Biomaterial Database

Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infant. 1983

H E Branson, and J Katz, and R Marble, and J H Griffin

A coumarin-responsive chronic relapsing purpura fulminans syndrome is described in a protein-C-deficient newborn infant. Episodes of acute disseminated intravascular coagulation (DIC) and cutaneous gangrene, which first appeared at age 11 h, were effectively controlled for 28 months with transfusions of fresh-frozen plasma. Cryoprecipitate and cryoprecipitate-poor plasma induced remissions as long as those induced by fresh-frozen plasma (less than or equal to 72 h). Coumarins sustained a cryoprecipitate-induced remission for 19 days: they were then electively discontinued and 17 h later the patient had an acute exacerbation of DIC with haemorrhaging. Family studies showed protein C levels of 31-40% in the subject's symptom-free mother and full and half brothers. DIC, the coumarin effect, and the inherited protein C abnormality appear to have contributed to the extremely low plasma levels (less than or equal to 6%) of protein C in the patient. This experience suggests that protein C deficiency may greatly compromise the ability of newborn infants to control consumptive disorders.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297	Male		Males
D008661	Metabolism, Inborn Errors	Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.	Inborn Errors of Metabolism,Metabolism Errors, Inborn,Error, Inborn Metabolism,Errors Metabolism, Inborn,Errors Metabolisms, Inborn,Errors, Inborn Metabolism,Inborn Errors Metabolism,Inborn Errors Metabolisms,Inborn Metabolism Error,Inborn Metabolism Errors,Metabolism Error, Inborn,Metabolism Inborn Error,Metabolism Inborn Errors,Metabolisms, Inborn Errors
D010375	Pedigree	The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.	Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D011486	Protein C	A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation.
D011693	Purpura	Purplish or brownish red discoloration, easily visible through the epidermis, caused by hemorrhage into the tissues. When the size of the discolorization is >2-3 cm it is generally called Ecchymoses (ECCHYMOSIS).	Petechiae,Purpuras
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002908	Chronic Disease	Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2).	Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D003374	Coumarins	Synthetic or naturally occurring substances related to coumarin, the delta-lactone of coumarinic acid.	1,2-Benzopyrone Derivatives,1,2-Benzopyrones,Coumarin Derivative,Coumarine,1,2-Benzo-Pyrones,Benzopyran-2-ones,Coumarin Derivatives,Coumarines,1,2 Benzo Pyrones,1,2 Benzopyrone Derivatives,1,2 Benzopyrones,Benzopyran 2 ones,Derivative, Coumarin,Derivatives, 1,2-Benzopyrone,Derivatives, Coumarin
D004211	Disseminated Intravascular Coagulation	A disorder characterized by procoagulant substances entering the general circulation causing a systemic thrombotic process. The activation of the clotting mechanism may arise from any of a number of disorders. A majority of the patients manifest skin lesions, sometimes leading to PURPURA FULMINANS.	Consumption Coagulopathy,Coagulation, Disseminated Intravascular,Disseminated Coagulation, Intravascular,Intravascular Coagulation, Disseminated,Intravascular Disseminated Coagulation,Coagulation, Intravascular Disseminated,Coagulations, Disseminated Intravascular,Coagulations, Intravascular Disseminated,Coagulopathies, Consumption,Coagulopathy, Consumption,Consumption Coagulopathies,Disseminated Coagulations, Intravascular,Disseminated Intravascular Coagulations,Intravascular Coagulations, Disseminated,Intravascular Disseminated Coagulations