DOCSLIB.ORG

Treatment of Primary Biliary Cirrhosis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Treatment of Primary Biliary Cirrhosis CONTRIBUTIONS to SCIENCE, 1 (1):79-85 (1999) Institut d’Estudis Catalans, Barcelona Treatment of primary biliary cirrhosis Llorenç Caballeria, Albert Parés and Juan Rodés* Unitat d’Hepatologia, Hospital Clínic i Provincial. Universitat de Barcelona Primary biliary cirrhosis (PBC) is a chronic liver disease of traceptives. In most cases, the first symptom, is pruritus unknown etiology predominantly affecting females. It is which may precede the appearance of jaundice by months characterized by inflammation and destruction of the intra- or even years. The initial clinical manifestations vary in hepatic biliary ducts and it leads to the development of length from one patient to another, until the manifestations of chronic cholestasis. The clinical manifestations are similar to the different stages are presented. Jaundice, xanthomas, those of cirrhosis, including pruritus, jaundice, xanthomas, steatorrhea and malnutrition may also be observed. steatorrhea and malabsorption [1]. The most common biochemical alteration of PBC is an in- The disease generally affects women between 40 to 60 crease in all those substances normally eliminated by the years of age. The age at the time of diagnosis, however, is bile. Alkaline phosphatases are usually elevated, with the decreasing with diagnosis being achieved earlier and in levels increasing with the evolution of the disease [7,8]. asymptomatic patients. The reason for the female predomi- Other cholestatic markers, such as 5’-nucleotidase and nance of 90% is unknown [2]. Primary biliary cirrhosis has gammaglutamyltransferase are also elevated. Bilirubin is been reported throughout the world. The prevalence in usually normal at initiation of PBC and increases on progres- Western Europe is 22 cases per one million inhabitants, al- sion of the disease. The levels of bile acids and cholesterol though this may vary from country to country and within a are usually elevated during the disease. These changes are country. Although familial cases have been reported, there probably due to inhibition of the hepatic lipase disease in the is no epidemiologic evidence that the disease is hereditary intitial stages and a decrease in the esterification of choles- or limited to certain social or ethnic groups [3]. terol in the most advanced phases [9]. A slight elevation in Scheuer classified PBC into 4 stages from a morphologic transaminases may be observed. Albumin and prothrombin point of view [4]. In Stage I, a lesion characteristic of the bil- time are usually normal in the initial stages of the disease, iary ducts (granuloma) may be seen. In Stage II there is duc- but may change with progression and the appearance of tal proliferation, hepatic fibrosis is observed in Stage III and signs of liver failure [7,8]. Moreover, hypergammaglobuline- hepatic cirrhosis in Stage IV. The stages are usually pro- mia, erythrocyte sedimentation rate and normocytic and nor- gressive and, in some cases, lesions of one or two stages mochronic anemia may be observed. In 75% of the cases, may overlap. This histologic classification was later modified hypergammaglobulinema is a consequence of the marked by Ludwig et al, and lesions were redefined as portal, peri- increase in immunoglobulin M. Immunoglobulin G is also portal, septal and cirrhotic [5,6]. usually increased, while immunoglobulin A is usually normal. The initiation of the disease is variable. It may be acci- One of the most important diagnostic tests is the determi- dently found during a routine medical examination with the nation of antimitochondrial antibodies (AMA) which are observation of hepatomegaly or splenomegaly or on deter- found in 90 to 95% of the cases of PBC. The AMA are non mining a marked increase in alkaline phosphatases or a organospecific antibodies that react against a lipoproteidic slight rise in transaminases (in the asymptomatic forms). On component of the internal membrane of the mitochondria other occasions, the initiation is insidious, similar to other [10]. To date, 9 types of AMA have been determined and it chronic liver diseases. In certain cases, the first clinical man- has been demonstrated that they may be associated with ifestations are like those of autoimmune chronic hepatitis several diseases, some of which are specific. Patients with and in other cases they are similar to hepatic cirrhosis. The PBC react with the M2, M4, M8 and M9 subtypes, with the disease sometimes becomes manifest with pruritus and most frequent in PBC being the M2 subtypes [11]. In addi- jaundice in pregnancy or after the consumption of oral con- tion to AMA, other non organospecific circulating antibodies, such as antinuclear [12], antithyroid and anti smooth muscle antibodies [13] may be observed. * Autor for corresponde: Juan Rodés, Unitat d’Hepatologia, Hos- pital Clínic i Provincial, Villarroel, 170. 08036 Barcelona, Catalonia (Spain). Fax: 34 93 451 55 22. Email: [email protected] 80 Llorenç Caballeria, Albert Parés, Juan Rodés Treatment of PBC Treatment of malabsorption Due to a lack of bile acids in the intestinal lumen, malabsorp- Treatment of PBC is directed at improving, or preventing, the tion of liposoluble vitamins is observed. To avoid these defi- effects of chronic cholestasis and reversing the mechanism ciences, vitamin D should be administered as 25-hydroxyc- or mechanisms determining its appearance by specific holecalciferol at a dose of 266 ␮g every 8-15 days. Calcium treatment. supplements such as calcium gluconate are given orally at 3 g/day, representing 1500 mg of calcium element/day [26]. Symptomatic treatment Vitamin A is administered at a dose of 50,000 IU every 15 Patients may carry out normal physical activity while their days to avoid hemeralopia or night blindness [27]. Vitamin K general state allows them to do so. No special diet is re- may be administered intramuscularly (10 mg every week) if quired, as long as correct protein and calory intake is main- the prothrombin time is decreased. Vitamin E is given orally tained. Moderate sunbathing is convenient and at least half at 200 mg/day to improve asthenia [28]. In some malnour- a liter of milk should be ingested daily to counterbalance the ished patients, medium chain triglycerides are recommend- deficit of intestinal vitamin D and calcium absorption. Only ed such as coconut oil extract at one soup spoon 3 or 4 alcohol intake is contra-indicated. times a day. Treatment of pruritis Treatment of osteodystrophy Pruritus is one of the most bothersome symptoms in PBC. Osteodystrophy is frequent in PBC patients, consisting of Many therapeutic measures have been used, among which the combination of a progressive reduction in bone volume the administration of cholestyramine and phenobarbitone (osteoporosis) and a bone mineralization deficit (osteomala- are the most well known. cia) [29]. The previously mentioned administration of vitamin Cholestyramine is a resin of ionic exchange which D and calcium at the doses indicated does not always pre- is bound to the bile salts in the intestinal lumen impeding vent the appearance of osteoporosis [30]. Since osteoporo- their absorption. It is administered orally at an intitial dose sis is fundamentally produced as a consequence of defi- of 12 g 3 times a day [14-17], with administration at break- cient osteoblastic function, sodium fluoride [31] has been fast being double that of the posterior dose since the biliary studied in these patients with good results and has been vesicle is full of bile in the morning. It is therefore recom- found to prevent a decrease in bone mass. At present, other mended that the dosage be divided as 4 g before break- antireabsorption therapies such as calcitonine and dephos- fast, 4 g after breakfast and the remaining 4 g before to phonates are being studied. lunch. A reduction in pruritus is not usually observed for about 3 days, when the reservoir of bile salts has de- Treatment of hyperlipidemia creased. Cholestyramine may cause constipation and in- There is currently no adequate treatment against hyper- testinal discomfort. In cases in which cholestyramine is not lipemia in PBC. In cases with intense neuritic pain by xan- effective at the dosis indicated, an increase in dosage is thomatous invasion of the nerve lining, plasmapheresis with not useful. activated charcoal is recommended. Phenobarbitone is a powerful enzyme inducer and cholerectic which increases bile flow, regardless of the bile Specific treatment salts. It is usually administered at an initial dose of 3 mg/kg Several specific therapies for PBC have been studied. These of b.w. over the first 4 days, and is thereafter reduced to 50- treatments are usually divided into 2 groups; immunomodu- 100 mg/day in only one nightly dose. Prolonged phenobarbi- lators, such as corticoids, azathioprine, cyclosporin and tone administration may produce somnolescence and alter methotrexate and other drugs which, in addition to their anti- vitamin D metabolism. inflammatory action, produce an antifibrogenetic effect, Rifampicin is an antibiotic with an intense induction ca- such as penicillamine and colchicine. The other group only pacity on the hepatic microsomal system. It significantly re- includes ursodeoxycholic acid, the beneficial effect of which duces pruritus and is much more effective than phenobarbi- is due to a decrease in toxic bile acids. tone [18-21]. In addition, it is accompanied by a marked decrease in alkaline phosphatase levels, transaminases Corticoids and even, despite the enzymatic induction effect, by a de- Corticoids have an immunosuppressive effect and were first crease in gammaglutamyltransferase. Rifampicin may be administered in 1955. In this study, promising beneficial clin- administered over long periods with a certain amount of ical and biochemical data were demonstrated but with the safety, although the transaminase levels should be monitor- disadvantage of a clear exacerbation of the metabolic bone ized for hepatotoxicity, which occurs in about 15% of the disease. In later studies [32], similar results were observed, cases. even with the addition of oral calcium and vitamin D supple- Other substances used against pruritus in PBC are opiate ments.
Load more

Recommended publications
  • Intro to Gallbladder & Pancreas Pathology
    Cholecystitis acute chronic Gallbladder tumors Adenomyoma (benign) Intro to Adenocarcinoma Gallbladder & Pancreatitis Pancreas acute Pathology chronic Pancreatic tumors Helen Remotti M.D. Case 1 70 year old male came to the ER. CC: 5 hours of right –sided abdominal pain that had awakened him from sleep ; also pain in the right shoulder and scapula. Previous episodes mild right sided abdominal pain lasting 1- 2 hours. 1 Case 1 Febrile with T 100.7 F, pulse 100, BP 150/90 Abdomen: RUQ and epigastric tenderness to light palpation, with inspiratory arrest and increased pain on deep palpation. (Murphy’s sign) Labs: WBC 12,500; (normal bilirubin, Alk phos, AST, ALT). Ultrasound shows normal liver, normal pancreas without duct dilatation and a distended thickened gallbladder with a stone in cystic duct. DIAGNOSIS??? 2 Acute Cholecystitis Epigastric, RUQ pain Radiate to shoulder Fever, chills Nausea, vomiting Mild Jaundice RUQ guarding, tenderness Tender Mass (50%) Acute Cholecystitis Stone obstructs cystic duct G.B. distended Mucosa disrupted Chemical Irritation: Conc. Bile Bacterial Infection 50 - 70% + culture: Lumen 90 - 95% + culture: Wall Bowel Organisms E. Coli, S. Fecalis 3 Culture Normal Biliary Tree: No Bacteria Bacteria Normally Cleared In G.B. with cholelithiasis Bacteria cling to stones If stone obstructs cystic duct orifice G.B. distended Mucosa Disrupted Bacteria invade G.B. Wall 4 Gallstones (Cholelithiasis) • 10 - 20% Adults • 35% Autopsy: Over 65 • Over 20 Million • 600,000 Cholecystectomies • #2 reason for abdominal operations
    [Show full text]
  • General Signs and Symptoms of Abdominal Diseases
    General signs and symptoms of abdominal diseases Dr. Förhécz Zsolt Semmelweis University 3rd Department of Internal Medicine Faculty of Medicine, 3rd Year 2018/2019 1st Semester • For descriptive purposes, the abdomen is divided by imaginary lines crossing at the umbilicus, forming the right upper, right lower, left upper, and left lower quadrants. • Another system divides the abdomen into nine sections. Terms for three of them are commonly used: epigastric, umbilical, and hypogastric, or suprapubic Common or Concerning Symptoms • Indigestion or anorexia • Nausea, vomiting, or hematemesis • Abdominal pain • Dysphagia and/or odynophagia • Change in bowel function • Constipation or diarrhea • Jaundice “How is your appetite?” • Anorexia, nausea, vomiting in many gastrointestinal disorders; and – also in pregnancy, – diabetic ketoacidosis, – adrenal insufficiency, – hypercalcemia, – uremia, – liver disease, – emotional states, – adverse drug reactions – Induced but without nausea in anorexia/ bulimia. • Anorexia is a loss or lack of appetite. • Some patients may not actually vomit but raise esophageal or gastric contents in the absence of nausea or retching, called regurgitation. – in esophageal narrowing from stricture or cancer; also with incompetent gastroesophageal sphincter • Ask about any vomitus or regurgitated material and inspect it yourself if possible!!!! – What color is it? – What does the vomitus smell like? – How much has there been? – Ask specifically if it contains any blood and try to determine how much? • Fecal odor – in small bowel obstruction – or gastrocolic fistula • Gastric juice is clear or mucoid. Small amounts of yellowish or greenish bile are common and have no special significance. • Brownish or blackish vomitus with a “coffee- grounds” appearance suggests blood altered by gastric acid.
    [Show full text]
  • Chronic Pancreatitis. 2
    Module "Fundamentals of diagnostics, treatment and prevention of major diseases of the digestive system" Practical training: "Chronic pancreatitis (CP)" Topicality The incidence of chronic pancreatitis is 4.8 new cases per 100 000 of population per year. Prevalence is 25 to 30 cases per 100 000 of population. Total number of patients with CP increased in the world by 2 times for the last 30 years. In Ukraine, the prevalence of diseases of the pancreas (CP) increased by 10.3%, and the incidence increased by 5.9%. True prevalence rate of CP is difficult to establish, because diagnosis is difficult, especially in initial stages. The average time of CP diagnosis ranges from 30 to 60 months depending on the etiology of the disease. Learning objectives: to teach students to recognize the main symptoms and syndromes of CP; to familiarize students with physical examination methods of CP; to familiarize students with study methods used for the diagnosis of CP, the determination of incretory and excretory pancreatic insufficiency, indications and contraindications for their use, methods of their execution, the diagnostic value of each of them; to teach students to interpret the results of conducted study; to teach students how to recognize and diagnose complications of CP; to teach students how to prescribe treatment for CP. What should a student know? Frequency of CP; Etiological factors of CP; Pathogenesis of CP; Main clinical syndromes of CP, CP classification; General and alarm symptoms of CP; Physical symptoms of CP; Methods of
    [Show full text]
  • Intro to Gallbladder & Pancreas Pathology Gallstones Chronic
    Cholecystitis Choledocholithiasis acute chronic (Stones in the common bile duct) Gallbladder tumors Pain: Epigastric, RUQ-stones may be passed Adenomyoma (benign) Intro to Obstructive Jaundice-may be intermittent Adenocarcinoma Gallbladder & Ascending Cholangitis- Infection: to liver 20%: No pain; 25% no jaundice Pancreatitis Pancreas acute Pathology chronic Pancreatic tumors Helen Remotti M.D. Gallstones (Cholelithiasis) • 10 - 20% Adults • 35% Autopsy: Over 65 • Over 20 Million • 600,000 Cholecystectomies • #2 reason for abdominal operations Acute cholecystitis = ischemic injury Cholesterol/mixed stones Chronic Cholecystitis • Associated with calculi in 95% of cases. • Multiples episodes of inflammation cause GB thickening with chronic inflammation/ fibrosis and muscular hypertrop hy . • Rokitansky - Aschoff Sinuses (mucosa herniates through the muscularis mucosae) • With longstanding inflammation GB becomes fibrotic and calcified “porcelain GB” 1 Chronic Cholecystitis • Fibrosis • Chronic Inflammation • Rokitansky - Aschoff Sinuses • Hypertrophy: Muscularis Chronic cholecystitis Cholesterolosis Focal accumulation of cholesterol-laden macrophages in lamina propria of gallbladder (incidental finding). Rokitansky-Aschoff sinuses Adenomyoma of Gall Bladder 2 Carcinoma: Gall Bladder Uncommon: 5,000 cases / year Fewer than 1% resected G.B. Sx: same as with stones 5 yr. survival: Less than 5% (survival relates to stage) 90%: Stones Long Hx: symptomatic stones Stones: predispose to CA., but uncommon complication 3 Gallbladder carcinoma Acute pancreatitis Case 1 56 year old woman presents to ER in shock, following rapid onset of severe upper abdominal pain, developing over the previous day. Hx: heavy alcohol use. LABs: Elevated serum amylase and elevated peritoneal fluid lipase Acute Pancreatitis Patient developed rapid onset of respiratory failure necessitating intubation and mechanical ventilation. Over 48 hours, she was increasingly unstable, with evolution to multi-organ failure, and she expired 82 hours after admission.
    [Show full text]
  • Chronic Pancreatitis
    CHRONIC PANCREATITIS Chronic pancreatitis is an inflammatory pancreas disease with the development of parenchyma sclerosis, duct damage and changes in exocrine and endocrine function. The causes of chronic pancreatitis Alcohol; Diseases of the stomach, duodenum, gallbladder and biliary tract. With hypertension in the bile ducts of bile reflux into the ducts of the pancreas. Infection. Transition of infection from the bile duct to the pancreas, By the vessels of the lymphatic system, Medicinal. Long -term administration of sulfonamides, antibiotics, glucocorticosteroids, estrogens, immunosuppressors, diuretics and NSAIDs. Autoimmune disorders. Congenital disorders of the pancreas. Heredity; In the progression of chronic pancreatitis are playing important pathological changes in other organs of the digestive system. The main symptoms of an exacerbation of chronic pancreatitis: Attacks of pain in the epigastric region associated or not with a meal. Pain radiating to the back, neck, left shoulder; Inflammation of the head - the pain in the right upper quadrant, body - pain in the epigastric proper region, tail - a pain in the left upper quadrant. Pain does not subside after vomiting. The pain increases after hot-water bottle. Dyspeptic disorders, including flatulence, Malabsorption syndrome: Diarrhea (50%). Feces unformed, (steatorrhea, amylorea, creatorrhea). Weight loss. On examination, patients. Signs of hypovitaminosis (dry skin, brittle hair, nails, etc.), Hemorrhagic syndrome - a symptom of Gray-Turner (subcutaneous hemorrhage and cyanosis on the lateral surfaces of the abdomen or around the navel) Painful points in the pathology of the pancreas Point choledocho – pankreatic. Kacha point. The point between the outer and middle third of the left costal arch. The point of the left phrenic nerve.
    [Show full text]
  • Difference Between Cholecystitis and Cholelithiasis Key Difference – Cholecystitis Vs Cholelithiasis
    Difference Between Cholecystitis and Cholelithiasis www.differencebetween.com Key Difference – Cholecystitis vs Cholelithiasis Bile is a substance produced by the liver and stored in the gallbladder. It emulsifies the fat globules in the food we eat and enhances their water solubility and their absorption into the bloodstream. When the bile stored in the gallbladder is abnormally concentrated, some of its constituents can precipitate, forming stones inside the gallbladder. In medicine, this condition is identified as cholelithiasis. Cholelithiasis can inflame the tissues of the gallbladder. This inflammatory process happening inside the gallbladder is called cholecystitis. Thus, the key difference between cholecystitis and cholelithiasis is that cholecystitis is the inflammation of the gallbladder while cholelithiasis is the formation of gallstones. Cholecystitis is actually a complication of cholelithiasis which is either not diagnosed or not properly treated. What is Cholecystitis? The inflammation of the gallbladder is known as cholecystitis. In most occasions, this is due to an obstruction to the outflow of bile. Such an obstruction increases the pressure inside the gallbladder resulting in its distension which compromises the vascular supply to the gallbladder tissues. Causes Gallstones Tumors in the gallbladder or biliary tract Pancreatitis Ascending cholangitis Trauma Infections in the biliary tree Clinical Features Intense epigastric pain which radiates to the right shoulder or the back in the tip of the scapula. Nausea and vomiting Occasionally fever Abdominal bloating Steatorrhea Jaundice Pruritus Investigations Liver function tests Full blood count USS CT scan is also performed sometimes MRI Figure 01: Chronic Recurrent Cholecystitis Management As in chronic pancreatitis, the treatment of gallbladder attacks also varies according to the underlying cause of the disease.
    [Show full text]
  • Malabsorption: Etiology, Pathogenesis and Evaluation
    Malabsorption: etiology, pathogenesis and evaluation Peter HR Green NORMAL ABSORPTION • Coordination of gastric, small intestinal, pancreatic and biliary function • Multiple mechanisms Fat protein carbohydrate vitamins and minerals 1 NORMAL ABSORPTION • Integrated and coordinated response involving different organs, enzymes, hormones, transport and secretory mechanisms • Great redundancy 2 DIFFERENTIAL SITES OF ABSORPTION • Fat, carbohydrate and protein can be absorbed along the entire length (22 feet) • Vitamins and minerals are absorbed at different sites Fat Protein CHO 3 ABSORPTION LUMINAL MUCOSAL REMOVAL FAT ABSORPTION • GASTRIC PHASE lingual lipase • INTESTINAL luminal mucosal lymphatic (delivery) 4 FAT ABSORPTION • Luminal phase chyme pancreatic secretion – lipase, colipase micelle formation – bile salts, lecithin • Intestinal phase transport, chylomicron formation, secretion • Transport (lymphatic) phase FAT MALABSORPTION • Luminal phase altered motility - chyme pancreatic insufficiency - pancreatic secretion – lipase, colipase micelle formation – bile salts, lecithin • Intestinal phase transport, chylomicron formation, secretion • Transport (lymphatic) phase 5 Pancreas FunctionalFunctional LipaseLipase Reserve Reserve FAT MALABSORPTION • Luminal phase altered motility - chyme pancreatic insufficiency –cancer, ductal obstruction, chronic pancreatitis biliary tract / liver disease – cirrhosis, bile duct cancer SMALL INTESTINAL BACTERIAL OVERGROWTH 6 SMALL INTESTINAL BACTERIAL OVERGROWTH BLIND LOOP SYNDROME JEJUNAL DIVERTICULOSIS
    [Show full text]
  • TUMOUR and TREATMENT SIDE-EFFECTS
    TUMOUR and TREATMENT SIDE-EFFECTS TUMOUR TUMOUR TREATMENT EFFECTS RADIATION SITE EFFECTS CHEMOTHERAPY SURGERY THERAPY Head & Neck Difficulty Nausea Mucositis Impaired chewing & chewing or Vomiting Stomatitis swallowing swallowing Diarrhea Dysgeusia, Xerostomia Stomatitis hypogeusia Xerostomia, dysphagia Difficulty chewing secondary to dental decay or infection Viscous saliva Oropharyngeal ulceration Osteoradionecrosis Fistula Trismus Esophagus Dysphagia Nausea Dysphagia Decreased gastric secondary to Esophagitis motility esophageal Esophageal fibrosis or Decreased gastric obstruction stricture acid production Regurgitation Fistula Fistula of meals Nausea Esophageal stenosis Edema Regurgitation Steatorrhea Stomach Early satiety Nausea Nausea Fat malabsorption & Vomiting after Vomiting Vomiting diarrhea meals Stomatitis Decreased gastric Diarrhea motility "Dumping" syndrome Hypoglycemia Protein malabsorption Deficiences in iron, calcium, fat-soluble vitamins & vitamin B12 Esophagitis Pancreas & Malabsorption Nausea Nausea Diabetes mellitus biliary tree &/or diabetes Vomiting Vomiting Malabsorption of fat, secondary to Stomatitis protein, & fat-soluble pancreatic Diarrhea vitamins & insufficiency Minerals Dysgeusia Nausea Small Bowel Nausea Gastrointestinal Loss of bile salts, intestine obstruction Stomatitis ulceration calcium, magnesium, Malabsorption Diarrhea Villous hypoplasia zinc Malabsorption Metabloic acidosis secondary to Increased risk of decreased enzyme renal stones production Postoperative gastric Intestinal fistula hypersecretion
    [Show full text]
  • Post-Whipple: a Practical Approach to Nutrition Management
    NINFLAMMATORYUTRITION ISSUES BOWEL IN GASTROENTEROLO DISEASE: A PRACTICALGY, SERIES APPROACH, #108 SERIES #73 Carol Rees Parrish, M.S., R.D., Series Editor Post-Whipple: A Practical Approach to Nutrition Management Nora Decher Amy Berry The classic pancreatoduodenectomy (PD), also known as the Kausch-Whipple, and the pylorus- preserving pancreatoduodenectomy (PPPD) are most commonly performed for treatment of pancreatic cancer and chronic pancreatitis. This highly complex surgery disrupts the coordination of tightly orchestrated digestive processes. This, in combination with a diseased gland, sets the patient up for nutritional complications such as altered motility (gastroparesis and dumping), pancreatic insufficiency, diabetes mellitus, nutritional deficiencies and bacterial overgrowth. Close monitoring and attention to these issues will help the clinician optimize nutritional status and help prevent potentially devastating complications. 63-year-old female, D.D., presented to the copper, zinc, selenium, and potentially thiamine University of Virginia Health System (UVAHS) (thiamine was repleted before serum levels were Awith weight loss and biliary obstruction. She was checked). A percutaneous endoscopic gastrostomy with diagnosed with a large pancreatic serous cystadenoma jejunal extension (PEG-J) was placed due to intolerance and underwent a pancreatoduodenectomy (PD) of gastric enteral nutrition (EN). After several more (standard Whipple procedure with partial gastrectomy) hospitalizations, prolonged rehabilitation in a nursing with posterior anastomosis and cholecystectomy. Seven home, 7 months of supplemental nocturnal EN, and months later she was admitted to UVAHS with nausea, treatment of pancreatic insufficiency with pancreatic vomiting, diarrhea and a severe weight loss of 47lbs enzymes (with her meals and EN), D.D. was able to (33% of her usual body weight).
    [Show full text]
  • Idiopathic Hemochromatosis Presenting As Malabsorption Syndrome
    CLEVELAND CLINIC QUARTERLY Volume 37, July 1970 Copyright © 1970 by The Cleveland Clinic Foundation Printed in U.S.A. Idiopathic hemochromatosis presenting as malabsorption syndrome Report of a case JOHN R. KRAMER, JR., M.D.* RICHARD G. FARMER, M.D. Department of Gastroenterology EMOCHROMATOSIS is a disease of altered iron metabolism, as- H sociated with parenchymal cell damage, particularly in the liver, pan- creas, and myocardium. The triad of hepatic disease, hyperpigmentation of the skin, and diabetes mellitus is well known. Additional clinical findings such as testicular atrophy, congestive heart failure, portal hypertension, and hepatoma have also been reported.13 The fundamental pathologic defect in idiopathic hemochromatosis is not known. There has been considerable controversy in the last decade4-9 as to whether or not the syndrome represents a clinical entity, or a variant of portal cirrhosis of the liver as suggested by MacDonald and associ- ates.4- 6-8 It has been noted that an increase in ingestion of exogenous iron, in excess of iron loss, may lead to increased deposition of iron in tissues, with characteristic clinical features.10 In addition, there is a body of evidence indicating that hemochromatosis may be the result of a genetic defect—an autosomal dominant with incomplete penetrance. Stud- ies of families have tended to support this view.9- 11 A portion of the renewed interest in the pathogenesis and clinical fea- tures of hemochromatosis has been the result of improved therapeutic measures, largely due to the efficacy of repeated venesections.3- 12 There- fore, although rare, the syndrome of hemochromatosis has received some- what disproportionate interest by clinical investigators.
    [Show full text]
  • Chronic Pancreatitis: Diagnosis and Treatment
    Chronic Pancreatitis: Diagnosis and Treatment Kathleen Barry, MD, University of Texas Health Science Center at Houston, Houston, Texas Chronic pancreatitis is an irreversible and progressive disorder of the pancreas characterized by inflammation, fibrosis, and scarring. Exocrine and endocrine functions are lost, often leading to chronic pain. The etiology is multifactorial, although alcoholism is the most significant risk fac- tor in adults. The average age at diagnosis is 35 to 55 years. If chronic pancreatitis is suspected, contrast-enhanced computed tomography is the best imaging modality for diagnosis. Computed tomography may be inconclusive in early stages of the disease, so other modalities such as magnetic resonance imaging, magnetic resonance cholangiopancreatography, or endoscopic ultrasonogra- phy with or without biopsy may be used. Recommended lifestyle modifications include cessation of alcohol and tobacco use and eating small, frequent, low-fat meals. Although narcotics and antide- pressants provide the most pain relief, one-half of patients eventually require surgery. Therapeutic endoscopy is indicated to treat symptomatic strictures, stones, and pseudocysts. Decompressive surgical procedures, such as lateral pancreaticojejunostomy, are indicated for large duct disease (pancreatic ductal dilation of 7 mm or more). Resection procedures, such as the Whipple procedure, are indicated for small duct disease or pancreatic head enlargement. The risk of pancreatic cancer is increased in patients with chronic pancreatitis, especially hereditary pancreatitis. Although it is not known if screening improves outcomes, clinicians should counsel patients on this increased risk and evaluate patients with weight loss or jaundice for neoplasm. (Am Fam Physician. 2018;97(6):385-393. Copyright © 2018 American Academy of Family Physicians.) Chronic pancreatitis is a permanent, progressive de­ about four and 12 per 100,000 persons per year.
    [Show full text]
  • Primary Biliary Cholangitis
    LIVER DISORDERS, SERIES #4 Rad Agrawal, MD, FACP, FACG, AGAF, FASGE Primary Biliary Cholangitis Duminda Suraweera Courtney Reynolds Shehan Thangaratnam Gaurav Singhvi Primary biliary cholangitis, previously known as primary biliary cirrhosis, is a chronic, cholestatic, inflammatory liver disease characterized by destruction of intrahepatic bile ducts resulting in progressive liver damage. The etiology of primary biliary cholangitis is still not fully understood but likely involves both environmental and genetic factors. If symptomatic, patients often present with fatigue and pruritus. About 95% of individuals with primary biliary cholangitis will have a positive anti- mitochondrial antibody. Current treatment options are limited primarily to ursodeoxycholic acid, while those with decompensated liver disease will require liver transplantation. We provide a review of the etiology, pathogenesis, natural history, diagnosis and management of primary biliary cholangitis. EPIDEMIOLOGY, ETIOLOGY AND PATHOGENESIS rimary biliary cholangitis (PBC) has an incidence The etiology of PBC is not fully understood, but rate of 0.9 to 5.8 per 100,000 people with an both genetic predisposition and environmental triggers Pestimated female to male ratio of 10 to 1.1-3 PBC likely play an important role. The prevalence of PBC is is more common in patients in Northern Europe, the higher in families with an affected member, indicating United Kingdom and the northern United States. The a possible genetic component. Approximately 1.2% of prevalence seems to have increased over time, currently children of PBC patients develop PBC, with the highest ranging from 1.91 to 40.2 per 100,000 people.3 The risk in daughters of women with PBC.4 Monozygotic increase in prevalence is likely multifactorial with twins have a high concordance rate of 0.63 for PBC.5 increased disease diagnosis and increased survival with Additionally, several studies have shown a link between improved treatment.
    [Show full text]