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NBME 7 block 1 q 1 to 50 - maryam2009
#11
8. A
I- Cell Disease: (Inclusion cell disease). Inherited lysosomal storage disorder; failure of addition to mannose 6 phosphate to lysosome proteins( enzime are secreted outside the cell instead of being targeted to the lysosme. Result in coarse facial future, clouded corneas, restricted join movement, and high plama levels of lysosomes. Often fatal childhood.
http://en.wikipedia.org/wiki/I-cell_disease
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#12
hi there
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#13
9.BB

10.EE

Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body.

The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. Certain muscles such as those that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are often, but not always, involved in the disorder.

In myasthenia gravis, antibodies block, alter, or destroy the receptors for acetylcholine at the neuromuscular junction which prevents the muscle contraction from occurring
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#14
hi there,keep going,I'll come later,Thank you
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#15
11. A

A constant rate of infusion of GnHR will cause a transient increase in LH and FHS secretion, followed by a decrease caused by the downregulation of gonadotroph receptors. kaplan physiology (Page 350).
http://en.wikipedia.org/wiki/Gonadotropi...ng_hormone
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#16
12. B
Crest Syndrome: Calcifications, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Limited skin involvement, often confined to fingers and face. More benign clinical course. Associated with Anticentromere Antibody.
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#17
13. A
Acute appendicitis
http://en.wikipedia.org/wiki/Appendicitis
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#18
14. D

AIDS
Parvovirus B19 is a cause of chronic anemia in individuals who have AIDS. It is frequently overlooked. Treatment with erythropoetin or intravenous immunoglobulin have been helpful in some patients. The parvovirus infection may trigger an inflammatory reaction in AIDS patients who have just begun antiretroviral therapy.[14].

http://en.wikipedia.org/wiki/Parvovirus_B19
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#19
15. G
When associated with the lung, it often causes ectopic production of parathyroid hormone-related protein (PTHrP), resulting in hypercalcemia.
Squamous-cell carcinoma of the lung is more common in men than in women. It is closely correlated with a history of tobacco smoking, more so than most other types of lung carcinoma. It most often arises centrally in larger bronchi, and while it often metastasizes to locoregional lymph nodes (particularly the hilar nodes) early in its course, it generally disseminates outside the thorax somewhat later than other major types. Large tumors may undergo central necrosis, resulting in cavitation. A squamous-cell carcinoma is often preceded for years by squamous-cell metaplasia or dysplasia in the respiratory epithelium of the bronchi, which later transforms to carcinoma in situ. In carcinoma in situ, atypical cells may be identified by cytologic smear test of sputum, bronchoalveolar lavage or samples from endobronchial brushings. However, squamous-cell carcinoma in situ is asymptomatic and undetectable on X-ray radiographs. Eventually, it becomes symptomatic, usually when the tumor mass begins to obstruct the lumen of a major bronchus, often producing distal atelectasis and infection. Simultaneously, the lesion invades into the surrounding pulmonary substance. On histopathology, these tumors range from well-differentiated, showing keratin pearls and cell junctions, to anaplastic with only minimal residual squamous-cell features.
http://en.wikipedia.org/wiki/Squamous-cell_carcinoma
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#20
16.AA

Paget's disease may be diagnosed using one or more of the following tests:

Pagetic bone has a characteristic appearance on X-rays. A skeletal survey is therefore indicated.

http://upload.wikimedia.org/wikipedia/co..._skull.jpg

An elevated level of alkaline phosphatase in the blood in combination with normal calcium, phosphate, and aminotransferase levels in an elderly patient are suggestive of Paget's disease.
Bone scans are useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone(s) should be X-rayed to confirm the diagnosis

The pathogenesis of Paget's disease is described in 3 stages, which are:
i. Osteoclastic activity
ii. Mixed osteoclastic-osteoblastic activity
iii. Exhaustive (burnt out) stage


Initially, there is a marked increase in the rate of bone resorption at localized areas caused by large and numerous osteoclasts. These localized areas of osteolysis are seen radiologically as an advancing lytic wedge in long bones or osteoporosis circumscripta in the skull. The osteolysis is followed by a compensatory increase in bone formation induced by osteoblasts recruited to the area. This is associated with accelerated deposition of lamellar bone in a disorganized fashion. This intense cellular activity produces a chaotic picture of trabecular bone ("mosaic" pattern), rather than the normal linear lamellar pattern. The resorbed bone is replaced and the marrow spaces are filled by an excess of fibrous connective tissue with a marked increase in blood vessels, causing the bone to become hypervascular. The bone hypercellularity may then diminish, leaving a dense "pagetic bone," also known as burned-out Paget's disease.

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