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Journal of Neurological Surgery Reports Apr 2024Langerhans cell histiocytosis (LCH) is a rare proliferative systemic disease characterized by the growth of abnormal dendritic cells and wide-ranging organ...
Langerhans cell histiocytosis (LCH) is a rare proliferative systemic disease characterized by the growth of abnormal dendritic cells and wide-ranging organ involvement. This condition can affect individuals of all ages, but most commonly children, with a peak incidence in toddlers. Symptoms may vary depending on the affected organ or system. A 43-year-old man presented with a left temporal stabbing headache unresponsive to management with therapy and nonsteroidal anti-inflammatory drugs. Initial evaluation revealed a contrast-enhanced left temporal extra-axial lesion with bone and muscle compromise. Differential diagnoses, including multiple myeloma, were explored. Initial laboratory tests and imaging studies showed no other abnormalities, except for splenomegaly and a residual granuloma in the left lung. En bloc resection of the lesion was recommended. The patient underwent surgical intervention, which included resection of the dural lesion and all borders of an infiltrating tumor within the temporalis muscle and the affected portion of the left temporal bone. Posterior pathological examination revealed LCH. Postoperative course was uneventful. Follow-up appointments were scheduled after pathology results confirmed the diagnosis. Patient has continued follow-up for the following 3 months after the surgical procedure. Further evaluations are pending. This case report corresponds to a patient with LCH. These patients are individualized and stratified based on local or systemic involvement to determine the most appropriate type of management. This is a rare case as LCH is rare in older patients and the initial presented lesion initially mimicked a meningioma; however, its atypical behavior and associated lytic compromise led to consideration of possible differential diagnoses. LCH can present with lytic bone lesions, mimicking other conditions, including infiltrative neoplastic lesions. Early diagnosis and appropriate surgical management are essential for optimal patient outcomes. Long-term follow-up is crucial to monitor disease progression and response to treatment.
PubMed: 38690582
DOI: 10.1055/s-0044-1786360 -
Clinical and Experimental Rheumatology Apr 2024To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children. (Comparative Study)
Comparative Study
OBJECTIVES
To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children.
METHODS
Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery.
RESULTS
87 patients (43 KD, 44 MIS-C) were included. Age was higher in MIS-C compared to KD group (mean 31±23 vs. 94±50 months, p<0.001). Extremities abnormalities (p=0.027), mucosal involvement (p<0.001), irritability (p<0.001), gallbladder hydrops (p=0.01) and lymphadenopathy (p=0.07) were more often recorded in KD. Neurological findings (p=0.002), gastrointestinal symptoms (p=0.013), respiratory involvement (p=0.019) and splenomegaly (p=0.026) were more frequently observed in MIS-C. Cardiac manifestations were higher in MIS-C (p<0.001), although coronary aneurisms were more frequent in KD (p=0.012). In the MIS-C group, the multiple linear regression analysis revealed that a higher IFN score at onset was related to myocardial disfunction (p<0.001), lymphadenopathy (p=<0.001) and need of ventilation (p=0.024). Both CXCL9 and CXCL10 were related to myocardial disfunction (p<0.001 and p=0.029). IL18 was positively associated to PICU admission (0.030) and ventilation (p=004) and negatively associated to lymphadenopathy (0.004). IFNγ values were related to neurological involvement and lymphadenopathy (p<0.001), IL1b to hearth involvement (0.006). A negative correlation has been observed between IL6 values, heart involvement (p=0.013) and PICU admission (p<0.001).
CONCLUSIONS
The demographic and clinical differences between KD e MIS-C cohorts confirm previous reported data. The assessment of biomarkers levels at MIS-C onset could be useful to predict a more severe disease course and the development of cardiac complications.
Topics: Humans; Mucocutaneous Lymph Node Syndrome; Male; Female; Child, Preschool; Systemic Inflammatory Response Syndrome; Child; Prospective Studies; Infant; COVID-19; Biomarkers
PubMed: 38683206
DOI: 10.55563/clinexprheumatol/l64q51 -
Cureus Mar 2024Lactic acidosis is a rare but severe complication of B-cell lymphoma, often associated with rapid disease progression and poor prognosis. We present a case of a...
Lactic acidosis is a rare but severe complication of B-cell lymphoma, often associated with rapid disease progression and poor prognosis. We present a case of a 60-year-old male admitted with fever, splenomegaly, hemophagocytic tendencies, and lactic acidosis. The patient underwent several dialysis sessions before bone marrow flow cytometry finally confirmed B-cell lymphoma. However, hyperlactatemia persisted and recurred. The case underscores the challenges in diagnosing lymphomas with atypical presentations and emphasizes the critical role of timely bone marrow analysis. Additionally, the paper discusses the association between B-cell lymphoma and lactic acidosis, highlighting the importance of early recognition and intervention.
PubMed: 38681314
DOI: 10.7759/cureus.57146 -
World Journal of Clinical Cases Apr 2024Systemic lupus erythematosus (SLE) is a chronic inflammatory disease primarily affecting young females. SLE can invade any organ, and various forms of splenic invasion...
BACKGROUND
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease primarily affecting young females. SLE can invade any organ, and various forms of splenic invasion have been reported. Manifestations include splenomegaly and splenic infarction, rupture, and calcification. The study encountered a rare case of splenic involvement, with nodules of various sizes without calcifications or ruptures.
CASE SUMMARY
A 15-year-old girl presented with arthralgia, weight loss, fever, increased levels of inflammatory markers, and positive antinuclear antibody test results. The patient was diagnosed with SLE. She was asymptomatic while taking steroids and hydroxychloroquine. Ten months after discharge, the patient developed a fever and abdominal pain. Lupus enteritis was suspected, and abdominopelvic computed tomography (AP-CT) was performed. There were no specific findings in the gastrointestinal tract, but multiple splenic nodules were observed. Infection or hemangioma was considered; however, no specific radiological findings were observed. A biopsy of the spleen was performed to determine the possibility of malignancy. The histological findings of the spleen included extensive periarteriolar necrosis with hematoxylin bodies and numerous karyorrhectic debris. Based on the biopsy results, the patient was diagnosed with an SLE flare-up and was maintained on high-dose steroids and immunosuppressants.
CONCLUSION
As disease activity increased, multiple nodules in the spleen that were previously unseen were observed using AP-CT and histologically confirmed. Spleen invasion by SLE can appear in multiple nodular forms and patterns. Therefore, physicians should consider these findings when differentiating these nodules from infections and malignancies.
PubMed: 38680264
DOI: 10.12998/wjcc.v12.i12.2128 -
Biomedical Journal Apr 2024Autoimmune hepatitis (AIH) is an immune-mediated hepatic disease associated with intense complications. AIH is more common in females and needs effective drugs to treat....
BACKGROUND
Autoimmune hepatitis (AIH) is an immune-mediated hepatic disease associated with intense complications. AIH is more common in females and needs effective drugs to treat. Guizhi Fuling Wan (GZFLW) is a traditional Chinese herbal formula used to treat various gynecologic diseases. In this study, we aim to extend the new use of GZFLW for AIH.
METHODS
The tandem MS-based analysis was used to identify secondary metabolites in GZFLW. Therapeutic effects of GZFLW were tested in a concanavalin A (Con A)-induced AIH model in mice. Ethnopharmacological mechanisms underlying the antiapoptotic, antioxidant, and immunomodulatory protective effects were determined.
RESULTS
Oral administration of GZFLW attenuates AIH in a Con A-induced hepatotoxic model in vivo. The tandem MS-based analysis identified 15 secondary metabolites in GZFLW. The Con A-induced AIH syndromes, including hepatic apoptosis, inflammation, reactive oxygen species accumulation, function failure, and mortality, were significantly alleviated by GZFLW in mice. Mechanistically, GZFLW restrained the caspase-dependent apoptosis, restored the antioxidant system, and decreased pro-inflammatory cytokine production in the livers of Con A-treated mice. Besides, GZFLW repressed the Con A-induced hepatic infiltration of inflammatory cells, splenic T cell activation, and splenomegaly in mice.
CONCLUSIONS
Our findings demonstrate the applicable potential of GZFLW in treating AIH. It prompts further investigation of GZFLW as a treatment option for AIH and possibly other hepatic diseases.
PubMed: 38677491
DOI: 10.1016/j.bj.2024.100731 -
Microorganisms Apr 2024poses a threat to both human and animal health. This work describes an outbreak in a Portuguese rabbit farm, detailing the isolates' clinical manifestations, necropsy...
poses a threat to both human and animal health. This work describes an outbreak in a Portuguese rabbit farm, detailing the isolates' clinical manifestations, necropsy findings, and phenotypic and genomic profiles. Clinical signs, exclusively observed in does, included lethargy and reproductive signs. examination of does revealed splenomegaly, hepatomegaly with a reticular pattern, pulmonary congestion, and haemorrhagic lesions in the uterus, with thickening of the uterine wall and purulent greyish exudates. Positive samples were identified in fattening and maternity units across different samples, encompassing does and environmental samples. Core-genome Multi Locus Sequence Typing (cgMLST) analysis confirmed the outbreak, with the 16 sequenced isolates (lineage II, CC31, and ST325) clustering within a ≤2 allelic difference (AD) threshold. Antimicrobial susceptibility testing for five antibiotics revealed that 15 out of 19 outbreak isolates were resistant to sulfamethoxazole-trimethoprim (SXT). Concordantly, all SXT-resistant sequenced isolates were found to exclusively harbour a plasmid containing a trimethoprim-resistance gene (), along with loci linked to resistance to lincosamides (lnuG), macrolides (mphB), and polyether ionophores (NarAB operon). All sequenced outbreak isolates carried the antibiotic resistance-related genes , , , , , , and . The outbreak cluster comprises isolates from does and the environment, which underscores the ubiquitous presence of and emphasizes the importance of biosecurity measures. Despite limited data on listeriosis in rabbit farming, this outbreak reveals its significant impact on animal welfare and production.
PubMed: 38674729
DOI: 10.3390/microorganisms12040785 -
Microorganisms Mar 2024Systemic infection, also known as cat-scratch disease (CSD), presents a diagnostic challenge due to the variability of clinical manifestations and the potential for...
Systemic infection, also known as cat-scratch disease (CSD), presents a diagnostic challenge due to the variability of clinical manifestations and the potential for serological cross-reactivity with other organisms. This study aimed to retrospectively analyze the epidemiological, clinical, laboratory, and imaging characteristics of pediatric patients diagnosed with systemic infection, to improve understanding and facilitate timely diagnosis and treatment. We conducted a 10-year retrospective study at the "Louis Turcanu" Children's Emergency Hospital and private clinics in Timisoara, Romania, reviewing records for confirmed cases of infection from January 2014 to January 2024. The study adhered to the Declaration of Helsinki and received approval from the Institutional Review Board. Diagnostic criteria included contact with animals, prolonged fever, hematological and/or hepatosplenic manifestations, and positive serological tests for . Nineteen pediatric patients were identified with a median age of 8.1 years. The majority were exposed to felines (94.7%), reflecting the disease's epidemiological profile. Clinical findings highlighted fever (47.4%), lymphadenopathy (78.9%), and less frequently, abdominal pain and headache (both 10.5%). Laboratory analyses revealed a mean hemoglobin of 12.6 mg/dL, WBC count of 13.1 × 10 cells/microliter, and platelet count of 340.6 × 10 per microliter. Significant findings included elevation in ESR and CRP in 47.4% and 21.1% of patients, respectively, and high seropositivity rates for IgM (63.2%) and IgG (94.7%). Imaging studies demonstrated widespread lymphadenopathy and occasional splenomegaly and hepatic microabscesses. All patients received antibiotic therapy, with azithromycin being the most commonly used (94.7%). Co-infections with Epstein-Barr Virus, Cytomegalovirus, and were documented, indicating the complex infectious status of the patients. Systemic infection in children predominantly manifests with fever and lymphadenopathy, with a significant history of exposure to felines. Laboratory and imaging findings support the diagnosis, which is further complicated by potential co-infections. Effective antibiotic therapy, primarily with azithromycin, underscores the need for comprehensive diagnostic and treatment strategies. This study emphasizes the importance of considering systemic infection in pediatric patients with prolonged fever and contact with cats, to ensure timely and appropriate treatment.
PubMed: 38674610
DOI: 10.3390/microorganisms12040666 -
Life (Basel, Switzerland) Apr 2024Myeloproliferative neoplasms (MPNs) are often associated with splanchnic vein thrombosis (SVT). Not all the factors involved in the thrombotic tendency are currently...
ADAMTS13, von Willebrand Factor, Platelet Microparticles, Factor VIII, and Impact of Somatic Mutations in the Pathogenesis of Splanchnic Vein Thrombosis Associated with BCR-ABL-Negative Myeloproliferative Neoplasms.
BACKGROUND
Myeloproliferative neoplasms (MPNs) are often associated with splanchnic vein thrombosis (SVT). Not all the factors involved in the thrombotic tendency are currently known.
OBJECTIVES
This study aims to evaluate a possible association between ADAMTS13, von Willebrand factor (VWF), platelet microvesicles (MV), and factor VIII activity (FVIII:C) with thrombotic events in MPN patients.
MATERIALS AND METHODS
In total, 36 consecutive MPN patients with SVT were enrolled. The MPNs were diagnosed based on clinical characteristics and one or more gene mutations among JAK-2, CALR, and MPL. As controls, 50 randomly selected patients with MPN without thrombosis, 50 patients with deep vein thrombosis without MPNs, and 50 healthy blood donors were evaluated. Complete blood count, ADAMTS13, VWF, MV, and FVIII:C in plasma were measured in all the subjects.
RESULTS
The JAK-2 mutation was found in 94% of the patients with SVT, but none were triple-negative for genetic mutations (JAK2 V617F, CALR, MPL, and exon 12). Compared to the normal subjects, in all the MPN patients (with or without SVT), the levels of ADAMTS13 were found to be significantly lower ( < 0.001) and the MV concentrations were significantly higher ( < 0.001). Among the MPN patients, the VWF and FVIII:C levels were significantly higher in the patients with SVT than those without thrombosis ( = 0.007 and = 0.04, respectively). Splenomegaly was present in 78% of MPN patients with SVT and in 30% of those without SVT ( < 0.001). The ADAMTS13/VWF ratio was reduced in all the patients, but not in the healthy blood donors ( < 0.001).
CONCLUSIONS
The significant increase in circulating MV, VWF, and FVIII:C in the MPN patients and in the patients with thrombosis supports the role of endothelium damage in promoting thrombotic events. In particular, a significant increase in VWF and FVIII:C levels was found in the MPN patients with SVT.
PubMed: 38672756
DOI: 10.3390/life14040486 -
Pathogens (Basel, Switzerland) Apr 2024Ferrets are highly susceptible to a wide range of mycobacteria, mainly , , and . Therefore, ferrets pose a risk of transmission of mycobacteriosis, especially...
Ferrets are highly susceptible to a wide range of mycobacteria, mainly , , and . Therefore, ferrets pose a risk of transmission of mycobacteriosis, especially zoonotically relevant tuberculosis. The aim of this study was to describe the findings of mycobacteriosis in a pet ferret and emphasize its zoonotic potential. A pet ferret had a history of weight loss, apathy, hyporexia, and hair loss. Abdominal ultrasound revealed splenomegaly with two solid masses and cystic lesions of the liver. Fine-needle aspiration cytology revealed numerous acid-fast bacilli in epithelioid cells, thus leading to the suspicion of mycobacterial infection. Because of its poor general condition, the ferret was euthanized. Necropsy examination revealed generalized granulomatous lymphadenitis, pneumonia, myocarditis, splenitis, and hepatitis. Histologically, in all organs, there were multifocal to coalescing areas of inflammatory infiltration composed of epithelioid macrophages, a low number of lymphocytes, and plasma cells, without necrosis nor multinucleated giant cells. Ziehl-Neelsen staining detected the presence of numerous (multibacillary) acid-fast bacteria, which were PCR-typed as . This is the first study showing the antimicrobial susceptibility testing of in veterinary medicine, describing the resistance to doxycycline. Overall, our results could facilitate further diagnosis and provide guidelines for the treatment protocols for such infections.
PubMed: 38668283
DOI: 10.3390/pathogens13040328 -
Heliyon Apr 2024Non-alcoholic fatty liver disease (NAFLD) can lead to irreversible liver damage manifesting in systemic effects (e.g., elevated portal vein pressure and splenomegaly)...
Non-alcoholic fatty liver disease (NAFLD) can lead to irreversible liver damage manifesting in systemic effects (e.g., elevated portal vein pressure and splenomegaly) with increased risk of deadly outcomes. However, the association of spleen volume with NAFLD and related type 2-diabetes (T2D) is not fully understood. The UK Biobank contains comprehensive health-data of 500,000 participants, including clinical data and MR images of >40,000 individuals. The present study estimated the spleen volume of 37,066 participants through automated deep learning-based image segmentation of neck-to-knee MR images. The aim was to investigate the associations of spleen volume with NAFLD, T2D and liver fibrosis, while adjusting for natural confounders. The recent redefinition and new designation of NAFLD to metabolic dysfunction-associated steatotic liver disease (MASLD), promoted by major organisations of studies on liver disease, was not employed as introduced after the conduct of this study. The results showed that spleen volume decreased with age, correlated positively with body size and was smaller in females compared to males. Larger spleens were observed in subjects with NAFLD and T2D compared to controls. Spleen volume was also positively and independently associated with liver fat fraction, liver volume and the fibrosis-4 score, with notable volumetric increases already at low liver fat fractions and volumes, but not independently associated with T2D. These results suggest a link between spleen volume and NAFLD already at an early stage of the disease, potentially due to initial rise in portal vein pressure.
PubMed: 38665588
DOI: 10.1016/j.heliyon.2024.e28123