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By L. Peratur. Chaminade University of Honolulu, Hawaii. 2018.

Denition Achalasia is a disordered contraction of the oesophagus Aetiology/pathophysiology of neuromuscular origin order nitrofurantoin 50 mg with mastercard. In co-ordinationbetweenthecontractionofthepharynx andrelaxationoftheupperoesophagealsphinctercauses Aetiology the pharyngeal mucosa to herniate posteriorly between Degeneration is seen in the vagus nerve associated with the upper and lower bres of the inferior constrictor adecrease in ganglionic cells in the Auerbach s nerve muscle (Killian s dehiscence) discount 50mg nitrofurantoin fast delivery. Surgical intervention is indicated panosoma cruzi causes destruction of the myenteric in those who fail to respond; a 10 12 cm incision is made plexus nitrofurantoin 50mg free shipping. Gastro-oesophageal reux is a The neuromuscular damage causes disordered motility complication with both procedures. On manom- etry there is aperistalsis and incomplete relaxation of Diffuse oesophageal spasm the lower oesophageal sphincter in response to swallow- ing. The gastrooesophageal sphincter classically remains Denition tightly closed and there is dilation of the oesophagus. Aetiology/pathophysiology Clinical features There is a generalised abnormality of the oesopha- Patients present with progressive dysphagia, regurgita- gus with resultant hypermotility leading to painful oe- tionandnocturnalaspiration. Clinical features Complications Painisretrosternalandrangesfrommildtoseverecolicky Patients may aspirate and develop respiratory symp- spasms that occur spontaneously or on swallowing. Achalasia may predispose to oesophageal car- cinoma even after successful treatment (incidence of Investigations 5 10%). Barium swallow may show a corkscrew appearance due to contracted muscle (nutcracker oesophagus). Manom- Investigations etry can be used to identify the diseased segment and is r Achest X-ray may reveal a uid level behind the heart. There may be Management supercial mucosal erosions with a very narrow pas- Calcium channel blockers can reduce the amplitude sage of barium (rat s tail) into the stomach through of the contractions. Surgical intervention with open or tho- r 24-hour pH and manometry studies can differentiate racoscopic myotomy is considered in refractory cases. The myotomy should extend the entire length of the r Upper gastrointestinal endoscopy is performed to ex- involved segment of oesophagus and through the lower clude a tumour. The gastrooesophageal ux a fundoplication should also be performed (see junction may or may not be tight. Biopsy reveals inammation and Mallory-Weiss tear mucosal ulceration in the oesophagus secondary to bacterial overgrowth. Denition Atear in the mucosa normally at or just above the oe- Management sophageal gastric junction. Investigations Management Young patients with a typical history do not require in- Small perforations occurring in the neck are managed vestigation. Other patients with an upper gastrointesti- with broad-spectrum antibiotics and nasogastric tube. Oesophageal perforation secondary to malignancy at or above the lower oesophageal sphincter Management can be treated with a covered metal stent placed endo- Almostallstopspontaneously. Oesophageal perforation Disorders of the stomach Denition Perforation of the oesophagus resulting in leakage of the Gastritis contents. Gastritis is inammation of the gastric mucosa, which Aetiology can be considered as acute or chronic and by the under- Arare complication of endoscopy, foreign bodies and lying pathology (see Fig. Occasionally a rupture following forceful vom- Thereislittlecorrelationbetweenthedegreeofinam- iting may occur (Boerhaave s syndrome). En- Pathophysiology doscopy can be performed to conrm the diagnosis but Perforationusuallyoccursatthepharyngeo-oesophageal is rarely indicated in acute gastritis. Acute erosive gastritis Clinical features Denition Presentations include surgical emphysema of the neck; Supercial ulcers and erosions of the gastric mucosa de- intense retrosternal pain, tachycardia and fever in velop after major surgery, trauma or severe illness. Gastritis Acute Chronic Acute gastritis Acute erosive Autoimmune Bacterial Reflux Ingested Atrophic gastritis e. Most duodenal ulcers oc- cal illness possibly due to the increased intracranial cur in the proximal duodenum, most gastric ulcers occur pressure causing an increased in vagal secretormotor on the lesser curve. Rare sites include the following: r The oesophagus following columnar metaplasia due stimulus. Pathophysiology Macroscopy/microscopy Ulcerationresultsfromanimbalancebetweenthegastric The gastric mucosa appears hyperaemic with focal loss secretion of acid and the ability of the mucosa to with- of supercial gastric epithelium (ulceration) and small stand such secretion. Identication and management of the underlying cause is required, specic interventions include the use of H2 Clinical features antagonists and proton pump inhibitors. Clinically patients present with dyspepsia, which they often describe as indigestion, nausea and occasionally Peptic ulcer disease vomiting. Duodenal ulcers tend Denition to cause well-localised epigastric pain that may radiate Apepticulcer is a break in the integrity of the stomach to the back. Macroscopy/microscopy Chroniculcershavesharplydenedborders,withoutany Age heaping up of the edges (which would be suggestive of a More common with increasing age. There is a break in the integrity of the epithelium extending down to the muscularis mucosa.

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Because the alveoli are designed to easily ex- pand when we breathe in and collapse when breathing out there is a risk that the thin walls would stick together purchase nitrofurantoin 50mg on line. To prevent this there is a layer of a protein called surfactant coating the alveolar membranes generic 50mg nitrofurantoin free shipping. Surrounding the alveoli is a complex network of capillaries that carry the blood and red blood cells through the lungs to pick up oxygen and discard the carbon dioxide quality nitrofurantoin 50 mg. Between the capillaries and the alveoli cells is a layer of protein called the basement membrane and the pulmonary interstitium. The latter contains a variety of cells, collagen and elastic fibers that facilitate the expanse of the lungs. Parenchyma The definition of parenchyma is: The tissue characteristic of an organ, as distinguished from associated connective or supporting tissues. The majority of the lung tissue consists of the airways and gas exchange membranes as discussed above. There is some interstitial tissue between the alveolar cells and the capillary wall. Cell Morphology and Function There are many different types of cells found in the airways of the lung. For example some cells are present for physical support, some produce secretions and others defend the body against infection. Type I pneumocyte: These are the flat epithelial cells of the alveolar wall that have the appearance of a fried egg with long processes extending out when seen under a microscope. They enter the alveoli from the blood through small holes in the wall called the pores of Kohn. Smooth muscle cells: As discussed above the airways down through the level of the terminal bronchioles contain bands of smooth muscle. The muscle cells are controlled by the autonomic nervous system and chemical or hor- mones released from other cells such as mast or neuroendocrine cells. Ciliated epithelia cells: The lining of the majority of the airways is com- posed of pseudostratified, tall, columnar, ciliated epithelial cells. The cilia are hair-like projections on the surface of these cells that beat in rhythmic waves, allowing the movement of mucus and particles out of the lungs. Goblet cells: This cell type is found interspersed with the ciliated epithelial cells. Basal cells: These are small epithelia cells that are found along the basement membrane of the epithelium. Lymphocytes and mast cells: These cells are part of the immune defense of the body. They make, store and secrete a variety of substances including lipids and proteins. They can also develop into other cell types as needed to replace the loss of cells. This is accomplished by the exchange of air in the lungs with the ambient air through the process of pulmonary ventilation. This is initiated when the diaphragm contracts causing it to descend into the abdomen. When this occurs the volume of the lungs increases and by the laws of physics the pres- sure within the lungs decreases leading to a rush of air into the lungs. When the diaphragm relaxes and the lung tissues naturally recoil, the pressure in the lungs increases pushing air out of the lungs. Respiration is controlled by a number of factors including the autonomic nervous system, the voluntary muscles of respiration, the levels of carbon dioxide and oxygen in the blood, and the level of acid in the blood. During normal respiration between 400 and 1000 ml of air is moved into and out of the lungs; however, all of this volume is not available for gas exchange. The airways proximal to these are referred to as the conducting airways or ana- tomic dead space. This is the amount or volume of air breathed each minute and is a function of the tidal volume (see table of lung volume definitions) and the breathing rate. During exercise this can increased as a result of increasing the rate breathing and volume of each breath to as much as 150 L. These are useful for the diagnosis and discussion of disease processes affecting the lungs. Through the upper airways and to the level of the terminal bronchioles, airflow occurs by bulk movement or convection. Because of the vast increase in cross-sectional area after the ter- minal bronchioles airflow slows and the gas molecules move by diffusion. The velocity of airflow is dependent on both airway resistance related to the size of the airway and lung compliance (stiffness) that results from the mechanical constraints of the chest wall. The base of the lung receives more ventilation per volume of lung than does the top or apex.

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Because varicella vaccine contains neomycin discount 50 mg nitrofurantoin with visa, individuals with neomycin hypersensitivity would be at potential risk for an allergic reaction ( 121) order nitrofurantoin 50 mg free shipping. There are several case reports of anaphylactic episodes after hepatitis B vaccine (122 purchase nitrofurantoin 50mg with amex,123). Until 1999, the only hepatitis B vaccines available contained thimerosal, making them difficult to administer to individuals with thimerosal allergy (124). Introducing MedWatch: a new approach to reporting and device adverse effects and product problems. The prevention of immediate generalized reactions to radiocontrast media in high risk patients. Immunologic mechanisms of penicillin allergy: a haptenic model system for the study of allergic diseases in man. Classification of allergic reactions responsible for clinical hypersensitivity and disease. The role of a documented allergic profile as a risk factor for radiographic contrast media reactions. Relationship of acetyltransferase activity to antinuclear antibodies and toxic symptoms in hypertensive patients treated with hydralazine. Effect of acetylator phenotype on the rate at which procainamide induces antinuclear antibodies and the lupus syndrome. Diagnosis of sulfonamide hypersensitivity reactions by in vitro rechallenge with hydroxylamine metabolites. Allergic reactions to antimicrobial drugs in patients with a history of prior drug allergy. Sensitization to aztreonam and cross-reactivity with other beta-lactam antibiotics in high-risk patients with cystic fibrosis. Pneumocystis carinii pneumonia: a comparison between patients with the acquired immunodeficiency syndrome and patients with other immunodeficiencies. Boston Collaborative Drug Surveillance Programs: drug induced anaphylaxis, convulsions, deafness, and extrapyramidal symptoms. Drugs and other agents involved in anaphylactic shock occuring during anesthesia: a French multicenter epidemiological inquiry. A prospective study of the risk of an immediate adverse reaction to protamine sulfate during cardiopulmonary bypass surgery. IgE against ethylene oxide-altered human serum albumin in patients with anaphylactic reactions to dialysis. Plasma histamine but not anaphylatoxin levels correlate with generalized urticaria from infusions of antilymphocyte monoclonal antibodies. Human serum sickness: a prospective analysis of 35 patients treated with equine anti-thymocyte globulin for bone marrow failure. Immunology of a serum sickness/vasculitis reaction secondary to streptokinase used for acute myocardial infarction. Serum sickness-like reactions to amoxicillin, cefaclor, cephalexin, and trimethoprim-sulfamethoxazole. Serum sickness and plasmacytosis: clinical, immunologic, and hematologic analysis. Human serum sickness: a prospective analysis of 35 patients treated with equine and thymocyte globulin for bone marrow failure. Antibodies to nuclear antigens in patients treated with procainamide or acetylprocainamide. Remission of procainamide-induced lupus erythematosus with N-acetylprocainamide therapy. Graft-versus-host reactions: clues to the etiopathology of a spectrum of immunological disease. The American College of Rheumatology 1990 criteria for the classification of vasculitis: introduction. Drug-induced cutaneous reactions: a report from the Boston Collaborative Drug Surveillance Program on 15,438 consecutive inpatients, 1975 to 1983. Drug eruptions: causative agents and clinical types: a series of in-patients during a 10-year period. Angio-oedema in relation to treatment with angiotensin converting enzyme inhibitors. Severe angioedema after long term use of an angiotensin-converting-enzyme inhibitor. Prevalence and relevance of allergic reactions in patients patch tested in North America 1984 to 1985.

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She thinks that her grandmother cheap 50 mg nitrofurantoin amex, who lived in Ireland purchase nitrofurantoin 50mg, had some bowel problems but she died 3 years ago order 50 mg nitrofurantoin with amex, aged 68. She is an infant- school teacher and spends a lot of her spare time in keep-fit classes and routines at a local gym. Examination of her abdomen showed no abnormalities and there are no other significant abnormalities to find in any other system. The report of a dimorphic blood film means that there are both small and large cells. This suggests that the anaemia is caused by a combination of the folate deficiency indicated by the red cell folate and by iron deficiency. The Howell Jolly bodies are dark blue regular inclusions in the red cells which are typically found in the blood of patients after splenectomy, or are associated with the splenic atrophy which is characteristic of coeliac disease. In coeliac disease, there is a sensitivity to dietary gluten, a water-insoluble protein found in many cereals. The proximal small bowel is the main site involved with loss of villi and an inflammatory infiltrate caus- ing reduced absorption. Causes of macrocytosis in the blood film Folate deficiency Vitamin B12 deficiency Excessive alcohol consumption Hypothyroidism Certain drugs, e. Other diagnoses which might be considered are anorexia nervosa (her age and sex, commitment to exercise); she does not appear depressed (a common cause of weight loss and bowel dis- turbance) and the laboratory findings clearly indicate physical disease. Diagnosis of coeliac disease can be confirmed by endoscopy at which a biopsy can be taken from the distal duodenum. The treatment is a gluten- free diet with a repeat of the biopsy some months later to show improvement in the height of the villi in the small bowel. Another common cause of failure to recover the villus architec- ture is poor compliance to the difficult dietary constraints. She has had three episodes of cough, fever and purulent sputum over the last 6 months. Recently she has had trouble with regurgitation and vomiting of recognizable food. She lived in the north-west coast of the United States for 4 years up until 10 years ago. She has always tended to be constipated and this has been a little worse recently. There are no abnormalities to find in the cardiovascular system, abdomen or other systems. The X-ray shows a dilated fluid-filled oesophagus with no visible gastric air bubble. The oesophagus has now dilated and there has been spill-over of stagnant food into the lungs giving her the episodes of repeated respiratory infections. Such aspiration is most likely to affect the right lower lobe because of the more vertical right main bronchus, although the result of aspiration at night may depend on the position of the patient. It tends to be present for all foods, indicating a motility problem, and there may initially be some relief from the mechanical load as the oesophagus fills. The diagnosis can be made at this stage by a barium swallow showing the dilated oesopha- gus. Earlier it may require careful cine-radiology with a bolus of food impregnated with barium, or oesophageal motility studies using a catheter fitted with a number of pressure sensors to detect the abnormal motility of the oesophageal muscle. A similar condition can be produced by the protozoan parasite Trypanosoma cruzi (Chagas disease), but this is limited to South and Central America and would not be relevant to her stay in the north-west United States. Other common causes of dysphagia are benign oesophageal structures from acid reflux, malignant structures, external compression or an oesophageal pouch. Achalasia may be managed by muscle relaxants when mild, but often requires treatment to disrupt the lower oesophageal muscle by dilatation or surgery. In his abdomen the only abnormality is that his spleen is palpable 4 cm below the left costal margin. This is due to abnormal proliferation of red cell precursors derived from a single haematopoietic progenitor cell with the capacity for differentiation down red cell, white cell and platelet lines. As a result, there is an increase in haemoglobin, white cell count and platelet level. Patients may present with a throm- botic event or with symptoms due to increased blood viscosity such as headaches, tinni- tus and blurred vision. Severe pruritus is characteristic and is particularly related to warmth occurring on getting into a warm bed or bath. Conditions associated with generalized pruritus without a rash Obstructive jaundice due to bile salt retention Iron deficiency Lymphoma Carcinoma, especially bronchial Chronic renal failure, partially due to phosphate retention This patient should be referred to a haematology unit for investigation. It is important to exclude relative polycythaemia due to dehydration from diuretic and alcohol use. The red cell mass will be raised in polcythaemia rubra vera, but normal in relative polycythaemia. The erythropoietin level is low in polycythaemia rubra vera and high in secondary poly- cythaemia.