Thrombotic Thrombocytopenic Purpura and Systemic Lupus Erythematosus: A Rare and Life-threatening Association

Introduction: The association between thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) is uncommon. Diagnosis is often difficult because of their clinical and biologicalsimilarities. The presence of TTP in SLE worsens the prognosis and causes high mortality in the absence of early therapeutic interventions.Case report: We report the case of a 20 year-old man, admitted with nephrotic range proteinuria, hematuria and rapidly progressive renal failure. He also had anemia, thrombocytopenia and pericardial effusion.The diagnosis of SLE was made based on these clinical findings along with positive antinuclear and anti dsDNA antibodies. Renal biopsy revealed class IV/ V lupus nephritis (LN) with active lesions of thrombotic microangiopathy. The evolution of neurological deficit, persistent thrombocytopenia and active microangiopathic changes suggested the diagnosis of associated TTP. The patient was treated initially with corticosteroids and cyclophosphamide. Plasmapheresis could only be started 16 days later. Mycophenolate mofetil and rituximab weresuccessively tried in the absence of improvement in renal function and persistent thrombocytopenia. The patient’s neurological condition deteriorated necessitating transfer to the intensive care unit and mechanical ventilation. There he developed pneumonia and died of septic shock two months after presentation.Conclusion: The coexistence of TTP and SLE needs to be considered early in SLE patients with complicated course. It may not respond to the conventional immunosuppressive treatment of SLE

Assessment of Commonly Used Pesticides in the Ground Water of the Shallow Aquifer Systems in Jericho and Jeftlik areas/ Lower Jordan Valley, Occupied Palestinian Territories

One of the most important pollutants that may reach the groundwater through agricultural return flow combined with abuse and ignorance is pesticides. This study focuses on the examination of the concentration of three pesticides: Abamectin, Imidacloprid, and ß-Cyfluthrin, all of which have been used in large quantities in the Lower Jordan Valley (LJV) for the last three decades. Twenty five groundwater samples were collected from water boreholes where water is abstracted from two phreatic aquifer systems which are the Plio-Plistocene aquifer system in Jericho and Lower Al Jeftlik areas and the Eocene carbonate aquifer system in the Middle of Al Jeftlik. The depth of the boreholes in both aquifer system ranges between 80 and 120 m. Water samples were analyzed for Abamectin, Imidacloprid, and ß-Cyfluthrin using the HPLC-UV method. These samples represent two main agricultural locations (Jericho, and the Al Jeftlik). Of the 25 wells sampled, Abamectin was detected in 11 wells in concentrations ranging between 1.24 ppb and 81.71ppb. Imidacloprid was detected in 24 wells in concentrations ranging between 1.60ppb and 325.0ppb. Finally, ß-Cyfluthrin was detected in 7 wells in concentrations ranging between 1.10 and 24.46ppb. Aquifer lithology, groundwater flow directions, type of agricultural activity are major factors in controlling pesticide concentrations in groundwater. The highest values were measured where the aquifer consists of gravel and sand sediments, combined with intensive agricultural activities, followed by sand-silt aquifer. The lowest concentrations were found in boreholes where carbonate aquifer is the main source of water which indicates that other source of water flow into the system. The results of this study demonstrate that these pesticides are used heavily and in an improper way in the lower Jordan Valley, increasing the risk of adverse environmental and public health effects. Much attention should be given to addressing the potential problem of environmental and groundwater contamination by these pesticides.This study was funded through BARD-project /USDA

Time Savings Using a Digital Workflow versus a Conventional for Intraocular Lens Implantation in a Corporate Chain Hospital Setting [Corrigendum]

Rombold F, Niederdellmann C, Pariti R, Khayat B. Clin Ophthalmol. 2024;18:113-119. On page 113, Abstract, Results subheading, “The digital cataract workflow resulted in shorter mean (± standard deviation [SD]) preoperative assessments with lesser variability among individual assessments than the existing workflow (14.15 ± 1.86 vs 21.41 ± 1.18 min, respectively); with a time saving of 35%” should have been “The digital cataract workflow resulted in shorter mean (± standard deviation [SD]) preoperative assessments with lesser variability among individual assessments than the existing workflow (14.15 ± 1.18 vs 21.41 ± 2.26 min, respectively); with a time saving of 35%”. On page 115, Results section, Preoperative Assessments, 2nd paragraph, “The overall mean (±SD) process time for the preoperative assessments was reduced in the digital cataract workflow (14.15 [±1.86] min) compared to the existing conventional workflow (21.41 [±1.18] min) with an overall time saving of approximately 35% (Table 1)” should have been “The overall mean (±SD) process time for the preoperative assessments was reduced in the digital cataract workflow (14.15 [±1.18] min) compared to the existing conventional workflow (21.41 [±2.26] min) with an overall time saving of approximately 35% (Table 1)”. On page 116, Table 1, Preoperative assessments# row, the standard deviation values in the 3rd and 4th columns were flipped in error, 14.15 ± 1.86 should be 14.15 ± 1.18 and 21.41 ± 1.18 should be 21.41 ± 2.26. The authors apologize for any inconvenience caused by these errors and for any confusion that may have arisen as a result. They would like to assure the readers that these corrections do not affect the results or conclusions of the paper

Histopathological Study of Pure Primary Nephrotic Syndrome in Adolescents and Young Moroccan Adults

Introduction: The primary nephrotic syndrome (PNS) is the most common glomerular nephropathy in children. Its diagnosis and management don’t require histopathological study. It occurs mainly in the form of minimal glomerular lesion and in most cases respond to corticosteroids. The literature on histological lesions of pure PNS in adolescents and young adults is rare. Thus, there are no criteria or recommendations regarding the indications for renal biopsy in patients aged 12-18 years. Methods: This is a retrospective study in which we encountered a total of 386 patients aged 12 to 25 years who were admitted and biopsied at the Nephrology Unit of Ibn Roshd Hospital in Casablanca during the period from January 1st, 2000 to September 30th, 2009 . Patients with pure PNS were 77 (20%), all were included in this study. Results: The average incidence of pure PNS was 7.7 cases per year. The study included 47 males (61%) and 30 females (39%). Patients were sent from all parts of Morocco and the average length of hospital stay was four days. Renal biopsies showed the following morphological lesions: minimal glomerular lesions in 61 cases (79.20%), focal segmental hyalinosis in 7 cases (9.10%), extramembranous glomerulonephritis in 7 cases (9.10%) and 2 cases of renal amyloidosis (2.6%). Conclusion: The minimal glomerular lesions were the most common cause of pure primary nephrotic syndrome in patients aged 12-25 years. Initial renal biopsy may not be indicated in this age group, and an empiric therapeutic trial with corticosteroids may be initially considered.Keywords: Glomerular Disease; Children; Nephrotic Syndrome; Renal Biops

GALLSTONES IN PATIENTS WITH INHERITED HEMOLYTIC DISEASES

The purpose is to provide an overview on the incidence of gallstone disease in patients with various types of inherited (chronic) hemolytic diseases at risk of cholelithiasis/choledocholithiasis with particular emphasis on its pathogenesis, genetic, risk factors and management. A detailed electronic literature search to determine the source of materials for this review article was done. The reported incidences of gallstones and choledocholithiasis vary according to the different types of inherited hemolytic diseases and the ethnicity of the studied populations. To date, no review article summarises the incidences of cholelithiasis in patients with various inherited haemolytic diseases was published. Regular ultrasound examination for the presence of gallstones recommended in patients with inherited haemolytic anaemias, particularly those with additional risk factors recommended. Further studies for evaluating the reasons for the higher incidence of cholelithiasis in thalassemia major and sickle cell anemia compared to hereditary spherocytosis; the effect of co inheritance of alpha thalassaemia on decreasing bilirubin level in patients with sickle cell disease and beta thalassaemia; the effect of the co inheritance of UGT1A1 and ABCG8 gene mutation on the incidence of gallstones in other blood diseases such as Hb-H disease, autoimmune haemolytic anaemias, congenital dyserythropoietic anaemia, hereditary elliptocytosis, Southeast Asian Ovalocytosis, glucose-6-phosphate and pyruvate kinase deficiency are recommended. Evaluation of the potential role of the solubility of the mutant proteins and haemoglobin subunit in the red blood cells as an additional mechanism for the development of gallstones in patients with inherited haemolytic anaemias recommended

Diagnosing growth in low-grade gliomas with and without longitudinal volume measurements: A retrospective observational study.

BACKGROUND: Low-grade gliomas cause significant neurological morbidity by brain invasion. There is no universally accepted objective technique available for detection of enlargement of low-grade gliomas in the clinical setting; subjective evaluation by clinicians using visual comparison of longitudinal radiological studies is the gold standard. The aim of this study is to determine whether a computer-assisted diagnosis (CAD) method helps physicians detect earlier growth of low-grade gliomas. METHODS AND FINDINGS: We reviewed 165 patients diagnosed with grade 2 gliomas, seen at the University of Alabama at Birmingham clinics from 1 July 2017 to 14 May 2018. MRI scans were collected during the spring and summer of 2018. Fifty-six gliomas met the inclusion criteria, including 19 oligodendrogliomas, 26 astrocytomas, and 11 mixed gliomas in 30 males and 26 females with a mean age of 48 years and a range of follow-up of 150.2 months (difference between highest and lowest values). None received radiation therapy. We also studied 7 patients with an imaging abnormality without pathological diagnosis, who were clinically stable at the time of retrospective review (14 May 2018). This study compared growth detection by 7 physicians aided by the CAD method with retrospective clinical reports. The tumors of 63 patients (56 + 7) in 627 MRI scans were digitized, including 34 grade 2 gliomas with radiological progression and 22 radiologically stable grade 2 gliomas. The CAD method consisted of tumor segmentation, computing volumes, and pointing to growth by the online abrupt change-of-point method, which considers only past measurements. Independent scientists have evaluated the segmentation method. In 29 of the 34 patients with progression, the median time to growth detection was only 14 months for CAD compared to 44 months for current standard of care radiological evaluation (p \u3c 0.001). Using CAD, accurate detection of tumor enlargement was possible with a median of only 57% change in the tumor volume as compared to a median of 174% change of volume necessary to diagnose tumor growth using standard of care clinical methods (p \u3c 0.001). In the radiologically stable group, CAD facilitated growth detection in 13 out of 22 patients. CAD did not detect growth in the imaging abnormality group. The main limitation of this study was its retrospective design; nevertheless, the results depict the current state of a gold standard in clinical practice that allowed a significant increase in tumor volumes from baseline before detection. Such large increases in tumor volume would not be permitted in a prospective design. The number of glioma patients (n = 56) is a limitation; however, it is equivalent to the number of patients in phase II clinical trials. CONCLUSIONS: The current practice of visual comparison of longitudinal MRI scans is associated with significant delays in detecting growth of low-grade gliomas. Our findings support the idea that physicians aided by CAD detect growth at significantly smaller volumes than physicians using visual comparison alone. This study does not answer the questions whether to treat or not and which treatment modality is optimal. Nonetheless, early growth detection sets the stage for future clinical studies that address these questions and whether early therapeutic interventions prolong survival and improve quality of life

Fibrinogen concentrate for treatment of bleeding and surgical prophylaxis in congenital fibrinogen deficiency patients

Background: Congenital fibrinogen deficiency is an ultra-rare disorder in which patients can experience severe and/or frequent bleeding episodes (BEs). Here, we present the largest prospective study to date on the treatment of this disorder. Methods: Hemostatic efficacy of human fibrinogen concentrate (HFC; FIBRYGA\uae, Octapharma AG) for treatment of bleeding or surgical prophylaxis was assessed by investigators and adjudicated by an independent data monitoring and endpoint adjudication committee (IDMEAC) according to a four-point scale, using objective criteria. Thromboelastometry maximum clot firmness (MCF) was also determined. Results: Twenty-five afibrinogenemia patients were treated with HFC: 24 for on-demand treatment of 89 BEs, and nine as prophylaxis for 12 surgeries. For BEs, treatment success (rating of excellent or good) evaluated by investigators was 96.6% (90% confidence interval [CI], 0.92-0.99; two missing ratings, classified as failures) and by the IDMEAC was 98.9% (90% CI, 0.95-0.999). Mean \ub1 standard deviation (SD) increase in MCF was 5.8 \ub1 2.5 mm one hour after the first HFC infusion (mean \ub1 SD dose, 61.88 \ub1 11.73 mg/kg). For the 12 surgeries (median [range] HFC dose/surgery, 85.80 mg/kg [34.09-225.36]), intraoperative and postoperative treatment success were both rated 100% (90% CI, 0.82-1.00) by investigators and the IDMEAC. Three adverse events were possibly treatment related, including a moderate case of thrombosis. There were no deaths, no severe allergic or hypersensitivity reactions, and no clinical evidence of neutralizing antifibrinogen antibodies. Conclusions: Human fibrinogen concentrate was efficacious for on-demand treatment of bleeding and as surgical prophylaxis, with a favorable safety profile, in patients with congenital afibrinogenemia

Interrelationship between TP53 gene deletion, protein expression and chromosome 17 aneusomy in gastric adenocarcinoma

Background: This study evaluates the existence of numerical alterations of chromosome 17 and TP53 gene deletion in gastric adenocarcinoma. the p53 protein expression was also evaluated, as well as, possible associations with clinicopathological characteristics.Methods: Dual-color fluorescence in situ hybridization and immunostaining were performed in twenty gastric cancer samples of individuals from Northern Brazil.Results: Deletion of TP53 was found in all samples. TP53 was inactivated mainly by single allelic deletion, varying to 7-39% of cells/case. Aneusomy of chromosome 17 was observed in 85% of cases. Chromosome 17 monosomy and gain were both observed in about half of cases. Cells with gain of chromosome 17 frequently presented TP53 deletion. the frequency of cells with two chr17 and one TP53 signals observed was higher in diffuse than in intestinal-type GC. Immunoreactivity of p53 was found only in intestinal-type samples. the frequency of cells with two chr17 and two TP53 signals found was higher in samples with positive p53 expression than in negative cases in intestinal-type GC.Conclusion: We suggest that TP53 deletion and chromosome 17 aneusomy is a common event in GC and other TP53 alterations, as mutation, may be implicated in the distinct carcinogenesis process of diffuse and intestinal types.Financiadora de Estudos e Projetos (FINEP CT-INFRA/FADESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fed Univ Para, Inst Biol Sci, Humans Cytogenet Lab, BR-66075900 Belem, Para, BrazilUniv Fed Piaui, Dept Biol, Campus Minist Reis Velloso Parnaiba, Teresina, PI, BrazilUniversidade Federal de São Paulo, Dept Morphol, Div Genet, São Paulo, BrazilUniv Fed Ceara, Sch Med, Dept Pathol, Mol Genet Lab, Fortaleza, Ceara, BrazilFed Univ Para, Joao de Barros Barreto Univ Hosp, BR-66075900 Belem, Para, BrazilUniversidade Federal de São Paulo, Dept Morphol, Div Genet, São Paulo, BrazilFinanciadora de Estudos e Projetos (FINEP CT-INFRA/FADESP): 0927-03. RRBWeb of Scienc