Block 10 Bone And Arthritis - Pt.1
Questions and Answers
- 1.
The presence of splenomegaly in a patient with osteopetrosis is usually due to:
- A.
Extramedullary hematopoiesis
- B.
Leukemia
- C.
Hemolysis
- D.
Adominal hemorrhage
- E.
Sequestered osteoclasts
Correct Answer
A. Extramedullary hematopoiesisExplanation
In osteopetrosis, there is a defect in bone resorption leading to increased bone density. This can result in decreased bone marrow space and impaired hematopoiesis. As a compensatory mechanism, hematopoiesis occurs in extramedullary sites, such as the liver and spleen. Splenomegaly is commonly seen in osteopetrosis due to the increased workload of extramedullary hematopoiesis in the spleen. Therefore, extramedullary hematopoiesis is the most likely explanation for the presence of splenomegaly in a patient with osteopetrosis.Rate this question:
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- 2.
A disease characterized by failure to mineralize within the zone of provisional calcification occurs with.
- A.
Paget's disease of bone
- B.
Rickets
- C.
Congenital syphilis
- D.
Osteoarthritis
- E.
Scurvy
Correct Answer
B. RicketsExplanation
Rickets is a disease that is characterized by failure to mineralize within the zone of provisional calcification. This means that the process of mineralization, which is necessary for the proper development and strengthening of bones, is impaired in individuals with rickets. This condition is typically caused by a deficiency in vitamin D, calcium, or phosphate, which are essential for bone health. Rickets can lead to skeletal deformities, weak and brittle bones, and growth retardation in children.Rate this question:
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- 3.
Brown tumors of bone are the result of
- A.
Occlusion of the nutrient artery
- B.
Hyperparathyroidism
- C.
Trauma
- D.
Metastatic carcinoma
- E.
Vitamin deficiency
Correct Answer
B. HyperparathyroidismExplanation
Brown tumors of bone are a rare complication of hyperparathyroidism, a condition characterized by excessive production of parathyroid hormone (PTH). Hyperparathyroidism leads to increased levels of calcium in the blood, which can cause the formation of bone tumors. These tumors are called brown tumors due to their characteristic brown color. They are not true tumors but rather a reactive process in response to the hormonal imbalance. Brown tumors can occur in any bone but are most commonly found in the long bones, ribs, and pelvis. Treatment involves addressing the underlying hyperparathyroidism and restoring normal calcium levels.Rate this question:
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- 4.
The pathogenic mechanism involved in osteogenesis imperfecta is a/an
- A.
Hereditary overgrowth of cortical bone
- B.
Failure of cartilage cell proliferation
- C.
Hereditary defect in synthesis of elastic fibers
- D.
Acquired vitamin deficiency
- E.
Hereditary defect in synthesis of collagen
Correct Answer
E. Hereditary defect in synthesis of collagenExplanation
Osteogenesis imperfecta is a genetic disorder characterized by brittle bones that are prone to fracture. The correct answer is "hereditary defect in synthesis of collagen" because collagen is a major component of bone tissue and is responsible for its strength and flexibility. In osteogenesis imperfecta, there is a defect in the production of collagen, leading to weak and fragile bones. This defect can be inherited from one or both parents and affects the body's ability to produce normal collagen, resulting in the symptoms seen in osteogenesis imperfecta.Rate this question:
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- 5.
An 18‑year‑old woman develops a sore, red, hot, swollen left knee. Fluid from the joint space is thin and cloudy, contains many neutrophils, and clots spontaneously. Which of the following types of arthritis best fits these findings?
- A.
Rheumatoid
- B.
Salmonella
- C.
Gonococcal
- D.
Gouty
- E.
Tuberculous
Correct Answer
C. GonococcalExplanation
This happened because something else was "red hot and swollen" at splash bashRate this question:
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- 6.
Rheumatoid arthritis usually has its origin in
- A.
Major weight bearing joints
- B.
Ball and socket joints
- C.
Lumbar sacral joints
- D.
Small peripheral joints
- E.
Knee and elbow joints
Correct Answer
D. Small peripheral jointsExplanation
Rheumatoid arthritis usually has its origin in small peripheral joints. This means that the condition typically starts in joints such as those in the hands, wrists, feet, and ankles. Rheumatoid arthritis is an autoimmune disease that causes inflammation and damage to the lining of these joints. It can eventually spread to other joints in the body, but it commonly begins in the smaller joints.Rate this question:
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- 7.
A 55‑year‑old man presents with painful hips bilaterally. Radiographs show marked "joint space" narrowing, osteophyte formation and round radiolucent cystic zones in the subchondral bone of both femoral heads. The BEST diagnosis for the joint condition in this patient is
- A.
Infectious arthritis
- B.
Osteoarthritis
- C.
Psoriatic arthritis
- D.
Ankylosing spondylitis
- E.
Rheumatoid arthritis
Correct Answer
B. OsteoarthritisExplanation
The given scenario describes a 55-year-old man with painful hips bilaterally. The radiographs show joint space narrowing, osteophyte formation, and round radiolucent cystic zones in the subchondral bone of both femoral heads. These findings are characteristic of osteoarthritis, which is the degenerative joint disease typically associated with aging. Infectious arthritis is unlikely as there are no signs of infection mentioned. Psoriatic arthritis, ankylosing spondylitis, and rheumatoid arthritis usually present with different clinical and radiographic features. Therefore, osteoarthritis is the most appropriate diagnosis in this case.Rate this question:
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- 8.
A 50‑year‑old woman with low back pain has several discrete lytic lesions in the lumbar back and pelvis, and elevated alkaline phosphatase. Serum calcium, serum protein and peripheral blood smears are normal. Aspiration biopsy of a pelvic lesion is MOST LIKELY to show
- A.
Metastatic carcinoma
- B.
Leukemia
- C.
Neoplastic plasma cells
- D.
Osteoporosis
- E.
Osteosarcoma
Correct Answer
A. Metastatic carcinomaExplanation
The presence of lytic lesions in the lumbar back and pelvis, along with elevated alkaline phosphatase, suggests a metastatic carcinoma. Metastatic carcinoma refers to the spread of cancer from its primary site to other parts of the body. The normal serum calcium, serum protein, and peripheral blood smears indicate that there is no involvement of the bone marrow or multiple myeloma. Osteoporosis is a condition characterized by low bone density and is unlikely to present with discrete lytic lesions. Osteosarcoma is a primary bone tumor and would not be expected to present with multiple lesions.Rate this question:
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- 9.
The appearance of multiple "punched out" lesions of bone (as detected by radiography) is highly suggestive of
- A.
Osteogenic sarcoma
- B.
Malignant giant cell tumor of bone
- C.
Multiple myeloma
- D.
Osteogenesis imperfecta
- E.
Paget's disease
Correct Answer
C. Multiple myelomaExplanation
The appearance of multiple "punched out" lesions of bone on radiography is highly suggestive of multiple myeloma. Multiple myeloma is a type of cancer that affects plasma cells in the bone marrow, leading to the formation of these characteristic lesions. These lesions are caused by the destruction of bone tissue by the abnormal plasma cells. Other conditions such as osteogenic sarcoma, malignant giant cell tumor of bone, osteogenesis imperfecta, and Paget's disease may also cause bone lesions, but the presence of multiple punched out lesions is most commonly associated with multiple myeloma.Rate this question:
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- 10.
A 15‑year‑old male is admitted to the hospital complaining of pain and swelling in the left leg. The pain has been present for two months but has become progressively worse over this period. Examination reveals mild fever, and a tender fusiform swelling of the left femur. X‑ray left femur shows a lvtic lesion in the medulla of midshaft, with cortical destruction and onion‑skin appearance. Chest x‑ray is normal. Karyotypic studies show translocation of the long arms of chromosomes 11 and 22. Biopsy of the bone lesion would MOST LIKELY reveal
- A.
Delicate trabeculae of woven bone surrounded by spindle cell stroma
- B.
Sheets of uniform small lymphocyte like cells, tending to form pseudorosettes
- C.
Polygonal cells surrounded by a lace like type of hyaline cartilage
- D.
Malignant mesenchymal cells making osteoid
- E.
Osteoclast like multinucleated giant cells in a plump spindle cell background
Correct Answer
B. Sheets of uniform small lymphocyte like cells, tending to form pseudorosettesExplanation
Ewings Sarcoma:
a primitive neuroectodermal tumor (PNET) of children under 20 yrs. Diaphysis, invades cortex & raises the periosteum --> onion-skin layers of new bone around the diaphysis (# x-ray sign)
sheets of small round cells (may resemble lymphoma or neuroblastoma)& occasional Homer-Wright pseudorosettes
may be mistaken for acute osteomyelitis
combined therapy --> 75% 5-year survivalRate this question:
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- 11.
A 55‑year‑old man has observed vague bony pains in the last few months and, more recently, that his favorite hat no longer seems to fit. On examining him you observe that the skin over one of the painful bony areas is distinctly warm. At this point you are leaning towards a diagnosis of
- A.
Osteopetrosis
- B.
Mucopolysaccharidosis
- C.
Osteomylitis
- D.
Paget's disease
- E.
Septic arthritis
Correct Answer
D. Paget's diseaseExplanation
The symptoms described in the question, including vague bony pains and a change in the fit of his hat, along with the observation of warm skin over a painful bony area, are consistent with Paget's disease. Paget's disease is a chronic disorder that affects the normal remodeling of bone, leading to weakened and enlarged bones. It can cause bone pain, deformities, and changes in hat size due to skull involvement. The warm skin over the affected area suggests increased blood flow and inflammation, which is commonly seen in Paget's disease.Rate this question:
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- 12.
A 68‑year‑old post‑menopausal woman has been experiencing pain in the upper portion of her back, is developing a "hump" back and her children tell her that she seems to be getting shorter. This condition might have been prevented had she taken:
- A.
Vitamin A supplements
- B.
Hormone replacement therapy
- C.
Corticosteroids
- D.
Antibiotics
- E.
A glass of wine daily
Correct Answer
B. Hormone replacement therapyExplanation
Hormone replacement therapy (HRT) is the correct answer because the symptoms described in the question are consistent with osteoporosis, a condition characterized by bone loss and increased risk of fractures. Post-menopausal women are particularly susceptible to osteoporosis due to the decline in estrogen levels, which plays a crucial role in maintaining bone density. HRT, which involves the use of estrogen and sometimes progestin, can help prevent or slow down bone loss and reduce the risk of fractures in post-menopausal women. Therefore, if the woman had taken hormone replacement therapy, it could have potentially prevented or minimized the development of her symptoms.Rate this question:
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- 13.
A 35‑year‑old female several weeks after a flu‑like illness developed weakness in the distal portion of the extremities, which rapidly ascended to involve the proximal muscle groups as well. The weakness extended to involve the respiratory muscles necessitating ventilatory support. The patient's condition improved after plasmapheresis and she completely recovered within three months. This patient MOST LIKELY had which of the following?
- A.
Guillain Barre Syndrome
- B.
A toxic neuropathy
- C.
An acute attack of Multiple Sclerosis
- D.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
- E.
Polymyositis
Correct Answer
A. Guillain Barre SyndromeExplanation
The patient's symptoms of weakness starting distally and rapidly ascending to involve proximal muscle groups, as well as the involvement of respiratory muscles requiring ventilatory support, are consistent with Guillain Barre Syndrome. Plasmapheresis is a treatment commonly used for this condition, and the fact that the patient completely recovered within three months further supports this diagnosis.Rate this question:
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- 14.
A 45‑year‑old female presents with the gradual onset of symmetric proximal muscle weakness. A light‑purple skin rash is also noted over the upper eyelids. A muscle biopsy reveals perifascicular atrophy. This patient MOST LIKELY has which of the following?
- A.
Dermatomyositis
- B.
Amyotrophic Lateral Sclerosis
- C.
Polymyositis
- D.
Myotonic Dystrophy
- E.
Becker Muscular Dystrophy
Correct Answer
A. DermatomyositisExplanation
The patient's presentation of symmetric proximal muscle weakness, light-purple skin rash over the upper eyelids, and perifascicular atrophy on muscle biopsy is consistent with dermatomyositis. Dermatomyositis is an autoimmune inflammatory myopathy that primarily affects the muscles and skin. The characteristic skin rash, known as heliotrope rash, is often seen in dermatomyositis. This condition typically affects women in their 40s to 60s and can be associated with other systemic symptoms such as fatigue, joint pain, and lung involvement.Rate this question:
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- 15.
A 62‑year‑old male developed a rapidly progressive dementia accompanied by myoclonus and ataxia. The patient expired 8 months after the onset of symptoms. At autopsy, spongiform degeneration was noted in the cerebral cortex, basal ganglia and cerebellum. The MOST LIKELY diagnosis is:
- A.
Creutzfeldt jakob Disease
- B.
Alzheimer Disease
- C.
Arbovirus Encephalitis
- D.
Huntington Disease
- E.
Progressive Multifocal Leukoencephalopathy
Correct Answer
A. Creutzfeldt jakob DiseaseExplanation
The patient's symptoms of rapidly progressive dementia, myoclonus, and ataxia, along with the presence of spongiform degeneration in the cerebral cortex, basal ganglia, and cerebellum, are characteristic of Creutzfeldt-Jakob Disease (CJD). CJD is a rare, degenerative neurological disorder caused by abnormal proteins called prions. It leads to the destruction of brain tissue and rapidly progressive neurological symptoms. The other options, such as Alzheimer's disease, arbovirus encephalitis, Huntington's disease, and progressive multifocal leukoencephalopathy, do not match the specific clinical and pathological findings described in the case.Rate this question:
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- 16.
An 18‑year‑old male presented with the acute onset of alterations in mood, behavior and memory. He subsequently went into a coma and died one week after the onset of symptoms. At autopsy, the brain exhibited a hemorrhagic necrotizing encephalitis, which was localized to the temporal lobes. The MOST LIKELY causative agent is:
- A.
HIV 1
- B.
HSV 1
- C.
HSV 2
- D.
CMV
- E.
Rabies virus
Correct Answer
B. HSV 1Explanation
The most likely causative agent for the presented symptoms and findings is HSV 1 (Herpes Simplex Virus 1). HSV 1 is known to cause encephalitis, which is inflammation of the brain. The symptoms of alterations in mood, behavior, and memory followed by coma and death are consistent with the progression of HSV 1 encephalitis. The localization of the hemorrhagic necrotizing encephalitis to the temporal lobes further supports the diagnosis, as HSV 1 commonly affects this area of the brain.Rate this question:
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- 17.
A 42‑year‑old Caucasian male of European decent developed the gradual onset of involuntary jerky movements involving the entire body. He also became depressed and developed a progressive dementia. He expired at age 57. His father died from this same disorder at age 59. Examination of this patient's brain at autopsy would MOST LIKELY reveal:
- A.
Neuritic plaques and granulovacuolar degeneration in the hippocampus
- B.
Marked atrophy of the caudate nucleus with neuronal loss and gliosis
- C.
Spongiform change and kuru plaques in the cerebral and cerebellar cortices
- D.
Lewy bodies and decreased numbers of pigmented neurons in the substantia nigra
- E.
Microglial nodules and foci of neuronophagia in the basal ganglia
Correct Answer
B. Marked atrophy of the caudate nucleus with neuronal loss and gliosisExplanation
The patient's symptoms of involuntary jerky movements, progressive dementia, and a positive family history suggest a neurodegenerative disorder. The most likely diagnosis is Huntington's disease, which is characterized by marked atrophy of the caudate nucleus with neuronal loss and gliosis. This is confirmed by the patient's age at onset and the fact that his father also had the same disorder. Examination of the patient's brain at autopsy would reveal these characteristic changes in the caudate nucleus.Rate this question:
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- 18.
Which of the following statements regarding neurofibromas is CORRECT?
- A.
A. Axons do not course through the tumor
- B.
B. Plexiform neurofibromas are associated with Neurofibromatosis Type 1
- C.
C. They commonly involve cranial nerves
- D.
D. They have Antoni A and Antoni B areas
Correct Answer
B. B. Plexiform neurofibromas are associated with Neurofibromatosis Type 1Explanation
Plexiform neurofibromas are indeed associated with Neurofibromatosis Type 1. Neurofibromatosis Type 1 is a genetic disorder characterized by the growth of tumors along the nerves, and plexiform neurofibromas are a specific type of tumor that commonly occurs in individuals with this condition. These tumors can cause various complications and symptoms depending on their location and size. Therefore, option B is the correct statement regarding neurofibromas.Rate this question:
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- 19.
A patient with a swollen knee has a joint aspirate. The aspirate is cloudy and lab exam shows monosodium urate crystals. The diagnosis is MOST LIKELY to be:
- A.
Gout
- B.
Osteoarthritis
- C.
SLE
- D.
Pseudogout
- E.
Rhematoid arthritis
Correct Answer
A. GoutExplanation
The presence of monosodium urate crystals in the joint aspirate indicates the deposition of uric acid crystals in the joint, which is a characteristic feature of gout. Gout is a form of arthritis that occurs due to the buildup of uric acid in the body, leading to the formation of crystals in the joints. This causes inflammation, pain, and swelling in the affected joints, commonly the big toe, ankle, or knee. Therefore, the diagnosis in this case is most likely to be gout.Rate this question:
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- 20.
A 24‑year‑old woman is anemic and has a blood transfusion. A direct Coombs test is then found to be positive. The MOST LIKELY reason for this is:
- A.
Sickle cell disease
- B.
Hereditary spherocytosis
- C.
Paroxysmal nocturnal hemoglobinemia
- D.
Incompatible blood transfusion
- E.
Drug sensitive hemolytic anemia
Correct Answer
D. Incompatible blood transfusionExplanation
The positive direct Coombs test suggests that there are antibodies coating the patient's red blood cells. This is most likely due to an incompatible blood transfusion, where the patient's immune system recognizes the transfused blood as foreign and produces antibodies against it. This can lead to hemolysis and anemia. Sickle cell disease, hereditary spherocytosis, paroxysmal nocturnal hemoglobinemia, and drug-sensitive hemolytic anemia do not typically cause a positive Coombs test in the context of a blood transfusion.Rate this question:
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- 21.
A Caucasian male at age 65 began to exhibit signs and symptoms of dementia. His dementia gradually progressed to the point where he was mute and bedridden and he died of pneumonia at age 76. At autopsy, histologic sections from the brain revealed numerous neurofibrillary tangles and neuritic plaques. The pathogenesis of this disorder is currently believed to be related to which of the following?
- A.
JC virus infection of oligodendrocytes
- B.
Accumulation of a-synuclein within CNS neurons
- C.
Accumulation of an abnormal prion protein within CNS neurons
- D.
Accumulation of amyloid b peptide within CNS neurons
- E.
Accumulation of huntingtin within CNS neurons
Correct Answer
D. Accumulation of amyloid b peptide within CNS neuronsExplanation
The correct answer is "Accumulation of amyloid b peptide within CNS neurons." This is because the presence of numerous neurofibrillary tangles and neuritic plaques in the brain, along with the patient's symptoms of dementia, are characteristic of Alzheimer's disease. In Alzheimer's disease, there is an abnormal accumulation of amyloid beta peptide, which forms plaques, and tau protein, which forms tangles, within the neurons of the central nervous system. This accumulation leads to neuronal dysfunction and ultimately results in the progressive cognitive decline seen in dementia.Rate this question:
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- 22.
Your patient has been diagnosed with Berger's Disease (IgA nephropathy). Important in helping you make the diagnosis was
- A.
Decreased serum C1q and C4 with absent glomerular C1q and C4.
- B.
Increased serum C1q and C4 and absent glomerular C1q and C4.
- C.
Decreased serum C1q and C4 and increased glomerular C1q and C4
- D.
Increased serum and glomerular C1q and C4.
- E.
Increased serum C1q and C3 and decreased glomerular C1q and C3
Correct Answer
A. Decreased serum C1q and C4 with absent glomerular C1q and C4.Explanation
The correct answer is "decreased serum C1q and C4 with absent glomerular C1q and C4." This answer suggests that the patient has Berger's Disease (IgA nephropathy). In this condition, there is deposition of IgA immune complexes in the glomeruli of the kidneys, leading to inflammation and damage. The decreased serum levels of C1q and C4 indicate activation of the classical complement pathway, which is commonly seen in immune complex-mediated diseases. The absence of C1q and C4 in the glomeruli further supports the diagnosis, as it suggests deposition of immune complexes in the kidney tissue.Rate this question:
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