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| 8156369633 | What are *osteoclasts* derived from? | Monocytes | 0 | |
| 8156369634 | What cells send signals to the *osteoclasts*? | Osteoblasts | 1 | |
| 8156369635 | What are the important *interleukins/ cytokines* that act on *osteoblasts*? | IL-1, IL-6, TNF | 2 | |
| 8156369636 | What *stimulatory signals* are produced by the *osteoblasts* and sent to the *osteoclasts*? | Rank-Ligand & M-CSF (Monocyte- Colony stimulating factor) | 3 | |
| 8156369637 | What *signals* act to *inhibit* osteoclasts? | OPG (), calcitonin, biphosphonates | 4 | |
| 8156369638 | *True or False:* Human calcitonin is what maintains bone in humans. | False: calcitonin is a vestigial hormone in humans! Doesn't do a thing to serum calcium levels. Whaaat | 5 | |
| 8156369639 | *True or False:* Harvested calcitonin from salmon can be administered to maintain bone in humans. | Truth We can take it from other animals just fine, human calcitonin is the only one inactive | 6 | |
| 8156369640 | What do we call congenital malformations, like *an extra rib, a missing rib, fusion of bones, or myelominingocele due to failure of the skull/spinal column to fuse*? | Bone dystoses | 7 | |
| 8156369641 | What's a *supernumerary bone*? | Like an extra finger or rib | 8 | |
| 8156369642 | What's *syndactyly*? | Fusion of adjacent digits | 9 | |
| 8156369643 | What do we call it when there's *failure of skull/spinal column to close*? | Craniorachischisis | 10 | |
| 8156369644 | What's it called if a person has a disorder that *affects cartilage and bone tissues globally* because of *underlying mutations in matrix components* or cell *signaling* mechanisms? | Bone dysplasia | 11 | |
| 8156369645 | What's the most common disease of the *growth plate*? | Achondroplasia | 12 | |
| 8156369646 | Importante: What's the gene defect in *Achondroplasia*? | Fibroblast Growth Factor Receptor 3 (FGFR3) | 13 | |
| 8156369647 | What's the normal action of * Fibroblast Growth Factor Receptor 3 (FGFR3)*? | Inhibition of cartilage proliferation | 14 | |
| 8156369648 | What's going on in a person with * Achondroplasia*? | Constant activation of the receptor, suppresses growth | 15 | |
| 8156369649 | Tyrion Lannister is c/o of pain in his legs. He's diagnosed with Achondroplasia. What causes the pain? | narrowing of spinal canal/ neural foramina | 16 | |
| 8156369650 | What's inhibited in *Achondroplasia/ FGFR3* mutation? | Type I collagen | 17 | |
| 8156369651 | What's the most common *lethal* form of *dwarfism*? | Thanatophoric dwarfism caused by frameshift or point mutation in FGFR3 | 18 | |
| 8156369652 | What makes * Thanatophoric dwarfism* or *homozygous achondroplasia* lethal? | Underdeveloped thoracic cavity that causes respiratory insufficiency | 19 | |
| 8156369653 | What's the problem with *Osteogenesis Imperfecta*? | Type I collagen is structurally messed up | 20 | |
| 8156369654 | What's the most common *genetic disease of the skeleton* (15-20k patients in U.S.)? | Osteogenesis Imperfecta | 21 | |
| 8156369655 | What are the *two ways* that *Osteogenesis Imperfecta* can be caused? | 1. Null Allele 2. Dominant negative | 22 | |
| 8156369656 | What's the defect in *Osteogenesis Imperfecta* caused by the *Null Allele Effect*? | Reduced collagen | 23 | |
| 8156369657 | What's the defect in *Osteogenesis Imperfecta* caused by *Dominant Negative*? | Abnormal chains of collagen | 24 | |
| 8156369658 | What's the *lethal form* of *Osteogenesis Imperfecta*? | Type II | 25 | |
| 8156369659 | A baby *dies* within days of birth. They have *skeletal deformities* and *blue sclera*. What did this baby have? | Type II Osteogenesis Imperfecta | 26 | |
| 8156369660 | A child comes in with *multiple fractures*, but is normal in height. Child abuse is not a factor. They also have *hearing impairment*. What is going on in this kid? | Type I Osteogenesis Imperfecta | 27 | |
| 8156369661 | An adult comes in with *multiple fractures*. They are abnormally short (*4ft tall*) and have *bowed* legs. When they were born, they note they had *blue sclera*, but now it's gone. What could be going on? | Type III Osteogenesis Imperfecta | 28 | |
| 8156369662 | What type of Osteogenesis Imperfecta is not characterized by blue sclera? | Type IV | 29 | |
| 8156369663 | A person has *very thick, sclerotic looking bones*, but they also have had *multiple fractures*. Their vertebrae have a *rugger-jersey* appearance. On X-Ray, their bones look like there's a "bone within a bone." What could this person have? | Osteopetrosis | 30 | |
| 8156369664 | What's 2 scary things that can happen with *osteopetrosis*? | 1. Cranial nerve compression 2. Anemia due to BMS | 31 | |
| 8156369665 | Patients with *osteopetrosis* can have *"erlenmeyer flask"* long bones. Who else can have that, again? | Patients with Gaucher | 32 | |
| 8156369666 | What causes *osteopetrosis*? | A bunch of mutations | 33 | |
| 8156369667 | How can the *chloride channel mutation* that may be seen in *osteopetrosis* inhibit *osteoclast* activity? | Osteoclasts need that to secrete acid to resorb bone | 34 | |
| 8156369668 | What does a *deficiency in M-CSF* cause? | Inability for osteoclasts to differentiate—> Osteopetrosis | 35 | |
| 8156369669 | What's the *take-home* issue that all those mutations cause in *Osteopetrosis*? | Osteoclasts cant resorb bone | 36 | |
| 8156369670 | A baby is born with a *large head, flat facies, bell-shaped thorax, and curved femoral bones*. There are no fractures. She died the day after birth. What does she likely have? | Thanatophoric dwarfism | 37 | |
| 8170504627 | What are the *risk factors* for *osteoporosis*? | 1. Genetic Factors (Vit D handling + estrogen) 2. Decreased Physical activity 3. Nutrition (Ca, Vit D) | 38 | |
| 8170504628 | What *genetic factors/ mutations* can be *risk factors* for *osteoporosis*? | 1. Vit D Receptor Allele COL1A1 2. Estrogen receptor IGF-1 | 39 | |
| 8170504629 | What's going on in *menopause* that makes women more prone to *osteoporosis*? | 1. Estrogen is down 2. Inflammatory cytokines are up 3. RANK & RANK-L expression increased | 40 | |
| 8170504630 | What's the effect of *increased RANK/ RANK-L expression* during menopause? | Increased osteoclast activity | 41 | |
| 8170504631 | What's the big thing going on in *aging* that increases the risk of *osteoporosis*? | Decreased osteoblasts activity (no replacement) | 42 | |
| 8170504632 | What are the common places for *osteoporosis* to show up? | 1. Lower thoracic/ lumbar vertebrae 2. acetabular area 3. wrist | 43 | |
| 8170504633 | What's going on in a vertebra with *osteoporosis* that causes the hunchback appearance? | Tiny little compression fractures that make the vertebra smaller and smaller, deforming them | 44 | |
| 8170504634 | What's the difference in a *T score* & a *Z score*? | *T:* how someone's result compares to the average *normal mean (young adult)* *Z:* how someone's result compares to the average normal mean for someone *their age* | 45 | |
| 8170504635 | A patient has a *T score of -0.5*. This patient has ____________ bone mass. | Normal (> -1) | 46 | |
| 8170504636 | A patient has a *T score of -2.5*. This patient has ____________ bone mass. | Low/ Osteopenic (-1 to -2.5) | 47 | |
| 8170504637 | A patient has a *T score of -2.6* and no fractures. This patient has ____________ bone mass. | Osteoporotic (< -2.5 with no fractures) | 48 | |
| 8170504638 | A patient has a *T score of -2.6* and an osteoporotic fracture. This patient has ____________ bone mass. | Severe/ established osteoporotic ( < -2.5 & with at least 1 fracture) | 49 | |
| 8170504639 | What score do we like to use to look at *bone mass density*— *T or Z*? | T | 50 | |
| 8170504640 | Why does *postmenopausal osteoporosis* occur? | Missing estrogen (which usually inhibits the inflammatory cytokines that activate osteoblasts) Bone resorption is favored over formation | 51 | |
| 8170504641 | Histology of the lungs of a 40 yo Danish patient shows *noncaseating epitheliod granulomas* that contain *multinucleated giant cells*. What's likely going on? | Sarcoidosis | 52 | |
| 8170504642 | Histology of patient with *sarcoidosis* shows *giant cells* containing *laminated concentrations of calcium and proteins*. What are these things? | Schaumann bodies | 53 | |
| 8170504643 | Histology of patient with *sarcoidosis* shows *giant cells* containing *stellate inclusions*. What are these things? | Asteroid bodies | 54 | |
| 8170504644 | Asteroid bodies, giant cells, & Schaumann bodies. | Sarcoidosis. | 55 | |
| 8170504645 | Why is there *bone resorption* in the hands and feet in *sarcoidosis*? | Granulomas produce factors that enhance Vit. D activation (So, now you've got all this Vit D activated saying "we need more calcium", but there isn't any more. So the hands and feet get resorbed to provide that Ca++) | 56 | |
| 8170504646 | What differentiates *osteomalacia* from *rickets*? | Demarcation of the growth plates | 57 | |
| 8170504647 | We always think of Vit D when we think about *Calcium deficiency*. Who's the *forgotten player* in that game? | Phosphorus! | 58 | |
| 8170504648 | A child has *bowed legs*, an indentation of the *rib cage*, and *bead-like bumps* along the growth plates of the ribs. What's likely going on in this kid? | Rickets | 59 | |
| 8170504649 | What's *Harrisons groove*? | An indentation of the rib cage in kids with rickets due to the diaphragm pulling on those poorly mineralized ribs | 60 | |
| 8170504650 | What's it mean if a kid as a *rachitic rosary*? | Bead-like bumps along the growth plates of the ribs caused by rickets | 61 | |
| 8170504651 | What's the big difference between *rickets* & *OI*? | Multiple fractures + blue sclera don't happen in rickets like they do in OI | 62 | |
| 8170504652 | Importante: What's the best/ most sensitive *serum test* for any disease that favors osteoblasts activity? | Alkaline phosphatase level (these patients are trying really hard to mineralize the bone* | 63 | |
| 8170504653 | What's the job of *alkaline phosphatase*? | Used by osteoblasts to mineralize bone | 64 | |
| 8170504654 | What's the *normal* result of a *Decalcified bone biopsy* with tetracycline administration 7 days apart? | Two layers of deposits with a gap | 65 | |
| 8170504655 | What's the result of a *Decalcified bone biopsy* with tetracycline administration 7 days apart that indicates *osteomalacia*? | No gap between depositions | 66 | |
| 8170504656 | A patient's bone biopsy shows *generalized osteitis fibrosa cystica*. What's this the hallmark of? | Hyperparathyroidism | 67 | |
| 8170504657 | Bone biopsy of a patient shows *dissecting osteitis, peritrabecular fibrosis, & cystic brown tumors*. X-Ray of their hands shows marked *thinning of the cortices* of the *middle phalanges* of the index & middle fingers. What could be going on in this patient? | Hyperparathyroidism | 68 | |
| 8170504658 | What will you *ALWAYS* see with *chronic renal disease*? | Widespread bone disease | 69 | |
| 8170504659 | What bone changes are seen in a *kid with renal failure*? | Rachitic & osteomalacic changes | 70 | |
| 8170504660 | What bone changes are seen in an *adult with renal failure*? | Secondary Hyperparathyroidism & Osteomalacic changes | 71 | |
| 8170504661 | What are the *3 stages* of *Paget's Disease*? | 1. Osteolytic 2. Mixed clast/blast 3. Osteosclerotic | 72 | |
| 8170504662 | What's the current *hypothesis* as to the cause of *Paget's Disease*? | Paramyxovirus infection | 73 | |
| 8170504663 | Importante: What are the most common places *Paget's Disease* shows up? | Vertebra or femur | 74 | |
| 8170504664 | What can cause *death* in a patient with *Paget's Disease*? | Cardiac hypertrophy | 75 | |
| 8198481824 | What does *bone biopsy* of a patient with *Paget's disease* show? | Mixed osteoblastic-osteoclastic stage with *cement lines* in the bone | 76 | |
| 8198481825 | A bone has *wedge-shaped necrosis*, with live overlying articular cartilage. Inside the dead bone, there are *ruptured adipocytes* and *calcium soap formation* & *"creeping substitution*. What infarct is going on here? | Subchrondral infarct | 77 | |
| 8198481826 | On X-ray, a bone has *sclerotic lesions its center*. What infarct could be happening? | Medullary infarct | 78 | |
| 8198481827 | What's the *#1 route* for *osteomyelitis* to get to bone? | Hematogenous spread | 79 | |
| 8198481828 | What are the most common *organisms* that cause *osteomyelitis* in adults? | 1. Staph aureus 2. UTI: E. Coli, pseudomonas, klebsiella | 80 | |
| 8198481829 | What are the most common *organisms* that cause *osteomyelitis* in *neonates*? | Haemophilus influenza & GBS | 81 | |
| 8198481830 | What are the most common *organisms* that cause *osteomyelitis* in *sickle cell pts*? | Salmonella | 82 | |
| 8198481831 | What serious complication can *osteomyelitis* lead to? | *Sequestrum* of dead bone with *involumcrum sleeve* reactive bone around it | 83 | |
| 8198481832 | Where does *TB* usually go when it spreads to *bone*? What is this called? | Vertebral column; Pott's disease | 84 |
