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114 Cards in this Set
- Front
- Back
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What is amyloidosis? |
Chronic, metabolic disorder where amyloid, a fibrous protein, is deposited through |
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What organ systems does amyloidosis usually affect? |
Kidneys, liver, heart |
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Who is at more risk for amyloidosis? |
Men 2:1 ratio AA at higher risk from cardiac complications Survival rate 1-4 years Average age at diagnosis 65 Link between alzheimers Immune and genetic contributing factors |
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What are some signs and symptoms that may make you suspect amyloidosis? |
Enlarged heart on x ray, arrythmias, difficulty breathing, speech and swallowing difficulties, tounge enlargement (macroglossia), facial deposits, weakness, fainting, constipation or malabsorption issues if GI component, bleeding complications |
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How do you diagnosis Amyloidosis? |
Take a tissue biopsy from involved organ system Or tissue biopsy from rectal mucosa or abdominal fat pads, skin, gums Test for amyloid with Congo red staining technique Other tests that may clue in to diagnosis: EKG, Serum alk phos, UA, BUN/CR, serum protein electrophoresis or urine electrophoresis |
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How do you treat amyloidosis? |
Symptomatic, no cure! Secure airway and address swallowing if tounge involvement. If renal involvement, bilateral nephrectomy/renal transplant, BP control. Dietary consult and ST consult. TREAT: Melphalan and Prednisone Stem Cell Transplant |
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What is multiple myeloma? |
MM results from malignant neoplasm of marrow plasma cells that infiltrate bone and cause osteolytic lesions throughout skeleton. Plasma cells make too much M protein and free light chain proteins, causing increased blood viscosity (impaired clotting) |
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What types of patients are at increased risk for multiple myeloma? |
Older adults Risk with radiation, overweight, family history 2:1 ratio men to women 2:1 ratio blacks to whites |
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What are the signs and symptoms of multiple myeloma? |
Hypercalcemia Renal disfunction Anemia Bone Pain/Back pain |
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What are some complications of multiple myeloma? |
Fractures, renal failure, hypercalcemia, hyperuricemia, dehydration, GIB, PNA, Renal calculi |
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How do you diagnose Mutliple Myeloma? |
Signs and symptoms Bone Marrow biopsy shows > 10% plasma cells Monoclonal protein spike on SPEP (serum protein electrophoresis) or UPEP Anemia on CBC ESR elecation UA with Bence-Jones proteins Skeletal survey with lytic bone lesions X rays |
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How is MM treated? |
Chemo/radiation Laminectomy for SC compression Dialysis Bone marrow transplant Melphalan and prednisone Treat hypercalcemia: hydration, loop diuretics, corticosteroids, oral phosphate or IV Biphosphonates Medium survival is 3 years |
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How do you treat hypercalcemia? |
Hydration, loop diuretics, oral phosphate or IV Bisphosphonates, Corticosteroids |
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What does your lymph system do? Name the analogy used in video. |
Lymph system can be compared to whenyou are at the airport and you randomly getpulled out of line to go to a smaller slower lineto get screened and patted down and then go backwith everyone else.Your lymph system pulls out fluid and lymphocytesexamine to see if there is any danger and then returnsto your systemic circulation via the IJ and SC veins. |
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What are some accessory organs (primary and secondary) to the lymph system? |
Thymus: T cell maturation Bone Marrow: B cell maturation Secondary: Lymph nodes, spleen, mucosal lymphoid tissues in lung/gut |
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What is innate immunity? |
Skin and mucosal linings Inflammation, fever Phagocytes, like neutrophils and macrophages Natural Killer Cells Dendritic cells |
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What is adaptive immunity? |
The part of your immune system that is able to remember specific pathogens and cause a systemic response Includes Humoral and Cellular System |
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What is the humoral system? |
Include antibodies and B cells Attacks pathogens that have not entered cell yet. Antibodies make memory B cells and effector cells when they come in contact with a pathogen. Antibodies dont kill pathogens directly, but they tag them and alert phagocytes to kill them. B cells recognize antigen and digests it,binds to MHC molecule, attracts matching T cells that help B cells proliferate into antibodies. |
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What is the cell mediated response? |
Involves T cells Kicks in once infection continues and takes over body's cells Phagocytes break up offenders and wear as MHC to show as antigen presenting cells. Macrophages, dendritic cells, and b cells wear MHC2 T cells work by recognizing MHC Helper T cells: Activate cells that kill pathogens, activate B cells as a check and balance to make sure antibodies dont go crazy and attack cells they shouldnt Cytotoxic T cells kill bad cells |
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What do antibodies do? |
Neutralize toxins and viruses by blocking binding sites on toxins and viruses so they cant hook up to bodys cells Opsonization: bind pathogens for recognition by other immune cells |
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What are some consequences of the immune system going wrong? |
Infection, Allergies, immune deficiencies, autoimmune disease, contact dermatitis, transplant rejection, transfusion reactions |
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Name ways of aquiring: Passive natural Passive artficial Acitve Artificial And Active natural immunitiy |
Breastfeeding iga, invetro igg IGG, such as with ebola Vaccines Get infection! |
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What immunizations utilize passive immunity? |
Dipheria, tetanus Gas ganrene Botulism Snake/scorpion sting Rabies Hypogammaglobulinemia |
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What are the risks with passive immunization? |
No long term protection, serum sickness, risk of hepatitis/aids, graft versus host disease |
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What are some examples of live vaccines? |
Polio, not standard vaccine MMR Varicella Zoster TB Yellow fever Hep A |
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What is a type I Immune reaction? What is it mediated by? What are some examples? |
Anaphylaxis, Allergies IGE 1-30 min onset First, antigen introduced, IGE stimulated, binds to mast cells Reaction when antigen binds to IGE on mast cells, causes degranulation and mediator release |
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Explain immediate and late phase reactions and chemical mediators following Type I Immune reaction. |
Immediate: release of histamine, prostaglandins, swelling Late Phase: Leukotrines, cytokines, chemickines, smooth muscle contraction, edema, inflammation |
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what diagnostic tests can be done in allergies? |
CBC diff, UA, ESR, ANA, thyrotropin, creatinine Screening for hematological abnoramlitiesi, thyroid, renal dysfunction Prick and intradermal test IGE adn IGE antibodies against suspeted allergies with ELIZA rest RAST testing, radioallergosorbent |
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How do you treat allergic reaction? |
Avoid allergen Epi IM 0.3-.5 Preferred in thigh! Antihistamines: Hydroxysine or Diphenhydramine Solumedrol IV Pepcid IV or PO Volume resuscitation, Pressors |
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What is an example of a primary immune deficiency? |
Usually inherited IGA immunodeficiency (since IGA is found in mucosal secretions, can lead to diarrhea and resp tract infections/sinus infections) Severe Combined immunodeficiency |
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What is an example of a seconary immune deficiency? |
Drugs, cancers, or infections |
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Give some examples of people who are immunocompromised secondary to diseases.`` |
Infections, HIV, toxins, immunosuppression Malignancy Malnutrition Splenectomy Metabolic: DM, Chronic renal failure Burns, trauma, surgery, anasthesia, elderly |
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What are some iatrogenic causes of immune deficiency? |
Steroids, cytotoxic drugs ALG Ionizing radiation Tacrolimus, cyclosporin |
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What are differential diagnoses or causes for chest pain? |
ACS, Thoracic aortic dissection, pneumo, PE, imitrex, cocaine, viagra, pancreatitis, herpes zoster, indigestion Order: Chest x ray, 12 lead, D dimer, VQ scan, CT, Lower extremitry doppler |
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What drugs can cause constipation? |
Ca channel blockers, calcium supplements, narcotics, iron supplements, aluminum containing antacids, antiparkinsons drugs, NSAIDs, anticholinergics |
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What are some differential diagnoses for cough? |
PNA, ACe inhibitors, heart disease, hiv, postnasal gtt syndrome from rhinosinusitus, GERD, asthma, upper airway cough syndrome, PE, cold, flu, broncitis, acute COPD exacerbation, viral syndrome, irritant, UACS, asthma, cancer, allergies |
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What are some differential diagnoses for headache? |
Migraine, Cluster, Tension, GCA, SAH, Encephalitis, meningitis, stroke |
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What are the differential diagnoses for hematuria? |
Infection, glomerular disease, kidney stone, bladder cancer, trauma, anatomical defects, anticoagulation therapy |
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Differential diagnoses for hemoptysis |
Airway disease, trauma, pulmonary infections, inflammation, cardiac disease, cocaine use, meds, tb |
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What are some pharmaceudical interventions for joint pain? |
Tylenol, NSAIDS, antirheumatic drugs, biological response modifiers, intraarticular injections |
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Differential for peripheral edema |
DVT, Ruptured bakers cyst, compartment syndrome, allergic reaction, trauma, burn, renal failure, CHF, venous insufficiency, lymphadema, pelvic tumor, chf |
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What can cause pruritis? |
Autoimmune diseases, DM, iron deficiency, CKD, liver disease, tumors, HIV, meds (Ca channel blockers, ACE inhibitors) |
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What are some potential causes of syncope? |
Basiliar artery insufficiency, metabolic disorders, hypoglycemia, hypoxemia, orthostatics, arrythmias, head disease, dehydration Check ECG, echo, EP studies, chest x ray, electroencephalogram and CT, tilt table testing, lab tests |
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What are some differentials for tremor? |
Physiological tremor is seen in everyone but hard to see at times. Enhanced physiological tremor is caused by stress, fatigue, exercise, cold and hungry., stimulants, electrolyte disturbances Essential tremor affects hands and forearms and is persistent, treat with b blocker, anticonvulstant, benzos. Parkinsonian: assymterical tremor, goes away in sleem, alternating tremor of thumb against index finger seen Cerebral tumors Wilsons disease, opthomologist |
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What are the 5 C's to consider prior to transplantation? |
Caregiver, Cost, Coping, Compliance, Commitment |
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What are complications post transplant? |
Infection, Acute/Chronic Rejection, side effects from medication, technical problems from surgery, such as anastomosis issues |
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What medications are used post transplant? |
Immunosuppressives: Calceurin inhibitors (Tacrolimus, cyclosporin) Cell cycle inhibitors (Imuran, Mycophenolate) Steroids (Prednisone, Methylprenisone) Antimicrobial prophylaxis (Valgancyclovir,Bactrim, Itraconazole) Sirolimus is an immunosupressive that has shown benefits in preventing skin cancers, but it can cause decreased healing early on in tranplant |
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How do you ID rejection? |
Decreased energy, decreased LFTS (esp FEV1), SOB, Decreased IS, infections, dyspnea, pyrexia, hypoxemia, pleural effusions, pulmonary infiltrates |
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Types of rejection |
A: Vascular rejection B: Airway rejection Identified on biospy after bronch |
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How do you treat rejection? |
Pulse dosing of steroids for a few days Changes in Immunosuppressive regiment, adding MTOR inhibitor Azithro GERD treatment Photopheresis or thymoglobulin |
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Name some opportunistic infections post lung transplant |
CMV, RSV, Flu, MRSA, Fungal infections like aspergillis, Psuedomonas |
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What are some screening tests done prior to transplantation? |
HLA matching, Panel reactive antibody screening, Serologies and ID screening (CMV, IGG/M antibodies), HIV, Hepatitis panel, PSA, UA, Pregnancy test |
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What is MHC? |
Major histocombatibility complex Group of genetic loci located on short arm of chromosome 6 that creates HLAs |
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What is HLA? |
Human leukocyte antigens, recognize self and foreign cells |
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What are cytokines? |
Proteins that induce the cell mediated and humoral responses |
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Indications for heart transplant |
Cardiac tumor, congenital deffects, valvular disease, cardiomyopathy, myocarditis |
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What are signs of acute rejection post heart transplantation? |
Heart failure signs, dysrhythmias, fever |
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What is cardiac allograft/Coronary artery vasculopathy? |
Leading cause of death in cardiac transplant Accelerated form of CAD, diagnosed on angio, presents as heart failure, ventricular dysryhmias Dont have chest pain because new heart is denervated and wont respond to CV meds like digoxin and atropine Do respond to inotropes |
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Bronchiolitis Obliterans Syndrome |
Form of chronic airway rejection Progressive obstructive ventilatory defect with air trapping Fall in FEV1 and Rise in FVC R/o other causes of this like acute cellular rejection, bronchial anastomotic narrowing, infection |
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Restrictive Allograft Dysfunction |
Progressive restrictive ventilatory defect with air trapping Fall in FEV1 and VC/TLC |
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Why may someone need renal transplant? |
Polycystic kidney disease, glomerulonephritis, diabetic nephropathy, HTN, SLE, IgA nephropathy |
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What are some post operative complications with renal transplant? |
Bleeding, ATN, hyperkalemia, Urine leaks, ureteral obstruction, graft thrombosis, lymphocele, rejection, infection (BK type polyoma vius, UTI), Delayed graft function |
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What are some signs of acute rejection in kidney transplant? |
Pyrexia, tenderness over graft site, olifuria, acute renal failure, increasing BUN/Creatinine, weight gain, HTN, edema, malaise |
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What are some post operative restrictions after kidney transplant? |
Dont drive for 4 weeks No exercise or lifting No undue stress to joints due to risk for avascular necrosis with steroids Avoid crowded areas, birds, litter boxes CV diet |
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Reasons someone may need liver transplant |
ETOH abuse, chronic hepatitis, cholestatic disease (Primary biliary cirrhosis, primary scleorsing cholangitis), familial hypercholesterolemia, hepatocellular carcinoma, wilsons disease (too much copper), hemochromatosis |
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Signs of liver rejection |
Decreased bile production, possible perigraft tenderness, increasing LFTs, changes in coags, fatigue, dark colored urine, ascites, jaundice, pruitis |
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Hepatic artery stenosis |
complication of liver transplant Associated with bile duct leaks, intrahepatic abcesses and graft necrosis Diagnosis on ultrasound or CT scan of liver Treat with thrombectomy, thrombolytics or regrafting |
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Portal Vein Thrombosis |
complication of liver transplant may lead to development of portal htn and esophageal varices Treat by managing portal htn |
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Anastomotic bile structure strictures |
surgical repair necessary after liver transplant |
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Primary graft dysfunction after liver transplant |
graft failure to function in first weeks after transplantantation signs include acidosis, elevated LFTs, encephalopathy, poor bile duct production (elevated T bili) with jaundice Poor prognosis if this happens Treat with mucomyst, prostaglandins, retransplantation |
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What are some complications that can occur after liver transplantation? |
Rejection Infection Biliary leaks (percutaneous or surgical drainage) Problems with hemostasis due to abnormal clotting factors Electrolyte imbalances: hyperglycemia, hyperkalemia, metabolic alkalosis, Ca/Phos/Mg |
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Biliary cast syndrome |
complication of liver transplant Bile duct stricture and clogging of billiary trees with cast sludge Symptoms of SEVERE pruritis If this happens, retransplanation and or perc cholianigiography |
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What are some reasons someone may need a pancreas transplant? |
End stage DM, DM type 1 |
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What is the function of the pancreas? |
It is both an endocrine and exocrine organ. Endocrine: makes insulin and glucagon Exocrine: Makes digestive substances |
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Explain how bleeding manifestations may manifest after pancreas transplant and why |
The new pancreas is anastatomosed to the bladder or small intestine for drainage Bleeding in urine or GIB may be complication of transplant |
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What are some things that may clue you into thinking there is acute rejection after pancreas transplant? |
Abdominal pain, hyperglycemia (LATE SiGN) bleeding, increasing amylase/lipase, sepsis (most frequent in pancreas of all other transplants), urinary amylase levels |
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Why would someone need an intestinal transplant? |
Chron's, gastochisis, necrotizing entercolitis, radiation enteritis, stenosis of small bowel, chronic intestinal pseudo-obstruction syndrome |
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What are some complications specific to intestinal transplant? |
Bowel obstruction, hypermotility and diarrhea |
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Why would someone need a stem cell transplant? |
Malignancies (Leukemias, MDS, hodgkins lymphoma, nonhodgkins lymphoma, multiple myeloma, selected solid tumors) Nonmalignancies (Aplastics, Sickle cell, Fanconi's anemia, metabolic disorders, immunodeficiency disorders |
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What are some complications after stem cell transplant? |
Infection (neutropenia/immunosuppression) Veno-occlusive disease of liver: complication of conditioning; hyperbilirubinemia, rapid weight gain, ascites, RUQ pain, hepatomegaly, splenomegaly, jaundice |
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What are signs of graft versus host disease? |
maculopapular skin rash, n/v/d/abdominal pain, increasing Tbili, anorexia, mucositis, GI enteritis, liver dysfunction Chronic: Lichen planus rash, cuteanous manifestations of scleroderma, dry mouth, sclerosis of GI tract |
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What systems are most affected as target organs in acute graft versus host disease? |
Skin, GI tract, liver |
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How do you treat Graft versus host disease? |
Depends on Severity If GRADE 1 < 50% skin surface rash and organ involvement, topical steroids and PPX with Cyclosporin, Methotrexate If GRADE 2, Add sysetmic IV steroids, solumedrol; if steroids dont help add second line agents like Cellcept, photophoresis, Etanercept, Sirolimus, Pentostatin |
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CMV |
Ganciclovir |
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TB |
4 drug regimen for one year INH, Rifampin, Ethambutol, Pyrazinamide |
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Listeria |
Ampicillin IV |
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Nocardia |
High dose PCN, consult ID Con spread to brain and lungs |
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Aspergillis |
Abelcet, Voriconazole |
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Pseudomonas |
Zosyn, Cefepime If resistant, Aminoglycosides with Cephalosporins, Meropenum |
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MRSA |
Vanc IF VRE, Linezolid or Daptomycin |
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PCP, PJP |
Bactrim If allergic: Dapson/Atovaquone Dapsone can cause hemolytic anemia, check for g6PD deficiency before stating |
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Mycobacterium Avium Complex |
2-3 drugs for 1 year Macrolides Ethambutol Rifamycins Aminoglycosides |
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Hypertension after Organ transplantation: What are causes and what is best treatment? |
Preexisting disease, calceurin inhibitors (Tacrolimus and cyclosporin) and steroids Treatment with Norvasc |
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What are causes of DM after transplantation and what is best treatment? |
Secondary to steroids and calceurin inhibitors Treat with Lantus and aspart, not orals! |
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Hyperlipidemia after organ transplantation |
IS, especially sirolimus, can increase lipids Treat with Crestor, Lipitor |
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DVT/PE after organ transplantation |
Daily ASA Avoid HRT, appetite stimulants, birth control |
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CKD/Renal insufficiency avoidance and treatment after organ transplant |
Run lower levels of IS if possible, monitor calceurin levels, manage DM/HTN |
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Osteopenia/Osteoporosis after Organ transplant: Why? And treatment? |
Steroid use DEXA scans recommended annually Minimize steroid use if able; Calcium/Vitamin D supplementation, Biphosphonate therapy |
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Malignancies after transplantation |
Lymphoma, Skin Ca, Kaposi sarcoma Reccrrent EBV viremia can increase risk or high doses of Prograf or cyclosporin |
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Calcineurin inhibitors Name some. How do they work? |
Cyclosporin, Tacrolimus Inhibit IL2 production by T cells |
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What are some side effects of calceurin inhibitors? |
Adverse: tremors, encephalopathy, posterior reversible encephalopathy syndrome, renal dysfunction, electrolyte abnormalities, hyperglycemia, htn |
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What drugs interact with calceurin inhibitors? |
Increased levels: Azoles, macrolides, CCB Decrease levels: AEDs, Riframpin |
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Antimetabolites: Name some |
Azathioprine (Imuran) Mycophenolate mofetil(CellCept) Mycophenolate acid (Myfortic |
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What are some side effects of antimetabolites? |
GI effects, especially with cellcept Skin cancers, leukopenia, hepatotoxicity, neoplasia, GI upset Stop if SCC |
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MTOR inhibitors |
Sirolimus, Everolimus |
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What are side effects of Mtor inhibitors? |
Check lipid panel first, can increase cholesterol and triglycerides and cause liver dysfunction Rapa lung, hypersensitivity pneumonitis, can occur. Stop around surgery, can decrease healing timesRash, edema, HLD, low electrolytes, elevated LFTS, thrombocytopenia, fatigue, fever, hyperglycemia, anorexia, htn, gi effects, myalgias |
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Calceurin inhibitors |
tacrolimus cyclosporine |
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side effects calceurin inhibitors |
renal toxicity, PRES/encephalopathy, electrolyte abnormalities, hyperglycemia, htn |
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Increased levels of calceurin inhibitors |
zoles, macrolides (azithro), CCB, grapefruit juice |
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Decreased levels calceurin inhibitors |
AEDs, Rifabutin, Rifampin |
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Cell cycle inhibitors |
Mycophenolate Imuran |
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S/e cell cycle inhibitors |
Hepatotoxicity, increased risk for skin cancers, leukopenia, GI upset |
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Steroid side effects |
hyperglycemia, fluid retention, delayed wound healing, aseptic necrosis of femoral head, PUD |
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What are risks with sirolimus? |
Risk for delayed wound healing Increased lipids Decreased risk of skin cancer Hypersensitivity pneumontitis, rappa lung Thrombocytopenia, faitgue, fever, myalgias |
