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Hemoglobinopathies are vast grouping of inherited disorders of the hemoglobin chain genes that affect over 270 million people globally. The most common hemoglobinopathies include sickle cell, alpha- thalassemia, and beta-thalassemia. Each of these diseases has numerous phenotypes and thus presentation and management of each will vary. Generally, the carrier or asymptomatic states have pregnancy outcomes that mirror the general population. Patients with clinically significant disease are at increased risk for numerous maternal and fetal complications. All patients desiring pregnancy and those that are currently pregnant should be screened for hemoglobinopathy and those found to have one provided with genetic counseling regarding any potential maternal or fetal risks.
Lifestyle and diet may affect the reproductive cycle. A dietary index called Diet Diversity Score (DDS) may be related to various reproductive outcomes. The present review aims to look over and conclude the prior studies on the relationship between the diversity of food ingredients and issues related to reproductive health and pregnancy. In the case of this relationship, our findings can increase clinical knowledge and help recommend a well-balanced diet for the target group. A comprehensive search was performed in major databases such as PubMed, Google Scholar, Web of Science, Scopus, and Scientific Information Database until March 2024. This research was combined with a search of Elsevier and SpringerLink databases, which led to the inclusion of relevant articles in this review. Our study was conducted based on 27 articles from 2012 to 2023, all containing a possible link between dietary diversity and reproductive complications. The Newcastle-Ottawa Scale quality assessment was used to evaluate the quality of included studies. Due to our results, a higher score in DDS, which led to an increased intake of major nutrients and a greater variety of foods, was correlated with a lower risk of reproductive health disorders such as polycystic ovary syndrome, maternal anaemia, and maternal bone status, as well as a reduced likelihood of certain birth outcomes, including low-birth weight infants, Apgar score and congenital heart defect. These findings highlight the importance of improving the DDS for maternal and infant health.
Bioarchaeologists commonly record porous cranial lesions (PCLs). They have varied etiologies but are frequently associated with nutritional anemia without a differential diagnosis. This article provides a literature review, evaluates diet in the US Southwest over time, and identifies issues with associating PCLs with poor diet in this region. Generally, diet was adequate across time and space. Although maize was a dietary staple, other food items such as rabbits and amaranth provided complementary micronutrients. PCLs exhibit varied morphologies, which generally correspond with age: those characterized by fine, scattered porosity are associated with younger ages at death. Variation in PCL morphology indicates different and sometimes unrelated etiologies. Nutritional anemia is an insufficient explanation for PCL frequency in the Southwest, partly because the diet was adequate across time.
Despite bold commitments to reduce anaemia, little change in prevalence was observed over the past decade. We aimed to generate subnational maps of anaemia among women of reproductive age (WRA), malaria transmission and hemoglobinopathies to identify priority areas but also explore their geographical overlap.
Design:
Using the most recent Demographic and Health Surveys (DHS), we first mapped anaemia clusters across sub-Saharan Africa and identified the West and Central Africa (WCA) as a major cluster. Geographic clusters with high anaemia and related aetiologic factors were identified using spatial statistics. Multilevel regression models were run to identify factors associated with any, moderate and severe anaemia.
Settings:
West and Central African countries (n 17).
Participants:
WRA (n 112 024) residing in seventeen WCA countries.
Results:
There was a significant overlap in geographical clusters of anaemia, malaria and hemoglobinopathies, particularly in the coastal areas of the WCA region. Low birth interval (0·86 (0·77, 0·97)), number of childbirth (1·12 (1·02, 1·23)) and being in the 15–19 age range (1·47 (1·09, 1·98)) were associated with increased odds of any anaemia. Unimproved toilet facility and open defecation were associated with any anaemia, whereas the use of unclean cooking fuel was associated with moderate/severe anaemia (P < 0·05). Access to healthcare facility, living in malaria-prone areas and hemoglobinopathies (HbC and HbS) were all associated with any, moderate or severe anaemia.
Conclusion:
Interlinkages between infection, hemoglobinopathies and nutritional deficiencies complicate the aetiology of anaemia in the WCA region. Without renewed efforts to integrate activities and align various sectors in the prevention of anaemia, progress is likely to remain elusive.
Edited by
Laurie J. Mckenzie, University of Texas MD Anderson Cancer Center, Houston,Denise R. Nebgen, University of Texas MD Anderson Cancer Center, Houston
Leukemias represent a range of bone marrow disorders that are broadly differentiated into acute and chronic. Acute leukemias, characterized by the proliferation of immature blood cells (blasts) and defined by peripheral blood or bone marrow blast percentage of 20 percent or more, are aggressive hematologic malignancies that are universally fatal without treatment. Chronic leukemias are mature leukemias with differentiated cells. Obstetric and gynecologic complications pose significant risk to the patient. Knowledge regarding uterine bleeding, fertility planning, and the management of the pregnant patient with leukemia are necessary in order to appropriately address these patients. In this chapter, we briefly review both acute and chronic leukemias, epidemiology, diagnosis and management, obstetric and gynecologic complications, teratogenicity of chemotherapies utilized in the treatment of each leukemia and finally detail our approach to the management of these patients.
The experimental microcirculatory analysis of treating anemia by blood transfusion shows that it minimally increases oxygen (O2) delivery by the capillary circulation, where O2 pressure (pO2), hematocrit (Hct) and capillary hydraulic pressure (pC) are normally 1/5 of systemic, and further depressed by anemia. The concomitant decrease of functional capillary density becomes lethal when falling below a specific threshold due to the decrease in pC, and requires re-pressurizing the microcirculation without increasing the heart’s work load. These effects are achieved by increasing plasma blood viscosity using a viscous plasma expander. This approach significantly elevates capillary blood viscosity and shear stress, increasing the production of the vasodilator NO, becoming significantbecause of the large capillary endothelial surface area, and because increased NO tends to lower O2 consumption. We show that using hyperviscous plasma expanders increases FCD, blood flow velocity and the rate of RBC delivery to the tissues. They could ultimately justify transfusing blood when DO2 falls to ¼ of normal, which is 4 times the normal organism metabolic requirement at rest.
Sickle cell disease (SCD) is caused by an autosomal recessive mutation in the β-globin chain of hemoglobin A (HbA), causing the mutated hemoglobin S (HbS). In a vaso-occlusive crisis (VOC), sickled red blood cells (RBCs) increase the viscosity of blood and cause microvascular occlusion, contributing to hypoxia, acidosis and further sickling. The clinical manifestations of SCD are diverse and are related to chronic hemolysis with periodic episodes of vascular occlusion that can affect nearly every organ system.
Spaceflight associated neuro-ocular syndrome (SANS) is a collection of distinct findings seen in some astronauts following prolonged spaceflight and is characterized by: optic disc edema, globe flattening, and choroidal folds. In this manuscript, we describe the potential mechanisms linking anemia and SANS. Future research aimed at understanding the relationship between these conditions may help to develop countermeasures and mitigation efforts for SANS.
The purpose of this exploratory study was to describe associations between NIH Toolbox-Cognition Battery subtests and legacy measures of neurocognitive function in two samples with neurological conditions (stroke and sickle cell disease (SCD)).
Method:
This exploratory secondary analysis uses data from two studies that assessed cognition at one time point using the NIH Toolbox-Cognition Battery, the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), and subtests from the Delis-Kaplan Executive Functions System (DKEFS). People with stroke (n = 26) and SCD (n = 64) were included. Associations between the NIH Toolbox-Cognition Battery subtests and corresponding legacy measures were examined using linear correlations, Bland–Altman analysis, and Lin’s Concordance Correlation Coefficient.
Results:
Linear correlations and Lin’s Concordance Correlation Coefficient were poor to strong in both samples on NIH Toolbox-CB subtests: Flanker Inhibitory Control and Attention (r = .35 to .48, Lin CCC = .27 to .37), Pattern Comparison Processing Speed (r = .40 to .65, Lin CCC = .37 to .62), Picture Sequence Memory (r = .19 to .55, Lin CCC = .18 to .48), Dimensional Change Card Sort (r = .39 to .77, Lin CCC = .38 to .63), Fluid Cognition Composite (r = .88 to .90, Lin CCC = .60 to .79), and Total Cognition Composite (r = .64 to .83, Lin CCC = .60 to .78). Bland–Altman analyses demonstrated wide limits of agreement across all subtests (–3.17 to 3.78).
Conclusions:
The NIH Toolbox-Cognition Battery subtests may behave similarly to legacy measures as an overall assessment of cognition across samples at risk for neurological impairment. Findings should be replicated across additional clinical samples.
Hematologic abnormalities occur commonly in the elderly. The prevalence of anemia appears to increase with age and may be caused by various underlying etiologies, including iron deficiency, anemia of inflammation, or myelodysplastic syndrome. Thrombocytopenia due to underlying comorbidities, medications, or immune thrombocytopenia (ITP) may also occur. Hematologic malignancies such as chronic lymphocytic leukemia (CLL) and multiple myeloma also become more prevalent with age. A systematic approach to the evaluation of these hematologic abnormalities is imperative to help guide diagnosis and management. For acute or progressive conditions, a multidisciplinary team of both geriatricians and hematologists is essential to ensure proper diagnosis, frailty assessment, and initiation of appropriate therapies. Novel therapies for the various hematological malignancies are well tolerated, turning life-threatening illnesses into chronic disease that can be managed while preserving quality of life.
A 40-year-old nulligravid woman presents to the office for management of abnormal uterine bleeding. She reports a four-year history of heavy menses, which often lasts for two weeks in length and requires at least 10 sanitary pads per day. She denies abdominal pain or associated dizziness during menses. She reports prior use of multiple hormonal medications, including oral medroxyprogesterone and combined oral contraceptive pills, without improvement in her bleeding patterns. She is not currently taking any medications other than over-the-counter ferrous sulfate. Her past medical history is significant for iron deficiency anemia. She denies any surgical history. She has no plans for future fertility and would like definitive surgical management. When signing the surgical consent, she refuses transfusion of blood products.
Twin pregnancies, especially those in which the twins share one placenta but have separate amnion sacs (referred to monochorionic, diamniotic or Mo-di twins), are at risk for a number of complications. Most notable of these is twin-twin transfusion syndrome. This condition can result in fetal morbidity or intrauterine fetal demise but is also amenable to surgical intervention. Severe discordance in amniotic fluid volumes between each twin is the prenatal finding that highlights this diagnosis. Selective laser photocoagulation has greatly improved the survival of twins with this condition. With a posteriorly located placenta, laser ablation of the abnormal placenta vascular communications can be achieved with local anesthesia and maternal sedation. Anterior placenta location may present a challenge for uterine access that may require modification of the anesthetic technique.
Anemia has created attention worldwide because of its adverse effects on the mother and the fetus during pregnancy. A large body of evidence has shown that pregnant women are the most vulnerable group to anemia.
Objectives:
This study aims to determine the prevalence of anemia, and associated risk factors, among pregnant women attending antenatal care (ANC) at government and private hospitals in Bangladesh.
Methods:
This cross-sectional study included 424 pregnant women, who visited hospitals for ANC from January to July 2019. We used a simple random sampling technique to select study subjects. Data were collected using a structured questionnaire and participant’s current medical record cards. SPSS software was used for analyzing data.
Results:
The prevalence of anemia was 62.5% and significantly (P < 0.001) higher in the subjects attending ANC in government hospitals (68.7%) than in private (55.0%) hospitals. The prevalence of the severity of anemia was 28.3% mild, 36.9% moderate, and 3.40% severe in government hospitals while in private hospitals was 14.7% mild, 39.8% moderate, and 0.5% severe anemia. Anemia was significantly associated with maternal age 20–25 years [adjusted odds ratio (AOR) = 1.9] and 26–30 years (AOR = 2.37), monthly family income (300–500) US$ (AOR = 2.76), and ANC in government hospitals (AOR = 2.02), the parity [multiparous (AOR = 1.92)], gravidity [multigravid (AOR = 1.63)], contraception [no contraception (AOR = 2.50), and iron supplement [no iron supplement (AOR = 0.64).
Conclusions:
The result suggests that pregnant women should receive routine ANC and recognize iron supplementation during pregnancy. Finally, the results of this study are particularly relevant for pregnant women who are receiving ANC.
The effects of iron deficiency on the prognosis of idiopathic sudden sensorineural hearing loss are unclear. This study aimed to investigate the association between serum iron levels and idiopathic sudden sensorineural hearing loss prognosis and its usefulness as an independent prognostic marker for idiopathic sudden sensorineural hearing loss.
Methods
The audiological and haematological data, including hearing recovery and serum iron levels, of 103 patients with idiopathic sudden sensorineural hearing loss evaluated between 2015 and 2018 were retrospectively analysed.
Results
The overall complete recovery rate was 16.5 per cent. Initial higher hearing threshold was associated with poor idiopathic sudden sensorineural hearing loss prognosis. Serum iron levels were significantly higher in the complete recovery group than in the non-complete recovery group (p < 0.05).
Conclusion
The possibility of complete recovery from idiopathic sudden sensorineural hearing loss was significantly lower with lower serum iron levels, suggesting that the serum iron level might be a novel prognostic marker for idiopathic sudden sensorineural hearing loss.
The history of Athens was influenced by the health of her citizens; from birth to old age, disease, injury, and warfare threatened the lives of citizens, or made them unable to participate in the life of the city. The study of skeletal remains from burials in Athens reveals the effect of these threats, and offers new information on historical plagues, attacks on the city, and ordinary events in the lives of Athenians.
Bone marrow (BM) hyperplasia, a non-neoplastic expansion of one or more of the haematopoietic cell lineages due to an increased number of cells, can manifest in a range of morphological appearances depending on the underlying cause. Similarly to other tissue types, hyperplasia is often associated with an increase in the number of cells with less mature morphology. It is this reactive atypia/dyshaemopoietic morphology that needs careful assessment and correlation/integration with clinical, biochemical, radiological and often molecular findings to correctly interpret the underlying process and avoid misdiagnosis as a neoplastic proliferation. This chapter will consider erythroid, myeloid and megakaryocytic hyperplasia. Reactive conditions of histiocytes are covered in Chapter 6.
role of global cognition in the association between Anemia and depression.
Background:
We examined the longitudinal relationships between hemoglobin concentrations or Anemia and depression and whether baseline cognitive function modifies these longitudinal relationships over 4 years of follow-up.
Methods:
A total of 1608 community-dwelling older adults from the International Mobility in Aging Study (IMIAS) aged 65 to 74 years were recruited in Natal (Brazil), Manizales (Colombia), Kingston (Ontario, Canada), and Saint-Hyacinthe (Quebec, Canada). The study outcome was depression, defined by a score of 16 or over in the Center for Epidemiologic Studies Depression Scale (CES-D). Longitudinal associations over four years follow-up were examined using generalized estimating equations. Models reported were either unadjusted and adjusted for research sites, alcohol drinking status, body mass index, chronic conditions, activities of daily life disabilities, and polypharmacy.
Results:
Longuitinal relationships suggested an evidence of multiplicative interaction by baseline global cognition in which 1g/dL increase in hemoglobin concentrations there was a significant reduction in the risk of depression with a stronger effect among participants with good cognitive function (Odds Ratio (OR)=0.85, 95% CI: 0.78-0.92) compared to those with poor cognition (OR=0.89, 95% CI: 0.80-0.97). Anemia and poor cognition at baseline were associated with an increased risk of depression over four years of follow-up (OR=5.80, 95% CI: 1.84-18.23). Global cognition was also an effect modifier of the longitudinal association between the severity of Anemia and depression.
Conclusion:
In international samples of older adults, hemoglobin concentrations, as well as the severity of Anemia, were independent risk factors for depression, and these associations differed by global cognitive function.
Chapter six, “The Struggle Against Hookworm Disease,” examines the early campaigns of the Rockefeller Foundation to reduce transmission of the widespread helminthic infection. Launched in the southern United States and then extended southward in the western hemisphere and into the eastern hemisphere, the anti-hookworm campaigns became the very first global health initiative. Although the campaigns utilized chemical therapies to reduce the intestinal worm load, their primary focus was on changing defecation habits, to encourage better sanitation. The campaigns failed to meet their goals, underscoring the limitations of mass drug treatment and the difficulties of changing entrenched defecation practices and the use of human waste as fertilizer.
This study investigated the health effects of Lake Urmia’s drought on adjacent urban and rural areas and people.
Methods:
The data for sociodemographic status, physical activity, dietary pattern, smoking, and angina of the subjects living in areas adjacent to and far from Lake Urmia were collected through validated questionnaires. Physical examinations, including blood pressure, anthropometrics, and biochemical measurements, were performed.
Results:
There were no significant differences between 2 areas in the case of age, sex, educational, and physical activity and smoking status (P > 0.05). The mean systolic and diastolic blood pressures and the prevalence of hypertension, prehypertension, and anemia in cases living in the adjacent areas were significantly higher than those in the control group (P < 0.05). No significant differences were observed between 2 districts in the prevalence of hyperlipidemia, overweight/obesity, asthma, angina, infraction, diabetes, and vitamin D insufficiency/deficiency.
Conclusions:
Our data showed that Lake Urmia’s drought has serious effects on hypertension and anemia. More longitudinal and well-designed studies are needed to confirm these results.