Pathoma Flashcards
Pathology
Neutrophil
Structure: Multi-lobed nucleus, granules containing lysozyme, myeloperoxidase (enzyme deficiency can cause absence of bleach, but presence of respiratory burst), and hydrolytic enzymes
Functions: Acute inflammatory response
Basophil
Bilobed, nucleus with dark blue granules containing heparin, histamines, leukotrienes
Function: Allergic reaction
Eosinophil
Bilobed nucleus, pinkish granules containing major basic proteins, histamines, arylsulfatase
Function: Defend against parasitic infections, levels increased in asthma, allergic processes, neoplasm, collagen vascular diseases
Synovial Joints
Articular cartilage covers and protects articular surface of long bones, Fibrous capsule and ligament maintains position of long bones in joint.
Synovial membrane: Forms the boundary of the joint space; attached to fibrous capsule.
Synovian: Inner surface of synovial joint capsule
synovial fluid: Formed by the synovium serves to lubricate movement of articular surfaces
Osteogenesis Imperfecta
Pathophysiology: Caused by inherited mutations resulting in deficient synthesis of type 1 collagen (bone, teeth, ears, eyes, and skin). Causes cortical and trabeculae thinning.
Osteogenesis Imperfecta
Inheritance
AD
4-variants, type 2 most sever resulting in perinatal death.
Osteogenesis Imperfecta
4 Clinical manifestations
Treatment and prognosis
Multiple fractures from minimal trauma due to thinning cortical region. Blue sclerae due to translucent of connective tissue over choroids.
Hearing loss due to middle ear abnormality.
Dental imperfections
Treatment: Pneumatic bracing, avoidance of trauma.
Achondroplasia, etiology
AD, mutation in fibroblast growth factor receptor 3 (FGFR3) gene on chromosome 4
Achondroplasia
Clinical manifestation
Cartilage calcification and remodeling.
Dwarfism-Short limbs with normal trunk and narrow epiphyseal plate with short thick bones.
A 2-month-old baby boy is brought to the emergency department by his adoptive parents, who tell you that their baby has been fussy and seems to cry whenever his left arm is touched. On PE, you notice hyperactivity of the baby’s left arm. X-ray of the baby’s arm reveal multiple fractures. Radiographs of the skull, chest, and all extremities looking for evidence of other fractures. On closer of PE, no bruising of the trunk, back, buttocks, making child abuse less likely, but you do observe blue sclerae.
Osteogenesis Imperfect. Due to collagen 1 deficient.
A 15-year old boy presents to the emergency department with suspected fractures of the left tibia and fibula following a fall. He tells you that he has broken several bones in the past and that his mother has a bone disease. PE-slight hearing loss, facial asymmetry and hepatosplenomegaly. Lab-demonstrate anemia and an x-ray of his left leg shows fractures of both the tibia and fibula a well as Erleymeye last of deformity. As you set this patient’s leg in a cast, you suspect that his condition is associated with defective osteoblasts.
Osteoporosis-Dysfunction of osteoclast activity that results in defective bone resorption/ There are multiple variants. AR-fatal
AD-less severe.
Osteoporosis
Pathophysiology+ clinical manifestations
Dysfunction of the osteoclast activity, therefore abnormal bone resorption.
Clinical manifestations
- Multiple fractures
- Anemia (owning to lack of bone marrow)
- Infections
- hepatospenomegaly (owing to extramedullary hematopoiesis)
- cranial nerve palsies (blindness, deafness, and facial paralysis
- narrow neural foramina-cause nerve compression.
Clinical manifestations
- Multiple fractures
- Anemia (owning to lack of bone marrow)
- Infections
- hepatospenomegaly (owing to extramedullary hematopoiesis)
- cranial nerve palsies (blindness, deafness, and facial paralysis
- narrow neural foramina-cause nerve compression.
Osteoperosis
treatment: corticosterone or bone marrow transplant
McCune- Albright syndrome
Disorder that occurs in young girls. Manifests with cafe-au-lait spots, precocious puberty, short stature and polyostotic fibrous dysplasia (fibrous replacement of medullary bone in multiple locations.
Disorder that occurs in young girls. Manifests with cafe-au-lait spots, precocious puberty, short stature and polyostotic fibrous dysplasia (fibrous replacement of medullary bone in multiple locations.
McCune-Albright Syndrome
68-year old woman presents to your odic complaint of aching lower Bain pain. Upon directed history, she tells you that she feels that she is getting shorter and you note that she is slightly hunched forward. An X-ray of the her spine reveals a compression of the fracture of the L4-vertebra. When bone density scan reveals decreased bone mass, you prescribe her a bisphosphonate as well as Calcium and vitamin D supplements to breast her condition.
Osteoporosis
Osteoporosis
A disease characterized by decreased in bone synthesis and increased in bone reabsorption resulting in low bone mass due to estrogen deficiency after menopause.
Osteoporosis
Pathophysiology
Qualitatively normal but with thinner and fewer trabecular.
Thin cortex and widened Haversian systems
Osteoporosis
CM
- Back pain
- Fractures-thoracolumar spine, hip, or femur
- loss of height
- kyphosis (owing to vertebral compression fractures).
Osteoporosis
Testings
Treatment
Dual-energy x-ray-absorptiometry (DEXA) scan
-Radioluecency, decreased bone mass.
Treatment
- Bisphosphonates (inhibits osteoclasts activity)
- Estrogen replacement
- Calcium
- Vitamin D supplements.
74-year old man presents to your clinic complaining of bone pain in his left thigh. He also states that his hearing has deteriorated significantly over the last 6 months. Direct questioning reveals that he has noticed an increase in his hat size. Laboratory results remonstrate an increase in serum aka;ine phosphate with normal calcium, phosphorus, and normal parathyroid levels. X-ray of his left thigh shows mixed thickening and lucency of the bone. You worry that this patient’s condition many put him as rick for osteosarcoma and high output cardiac failure.
Paget disease of Bone
Paget disease of Bone
74-year old man presents to your clinic complaining of bone pain in his left thigh. He also states that his hearing has deteriorated significantly over the last 6 months. Direct questioning reveals that he has noticed an increase in his hat size. Laboratory results remonstrate an increase in serum aka;ine phosphate with normal calcium, phosphorus, and normal parathyroid levels. X-ray of his left thigh shows mixed thickening and lucency of the bone. You worry that this patient’s condition many put him as rick for osteosarcoma and high output cardiac failure.
Paget disease of Bone
Caused by an increase of both osteoblastic activity and osteoclastic activity triggered by a viral infection. (Paramyovirus)
Paget disease of Bone
Involves what bones?
Morphological stages of bone?
Gross- Can be in one bone (monostotic) or many (polystotic) involving skull, pelvis, femur, tibia and spine.
Morphological stages
1) Osteolytic-large osteoclasts with multiple resorption pits
2) Osteoclastic osteoblastic: mosaic pattern of lamellar bone demonstrating both bone destruction and bone formation.
3) Late osteosclerotic: sclerotic bone with predominance of thick trabecular, dark mosaic.
Paget disease of bone
CM
- Back pain
- Bone growth (increase in hat size)
- High output cardiac failure (increase vascularity results in A-v SHUNTS.
- Osteosarcoma
- Hearing loss (due to narrowing of foramen.
- long bone fracture.
Paget disease of bone
Treatment
Lab finding
- Thickening and lucency of bone
- Increase Alkaline phosphatase
- Normal Ca2+, Phosphorus, PTH
Treatment
-Bisphosphonates inhibit osteoclastic activity.
