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a genetic disorder characterized by a disturbance in porphyrin metabolism with resultant increase in the formation and excretion of porphyrins ( uroporphyrin and coproporphyrin ) or their precursors; called also hematoporphyria. Porphyrins, in combination with iron, form hemes , which in turn combine with specific proteins to form hemoproteins . hemoglobin is a hemoprotein, as are many other substances essential to normal functioning of the cells and tissues of the body.

Two general types are known: the erythropoietic porphyrias, which are concerned with the formation of erythrocytes in the bone marrow; and the hepatic porphyrias, which are responsible for liver dysfunction. Manifestations of porphyria include gastrointestinal, neurologic, and psychologic symptoms, cutaneous photosensitivity, pigmentation of the face (and later of the bones), and anemia with enlargement of the spleen. Large amounts of porphyrins are excreted in the urine and feces.

Treatment of this condition has been primarily symptomatic and varies in its effectiveness. Emphasis is on prevention of attacks by avoiding fasting and drugs that precipitate the symptoms. Photosensitivity may be controlled by avoiding exposure to light. Removal of the spleen is useful in some cases of the erythropoietic type of porphyria. Drug therapy includes the use of phenothiazines, chlorpromazine and promazine in particular. These drugs allay pain and nervousness and apparently allow a period of remission from symptoms. Meperidine hydrochloride (Demerol) may be given for pain and hydroxypheme (Hemetin) is given intravenously to compensate for genetic impairment of heme synthesis.

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Patients with porphyria must not be given barbiturates, sulfonamides, alcohol, or chloroquine as these chemicals may precipitate or intensify attacks. It is recommended that persons with this disease carry with them at all times identification saying that they have porphyria so that in an emergency they will not be given medication that could precipitate an attack or even death.

Valley Park School District

One Main St, Valley Park, MO 63088

(636) 923-3500

Welcome

Valley Park School District is driven by a tradition of strong character and outstanding academic performance. Our district is Accredited with Distinction by the State of Missouri and our schools are regularly acknowledged for their achievements. Find out more about our district and school by clicking here.

Our mission is to provide a supportive, personalized environment in which students will grow strong intellectually, emotionally, physically, and socially today in preparation for tomorrow.

Superintendent:
Dr. Tim Dilg
(636) 923-3695
[email protected]

Upcoming Events

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District Announcements

It’s that time of year again! Cold weather means the possibility of school delays and closures. When this happens parents, students and the community will be notified in multiple ways.

In the event of a snow day, staff, parents and students will be notified through Infinite Campus via text, phone call, and email. Parents can update any emergency notification information here.

Additionally, we will notify all news outlets of the decision to cancel school. We will have an announcement posted on our website and posted on all social media outlets; Facebook, Twitter and Instagram.

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