Exclusive Involvement Research Articles

Real-World Evaluation of the Effectiveness and Safety of Dupilumab in Bullous Pemphigoid: An Ambispective Multicenter Case Series.

Bullous pemphigoid affects elderly individuals with multiple comorbidities, making conventional treatments unsuitable. Evaluate the effectiveness and safety of dupilumab in the treatment of bullous pemphigoid. A multicenter ambispective cohort study was conducted in 34 hospitals. Patients with bullous pemphigoid treated with Dupilumab were included. Most of patients (97.1%) received an initial 600 mg dose followed by 300 mg every two weeks. The primary outcome was the proportion of patients achieving complete remission within 4 weeks, defined as Investigator Global Assessment score of 0 or 1. Complete remission at weeks 16, 24, and 52, adverse events, reductions in peak pruritus numerical rating scale, and systemic glucocorticoid use were also assessed. The study included 103 patients with a median age of 77.3 years, 58.0% male. Complete remission was achieved by 53.4% within 4 weeks and 95.7% by week 52. Peak pruritus scale reduced by 70.0% by week 4 and was completely controlled by week 24. Thirteen patients presented adverse events, most of which were mild. Systemic glucocorticoid use reduced by 82.1% by week 52. Shorter disease duration and exclusive cutaneous involvement predicted better response at 16 weeks. No differences in response rates to dupilumab were observed between drug-associated bullous pemphigoid and idiopathic cases. No significant difference in response rates was observed between patients treated with dupilumab in monotherapy and those receiving dupilumab with concomitant treatments. Dupilumab is effective, rapid, and safe in managing bullous pemphigoid, reducing the need for corticosteroids and other treatments. Early initiation and exclusive skin involvement predict better outcomes.

Percheron Artery Stroke and Reperfusive therapies: A systematic review and meta-analysis

Background and AimsPercheron Artery Stroke, affecting bilateral paramedian thalamic structures, accounts for 0.1–0.4 % of all acute ischemic strokes. Little is known about disability or mortality outcomes in patients receiving intravenous thrombolysis (IVT) or endovascular treatment (EVT), therefore a systematic review was carried out. MethodsA systematic search of Pubmed, Embase and Scopus databases for all kind of studies regarding Percheron Artery Stroke was performed. The review is registered in INPLASY (202410059). In total, 1895 papers were screened; 197 studies were included, consisting exclusively in case reports, retrospective series and conference abstracts. Data from 448 patients were analyzed in logistic regression. ResultsBoth IVT (ORIVT = 3.08; 95 % CI 1.4 – 6.7; p = 0.005) and EVT (OREVT = 5.77; 95 % CI 1.69 – 19.7; p = 0.005) were associated with reduced disability, as well as exclusive thalamic involvement (OR = 3.24; CI 2.1 – 5.1; p < 0.001) and higher Glasgow Coma Scale score (GCS) on admission (OR = 1.2; 95 % CI 1.12 – 1.28; p < 0.001). GCS (OR = 0.73, 95 % CI 0.63 – 0.82; p < 0.001) and exclusive thalamic involvement (OR = 0.36; 95 % CI 0.16 – 0.74; p = 0.005) were associated with lesser mortality, while IVT displayed a non-significant trend towards reduced mortality (ORIVT = 0.13; 95 % CI 0.01 – 1.04; p = 0.056). ConclusionThe present work, albeit summarizing data coming from low quality sources, suggests an association between both EVT/IVT and better outcomes in paramedian bilateral thalamic strokes.

Effect of Ni/Co ratio on Ce-Sc-ZrO2 catalysts for selective H2 production via methane partial oxidation

Partial oxidation of methane (POM) is a promising route for hydrogen production, and achieving a high H2 yield with an H2/CO ratio >3 is highly appealing. Optimization of Ni/Co ratios over Ce-Sc-ZrO2 (CSZ) is investigated for POM reaction and characterized by X-ray diffraction, Raman spectroscopy, temperature-programmed reduction/oxidation/desorption, and transmission electron microscopy. The active site derived from the reduction of “strongly interacted NiO” is responsible for the dissociation of C–H (of CH4), resulting high activity towards POM. 5Ni/CSZ has the highest amount of such active sites and attains the highest activity. 5Co/CSZ catalyst has cobalt-based active sites, and there is an inert carbon deposit during the reaction, causing the least activity. 3.75 wt% Ni and 1.25 wt% Co combination over CSZ support surges the highest density of basicity, oxide vacancy, and adequate amount of active sites derived from “strongly interacted NiO”. The active sites with enhanced metal-support interaction are further grown under exposure to oxidizing gas (O2) and reducing gas (H2) during the POM reaction. The highest density of basicity and oxide vacancy involves more CO2 and H2O in the sequential oxidation of CH4 under indirect pathways. The exclusive involvement of indirect pathways of POM and inhibition of hydrogen consuming reaction (like reverse water gas shift reaction) over 3.75Ni1.25Co/CSZ results into 48 % H2 yield and 3.26 H2/CO ratio up to 24 h time on stream at 600 °C. The H2 yield doubles to ∼97 %, and the H2/CO ratio comes close to 2 over 3.75Ni1.25Co/CSZ catalyst at 900 °C.

Hand eczema in Italian patients referred for patch testing: A retrospective SIDAPA study (2016-2023).

Hand eczema (HE) is a common skin disease with a negative impact on patients' quality of life in occupational and non-occupational settings. Up-to-date, data on HE in Italian patients referred for patch testing are lacking. To characterise the profile in terms of demographics, aetiology and patch test results of Italian patients affected by HE referred for patch testing. A retrospective descriptive study on consecutive patients affected by HE who underwent patch testing from 2016 to 2023 in eight dermatology clinics was performed. HE patients were divided into two groups according to the exclusive (HE-only group) and not-exclusive (HE+ group) hand involvement, and compared to patients with eczema localised in body areas other than hands (NHE group). One thousand five hundred and ninety-seven patients were affected by HE (35.3% males; mean age 42.7 years), 60.2% belonging to the HE-only group and 39.8% to the HE+ group. Occupational dermatitis was diagnosed in 33.2% of HE-only patients, 25.0% of HE+ patients and 5.2% of NHE patients (p < 0.001). HE-only patients presented: Allergic Contact Dermatitis (ACD), Irritant Contact Dermatitis (ICD), atopic HE in 48.1%, 47.5% and 7.1%, respectively; hyperkeratotic palmar, acute recurrent vesicular and nummular clinical subtypes in 52.2%, 43.9% and 11.9%, respectively; relevant positive patch test reactions in 48.1% (nickel sulphate 18.9%, methylchloroisothiazolinone/methylisothiazolinone 10.6%, methylisothiazolinone 8.6%, p-phenylenediamine 6.0% and potassium dichromate 4.7%). HE patients, and particularly those with exclusive hand involvement, show a particular profile in terms of demographic and clinical characteristics, etiologies and relevant positive patch test reactions.

A Retrospective Study of the Histopathological Spectrum of Erythema Nodosum Leprosum.

There are no recent studies with a focus on the histopathology of erythema nodosum leprosum (ENL). To describe the histopathological spectrum of ENL. Digital records from the pathology department were searched, and 125 slides were included. The histopathologic findings were recorded using a pre-designed proforma. Several patterns were noted with the most common being a superficial and deep, perivascular and peri-appendageal, well-circumscribed dermal infiltrate that was seen in 70 (56.0%) biopsies. Other dermal patterns included a similar but loose infiltrate in 19 (15.2%) biopsies, diffuse dermal involvement in 9 (7.2%), top-heavy in 9 (7.2%), and bottom-heavy infiltrates in 12 (9.6%). Subcutaneous tissue was included in 107 biopsies. Extension of dermal infiltrates to the subcutis was noted in 71 (66.4%) biopsies and predominant involvement of the subcutis was noted in 6 (4.8%) biopsies, with lobular involvement in 60 (56.1%), septal involvement in 3 (2.8%), and septo-lobular involvement in 14 (13.1%). In 30 (28.0%) biopsies, the subcutaneous fat was uninvolved. The infiltrates contained neutrophils and foamy histiocytes in variable proportions, along with lymphocytes and plasma cells. Eosinophils were noted occasionally. Medium and/or small vessel vasculitis was noted in 11 (8.8%) biopsies. Fite-Faraco staining was available for 112 biopsies and revealed mainly fragmented and granular acid-fast bacilli (AFB) in 29 (25%) biopsies. Our study had a retrospective design; we could not compare the lesional age and clinical characteristics of patients with the histological features. ENL is characterized by dermal infiltrates composed of foamy histiocytes and neutrophils in varying proportions arrayed in different dermal patterns. Extension of dermal infiltrates into the subcutis was frequent but absent in some. Predominant or exclusive involvement of the subcutis was rare. Vasculitis was noted in a small minority, while AFB were demonstrated in about a quarter of cases.

The Effect of Hemithyroidectomy in Papillary Thyroid Carcinoma with an Exclusive Involvement of the Recurrent Laryngeal Nerve: A Retrospective Study with a Propensity Score-Matched Analysis.

Involvement of the recurrent laryngeal nerve (RLN) in papillary thyroid carcinoma (PTC) is an important prognostic factor and is associated with a higher risk of recurrence. This study aimed to retrospectively analyze the outcomes of patients treated with hemithyroidectomy (HT) in PTC patients with an exclusive RLN invasion who could not tolerate staged surgery, did not wish to undergo another operation, or had other reasons. A retrospective review was conducted on 163 patients with PTC and exclusive RLN involvement at our institution between 2013 and 2019. Patients were divided into a total thyroidectomy (TT) group and HT group. The clinicopathologic factors and prognostic outcomes were compared between the two groups. A propensity score-matched analysis was carried out to reduce selection bias, with the following covariates: gender, age, tumor size, multifocality, central lymph node metastasis (CLNM), and RLN resection. The Kaplan-Meier method was used for a comparison of recurrence outcomes. In the baseline data of the 163 PTC patients, tumor size (p < 0.001), multifocality (p = 0.011), CLNM (p < 0.001), and RLN resection (p < 0.008) in the TT and HT groups differed significantly, whereas age and gender did not differ between the two groups. The TT group reported significantly higher temporary and permanent hypoparathyroidism than the HT group (p < 0.001 and p = 0.042, respectively). With 72-month median follow-up, 11 (6.7%) patients developed recurrence. After propensity score matching, 24 patients with HT and 43 patients with TT were included. Recurrence-free survival (RFS) in the matched samples showed no difference between the TT and HT groups (p = 0.092). Our results indicate that HT may be a feasible treatment for PTC patients with exclusive RLN involvement in specific circumstances without significantly increasing the risk of recurrence. Performing a thorough preoperative examination is crucial to exclude multifocal tumors and lymph node metastasis before undergoing HT.

The value of axillary, facial, occipital, subclavian and common carotid arteries ultrasound in the diagnosis of giant cell arteritis

Abstract Objective To assess the diagnostic value for GCA in adding the axillary arteries (AX) to the temporal artery (TA) ultrasound, particularly in patients with a cranial phenotype of the disease; and to investigate the utility of facial (FA), occipital (OC), subclavian (SC) and common carotid (CC) ultrasound in patients with suspected GCA. Methods Patients with new-onset GCA and a positive ultrasound of the TA, AX, FA, OC, SC or CC, followed at the rheumatology departments of two academic centres, were retrospectively included. Results Two hundred and thirty patients were assessed. TA halo sign was identified in 206/230 (89.6%) cases, FA in 40/82 (48.8%), OC in 17/69 (24.6%), AX in 56/230 (24.3%), SC in 31/57 (54.4%) and CC in 14/68 (20.6%). Negative TA ultrasound was found in 24/230 (10.4%) patients: 22 had AX involvement, one exclusive OC involvement and one exclusive SC involvement. Adding AX evaluation to the TA ultrasound increased the diagnostic yield for GCA by 9.6%, whereas adding OC or SCs to the TA and AX ultrasound increased it by 1.4% and 1.8%, respectively. No value was found in adding the FA or CCs. Notably, 13 patients with cranial symptoms and four with exclusively cranial symptoms showed negative TA ultrasound but positive AX ultrasound. Conclusion Adding the evaluation of AXs to the TA ultrasound increased the number of patients diagnosed with GCA, even in cases of predominantly cranial symptoms. In the subset of patients where these arteries were assessed, no substantial benefit was found in adding the FA, OC, SC or CC arteries to the TA and AX ultrasonographic assessment.

KHRONOS Project: Is time to surgery really important in the treatment of early breast cancer?

e12598 Background: Recent studies suggest that delaying surgery in early breast cancer (BC) longer than 8 weeks(w) has a worse overall survival compared to those who undergo surgery between 0-4 w. Because BC has developmental heterogeneity over time, the aim of our study is to demonstrate that time from diagnosis to primary surgery is not the most important factor and focus on the value of biology in BC development. Methods: Descriptive, observational and retrospective study in our centre with patients (pts) diagnosed with early stage BC from 2015-2022 without radiological lymph node involvement, whose first therapeutic strategy was surgery. We used chi-square test to analyse the association between categorical variables using SPSS statistical software. Results: 927 pts were included, of which 75.3% (696) maintained pre-surgical staging (N0), while 24.7% (228) changed from N0 to N+ after surgery, with 157 cases having exclusive sentinel lymph node involvement. 98 pts (11.1%) underwent axillary emptying. The histological characteristics were 789 cases (84.9%) ductal while 126 cases (13.6%) were lobular carcinoma. Tumor grades were distributed as follows: G1 (296 cases), G2 (419 cases), and G3 (205 cases). Hormone receptor expression was ER+ (51 cases), ++ (45), +++ (755), and PR + (77), ++ (134), +++ (537). HER2 expression in 516 cases were negative, HER2+ (195), ++ (151), +++ or FISH+ (56). Regarding surgery, 71.8% underwent breast conservative surgery while 28.2% underwent mastectomy. 583 pts were operated below 8w (62.9%), and in 344 (37.1%) time to surgery were &gt; 8w. 583 pts were operated &lt;8w (62.9%), 134 transitioned from N0 to N+ post-surgery, while 448 maintained negative nodal after surgery. In 344 (37.1%) time to surgery were &gt; 8w, 94 pts went from radiologically N0 to nodal involvement after surgery; however, 248 maintained negative nodal status. Although there was a tendency for pts who underwent surgery beyond 8 w to have a greater change in nodal involvement, the differences were not statistically significant (p=0.129). When we performed a stratified analysis by subgroups, in terms of tumour grade, we observed that among pts with G3 tumours operated on &gt;8w, 37.8% had gone from N0 to N+ vs. 23.8% of the group operated &lt;8w, with statistically significant differences, p=0.034. No statistically significant differences were found stratifying by hormone receptors, HER2 or Ki67. Conclusions: In our study we do not find that delay from diagnosis to surgery in BC ≥ 8w worsens lymph node staging. However, we observe that changes in nodal staging occur (transitioning from N0 to N+ in early BC) in poorly differentiated tumors (G3). Therefore, due to BC developmental heterogeneity over time, we believe tumour biology is more important than time to surgery, this may be useful for prioritising pts with these characteristics on the surgical waiting list. Further studies including other biological factors are needed to confirm it.

Impact of thyroid carcinoma invasion of recurrent laryngeal nerve on cervical lymph node metastasis.

Papillary thyroid carcinoma (PTC) has a favorable prognosis. However, involvement of the recurrent laryngeal nerve (RLN) significantly increases the risk of recurrence. RLN invasion was an important factor in determining the extent of thyroid surgery. The purpose of this study was to compare clinicopathologic features and characterize risk factors of central and lateral lymph node metastasis (LLNM) of RLN invasion in patients with PTC. A retrospective review was conducted of 130 patients with PTCs who had exclusive tumor involvement of the RLN at our institution between January 2014 and February 2019. All patients underwent total thyroidectomy and high-dose radioactive iodine (RAI) therapy. The clinicopathologic factors and prognostic outcomes of the patients with solitary and multiple RLN involvements were compared. Kaplan-Meier method was performed to compare the outcomes of tumor recurrence. Univariate and multivariate logistic regression analyses were used to identify risk factors associated with LLNM. The invasion of the RLN was similar on both sides, with 58.5% on the right, 40.0% on the left, and 1.5% on both sides. Significant differences were observed in tumor size (p < 0.001), lymph node metastasis classification (p = 0.002), RLN resection (p < 0.001), and thyroglobulin (p = 0.010) in the solitary and multiple groups. During the median follow-up of 67 months, 9 (6.9%) patients developed recurrence. There were no statistical differences in recurrence for age, tumor size, gender, multifocality, lymph node metastasis (LNM), and RLN resection. According to receiver operating characteristic (ROC) curve analyses, a cut-off of tumor size > 1.7 cm was identified as the most sensitive and specific predictor of RLN with multiple involvements or LNM invasion. Univariate and multivariate analyses revealed that central lymph node metastasis (CLNM) and RLN invasion by LNM can serve as independent risk factors for LLNM (p = 0.006 and p < 0.001, respectively). Our results indicate that recurrence was comparable in patients with solitary and multiple RLN involvements. Multiple RLN involvement was associated with pathological features such as larger tumors, RLN resection, and LLNM. The presence of LNM invading RLN and multiple nerve involvement increases the risk of intraoperative RLN resection. A higher risk of multiple invasion or LNM invasion should be considered when tumor size > 1.7 cm. The presence of CLNM and RLN invaded by LNM were independent predictors for LLNM, which could aid surgeons in deciding on lateral lymph node dissection.

Nodular/Keloidal Scleroderma with No Systemic Involvement-A Case Report and a Review of the Literature.

Nodular or keloidal scleroderma is a rare condition with unclear cause and sporadic mentions in the medical literature. It was first recognized in the 19th century, yet its classification is still debated due to the limited number of reported cases. This rare variant of scleroderma is associated with either progressive systemic sclerosis or localized morphea. Clinically, it presents with asymptomatic nodules or plaques, resembling spontaneous keloid formation, often found on the trunk and proximal extremities. Recent literature reviews show a predominance of women with a mean age of 44 years. Diagnosis relies on clinical and histopathological findings, which usually show overlapping features of both scleroderma and true keloids, secondarily to an excessive fibrosing reaction attributed to collagen formation. We present an unusual case of a 70-year-old female patient who displayed the coexistence of two distinct subtypes of morphea (nodular/keloidal and linear), and exclusive skin involvement, which contrasts with the typical presentation of nodular/keloidal scleroderma, often associated with organ-specific disease. However, recent publications have diverged from previous ones regarding systemic sclerosis, with no systemic involvement reported between 2018 and 2024, which we evaluated in our descriptive literature review. With less than 50 cases reported in total, our case underlines the importance of recognizing this rare disease, ensuring appropriate evaluation, treatment, and follow-up.

Sex-Related Differences in the Phenotype and Course of Inflammatory Bowel Disease: SEXEII Study of ENEIDA

The impact of patient sex on the presentation of inflammatory bowel disease (IBD) has been poorly evaluated. Our aims were to assess potential disparities in IBD phenotype and progression between sexes. We performed an observational multicenter study that included patients with Crohn's disease (CD) or ulcerative colitis from the Spanish Estudio Nacional en Enfermedad Inflamatoria intestinal sobre Determinantes genéticos y Ambientales registry. Data extraction was conducted in July2021. A total of 51,595 patients with IBD were included, 52% were males and 25,947 had CD. The median follow-up period after diagnosis was 9 years in males and 10 years in females. In CD, female sex was an independent risk factor for medium disease onset (age, 17-40 y) (relative risk ratio, 1.45; 95% CI, 1.31-1.62), later disease onset (age, >40 y) (relative risk ratio, 1.55; 95% CI, 1.38-1.73), exclusive colonic involvement (odds ratio, 1.24; 95% CI, 1.14-1.34), inflammatory behavior (odds ratio, 1.14; 95% CI, 1.07-1.21), and extraintestinal manifestations (odds ratio, 1.48; 95% CI, 1.38-1.59). However, female sex was a protective factor for upper gastrointestinal involvement (odds ratio, 0.84; 95% CI, 0.79-0.90), penetrating behavior (odds ratio, 0.76; 95% CI, 0.70-0.82), perianal disease (odds ratio, 0.77; 95% CI, 0.71-0.82), and complications (odds ratio, 0.73; 95% CI, 0.66-0.80). In ulcerative colitis, female sex was an independent risk factor for extraintestinal manifestations (odds ratio, 1.48; 95% CI, 1.26-1.61). However, female sex was an independent protective factor for disease onset from age 40 onward (relative risk ratio, 0.76; 95% CI, 0.66-0.87), left-sided colonic involvement (relative risk ratio, 0.72; 95% CI, 0.67-0.78), extensive colonic involvement (relative risk ratio, 0.59; 95% CI, 0.55-0.64), and abdominal surgery (odds ratio, 0.78; 95% CI, 0.69-0.88). There is sexual dimorphism in IBD. The patient's sex should be taken into account in the clinical management of the disease.

An interesting case of orbital tuberculosis with intracranial extension

Tuberculosis (TB) is a chronic airborne infectious disease caused by Mycobacterium tuberculosis and is one of the causes of morbidity and mortality in the underdeveloped nations. TB most commonly involves the lungs, but it can affect nearly any tissue in the body, including the orbit. Orbital TB is a rare manifestation even in endemic countries and mostly occurs as part of systemic involvement secondary to pulmonary disease. Exclusive extrapulmonary involvement of the orbit with intracranial extension is very rare; diagnostic dilemma, unique management, and the outcome make our case interesting and worthy of reporting.

Mecp2 knock-out astrocytes affect synaptogenesis by interleukin 6 dependent mechanisms

Synaptic abnormalities are a hallmark of several neurological diseases, and clarification of the underlying mechanisms represents a crucial step toward the development of therapeutic strategies. Rett syndrome (RTT) is a rare neurodevelopmental disorder, mainly affecting females, caused by mutations in the X-linked methyl-CpG-binding protein 2 (MECP2) gene, leading to a deep derangement of synaptic connectivity. Although initial studies supported the exclusive involvement of neurons, recent data have highlighted the pivotal contribution of astrocytes in RTT pathogenesis through non-cell autonomous mechanisms. Since astrocytes regulate synapse formation and functionality by releasing multiple molecules, we investigated the influence of soluble factors secreted by Mecp2 knock-out (KO) astrocytes on synapses. We found that Mecp2 deficiency in astrocytes negatively affects their ability to support synaptogenesis by releasing synaptotoxic molecules. Notably, neuronal inputs from a dysfunctional astrocyte-neuron crosstalk lead KO astrocytes to aberrantly express IL-6, and blocking IL-6 activity prevents synaptic alterations.

P1067 Pan-enteric mucosal inflammation in CD patients treated with vedolizumab assessed by capsule endoscopy– interim results of a prospective observational study

Abstract Background Mucosal healing (MH) is a paramount treatment goal in Crohn’s disease (CD). The vast majority of data pertains to MH in the colon and terminal ileum; nonetheless, proximal small-bowel involvement can be detected in up to 50% of CD patients assessed by capsule endoscopy (CE). Data regarding pan-enteric MH among patients with active CD who were treated with vedolizumab (VDZ) are lacking. We aimed to evaluate the efficacy of VDZ for achieving pan-enteric MH using pan-enteric CE. Methods This was a prospective open-label observational study. Patients with CD who have started on VDZ, were included. Patients underwent small-bowel patency-assessment using patency capsule (PC) and were followed by CE (PillCam Crohn’s, Medtronic, USA) before /within 40 days of treatment onset and after 14 and 52 weeks. In patients with exclusive small-bowel involvement, colonic preparation was not performed and colon was not assessed in subsequent CE. Accordingly, Lewis score (LS) and Pan-enteric Pillcam score (PS) were calculated, when available. The primary outcome was pan-enteric MH defined as LS&amp;lt;135 and LS&amp;lt;135 &amp; PS&amp;lt;4 for CE confined to the SB/SB &amp;colon, respectively. The main secondary outcomes were small-bowel MH (LS&amp;lt;135) and colonic-MH (colonic PS&amp;lt;4). Results 57 patients were recruited, 41 patients were enrolled (median age: 28 [23-45] years, male-44%) and 16 patients were excluded (6- retained PC, 1- technical reason, 2 -did not start VDZ, 7-withdrew consent). Of them, six patients (1- retained PC, 1- multiple strictures, 1- lost to follow-up, 3- clinical flare) and eight patients (4- discontinued VDZ, 2- capsule adverse events, 1- lost to follow-up, 1- clinical flare) were dropped-out before week 14 and week 52, respectively. Pan-enteric MH was observed in 7/39 (18%) patients at week 14, and in 7/30 (23%) patients at week 52 (two patients and 11 patients have not yet reached 14/52-week, respectively). We observed higher rates of both small-bowel MH and colonic-MH during follow-up compared to baseline (Figure 1), and it was consistent with significant improvement at week 14 in both LS (900 [225-900] vs. 450 [0-900], p&amp;lt;0.001) and PS (12 [2-18] vs. 6 [0-14], p=0.001) compared to baseline. Improvement was even more prominent at week 52 (LS- 0 [0-300] PS- 2 [2-6]). No cases of retained capsule were observed during follow-up. Conclusion VDZ induces MH in both the small-bowel and the colon among patients with CD, and this effect may persist up to 52 weeks of treatment.

An Unusual Case of Early-onset Ankylosing Spondylitis with Predominant Cervical Spine Involvement

Introduction: Ankylosing spondylitis (AS) is a chronic, inflammatory disease of the axial spine characterized by the involvement of the spine and sacroiliac (SI) joints. Increasing spinal stiffness and persistent back pain are the most typical symptoms of this systemic illness. The most often affected areas in AS are the lumbosacral spine and the sacroiliac joints. Case Report: We report this uncommon case of a 30-year-old male patient recently diagnosed with AS having an exclusive cervical spine involvement corroborated by X-rays and magnetic resonance imaging. Conclusion: The results of the lumbosacral spine, fingers, chest, and sacroiliac joint examinations were normal. The examination of the sacroiliac joint, lumbosacral spine, chest, and digits was normal. The pain and neck stiffness were partially relieved by pharmacotherapy (NSAIDS) and physiotherapy. This is a rare presentation of AS, the reported literature on such case reports are very rare. Keywords: Recently diagnosed ankylosing spondylitis, young male patient: exclusive cervical spine involvement.

Renal manifestations in adults with mitochondrial disease from the mtDNA m.3243A>G pathogenic variant

Mitochondrial diseases are a phenotype and genotype heterogeneous group of disorders that typically have a multisystemic involvement. The m.3243A>G pathogenic variant is the most frequent mitochondrial DNA defect, and it causes several different clinical syndromes, such as mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS), and the maternally inherited diabetes and deafness (MIDD) syndromes.Not frequently reported, renal involvement in these diseases is probably underestimated, yet it increases morbidity. It generally manifests as subnephrotic proteinuria and progressive deterioration of kidney function. Adult presentation of mitochondrial diseases is hard to recognize, especially in oligosymptomatic patients or those with exclusive kidney involvement. However, suspicion should always arise when family history, particularly on the maternal side, and multisystemic symptoms, most often of the central nervous system and skeletal muscles, are present.In this review we discuss the clinical diagnosis and approach of patients with renal manifestations in the context of the mtDNA m.3243A>G pathogenic variant.

Clinical and epidemiological features of mpox in a Brazilian reference center for HIV and sexually transmitted infections: A cross-sectional study

BackgroundThe 2022 mpox outbreak has affected disproportionately people living with HIV (PLWH) and pre-exposure prophylaxis (PrEP) users. MethodsWe conducted a cross-sectional study to evaluate factors associated with laboratory diagnosis of mpox among suspected cases, and access differences between PrEP users and PLWH with confirmed diagnostic. Results394 mpox suspected cases were analyzed, 309 (78.4%) confirmed. Most patients with mpox were PLWH (54.4%) and 99 (32%) PrEP users. Mpox cases were likely to be between 25 and 39 years old (aOR=2.8; p=0.042), men who have sex with men/bisexual or transgender women (aOR=17.2; p< 0.001) and to have fever (aOR=4.7; p< 0.001), adenomegaly (aOR=7.2; p< 0.001) and multiple vesicular lesions (aOR=4.2; p< 0.001). Comparing PrEP users to PLWH with confirmed mpox, PrEP users had lesions predominantly with exclusive genital involvement (p=0.016); while PLWH had higher extragenital involvement (p=0.018). ConclusionsPrEP users and PLWHA were the main epidemiological groups in our cohort. Recognizing the differences between vulnerable populations can contribute to the development public policies to control mpox in settings with reduced access to vaccines

IgG4-related diseases involving pleura: a case report and literature review

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease with the potential to involve virtually all organs, including the pancreas, kidneys, lungs, and pleura, amongst others. IgG4-RD pleural involvement may cause diverse complications such as pleural effusion, pleural thickening, pleural nodules, and additional lesions, which can be presented in many clinical diseases. However, isolated cases of pleurisy are still rare in IgG4-RD. We report a 72-year-old patient who was admitted to our hospital with cough, expectoration, and fatigue. He had a right-sided pleural effusion, and the tissue evaluation of the pleural biopsy by medical thoracoscopy met the diagnostic criteria of IgG4-RD. His serum IgG4 levels were elevated and he was finally diagnosed with IgG4-RD pleural involvement. He was subsequently started on prednisone 40 mg daily and his pleural effusion was almost disappeared 2 weeks later. This paper reported a case of IgG4-RD who had exclusive involvement of the pleura and highlighted the significance of considering IgG4-RD as a potential diagnosis in patients with unexplained pleural effusion.

Genetic and Epigenetic Characteristics in Isolated Pancreatic Metastases of Clear-Cell Renal Cell Carcinoma.

Isolated pancreatic metastases of renal cell carcinoma (IsPMRCC) are a rare manifestation of metastatic, clear-cell renal cell carcinoma (RCC) in which distant metastases occur exclusively in the pancreas. In addition to the main symptom of the isolated occurrence of pancreatic metastases, the entity surprises with additional clinical peculiarities: (a) the unusually long interval of about 9 years between the primary RCC and the onset of pancreatic metastases; (b) multiple pancreatic metastases occurring in 36% of cases; (c) favourable treatment outcomes with a 75% 5-year survival rate; and (d) volume and growth-rate dependent risk factors generally accepted to be relevant for overall survival in metastatic surgery are insignificant in isPMRCC. The genetic and epigenetic causes of exclusive pancreatic involvement have not yet been investigated and are currently unknown. Conversely, according to the few available data in the literature, the following genetic and epigenetic peculiarities can already be identified as the cause of the protracted course: 1. high genetic stability of the tumour cell clones in both the primary tumour and the pancreatic metastases; 2. a low frequency of copy number variants associated with aggressiveness, such as 9p, 14q and 4q loss; 3. in the chromatin-modifying genes, a decreased rate of PAB1 (3%) and an increased rate of PBRM1 (77%) defects are seen, a profile associated with a favourable course; 4. an increased incidence of KDM5C mutations, which, in common with increased PBRM1 alterations, is also associated with a favourable outcome; and 5. angiogenetic biomarkers are increased in tumour tissue, while inflammatory biomarkers are decreased, which explains the good response to TKI therapy and lack of sensitivity to IT.

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.