Biomaterial Database

[A case of systemic Pfeiffer-Weber-Christian Syndrome complicated by DIC (author's transl)]. 1977

A Kosztovics, and J Bédi, and S Egedy

The authors report on a case of fatal systemic P-W-Chr syndrome accompanied by DIC. The coagulation disturbance of the patient stopped after the administration of heparin, but this had no effect on the prognosis of the illness. The basic illness that had caused the syndrome could not be demonstrated either in vivo, or during the autopsy so that the above case is thought to be a sui generis form. According to the author's opinion the DIC is not an inducing factor but an accompanying phenomenon in the course of the basic disease.

UI	MeSH Term	Description	Entries
D010201	Panniculitis, Nodular Nonsuppurative	A form of panniculitis characterized by recurrent episodes of fever accompanied by the eruption of single or multiple erythematous subcutaneous nodules on the lower extremities. They normally resolve, but tend to leave depressions in the skin. The condition is most often seen in women, alone or in association with other disorders.	Weber-Christian Disease,Disease, Weber-Christian,Nodular Nonsuppurative Panniculitides,Nodular Nonsuppurative Panniculitis,Nonsuppurative Panniculitides, Nodular,Nonsuppurative Panniculitis, Nodular,Panniculitides, Nodular Nonsuppurative,Weber Christian Disease
D011379	Prognosis	A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.	Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
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
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults