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.
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.
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.
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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