SUMMARY Ketosis-prone type 2 diabetes (KPD) is an emerging form of diabetes mellitus characterized by unprovoked ketoacidosis, absence of autoimmunity and beta-cell dysfunction. The KPD may improve after initial glycemic compensation and evolve to exogenous insulin independence, most cases were observed in populations with African or Hispanic backgrounds. We reviewed the literature on KPD and, to date, only one case of KPD has been described in Brazil's multi-ethnic population. A group of adult Brazilian KPD patients without autoimmunity and insulinopenia was identified for this study. We report a retrospective study of four KPD cases (3 males) evaluated in southeast Brazil, the patients were overweight or obese, age between the third and fifth decades of life, had a family history of type 2 diabetes, hyperglycemia (809.5 ± 344.2 mg/dL), acidosis (pH 7.21 ± 0.07; normal range (nr): 7.35-7.45 and bicarbonate 9.1 ± 6.2; nr: 22-26 mEq/mL), ketonuria (142.5 ± 114.4 mg/dL; nr: absence), absence of glutamic acid decarboxylase antibodies (GAD-65), and beta-cell function reserve (C-peptide 1.19 ± 0.53 ng/mL - nr: 1.1-4.4 ng/mL) on diagnosis. After glycemic compensation, there was increase of C-peptide (2.21 ± 0.41) indicating the recovery of beta-cell function and the time to insulin independence was 7.7 ± 3.5 months. They evolved after the period of glucotoxicity with insulin withdrawal and could be treated with oral antidiabetic therapy. This is the first case series of KPD described in Brazil being characterized by ketoacidosis at diagnosis, absence of autoimmunity, recovery of beta-cell function and insulin independence.

FUNDAMENTO: A aterosclerose ocorre mais cedo em pacientes com diabetes mellitus tipo 1 (DM-1) e a doença arterial coronariana (DAC) constitui a mais importante causa de morte. OBJETIVO: Avaliar a prevalência e as características anatômicas da DAC em pacientes com DM-1 e insuficiência renal crônica, submetidos à diálise. MÉTODOS: Este é um estudo descritivo de 20 pacientes com DM-1 submetidos à diálise sem DAC conhecida. A DAC foi avaliada através de angiografia coronariana quantitativa (ACQ) e ultra-som intravascular (USIV). A ACQ foi realizada em todas as lesões >30%, visualmente Todos os segmentos proximais de 18 mm das artérias coronárias foram analisados por USIV. Todos os outros segmentos coronarianos com estenose >30% também foram analisados. RESULTADOS: A angiografia detectou 29 lesões >30% em 15 pacientes (75%). Onze (55%) das lesões eram >50% e 10 (50%) >70%. Treze pacientes tiveram as 3 principais artérias avaliadas pelo USIV. A aterosclerose estava presente em todos os pacientes e em todos os 51 segmentos proximais de 18 mm analisados. Esses segmentos significam que a medida do diâmetro dos vasos apresentava-se significantemente maior no USIV do que na ACQ, em todos os vasos. As imagens do ISIV de 25 (86,2%) das 29 lesões >30% foram obtidas. Placas fibróticas eram comuns (48%) e 60% apresentavam remodelamento intermediário de vasos. CONCLUSÃO: A DAC estava presente em todos os vasos de todos os pacientes com diabete tipo 1 submetidos a hemodiálise. Esses achados estão de acordo com outros estudos de autópsia, angiografia e USIV. Além disso, eles indicam a necessidade de estudos adicionais epidemiológicos e de imagem, para um melhor entendimento e tratamento de uma condição clínica complexa e grave que afeta jovens indivíduos.

BACKGROUND: In patients with type 1 diabetes mellitus, atherosclerosis occurs earlier in life and coronary artery disease (CAD) constitutes the major cause of death. OBJECTIVE: Evaluate the prevalence and anatomic characteristics of coronary artery disease (CAD) in type 1 diabetic patients with chronic renal failure undergoing hemodialysis. METHODS: This is a descriptive study of 20 patients with type 1 diabetes mellitus undergoing hemodialysis without known CAD. CAD was assessed by quantitative coronary angiography (QCA) and intravascular ultrasound (IVUS). QCA was performed in all lesions >30%, visually. All proximal 18-mm segments of the coronary arteries were analyzed by IVUS. All other coronary segments with stenosis >30% were also analyzed. RESULTS: Angiography detected 29 lesions >30% in 15 patients (75%). Eleven (55%) of the lesions were >50% and 10 (50%) >70%. Thirteen patients had all 3 major arteries interrogated by IVUS. Atherosclerosis was present in all patients and in all 51 proximal 18-mm segments analyzed. The mean vessel diameter of these segments was significantly larger at the IVUS than at the QCA, for all vessels. IVUS images of 25 (86.2%) of the 29 lesions >30% were obtained. Fibrotic plaques were common (48%) and 60% had intermediate vessel remodeling. CONCLUSION: CAD was present in all vessels of all type 1 diabetic patients undergoing hemodialysis. These findings are in agreement with other autopsy, angiography and IVUS studies. Additionally, they indicate the need for additional epidemiological and imaging studies to better understand and treat such a complex and serious clinical condition affecting young people.

FUNDAMENTO: La aterosclerosis ocurre más temprano en pacientes con diabetes mellitus tipo 1 (DM-1) y la enfermedad arterial coronaria (EAC) constituye la más importante causa de muerte. OBJETIVO: Evaluar la prevalencia y las características anatómicas de la EAC en pacientes con DM-1 e insuficiencia renal crónica, sometidos a diálisis. MÉTODOS: Este es un estudio descriptivo de 20 pacientes con DM-1 sometidos a diálisis sin EAC conocida. La EAC se evaluó mediante angiografía coronaria cuantitativa (ACC) y ultrasonido intravascular (IVUS). La ACC se realizó en todas las lesiones >30%, se llevó a cabo el análisis visual por IVUS en todos los segmentos proximales de 18 mm de las arterias coronarias. También se analizaron todos los otros segmentos coronarios con estenosis >30%. RESULTADOS: La angiografía detectó 29 lesiones >30% en 15 pacientes (75%). Once (55%) de las lesiones eran >50% y 10 (50%) >70%. Trece pacientes tuvieron las tres arterias principales evaluadas por el IVUS. La aterosclerosis estaba presente en todos los pacientes y en todos los 51 segmentos proximales de 18 mm analizados. Esos segmentos significan que la medición del diámetro de los vasos resultaba significantemente mayor en el IVUS que en la ACC, en todos los vasos. De las imágenes del IVUS se obtuvieron 25 (86,2%) de las 29 lesiones >30%. Placas fibróticas eran comunes (48%) y el 60% presentaban remodelamiento intermediario de vasos. CONCLUSIÓN: La EAC estaba presente en todos los vasos de todos los pacientes con diabetes tipo 1 sometidos a hemodiálisis. Esos hallazgos están de acuerdo con otros estudios de autopsia, angiografía e IVUS. Además de ello, indican la necesidad de estudios adicionales epidemiológicos y de imagen, para una mejor comprensión y tratamiento de una condición clínica compleja y severa que afecta a jóvenes individuos.