Dan Engelhard, M.D.

• Associate Professor
• Department of Pediatrics
• The Hebrew University Hadassah Medical School
• Chief
• Department of Pediatrics
• Hadassah University Hospital
• Jerusalem, Israel

Red cell production can be increased more than tenfold in the adult by increasing the cellularity of existing haemopoietic marrow gastritis diet ���� aciphex 20 mg overnight delivery, as well as by expansion of haemopoietic marrow into the long bones gastritis chronic diarrhea buy aciphex cheap. In the newborn gastritis blog purchase aciphex american express, and during infancy gastritis diet zen purchase online aciphex, marrow expansion depends on expanding the medullary cavity of bones gastritis symptoms during pregnancy purchase aciphex 10mg free shipping, leading to thinning of cortical bone gastritis green stool purchase aciphex 10mg with mastercard. These bony changes are most extreme in the -thalassaemia syndromes, but some skeletal changes, usually some bossing of the frontal bones, may be seen in more extreme hereditary haemolytic anaemias of other causes. Increased red cell destruction is often completely matched by increased production, resulting in compensated haemolysis. When the rate of haemolysis exceeds the maximum erythropoietic capacity of the bone marrow, or when the latter is limited. General features of haemolysis the clinical and laboratory aspects of haemolysis depend on the consequences of increased red cell destruction and production as well as the main process by which destruction takes place. Increased red cell destruction leads to an increase in unconjugated bilirubin from increased haemoglobin turnover. Unconjugated bilirubin does not appear in the urine, although there will be an increase in urinary urobilinogen. The bilirubin level is usually not more than two to three times normal because the normal liver is able to increase excretion to compensate for at least some of the increased production. Jaundice is usually mild in hereditary haemolytic anaemias although there are important exceptions. In the neonate, particularly premature infants, liver function is not fully developed and more severe jaundice requiring urgent therapeutic intervention may occur. A rare but potentially confusing problem is the coinheritance of Gilbert syndrome, which comprises a group of congenital liver enzyme deficiencies that impair bilirubin conjugation. On its own, Gilbert syndrome does not produce clinical jaundice except when there is inadequate calorie intake, but in conjunction with haemolytic anaemia the hyperbilirubinaemia may be considerable. The increased bilirubin of haemolysis does increase the risk of gallstones and cholecystitis, which in turn may lead to an increase in serum bilirubin. In the degradation of haemoglobin, the molecule is broken down to two subunits, which are bound to haptoglobin, the complex being rapidly internalized in the hepatocyte after binding to the haptoglobin complex receptor. In the presence of haemolysis, serum haptoglobin levels are greatly reduced or absent. However, haptoglobin is an acute-phase protein and levels will increase in the presence of inflammation. Chronic haemolytic anaemia may increase the iron content of the body through increased iron absorption as a result of anaemia coupled to the retention of the haem iron following binding to haptoglobin and haemopexin. In rare cases of inherited haemolytic anaemia, this iron overload may be sufficient to produce clinically important effects, particularly if there is coinheritance of a haemochromatosis gene. In most haemolytic anaemias, owing to membrane defects, the destruction of red cells takes place extravascularly in the reticuloendothelial system, particularly in the spleen, and the iron is retained. When destruction is intravascular, free haemoglobin will be released into the plasma, producing haemoglobinaemia and methaemalbuminaemia, and will pass through the glomerulus to produce haemoglobinuria and haemosiderinuria. Increased red cell production leads to expansion of the red cell precursor compartment of the bone marrow, as described above. There are also changes in the structure of the marrow as a consequence of the chronic anaemia, which allows the early release of reticulocytes and, in more marked cases of haemolytic anaemia, nucleated red cells and even myelocytes. In the peripheral blood, the polychromasia and macrocytosis of reticulocytosis are the result of this increased throughput and release. The increased cell production requires an increased supply of folate which, at least theoretically, can produce folate deficiency unless supplements are given. It is usual to give folic acid (400 g daily or 5 mg once weekly) to people with chronic haemolytic anaemia. Increased red cell destruction Unconjugated hyperbilirubinaemia Mild jaundice Increased risk of gallstones Increased urinary and faecal urobilinogen Decreased serum haptoglobin and haemopexin Extravascular changes Increased iron stores Splenomegaly Intravascular changes Haemoglobinaemia and haemoglobinuria Haemosiderinuria Methaemalbuminaemia Decreased iron stores Increased red cell production Marrow expansion: bone changes Increased erythropoiesis: myeloid/erythroid ratio Reticulocytosis: polychromasia Increased folate requirements: macrocytosis by appropriate metabolic machinery and structural organization of the proteins. Accordingly, inherited haemolytic disorders can be classified into three major groups: (i) genetic disorders of haemoglobin (see Chapter 6); (ii) abnormal membrane (including the cytoskeleton) and (iii) abnormal metabolism (enzymopathies). Classification Because of the unique structural and functional specialization of the mature red cell, the impact on it of a wide range of exogenous or endogenous changes is relatively uniform: the cell will be destroyed prematurely. According to the site of the primary change, haemolytic disorders have been traditionally classified as being due either to intracorpuscular or to extracorpuscular causes. According to the nature of the primary change, haemolytic disorders have also been classified as inherited or acquired. These two classifications correlate almost completely with each other, in that extracorpuscular causes are usually acquired, whereas intracorpuscular causes are usually inherited. One notable exception is paroxysmal nocturnal haemoglobinuria, a disease in which an intracorpuscular defect is acquired as a result of a somatic mutation (see Chapter 9). Although in every cell all molecules and organelles are naturally interdependent, it is convenient to consider the red cell as a conveyance for a large amount of haemoglobin contained in a plasma membrane, the stability of which is maintained Red cell membrane disorders the red cell membrane the red cell membrane, like all other cell membranes, consists of a lipid bilayer that is stabilized and given specific properties by the proteins, glycolipids and other specialized molecules and structures with which it is associated. The lipid bilayer consists of approximately equal molar quantities of phospholipids and cholesterol molecules. The charged phosphatidyl groups of the phospholipids are hydrophilic and form the outer and inner surfaces of the bilayer. Maintenance of the asymmetry and the proper function of the membrane requires energy. The acyl chains of the diacylphosphatidylglycerides are hydrophobic non-polar domains and they form hydrophobic bonds with the acyl groups of the opposite layer. Cholesterol is present in roughly equimolar amounts and determines the fluidity of the membrane. The normal biconcave shape and function of the red cell membrane are determined by the membrane proteins and their interactions with the lipid bilayer and with each other. The integral proteins have strong hydrophobic domains that associate with the hydrophobic part of the bilayer. Many of these integral proteins span the membrane and provide channels between the plasma and cytosolic compartments. The cytosolic inner domains of these proteins interact with each other and with the second main group, the proteins of the membrane skeleton. Genetic abnormalities that produce spherocytosis mainly have mutations affecting the vertical connections. Mutations of the horizontal system usually produce elliptocytosis or more bizarre-shaped changes. In addition to the compartments mentioned so far, there are numerous surface proteins that provide the main interface with the plasma, including the blood group systems and other receptors. Many of these molecules are heavily glycosylated, as are the integral proteins, the glycophorins; sialic acid, which comprises the main side-chain of the glycophorins, contributes the most to the negative surface change of the erythrocyte. The integral proteins and vertical interaction the two major integral proteins that span the lipid bilayer are band 3 (the anion channel protein) and glycophorin C. The main protein of the membrane skeleton is spectrin, consisting of two subunits, and, which associate side by side to produce a heterodimer. The tail end of the dimer makes contact with a short actin filament composed of 14 monomers; the interaction between spectrin and actin is stabilized by protein 4. Binding of spectrin dimers to actin filaments produces the more or less hexagonal network of spectrin tetramers on the inner surface of the membrane associated with the lipid bilayer. Spectrin qualitative defects that affect these horizontal interactions tend to induce a loss of structural stability of the membrane and elliptocytosis. The outer, plasma, layer contains mostly neutral lipids, sphingomyelin and phosphatidylcholine (lecithin). The inner, cytoplasmic, layer contains mostly acidic groups, phosphatidylserine, phosphatidylethanolamine and phosphatidylinositol. The membrane is a composite structure in which the lipid bilayer is linked to the spectrin-based membrane skeleton. The linking of the lipid bilayer to the membrane skeleton is mediated by band 3-ankyrin-protein 4. Many of the conditions are inherited as autosomal dominant disorders, homozygosity for major defects mainly being lethal. Severe, bizarre or unexpected red cell morphology is often produced by double heterozygosity or inheritance of more than one defect of the membrane proteins. Mutations affecting the red cell membrane are many and heterogeneous, but the effect on the phenotype can be classified in five main categories: (i) hereditary spherocytosis; (ii) hereditary elliptocytosis and hereditary pyropoikilocytosis (severe form of elliptocytosis); (iii) Southeast Asian ovalocytosis; (iv) hereditary acanthocytosis and (v) hereditary stomatocytosis. The usual clinical picture is of mild to moderate haemolytic anaemia, but varies from severe neonatal haemolysis with kernicterus (rare) to clinically silent and asymptomatic (usual) haemolysis. Autosomal recessive inheritance occurs in a few mutations, often producing severe haemolysis. The occurrence of clinically silent cases probably means that the overall prevalence is likely higher. The functions of the spleen become mature only after birth, so severe anaemia in utero is rare. Erythropoiesis is highly active before birth, but enters a phase of reduced activity in the neonatal period. The diagnosis is usually made on the basis of red cell morphology, backed up where possible with a family history. In normal red cells with the biconcave disc shape, 50% lysis occurs when the saline solution reaches about 0. An alternate to the osmotic fragility test is to mea100 90 80 70 Haemolysis (%) 60 50 40 30 20 10 0 0. Clinical course and complications In most kindreds, the course of the disorder is similar in affected members although, as with most inherited defects, there is some variable penetrance and it is not rare to find a very mildly affected parent with more severely affected offspring. As with all congenital haemolytic anaemias, the anaemia may be aggravated by environmental factors. This may be consequent on an increase in the red cell destruction or a decrease in production. Primary infection with parvovirus 19 produces a specific and marked inhibition of erythropoiesis, often characterized as an aplastic crisis. In normal individuals with a red cell lifespan of 120 days, such an inhibition produces no clinical effect. The diagnosis is made by finding absent parvovirus antibodies with subsequent appearance of IgM antibodies. Malnutrition may increase anaemia because of folate deficiency, but also from increased jaundice through the effect of low-calorie input on unconjugated bilirubin levels in the blood. Osmotic fragility is increased in the microspherocytes (right shift), but there is also a small population of resistant cells due to increased reticulocytes (a). After splenectomy, the microspherocytes remain, but the proportion of reticulocytes is reduced to normal values and the resistant cells are not seen (b). Recurrent cholecystitis or biliary colic may require cholecystectomy accompanied by splenectomy (see below). Management Patients with well-compensated haemolysis and no transfusion requirements need no treatment other than reassurance and folic acid supplements. For people with a well-balanced and adequate diet, folic acid supplements are probably unnecessary, but custom dictates the practice should be continued. Radiolucent gallstones, if detected by chance on ultrasound, are common and need no treatment unless complications arise. Gallstones without recurrent inflammation are not a risk factor for carcinoma of the gallbladder. Recurrent cholecystitis or obstruction would be an indication for cholecystectomy, which would also be an indication for splenectomy. However, splenectomy carries short- and long-term risks that must be weighed against the benefits in any individual patient. After splenectomy, the blood film continues to show spherocytosis together with common changes associated with splenectomy. Risks of splenectomy (see also Chapter 17) the immediate risks associated with splenectomy include those of any abdominal operation together with an increased risk of thrombosis, associated with a marked rise in platelet count that occurs promptly after splenectomy. In conditions where haemolysis persists, the platelet count remains elevated, sometimes markedly, and the increased risk of thrombosis continues. The spleen plays an important role in filtering and phagocytozing bacteria, and removing parasitized erythrocytes from the blood. The spleen is the major source for mounting the rapid, specific IgM response to organisms that enter through the gut. The main organisms of 120 this class are the encapsulated organisms, Streptococcus pneumoniae, Haemophilus influenzae type B and Neisseria meningitidis. Lack of a spleen greatly increases the virulence of the infection, with progression from the first feeling of fever and non-specific flu-like symptoms to irreversible endotoxic shock occurring in a matter of hours. Patients may present with purpura, evidence of disseminated intravascular coagulation, multiorgan failure, hypotension and peripheral limb ischaemia. It is this speed of progression that makes the prophylaxis of this fortunately uncommon complication so important. Prophylaxis depends on education and awareness for the patient, specific measures to reduce the risk from particular organisms and the provision of information concerning the splenectomy for healthcare workers (Table 8. It is on this evidence that such antibiotic prophylaxis (or erythromycin 250 mg b. The risk is greatest in the first two years after splenectomy, but continues lifelong. Children under the age of 5 years are particularly susceptible and splenectomy should be avoided in this group if at all possible.

The features are due to gross distension of the colon with gas shadows and depicted by the following signs: a gastritis pain treatment buy online aciphex. The dilated and distended colon appears twisted like a rubber tube with a cut off distally gastritis tips purchase aciphex amex. The two outer lines indicate the outer margins and the intervening line is formed by two inner wall of gut gastritis diet alkaline buy aciphex 10mg. Water-soluble or barium contrast stops at the site of obstruction and tapers to a point like the beak of a bird gastritis and gerd quality 10 mg aciphex. Volvulus is defined as the twisting of a loop of bowel around its mesenteric axis gastritis symptoms treatment diet discount 10mg aciphex with amex, which results in a combination of bowel obstruction together with ischemia due to occlusion of the main vessels at the base of the involved mesentery chronic atrophic gastritis definition generic aciphex 20 mg line. It is a type of closed loop obstruction and may result in strangulation and perforation. Sometimes a loop of ileum gets twisted with volvulus of sigmoid colon and there may be gangrene in either ileum, sigmoid colon or both. The patient is usually an elderly male complaining of sudden abdominal pain with distension. History of similar episode of pain and and upper part of pelvis with features of sigmoid volvulus abdominal distension earlier. About 90 percent of kidney stones are radiopaque but only 10 percent of gallstones are radiopaque. This is plain X-ray of abdomen and pelvis showing a radiopaque shadow in the pelvis, possibly a vesical calculus. This type of calculus originates in the kidney and then passes into the bladder where it enlarges. These stones are formed in infected urine and more usually de novo in the bladder. Chemically this type of calculus is made up of triple phosphate (ammonium, magnesium and calcium phosphate). Silent type-Here vesical calculus lies in a postprostatic pouch or diverticulum of bladder and remains asymptomatic for a long time. These stones are usually discovered during cystoscopy or plain X-ray abdomen done for some other purpose. Masked type-In this type the patient presents with symptoms of cystitis and the stone is masked. Thus a patient presenting frequently with symptoms of cystitis should be thoroughly investigated to exclude vesical calculus. Chest radiographs are taken in which the different routes of spread to lungs are phase of respiration Lymphatic spread-The tumor cells should be taken on full inspiration with reach the lungs through the thoracic the patient in erect position. The diagnosis of pneumothorax depends on recognizing the line of the pleura separated by air from the chest wall, mediastinum or diaphragm with no vessels beyond this line. With tension pneumothorax there is mediastinal shift and the hemidiaphragm is often flattened. Majority of pneumothoraces occur in young patients with no obvious lung disease but having small blebs or bullae at the periphery of their lungs which burst. This is a contrast film showing barium swallow X-ray of esophagus with smooth pencil-shaped narrowing at the lower end of esophagus and dilatation proximally. Achalasia is a neuromuscular abnormality resulting in failure of relaxation at the cardiac sphincter which is shown as a smooth tapered narrowing at barium swallow examination. This is a condition of incoordinate contraction of esophagus associated with high intraesophageal pressure (more than 400 mm Hg due to marked hypertrophy of circular muscle of the esophagus. This is a contrast film showing barium swallow X-ray of esophagus with irregular narrowing at the lower end. What is the palliative treatment for dysphagia in advanced carcinoma of esophagus Supine, ach and duodenum showing a benign oblique and lateral views are taken to ulcer crater in the lesser curvature of show all parts of the stomach. The ulcer crater projects beyond the Mucosal folds do not reach upto the base lumen of the stomach. This is a contrast X-ray of barium meal follow through examination showing the appendix with multiple filling defects in its lumen. This is a barium meal X-ray of stomach and duodenum showing a hugely dilated stomach which contains residual fluid and food materials. This is contrast X-ray of Barium enema showing a filling defect at the junction of right colic flexure and the ascending colon. It is the X-ray study of lower intestinal tract following administration of barium, a radiopaque medium. Osmotic purgatives like lactulose for 2 consecutive nights before the date of examination. Connections are made to the Barium reservoir and to the hand pump for injecting the air. Air is gently pumped into the bowel forcing the column of barium towards the cecum and producing the double contrast effect. Various films are taken of the entire colon and rectum and the spot films if necessary depending on the pathology. This is done in children as mucosal pattern demonstration is not necessary in them and for reducing the intussusception. The liver is grossly damaged and fails to showing multiple filling defects within the excrete the dye into the bile. Patient is suffering from jaundice (so, Iopanoic acid (Telepaque) between 8 to liver can excrete the dye into the bile). The air bubble shadow changes its position; with change of position of the patient on the table. A filling defect due to a calculus changes direction opposite to the air bubble with change of posture. If the stone is impacted there will be no change of position of the filling defect with change of posture. Estimation of blood urea and creatinine because passage of contrast medium requires efficacy of glomerular filtration. Oral purgative and antiflatulent tablet are taken the night before the procedure to evacuate bowel gas for better visualization. Hydronephrosis-Clubbing of calices in early stage and widely dilated pelvicalyceal system in the advanced stage. Postoperatively this is done before removal of the tube at 7th to 10th postoperative day. The contrast material used is 10 to 15 ml of 50 percent Hypaque diluted with 30 ml normal saline. To relieve the spasm of the sphincter produced due to passage of bougie through the papilla. In choledocholithotomy, papillary edema and spasm occur due to passage of bougie through the papilla which persists for at least seven days. Under fluoroscopic control, Chiba needle is introduced through the 8th intercostal space in the midaxillary line. It is the intravenous contrast X-ray of the urinary system showing normal pelvicaliceal system, ureters and bladder. Hypernephroma-spider leg deformity of the calices (splaying of the calices) usually at one pole. Polycystic kidney disease-There is spider leg deformity of the calices in both the kidneys as the disease is bilateral. So, if the pelvicalyceal system is visualized, the kidney is normally functioning. If 100 percent parenchyma function is lost, then only isotope renogram will not show any density. Retrograde pyelography-It is the contrast imaging of the urinary system through ureteric catheters. Antegrade pyelography-Here the contrast agent is injected into the renal calices or pelvis. So the lungs containing air and the brain within the bony cavity cannot be imaged by ultrasonography. This is the straight X-ray of wrist both anteroposterior and lateral views showing 5. This is a straight X-ray of clavicle with shoulNote: All displacements in fractures are der joint showing fracture clavicle. This is the straight X-ray of humerus showing a fracture (oblique or transverse or spiral). This is a straight X-ray of left femur and pelvis showing fracture of the shaft of the left femur in its middle third. This is straight X-ray of leg showing extensive periosteal reaction and sequestrum formation at the lower end of femur. Identification of the instrument- proper identification and complete nomenclature is very important. Some uses of the instrument-The student should know the name and steps of operation in which the instrument is used. Sterilization of the instrument-The candidate should mention one method of sterilization which is most suitable for the instrument. A catch or a rachet-Once the catches are pressed the blades are kept in a closed position. The finger bow, together with the shaft and rachet constitutes the handle of the instrument. Removal of the laminated membrane and daughter cysts during operation of hydatid cyst. To strip off the peritoneum from the fascia transversalis by holding a swab in the forceps while kidney exposure or lumbar sympathectomy. To clean the blood in the suture line with a swab held in the forceps while performing gastrojejunostomy and intestinal anastomosis. An area extending from midchest to midthigh is to be cleaned with antiseptic solution. The instrument is made long to apply antiseptic dressings from a distance so that sterile gloved hand of the surgeon is not contaminated. To cover cut margins of incisions with sterile sponges to decrease chances of contamination. Blades with numbers 18, 19, 20, 21, 22, 23 criss-cross instead of transverse serand24fitinhandlenumber4. To hold free ends of sutures as they are passed one after the other without tying. Blunt dissection while doing lymph node biopsy, excision of sebaceous cyst, lipoma, etc. Uses It is the most versatile instrument in surgical practice and used as follows: a. As a hemostat to hold bleeding vessels while cutting through different layers of tissues. Features are the same as the artery forceps except that at the tip of the blades there is a tooth in one blade and a groove in the other, where the tip fits when the rachet is closed. Partial occlusion of major vessels like inferior vena cava, aorta, for their repair. The jaws may be additionally covered with a rubber tubing to prevent crushing of artery. To dissect anterior and posterior vagus nerves and to pass ligatures around them during vagotomy. During splenectomy to dissect splenic artery and vein and to pass ligature around them. It is available in different sizes depending for better retraction of the tissues. To retract the abdominal wall while mobilizing the colon from the paracolic gutter 2. To retract the bladder in male or uterus in Sterilization female during pelvic dissection in anterior resection or abdominoperineal resection. Self-Retaining Abdominal Features are same as the double hooked Uses retractor but the number of hooks are generally4or5,i. Solid retractor with a long handle and a blade which is curved at right angle to the handle.

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Lower midline incision is better avoided as the linea alba is thin and narrow and posterior sheath is deficient gastritis fundus best 10 mg aciphex. This is a time honored incision made about 1" (one inch) away and parallel to the midline chronic gastritis meaning buy aciphex 20mg without prescription. In fatty patient gastritis operation buy aciphex 20 mg free shipping, cholecystectomy some times becomes difficult by this incision ii gastritis diet ������ purchase aciphex 20 mg with amex. Rectus muscle medially and a part of external oblique nodular gastritis definition trusted aciphex 10mg, internal oblique and transverses abdominis laterally gastritis diet ��� aciphex 20 mg low price. Indication: this incision is commonly used Rutherford Morrison Incision for cholecystectomy in obese patients. Fibers of internal oblique and trans Left subcostal incision-indicated for versus abdominis are cut across splenectomy. A curved skin incision is made below iron incision is converted into and parallel to both costal margins. Extent: this incision is placed three tion of medial 2/3rd and lateral 1/3rd of fingers breadth above the umbilicus spinoumbilical line. The rest of the steps are similar to grid times be inadequate especially in obese iron incision. Extent: this incision is made along the interspinous crease with its center 5 cm (2") above the symphysis pubis. The incision is deepened through skin and subcutaneous tissue and hemostasis are: a. Positive diagnostic peritoneal lavage ments by the transverse colon and to open the abdomen. Various emergencies which constitute the divided into right upper and left lower female pelvic organs. This is a continuous full thickness clo compartment are examined first in the exploration after exclusion of fecal sure except skin of abdominal wounds following order: stomach and first part impaction by enema and/or manual with nonabsorbable sutures like no. Layered closure: Studies have revealed Steps of Operation 2nd and 3rd part of duodenum, duo that: 1. Anesthesia: General anesthesia with appendix, cecum, ascending trans tage over mass closure. To detect a second pathology, a thorough exploration of abdomen is often necessary. The kidneys are also examined done at the sigmoid colon and open at their anatomical sites. Loop colostomy-Here a loop of Definition colon is taken out and colostomy is performed. It may be in the sigmoid External drainage of colon is called or transverse colon. The purposes of colostomy colostomy and most commonly prac include the following: tised. Diverting fecal stream for some distal mies to be opened (double stoma) at pathology. Carcinoma descending colon trans Steps of Operation of Pelvic (Loop) Colostomy verse colostomy is done. The stoma is made in the proximal part of the mobile loop of pelvic colon in order to carcinoma sigmoid colon. Opening the colostomy: the colon is opened immediately along a tinea in fecal peritonitis. Position of patient, anesthesia and antiseptic dressing are the same as in case of pelvic (loop) colostomy. Incision - A transverse incision is made midway between umbilicus and costal margin placed over the rectus abdominis muscle. Fecal impaction stoma without tension and a window is eter is passed between two ends of the iii. Colostomyherniation a tissue forceps and ileum is opened by toneum by a few interrupted sutures. Application of colostomy bag after colostomy to defunction distal colorectal the functional side. Thus fatty analogous to those of the anterior vagus diarrhea and gallbladder dyskinesia, com except instead of hepatic branch it gives off mon complications of truncal vagotomy the celiac branch which supplies the pancreas are avoided. Highly selective vagotomy (syn- Parietal Surgical anatomy of VaguS cell vagotomy, or proximal gastric celiac and gastric branches is continued as the nerVeS vagotomy). Hence Since 1940s, Dragsted and his colleagues at the University of Chicago revolutionized gas troduodenal surgery by bringing vagotomy in the treatment of duodenal ulcer. Truncal vagotomy is nowadays occasion ally performed in the treatment of chronic duodenal ulcer along with gastrojejunos tomy or pyloroplasty. Gastric outlet obstruction following chronic duodenal ulcer may require gas trojejunostomy and truncal vagotomy. The chronic duo denal ulcer is evidenced by the white scar on the anterior aspect of the 1st part of duodenum and on rubbing the scar, with a dry gauge, petechial hemorrhage appears. The stomach is pulled downwards so that the cardioesophageal junction becomes exposed. The peritoneum over the cardioesopha geal junction is incised and anterior vagus is dissected out either by finger dissection or by a right angle forceps. Posterior vagus is stouter and stronger than the left and lies in the loose areolar tissue behind the esophagus. Esophagus is mobilized by finger dissec tion and retracted to left, with the help of a simple rubber catheter. Vagotomy is completed after which posterior gastrojejunostomy is decided and started. Now the selected portions of the stomach definition and jejunum are placed side by side, and anastomotic clamps are applied. Anastomosis is done in 4 layers in the fol Types lowing order from below upwards using 2/0 polyglactin sutures (Vicryl). The margin of mesocolon rent is fixed to the stomach wall with interrupted suture ulcer along with truncal vagotomy. Closure - Transverse colon is put back in Preoperative Preparation normal position and abdomen is closed in layers. Stomal obstruction which is detected high protein diet, amino acid or human by increased gastric suction even after albumin infusion. It is of medicolegal importance to prove that the structure removed is really the vagus nerve and no other structure. The local operability of the tumor is assessed by making a small opening in the gastro colic ligament which provides access to the posterior wall of stomach to see whether the tumor is free from the pancreas. The greater omentum is now reflected and mobilized from its colonic attach ment and lesser sac is completely opened. The omentum is dissected from the trans verse mesocolon carefully till the upper border of pancreas is reached. At the left extremity of greater omentum the left gastroepiploic vessels pass for wards in the gastrosplenic omentum in the hilum of the spleen. The lymph nodes at the origin of left gastroepiploic artery are carefully dissected out, and then the artery and vein are doubly ligated and divided. At the right extremity of the greater omentum, the right gastroepiploic vessels are carefully isolated along with the sub pyloric nodes before doubly ligating and dividing them at their origins from the gastroduodenal vessels. Now the stomach is drawn caudally to put on stretch the free edge of lesser omen tum. An incision is made carefully in the anterior leaf to expose the right gastric vessels and the suprapyloric lymph nodes. The nodes are dissected and the right gas tric vessels are doubly ligated and divided. Kocherization of duodenum is performed to dissect the first part from the head of the pancreas. Lesser omentum is divided as close to the porta hepatis and liver as possible safe guarding the vital structures in its right free border. The celiac nodes are dissectedfree and the left gastric artery is divided as close to its origin as possible. The stomach is turned cranially and a pair of noncrushing gastroenterostomy clamps is applied 5 cm proximal to tumor margin for proximal division of stomach. When lesser curvature of stomach is anas tomosed to proximal jejunum, it is called isoperistaltic. If there is any anastomotic leak, it col lects in greater sac, and may collect in pelvis. Nowadays, it is seldom performed in the treatment of chronic duodenal ulcer because of the success of medical treatment with antibiotics, H2 blockers and potent antacids. This is an antecolic anastomosis, which is occa sionally indicated for malignant gastric outlet obstruction as a palliative procedure, when posterior gastrojejunostomy is not possible. Here more than 80 percent of the stomach is removed along with the tumor and the lymph nodes. Radical subtotal gastrectomy Indications Carcinoma of the gastric pylorus, antrum and the distal third of body of stomach. The right gas Alternately a RouxenY gastrojejunos tric artery along the lesser curve and tomy is performed. A feeding jejunostomy may be done for greater curve are identified, ligated and enteral feeding in the early postopera divided. Size are placed loic arteries are ligated and divided at the one at the duodenal stump and the sec cardiac end of stomach. Duodenum is mobilized by Kocherization and divided at a point of about 4cm from Radical Total gastrectomy the duodenopyloric junction. The perigastric lymph nodes within 3cm of the primary tumor as well as the nodes 1. Carcinoma of the proximal body and fun along the celiac trunk and its branches dus of stomach. Linitis plastica in which the tumor cells left gastric arteries are dissected and infiltrate the submucosa, subserosa and the removed. Two tube drains are placed - One near sels, root of the mesentery, mesocolon and the duodenal stump and other near the the pancreas are involved or not. Anesthesia - General anesthesia with endotracheal intubation but local anesthe sia is also a practical alternative. The rectus abdominis muscle is divided in the line of the incision and peritoneum is opened. The liver is retracted upwards as it lies well below the costal margin in the neonate to expose the pyloric end of stomach. The thickened pylorus is pinched between the finger and thumb so as to stretch the anterior wall, which is least vascular. The hypertrophied muscle fibers (longi tudinal and circular) are split along the incision by a small artery forceps, taking care not to penetrate the mucosa. Presence of air or bile at the duodenal end of the incision is an indication of perfo indications ration. When instrumental dilatation with esopha muscle coat (seromyotomy) until sub geal bougies fail to relieve symptoms. When secondary changes like ulceration are carefully dissected apart with an occurs in the esophagus. Exposure of esophagus - the left lobe of liver is mobilized and retracted after division of left triangular ligament as in vagotomy. Exposure: the rectus muscle is divided to expose parietal peritoneum which is cut to open the peritoneal cavity. Retraction and exposure-Retractors are placed and held by the assistants to facilitate further exposure and access. As of traction gallbladder is dissected, the peritoneal folds are divided with scissors or diais continued to expose cystic duct thermy knife. Bleeding and division of cystic artery is preferpoints can be secured by diathermy or by ably done first with 1-0 silk to avoid trnsfixationsutures. Hepatobiliar y and Pancreas 1/3 and lower 2/3rd of a line joining the xiphisternumandumbilicus. One 5 mm port through the 5 mm incision is made at the right, midclavicular line 2 cm below the costal margin. Dissection of gallbladder from the liver bed: Once the cystic duct and artery are divided, the gallbladder is dissected from the liver bed by using a diathermy hook from the liver bed. Extraction of gallbladder-The separated gallbladder is held by a crocodile forceps and removed through the epigastric port. Portsiteinfection:Tubercularinfection has been reported at the port site which requires antitubercular drugs.

Both symptoms are distressing gastritis symptoms right side buy 10mg aciphex overnight delivery, cause significant morbidity and reduce quality of life gastritis symptoms patient order aciphex 10mg without a prescription. Nausea and vomiting contribute significantly to nutritional deterioration and increased pain intensity gastritis beer order aciphex overnight. Fluid input/output monitoring should take place several times within each 24-hour period in order to be able to observe unwanted changes in fluid balance and to be able to intervene gastritis diet ��������� order 10 mg aciphex with amex, if necessary gastritis diet livestrong discount 10mg aciphex otc. Chapter 23 Supportive care in the management of leukaemia Vomiting is pretty common in patients on chemotherapy and in combination with diarrhoea and sweating due to pyrexia might rapidly lead to dehydration gastritis diet for diabetics purchase aciphex cheap. Potential vasodilatation due to sepsis might exacerbate hypovolaemia and therefore strict monitoring and fluid replacement should be undertaken. Hydration prior to cytotoxic agents Hydration, urine alkalinization (pH 7 to 8), and electrolyte monitoring can prevent the hyperuricaemia, hyperphosphataemia, hypocalcaemia and hyperkalaemia. This is more relevant at diagnosis as the disease bulk is increased and the risk appears to be higher. Close monitoring is very important to prevent complications associated with tumour lysis syndrome or fluid overload. Catabolism of the nucleic acids to uric acid leads to hyperuricaemia, and the marked increase in uric acid excretion can result in the precipitation of uric acid in the renal tubules and can also induce renal vasoconstriction, decreased renal blood flow and inflammation, resulting in acute kidney injury. Hyperphosphataemia with calcium phosphate deposition in the renal tubules can also cause acute kidney injury. They include nausea, vomiting, diarrhoea, anorexia, lethargy, haematuria, heart failure, cardiac dysrhythmias, seizures, muscle cramps, tetany, syncope and possible sudden death. Diuretics can be used to maintain the urine output, if necessary, but should not be required in patients with relatively normal renal and cardiac function. Allopurinol is a hypoxanthine analogue that competitively inhibits xanthine oxidase, blocking the metabolism of hypoxanthine and xanthine to uric acid. Because it acts by decreasing uric acid formation, allopurinol does not reduce the serum uric acid concentration before treatment is initiated. Allopurinol increases serum levels of the purine precursors hypoxanthine and xanthine, which may lead to xanthinuria, deposition of xanthine crystals in the renal tubules, and acute kidney injury. Allopurinol has the potential to interact with a number of other drugs, including cyclophosphamide, high-dose methotrexate, ampicillin and thiazide diuretics. The usual allopurinol dose in adults is 100 mg/m2 every 8 hours (maximum 800 mg per day). An alternative approach to allopurinol for lowering serum uric acid levels is to promote the degradation of uric acid by the administration of urate oxidase (uricase), which catalyses oxidation of uric acid to the much more water-soluble compound allantoin. This rapid reduction in serum uric acid is in contrast to the effect of allopurinol, which decreases uric acid formation and therefore does not acutely reduce the serum uric acid concentration. Anaemic patients with leucocytosis should be transfused with caution as there is a risk of increased plasma viscocity. It typically occurs during induction therapy with differentiating agents, especially in patients presenting with high white cell count at diagnosis or relapsed disease. This syndrome is characterized by unexplained fever, weight gain, hypotension, acute respiratory distress with interstitial pulmonary infiltrates and/or a vascular capillary leak syndrome leading to acute renal failure. Most patients will show rapid improvement and complete resolution of signs and symptoms after starting intravenous dexamethasone. Corticosteroid therapy should be continued until the complete disappearance of symptoms, and then tapered. Some cases with refractory acute renal failure and/or fluid overload may need renal replacement therapy. Invasive or noninvasive mechanical ventilation is indicated in some patients with severe acute respiratory failure who do not respond to high-flow oxygen therapy. Patients with prerenal failure and hypotension require careful fluid monitoring in conjunction with inotropic support. Skin, nail and dental problems Skin reactions to drug therapy are extremely common. Chemotherapy drugs can cause dry skin, hyperpigmentation, discoloration, serpentine hyperpigmentation, photoallergic reactions and acne (mainly treatment with steroids). Nails should be trimmed and kept clean and gloves should be worn for house-cleaning at discharge. Occasionally antibiotics and antifungal agents might be necessary to treat underlying infections. Radiation recall Radiation recall is a skin reaction that can occur when certain chemotherapy drugs are given after radiation therapy. Radiation recall may be treated with 416 Chapter 23 Supportive care in the management of leukaemia corticosteroids to lessen inflammation or chemotherapy may be delayed until the skin heals. Pain Many leukaemia patients experience bone or joint pain that results from the bone marrow being heavily infiltrated by leukaemia cells. Oral and gut mucocytis are probably the most common site of pain, especially in recipients of high-dose chemotherapy or total body irradiation. Infections can also cause pain, especially in areas such as the skin, mouth, eyes and perianally. Avascular necrosis of bone is another cause of pain in patients with acute lymphoblastic leukaemia and is often severe enough to need relief with opiates. Peripheral neuropathy and associated neuropathic pain is a widespread side-effect of treatment with certain chemotherapeutic agents. The pain associated with these neurotoxic effects can be prolonged, severe and relatively resistant to intervention. The overall incidence is not clearly delineated, although it is documented frequently with vincristine and platinum-based agents. Leukaemia and chemotherapy can make the mouth sensitive, easily infected and likely to bleed. Patients should receive a complete dental examination and, if possible, undergo necessary dental care before chemotherapy begins, since any existing lesions that might normally lie dormant can flare up and become life-threatening once the patient is immunosuppressed. Dental extractions should preferably be performed at least 10 to 14 days before the commencement of chemotherapy, so that epithelization of the extraction site has been completed prior to the initiation of treatment. Dental procedures that could trigger a bleeding episode or/and bacteraemia during the aplastic phase should be avoided. The use of chlorhexidine mouthwash is very important, primarily during the phase of aplasia. Aesthetic restorations of the enamel defects are also often necessary, especially after completion of treatment. Antibiotics, such as the aminoglycosides, and chemotherapeutic agents, such as cisplatin, can cause considerable ototoxicity in as many as 40% of patients. Dosage reduction and regular assessment with auditory studies during the course of therapy might reduce toxicity and prevent further damage. Visual toxicity Ophthalmic complications induced by cytotoxic chemotherapy are often under-estimated and under-reported due to priority given to other life-threatening conditions; however, most of the ophthalmic complications are reversible if detected early enough. Among the functional complaints are blurred vision, decreased colour vision, diminished visual acuity, diplopia, night blindness and photophobia. Among the drugs used on a regular basis to treat leukaemia, cytarabine can cause conjunctivitis and methotrexate can cause oedema, ocular pain, blurred vision, photophobia, conjunctivitis and blepharitis. Chlorambucil has been reported to cause keratitis and daunorubicine excessive lacrimation and conjunctivitis. The possible reversal of some of these side-effects, if discovered in time, emphasizes the need for clinicians to be aware of these ocular reactions and suggest immediate consultation by an ophthalmologist. Palliation Relapse post chemotherapy or following a bone marrow transplant is a common theme in leukaemia. Intensive salvage chemotherapy or other modalities can often achieve prolonged remission; however, refractory unresponsive-to-chemotherapy disease is not uncommon in this setting. Several experimental treatments can be offered; however, it is important to weigh the possible limited benefits of a new treatment against the possible downsides as quite often further treatment might not improve the health or change the outcome or survival. As well as controlling pain and other distressing symptoms, it applies a holistic approach to meeting the physical, practical, functional, social, emotional and spiritual needs of patients and carers facing progressive illness and bereavement. It is not uncommon for patients to ask to remain under the care of the same haematology team; however, the palliative care team should also be involved as the choice of dying at home versus 417 Postgraduate Haematology dying in a hospice requires some expertise and external support better managed by the palliative care services. Selected bibliography Karaiskos I, Giamarellou H (2014) Multidrug-resistant and extensively drug-resistant Gram-negative pathogens: current and emerging therapeutic approaches. This genetic event that encodes for a constitutively active tyrosine kinase occurs in a haemopoietic progenitor and confers proliferative and antiapoptotic effects. The disease is most commonly diagnosed in the fifth and sixth decades of life in developed countries. In the developing world, presentations in the third and fourth decades are more common, possibly reflecting younger population demographics. Many are asymptomatic and the diagnosis is often incidental to investigation for an unrelated medical problem. The most common presentations include unintentional weight loss, night sweats, fatigue and splenomegaly (which may cause early satiety and anorexia). The most striking haematological abnormality is usually a leucocytosis, with the differential heavily favouring mature neutrophils, band forms and myelocytes. Absolute monocytosis is uncommon and, if present, usually associates with the e1a2 breakpoint (see below). Rarely, patients may present with a marked thrombocytosis with only mild leucocytosis. Myeloid maturation is normal or slightly left shifted, and blasts are usually less than 5%. The myeloid to erythroid ratio is markedly increased and megakaryocyte numbers may be either decreased or increased compared to the normal marrow; small, hypolobated forms are common. Increased reticulin fibres are seen in 30% of cases and correlate with larger spleen size. Pseudo-Gaucher cells and sea blue histiocytes may be seen secondary to increased numbers of phagocytic cells 420 amidst increased cell turnover. There may be an increased myeloblast percentage in peripheral blood or marrow, as well as increasing leucocytosis, splenomegaly, basophilia or thrombocytosis, despite ongoing therapy. These abnormalities frequently include duplication of the Ph chromosome, trisomy 8 or 19 and isochromosome 17q. Leucocytosis is prominent, with increased neutrophils and myelocytes, but no obvious dysplastic features. Several myelocytes, a basophil and an eosinophil can be seen towards the bottom of the photomicrograph. Red cell lysis agent is added to whole blood and a white cell pellet is obtained after centrifugation. The remaining red and green signals originate from the normal chromosome, which is not rearranged. The resulting 422 fusion transcripts are named e13a2 and e14a2 transcripts (previously known as b2a2 and b3a2); both may coexist in one individual due to alternate splicing. The translocation is carried by the leukaemic stem cell population, such that both myeloid and lymphoid progeny may carry t(9;22) in an affected individual. When patients are successfully treated with tyrosine kinase inhibitors that specifically target the Ph+ clone, the risk of transformation starts to fall when a reduction in the overall number of leukaemic cells can be achieved. Examination of marrow metaphases will reveal a decreasing proportion of cells with the Ph chromosome as a proportion of total metaphases examined, as normal haemopoietic stem cells and their progeny recover. The proportion of residual Ph+ cells correlate to different levels within a formal classification of cytogenetic responses. Although cytogenetic examination is a valuable tool for monitoring disease response, it is labour intensive, and entails significant inconvenience and discomfort for the patient. Furthermore, it is routine in some laboratories to examine only 20 metaphases per patient, resulting in a detection sensitivity for residual disease of 5%. Peripheral blood is more commonly studied as it is convenient to collect and entails less discomfort for the patient. As well as a ratio, it is also common to describe the residual tumour burden as a log10 reduction from the standardized baseline, with 10%, 1%, 0. Patients who fail to achieve timedependent treatment targets (see later) may benefit from more frequent testing. Oral cytotoxics, such as hydroxycarbamide (hydroxyurea), are used commonly for cytoreduction of leucocytosis before confirmation of diagnosis (leucapheresis can also be used in more extreme cases). During the initial cytoreduction phase, it is common to coadminister allopurinol to blunt any rise in serum urate, and to monitor for electrolyte disturbances carefully. Although interferon- results in durable complete cytogenetic response in 20% of patients, its toxicity precludes generalized use. Its introduction transformed a previously fatal disease for many into a manageable chronic illness with excellent long-term survival. Superiority of the imatinib arm was demonstrated unequivocally, leading to crossover of 89. The most common resistance mechanism identified is kinase domain mutations (see below). Clonal evolution, such as acquisition of an extra copy of the Ph+ chromosome or development of isochromosome 17q may also be involved.