Hepatobiliary Disease In Patients Research Articles

Hepatobiliary disease in children and adolescents with cystic fibrosis

ObjectivesThe aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. MethodsThis was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed‐up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. ResultsHepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non‐white, with a predominance of females (77.8%) and median (IQR) of 54 (27–91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p=0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p=0.007). ConclusionThe frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

Hepatobiliary disease in children and adolescents with cystic fibrosis

ObjectivesThe aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. MethodsThis was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. ResultsHepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27–91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs. 39.6%, p=0.033) and severe pancreatic insufficiency (88.9% vs. 31.6%, p=0.007). ConclusionThe frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

Endoscopic ultrasound-guided creation of a transgastric fistula for the management of hepatobiliary disease in patients with Roux-en-Y gastric bypass.

Background and aims Endoscopic retrograde cholangiopancreatography (ERCP) in patients who have undergone Roux-en-Y gastric bypass (RYGB) is technically challenging. We describe our multicenter experience using lumen-apposing metal stents (LAMSs) to create an endoscopic ultrasound-guided transgastric fistula (EUS-TG) to facilitate peroral ERCP in these patients. Patients and methods Thirteen patients with RYGB who underwent EUS-TG at three tertiary centers were included. EUS was used to guide puncture of the excluded stomach from the gastric pouch or jejunum; a LAMS was placed across the transgastric fistula. ERCP was performed via a duodenoscope passed through the LAMS. Results The technical success of EUS-TG was 100 % (13/13). ERCP through the LAMS was successful and clinical success was achieved in all patients. LAMS dislodgement during ERCP occurred in two patients and the stent was successfully repositioned without sequelae. After removal of the LAMS, the fistula was closed in 92 % of patients, either by endoscopic closure devices or argon plasma coagulation. None of the patients experienced procedure-related adverse events. Conclusion EUS-TG is an effective and safe method of accessing the excluded stomach and performing ERCP in patients with RYGB.

Lipids and liver dysfunction in patients receiving parenteral nutrition

Abnormalities of liver function tests are common in patients with intestinal failure receiving parenteral nutrition. Lipid emulsions have been implicated in the development of hepatobiliary disease in patients receiving parenteral nutrition. Lipid emulsions with reduced polyunsaturated fatty acids and specific ω6 : ω3 fatty acid ratios have been shown to have some beneficial effects on liver function, although the studies are small and generally of short duration in paediatric and adult patients. There is good evidence to suggest that the parenteral lipid dose should be less than 1 g/kg body weight/day, but this may not apply to all patients. The evidence is presented for the different lipid emulsions and their effect on liver function. The benefit of these emulsions compared with simply giving a lower lipid dose has yet to be studied.

Hepatobiliary disease in patients with cystic fibrosis

This review explores the recent advances in knowledge regarding hepatobiliary disease in patients with cystic fibrosis. Hepatobiliary abnormalities associated with cystic fibrosis are varied in nature and range from defects attributable to the underlying genetic defect to those related to systemic disease and malnutrition. Novel research into the underlying pathogenesis of cystic fibrosis liver disease and the primary role of cystic fibrosis transmembrane conductance regulator in biliary secretory epithelium is presented. This work has been fostered by the development of new animal models of cystic fibrosis transmembrane conductance regulator dysfunction. Of the wide range of hepatobiliary complications associated with cystic fibrosis the most clinically relevant problem is progression of focal biliary cirrhosis to multilobular cirrhosis with its attendant complications of portal hypertension and potentially end-stage liver disease. However, recent studies suggest that liver transplantation may not improve survival in patients with cystic fibrosis and significant portal hypertension. Hepatobiliary disease is a common finding in patients with cystic fibrosis; the pathogenesis is multifactorial in nature. As new therapeutic strategies emerge, life expectancy will continue to increase as will the impact of liver disease on quality of life and survival of patients with cystic fibrosis. This review will discuss novel insights into pathogenesis as well as diagnostic and management options.

Three-dimensional drip infusion CT cholangiography in patients with suspected obstructive biliary disease: a retrospective analysis of feasibility and adverse reaction to contrast material.

BackgroundComputed Tomography Cholangiography (CTC) is a fast and widely available alternative technique to visualise hepatobiliary disease in patients with an inconclusive ultrasound when MRI cannot be performed. The method has previously been relatively unknown and sparsely used, due to concerns about adverse reactions and about image quality in patients with impaired hepatic function and thus reduced contrast excretion. In this retrospective study, the feasibility and the frequency of adverse reactions of CTC when using a drip infusion scheme based on bilirubin levels were evaluated.MethodsThe medical records of patients who had undergone upper abdominal spiral CT with subsequent three-dimensional rendering of the biliary tract by means of CTC during seven years were retrospectively reviewed regarding serum bilirubin concentration, adverse reaction and presence of visible contrast media in the bile ducts at CT examination. In total, 153 consecutive examinations in 142 patients were reviewed.ResultsContrast media was observed in the bile ducts at 144 examinations. In 110 examinations, the infusion time had been recorded in the medical records. Among these, 42 examinations had an elevated bilirubin value (>19 umol/L). There were nine patients without contrast excretion; 3 of which had a normal bilirubin value and 6 had an elevated value (25–133 umol/L). Two of the 153 examinations were inconclusive. One subject (0.7%) experienced a minor adverse reaction – a pricking sensation in the face. No other adverse effects were noted.ConclusionWe conclude that drip infusion CTC with an infusion rate of the biliary contrast agent iotroxate governed by the serum bilirubin value is a feasible and safe alternative to MRC in patients with and without impaired biliary excretion.In this retrospective study the feasibility and the frequency of adverse reactions when using a drip infusion scheme based on bilirubin levels has been evaluated.

Primary biliary cirrhosis and ulcerative colitis: A case report and review of literature

To summarize the characteristics of patients suffered from primary biliary cirrhosis associated with ulcerative colitis. To report a new case and review the literature. There were 18 cases (including our case) of primary biliary cirrhosis complicated with ulcerative colitis reported in the literature. Compared with classical primary biliary cirrhosis, the patients were more often males and younger similar. The bowel lesions were usually mild with proctitis predominated. While ulcerative colitis was diagnosed before primary biliary cirrhosis in 13 cases, the presentation of primary biliary cirrhosis was earlier than that of ulcerative colitis in our new case reported here. The prevalence of primary biliary cirrhosis among patients of ulcerative colitis was almost 30 times higher than in general population. Association of primary biliary cirrhosis with ulcerative colitis is rare. It should be considered in the differential diagnosis of hepatobiliary disease in patients with ulcerative colitis, and vice versa.

Surgical therapy for 101 patients with acquired immunodeficiency syndrome and symptomatic cholecystitis

Hepatobiliary disease in patients with acquired immunodeficiency syndrome (AIDS) has been well documented. Cytomegalovirus and Cryptosporidium are the pathogens most frequently associated. Previous reports of cholecystectomies and AIDS have had conflicting results on morbidity and mortality. Retrospective review of 101 patients with AIDS and symptomatic cholecystitis who underwent cholecystectomy from December 1989 to May 1995. All patients had symptoms characteristic of gallbladder disease, the most common being abdominal pain and fever. Thickening of the gallbladder was the most common diagnostic finding. Fifty-six patients underwent open cholecystectomy and 45 laparoscopic cholecystectomy. Pathologic examination revealed an abnormal gallbladder in all cases and gallstones in 29%. A specific pathogen or malignancy was identified as the etiologic agent in 44% of patients. Perioperative morbidity was similar (<5%) in both surgical groups. Perioperative mortality was 4% among all the patients treated. Both open and laparoscopic cholecystectomy improved the quality of life of these patients and should be considered as the treatment for persistent hepatobiliary symptoms in patients with AIDS.

AGA technical review: Malnutrition and cachexia, chronic diarrhea, and hepatobiliary disease in patients with human immunodeficiency virus infection

Disorders of the gastrointestinal tract and hepatobiliary system are among the most common complications associated with human immunodeficiency virus (HIV) infection. These disorders not only result in major morbidity but mortality as well. With increasing use of prophylaxis against Pneumocystis carinii pneumonia, the incidence of opportunistic gastrointestinal disorders has increased. 1–3 Significant progress has been made in the last decade in characterizing the spectrum of pathogens involving the gastrointestinal and hepatobiliary systems, determining the pathophysiological mechanisms of these diverse processes, and defining management options. Despite these encouraging advancements, many questions remain unanswered. The purpose of this review is to evaluate and synthesize the published clinical research pertaining to three important HIV-related complications: malnutrition and cachexia, chronic diarrhea, and hepatobiliary disease. A separate review addresses disorders of the esophagus. 4 Attention here is focused on etiology and pathogenesis, clinical features, diagnostic strategies, and efficacy of current treatment options. Recommendations for managing patients with these complications are provided based on the weight of the clinical evidence. GASTROENTEROLOGY 1996;111:1724-1752

American Gastroenterological Association medical position statement: Guidelines for the management of malnutrition and cachexia, chronic diarrhea, and hepatobiliary disease in patients with human immunodeficiency virus infection

Human immunodeficiency virus (HIV) infection is a devastating illness with major public health implications throughout the world. Gastrointestinal and hepatobiliary complications are common in patients with acquired immunodeficiency syndrome (AIDS) and affect most patients at some point during the course of the disease. Gastrointestinal complications are important not only because of their frequency but because they result in significant morbidity, mortality, and use of health care resources. Management of these patients provides a challenge to the practicing physician for several reasons, including the atypical presentations, the broad spectrum of potential causes, and the multisystem nature of these complications. In addition, because these complications often occur in patients with advanced disease, ethical considerations must be taken into account. GASTROENTEROLOGY 1996;111:1722-1723

The incidence of hepatobiliary disease in patients with ulcerative colitis in Stockholm county 1955–1979

The incidence of hepatobiliary disease in patients with ulcerative colitis in Stockholm county 1955–1979

Plasma bile salt levels in patients presenting with generalised pruritus: an improved indicator of occult liver disease.

Fasting plasma bile salt concentrations were measured in 26 patients presenting to a Skin Department who had generalised pruritus without primary skin disease, as part of a screening investigation for systemic causes of pruritus. The results were compared with conventional tests of hepatic function. Plasma-conjugated cholate measurements identified all patients with hepatobiliary disease. Conventional liver function tests were abnormal in these patients but also in five patients with no other evidence of hepatic dysfunction. Fasting conjugated-cholate measurements offer a useful screening test for identifying hepatobiliary disease in patients presenting with generalised pruritus.

Is ERCP a reasonable diagnostic method for excluding pancreatic and hepatobiliary disease in patients with a Billroth II resection?

ERCP was performed in 57 patients who had undergone Billroth II resection. Cannulation of the papilla of Vater was successfully accomplished in 31 cases (55%). The success rate of ERCP was clearly dependent on the type of Billroth II resection presenting.

Gamma-glutamyl transpeptidase and 5'-nucleotidase. Comparison as diagnostics for hepatic disease.

Enzymic activities of both γ-glutamyl transpeptidase and 5'-nucleotidase have been evaluated in patients in whose cases hepatobiliarγ-tree disease was suspected. γ-Glutamyl transpeptidase was a better indicator of diseases of the liver, bile duct, or pancreas than 5'-nucleotidase in nine of the ten conditions studied. 5'-Nucleotidase was found to be almost as useful as γ-glutamyl transpeptidase in indicating hepatobiliary disease in patients with granuloma of the liver, acute pancreatitis, congestive heart failure, rheumatoid arthritis, and carcinoma metastatic to the liver. γ-Glutamyl transpeptidase was most elevated in patients with primary carcinoma of the pancreas. Patients with carcinoma metastatic to bone or with neurologic conditions were found to have both enzymes elevated, but elevations occurred in a much lower proportion of patients than in those with hepatobiliary-tree disease. Liver scan findings correlate better with γ-glutamyl transpeptidase than with 5'-nucleotidase or alkaline phosphatase enzymic activity.