Cardiac tamponade as a first manifestation of possible systemic lupus erythematosus in a 3-year-old female child.

Cardiac manifestations of pediatric systemic lupus erythematosus (SLE) usually occur as an initial manifestation of the disease or within six months after the diagnosis of SLE. Pericarditis is the most frequent cardiac manifestation of SLE, but pericardial effusion causing tamponade, which has a very serious prognosis, rarely occurs, and it is even less frequent for the pericardial tamponade to be the presenting feature of SLE. In the present case which is the youngest case in the literature we report a 3 year old girl who presented to the emergency room with solely pericardial effusion causing tamponade, bilateral pleural effusion and diagnosed "possible SLE" based on American College of Rheumatology criteria.

The adjustment of post-dialysis dry weight based on non-invasive measurements in children.

BACKGROUND: The clinical criteria to assess hydration status are not always reliable. Hence, the development of techniques to estimate more accurately post-dialysis dry weight (DW) remains a major challenge. The present study evaluates the value of the inferior vena cava (IVC) diameter, plasma concentration of atrial natriuretic peptide (ANP), and plasma renin activity (PRA) in determining the DW in chronic haemodialysis children. METHODS: Twelve overhydrated haemodialysis patients (4 girls, 8 boys) with a mean age of 12.8 were admitted to the study. Clinical, electrocardiographic, telecardiographic and echocardiographic findings, IVC and collapsibility indices and plasma concentrations of ANP and PRA were investigated before and after ultrafiltration (UF) therapy. Twelve age-matched normal children were studied as controls. Analysis of variance and Dunnett's test were applied for comparisons between patients and controls. RESULTS: Following UF therapy the patients' mean IVC collapsibility index was increased from 42.3 to 53.6% and IVC index was decreased from 1.08 to 0.81 cm/m2, both statistically significant. The pre-UF therapy collapsibility and IVC indices of the patient group were significantly different from those of the control group (56.9% and 0.70 cm/m2 respectively). The patients' mean plasma concentrations of ANP were 171 +/- 47.4 pg/ml before UF, 129 +/- 51.3 pg/ml after UF and 102 +/- 38.7 pg/ml in the control group. The ANP levels of the patients showed a significant decrease following the UF therapy. PRA was measured as 0.82 ng/ml/h before UF and 1.08 ng/ml/h after UF, but the increase was not statistically significant. CONCLUSIONS: Our findings revealed increased diameter of the IVC and plasma ANP concentrations and decreased collapsibility due to overhydration. Echography of IVC may be a promising non-invasive tool to estimate the DW in haemodialysis children. Further studies providing normative values for the IVC indices in both haemodialysis and normal children are required.

A case of Marden-Walker syndrome with Dandy-Walker malformation.

A 5-month-old girl with Marden-Walker syndrome is presented. This is a rare autosomal recessive syndrome. So far, approximately 20 cases have been described in the literature. The patient was hospitalized because of difficulty in feeding and slow spontaneous movements. Her parents were first cousins. She was diagnosed with clinical findings of growth and motor retardation, typical facial appearance, congenital heart disease, arachnodactyly, joint contractures, and a Dandy-Walker malformation on magnetic resonance imaging.