Goljan facts Flashcards
Pathological fractures at birth, deafness, and blue sclera. What is the treatment?
OI–> Bisphosphanates= increase bone mineralization
Impaired proliferation of cartilage at growth plate causing normal sized head with short limbs. What is the genetics?
AD mutation in FGFR 3 gene
“Marble bone” disease causing TOO much bone that is brittle caused by deficiency of osteoclasts?
Osteopetrosis
PT with pathological fractures, Anemia, visual and hearing loss due to nerve compression. Dx?
Osteopetrosis
Most common route and site of spread of osteomyelitis?
Hematogenous
METAPHYSIS
MCC of osteomelitis?
S. aureus
MCC of osteomyelitis in SCD patients?
Salmonella
Hematogenous spread of lung infection to vertebral column?
TB osteomyelitis= POTT’s disease
Osteomyelitis caused by puncture of foot through rubber footwear?
P. aeruginosa
What are sequestra and involucrum?
Sequestra–> Dead bone
Involucrum–> Reactive bone formation
What is a consequence of Draining sinus tracts associated with Osteomyelitis?
SCC
Pt with Fever, bone pain, and CT showing dead bone with a rim of bone formation. MCC?
Osteomyelitis= S. aureus
Loss of both organic bone matrix and minerals with associated Decreased thickness of Cortical and trabecular bone?
Osteoporosis
Diagnosis of osteoporosis is done by?
Dual photon absorptiometry
What are the MCC of avascular necrosis?
Corticosteroids
Alcohol
SCD
MC site of avascular necrosis?
Femoral head and condyle
*Scaphoid bone
Pt w. localized pain that is exacerbated with movement, MRI showing increased density known as “double line sign” of bone?
Avascular necrosis
Trauma–> ischemia–> separation of cartilage from bone –> articular epiphysis fails MC in Distal Femur?
Osteochondritis dissecans
Men >50 yo, targets pelvis + femur and ENLARGED skull, possibly caused by viruses?
Paget’s disease
Weak, thick, mosiac bone, with high vascularity, increased ALK, Bone pain. What are other complications of this disease?
Osteogenic Sarcoma
High OUTPUT Heart Failure
Hearing Loss
Increased Hat size?
Pagets disease== increased Skull size during osteoblastic phase
Defect in Osteoblastic differentiation and maturation?
Fibrous dysplasia
Medullary bone replacement by fibrous tissue and cyst formation most commonly in RIBS and femur?
Fibrous dysplasia
Fibrous dysplasia involving many bones (polyostotic) + Cafe au lair spots + precocious puberty?
Albright syndrome
Most common benign tumor of bone?
Osteochondroma
Bone tumor in Epiphysis of Distal femur or proximal Tibia?
Giant Cell tumor of bone
Negatively birefringent–> Yellow= Parallel = ?
Monosodium urate crystals
Positively birefringent–> Blue= parallel = ?
Calcium pyrophosphate crystals
10-30 yo Male pt w/ an outgrowth of his Distal femur?
Osteochondroma
Medullary tumor in Small tubular bones of Hand/ Feet Dx?
Enchondroma
Male pt with mass in the Facial bones and hx of multiple GI polyps?
Osteoma –> associated with Gardner’s polyposis syndrome
Male, 10-20 yo w. “Radiolucent focus surrounded by sclerotic bone” in cortex of his Proximal femur. Tx of his Nocturnal bone pain is?
Osteoid osteoma–> Aspirin
Radiolucent focus surrounded by sclerotic bone?
Osteoid–> sclerotic area
Osteoma–> FOCUS
Osteoid Osteoma occurring in Vertebrae and not relieved by Aspirin?
Osteoblastoma
Female 30-60 yo pt with Neoplastic mononuclear cells in the epiphysis around Knee. Describe Histology?
Giant cell tumor= Multinucleated giant cells
Male with growing mass in Pelvic bones that has metastasized to his lungs?
Chondrosarcoma
Male 10-25yo with growing mass in the Metaphysis around his KNEE, FHx of Blind sister, fibrous dysplasia. What are the MC radiologic findings?
Codmans triangle
“Sunburst”–> speculation of calcified tumor
What are the risk factor for Osteogenic sarcoma?
Pagets disease
Retinoblastoma
Radiation
Fibrous dysplasia
Male 10-20 yo with small round blue cell tumor in the Diaphysis of his Femur or Pelvic girdle. MC radiological finding is?
Ewing sarcoma–> “Onionskin” appearance
“Onionskin” appearance of bone on radiography?
Ewing sarcoma
What forms of arthritis show “morning stiffness?”
RA
SLE
Polymyalgia rheumatica
Homogentisic acid deposits in intervertebral disks showing Black color and leading to Osteoporosis. Dx?
Alkaptonuria
Articular cartilage is made up of?
Type II collagen
Proteoglycans
Osteophytes/ enlargement of DIP joints are called?
Heberdens nodes
Osteophytes/ enlargement in PIP joints are called?
Bouchards nodes
What are the common joint features in Osteoporosis?
Subchondral sclerosis Bony spur Osteophytes Joint mice-> cartilage fragments NO ANKYLOSIS
Noninflammatory joint disease 2nd to neurological disease causing loss of Proprioception?
Neuropathic arthropathy
Causes of Neuropathic arthropathy?
Diabetes Mellitus
Syringomyelia
Tabes dorsalis
Disease of Women associated with HLA-DR4?
RA
B cells producing antibodies against Fc portion of IgG causes?
Rheumatoid factor–> RA
Granulation tissue that releases cytokines and destroys articular cartilage?
PANNUS–> repair causes Ankylosis
Ulnar deviation, Swan neck finger, Boutonniere deformity seen in?
RA
Describe joint space in RA?
Inflammation
PANNUS
eroding cartilage
Fibrous + BONE ankylosis
What are some of the complications of RA?
Chronic pleuritis Interstitial Fibrosis (NSIP pattern) Anemia of chronic disease Carpal Tunnel Bakers cysts (popliteal) Rheumatoid nodules (Caplan syndrome in LUNGS) Sjogrens syndrome
Initial therapy for RA is?
NSAIDS
Sjogrens syndrome is associated with developing?
Lymphomas
Male dominance arthritis caused by Alcohol, high meat diets, treating leukemias?
Gout
What are some inborn errors of metabolism associated with GOUT?
HGPRT deficiency-> Lesch Nyhan syndrome
Complications of Gout include?
Urate nephropathy
Uric acid stones
HTN
Chronic gout leads to the formation of?
Tophi–> MSU deposits in soft tissue around joints
RF negative, arthritis of Skeleton/spine, Male dominance, Sacroiliitis, HLA-B27?
Seronegative Spondyloarthropathy
Ex: Ankylosing spondylitis + Reiters syndrom + Psoriasis + Enterhepatic
HLA-B27?
Spondyloathritis
Male with Sacroiliitis morning stiffness?
Ankylosing Spondylitis
Longterm consequences of Ankylosing Spondylitis?
Bamboo spine–> ankylosis of vertebrae
Aortitis w. Regurgitation
Uveitis –> Blindness
Urethritis due to Chlamydia trochomatis, Achilles Tendon periostitis, conjunctivitis, Arthritis?
Reiters syndrome
Conjunctivitis + Urethritis + Arthritis ?
Reiters syndrome
can’t SEE, cant PEE, cant climb TREE
Sausage shaped DIP joints, “pencil-in cup” deformity” + nail pitting?
Psoriatic arthritis
20yo female w. deficient in C5-C9, Knee arthritis, dermatitis?
Septic arthritis== Gonococcal
FIbers rich in mitochondria, oxidative enzymes, poor ATPase enzymes?
Type I = Slow twitch
Specailized for fine, skilled movements, poor mitochondria, rich in ATPase and glycolytic enzymes?
Type II== Fast twitch
Weakness and wasting of pelvic muscles, Gower’s maneuver, Waddling gait. What is the genetics?
DMD–> X- recessive–> absent Dystrophin
What lab finding is seen in DMD?
Increased CK @ birth
Male adult pt, unable to relax grip, sagging face/ cant close mouth, testicular atrophy, cataracts. Genetics?
AD Tri-nulceatide repeats (CTG)–> Myotonic dystrophy
Antibodies against ACh receptors, thymic hyperplasia, endrophium test positive. What are the clinical signs of this disease?
Ptosis--> Upward gaze Diplopia Proximal muscle weakness-> WORSE w. exercise Dysphagia for solids + liquids NO sensory or reflex loss
5ps; pain, paresthesia, pallor, paralysis, pulselessness?
Compromised blood supply–> COMPARTMENT syndrome
Risk factors of Gonococcal arthritis?
C5-C9 deficiency
Recent menstruation
Pregnancy or postpartum
SLE
Symmetric proximal muscle weakness + heliotrope rash + Elevated muscle enzymes + myopathic changes on EM + biopsy abnormality?
Polymyositis or Dermatomyositis
Heliotrope rash is high specific for?
Dermatomyositis
Anti-Jo antibodies?
Dermatomyositis or Polymyositis
Specific mutation significant for Pg of Osteopetrosis?
Carbonic Anhydrase II –> Acidic environment is required for Bone resorption
Fractures + leukopenia + hearing impairment + Renal tubule acidosis. Tx?
Osteopetrosis–> TRANSPLANT
Pigeon breast deformity + Frontal bossing + Beads in the Ribs?
RICKETS
Beads= rachitic rosary
Decreased Ca++ & phosphorus, increased PTH & ALK?
Osteomalacia
Activation of Osteoblasts causes and increase in what serum test?
ALK –> Alkaline environment is required to LAY down bone
Where does Osteomyelitis usually occur in children?
Metaphysis–> Growing bone need more Blood at the Metaphysis which brings in the Infection
Adults with osteomyelitis will the infection located where?
Epiphysis–> blood supply goes through growth plate and takes infection more distal
Main causes of avascular necrosis?
Trauma/ fracture
SCD
Steroids
Caissons