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mhewett's version from 2016-06-27 22:54

Section 11.10 Jaundice

Question Answer
Jaundice is d/t deposition ofbilirubin pigments
When does jaundice become apparent?serum bili > or = 2 mg/dL
When is sublingual icterus apparent? >/=5 mg/dL; should suspect biliary or hepatic issues
What are 4 primary causes for jaundice?hemolysis, deficiency of glucuronosyl transferase, defects in transport of bilirubin from hepatocyte to bile duct, obstruction of biliary system
hemolysis causes elevated what?unconjugated bilirubin
deficiency of glucuronosyl transferase causes elevated what?unconjugated bilirubin
defects in transport of bilirubin from hepatocyte to bile duct causes elevated what?conjugated bilirubin
obstruction of biliary system causes elevated what?conjugated bilirubin
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Section 11.11 Congenital Causes of Jaundice

Question Answer
What is the issue in patients with Gilbert's and Crigler-Najjar syndrome?decreased uridine diphosphate glucuronosyl transferase
Gilbert's: mild, apparent during times of stress
C-N Type 2: like Gilbert's but more severe/chronic
C-N Type 1: fatal by 1yoa d/t complete lack of UDGT
What is the inheritance pattern of Crigler-Najjar Type 1? Type 2?Type 1: Auto recessive - has to be or the genes would have stopped long ago b/c it's fatal by 1yoa
Type 2: Auto dom
What is the issue with Rotor and Dubin-Johnson syndrome and what test will be elevated?Carrier defect resulting in impaired hepatocellular secretion; causes elevated direct bilirubin (will also see darkly pigmented liver in dubin-johnson)
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Section 11.12 Hepatitis

Question Answer
What type of virus is HAV? HBV? HCV? HDV? HGV?HAV: RNA picornavirus
HBV: DNA hepadnavirus
HCV: RNA flaviviridae
HDV: Delta, RNA virus
HGV: RNA flaviviridae
What is the transmission of HAV? HBV? HCV? HDV? HEV? HGV?HAV: fecal/oral
HBV: parenterally, sexually, vertically
HCV: parenterally
HDV: parenterally, sexually
HEV: fecal/oral
HGV: parenterally, sexually, vertically
What lab test is indicative of a current HBV infection and what is its transmission?+ HBsAg
What kind of changes does HCV cause in AST/ALT tests?EPISODIC increases in ALT/AST
HDV occurs only in conjunction with what virus and why?HBV bc it requires HBV's antigen coat for replication
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Section 11.13 Hepatitis B Serology

Question Answer
How long must a pt have a +HBsAg test before they are considered to be in a carrier state?>6 months
What does HBsAg indicate?current hepatitis presence (current infection)
What does anti-HBc indicate?exposure to HBV (past or present)
What does HBeAg indicate?state of extreme infectiousness
When is HBV DNA present?in serum of those with current infection; used to determine viral load
Why does it take so long for the body to produce Anti-HBsAg?It's a lipid antibody to the hep B surface antigen and appears after sxs disappear (indicates immunity); the immune system isn't as robust at developing those types of abs.
What will and won't be positive during the window periodnot present: HBsAg, anti-HBs
present: anti-HBc, HBV DNA
What tests will return as positive if a pt is symptomatic of hepatitis but has - HBsAg and - Anti-HBsAg tests?Anti-HBcAg will be positive as well as HBV DNA load
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Examples page 188

Section 11.14 Toxic Hepatitis

Question Answer
What drugs commonly cause toxic hepatitis?Methotrexate, Isoniazid, Chloramphenicol, Halothane
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Section 11.15 Cirrhosis

Question Answer
What is cirrhosis?diffuse disorganization of normal hepatic structure by regenerative nodules surrounded by fibrotic tissue
the end stage of many liver diseases
top causes of death in 45-65 in USA
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Section 11.16 Cirrhosis: Signs and Symptoms

Question Answer
Why does cirrhosis result in increased infections, weakness/weight loss, and bleeding problems?Infxn: d/t decreased production of complement
Weakness/Weight loss: major organ of metab is no longer fxning properly and fat malabsorption
Bleeding: decreased production of clotting factors
Why does cirrhosis cause ascites?Decreased albumin production by the liver results in decreased oncotic pressure and the back pressure on the portal vein allows for transudation of fluids into the peritoneum
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Section 11.17 Types of Cirrhosis

Question Answer
What signs and symptoms does alcoholic cirrhosis produce?Testicular atrophy (toxic to testes)
Spider angiomata, gynecomastia, and palmar erythema (d/t high E that can't be removed via liver)
Hepatic encephalopathy (can't remove ammonia)
Ascities (transudate and decreased oncotic pressure from dec albumin)
Varices
Jaundice (duh)
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Section 11.18 Other Causes of Cirrhosis

Question Answer
What is the issue with hemochromatosis and what HLA is it associated with?d/t increased Fe absorption leading to deposits in heart (CHF) liver (cirrhosis), pancreas (new onset diabetes) and skin (bronze color)
HLA A3
How is Wilson's disease inherited and what are the signs and symptoms of Wilson's disease?AR
Copper is Hella BADD
ceruloplasmin decreased, cirrhosis, corneal deposits, carcinoma (hepatoma)
Hemolytic anemia
Basal ganglia degeneration (Parkinson's symptoms), Asterixis (flapping tremor of wrists)
Dyskinesia, Dysarthria, Dementia
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Section 11.19 Liver Cancer

Question Answer
What lab test would be massively elevated in a pt with a hepatoma?Alk phos since the tumor is producing its own
ALT/AST will only be modestly elevated b/c most of the liver is still fxning, AFP is the tumor marker
What are the most common causes of hepatoma?HBV, HCV, Aflatoxin exposure (from grains)
What is the greatest risk factor for cholangiocarcinoma?Clonorchis sinensis (liver fluke; Asia)
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Section 11.20 Pancreatitis

Section 11.21 Synopsis of Major Pediatric GI problems

Question Answer
What area of the bowel is most likely to be involved in intussusception?Ileocecal valve (terminal ileum into ascending colon)
A pt presents with severe epigastric pain with radiation to her back, anorexia, and nausea. You suspect acute pancreatitis. What are the most common causes?GET SMASHED- Gallstones, EtOH, Trauma, Scorpions, Mumps, Autoimmune, Steroids, Hyper Ca++ or Hypertriglyceridema, ERCP, Drugs
What fails to develop in Hirschprung's dz?Auerbach and Meissner's plexuses d/t failure of migration of neural crest cells
Risk is increased with Down's syndrome
Why can maternal NSAID use during pregnancy cause necrotizing enterocolitis?Inhibits COX and prostaglandin synthesis resulting in vasoconstriction which compromises the neonatal bowel's blood supply resulting in ischemia and death of the bowel mucosa which will eventually lead to full thickness necrosis. (PGs produce vasodilation)
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Section 12.0 Arthritis

Question Answer
What type of disorder is osteoarthritis?Disorder of hyaline cartilage and subchondral bone
In OA, how does eburnation occur and what are joint mice?Eburnation: d/t cartilage loss and wearing down of underlying bone
Joint mice: pieces of osteophytes that have broken off into the joint space contributing to damage to cartilage
What are the 7 criteria for RA and how many must a pt have to be dx with RA?Arthritis involving the hands
Arthritis involving at least 3 joints
Symmetrical arthritis
Changes on Xray (rat bite lesions)
Pain & stiffness lasting for >1h in the morning
+Rheumatoid factor
Rheumatoid nodues (palpable subQ nodes on extensor surfaces of hands and arms)
4 of 7
What antibodies cause RA?Anti-IgG abs
The Ag-Ab complexes preferentially deposit in small joints, especially MCP and PIP
What occurs in RA with respect to the synovial membrane, cartilage, and subchondral bone?Synovial membrane proliferates (pannus formation - granulation tissue)
Cartilage and subchondral bone are eroded
Also have soft tissue swelling
What labs will typically be elevated with RA and what HLA types is it associated with?Increased ESR (fibrinogen sticking to RBCs making them heavy; acute phase liver product) and CRP (acute phase response product of liver)
+RF (usually)
HLA-DR4, Dw4, or Dw14 (4 criteria needed for RA)
What are Still's dz and Felty's syndrome?Still's dz: juvenile RA before 16yoa (acutely febrile, RF-, micrognathia)
Felty's synd: severe RA with splenomeg, neutropenia, leg ulcers, and increased infxn
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Section 12.1 Bone Disorders

Question Answer
What defect is present in osteogenesis imperfecta?Disorder of collagen synthesis causing abnormal fragility
Osteogenesis imperfecta signs and symptomsincreased fractures, blue thin sclera
Most severe osteogenesis imperfecta typeNewborn type most severe; skull feels like bag of bones - soft w/ Wormian bones
What are other names for osteopetrosis?Marble Bones or Albers-Schonberg Dz
What is osteopetrosis?failure of normal bone resorption d/t defective osteoclasts
Osteopetrosis clinical symptomssclerosis of vertebral endplate; increased density; brittle bones
What causes achondroplasia?defective cartilage synthesis causing decreased epiphysis formation
What is achondroplasia?dwarfism, autosomal dominant, causes short limbs with normal sized head and trunk
What areas of the body are particularly susceptible to aseptic necrosis? What diseases are associated?Scaphoid bone, Femoral head, Tibial tuberosity
Legg-Calve-Perthes dz (necrosis of femoral head seen in boys ~10yoa most commonly)
Osgood-Schlatter dz (necrosis of tibial tuberosity d/t avulsion frx seen at 11-13 yoa when kids become active in sports)
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Question Answer
What is osteoporosis?thinned cortical bone and enlarged medullary cavity resulting in fragile bones
Who does osteoporosis affect?caucasians, petite, women
What are the causes of osteoporosis?Estrogen deficient state (estrogen maintains osteoblasts, Physical inactivity (osteoblasts are activated with stress to bones), Hypercortisolism (Cushing's, steroids), Hyperthyroidism, Subclinical Ca++ deficiency
What are the different types of primary osteoporosis?idiopathic- occurs in children and adolescents
Type I- postmenopausal (w/in 15 yrs of menopause)
Type II- involutional/senile osteoporosis (>70 yrs old both men and women)
What is secondary osteoporosis?d/t secondary causes - ex: immobility, Cushing's, ovariectomy, etc.
DOC for prevention of osteoporosis in osteopenic patientsbisphosphonates
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Question Answer
What is osteomalacia?d/t inadequate bone mineralization (like adult rickets)
symptoms include bony pain, skeletal deformities (like long bone bowing), frequent fractures
Serum findings of osteomalacialow Ca, low phosphate, high alkaline phosphatase
What is a characteristic sign of osteomalacia on x ray?Looser's Zones - radiolucent bands perpendicular to periosteum d/t pseudofractures
Why do liver and kidney disease lead to vitamin D deficiency?Liver converts cholecalciferol to 25-OH-cholecalciferol
Kidney converts 25-OH-vitD3 to 1,25-(OH)2-vitD3 in the PCT
What are 6 etiologies of osteomalacia?vit D deficiency- vit D encourages Ca and phosphate absorption from the gut
severe liver disease, anticonvulsant drugs, renal osteodystrophy
Fanconi's syndrome - destruction of PCT
malnutrition/malabsorption
What is osteitis fibrosa cystica?focal bony damage caused by severe hyperparathyroidism
results from microfractures and secondary hemorrhages that cause an ingrowth of fibrous tissue along with hemosiderin deposition which then undergo cystic degeneration
What are common symptoms of Paget's disease?Frontal bossing, Deafness, Bony pain, Pain d/t compression of nerves, chalkstick and vertebral compression frxs, High Output cardiac failure
What is Paget's disease?increased uncontrolled bone turnover, resulting in disorganized bone (so it's weak)
lab findings of Paget'sextremely high alk phos
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Section 12.2 Cartilage

Question Answer
What is osteochondroma?benign bone/cartilage tumor near end of a long bone covered by a cartilaginous cap; most common benign bone tumor
What bony change is seen with osteochondroma?Exostosis - cartilage covered bony outgrowth at the metaphyses
What age does osteochondroma affect?10-20 yo
What is an enchondroma?benign cartilaginous tumor where there isn't normally cartilage growing within/inside bone
What is a chondroblastoma?benign tumor of cartilage producing cells
Who does chondroblastoma affect?10-20 yo, rare
What region does chondroblastoma affect?femur, tibia, humerus epiphyses
What is a chondrosarcoma?malignant sarcoma containing cartilage
Where does chondrosarcoma occur?spine, pelvic bones
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Section 12.3 Bone Tumors

Question Answer
What are the locations of osteoma, osteoid osteoma, and osteoblastoma?Osteoma: face/skull
Osteoid osteoma: extremities
Osteoblastoma: spine
How does osteoma present?multiple, painless
If you had a pt present with osteoid osteoma, what would you expect to see?A teen to young adult with small, PAINFUL tumors in their extremity(ies)
How does osteoblastoma present?larger, milder pain - achy, dull
What malignant bone tumor is associated with Codman's triangle and/or a sunburst pattern seen on x-ray?Osteosarcoma - most painful bone cancer
What is the first symptom of osteosarcoma?pathologic fracture
Who does osteosarcoma affect?bimodal peak: mostly <20 yo and elderly
What malignant bone tumor appears as a tender, swollen area that is commonly mistaken as an infection?Ewing's sarcoma - onion skin, t(11;22), PAINFUL
Who does Ewing's sarcoma affect?10-15 years old; caucasian males
What do we see histologically with Ewing's sarcoma?Homer-Wright rosettes
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Section 13.0 Muscular Dystrophies

Question Answer
What labs do you expect with all muscular dystrophies?elevated creatine kinase
What type of patient would you expect to have a +Gower's sign?Duchenne Muscular Dystrophy; pts - walk themselves upright with their hands
What causes DMD?lack of dystrophin
What causes Becker's?decreased amount of dystrophin
How does DMD progress?pectoral muscles weaken first and later pelvic girdle; will also have delayed motor milestones, cognitive impairment, hypertrophied calves
Onset of DMD2-3 years; wheelchair bound by 12
Death of pts with DMD d/tin 20's d/t pneumonia
Onset of Becker's5-15
Limb girdleAR; proximal hip and shoulder girdle weakness - very heterogenous
FacioscapulohumeralAD; onset late childhood/adolescence; face & shoulder girdle weakness +/- cardiac defects, intellectual disability
MyotonicAD; onset any age - pts have long face; can't voluntarily relax after forceful contraction with primarily distal weakness; temporal and masseter muscle wasting, ptosis, hypersomnolence, formation of cataracts, premature frontal balding, atrophy of gonads with infertility, MR, hyperglycemia, cardiac arrhythmias
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