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100 Cards in this Set
- Front
- Back
Nephron
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-functioning part if the kidney
-nephron filters filter the blood into the kidney (Loop of Henly) **glomerulus forms ultrafiltrate **tubules resorb and secrete fluids and electrolytes |
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Kidney
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-Controls electrolytes and fluids
-Acid/Base balance -Gets rid of end products of metabolism |
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Azotemia
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-Uremia
-Back up of fluids, electrolytes, and nitrogen due to improper kidney function |
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Endocrine function of the kidney
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-1,25 diOH cholecalciferol--->is hydroxylated in the kidney and sent to the gut (improper function can cause bone problems and vit. D deficiency)
-erythropoietin ---> can decreased from from reduced levels of RBC's causing anemia -renin --> angiotensin (vasodialator of blood vessels) and aldosterone controls fluids |
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Nephrotic Syndrome
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-Glomueral disease
-Large amounts of protein are lost in the urine -Causes hypoalbuminemia which leads to edema, hypercholesterolemia, hypercoagulability, and abnormal bone metabolism |
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Nutrition Care for nephrotic syndrome
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-Protein - 0.8 -1.0 g/kg DesiredBodyWeight
50-60% HighBiologicalValue protein (to increase plasma albumin and control edema) -Energy - ~35 kcal/kg/day -Na - use modest restriction ~3 g/day -Cholesterol lowering diet |
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Acute glomerulonephritides
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-Usually in children or young adults due to streptococcal infections
-Characterized by hematuria, hypertension, mild loss of renal function -Nutrition care: reduce Na in hypertension. |
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Chronic glomerulonephritis may lead to...
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-Uremic symptoms, nephrotic syndrome or EndStageRenalDisease
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Tubule disorders
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-Acute renal failure
-Characterized by sudden reduction in GlomerularFiltrationRate and altered ability to excrete waste |
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Three categories of tubule disorders
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-Prerenal = impaired blood flow to kidneys due to hypovolumia, hypotension, dehydration or cardiovascular failure
-Postrenal = obstruction in urine flow due to bladder or prostate cancer or stones -Intrarenal (intrinsic) = damage to the parenchyma due to ischemia (shock, trauma, or surgical accident) and nephrotoxic drugs |
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Phases of acute renal failure
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-Oliguric = urine output less than 500 ml Increased BUN, creatinine, K, P, Mg Decreased Na, CO2, Ca, hemoglobin
-Diuretic = increased urine output 150-200%. Risk of dehydration, electrolyte imbalance, decreased K, increased or decreased Na -Convalescent = with reversible ARF, Renal function normalizes, lab values become stable |
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Nutrition care for acute renal failure (TPN)
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***Uremia, metabolic acidosis, fluid and electrolyte imbalance must be balanced against increases in protein needs due to stress
-Often begin with TPN --> glucose, lipid and EAA and NEAA (TPN decreases protein catabolism and urea production to minimum until patient can take oral food) |
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Nutrition care for acute renal failure (oral)
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-Protein - 0.5-0.8 g/kg nondialysis 1.0-2.0 g/kg dialysis
0.8-1.0 g/kg stable patient before renal function returns -Energy - 30-40 kcal/kg, use indirect calorimetry -Potassium - 30-50 meq/day -Sodium - 20-40 meq/day -Fluid - 500 ml plus output from prior day -Phosphorus - limit as needed -Calcium - increase as needed |
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Chronic renal failure
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-Congenital disorder, virus, or something not taken care of
-Kidney attempts to adapt to decreased function, adaptation leads to increased pressure which may lead to nephron failure (What's left tries to work harder --> lose ability filter) -Once half to two-thirds of kidney function is lost, progressive failure usually occurs thought due to increased glomerular pressure (thickening of membrane occurs from trying to work hard) -Controversy as to whether protein restriction may slow process -See malaise, nausea, vomiting, muscle cramps, neurologic change |
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Pluritus
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-Nitrogen compounds can cause itching
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Nutrition Care for Pre-End Stage or Progressive Renal Disease
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***For people not on dialysis
-Protein-0.8 g/kg/day 60% HBV for GFR of 55 ml/min 0.6 g/kg/day 60% HBV for GFR of 25 to 50 ml/min -Calories-35 kcal/kg -Systemic hypertension must be controlled -Blood glucose must be controlled -Evaluate protein restriction against possible protein malnutrition |
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Nutrition Goals for chronic kidney disease
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-Prevent nutritional deficiencies
-Control edema -Prevent renal osteodystrophy and vascular calcification -Provide for a palatable diet |
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What to do for people at different stages of kidney failure
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-Stage 1,2 = people are watched (not too much can be done)
~Keep well nourished, not excessive protein, control fluids and electrolytes, give vit.D and Fe (may have small amounts of protein in the urine) -Stage 3,4 = Medications --> diuretics to get rid of Na and N -Stage 5 = kidney transplant or dialysis (increase protein amount by 1.4 g/kg ~Healthy diet and lifestyle |
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Types of Dialysis during end stage renal disease
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-Hemodialysis = Fistula connects artery to vein. Blood drawn off into dialysis machine where blood is reduced in wastes by osmosis across the membrane and then returned to body
~Takes 3-5 hours (slow), more efficient machines take less time ~Control Na,K,Water, and protein intake ***blood flow to lower concentration solution (concentraion gradient is not high) -Peritoneal (no machine) = Semipermeable membrane in peritoneum acts as filter. Filtrate placed in peritoneal cavity, allowed to equalize and then drawn off. (1 liter) ~Takes 10-12 hours Continuous ambulatory peritoneal dialysis (CAPD) -->can use while walking around ** Lastly = Transplantation - donor or cadaver |
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Metastatic Calcification
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-Deposition of serum calcium-phosphate product in soft tissues due to hyperphosphatemia and PTH causing Ca resorption form bone and renal resorption of Ca
***seen in CKD |
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Osteitis Fibrosa
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-Bone inflammation due to increased PTH
***seen in CKD |
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Causes of Nephrolithiasis
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***Kidney stones
-Unknown -Increased parathyroid secretion with loss of CaPO4 in urine, immobilization, infections, inadequate fluid, or lesions which obstruct flow |
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Types of Nephrolithiasis
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-Calcium oxalate = common
-Uric acid = associated with gout -Cystinine = rare metabolic disorder -Struvite = associated with urinary tract infections -Calcium-phosphate = renal acidosis |
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Treatment for Nephrolithiasis
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-Encourage fluids - 1.5-3.0 liters/day
-Increase Ca intake to bind oxalate and reduce oxalate excretion ~avoiding rhubarb, spinach, strawberries, chocolate, wheat bran, nuts, beets, and tea. -Alter acidity or alkalinity of urine -Dissolution of uric acid stones at 6.5 pH -Dissolution of cystine stones at 7-7.5 pH -Dissolution of CaPO4 stones at 5.5 pH |
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Acid-Alkaline Potential of Foods
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-Meat, Egg - acid ash due to H2SO4, H2PO4
-Cereals, Bread - acid ash due to P, Fe, S -Corn, lentils, cranberries, plums – acid ash -Milk - fairly high alkaline ash -Fruit, Vegetables - alkaline due to K, Ca, Mg -Usually citrate or bicarbonate use to increase alkalinity ***Cranberry and blueberry juice may inhibit adherence of E coli bacteria in urinary tract infections but does not effect pH for kidney stones. Cranberry concentrate pills increase urinary oxalate, sodium and calcium |
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Early starvation
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1. Glycogen used first***
2. Glucose from hydrolysis of skeletal muscle 3. Gluconeogenesis ~75 g. protein lost/day ~160 g. TGL lost/day |
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Prolonged starvation
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-Adaptive mechanism
-20 g. protein lost /day -150 g. TGL lost/day |
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Hypermetabolism
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-Metabolic Response to Stress, gun shot wounds, surgery, MVA, burns and sepsis
-Characterized by an accelerated catabolism of LBM or skeletal mass that results in negative N balance and muscle wasting -Duration and extent depends on general health of the individual and the severity of the trauma ***adaptive state doesn't occur |
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Ebb period
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-immediately after injury.
-Characterized by hypovolemia (excessive bleeding, skin exposed from burn victim, or lose blood from surgery), shock and tissue hypoxia (less O2 delivered to tissue) **This is due to decreased cardiac output, oxygen consumption and body temperature -Also see increased blood sugar due to decreased insulin and increased glucagon ***this is when the catabolic phase starts |
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Anabolic phase
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-Body lays down protein
-Increase in muscle mass |
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Flow period
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-Catabolism predominates
-Follows fluid resuscitation and restoration of oxygen transport. -Characterized by increased cardiac output, oxygen consumption, body temperature, energy expenditure, total body protein catabolism and mineral losses. **Negative N balance = decreased muscle mass |
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Hormonal Changes due to metabolic stress
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-Stress causes production of a stimulatory mediator, this acts on the hypothalamus causing hormonal response (ACTH) leading to increased metabolic rate.
-Increased cortisol - mobilizes a.a. from skeletal muscle (breakdown of a.a. = Addison's disease) -Increased catecholamines - stimulates glycogenolysis (makes glucose from protein), fat mobilization (makes glucose from fat sources), and gluconeogenesis -Increased ADH - causes renal water resorption -Increased aldosterone - renal Na retention |
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Cytokines in metabolic stress
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-Interleukin-1
-Interleukin-6 -Tumor necrosis factor **These are released from phagocytic cells with tissue damage, infection or inflammation. **They stimulate hepatic amino acid uptake, protein synthesis (acute phase proteins such as c-reactive protein, fibronectin, ceruloplasmin, serum amyloid A) **They accelerate muscle breakdown and induce gluconeogenesis. |
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Nutrition assessment in metabolic stress
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**Techniques may not be useful or possible in severe metabolic stress or in organ failure due to edema, severity of wound, inability of patient to provide information (use a reported weight)
-Things will be highly concentrated and will mess up numbers due to hypovolemia and edema **Objectives 1. Correct preexisting malnutrition 2. Prevent progressive protein calories malnutrition 3. Optimize metabolic state by managing fluid and electrolytes |
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Energy and protein for metabolic stress
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-25-30 nonprotein calories/kg/day
-60-70% of nonprotein calories from CHO -15-40% of nonprotein calories from fat - 1.5-2.0 g/kg/day 100:1 nonprotein kcal/g N BCAA **Increase N retention **Increase hepatic protein synthesis **Decrease protein degradation **Achieve N equilibrium in less time **Glutamine-aids enterocyte production |
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Nutrients with metabolic stress
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-Dietary fiber maintains colon integrity
-Arginine may be an EAA in stress, but some studies show negative effects in sepsis -Fermented soluble fibers form SCFA (buterate) --> colon integrity -Omega-3 fatty acids may aid in general and GI inflammation |
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Moving from catabolism to anabolism
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-Monoclonal antibodies to block cytokine response
-Provision of specific a.a. such as glutamine, arginine, or BCAA -Modify immune response with n-3 fatty acids -Administration of growth hormone to promote protein synthesis |
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Multiple organ failure
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-Syndrome usually begins with lung failure (ARDS)
-Followed by liver (jaundice and hepatocyte damage) -Intestine (ileus = no movement in the G.I. tract and stress ulceration) -Kidney (acute oliguric renal failure) failure ***organ muscle retained over skeletal muscle |
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SIRS (systemic inflammatory response system) diagnosis
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***Needs any of the 2 following
-Temperature >38oC or <36oC -Heart rate >90 beats/min -Respiratory rate >20 breaths/min -Partial pressure of PCO2 <32 mm Hg -Leukocytosis (white blood cell [WBC] count >12,000mcL-1) -Leukopenia (WBC count <4000 mcL-1) -Normal WBC with >10% immature cells ******WBC count shows infection or inflammation |
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Glascow Coma Scale
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-14-15 minor
- 9-13 moderate - < 8 severe injury |
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Nutrient needs for head injury trauma
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-Energy = 40 % above Harris-Benedict prediction
-Protein = 1.5 - 2.2 g/kg -Control cerebral swelling |
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Degrees of burn
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-First degree = only epidermis, characterized by erythema, pain, some edema
-Second degree = all epidemis and portions of dermis, characterized by blisters and edema. Partial thickness burn. -Third degree = entire dermis down to subcutaneous fat, characterized by tissue thrombosis, eschar, loss of skin function. Full thickness burn |
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Eschar
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-Burned tissue which needs to be removed before new synthesis can occur
**fluid and electrolyte balance can be a huge problem if its not taken care of |
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What % of BSA causes severe alteration in metabolic response?
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>20%
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Phases of treatment for burn victims
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-Shock phase = fluid replacement by IV
~colloids = protein like compounds put in the blood to keep fluid in the blood stream ~4 ml Ringer’s lactate X wt (kg) X 5 BSA burned -Hypermetabolic = until all skin is grafted and wound covered ~Curreri formula -->(24 kcal/kg IBW) + (40 kcal X % BSA burned) = total kcal /day ~ 1 g/kg IBW) + (3 g X % BSA burned) = total g/day --> Glutamine and arginine ~2-3 g protein/kg or 20-25% of calories has been recommended -Anabolic phase |
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Nutrition effects of cancer cachexia
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-Cytokines and protein hormones (TNF, interleukins and interferon-a) may be mediators of cachexia syndrome
-Poor food intake at a time of heightened nutrient demands leads to muscle wasting and general poor health which further contribute to inadequate nutrient intake ***cytokines are thought to be a cause to the decrease of appetite |
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Anorexia and malnutrition
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- >5% weight loss shows loss of LBM, increased protein turnover, less fat mobilization
- Advanced cancer-40% increased energy needs - Energy needs in cancer can increase, decrease or stay the same |
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What is the tumor's source of nutrients?
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-Preferentially uses glucose especially from gluconeogenesis (100 g/day)
***can form lactate - Cori cycle-energy consumption pathway --> takes more energy then making pyruvate thru normal glycolysis |
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What can tumors deplete?
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-Glutamine -->this causes the breakdown of mucosal cells --> bacteria into the blood strea --> spesis
(Bacterial translocation) |
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Types of treatment for tumors
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-Radiation = local
-Chemotherapy = systemic -Surgery = local -Bone marrow transplant = stem cells can boost the immune system (given healthy cells) ~"harvest" stem cells by drawing them out of the blood of the patient or a donor (can freeze the stem cells) -->lower the person's immune system so they do not reject the new cells (can not eat raw foods after treatment) -Immunotherapy ***G.I. tumors = primarily the ones to do surgery on ***Treatment can kill normal cells as well ***Top 3 ***Interferes with mitosis or cell division and attempts to destroy tumors and cancer cells |
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Effects of Chemotherapy on nutrition
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-Food intake affected by mucositis, cheilosis (cracks on the corners of the mouth-->can be caused by riboflavin deficiency), glossitis, stomatitis, esophagitis
-Nausea and vomiting controlled by antiemetics -Taste changes |
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Effects of Radiation on nutrition
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-Can cause inflammation and decreased nutrient absorption
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Effects of surgery on nutrition
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-Food intake affected by absorption -Dumping syndrome
-Availability of pancreatic enzymes (surgery on the pancreas can reduce the enzymes produced) |
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Effects of Immunotherapy on nutrition
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-Fatigue
-Chills -Fever -Flu-like symptoms |
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Effects of Bone marrow transplant on nutrition
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-Nausea
-Vomiting -Diarrhea in acute phase and inflammation later |
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Nutrition for a cancer patient undergoing treatment
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-Provide adequate nutrition
-Food will feed the body to keep it strong thru treatment regardless of the tumor getting the nutrients from the patient eating -ABW = absolute body weight/desirable body weight |
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How tom improve oral intake of cancer patients
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-Good counseling can increase intake by 450 kcal/day
-Breakfast seems to be best meal -Increase protein and fat, small meals -Enhance flavors (aversion to the taste of meat --> amino acids taste metallic) -Provide patient with sense of control -Reglan decreases gastric emptying time -Progressive --> increase appetite, weight gain -Use antidepressants |
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Terminal cancer patient
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-Aggressive nutritional support may prolong life
-TPN usually not used in patients nonresponsive to chemotherapy or radiation -Dehydration is not painful --> decreased pain, increased mental acuity and decreased apnea seen in patients not hydrated artificially **Apnea-->breath very deeply then not breath for a period of time (hear gurgling when person is hydrated = death rattle -Oral intake “as tolerated” -Promote pleasurable aspects of food |
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Metabolic Therapy to attack cancer
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-Detoxification
-Strengthen immune system -Special modalities (diets and vitamins) ***Coffee enemas may lead to electrolyte imbalances, infection, and sepsis |
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Oxymedicine
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-Use of ozone and hydrogen peroxide, reactive forms of oxygen, to destroy tumors
***Excess free radicals may lead to oxidation of tissues |
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Macrobiotics
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--Religious/philosophical system using a diet consisting of 50-60% whole grains, 25-30% vegetables, and rest from beans, seaweed, and soup
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Megavitamin therapy
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-Megadoses of one or more vitamins plus coenzyme Q and pangamic acid as coenzymes
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The causative factor of HIV and how it works
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-A retrovirus of the human T cell, called Human Immunodeficiency Virus
-This debilitates the body’s immune system by attacking the WBC or lymphocytes and macrophages-->T helper cells decrease --> fewer helper cell = B cells do not produce antibodies--> consequences lead to immunodeficiency -Secondary infections and neoplasms may end in severe cachexia (similar to cancer cachexia) and wasting **drugs prolong asymptomatic effects |
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Clinical categories of HIV
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-A = Asymptomatic or persistent generalized lymphadenopathy
-B = Symptomatic, not A or C conditions -C = AIDS Indicator Condition CD4+ T cell (helper) categories ≥500 cells/μl 200-499 cells/ <200 cells/μl **Can determine status by the amount of T cells and health (symptomatic) |
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Acute HIV infection
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-4 to 7 weeks after primary infection where there is rapid viral replication
-30-60% of persons develop fever, malaise, sore throat, swollen lymph nodes and rash |
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Asymptomatic HIV
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-Few noticeable symptoms; a decrease in LBM, B12 deficiency (may take a while to develop this if previously healthy) and increased susceptibility to food or water-borne pathogens may occur
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Symptomatic HIV
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-Symptoms occur like fever, sweats, fatigue, skin problems and a decline in nutrient status and body composition may be seen.
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AIDS
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Have one well defined life threatening clinical condition.
-Candidiasis -HIV encephalopathy - forgetfulness (to eat) and concentration difficulties -Kaposi’s sarcoma - eating problems and malabsorption -Burkitt’s lymphoma - " " -recurrent pneumonia -Cytomegaloviris retinitis -Herpes simplex ***unsure of the main cause of wasting **Opportunistic infections cause diarrhea, malabsorption, fever and weight loss |
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Anitviral drugs for HIV/AIDS treatment
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- Fusion Inhibitors = inhibits fusion of the HIV virus to the target cell (Fuseon)
***Prevents virus from getting into the cell -Nucleoside Reverse Transcriptase Inhibitors = prevent viral RNA from being transcribed to DNA (Retovir) **If virus gets into the cell, then can't transcribe -Non-Nucleoside Reverse Transcriptase Inhibitors (Sustiva) -Nucleotide Reverse Transcriptase Inhibitors (Viread) -Protease Inhibitors = prevents cleaving of viral proteins from assembling into a viral core (Agenerase and Norvir) ***Deals with enzymes to prevent reactions -HAART = use drugs in combination |
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Side effects of HIV/AIDS drugs
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-Chills
-Fever -Nutrient interactions |
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AIDS related wasting syndrome (CDC)
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-10% weight loss from baseline in a 6-month period accompanied by diarrhea and chronic weakness and fever for more than 30 days without a known cause
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Osteoarthritis
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-Non-inflammatory degenerative joint disease
-Occurs more in older people, females, and those with a family history -Characterized by degeneration of the joint cartilage, hypertrophy of bone at the margins, and changes in synovial membrane -Articular cartilage appears to thicken but then thins and softens and cracks form down to bone -Bone cysts form when synovial fluid comes in contact with bone -Abnormal growth of bone and cartilage forms bone spurs pain |
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Treatment for osteoarthritis
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--Medication -->aspirin, NSAID, COX-2 (cyclooxygenase) inhibitors such as celecoxib and valdecoxib --->Celebrex (no better than NSAID), corticosteroids (for pain relief), physical therapy and reconstructive surgery.
***Decrease pain, keep joint moving, or replace -Diet --> maintain normal weight as excess weight is a burden on weight-bearing joints. -Use of glucosamine and chondroitin may provide joint protection and pain relief for moderate pain. (maintain cartilage) |
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Rheumatoid Arthritis
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-Chronic autoimmune, systemic disorder that involves an inflammatory process involving cytokines beginning in the synovial membrane and progressing to the joint cartilage
-Small joints and extremities most effected -Seen more commonly in older people and females. -Inflammation thickens the synovial membrane called pannus. -Enzymes produced from pannus digest articular cartilage and erode bone. |
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Treatment for rheumatoid arthritis
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-Drugs --> Disease modifying antirheumatic drugs (DMARDs), such as methotrexate, gold salts, antimalarials, D-penicillamine, are used to slow or prevent joint damage. Aspirin, NSAID, corticosteroids (reduces inflammation), biological response modifiers that block reaction to cytokines are used to reduce inflammation.
-Lower intakes of vegetables. Fruit, and vitamin C are associated with increased risk -Omega 3’s may decrease inflammation ***Reduce inflammation |
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Gout
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-Inflammatory disease resulting in swelling, redness, heat, pain, and stiffness of the effected joint -Abnormal levels of uric acid accumulate in the blood.
-Urate crystals (not dissolved in synovial fluid) form and are deposited in small joints and soft tissue causing pain and arthritis -Risk factors include genetics, male sex, older age, overweight, excessive alcohol (because it prevents the excretion of uric acid) consumption and a diet rich in purines. ***May have pain in big toe --> cool part of the body |
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Treatment of gout
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-Drugs --> NSAIDs relieve pain and inflammation; probenecid and sulfinpyrazone increase excretion of uric acid; allopurinol inhibits uric acid production; colchicine and glucocorticoids may decrease inflammation
-Low purine diet, increased fluids, high CHO, moderate protein (dairy and soy), low fat aids in urate excretion **High purine foods include sardines, fish roe, anchovies, meat extracts and gravies, food yeast and most organ meats. *Asparagus, cauliflower, mushrooms, spinach are moderate |
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Parkinson's disease
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-Characterized by neuronal degeneration resulting in a gradual depletion of dopamine, a neurotransmitter.
**Nerve messages don't get sent from one nerve to another **See decrease in muscle activity |
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Symptoms of Parkinson's
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-Expressionless face
-Slowness and decreased voluntary movement -Resting tremor -Stooped posture -Shuffling gait -Soft monotonous voice |
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Treatment of Parkinson's
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-Drug treatment --> levadopa which is converted to dopamine, decarboxylase inhibitors reduce breakdown of L-dopa, dopamine agonists activate dopamine receptors, COMT inhibitors block L-dopa metabolism, cholinesterase inhibitors affect dopamine release, -MAO inhibitors may be neuroprotective
-Surgical implants to stimulate brain Ketogenic diets |
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Diet for a patient with Parkinson's
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-Levodopa may compete with large neutral amino acids (LNAA--> have less amine and carboxyl groups-->tryptophan and phenylalanine) for absorption and at the blood brain barrier
-the amount and distribution of protein intake is questioned -7-10 g. protein before 5 PM, eat fats, fruit, vegetables, minimal grains unless low protein. -Consume protein after 5 PM, meat and milk ***have most of the protein earlier in the day before the medicine and later at night when the medicine wears out -Improves daytime mobility with symptomatic periods at night -Vitamin B6 required to convert L-dopa to dopamine |
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Epilepsy and Seizure Disorders
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-A chronic nervous disorder with disorderly, excessive electrical discharges of the cerebral neurons where the patient’s have loss of consciousness and/or convulsions
**Grand-mal seizures = stiffness and/or thrashing of body **Petit-mal seizures = blank spells or daydreaming |
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Drug treatment for epilepsy
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-Anticonvulsants like phenytoin, valproate, primidone, phenobarbital, newer ones-->levetiracetam and zonisamide
**older medicines have more side effects |
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Ketogenic diet
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-Used for patients with epilepsy
-Used for children not well controlled by drugs -Ketone body behaves like an inhibitory neurotransmitter, producing an anticonvulsant effect on the body Traditional approach - 4:1 ratio of calories from fat to calories from protein and carbohydrate. Protein given as 1 g/kg MCT approach (oils or powders) --> 50-70% of calories as MCT. -Low CHO with high fat and protein (liver can not break down all of the fat-->produces ketones) **Atkin's diet |
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Alzheimer’s Disease
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-A progressive, neurodegenerative disease characterized in the brain by abnormal clumps of B-amyloid and neurofibrillary tangles composed of misplaced amyloid tissue
-Most common form of dementia and progresses from confusion, personality and behavioral changes to impaired judgment, loss of independence, disordered eating, weight loss, to loss of bowel and bladder control and a vegetative state. **Antioxidants and garlic and resveratrol can decrease symptoms **Can forget to eat **Antioxidants can be preventative measures |
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PKU
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-Lack of phenylalanine hydroxylase
Phenylalanine --> Tyrosine -Prevents normal brain and CNS development ***High levels of phenyalanine are toxic (can cross the blood-brain barrier)--> can cause mental retardation in young children |
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Diet for PKU
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-Restrict Phe
-Give Phe, Tyr, and protein for growth and development -Calories, vitamins, minerals for growth and development -Maintain blood phenylalanine concentration between 2-6 mg/dl ***Regulate diet ***Use formulas and eat foods with low protein |
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Tyrosinemia
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-Decreased or lacking fumaryl-acetoacetate hydroxylase
-See increased Tyrosine and Methionine - Hereditary type = failure to thrive, liver disease, hypophospatemia, rickets, renal damage, mental retardation - Neonatal type = liver not mature enough to produce enzymes ***Diet: Restrict Phe, Tyr and Met |
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Homocystinuria
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-Defect in cystathionine synthetase
Methionine-->Cysteine -Mental and growth retardation, light complexion, dislocated retinas, hepatomegaly, thrombosis ***Diet: Low protein, low methionine, give L-cystine |
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Maple Syrup Urine Disease
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-Impaired decarboxylase
-See increase in alpha keto acids of BCAA -Eat poorly, muscle hypertonicity, lethargy, abnormal eye movement and convulsions ***Diet: Limit valine, leucine, isoleucine, purified amino acid diet, low leucine may be best for long-term care |
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Galactosemia
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-Lack of galactose-1-phosphate uridyl transferanse
Galactose--> glucose ***High levels of galactose in the blood -Symptoms: vomiting, diarrhea, failure to thrive, mental retardation, cataracts, hepatomegaly ***Diet: Avoid lactose and galactose ***Requires lifelong treatment |
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Glycogen Storage Disease Type I
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-Lack of glucose-6-phosphatase
Glycogen---> Glucose -Unable to metabolize stored glycogen in the liver thus severe hypoglycemia results ***Diet: Raw corn starch administered at regular intervals (slowly digested and lasts a long time in the G.I. tract and then released into the blood to control blood glucose levels), high CHO, low fat food pattern. Infants may require pancreatic enzymes for digestion of starch. Iron supplements may be required |
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Primary lactase deficiency
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-Lactase enzyme activity level falls postweaning and is common with aging
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What is secondary lactace deficiency attributed to?
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-Muscosal injury due to gluten sensitive enteropathy
-Crohn’s disease -Ulcerative colitis -Bowel resection |
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Organic Acid Disorders (Propionic Acidemia)
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-Defect in propionyl CoA carboxylase
Propionyl CoA -->Methylmalonyl CoA -Vomiting, lethargy, hypotonia, dehydration, coma, seizures, metabolic acidosis, hyperammonemia -Use low protein diet and formulas without isoleucine, methionine, threonine, valine |
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Methylmalonic Acidemia
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-Defect of methylmalonyl mutase apoenzyme
-Acidosis, hypoglycemia, ketonemia, increased plasma ammonia and lactate -Low protein diet and formulas without isoleucine, methionine, threonine, valine |
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Ornithine transcarbamylase deficiency
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-Block in conversion of ornithine and carbamyl phosphate to citrulline
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Citrullinemia - deficiency of argininosuccinic acid synthetase
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-Blocks conversion of citrulline to argininosuccinic acid
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Argininosuccinic aciduria-deficiency of argininosuccinate lyase
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-Blocks argininosuccinic acid to arginine
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Carbamyl-phosphate synthetase deficiency
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-Neurologic outcome and intellectual development vary from normal to severe mental retardation and cerebral palsy. Use of alternative pathways for waste N excretion and protein restricted diet or formulas low in protein without nonessential amino acids to control ammonia levels improve outcome
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