Evaluation and novel treatment in a case of refractory small bowel-small bowel intussusception.

Small bowel into small bowel intussusception can present with symptoms similar to those observed in patients with more common small bowel into large bowel intussusception. In most cases, intussusceptions isolated to the small bowel are self-limited and less likely to result in bowel ischemia. Nonetheless, any patient with recurrent intussusception should be evaluated to assess location and for the presence of a pathologic lead point. We report a patient with recurrent small bowel into small bowel intussusception who underwent a comprehensive evaluation that revealed lymphoid hyperplasia in the absence of a pathologic lead point. His symptoms resolved after a dose of oral dexamethasone.

[Epilepsy with PCDH19 mutation: polypharmacy as a consequence of the complexity and diversity of pathogenesis mechanisms]. / Epilepsiya pri mutatsii gena PCDH19: polipragmaziya kak sledstvie slozhnosti i mnogoobraziya mekhanizmov patogeneza.

Mutations in the human PCDH19 gene lead to epileptic encephalopathy of early childhood. It is characterized by the early onset of serial seizures, cognitive impairment and behavioral disorders (including autistic personality traits). In most cases, difficulties arise in selecting therapy due to pharmacoresistance. The pathogenesis of the disease is complex. The data available to us at the moment from numerous studies present the pathogenesis of «PCDH19 syndrome¼ as multi-level, affecting both the epigenetic support of cell life, and development of stem cells and progenitor cells in the process of neuroontogenesis, and the influence on the neurotransmitter mechanisms of the brain, and disruption of the formation of neural networks with an inevitable increase in the excitability of the cerebral cortex as a whole, and local changes in the highly labile regulatory structures of the hippocampal region. And it is not surprising that all these changes entail not only (and perhaps not so much) epileptization, but a profound disruption of the regulation of brain activity, accompanied by autism spectrum disorders, more profound disorders in the form of schizophrenia or cyclothymia, and the formation of delayed psychomotor development. A «side branch¼ of these pathogenetic processes can also be considered the participation of PCDH19 dysfunctions in certain variants of oncogenesis. The need for polypharmacy (in most cases) confirms the diversity of mechanisms involved in the pathogenesis of the disease and makes the prospects for the development of effective and rational treatment regimens very vague. Cautious optimism is caused only by attempts at relatively specific treatment with ganaxolone.

EGFR-TKI-induced Factor V deficiency in a patient with advanced non-small cell lung cancer: The first case report.

Osimertinib, a third-generation epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI), is routinely prescribed as first-line therapy for advanced non-small cell lung cancer, regardless of the presence of the T790M resistance mutation. This study reports a rare case of Factor V inhibitor detection during osimertinib therapy in a patient with lung adenocarcinoma. These findings underscore the importance of vigilant monitoring for coagulation abnormalities during EGFR-TKI therapy.

Unusual course of glyphosate-induced acute kidney injury: a case report of tubulointerstitial nephritis treated with steroids.

Glyphosate is a widely used herbicide that is generally considered safe; however, acute kidney injury (AKI) caused by glyphosate ingestion can be severe and require hemodialysis. We present a unique case of a 68-year-old Japanese man who developed AKI after accidental ingestion of glyphosate and required hemodialysis. Based on the clinical presentation and findings, the patient was diagnosed with renal AKI with severe tubulointerstitial damage. However, the precise pathogenesis of the tubulointerstitial damage remained unclear. An elevated beta-2 microglobulin level discovered by the urinalysis during admission raised the suspicion of tubulointerstitial nephritis caused by glyphosate. Gallium scintigraphy revealed accumulation in both kidneys. A renal biopsy revealed acute tubulointerstitial nephritis rather than acute tubular necrosis, which is commonly observed with glyphosate-induced renal injury. After initiating steroid therapy, his kidney function gradually improved and he was weaned from hemodialysis. This report is the first to describe glyphosate-induced acute tubulointerstitial nephritis that was successfully treated with immunosuppressive therapy. Furthermore, this report highlights the importance of steroid therapy for cases of persistent kidney injury after the discontinuation of agents associated with acute tubulointerstitial nephritis.

The role of tissue IgG4 levels in steroid therapy in patients with idiopathic granulomatous mastitis.

Idiopathic granulomatous mastitis (IGM) is a benign, chronic inflammatory lesion of the breast. Immunoglobulin G4 (IgG4) associated disease is rare in the breast. In our study, we aimed to evaluate the efficacy of steroid treatment on IgG4 levels in tissue in patients diagnosed with IGM. Between 2008 and 2017, 55 patients diagnosed with IGM in our clinic were included in the study. Demographic, clinical, microbiologic and histopathologic characteristics, treatment modality and recovery time were evaluated retrospectively. Patients were divided into 3 groups according to tissue IgG4 levels: negative (Group I), infrequently and slightly positive (Group II), and highly positive (Group III). Group I patients had a complete response rate of 77.8%. In the rest of the patients (22.2%), insufficient response was detected from the beginning of the treatment. In Group II, the response rate was 91.3% and the permanent success rate after treatment was 87.0%. Although group III patients had a complete response at the beginning (95.65%), they relapsed in a short period of time (26.1%) after discontinuation of steroid treatment. At least one steroid-related side effect was observed in 47 (85.8%) patients in all groups. There is no consensus on the dose and duration of immunosuppressive treatment in IGM. In this study, responses to steroid treatment according to IgG4 concentration in pathologic breast tissue and recurrences after the end of treatment were determined. We think that high IgG4 concentration in the tissue is associated with recurrence and other immunosuppressive drugs should be added as maintenance after steroid treatment.

Real-World Outcomes of Graft-versus-Host Disease after Allogeneic Hematopoietic Stem Cell Transplantation in Japan: Retrospective Analysis of the Transplant Registry Unified Management Program Registry.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an important therapeutic option for patients with hematologic malignancies. However, the development of graft-versus-host disease (GVHD) after allo-HSCT remains a challenge. Although systemic steroid therapy is the established first-line therapy for acute GVHD (aGVHD) and chronic GVHD (cGVHD), many patients are unresponsive or resistant to corticosteroid therapy, and the response is insufficient. This study aimed to evaluate the clinical characteristics of patients who developed aGVHD and cGVHD after allo-HSCT. This noninterventional, retrospective study used large national registry data from the Transplant Registry Unified Management Program. The study included 29,690 patients with a hematologic disease who underwent their first allo-HSCT between January 2010 and December 2019. The primary study endpoints were the cumulative incidence of aGVHD and cGVHD. The secondary endpoints were overall survival (OS) and nonrelapse mortality (NRM) of patients with aGVHD and cGVHD and OS and NRM of patients who received second-line therapy for aGVHD. Of 29,690 patients who underwent allo-HSCT, the graft source was related bone marrow (RBM) in 2807, related peripheral blood (RPB) in 6167, unrelated bone marrow in 10,556, unrelated peripheral blood (UPB) in 774, and unrelated cord blood in 9339. The cumulative incidence of grade II-IV aGVHD at 100 days was high after the related and unrelated mismatched transplantation. The response rates for first- and second-line therapy for aGVHD were low in the RBM/RPB-mismatched (59.6%/61.6%) and UPB-mismatched subgroups (45.5%), respectively. The 3-year NRM in patients with aGVHD was high in the RPB and UPB mismatched subgroups (37.9% and 31.2%, respectively). Developing a novel treatment for steroid-refractory aGVHD is necessary to improve transplantation outcomes, particularly for patients undergoing HLA-mismatched allo-HSCT.

Steroid therapy in acute exacerbation of fibrotic interstitial lung disease.

BACKGROUND AND OBJECTIVE: Evidence for the benefit of steroid therapy in acute exacerbations (AEs) of idiopathic pulmonary fibrosis (IPF) is limited; however, they remain a cornerstone of management in other fibrotic interstitial lung diseases. This retrospective observational study assesses the effect of steroid treatment on in-hospital mortality in patients with acute exacerbation of fibrotic interstitial lung disease (AE-FILD) including IPF and non-IPF ILDs. METHODS: AE-FILD cases over a 10-year period were filtered using a code-based algorithm followed by individual case evaluation. Binary logistic regression analysis was used to assess the relationship between corticosteroid treatment (defined as ≥0.5 mg/kg/day of prednisolone-equivalent for ≥3 days within the first 72 h of admission) and in-hospital mortality or need for lung transplantation. Secondary outcomes included readmission, overall survival, requirement for domiciliary oxygen and rehabilitation. RESULTS: Across two centres a total of 107 AE-FILD subjects were included, of which 46 patients (43%) received acute steroid treatment. The steroid cohort was of younger age with fewer comorbidities but had higher oxygen requirements. Pre-admission FVC and DLCO, distribution of diagnoses and smoking history were similar. The mean steroid treatment dose was 4.59 mg/kg/day. Steroid use appeared to be associated with increased risk of inpatient mortality or transplantation (OR 4.11; 95% CI 1.00-16.83; p = 0.049). In the steroid group, there appeared to be a reduced risk of all-cause mortality in non-IPF patients (HR 0.21; 95% CI 0.04-0.96; p = 0.04) compared to their IPF counterparts. Median survival was reduced in the steroid group (221 vs. 520.5 days) with increased risk of all-cause mortality (HR 3.25; 95% CI 1.56-6.77; p < 0.01). CONCLUSION: In this two-centre retrospective study of 107 patients, AE-FILD demonstrates a high risk of mortality, at a level similar to that seen for AE-IPF, despite steroid treatment. Clinicians should consider other precipitating factors for exacerbations and use steroids judiciously. Further prospective trials are needed to determine the role of corticosteroids in AE-FILD.

Löfgren Syndrome: A Case Report and Treatment Challenge.

Sarcoidosis is an inflammatory and immune-mediated multisystemic disorder of unknown etiology, characterized by the presence of non-caseating granulomas, impacting various organs. This indolent condition manifests with numerous nonspecific symptoms and lacks a definitive diagnostic test, typically requiring histopathologic confirmation. However, a distinct and more readily diagnosable form of sarcoidosis does exist. The Löfgren syndrome (LöS) is characterized by the triad of erythema nodosum (EN), bilateral hilar lymphadenopathy, and symmetrical inflammatory arthralgias or arthritis. The simultaneous presence of these elements obviates the necessity for a biopsy. Predominantly affecting women in their second and third decades of life, this syndrome generally carries a favorable prognosis with spontaneous resolution or the requirement for a nonsteroidal anti-inflammatory drug (NSAID) alone. Despite its rarity, in particular cases, the treatment can be more challenging. This article presents a case study of LöS in a young woman, whose more aggressive disease course led to the need for steroidal therapy.

Recurrent Reactive Infectious Mucocutaneous Eruption in an Adult Male Secondary to Mycoplasma Infection: A Case Report.

Mycoplasma pneumoniae commonly causes respiratory tract infections but can also involve the skin and mucosal surfaces. Reactive infectious mucocutaneous eruption (RIME) secondary to mycoplasma infection is uncommon in adults but is an important clinical entity. We present the case of a 26-year-old male who experienced recurrent episodes of erythematous and painful oral ulcers without any prodromal or respiratory symptoms. Serological testing confirmed a recent mycoplasma infection. The patient was successfully treated with oral steroids and supportive therapy. This case underscores the challenges of diagnosing RIME, particularly in the absence of typical respiratory symptoms. Moreover, oral steroid therapy with supportive treatment may suffice to manage RIME if the patient lacks an ongoing infection or other underlying pathologies.

Peripapillary pachychoroid syndrome treated with dexamethasone implant: A case report.

PURPOSE: To describe a case of peripapillary pachychoroid syndrome (PPS) in a diabetic patient with cystoid macular edema (CME), treated with intravitreal dexamethasone implant (IDI) injection. This report also illustrates the history of the disease after repeated IDI and dexamethasone topical treatment. METHODS: A case report. RESULTS: A 77-year old male patient with PPS and good diabetic control was treated with dexamethasone implant for CME. After an initial morphofunctional improvement associated with a first IDI, the disease relapsed after the second dexamethasone implant injection. This was associated with a significant increase in both intraretinal fluid and choroidal thickness, with subsequent visual acuity (VA) decrease. At this point, a topical dexamethasone treatment was performed and, despite a morphological improvement, VA worsened compared with baseline, likely because of anatomical damage. CONCLUSION: In this report, the importance of the recognition of PPS is underlined and the possible occurrence of a "rebound" effect due to repeated IDI is described.

Neuromyelitis Optica and Immune Thrombocytopenic Purpura Treated With Rituximab in a Patient With Combined Neurologic and Hematologic Disorder: A Case Report.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory demyelinating disease of the central nervous system affecting the optic nerves and spinal cord. Immune thrombocytopenia (ITP), on the other hand, is an autoimmune disorder characterized by a platelet count of <100 in the absence of any known condition that could be associated with thrombocytopenia. This case report focuses on a 56-year-old female presenting with the unique coexistence of NMOSD and ITP. A 56-year-old woman of Russian descent had a sudden onset of right eye blindness at the age of 24 and was diagnosed with multiple sclerosis. She developed petechial rashes on both lower extremities two weeks before consultation with no associated findings. Cranial MRI revealed multiple nodular and patchy areas of hyperintense signals on T2-weighted/fluid-attenuated inversion recovery without restricted diffusion. A thoracolumbar MRI revealed long segment foci of intramedullary cord non-enhancing abnormal hyperintense signal from T2 to T11. Cerebrospinal fluid aquaporin 4 IgG was negative. A complete blood count revealed platelets of 4 × 109/L, leading to the management of ITP. She was started on methylprednisolone 1 g/day for five days. Her platelet count improved eventually and rashes resolved. Rituximab treatment was initiated at a dose of 1 g on day 1 and day 15. On the 18th day of admission, the Expanded Disability Status Scale and functional score improved to 6.0 from 7.0 upon admission.

Isolated Depo-Medrol Administration under Tenon's Capsule for Post-COVID-19 Uveitis in a Child: A Case Report and Literature Review.

Coronavirus disease 2019 (COVID-19) can manifest with ocular symptoms. These symptoms can be divided into isolated events attributed to COVID-19, and those occurring in multisystem inflammatory syndrome in children (MIS-C), a newly diagnosed disease entity associated with COVID-19 infection. Currently, the literature lacks specific guidelines and treatment regimens for COVID-19 ocular symptoms, especially in children. The authors present the case of a 14-and-a-half-year-old boy with bilateral uveitis of the anterior and posterior segments along with vasculitis and optic neuritis associated with SARS-CoV-2 infection. The authors also perform an up-to-date review of all available publications on the treatment of post-COVID-19 uveitis in children described in the literature between 2020 and 2023. In the case described by the authors, the treatment involved a Depo-Medrol 40 mg/mL injection uder the Tenon capsule, with two subconjunctival injections of epinephrine, topical steroid therapy and non-steroidal anti-inflammatory drugs: dexamethasone 0.1%; diclofenac eye drops. In addition, acetylsalicylic acid (150 mg) and pentoxifylline (100 mg, orally) were administered throughout the course of the disease as well as up to 12 months after its termination, until a complete improvement in visual acuity and the withdrawal of ocular lesions were achieved. It can be assumed that this type of treatment is far more beneficial for pediatric patients, with an effect comparable to systemic steroid administration with a preserved improvement in retinal-vascular circulation, without exposing the child to systemic post-steroid complications.

Impact of Corticosteroid Therapy on ICU Patient Outcomes in Severe COVID-19 Cases: A Retrospective Cohort Study in Saudi Arabia.

BACKGROUND: The COVID-19 pandemic has presented significant challenges in clinical management, and intensive care units (ICUs) worldwide have become epicenters of high-stakes treatment decisions. Among these, corticosteroid therapy has risen as a pivotal, yet controversial, treatment modality. In Saudi Arabia, where unique demographic and health system characteristics intersect, understanding the specific effects of corticosteroids on ICU patient outcomes is not just critical but a pressing necessity in tailoring effective COVID-19 management strategies. OBJECTIVE: This study aims to elucidate the effects of corticosteroid therapy on the outcomes of severe COVID-19 patients in Saudi Arabian ICUs, providing critical insights into treatment efficacy and guiding future clinical practices. MATERIALS AND METHODS: In this cohort study, we meticulously reviewed the medical records of 1085 severe COVID-19 patients admitted to Saudi Arabian ICUs. Our analysis focused on demographic details, ICU outcomes, and the extent and implications of corticosteroid therapy. The study employed comprehensive methods for data collection, evaluation criteria, and statistical analysis, ensuring a thorough understanding of the impact of corticosteroids in this context. RESULTS: The study encompassed 1085 patients, predominantly male (74.5%, N=806), with an average age of 56 and a mean BMI of 30.07. A significant portion (72.3%, N=784) received corticosteroid therapy. These patients generally experienced longer ICU (mean 23 days) and hospital stays (mean 16 days), along with higher rates of microbiological cure (72.3%, N=648) and increased ICU discharge likelihood. Conversely, corticosteroid recipients showed higher mortality rates at ICU discharge. The statistical analysis confirmed the significance of these findings, reinforcing their importance in managing COVID-19 in ICUs. CONCLUSION: The research highlights the intricate dynamics of corticosteroid use in treating severe COVID-19 cases in ICUs. While associated with prolonged ICU stays and increased mortality, corticosteroids also correlate with higher microbiological cure rates and discharge likelihood. These insights call for careful deliberation in applying corticosteroid therapy, with implications for enhancing clinical protocols and guiding future research in severe COVID-19 treatment.

Modified intratympanic steroid therapy for sudden sensorineural hearing loss via tympanic tube and gelfoam as a salvage treatment.

Background: Sudden sensorineural hearing loss (SSNHL) is a prevalent emergency in ear, nose, and throat practice. Previous studies have demonstrated that intratympanic steroid therapy (IST) can serve as a salvage treatment for SSNHL after the failure of systemic steroid therapy (SST). Objective: This study aimed to analyze the efficacy of modified IST involving the insertion of a tympanic tube and gelfoam as a salvage treatment for patients with SSNHL, and to explore its associated factors. Methods: Totally, 74 patients who were aged 22-81 years with SSNHL were enrolled and allocated to either the control group (n = 25) or the treatment group (n = 49) based on their treatment modalities. All patients received SST lasting for at least 7 days. Subsequently, patients in the treatment group, after SST failure, underwent IST twice a week for 2-6 weeks, while the control group did not. Efficacy was assessed by the improvement in pure tone average at the affected frequency at the beginning and end of IST. Results: Hearing improvement in all patients after IST in the treatment group was 9.71 ± 14.84 dB, with significant improvement at affected frequencies (250-8000 Hz) compared with the control group (P < 0.05). The findings indicated the duration from the onset of SSNHL to the beginning of IST as an independent factor for pure tone average improvement after treatment (P = 0.002), whereas age, duration of SST, and time of IST were not (P > 0.05). Conclusion: The modified IST was demonstrated to be a safe and effective method as a salvage treatment for SSNHL. This study explored the efficacy of a modified IST approach, incorporating the utilization of tympanic tubes and gelfoam as key components. The findings underscore the advantages of gelfoam as a strategic drug carrier placed in the round window niche. By minimizing drug loss, extending action time, and increasing perilymph concentration, gelfoam enhances the therapeutic impact of IST, contributing to improved hearing outcomes in patients with SSNHL.

Assessment of the Level of Knowledge and Awareness of the Risk of Chronic Use of Steroids in Causing Cataracts Among the General Population in the Western Region of the Kingdom of Saudi Arabia (KSA): A Cross-Sectional Study.

BACKGROUND/AIM: Cataracts consequence blindness to burden and impose health and economic burdens on communities. Steroid-induced cataracts have scarcely been highlighted in previous literature, creating a demand for reinvestigating this issue among the general population of western Saudi Arabia. METHODS: A cross-sectional study was conducted in 2022 using an online survey distributed among the target participants via social media platforms. The data were gathered and statistically analyzed using IBM Corp. Released 2015. IBM SPSS Statistics for Windows, Version 23.0. Armonk, NY: IBM Corp. software. RESULTS: Overall, 866 respondents (males = 42.5%, females = 57.5%) were enrolled in this survey (mean age = 28.08, SD = 13). The participants' correct responses to questions about steroid-induced cataracts showed inadequate representation (below 50%). Additionally, most of the participants (94.23%) had a poor understanding of steroid-induced cataracts. CONCLUSION: The participants' level of understanding of the risk factors associated with chronic steroid usage and its impact on cataracts was inadequate.

Deciphering the Neuropsychiatric Lupus Enigma: Navigating the Intersection of Acute Inflammatory Demyelinating Polyneuropathy (AIDP), Infection, and Flare.

A 17-year-old male diagnosed with systemic lupus erythematosus (SLE), showing poor compliance with medication, presented to our facility with a 20-day history of fever, polyarthritis, and cough. Additionally, he had experienced a seizure episode, followed by a one-day history of altered mentation. Subsequently, he developed pneumonia, respiratory distress, and shock, necessitating ventilator and inotropic support. Neuropsychiatric lupus (NP-lupus) was suspected, and hence high-dose steroids, hydroxychloroquine, and broad-spectrum antibiotics were initiated. Following successful extubation, he manifested ascending flaccid paralysis. The presence of albumin-cytological dissociation and axonal neuropathy confirmed the diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP). He underwent further management with pulse steroids and plasmapheresis. Upon recovery, he was discharged on a regimen of steroids, cyclophosphamide, and hydroxychloroquine. During follow-up, he maintained ambulatory status with no residual neurological sequelae.

[Endocrine orbitopathy]. / Endokrine Orbitopathie.

Endocrine orbitopathy (EO), also known as Graves' orbitopathy or thyroid-associated orbitopathy, is a self-limiting, immunologically induced co-reaction of the retrobulbar tissue of the eye triggered by an autoimmune disease of the thyroid gland. It is particularly associated with Graves' disease and is its most common extrathyroidal manifestation. In addition to typical anamnestic data, characteristic local findings and laboratory changes in immunothyroidism, orbital imaging plays a central role in the diagnosis and management of this disease. This review article provides comprehensive insight into various imaging modalities used to assess morphologic changes associated with EO. A detailed presentation of imaging findings provides a better understanding of orbital physiology.

Medication utilization for patients with chronic rhinosinusitis with nasal polyposis and asthma in 12 months pre- and post-dupilumab initiation.

KEY POINTS: This study examines the impact of dupilumab on medication use for chronic rhinosinusitis with nasal polyposis (CRSwNP) and asthma patients. Patients on dupilumab had a reduction in oral/inhaled/topical steroids, antibiotics, and leukotriene receptor antagonists (LTRAs). The reduction in medication use had no impact on total polyp or SNOT-22 scores.

Drug resistant epilepsies: A multicentre case series of steroid therapy.

PURPOSE: Our study aimed to evaluate the effectiveness of corticosteroids on seizure control in drug-resistant epilepsies (DREs). Our primary goal was to assess the response to steroids for various underlying etiologies, interictal electroencephalographic (EEG) patterns and electroclinical seizure descriptions. Our second goal was to compare steroid responsiveness to different treatment protocols. METHODS: This is a retrospective multicentre cohort study conducted according to the STROBE guidelines (Strengthening the Reporting of Observational Studies in Epidemiology). The following data were collected for each patient: epilepsy etiology, interictal EEG pattern, seizure types and type of steroid treatment protocol administered. RESULTS: Thirty patients with DRE were included in the study. After 6 months of therapy, 62.7 % of patients experienced reduced seizure frequency by 50 %, and 6.6 % of patients experienced complete seizure cessation. Findings associated with favourable response to steroids included structural/lesional etiology of epilepsy, immune/infectious etiology and focal interictal abnormalities on EEG. Comparing four different steroid treatment protocols, the most effective for seizure control was treatment with methylprednisolone at the dose of 30 mg/kg/day administered for 3 days, leading to greater than 50 % seizure reduction at 6 months in 85.7 % of patients. Treatment with dexamethasone 6 mg/day for 5 days decreased seizure frequency in 71.4 % of patients. Hydrocortisone 10 mg/kg administered for 3 months showed a good response to treatment in 71 %. CONCLUSIONS: In our study, two-thirds of patients with DRE experienced a significant seizure reduction following treatment with steroids. We suggest considering steroids as a potential therapeutic option in children with epilepsy not responding to conventional antiseizure medicines (ASM).