scholarly journals A great mimicker of primary biliary cholangitis

2021 ◽  
Vol 84 (3) ◽  
pp. 527
Author(s):  
B Zwaenepoel ◽  
E Padalko ◽  
A Hoorens ◽  
S Vanooteghem ◽  
P Hindryckx

A 59 year-old man without past medical history was referred with biochemical features of cholestasis (aspartate aminotransferase (AST) 117 U/L, alanine aminotransferase (ALT) 83 U/L, gamma-glutamyl transferase (GGT) 1307 U/L, alkaline phosphatase (AP) 1803 U/L, total bilirubin 0,7 mg/dL), a strongly positive antimitochondrial M2 antibody (AMA-M2) titer (88 U), weight loss and abdominal pain since several months. He did not take any medications, nor there was a history of alcohol abuse or sexual risk behavior. Upon presentation, clinical examination showed a rather cachectic patient (body mass index 19 kg/m²), without further abnormalities. As primary biliary cholangitis (PBC) was suspected, treatment with ursodeoxycholic acid had been initiated but did not improve the cholestasis nor the pain. Additional investigations didn’t reveal any other irregularities, except for a mildly dilated aortic sinus root of 42 mm. The histopathological findings of a liver biopsy are shown below (Figure 1). This showed granulomas with cholangitis and increased presence of neutrophils, which raised suspicion for an infectious cause.

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Mohammad K. Choudhry ◽  
Bei Xiong ◽  
Antony Anandaraj ◽  
John Trillo

The patient is a 75-year-old man with history of diabetes and hypertension who presented with syncope after experiencing sharp, 10/10 right flank and abdominal pain worsening over three weeks associated with decreased appetite. Physical examination revealed hepatomegaly and right lower quadrant (RUQ) tenderness, negative for peritoneal signs. Bloodwork showed leukocytosis (13 K/mcl), alkaline phosphatase (141 U/L), total bilirubin (2.0 mg/dL), and gamma-glutamyl transferase (172 U/L). Computed Tomography (CT) revealed multiple hepatic cysts with the largest measuring 17 × 14 × 18 cm (Figure 1). Parenteral opiates provided minimal relief. Cardiac and neurologic etiologies of syncope were ruled out. The patient’s course was complicated by opioid-induced delirium as his abdominal pain progressively worsened despite escalating doses of parenteral and oral analgesics. Gastroenterology and interventional radiology consulted to evaluate for Glisson’s capsular stretch. Therapeutic aspiration yielded 2.5 L of serous fluid, which alleviated the patient’s pain. Cytology was negative for malignancy. Opiates were titrated down. Repeat CT (Figure 2) showed cysts that were significantly reduced in size. The patient showed complete resolution of symptoms and was subsequently discharged. We present a rare case of a large hepatic cyst causing syncope. In the appropriate clinical setting, syncope with RUQ tenderness and hepatomegaly should raise the index of suspicion for hepatic cysts.


Author(s):  
Sheila X. Soh ◽  
Tze Ping Loh ◽  
Sunil K. Sethi ◽  
Lizhen Ong

Abstract Objectives Lipemia is the presence of abnormally high lipoprotein concentrations in serum or plasma samples that can interfere with laboratory testing. There is little guidance available from manufacturers or professional bodies on processing lipemic samples to produce clinically acceptable results. This systematic review summarizes existing literature on the effectiveness of lipid removal techniques in reducing interference in clinical chemistry tests. Methods A PubMed search using terms relating to lipid removal from human samples for clinical chemistry tests produced 1,558 studies published between January 2010 and July 2021. 15 articles met the criteria for further analyses. Results A total of 66 analytes were investigated amongst the 15 studies, which showed highly heterogenous study designs. High-speed centrifugation was consistently effective for 13 analytes: albumin, alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin, creatine kinase (CK), creatinine (Jaffe method), gamma-glutamyl transferase (GGT), glucose (hexokinase-based method), lactate dehydrogenase (LDH), phosphate, potassium, and urea. Lipid-clearing agents were uniformly effective for seven analytes: ALT, AST, total bilirubin, CK, creatinine (Jaffe method), lipase, and urea. Mixed results were reported for the remaining analytes. Conclusions For some analytes, high-speed centrifugation and/or lipid-clearing agents can be used in place of ultracentrifugation. Harmonized protocols and acceptability criteria are required to allow pooled data analysis and interpretation of different lipemic interference studies.


2020 ◽  
Vol 8 (2) ◽  
pp. e001229
Author(s):  
Sylvia H Ley ◽  
Jorge E Chavarro ◽  
Stefanie N Hinkle ◽  
Mengying Li ◽  
Michael Y Tsai ◽  
...  

IntroductionLonger duration of lactation is associated with lower cardiometabolic disease risk, but pathogenic pathways involved in the disease progression are unclear, especially among high-risk women. We aimed to examine the associations of lifetime lactation duration with cardiometabolic biomarkers among middle-aged women with a history of gestational diabetes (GDM).Research design and methodsWomen with a history of GDM participating in the Nurses’ Health Study II, a prospective cohort study, were identified and followed through biennial questionnaires beginning in 1991. Lactation history was asked in three follow-up questionnaires to calculate lifetime duration. In 2012–2014, fasting blood samples were collected through the Diabetes & Women’s Health Study to measure inflammatory (C-reactive protein (CRP), interleukin (IL) 6), liver enzyme (alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyl transferase), and lipid biomarkers (total cholesterol, low-density lipoprotein cholesterol, and high-density lipoprotein cholesterol).ResultsAt follow-up blood collection, women were at median age 58.2 (95% CI 51 to 65) years and 26.3 (95% CI 15.7 to 34.1) years since GDM index pregnancy. After multiple adjustment including prepregnancy body mass index (BMI), longer duration of lactation was significantly associated with lower CRP (least squares (LS) mean 1.90 mg/L (95% CI 1.47 to 2.45) for 0-month lactation, 1.98 mg/L (95% CI 1.68 to 2.32) for up to 12-month lactation, 1.67 mg/L (95% CI 1.42 to 1.97) for 12–24 month lactation, and 1.39 mg/L (95% CI 1.19 to 1.62) for >24-month lactation; p trend=0.003) and IL-6 (1.25 pg/L (95% CI 0.94 to 1.68), 1.19 pg/L (95% CI 0.99 to 1.42), 1.04 pg/L (95% CI 0.87 to 1.25), and 0.93 pg/L (95% CI 0.78 to 1.11); p trend=0.04). Longer duration of lactation was associated with lower risk for chronic inflammation using CRP 3 mg/L cut-off in middle-aged women (OR 0.81 (95% CI 0.69 to 0.940 per 1-year increase) with multiple adjustment.ConclusionsLonger lifetime duration of lactation was associated with favorable inflammatory biomarker concentrations in middle-aged women with a history of GDM. Chronic inflammatory pathways may be responsible for previously reported associations between lactation and long-term risk for cardiometabolic diseases.


2019 ◽  
Vol 10 (1) ◽  
Author(s):  
Danielle Cristiane Baldo ◽  
Alessandra Dellavance ◽  
Maria Lucia Gomes Ferraz ◽  
Luis Eduardo C. Andrade

Abstract Background Anti-mitochondria autoantibodies (AMA) occur in > 95% primary biliary cholangitis (PBC) patients. Biochemically normal AMA-positive (BN/AMA+) individuals, occasionally noticed by indirect immunofluorescence (IIF) on HEp-2 cells and confirmed in AMA-specific assays, may represent early stages of PBC. The Enhanced Liver Fibrosis (ELF) score is a surrogate marker for liver fibrosis. This prospective study investigated the ELF score in BN/AMA+ individuals and PBC patients, considering autoantibody avidity and serum levels along the years. Methods 327 samples from 35 PBC and 59 BN/AMA+ were prospectively obtained in average 3.83 (range 0.50–7.40) years apart. Samples were tested by IIF on rat-kidney (IIF-AMA), western-blot for AMA (WB-AMA), and ELISA for antibodies against pyruvate-dehydrogenase (PDC-E2), gp210, sp100 and CENP-A/B. Anti-PDC-E2 avidity was determined by 6 M urea-elution ELISA. Alkaline phosphatase (ALP), gamma glutamyl transferase (ɣGT) and ELF score were measured by automated methods. Results Along the follow-up period BN/AMA+ subjects and PBC patients presented significant increase in serum anti-PDC-E2 (mean 10.45% and 8.86% per year; respectively), anti-PDC-E2 avidity (3.02% and 4.94%/year) and ELF score (3.24% and 2.71%/year). IIF-AMA and ɣGT increased in BN/AMA+ (6.59% and 2.36%) and decreased in PBC (− 4.89%/year and − 3.88%/year). In BN/AMA+ individuals there was positive correlation of ELF with IIF-AMA titer (r = 0.465; p < 0.001) and with anti-PDC-E2 levels (r = 0.239; p < 0.001). Expansion of autoantibody targets along time occurred in 39% BN/AMA+ and 49% PBC patients. The frequency of BN/AMA+ with high probability of having established PBC increased from 7 to 14%. Conclusions BN/AMA+ individuals present an orchestrated increase in ELF score and humoral autoimmune response over time, indicating an opportunity for early therapeutic intervention and prevention in autoimmunity.


2003 ◽  
Vol 17 (12) ◽  
pp. 713-718 ◽  
Author(s):  
Murat Kiyici ◽  
Macit Gulten ◽  
Selim Gurel ◽  
Selim Giray Nak ◽  
Enver Dolar ◽  
...  

BACKGROUND/AIMS: Nonalcoholic steatohepatitis (NASH) is a serious disorder with the potential to gradually progress to cirrhosis. It is generally associated with obesity, diabetes and hyperlipidemia. Currently, there is no established therapy for NASH. The aim of the present study was to evaluate the effectiveness of atorvastatin and ursodeoxycholic acid (UDCA) in the treatment of NASH.METHODS: This prospective study included 44 adult patients (24 men, 20 women) with a mean age of 48.90±7.69 years and mean body mass index (BMI) of 29.40±3.82. Ten patients had a history of diabetes. Serological markers for viral hepatitis were negative in all patients and there was no history of alcohol or drug abuse. Patients who had autoimmune hepatitis were excluded from the study. Liver biopsy was performed before therapy to confirm the diagnosis. Among NASH patients, 17 normolipidemic cases received UDCA 13 to 15 mg/kg/day (group 1), while hyperlipidemic cases (n=27) received atorvastatin 10 mg/day (group 2) for six months. The BMI, serum lipids, liver function tests and liver density, assessed by computerized tomography, were evaluated before and after the treatment period. The BMI, serum aminotransferase levels, histological parameters (steatosis, inflammation, fibrosis scores) and liver densities were not statistically different between the groups at the beginning of therapy.RESULTS: The BMI, serum glucose, and triglyceride levels did not change in either group after the treatment period. In group 1, serum alanine aminotransferase (ALT) and gamma-glutamyl-transferase (GGT) levels reduced significantly, and in group 2, serum cholesterol, aspartate aminotransferase, ALT, alkaline phosphatase and GGT levels reduced significantly. Liver densities increased only in group 2, probably as a result of diminishing fat content of liver. The normalization of transaminases was also more prevalent in group 2. Liver steatosis was closely correlated with liver density, but inflammation and fibrosis were not.CONCLUSIONS: The use of atorvastatin in NASH patients with hyperlipidemia was found to be both effective and safe. The benefit of statin and UDCA therapy in normolipidemic patients with NASH requires confirmation with further placebo-controlled trials.


2021 ◽  
pp. 131-136
Author(s):  
Jose Vargas-Jiménez ◽  
Jorge Vargas-Madrigal ◽  
Roberto Arias-Mora ◽  
Diana Ulate-Ovares ◽  
Bruno Solis-Ugalde

Pancreatic adenocarcinoma is the most common malignancy of the pancreas; on rare occasions, metastatic tumors are present. Differentiating a primary neoplasm from a metastatic one is important for ensuring adequate treatment for the patient. We present a case of metastatic melanoma to the pancreas. A 60-year-old man presented with a history of weight loss, vague abdominal pain, jaundice, and pruritus. Laboratory tests showed increased total bilirubin, with a direct fraction predominance, as well as increased alkaline phosphatase and gamma glutamyl transferase. Imaging studies revealed a mass in the head of the pancreas. Endoscopic ultrasound (EUS)-guided fine needle biopsy was performed, and histologic examination confirmed the diagnosis of metastatic melanoma. This case report illustrates the invaluable use of EUS-guided tissue acquisition in the study of pancreatic solid lesions to obtain an accurate diagnosis. Melanomas should always be part of a differential diagnosis when evaluating patients with pancreatic masses.


2020 ◽  
Vol 7 (50) ◽  
pp. 3001-3005
Author(s):  
Nitin Kumar Gupta ◽  
Abhilasha Singh ◽  
Rajan Goyal ◽  
Abhishek Kamendu

BACKGROUND Most cases of ACS (Acute Coronary Syndrome) are caused by rupture of an atherosclerotic plaque in a coronary artery, resulting in the formation of a thrombus. Atherosclerosis results from an over balance between radical generating, compared with radical scavenging systems, a condition called oxidative stress. Bilirubin had some role in mechanism of ACS. We wanted to evaluate the association between raised total bilirubin level and GGT levels with different subsets of acute coronary syndrome. METHODS The present descriptive cross-sectional study was conducted at Narayan Medical College and Hospital from July 2019 to April 2020. Total serum bilirubin was measured in the laboratory by spectrophotometry method. Gamma glutamyl transferase levels were measured in all the patients using a standardized photometric method. RESULTS In our study the mean value of triglyceride is 159.86 ± 42.36 with range 60 - 246. Total bilirubin 2.23 ± 0.827, with range 1 - 4. The value of gamma glutaraldehyde was 56.67 ± 26.48 with range 22 - 104. On studying the correlation of serum bilirubin and serum triglyceride in our study subjects, we found very mild correlation with an R square of 0.028. On finding correlation of GGT and serum triglyceride in our study subjects. We found almost no correlation with R square 0. When we do multivariate analysis of effect of serum bilirubin, GGT on total cholesterol level we found that there is very mild correlation with R square 0.031. CONCLUSIONS In our study there was very mild correlation between serum bilirubin and gamma glutamyl transferase with marker of acute coronary syndrome such as serum triglyceride level and total cholesterol level but the association was significant. KEYWORDS Acute Coronary Syndrome, Triglyceride, Bilirubin, Gamma Glutaraldehyde


2021 ◽  
Vol 11 ◽  
Author(s):  
Ramon V. Cortez ◽  
Luana N. Moreira ◽  
Marina Padilha ◽  
Mariana D. Bibas ◽  
Ricardo K. Toma ◽  
...  

Few studies reported the relation of intestinal microbiome composition and diversity in pediatric patients with primary sclerosing cholangitis (PSC) and ulcerative colitis (UC). In this cross-sectional study, we selected patients younger than 19 years old from the pediatric gastroenterology and hepatology outpatient clinic of a tertiary hospital to describe the intestinal microbiome of pediatric patients with PSC associated or not to UC. Patients were divided in PSC, PSC+UC, and UC diagnosis. A stool sample was collected from each patient (n=30) and from a healthy relative/neighbor (n=23). The microbiome composition was assessed using MiSeq (Illumina) platform. Differences in microbial composition were found between PSC and PSC+UC groups. The relative abundance of Veillonella and Megasphaera genera were increased depending on patients’ age at diagnosis. Veillonella was also increased in patients who were in an active status of the disease. Both genera were positively correlated to total bilirubin and gamma-glutamyl transferase. As a conclusion, the disease, the age and the disease activity status seem to influence the intestinal microbiome, highlighting the difference of intestinal microbiome profile for patients depending on age at diagnosis. We also showed an increase of Veillonella in patients with PSC and PSC+UC, and a positive correlation of dysbiosis and higher gamma-glutamyl transferase and total bilirubin in PSC+UC patients. Our findings are promising in the diagnosis, prognosis, and future therapeutic perspectives for PSC patients.


2020 ◽  
Vol 148 (7-8) ◽  
pp. 423-429
Author(s):  
Aleksandra Klisic ◽  
Nebojsa Kavaric ◽  
Ana Ninic

Introduction/Objective. Given the contradictory results regarding the association of liver function biomarkers [e.g., alanine-aminotransferase (ALT), gamma-glutamyl transferase (GGT) and total bilirubin)] and the risk of cardiovascular disease (CVD), we aimed to explore the relationship between these biomarkers and Framingham risk score (FRS), an established tool used in the prediction of 10-year CVD risk in the cohort of women. Methods. A total of 278 women participated in this cross-sectional study. Anthropometric, biochemical parameters, and blood pressure were obtained. Results. There was a significant increase in ALT and GGT activity, as well as a decrease in total bilirubin level in the high-risk FRS group compared to moderate-, and low-risk FRS (p for trend = 0.025, p < 0.001, p < 0.001, respectively). Multivariate logistic regression analysis showed that body mass index, triglycerides, creatinine, and high sensitivity C-reactive protein levels were the independent predictors of FRS in women [odds ratio (OR) = 1.234, p = 0.001; OR = 2.856, p = 0.001; OR = 1.090, p = 0.002, and OR = 1.295, p = 0.045, respectively]. In contrast, total bilirubin, ALT and GGT lost their independent predictions for high CVD risk. Conclusion. Liver function biomarkers (i.e. ALT, GGT, and total bilirubin) are not independently associated with FRS. It seems that some other cardiometabolic disturbances might modulate this relationship.


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