A 22-year-old female with...

A 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 before pregnancy, but the patient did not meet the criteria to diagnose diabetes mellitus. The pertinent medical history before pregnancy included a small multicystic dysplastic right kidney depicted on ultrasound. The patient's left kidney showed compensatory hypertrophy and a small midpole cyst. Kidney function has been unremarkable. CT scan was done to rule out renal stones and did show pancreatic tail hypoplasia in the past. The patient also experienced other extrapancreatic symptoms, including low magnesium (1.3 mg/dl) and high uric acid (11.1 mg/dl). The 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 medical history, a polycystic disease panel was performed, which was positive for a pathologic mutation to HNF-1β with deletion of exons 1–9 with genomic coordinates chr17 : 36047329_36105161 (GRCh37).