A Medley of Malnutrition and Myotonic Dystrophy: Twice Unlucky.

Micronutrient and vitamin deficiencies in young adults in the United States are relatively rare and often pose a diagnostic challenge. Here, we present the case of a young female who developed acute encephalopathy and muscular weakness two days following an endoscopic procedure performed to investigate the patient's four-month history of intractable nausea, vomiting, dysphagia, and weight loss. She was diagnosed with severe Wernicke encephalopathy due to thiamine deficiency as well as myotonic dystrophy type 1 (DM1). The patient's family history revealed an undiagnosed muscular disorder that required her father to ambulate with a wheelchair in the fourth decade of his life. DM1 with 11 trinucleotide repeats of cytosine-thymine-guanine in allele 1 and more than 150 in allele 2 of the myotonic dystrophy protein kinase (DMPK) gene were found on genetic testing. The patient's progressive dysphagia was likely a chronic manifestation of myotonic dystrophy that became more apparent following general anesthesia. DM1 is a multisystem genetic disorder of both skeletal and smooth muscles caused by deviation in the DMPK gene. Due to the involvement of esophageal smooth muscle, dysphagia may also be present. The long-standing dysphagia in our patient resulted in significant vitamin deficiency presenting as Wernicke encephalopathy. This case highlights the need for detailed, accurate family history and heightened suspicion for malnutrition in young adults who have eating disorders, dysphagia, and unexplained neurological changes.

Safety and Efficacy of Bariatric Surgery in Cirrhosis Patients With Extreme Obesity.

OBJECTIVE: To assess the safety and efficacy of bariatric surgery in patients with cirrhosis. SUMMARY BACKGROUND DATA: Bariatric surgery may be a viable option for patients with cirrhosis and extreme obesity. However, the risk of liver decompensation after surgery is not thoroughly investigated. METHODS: We conducted a case-controlled study with 106 obese patients with cirrhosis (cases) and 317 age, sex, body mass index-, and type of surgery-matched obese patients without cirrhosis (controls) who underwent bariatric surgery. RESULTS: Patients with cirrhosis were predominantly Child-Pugh class A (97%) with the diagnosis established prior to surgery in only 46%. In the cirrhosis group, there was no death in the first 30 days compared with 1 patient in the control group. At 90 days there was 1 death in the cirrhosis group but no additional deaths in the control group. In total, 12 months after the surgery, there were 3 deaths in the cirrhosis group and 1 in the control group (2.8% vs 0.6%, P = 0.056). The surgery-related length of stay was significantly longer in patients with cirrhosis (3.7 ±â€Š4.0 vs 2.6 ±â€Š2.4 d, P = 0.001), but the 30-day readmission rate was lower (7.5% vs 11.9%, P = 0.001). The percent of total weight loss at 30 and 90-days was not significantly different between the groups and remained that way even at 1 year (29.1 ±â€Š10.9 vs 31.2 ±â€Š9.4%, P = 0.096). CONCLUSIONS: Bariatric surgery in obese cirrhotic patients is not associated with excessive mortality compared with noncirrhotic obese patients.