- Suspecting Hyperferritinemic Sepsis in Iron-Deficient Population: Do We Need a Lower Plasma Ferritin Threshold? [Journal Article]
- PCPediatr Crit Care Med 2018 May 18
- CONCLUSIONS: Ferritin rises significantly in septic shock patients despite iron deficiency and seems to correlate with the severity of inflammation and organ dysfunction. Even a lower threshold (of 500 or 1,000 ng/mL) could predict higher mortality. It may suggest the need for redefining the plasma ferritin threshold for suspecting hyperferritinemic sepsis and sepsis-induced macrophage activation syndrome in these patients. Larger studies with frequent ferritin measurements are desirable to validate these initial observations.
- StatPearls [BOOK]
- BOOKStatPearls Publishing: Treasure Island (FL)
- Anemia is a condition of low hemoglobin (Hb). It can be subdivided several different ways: symptomatic versus nonsymptomatic, or more frequently, by laboratory findings such as macrocytic versus micr...
Anemia is a condition of low hemoglobin (Hb). It can be subdivided several different ways: symptomatic versus nonsymptomatic, or more frequently, by laboratory findings such as macrocytic versus microcytic or normochromic versus hypochromic. Normal Hb-specific laboratory cut-offs will differ slightly, but in general, the normal ranges are as follows: 135 to 180 g/L in men. 120 to 150 g/L in women. 110 to 160 g/L in children. Varied in pregnancy depending on the trimester, but generally greater than 100 g/L. Although there are conditions where much lower hemoglobin levels are acceptable, patients require a transfusion if they are symptomatic with a hemoglobin of less than 70 g/L, or less than 90 g/L if they have cardiac disease due to the increased risk of impaired perfusion of the myocardium in anemia.
- Methods for Studying Iron Regulatory Protein 1: An Important Protein in Human Iron Metabolism. [Journal Article]
- MEMethods Enzymol 2018; 599:139-155
- Iron regulatory proteins 1 and 2 (IRP1 and IRP2) are two cytosolic proteins that maintain cellular iron homeostasis by regulating the expression of genes involved in iron metabolism. IRPs respond to ...
Iron regulatory proteins 1 and 2 (IRP1 and IRP2) are two cytosolic proteins that maintain cellular iron homeostasis by regulating the expression of genes involved in iron metabolism. IRPs respond to cellular iron deficiency by binding to iron-responsive elements (IREs) found in the mRNAs of iron metabolism transcripts, enhancing iron import, and reducing iron storage, utilization, and export. IRP1, a bifunctional protein, exists in equilibrium between a [Fe4S4] cluster containing cytosolic aconitase, and an apoprotein that binds to IREs. At high cellular iron levels, this equilibrium is shifted more toward iron-sulfur cluster containing aconitase, whereas IRP2 undergoes proteasomal degradation by an E3 ubiquitin ligase complex that contains an F-box protein, FBXL5. Irp1-/- mice develop polycythemia and pulmonary hypertension, whereas Irp2-/- mice develop microcytic anemia and progressive neurodegeneration, indicating that Irp1 has important functions in the erythropoietic and pulmonary systems, and Irp2 has essential roles in supporting erythropoiesis and nervous system functions. Mice lacking both Irp1 and Irp2 die during embryogenesis, suggesting that functions of Irp1 and Irp2 are redundant. In this review, we will focus on the methods for studying IRP1 activities and function in cells and animals.
- [Successful treatment of X-linked sideroblastic anemia with ALAS2 R452H mutation using vitamin B6]. [Journal Article]
- RKRinsho Ketsueki 2018; 59(4):401-406
- A 45-year-old man presented with fatigue and pain in the finger joints. Despite having a history of suspected sideroblastic anemia since the age of 18 years, he had not been followed up for years. Up...
A 45-year-old man presented with fatigue and pain in the finger joints. Despite having a history of suspected sideroblastic anemia since the age of 18 years, he had not been followed up for years. Upon presentation, laboratory data revealed microcytic anemia and elevated serum ferritin levels. In addition, ringed sideroblasts were increased in the bone marrow. A liver biopsy revealed hemochromatosis and cirrhosis. Furthermore, genetic analysis revealed that he harbored the ALAS2 R452H mutation, leading to the diagnosis of X-linked sideroblastic anemia (XLSA). Accordingly, oral folate or vitamin (Vit) B12 was administered, but his anemia did not respond. However, his hemoglobin level increased from 7 to 11 g/dl with an additional prescription of oral VitB6, which facilitated the patient to undergo phlebotomy to ameliorate organ dysfunctions caused by iron overload. Previous research has revealed that ALAS2 R452 mutations confer poor responses to VitB6 therapy. Hence, accrual of patients with an unexpectedly better response, which was observed in our case, may help elucidate the pathogenesis of and therapies for XLSA.
- Role of Melatonin in Aluminum-Related Neurodegenerative Disorders: a Review. [Journal Article]
- BTBiol Trace Elem Res 2018 May 07
- Aluminum (Al), a potentially neurotoxic element, provokes various adverse effects on human health such as dialysis dementia, osteomalacia, and microcytic anemia. It has been also associated with seri...
Aluminum (Al), a potentially neurotoxic element, provokes various adverse effects on human health such as dialysis dementia, osteomalacia, and microcytic anemia. It has been also associated with serious neurodegenerative diseases such as Alzheimer's disease (AD), amyotrophic lateral sclerosis, and Parkinsonism dementia of Guam. The "aluminum hypothesis" of AD assumes that the metal complexes can potentiate the rate of aggregation of amyloid-β (Aβ), enhancing the toxicity of this peptide, and being able of contributing to the pathogenesis of AD. It has been supported by a number of analytical, epidemiological, and neurotoxicological studies. On the other hand, melatonin (Mel) is a potent direct free radical scavenger and indirect antioxidant, which acts increasing the activity of important related antioxidant enzymes, and preventing oxidative stress and cell death of neurons exposed to Aβ-induced neurotoxicity. Therefore, Mel might be useful in the treatment of AD by reducing the Aβ generation and by inhibiting mitochondrial cell death pathways. The present review on the role of Mel in Al-related neurodegenerative disorders concludes that the protective effects of this hormone, together with its low toxicity, support the administration of Mel as a potential supplement in the treatment of neurological disorders, in which oxidative stress is involved.
- The impact of erythropoietin and iron status on brain myelination in the newborn rat. [Journal Article]
- JNJ Neurosci Res 2018 Apr 26
- Erythropoietin (Epo) drives iron (Fe) utilization for erythropoiesis, but the potentially resultant tissue iron deficiency (ID) can also impede brain development. Conversely, Epo binds to Epo recepto...
Erythropoietin (Epo) drives iron (Fe) utilization for erythropoiesis, but the potentially resultant tissue iron deficiency (ID) can also impede brain development. Conversely, Epo binds to Epo receptors (EpoR) on immature brain oligodendrocytes and neurons, promoting growth and differentiation. The objective of the study was to examine the interaction between Epo and Fe on myelination in brain development during daily Epo treatment. Male and female Sprague-Dawley rats from postnatal day (P) P4-P12 modeled premature newborns. Dam-fed Fe-sufficient (IS) or postnatal ID groups were given daily subcutaneous sham or erythropoietic Epo injections (425 U. kg-1. d-1 ), ± oral Fe (6 mg. kg-1. d-1 ). Tissues and blood were collected and studied at P12. Epo in the ID groups, in the absence of oral Fe, stimulated microcytic ID anemia along with raising inflammatory markers. Both the microcytic anemia and inflammation improved in the ID + Epo + Fe group. Fe treatment positively impacted erythropoiesis and body Fe (µg/g) in all groups. Relative brain Fe (µg/g rat) was improved in the IS + Epo + Fe group. Brain Fe was not worsened in +Epo groups. Brain weight and brain Fe were related to plasma Epo levels. Amount of myelination was impacted by feeding type, but was not inhibited by Epo. Expression of a protein in myelin, mylein basic protein, was greater in all +Fe groups than -Fe groups. With therapeutic Epo, available body Fe was prioritized for erythropoiesis instead of brain, but Epo did not worsen brain Fe and potentially Epo improved myelination and maturation in the brain.
- Clinical Course of Homozygous Hemoglobin Constant Spring in Pediatric Patients. [Journal Article]
- JPJ Pediatr Hematol Oncol 2018 Apr 17
- CONCLUSIONS: Pediatric patients with homozygous Hb Constant Spring developed severe anemia in utero and up to the age of 2 to 3 months postnatal, requiring blood transfusions. Subsequently, their anemia was mild with no evidence of hepatosplenomegaly. Their Hb level was above 9 g/dL with hypochromic microcytic blood pictures as well as wide RDW. Blood transfusions have not been necessary since then.
- KARS-related diseases: progressive leukoencephalopathy with brainstem and spinal cord calcifications as new phenotype and a review of literature. [Journal Article]
- OJOrphanet J Rare Dis 2018 Apr 04; 13(1):45
- CONCLUSIONS: With our report we define the molecular basis of the previously described Leukoencephalopathy with Brainstem and Spinal cord Calcification widening the spectrum of KARS related disorders, particularly in childhood onset disease suggestive for mitochondrial impairment. The review of previous cases does not suggest a strict and univocal genotype/phenotype correlation for this highly heterogeneous entity. Moreover, our cases confirm the usefulness of search for common brain and spine MR imaging pattern and of broad genetic screening, in syndromes clinically resembling mitochondrial disorders in spite of normal biochemical assay.
- Determining mean corpuscular volume and red blood cell count using electrochemical collision events. [Journal Article]
- BBBiosens Bioelectron 2018 Jul 01; 110:155-159
- Blood tests (e.g., red blood cell (RBC) count) are crucial for detecting, diagnosing, and monitoring the progression of blood disorders. Here, we report the development of a new and rapid method for ...
Blood tests (e.g., red blood cell (RBC) count) are crucial for detecting, diagnosing, and monitoring the progression of blood disorders. Here, we report the development of a new and rapid method for electrochemically detecting RBCs using single-particle collision events. The principle of this method relies on the electrochemical oxidation of an electroactive redox species (potassium ferrocyanide) hindered by an RBC attached to an electrode surface. A decrease in staircase current, caused by the collision of RBCs on the electrode, was observed. The magnitude of this current decrease could provide quantitative information on the size and concentration of RBCs, which could be converted into the mean corpuscular volume (MCV) and used for diagnosis. Anemia-related diseases caused by abnormal count of RBCs (e.g., erythrocytosis, pernicious anemia) or abnormal RBC size (e.g. megaloblastic anemia, microcytic anemia) could be detected easily and quickly using this electrochemical collision method, potentially leading to extensive applications in hematology and point-of-care blood testing devices.
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- Microcytic anemia due to ileocolic anastomotic ulcer. [Journal Article]
- GHGastroenterol Hepatol 2018 Mar 24