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Forensic Pathology for Police - Brainshare Public Online Library

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Multisystem and Other Disorders 251<br />

with its own degree of severity. Clinically, patients with CF have abnormally viscous<br />

(thick) secretions. The lungs, pancreas, small intestines, and salivary glands<br />

can become obstructed, damaged, and infected, leading to death. Sweat glands are<br />

also affected, leading to the commonly-employed “sweat chloride test” (in living<br />

patients).<br />

Ehlers–Danlos Syndrome: Ehlers–Danlos syndrome is a group of genetic disorders,<br />

each with an abnormality in genes responsible <strong>for</strong> normal collagen <strong>for</strong>mation.<br />

The most serious type is type IV, in which arteries and organs (intestines, spleen,<br />

uterus) are at risk <strong>for</strong> rupture. These patients may or may not have a history of<br />

easy bruising. They may or may not have hyperflexible skin and hypermobile joints<br />

(double-jointed).<br />

Marfan Syndrome: Marfan syndrome is a genetic disorder characterized by<br />

abnormalities in the gene <strong>for</strong> a connective tissue substance called fibrillin-1.<br />

Clinically, patients tend to be very tall and lanky, with long fingers. Abnormalities<br />

of the skeleton, eyes, and cardiovascular system are common. Most significant in<br />

terms of death investigations are a predisposition to thoracic aortic dissections and<br />

mitral valve prolapse.<br />

Sarcoidosis<br />

Sarcoidosis is a disorder characterized by the presence of multiple “granulomas”<br />

(inflammatory nodules containing lymphocytes and macrophages). The cause of the<br />

disease is unknown. No micro-organism or <strong>for</strong>eign substance can be identified in the<br />

granulomas. The disease can affect anyone, but women and those of African descent<br />

are at greatest risk. The granulomas can become quite large, and tend to involve the<br />

lymph nodes, particularly those within the mediastinum (central thorax area), where<br />

the enlarged nodes are sometimes referred to as “potato nodes” (Disc Image 10.54).<br />

The lungs are also frequently involved, sometimes quite extensively. Death can be<br />

related to severe respiratory compromise, but more frequently is related to the fact<br />

that the heart can also be involved. Virtually any other organ can also be involved<br />

with the disease.<br />

Psychiatric Disease<br />

A variety of psychiatric conditions may be associated with sudden, unexpected<br />

death, including various eating disorders (see Chapter 21), and numerous disorders<br />

characterized by psychotic behavior, such as schizophrenia or manic-depression.<br />

Some persons are at risk <strong>for</strong> various non-natural events, such as suicide or adverse<br />

drug reactions. Some individuals may experience excited delirium followed by sudden<br />

death. The excited delirium is essentially identical to that seen in drug-induced<br />

excited delirium, such as occurs with cocaine (refer to Chapter 11). A ruling on such<br />

a case might read as follows: “excited delirium due to schizophrenia.” Occasionally,<br />

the condition is referred to as “acute exhaustive mania.”

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