Due to the kindness of Microsoft, Miranda has been furnished with her very own copy of Microsoft® Encarta® Premium Suite 2003. This means that her research on topics of quirky interest will be vast and far reaching. For more information on Encarta or to experience it for yourself, visit: here.
A few weeks ago in the episode of CSI: Crime Scene Investigators called ‘Justice is served,’ Grissam comes across a nutritionist that has trained her dogs to murder people so she can consume their organs. When Grissam discovers that she is a sufferer of porphyria, he comments that she has “The Madness of King George.” Naturally this peaked my interest, so I did some research on the ole’ Encarta….
Porphyria is a rare, usually inherited disorder concerned with the formation of the vital biochemical substance haem. The conditions affect about 1 person in 50,000 in Britain, but are commoner in South Africa where the prevalence may be as high as 1 in 1,000 of the white population. This is thought to have arisen from a gene mutation in one or more individuals that was passed on to descendants.
How Haem Works Haem binds with various proteins to form a range of haemoproteins, of which the most important is haemoglobin, which is the protein and iron-containing compound that fills the red blood cells and transports oxygen from the lungs to the tissues of the body. The other haemoproteins include a number of essential liver enzymes based on the haem molecule. The formation of haem is a complex biochemical process involving, consecutively, a series of nine enzymes.
Causes and Diagnosis The various porphyrias are due to deficiencies of individual enzymes in the process of haem production resulting from mutations in these genes. Because different enzymes may be affected, the porphyrias may take various forms—but in all types of the disease there is overproduction of haem precursor substances. A specific diagnosis of the type is made clinically by determining the proportion of precursor protoporphyrins in the urine and stools. The urine is usually red, but it may be orange or brown, or may darken on exposure to light.
Symptoms
The acute porphyrias are the most severe. They feature intermittent attacks of neurological, digestive system, and skin disorders, which may be precipitated by various drugs and other factors. Acute intermittent porphyria is the commonest and the most severe. Although inherited, the disease inexplicably becomes manifest in females five times as often as in males. Attacks are commonest among sufferers in their 30s. Often there is no known family history and it is thought that the condition sometimes remains latent or unidentified for generations. Nearly all attacks start with severe, diffuse abdominal pain that can often be controlled only by strong painkillers. There is nausea and vomiting. People with first attacks sometimes undergo unnecessary surgery.
Nerve malfunction with tingling progressing to paralysis occurs in some 50 per cent of attacks. In the most severe cases, respiratory paralysis, unless skilfully managed, may prove fatal. Psychiatric manifestations are also a common feature during attacks. These may involve severe agitation, or mania, or depression. Often they include hallucinations which may appear like schizophrenia. Sometimes these patients are misdiagnosed as suffering from a primary psychiatric disorder. The psychiatric complications may, however, persist between attacks.
Treatment
Treatment involves the avoidance, if possible, of precipitating factors; the control of pain; a high carbohydrate intake (intravenously if necessary), and the administration of haem which is available in drug form. Skin photosensitivity may be treated by beta-carotene, and porphyria cutanea tarda responds to the removal of iron. Patients with respiratory paralysis must be maintained on an artificial respirator in an intensive care unit. Recovery is possible but may take months.
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Due to the kindness of Microsoft, Miranda has been furnished with her very own copy of Microsoft® Encarta® Premium Suite 2003. This means that her research on topics of quirky interest will be vast and far reaching. For more information on Encarta or to experience it for yourself, visit: 
A few weeks ago in the episode of CSI: Crime Scene Investigators called ‘Justice is served,’ Grissam comes across a nutritionist that has trained her dogs to murder people so she can consume their organs. When Grissam discovers that she is a sufferer of porphyria, he comments that she has “The Madness of King George.” Naturally this peaked my interest, so I did some research on the ole’ Encarta….
Porphyria is a rare, usually inherited disorder concerned with the formation of the vital biochemical substance haem. The conditions affect about 1 person in 50,000 in Britain, but are commoner in South Africa where the prevalence may be as high as 1 in 1,000 of the white population. This is thought to have arisen from a gene mutation in one or more individuals that was passed on to descendants. 
Haem binds with various proteins to form a range of haemoproteins, of which the most important is haemoglobin, which is the protein and iron-containing compound that fills the red blood cells and transports oxygen from the lungs to the tissues of the body. The other haemoproteins include a number of essential liver enzymes based on the haem molecule. The formation of haem is a complex biochemical process involving, consecutively, a series of nine enzymes. 
The various porphyrias are due to deficiencies of individual enzymes in the process of haem production resulting from mutations in these genes. Because different enzymes may be affected, the porphyrias may take various forms—but in all types of the disease there is overproduction of haem precursor substances. A specific diagnosis of the type is made clinically by determining the proportion of precursor protoporphyrins in the urine and stools. The urine is usually red, but it may be orange or brown, or may darken on exposure to light.
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