anemia

Systemic sclerosis sine scleroderma is a rare subset of systemic sclerosis with isolated organ involvement. Scleroderma renal crisis is a severe manifestation of systemic sclerosis characterized by malignant hypertension, oligo/anuric renal failure, and thrombotic microangiopathy. We present a case of a 55-year-old male with uncontrolled hypertension who presented with hematospermia and was found to have acute renal failure, microangiopathic hemolytic anemia, concerning thrombotic microangiopathy. Empiric management for thrombotic thrombocytopenic purpura (TTP) with plasma exchange and corticosteroids yielded a paradoxical response, ultimately leading to the diagnosis of systemic sclerosis sine scleroderma presenting as scleroderma renal crisis (SRC) after serological confirmation. Given the morbidity and mortality associated with scleroderma renal crisis, it should be increasingly considered as a differential for thrombotic microangiopathy even without outward manifestations of systemic sclerosis. Additionally, the empiric management of TTP can include the use of corticosteroids which can exacerbate SRC, an early clinical clue in the diagnosis of this disease.

Background: 800 women die and 2.6 million stillbirths occur worldwide related to pregnancy complications. Racial/ethnic disparities in pregnancy-related mortality have continued to be significantly higher among black than whites due to various factors. We sought to investigate complications among pregnant women of different race/ethnicity.Methods: Cross-sectional observational study of 2030 obstetric cases randomly selected for the period January 1 to December 31, 2021. Data was collected from the hard copy and electronic inpatients’ records. Analysis was performed using SPSS version 23. Descriptive statistics analyzed the pregnancy complication frequencies, standard deviations, range, minimum and maximum values. Maternal characteristics were analyzed using an independent samples t-test. Maternal characteristics were evaluated using the two samples t-test. The odds ratios and confidence intervals were calculated as measures of association between ethnicity/race and pregnancy complications using a binary logistic regression model. Confidence interval was set at 95% and p < 0.05 (2-tailed) was considered statistically significant.Results: 76.25% of Chinese and 67.86% of Zambians were affected by one or more complications. The mean ± standard deviation for MGH [age (26.69 ± 7.33), gravidity (3.35 ± 2.08), and parity (2.07 ± 1.68)] and for 2nd affiliated hospital was [age (30.04 ± 4.29), gravidity (2.19 ± 1.38) and parity (0.45 ± 0.55)]. Prevalence of top five pregnancy complications in the Chinese group was gestational diabetes mellitus at 18.41%, hypothyroidism at 15.91%, oligohydramnios at 14.39%, premature rupture of membranes at 12.17%, and anemia at 5.73%. The prevalence of the top five pregnancy complications in the Zambian group was preeclampsia at 13.80%, PIH at 12.74%, PROM at 12.45%, eclampsia at 7.53%, and placenta abruption at 7.43%. Statistical significance findings were noted as follows: Oligohydramnios [OR 0.02, CI (0.01 - 0.05), p = 0.000], placenta praevia [OR 0.08, CI (0.01 - 0.61), p = 0.015], preeclampsia [OR 13.10, CI (7.22 - 23.78), p = 0.000], placenta abruptio [OR 79.73, CI (11.07 - 574.38), p = 0.000], PIH [OR 11.95, CI (6.57 - 21.73), p = 0.005], eclampsia [OR 162.90, CI (10.08 - 2631, p = 0.000), PPROM [OR 0.03, CI (0.00 - 0.45), p = 0.012], GDM [OR 0.11, CI (0.07 - 0.17), p = 0.000], hypothyroidism [OR 0.01(0.00-0.03), p = 0.000], anemia [OR 0.18, CI (0.92-0.34), p = 0.000], ICP [OR 0.03, CI (0.00 - 0.48), p = 0.013], syphilis [OR 7.17, CI (2.14 - 24.02), p = 0.001], UTI [OR 22.55, CI (3.04 - 17.26), p = 0.002], HBV [OR 0.05, CI (0.00 - 0.86), p = 0.039] and GBS [OR 0.06, CI (0.00 - 1.11), p = 0.059].Conclusion: Highest odds for obstetrical and infection-related pregnancy complications were associated with Zambian cases. The highest odds for medical complications were associated with Chinese cases.

Although intussusception occurs in children and adults with celiac disease, it is a relatively uncommon symptom. Even more rare is the occurrence of intussusception as the presenting symptom of the disease. In the two cases we report here, transient intussusception, occurring at three years of age, was the first and only physical sign of celiac disease, and lead to a timely diagnosis by immunoserology and histology, followed by implementation of a gluten-free diet before sequelae such as significant anemia or Failure to Thrive (FTT) developed. In both cases, neither immunoserological nor physical signs of disease were present at the follow-up examination after 6 months on a gluten-free diet. In addition, genetic screening of the patients’ families revealed HLA-DQ2 positivity in two cases, leading to the additional diagnosis of celiac disease in the pregnant mother of one of the patients.

Immune cytopenias occur when the body produces antibodies that target specific hematopoietic cells, inducing extravascular antibody-mediated phagocytosis by monocyte-macrophages in the spleen and/or liver through activation of Fcγ Receptors (FcγRs). Immune cytopenias include Immune Thrombocytopenia (ITP), Autoimmune Hemolytic Anemia (AIHA), Hemolytic Transfusion Reactions (HTR), Hemolytic Disease of the Fetus and Newborn (HDFN), and Autoimmune Neutropenia (AIN). Thus, novel therapeutics that inhibit phagocytosis would be useful, especially for short-term use while other therapies are being evaluated. In our earlier studies, we successfully identified two small-molecule drugs able to inhibit in vitro phagocytosis with a low IC50 concentration and negligible toxicity. These drugs, known as KB-151 and KB-208, have the potential to be utilized as lead compounds for further studies, once their mechanism of action is more clearly understood. In this regard, we have developed preliminary results that suggest that these small molecules may bind to the Fc receptors on monocyte macrophages and block the subsequent attachment of antibody-opsonized red blood cells to prevent phagocytosis.

Introduction: In the present study we evaluated and compared RBC parameters, iron status, and ferritin for discriminating between patients with iron deficiency anemia and anemia of chronic disease. Anemia that accompanies infection, inflammation, and cancer, is commonly termed anemia of chronic disease (ACD). Methods: We compared the ability of serum ferritin concentration, using the microplate immunoenzymometric assay method with other, more traditional indicators of iron status like total iron binding capacity [TIBC], mean corpuscular volume [MCV], percent transferrin saturation [%TS], RBC distribution width [RDW], and serum iron concentration [SIC]. The ferritin concentration was determined in 80 serum samples selected from men and women above the age of 18 years. The patients were assigned to IDA and ACD groups based on serum ferritin concentration.Observation: By studying the ROC Curve for various red cell parameters for the diagnosis of IDA and ACD, we found that diagnostic accuracy of various indicators was as follows TIBC>TS%>MCV>MCH>SI>MCHC for anemia of chronic diseases, and TIBC>MCH>MCV>MCHC>TS%>SI for iron deficiency anemia. When both the value of AUCs (Area under Curve) of ROC were compared it is apparent that TIBC, TS%, MCV, and MCH are important discriminating factors between IDA and ACD. Conclusion: Conventional laboratory parameters play an important role in distinguishing overt causes of IDA and ACD. MCV, MCH, and TIBC were found to be (p -value < .05) significantly discriminated against IDA and ACD. Serum ferritin is an important diagnostic tool with reasonable accuracy for the detection and differentiation of iron deficiency anemia and anemia of chronic disease.

Background: Stroke occurs in 11% of patients with SCA before 20 years of age. In Northwestern Tanzania, the prevalence of stroke among children living with SCA under the age 15 years is 16.9%, of which might be attributed to the absence of routine screening for the risk of stroke by using Transcranial Doppler Ultrasound (TCD). Screening with TCD allows preventive measures such as chronic blood transfusion to be done which has led to the reduction of stroke by 92%.Methods: This was a prospective analytical cross sectional study which enrolled 267 SCA children aged 2 to 16 years attending Bugando Medical Centre Pediatric Sickle Cell Clinic from July 2019 to June 2020. Assessment of factors associated with elevated TCD included a clinical history of stroke in sibling, death in sibling, temperature, oxygen saturation in room air, blood pressure, hemoglobin level and total white blood cell count. TCD was done by accessing transtemporal window and recording the highest time average mean of maximum velocity (TAMMV) of major vessels mainly, middle cerebral artery (MCA) and distal internal carotid artery (dICA).Results: The median age of enrolled was 6.6 (IQR: 4-9) years. The prevalence of elevated TCD (> 170 cm/s)was found to be 21% (56/267). By multivariate logistic regression, low oxygen saturation in room air, p - value = 0.037, OR 1.08 [95% CI 1.00-1.17] and low hemoglobin level, p - value = 0.001, OR 1.76 [95% CI 1.26-2.45] were statistically significantly associated with elevated TCD among children living with SCA.Conclusion: The high prevalence of elevated TCD velocity, with low hemoglobin and low oxygen saturation in room air as associated factors under multivariate logistic regression, warrants routine TCD screening for children with SCA aged 2 to 16 years.

Background: Grand multiparty is common obstetrical problem, in Sudan large families is desirable for cultural and religious backgrounds and higher incidence of grandmultipra is expected, the risk factors associated with adverse maternal outcomes have yet to be adequately investigated among grand multiparity need to delivered by primary cesarean section.Objective: The main objective was to determine impact of primary cesarean section on grand multiparous, it is indications and complications.Methodology: It was a descriptive prospective cross-sectional hospital-based study conducted at Omdurman Maternity Hospital during period October 2016 to March 2017.An interview questionnaire was used for data collection. Demographic and clinical data concerning personal history, parity, indications of primary cesarean section, type of Cs, maternal complication and neonatal complications were recorded. Also, multiparous less than five delivery, previous lower segment caesarean section, known medical disorders except anemia and twin pregnancy were excluded.Results: During the study period total of 113 grand multipara included, incidence of primary cesarean section in grand multipara was 10%. Indication in our study 22.1% due to malpresentation, fetl distress 15% and prolonged first stage 13.4%, prolonged second stage 12.4% and antepartum haemorrhage 11.5%.  Postpartum haemorrhage developed in 9.7%, hysterectomy 1.8%, uterine tear 5.4% bladder injury fetal laceration 3.6%, spinal anesthesia headache 7%, post-partum pyrexia 5.3%, sepsis 4.4%, urinary tract infections were 2.7%.Conclusion: The finding in this study showed 10% incidence of primary cesarean section in grandmultipra. The most indications of primary cesarean section in grandmultipra malpresentation, fetal distress, prolonged first and second stage of labour. Most CS were emergency. 

Despite decades of global and country commitments towards eradicating malaria, malaria remains the most hazardous parasitic disease and the most common cause of fever for humans, especially in tropical countries. Plasmodium falciparum causes 90% of malaria cases. Coma [Cerebral Malaria (CM)], acidosis, hypoglycemia, severe anemia, renal dysfunction, and pulmonary edema are the most common complications of malaria caused by Plasmodium falciparum and the most common cause of death related to malaria. People from less prevalent malaria areas are at high risk of developing these complications. A 16-year-old male from a low malaria transmission area was diagnosed with CM. Prior to developing CM, he was treated with Coartem. CM is a medical emergency and one of the forms of severe malaria. CM has high mortality and morbidity rates. Yet, international health-related agencies, funders, and policy-makers are unfamiliar with it. The continuous occurrence of CM validates the considerable need for global investment in malaria control and elimination programs. Early administration of Artesunate to all patients suspected of having severe malaria would reduce global malaria-related mortality and morbidity. Simple tests, such as the determination of malaria parasitic density either with thin or thick blood smears, may influence the proper management of all severe malaria cases. However, in clinical practice, the determination of malaria parasitic density is not routinely done. Further commitments are needed to ensure routine determination of malaria parasitic density for all suspected severe malaria cases. Moreover, further commitments are needed to guarantee the proper management of CM because it is a major cause of reversible encephalopathy in tropical countries.

Introduction: Factor VII (FVII) deficiency, a rare bleeding disorder, can manifest as an autosomal recessive congenital or an acquired coagulopathy. Acquired FVII deficiency, although infrequently reported, presents unique challenges in understanding its mechanisms and identifying underlying causes. Case presentation: We present a case of acquired FVII deficiency discovered in a 23-year-old female patient with no apparent underlying disease. The patient exhibited spontaneous ecchymosis and gingival hemorrhage, along with low FVII activity and isolated prolongation of prothrombin time. Extensive laboratory investigations excluded liver dysfunction, familial deficiency, vitamin K deficiency, and inhibitory antibodies. Prompt treatment with Fresh Frozen Plasma (FFP) and bypassing agents resulted in a favorable response and resolution of hematomas. Conclusion: Acquired FVII deficiency was identified with bleeding symptoms in association with prolonged prothrombin time and a low level of FVII activity. In literature, this deficiency has been associated with various conditions such as sepsis, aplastic anemia, stem cell transplantation, and neoplasms, although approximately 14% of cases remain idiopathic. Clinical outcomes remain generally poor, with limited complete remissions reported.

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.

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.

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).

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.

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.

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.

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].

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. 

A 24-year-old male presented with epistaxis and gingival bleeding, physical examination revealed pallor and purpuric spots on the left upper limb. Laboratory findings showed pancytopenia. As part of the etiological workup for pancytopenia, a bone marrow aspirate was performed, revealing moderately cellular marrow with rare megakaryocytes and intranuclear inclusions in proerythroblasts. Parvovirus B19 testing in the bone marrow was conducted via nucleic acid extraction followed by PCR.