Chiefs’ Inquiry Corner – 11/1/2021

 Necrotizing pancreatitis develops in approximately 20 to 30% of patients with acute pancreatitis. Infected necrotizing pancreatitis is a potentially fatal disease for which the current standard of care is treatment with antibiotics and postponement of catheter drainage of the necrotic area until the area of the infected pancreatic necrosis has become encapsulated, which takes about 4 weeks. These guidelines were largely established in a prior era of necrosectomy, when areas of infected pancreas were debrided with possible open surgery. As newer minimally invasive drainage techniques have emerged, it is unclear whether urgent drainage within 24 hours could improve patient outcomes. A recent multi-center, randomized superiority trial was conducted by the Dutch Pancreatitis Study group and looked at immediate drainage (within 24 hours of diagnosis) vs. later drainage and compared rates of complications within 6 months of the intervention. 104 patients were randomly assigned to immediate drainage (55 patients) or postponed drainage (49 patients) and followed up at 6 months for post-op complications via the Comprehensive Complication Index. There was no statistically significant difference in the early, immediate drainage group vs the postponed-drainage group with regards to complications and overall mortality. Of note, 28 patients (51%) in the immediate-drainage group required necrosectomy as compared with 11 patients (22%) in the postponed-drainage group. Ultimately, this trial does not show superiority of immediate drainage despite more recent advanced minimally invasive techniques. 

References: Immediate versus Postponed Intervention for Infected Necrotizing Pancreatitis

 Drug rash with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous eruption that typically presents within 2 to 6 weeks of drug initiation. Common clinical features include maculopapular rash, elevated liver enzyme levels, lymphadenopathy, facial edema, and eosinophilia. The aromatic anticonvulsants (phenytoin, phenobarbital, carbamazepine) are the most commonly implicated in DRESS syndrome, but several other drugs, including lamotrigine and allopurinol, have also been commonly associated. Cases of recurrence are most commonly attributed to the same generic drug or to compounds chemically closely related, and patients should be advised to avoid re-exposure to the offending drug and cross-reacting drugs.

References: Management of drug rash with eosinophilia and systemic symptoms (DRESS syndrome): an update.

 Maturity Onset Diabetes of the Young is a rare form of diabetes that occurs due to a single gene mutation. MODY accounts for 1-2% of all patients with diabetes. It’s estimated that >80% of patients with MODY are initially misdiagnosed with either Type I or Type II Diabetes with a delay of over 10 years to a molecular diagnosis. Patients typically present in their 20s-40s with a more subacute clinical picture. Initial assessment in patients presenting below the age of 35 should include a Hemoglobin A1c, C-peptide level, ketones, and GAD antibodies (the most common type of pancreatic beta-cell antibodies). In MODY patients, the c-peptide levels remain normal and beta-cell antibodies are negative. They often lack typical characteristics associated with insulin resistance and may have a normal BMI. One probability calculator has been used more widely in the UK as a means to estimate risk and justify genetic testing; this takes into account several of these factors such as age, sec, BMI, family history, and degree of insulin dependence. Importantly, making this diagnosis can be important because patient characteristics and response to treatment will vary based on the specific gene mutation. Some patients may respond well to diet and lifestyle changes alone, while others will require more aggressive therapy with oral agents and insulin. 

References: When to consider a diagnosis of MODY at the presentation of diabetes: aetiology matters for correct management