Impact of Cirrhosis Etiology on the Risk for Venous Thromboembolism.

BACKGROUND: Cirrhosis is associated with an increased risk for both bleeding and venous thromboembolic (VTE) complications. The data regarding the impact of etiology of cirrhosis on VTE risk is poorly understood. METHODS: In this retrospective observational analysis of the US Nationwide readmissions database 2019, we identified hospitalized patients who had cirrhosis from alcohol, viral, or nonalcoholic steatohepatitis (NASH) etiologies. We identified patients who had acute VTE, chronic/history of VTE, and portal venous thrombosis (PVT). Overall VTE risk was defined as the composite of acute and chronic VTE or PVT. The impact of etiology of cirrhosis on the crude and risk adjusted rates of VTE and PVT was studied. RESULTS: Of 432,383 patients with cirrhosis, 41.4% patients had NASH-cirrhosis, 39.7% had alcohol-related cirrhosis, and 18.9% had viral cirrhosis. The overall VTE rate was highest in patients with NASH cirrhosis (10.8%) followed by viral cirrhosis (9.7%) and alcohol-related cirrhosis (7.5%; P < 0.001). Similar results were observed for acute and chronic VTE. After risk adjustment, patients with NASH (OR 1.48 95% CI 1.42-1.54) and viral cirrhosis (OR 1.22 95% CI 1.17-1.29) had significantly higher overall VTE risk compared with alcohol-related cirrhosis. When separately evaluated, the adjusted risk for acute and chronic VTE was similar between patients with alcohol-related and viral cirrhosis but higher with NASH cirrhosis. PVT rate was highest with viral cirrhosis (4.3%) followed by NASH (2.8%) and alcohol-related cirrhosis (2.4%; P < 0.001). The adjusted risk of PVT was higher with viral (OR 1.61 95% CI 1.50-1.72) and NASH cirrhosis (OR 1.41 95% CI 1.31-1.52). CONCLUSION: NASH cirrhosis was associated with a higher VTE risk compared with alcohol-related and viral etiologies. As NASH cirrhosis increases in prevalence as the major etiology of cirrhosis, it is important to understand the increased VTE risk associated with this condition to improve management strategies and patient outcomes.

National Trends in the Incidence of Sporadic Malignant Colorectal Polyps in Young Patients (20-49 Years): An 18-Year SEER Database Analysis.

Background and Objectives: Conflicting guidelines exist for initiating average-risk colorectal cancer screening at the age of 45 years. The United States Preventive Services Task Force (USPSTF) changed its guidelines in 2021 to recommend initiating screening at 45 years due to an increasing incidence of young-onset colorectal cancer. However, the American College of Physicians (ACP) recently recommended not screening average-risk individuals between 45 and 49 years old. We aim to study the national trends in the incidence of sporadic malignant polyps (SMP) in patients from 20 to 49 years old. Materials and Methods: We analyzed the Surveillance, Epidemiology, and End Results database (2000-2017) on patients aged 20-49 years who underwent diagnostic colonoscopy with at least a single malignant sporadic colorectal polyp. Results: Of the 10,742 patients diagnosed with SMP, 42.9% were female. The mean age of incidence was 43.07 years (42.91-43.23, 95% CI). Approximately 50% of malignant polyps were diagnosed between 45 and 49 years of age, followed by 25-30% between 40 and 45. There was an upward trend in malignant polyps, with a decreased incidence of malignant villous adenomas and a rise in malignant adenomas and tubulovillous adenomas. Conclusions: Our findings suggest that almost half of the SMPs under 50 years occurred in individuals under age 45, younger than the current screening threshold recommended by the ACP. There has been an upward trend in malignant polyps in the last two decades. This reflects changes in tumor biology, and necessitates further research and support in the USPSTF guidelines to start screening at the age of 45 years.

Endoscopic retrograde cholangiopancreatography-related early perforations: A study of effects of procedure duration, complexity, and endoscopist experience.

BACKGROUND: Perforations (Perf) during endoscopic retrograde cholangiopancreatography (ERCP) are rare (< 1%) but potentially fatal events (up to 20% mortality). Given its rarity, most data is through case series studies from centers or analysis of large databases. Although a meta-analysis has shown fewer adverse events as a composite (bleeding, pancreatitis, Perf) during ERCP performed at high-volume centers, there is very little real-world data on endoscopist and center procedural volumes, ERCP duration and complexity on the occurrence of Perf. AIM: To study the profile of Perf related to ERCP by center and endoscopist procedure volume, ERCP time, and complexity from a national endoscopic repository. METHODS: Patients from clinical outcomes research initiative-national endoscopic database (2000-2012) who underwent ERCP were stratified based on the endoscopist and center volume (quartiles), and total procedure duration and complexity grade of the ERCP based on procedure details. The effects of these variables on the Perf that occurred were studied. Continuous variables were compared between Perf and no perforations (NoPerf) using the Mann-Whitney U test as the data demonstrated significant skewness and kurtosis. RESULTS: A total of 14153 ERCPs were performed by 258 endoscopists, with 20 reported Perf (0.14%) among 16 endoscopists. Mean patient age in years 61.6 ± 14.8 vs 58.1 ± 18.8 (Perf vs. NoPerf, P = NS). The cannulation rate was 100% and 91.5% for Perf and NoPerf groups, respectively. 13/20 (65%) of endoscopists were high-volume performers in the 4th quartile, and 11/20 (55%) of Perf occurred in centers with the highest volumes (4th quartile). Total procedure duration in minutes was 60.1 ± 29.9 vs 40.33 ± 23.5 (Perf vs NoPerf, P < 0.001). Fluoroscopy duration in minutes was 3.3 ± 2.3 vs 3.3 ± 2.6 (Perf vs NoPerf P = NS). 50% of the procedures were complex and greater than grade 1 difficulty. 3/20 (15%) patients had prior biliary surgery. 13/20 (65%) had sphincterotomies performed with stent insertion. Peritonitis occurred in only 1/20 (0.5%). CONCLUSION: Overall adverse events as a composite during ERCP are known to occur at a lower rate with higher volume endoscopists and centers. However, Perf studied from the national database show prolonged and more complex procedures performed by high-volume endoscopists at high-volume centers contribute to Perf.

Sex differences in clinical characteristics and outcomes associated with alcoholic hepatitis.

BACKGROUND: Alcohol-associated liver disease is increasing among females with an earlier onset and more severe disease at lower levels of exposure. However, there is paucity of literature regarding sex differences related to alcoholic hepatitis. METHODS: Hospitalized patients with alcoholic hepatitis were selected from the US Nationwide readmissions database 2019. In this cohort, we evaluated sex differences in baseline comorbidities, alcoholic hepatitis related complications and mortality. A subset of patients with alcoholic hepatitis who were hospitalized between January and June 2019 were identified to study sex differences in 6 month readmission rate, mortality during readmission, and composite of mortality during index hospitalization or readmission. RESULTS: Among 112 790 patients with alcoholic hepatitis, 33.3% were female. Female patients were younger [48 (38-57) vs. 49 (39-58) years; both P  < 0.001] but had higher rates of important medical and mental-health related comorbidities. Compared with males, females had higher rates of hepatic encephalopathy (11.5% vs. 10.1; P  < 0.001), ascites (27.9% vs. 22.5%; P  < 0.001), portal hypertension (18.5% vs. 16.4%; P  < 0.001), cirrhosis (37.3% vs. 31.9%; P  < 0.001), weight loss (19.0% vs. 14.5%; P  < 0.001), hepatorenal syndrome (4.4% vs. 3.8%; P  < 0.001), spontaneous bacterial peritonitis (1.9% vs. 1.7%; P  = 0.026), sepsis (11.1% vs. 9.5%; P  < 0.001), and blood transfusion (12.9% vs. 8.7%; P  < 0.001). Females had a similar in-hospital mortality rate (4.3%) compared to males (4.1%; P  = 0.202; adjusted odds ratio (OR) 1.02, 95% CI (cardiac index) 0.89-1.15; P  = 0.994). In the subset of patients ( N  = 58 688), females had a higher 6-month readmission rate (48.9% vs. 44.9%; adjusted OR 1.12 (1.06-1.18); P  < 0.001), mortality during readmission (4.4% vs. 3.2%; OR 1.23 (1.08-1.40); P  < 0.01), and composite of mortality during index hospitalization or readmission (8.7% vs. 7.2%; OR 1.15 (1.04-1.27); P  < 0.01). CONCLUSION: Compared to their male counterparts, females with alcoholic hepatitis were generally younger but had higher rates of comorbidities, alcoholic hepatitis related complications, rehospitalizations and associated mortality. The greater risks of alcohol-associated liver dysfunction in females indicate the need for more aggressive management.

Cancer-specific survival in non-mucinous appendiceal adenocarcinomas after local resection versus right hemicolectomy: A Surveillance, Epidemiology, and End Results database study.

BACKGROUND: Adenocarcinomas of the appendix are rare cancers for which no National Comprehensive Cancer Network guidelines exist, and for patients who undergo resection with curative intent, there is a paucity of data on prognostic factors affecting long-term cancer-specific survival. We aimed to compare the cancer-specific survival outcomes in adult patients with appendiceal non-mucinous adenocarcinoma undergoing either local resection versus right hemicolectomy. METHODS: This was a retrospective study from the National Cancer Institute Surveillance, Epidemiology, and End Results of patients who underwent curative resection over a 15-year period (2004-2019) for primary appendiceal adenocarcinoma. Out of 16,699 patients, 14,945 were excluded (exclusion criteria were non-adenocarcinoma histological types and patients with regional or distant metastasis as per National Cancer Institute Surveillance, Epidemiology, and End Results stage). Effects of factors (age, race, tumor biology [mucinous versus non-mucinous tumors], the extent of resection of the primary lesion, and lymph nodes) on cancer-specific long-term survival were studied. Survival analysis was performed using the Kaplan-Meier method. Survival outcomes were reported as mean survival (months). RESULTS: Of 1,754 patients, 827 (47.1%) were women, and 927 (52.1%) were men. The mean age in years (± standard deviation) was 62.43 ± 14.3. The racial distribution was as follows: Black 237 (13.5%), White 1,398 (79.7%), and Other 119 (6.8%). A total of 771 (44.6%) underwent local resection (appendectomy or segmental resection of colon without lymph node resection), and 983 (55.4%) underwent hemicolectomy with lymph node resection. Favorable survival prognosticators were age <50 years, White race, and well-differentiated histology. Patients with mucinous tumors experienced better survival. Patients who underwent right hemicolectomy with lymph node resection experienced better survival compared with those who had an appendectomy or segmental colonic resection for non-mucinous tumors rather than mucinous tumors. CONCLUSION: We report novel demographic, tumor-related, and operative prognostic factors impacting long-term cancer-specific survival in patients who undergo resection for appendiceal adenocarcinoma. The extent of resection of the primary lesion with draining lymph nodes determines long-term cancer-specific survival in non-mucinous appendiceal adenocarcinomas.

Six-month cardiovascular prognostic impact of type 1 And type 2 myocardial infarction in patients hospitalized for gastrointestinal bleeding.

BACKGROUND: Patients with gastrointestinal bleeding (GIB) are at an increased risk of cardiovascular events and myocardial infarction (MI). Myocardial supply-demand mismatch results in type 2 MI(T2MI) and atherosclerotic plaque rupture leads to type 1 MI(T1MI). Data comparing the prognostic impact of these MI types in GIB are sparse. METHODS: Patients hospitalized for GIB were identified in the 2019 US Nationwide Readmissions Sample. In this population, we studied the differences in management of T1MI and T2MI, and the association of these MI types with in-hospital mortality and risk for 6-month MI and MI-related mortality. RESULTS: Of 444,475 patients admitted for a GIB, 12,860 (2.9%) had an MI (1.7% T2MI, 1.2% T1MI). Patients with T1MI were more likely to receive coronary angiography and revascularization than patients with T2MI. In-hospital mortality occurred in 2.0% patients, at a significantly higher rate in patients with an MI (7.9% vs 1.8%; P < 0.001), and higher with T1MI (11.9%) than T2MI (5.3%; P < 0.001). Among the survivors, 2.2% patient had an MI within 6 months, at a significantly higher rate in patients with index MI (13.1% vs 2.0%, adjusted OR 4.3 95% CI 3.83-4.90; P < 0.001). Mortality during the subsequent MI occurred in 0.3% of all patients (12% with an MI), at a 6-fold higher rate in patients with index MI (1.7% vs 0.3%; adjusted OR 3.69 95% CI 2.75-4.95; P < 0.001). The elevated risks were associated with both MI types. The risks for 6-month MI and related mortality were similar between T1MI and T2MI (6-month AMI: adjusted OR for T2MI = 1.03, 95% 0.83-1.29; fatal MI: adjusted OR for T2MI = 1.5, 95% CI 0.85-2.7). CONCLUSION: The occurrence of an MI is associated with a substantially elevated risk for subsequent AMI and related mortality in patients hospitalized for a GIB. This future prognostic impact was similar between T1MI and T2MI.

Fibrolamellar hepatocellular carcinoma: An epidemiologic and 5-year cancer survival assessment based off SEER data.

The fibrolamellar variant of hepatocellular carcinoma makes up a small percentage of liver tumors. Despite being a subset, it has been noted in the literature to have variations in terms of its epidemiology and intervention recommendations. Using the Surveillance, Epidemiology, and End Results database, 339 cases from 1988 to 2016 were studied. Favorable prognostic epidemiological factors included male sex, younger ages, and white race. Those who underwent any lymph node resection (combined with liver resection) did better than those without lymph node resection; chemotherapy proved beneficial for those where surgery was contraindicated. To our knowledge, this report is the largest conglomerate dataset analyzing prognostic profiles and treatment strategies for fibrolamellar hepatocellular carcinoma.

Evidence-based approach to management of hepatic encephalopathy in adults.

Hepatic encephalopathy (HE) is a reversible syndrome of impaired brain function and represents one of the many complications of portal hypertension and decompensated liver disease. Although ammonia is clearly implicated in the pathogenesis of HE, the pathogenesis of HE is multifactorial with numerous mechanisms that results in functional impairment of neuronal cells. The initial management of HE focuses on supportive care and stabilization which includes providing appropriate nutritional support. Thereafter, focus should be on identifying and treating the precipitating factors. There are many therapeutic agents available for the management of HE, most of which are directed towards lowering the gut nitrogen load and thus the serum ammonia level. This review aims to provide an update on the conventional and emerging treatment options for HE.

Crosstalk of hepatocyte nuclear factor 4a and glucocorticoid receptor in the regulation of lipid metabolism in mice fed a high-fat-high-sugar diet.

BACKGROUND: Hepatocyte nuclear factor 4α (HNF4α) and glucocorticoid receptor (GR), master regulators of liver metabolism, are down-regulated in fatty liver diseases. The present study aimed to elucidate the role of down-regulation of HNF4α and GR in fatty liver and hyperlipidemia. METHODS: Adult mice with liver-specific heterozygote (HET) and knockout (KO) of HNF4α or GR were fed a high-fat-high-sugar diet (HFHS) for 15 days. Alterations in hepatic and circulating lipids were determined with analytical kits, and changes in hepatic mRNA and protein expression in these mice were quantified by real-time PCR and Western blotting. Serum and hepatic levels of bile acids were quantified by LC-MS/MS. The roles of HNF4α and GR in regulating hepatic gene expression were determined using luciferase reporter assays. RESULTS: Compared to HFHS-fed wildtype mice, HNF4α HET mice had down-regulation of lipid catabolic genes, induction of lipogenic genes, and increased hepatic and blood levels of lipids, whereas HNF4α KO mice had fatty liver but mild hypolipidemia, down-regulation of lipid-efflux genes, and induction of genes for uptake, synthesis, and storage of lipids. Serum levels of chenodeoxycholic acid and deoxycholic acid tended to be decreased in the HNF4α HET mice but dramatically increased in the HNF4α KO mice, which was associated with marked down-regulation of cytochrome P450 7a1, the rate-limiting enzyme for bile acid synthesis. Hepatic mRNA and protein expression of sterol-regulatory-element-binding protein-1 (SREBP-1), a master lipogenic regulator, was induced in HFHS-fed HNF4α HET mice. In reporter assays, HNF4α cooperated with the corepressor small heterodimer partner to potently inhibit the transactivation of mouse and human SREBP-1C promoter by liver X receptor. Hepatic nuclear GR proteins tended to be decreased in the HNF4α KO mice. HFHS-fed mice with liver-specific KO of GR had increased hepatic lipids and induction of SREBP-1C and PPARγ, which was associated with a marked decrease in hepatic levels of HNF4α proteins in these mice. In reporter assays, GR and HNF4α synergistically/additively induced lipid catabolic genes. CONCLUSIONS: induction of lipid catabolic genes and suppression of lipogenic genes by HNF4α and GR may mediate the early resistance to HFHS-induced fatty liver and hyperlipidemia.

Epidemiology and outcomes of gastrointestinal mucosal melanomas: a national database analysis.

AIM: Gastrointestinal malignant melanoma is a rare mucosal melanoma (MM). Other MM include the respiratory and the genitourinary tract. All mucosal melanomas have a poor prognosis when compared to cutaneous melanomas. Ano-rectal melanomas are by far the most common and most studied gastrointestinal MM. Large-scale clinical data is lacking due to the rarity of the disease. We aim to analyze epidemiology and survival of the Gastrointestinal (G.I.) MM over 45 years using a national database. METHODS: The Surveillance, Epidemiology and End Results (SEER) database was queried to identify patients with biopsy-proven G.I. Melanomas. We selected tumor site, intervention, and survival information for oncology codes as per the international classification of diseases. Survival analysis was performed using the SPSS v 27 ® IBM software. RESULTS: Of the 1105 biopsy-proven confirmed cases of primary G.I. melanoma's, 191 (17.3%) received chemotherapy (C.T.), 202 (18.3%) received radiotherapy (R.T.), 63 (5.7%) received both C.T and R.T., while 684 (61.9%) of the population received surgery alone or combined with C.T. and/or R.T. Statistically significant improvement in survival was noted in all treatment strategies that utilized surgery and also when site-specific MM cohorts underwent a surgical approach with or without C.T and/or R.T. CONCLUSION: This is the most extensive study reporting epidemiological and survival data of treatment strategy outcomes of primary G.I. mucosal melanoma elucidating best overall survival with a management strategy involving surgical intervention.

Fecal Microbiota Transplant for Hematologic and Oncologic Diseases: Principle and Practice.

Understanding of the importance of the normal intestinal microbial community in regulating microbial homeostasis, host metabolism, adaptive immune responses, and gut barrier functions has opened up the possibility of manipulating the microbial composition to modulate the activity of various intestinal and systemic diseases using fecal microbiota transplant (FMT). It is therefore not surprising that use of FMT, especially for treating relapsed/refractory Clostridioides difficile infections (CDI), has increased over the last decade. Due to the complexity associated with and treatment for these diseases, patients with hematologic and oncologic diseases are particularly susceptible to complications related to altered intestinal microbial composition. Therefore, they are an ideal population for exploring FMT as a therapeutic approach. However, there are inherent factors presenting as obstacles for the use of FMT in these patients. In this review paper, we discussed the principles and biologic effects of FMT, examined the factors rendering patients with hematologic and oncologic conditions to increased risks for relapsed/refractory CDI, explored ongoing FMT studies, and proposed novel uses for FMT in these groups of patients. Finally, we also addressed the challenges of applying FMT to these groups of patients and proposed ways to overcome these challenges.

Enlarging Liver Mass: Inflammatory Pseudotumor in a Patient With Polymyalgia Rheumatica.

Inflammatory pseudotumors of the liver are rare, non-neoplastic liver tumors. Due to the nonspecific clinical presentation, imaging features, and histopathological findings, they can mimic malignant tumors requiring invasive diagnostics. We present a case of a 61-year-old female patient with a history of type 2 diabetes mellitus, hypothyroidism, hyperlipidemia, and polymyalgia rheumatica who had initially presented with abdominal pain for 3 weeks. Further workup showed normal liver chemistries and tumor markers: AFP and CA 19-9. Magnetic resonance imaging (MRI) of the abdomen showed a segment 6 lesion measuring 4.1 × 4.0 × 3.7 cm. A liver biopsy then confirmed the diagnosis of an inflammatory pseudotumor of the liver with negative IgG4. On follow-up imaging, a rapid growth of this liver lesion was noted. Laparoscopy was done but did not show any distinct liver lesion. Follow-up imaging confirmed a decrease in the size of the mass. Interestingly, the patient had been on a higher dose of steroids for her polymyalgia rheumatic leading up to the follow-up imaging. This is the first case of an inflammatory pseudotumor of the liver in a patient with polymyalgia rheumatica. With this case, we would like to increase the awareness for inflammatory pseudotumors of the liver as a differential diagnosis of liver lesions in patients with underlying autoimmune disorders.

Persistent lactic acidosis in the Mauriac syndrome in type 1 diabetes mellitus.

Mauriac syndrome is an uncommon clinical condition arising in the setting of poorly controlled type 1 diabetes mellitus. Clinical features include hepatomegaly and liver abnormalities indistinguishable from nonalcoholic fatty liver disease. Early diagnosis and management are essential, as Mauriac syndrome is reversible.

Polyethylene glycol versus lactulose in the treatment of hepatic encephalopathy: a systematic review and meta-analysis.

BACKGROUND: Hepatic encephalopathy (HE) is defined as brain dysfunction that occurs because of acute liver failure or liver cirrhosis and is associated with significant morbidity and mortality. Lactulose is the standard of care till this date; however, polyethylene glycol (PEG) has gained the attention of multiple investigators. METHODS: We screened five databases namely PubMed, Scopus, Web of Science, Cochrane Library and Embase from inception to 10 February 2021. Dichotomous and continuous data were analysed using the Mantel-Haenszel and inverse variance methods, respectively, which yielded a meta-analysis comparing PEG versus lactulose in the treatment of HE. RESULTS: Four trials with 229 patients were included. Compared with lactulose, the pooled effect size demonstrated a significantly lower average HE Scoring Algorithm (HESA) Score at 24 hours (Mean difference (MD)=-0.68, 95% CI (-1.05 to -0.31), p<0.001), a higher proportion of patients with reduction of HESA Score by ≥1 grade at 24 hours (risk ratio (RR)=1.40, 95% CI (1.17 to 1.67), p<0.001), a higher proportion of patients with a HESA Score of grade 0 at 24 hours (RR=4.33, 95% CI (2.27 to 8.28), p<0.0010) and a shorter time to resolution of HE group (MD=-1.45, 95% CI (-1.72 to -1.18), p<0.001) in favour of patients treated with PEG. CONCLUSION: PEG leads to a higher drop in the HESA Score and thus leads to a faster resolution of HE compared with lactulose.

Hyponatremia in Cirrhosis: An Update.

Hyponatremia is frequently seen in patients with ascites secondary to advanced cirrhosis and portal hypertension. Although not apparent in the early stages of cirrhosis, the progression of cirrhosis and portal hypertension leads to splanchnic vasodilation, and this leads to the activation of compensatory mechanisms such as renin-angiotensin-aldosterone system (RAAS), sympathetic nervous system, and antidiuretic hormone (ADH) to ameliorate low circulatory volume. The net effect is the avid retention of sodium and water to compensate for the low effective circulatory volume, resulting in the development of ascites. These compensatory mechanisms lead to impairment of the kidneys to eliminate solute-free water in decompensated cirrhosis. Nonosmotic secretion of antidiuretic hormone (ADH), also known as arginine vasopressin, further worsens excess water retention and thereby hyponatremia. The management of hyponatremia in this setting is a challenge as conventional therapies for hyponatremia including fluid restriction and correction of hypokalemia are frequently inefficacious. In this review, we discuss the pathophysiology, complications, and various treatment modalities, including albumin infusion, selective vasopressin receptor antagonists, or hypertonic saline for patients with severe hyponatremia and those awaiting liver transplantation.

Case series and review of liver dysfunction in COVID-19 patients.

Coronoviraus disease 2019 (COVID-19) has infected over two million people worldwide and the number keeps growing every day. While the pulmonary complications of COVID-19 are obvious, the effect of the virus on the other organs and the chronicity of the organ dysfunction remain unknown. The virus causes a debilitating infection with multiorgan injury and has a high mortality rate estimated to be around 3.70%. Several hypotheses are formulated to explain the liver dysfunction in COVID-19 patients which include collateral damage from cytokine storm, drug-induced liver injury, viral-induced hepatitis and hypoxia-induced damage. Through this case series, we would like to highlight that liver enzyme abnormalities are often seen in COVID-19 patients and would like to highlight that physicians need to serially monitor biochemical testing until the liver enzymes return to baseline. Physicians also need to be vigilant of liver enzyme abnormalities in these patients, especially before starting new medications.

Glycogenic Hepatopathy.

Glycogenic hepatopathy is excessive intrahepatic glycogen accumulation. It is a rare complication of long-standing, poorly controlled type 1 diabetes mellitus. We report a case of a 19-year-old woman with a history of poorly controlled diabetes mellitus and frequent admissions for diabetic ketoacidosis, who presented with abdominal pain, nausea, vomiting, and hepatomegaly. She was found to have diabetic ketoacidosis with persistently elevated serum lactate that did not improve with insulin infusions. She eventually underwent a liver biopsy, which showed excessive intracytoplasmic glycogen accumulation consistent with glycogenic hepatopathy.