anemia

A Case of Catastrophic Atypical Hemolytic Uremic Syndrome Unresponsive to Eculizumab and the use of Ravulizumab Off-label

Published on: 5th October, 2023

“A 40-year-old woman with melanoma, under treatment with Dabrafenib and Trametinib, was evaluated in our hospital for rapidly progressive deterioration of renal function”. 8 months before the current admission, the patient had been diagnosed with melanoma, and underwent radical surgery and subsequent therapy with Dabrafenib and Trametinib.After 5 months of therapy, the patient was brought to this hospital for precordial pain, with a diagnosis of myopericarditis, therapy was started for heart failure with a good response. However, the patient developed a progressive impairment of renal function, associated with hemolytic anemia and thrombocytopenia. The peripheral smear showed the presence of schistocytes.The suspicion of atypical Hemolytic Uremic Syndrome (aHUS) was confirmed by the assay of C5B-9 induced by serum on endothelial cells, which showed a deposition of 331%, treatment with Eculizumab was initiated.After 3 administrations the patient did not improve, with further worsening of the hemolytic condition, and progression of renal damage.Due to the failure of Eculizumab, we considered the use of Ravulizumab. However, in Italy only can be administered to patients in Eculizumab stable treatment for at least three months. Nevertheless, faced with the catastrophic condition, it was decided to shift the therapy and use off-label Ravulizumab. After 10 days of the first administration, the laboratory tests showed a continuous rise in the values of haptoglobin, platelets, and hemoglobin, and a decrease in LDH. The renal function failed to return to normal values but after 20 days of therapy with Ravulizumab, there was complete resolution of the hemolytic condition.
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Near-miss Women Causes and Prevalence in Alobied Maternity Hospital

Published on: 13th November, 2023

Background: Maternal near-miss (MNM) events occur more frequently than maternal deaths; therefore, more detailed and comprehensive studies on maternal morbidity have been conducted and are of value to clinical audits and practices. Purpose: This study aimed to determine the frequency of maternal near misses and the nature of near-missevents. Methodology: This descriptive, retrospective, cross-sectional study over 12 months duration was conducted at the Alobied Teaching Hospital in 2018. Data were collected from patient notes, partographs, and other relevant documents. Demographic and clinical data concerning personal history, obstetric history, and near-miss events. Results: A total of 15202 women were admitted, 339 cases of maternal near misses, maternal near-missrate (MNMR) of 22.3|1000 live births, 200(59%) had an infection, 80(23.6%) hemorrhage, 20(5.9%) severe pre-eclampsia,12(3.5%) eclampsia, 20(5.9%) anemia, convulsions 5 (1.5%) 17(5%) of the cases were admitted to intensive care unit (ICU), 9(2.7%) had liver dysfunction, 9(2.7%) coagulation dysfunction, 8(2.4%) renal dysfunction, 5(1.5%) cerebral problems, 4(1.2%) cardiac dysfunction, and 2(0.6%) had developed respiratory dysfunction.Conclusion: The maternal near-miss rate was 22.3|1000 live births. Most near-miss cases occurred before the women arrived at the hospital. The major causes of maternal near misses were infection, hemorrhage anemia, pre-eclampsia, and eclampsia.
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Mechanism of Action and Validation of the Traditional Medicinal use of Grewia Tenax Fruits in Sudan to Encounter Iron Deficiency Anemia

Published on: 27th December, 2023

Introduction: In Sudan, Grewia tenax fruits, are known commonly as Goddaim. The fruit’s pericarp is used traditionally for a long time as a juice or a porridge to treat iron deficiency anemia (IDA). Traditional Goddaim users have a very strong belief in its effectiveness. However, the pattern of hemoglobin improvement follows an initial fast increase followed by a decline upon continuing its use. Some previous studies have attributed its effect to high iron content, while the iron quantity was too small. This work attempts to find an explanation for its mechanism of action by screening the fruit extract and its respective fractions for secondary metabolites, minerals, vitamins, and fibre. Methods: Entailed three methodologies: Chemical analysis to identify quantified minerals, ascorbic acid and non-digestible fibers, Phytochemical Analysis to separate and identify secondary metabolites using high-performance liquid chromatography coupled to tandem mass spectrometry (HPLC-MS-MS technique), and estimation of radical scavenging activities of crude fruit extract and its respective chloroform and ethyl acetate fractions by inhibition of the 2,2-diphenylpicrylhydrazyl (DPPH).Results: Ascorbic acid and indigestible fibres were revealed in the aqueous extract. Secondary metabolites were flavonoids (e.g., quercetin, kaempferol), organic acids (e.g., ferulic acid, chlorogenic acid), β-carboline alkaloids (e.g., 3-hydroxy-tetrahydroharman) identified in the chloroform, and ethyl acetate fractions. The in vitro antioxidant activity of G. tenax fruit extract was confirmed spectrophotometrically. Conclusion: It can be concluded that the initial enhancement of iron absorption through ascorbic acid and fibres, followed by iron uptake inhibition, could be explained by iron chelation by the chelators in the fruits. This paradoxical effect may qualify G. tenax fruits as a safety gauge for improving haemoglobin levels without compromising iron excess once iron stores are filled by keeping the oxidative stress in check. This may present G. tenax fruits as a good and safe remedy that optimizes the treatment of IDA.
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Autoimmune Diseases Associated with Iron Deficiency Anemia; Coexistence of Demyelinating Disease and Inflammatory Bowel Disease

Published on: 24th January, 2024

In a young patient with iron deficiency anemia and low hemoglobin levels, we identified a very rare association: Demyelinating disease with Inflammatory Bowel Disease (IBD).
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Case Report: An Elusive Case of Septic Arthritis

Published on: 2nd May, 2024

Septic arthritis is a serious inflammatory infectious state of the joint secondary to microbial infection. In the pediatric population the most common route of infection is haematogenous spread. Less than fifty percent of patients with septic arthritis will yield positive culture results with a mortality rate of up to 42% in some cases. Due to the challenge in obtaining culture and identification of the causative organism the management of septic arthritis has been more of empirical in nature with the chosen antibiotic synchronized with the epidemiological data. Here is a case of a 14 months old female patient presenting at our hospital with a left knee and lower thigh swelling for three days with failure to bare weight on the limb. In addition, she had fever and diarrhea for three days. Upon evaluation clinical, laboratory and radiological findings supported septic arthritis expect for her blood, pus and synovial fluid culture of which all came back negative. She had poor response to intravenous ceftriaxone, gentamycin, metronidazole, ampicillin- cloxacillin and amoxicillin clavunate. Over the course of therapy, she developed septic shock, severe anemia and acute liver failure and was admitted to the intensive care unit. Afterwards she was initiated vancomycin and developed a hypersensitivity reaction with generalized edema which prompted cessation of the drug. Due to her critical state and poor response a triple therapy regimen composing of meropenem, ciprofloxacin and metronidazole was selected and maintained for three weeks followed by an oral clindamycin course for another three weeks of which she responded. In addition, surgical debridement arthrotomy, irrigation and drainage were done. Physiotherapy for rehabilitation is ongoing with patient recovering well.
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A Retrospective Analysis of Maternal Mortality in a Tertiary Care Centre at District Level

Published on: 23rd May, 2024

Background: Maternal mortality is a very sensitive indicator of our health system and services. Epidemiological data about maternal mortality is a vital requirement in every setting not only to design interventional programs but also to identify gaps in the existing structures, to reduce the ratio favorably. This study was an attempt to shed light on the mortality rate in our hospital, to analyze the epidemiological aspects, causes of maternal mortality, and types of delay, and to suggest recommendations for improvement. Methods: A retrospective study was done at a tertiary care center at the district level from January 2022 to December 2022. Demographic data and details of selected parameters were obtained from maternal death review forms and case records. Data analyzed and presented.Results: The majority of the maternal deaths could be attributed to direct obstetric causes like Eclampsia (29.41%), Pre-eclampsia (20.59%), and anemia (20.59%) followed by hemorrhage (14.71%), and septicemia (11.76%). Medical conditions contributing to death included liver disorders (5.8%), COVID-19 infection (5.8%), and cardiac disorders (2.9%). Conclusion: It is evident that the maternal mortality ratio is significantly high, with a majority of the deaths occurring due to avoidable causes. Leading contributory factors were due to delay in seeking help or delay in referral. High-risk cases must be identified at the earliest and referred to higher centers for management from the first trimester itself. Generating awareness among the common public and counseling the gravidas and their caretakers throughout pregnancy is equally important to prevent maternal mortality and near-miss cases.
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Bicytopenia Revealing an Intramedullary Spinal Cord Metastatic Prostatic Carcinoma

Published on: 30th May, 2024

Usually revealed by lower urinary tract symptoms, prostate neoplasms are the most frequent urological cancer.The patient was a 70-year-old man being explored for bicytopenia in the hematology department: anemia and bicytopenia with no urinary symptoms in the first plan.Bone marrow biopsy was done and the histological and immunohistochemical concluded in an intramedullary spinal cord metastasis of an undifferentiated prostatic carcinoma.We have reviewed the literature one other case of intramedullary metastasis of prostatic cancer was detected but the main symptoms were neurological and the patient was already diagnosed with his cancer.
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Pure Red Cell Aplasia - Post Major ABO Incompatible Allogenic Stem Cell Transplantation Role of Ibrutinib

Published on: 24th July, 2024

Pure Red Cell Aplasia (PRCA) is a well-recognized complication of Major ABO-incompatible allogeneic stem cell transplantation. It is featured by anemia, Reticulocytopenia, and the absence of erythroblasts in a normal-appearing bone marrow biopsy. The mechanism for PRCA is presumed to be the persistence of recipient isoagglutinins, produced by residual host B lymphocytes or plasma cells, which probably interfere with the engraftment of donor erythroid cells. Several risk factors for PRCA have been reported, such as the presence of Anti-A Isoagglutininsbefore transplantation, reduced intensity conditioning, absence of Graft Versus Host Disease (GVHD), sibling donor and Cyclosporin A(CsA) as GVHD prophylaxis. PRCA is not a barrier to going ahead with Hematopoietic Stem Cell Transplantation (HSCT). There are many therapeutic options however few recover spontaneously, among the available options include high-dose steroids, Erythropoietin(EPO), Plasma exchange, Donor lymphocyte Infusion (DLI), treatment with Rituximab, Bortezomib, Daratumumab and tapering or discontinuation of immunosuppression. All these options have variable success in the literature ranging from 30% - 70%, Non-responders become red cell transfusion dependent and their quality of life is impaired. We are reporting a novel therapeutic option, Ibrutinib as an armamentarium in treating the PRCA post-HSCT, which works by blocking the Bruton Tyrosine Kinase (BTK) pathway thereby inhibiting the host B cell isoagglutinins production and good clinical response.
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Treatment Options for Congenital Dyserythropoietic Anemias (CDAs): Advances in Bone Marrow Transplantation, Gene Therapy, and Targeted Therapies

Published on: 30th November, 2024

Congenital Dyserythropoietic Anaemia (CDA) is a rare genetic disorder that affects the maturation of red blood cells. The disorder is classified into different types, with a prevalence ranging from 1 in 100,000 to 1 in 1,000,000 individuals. Treatment strategies are designed with the primary focus on symptom management, the prevention and treatment of complications, and the underlying disease pathophysiology. The advent of bone marrow transplantation, gene therapy, and targeted therapies has considerably expanded the scope for therapeutic intervention in CDAs. Supportive care, including blood transfusions and iron chelation therapy, has demonstrated efficacy in managing iron overload and improving overall survival rates. The potential of gene therapy, targeted therapies, and hematopoietic growth factors in the treatment of CDA is currently being investigated. Further research and clinical trials are required to develop more effective and personalized therapeutic interventions.
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A Study of Endoscopic and Clinical Profile of Dyspepsia in Afghan Momand Medical Complex & Research Center, Eastern Region, Nangarhar, Afghanistan

Published on: 10th January, 2025

Dyspepsia refers to acute, chronic, or recurrent pain or discomfort centered in the upper abdomen. An international committee of clinical investigators (Rome III Committee) has defined Dyspepsia as Epigastric pain or Burning, early satiety, or postprandial fullness Dyspepsia occurs in 15 % of the Adult population and accounts for 3% of general medical office visits. The descriptive study was carried out in Eastern Afghanistan, Afghan Momand Medical Complex & Research Center, Department of Gastroenterology, during the years 2023-2024. To know the Clinico-Endoscopy of Dyspepsia, for patients who present with investigated Dyspepsia to Gastroenterological OPD & IPD. The study result showed, there were a total of patients 467, and of these patients 230 were Male and 237 were Female. According to the Age, 45 patients were under Age <20, 219 patients were between the ages 21-40 years, 130 patients were between ages 41-60 years and 73 patients were over 60 years of Age. However according to the presentation, 357 patients had Dyspepsia, 59 patients had Dysphagia, 13 patients had Chronic Liver Diseases & Screened for Varices, 3 patients presented after Acid ingestion, 2 patients had Anemia, 11 patients had persistent vomiting and 22 patients had suspected GI-Bleeding. Furthermore in Esophageal findings from a total of 467 patients 34 patients had Esophageal Candidacies, 6 patients had Esophageal Narrowing, 25 patients had Esophageal Growth, 71 patients had Hiatus Hernia, 180 patients had LA Grade A Esophagitis, 21 patients had LA Grade B Esophagitis, 6 patients had LA Grade C Esophagitis, 3 patient had LA Grade D Esophagitis. 16 patients had Lax Lower Esophageal Sphincter, 9 Patients had High-Risk Esophageal Varices, 3 patients had Small Esophageal Varices and 150 patients had Normal Esophagus and in Gastric findings from total all above patients, 4 patients had Antral Gastritis, 50 patients had Corpus Gastritis, 1 patient had Fundal Gastritis, 4 patients had Nodular Gastritis, 199 patients had Pan-Gastritis, 108 patients have Severe Pan Erosive Gastritis, 23 patient had Gastric Erosions, 6 patients had PHG (Portal Hypertensive Gastropathy), 3 Patients had Fundal Varices, 5 patients had Gastric Polyps, 5 patients had Gastric Growth, 2 patients had Pyloric Stenosis, 8 patients stomach not seen due to Esophageal Growth, 67 patients stomach were normal. In the present study there were Duodenal findings from a total of the above patients 48 had Duodenitis, 2 patients had Duodenal Polyps, 2 patients had duodenal ulcers, 1 patient had Duodenopathy, 2 patients had Duodenopathy, 2 patients Duodenum not seen due to Pyloric Stenosis and 413 patients Duodenum were normal. Regarding the habits in our study in which a total of 467 patients were examined 135 of the above patients had snuff habits, 103 patients had smoking habits, 2 patients were Heroin addicts and 227 of the total patients didn’t have any habits (the majority of them were using spicy food, overfeeding, drinking of nonalcoholic beverages, fatty foods, NSAIDs ...) and the higher incidence of Dyspepsia was in Female patients, Also with the age between 21-40 years (43%) patients, Also the higher incidence of Dysphagia 47% were in patients with Age >60 years, the Endoscopy showed that 83% patients had Gastritis also higher incidence regarding the habits 29% had snuff habit and 22% had a smoking habit.
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Insights into the Complexity of Paradoxical Antioxidants Behavior. And the Reasons for it’s almost Zero or no Effect on Stroke

Published on: 31st January, 2025

Antioxidants are groups of compounds that neutralize free radicals and Reactive Oxygen Species (ROS) in the cell [1]. Antioxidant activity in food and beverages has become one of the most interesting features in the science community. These antioxidants provide protection against damage caused by free radicals played important roles in the development of many chronic diseases including cardiovascular diseases, aging, heart disease, anemia, cancer, and inflammation [2].
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Exploring the Potential of Medicinal Plants in Bone Marrow Regeneration and Hematopoietic Stem Cell Therapy

Published on: 31st January, 2025

Blood cell production through hematopoiesis within the bone marrow serves both to maintain blood equilibrium and to respond to tissue injury and infectious demands. Hematopoietic stem cell (HSC) therapy developments have revolutionized medical treatment approaches for anemia leukemia and bone marrow failure caused by chemotherapy or radiation exposure. The therapeutic compounds present in medicinal plants have traditionally supported blood health and researchers now understand these plants could help regenerate bone marrow tissue. The analysis investigates how phytochemicals affect HSC proliferation and differentiation while supporting HSC survival. The medicinal plants Panax ginseng, Astragalus membranaceus, and Curcuma longa receive special attention for their documented ability to enhance hematopoiesis in preclinical and clinical settings. This review examines the challenges that include standardization issues, toxicity concerns, and regulatory barriers alongside future perspectives about combining plant-based therapies with traditional treatments to improve bone marrow recovery and health results. 
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