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Microscopic polyangiitis (MPA) is a form of necrotizing vasculitis affecting the small vessels. Our study is the first study with the objective of describing the difference in clinical presentation of MPA and response to treatment at 6 months based on the age of disease onset. All patients seen at a tertiary vasculitis clinic between 2015 and 2023 with a diagnosis of MPA were included. Patients were divided into an elderly group (age > = 65 years) and a younger group (age < 65). Comparative analysis was conducted to characterize differences amongst the elderly and younger patients, including differences in organ involvement and clinical presentation, Birmingham Vasculitis Activity Score (BVAS) scores, Vasculitis Damage Index(VDI) scores, and response to treatment at 6 months. Thirty-one patients were included in our study. Younger MPA patients (n = 18) with mean age at diagnosis of 53.17 years were compared with older MPA patients(n = 13) with mean age at diagnosis of 76.08 years. The younger patients had statistically significant higher BVAS scores (p = 0.009), along with higher incidence of renal (p = 0.028), pulmonary (p = 0.0069), and cutaneous (p = 0.026) manifestations at disease onset. Furthermore, amongst the younger population, there was noted statistically significant clinical improvement at 6 months following treatment induction, particularly in the domains of general symptoms (p = 0.011), MSK (p = 0.019), cutaneous (p = 0.011), and pulmonary symptoms (p = 0.04). In contrast, the elderly population presented with a predominant of non-specific constitutional symptoms, with statistically significant improvement in the domain of non-specific general symptoms at 6 months (p = 0.00008). All MPA patients responded well to treatment, with statistically significant improvement amongst younger patients (p = 0.0032), but no statistically significant improvement amongst elderly patients (p = 0.67). Our study findings concluded that MPA's clinical presentation differed by age group. Younger patients had more aggressive vasculitis disease presentation with better response to treatments, whereas, elderly patients had less severe disease presentation with predominant of general symptoms at disease onset. Key Points • MPA clinical presentation differed by age at disease onset. Younger patients presented with more aggressive and classic vasculitis-like presentations, with multi-system organ involvement and statistically significant higher incidence of renal, pulmonary, and cutaneous manifestations. In contrast, elderly patients had a predominant of constitutional and non-specific symptoms with often delayed diagnosis. • All MPA patients responded well to treatment. Amongst the younger population, there was statistically significant improvement in BVAS scores after treatment induction at 6 months; however, there was no statistically significant improvement of BVAS scores in the elderly population. • Future studies are required to further understand the difference in the clinical presentation of MPA based on the age at disease onset, and the implication on disease diagnosis and management. [ABSTRACT FROM AUTHOR]

Introduction: Patients with spontaneous intracranial hypotension (SIH) frequently present with several symptoms and impaired mental health. This study systematically investigates the somatic and mental symptom burden of SIH and the effect of surgical treatment. Methods: In this single-center retrospective study, patients with surgical closure of a spinal cerebrospinal fluid leak between September 2022 and July 2023 completed the Somatic Symptom Scale (SSS-8), the Somatic Symptom Disorder - B Criteria Scale (SSD-12), and the Patient Health Questionnaire (PHQ-8) preoperatively and three and 6 months postoperatively. Results: Fifty-seven patients were included. All three scores showed clearly pathological values before surgery (SSS-8: 12 [IQR 6.5-16], SSD-12: 26 [IQR 19.5-33.5], PHQ-8: 11 [IQR 6.5-15]) representing a high somatic symptom burden and relevant current depression. After surgery, there was a significant and sustainable improvement (SSS-8: 8 [IQR 3-11.75], SSD-12: 12.5 [IQR 5-21.75], PHQ-8: 4.5 [IQR 2-9], p < 0.001, respectively) that exceeded the minimal clinically important difference for every score. Conclusion: SIH presents with high somatic and mental symptom burden. Surgical treatment leads to a relevant improvement of somatic and depressive symptoms. However, even after surgical success some patients still exhibit elevated depressive scores. Depressive symptoms might be added to the typical symptomatology of SIH. [ABSTRACT FROM AUTHOR]

Rapidly progressive hip osteoarthritis (RPOH) is a rare and severe form of osteoarthritis (OA), marked by the rapid degeneration and destruction of the femoral head, often within months. Despite its unclear etiology, several factors such as subchondral fractures and immune responses have been proposed as possible contributors. This narrative review aims to synthesize current knowledge on the pathogenesis, risk factors, clinical presentation, imaging features, and grading systems of RPOH. Predominantly affecting elderly females, RPOH presents distinctive challenges in both diagnosis and management due to its abrupt onset and severity. Known risk factors include advanced age, female gender, obesity, intra-articular corticosteroids use, and long-term hemodialysis. Clinically, RPOH is characterized by severe pain during active weight-bearing movements, despite patients presenting a normal range of motion during passive examination in the early stages. While several classification systems exist, there is no universal standard, complicating differential diagnosis and clinical approaches. This review emphasizes the necessity for early diagnostic methods utilizing specific biomarkers, rapid differential diagnosis, and targeted, personalized interventions based on individual risk factors. [ABSTRACT FROM AUTHOR]

Background: Differentiated thyroid carcinoma (DTC) in children is uncommon; clinical presentation over recent decades is incompletely characterized. Methods: This retrospective cohort study analyzed demographic and disease characteristics of consecutive juveniles with DTC treated from 1970 to 2015 at Poland's largest pediatric DTC referral center, Maria Sklodowska-Curie National Research Institute of Oncology, Gliwice, who had available records. Sex, age, histopathological characteristics, and DTC stage were documented. We aimed to identify changes in these variables over time and independent risk factors for lymph node or distant metastases. Trends in these variables were assessed using the Cochran–Armitage test and Spearman correlation. Multivariable logistic regression was performed to identify risk factors associated with lymph node or distant metastases. Results: 475 of 479 patients (99.2%) were included in the analysis; roughly half were age ≥15 years, 10%, <10 years. Papillary thyroid carcinoma (PTC) represented 88% of cases and follicular thyroid carcinoma (FTC) 11%. Tumors ≤2 cm constituted 56% of cases with relevant data; those >4 cm accounted for 12%. Multifocality was observed in 37% and extrathyroidal invasion in 22%. Lymph node metastases were noted in 59% and distant metastases in 16%. Over the observation period, significant trends among new cases included: increased proportion of adolescents >15 years; increased frequency of tumors ≤2 cm, decreased multifocality rates, and increased proportion of PTC versus FTC. Extrathyroidal invasion rates remained appreciable throughout, ranging from 17 to 28% during the 5-year study subperiods after 1990. Lymph node metastases significantly increased in frequency in the central neck, remaining consistently common in lateral sites; presence of distant metastases significantly decreased. In multivariable analysis, multifocality, extrathyroidal invasion, and tumor size were independently associated with lateral lymph node metastases and multifocality, larger tumor size, and N1b metastases with distant spread. Conclusions: Our observations of a rising proportion of diagnoses in adolescence, reductions in primary tumor size, and decreased frequency of multifocality and distant metastases may reflect increased detection of patients with less aggressive DTC at earlier disease stages. Nonetheless, we found persistently substantial rates of locoregionally advanced disease features (multifocality, extrathyroidal invasion, and lymph node metastases), which multivariable analyses suggested have significant associations with lateral lymph node and/or distant metastases. [ABSTRACT FROM AUTHOR]

Background and aims: The clinical presentation of celiac disease (CD) has changed over time with more patients presenting with non-classical symptoms, extra-intestinal manifestations (EIM) or no symptoms. We aimed to investigate the main symptoms/signs leading to the diagnosis of CD in adult patients. As secondary end-point, we evaluated the outcome of gastrointestinal (GI) symptoms following gluten-free diet (GFD). Methods: All consecutive CD adult patients referring to our University Hospital from September 2022 to February 2024 were included. Clinical data were retrospectively evaluated. Results: 134 patients, 104 females/30 males, median age at diagnosis 35 years, were included. 79 patients reported GI symptoms (i.e., diarrhea, abdominal bloating, dyspepsia) as the main symptom leading to CD diagnosis. In 40 patients, the leading symptom/sign was an EIM (i.e., iron deficiency anemia, infertility/miscarriages, dermatitis, osteoporosis, elevated transaminase levels). Fifteen patients were asymptomatic, being diagnosed because of a positive family history or concomitant autoimmune hypothyroidism. Of the 79 patients reporting GI symptoms, 20 did not experience complete resolution with the GFD. Among the 17 patients who reported a strict adherence to GFD (vs 1 patient with low-adherence, 2 non-compliant), lactose intolerance and irritable bowel syndrome overlap were diagnosed in 2 and 15 patients, respectively. Conclusion: GI manifestations remain the main symptoms at presentation of CD, however clinicians should be aware of the EIM of CD and the association with other autoimmune disorders. In non-responsive CD patients, an overlap with functional disorders might be considered. [ABSTRACT FROM AUTHOR]

Fibrous histiocytoma (FH) within the finger, particularly involving the flexor tendon sheath, is a rare entity. While cutaneous fibrous histiocytomas have been documented in the fingers, their occurrence around the flexor sheath presents a unique diagnostic challenge, often resembling giant cell tumor of the tendon sheath (GCTTS). We report a case of benign fibrous histiocytoma involving the tendon sheath in the index finger of a 70-year-old patient and discuss its clinical presentation, differential diagnoses, histopathological features, and management. [ABSTRACT FROM AUTHOR]

Congenital adrenal hyperplasia is an autosomal recessive disorder caused by complete or partial defects in one of the several steroidogenic enzymes involved in synthesizing of cortisol from cholesterol in the adrenal gland. Prompt and proper treatment of the disease would reduce symptoms and the level of androgens in patients. The present study aimed to evaluate the demographic characteristics and clinical findings of these patients. This retrospective investigation was conducted in 146 patients with congenital adrenal hyperplasia participated. Their clinical and paraclinical findings were accurately recorded in the file and extracted from the records. Among all 146 patients, 119(81.5 %) was 21-OH Deficiency type;11-OH Deficiency type was 13(8.9 %), 10(6.8 %) was 3β-HSD type, StAR was 2(1.4 %) and 17 alpha(α)-hydroxylase Deficiency was 2(1.4 %). The mean age of disease onset in these patients was 2.45 ± 1.16 years. Macropenis was the most frequent clinical finding in 39 cases of 64 boys (60.9 %), and Clitoromgaly was the most clinical presentation in 40 cases of 82 girls (48.7 %). The levels of testosterone, dehydroepiandrosterone sulfate, and 17-OHP significantly decreased in the last visit compared to the initial diagnosis. Based on the clinical findings in every infant or child with ambiguous genitalia, macropenis, clitoromegaly, hirsutism, and premature pubarche, we should consider congenital adrenal hyperplasia. Prompt and proper treatment and disease control would reduce symptoms and the level of androgens in patients. [ABSTRACT FROM AUTHOR]

Background: Atrial fibrillation (AF) is the most common and persistent cardiac arrhythmia in clinical practice, with an overall prevalence in the world is estimated to be 0.47%, but there is significant regional variation. Developing countries including India lacks data on atrial fibrillation, hence this study was conducted to study clinical presentation of patients with atrial fibrillation. Methodology: A descriptive study was conducted to assess clinical presentation of patients with atrial fibrillation admitted in all the in-patient departments of tertiary care hospital for a period of 1 year. A pretested, semi structured questionnaire was used to interview study subjects. In this study, the minimum age of the study population with atrial fibrillation was 22 years and the maximum age was 85 years. The male to female ratio was almost equal with a slight male predominance of 33 cases (55%). The most common symptom was shortness of breath i.e. in 39(65%) patients. The prevalence of atrial fibrillation increases with age. There was no significant difference in clinical presentation between males and females. Clinical presentation such as breathlessness, orthopnoea and pedal oedema was more frequently seen in study subjects below 60 years than individuals who are 60 years and above. [ABSTRACT FROM AUTHOR]

Introduction: Non - Anterior ischemic optic neuropathy (NAION) is the most common acute optic neuropathy mainly affecting adults more than 50 yrs. NAION is mainly because of ischemia of optic nerve head. It’s mainly of two types arteritic and non arteritic. The exact mechanism of vasculopathy in NAION remain unknown, however several risk factors have been found to be associated with NAION, which can be further classified into ocular risk factor and systemic risk factor. Ocular risk factor includes small crowded disc often defined as disk at risk, optic nerve head drusen’s, vascular disorder in the nutrient vessels of the optic nerve head, cataract extraction, markedly increased intra ocular pressure and marked optic disc edema due to any cause. 60% of NAION patients have systemic risk factor affecting small vessels like arterial hypertension, diabetes mellitus, arteriosclerosis, atherosclerosis, cigarette smoking, nocturnal arterial hypotension, ischemic heart diseases, cerebrovascular accidents, hyperhomocystenemia, thyroid disease. Recently OSA as a risk factor has also been identified. Materials and Methods: This case control study was carried out in the Department of Ophthalmology, S.S. Medical College and associated GM Hospital, Rewa, over a period of 18 months among 32 consented patients presenting to the department with features suggestive of NAION and 32 age and gender matched control, with no ocular pathology. Detailed history was recorded and thorough clinical and comprehensive ophthalmic examination was carried out. Data was collected, compiled and analyzed using SPSS 22.0 (trial version). Result: Mean age of study group was 53.34±9.10 years. Mean VA in affected eye was 1.36±0.74 log MAR units. Color vision of NAION patient was defective in almost all patients. Mean number of Ishihara plates read was 5.41±1.55. Mean contrast sensitivity was 0.67±0.43 log units. The most common systemic disease associated with NAION was diabetes mellitus (62.5%) followed by hypertension (46.87%). NAION patients had higher incidence of OSA. None of patients had history of IHD, TIA or stroke. Conclusion: The data for NAION comes mainly from western literature as there have been only few studies in Indian population. Through this study, we aimed to provide an overview of NAION, systemic risk factors associated with it in Indian Population. [ABSTRACT FROM AUTHOR]

Routine evaluation and surveillance imaging after pituitary adenoma (PA) endoscopic endonasal transsphenoidal resection (EETS) is a neurosurgical practice to identify tumor recurrence. This study aims to identify social and clinical factors that may contribute to patients missing their initial 1-year follow-up appointment and provide guidance for targeted education to improve patient adherence with postoperative treatment plans, ultimately reducing unknown adenoma recurrence. The authors performed a single-center retrospective review of patients who underwent EETS for PAs from 2007 to 2023. Patients were analyzed for sociodemographic factors, presenting symptoms, time to surgery, surgical outcomes, and adherence to postoperative follow-up visits at 1 year after surgery. A total of 256 patients with PAs treated by EETS met inclusion criteria; 218 (85%) of these patients attended 1-year follow-up, and 38 (15%) missed this visit. Twenty-nine (76%) individuals who missed their 1-year follow up were men (P = 0.006). Divorced/widowed/separated patients were 2 times more likely to miss their follow-up compared with their married counterparts (P = 0.008). Additional significant risk factors included older age, as the mean age for patients who missed their 1-year appointment was 60.1 years compared with 54.7 years (P = 0.028). Patients with visual field deficits at initial presentation were also less likely to follow-up at 1 year (P = 0.03). Risk factors of missed 1-year follow-up appointments after PA resection include male sex, divorced/widowed/separated marital status, older age, and the presence of visual deficits at initial presentation. Increased education efforts can be selectively aimed at these at-risk patient cohorts to improve patient compliance and reduce consequences of undetected tumor recurrence. [ABSTRACT FROM AUTHOR]

Purpose: Angioleiomyoma, predominantly arising from the extremities, is a benign soft tissue tumor. Reports on its intracranial location are rare. We assessed clinical, radiological, and pathological features of intracranial angioleiomyoma (iALM) treated at our neurosurgical institution. Methods: We consecutively enrolled all patients with neuropathologically confirmed iALM treated at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were analyzed. A review of the literature on iALM was conducted. Results: Seven patients with iALM (four female) with a median age of 45 years (range: 32–76 years) were identified. In three cases, the lesion was found incidentally. In magnetic resonance imaging (MRI), all tumors were hypo- to isointense on T1-weighted, hyperintense on T2-weighted sequences, and gadolinium-enhancing. A strong FLAIR signal was seen in six patients. Surgery consisted of gross total resection in all cases without perioperative complications. Neuropathological staining was positive for smooth muscle actin (SMA) in all lesions. Mature smooth muscle cells arranged around blood vessels were typically observed. The Ki-67 index was ≤ 3%. The patients were discharged after a median of 6 days (range: 4–9 days). During a median follow-up time of 14 months (range: 4–41 months), no tumor recurrence occurred. In the current literature, 42 additional cases of iALM were identified. Conclusion: Intracranial angioleiomyoma is a benign soft tissue tumor treated by gross total resection. Tumor morphology and positive staining for SMA lead to the neuropathological diagnosis. [ABSTRACT FROM AUTHOR]

Introduction The aim of the study was to investigate the associations between the presence and level of rheumatoid factor (RF) in the blood serum and the clinical and laboratory characteristics of patients with systemic lupus erythematosus (SLE). Material and methods This retrospective tricentric cross-sectional study analyzed a Ukrainian contingent of SLE patients. Medical records of 495 patients were evaluated. Rheumatoid factor serum concentration was tested in 206 of them (41.6%) using turbidimetry technique. Clinical manifestations, routine laboratory parameters, specific immunological tests, disease activity (SLEDAI-2K), and damage indices (SLICC/ACR DI) were evaluated. Results Our study revealed that RF was elevated in 27.7% of patients. The RF-positive patients experienced a longer delay in SLE diagnosis (2.0 vs. 0.5 years, p = 0.046), less frequent kidney involvement (42.1% vs. 59.4%, p = 0.045) and fever (42.1% vs. 59.2%, p = 0.046), and more frequent lymphadenopathy (59.6% vs. 42.3%, p = 0.039) compared to RF-negative patients. Patients with RF positivity had higher levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer, and were more frequently positive for antibodies to Ro/SSA and La/SSB. Rheumatoid factor concentration directly correlated with CRP ( r = 0.318; p < 0.01) and ESR ( r = 0.228; p = 0.04) levels. However, no associations were found between RF levels and SLEDAI-2K, joint involvement frequency, SLICC/ACR DI or drug therapy content. Univariate logistic regression analysis showed that RF positivity was independently associated with lymphadenopathy, presence of anti-Ro/SSA and anti-La/SSB antibodies, and negatively associated with kidney involvement. Conclusions In RF-seropositive SLE patients (approximately 28%), the diagnosis is established later compared to RF-seronegative ones; kidney involvement and fever are less common, while lympha­denopathy develops more frequently. Rheumatoid factor seropositivity is associated with higher levels of ESR, CRP, ANA, and the presence of antibodies to Ro/SSA and La/SSB. According to the results of univariate logistic regression analysis, an independent association with RF positivity was confirmed only for kidney involvement, lymphadenopathy, and antibodies to Ro/SSA and La/SSB.

Introduction: The ovaries, fallopian tubes, and the broad ligament make up the anatomical adnexa. Adnexal masses represent a commonly occurring issue in gynaecology. The majority of ovarian malignancies are detected at advanced stages. The clinical manifestations of adnexal masses vary, with many cancers either showing no symptoms or presenting with vague signs. In the reproductive age group, the most typical manifestation is a benign functional cyst. Aim: To conduct a clinico-pathological analysis of adnexal masses among women presenting at a tertiary healthcare centre. Materials and Methods: A retrospective observational study was conducted in the Department of Obstetrics and Gynaecology at Akash Institute of Medical Science and Research Centre, Devanahalli, Karnataka, India, from November 2022 to April 2024. The diagnosis of adnexal mass was based on clinical symptoms, abdominal examination, bimanual examination, and trans-abdominal ultrasound. The CA-125 and other tumour markers were sent depending on the risk of malignancy. All tissues were sent for histopathological examination. The data were collected and tabulated in a Microsoft excel sheet, and percentages were calculated. Results: This study included 38 cases, in which benign ovarian and para-ovarian cysts were the most common findings, observed in 21 cases (55.3%). There were six cases of benign serous cystadenoma (15.8%), three cases of teratoma (7.9%), two cases of high-grade serous carcinoma (5.3%), one case of germ cell tumour (2.6%), one case of mucinous cystadenoma (2.6%), two cases of endometriotic cysts (5.3%), one case of adnexal cystic degeneration of a fibroid (2.6%), and one rare case of primary fallopian tube carcinoma (2.6%). The most common presenting complaint was abdominal pain, reported in 21 cases (55.3%). Conclusion: Adnexal masses can present in a variety of forms, ranging from benign cysts to infections and cancers. A thorough history and clinical examination are essential for accurate diagnosis. Investigations support the diagnosis, and histology confirms it.

Long COVID, also known as PASC (post-acute sequelae of SARS-CoV-2), is a complex infection-associated chronic condition affecting tens of millions of people worldwide. Many aspects of this condition are incompletely understood. Among them is how this condition may manifest itself in older adults and how it might impact the older population. Here, we briefly review the current understanding of PASC in the adult population and examine what is known on its features with aging. Finally, we outline the major gaps and areas for research most germane to older adults. [ABSTRACT FROM AUTHOR]

Background: HIV/AIDS is a major cause of infant and childhood morbidity and mortality in Africa. It is also an escalating problem of frightening proportions in Nigeria. Objectives: To determine the mode of transmission, clinical presentation, co-morbidity and the outcome among children with HIV/AIDS at the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt. Methods: From January 2003 to December 2007, all children with HIV infection who registered in our clinic were followed up prospectively. Clinical and laboratoryevaluation were performed at presentation, the children were managed according to standard treatment guidelines and the outcome noted. Results: Of the 384 children with HIV infection, 190 (49.5 percent) were males and 194 (50.5 percent) females; a M: F ratio of 0.98:1. Their ages ranged from five months to 180 months. Two hundred and sixty three (68.5 percent) of the children were less than 18 months of age at presentation. Three hundred and forty six (90.1 percent) of the children acquired the infection vertically. The common symptoms at presentation in the 336 (87.5 percent) of the children who were symptomatic included fever in 75.3 percent, cough in 64.9 percent, weight loss in 41.1 percent and diarrhoea in 40.8 percent. The common signs were generalized lymphadenopathy in 44.1 percent, pallor in 39.3 percent and hepatomegaly in 38.1 percent. Common co-morbidities included tuberculosis in 23.7 percent and pneumonia in 15.4 percent. Majority (66.9 percent) presented in WHO stage 3 and 4. Twenty seven (7.0 percent) have died while 67 (17.4 percent) have been lost to follow up. Age specific mortality was highest among those aged below 18 months. The greatest contributors to case fatality were pneumonia (44.4 percent) and malnutrition (33.3 percent). Conclusion: Paediatric HIV/AIDS is predominantly transmitted from mother to child and constitutes a significant cause of childhood morbidity and mortality at the UPTH. In view of a high rate of those lost to follow up, it is advocated that strategies such as support groups and expert patient training should be put in place to track down defaulters.

Abstract Objective To examine the clinical characteristics, diagnosis and treatment, and prognosis of ocular amyloidosis in a Chinese population. Methods A retrospective case series study was conducted. The clinical data of 37 patients with ocular amyloidosis were collected and the clinical characteristics, diagnosis and treatment, and prognosis were summarized and analyzed. Results The 37 patients included 12 males and 25 females ranging in age from 22 to 75 years, with median age of 49 years. The clinical signs and symptoms included a conjunctival mass in 37 patients (100%), periorbital discomfort or pain in 29 patients (61.9%), ptosis in 18 patients (23.8%), exophthalmos or eyeball displacement in 3 patients (14.3%), restricted eye movement in 2 patients (9.52%), vision loss in 1 patient (4.76%), and diplopia in 1 patient (4.76%). A total of 29 patients had only conjunctival involvement and 8 patients had concomitant orbital and conjunctival involvement. The main treatment for patients with conjunctival involvement was surgical resection. Thirty-one patients had stable disease, 4 patients progressed or relapsed, and 2 patients were lost to follow-up. Conclusion Ocular amyloidosis most commonly presents as an eyelid or conjunctival mass or diffuse thickening and can also present as an orbital mass. Diagnosis is mainly dependent on histopathological examination. Surgery is the main treatment and is done to confirm the diagnosis to guide further treatment, preserve function, and prevent complications that threaten visual acuity. Close postoperative follow-up is necessary.

Abstract Introduction Tuberculous lymphadenitis (TBLN) is an infection of the lymph node caused by Mycobacterium tuberculosis. Histological diagnoses of presumptive patients are often accompanied by cytomorphological features. However, the sensitivities of these features are often precluded by the variable degrees of narrative similarities compared to other diagnostic modalities. Objective The aim of this study was to investigate and compare the cytomorphological and clinical features of presumptive TBLN patients with bacteriological detection methods. Methods A similar cohort of TBLN patients from our previous study who were enrolled prospectively from the ALERT Specialized Hospital, Addis Ababa, Ethiopia, was considered for this analysis. SPSS version 26 was used for data analysis. Descriptive analysis was conducted to characterize the study population using the independent variable and presented with frequency tables. The chi-square test was used to measure the association. A P-value of

Polycystic Ovary Syndrome (PCOS) is a multifaceted endocrine disorder affecting women across various life stages, presenting a wide array of symptoms that differ markedly during adolescence, reproductive age, and menopause. In adolescence, the overlap of PCOS symptoms with normal pubertal changes complicates diagnosis, with common presentations including menstrual irregularities, acne, and hirsutism. During the reproductive years, PCOS is often associated with infertility, menstrual dysfunction, and metabolic complications such as insulin resistance and type 2 diabetes. In menopausal women, reproductive symptoms diminish, but the risk of cardiovascular disease, persistent hyperandrogenism, and metabolic syndrome becomes more pronounced. This review aims to define the distinct clinical presentations of PCOS across these life stages, highlighting the diagnostic and management challenges inherent to its heterogeneous nature. By understanding the age-specific manifestations and complications of PCOS, healthcare providers can develop more effective, individualized therapeutic strategies, ultimately improving patient outcomes and quality of life.

Abstract Background Invasive meningococcal disease (IMD) cases declined upon the implementation of non-pharmaceutical interventions (NPI) (social distancing and mask wearing) to control the COVID-19 pandemic but rebounded in 2022 in numbers with genotypical changes of the strains. We explored here associated modifications in the clinical presentations of IMD. Methods We conducted a retrospective descriptive study using the Database of the French National Reference Centre for meningococci and Haemophilus influnezae for IMD cases between 2015 and 2022. We scored serogroups, sex, age groups, clinical presentations and clonal complexes of the corresponding patients and isolates. Findings Non-meningeal forms of IMD increased significantly upon easing of NPI, such as bacteremic meningococcal pneumonia and bacteremic abdominal forms. They represented 6% and 8% of all IMD forms and were significantly linked to serogroups Y and W respectively, to older adults for bacteremic pneumonia and to young adults for bacteremic abdominal presentations. These forms were significantly associated with more early mortality and clonal complexes 23, 11 and 9316. Interpretation The increase in atypical IMD forms may lead to higher burden of IMD due to delayed diagnosis and management. Updating prevention may be needed through by adapting the current vaccination strategies to epidemiological changes.

Objectives: In this study, hematological changes in both symptomatic and asymptomatic one-humped dromedary camels were investigated. Materials and Methods: Thirty dromedary camels, aged between 1 to 10 years, from different regions of Northern Nigeria and neighboring African countries, were sampled at the Mai'adua International Livestock Market in Katsina State, Nigeria. The camels were randomly categorized into symptomatic (10) and asymptomatic (20) groups based on clinical presentations such as diarrhea, poor body condition, tick infestation, and cutaneous lesions. Blood samples were collected from each camel into labeled vacutainers containing ethylenediaminetetraacetic acid, and subsequently analyzed using a veterinary hematology analyzer. Results: The findings indicated a significantly (p < 0.05) increased packed cell volume (PCV), red blood cell count (RBC), hemoglobin concentration (Hb), and neutrophil count in symptomatic camels compared to asymptomatic ones. Conversely, there was a significant (p < 0.05) increased lymphocyte count in symptomatic camels compared to their asymptomatic counterparts. Conclusion: The hematological parameters, including RBC, PCV, Hb, neutrophil, and lymphocyte counts, exhibited significant alterations in symptomatic camels compared to asymptomatic ones. Further research is recommended to investigate the underlying causes of hematological alterations in symptomatic camels and the potential impact of factors such as age, nutrition, and regional variations on camel hematological parameters. [Vet. Res. Notes 2024; 4(6.000): 65-68]

The presenting pattern of celiac disease (CD) at diagnosis in children has changed over time, with a reduction of malabsorption-related phenotypes and an increase in regular or even excessive growth patterns. We retrospectively reviewed the body mass index (BMI) distribution of all patients with a new diagnosis of CD made in a Pediatric Gastroenterology Outpatient Clinic in 1990–2011, compared to those diagnosed in 2012–2022, according to their clinical and serological characteristics. The 1990–2011 and 2012–2022 cohorts included 250 (M:F = 90:160, mean age 7.3 ± 6.1 years) and 243 children (M:F = 81:162, mean age 7.1 ± 3.7 years, NS), respectively. The prevalence of underweight (UW) was higher in the 1990–2011 cohort (61/250, 24.4% in 1990–2011 vs. 31/243, 12.7% in 2012–2022, p = 0.0001), whereas that of overweight (OW) and obese (OB) subjects was significantly higher in 2012–2022 (10/250, 4% in 1990–2011 vs. 24/243, 9.9% in 2012–2022, p = 0.012, and 1/250, 0.4% in 1990–2011 vs. 8/243, 3.3% in 2012–2022, p = 0.018, respectively). In both cohorts, gastrointestinal symptoms were more frequent in OW/OB than in UW children (6/11, 54.5% vs. 5/61, 8.2% in 1990–2011, p < 0.0001, and 24/32, 75% vs. 10/31, 32.3%, p < 0.0001 in 2012–2022), and the extent of anti-transglutaminase antibody increase was similar in OW/OB and UW subjects. The prevalence of children with a normal or even high BMI at CD diagnosis has increased in the past three decades; therefore, CD should be suspected regardless of BMI status. [ABSTRACT FROM AUTHOR]

Objective: To examine the clinical characteristics, diagnosis and treatment, and prognosis of ocular amyloidosis in a Chinese population. Methods: A retrospective case series study was conducted. The clinical data of 37 patients with ocular amyloidosis were collected and the clinical characteristics, diagnosis and treatment, and prognosis were summarized and analyzed. Results: The 37 patients included 12 males and 25 females ranging in age from 22 to 75 years, with median age of 49 years. The clinical signs and symptoms included a conjunctival mass in 37 patients (100%), periorbital discomfort or pain in 29 patients (61.9%), ptosis in 18 patients (23.8%), exophthalmos or eyeball displacement in 3 patients (14.3%), restricted eye movement in 2 patients (9.52%), vision loss in 1 patient (4.76%), and diplopia in 1 patient (4.76%). A total of 29 patients had only conjunctival involvement and 8 patients had concomitant orbital and conjunctival involvement. The main treatment for patients with conjunctival involvement was surgical resection. Thirty-one patients had stable disease, 4 patients progressed or relapsed, and 2 patients were lost to follow-up. Conclusion: Ocular amyloidosis most commonly presents as an eyelid or conjunctival mass or diffuse thickening and can also present as an orbital mass. Diagnosis is mainly dependent on histopathological examination. Surgery is the main treatment and is done to confirm the diagnosis to guide further treatment, preserve function, and prevent complications that threaten visual acuity. Close postoperative follow-up is necessary. Keypoints: • Ocular amyloidosis most commonly presents as an eyelid or conjunctival mass or diffuse thickening and can also present as an orbital mass. • Diagnosis is mainly dependent on histopathological examination. Surgery is the main treatment and is done to confirm the diagnosis to guide further treatment, preserve function, and prevent complications that threaten visual acuity. [ABSTRACT FROM AUTHOR]

Introduction: Tuberculous lymphadenitis (TBLN) is an infection of the lymph node caused by Mycobacterium tuberculosis. Histological diagnoses of presumptive patients are often accompanied by cytomorphological features. However, the sensitivities of these features are often precluded by the variable degrees of narrative similarities compared to other diagnostic modalities. Objective: The aim of this study was to investigate and compare the cytomorphological and clinical features of presumptive TBLN patients with bacteriological detection methods. Methods: A similar cohort of TBLN patients from our previous study who were enrolled prospectively from the ALERT Specialized Hospital, Addis Ababa, Ethiopia, was considered for this analysis. SPSS version 26 was used for data analysis. Descriptive analysis was conducted to characterize the study population using the independent variable and presented with frequency tables. The chi-square test was used to measure the association. A P-value of < 0.05 was considered statistically significant. Results: Using FNAC, 60/126 (47.6%) of the participants were reported to have features consistent with TB. Of the total FNAC-positive cases, many (30/60 and 27/60) showed pattern B (caseous necrosis only) and pattern C (epithelioid granuloma with caseous necrosis), respectively. Strong concordance was observed in Pattern A (abundant caseous necrosis with few epithelioid macrophages) followed by patterns B and C with GeneXpert and MGIT culture (P value < 0.001). Night sweats and alcohol intake were shown to correlate with positive cases as reported by FNAC (P value = 0.008 respectively), GeneXpert (P value = 0.02 & 0.001), and culture methods (P-value = < 0.001 & 0.002). Conclusion: Cytomorphological features, particularly patterns A, B, and C, could be considered in the diagnosis of TBLN given their comparable outcomes with bacteriological detection methods. On another note, we recommend that due care and attention be given when treating TBLN patients based solely on clinical presentation, as these diagnostics may be prone to false results, leading to inappropriate administration of anti-TB drugs and other consequences. [ABSTRACT FROM AUTHOR]

Introduction: The aim of the study was to investigate the associations between the presence and level of rheumatoid factor (RF) in the blood serum and the clinical and laboratory characteristics of patients with systemic lupus erythematosus (SLE). Material and methods: This retrospective tricentric cross-sectional study analyzed a Ukrainian contingent of SLE patients. Medical records of 495 patients were evaluated. Rheumatoid factor serum concentration was tested in 206 of them (41.6%) using turbidimetry technique. Clinical manifestations, routine laboratory parameters, specific immunological tests, disease activity (SLEDAI-2K), and damage indices (SLICC/ACR DI) were evaluated. Results: Our study revealed that RF was elevated in 27.7% of patients. The RF-positive patients experienced a longer delay in SLE diagnosis (2.0 vs. 0.5 years, p = 0.046), less frequent kidney involvement (42.1% vs. 59.4%, p = 0.045) and fever (42.1% vs. 59.2%, p = 0.046), and more frequent lymphadenopathy (59.6% vs. 42.3%, p = 0.039) compared to RF-negative patients. Patients with RF positivity had higher levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer, and were more frequently positive for antibodies to Ro/SSA and La/SSB. Rheumatoid factor concentration directly correlated with CRP (r = 0.318; p < 0.01) and ESR (r = 0.228; p = 0.04) levels. However, no associations were found between RF levels and SLEDAI-2K, joint involvement frequency, SLICC/ACR DI or drug therapy content. Univariate logistic regression analysis showed that RF positivity was independently associated with lymphadenopathy, presence of anti-Ro/SSA and anti-La/SSB antibodies, and negatively associated with kidney involvement. Conclusions: In RF-seropositive SLE patients (approximately 28%), the diagnosis is established later compared to RF-seronegative ones; kidney involvement and fever are less common, while lymphadenopathy develops more frequently. Rheumatoid factor seropositivity is associated with higher levels of ESR, CRP, ANA, and the presence of antibodies to Ro/SSA and La/SSB. According to the results of univariate logistic regression analysis, an independent association with RF positivity was confirmed only for kidney involvement, lymphadenopathy, and antibodies to Ro/SSA and La/SSB. [ABSTRACT FROM AUTHOR]

Background/Aim: Craniopharyngiomas pose challenges in diagnosis and management due to their rare occurrence and diverse clinical manifestations. This study aimed to provide a comprehensive analysis of craniopharyngioma, including its epidemiological trends, clinical presentations, radiological characteristics, surgical interventions, and the role of radiotherapy. Patients and Methods: A retrospective observational study was conducted on 23 patients diagnosed with craniopharyngioma at our hospital from August 2017 to July 2019. Data regarding demographics, clinical presentation, radiological findings, surgical interventions, and adjuvant therapies were collected and analyzed. Results: Craniopharyngiomas exhibited a bimodal age distribution, with peaks in childhood and late adulthood. Clinical presentations varied between pediatric and adult patients, with headache and nausea/vomiting predominant in children, and visual disturbances and hypogonadism more common in adults. Radiological imaging revealed predominantly suprasellar localization and varying tumor consistency. Surgical resection was the primary treatment modality, with post-operative complications including diabetes insipidus and cerebrospinal fluid leak. Histological analysis showed distinct subtypes, with the adamantinomatous subtype predominant in children and the papillary subtype in adults. Adjuvant radiotherapy was administered in cases of incomplete resection or tumor recurrence. Conclusion: This study provides comprehensive insights into the epidemiology, clinical characteristics, radiological features, surgical interventions, and role of radiotherapy in craniopharyngioma management. Understanding these aspects is crucial for tailoring optimal treatment strategies and improving patient outcomes in this complex clinical scenario. [ABSTRACT FROM AUTHOR]

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Background: Invasive meningococcal disease (IMD) cases declined upon the implementation of non-pharmaceutical interventions (NPI) (social distancing and mask wearing) to control the COVID-19 pandemic but rebounded in 2022 in numbers with genotypical changes of the strains. We explored here associated modifications in the clinical presentations of IMD. Methods: We conducted a retrospective descriptive study using the Database of the French National Reference Centre for meningococci and Haemophilus influnezae for IMD cases between 2015 and 2022. We scored serogroups, sex, age groups, clinical presentations and clonal complexes of the corresponding patients and isolates. Findings: Non-meningeal forms of IMD increased significantly upon easing of NPI, such as bacteremic meningococcal pneumonia and bacteremic abdominal forms. They represented 6% and 8% of all IMD forms and were significantly linked to serogroups Y and W respectively, to older adults for bacteremic pneumonia and to young adults for bacteremic abdominal presentations. These forms were significantly associated with more early mortality and clonal complexes 23, 11 and 9316. Interpretation: The increase in atypical IMD forms may lead to higher burden of IMD due to delayed diagnosis and management. Updating prevention may be needed through by adapting the current vaccination strategies to epidemiological changes. [ABSTRACT FROM AUTHOR]

IntroductionExternal injuries in elasmobranchs are frequent findings, either due to inter- or intraspecific interactions or as a result of interaction with human activities. However, the resilience of these species to traumatic injury remains poorly understood. This work provides an insight into the clinical presentation, diagnostic imaging, and pathological features of a severe traumatic injury to the cartilaginous skeleton of a spiny butterfly ray (Gymnura altavela).MethodsAn adult female was found lethargic in the bottom of the coast of Gran Canaria, with an external incised-contused traumatic lesion of 2 cm diameter in the scapulocoracoid cartilage. It was captured and transferred to the Poema del Mar Aquarium for its clinical evaluation and treatment. Despite these efforts, the animal eventually died and was transfer to the Institute of Animal Health and Food Safety (IUSA) for its pathological diagnosis, including a Computed Tomography (CT) study and necropsy.ResultsThe animal presented a marked reduction in hematocrit and hepatosomatic index due a chronic debilitation process. The CT scan revealed a destructive lesion with irregular margins at the level of the right scapulocoracoid cartilage. The main pathological findings were the disorganization of the tesserae layer, appearing as whitish square to rectangular geometric pieces separated from the cartilaginous core. Histologically, these pieces of tesserae were separated from the unmineralized cartilage core and displaced from the adjacent perichondrium, where inflammatory cells infiltrate. Edema and hemorrhages were also observed.ConclusionsThis study reports the first comprehensive description of skeleton trauma in a spiny butterfly ray, including the clinical presentation, diagnostic imaging and the anatomopathological features.

IntroductionPatients with spontaneous intracranial hypotension (SIH) frequently present with several symptoms and impaired mental health. This study systematically investigates the somatic and mental symptom burden of SIH and the effect of surgical treatment.MethodsIn this single-center retrospective study, patients with surgical closure of a spinal cerebrospinal fluid leak between September 2022 and July 2023 completed the Somatic Symptom Scale (SSS-8), the Somatic Symptom Disorder – B Criteria Scale (SSD-12), and the Patient Health Questionnaire (PHQ-8) preoperatively and three and 6 months postoperatively.ResultsFifty-seven patients were included. All three scores showed clearly pathological values before surgery (SSS-8: 12 [IQR 6.5–16], SSD-12: 26 [IQR 19.5–33.5], PHQ-8: 11 [IQR 6.5–15]) representing a high somatic symptom burden and relevant current depression. After surgery, there was a significant and sustainable improvement (SSS-8: 8 [IQR 3–11.75], SSD-12: 12.5 [IQR 5–21.75], PHQ-8: 4.5 [IQR 2–9], p

Niemann Pick Type C disease is a rare and progressive neurodegenerative lysosomal storage disorder caused by autosomal recessive mutations in the NPC1 and NPC2 genes. It is characterized by the accumulation of multiple lipid species in the endolysosomal compartment, leading to neurodegeneration and involvement of the liver, spleen, and lungs. Niemann Pick Type C has a wide range of presentations and severities at different ages with different progression rates. According to the Human Gene Mutation Database, to date, 486 disease-causing mutations in the highly polymorphic NPC1 gene and >20 mutations in the NPC2 have been reported. In the present study, we described the clinical, biochemical, and molecular profiles of 18 Iranian patients with Niemann-Pick Type C disease. Also, we describe six novel variants of the NPC1 gene, to our knowledge, not reported to date.

Introduction: The ovaries, fallopian tubes, and the broad ligament make up the anatomical adnexa. Adnexal masses represent a commonly occurring issue in gynaecology. The majority of ovarian malignancies are detected at advanced stages. The clinical manifestations of adnexal masses vary, with many cancers either showing no symptoms or presenting with vague signs. In the reproductive age group, the most typical manifestation is a benign functional cyst. Aim: To conduct a clinico-pathological analysis of adnexal masses among women presenting at a tertiary healthcare centre. Materials and Methods: A retrospective observational study was conducted in the Department of Obstetrics and Gynaecology at Akash Institute of Medical Science and Research Centre, Devanahalli, Karnataka, India, from November 2022 to April 2024. The diagnosis of adnexal mass was based on clinical symptoms, abdominal examination, bimanual examination, and trans-abdominal ultrasound. The CA-125 and other tumour markers were sent depending on the risk of malignancy. All tissues were sent for histopathological examination. The data were collected and tabulated in a Microsoft excel sheet, and percentages were calculated. Results: This study included 38 cases, in which benign ovarian and para-ovarian cysts were the most common findings, observed in 21 cases (55.3%). There were six cases of benign serous cystadenoma (15.8%), three cases of teratoma (7.9%), two cases of high-grade serous carcinoma (5.3%), one case of germ cell tumour (2.6%), one case of mucinous cystadenoma (2.6%), two cases of endometriotic cysts (5.3%), one case of adnexal cystic degeneration of a fibroid (2.6%), and one rare case of primary fallopian tube carcinoma (2.6%). The most common presenting complaint was abdominal pain, reported in 21 cases (55.3%). Conclusion: Adnexal masses can present in a variety of forms, ranging from benign cysts to infections and cancers. A thorough history and clinical examination are essential for accurate diagnosis. Investigations support the diagnosis, and histology confirms it. [ABSTRACT FROM AUTHOR]

Background: Pancreatic neuroendocrine tumors (P-NETs) constitute a subset of pancreatic mass lesions characterized by diverse clinical presentations. Despite their inherent malignant potential, the timely identification and treatment of these tumors are critical for achieving favorable clinical outcomes. This study aims to shed light on the heterogeneous tumor biology of P-NETs and the management strategies employed at a tertiary care center in Pakistan.Method: A retrospective study encompassing all patients with a biopsy-confirmed diagnosis of P-NETs at Shifa International Hospital between January 1st, 2016, and June 30th, 2021, was conducted. Meticulous data extraction from pathology records and thorough searches of medical records were performed to gather relevant demographic and clinical information.Results: A total of 24 patients were retrieved from our database, with 13 (54%) female patients. The mean age was 49.5 ± 16.3 years. Eight out of the 24 patients presented with abdominal pain. Most patients (14 out of 24) had lesions in the pancreatic head region. In three cases, lesions exhibited multicentricity. The mean lesion size measured 4.4 ± 2.3 cm. Three of the 24 patients displayed distant liver metastasis at the presentation time. 19 out of the 24 patients underwent surgical resections, while endoscopic ultrasound (EUS)-guided biopsy was performed in 4 out of 24 cases. EUS-guided tissue biopsy yielded accurate diagnoses in all four cases.Conclusion: Most P-NETs are non-functional, and there is an almost equal distribution between male and female patients. Solitary lesions predominate, and metastasis is uncommon at initial presentation. EUS-guided fine needle biopsy stands out as a dependable diagnostic modality for P-NETs.

Abstract About four years have passed since the detection of the first cases of COVID-19 in China. During this lethal pandemic, millions of people have lost their lives around the world. Since the first waves of COVID-19 infection, various pharmacotherapeutic agents have been examined in the management of COVID-19. Despite all these efforts in pharmacotherapy, drug repurposing, and design and development of new drugs, multiple organ involvement and various complications occurred during COVID-19. Some of these complications became chronic and long-lasting which led to the “long COVID” syndrome appearance. Therefore, the best way to eradicate this pandemic is prophylaxis through mass vaccination. In this regard, various vaccine platforms including inactivated vaccines, nucleic acid-based vaccines (mRNA and DNA vaccines), adenovirus-vectored vaccines, and protein-based subunit vaccines have been designed and developed to prevent or reduce COVID-19 infection, hospitalization, and mortality rates. In this focused review, at first, the most commonly reported clinical presentations of COVID-19 during these four years have been summarized. In addition, different therapeutic regimens and their latest status in COVID-19 management have been listed. Furthermore, the “long COVID” and related signs, symptoms, and complications have been mentioned. At the end, the effectiveness of available COVID-19 vaccines with different platforms against early SARS-CoV-2 variants and currently circulating variants of interest (VOI) and the necessity of booster vaccine shots have been summarized and discussed in more detail.

Abstract Background The ‘diagnostic odyssey’ is a common challenge faced by patients living with rare diseases and poses a significant burden for patients, their families and carers, and the healthcare system. The diagnosis of rare diseases in clinical settings is challenging, with patients typically experiencing a multitude of unnecessary tests and procedures. To improve diagnosis of rare disease, clinicians require evidence-based guidance on when their patient may be presenting with a rare disease. This study aims to identify common experiences amongst patients with rare diseases, to inform a series of ‘red flags’ that can aid diagnosis of rare diseases in non-specialist settings. A questionnaire was developed by Medics for Rare Diseases, informed by the experiences of clinicians, rare disease patients and patient advocates, and was shared with UK-based rare disease patient groups. Study participants were engaged via social media platforms, blogs and email newsletters of three umbrella rare disease organisations. The questionnaire, comprising 22 questions, was designed to identify typical experiences relating to physical and psychosocial manifestations and presentation of disease, patient interactions with healthcare providers, and family history. Results Questionnaire responses were received from 79 different rare disease patient groups and the common experiences identified were used to inform seven red flags of rare disease: multi-system involvement (3 or more); genetic inheritance pattern; continued presentation throughout childhood and adulthood; difficulties at school, especially relating to absences, difficulty participating in physical education and experiences of bullying or social isolation; multiple specialist referrals; extended period with unexplained symptoms; and misdiagnosis. In light of the red flags identified, recommendations for primary care and education settings have been proposed, focusing on the need for holistic assessment and awareness of both physical and psychosocial factors. Conclusions This study identified key commonalities experienced by patients with rare disease across physical and psychosocial domains, in addition to understanding patients’ history and experiences with healthcare providers. These findings could be used to develop a clinical decision‑making tool to support non-specialist practitioners to consider when their patient may have an undiagnosed rare condition, which may minimise the challenges of the ‘diagnostic odyssey’ and improve the patient experience.

Joseph F Merola,1,&ast; Ahmad Z Amin2,&ast; 1Brigham and Women’s Hospital, Boston, Massachusetts, USA; 2Northwestern University Feinberg, School of Medicine, Chicago, Illinois, USA&ast;These authors contributed equally to this workCorrespondence: Dr Joseph F Merola, Email jfmerola@bwh.harvard.eduAbstract: Generalized pustular psoriasis (GPP) is the most severe form of pustular psoriasis and affects large areas of the body. GPP is a rare disease, and has a variable presentation; thus, its diagnosis is challenging. The onset of symptoms is rapid, with the appearance of painful skin erythema, followed by the widespread eruption of sterile pustules. Acute GPP (called a flare) is often accompanied by systemic symptoms, including high fever, pain in skin lesions, malaise, and fatigue. Approximately half of GPP flares require hospitalization, with an average inpatient duration of 10– 14 days. GPP prevalence estimates range from approximately 2– 124 cases per million persons, with a female predominance. The most common age of onset of GPP is 40– 60 years, although cases have been described in younger adults and children. GPP affects every aspect of patients’ lives and has a high physical and psycho-social impact. Recent research on the interleukin-36 pathway associated with GPP led to the development of a GPP-specific treatment, spesolimab, which was approved by the US FDA in September 2022. This podcast explores the clinical presentation, disease course, and burden of disease in GPP, including differential diagnosis and common triggers of an acute flare.Keywords: generalized pustular psoriasis, interleukin-36 pathway, clinical presentation, disease burden, diagnosis

Background: Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular condition characterized by the formation of blood clots in the cerebral venous sinuses, which are responsible for draining blood and cerebrospinal fluid from the brain. CVST represents a significant cause of stroke in young individuals, with a mortality rate ranging from 6% to 15%. Methods: Common clinical presentations include symptoms like headaches, seizures, altered mental state, and focal neurological deficits. In India, a noteworthy occurrence of CVST is observed among postpartum women, while alcoholism poses a significant risk factor for males. Results: This study identifies headaches as the most prevalent initial symptom of CVST, followed by seizures and focal neurological deficits. The superior sagittal sinus is the most frequently affected in these patients. Notably, 83.3% of patients in this study achieved a favorable outcome. However, a midline shift exceeding 10mm was identified as a predictive factor for an unfavorable outcome in this series. Conclusions: Contrary to previous perceptions, CVST is not uncommon in males. The early diagnosis and prompt intervention have a positive impact on overall patient outcomes. This research sheds light on the importance of recognizing CVST in a broader demographic, its common symptoms, and the critical role of timely intervention for improved patient prognosis.

Olfactory Nerve Schwannomas (ONS) affect cranial nerve I (CN I), responsible for the sense of smell. This systematic review aims to comprehensively examine the risk factors, etiology, clinical presentation, and management of ONS by analyzing 44 case reports/series published from 1978 to 2022. Our systematic review utilized a comprehensive search strategy across various scientific databases, including PubMed, Scopus, and Web of Science in March 2023. The inclusion and exclusion criteria included minimum information on demographics (i.e., age), clinical presentation, syndrome, area involved, and extent of resection. The selected articles were case reports, so there is no question about bias. Articles were meticulously reviewed for the above information and underwent both qualitative and quantitative analysis. A total of 44 case reports/series, consisting of 45 patients, were included in this study (mean: 38.45). The anterior cranial fossa was the most frequently involved area (48 %). The most common surgical procedure was bifrontal craniotomy (n=16), followed by left frontal craniotomy (n=7). Approximately 95.5 % of patients underwent Gross Total Resection (GTR), while 4.5 % had Subtotal Resection (STR). Radiotherapy was applied to two patients. Olfactory function varied, with 35.5 % preserving it and 28.88 % experiencing damage or loss. Positive outcomes were observed in approximately 66.6 % of cases, with complications reported in 15 % of instances, including cerebrospinal fluid (CSF) rhinorrhea and diplopia. Long-term follow-up and regular screening of patients are imperative. Further research is essential to elucidate the precise pathogenesis and to develop improved treatment modalities for ONS. This review provides a comprehensive overview of the current state of knowledge regarding ONS; however, as the disease is very rare, only case reports are available as a primary study, making it difficult to reach a strong conclusion. [ABSTRACT FROM AUTHOR]

BACKGROUND: Chlorfenapyr is used to kill insects that are resistant to organophosphorus insecticides. Chlorfenapyr poisoning has a high mortality rate and is difficult to treat. This article aims to review the mechanisms, clinical presentations, and treatment strategies for chlorfenapyr poisoning. DATA RESOURCES: We conducted a review of the literature using PubMed, Web of Science, and SpringerLink from their beginnings to the end of October 2023. The inclusion criteria were systematic reviews, clinical guidelines, retrospective studies, and case reports on chlorfenapyr poisoning that focused on its mechanisms, clinical presentations, and treatment strategies. The references in the included studies were also examined to identify additional sources. RESULTS: We included 57 studies in this review. Chlorfenapyr can be degraded into tralopyril, which is more toxic and reduces energy production by inhibiting the conversion of adenosine diphosphate to adenosine triphosphate. High fever and altered mental status are characteristic clinical presentations of chlorfenapyr poisoning. Once it occurs, respiratory failure occurs immediately, ultimately leading to cardiac arrest and death. Chlorfenapyr poisoning is difficult to treat, and there is no specific antidote. CONCLUSION: Chlorfenapyr is a new pyrrole pesticide. Although it has been identified as a moderately toxic pesticide by the World Health Organization (WHO), the mortality rate of poisoned patients is extremely high. There is no specific antidote for chlorfenapyr poisoning. Therefore, based on the literature review, future efforts to explore rapid and effective detoxification methods, reconstitute intracellular oxidative phosphorylation couplings, identify early biomarkers of chlorfenapyr poisoning, and block the conversion of chlorfenapyr to tralopyril may be helpful for emergency physicians in the diagnosis and treatment of this disease. [ABSTRACT FROM AUTHOR]

Introduction: Chronic venous disease is a global public health problem, with high morbidity and economic distress. There is scarcity of data on this disease in sub-Saharan Africa. Methods: We conducted the first population-based study over a period of 20 months from 1st February 2020 to 30th September 2021 in the 10 regions of Cameroon. A stratify sampling method was chose to select study site. Socio-demographic data, personal and family history, anthropometric parameters, clinical signs, illustrative images, CEAP (Clinical-Etiological-Anatomical-Pathophysiological) classification revised in 2004, VCSS (venous Clinical Severity Score) and risk factor assessment score were used to construct the survey form. Chi-squared test and Fischer exact test were used to compare the prevalence of chronic venous disease across different potential risk factors (sex, age category, previous history of deep vein thrombosis, hypertension, diabetes, smoking status, obesity). Simple and multiple logistic regression models were used to obtain crude and adjusted odds ratio for risk factors associated with chronic venous insufficiency. Statistical analyses were done with R version 4.2 for Linux and the threshold for statistical significance was 0.05. Results: A total of 6578 participants were included in the study, with a mean age of 41.09 ± 16.02 years with female predominance (54.3%). The prevalence of chronic venous disease was 21.8% (95% CI: 20.8–22.9) and the prevalence of chronic venous insufficiency (C3–C6) was 7.02% (n = 462). Night cramps (43.2%), oedema (21.7%), lower limbs pain (20.4%) mostly worsens by walking and heavy legs (16.2%) were more common symptoms. The mean total venous clinical severity score was 0.69 ± 1.76 and this score had a significant positive correlation with C classification (p <.001). In the multivariate analysis, the following factors were independently associated with CVD: Male gender (aOR: 1.27; 95%CI: 1.04–1.56; p =.021), retired people (aOR: 46.9; 95% CI: 12.6–174.5; p <.001), hypertension (aOR: 289.5; 95%CI: 169.69–493.1; p <.001), diabetes (aOR: 2.19; 95% CI: 1.21–3.96; p =.009), obesity (aOR: 10.22; 95%CI: 7.67–13.62; p <.001). Smoking appears as a protective factor (aOR: 0.18; 95%CI: 0.10–0.30; p <.001). Conclusion: Chronic venous disease is frequent in Cameroon and main traditional cardiovascular risk factors are associated to this condition. Systematic screening of the CVD in these specific groups could reduce the burden of the disease and its economic impact. [ABSTRACT FROM AUTHOR]

About four years have passed since the detection of the first cases of COVID-19 in China. During this lethal pandemic, millions of people have lost their lives around the world. Since the first waves of COVID-19 infection, various pharmacotherapeutic agents have been examined in the management of COVID-19. Despite all these efforts in pharmacotherapy, drug repurposing, and design and development of new drugs, multiple organ involvement and various complications occurred during COVID-19. Some of these complications became chronic and long-lasting which led to the "long COVID" syndrome appearance. Therefore, the best way to eradicate this pandemic is prophylaxis through mass vaccination. In this regard, various vaccine platforms including inactivated vaccines, nucleic acid-based vaccines (mRNA and DNA vaccines), adenovirus-vectored vaccines, and protein-based subunit vaccines have been designed and developed to prevent or reduce COVID-19 infection, hospitalization, and mortality rates. In this focused review, at first, the most commonly reported clinical presentations of COVID-19 during these four years have been summarized. In addition, different therapeutic regimens and their latest status in COVID-19 management have been listed. Furthermore, the "long COVID" and related signs, symptoms, and complications have been mentioned. At the end, the effectiveness of available COVID-19 vaccines with different platforms against early SARS-CoV-2 variants and currently circulating variants of interest (VOI) and the necessity of booster vaccine shots have been summarized and discussed in more detail. [ABSTRACT FROM AUTHOR]

Introduction: Oral cancer is a global health challenge. In India, there is a huge burden on the healthcare system. The prevalence of oral cancer globally and in India, coupled with the specific risk factors associated with the Indian population, necessitates targeted research to improve early detection and treatment outcomes. The present study analysed 93 patients with oral cancer over 5 years to determine the incidence corresponding to different stages of the disease. Materials and Methods: This retrospective cross-sectional study reviewed medical records from the hospital database. The epidemiological data included age, gender, tobacco habit, duration of tobacco use, site of the lesions, stages of cancer and the treatment provided. We analysed the relationship of epidemiological characteristics with age by the Chi-square test. The statistical test significance level was set at P < 0.05. Results: The incidences of oral cancer each year range from 8% to 12%. A male patient over 40 years of age was most affected by oral cancer 42 (45.16%). The most affected sites were buccal mucosa 36 (38.70%) and alveolus 33 (35.48%) amongst patients over 40 years of age. The predominant stage for the majority of cancer cases was Stage IV, accounting for 49 cases (52.68%). Additionally, the largest lesions, exceeding 4 cm in size, were observed in 64 cases (68.81%), and these instances were predominantly found in patients aged 40 years or older. Chewing tobacco emerged as a significant contributing factor to cancer, affecting 41 cases (42.08%), particularly among those with a habit duration exceeding 10 years, representing 48 cases (51.61%) in patients aged 40 years or older. Conclusion: There is an urgent need to spread awareness of oral cancer, followed by extensive screening in India. Tobacco in India is not only used widely but also associated with cultural relevance, and it is the need of the hour to implement the tobacco policy to reach out evenly to all communities. [ABSTRACT FROM AUTHOR]

Background: Pancreatic neuroendocrine tumors (P-NETs) constitute a subset of pancreatic mass lesions characterized by diverse clinical presentations. Despite their inherent malignant potential, the timely identification and treatment of these tumors are critical for achieving favorable clinical outcomes. This study aims to shed light on the heterogeneous tumor biology of P-NETs and the management strategies employed at a tertiary care center in Pakistan. Method: A retrospective study encompassing all patients with a biopsy-confirmed diagnosis of P-NETs at Shifa International Hospital between January 1st, 2016, and June 30th, 2021, was conducted. Meticulous data extraction from pathology records and thorough searches of medical records were performed to gather relevant demographic and clinical information. Results: A total of 24 patients were retrieved from our database, with 13 (54%) female patients. The mean age was 49.5 ± 16.3 years. Eight out of the 24 patients presented with abdominal pain. Most patients (14 out of 24) had lesions in the pancreatic head region. In three cases, lesions exhibited multicentricity. The mean lesion size measured 4.4 ± 2.3 cm. Three of the 24 patients displayed distant liver metastasis at the presentation time. 19 out of the 24 patients underwent surgical resections, while endoscopic ultrasound (EUS)-guided biopsy was performed in 4 out of 24 cases. EUS-guided tissue biopsy yielded accurate diagnoses in all four cases. Conclusion: Most P-NETs are non-functional, and there is an almost equal distribution between male and female patients. Solitary lesions predominate, and metastasis is uncommon at initial presentation. EUS-guided fine needle biopsy stands out as a dependable diagnostic modality for P-NETs. [ABSTRACT FROM AUTHOR]

Background: Pneumocytis jirovecii pneumonia (PJP) has high mortality rates in immunocompromised children, even though routine prophylaxis has decreased in incidence. The aim of this case series is to present the radiological and clinical pathway of PJP in a pediatric population. Description of Cases: All PJP cases in non-HIV/AIDS patients diagnosed at Istituto Giannina Gaslini Pediatric Hospital in Genoa (Italy) from January 2012 until October 2022 were retrospectively evaluated. Nine cases were identified (median age: 8.3 years), and of these, 6/9 underwent prophylaxis with trimethoprim/sulfamethoxazole (TMP/SMX; five once-a-week schedules and one three times-a-week schedule), while 3/9 did not receive this. PJP was diagnosed by real-time PCR for P. jirovecii-DNA in respiratory specimens in 7/9 cases and two consecutive positive detections of β-d-glucan (BDG) in the serum in 2/9 cases. Most patients (6/8) had a CT scan with features suggestive of PJP, while one patient did not undergo a scan. All patients were treated with TMP/SMX after a median time from symptoms onset of 3 days. In 7/9 cases, empirical TMP/SMX treatment was initiated after clinical suspicion and radiological evidence and later confirmed by microbiological data. Clinical improvement with the resolution of respiratory failure and 30-day survival included 100% of the study population. Discussion: Due to the difficulty in obtaining biopsy specimens, PJP diagnosis is usually considered probable in most cases. Moreover, the severity of the clinical presentation often leads physicians to start TMP/SMX treatment empirically. BDG proved to be a useful tool for diagnosis, and CT showed good accuracy in identifying typical patterns. In our center, single-day/week prophylaxis was ineffective in high-risk patients; the three-day/week schedule would, therefore, seem preferable and, in any case, should be started promptly in all patients who have an indication of pneumonia. [ABSTRACT FROM AUTHOR]

Purpose: To investigate sex-related differences in the clinical presentation of multiple system atrophy (MSA) through a literature review and an analysis of a retrospective cohort. Methods: The PubMed database was searched for articles including sex-related information in MSA. In a retrospective Innsbruck cohort, we investigated the baseline to last available follow-up clinical-demographic differences between men and women with MSA in a univariate fashion, followed by multivariable binary regression analysis. Results: The literature search yielded 46 publications with sex-related information in MSA. Most studies found comparable survival rates between the sexes, while some recent reports suggested a potential survival benefit for women, possibly due to initial motor onset and overall less severe autonomic failure compared to men. The retrospective Innsbruck MSA cohort comprised 56 female and 60 male individuals with a comparable median follow-up of 27 months. At baseline, female sex was independently associated with depression (odds ratio [OR] 4.7; p = 0.007) and male sex with severe orthostatic hypotension (OR 5.5; p = 0.016). In addition, at last follow-up, female sex was associated with the intake of central nervous system-active drugs (OR 4.1; p = 0.029), whereas male sex was associated with the presence of supine hypertension (OR 3.0; p = 0.020) and the intake of antihypertensive medications (OR 8.7; p = 0.001). Male sex was also associated with initiation of antihypertensive medications over the observation period (OR 12.4; p = 0.004). Conclusion: The available literature and findings of the present study indicate sex-related differences in the clinical presentation of MSA and its evolution over time, highlighting the importance of considering sex in symptom exploration, therapeutic decision-making, and future clinical trial design. [ABSTRACT FROM AUTHOR]