Department of Pediatrics, The Division of Diabetes and Endocrinology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, TX, USA.
Pediatr Diabetes. 2024;2024. doi: 10.1155/2024/5907924. Epub 2024 Mar 4.
ketosis-prone diabetes (KPD) in adults is characterized by presentation with diabetic ketoacidosis (DKA), negative islet autoantibodies, and preserved -cell function in persons with a phenotype of obesity-associated type 2 diabetes (T2D). The prevalence of KPD has not been evaluated in children. We investigated children with DKA at "T2D" onset and determined the prevalence and characteristics of pediatric KPD within this cohort.
We reviewed the records of 716 children with T2D at a large academic hospital and compared clinical characteristics of those with and without DKA at onset. In the latter group, we identified patients with KPD using criteria of the Rare and Atypical Diabetes Network (RADIANT) and defined its prevalence and characteristics.
Mean age at diagnosis was 13.7 ± 2.4 years: 63% female; 59% Hispanic, 29% African American, 9% non-Hispanic White, and 3% other. Fifty-six (7.8%) presented with DKA at diagnosis and lacked islet autoantibodies. Children presenting with DKA were older and had lower C-peptide and higher glucose concentrations than those without DKA. Twenty-five children with DKA (45%) met RADIANT KPD criteria. They were predominantly male (64%), African American or Hispanic (96%), with substantial C-peptide (1.3 ± 0.7 ng/mL) at presentation with DKA and excellent long-term glycemic control (HbA1c 6.6% ± 1.9% at follow-up (median 1.3 years postdiagnosis)).
In children with a clinical phenotype of T2D and DKA at diagnosis, approximately half meet criteria for KPD. They manifest the key characteristics of obesity, preserved -cell function, male predominance, and potential to discontinue insulin therapy, similar to adults with KPD.
成人酮症倾向型糖尿病(KPD)的特点是表现为糖尿病酮症酸中毒(DKA)、阴性胰岛自身抗体以及肥胖相关 2 型糖尿病(T2D)表型患者的β细胞功能保留。在儿童中尚未评估 KPD 的患病率。我们研究了以 DKA 起病的儿童,并在该队列中确定了儿科 KPD 的患病率和特征。
我们回顾了一家大型学术医院的 716 名 T2D 患儿的记录,并比较了起病时伴有和不伴有 DKA 的患儿的临床特征。在后一组中,我们使用罕见和非典型糖尿病网络(RADIANT)的标准确定了 KPD 患者,并定义了其患病率和特征。
诊断时的平均年龄为 13.7 ± 2.4 岁:63%为女性;59%为西班牙裔,29%为非裔美国人,9%为非西班牙裔白人,3%为其他种族。56 名(7.8%)患儿在诊断时出现 DKA,且缺乏胰岛自身抗体。与不伴 DKA 的患儿相比,出现 DKA 的患儿年龄更大,C 肽和血糖浓度更低。25 名 DKA 患儿(45%)符合 RADIANT KPD 标准。他们主要为男性(64%),非裔美国人或西班牙裔(96%),在 DKA 发病时 C 肽水平较高(1.3 ± 0.7 ng/mL),且长期血糖控制良好(HbA1c 6.6% ± 1.9%,随访时(诊断后中位数 1.3 年))。
在以 T2D 为临床表型且起病时伴有 DKA 的儿童中,约有一半符合 KPD 标准。他们表现出肥胖、β细胞功能保留、男性优势和潜在的胰岛素治疗停药等关键特征,与成人 KPD 相似。