Shift in emergency department utilization by frequent attendees with sickle cell disease during the COVID-19 pandemic: A multicentre cohort study.

While the coronavirus disease-2019 (COVID-19) might have increased acute episodes in people living with sickle cell disease (SCD), it may also have changed their reliance on emergency department (ED) services. We assessed the impact of the COVID-19 pandemic and lockdowns on ED visits in adult SCD people followed in five French reference centres, with a special focus on 'high users' (≥10 visits in 2019). We analysed the rate of ED visits from 1 January 2015 to 31 December 2021, using a self-controlled case series. Among 1530 people (17 829 ED visits), we observed a significant reduction in ED visits during and after lockdowns, but the effect vanished over time. Compared to pre-pandemic, incidence rate ratios for ED visits were 0.59 [95% CI 0.52-0.67] for the first lockdown, 0.66 [95% CI 0.58-0.75] for the second and 0.85 [95% CI 0.73-0.99] for the third. High users (4% of people but 33.7% of visits) mainly drove the reductions after the first lockdown. COVID-19 lockdowns were associated with reduced ED visits. While most people returned to their baseline utilization by April 2021, high users had a lasting decrease in ED visits. Understanding the factors driving the drop in ED utilization among high users might inform clinical practice and health policy.

Exploration de l'hémolyse associée à la drépanocytose et perspectives thérapeutiques spécifiques: Sickle cell disease hemolysis investigations and targeted therapeutics prospects.

Sickle Cell Anemia is a disease with a strong vascular tropism. Beyond anemia, the pathophysiological mechanisms responsible for hemolysis, directly affect both acute and chronic vascular damages, thus resulting in a systemic disease. Understanding the different types of hemolysis underline the need for novel specific biomarkers. Targeted therapeutic approaches for these pathophysiological pathways are necessary to improve Sickle Cell patients' prognosis. Finally, given its complexity, Sickle Cell Disease is often used as a "proof of concept" for other pathologies. It seems likely that the rapidly evolving knowledge in this field will also benefit other diseases. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Evaluation of a prophylactic transfusion program on obstetric outcomes in pregnant women with sickle cell disease: A single centre retrospective cohort study.

OBJECTIVE: To evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD). METHODS: This retrospective cohort study included all singleton pregnancies among women with SCD in a French university tertiary care center between 1 January 2004 and 31 December 2017. The TP group included patients selected according to the French guidelines who received regular red blood cell transfusions during pregnancy until delivery. The factors associated with TP indication [year of birth, SCD genotype, history of acute chest syndrome and delayed hemolysis transfusion reaction (DHTR) risk score] were taken into account in a propensity score. A composite obstetric adverse outcome was defined associating birth before 34 gestational weeks and/or pre-eclampsia and/or small for gestational age and/or abruption and/or stillbirth and/or maternal death and/or neonatal death. RESULTS: In total, 246 pregnancies in 173 patients were analyzed. Twenty-two pregnancies with a history of DHTR were excluded. A higher frequency of TP was found before 2013 [119/148 (80.4%) vs 38/76 (50%); p < 0.001]. Rates of preterm birth before 34 gestational weeks (5.6% vs 19.7%; p = 0.001), vaso-occlusive crisis (36.5% vs. 61.8%; p < 0.001), and acute chest syndrome (6.1% vs. 14.5%; p = 0.04) during pregnancy were decreased significantly in the TP group. Among the groups with and without composite obstetric adverse outcomes, the frequency of TP was 52.6% and 74.7%, respectively [odds ratio (OR) 0.30, 95% confidence interval (CI) 0.09-1.02]. The multivariate analysis shows that the TP was associated with a significant reduction in the risk of composite obstetric adverse outcomes (OR 0.28, 95% CI 0.08-0.97; p = 0.04). CONCLUSION: A red blood cell TP may have an independent protective effect on maternal and perinatal adverse outcomes during pregnancy in women with SCD.

Tuberculosis in sickle cell disease patients.

OBJECTIVE: Tuberculosis (TB) disease has rarely been reported in patients with sickle cell disease, but it is associated with an increased risk of bacterial infections. In France, sickle cell disease is frequent in populations with the highest prevalence of TB disease. We aimed to highlight clinical aspects of TB disease in patients with sickle cell disease. PATIENTS AND METHODS: Over a 10-year period, we retrospectively included all adults with sickle cell disease who had a positive culture for Mycobacterium tuberculosis managed in the adult sickle cell center of Henri-Mondor hospital. Sickle cell patients with TB disease were matched for comparison to adults without hemoglobinopathy and with documented TB disease in a 1:2 ratio. Logistic regression mixed models were performed. RESULTS: Twelve patients with sickle cell disease and documented TB disease (median age: 29years; IQR [25-34]) were compared to 24 non-sickle cell patients (median age: 33years; IQR [27.5-38.5]). Baseline characteristics were similar between groups except for sickle cell disease. Ten of the 12 patients with sickle cell disease had pulmonary TB. TB disease characteristics were similar between sickle cell and non-sickle cell patients although sickle cell patients had fewer positive sputum smears for acid-fast bacilli (P=0.003) and fewer lung cavitations (P=0.03). CONCLUSIONS: TB disease in sickle cell patients was globally similar to non-sickle cell patients, even though less infectious. Regular follow-up in specialized centers might allow for earlier TB disease diagnosis in sickle cell patients.

Molecular testing for respiratory pathogens in sickle cell disease adult patients presenting with febrile acute chest syndrome.

BACKGROUND: Differentiating acute chest syndrome (ACS) from community-acquired pneumonia (CAP) is challenging in adults presenting with major sickle cell disease (SCD) (semiological similarity, rare microbiological documentation). We aimed to assess the usefulness of nucleic acid amplification test (NAAT) for respiratory pathogens, in combination with standard bacteriological investigations, in febrile ACS adult patients presenting with major SCD. METHODS: We performed a prospective, monocentric, observational study of 61 SCD adults presenting with febrile ACS from February 2015 to April 2016. Systematic blood, urine, and respiratory specimens were collected, before antibiotic initiation, for culture, urinary antigen tests, serology, and NAAT for respiratory pathogens. RESULTS: A pathogen was detected in 12 febrile ACS (19.7%): four viruses (6.6%) (Rhinovirus; Influenza A/B), seven bacteria (11.4%) (S. aureus, S. pneumoniae, K. pneumoniae, L. pneumophila, M. pneumoniae), one mixed infection (1.6%) (S. aureus and Influenza B). NAAT only detected L. pneumophila in one case (serogroup 2). Apart from a significantly shorter antibiotic therapy duration (6.1 vs. 7.8 days, P=0.045), no difference was observed between undocumented and microbiologically-documented febrile ACS. CONCLUSION: Using NAAT for the detection of respiratory pathogens in adults presenting with SCD slightly improved the microbiological diagnostic of febrile ACS, although respiratory infections are not the main etiological factor.

Management of delayed hemolytic transfusion reaction in sickle cell disease: Prevention, diagnosis, treatment.

Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally underdiagnosed because its biological and clinical features resemble those of vaso-occlusive crisis, and red blood cell antibodies are frequently absent. Further transfusions may aggravate the symptoms, leading to severe multiple organ failure and death. It is therefore essential to prevent, diagnose and treat this syndrome efficiently. Prevention is based principally on the attenuation of allo-immunization through the provision of extended-matched RBCs or the use of rituximab. However, such treatment may be insufficient. Early diagnosis might make it possible to implement specific treatments in some cases, thereby avoiding the need for secondary transfusion. Diagnosis is dependent on the knowledge of the medical staff. Finally, many treatments, including steroids, immunoglobulins, erythropoietin and eculizumab, have been used to improve outcome. Improvements in our knowledge of the specific features of DHTR in SCD should facilitate management of this syndrome.

Improved Fetal Hemoglobin With mTOR Inhibitor-Based Immunosuppression in a Kidney Transplant Recipient With Sickle Cell Disease.

Fetal hemoglobin induction is a key point in the management of sickle cell disease (SCD). We report the case of a kidney transplant recipient with SCD who was treated with everolimus, a mammalian target of rapamycin inhibitor. At 10 months after initiating therapy, the patient's fetal hemoglobin level was dramatically increased (from 4.8% to 15%) and there was excellent tolerance to treatment.

[Symptomatic extramedullary haematopoiesis in ß-thalassemia: A retrospective single centre study]. / Hématopoïèse extra-médullaire symptomatique au cours des ß-thalassémies: étude rétrospective monocentrique.

INTRODUCTION: Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in ß-thalassemia. There are no treatment guidelines. METHODS: Retrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes. RESULTS: Among 182 ß-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients. CONCLUSION: EH must be suspected in ß-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain.

Appendicitis and abscess in an adult patient with intestinal nonrotation: Case report.

The aim of the article is showing a particular case of midgut nonrotation. It is a congenital defect of the bowel development, during which fails the rotation of 270° around the vascular pedicle. This anomaly causes a different intestinal arrangement: the small bowel is located in the right side of abdominal cavity while the large bowel is situated in the left side. We present a case of acute appendicitis and abscess treated successfully with urgent surgical intervention in a patient completely asymptomatic for nonrotation. Nonrotation may lead to acute symptoms, vague abdominal pain or may remain asymptomatic throughout all life and be discovered only accidentally. Radiological exams and laparoscopy can help to make a correct diagnosis. A conservative treatment could be preferred in asymptomatic patients and Ladd's surgical procedure should be performed in selected cases.

[French guidelines for the management of adult sickle cell disease: 2015 update]. / Recommandations françaises de prise en charge de la drépanocytose de l'adulte: actualisation 2015.

Sickle cell disease is a systemic genetic disorder, causing many functional and tissular modifications. As the prevalence of patients with sickle cell disease increases gradually in France, every physician can be potentially involved in the care of these patients. Complications of sickle cell disease can be acute and chronic. Pain is the main symptom and should be treated quickly and aggressively. In order to reduce the fatality rate associated with acute chest syndrome, it must be detected and treated early. Chronic complications are one of the main concerns in adults and should be identified as early as possible in order to prevent end organ damage. Many organs can be involved, including bones, kidneys, eyes, lungs, etc. The indications for a specific treatment (blood transfusion or hydroxyurea) should be regularly discussed. Coordinated health care should be carefully organized to allow a regular follow-up near the living place and access to specialized departments. We present in this article the French guidelines for the sickle cell disease management in adulthood.

The use of rituximab to prevent severe delayed haemolytic transfusion reaction in immunized patients with sickle cell disease.

BACKGROUND: Delayed haemolytic transfusion reaction (DHTR) is mainly caused by an immune response to transfused red blood cells (RBCs). Immunized patients have a high risk of producing antibodies in response to further transfusion. Controlling the immune response to RBCs is therefore a major goal in sickle cell disease (SCD). STUDY DESIGN: We report an observational study of eight alloimmunized SCD patients with history of severe DHTR who were treated with rituximab before a new transfusion to prevent further immunization and DHTR. RESULTS: Five patients showed a good clinical outcome following transfusion preceded by preemptive treatment with rituximab. The remaining patients presented mild DHTR. In all patients, the results of post-transfusion screening tests were identical to those of pretransfusion tests; no newly formed antibodies were detected. CONCLUSION: These cases suggest that rituximab prevents at least occurrence of newly formed antibodies in high responders and minimizes the risk of severe DHTR. This study confirms that DHTR is complex in SCD and does not rely only on the classical antigens/antibodies conflict. Considering potentially serious adverse effect of rituximab, this treatment should be considered cautiously, and only when transfusion is absolutely necessary in patients with history of severe DHTR linked to immunization.

Abdominal emergency in young women: gynecological or bowel disease? Two case reports.

Abdominal pain is a frequent symptom in Emergency Departments. Often is not so easy make a diagnosis of cause. Particular importance in young women has differential diagnosis with gynecological diseases. Often laboratory exams have not good specificity. US and TC are the imaging techniques most used to make a diagnosis, but both have ours limits. Definitely surgeon's experience is the most important resource for a correct approach to abdominal pain. We present two cases of low abdominal pain in young women due to ovarian teratoma erroneously diagnosed as appendicitis.

VAC (Vacuum Assisted Closure) treatment in Fournier's gangrene: personal experience and literature review.

INTRODUCTION: Fournier's gangrene (FG) is a rapidly developing necrotizing fasciitis that originates in genital and perineal region. The mortality rate is high and requires prompt diagnosis, antibiotic treatment and extensive necrosectomy with derivative colostomy. Vacuum Assisted Closure (VAC) is a wound care system of paramount importance in the treatment of complex wounds, including the perineum. MATERIALS AND METHODS: We evaluated 6 cases of FG (males, mean age: 54.6 yrs) of the last 3 years (February 2008-August 2010). All patients were diabetics. We used intravenous antibiotic treatment and early surgical debridement with colostomy, followed by immediate positioning of a VAC device (NP 125-200 mmHg). The dressing changes were done every 3-4 days. Hyperbaric oxygen therapy (HBOT) was given only to one patient. Microbiological etiology was assessed by multiple cultures to tailor the antimicrobial treatment. RESULTS: The VAC therapy reduces the number of dressings and the hospital length of stay (LOS), in agreement with the literature; in one of the cases a secondary reconstructive surgical intervention was possible. The colostomy was reversed in all patients within 3 months. CONCLUSIONS: Negative pressure is a time saving device, reducing days of hospitalization, patient's discomfort and number of medications. The possibility of a early reconstructive surgery improves significantly quality of life.

Ascaris Lumbricoides infestation and intestinal MZBCL: a surgical and radiological perspective.

Ascaris Lumbricoides is the most common worm found in human beings and it is the largest of the intestinal nematodes parasitizing humanity. The most common complication of Ascariasis is mechanical bowel obstruction caused by a large number of worms. Bowel obstruction can also be caused by various toxins released by the worms. A large worm bolus can also cause volvulus or intussusception. We report a case of Intestinal Obstruction due to an Ileal MZBCL in an Ascaris. Lumbricoides infestation setting.

[Guidelines for management of adult sickle cell disease]. / Recommandations pratiques de prise en charge de la drépanocytose de l'adulte.

Sickle cell disease is a systemic disease that can potentially involve all organs. As the prevalence of patients with sickle cell disease increases gradually in France, every physician can be potentially involved in the care of these patients. Complications of sickle cell disease can be acute or chronic. Pain is the main symptom and should be treated quickly and aggressively. Acute chest syndrome is the leading cause of acute death and must be prevented, detected, and treated without delay. Chronic complications are one of the main concerns in adults and should be identified as early as possible in order to prevent sequels. Many organs can be involved, including the bones, kidneys, eyes, lungs... The indications for a specific treatment (blood transfusion or hydroxyurea) should be discussed. Health care should be carefully organized to allow both a regular follow-up near the living place and access to specialized departments. We present in this article the French guidelines for the sickle cell disease management in adulthood.

Primary psoas abscess in a patient affected by acquired immunodeficiency syndrome: a rare case according to the reviewed literature.

Abscesses of the psoas muscle can be divided into primary and secondary. In the primary ones, it is not possible to identify any further infected site. The localization to this muscle is due to its rich vascularization and Staphylococcus aureus is the most frequent aetiological agent of the infection. Treatment requires the use of appropriate antibiotics, as well as surgical or percutaneous drainage of the abscess. The percutaneous drainage is much less invasive and a low risk in the patients with acquired immunodeficiency syndrome, and is effective for draining even multiloculated abscess. The authors present a rare case of primary psoas abscess in patient affected by acquired human immunodeficiency syndrome, showing a mass in the inferior lumbar region through the lumbar triangle of Petit and fever. Treatment consisting in percutaneous drainage combined with systemic antibiotic administration was successful.

Adjunctive plasma exchanges to treat neuropsychiatric lupus: a retrospective study on 10 patients.

Neuropsychiatric manifestations of systemic lupus erythematosus (NPSLE) are among the main causes of morbidity and mortality attributed to lupus activity. Conventional NPSLE treatment combines CS and immunosuppressants, but some symptoms do not respond. We retrospectively evaluated the adjunction of plasma exchanges (PE) to treat 13 NPSLE flares occurring in 10 patients (mean age, 30 years) between 1989 and 2002. NP manifestations were the first SLE symptoms for seven patients, with a mean of 3.2 NP manifestations/flare. All patients received CS and cyclophosphamide pulses. A mean of 15 PE/flare were performed. All patients improved within a mean of 3 (median: 2.5; range: 1.5-8) weeks thereafter. Complete remissions of 7/13 flares were obtained within a mean of 7 (median: 4; range: 2-22) weeks. Partial remissions were achieved for the remaining six flares, characterized by new NP manifestations during three and insufficient control of the others. Other SLE manifestations regressed for all patients with the mean European consensus lupus activity measurement score declining from pretreatment 6.9 to 1.2. A regimen combining CS, cyclophosphamide and PE is effective against severe NPSLE, with acceptable toxicity.