Normal Liver Function Tests Research Articles

OGBN P32 Outcomes of patient with Biliary Dyskinesia undergoing laparoscopic cholecystectomy - A multicentre cohort study

Abstract Background Biliary dyskinesia (BD) is a benign disorder characterized by recurrent episodes of biliary pain in the absence of gallstones on ultrasound, normal liver function tests and an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid (HIDA) scan. Laparoscopic cholecystectomy (LC) remains the only known effective treatment for BD. However, many surgeons are reluctant to offer LC in such patients due to the fear of non-resolution of symptoms. Our aim was to analyse the outcome of patients who underwent LC for BD in terms of resolution of the biliary symptoms. Methods Multicentre retrospective cohort study of patients who have BD proven on HIDA scan from January 2015 to December 2022. BD was defined as EF ≤35% on HIDA scan. Those with EF ≤35% but had evidence of gallstones, microlithiasis or post op leak were excluded from the study. Electronic case notes were reviewed for demographic details, duration of symptoms, body mass index (BMI), liver function test (LFT), radiological investigations, endoscopy report and histopathology results of cholecystectomy. Rome IV criteria of functional gastrointestinal (GI) disorders was used to confirm symptoms were biliary in origin. Results Of the 222 patients who had HIDA scan, 35 (15.7%) patients had BD. 26 (74.2%) were female and median age was 42 years (IQR 33-55). Median time from the onset of symptoms and HIDA scan was 18 months. 19 (54.2%) patients had LC preformed, 9 (25.7%) were discharged with decision of LC not required, 4 (11.4 %) had no further review and 2 (5.7%) still having LC under consideration. Histopathology of LC was, chronic inflammation in 10 (52.6%), cholesterosis in 3 (15.8%) and 4 (20.1%) had normal gallbladder. 13 (68.4%) patients who underwent LC were symptom free 12 months post-surgery. Conclusions BD although painful and potentially lifestyle limiting, does not progress to more serious consequences if untreated. Diagnosis is usually difficult, and patients go through extensive investigations before they have HIDA scan. LC improves the symptoms in two thirds of patients and should be offered to all patients with BD with appropriate counselling.

The efficiency and safety of low-dosage isotretinoin therapy for Chinese acne vulgaris patients.

Acne vulgaris is one of the most common skin conditions in dermatology clinics. Accumulating evidence has implicated oral low-dosage isotretinoin was an effective treatment for acne with fewer side effects. Currently, the data on low-dosage isotretinoin use in Chinese is limited. To investigate the efficiency and safety of low-dosage isotretinoin therapy for Chinese acne patients. Three hundred and eighty-eight patients treated with low-dosage isotretinoin (0.2-0.4 mg/kg/d) and who completed the course (120 mg/kg) were enrolled. Medical information on the severity, duration, adverse effects, and outcome of acne was reviewed. The majority (90.2%, n = 350) of patients achieved complete remission, and on average, patients received 13.5 months of treatment. The time between isotretinoin start and the clear date between the mild and moderate groups was not significantly different (74 ± 24 vs. 84 ± 24 days). However, it took longer to resolve for the severe acne group (112 ± 25 days). Follow-up 1 year after completion of the isotretinoin course, 37/350 (10.6%) patients relapsed, but there was no difference in the severity of acne. There were 133 (34.3%), 40 (10.3%), and 14 (2.6%) patients who developed hypercholesterolemia, hypertriglyceridemia, and high LDL, respectively. Thirty-two (8.2%) and 28 patients (7.2%) had elevated serum levels of alanine and aspartate aminotransferases. No values above grade 2 were detected. This study reaffirms the efficacy and safety of low-dosage oral isotretinoin in Chinese patients with acne vulgaris. Lab investigation could be performed after 2 months of therapy in healthy patients with normal baseline liver function and lipid panel tests.

Paediatric Mesenchymal Hamartoma of Liver-A Diagnostic Dilemma

Introduction and Importance: Benign tumors of the liver are relatively rare in children. Mesenchymal hamartoma (MH) is a benign tumor of the liver. It is the second most common benign pediatric hepatic tumor after infantile hemangioma. Mostly it is seen in children less than 2 years of age. The diagnosis of this tumor is difficult because of nonspecific clinical symptoms and lack of definitive laboratory studies. The precise diagnosis of mesenchymal hamartoma relies on histological evaluation of the tissue.We hereby present a rare case in a 2 year old child with mesenchymal hamartoma and its diagnostic challenges. Case presentation: A 2 year old previously healthy male was referred to our institution for evaluation of a liver mass. He complained of loss of apetite and abdominal distension since last 10 weeks. He had no complaints of fever/trauma. Clinical findings and Investigations: On per abdomen examination,there was a presence of large, non tender hepatomegaly with no shifting dullness present. On radiological evaluation, Ultrasound abdomen showed solitary right lobe mass. On further imaging, CT scan of the abdomen was done which showed a large multiloculated lesion in right lobe liver with arterial enhancing intervening septae within.The lesion was compressing and displacing right portal vein and right hepatic vein. At that time, laboratory evaluation showed normal liver function tests, serum AFP and other blood parameters. Differentials of liver cyst, hydatid cyst and biliary cystadenoma was made. Intervention and outcome: After discussion in multidisciplinary care, he was planned for surgery which would provide the exact histological diagnosis also in such cystic lesion of liver.He was optimized for surgery and subsequently underwent right hepatectomy with resection of the ~14× 12 cm mass. No major periperative complications developed and patient was discharged from hospital on POD6. The pathological review of the resected mass showed mixture of bile ducts, vessels, irregular liver cell plates and loose mesenchyme containing variable sized cysts consistent with a mesenchymal hamartoma. He is asymptomatic at the time of regular follow-up visits. Relevance and Impact: Benign tumors of the liver are relatively rare in children. The diagnosis of mesenchymal hamartoma still remains a challenge with non –specific complaints, normal laboratory findings and delay in diagnosis can lead to complications from local compression. Though advance imaging can diagnose this entity if index of suspicion is high but Differentiating it from other tumors such as an undifferentiated embryonal sarcoma is difficult on imaging alone. Hence surgical excision is the gold standard for diagnosing to reach the final histological diagnosis and treating these entities.

THU241 Novel PPARG Pathogenic Gene Variant In Newly Diagnosed Familial Partial Lipodystrophy With Childhood Onset

Abstract Disclosure: E.K. Chang: None. K. Greve: None. L. Escobar: None. Background: Familial partial lipodystrophy (FPL) is characterized by progressive, selective body fat loss and is often associated with various metabolic complications including insulin resistance. In particular, FPL Type 3 due to PPARG (peroxisome proliferator-activated receptor gamma) mutations is a very rare subtype with a prevalence of less than 1 per 1,000,000 people overall. This case report describes a clinical case of a child presenting with genetic testing revealing a novel PPARG pathogenic gene variant. Clinical Case: A 9 year old female with history of adoption and unknown family history presented with severe abdominal pain and intractable emesis in the setting of COVID19 infection. Her hospital CT scan of the abdomen was remarkable for pancreatitis and hepatosplenomegaly. Glucose was elevated at 356 mg/dL with normal liver function tests. HgA1c was 11%. Triglycerides were above 4425 mg/dL. Her overall clinical picture was consistent with new onset diabetes mellitus and acute pancreatitis. Physical exam was notable for xanthomatosis and acanthosis nigricans. She was later noted to have partial subcutaneous fat loss of the upper and lower extremities, with preserved fat in the face, neck, and truncal region. She was initiated on insulin therapy and discharged to home once symptomatically improved from her pancreatitis with downtrending triglycerides to less than 450 mg/dL. Her diabetes autoantibodies (Glutamic acid decarboxylase, Islet Antigen-2, Zinc transporter 8 antibodies) resulted as normal, and she was able to discontinue insulin with HgA1c trending down to 4.8% within 4 months of hospital discharge. She underwent whole exome sequencing per recommendation of her lipid specialist and geneticist, which identified a likely pathogenic, previously undescribed variant of PPARG: c.1050_1054dup, p.(Glu352Alafs*12) and a variant of uncertain significance for MC4R: c.409A>G, p.(Ile137Val). This particular variant of PPARG represents a frameshift mutation, resulting in a premature stop codon, likely leading to a loss of normal PPARG function. PPARG mutations are specific to FPL Type 3, which is reportedly associated with atypical fat distribution, diabetes mellitus, hypertriglyceridemia, hypertension, and pancreatitis, consistent with many features of this case. Conclusion: This is the first case to describe the unique PPARG variant c.1050_1054dup, p.(Glu352Alafs*12) which is associated with FPL Type 3. This child's clinical course and evaluation can help with identifying affected individuals of this very rare type of FPL. Presentation: Thursday, June 15, 2023

THU131 Functional Hypogonadotropic Hypogonadism And Failure To Thrive In A Poorly Controlled Type 1 Diabetes Patient

Abstract Disclosure: P. Rakhra: None. V. Wright: None. I. Athanassaki: None. Background: Multiple reports exist of obese males with type 2 diabetes linked to hypogonadotropic hypogonadism. We report a rare case of poorly controlled type 1 diabetes leading to functional hypogonadotropic hypogonadism as well as failure to thrive. Clinical Case: A 16-year-old Caucasian male with type 1 diabetes, antibody positive, diagnosed at 3 years of age presented in severe diabetic ketoacidosis. Patient had been lost to follow-up and had not had a clinic visit in over three years. He had reportedly been using a family member’s Tresiba and over the counter Humulin in the interim. Upon presentation, his hemoglobin A1c was found to be 11.8%. It was last documented at greater than 14%. He was also at less than the first percentile for height and weight, with a BMI of 16 kg/m2. His mid-parental height was 68.5 inches (25 – 50th percentile). His physical exam was remarkable for Tanner III pubic hair with 12 cc testicular volume bilaterally. Biochemical analysis revealed LH was in pubertal range of 0.5 mIU/mL (0.2 – 2.3 mIU/mL), low FSH <0.7 mIU/mL (0.7 – 6.2 mIU/mL), and low testosterone 7.7 ng/dL (200 – 1036 ng/dL). IGF-1 was low at 49 ng/mL (230 – 769 ng/mL) and low IGF-BP3 at 2.6 mg/L (3.1 – 8.9 mg/L). Bone age per the standards of Greulich and Pyle was delayed at 14 years with a predicted adult height of 68 inches. Associated conditions known to cause failure to thrive in patients with diabetes, such as adrenal insufficiency, hyperthyroidism, and celiac disease were ruled out. ACTH stimulation test revealed cortisol peak of 24.6 ug/dL (>18.0 ug/dL). TSH was normal 2.474 mIU/mL (0.5 – 3.4 mIU/mL) and normal Free T4 0.8 ng/dL (0.8 – 2 ng/dL). Mauriac syndrome was considered however, patient lacked any Cushingoid features, did not have hepatomegaly on exam, and had normal liver function tests. A brain/pituitary MRI was performed prior to discharge and was found to be normal, ruling out concerns for a central lesion causing delayed puberty. Patient was able to gain weight during the admission with intensive insulin management. However, he remains less than the first percentile for height and weight. A growth hormone stimulation test (with clonidine and arginine) was performed outpatient. Patient was primed with estrace prior to the test and results revealed a peak of 15.0 ng/mL (>10 ng/mL) ruling out concerns for growth hormone deficiency. Conclusion: We report this case to highlight that although uncommon in the developed world, delayed puberty and growth failure are known but rare complications of poorly controlled type 1 diabetes. Presentation: Thursday, June 15, 2023

SAT580 Incidental Diagnosis Of Genetic Variant PTPN11 Noonan Syndrome With Multiple Lentigines, Sensorineural Hearing Loss And Rheumatoid Arthritis In Adult Female With Hashimoto’s Thyroiditis. Case Report

Abstract Disclosure: M.M. Eid: None. T. Zahra: None. J. Vargas-Jerez: None. Introduction: Hashimoto’s thyroiditis (HT, chronic lymphocytic thyroiditis, autoimmune thyroiditis) and Rheumatoid arthritis (RA) are autoimmune disorders, affect mainly females (1). Noonan syndrome with Multiple Lentigines (NSML, Leopard Syndrome) is a rare multigenetic autosomal dominant disorder. It includes cerebrovascular and cardiac anomalies, facial phenotype, hearing abnormality and developmental delay (2). Case: 41 yo, Female with history of HT since 2002 (high TSH, thyroid peroxidase antibody more than 1000 u/ml, low FT4 and FT3) on levothyroxine 150mcg/day. Recently moved to the USA from EL Salvador. Presented for initial evaluation. Denied smoking, alcohol, allergy. Maternal aunt with thyroid disease. Had menstrual irregularities, no previous attempt to conceive. Review of system: 5 months of pain, swelling and morning stiffness for 1 hour in hand joints, hearing difficulty for 2 years, painless brown skin spots for years, childhood learning difficulty and constipation. Physical examination body weight 139Ib, height 162cm, Stable vitals, diffuse lentigines. Head: board forehead, hypertelorism, high arched eye brows, micrognathia, low set ears. Heart: normal heart sounds, no murmur. No thyromegaly, nystagmus or vertigo. Hand: swelling of metacarpophalangeal and proximal interphalangeal joints. Laboratory work up TSH 8 normal 0.27 to 4.2 uIU/ml, FT4 0.8 normal 0.9-1.8 ng/dl, A1C 5.7, positive rheumatoid factor 199IU/ml, anti-CCP antibody > 250U, ANA 1/320, ESR 61ml/hr, negative anti-tissue transglutaminase antibody, normal liver and kidney function tests and complete blood count. Hand X-ray: decrease joint space and bone erosion. Thyroid US: normal size, vascularity, no nodule. Normal Echocardiogram, EKG and chest X ray. Internal auditory canal MRI: no masses or abnormal enhancement. DEXA Scan: osteopenia of femur and lumbar spine. She was diagnosed with seropositive RA, B/L sensorineural hearing loss (SNHL) more on right side. Genetic test revealed heterozygous pathogenic variant of PTPN11 gene consistent with NSML and negative for Neurofibromatosis type 1. Kept on prednisone and methotrexate, levothyroxine was increased 162mcg/day. Conclusion: Autoimmune disorders have a common pathogenesis including humoral and cell mediated immunity, autoimmune oxidative stress, UV radiation exposure, genetic linkage and biochemical changes. Diagnosis of one autoimmune disorder increases the possibility of other autoimmune disorders (1). NSML has a variable presentation. PTPN11-NSML is usually associated with SNHL and cardiac anomalies as pulmonary stenosis. It is infrequent to diagnose NSML with HT and structurally normal heart like our case(2).

THU470 Blood - Bone Connection? A Case Of A Young Woman With Bilateral Hip Osteonecrosis And Prothrombin Mutation

Abstract Disclosure: A. Sliwinska: None. C. Vanek: None. Introduction: Osteonecrosis of the femoral head (ONFH) is a localized bone disease responsible for 10% of total hip replacements in the United States. Although the condition is a separate entity from osteoporosis, it may co-occur representing underlying systemic bone disease. Many risk factors (steroids, trauma, infections, thrombophilia, blood cell dyscrasias) can predispose to ONFH, therefore, diligent evaluation is required to reveal the underlying cause. There are no FDA approved therapies. Here, we present a case of young woman with bilateral osteonecrosis of femoral head who was found to have prothrombin gene mutation, severe osteoporosis, and was successfully treated with PTH analog therapy with abaloparatide. Case Description: 33-year-old Asian female with no past medical history presented to the Endocrinology clinic for a metabolic bone disease assessment. Two years prior, she developed progressive bilateral hip pain which prompted radiographic evaluation, discovering bilateral ONFH. She was managed non-surgically with a 6-month trial of oral alendronate. Worsening pain prompted left hip decompression surgery with stem cell transfer. Patient had no history of fragility fractures nor family history of bone disease. A bone density revealed Z scores of -3.9 and -3.5 in lumbar spine and total hip, respectively. She was taking 2000 IU vitamin D3 and 600 mg of elemental calcium daily. Her blood tests revealed normal basic metabolic panel, normal complete blood count and liver function tests. Calcium was 9.0 mg/dl (ref:8.6-10.2), albumin 4.6 mg/dl (3.5-4.7 mg/dl), phosphorus 4.0 mg/dl (2.4-4.7 mg/dl), PTH of 28 pg/ml (18-88 pg/ml), Vitamin D 25-hydroxy 41.3 ng/ml (30-80 ng/ml), and TSH was 1.66 mIU/L (0.39-4.17 mIU/L). Patient had an infectious, metabolic and rheumatological work-up that was negative. A thrombosis workup showed a heterozygous prothrombin gene (G20210A) mutation. Factor V Leiden mutation was negative. Repeat Hip MRI showed progression of her ONFH with marked bone marrow edema in femoral head bilaterally. Patient was then started on abaloparatide. After 6 months, hip MRI showed marked improvement with no bone marrow edema. Discussion: Osteonecrosis of the femoral head is a complex disease that can affect young individuals. Bilateral ONFH suggests systemic disease and a thorough work-up is necessary. Although the exact mechanism is unclear, it has been hypothesized that thrombin plays a role in bone homeostasis therefore hematologic disorders may predispose patients to both ONFH and osteoporosis. Medical treatment of ONFH, including bisphosphonates, statins, or anticoagulants, have been suggested. Recently the PTH analog, teriparatide, has shown promising results and found to be beneficial in treating osteonecrosis. Our case highlights importance of thorough work-up for patients with bilateral ONFH and successful use of anabolic agent in this population. Presentation: Thursday, June 15, 2023

FRI196 Non-Hemorrhagic Adrenal Infarction in Pregnancy with Tuberous Sclerosis

Abstract Disclosure: A. Mahaldar: None. H. Al Jumaili: None. S.R. Campbell: None. M.Z. Baker: None. Background: Non hemorrhagic adrenal infarction is a rare diagnosis in pregnancy with less than 20 cases reported in literature so far. No cases have been reported in patients with tuberous sclerosis. Most patients present with abdominal pain. Clinical Case: 29 year old woman G2P1 presented at 35 weeks gestation with right upper quadrant pain. She reported two episodes of dizziness in first trimester but otherwise had an uneventful pregnancy prior to presentation. First pregnancy was uncomplicated. Past medical history included asymptomatic tuberous sclerosis. No previous history of blood clots. Physical examination revealed a distended abdomen appropriate for gestational age and right upper quadrant tenderness to palpation. Initial laboratory data was unremarkable except for leukocytosis with WBC 16.6 k/mm3 (4-11 k/mm3). Normal electrolytes and liver function tests. Abdominal ultrasound revealed cholelithiasis without evidence of acute cholecystitis and unremarkable renal findings. CT abdomen pelvis with contrast showed thickening with surrounding stranding of right adrenal gland suggesting acute adrenal infarction and multiple pancreatic cysts. 8 am cortisol was 4.9 mcg/dl (4.1-16.6 mcg/dl) and ACTH was 23 pg/ml (7.2-63.3 pg/ml). Patient had received 2 doses of betamethasone due to concern for preterm delivery at 48 hours and 36 hours prior to this blood draw. Hypercoagulable panel was sent prior to starting enoxaparin. Elevated Factor VIII >150% (50 to 150%) was noted as expected in pregnancy. Lupus anticoagulant was weakly positive with LA sensitive PTT 50.1 (0 to 51.9 sec). Anticardiolipin antibody and beta 2 glycoprotein antibody were normal. Patient was treated with hydrocortisone 15 mg by mouth daily and full dose enoxaparin for anticoagulation. At one month follow up 8 am cortisol was 24 mcg/dl. Hydrocortisone replacement was discontinued. Labor was uncomplicated with vaginal delivery at 38 weeks gestation. Day 1 postpartum at 7:30 AM cortisol was 20.94 mcg/dl. Patient was discharged home in a stable condition with continued anticoagulation with enoxaparin and no further hydrocortisone treatment. Conclusion: We present a case of non hemorrhagic adrenal infarction in a pregnant patient with tuberous sclerosis. Adrenal infarction without hemorrhage is rare. Anatomy of adrenal gland makes it prone to microvascular thrombosis and infarction in a thrombotic state because of single draining vein for venous drainage. Pregnancy as a hypercoagulable state has been previously associated with non hemorrhagic adrenal infarction in few case reports. Whether tuberous sclerosis played any role in our patient’s pathogenesis remains unclear as there are no data or case reports linking the two. In conclusion, the diagnosis of non hemorrhagic adrenal infarction needs to be considered in hypercoagulable or pregnant patients presenting with abdominal pain. Presentation: Friday, June 16, 2023

Mortality and Cardiovascular Outcomes in Adult Non-Liver Solid Organ Transplant Patients With Nonalcoholic Steatohepatitis

The effect of nonalcoholic steatohepatitis (NASH) on mortality or major adverse cardiovascular events (MACE) in non-liver solid organ transplant recipients (NL-SOT) is unknown. Using a retrospective design, adult NL-SOT recipients who had biopsy-proven NASH were compared NL-SOT recipients with normal liver function tests and imaging; propensity matched at a 1:10 ratio on the following: age, sex, race, transplant year, transplant organ, smoking status, and diabetes status. Both deceased and living donor recipients were included; heart and liver transplant patients were excluded. Primary outcome was incidence of all-cause mortality and MACE (a composite outcome of coronary artery disease, ischemic stroke, and peripheral arterial disease). Seven patients (3 kidney and 4 lung transplants) had biopsy-proven NASH and 70 patients without NASH, both groups were predominantly male (53%-57%), White (86%-91%), and overweight (mean body mass index ∼ 26). The majority of patients were on calcineurin inhibitors (≥85%), antimetabolites (≥97%), and prednisone (≥50%). Survival analysis showed that NASH patients had a higher risk of death (hazard ratio [HR], 3.24; 95% confidence interval [CI], 1.26-8.33, P=0.02). NASH did not affect the risk of death-censored graft failure (HR, 1.08; 95% CI, 0.14-8.67; P=.94) or the risk of MACE (HR, 1.03; 95% CI, 0.23-4.62; P=.97). In NL-SOT recipients, NASH is significantly associated with mortality but not with MACE.

Prevalence and Molecular Characterization of Hepatitis C in Murree

Background: Hepatitis C infection can result in liver damage ranging from inflammation to cirrhosis and hepatocellular carcinoma. The aim of this study was to determine the prevalence and molecular characterization of hepatitis C virus (HCV) infection in the city of Murree. Methods: A descriptive study was conducted at THQ Hospital Murree, Pakistan from June 2021 to November 2021. The prevalence of hepatitis C was estimated using two major laboratory diagnostic methods: immunochromatographic testing (ICT) and polymerase chain reaction (PCR). A total of 2,163 patients were tested for HCV antibodies by ICT, of whom 446 tested positive for ICT (prevalence 20%). The positive ICT samples were further analyzed using the molecular gold standard method PCR. Of the 446 samples, 381 (85.6%) tested positive by PCR, 38 (8.4%) negative and 27 (6%) had to be re-sampled due to sample inaccuracy. Results: A total of 381 people were included in the study. Of the 2,163 patients screened with ICT, 446 tested positive. All 446 samples underwent PCR, and 381 (85.6%) cases were positive, 38 (8.4%) were negative and 27 (6%) were requested to be re-sampled. The most affected age group was 30- to 39-year-olds (27.4%). Of the subjects with positive HCV results by PCR, 66% had abnormal liver function test results (elevated ALT, ALP, and bilirubin), while 34% had normal liver function test results. The viral load of HCV-positive cases ranged from 1,000,000 to 7,000,000 IU/L in 35.7% of cases and from 100,000 to 999,999 IU/L in 43.8% of cases. Conclusion: The study revealed a high prevalence of hepatitis C (17%) in the population served by Tehsil Headquarters Hospital in Murree, which poses a significant challenge. It is crucial to promote the use of gold standard methods such as PCR in laboratory diagnostics to ensure accurate detection and characterization of HCV infection period.

A common presentation of uncommon disease - Biliary Fasciola Hepatica.

Hepatobiliary fascioliasis is one of the rare but important parasitic infection in endemic areas such as Pakistan. Clinical manifestations overlap with common clinical conditions such as hepatitis and biliary stones causing common bile duct obstruction. Fascioliasis is a zoonotic infection caused by a leaf-shaped organism known as Fasciola Hepatica. Humans are accidentally infected by consuming contaminated water or food, especially undercooked cattle and sheep liver or raw green vegetables. We report three cases with similar clinical manifestations presenting in two different cities in Pakistan. After detailed clinical evaluation and laboratory investigations, the diagnosis was established by abdominal ultrasound and diagnostic and therapeutic cholangiography. Patients were followed till complete resolution of their symptoms and normalisation of liver function tests.

E007 Is there an urgent need to change the methotrexate monitoring guidelines to incorporate fibroscan?

Abstract Background/Aims Methotrexate (MTX) is a widely used DMARD for which routine blood monitoring is required to identify hepatotoxicity and bone marrow suppression. Despite routine monitoring and normal liver function tests some patients develop hepatic fibrosis. Transient elastography (fibroscan) offers an excellent non-invasive approach to measure the stiffness of the liver as a form of assessing hepatic fibrosis and would prove extremely useful to monitor the effects of MTX in our patient groups. Methods A retrospective study was conducted between January 2021 and August 2022. Inflammatory arthritis patients who have been on MTX for more than five years were selected for the study. Their fibroscan results were obtained and their median value (kilopascals), if they had an ultrasound in the past showing cirrhosis, metabolic risk factors such as obesity (BMI>30), presence of type 2 diabetes and alcohol consumption (>14 units/week) were recorded from their clinic letters. Results Of the 56 patients, there were 18 males and 38 females. 29% of the patients had a median fibroscan value of more than 7 kpa (n = 16). Of these 16 patients, 50% of the patients had a metavir score of F2 (n = 8), 25% of the patients had a metavir score of F3 (n = 4) and 25% of the patients had a metavir score of F4 (n = 4). Of the 16 patients (11 RA and 5 PSA) with high median value, 46% had a BMI > 30 kg/m2 (n = 7), 13% had a diagnosis of type 2 diabetes mellitus (n = 2) and none of the patients had consumed more than 14 units/week of alcohol. Of the 16 patients with high median value, 12% (n = 2) had a diagnosis of cirrhosis on ultrasound. One patient had extremely high median value of 26 had underlying ILD, right heart failure and possible cardiac cirrhosis. 1 patient had developed frank cirrhosis and the high fibroscan results were noted subsequently. For all the patients with high metavir score of F4 and above, MTX therapy was discontinued. Conclusion A significant percentage of the cohort (29%) had a high median value above 7 of which 25% of the patients had F4 fibrosis despite having normal liver function tests. The study illustrates the need for a different modality in addition to the routine blood monitoring for the patients on MTX with metabolic risk factors. Therefore, it is vital to obtain a baseline fibroscan in high-risk patient group with preexisting metabolic risk factors prior to initiating MTX therapy and monitor every 5 years thereafter. This will help the clinician to safely continue MTX in this group of patients without the fear of worsening liver fibrosis. Disclosure S. Ramalingam: None. M. Sayed: None. S. Alam: None. D. Makkuni: None.

Liver manifestations of tick-borne diseases.

Liver manifestations of tick-borne diseases.

Intraoperative Fluorescent Cholangiography Using Indocyanine Green Fluorescence for Identification and Treatment of Bile Leaks After Urgent Laparoscopic Cholecystectomy

Introduction: Laparoscopic cholecystectomy (LC) is one of the most common surgical procedures performed worldwide.1 Postoperative bile leaks caused by bile duct injuries (BDIs) are serious complications after LC, resulting in a prolonged length of hospital stay, increased costs, morbidity, and mortality rates.2 Incidence of BDIs during LC is ~0.4%–1.5%.1 Intraoperative fluorescent cholangiography using indocyanine green fluorescence (ICG-C) is a safe method to observe the biliary tree and may be useful to prevent postoperative bile leaks after LC and hepatic surgery.1,2 However, detection of bile leaks using the ICG-C during LC has limited evidence in international literature.3–8 This video illustrates how to use ICG-C to identify and treat a bile leak after an urgent LC. Materials and Methods: A 54-year-old man with a history of hypertension, diabetes mellitus, cholelithiasis, and hepatic abscesses was admitted with right upper quadrant pain and fever. An urgent CT scan of the abdomen showed acute cholecystitis associated with a large pericholecystic abscess. A percutaneous cholecystostomy (PC) was placed and nonoperative management with intravenous antibiotic was started. On the seventh postprocedural day, the PC was accidentally removed, and a second CT scan of the abdomen showed a reduction of the pericholecystic abscess size, and a subhepatic fluid collection. An urgent LC was performed, and two surgical drains were placed (one subhepatic and one suprahepatic). On the first postoperative day, a low-flow biliary output (250 mL/day) from the subhepatic drainage was associated with a gradual deterioration of the patient's clinical condition. Considering the low flow rate of the biliary fistula, nonoperative management was carried out; on the second post-operative day the patient presented hypotension, oliguria, fever (temperature 38.2°C), and worsening right upper abdominal pain with signs of diffuse peritonitis. The hematologic investigations showed a WBC 17,560/mL, Hb 10.7 g/dL, C-Reactive Protein 17.66 mg/dL, normal liver function tests, and normal bilirubin levels. Owing to signs of septic shock and diffuse signs of peritonitis, an urgent surgical exploration was performed. After the induction of anesthesia, 2.5 mg of Indocyanine Green was intravenously injected. Eight minutes later, the ICG-C demonstrated exactly and in real-time the biliary leak on the liver surface, coming from a subvesical bile duct. Two laparoscopic Roeder's knots were placed to repair the injury. Two surgical drains were placed. Results: The postoperative period was uneventful, with a progressive improvement of the patient's performance status, vital parameters, and laboratory tests. The surgical drains were serosanguinous with low output and both were removed on postoperative day 9. The patient was discharged on day 10 in good clinical condition. Conclusions: One of the most common causes of LC-associated biliary leaks are injuries to the subvesical bile ducts. ICG-C clearly identifies these bile spots on the liver surface during surgery, allowing them to be repaired immediately and prevent biliary fistulas. ICG-C is a safe, easy, repeatable, and noninvasive method to identify bile leaks during surgery. ICG-C may be useful to prevent and treat bile leaks after LC, especially in complicated cholecystitis.5,6 Authors have received and archived patient consent for video recording/publication in advance of video recording of procedure. No competing financial interests exist. Running time of video: 8 mins 27 secs

Study of serum beta HCG and liver function test in hypertensive disorder of pregnancy

Introduction: One of the most prevalent medical conditions during pregnancy is hypertensive disorders of pregnancy (HDP), which is also one of the leading causes of maternal and perinatal morbidity and mortality globally. This study was carried out to correlate the elevated liver function test (LFT) and serum beta HCG observed in the early second trimester with the increased maternal and foetal morbidity and mortality associated with HDP. Material and Method: The Rajkiya Manila chikitchalay JLN Medical college Ajmer's department of obstetrics and gynaecology conducted this hospital-based observational study. 375 pregnant women with a gestational period longer than 20 weeks were used in the correlation study between LFT and beta HCG and HDP, with 250 pregnant women with HDP serving as the study group and 125 pregnant women without hypertension serving as controls. Serum-HCG levels and LFT were tested and their levels in the two groups were compared. Results: In the 25-year-old age group and younger, severe pre-eclampsia was observed. In hypertension cases, the likelihood of developing severe preeclampsia was 78%. The incidence was 88 percent in patients with elevated LFTs. The maternal mean serum levels of Beta-HCG in the HDP study group (51161.08±30038.21 IU/L) were greater than those in the control group with normal blood pressure (17603.23±16748.21 IU/L). The mean blood levels of non-severe preeclampsia were 36417.32±23876.74 IU/L and severe preeclampsia were 60030.34±28771.31 IU/L. Higher amounts were seen in early-onset preeclamptic moms compared to late-onset preeclamptic mothers, which was statistically significant (p<0.001). 32077 IU/L was the cut-off mark for beta-HCG with a sensitivity of 65% and a specificity of 86% for predicting HDP.Conclusions: HDP women have greater serum levels of beta-HCG than normotensive women. Increased severity of hypertensive disorders during pregnancy is linked to higher levels of beta-HCG. Due to its low sensitivity and difficulties in determining the cut-off value, serum beta-HCG has limited application as a diagnostic test. In contrast to severe pre-eclampsia with normal LFTs, detection of elevated LFTs in cases of severe pre-eclampsia is a risk group linked to a higher rate of feto-maternal problems.

How to Interpret Liver Function Test in Daily Practice

Background: The liver is a multi-function organ that plays a role in producing various important secrete, synthesis and metabolic functions. The term liver function test is also used to refer to the hepatocyte synthesis function, such as serum albumin and prothrombin time. In this paper, we will discuss how to interpret various liver function test results in daily practice as well as approach to abnormal liver function tests.&#x0D; Discussion: Abnormal result of liver function test is frequently found in asymptomatic healthy patients who are undergoing routine screening. On the contrary, some patients with liver disease may appear with normal liver function test. As such, there are some limitations regarding this particular test. ALT (SGPT) is the primary marker of hepatocellular injury due to it being more sensitive and specific than AST (SGOT). Conjugated bilirubin level of more than 20% of total bilirubin level is a strong indication of hepato-biliary disease and is always pathogenic. GGT is only increased in cholestatic conditions and not in bone disease. As such, GGT levels can help to distinguish abnormalities in the liver or bones in conditions of increased ALP levels in the blood.&#x0D; Conclusion: Serum liver biochemistry examination is very useful and effective in assessing liver function. Understanding the proper interpretation of liver enzymes will help clinicians easier to diagnose or predict a disease as well as condition related to the liver in daily practice.

Gastrointestinal and Hepatic Manifestations in Patients with Covid-19

BACKGROUND Gastrointestinal symptoms and Liver injury have been frequently described in patients with COVID-19.In this study, we aim to study the prevalence of Gastrointestinal symptoms and Liver injury and their impact on disease severity and clinical outcomes.&#x0D; METHODS This is a descriptive, cross sectional observational study done in 195 patients admitted to Bir Hospital with Corona virus Disease-19(COVID-19) from May 2021 to July 2021.Patients presenting symptoms and liver function tests (LFTs) were noted at admission. Gastrointestinal symptoms were defined as the presence of one of Nausea, Vomiting, Abdominal pain or Diarrhea. Liver injury was defined as elevation of Total Bilirubin &gt; 1mg/dl or Alanine transaminase (ALT) &gt;40U/L, Aspartate Transaminase(AST) &gt;40U/L or Alkaline Phosphatase(ALP)&gt;135 U/L. Patients were followed during hospitalization and outcomes assessed at discharge.&#x0D; RESULTS Gastrointestinal symptoms were present in 32.3% among which Nausea (27.7%) was the commonest. There was no significant association of Gastrointestinal symptoms with duration of hospital stay, ICU admission or mortality. Liver injury was present in 72.8% patients and significantly more in those with GI symptoms than without GI symptoms. Patients with liver injury had a significantly longer hospital stay (8.92 ± 3.7 days vs 6.70±3.2 days, p=0.004), higher ICU admissions (38.02% vs 7.54% p=0.000018) and mortality (23.23% vs 1.88%, p=0.001) compared to patients with normal LFTs.&#x0D; CONCLUSION Gastrointestinal symptoms and Liver Injury are common in patients with COVID-19. Liver injury at admission is associated with severe disease and higher mortality. More attention should be given in the care of such patients to avoid poor outcomes.

A rare cause of central diabetes insipidus with arthritis (Erdheim-Chester disease)

Abstract Mr. Haider is a 26-year-old resident doctor who presented with intermittent fever relieved by NSAIDS fatigue, weakness, loss of libido, fever with no rigor, night sweat, bone, joint pain and knee swelling. He lost weight. He was diagnosed with central chronic D.I for the last 3 years after several weeks history of polyurea and thirst without identifiable cause which follows elbow fracture with trivial trauma and was taking nasal desmopressin as required. His last symptoms were followed a Covid 19 attack. Physical examination reveals high fever 39 °C, pallor, thin built, no thyroid or lymph node enlargement. Chest examination is normal with no organomegaly on abdominal examination. Knee examination shows swollen red tender joint. Initial investigation shows ESR 110 mm/hr. CRP 10 mg/dl Negative screening for tuberculosis, hepatitis, HIV, vasculitis and Brucellosis. Normal kidney and liver function tests and electrolytes.Mild hyopochromic anemia. Normal free T4 and TSH, prolactin and cortisol. Normal SHBG Low morning testosterone twice 60 ng/dl. and 64 ng/dl. FSH and LH normal Normal Dexa scan. Thus till now there is feature of hypogonadism, D.I and arthritis. Due to low testosterone MRI pituitary requested and reveals small size pituitary gland, absent post bight spot with mild thickening of the pituitary stalk with no mass lesion. Suspicion of inflammatory or infiltrative lesion of pituitary was raised. For proximal tibial lesion bone biopsy was done and revealed marked fibrosis with infiltration of bone marrow by foamy cells with multinucleated cells positive for CD 68 and negative for CD 1a by immunohistochemistry. Further evaluation by PET scan confirms the diagnosis and shows multifocal hypermetabolic symmetrical bone lesions most intense around elbows and knees consistent mostly with Erdheim Chester disease (ECD). Patient was treated with testosterone injections with improved libido and consultation with oncologist was done to arrange the need for chemotherapy. Molecular and genetic test is not available in Iraq and a sample was sent abroad. To our knowledge this is the first reported case of ECD in Iraq. Figure 1. PET image.

Valproate enduced encephalopathy in a psychiatric patient with normal liver function tests

Sodium valproate is a broad spectrum antiepileptic drug (AED) and is being widely used in various neuro-psychiatric conditions. It can cause hyper-ammonemic encephalopathy (HE) especially when used with other anti-epileptics like topiramate, phenobarbitone, phenytoin etc. Here we describe such a patient who was taking valproate for bipolar disorder with no other AEDs and earlier misdiagnosed as viral encephalitis, which on further evaluation was found to be suffering from HE. The patient was managed accordingly and recovered completely.

Оценка эффективности Ремаксола у пациентов с COVID-19 на противовирусной терапии

Management of COVID-19 patients with drug-induced liver lesions requires a comprehensive approach to treatment with correction of metabolic disorders and prevention of adverse events. We describe our experience with using Remaxol, a drug with hepatoprotective, antihypoxic, antioxidant, and cytoprotective effects. It is applied for hepatotropic support in COVID-19 patients receiving antiviral therapy. Intravenous infusions with Remaxol (400 mL) in comprehensive treatment of COVID-19 patients promoted normalization of liver function tests, which made it possible to continue antiviral therapy without dose correction and interruptions. Remaxol was well tolerated and caused no side effects; none of the patients required its cessation. Key words: hepatotoxicity, hepatoprotectors, Remaxol, COVID-19