The lack of oral anticoagulant guidelines specific to paediatric practice has led to the adoption of

The study aims were to review the clinical features of a group of patients with Noonan syndrome and to further elucidate their bleeding tendency. Eighteen patients (12M, 6F) aged 2.6–13.3 years underwent a clinical assessment, a questionnaire of their bleeding tendency and laboratory coagulation studies. Nine had cyanotic spells or breathing difficulties after birth; 11 had poor feeding or weight gain. Increased bruising or bleeding was reported in 12 (67%), four of whom had bleeding from the oral cavity. Excessive bleeding was not reported from operative procedures in other sites. Partial thromboplastin time was prolonged in 10 (56%) associated with low levels of clotting factors, particularly XI and XII. Bleeding times were normal; one had marginal thrombocytopenia. Coagulation results did not correlate with bruising history and may not predict bleeding risk. Care is required when Noonan syndrome patients undergo surgery, particularly of the oropharynx, with immediate availability of suitable blood products.

Five cases are reported of spontaneous remission of chronic childhood thrombocytopenia four or more years after 4diagnosis. Other than typical features of chronic idiopathic thrombocytopenic purpura there were no obvious markers predictive of late remission, although a slow progressive recovery was common to four of the patients. In light of this experience splenectomy is not recommended in clinically mild thrombocytopenia.

The average annual incidence in a patients admitted to hospital with ischaemic heart disease is lower among Maori than non-Maori but Maori females under 55 years are particularly susceptible. The age adjusted incidence in this group is twice that of white females. One-third of Maori patients had auricular fibrillation and large hearts and it is suggested that these patients have cardiomyopathy, probably alcohol induced, in addition to ischaemic heart disease. This group had the highest hospital mortality rate, 43%. The 30% hospital mortality rate among all Maori is three times that of non-Maori. Risk factors examined in the Maori included obesity (present in 65%), diabetes (in 30%), gout (in 23%) and hypertension (in 17%) of patients with ischaemic heart disease.