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J. Kelvin, MD

Assistant Professor, Louisiana State University School of Medicine in New Orleans

A gluten-free diet usually relieves symptoms and restores mucosal histology, and therefore it is the most appropriate therapeutic measure. Corticosteroids may be employed in the treatment of refractory sprue, but a gluten-free diet is the most appropriate step at this time. While patients with celiac sprue are often deficient in B vitamins, treating the underlying pathology is the best course. While patients with celiac sprue are often deficient in fat-soluble vitamins, treating the underlying pathology is the best course. As a result, interferon is contraindicated in severely depressed or suicidal patients. Although interferon is not a cure for hepatitis, it is recommended to slow the progression of cirrhotic liver disease in some patients. Tumor necrosis factor-a is a cytokine involved in the antiviral and antitumor response. When the right leg is hyperextended, the iliopsoas muscle group pushes against the appendix and causes significant pain and irritation. Pain with hyperextension will also be present in pancreatic cancers and inflammation of the cecum and the sigmoid colon. The psoas muscle is innervated by the lumbar plexus, and the iliacus muscle is innervated by the femoral nerve. The obturator nerve originates from the lumbar plexus and innervates the medial thigh muscles. The sciatic nerve innervates the hip joint, the muscular knee flexors in the thigh, and all the leg and foot muscles. The superior gluteal nerve innervates the gluteus medius and gluteus minimus muscles. Shigella species produce gastroenteritis characterized by abdominal pain, bloody diarrhea, and nausea and/ or vomiting. Additionally, because Shigella species invade intestinal epithelial cells, the illness is accompanied by fever. Shigella is a nonlactose fermenter, and it does not produce gas or hydrogen sulfide. Infection usually affects preschool-age children and populations in nursing homes. Transmission occurs by the fecal-to-oral route via fecally contaminated water and hand-to-hand contact. This syndrome develops after the endothelium is damaged in the kidney and results in renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. These two symptoms, along with diarrhea and fever, are characteristic of typhoid fever caused by Salmonella typhi. This describes an inhaled infection by Bacillus anthracis, which is not connected to any of the other symptoms this child has. These symptoms describe rheumatic fever, which is caused by an infection by Streptococcus pyogenes. S pyogenes does not cause dysentery, nor does it fit the laboratory description of the causative organism in this case. The intern is thinking about octreotide, a somatostatin analog used to treat acromegaly. Gastrin is a hormone released by stomach G cells in response to stomach distention, vagal stimulation, and proteins. Through the actions of gastrin on parietal cells, the end result of gastrin release is increased acid secretion. Gastrin is not indicated for the treatment of acromegaly and does not have a role in exocrine pancreas function. Secretin is a hormone produced by duodenal S cells and secreted in response to increased duodenal fatty acids and acidity.

Early symptoms, such as forgetfulness, a lack of muscle coordination, or a loss of balance, are often ignored, delaying the diagnosis. Within a few years characteristic involuntary movement (chorea) of the body, limbs, and facial muscles appears. Eventually, nearly all patients must be institutionalized, and they usually die as a result of choking or infections. In some cases it is even possible to make a prenatal diagnosis on an unborn child. Many people, however, prefer not to know whether or not they carry the defective gene. Currently, researchers are trying to determine if the exact number of excess triplets indicates when in life a person will be affected by the disease. Some scientists fear that the ability to tell people that they are going to develop an incurable disease and pinpoint when they will develop it will make genetic testing, already a difficult decision, even more complicated. Each variant of the disease is caused by defects in the genes that play important roles in the growth and development of muscles. Unable to function properly, the muscle cells die and are replaced by fat and connective tissue. Dystrophin provides structural support for muscle cells and without it the cell membrane becomes penetrable, allowing extracellular components into the cell. With myotonic dystrophy the muscles contract but have diminishing ability to relax, and there is muscle weakening and wasting. Typically, the initial complaints are the loss of hand strength or tripping while walking or climbing stairs. Along with decreased muscle strength, myotonic dystrophy may cause mental deficiency, hair loss, and cataracts. The myotonic dystrophy gene is a protein kinase gene found on the long arm of chromosome 19. In contrast, people with the mild phenotype of myotonic dystrophy have between 40 and 170, and those with more serious forms of the disease have between 100 and 1,000 iterations. They vary, however, in terms of the usual age at the onset of symptoms, rate of progression, and initial group of muscles affected. Females who inherit the defective gene generally do not manifest symptoms-they become carriers of the defective genes, and their children have a 50% chance of inheriting the disease. In 1992 scientists discovered the defect in the gene that causes myotonic dystrophy. National Library of Medicine, National Center for Biotechnology Information, 2004 HbS is responsible for the premature destruction of red blood cells, or hemolysis. In addition, it causes the red cells to become deformed, actually taking on a sickle shape, particularly in parts of the body where the amount of oxygen is relatively low. These abnormally shaped cells cannot travel smoothly through the smaller blood vessels and capillaries. This blockage produces anoxia (lack of oxygen), which in turn causes more sickling and more damage. Genetic research offers hope of finding effective treatments, and even cures, for these diseases. Research teams have identified the crucial proteins produced by these genes, such as dystrophin, beta sarcoglycan, gamma sarcoglycan, and adhalin. One experimental treatment approach involves substituting a protein of comparable size, such as utrophin for dystrophin, to compensate for the loss of dystrophin. Muscle cells, unlike other cells in the body, fuse together to become giant cells. New delivery methods called vectors are also being tested, such as implanting a healthy gene into a virus that has been stripped of all of its harmful properties and then injecting the modified virus into a patient. As a result of the defect, the affected individual is unable to convert phenylalanine into tyrosine.

Haemoglobinlevelsaregenerallyapoorindicatoroftheneedtotransfusebecauseanaemia does not develop immediately as haemodilution has not taken place. Endoscopy shouldbe carried outas soon as possibleafter the patienthas been resuscitated. Patients with Rockall scores of 0 or 1 pre-endoscopy may be candidates for immediate discharge(seebelow)andoutpatientendoscopythefollowingday,dependingonlocalpolicy. Uncontrolledorrepeatbleeding Endoscopy should be repeated to assess the bleeding site and to treat, if possible. Ifthis is not available locally or is unsuccessful, surgery is used to control the haemorrhage primarily. Ingeneral,all patients who are haemodynamically stable and have no stigmata of recent haemorrhage on endoscopy (Rockall score pre-endoscopy 0, post-endoscopy <1) can be discharged from hospital within 24hours. All shocked patients and patients with co-morbidity need longer inpatientobservation. Early endoscopy confirms diagnosis and allows therapy such as clipping if necessary. If bleeding is not controlled, the patient should either undergo angiography and embolizationorbereferreddirectlyforsurgery. Gastriccarcinoma Most of these patients do not have large bleeds but surgery is occasionally necessary for uncontrolledorrepeatbleeding. Ithasbecomeincreasinglyevidentinthispatientgroupthatgastroscopy should be performed on an urgent basis and not deferred for days or weeks. Management is difficult, as cessation of antiplatelet therapy has a high risk of acute stent thrombosis and also an associated high mortality. The lowest mortality rates are achieved in dedicated medical/surgicalgastrointestinalunits. The few patients who continue bleeding and are haemodynamically unstable need resuscitation using the same principlesasforuppergastrointestinalbleeding(seep. Isolated episodes of rectal bleeding in the young (<45years) usually only require rectal examination and flexible sigmoidoscopy because the probability of a significant proximal lesionisverylow,unlessthereisastrongfamilyhistoryofcolorectalcanceratayoungage. Chronicgastrointestinalbleeding Patients with chronic bleeding usually present with iron deficiency anaemia (see pp. Theprimaryconcernisto excludecancer,particularly of the stomach or right colon, and coeliac disease. Capsuleendoscopyisthediagnosticinvestigationofchoice but currently has no therapeutic ability. Its surface area is enormously increased by circumferential mucosal folds that bear multiplefinger-likeprojectionscalledvilli. The lamina propria contains plasma cells, lymphocytes, macrophages,eosinophilsandmastcells. On its luminal side, the enterocyte is covered by microvilli and a gelatinous layer called the glycocalyx. Most of the blood supply to the small intestine is via branches of the superior mesenteric artery. The terminal branches are end arteries; there are no local anastomotic connections. It is estimated to contain 108 neurones (as many as the spinal cord), organized in two major ganglionatedplexuses:themyentericplexusbetweenthemuscularlayersoftheintestinalwall, and the submucosal plexuses associated with the mucosa. Coordination of small intestinal function involves a complex and only partly understood interplay between many neuroactive mediators and their receptors, ion channels, gastrointestinal hormones, nitric oxide and other transmitters. The irregular contractions of the fed pattern have a mixing function,movingintraluminalcontentstoandfroandaidingthedigestiveprocess. Neuroendocrinepeptideproduction the hormone-producing cells of the gut are scattered diffusely throughout its length and also occurinthepancreas. Regulatesintestinalbloodflow Motilin Ghrelin Obestatin Wholegut Stomach Stomachand small intestine Colon Wholegutand pancreas Stomachand pancreas(D cells) Smallandlarge intestine Entericnerves Ileum Increasesgastricemptyingandsmallbowelcontraction Stimulatesappetite,increasesgastricemptying Opposesghrelin Oxyntomodulin Gastrinreleasing-polypeptide(bombesin) Somatostatin Inhibitsappetite Stimulatespancreaticexocrinesecretionandgastricacid secretion Inhibitssecretionandactionofmosthormones SubstanceP Neurotensin Insulin Chromogranins Enhancesgastricacidsecretion,smoothmusclecontraction Affectsgutmotility. Increasesjejunalandilealfluidsecretion Pancreaticcells Increasesglucoseutilization Neuroendocrine cells Precursorforotherregulatorypeptidesthatinhibit neuroendocrinesecretion Physiology In the small bowel, digestion and absorption of nutrients and ions takes place, as does the regulation of fluid absorption and secretion.

Scarlet fever may be complicated by peritonsillar or retropharyngeal abscesses and otitis media. Clinicalfeatures Diphtheria was formerly a disease of childhood but may affect adults in countries where childhoodimmunizationhasbeeninterruptedoruptakeispoor. Nasal diphtheria is characterized by the presence of a unilateral, serosanguineous nasal dischargethatcrustsaroundtheexternalnares. Pharyngeal diphtheria is associated with the greatest toxicity and is characterized by marked tonsillar and pharyngeal inflammation and the presence of a membrane. Clinically evident myocarditis occurs, often weeks later, in patients with pharyngeal or laryngeal diphtheria. Acute circulatory failure due to myocarditis may occur in convalescent individuals around the tenth day of illness and is usually fatal. Typically,theulcerispunched-outwithunderminededgesandis covered with a greyish white to brownish adherent membrane. Diagnosis this must be made on clinical grounds since therapy is usually urgent; the mortality rate is about10%. It must be given promptly to prevent further fixationoftoxintotissuereceptors,sincefixedtoxinisnotneutralizedbyantitoxin. Antibiotics should be administered concurrently to eliminate the organisms and thereby remove the source of toxin production. Prevention Patients with suspected diphtheria must be isolated, and the local public health authorities shouldbeinformed. All contacts of the patient should have throat swabs sent for culture; those with a positive resultshouldbetreatedwithpenicillinoramacrolide,andbegivenactiveimmunizationora boosterdoseofdiphtheriatoxoid. In its early stages, it is indistinguishable from other types of upper respiratory tract infection. Whoopingcoughismainlyadiseaseofchildhood,with 90% of cases occurring below 5years of age. However, no age is exempt, as the antibody levelsfallovertheyears,althoughinadultsmildinfectionmaynotberecognised. During the catarrhal stage, the patient is highly infectious and cultures from respiratory secretions are positive in over 90% of patients. The paroxysmal stage, so called because of the characteristic paroxysms of coughing, begins about 1week later. Paroxysms with the classic inspiratory whoop are seen only in younger individuals in whom the lumen of the respiratory tract is compromisedbymucussecretionandmucosaloedema. Conjunctival suffusion and petechiae, and ulceration of the frenulum of the tongue, are common. Thisstagelastsapproximately2weeksandmaybeassociatedwith several complications, including pneumonia, atelectasis, rectal prolapse and inguinal hernia. Cerebral anoxia may occur, especially in younger children, resulting in convulsions. Pertussis is an easily preventable disease and effectiveactiveimmunizationisavailable(seeBox11. Convulsionsandencephalopathy have been reported as rare complications of vaccination but they are probably less frequent thanafterwhoopingcoughitself. Thecausativeorganism, Chlamydiapsittaci,isexcretedinaviansecretions;itcanbeisolated forprolongedperiods from birds that have apparently recovered from infection. It is a cause of community-acquired infections in tropical countriesandisassociatedwithwarsandnaturaldisasters. Otherrespiratoryinfections Chlamydophila pneumoniae causes a relatively mild pneumonia in young adults, clinically resembling infection caused by Mycoplasma pneumoniae. Other groups who are at increasedrisk ofinfectiousdiarrhoeainclude travellerstodeveloping countries, men having sexwithmen,andinfantsinday-carefacilities. It is a major cause of morbidity and mortality among infantsinlow-incomecountries.