RESUMEN
Nonkeratinizing squamous cell carcinoma (NKSCC) of the sinonasal tract is a rare malignancy that infrequently presents with cutaneous extension. This case describes an unusual instance of extensive facial skin involvement in an elderly male with multiple comorbidities, diagnosed with a biopsy-proven NKSCC. The tumor was p16 positive, suggesting an HPV-related etiology. This case emphasizes the critical role of dermatologists in the early diagnosis and multidisciplinary management of such aggressive tumors. It highlights the necessity of including NKSCC in the differential diagnosis of destructive facial tumors. Dermatologists should remain vigilant for rare presentations and employ early biopsy and histopathological examinations to facilitate prompt multidisciplinary intervention. Recognizing p16 positivity can indicate a human papillomavirus (HPV)-related etiology, potentially influencing prognosis and management strategies.
RESUMEN
Multicentric reticulohistiocytosis (MRH) is a rare type of non-Langerhans cell histiocytosis characterized by coral-toned papules with predilection for dorsal surfaces in addition to severe arthropathy. It sometimes proves difficult to differentiate these joint and skin findings clinically from certain rheumatologic diseases, primarily dermatomyositis. Herein, we present an 82-year-old woman who presented with the clinical findings described above and was subsequently diagnosed with MRH after biopsy and review of relevant clinical history. Because about 25% of patients diagnosed with MRH have an underlying occult malignancy, our patient underwent a complete malignancy workup that was negative. She was treated with systemic corticosteroids and methotrexate, which resulted in an improvement of the arthritis and constitutional symptoms. This case demonstrates that in patients with both rheumatologic and dermatologic symptoms, particularly on acral surfaces, MRH must be a diagnostic consideration. Identifying this disease early in its course can prevent negative consequences for the patients, specifically arthritis mutilans and upper airway involvement.
Asunto(s)
Enfermedades del Tejido Conjuntivo/diagnóstico , Dermatomiositis/diagnóstico , Histiocitosis de Células no Langerhans/diagnóstico , Anciano de 80 o más Años , Fármacos Dermatológicos/uso terapéutico , Diagnóstico Diferencial , Femenino , Glucocorticoides/uso terapéutico , Histiocitosis de Células no Langerhans/tratamiento farmacológico , Histiocitosis de Células no Langerhans/patología , Humanos , Metotrexato/uso terapéutico , Prednisona/uso terapéuticoRESUMEN
Delayed emergence from anesthesia was previously reported in a case study of a child with Glycine Encephalopathy. To investigate the neural basis of this delayed emergence, we developed a zebrafish glial glycine transporter (glyt1 - / -) mutant model. We compared locomotor behaviors; dose-response curves for tricaine, ketamine, and 2,6-diisopropylphenol (propofol); time to emergence from these anesthetics; and time to emergence from propofol after craniotomy in glyt1-/- mutants and their siblings. To identify differentially active brain regions in glyt1-/- mutants, we used pERK immunohistochemistry as a proxy for brain-wide neuronal activity. We show that glyt1-/- mutants initiated normal bouts of movement less frequently indicating lethargy-like behaviors. Despite similar anesthesia dose-response curves, glyt1-/- mutants took over twice as long as their siblings to emerge from ketamine or propofol, mimicking findings from the human case study. Reducing glycine levels rescued timely emergence in glyt1-/- mutants, pointing to a causal role for elevated glycine. Brain-wide pERK staining showed elevated activity in hypnotic brain regions in glyt1-/- mutants under baseline conditions and a delay in sensorimotor integration during emergence from anesthesia. Our study links elevated activity in preoptic brain regions and reduced sensorimotor integration to lethargy-like behaviors and delayed emergence from propofol in glyt1-/- mutants.
Asunto(s)
Retraso en el Despertar Posanestésico/genética , Proteínas de Transporte de Glicina en la Membrana Plasmática/genética , Glicina/metabolismo , Hiperglicinemia no Cetósica/genética , Neuronas/metabolismo , Área Preóptica/metabolismo , Proteínas de Pez Cebra/genética , Aminobenzoatos , Anestesia General , Anestésicos , Animales , Animales Modificados Genéticamente , Craneotomía , Retraso en el Despertar Posanestésico/metabolismo , Retraso en el Despertar Posanestésico/fisiopatología , Retraso en el Despertar Posanestésico/prevención & control , Modelos Animales de Enfermedad , Expresión Génica , Glicina/farmacología , Proteínas de Transporte de Glicina en la Membrana Plasmática/deficiencia , Hiperglicinemia no Cetósica/tratamiento farmacológico , Hiperglicinemia no Cetósica/metabolismo , Hiperglicinemia no Cetósica/fisiopatología , Ketamina , Locomoción/fisiología , Neuronas/efectos de los fármacos , Neuronas/patología , Área Preóptica/efectos de los fármacos , Área Preóptica/patología , Propofol , Pez Cebra , Proteínas de Pez Cebra/deficiencia , eIF-2 Quinasa/genética , eIF-2 Quinasa/metabolismoAsunto(s)
Antagonistas Colinérgicos/administración & dosificación , Glicopirrolato/administración & dosificación , Pénfigo Familiar Benigno/tratamiento farmacológico , Administración Tópica , Antagonistas Colinérgicos/efectos adversos , Estreñimiento/inducido químicamente , Esquema de Medicación , Glicopirrolato/efectos adversos , Humanos , Masculino , Persona de Mediana Edad , Pénfigo Familiar Benigno/patología , Escroto/patología , Xerostomía/inducido químicamenteRESUMEN
IMPORTANCE: Clear and comprehensive descriptions of clinical trial interventions are necessary to translate new results into clinical practice. The TIDieR checklist was developed to be a minimum set of key items considered essential to high-quality reporting of clinical trial interventions. OBJECTIVE: To determine the quality of reporting of recent Food and Drug Administration (FDA)-approved oncology interventions. DESIGN: Cross-sectional investigation. SETTING/PARTICIPANTS/INTERVENTION: Recent, FDA-approved haematology/oncology anticancer interventions. MAIN OUTCOME MEASURE: Quality of reporting. RESULTS: Across all included trials (n=96), a median of 8-9 (out of 12) TIDieR items were reported. Seven TIDieR items had >90% adherence, including individual-level and study-level modifications of drugs and dosing schedules. Three items were less often reported: intervention provider, including training and expertise (7/192, 3.6%); trial institution infrastructure (0/192, 0.0%); and how intervention compliance was assessed (59/192, 30.7%). Publication of a protocol improved intervention reporting (p<0.001). CONCLUSIONS: In this analysis of clinical trials of recent, FDA-approved anticancer interventions, we found good adherence to the TIDieR checklist. These studies were homogeneous in their structure and included information; some TIDieR items were always or never/rarely reported. Clinical trial effect sizes may not translate to real-world practice for a number of reasons. Thus, to aid the translation of trial effect sizes to real-world practice, we recommend authors adhere to the TIDieR checklist and describe the infrastructure of trial centres and describe who provided the intervention, along with their expertise.