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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathology
|MainCategory=Pathology
Line 20: Line 20:
|MainCategory=Pathology
|MainCategory=Pathology
|SubCategory=Musculoskeletal/Rheumatology
|SubCategory=Musculoskeletal/Rheumatology
|Prompt=A 12 year old boy is brought by his mother to the physician's clinic for multiple bone fractures and abnormal facial bone.  Lab work-up reveals no abnormality.  After further imaging and genetic testing, the diagnosis is made.  The physician explains to the mother that her child's condition is caused by primary spongiosa filling the medullary cavity and causing an "Erlenmeyer flask" bones on xray.  Which of the following the most likely diagnosis?
|Prompt=A 12-year-old male is brought by his mother to the physician's office for multiple bone fractures.  While lab work-up reveals no abnormality, following further imaging and genetic testing, you diagnose the patient with a condition caused by primary spongiosa filling the medullary cavity, manifesting with "Erlenmeyer flask" bones on x-ray.  Which of the following diagnoses most likely applies to this patient?
|Explanation=Osteopetrosis (marble bone disease) is a disease that is characterized by failure of nromal bone resorption.  It causes thickening of bones that become dense but more predisposed to fractures. On xray, "Erlenmeyer flask" appearance of bones is characteristic.  Osteopetrosis is further described as primary spongiosa filling the medullary cavity.  However, the diagnosis may be missed by lab work-up because serum values of calcium, phosphage, and ALP are typically within normal limits.   
|Explanation=[[Osteopetrosis]] (marble bone disease), a disease characterized anormal bone resorption, causes thickening of bones that become dense and predisposed to fractures. An "Erlenmeyer flask" appearance of bones is characteristic upon x-ray.  [[Osteopetrosis]] results from primary spongiosa filling the medullary cavity, but the diagnosis may be overlooked by lab work-up because serum values of calcium, phosphage, and ALP are typically within normal limits.   


The primary defect in osteopetrosis is believed to be due to abnormal functioning of osteoclasts, which are derived from monocyte lineage.  As a result, bone marrow transplantation (BMT) is thought to be the only appropriate cure for patients with osteopetrosis.
The primary defect in [[osteopetrosis]] is due to abnormal functioning of osteoclasts, which are derived from monocyte lineage.  As a result, bone marrow transplantation (BMT) is thought to be the only appropriate cure for patients with [[osteopetrosis]].


Educational Objective:
|EducationalObjectives= [[Osteopetrosis]] is characterized by abnormal bone resorption, due to a defect of osteoclasts.  In [[osteopetrosis]], primary spongiosa fills the medullary cavity and an "Erlenmeyer flask" appearance of bones is observed upon x-ray. Lab values are usually within normal limits.
Osteopetrosis is characterized by failure of normal bone resorption due to defect of osteoclasts.  In osteopetrosis, primary spongiosa fills the medullary cavity. Lab values are usually within normal limits.  Xray often shows visible "Erlenmeyer flask" bones.
|AnswerA=Osteoporosis
|AnswerA=Osteoporosis
|AnswerAExp=Osteoporosis is characterized by loss of interconnections of trabeculated bone.
|AnswerAExp=Osteoporosis is characterized by the loss of interconnections of trabeculated bone.
|AnswerB=Osteopetrosis
|AnswerB=Osteopetrosis
|AnswerBExp=Osteopetrosis is characterized by primary spongiosa filling the medullary cavity.
|AnswerBExp=[[Osteopetrosis]] is characterized by primary spongiosa filling the medullary cavity.
|AnswerC=Paget's disease
|AnswerC=Paget's disease
|AnswerCExp=Paget's disease is characterized by abnormal bone architecture with mosaic bone pattern that cause long bone chalk-stick fractures.
|AnswerCExp=[[Paget's disease]] is characterized by abnormal bone architecture, manifesting as a mosaic bone pattern, resulting in long bone chalk-stick fractures.
|AnswerD=Osteomalacia
|AnswerD=Osteomalacia
|AnswerDExp=Osteomalacia is characterized by defective mineralization or calcification of osteoid.
|AnswerDExp=[[Osteomalacia]] is characterized by the defective mineralization or calcification of osteoid.
|AnswerE=Polyostotic fibrous dysplasia
|AnswerE=Polyostotic fibrous dysplasia
|AnswerEExp=Polyostotic fibrous dysplasia is characterized by the replacement of bone by fibroblasts, colalgen, and irregular bone trabeculae.
|AnswerEExp=[[Polyostotic fibrous dysplasia]] is characterized by the replacement of bone by fibroblasts, colalgen, and irregular bone trabeculae.
|RightAnswer=B
|RightAnswer=B
|WBRKeyword=osteopetrosis, marble, bone, disease, marble bone disease, calcium, phosphate, ALP, alkaline, phosphatase, alkaline phosphatase, osteoclast, defect, bone, marrow, transplantation, bone marrow transplantation, BMT, spongiosa, medullary, cavity, flask, erlenmeyer, xray
|WBRKeyword=osteopetrosis, bone, marble bone disease, calcium, phosphate, ALP, alkaline phosphatase, osteoclast, bone marrow transplantation, BMT, spongiosa, medullary cavity, skeletal, formation abnormality, x-ray
|Approved=No
|Approved=Yes
}}
}}

Revision as of 17:34, 28 July 2014
Author [[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Musculoskeletal/Rheumatology
Prompt [[Prompt::A 12-year-old male is brought by his mother to the physician's office for multiple bone fractures. While lab work-up reveals no abnormality, following further imaging and genetic testing, you diagnose the patient with a condition caused by primary spongiosa filling the medullary cavity, manifesting with "Erlenmeyer flask" bones on x-ray. Which of the following diagnoses most likely applies to this patient?]]
Answer A AnswerA::Osteoporosis
Answer A Explanation AnswerAExp::Osteoporosis is characterized by the loss of interconnections of trabeculated bone.
Answer B AnswerB::Osteopetrosis
Answer B Explanation [[AnswerBExp::Osteopetrosis is characterized by primary spongiosa filling the medullary cavity.]]
Answer C AnswerC::Paget's disease
Answer C Explanation [[AnswerCExp::Paget's disease is characterized by abnormal bone architecture, manifesting as a mosaic bone pattern, resulting in long bone chalk-stick fractures.]]
Answer D AnswerD::Osteomalacia
Answer D Explanation [[AnswerDExp::Osteomalacia is characterized by the defective mineralization or calcification of osteoid.]]
Answer E AnswerE::Polyostotic fibrous dysplasia
Answer E Explanation [[AnswerEExp::Polyostotic fibrous dysplasia is characterized by the replacement of bone by fibroblasts, colalgen, and irregular bone trabeculae.]]
Right Answer RightAnswer::B
Explanation [[Explanation::Osteopetrosis (marble bone disease), a disease characterized anormal bone resorption, causes thickening of bones that become dense and predisposed to fractures. An "Erlenmeyer flask" appearance of bones is characteristic upon x-ray. Osteopetrosis results from primary spongiosa filling the medullary cavity, but the diagnosis may be overlooked by lab work-up because serum values of calcium, phosphage, and ALP are typically within normal limits.

The primary defect in osteopetrosis is due to abnormal functioning of osteoclasts, which are derived from monocyte lineage. As a result, bone marrow transplantation (BMT) is thought to be the only appropriate cure for patients with osteopetrosis.
Educational Objective: Osteopetrosis is characterized by abnormal bone resorption, due to a defect of osteoclasts. In osteopetrosis, primary spongiosa fills the medullary cavity and an "Erlenmeyer flask" appearance of bones is observed upon x-ray. Lab values are usually within normal limits.
References: ]]

Approved Approved::Yes
Keyword WBRKeyword::osteopetrosis, WBRKeyword::bone, WBRKeyword::marble bone disease, WBRKeyword::calcium, WBRKeyword::phosphate, WBRKeyword::ALP, WBRKeyword::alkaline phosphatase, WBRKeyword::osteoclast, WBRKeyword::bone marrow transplantation, WBRKeyword::BMT, WBRKeyword::spongiosa, WBRKeyword::medullary cavity, WBRKeyword::skeletal, WBRKeyword::formation abnormality, WBRKeyword::x-ray
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