Manifestaciones clínicas post supresión de sedoanalgesia en pacientes adultos de una terapia intensiva / Clinical manifestations after suppression of sedoanalgesia in adult patients in intensive care

Introducción: en la unidad de cuidados intensivos (UCI), las personas asistidas con patologías relevantes se encuentran bajo sedación, una vez que estas se encuentran bajo los principios de supresión de la sedación, es importante identificar cuáles son las manifestaciones que presentan, propias de las sedaciones. Objetivo: describir las manifestaciones clínicas del síndrome de supresión de la sedoanalgesia presentes en pacientes asistidos en un Hospital Público de la Ciudad de Corrientes de enero a diciembre del 2022. Metodología: estudio cuantitativo, descriptivo, transversal y observacional. La muestra incluyó pacientes adultos de UCI. El cálculo del tamaño muestral se realizó a través del método probabilístico aleatorio simple resultando de éste una muestra de 100 historias clínicas. Para la recolección de datos se utilizó la observación y como instrumento un formulario semiestructurado, de carácter anónimo. Cada formulario contenía datos específicos donde se categorizan las variables en estudio como ser edad, sexo, comorbilidades, tiempo de sedoanalgesia, tipo de sedación, sedoanalgesia utilizada, agitación, confusión, alucinación, diaforesis, taquicardia. Resultados: en cuanto a la edad se obtuvo un promedio de 49 años, el sexo predominante fue el masculino con 52%, en cuanto a las comorbilidades más frecuentes, el 20% presentó Insuficiencia Respiratoria Aguda y el 16% Insuficiencia renal. El motivo de ingreso a UCI en mayor medida con el 33% fue por dificultad respiratoria y Post Quirúrgicos complicados 32%. Los fármacos de mayor elección fueron midazolam 94%, seguido del fentanilo 80%. En cuanto al tiempo de sedación de los pacientes, se encontró una media de 1265 horas. Las manifestaciones clínicas que se observaron en la muestra en mayor medida corresponden a taquicardia 70%, agitación 52%, un 37% confusión e hipertensión y un 24% alucinación. Conclusión: las manifestaciones que se presentaron con mayor frecuencia fueron taquicardia, agitación, confusión, hipertensión y con menor frecuencia alucinación[AU]

Introduction: in the intensive care unit (ICU), people treated with relevant pathologies are under sedation. Once they are under the principles of sedation suppression, it is important to identify the manifestations they present, typical of sedations. Objective: To describe the clinical manifestations of sedation suppression syndrome present in patients treated at a Public Hospital in the City of Corrientes from January to December 2022. Methodology: quantitative, descriptive, cross-sectional and observational study. The sample included adult ICU patients. The calculation of the sample size was carried out through the simple random probabilistic method, resulting in a sample of 100 medical records. Manifestaciones clínicas post supresión de sedoanalgesia en pacientes adultos de una terapia intensiva. Observation was used to collect data and a semi-structured, anonymous form was used as an instrument. Each form contained specific data where the variables under study were categorized, such as age, sex, comorbidities, sedation time, type of sedation, sedation used, agitation, confusion, hallucination, diaphoresis, tachycardia. Results: regarding age, an average of 49 years was obtained, the predominant sex was male with 52%, regarding the most frequent comorbidities, 20% presented Acute Respiratory Failure and 16% Renal failure. The reason for admission to the ICU to a greater extent with 33% was due to respiratory difficulty and complicated Post-Surgeries 32%. The drugs of greatest choice were midazolam 94%, followed by fentanyl 80%. Regarding the sedation time of the patients, an average of 1265 hours was found. The clinical manifestations that were observed in the sample to a greater extent correspond to tachycardia 70%, agitation 52%, confusion and hypertension 37% and hallucination 24%. Conclusion: the manifestations that occurred most frequently were tachycardia, agitation, confusion, hypertension and, less frequently, hallucination[AU]

Introdução: na unidade de terapia intensiva (UTI), as pessoas tratadas com patologias relevantes estão sob sedação. Uma vez sob os princípios da supressão da sedação, é importante identificar as manifestações que apresentam, típicas das sedações. Objetivo: Descrever as manifestações clínicas da síndrome de supressão da sedação presentes em pacientes atendidos em um Hospital Público da Cidade de Corrientes no período de janeiro a dezembro de 2022. Metodologia: estudo quantitativo, descritivo, transversal e observacional. A amostra incluiu pacientes adultos internados em UTI. O cálculo do tamanho amostral foi realizado pelo método probabilístico aleatório simples, resultando em uma amostra de 100 prontuários. A observação foi utilizada para a coleta de dados e um formulário semiestruturado e anônimo foi utilizado como instrumento. Cada formulário continha dados específicos onde foram categorizadas as variáveis em estudo, como idade, sexo, comorbidades, tempo de sedação, tipo de sedação, sedação utilizada, agitação, confusão, alucinação, sudorese, taquicardia. Resultados: em relação à idade obteve-se uma média de 49 anos, o sexo predominante foi o masculino com 52%, quanto às comorbidades mais frequentes, 20% apresentavam Insuficiência Respiratória Aguda e 16% Insuficiência Renal. O motivo de internação na UTI em maior proporção com 33% foi por dificuldade respiratória e pós-cirúrgicos complicados 32%. Os medicamentos de maior escolha foram midazolam 94%, seguido de fentanil 80%. Quanto ao tempo de sedação dos pacientes, foi encontrada uma média de 1265 horas. As manifestações clínicas mais observadas na amostra correspondem a taquicardia 70%, agitação 52%, confusão e hipertensão 37% e alucinação 24%. Conclusão: as manifestações que ocorreram com maior frequência foram taquicardia, agitação, confusão, hipertensão e, menos frequentemente, alucinação[AU]

Blood-dominant disease in late-and-early-onset lupus: A systematic review and meta-analysis.

OBJECTIVES: Numerous studies have explored hematological manifestations in early-onset systemic lupus erythematosus (erSLE) (age ≤ 50) and late-onset SLE (ltSLE) patients (age > 50), yielding diverse results. This study employs a meta-analysis to examine differences in hematologic manifestations between ltSLE and erSLE. METHODS: Studies investigating the frequency of hematological manifestations in ltSLE patients were included. The frequencies of autoimmune hemolytic anemia (AIHA), thrombocytopenia (TP), lymphopenia, leukopenia, lymphadenopathy, and thrombosis were compared between erSLE and ltSLE groups. Two authors independently reviewed and assessed data consistency among abstracts, tables, and text to mitigate bias. Forest plots were utilized to compare odds ratios (95 % CI) of hematological manifestations by age groups, and study heterogeneity was evaluated using I2. RESULTS: The analysis included 39 eligible studies with 19,103 SLE patients (16,314 erSLE, 2789 ltSLE). Among these studies, 28 reported AIHA which was found to be more frequent in erSLE (OR = 1.29, 95 %CI = 1.11-1.39, p = 0.0008). Twenty studies provided data on lymphopenia which was found to be more frequent in erSLE (OR = 1.184, 95 %CI = 1.063-1.318, p = 0.0021). 32 studies included data on leukopenia and the frequency was higher in erSLE (OR: 1.338, 95 %CI: 1.22-1.47, p < 0.0001). Lymphadenopathy was more prevalent in erSLE (OR = 2.32, 95 % CI = 1.61-3.34, p < 0.0001). No significant difference was observed in thrombosis and TP frequency between the two groups. CONCLUSION: Attributing hematological findings to SLE in late-onset patients presents challenges due to comorbidities and polypharmacy. Overall, the frequencies of AIHA, lymphopenia, leukopenia, and lymphadenopathy were more common in erSLE patients compared to ltSLE in this study.

Multiple sclerosis in a patient with Takayasu's Arteritis: A case report.

Background: Multiple sclerosis (MS) and Takayasu's arteritis (TAK) are two autoimmune diseases that affect the Central nervous system (CNS), but the relationship between them has not been established. Case Presentation: Here we report the emergence of MS during treatment. Takayasu's arteritis in a 24-year-old Iranian woman with a severe presentation. She was treated aggressively with IV methylprednisolone 1 g/day for 3 days and continued with oral prednisolone, also IV cyclophosphamide monthly. After 2 months, loss of vision led to a diagnosis of Optic neuritis (ON) caused by concomitant MS. Conclusion: Differentiating CNS vasculitis associated with Takayasu's arthritis from coexisting MS affecting the CNS is challenging and what is important is to avoid giving a TNF inhibitor.

Isolated Adrenocorticotropic Hormone Deficiency Presenting With Arthritis: A Case Report.

This case report describes a case of isolated adrenocorticotropic hormone (ACTH) deficiency that presented with arthritis. Initial investigations, including blood tests, imaging, and musculoskeletal ultrasonography, did not confirm to any specific connective tissue disease, making it initially difficult to identify the cause of the arthritis. Subsequent adrenal crisis led to the diagnosis of isolated ACTH deficiency through comprehensive endocrine evaluation. The patient's musculoskeletal symptoms were resolved following corticosteroid replacement therapy together with generalized symptoms. This report highlights isolated ACTH deficiency as a rare but one of the causes of polyarticular joint pain and underscores the importance of considering endocrine disorders in the differential diagnosis of unexplained arthritis, particularly when accompanied by systemic symptoms.

Insights into renal and urological complications of inflammatory bowel disease.

Inflammatory bowel disease (IBD) is a chronic condition characterized by immune-mediated inflammation in the gastrointestinal tract, which follows a relapsing and remitting course. Apart from affecting the gastrointestinal tract, IBD also has extra-intestinal manifestations (EIMs). While the etiology of extraintestinal manifestation remains unclear, it is theorized to be based on immunological responses influenced by genetic factors. Renal involvement is one of the EIMs observed in ulcerative colitis and Crohn's disease. The renal manifestations in IBD patients encompass a range of conditions including nephrolithiasis, amyloidosis, tubulointerstitial nephritis, glomerulonephritis (GN), obstructive pathologies, and chronic kidney disease (CKD). The incidence of CKD in IBD patients varies from 5%-15%. The decline in renal function can stem from various factors such as direct inflammatory damage to the kidneys leading to glomerular or tubular injury, or from complications like recurrent stones, amyloidosis, or GN. Additionally, nephrotoxic medications used in treating IBD, such as TNF-α inhibitors, calcineurin inhibitors, and aminosalicylates, can exacerbate the decline in renal function. Currently, there is a lack of consensus regarding these patients' screening and renal function monitoring. This review aims to assess the existing literature on the different renal complications among individuals with IBD, shedding light on their pathophysiology and management.

The Burden of Psychiatric Manifestations in Inflammatory Bowel Diseases: A Systematic Review With Meta-analysis.

BACKGROUND: Psychiatric disorders in patients with inflammatory bowel disease (IBD) represent a significant but uncertain facet of the disease, with unsolved questions regarding their overall magnitude, their impact on intestinal disease, and the whole burden of psychiatric manifestations. AIM: This systematic review summarizes the evidence on the prevalence and impact of psychiatric disorders, including depression, anxiety, bipolar disorder (BD), and schizophrenia, among patients with IBD. METHODS: A systematic search across PubMed/MEDLINE, Embase, and Scopus databases from January 2010 to January 2023 was performed to identify relevant studies. The focus was on studies exploring the prevalence of specific psychiatric disorders in IBD patients compared to the general population and that reported specific outcome measures. A subsequent meta-analysis (MA) assessed the strength of the association between IBD and these psychiatric disorders, with data reliability ensured through rigorous extraction and quality assessment. RESULTS: Out of 3,209 articles, 193 met the inclusion criteria and only 26 provided complete data for comprehensive analysis. These studies showed a significantly higher overall prevalence of psychiatric comorbidities in IBD patients compared to the general population. The MA showed a significant association between IBD and depression (pooled OR 1.42, 95% CI = 1.33-1.52, P < .0001) and anxiety (pooled OR 1.3, 95% CI = 1.22-1.44, P < .0001). The association between IBD and BD was significant (pooled OR 1.64, 95% CI = 1.20-2.24, P < .0001) but showed considerable heterogeneity (I2 = 94.01%). Only 3 studies examined the association between schizophrenia and IBD, providing widely heterogeneous results, with an inconclusive OR, estimated at 0.93 (95% CI = 0.62-1.39, P = .73). CONCLUSIONS: This MA highlights the high prevalence of psychiatric disorders, particularly depression and anxiety, in IBD patients, which exceeds rates in the general population. BD in IBD is proving to be an important but under-researched area. The sparse and contradictory data on schizophrenia requires further investigation. These findings highlight the need for better understanding, early detection, and tailored mental health interventions in the management of IBD to significantly improve patients' quality of life.

This systematic review with meta-analysis establishes a substantial association between inflammatory bowel diseases (IBD) and psychiatric disorders, primarily depression and anxiety. The study emphasizes the need for comprehensive mental health care in IBD management for improved patient outcomes.

[Life style drug laughing gas (nitrous oxide, N2O) and the dependency criteria according to ICD-10]. / Trenddroge Lachgas (Distickstoffmonoxid, N2O) und die Abhängigkeitskriterien nach ICD-10.

OBJECTIVE: The increasing popularity of nitrous oxide (N2O) as a recreational drug raises questions about its potential for dependency. This narrative review examines the dependency risk of N2O using the ICD-10 criteria for substance use disorders and evaluates the current literature. MATERIAL AND METHODS: A comprehensive literature search until April 2024 was conducted to identify publications addressing N2O consumption in the context of dependency criteria. The results were analyzed based on the ICD-10 criteria. RESULTS: Studies showed mixed results regarding craving and loss of control among N2O users. There is evidence of neglect of other interests and potential tolerance development, while data on withdrawal symptoms are limited. Persistent consumption despite harmful consequences has been described but objective diagnostic methods to determine consumption intensity are lacking. DISCUSSION: The data on the dependency potential of N2O are inconsistent. The discussion on its classification as an addictive substance remains controversial; however, the clinical indications suggest a possible risk of dependency, especially with excessive consumption. CONCLUSION: Nitrous oxide is currently primarily regarded as a substance of abuse with the potential to foster psychological dependence, manifesting particularly through loss of control and neglect; however, the criteria for physical dependence, such as the occurrence of withdrawal syndromes and the development of tolerance, have not yet been convincingly documented. Further research is needed to better understand the dependency potential of N2O and develop appropriate preventive and therapeutic measures.

Aberrant copper metabolism and hepatic inflammation cause neurological manifestations in a mouse model of Wilson's disease.

Pathogenic germline mutations in the P-type copper-transporting ATPase (ATP7B) gene cause Wilson's disease (WD), a hereditary disorder characterized by disrupted copper metabolism. The Arg778Leu (R778L) mutation in exon 8 is prevalent among individuals with WD in East Asia and is associated with more severe phenotypes. In this study, we generated a WD mouse model harboring R778L mutation (R778L mice) using CRISPR/Cas9. R778L mice exhibit a range of pathological characteristics resembling those of patients with WD and the same point mutations, including aberrant copper metabolism, pathological cellular injury, inflammation, and severe hepatic fibrosis. At 3-5 months of age, these mice started to display neurological deficits in motor coordination and cognitive dysfunction, accompanied by increased expression of inflammatory cytokines in the central nervous system. Microglia in the striatum and cortex exhibit significant activation, shorter processes, and decreased branch points. However, the Cu2+ levels in the brain tissue of R778L mice did not differ significantly from those of wild-type mice. Notably, inhibition of hepatic inflammation with PJ34 or siNfkb markedly alleviated the deficiencies in cognitive performance and improved locomotor activity in R778L mice. Thus, this study establishes a novel murine model to investigate the pathophysiology of WD, highlights the liver-brain crosstalk responsible for neurological manifestations in individuals with WD caused by the R778L point mutation, and demonstrates the potential of modulating liver inflammation as a therapeutic strategy for alleviating the neurological manifestations of WD.

Main oral characteristics and treatment of ligneous gingivitis and periodontitis in individuals with plasminogen deficiency: A systematic review.

OBJECTIVE: This systematic review evaluated the main clinical, radiographic, histopathological and treatment-related characteristics of ligneous gingivitis (LG) and periodontitis (LP) in individuals with plasminogen deficiency (PD). MATERIAL AND METHODS: Studies in humans diagnosed with PD, focusing on the evaluation of oral characteristics and treatment of the LG/LP were considered for inclusion criteria. Electronic searches were performed up to April 2024 in five databases and in the grey literature. Risk of bias was assessed according to the Joanna Briggs Institute Critical Appraisal Checklists for case reports. It was provided a narrative synthesis of the results. RESULTS: A total of 17 studies were included. All were case reports that analyzed 17 individuals with PD who presented with LG/LP. The relative frequency of PD type I was 56%, while type II constitutes the remaining 44%. In most studies, patients exhibited ulceration clinically, bone loss radiographically, and subepithelial eosinophilic material accumulation on histopathological evaluation. Conventional periodontal scaling was the most used management. All included studies provided well-described clinical characteristics and confirmed plasminogen deficiency through laboratory testing. Only three studies had a risk of bias values lower than 15%. CONCLUSIONS: Current evidence is limited and varied, complicating the diagnosis and treatment of GL/PL. Future studies should provide a more detailed account of treatments and include extended clinical and radiographic follow-up.

Management of COVID-19 in Ophthalmology.

From its outbreak in early 2020, the new SARS-CoV-2 infection has deeply affected the entire eye care system for several reasons. Since the beginning of the COVID-19 pandemic, ophthalmologists were among the "high-risk category" for contracting the SARS-CoV-2 infection based on the notion that the eye was suspected to be a site of inoculation, infection, and transmission. Clinical ophthalmologists have been required to learn and promptly recognize the ocular manifestations associated with the COVID-19 disease, with its treatments and vaccinations. Restriction measures, lockdown periods, guidelines to prevent SARS-CoV-2 infection transmission, and the use of telemedicine and artificial intelligence modalities have induced profound modifications. These changes, which are most likely to be irreversible, influence routine clinical practice, education, and research, thus giving rise to a "new ophthalmology in the COVID era". This book chapter aims to provide several notions regarding COVID-19 in ophthalmology, including the SARS-CoV-2 virus infection and transmission modalities; the ocular manifestation associated with the COVID-19 disease; the drugs and vaccines used for COVID-19; the precautions adopted in the ophthalmic practice to limit the spread of the virus; the consequences of the pandemic on the ophthalmic patients, clinicians, and the entire eye care system; and, the future of ophthalmology in the era of "COVID new normal".

Appropriateness of Ketoanalogues of Amino Acids, Calcium Citrate, and Inulin Supplementation for CKD Management: A RAND/UCLA Consensus.

BACKGROUND: Current treatment for chronic kidney disease (CKD) focuses on improving manifestations and delaying progression. Nutritional approaches play a crucial role in CKD management, and various supplements have become available. Ketoanalogues of amino acids (KAs), calcium citrate, and inulin have been proposed as suitable supplements, yet their widespread use has been limited due to insufficient evidence. This study aimed to generate general guidance statements on the appropriateness of these supplements through a RAND/UCLA consensus process. METHODS: A RAND/UCLA consensus panel was convened to evaluate the appropriateness of these supplements in different clinical scenarios. In this study, we present a subgroup analysis focusing on a panel of eleven clinical nephrologists from among the experts. RESULTS: Supplementation of low-protein diets (LPDs) and very low-protein diets (VLPDs) with KA was considered appropriate to reduce manifestations and delay CKD outcomes, supplementation with calcium citrate is considered appropriate to reduce CKD manifestations, and supplementation with inulin is considered appropriate to delay CKD outcomes and manage comorbidities. CONCLUSIONS: Based on a combination of clinical experience and scientific evidence, the panel reached a consensus that KA supplementation of LPD and VLPD, calcium citrate, and inulin are appropriate in patients with CKD across various scenarios.

Identification of severe fever with thrombocytopenia syndrome virus isolates in the northwest of Hubei Province, China.

Severe fever with thrombocytopenia syndrome (SFTS) is a tick-borne viral disease that is increasingly affecting human being worldwide. The clinical manifestations and mortality rates of SFTS can vary depending on the geographic region and the specific genotype of the SFTS virus (SFTSV). From July 2022 to August 2023, we collected serum samples from 83 patients with suspected SFTSV infection in the northwest of Hubei Province, China. From which, 13 patients tested positive for SFTSV. Phylogenetic analysis of the SFTSV L, M, and S gene segments was performed using the maximum likelihood method to determine the genetic diversity of the isolates. At least 2 SFTSV genotypes (A and F) were identified in the northwest of Hubei Province. The clinical manifestations and laboratory findings on the first day of admission were investigated. Results showed that bleeding and disturbance of consciousness, and significant elevated AST and APTT, are valuable for assessing the prognosis for SFTS patients. This study disclosed the genomic sequences and genotypes of SFTSV spreading in the northwest of Hubei Province for the first time, providing information of genetically etiology for SFTS in the local district. Furthermore, certain symptoms and/or laboratory findings may indicate adverse clinical outcomes, highlighting the importance of identifying the symptoms and monitoring specific laboratory markers. Future research is needed to investigate the threshold values of these markers and to closely observe the indicative symptoms in order to early identify and timely management of critically ill patients within clinical settings.

Ocular Manifestations of Systemic Diseases: Implications for Comprehensive Patient Care.

Background: Ocular manifestations of systemic diseases pose significant challenges to clinicians due to their diverse presentations and potential impact on vision. Understanding these manifestations is crucial for effective patient management and prevention of vision loss. Materials and Methods: In this prospective clinical study, we investigated ocular manifestations in 50 patients with various systemic diseases, including diabetes mellitus, hypertension, autoimmune disorders, and infectious diseases. Patients underwent comprehensive ocular examinations and systemic evaluations to assess the prevalence and characteristics of ocular complications associated with each systemic condition. Conclusion: Diabetic retinopathy was the most common ocular manifestation among diabetic patients, while hypertensive retinopathy predominated in hypertensive individuals. Autoimmune disorders were associated with uveitis and retinal vasculitis, whereas infectious diseases presented with ocular infections. Correlation analyses revealed significant associations between systemic parameters and ocular findings, emphasizing the importance of systemic disease control in preventing vision-threatening complications.

Ambispective epidemiological observational study of varicella-zoster virus infection: An 18 year-single-center Bulgarian experience.

BACKGROUND: Varicella (chickenpox) and herpes zoster (shingles) are outcomes of varicella-zoster virus (VZV) infection, and understanding their incidence trends is vital for public health planning. AIM: To conduct an ambispective epidemiological study by analyzing the main epidemiological characteristics of VZV infection during an 18 year-period (2000-2018). METHODS: We used descriptive and epidemiological methods to characterize chickenpox in Bulgaria, the city of Plovdiv and the region for a period of 18 years (2000-2018). RESULTS: The average incidence of varicella-zoster infection for the period 2000-2018 in the Plovdiv region was estimated at 449.58‰. The highest relative share of the infection was assessed in the month of January at 13.6%, and the lowest in the months of August and September at 2.9% (both months). The age group most affected by the infection was 1-4 years, followed by 5-9 years. This corresponds to the so-called "pro-epidemic population" - a phenomenon typical for airborne infections, confirming their mass impact on the perpetuation of VZV infection. CONCLUSION: Our findings reveal significant insights into VZV epidemiology, including age-specific incidence rates, clinical manifestations, and vaccination impact. This comprehensive analysis contributes to the broader understanding of VZV infection dynamics and may inform evidence-based preventive measures.

Pulmonary Manifestations of IBD: Case Report and Review of the Literature.

This article explores the pulmonary complications associated with inflammatory bowel disease (IBD). It presents a detailed case study of a 22-year-old male with Crohn's disease exhibiting pulmonary symptoms. The review delves into the spectrum of pulmonary involvement in IBD, covering clinical presentations, diagnostic challenges, underlying pathophysiology, and management strategies. It highlights the significance of these extraintestinal manifestations on patient outcomes and quality of life. The article underscores the need for heightened clinical awareness and a systematic approach to diagnosis and management, integrating the expertise of multiple specialists. The review identifies gaps in current research, suggesting avenues for future investigation to enhance the understanding and treatment of these complex manifestations.

Measles-Clinical and Biological Manifestations in Adult Patients, Including a Focus on the Hepatic Involvement: Results from a Single-Center Observational Cohort Study from Romania.

Background: Hepatic involvement in measles, particularly among adolescents and adults, has been recognized since 1960. This involvement typically manifests during the eruptive phase of the disease and is primarily characterized by hepatocellular dysfunction, with jaundice being a less common occurrence. Studies have reported hepatic involvement in 80-86% of measles cases among young adults associated with severe forms of the disease with intra-infectious hepatitis. Recent data from Romania indicated 20,035 confirmed measles cases between January and June 2024, including 17 fatalities and significant hepatic alterations. These findings underscore the need for the further investigation of the hepatic manifestations of measles. The primary objective of our study was to evaluate the clinical and baseline characteristics of the enrolled patients with a brief assessment of liver impairment. Methods: In light of these observations, we conducted a retrospective analysis between 1 November 2023 and 15 June 2024 in patients aged >16 years who were confirmed, by the detection of measles IgM in serum samples, to have acute measles infection and hospitalization. Results: During the study period, 71 hospitalized patients were diagnosed with measles, of whom 37 were female (52.1%), with ages ranging from 16 to 64 years (mean age 34.21 years). Most cases (77.5%) exhibited moderate clinical forms of measles, while 22.5% had severe forms. Respiratory failure requiring oxygen therapy was uncommon (25.4% of severe pneumonia cases). Although a Pearson chi-square test indicated no significant association between the presence of pneumonia and clinical form (p = 0.066), the likelihood ratio test suggested a potential link (p = 0.018). Hepatic involvement was common with elevated AST (87.3%) and ALT (76%) levels. Jaundice was observed in 12.7% of patients. GGT changes were noted in 35.2% of cases, with significant correlations between GGT levels and disease severity (p = 0.001). Analysis of various symptoms revealed significant associations between nausea, dyspnea, and severe clinical forms. Anorexia, diarrhea, and nausea were the most frequently reported symptoms. Thrombocytopenia was observed in 11 patients, with no significant correlation with disease severity. Comorbidities, such as COPD, were significantly associated with disease severity (p = 0.010). Conclusions: Our findings highlight that, while cytolytic hepatic damage is a typical response to measles infection, cholestatic involvement may serve as an indicator of more severe disease progression.

Renal Manifestations of Chronic Hepatitis C: A Review.

Hepatitis C virus (HCV) has emerged as a major global health concern and, if left untreated, can lead to significant liver damage, including cirrhosis, decompensated liver disease, and hepatocellular carcinoma (HCC). Approximately 40% of patients with HCV infection experience extrahepatic manifestations, including renal involvement. HCV-related renal disease is of significant importance among patients with chronic kidney disease (CKD), leading to higher morbidity and mortality. The renal damage due to HCV infection primarily results from cryoglobulinemia and glomerulonephritis, with conditions such as membranoproliferative glomerulonephritis (MPGN) and membranous nephropathy (MN) being most prevalent. Despite advancements in treatment, including the use of directly acting antiviral agents (DAAs), renal complications remain a significant burden in untreated patients. HCV-positive patients on hemodialysis (HD) or those who have undergone kidney transplantation face increased mortality rates compared to their HCV-negative counterparts. Managing HCV infection before kidney transplantation is crucial to mitigate the risk of HCV-related renal complications. Conversely, kidney transplantation from HCV-infected donors is well established, as post-transplant treatment for HCV is safe and effective, potentially reducing mortality and morbidity for patients on transplant waiting lists. This review aims to provide a comprehensive analysis of the renal manifestations of HCV, emphasizing the importance of early diagnosis and treatment to improve patient outcomes.

Systemic complications of Aicardi Goutières syndrome using real-world data.

OBJECTIVE: Aicardi Goutières Syndrome (AGS) is a rare genetic interferonopathy associated with diverse multisystemic complications. A critical gap exists in our understanding of its longitudinal, systemic disease burden, complicated by delayed diagnosis. To address this need, real-world data extracted from existing medical records were used to characterize the longitudinal disease burden. METHODS: All subjects (n = 167) with genetically confirmed AGS enrolled in the Myelin Disorders Biorepository Project (MDBP) were included. As available in medical records, information was collected on subject demographics, age of onset, and disease complications. Information from published cases of AGS (2007-2022; n = 129) with individual-level data was also collected. Neurologic severity at the last available encounter was determined by retrospectively assigning the AGS Severity Scale [severe (0-3), moderate (4-8), and mild (9-11)]. RESULTS: The genotype frequency in the natural history cohort was TREX1 (n = 26, 15.6 %), RNASEH2B (n = 50, 29.9 %), RNASEH2C (n = 3, 1.8 %), RNASEH2A (n = 7, 4.2 %), SAMHD1 (n = 25, 15.0 %), ADAR (n = 34, 20.4 %), IFIH1 (n = 19, 11.4 %), and RNU7-1 (n = 3, 1.8 %). The median age of systemic onset was 0.15 years [IQR = 0.67 years; median range by genotype: 0 (TREX1) - 0.62 (ADAR) years], while the median neurological onset was 0.33 years [IQR = 0.82 years; median range by genotype: 0.08 (TREX1) - 0.90 (ADAR) year]. The most common early systemic complications were gastrointestinal, including dysphagia or feeding intolerance (n = 124) and liver abnormalities (n = 67). Among postnatal complications, thrombocytopenia appeared earliest (n = 29, median 0.06 years). Tone abnormalities (axial hypotonia: n = 145, 86.8 %; dystonia: n = 123, 73.7 %), irritability (n = 115, 68.9 %), and gross motor delay (n = 112, 7.1 %) emerged as the most prevalent neurological symptoms. Previously published case reports demonstrated similar patterns. The median AGS score for the entire cohort was 4 (IQR = 7). The most severe neurologic phenotype occurred in TREX1-related AGS (n = 19, median AGS severity score 2, IQR = 2). Time to feeding tube placement, chilblains, early gross motor delay, early cognitive delay, and motor regression were significantly associated with genotype (Fleming-Harrington log-rank: p = 0.0002, p < 0.0001, p = 0.0038, p < 0.0001, p = 0.0001, respectively). Microcephaly, feeding tube placement, and seizures were associated with lower AGS scores (All: Wilcoxon rank sum test, p < 0.0001). Among the qualifying case reports (n = 129), tone abnormalities were the most prevalent disease feature, with spastic quadriplegia reported in 37 of 96 cases (38.5 %) and dystonia in 30 of 96 cases (31.2 %). CONCLUSIONS: AGS is a heterogeneous disease with multi-organ system dysfunction that compounds throughout the clinical course, resulting in profound neurological and extra-neurological disease impact. Systemic symptoms precede neurologic disease features in most cases. Disease onset before the age of one year, microcephaly, feeding tube placement, and seizures were associated with worse neurological outcomes. This work will inform evidence-based clinical monitoring guidelines and clinical trial design.