Introduction the importance of recognizing congenital hyperbilirubinemia lies mainly in distinguishing it from other pregnancy 6 weeks proven estradiol 2 mg, more serious hepatobiliary diseases women's health center evergreen buy 2mg estradiol otc. Except for Crigler-Najjar syndrome women's health center at presbyterian dallas order estradiol 1 mg amex, congenital hyperbilirubinemias do not impair either the quality of life or the life expectancy of affected subjects pregnancy 3 weeks symptoms purchase estradiol 1mg visa. Persons with congenital (recessive) hyperbilirubinemia have normal standard liver tests (except of course for elevated serum bilirubin concentrations). The liver histology is also normal, (except for the black pigment accumulation in centrolobular hepatocytes Dubin-Johnson syndrome). With the exception of Gilbert’s syndrome, these syndromes are distinctly uncommon and are divided into two groups on the basis of the type of the serum hyperbilirubinemia (Table 1). Practice points: o Unconjugated hyperbilirubinemia in absence of hemolysis, is usually secondary to congenital defect in glucuronidation of bilirubin. Gilbert’s Syndrome Gilbert’s syndrome is the most common congenital hyperbilirubinemia syndrome. Approximately 9% of the general population in Western countries are homozygous for the variant promoter, and 30% are heterozygous. Other factors are probably involved in the clinical expression of the Gilbert’s phenotype since not all homozygous carriers develop hyperbilitubinemia. The syndrome manifests itself only in homozygous individuals; its inheritance is therefore consistent with an autosomal recessive trait. Gilbert’s syndrome is usually detected in adolescents and young adults, most commonly in males. Clinically, it does not cause symptoms, scleral icterus may be present slight, and fluctuating, and the physical examination is otherwise normal. Liver tests and hemogram (to exclude hemolysis) are normal except for unconjugated serum bilirubin which is elevated between 20 and 100 Mol/L. Certain conditions can increase the plasma bilirubin concentrations to higher values (usually less than 200 Mol/L): hemolysis, fasting, a normocaloric diet without lipids, intercurrent febrile illnesses, stress, physical exertion. Hepatic enzyme inducers such as phenobarbital and clofibrate can normalize plasma bilirubin concentrations within a few weeks. The long-term outcome of subjects with Gilbert’s syndrome is similar to that of the general population. In neonates, it may be associated with increased duration or severity of normal post-partum physiological jaundice. Diagnostic tests are usually not necessary but genetic testing is available in certain laboratories. The most important aspect is to recognize the syndrome distinguishing it from other causes of elevated unconjugated hyperbilirubinemia. Crigler-Najjar Syndrome the Crigler-Najjar syndrome is a rare autosomal recessive disorder. Type 1 is a serious disease characterized by unconjugated hyperbilirubinemia often greater than 400-500 Mol/L. Jaundice occurs almost immediately after birth and may lead to kernicterus with consequent neurologic damage and mental retardation. Kernicterus involves damage to the basal ganglia and cerebral cortex because unconjugated bilirubin is able to penetrate the immature blood-brain barrier of infants. Nowadays, most patients treated with phototherapy and plasmapheresis survive postpuberty without significant brain damage. Subsequently, due to thickening of the skin making phototherapy less effective, patients succumb to kernicterus later in life. Type 2 syndrome is a much more benign condition in which unconjugated hyperbilirubinemia usually does not exceed 400 mol/L. If necessary, phenobarbital or clofibrate reduces bilirubin levels by at least 25% and may improve quality of life of some individuals. An important research agenda explore gene therapy for Criggler Najjar type I syndrome. Conjugated Hyperbilirubinemia Two conditions characterized by congenital conjugated hyperbilirubinemia without cholestasis have been described, Dubin-Johnson and Rotor syndrome are inherited as autosomal recessive traits. Both are uncommon, believed to result from specific defects in the hepatobiliary excretion of bilirubin. These conditions are benign, and their accurate diagnosis provides reassurance to the patient. Plasma bilirubin may increase further in both conditions during intercurrent infection, pregnancy or use of oral contraceptives. Pruritus is absent and serum bile acid levels are normal, as are routine liver tests, except for the serum bilirubin concentration. Diagnosis is made by documenting conjugated hyperbilirubinemia (where at least 50% of the total bilirubin is the direct fraction) while other liver tests are normal. Distinction of the two syndromes is made by the characteristic urinary coproporphyrin excretion (D-J, isomer 1>80%; Rotor, isomer 1<80%). Patients have a black liver, which results from the accumulation of a melanin-like pigment in lysosomes. Visualization of the gallbladder during oral cholecystography is usually delayed or absent. Urinary excretion of total coproporphyrin is normal, whereas the proportion of isomer 1 is higher than in normal controls (>80%). Total coproprophyrin excretion is greater than normal, as in other hepatobiliary disorders, and isomer 1 makes a smaller proportion (<80%) than in Dubin-Johnson patients. Background Drugs are the second most common cause of acute liver failure, and are the predominant cause of liver injury in the Western world. Rarely do these patients have a background history of liver disease (Andrade et al. If a medication is having a toxic effect on the liver, it should be stopped quickly in the hope that the liver damage will not progress. However, despite that, many cases follow a sub-acute course with progression to liver failure. Excluding the cases of acetaminophen (45%) overdose, 15% of cases were idiosyncratic reactions to drugs. Marked elevations in transaminases reflect an hepatocellular pattern in 87% of patients, while 13% had a cholestatic pattern. Hepatocellular variety has been documented to carry worse prognosis, compared to cholestatic. There are numerous mechanisms of liver injury, but it is not always possible to determine which mechanism of injury is responsible for the hepatotoxicity (Table 5). Finally, drugs or drug metabolites may act as haptens and bind covalently to + hepatic proteins. Clinical Presentation There are a variety of presentations for the persons with drug associated hepatotoxicity, ranging from the finding of abnormal liver enzyme tests in the asymptomatic persons, to life First Principles of Gastroenterology and Hepatology A. Clinical features of acute liver failure Whole body o Systemic inflammatory response o High energy expenditure and catabolism Liver o Loss of metabolic function o Decreased gluconeogenesis leading to hypoglycemia o Decreased lactate clearance leading to lactic acidosis o Decrease ammonia clearance leading to hyperammonemia o Decreased synthetic capacity leading to coagulopathy Lungs o Acute lung injury o Adult respiratory distress syndrome Adrenal gland o Inadequate glucocorticoid production contributing to hypotension Bone marrow o Frequent suppression, especially in viral and seronegative disease Circulating leukocytes o Impaired function and immunoparesis contributing to high risk of sepsis Brain o Hepatic encephalopathy o Cerebral edema o Intracranial hypertension Heart o High output state o Frequent subclinical myocardial injury Pancreatitis o Particularly in paracetamol-related acute liver failure Kidney o Frequent dysfunction or failure Portal hypertension First Principles of Gastroenterology and Hepatology A. Shaffer 488 o Might be prominent in subacute disease and confused with chronic liver disease Permission to reprint: Bernal et al. Acetaminophen Acetaminophen is an effective over-the-counter analgesic, and is safe when taken in a daily dose that does not exceed 4 gm. For example the malnourished, alcoholic taking an acute dose of acetaminophen of >100mg/kg or a 10 to 20 gm dose over three days will develop: acute zone 3 necrosis, extending to bridging or panacinar (massive) necrosis. Liver failure may result from attempted suicide or therapeutic misadventure, as confirmed by a recent literature review (Larson et al. Fatal cases usually involve 20 gm acetaminophen (caution in the heavy alcohol abuser, where even 2 gm may be fatal). Over 20% develop severe liver injury, and of these, 20% die from the hepatotoxicity. This in turn leads to the production of reactive oxygen species, hepatocellular apoptosis, and centrilobular necrosis. Hyperacute injury to the liver occurs within 48 to 72 hours after acetaminophen ingestion. Death can occur in 4 to 8 days from cerebral edema, sepsis, liver and multi-organ failure. After 4 hours of taking an overdose, when most of the acetaminophen has been emptied from the stomach and absorbed, blood levels reflect the prognosis.
Management of common bile-duct stones and associated gallbladder stones: Surgical aspects menstruation jokes arent funny period cheap estradiol 1 mg without prescription. Wait-and-see policy or laparoscopic cholecys tectomy after endoscopic sphincterotomy for bile-duct stones: a randomised trial breast cancer 2 day atlanta discount estradiol. Randomised trial of laparoscopic exploration of common bile duct versus postoperative endoscopic retrograde cholangiography for common bile duct stones menopause uk discount estradiol 1mg. Comparison of laparoscopic common bile duct explora tion and endoscopic retrograde cholangiopancreatography plus laparoscopic cholecystec tomy for choledocholithiasis: a prospective randomized study women's health center queens ny purchase cheap estradiol. A prospective randomized trial comparing two stage versus single-stage management of patients with gallstone disease and common bile duct stones. Laparoscopic bile duct exploration: results of 160 consec utive cases with 2-year follow up. Laparoscopic endobiliary stenting: a simplified approach to the management of occult common bile duct stones. Management of common bile duct stones in a rural area of the United States: results of a survey. Routine intraoperative cholangiography during single incision laparoscopic cholecystectomy: a review of 196 consecutive patients. Intraoperative endoscopic sphincterotomy for common bile duct stones during laparoscopic cholecystectomy. Laparo-endoscopic “rendezvous”: a new technique in the choledocholithiasis treatment. Preoperative versus intraoperative endoscopic sphincterotomy in patients with gallbladder and suspected common bile duct stones: system review and meta-analysis. Laparoscopic cholecystectomy and endoscopic sphinc terotomy under a single anesthetic: a case report. Laparoscopic transgastric endoscopy after Roux-en-Y gastric bypass: case series and review of the literature. Endoscopic therapy for bile leak based on a new classification: results in 207 patients. Single incision laparoscopic cholecystectomy is associated with a higher bile duct injury rate: a review and a word of caution. Bile duct injury during laparoscopic cholecystec tomy: results of an Italian national survey on 56591 cholecystectomies. Association between cholecystectomy with vs without intraoperative cholangiography and risk of common duct injury. Intraoperative management and repair of bile duct injuries sustained during 10, 123 laparoscopic cholecystectomies in a high-volume referral center. Treatment of common bile duct injuries dur ing laparoscopic cholecystecotmy: endoscopic and surgical management. Long-term outcome in patients with benign biliary strictures treated endoscopically with multiple stents. Endotherapy of postoperative biliary stric tures with multiple stents: results after more than 10 years of follow-up. Bile duct obstruction after cholecystectomy caused by clips: undo what has been undone, then do what you normally do. Case An 84-year-old female post remote cholecystectomy, having had a myocardial infarction 2 years ago, and now on aspirin, presents to the local emergency department with a 2-day history of abdominal pain. Her initial physical examination is unremarkable except for right upper quadrant tenderness. Initial investigations reveal elevated alanine aminotransferase, aspartate aminotransferase, and total bilirubin. In this chapter, we performed a systematic review summarizing the clinical results of specific contemporary management options for the removal of large choledocholithiasis. Different techniques may be required, and even a combination thereof, to achieve favorable results. General conceptual approaches include techniques aimed at increasing the opening diameter of the bile duct into the duodenum, and methods to decrease the size of the stone (Table 16. We will also briefly discuss the specific role of biliary stenting, and alternatives to endoscopic methods, as well as specific issues related to patients exhibiting reconstructed anatomy, Mirizzi’s syndrome, impacted stones, and intrahepatic stones. Reference lists from selected articles were reviewed manually to identify additional citations. All data were abstracted and recorded in dedicated forms and reviewed by two independent individuals (J. Resolution of any discrepancies in data abstraction was achieved through consensus. Although effective, it is associated with immediate and delayed complications [3, 4]; theoretically, because it destroys the biliary sphincter permanently, it may lead to long-term complications such as pancreatico biliary reflux, and chronic inflammation of the biliary system, although the actual clinical impact of these remains poorly documented and subject to controversy. Acute pancreatitis occurred at the same frequency in both groups (7% in each group). Although they initially planned to exclude all patients with stones larger than 1cm, 3 patients with such stone sizes were included, in a total population of 237 patients. Based on this multicenter trial with the two case fatalities from pancreatitis, this approach has fallen out of favor, at least in North America. In 70 patients with a mean stone size of 8 mm, using 15-mm diameter balloons, Bang and colleagues compared a dilation time of 20 versus 60sec . The use of 60-sec dilation was associated with more cases of mild pancreatitis, although this finding was not statistically significant (11. Longer duration of dilation, 1 versus 5min, was assessed by a separate group . The 5-min dilation group was associated with less failed 254 Chapter 16 stone extraction and pancreatitis (4. Specifically focusing on large stones (12–20 mm), they found sim ilar efficacy but less complications including perforation (0% versus 2. Based on these considerations, multiple modalities are available to aid the endoscopist perform successful extraction of large bile duct stones. Methods to decrease the size of stones Lithotripsy can be performed mechanically using specialized baskets to crush the stones, or through shock wave fragmentation of bile duct stones, either extracor poreally using external generators or intracorporeally through placement of a shock wave–delivering catheter positioned under fluoroscopic guidance or direct visualization at cholangioscopy. Jakobs and colleagues randomized 34 patients to receive one of the two treatments for patients with retained stones . They randomized 30 patients in each arm, including a total of 8 with complex bile duct stones. Temporary stents using plastic, pigtail, multiple, or metallic stents have all been assessed. Biliary drain age was achieved after 1 session in 98% in the former group versus 56% in the latter. Stent placement was associated with fewer complications after 3 days compared to the conventional group (7% versus 16%), but more complications at 20 months (36% versus 14%). During a median follow-up of 38 months, stent patency was similar in both groups; two patients died of biliary-related causes. Katsinelos and colleagues studied 41 subjects with stones difficult to extract . Stone size decreased significantly during the follow-up period of 6 months from 19 to 13mm and 21 to 14mm, but there were no differences in successful stone removal rates between the two treatment arms (73. Therefore, in a selected population with high-risk, long-term stent placement can be considered. Large bile duct stones 259 Backup methods When endoscopic therapy is unsuccessful, other alternatives must be considered. A laparoendoscopic rendezvous approach has also been assessed and described in two trials, which included a total of 191 patients. Indeed, these may lead to difficult biliary cannulation when impacted in the ampulla, in which case a precut sphincterotomy may be of use. In recent years, case reports have described successful stone removal using litho tripsy in elderly patients [42, 43]. Indeed, it may be difficult to identify the afferent loop; the distance between the mouth and the biliary intestinal anastomosis is usually greater than in nonsurgically altered anatomy, such that the biliary system needs to be accessed from an intestinal retrograde approach leading to an inverted biliary anatomy. Surgically altered anatomy has also been assessed retrospectively in patients with a Roux-en-Y surgical hook-up. Kawasura and colleagues assessed a cohort of 204 patients, demonstrating intubation of the blind loop in 91% of patients using single-balloon enteroscopy, compared to 33% before its advent (P= 0. Intrahepatic stones may result from localized strictures with associated bile stasis, further complicating endoscopic treatment. Endoscopy may also play a role in assessing the malignancy potential of the strictures.
In our experience womens health 15 minute workout book buy estradiol 1 mg otc, routine antibiotic prophylaxis is unnecessary in neonates with cholestasis menstruation hinduism cheap 1 mg estradiol visa. Prophylactic antibiotics should be used to menstrual man estradiol 1 mg low price prevent endocarditis in susceptible patients in the same manner as for upper gastrointestinal endos copy pregnancy 0 negative blood type cheap 2mg estradiol visa. Special situations that require a valvular prosthesis, vascular graft material, indwelling catheters, or transplanted organ in an immunosup pressed patient need individual consideration. Antibiotics are used in the setting of high-grade biliary or pancreatic duct obstruction, biliary or pancreatic duct disruption, and encapsulated pancreatic collections (pseudo cyst or walled-off necrosis). Therapeutic maneuvers, such as placement of endoprostheses and passage of some dilators and retrieval baskets, require instruments with a larger channel. Pediatric endoscopy assistants and spe cially trained nurses can help reduce preprocedure anxiety, monitor the clinical status of the patient, and assist in holding and reassuring, administering medica tion, handling catheters, and injecting contrast material. A recovery area equipped with monitors and specialized pediatric nurses familiar with the needs of children is necessary. The principles of cannulation are those used in adult patients, with the additional limitations of space within the duodenum that depend on age. In young infants, such as those undergoing investigation for neonatal cholestasis, it is important to minimize the procedure time to avoid abdominal overdistension and respiratory compromise. In our unpublished experience with 184 neonates and young infants with neonatal cholestasis, the procedure was successful techni cally in 93% of cases. Failure was due to duodenal malrotation in two cases and inability to cannulate in six. In older children, the success rate for cannulation of the desired duct is 97–98% comparable to that achieved in adults [1–5, 11–18]. Complications the incidence of complications in pediatric patients is not well established. In neonates and young infants with neonatal cholestasis, there were no major complications in the series reported in the literature [1–5]. In our unpublished experience with 184 neonates and young infants, minor complications without clinical significance occurred in 24 patients (13%). In 17 patients, minor acute duodenal erosions were observed without clinical conse quences. Complications in children older than 1 year vary according to the system studied, biliary or pancreatic. Equivalent high rates of technical success and low rates of adverse events were found. Studies are needed to evaluate the use of prophylactic rectal indomethacin in children. Biliary findings Biliary atresia versus neonatal hepatitis the differential diagnosis of neonatal cholestasis is critical in the first 2 months of life. In approximately 30% of patients, a specific metabolic or infectious disease can be recognized (Table 23. Discriminating analysis using duodenal drainage, high-quality diagnostic ultrasound, scintigraphy imaging, and liver biopsy permitted accurate diagnosis 346 Chapter 23 Table 23. Congenital anomalies Biliary atresia versus neonatal hepatitis Alagille syndrome and paucity syndrome Congenital hepatic fibrosis Caroli’s disease and Caroli’s syndrome Biliary strictures due to cystic fibrosis Choledochal cyst Benign biliary strictures Acquired diseases Bile plug syndrome Primary sclerosing cholangitis Biliary obstruction due to parasitic infestation Choledocholithiasis Benign biliary strictures Malignant biliary strictures Common bile duct complications after liver transplantation of either biliary atresia or neonatal hepatitis in 80–90% of patients [20, 21]. When the biliary tree was partially visualized (Type 2 and Type 3), the diagnosis of biliary atresia was made and confirmed by surgery. When the biliary tree was not opacified and only the pancreatic duct was visualized (Type 1), the diagnosis of biliary atresia was suspected and exploratory laparotomy was indicated. Visualization of narrow and irregular distal common bile duct and common hepatic duct with biliary lakes (arrow) at the portahepatis. Miscellaneous genetic cholestatic diseases In Alagille syndrome, the extrahepatic ducts are normal. Congenital hepatic fibrosis is characterized by disordered terminal interlobular bile ducts, which form multiple macroscopic and microscopic cysts (Figure 23. Bile plug syndrome Bile plug syndrome represents a correctable cause of obstruction of the extrahe patic bile ducts by bile sludge in patients with a normal biliary tract. Choledochal cyst Choledochal cyst is a congenital malformation of the biliary tract characterized by saccular dilatation of the biliary tree. Choledochal cyst is primarily a disease of children and young adults, and 60% of reported cases are diagnosed before age 10 . Pathogenesis of choledochal cyst Many theories have been proposed to explain the development of choledochal cysts. The majority of patients with choledochal cysts have an anomalous pancreaticobili ary union [25–27] located outside the duodenal wall (Figure 23. According to this theory, there is reflux of pancreatic juice upward into the biliary system that can produce damage to the common duct lining, resulting in saccular dilatation of the duct . The maximum normal length of the common channel in neonates and infants younger than 1 year is 3mm. It increases with age to a maximum of 5mm in children and adolescents between 13 and 15 years of age . Classification of anomalous ductal union There are three types of anomalous ductal union . If it appears that the pancreatic duct is joining the common bile duct, it is denoted as the P–B type. If the common bile duct appears to join the main pancreatic duct, it is denoted as the B–P type, and if there is only a long common channel, it is denoted as the long Y type (Figure 23. The anomalous pancreaticobiliary union is located outside the duodenal wall and is not under the influence of the sphincter of Oddi mechanism. Classification of choledochal cysts the anatomical classification by Todani et al. Choledochocele Although classified as one of the forms of choledochal cysts, choledochocele is probably not related. The presence of a distal bile duct stricture at its point of connection with the pancreatic duct is frequently observed (Figure 23. Primary cystolithiasis occurs in 8% of patients and is usually multiple (Figure 23. Patients with long common channel with no cystic dilation in the biliary tree have an increased risk of gallbladder cancer and may develop gallbladder cancer at a younger age . Treatment of choledochal cysts the anomalous anatomical configuration of the pancreaticobiliary ductal system observed in most patients with choledochal cysts has certain technical implications with regard to management. In selected cases, with fusiform bile duct dilatation and widely dilated common channel, endoscopic sphincterotomy has been attempted, with encouraging results . Fusiform choledochal dilatation and carcinoma Fusiform choledochal dilatation, as opposed to cystic dilatation, has been observed to be more commonly associated with low-grade, short strictures located at or distal to the pancreaticobiliary junction. The association with inflammatory bowel dis ease is relatively uncommon (14%), suggesting that genetic and immunological features are the most important factors . The cholangiogram will show pruning of the peripheral biliary tree and areas of stenosis and ectasia. Patients with major ductal strictures are candidates for endoscopic treatment with sphincterotomy and balloon dilatation to relieve the obstruction in order to delay the progression to cirrhosis . Severe narrowing of both right and left hepatic ducts without opacification of intrahepatic ducts. Cholangiogram obtained immediately after dilatation shows visualization of irregular areas of stenosis and ectasia of intrahepatic ducts. The authors developed a tapered hydrophilic balloon to dilate hepatic duct strictures and to avoid small intrahepatic duct rupture (Figure 23. Choledocholithiasis Choledocholithiasis occurs rarely in both infants and children. Conditions associ ated with the presence of stones include biliary tract malformations such as cho ledochal cyst, chronic liver disease, hemolysis, infection, prematurity, total parenteral nutrition, and the use of certain medications. The diagnostic approach is more difficult, and identification of the cause of obstruction by ultrasonography is often impossible. Gallstones in children are usually black pigment stones consisting of calcium carbonate, which is rarely found in adults . Sphincterotomy with common bile duct stone removal has been successfully performed in young infants , children, and adolescents [2, 43–45]. Endoscopic papillary balloon dilatation with stone extraction is an alternative technique for stone removal. Most infants with asymptomatic gallstones and no factors that would make them susceptible to stone formation can be managed conservatively .
Signs and symptoms: • Fatigue menopause 60 years discount estradiol 2mg with visa, weakness pregnancy upper back pain order 1 mg estradiol with amex, lethargy pregnancy calendar due date cheap 1 mg estradiol visa, slow movement breast cancer oakley sunglasses purchase generic estradiol, cold intolerance • Slight to moderate weight gain, but appetite tends to be diminished • Carpal tunnel syndrome, edema of the face and extremities, • Hearing loss, hoarseness of the voice • Dry skin, hair loss, sparse eyebrows with loss of the lateral half 465 Internal Medicine • Pericardial effusion and ascites occasionally occur • Constipation • Menorrhagia, • Memory impairment • Psychosis may develop with long-standing hypothyroidism and may be precipitated by thyroid hormone replacement Physical examination • Thickened, puffy features is due to accumulation of mucinous mucoplysaccharide-rich material in tissues. This is known and myxedema • Yellowish dry skin • Nonpitting edema • Hypothermia • Bradycardia • A delay in return phase of Achilles and other deep tendon reflexes is a specific finding. Cretinism: is sever hypothyroidism beginning in infancy infants may have • Hypotonia, umbilical hernia, • Delayed mental and physical development, and mental retardation may result if hypothyroidism goes untreated in the first few years of life. Initiation of treatment: Slow initiation: • Patients with severe hypothyroidism, older patients and patients with cardiovascular disease, may have an increased sensitivity to thyroid hormone, and are at risk of acute cardiovascular and other complications, if hypothyroidism is corrected too quickly. Rapid initiation • Younger patients and patients with less sever hypothyroidism, may be started on slightly higher dose (50 g of L-thyroxin) and advanced to a full replacement dose more quickly. Myxedema Coma Definition: Myxedema coma results from severe chronic hypothyroidism, which is left untreated, and is life threatening clinical condition. This serious condition may occur gradually (over years) or more acutely in response to a precipitating factor such as exposure to cold, infection, hypoglycemia, respiratory depressants, allergic reactions, or other metabolic stress. Ancillary treatment: • Temporary use of glucocorticosteriods • Respiratory support • Hypothermia and heat loss should be avoided D. Thyroid enlargement Goiter • A goiter is a simple enlargement of the thyroid gland. Signs and symptoms: Symptoms: • Thyromegaly, occasionally with rapid enlargement and tenderness secondary to haemorrhage into a cyst. Occasionally it may be difficult to distinguish from the typically lobulated, irregular Hashimoto’s gland. Diagnosis: • Thyroid function tests: Performed to rule out hypo or hyperthyroidism • Malignant transformation is rare, but should be considered if the gland is enlarging rapidly or hoarseness develops. Treatment: the main indications for treatment are compression of the trachea or esophagus and venous outflow obstruction. For toxic multinodular goiter, options are • Antithyroid agent • Surgery • Radioiodine, and more recently • Percutaneous injection of ethanol in to the toxic nodule Solitary Nodules • They are usually benign. Diagnosis: • History and • Radio iodine thyroid scan should be done on every patient with a solitary nodule. Hot nodules that take up the radioisotope are generally benign but fine-needle aspiration of a solitary nodule is prudent. Treatment: • Is indicated if signs of compression of trachea, esophagus, significant growth, and recurrence of a cystic nodule after aspiration. Subacute Thyroiditis/granulomatous thyroiditis Etiology: the cause is generally considered as viral. Clinical features: • Early symptoms: prodromal phase of malaise, upper respiratory symptoms, and fever that lasts 1-2 weeks. Then the Thyroid gland becomes enlarged, firm and tender, with pain radiating to the ears, neck, or arms. The gland usually return to normal size, if enlargement persists, chronic thyroiditis should be suspected. Diagnosis; • Acutely swollen, tender an painful thyroid gland associated with symptoms of hyperthyroidism • Radioactive uptake: low radioactive iodine uptake in the face of high serum T3 and T4 level. Chronic Thyroiditis (Hashimoto thyroiditis) Etiology: it is an autoimmune disorder that mainly affects women. Clinical features • Thyroid gland enlargement: is the main clinical manifestation, is the result of autoimmune damage that leads to lymphocytic infiltration, fibrosis and weakens ability of the thyroid to produce hormone. Types/ Classification: • Papillary carcinoma: which accounts for 60 % of all thyroid cancer o Affects younger age group – 50 % of patients are younger than 40 years o Papillary Ca metastasize through the lymphatic system • Follicular carcinoma; comprises 25 % of all thyroid cancer o Histologicaly resembles normal thyroid tissue, o Follicular Ca metastasizes hematogenously • Medullary carcinoma: which accounts for 5 % of all thyroid cancers. Diseases of the adrenal gland Learning objectives: at the end of this lesson the student will be able to: 1. Identify the clinical manifestation of diseases of the adrenal gland, with special emphasis on Cushing’s syndrome and Addison’s diseases. Disease of the adrenal cortex a) Resulting from excess production of hormones • Cushing’s syndrome: excess cortisol production • Primary hyperaldosteronism: excess production of aldosteron b) Inadequate production; • Addison’s diseases: inadequate production of cortisol and aldosteron 2. Disease of the adrenal medulla • Pheochromocytoma: excess production of catecholamine Cushing’s Syndrome (Hypercortisolism) Cushing’s syndrome: is caused by excessive concentration of cortisol or other glucocorticoid hormones in the circulation Etiology: a) Bilateral adrenal hyperplasia (Cushing’s diseases) is the commonest cause of Cushing’s syndrome. Pituitary tumors large enough to be seen by skull x-ray, are present in more than 10 % of these patients, and smaller basophilic adenomas are found in more than 50 % of patients. It is an expected complication in patients receiving long term glucocorticiod treatment for asthma, arthritis, and other conditions. Clinical features • Central obesity is caused by the effect of excess cortisol on fat distribution. The “moon face”, “buffalo hump” (cervical fat pad) and supraclavicular fat pads contribute to the Cushingiod appearance • Hypertension: result from the vascular effects of cortisol and sodium retention • Decreased glucose tolerance: is common, 20 % of patients have overt diabetes. This is a result of hepatic gluconeogenesis, and decreased peripheral glucose utilization. Because this test is sensitive, the diagnosis of Cushing’s syndrome need not be considered further in these cases. Serum cortisol level: in normal in individuals is highest in early morning and decreases throughout the day, reaching a low point at about midnight. Although the morning level may be increased in patients with Cushing’s syndrome, a loss of the normal diurnal variation and an increase in the evening level are more consistent findings. The 24 hrs urinary free cortisol excretion rate: is increased in most patients with Cushing’s syndrome 6. Other tests: lukocytosis, with relatively low percentage of lymphocytes and eosinophils 7. Skull x-ray: enlargement of sella turcica in 10 % of patients with Cushing’s syndrome who have macroadenoma. Adrenal adenoma: complete surgical resection of the adenoma cures the disease, but patients may need cortisol replacement post operatively for several months 2. Pituitary radiation: is effective in children but it cures fewer than 1/3 of adult patients b. Disadvantages • Patients will develop Addison’s disease and need lifelong Cortisol replacement • Nelson’s syndrome: in which pituitary adenomas undergo rapid growth, perhaps because it is no longer inhibited by above normal level of cortisol. Hyperaldosteronism Aldosteronism: is a syndrome associated with hypersecretion of the mineralocorticoid, aldosterone. Primary aldosteronism: the cause of excess aldosterone production resides with in the adrenal gland • Aldosterone producing adrenal adenoma (Conn’s syndrome): in most cases, unilateral small adenoma which can occur on either side • Adrenal carcinoma: rare cause of aldosteronism • Bilateral cortical nodular hyperplasia /idiopathic hyperaldosteronism 2. Secondary aldosteronism: the stimulus for excess aldosterone production is outside the adrenal gland. Signs and symptoms: • Moat patients have diastolic hypertension resulting from sodium retention. Patients may complain headache and symptoms of other organ damage • Hypokalemia and associated symptoms: muscle weakness and fatigue. While raised aldosteron level with reduced plasma renin activity suggests primary aldosteronism. Surgery: removal of solitary adenoma results cure of hypertension in about 60 % of cases and improvement in another 25 %. Adrenalectomy is done after 4 week treatment with spironolactone (in case of adenoma, hyperplasia) In contrast only 20%-50 % of patients with bilateral hyperplasia are improved with surgery, even if bilateral adrenalectomy is performed. Medical Therapy: Spironolactone inhibits the effects of aldosteron on renal tubule. In idiopathic form: Spironolactone (50-100 mg/d), possibly combined with potassium sparing diuretics correct the hypokalemia and with anti-hypertensive medication, high blood pressure can be controlled. Anterior pituitary diseases may result from: i) Insufficient production of pituitary hormones: hypopituitarism ii) Excess production of pituitary hormones: a. Posterior Pituitary diseases I) Hypopituitarism (Insufficient production of anterior pituitary hormones) • Hyposecretion may be generalized (hypopituitarism) or caused by the selective loss of one or more pituitary hormones. Generalized hypopituitarism Definition: Endocrine deficiency syndromes due to partial or complete loss of anterior lobe pituitary function. Infarction of ischemic necrosis of the pituitary • Shock, especially post partum (Sheehan’s syndrome) or in Debates mellitus or Sickle cell anemia • Vascular thrombosis or aneurysm of the anterior cerebral artery • Hemorrhagic infarction: pituitary apoplexy 3. Inflammatory /infectious process: meningitis (tuberculus), pituitary abscess 4.
Nevertheless maria pregnancy purchase estradiol 2 mg on-line, after 6 months elderly women's health issues best 1mg estradiol, a high percentage of stent occlusion was noted because of epithelial hyperplasia menopause uk generic estradiol 1mg on-line. Simple small stones can be retrieved by endoscopic techniques alone using either stone extraction balloons or baskets (Figure 21 breast cancer in situ discount estradiol american express. Factors favoring endoscopic removal include the presence of stones in the head and/or body of the pancreas, the absence of a downstream stricture, stones measuring 10 mm or less, the presence of three stones or less, and the absence of impacted stones . In contrast to bile duct stones, pancreatic stones will often require lithotripsy for successful removal. Although mechanical lithotripsy is an option, capturing an impacted stone to use this technique is rarely accomplished. Visually directed electrohydraulic and laser lithotripsy using a pancreatoscope has been more commonly utilized with the advent of single-operator pancreatoscopes [2, 17, 34–38]. For the 272 patients followed up for more than 5 years, 76% had complete duct clearance. Key poInts • Endoscopic pseudocyst drainage techniques have similar efficacy as open surgery at a lower cost and shorter hospital stay. While surgical drainage has been the traditional treat ment for pancreatic pseudocysts, endotherapy provides a less invasive approach. A recent randomized trial in 40 pseudocyst patients showed equal efficacy of endoscopic and open surgical cystogastrostomy . After a follow-up period of 24 months, there was no pseudocyst recurrence in patients undergoing endo scopic drainage and one in the surgically treated group. However, endoscopic treatment was associated with a shorter hospital stay, better physical and mental health of patients, and lower costs. The route of endoscopic drainage for pseudocysts involves transpapillary, transmural, or combined transpapillary and transmural methods. It is critical to treat associated ductal disease to achieve the best outcome from therapy. Persistent cyst resolution was seen in 61–90% during a follow-up time of 5–37 months. However, the transpapillary technique was performed in patients with smaller pseudocysts than transmural drainage. Since it is less invasive, endotherapy may still be a first-line treatment option in a properly selected group. Endoscopic versus surgical drainage of the pancreatic duct in chronic pancreatitis. Long-term outcomes of endoscopic vs surgical drain age of the pancreatic duct in patients with chronic pancreatitis. Stenting in severe chronic pancreatitis: results of medium-term follow-up in seventy-six patients. Endoscopic pancreatic stent drainage in chronic pan creatitis and a dominant stricture: long-term results. Endoscopic treatment of chronic pancreatitis: a multicenter study of 1000 patients with long-term follow-up. Endoscopic stent therapy for patients with chronic pancreatitis: results from a prospective follow-up study. Endoscopic stent therapy in patients with chronic pancreatitis: a 5-year follow-up study. Multiple stenting of refractory pancreatic duct strictures in severe chronic pancreatitis: long-term results. Temporary placement of a fully covered self-expandable metal stent in the pancreatic duct for management of symptomatic refractory chronic pancreatitis: preliminary data (with videos). Modified fully covered self-expandable metal stents with antimigration features for benign pancreatic-duct strictures in advanced chronic pan creatitis, with a focus on the safety profile and reducing migration. Fully covered self-expanding metal stents for refractory pancreatic duct strictures in chronic pancreatitis. Effect of pancreatic duct stent diameter on hospitaliza tion in chronic pancreatitis: does size matter Pancreatic ductal stones: frequency of successful endoscopic removal and improvement in symptoms. Endoscopic treatment of pancreatic duct stones using a 10F pancreatoscope and electrohydraulic lithotripsy. Pilot study of pancreatoscopic lithotripsy using a 5-fr instrument: selected patients may benefit. Clinical outcomes in patients who undergo extra corporeal shock wave lithotripsy for chronic calcific pancreatitis. Single-operator cholangioscopy-guided laser lithotripsy in patients with difficult biliary and pancreatic ductal stones (with videos). Update on endoscopic management of main pancreatic duct stones in chronic calcific pancreatitis. Long-term clinical outcomes of extracorporeal shockwave lithotripsy in painful chronic calcific pancreatitis. Endoscopic pancreatic drainage in chronic pancreatitis associated with ductal stones: long-term results. Long term follow up of patients with chronic pan creatitis and pancreatic stones treated with extracorporeal shock wave lithotripsy. Use of intravenous secretin during extracorporeal shock wave lithotripsy to facilitate endoscopic clearance of pancreatic duct stones. Prospective evaluation of morphology, function, and quality of life after extracorporeal shockwave lithotripsy and endoscopic treatment of chronic cal cific pancreatitis. Pancreatic duct stones in chronic pancreatitis: criteria for treatment intensity and success. Extracorporeal shock wave lithotripsy and endo therapy for pancreatic calculi—a large single center experience. Long-term outcomes associated with pancreatic extracorporeal shock wave lithotripsy for chronic calcific pancreatitis. Treatment of pancreatic stones with extracorporeal shock wave lithotripsy: results of a multicenter survey. Treatment for painful calcified chronic pancreatitis: extracorporeal shock wave lithotripsy versus endoscopic treatment: a ran domised controlled trial. Equal efficacy of endoscopic and surgical cystogas trostomy for pancreatic pseudocyst drainage in a randomized trial. Endoscopic transpapillary therapy for disrupted pancreatic duct and peripancreatic fluid collections. Treatment of pancreatic pseudocysts with ductal communication by transpapillary pancreatic duct endoprosthesis. Outcome differences after endoscopic drainage of pancreatic necrosis, acute pancreatic pseudocysts, and chronic pancreatic pseudocysts. Endoscopic ultrasound drainage of pancreatic pseudocyst: a prospective comparison with conventional endoscopic drainage. Endoscopic ultrasound-guided versus conventional trans mural drainage for pancreatic pseudocysts: a prospective randomized trial. Endoscopic drainage of pancreatic-fluid collections in 116 patients: a comparison of etiologies, drainage techniques, and outcomes. Endoscopic ultrasound guided versus conventional transmural techniques have comparable treatment outcomes in draining pancreatic pseudocysts. Both forms can have the same etiologies but usually result in divergent clinical out comes. Most have a prolonged course of sterile necrosis while others develop delayed infection. Gallstone pancreatitis is suspected based upon the presence of cholelithiasis and elevated serum transaminases in the absence of other risk factors for pancreatitis (such as heavy alcohol abuse, hypertriglyceridemia, and drugs). The vast majority of patients will have already passed stones and the mainstay of therapy is laparoscopic cholecystectomy. This makes identification of the papilla and/ or cannulation difficult, if not impossible.
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