29.8% of 1064 AG patients, respectively. We also found anemia, serum iron, vitamin B12, and folic acid deficiencies, and hyperhomocysteinemia in 19.0%, 16.9%, 5.3%, 2.3%, and 11.9% of 1064 AG patients, respectively. Moreover, GPCA-positive AG patients tended to have relatively higher frequencies of hemoglobin, iron, and vitamin B12 deficiencies and hyperhomocysteinemia than GPCA-negative AG patients. Supplementations with vitamin BC capsules plus corresponding deficient hematinics for those AG patients with hematinic deficiencies can achieve complete remission of oral symptoms and AG in some AG patients. Therefore, it is very important to examine the complete blood count, serum hematinic, homocysteine, and autoantibody levels in AG patients before we start to offer treatments for AG patients.
-
6.
Successful management of anemia with sirolimus in blue rubber bleb nevus syndrome: case report and update.
Fernández Gil, M, López Serrano, P, García García, E
Revista espanola de enfermedades digestivas. 2019;(8):643-647
Abstract
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder with characteristic skin hemangiomas and vascular malformations, mostly in the gastrointestinal (GI) tract. The GI lesions are mainly located in the stomach and small intestine, usually more than a hundred, leading to gastrointestinal bleeding and severe chronic anemia. Parenteral iron infusions and scheduled transfusions are frequently necessary. We describe the case of a 21-year-old male with anemia secondary to BRBNS, who becomes unresponsive to octreotide and shows an excellent response to sirolimus (SRL), dismissing the intravenous iron supplementations and being free of transfusions. During the treatment, the patient presents avascular hip necrosis, which is adequately treated with an injection of stem cells with complete recovery, and without the suspension of SRL. Two years later, adequate response persists with no other relevant side effects.
-
7.
Treating Preoperative Anemia to Improve Patient Outcomes After Orthopaedic Surgery.
Shanbhag, SP, Solano, MA, Botros, MA, Khanuja, HS
The Journal of the American Academy of Orthopaedic Surgeons. 2019;(24):e1077-e1085
Abstract
For patients undergoing orthopaedic surgery, preoperative risk modification and control of comorbidities can maximize safety and improve outcomes. Anemia is common among orthopaedic patients, and its prevalence increases with patient age. Although surgeons are well versed in intraoperative blood conservation, preoperative anemia treatment is often deferred to primary care physicians, who may not understand the importance of a thorough assessment and treatment. Orthopaedic surgeons should understand the causes and treatments of anemia to advocate that patients receive appropriate preoperative care. Mean corpuscular volume and reticulocyte count can help determine the cause of anemia and assess the bone marrow's ability to produce red blood cells. These values can be used to aid in diagnosis and treatment plans. Iron deficiency anemia, the most common type, is a microcytic anemia easily treated with iron supplementation. In cases of trauma, anemia can be related to acute blood loss and underlying conditions. Fracture patterns and preexisting comorbidities should be assessed. The role of intravenous iron supplementation in this setting has not been clearly shown. Patients needing urgent procedures that might involve substantial blood loss should receive transfusions if they have hemoglobin levels <8 g/dL or symptomatic anemia.
-
8.
Recurrent aphthous stomatitis - Etiology, serum autoantibodies, anemia, hematinic deficiencies, and management.
Chiang, CP, Yu-Fong Chang, J, Wang, YP, Wu, YH, Wu, YC, Sun, A
Journal of the Formosan Medical Association = Taiwan yi zhi. 2019;(9):1279-1289
Abstract
Recurrent aphthous stomatitis (RAS) is one of the most common oral mucosal diseases characterized by recurrent and painful ulcerations on the movable or nonkeratinized oral mucosae. Clinically, three types of RAS, namely minor, major, and herpetiform types, can be identified. RAS more commonly affects labial mucosa, buccal mucosa, and tongue. Previous studies indicate that RAS is a multifactorial T cell-mediated immune-dysregulated disease. Factors that modify the immunologic responses in RAS include genetic predisposition, viral and bacterial infections, food allergies, vitamin and microelement deficiencies, systemic diseases, hormonal imbalance, mechanical injuries, and stress. Our previous study found the presence of serum gastric parietal cell antibody, thyroglobulin antibody, and thyroid microsomal antibody in 13.0%, 19.4%, and 19.7% of 355 RAS patients, respectively. We also found anemia, serum iron, vitamin B12, and folic acid deficiencies, and hyperhomocysteinemia in 20.9%, 20.1%, 4.8%, 2.6%, and 7.7% of 273 RAS patients, respectively. Therefore, it is very important to examine the complete blood count, serum autoantibody, hematinic, and homocysteine levels in RAS patients before we start to offer treatments for RAS. Because RAS is an immunologically-mediated disease, topical and systemic corticosteroid therapies are the main treatments of choice for RAS.
-
9.
Oral iron-based interventions for prevention of critical outcomes in pregnancy and postnatal care: An overview and update of systematic reviews.
Abraha, I, Bonacini, MI, Montedori, A, Di Renzo, GC, Angelozzi, P, Micheli, M, Germani, A, Carloni, D, Scaccetti, A, Palmieri, G, et al
Journal of evidence-based medicine. 2019;(2):155-166
Abstract
OBJECTIVE The aim of this work was to summarize and update the evidence concerning oral iron-based interventions compared to placebo or no iron-based interventions to prevent critical outcomes in pregnancy or treat critical outcomes in the postpartum phase. METHOD Published systematic reviews (Feb 2018) and primary studies (from 2015 to March 2018) retrieved from MEDLINE, EMBASE, and the Cochrane Library were examined. The AMSTAR (Assessing the Methodological Quality of Systematic Reviews) tool was used to assess the quality of reviews. GRADE was used to rate the quality of the evidence for critical outcomes. RESULTS Antenatal care: Compared to placebo/no treatment, iron-based therapies reduced maternal anemia at term by 59% (seven trials at low risk of bias, RR 0.41, 95% CI 0.23-0.73; I2 = 86%; moderate-quality evidence) and maternal iron deficiency anemia by 67% (RR 0.33, 95% CI 0.16-0.69; I2 = 49%). There was no evidence of difference between iron-based therapies vs control in terms of side effects (RR 1.42, 95% CI 0.91-2.21), preterm delivery (13 studies: RR 0.93, 95% CI 0.84-1.03; low-quality evidence), low birthweight (RR 0.94, 95% CI 0.79-1.13; low-quality evidence) and infant mortality (RR 0.93, 0.72-1.20; low-quality evidence). POSTNATAL CARE There was insufficient evidence to determine whether iron-based therapies can reduce postpartum anemia. CONCLUSION Iron supplementation is effective in preventing maternal anemia at term but not low birthweight, preterm delivery or infant mortality.
-
10.
An overview of the relationship between anaemia, iron, and venous leg ulcers.
Ferris, AE, Harding, KG
International wound journal. 2019;(6):1323-1329
-
-
Free full text
-
Abstract
The factors preventing healing in venous leg ulcers are still not fully understood. Iron-mediated tissue damage has been hypothesised, yet anecdotally anaemia is also thought to have a negative effect on wound healing. This article summarises the current evidence for these theories and their likely effects in the context of venous ulceration. A comprehensive search of the literature was conducted. Studies suggest that a number of forms of iron including haemosiderin and ferritin are implicated in progression of venous disease, ulcer formation, and impaired healing, which is thought to be primarily free radical mediated. There is a paucity of evidence for the role of iron deficiency and anaemia on ulcer healing; however, there is likely to be a highly complex interplay between the damaging effects of iron on local tissues and the negative effects of anaemia-mediated tissue hypoxia. Studies looking at options to increase local oxygen delivery such as topical haemoglobin suggest that this may have an impact on some aspects of healing, but findings are generally inconclusive. There is growing evidence that locally elevated iron levels may have a detrimental effect on ulcer healing and formation; however, more robust research is needed.