Year: 1968
Type: article
Study design: Case Report
Abstract: The first clear account of this syndrome was published by Gasser et al. (1955), who gave it its name.Since then some 200 cases have been reported from all parts of the world (Piel and Phibbs, 1966).The syndrome is characterized by non-immune, Coombs negative, haemolytic anaemia, with bizarre red cells, variable thrombocytopenia, and acute renal failure.At necropsy the main findings are necrotizing glomerular lesions with fibrinous capillary thromboses.The aetiology remains obscure, though the occurrence of a viral-like, prodromal illness (Gianantonio et al., 1964) and a tendency for cases to occur in groups (McLean, Jones, and Sutherland, 1966) have both been noted.Despite intensive research, however, no viral, bacterial, or toxic agent has yet been convincingly demonstrated.Campbell and Carre (1965) were the first to report a familial incidence in a pair of monozygotic twins who were also affected by idiopathic hyperlipaemia.Recently Hagge et al. (1967) added two cases in sibs without hyperlipaemia.The possibility, therefore, that a genetic factor may be involved has prompted us to record two further cases in sibs, neither of whom showed evidence of hyperlipaemia and in both of whom the syndrome developed in the neonatal period. Case ReportsThe mother was 21 years of age when her first child (Case 1) was bom in January 1962.In the same year she became pregnant again, but an antepartum haemor- rhage at the 33rd week resulted in stillbirth.At necropsy no abnormality was found other than evidence of intra- uterine asphyxia.Her third pregnancy ended at term in July 1964, with the delivery of a healthy infant who remains alive and well.The fourth child (Case 2) was bom in September 1965.Both parents are of Irish descent and are unrelated.The mother's blood group is A Rhesus positive, and the Wassermann reaction was negative in all her pregnancies.Atypical blood group antibodies were never demonstrated.The past, per- sonal, and family histories are non-contributory.
Source: Archives of Disease in Childhood
Authors: P. P. Anthony, A. B. Kaplan
Institution: Central Middlesex Hospital
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: bronze
OpenAlex ID: W1990349042