bluuebunny/crossref_metadata_2025_split huggingface dataset:
2279 DRESS Causing a Subacute Fulminant Hepatitis Secondary to Sulfasalazine
Authors: Zaibaq-Krill Jenine , Sydor Michael , Merwat Sheharyar , Merwat Shehzad N. , Luthra Gurinder 
Published: October 2019

INTRODUCTION:
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and life-threatening hypersensitivity reaction most often due to anti-epileptics. It is characterized by a maculopapular rash, eosinophilia, lymphadenopathy, and visceral organ involvement. Here we present a case of DRESS caused by sulfasalazine which resulted in subacute fulminant hepatitis.

CASE DESCRIPTION/METHODS:
A 24-year-old male with ulcerative colitis was started on sulfasalazine. After being on sulfasalazine for one month, he ran out of the medication. Ten days later, he developed a diffuse pruritic morbilliform rash. He initially presented with the following lab values: total bilirubin 0.7 mg/dL, AST 175 U/L, ALT 354 U/L, alkaline phosphatase 208 U/L, and INR of 1.1. Skin biopsy revealed focal vacuolar interface dermatitis and superficial perivascular lymphocytic infiltrate with neutrophils and eosinophils, consistent with drug eruption. He was started on prednisone 50mg daily and was discharged home. Ten days later, he returned to the hospital with jaundice and the following lab values suggestive of subacute fulminant hepatitis: total bilirubin 6.5 mg/dL (conjugated bilirubin 3.4 mg/dL), AST 3475 U/L, ALT 4171 U/L, alkaline phosphatase 308 U/L, and INR 2.6. Due to the degree of his hepatocellular injury, he was started on prednisone 2mg/kg. The rash, liver chemistries and INR improved over the next week.

DISCUSSION:
Sulfasalazine is a rare, but known cause of DRESS and idiosyncratic liver injury. This case is notable because he developed a subacute fulminant hepatitis 10 days after initial presentation and 20 days after discontinuation of sulfasalazine. This highlights the fact that liver injury can evolve into acute liver failure despite withdrawal of the culprit drug several days prior; this latency period is characteristic of sulfasalazine induced acute liver injury and thus supports our diagnosis. AST and bilirubin levels are the most important prognostic indicators for liver transplantation and/or death. DRESS due to sulfasalazine with significantly elevated transaminase levels ( >2000 U/L) has been reported, however, to our knowledge, this is the third reported case with this degree of hepatocellular injury that did not result in liver transplantation. Given the fact that acute liver failure is often the cause of mortality in patients with DRESS, this case illustrates that serial lab monitoring is essential in such patients as liver injury can persist long after the offending agent has been discontinued.
2281 Symptomatic Isolated Polycystic Liver Disease Causing Respiratory Compromise
Authors: Rivas Cynthia , Castano Juan J. , Pozo Leonardo , GrigoriyRapoport MD , Zamir Asif 
Published: October 2019

INTRODUCTION:
Isolated Polycystic Liver Disease (PCLD) is defined by the presence of more than 20 hepatic cysts. It has an autosomal dominant inheritance and is largely asymptomatic with only 5% of patients who report pain or other complaints. The disorder is considered rare with an estimated incidence of < 0.01%. We present a case of isolated PCLD causing respiratory failure.

CASE DESCRIPTION/METHODS:
A 45-year-old woman with history of an isolated liver cyst, was admitted for acute respiratory failure. No significant family history. On physical exam, oxygen saturation was 98% on 2 liters nasal cannula. She had prolonged expiratory phase, decreased breath sounds and marked hepatomegaly. FEV1 = 23%, FEV1/FVC = 117%. Liver studies were significant for alkaline phosphatase 131 IU/L and albumin 3.3 gm/dl. Abdominal CT showed a markedly enlarged liver measuring 21 cm, liver volume of 3588 ml and the presence of numerous thin-walled-fluid-filled cysts ranging from 2 mm to 4 cm, consistent with PCLD. It was determined that the mass effect from the liver pressing against the diaphragm was the cause of her respiratory failure. She was started on BiPAP with improvement of respiratory function. She underwent evaluation for orthotopic liver transplantation(OLT) but was not a candidate due to high risk for hypercarbic respiratory failure, and prolonged mechanical ventilation. She was discharged on non-invasive ventilation with oxygen supplementation and was scheduled for re-evaluation for OLT.

DISCUSSION:
Isolated PCLD is caused by germline mutations of the PRKCSH and SEC63 genes. Genetic testing is rarely necessary and does not affect management. Liver function is often altered once symptoms develop. The number and size of liver cysts often increase with age. Women often present with a massive and symptomatic cystic liver more frequently than men and symptoms are likely attributed to the compression of adjacent organs due to mass effect, which often leads to poor body image and depression. When symptoms develop they include pain, nausea, early satiety, vomiting, or dyspnea. Complications are rare and can include infection, portal hypertension, and Budd-Chiari Syndrome. Treatment includes aspiration-sclerotherapy, laparoscopic or open surgical fenestration techniques, and hepatectomy. Somatostatin analogues show promise. Liver transplantation may be required in extensive disease. One should be cognizant that symptomatic isolated PCLD often leads to greatly diminished quality of life.
2292 A Case Report of Glycogen Hepatopathy: A Reversible, yet Relapsing Cause of Hepatitis in Type 1 Diabetics
Authors: Azhar Mishah , Hammami Muhammad B. , Musmar Ahmad , Bromer Matthew 
Published: October 2019

INTRODUCTION:
Glycogen hepatopathy (GH), a rare glycogen storage disease caused by genetic or acquired overactivation of hepatic glycogen synthesis enzymes, can mimic non-alcoholic fatty liver disease (NAFLD). We describe a case of biopsy proven GH in an adult with Type 1 DM (T1DM). Similar to cases described in the current literature, our case not only portrays the challenges faced by physicians when diagnosing this condition, but also the importance of distinguishing this from NAFLD.

CASE DESCRIPTION/METHODS:
A 33-year-old Honduran woman with a 25-year history of T1DM complicated by gastroparesis, multiple episodes of DKA and hypoglycemia, and recurrent pancreatitis was referred for abnormal liver enzymes. Family history was negative for liver disease. There was no history of alcohol or recreational drug use. Patients medications included insulin and thyroxine. Physical exam showed hepatomegaly but no stigmata of chronic liver disease. AST and ALT had ranged from 100’s to over 7000 while ALP was elevated to over 400. Albumin, Total bilirubin, platelets, INR, eosinophils, viral hepatitis panel, ANA, smooth muscle AB, LKM Ab, celiac serologies, ceruloplasmin, alpha 1 antitrypsin, iron studies, and acetaminophen levels were all normal. An abdominal ultrasound showed “fatty liver” and an atrophic pancreas. CT abdomen showed hepatomegaly. CBD was found to be normal on EUS and MRCP. A liver biopsy demonstrated glycogenotic hepatocytes.

DISCUSSION:
GH is frequently misdiagnosed as NAFLD, a more common liver disease that is also associated with diabetes. While GH is known to be reversible with otherwise no known long-term complications, NAFLD has been shown to progress to cirrhosis and cause hepatocellular carcinoma. Definite diagnosis often requires liver biopsy because of overlapping clinical and radiographical pictures. Elevation of both glucose and insulin levels in the setting of fragile DM control is thought to play a role via overstimulation of glycogen synthesis. Recommended treatment is stable “tight” glycemic control; pancreatic transplantation has resulted in sustained GH remission in two case reports. The required degree of stability and tightness of glucose control is not yet known. An increased awareness of GH is needed in an attempt to prevent delay in diagnosis, in a condition with an otherwise unknown incidence.
2293 An Unusual Complication of Acute Viral Hepatitis A: Hemolytic Anemia
Authors: Korada Sai Krishna , Sobotka Lindsay , Wellner Michael , Michaels Anthony 
Published: October 2019

INTRODUCTION:
Hepatitis A is one of the most prevalent causes of acute hepatitis worldwide. Given the availability and effectiveness of vaccination, rates of viral hepatitis A (HAV) decreased in the United States until recently with multiple areas across the United States experiencing outbreaks. Disease presentation can be variable with the majority of patients presenting with abdominal pain, nausea, vomiting, and jaundice. Few patients present with fulminate liver failure and have required liver transplantation. Other rare extrahepatic presentations of hepatitis A include acute kidney injury, neurologic complications, and anemia.

CASE DESCRIPTION/METHODS:
A 38-year-old female with a medical history of type 2 diabetes and hypertension presented with worsening anemia and fatigue. The patient was admitted to an outside hospital two months prior with abnormal liver function tests and was ultimately diagnosed with acute HAV. Physical exam revealed diffuse jaundice, scleral icterus, and abdominal tenderness. Lab results on admission are demonstrated in Table 1. She had no active signs of GI bleeding. Prednisolone 40mg daily was initiated given concern for hemolytic anemia. Analysis to evaluate for hemolysis is demonstrated in Table 2. Steroids were stopped due to side effects. The patient was treated supportively and hemoglobin stabilized.

DISCUSSION:
Rare extrahepatic presentations of acute hepatitis A occur in less than 10% of affected patients. Prompt recognition of hemolytic anemia is crucial. Patients recently diagnosed with HAV that develop acute anemia without overt signs of bleeding should be evaluated for hemolysis. Analysis with haptoglobin, lactate dehydrogenase, direct Coombs test, reticulocyte count and peripheral blood smear should be performed to confirm the diagnosis. No studies have been completed to determine factors that could predispose specific patients to develop hemolytic anemia but multiple studies have suggested increased frequency in patients with underlying glucose-6-phosphate dehydrogenase deficiency. Multiple treatment options have been proposed for patients that have developed hemolytic anemia as a complication of HAV, with most patients receiving steroids. Other studies highlight the use of plasmapheresis though the effectiveness of this intervention remains uncertain. Prior literature suggests that supportive care or a shorter course of steroids may be sufficient. However, no studies prove which intervention is the most effective. Anemia typically recovers as acute HAV resolves.
2300 Chronic Lymphocytic Leukemia of the Liver: A Rare Cause of Elevated Liver Function Tests
Authors: Buckshaw Ricky , Malik Anam , Kloska Michal , Vaze Reema , Wong She-Yan 
Published: October 2019

INTRODUCTION:
Chronic lymphocytic leukemia is a lymphocytic proliferative malignancy that can infiltrate multiple different organs. It is one of the most common forms of leukemia in the adult population. CLL affecting the liver is historically rare, however since recent it has been becoming more prevalent due to certain advancements in chemotherapy. With metastatic CLL becoming more prevalent, we should focus expansion of research to this subset of patients.

CASE DESCRIPTION/METHODS:
A 76-year-old male with a past medical history of CLL 13q14 mutation+ and a recent diagnosis of renal insufficiency was evaluated by his Gastroenterologist for elevated liver enzymes. He was found to have an AST of 112 and an ALT of 348. His ferritin was also elevated to 953 with a transferrin saturation of 27%. He was referred to a Hepatologist for a more in-depth workup, which revealed worsening lymphadenopathy on CT scan along with worsening hepatomegaly and splenomegaly on abdominal ultrasound. Blood work was ordered for evaluation of infectious, inflammatory and autoimmune causes of his elevated liver enzymes. The patient’s blood work showed a negative ANA/SMA/AMA, a negative HAV/HBV/HCV. The patient underwent percutaneous liver biopsy for further evaluation. The biopsy showed involvement of his liver with dense lymphoid infiltrate of the portal tracts, consistent with chronic lymphocytic leukemia. The pathology also resulted showing the liver parenchyma showed the same biomarkers as the patients serum studies. The positivity of CD5, CD23 and PAX5 was consistent with the previous serum studies. The patient was initiated on Imbruvica, which provided improvement in his liver function tests. He continues to follow with Oncology for continued treatment on Imbruvica.

DISCUSSION:
The increased prevalence of liver dysfunction brings up new conversations in CLL treatment. Liver infiltration has not been studied as much as other organ systems and this limits our understanding of this chronic, debilitating disease process. There has been a documented shorter survival for patients diagnosed with liver infiltration of CLL, which limits our patient’s quality of life if we do not better grasp this potential complication of CLL.
2303 A Rare Case of Atraumatic Intercostal and Intrathoracic Liver Herniation Related to Influenza A
Authors: Harne Prateek S. , Mukherjee Samiran , Shepherd Zachary 
Published: October 2019

INTRODUCTION:
A non-traumatic intercostal hernia is sparsely reported and is defined as a protrusion of intra-abdominal contents through acquired or congenital defect of the abdominal and thoracic walls. There have been individual cases reporting such herniation of lungs, small and large bowel, omentum, and liver. We describe a case in which a patient had both intercostal and diaphragmatic liver herniation secondary to increased cough. The literature relies on CT for diagnosis and for assessing damage to liver parenchyma which guides management. While intercostal liver herniations can be surgical emergencies, our case was managed conservatively given minimal liver injury and rapidly improving symptoms.

CASE DESCRIPTION/METHODS:
A 58-year-old female with a past medical history of hypothyroidism and essential hypertension presented with severe dry cough and nasal congestion for two weeks. Three days before presentation, she noticed a bruise on the right lateral chest wall which gradually increased and became painful with radiation to her back. The patient denied any recent history of trauma to the area. Physical exam revealed dry cough, large palpable tender bulging ecchymosis over right 9th and 10th ribs, and clear lungs. Vitals were unremarkable. Labs were significant for ALT 41, AST 62, CK 493, white count 17.8, platelets 567, INR 0.9 and positive nasal swab for influenza A. CT abdomen showed herniation of uninjured liver between 9th and 10th ribs with abdominal wall soft tissue stranding (or edema) with part of liver herniating through a diaphragmatic defect into the thoracic cavity (“hump sign”). Surgical services were consulted, and the patient was managed conservatively with opiate analgesia, IV hydration with normal saline, cough suppression, Tamiflu, and discharged with medical and surgical follow up.

DISCUSSION:
In patients with poor abdominal muscle strength having sudden worsening of pain after coughing and physical exam findings consistent with herniation, there should be high suspicion for abdominal and transdiaphragmatic herniation of liver. Given the rarity of such events, these patients can present as a diagnostic challenge and delay in care can result in poor outcomes especially in patients with organ damage. A consensus for management can be difficult due to sparsely reported cases, however, while injured or strangulated liver herniation is a surgical emergency, a conservative approach can be considered for uninjured liver herniation with close medical and surgical follow up.
2304 Glycogen Hepatopathy: An Unusual Cause of Liver Enzymes Elevation in Diabetic Patients
Authors: Sourabh Fnu , Jain Anubhav , Aggarwal Ankita , Kumar Sarwan , Ralh Neeharika 
Published: October 2019

INTRODUCTION:
Liver enzyme elevation is a common condition among diabetic patients and often Nonalcoholic Fatty Liver Disease is responsible for this,which can lead to fibrosis and cirrhosis. GH should be one of the differential diagnoses upon exam of a patient with uncontrolled DM with elevated transaminases and hepatomegaly. Compared to other liver diseases associated with DM, GH is a favorable diagnosis due to its benign nature and good prognosis.

CASE DESCRIPTION/METHODS:
An 18 years old female with type I diabetes had been non compliant with her basal insulin frequently. This had led to 7-8 hospitalizations within last year. This time, she was in the hospital for a ketoacidosis episode with HbA1C of 17%. During P/E, her liver was enlarged. Liver enzymes were, ALP-201 U/L, AST-120 U/L, ALT-125 U/L, GGT 98 U/L).Lipid profile- Cholesterol 315 mg/dl, HDL 50.4 mg/dl, LDL 205 mg/dl, Triglyceride 298 mg/dl. Her USG showed 19 cm liver with normal parenchyma. CT scan reiterated the findings with hyperdense liver. It was noted that the liver enzymes had great fluctuations within the last year, when she was hospitalized due to hyperglycemia ,and her liver enzymes declined when blood sugar was regulated. The patient was examined with regard to chronic hepatitis differential diagnosis since the aminotransferase levels were found to be elevated , intermittently from last year. Serology for viral hepatitis and autoimmune hepatitis had normal results. So, a liver biopsy was performed. Significant Glycogen rich hepatocytes were observed in the tissue, without fibrosis or inflammation. The case was diagnosed with GH due to the present clinical, laboratory, and pathological findings. Patient was advised diet and intensive insulin regimen. Though she remained non compliant. 6 years follow up revealed persistent elevated enzymes with CT scan showing liver size of 21 cm with liver enzymes elevation.

DISCUSSION:
Glycogenic hepatopathy is a rare complication of the poorly controlled diabetes mellitus characterized by the liver dysfunction with elevated liver enzymes and hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. Future progress is required in understanding the biochemical defects underlying GH and an ideal diagnostic test instead of biopsy to avoid the extensive workup in evaluating suspected cases of GH.
2306 Hydroxychloroquine-Induced Acute Liver Injury
Authors: Cheema Bahar , Triplett Drew , Krishnamurthy Padmini 
Published: October 2019

INTRODUCTION:
Hydroxychloroquine is a commonly prescribed medication with antimalarial and anti-inflammatory properties. A very rarely reported side effect of this medication is drug induced acute liver injury.

CASE DESCRIPTION/METHODS:
A 62-year old female with a history of rheumatoid arthritis, on long term prednisone, presented to the emergency department with two day history of arthralgias, generalized weakness, and fever of 104 F. She had no history of alcohol use or any risk factors for viral hepatitis. Clinical exam was unremarkable. Laboratory data showed Aspartate aminotransferase (AST) or 798 IU/L and Alanine aminotransferase (ALT) of 1254 IU/L, with normal serum bilirubin, alkaline phosphatase and coagulation profile. Serologic tests for viral etiology including Hepatitis A, Hepatitis B, Hepatitis C, Cytomegalovirus, Epstein-Barr virus, and Human immunodeficiency virus were non-reactive. Anti-nuclear antibody, smooth muscle antibody, and anti-double-stranded DNA antibody were unremarkable. Her urine and blood cultures showed no growth. Abdominal ultrasound showed an echogenic liver consistent with hepatic steatosis and no evidence of biliary obstruction. Chest X-ray was normal. Upon review of her medications, patient had started hydroxychloroquine two weeks prior to admission. The medication was discontinued upon admission, after which she continued to improve. At follow up ten days later, she remained asymptomatic and her liver function tests continued to improve with AST of 46 and ALT of 161.

DISCUSSION:
Acute transaminitis due to hydroxychloroquine is rare. Only four reported cases have been noted per literature review. Mechanism of injury to hepatic cells is unknown however the drug is metabolized in the liver and may alter the metabolism of other drugs. Porphyria cutanea tarda is a disease that may be treated with low doses of hydroxychloroquine. It is known that higher doses of hydroxychloroquine usually greater than 100 mg twice a week can trigger acute liver injury in patients with history of Porphyria cutana tarda. These patients present with sudden onset of fever and marked serum enzyme elevations with increased excretion of porphyrins and photosensitivity. The presented patient in the case report did not have underlying Porphyria cutanea tarda.
2310 A Mysterious Case of Non-Cirrhotic Recurrent Severe Hyperammonemia: What Urea-lly Need to Know
Authors: Pendela Venkata Satish , Munoz Anisleidys , Razzouk Gaby 
Published: October 2019

INTRODUCTION:
Ammonia is a well-known toxin, mostly studied in Hepatic encephalopathy. While, it is related to severe liver disease in 90% cases, the remaining 10% is constituted by disturbances in its excretion or related to over production from other etiologies. [1] Awareness of hyperammonemia in absence of severe hepatic disease may lead to lifesaving prompt diagnosis and treatment. We present a mysterious case of recurrent severe hyperammonemia without known liver disease.

CASE DESCRIPTION/METHODS:
A 53 year old male with prior history of well controlled hypertension presented to the emergency department with unresponsiveness. On exam, he was unconscious with occasional spontaneous eye opening and a left gaze preference. CT head and CT angiogram of neck did not reveal any acute abnormalities. Liver enzymes and Bilirubin were normal. Blood alcohol level was 0.089gm/dl%. EEG showed diffuse encephalopathy. Serum ammonia was 705 umol/L. He was started on lactulose. His mentation improved in a day and repeat serum ammonia was 25umol/L. Ultrasound liver and CT abdomen ruled out cirrhosis and gall bladder disease. He was discharged home with a prescription of lactulose. He returned to the ED with another episode of unresponsiveness a month later and was found to have elevated serum ammonia of 213 umol/L. Levetiracetam was started empirically with suspicion of recurrent seizures causing this unresponsiveness. He continued to have similar episodes of hyperammonemic encephalopathy. Orotic acid in urine was high (>200 mmol/mol creatinine ), clinching the diagnosis of ornithine transcarbamylase (OTC) deficiency. He was advised to consume a low protein diet and was prescribed L-carnitine along with lactulose.

DISCUSSION:
Ammonia directly affects neuronal electric activity by inhibiting the generation of both excitatory and inhibitory postsynaptic potentials. Secondary hyperammonemia occurs commonly in the presence of hepatic disorders leading to portosystemic encephalopathy. Less common causes include drugs like valproate, post-chemotherapy, gastric bypass surgery. Causes of primary hyperammonemia in adults include milder forms of urea cycle enzyme deficiencies like OTC, argininosuccinate lyase. Noncirrhotic hyperammonemia as a cause of altered mental status remains a diagnosis of exclusion and requires high index suspicion. Treatment options include lactulose, l-carnitine, and drugs to lower cerebral edema if patient has MRI findings. Liver transplantation is an important treatment modality in urea-cycle disorders.