Rare presentations of small bowel endometriosis.

Despite endometriosis being a relatively common chronic gynecological condition in women of childbearing age, small bowel endometriosis is rare. Presentations can vary from completely asymptomatic to reported symptoms of abdominal pain, bloating, and diarrhea. The following two cases depict very atypical manifestations of ileal endometriosis that presented as obscure intermittent gastrointestinal bleeding and bowel obstruction requiring surgical intervention. The first case describes a previously healthy 40-year-old woman with severe symptomatic iron deficiency anemia and intermittent melena. A small bowel enteroscopy diagnosed multiple ulcerated strictures in the distal small bowel as the likely culprit. Despite nonsteroidal anti-inflammatory drug-induced enteropathy being initially considered as the likely etiology, histopathological examination of the resected distal ileal segment revealed evidence of endometriosis. The second case describes a 66-year-old with a presumptive diagnosis of Crohn's disease who reported a 10-year history of intermittent perimenstrual abdominal pain, diarrhea, and nausea with vomiting. Following two subsequent episodes of acute bowel obstruction and surgical resection of the patient's stricturing terminal ileal disease, histopathological examination demonstrated active chronic inflammation with endometriosis. Small bowel endometriosis should be considered as an unusual differential diagnosis in women who may present with obscure gastrointestinal bleeding from the small bowel or recurrent bowel obstruction.

Doença falciforme: cuidado com pessoas com úlcera da perna nos serviços de atenção à saúde / Sickle cell disease: caring for people with leg ulcers in heal-thcare services / Enfermedad falciforme: cuidado de personas con úlcera de pierna en los servicios de atención a la salud

Objetivo: identificar o local e os cuidados diretos recebidos por pessoas com úlceras da perna por doença falciforme nos serviços de atenção à saúde. Método: estudo transversal, realizado em 11 centros, no período de agosto de 2019 a abril de 2020. Fizeram parte do estudo 72 pessoas com úlcera da perna ativa. O estudo foi aprovado pelo Comitê de Ética em Pesquisa. Resultado: apresentavam anemia falciforme 91,7% dos participantes, com mediana de três anos de existência da úlcera; 77,8% eram redicivantes; 40,3% compravam os insumos; 66,7% trocavam o próprio curativo no domicílio; 52,8% realizavam uma ou mais trocas diárias; 45,8% dos tratamentos foram prescritos pelo médico; 37,5% eram pomada (colagenase ou antibiótico); 89% não utilizavam compressão para o manejo do edema. Conclusão: a maioria dos participantes não estava inserida na Rede de Atenção à Saúde para o tratamento da úlcera, e não recebia assistência sistematizada e nem insumos apropriados.

Objective: to identify the location and direct care received by people with leg ulcers due to sickle cell disease in health care services. Method: a cross-sectional study carried out in 11 centers from August 2019 to April 2020. The study included 72 people with active leg ulcers. The study was approved by the Research Ethics Committee. Results: a total of 91.7% of the participants had sickle cell anemia, with a median of three years of ulcer existence; 77.8% were recurrent; 40.3% bought the supplies; 66.7% changed their own dressings at home; 52.8% did one or more changes a day; 45.8% of the treatments were prescribed by physician; 37.5% were ointments (collagenase or antibiotics); and 89% did not use compression to manage edema. Conclusion: most of the participants were not included in the Health Care Network for ulcer treatment and did not receive systematized care or appropriate supplies.

Objetivo: identificar el lugar y los cuidados directos recibidos por personas con úlceras de pierna por enfermedad falciforme en los servicios de atención a la salud. Método: estudio transversal, realizado en 11 centros, en el período de agosto de 2019 a abril de 2020. Participaron 72 personas con úlcera de pierna activa. El estudio fue aprobado por el Comité de Ética en Investigación. Resultado: presentaban anemia falciforme 91,7% de los participantes, con una mediana de tres años de existencia de la úlcera; 77,8% eran recidivantes; 40,3% compraban los insumos; 66,7% cambiaban su propio vendaje en el domicilio; 52,8% realizaban uno o más cambios diarios; 45,8% de los tratamientos fueron prescritos por el médico; 37,5% eran pomada (colagenasa o antibiótico); y 89% no utilizaban compresión para el manejo del edema. Conclusión: la mayoría de los participantes no estaba integrada en la Red de Atención a la Salud para el tratamiento de la úlcera, y no recibía asistencia sistematizada ni insumos apropiados.

Prevalencia y caracterización de la anemia en cirugía electiva en un Hospital Universitario

está disponible en el texto completo

Introduction: Anemia is a highly prevalent disorder. Preoperative anemia is associated with higher mortality, more complications, longer hospital stays, and higher healthcare costs. Red blood cell transfusion (RBC) does not improve these outcomes. The World Health Organization recommends implementing Patient Blood Management (PBM) programmes, as they can improve these clinical outcomes, reduce unnecessary RBC transfusions, and save costs. Despite compelling evidence, the implementation of these measures has yet to be effectively achieved. The objective of this study is to conduct a situational analysis to raise awareness about this issue and encourage the implementation of these measures. Methodology: An observational, longitudinal, retrospective cohort study was conducted at a single center. All patients undergoing elective surgery from 01/01/2022 to 01/04/2022 at the Hospital de Clínicas were included. Exclusion criteria: absence of a complete blood count in the three months prior to surgery and refusal to participate in the study. Results: A total of 329 surgeries were analyzed. 52 out of 100 procedures were performed on patients with anemia. A statistically significant association was found between preoperative anemia and receiving RBC transfusion during hospitalization. OR 11.746 (4.518 - 30.540). Anemia and RBC transfusions significantly prolonged hospital stay. Length of hospitalization based on patient condition: No anemia: 10.1 ± 1.1 days, with anemia: 27.2 ± 2.3 days. Value of p < 0.001. Non-transfused: 14.5 ± 1.3 days, transfused: 41.8 ± 4.4 days. Value of p < 0.001. Only 49 (28.6%) of the 171 patients with anemia had iron metabolism assessed before surgery. Among the 140 patients with Hb < 12 g/dL undergoing surgeries with non-insignificant bleeding, only 4 received specific treatment to optimize Hb. A total of 185 units of red blood cells (RBC) were administered during hospitalization. 49 to unstable patients (intraoperative or acute hemorrhage) and 136 to stable patients. From the analysis of the latter group, 42.5% of the patients received 3 or more RBC units. The average pre-transfusion hemoglobin was 7.0 ± 0.1. A statistically significant association was found between receiving RBC units and dying during hospitalization. OR 17.182 (3.360 - 87.872). Conclusiones: A situational analysis was conducted, revealing a high prevalence of preoperative anemia, scarce study and treatment of anemia before surgeries, and an excessive amount of blood transfusions received by some patients. This work establishes the need to implement Patient Blood Management programs to reduce the prevalence of preoperative anemia and improve our transfusion practices. It also sets a comparative framework to evaluate the progress of these measures and indicates possible indicators to assess the benefits of their implementation.

Introdução : A anemia é um distúrbio altamente prevalente. A anemia pré-operatória está associada a maior mortalidade, mais complicações, tempo prolongado de internação e maiores custos de saúde. A transfusão de glóbulos vermelhos (TGV) não melhora esses resultados. A Organização Mundial da Saúde recomenda a implementação de medidas de Gerenciamento de Sangue do Paciente (GSP), pois permitem melhorar esses resultados clínicos, reduzir TGV desnecessárias e economizar custos. Apesar da evidência contundente, a implementação dessas medidas ainda está aquém de ser efetivada. O objetivo deste trabalho é realizar uma análise da situação para conscientizar sobre o problema e incentivar a implementação dessas medidas. Metodologia: Foi realizado um estudo observacional, longitudinal, retrospectivo de coorte histórica, unicêntrico. Foram incluídos todos os pacientes submetidos a cirurgias de coordenação de 01/01/2022 a 01/04/2022 no Hospital de Clínicas. Critérios de exclusão: ausência de hemograma nos três meses anteriores à cirurgia e recusa em participar do estudo. Resultados: Foram analisadas um total de 329 cirurgias. 52 a cada 100 procedimentos foram realizados em pacientes com anemia. Foi encontrada uma associação estatisticamente significativa entre a anemia pré-operatória e a recepção de TGR durante a internação. OR 11,746 (4,518 - 30,540). A anemia e as TGR prolongaram significativamente a internação hospitalar. Dias de internação em função da condição do paciente: Sem anemia: 10,1 ± 1,1 dias, com anemia: 27,2 ± 2,3 dias. Valor p < 0,001. Não transfundidos: 14,5 ± 1,3 dias, transfundidos: 41,8 ± 4,4 dias. Valor p < 0,001. Apenas 49 (28,6%) dos 171 pacientes com anemia tinham metabolismo do ferro antes da cirurgia. Dos 140 pacientes com Hb < 12 mg/dL submetidos a cirurgias com sangramento não insignificante, 4 receberam tratamento específico para otimizar a Hb. Foram administradas um total de 185 unidades de glóbulos vermelhos (UGV) durante a internação. 49 em pacientes instáveis (intraoperatório ou hemorragia aguda) e 136 em pacientes estáveis. Da análise desses últimos, 42,5% dos pacientes receberam 3 ou mais UGV. A hemoglobina pré-transfusional média foi de 7,0 ± 0,1. Foi encontrada uma associação estatisticamente significativa entre receber UGV e falecer durante a internação. OR 17,182 (3,360 - 87,872). Conclusões: Foi realizado uma análise da situação na qual foi observada uma elevada prevalência de anemia pré-operatória, um estudo e tratamento escasso da anemia antes das cirurgias e uma quantidade excessiva de UGV recebidas por alguns pacientes. Este trabalho estabelece a necessidade de implementar programas de Gerenciamento de Sangue do Paciente para reduzir a prevalência de anemia pré-operatória e melhorar nossas práticas transfusionais. Além disso, estabelece um quadro comparativo para avaliar o progresso dessas medidas e aponta possíveis indicadores para avaliar os benefícios de sua implementação.

Impact of iron deficiency anemia on academic achievement among female university students in Saudi Arabia.

Anemia, particularly iron deficiency anemia (IDA), is a prevalent health issue globally and in Saudi Arabia, especially among young adult females. This study investigates the association between anemia and academic achievement among female students at the Female Health Campus of Jazan University, Jazan, Saudi Arabia. This cross-sectional study included 118 randomly selected participants aged 18-22. A validated questionnaire was administered to the study participants. The participants underwent blood tests for complete blood count (CBC) parameters and iron profile analysis. The study reported that 52.5% of the female students had anemia and low serum iron levels. Regarding academic performance, significant positive correlations were found between various CBC parameters including white cell count, hemoglobin, hematocrit, mean cell volume, mean cell hemoglobin, mean cell hemoglobin concentration, and serum iron. However, no significant correlations were observed between red blood cell count and platelet count with academic performance. Demographic variables were also associated with a higher odds ratio of anemia. The study highlights the prevalence of anemia among female students and its potential impact on academic achievement. The current study underscores the importance of addressing anemia in young adult females and implementing appropriate interventions to improve their educational outcomes.

L'anémie, en particulier l'anémie ferriprive (IDA), est un problème de santé répandu dans le monde et en Arabie Saoudite, en particulier chez les jeunes femmes adultes. Cette étude examine l'association entre l'anémie et la réussite scolaire chez les étudiantes du Campus de santé féminine de l'Université de Jazan, Jazan, Arabie Saoudite. Cette étude transversale a inclus 118 participants sélectionnés au hasard âgés de 18 à 22 ans. Un questionnaire validé a été administré aux participants à l'étude. Les participants ont subi des analyses de sang pour les paramètres de la formule sanguine complète (CBC) et une analyse du profil en fer. L'étude a révélé que 52,5 % des étudiantes souffraient d'anémie et de faibles taux de fer sérique. En ce qui concerne les résultats scolaires, des corrélations positives significatives ont été trouvées entre divers paramètres de CBC, notamment le nombre de globules blancs, l'hémoglobine, l'hématocrite, le volume cellulaire moyen, l'hémoglobine cellulaire moyenne, la concentration moyenne d'hémoglobine cellulaire et le fer sérique. Cependant, aucune corrélation significative n'a été observée entre le nombre de globules rouges et le nombre de plaquettes avec les résultats scolaires. Les variables démographiques étaient également associées à un rapport de cotes plus élevé d'anémie. L'étude met en évidence la prévalence de l'anémie chez les étudiantes et son impact potentiel sur la réussite scolaire. L'étude actuelle souligne l'importance de lutter contre l'anémie chez les jeunes femmes adultes et de mettre en œuvre des interventions appropriées pour améliorer leurs résultats scolaires.

FANCD2 genome binding is nonrandom and is enriched at large transcriptionally active neural genes prone to copy number variation.

Fanconi anemia (FA) is a rare genetic disease characterized by congenital abnormalities and increased risk for bone marrow failure and cancer. Central nervous system defects, including acute and irreversible loss of neurological function and white matter lesions with calcifications, have become increasingly recognized among FA patients, and are collectively referred to as Fanconi Anemia Neurological Syndrome or FANS. The molecular etiology of FANS is poorly understood. In this study, we have used a functional integrative genomics approach to further define the function of the FANCD2 protein and FA pathway. Combined analysis of new and existing FANCD2 ChIP-seq datasets demonstrates that FANCD2 binds nonrandomly throughout the genome with binding enriched at transcription start sites and in broad regions spanning protein-coding gene bodies. FANCD2 demonstrates a strong preference for large neural genes involved in neuronal differentiation, synapse function, and cell adhesion, with many of these genes implicated in neurodevelopmental and neuropsychiatric disorders. Furthermore, FANCD2 binds to regions of the genome that replicate late, undergo mitotic DNA synthesis (MiDAS) under conditions of replication stress, and are hotspots for copy number variation. Our analysis describes an important targeted role for FANCD2 and the FA pathway in the maintenance of large neural gene stability.

Role of iron deficiency anemia in inflammatory bowel disease.

Inflammatory bowel disease (IBD) is a relapsing chronic inflammatory disorder of the small and large gut with rising incidence and prevalence worldwide. Iron deficiency anemia is one of the most common extraintestinal manifestations of IBD, which correlates with the disease activity and tendency to relapse even after successful management. Anemia affects various aspects of quality of life, such as physical, cognitive, emotional, and workability, as well as healthcare costs. The anemia in IBD can be due to iron deficiency (ID) or chronic disease. The relative frequency of ID in IBD is 60%, according to some studies, and only 14% receive treatment. The evaluation of ID is also tricky as ferritin, being an inflammatory marker, also rises in chronic inflammatory diseases like IBD. The review of anemia in IBD patients involves other investigations like transferrin saturation and exploration of other nutritional deficiencies to curb the marker asthenia with which these patients often present. It underscores the importance of timely investigation and treatment to prevent long-term sequelae. We can start oral iron therapy in certain circumstances. Still, as inflammation of the gut hampers iron absorption, an alternative route to bypass the inflamed gut is usually recommended to avoid the requirement for blood transfusions.

Hematological abnormalities in liver cirrhosis.

Hematological abnormalities are common in cirrhosis and are associated with various pathophysiological mechanisms. Studies have documented a prevalence of thrombocytopenia, leukopenia, and anemia in patients with compensated cirrhosis of 77.9%, 23.5%, and 21.1%, respectively. These abnormalities carry significant clinical implications, including considerations for invasive procedures, infection risk, bleeding risk, and prognosis. Previously, cirrhosis was believed to predispose patients to bleeding due to alterations observed in classical coagulation tests such as prothrombin time, partial thromboplastin time, international normalized ratio, and thrombocytopenia. However, this understanding has evolved, and cirrhosis patients are now also acknowledged as being at a high risk for thrombotic events. Hemostasis in cirrhosis patients presents a complex phenotype, with procoagulant and anticoagulant abnormalities offsetting each other. This multifactorial phenomenon is inadequately reflected by routine laboratory tests. Thrombotic complications are more prevalent in decompensated cirrhosis and may correlate with disease severity. Bleeding is primarily associated with portal hypertension, endothelial dysfunction, mechanical vessel injury, disseminated intravascular coagulation, endotoxemia, and renal injury. This review comprehensively outlines hematologic index abnormalities, mechanisms of hemostasis, coagulation, and fibrinolysis abnormalities, limitations of laboratory testing, and clinical manifestations of bleeding and thrombosis in patients with liver cirrhosis.

Proton Density Fat Fraction Quantification (PD-FFQ): Capability for hematopoietic ability assessment and aplastic anemaia diagnosis of adults.

PURPOSE: The purpose of this study was to determine the capability of proton density with fat fraction (PD-FFQ) imaging to help assess hematopoietic ability and diagnose aplastic anemia in adults. METHODS: Between January 2021 and March 2023, patients diagnosed with aplastic anemia (AA: n = 14) or myelodysplastic syndrome (MDS: n = 14) were examined by whole-body PD-FFQ imaging, and 14 of 126 age and gender matched patients who had undergone the same PD-FFQ imaging were selected as control group. All proton density fat fraction (PDFF) index evaluations were then performed by using regions of interest (ROIs). Pearson's correlation was used to determine the relationship between blood test results and each quantitative index, and ROC-based positive test and discrimination analyses to compare capability to differentiate the AA from the non-AA group. Finally, sensitivity, specificity and accuracy of all quantitative indexes were compared by means of McNemar's test. RESULTS: Mean PDFF, standard deviation (SD) and percentage of coefficient of variation (%CV) for vertebrae showed significant correlation with blood test results (-0.52 ≤ r ≤ -0.34, p < 0.05). Specificity (SP) and accuracy (AC) of %CV of PDFF in vertebrae were significantly higher than those of mean PDFF in vertebrae and the posterior superior iliac spine (SP: p = 0.0002, AC: p = 0.0001) and SD of PDFF in vertebrae (SP: p = 0.008, AC: p = 0.008). Moreover, AC of SD of PDFF in vertebrae was significantly higher than that of mean PDFF in vertebrae and the posterior superior iliac spine (p = 0.03). CONCLUSION: Whole-body PD-FFQ imaging is useful for hematopoietic ability assessment and diagnosis of aplastic anemia in adults.

[Pathophysiology of sideroblastic anemia].

Sideroblastic anemias (SAs) are a diverse group of congenital and acquired disorders, characterized by anemia and the presence of ring sideroblasts in bone marrow. Congenital SA is a rare disorder that results from genetic mutations that impair heme biosynthesis, iron-sulfur [Fe-S] cluster biosynthesis, and mitochondrial protein synthesis. The predominant type of congenital SA is X-linked sideroblastic anemia, caused by mutations in the erythroid-specific δ-aminolevulinate synthase (ALAS2) gene, a key enzyme in the heme biosynthesis pathway in erythroid cells. SAs can also arise due to exposure to certain drugs or alcohol or to copper deficiency (secondary SAs). They are also often associated with myelodysplastic syndrome (idiopathic SA), and idiopathic SAs are the most frequently encountered type. This review discusses the current understanding of the pathophysiology underlying SA.

[Diagnosis and treatment of aplastic anemia and lower-risk myelodysplastic neoplasms].

Myelodysplastic neoplasms (MDS) are clonal hematological malignancies arising from gene mutations. Immunosuppressive therapies (IST) are effective in lower-risk MDS (LR-MDS) with characteristics such as hypoplastic marrow with low blasts or low ring sideroblasts, and with a small increase of PNH clones or decrease of megakaryocytes. Differential diagnosis of these LR-MDS cases from AA can be difficult, and precise diagnosis requires careful evaluation of bone marrow cellularity and dysplasia. To decide on an appropriate treatment strategy for LR-MDS, it is important to evaluate the underlying pathology, and preferentially select IST as first-line therapy in patients with features that indicate immune-mediated bone marrow failure.

Disentangling Anemia in Frailty: Exploring the role of Inflammation.

BACKGROUND: In older patients, frailty and anemia frequently coexist. However, only few studies have been conducted in older patients with multimorbidity and several overlapping causes of anemia, such as inflammation, inadequate nutrition or certain pathologies. This analysis aims to decipher potential factors associated with anemia in older hospital patients with frailty. METHODS: Patients (n=208, age: 62-98 years) were categorized as pre-frail (n=68) and frail (n=140) using the Fried frailty phenotype. We quantified serum concentrations of markers of iron-metabolism (iron, ferritin, transferrin, soluble transferrin receptor, hepcidin), inflammation (interleukin (IL) 6, IL-10 C-reactive protein) and haematology (hemoglobin). Principal component analysis was conducted to evaluate biomarker patterns and associations with frailty were assessed with logistic regression analysis. RESULTS: Anemia prevalence was higher in patients with frailty (84.3% versus 70.6%, p=0.021). Three principal components (PC1-3) were identified. PC1 was characterized by high factor loadings representing inflammation and factor scores differed between patients with pre-frailty and frailty [-0.04 (IQR:1.45) versus -0.51 (IQR:0.87), p<0.001]. PC2 represents macrocytic anemia and thus vitamin B12 or folate deficiency, whereas PC3 indicates hematological pathologies. Only PC1 was associated with frailty status when controlled for age, sex, number of drugs and comorbidities (OR: 2.018, 95%CI: 1.316; 3.094, p=0.001). PC2 and PC3 were not associated with frailty. CONCLUSION: Our results suggest that anemia in patients with frailty is driven by inflammation rather than being disease-related or solely the result of micronutrient deficiencies.

Burden and awareness of anemia among adolescent girls of residential school in Telangana.

OBJECTIVES: Adolescence is an important transition phase and the burden of anemia among adolescent school-going children is not well explored. The present study was carried out to determine the prevalence of anemia among school-going adolescent girls and to explore their awareness on anemia. METHODS: A cross-sectional study was conducted among 217 adolescent girls studying in a residential school of Bibinagar village, Telangana. A pre-tested, semi-structured questionnaire was administered to collect the data. Hemoglobin (Hb) was estimated using TrueHb. Data was entered into excel and was analyzed using SPSS software version 21. RESULTS: The mean (±SD) age of the adolescent girls was 13.8 (±0.99) years. About 74.2 % of the girls were in 12-14 years' age group. About 91.7 % had attained menarche. The prevalence of anemia was 81.6 % with a mean (±SD) Hb of 9.5 (±2.7) g/dl among adolescent girls. Among the adolescent girls, 32.7 % had severe anemia, 31.8 % had moderate anemia and 17.1 % had mild anemia. About 86.2 % adolescent girls had heard of anemia while 60.8 % adolescent girls felt that anemia is preventable and only 27.6 % were aware about iron-rich foods. About 38 % of adolescent girls were not aware of any causes of anemia. CONCLUSIONS: The prevalence of anemia was very high among the school-going adolescent girls of rural Telangana. The awareness regarding causes of anemia and iron-rich foods was low among study adolescent girls. Thus, there is a dire need to conduct frequent Test, Treat and Talk (T3) Anemia camps in schools through district school health program.

Anemia as an indicator of a higher retention rate for tocilizumab versus tumor necrosis factor inhibitors in patients with rheumatoid arthritis from a Korean multi-center registry.

Objective: To examine whether simple laboratory tests can guide selection between tocilizumab (TCZ) and tumor necrosis factor inhibitors (TNFi) in biologic-naive patients with rheumatoid arthritis (RA), by investigating their influence on drug retention. Methods: Data of RA patients prescribed TCZ or TNFi as the initial biologics from March 2013 to December 2021 were obtained from the KOrean College of Rheumatology BIOlogics and Targeted Therapy (KOBIO) registry. Propensity score matching was performed to adjust for baseline confounding factors. Hazards of drug discontinuation for TCZ were calculated compared to those for TNFi. Interaction analyses with a Bonferroni-corrected p-value threshold were conducted to determine whether the hemoglobin level, C-reactive protein level, erythrocyte sedimentation rate, and platelet count affected the hazards of drug discontinuation. Results: Overall, 893 patients were analyzed, of whom 315 and 578 were treated with TCZ and TNFi, respectively. The hazards of drug discontinuation in all patients were lower for TCZ than for TNFi (hazard ratio [HR] 0.53, 95% confidence interval [CI] 0.44~0.66). Notably, only the presence of anemia indicated a significant interaction (p for interaction=0.010); the HRs for drug discontinuation were 0.41 (95% CI 0.30~0.55) and 0.70 (95% CI 0.53~0.92) in the anemic and non-anemic groups, respectively. In the anemic subgroup, biologics were discontinued because of a lack of efficacy in 35.0% of TNFi initiators and 7.4% of TCZ initiators.Conclusion The drug discontinuation rate in biologic-naïve patients with RA was significantly lower for TCZ than for TNFi, particularly in those with anemia.

Prevalence of anemia and related factors among Tabari cohort population: a cross-sectional study.

BACKGROUND: Anemia presents a considerable public health challenge, standing as a leading contributor to elevated rates of mortality and morbidity. Therefore, this study aimed to investigate the prevalence of anemia and related factors among Tabari cohort population. METHODS: This study involved a cross-sectional investigation carried out during the enrollment phase of the Tabari cohort. The Tabari cohort is a subset of the larger nationwide cohort study known as the "Prospective Epidemiological Research Studies in IrAN" (PERSIAN) cohort. The collected data included general information, anthropometric measurements, medical history and blood samples. Anemia was defined as a hemoglobin level less than 13 mg/dL for men and less than 12 mg/dL for women. Data were analyzed using SPSS V.16. RESULTS: Out of the 10,073 participants included in the analysis, 1,352 individuals (13.4%) were diagnosed with anemia. In the multiple regression analysis, the odds of anemia were significantly 2.31 times in females compared to males, 3.69 times in urban residents compared to rural residents, 1.41 times in social economic categories of IV and 1.35 in social economic categories of V compared to social economic categories of I, 1.70 times in drug abuse compared to non-drug abuse, 0.71 times in body mass index (BMI) categories of 25-29.9 kg/m² and 0.70 in BMI ≥ 30 kg/m² compared to BMI < 25, 0.77 times for triglycerides(TG) > 150 compared to below 150, 0.76 times for total cholesterol(TC) > 200 compared to below 200, 0.83 times for high waist-to-hip ratio (WHR) compared to low WHR, 1.33 times in low High-density Lipoprotein (HDL) compared to high HDL, 1.18 times in diabetics (DM) compared to non-DM, and 1.37 times in individuals with coronary heart diseases (CHD) compared to healthy individuals. CONCLUSION: Anemia was a prevalent condition among Tabari cohort population. Several conditions including female gender, urban residence, Social economic level of IV and V, drug abuse, low HDL, high WHR, DM, and CHD conditions were significantly associated with increased odds of anemia. Furthermore, BMI categories of 25-29.9 kg/m² and ≥ 30 kg/m², high TC and high TG were significantly associated with decreased odds of anemia among this population.

Severe Autoimmune Hemolytic Anemia Following Pneumococcal Vaccination in an Infant With Underlying Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency: Report of a Case and Review of the Literature.

Autoimmune hemolytic anemia (AIHA) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are two distinct causes of hemolysis in children and a combination of both diseases is considered rare, especially in early infancy. We present such a rare case of severe AIHA in an infant with G6PD deficiency in the setting of Escherichia coli urinary tract infection and recent pneumococcal vaccination history, with the goal of analyzing potential links between them, examining the causative role of vaccines, and reviewing available literature.

Acute Mental Disorder Caused by Vitamin B12 Deficiency Eight Years After Total Gastrectomy.

Vitamin B12 deficiency can cause a variety of diseases. The most common disease is macrocytic anemia, but it has also been found to be a cause of psychiatric disorders. The causes of deficiency are varied, and diagnosis is often difficult. Here, we report a patient who developed mental disorders due to vitamin B12 deficiency after total gastrectomy. A 37-year-old female, eight years after total gastrectomy, was withdrawn at her workplace, talking and acting abnormally. The family had seen unusual behavior for three days. The patient had no particular history of mental illness. The possibility of herpes encephalitis was suspected, and the patient was referred to our hospital, but there were no specific findings in the head on imaging examination. Blood tests showed no macrocytic anemia. Spinal fluid cytology and electroencephalography showed no specific findings, and herpes DNA was negative. Metabolic factors such as vitamin deficiency were considered, and intravenous vitamin replacement therapy was initiated. The psychiatric symptoms improved rapidly after vitamin B12 supplementation was started. On the fifth day of her hospitalization, it was discovered that her vitamin B12 level at the time of admission was extremely low. Typically, vitamin B12 deficiency is associated with macrocytic anemia, but in this patient, serum iron was also decreased, indicating a mixed anemia, making the diagnosis difficult. The patient had undergone a total gastrectomy for gastric cancer eight years ago, and the psychiatric symptoms were thought to be due to impaired vitamin B12 absorption caused by intrinsic factor deficiency. Since then, oral replacement therapy and intramuscular injection have been continued without recurrence of symptoms. Disturbances of consciousness may have many causes, but when there is a history of gastrectomy, we should also consider vitamin B12 deficiency when examining patients.

Recurrent Rash and Anemia: A Diagnostic Challenge of Angioimmunoblastic T-cell Lymphoma Mimicking Parvovirus B19 Infection.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare and challenging subtype of T-cell lymphoma often presenting with skin rashes and difficult diagnostic features. Its presentation can mimic other conditions, complicating accurate diagnosis. This case shows AITL in a 74-year-old man initially presenting with anemia that mimicked pure red cell anemia caused by parvovirus B19. The patient exhibited direct Coombs-positive anemia and recurrent urticarial-like rashes, which were initially misleading. This case emphasizes the critical need for considering lymphoma in patients presenting with direct Coombs-positive anemia and recurrent urticarial-like rashes It underscores the importance of revisiting and thoroughly assessing medical histories to enable accurate diagnosis, even when initial presentations suggest alternative diagnoses. Early recognition and appropriate management of AITL are crucial for improving patient outcomes.

Potassium dynamics in sickle cell anemia: clinical implications and pathophysiological insights.

Potassium dynamics are critical in the pathophysiology of sickle cell anemia (SCA), a genetic disorder characterized by the presence of abnormally shaped red blood cells that lead to various complications such as vaso-occlusive crises and hemolytic anemia. This review focuses on the clinical implications and pathophysiological insights of potassium regulation in SCA, highlighting its impact on disease progression and potential therapeutic strategies. The dysregulation of potassium transport in SCA leads to significant K+ efflux and cellular dehydration, exacerbating the sickling process. Dehydrated sickle cells, due to potassium loss, become more rigid and prone to causing blockages in small blood vessels, leading to painful vaso-occlusive crises and ischemia. Furthermore, chronic hemolysis in SCA, aggravated by potassium imbalance, contributes to severe anemia and systemic complications. These insights underscore the importance of maintaining potassium homeostasis to mitigate disease severity and improve patient outcomes. Therapeutic strategies targeting potassium regulation show promise in managing SCA. Inhibitors of the Gardos channel, such as senicapoc, have demonstrated potential in reducing sickling and hemolysis. Additionally, hydration therapy plays a crucial role in maintaining electrolyte balance and preventing RBC dehydration. A comprehensive approach that includes monitoring and correcting electrolyte imbalances, along with standard treatments like hydroxyurea and blood transfusions, is essential for effective disease management.

Advancing life: innovative approaches to enhance survival in sickle cell anemia patients.

Sickle cell anemia (SCA) is a severe genetic disorder characterized by the production of abnormal hemoglobin S, leading to the formation of sickle-shaped red blood cells that cause chronic anemia, pain, and organ damage. This review explores recent innovative strategies aimed at improving survival rates and quality of life for SCA patients. Genetic therapies, particularly gene editing with CRISPR-Cas9 and gene therapy using lentiviral vectors, have shown significant potential in correcting the genetic defects responsible for SCA. Clinical trials demonstrate that these approaches can reduce sickle cell crises and minimize the need for blood transfusions by enabling the production of healthy red blood cells. Novel pharmacological treatments such as voxelotor, crizanlizumab, and L-glutamine provide additional mechanisms to prevent hemoglobin polymerization, reduce vaso-occlusive episodes, and decrease oxidative stress, respectively. These therapies offer new hope for patients, particularly those who do not respond adequately to existing treatments. Improved blood transfusion protocols, including automated red cell exchange and advanced donor-matching techniques, have enhanced the safety and efficacy of transfusions, reducing complications like alloimmunization. Comprehensive care models, integrating multidisciplinary care teams, patient education, and telemedicine, have further contributed to better disease management. By providing holistic care that addresses both medical and psychosocial needs, these models improve patient adherence to treatment and overall health outcomes. This review highlights the importance of these innovative strategies and calls for continued research and development to sustain and expand these advancements in SCA care.

Clinical and Biochemical Analysis of Glutamate-Cysteine Ligase Deficiency Presented with Late-Onset Spinocerebellar Ataxia and Hemolytic Anemia.

Introduction: Glutamate-cysteine ligase catalytic subunit (GCLC), previously known as gamma-glutamyl-cysteine synthetase, is an essential rate-limiting step in glutathione synthesis. Glutathione modulates multitudes of critical cellular processes and scavenges free radicals. Its deficiency is reported to cause hemolysis of variable severity and is a rare cause of neurological abnormalities such as spinocerebellar ataxia. Clinical Presentation: We report a 55-year-old female patient with progressive late-onset ataxia, lower limb spasticity, and chronic hemolytic anemia found to have a GCLC pathogenic variant and low glutathione level. Magnetic resonance imaging of the head and cervical spine showed global cerebellar atrophy with widened folia and decreased diameter of the upper cervical spine. Blood workup revealed hemolytic anemia with genetic testing confirmed a homozygous variant, c.514 T>A in exon 4 of the GCLC gene, resulting in Ser172Thr (TCC>ACC). Management encompassed a multidisciplinary approach with a trial of high-dose alpha-lipoic acid, glutathione supplement, and physical therapy. Conclusions: GCLC deficiency manifesting with hemolysis has been reported in 12 cases worldwide from 6 independent families, with only 4 cases having additional neurological manifestations. To date, no specific GCLC gene mutation has been attributed to the reported neurological constellation of symptoms. To the best of our knowledge, this is the first case report of late-onset spinocerebellar degeneration as a manifestation of c.514T>A (p. S172T) GCLC pathological variant genetic mutation.