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110 Cards in this Set
- Front
- Back
what is an absolute reduction in the number of granulocytes in the blood to below 1000/mm3
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granulocytopenia (primarily a neutropenia)
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what 3 drugs are associated with drug-induced cessation of granulopoiesis
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aminopyrine, sulfonamides, and chloramphenicols
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what drug results in immune mediated cytolysis or leukoagglutination
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aminopyrine
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what is a replacement of hematopoietic tissue in the bone marrow by abnormal tissue, usually fibrous tissue or malignant tumors which are most commonly metastatic carcinomas
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myelophthisis
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what is an intrinsic neutrophil defect which allows normal proliferation of bone marrow precursor cells, but results in abnormal granulogenesis and inability to form secondary granules.
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familial neutropenia
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children affected with familial neutropenia develop what and what is their blood neutrophil count
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persistant bacterial infections. count is less than 1000/mm3
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what is an autosomal dominant condition in which no bone marrow granulocytes are produced beyond the myelocyte stage because of cyclic interruption of the production of the pluripotent bone marrow stem cells. the cycle occurs every 21-28 days, and the 4-10 day neutropenia results in illness, malaise, sore throat, gingival ulcers, and skin abscesses
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cyclic neutropenia
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aplastic anemia results in what
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suppression of myeloid stem cells
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what condition causes intrasplenic destruction or sequestration of neutrophilic leukocytes, and is also observed with rheumatoid arthritis and antineutrophil antibody production
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Felty's syndrome (hypersplenism)
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vitamin b12 and folate deficiencies result in what
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in ineffective granulopoiesis
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what condition results in antibodies produced against cell surface antigens that can result in cytolysis and or leukoagglutination. usually associated with aplastic and hemolytic anemia, and thrombocytopenia.
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idiopathic autoimmune neutropenia
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what neutropenia is due to transplacental transfer of IgG immunoglobulin produced by the mother against granulocyte surface antigens of her infant, and granulocyte survival is shortened
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isoimmune neutropenia
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what can be used to improve prognosis for granulocytopenia
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antibiotic therapy and or recombinant human granulopoietic factors
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what condition shows neutrophils that phagocytize normally but do not destroy the ingested pathogen. the defect is an absence or inactivity of the membrane-associated NADPH oxidase enzyme that generates superoxide anions. often seen in children. is autosomal recessive. and patient suffereing with the condition have recurrent infections caused by staph and gram - rods.
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chronic granulomatous disease
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what condition is a deficiency that lies with the lack of NADPH production by the hexose monophosphate shunt, and in children an increase in susceitibility to infection is seen due to the inability of neutrophils to generate the respiratory burst
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glucose 6 phosphate deficiency
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what condition in children is an autosomal recessive inheritance. it involves a delay in fusion of abnormal azurophil granules with phagolysosomes, and is characterized by dilution in color of the hair and eyes, a platelet storage defect, and an increased susceptibility to bacterial and viral diseases
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chediak-higashi syndrome
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what condition is a benign anomaly of leukocytes inherited as an autosomal dominant inheritance, with a reduced number of nuclear segments, best seen in neutrophils, where the incompletely segmented nuclei appear round or oval
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pelger-huet anomaly
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what results from a neutrophil mobility difficulty with resultant infection with capnocytophaga, an anaerobic bacillus
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juvenile periodontitis
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what condition is a failure of B-cell precursors to differentiate into mature B cells, because of a mutation in cytoplasmic tyrosine kinase. it shows a marked depression of all serum immunoglobulins, is x-linked inheritance, and shows recurrent infections with pyogenic bacteria when maternal immunoglobulins are lost.
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x-linked agammaglobulinemia of Bruton
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what condition shows a lack of germinal center in the lymph nodes, tonsils, appendix, and spleen, and the gut lacks peyers patches. also plasma cells are absent and B cell compoenent is nearly absent. this condition often develops autoimmune CT disorders
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x-linked agammaglobulinemia of Bruton
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what is used to diagnose and treat x-linked agammaglobulinemia of Bruton
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diagnosed with immunoelectrophoresis and treated with gamma globulins and antibiotics
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what is a very common selective immunodeficiency disease showing a near absence of serum and secretory IgA, causing a mucosal defensive weakining which results in infections in the gi, respiratory, and urogenital systems. symptoms inclue recurring sinopulmonary infections and diarrhea, and is treated with antibiotics
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isolated IgA deficinecy
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what form of isolated IgA deficiency results from a basic defect int he terminal differentiation of B lymphocytes to plasma cells
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hereditary form
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what form of isolated IgA deficiency results from certain viral infections
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acquired form
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what condition is a selective T-cell deficiency resulting from failure of devt of the 3rd and 4th pharyngeal pouches, the thymus is absent and a total lack of cell-mediated immune response is seen. the peripheral lymphocyte count is decreased and patients suffer from an increased susceptibility to viral and fungal infections. also paracortical areas of the spleen and lymph nodes are depleted, and can be associated with cardiovascular, cerebral, and endocrine defects
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DiGeorge syndrome
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lack of parathyroids in digeorge syndrome results in what
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tetany
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what is digeorge syndrome treated with
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fetal thymic transplants
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what condition is a defect in both T and B cells being deficient in the blood, lymph, and spleen. the thymus gland fails to descend from the neck into the anterior mediastinum and is devoid of lymphoid cells and hassalls corpuscles. IG are not produced and children suffering from this often die before age one due to infections with pseudomonas, candida or viruses
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severe combined immunodeficinecy disease
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pneumonias from severe combined immunodeficiency diseases are caused by what
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pneumocystis carinii
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what is used to treat severe combined immunodeficiency diseases
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germ-free envt, bone marrow stem cell transplants and or gene therapy
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in what condition is there a delay in the maturation of B cells and their immunologic function. the gamma globulin level is much lower than usual for that age of infant, and they suffer from overt infections, fever and bronchitis
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transient hypogammaglobulinemia
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what condition is due to deficiency in C1 esterase inhibitor, is transmitted as an autosomal dominant trait, and allows complement cascades to continue unchecked. results in recurrent episodes of edema and swelling due to an increased permeability of the vasculature. it is provoked by emotional distress or trauma
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hereditary angioneurotic edema
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what is the tx for hereditary angioneurotic edema
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c1 esterase inhibitor concentrates prepared from human plasma
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most malignant lymphomas are of what cell origin
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monoclonal B cell origin
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what NHL classification type is based on cell types, where cell types and morphology are identified with immunohistochemical cell markers
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lukes collins
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what type of lymphoma can be of african or american variety, and appears to be associated iwth the epstein barr virus. it is different from other lymphomas because it rarely arises within peripheral lymph nodes, and usually arises from mature B cells in primarily extranodal locations
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Burkitt's lymphoma
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what NHL classification system is based solely on morphologic criteria, specifically on the distribution of the neoplastic cells within the lymphoma and the cytologic characteristics of the neoplastic cells
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rappaport
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lymphoid neoplasms tend to disrupt normal immune regulatory mechanisms resulting in what
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increased susceptibility to infection and autoimmunity
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what type of lymphoma are distinguished by their distinctive morphology (Reed-sternberg cell), origin, and route of spread. more common in men than women. almost alwasy arises within lymph nodes, spreads in predictable fashion from lymph nodes to spleen to liver to bone marrow and then extranodal involvement.
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hodgkins lymphoma
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what classification system is based primarily on lymphocyte population in comparison to the histiocytes and RS cells
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rye classificaiton
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what is the most common type of hodgkins disease
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nodular sclerosis
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describe an Reed-steinberg cell
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large, often binucleated or bilobed and contains owl eyed nucleoli
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what is a malignant hematopoietic neoplasm characterize by a disordely purposeless proliferation of blood cells or their precursors, where the bone marrow is always involve, and an abnormal number of immature white blood cells are often present int he peripheral blood
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leukemia
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what type of leukemia is seen in people over 50, it has a subtle onset, a sluggish course often lasting 10 to 15 years and increased number of small lymphocytes. usually involves immunologically defective B cells, and death results from infections and organ infiltration. it is also the most common form of leukemia in adults of the western world
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chronic lymphocytic leukemia
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what type of leukemia occurs most often in children, has a sudden onset, marrow is packed with lymphoblasts, and causes death quickly if untreated. it is the most common form of cancer in children under 15.
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acute lymphoblastic leukemia
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what type of leukemia is predominated by the presence of myeloblasts, and is seen in patients 15-39
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acute myeloblastic leukemia
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what type of leukemia has onset in the 3rd and 4th decade of life, features splenomegaly without lymphadenopathy, and leukocytosis due to increased numbers of fairly mature granulocytes. also has chromosomal aberrations, especially the philadelphia chromosome
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chronic myelocytic leukemia
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what type of leukemia includes plasma cell tumors and multiple myeloma. results in gammopathies and punched out bone lesions
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plasmacytic leukemia
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splenomegaly is most common with what type of leukemia
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CML
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what is the most common developmental abnormality of the esophagus
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atresia or fistula
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what is the most common collagen disease affecting the esophagus
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scleroderma
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what are annular narrowings of the upper esophagus, often associaed iwth iron deficiency in women over 40.
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esophageal webs and rings
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the combination of iron deficiency anemia, atrophic glossitis, cheilosis, and dysphage is known as what
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plummer-vinson or patterson-brown-kelly sydrome
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what is a disorder of esophageal motility resulting from a failure of the gastroesophageal sphincter to relax and loss of normal peristalsis of the distal two thirds of the esophagus due mostly to loss of myenteric ganglion cells. leads to progressive dysphagia, regurgitation of ingested food and possibly aspiration of ingesta with resulting aspiration pneumoniae
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achalasia
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what is a disorder of the gastroesophageal junction, resulting in a saclike dilation of the stomach protruding above the diaphragm.
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hiatal hernia
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the reflux of gastric contents into the lower esophagus often induces what
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GERD
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what is an abnormal outpouching of the esophageal mucosa and submucosa through a weakness in the muscular wall usually due to an increased intraluminal pressure or eversion of all layers of the esophagus resulting from healing of an inflammatory process external to the esophagus
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diverticula
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what condition is characterized by linear longitudinal lacerations of the mucosa and submucosa involving the distal esophagus most often seen after prolonged vomiting, especially in chronic alcoholics
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mallory-weiss syndrome
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what cells secrete mucus and pepsinogen I and II
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mucous cells
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what cells secrete acid and intrinsic factor which allows absorption of vitamin B12 in the ileum)
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parietal cells
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what cells secrete the proteolytic pepsinogen I and II
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chief cells
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what cells are gastrin secreting endocrine cells
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G cells
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what do enteroendocrine cells release
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histamines
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what is a weakness or partial to total absence of a region of the diaphragm which allows the abdominal contents to herniate into the thorax. this may lead to vomiting or burping
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diaphragmatic hernias
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what is a narrowing of the pyloric lumen
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pyloric stenosis
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what condition is an acute mucosal inflammation that is usually transient. can be caused by smoking, aspirin, alcohol, and drugs. can vary from mild neutrophil infiltration to diffuse sloughing of the gastric mucosa with hemorrhage. can be associated with epigastric pain, nausea, and vamiting along with hematemesis.
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acute gastritis
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what condition is often idiopathic but can be associated iwth pernicious anemia. patients produce autoantibodies directed against intrinsic factor and gastric parietal cells. the pt develop vitamin b12 deficiency anemia, atrophy of gastric glands with resulting hypo or achlorhydria and loss of parietal cells.
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chronic gastritis
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what lesions represent extension of the acute erosive gastritis through the muscularis mucosa to become an ulcer. they are associated with extensive burns, acute brain damage, surgery, shock, asprin abuse, steroids, and smoking
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acute gastric ulceration
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what lesions are usually solitary lesions resulting from acid digestion of the mucosa and occur in the duodenum and stomac (4:1 ratio). not common before 20 yoa and more common in men than women
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chronic gastric ulcers
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which appear first: gastric ulcers or duodenal ulcers
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duodenal ulcers
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what condition is often associated with zollinger-ellison syndrome (in which individuals contain a gastrin producing tumor of the pancreatic islets)resulting hypergastrinemia and hypercholrhydria leads to gastric mucosal hyperplasia and hypertrophy and multiple gastric ulcers. gastric rugae become greatly thickened.
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chronic hypertrophic gastropathy
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what congenital anomalie of the small intestine causes intestinal obstruction that leads to persistant vomiting, usually within the first wo weeks of life, it results in failure of one or more segments of the small bowel to develop a lumen
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atresia and stenosis
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what is a herniation of the mucosa and submucosa of the small intestine through the muscle wall where the mesenteric vessels and nerves enter and provide a focus of weakness
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multiple diverticula
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what is a persistence of the omphalomesenteric duct remnant. located about 12 inches from the ileocecal valve. can contain heterotropic nests of gastric mucosa and can develop peptic ulcers and bleed. when these become infected and or inflamed they will mimic acute apendicitis
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meckels diverticulum
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what is an occlusion of either the arterial supply or the venous drainage of the small bowel, mostly related to emboli from vegetative valvular endocarditis or atherosclerosis of mesenteric vasculature. can result in transmural hemorrhagic infarction. bowel gangrene and perforation with resulting death are potential outcomes
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mesenterich thrombosis
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what is a non thrombotic intestinal infarction due to hypoperfusion of the gut wall following shock, cardiac failure, or vasoactive drug administration. can result in hemorrhagic mucosal or mural infarction. usually the serosa and subserosal tissues are spared
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acute hemorrhagic enteropathy
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what is a complete twisting of a loop of bowel about its mesenteric base of attachment leading to intestinal obstruction and infarction
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volvulus
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what condition is due to a failure of the meissners and auerbachs plexus to develop. occurs at the anorectal junction and extends proximally to a variable extent but usually is limited to the rectum or sigmoid colon. the loack of peristalsis creates a functional obstruction which results in dilation of the proximal bowel. is ten times more frequent in downs patients and four times more frequent in males
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congenital aganglionic megacolon (Hirschsprugs disease)
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what condition is a transmural, chronic relapsing granulomatous inflammatory disease which may affect any portion of the GI tract, especially the ileum and colon. caused predominantly by autoimmunity. gross morphologic lesions have a segmented arrangement (skip lesions) and include transmural fibrosis and thickening of the bowel wall, narrowing of the bowel lumen, and serpentine ulceration of the mucosal surface, fissures, and fistulas. characterized microscopically by noncaseating granuloma formation
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crohn disease
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what is the most worrisome polyp
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villous adenoma greater than 4 cm
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what is any tumorous mass that protrudes above the surface of the srrounding mucous membrane originating from mucosa or submucosa.
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polyp
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an inflammatory bowel disease of unknown etiology that oriniates in the rectus, spreads proximally, and it is characterized by mucosal ulceration, crypt, abscesses, and the absence of skip lesions
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ulcerative colitis
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the trachea is lined by what type of cell
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pseudostratified ciliated columnar epithelium
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the lower airway epithelium consist of what three primary cell types
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ciliated, reserve cells, and goblet cells
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what is ther terminal respiratory unit that includes all structures beyond the terminal bronchiole, such as the respiratory bronchiole, alveolar duct, alveolar sac, with the corresponding circulation
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acinus
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what is the pyramidal structure bordered on its sides by a discontinuous layer of pulmonary septum and is comprised of three to five acini
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primary lobule
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the cytosol of type I pneumocytes can extend up to 50 microns away from the nucleus and into what structure which connects alveoli of different lobules
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pores of Kohn
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what are the reserve cells of the alveolus that are also responsible for the production of surfactant
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type II pneumocytes
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what is the term for either an incomplete expansion of the lungs or to a collapse of previously inflated lungs, primarily involving the alveoli. most of these are reversibl
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atelectasis
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what condition is caused by severe diffuse alveolar capillary membrane injury related to numerous potential infectious and non-infectious etiologies. best characterized by edema, fibrin exudation, and hyaline membrane formation within the alveolar sacs, and type II pneumocyte proliferation. It often becomes chronic resulting in fibrosis and scarring with permanent loss of lung compliance
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adult respiratory distress syndrome
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what condition most common in women from 20-40 is involved with BMPR2 (morphogenic protein receptor 2) where the artery walls become thickened due to medial hypertrophy, elastic thickening of the intima, duplication of the interal elastic membrane, and plexogenic lesion formation.
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pulmonary hypertension and vascular sclerosis
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what group of conditions is characterized by dyspnea and results from the narrowing or obstruction of the trachebronchial tree or from destruction of the lung parenchyma.
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obstructive pulmonary disease
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what condition is an abnormal permanent enlargement of the air spaces distal to the terminal bronchiole, accompanied by destruction of their walls, without obvious fibrosis. it involves a protease-antiprotease mechanism, whereby deposition of particles or smoke at the terminal bronchioles induces an inflammatory reaction. Ultimate result is from the destructive effect of high protease activity in people with a low antiprotease activity. symptoms do not appear until at least 1/3 of the functional lung parenchyma is incapacitated, and the major disruption is the distal airway tissue destruction with resultant loss of elastic recoil rather than the large airway obstruction observed with chronic bronchitis
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emphysema
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what type of emphysema involves the respiratory bronchioles (central or proximal portion of the acinus) and spares the distal alveoli. it occurs most commonly in heavy smokers and is observed along with chronic bronchitis. represents greater than 95% of all emphysema cases
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centriacinar or centrilobular emphysema
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what type of emphysema involves all areas of the acinus. it occurs mostly in people with autosomal recessive genetically determined alpha-1-antitrypsin deficiency and is enhanced by smoking
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panacinar (panlobular) emphysema
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what type of emphysema involves only the distal portion of the acinus and spares the respiratory bronchiole. it may lead to spontaneous pneumothorax, especially in young adults
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paraseptal (distal acinar) emphysema
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what type of emphysema is often associated with scarring and has minimal clinical significance. it is often present in older adults and occurs within old scards due to previous infections
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irregular
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what condition is characerized by pts who have a persistant cough with sputum production for at least three months in at least two consecutive years with no other recognized cause. associated with chronic irritation by inhaled substanced and is exacerbated by microbiologic infections. increased mucus secretion, stasis of cilia, and decreased clearance of substances from the lungs are often clinical features.
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chronic bronchitis
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what condition involves a chronic relapsing inflammatory condition associated with widespread narrowing of the bronchial airways which changes in severity over short periods of time. occurs in 5% of children, is reversible, responds to bronchodilaters, with elevated serum IgE levels. coughing and wheezing usually result from the excess mucus production and bronchoconstriction
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bronchial asthma
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what condition is an abnormal permanent dilation of bronchi and bronchioles resulting from inflammtory damage to their walls. it is often associated with airway obstruction due to a chronic necrotizing infection. involves coughing up copious amounts of purulent sputum and is most commly found in those hereditary diseases that produce ciliary dysfunction
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bronchiectasis
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what condition results from impaired defense mechanisms of the lung, usually seen in infants and the elderly, and caused by many different bacteria.
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pneumoniae
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what type of pneumonia is characteized by a patchy consolidiation
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bronchopneumonia
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what type of pneumonia involves a large portion or an entire lung lobe.
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lobar pneumoniae
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what are the best two examples of chronic granulomatous pneumonia
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pulmonary tuberculosis and fungal infections
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what group of disorders is characterized by diffuse and usually chronic involvement of the pulmonary CT, especially the interstitium in the alveolar walls (alveolitis) resulting in loss of lung capacity. can lead to severe chronic interstitial fibrosis. causes include pneumoconiosis, hypersensitivity pneumonitis, diffuse interstitial fibrosis, and granulomatous diseases. similar pathogenesisi to ARDS but more directed at type I pneumocyte damage and resulting fibrosis. ends in honecomb lung characterized by numerous cystic spaces seperated by dense fibrous scars and often filled with mucus and cellular debris
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diffuse interstitial diseases
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what lung neoplasm makes up 95% of all lung neoplasms. it is the most common lethal visceral malignancy in both females and males. related to smoking, industrial hazards, and air pollution as well as genetic factors. can be of squamous cell, adenocarcinomic, or small cell or large cell carcinomic types
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bronchogenic carcinoma
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what type of bronchiogenic carcinoma is most often seen in smokers and which of these two has the poorest prognosis
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squamous cell and small cell carcinomas are most frequently seen in smokers and small cell carcinoma has the poorest prognosis
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what type of neoplasm represents a variant of the adenocarcinoma and usually arises from the alveolar lining cells or from terminal bronchioles
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bronchioalveolar carcinoma
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what type of neoplasm of the lungs arises from argentaffin (kulchitsky) cells
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bronchial carcinoid
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what is the most common site for metastatic cancers
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the lungs
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