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The complications of iron overload in hemochromatosis can be avoided by early diagnosis and appropriate management. Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 microg/L or more and in women with serum ferritin levels of 200 microg/L or more, regardless of the presence or absence of symptoms. Typically, therapeutic phlebotomy consists of 1) removal of 1 unit (450 to 500 mL) of blood weekly until the serum ferritin level is 10 to 20 microg/L and 2) maintenance of the serum ferritin level at 50 microg/L or less thereafter by periodic removal of blood. Hyperferritinemia attributable to iron overload is resolved by therapeutic phlebotomy. When applied before iron overload becomes severe, this treatment also prevents complications of iron overload, including hepatic cirrhosis, primary liver cancer, diabetes mellitus, hypogonadotrophic hypogonadism, joint disease, and cardiomyopathy. In patients with established iron overload disease, weakness, fatigue, increased hepatic enzyme concentrations, right upper quadrant pain, and hyperpigmentation are often substantially alleviated by therapeutic phlebotomy. Patients with liver disease, joint disease, diabetes mellitus and other endocrinopathic abnormalities, and cardiac abnormalities often require additional, specific management. Dietary management of hemochromatosis includes avoidance of medicinal iron, mineral supplements, excess vitamin C, and uncooked seafoods. This can reduce the rate of iron reaccumulation; reduce retention of nonferrous metals; and help reduce complications of liver disease, diabetes mellitus, and Vibrio infection. This comprehensive approach to the management of hemochromatosis can decrease the frequency and severity of iron overload, improve quality of life, and increase longevity.
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PMID:Management of hemochromatosis. Hemochromatosis Management Working Group. 986 45

Gallstone disease remains one of the most common medical problems leading to surgical intervention. Every year, approximately 500,000 cholecystectomies are performed in the US. Cholelithiasis affects approximately 10% of the adult population in the United States. It has been well demonstrated that the presence of gallstones increases with age. An estimated 20% of adults over 40 years of age and 30% of those over age 70 have biliary calculi. During the reproductive years, the female-to-male ratio is about 4:1, with the sex discrepancy narrowing in the older population to near equality. The risk factors predisposing to gallstone formation include obesity, diabetes mellitus, estrogen and pregnancy, hemolytic diseases, and cirrhosis. A study of the natural history of cholelithiasis demonstrates that approximately 35% of patients initially diagnosed with having, but not treated for, gallstones later developed complications or recurrent symptoms leading to cholecystectomy. During the last two decades, the general principles of gallstone management have not notably changed. However, methods of treatment have been dramatically altered. Today, laparoscopic cholecystectomy, laparoscopic common bile duct exploration, and endoscopic retrograde management of common bile duct (CBD) stones play important roles in the treatment of gallstones. These technological advances in the management of biliary tract disease are not infrequently accomplished by a multidisciplinary team of physicians, including surgeons trained in laparoscopic techniques, interventional gastroenterologists, and interventional radiologists. With the evolution of laparoscopic cholecystectomy, there has been a global reeducation and retraining program of surgeons. However, the treatment of choice for gallstones remains cholecystectomy. In recognition of the revolutionary advances in the treatment of cholelithiasis, it is the purpose of this collective review to describe recent information on the following topics: types of gallstones, asymptomatic gallstones, symptomatic gallstones, chronic cholecystitis, acute cholecystitis, and other complications of gallstones. Gross and compositional analysis of gallstones allows them to be classified as cholesterol, mixed, and pigment gallstones. When asymptomatic gallstones are detected during the evaluation of a patient, a prophylactic cholecystectomy is normally not indicated because of several factors. Only about 30% of patients with asymptomatic cholelithiasis will warrant surgery during their lifetime, suggesting that cholelithiasis can be a relatively benign condition in some people. However, there are certain factors that predict a more serious course in patients with asymptomatic gallstones and warrant a prophylactic cholecystectomy when they are present. These factors include patients with large (>2.5 cm) gallstones, patients with congenital hemolytic anemia or nonfunctioning gallbladders, or during bariatric surgery or colectomy. Epigastric and right upper quadrant pain occurring 30-60 minutes after meals is frequently associated with gallstone disease. The diagnosis of chronic cholecystitis is made by the presence of biliary colic with evidence of gallstones on an imaging study. Ultrasonography is the diagnostic test of choice, being 90-95% sensitive. The surgical literature suggests that 3-10% of patients undergoing cholecystectomy will have CBD stones. Intraoperative laparoscopic ultrasonography has recently replaced cholangiography as the method of choice for detecting CBD stones. Ultrasonography and radionuclide cholescintigraphy (HIDA scan) are useful in establishing a diagnosis of acute cholecystitis. Laparoscopic cholecystectomy should also be used in the treatment of acute cholecystitis. Laparoscopic cholecystectomy is more likely to be successful when performed within 3 days of the onset of symptoms. It is important to remember that gallstones can lead to a variety of other complications including choledocholithiasis, gallstone ileus, and acute gallstone pancreatitis.
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PMID:Cholelithiasis and cholecystitis. 1602 43

Non-alcoholic steatohepatitis (NASH) is one of the most common liver disorders. This is highly prevalent in obese and diabetic subjects. Persons with central obesity are at particular risk. Other clinical predictors are age more than 40-50 years and hyperlipidemias, but none of these factors is invariable for causation of NASH. Other reported associations are, celiac disease, Wilson's Disease and few other metabolic diseases. Drugs, particularly amiodarone, tamoxifen, nucleoside analogues and methotrxate have also been linked to NASH. The disease is evenly distributed in both sexes but advanced disease is more common in women. Ethnic variation exists and African Americans are less affected than Hispanic Americans. Specific clinical features of NASH are infrequent. Patients usually come to clinical attention by elevated liver enzymes found on routine evaluation but on history, about two third of patients will admit to have mild fatigue and about half will report right upper quadrant pain. Rarely, patient may present with a complication of cirrhosis. Physical examination may reveal hepatomegaly and splenomegaly. Research in last few years has stressed that development of steatosis, stetohepatitis, fibrosis with subsequent cirrhosis are most probably the result of insulin resistance. Therefore, clinical features may reflect existence of insulin resistance. Obesity, particularly central obesity is most important of these. Patients may have sleep apnea syndrome. Hypertension and manifestations of diabetes mellitus like polyuria, polydypsia, and neurological deficits may occur. Patients may have varying combination of obesity, diabetes, hyperlipidemia, hypertension and impaired fibrinolysis (syndrome X). Children with insulin resistance may show acanthosis nigricance. Patients with polycystic ovary syndrome, which consists of insulin resistance, diabetes, obesity, hirsutism, oligo or polymenorrha and hyperlipidemia may have NASH. Other rare manifestations of insulin resistance, which can be seen in patients of NASH are lipomatosis, lipoatrophy/lipodystrophy and panniculitis. Most other rare conditions known to cause NASH like peroxisomal diseases, mitochondialpathies, Weber-Christian disease, Mauriac syndrome, Madelung's lipomatosis and abetaliopprotenemia also have insulin resistance. This is believed that primary defect underlying insulin resistance is impairment in postreceptor pathways (through tyrosine kinase activity) of insulin action. Primary defect in insulin receptors appear uncommon. This results in down regulation of insulin receptor substance 1 (IRS-1) signaling by excess free fatty acids. In muscle, activated IRS-1 promotes translocation of glucose transporter protein 4 (GLUT4) to cell membrane. As a result, monocyte glucose uptake by GLUT4 increases glucose disposal from blood and reduced need for insulin. PKC-0 is a likely candidate as serine kinase in muscle regulated by fatty acids that can impair the activation of IRS-1. Insulin resistance is usually evaluated by fasting insulin levels, Quantitative Insulin Check Index (QUICKI) and Homeostasis Model Assessment of Insulin Resistance (HOMA), C-peptid/insulin ratio oral glucose tolerance test and hyper insulinemic euglycemic clamp. The clamp technique is considered the gold standard.
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PMID:Insulin resistance and clinical aspects of non-alcoholic steatohepatitis (NASH). 1619 20

The case of a 36-year-old male professional bodybuilder is reported. He presented to the accident and emergency department with right upper quadrant pain. This was on the background of a 15-year history of anabolic steroid and growth hormone misuse. Examination revealed mild hepatomegaly and a random blood sugar of 30.2 mmol/l. There was no evidence of ketonuria or acidosis. Biochemical evidence of hepatitis was found, and the patient was in acute renal failure. He was given a sliding scale of insulin and an intravenous infusion of crystalloid. The hepatitis and hyperglycaemia settled with conservative treatment. It is believed that this is the first reported case of frank diabetes precipitated by supraphysiological recreational growth hormone misuse.
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PMID:Strong diabetes. 1732 62

A 65-year-old Greek man with a history of diabetes mellitus and hypertension was admitted because of right upper quadrant pain, nausea and palpable right quadrant mass. On admission the patient was febrile (38.8 degrees C) with a total bilirubin level of 1.99 mg/dl (direct 0.59 mg/dl); SGOT 1.26 mg/dl; Na 135 mmol/l and K 2.9 mmol/l. The white blood count was 15200/mul with 92.2% neutrophiles. Axial sections of single slice CT imaging (section thickness 10 mm), revealed emphysematous cholecystitis with thickening of gallbladder wall and wall enhancement after iv contrast enhancement, as well as, dilatation of the gallbladder with multiple gallstones precipitate and intraluminal air. The patient underwent subtotal cholecystectomy and a cholecystostomy was placed. The culture of the bile showed positivity to toxin A of Clostridium Difficile and to Escherichia Coli. The postoperative course of the patient was uneventful.
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PMID:Emphysematous cholecystitis: a case report. 1868 28

The case of a 36-year-old male professional bodybuilder is reported. He presented to the accident and emergency department with right upper quadrant pain. This was on the background of a 15-year history of anabolic steroid and growth hormone misuse. Examination revealed mild hepatomegaly and a random blood sugar of 30.2 mmol/l. There was no evidence of ketonuria or acidosis. Biochemical evidence of hepatitis was found, and the patient was in acute renal failure. He was given a sliding scale of insulin and an intravenous infusion of crystalloid. The hepatitis and hyperglycaemia settled with conservative treatment. It is believed that this is the first reported case of frank diabetes precipitated by supraphysiological recreational growth hormone misuse.
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PMID:Strong diabetes. 2168 71

Nonalcoholic fatty liver disease (NAFLD) defines a condition of hepatic steatosis with or without hepatic injury. NAFLD is increasing in prevalence worldwide and presents a public health burden. Most patients are asymptomatic, although some present with fatigue and right upper quadrant pain. NAFLD is discovered incidentally when patients have elevated liver enzymes or fatty liver is seen on imaging modalities. Imaging studies can confirm fatty deposits in the liver, but needle biopsy is needed to determine degree of inflammation. The mainstay of treatment is weight loss and controlling diabetes and hyperlipidemia. Liver transplantation is considered when disease progresses to cirrhosis.
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PMID:Nonalcoholic Fatty Liver Disease. 2913 22

Commonly reported complications of hepatic cysts are spontaneous hemorrhage, rupture into the peritoneal cavity, infection and compression of the biliary tree however cardiac complications are not commonly reported. We are presenting a case of a large liver cyst presenting with right atrial and ventricular inflow tract impingement resulting in cardiac symptoms. A 68 year-old Hispanic female presented with one month of fatigue and shortness of breath after household work and walking less than one block, right upper quadrant pain and weight loss. She had history of multiple hepatic cysts for more than 12 years, well-controlled diabetes and hypertension. Examination of the heart revealed tachycardia with regular heart sounds. There were no murmurs. She had tenderness in her right upper quadrant on palpation and an enlarged smooth liver. Rest of physical examination was unremarkable. CT scan of the abdomen showed multiple non-enhancing liver cysts in both lobes, with the largest measuring 12 x 15 x 17 cm which was significantly increased from her baseline of 7 x 8 x 10 cm in 2003. Echocardiogram showed normal left ventricular ejection fraction, grade 1 diastolic dysfunction and a hepatic cyst impinging RA and RV inflow tract. She had successful laparoscopic enucleation of liver cyst and subsequent relief from tachycardia, fatigue and shortness of breath. In conclusion, this case illustrates that hepatic cysts may become symptomatic after remaining quiescent for an extended period. They may present with unusual symptoms and clinicians should be mindful of rare complications, such as in this case.
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PMID:Hepatic Cyst Compressing The Right Atrial and Ventricular Inflow Tract: An Uncommon Cardiac Complication. 2931 1

Haemorrhagic cholecystitis is a rare entity of acute cholecystitis that carries a high morbidity and mortality rate if management is delayed. Its clinical course can mirror that of acute cholecystitis. Characteristic findings on ultrasound or CT scan are useful clues to early diagnosis. Urgent cholecystectomy is required prior to progressing to perforation of gallbladder. Most of the literature are case reports with causes associated with anticoagulation. Herein, we described a morbidly obese patient with poorly controlled diabetes presenting with non-specific right upper quadrant pain and was subsequently diagnosed with haemorrhagic cholecystitis. A review of the literature was also performed to summarise the potential clinical presentations, distinctive imaging findings and management options available for this rare condition.
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PMID:Haemorrhagic cholecystitis: a rare entity not to be forgotten. 3024 28