A previously healthy 22-year-old female was diagnosed with Gestational Diabetes after failing her 1-hour glucose tolerance test at 28 weeks gestation with a blood glucose level of 241. Elevated glucose levels were noted on random basic metabolic panels done prior to pregnancy, but not meeting criteria to diagnose diabetes mellitus. Patient had no other symptoms at the time of the abnormal oral glucose tolerance test. Pertinent past medical history prior to pregnancy included ultrasounds showing a small multicystic dysplastic right kidney. Patient’s left kidney showed compensatory Hypertrophy and a small midpole cyst. Kidney function is followed by nephrology and has been unremarkable. Family history is positive for polycystic kidney disease in her mother and maternal grandmother. Due to her diagnosis of gestational diabetes, prior elevated glucose readings, and pertinent past medical history, a polycystic disease panel was performed to test for Mature Onset Diabetes of the Young (MODY). This panel came back positive for a pathologic mutation to HNF-1β with a deletion of exon 1-9 with genomic coordinates chr17:36047329_36105161 (GRCh37). The patient was subsequently diagnosed with MODY5. Patient was originally managing glycemic control with diet and exercise but eventually was placed on Basal Insulin and titrated to meet the glycemic targets during pregnancy. Patient has required no insulin since giving birth to her baby  Patient did experience other extra-pancreatic symptoms that included low magnesium (1.3mg/dl), high uric acid (11.1mg/dl), and pancreatic hypoplasia noted on previous CT done two years prior to her diagnosis. CT scan was done to rule out renal stones and did show pancreatic tail hypoplasia. Patient most recent blood work done 6 weeks following delivery. She delivered a healthy baby girl via spontaneous vaginal delivery at 39 weeks of gestation weighing 3170 g with APGAR score of 8 and 9 at 1 and 5 minutes respectively. Her daughter is born with normal kidney.

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With Regards
Angelina
Journal Coordinator
Medical Case Reports