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Monoclonal gammopathy-related peripheral neuropathies encompass a spectrum of clinical presentations in which the monoclonal protein directly damages the tissues, including the peripheral nervous system. Given the prevalence of both peripheral neuropathy and monoclonal gammopathy in the general population, these conditions may overlap in clinical practice, posing a challenge for clinicians in determining causality. Therefore, a comprehensive understanding of primary clinical syndromes and their neurophysiological patterns is of great importance for accurate differential diagnoses and effective treatment strategies. In this article, we examine the main forms of monoclonal gammopathies that affect the peripheral nerve. We explore the clinical and electrophysiological aspects and their correlation with each syndrome's corresponding monoclonal protein type. This knowledge is essential for healthcare professionals to diagnose better and manage patients presenting with monoclonal gammopathy-related peripheral nervous system involvement.
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Paraproteinemias , Enfermedades del Sistema Nervioso Periférico , Humanos , Paraproteinemias/complicaciones , Paraproteinemias/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/etiología , Enfermedades del Sistema Nervioso Periférico/fisiopatologíaRESUMEN
BACKGROUND: Steroid-refractory acute graft-vs.-host disease (SR-aGVHD) is a complication of allogeneic hematopoietic stem cell transplantation with a dismal prognosis and for which there is no consensus-based second-line therapy. Ruxolitinib is not easily accessible in many countries. A possible therapy is the administration of mesenchymal stromal cells (MSCs). METHODS: In this retrospective study, 52 patients with severe SR-aGVHD were treated with MSCs from umbilical cord (UC-MSCs) in nine institutions. RESULTS: The median (range) age was 12.5 (0.3-65) years and the mean ± SD dose (×106/kg) was 4.73 ± 1.3 per infusion (median of four infusions). Overall (OR) and complete response (CR) rates on day 28 were 63.5% and 36.6%, respectively. Children (n = 35) had better OR (71.5% vs. 47.1%, p = 0.12), CR (48.6% vs. 11.8%, p = 0.03), overall survival (p = 0.0006), and relapse-free survival (p = 0.0014) than adults (n = 17). Acute adverse events (all of them mild or moderate) were detected in 32.7% of patients, with no significant difference in children and adult groups (p = 1.0). CONCLUSIONS: UC-MSCs are a feasible alternative therapy for SR-aGVHD, especially in children. The safety profile is favorable.
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BACKGROUND AND AIMS: Over 4 million deaths from coronavirus disease (COVID)-19 have been reported in the world. Several biomarkers have been identified that predict disease severity, but there is still a need to identify biomarkers for death risk in severe COVID-19. We aim to define amongst the biomarkers already identified those which are mostly associated with increased death rate in patients with severe COVID-19. METHODS: In this retrospective study conducted in three public hospitals linked to the Medical School of Ribeirão Preto, Brazil, patients with severe COVID-19 were evaluated regarding biomarkers (neutrophil-to-lymphocyte ratio-NLR, D-dimer, fibrinogen) of death risk, obtained before administration of corticosteroids. RESULTS: Thirty-nine (32.8%) of the 119 patients included (104 [87.4%] on mechanical ventilation) died during hospitalization. Non-survivor group had higher median (range) NLR (12.63 [2.6-115] vs 7.43 [0.43-31.8]; P = .001), D-dimer (2.17 [0.27-20.00] vs 1.57 [0.28-20.00]; P = .03), but lower fibrinogen (631 [353-1078] vs 705 [407-1200]; P = .02). The group with NLR ≥ 10 and D-dimer ≥ 2 µg/mL had a higher death risk than the group with NLR < 10 and D-dimer < 2 µg/mL (OR: 5.39; CI 95%: 1.5-19.42; P = .01). CONCLUSION: High NLR and D-dimer, especially when combined, are predictors of death risk for patients with severe COVID-19 and should be incorporated into their evaluation.
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INTRODUCTION: The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the inability of antidiuretic hormone (ADH) suppression, compromising the mechanisms of water excretion and urinary concentration. It manifests as hyponatremia and its symptoms, especially neurological. There are many causes that trigger such disease, notably: central nervous system disorders, malignant neoplasm, drugs and others. CASE REPORT: A 65 years female hypertensive patient presented clinical and laboratory manifestations of hyponatremia due to SIADH. It happened twice under use of herbal medication for osteoarthritis treatment. DISCUSSION: The drug-related hyponatremia can be triggered by direct effect of the drug or by association with SIADH. The clinical manifestations presented could have been related to psychiatric condition and may have severe outcome if not properly diagnosed. The association of an herbal medicine to SIADH could be confirmed after a new episode of hyponatremia related to Harpagophytum procumbers reintroduction. Our literature review did not find this herbal medicine associated with SIADH, so far. CONCLUSION: SIADH may be caused by herbal medicine described from now on their association in the literature.
Asunto(s)
Harpagophytum , Síndrome de Secreción Inadecuada de ADH/inducido químicamente , Fitoterapia/efectos adversos , Preparaciones de Plantas/efectos adversos , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Abstract Introduction: The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the inability of antidiuretic hormone (ADH) suppression, compromising the mechanisms of water excretion and urinary concentration. It manifests as hyponatremia and its symptoms, especially neurological. There are many causes that trigger such disease, notably: central nervous system disorders, malignant neoplasm, drugs and others. Case Report: A 65 years female hypertensive patient presented clinical and laboratory manifestations of hyponatremia due to SIADH. It happened twice under use of herbal medication for osteoarthritis treatment. Discussion: The drug-related hyponatremia can be triggered by direct effect of the drug or by association with SIADH. The clinical manifestations presented could have been related to psychiatric condition and may have severe outcome if not properly diagnosed. The association of an herbal medicine to SIADH could be confirmed after a new episode of hyponatremia related to Harpagophytum procumbers reintroduction. Our literature review did not find this herbal medicine associated with SIADH, so far. Conclusion: SIADH may be caused by herbal medicine described from now on their association in the literature.
Resumo Introdução: A síndrome da secreção inapropriada do hormônio antidiurético (SIADH) consiste na incapacidade de supressão do hormônio antidiurético (ADH), comprometendo os mecanismos de excreção da água e concentração urinária. Possui como manifestações a hiponatremia e seus sintomas, sobretudo neurológicos. Há variadas causas que desencadeiam tal distúrbio, a se destacarem: distúrbios do sistema nervoso central, neoplasias malignas e drogas, dentre outros. Relato de Caso: Paciente feminina, 65 anos, hipertensa, apresentando manifestações clínicas e laboratoriais correspondentes à hiponatremia. O fato ocorreu em duas ocasiões em vigência de medicação fitoterápica para tratamento de osteoartrite. Discussão: A hiponatremia relacionada às drogas pode ser provocada pelo efeito direto do medicamento ou por desencadear SIADH. As manifestações clínicas apresentadas poderiam ter sido atribuídas a um quadro psiquiátrico, o que poderia ter desfecho grave, caso não diagnosticada corretamente. A associação de um fitoterápico à SIADH pôde ser confirmada após novo episódio de hiponatremia relacionado à reintrodução do Harpagophytum procumbers. Nossa revisão da literatura não encontrou este fitoterápico associado à SIADH, até o momento. Conclusão: SIADH pode ser ocasionada por medicamento fitoterápico doravante descrita sua associação na literatura.