Presentation
A rationalisation of the 1350 boxes used throughout the book gives a simpler and clearer presentation of the various categories. [books.google.com]
[…] with a broad spectrum of presentations from mild alteration of liver function tests to deep jaundice with complete biliary obstruction, recurrent cholangitis, secondary biliary cirrhosis and liver failure. [freethesaurus.com]
This case report documents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. [ncbi.nlm.nih.gov]
In such a case presenting as persisting and fluctuating obstructive jaundice in HCC, an intrabile duct tumor growth should be suspected. [jpatholtm.org]
Entire Body System
- Weight Loss
The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy. [ncbi.nlm.nih.gov]
loss, weakness, fatigue, and even osteoporosis as a result of vitamin D malabsorption and subsequent calcium deficiency. [aafp.org]
As the disease progresses, symptoms may include weakness, fatigue, loss of appetite, nausea, vomiting, weight loss, abdominal pain and bloating, itching, and spiderlike blood vessels on the skin. [surgery.ucsf.edu]
loss Causes It's not clear what causes primary biliary cholangitis. [mayoclinic.org]
Hematological
- Easy Bruising
The most common initial symptoms are: Itchy skin Fatigue Jaundice symptoms like yellowing of the eyes and skin Other manifestations may include: Darkening of exposed areas of the skin Fever Bone pain Pale-colored, foul-smelling, bulky stools Easy bruising [digestion.ygoy.com]
Cirrhosis can lead to Easy bruising or bleeding, or nosebleeds Swelling of the abdomen or legs Extra sensitivity to medicines High blood pressure in the vein entering the liver Enlarged veins called varices in the esophagus and stomach. [icdlist.com]
Some of the signs and symptoms of liver disease include: jaundice (yellowing of the skin and eyes) pruritus (itching) dark, tea-colored urine weight loss muscle wasting ascites (swelling of the abdomen with fluid) fatigue easy bruising and bleeding (bleeding [surgery.uic.edu]
Easy bruising and bleeding. Periods may become heavier. Bleeding into the bowel and stomach may occur. Stomach ulcers are more common. There may be swelling of the stomach (ascites) due to fluid leak from the liver. [patient.info]
Gastrointestinal
- Abdominal Pain
The indication for the procedure was recent acute pancreatitis and episodic abdominal pain. [acgcasereports.gi.org]
It has a lengthy latent period, usually followed by the sudden appearance of abdominal pain and swelling, hematemesis, dependent edema, or jaundice. [medical-dictionary.thefreedictionary.com]
Some patients experience no symptoms, while others may experience symptoms such as: Fatigue Itchy skin Abdominal pain Dry eyes Dry mouth Yellowing of the skin and eyes (jaundice) Darkening of the skin Swollen feet and ankles Accumulation of abdominal [columbiasurgery.org]
As the disease progresses, symptoms may include weakness, fatigue, loss of appetite, nausea, vomiting, weight loss, abdominal pain and bloating, itching, and spiderlike blood vessels on the skin. [surgery.ucsf.edu]
- Loss of Appetite
As the disease progresses, symptoms may include weakness, fatigue, loss of appetite, nausea, vomiting, weight loss, abdominal pain and bloating, itching, and spiderlike blood vessels on the skin. [surgery.ucsf.edu]
Case presentation A 58-year-old female patient, who complained of icterus appearing in the last 6-7 months, along with the symptoms of fatigue and loss of appetite continued for 2-3 years, was referred to our clinic. [link.springer.com]
- Abdominal Mass
Characterize abdominal masses and adenopathy with the aid of diffusion-weighted MR imaging. [books.google.com]
Liver, Gall & Pancreas
- Jaundice
In such a case presenting as persisting and fluctuating obstructive jaundice in HCC, an intrabile duct tumor growth should be suspected. [jpatholtm.org]
The diagnosis of cirrhosis should never be established without thorough visualization of the entire biliary tract in patients with biochemical or clinical jaundice of unknown origin. [ncbi.nlm.nih.gov]
It has a lengthy latent period, usually followed by the sudden appearance of abdominal pain and swelling, hematemesis, dependent edema, or jaundice. [medical-dictionary.thefreedictionary.com]
SECONDARY BILIARY CI RR H OS I S Symptoms Clinical features · Jaundice · Pruritus · Biliary colics. SECONDARY BILIARY CI RR H OS I S Diagnosis Invest i gatio n · Cholangiography · Liver biopsy. [medicscientist.com]
- Hepatomegaly
P hy sica l e x am i natio n : May be normal (no finding) Jaundice Melanosis Xanthomas Hepatomegaly Splenomegaly Clubbing Bone tenderness Sicca syndrome (drying up of mucus membranes) CREST syndrome. [medicscientist.com]
In later phases of the disease, patients may develop hepatomegaly, xanthomas/xanthalasmas, melanotic patches, or evidence of portal hypertension (splenomegaly, ascites, edema, encephalopathy). [clinicaladvisor.com]
In her physical examination, icteric appearance, moderate hepatomegaly and kyphosis was detected. [link.springer.com]
[…] osteoarthropathy Constitutional symptoms, including anorexia, fatigue, weakness, and weight loss Cruveilhier-Baumgarten murmur—a venous hum in patients with portal hypertension Dupuytren’s contracture Fetor hepaticus—a sweet, pungent breath odor Gynecomastia Hepatomegaly [aafp.org]
• Clinical examination for hepatomegaly should be performed regularly • Upper abdominal ultrasound and blood liver function tests should form part of the annual assessment • Treatment with UDCA should begin early when abnormalities are detected clinically [cfmedicine.com]
- Asterixis
Tremor of the outstretched hands (asterixis) is common. These symptoms become more severe and may eventually progress to delirium, suicidal tendencies, and coma. Treatment. [medical-dictionary.thefreedictionary.com]
View/Print Table TABLE 2 Common Physical Examination Findings in Patients with Cirrhosis Abdominal wall vascular collaterals (caput medusa) Ascites Asterixis Clubbing and hypertrophic osteoarthropathy Constitutional symptoms, including anorexia, fatigue [aafp.org]
- Fetor Hepaticus
hepaticus—a sweet, pungent breath odor Gynecomastia Hepatomegaly Jaundice Kayser-Fleischer ring—brown-green ring of copper deposit around the cornea, pathognomonic for Wilson’s disease Nail changes: Muehrcke’s nails—paired horizontal white bands separated [aafp.org]
- Scleral Icterus
icterus Vascular spiders (spider telangiectasias, spider angiomata) Splenomegaly Testicular atrophy Most patients with cirrhosis severe enough to lead to ascites have additional stigmata of cirrhosis on physical examination. [aafp.org]
Musculoskeletal
- Osteoporosis
Medications Medications are used to reduce cholestasis, improve liver function, relieve itching and fatigue, and to treat complications such as varices and osteoporosis. [columbiasurgery.org]
[…] yellowing of the eyes and skin Other manifestations may include: Darkening of exposed areas of the skin Fever Bone pain Pale-colored, foul-smelling, bulky stools Easy bruising Night blindness Abdominal or stomach pain on the right side Bone thinning or osteoporosis [digestion.ygoy.com]
Weak bones (osteoporosis). Exercise most days of the week can help increase your bone density. If you have osteoporosis, your treatment may include calcium and vitamin D supplements. Vitamin deficiencies. [mayoclinic.org]
There are few studies on dietary management in primary biliary cirrhosis and most of them have focused on micronutrients specifically vitamin D intake to prevent osteoporosis, and lipid control to prevent hyperlipidemia, but few recommendations have been [clinicaltrials.gov]
'Thinning' of the bones (osteoporosis) is more common in women with PBC. The prevention and treatment of osteoporosis are the same as for any other woman. They are discussed in the separate leaflet called Osteoporosis. [patient.info]
- Arthralgia
A trial of corticosteroids in patients with idiopathic granulomatous hepatitis who are symptomatic, with fever, myalgias, and arthralgias, may be helpful. Empirical antituberculous therapy should be considered before instituting corticosteroids. [clevelandclinicmeded.com]
Skin
- Pruritus
SECONDARY BILIARY CI RR H OS I S Symptoms Clinical features · Jaundice · Pruritus · Biliary colics. SECONDARY BILIARY CI RR H OS I S Diagnosis Invest i gatio n · Cholangiography · Liver biopsy. [medicscientist.com]
The typical patient is a middle-aged woman presenting with symptoms of fatigue and pruritus and laboratory test evidence of cholestasis. [radiopaedia.org]
The pruritus has a diurnal variation and tends to be worst in the evenings. [clinicaladvisor.com]
The hallmark clinical feature of intrahepatic cholestasis of pregnancy is pruritus. [clevelandclinicmeded.com]
Clinical description Clinical signs of cholestasis (discolored stools, dark urine) appear within the first year of life in about one third of patients, or later with recurrent episodes of jaundice and mild pruritus. [orpha.net]
- Xanthoma
Patients will display the general clinical features of cholestasis such as pruritis, xanthomas, jaundice, steatorrhea, and deficiencies of the fat soluble vitamins A, D, E, and K. [pathwaymedicine.org]
[…] mouth Yellowing of the skin and eyes (jaundice) Darkening of the skin Swollen feet and ankles Accumulation of abdominal fluid (ascites) Fatty deposits on the skin around your eyes, your eyelids, or in the creases in your palms, soles, elbows or knees (xanthomas [columbiasurgery.org]
P hy sica l e x am i natio n : May be normal (no finding) Jaundice Melanosis Xanthomas Hepatomegaly Splenomegaly Clubbing Bone tenderness Sicca syndrome (drying up of mucus membranes) CREST syndrome. [medicscientist.com]
Affecting chiefly middle-aged women, it is characterized by chronic cholestasis (accumulation of bile in the liver) with pruritis, jaundice, and hypercholesterolemia with xanthomas, and malabsorption. [healthcentral.com]
Xanthomas are more common in PBC than in any other liver diseases associated with cholestasis. Most xanthomas do not cause symptoms, but those on the palms sometimes can be painful. [emedicinehealth.com]
Neurologic
- Encephalopathy
Other complications include hepatic encephalopathy, bleeding from dilated veins in the esophagus or dilated stomach veins, and liver cancer. Hepatic encephalopathy results in confusion and possibly unconsciousness. [icd.codes]
These are all symptoms of hepatic encephalopathy. With encephalopathy, a persons may have problems driving, writing, calculating, and performing other activities of daily living. Signs of encephalopathy are trembling and hand "flapping." [surgery.ucsf.edu]
NIH: National Institute of Diabetes and Digestive and Kidney Diseases Abdominal tap (Medical Encyclopedia) Ascites (Medical Encyclopedia) Cirrhosis (Medical Encyclopedia) Cirrhosis - discharge (Medical Encyclopedia) Hepatic encephalopathy (Medical Encyclopedia [icdlist.com]
Patients with chronic hepatic encephalopathy are placed on a protein-restricted diet. An antibiotic may be prescribed to reduce bacterial flora in the intestine. [medical-dictionary.thefreedictionary.com]
In later phases of the disease, patients may develop hepatomegaly, xanthomas/xanthalasmas, melanotic patches, or evidence of portal hypertension (splenomegaly, ascites, edema, encephalopathy). [clinicaladvisor.com]
- Personality Change
Some people with primary biliary cholangitis with liver failure have personality changes and problems with memory and concentration. Increased risk of other disease. [mayoclinic.org]
Signs of decreased mental function include confusion, personality changes, memory loss, trouble concentrating, and a change in sleep habits. [surgery.ucsf.edu]
Urogenital
- Dark Urine
Clinical description Clinical signs of cholestasis (discolored stools, dark urine) appear within the first year of life in about one third of patients, or later with recurrent episodes of jaundice and mild pruritus. [orpha.net]
Jaundice, dark urine, and darkening of the skin exposed to the sun occur as the liver disease progresses. The slow progression results eventually in portal hypertension, the accumulation of fluids, and eventually liver failure and death. [healthcentral.com]
Workup
[…] with seemingly intact, newly formed limiting plates Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present See Workup [emedicine.medscape.com]
Serum
- Hypercholesterolemia
Clinical features include 12 : pruritus (often the first symptom) jaundice skin hyperpigmentation, especially of arms and trunk xanthomata and xanthelasma due to hypercholesterolemia interestingly hypercholesterolemia does not confer an increased risk [radiopaedia.org]
Affecting chiefly middle-aged women, it is characterized by chronic cholestasis (accumulation of bile in the liver) with pruritis, jaundice, and hypercholesterolemia with xanthomas, and malabsorption. [healthcentral.com]
Affecting chiefly middle-aged women, it is characterized by chronic cholestasis with pruritus, jaundice, and hypercholesterolemia with xanthomas, and malabsorption. secondary biliary cirrhosis cirrhosis resulting from chronic bile obstruction due to congenital [medical-dictionary.thefreedictionary.com]
Hypercholesterolemia is commonly seen in PBC, but it has not been demonstrated that this is associated with increased cardiovascular risk. Hypercholesterolemia in these patients should be managed based on each patient’s cardiovascular risk profile. [clevelandclinicmeded.com]
Hypercholesterolemia and hyperlipidemia have been identified in 85% of patients. Esophageal motility problems can occasionally occur, with asymptomatic or symptomatic reflux causing esophagitis and, possibly, stricture. [emedicine.medscape.com]
Treatment
However, even after cirrhosis occurs, aggressive treatment does not increase the mortality of treatment or the treatment failure rate. [ncbi.nlm.nih.gov]
Biliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment BILI ARY CIRRHOSIS Biliary cirrhosis occurs due to injury or prolonged obstruction of intrahepatic or extrahepatic biliary system. [medicscientist.com]
Without treatment, it may eventually lead to liver failure. [nhs.uk]
Prognosis
[…] hyperintensity (cuffing) segmental hypertrophy, notably of the caudate lobe hepatic surface irregularity due to regenerative nodules regional lymphadenopathy: tends to dominate in the gastrohepatic ligament and porta hepatis 4 splenomegaly Treatment and prognosis [radiopaedia.org]
Presents as asymptomatic elevation in alkaline phosphatase (better prognosis) or with pruritus, S e con d a ry b i li a ry c irrho sis i s due to l ong-stan d ing obst ru c ti o n, of l a r ge r extrahepatic ducts. [medicscientist.com]
Prognosis The most reliable determinants of patient prognosis in primary biliary cholangitis are the height of the serum bilirubin level and the Mayo risk score. [emedicine.medscape.com]
People with primary biliary cholangitis and cirrhosis have a poor prognosis and higher risk of other complications. Increased pressure in the portal vein (portal hypertension). [mayoclinic.org]
Normalization of ALP in response to UDCA is associated with an excellent prognosis. A listing of a subset of second-line therapies, including guidelines for choosing and using these salvage therapies. [clinicaladvisor.com]
Etiology
Physical examination of patients with cirrhosis may reveal a variety of findings that necessitate a hepatic- or gastrointestinal-based work-up to determine the etiology. [aafp.org]
Etiology PFIC3 is due to mutations in the ABCB4 gene (7q21), encoding the multi-drug resistant 3 protein (MDR3), resulting in impaired biliary phospholipid (PL) secretion. [orpha.net]
[…] macronodular) resulting as a sequel to acute hepatitis. postnecrotic cirrhosis cirrhosis following submassive necrosis of the liver (subacute yellow atrophy) due to toxic or viral hepatitis. primary biliary cirrhosis a rare form of biliary cirrhosis of unknown etiology [medical-dictionary.thefreedictionary.com]
[…] unknown etiology, but various factors have been implicated as the causes of this illness, such as the following: Genetic factors: First-degree relatives have a 570- to 1000-fold increased chance of developing primary biliary cholangitis. [emedicine.medscape.com]
The etiology is unknown, although some of these patients have biliary parasites. The disease is characterized by the presence of intrahepatic pigmented stones and recurrent infection. [radiologyassistant.nl]
Epidemiology
Summary Epidemiology Estimated prevalence at birth of PFIC types 1-3 varies between 1/50,000 and 1/100,000. PFIC3 represents one third of PFIC cases. [orpha.net]
Epidemiology United States statistics Although the epidemiology of primary biliary cholangitis has not been studied systematically, the published prevalence is 65.4 cases for women and 12.1 cases for men (40.2 cases overall) per 100,000 population. [emedicine.medscape.com]
Two large epidemiological studies from the United States and Europe sought potential risk factors for PBC and found slightly increased odds ( Diseases that may occur with primary biliary cholangitis PBC is very commonly associated with other autoimmune [clinicaladvisor.com]
Relation of hemochromatosis with hepatocellular carcinoma: epidemiology, natural history, pathophysiology, screening, treatment, and prevention. Med Clin North Am. 2005;89:391–409. 19. Neimark E, Schilsky ML, Shneider BL. [aafp.org]
Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 2004; 41: 920-925. [PubMed] Lewindon PJ, Pereira TN, Hoskins AC, et al. The role of hepatic stellate cells and transforming growth factor-beta (1) in cystic fibrosis. [cfmedicine.com]
Pathophysiology
Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune [clevelandclinicmeded.com]
Although numerous pathophysiologic mechanisms of injury exist, the final common pathway is persistent wound healing resulting in hepatic parenchymal fibrosis. [aafp.org]
Pathophysiology The exact mechanism of the liver damage is unknown, although evidence indicates that it can be of autoimmune origin. [emedicine.medscape.com]
Prevention
There are few studies on dietary management in primary biliary cirrhosis and most of them have focused on micronutrients specifically vitamin D intake to prevent osteoporosis, and lipid control to prevent hyperlipidemia, but few recommendations have been [clinicaltrials.gov]
Decompression of biliary obstruction may be achieved surgically, endoscopically and via the percutaneous transhepatic route, bacterial infections can be controlled by powerful antibiotics, thus preventing the progress of cholangitis to secondary biliary [ncbi.nlm.nih.gov]
Safety Education of Pedestrians for Injury Prevention: A Systematic Review of Randomised Controlled Trials. British Medical Journal, Vol. Página xvii - América, y los Centros para el Control y la Prevención de Enfermedades de los Estados Unidos. [books.google.es]
Doherty Lippincott Williams & Wilkins, 2006 - 826 من الصفحات This volume offers authoritative, evidence-based recommendations for preventing and managing complications in all current general surgery procedures. [books.google.com]
[…] function, (4) to prevent or resolve gastrointestinal bleeding, and (5) to provide adequate nutritional intake and a positive nitrogen balance. [medical-dictionary.thefreedictionary.com]