A Case of Nonislet Cell Tumor Hypoglycemia Due to Metastatic Salivary Myoepithelial Carcinoma.

Background/Objective: Nonislet cell tumor hypoglycemia (NICTH) is an uncommon cause of hypoglycemia due to a relative surplus of insulin-like growth factor 2 (IGF-2) or its precursor molecule. The diagnosis is confirmed by an elevated ratio of IGF-2 to insulin-like growth factor 1 (IGF-1). Myoepithelial carcinoma (MECA) is a rare and aggressive salivary gland cancer that has not been previously associated with NICTH. Case Report: A 63-year-old female with a past medical history of metastatic salivary MECA, type 2 diabetes mellitus previously on metformin, hypertension, and hypothyroidism presented to her oncologist for chemotherapy and was found to have a serum glucose of 30 mg/dL (reference: 65-99). She was admitted for further diagnostic work-up which revealed an insulin level of <1 µU/mL (reference: 3-25), C-peptide <0.5 ng/mL (reference: 1.1-4.3), IGF-1 of 15 ng/mL (reference: 41-279), and IGF-2 of 147 ng/mL (reference: 180-580) with an IGF-2:IGF-1 molar ratio of 10, consistent with NICTH. The patient's hypoglycemia unfortunately was quite resistant to treatment, requiring a combination of corticosteroids, continuous dextrose infusion, and somatostatin injections. The patient died 3 weeks after presenting with hypoglycemia. Discussion: Salivary MRCAs commonly contain pleomorphic adenoma gene 1 oncogene rearrangements which are associated with increased IGF-2 production and may predispose patients to hypoglycemia. Conclusion: This case demonstrates that NICTH can be associated with metastatic salivary MECA. The hypoglycemia in this scenario is challenging to manage and is associated with poor prognosis.

Current approaches to diagnosing and treating idiopathic granulomatous mastitis: A summary from in-depth clinician interviews.

Background: Idiopathic granulomatous mastitis (IGM) is a rare, chronic inflammatory breast condition primarily affecting women of reproductive age. Its diagnosis is challenging due to similarities with other breast disorders, necessitating exclusion of other granulomatous diseases. The management of IGM remains inconsistent and unclear, with high recurrence rates and varying practices. Methods: This qualitative study involved semi-structured interviews with nine clinicians from Singapore, Malaysia, and Egypt to examine current diagnostic and therapeutic approaches for IGM. Transcripts were analysed using NVivo software for coding and summarisation. Findings: Clinicians predominantly used imaging and histopathology for diagnosis. Treatment commonly involved corticosteroids, though dosages and tapering regimens varied widely. Methotrexate was used sparingly for refractory cases due to associated risks. Surgical interventions were infrequent, reflecting a preference for medical management. There was a consensus on the need for randomised controlled trials (RCTs) to establish standardised treatment protocols. Interpretation: This study reveals the complex nature of IGM diagnosis and treatment from clinicians in Singapore, Malaysia and Egypt. This underscores the need for more specific and definitive diagnostic tests, rather than relying on exclusionary methods, and standardised treatment guidelines. Multi-centre RCTs are essential for developing evidence-based protocols to improve patient outcomes and address regional differences effectively.

[Efficacy and toxicity of short-course corticosteroid therapy in chronic bronchial diseases]. / Efficacité et toxicité de la corticothérapie en cures courtes dans les pathologies bronchiques chroniques.

Chronic respiratory diseases such as asthma and chronic obstructive pulmonary disease (COPD) are characterized by airway inflammation. While corticosteroids (CS) are frequently prescribed during exacerbations of these conditions, their repeated use is associated with numerous side effects. The aim of this review is to synthesize the recent literature on the indications, benefits, and risks of short-term CS therapy for these two diseases. French guidelines recommend short-term CS as a first-line treatment during asthma exacerbation (0,5 to 1mg/kg/day, not exceeding 60mg/day, for at least 5 to 7 days) or as a second-line treatment for COPD exacerbation (5 days, 30 to 40mg/day). However, these recommendations are not without limitations; they are primarily based on studies conducted in hospital settings, raising questions about the generalizability of their results to primary care, and as they employ a "one size fits all" strategy, they do not take into account the phenotypic heterogeneity of different patients. Moreover, repeated short-term CS courses generate side effects that even at low doses can appear early in young asthma patients, and they can exacerbate pre-existing comorbidities in COPD patients. The concept of a threshold dose should be employed in routine practice in view of accurately assessing the risk of side effects. In the near future, it will be important to consider recently published data supporting the use of predictive biomarkers for responses to CS, particularly in COPD cases.

Allergic rhinitis management: a survey on Italian primary care pediatricians.

Summary: Background. Allergic rhinitis (AR) is a widespread condition. The Italian Society of Pediatric Allergology and Immunology (SIAIP) promoted an initiative to update the knowledge on AR in children and adolescents. The present survey directly addressed primary care pediatricians, thus reflecting the real-world management of AR in children and adolescents. The aim was to investigate common practice in managing AR children. Methods. A panel of experts drafted a series of questions concerning the practical management of children with AR in clinical practice. The questionnaire was administered to a large sample of primary care pediatricians (864). Results. 864 primary care pediatricians participated to the survey. Each pediatrician on average follows 94 children with AR; globally 81,231 children. More than 70% of participants follow ARIA guidelines. Accordingly, 42% of children have mild AR and 58% moderate/severe. Asthma, conjunctivitis and adenoid hypertrophy are the most common comorbidity. Most pediatricians autonomously follow their patients. The intensity of treatment (use of medication) is directly proportional to the symptom severity.  Intranasal corticosteroids are the most common medication used followed by oral antihistamines and nasal lavages (with hypertonic or isotonic solution). Up to 20% of participants prescribe the fixed association topical corticosteroids plus antihistamine. Conclusions. The present survey demonstrated that Italian primary care pediatricians accomplish ARIA guidelines and adapt treatment on the basis of the intensity of symptoms. Corticosteroids and antihistamines are the most common prescribed medications. Nasal lavages are also popular.

Comparative Risk of Neuropsychiatric Adverse Events Associated With Leukotriene-Receptor Antagonists Versus Inhaled Corticosteroids.

BACKGROUND: Leukotriene-receptor antagonists (LTRA) and inhaled corticosteroids (ICS) are common controller medications for asthma, but limited studies examine their comparative risks on neuropsychiatric adverse events (NAEs) in asthma patients. OBJECTIVE: To investigate the comparative risks of LTRA versus ICS on seven distinct categories of NAEs in asthma patients at a nationwide level. METHODS: We conducted a nationwide cohort study during 2010-2021. Incident NAEs and its clinical subgroups (e.g., psychotic disorders, anxiety disorders, movement disorders, behavioral and emotional disorders, mood disorders, sleep-related disorders, and personality disorders) were assessed. Cox proportional hazards regressions were employed to quantify the comparative risks. RESULTS: There were 1,249,897 asthma patients aged 6-64 years. Incidence rates for NAEs were 25.10 per 1000 person-years among patients treated with LTRA, and 23.46 per 1000 person-years among those treated with ICS. The incidence rate difference was 1.64 [95%CI: 0.30-2.98] per 1000 person-years. Positive associations of NAEs and three clinical subgroups were found in patients treated with LTRA compared to ICS (hazard ratios (HR): 1.06 [95%CI: 1.00-1.12] for NAEs; HR: 1.88 [95%CI: 1.24-2.84] for psychotic disorders; HR: 1.10 [95%CI: 1.01-1.20] for anxiety disorders; and HR: 1.27 [95%CI: 1.02-1.58] for behavioral and emotional disorders), but not for movement disorders, mood disorders, sleep-related disorders, and personality disorders. CONCLUSIONS: This nationwide cohort study identified heightened risks, ranging from 6% to 88%, of NAEs and three clinical subgroups in asthma patients treated with LTRA compared to ICS. These findings underscore the necessity for clinicians to communicate with patients regarding potential neuropsychiatric harms when prescribing LTRA.

Resolution versus persistence of childhood idiopathic nephrotic syndrome-A population-based study.

AIM: To determine the duration of relapsing childhood idiopathic nephrotic syndrome (INS). METHODS: In this population-based study, we retrospectively analysed the computerised database of Israel's largest health maintenance organisation. Children (age 2-10 years) with a new INS diagnosis and a corticosteroid (CS) prescription between 2000 and 2010 were included. NS category was determined, according to CS and/or steroid-sparing agents (SSA) purchases. RESULTS: Out of 1 669 977 eligible children, 608 fulfilled inclusion criteria. Patients in the fourth quartile of purchases (n = 132) had an older age at last relapse (17.9 ± 6.3 vs. 11.3 ± 5.9 years, p < 0.001) and more SSA use (78.8% vs. 20%, p < 0.001) compared to the remaining three quartiles. A single episode occurred in 84 patients. Of the remaining 524 patients (males 66%, diagnosis age: 4.8 ± 2.2 years, SSA prescribed: 35%) who were followed for 15.5 ± 5.1 years, 113 (21.6%) had a continuing disease at an age of 19.3 ± 6.3 years. The leftover 411 entered long-lasting treatment-free remission at age 11.2 ± 5.7 years. CONCLUSION: In this multicentre study, we identified INS disease course by medication delivery. NS long-standing remission occurs at age 11.2 ± 5.7 years in most cases. However, the disease continues into adulthood in a fifth of the relapsing patients, implicating the need for proper transition to adult care.

The effect of dexamethasone and bupivacaine on postoperative pain and nausea and vomiting by preperitoneal nerve blocking in laparoscopic cholecystectomy: a randomized clinical trial.

Background: This study aims to examine the effects of preperitoneal administration of dexamethasone and bupivacaine surrounding laparoscopic trocars on postoperative pain (POP) and nausea and vomiting (PONV) in patients undergoing laparoscopic cholecystectomy (LC). Method: In this randomized triple-blinded trial with a 1:1 randomization ratio, 104 patients with chronic cholecystitis were candidates for elective LC. A total of 40 mg (8 ml) of bupivacaine was mixed with 8 mg (2 ml) of dexamethasone or normal saline. The solution was injected preperitoneally via an 18G needle parallel and lateral to trocars until a bulge in the interior surface of the parietal peritoneum was observed by the camera. Primary outcomes were the severity of POP based on 0-10 Likert visual analog scale (VAS) and rates of PONV and secondary outcomes were rate of postoperative opioid usage and any side-effects. Result: The mean VAS score was significantly lower in the dexamethasone group (3.5 vs. 6.2, P<0.001). The dexamethasone group had 46.2% and 26.9% lower rates of nausea and vomiting after LC compared to the other group (P=0.001 and 0.015, respectively). Postoperative opioid use was lower in the dexamethasone group, but its difference was insignificant (P=0.3). Conclusions: Preperitoneal dexamethasone injection around laparoscopic trocars may lower the intensity of POP and PONV rates. Perioperative local corticosteroids can be used as an effective, available, and inexpensive analgesic and antiemetic prevention for laparoscopic procedures.

Effect of Continuous Infusion vs Bolus Dose of Hydrocortisone in Septic Shock: A Prospective Randomized Study.

Aim and background: Corticosteroids are recommended for use in adult patients with septic shock requiring vasopressors for blood pressure maintenance. However, this predisposes them to hyperglycemia, which is associated with a poor outcome. This prospective randomized study compares the effect of continuous infusion with bolus hydrocortisone on blood glucose levels in septic shock. Materials and methods: Forty adult patients with sepsis and septic shock requiring vasopressor support were randomly allocated to either group C (continuous infusion of hydrocortisone 200 mg/day) or group B (intermittent bolus dose of hydrocortisone 50 mg IV 6 hourly). Blood glucose level (primary objective), number of hyperglycemic and hypoglycemic episodes, daily insulin requirement, shock reversal incidence, time to shock reversal, and nursing workload required to maintain blood glucose within the target range (82-180 mg/dL) were compared. Results: The mean blood glucose level was comparable in the two groups (136.5 ± 22.08 mg/dL in group C vs 135.85 ± 19.06 mg/dL in group B; p = 0.921). The number of hyperglycemic and hypoglycemic episodes (p = 1.000 each), insulin requirement/day (p = 1.000), and nursing workload (p = 0.751) were also comparable among groups. Shock reversal was seen in 7/20 (35%) patients in continuous group and 12/20 (60%) patients in bolus group (p = 0.113). Time to shock reversal (p = 0.917) and duration of ICU stay (p = 0.751) were also statistically comparable. Conclusion: Both the regimes of hydrocortisone, continuous infusion, and bolus dose, have comparable effects on blood glucose levels in patients with septic shock.The study was registered prospectively with ctri.nic.in (Ref. No. CTRI/2021/01/030342; registered on 8/1/2021). How to cite this article: Salhotra R, Sharahudeen A, Tyagi A, Rautela RS, Kemprai R. Effect of Continuous Infusion vs Bolus Dose of Hydrocortisone in Septic Shock: A Prospective Randomized Study. Indian J Crit Care Med 2024;28(9):837-841.

Current Pharmacotherapy and Nutrition Therapy of Alcohol-Associated Liver Disease.

Patients with alcohol-associated liver disease (ALD) consume large amounts of empty calories and are at risk for malnutrition. Malnutrition can present with micro- or macro-nutrient deficiencies. The standard-of-care drug treatment for severe alcohol-associated hepatitis (AH) is corticosteroids. While still in the standard treatment there are limitations in efficacy and certain patients do not respond to treatment (Lille score ≥.45). This article will focus on important concepts related to nutrition and ALD and on recent findings on predicting corticosteroid response and prognosis for AH patients.

Impact of systemic steroids on the efficacy of first line imatinib treatment of patients with advanced gastrointestinal stromal tumors (GISTs).

BACKGROUND: Effective management of adverse events is required to maintain sufficient imatinib dosing when treating patients with gastrointestinal stromal tumors (GISTs). Skin rash is a common adverse event of imatinib, which can be effectively controlled by systemic steroid treatment without imatinib dose modification or interruption. However, the impact of the use of systemic steroids on the efficacy of imatinib treatment remains unclear. METHODS: Between October 2014 and February 2022, 277 consecutive patients from a prospective registry of GIST patients were included as the study population. Patients who started systemic steroids due to grade ≥ 3 skin rash or grade 2 skin rash with grade 2 pruritis were classified as the steroid group, whereas patients who did not develop a skin rash or those who did not require steroids for a mild skin rash were classified as the control group. Efficacy outcomes were compared between the two groups. RESULTS: Among the 277 patients, 30 (10.8%) were treated with systemic steroids for skin rash. There was no significant difference in progression free survival (PFS) or overall survival (OS) between the steroid and control groups (3-year PFS, 67.7% vs. 65.1%, p = 0.53; 3-year OS, 91% vs. 89.9%, p = 0.67, respectively). The use of systemic steroids was not an independent factor associated with PFS (hazard ratio 0.73, 95% confidence interval 0.36-1.49, p = 0.39) and OS (hazard ratio 0.37, 95% confidence interval 0.12-1.18, p = 0.09). In the steroid group, patients who successfully maintained the imatinib dosage showed a trend toward more favorable survival outcomes than those who did not (3-year PFS, 73.3% vs. 44.4%, p = 0.34; 3-year OS, 95.8% vs. 75.0%, p = 0.15, respectively). CONCLUSIONS: The use of systemic steroids for the control of imatinib induced severe skin rash did not adversely affect the efficacy outcomes of imatinib in patients with advanced GIST.

Efficacy of Ruxolitinib with corticosteroids in idiopathic pneumonia syndrome post-allogeneic hematopoietic stem cell transplantation: A single-center experience and systematic review.

BACKGROUND: Idiopathic Pneumonia Syndrome (IPS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a life-threatening complication with high morbidity and mortality. IPS is thought to arise from damage caused by various inflammatory mediators. This study assesses the effectiveness of Ruxolitinib, a Janus Kinase (JAK) 1 and 2 inhibitor that blocks cytokine production, in combination with corticosteroids (CS) for managing IPS after allo-HSCT, compared to the conventional use of CS alone in a case series and a systematic review of previously published literature. METHODS: The study includes a retrospective case series of three patients treated for IPS with Ruxolitinib and CS from the University of Kansas Medical Center and a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Statement 2020 guidelines. The systematic review encompassed seven studies involving 346 cases including three cases from the case series. Statistical analyses were conducted using SPSS v.25. RESULTS: The case series included three patients with IPS after allo-HSCT who received ruxolitinib and CS with favorable results. All patients showed substantial improvement with no IPS-associated mortality. Two of the three patients in the case series were discharged on a 2 L nasal cannula, which was later discontinued during follow-up visits, while the third was discharged on room air. There was marked improvement observed on the computed tomography (CT) following the use of ruxolitinib. Of the total 346 cases included in the systematic review, the median age was 46.6 years (Range 5-72), and 62 % were males. The primary disorders were acute leukemia (52 %), chronic myeloid leukemia (12 %), myelodysplastic syndrome (11 %), Lymphoma (10 %), and others (21 %). Stem cell sources were peripheral blood (45 %), bone marrow (49 %), and cord blood (6 %). Donor types involved match unrelated (55 %), match related (36 %), and mismatched related (4.5 %). Most patients received myeloablative conditioning (81 %). Acute GVHD was observed in 47 %, and chronic GVHD in 38 %. The primary treatment was CS (96 %), with limited use of ruxolitinib (1 %) and etanercept (9.5 %). The mortality rate was 63.3 %, whereas in our case series with the use of ruxolitinib, it was zero. CONCLUSION: The combination of Ruxolitinib and CS for treating IPS post-allo-HSCT suggested promising results in the case series, with favorable response and improved survival by blocking the cytokine production contributing to IPS. The significant mortality difference in the systematic review supports the need for innovative treatment approaches, highlighting the potential role of Ruxolitinib in CS-refractory cases. Despite the positive outcomes in the case series, the absence of randomized controlled trials emphasizes the necessity for further research.

Overall Survival and Treatment Patterns Among Patients With Warm Autoimmune Hemolytic Anemia in Sweden: A Nationwide Population-based Study.

OBJECTIVES: Warm autoimmune hemolytic anemia (wAIHA) is a rare autoantibody-mediated disorder, and first-line treatment primarily relies on corticosteroids. This study assessed overall survival (OS) and treatment patterns of wAIHA in Sweden. METHODS: Adults with ≥ 1 primary diagnosis code for wAIHA (or AIHA plus oral corticosteroids (OCS)/immunosuppressants as sensitivity analyses) between 2011 and 2022 were identified from five Swedish national registers and linked through each patient's unique identity number. Kaplan-Meier curves with log-rank tests and Cox regressions were performed to assess OS for patients with primary versus secondary wAIHA and patients with wAIHA and long-term versus short-term (≥ 3 vs. < 3 months) OCS users. RESULTS: The main analysis included 292 patients; 1791 patients were included in the sensitivity analysis. At a median 3.7-year follow-up, a median OS in primary wAIHA was not reached versus 6.0 years for secondary wAIHA (log-rank test: p = 0.003). Subgroup analyses showed no significant difference in risk of death between long-term and short-term OCS users; however, in the sensitivity analysis, long-term OCS users showed significantly higher risk of death (adjusted hazard ratio: 1.45; 95% confidence interval: 1.180, 1.781; p < 0.001) versus short-term OCS users. CONCLUSION: Secondary wAIHA or long-term OCS use was associated with lower OS, underscoring the disease burden and unmet need for efficacious wAIHA treatments.

Inhaled Corticosteroids Particle Size and Risk of Hospitalization Due to Exacerbations and All-Cause Mortality in Patients with Chronic Obstructive Pulmonary Disease. A Nationwide Cohort Study.

Background: Extra-fine particle inhaled corticosteroids (ICS) improve peripheral airway distribution, but their effect on risk of exacerbations and all-cause mortality in patients with chronic obstructive pulmonary disease (COPD) is unclear. Methods: This observational cohort study compares patients with COPD who received extra-fine particle ICS to those who received standard particle size ICS from 2010 to 2017 while followed in outpatient clinics. The primary outcome was the time to a COPD exacerbation that required hospitalization, with all-cause mortality as a secondary outcome. Data were analyzed using an adjusted Cox proportional hazards model and a competing risk analysis. Two predefined subgroup analyses of patients treated with pressurised metered dose inhalers (pMDIs) and patients with a previous exacerbation history, was carried out. Lastly, we created a propensity score matched cohort as a sensitivity analysis. Results: Of the 40,489 patients included, 38,802 (95.8%) received stand particle size ICS and 1,687 (4.2%) received extra-fine particle ICS. In total 7,058 were hospitalized with a COPD exacerbation, and 4,346 died. No significant protective effect of extra-fine particle ICS against hospitalization due to COPD exacerbations (HR 0.93, 95% CI 0.82-1.05, p=0.23) or all-cause mortality (HR 1.00, 95% CI 0.85-1.17, p=0.99) was found when compared to standard particle size ICS. However, in the subgroup analysis of patients treated with pMDIs, extra-fine particle ICS was associated with reduction in risk of exacerbations (HR 0.72, 95% CI 0.63-0.82, p<0.001) and all-cause mortality (HR 0.72, 95% CI 0.61-0.86, p<0.001). Conclusion: The administration of extra-fine particle ICS was not associated with reduced risk of exacerbations or all-cause mortality in our primary analysis. A subgroup consisting of patients treated with pMDIs suggested potential protective benefits.

Eosinophilic Ascites: A Rare Diagnosis With an Even Rarer Etiology.

Eosinophilic ascites (EA) is a rare and often challenging clinical manifestation of eosinophilic gastroenteritis (EGE), a condition characterized by eosinophilic infiltration in various layers of the gastrointestinal tract. EA specifically involves the abnormal accumulation of eosinophils in the peritoneal cavity, which can lead to significant abdominal distension and discomfort. EGE is an inflammatory disorder that can affect the mucosal, muscular, or serosal layers of the gastrointestinal tract, primarily resulting from a combination of genetic predisposition, environmental triggers, and immune responses. This case report discusses a 39-year-old male who presented with persistent abdominal distension, significant weight loss, vomiting, and chronic diarrhea. Clinical evaluation revealed marked eosinophilia and EA, prompting a series of diagnostic tests to differentiate it from other potential causes such as parasitic infections and malignancies. Imaging studies indicated moderate ascites and intestinal wall thickening. The patient was diagnosed with eosinophilic enteritis, and treatment with corticosteroids led to substantial clinical improvement. This case highlights the diagnostic challenges and management strategies associated with both EA and EGE, emphasizing the importance of recognizing these rare manifestations of eosinophilic gastrointestinal disorders.

Isolated intestinal manifestation of an invasive varicella zoster virus reactivation in an immunocompromised patient: a case report.

Invasive varicella zoster infection is a rare but severe infectious disease, potentially affecting almost every organ system and presenting with a variety of symptoms. It is usually seen in immunocompromised patients, but also occurs in immunocompetent patients. Isolated intestinal manifestations without skin lesions are even more scarce. We present a case of a 78-year old immunocompromised man with an isolated intestinal Varicella Zoster reactivation. If not early diagnosed and treated, an invasive infection can lead to life-threatening complications. Therefore, awareness in both immunocompromised as immunocompetent patients is very important in the daily clinical practice.

Antenatal corticosteroids for late small-for-gestational-age fetuses.

OBJECTIVES: To compare neonatal morbidity in late preterm pregnancies with small-for-gestational-age fetuses, between those exposed and not exposed to antenatal corticosteroids (ACS). METHODS: A retrospective study which included growth-restricted fetuses delivered at gestational week 34+0 to 36+6 weeks at a tertiary university-affiliated hospital, from March 2016 to March 2022. The primary composite outcome included the need for oxygen therapy or ventilation, respiratory distress syndrome, transient tachypnea of the newborn, bronchopulmonary dysplasia, necrotizing enterocolitis, intraventricular hemorrhage grade III/IV and neonatal mortality. RESULTS: The primary composite outcome was comparable between those who did and did not receive ACS (26.1 vs. 20.8 %, p=0.512). Neonatal morbidity rates did not differ significantly between the groups, except for hypoglycemia, which was more common among neonates from ACS-exposed mothers (37.0 vs. 19.5 %, p=0.037). Multivariate analysis, adjusted for gestational diabetes and the mode of delivery showed no significant difference in the composite outcome between the groups (OR=2.03, 95 % CI 0.79-5.20, p=0.142). Cesarean delivery was associated with a higher risk of the primary outcome (OR=2.13, 95 % CI 1.17-3.85, p=0.013). After excluding those who did not receive the initial betamethasone dose within 2-7 days before delivery, the primary composite outcome remained similar between the groups. The primary composite outcome was similar among severely growth-restricted fetuses (<5th percentile) exposed and not exposed to ACS (29.2 vs. 22.0 %, p=0.560). CONCLUSIONS: Among preterm pregnancies complicated by small-for-gestational-age fetuses, ACS did not lower the rate of neonatal morbidity.

A case of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome presenting as progressive multisystem involvement with parenchymal infiltrates following infection with Epstein Barr virus.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic) syndrome is a rare multisystem disease affecting predominantly males over 50 and manifesting as widespread progressive inflammatory sequelae and haematological dysfunction. We describe a patient who presented with systemic symptoms of fevers, night sweats and weight loss, and developed progressive inflammatory sequelae including cutaneous lesions, haematological dysfunction, lymphadenopathy, migratory inflammatory arthropathies, with new pulmonary infiltrates, following infection with Epstein Barr Virus. Laboratory investigations, bronchoscopy, bone marrow biopsy and imaging were consistent with an inflammatory aetiology. The constellation of organ system involvement, laboratory, biopsy, and imaging results were suspicious for VEXAS syndrome, and this diagnosis was confirmed by identification of a somatic mutation in the UBA1 gene following extensive exclusion of infectious and autoimmune causes. Interestingly the onset of the VEXAS syndrome coincided with serological confirmation of Epstein Barr Virus raising the importance of further exploration into the underlying aetiology of VEXAS syndrome.

Treatment of chronic hand eczema with topical anti-inflammatory drugs other than topical corticosteroids.

Chronic hand eczema is a fluctuating, inflammatory, pruritic disease of the hands and wrists that is commonly treated with topical corticosteroids and emollients. In a recent report in The Lancet, Bissonnette et al. demonstrated that delgocitinib cream showed superior efficacy versus a cream vehicle and was well tolerated over 16 weeks in adults with moderate to severe chronic hand eczema.