Patients Received Corticosteroid Therapy Research Articles

Ophthalmic corticosteroids-related adverse events: the FDA adverse event reporting system (FAERS) database pharmacovigilance study.

Corticosteroids are extensively used in ophthalmology, particularly for treating various inflammatory conditions. Despite their effectiveness, prolonged or high-dose corticosteroid use is associated with significant adverse drug reactions (ADRs), such as increased intraocular pressure, cataract formation, and secondary infections. However, there is currently no systematic study comparing the side effects of ophthalmic corticosteroids. This study aims to investigate the safety profiles of ophthalmic corticosteroids through pharmacovigilance analysis using the FDA Adverse Event Reporting System (FAERS) database. We conducted a retrospective analysis of ADR reports related to commonly used ophthalmic corticosteroids from the FAERS database, covering the period from Q1 2004 to Q4 2023. Clinical features such as gender, age, administration route, and dosage form were also analyzed. Signal detection methods, including Reporting Odds Ratio (ROR), Proportional Reporting Ratio (PRR), Bayesian Confidence Propagation Neural Network (BCPNN), and the Multi-Item Gamma Poisson Shrinker (MGPS), were used to identify potential safety signals. A total of 9,854 ADRs related to ophthalmic corticosteroids were retrieved, with the most frequently reported drugs being Ozurdex (1,784 cases), Lotemax (3,239 cases), and Durezol (2,789 cases). Women accounted for a higher proportion of ADRs across most corticosteroids. ADR induction time analysis results showed that ADRs tend to occur in the early stages of drug use. The most common ophthalmic ADRs identified included eye inflammation, cataract, visual impairment, uveitis, eye pain, blurred vision, and retinal detachment. Additionally, Maxidex has been linked to endocrine disorders, while Ozurdex, Iluvien, and Triesence exhibited significant signals for product issues, likely related to their intraocular injection procedures. Notably, cataract was the most common PT among these drugs. Our study reveals significant safety concerns related to using ophthalmic corticosteroids, particularly regarding adverse reactions that can impact visual function. These findings highlight the need for careful monitoring and individualized treatment plans to minimize the risk of ADRs in patients receiving corticosteroid therapy. Future studies combining FAERS data with large-scale clinical research are needed to explore these safety concerns further.

Prolonged corticosteroid therapy and lung abnormalities in patients after severe COVID-19 pneumonia.

Some of the hospitalized patients after severe COVID-19 pneumonia experience significant fall in peripheral saturation despite optimal treatment. Because of immune dysregulation in COVID-19 there are indications that prolonged corticosteroids could be considered in treating patients for persistent radiological sequelae and respiratory symptoms. to investigate lung function and lung sequelae on high-resolution CT (HRCT) im COVID-19 patients who were treated with glucocorticoid therapy in two dose regimens with a control group of patients who did not receive additional glucocorticoid therapy. In this prospective cohort research we studied patients who suffered from prolonged respiratory insufficiency after severe COVID-19 pneumonia. Patients received corticosteroid therapy in two dose regimens: for 14 days and for 3 months after discharge from the hospital. Control group of patients did not receive additional corticosteroid therapy. Lung function, post-COVID-19 symptoms, and lung abnormalities on CT scans were analyzed in three months follow-up and compared with the control group of patients. Patients who received prolonged corticosteroid therapy for three months did not have better CT findings of lung abnormalities, lung function, or symptoms recovery in comparison to the patients with 14 days of therapy and control group of patients. Onwards, control group had significantly fewer dyspnea symptoms (Chi-square test, p=0,04) and higher DLCO (Kruskal Wallis test, p=0,03). Supplementary corticosteroid therapy for patients after severe pneumonia and prolonged respiratory insufficiency with lung abnormalities after COVID-19 did not improve lung function or lung lesions on CT.

Frequency and Factors Associated with Disabilities among Leprosy Patients Admitted to the Kindia Disability Prevention and Physical Rehabilitation Centre (Pirp) in Guinea from 2017 to 2021.

This study aims to estimate the prevalence and analyze the factors associated with leprosy-related disabilities at the Kindia Disability Prevention and Physical Rehabilitation Centre (PIRP) in Guinea. It is a cross-sectional study using routine data from the centre from 2017 to 2021. Of 115 patients, 76% had a disability, 49% of which were grade II and 27% grade I. The age range of 15 to 30 years was the most represented (43.5%), with the average age (standard deviation) being 38 (16.5) years. Children under 14 years of age represented 3.5% of the total. Most (89%) patients had newly diagnosed leprosy. The majority (66.1%) had never come in contact with people with leprosy symptoms. Almost all (99.1%) patients had type 1 reactions on admission. Patients with multibacillary leprosy were in the majority (83.5%), and those with symptoms lasting 7-12 months represented 56.5% of the sample. In total, 79.1% of the patients received corticosteroid therapy, and 92.1% were reported cured at discharge. This neglected tropical disease continues to be a challenge in Guinea, even though leprosy care is free.

Paradoxical Reaction to Antituberculosis Complicating Bacilifere Pulmonary Tuberculosis

Tuberculosis is still an endemic disease in many countries. Paradoxical reaction (PR) to antituberculosis drugs is a rare complication seen in patients on antituberculosis treatment. We report the case of a 58-year-old man who had been presenting for two months with a cough, signs of tuberculosis impregnation and uncalculated weight loss. Bacciloscopies were positive. We started the patient on antituberculosis treatment. One month later, the patient developed confusion and abdominal pain. The laboratory work-up and various radiological examinations (cerebral CT scan, cerebral MRI) revealed no abnormalities. Abdominal CT scan showed: a peritoneal effusion of low abundance with nodular infiltration of the fat of the great epiploon. After ruling out all other etiologies for the confusion, we retained the diagnosis of paradoxical reaction to anti-tuberculosis drugs. The patient received corticosteroid therapy with marked neurological improvement.

Case Report: Atypical post-COVID Cogan's syndrome

Background Cogan’s syndrome is a rare autoimmune disorder characterized by ocular inflammation, vestibulocochlear dysfunction, and systemic vasculitis. Case Presentation We report a 28-year-old female who experienced decreased visual acuity and ocular redness one month after a COVID-19 infection, with ophthalmological signs linked to keratitis, uveitis and retinal vascularitis. Two weeks later, she developed vertigo, tinnitus, and sudden hearing loss, leading to a diagnosis of Cogan’s disease. The patient received corticosteroid therapy, resulting in regression of ophthalmological signs, but progressed to complete deafness. One month later, she presented with lymphocytic meningitis and high intracranial pressure, which improved under treatment. The patient later received cochlear implants. Objective This case report aims to highlight an atypical presentation of Cogan’s syndrome with neurological involvement following a COVID-19 infection. This case contributes to the limited literature on such presentations. Conclusion Our case is one of only two reported instances of Cogan’s syndrome presenting with neurological signs post-COVID-19 infection, underscoring the rarity and complexity of this condition.

Treatment Outcome of Flow Diverter Device for Medium-Sized Cerebral Aneurysms: A Single-Center Report.

Flow diverters (FDs), first introduced in Japan in 2015, were initially limited to wide-necked large cerebral aneurysms, which pose a high treatment risk. However, based on the results of the PREMIER study, the indications have expanded since 2020, and the number of treatment cases is increasing in Japan. At our hospital, FD placement with adjunctive coil embolization has been actively performed for medium-sized cerebral aneurysms, as indicated in the PREMIER study; herein, we report the outcomes of this treatment. Of the 25 patients with 28 aneurysms who underwent FD placement at our institution between April 2022 and June 2023, 15 with 17 wide-necked unruptured cerebral aneurysms with a maximum diameter of <12 mm in the internal carotid artery (ICA) or vertebral artery (VA) were included. Postoperative complications were investigated in each case, and the aneurysm occlusion status was assessed using ultrashort echo time (UTE)-MRA at 3 months postoperatively and angiography at 6 months postoperatively. Fifteen patients who underwent coiling or stent-assisted coiling (SAC) for the same criteria during the same period were compared. Baseline characteristics and treatment results were compared between FD and coiling/SAC cases. Four males and 11 females with a mean age of 61.7 ± 12.8 years were included, and the median follow-up period was 9 months (6-18 months). There were 14 aneurysms of the ICA and 3 of the VA, and the mean maximum aneurysm diameter was 7.9 ± 1.7 mm. All patients were treated using the Pipeline Flex with Shield Technology (Medtronic, Minneapolis, MN, USA), and 14 aneurysms (82.4%) were treated with adjunctive coil embolization. There were no symptomatic strokes in the perioperative period; only one patient receiving corticosteroid therapy for thyroid eye disease had asymptomatic ICA occlusion at 3 months. Fifteen aneurysms (88.2%) were not visible on UTE-MRA at 3 months postoperatively, and angiography at 6 months showed complete occlusion in 16 (94.1%) aneurysms. The coiling/SAC group had a smaller neck size and higher volume embolization ratio than the FD group; however, complete occlusion was higher in the FD group. FD placement with adjunctive coil embolization for medium-sized cerebral aneurysms is expected to result in good occlusion rates in the early postoperative period.

Mucormycosis in COVID-19 Pandemic

Background Coronavirus disease-2019 (COVID-19) has been associated with a wide range of opportunistic fungal infections. Many countries have seen an unprecedented rise in cases of COVID-19-associated mucormycosis (CAM). The purpose of this study is to identify the correlation between the COVID-19 epidemic and mucormycosis. Our study also provides details on factors causing CAM. Methods A retrospective study from November 2020 to February 2022 includes four cases of COVID-19-associated rhino-orbital mucormycosis treated at Habib Thameur Hospital’s ENT Department. Results The mean age of patients was 67.5-years, 75% were males, and the most frequent underlying conditions were diabetes mellitus (100%). The median time interval between the diagnosis of COVID-19 and the first evidence of mucormycosis infection was 15-days; two patients required hospitalization in a COVID unit, and two patients received oxygen therapy at home. All patients received corticosteroid therapy at a dose of 1 mg/kg/d for an average of 10-days. Treatment using antifungals and surgery was described in all the patients. Amphotericin B deoxycholate was used in 75% and lipid formulation in 25%. Two patients presented renal insufficiency due to amphotericin B; the doses were adapted with good evolution. In that report, the case fatality rate was 75%. Conclusion Mucormycosis is a fatal fungal infection that usually affects patients with altered immunity. Several cases of mucormycosis in people with COVID-19 have been increasingly reported worldwide. Diabetes mellitus (DM), steroid overdose, immunosuppression, and high iron levels, combined with other factors such as prolonged hospitalization, unhygienic conditions, and the use of ventilators, combine to create an ideal environment for contracting mucormycosis. Lastly, there is a need to create awareness about fungal diseases among clinicians in order to help identify early symptoms and restrict the spread of lethal fungal diseases. A collaborative team of otolaryngologists, ophthalmologists, anesthesiologists, and resuscitators will be required in hospitals to accelerate optimal management.

Lucio’s Phenomenon in Lepromatous Leprosy Patient: A Rare Case Report

BACKGROUND: Lucio’s phenomenon is a rare leprosy reaction, characterized by severe necrotic cutaneous lesions that generally occur in patients with untreated or inadequately treated lepromatous leprosy (LL). OBJECTIVE: to describe and comprehend the diagnosis and management of Lucio’s phenomenon in leprosy patients CASE: Male, 34 years old with extensive wounds on both arms and legs. About 3 years earlier, the patient felt burning heat in both hands and feet followed by loss of eyebrows and eyelashes, and a change in the shape of the nose. The patient had not received previous therapy. Physical examination showed that the patient appeared anaemic, leonine facies, megalobuli, madarosis, saddle nose, thickening of the ulnar and posterior tibial nerves, deformities of the fingers and toes, and amputations of several fingers. The dermatological status showed multiple ulcers of irregular shape and varying sizes with pus and necrotic tissue. Reitz serum test revealed a bacterial index of +3 with a morphological index of 90%, and routine blood showed hypochromic microcytic anaemia and leucocytosis. The patient received corticosteroid therapy, anti-leprosy combination therapy, antibiotics, iron tablets and wound care. DISCUSSION: Lucio’s phenomenon consists of skin lesions that range from painful red-black patches to flaky blisters and ulcerations. These lesions usually occur on the lower extremities, and may extend proximally and distally. The lesions improved after administration of corticosteroids and anti-leprosy. Delayed diagnosis leads to significant disability and community transmission of the disease. The later the diagnosis, the more serious the disease becomes and can lead to death due to sepsis. Early diagnosis and appropriate treatment are important. CONCLUSION: Early detection is crucial in order to deliver therapy earlier and prevent disease worsening.

CORTICOSTEROIDS USAGE IN THE INTENSIVE CARE UNIT: SPECIFIC RECOMMENDATIONS BASED ON RECENT EVIDENCE

Corticosteroids are a class of drugs that mimic the effects of cortisol, a hormone naturally produced by the adrenal glands. They exert a wide range of effects on the body's immune, metabolic, and inflammatory responses. In the context of critically ill patients in the intensive care unit (ICU), corticosteroids are frequently utilised due to their potent anti-inflammatory and immunosuppressive properties. In the ICU setting, corticosteroids are commonly employed to manage various conditions such as severe sepsis, acute respiratory distress syndrome (ARDS), exacerbations of chronic obstructive pulmonary disease (COPD), and adrenal insufficiency. Their mechanism of action involves suppression of pro-inflammatory cytokines, inhibition of leukocyte migration, and stabilisation of cell membranes, among other effects. The choice of corticosteroid, dosing regimen, and duration of therapy in the ICU depends on the specific clinical condition being treated and individual patient factors. For instance, corticosteroids such as hydrocortisone are often administered to attenuate the systemic inflammatory response and improve hemodynamic stability in the management of septic shock. Dosing may vary but commonly involves an initial bolus followed by continuous infusion or intermittent dosing. In ARDS, corticosteroids may reduce lung inflammation and prevent further tissue damage. Methylprednisolone is a commonly utilised corticosteroid in this context, with dosing typically initiated at a high dose and then tapered gradually based on clinical response. For patients with exacerbations of COPD, corticosteroids help to reduce airway inflammation and improve lung function. Oral or intravenous corticosteroids such as prednisone or methylprednisolone are often prescribed for a short duration during exacerbations. In cases of adrenal insufficiency, corticosteroid replacement therapy is essential to restore physiological cortisol levels and prevent adrenal crisis. Hydrocortisone is the corticosteroid of choice in this scenario, with dosing adjusted based on the degree of adrenal dysfunction and stress level. Despite their efficacy, corticosteroids are associated with a range of potential adverse effects, including immunosuppression, hyperglycemia, fluid retention, electrolyte abnormalities, and increased risk of infection. Therefore, carefully monitoring patients receiving corticosteroid therapy in the ICU is paramount, with adjustments to minimise risks while optimising therapeutic benefits. In summary, corticosteroids play a crucial role in managing critically ill patients in the ICU, offering potent anti-inflammatory and immunomodulatory effects. However, their use requires careful consideration of the underlying condition, patient characteristics, and potential adverse effects, with dosing and duration tailored to individual needs.

Long-Term Hearing Loss after Acute Acoustic Trauma inthe French Military: A Retrospective Study.

Acute acoustic trauma (AAT) is characterized by cochlea-vestibular signs following intense noise exposure, often caused by impulse noise. French military faces a high risk of AAT because of the use of weapons with peak sound levels exceeding 150 dB. Hearing loss (HL) resulting from AAT can have a significant impact on quality of life and operational capacity. The aim of this study was to assess the prevalence of long-term hearing impairment after AAT. The study involved a retrospective review of computer-based patient records from four military medical centers in Northeast France between January 2016 and December 2021. The inclusion criteria required the presence of cochlea-vestibular signs following impulse acoustic exposure and the absence of other causes. Sociodemographic and clinical data were collected, including audiometric data before and after exposure. The primary end point was the presence of a threshold elevation greater than 10 dB between reference and late audiograms. A total of 419 patients were included in the analysis, with a majority of males (n = 419; 84.7%) and a mean age of 23.6 yrs. The most common causative agent was the 5.56-mm assault rifle (n = 327; 78.0%). Tinnitus was the most frequent symptom (n = 366; 87.4%), followed by hypoacusis (n = 147; 35.1%) and earache (n = 89; 21.2%). The initial audiograms showed no HL in 31.0% of cases, while the mean deficit across all frequencies was 15.4 dB. All patients received corticosteroid therapy, with a mean duration of 6.0 d. Late audiograms conducted at an average interval of 448.0 d after AAT revealed a prevalence of long-term HL exceeding 20%. Higher doses of corticosteroid therapy (>1 mg/kg) were associated with a reduced frequency of long-term HL. This study highlights the prevalence of long-term hearing impairment after AAT in the French military. The findings emphasize the importance of preventive measures, including proper use of hearing protection devices, and the need for timely diagnosis and treatment. Further research is warranted to explore gender susceptibility to AAT and evaluate the impact of different weapons on AAT characteristics. The study also underscores the potential benefits of higher doses of corticosteroid therapy in reducing the risk of long-term hearing impairment. Overall, the findings contribute to a better understanding of AAT and can inform strategies for its prevention and management in military settings.

Clinical features, diagnosis, and management of COVID-19 vaccine-associated Vogt-Koyanagi-Harada disease

ABSTRACT Vogt-Koyanagi-Harada (VKH) disease is a rare and serious ocular adverse reaction following COVID-19 vaccination. This study aimed to evaluate the clinical features, diagnosis and management of COVID-19 vaccine-associated VKH disease. Case reports of VKH disease after COVID-19 vaccination were collected up to February 11, 2023 for retrospective analysis. Twenty-one patients (9 males and 12 females) were included, with a median age of 45 years (range 19–78), from three main regions, Asia (12/21), the Mediterranean region (4/21), and South America (5/21). Fourteen patients developed symptoms after the first dose of the vaccine, and 8 after the second dose. Vaccines included mRNA vaccine (10 cases), virus vector vaccine (6 cases), and inactivated vaccine (5 cases). The average time interval from vaccination to onset of symptoms was 7.5 days (range 12 hours to 4 weeks). All 21 patients experienced visual impairment after vaccination, with 20 cases involving both eyes. Sixteen patients showed symptoms of meningitis. Serous retinal detachment was observed in 16 patients, choroidal thickening was observed in 14, aqueous cell in 9, and subretinal fluid in 6. CSF pleocytosis was detected in 7 patients and skin symptoms were found in 3 patients. All patients received corticosteroid therapy, and 8 also received immunosuppressive agents. All patients recovered well, with a mean recovery time of 2 months. Early diagnosis and early treatment are crucial to the prognosis of patients with VKH after vaccination with COVID-19 vaccine. The risk of vaccination against COVID-19 in patients with a history of VKH disease should be evaluated clinically.

Yeast Bloodstream Infections in the COVID-19 Patient: A Multicenter Italian Study (FiCoV Study)

Fungemia is a co-infection contributing to the worsening of the critically ill COVID-19 patient. The multicenter Italian observational study FiCoV aims to estimate the frequency of yeast bloodstream infections (BSIs), to describe the factors associated with yeast BSIs in COVID-19 patients hospitalized in 10 hospitals, and to analyze the antifungal susceptibility profiles of the yeasts isolated from blood cultures. The study included all hospitalized adult COVID-19 patients with a yeast BSI; anonymous data was collected from each patient and data about antifungal susceptibility was collected. Yeast BSI occurred in 1.06% of patients, from 0.14% to 3.39% among the 10 participating centers. Patients were mainly admitted to intensive or sub-intensive care units (68.6%), over 60 years of age (73%), with a mean and median time from the hospitalization to fungemia of 29 and 22 days, respectively. Regarding risk factors for fungemia, most patients received corticosteroid therapy during hospitalization (61.8%) and had a comorbidity (25.3% diabetes, 11.5% chronic respiratory disorder, 9.5% cancer, 6% haematological malignancies, 1.4% organ transplantation). Antifungal therapy was administered to 75.6% of patients, mostly echinocandins (64.5%). The fatality rate observed in COVID-19 patients with yeast BSI was significantly higher than that of COVID-19 patients without yeast BSI (45.5% versus 30.5%). Candida parapsilosis (49.8%) and C. albicans (35.2%) were the most fungal species isolated; 72% of C. parapsilosis strains were fluconazole-resistant (range 0-93.2% among the centers). The FiCoV study highlights a high prevalence of Candida BSIs in critically ill COVID-19 patients, especially hospitalized in an intensive care unit, a high fatality rate associated with the fungal co-infection, and the worrying spread of azole-resistant C. parapsilosis.

The Association of Corticosteroid Therapy With Mortality and Length of Stay Among Children With Septic Shock: A Retrospective Cohort Study.

Introduction Septic shock remains a leading cause of mortality in pediatric patients. Corticosteroids have been used in the management of sepsis and septic shock, but there is conflicting evidence on the potential benefit of corticosteroid therapy. This study assessed the risk of mortality and length of stay in the pediatric intensive care unit (PICU) among pediatric patients admitted with a septic shock diagnosis. Method A retrospective cohort study was conducted among pediatric patients (up to 14 years old) admitted with a septic shock diagnosis to the PICU of King Abdullah Specialist Children's Hospital in Riyadhfrom January 2016 to December 2021. The clinical outcomes of patients receiving corticosteroid therapy were compared to those of control patients who were not given corticosteroids. Electronic medical records provided clinical data, severity scores, and the management given for each patient. The patients were followed up from the date of sepsis diagnosis to hospital discharge. Proportional hazard ratios (HRs) were calculated to compare the risk of mortality, length of PICU stay, and length of hospital stay. Results A total of 182 pediatric patients were included in the study, and 86 (47%) received corticosteroid therapy. The median age of the study population was 15 months (interquartile range [IQR]: 2-72 months). Compared to the controls, the patients who received corticosteroids had a higher total Sequential Organ Failure Assessment (SOFA) score (mean±SD: 5.5±3vs. 7.1±3.3, respectively; p<0.01) and required more ventilation support (72% vs. 28%, respectively) and the use of inotropes and vasopressors (74% vs. 34% and 32% vs. 6%, respectively). In-hospital mortality did not significantly differ between the groups (adjusted HR: 2.66; 95% confidence interval [CI]: 0.66-10.28). Those patients who received corticosteroids had 42% less risk of staying in the PICU for over six days than those not receiving steroids (HR: 0.35; 95% CI: 0.13-0.98) Conclusion After adjusting for baseline characteristics, severity scores, and medical intervention, no association was found between receiving corticosteroids and mortality (p=0.492). Furthermore, patients who received corticosteroids had less risk of a prolonged stay in the PICU than those who did not.

Clinical Characteristics of Patients with Probable Coronavirus Disease 2019-associated Pulmonary Aspergillosis at a Tertiary Care Hospital in the Republic of Korea: A Case Series

Background: Coronavirus disease 2019 (COVID-19)-associated pulmonary aspergillosis (CAPA) is a life-threatening invasive fungal infection in critically ill patients with COVID-19. However, only a few studies have reported CAPA in the Republic of Korea. Objective: To describe clinical characteristics of CAPA in patients at a tertiary care hospital in the Republic of Korea. Methods: This retrospective, observational consecutive case series study was conducted by reviewing the electronic medical records of patients who developed CAPA at Seoul National University Hospital from January 1, 2020, to August 31, 2021. CAPA was defined by European Confederation of Medical Mycology/International Society for Human and Animal Mycology (ECMM/ISHAM) consensus criteria. Patient demographics, comorbidities, corticosteroid use, clinical presentation, treatment, and outcomes were investigated. Results: Eleven patients were diagnosed with probable CAPA according to the ECMM/ISHAM criteria. One patient had classical host factor for invasive pulmonary aspergillosis before admission. All patients received corticosteroid therapy before CAPA diagnosis. The mean total corticosteroid administered before CAPA diagnosis was 220 mg of dexamethasone equivalent dose (range, 80~572 mg), and the mean duration of steroid therapy was 15 days (range, 4~34 days). The median time from intensive care unit admission to CAPA diagnosis was 12 days (range, 5~36 days). All individuals showed aggravation on chest X-rays. Ten patients were diagnosed with positive serum galactomannan (GM), and one was diagnosed with positive GM in a bronchoalveolar lavage specimen. Of the 11 patients, 8 received voriconazole-based antifungal therapy for a median of 30.5 days. Only two patients survived after antifungal treatment. Conclusion: These cases illustrate CAPA complicated in critically ill COVID-19 patients. The challenges in diagnosis and poor outcomes of CAPA emphasize the clinical suspicion and needs for further investigation.

Infantile Periarteritis Nodosa (About 5 Cases and Review of the Literature)

PAN is an inflammatory and necrotizing vasculitis affecting small and medium caliber arteries of the body, rarely described in children. Its pathophysiology is complex and remains poorly elucidated, involving the deposition of circulating immune complexes in the vascular wall, neutrophil anti-cytoplasmic antibodies, the role of cytotoxic lymphocytes, cytokines, environmental factors (infections), as well as genetic susceptibility (mutations in ADA 2 and the MEFV gene). There are two main forms: a classic systemic form with visceral involvement (60% of cases) and a cutaneous form with lesions limited to the skin and muscles (30%), which usually evolve less severely. Positive diagnosis is based on the Eular/Printo/Pres criteria. The treatment depends on the form of PAN, and relies essentially on NSAIDs, colchicine, corticotherapy and immunosuppressants: azathioprine and cyclophosphamide. Our work is a retrospective study of 5 cases of PAN, collected at the IV pediatric department and the pediatric rheumatology consultation of the children's hospital of Rabat between 2009 and 2014: The diagnosis was retained on the criteria of Eular/Printo/Pres. The clinical picture was dominated by skin and joint involvement. One of our patients presented an atypical picture of PAN associated with infective endocarditis and large vessel involvement. All of our patients received corticosteroid therapy, and one of our patients benefited́ in addition to colchicine.

Survival of critically ill patients with COVID-19 pneumonia-a single-center experience.

The aim of the study was to determine the survival probability of critically ill patients with COVID-19 infection who needed mechanical ventilation and to determine the efficacy of Tocilizumab use. The study was designed as a retrospective analysis of consecutive patients older than 18 years, treated in an intensive care unit. The criteria for admission to the intensive care unit was severe respiratory failure requiring mechanical ventilation. All patients received corticosteroid therapy (methylprednisolone 1-2 mg/kg). Tocilizumab was used at a dose of 8 mg/kg in patients with a severe form of the disease (onset, or developed ARDS), followed by cytokine storm (IL-6 ≥ 40 ng/L and CRP ≥ 50 mg/L). 88 patients were included in the study. Intrahospital mortality was 48.86%. No statistically significant difference was observed between patients with and without tocilizumab therapy. In the group of patients in whom this therapy was applied, the values of intrahospital survival were 45.7%, while in the group without this therapy the probability of intrahospital survival was only 0.93%. The probability of survival in the group with noninvasive mechanical ventilation (NIV) was 94.7%, while in the group with invasive mechanical ventilation (IMV) 0.78%. The duration of symptoms before hospitalization (RR-1.088 CI 1.025-1.155, p < 0.05), as well as the duration of IMV (RR-0.906 CI 0.841-0.976, p < 0.05), were shown to be an independent predictor of poor outcome. The mortality of patients with the most severe form of respiratory failure caused by COVID-19 infection remains high. Independent predictors of poor outcomes were needed for invasive mechanical ventilation and the duration of symptoms before hospitalization or late initiation of appropriate therapy.

Patient demographics and characteristics from an ambispective, observational study of patients with duchenne muscular dystrophy in Saudi Arabia.

Duchenne muscular dystrophy (DMD) is a rare neuromuscular disorder that is characterized by progressive muscle weakness, resulting in disability and premature death. Onset of symptoms typically occurs at 2–3 years of age, and disease progression is managed through treatment with corticosteroids. The aim of this interim analysis is to increase disease awareness and improve patient management in Saudi Arabia (SA) through the use of data from an ongoing ambispective, observational, multicenter study evaluating characteristics of patients aged 1–14 years with genetically confirmed DMD in SA. This interim analysis examined the secondary outcomes from the study–the demographics and clinical characteristics of patients included retrospectively [data recorded (enrollment visit) between January 2014 and September 2020] and prospectively between September 2020 and April 2021. The primary outcome–the list of DMD gene mutations for the study population–will be reported at a later date. There were 177 eligible patients. Mean, standard deviation (SD) age at enrollment was 7.5 (3.0) years. Median (min, max) age at diagnosis was 7.0 (1.3, 13.8) years. At enrollment, 28.9% of patients were full-time wheelchair users, 50.0% of ambulatory patients could run, and 63.9% could climb stairs. The mean (SD) ages of patients at enrollment who were unable to run and climb stairs were 8.0 (2.7) and 7.6 (3.0) years, respectively. Speech delay (19.4%) and learning difficulties (14.9%) were the most commonly reported intellectual impairments. Physical therapy (84.2%) was the most common choice for initial management of DMD. Only 40.7% of patients received corticosteroid therapy as part of their initial management plan, rising to 59.1% at enrollment. Devices were given to 28.8% of patients for initial management, most commonly ankle-foot orthoses (26.0%) and wheelchairs (6.2%). This analysis reports data from the largest study to date to capture demographics and clinical characteristics of DMD patients in SA. The interim results show a relatively late DMD diagnosis age compared with that in other countries, and a need for improved adherence to international DMD standard of care guidelines. Therefore, there is an urgent requirement for improved DMD education and awareness among healthcare professionals and the public in SA.

Effect of corticosteroid therapy on mortality in COVID-19 patients-A systematic review and meta-analysis.

The effect of corticosteroid therapy is still controversial on prevention of mortality in coronavirus disease‐2019 (COVID‐19). The objective of this study is to investigate the effect of corticosteroids on mortality. This systematic review was performed as per preferred reporting items for systematic reviews and meta‐analyses guidelines. A systematic search was performed at different databases namely Medline/PubMed, Cochrane and Google scholar on 10 February 2022. A pooled estimate for effect of corticosteroid therapy on mortality was calculated as outcome of study. Risk bias analysis and Newcastle Ottawa Scale were used to assess the quality of randomized control trial (RCT) and cohort studies, respectively. Cochran's Q test and the I2 statistic were conducted for heterogeneity and accordingly study model was applied. A total 43 studies were included, having sample size of 96,852 patients. Amongst them, 19,426 and 77,426 patients received corticosteroid therapy (intervention group) or standard treatment without corticosteroid (control group), respectively. Mortality observed in the intervention and control group was 14.2% (2749) and 7.1% (5459), respectively. The pooled estimate 2.173 (95% CI: 2.0690–2.2820) showed significantly increased mortality in intervention as compared to control. The pooled estimate of methyprednisolone 1.206 (95% CI: 1.0770–1.3500) showed significantly increased mortality while the pooled estimate of dexamethasone 1.040 (95% CI: 0.9459–1.1440) showed insignificantly increased mortality as compared to control. In conclusion, corticosteroid therapy produced a negative prognosis as depicted by increased mortality among COVID‐19 patients. The possible reasons might be delay in virus clearance and secondary infections due to corticosteroids initiated at high dose in the early stage of infection.

Two entities in pulmonary nodules of a diabetic patient receiving corticosteroid therapy for bullous pemphigoid: an autopsy case report

BackgroundInvasive pulmonary aspergillosis (IPA) is a serious complication occurring in immunocompromised patients, who often show multiple nodular lesions with or without cavitation. Due to high mortality and poor prognosis, the earlier detection and initiation of treatment are needed, while the definitive diagnosis is often difficult to make in clinical settings. Septic pulmonary embolism (SPE) is a complication that occurs in patients with bloodstream infections (e.g., infectious endocarditis). Patients with SPE also present with multiple nodules, nodules with or without cavitation, which are quite similar to the findings of IPA. We herein report an autopsy case that showed multiple nodules due to IPA and infectious endocarditis-related SPE.CaseA 69-year-old man receiving maintenance hemodialysis due to diabetic nephropathy was admitted with worsening skin rash due to bullous pemphigoid and toxic epidermal necrolysis. He was treated with intravenous methylprednisolone followed by an increased dose of oral prednisolone. On the 6th week of admission, he was diagnosed with infectious endocarditis after the isolation of Corynebacterium in blood samples, with a nodule lesion with cavitation in the right lung. Intravenous vancomycin was initiated. After antibacterial treatment, the nodules in the right lung gradually diminished, whereas a nodule with cavitation in the left lung emerged. The nodule in the left lung showed rapid growth along with elevation of serum β-d-glucan and galactomannan antigen. Despite starting treatment with antifungal agents, he died from respiratory failure. An autopsy revealed Groccott staining-positive aspergillus in the left lung, but not in the right lung. We found fibrosis with mitral valve vegetation, indicating a recovery from infectious endocarditis.ConclusionAlthough similar features of nodules with cavitation on CT imaging were shared with SPE and IPA, this case demonstrated that these heterogeneous diseases can occur within the lungs and the distinctly different transitions of CT imaging are helpful for suspecting the presence of multiple pathogeneses.

A case of minimal change disease associated to Rubinstein Taybi syndrome

Introduction: Focal segmental glomerulosclerosis (FSGS) is the main glomerular nephropathy secondary to malformative uropathies. The latter can be included in the poly malformative syndromes in the pediatric population. However, other glomerular impairments may rarely be associated with some polymalformative syndromes as reported in this rare case of a minimal change disease (MCD) associated with Rubinstein Taybi syndrome (RSTS). Materials and methods: We report a case of pure nephrotic syndrome in a young male patient with RSTS. Case presentation: It is about a 27 year old young patient diagnosed since birth with a Rubinstein Taybi. In 1994, he was hospitalized in pediatrics for acute pyelonephritis. Retrograde urethrocystoscopy (RUC) showed bilateral vesicoureteral reflux (VUR) grade 3 with functional renal asymmetry on scintigraphy. The patient was admitted in nephrology department in 2019 for a pure and intense nephrotic syndrome with abrupt onset. The initial diagnosis retained was glomerular nephropathy particularly a FSGS secondary to his malformative uropathy. Subsequently, in view of the persistence of an intense nephrotic syndrome, the absence of VUR at the RUC, as well as the functional renal symmetry at the scintigraphy, it was decided to perform a renal biopsy. The biopsy showed an optically normal kidney with the presence of some meningial C3 deposits. MCD was then retained. The patient received corticosteroid therapy with a favorable clinical and biological response. Conclusion: Even if reflux nephropathy remains the most frequent cause of glomerulopathy secondary to malformative uropathy, we should not hesitate to perform a renal biopsy in front of an intense nephrotic syndrome with no functional renal impact of the VUR.