Harinarayanan Shyam, Merritt Taylor, White Perrin C
Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX 75390-9063, USA.
JCEM Case Rep. 2025 May 21;3(7):luaf114. doi: 10.1210/jcemcr/luaf114. eCollection 2025 Jul.
Cystic fibrosis (CF), a genetic disorder caused by pathogenic variants in the gene, is associated with various complications including cystic fibrosis-related diabetes (CFRD). CFRD is an entity distinct from type 1 or type 2 diabetes. We report a rare case of diabetic ketoacidosis (DKA) in a pediatric patient with CFRD, occurring during a significant pulmonary exacerbation. The patient's management involved addressing fluid and electrolyte imbalances, careful monitoring of nutritional status, and correction of hyperglycemia with insulin. This case serves as a reminder to consider DKA in the differential diagnosis of patients with CF presenting with respiratory distress, even in the absence of typical symptoms such as polyuria and polydipsia.
囊性纤维化（CF）是一种由该基因的致病变异引起的遗传性疾病，与包括囊性纤维化相关糖尿病（CFRD）在内的各种并发症相关。CFRD是一种有别于1型或2型糖尿病的疾病。我们报告了一例患有CFRD的儿科患者发生糖尿病酮症酸中毒（DKA）的罕见病例，该病例发生在严重的肺部病情加重期间。对该患者的治疗包括纠正液体和电解质失衡、仔细监测营养状况以及用胰岛素纠正高血糖。该病例提醒我们，即使在没有多尿和多饮等典型症状的情况下，对于出现呼吸窘迫的CF患者进行鉴别诊断时也要考虑到DKA。
