Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate

Article Abstract:

Normal blood clotting is an important physiological function, as are the normal restraining mechanisms that turn off the clotting process when it is no longer needed. Without appropriate control mechanisms, the normal clotting mechanisms might result in clotting, or thrombosis, throughout the circulatory system. Protein C is a part of the natural anticoagulant process that inactivates clotting factors after they have been activated during the normal clotting process. Protein C circulates in the blood in an inactive form and is converted into the active form by enzymatic cleavage. There have been at least 19 cases reported in which an inherited deficiency in protein C has resulted in life-threatening coagulation disorders in newborns including purpura fulminans, a rapidly progressive and often fatal disease in which bruise-like hemorrhages appear throughout the body. The authors report the case of a newborn boy with protein C deficiency and purpura fulminans who was successfully treated purified protein C. Within 24 hour of birth, the patient developed ecchymosis, the appearance of large irregular hemorrhagic regions in the skin. Laboratory analysis revealed an almost total absence of protein C. Treatment of the patient was begun using plasma, which halted the increase in skin lesions and improved the disseminated blood coagulation in the patient's blood vessels. However, this treatment actually decreased that already low amounts of platelets and increased fragmented red blood cells in the circulation. On the fourth day, treatment with purified protein C was begun. This treatment proved to be successful and was well tolerated as long-term therapy. The patient is now over 23 months old and taking oral anticoagulants. The boy is developing normally and has no signs of heart problems, eye problems, or neurological difficulties. It is likely that the early diagnosis and treatment of this patient not only saved his life, but also prevented irreversible damage to many of his organs from the disseminated blood coagulation. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Care and treatment, Protein C, Protein C deficiency, Disseminated intravascular coagulation

---

Preclinical evidence of Alzheimer's disease in persons homozygous for the epsilon-4 allele for apolipoprotein E

Article Abstract:

Middle-aged people with two copies of the epsilon-4 allele, but without cognitive impairment, may have decreased levels of glucose metabolism in the same parts of the brain as people who appear to have Alzheimer's disease. The epsilon-4 allele for apolipoprotein E is associated with an increased risk of Alzheimer's disease. Researchers administered psychiatric and neurologic tests, as well as positron-emission tomography (PET) scans to 11 people with two copies of the epsilon-4 allele and 22 people (controls) without this allele. All the participants ranged in age from 50 to 65 years and had a family history of Alzheimer's. In comparison to the controls, the epsilon-4 carriers had slightly lower cognitive scores on a few of the tests, but were cognitively normal on the whole. Like people believed to have Alzheimer's, the epsilon carriers had reduced levels of glucose metabolism in the following regions of the brain: posterior cingulate, parietal, temporal, and prefontal.

Genetic aspects, Alzheimer's disease

---

Antenatal glucocorticoid treatment and cystic periventricular leukomalacia in very premature infants

Article Abstract:

The corticosteroid drug betamethasone appears to have fewer side effects than dexamethasone when used to treat very premature fetuses. Many pregnant women at risk of a premature delivery are given corticosteroids before delivery to prevent several complications of premature birth. In a study of 883 premature deliveries, fetuses whose mothers received betamethasone were less likely to develop cystic periventricular leukomalacia than fetuses whose mothers received dexamethasone. Cystic periventricular leukomalacia is a common cause of cerebral palsy.

Complications and side effects, Infants (Premature), Premature infants, Dexamethasone, Betamethasone

Similar abstracts:

• Abstracts: Treatment of speech and voice disorders with botulinum toxin. Fish botulism - Hawaii, 1990
• Abstracts: Transmission of the human immunodeficiency virus in the health care setting - time for action. Screening for HIV infection - benefits and costs
• Abstracts: Diagnosis of adrenal insufficiency. The changing clinical spectrum of adrenal insufficiency. Recovery of adrenal function after failure resulting from traumatic bilateral adrenal hemorrhage
• Abstracts: Decreased stature associated with moderate blood lead concentrations in Mexican-American children. A growth-limiting, mild zinc-deficiency syndrome in some southern Ontario boys with low height percentiles

This website is not affiliated with document authors or copyright owners. This page is provided for informational purposes only. Unintentional errors are possible.