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Fatal haemolytic uraemic syndrome and idiopathic hyperlipaemia in monozygotic twins.

1965; BMJ; Volume: 40; Issue: 214 Linguagem: Inglês

10.1136/adc.40.214.654

1468-2044

S. Campbell, I. J. Carré,

Hemoglobinopathies and Related Disorders

In 1932 Burger and Grutz described a previously unrecognized disorder characterized by triglyceride hyperlipaemia with variable clinical features, includ- ing attacks of abdominal pain, xanthomatous skin lesions, and hepatosplenomegaly.This condition is now usually called 'idiopathic hyperlipaemia' (Hsia, 1959).More recently, Gasser, Gautier, Steck, Sieben- mann, and Oechslin (1955) have defined the haemo- lytic-uraemic syndrome in young children.The features of this condition are renal damage with uraemia, a haemolytic anaemia with fragmentation of red blood cells, and thrombocytopenia.In this report we record the association of these two relatively rare conditions in monozygotic twins. Case ReportsTwin girls, the first children of young unrelated parents, were born in hospital on March 3, 1961.The mother was told the twins were identical; each weighed 4 lb.(1 -8 kg.).The neonatal period was uneventful and they were discharged from the maternity hospital when 51 lb.(2-5 kg.).Twin I. On August 30, 1961, she was admitted to the Royal Belfast Hospital for Sick Children.She had been ill for 12 hours with a cough, vomiting, and rapid breath- ing.On examination her temperature was 1020 F. (38. 9 C.) due to a chest infection; this was treated with a 5-day course of sulphadimidine.The haemoglobin on admis- sion was 8 g./100 ml.; 6 days later this had fallen to 6-2 g./ 100 ml.(Fig. 1).By September 7, 8 days after admission, she had be- come extremely pale and was passing blood-stained urine.There was no evidence of oedema.