Dilated cardiomyopathy in children: Review of all presentations to a children's hospital over a 5-year period and the impact of family cardiac screening.

AIM: This study aims to report the aetiology and outcome of dilated cardiomyopathy (DCM) in children from New Zealand and to assess the outcome of protocolised family cardiac investigation introduced in 2007. METHODS: Retrospective review of all patients with DCM, aged <15 years, presenting to the children's hospital between 01/01/2007 and 01/03/2012. Oncology patients were excluded. Family screening results were assessed. RESULTS: Thirty-five patients were identified. Median age 1 year: 26/35 (74%) <5 years. Ethnicity: New Zealand European 14/35 (40%), Maori 11/35 (31%), Pacific Island 5/35 (14%) and other 5/35(14%). Excluding the four cases with biopsy-proven myocarditis, family cardiac screening was documented in 15/31 (48%). Of these, 7/15 (47%) had a positive family screen. Six previously undiagnosed DCM cases were found in these families. Five of the seven familial DCM cases had evidence of viral infection at presentation. Final aetiology: Myocarditis, 15/35 (43%), with Parvovirus being the commonest virus identified 10/15(67%). Familial, seven (20%), Idiopathic, seven (20%), Others, six (17%). Overall outcome was death 10/35(28%), ongoing dysfunction 16/35 (46%) and normalisation in 9/35 (26%). The median time to death from diagnosis was 1.5 months (3 days to 3 years). Death occurred in 4/7 with familial DCM (57%) compared with 6/28 (21%) among the others (ns). Recovery occurred in 0/7 with familial DCM versus 4/4 with biopsy proven myocarditis (P < 0.05). CONCLUSIONS: Almost half of families screened had evidence of familial DCM, and these children carried a worse prognosis. Maori and Pacific Island children seem to be over-represented. Family investigation led to the identification of many pre-symptomatic individuals even when viral aetiology was suspected initially.

Sore throat: Is it such a big deal anymore?

Sore throat remains a common disease of childhood, and a major cost and cause for antibiotic prescriptions. The management of sore throat remains controversial in affluent countries with various guidelines available and overall poor adherence to those guidelines. Group A streptococcus is the commonest bacterial cause with important sequelae including acute rheumatic fever (ARF). The driver for diagnosis and treatment is still questionable. In most affluent populations it is difficult to justify antibiotic treatment on the basis of preventing ARF, whereas this remains the major driver for sore throat management in populations at higher risk of ARF. Reduction in severity and duration of symptoms may be a reasonable basis to consider antibiotic treatment, and thus accurate diagnosis of GAS pharyngitis, particularly in those with more severe symptoms. The potential role of rapid tests in diagnosis appears to be increasing.