Normal Liver Function Tests Research Articles

An Implicated Innocent Bystander – An Uncommon Cause of Colonic Obstruction

Question: An elderly gentleman presented with a 1-week history of colicky epigastric pain, associated with nonbilious vomiting and nonbloody diarrhea. He also has a background of hypertension, type 2 diabetes mellitus, chronic alcoholism with recurrent pancreatitis, and longstanding normocytic normochromic anemia. Physical examination revealed epigastric tenderness with no other signs of peritonism. Laboratory investigations showed a normal white cell count of 8.17 × 103 /μL (reference range, 4–11 × 103 /μL) and normal liver function tests.

Влияние курсового приема L-орнитин- L-аспартата на фиброз и стеатоз печени у больных ассоциированной с нарушениями метаболизма жировой болезнью печени (неалкогольной жировой болезнью печени), имеющих гипераммониемию

Introduction.Hyperammonaemia develops both in cirrhosis and earlier fibrotic stages during metabolic-associated fatty liver disease (MAFLD). Besides neurotropic, ammonia exerts the hepatotoxic and profibrotic effects. L-ornithine-L-aspartate (LOLA) has been proved effective in treatment for hyperammonaemia in cirrhosis patients.Aim.An impact study of the LOLA course therapy on inflammation, steatosis and liver fibrosis biomarkers in MAFLD.Materials and methods.A total of 90 patients were divided between two cohorts. The control cohort included patients with liver steatosis S0–S1, absent liver fibrosis, normal liver function tests, clean history of liver disease, while MAFLD cohort gathered liver steatosis S2–S3 and METAVIR fibrosis F1. Steatosis and fibrosis were assessed with a Fibroscan 502 unit with CAP measurement. All patients had ammonia estimated from whole blood. At high ammonia, LOLA was ordered at 9 g/day for 8 weeks, with control of blood ammonia, AST, ALT, GGT, CRP and ferritin, as well as fibrosis and steatosis post-therapy.Results.The study enrolled 45 patients of the MAFLD and 45 — of control cohort. Hyperammonaemia was revealed in 26 (58 %) MAFLD and 3 (7 %) control patients (p <0.001). MAFLD-hyperammonaemic patients also had the significantly higher male ratio, type 2 diabetes and severer hepatic steatosis rates vs. hyperammonaemia-negative MAFLD individuals. In 8 weeks of LOLA therapy, the ALT, AST, GGT, ferritin and CRP levels decreased significantly, and blood ammonia attained normal range (p <0.001). Elastometry liver stiffness decreased in 22 (85 %) patients, reaching F0 values in 6 cases (p <0.001). The steatosis grade reduced in 18 (69 %) individuals.Conclusion.LOLA normalises blood ammonia levels and reduces the severity of inflammation, steatosis and liver fibrosis.

A Percutaneous Portal Vein Puncture Under Artificial Ascites for Intraoperative Hepatic Segmentation Using Indocyanine Green Fluorescence: A Technical Report of Laparoscopic Anatomic Liver Resection.

Background:Laparoscopic liver resection have developed and is widely spread as standard procedure in these days, however, laparoscopic anatomic liver resection is still challenging, especially for posterosuperior lesions because of difficulties in segmental mapping and surgical techniques. Recently, the positive staining and negative staining method using fluorescent imaging techniques have been reported from experienced Asian centers, allowing to identify the tumor-bearing portal territory to be resected including the posterosuperior segment in laparoscopy. Those techniques are applicable in some cases; hence, it remains the room for improvement to establish as a feasible approach. Herein, we describe a percutaneous tumor-bearing portal vein puncture method under artificial ascites after the pneumoperitoneum for laparoscopic segmentectomy for segment 8.Case Presentation and Surgical Procedure:A male patient in his 60s was admitted for an incidentally diagnosed hepatic mass in segment 8. Findings of the computed tomography scan showed a 2.5-cm-sized hepatocellular carcinoma lesion. Then, laparoscopic anatomic liver resection for segment 8 was planned. The segmentation of the segment 8 was performed through a percutaneous tumor-bearing portal vein puncture using indocyanine green injection with extracorporeal ultrasound guidance under artificial ascites. According to indocyanine green fluorescence navigation, anatomic liver resection was completed. Operative time was recorded as 375 minutes. The estimated intraoperative blood loss was 50 mL without the requirement for an intraoperative transfusion. The planned resections were successful with histologically negative surgical margins. The patient was discharged on the 19th postoperative day with normal liver function test results. There was no operation-related complication during hospitalization.Conclusion:The intraoperative percutaneous portal vein puncture method under artificial ascites was useful for the identification of posterosuperior segment in laparoscopic anatomic segmentectomy.

921. Acute HAV Infection in an Inpatient Psychiatry Unit

BackgroundThe incidence of hepatitis A virus (HAV) infection has been rising in the US since 2016, and in New York State since 2019. New York City has also seen an increase of HAV infection among high risk populations. We present a case of acute HAV infection in an inpatient psychiatry unit which has its own unique isolation and management challenges. MethodsA patient was admitted on 3/21/21 from a group home. He developed abdominal pain, diarrhea and vomiting on 4/15, with elevated liver function tests (LFT). He was transferred to Medicine on 4/17 and HAV IgM and IgG resulted positive on 4/18. Visitation to the unit has been halted for over a year, and no outside food has been allowed. The patient has not been observed to have any sexual exposure to others. InvestigationExposure window: 15 days prior to start of symptoms. Patients in the unit were screened for symptoms, tested for HAV IgM/IgG, LFTs. Discharged patients were contacted and referred straight for vaccination (difficult to have multiple visits). Staff members with contact to the unit were screened, via email and phone calls. If no previous vaccination and there was presence of exposure or symptoms, staff were referred to Occupational Health Services (OHS). Other Measures: The unit was terminally cleaned and daily enhanced cleaning with bleach ensued. Daily assessment of patients and staff for symptoms. Admissions were held for 2 days until all the patients were tested and given vaccine. Further admissions were screened for HAV. Results32 inpatients screened. One patient was positive for HAV IgM, but was asymptomatic with normal LFTs. On investigation, patient had acute hepatitis in February 2021. Patients with no immunity were vaccinated. Two immunocompromised patients were also given HAV immunoglobulin. On chart review, 6 out of 29 discharged patients had evidence of immunity. 133 staff were screened and 54 referred to OHS (see table).Exposure Investigation ConclusionAs evident with numerous COVID outbreaks in inpatient Psychiatry units, communicable diseases are difficult to control. Patients are in an interactive communal setting and participate in group sessions. For better care and safety of patients and staff, our unit will screen and offer HAV vaccine to new admissions.Disclosures Gregory Weston, MD MSCR, Allergan (Grant/Research Support) Inessa Gendlina, Nothing to disclose

Hepatitis in Idiopathic Hypereosinophilic Syndrome: Report of an Unusual Case

World Health Organization defines a rare diagnosis Idiopathic hypereosinophilic syndrome (HES) as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. Most of the patients present with nonspecific symptoms, while others will present with symptoms of the affected organs, commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with idiopathic HES related hepatitis and achalasia. Hypereosinophilic syndrome has been reported to be associated with hepatic dysfunction; liver histology is mainly characterized by a diffuse eosinophilic inflammatory infiltrate. A 49-yr-old woman, diagnosed as a case of idiopathic hypereosinophilic syndrome with bone marrow and pulmonary eosinophilic infiltrates associated with peripheral eosinophilia,high IgE level developed features of chronic gatrornteritis, hepatitis, with a significant eosinophil component. She responded well to systemic glucocorticoid and Imatinib therapy with normalization of liver function tests within a few weeks.

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan.

Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high-difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short-term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC-CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS.

Hepatic Adenomatosis in a Young Asymptomatic but High-risk Patient: An Incidental Diagnosis with Potentially Severe Clinical Implications.

Hepatic adenomatosis (HA) is a very rare condition and defined as the presence of 10 or more adenomas in an otherwise normal liver. HA has an incidence of 10–24% in patient with hepatic adenoma and it is more common in women. Most patients with HA are asymptomatic with a normal liver function test and half of cases are detected incidentally on imaging. Although HA is considered a benign disease, some patients may develop potentially fatal complications, such as hypovolaemic shock due to rupture of the liver lesion or malignant transformation to hepatocellular carcinoma.We report the case of a 29-year-old woman who presented to the emergency room after a car accident. Whole-body computed tomography revealed multiple focal hepatic hypervascular lesions in the right lobe of the liver together with a fatty liver. Subsequent hepatic magnetic resonance imaging suggested the diagnosis of HA with a suspicion of focal nodular hyperplasia (FNH). The patient refused to undergo liver biopsy, so we instituted a 3-month surveillance program, which included clinical assessment, liver function tests, tumour marker assessment and blood tests as well as sonographic evaluation for follow-up of the liver lesions.LEARNING POINTSHepatic adenomatosis is an extremely rare disease which predominantly affects women and is associated with hormone consumption, liver steatosis, glycogen storage disease, obesity, metabolic syndrome, abnormalities of hepatic vasculature and maturity onset diabetes of the young (MODY).The two main differential diagnoses include focal nodular hyperplasia (FNH) and well-differentiated hepatocellular carcinoma. Histological confirmation is required when MRI findings are inconclusive or when a malignancy is suspected.In men, resection of adenomas is mandatory due to the high risk of malignant transformation. In woman, a conservative approach with contraceptive discontinuation and biannual follow-up with MRI and alpha-fetoprotein is recommended; resection is needed in case of positive immunohistochemical staining for β-catenin on biopsy, symptomatic adenomas, adenoma increasing in size, and when malignancy cannot be ruled out.

EP.TU.439Falciform ligament torsion – a rare cause of the acute abdomen

Abstract The falciform ligament attaches the liver to the anterior abdominal wall and diaphragm. Acute falciform ligament related pathology is rare. In this case report we present a case of acute fat necrosis related to the falciform ligament. A 53 year old women presented with acute upper abdominal pain localised to the right hypochondrium. He was tender to palpation in the same region with a positive Murphy’s sign. A provisional diagnosis of acute cholecystitis was made. Blood work revealed raised inflammatory markers but normal liver function tests. Abdominal ultrasound revealed no gallbladder pathology nor gallstones. Thus computed tomogram (CT) scan of the abdomen was performed, showing hyper-attenuation rim signal present within the inferior aspect of the falciform ligament consistent with local vascular occlusion. The patient was managed with intravenous antibiotics with liberal analgesia and went on to make a successful recovery. Only 10 cases have been reported in the literature related to falciform ligament necrosis. We present this unusual pathology encountered on our acute surgical take, to alert surgeons to this rare diagnosis and provide a review of the literature and provide detail of how such a pathology manifests on CT scan.

S2668 Sorafenib-Induced Autoimmune Hepatitis: A Case Report,

Introduction: Drug-induced liver injury (DILI) with autoimmune hepatitis-like features (DIAIH) is a specific phenotype of DILI with clinical features indistinguishable from de-novo autoimmune hepatitis (AIH). We describe a case of DIAIH due to Sorafenib. Case Description/Methods: A 61-year-old man and teetotaller presented with 1-week history of jaundice and malaise. He was on Atorvastatin 40mg daily for 4 years for hyperlipidaemia. Sorafenib was recently initiated six weeks ago for recurrent sarcoma of the left thigh. Clinical examination was unremarkable apart from scleral icterus. Liver function test (LFT) showed severe hepatocellular (HC) injury (bilirubin 4.56 mg/dL, alkaline phosphatase 190 U/L, alanine transaminase 1004 U/L, aspartate transaminase 790 U/L peak INR 1.53). Viral hepatitis screening, AIH specific antibodies and abdominal imaging were unremarkable. Serum IgG was raised (18.6 g/L). Liver biopsy was supportive of DILI and AIH histological features were also noted (Fig.1a-f). Simplified AIH score was 6. Roussel Uclaf Causality Assessment Model (RUCAM) score was 9 for Sorafenib and 6 for Atorvastatin. Diagnosis of Sorafenib-induced AIH was made. His LFT improved spontaneously with normalization of LFT eight weeks after stopping Sorafenib. Discussion: Sorafenib often causes idiosyncratic DILI with mild elevation of transaminases. However, it rarely causes severe DILI and has no known association with DIAIH, unlike Atorvastatin and immune checkpoint inhibitors (e.g Nivolumab and Atezolizumab). To our knowledge, this is the first case report of DIAIH due to Sorafenib. In patients with severe DILI or DILI with probable AIH based on AIH scoring, liver biopsy should be considered for evaluation. However, differentiating DIAIH from de-novo AIH is often challenging as both present with HC injury, may have positive AIH specific antibodies, raised serum IgG and share similar histological findings. The key distinguishing features are the history of drug exposure within a time frame and no requirement for long term immunosuppressant. DIAIH may resolve spontaneously after stopping culprit drug. Corticosteroids should be initiated in cases without improvement upon drug cessation. In DIAIH requiring corticosteroids, relapse is rare after stopping corticosteroids and long-term immunosuppression is rarely needed. In conclusion, DIAIH should be considered in patients who present with severe HC DILI. Differentiating DIAIH from AIH is crucial as the former rarely requires long term immunosuppression.Figure 1.: Liver biopsy specimen [a] H&E 200X: A portal tract, zone 1, showing moderate chronic inflammation with interface damage and rare bile droplets. [b] H&E 200X: Lobulitis characterized by aggregates of plasma cells, swollen hepatocytes and rosetting within liver parenchyma zone 2. [c] H&E 200X: Centrilobular region zone 3 showing hepatocyte drop-out, aggregates of plasma cells, rosetting and bile pigment. Reticulin [d, 40X] and Sirius Red [e, 40X] special stains show collapse with mild early young fibrosis without elastic fibers demonstrated on Orcein [f, 40X], consistent with subacute injury. Overall, the appearances are supportive of subacute DILI in association with features suggestive of AIH (i.e. interface damage, plasma cell aggregates and resetting).

S2088 Acute Esophageal Necrosis: Patient with a History of Liver Cancer on Capecitabine

Introduction: Acute esophageal necrosis (AEN) is a life-threatening medical condition. It is characterized endoscopically by extensive black mucosal discoloration of the esophagus (1). To date, the etiology of the AEN is unknown, but it was suggested that vasculopathy or hemodynamic instability is the main contributor. Although esophageal necrosis seems a rare condition, gastric mucosal necrosis is even more rarely seen given the rich network of collaterals supplying this part of the gastrointestinal tract (2,3). Case Description/Methods: A 75-year-old female with intrahepatic cholangiocarcinoma on Capecitabine presented to the emergency room with dysphagia, odynophagia, and palpitation. Vital signs were remarkable for a heart rate (HR) of 151 bpm irregularly irregular, respiratory rate 23/min, blood pressure (BP)145/81mmHg. She had white blood count (WBC) 10,800/L, hemoglobin (Hgb) 16.7 g/dL, hematocrit 51%, platelet count 182,000/uL, albumin 2.9 g/d, prothrombin time 21.9 sec, INR 1.99, partial thromboplastin time 67.7 sec, and normal liver function test. Patient had sudden onset of maroon output from orogastric tube, and bright red blood per rectum with acute drop in BP to 90/38 mmHg, Hgb level 9.2 g/dL, lactic acid 13.3 mmol/L, WBC 27,3500/uL. Alanine aminotransferase and total bilirubin were 3100 unit/L, and 3.4 mg/dL, respectively. Patient was started on amiodarone, vasopressors, broad spectrum antibiotics, protonix and octreotide drips for hypovolemic shock due to acute gastrointestinal blood loss complicated with sepsis. Patient was intubation on mechanical ventilation for airway protection. Urgent esophagogastroduodenoscopy showed black necrotic esophagus (Fig.1a & b). The gastric mucosa was oozing and congested with a purplish discoloration (Fig. 1c). No endoscopic treatment was applied, and patient was continued on protonix drip. Discussion: We here discuss previously unseen association of esophageal and gastric necrosis in a patient on Capecitabine. To date, no clinical cases discussed Capecitabine-associated AEN. Although the mechanism of Capecitabine-induced ischemia is not fully understood, mesenteric vasoconstriction and thrombosis seem to cause ischemia (4,5). Also, the mucosal injury from chemotherapy causing massive release of proinflammatory markers contributing to tissue necrosis (6). The outcome of our patient illustrates that it is essential to keep high level of suspicion in AEN predisposing factors, and that the association of Capecitabine and AEN also needs to be looked at further.Figure 1.: Endoscopy image showing esophageal stricture.

S2369 Ileocecal Dieulafoy’s Lesion: A Rare Case of Lower Gastrointestinal Bleeding

Introduction: A Dieulafoy’s lesion (DL) is one of the rare causes of gastrointestinal bleeding characterized by a submucosal arterial lesion that becomes exposed due to a mucosal defect. These lesions are most commonly located in the proximal stomach, but the involvement of the small intestine is extremely rare. A Dieulafoy’s lesions can be asymptomatic; however, lesions with profound bleeding require medical attention. Here we report a rare case of the lower GI bleed secondary to an ileocecal valve DL in an elderly female. Case Description/Methods: A 79-year-old female with a past medical history of hypertension, Type 2 diabetes, ESRD on hemodialysis, and Atrial Fibrillation on apixaban presented with generalized weakness and melena for 2 weeks. No abdominal pain, weight loss, or hematemesis were reported. There was no history of GI bleeding or the use of non-steroidal anti-inflammatory drugs. On admission, blood pressure was 100/67 mm Hg, and pulse was 104 beats/min. Abdomen was soft, non-tender, non-distended without hepatosplenomegaly, bowel sounds were normal, and no hemorrhoids, nor masses on rectal exam. Hb level was 6.7gm/dl, HCT 21.3%, MCV 103.4. The patient also had a normal platelet count and liver function tests. Apixaban was discontinued and four units of blood were transfused. Urgent endoscopy was unremarkable. Colonoscopy showed a Dieulafoy’s lesion at the ileocecal valve that was actively oozing. Injection of epinephrine solution failed to stop the hemorrhage, but the placement of three hemoclips resulted in immediate hemostasis. She remained stable and was subsequently discharged home on day 5. Discussion: Although Dieulafoy’s lesions are extremely rare, they should be considered in the differential diagnosis of lower GI bleeding. The endoscopic approach is the gold standard for the diagnosis and management of these lesions. Our patient, who presented with overt GI bleeding resulting from Dieulafoy’s lesion at the ileocecal valve was successfully treated with an application of three hemoclips after failed injection therapy.Figure 1.: (A) DL lesion at Ileocecal valve area. (B) Hemostasis after placing hemoclips.

GRANULOCYTIC SARCOMA OF THE CNS AS A CAUSE OF MEDULLARY COMPRESSION: A CASE REPORT

A 24-year-old man, previously healthy, presented to the hospital emergency in February 2021, complaining of pain in the back region radiating to the flanks and hips for 12 days. He had previously sought care on three occasions, without relief with anti-inflammatory drugs. On admission, his physical examination was normal, and laboratory tests showed 23900 leukocytosis and 70% blasts, no anemia or thrombocytopenia, normal renal and liver function tests, and negative serologies. Chest radiography and ultrasound of the abdomen plus urinary tract showed no particularities. Immunophenotyping was requested, which diagnosed maturation acute myeloid leukemia; the patient was hospitalized for therapy. He still complained of intermittent pain crises in the lumbar region, evolving with bladder dysfunction, weakness, and paresthesia in both lower limbs. Upon starting protocol with Cytarabine + Daunorubicin on the fourth day of hospitalization, the man evolved with proprioceptive loss and paraplegia of the lower limbs. Urgent magnetic resonance was requested, showing an expansive formation in the posterior epidural space at the level of T9-T11, suggestive of granulocytic sarcoma. Lesion biopsy was not performed. A course of dexamethasone was carried out and a response to chemotherapy was observed; the patient had a partial return of strength in both lower limbs and completed the systemic treatment. Acute myeloid leukemia (AML) is the most prevalent acute leukemia in adults, representing about 1% of all neoplasms, 10% of which are of the subtype called AML with maturation. This is the subtype most associated with granulocytic sarcoma, also called chloroma. Granulocytic sarcoma is a solid tumor of granulocyte precursor cells located in various extramedullary sites, which can cause a copious constellation of symptoms. In this case, MRI showed a highly suspicious lesion for infiltrative chloroma in the CNS, causing a medullary compression syndrome with serious clinical repercussions. In other tissues, local management of the tumor can be performed with surgical resection or radiotherapy, which were unfeasible therapeutic options for the case presented. Although rare, granulocytic sarcoma should be considered a differential diagnosis in patients with acute leukemia, who present with compressive symptoms. In some cases, it may represent a hematological urgency, even before the primary neoplasm diagnosis, and its correct management is essential in such situations.

UNILATERAL CRYSTAL METHAMPHETAMINE LUNG

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Methamphetamine is a drug manufactured from ephedrine and pseudoephedrine mixed with ingredients such as ammonia, lithium, iodine, and phosphorous but adulterants such as alcohol, acetone, and energy drinks have also been used. Smoking is the most common form of abuse with complications such as eosinophilic pneumonia, alveolar hemorrhage, acute interstitial pneumonia, and pulmonary fibrosis. We present an atypical methamphetamine-induced lung injury. CASE PRESENTATION: A 57-year-old female with a 40-pack year smoking history and polysubstance abuse presented with acute onset shortness of breath. On admission, she was hypoxic requiring 25 liters of high flow oxygen. On examination, the patient was afebrile with normal blood pressure. Chest auscultation revealed wheezing, most prominent in the left lung fields, while the rest of the systemic examination was unremarkable. Laboratories showed leukocytosis, normal liver, and renal function test. Sputum culture had no bacterial growth and Covid-19 PCR negative. Urine toxicology was positive for methamphetamine. Contrast CT chest showed diffuse interstitial thickening and ground-glass opacities throughout the right lung but normal left lung parenchyma with narrowing of the distal left mainstem bronchus by an endobronchial mass. The patient's hypoxemia improved with Methylprednisolone. Bronchoscopy showed a left mainstem lesion with near-complete obstruction of the left upper and lower lobes with biopsy revealing squamous cell carcinoma. There was normal airway mucosa on the right lung segments and scant inflammation without malignant cells on biopsy. The patient underwent successful tumor debulking with follow-up PET CT demonstrating decreased but persistence of alveolar septal thickening on the right lung with FDG positive mediastinal lymph nodes. The patient was weaned off supplemental oxygen 4 weeks later. DISCUSSION: Diagnosis of Methamphetamine-induced lung injury depends on the history of recent use, ground-glass opacities on imaging, and exclusion of other infections which was fulfilled by our patient but with the question of left lung sparing. Crystalline particles range in size from less than 250 nm-10 μm, with clusters as large as 80 μm where the obstructing mass may have prevented the flow of the drug vapor. Methamphetamine has also been postulated to release early response cytokines including TNF-alpha and IL-6 leading to recruitment of neutrophils and free radicals release causing acute lung injury. The management is mainly supportive with empiric steroids with improvement via impeding this inflammatory cascade. CONCLUSIONS: Our case demonstrates multiple learning points including particle size factor in passage through an obstructed airway, response to steroids supporting in settings of acute inflammation, and the persistence of residual lung disease on imaging in contrast to clinical improvement 4 weeks post-exposure. REFERENCE #1: Javed A, Nasrullah A, Malik K (February 08, 2021) A Rare Case of Methamphetamine-Induced Lung Injury During the Ongoing COVID-19 Pandemic. Cureus 13(2): e13215. doi:10.7759/cureus.13215 REFERENCE #2: Kinner SA, Degenhardt L. Crystal methamphetamine smoking among regular ecstasy users in Australia: increases in use and associations with harm. Drug Alcohol Rev. 2008 May;27(3):292-300. doi: 10.1080/09595230801919452. PMID: 18368611. REFERENCE #3: Lin SS, Chen YC, Chang YL, Yeh DY, Lin CC. Crystal amphetamine smoking-induced acute eosinophilic pneumonia and diffuse alveolar damage: a case report and literature review. Chin J Physiol. 2014 Oct 31;57(5):295-8. doi: 10.4077/CJP.2014.BAC201. PMID: 25241990. DISCLOSURES: No relevant relationships by Uchit Thapa, source=Web Response

Синдром Жильбера: клініка, діагностика, диференціальна діагностика та лікування (частина 2)

роведений огляд наукової літератури за ключовими словами «синдром Жильбера», «клініка», «діагностика», «лікування» з використанням як пошукової системи PubMed. Беручи до уваги дослідження, що проведені за останні 10 років, проаналізовано тези 123 статей. Критерій відбору статей для дослідження був заснований на актуальності їх теми. Більш детально вивчено результати дослідження, що висвітлені в 50 статтях. Клінічні прояви синдрому Жильбера (CЖ) можливі як при гомозиготному статусі за геном UGT1А1, так і при гетерозиготному. У осіб із гетерозиготним статусом переважає латентний варіант перебігу захворювання. У 30 % гомозиготних осіб за дефектним геном СЖ хвороба також перебігає безсимптомно. Клінічні варіанти перебігу СЖ: диспептичний — 43,2 %, астеновегетативний — 15,9 %, жовтяничний — 14,8 %, безсимптомний — 26,1 %. Провокуючими факторами є погрішності в харчуванні, розумова перевтома, стрес, травми, гострі інфекції, зокрема вірусний гепатит, прийом деяких препаратів. У 86,4 % дітей із СЖ виявлена патологія верхніх відділів шлунково-кишкового тракту: дуоденіт — у 58 %, гастрит — у 56,8 %, езофагіт — у 12,5 %, виразкова хвороба дванадцятипалої кишки — у 2,3 %, у 39,7 % хворих зареєстровані сфінктерні порушення (гастроезофагеальний, дуоденогастральний рефлюкси). Для пацієнтів із СЖ характерно зниження детоксикаційної функції печінки, зокрема у 2/3 хворих відзначається порушення поглинальної здатності гепатоцитів, у половини — страждає екскреторна функція печінкових клітин, у 88 % спостерігаються дисфункція жовчовивідних шляхів, підвищений ризик утворення жовчних каменів. В окремих пацієнтів із СЖ відзначають особливості психіки, зокрема наявність тривожності. Діагностичні критерії СЖ: 1) своєрідний світло-жовтуватий колір шкіри (teinte bilieuse), особливо обличчя, кистей і стоп, без вираженої жовтяниці склер. Іноді розвиваються повторно інтермітуючі напади жовтяниці з високою білірубінемією (непрямий білірубін) без ознак гемолізу (диференціально-діагностична ознака); 2) схильність до розвитку пігментних і судинних невусів і ксантелазм у ділянці повік, а також гіперпігментації навколо очей, до брадикардії, гіпотермії, мігрені, ортостатичної, переміжної альбумінурії або аліментарної глюкозурії; 3) посилена тенденція до пігментоутворення під впливом світла та тепла, а також хімічних і механічних подразників; 4) нейром’язова гіперзбудливість; 5) підвищена чутливість до холоду; 6) диспептичні скарги (біль, нудота, відчуття повноти шлунка, пронос або запор); 7) відсутність ознак підвищеного гемолізу (диференціально-діагностична ознака) при збільшенні вмісту в сироватці непрямого білірубіну (диференціально-діагностична ознака); 8) у більшості хворих функціональні проби печінки нормальні (диференціально-діагностична ознака), проба з виділенням бромсульфалену також зазвичай нормальна (диференціально-діагностична ознака); 9) біохімічна аномалія не виявляється гістологічними методами (диференціально-діагностична ознака); 10) часто виявляють сімейну захворюваність печінки. Диференціальну діагностику СЖ проводять з усіма типами гіпербілірубінемій, гемолітичними анеміями, уродженим цирозом печінки, гепатитами, холецистопатіями, атрезією жовчних ходів або тонкої кишки. Медикаментозні препарати застосовують виключно при вираженій гіпербілірубінемії та як супутню терапію за наявності в клінічній картині симптомів гіповітамінозу, порушень моторно-евакуаторної функції верхніх відділів травного тракту, для запобігання ускладненням (жовчнокам’яна хвороба).

The role of endoscopic ultrasound in evaluating patients with bile duct dilation of unclear etiology.

Bile duct dilation (BDD) of unclear etiology is a common indication for further imaging via endoscopic ultrasound (EUS). We aimed to assess the yield of EUS in determining BDD etiology in patients with prior non-diagnostic imaging studies. A retrospective chart review was performed at a single, tertiary-care university hospital for patients referred for EUS for BDD with or without pancreatic duct dilation (PDD). EUS-guided fine needle aspiration (FNA) was performed if a focal lesion was identified. Cases with an etiology of BDD diagnosed or strongly suggested by prior imaging were excluded. EUS findings believed to represent a structural cause for BDD included a wide range of pancreaticobiliary and luminal pathology as well as patients' clinical factors. In total, 307 patients were identified. Findings to explain BDD were found by EUS in 213 patients for a diagnostic yield of 69.4%. Patients with jaundice were significantly more likely to receive a diagnosis by EUS than those without (78.8% vs 55.3%, P < 0.01). Notably, 8.1% of patients with normal liver function test (LFT) had a EUS-diagnosed malignancy. Patients' age, narcotic use, concurrent PDD and prior cholecystectomy did not appear to influence the EUS yield. EUS continues to play a substantial role in evaluating BDD of unclear etiology, most notably in patients with jaundice. In addition, given that 8.1% of asymptomatic patients without jaundice or abnormal LFT had malignancy diagnosed on EUS, the use of EUS for BDD of unclear etiology remains warranted.

Fibroblast growth factor 7 releasing particles enhance islet engraftment and improve metabolic control following islet transplantation in mice with diabetes

Transplantation of islets in type 1 diabetes (T1D) is limited by poor islet engraftment into the liver, with two to three donor pancreases required per recipient. We aimed to condition the liver to enhance islet engraftment to improve long‐term graft function. Diabetic mice received a non‐curative islet transplant (n = 400 islets) via the hepatic portal vein (HPV) with fibroblast growth factor 7‐loaded galactosylated poly(DL‐lactide‐co‐glycolic acid) (FGF7‐GAL‐PLGA) particles; 26‐µm diameter particles specifically targeted the liver, promoting hepatocyte proliferation in short‐term experiments: in mice receiving 0.1‐mg FGF7‐GAL‐PLGA particles (60‐ng FGF7) vs vehicle, cell proliferation was induced specifically in the liver with greater efficacy and specificity than subcutaneous FGF7 (1.25 mg/kg ×2 doses; ~75‐µg FGF7). Numbers of engrafted islets and vascularization were greater in liver sections of mice receiving islets and FGF7‐GAL‐PLGA particles vs mice receiving islets alone, 72 h posttransplant. More mice (six of eight) that received islets and FGF7‐GAL‐PLGA particles normalized blood glucose concentrations by 30‐days posttransplant, versus zero of eight mice receiving islets alone with no evidence of increased proliferation of cells within the liver at this stage and normal liver function tests. This work shows that liver‐targeted FGF7‐GAL‐PLGA particles achieve selective FGF7 delivery to the liver‐promoting islet engraftment to help normalize blood glucose levels with a good safety profile.

Liver dysfunction in idiopathic pulmonary arterial hypertension: prevalence, characteristics and prognostic significance, a retrospective cohort study in China

ObjectivesThe aim was to elucidate the relationship between liver function and idiopathic pulmonary arterial hypertension (IPAH).Design and settingRetrospective, longitudinal study in urban tertiary care centre in Shanghai, China.Participants407 IPAH consecutive...

Description of patient with telangiectasis and palmar erythema without any structural liver changes or changes of liver laboratory parameters due to treatment of Her -2 -positive breast cancer with Ado-Trastuzumab-Emtansine

Ado- Trastuzumab-Emtansine is approved by EMA and FDA for treatment of HER-2-positive metastatic breast cancer. The drug combines the cytotoxic activity of emtansine with trastuzumab [1,2,3]. Here we are describing 50- year old woman with 3-ple positive left breast cancer with metastasis to left axillary lymph nodes treated with the drug. The patient had normal liver and spleen structure on CT of the abdomen with I.V. contrast, along with a normal liver function test. However she did have a mild elevation of her indirect bilirubin after the initiation of the drug. Despite the normal liver and spleen and absence of Cirrhosis the patient developed drug induced spider angiomas on the upper chest, upper back, shoulders along with palmar erythema. The association of the Ado-Trastuzumab-Emtansine with spider angiomas and palmar erythema in those with normal liver and spleen function, with only a mild elevation of indirect bilirubin is discussed in the article [1].

Intrahepatic intraductal papillary mucinous neoplasm (IPNM): Case report

Background: IPMN is a rare condition in which the bile ducts are dilated and filled with papillary or villous neoplastic epithelium. IPMNs usually are of pancreatic or extrahepatic origins. Intrahepatic variation of IPMNs is increasingly being reported [4], and a common feature in reported cases is bile duct dilatation. Methods: 65-year-old man was being investigated for iron deficiency anaemia (IDA) and there was an incidental finding of a liver lesion on Commuted Tomography (CT) scan. He was then referred to the Hepatobiliary (HPB) team for management. Following management, his case files including clinic letters, test results, radiological imaging, operative notes, records of admissions and histopathology reports were reviewed for this retrospective case report. Results: At first presentation in the HPB clinic, he was asymptomatic with normal liver function tests and the plan was referral for Multidisciplinary team (MDT) discussion. The case was discussed, and decision was to manage as suspected early cholangiocarcinoma with liver resection. He was electively admitted and had a left lateral sectionectomy. He had an uneventful peri-operative period and discharged home on recovery. Histology reported the specimen as a cystic variant of IPMN arising in intrahepatic bile duct with no evidence of invasive cancer. After this, there was a further discussion at MDT and the plan was for discharge. This happened in follow up clinic, at which time patient remained asymptomatic and well overall. Conclusion: Intrahepatic IPMNs are rare premalignant lesions. There are no specific symptoms, so diagnosis is often post liver resection.

Maternal and fetal outcome in liver disorders in pregnancy

Background: Liver disorders comprise 3% of all pregnancy complications. All liver disorders, pregnancy specific, pregnancy related and pregnancy unrelated disorders have both maternal and fetal effects. The aim of the study was to determine the effect of liver disorders in pregnancy. The objective of this study was to determine the effect of liver disorders on pregnancy outcome.Methods: All pregnant patients with clinical and biochemical evidence of liver dysfunction were taken as cases. Pregnant women with normal liver function tests were taken as controls. All patients were followed during pregnancy and postpartum. Maternal and fetal outcome was studied.Results: A total of 140 patients were included- 70 cases and 70 controls. Maternal and fetal outcome was studied in both the groups. PPH and oligohydroamnios were most frequent among cases with p value of 0.034 and 0.035 respectively. Similarly, pre-term birth, RDS and perinatal asphyxia was more common in cases with a p value of 0.011, 0.001 and 0.005 respectively.Conclusions: Study concludes that liver disorders in pregnancy have adverse maternal and fetal complications.