Polyglandular Syndrome Type III and Severe Peripheral Neuropathy: An Unusual Association

Introduction: Polyglandular syndrome is characterized by the association of autoimmune, organ-specific, endocrine and non-endocrine diseases. Objective: To present a case of polyglandular syndrome type III (b) accompanied by pernicious anemia and autoimmune thyroiditis. Method: Report the clinical case of a young patient that developed progressive and disabling peripheral neuropathy framework, triggered by vitamin B12 deficiency. Discussion: It was proven that atrophic gastritis with positive intrinsic anti-factor was responsible for the framework of pernicious anemia, which in turn dangerously reduced the serum levels of vitamin B12, leading to myelopathy. There was a progressive neurological improvement after parenteral cyanocobalamin replacement, keeping the patient at ambulatory, under maintenance therapy. Conclusion: The importance of suspecting on polyglandular syndrome in the presence of autoimmune frameworks is important, especially those involving diabetes, thyroiditis, atrophic gastritis, pernicious anemia, vitiligo, among others

Escalas de Ramsay e Richmond são equivalentes para a avaliação do nível de sedação em pacientes gravemente enfermos Ramsay and Richmond's scores are equivalent to assessment sedation level on critical patients

OBJETIVO: O objetivo principal deste estudo foi comparar o desempenho das escalas de sedação de Ramsay e Richmond em pacientes críticos sob ventilação mecânica em um hospital universitário. MÉTODOS: Estudo prospectivo onde foram incluídos todos os pacientes sob ventilação mecânica com pelo menos 48 horas de internação, durante quatro meses, totalizando 45 pacientes. Foram avaliados diariamente a modalidade de sedação, dose dos sedativos e analgésicos e o nível de sedação através das escalas de Ramsay e Richmond. O teste T de Student, os índices de correlação de Pearson e Spearman, e a elaboração de curvas Receiver Operating Characteristic (ROC) foram utilizados para a análise estatística. RESULTADOS: A mortalidade geral observada foi de 60%. Nesta série, o tempo de sedação e a dose de sedativos utilizada não se correlacionaram com a mortalidade. Sedação profunda (Ramsay > 4 ou Richmond < -3) correlacionou-se positivamente com uma maior probabilidade de morte, com uma área sob a curva (ASC) > 0,78. Níveis adequados de sedação (Ramsay 2 a 4 ou Richmond 0 a -3) correlacionaram-se sensivelmente à probabilidade de sobrevivência, com uma ASC > 0,80. Em 63 evoluções (8,64%) foram observados níveis baixos de sedação, porém não se evidenciou nenhuma correlação entre a ocorrência de agitação e prognósticos desfavoráveis. Houve uma boa correlação entre as escalas Ramsay e Richmond (Pearson > 0,810 - p<0,0001). CONCLUSÃO: Neste estudo, as escalas de Ramsay e Richmond mostraram-se equivalentes para a avaliação de sedações profunda, insuficiente e adequada e ambos demonstraram boa correlação com mortalidade em pacientes excessivamente sedados.<br>OBJECTIVE: The main purpose of this study was to compare performance of the Ramsay and Richmond sedation scores on mechanically ventilated critically ill patients, in a university-affiliated hospital. METHODS: This was a 4-month prospective study, which included a total of 45 patients mechanically ventilated, with at least 48 hours stay in the intensive care unit. Each patient was assessed daily for sedation mode, sedative and analgesic doses and sedation level using the Ramsay and Richmond scores. Statistical analysis was made using Student's t-test, Pearson's and Spearman's correlation, and constructing ROC-curves. RESULTS: A high general mortality of 60% was observed. The length of sedation and daily dose of medication did not correlate with mortality. Deep sedation (Ramsay > 4 or Richmond < -3) was positively correlated with probability of death with an AUC > 0.78. An adequate level of sedation (Ramsay 2 to 4 or Richmond 0 to -3) was sensitively correlated with probability of survival with an AUC > 0.80. A low level of sedation was observed in 63 days evaluated (8.64%), and no correlation was found between occurrence of agitation and unfavorable outcomes. Correlation between Ramsay and Richmond scores (Pearson's > 0.810 - p<0.0001) was good. CONCLUSION: In this study, Ramsay and Richmond sedation scores were similar for the assessment of deep, insufficient and adequate sedation. Both have good correlation with mortality in over sedated patients