Diabetes Mellitus Secondary to Acute Pancreatitis in a Child
Cooper and Hirschhorn first documented Wolf-Hirschhorn syndrome (WHS) in 1961. The syndrome is caused by a molecular deletion in the short arm of chromosome 4 (4p). It is characterized by the typical fascial features of the Greek warrior helmet appearance of the nose and forehead. Patients with WHS have a varying degree of intellectual disabilities and systemic involvement. The American Diabetes Association classified diabetes resulting from exocrine damage as type 3c. The exocrine damage can be related to pancreatitis, cystic fibrosis, hemochromatosis, pancreatic cancer, pancreatectomy, and pancreatic agenesis.

A meta-analysis highlighted the risk of diabetes following pancreatitis. 24 prospective studies of 1,102 patients with pancreatitis were studied. 37% developed prediabetes or diabetes. 16% who developed diabetes needed insulin. It was shown that a diagnosis of acute pancreatitis increases the risk of developing diabetes by over twofold over 5 years. Pooled prevalence of newly diagnosed diabetes within 1 year was 15% and increased to 40% after 5 years of the acute pancreatitis.

This is the case of an 18-year-old girl with WHS who presented acutely with nonketotic Hyperglycemic Hyperosmolar Status (HHS) in association with severe acute pancreatitis. Her presentation was preceded by febrile illness with preauricular abscess. She was treated with fluids and insulin infusion and remained on insulin 18 months after presentation. Her parents are cousins and the mother was diagnosed with type 2 diabetes.

She had negative autoantibodies and no signs of insulin resistance and her monogenic diabetes genetic testing was negative. Microarray study using WHS probe confirmed deletion of 4p chromosome. Acute pancreatitis is uncommon in children and development of diabetes following pancreatitis has not been reported in WHS. HHS is considerably less frequent than diabetes ketoacidosis in children. We highlight the complex presentation with HHS and acute pancreatitis leading to diabetes that required long term of insulin treatment.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5674493/
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