H aem olytic-u raem ic syndrome (HUS) is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency, and is a major cause of acute renal failure in childhood.' HUS has been broadly classified into two forms: the typical or epidemic form, also known as diarrhoeaassociated (D+) HUS, and the atypical or sporadic form, diarrhoea-negative (D-) HUS.2,3 Most children with D+ HUS recover spontaneously, whereas those with D-HUS have a poorer prognosis.v' Acute mortality ranges from 5% to 10%.4,5We conducted a retrospective survey of patients with HUS in the Brisbane area over a 16-year period. Our aim was to review the features and management of HUS and to determine whether features that differed between D+ and D-HUS were predictive of clinical course and development of chronic renal failure.
Methods
AbstractObjective: To review the clinical course of haemolytic-uraemic syndrome (HUS) in children admitted to Brisbane children's hospitals between April 1979 and October 1995. Design: Retrospective case survey. Setting: Royal Children's Hospital and Mater Misericordiae Children's Hospital (the two major children's hospitals in Brisbane).Subjects: All children hospitalised for HUS. Outcome measures: Clinical and laboratory features on presentation (including typical [diarrhoea-positive, D+] or atypical [diarrhoea-negative, D-] presentation), clinical course, treatment and features on subsequent outpatient follow-up (1, 3, 6 and 12 months later), renal outcome on long term follow-up (3-16 years later).Results: 55 children (aged 2 months to 13 years) were hospitalised for HUS, but no epidemic was detected. Seven children (13%) had D-presentations, including three (5%) with T-activation caused by pneumococcal pneumonia. Thrombocytopenia was more severe and prolonged in D-patients (P
A girl presented at the age of 8 months with idiopathic infantile hypercalcaemia complicated by hypercalciuria, nephrocalcinosis and failure to thrive. Her hypercalcaemia was partially corrected by prednisolone, but resolved with the addition of cellulose phosphate. Her height and weight showed significant improvement during the treatment period. Cellulose phosphate should be considered in the management of children with idiopathic infantile hypercalcaemia and nephrocalcinosis.
Forty-five children aged 6-14 years with primary nocturnal enuresis were randomised to determine whether desmopressin is more effective than amitriptyline and whether the combination of amitriptyline/desmopressin is more effective than amitriptyline or desmopressin alone. Amitriptyline dosage was 25 mg for children 6-10 years and 50 mg for children aged 10-14 years. Desmopressin (20 micrograms) was given in the same dosage for all age groups. After a run-in period of 2 weeks, children were treated for 16 weeks and then observed for 12 weeks. In the amitriptyline group mean wet nights per week decreased from 5.8 +/- 0.9 to 3.3 +/- 1.9 (P < 0.0005); in the desmopressin group mean wet nights per week decreased from 6.0 +/- 0.9 to 4.7 +/- 1.7 (P < 0.02); in the amitriptyline/desmopressin group mean wet nights per week decreased from 6.3 +/- 0.9 to 3.3 +/- 2.5 (P < 0.0006). When comparing the groups, amitriptyline/desmopressin and amitriptyline were statistically more effective than desmopressin in week 6 (P < 0.009), week 8 (P < 0.03) and week 10 (P < 0.04). No significant side effects occurred. At this dose amitriptyline was more effective than desmopressin and the combination of desmopressin and amitriptyline did not confer any additional benefit.
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