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Mast-cell expressed membrane protein-1 (MCEMP1) is higher in patients with idiopathic pulmonary fibrosis (IPF) with an increased risk of death. Here we aimed to establish the mechanistic role of MCEMP1 in pulmonary fibrosis. We identified increased MCEMP1 expression in classical monocytes and alveolar macrophages in IPF compared with controls. MCEMP1 is upregulated by transforming growth factor beta (TGFß) at the mRNA and protein levels in monocytic leukemia THP-1 cells. TGFß-mediated MCEMP1 upregulation results from the cooperation of SMAD3 and SP1 via concomitant binding to SMAD3/SP1 cis-regulatory elements within the MCEMP1 promoter. We also found that MCEMP1 regulates TGFß-mediated monocyte chemotaxis, adhesion, and migration. Our results suggest that MCEMP1 may regulate the migration and transition of monocytes to monocyte-derived alveolar macrophages during pulmonary fibrosis development and progression.NEW & NOTEWORTHY MCEMP1 is highly expressed in circulating classical monocytes and alveolar macrophages in IPF, is regulated by TGFß, and participates in the chemotaxis, adhesion, and migration of circulating monocytes by modulating the effect of TGFß in RHO activity.
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Fibrosis Pulmonar Idiopática , Macrófagos Alveolares , Humanos , Macrófagos Alveolares/metabolismo , Monocitos/metabolismo , Proteínas de la Membrana/metabolismo , Quimiotaxis , Mastocitos/metabolismo , Factor de Crecimiento Transformador beta/metabolismo , Fibrosis Pulmonar Idiopática/genética , Fibrosis Pulmonar Idiopática/metabolismoRESUMEN
Tyrosine kinase inhibitors (TKIs) target the signal transduction pathways of protein kinases by several modes of inhibition. Adverse effects are generally dose dependent, with certain side-effects unique to each drug. However, due to similarities in target sites, different classes of TKIs may have identical or overlapping side-effect profiles. This narrative review is an attempt to summarize the common and uncommon adverse effects of different classes of TKIs.
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Antineoplásicos , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Humanos , Antineoplásicos/efectos adversos , Proteínas Tirosina Quinasas , Transducción de Señal , Inhibidores de Proteínas Quinasas/efectos adversosRESUMEN
PURPOSE OF REVIEW: Pulmonary hypertension in sarcoidosis is a well known entity. Sarcoidosis-associated pulmonary hypertension (SAPH) incurs substantial morbidity and mortality. This review examines recent literatures published on epidemiology, prognosis and therapeutic management in SAPH. RECENT FINDINGS: Several registries have been published between 2017 and 2020. The consensus conclusion - SAPH is a harbinger for poor prognosis. Several factors were noted for predicting adverse outcome in SAPH like reduced 6-min walk distance and diffusing capacity for carbon monoxide. Given its adverse outcome, experts have now focused on methods for early screening of SAPH in sarcoid patients. The exploration of pulmonary vasodilator drugs in SAPH is ongoing. In recent times, trials have been published utilizing Macitentan and parenteral prostacyclin in severe SAPH. Although these trials show encouraging results, the evidence from these studies are limited to approve these agents as preferred drugs for treating SAPH. A large multicentric trial of drugs used for pulmonary arterial hypertension with meaningful, yet feasible, event driven endpoint is still lacking. Lately, interventional treatment by pulmonary artery balloon pulmonary angioplasty and stenting has gained traction for treating pulmonary artery stenosis and chronic thromboembolic pulmonary hypertension. However, the conclusion is still based on small cohorts or case series. SUMMARY: Several registries have highlighted SAPH portends an unfavorable consequence. On the contrary, no published guideline exists to treat SAPH. The precise role of immunosuppressive agents is unclear. The limited evidence favoring use of pulmonary vasodilators arise from small retrospective case series and/or single-center nonrandomized observational studies. Further multicenter randomized research is warranted to better define patient population to treat and how best to treat them.
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Hipertensión Pulmonar/fisiopatología , Sarcoidosis Pulmonar/fisiopatología , Antagonistas de los Receptores de Endotelina/uso terapéutico , Glucocorticoides/uso terapéutico , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Inmunosupresores/uso terapéutico , Pulmón/fisiopatología , Pronóstico , Hipertensión Arterial Pulmonar/diagnóstico , Hipertensión Arterial Pulmonar/tratamiento farmacológico , Hipertensión Arterial Pulmonar/etiología , Hipertensión Arterial Pulmonar/fisiopatología , Arteria Pulmonar/fisiopatología , Pirimidinas/uso terapéutico , Estudios Retrospectivos , Sarcoidosis/complicaciones , Sarcoidosis/fisiopatología , Sarcoidosis Pulmonar/complicaciones , Sulfonamidas/uso terapéutico , Vasodilatadores/uso terapéuticoRESUMEN
BACKGROUND: By comparing diagnoses made by pre-transplant surgical lung biopsy (SLB) and the final pathologic diagnosis of the explanted pathology (EP), we aimed to study the factors that could impact pathologic diagnoses in patients with interstitial lung disease (ILD). METHODS: We retrospectively reviewed the lung transplant database at Cleveland Clinic [01/01/2006-12/31/2013] to include all lung transplant recipients with a prior diagnosis of ILD. Two pulmonary pathologists independently reviewed each SLB and lung explant. The diagnoses were labeled as concordant (same diagnosis on SLB and explant) or discordant (diagnosis on SLB and explant were different) by consensus. RESULTS: Of 389 patients transplanted for ILD, 217 had an SLB before transplant. Pathological diagnoses were concordant in 190 patients (87.6%) [165 UIP (86.8%), 13 NSIP (6.8%), 8 CHP (4.2%) and 4 other diagnoses (2.1%). In 27 cases (12.4%), the diagnosis on SLB differed from EP. 8/27 were diagnosed with UIP on SLB and of these, 5 were re-classified as NSIP. 14/19 (73.7%) patients with a SLB diagnosis "other than UIP" were re-categorized as UIP based on explant. Discordant cases had a greater time between SLB and EP than concordant cases (1553 days vs 1248 days). CONCLUSIONS: The pathologic diagnosis of ILD by SLB prior to lung transplant is accurate in most patients, but may be misleading in a small subset of patients. The majority of discordant cases that were reclassified as UIP could be due to a sampling error, or perhaps, an increased time from the date of the SLB to transplant. Future studies examining how multidisciplinary consensus diagnosis affects this discordance are necessary.
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Enfermedades Pulmonares Intersticiales/patología , Trasplante de Pulmón , Pulmón/patología , Alveolitis Alérgica Extrínseca/diagnóstico , Alveolitis Alérgica Extrínseca/patología , Alveolitis Alérgica Extrínseca/cirugía , Biopsia , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/patología , Fibrosis Pulmonar Idiopática/cirugía , Pulmón/cirugía , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/cirugía , Masculino , Persona de Mediana Edad , Neumonectomía , Estudios RetrospectivosRESUMEN
Dyschromatosis universalis hereditaria (DUH) is a rare pigmentary disorder characterized by the presence of mottled hyperpigmented and hypopigmented macules over the trunk, extremities, and face. We have presented a case series comprised of six members of a family who had numerous hyperpigmented and hypopigmented macules distributed all over the body. Histological findings were suggestive of dyschromatosis universalis hereditaria.
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Familia , Trastornos de la Pigmentación/congénito , Enfermedades Cutáneas Genéticas/diagnóstico , Adolescente , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Linaje , Trastornos de la Pigmentación/diagnóstico , Trastornos de la Pigmentación/patología , Enfermedades Cutáneas Genéticas/patología , Adulto JovenRESUMEN
The utility and safety of ß-blockers in pulmonary hypertension is controversial. Anecdotal reports suggest that ß-blockers may be harmful in these patients. The aim of our study was to evaluate outcomes of ß-blocker use in pulmonary hypertension.We reviewed patients from our pulmonary hypertension registry between 2000 and 2011. Patients who continued to use ß-blockers were compared to those who never used ß-blockers for all-cause mortality, time to clinical worsening events, defined as death, lung transplantation and hospitalisation due to pulmonary hypertension. We also evaluated the effect of ß-blockers on 6-min walking distance and New York Heart Association (NYHA) functional class.133 patients used ß-blockers and 375 patients never used ß-blockers. Mean±sd age was 57±16â years and the median follow-up period was 78â months. Propensity-matched analysis showed that the adjusted odds ratio (95% CI) for mortality with ß-blocker use was 1.13 (0.69-1.82) and for clinical worsening events was 0.96 (0.55-1.68). No significant difference was noted in probability of survival and time to clinical worsening events. Patients on ß-blockers walked a shorter distance on follow-up 6â min walk test; follow-up NYHA class was similar between groups.Pulmonary hypertension patients receiving ß-blockers had a similar survival and time to clinical worsening events compared to patients not receiving them. Functional outcomes were similar, although ß-blocker use was associated with a tendency towards shorter walking distance.
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Antagonistas Adrenérgicos beta/efectos adversos , Antagonistas Adrenérgicos beta/uso terapéutico , Causas de Muerte , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/mortalidad , Adulto , Anciano , Estudios de Casos y Controles , Intervalos de Confianza , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Pronóstico , Presión Esfenoidal Pulmonar/efectos de los fármacos , Valores de Referencia , Sistema de Registros , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Estadísticas no Paramétricas , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
A 51-year-old otherwise healthy farmer presented with a 1-year history of numerous extremely itchy bumps on his skin. The lesions came in crops, were pinhead-sized, and subsequently enlarged to form nodules of varying sizes. There was no history of ocular or mucosal involvement or of spontaneous healing of any of the lesions. His medical history was unremarkable. There was neither any family history of similar illness nor any personal or family history of atopy or malignancy. He was previously treated with potent topical steroids and antihistamines without any appreciable benefit.
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Ciclofosfamida/uso terapéutico , Inmunosupresores/uso terapéutico , Queratoacantoma/diagnóstico , Humanos , Queratoacantoma/tratamiento farmacológico , Masculino , Persona de Mediana EdadRESUMEN
Trichoepitheliomas are epidermal appendageal hamartomas, which usually present as solitary lesions; rarely multiple lesions may be present, mainly involving the centrofacial skin symmetrically. We report herein an adolescent male patient with multiple trichoepitheliomas, linearly arranged and dermatomal, present since birth, along the left side of the neck.
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Cuello/patología , Neoplasias Cutáneas/patología , Adolescente , Humanos , Masculino , Cuello/cirugía , Neoplasias Cutáneas/cirugíaRESUMEN
Syringocystadenoma papilliferum is a rare benign adnexal tumor commonly located on the head and neck region and is usually associated with a nevus sebaceous. Linear lesions are uncommon and lesions on the breast are extremely rare. We report here a case of linear SCAP occurring de novo on the left breast of a 35-year-old healthy woman. Histopathology showed the characteristic papillary projections lined by double layer of cells inside epidermal invaginations.
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Neoplasias de la Mama/patología , Cistoadenoma/patología , Neoplasias Cutáneas/patología , Siringoma/patología , Adulto , Femenino , HumanosRESUMEN
Rationale: Hospital readmission within 30 days poses challenges for healthcare providers, policymakers, and patients because of its impact on care quality, costs, and outcomes. Patients with interstitial lung disease (ILD) are particularly affected by readmission, which is associated with increased morbidity and mortality and reduced quality of life. Because small sample sizes have hindered previous studies, this study seeks to address this gap in knowledge by examining a large-scale dataset. Objective: To determine the rate and probability of 30-day all-cause readmission and secondary outcomes in patients with coronavirus disease (COVID-19) or ILD admitted to the hospital. Methods: This study is a nested cohort study that used the PearlDiver patient records database. Adult patients (age ⩾18 yr) who were admitted to hospitals in 28 states in the United States with COVID-19 or ILD diagnoses were included. We defined and analyzed two separate cohorts in this study. The first cohort consisted of patients with COVID-19 and was later divided into two groups with or without a history of ILD. The second cohort consisted of patients with ILD and was later divided into groups with COVID-19 or with a non-COVID-19 pneumonia diagnosis at admission. We also studied two other subcohorts of patients with and without idiopathic pulmonary fibrosis within the second cohort. Propensity score matching was employed to match confounders between groups. The Kaplan-Meier log rank test was applied to compare the probabilities of outcomes. Results: We assessed the data of 2,286,775 patients with COVID-19 and 118,892 patients with ILD. We found that patients with COVID-19 with preexisting ILD had an odds ratio of 1.6 for 30-day all-cause readmission. Similarly, an odds ratio of 2.42 in readmission rates was observed among hospitalized individuals with ILD who contracted COVID-19 compared with those who were hospitalized for non-COVID-19 pneumonia. Our study also found a significantly higher probability of intensive care admission among patients in both cohorts. Conclusions: Patients with ILD face heightened rates of hospital readmissions, particularly when ILD is combined with COVID-19, resulting in adverse outcomes such as decreased quality of life and increased healthcare expenses. It is imperative to prioritize preventive measures against COVID-19 and establish effective postdischarge care strategies for patients with ILD.
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COVID-19 , Enfermedades Pulmonares Intersticiales , Neumonía , Adulto , Humanos , Estados Unidos/epidemiología , Readmisión del Paciente , Estudios de Cohortes , Calidad de Vida , Cuidados Posteriores , COVID-19/epidemiología , COVID-19/complicaciones , Alta del Paciente , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/terapia , Enfermedades Pulmonares Intersticiales/complicaciones , Neumonía/complicacionesRESUMEN
RATIONALE: Inhaled nitric oxide (iNO) has been shown to result in benefits in moderate to vigorous physical activity (MVPA) in patients with fibrotic interstitial lung disease (if-ILD) on supplemental oxygen in two independent trials. OBJECTIVE: This phase 3 randomized double-blind placebo-controlled study sought to validate the benefit of ambulatory iNO in patients with f-ILD requiring supplemental oxygen. METHODS: Patients with f-ILD on supplemental long-term oxygen were randomized in a 1:1 fashion to inhaled nitric oxide at 45 µg/kg ideal body weight/hour or placebo for 16 weeks. The primary outcome was the change from baseline to week 16 in MVPA assessed by accelerometry. Secondary outcomes included overall activity, six-minute walk distance and patient reported outcomes. MEASUREMENT AND MAIN RESULTS: 145 patients were enrolled; 75 were assigned to receive iNO and 70 to placebo. The change from baseline in MVPA at 16 weeks was -9.2 minute/day (SE 3.51) in the iNO45 group versus -3.7 (3.76) minute/day in the placebo group (difference, 5.5; P=0.265). No statistically significant differences between the two treatment arms were found for any of the secondary outcomes. A subgroup analysis of patients with intermediate or high probability of pulmonary hypertension on echocardiography did not demonstrate any benefit. The most common adverse events reported were respiratory tract infections, but the therapy was generally very well tolerated. CONCLUSIONS: There was no demonstrable benefit to iNO in patients with f-ILD on supplemental oxygen in daily physical activity assessed by actigraphy, a potential novel clinical trial endpoint. Clinical trial registration available at www. CLINICALTRIALS: gov, ID: NCT03267108.
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Natural arsenic pollution is a major global health problem. The two worst affected areas e Bangladesh and West Bengal, India. Arsenic is a well-documented human carcinogen that affects many organs including the skin. The authors sought to find out the clinical patterns of different malignant and premalignant conditions associated with chronic arsenicosis from drinking contaminated ground water in a group of patients from eastern India. This was a clinical observational study. Patients with chronic arsenicoses with suspected cutaneous malignancies for whom dermatology service was sought were enrolled in the study. A total of 24 patients (male to female ratio, 11:1; age range, 32-71 years; mean age, 52.2 years) were evaluated. Squamous cell carcinoma (SCC) was the commonest malignancies in our series, seen in 10 (41.7%) patients. This was followed by Bowen's disease (9 [37.5%]) and basal cell carcinoma (8 [33.3%]). Three patients (12.5%) had > 1 type of cutaneous malignancies. Multicentric lesions were seen in 3 cases. The most common site of involvement was the chest (8 [33.3%]). No statistically significant correlation was found between number of lesions and arsenic content in the hairs and nails of the patients.
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Intoxicación por Arsénico/complicaciones , Carcinoma Basocelular/inducido químicamente , Carcinoma de Células Escamosas/inducido químicamente , Agua Potable , Lesiones Precancerosas/inducido químicamente , Neoplasias Cutáneas/inducido químicamente , Adulto , Anciano , Enfermedad de Bowen/inducido químicamente , Enfermedad Crónica , Femenino , Dermatosis del Pie/inducido químicamente , Dermatosis de la Mano/inducido químicamente , Humanos , India , Queratosis/inducido químicamente , Masculino , Persona de Mediana EdadRESUMEN
Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. A minority of patients with sarcoidosis develop sarcoidosis-associated pulmonary fibrosis (SAPF), which may become progressive. Genetic profiles differ between patients with progressive and self-limiting disease. The mechanisms of fibrosis in SAPF are not fully understood, but SAPF is likely a distinct clinicopathological entity, rather than a continuum of acute inflammatory sarcoidosis. Risk factors for the development of SAPF have been identified; however, at present, it is not possible to make a robust prediction of risk for an individual patient. The bulk of fibrotic abnormalities in SAPF are located in the upper and middle zones of the lungs. A greater extent of SAPF on imaging is associated with a worse prognosis. Patients with SAPF are typically treated with corticosteroids, second-line agents such as methotrexate or azathioprine, or third-line agents such as tumour necrosis factor inhibitors. The antifibrotic drug nintedanib is an approved treatment for slowing the decline in lung function in patients with progressive fibrosing interstitial lung diseases, but more evidence is needed to assess its efficacy in SAPF. The management of patients with SAPF should include the identification and treatment of complications such as bronchiectasis and pulmonary hypertension. Further research is needed into the mechanisms underlying SAPF and biomarkers that predict its clinical course.
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Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Sarcoidosis , Humanos , Fibrosis Pulmonar/diagnóstico , Fibrosis Pulmonar/tratamiento farmacológico , Fibrosis Pulmonar/etiología , Pulmón , Sarcoidosis/complicaciones , Sarcoidosis/patología , Factores de Riesgo , Progresión de la EnfermedadRESUMEN
Background Though higher doses of terbinafine are often prescribed to treat dermatophyte infections, it is unknown if such doses are more effective than the conventional dose because comparative data are unavailable. Aim To compare the efficacy and safety of a once-daily dose of oral terbinafine 250 mg with 500 mg along with topical clotrimazole in the treatment of tinea infections. Methods A randomised, assessor-blinded, comparative study was carried out. Each group of subjects were administered either 250 mg or 500 mg oral terbinafine once daily for four weeks, along with topical clotrimazole. Clinical improvement was assessed after two weeks and again after four weeks from treatment initiation. Result A total of 60 patients with tinea corporis and cruris were randomised into two groups receiving either 250 mg (group A) or 500 mg (group B) oral terbinafine, along with clotrimazole cream in both groups. Baseline clinical parameters such as lesional activity (papules, vesicles and pustules), degree of erythema, scaling and severity of itching were comparable between both treatment arms. At the first and second follow-ups, no significant differences were found in the clinical parameters between the two groups. At the end of two weeks 13.8% of group A and 14.3% of group B and after 4 weeks 25.9% of group A and 33.3% of group B participants became KOH negative (P = 1.00 and 0.76, respectively). No significant difference in culture negativity was reported at the end of therapy (four weeks) between the two treatment arms (P = 0.78). Overall cure rates were 20% and 33.3% in the two treatment arms respectively at the end of the study (P = 0.82). Conclusion Oral terbinafine 250 mg daily yielded a poor cure rate in tinea cruris and corporis after 4 weeks of treatment and an increased dose of 500 mg did not have any additional benefit.
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Antifúngicos , Tiña , Humanos , Terbinafina/uso terapéutico , Clotrimazol/efectos adversos , Naftalenos , Tiña/diagnóstico , Tiña/tratamiento farmacológicoRESUMEN
Interstitial lung diseases (ILD) are a spectrum of disorders often complicated by pulmonary hypertension (PH) in its course. The pathophysiologic mechanism of WHO group 3 PH is different to other forms of PH. The advent of PH is a harbinger for adverse events like mortality and morbidity, implying that the PH component of disease expedites deteriorated clinical outcomes. In fact, WHO group 3 PH due to ILD has the worse prognosis among all groups of PH. Hence, early detection of PH by a comprehensive screening method is paramount. Given considerable overlap in clinical manifestations between ILD and PH, early detection of PH is often elusive. Despite, the treatment of PH due to ILD has been frustrating until recently. Clinical trials utilizing PAH-specific pulmonary vasodilators have been ongoing for years without desired results. Eventually, the INCREASE study (2018) demonstrated beneficial effect of inhaled Treprostinil to treat PH in ILD. In view of this pioneering development, a paradigm shift in clinical approach to this disease phenotype is happening. There is a renewed vigor to develop a well validated screening tool for early detection and management. Currently inhaled Treprostinil is the only FDA approved therapy to treat this phenotype, but emergence of a therapy has opened a plethora of research toward new drug developments. Regardless of all these recent developments, the overall outlook still remains grim in this condition. This review article dwells on the current state of knowledge of pre-capillary PH due to ILD, especially its diagnosis and management, the recent progresses, and future evolutions in this field.
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Background: Mast-Cell Expressed Membrane Protein-1 (MCEMP1) is higher in Idiopathic Pulmonary Fibrosis (IPF) patients with increased risk of death and poor outcomes. Here we seek to establish the mechanistic role of MCEMP1 in pulmonary fibrosis. Methods: MCEMP1 expression was analyzed by single-cell RNA sequencing, immunofluorescence in Peripheral Blood Mononuclear Cells (PBMC) as well as in lung tissues from IPF patients and controls. Chromatin Immunoprecipitation (ChiP) and Proximity Ligation Assay (PLA) were used to study the transcriptional regulation of MCEMP1 . Transient RNA interference and lentivirus transduction were used to knockdown and knock-in MCEMP1 in THP-1 cells to study chemotaxis, adhesion, and migration. Bulk RNA sequencing was used to identify the mechanisms by which MCEMP1 participates in monocyte function. Active RHO pull-down assay was used to validate bulk RNA sequencing results. Results: We identified increased MCEMP1 expression in classical monocytes and alveolar macrophages in IPF compared to controls. MCEMP1 was upregulated by TGFß at the mRNA and protein levels in THP-1. TGFß-mediated MCEMP1 upregulation results from the cooperation of SMAD3 and SP1 via concomitant binding to SMAD3/SP1 cis -regulatory elements within the MCEMP1 promoter. In terms of its function, we found that MCEMP1 regulates TGFß-mediated monocyte chemotaxis, adhesion, and migration. 400 differentially expressed genes were found to increase after TGFß stimulation of THP-1, further increased in MCEMP1 knock-in cells treated with TGFß and decreased in MCEMP1 knockdown cells treated with TGFß. GO annotation analysis of these genes showed enrichment for positive regulation of RHO GTPase activity and signal transduction. While TGFß enhanced RHO GTPase activity in THP-1 cells, this effect was attenuated following MCEMP1 knockdown. Conclusion: MCEMP1 is highly expressed in circulating classical monocytes and alveolar macrophages in IPF. MCEMP1 is regulated by TGFß and participates in the chemotaxis, adhesion, and migration of circulating monocytes by modulating the effect of TGFß in RHO activity. Our results suggest that MCEMP1 may regulate the migration and transition of monocytes to monocyte-derived alveolar macrophages during pulmonary fibrosis development and progression.
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A 55-year-old man presented with multiple gradually progressing asymptomatic swellings on his body for the preceding 6 months. He had no personal or family history of any skin disease. There was no systemic symptom apart from occasional constipation. Examination revealed multiple discrete, firm, nontender, skin-colored nodules of varying sizes, fixed to the skin but free from the underlying structures on his chest, abdomen, and back. The overlying skin of the nodules was erythematous at places (Figure 1). A solitary depigmented, nonanesthetic patch (measuring 3 cm x 3 cm) was noted around a central gray macule (4 mm x 4 mm) on his left shin (Figure 2). The surface of this lesion was otherwise normal. Wood's lamp examination of this area showed attenuation of pigmentation in the central area and total depigmentation surrounding it. No dyspigmented area was noted on Wood's lamp examination of the other areas. There was no abnormality of the orogenital mucosae. General examination revealed mild pallor and multiple discrete, nontender, firm lymph nodes, measuring 3 cm x 3 cm, attached to the skin in the left inguinal region. The overlying skin was normal. Ocular examination (including direct and indirect ophthalmoscopy) and otolaryngologic evaluation were normal. Proctoscopic examination revealed a reddish-black indurated mass at the right lateral wall of the lower third of the rectum. Examination of the other system was noncontributory. Complete hemogram showed mild anemia (hemoglobin % = 10 gm%) and raised ESR, (80 mm in the first hour; Westergren's method). Biochemistry panel was normal apart from raised levels of aspertate transaminase (78 U/L), alanine transaminase (68 U/L), alkaline phosphatase (386 U/L), and lactate dehydrogenase (692 U/L). Chest x-ray showed a rounded opacity in the left apical area suggestive of cannon-ball metastasis (Figure 3). Ultrasonography and computed tomography of the abdomen revealed multiple liver nodules suggestive of hepatic metastasis. Findings from omputed tomography of the brain and upper gastrointestinal endoscopy were normal. Colonoscopic examination did not reveal any colonic lesion. Fine needle aspiration cytology of the enlarged inguinal lymph node was suggestive of malignant melanoma (MM). Histopathologic examination of the excision biopsy specimen of a skin nodule showed a tumor mass in the dermis composed of nests of oval to polygonal cells with vesicular nuclei and large prominent nucleoli (Figure 4). Extensive areas of necrosis were also seen. The tumor cells contained brownish pigment that stained positively with Masson Fontana stain. Pearl's stain was negative. Excision biopsy of the depigmented patch on the left shin showed mild hyperkeratosis, proliferation of capillary-sized blood vessels in the dermis, and thick-walled blood vessels in the subcutaneous tissue (Figure 5). No cellular atypia or any other evidence of malignancy was noted in this specimen. Biopsy from the rectal growth was suggestive of MM. Tumor cells were positive for S100 and human melanin black 45. Based on the clinical presentation, histopathology, and laboratory investigations, a diagnosis of metastatic MM (stage IV: T(x)N(3)M(1c)) with unknown primary site was made. Treatment was started with injection dacarbazine 250 mg/m2 intravenous infusion daily for 5 days intended to be given every 3 weeks for palliation. The patient died 2 week after the first treatment with chemotherapy.
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Melanoma/patología , Neoplasias Primarias Desconocidas , Neoplasias Cutáneas/patología , Antineoplásicos Alquilantes/administración & dosificación , Antineoplásicos Alquilantes/uso terapéutico , Dacarbazina/administración & dosificación , Dacarbazina/uso terapéutico , Resultado Fatal , Humanos , Masculino , Melanoma/tratamiento farmacológico , Melanoma/secundario , Persona de Mediana Edad , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/secundarioRESUMEN
Syringocystadenoma papilliferum (SCAP) is a rare hamartoma with predominant apocrine differentiation. Clinically, presenting as solitary nodule, nodular plaque, or linear nodules, SCAP usually occurs over the head and neck region in children. Out of these three morphological presentations, the linear type is the most rare. We report herein a case of a 36-year-old man with linear SCAP over the right inguinal fold, for its rarity. Besides the typical histologic features, a marked increase in lesional mast cell concentration as compared to a control specimen obtained from an unaffected site and a strikingly raised number of spreading and degranulated mast cells were demonstrated.
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Adenoma de las Glándulas Sudoríparas/patología , Mastocitos/patología , Neoplasias de las Glándulas Sudoríparas/patología , Adulto , Ingle , Humanos , MasculinoRESUMEN
Background: Psoriasis is a common inflammatory dermatological condition and affects 2-3% population worldwide. Psoriasis area and severity index (PASI) and body surface area (BSA) are two commonly used scales used to measure disease severity in psoriasis patients. However, these scales are plagued by weaknesses as inter-observer variation and insufficient evaluation of micro-vascular inflammation. Thus, it is necessary to have an objective and simple measure of the severity of inflammation. The ratio of neutrophils-to-lymphocytes (NLR) and platelets-to-lymphocytes (PLR) are simple and inexpensive markers of systemic inflammatory response that can be measured as part of a complete blood count and are already used in the setting of inflammatory diseases. The utility of the NLR and PLR in psoriasis however, remains relatively unexplored. Aims and Objectives: The present study was undertaken to assess if NLR, PLR and C-reactive protein were altered in chronic plaque psoriasis patients as compared to controls and also to determine correlation of NLR and PLR values with disease severity as measured by PASI. Methods: This case control study consisted of equal numbers (45 each) chronic plaque psoriasis patients and control subjects. The subjects were evaluated by way of history taking, clinical and blood examination. Thereafter, the results were tabulated and examined statistically. Results: Our study results indicate that psoriasis patients tended to have a higher neutrophil count, lymphocyte count, NLR and C-reactive protein in comparison the control subjects (P < 0.05). Conclusion: We concluded that such easily available and low cost indices of systemic inflammation are raised in psoriasis patients and are positively correlated with the severity of involvement. They can thus not only be used to monitor the effect of systemic drugs in psoriasis.
RESUMEN
Common wart, also known as verruca vulgaris is characterized by focal proliferation of keratinocytes caused by multiple strains of human papilloma virus (HPV). Conventional treatments like chemical cautery, cryotherapy, electro-cautery, etc often fail to cure verruca satisfactorily. The present work was a randomized, parallel-group, non-inferiority clinical trial with an objective of comparing the effectiveness and safety of subcutaneous MMR versus intralesional MMR vaccine in the treatment of multiple warts. Method: Consenting patients of both sexes of 18-65 years age, who have viral warts and did not receive anti-wart treatment in the last 4 weeks and devoid of any active bacterial or viral skin diseases were included in the study. Interventions: Eligible patients were randomized into either group A (receiving 0.3 ml of intralesional MMR) or group B (receiving 0.5 ml of subcutaneous MMR). A total of three injections were administered at two weeks interval. Outcome Measure: The response was considered complete if there was disappearance of the wart(s) and return of the normal skin markings, partial if the wart(s) was regression in size by 50-99% and no response if there was be 0-49% decrease in wart size. Results: Thirty patients were recruited in each group; 5 of group A and 3 of group B were lost to follow up. Modified intention to treat analysis was performed, so, the last observation of such patients was carried forward and all 60 participants were analysed. Number of warts and size of the largest wart were declined significantly (P < 0.001 and P = 0.001 respectively) in both the treatment arms. No significant difference between two groups were seen. Complete clearance including distant lesions was achieved in 22 patients; 12 (48%) in group A and 10 (37.04%) in group B, but the final outcome at the end of the study showed no significant difference between the two t groups. (P = 0.64). Adverse Events: Only one patient had developed mild fever with tender, enlarged parotid gland after first injection of subcutaneous MMR which resolved within two weeks. Conclusion: Efficacy and safety profile of Subcutaneous and intralesional MMR were almost same. Both can be considered as safe and cost effective treatment of warts while the subcutaneous route may be easier to administer.