Pathological Entity Research Articles

Mixed squamous – neuroendocrine carcinoma of the gallbladder: A case report of a rare pathological entity

Gallbladder cancers are the most common biliary tract malignancies in the world. Adenocarcinoma constitutes the most common histology in gallbladder cancer. Neuroendocrine neoplasms of the gallbladder account for about 0.5% of all neuroendocrine neoplasms and 2.1% of all gallbladder tumors. They are rare tumors and present with non-specific symptoms such as abdominal pain, weight loss, anorexia, and obstructive jaundice, and, therefore, are often challenging to diagnose and treat. Mixed neuroendocrine-non-neuroendocrine neoplasm is a subtype of neuroendocrine neoplasm. To add to the literature, we report a rare case of a patient who presented with pain in the abdomen and non-bilious vomiting and was diagnosed with mixed neuroendocrine-non-neuroendocrine gallbladder cancer, identified by immunohistochemistry, and treated with palliative chemotherapy. Due to non-specific symptoms, patients may present at an advanced stage. Further, immunohistochemistry may assist in clinching the diagnosis.

Myelomeningocele: Long-Term Neurosurgical Management.

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.

Association of Anti-Ro/SSA and Anti-La/SSB Autoantibodies With Pregnancy Outcome in the General Population.

Autoimmune diseases encompass a diverse array of disorders that disturb the optimal functioning of the immune system, which is to eliminate the 'foreign or/and dangerous' to mistakenly target the body's own tissues. The aim of this research is to evaluate the most effective approach to managing autoimmune diseases within the framework of pregnancy. The exact causes and etiologies of these diseases are multifactorial and mostly still unclear. Ro/SSA autoantibodies and La/SSB, could be found in Sjögren's disease (SJ), systemic lupus (SLE) and other autoimmune disorders. Smoking, stress, UV exposure, vitamin D deficiency, and other genetic and environmental factors have been identified as risk factors for rheumatic diseases. Over the years, an ever-increasing incidence of these diseases has been observed in the general population, with the female sex being at increased risk for their occurrence. This fact raises the question of what should be the management of these pathological entities during pregnancy. Taking into account the very significant impact on the quality of paitients'daily life and the seemingly augmented prevalence of autoimmune diseases, as well as their preference in the female population, the reasonable question arises as to what should be the optimal management of these diseases in the context of pregnancy. Given the limited data of the global medical community regarding the etiological factors and mechanisms that trigger the onset of rheumatic diseases, the management of pregnant women is a complex conundrum that health professionals are challenged to face and solve.

Visualizing the Spectrum: B-scan Ultrasonography Across Diverse Ocular Abnormalities: A Pictorial Review

B-scan ultrasonography (USG) is a simple, non-invasive tool for diagnosing lesions of the posterior segment of the eye. Ocular conditions that hinder fundoscopy such as cataract, lens dislocation/ subluxation, vitreous degeneration, vitreous haemorrhage, retinal detachment, ocular trauma, ocular tumours such as metastasis, choroidal melanoma can be accurately evaluated with this modality. In addition to being cost effective, it is non-invasive, easily available, produces high resolution images and its results are reproducible. An understanding of the basic anatomy of the eye, the US technique, and common pathological entities affecting the eye will allow radiologists to readily diagnose these entities and thus guide the referring ophthalmologist in choosing the treatment. This article focuses on the common ocular pathologic conditions that affect the eye particularly the posterior ocular segment and their pictorial representation in B scan ultrasonography.

Contemporary Management of Pediatric Brainstem Tumors.

Brain tumors are the second most common malignancy in childhood. Around 15-20% of pediatric brain tumors occur in the brainstem. The most common type of brainstem tumor are diffuse tumors in the ventral pons, whereas focal tumors tend to arise from the midbrain, medulla, and dorsal pons. Glioma is the most common pathological entity. Contemporary management consists of surgery, radiotherapy, chemotherapy, and other adjuvant treatment. Surgical options range from biopsy to radical excision. Biopsy can be performed for diagnostic and prognostic purposes, or in the setting of clinical trials, mainly for diffuse intrinsic pontine gliomas. For focal tumors, surgeons need to carefully balance clinical outcomes against possible neurological sequelae in order to achieve maximal safe resection. Radiotherapy is essential for control of high-grade tumors and may be applied to residual or recurrent low-grade tumors. Proton therapy may provide similar efficacy and less neurotoxicity in comparison to conventional photon therapy. Oncological treatment continues to evolve from conventional chemotherapy to targeted therapy, immunotherapy, and other novel treatment methods and holds great potential as adjuvant therapy for pediatric brainstem tumors.

A Systematic Review on the Efficacy of Multidisciplinary Approaches in the Management of Chronic Pain

Background: Chronic pain presents widespread challenges globally. Multidisciplinary team (MDT) interventions have emerged as comprehensive approaches involving specialists from various medical fields to address the complexities of chronic pain management. This systematic review aims to synthesize evidence about the efficacy of multidisciplinary approaches in management of chronic non-specific musculoskeletal pain. Methods: This systematic review included randomized controlled trials (RCTs) of adults with chronic non-specific musculoskeletal pain. Multidisciplinary treatments involved at least three of: psychotherapy, physiotherapy, relaxation techniques, medical treatment, patient education, or vocational therapy. Exclusions were specific pathological entities, non-English studies, and follow-up less than one month. Searches were conducted across Cochrane evidence library, PubMed, and EMBASE. Primary outcomes encompassed pain intensity, quality of life, disability, and other functional measures. Results: Of the 100 articles identified, 6 studies met the inclusion criteria. These RCTs were conducted in Western countries, published between 2008 and 2019, and their sample size ranged from 132 to 401. Their interventions included Comprehensive Symptom Management, Assistance with Pain Management, physiotherapy, and telecare management. Two studies found significant improvements in pain intensity, while one demonstrated improved social functioning. However, no study found significant differences in disability scores or mental health outcomes. None of the studies assessed patient satisfaction with the interventions. Conclusion: Multidisciplinary team interventions for chronic pain show promise in improving pain intensity, particularly with Comprehensive Symptom Management and Pain Management Assistance. To strengthen these findings and address study limitations, future research with larger sample sizes is necessary.

Secondary vs. primary pituitary xanthogranulomas: which yellow is more mellow?

Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -"pure" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: "pure"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke's cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.

Type 1 VWD classification revisited: novel insights from combined analysis of the LoVIC and WiN studies

AbstractThere is significant ongoing debate regarding type 1 von Willebrand disease (VWD) defintion. Previous guidelines recommended patients with von Willebrand factor (VWF) levels <30 IU/dL be diagnosed type 1 VWD, whereas patients with significant bleeding and VWF levels from 30 to 50 IU/dL be diagnosed with low VWF. To elucidate the relationship between type 1 VWD and low VWF in the context of age-induced increases in VWF levels, we combined data sets from 2 national cohort studies: 162 patients with low VWF from the Low VWF in Ireland Cohort (LoVIC) and 403 patients with type 1 VWD from the Willebrand in The Netherlands (WiN) studies. In 47% of type 1 VWD participants, VWF levels remained <30 IU/dL despite increasing age. Conversely, VWF levels increased to the low VWF range (30-50 IU/dL) in 30% and normalized (>50 IU/dL) in 23% of type 1 VWD cases. Crucially, absolute VWF antigen (VWF:Ag) levels and increase of VWF:Ag per year overlapped between low VWF and normalized type 1 VWD participants. Moreover, multiple regression analysis demonstrated that VWF:Ag levels in low VWF and normalized type 1 VWD patients would not have been different had they been diagnosed at the same age (β = 0.00; 95% confidence interval, −0.03 to 0.04). Consistently, no difference was found in the prevalence of VWF sequence variants; factor VIII activity/VWF:Ag or VWF propeptide/VWF:Ag ratios; or desmopressin responses between low VWF and normalized type 1 VWD patients. In conclusion, our findings demonstrate that low VWF does not constitute a discrete clinical or pathological entity. Rather, it is part of an age-dependent type 1 VWD evolving phenotype. Collectively, these data have important implications for future VWD classification criteria.

Differential Expression of Circulating Damage-Associated Molecular Patterns in Patients with Coronary Artery Ectasia.

Coronary artery ectasia (CAE) is defined as abnormal dilation of a coronary artery with a diameter exceeding that of adjacent normal arterial segment by >1.5 times. CAE is a pathological entity of the coronary arteries and characterized as a variant of coronary atherosclerosis. CAE frequently coexists with coronary artery disease (CAD). While inflammation appears to be involved, the pathophysiology of CAE remains unclear. Damage-associated molecular patterns (DAMPs), defined as endogenous molecules released from stressed or damaged tissue, are deemed as alarm signals by the innate immune system. Inflammatory agents can generate DAMPs and DAMPs can create a pro-inflammatory state. In a prospective cross-sectional study, we enrolled 29 patients with CAE and non-obstructive CAD, 19 patients with obstructive CAD without CAE, and 14 control subjects with normal (control) coronary arteries age- and sex-matched with the CAE patients, to investigate the differential expression of plasma DAMPs. Patients with CAE and non-obstructive CAD had increased plasma levels of the DAMPs S100B, S100A12, HMGB1, and HSP70, the DAMPs receptor TLR4, and miR328a-3p compared to CAD and controls. Plasma levels of the mir328a-3p target the protective soluble form of the DAMPs receptor for advanced glycation end products (sRAGE), and the antioxidant DJ-1 was decreased in both CAE and CAD compared to controls. In an in vitro human umbilical vein endothelial cells model, circulating levels of S100B, HMGB1, HSP70 as well as CAE patient plasma induced inflammatory responses. The differential expression of the DAMPs S100B, HSP70, HMGB1, and their receptors TLR4 and sRAGE in CAE versus CAD makes them attractive novel biomarkers as therapeutic targets and therapeutics.

Laparoscopic Approach in the Surgical Management of McKittrick–Wheelock Syndrome

Mckittrick-Wheelock syndrome is a pathological entity that is represented by the presence of a large rectal tumour with histopathological findings of tubular-villous adenoma that associates important secretion of electrolyte-rich mucin.

Immunohistochemical Comparison of Ki-67 and MCM-3 in Odontogenic Cysts: An Observational Study.

Odontogenic cysts are a diverse group of pathologic entities with different proliferation potential, leading to variations in their biological behavior. One of the most cited proliferation markers used in diagnostic histopathology is Ki-67. Another group of proteins recently investigated is minichromosome maintenance (MCM-3) and its expression has been evaluated in several odontogenic lesions but the results were controversial. Thus, the present study endeavored to compare the expression of MCM-3 and Ki-67 in odontogenic cysts. Furthermore, a pioneer attempt was made to evaluate the sensitivity of these markers to inflammation. A total of 101 cases (37 dentigerous cysts, 37 odontogenic keratocysts, and 27 radicular cysts) were included. Immunohistochemical expression of Ki-67 and MCM-3 were investigated using a labeling index (LI). In addition, they were scored for inflammation, followed by correlation with both markers. The data obtained were subjected to statistical analysis ( P <0.05). Overall, a higher LI of MCM-3 than Ki-67 was obtained in all study groups along with a positive correlation of Ki-67 LI with inflammation. Thus, MCM-3 proteins proved to be a more accurate means to determine the proliferation potential and were not sensitive to external stimuli like inflammation than conventional markers, such as Ki-67.

Primary Small Cell Neuroendocrine Carcinoma of Bladder – A Rare Diagnostic Entity

Primary small cell carcinoma of the bladder is very rare pathological entity, follows an aggressive course and carries poor prognosis. The literature carrying these articles are scarce and due to which it poses a diagnostic challenge to the radiologist and urologists. Herein, we present a case of small cell carcinoma of urinary bladder in a 75 year old male patient initially reported as poorly differentiated urothelial carcinoma on transurethral resection of bladder tumor specimen to emphasize its rarity as well as the role of immunohistochemistry to differentiate between the two.

Fetal Type Morphologies Suggest the Presence of DICER1 Hotspot Mutations in Non-small Cell Lung Cancer.

Germline and somatic pathogenic variants (PVs) in DICER1, encoding a miRNA biogenesis protein, are associated with a wide variety of highly specific pathologic entities. The lung tumors pleuropulmonary blastoma, pulmonary blastoma (PB), and well-differentiated fetal lung adenocarcinoma (WDFLAC) are all known to harbor DICER1 biallelic variants (loss of function and/or somatic hotspot missense mutations), and all share pathologic features reminiscent of the immature lung. However, the role of DICER1 PVs in non-small cell lung cancer (NSCLC) is relatively unknown. Here, we aimed to establish the spectrum of lung pathologies associated with DICER1 hotspot PVs and to compare the mutational landscape of DICER1-mutated NSCLC with and without hotspots. We queried DNA sequencing data from 12,146 NSCLCs featuring somatic DICER1 variants. 235 (1.9%) cases harboring ≥ 1 DICER1 PV were found and 9/235 (3.8%) were DICER1 hotspot-positive cases. Histologic review of DICER1 hotspot-positive cases showed that all but one tumor were classified as within the histologic spectrum of PB/WDFLAC, whereas all the DICER1 non-hotspot double variants were classified as lung adenocarcinomas, not otherwise specified. Comparison between the mutational landscape of DICER1 hotspot-positive and hotspot-negative cases revealed a higher frequency of CTNNB1 mutations in the hotspot-positive cases (5/9 vs. 2/225; P<0.00001). We conclude that DICER1 somatic hotspots are not implicated in the most common forms of NSCLC but rather select for morphologic features of lung tumor types such as PB and WDFLAC. As a corollary, cases showing this tumor morphology should undergo testing for DICER1 variants, and if positive, genetic counseling should be considered.

Covid-19 infection and arterial hypertension: the relationship between the two entities

Background: The aim of the study was to breakdown the relationship and its nature between the two pathological entities arterial hypertension (AHT) and SARS-CoV-2 infection. The analysis of existing literature on both topics has been conducted. Sources including journals, books, existing publications and online platforms like Google Academic, PubMed, HINARI were used as search engines. Additionally, guidelines and circulars from European Society of Cardiology, American Heart Association and other respective bodies were also referred to. Data from large meta-analyses and clinical studies were included to bring out the relationship study. Pathophysiological breakdown of the two entities, contradicting proposals regarding treatment, new treatment modalities and de novo onset of AHT in post-Covid infection were included to delineate the relationship between the two pathological entities. Conclusions: Findings emphasize that the role of arterial hypertension in SARS-CoV-2 infection is mediated through its effect on the regulation of Renin Angiotensin Aldosteron System (RAAS), inflammation and immune responses. De-novo arterial hypertension was also reported in post SARS-CoV-2 infection patients. Though some initial studies hypothesized that RAAS inhibitors may add to clinical adversities, most studies afterward disproved the same and in fact revealed a protective role of the same. Angiotensin Converting Enzyme 2 (ACE2) is proposed as a treatment option in SARS-CoV-2 infection.

Pathways to selective vulnerability in neuromodulatory subcortical structures during the early pathogenic stages of Alzheimer’s disease

AbstractBackgroundThe two main catecholaminergic members of the isodendritic core nuclei are the substantia nigra (SN) and the locus coeruleus (LC). Despite cytoarchitectural and neurochemical similarities between the two nuclei, the LC is significantly more vulnerable to early degenerative changes in tauopathies like Alzheimer’s disease (AD) as compared to the SN. Here, we seek to use the gradient of vulnerability in these catecholaminergic nuclei to identify pathways associated with vulnerability in early AD pathological stages.MethodThis cohort includes individuals with no neuropathologic entities and Alzheimer’s disease proteinopathic stages. Individuals with TDP‐43, Lewy Body, or Chronic Traumatic Encephalopathy pathologic entities were excluded. The LC and SN was dissected from frozen human brainstem tissue using macroscopic anatomic features. To compare the LC and SN, only cases below Braak III with sufficient RNA from both nuclei were included. The Nanostring nCounter platform was used to probe specific pathways related to neurodegenerative processes. DBH and SLC6A3 served as confirmatory readouts of accurate anatomic sampling of the LC and SN. Differential gene expression analysis was used to evaluate patterns of gene expression that differentiate the two nuclei. Annotated gene sets were evaluated using global significance scores (GSS), a representation of cumulative t‐statistics for relevant genes.ResultThe LC and SN were collected from 26 cases (Figure 1) eligible for this analysis. Accurate dissections were confirmed by differential expression of DBH (p = 1.38e‐9) and SLC6A3 (p = 4.5e‐16). Among the differentially expressed genes (Figure 2), genes associated with microglia activation (GSS = 2.35), oxidative stress (GSS = 2.17), and lipid metabolism (GSS = 2.00) significantly differentiated the LC and SN.ConclusionThe LC and SN serve as examples of differentially vulnerable nuclei at the earliest neuropathologic stages of AD. Cell intrinsic factors, physiological demands, vascular contributions, and exposure to extrinsic factors like toxins are thought to contribute to the LC’s early vulnerability. Here, we show that neuroinflammation and oxidative stress pathways are likely central to differential vulnerability at the earliest stages of Alzheimer’s disease and may represent targets for intervention in prodromal stages.

The Relationship between Low Von Willebrand Factor, Type 1 Von Willebrand Disease and Ageing - Novel Insights from the Lovic and Win Cohort Studies

Introduction For many years, there has been debate about how to optimally diagnose type 1 VWD. Initial guidelines proposed that only individuals with plasma VWF levels &amp;lt; 30 IU/dL should be diagnosed with ‘type 1 VWD’. This was revised in recent ASH/ISTH/WFH/NHF panel which recommended that patients with a significant bleeding history and plasma VWF of 30-50 IU/dL should also be diagnosed with type 1 VWD, as opposed to the discrete ‘Low VWF’ entity proposed in previous guidelines. In this study, we investigated whether Low VWF is a discrete clinical entity, or whether it is instead part of an age-dependent type 1 VWD evolving phenotype. Methods We utilized datasets from two renowned national cohort studies - the Low VWF in Ireland Cohort (LoVIC) and Willebrand in the Netherlands (WiN) studies. In the LoVIC study, patients had a personal bleeding history and historically lowest VWF levels in the 30-50 IU/dL range. In the WiN study, patients had either a personal bleeding history or positive family history, combined with historically lowest VWF levels ≤ 30 IU/dL. Based upon levels at study inclusion versus original diagnosis, WiN patients were categorized into three groups - (i) patients with persistent VWF levels &amp;lt;30 IU/dL; (ii) patients with partial correction in VWF levels into the range of 30-50 IU/dL; and (iii) patients with normalization of VWF levels &amp;gt; 50 IU/dL. The FVIII:C/VWF:Ag ratio was used to assess the synthesis/secretion of VWF and the VWFpp/VWF:Ag ratio was used to assess the clearance of VWF. Results In total, 565 patients were included (403 WiN patients with type 1 VWD and 162 LoVIC patients with Low VWF). Mean age at diagnosis was significantly increased in the LoVIC cohort compared to the three WiN subgroups (32.5 years versus 23.3 years, 26.5 years and 25 years respectively; p&amp;lt;0.001). Conversely, there was no difference in age at time of enrollment into both national studies (p=0.532). To assess the significance of age, we first investigated how plasma VWF:Ag varied over time in WiN type 1 VWD patients compared to the LoVIC cohort. Among the total WiN cohort (with initial VWF levels &amp;lt; 30 IU/dL), 47% of subjects (n=188) had VWF levels that remained &amp;lt; 30 IU/dL despite advancing age. Conversely, 30% of type 1 VWD patients (n=121) increased their plasma VWF levels into the Low VWF range (30-50 IU/dL) over time, whereas 23% (n=94) had age-dependent increments that led to complete normalization in VWF levels &amp;gt; 50 IU/dL. Similarly, 39% (n=63) of patients from the LoVIC study had normalization of VWF levels over time. Crucially, we observed that VWF:Ag in Low VWF patients clearly overlapped with those in normalized (&amp;gt; 50 IU/dL) type 1 VWD subjects, indicating that the LoVIC cohort is a subgroup within the WiN normalized subgroup (Figure 1). To further investigate this concept, we performed multiple regression analysis. Importantly, we observed that plasma VWF:Ag in the LoVIC cohort and the WiN normalized (&amp;gt; 50 IU/dL) subgroup would have not been different had they been diagnosed at the same age (difference of β=0.00 (95% CI -0.03 to 0.04)). Cumulatively, these results indicate that significant heterogeneity exists amongst type 1 VWD patients with respect to the effect of ageing on their plasma VWF levels. In addition, our findings demonstrate that because of age-dependent increases in plasma VWF:Ag, the majority of Low VWF patients would have been diagnosed with type 1 VWD had they undergone assessment earlier in life. Consistently, no difference in prevalence of VWF mutation (36.4% vs 22.6% respectively, p=1.000), FVIII:C/VWF:Ag ratio (1.24 ±0.24 vs 1.46 ±0.27 respectively, p=0.444) or VWFpp/VWF:Ag ratio (1.36 ±0.99 vs 1.75 ±0.38 respectively, p=1.000) was found between LoVIC and normalized WiN patients (Figure 2). Contrarily, WiN patients with persistent VWF levels &amp;lt;30 IU/dL had more often VWF gene variants (93.0%), higher FVIII:C/VWF:Ag ratio (2.65 ±1.62) and higher VWFpp/VWF:Ag ratio (6.14 ±7.05) compared to the other groups (Figure 2, p&amp;lt;0.001). Conclusion Our findings clearly demonstrate that Low VWF does not constitute a discrete clinical or pathological entity. Rather, it is part of an age-dependent type 1 VWD evolving phenotype, as demonstrated by the same VWF levels throughout life, genetic background and pathophysiology. This study has direct consequences for VWD diagnosis, the overall understanding of type 1 VWD as an evolving disease and for patients with low VWF/type 1 VWD.

Epstein-Barr virus positive mucocutaneous ulcer – A diagnostic challenge

A significant portion of the global population carries the asymptomatic Epstein-Barr virus (EBV). Transfusions of blood and blood derivatives, organ and tissue transplantation, and oropharyngeal secretions are the main routes of transmission. The World Health Organization recently recognized the EBV mucocutaneous ulcer (EBVMU) as a provisional pathological entity in its classification of hematopoietic and lymphoid tissues. It affects patients who are elderly or immunosuppressed. Histologically, it may be difficult to distinguish from Hodgkin lymphoma (HL). Although skin or mucosa are uncommon sites of HL involvement, diagnosis at these sites should be made with extreme caution. The prognosis for EBVMU is good; cases regress on their own or after immunosuppressive therapy is reduced. We present a 67-year-old woman with a non-healing oral cavity ulcer which was diagnosed as EBV-positive mucocutaneous ulcer.

Necrotizing Vasculitis Post COVID 19: About A Series of 5 Cases

Post-covid19 necrotising vasculitis is a recently introduced pathological entity, representing one of the rare extra-respiratory covid 19 infections that can threaten vital and functional prognosis. Our series included 5 children, three girls and two boys. The average age of our patients was 6 years. The median time between exposure to the SARS Cov2 virus and the onset of clinical symptoms was 4.5 weeks. The clinical expression of post-covid vasculitis was polymorphic, including mainly skin lesions with cyanosis in 60% and necrotic purpura in 40% of cases. Cardiac anomalies were noted in only one patient presenting myocarditis with pulmonary arterial hypertension. No renal or ocular anomalies were observed in any of our patients. The PCR COVID 19 was positive in only one patient, associated to positive SARS Cov2 IgM and IgG serology. The other four patients had only positive IgG serology. The diagnosis of necrotising vasculitis was confirmed by histological study of skin biopsies. On the basis of all the clinical, biological, radiological and histological criteria, the diagnosis of acute necrotising vasculitis post SARS COV 2 infection was set, since all the biologic tests were normal apart from the recent coronavirus infection. Our patients received medical treatment including oral corticosteroids and anticoagulants, with a good clinical and biological outcome, except for two children who underwent amputation of both legs.

Distinct ways to perform a liver biopsy: The core technique setups and updated understanding of these modalities.

There is dramatically increased incidence of several liver diseases worldwide; thus, an unmet need to diagnose and stage these pathological entities heralds the wide application of liver biopsy (LB) techniques. The ways of LB are versatile, including percutaneous LB, transjugular LB, and more recently an approach of minimal invasiveness, that is, EUS-guided LB (EUS-LB). In this review article, we come to the conclusion that EUS-LB may serve as a feasible, reliable, and safe alternative to percutaneous LB and transjugular LB in terms of improved diagnostic yield, excellent sampling performance, and controlled adverse events among patients with focal, infiltrative, and parenchymal liver diseases. Furthermore, extensive efforts have been made to optimize and refine several technical pillars within EUS-LB modality such as the selection of needle size/type, priming manner of biopsy needle, and choice of pass/actuation technique, all of which aim at obtaining better specimen quantity and quality. Another advantageous aspect and unique property pertinent to EUS-guided modality indicate that multiple screening, surveillance, and intervention procedures can be combined into one single endoscopic session. Accordingly, some pilot studies have clarified the clinical usefulness by integrating EUS-LB with simultaneous measurement of portal pressure gradient or examination of liver stiffness. However, more studies, in particular, randomized controlled trials or real-world evidence, are practically warranted to elucidate the validity and safety of EUS-LB as a regular/routine part of managing liver diseases.

A COMPARATIVE STUDY TO KNOW THE EFFECT OF POSITION DURING SPINAL ANESTHESIA ON POSTDURAL PUNCTURE HEADACHE IN LOWER ABDOMINAL SURGERIES

Background: Pain is pathologic entity, noxious stimulus and a most common problem challenging the anaesthetists. Spinal anaesthesia is a commonly used technique for providing surgical anaesthesia to patients undergoing urological, gynecological, abdominal and lower limb surgeries (1). PDPH was first described by August Bier in 1898. The incidence of PDPH after spinal anaesthesia in obstetric anaesthesia is 1% - 6%. The risk factors of PDPH after spinal anaesthesia are needle size, direction of bevel, needle design, number of lumbar puncture attempts, age, sex, pregnancy, and previous history of PDPH(4). Objectives: To evaluate the effect of position during spinal anaesthesia on PDPH in patients undergoing lower abdominal surgeries and to study complications associated with spinal anaesthesia. Methods: After approval from the ethical committee and informed written consent from the patient. A prospective randomised study was conducted with hundred ASA grade I and II patients aged between 18-60 years undergoing lower abdominal surgeries and were divided into two groups of 50 each .Group A and Group B, where first group were given spinal in lateral position and latter group in sitting position. The intensity of PDPH was assessed postoperatively using a Numeric Rating Scale immediately on either postoperative day (POD) one, two, or three as described to the patient during the preoperative visit. Results: There was a significant decrease in incidence and severity of PDPH when the subarachnoid block was given in the lateral recumbent position as compared to that given in the sitting position. Conclusion: Hence, lateral position is preferred for spinal anaesthesia for patients undergoing lower abdominal surgeries.